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Biomedical subjects

R E Scully

Publications and source records attributed to R E Scully.

At least 217 records · Page 12Linked to original sources
PubMed1986

Functioning ovarian tumors with peripheral steroid cell proliferation: a report of twenty-four cases.

Twenty-four cases of an unusual type of ovarian tumor with functioning stroma in which bands of steroid-hormone-producing cells were located adjacent to the tumor are presented. The cells were of stromal lutein cell type in 14 cases, stromal Leydig cell type in two cases, and hilus cell type in eight cases. Of the tumors with peripheral lutein cells, 11 were monodermal teratomas, including eight examples of struma ovarii, two strumal carcinoids, and one trabecular carcinoid; the other three tumors in this group were a dermoid cyst, a dysgerminoma containing syncytiotrophoblast cells, and a metastatic carcinoid. The tumors with peripheral stromal Leydig cells were a struma ovarii and a strumal carcinoid. Three of the tumors with peripheral hilus cells were mucinous cystadenomas, four were cysts of probable rete origin, and the eight was a dermoid cyst. The patients' ages ranged from 13-82 (average 58) years. Eighty percent of the patients were postmenopausal; one was pregnant. Forty-two percent of the patients had androgenic manifestations, 29% had evidence of hyperestrinism, and one (4%) had evidence of progesterone excess. In 29% of the cases there was no clinical evidence of hormonal imbalance. Those tumors with peripheral stromal Leydig cells or hilus cells were accompanied by androgenic manifestations in over one-half the cases, and those tumors with peripheral lutein cells had a slightly higher association with estrogenic rather than androgenic changes. Human chorionic gonadotropin and its beta subunit were demonstrated by the immunoperoxidase technique in some neoplastic cells in 4 of the 17 cases in which it was performed. Ovarian tumors with peripheral steroid-hormone-producing cells should be distinguished from the typical ovarian tumor with functioning stroma, which contains lutein cells scattered throughout its stroma.

17-Ketosteroids↗
PubMed1986

Nephrogenic adenoma. A report of 15 cases, review of the literature, and comparison with clear cell adenocarcinoma of the urinary tract.

The clinical and pathological features of 15 previously unreported and 98 reported cases of nephrogenic adenoma were reviewed and compared with those of the 19 reported clear cell adenocarcinomas of the urinary bladder and urethra. Nephrogenic adenoma has occurred in patients from 4 to 83 (average 41) years of age. Two thirds of the patients were male, and most had a history of a genitourinary operation or procedure, genitourinary trauma, urinary calculi, or a renal transplant. Nephrogenic adenomas are typically small but have measured up to 7 cm. They may be papillary, polypoid, or sessile, and approximately 18% have been multiple. Microscopic examination shows, singly or in combination, tubules, cysts, and papillae lined by cells that typically have scanty cytoplasm and exhibit little cytological atypia and no or only very rare mitotic figures. Clear cell adenocarcinomas, in contrast, occur in a generally older age group, have a strong predilection for females, and typically lack the clinical features associated with nephrogenic adenomas. The carcinomas are almost always solitary and usually large. The microscopic findings of a diffuse growth pattern, clear cells with a high cytoplasmic glycogen content, and significant nuclear atypia and mitotic activity are characteristic of carcinomas in contrast to nephrogenic adenomas.

Adenocarcinoma↗
PubMed1986

Ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal-type epithelium. An immunohistochemical analysis.

Eight ovarian heterologous Sertoli-Leydig cell tumors containing gastrointestinal-type cells, including two tumors that contained carcinoids, were stained for argyrophilia and argentaffinity; in addition, these specimens were stained by immunohistocytochemical techniques for the demonstration of chromogranin, serotonin, and a variety of peptide hormones. Intestinal- and gastric-type epithelial and carcinoid cells within the tumors were focally argyrophilic and chromogranin-positive, but only intestinal-type epithelial and carcinoid cells contained argentaffin granules, serotonin, and corticotropin. Somatostatin, gastrin, neurotensin, and glucagon were demonstrated additionally in varying numbers of specimens containing intestinal-type epithelium and carcinoid, and somatostatin was present in gastric-type epithelium in one case. Staining for calcitonin and insulin was negative. Despite the frequent identification of serotonin and peptide hormones in the tumors in the present series, evidence of the carcinoid syndrome or syndromes associated with peptide hormone excess was lacking on review of the patients' records.

