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Sebaceous carcinoma is a rare malignant skin neoplasm arising from sebaceous glands. Its clinical diagnosis is difficult and reports of dermoscopic findings have been limited. This study aims to analyze its dermoscopic features and differential diagnosis in dermoscopic examination. The study included patients diagnosed with histologically proven sebaceous carcinomas as well as diagnosed cases of sebaceous hyperplasia, sebaceoma, squamous cell carcinoma and basal cell carcinoma for comparison of dermoscopic findings. The dermoscopic criterion of presence of sebaceous carcinoma was scored only if the two evaluators reached a consensus. Fifteen cases of histologically diagnosed sebaceous carcinoma were included in our study. All cases were extraocular sebaceous carcinoma. A total of 60 (15 basal cell carcinomas, 15 squamous cell carcinomas, 15 sebaceous hyperplasias and 15 sebaceomas) cases were collected for comparing dermoscopic features with sebaceous carcinoma. In dermoscopic analysis of sebaceous carcinoma, the majority of tumors (66.67%) presented polymorphic vessel pattern. Other features included whitish-pink areas (80%), yellowish structures (73.33%) and yellowish structureless areas (60%). Yellowish structures in sebaceous carcinomas are the main dermoscopic findings to differentiate squamous cell and basal cell carcinomas (P < 0.001), whereas purplish globules, shiny white blotches and strands and whitish-pink area distinguish sebaceous carcinomas from other sebaceous tumors (P < 0.05).

Histopathologically both hidroacanthoma simplex (HS) and clonal seborrheic keratosis (CSK) are characterized by intraepidermal nests of tumor cells. Although they show subtle microscopic differences, they can be difficult to accurately differentiate. Previous immunohistochemical studies have been inconclusive. We conducted an immunohistochemical study with GATA3 and p63 on cases of HS and CSK tentatively identified by their microscopic appearances and cases of eccrine poroma and seborrheic keratosis as their respective controls. The clinical, histopathological, and dermoscopic findings of these cases were also reviewed. All cases of HS and poroma were negative for GATA3, whereas all cases of CSK and seborrheic keratosis were positive for GATA3. HS, CSK, and their controls were all positive for p63. Microscopic, clinical, and dermoscopic differences were also found between HS and CSK. Our study demonstrated that GATA3 is useful for differentiating HS from CSK. Our initial microscopic observations also proved to be reliable, but immunostaining with GATA3 is helpful for confirming the diagnosis or establishing the diagnosis of uncertain cases. Awareness of the clinical and dermoscopic features of these 2 entities could also avoid misdiagnosis based solely on pathological observation.

AIMS: In thymic carcinomas, focal clear cell change is a frequent finding. In addition to a prominent, diffuse clear cell morphology, some of these carcinomas show an exuberant hyalinised extracellular matrix, and therefore probably represent a separate entity. However, a characteristic genomic alteration remains elusive. We hypothesised that, analogous to hyalinising clear cell carcinomas of the salivary gland, hyalinising clear cell carcinomas of the thymus might also harbour EWSR1 translocations. METHODS AND RESULTS: We identified nine archived cases of thymic carcinoma with focal clear cell features and two cases that showed remarkable hyalinised stroma and prominent, diffuse clear cell morphology. These two cases expressed p40 and were negative for Pax8, CD5, and CD117. Programmed death-ligand 1 was highly positive in one case (70%), and negative in the other one. EWSR1 translocation was identified in both cases of hyalinising clear cell carcinoma, and was absent in all nine carcinomas that showed clear cell features without substantial hyalinisation. In one of the EWSR1-translocated cases, a fusion between exon 13 and exon 6 of EWSR1 and ATF1, respectively was identified by next-generation sequencing. CONCLUSIONS: These findings suggest that the EWSR1 translocation and possibly the EWSR1-ATF1 fusion might be unifying genomic alterations for thymic clear cell carcinomas with prominent hyalinised stroma, for which we propose the term 'hyalinising clear cell carcinoma of the thymus'. Because the immunophenotype is unspecific, testing for the EWSR1 translocation might be helpful in discriminating this entity from other thymic neoplasms or metastases, in particular those with clear cell change. ispartof: HISTOPATHOLOGY vol:75 issue:3 pages:431-436 ispartof: location:England status: published

The significance of granuloma and amyloid deposition in primary nasopharyngeal carcinoma (NPC) has yet to be investigated. This study aimed to evaluate their clinicopathologic associations. The histopathologic findings of 747 consecutive patients with primary NPC were retrospectively reviewed between January 2001 and December 2015. The presence of granulomas and amyloid deposits was observed in 68 (9.1%) and 62 (8.3%) patients, respectively. Granulomas were significantly associated with lower T classification, N classification, and overall TNM stage (p = 0.014, p = 0.006, and p = 0.001, respectively). Their presence was an independent predictor of overall survival (p = 0.033), disease-free survival (p = 0.034), and recurrence-free survival (p = 0.040). Conversely, amyloid deposition was not a predictor in any survival analyses. The present study demonstrated the prevalence of granuloma and amyloid deposition in the largest single institution cohort of primary NPC patients so far. Our results provide evidence that granulomas are significantly associated with better prognosis and treatment outcome. Further studies are needed to elucidate the mechanism of action of granuloma formation on the anti-tumor immunity of NPC.

