Kohji Taguchi

Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von Hippel–Lindau disease

Here we report tumor‐to‐tumor metastases identified in two patients with von Hippel–Lindau (VHL) disease. The first patient had bilateral renal carcinomas and multiple cerebellar hemangioblastomas, and the second patient had a renal carcinoma and multiple hemangioblastomas in the retina, cerebellum and spinal cord. A cerebellar lesion from the first patient and a spinal lesion from the second patient contained two distinct components. The inner part of these tumors consisted of a nested mass of polygonal clear cells that expressed cytokeratin and epithelial membrane antigen, while the outer part of the tumors showed proliferation of capillaries and intervening foamy stromal cells that were negative for cytokeratin and epithelial membrane antigen. The tumors were thus considered to be hemangioblastomas complicated by metastatic lesions of renal cell carcinoma of clear cell type. These cases indicate that tumor‐to‐tumor metastasis should be considered when hemangioblastoma contains a clear cell carcinoma component in the setting of VHL disease, and that immunohistochemical staining for cytokeratin and epithelial membrane antigen is useful for the diagnosis.

Malignant Schwannoma Arising in the Intracranial Trigeminal Nerve. A Report of an Autopsy Case and a Review of the Literature

An autopsy case of malignant schwannoma arising in the intracranial trigeminal nerve is reported. The tumor involved the right cerebellopontine angle of the brain stem in an 18‐year‐old man. The spindle‐shaped tumor cells with eosinophilic cytoplasm proliferated in fascicles and exhibited hypercellularity, pleomorphisrn, increased mitotic activity and invasive growth. Ultrastructurally, inter‐digitating cytoplasmic processes and a few fragmented basal lamina‐like structures were observed. Immunohistochemically, some tumor cells were reactive with conventional anti‐S‐100 protein antibody, but negative for β subunit. Most tumor cells were positive for LY subunit of S‐100 protein. This is the eighth reported case of malignant schwannoma arising in the intracranial trigeminal nerve. Acta Pathol Jpn 40: 219‐225, 1990.

Immunohistochemical study on the distribution of α and β subunits of S-100 protein in brain tumors

SummaryThe immunohistochemical distribution of α and β subunits of S-100 protein (S-100α, S-100β, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100α (+) and/or S-100β (+)]; astrocytoma, glioblastoma, ependymoma, subependymoma, oligodendroglioma, choroid plexus papilloma, gangliocytoma, meningioma, chordoma, malignant melanoma. Group 2 [S-100α (+) and S-100β (-)]; pineoblastoma, pituitary adenoma, craniopharyngioma, rhabdomyosarcoma. Group 3 [S-100α (-) and S-100β (+)]; acoustic Schwannoma. Group 4 [S-100α (-) and S-100β (-)]; medulloblastoma, malignant lymphoma, germinoma. The S-100β immunoreactivity pattern in brain tumors was similar to those obtained using conventional anti-S-100 protein sera. In the first group of brain tumors both the number of positively stained tumor cells and the staining intensity were generally greater for S-100β than for S-100α with a few exceptions including one gemistocytic astrocytoma, one subependymoma, one malignant melanoma, and some cases of glioblastomas. As to the relationship between malignancy and S-100 protein in glioma, S-100β immunoreactivity decreased according to degree of malignancy, while that of S-100α varied, suggesting a heterogeneity of tumor cells in glioblastomas. Immunostaining for S-100α and S-100β might become a useful diagnostic procedure in brain tumors and may give us more detailed and precise data of S-100 protein in brain tumors.

Spindle cell hemagioendothelioma: A report of two cases

Two cases of spindle cell hemangioendothelioma (SCH) are reported. One of the patients was a 16 year old Japanese female, who had been suffering from Olliers disease (multiple enchondrornatosis) since 3 years of age and had developed multiple SCH in the right leg at the age of 11 years. Spindle cell hemangioendothelioma lesions coincided with the site of enchondromatosis and increased in number thereafter. This is the first report of Olliers disease complicated with multiple SCH. Another patient, a 33 year old Japanese female, who was a carrier of hepatitis B virus (HBV), developed solitary SCH in the lateral aspect of the right ankle where a lipoma was extirpated 10 years previously. Tumor cells of both cases were composed of four cell types: (i) spindle cells; (ii) epithelioid cells; (iii) vacuolated endothelial cells; and (iv) usual endothelial cells. Endothelia in the cavernous area and vacuolated cells reacted to Ulex europaeus agglutin 1 (UEA‐I), factor VIII‐related antigen and vimentin. Spindle cells and epithelioid cells reacted only to vimentin.

ELASTOFIBROMA OF THE GREATER OMENTUM

A rare case of elastofibroma developing in the greater omentum of a 76 years old woman was reported. The greater omentum was thickened in a plate‐like shape to a size of 24 × 15 × 2 cm, and a firm mass as large as 5 × 4 × 3 cm was formed in a portion of the omentum. Granulomatous inflammation associated with the calcification was present in the center region of the mass. Collagen fibers and elastofibroma fibers were markedly proliferated around the granuloma. Fibrosis accompanied with the formation of elastofibroma fibers was observed not only around the granulomatous inflammatory focus but also throughout the thickened omentum. The elastofibroma fiber in this case lost its stainability to resorcin‐fuchsin and orcein after 4 hours treatment with elastase. It may be inferred that not only mechanical stresses but also inflammatory stimuli contribute to the development of elastofibroma.