Carcinoid Tumor↗
PubMed1985

Benign and borderline clear cell adenofibromas of the ovary.

The criteria for the diagnosis of benign and borderline clear cell adenofibromas and their biologic behavior were investigated by examination of the clinical and pathologic features of 18 tumors in these categories. Three tumors that showed no significant epithelial atypicality were classified as benign. Twelve tumors that contained glands or small solid nests composed of epithelial cells with nuclear characteristics of low-grade malignancy without invasion of the stromal component of the tumor were designated as borderline. Three predominantly borderline tumors with focal microinvasion of the stromal component were also studied. The 17 patients had nonspecific complaints. Sixteen of the tumors were unilateral without surface involvement; one patient had bilateral borderline tumors. Most of the women were treated by hysterectomy and bilateral salpingo-oophorectomy. Follow-up information was available for 16 of the 17 patients. No recurrences or deaths from tumor occurred in 14 patients (2 benign, 10 borderline, 2 microinvasive). One patient with a borderline adenofibroma had questionable lung metastasis 4 years after presentation, and another patient who had a microinvasive tumor had a pelvic recurrence 3.3 years postoperatively.

Adenofibroma↗
PubMed1985

Puberty without gonadotropins. A unique mechanism of sexual development.

Recent evidence suggests that a group of children exists in whom premature sexual maturation occurs in the absence of pubertal levels of gonadotropins; that is, they have gonadotropin-independent precocious puberty. We compared six boys and one girl with this disorder with four boys and five girls with central precocious puberty, in which there is a pubertal pattern of gonadotropin release. The two groups were similar in age of onset, degree of sexual development, growth velocity, and rate of skeletal maturation. A family history of precocity was noted in four of the boys with gonadotropin-independent precocity, and the girl had McCune-Albright syndrome. Children with central precocious puberty demonstrated a pulsatile release of gonadotropins, pubertal responses to luteinizing hormone-releasing hormone, and complete suppression of gonadarche after exposure to an analogue of luteinizing hormone-releasing hormone (LHRHa). In contrast, children with gonadotropin-independent precocity demonstrated an absence of gonadotropin pulsations, variable responses to luteinizing hormone-releasing hormone, lack of suppression of puberty in response to LHRHa, and cyclic steroidogenesis. Tissue from testicular biopsies performed in five of six boys with gonadotropin-independent precocity showed a range from incipient pubertal development of the tubules with proliferation of Leydig cells to the appearance of normal adult testes. We conclude that gonadotropin-independent precocious puberty is a distinct syndrome, of unknown cause, that may be familial and may have been responsible for many previously reported cases of precocious puberty.

Child↗
PubMed1985

Endometrial "sarcomas" complicating ovarian thecoma, polycystic ovarian disease and estrogen therapy.

Unopposed endogenous and exogenous estrogenic stimulation has been considered by most investigators to have a role in the pathogenesis of carcinoma of the endometrium. Although a few cases of "sarcomas" of the endometrium that had developed in an estrogenic setting have been reported, a clear-cut association between estrogenic stimulation and these forms of endometrial cancer has not been established. We report six cases of endometrial sarcomas complicating ovarian thecomas, polycystic ovarian disease, or prolonged estrogen therapy. Three ovarian thecomas, which are considered to be estrogenic tumors, were associated with endometrial malignant mullerian mixed tumor, mullerian adenosarcoma, and low-grade stromal sarcoma in postmenopausal women. Polycystic ovarian disease, a condition characterized by unopposed estrinism due to the peripheral conversion of excessive androstenedione to estrone, was found in a 27-year-old infertile woman with an endometrial malignant mullerian mixed tumor. A pure osteogenic sarcoma of endometrial stromal origin developed in a 28-year-old woman with gonadal dysgenesis (Turner's syndrome) who had received estrogens for 18 years. The sixth woman, with an empty sella turcica after radiation therapy of a pituitary adenoma, had an endometrial mullerian adenosarcoma at the age of 40 years after 16 years of estrogen therapy. None of these patients had had pelvic radiation therapy. The evidence from this series of cases and from six additional cases identified in the literature suggests that the risk of endometrial sarcomas may be increased by estrogen therapy or endogenous disorders that lead to unopposed estrogenic stimulation of the uterus.

