ABSTRACT
OBJECTIVE: To report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies. METHODS: We present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature. RESULTS: A 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal, <100) and a 24-hour urine epinephrine excretion of 55 microg (normal, <20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an alpha-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen's disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors. CONCLUSION: The current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies.
Subject(s)
Adrenal Gland Neoplasms/diagnosis , Ganglioneuroma/diagnosis , Neurofibromatosis 1/complications , Pheochromocytoma/diagnosis , Adrenal Gland Neoplasms/blood , Adrenal Gland Neoplasms/complications , Adrenal Glands/diagnostic imaging , Adult , Epinephrine/blood , Female , Ganglioneuroma/blood , Ganglioneuroma/chemically induced , Humans , Pheochromocytoma/blood , Pheochromocytoma/complications , Radiography , UltrasonographyABSTRACT
Lymphovascular invasion (LVI) by tumor cells is histologically evident in approximately 15% of invasive mammary duct carcinomas and is present in approximately 10% of cases with pathologically negative lymph nodes. LVI is indicative of unfavorable prognosis in the breast cancer-as manifested by increased local failure and reduced overall survival. It is for this reason that LVI is routinely included in the evaluation and reporting of all breast cancers. There are a variety of interpretative difficulties in the histopathologic assessment of LVI, and the clinical implications of any misinterpretation can be profound. This brief review seeks to highlight the difficulties in the evaluation of LVI in breast cancer.
Subject(s)
Adenocarcinoma/pathology , Breast Neoplasms/pathology , Female , Humans , Lymphatic Metastasis/pathology , Lymphatic System/pathology , Neoplasm Invasiveness/pathologyABSTRACT
Sentinel lymph node biopsy has attained "standard of care'' status in the management of breast carcinoma. However, the pathological interpretation and clinical consequences of "minimally involved'' sentinel lymph nodes remain controversial. Herein, we present some of the complex and challenging pathological problems inherent in this evolving setting. Clearly, at least some of our current concepts regarding "minimally involved'' sentinel lymph nodes need reappraisal.
