Susumu Matsukuma

Gastric stump carcinosarcoma with rhabdomyosarcomatous differentiation

Gastric carclnosarcoma is an unusual tumor and its occurrence in the gastric stump Is extremely rare. A report is presented here of a unique case of gastric stump carcinosarcoma with rhabdomyosarcomatous differentiation In a 74‐year‐old man. The patient had undergone partial gastrectomy with gastrojejunostomy (Billroth II method) 30 years previously. The tumor had both adenocarcinoma and sarcoma components, and an immunohistochemlcal study suggested a focal transition between these components. The main sarcomatous components showed fibrosarcomatous features with a scattered distribution of rounded tumor cells, whose rhabdomyosarcomatous differentiation was lmmuno‐histochemlcaily determined. Ultrastructural examination supported the rhabdomyosarcomatous natures. Experience with the present tumor indicates that carclnosarcoma with rhabdomyosarcomatous differentiation can occur in the gastric stump and that this disease Is capable of aggressive behavior.

Localized malignant peritoneal mesothelioma containing rhabdoid cells

A case Is presented of localized malignant peritoneal mesothelioma appearing as a liver neoplasm. The patient underwent tumor resection but developed a recurrent growth and died 10 months after the Initial surgery. The primary tumor showed sarcomatous features with rhabdoid cells. Examination revealed the presence of hyaluronic acid, co‐Immunoreactivlty for cytokeratln epithelial membrane antigen and vlmentln, cellular contacts with small desmosomes, and intracytoplasmic lumina. These findings supported the mesothelial nature of this tumor. The recurrent tumor was composed of predominantly tubulopaplllary proliferation. It was concluded that the present tumor was a localized malignant mesothelioma of a blphasic type showing a predominantly sarcomatous component in the primary tumor.

Paget sarcoma of the cervical vertebrae: An autopsy case report and review of the literature

A 69 year old Japanese woman was hospitalized for emergency treatment of sudden onset of tetraplegia and somnolence. The patient had a long history of occipital pain without definite diagnosis. After admission, the patient progressively developed generalized palsy including respiratory paralysis, and died of bronchopneumonia. Autopsy revealed osteosarcoma of the cervical vertebrae with the features of Pagets disease involving the skull and the cervical vertebrae. Paget sarcoma is rare in Japan, where Pagets disease of the bone is an uncommon condition. A review of the world literature failed to reveal any reports describing Paget sarcoma of the cervical vertebrae. The present report indicates that the development of Paget sarcoma in the upper cervical vertebrae may cause life‐threatening neurologic complications.

Peritoneal seeding of hepatocellular carcinoma: clinicopathological characteristics of 17 autopsy cases.

Peritoneal seeding (PS) of hepatocellular carcinoma (HCC) is uncommon, and the clinicopathological features are poorly understood. A total of 181 autopsy cases of HCC, including 171 cases with detailed clinical information, was investigated for PS and evaluated. PS was identified in 17 cases (9.4%), and was locally (70.6%) or entirely (29.4%) distributed in the peritoneal cavity, involving the diaphragm (76.5%), omentum (47.1%), or alimentary tract serosa (47.1%). Compared with primary HCC, PS showed similar or slightly undifferentiated features (88.2%) and exhibited more differentiated features (11.8%). In 15 cases (88.2%) of HCC with PS, primary HCC showed membranous β‐catenin immunoreactivity. However, in five cases (33.3%), respective PS lost this immunoreactivity. PS was significantly associated with rupture of HCC (P= 0.012), direct diaphragmatic invasion (P= 0.001), and lymph node metastasis (P < 0.001), indicating these are high risk factors for PS; there was no significant association with a past history of percutaneous fine‐needle biopsy, percutaneous ethanol injection and/or radiofrequency ablation (P= 0.97), or metastasis to lung (P= 0.13), bone (P= 0.71), or adrenal gland (P= 0.79). PS can infrequently proliferate aggressively with more differentiated features. Loss of membranous β‐catenin expression may be associated with PS of HCC.

