Gastrointestinal Stromal Tumors (GISTs) as Incidental Findings in Gynecological Surgery.

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract that may be diagnosed incidentally as a part of intra-abdominal surgery for other diseases. This is a single center review to document the incidental finding of GIST at surgery for gynecological malignancies during a 10-yr period. Sixteen cases of incidental GISTs were identified in women ranging in age from 39 to 82 yr. GISTs presented as incidental secondary lesions in women undergoing surgery for other indications, typically primary debulking surgery for tubo-ovarian high-grade serous carcinoma. The GIST was located in the stomach wall in 9 cases. Other sites were cecum, omentum, and mesentery. Diagnosis of GIST was supported by immunohistochemistry in all cases and by molecular studies in 3 cases. Seventy-five percent of cases were micro-GISTs, measuring <2 cm in diameter and, where Miettinen and Lasota criteria could be applied, fitted into "no risk," "very low risk" or "low risk" prognostic groups. Seventy-five percent of women for whom survival data was available, showed disease-free survival at follow-up. The 2 women who died had concurrent high stage or high-grade gynecological malignancy at initial diagnosis.

Micropapillary Cervical Adenocarcinoma: A Clinicopathologic Study of 44 Cases.

Micropapillary adenocarcinoma has been reported as an aggressive variant of adenocarcinoma in several organs, where it is associated with poor clinical outcome. This study reports the clinicopathologic features and outcomes of cervical adenocarcinomas with a micropapillary component (micropapillary cervical adenocarcinomas); this represents the largest reported study of these neoplasms. The study comprised 44 cervical adenocarcinomas of usual (human papillomavirus-related)-type (84%), mucinous, not otherwise specified (4.5%), gastric-type (4.5%), endometrioid (4.5%), and adenosquamous carcinoma (2%). The micropapillary component comprised >50% of the neoplasm in 34 cases (77%) (group 1), and 10% to 50% in 10 cases (23%) (group 2). Lymph node metastasis was present in 41 of 44 (93%) cases and typically the nodal tumor retained a prominent micropapillary morphology. Follow-up ranged from 7 to 123 months (mean, 65.9 mo). Seventeen of 44 (38.6%) patients had no evidence of disease on follow-up, 6/44 (13.6%) were alive with disease, and 21/44 (47.7%) died of disease. There were no survival differences between group 1 and group 2. On univariate analysis, lymph node metastasis (P=0.0015), lymphovascular space invasion (P=0.002), parametrial involvement (P=0.03), and depth of stromal invasion (P=0.045) were related to tumor recurrence. On multivariate analysis, lymph node metastasis (P=0.001), and extent of lymphovascular space invasion (P=0.027) were significant independent predictors of tumor recurrence. Our study shows that a micropapillary component in cervical adenocarcinoma may be associated with aggressive behavior and that a micropapillary architecture may occur within a variety of types of cervical adenocarcinoma.

The diagnostic conundrum of chronic ectopic pregnancy: A case report.

Chronic ectopic pregnancy (CEP) is a rare condition caused by implantation of trophoblastic tissue in the fallopian tube, which causes protracted tissue destruction at the site of attachment. The process of minor rupture and bleeding results in chronic inflammation, giving rise to a haematocele which often resembles a pelvic mass. Unlike ectopic pregnancy, the level of serum human chorionic gonadotropin (hCG) in patients with CEP is usually low or undetectable as chorionic villi are generally sparse. Therefore, CEP often poses a specific diagnostic challenge for clinicians, as both biochemical markers and imaging modalities are unreliable in its diagnosis. Nevertheless, in cases where serum bhCG is significantly elevated in the presence of a large pelvic mass, the possibility of a malignant ovarian germ cell tumour (MOGCT) must be considered and investigated appropriately. Here, we present a rare case of a young woman who was referred to a gynaecological cancer centre with an acute abdomen for the treatment of MOGCT but was subsequently diagnosed with CEP following laparotomy. In our case report, we highlight the diagnostic conundrum of CEP and MOGCT and discuss the surgical challenges both these conditions pose, especially as many of these women are young and desire fertility preservation.

CD34 and BerEP4 Are Helpful to Distinguish Basaloid Tricholemmoma From Basal Cell Carcinoma.