Acquired perforating calcific collagenosis (APCC), which is characterized by the calcification of dermal collagen fibers with subsequent transepidermal elimination and perforation, is an extremely rare entity. Thus far, it has only been reported in a patient with direct contact exposure to calcium chloride. Here, we report a unique case of APCC occurring in a drug addict admitted for rhabdomyolysis. The present case is a 20-year-old male patient hospitalized for drug-related rhabdomyolysis and multiple organ damage. During hospitalization, he gradually developed unusual skin rashes. There were multiple confluent umbilicated and keratotic erythematous to brownish papules and plaques with scratch-like linear plaques on his lower abdomen, inguinal areas and gluteal sulci. Also, multiple well-demarcated flesh-colored rough, hard and thin plaques with a "crepe paper"-like texture were found on the bilateral popliteal fossae, olecranon fossae and axillae. The histopathology of two biopsied lesions demonstrated acquired perforating calcific collagenosis. The lesions appeared during the rhabdomyolysis-related hypercalcemia phase and resolved spontaneously after the calcium level returned to normal. This is the first reported case of disseminated APCC occurring during transient hypercalcemia due to rhabdomyolysis.

Idiopathic lymphoplasmacellular mucositis (ILPM) is a group of disorders histologically characterized by dense infiltrates of lymphocytes and plasma cells in submucosa. These lesions were initially observed on the glans penis and vulva, and later on buccal mucosa, lips, gingiva, tongue, epiglottis, larynx and other mucosal surfaces have also been reported. We have reviewed the previously reported cases of ILPM with lip involvement, outlining the clinical presentation, treatment and outcome of the entity to date. The etiology of ILPM is unknown, and the diagnosis is largely one of exclusion. To date, no consensus or consistently effective treatment for ILPM is available. Herein, we report an 18-year-old woman presented with markedly swollen lips with severe erosions, bleeding and yellow to black crusts who was diagnosed to have ILPM by histopathological study. The case occurred on rare lip site with an unusual appearance. Various treatment modalities were tried, but the responses were unsatisfactory and the lesion only responded to long-term administration of oral steroids.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that usually occurs in elderly individuals and manifests with skin lesions followed by involvement of the lymph nodes, bone marrow, and peripheral blood. We report our experience of establishing the diagnosis of a case of BPDCN in an 86-year-old man. The related literature is reviewed, and the differential diagnosis and treatment modality of this rare entity are discussed.

Post-transplantation lymphoproliferative disorder (PTLD) is one of the most common de novo malignancies in patients who receive immunosuppressive therapy after solid organ transplantation. We report a case of a 5-year-old girl who presented with indurated violaceous skin nodules 3.5 years post-liver transplantation, diagnosed as polymorphic PTLD, also involving Waldeyer's ring, spleen, and multiple lymph nodes. Through reduction of immunosuppression, most of the lesions resolved and the liver allograft was preserved.

Oncocytic lipoadenoma is a rare tumor, with only 18 cases having been reported since the first in 1998. We encountered a case of oncocytic lipoadenoma presenting as a slowly growing parotid mass in a 71-year-old man. This tumor is characteristically comprised of a mixture of oncocytes and adipocytes. The present case is one of five reported cases of oncocytic lipoadenoma showing sebaceous differentiation. The results of immunohistochemical study with DOG1 antibody supported the origination of this tumor in the striated duct.

Nevus sebaceus is known to have the potential to develop into various secondary tumors. We observed a sebaceoma arising from a nevus sebaceus excised from the left cheek of a 51-year-old woman. This sebaceoma showed desmoplastic change similar to that observed in desmoplastic trichoepithelioma and desmoplastic trichilemmoma. This heretofore undescribed desmoplastic variant of sebaceoma should not be mistaken for invasive sebaceous carcinoma.

An erythematous and moist tumor nodule on the left temple of a 92-year-old woman was biopsied and identified as a clear cell acanthoma (CCA)-like tumor with malignant cytology and high proliferation activity. This case is similar to 2 cases reported previously as atypical CCA. The authors believe that these tumors are malignant counterparts of CCA and propose to call them malignant CCA. The clinicopathologic features of the present case are described along with dermoscopic findings.

Unilateral eyelid involvement, as the only manifestation of discoid lupus erythematosus (DLE), is very rare. Here, we report the case of a 41-year-old woman who presented with erythema and swelling of her left upper eyelid, which was initially misdiagnosed as cutaneous lymphoid hyperplasia. She had initially received radiotherapy at another institute for treatment. Our examinations established the diagnosis of DLE, and direct immunofluorescence confirmed this diagnosis. The skin lesion improved after treatment with topical steroids and oral hydroxycholoroquine.

Lipedematous alopecia is an uncommon disease that mainly affects African American and Egyptian women. This report is of an 18-year-old Taiwanese woman who presented with asymptomatic boggy and thickened scalp for 10 years. In the last 6 months, there was diffuse hair loss on the affected scalp without scarring. Histopathologically, there were increased thickness of subcutaneous fat layer, mild perivascular lymphocytic infiltration, and separated collagen bundles in the dermis. Alcian blue stain demonstrated mucin deposition in the dermis and subcutis, whereas magnetic resonance imaging showed thickened scalp with expanded subcutaneous fat layer. The clinical findings and imaging study established the diagnosis of lipedematous alopecia. The pathogenesis and disease etiology remain unclear. The coexistence of mucin is extremely rare and its significance should be further investigated.

Myopericytoma is a benign tumor composed of myoid-appearing oval to spindle-shaped cells arranged in a concentric perivascular pattern of growth. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults, and presents as a well-circumscribed, slow-growing, painless firm tumor. We describe a case of myopericytoma with unusual clinical presentation as scarring alopecia in a 14-year-old girl without any underlying disease, who presented with a 2-month history of a pink patch with hair loss on her frontal scalp. Under the diagnosis of discoid lupus erythematosus, an incisional biopsy was also performed. The biopsy specimen revealed a myopericytoma characterized by a subcutaneous large vein-like structure surrounded by spindled, stellate, and cuboidal cells with multiple small branching vascular spaces. The overlying dermis was fibrotic with dilated vessels and the number of hair follicles was decreased. No features of lupus erythematosus were noted and a lupus band test was negative. Total excision was performed. No recurrence occurred in the following 2 years.

