Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review

Abstract

Solitary fibrous tumors (SFT) represent a unique subset of mostly benign heterogeneous tumors with mesenchymal cell origins. These tumors have been reported in the past as being mostly indolent, with a slowly evolving clinical course and low potential for malignancy. Although found systemically, the incidence of SFT arising intrathoracically, from the pleura of the lung, is relatively poorly documented in the medical literature. SFT is a rare phenomenon, but in even rarer circumstances, these tumors are associated with distinctive paraneoplastic syndromes, such as Pierre-Marie-Bamberger syndrome (PMBS) and Doege-Potter syndrome (DPS). PMBS presents as digital clubbing and hypertrophic pulmonary osteoarthropathy. DPS has been characterized as a non-islet cell tumor hypoglycemia due to the ectopic secretion of insulin-like growth factor 2 (IGF-2), a pattern seen in fewer than 5% of cases of SFT. Treatment is typically through surgical resection. In our research of the medical literature, we found only very few cases in which the association with SFT and both paraneoplastic syndromes were described. Here, we report an uncommon case of a 68-year-old male patient found to have an incidental right hemithoracic tumor with digital clubbing and intermittent severe episodes of fasting hypoglycemia after initially presenting with a syncopal episode.

Keywords: doege-potter syndrome; pierre-marie-bamberger syndrome; solitary fibrous tumor.

Figure 1. Chest X-ray showing right lower lung diffuse opacity/mass (arrow). The opacity displaces the heart posteriorly, extends inferiorly below the field of view. The opacity obscures the right heart border and right hemidiaphragm.

Figure 2. (A) Cross-sectional image of CT chest demonstrating 14 x 16 x 18 cm heterogenous right lung/thoracic mass (arrow) arising from right hilum/middle mediastinum or right hemidiaphragm. (B) Coronal CT scan showing right lung mass (arrow).

Figure 3. Transthoracic echocardiogram showing lung mass (white arrow) compressing the right atrial cavity (red arrow).

Figure 4. Histochemical analysis showed a spindle cell lesion composed of sheets of spindle cells with oval or elongated nuclei separated by collagen. Focal areas with mitotic figures up to 3 per 10 HPF were identified (arrow).

Figure 5. Immunohistochemistry showing positive stains for (A) Vimentin (B) Desmin (C) CD34 (D) BCL-2 (E) CD99.

Figure 6. STAT6 showed diffuse strong positive nuclear staining.