Poorly differentiated angiosarcoma without vasoformative channels but with focal intracytoplastic vacuoles mimicking liposarcomas

AbstractAngiosarcoma (AS) showed diverse morphologies from well formed malignant vasculatures to poorly differentiated tumor with only a few clues of endothelial differentiation. Herein reported are two cases of AS without primitive vasoformative channels (VC). They showed, instead, a very few foci of intracytoplasmic vacuoles (ICV) that mimicked liposarcoma. The two cases were found in 12 cases of AS in computer database. Both are men, 57 and 68years. One is cutaneous (foot) AS and another is soft tissue (thigh) AS. The largest diameter of cutaneous AS was 5cm, and that of soft tissue AS 9cm. The prognosis of both patients was poor; both died of metastases 4 and 6years after initial presentation. In both cases, hematoxylin and eosin (HE) diagnosis was difficult because there were no VC, and most of the tumors were composed of primitive mesenchymal tissues. In both cases, however, a few very tiny foci consisting of ICV were seen. At first, the author considered them as mucins or fat, and suspected liposarcoma. In fact, they were pseudolipoblasts. Several mucin stains showed no mucins, and fat stains of frozen sections of formalin fixed tissue were negative for fat. Immunohistochemically, the vacuoles were positive for factor VIII-related antigen (F-VIII-RA), Ulex lectin, CD31, CD34, vimentin, p53 and Ki-67 (labeling index=64% and 75%), but negative for various types of cytokeratins (CK), EMA, CEA, CA19-9, CD45, smooth muscle actins, S100 protein, myoglobin, HMB-45, Melan A, NCAM, and NSE. F-VIII-RA is specific and Ulex lectin and CD31 are relatively specific for endothelium. Therefore, the pathological diagnosis of AS could be made by the combined histologic features (ICV) and Immunohistochemical positivity of F-VIII-RA, Ulex lectin, and CD31. Thus, it appeared that the ICV may be the only clue of poorly differentiated or undifferentiated AS. In such undifferentiated cases, combined observations of meticulous histologic observations (intracytoplasmic lumens and ICV) immunohistochemistry of F-VIII-RA, Ulex lectin, and CD31 may be helpful in the diagnosis of poorly differentiated and undifferentiated AS. Electron microscopic observations were not done in the present study

Adenosquamous carcinoma of the ovary arising from endometriosis: two case reports

The author reports two cases of adenosquamous carcinoma arising from endometriosis of ovaries. The tumor patients were 38-year-old and 53-year-old women. Both patients underwent hysterectomy and bilateral salpingo-oophorectomy for ovarian carcinomas. Grossly, both ovarian tumors were located in the left ovaries, and were cystic tumors with mural tumors. Histologically, the cystic areas consisted of endometrial glandular epithelium. Both mural tumors were composed of grade I endometroid adenocarcinoma and squamous cell carcinoma. These two elements were admixed in some areas. A differentiation of endometrioid adenocarcinoma from the endometriosis were present in a few areas. Likewise, a differentiation of squamous cell carcinoma from the endometriosis were recognized in several areas. The pathologic diagnosis was adenosquamous carcinoma arising from endometriosis of the ovary in both cases, rather than endometrioid adenocarcinoma with malignant squamous differentiation. No tumors were present in other organs

Adenosquamous Carcinoma and Pure Squamous Cell Carcinoma of the Pancreas: Report of two Cases

Adenosquamous carcinoma (ASC) and pure squamous cell carcinoma (SCC) of the pancreas are very rare diseases. The author herein reports two cases of ASC and SCC of the pancreas. The first case is ASC. An 80-year-old woman was admitted to our hospital because of abdominal pain and weakness. Imaging modalities including CT, MRI and ERCP revealed a pancreatic body tumor. Distal partial resection of the pancreas and splenectomy were performed. Grossly, an infiltrative solid tumor measuring 3 × 4 × 4 cm was present in the pancreatic body. Histologically, it was an ASC consisting of poorly differentiated adenocarcinoma element (20% in area) and SCC element (80%). There was a gradual transition between the two. Many perineural invasions and lymphovascular permeations were recognized. The patient died of systemic metastasis five months after operation. The second case is an SCC. A 69-year-old woman presented with abdominal pain and jaundice. Imaging modalities including CT, MRI and ERCP revealed a tumor in the head of the pancreas. Pancreaticoduodenectomy was performed. Grossly, an infiltrative solid tumor measuring 5 × 5 × 6 cm was present. Histologically, the tumor was pure SCC. The SCC was moderately differentiated SCC. A large number of perineural invasions and lymphovascular permeations were present. The patient died of systemic metastasis three months after operation. The author speculates that ASC of the pancreas may be derived from squamous tansdifferentiation of adenocarcinoma element or from pluripotent stem cells, and that SCC of the pancreas may arise from malignant transformation of squamous metaplasia of pancreatic ducts or from pluripotent stem cells

Spindle Cell Carcinoma of the Common Bile Duct: Case Report with Immunohistochemical Analysis

