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Oxyntic gastric heterotopia (GH) in the colon is not common. Its presence in a colon tubular adenoma is even rare. A 73-year-old woman with a history of resected colon carcinoma underwent periodical colonoscopies for the removal of tubular adenomas for 12 years. In the last colonoscopy, a sessile, non-ulcerated polyp, centrally depressed, with a smooth surface, measuring 20 mm, located at 50 cm from the anal verge was excised. A histological study identified a tubular adenoma with focal low-grade dysplasia and ectopic gastric oxyntic epithelium. The GH, composed of parietal and chief cells, and was found incidentally. Oxyntic GH in a tubular adenoma is extraordinarily rare. To the best of our knowledge, there is only one previously published case. The main possible difficulties and/or errors in the diagnosis include a tissue floater or a cross-contaminant. Precise diagnosis of oxyntic GH is basic for appropriate management. Diagnosis relies on histopathological examination. The immunohistochemical study for mucin 6 (MUC6) can confirm the nature of the epithelium. Oxyntic GH has the potential to produce serious complications including tumor development. However, GH is considered a benign disease and adenocarcinoma rarely occurs in the heterotopic mucosa. The optimal treatment of oxyntic GH associated with a tubular adenoma is endoscopic complete polypectomy.

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.

Background Fine needle aspiration cytology (FNAC) is an established technique in the management of salivary gland lesions. The Milan System for reporting salivary gland cytopathology (MSRSGC) intents to standardize diagnostic categories. Current studies are trying to evaluate the diagnostic approach of this system. Methods FNAC of salivary gland lesions were retrieved over an 11-year period. 185 FNAC specimens from 182 patients were reviewed blindly and classified according to the criteria established by the MSRSGC. 136 (74.7%) patients had follow-up of their processes. Results The total number of diagnostic categories and risk of malignancy (ROM) in 185 specimens were the following: non-diagnostic 39 (21.1%; ROM 12%), non-neoplastic 35 (18.9%; ROM 0%), atypia of undetermined significance (AUS) 17 (9.2%; ROM 46.1%), benign neoplasm 75 (40.5%; ROM 4.9%), salivary gland neoplasm of uncertain malignant potential 4 (2.2%; ROM 100%), suspicious for malignancy 7 (3.8%; ROM 100%), malignant 8 (4.3%; ROM 100%). No false positives were observed in groups IVb, V, and VI in this series. The overall sensitivity, specificity, negative predictive value, positive predictive value and diagnostic accuracy were 88%, 91.8%, 96.3%, 76.7%, and 91% respectively. Conclusion The ROM reported in our study was in keeping with ROM published by the MSRSGC. This system provides standardized information for risk stratification. The category AUS encompassed cases causing uncertainty representing a challenge in management. Defining criteria for AUS category need to be refined. The system facilitates communication between pathologists and clinicians favoring improvement in patient care.

Background This study aims to evaluate the usefulness of endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) in the diagnosis of the gastric gastrointestinal stromal tumor (GIST). Methods We retrospectively investigated the efficacy and accuracy of EUS-FNAC in the diagnosis of gastric GIST. Cytological smears and cytoblock sections including immunohistochemistry and mutational studies from patients diagnosed as gastric GISTs were retrieved. Results Thirty patients (mean age 68.8 years, range 32-88 years, Male:Female 1:1.7) were diagnosed by cytological and cytoblock study to have GIST. The size of tumors ranged from 1.6 to 25 cm (mean 6.0 cm). 7 (23%) cases were incidentally discovered. Location was: gastric body 13 (43.3%), fundus 8 (26.7%), antrum 7 (23.3%), cardia 2 (6.7%). The study of removed tumors was correlated with the cytological findings. The sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy were 75%, 100%, 100%, 46%, and 96%. There were no false-positive cases. The preoperative risk assessment of 27 cases with cytoblock were: none 3 (11.1%), very low 8 (29.6%), low 12 (44.4%), high 3 (11.1%), insufficient clinical data 1 (3.7%). The follow-up varied from 2 to 120 months (mean 46.7 months). Only 1 patient of the high-risk group died. The most frequent mutations found were those of c-KIT in exon 11. Conclusions Pathological diagnosis was based on a combination of cytological, histopathological, and immunohistochemical features. EUS-FNAC is a reliable, accurate, and safe method for the diagnosis of GIST. The cytoblock allows tumor risk classification and mutational study of the cases.

We investigated the efficiency and accuracy of endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) in the diagnosis of gastrointestinal leiomyoma (GIL). Between January 2009 and May 2018 we performed 795 EUS-FNAC studies of lesions of the gastrointestinal (GI) tract for various clinical indications. A diagnosis of GIL by cytological and cell block study was made in 14 patients (57.1% males, mean age 53.6 years, range 22-84 years). 7 tumors (50%) were detected incidentally. The lesions ranged in size from 2 to 10cm (mean size 4.4cm). The location of the tumors was: esophagus 7 (50%), stomach 6 (42.9%) and rectum 1(7.1%). The mean size of the symptomatic tumors was 5.2cm (range 3-10cm). The follow-up of the 14 patients varied from 1 to 108 months (median 39.5 months), during which no recurrence or evidence of lesion progression was observed. Imaging alone was not sufficient for an accurate diagnosis to be made. The pathological diagnosis was based on a combination of cytological, histopathological, and immunohistochemical features. The intracytoplasmic eosinophilic globule is a useful marker of paucicellular GIL differentiating it from gastrointestinal stromal tumor and leiomyosarcoma. EUS-FNAC is a reliable, accurate, and safe method for the diagnosis of GIL.

Type I spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by the loss or mutation of the survival motor neuron 1 (SMN1) gene. The reduction in SMN protein levels in SMA leads to the degeneration of motor neurons and muscular atrophy. In this study, we analyzed the nuclear reorganization in human skeletal myofibers from a type I SMA patient carrying a deletion of exons 7 and 8 in the SMN1 gene and two SMN2 gene copies and showing reduced SMN protein levels in the muscle compared with those in control samples. The morphometric analysis of myofiber size revealed the coexistence of atrophic and hypertrophic myofibers in SMA samples. Compared with controls, both nuclear size and the nuclear shape factor were significantly reduced in SMA myonuclei. Nuclear reorganization in SMA myonuclei was characterized by extensive heterochromatinization, the aggregation of splicing factors in large interchromatin granule clusters, and nucleolar alterations with the accumulation of the granular component and a loss of fibrillar center/dense fibrillar component units. These nuclear alterations reflect a severe perturbation of global pre-mRNA transcription and splicing, as well as nucleolar dysfunction, in SMA myofibers. Moreover, the finding of similar nuclear reorganization in both atrophic and hypetrophic myofibers provides additional support that the SMN deficiency in SMA patients may primarily affect the skeletal myofibers.