Adult↗
PubMed1985

Ovarian metastases from cancer of the lung: problems in interpretation--a report of seven cases.

Seven women, 26 to 66 (average 42) years of age, from whom ovarian tumors were removed before (three cases), synchronously with (three cases), or less than 1 year after (one case) the discovery of a pulmonary neoplasm are the subjects of this report. The ovarian tumor was unilateral in six cases and bilateral in one case and was unassociated with intraabdominal spread. The lung tumors, which were similar to the ovarian tumors on microscopic examination, were central in six cases and peripheral in one; three of them were small cell undifferentiated carcinomas, two large cell undifferentiated carcinomas, one a poorly differentiated adenocarcinoma, and one an atypical spindle cell carcinoid tumor. In each case the question of the primary site of the tumor had been raised clinically. The histological similarity of the ovarian and pulmonary tumors in all the cases to well-recognized forms of pulmonary neoplasia as well as additional clinical and pathological features of the cases led to an interpretation of a pulmonary origin of the tumors in all the cases.

Adenocarcinoma↗
PubMed1985

A distinctive glomerular lesion complicating placental site trophoblastic tumor: report of two cases.

Renal disease with distinctive pathologic features developed in two young women who had placental site trophoblastic tumors. The renal abnormalities were manifested by proteinuria in both cases and by hematuria in one case; blood pressure was elevated in one of the patients. Pathologic examination of the kidneys showed distinctive glomerular abnormalities, characterized mainly by the presence of occlusive eosinophilic deposits in many of the glomerular capillary lumina, most of which stained for fibrinogen-related antigens and IgM by immunohistochemical techniques. Ultrastructural examination showed the deposits to consist mainly of granular material that contained packets of fibrillar material with the appearance of fibrin. The uterine tumors were composed of mononucleated and multinucleated cells with abundant cytoplasm that infiltrated between the muscle bundles of the myometrium; in both tumors there was prominent deposition of eosinophilic material that had the tinctorial properties of fibrin and that stained for fibrinogen and IgM in immunoperoxidase studies. The renal abnormalities disappeared after hysterectomy in one case; the other patient, who was receiving chemotherapy and had disseminated intravascular coagulation, died with leukopenia and sepsis. The clinical and pathologic features in these cases suggest that the renal abnormalities were related to the uterine tumors and that the production of immune complexes and/or the activation of intravascular coagulation by the tumors were pathogenetic mechanisms.

Adult↗
PubMed1985

Juvenile granulosa cell tumor of the infantile testis. A report of 14 cases.

Fourteen testicular tumors diagnosed in infants less than 6 months of age had a distinctive appearance resembling that of the juvenile granulosa cell tumor of the ovary. One of them was discovered at autopsy in an infant of 30 weeks' gestational age; seven were diagnosed during the first few days of life, and the remainder between 3 weeks and 4 1/2 months of age. Enlargement of a scrotal testis was the presenting manifestation in 10 cases and abdominal swelling in one case; one tumor was found in a descended testis that had undergone torsion and one was discovered in an inguinal testis at the time of inguinal herniorrhaphy. The tumors ranged from 0.8 to 5.0 cm in diameter and were cystic or partly cystic and partly solid. Microscopic examination disclosed both follicular and solid components. The follicles were of varying sizes and contained eosinophilic or basophilic fluid that stained positively for mucin in some of the cases. The solid foci typically had a nodular arrangement and occasionally were hyalinized or had a basophilic background due to the presence of intercellular mucin. The neoplastic cells contained moderate to large amounts of eosinophilic cytoplasm and round to oval hyperchromatic nuclei, which typically lacked grooves. The mitotic rate varied from less than 1-24/10 high-power fields. Limited follow-up examination revealed no evidence of recurrence. The microscopic features of these neoplasms warrant their designation as "juvenile granulosa cell tumor."

Follow-Up Studies↗
PubMed1985

Leydig cell tumors of the testis. A clinicopathological analysis of 40 cases and review of the literature.

