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Key Clinical Message: Odontogenic carcinosarcoma, a rare and challenging diagnosis, was identified in a 60‐year‐old male through histopathology, revealing a biphasic neoplasm with malignant epithelial and mesenchymal components. Surgical resection is crucial for management, highlighting the importance of vigilant postoperative follow‐up to ensure early detection of any recurrence. One rare mixed malignant odontogenic tumor is odontogenic carcinosarcoma, which comprises malignant epithelial and mesenchymal components. Diagnosing odontogenic carcinosarcoma is challenging due to its rarity and atypical clinical presentation. This study reports a 60‐year‐old male patient who presented with a painless swelling on the right side of his face and experienced facial asymmetry for 6 months, ultimately diagnosed with odontogenic carcinosarcoma. A biphasic neoplasm with malignant alterations in both epithelial and mesenchymal components was identified upon histopathological examination. MRI imaging showed an expansile multilobulated lytic lesion with cortical erosion and extraosseous extension in the posterior region of the right mandibular body and ramus. Following contrast administration, homogeneous lesion enhancement was observed, with a few small non‐enhancing necrotic areas in central parts. The patient subsequently underwent a right hemi‐mandibulectomy with resection of adjacent soft tissues and neck dissection due to lymph node involvement. The resulting defect was reconstructed using a pectoralis major flap. No recurrence or metastasis was reported during the 6‐month follow‐up, reinforcing the positive results. This case highlights the importance of recognizing odontogenic carcinosarcoma and underscores the challenges in diagnosing and managing this rare tumor. Early identification and aggressive treatment can lead to positive outcomes, as evidenced by the absence of recurrence or metastasis in this patient during the follow‐up period. [ABSTRACT FROM AUTHOR]

Chondroid syringoma (CS) is a rare benign skin adnexal neoplasm. Its incidence is very low among all primary skin tumors. Diagnosing it clinically can be difficult as it closely resembles other benign salivary or adnexal neoplasms. Until now, only a few cases have been reported from the periorbital region. Clinically, it can be mistaken for epidermal cysts, but ectopic salivary glands are a close differential diagnosis. To diagnose it, a complete removal is usually required, followed by a histopathological examination. We present a unique case of a 45-year-old female patient with a CS in her right supraorbital ridge and review the pertinent literature. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Subcutaneous tumors of the head and neck resembling cutaneous mixed tumors may be misdiagnosed pleomorphic adenomas of salivary gland origin. Physicians should consider salivary mixed tumors in the differential diagnosis for suspected cutaneous tumors. [ABSTRACT FROM AUTHOR]

(CHC) requires attention clinically and pathologically after liver transplantation (LT) because of its unique biology, difficulties in diagnosis, and being rare. We aimed to present our single-center experience for this incidental combined tumor. It is aimed to present our single-center experience for this incidental combined tumor. Materials and Methods: Seventeen patients with CHC were included in the study. There were 260 hepatocellular carcinoma (HCC) patients determined as the control group. Patients were evaluated for demographic, etiological, pathological features, and survival. Results: Macrovascular and microvascular invasion levels were significantly higher in the CHC group (p<0.05). P53, CK19, and CK7 levels were significantly higher in the CHC group (p<0.05). Hepatocyte-specific antigen level was significantly higher in the HCC group. The mean overall survival was significantly higher in the HCC group (p<0.05). Conclusion: Even though CHC is a rare liver tumor, it has features that need to be clarified regarding both survival and tumor biology. İnvestigating prognostic factors, especially in terms of survival and recurrence, will be very beneficial to identify candidates who will benefit from LT and be included in the indications for LT for CHC. This study evaluated the outcomes of patients showing combined HCC-intrahepatic cholangiocarcinoma in explant pathology. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Benign mixed salivary gland tumor is comprised of epithelial and myoepithelial cells and represents up to 80% of tumors of the parotid gland. It is relatively rare in the soft palate and in other minor salivary glands. Surgery is the standard care. Salivary gland tumors are relatively rare and morphologically diverse group of lesions. Pleomorphic adenoma (PA) is the most common salivary gland tumor, accounting for approximately 80% of all major and minor salivary gland tumors. PA usually affects the parotid gland. Huge PA occurring in soft palate is extremely rare. Patients with these tumors are usually between at the age of 40 and 60 years. The tumors exhibit pleomorphic nature microscopically that may pose diagnostic challenges to pathologists as may confuse PA histopathologically with other salivary gland tumors. Surgery is the standard treatment. The purpose of writing this case study is to describe unusual case of a giant PA of the soft palate found in a 44‐year‐old male successfully managed at our facility. [ABSTRACT FROM AUTHOR]

Malignant mixed mullerian tumors (MMMT) are infrequent and highly malignant tumors. MMMTs, emerging from the uterine cervix, are very rare and to the best of our knowledge, there are no specific symptoms for the diagnosis of MMMTs whose early diagnosis is challenging. Almost all of them are diagnosed with pathological tests and reports. A 52-year-old post-menopause woman was referred to us, who suffered from postmenopausal bleeding from four months earlier. Upon pelvic examination, the position of the biopsy was identified in the anterior lip of the cervix. In former fractional dilatation and curettage, we found a pathology report with MMMT in the anterior lip of cervix. We performed a radical hysterectomy type II. In the permanent pathological report, MMMTs stage IBI was established. The patient was followed by chemoradiation. After 20 months, examination showed no evidence of recurrence.

