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Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature

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Abstract

Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan–guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.

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References

  1. Mirra JM, Kessler S, Bhuta S, Eckardt J (1992) The fibroma-like variant of epithelioid sarcoma: a fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors. Cancer 69:1382–1395

    Article  CAS  PubMed  Google Scholar 

  2. Billings SD, Folpe AL, Weiss SW (2003) Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol 27:48–57. https://doi.org/10.1097/00000478-200301000-00006

    Article  PubMed  Google Scholar 

  3. Hornick JL, Fletcher CD (2011) Pseudomyogenic hemangioendothelioma: a distinctive, often multicentric tumor with indolent behavior. Am J Surg Pathol 35:190–201. https://doi.org/10.1097/PAS.0b013e3181ff0901

    Article  PubMed  Google Scholar 

  4. Jo VY, Fletcher CD (2014) WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology 46:95–104. https://doi.org/10.1097/PAT.0000000000000050

    Article  CAS  PubMed  Google Scholar 

  5. Amary MF, O’Donnell P, Berisha F et al (2013) Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases. Skeletal Radiol 42:947–957. https://doi.org/10.1007/s00256-013-1577-8

    Article  PubMed  Google Scholar 

  6. Al-Qaderi A, Mansour AT (2019) Pseudomyogenic hemangioendothelioma. Arch Pathol Lab Med 143:763–767. https://doi.org/10.5858/arpa.2017-0430-RS

    Article  CAS  PubMed  Google Scholar 

  7. Inyang A, Mertens F, Puls F et al (2016) Primary pseudomyogenic hemangioendothelioma of bone. Am J Surg Pathol 40:587–598. https://doi.org/10.1097/PAS.0000000000000613

    Article  PubMed  Google Scholar 

  8. Lefebvre C, Glanville J, Briscoe S, Littlewood A, Marshall C, Metzendorf M-I, Noel-Storr A, Rader T, Shokraneh F, Thomas J, Wieland LS. Chapter 4: searching for and selecting studies. In: Higgins JPT, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, Welch VA (editors). Cochrane handbook for systematic reviews of interventions version 6.2 (updated February 2021). Cochrane, 2021. Available from www.training.cochrane.org/handbook

  9. Murshed KA, Torres-Mora J, ElSayed AM, Ammar A, Al-Bozom I (2021) Pseudomyogenic hemangioendothelioma of bone with rare WWTR1-FOSB fusion gene: case report and literature review. Clin Case Rep 9:1494–1499. https://doi.org/10.1002/ccr3.3808

    Article  PubMed  PubMed Central  Google Scholar 

  10. Rekhi B, Ramadwar M (2021) Cytomorphological features of an unusual case of a multifocal pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma in a young adult male. Diagn Cytopathol 49(3):E106–E112. https://doi.org/10.1002/dc.24606

    Article  PubMed  Google Scholar 

  11. Rekhi B, Rane S, Puri A (2021) Two cases of intraosseous pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma with unusual features, expanding the clinicopathological spectrum. Int J Surg Pathol 29:454–461. https://doi.org/10.1177/1066896920951841

    Article  CAS  PubMed  Google Scholar 

  12. Sun Y, Zhao M, Lao IW, Yu L, Wang J (2020) The clinicopathological spectrum of pseudomyogenic hemangioendothelioma: report of an additional series with review of the literature. Virchows Arch 477:231–240. https://doi.org/10.1007/s00428-020-02753-4

    Article  CAS  PubMed  Google Scholar 

  13. Choi ME, Lim DJ, Chang SE, Lee MW, Choi JH, Lee WJ (2020) A case of pseudomyogenic hemangioendothelioma of the lower extremity. Ann Dermatol 32:426–429. https://doi.org/10.5021/ad.2020.32.5.426

    Article  PubMed  PubMed Central  Google Scholar 

  14. Plouznikoff N, Mourad SA, Turpin S, Turcotte RE, Mitchell DA, Lambert R (2020) Case report 18F-FDG PET/CT of pseudomyogenic hemangioendothelioma. Clin Nucl Med 45:368–369. https://doi.org/10.1097/RLU.0000000000002973

    Article  PubMed  Google Scholar 

  15. Wei J, Liao Z, Zhao G, Nahar N, Zhang C, Lu J, Yang Y, Yang J (2020) Clinicopathological features of pseudomyogenic hemangioendothelioma and precision therapy based on whole exome sequencing. Cancer Commun (Lond) 40:197–201. https://doi.org/10.1002/cac2.12020

