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J Thorac Cardiovasc Surg 2007;134:541-542
© 2007 The American Association for Thoracic Surgery

Brief Communication

Unusual survival of an adult with pleuropulmonary blastoma and neurofibromatosis

Noah B. Zuker, MDa, Charles A. Dietl, MDb,*, Sherry Kenna, RNb, Edgar G. Fischer, MD, PhDc, David Gardner, MDc

a Department of Surgery, Division of Thoracic and Cardiovascular Surgery, University of New Mexico Health Sciences Center, Albuquerque, New Mexico
b Department of Surgery, University of New Mexico Health Sciences Center, Albuquerque, New Mexico
c Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico.

Received for publication April 14, 2007; accepted for publication April 26, 2007.

* Reprint requests: Charles A. Dietl, MD, Thoracic and Cardiovascular Surgery, Department of Surgery (ACC-2), University of New Mexico Health Sciences Center MSC 10 5610, Albuquerque, NM 87131-0001. (Email: CDietl@salud.unm.edu).

The first 20% of the full text of this article appears below.

A 19-year-old white man, with neurofibromatosis type I, optic glioma, and history of blebectomy for spontaneous left pneumothorax at age 17 years, presented to the University of New Mexico Health Sciences Center with chronic pain in the left side of his chest.

Clinical Summary

Chest radiography showed a solid mass encased within a large cystic structure, suspicious for aspergilloma of the left lung. A chest computed tomographic scan revealed a huge multiseptated cystic structure involving the entire major fissure of the left lung, containing a multilobulated soft tissue mass (Figure 1), and measuring approximately 10 x 20 cm. The mass and cysts were completely excised through a left thoracotomy. The specimen weighed 85 g in aggregate and consisted of thin-walled cysts, imparting a botryoid (ie, grape-like) appearance, with minor solid components (Figure 2, A). Microscopically, small immature tumor cells with ovoid to spindle-shaped nuclei were set in . . . [Full Text of this Article]






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