Carcinosarcomas are endometrial neoplasms with malignant epithelial and mesenchymal components. These are rare tumors, corresponding to

Pleomorphic adenoma (PA) is the most common benign salivary gland tumor of which parotid gland involvement is the most common. PA may arise from minor salivary glands as well, however, PA is very rare in the sinonasal and nasopharyngeal areas. It usually affects middle aged females. They are frequently misdiagnosed due to high cellularity and myxoid stroma, leading to delay in diagnosis & further appropriate management. Here we present a case report of a female who presented with gradually progressive nasal obstruction, on examination found to have a nasal mass in right nasal cavity. Imaging was done and the nasal mass was excised. Histopathological report revealed a PA. Common tumor in an uncommon location: Pleomorphic adenoma of the nasal cavity – a case report. [ABSTRACT FROM AUTHOR]

Synopsis for table of contents: An exceptional number of synchronous MTC/PTC in the same thyroid gland is presented. This may be the most numerous case series reported in the literature. Synchronous PTC/MTC in the same thyroid gland were classified into 4 subtypes and the clinical and pathological aspects as well as the results are presented. Background and objectives: The synchronous occurrence of multiple neoplastic processes in the thyroid gland is unusual. We investigated the clinicopathological features of 30 medullary thyroid carcinomas (MTC) in association with papillary (PTC). Method: Retrospective analysis of operated patients for thyroid tumors. Synchronous PTC/MTC in the same thyroid gland were classified into 4 subtypes: (type I) True mixed MTC/PTC, MTC and PTC closely intermingled. (Type II) Collision MTC/PTC, i.e. tumors that meet at the same site, invade each other and appear as a single mass in the thyroid gland, i.e. MTC and PTC merge. (Type III) Synchronous anatomically separate tumors in the same thyroid lobe, i.e. separated from each other by non-tumorous thyroid parenchyma. (Type IV) Synchronous tumors occurring in separate anatomical lobes or in the isthmus. Clinical and pathological data were reviewed. Location: Department of thyroid surgery, China-Japan Union Hospital, Jilin University. Time frame: 14 years (June 2008-November 2022). Results: Thirty patients were identified with an overall prevalence of 28621 (0.1%). 17 (56.7%) were male, 13 (43.3%) female, mean age 51.3 ± 11.0 years, mean BMI 23.6 ± 3.6kg/m2. Mean duration of symptoms was 11.2 ± 18.4 months. Mean calcitonin level was 133.7 ± 196.4 pg/ml. Fine needle aspiration (FNA) was offered in 21 cases: 9 (42.9%) were suspected carcinoma, 9 (42.9%) PTC, 1 (4.8%) MTC, 2 (9.4%) MTC/PTC. Pathology revealed type I 4 (13.3%), type II 2 (6.7%), type III 14 (46.7%), type IV 10 (33.3%). The mean diameter of MTC was 1.6 ± 2.0cm, 18 (60%) were micro-MTC. The mean diameter of PTC was 0.9 ± 1.9 cm, 26 (86.7%) were micro-PTC. In 16 (53.3%) micro-PTC/-MTC occurred in synchronous sequence. Four patients had a recurrence: 2 had to be re-operated due to MTC recurrence, 2 died due to distant metastases (bone, liver). Conclusion: We report an exceptional number of MTC/PTC in the same thyroid gland. This may be the most numerous case series reported in the literature. The clinical and pathological aspects as well as the results are presented. [ABSTRACT FROM AUTHOR]

Soft tissue, or cutaneous, myoepitheliomas are rare tumors arising solely from a myoepithelial origin. These neoplasms are typically associated with uncertain differentiation and can contain cellular morphologies that include spindle, plasmacytoid, epithelioid, or clear cell forms. Soft tissue myoepitheliomas are commonly found on the lower limbs and in the pelvic girdle but can occur throughout the body. A small minority display heterogenous differentiation, typically osseous or cartilaginous in nature. Squamous and adipocytic cell types are much rarer. We report the case of myoepithelioma of soft tissue with both squamous and adipocytic metaplasia. In the largest myoepithelioma series of 101 soft tissue myoepitheliomas, there were only 2 cases of squamous metaplasia and 1 case of adipocytic metaplasia. Our case displays the unique occurrence of 2 rare histologic findings occurring simultaneously within an already uncommon neoplasm.