    Article  PubMed  Google Scholar 

  16. Liu Y (2020) Pseudomyogenic Hemangioendothelioma: Distinctive FDG PET/CT Findings with numerous multilayer lesions in a single distal extremity. Clin Nucl Med 45:248–249. https://doi.org/10.1097/RLU.0000000000002895

    Article  PubMed  Google Scholar 

  17. Xia RH, Zhu L, Wang LZ, Tian Z, Zhang CY, Hu YH, Gu T, Li J (2020) Primary pseudomyogenic hemangioendothelioma of right maxilla: a case with immunohistochemistry and FOSB rearrangement study. Oral Surg Oral Med Oral Pathol Oral Radiol 130:e96–e105. https://doi.org/10.1016/j.oooo.2019.12.013

    Article  PubMed  Google Scholar 

  18. Panagopoulos I, Lobmaier I, Gorunova L, Heim S (2019) Fusion of the genes WWTR1 and FOSB in pseudomyogenic hemangioendothelioma. Cancer genomics proteomics 16:293–298. https://doi.org/10.21873/cgp.20134.

  19. Otani S, Nakayama R, Sekita T, Hirozane T, Asano N, Nishimoto K, Sasaki A, Okita H, Morioka H, Nakamura M, Matsumoto M (2019) Pseudomyogenic hemangioendothelioma of bone treated with denosumab: a case report. BMC Cancer 19:872. https://doi.org/10.1186/s12885-019-6072-8

    Article  PubMed  PubMed Central  Google Scholar 

  20. Dianat S, Yousaf H, Murugan P, Marette S (2019) Pseudomyogenic hemangioendothelioma-a case report and review of the literature. Radiol Case Rep 14:1228–1232. https://doi.org/10.1016/j.radcr.2019.06.029

    Article  PubMed  PubMed Central  Google Scholar 

  21. Kosemehmetoglu K, Rekhi B, Wakely PE Jr, Pant V, Dervisoglu S, Aydingoz U (2019) Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: clinicopathologic features of 5 cases. Ann Diagn Pathol 41:116–123. https://doi.org/10.1016/j.anndiagpath.2019.06.003

    Article  PubMed  Google Scholar 

  22. Danforth OM, Tamulonis K, Vavra K, Oh C, Brickman A, Ebersole J, Cameron J, Mahon B, Kent P (2019) Effective use of sirolimus and zoledronic acid for multiosteotic pseudomyogenic hemangioendothelioma of the bone in a child: case report and review of literature. J Pediatr Hematol Oncol 41:382–387. https://doi.org/10.1097/MPH.0000000000001459

    Article  PubMed  Google Scholar 

  23. Squillaci S, Pitino A, Spairani C, Rassu PC, Chiapuzzo E, Kutzner H (2018) Primary pseudomyogenic hemangioendothelioma of bone: case report and review of the literature. Pathologica 110:96–101

    CAS  PubMed  Google Scholar 

  24. Agaram NP, Zhang L, Cotzia P, Antonescu CR (2018) Expanding the spectrum of genetic alterations in pseudomyogenic hemangioendothelioma with recurrent novel ACTB-FOSB gene fusions. Am J Surg Pathol 42(12):1653–1661. https://doi.org/10.1097/PAS.0000000000001147

    Article  PubMed  PubMed Central  Google Scholar 

  25. Pradhan D, Schoedel K, McGough RL, Ranganathan S, Rao UNM (2018) Pseudomyogenic hemangioendothelioma of skin, bone and soft tissue-a clinicopathological, immunohistochemical, and fluorescence in situ hybridization study. Hum Pathol 71:126–134. https://doi.org/10.1016/j.humpath.2017.10.023

    Article  PubMed  Google Scholar 

  26. Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K (2018) Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma. Pediatr Blood Cancer 65(2). https://doi.org/10.1002/pbc.26781.

