Prophylactic Risk-reducing Hysterectomies and Bilateral Salpingo-oophorectomies in Patients With Lynch Syndrome: A Clinicopathologic Study of 29 Cases and Review of the Literature.

Lynch syndrome (LS) is associated with an increased risk for colorectal, endometrial, and ovarian carcinomas in women. Risk-reducing hysterectomy and bilateral salpingo-oophorectomy (RRHSO) has been shown to be a cost-effective form of management and prevention of gynecological malignancies in patients with LS. Studies of incidental gynecologic malignancies identified in RRHSO are limited. In addition, recommendations on optimal handling of this type of specimen have ranged from submitting for microscopic examination the entire endometrium, fallopian tubes and ovaries to submitting only routine representative sections of these organs. In this study, we present the clinicopathologic findings of 29 cases of LS patients that underwent risk-reducing gynecologic surgery at our institution over a period of 13 yr. Clinical-pathologic information was obtained from the patients' charts and pathology reports. Significant pathologic abnormalities were identified in 17% (5/29) of cases, all showing endometrial hyperplasia. Four of them with atypical and 1 without atypical. All of our cases with endometrial pathology had significant findings on preoperative endometrial sampling. To further study the recommendation of in toto submission of the endometrium, ovaries and fallopian tubes and the utility of preoperative endometrial sampling, we undertook a literature review of all the reported cases of incidental pathologic findings identified in RRHSO. The findings of our cohort and the literature reviewed support in toto submission of endometrium, and adnexal structures in the absence of gross lesions. In addition, our findings show a definite benefit for preoperative endometrial sampling as part of the workup for LS patients undergoing RRHSO.

Cholangiocarcinoma Presenting as Linitis Plastica with Unusual Metastases to the Psoas Muscle and Urinary Bladder.

Cholangiocarcinoma offers poor prognosis. Infrequent sites of metastasis are poorly described and often diagnostically delayed or missed. Bile duct brush cytologies provide poor diagnostic sensitivity/specificity. We present an unusual case of cholangiocarcinoma in a 34-year-old woman with rare distant metastasis to the psoas muscle and urinary bladder. It is the first case of metastatic cholangiocarcinoma presenting as linitis plastica, and our patient is the youngest to be described with metastatic cholangiocarcinoma to the psoas muscle leading to diagnosis. We conclude that seemingly idiopathic biliary strictures that fail to respond to testing should prompt alarm and referral for cholangioscopy, where available.

Follicular dendritic cell sarcoma of the head and neck expressing thyroid transcription factor-1: a case report with clinicopathologic and immunohistochemical literature review.

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm of follicular dendritic cells, most commonly affecting the lymph nodes and extranodal soft tissues of the head and neck, but also potentially arising in any visceral organ. FDCS with its diverse morphologies raises an occasionally challenging differential of primary and metastatic tumors with overlapping histologic and immunohistochemical features. When involving the head and neck, FDCS may be confused with squamous cell carcinoma, undifferentiated carcinoma, extracranial meningioma, and variants of papillary thyroid carcinoma. We describe here a case of FDCS showing nuclear grooves, intranuclear pseudoinclusions, diffuse epithelial membrane antigen and focal cytokeratin staining, and the first documented report of positivity for thyroid transcription factor-1. A discussion of the differential diagnosis and potential diagnostic pitfalls in FDCS brought forth by thyroid transcription factor-1 immunoreactivity and a full review of clinicopathologic and immunohistochemical features of head and neck FDCS are presented.

Nerve sheath tumor with degenerative atypia and multivacuolated lipoblasts.

Lipoblastic nerve sheath tumor is a recently described benign soft tissue tumor consisting of lipoblasts in a neural/schwannian background. The 6 reported cases have exclusively comprised signet ring lipoblasts and showed no cellular atypia. The authors describe the first lipoblastic nerve sheath tumor to harbor multivacuolated lipoblasts and degenerative atypia, underscoring its important differential diagnosis with well-differentiated liposarcoma. The purpose of this report is to expand the morphologic spectrum of this unusual neoplasm, and reemphasize the potential of benign nonadipocytic tumors to harbor multivacuolated lipoblasts and mimic liposarcoma.

Clinicopathologic characteristics of carcinomas that develop after a biopsy containing columnar cell lesions: evidence against a precursor role.

BACKGROUND: Columnar cell lesions are frequently associated with atypical ductal hyperplasia, lobular neoplasia, and tubular carcinoma, and have been suggested as a precursor lesion for low-grade carcinomas. However, in long-term follow-up studies, columnar cell lesions are associated with only a slight increase in later breast cancer development. If columnar cell lesions are precursor lesions, one would expect subsequent cancers to develop at the same site as the biopsy and to be preferentially of low grade. The goal of this article is to review the clinical and pathologic features of carcinomas that develop after a diagnosis of columnar cell lesion to try to establish whether these lesions are precursors to low-grade invasive carcinoma. METHODS: The authors reviewed biopsies containing columnar cell lesions, using the criteria of Schnitt and Vincent-Salomon, from 77 women in the Nashville Breast Cohort who developed subsequent breast carcinoma. Clinicopathologic features including laterality, type, and grade of the subsequent cancer were recorded. RESULTS: Breast cancer developed a median of 11 years after initial biopsy. The median age at diagnosis was 60 years. The majority of invasive carcinomas were of no special type and of intermediate grade. Moreover, the carcinomas were as likely to occur in the contralateral breast as in the breast that was originally diagnosed with columnar cell lesion, regardless of columnar cell lesion subtype (P = .48). CONCLUSIONS: Carcinoma subsequent to columnar cell lesions may occur in either breast and tends to show a similar grade and type distribution as sporadic breast cancer. These findings argue against columnar cell lesions being a true precursor for low-grade invasive carcinoma.