Nine cases of malignant mesothelioma (MM) presenting as ovarian masses occurred in female patients aged 16 to 63 (median, 52) years. In most of the cases, the referring pathologist initially misdiagnosed the neoplasm or was uncertain about its nature. In two cases, the tumors were confined to one or both ovaries, representing primary ovarian MMs; only one similar case has been previously reported. In the other cases, widespread peritoneal tumor precluded definite conclusions about the primary or secondary nature of the ovarian involvement. That at least some of the latter were also primary ovarian MMs is suggested by a degree of ovarian enlargement, the striking parenchymal replacement, or both, which are not usually seen in cases of secondary ovarian involvement by peritoneal MMs. The clinical presentation was usually that of abdominal or pelvic pain or abdominal swelling, an adnexal mass on pelvic examination or at laparotomy, or combinations thereof. One tumor was an autopsy finding. There was no history of asbestos exposure in any patient. Eight patients underwent bilateral oophorectomy, usually with hysterectomy and biopsies of extraovarian tumor. Four patients were given chemotherapy and one, radiation therapy. Follow-up in five cases revealed that three patients had died of tumor at postoperative intervals of 8 to 44 months, one was alive with persistent tumor at 18 months, and one was alive with no clinical evidence of tumor at 11 years. The ovaries were replaced by tumors 3 to 15 cm in maximum diameter; seven were bilateral. The neoplastic tissue was typically solid, but small cysts were present in two cases, and one tumor was a unilocular cyst with a solid mural nodule. On microscopic examination, tumor involved both the serosa and the parenchyma of the ovary in seven cases, the serosa only in one case, and the parenchyma only in one case. Seven tumors were exclusively epithelial, with papillary, tubular-glandular, and solid patterns, and two were biphasic. The cells in the epithelial mesotheliomas usually exhibited moderate atypicality and a low mitotic rate. The stroma was typically hyalinized, and in three of the cases with a papillary pattern papillae with hyalinized cores were a striking finding. Psammoma bodies were present in three cases. Histochemical and immunohistochemical stains confirmed the mesothelial nature of the tumor cells. Because of the wide variety of microscopic patterns in MMs, the differential diagnosis of ovarian MM includes a variety of primary and metastatic ovarian tumors as well as other peritoneal mesothelial lesions.
Six tumor-like glandular lesions characterized by a prominent component of endocervical-type epithelium involved the wall of the urinary bladder in women of reproductive age (31 to 44 years; mean, 37). All of the lesions posed problems in histologic diagnosis; indeed, a diagnosis of adenocarcinoma was initially rendered in three cases. Five patients presented with bladder symptoms, including—alone or in combination—suprapubic pain, dysuria, frequency, and hematuria. There was catamenial exacerbation of the symptoms in one case. The sixth patient—the only one with documented pelvic endometriosis—presented with dysmenorrhea, dyspareunia, and lower abdominal tenderness. In each patient, a mass that ranged from 2 to 5 cm in maximum dimension was typically located in the posterior wall or posterior dome of the urinary bladder. A partial cystectomy (five cases) or transurethral resection (one case) was performed. In one patient, extravesical pelvic soft tissue was involved, precluding complete resection of the lesion. Microscopic examination revealed extensive involvement of the bladder wall by irregularly disposed, benign-appearing, or mildly atypical endocervical-type glands, some of which were cystically dilated. Other findings included occasional ciliated cells, typically interspersed with the endocervicaltype cells (four cases), a minor component of endometrioid glands (three cases), and glands lined by nonspecific cuboidal or flattened cells with eosinophilic cytoplasm (all cases). Some of the glands were surrounded only by the smooth muscle of the muscularis propria, but in other areas, the periglandular tissue was fibrous or edematous. In three cases, rare glands were surrounded by thin rims of endometriotic stroma. Gland rupture resulted in stromal extravasation of mucin in all cases and was a prominent feature in one. All patients had uneventful postoperative follow-up periods ranging from 1.5 to 14 years. The findings indicate that these bladder lesions are müllerian in nature and represent examples of endocervicosis, the mucinous analogue of endometriosis. Awareness of the lesion, which with one possible exception is hitherto undescribed in the bladder, and attention to its typical histologic features should facilitate its crucial distinction from adenocarcinoma.