Objective Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique for investigating hilar and mediastinal lymphadenopathy. This study reports eleven cases in which EBUS-TBNA was used to assess mediastinal and hilar lymph nodes for the presence of metastatic melanoma. Material and method A retrospective study was performed of all patients who had a history of melanoma and underwent EBUS-TBNA to assess hilar or mediastinal lymphadenopathy for the presence of metastatic melanoma. In seven cases, molecular analysis to detect mutations in the BRAF gene was also used. Results Eight patients had been diagnosed with malignant melanoma in the past (mean 54.4 months, range 18 to 115 months) while in the other three this tumor was primarily diagnosed in the staging phase. The male-female ratio was 6:5, and the mean age was 60.3 years (range 42 to 88 years). The mean hilar or mediastinal lymph node size detected with computed tomography was 3.0 cm (range 1.1 to 8.1 cm). Eight (72.7%) cases had metastases to the lung associated with metastases in the mediastinal lymph nodes. In four (50%) of these cases, the lung metastasis was solitary. Three (27.3%) cases had metastases in the mediastinal lymph nodes in absence of lung metastases. Metastatic melanoma was diagnosed by cytology and confirmed by cell block study with immunohistochemistry in all cases. BRAF mutations were detected in two (28.6%) of seven cases studied. Conclusion Cytology and tissue samples obtained from EBUS-TBNA are adequate to detect metastatic melanoma and permit in some cases the determination of biomarkers and identify the presence or absence of mutations in the BRAF gene. The procedure is safe, fast, and precise for the staging of melanoma.

Abdominal elastotic deposits are uncommon lesions that often presents as polyps. They show three histological patterns: fibroelastosis, angioelastosis, and elastofibroma. We describe 23 cases including rare locations, such as mesentery, greater omentum, hernia sac, spleen, peripancreatic fat, and hypodermal fat. The age of the patients ranged from 49 to 93 years (mean, 76.8 years). Most lesions were discovered incidentally in the microscopic study. The most frequent locations were peritoneal subserosa (43.5%) and mesentery∕mesocolon∕greater omentum (39.1%). The most common pattern was fibroelastosis (69.6%) followed by angioelastosis (26.1%). We observed one case of omental elastofibroma. A review of the 14 abdominal elastofibromas described including our case revealed that the age of the patients ranged from 45 to 88 years (mean, 68.5 years). Female predominance is striking (M:F, 1:12). The most common site was the stomach (50%). The greater omentum (14.3%), small intestine (7.1%), and pancreas (7.1%) are very rare sites for this lesion. Only one case before ours has been published in the greater omentum. The size of the lesions ranged from 0.7 cm to 8 cm (mean 3.2 cm). In 36.4% of the cases located in the digestive tract, the mucosa did not show alterations. Ulcerations (36.4%) or polypoid excrescences (18.2%) were mostly observed. Six (42.9%) cases were asymptomatic and six (42.9%) cases simulated a neoplasm. Two cases were associated with elastofibromas in other locations. Differential diagnosis includes amyloidoma, elastofibrolipoma, mesenteric elastic vascular sclerosis in neuroendocrine tumors, diverticular disease elastosis, pseudoxanthoma elasticum, pulse granuloma, and digestive lesions in patients treated with D-Penicillamine.

Clear cells in thyroid neoplasms can take two main forms: balloon-shaped and signet-ring cells. Balloon-shaped cell change in follicular adenoma is rare. A review of the literature has revealed only 20 previously published cases. We report herein a new case in the right thyroid lobe of a 45-year-old man. The clinicopathological data of the 21 cases including our paper have revealed that the ages of the patients ranged from 22-70 years, with a mean of 41.6 years. There was a clear predominance in women (M:F, 1:6). The most frequent location was in either of both lobes (81.8%), rarely affecting the isthmus. One case was observed in an ectopic thyroid in the submandibular region. The size ranged from 0.7-5.5 cm (mean 2.9 cm). The type of surgical intervention where this data was reported it was lobectomy for 10 (55.5%) cases, thyroidectomy for six (33.3%) cases, and simple excision for two (11.1%) cases. In one patient, the lobectomy was accompanied by cervical lymph node dissection. No cases recurred or extended outside the thyroid. The main differential diagnoses include intrathyroidal clear cell tumor of parathyroid origin, clear cell carcinoma of follicular, oncocytic, papillary, medullary or undifferentiated (anaplastic) origin, paraganglioma, metastatic clear cell carcinoma, especially of renal origin, metastatic balloon cell melanoma, and clear large-cell lymphoma. A thyroid lesion showing clear cell change constitutes a diagnostic challenge in cytological and biopsy diagnosis. Careful observation of the routine techniques along with the aid of an adequate immunohistochemical panel is essential to reach a correct biopsy diagnosis.

Antrochoanal polyps (ACPs) are an infrequent clinical entity. Cholesterol granulomas (CGs) are commonly associated with chronic middle ear disease but are rare in the paranasal sinuses. We describe a case of a 10-year-old girl with a concomitant CG in an ACP which was surgically excised by nasosinusal endoscopic surgery. To our knowledge, there are only five previously published cases of CGs in ACPs and of these, only two were pediatric cases. We describe a third case in the youngest patient yet reported. Increased intrasinus pressure may affect venous and lymphatic drainage, leading to hemorrhages with hemolysis and deposition of cholesterol crystals and their esters initiating the formation of granulomas in the polyp. In addition, the insufficient lymphatic drainage prevents the complete elimination of lipids, contributing to the formation of cholesterol granulomas. The treatment and the outcome of an ACP associated with a CG are the same as for usual ACPs.

