Duodenal gangliocytic paraganglioma with lymph node metastasis: a case report and review of the literature

A case of duodenal gangliocytic paraganglioma (DGP) in a 67-year-old woman is presented. The DGP arose in the second part of the duodenum. Although most of the reported cases of DGP are considered benign, in the present case, we found regional lymph nodes containing metastatic tumor. Previous reports have documented metastases containing only epithelioid cells. The current case demonstrates metastatic tumor in regional lymph nodes containing all 3 of the DGP components (spindle cells, ganglion-like cells, and epithelioid cells).     

Endoscopic ultrasound-guided fine-needle aspiration of undifferentiated carcinoma with osteoclast-like giant cells of the pancreas: a report of 2 cases with literature review

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas is rare. Histologically it mimics the giant cell tumor of the bone and may be associated with a ductal adenocarcinoma. We recently encountered two such cases, both of which were biopsied by EUS-guided FNA. Abundant multinucleated osteoclast-like giant cells and many uniform mononuclear cells were present in case 1 so that the diagnosis was made. In case 2, many mononuclear tumor cells with vacuolated and basophilic cytoplasm were present, and rare osteoclast-like giant cells were seen. A diagnosis of adenocarcinoma was made. In both cases, no conspicuous nuclear pleomorphism was noted in the mononuclear cells or the multinucleated giant cells. The histology of case 2 revealed a pure undifferentiated carcinoma with osteoclast-like giant cells. In addition, a liver biopsy revealed globular amyloidosis. To our knowledge, this is the first report of pancreatic undifferentiated carcinoma with osteoclast-like giant cells sampled by EUS-guided FNA and the first case of hepatic globular amyloidosis associated with this tumor.     

Childhood Ki-1 lymphoma. A report of two cases

Two cases of childhood Ki-1 lymphoma occurred with the expression of Ki-1+/HLA-DR+/IL-2R+/EMA+/Leu-M1-/pan-T antigens-/pan-B antigens- in neoplastic cells. Patient 1 with nodular skin lesions expressed Leu-2a+ in the neoplastic cells and died 14 months later. Patient 2 with lymph node swelling and hepatosplenomegaly exhibited Leu-3a+ in the neoplastic cells and remains free of disease. The Leu-2a+ (case 1) or Leu-3a+ (case 2) findings suggest that the neoplastic cells in both cases were derived from T-lymphocyte lineage. However, Southern's blot analysis did not reveal any clonal rearrangements of T-cell receptor genes in the autopsy material from case 1. The Leu-3a+/Mcs-2+ finding in case 2 may indicate that the neoplastic cells were derived from monocyte/macrophage lineage.     

Malignant giant cell tumor of synovium and locally destructive pigmented villonodular synovitis: ultrastructural and immunohistochemical study and review of the literature

The first reported case of an intraarticular malignant giant cell tumor of synovium studied with electron microscopic and immunohistochemical examination is presented, together with a case of diffuse intraarticular pigmented villonodular synovitis with extensive bone destruction. The malignant case was dominated by uniform cells positive for histiocytic markers, the fine structure showing a gradual change from cells dominated by organelles serving a secretory function to cells with phagocytic activity. The reported cases of giant cell tumor of the tendon sheath indicate that the pertinent histologic changes regarding malignancy are an increase in cell polymorphism and in the number of mitoses, and a decrease in the number of multinucleated giant cells.     

The increase in the number of liver sinusoidal pit cells in four patients with primary or metastatic cancer of the liver

Four patients with liver carcinoma (case 1: hepatocellular carcinoma; cases 2 and 3: metastases; case 4: adenocarcinoma possibly of hepatic origin) underwent a wedge liver biopsy taken at some distance from the tumor. Liver histology was normal in cases 2 and 3. Sinusoids were dilated in case 4. Fibrosis formed bridges between portal tracts in case 1. In all 4 cases, sinusoids contained lymphocytic cells. By electron microscopy (perfusion-fixation with glutaraldehyde) numerous lymphocytes could be identified as pit cells with characteristic dense granules and occasional rod-cored vesicles. The majority of the pit cells were luminal cells in contact with endothelial or Kupffer cells; some were in the Disse space. It is now accepted that pit cells are resident large granular lymphocytes with natural killer activity. The increase in the number of pit cells in liver carcinoma compared to the number observed in the control group (uncomplicated gallbladder lithiasis) could be hypothetically interpreted as a defense mechanism against tumor extension.     

