Cotyledonoid dissecting leiomyoma of the uterus. The Sternberg tumor.

We report four cases of an unusual uterine smooth muscle neoplasm. All patients were of reproductive age, ranging from 23 to 41 years old, and had a pelvic mass or an enlarged uterus. Two also had menstrual irregularities. At laparotomy, an exophytic congested bulky tumor resembling placental tissue extended from the uterine wall into the broad ligament and pelvic cavity. In one case the tumors were bilateral but unequal in size. On gross examination, these exophytic components were the most distinctive feature. On microscopic examination the basic lesion appeared to be a dissecting leiomyoma with growth at its periphery in sinuous dissecting patterns and extensive degenerative changes. Some microscopic features, including dissecting growth patterns, distinctive connective tissue alterations, and a rich component of vessels, overlap with those seen in several leiomyoma variants, including infiltrating leiomyoma, leiomyoma with perinodular hydropic degeneration, and intravenous leiomyomatosis. An intravascular component was not a feature. The distinctive gross appearance and microscopic features are embodied in the designation, "cotyledonoid dissecting leiomyoma." The follow-up information available for three cases supports the benign nature of the tumor.

Endometriosis of the prostate.

Endometriosis occurred within the prostate of a 78-year-old man after a long course of estrogen therapy. The presence of endometrial tissue in the region of the urethral crest has potential histogenic and therapeutic implications for some of the neoplasms that arise in this area.

Partly luteinized theca cell tumor of the ovary.

The partly luteinized theca cell tumor is a variant of the theca cell tumor in which extensive foci of luteinization occur. This neoplasm belongs to a set of tumors, which includes the theca cell tumor, that is believed to be derived from mature ovarian stroma. This tumor can be classified as a tumor of specialized gonadal stroma of "ovarian cell type" intermediate between the theca cell tumor and the stromal luteoma. Two patients were virilized, and one had evidence of endometrial hyperplasia, whereas in the fourth no endocrine function was evident. None of the patients were pregnant at the time of discovery of the tumor. On gross examination these tumors consisted of a mixture of firm gray-white and yellow tissue. The presence of multiple yellow nodules in two tumors distinguished it from the usual theca cell tumor. A third tumor was distinctly lobulated. The tumor in only one of the cases arose in a background of ovarian stromal hyperplasia. Although the number of cases reported up to now is small and variability exists, the tumors tend to occur in patients in the early reproductive age group, often produce significant quantities of steroid hormones, and the clinical course has been benign.

Massive ovarian edema. A clinicopathologic study of five cases including ultrastructural observations and review of the literature.

Massive ovarian edema is a tumor-like condition occurring in young women considered to be the result of torsion of the ovary to the extent that it interferes with venous and lymphatic drainage, but is insufficient to cause necrosis. Marked enlargement of the ovary occurs, and the patient usually presents with an adnexal mass. If the torsion occurs acutely abdominal pain is prominent. If it occurs gradually, the patient may be virilized and stromal luteinization is often observed microscopically within the involved ovary. Edema fluid accumulates in the stroma but the tunica albuginea and superficial cortical zone are characteristically uninvolved. In one patient, precocious puberty was the presenting finding and this regressed following excision of the mass. This unique finding suggests that the lesion can result in the production of significant quantities of estrogen, and that this is less likely to be recognized after menarche. In the case studied by electron microscopy, the principal finding was the presence of both fibroblasts and myofibroblasts in the edematous stroma. The increased number of myofibroblasts may be a response to the edema.

Functional ovarian tumors of stromal and sex cord origin.

Most functional ovarian tumors are of specific stromal or sex cord origin, capable of differentiating in either a female direction or, less commonly, a male direction. Tumors of stromal origin such as thecoma, stromal luteoma, and Leydig cell tumors are for all practical purposes benign, and evolve from mature ovarian stroma, recapitulating common non-neoplastic transformations such as stromal changes associated with follicle development and nodular stromal hyperplasia. Sex cord tumors of granulosa or Sertoli cell types are generally of a low order of malignancy, tending to late recurrence, occasional peritoneal seeding, and only rarely to distant metastasis. Nonfunctional tumors of the ovary may trigger hormone production from adjacent reactive stroma. Massive edema of the ovary due to partial torsion may simulate neoplasm and may produce hormonal syndromes by an unknown mechanism.

Hypercalcemia and neoplasia. Biologic, biochemical, and ultrastructural studies of a hypercalcemia-producing Leydig cell tumor of the rat.

A localized, transplantable testicular tumor of the Fischer rat regularly produces hypercalcemia and increased phosphorus clearance in host animals. Light and electron microscopic examinations of the tumor indicate that it is of Leydig origin. There is no evidence that the tumor secretes any biologically active sex steroids, judges by weights of target tissues, when the tumor is grown in castrated or spayed rats. No radioactive steroid hormone formation in vitro was detected using 1-14C-acetate as a precursor although 14C was incorporated into the "C27" sterol fraction. Mass (micrograms) amounts of sex steroids were not detected after purifying large amounts of tumor extracts. The phytosterols, beta-sitosterol, stigmasterol, campesterol, were tentatively identified in tumor extracts but were also found in other tissues and in tumors not associated with hypercalcemia. Administered in vivo, human chorionic gonadotropin caused an acute rise in serum calcium in 3 to 5 hours in tumor-bearing hypercalcemic rats. Only trophic hormones with luteinizing hormone activity were found to compete with 125I-human chorionic gonadotropin for binding to the tumor homogenate in vitro indicating the tumor possessed luteinizing hormone receptors. When the tumor was transplanted intrasplenically, hypercalcemia did not occur unless adhesions formed, suggesting that the tumor hormone was rapidly metabolized by the liver and was probably of small molecular weight. Secretory granules, usually thought to be associated with peptide hormone secretion, were not detected at the ultrastructure level. Cortisol, conjugated estrogen, and an inhibitor of sterol biosynthesis (AY-9944) were effective in lowering the elevated serum calcium. Definitive identification of the agent causing lethal hypercalcemia has not been accomplished. The available data suggest it is not parathyroid hormone or vitamin D. The Leydig cell origin of the tumor, its response to human chorionic gonadotropin in vivo, the lack of secretory granules at the ultrastructural level, and biologic characteristics, all lead to the speculation that the secretory product of the tumor is a new hormonal substance, possibly a steroid precursor or related substance not previously described or is a known substance of small molecular weight whose calcium-mobilizing properties have not been fully characterized. This transplantable tumor may represent a model for one form of neoplastic hypercalcemia occurring in man and may have important implications in the general area of calcium and phosphorus homeostasis.