Myofibroblastic sarcoma (MFS) is an uncommon tumor rarely located in the breast. Ionizing radiation is a carcinogen capable of inducing sarcomas through DNA damage. A 42-year-old woman was diagnosed with synchronous bilateral breast infiltrating ductal carcinoma with axillary lymph node metastases on the left side. After modified left radical mastectomy and simple right mastectomy, she underwent postoperative radiation with a total volume dose of 50 Gy that included the thoracic wall and the left axillary-supraclavicular region. After a latency period of 6 years and 4 months, the patient developed an MFS in the area of radiation (mammary upper outer quadrant). To our knowledge, only 11 cases of MFS have been previously published in the breast. The study of the 12 cases including the present one revealed that the ages of the patients ranged from 42 to 86 years (mean 60.3 years). There was a clear difference concerning sex (M:F, 1:5). The average duration of the lesion varied from 1 week to 8 months (mean 3.3 months). The size ranged from 2.2 to 22 cm (average 5.1 cm). The tumors showed frequent mitosis and areas of necrosis. The percentage of recurrences, metastasis, and death due to the tumor was 27.3%, 36.4%, and 27.3% respectively. MFS cases differ from those affecting extramammary regions. They are more common in females and show a greater degree of aggressiveness. Correct diagnosis of mammary MFS requires morphological and immunohistochemical study. We present for the first time a case of MFS of the breast induced by radiotherapy.

Mesonephric remnants are embryonic vestiges of the mesonephric (Wolffian) ducts which regress during normal development. These remnants have been uncommonly reported in the female and male reproductive tract as a spectrum of morphologic lesions that can be misdiagnosed as carcinoma. One case of mesonephric remnant hyperplasia of the jejunal mesentery incidentally found in a 47-year-old man is herein reported. This is the first description of mesonephric hyperplasia arisen in the mesentery. The presence of ducts, tubules, and cysts lined by bland, epithelial, cuboidal cells with scant cytoplasm, and diffuse pseudoinfiltrative growth pattern can raise the possibility of neoplasia. Immunohistochemically, mesonephric epithelia have a characteristic staining. CD10 highlights the apical-luminal aspect of the cells. Besides, intense reactivity is showed for high-molecular-weight cytokeratin (CK), CK7, bcl2, and vimentin. The main differential diagnosis includes mesothelial hyperplasia, epithelial mesothelioma, well-differentiated neuroendocrine tumor, and infiltration due to acinar adenocarcinoma of the prostate. However, a detailed microscopic study with the aid of immunohistochemistry helps separate mesonephric remnants from malignant processes. The mesonephric hyperplasia of the mesentery we have reported adds to the spectrum of mesonephric remnants a new location. Familiarity with this lesion is indispensable to avoid overdiagnosis.

Müllerianosis is the term used to designate lesions composed of an admixture of two or three types of müllerian-derivation glands in heterotopic location. In this report, we describe a case of incidental vaginal müllerianosis in a 59-year-old woman who underwent rectosigmoidectomy for rectal adenocarcinoma. In the vaginal cuff removed for neoplastic invasion, a separate multilocular mass measuring 1.5cm was found. The microscopic examination of the vaginal wall revealed endosalpingeal, endocervical and endometrial dilated or cystic glands with predominance of the endosalpingeal epithelium. Müllerian epithelium showed positivity for cytokeratins 7 and 8/18, high molecular weight cytokeratin, estrogen receptor alpha, and androgen receptor. The periglandular stroma was condensed and reactive for smooth-muscle actin, h-caldesmon, and CD10. To the best of our knowledge, a case of vaginal müllerianosis has not been previously reported. This lesion should be differentiated form vaginal adenosis and primary well-differentiated vaginal adenocarcinoma. The vagina should be added to the list of locations in which müllerianosis can be observed.

Background Benign melanocytic nevi (MN) of the anal canal are exceptional and require adequate differential diagnosis. There are no data on incidence of these lesions. Only a single case report of hemorrhoid with an MN has been reported. The necessity of routine pathologic evaluation of hemorrhoidectomy specimens has been questioned. Material and methods The authors undertook a retrospective histologic study of the hemorrhoidal tissue obtained in a series of 1918 consecutive hemorrhoidectomies performed between January 2004 and November 2012. Results Incidental hemorrhoidal MN were detected in 4 (0.21%) patients. The ratio observed was 1 nevus in 480 specimens. Lesions were intradermal or purely junctional in nature. There were no mitoses or architectural disorder. An intradermal nevus showed localized pagetoid melanocytes. The mean age of the patients was 56.5 years (range 47-73 years). The mean size of nevi was 5.86 mm (range 1.89-13.86 mm). All cases were present in external hemorrhoids. Conclusions Although uncommonly, incidental MN can be observed in hemorrhoidal tissue. They may show features of flexural nevi and pagetoid melanocytes. Routine histopathological study of hemorrhoidectomy specimens would help to detect benign or malignant melanocytic tumors of the anal canal as these neoplasms can be easily missed clinically. Furthermore, this practice would allow early diagnosis of significant associated processes.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare condition of uncertain etiopathogenesis which usually presents with either intermittent or progressive dysphagia. A review of the literature revealed very few studies of its histopathology. We report the case of a 70-year-old diabetic man who died after a road traffic accident. The patient had no history of dysphagia. The entity was discovered incidentally during the medico-legal autopsy. The esophagus showed numerous dilated pseudodiverticula confined to the submucosa and lined by both stratified squamous and cuboidal epithelium. EIPD can remain stable and asymptomatic. This case demonstrates the importance of a thorough investigation during a forensic autopsy.

Intravascular histiocytosis (IVH) is a rare condition of uncertain pathogenesis often associated with rheumatoid arthritis (RA) exclusively observed in the skin. In a retrospective study of 207 consecutive cases of aortic valve disease, we observed two cases of IVH characterized by the presence of thin-walled, dilated blood vessels containing collections of CD68+ and CD163+ mononuclear histiocytes. Immunostains for CD31, CD34, and D2-40 confirmed the intravascular location of these histiocytes. One of the two cases was associated with RA. This case was observed among 41 cases of RA with calcific aortic valve stenosis. The other case was detected among 152 cases of calcific aortic valve stenosis in isolation. A total of 14 valves showed no calcification. IVH can manifest in the aortic valve and be associated with systemic disease. In contrast to other cases, the vessels observed in this study exhibited negative expression of the lymphatic marker D2-40. Our findings expand on the previously described location features of IVH.

A woman underwent surgical intervention for a carcinoma of the ovary. In the intervention, a submucosal nodule of the ileum was found. Pathological study revealed a spindle cell lipoma (SCL). This case revealed the presence of CD34-positive spindle and stellate cells with dendritic cytoplasmic prolongations, a feature shared with dendritic fibromyxolipoma. Fluorescence iniiiin situ/i/i/ihybridisation analysis showed 13q14 heterozygous deletion. Spindle cell lipoma of the small intestine has not been previously reported. Spindle cell lipoma, although rare, should be included among the benign mesenchymal lesions of the small intestine. This report extends the range of locations in which this tumour is found to arise.