Malignant mixed mullerian tumors (MMMT) are infrequent and highly malignant tumors. MMMTs, emerging from the uterine cervix, are very rare and to the best of our knowledge, there are no specific symptoms for the diagnosis of MMMTs whose early diagnosis is challenging. Almost all of them are diagnosed with pathological tests and reports. A 52-year-old post-menopause woman was referred to us, who suffered from postmenopausal bleeding from four months earlier. Upon pelvic examination, the position of the biopsy was identified in the anterior lip of the cervix. In former fractional dilatation and curettage, we found a pathology report with MMMT in the anterior lip of cervix. We performed a radical hysterectomy type II. In the permanent pathological report, MMMTs stage IBI was established. The patient was followed by chemoradiation. After 20 months, examination showed no evidence of recurrence. [ABSTRACT FROM AUTHOR]

Mixed neuroendocrine non-neuroendocrine neoplasms are exceedingly rare tumors, especially those of gallbladder origin. Anomalous pancreaticobiliary junction is an uncommon congenital abnormality that can predispose various types of hepatobiliary malignancies. We present an unusual case of a 46-year-old woman with anomalous pancreaticobiliary junction who presented to the emergency department with nausea, vomiting, and right upper quadrant pain. Magnetic resonance imaging revealed a gallbladder mass concerning for primary malignancy and liver lesions. Endoscopic ultrasound and fine-needle biopsy were valuable diagnostic tools used to sample the gallbladder, liver lesions, and common hepatic duct under one minimally invasive procedure. Pathology showed a gallbladder mixed neuroendocrine non-neuroendocrine neoplasms, with neuroendocrine components in the liver and adenocarcinoma components in the common hepatic duct., (© 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)

Introduction Mixed tumor in the same lymph nodes is extremely rare and no previous reports have described mixed tumor comprising urothelial carcinoma and malignant lymphoma. Case presentation A 71‐year‐old woman visited a local clinic with a main complaint of hematuria. Imaging revealed right hydronephrosis and a mid‐ureter tumor shadow. Positron emission tomography–computed tomography showed high uptake of fluorodeoxyglucose in para‐aortic lymph nodes. Abdominal para‐aortic lymph node biopsy was performed. Pathology showed urothelial carcinoma and malignant lymphoma in the same lymph nodes, where a mixed tumor was diagnosed. Conclusion We encountered a case of mixed tumor of urothelial carcinoma and Hodgkin lymphoma, which metastasized to the same tissues.

Chondroid syringomas are rare, benign tumors originating from sweat glands occurring as singular lesions of the head and neck. This case report presents a patient in whom multiple chondroid syringoma lesions were found. Thus illuminating the possibility for such occurrences in the future of which physicians should be aware. [ABSTRACT FROM AUTHOR]

Objective: The present study aimed to investigate the histopathological types and distribution characteristics of gastric mixed tumors. Methods: Detailed histological observations, together with related immunohistochemical and genetic tests, were analyzed on 960 surgically resected samples in 6 hospitals with gastric mixed tumors from May 2017 to May 2021 in this retrospective study. Results: Epithelial-derived tumors accounted for 80.10% (769/960) of the gastric mixed tumor samples studied, and tumors of different tissue origins accounting for 10.83% (104/960), mesenchymal-derived tumors accounting for 6.25% (60/960), neuroendocrine tumors accounting for 2.40% (23/960), and lymphoma accounting for 0.42% (4/960). The histological types of gastric mixed tumors identified as most commonly were epithelial originated, followed by mixed tumors of different tissue originated, then mixed neuroendocrine, lymphoma, and mesenchymal originated in sequence. The histological number of gastric mixed tumors was ≤ 3 in 83.23% (799/960) of cases and > 4 in 16.77% (161/960) of cases. The mixed histological patterns of gastric mixed tumors were divided into three types: those with tumor cells interspersed with each other, those with incomplete fibrous tissue separation, and those without fibrous tissue separation. The gene target characteristics of gastric mixed tumors were the existence of multi-gene mutation, including human epidermalgrowth factor receptor-2 (HER2) gene amplification, key result areas (K-ras) and platelet-derived growth factor receptor alpha (PDGFRA). Conclusion: Gastric mixed tumors should be adequately sampled, each piece of tissue should be involved in the morphological proportional division of the tumor, and any independent histological component should be written into the pathological examination report. [ABSTRACT FROM AUTHOR]

Amaç: Bu çalışmada miks endometrial karsinomun (MEK) klinik ve patolojik özelliklerini değerlendirilmesi amaçlanmıştır. Gereç ve Yöntem: Ocak 2000 - Aralık 2019 tarihleri arasında kliniğimizde ameliyat edilen ve düzenli takip edilen 29 MEK hastasının klinik ve patolojik kayıtları gözden geçirildi. MEK grubundaki (n = 29) klinik-patolojik özellikler ve sağkalım, saf seröz (n = 42) ve saf berrak hücreli adenokarsinomlar (n = 13) ile karşılaştırıldı. Hastaların klinik bulguları, operasyon özellikleri, patolojik bulguları, miyometriyal invazyon derecesi (MI), lenf nodu tutulumu (LNI), lenfovasküler alan invazyonu (LVSI), adjuvan tedaviler ve takip verileri ve sağkalıma etkileri araştırıldı. Bulgular: Olguların 18'inde endometrioid + seröz, 7'sinde endometrioid + berrak hücreli, 3'ünde endometrioid + seröz ve 1'inde berrak hücreli+ seröz histopatoloji vardı. Hastaların ortalama yaşı 63,2 ± 12,1 yıldı. Mikst gruptaki olguların 8'ine (% 27,6) laparoskopik cerrahi uygulandı. Evre, LVSI, LNI, MI ≥% 50 ve omental metastaz oranları gruplar arasında benzerdi. Adjuvan tedavi alma oranları için gruplar arasında önemli bir fark yoktu. Genel olarak hayatta kalma süresi gruplar arasında benzerdi. Sonuç: MEK'ler, teşhis edilmesi ve yönetilmesi zor olabilen tümörlerdir. Klinikopatolojik özellikler ve prognoz açısından MEK ile saf seröz karsinom ve saf berrak hücreli karsinom arasında fark yoktu. Histopatolojik özelliklerinin yanı sıra moleküler özelliklerinin ortaya çıkarılması ve değerlendirilmesi bu grup tümörleri daha iyi anlamamıza yardımcı olacaktır.