The clinical and pathological features of 40 Leydig cell tumors of the testis were analyzed. The patients ranged from 2 to 90 (average 46.5) years of age. The most common initial manifestation was testicular swelling, which was sometimes associated with gynecomastia; 15% of the patients presented because of gynecomastia and were found to have palpable testicular tumors. All three children were brought to the physician because of isosexual pseudoprecocity. The tumors, one of which was asynchronously bilateral, ranged from 0.5 to 10.0 (average 3) cm in greatest diameter. They were usually well circumscribed, but in seven of them the margin with the adjacent testis was ill-defined. On microscopic examination the most common pattern was that of diffuse sheets of neoplastic cells, but insular, trabecular, pseudotubular, and ribbon-like patterns were also encountered. The neoplastic cells were most often large and polygonal with abundant eosinophilic, slightly granular cytoplasm; occasionally the cytoplasm was abundantly vacuolated. In eight tumors some of the cells were spindle-shaped, and in six some had scanty cytoplasm. Crystalloids of Reinke were identified in 35% of the tumors. Conspicuous nuclear atypicality was present in 12 tumors and the mitotic rate ranged from less than 1 to 32/10 high-power fields. Blood vessel invasion, lymphatic invasion, or both were identified in four tumors. Follow-up of 2 months to 22 years (average 4 years) was available for 30 patients. Five of them died as a result of spread of their tumor. A comparison of the clinically malignant tumors with those associated with survival for 2 or more years postoperatively revealed that the former occurred in older patients and were accompanied by symptoms of shorter duration and an absence of endocrine manifestations. The malignant tumors were larger, often had an infiltrative margin and had spread beyond the testis, frequently exhibited blood vessel or lymphatic invasion, and had a greater degree of cellular atypia and necrosis and a higher mitotic rate than the benign tumors.

Adult↗
PubMed1985

Atypical and borderline endometrioid adenofibromas of the ovary. A report of 27 cases.

The significance of varying degrees of epithelial atypicality was investigated in 27 endometrioid adenofibromas and cystadenofibromas of the ovary. The tumors were classified as atypical or borderline on the basis of the degree of atypicality of the epithelial element. All tumors were confined to the ovary at the initial operation and most of them were treated by a hysterectomy and bilateral salpingo-oophorectomy. Seven tumors with mild to severe cytologic and architectural atypicality similar to that encountered in atypical endometrial hyperplasia were classified as atypical adenofibromas. No recurrences or deaths from tumor occurred in patients with this type of tumor during 1-18.5 years of follow-up. Twenty tumors that contained closely packed glands or epithelial islands with a cribriform pattern composed of cells with low-grade malignant nuclear characteristics embedded in an abundant fibromatous stroma without evidence of invasion were classified as borderline. Nineteen patients with tumors of this type had no evidence of disease 1-13 years after initial therapy. One of these tumors was discovered at autopsy. These data, although based on a relatively small series of cases, suggest that atypical endometrioid adenofibromas and those of borderline malignancy have an excellent prognosis.

Adenofibroma↗
PubMed1985

Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases.

The clinical and pathological features of 207 ovarian Sertoli-Leydig cell tumors from our consultation and hospital files were reviewed. The patients ranged in age from 2 to 75 (average 25) years. Seventy-five percent of them were 30 years of age or younger and less than 10% were over 50 years of age. One-third of the patients presented because of unequivocal evidence of androgen excess, and an additional 10% had a history suggesting androgen excess; most of the remaining patients complained of abdominal swelling or pain. At operation, 97.5% of the tumors were Stage I, 1.5% were Stage II, and 1% were Stage III. Both ovaries were involved in 1.5% of the cases. The tumors ranged from microscopic to 51 cm in diameter (average 13.5 cm); 15% of them were ruptured. Thirty-eight percent of the tumors were solid, 58% were solid and cystic, and 4% were cystic. The solid tissue was typically lobulated and yellow. On microscopic examination, 11% of the tumors were well differentiated, 54% were of intermediate differentiation, 13% were poorly differentiated, and 22% contained heterologous elements according to the criteria of the World Health Organization; a prominent retiform pattern was present in 15% of them. Follow-up was obtained for 164 patients. The tumor was clinically malignant in 18% of them. The prognosis correlated most meaningfully with the stage and degree of differentiation of the tumor. The high-stage tumors were all clinically malignant. All the well-differentiated tumors were benign, but 11% of those of intermediate differentiation, 59% of the poorly differentiated tumors, and 19% of those with heterologous elements were malignant. In a few cases radiation therapy, chemotherapy, or a combination of the two, in addition to surgical excision, was of benefit in the management of the malignant tumors.

