上皮样型恶性腹膜间皮瘤透明细胞亚型2例

上皮样型恶性腹膜间皮瘤透明细胞亚型是罕见肿瘤。肿瘤细胞呈乳头状、片状、腺管状排列, 肿瘤细胞圆形或多角形, 细胞膜明显、胞质透亮、细胞核偏位, 核分裂象罕见。需要和多种透明细胞肿瘤鉴别, 临床、影像、免疫组织化学有助于诊断和鉴别诊断。该文报道2例上皮样型恶性腹膜间皮瘤透明细胞亚型。     

Synchronous pleural and peritoneal malignant mesothelioma: a case report and review of literature

The coexistence of mesothelioma and other primary malignancies has been previously reported in literature, but the finding of a pleural mesothelioma with a synchronous peritoneal mesothelioma has not been reported so far. We report a case of a 58-years-old woman that came to our attention for the incidental finding of an inguinal mass. Fine-needle biopsies of the mass and a thoracoscopy with pleural biopsies were performed, after imaging studies showed pleural thickenings suspicious for malignancy. Histological morphology and growth pattern were similar in both cases. Both tumors stained for calretinin, but only the pleural mesothelioma showed positivity for Wilms-Tumor 1 antibody. We tried to demonstrate with molecular biology techniques whether they were synchronous or one was the metastasis of the other, but our studies did not give informative results. The prognosis in this case is poor, and after 6 months the patient is still following a chemotherapy regimen, which is the only practicable approach given the extent of the disease.     

Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: report and review of the literature

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor described in diverse locations including lung and liver. Relative to these sites, primary EHE of the serous cavities is uncommon. EHE in the serous cavities mimics mesothelioma and adenocarcinoma clinically, radiographically, cytologically, and histologically. EHEs have plasmacytoid epithelioid cells with cytoplasmic vacuoles. In addition to these features, we noted eccentric nuclei with abundant eosinophilic cytoplasm and nuclei displaced peripherally by globular cytoplasmic inclusions imparting a "rhabdoid" phenotype. These cells were often seen surrounding a hyaline core. Rhabdoid features are not unique to a single entity, and a comprehensive immunohistochemical panel is essential. We report the occurrence of pleural EHE with rhabdoid features presenting in a pleural effusion, and review the literature of primary serosal EHEs.     

Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour of intermediate behaviour. It can arise from various sites including the liver, spleen, pleura, or lung. Cutaneous EHE can be primary or secondary. This report describes the case of a 51 year old man who presented with a history of dry cough, shortness of breath, and pleural effusion, and who developed two cutaneous nodules in the anterior abdominal wall a few weeks later. He had a previous history of asbestos exposure. Computed tomography scan showed a left sided pleural effusion and nodular pleural mass. Histology of both the pleural and cutaneous lesions was compatible with EHE. Electron microscopic examination demonstrated the presence of Weibel-Palade bodies. The patient underwent elliptical excision of the metastatic cutaneous nodules after decortication of the primary pleural tumour and adjuvant treatment. A few reports have described metastasis of intrathoracic EHE to the skin. Despite treatment with interferon, the patient developed more cutaneous lesions two years after the initial diagnosis. Even though the tumour has the classic light histological and ultrastructural features of EHE, it behaved in an aggressive manner.     

Primary epithelioid angiosarcoma of the pleura: a case report and review of literature

Primary angiosarcoma of the pleura is an extremely rare malignancy. Herein, we report the case of an elderly Chinese patient with primary left pleural epithelioid angiosarcoma. The 76-year-old man presented with a 4-month history of a cough with sputum expectoration and weight loss of 4 kg within one month. A chest scan showed a massive oval-shaped mass in the left pleural cavity. We then performed a left thoracotomy for tumor resection and surgical exploration. Histological examination of the resected specimen showed few viable tumor cells with significant atypia; tumor cells had large nuclei and prominent nucleoli and were arranged in a crack-like, sheeted pattern. Moreover, there was a significant amount of fibrinous exudates, hemorrhage, degeneration, and necrosis. With immunohistochemical analysis, tumor cells had strong expression of CD31, CD34, FLI-1, vimentin. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma.     

G-CSF-producing malignant pleural mesothelioma: an autopsy case report with literature review

This study reports a 54-year-old man who was a carpenter by occupation. He suffered from left chest and back pain and left pleural effusion. Peripheral blood showed granulocytosis and high serum titers of granulocyte-colony stimulating factor (G-CSF) and CYFRA. He died 20 months later. At autopsy, a pleural tumor located around the left lung and thickening of the pericardium, diaphragm, and esophagus by tumor infiltration was seen. The tumor proliferated in papillary and solid alveolar patterns by neoplastic cells. They were positive for calretinin, D2-40, CK5/6, HBME-1, G-CSF, CK19, and E-cadherin. He was diagnosed with G-CSF-producing epithelioid malignant pleural mesothelioma.     