ObjectiveThe present study aimed to investigate the histopathological types and distribution characteristics of gastric mixed tumors.MethodsDetailed histological observations, together with related immunohistochemical and genetic tests, were analyzed on 960 surgically resected samples in 6 hospitals with gastric mixed tumors from May 2017 to May 2021 in this retrospective study.ResultsEpithelial-derived tumors accounted for 80.10% (769/960) of the gastric mixed tumor samples studied, and tumors of different tissue origins accounting for 10.83% (104/960), mesenchymal-derived tumors accounting for 6.25% (60/960), neuroendocrine tumors accounting for 2.40% (23/960), and lymphoma accounting for 0.42% (4/960). The histological types of gastric mixed tumors identified as most commonly were epithelial originated, followed by mixed tumors of different tissue originated, then mixed neuroendocrine, lymphoma, and mesenchymal originated in sequence. The histological number of gastric mixed tumors was ≤ 3 in 83.23% (799/960) of cases and > 4 in 16.77% (161/960) of cases. The mixed histological patterns of gastric mixed tumors were divided into three types: those with tumor cells interspersed with each other, those with incomplete fibrous tissue separation, and those without fibrous tissue separation. The gene target characteristics of gastric mixed tumors were the existence of multi-gene mutation, including human epidermalgrowth factor receptor-2 (HER2) gene amplification, key result areas (K-ras) and platelet-derived growth factor receptor alpha (PDGFRA).ConclusionGastric mixed tumors should be adequately sampled, each piece of tissue should be involved in the morphological proportional division of the tumor, and any independent histological component should be written into the pathological examination report.

The stage I uterine malignant mixed mullerian tumor (MMMT) shows different potential for progression. We reason that MMMTs with high-grade carcinomatous component and positivity for HB-EGF are prone to recurrence/metastasis in the early stage. A retrospective clinical and histopathologic review with immunohistochemical staining for HB-EGF, EGFR, and integrin-α5 was performed for 62 surgically staged MMMT cases. Recurrence/metastasis (RM) is 6/18 (33%) in stage I disease. Of all the clinicopathologic variables and biomarkers analyzed for stage I MMMT, serous carcinomatous component (83% [5/6] versus 17% [1/12], P = .0015) and HB-EGF expression (100% [6/6] versus 50% [6/12], P=.0339) were significantly different between groups with RM and without RM. The presence of serous carcinoma in all stages was 83% (5/6) in stage I with RM, 8% (1/12) in stage I without RM, 20% (1/5) in stage II, 36.4% (8/22) in stage III and 64.7% (11/17) in stage IV; this was paralleled by HB-EGF expression of 100% (6/6), 50% (6/12), 40% (2/5), 50% (11/22) and 71% (12/17) with a correlation coefficient r=0.9131 (P=.027). HB-EGF and integrin-α5 were highly expressed in MMMTs bearing serous carcinoma component, compared to endometrioid and unclassifiable/miscellaneous subtypes (84.6%/47.6%/33.3%, P=.025 for HB-EGF; and 61.5%/42.9%/20.0%, P=.021 for integrin-α5). The EGFR positivity was comparable among the three subtypes (48.1%, 47.6% and 26.7%, P=.326). This study indicates that serous carcinomatous component championed by expression of HB-EGF predisposes to recurrence/metastasis in stage I MMMT. This process might involve integrin-α5 and does not seem to require overexpression of EGFR. Further study is required.