Taxanes are antineoplastic drugs that can cause dermatotoxicity which can mimic an intraepidermal carcinoma. A 65-year-old woman presented with a cutaneous eruption suggestive of a paraneoplastic syndrome. Imaging studies showed multiple peritoneal nodules and associated ascites. A sample taken from the greater omentum revealed an adenocarcinoma. Clinical data and family history pointed to a gynecological origin of the tumor and the patient was treated with carboplatin and paclitaxel. A new cutaneous biopsy showed that the epidermis was acanthotic with atypical keratinocytes, abundant mitoses, and apoptotic figures, arising concerns of malignancy. According to the Plummer and Shea criteria, the lesion was ultimately interpreted as reactive cutaneous hyperplasia and expression of the taxane effect. We report, for the first time, paclitaxel-induced histologic changes on a previous cutaneous eruption. Pathologists should be aware of the profound cytopathic effects of taxane therapy in order to interpret skin biopsies of patients undergoing this treatment.

Pyogenic granuloma (PG) is a polypoid lobular capillary hemangioma rarely described in the stomach. We report two cases aged 72 and 66 years. A review of the literature on gastric PG, including the present cases, yielded ten patients. There were six males and four females. The age of the patients ranged from 35 to 82 years with a mean of 58.9 years. The lesions were all solitary, except one case of multiple lesions in the corpus and antrum. PG can be located in any part of the stomach. The most common site was the antrum. The mean maximum diameter of the lesions was 14.5mm (range 7-30mm). Most lesions were pedunculated. Pain or discomfort (epigastric, right upper quadrant or chest), upper gastrointestinal bleeding, and melena were the most common clinical symptoms. Iron deficiency anemia was the rule, often requiring blood transfusion. Five patients underwent snare polypectomy, four endoscopic mucosal resection, and one laser irradiation. The follow-up ranged from two weeks to two years. There were no recurrences. Pathologists should be familiar with this condition in order to avoid overdiagnosis as a malignant vascular tumor.

Very rarely, a primary peritoneal serous carcinoma can be observed in a hernia sac. We herein describe a low-grade serous primary peritoneal carcinoma incidentally found in a postmenopausal woman following examination of the femoral hernia repair sac. Our case is significant for its unusual presentation. The lesion initially appeared as a 0.3-cm tumor that disseminated in the peritoneum, persisted, and progressed for 75 months. The absence of ovarian disease indicated a primary peritoneal origin. Tumor cells were immunohistochemically positive for PAX8, claudin-4, and VE1, excluding the possibility of being of mesothelial origin. Recognition that a low-grade serous primary peritoneal carcinoma can be incidentally found in a hernia sac should simplify future diagnoses. Immunohistochemistry is helpful in making the correct diagnosis.

We report the case of a 60-year-old man who was receiving immunosuppressive therapy for a bilateral lung transplant and presented with a crusted, violaceous plaque on the left hand. Based on histopathology and microbiological culture the patient was diagnosed with infection by Alternaria species. Treatment with itraconazole led to complete resolution of the skin lesion. Forty months later he developed four reddish, nodular, skin lesions on the left leg. Analysis of a biopsy from one of these lesions using histopathologic and molecular techniques identified a mold that shared 98% homology with a strain of Alternaria triticina. Alternaria species belong to a group of dematiaceous fungi that cause opportunistic infections in humans. The incidence of these infections is increasing, mainly in transplant centers. To the best of our knowledge, this is the first reported case of a human infection caused by A. triticina Presentamos el caso de un hombre de 60 años en tratamiento inmunosupresor por un trasplante pulmonar bilateral que presentó una placa violácea costrosa en la mano. Se diagnosticó de infección por Alternaria spp mediante estudio histopatológico y cultivo microbiológico. El tratamiento con itraconazol condujo a la resolución completa de la lesión cutánea. Cuarenta meses más tarde el paciente desarrolló 4 lesiones nodulares rojizas en la pierna izquierda. Las muestras obtenidas de una de las lesiones fueron estudiadas mediante histopatología y técnicas moleculares. La secuencia de ADN que se obtuvo del germen causal mostraba una homología del 98% con una cepa de Alternaria triticina. Las especies de Alternaria forman parte del grupo de hongos que componen las feohifomicosis susceptibles de causar infecciones oportunísticas humanas. La incidencia de estas infecciones está aumentando, sobre todo en centros de trasplante. De acuerdo con nuestra información el presente caso es el primero en el que A. triticina es causante de una infección humana Acknowledgement: We thank Manuel Cuenca Estrella and Emilia Mellado of the Instituto Nacional de Salud Carlos III in Madrid for confirmation of the identity of the fungus.

Well-differentiated papillary mesothelioma (WDPM) is a tumor of uncertain malignant potential that usually occurs as a multifocal lesion of the female peritoneum, and is incidentally found at the time of surgery. We present here a multifocal case that had arisen from the lining of a hernia sac. To our knowledge, only four cases of this event have been previously described. A review of the five cases reported, including our case, revealed that the mean age of the patients was 56.6 ± 8.35 years. There was predominance in men (4:1). In four cases, the lesion was incidental. Most tumors were found in inguinal hernias. Four cases presented with gross abnormalities in the hernia sac. All the five patients were alive with no evidence of WDPM after a mean follow-up of 38.6 months. Extensive sampling of this rare lesion helps to rule out an epithelial malignant mesothelioma and prevents overtreatment.

Context.—Neuroendocrine lung neoplasms are a heterogeneous group of tumors with different clinical behavior and prognosis. Objectives.—To evaluate the expression of p53, KLF4, and p21 in neuroendocrine lung neoplasms and to analyze the influence that expression has on the prognosis of those tumors. Design.—All neuroendocrine lung neoplasms (N = 109) resected in our institution were reviewed, with the collection of histologic slides and paraffin blocks of 47 typical carcinoids (43%), 9 atypical carcinoids (8%), 35 large cell neuroendocrine carcinomas (32%), and 18 small cell lung carcinomas (17%), as well as 10 tumorlets (100%). Four tissue microarrays were performed. Follow-up was assessed in all cases (119 of 119; 100%). Results.—p53 protein immunostaining results were negative in both the tumorlets and typical carcinoids and were overexpressed in 11% (1 of 9) of the atypical carcinoids and in 68% (36 of 53) of the carcinomas. KLF4 results were positive in all tumorlets (10 of 10; 100%), 32% (15 of 47) of the typical carcinoids, 44% (4 of 9) of the atypical carcinoids, and 62% (33 of 53) of the carcinomas. p21 expression did not differ among the groups. The lack of KLF4 and p21 expression was associated with an accumulation of aggressive features in typical carcinoids (P = .04 and P = .004, respectively, Fisher exact test). Conclusions.—p53, KLF4, and p21 showed altered expression patterns in pulmonary neuroendocrine neoplasms. Lack of KLF4 and p21 expression was associated with accumulation of aggressive features in typical carcinoids.

