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Biomedical subjects

R E Scully

Publications and source records attributed to R E Scully.

At least 127 records · Page 7Linked to original sources
PubMed1991

The androgen insensitivity syndrome (testicular feminization): a clinicopathologic study of 43 cases.

Forty-three patients with the androgen insensitivity syndrome (AIS), ages 14 to 83 (average 27) years, were studied. Forty patients had complete AIS and three patients had incomplete AIS. Microscopic examination of the testes revealed immature tubules, which contained rare spermatogonia in 28% of the cases. Prominent Leydig cells and a spindle-cell stroma resembling ovarian stroma were found in a majority of cases. The organization of the testicular parenchyma could be classified into one of four patterns: diffuse tubulostromal, lobular tubulostromal, mixed tubulostromal, or stromal-predominant. Hamartomas were present in 63% and Sertoli cell adenomas in 23% of the cases. Malignant tumors developed in 9% of the patients and comprised two seminomas, one intratubular germ cell neoplasm with early stromal invasion, and a malignant sex cord tumor. At least one fallopian tube was present in 35% of the cases.

Adenoma↗
PubMed1991

Endometrial adenocarcinoma with a component of giant cell carcinoma.

We report six high-grade endometrial adenocarcinomas with a malignant giant cell component. The patients ranged in age from 43 to 85 years (mean 65); five were postmenopausal. All the patients presented with vaginal bleeding. The tumors all had a giant cell component that was composed of poorly cohesive sheets and nests of bizarre multinucleated giant cells admixed with an approximately equal number of mononucleate tumor cells. A sarcomatoid pattern and a marked inflammatory infiltrate were each present in three cases. All the tumors contained at least focal areas of endometrial adenocarcinoma of one of the usual types. Occasional malignant giant cells were positive for cytokeratins (AE1/AE3 or CAM 5.2) and epithelial membrane antigen in all three tumors tested. Three tumors were in stage I, one stage III and two stage IV. Two of the patients with stage I disease (each with superficial myometrial invasion) were alive and well 6 and 2 years later. Of the remaining four patients, three died of disease 2.5 years, 6 months, and 5 months after presentation, and one was alive with extensive abdominal disease after 1 year. Giant cell carcinoma of the endometrium is an aggressive tumor that should be distinguished from other endometrial tumors with a prominent giant cell component, including trophoblastic tumors, certain primary sarcomas, and malignant mixed müllerian tumors. It is imperative that malignant giant cells and non-neoplastic giant cells not be confused, as is possible in a curettage or biopsy specimen.

Adenocarcinoma↗
PubMed1991

Sebaceous tumors arising in ovarian dermoid cysts.

The clinicopathologic features of five cases of sebaceous tumors arising in ovarian dermoid cysts and of three previously reported cases are reviewed. They occurred in women with an average age of 58 years and were classified as sebaceous adenoma (five cases), basal cell carcinoma with sebaceous differentiation (two cases), and sebaceous carcinoma (one case). Follow-up information was available for all cases. One patient with basal cell carcinoma with sebaceous differentiation had a pelvic recurrence 2 1/2 years after diagnosis. In no other case did the sebaceous tumor recur or metastasize during follow-up periods of 1 to 6 years. One patient died of a squamous cell carcinoma that arose in the same dermoid cyst as the sebaceous tumor. These tumors represent a rare form of monodermal neoplasia in dermoid cysts.

Adenocarcinoma↗
PubMed1991

Ovarian and juxtaovarian adenomatoid tumors: a report of six cases.

Five ovarian adenomatoid tumors and one juxtaovarian tumor of this type in women 23 to 79 (average 54) years of age are described. Four were less than or equal to 7 mm in diameter; the other two were 1.4 cm and 5 cm. The five ovarian tumors were located predominantly in the hilus, with focal extension into the medulla. Microscopic examination showed the lesions to be characterized by round, oval, or elongated tubules, some of which were cystically dilated, and vacuoles occasionally containing basophilic secretion. Hyaline bodies were present in one case. Follow-up in these cases and the 10 previously reported similar cases that include follow-up information has been benign. The microscopic features of the ovarian adenomatoid tumor are typically diagnostic, but, as illustrated by one of our cases and another in the literature, some features that simulate those of yolk sac tumor may cause diagnostic difficulty when the tumor occurs in a young patient.

Adult↗
PubMed1991

Diffuse laminar endocervical glandular hyperplasia. A benign lesion often confused with adenoma malignum (minimal deviation adenocarcinoma).