Primary adrenal gland epithelioid sarcoma: A case report and literature review

Epithelioid sarcoma is a malignant mesenchymal neoplasm with morphologic and immunophenotypic epithelioid differentiation, which rarely arises in solid organs. We report a case of primary epithelioid sarcoma in the adrenal gland of a 31‐year‐old female. The patient initially presented with nausea and rectal bleeding, and subsequent imaging studies revealed a 4.4 cm left adrenal gland mass and left retroperitoneal lymphadenopathy. Clinical and radiological studies did not reveal tumor elsewhere in the patient. Histologic features were those of epithelioid sarcoma, proximal type with cohesive clusters of epithelioid tumor cells harboring frequent mitoses, and areas of necrosis. Immunohistochemical stains showed strong, diffuse expression of epithelial markers (pancytokeratin), and CD34 and Fli‐1. Partial and focal positive staining of CK7 was also noted. Nuclear expression of SMARCB1 (INI‐1) protein was lost. ERG was negative in this case. We believe that this is the second‐case report of a primary adrenal gland epithelioid sarcoma. Fli‐1 positivity was seen in our case, and ERG was negative as shown in some recent publications regarding epithelioid sarcomas.     

Renal epithelioid angiomyolipoma: a case report and literature review

BACKGROUND: Renal epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, closely simulating renal cell carcinoma both clinically and histologically. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported. AIM: To highlight clinicopathological features of this rare tumour. OBSERVATION: We report herein a new case of renal epithelioid angiomyolipoma in a 38-year-old male with no stigmata of tuberous sclerosis. The tumour was composed of diffuse sheets of epithelioid cells, small numbers of adipocytes and occasional blood vessels. Immunohistochemically, neoplastic cells were immunoreactive for HMB-45, but negative for cytokeratin. The patient showed no evidence of recurrence or metastatic disease one year after radical nephrectomy. CONCLUSIONS: Epithelioid angiomyolipoma may be locally aggressive and can metastasise; therefore, long-term post-operative follow-up is mandatory.     

Primary perivascular epithelioid cell tumor in the rectum: A case report and review of the literature

Perivascular epithelioid cell tumor (PEComa) is a rare collection of tumors arising in a wide array of anatomic locations. It is characterized by the presence of a peculiar population of myomelanocytic marker-positive perivascular epithelioid cells, and is commonly detected in the uterus. The colorectal area is an uncommon site for primary PEComa. In this study, we describe a 17-year-old patient presenting with a rectal polyp. Histologically, the tumor consisted of sheets of round to polygonal epithelioid cells with clear and granular cytoplasm, and a prominent capillary network. Some of the tumor cells were positive for Fontana-Masson staining. Immunohistochemically, the tumor cells were positive for HMB-45, and were negative for cytokeratin, vimentin, S-100 protein, actin, desmin, EMA, CD34, and c-kit. After finding melanosomes or premelanosomes at the ultrastructural level, the diagnosis of PEComa was made. Although PEComa arising within the intestinal tract is unusual and clinically unexpected, PEComa should be considered in the differential diagnosis of rectal polypoid lesions.     

Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.     

Perivascular epithelioid cell tumor of the pancreas: case report and literature review

Neoplasms with perivascular epithelioid cell differentiation (PEComas) of the pancreas are rare, and only 22 cases have been reported globally. Therefore, clinician and pathologist knowledge of this tumor’s biologic behavior and molecular genetics has been limited. A 40-year-old female patient presented with a space-occupying mass in the pancreas found by abdominal B-mode ultrasonography upon physical examination. Laparoscopic resection of the pancreatic body and tail was performed, and a cystic-solid tumor of about 2 × 2 cm was identified. PEComa is a type of mesenchymal tumor with uncertain biologic behavior, more frequently found in females. PEComa features a unique histomorphology and immunophenotype. We summarize the characteristics and research progress of the pancreatic PEComa, which will be convenient for physicians and pathologists to fully understand the disease to avoid misdiagnosis and to provide a reference for treatment and prognosis.     

Unusual clear cell variant of epithelioid mesothelioma.

Clear cell mesothelioma is an extremely rare neoplasm of the pleura, which can easily be mistaken for a metastasis of clear cell carcinoma to the pleura. We report here the histochemical, immunohistochemical, and ultrastructural aspects of a new case of clear cell pleural mesothelioma in a 52-year-old man with no known asbestos exposure. He was admitted to the hospital for recurrent pleural effusion, which was negative for neoplastic cells at the cytologic examination. A partial decortication of the right pleura was performed. The morphologic, immunohistochemical, and ultrastructural features reported for this case are consistent with the diagnosis of clear cell mesothelioma. The differential diagnosis and immunohistochemical features in comparison with other clear cell neoplasms are discussed.     

Primary epithelioid haemangioendothelioma of the liver: case report and review of the literature.

