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Hypoglycemia and Finger Clubbing—Consider Doege-Potter

      To the Editor:
      Schattner et al
      • Schattner A.
      • Sokolovski N.
      • Altman E.
      • Adi M.
      Major crush: a solitary fibrous pleural tumor.
      describe a patient with increasing dyspnea in which the final diagnosis revealed a solitary fibrous tumor of the pleura. We read this report with great interest because we recently diagnosed and treated a comparable case in our institution.
      In solitary fibrous tumors of the pleura symptoms occur in up to 75%, mostly cough, dyspnea, and chest pain.
      • Abu Arab W.
      Solitary fibrous tumours of the pleura.
      Manifestations of hypoglycemia and finger clubbing, known as Doege-Potter syndrome, are exceedingly rare.
      • Kalebi A.Y.
      • Hale M.J.
      • Wong M.L.
      • Hoffman T.
      • Murray J.
      Surgically cured hypoglycaemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
      Hypoglycemia is induced by high-molecular-weight insulin-like growth factor type II and occurs in <5% of cases; finger clubbing occurs in up to 20%.
      • Cardillo G.
      • Lococo F.
      • Carleo F.
      • Martelli M.
      Solitary fibrous tumor of the pleura.
      In our case, a female patient presented with acute hypoglycemic shock, and history revealed weight loss, increasing dyspnea, episodes of dizziness, and loss of consciousness in the previous 5 months. Physical examination revealed decreased breath sounds on the right side of the chest and finger clubbing on both hands (Figure, A). Normal glucose levels were achieved only by continuous intravenous infusion of 10% glucose. Chest radiograph and computed tomography scan revealed a huge intrathoracic tumor, suggestive for a solitary fibrous tumor of the pleura (Figure, B). Together with the symptoms of hypoglycemia and finger-clubbing, the diagnosis was Doege-Potter syndrome and the tumor was excised. Treatment of choice is complete surgical excision of the tumor and remains the preferred treatment in the case of recurrence.
      • Robinson L.A.
      Solitary fibrous tumour of the pleura.
      Symptoms, in particular finger clubbing and hypoglycemia, resolve after complete excision of the tumor, but may recur with recurrence of the lesion.
      • Kalebi A.Y.
      • Hale M.J.
      • Wong M.L.
      • Hoffman T.
      • Murray J.
      Surgically cured hypoglycaemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
      Figure thumbnail gr1
      Figure(A) Finger clubbing. (B) Intrathoracic tumor.
      As stated by Schattner et al,
      • Schattner A.
      • Sokolovski N.
      • Altman E.
      • Adi M.
      Major crush: a solitary fibrous pleural tumor.
      most solitary fibrous tumors of the pleura show benign histological features. However, up to 20% of all cases are malignant.
      • Cardillo G.
      • Lococo F.
      • Carleo F.
      • Martelli M.
      Solitary fibrous tumor of the pleura.
      • Robinson L.A.
      Solitary fibrous tumour of the pleura.
      Criteria for malignancy are more than 4 mitotic figures in 10 high-power fields, hemorrhage, high cellularity, pleomorphism, or necrosis.
      • Robinson L.A.
      Solitary fibrous tumour of the pleura.
      In our case, histological examination revealed a malignant solitary fibrous tumor of the pleura, positive for CD34, with 2 mitotic figures in 10 high-power fields, widespread focal necroses, and elevated proliferative activity. Puncture of a pulmonary nodule 2 months after surgery revealed a metastasis of the excised solitary fibrous tumor of the pleura. The patient refused the proposed resection of the metastasis but agreed to undergo stereotactic radiotherapy. Radiotherapy and chemotherapy are of limited importance but may be beneficial in cases where resection is incomplete or impossible.
      • Kalebi A.Y.
      • Hale M.J.
      • Wong M.L.
      • Hoffman T.
      • Murray J.
      Surgically cured hypoglycaemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
      All cases of solitary fibrous tumor of the pleura should be followed up with periodic chest computed tomography scans in order to monitor for recurrence, especially in malignant cases.
      • Kalebi A.Y.
      • Hale M.J.
      • Wong M.L.
      • Hoffman T.
      • Murray J.
      Surgically cured hypoglycaemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
      The presence of hypoglycemia in nondiabetic patients should raise the suspicion of a rare cause like solitary fibrous tumor of the pleura.

      References

        • Schattner A.
        • Sokolovski N.
        • Altman E.
        • Adi M.
        Major crush: a solitary fibrous pleural tumor.
        Am J Med. 2013; 126: 685-686
        • Abu Arab W.
        Solitary fibrous tumours of the pleura.
        Eur J Cardiothorac Surg. 2012; 41: 587-597
        • Kalebi A.Y.
        • Hale M.J.
        • Wong M.L.
        • Hoffman T.
        • Murray J.
        Surgically cured hypoglycaemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
        J Cardiothorac Surg. 2009; 4: 45
        • Cardillo G.
        • Lococo F.
        • Carleo F.
        • Martelli M.
        Solitary fibrous tumor of the pleura.
        Curr Opin Pulm Med. 2012; 18: 339-346
        • Robinson L.A.
        Solitary fibrous tumour of the pleura.
        Cancer Control. 2006; 13: 264-269

      Linked Article

      • Major Crush: a Solitary Fibrous Pleural Tumor
        The American Journal of MedicineVol. 126Issue 8
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          A 78-year-old woman's diagnosis truly weighed heavily on her heart. The patient presented with a history of increasing dyspnea over the previous 2 months, and this was accompanied by a dry cough and occasional right scapular pain. She denied fever, sweats, or weight loss. Her medical history included diabetes and hypertension, but she had no prior hospital admissions.
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      • The Reply
        The American Journal of MedicineVol. 126Issue 12
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          We thank Fritzsche et al for their comments and report of an intriguing rare subset of a rare tumor; however, their final conclusion may not be quite accurate.
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