Pleomorphic hyalinizing angiectatic tumor of renal hilum

Idrees, Muhammad T.; Kieffer, Theodore; Badve, Sunil V.

doi:10.1016/j.anndiagpath.2011.03.010

Cited by 14 publications

(13 citation statements)

References 15 publications

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“…Plain CT and MRI scan showed it was a well-defined mass with inhomogenous dense or signal. Previously reported cases occurred in the neck or renal hilum also had clear boundary [ 7 , 9 ]. However, some cases arising in extremities were ill-defined [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…Positive findings of CD34 and vimentin only indicate a mesenchymal tumor. The negative S-100 protein excludes a schwannoma from the differential diagnosis, which is the most common lesion that can be confused with this entity [ 9 ]. SFT shows a similar immunoprofile to PHAT, both express vimentin, CD34, and CD99 [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, the histopathological findings of PHAT are obviously different from those of SFT [ 21 ]. Malignant fibrous histiocytomas can occasionally be CD34 positive but are negative for CD99 [ 9 ]. In addition, the negative CD56, SMA, Desmin, DOG-1, ALK-1 and actin in PHAT is distinguishable from neurogenic or myogenic tumors and GIST on pathology.…”

Section: Discussionmentioning

confidence: 99%

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Pelvic retroperitoneal pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissue: a case report

et al. 2016

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BackgroundPleomorphic hyalinizing angiectatic tumor (PHAT) is a rare soft tissue tumor of low malignant potential, which most often arises in the lower extremities. Lesions occurred in other anatomic locations have been rarely reported. Moreover, their imaging features have not been well discussed. Here we report a case of PHAT arising primarily in the pelvic retroperitoneum. To our knowledge, this is the first radiological description for retroperitoneum PHAT.Case presentationA 26-year-old female was referred to our hospital for evaluation of a pelvic mass incidentally noted in routine pre-pregnancy ultrasonography examination. Magnetic resonance imaging (MRI) and computed tomography (CT) scan revealed an irregular mass with clear boundary in the pelvic retroperitoneum. Its signal intensity or density was inhomogeneous. On MRI images, it mainly showed isointense and slight hypointense on T1 weighted image and isointense and hyperintense on T2 weighted image. On contrast-enhanced images, it showed marked but heterogenous enhancement. With the delay time increasing, the enhanced area in the lesion increased but the CT value decreased. Dilated vessels and hemorrhage were detected in the tumor. With patience and careful separation, it was completely excised with great amount of bleeding during operation. Pathological and immunohistochemistry analysis confirmed the diagnosis of PHAT of the soft parts. We found no evidence of recurrence 18 months after operation.ConclusionWe present an extremely rare case of PHAT arising primarily in the pelvic retroperitoneum. To our knowledge, this is the first radiological description for retroperitoneum PHAT. The provided information is useful for summarizing the characteristics of this kind of tumor. It should be included in the differential diagnosis of a well-defined, inhomogenously enhanced hypervascular soft-tissue mass in pelvic cavity.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pelvic retroperitoneal pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissue: a case report

et al. 2016

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“…1 Fewer than 100 cases have been reported to date; the largest series included 41 cases. 1,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] PHATs mainly occur in adults of both sexes during their fifth or sixth decade of life (range, 10-86 years). Tumor sizes range from 0.3 to 19.7 cm.…”

Section: Pleomorphic Hyalinizing Angiectatic Tumors Of Soft Partsmentioning

confidence: 99%

“…Both spindle and pleomorphic tumor cells are consistently positive for vimentin, with varied expression of CD34, factor XIIIa, VEGF, and CD99, but are negative for S100 protein, cytokeratin, desmin, epithelial membrane antigen, smooth muscle actin, HMB-45, MyoD1, p63, and CD31 (determined by immunohistochemistry). 11,12,14,15 Tu-mor cells have low proliferative indices, which are determined by either Ki-67 positivity (1%-3%) or percentage of S-phase cells, measured via immunohistochemistry or flow cytometry, respectively. Most Ki-67 þ cells are large and pleomorphic.…”

Section: Pleomorphic Hyalinizing Angiectatic Tumors Of Soft Partsmentioning

confidence: 99%

The t(1;10)(p22;q24) TGFBR3/MGEA5 Translocation in Pleomorphic Hyalinizing Angiectatic Tumor, Myxoinflammatory Fibroblastic Sarcoma, and Hemosiderotic Fibrolipomatous Tumor

Liu

Sukov

2018

Archives of Pathology &Amp; Laboratory Medicine

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- Diagnosis of HFLT, PHAT, and MIFS is challenging because of a lack of unique morphologic, immunophenotypic, molecular, and cytogenetic markers. The recurrent t(1;10)(p22;q24) translocation and/or TGFBR3/MGEA5 rearrangement was reported in 55 patients, with a relatively even distribution among HFLT, PHAT, and MIFS (17 HFLT, 15 MIFS, 13 MIFS/HFLT, and 10 PHAT). This indicates that current morphology-based diagnostic criteria do not identify reliably the subset of soft tissue tumor with t(1;10) translocation. Genetic heterogeneity of these tumors is supported by the recent detection of a mutually exclusive, second recurrent genetic change, t(7;17) TOM1L2-BRAF translocation or BRAF amplification, in a subset of MIFS.

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