Low-molecular weight heparins (LMWHs) are the standard agents used for the prevention and treatment of thromboembolic disorders. Despite their widespread use, reports on adverse effects from LMWHs are uncommon. The present report describes a 23-year-old woman with a 3.8-cm solitary, fibrofatty, poorly demarcated nodule involving the subcutaneous tissue of the infraumbilical abdominal wall that appeared 4 years after iterative local administration of LMWH. Microscopically, the nodule showed atypical cells, with bizarre nuclei in the septa of cellular fibrous tissue dissecting the adipose lobules, in the absence of any other malignant changes. To the best of our knowledge, this kind of LMWH-related lesion has not been previously reported. The lesion may result from the combination of an idiosyncratic reaction to the LMWH with local trauma being a probable promoting factor. Alternatively, this reactive proliferation may be entirely due to the trauma represented by repeated injections. This rare process could easily be confused with liposarcoma. As the clinical history of injections is often not provided in the requisition form, pathologists should be aware of this adverse effect from LMWH to avoid misdiagnosis and excessive treatment.

Cryptosporidium localization to the stomach is uncommon. We report a case of a 56-year-old, HIV-positive, male patient with severe immunosuppression after antiretroviral treatment dropout who presented with epigastric discomfort, nausea and vomiting. The abdominal CT with contrast showed an important concentric thickening of the gastric antrum wall. Upper gastrointestinal endoscopic evaluation revealed lack of distensibility of the gastric wall and marked thickening, stiffness, distortion, and erosions of mucosal folds involving the antrum region. The endoscopy was clinically reported as gastric malignancy suggestive of lymphoma. The histopathologic diagnosis was cryptosporidiosis. To our knowledge, gastric cryptosporidiosis simulating malignancy has not been previously described. This type of lesion should be included in the differential diagnosis of AIDS patients with lesions simulating gastric malignancy.

Papillary dermal elastosis is a rare clinicopathological entity with only 1 case reported. The authors herein report a new case. A 38-year-old woman presented with a 10-month history of slowly progressive appearance of multiple, 1-2 mm, pruritic nonfollicular papules, located on the back. The study of the biopsy with the ordinary staining revealed numerous dermal papillae showing increased elastosis. The Verhoeff elastic stain demonstrated numerous foci of clumped and curled elastic fibers in the papillary dermis. The elastic component of the reticular dermis was normal. This case report confirms this entity and can contribute to a better understanding of the spectrum of the fibroelastolytic entities.

Valvular hemangioma incidence is extremely low. In this report, we describe a 62-year-old man who presented with mild edema of the lower limbs. An echocardiogram revealed an incidental 1.3-cm diameter mass on the anterior mitral valve leaflet for which he underwent surgical resection and mitral valve replacement. Histopathological examination showed a lymphocyte-rich capillary-cavernous hemangioma. The exuberant lymphoid stroma is unusual for hemangioma and represents an undescribed pattern of cardiac hemangioma. Including the present report, only 13 cases of mitral valve hemangioma have been reported to date. Most patients are adult. Mitral hemangioma originates in the atrial aspect of the valve and involves more commonly the anterior leaflet. The average maximum diameter of the lesion is 1.7 (S.D.=0.75) cm. Pure cavernous hemangioma is the predominant type of mitral hemangioma. Most of them are described as pedunculated or polypoid. Surgical excision appears to be curative. Recurrences have not been reported. Lymphocyte-rich cardiac hemangioma represents a peculiar type of hemangioma which should be included in the differential diagnosis of other vascular lesions.

Accessory breast tissue is a subcutaneous remnant persisting after normal embryological development of the breast. It occurs most frequently in the axilla. Fibroadenomas in axillary breast tissue are rare. We report the case of a 29-year-old female patient who presented a fibroadenoma arising in the accessory breast tissue of the right axillary fossa. The neoplasm showed foci of sclerotic fibroma-like stroma. The patient had no signs of Cowden's syndrome. To the best of our knowledge, a lesion of this kind has not been previously reported. This stromal change suggests an uncommon involutional phase of the fibroadenoma with production of sclerotic and hypocellular collagen. The lesion should be differentiated from extraneural perineuroma, from the changes in fibroadenomas in Cowden's syndrome, from sclerosing lobular hyperplasia (fibroadenomatoid mastopathy) and from pseudoangiomatous stromal hyperplasia.

We report here one case of renal oncocytoma producing focal extracellular mucinous secretion in a 47-year-old woman. To the best of our knowledge, the presence of mucinous secretion in this tumor has not yet been reported. Mucin production, despite its low frequency, can be considered an additional feature of renal oncocytoma. Therefore, oncocytoma should be added to the list of parenchymal renal tumors that can show significant mucin secretion, and it should be included in the inventory of morphologic variations of oncocytoma which may cause diagnostic difficulties.

Xanthogranulomatous orchiepididymitis (XGO) is a rare inflammatory and destructive disease of the testis that may simulate testicular malignancy. To the authors' knowledge only 19 cases of XGO have been previously reported. Herein is reported the case of a 52-year-old man with XGO who presented with a left testicular mass and pyogenic discharge from the scrotum. Ultrasound examination indicated heterogeneous testicular areas suggestive of neoplasia. Testicular tumor markers were normal. Orchidectomy was performed and histopathology showed XGO and a concurrent, occult typical seminoma. As far as the authors know the coincidence of these two lesions has not been documented previously. XGO may mimic testicular malignancy and may coexist with it. Pathologists should be aware of this concurrence of lesions to avoid potential misdiagnosis. Wide sampling of the orchidectomy specimens with XGO is mandatory to exclude an occult seminoma.

