Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2021.353
Autopsy and Case Reports
Clinical Case Report and Review

Pleomorphic hyalinizing angiectatic tumor of the vulva: literature review based on a rare presentation

Eveline Cristina da Silva; Rodrigo Fonseca Abreu; Antônio Geraldo Nascimento; Louise De Brot Andrade

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Abstract

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissues is a rare, non-metastatic tumor of unknown etiology and uncertain behavior, which may recur locally. There are few reports on this condition, and due to the rarity of the disease, its lineage has not yet been fully elucidated. The present study aims to report the case of an unusual entity observed for the first time in vulval topography. A female patient, 83 years old, presented with a tumor in the vulvar region that had evolved for approximately 4 months. Magnetic resonance imaging showed an expansive perineal formation of 8.5 × 3.5 cm, and a hemivulvectomy with a flap rotation was performed. The review of the slides revealed a mesenchymal lesion without significant atypia, which was richly vascularized. In the areas of interest, the immunohistochemical (IHC) study demonstrated positivity for CD34, estrogen, and progesterone receptors; it was negative for the other tested markers. Morphological findings associated with the IHC staining panel supported the diagnosis of PHAT. The main morphological features of PHAT are clusters of ectatic vessels of different sizes that show deposits of subendothelial and intraluminal fibrin. Fusiform and pleomorphic cells randomly arranged in leaves or long fascicles intermingle these vessels. It is essential to recognize this entity and consider it among the differential diagnoses of a mesenchymal lesion, given the wide variety of entities that comprise this group of lesions.

Keywords

Soft Tissue Neoplasms, Vulvar Neoplasms, Sarcoma

References

1 Smith MEF, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumour of soft parts: a low-grade neoplasm resembling neurilemoma. Am J Surg Pathol. 1996;20(1):21-9. http://dx.doi.org/10.1097/00000478-199601000-00002. PMid:8540605.

2 The WHO Classification of Tumours Editorial Board. WHO classification of tumours soft tissue and bone tumours. 5th ed. Lyon: IARC Press; 2020.

3 Lindeberg M. Diagnostic pathology: soft tissue tumors. 2nd ed. Philadelphia: Elsevier; 2015.

4 Folpe AL, Weiss SW. Pleomorphic Hyalinizing Angiectatic Tumor Analysis of 41 Cases Supporting Evolution From a Distinctive Precursor Lesion. Am J Surg Pathol. 2004;28(11):1417-25. http://dx.doi.org/10.1097/01.pas.0000141406.02581.fb. PMid:15489645.

5 Cazzato G, Colagrande A, Cimmino A, et al. Pleomorphic Hyalinizing Angiectatic Tumor (PHAT): review of the literature with case presentation. Dermatopathology. 2021;8(2):97-102. http://dx.doi.org/10.3390/dermatopathology8020015. PMid:33916538.

6 Peng HC, Huang MT, Chen DJ, Leung TK, Chu JS. Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts. J Formos Med Assoc. 2010;109(8):616-20. http://dx.doi.org/10.1016/S0929-6646(10)60100-3. PMid:20708514.

7 Balasubiramaniyan V, Muduly D, Samal S, et al. Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature. Autops Case Rep. 2019;9(3):e2019097. http://dx.doi.org/10.4322/acr.2019.097. PMid:31440480.

8 Mohajeri A, Kindblom LG, Sumathi VP, et al. SNP array and FISH findings in two pleomorphic hyalinizing angiectatic tumors. Cancer Genet. 2012;205(12):673-6. http://dx.doi.org/10.1016/j.cancergen.2012.10.008. PMid:23177593.

9 Illueca C, Machado I, Cruz J, et al. Pleomorphic hyalinizing angiectatic tumor: a report of 3 new cases, 1 with sarcomatous myxofibrosarcoma component and another with unreported soft tissue palpebral location. Appl Immunohistochem Mol Morphol. 2012;20(1):96-101. http://dx.doi.org/10.1097/PAI.0b013e31821a24ab. PMid:22024990.