The distribution of alpha and beta subunits of S-100 protein in malignant schwannomas arising from neurofibromatosis of von Recklinghausen's disease.

The immunohistochemical localization of the alpha and beta subunits of S-100 protein in 4 cases of the malignant Schwannomas arising from von Recklinghausens disease was investigated by the indirect immunoperoxidase method. S-100 alpha-positive cells were few in presumably normal peripheral nerves, moderate in numbers in plexiform neurofibromas and numerous in malignant Schwannomas. In contrast, S-100 beta immunoreactivity, abundantly detected in normal peripheral nerves and plexiform neurofibromas, was completely negative in all of 4 cases of malignant Schwannoma. In addition, double immunostaining method for both subunits revealed their simultaneous existence in cells in the normal nerves and neurofibroma. These results suggest that malignant change of Schwann cells convert their subunit composition of S-100 protein from beta to alpha in these malignant cells. Although the mechanisms for the proportional conversion of the subunits are as yet undetermined, the immunoreactivity of S-100 alpha subunit may be a useful marker for Schwannoma in malignancy.

Titanium dioxide deposition and adenocarcinoma of the lung.

A case of titanium dioxide pneumoconiosis accompanied by lung cancer is reported. The patient was a fifty‐three‐year‐old male, who was engaged in packing titanium dioxide for about thirteen years. At autopsy, a papillary adenocarcinoma was located in the right lung. Titanium was diffusely deposited in the lung and was engulfed by macrophages in the interstitium and alveolar spaces. Slight fibrosis of the interstitium around bronchioles and vessels was noticed as an effect of titanium deposition.

SEBACEOUS CARCINOMA OF THE SUBMANDIBULAR GLAND WITH HIGH-GRADE MALIGNANCY : REPORT OF A CASE

A case of sebaceous carcinoma arising in the left submandibular gland of a 66‐year‐old man is reported. The clinical and pathological examinations revealed a carcinoma, which was of salivary gland In origin, with regional lymph nodal metastases. Pathological findings showed features of highgrade sebaceous carcinoma with spindle myoepitheliomatous differentiation. Neither squamous cell nor duct epithelial‐like cell differentiation was noted. lmmunohistochsmically, tumor cells were positive for cytokeratin, S‐100 protein and vimentin. Lipid was demonstrated in the cytoplasm of the tumor cells. Ultrastructurally, tumor cells contained numerous intracyto‐plasmic lipid droplets. Myoepitheliomatous differentiation is rare in sebaceous carcinoma of the salivary gland. Presented is the second reported case of sebaceous carcinoma arising in the submandibular gland.

MALIGNANT FIBROUS HISTIOCYTOMA OF THE MAXILLARY SINUS

A case of malignant fibrous histiocytoma arising primarily in the left maxillary sinus is described. The patient, a 39‐year‐old male, who had suffered from sinusitis for 20 years, began to have paresthesia or sharp pain of the left side of the face and toothaches of the left maxilla. At operation a white fibrous tumor developing extensively from the lateral wall to the upper and medial walls of the left maxillary sinus and into the ethmoidal sinus was noted. Following gradual progression of dyspnea, he died approximately one year after the onset in spite of radiation therapy and anticancer chemotherapy. An autopsy revealed recurrence of the tumor in the left maxillary sinus with wide‐spread metastases to the lungs, pleurae, pancreas, kidneys and bone marrows. The direct cause of death was respiratory failure due to extensive growths of the pulmonary and pleural metastases.

AN IMMUNOHISTOCHEMICAL STUDY ON THE DISTRIBUTION OF GLIAL FIBRILLARY ACIDIC PROTEIN, S-100 PROTEIN, NEURON-SPECIFIC ENOLASE, AND NEUROFILAMENT IN MEDULLOBLASTOMAS

In order to clarify the differentiation of medulloblastomas, the authors studied on the morphological features and immunohistochemical expression of glial flbrillary acidic protein (GFAP), S‐100 protein, neuron‐specific enolase (NSE), and neuroftlament (NF) in 31 medulloblastomas. GFAP was detected only in a small number of tumor cells of 5 medulloblastomas; S‐100 protein in both small tumor cells and some so‐called spongloblastic cells in 16 medulloblastomas; NSE in the more abundant tumor cells and the matrix in 28 medulloblastomas; NF in a few tumor cells of 12 medulloblastomas; GFAP and NF in 2 medulloblastomas, but each of them in different tumor cells. These results suggest that medulloblastomas have a capacity of differentiation along neuronal and/or glial lines. The conventional morphological markers of differentiation in medulloblastomas such as spongioblastic cells and Homer Wright rosettes were not necessarily compatible with expression of immunohistochemical markers such as GFAP or NF. NSE and S‐100 protein seem less valuable markers of differentiation because they were detected in both neuronal and glial elements. But NSE, which was observed in most medulloblastomas, might have a value as a marker for medulloblastomas.