[Multimodal treatment of malignant mixed Müllerian tumor]. / Malignus kevert Müller-cso-eredetu tumor komplex kezelése.

INTRODUCTION AND AIM: The aim of our study was to evaluate the prognostic factors and treatment options of a very rare and highly aggressive type of uterine neoplasms, the malignant mixed Müllerian tumor, known as carcinosarcoma. METHOD: Between 2009 and 2017, 29 patients were treated with malignant mixed Müllerian tumor. At stage I, surgery and postoperative radiotherapy were performed. At stages II-IV, trimodal treatment (surgery, chemotherapy and radiotherapy) was administered. RESULTS: The average age of patients was 68.51 (49-90) years, mean body mass index was 30.22 (20.90-37.22). We have experienced recurrence of disease after complete resection in 6 cases (4 of 6 patients did not accept radiation therapy). Local recurrence has occurred after an average 15.52 (6-36) months, distant metastasis with an average 19.2 (8-32) months. Overall survival was 11.92 (1-75) months. Six patients are free of tumours at the moment. CONCLUSIONS: As overall survival has not increased in recent decades by using combined chemotherapy, there is no congruent consensus associated with the optimal treatment. The standard surgical treatment is total abdominal hysterectomy with bilateral oophorectomy, although due to high rates of recurrence and metastases, the necessity of lymphadenectomy and postoperative treatment is in the focus of recent studies. Though postoperative irradiation improves local control, the beneficial effect on overall survival is still not proven. Adjuvant chemotherapy decreases the rate of both pelvic and extrapelvic recurrence at the same time, although there is no recommendation for the optimal chemoterapeutic agent. Multimodal therapy should lead to better outcomes. Recently there are many ongoing studies with biologic and target therapies to improve efficiency, however, the relevant results will be disclosed in many years only, due to the small number of patients. Orv Hetil. 2018; 159(19): 741-7747.

[About a rare tumor of the kidney: Malignant mixed epithelial and stromal tumor]. / À propos d'une tumeur rarissime du rein: la tumeur mixte épithéliale et stromale maligne.

Mixed epithelial and stromal tumor of the kidney is a recently described neoplasm that predominantly affects perimenopausal women. Few cases with malignant features have been reported. Here, we report the case of malignant mixed epithelial and stromal tumor of the kidney with sarcomatous transformation arising in a 27-year-old female. She presented with abdominal discomfort. Computed tomography of the abdomen revealed a large compressive mass arisen from the left kidney with solid and focal cystic components. The patient underwent left radical nephrectomy. Histologic sections showed benign and malignant components. The benign component consisted of multiple tubules and variably sized cysts lined by benign epithelium. The malignant component was composed of undifferentiated cellular spindle cell sarcoma. By immunohistochemical studies, the epithelial component was positive for cytokeratins and epithelial membrane antigen (EMA). The stromal component displayed strong immunohistochemical expression of vimentin, CD99, bcl2; and was negative for cytokeratins, desmin, SMA, S-100, estrogen receptor (ER) and progesterone receptor (PR). Analysis by reverse transcriptase polymerase chain reaction (RT-PCR) failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma.

Combined hepatocellular-cholangiocarcinoma (cHCC-CC): a distinct entity.

Patients with hepatocellular carcinoma (HCC), the fifth most common cancer worldwide, display a highly variable clinical course, suggesting that HCC encompasses several biologically distinct subtypes. This heterogeneity has the potential to impede both treatment decisions and prognostic predictions for patients with HCC. One distinct, albeit rare, subtype of HCC is combined hepatocellular-cholangiocarcinoma (cHCC-CC), which overall carries a poorer prognosis than HCC and cholangiocarcinoma (CC) alone. This review discusses predominantly the histopathologic and pathogenetic intricacies of this tumor and highlights the need for an accurate diagnosis of this specific HCC subtype.

Uterine endometrial carcinoma with trophoblastic differentiation: a case report with literature review.

Choriocarcinoma is categorized as either gestational or nongestational depending on its origin. Nongestational choriocarcinoma originated in the trophoblastic differentiation is a rare but an aggressive tumor. This article reports a nongestational case of a uterine endometrial carcinoma with trophoblastic differentiation. A 54-year-old woman with a history of atypical genital bleeding that underwent semi-radical hysterectomy, bilateral salpingo-oophrectomy, and pelvic lymph nodes dissection. Pathological investigation showed that the tumor had endometrioid adenocarcinoma and choriocarcinomatous components. Although a series of multimodality treatments including craniotomy were performed, she died of aggressive lung and brain metastases one year after the primary surgery.

Fine needle aspiration of an unusual malignant mixed tumor in the parotid gland.