Eosinophilic (Langerhans' cell) granuloma is most common in children and is unusual in the elderly. A cutaneous lesion as the sole manifestation of adult Langerhans' cell histiocytosis is rare. We report an 81-year-old man who presented with a noduloulcerative lesion on the lower lip. A punch biopsy suggested a lymphoepithelioma-like carcinoma. In the absence of immunohistochemistry, the large pale cells with atypical lobular nuclei and patent nucleoli, with an overlying ulceration, were interpreted as carcinoma cells. Definitive diagnosis was established after complete resection of the lesion aided with the use of immunohistochemistry. The proliferating cells were S100, CD1a and CD207 (langerin) positive and cytokeratin, epithelial membrane antigen, CD15, CD30, melan A and carcinoembryonic antigen negative. To our knowledge, eosinophilic (Langerhans' cell) granuloma of the lip has not been described. The case constituted a diagnostic challenge because of its unusual clinical presentation and its histopathological similarity to certain other benign and malignant entities. Because there was no other organ involved, the patient was treated with complete surgical excision without recurrence or other organ involvement in the 4-month follow-up period. Labial eosinophilic (Langerhans' cell) granuloma should be considered in the histological differential diagnosis in cases with atypical polymorphous infiltrates including large pale cells.

Background: Melanocytic nevi from the genitalia are uncommon. Nevi on the vulva are much better described than nevi on male genitalia. To our knowledge, a systematic study of preputial melanocytic nevi has not been reported. Objective: To investigate the frequency of clinically unapparent melanocytic nevi on a series of preputial excisions. Materials and Methods: We undertook a prospective histologic study of the prepuce obtained in a series of 372 consecutive circumcisions for phimosis performed during the period between January 2000 and December 2002. Results: Incidental preputial melanocytic nevi were detected in four (1.1%) patients. Lesions were dermal in nature, most of them showed pigment in superficial dermal nests and had pseudovascular spaces. There were no cytologic atypia, mitotic figures, architectural disorder or inflammatory infiltrate. The mean (standard deviation, SD) of the maximum diameter was 1.08 (0.85) mm (range 0.34–1.79 mm). The mean age (SD) of the patients was 41.5 (4.95) years (range 29–58). Conclusions: Incidental melanocytic nevi, although uncommonly, can be observed in the prepuce. They are detected in adults and may pose a diagnostic challenge when they are detected in the setting of concurrent malignant melanoma.

Development of malignant tumors is a rare but well known complication in chronic lymphedema (CL). We report herein a cutaneous diffuse large B-cell lymphoma of the leg associated with CL. An 89-year-old man presented with multiple cutaneous lesions on his right limb that showed a CL. Dermatological examination disclosed multiple violaceous, firm, slightly infiltrated nodules on the anterior aspect of the leg and the dorsum and sole of the foot. A biopsy of one nodule of the leg disclosed a diffuse large B-cell lymphoma, type of the legs. There was no evidence of lymphadenopathy on computed tomography (CT) scans of the chest, abdomen, and pelvis. A bone marrow aspiration and biopsy showed normal results. The patient was treated with local radiotherapy at a dose of 40 Gy, obtaining a highly significant, almost complete, clinical remission. A literature search identified 11 additional cases of primary cutaneous lymphoma associated with CL. An inadequate lymphatic drainage may make the lymphedematous region an immunologically vulnerable area, predisposing to neoplasia.

A uterine arteriovenous malformation (AVM) is an uncommon cause of uterine bleeding. Location of this lesion in the uterine cervix is exceptional. We report a case of a 34-year-old woman who presented with chronic menorrhagias and hypochromic anemia. A sonographic study revealed a 10-cm, fundal, intramural, uterine well-circumscribed mass that distorted the endometrial cavity. The patient underwent hysterectomy for a large uterine leiomyoma. The pathological study revealed an incidental AVM of the posterior half of the cervix measuring 5.5 cm in major diameter. We suggest that in our case cervical AVM might have occurred due to a large corporal leiomyoma distorting the uterine circulation. Differential diagnosis includes capillary hemangioma, venous malformation, or arteriovenous fistula.

Pseudoepitheliomatous hyperplasia is a reactive proliferation of benign squamous epithelium that can mimic squamous cell carcinoma. Herein is a report on this type of reaction on two subgemmal neurogenous plaques in a lingual circumvallate papilla. To the authors' knowledge this is the first report on this combination of lesions. A 66-year-old man died with disseminated squamous cell carcinoma of the lung. At autopsy the tongue was found to have an incidental pseudoepitheliomatous hyperplasia on two subgemmal neurogenous plaques with ganglion cells. Subgemmal neurogenous plaque should be included in the differential diagnosis of causes of lingual pseudoepitheliomatous hyperplasia. Awareness of the morphological spectrum of subgemmal neurogenous plaques can avoid the overdiagnosis of these lesions as neural tumors or squamous cell carcinoma.

Adenoid squamous cell carcinoma (ASCC) or acantholytic squamous cell carcinoma is a well-recognized variant of squamous cell carcinoma. ASCC commonly occurs in the sun-exposed areas of the skin and has only rarely been observed in mucosal sites. We report a case of ASCC in the larynx of a 75-year-old man with a history of odynophagia and dysphonia of 3 months' duration that presented as an exophytic lesion on the right vocal cord. Biopsy was performed and the histological diagnosis was squamous cell carcinoma. Treatment consisted of total laryngectomy and radical neck dissection. Microscopically, the tumor showed a prominent alveolar pattern with cystic degeneration of the neoplastic epithelium and formation of pseudoglandular structures. In the lumina there were acantholytic cells. Transition areas from conventional squamous cell carcinoma to adenoid pattern were found. To the best of our knowledge this is only the third case of ASCC reported in the larynx. We reviewed the nine mucosal ASCCs of the upper aerodigestive tract reported. The assertion that these tumors are associated with an aggressive behavior and poor prognosis cannot be estimated in this review as the number of cases is small and the tumors are located in different places.

Isolated left ventricular non-compaction is a rare unclassified cardiomyopathy characterized by arrest of normal embryogenesis that results in the persistence of intertrabecular recesses and the development of a spongy myocardium. The pathological diagnosis is often first established at autopsy. Described herein is the case of a 57-year-old man with isolated non-compaction of the left ventricle who had a 17 year history of worsening heart failure and was successfully treated with heart transplantation. To the authors' knowledge only seven adult patients with non-compaction cardiomyopathy have been reported to have undergone heart transplantation. Including the present case the mean age of the adult patients at transplantation was 39.5 years (range, 18-60 years). The male : female ratio was 3:1. Both ventricles were involved in 37.5% of cases. The mean follow up was 6.3 years (range, 2 months-17 years). One patient died because of a malignant tumor 9 years after transplantation. The morphological pattern of the isolated ventricular non-compaction represents a pathological entity discernible from other cardiomyopathies and should be classified as a specific cardiomyopathy. It is likely that surgical pathologists will encounter this entity more frequently due to involvement in transplantation teams.

