Michiyo Nasu

An autopsy case of Menkes kinky hair disease.

An autopsy cases of Menkes kinky hair disease in a 1 year and 8 months old male infant is presented and compared with the morphological findings of the previous literatures. The main pathological changes are atrophy of the whole cerebellar cortex and bilateral temporal lobe, atrophy with demyelination of the white matter, tortuous running of the cerebral arteries, multiple diverticulosis of the urinary bladder and hyaline‐like deposition in the gastric submucosa. Microscopically, the peculiar degenerative change of Purkinje cell (somal sprout) is the only characteristic lesion in our case and the others. It is suggested that Menkes kinky hair disease may be a syndrome due to metabolic disturbance appearing not only in ectoderm such as the central nervous system, but also in mesoderm such as connective tissue and bone.

Primary ductal adenocarcinoma of the lacrimal gland

The morphological similarity of salivary and lachrymal gland tumors is known. Many clinicopathological studies and characteristics of salivary duct carcinoma, which bears histological similarlties to mammary duct carcinoma, have been recently reported; however, only one case of lacrimal duct carcinoma is reported. A second case of lacrimal duct carcinoma is presented. A 67‐year‐old male with a painlass mass in the right upper eyelid underwent total removal of the tumor mass. Microscopic examination of the tumor mass revealed ductal adenocarcinoma of the lacrimal gland, which was the equivalent of salivary duct carcinoma. The Immunohistological studies of the lacriml duct carcinoma showed similar results to those reported for salivary duct carcinoma. The recurrent tumor in the subdural spaces was removed 2 years after the initial surgery and the patient is followed as an outpatient.

Ovarian leiomyosarcoma: an autopsy case report.

Primary non‐specific sarcoma of the ovary is extremely rare, and only 22 reported cases of pure leiomyosarcoma (LMS) are known to the authors. We present an autopsy case of a primary ovarian leiomyosarcoma in a 73‐year‐old woman. She had noticed an abdominal mass after difficulty in defecating for several months. The excision of tumor with bilateral salpingo‐oophorectomy and hysterectomy was carried out. A diagnosis of pure leiomyosarcoma of the left ovary was made on pathological examination with immunohistochemistry. Adjuvant radio–chemotherapy was not given. At 18 months’ follow up, abdomino‐pelvic sonography revealed an abdominal tumor and hepatic metastasis. The patient died 3.5 years after the initial surgery. The post‐mortem examination revealed a peritoneal recurrent tumor and extensive distant metastases of the liver, lungs, pancreas, gastric mucosa, muscle and skin. The prognosis of the ovarian LMS is poor from the pertinent literature. Several prognostic indicators on histology including mitotic activity, proliferative activity and p53 status of the tumor are discussed.

SOLITARY NEUROFIBROMA OF THE ESOPHAGUS

A rare case of solitary neurofibroma located in the esophagus is reported. A large (4.2 × 4.0 × 3.0 cm) submucosal tumor was surgically removed from the midportion of the esophagus of a 64‐year‐old woman. Light and electron microscopic examination, and immunohistochemistry of S‐100 protein in the tumor tissue confirmed neurofibroma. The patient had no evidence of von Recklinghausens neurofibromatosis. A review of literature failed to find other reports of solitary neurofibroma of the esophagus with reliable histological diagnosis. ACTA PATHOL. JPN. 35 : 527–531, 1985.

Pancreatic cancer with metastasis to the larynx.

A laryngeal metastasis from pleomorphic carcinoma of the pancreas is described. The patient was a 70-year-old male. The tumour was located on the right margin of the epiglottis. Bilateral neck lymph node metastases were also found. The patient underwent supraglottic laryngectomy, right radical neck dissection and left suprahyoid neck dissection. Following surgery, the patients general condition deteriorated day by day, and one month after the surgery he died. The autopsy findings demonstrated a big mass of cancer in the pancreatic tail, multiple lymphatic metastases, carcinomatous peritonitis, and possibly a lymphatic spread of the pancreatic cancer to the larynx.

Primary chondroid chordoma arising from the base of the temporal bone. A 10-year post-operative follow-up.

A 36-year-old male with a primary chondroid chordoma is presented. This tumour arose from the base of the temporal bone and extended to the mastoid cavity. It involved the facial nerve and was adherent to the internal jugular vein and internal carotid artery. The tumour was excised and the patient has been carefully followed up for 10 years. He has shown no evidence of local recurrence, intracranial extension of the residual tumour and distant metastasis.

Gastric plasmacytoma and multisystem autoimmune disease

A 54-year-old women with solitary gastric plasmacytoma, Ig M and kappa-light chain type, associated with multisystem autoimmune disease is described. The gastric plasmacytoma developed seven years after the diagnosis of Hashimotos thyroiditis, primary biliary cirrhosis and Sjögrens syndrome. We speculate that this plasmacytoma developed in association with an immunodeficient and/or immunosuppresed state resulting from multisystem autoimmune disease and therapy.

Pathological changes of the lungs after prolonged inhalation of high concentrations of oxygen.

The pathological changes in the lungs of six patients who were treated by an artificial ventilation with a Bird or Bennett type respirator for three days to six months with oxygen concentrations of 24–100% were examined by light microscopy, studies on thick-sections, reconstruction models and vascular casts, and morphometric methods. After prolonged inhalation of high concentrations of oxygen the lungs showed thickening of the alveolar wall, marked deposition of reticulin fibers and fibroblastic proliferation in the alveolar wall, reduction in the number of capillaries, an abnormal configuration of the capillary network and hyperplasia of alveolar lining cells. These lesions are not specific to this condition, and seemed to be less marked than similar lesions in cases of chronic forms of fibrosing alveolitis, chronic interstitial pneumonia, usual interstitial pneumonia and so-called pulmonary fibrosis. Morphometric results confirm these histological observations and show not only the concentrations of oxygen but also the duration of high and pure oxygen inhalation have important roles for these pulmonary lesions. The main reasons for these lesions seemed to be repeated damage to the capillaries and loss of the normal configuration of the capillary network, accompanied by rearrangement and reconstruction of the two types of reticulin fibers.

Desmoid tumor in the maxillary sinus: a case report

Desmoid tumor in the maxillary sinus is a rare condition. The present case occurred in a 37-year-old male complaining of left nasal obstruction and dull pain in the nape of the neck. The clinical picture, pathology, diagnosis and prognosis are discussed and the literature is reviewed.