High pathologic misdiagnosis of cervical adenocarcinoma in situ.

OBJECTIVE: The objectives of this research were to evaluate cases of adenocarcinoma in situ (AIS) and early invasive adenocarcinoma (AC) of the uterine cervix in order to: (1) calculate the pathologic discordance between initial biopsies and final surgical excision specimens and (2) describe the clinical and pathologic factors associated with discordance. MATERIALS AND METHODS: The University of California, Irvine and Long Beach Memorial tumor registries were used to identify 105 women with AIS and early AC treated between 1990 and 2008. The primary endpoint measured was change in diagnosis when comparing pathology from the initial biopsy to specimens from a large loop excision of the transformation zone (LLETZ), cold knife cone (CKC), or hysterectomy. The variables studied were: age, endocervical curettage (ECC), co-existing cervical intraepithelial neoplasia (CIN), race, and insurance type, as surrogates for socioeconomic status. RESULTS: Initial biopsies were diagnosed as AIS and AC in 44% and 56% of patients, respectively. Of the patients with a biopsy diagnosis ofAIS, 29% had a final diagnosis of AC after excisional procedure, and this discordance was not associated with any of the factors studied. CONCLUSIONS: A concerning high rate of discordance between colposcopic-guided punch biopsy and final pathology reinforces the current guidelines to always perform an excisional biopsy following diagnosis of AIS on punch biopsy.

Adenocarcinoma and dysplasia in an ileal neobladder after ileocystoplasty for interstitial cystitis.

We report a 54-year-old woman with an adenocarcinoma of an ileal neobladder arising upon a background of ileal mucosal dysplasia. We believe that no case study or report has previously documented neobladder ileal mucosal dysplasia adjacent to an ileal neobladder adenocarcinoma. This observation supports the current hypothesis that ileal neobladders are dynamic environments for potential malignancy, and moreover, suggests a sequence of morphologic and molecular derangements similar to that seen in colorectal carcinoma. Those patients status post ileal neobladder are at risk for glandular dysplasia and malignancy and should be followed closely.

Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA. A case report.

BACKGROUND: Multiple endocrine neoplasia syndrome type IIA (MEN IIA) has rarely been encountered in pregnancy. CASE: A 22-year-old, nulliparous woman developed bilateral pheochromocytomas during pregnancy. This finding aroused suspicion for MEN IIA, and close endocrinologic follow-up was arranged. Four years later, hyperparathyroidism developed, and the diagnosis was established. The patient underwent prophylactic total thyroidectomy with parathyroid exploration. CONCLUSION: This was the first case of MEN IIA in pregnancy in which the diagnosis was established prior to the development of medullary thyroid cancer, thereby allowing prophylactic thyroidectomy. The presence of bilateral neoplastic disease in young patients may be indicative of a hereditary predisposition to malignancy.

The evolution of Linitis plastica as a consequence of advanced gastric carcinoma in pregnancy.

A 40-year-old multigravid woman was examined on multiple occasions during pregnancy because of persistent gastrointestinal symptoms. A metastatic, unresectable gastric carcinoma that had evolved to linitis plastica was diagnosed at 26 weeks' gestation. The patient was delivered of a viable infant at 27 weeks' gestation, and she died of disease 1.5 months after the diagnosis was made.

Occipital encephalocele and MURCS association: case report and review of central nervous system anomalies in MURCS patients.

The combination of MURCS association (Müllerian duct and renal agenesis, upper limb and rib anomalies) and occipital encephalocele occurred in a stillborn girl of 41 weeks gestation. The malformations are compatible with a defect in the organization of the paraxial mesoderm that gives rise to occipital, cervical, and thoracic somites and adjoining intermediate mesoderm. These structures contribute to the occipital bone, cervical spine, upper limbs, and urogenital system. Brain imaging may be useful in assessing MURCS patients, if cranial malformations prove to be clinically important in these individuals.

Myometrial expression of mRNA encoding epidermal growth factor receptor (EGFR) throughout the menstrual cycle.