It is well known that the cell nucleus is organized in structural and functional compartments involved in transcription, RNA processing and protein modifications such as conjugation with SUMO-1 and proteolysis. Promyelocytic leukaemia (PML) bodies are dynamic nuclear structures that concentrate PML protein, SUMO-1 and several sumoylated and non-sumoylated protein regulators of nuclear functions. PML bodies and their associated CBP has been involved in neuronal survival. By light and electron microscopy immunocytochemistry and in situ hybridization we reported the presence, in non-pathological conditions, of a large PML-nuclear inclusion (PML-NI) in human supraoptic neurons. This inclusion appears as a single nuclear structure composed of a capsule enriched in PML, SUMO-1 and CBP proteins and a central lattice of filaments immunoreactive for class III β-tubulin, ubiquitinated proteins and proteasomes. Furthermore, the PML-NI concentrates the SUMO-conjugating enzyme E2 (UBC9). The PML-NI may be considered a nuclear factory involved in sumoylation and proteolysis via ubiquitin-proteasome system, two nuclear pathways engaged in the control of the nucleoplasmic concentration of active transcriptional regulators. Interestingly, the structural and molecular organization of the PML-NI is related to the Marinesco bodies, age-associated ubiquitinated intranuclear inclusions, and to the intranuclear rodlets enriched in class III β-tubulin, which are nuclear structures markedly decreased in Alzheimer's disease. © 2005 Elsevier Inc. All rights reserved., This study was supported by “Dirección General de Investigación Científica” from Spain (BFI2002-0454; BFU2005-01030), “Fondo de Investigación Sanitaria” from Spain (Red CIEN C03-C06) and Fundación Marqués de Valdecilla from Santander (API/05/04).

Valvular epithelioid hemangioendotheliomas (EHE) are exceptional. To the authors' knowledge only four cases have been reported. Herein is described an EHE incidentally detected in the mitral valve of a 69-year-old woman with chronic rheumatic valvular heart disease. The 0.4 cm lesion was situated in the anterior leaflet and was discovered in the pathological study after mitral valve replacement. The patient was alive and well 6 months after operation. Review of the literature including the present report, revealed that the mean age was 60.2 years (range, 49-69 years). Symptomatic patients had clinical features of valvular disease or embolism. Each of the four cardiac valves can be affected. Mean tumor size was 2.4 cm (range, 0.4-8 cm). In 40% of cases the EHE was an incidental finding at autopsy or in a removed valve. In two cases the involved cusp was affected by chronic rheumatic disease. In conclusion, EHE is a rare lesion that can be an incidental finding and it should be considered in the differential diagnosis of valve tumors. Although EHE can present a histologically benign appearance, the correct pathological diagnosis is clinically important because the lesion can be potentially malignant. Regular follow up is suggested due to this potential.

Bronchioloalveolar carcinoma is a distinctive subtype of pulmonary adenocarcinoma, without effective therapy, although there have recently been some attempts to use lung transplantation. However, a high post-transplantation local recurrence rate is described with some controversy regarding the possible involved mechanisms, the main possibilities being the lymphatic spread and aerosolization. Presented herein is a case of a bilateral lung transplantation for a bilateral and pneumonic form of non-mucinous bronchioloalveolar carcinoma in a 43-year-old woman. The histological analysis of mediastinal lymph nodes during surgery did not show neoplastic cells. Thirty-five months after transplantation several nodular opacities in donor lungs were detected. Three pulmonary wedge resections were performed showing a non-mucinous bronchioloalveolar carcinoma with the same histological characteristics as the primary. Again, the mediastinal lymph nodes were tumor free. A complete microsatellites molecular analysis was performed to compare the primary and recurrent carcinoma using capillary electrophoresis, showing that the recurrent tumor was generated in a recipient cellular clone. The absence of lymph node metastasis and the molecular evidence of the recipient origin of the neoplasm supports the contamination of the new lungs at the time of implantation as being the reason for the high incidence of recurrence after lung transplantation in this kind of disease.

Pyogenic granuloma (PG) is a polypoid form of lobular capillary hemangioma that usually occurs in the skin and some mucosal surfaces. However, it is extremely rare in the intestinal tract and its preoperative diagnosis is difficult. We report on a case of PG of the sigmoid colon in a 62-year-old woman with a 6-month history of rectal bleeding. A pedunculated polypoid lesion was detected by colonoscopy and a polypectomy was performed. A microscopical study revealed a lobular arrangement proliferation of varying sizes of capillaries within an edematous stroma. A critical review of the English literature yielded only another well-documented case of PG in the large intestine. Diagnostic consideration includes inflammatory polyp and other vascular tumors such as bacillary angiomatosis and the angiomatous variant of Kaposi's sarcoma. Precise recognition of this distinctive vascular neoplasm in the gastrointestinal tract is essential to avoid misdiagnosis and inappropriate treatment.

Myxomas are uncommon soft-tissue neoplasms, which are extremely rare in the kidney, with only five cases documented in the intraparenchymal location. However, renal capsular myxoma has not yet been reported. We describe a unique case of a clinically detected renal myxoma arising in the capsule. A 37-year-old man receiving treatment for epididymitis sought medical assistance for infertility. A radiological examination incidentally discovered a right renal tumor. The mass intruded into the perirenal tissue and measured 6 cm in major diameter. The resected kidney contained a well-circumscribed gelatinous capsular tumor. It was composed of sparse, bland, slender, spindle-shaped cells scattered in large amounts of basophilic interstitial mucoid material. The tumor cells showed diffuse immunoreactivity for vimentin. Occasional cells stained for alpha-smooth muscle actin and calponin. Reactivity was negative for S100 protein, epithelial membrane antigen, pancytokeratin, neurofilament protein, and h-caldesmon antibodies. Ultrastructural examination revealed fibroblast-like cells with long thin cytoplasmic processes, prominent rough endoplasmic reticulum, a well-developed Golgi complex, and secretory vesicles. No basal lamina was identified around the tumor cells. The differential diagnosis includes many other benign and malignant soft-tissue lesions exhibiting prominent secondary myxoid features. It is important to consider a renal capsular myxoma when examining lesions at this anatomic site to avoid misdiagnoses and to ensure that the patient receives appropriate treatment and prognostic information.

