Twelve carcinoid tumors of the uterine cervix were studied. Based on the microscopic structure, they were divided into well-differentiated a n d poorly differentiated types. Both tumor varieties affected adult women. The clinical and gross features of these tumors were indistinguishable from those of invasive squamous cell carcinoma. Of the 12 patients, six died, four with poorly differentiated carcinoid and two with the well-differentiated type. Three patients are alive and symptom-free 9 months to 2 years after treatment; the remaining three were lost to follow-up. Microscopically, four well-differentiated carcinoids showed argyrophil cells, and ultrastructurally two had neurosecretory granules. One of the well-differentiated carcinoids resembled islet cell carcinoma and two had areas resembling medullary carcinoma of the thyroid. The poorly differentiated type of carcinoid had a histologic structure and a cell population very similar to that of the oat cell carcinoma of the lung. Of this group, four were argyrophilic, and by electron microscopy four contained pleomorphic secretory granules. It is considered that these tumors arise from the normal argyrophil cell of the cervix and are part of the group of neoplasms of the diffuse endocrine cell system (APUD).
Abstract.
An inherited form of incomplete male pseudohermaphroditism was studied in two post-pubertal and one pre-pubertal sibling. All patients presented a 46XY karyotype, infantile female external genitalia, lack of breast development and sexual hair. Persistently elevated serum levels of gonadotrophins with normal pituitary responsiveness to LRH were found. Serum 17-OH progesterone, androstenedione, and testosterone levels were extremely low before and after gonadal stimulation with hCG. Laparotomy revealed absence of Wolffian and Mullerian derivatives. Testes were small and cryptorchidic. Microscopic and ultrastructural examination revealed seminiferous tubules with absence of spermatogenesis and normal Sertoli cells. The interstitial spaces were mainly occupied by poorly differentiated cells although in the post-pubertal patients there were small and randomly distributed nodules of Leydig cells without crystaloids. Incubation of testicular tissue from one post-pubertal patient with [14C]acetate showed lack of 14C-incorporation into appropriate steroid carriers. These data were interpreted as demonstrating that gonadotrophin resistance was the underlying abnormality of this syndrome, representing the human counterpart of the 'vet' pseudohermaphroditic rat.
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