Pleomorphic Hyalinizing Angietactic Tumors: Recognizing A Novel Source Of Cancer In The Upper Extremity
Johnny Ionut Efanov, MD; Katherine Larose, MD; Michèle Tardif, MD
University of Montreal's Hospital Centre (CHUM), Montreal, QC, Canada
Although most soft tissue masses of the upper extremity are benign pathological entities, astute hand surgeons should not neglect the possibility of malignancy. An increasingly common tumor to include in a differential diagnosis is the pleomorphic hyalinizing angiectactic tumors (PHAT). There have been several cases reported in the literature, but the prevalence in upper extremities remains unknown. The aim of this study is to report our experience with PHAT in the hand and to perform a systematic review of the literature describing diagnostic findings and management.
Materials & Methods
Patients charts were reviewed and a systematic review of the literature was performed for all publications reporting a case of pleomorphic hyalinizing angiectactic tumor in the upper extremity. Publications from 1965 to 2016 were selected from databases such as PubMed/Medline, Cochrane Review and Google Scholar. Data collection included patient characteristics, anatomical sites of predisposition, tumor sizes, clinical signs, imaging findings, treatment options and recurrence rates. Results are reported as means and interquartile ranges.
A total of 48 publications have been retrieved with the systematic review, but only 8 articles reported outcomes in the upper extremity. From these publications, there were 11 patients diagnosed with PHAT. Tumor size ranged from 4 to 26 cm in largest diameter, with clinical findings significant for pain on direct palpation in 91% of cases. Treatment strategies included surgical excision with wide margins (91%) and radiotherapy (9%), without any chemotherapy. No local recurrences have been reported at 10 years of follow-up.
Pleomorphic hyalinizing angiectactic tumors are rare soft tissue masses that can arise in the upper extremity. Early recognition of clinical signs and surgical resection with wide margins have not resulted in recurrence so far.
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