Soft tissue myoepithelioma (STM), a rare mesenchymal neoplasm morphologically analogous to its more common salivary gland (SG) counterpart, is the subject of single case reports regarding its fine-needle aspiration (FNA) biopsy. To our knowledge, ours is the first case series of STM. A search was made of our pathology databases for cases diagnosed as STM. FNA biopsy smears and cell blocks were performed using standard techniques. Seven cases were retrieved from 4 men and 3 women (M:F = 1.3:1; age range: 25-79 years, x = 54 years). All but 1 presented as a primary neoplasm. Six aspirates were from the extremities, and 1 from the abdominal wall. Mean tumor size was 5.7 cm. Cytologic diagnosis of STM or suspicious for STM was made in 3 cases (43%). Remaining FNA diagnoses were spindle cell neoplasm/lesion (2), spindle cell sarcoma (1), and extraskeletal myxoid chondrosarcoma (1). Three cases were composed primarily or solely of uniform spindle cells, 3 primarily of uniform epithelioid cells with plasmacytoid features, and 1 case a mixture of these 2 cell types. Myxoid/chondromyxoid stroma was relatively abundant except in the single hypocellular example. Immunohistochemical (IHC) testing performed in 71% was nonspecific, but positive with S-100 in 4 of 5, EMA in 3 of 3, calponin in 2 of 2, and keratin in 1 of 3 examples. FNA biopsy smears of STM are remarkably similar cytomorphologically to their SG equivalent. However, STM can be misidentified principally as extraskeletal myxoid chondrosarcoma, thus requiring a relatively broad IHC panel for a specific diagnosis. • A series of 7 fine-needle aspiration (FNA) biopsy cases of soft tissue myoepithelioma (STM) is presented. • Correct FNA diagnosis was suggested in 43% of cases. • STM cytomorphology is composed of spindle and epithelioid cells, but 1 cell type is usually predominant. • STM FNA cytopathology imitates its salivary gland counterpart. • Extraskeletal myxoid chondrosarcoma is a close mimic of STM in FNA smears. [ABSTRACT FROM AUTHOR]

Pleomorphic adenoma is most common benign neoplasm of the major salivary glands that predominantly affects the superficial lobe of the parotid gland and rarely minor salivary glands. These tumors commonly occur in the fourth, fifth and sixth decades with increased predilection towards females and clinically manifests as unilateral, slow growing, firm and painless mass. Their occurrence on the tongue has been very rarely reported in the literature. This case report describes a rarest case of pleomorphic adenoma involving the right dorsolateral surface of tongue in a 28 year old male patient.

Pleomorphic adenoma is the most common salivary gland tumor which accounts for about 60% of all salivary neoplasms. It is also known as "mixed tumor because of its wide cytomorphologic diversity". Pleomorphic adenoma salivary glands mostly occurs on the palate, but the involvement of the upper lip is rare. The present report describes a case of a 62-year-old male with asymptomatic firm nodular swelling attached with upper lip which was later diagnosed as pleomorphic adenoma in the excisional biopsy. [ABSTRACT FROM AUTHOR]

Chondroid syringoma (CS) represents the cutaneous counterpart of mixed tumor (pleomorphic adenoma) of salivary glands. Definitive diagnosis is made on histopathology and is based on the presence of characteristic epithelial and stromal components. We report a case of an atypical CS arising on the extremity of an elderly male patient. Histomorphologic features of necrosis and cellular atypia raised suspicion for malignant degeneration, an exceptionally rare circumstance in this context. To further support the diagnosis of malignancy, array comparative genomic hybridization was performed from both low and higher grade areas of the tumor. Both regions demonstrated multiple copy number gains and losses, with additional loss of q7p (TP53), loss of 19p, and loss of heterozygosity on16q demonstrated in the more atypical foci. To our knowledge, this is the first case description of malignant degeneration of a CS with correlative microarray analysis. The findings in this case may prove useful in confirming the diagnosis in future ambiguous cases. [ABSTRACT FROM AUTHOR]

Background: Mixed adenoneuroendocrine carcinoma of the gallbladder (gMANEC) is an extremely rare cancer. Most of the cases are reported in Asia, North America, and Europe, with no cases reported in Latin America; this is the first report for this region, and the 24th case reported worldwide. Case Report: A 68-year-old woman was referred to our department due to asthenia and moderate abdominal pain in the right upper quadrant for 6 months, with imaging examinations showing a solid heterogeneous expansive lesion in gallbladder topography and segment IV of the liver. The MRI displayed an expansive and heterogeneous lesion with inaccurate limits in the gallbladder affecting segment IVb of the liver, in addition to lymphadenopathy in the hepatic hilum. A cholecystectomy with resection of segments IV-B and V of the liver (radical cholecystectomy) and hepatic hilar lymphadenectomy were performed. Anatomopathological examination and immunohistochemistry confirmed a primary mixed adenoneuroendocrine carcinoma of the gallbladder. The patient received adjuvant chemotherapy and radiotherapy; however, after the patient reported experiencing low back pain, a CT was performed, revealing retroperitoneal metastasis, and the radiotherapy was interrupted. Currently, the patient has a stable disease, following a protocol of 5-Fluorouracil and somatostatin, and she reports having low back pain of low intensity. Conclusions: This is the 24th gMANEC case reported in the literature. The tumor was successfully resected; however, the patient presented retroperitoneal metastasis 6 months after surgery, despite combined adjuvant therapy. [ABSTRACT FROM AUTHOR]

Summary: Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.