The author reports a very rare case of spindle cell carcinoma of the common bile duct with an emphasis on immunohistochemical features. A 65-year-old man was admitted to our hospital because of jaundice. Imaging modalities revealed a tumor of the common bile duct, and bile cytology demonstrated malignant cells. A pancreatico-duodenectomy was performed. Grossly, an elevated tumor (15 × 10 × 3 mm) was present in the middle portion of the common bile duct. Microscopically, the tumor consisted of well-differentiated adenocarcinoma (20% in area) and spindle cell carcinoma (80% in area). There were gradual transitions between the two. The tumor cells invaded into the serosa. Immunohistochemically, the spindle cell carcinoma element was positive for four types of pancytokeratins, cytokeratin 7 (CK7), CK8, CK18, CK19, CK20, epithelial membrane antigen, vimentin, p53 protein, Ki-67 (labeling = 52%), and CEA. It was negative for high-molecular-weight CK, CK5/6, CK14, p63, neuron-specific enolase chromogranin, synaptophysin, CD56, CA19-9, CD34, desmin, S100 protein, myoglobin, a-smooth muscle antigen, CD34, CD68 and KIT. The adenocarcinoma element showed similar immunoreactivity except for negative vimentin, and positive CA19-9. The present case is the first report of spindle cell carcinoma of the common bile duct demonstrating an extensive immunohistochemistry. The spindle cell carcinoma in the present case may be derived from sarcomatous transformation of the adenocarcinoma element. CK20 newly emerges in the development of spindle cell carcinoma of the common bile duct

Occult very small lung carcinoma with a solitary brain metastasis that is clinically diagnosed as cavernous hemangioma: a case report

The author reports herein a case of occult very small lung carcinoma with a solitary brain metastasis that is clinically diagnosed as cavernous hemangioma, with an emphasis on pathologic findings. A 48-year-old Japanese man was admitted to our hospital complaining of mild paresis of left leg. Brain CT and MRI showed a solitary tumor (2 cm) with features of cavernous hemangioma in the right temporal lobe. Tumorectomy was performed, and it was pathologically undifferentiated carcinoma. An immunohistochemical analysis reveled that the carcinoma cells were positive for four types of pancytokeratin, cytokeratin (CK) 5/6, CK7, CK18, CK19, p63, and Ki-67 (78%). They were negative for high molecular weight CK, CK14, CK20, TTF-1, PE-10, melanosome, S100 protein, EMA, vimentin, CD34, myoglobin, CEA, p53, desmin, α-smooth muscle actin, chromogranin, synaptophysin, CD56, neuron-specific enolase, CD68, KIT, and PDGFRA. The positive CK7 and negative CK20 suggested lung origin, and cytokeratin profiles and positive CK5/6 and p63 suggested a squamous differentiation. The pathological diagnosis was undifferentiated carcinoma with squamous differentiation probably of lung origin. Later, systemic CT, MRI and PET were performed, and they detected a small lung tumor (8 mm) in the right apex. The lung biopsy revealed an undifferentiated carcinoma with focal squamous differentiation; the immunohistochemical findings were the same as those of the brain tumor. These findings suggest that occult very small lung carcinoma can metastasize to brain and such a metastasis may mimic cavernous hemangioma radiologically. Pathologic observations using many antibodies are very useful to determine the origin and histological type in solitary brain nodule

Heterotopic Pancreatic Tissue of the Stomach: Report of Three Cases and Consideration of Its Histogenesis

Heterotopic pancreatic tissue of the stomach is a rare condition. The author herein reports three cases of heterotopic pancreatic of the stomach. The three cases were found in the 8,154 gastric specimens (biopsy 6,122 cases, gastrectomy 2,032 cases) of our pathology laboratory; the frequency was 0.04%. The first case was a 57-year-old woman who presented with epigastralgia. Endoscopic examination revealed a submucosal tumor in the body of the stomach. Partial gastrectomy was performed. Histologically, the lesion measured 25 × 20 × 25 mm and was submucosal heterotopic pancreatic tissue with hemorrhage and cystic changes. It was composed of acinar cells, ducts, and islets (Heinrich type I). The second case was a 57-year-old man without symptoms. Endoscopic examination revealed a submucosal tumor, and biopsy was taken. The biopsy showed heterotopic pancreatic tissue in the mucosa. It consisted of acinar cells and ducts (Heinrich type II). The third case was a 54-year-old woman without symptoms. Endoscopic examination showed chronic gastritis, and biopsy was obtained. The biopsy revealed heterotopic pancreatic tissue in the mucosa. It consisted of acinar cells and ducts (Heinrich type II). The first case appears to be a true heterotopic pancreas of congenital abnormality. The second and third cases seem to be heterotopic pancreatic tissues of congenital anomaly of the gastric mucosa. The histogenesis of heterotopic pancreatic tissue is discussed

Brain metastasis from thyroid adenomatous nodules or an encapsulated thyroid follicular tumor without capsular and vascular invasion: a case report