10 Subhawong TK, Subhawong AP, Montgomery EA, Fayad LM. Pleomorphic hyalinizing angiectatic tumor: imaging findings. Skeletal Radiol. 2012;41(12):1621-6. http://dx.doi.org/10.1007/s00256-012-1443-0. PMid:22660836.

11 Idrees MT, Kieffer T, Badve S. Pleomorphic hyalinizing angiectatic tumor of renal hilum. Ann Diagn Pathol. 2012;16(6):489-93. http://dx.doi.org/10.1016/j.anndiagpath.2011.03.010. PMid:21724433.

12 Kuang P. CT findings of pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts of the neck. Jpn J Radiol. 2013;31(3):204-7. http://dx.doi.org/10.1007/s11604-012-0157-5. PMid:23179786.

13 Rekhi B, Aggarwal S. Pleomorphic hyalinizing angiectatic tumor exhibiting intricate branching vasculature: an interesting pattern in a rare tumor. Indian J Pathol Microbiol. 2013;56(3):321-3. http://dx.doi.org/10.4103/0377-4929.120414. PMid:24152526.

14 Suzuki K, Yasuda T, Hori T, et al. Pleomorphic hyalinizing angiectatic tumor arising in the thigh: a case report. Oncol Lett. 2014;7(4):1249-52. http://dx.doi.org/10.3892/ol.2014.1883. PMid:24944701.

15 Changchien YC, Bocskai P, Kovács I, Hargitai Z, Kollár S, Török M. Pleomorphic hyalinizing angiectatic tumor of soft parts: case report with unusual ganglion-like cells and review of the literature. Pathol Res Pract. 2014;210(12):1146-51. http://dx.doi.org/10.1016/j.prp.2014.04.016. PMid:24857338.

16 Felton SJ, Ramlogan D. Spontaneous bruising overlying a subcutaneous swelling: pleomorphic hyalinizing angiectatic tumor, a relatively new entity of intermediate malignancy. Int J Dermatol. 2015;54(2):204-6. http://dx.doi.org/10.1111/ijd.12003. PMid:24738999.

17 Fan C, Lin X, Yang L, Wang E. A subcutaneous pleomorphic hyalinizing angiectatic tumor of soft parts of the right chest wall: report of a rare case. Int J Clin Exp Pathol. 2015;8(9):11804-8. PMid:26617930.

18 Morency E, Laskin W, Lin X. Cytologic and histologic features of pleomorphic undifferentiated sarcoma arising in a hybrid hemosiderotic fibrolipomatous tumor and pleomorphic hyalinizing angiectatic tumor: report of an unusual case with a literature review. Acta Cytol. 2015;59(6):493-7. http://dx.doi.org/10.1159/000443319. PMid:26841226.

19 Fukunaga M, Ushigome S. Pleomorphic hyalinizing angiectatic tumor of soft parts. Pathol Int. 1997;47(11):784-8. http://dx.doi.org/10.1111/j.1440-1827.1997.tb04458.x. PMid:9413039.

20 Yorita K, Ishihara A, Tokumitsu T, et al. A case of a pleomorphic hyalinizing angiectatic tumor of soft parts with intracytoplasmic hemosiderin pigment apparent upon fine-needle aspiration cytology. Diagn Cytopathol. 2015;43(5):407-11. http://dx.doi.org/10.1002/dc.23233. PMid:25427127.

21 Gallo C, Murer B, Roncaroli F. Pleomorphic hyalinizing angiectasic soft-tissue tumor: description of a case. Pathologica. 1997;89(5):531-5. PMid:9489401.

22 Chu ZG, Liu MQ, Zhu ZY, Lv FJ, Ouyang Y. Pelvic retroperitoneal pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissue: a case report. BMC Med Imaging. 2016;16(1):28. http://dx.doi.org/10.1186/s12880-016-0130-3. PMid:27044420.