Necrobiotic xanthogranuloma is an uncommon granulomatous disease involving the skin and extracutaneous tissues. It is characterized by indurated, yellow-red plaques and nodules, involving primarily the face and less frequently the trunk and extremities. The disease has a strong association with paraproteinemia and other hematologic or lym-phoproliferative disorders. Histologically, the dermal part shows xanthogranulomatous change with extensive necrobiosis and many Touton and foreign-body giant cells. Here, we describe a case of a 46-year-old man with a 1-year history of multiple cutaneous lesions over the trunk and thighs. Necrobiotic xanthogranuloma was diagnosed by histology and clinically associated with paraproteinemia. This case is also unusual in that there was no periorbital involvement, which is believed to be a typical feature of this disease.

Dermatofibrosarcoma protuberans (DFSP) is an indolent sarcoma known for its propensity for local invasive growth and recurrence. It typically presents as a protuberant tumor mass. Rare nonprotuberant presentations have recently been described; these invariably present as pigmented or depressed plaques. Lesions arising in the subcutaneous compartment and without cutaneous manifestations have rarely been reported or emphasized in the literature. Here we report a case of deep DFSP that lacked discernible epidermal or dermal changes, was mistaken for a lipoma, and localized entirely within the subcutaneous compartment. Ultrasonography may not be useful in differentiating DFSP from benign tumors. In addition, a review of the English language literature revealed that these deep-seated tumors might be more common than originally believed. For this purpose, the current case is presented to raise awareness for DFSP, which can be present entirely in the subcutis without cutaneous manifestations and thus easily overlooked.

Sclerosing angiomatoid nodular transformation (SANT) is a peculiar splenic vascular lesion that is characterized by marked stromal sclerosis and the presence of plasma cells, which shares histopathological features associated with IgG4-related sclerosing disease. The cinicopathological features of 10 cases of SANT were reviewed and immunohistochemistry with IgG4 and IgG antibodies was performed. Nine other various splenic lesions served as controls. Five cases of SANT were found incidentally. Three cases of SANT had multiple tumors and five had associated abdominal disseminated calcifying fibrous tumors (CFT). IgG4+ plasma cells were found in all of the cases of SANT and in calcifying fibrous tumors. The densities of IgG4+ and IgG+ cells and the IgG4/IgG ratios were significantly higher in SANT than in control spleens (P= 0.001, 0.006, and 0.028, respectively). Serum IgG4 concentration was elevated in one case. In conclusion, SANT can occur either as a solitary or as multiple tumors. Frequent association with abdominal disseminated CFT simulating carcinomatosis was observed. A statistically significant number of IgG4+ plasma cells was found in all of the cases of SANT and the associated CFT. Whether they are related to the IgG4-related sclerosing disease or not is debatable and warrants further investigation.

Background: Cutaneous Rosai-Dorfman disease (CRDD) shares the histopathological features of abundant plasma cells and stromal fibrosis with IgG4-related sclerosing disease. The possible role of IgG4+ plasma cells in CRDD was investigated. Methods: Twelve cases of CRDD were reviewed, and their lesions were immunostained with anti-IgG4 and anti-IgG antibodies. The number of IgG4+ and IgG+ plasma cells and their ratios were estimated. Serum IgG4 and IgG concentrations were measured in two recent cases. Results: Many IgG4+ and IgG+ plasma cells were found in all 12 cases. IgG4+ plasma cells ranged from 21 to 204 per high-power field (HPF) (mean 117/HPF), and IgG+ plasma cells ranged from 114 to 759/HPF (mean 349/HPF). All cases had more than 30 IgG4+ cells/HPF, except one case. The IgG4/IgG ratio ranged from 16% to 51% (mean 34%). Serum IgG4 concentration and serum IgG4/IgG ratio were increased in one recent case. Various degrees of stromal fibrosis were present in all cases. Conclusions: The presence of many IgG4+ plasma cells and stromal fibrosis suggests that CRDD may be related to IgG4-related sclerosing disease. Many IgG4+ plasma cells is another feature of CRDD, and serum IgG4 may be elevated.

Normolipemic plane xanthoma (NPX) is a histiocytic disorder characterized by yellow-orange plaques in the periorbital areas, neck, upper trunk, and flexural folds. Association with systemic disease or paraproteinemia has been reported previously, but rarely with Langerhans cell histiocytosis (LCH). We report a case of Hand-Schüller-Christian disease (a type of LCH) in a patient who developed NPX with supraglottic involvement. NPX developed after several courses of chemotherapy and the supraglottic xanthoma occurred about 2 years later. The coexistence of LCH and non-LCH histiocytic lesions in this patient could be a result of chemotherapy-induced changes or may be just coincidental.