Previous studies have indicated that mucinous carcinomas of the ovary associated with extraovarian spread at the time of presentation or follow-up almost always have extensive infiltrative invasion within the primary tumor. We present four cases of stage I ovarian mucinous tumors that lacked extensive infiltrative invasion but were associated with an unexpectedly aggressive behavior. The patients were 18, 20, 41, and 45 years of age at presentation. All four of them presented with an abdominal mass or increased abdominal girth. The tumors were all stage Ia, 17 to 37 centimeters in maximal dimension, and typically multicystic with solid areas. The number of blocks per centimeter of tumor diameter was 0.65, 0.88, 0.92, and 1.0 in the four tumors, respectively, a degree of sampling within that recommended in previous studies. Clinical findings supported that they were primary tumors rather than metastatic from an occult primary tumor. On microscopic examination, the tumors all contained foci of intraepithelial carcinoma and foci of invasion as follows: expansile invasion only (two cases), expansile invasion and microinvasive carcinoma (one case), and microinvasive carcinoma only (one case). The expansile invasion was extensive in each of the three cases in which it was present. On follow-up, each patient experienced recurrent disease 7 months to 4.5 years after diagnosis, including hematogenous spread to lung and/or bone and liver in three patients. Three of four patients developed intraperitoneal spread. Three of four patients died of disease, and one patient is alive with persistent disease. Although ovarian mucinous tumors with only expansile invasion or only microinvasive carcinoma are usually associated with an excellent prognosis, this study indicates that these tumors can rarely behave in an aggressive fashion with hematogenous spread and a fatal outcome. Some of these tumors may have contained unsampled foci of infiltrative invasion. Although the optimum level of sampling in mucinous tumors remains to be determined, we recommend additional sampling of tumors in which the initial sections reveal intraepithelial carcinoma, microinvasive carcinoma, expansile invasion, or combinations thereof.
Uterine tumors composed of a prominent component of smooth muscle (SM) and endometrial stroma (ES) (so-called stromomyomas) have received little attention in the literature. The features of 15 of these tumors, defined as those containing more than 30% of each component, were evaluated. Many of the tumors were referred because of problems in the differential diagnosis. Patient age ranged from 29 to 68 years (mean, 46 years). The tumors ranged from 3 to 27 cm (average 9.6 cm) in diameter, and most were grossly well circumscribed. The sectioned surfaces often had soft, tan-yellow areas admixed with firm, whorled areas. Microscopic evaluation disclosed that nine tumors were well circumscribed, and six had infiltrating tongues typical of endometrial stromal sarcoma (ESS). The endometrial stromal component, which predominated in five cases, typically was characterized by a diffuse growth of closely packed, minimally atypical small cells accompanied by numerous arterioles and was desmin-negative in all cases tested, except for rare desmin-positive cells in three tumors. Five tumors showed sex-cord-like differentiation in these areas. The smooth muscle component, which predominated in seven cases, was composed predominantly of spindle cells in disorganized short fascicles, longer fascicles, or nodules with prominent central hyalinization. This component appeared benign, except in one case with moderate cytologic atypia, focal tumor cell necrosis, and 4 mitotic figures/10 high-power fields. The smooth muscle component was strongly desmin-positive in all the tumors tested. Follow-up of more than 1 year was available for seven patients. Six patients were alive and well, but one tumor with infiltrative borders recurred at 48 months as a pure endometrial stromal sarcoma. Mixed endometrial stromal and smooth muscle tumors should be distinguished from highly cellular leiomyomas, pure endometrial stromal tumors, and "uterine tumors resembling ovarian sex cord tumors," at least until knowledge of their clinicopathologic features is more complete. For treatment purposes, these tumors should be reported as endometrial stromal nodules or as endometrial stromal sarcomas with smooth muscle differentiation and any unusual features of either component recorded in a notation.