We report seven examples of a hitherto undescribed pseudoneoplastic glandular lesion of the uterine cervix that we have designated "diffuse laminar endocervical glandular hyperplasia." The patients ranged in age from 22 to 48 (mean 37) years. Only one of them had a history of hormone intake. All the lesions were incidental findings in hysterectomy specimens performed for dysfunctional uterine bleeding (five cases) or symptomatic leiomyomas (two cases). Microscopic examination revealed a proliferation of moderate-sized, evenly spaced, differentiated endocervical glands within the inner one-third of the cervical wall, sharply demarcated from the underlying cervical stroma. Reactive atypia was seen in some cases but significant cytologic atypia was absent. A marked inflammatory response was present in five of the seven cases. The main consideration in differential diagnosis was adenoma malignum (minimal deviation adenocarcinoma). Major features that helped in this distinction were a lack of irregular stromal infiltration and a desmoplastic stromal response, and an absence of focal malignant cytologic features. Follow-up for an average of 6.5 years in five of the seven cases was uneventful.

Adenocarcinoma↗
PubMed1991

Clinical syndromes associated with tumors of the female genital tract.

Tumors of the female genital tract may be associated with a variety of unusual clinical manifestations. Uncommon endocrine and paraendocrine syndromes include production of human chorionic gonadotropin by tumors other than those of germ cell origin, hyperthyroidism associated with struma ovarii and gestational trophoblastic disease, the carcinoid syndrome, the Zollinger-Ellison syndrome, hypercalcemia, Cushing's syndrome, hypoglycemia, hypertension related to renin or aldosterone production, hyperprolactinemia, inappropriate secretion of antidiuretic hormone, and virilization associated with Nelson's syndrome and placental site trophoblastic tumor. Paraneoplastic syndromes associated with gynecological tumors include disorders of the nervous system, connective tissue, and skin, as well as hematologic abnormalities and the nephrotic syndrome. Heritable and other congenital syndromes associated with these tumors are the Peutz-Jeghers syndrome, the nevoid basal-cell carcinoma syndrome, Ollier's disease and Maffucci's syndrome, hereditary leiomyomatosis, ataxia-telangiectasia, von Hippel-Lindau's disease, thyroid abnormalities associated with Sertoli-Leydig cell tumors, and Carney's complex. Other syndromes associated with tumors of the female genital tract include Meigs' syndrome, hyperamylasemia, uveal melanocytic lesions, and pyrexia.

Cushing Syndrome↗
PubMed1991

Metastatic tumors in the ovary: a problem-oriented approach and review of the recent literature.

Metastatic tumors account for a significant proportion of the ovarian tumors that cause major problems in diagnosis for the surgical pathologist. This subject is reviewed with a consideration of the various patterns and cell types encountered in these tumors. The first two categories considered, tumors with endometrioid-like and mucinous appearances, include a variety of neoplasms originating in the gastrointestinal tract and its appendages that simulate closely primary ovarian endometrioid and mucinous tumors. Other important categories reviewed include several varieties of metastatic tumors with follicle-like spaces, small cell malignant tumors, and tumors characterized by cells with abundant eosinophilic cytoplasm. The review also includes a discussion of the general considerations that are helpful in the distinction of primary and metastatic ovarian tumors. Important recent reports in the literature concerning metastatic tumors are additionally summarized.

Female↗
PubMed1991

Ovarian myxoma: clinicopathologic and immunocytologic analysis of five cases and a review of the literature.

The clinical and pathological features of five personally observed ovarian myxomas and three similar tumors previously described in the literature are analyzed. The patients' ages ranged from 16 to 45 years (mean, 33 years). An asymptomatic unilateral adnexal mass was the usual presentation. The tumors were 5 to 22 cm (mean, 11 cm) in greatest diameter, intraovarian, solid, and cystic, and they were occasionally blood-filled. None had ruptured. The intercellular matrix contained abundant hyaluronic acid in each of the five personally observed cases. The tumor cells were immunoreactive for vimentin and usually for actin, but not for desmin, S100-protein, neuron-specific enolase, neurofilament, Leu-7, epithelial membrane antigen, keratin (AE1/3, CAM5.2, MAK6), or factor VIII-related antigen, and did not bind Ulex europaeus agglutinin 1. Vascularity was a notable feature on microscopic examination. All the patients were treated by a surgical procedure alone, usually a conservative operation, and none of the five tumors with a follow-up period of 1 to 13 years (mean, 5 years) recurred. The ovarian myxoma is a distinctive type of ovarian neoplasm that should be distinguished from massive edema, edematous fibroma, and myxoid sarcomas.

