"A Rare Case of Primary Sclerosing Pneumocytoma in a Healthy Female" by Nitya Batra, Atulya Aman Khosla et al.
 

Document Type

Conference Proceeding

Publication Date

5-3-2024

Abstract

Introduction: Pulmonary sclerosing pneumocytoma is a rare group of benign pulmonary neoplasms that usually affects older adults over 50 years of age . This neoplasm is more commonly seen in women, with female to male ratio of 5:1 and is predominantly seen in Asian ethnicities. These tumors originate from type II alveolar pneumocytes and are hence classified as adenoma under the 2021 World Health Organization (WHO) classification of tumors . They account for 3-5 percent of total benign lung tumors. One of the striking aspects of PSP is its clinical presentation, or rather the lack thereof in many cases. It is often discovered incidentally during routine chest imaging, such as chest X-rays or CT scans, due to its asymptomatic nature. Moreover, it is essential to consider the potential for malignant transformation, as a subset of PSP cases have exhibited aggressive behavior Case Description: A 73-year-old female was seen in her cardiologist’s office for a routine visit. The patient had a past medical history significant for hypertension, hyperlipidemia, and coronary artery disease diagnosed on CT imaging. The patient was incidentally also found to have a lung nodule in her right middle lobe. In the office, the patient was completely asymptomatic without any chest pain, cough, shortness of breath, recent weight loss, loss of appetite, fevers, or night sweats. The physical exam was within normal limits. The patient had no history of smoking nicotine or any other drugs, worked as a chef in the food service industry, and denies any occupational metal or fume exposures. CT showed marginated, noncalcified pulmonary nodule in the right middle lobe measuring 2.6* 2.4 cm. There was no evidence of hilar/mediastinal lymphadenopathy, other nodules, septal thickening, or interstitial lung disease. The patient had a chest CT 4 years prior, on which this nodule measured 1.6*1.6 cm. The patient was referred to pulmonologist and was recommended for a PET scan, which revealed a 2.8* 2.9 cm well-circumscribed mildly hypermetabolic lesion in the with SUV of 2.68. CT-guided biopsy revealed pulmonary sclerosing pneumocytoma with hematoxylin and eosin staining showing classic cuboidal surface cells and round stromal cells. Immunohistochemistry was positive for AE 13, TTF-1 and EMA; the tumor was negative for Napsin and ERG. The patient was referred to see oncology and thoracic surgery. Her case was discussed at thoracic tumor board and was recommended video assisted thoracic surgery guided right middle lobectomy. The patient opted for active surveillance instead, with repeat imaging in 4 months and regular flow up with oncology, pulmonology, and surgery. Discussion: Although benign, these tumors have high potential for proliferation, local recurrence and metastasis to lymph nodes, pleura, and bones . Pathologically four different patterns have been described, namely papillary, solid, hemorrhagic, and sclerotic. Molecular alterations that have been most commonly described include mutations in the AKT-1, beta-catenin, BRAF genes. The prognosis of PSP is excellent due to its benign nature. First-line treatment modality is usually surgical resection. Interestingly, in a retrospective in South Korea, no difference was observed in all-cause mortality between surgical management and active surveillance.

Comments

American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day, May 3, 2024, Troy, MI

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