Radical hysterectomy and pelvic lymphadenectomy for stage IB carcinoma of the cervix: 21 years experience.

From September 1971 through December 1982, 153 patients with Stage IB carcinoma of the cervix underwent radical hysterectomy and pelvic lymphadenectomy at two of the teaching hospitals of the Uniformed Services University of the Health Sciences. Records were retrospectively analyzed and independent pathologic review was performed. All surgical procedures were performed by fellows or senior residents under the direct supervision of the gynecologic oncology staff of the Walter Reed Army Medical Center or the Naval Hospital, Bethesda, Maryland. In this series, IB carcinoma was defined as squamous carcinoma clinically confined to the cervix with invasion greater than 5 mm from the basement membrane or any adenocarcinoma confined to the cervix. The average age of the patients was 38.3 years. The histologic types were squamous in 72%, adenocarcinoma in 16%, and adenosquamous in 10.5%. The mean operating time was 5 hr and 40 min with an average blood loss of 1800 cc. There were two ureterovaginal and two vesicovaginal fistulae for an overall fistula rate of 2.6%. Actuarial survival for these 153 patients is 84%. This extends the previous series of R. C. Park, W. E. Patow, R. E. Rogers, and E. A. Zimmerman, Obstet. Gynecol. 41, 117-122 (1973) of 122 cases collected from 1961 to September 1971 to 275 cases. In comparing the two time periods, no significant differences were found in operative technique or complications, but there was a change in the incidence of adenocarcinoma and mixed cell types and a difference in survival. A relatively higher incidence of more aggressive tumors may indicate the need for different therapeutic approaches in the future.

Prognostic factors associated with radical hysterectomy failure.

Two hundred seventy-five patients who underwent radical hysterectomy and pelvic lymphadenectomy for FIGO stage IB carcinoma of the cervix between 1961 and 1982 were retrospectively analyzed to identify specific risk factors associated with treatment failure. Patients were classified as high or low risk on the basis of tumor spread to pelvic lymph nodes or surgical margins. Thirty-eight patients had tumor involvement of pelvic nodes or surgical margins. Despite postoperative whole pelvis radiation therapy in 88% of patients, 13 (34.2%) developed recurrence. All patients with involved nodes or margins who recurred died of disease. Patients with pelvic lymph node or surgical margin involvement clearly constitute a high risk group and should be considered candidates for some form of adjuvant therapy. Two hundred thirty-seven patients had negative nodes and clear surgical margins. There were 18 recurrences (7.6%) in this group. Pathologic specimens were reviewed to evaluate additional histologic criteria which might identify those patients at greatest risk for tumor recurrence in this low risk group. Patients whose tumors contained vascular-lymphatic space invasion or adenomatous histologic components recurred more frequently than patients whose tumors did not (P less than 0.05). Eighty-three percent of low risk patients who recurred had tumors with at least one of these features. Degree of differentiation and depth of invasion did not correlate with risk of recurrence. Prospective randomized trials are needed to determine the effectiveness of postoperative adjuvant therapy for patients at risk for recurrent disease.

Short-term responses in neonatal lambs after infusion of group B streptococcal extract.

Short-term (0 to 30 minutes) physiologic responses of neonatal lambs infused with a trichloroacetic extract of a type III (strain 878) group B streptococcus (878-TCA) were studied. Bolus injections of 878-TCA were associated with pulmonary hypertension, peripheral arterial hypoxemia, and reductions in circulating white blood cell and platelet counts. These events were associated with a rise in plasma levels of prostaglandins F2 alpha and E and could be prevented by proper treatment with ibuprofen. Continuous infusions of 878-TCA were associated with a dose-dependent rise in systemic and pulmonary arterial pressures and a fall in arterial PO2. During infusion, inhibition of prostaglandin synthesis resulted in a return toward preinfusion values. The authors conclude that venous infusions of extracts of 878-TCA induce significant pulmonary and systemic arterial vascular perturbations in the neonatal lamb and that some of these alterations are associated with the release of prostaglandins or other arachidonic acid metabolites.

Endotoxemia in the neonatal lamb.