Fine-needle aspiration cytology of benign and malignant adenomyoepithelioma: report of two cases.

Cytological features of a malignant spindle-cell variant and a benign tubular variant of adenomyoepithelioma were examined to improve diagnosis of this tumor. Fine-needle aspiration cytology, of both a malignant and a benign case, characterized cellular and cohesive cell clusters composed of epithelial and myoepithelial cells. The smears of the malignant case were rich in spindle-shaped myoepithelial cells, admixed with a few epithelial cells. In about a fourth of the clusters, atypical cells with enlarged hyperchromatic nuclei and prominent nucleoli comprised more than 20% of cells. The smears of the benign case were composed of tubular epithelial cells surrounding one to several layers of myoepithelial cells with clear cytoplasm. Mild atypia was occasionally noticed. A review of the literature showed that a cytological diagnosis of malignancy is not warranted, if nuclear atypia is not generally severe. Focal severe atypia is not definitively indicative of benignity or malignancy.     

Ovarian granulosa cell proliferations of pregnancy: a report of nine cases

Granulosa cell proliferations that simulated small neoplasms were incidental findings in the ovaries of nine pregnant women. The lesions were typically multiple and associated with atretic follicles. In eight cases, the granulosa cells were arranged in solid, insular, microfollicular, and trabecular patterns, but in the ninth case, there was a prominent sertoliform tubular pattern. The granulosa cells typically contained scanty cytoplasm and grooved nuclei, resembling the cells of adult-type granulosa cell tumors. However, in one case large nodules of markedly luteinized cells with variably sized, round, nongrooved nuclei were present; the nodules in this case superficially resembled pregnancy luteomas. In the case with a sertoliform pattern, the cells had abundant vacuolated cytoplasm. The lesions described probably reflect an unusual physiological response to the elevated chorionic gonadotropin level of pregnancy rather than early stages of neoplasia.     

Giant cell fibroma of the oral mucosa. Report of a case with ultrastructural study

A case of giant cell fibroma of the oral mucosa found in a 3-year-old female is reported. The lesion was characterized histologically by the presence of numerous large stellate cells and multinucleated giant cells scattered in a loosely arranged collagen-background. The large stellate cells had a large hyperchromatic nucleus, while the cytoplasm was well demarcated and frequently the cells had dendritic-like processes. The multinucleated giant cells had similar morphology and occasionally resembled Langhans' giant cells. Ultrastructural examination suggested that the multinucleated giant cells in the lesion were unusual fibroblasts. This is the first reported case of oral giant cell fibroma in a Japanese patient.     

ELECTRON MICROSCOPIC OBSERVATION OF EWING'S SARCOMA —A CASE REPORT—

Ewing's sarcoma originating from the 2nd lumbar vertebra, found in an autopsy case of a 12-year-old girl was investigated histologically and electron microscopically. The distinguished features of neoplastic cells of this case were phagocytosis, desmosomes, and abundant glycogen in their cytoplasm. The origin of neoplastic cells is assumed to be immature reticulum cells.     

Thymic epithelial cells expressed unusual follicular dendritic cell markers: thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue

Described herein is a case of thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Using immunohistochemical double staining it was found that most of the thymic lymphoid follicles in this case possessed cytokeratin-positive and follicular dendritic cell (FDC) marker-positive cells. Moreover, using immunoelectron microscopy it was confirmed that some of the double-positive cells were thymic epithelial cells. The candidate of cytokeratin subtype expressed on the double-positive cells was cytokeratin 1 (CK1), which was expressed only by the epithelium of Hassall's corpuscles in thymuses from age-matched patients with myasthenia gravis. The present case indicates a possibility that some thymic epithelial cells become FDC, although it was uncertain whether they were derived from the epithelia of Hassall's corpuscles or whether they were at the same differentiation stage as Hassall's corpuscles.     