Primary cutaneous marginal zone B-cell lymphoma is considered the cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Although its molecular pathogenesis is currently unknown, an etiological link with Borrelia burgdorferi infection has been identified in European, but not in American or Asian cases. To better understand the pathogenesis and the geographical differences of cutaneous marginal zone B-cell lymphoma, 60 cases from the East Asia, Germany, and the United States at their initial presentation were subjected to the following analyses; (1) clinicopathological comparison between the geographical regions, (2) detection of B. burgdorferi DNA, (3) detection of the API2-MALT1 fusion transcript, a gene alteration specific to mucosa-associated lymphoid tissue lymphoma, and (4) inactivation of tumor suppressor genes (death-associated protein kinase (DAPK), p16(INK4a), p14(ARF), MGMT, TIMP3, CDH1, and RARB) by hypermethylation of the CpG islands. Cases from the three geographical regions showed similar clinicopathological features. However, moderate/marked tissue eosinophilia was found in 9/25 Asian cases, but only 1/23 German cases (P=0.011) and 0/12 American cases (P=0.015). All 60 cases were negative for either Borrelia DNA or API2-MALT1 fusion. Tumors from the three regions were highly methylated for DAPK (38-50% of the cases, mean 43%) and p16(INK4a) (42-70%, mean 49%), and the positivities were significantly higher than those of nonneoplastic skin (8%, P=0.0010 and 14%, P=0.0032, respectively). Methylation of these genes had no significant association with progressive features of the tumor. Primary cutaneous marginal zone B-cell lymphomas from the three geographical regions have common clinicopathological features, however, moderate/marked tissue eosinophilia is a feature found almost exclusively in Asian cases. Borrelia infection and API2-MALT1 fusion are not significant in this tumor. Methylation of DAPK and p16(INK4a) genes is a frequent event in this lymphoma at its initial presentation, but may not be associated with tumor progression.

Only 2 cases of nasopharyngeal small cell carcinoma have been reported. Another case of this rare type of nasopharyngeal tumor confirmed by immunopositivity for CD56, synaptophysin, and chromogranin A is described in this study. The tumor was also negative for cytokeratin (CK) 20 and thyroid transcription factor-1. Clinical investigation did not find any primary tumor in other anatomic sites. Therefore, this case was established as a primary nasopharyngeal small cell carcinoma. Epstein-Barr virus was shown to be absent by Epstein-Barr virus encoded RNA in situ hybridization study in this case, which further distinguished it from conventional nasopharyngeal carcinoma. Clinical features of this case and 2 previously reported cases are compared. They do not differ from conventional nasopharyngeal carcinoma in clinical presentation, but the prognosis seems to be worse. More cases are needed to confirm the observation and for understanding the clinical behavior of this rare type of nasopharyngeal tumor.

Background Although an umbilical nodule is common in neonates and young infants, an umbilical nodule of poor therapeutic response will increase the likelihood of other uncommon etiology. Clear cell acanthoma (CCA) has never been described as an oozing umbilical nodule on infants. Methods To present a case of CCA which occurred on an 8-month-old female infant presenting with one weeping nodule on the umbilicus since early neonate. The lesion underwent skin biopsy and subsequent hematoxylin-eosin and periodic acid-Schiff staining. Result Three courses of liquid nitrogen cryosurgery were performed after the diagnosis of CCA was confirmed. The lesion resolved rapidly. Conclusion This report presents the first and youngest case of umbilical CCA in the English literature. This case supports the inflammatory dermatosic nature of CCA.

An inevitable outcome of modern Medicine in any country is that some patients will experience adverse events, some of which would have been preventable. Different nations have developed various approaches to such cases; their legal efficacies are probably dissimilar and dependent on a number of disparate variables. An international "snapshot" of the results of the interacting forces can be obtained by asking physicians in several countries how they view selected subjective facets of their tort systems. In the U.S., many physicians view the structure of malpractice torts as unfair, and that belief is shared by at least some pathologists. The American Medical Association has declared that a multiregional malpractice "crisis" exists which raises medical costs and threatens access to care. Furthermore, malpractice tort decisions are often flawed scientifically because lay jurors and judges cannot properly evaluate the quality of "expert" testimony given by adversarial witnesses. Despite these factors, there has been little effort to investigate the views of pathologists on malpractice actions outside the U.S. In this paper, the authors have collected the responses of an international group of pathologists to a questionnaire on that topic. The respondents practice in academic centers in 15 countries outside the U.S. As expected, a range of views was represented, with some pathologists reporting that malpractice litigation was uncommon and others noting a worrisome trend toward its growth. Interestingly, so-called "defensive medicine" was found to be relatively common in pathology in many countries.

Cutaneous verrucous carcinoma is a rare variant of low-grade squamous cell carcinoma. It usually involves distal extremities and is often misdiagnosed as giant warts. Multiple cutaneous verrucous carcinomas are rare in the English-language literature. We describe a 41-year-old man with multiple verrucous plaques on both feet and ankles, as well as the left thigh. Immunohistochemical study with proliferating cell nuclear antigen revealed positive staining of the basal and suprabasal layers. The patient's condition was successfully treated with systemic acitretin.

Hassall's corpuscles are regular structures in the medulla of the normal thymus and in non-neoplastic thymic conditions, e.g. in multilocular thymic cysts. In thymomas, however, they are inconsistently found, and are believed to indicate medullary differentiation of WHO type B1-3 thymomas. We present five organotypical thymomas resembling WHO type B2 and B3 thymomas, but with an abundance of Hassall's corpuscles. We wonder whether this exceedingly rare observation might herald a distinct entity. Four tumors were asymptomatic, incidental findings and of low Masaoka stage (I or II) [20] . One patient suffered from myasthenia gravis which disappeared upon surgical removal of the thymus, while all other patients had no concomitant autoimmune disease. Two patients had a relapse-free follow-up of 12 and 2 years, respectively, upon curative surgery, and another tumor was an autopsy finding; follow-up data of two more recent cases was not yet available. The neoplastic epithelium other than Hassall's corpuscles was arranged either in a cortical type B2 pattern or in type B3 solid cords. In all examples, there was cyst formation, inflammatory reaction and repair, indicative of a long-standing condition. Immature T cells were present in all instances. “Corpuscular thymomas” morphologically resemble WHO type B2 and B3 thymomas, but appear biologically indolent and are rarely associated with myasthenia gravis. Whether they qualify for a separate entity has to be proven by larger series, including genetic studies.

