Low-grade serous carcinoma (LGSC): A Canadian multicenter review of practice patterns and patient outcomes.

OBJECTIVE: Patients with advanced low-grade serous carcinoma (LGSC) have poor long-term survival rates. As a rare histotype, there are uncertainties regarding the use of current therapies. Thus, we studied practice patterns and treatment outcomes as part of a national initiative to better understand and improve the care of women with advanced LGSC. METHODS: This retrospective cohort study was conducted in 5 Canadian referral institutions from 2000 to 2016. Data collection and pathology reporting were standardized. Outcome measures included overall survival (OS), progression-free survival (PFS), progression-free intervals (PFI), and time to next treatment (TTNT). Cox regression analysis was used to evaluate the effects of clinical and pathologic factors on outcomes and prognosis. RESULTS: There were 134 patients (stage II-IV) with a median follow-up of 32.4 months (range 1.6-228). Four primary treatments were compared across institutions: 1) surgery followed by chemotherapy (56%), 2) neoadjuvant chemotherapy (NACT) followed by surgery (27%), 3) surgery alone (9%), and 4) surgery followed by anti-hormone therapy (4%). Primary platinum/paclitaxel chemotherapy was used in 81%. Patients treated with NACT had worse PFS. Multivariable Cox regression analysis identified lesser residual disease, younger age, and primary peritoneal origin as variables significantly associated with better OS/PFS (p < 0.03). One institution had significantly better PFS than the others (p = 0.025), but this finding could be related to a higher frequency of primary peritoneal LGSC. PFI and TTNT intervals in patients with relapsed disease were not significantly different after the first relapse irrespective of treatment type. CONCLUSIONS: There are notable differences in practice patterns across Canada. This underscores the need for ongoing strategies to measure, evaluate and achieve optimal patient outcomes for women with advanced LGSC.

Signet Ring Stromal Cell Tumor: A Legitimate (Benign) Mimic of Krukenberg Tumor.

Signet ring stromal cell tumor is a rare, benign ovarian neoplasm thought to arise from ovarian stromal cells. The pathophysiology of these tumors is poorly understood. They present in women in a wide age range, often with nonspecific symptoms including lower abdominal or pelvic pain. Their morphologic appearance raises a critical differential diagnosis of Krukenberg tumor, an aggressive malignancy with significant implications for patient management. For this reason, it is important for the pathologist to be aware of signet ring stromal cell tumor and its differentiating features, including useful histochemical and immunohistochemical ancillary tests. These tumors are curable with surgical excision, and there have been no recurrences or metastases among reported cases.

Uterine Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy).

We report a unique case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) involving the uterus. A 63-yr-old female with a history of parathyroid adenoma and cavernous sinus meningioma underwent total abdominal hysterectomy for a possible uterine malignancy. The histologic findings consisted of a nodular, mass-like infiltration of the myometrium by clusters, cords, and sheets of CD163-positve, S100-positive histiocytes with lymphocytophagocytosis (emperipolesis). The cells were negative for CD1a and langerin. Occasional plasma cells and erythrocytes were also present. Most of the histiocytes had pale, vacuolated, or foamy cytoplasm. In all cases, the nuclei were small and eccentric. No mitotic figures were identified. Two prior cases of Rosai-Dorfman disease have been reported in the female genital tract: 1 in the cervix and 1 in the bilateral ovaries. Rosai-Dorfman disease should be added to the differential diagnosis of histiocyte-rich lesions in the female genital tract. The diagnosis should be strongly considered in the presence of the characteristic histology with lymphocytophagocytosis (emperipolesis). A limited immunohistochemical panel consisting of CD163, S100, and CD1a and/or langerin will confirm the diagnosis in most cases.

Clear cell carcinoma of the female genital tract (not everything is as clear as it seems).

Clear cell carcinoma has a storied history in the female genital tract. From the initial designation of ovarian clear cell adenocarcinoma as "mesonephroma" to the linkage between vaginal clear cell carcinoma and diethylstilbestrol exposure in utero, gynecologic tract clear cell tumors have puzzled investigators, posed therapeutic dilemmas for oncologists, and otherwise presented major differential diagnostic challenges for pathologists. One of the most common errors in gynecologic pathology is misdiagnosis of clear cell carcinoma, on both frozen section and permanent section. Given the poor response to platinum-based chemotherapy for advanced-stage disease and increased risk of thromboembolism, accurate diagnosis of clear cell carcinoma is important in the female genital tract. This review (1) presents the clinical and pathologic features of female genital tract clear cell carcinomas; (2) highlights recent molecular developments; (3) identifies areas of potential diagnostic confusion; and (4) presents solutions for these diagnostic problems where they exist.

Bilateral renal sinus myelolipomas.

Adrenal myelolipomas are benign neoplasms consisting of hematopoietic cellular elements and adipose tissue. They are uncommon, found in 0.4% to 1% of the population at autopsy. Extra-adrenal myelolipomas (EM) are extremely rare with fewer than 50 cases reported. We describe the first case of bilateral EM of the renal sinus. They are difficult to diagnose on imaging alone when arising in this location and biopsies may not yield a definitive answer. Management options include both conservative and surgical approaches depending upon the certainty of the diagnosis, progression of the patient's symptoms and evidence of growth.

The vascular basis of perforator flaps based on the source arteries of the lateral lumbar region.

BACKGROUND: Perforator flaps based on the integument of the trunk have been well described in the literature; however, the anatomy of many donor sites has yet to be adequately documented. The integument of the lateral lumbar region of the trunk is supplied by a number of source arteries (lower posterior intercostal, lumbar, superior epigastric, deep inferior epigastric, superficial inferior epigastric, superficial circumflex iliac, deep circumflex iliac) whose large perforators may be suitable for perforator flap harvest. The purpose of the current study was to describe the vascular anatomy of these perforators in the lateral lumbar region. METHODS: A series of five fresh human cadavers were studied using a lead oxide-gelatin injection technique. The integument of the trunk (10 sides or hemitrunk specimens) was dissected, and the perforating vessels (diameter > or =0.5 mm) were identified, noting vascular origin, diameter, and pedicle length. Radiographs of tissue specimens were digitally analyzed using the software Scion Image for Windows (Scion Corp., Frederick, Md.) to determine vascular territories. RESULTS: The source vessels contributed a summed mean of 33 perforators per hemitrunk, with a mean emerging vessel diameter of 0.7 +/- 0.2 mm and a corresponding mean superficial pedicle length of 31 +/- 24 mm. The total area of skin supplied directly by these 33 perforators was 1200 cm2, equating to a mean area of 37 cm2 per perforator. CONCLUSION: The authors have comprehensively described the anatomy of perforators of the lateral lumbar region of the trunk.