Mixed mesodermal tumor of the ovary: Immunohistochemical study with histogenetic consideration

The clinical, histological and immunohistochemical features of three cases of ovarian mixed mesodermal tumor (MMT) were examined. The epithelial component was serous papillary cystadenocarclnoma in case 1 and 3, and endometrioid adenocarcinoma in case 2. In case 1, undifferentiated adenocarcinoma was also seen. The mesenchymal component was fibrosarcomatous and chondrosarcomatous in case 1 and 2. In case 3, only fibrosarcomatous area was seen. No endometriosis was observed. Immunohistochemically, the epithelial component showed positivity for epithelial membrane antigen in all three cases. S-100 protein was positive in two cases with chondrosarcomatous differentiation. The fibrosarcomatous area showed positivity for vimentin in all three cases. However desmin, myosin and myoglobin were negative. The antibodies thought to be epithelial or mesenchymal markers unexpectedly reacted positively in some cells; for example, EMA was positive in fibrosarcomatous and chondrosarcomatous cells. Therefore, it was speculated that because the undifferentiated tumor cells had a biphasic character, MMT might originate from immature multipotential cells of surface epithelium and subcapsular connective tissue of the ovary.     

GIANT CELL FIBROMA OF THE ORAL MUCOSA: Report of a Case with ultrastructural Study

A case of giant cell fibroma of the oral mucosa found in a 3-year-old female is reported. The lesion was characterized histologically by the presence of numerous large stellate cells and multinucleated giant cells scattered in a loosely arranged collagen-background. The large stellate cells had a large hyperchromatic nucleus, while the cytoplasm was well demarcated and frequently the cells had dendritic-like processes. The multinucleated giant cells had similar morphology and occasionally resembled Langhans giant cells. Ultrastructural examination suggested that the multinucleated giant cells in the lesion were unusual fibroblasts. This is the first reported case of oral giant cell fibroma in a Japanese patient. ACTA PATHOL. JPN. 36: 1571-1576,1986.     

The lymph node in mycosis fungoides: a light and electron microscopy and immunohistological study supporting the Langerhans'' cell-retrovirus hypothesis

This paper describes the light and electron microscopy and monoclonal antibody findings in the lymph nodes of nine patients with mycosis fungoides. Four cases showed dermatopathic change characterized by close association between small convoluted T4 lymphocytes and T6 antigen-presenting cells (Langerhans' and indeterminate dendritic cells) in the sinuses and paracortical zones. The T4:T8 ratio was between 3 and 5:1. One case showed dermatopathic change but included large convoluted T4 lymphocytes and occasional T10 lymphocytes. The T4:T8 ratio was 4:1. The antigen-presenting cells were mostly T6 negative (interdigitating reticulum cells). One case showed extensive paracortical expansion by small and large convoluted T4 lymphocytes. The T4:T8 ratio was 20:1. Few B lymphocytes and antigen-presenting cells were present. Two cases showed partial and one case total effacement by atypical lymphoid tissue. This included small and large convoluted T4 lymphocytes and T4 immunoblasts. The T4:T8 ratios were between 30 and 40:1. The changes in their antigen-presenting cell population were complex. Langerhans' cells in one case generally failed to express T6 antigen and in two displayed histiocyte features. In one case, both immature and budding type C retrovirus-like particles were identified in Langerhans cells. One extracellular mature type C virus-like particle was identified in another case. No ultrastructural distinction could be made between similarly sized primary lysosomes and possible intracytoplasmic mature type C retrovirus particles. These findings support the hypothesis that mycosis fungoides may represent an altered dermatonodal cycle resulting from an interaction between retrovirus, Langerhans' cells and lymphocytes.     

Exfoliated `myeloma cells' in the urine of a case of multiple myelomatosis

The clinical features and special investigations in a case of multiple myelomatosis are described. The finding of exfoliated `myeloma cells' in the urine of this case is thought to be unique, these cells closely resembling the myeloma cells seen in the bone marrow. The necropsy findings indicated that the origin of the cells was from a plasma cell infiltrate of the kidneys rather than from isolated plasmacytoma of any part of the urinary tract.     