23 Silverman JS, Dana MM. Pleomorphic hyalinizing angiectatic tumor of soft parts: immunohistochemical case study shows cellular composition by CD34+ fibroblastos and fator XIIIa+ dendrophages. J Cutan Pathol. 1997;24(6):377-83. http://dx.doi.org/10.1111/j.1600-0560.1997.tb00807.x. PMid:9243366.

24 Brazio PS, Morrison AL, Oh M, Goldberg N, Boutros CN. Large pleomorphic hyalinizing angiectatic tumor of the forearm: a multidisciplinar perspective. Surgery. 2016;159(5):1471-3. http://dx.doi.org/10.1016/j.surg.2015.04.033. PMid:26050546.

25 Brim SP, Allerding TJ, Buck K. Pleomorphic hyalinized angiectatic tumor of soft parts. J Am Podiatr Med Assoc. 1999;89(6):307-11. http://dx.doi.org/10.7547/87507315-89-6-307. PMid:10384757.

26 Kane PM, Gaspar MP, Whiting BB, Culp RW. Pleomorphic hyalinizing angiectatic tumor arising in the hand: a case report. Hand. 2016;11(3):NP20-3. http://dx.doi.org/10.1177/1558944715627275. PMid:27698646.

27 Groisman GM, Bejar J, Amar M, Ben-Izhak O. Pleomorphic hyalinizing angiectatic tumor of soft parts: immunohistochemical study including the expression of vascular endothelial growth factor. Arch Pathol Lab Med. 2000;124(3):423-6. http://dx.doi.org/10.5858/2000-124-0423-PHATOS. PMid:10705399.

28 Fujiwara M, Yuba Y, Wada A, Ozawa T, Tanaka T. Pleomorphic hyalinizing angiectatic tumor of soft parts: report of a case and review of the literature. J Dermatol. 2004;31(5):419-23. http://dx.doi.org/10.1111/j.1346-8138.2004.tb00696.x. PMid:15187312.

29 Onyemkpa C, Oyasiji T. Pleomorphic hyalinizing angiectatic tumor arising in the groin: a case report. Int J Surg Case Rep. 2016;28:227-30. http://dx.doi.org/10.1016/j.ijscr.2016.10.006. PMid:27736710.

30 Ide F, Shimoyama T, Horie N. Pleomorphic hyalinizing angiectactic tumor of the buccal mucosa. J Oral Pathol Med. 2004;33(8):451-3. http://dx.doi.org/10.1111/j.1600-0714.2004.00211.x. PMid:15315480.

31 Scalici Gesolfo C, Serretta V, Di Maida F, et al. Clinical implications of a rare renal entity: Pleomorphic Hyalinizing Angiectatic Tumor (PHAT). Pathol Res Pract. 2017;213(2):165-9. http://dx.doi.org/10.1016/j.prp.2016.11.005. PMid:27894620.

32 Husek K, Veselý K. Pleomorphic hyalinizing angiectatic tumor. Cesk Patol. 2001;37(4):177-81. PMid:11813637.

33 Lin O, Crapanzano JP. Fine-needle aspiration cytology of pleomorphic hyalinized angiectatic tumor: a case report. Diagn Cytopathol. 2005;32(4):238-42. http://dx.doi.org/10.1002/dc.20219. PMid:15754361.

34 Chalmeti A, Arakeri SU, Javalgi AP, Goyal S. Pleomorphic Hyalinizing Angiectatic Tumor: A Rare Case Report and Discussion of Differential Diagnosis. J Clin Diagn Res. 2017;11(8):ED15-6. PMid:28969147.

35 Matsumoto K, Yamamoto T. Pleomorphic hyalinizing angiectatic tumor of soft parts: a case report and literature review. Pathol Int. 2002;52(10):664-8. http://dx.doi.org/10.1046/j.1440-1827.2002.01406.x. PMid:12445140.

36 Lee JC, Jiang XY, Karpinski RH, Moore ED. Pleomorphic hyalinizing angiectatic tumor of soft parts. Surgery. 2005;137(1):119-21. http://dx.doi.org/10.1016/S0039-6060(03)00385-4. PMid:15614293.

