A case of cerebral ganglioneuronal tumor in the parietal lobe of an adult

Central nervous system (CNS) neuroblastoma/ganglioneuroblastoma is one of the embryonal tumors with neuronal differentiation found in young adults, but it is most common in children, especially in those below the age of 5 years, whereas extraventricular neurocytoma, a rare neuroepithelial tumor with neuronal differentiation, mostly affects young adults. Here we present a rare case of cerebral ganglioneuronal tumor that occurred in a 32-year-old woman. The patient suffered from tonic convulsion, and computed tomography demonstrated a well-demarcated, round tumor 3.3 cm in size with marked calcification in the right parietal lobe. Histological analysis revealed diffuse infiltration of small, round cells with scattered large ganglion-like cells. Immunohistochemically, the tumor cells did not react with any neuronal molecules, except for chromogranin A in ganglion-like large tumor cells, but electron microscopy demonstrated the presence of synapse-like nerve terminal structures without mature postsynaptic density, suggesting the presence of neoplastic tumor components with neuronal differentiation; thus, this tumor was diagnosed as CNS ganglioneuroblastoma with possible low-grade malignancy because the Mib-1 labeling index was less than 3%–4%. Here we discuss the histological entity of cerebral ganglioneuronal tumors, including extraventricular neurocytoma.     

Pulmonary and Intestinal Involvement in a Patient with Myeloperoxidase-specific Antineutrophil Cytoplasmic Antibody-positive Hermansky-Pudlak Syndrome

A 26-year-old Japanese woman was admitted with a 1-month history of diarrhea, a high fever for a few days, and exacerbation of dyspnea. She was treated with an antifibrotic drug and long-term oxygen therapy for Hermansky-Pudlak syndrome-related pulmonary fibrosis. New ground-glass attenuation appeared on chest computed tomography (CT), and a colon biopsy showed an inflammatory cell accumulation with a high titer of myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (ANCA). Systemic inflammation related to MPO-ANCA titer elevation was suspected. Steroid pulse therapy and intravenous cyclophosphamide improved chest CT findings and diarrhea. Therefore, immunosuppressant treatment should be considered for systemic inflammation related to MPO-ANCA.     

Evaluation of the internal mammary nodes by lymphoscintigraphy and ultrasonography in patients with breast cancer]

The efficacy of lymphoscintigraphy and ultrasonography was evaluated in the detection of internal mammary lymph node (IMN) metastases in patients with breast cancer. On histological examination of IMN, 10 patients had metastases (positive group) and 47 did not (negative group). On lymphoscintigraphy, lack of uptake in the parasternal region of the affected side was classified as abnormal. The frequency of this abnormal finding was not significantly different between both groups. On sonography, the thickness of the sonolucent internal mammary area was measured. The thickness of the affected side was 6 mm or more in two patients of the positive group and only one of the negative group. A thickness of over 6 mm on the affected side was statistically significant (p less than 0.05). The difference in thickness between the affected side and the healthy side was 3 mm or more in four patients of the positive group, and was less than 3 mm in all patients of the negative group. A difference in thickness of more than 3 mm between the two sides was extremely significant (p less than 0.001). In conclusion, sonography is valuable in detecting IMN metastases, while lymphoscintigraphy is not useful. Sonography is recommended as an efficient diagnostic modality for IMN metastases.     

Spindle cell carcinoma of the gingiva: report of an autopsy case

An autopsied case of an 80-year-old man with spindle cell carcinoma of the gingiva is reported. The tumor was polypoid and mostly composed of a sarcomatous proliferation of spindle cells with a small focus of squamous cell carcinoma at the stalk portion. The carcinoma metastasized to a cervical lymph node, lungs and pleura with extension to the diaphragm. In the metastatic lymph node, the squamous cell component was more prominent than the spindle cell one, while only anaplastic pleomorphic carcinoma cells were found in the lungs. The spindle or anaplastic cells were immunohistochemically positive for vimentin and carci-noembryonic antigen (CEA) but not for other epithelial antigens. We have concluded that the sarcomatoid component arose from the oral squamous cell carcinoma by a metaplastic process. This is the first case report of an oral spindle cell carcinoma examined by autopsy.     

An autonomously functioning thyroid carcinoma associated with euthyroid Graves' disease.

A 39-yr-old man with an autonomously functioning thyroid carcinoma is presented. Only 17 similar cases have been reported in the literature. The patient had unilateral Graves' ophthalmopathy. He was euthyroid as reflected by normal TSH concentration, whereas the results of a T3 suppression test established the presence of autonomous thyroid function. A thyroid scan with (123)I revealed a hot nodule corresponding to the location of a papillary carcinoma and remained substantially unchanged after T3 administration. The hyperfunction of the carcinoma itself was clearly confirmed by the intense concentration of (131)I within the tumor on microautoradiograms. While a hot nodule on radioiodine scan is unlikely to be malignant, the possibility of carcinoma should not be overlooked.