  27. Ozeki M, Nozawa A, Kanda K, Hori T, Nagano A, Shimada A, Miyazaki T, Fukao T (2017) Everolimus for treatment of pseudomyogenic hemangioendothelioma. J Pediatr Hematol Oncol 39:e328–e331. https://doi.org/10.1097/MPH.0000000000000778

    Article  PubMed  Google Scholar 

  28. Ye C, Yu X, Zeng J, Liu H, Dai M (2016) Pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia of the left lower extremity in a 14-year-old female: a case report. World J Surg Oncol 14:198. https://doi.org/10.1186/s12957-016-0955-9

    Article  PubMed  PubMed Central  Google Scholar 

  29. Rekhi B, Gulia A, Rangarajan V (2016) A rare case of multifocal pseudomyogenic hemangioendothelioma, involving soft tissues and bone, misdiagnosed as a rhabdomyosarcoma: diagnostic and treatment implications. Indian J Pathol Microbiol 59:382–385. https://doi.org/10.4103/0377-4929.188144

    Article  PubMed  Google Scholar 

  30. Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V (2015) Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma. Clin Sarcoma Res 5:22. https://doi.org/10.1186/s13569-015-0037-8

    Article  PubMed  PubMed Central  Google Scholar 

  31. Shah AR, Fernando M, Musson R, Kotnis N (2015) An aggressive case of pseudomyogenic haemangioendothelioma of bone with pathological fracture and rapidly progressive pulmonary metastatic disease: case report and review of the literature. Skeletal Radiol 44:1381–1386. https://doi.org/10.1007/s00256-015-2168-7

    Article  PubMed  Google Scholar 

  32. Davis AT, Guo AM, Phillips NJ, Greenberg DD (2015) A novel treatment for bone lesions of multifocal epithelioid sarcoma-like hemangioendothelioma. Skeletal Radiol 44:1013–1019. https://doi.org/10.1007/s00256-014-2089-x

    Article  PubMed  Google Scholar 

  33. Righi A, Gambarotti M, Picci P, Die Toss AP, Vanel D (2015) Primary pseudomyogenic hemangioendothelioma of bone: report of two cases. Skeletal Radiol 44(5):727–731. https://doi.org/10.1007/s00256-014-2024-1

    Article  PubMed  Google Scholar 

  34. Walther C, Tayebwa J, Lilljebjörn H, Magnusson L, Nilsson J, von Steyern FV, Øra I, Domanski HA, Fioretos T, Nord KH, Fletcher CD, Mertens F (2014) A novel SERPINE1-FOSB fusion gene results in transcriptional up-regulation of FOSB in pseudomyogenic haemangioendothelioma. J Pathol 232:534–540. https://doi.org/10.1002/path.4322

    Article  CAS  PubMed  Google Scholar 

  35. Karakasli A, Karaaslan A, Erduran M, Capkin S, Tuna EB, Havitcioglu H (2014) Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma with bone invasion. J Orthop 11:197–199. https://doi.org/10.1016/j.jor.2014.06.019

    Article  PubMed  PubMed Central  Google Scholar 

  36. Amary MF, O’Donnell P, Berisha F, Tirabosco R, Briggs T, Pollock R, Flanagan AM (2013) Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases. Skeletal Radiol 42:947–957. https://doi.org/10.1007/s00256-013-1577-8

    Article  PubMed  Google Scholar 

  37. McGinity M, Bartanusz V, Dengler B, Birnbaum L, Henry J (2013) Pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, fibroma-like variant of epithelioid sarcoma) of the thoracic spine. Eur Spine J 22:S506–S511. https://doi.org/10.1007/s00586-013-2727-3

    Article  PubMed  Google Scholar 

  38. Sheng WQ, Wang J (2012) Primary pseudomyogenic haemangioendothelioma of bone. Histopathology 61:1219–1224. https://doi.org/10.1111/j.1365-2559.2012.04347.x

    Article  CAS  PubMed  Google Scholar 

  39. Hung YP, Fletcher CD, Hornick JL (2017) FOSB is a useful diagnostic marker for pseudomyogenic hemangioendothelioma. Am J Surg Pathol 41:596–606. https://doi.org/10.1097/PAS.0000000000000795

    Article  PubMed  Google Scholar 

  40. Doyle LA, Fletcher CD, Hornick JL (2016) Nuclear expression of CAMTA1 distinguishes epithelioid hemangioendothelioma from histologic mimics. Am J Surg Pathol 40:94–102. https://doi.org/10.1097/PAS.0000000000000511

    Article  PubMed  Google Scholar 

  41. Caballero GA, Roitman PD (2020) Pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma). Arch Pathol Lab Med 144:529–533. https://doi.org/10.5858/arpa.2018-0395-RS

    Article  CAS  PubMed  Google Scholar 

  42. Wagner AJ, Malinowska-Kolodziej I, Morgan JA et al (2010) Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 28:835–840. https://doi.org/10.1200/JCO.2009.25.2981

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Online Mendelian Inheritance in Man, OMIM®. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD), World Wide Web URL: http://omim.org/, Accessed date: 12 June 2021.