Primary mesotheliomas of the pericardium are rare tumors. They may occur in diffuse, multiple, and localized forms. Most of the pericardial mesotheliomas are multiple or diffuse growths encasing the heart, localized forms being distinctly uncommon. We report a localized mesothelioma of the pericardium found incidentally at the autopsy of a 76-year-old woman. The neoplasm measured 3.6 x 2.4 x 2.5 cm., was well circumscribed, and affected the entire thickness of the myocardium extending from the epicardium to the endocardium of the anterior wall of the right ventricle. The tumor was epithelial in type, showed an immunohistochemical profile compatible with mesothelioma, and was DNA aneuploid. A review of the literature yielded four cases of localized pericardial mesothelioma, including the present. Most cases are seen in women and are of the epithelial variant. There is a wide age range at presentation. Localized mesotheliomas are capable of aggressive behavior. Nevertheless, in contrast to diffuse tumors, complete surgical excision may be curative. Differential diagnosis includes solitary fibrous tumor, synovial sarcoma, epithelioid angiosarcoma, and adenomatoid tumor of the pericardium.

Kidney sarcomas are rare, representing only 1% of malignant renal tumors. We herein report the case of a 70-year-old woman that was admitted for an episode of confusion in relation to hypertensive encephalopathy. Imaging investigations revealed a large mass in the right kidney with extension to the renal hilum. The patient underwent right open radical nephrectomy. The histopathologic study disclosed a 15-cm, myxoid and cellular, pleomorphic tumor with elongated, curvilinear, thin-walled vessels, and focal necrosis that involved the upper and middle segments of the kidney. Immunohistochemically, the tumor cells showed strong positivity for vimentin, bcl2 protein (nuclear staining pattern), CD34, CD99, and alpha-methylacyl coenzyme A racemase. The tumor was diagnosed as myxofibrosarcoma (MFS) grade 2 according to the FNCLCC system. To the best of our knowledge, this is the first report of an MFS arising from the kidney. Thus, MFS is an uncommon soft tissue tumor that can exceptionally arise from the kidney. The differential diagnosis with other myxoid tumors is of vital importance because it includes lesions with subtle differences and extremely variable biological behavior. Radical surgery is the treatment of choice. Long-term follow-up is recommended because of the tumor's capability for local recurrence and distant metastasis.

Prichard’s structures of the fossa ovalis are not histogenetically related to cardiac myxoma Aims: Cardiac myxomas are neoplasms of unknown histogenesis. They are thought to arise from hypothetical subendothelial vasoformative reserve cells or from primitive cells which reside in the fossa ovalis and surrounding endocardium. In 1951 Prichard described a kind of microscopic endocardial structure with a predilection for the interatrial septum, which were suggested to be related to cardiac myxomas. To confirm the existence of Prichard’s structures and to clarify their role in the genesis of cardiac myxomas, we examined histologically the fossa ovalis and we performed an immunohistochemical study of the endocardial abnormalities that were found. Methods and results: A prospective histological study of 100 interatrial septa and an immunohistochemical study of three out of the 12 endocardial abnormalities that were detected, as well as of four conventional cardiac myxomas were accomplished. Antibodies were used to vimentin, CD31, CD34, α-smooth muscle actin, S100 protein, thrombomodulin, calretinin and c-kit (CD117), a tyrosine kinase growth factor receptor for stem cell factor usually expressed by embryonic/fetal endothelium. Structures similar to the ones described by Prichard were found in 12% of septa, most of them in the left side of the fossa ovalis. The hearts with these structures were from patients 10 years older than the ones without them (72 ± 10 versus 62 ± 16 years, P=0.006). Immunohistochemically the cells comprising Prichard’s structures were positive for vimentin, CD31, CD34 and thrombomodulin, and negative for α-smooth muscle actin, S100 protein, calretinin and c-kit. Therefore these cells seem to be mature endothelial cells, but not primitive multipotential mesenchymal cells. Furthermore, these cells were not found in the atrial tissue from the bases of any of the conventional cardiac myxomas. Conclusions: Our study suggests that there is no apparent relation between Prichard’s structures and cardiac myxomas, and that Prichard’s minute endocardial deformities are age-related phenomena.

The synchronous occurrence of three different types of renal tumor in a patient is rare. We report a case of conventional (clear cell) renal cell carcinoma harboring metastatic foci of mammary carcinoma associated with two angiomyolipomas in the left kidney incidentally discovered at the autopsy. The patient was a 75-year-old woman, without the tuberous sclerosis complex, who had undergone left radical mastectomy and radiotherapy for an infiltrating duct carcinoma of breast 30 years before. This tumor was widely disseminated at autopsy, but the nontumorous renal parenchyma was free of metastases. To the best of our knowledge this combination of neoplasms has not been described before. This case shows the important role played by autopsy in the accurate investigation of interrelations among coexisting tumors. Ann Diagn Pathol 5:293-299, 2001.

Pagetoid dyskeratosis is an incidental finding in a variety of lesions of the skin and squamous mucosa. The lesion is considered a selective keratinocytic response in which a small part of the normal population of keratinocytes is induced to proliferate in response to friction. As far as we know, pagetoid dyskeratosis has not been reported in the lips. In this article, we describe the location of the lesion in the lips and its incidence in a group of 90 unselected patients who underwent biopsy or were surgically treated for diverse labial lesions. Histochemical staining and immunohistochemical studies were performed in selected cases. Pagetoid dyskeratosis was found in 38 cases (42.2%) but only in 6 cases (6.7%) the lesion was conspicuous. There was no significant difference between the upper and the lower lip in terms of incidence of the lesion. Labial pagetoid dyskeratosis was more frequent in younger patients (46.7 +/- 25.0 versus 58.5 +/- 20.5; p0.05) and in women (chi(2) = 3.89; p0.05). Pagetoid cells were more common in suprabasal location and in the labial mucosa. These cells showed positivity for high-molecular weight cytokeratin and negative reaction for low-molecular weight cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and human papilloma virus. The immunohistochemical profile is different from the surrounding keratinocytes, indicating premature keratinization. The main differential diagnoses include white sponge nevus, leukoedema, oral koilocytoses, hairy leukoplakia, pagetoid squamous cell carcinoma in situ, and extramammary Paget's disease of the oral mucosa. The morphologic features of dyskeratotic pagetoid cells are distinctive and easily recognized as an incidental finding, thus preventing confusion with other important entities including an intraepidermal tumor.

Background: Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases. Methods and results: We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now. Conclusion: Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.