Hypertrophic osteoarthropathy (HOA) is a disabling condition that may occur secondarily to primary lung cancer. It is characterized by digital clubbing, arthralgia/arthritis, and periostosis of the tubular bones. The pain associated with HOA can be disabling and often refractory to conventional analgesics. We performed a comprehensive review of the literature using the PubMed database on treatment modalities available for HOA. We found 52 relevant articles-40 case reports, six case series, two review papers, and four combined case series and review papers. There were no randomized controlled trials reported. We then classified treatments used for HOA into two categories: (1) treatment of primary cause (i.e., resection of tumor, chemotherapy, radiotherapy, treatment of infection, etc.) and (2) symptomatic treatments (i.e., bisphosphonates, octreotide, NSAIDs, vagotomy, etc.). Subsequently, we summarized the main findings for each treatment. Although the clinical diagnosis of HOA has existed for over 100 years, the pathogenesis mechanism has not yet been elucidated, and treatment options for this condition remain experimental. Primary treatment is the most widely reported modality to be efficacious. In cases which primary therapy is not possible, several symptomatic treatment modalities are suggested, with various degree of success. Further research is needed to clarify the pathophysiological mechanism of HOA as to appropriately direct therapy.

Fanconi anemia (FA) is characterized by pancytopenia, congenital malformations, and susceptibility to malignancies. We describe a 31-year-old man with FA, who had undergone bone marrow transplantation and whole body irradiation at the age 17 years for FA. Fourteen years later, he presented with squamous cell carcinoma of the bronchus intermedius in the right lung. The tumor was located next to the main pulmonary artery and between the superior and inferior pulmonary veins. Two cycles of neoadjuvant therapy were given in an attempt to decrease tumor size and avoid a potential right pneumonectomy. Treatment consisted of a 21-day cycle with carboplatin (area under the curve 3) given on day 1 and gemcitabine (1250 mg/m) on day 1 and 8. Because FA cells are hypersensitive to DNA crosslinking agents, we reduced the carboplatin dose to minimize treatment-related toxicity. The tumor regressed sufficiently to permit performance of a right middle and lower lobectomy. In our case, neoadjuvant therapy with gemcitabine and low-dose carboplatin exhibited antitumor activity with manageable side-effects, suggesting that this chemotherapy regimen can be safely and effectively used in the treatment of NSCLC in FA patients who have achieved hematopoietic reconstitution after bone marrow transplantation.

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Hypertrophic pulmonary osteoarthropathy (HOA) is a disabling condition that may occur secondarily to primary lung carcinoma. Management of joint pain in patients with HOA is challenging, and treatment options are experimental. Here we report an unusual case of HOA in a 54 year-old man who presented with fever, rash and arthralgia as initial symptoms of an underlying non-small cell lung cancer. He did not respond to various treatment modalities including non-steroidal anti-inflammatory drugs (NSAIDs), pamidronate, and octreotide. His pain symptoms only improved once chemotherapy was administered. This case exemplifies the diagnostic and therapeutic challenge in patients with HOA, and underlines the need for further research to better define this disease and appropriately direct therapy.