Konald A. Prem

Hereditary factors in the Stein-Leventhal syndrome.

Abstract Eighteen families in which the Stein-Leventhal syndrome had appeared were studied. Chromosomal analyses were made for the propositi. Clinical examinations and steroid evaluations were conducted for mothers and sisters of the propositi and for two matched control groups. Culdoscopy was performed for most of those persons showing clinical signs of the syndrome. The results permit the following conclusions: (1) The Stein-Leventhal syndrome has an hereditary basis. (2) A typical and consistent chromosomal abnormality is apparently not associated with the syndrome. (3) The transmission of the hereditary potential for the disease is consistent with a dominant mode of inheritance, probably autosomal. (4) Bilaterally enlarged cystic ovaries with pale, smooth, thickened capsules may not be an essential manifestation of the hereditary potential for the disease, but is probably the best single indication.

Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience

OBJECTIVE To present the experience of a large referral center with patients with the rare but specific syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. DESIGN, PATIENTS Between 1953 and 1991, 15 patients with the syndrome were evaluated by the authors; each patients records were carefully reviewed. MAIN OUTCOME MEASURES For each patient, the clinical course, specific anatomic findings, treatment(s) offered, and obstetrical outcome are presented. RESULTS The specificity of the association of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis was confirmed by our series, the largest in the literature to date. The findings suggest a specific development anomaly of the müllerian ductal system, probably secondary to a wolffian duct anomaly. The most common clinical presentation was that of the onset of pelvic pain and/or dysmenorrhea shortly after menarche, in association with the finding of a vaginal/pelvic mass. When a communication between the two sides existed, symptoms of abnormal vaginal discharge and bleeding were common. Optimal outcome appears to have occurred for patients initially managed using a conservative surgical approach, i.e., simple vaginal septum resection combined with conservative treatment of associated endometriosis. Obstetrical outcome in our patients was similar to that in patients with the more common combination of uterus didelphys and sagittal vaginal septum. CONCLUSION A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity.

Pretreatment surgical staging in cervical carcinoma: Therapeutic efficacy of pelvic lymph node resection

Pretreatment surgical staging in cervical carcinoma has been studied extensively to define a group for extended field radiation or adjuvant chemotherapy. A theoretical, but as yet unproved, benefit from this surgery is the resection of large, presumably radioresistant, pelvic nodal metastases before radiation therapy. One hundred fifty-six patients were divided by pelvic nodal status after surgical staging with excision of pelvic lymph nodes: group A, negative (n = 81); group B, microscopic metastases only (n = 18); group C, macroscopic nodal metastases resected (n = 48); and group D, unresectable nodal metastases (n = 9). The 5-year recurrence-free survival in group C (51%) approached that of group B (57%) and was significantly better than that of group D (0%). The groups are compared by International Federation of Gynecology and Obstetrics stage, grade, histology, and incidence of paraaortic metastases. Patterns of recurrence imply improved pelvic control in patients undergoing resection of pelvic nodal metastases. Surgical removal of pelvic nodal metastases before radiation therapy is recommended.

Postpubertal evaluation of gonadal function following cyclophosphamide therapy before and during puberty.

Evaluation of pituitary gonadotropins, gonadal steroids, spermatogenesis, and menstrual function was undertaken in 32 patients (19 males and 13 females) treated with cyclophosphamide because of nephrotic syndrome. Patients were treated before, during, or after puberty. Evaluations took place after or in very late puberty. Spermatogenic dysfunction occurred in six of 15 boys who received the entire course before and during puberty and was probably dose related. Menstrual dysfunction did not occur following treatment of six prepubertal or pubertal girls, though only low total doses were used. Therapy after puberty was associated with spermatogenic dysfunction in all four boys, but did not cause menstrual dysfunction in any of seven women. Tentative guidelines are suggested that many minimize gonadal toxicity when cyclophosphamide is used in children with nephrotic syndrome. Factors of particular importance in the interpretation of gonadotropin determinations and of sperm counts in young cyclophosphamide-treated patients are discussed.