Tricholemmoma, a benign follicular neoplasm with outer root sheath differentiation, typically comprises clear or pale cells, and when multiple is pathognomic of Cowden's syndrome. The tumor is probably underrecognized and in basaloid examples can be difficult to distinguish from basal cell carcinoma (BCC). We studied 55 tricholemmomas (including 15 basaloid cases) and compared immunohistochemical profile with nodular BCC from our archives. Basaloid and non-basaloid tricholemmomas had similar staining characteristics. BerEP4 was focally positive (range 10%-20%) in only 3/39 (7.7%) tricholemmomas compared with widespread positivity in BCC (90.8%, 139 of 151 cases with ≥50% tumor area stained). CD34 was expressed, usually focally (median 20%, range 10%-90%), in 52/53 (98.1%) tricholemmomas and was negative in all 21 BCCs stained. EMA staining lacked sensitivity or specificity in differentiating tricholemmoma from BCC. Five or more Merkel cells were found in 7/17 (40.1%) tricholemmomas and 1/23 (4.3%) nodular BCCs studied. In summary, immunohistochemistry is helpful in distinction between tricholemmoma, including difficult basaloid examples (BerEP4 negative or focal, CD34 positive) compared with BCC (BerEP4 widespread in most cases, CD34 negative). The presence of 5 or more Merkel cells is a relatively specific but not a particularly sensitive discriminator.

Review of Lung and Pleural Biopsies Received in a Gynecologic Pathology Department Over a 14-Yr Period.

Review of pulmonary biopsies received by Birmingham Women's Hospital to identify which gynecologic tumors most commonly metastasize to lung or pleura, and which may first present with pulmonary metastases. We reviewed all pulmonary biopsies over a 14-yr period. There were 25 lung and 9 pleural biopsies, from 33 patients. Twenty-one patients had known gynecologic tumors (1 vulval, 1 cervical, 9 endometrial, 4 uterine mesenchymal, and 6 ovarian). Eighteen of the 21 biopsies had been referred from other hospitals; in 4 cases review lead to an altered diagnosis. Three of the 21 biopsies had been sent directly to Birmingham Women's Hospital. The interval between primary diagnosis and pulmonary metastasis was known in 18/21 cases and ranged from 1 to 17 yr. Nine of 21 (43%) had metastatic endometrial carcinoma; the International Federation of Gynecology and Obstetrics (FIGO) stage was known in 7/8 cases: Stage I in 5, and II and IIIA in the remaining 2 cases. Of the further 12 patients with no history of gynecologic malignancy, 4 had pleural metastases from ovarian carcinoma, 3 had primary lung carcinoma, 3 had carcinoma of unknown primary, 1 had endometrial stromal sarcoma, and 1 with a suspected Müllerian tumor was lost to follow-up. Pulmonary metastasis can occur many years after a diagnosis of gynecologic neoplasia-usually endometrial carcinoma, even after initial presentation at low stage. It may also be the initial manifestation in some cases-particularly ovarian carcinoma with pleural involvement. Specialist review of lung and pleural biopsies is important to confirm the diagnosis and optimize patient management.

Hemophagocytic lymphohistiocytosis in a patient with angioimmunoblastic lymphoma: a case report and review of the literature.

Hemophagocytic lymphohistiocytosis is a rare disorder characterized by a proliferation of phagocytic histiocytes in hematopoietic organs. It is accompanied by systemic manifestations and frequently has an abrupt onset with a fulminant clinical course and high mortality. Awareness of this condition is important since early diagnosis and initiation of treatment is critical for a successful outcome. The authors report a patient with hemophagocytic lymphohistiocytosis associated with angioimmunoblastic lymphoma, describe the clinical and histological features of hemophagocytic lymphohistiocytosis, and review the literature on this condition.

Müllerianosis of the urinary bladder.

Müllerianosis of the urinary bladder is a rare condition that encompasses 3 histological entities (endometriosis, endocervicosis, and endosalpingiosis). The authors report 2 patients with bladder müllerianosis, one of whom had endometriosis and the other a history of past pelvic surgery, describe the histological and cystoscopic features, and review the literature on this condition. Mucosal hyperemia and submucosal nodules or cysts with associated fibrosis and distortion of the bladder wall may mimic malignancy on cystoscopy, as may the infiltrative growth pattern sometimes evident histologically. Recognition of this complex diagnostic entity is important to avoid misdiagnosis and inappropriate investigation. There is a clinical association with endometriosis and past pelvic surgery.