Polyvinylpyrrolidone (PVP), a polymer of the monomer N-vinylpyrrolidone with various molecular weights, was originally developed as a plasma expander. Currently, it is widely used in hair sprays, skin care products, fruit juices, and as a retarding agent in drugs such as procaine and hormones. PVP polymers with a molecular weight greater than 20,000 cannot be excreted by the kidneys and therefore are phagocytosed and permanently stored in the reticular endothelial system, leading to the so-called PVP storage disease. We report a case of localized cutaneous PVP storage disease presenting with persistent upper lip swelling and mimicking cheilitis granulomatosa, which has never been reported before.

Summary Background Porokeratosis (PK) is an uncommon disorder of epidermal keratinization with a unique clinical appearance, unknown aetiology, and an unpredictable outcome. Genital PK (defined as localized PK confined to the genital area in this study) is extremely rare and is not well documented. Objectives To evaluate the clinical manifestations, histopathology, clinical course and treatment response for genital PK. Methods We reviewed the clinicohistological data from 10 patients with genital PK seen at Chang Gung Memorial Hospital from 1990 to 2005. Results Seven patients had lesions only in the genital area, and three patients had genital and adjacent areas involved. All patients were male and the mean age at initial diagnosis was 46·0 years (range 36–59). All but one patient presented with pruritic lesions. Three patients (30%) had diabetes mellitus. No malignant transformation was observed. Surgical excision was the most effective treatment if it could be performed. Conclusions Genital PK appeared more frequently in the Asian population than in reports from western countries. Genital PK presented mostly as pruritic lesions in Taiwan, with a wide age distribution. Long-term follow-up might be needed.

The differential diagnosis between hemangioblastoma of the central nervous system and metastatic clear cell renal cell carcinoma can be problematic, because they may share striking morphologic similarities. Since CD10 is expressed in clear cell renal cell carcinoma, while inhibin alpha is expressed in hemangioblastoma, we used CD10 and inhibin alpha (inhibin A) to study their possible use in the distinction of these two entities. A total of 22 cases of cerebellar hemangioblastoma, five cases of metastatic clear cell renal cell carcinoma to the central nervous system, and 16 primary cases of clear cell renal cell carcinoma were studied with immunohistochemical staining of both CD10 and inhibin A. All 22 cases of hemangioblastoma were immunonegative for CD10 in the stromal cells. In contrast, all five cases of metastatic clear cell renal cell carcinoma and 16 cases of primary clear cell renal cell carcinoma showed positive CD10 membranous staining. In all, 20 cases of hemangioblastoma (20/22, 91%) expressed inhibin A in the stromal cells. Two cases of primary clear cell renal cell carcinoma (2/16, 13%) and three cases of metastatic clear cell renal cell carcinoma (3/5, 60%) showed immunopositivity for inhibin A. In conclusion, in addition to the immunostaining of inhibin A, CD10 is a superior marker for distinguishing between a hemangioblastoma and a metastatic clear cell renal cell carcinoma.

Xanthogranuloma (XG) is a benign cutaneous histiocytic tumor occurring mainly in young children. Onset in adulthood is rarely observed. We encountered an unusual case of an XG-like cutaneous tumor on the scalp of a 50-year-old man. The tumor recurred with multiple satellite nodules soon after surgical excision. This unusual clinical behavior has not previously been described for XG and caused a diagnostic challenge; it was unclear whether the tumor was an atypical XG or a malignant dermal tumor mimicking an XG. Our analyses favored an XG-like dermal histiocytic tumor. A longer follow-up and reports of similar cases will reveal its true nature.

Purpose To report a patient who was diagnosed with combined adult inclusion conjunctivitis (AIC) and mucosa-associated lymphoid tissue (MALT) lymphoma. Methods This is a case report. Results An 18-year-old male patient presented with chronic conjunctivitis and giant follicles. Evaluation by chlamydial antigen assay was positive. Conjunctival biopsy for the immunohistochemical stain and polymerase chain reaction of the left eye showed MALT lymphoma. Conclusions MALT lymphoma can masquerade as other ocular surface diseases. Chlamydial infection causes chronic inflammation of the conjunctiva. Both of these diseases should be considered as a differential diagnosis of refractory follicular conjunctivitis. It is worthy of further study to determine whether chronic inflammation resulting from chlamydial infection increases the risk of MALT lymphoma or it is coincidental.

Twenty-three patients with primary salivary gland lymphoma were diagnosed between 1990 and 2001. The sites of involvement were the parotid gland in 13, the submandibular gland in 9 and the minor salivary gland in 1. The sites of lymphoma involvement beyond the salivary glands were the cervical lymph nodes in 7, bone marrow in 3, the axillary lymph nodes in 3, the nasopharynx in 2, the abdominal lymph nodes in 2, the palate, the subconjunctiva, and the spleen in 1 each patient. Histologically, 19 patients had lymphomas of mucosa-associated lymphoid tissue (MALT) with myoepithelial sialadenitis in 13, 3 patients had diffuse large cell lymphomas and 1 had follicular lymphoma. Six patients were in stage I, 4 in II, 1 in III and 12 in IV. Eight of 23 patients (35%) had autoimmune diseases before or after the diagnosis of NHL and all suffered from MALT lymphoma. Four patients with parotid MALT lymphoma had primary or secondary Sjögren’s syndrome. One each patient suffered from hyperthyroidism, systemic lupus erythematosus, membranoproliferative glomerulonephritis and cryoglobulinemia, respectively. All the 6 stage I patients had achieved complete remission (CR) without relapses 17–84 months (median 44 months) after treatment. Excluding a stage IV patient with follicular lymphoma who died at 3.5 months without treatment, CR was achieved in all of the remaining 16 patients. However, a high relapse rate (9/16, 56%) was noted in stage II–IV patients. These patients tended to relapse in the original sites, but achieved CR again after chemotherapy or radiotherapy. One patient with MALT lymphoma developed histologic transformation into diffuse large lymphoma during relapse and died of refractory disease. Overall, only 2 patients succumbed. The overall survival and relapse-free survival rates at 5 years were 94.7 and 51.4%, respectively. Thus, salivary gland lymphoma proved to be an indolent disease.