Although gram-negative sepsis is a major cause of neonatal morbidity and mortality, our understanding of endotoxemia in the neonate has been hampered by the lack of experimental models. Previous studies have suggested neonatal hyporesponsiveness to endotoxin. We studied unanesthetized neonatal lambs which had been exposed to the environment prior to study. These animals demonstrated the classic early phase changes of endotoxemia including pulmonary hypertension which was dependent upon prostanoid production. This model allows further studies of endotoxemia in the neonate.

Whole abdominal and pelvic irradiation in patients with minimal disease at second-look surgical reassessment for ovarian carcinoma.

Aggressive cytoreductive surgery followed by combination chemotherapy for stage III ovarian carcinoma has resulted in a significant percentage of complete clinical responses. However, 30-50% of patients with no clinical evidence of disease are found to have residual carcinoma at second-look surgical reassessment. Because recent reports have indicated a high degree of effectiveness utilizing abdominal and pelvic irradiation as primary therapy for ovarian carcinoma with small residual disease, the authors treated eight patients found to have residual disease of less than 1 cm at second-look reassessment with either open field or split field abdominal and pelvic irradiation. All eight patients had initially undergone aggressive cytoreductive surgery and seven of the eight patients had received multidrug chemotherapy. Patients were treated either at the National Cancer Institute or the Naval Hospital Bethesda both with and without intraperitoneal radiation sensitizers. Fifty percent of the patients required early termination of therapy due to myelosuppression. All eight patients have recurred and three have died. Six of the eight patients have required major surgical procedures for gastrointestinal complications. Based on this experience, we cannot advocate this form of therapy in patients with minimal residual ovarian carcinoma following second-look surgical reassessment.

Serial transplantation of a heterologous, mixed Müllerian tumor in the nude (athymic) mouse: growth characteristics and morphology.

In view of the poor prognosis for patients with mixed Müllerian tumors, transplantation of these tumors into nude mice offers an important model for the study of the physiologic processes occurring in this type of malignancy. We report the growth and serial transplantation of all elements of this tumor in the nude mouse. The lag period for establishment of the original tumor was 5 weeks. The tumor required 11 weeks to achieve a 2 X 2 X 1 cm volume at the initial pass, but on subsequent passes reached this volume in 6-12 weeks (mean = 8.3). Microscopic examination revealed that the epithelial, stromal and heterologous elements were serially transplanted. An osteoid matrix, confirmed by special staining for bone, was identifiable at both the light and electron microscopic levels. Throughout successive passages, the tumor retained the morphologic features of the original tumor. This tumor line is stable and reproducible, providing a readily available source of this ovarian carcinoma for clinical research studies.

A unique case of Sertoli cell only syndrome with normal gonadotropins.

A 23-year-old male presented with primary infertility, normal male phenotype, and azoospermia. He had normal basal T, FSH, and LH levels and responded normally to clomiphene citrate stimulation. He also had normal androgen receptors in cultured pubic skin fibroblasts. A testis biopsy showed only Sertoli cells and no evidence of seminiferous tubule damage, lacking the fibrosis or Leydig cell hyperplasia usually seen in SCOS. This case of SCOS, combined with those previously reported, suggests that the etiology of SCOS is heterogeneous, with a single common end point, or that it is a single process that has been studied at different developmental stages by different investigators.

Pulmonary blastoma. Case report and literature review of chemotherapy experience.

Pulmonary blastoma is a rare primary lung neoplasm, occurring in both children and adults, which is pathologically, clinically, and prognostically distinct from other lung tumors. Usually it has been treated with surgery, but both chemotherapy and radiotherapy have been used for metastatic disease, and in the adjuvant setting. A patient is described who presented with metastatic pulmonary blastoma. Treatment with cyclophosphamide, vincristine, doxorubicin, and dactinomycin resulted in an objective response as judged by standard criteria. The literature is reviewed for other experience with chemotherapy in this rare lung tumor. This four-drug combination appears to show promise for tumor response, and is deserving of further trial.

Focal dermal hypoplasia: ocular manifestations in a male.

Focal dermal hypoplasia (Goltz Syndrome) is a rare congenital disorder resulting from ectodermal and mesodermal dysplasia. It involves ocular tissues in over 40% of cases. Considered to be X-linked dominant, the disorder characteristically occurs in females. This case represents, however, the ninth male affected by this disease to be reported in the literature. Ocular abnormalities included colobomatous microphthalmia, aniridia, and recurrent papillomas arising from the conjunctiva and lid margins. Both light and electron microscopic studies performed on papillomatous tissue failed to demonstrate the presence of viral particles. Prometaphase chromosome analysis performed on peripheral blood cells, and on papilloma cells grown in tissue culture, showed a normal male karyotype of 46, XY.

