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Biomedical subjects

G J Mark

Publications and source records attributed to G J Mark.

11 recordsLinked to original sources

Laryngeal mycosis fungoides.

Mycosis fungoides presented as a tumor of the arytenoid cartilage and epiglottis of the larynx of an 80-year-old female. The tumor was initially interpreted as an undifferentiated small cell malignant neoplasm and treated with radiation. Two years later the patient developed widespread cutaneous involvement with mycosis fungoides, including mycosis fungoides bullosum. The mycosis fungoides progressed to death over the next two years. At autopsy widespread visceral involvement was present. The larynx was extensively infiltrated with mycosis fungoides, and the histology was similar to the original laryngeal biopsy. Although mycosis fungoides is generally thought of as a cutaneous disease, it may rarely present in a squamous epithelium-lined organ other than the skin.

Aged

Histological changes produced in skin by equally erythemogenic doses of UV-A, UV-B, UV-C and UV-A with psoralens.

The sequential light microscopic histological changes produced in human skin by a single exposure of UV-A, UV-B, UV-C and oral 8-methoxypsoralen plus UV-A (PUVA) causing approximately equal degress of delayed erythema response, have been evaluated. UV-C and UV-B affect the epidermis to a greater degree than UV-A, while UV-A affects the dermis to a greater degree than UV-B and UV-C. PUVA has prominent effects on both epidermis and dermis, differing in degree from those changes induced by UV-A, UV-B, and UV-C and are longer lasting. The sequence of histological changes following UV exposure is completed more rapidly after exposure to shorter UV wavelengths.

Adult

Mixed pneumoconiosis: silicosis, asbestosis, talcosis, and berylliosis.

Mixed pneumoconiosis is pulmonary disease due to two or more inhaled mineral irritants. Chronic disease due to beryllium has not been a component of any described mixed pneumoconiosis. A man with occupational exposure to a combination of dusts developed severe pulmonary disease. Silicosis, talcosis, asbestosis, and berylliosis were all documented by an open biopsy of the lung. The varieties of mixed pneumoconiosis are summarized.

Asbestosis

Tracheal carcinoids.

Three cases of carcinoid tumor arising in the trachea are reported and contrasted with carcinoids arising in bronchi and carcinoids in general. Only eleven other documented examples of tracheal carcinoids are found in the English literature. The true prevalence of carcinoids primary in the trachea cannot be accurately determined from the literature because of imprecise nomenclature or because of the failure to distinguish this tumor from carcinoids primary in the bronchus. Presenting symptoms are hemoptysis, dyspnea and wheezing, often persisting for many years before the correct diagnosis is made. The treatment of choice is surgical resection of the involved segment of trachea and primary reconstruction. The prognosis is generally good. The tumor metastasized in one of our three cases and in none of the eleven cases in the English literature.

Adolescent

Pulmonary vascular changes in scleroderma.

To determine the incidence and significance of pulmonary vascular changes in scleroderma, all necropsy reports of patients with scleroderma who died at the Massachusetts General Hospital were analyzed and correlated with clinical data. The records of 30 such patients were available. Fourteen had moderate or marked abnormalities in the pulmonary arterial tree. Nine of these 14 patients had predominantly respiratory symptoms. The arterial changes consisted pathologically of intimal and medial hyperplasia affecting pulmonary arteries of all sizes. Of the eight patients with the most severe pathologic changes in the pulmonary arteries, five had slight or no interstitial fibrosis. Three of these five patients had rapidly progressive respiratory failure and severe pulmonary hypertension leading to death. Such cases form a distinct clinicopathologic entity of malignant pulmonary hypertension in scleroderma, comparable to the better recognized entity of malignant renal hypertension in scleroderma.

Adolescent

Multiple peripheral pulmonary carcinoids and tumorlets of carcinoid type, with restrictive and obstructive lung disease.

Multiple peripheral carcinoid tumors were found on lung biopsy in a 53 year old woman with an eight year history of pulmonary nodules. The biopsy specimen showed nodules varying from 1 to 7 mm in greatest diameter. The larger lesions were typical bronchial carcinoids whereas the smaller ones resembled pulmonary "tumorlets." Electron microscopy showed neurosecretory granules typical of carcinoids. The common multicentric origin of carcinoids and tumorlets from Kulchitsky cells is discussed. Pulmonary function testing revealed mild obstructive and restrictive defects, both attributed to the carcinoid tumors. The history, pathology and physiology of this unusual case are presented in detail, with a review of the literature. Obstructive airways disease, presumably due to the extensive bronchiolar involvement, has not previously been reported in this entity.

Airway Obstruction

Cyclophosphamide pneumonitis.

Cyclophosphamide can rarely cause interstitial pneumonitis and fibrosis. Although it has been reported previously in patients being treated for lymphoma, it occurred in this case in a man under treatment for glomerulonephritis. The temporal sequence of the respiratory insufficiency and the histopathology, when compared to the previous examples in the literature, suggest that cyclophosphamide was aetiologically responsible for the lung disease. There may be an interval of one or more months after discontinuation of cyclophosphamide therapy before clinical or radiological improvement occurs.

Adult

Benign blood vascular tumors of the mediastinum. Report of four cases and review of the literature.

The authors present 4 new cases and review 77 previously reported cases of benign blood vascular tumors of the mediastinum. The vast majority are hemangiomas (90%), occur in the first four decades of life (75%), and arise in the anterior mediastinum (68%). Compression and/or invasion of adjacent structures is not uncommon. Concomitant extrathoracic extension (2%) and multiple sites of involvement (2%) are rare. The tumors are smoothly outlined, occasionally lobulated and can be accurately identified when phleboliths are present (10%).

Adolescent

Multiple hepatic adenomas and a hepatocellular carcinoma in a man on oral methyl testosterone for eleven years.

Numerous small hepatic adenomas and hepatocellular carcinoma developed in a man after 11 years of methyl testosterone ingestion. The man presented with an acute surgical abdomen and a large filling defect in the liver. Laparotomy disclosed hemoperitoneum and a large hepatic hematoma. Focal hemorrhagic infarction in the excised right liver lobe involved both adenomas and normal parenchyma. Review of the English literature reveals no other case of both a benign and a malignant hepatocellular neoplasm associated with anabolic steroid therapy. Hemorrhagic benign liver tumor must be considered in the differential diagnosis in both female and male patients on hormone therapy who present with acute abdominal pain.

Adult

Basal cell carcinoma with intraneural invasion.

Two cases of basal cell carcinoma had diffused infiltration that included not only perineural but endoneurial invasion. One patient presented with trigeminal neuralgia, and the other patient developed localized neuralgia when the tumor recurred. Nests and indian files of carcinoma cells were in the endoneurium, a region that anatomically is devoid of lymphatics. The involved nerves displayed axonal degeneration. Such intraneural invasion by basal cell carcinoma is very rare, even in the scelerosing variety of basal cell carcinoma.

Basal Cell Carcinoma