OBJECTIVE: The use of fine needle aspiration (FNA) biopsy in the triage of salivary gland tumors has been well established. The sensitivity and specificity of FNA biopsy for tumor diagnosis is generally very good. However, the diagnosis can be challenging due to the limited amount of tissue sampled, the occasional overlapping tumor morphology, and the presence of a malignant counterpart of a benign tumor. METHODS: FNA biopsy was performed with ultrasound guidance. Air-dried slides and alcohol-fixed slides were made for Diff-Quik staining and Papanicolaou staining, respectively. The syringes were rinsed and a cell block was prepared. The resected specimen was fixed in 10% formalin and processed by routine histology techniques. RESULTS: We report a rare case of a salivary gland neoplasm with 2 distinct components: adenoid cystic carcinoma and pleomorphic adenoma. These 2 components are clearly identifiable in both cytological materials from fine needle aspiration and histological analysis of surgical resection. CONCLUSION: Pleomorphic adenoma is the most common salivary gland tumor for patients in all age groups. The characteristic cytological feature is fibrillary extracellular matrix intermixed with epithelial cells. Malignant transformation occurs in 5% to 10% of cases. Rarely, pleomorphic adenoma exists as one component of a hybrid tumor. The surgical resection will be needed in those cases. The final diagnosis relies on the combination of the clinical information, histological findings and molecular study.

Mixed neuroendocrine tumor of the common bile duct.

CONTEXT: Mixed adenoendocrine tumors of the extrahepatic bile ducts are exceedingly rare and most of those who are rarely diagnosed are adenocarcinomas. Neuroendorine tumors accounts for only 0.2-2%. CASE REPORT: We report a case of mixed adenoneuroendo-carcinoma of the common bile duct in an 82-year-old male. CONCLUSION: Clinical experience suggests that the neuroendocrine component of mixed tumors behave more aggressively than the regular biliary adenocarcinoma component. This clinical behavior may have an important role in the management of this clinical entity.

[Plexiform angiomyxoid myofibroblastic tumour of the stomach]. / Plexiformer angiomyxoider myofibroblastischer Tumor des Magens.

Recently the so-called plexiform angiomyxoid myofibroblastic tumors (PAMT) have emerged as a new entity of gastric soft tissue tumors and the light microscopic and immunohistological characteristics have now been well described. Until now PAMTs have not yet been reported in the German speaking literature. Worthy of note is that PAMTs can be diagnosed safely in sufficient biopsy material which enables adequate therapeutic steps to be initiated without delay, because PMATs although considered to be benign, can cause serious complications.

Cholangiolocellular carcinoma: an innocent-looking malignant liver tumor mimicking ductular reaction.

The authors present an interesting case of a 60-year-old man who underwent right hepatectomy for a diagnosis of hepatocellular carcinoma (HCC) on a background of noncirrhotic chronic hepatitis C. Pathologic examination confirmed the presence of HCC near the porta hepatis, which invaded the right portal vein branch. In addition, a well-demarcated 13.5 × 7.8 × 4.0 cm yellow and firm area upstream of the HCC was noted. This yellow area corresponded to a tumoral ductular proliferation, which cytologically was extremely bland, but invaded portal tracts and the adjacent liver parenchyma. This tumoral proliferation mimicked ductular reaction, except that it had more anastomosing structures and was associated with abundant hyalinized fibrotic stroma. Cytologically, the tumor cells had round to oval nuclei with fine chromatin, indistinct nucleoli, and scant cytoplasm. They exhibited immunohistochemical features of hepatic progenitor cells, i.e., expressing CK7, CK19, and N-CAM; and their malignancy was supported by the p53 and Ki67 immunoreactivity. The authors concluded that the patient had cholangiolocellular carcinoma with an aggressive hepatocellular carcinoma component. Cholangiolocellular carcinoma has been reported to be associated with chronic hepatitis C viral infection and to derive from hepatic progenitor cells, which explains why hepatocellular carcinoma and/or cholangiocarcinoma component may be present.

Metaplastic breast carcinoma: a case report and systematic review of the literature.

A 78-year-old retired woman was diagnosed with metaplastic breast carcinoma (MBC), a rare tumor, in our hospital. We reviewed 15 articles with a total of 1328 patients to determine the epidemiology, clinical features, biomarkers, histology, management and outcome of patients with this tumor. The mean age at presentation is 58.5 years (range 32-83). Eighty-one percent of patients presented either with a breast mass or abnormal mammographic finding. Twenty-three percent of patients had a family history of breast cancer. Estrogen receptors were only found in 12%, progesterone receptors in 10% and HER2 in 6% of patients. The main method of treatment was mastectomy (66.9%) in combination with chemotherapy (57%) and radiotherapy (47%). Five-year disease-free survival ranged between 40% and 84% and 5-year overall survival ranged between 64 and 83%. We have further reviewed the nature of this disease in the light of advancement in genetics, such as microarray gene expression profiling. The relationship of MBC with triple-negative tumor and basal-like tumor is discussed. It is hoped that advances in genetics and biomarkers will bring forward the era of personalized medicine in the treatment of breast carcinoma.