Because benign-appearing thyroid nodules with metastasis are very rare, the author reports herein four thyroid nodules (one follicular adenoma and three adenomatous nodules) with brain metastasis. A 75-year-old Japanese woman was admitted to our hospital because of thyroid mass. Imaging modalities revealed four distinct nodules in the thyroid, and tumorectomies of all nodules were performed under the clinical diagnosis of benign thyroid nodules. Grossly, one of them was a completely encapsulated tumor (35 mm in diameter). Other three nodules were non-encapsulated nodules (10 mm, 8 mm, and 7 mm in diameters). Multiple sections were obtained from the largest nodule. One section was obtained from each of the smaller three nodules. Microscopically, the largest tumor was an encapsulated follicular adenoma. The tumor consisted of normofollicles and microfollicles surrounded by a fibrous capsule. Neither capsular invasion nor vascular permeation was recognized. The parenchyma lacked nuclear atypia, mitotic figures, degenerative changes, papillary structures, nuclear inclusions, nuclear ground-glass features, and nuclear grooves. Thus, the largest tumor was diagnosed as follicular thyroid adenoma. The remaining three small nodules were typical adenomatous nodules composed of normofollicles and macrofollicles without nuclear atypia, mitotic figures, degenerative changes, papillary structures, nuclear inclusions, nuclear ground-glass features, and nuclear grooves. Therefore, a diagnosis of adenomatous nodules (goiters) was made. However, six years later, the patient showed a brain metastasis of thyroid tumor composed of macrofollicles without cellular and nuclear atypia. A diagnosis of metastatic follicular thyroid carcinoma was made. The present case suggests that benign thyroid nodules can metastasize

Malignant melanoma of the nasal cavity: a case report with examination of KIT and platelet derived growth factor receptor-α(PDGFRA)

Although several clinicopathological studies of malignant melanoma of the nasal cavity have been reported, there are no studies of the expression and gene mutation of KIT and platelet derived growth factor receptor-α (PDGFRA) in melanoma of the nasal cavity. A 92-year-old Japanese woman consulted to our hospital because of right nasal obstruction and epistaxis. Physical examination and imaging modalities showed a tumor of the right nasal cavity. A biopsy was taken, and it showed malignant epithelioid cells with melanin deposition. Immunohistochemically, the tumor was positive for S100 protein, HMB45, p53, Ki-67 (labeling=20%), KIT and PDGFRA. The tumor was negative for cytokeratins (AE1/3 and CAM5.2). A genetic analysis using PCR-direct sequencing revealed no mutation of KIT gene (exons 9, 11, 13, and 17) or the PDGFRA gene (exons 12 and 18). The pathological diagnosis was primary malignant melanoma of the nasal cavity. The tumor was reduced in size by local resection and chemotherapy (Darthmose regimen: dacarbazine, carmustine, cisplatine, and tamoxifen), and the patient is now alive and free from metastasis 9 months after the first manifestation. In conclusion, the author reported a case of melanoma of the nasal cavity expressing KIT and PDGFRA without gene mutations of KIT and PDGFRA

Cytokeratin-negative small cell lung carcinoma

Cytokeratins (CK) are good markers of epithelial tumors, and most of carcinomas including small cell lung carcinoma (SCLC) expresses CK. Herein reported is a case of SCLC without CK expression

Myopericytoma of low grade malignancy in the oral cavity

Myopericytoma (MPC) of the oral cavity is extremely rare. Herein reported is a case of MPC of low grade malignancy in the oral cavity. A 61-year-old man noticed a tumor of the cheek mucosa, and admitted to our hospital. Oral examination revealed a reddish elevated tumor of the cheek mucosa. Tumorectomy with wide margins was performed. The clinical diagnosis was pyogenic granuloma. Grossly, the tumor was reddish, and measured 1×1×1 cm. Microscopically, oval to spindle tumor cells with hyperchromatic vesicular nuclei and many vasculatures were seen. The tumor cells were contiguous and mixed with endothelial cells in many blood vessels, thus resembling pericytes. Mitotic figures were scattered. The surgical margins were negative for tumor cells. Immunohistochemically, the tumor cells were positive for vimentin, α-smooth muscle actin and p53. The Ki67 labeling was 40%. The tumor cells were negative for cytokeratins (AE1/3 and CAM5.2), CD31, CD34, S100 protein, HMB45, CD10, vimentin, desmin, and factor VIII-related antigen. The endothelium of the vessels were positive for vimentin, CD31, CD34 and factor VIII-related antigen, but negative for α-smooth muscle actin, p53, cytokeratins (AE1/3 and CAM5.2), S100 protein, HMB45, CD10, vimentin, and desmin. The Ki67 labeling was 5%. Because the pericytoid tumor cells showed α-smooth muscle actin and negative for endothelial markers, MPC was diagnosed. In addition, because there was some atypia and mitotic figures were scatters and also because the tumor cells were positive for p53 and Ki67 labeling was high, a pathological diagnosis of MPC with low grade malignancy was made. No recurrence was observed, and the patient is now free from tumor 6 months after the operation