Jejunal endocrine tumour composed of somatostatin and gastrin cells and associated with duodenal ulcer disease

Summary A case of malignant endocrine tumour of the jejunum, associated with severe duodenal ulcer is described. The tumour and a local metastasis were examined by immunohistochemistry and found to contain abundant somatostatin-immunoreactive cells together with less numerous cells displaying gastrin immunoreactivity. This is to our knowledge the first case of intestinal somatostatinoma. The presence of gastrin cells in the tumour may explain the ulcer diathesis.     

A rare case of extramedullary spinal cord germinoma

A rare case of spinal cord germinoma in a 20-year-old Japanese female is presented. The tumor was detected at the level of T11 and L3 by myelography. Histological findings of the resected tumor were identical to germinoma, which is characterized by polygonal tumor cells, lymphocytic infiltrate, and granuloma. Tumor cells and lymphocytes reacted with antibodies for placental alkaline phosphatase and CD45, respectively. A few multinucleated giant cells, negative immunohistochemically for human chorionic gonadotropin were also seen. The present case is the 10th case of spinal cord germinoma and is thought to be the first case of such a tumor located extramedullary. Diagnosis of such a rare tumor in an unusual location needs proper histological, ultrastructural and immunohistochemical evaluation.     

Sclerosing stromal tumour of the ovary--a clinicopathologic spectrum

Majority of the Sclerosing stromal tumours of the ovary documented in the literature are single case reports. We report a series of 4 cases. Among the 4 cases encountered the mean age at presentation was 22.2 years. The clinical presentation varied from asymptomatic mass per abdomen (2 cases), menorrhagia (1 case) and amenorrhoea (1 case). The tumour was unilateral in all the cases with an average size of 10 cms. Grossly the appearances varied from a solid, partly cystic, edematous tumour (2 cases) to solid,firm tumour with yellow flecks (1 case) to unilocular cystic tumour (1 case). Microscopically, the tumour was characterized by cellular pseudolobules composed of a disorderly admixture of collagen-producing fibroblasts and lipid rich lutein cells with shrunken nuclei. In one case the lutein cells had a robust appearance with abundant cytoplasm and vesicular nuclei. The pseudolobules were very vascular and separated by hypocellular dense to oedematous fibrous tissue. Frozen section demonstrated fat in luteinized cells in 3 cases.     

Immunohistochemical characteristics of bone forming cells in pleomorphic adenoma

Histopathological and immunohistochemical examinations were carried out in a case of pleomorphic adenoma with bone formation, occurring in the chin of a 34-year-old Japanese man. Examination results showed the modified neoplastic myoepithelial cells reacted positively to S-100 protein. The S-100-positive modified neoplastic myoepithelial cells were proliferated in the closely related area of the bone tissue. Furthermore, positive reaction was detected in the bone forming cells: osteoblasts and osteocytes. These cells also reacted positively to Runx2 as a marker of bone forming cells. These results suggest that the origin of the bone forming cells in this case of pleomorphic adenoma was modified neoplastic myoepithelial cells.     

Dendritic cell sarcomas/tumours of the breast: report of two cases

Extranodal follicular dendritic cell sarcoma/tumours (FDCS/Ts) and interdigitating dendritic cell sarcoma/tumours (IDCS/Ts) are rare neoplasms. We present two cases of FDCS/T and IDCS/T of the breast. The FDCS/T case (case 1) presented in a 31-year-old woman and the IDCS/T case (case 2) in a 67-year-old woman who both showed a firm lump in the left breast. The FDCS/T lesion superficially appeared as an anaplastic carcinoma and the IDCS/T was reminiscent of a spindle cell sarcomatoid carcinoma. Nevertheless both lesions were negative for keratins while case 1 displayed neoplastic cells strongly positive for CD21, vimentin and focally for CD68 and S-100 protein. The tumour cells of case 2 were positive for S-100, CD68 and CD45. In breast, an unusual keratin negative tumour composed predominantly of spindle cells arranged in fascicles, storiform pattern or whorls with a lymphoid rich stroma should raise suspicion for FDCS/Ts or IDCS/Ts. The distinction from malignant tumours with similar features is discussed.