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Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.

Purpose: To describe a clinical case of an orbital paraganglioma that displayed regression after biopsy alone., Methods: Case report., Results: A 75-year-old female was examined for a right orbital tumor suspected to be metastatic breast carcinoma. An orbital biopsy was performed with significant hemorrhage encountered requiring extensive cautery. There was apparent clinical regression of the tumor with no signs of proptosis or eye movement restriction two years after this patient's biopsy. Histology was consistent with paraganglioma (glomus tumor)., Conclusion: Although we cannot rule out spontaneous regression of this unique tumor, we postulate that tissue necrosis caused by the use of cautery induced regression. Unless encapsulated and easily accessible, we suggest that the best management of this rare tumor is that of observation after being found negative for malignancy by biopsy given their propensity for slow progression and in rare cases, regression.

The usual complications after a definitive pull-through procedure for Hirschsprung's disease include stricture formation, enterocolitis, bowel obstruction, and, occasionally, wound infection. The authors report a case of mucinous adenocarcinoma arising 32 years later at a stricture site from a previous pull-through procedure., (Copyright 2001 by W.B. Saunders Company.)

Objectives: Histologic findings in biopsy specimens obtained from patients with atypical glandular cells of uncertain significance were studied to define the utility and limitations of this category., Study Design: Computerized records over a 3-year period were retrospectively analyzed. The most significant histologic diagnosis from all biopsy specimens submitted was compared with the subcategory of the first Papanicolaou smear obtained showing atypical glandular cells of uncertain significance., Results: Biopsy results were available for 531 of 1117 patients with atypical glandular cells of uncertain significance (48%). Biopsy-proved preinvasive (83%) or invasive (17%) lesions were present in 191 patients (36%). Eighty-nine percent of the preinvasive lesions were squamous, whereas 97% of the invasive lesions were glandular. Glandular lesions were more likely to be invasive, whereas squamous lesions were more likely to be preinvasive (p < 0.001). Twenty-eight patients had endometrial carcinoma, which represents 88% of all invasive carcinomas detected., Conclusions: Almost three fourths of patients with atypical glandular cells of uncertain significance and with lesions have squamous lesions, not glandular as suggested by the name of the category. Unlike patients with atypical squamous cells of uncertain significance, patients with atypical glandular cells of uncertain significance have a significant risk of malignant lesions, which are nearly all glandular and predominantly arise from the endometrium.

Objective: To establish the frequency of the atypical glandular cells of uncertain significance (AGCUS) category, and its subcategories, as defined by the Bethesda System (TBS)., Methods: Our computerized records of cervical/vaginal cytology specimens submitted from January 1, 1993, through December 31, 1995, were retrospectively reviewed for specimens diagnosed as AGCUS. When appropriate, our subcategory of "AGCUS favor premalignant/malignant lesion" was further qualified as "favor endocervical adenocarcinoma in situ" or "suspicious for endometrial carcinoma." The number of specimens and patients diagnosed for each subcategory were grouped by calendar year. Differences in frequency between time periods were tested for statistical significance using chi 2 analysis., Results: AGCUS was diagnosed in 1181 of 177,715 submitted specimens (0.66%). The frequency of subcategories was as follows: "favor reactive" (65%), "unable to further classify" (30%), "favor premalignant/malignant" (2.9%), "suspicious for endometrial carcinoma" (1.9%), and "favor endocervical adenocarcinoma in situ" (0.4%). From 1993 to 1995 there was an increase in the rate of diagnosis of AGCUS (0.55 to 0.73%; P < 0.001) and a decrease in the percentage of specimens with AGCUS subclassified as "favor premalignant/malignant" (6.2 to 0.5%; P < 0.001). Other subcategories showed no significant change in frequency over this time period. The rate of biopsy-proven preinvasive or invasive lesions in AGCUS patients also showed no significant change from year to year over this time period., Conclusion: The AGCUS diagnosis can be anticipated at a low but consistent rate from a cytology laboratory using TBS. Any comparison of laboratories should take into consideration the change in reporting frequencies that occurs as part of the "learning curve" following introduction of TBS reporting. Uniform diagnostic criteria and additional reports with large numbers of cytologic specimens will be needed to establish the expected frequency of AGCUS and its subcategories.

Squamous carcinoma primary in the endometrium has been rarely reported. Diagnosis of this condition requires adherence to strict histologic criteria. Most patients are postmenopausal at presentation, and long-term survival is poor. We present a patient with peritonitis and small bowel obstruction. Chemotherapy was given after disease recurrence and was ineffective. Receptor data and serum markers were obtained.

To examine the importance of immunocytochemically detectable occult axillary lymph node metastases in patients with lobular carcinoma of breast, tumor registry data from 54 cases indexed as lobular carcinoma during the period 1973-82 were reviewed. Recurrences and/or deaths due to cancer were essentially confined to the group of patients with a component of invasive lobular carcinoma (ILC), therefore this subset was selected for further study. Seven of 20 cases had lymph node metastases diagnosed histologically at the time of mastectomy. Follow-up of these patients showed four dead of disease (DOD) at one, three, three, and seven years; one alive with disease (AWD) at one year; and two with no evidence of disease (NED) at four and five years. Eleven of 20 were node negative. Follow-up of this group showed nine NED and two DOD at two and four years. Two of 20 had unknown node status. Formalin-fixed, paraffin embedded lymph node blocks were available in 12 of 20 cases with a component of ILC. Of these, 4/12 cases had histologically positive nodes while 8/12 were originally diagnosed as negative. A cytokeratin monoclonal antibody cocktail (MAK-6, CAM 5.2 and AE1/AE3) was applied to all 12 cases. Cytokeratin immunoreactivity (CK-IR) was found in all four cases that were histologically positive. Five of eight histologically negative nodes lacked CK-IR, however the other three cases showed CK-IR in micrometastases. Review of newly prepared hematoxylin-eosin sections from the paraffin blocks failed to demonstrate metastases.(ABSTRACT TRUNCATED AT 250 WORDS)

Necrotizing fasciitis is an often fatal, often initially unrecognized condition. Although it was first described over 60 years ago, occurrence in the vulva was only first recognized in 1972. The condition is most often associated with diabetes, prior injury, surgery, or irradiation. Aggressive surgical excision is required, early in the course of the disease, to salvage the patient. An association with vulvar carcinoma in a nondiabetic patient has not been previously reported. We report such a case, with a poor outcome, because surgical intervention was not possible until late in the course of the disease.