INTRODUCTION: The pleomorphic adenoma is the most common benign mixed tumor of salivary glands, rarely found in the nasal cavity. Its typical clinical presentation is a unilateral nasal obstruction associated with an epistaxis and a presence of a polypoid, smooth, lobulated, and firm mass in the nasal fossa. It is diagnosed by anatomopathological examination, and the treatment of choice is surgical with tumor resection, keeping a safety margin. CASE REPORT: The case reported is a female 49-year-old patient, diagnosed with a nasal pleomorphic adenoma that was surgically treated. CONCLUSION: The importance of the case reported lies in the rarity of the pleomorphic adenoma located in this area. Therefore, this is a differential diagnosis that must be remembered for nasal tumors. [ABSTRACT FROM AUTHOR]

ObjectivePleomorphic adenomas (PAs) with divergent clinical behavior, differing from the vast majority of PAs, were distinguished. “Fast” PAs are characterized by an unexpectedly short medical history and relatively rapid growth. The reference group consisted of “slow” PAs with very stable biology and long-term progression. We divide the PA group as a whole into three subsets: “fast,” “normal,” and “slow” tumors. Our goal is a multifactorial analysis of the “fast” and “slow” PA subgroups.MethodsConsecutive surgeries in a tertiary referral center, the Department of Otolaryngology and Laryngological Surgery, Poznan University of Medical Sciences, Poland, were carried out between 2002 and 2011. Out of 1,154 parotid tumors, 636 (55.1%) were PAs. The data were collected prospectively in collaboration with the Polish National Registry of Benign Salivary Gland Tumors. The main outcome measure was the recurrence rate in “fast” and “slow” PA subgroups. All surgical qualifications and surgeries were performed by two experienced surgeons.ResultsSlow PAs, compared to fast PAs, presented in older patients (53.25 ± 15.29 versus 47.92 ± 13.44 years). Multifactor logistic regression analysis with recurrence (yes/no) as the outcome variable, fast/slow as the predictor variable and age, gender, margin, FN status as covariates showed that fast PAs were significantly predicting recurrence vs. slow PAs (p = 0.035). Fast PAs were increasing the risk of PAs 10-fold vs. slow PAs, exp β = 10.20, CI95 [1.66; 197.87]. The variables impacting relapse were recent accelerated growth of the tumor OR = 3.35 (SE = 0.56), p = 0.030, positive margins OR = 7.18 (SE = 0.57), p < 0.001, incomplete or bare capsule OR = 9.91 (SE = 0.53), p = 0.001 and location III OR = 3.12 (SE = 0.53), p = 0.033. In the multivariate model only positive margin was selected as the best predictor of relapse, OR = 5.01 (SE = 0.60), p = 0.007.ConclusionsThe simple clinical aspect of slow or fast PA progression is of great practical importance and can constitute a surrogate of the final histopathological information that is derived from the surgical specimen. The slow or fast nature of the PA to some extent indicates prognostic features such as recurrence risk. This finding requires correlation with histological and molecular features in further stages of research.

Objective: Pleomorphic adenomas (PAs) with divergent clinical behavior, differing from the vast majority of PAs, were distinguished. "Fast" PAs are characterized by an unexpectedly short medical history and relatively rapid growth. The reference group consisted of "slow" PAs with very stable biology and long-term progression. We divide the PA group as a whole into three subsets: "fast," "normal," and "slow" tumors. Our goal is a multifactorial analysis of the "fast" and "slow" PA subgroups. Methods: Consecutive surgeries in a tertiary referral center, the Department of Otolaryngology and Laryngological Surgery, Poznan University of Medical Sciences, Poland, were carried out between 2002 and 2011. Out of 1,154 parotid tumors, 636 (55.1%) were PAs. The data were collected prospectively in collaboration with the Polish National Registry of Benign Salivary Gland Tumors. The main outcome measure was the recurrence rate in "fast" and "slow" PA subgroups. All surgical qualifications and surgeries were performed by two experienced surgeons. Results: Slow PAs, compared to fast PAs, presented in older patients (53.25 ± 15.29 versus 47.92 ± 13.44 years). Multifactor logistic regression analysis with recurrence (yes/no) as the outcome variable, fast/slow as the predictor variable and age, gender, margin, FN status as covariates showed that fast PAs were significantly predicting recurrence vs. slow PAs (p = 0.035). Fast PAs were increasing the risk of PAs 10-fold vs. slow PAs, exp β = 10.20, CI95 [1.66; 197.87]. The variables impacting relapse were recent accelerated growth of the tumor OR = 3.35 (SE = 0.56), p = 0.030, positive margins OR = 7.18 (SE = 0.57), p < 0.001, incomplete or bare capsule OR = 9.91 (SE = 0.53), p = 0.001 and location III OR = 3.12 (SE = 0.53), p = 0.033. In the multivariate model only positive margin was selected as the best predictor of relapse, OR = 5.01 (SE = 0.60), p = 0.007. Conclusions: The simple clinical aspect of slow or fast PA progression is of great practical importance and can constitute a surrogate of the final histopathological information that is derived from the surgical specimen. The slow or fast nature of the PA to some extent indicates prognostic features such as recurrence risk. This finding requires correlation with histological and molecular features in further stages of research. [ABSTRACT FROM AUTHOR]