BACKGROUND Nasopharyngeal carcinoma (NPC) is the most common head and neck malignancy in southeastern China and Taiwan. Early detection of the local disease followed by timely and appropriate treatment is essential to increasing cure and survival rates. Detection of Epstein–Barr virus (EBV) genomic DNA, such as the latent membrane protein 1 gene (LMP-1), in patients postirradiation during follow-up may indicate mucosal recurrence. METHODS Seventy-one patients with NPC underwent serial nasopharyngeal swabs for LMP-1 polymerase chain reaction assay before, during, and after irradiation. All of patients achieved a complete disease remission of the LMP-1 gene after irradiation that lasted for at least 6 months. RESULTS The median LMP-1 disease remission time after the beginning of irradiation was 4.3 weeks. Patients with early LMP-1 disease remission (≤ 4 weeks after the beginning of irradiation) and delayed LMP-1 disease remission (> 4 weeks) had 3-year local control rates of 93.5% and 76.9%, respectively (P = 0.0529). The LMP-1 gene was detected again (reexpression of LMP-1 [re-LMP-1]) in 10 patients after irradiation with at least 6 months of follow-up. Nine of 10 patients (90%) in the re-LMP-1 positive group and 2 of 61 patients (3.3%) in the re-LMP-1 negative group developed local recurrence. Mucosal recurrence developed in nine patients, and all displayed re-LMP-1. By detecting re-LMP-1 using nasopharyngeal swabs, mucosal recurrence was diagnosed with a sensitivity of 100% (9 of 9 patients) and a specificity of 98.4% (61 of 62 patients). The 3-year overall survival rate, the disease free survival rate for the entire group, and the estimated local mucosal control rates in the re-LMP-1 positive and re-LMP-1 negative groups were 86.5%, 76.5%, 19.4%, and 96.7%, respectively. CONCLUSIONS Expression of EBV LMP-1 in nasopharyngeal swab specimens from patients with irradiated/treated NPC can provide a highly sensitive and specific method of forecasting mucosal recurrence. This investigation confirmed the reliability and feasibility of nasopharyngeal swabs in screening for mucosal recurrences in patients with NPC. Cancer 2003. © 2003 American Cancer Society.

Anticonvulsant hypersensitivity syndrome caused by sodium valproate (Depakene) has rarely been reported. Here we describe a patient who presented with nonspecific polymorphous eruptions, fulminant hepatitis, and jaundice. Lichenoid dermatitis was found in the skin biopsy specimen. Toxic hepatitis and microvesicular steatosis were found in the liver biopsy specimen, consistent with valproate hepatotoxicity.

Objective The object of the study was to determine the incidence of the presence of Epstein-Barr virus–derived latent membrane protein-1 (LMP-1) gene in various head and neck cancers by polymerase chain reaction method. Study Design Prospective study. Methods During a 5-year period, polymerase chain reaction was used to investigate the presence of LMP-1 gene in various head and neck cancers from different locations and histopathological types, noncancerous nasopharyngeal biopsy samples, and tonsillectomy specimens from patients with chronic hypertrophic tonsillitis. Results Of 202 patients enrolled in the study, 53 were diagnosed by pathological study with oropharyngeal carcinoma, 45 with nasopharyngeal carcinoma, 26 with oral cavity carcinoma, 26 with laryngohypopharyngeal carcinoma, 31 with nasopharyngeal lymphoid hyperplasia, and 21 with tonsils with lymphoid hyperplasia. After the application of polymerase chain reaction, the LMP-1 gene was not detected in any sample from oral cavity carcinoma, laryngohypopharyngeal carcinoma, or nasopharyngeal lymphoid hyperplasia or from tonsillectomy specimens but was detected in only one case of tonsillar carcinoma. On the contrary, the LMP-1 gene was detected in 43 (95.6%) of 45 samples from patients with nasopharyngeal carcinoma. The statistical analysis shows a significant association (P

Background: Syringoma of the vulva has been rarely reported. No effective treatment modality has been documented. Objective: The purpose of this study was to describe the clinicopathologic features of vulvar syringoma, to investigate the hormonal influence on its growth, and to establish an effective treatment modality. Methods: A total of 18 cases of vulvar syringoma were selected from the surgical pathologic file at Chang Gung Memorial Hospital. In all, 15 formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for estrogen receptor and progesterone receptor. Results: The age of first presentation at our vulvar clinic ranged from 21 to 60 years with a median age of 29.5 years. Of patients, 13 (72%) had vulvar pruritus and 7 noticed aggravation during summer or during menstruation. The most common clinical appearance was multiple flesh-colored or brownish papules on bilateral sides of the vulva (9 of 18). One third of our series was found to have coexisting eyelid syringoma and 4 of them also had a family history of periorbital syringoma. Immunohistochemical stainings for estrogen receptor and progesterone receptor were all negative on the 15 cases studied. Of our patients, 7 with intense pruritus were treated with carbon dioxide laser vaporization. Their lesions resolved and pruritus subsided. Conclusion: Vulvar syringoma is not very rare and should be considered in the differential diagnosis of vulvar pruritus. In our study, estrogen receptor and progesterone receptor were not detected in vulvar syringoma. Carbon dioxide laser was an effective therapeutic modality in treating patients with intractable symptoms. (J Am Acad Dermatol 2003;48:735-9.)