Actins↗
PubMed1990

Pathology of gynecologic malignancies.

This review encompasses advances in the pathology of female genital tract tumors that have been deemed to have clinical significance. A number of newly described neoplasms are discussed as are the results of analyses of large series of cases of previously described tumors. Recent revision in terminology and the application of newer techniques for evaluating neoplasms, such as flow cytometry, are also briefly reviewed.

Female↗
PubMed1990

Mullerian adenosarcoma of the uterus: a clinicopathologic analysis of 100 cases with a review of the literature.

One hundred cases of mullerian adenosarcoma of the uterus were encountered in patients 14 to 89 years of age (median, 58 years), who usually had the symptom of abnormal vaginal bleeding. An enlarged uterus and tissue protruding from the external os were the most common findings on pelvic examination. Five patients presented on multiple occasions with "recurrent polyps" that were interpreted retrospectively as adenosarcomas. Primary treatment, known in 97 cases, included some form of hysterectomy in 93 of them, and conservative resection in four cases. Gross examination of the excised uteri disclosed polypoid masses, some of which had spongy cut surfaces, usually filling the endometrial cavity; less commonly, the tumors were confined to the endocervix or the myometrium or involved more than one site. Histologic examination revealed benign or atypical neoplastic glands within a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity, intraglandular polypoid projections, or both. The sarcomatous stroma was homologous in 78% of the cases and contained heterologous elements in the remainder. The stromal mitotic rate varied from 1 to 40 mitotic figures (MFs) (mean 9) per 10 high-power fields (HPFs). Extensive areas of stromal fibrosis that focally imparted a deceptively benign appearance to the tumor were common. Myometrial invasion was present in 15 cases, but was deep in only four. Recurrent tumor developed in 23 cases at postoperative intervals of 0.5 to 9.5 years (mean 3.4); in one third of such cases, the interval was 5 years or longer. Recurrent tumor was almost always confined to the vagina, pelvis, or abdomen; hematogenous spread occurred in only two cases. The only feature associated with an increased risk for recurrence was the presence of myometrial invasion. Criteria found useful in separating mullerian adenosarcomas from mullerian adenofibromas included, alone or in combination: two or more stromal MFs per 10 HPFs, marked stromal cellularity, and significant stromal cell atypia.

Adolescent↗
PubMed1990

Ovarian serous borderline tumors with stromal microinvasion: a report of 21 cases.

The clinicopathologic features of 21 cases of otherwise typical serous borderline tumors that contained small foci of stromal invasion were reviewed. The mean age of the patients was 43 years and six of them were pregnant at the time of diagnosis. Nineteen tumors were stage I, one was stage III (para-aortic lymph node involvement) and one was stage IV (parenchymal liver metastasis). The tumor invaded the stroma predominantly as individual cells or nests or clusters of cells with abundant eosinophilic cytoplasm (17 cases), as small confluent nests with a cribriform pattern (two cases) and as rounded aggregates of papillae (two cases). Seven women were treated with bilateral salpingo-oophorectomy and hysterectomy; 13 had less than bilateral oophorectomy. Of the 17 patients for whom followup data were available, 16 were without evidence of disease 1 to 11 (mean, 5.2) years postoperatively, and one patient had a serous borderline tumor with microinvasion in a conserved contralateral ovary 2.8 years postoperatively, but was well 6 months after a partial oophorectomy. These data suggest that serous borderline tumors with microinvasion have a prognosis similar to that of the usual serous borderline tumor, and that conservation of the contralateral ovary and uterus may be acceptable therapy in young women who wish to preserve their fertility.

Adolescent↗
PubMed1990

Serous borderline tumors of the peritoneum.

The clinicopathological features of 25 cases of peritoneal serous neoplasms histologically identical to noninvasive implants of ovarian serous borderline tumors but with minimal or no ovarian surface involvement were reviewed. The patients ranged in age from 19 to 53 (mean, 31) years; 18 of them were under 35 years of age. Infertility and abdominal pain were the most common presenting complaints. An extraovarian mass was present in two patients; adhesions or granularity of peritoneal surfaces were described in 23 of them. In 21 cases only the pelvic peritoneum was involved; the upper abdominal peritoneum was involved additionally in four cases. Most of the women were treated by hysterectomy, bilateral salpingo-oophorectomy, and omentectomy; six of them received chemotherapy postoperatively and two received both chemotherapy and radiation therapy. Nine women had a more limited operation to preserve their fertility. The 25 patients were followed for 4 to 13.9 (mean, 8) years. There was no clinical evidence of recurrence in 21 women. Borderline tumor recurred in two patients, who remained well for 1.7 and 2 years after excision of the recurrent tumor. Invasive low-grade serous carcinoma of the peritoneum developed in one woman who was living with extensive intra-abdominal tumor at the last follow-up examination. One woman died of disseminated SBT, which was diagnosed cytologically but not confirmed by biopsy.