Cysts containing renal cell carcinoma in von Hippel-Lindau disease.

We report on a patient with von Hippel-Lindau disease. Exploration revealed 15 solid tumors and 18 cysts filled with clear fluid throughout both kidneys. All solid tumors consisted of clear cells consistent with renal cell carcinoma. All cysts were lined with similar clear cells. The solid tumors contained areas of clear cells surrounding small cystic spaces and the large cysts appeared to be an exaggeration of this phenomenon. There is a place for radical ablative surgery in patients with multiple renal cell carcinomas in von Hippel-Landau disease. Unroofing and gross examination of apparently simple cysts may not be adequate to rule out the presence of carcinoma in these patients.

Benign clinical behavior of immature mediastinal teratoma in infancy and childhood: report of two cases and review of the literature.

Germ cell tumors of the mediastinum can be divided into three categories: 1) mature teratomas which have all elements at a mature level; 2) immature teratomas which are similar to the mature teratomas, but also contain immature epithelial or mesenchymal elements or blastema; and 3) embryonal tumors which contains elements which are recognized as dysgerminoma, embryonal carcinoma with or without yolk sac elements, and choriocarcinoma. Immature teratomas are the rarest type, accounting for only about 1% of mediastinal teratomas. Two cases of immature teratoma of the mediastinum occurring in infants are reported. One tumor was completely excised. The child is well without evidence of disease two years later. The other immature teratoma was unresectable. Biopsy showed it to be similar to the teratoma that was excised. No postoperative treatment was given. The tumor has not changed appreciably in size, but the child has grown normally for six years so that the tumor mass, which initially filled his chest, is now evidenced as mediastinal widening. A review of the reported cases of immature teratomas in the mediastinum shows that the prognostic value of the histologic appearance of these tumors has not been developed to the same degree as it has for teratomas in the ovary or the sacrococcygeal region. The immature teratomas that occur in infants behave as mass lesions as do the mature teratomas. Immature teratomas in the mediastinum of children in their late teens and in young adults behave as highly malignant tumors similar to the embryonal carcinomas.

Elastofibroma dorsi: an immunochemical study of collagen content.

The types of collagen present in a case of elastofibroma dorsi were determined using type specific, characterized collagen antibodies. The presence of type II collagen (normally present only in articular cartilage and in selected ocular structures) is discussed with regard to the pathogenesis of this lesion, and the use of collagen antibodies is discussed with regard to their potential value in better characterizing and classifying mesenchymal tumors.

Colonic ganglioneuroma.

Ganglioneuromas are neuroectodermal tumors that are found in diverse anatomic sites, but they are very uncommon in the colon. We observed a patient with partial colonic obstruction whose roentgenogram had a napkin-ring construction that was thought to be carcinoma. On resection, pathologic examination showed a ganglioneuroma. Mesenchymal neoplasms of various histologic types occur thoughout the gastrointestinal tract. Neurogenic tumors are not uncommon in Von Recklinghausen's neurofibromatosis and multiple mucosal neuroma syndrome, but solitary lesions unassociated with these conditions are rare. To our knowledge, this complication in the colon is only the second such reported case. We believe that this case is noteworthy because of its clinical and roentgenographic resemblance to carcinoma.

Adenosarcoma of the uterus: ultrastructural observations.

Müllerian adenosarcoma has been recently described as a distinctive biphasic uterine tumor with benign epithelial and malignant stromal components. The lesion belongs generally to the group of mixed müllerian tumors, but the benignity of the epithelium separates it from the other neoplasms of this type. This entity usually affects postmenopausal patients who follow a protracted course. This paper reports a case fulfilling the original clinical and pathologic description of Clement and Scully and adds ultrastructural observations. Electron microscopic examination confirmed the optical impression of a biphasic neoplasm with benign epithelial and malignant stromal components. The epithelium resembled proliferative endometrium, and the stroma had features of endometrial stromal sarcoma or mixed müllerian tumor. The findings of stromal resemblance to endometrial stromal sarcoma or mixed müllerian tumor and the presence of a variety of müllerian epithelia support the proposed müllerian derivation of this lesion.