Aberrant cytokeratin 7 expression of centrilobular hepatocytes: a clinicopathological study

Matsukuma S, Takeo H, Kono T, Nagata Y & Sato K 
(2012) Histopathology 61, 857–862

Primary pulmonary myxoid sarcoma with EWSR1‐CREB1 fusion, resembling extraskeletal myxoid chondrosarcoma: Case report with a review of Literature

Reported herein is an extremely rare case of primary pulmonary myxoid sarcoma (PPMS). A 31‐year‐old man presented with a 2.7 cm‐sized pulmonary tumor surrounded by capsule‐like fibrosis. The patient has been free of disease for 5.8 years after surgery. This tumor focally showed endobronchial features, and consisted of reticular cords of oval, short spindle, or polygonal cells with swollen vesicular nuclei accompanied by an abundant myxoid stroma, closely resembling extraskeletal myxoid chondrosarcoma. Tumor cells were diffusely positive for vimentin and focally positive for epithelial membrane antigen, but were negative for cytokeratin, TTF‐1, Napsin A, S‐100 protein, CD34, desmin, smooth‐muscle actin, CD10, p63, calponin, h‐caldesmon, c‐kit, HMB‐45, synaptophysin, or glial fibrillary acid protein. Our reverse transcription‐polymerase chain reaction using the formalin‐fixed, paraffin‐embedded tumor tissues detected EWSR1‐CREB1 fusion transcript, but could not demonstrate EWSR1‐ATF1 fusion or EWSR1/TAF15/TFG‐NR4A3 fusion. These findings indicate that the current tumor is an additional case of PPMS with EESR1‐CREB1 fusion, recently reported by Thway et al. Some cases of PPMS can behave in an indolent manner.

Numerous eosinophilic globules (skeinoid fibers) in a duodenal stromal tumor : An exceptional case showing smooth muscle differentiation

A unique case of duodenal stromal tumor In a 51‐year‐old man is reported. The tumor histologically showed spindle cell proliferation and numerous eosinophilic globules. Most globules were composed of tangled 45 nm thick fibrils, which were ultrastructurally Identical to ‘skelnoid fibers’. The presence of glycogen granules in the tumor cells and the Immunoreactivity for α‐smooth muscle actin suggested smooth muscle differentiation. Focal ultrastructural findings also supported the smooth muscle nature of this tumor. There were no immunohistochemical and ultra‐structural features indicating neural differentiation. In previous studies, the presence of such ‘skeinoid fibers’ was suggested to be a histological marker for neural differentiation in gastrointestinal stromal tumor. However, the findings In the present case suggest that numerous ‘skeinoid fibers’ can be Identified in duodenal stromal tumor with smooth muscle differentiation, although this condition may be rare.

Large cystic uterine adenomyoma showing marked epithelial metaplasia and exophytic polypoid growth

Case reportThis report describes an unusual case of large cystic uterine adenomyoma in a 48-year-old woman. Under the clinical diagnosis of ovarian cancer, she underwent hysterectomy with bilateral salpingo-oophorectomy. Macroscopically, the large cystic mass containing bloody material was connected with the uterine isthmus and separate from both ovaries. Histological examination showed various epithelial metaplasia lining the cystic surface and revealed focal endometrial stroma in the cystic wall.ConclusionsHence, cystic adenomyoma showing exophytic polypoid growth is suggested, although its occurrence is extremely rare. The present case emphasizes that cystic adenomyoma showing extensive metaplastic changes and exophytic growth can occur.

Paget's Disease of the Esophagus A Case Report with Review of the Literature

A 59-year-old Japanese man with Pagets disease of the esophagus is reported. The epithelium in the upper-to-middle portion of the esophagus was extensively infiltrated by Pagets cells. There was a minimal submucosal invasive area showing features of undifferentiated carcinoma. Histochemical and immunohistochemical studies showed glandular differentiation of the intraepithelial Pagets cells. Electron microscopic study demonstrated distinctive intraepithelial growth of the Pagets cells with the formation of desmosomes with nonneoplastic squamous cells, but there was no definitive evidence of glandular differentiation of the Pagets cells. These findings suggest that the entity in this case was probably adenocarcinoma in situ originating in the components of the esophageal epithelium, such as the intraepithelial or other portions of the esophageal gland ducts, with focal invasion and anaplasia. The additional control study of 154 cases of esophageal carcinoma revealed two other cases showing localized pagetoid extensions.

Metastatic cancer involving pancreatic duct epithelium and its mimicry of primary pancreatic cancer.

We investigated 47 autopsy cases of metastatic cancer involving the pancreas. Metastatic disease in nine cases involved the pancreatic duct epithelium. In two cases, metastatic cancer cells showed Pagetoid features. In three cases, pancreatic metastatic disease showed solitary proliferation with focal in situ carcinoma‐like lesions mimicking primary pancreatic cancers. Each of these three cases had primary lung adenocarcinomas. Serial sections revealed abrupt borders between the in situ carcinoma‐like lesions and the non‐cancerous epithelium. Primary pancreatic cancers did not show Pagetoid features or abrupt borders between the cancerous and non‐cancerous epithelium. We conclude that the possible diagnosis of pancreatic metastasis should be carefully ruled out in the histological detection of latent primary pancreatic cancer.