The role of surgical debulking in cancer of the uterine cervix

From 1978 to 1985, 159 women with advanced cervical cancer received definitive radiation therapy following extraperitoneal surgical staging including pelvic lymph node dissection and periaortic lymph node sampling. Relapse-free survival was a strong function of peritoneal and nodal metastases but was independent of clinical stage. The 5-year relapse-free rate fell from 86% in women without pelvic node metastases to 0% in those with unresectable pelvic node metastases. Women with microscopically positive pelvic node metastases had virtually the same (56%) relapse-free rate as those with grossly positive but completely resected metastases (57%). The overall pelvic failure rate was 16.4% and was significantly higher in women with unresectable pelvic node metastases. Periaortic and peritoneal metastases substantially increased the probability of recurrence. Although histologic grade was prognostically significant, histopathologic category was not. Severe enteric morbidity occurred in 3.6% of patients treated solely to the pelvis and in 7.9% of patients treated to the pelvis and periaortics. Therapeutic implications of debulking pelvic node metastases are discussed.

Prognostic significance of the presence of human papillomavirus DNA in patients with invasive carcinoma of the cervix

Cases of invasive carcinoma of the uterine cervix were analyzed to determine whether the presence or absence of human papillomavirus (HPV) DNA in the neoplasms was a contributing factor to their outcome. The presence of HPV DNA was evaluated using in situ hybridization on formalin‐fixed, paraffin‐embedded tissue sections. Eighty‐five patients with cervical carcinoma who had been surgically evaluated were included in the study. Data from these patients was analyzed retrospectively to determine survival, recurrence, presence of nodal metastases, tumor grade, mode of therapy, peritoneal fluid cytologic results, and age in relation to presence or absence of HPV DNA. No significant statistical differences were found between the HPV‐16‐positive, HPV‐18‐positive, and HPV DNA‐negative patients.

Role of whole abdominal radiation therapy in the management of endometrial cancer; prognostic importance of factors indicating peritoneal metastases

From 1973 through 1983, 27 women received postoperative open-field external beam abdominal radiotherapy as primary treatment of endometrial carcinoma. The 5-year survival rate was 71%. Two distinct prognostic groups were demonstrated. Patients with spread to the adnexa, peritoneal fluid, or both, had a 5-year relapse-free rate of 90%. Patients with macroscopic spread of cancer beyond the adnexa had a 5-year relapse-free rate of 0%. Guidelines are suggested for the radiotherapeutic management of endometrial cancer metastatic to the peritoneal cavity.

Postirradiation mixed müllerian tumors of the uterus. A comparative clinicopathologic study

Nine patients with mixed müllerian tumors (MMT) of the uterus associated with a history of prior pelvic irradiation were compared clinically and pathologically with 8 nonirradiation‐associated MMT control patients. Patients with postirradiation tumors presented at a younger age and with symptoms indicative of extensive intraabdominal disease while the nonirradiation‐associated control patients were initially evaluated for abnormal uterine bleeding only. Two‐thirds of the postirradiation patients neoplasms were classified as heterologous MMT (mixed mesodermal tumor) whereas 62% of the control patients neoplasms were homologous MMT (carcinosarcoma). Regardless of clinical presentation or histologic composition, both groups fared equally poorly with average survival times of six and seven months, respectively. We were unable to substantiate that some histologic components of uterine MMT denote a poorer prognosis than others or that the postirradiation MMT behaves any differently, in the course of time, from the sporadic type. When uterine MMT is encountered in a patient under 55 years of age, the possibility that pelvic irradiation was administered some years before should be raised.

Operative treatment of adenocarcinoma of the endometrium in obese women

Abstract A series of 98 very obese patients with adenocarcinoma of the endometrium is presented in which a surgical applicability rate of 90 per cent was achieved. The results show that standard simple techniques for hysterectomy can be safely applied to this type of patient with no increase in operative mortality. The survival among these obese patients compares favorably with that among nonobese patients with the same disease and treated in the same manner.

The morbidity and utility of periaortic radiotherapy in cervical carcinoma

From 1971 through 1981, 81 women received 4350 to 5075 rad to the periaortic lymph nodes as part of their primary management for carcinoma of the uterine cervix. While two patients developed chronic small bowel damage, only one required surgical intervention. Five-year disease-free survival was 40%. Approximately one-third of the first recurrences were within the pelvic and periaortic radiation portals, with the remainder in the lungs, liver, bones, abdomen, and supraclavicular lymph nodes. Radiation dose and volume guidelines are presented in order to minimize enteric morbidity.