Subcorneal pustular dermatosis (SPD) and pyoderma gangrenosum (PG) are two neutrophilic dermatoses. Coexistence of these diseases in the same patient is rare and may be a strong indicator of IgA dysglobulinemia. We describe a 69-year-old man who presented with waxing and waning flaccid pustules covering his trunk and four limbs. Poorly healing ulcerations, which usually progressed into larger nodules after debridement, were also noted. Repeated cultures were negative for bacteria, and the patient was diagnosed with SPD and PG. Serum protein electrophoresis and immunofixation revealed a monoclonal IgA lambda protein. A subsequent bone marrow biopsy revealed a normocellular marrow. While PG and SPD can occur individually in a variety of associated diseases, such as rheumatoid arthritis, systemic lupus erythematosus and inflammatory bowel disease or infection; however, their coexistence is strongly indicative of IgA dysglobulinemia.

Background Cutaneous plasmacytosis is clinically characterized by multiple pigmented papules and plaques that occur mainly on the trunk and many plasma cells in the lesional skin. Most of the cases reported have occurred in Japan. Whether cutaneous plasmacytosis is a unique reactive disease or a neoplastic condition has not been established. Methods We collected 12 cases fulfilling the criteria for a diagnosis of cutaneous plasmacytosis at our institution from 2001 to 2013. We analyzed clinicopathologic features and performed immunohistochemical and immunoglobulin gene rearrangement studies. Results All 12 patients presented with characteristic cutaneous lesions mainly on the trunk and proximal extremities. Many plasma cells were observed in the lesional skin, and the basal layer was hyperpigmented. Four patients had numerous plasma cells in lymph nodes, and one patient also had increased plasma cells in bone marrow. One patient fulfilled the criteria for immunoglobulin G4 (IgG4)-related lymphadenopathy. Elevated serum IgG and IgG4 concentrations were found in seven patients. Monoclonal immunoglobulin gene rearrangements were detected in four patients. The median duration of follow-up was four years. One patient was lost from follow-up. Partial remission of the cutaneous lesions was observed in five patients, and progressive disease was noted in five patients. One patient died of respiratory failure. Conclusions Our study suggests that the so-called “cutaneous plasmacytosis” has heterogeneous underlying causes with or without systemic involvement and may be associated with clonal immunoglobulin gene rearrangements and IgG4-related lymphadenopathy. No effective treatment is available for this condition.

The classification of thymic epithelial neoplasms has been one of the most controversial issues in tumor pathology. There are two opposing schools of pathologists holding different views regarding the classification of thymic epithelial neoplasms. One school of pathologists believe that histological classification of thymomas is not possible or useful. Another school of pathologists believe that thymomas can be histologically subclassified despite their complex histomorphology and that these histological subtypes correlate with their aggressiveness and clinical behavior. A compromised histological classification has been established by World Health Organization (WHO) to designate thymic epithelial neoplasms with letters and numbers. This classification should be adopted internationally to facilitate the communication among concerned pathologists and oncologists. A simple histological classification of thymomas based on cytomorphology and supported by cytokeratin expressions is proposed and compared to the WHO and Müller‐Hermelink's histogenetic classifications.

Toxoplasmosis is a common cause of lymphadenopathy, but toxoplasmic cysts are not usually found in histological sections used for establishing diagnosis, except on extremely rare occasions. The histopathological triad of florid reactive follicular hyperplasia, clusters of epithelioid histiocytes, and focal sinusoidal distention by monocytoid B cells has been considered to be diagnostic of toxoplasmic lymphadenitis, but the validity of the histopathological triad is based indirectly on serological correlation only. The demonstration of Toxoplasma gondii DNA in lymph nodes displaying the histopathological triad will indicate the validity of the histopathological triad as the criterion for the histopathological diagnosis of toxoplasmic lymphadenitis. We used frozen tissues of 12 lymph nodes with the histopathological triad and tissues of 27 lymph nodes from patients with various other conditions (including 13 cases of follicular lymphoid hyperplasia, FLH; three cases of dermatopathic lymphadenopathy, DPL; two cases of plasmacytosis; two cases of Castleman's disease; two cases of metastatic adenocarcinoma; and five cases of lymphoma) to detect T. gondii DNA by polymerase chain reaction. Ten out of 12 lymph nodes with the triad and six out of 27 lymph nodes without the triad were positive for T. gondii DNA. Thus, the sensitivity of the triad was 62.5% (10/16) and the specificity was 91.3% (21/23). The predictive value of positive tests was 83.3% (10/12) and the predictive value of negative tests was 77.7% (21/27). The six cases positive for T. gondii DNA without the triad were four cases of FLH, one case of DPL, and one case of plasmacytosis. None of the neoplastic diseases was positive. The false positive and negative cases could be due to sampling problems or past T. gondii infection. The results confirm that the histopathological triad is highly specific for the diagnosis of toxoplasmic lymphadenitis and can be used confidently.

