PRIMARY PULMONARY ADENOID CYSTIC CARCINOMA: AN ELUSIVE DIAGNOSIS WITH CRITICAL IMPLICATIONS drruchis@aiimspatna.org

PE004

PRIMARY PULMONARY ADENOID CYSTIC CARCINOMA: AN ELUSIVE DIAGNOSIS WITH CRITICAL IMPLICATIONS

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India

Oral or Poster

Malignant salivary gland-type neoplasms are uncommon outside the head and neck region. Primary pulmonary adenoid cystic carcinoma (ACC) an extremely rare malignancy of peribronchial glands, accounts for 0.04–0.2% of all primary lung tumors. Unlike other bronchopulmonary malignancies, ACC is not associated with smoking and is classified as a low-grade tumor with an indolent but locally aggressive course. Due to its rarity, optimal management strategies remain poorly defined.

A non-smoker 40-year woman presented with a six-month history of voice change. Imaging revealed a 12 x 9.4 cm soft-tissue mass in right upper lobe of lung, encasing right pulmonary artery and bronchus. The patient underwent right pneumonectomy. Microscopy confirmed pulmonary ACC with a cribriform growth pattern, perineural invasion, and lymphovascular involvement. Postoperatively, she developed severe complications, leading to her demise on postoperative day 7.

Primary ACC of the lung is a rare malignant tumor of salivary gland origin, characterized by slow progression and late metastasis. They occur in younger individuals, with slight female predominance. It typically presents with non-specific symptoms, leading to diagnostic delays. Histologically, it exhibits cribriform, tubular, and solid patterns, with perineural invasion being a key feature. Immunohistochemical markers P63, smooth muscle actin (SMA), c-KIT, and S-100 aid in diagnosis. Surgery remains the mainstay of treatment, with radiotherapy recommended for incomplete resection. Targeted therapies have shown potential efficacy in c-KIT-positive cases.

Primary pulmonary ACC is a rare and challenging malignancy with an indolent yet locally aggressive course. Early diagnosis and complete surgical resection are crucial for optimal outcomes.