Gómez-Gómez E, Garduño-López AL, Mondragón-Sánchez R, Meneses-García A

Language: Spanish
References: 12
Page: 221-224
PDF size: 161.33 Kb.

ABSTRACT

Objective: To describe the case of a patient with type I neurofibromatosis that developed a biliary cystadenocarcinoma.
Setting: Third level health care hospital.
Design: Case report.
Description of the case: A woman, 59 years old, who presented abdominal pain in the epigastrium and right hypochondrium, and was diagnosed with a cystic mass in the left hepatic lobe. The biopsy, plus clinical data such as central and basal Lisch nodules, multiple, hyperpigmented, skin lesions, headache, vertigo, and neuralgia of the trigeminus led to the diagnosis of type I neurofibromatosis. Imaging studies (CAT) revealed a hypodense mass with semi-liquid features, of irregular borders, without metastases to intra-abdominal masses nor ganglionary growths. A laparotomy was performed, revealing two nodular cystic lesions of 3.9 x 3.7 and 3.5 x 2.7 cm, soft, with hemorrhagic zones and abundant gelatinous-aspect material. A left hepatectomy was performed (segments II, III, and IV) and the histopathologic study revealed a biliary cystadenoma. She received no adjuvant therapy, and 18 months later another CAT study revealed local tumor recurrence and pulmonary metastases.
Conclusion: Type I neurofibromatosis is an inherited disease, autosomically dominant, associated to malignant neoplasias such as biliary cystadenocarcinoma.

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