Atypical Carcinoid of the Lung with Brown Tumor of the Femur – A Case Report of a Rare Association

Dina Zenezan, Abhimanyu Tushir,Jiejing Yin,Aileen Grace Arriola, Daniela M. Proca

Research Square (Research Square)(2023)

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摘要
Abstract Background: A diagnosis of neuroendocrine tumor (NET) is challenging, but critical, bearing serious implications concerning treatment and prognosis. Case Report: We describe a 39-year-old female presented with 2 weeks of increasing right leg pain preventing her from ambulating. She had a 6 month-long history of nausea, vomiting, and weight loss. Lab investigations showed hypercalcemia (13.1 mg/dl) and extremely elevated PTH (>2000 pg/ml). Differential diagnosis included primary hyperparathyroidism, as well as secondary hyperparathyroidism due to ectopic PTH production. Additional Imaging studies revealed a large lytic lesion in the femoral metaphysis and a well-circumscribed homogenous nodule in the right lower lobe. A lung biopsy showed a well-differentiated neuroendocrine neoplasm. Subsequently, right lower lobectomy was performed. The histologic and immuohistochemical features were consistent with a well differentiated neuroendocrine tumor, grade 2/3 (4.0 cm greatest dimension) with focal necrosis, and focally positive with Chromogranin, Synaptophysin and CD56, diffusely and strongly positive with AE1/AE3 and PTH, and negative with P-40, CDX2 and TTF1. A ki-67 showed approximately 15% nuclear positivity. Presence of necrosis, CDX2 negativity, and no involvement of other organs confirmed it as a primary lung neuroendocrine tumor with ectopic PTH secretion and secondary hyperparathyroidism. Conclusion: This a very rare occurrence that raises awareness to the possible different etiologies of secondary hyperparathyroidism. Well-differentiated NETs are rare overall, while the hormone secreting NETs are very unusual.
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brown tumor,atypical carcinoid,femur,lung
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