Salivary gland type nasopharyngeal carcinomas (NPCs) are rare and not well characterized. Fifteen NPCs with adenocarcinomatous differentiation were studied for their histologic types, immunohistochemical features, and association with Epstein-Barr virus (EBV) by EBER in-situ hybridization (ISH) and by polymerase chain reaction (PCR) to detect latent membrane protein-1 (LMP-1) gene with formalin-fixed, paraffin-embedded tissues. Two cases of conventional NPC were included for comparison. The prevalence rate of salivary gland type NPCs was 1.3% of the total NPCs in this series. The patients consisted of 11 men and 4 women with ages ranging from 15 to 74 years (median, 50 yrs). Mucoepidermoid carcinoma was the most common type (53%) and an unusual psammomatous variant was found. Others were adenoid cystic carcinomas and various adenocarcinomas, including a papillary adenocarcinoma. A composite tumor of adenocarcinoma and undifferentiated carcinoma was also observed. The tumors were positive for AE1 and CK19 and generally negative for AE3 and CK20. Most cases were positive for epithelial membrane antigen and CA 19-9 with sporadic expression of carcinoembryonic antigen (CEA) and S-100 protein. Conventional NPC differed only in consistent immunonegativity for CA19-9 and CEA. EBV was detected by EBER ISH in 9 of 15 cases (60%) and by PCR of the LMP-1 gene in 10 of 15 cases (67%). Six of 10 LMP-1 gene-positive cases (60%) had a specific deletion of 30-base pairs (bp) of the LMP-1 gene. The result also supports the possible importance of a 30-bp deletion in conferring the oncogenecity of the LMP-1 gene. The prognosis of this series of salivary gland type NPCs was poor. Six of 13 patients with follow-up information already died of the disease with a median survival of only 1 year.

The histologic type of thymomas associated with pure red cell aplasia (PRCA) has generally been regarded to be predominantly the spindle cell type. The 5 thymomas associated with PRCA we studied showed various histologic types and none of them was a spindle cell thymoma. Some cases reported as spindle cell thymoma associated with PRCA in the literature might not be spindle cell type. The discrepancy could be due to the lack of consensus in the histologic classification of thymomas in the past. Our study showed that thymomas associated with PRCA lacked a strong correlation with a particular histologic type and the pathogenesis of PRCA associated with thymoma did not seem to be related to the histologic type. One of our cases was an unusual composite tumor of an organoid thymoma (WHO type B1) and a hitherto undescribed “lipofibroadenoma.” The clinical outcome was influenced by the invasive character and the histologic type of the thymoma.

Background Nasopharyngeal carcinoma (NPC) is the most common head and neck malignancy in southeastern China and Taiwan. Early detection of the local disease followed immediately by proper treatment is essential to increase the cure and survival rates. Because every NPC tumor cell carries Epstein-Barr Virus (EBV) genomes, detection of EBV genomic DNA such as latent membrane protein 1 gene (LMP1) might indicate the presence of NPC. We developed a simple and noninvasive technique of nasopharyngeal swabbing to acquire nasopharyngeal cells for detecting the presence of EBV genome. The aim of this study was to investigate the feasibility and reliability of this technique. Methods We collected nasopharyngeal cells by means of a nasopharyngeal swabbing technique and detected the presence of EBV LMP1 with polymerase chain reaction (PCR). Thirty-eight swab specimens were obtained from patients with NPC who were newly diagnosed or were just beginning radiotherapy. Two groups of control subjects were recruited, including 20 patients with other head and neck cancers and eight family members of the NPC patients. An additional group of 65 NPC patients were enrolled in the course of regular follow-up after definitive radiotherapy. Results All of the samples yielded sufficient DNA for PCR amplification. Thirty-six of 38 NPC swab samples were positive for EBV LMP1, and all the control subjects had swab sample results negative for EBV. All five patients with suspected local recurrence exhibited positive EBV test results. Conclusions Demonstration of EBV LMP1 in the nasopharyngeal swab specimens detected NPC with a sensitivity of 94.7% and specificity of 100%. This study confirms the reliability and feasibility of nasopharyngeal swab in the predicting and screening of NPC. © 2001 John Wiley & Sons, Inc. Head Neck 23: 194–200, 2001.

Benign fibrous histiocytoma of the skin or dermatofibroma (DF) has been regarded as a fibrohistiocytic tumor. Whether DF is a neoplastic growth or a reactive process has not been settled. Since a neoplastic process is clonal in nature, clonal analysis of DF was conducted to see if DF is a clonal disease. Fresh specimens of 13 DFs and 2 hypertrophic scars obtained from female patients were studied. The adjacent nonlesional skin tissues served as controls. The clonal analysis was based on the methylation pattern of the polymorphic X-chromosome linked androgen-receptor gene (HUMARA). Eight DFs and 1 hypertrophic scar were heterozygous at the androgen receptor gene and could be analyzed. All 8 informative DFs showed a significant reduction in one of the allelic bands compared with the corresponding bands of the nonlesional tissue after Hha I digestion. Therefore, DF is a clonal proliferative disease. In contrast, 1 hypertrophic scar showed a polyclonal pattern of X-chromosome inactivation. We conclude that DF is a clonal disease favoring a neoplastic process.

Metastasis to the skin from cervical carcinoma is relatively uncommon. Herein we present a 41-year-old woman with a history of cervical carcinoma with severe facial erythematous swelling and telangiectasia. She was initially treated for cellulitis without improvement. A skin biopsy specimen revealed widespread intravascular tumor emboli in the dermis and subcutis, resembling the so-called inflammatory carcinoma of the breast. As this is an unusual clinical presentation for the metastasis of cervical carcinoma, this case is reported.

"PVP storage disease" is a disorder occurring in patients who have received high molecular weight polyvinylpyrrolidone (PVP), which cannot be excreted from the body. These large polymers deposit in the histiocytes and cause proliferation and infiltration of histiocytes in the reticuloendothelial system. There was usually no significant damage to these organs except that prolonged administration might cause bone destruction, skin lesions, arthritis, and polyneuropathy. We describe a patient who had received a large amount of PVP-containing solution for years. Severe bone marrow failure with extensive infiltration of bone marrow by foamy histiocytes occurred later. In addition, she suffered from multiple pathological fractures with spinal cord compression and arthritis of bilateral knee joints.