  44. Fleisch H (1998) Bisphosphonates: mechanisms of action. Endocr Rev 19:80–100. https://doi.org/10.1210/edrv.19.1.0325

    Article  CAS  PubMed  Google Scholar 

  45. Drake MT, Clarke BL, Khosla S (2008) Bisphosphonates: mechanism of action and role in clinical practice. Mayo Clinic Proc 83:1032–1045. https://doi.org/10.4065/83.9.1032

    Article  CAS  Google Scholar 

  46. Odri GA, Dumoucel S, Picarda G et al (2010) Zoledronic acid as a new adjuvant therapeutic strategy for Ewing’s sarcoma patients. Cancer Res 70:7610–7619. https://doi.org/10.1158/0008-5472.CAN-09-4272

    Article  CAS  PubMed  Google Scholar 

  47. Brance ML, Castiglioni A, Cóccaro N, Palatnik M (2017) Two cases of Gorham-Stout disease with good response to zoledronic acid treatment. Clin Cases Miner Bone Metab 14:250–253. https://doi.org/10.11138/ccmbm/2017.14.2.250.

  48. Meyers PA, Healey JH, Chou AJ, Wexler LH, Merola PR, Morris CD, Laquaglia MP, Kellick MG, Abramson SJ, Gorlick R (2011) Addition of pamidronate to chemotherapy for the treatment of osteosarcoma. Cancer 117:1736–1744. https://doi.org/10.1002/cncr.25744

    Article  CAS  PubMed  Google Scholar 

  49. Piperno-Neumann S, Le Deley MC, Rédini F et al (2016) Zoledronate in combination with chemotherapy and surgery to treat osteosarcoma (OS2006): a randomised, multicentre, open-label, phase 3 trial. Lancet Oncol 17:1070–1080. https://doi.org/10.1016/S1470-2045(16)30096-1

    Article  CAS  PubMed  Google Scholar 

  50. Coppo P, Lassoued S, Billey T, Lassoued K (2005) Successful treatment of osteolytic epithelioid hemangioendothelioma with pamidronate. Clin Exp Rheumatol 23:400–401

    CAS  PubMed  Google Scholar 

  51. Tang L, Chen G, Wang Q, John J, Lu C (2019) Bisphosphonates as a therapeutic choice for multifocal epithelioid hemangioma of bone: a case report. Medicine (Baltimore) 98:e18161. https://doi.org/10.1097/MD.0000000000018161

    Article  PubMed  Google Scholar 

  52. D’Oronzo S, Wood S, Brown JE (2021) The use of bisphosphonates to treat skeletal complications in solid tumours. Bone 147:115907. https://doi.org/10.1016/j.bone.2021.115907

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Bone Biology Laboratory, School of Medicine, Rosario National University, Santa Fe, Argentina

    María Lorena Brance & Lucas R. Brun

  2. Reumatología Y Enfermedades Óseas Rosario, Santa Fe, Argentina

    María Lorena Brance

  3. National Council of Scientific and Technical Research (CONICET), Buenos Aires, Argentina

    María Lorena Brance & Lucas R. Brun

  4. Department of Imagen, Sanatorio Británico, Rosario, Argentina

    Nicolás M. Cóccaro

  5. Pathology Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

    Pablo Roitman

  6. Orthopaedic Oncology, Sanatorio Británico, Rosario, Argentina

    Alejandro Castiglioni, Florencia Agostinis & Mariel Spense

  7. Sanatorio Británico, Rosario, Argentina

    Bárbara Scheitlin

  8. Department of Radiation Oncology, Centro de Radioterapia, Rosario, Argentina

    Nicholas Rene

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  1. María Lorena Brance
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Contributions

MLB referred the patient and guided the clinical and research evaluations. NMC delineated the radiological findings, PR described the anatomophatologic findings, and all authors participated in the discussion and clinical case management. MLB and LRB performed the systematic review and drafted the manuscript. All authors read and approved the submitted manuscript.

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Written informed consent was obtained to publish the patient’s data and images (sentence included in the manuscript).

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Correspondence to María Lorena Brance.

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Brance, M.L., Cóccaro, N.M., Roitman, P. et al. Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature. Arch Osteoporos 17, 28 (2022). https://doi.org/10.1007/s11657-022-01062-4

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