Synchronous Low-grade Appendiceal Mucinous Neoplasm and Primary Peritoneal Low-grade Serous Carcinoma: A First Description of These 2 Neoplasms Presenting Together as Suspected Peritoneal Carcinomatosis.

Low-grade appendiceal mucinous neoplasm is a neoplasm typically of appendiceal origin, which is characterized by diffuse peritoneal involvement by pools of mucin with mucinous epithelium lacking high-grade cytologic atypia, and clinically presents as suspected peritoneal carcinomatosis. A similar clinical presentation can sometimes be seen with disseminated low-grade serous carcinomas of the peritoneum, fallopian tubes, or ovaries; however, this neoplasm is histologically characterized by tubal-type epithelium and invasive or confluent growth. In this case report, we describe a patient presenting with a clinical examination and radiologic features suggestive of peritoneal carcinomatosis and a prominent pelvic mass; however, after pathologic review, the patient was proven to have peritoneal involvement by both low-grade appendiceal mucinous neoplasm of appendiceal origin and a low-grade peritoneal primary serous carcinoma. In short, we present the first description of low-grade appendiceal mucinous neoplasm and serous carcinoma of the peritoneum presenting synchronously, providing morphologic characterization and immunohistochemical studies supporting the diagnosis, and illustrating a rare instance in which 2 neoplastic processes are underlying clinically suspected peritoneal carcinomatosis.

IgG4-related Disease of the Ovary: A First Description.

IgG4-related disease has been previously described to involve numerous organs and anatomic sites, however, involvement of the ovary has not yet been reported in the literature. In this case report we describe an ovary involved with an inflammatory process with histopathologic features supportive of involvement by IgG4-related disease: a dense lymphoplasmacytic infiltrate with an eosinophilic component, obliterative phlebitis, and a prominent proportion of IgG-positive cells with IgG4 expression by immunohistochemistry (40%-50%). The differential diagnosis for this case would include eosinophilic perifolliculitis involving the ovary, another rare entity that shares the eosinophilic component of IgG4-related disease. In short, we present the first description of IgG4-related disease of the ovary providing morphologic characterization and immunohistochemical studies supporting the diagnosis.

Cytohistopathologic correlation of ovarian mesonephric-like carcinoma and female adnexal tumor of probable Wolffian origin.

Recently we encountered two cases with mesonephric features, mesonephric-like carcinoma (MLC) of the ovary, and female adnexal tumor of probable Wolffian origin (FATWO). They are thought to be related to mesonephric remnants (or Wolffian duct remnants). Herein we describe the cytohistolgical features, differential diagnoses, and potential pitfalls in diagnosis of these neoplasms. On cytological examination, the case of MLC showed tight 3-dimensional clusters of overlapping round cells, corresponding to solid growth pattern seen on histological examination. Tubular architecture and papillary formations composed of neoplastic cells of medium size with scant cytoplasm were readily identified. Intraluminal eosinophilic secretions were better seen on histological examination. Additionally, areas resembling features of papillary thyroid carcinoma were noted. Mitoses and apoptotic bodies were not identified on cytology but seen on histological sections. The neoplastic cells were positive for CK7, CD10, PAX-8, TTF-1, and GATA-3, and negative for ER, PR, and WT-1 immunostains. In contrast to MLC, cytological examination of FATWO showed smaller oval to spindle monotonous cells without mitotic figures. Some cells contained paranuclear vacuoles and were arranged individually or in loose cohesive clusters. Other cells were closely associated with pericellular hyalinized basement membrane-like material and they were arranged in cohesive clusters as well. On histological examination, similar to MLC, the FATWO had areas with thyroid-like features, such as, intraluminal eosinophilic secretions, paranuclear vacuoles, in the background of collagenous stroma. The neoplastic cells were positive for CK AE1/AE3, calretinin, WT-1, inhibin, and CD10, and negative for CK7, PAX-8, GATA-3, ER, PR, and C-kit immunostains.

Sixteen year-old with leiomyosarcoma in a prior benign myomectomy site.

Uterine leiomyosarcoma in a prior myomectomy site is a rare phenomenon. We report an unusual case of a leiomyosarcoma arising six months post myomectomy in a 16-year old female.

Disseminated peritoneal leiomyomatosis postmorcellated resection of uterine leiomyomatous tissue.

