Treatment modalities and outcomes of pleomorphic hyalinizing angiectatic tumor: a systematic review of the literature

• Published in: Musculoskeletal surgery 2018-12, Vol.102 (3), p.213-221
• Main Authors: Rush, A. J., Patel, P. D., Shemesh, S., Subhawong, T. K., Pretell-Mazzini, J.
• Format: Article
• Language: English
• Subjects: Age Distribution; Combined Modality Therapy; Disease Management; Disease-Free Survival; Follow-Up Studies; Histology; Humans; Medicine; Medicine & Public Health; Neoplasm Recurrence, Local; Orthopedics; Radiotherapy; Review; Sex Distribution; Soft Tissue Neoplasms - epidemiology; Soft Tissue Neoplasms - radiotherapy; Soft Tissue Neoplasms - surgery; Soft Tissue Neoplasms - therapy; Surgery; Surgical Orthopedics; Systematic review; Treatment Outcome; Tumors
• ISSN: 2035-5106; 2035-5114
• DOI: 10.1007/s12306-018-0532-1

A systematic review of the cases documented in the literature regarding Pleomorphic hyalinizing angiectatic tumor of soft parts (PHAT) was performed in order to identify (1) location on presentation (2) surgical treatment modality (3) recurrence rate (4) any associations between location, age, histology, surgery type on recurrence. A systematic review of medical literature listed on PubMed was conducted identifying any prior case report and/or case series of diagnosed PHAT, with no exclusion based on language or time. Twenty-nine articles were identified removing any articles with duplicated cases yielding a total of 93 cases. Cases were broken down by gender, presenting location (UE/LE/axial), surgery type [wide local resection, non-wide local, wide local with radiation therapy (RT), non-wide local with RT], recurrence, and time to recurrence. The mean age at presentation was 54.5 ± 17.1 (range 10–89) with the 76% of cases appearing in the lower extremity (15% UE, 9% Axial). Of the 93 patients, 74 had a known surgical procedure, 31% WL, 40% NWL, 8% WL + RT, 1% NWL + RT. Of those treated surgically, 63 pts had documented follow-up and 18 (29%) had recurrence. A strong association was observed between surgery type and recurrence. Local recurrence was more common within the group undergoing NWLE in 52% (16/41) of cases ( p  = 0.002). Kaplan–Meier analysis showed an estimate mean time for recurrence of 43.87 months [95% confidence interval (CI) 24.52–63.22; and standard error (SE) 7.59] for the entire population. A trend was also seen toward males having a shorter disease-free survival than females (29.4 mos. vs. 69.5 mos.). No significant association seen between size, location, histology type and recurrence. PHAT has a characteristic presentation in the LE with a relatively high rate of local recurrence and slow-growing potential. Wide local excision appears to be superior in decreasing recurrence rates and a long-term follow-up period is needed.