PROBLEM: To investigate the quantitative changes in expression of EGFR mRNA in the myometrium throughout the menstrual cycle. METHOD: Myometrium was collected at hysterectomy from 27 women with a history of regular cycles. Total RNA (20 micrograms) was isolated and analyzed by Northern blot using a human EGF-R specific 32P-labeled cDNA probe. The hybridization signals were quantified by densitometry, standardized, and reported in densitometry signal units (DSU). Endometrial specimens from the same uteri were simultaneously evaluated for histologic dating of menstrual cycle day. EGFR gene expression in endometrium was previously reported. Statistical significance of the differences in myometrial gene expression between menstrual phases was evaluated by student's t test. RESULTS: EGFR mRNA was expressed in all myometrial tissues tested. Levels were higher in the late proliferative phase than in all other phases (P < .05). Women over 40 years old had lower proliferative phase expression than younger women. CONCLUSION: This data suggests that myometrial EGFR mRNA expression varies in association with the histologic phases of the normal menstrual cycle, and may be affected by aging, even when cycles occur at regular intervals.

Endometrial expression of mRNA encoding epidermal growth factor receptor in normal and leiomyomatous uteri throughout the menstrual cycle.

PROBLEM: To investigate the relationship between the histologic phase and the quantitative expression of epidermal growth factor receptor (EGFR) mRNA in the endometrium of normal and leiomyomatous uteri. METHODS: To test this relationship, endometrium was collected at hysterectomy from 38 women with a history of regular cycles. Total RNA (20 micrograms) was isolated and analyzed by Northern blot using a human EGFR-specific 32P labeled cDNA probe. The hybridization signals were quantified by densitometry, standardized, and reported in densitometry signal units. Separate portions of the same endometrial specimens were simultaneously prepared for histologic evaluation. The differences between menstrual phases were evaluated by Student's t-test. RESULTS: EGFR mRNA was expressed in all endometrial tissues tested. Levels were higher in the midproliferative phase than in all other phases for both myomatous and nonmyomatous uteri (P < .05). Myomatous uteri showed lower expression than nonmyomatous uteri in the early and late proliferative phase (P < .05). Age did not appear to alter endometrial EGFR mRNA expression. CONCLUSION: This data suggest that endometrial EGFR mRNA expression varies in association with the histologic changes of the normal menstrual cycle, and may be associated with the presence of leiomyomata uteri.

Leydig cell neoplasia in a patient with Reifenstein syndrome.

We present the rare coincidence of a Leydig cell tumor in both testicles of a patient with partial androgen insensitivity syndrome (PAIS). The clinical picture with perineoscrotal hypospadia, micropenis, gynecomastia and delayed puberty and the serum hormone levels with elevated concentrations of testosterone, luteinising hormone (LH) and follicle-stimulating hormone were entirely consistent with PAIS. Ultimately, the diagnosis was confirmed by determination of genital skin fibroblast androgen receptor binding capacity for 5 beta-dihydrotestosterone, which demonstrated a qualitatively abnormal androgen receptor. At 44 years of age, a nodule in the left testis led to orchidectomy. At that time, the right testis was inconspicuous sonographically. But 3 years later the right testis developed nodules and was removed. Review of testicular histology revealed the presence of Leydig cell hyperplasia (LCH), multifocal nodular hyperplasia and Leydig cell neoplasia (LCN) in both testes. Many micronodules of Leydig cells in transition from hyperplasia to neoplasia were also identified. The simultaneous development of histologically identical nodes of LCN independently from each other and a different sites of both tests indicates the presence of a tumorigenic factor acting on the Leydig cells. Furthermore, the observation of multiple foci of cells in all stages of transition from hyperplasia to neoplasia demonstrates the persistent process of transformation. We speculate, that in this patient the grossly elevated LH levels present over 30 years have enhanced, if not provoked, the formation of LCN. In addition, the defective androgen receptor might have prevented suppressive effects of androgens on the Leydig cells.