Context.—

The great majority of primary pulmonary neoplasms are represented by non-small cell carcinomas—adenocarcinoma and squamous cell carcinoma. In addition, there is another group of neoplasms such as those of neuroendocrine origin that also represent a meaningful subset of primary lung neoplasms. Basically, any other tumor that is not in these groups of tumors may represent an unusual lung neoplasm.

Objective.—

To highlight more recently described unusual tumoral entities that may represent a challenge in diagnosis and that require awareness of their existence.

Data Sources.—

This is a review of 3 different entities: bronchiolar adenoma, adenofibroma, and hemangioblastoma-like clear cell stromal tumor. These tumoral conditions are rare, and a review of the literature is presented. The most relevant morphologic, immunohistochemical, and molecular aspects of bronchiolar adenoma, adenofibroma, and hemangioblastoma-like clear cell stromal tumor are presented. The difficulty of arriving at an unequivocal diagnosis in small biopsies is highlighted.

Conclusions.—

The 3 entities represent uncommon tumors occurring primarily in the lung and a diagnostic challenge not only in biopsy specimens but also often in surgically resected specimens. The use of immunohistochemical stains and in some cases of molecular diagnostics is of aid in arriving at final interpretation.

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Competing Interests

The author has no relevant financial interest in the products or companies described in this article.

Author notes

Presented in part at the Tenth Princeton Integrated Pathology Symposium; April 1, 2023; virtual.