Primary malignant mixed müllerian tumor of the peritoneum a case report with review of the literature.

Malignant mixed Müllerian tumor is a rare malignancy of the genital tract and extremely uncommon in extragenital sites. This report describes a case of malignant mixed Müllerian tumor arising in the lower peritoneum of a 72-year-old female patient. The patient presented with ascites, lower abdominal mass and pleural effusion. The serum level of CA125 was elevated. At operation a diffuse carcinosis associated with tumor mass measuring 20 × 15 × 10 cm in the vesicouterine and Duglas' pouch were found. The uterus and the adnexa were unremarkable. Histopathology revealed a typical malignant mixed Müllerian tumor, heterologous type. The epithelial component was positive for cytokeratin 7 and vimentin whereas the mesenchymal component was positive for Vimentin, S100 and focally for CK7. The histogenesis of this tumor arising from the peritoneum is still speculative. Based on the previous reports and the immunohistochemical analysis of our case, we believe that this is a monoclonal tumor with carcinoma being the "precursor" element. Nevertheless, further molecular and genetic evidence is needed to support such a conclusion.

Mixed epithelial and stromal tumor of the kidney with elevated serum level of cancer antigen 125.

Mixed epithelial and stromal tumor of the kidney is a newly categorized lesion, with few reported cases. We report a rare case of a 45-year-old woman with a palpable abdominal mass and elevated serum level of serum cancer antigen 125, who was not receiving hormones or contraceptive agents. Abdominal magnetic resonance imaging revealed a large multilocular cystic tumor that arose in the left central kidney. Nephrectomy was performed under the initial impression of cystic renal cell carcinoma; however, a diagnosis of mixed epithelial and stromal tumor was confirmed according to pathological and immunohistochemical findings. Serum level of cancer antigen 125 returned to normal after 1 month postoperatively and no recurrence was found in the following 18 months.

A case of mixed ductal-endocrine carcinoma of the pancreas.

A 66-year-old male patient underwent a stomach-preserving pancreatoduodenectomy procedure because of a tumor located around the lower bile duct under the diagnosis of carcinoma of the lower bile duct. The tumor (3.5 × 2.5 cm) was found at the head of the pancreas and had invaded the papillae of Vater at the duodenum. Histology findings indicated both ductal adenocarcinoma and endocrine tumor. The ductal adenocarcinoma component expressed carcinoembryonic antigen, cytokeratin (CK)-19, CK-20, carbohydrate 19-9, and amylase, whereas the endocrine component, which occupied about one-third of the tumor, expressed glucagon, neuron-specific enolase, and chromogranin A. The Ki-67 labeling indices of the two components were 49.7% and 5.3%, respectively. Herein, we present this case of mixed ductal-endocrine carcinoma of the pancreas. Our findings indicate that its aggressive mass may be ascribable to the adenocarcinoma component with a high proliferative potential.

[Mixed tumor in the lower eyelid of Moll's gland origin].

BACKGROUND: Mixed tumors originating from both tubular epithelial and myoepithelial cells usually develop in the major lacrimal gland of the ocular adnexa. They rarely develop in the eyelid. Mixed tumors arising from Moll's glands are extremely rare. CASE: A 35-year-old woman reported a mass in the margin of the left lower eyelid of 5 years' duration. The tumor size was 4 mm and total resection of the lesion was performed. Histopathological examination showed a well-circumscribed nodular lesion located in the dermis; containing fibrous, hyaline and myxoid elements. The tumor growth showed cords and nests of proliferating tubular epithelial and myoepithelial cells. Spindle-shaped myoepithelial cells were observed. The tumor was diagnosed as a mixed tumor. Immunohistochemically, the tumor cells were positive for S-100 protein, and proliferative lesions of tubular epithelial cell origin were strongly positive for gross cystic disease fluid protein-15 (GCDFP-15). CONCLUSION: Mixed tumors of the eyelid may arise from the accessory lacrimal glands of Krause and Wolfring, eccrine sweat glands or apocrine (Moll's) sweat glands. This case was considered to be of Moll's gland origin because of its location, the features of the surrounding tissue, its composition of tubular epithelial cells and its positive staining for GCDFP-15.

Mixed endocrine somatostatinoma of the ampulla of vater associated with a neurofibromatosis type 1: a case report and review of the literature.