Adult↗
PubMed1990

Stromal endometriosis of the uterine cervix. A variant of endometriosis that may simulate a sarcoma.

Six cases of endometriosis of the uterine cervix characterized by the exclusive or almost exclusive presence of endometriotic stroma are reported. These cases of "stromal endometriosis" were encountered in women 29 to 64 (mean, 43) years of age. Three of them presented with abnormal bleeding, one presented with weight loss and abdominal swelling due to an ovarian clear cell adenocarcinoma, and two were asymptomatic. None of the patients had a history of pelvic endometriosis. In three cases, red lesions of the ectocervical mucosa were recognized on pelvic examination. Histological examination of the lesions showed well circumscribed foci within the superficial stroma of the cervix that were composed of an admixture of closely packed cells resembling endometrial stromal cells, small blood vessels, and extravasated erythrocytes. Endometrial-type glands were absent in the initial sections in all of the cases, but rare glands were found in deeper sections in one case. Apart from the absence or paucity of endometrial glands, the clinical and pathological features of the lesions were similar to those of previously described cases of superficial endometriosis of the cervix. Because of the pure or almost pure stromal composition of the lesion, however, it can be confused histologically with benign and malignant neoplasms, particularly low-grade endometrial stromal sarcoma and Kaposi's sarcoma.

Adult↗
PubMed1990

Peritoneal keratin granulomas with carcinomas of endometrium and ovary and atypical polypoid adenomyoma of endometrium. A clinicopathological analysis of 22 cases.

Twenty-two cases of keratin granulomas of the peritoneum associated with endometrioid adenocarcinoma with squamous differentiation of the endometrium, the ovary, or both, and with an atypical polypoid adenomyoma of the endometrium were reviewed. Follow-up data were available in 18 cases. Twelve patients were well and disease free 13 months to 15.2 years postoperatively; one patient died of unrelated disease 21 years postoperatively; three patients were tumor free with a short duration of follow-up; one patient, who had a stage Ic ovarian tumor, died of pulmonary embolism during the treatment of recurrent tumor 1 year after operation; and a final patient, who had been followed for 3 months after operation for stage IV disease, was alive with residual tumor. At least six patients with stage I carcinomas were treated with postoperative irradiation because the granulomas had raised a suspicion of advanced disease. Follow-up data on the patients in this series suggest that peritoneal keratin granulomas have no prognostic significance and should be distinguished from viable tumor implants on microscopic examination.

Adenocarcinoma↗
PubMed1990

Placental site nodules and plaques. A clinicopathologic analysis of 20 cases.

The clinical and pathological features of 20 cases of a nodular or plaquelike trophoblastic lesion at the placental site, which we have designated "placental site nodule or plaque" (PSN-P), are described. The lesions occurred in patients 27 to 45 years of age, and were discovered in an endometrial curettage specimen performed because of menorrhagia or irregular uterine bleeding, or were incidental findings in curettage or hysterectomy specimens. They were grossly visible in five cases. Microscopic examination disclosed single or multiple, mostly well-circumscribed, oval, or plaquelike, variably cellular nodules that were characteristically extensively hyalinized. The lesional cells typically had abundant cytoplasm that was amphophilic, eosinophilic, or occasionally vacuolated, and irregular, often degenerative-appearing nuclei; mitotic figures were absent or rare. Immunohistochemical staining for human placental lactogen was focally positive in all but one specimen stained; most of the lesional cells stained for cytokeratin. An uneventful follow-up of 1 to 7 years was obtained in 14 cases, including five in which the only treatment was dilatation and curettage. The major lesion to be differentiated from the PSN-P is the placental site trophoblastic tumor. Features favoring the former diagnosis include its usually small size, circumscription, extensive hyalinization, degenerative appearance, and mitotic inactivity. These features, as well as the absence of squamous cells, help distinguish PSN-P from the rare hyalinizing squamous cell carcinoma of the cervix. The available evidence indicates that PSN-P is benign.

Adult↗