37 Jaramillo CJ, Wojcik J, Weber K, Sebro R. Imaging and histological appearance of pleomorphic hyalinizing angiectatic tumors: a case series and literature review. Oncol Lett. 2018;15(4):4720-30. http://dx.doi.org/10.3892/ol.2018.7929. PMid:29552111.

38 El-Tal AEK, Mehregan D. Pleomorphic hyalinizing angiectatic tumor of soft parts: case reporrt and literature review. J Cutan Pathol. 2006;33(5):361-4. http://dx.doi.org/10.1111/j.0303-6987.2006.00416.x. PMid:16640543.

39 Capovilla M, Birembaut P, Cucherousset J, et al. Pleomorphic hyalinizing angiectatic tumor of soft parts: ultrastructural analysis of a case with original features. Ultrastruct Pathol. 2006;30(1):59-64. http://dx.doi.org/10.1080/019131290969442. PMid:16517471.

40 Majtan J, Szep Z, Majernikova A, Rychly B. Pleomorphic hyalinizing angiectatic tumor of soft parts with unusual lipoma-like clinical morphology. Indian J Dermatol Venereol Leprol. 2018;84(5):619-21. http://dx.doi.org/10.4103/ijdvl.IJDVL_693_17. PMid:30073988.

41 Lum D. Atypical pleomorphic hyalinising angiectatic tumour. Pathology. 2006;38(1):80-3. http://dx.doi.org/10.1080/00313020500470133. PMid:16484018.

42 Kazakov DV, Pavlovsky M, Mukensnabl P, Michal M. Pleomorphic hyalinizing angiectatic tumor with a sarcomatous componente recurring as high-grade myxofibrosarcoma. Pathol Int. 2007;57(5):281-4. http://dx.doi.org/10.1111/j.1440-1827.2007.02094.x. PMid:17493176.

43 Rougemont AL, Berczy M, Lin Marq N, McKee TA, Christinat Y. Targeted RNA-sequencing identifies FBXW4 instead of MGEA5 as fusion partner of TGFBR3 in pleomorphic hyalinizing angiectatic tumor. Virchows Arch. 2019;475(2):251-4. http://dx.doi.org/10.1007/s00428-019-02556-2. PMid:30911815.

44 Jaggon JR, Aitken RDC. Pleomorphic hyalinizing angiectatic tumour of soft parts – a case report and review of the literature. West Indian Med J. 2007;56(6):544-6. PMid:18646501.

45 Yamamoto A, Kikuchi Y, Abe S, Ishida T, Kaminaga T. High FDG Uptake in Pleomorphic Hyalinizing Angiectatic Tumor. Clin Nucl Med. 2020;45(5):407-9. http://dx.doi.org/10.1097/RLU.0000000000003001. PMid:32209877.

46 Ke Q, Erbolat, Zhang H, et al. Clinicopathologic features of pleomorphic hyalinizing angiectatic tumor of soft parts. Chin Med J. 2007;120(10):876-81. http://dx.doi.org/10.1097/00029330-200705020-00005. PMid:17571460.

47 Kökoğlu K, Gülmez E, Çağlı S, Yüce I, Deniz K. Pleomorphic hyalinizing angiectatic tumor in the oral cavity: report of a rare case. J Stomatol Oral Maxillofac Surg. 2020;121(2):189-91. http://dx.doi.org/10.1016/j.jormas.2019.02.021. PMid:30836193.

48 Iascone C, Sadighi A, Ruperto M, Paliotta A, Borrini F, Mingazzini P. Pleomorphic hyalinizing angiectatic tumour of the mesorectal soft tissue: a case report and review of the literature. Chir Ital. 2008;60(1):159-63. PMid:18389762.

49 Tallarigo F, Squillaci S, Putrino I, Zizzi N, Bisceglia M. Pleomorphic hyalinizing angiectatic tumor of male breast: A heretofore unreported occurrence. Pathol Res Pract. 2009;205(1):69-73. http://dx.doi.org/10.1016/j.prp.2008.07.010. PMid:18789606.