CONTEXT: Mixed endocrine tumors are double neoplasms with both glandular and endocrine components; these tumors are rare, especially those arising in the ampulla of Vater. Ampullary somatostatinomas are classically associated with neurofibromatosis type 1. We herein describe the first reported case of a mixed endocrine somatostatinoma of the ampulla of Vater associated with neurofibromatosis type 1; we also present a review of the literature of the 7 mixed endocrine tumors of the ampulla which have been reported so far. CASE REPORT: A 49-year-old woman presented with atypical abdominal pain. Endoscopic examination revealed a tumor involving the ampulla of Vater and a CT scan identified stenoses of both the distal common bile duct and the main pancreatic duct. A pancreaticoduodenectomy was performed and pathological examination revealed two tumor components, exocrine (high grade adenoma with infiltrative adenocarcinoma) and endocrine (expressing somatostatin hormone) with lymph node metastases originating from both types. The patient was treated with adjuvant chemotherapy and has had no recurrence for 3 years. DISCUSSION: In ampullary somatostatinomas, psammoma bodies are pathognomonic and chromogranin A is rarely expressed: these features should alert the pathologist to an association with neurofibromatosis type 1. The management of patients with mixed tumors is challenging. The treatment of choice is surgery, and adjuvant chemotherapy should be adapted to the most aggressive component, i.e. the exocrine one. CONCLUSION: Because of their rarity, the diagnosis of ampullary mixed endocrine tumors is difficult. Our case points out the characteristic features of these neoplasms and their possible association with neurofibromatosis type 1.

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: a case report and literature review.

Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor composed of both adenocarcinoma and neuroendocrine components. Here, we report the case of a 75-year-old woman with ampullary MANEC. She visited a physician with the chief complaint of dark urine and was diagnosed with advanced jaundice. Subsequently, she was referred to our hospital. Contrast-enhanced computed tomography scan revealed a neoplastic lesion measuring approximately 2 cm with a contrast effect at the duodenal papilla. Upper endoscopy showed a non-exposed tumor at the duodenal papilla. After biliary drainage, a subtotal stomach-preserving pancreaticoduodenectomy was performed. Histopathological examination revealed that the tumor components were composed of circular-to-oval atypical cells admixed with tubular adenocarcinoma tissue. These atypical cells were immunohistochemically positive for synaptophysin and diagnosed as neuroendocrine carcinoma with a Ki-67 labeling index of 63%. The patient was diagnosed with MANEC with a neuroendocrine carcinoma component of approximately 40%. The neuroendocrine carcinoma component had metastasized to the posterior pancreatic lymph nodes. Despite starting adjuvant chemotherapy with S-1, computed tomography revealed the presence of multiple liver metastases within 4 months after surgery. MANEC with neuroendocrine carcinoma is well known to have an extremely poor prognosis. Therefore, establishing a multidisciplinary therapy including chemotherapy is crucial.

An endocrine cell carcinoma with gastric-and-intestinal mixed phenotype adenocarcinoma component in the stomach.

A 77-year-old man complained of bodyweight loss, and a Borrmann 3 type lesion was observed endoscopically in the anterior wall of angular region of the stomach. The endocrine cell carcinoma (ECC) having the cytoplasmic staining of chromogranin A (CgA) was detected pathologically in the biopsy samples. The patient underwent distal gastrectomy plus systemic lymph node (LN) dissection (D2 LN dissection), and pathological examination revealed ECC invading the subserosa, and no LN metastasis (pT2N0M0). None of the gastric and intestinal endocrine cell marker expression was apparent in the ECC cells. The lesion also contained a moderately differentiated type tubular adenocarcinoma component, which was judged to be gastric-and-intestinal mixed (GI type) phenotype, using gastric and intestinal exocrine cell markers. After the surgery, he left the hospital and started oral doxifluridine (600 mg/day). The patient now (March 2008, about 19 months since the surgery) continues this chemotherapy with no recurrence. In conclusion, we experienced ECC with a GI type adenocarcinoma component. The ECC cases with the GI type adenocarcinoma component may have a relatively good prognosis, being similar to the results of advanced gastric cancers from the viewpoint of gastric and intestinal phenotypic expression.

Nonhepatocytic malignant mixed tumor of the liver in adults: report of a long surviving case.

A primary nonhepatocytic malignant mixed tumor in the liver contains both epithelial and mesenchymal components, and the incidence in adults is extremely rare. A 45-year-old female was admitted because of abdominal fullness. Abdominal imaging studies revealed a huge cystic tumor with a mural nodule in the right lobe. A right trisegmentectomy and an invaded partial diaphragm resection were performed. Diagnosis was established after surgery. The patient is still alive 11 years after surgery, and to our knowledge is the longest surviving patient with a primary nonhepatocytic malignant mixed primary tumor of the liver.