AB 51. Rare case of granular cell tumor of the lung in a patient with multiple endocrine neoplasia syndrome and mass of the left lung

Background: Granular cell tumor is a soft tissue neoplasm that originates in the nervous system and arises at virtually any body site, but is mainly found in the skin, oral cavity or digestive tract. Most are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases. We describe a rare localization of the tumor to the lung in a patient with a known age, multiple endocrine neoplasia syndrome (MEN).

Patients and methods: Male, 50 years old, smoker of 30 p/y patient of MEN 1[hyperparathyroidism with parathyroid gland removal and substitution treatment from 24 years, adenoma of pituitary gland (prolactinoma)] revealed because of persistent cough, in chest CT scan. Findings of the exam is a mass in the apical segment of the left lower lobe with slightly enlarged ipsilateral hilar nodes.

Results: Bronchoscopy to which the patient underwent was not revealed direct or indirect elements of filtration in the described fault but the presence of neoplastic tissue with papillary configuration (cauliflower-like) in the middle of the inner wall of the left stem bronchi (histological and immunohistochemical confirmation of granular cell tumor). The patient underwent complete staging [abdominal CT scan revealed adrenal adenomas, head CT without findings, chest MRI with findings in proportion to PET-CT with 18-FDG which was followed : no increased metabolic activity by PET-CT , not indicative of malignancy of the lesion (SUVmax=3.6) of the left hilar nodes (SUVmax=2.8) and the lesion in the left subscapularis muscle (SUVmax=3.3) with relatively little cellular activity (immunohistochemistry Ki-67<2%)]. It was recommended removal of that lesion and thoracic assessment (consent of the patient is expected).

Conclusions: The granular cell tumors are usually asymptomatic. They appear as solitary or multiple nodules, up to 3cm in size, slight superiority to women and normal appearance in fourth to sixth decade of life. They are usually benign and only 2% malignant, so then are larger (up to 15 cm), creating distant metastases with poor prognosis. The Schwann cells are the substrate tissue. Rarely found intrabronchial, can coexist with lung cancer and until now, there is no reference in the medical literature of correlation or coexistence with the multiple neuroendocrine syndrome, in which usually exist other non-endocrine type lesions (lipoma, facial angiofibroma, kollagonoma or rarely pheochromocytoma or ependymoma).