We report 13 cases of inflammatory pseudotumor of the urinary bladder in patients having no history of recent local trauma. The average age of the patients (eight females, five males) was 35.4 years (range, 19 to 60 years). Gross hematuria (nine of 13 cases) and recurrent cystitis (three of 13 cases) were the most common presentations. Cystoscopy and gross examination revealed either a polypoid intraluminal mass or a submucosal mural mass, ranging in size from 2 to 7 cm. The lesions were commonly gelatinous. Histological examination showed that the lesions consisted of spindle cells with tapering eosinophilic cytoplasm, typically widely separated in a vascular myxoid matrix with acute and chronic inflammatory cells. In four cases the lesions had more compact cellularity with areas of fibrosis and less myxoid change. The muscularis propria was involved in 10 cases, the perivesical fat in two cases. The spindle cells were immunoreactive for vimentin (10 of 10) and muscle-specific actin (10 of 10). A few cases exhibited immunoreactivity for smoothmuscle- specific actin (three of eight), cytokeratin (two of 10), desmin (two of nine), and epithelial membrane antigen (two of eight). Ultrastructural examination of four cases revealed myofibroblasts, fibroblasts, or a mixture of the two cell types. DNA content analysis by flow cytometry yielded diploid histograms (six of six). Clinical follow-up in all cases demonstrated no evidence of recurrence (mean follow-up, 25.8 months). The findings indicate that this lesion is a benign, likely inflammatory or reparative, mesenchymal lesion that can be recognized by its distinctive pathological features
Thirty-three highly cellular leiomyomas of the uterus from patients 29 to 65 (mean, 46) years of age and six endometrial stromal nodules from patients 41 to 53 (mean, 46) years of age are described. The patients usually presented with irregular uterine bleeding. Twenty-eight of the leiomyomas were intramural and five, submucosal. Eleven of 18 whose consistency is known were soft, fish-flesh, or rubbery, 10 were yellow or yellow-tan; one had a prominent cystic component. The tumors ranged from 0.5 to 15 cm (mean, 4.6 cm) in maximum dimension. On microscopic examination, they were densely cellular and composed of cells that ranged from round to spindle-shaped and had scanty cytoplasm. A focal fascicular pattern was present in all of the cases. Blood vessels were typically large; thick muscular walls and focally dilated lumens were a conspicuous feature of the majority of the neoplasms. Arterioles were evident focally in most of the tumors but were prominent in only one of them. Cleft-like spaces, some representing compressed vessels, others due to internodular edema, were present in 24 of the tumors and were conspicuous in 15 of them. The neoplasms typically exhibited focal irregular extension into the adjacent myometrium, and this feature was conspicuous in 18 of them. They often merged almost imperceptibly with the adjacent myometrium. All tumors were immunoreactive for desmin and 32 of 32 for alpha-smooth muscle actin. Two of the stromal nodules were polypoid intracavitary masses, three were submucosal, and one intramural. Five were completely or predominantly solid with cysts present focally in three of them; one tumor was predominantly cystic. The solid tissue was frequently yellow. Microscopic examination disclosed a diffuse growth of closely packed small cells with scanty cytoplasm and nuclei that lacked atypia. Three neoplasms contained cells with abundant foamy cytoplasm that were immunoreactive for CD68, indicating that they were histiocytes and not neoplastic cells. All the neoplasms had a prominent component of arterioles, which in one tumor had hyalinized walls. Five tumors were entirely well circumscribed and one predominantly well circumscribed with limited extension into the adjacent myometrium. The tumors were immunohistochemically negative for desmin.(ABSTRACT TRUNCATED AT 400 WORDS)
Summary We studied four endometrial carcinomas with a conspicuous component that resembled patterns in Sertoli cell tumors. The patients presented at age 44–83 years (mean 65 years), with abnormal or postmenopausal vaginal bleeding in three and abnormal cervical cytology in one. All were multiparous, moderately to markedly obese, and hypertensive, and three patients had noninsulin-dependent diabetes mellitus. One tumor was suspected to be an endometrial stromal sarcoma with sex-cord-like differentiation on biopsy. Gross examination of the hysterectomy and bilateral salpingo-oophorectomy specimens showed solid polypoid endometrial tumors in each case. Light microscopic examination showed three to be superficially invasive of the myometrium and one to be confined to the endometrium; none of the tumors showed the tonguelike pattern of myoinvasion or the angiolymphatic invasion characteristic of low-grade endometrial stromal sarcomas. The sertoliform component, which predominated in one case and was only focal in the three others, was composed of uniform small hollow tubules lined by columnar cells with apical cytoplasm and of compact slender cords. The tubules and cords were often present between benign-appearing or carcinomatous glands. In the case with predominant sertoliform areas, the lesional cells had clear cytoplasm suggesting a lipid-rich variant; special stains of this case demonstrated cytoplasmic glycogen but no fat. In none of the cases was cytoplasmic mucin, argyrophil granules, or argentaffinity demonstrated. The nonsertoliform areas of the tumors consisted of typical endometrioid adenocarcinoma; concurrent endometrial hyperplasia was also present in each case. Squamous differentiation and minor foci of anaplastic carcinoma with bizarre tumor giant cells were present in three tumors. Immunoperoxidase stains showed staining for two or more markers of epithelial or glandular differentiation in the sertoliform areas in all cases (keratin, epithelial membrane antigen, carcinoembryonic antigen, CA125, TAG72), with focal expression of vimentin in all cases. In none of the cases was desmin or actin staining observed. The evidence indicates that tumors in this series are variants of endometrioid adenocarcinoma and are distinct from uterine tumors resembling ovarian sex-cord tumors and stromal sarcomas with sex-cord-like differentiation.