In a retrospective review, we identified six cases of disseminated peritoneal leiomyomatosis (DPL) that occurred after resection for uterine leiomyoma(ta) using a morcellation procedure between 2010 and 2016. DPL occurred in less than 1% of all patients who underwent a prior hysterectomy with morcellation, and DPL never occurred without having underwent such a resection. The median age of women at the time of their original resection of uterine tissue was 38.6 years; the median time interval until resection of DPL after the primary morcellation procedure was 73 months and the median age was 48 years. At the time of DPL resection, a median of 6.5 individual lesions was present per patient, with each lesion having a median size of 1.2 cm in the greatest dimension. The most common peritoneal sites of involvement included the sigmoid colon serosa, right pelvis/pelvic side wall, and anterior abdominal parietal peritoneum. The same parameters are described for previously reported cases of DPL in the literature developing after a morcellated resection of uterine leiomyoma(ta). The use of morcellating hysterectomy specimens with leiomyomata may lead to the development of DPL by seeding, may involve numerous peritoneal sites, and often presents 2 years after the original resection.

Calretinin, a more sensitive but less specific marker than alpha-inhibin for ovarian sex cord-stromal neoplasms: an immunohistochemical study of 215 cases.

Although inhibin has been shown to be a sensitive marker for ovarian sex cord-stromal and fibrous neoplasms, it may be negative in some cases. Calretinin, a mesothelial marker, has shown promise as a marker for sex cord-stromal neoplasms. The aim of this study was to evaluate and compare calretinin and inhibin as immunohistochemical markers for sex cord-stromal and fibrous neoplasms. A total of 215 ovarian neoplasms were immunostained with commercially available antibodies to calretinin and inhibin. These tumors included 87 sex cord-stromal (39 granulosa cell, 13 Sertoli-Leydig, 4 Sertoli, 9 thecomas, 14 fibrothecomas, and 8 other stromal tumors), 37 fibrous (20 fibromas, 9 adenofibromas, and 8 fibrosarcomas), 65 epithelial, 22 germ cell, and 4 miscellaneous neoplasms. The staining was evaluated on a 0-4 scale based on percentage of neoplastic cells labeling: 0 = none; 1+ = 1-25%; 2+ = 26-50%; 3+ = 51-75%; 4+ = 76-100%. Calretinin reactivity was detected in 100% of sex cord-stromal and 90% of fibrous neoplasms, including 32 that were inhibin negative (2 granulosa cell tumors, 1 Sertoli-Leydig cell tumor, 1 thecoma, 3 fibrothecomas, 16 fibromas, 6 adenofibromas, and 3 fibrosarcomas). All four calretinin-negative fibrous neoplasms were inhibin negative. Calretinin staining was also detected in 22% of epithelial neoplasms but none of the germ cell and miscellaneous neoplasms tested. Inhibin staining was detected in 92% of sex cord-stromal neoplasms, 22% of fibrous neoplasms, 2% of epithelial neoplasms, and none of the germ cell and miscellaneous neoplasms tested. Calretinin has a 97% sensitivity and 85% specificity for sex cord-stromal and fibrous neoplasms, whereas inhibin has a 71% sensitivity and 99% specificity. This study shows that both calretinin and inhibin are useful in the diagnosis of ovarian sex cord-stromal and fibrous neoplasms. Calretinin is a more sensitive but less specific immunohistochemical marker than inhibin. Calretinin is particularly useful in the diagnosis of sex cord-stromal and fibrous neoplasms that are inhibin negative. The high frequency of calretinin in fibrous neoplasms suggests that a subgroup of these neoplasms may be derived from specialized gonadal stromal cells, perhaps thecal cells.

Primitive neuroectodermal tumors of the pancreas: a report of seven cases of a rare neoplasm.

Primitive neuroectodermal tumors (PNETs) have rarely been described in solid organs. We report a series of seven PNETs of the pancreas. The clinical, gross, microscopic, and immunohistochemical features of these seven PNETs of the pancreas are described, as are the genetic analyses in five cases. The patients ranged in age from 6 to 25 years (mean 18 years). Four of the patients were male. All of the patients presented with jaundice and/or abdominal pain. All of the tumors were located in the head of the pancreas, and they ranged in size from 3.5 to 9.0 cm. Light microscopy revealed the typical morphologic features of PNETs. By immunohistochemistry the neoplastic cells in all seven cases expressed O13 (CD99, p30/32MIC2). In five of six tested cases, the neoplastic cells also expressed cytokeratin. All of the tumors expressed neural-neuroendocrine markers. Two of the three cases examined ultrastructurally showed prominent epithelial features. There was cytogenetic or molecular genetic evidence of the t(11;22)(q24;q12) in four of five cases examined. Clinical follow-up was available in five cases. Two of the patients were alive with no evidence of disease at 33 and 43 months. One patient was alive with disease at 27 months. One patient died of postoperative complications. Another patient died of disease 4 years after diagnosis. PNET can sometimes arise as a primary neoplasm of the pancreas. Like PNETs arising in more conventional sites, pancreatic PNETs occur in the pediatric and adolescent population, show the characteristic staining with O13, and typically harbor the t(11;22)(q24;q12) chromosomal translocation. PNETs should be included in the differential diagnosis of poorly differentiated small round cell tumors of the pancreas. Moreover, they should not be confused with pancreatic endocrine tumors, which also demonstrate dual epithelial and neuroendocrine differentiation by immunohistochemistry and express O13 in 30% of cases.