50 Parameshwarappa S, Rodrigues G, Nagpal N, Rao L. Pleomorphic hyalinizing angiectatic tumor of soft parts involving the upper limb. Indian J Surg. 2010;72(3):263-4. http://dx.doi.org/10.1007/s12262-010-0099-z. PMid:23133262.

51 Cimino-Mathews A, Ali SZ. Pleomorphic hyalinizing angiectatic tumor: cytomorphologic features on fine-needle aspiration. Diagn Cytopathol. 2011;39(3):214-7. PMid:21319325.

52 Labanaris AP, Zugor V, Witt JH, Kühn R. Pleomorphic hyalinizing angiectatic tumor of the scrotum. Urol J. 2011;8(3):248-50. PMid:21910109.

53 Wei S, Pan Z, Siegal GP, Winokur TS, Carroll AJ, Jhala D. Complex analysis of a recurrent pleomorphic hyalinizing angiectatic tumor of soft parts. Hum Pathol. 2012;43(1):121-6. http://dx.doi.org/10.1016/j.humpath.2011.02.023. PMid:21733556.

54 Choong MY, Ke PZ, Huang CY, Li TS. Pleomorphic hyalinizing angiectatic tumor in the inguinal area: a potential diagnostic pitfall. Am Surg. 2012;78(1):E45-6. http://dx.doi.org/10.1177/000313481207800128. PMid:22273308.

55 Weiss SW, Goldblum JR, Folpe AL. Enzinger and Weiss’s soft tissue tumours. 6th ed. Elsevier, Philadelphia; 2014: 986-91.

56 Carter JM, Sukov WR, Montgomery E, et al. TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms. Am J Surg Pathol. 2014;38(9):1182-992. http://dx.doi.org/10.1097/PAS.0000000000000212. PMid:24705316.

57 Boland JM, Folpe AL. Hemosiderotic fibrolipomatous tumor, pleomorphic hyalinizing angiectatic tumor, and myxoinflammatory fibroblastic sarcoma: related or not? Adv Anat Pathol. 2017;24(5):268-77. http://dx.doi.org/10.1097/PAP.0000000000000151. PMid:28375867.

58 Liu H, Sukov WR, Ro JY. The t(1;10)(p22;q24) TGFBR3/MGEA5 translocation in pleomorphic hyalinizing angiectatic tumor, myxoinflammatory fibroblastic sarcoma, and hemosiderotic fibrolipomatous tumor. Arch Pathol Lab Med. 2019;143(2):212-21. http://dx.doi.org/10.5858/arpa.2017-0412-RA. PMid:29979612.

59 Raghavan V, Shivaprashanth K, Rao KR. Pleomorphic hyalinizing angiectatic tumor of soft parts: immunohistochemical study with review of literature. JSS Case Rep e Ver. 2015;1:46-9.

60 Moretti VM, Brooks JS, Lackman RD. Pleomorphic hyalinizing angiectatic tumor: a clinicopathologic characterization. Univ Pa Orthop J. 2010;20:121-4.

61 Clement PB, Stall JN, Young RH. Atlas of Gynecologic surgical pathology. 4th ed. Philadelphia: Elsevier; 2020.

62 Rush AJ 3rd, Patel PD, Shemesh S, Subhawong TK, Pretell-Mazzini J. Treatment modalities and outcomes of pleomorphic hyalinizing angiectatic tumor: a systematic review of the literature. Musculoskelet Surg. 2018;102(3):213-21. http://dx.doi.org/10.1007/s12306-018-0532-1. PMid:29392614.

63 Salim AA, Samman A, Assiri AH. Pleomorphic hyalinizing angiectatic tumor of breast. Hum Pathol Case Rep. 2020;21:20040.
 


Submitted date:
11/11/2021

Accepted date:
12/22/2021

Publication date:
02/11/2022

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