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Objective: Unusual clinical course Background: Testicular mixed germ cell tumors (GCTs) represent a spectrum of malignancies that differ in terms of histopathology, clinical complications, and overall outcome. A variety of aggressive combinations containing different histological types have been described among such testicular tumors. However, a histopathology characterized by a combination of teratoma and choriocarcinoma, as seen in this case, in which the teratomatous component shows a secondary transformation to chondrosarcoma, is considered very rare. Case Report: The patient presented with progressive hemoptysis and dyspnea secondary to bilateral pulmonary cannon-ball lesions indicative of a metastatic process. His workup was remarkable for primary testicular cancer complicated by liver metastasis and very high levels of B-HCG at more than 175 000 mlU/ml. He deteriorated quickly with no improvement following the first cycle of Etoposide/Cisplatin (EP) chemotherapy regimen and died 15 days after starting cancer treatment. Such non-seminomatous GCTs with extrapulmonary visceral metastasis associated with very high tumor markers are deemed poor risk based on the International Germ Cell Cancer Collaborative Group (IGCCCG) criteria, with a reported 5-year overall survival rate reaching up to 73%. Conclusions: This case is considered unique in terms of rapid clinical deterioration and lack of improvement following the standard EP chemotherapy regimen. This unusual dramatic presentation should draw attention to the possible association between the aggressiveness of the disease and its very rare histopathology. [ABSTRACT FROM AUTHOR]

Background: The aim of the study was to present a case of mixed olfactory neuroblastoma (ONB) and carcinoma, an extremely rare tumor with only a few cases in the published literature. Case Description: An otherwise healthy 27-year-old male presented with sinus complaints, headache, and unilateral eye discharge. Imaging and endoscopy revealed a mass presumed to represent a juvenile nasopharyngeal angiofibroma. Unexpectedly, the final pathology report revealed high grade mixed ONB and carcinoma. This tumor is the sixth and youngest documented patient with mixed ONB and carcinoma. Conclusion: Physicians should remain vigilant for the possibility of malignancy in their approach to nasal cavity masses, even in young otherwise healthy patients. Careful review of the immunohistopathology should also be taken, as mixed olfactory tumors such as these are aggressive, rare entities that require multidisciplinary oncologic care. [ABSTRACT FROM AUTHOR]

Carcinosarcoma, a biphasic malignant mixed tumor, is an extremely rare neoplasm with >1% incidence. This aggressive malignancy is characterized by the presence of two components admixed with each other, i.e., the epithelial component and the mesenchymal component arising from a monoclonal/multiclonal origin or de novo. Most patients usually present between 60 and 65 years of age with no sex predilection. The authors present a case of carcinosarcoma arising as a mass in the neck region of a 14-year-old male. The case is been presented for its rarity of occurrence in the younger age group. [ABSTRACT FROM AUTHOR]

Background and Aim: Recent study showed that early gastric cancer (EGC) with Lauren mixed‐type (MT) histology is associated with worse prognosis. We aimed to evaluate the clinicopathologic features and prognostic significance of Lauren MT‐EGCs that meets the criteria for endoscopic submucosal dissection (ESD). Methods: We reviewed 2665 patients with EGC who underwent surgery between 2010 and 2015. The clinicopathologic features and invasiveness including lymph node metastasis (LNM) and lymphovascular invasion (LVI) of MT‐EGC were compared with those of intestinal type and diffuse type by Lauren histology. Results: Among 2665 patients, EGCs in 241 (9%) patients were classified as MT. Tumor size was larger and depth of invasion was greater than other histology. Among patients with MT‐EGC, 16.6% (40/241) showed LNM and 22.8% (55/241) showed lymphatic invasion, which were significantly higher than that of patients with other Lauren types (8.2% and 15.3% in intestinal type and 9.1% and 8.7% in diffuse type, P < 0.001). This finding remained significant even after adjusting for depth of invasion. However, when we analyzed the patient groups who met the absolute or expanded criteria of ESD, no significant difference was observed in the rates of LNM or LVI or cancer mortality by Lauren classification. Conclusion: Mixed‐type early gastric cancer (MT‐EGC) exhibited larger tumor size, greater depth of invasion, and higher risk of LNM and LVI. However, among the patients who met the absolute or expanded criteria of ESD, no significant difference was observed in LNM, LVI, and gastric cancer mortality risk. [ABSTRACT FROM AUTHOR]

Primary myoepithelial neoplasms of soft tissue have been shown to be related to their salivary gland counterparts, with which they often share morphologic, immunophenotypic, and molecular genetic features, such as the presence of PLAG1 rearrangement in both soft tissue mixed tumor and salivary pleomorphic adenoma. However, important distinctions remain between soft tissue and salivary myoepithelial neoplasms, namely differing criteria for malignancy. This review provides an overview of the current understanding of the clinicopathologic and molecular features of soft tissue myoepithelial neoplasms, including discussion of the similarities and differences between soft tissue and salivary counterparts and relevant diagnostic issues specific to head and neck pathology practice. [ABSTRACT FROM AUTHOR]

Odontomas are developmental malformations of dental tissues, and they may interfere with the eruption of the associated tooth. Odontomas have limited growth potential, and their occurrence in the primary dentition is uncommon. This article describes a case of a large complex odontoma in a 5.2-year-old boy that prevented eruption of the mandibular left lateral incisor. The odontoma was surgically removed, routine follow-up was done for more than a year, and no recurrence was seen. [ABSTRACT FROM AUTHOR]