A 31 year old female patient presented with a one and a half year history of pain in the upper abdomen. The pain was mild, constant, dull aching, and increased with change in posture or sudden movements. There was no definite relation to meals. She also had a lump in the right upper quadrant, which had been gradually increasing in size over three months. She had mild anorexia and reported a 5 kg weight loss over one year. She had no history of intake of oral contraceptive drugs, exposure to vinyl chloride, thorotrast or any other industrial toxin. Ultrasonography of the abdomen revealed multiple space occupying lesions of altered echotexture in both lobes of the liver. The portal venous system and hepatic vascular system were normal. Computed tomography of the abdomen confirmed the ultrasound findings. Histopathology was diagnostic for primary epithelioid haemangioendothelioma; the first such case reported from India. The patient has been put on a waiting list for a liver transplant.     

硬化性上皮样纤维肉瘤临床病理特点及其诊断

目的探讨硬化性上皮样纤维肉瘤(sclerosing epithelioid fibrosarcoma,SEF)的临床病理学特征、诊断及鉴别诊断。方法对1例SEF进行组织学观察和免疫组化标记,同时复习相关文献。结果SEF主要以上皮样细胞伴大量胶原纤维为特征,瘤细胞胞质透亮,似上皮细胞,排列呈条索状,间质中大量胶原纤维发生透明变性,形成硬化性基质。肿瘤细胞vimentin阳性,cytokeratin和actin阴性。结论SEF是一种较为罕见的软组织肿瘤,应注意与一些具有上皮样形态和硬化性间质的肿瘤相鉴别。     

Primary intrahepatic malignant mesothelioma of epithelioid type

A case of epithelioid mesothelioma presenting as a primary intrahepatic tumor is described. The patient was a 62-year-old man with a 5.8-cm intrahepatic mass on an incidental intra-abdominal CT scan. Thorough clinical and radiographic examination did not reveal any evidence of tumor elsewhere. Macroscopic and microscopic examination demonstrated an intrahepatic tumor consisting of tubular and papillary proliferations of large epithelioid cells, surrounded by a densely mixed inflammatory infiltrate. The tumor cells were strongly positive for pancytokeratin, CK7, CA-12.5, and calretinin, as well as D2-40, and were faintly positive for thrombomodulin and vimentin. The proliferative rate was focally increased up to 20% by Ki-67 staining and the tumor expressed focally p53. Ultrastructurally, numerous microvilli on the cell surface, and abundant desmosomal plaques, characteristic of mesothelial cells, were found. To date, this is the third reported case of a primary intrahepatic mesothelioma.     

Coexistence of multiple myeloma and clear cell renal cell carcinoma: a case report and review of literature

Coexistence of multiple myeloma (MM) and renal cell carcinoma (RCC) is an extremely rare condition. Nevertheless, there is a higher than expected incidence of co-occurrence of these two malignancies. Several case series, in the recent past, have postulated an association between MM and RCC. Population-based data analyses have revealed a bi-directional association between these two malignancies. However, the cause still remains speculative up to date. Here, we aim to describe a patient with MM and clear cell renal cell carcinoma (CCRCC) one after another for the second time from China. Clinical implications are discussed with a critical review of existing literature and we expect to draw much more awareness among clinicians regarding such association.     

Primary pleural synovial sarcoma. A case report and review of the literature

Synovial sarcoma (SS) is an uncommon soft tissue tumor that occurs primarily in the extremities of young adults, especially in the periarticular region. In this report, we describe the rare occurrence of primary SS of the pleura in a 15-year-old boy. Histologically, the tumor demonstrated monophasic morphologic findings and showed positive staining with vimentin and Bcl-2 and focally for cytokeratin CK7. Fluorescent in situ hybridization identified t(X;18) translocation. The patient developed recurrences 20 months following resection of the tumor. The literature on this uncommon entity is reviewed, and its histogenesis, differential diagnoses, and cytologic features are also discussed.     

肝脏单形性上皮样血管平滑肌脂肪瘤

目的：探讨肝脏单形性上皮样血管平滑肌脂肪瘤的临床病理学特征及诊断、鉴别诊断要点。方法：对1例单形性上皮样血管平滑肌脂肪瘤进行临床病理学分析及免疫组织化学研究。结果：肝脏单形性上皮样血管平滑肌脂肪瘤临床多无症状,光镜下单形性上皮样血管平滑肌脂肪瘤由形态多样的上皮样细胞构成,胞质透明或嗜酸,无脂肪组织及异常血管;免疫表型;HMB45阳性,SMA及vimentin部分阳性,desmin少数阳性,S－100蛋白弱阳性,cytokeratin及AFP阴性,CD34血管内皮细胞阳性。结论：肝脏单形性上皮样血管平滑肌脂肪瘤是极为罕见的间叶性肿瘤,组织起源至今不明,其诊断及鉴别诊断主要依靠病理组织学及免疫组织化学。