The College of American Pathologists and National Society for Histotechnology workload study.

Limited data exist in regard to productivity and staffing in the anatomic pathology laboratory. In 2004, the National Society for Histotechnology (NSH) conducted a pilot study to examine productivity and staffing in the histology laboratory. After review of the data, The College of American Pathologists (CAP)/NSH Histotechnology Committee concluded that a larger survey was required to further address and expand on the pilot study findings. In 2007, a total of 2674 surveys were sent out to North American laboratories. From the responses, comparisons of laboratory demographics and productivity were examined by institution type and workload volume. Productivity was measured as the number of paraffin-embedded tissue blocks processed per full-time equivalent per year. This manuscript presents and discusses the data collected from the CAP/NSH Workload Study.

Estrogen receptor alpha and progesterone receptor expression in ovarian adult granulosa cell tumors and Sertoli-Leydig cell tumors.

The biologic role that estrogen receptor (ER) and progesterone receptor (PR) play in ovarian sex cord-stromal tumors is poorly understood. Furthermore, immunohistochemical data on these hormone receptors in this group of neoplasms are limited and conflicting, with many reports suggesting that expression of ERalpha and/or PR is either infrequent or present at low levels in granulosa and Sertoli cell tumors. Immunohistochemical staining for ERalpha and PR was performed in 69 ovarian sex cord-stromal tumors: 41 adult granulosa cell tumors and 28 Sertoli-Leydig cell tumors. Extent of expression was scored based on the percentage of positive cells: 0, 5% or less; 1+, 6% to 25%; 2+, 26% to 50%; 3+, 51% to 75%; and 4+, 76% to 100%. Estrogen receptor alpha and PR were frequently expressed in adult granulosa cell tumors (66% and 98%, respectively) and Sertoli-Leydig cell tumors (79% and 86%, respectively). Diffuse (3+ or 4+) expression of PR was more common in adult granulosa cell tumors (68% vs. 36%; P = 0.013), whereas diffuse (3+ or 4+) expression of ERalpha was more frequent in Sertoli-Leydig cell tumors (50% vs. 20%; P = 0.010). In cases positive for both markers, adult granulosa cell tumors exhibited a focal (1+ or 2+) ERalpha/diffuse (3+ or 4+) PR coordinate profile more commonly than Sertoli-Leydig cell tumors (52% vs. 18%; P = 0.02), whereas Sertoli-Leydig cell tumors displayed a diffuse (3+ or 4+) ERalpha/focal (1+ or 2+) PR profile more frequently than adult granulosa cell tumors (36% vs. 0%; P = 0.0007). We conclude that expression of hormone receptors (based only on frequency of immunostaining) does not allow for distinction from other tumors in the differential diagnosis that are known to be frequently positive for ERalpha and PR such as endometrioid neoplasms. Most adult granulosa cell tumors and Sertoli-Leydig cell tumors share overlapping patterns of expression of ERalpha and PR with each other, but a subset of cases in each tumor category exhibits unique ERalpha/PR immunoprofiles (eg, focal ERalpha/diffuse PR in adult granulosa cell tumors and diffuse ERalpha/focal PR in Sertoli-Leydig cell tumors). These patterns of expression of ERalpha and PR may aid our understanding of the biologic differences between granulosa and Sertoli cell tumors.

Cutaneous Bipolaris infection in a neutropenic patient with acute lymphoblastic leukemia.

Bipolaris spicifera is a darkly pigmented (dematiaceous) fungus that uncommonly causes infections in humans. There are few cases of reported cutaneous Bipolaris infection in the literature. We report a case of a five-year-old boy with B-precursor-cell acute lymphoblastic leukemia who developed a cutaneous fungal infection on his left cheek Histopathological and microbiological findings identified the fungus as Bipolaris spicifera. Surgical excision and systemic antifungal therapy are the mainstay of treatment.