Among these, the most common pathological type is pleomorphic adenoma also called the mixed tumor. It is a kind of tumor containing glandular tissue, myxoid and cartilage-like tissue. As the structure of the tissue is diverse, it is called a "mixed tumor." Actinomycosis is a chronic suppurative bacterial infection caused by Actinomyces israelii. In this case report, we are presenting a rare co-existence of pleomorphic adenoma and Actinomycosis in the infratemporal fossa. Case description: A 40-year-old female patient complains of pain over the upper left back tooth region for the past 6months. The patient had a past history of a small lesion in 28 region which resulted in constant irritation. The patient had consulted the dentist for the same and underwent extraction of 28 and 38 three years back, which was thought to be the cause of the swelling. Conclusion: The case highlights the importance of proper history taking, clinical examination, and histopathological examination in arriving at a precise diagnosis of a lesion irrespective of size and clinical presentation. [ABSTRACT FROM AUTHOR]

The article describes a case of pleomorphic adenoma in a 61-year-old patient, its diagnostic methods and treatment.

Pleomorphic adenoma (PA) is the most common benign tumor of major salivary glands, and it rarely arises in minor salivary glands. PA has been reported at various sites where minor salivary glands are found and include nasal cavity, nasopharynx, pharynx, larynx, hypopharynx, and even lacrimal glands. We report one such a case in the nasal cavity in a middle-aged woman. The patient presented with nasal mass and symptoms of obstruction and epistaxis. After clinical and radiological evaluation, the mass was excised endoscopically, and histopathological examination revealed a biphasic benign cellular tumor with scant hyalinized stroma. On immunohistochemistry, the tumor was diffusely positive for CK7 and focally positive for P40 and smooth muscle actin (SMA). Proliferation index was 2%–3%. Finally, the diagnosis of PA was rendered. PA is generally restricted to the cavity due to paucity of space and presents early in the course. Surgical excision with wide margins is the treatment of choice. There are chances of recurrences and malignant transformation of the tumor. To conclude, although rare, one should consider a possibility of PA in the differentials of nasal masses, and accurate diagnosis of this entity is essential to ensure follow-up as recurrence and malignant transformation are reported in PA. [ABSTRACT FROM AUTHOR]

Mixed adenoneuroendocrine carcinomas are rare and usually occur in the gastrointestinal tract. Although there have been several investigations regarding their developmental mechanism, the molecular origin of these tumors remains unclear. In this article, we present an exceedingly rare case of a mixed tumor of the urinary bladder with an adenocarcinomatous and a neuroendocrine component and a concomitant urothelial carcinoma in situ (CIS). Due to this extraordinary combination of tumor components, our goal was to extensively examine the 3 tumor components with regard to a representable common origin. Therefore, a comprehensive immunohistochemical analysis and review of the literature was performed. Besides expected outcome, our examination also revealed surprising staining results. Urothelial CIS, like the adenocarcinomatous component, showed strong staining for CDX2. In addition, parts of the adenocarcinoma were positive for synaptophysin like the neuroendocrine tumor component. All 3 components showed a significant overexpression of p53 and a moderate to strong membranous and cytoplasmatic staining for β-catenin. To our knowledge, we are the first to describe a case of a mixed tumor of the urinary bladder with an adenocarcinomatous and a neuroendocrine component and a concomitant CIS. The components share striking molecular features that argue for a common clonal origin and a development of the invasive tumor via the urothelial precursor lesion. [ABSTRACT FROM AUTHOR]

Mixed carcinomas in the esophagus are highly uncommon neoplasms that represent a diagnostic challenge on small tissue biopsies. We present a case of a primary mixed sarcomatoid–small cell carcinoma of the esophagus that was diagnosed after repeat sampling of the lesion. The components were morphologically distinct and could be further classified by immunohistochemistry. Next-generation sequencing identified mutations in PIK3CA and CDKN2A. The small cell component morphology was also identified in brain metastasis. [ABSTRACT FROM AUTHOR]

Myoepithelial neoplasms of soft tissue represent a rare entity which has been described only recently when compared to salivary gland tumors with whom they share histopathological and molecular features. The most common locations are the superficial soft tissues of the limbs and limb girdles. However, they can rarely occur in the mediastinum, abdomen, bone, skin and visceral organs. Benign forms (myoepithelioma and mixed tumor) are more frequent than myoepithelial carcinoma and the latter mostly affects children and young adults. Diagnosis is mainly based on histology, which shows a proliferation of myoepithelial cells of variable morphology with or without glandular structures in a myxoid background, and immunohistochemistry, which shows co-expression of epithelial and myoepithelial markers. Molecular tests are not mandatory, but in selected cases FISH analysis can prove useful as about 50% of myoepitheliomas show EWSR1 (or rarely FUS) rearrangements and mixed tumors show PLAG1 rearrangements. Here, we present a case of a mixed tumor of the soft tissue occuring in the hand with expression of PLAG1 in immunohistochemistry., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)