Adolescent↗
PubMed1985

Juvenile granulosa cell tumor--another neoplasm associated with abnormal chromosomes and ambiguous genitalia. A report of three cases.

Three infants, 3 months of age or younger with abnormal karyotypes and ambiguous genitalia, had gonadal juvenile granulosa cell tumors. Two of the patients had mixed gonadal dysgenesis and the third had an intersexual disorder of undetermined type. Two tumors arose in undescended testes, and the third in an undescended gonad of uncertain nature. The occurrence of this uncommon neoplasm in these infants indicates that it is another type of neoplasm that may develop in the gonad of a patient with an abnormal karyotype and ambiguous genitalia.

Chromosome Aberrations↗
PubMed1985

Clear cell adenocarcinoma of the bladder and urethra. A report of three cases and review of the literature.

The clinical and pathological features of three hitherto unreported and 16 previously reported examples of clear cell adenocarcinoma of the urinary bladder and urethra are presented. Six tumors arose in the bladder and 13 in the urethra. Sixteen of the patients were female; they ranged in age from 35 to 78 years. The majority of the tumors were papillary but some were sessile. Microscopic examination revealed a variety of patterns, including tubular glands, cysts, papillae, and diffuse areas. Cells with abundant glycogen-rich clear cytoplasm and hobnail cells were identified in most of the tumors. These tumors must be distinguished from nephrogenic adenomas. A young age or a history of genitourinary trauma, operation, or calculous disease may be a clue to the latter diagnosis; microscopic features such as sheets of clear cells, significant pleomorphism, or mitotic activity favor the diagnosis of clear cell adenocarcinoma. Follow-up of most of the patients, the majority of whom were treated by a radical operation, was short; five tumors are known to have metastasized.

Adenocarcinoma↗
PubMed1985

Lymphoma-like lesions of the lower female genital tract: a report of 16 cases.

The microscopic differential diagnosis of malignant lymphoma and benign lymphoid infiltrates in the lower female genital tract may be difficult. We have reviewed the clinical and pathological features of 16 lymphoma-like lesions of the cervix (10 cases), endometrium (five cases), and vulva (one case) and compared these features with those of malignant lymphoma. One patient with a cervical lesion and one with a vulvar lesion had infectious mononucleosis. Microscopic examination of the lesions revealed an infiltrate of large lymphoid cells with prominent mitotic activity, including cleaved and noncleaved follicular center cells and immunoblasts; a starry-sky pattern was present in three cases. Plasma cells, polymorphonuclear leukocytes, and small lymphocytes were present within the infiltrate in many of the cases. Immunohistochemical stains were performed in seven cases. In six they indicated the presence of polyclonal plasma cells; most of the atypical large cells did not stain for cytoplasmic immunoglobulin. In the seventh specimen many immunoblasts had cytoplasmic staining of polyclonal type. The microscopic features of the lesions differ from those of lymphomas. Surface ulceration and intralesional acute inflammatory cells and plasma cells are rarely seen in lymphomas, while a large size, deep invasion, cellular monomorphism, and prominent sclerosis were not seen in the reactive lymphoma-like lesions. Follow-up of from 6 months to 12 years was available for 10 of the 16 patients, nine of whom were treated by procedures inadequate for the cure of malignant lymphoma, and has been uneventful in all of them. The clinical and pathologic features of these cases suggest that the atypical appearances in most of them resulted from a focally florid lymphoid proliferation in association with chronic cervicitis or endometritis, or a generalized disorder of lymphoid cells such as infectious mononucleosis.

Adult↗
PubMed1985

H-Y antigen in a 46,XX female with dysgenetic ovaries.

Hypogonadism secondary to ovarian dysgenesis or resistant ovary syndrome was diagnosed in a 19-yr-old obese woman with primary amenorrhea, a 46,XX karyotype, and an H-Y+ cellular phenotype. Small ovoid gonads (1.5 X 0.6 cm) were found found bilaterally; these were encased in a dense venous network. The stroma was ovarian, and primordial follicles and some primary follicles were present, but there were no follicles at or beyond the antrum stage. There was no evidence of testicular tissue and no evidence of malignancy. Analysis of serological data indicated the possibility of residual H-Y antigen in the blood cells of the mother.

Adult↗