A Case Report of Scapular Hemangioma with Kasabach–Merritt Syndrome: An Insight into Various Treatment Strategies with Review of Literature

Rajesh Bahadur Singh; Nitesh Joshi; Saravana Kumar Jagannathan; Raghawesh Ranjan; Zachariah Chowdhury; Paramita Rudra Pal

doi:10.1055/s-0045-1806777

Indian Journal of Medical and Paediatric Oncology, Inhaltsverzeichnis

CC BY 4.0 · Indian J Med Paediatr Oncol
DOI: 10.1055/s-0045-1806777

Case Report with Review of Literature

A Case Report of Scapular Hemangioma with Kasabach–Merritt Syndrome: An Insight into Various Treatment Strategies with Review of Literature

Authors

Rajesh Bahadur Singh

¹Orthopaedic Oncosurgery Unit, Department of Surgical Oncology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India
Nitesh Joshi

²Department of Surgical Oncology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India
Saravana Kumar Jagannathan

¹Orthopaedic Oncosurgery Unit, Department of Surgical Oncology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India
Raghawesh Ranjan

³Department of Paediatric Oncology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India
Zachariah Chowdhury

⁴Department of Oncopathology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India
Paramita Rudra Pal

⁴Department of Oncopathology, Mahamana Pandit Madanmohan Malaviya Cancer Centre and Homi Bhabha Cancer Hospital, Tata Memorial Centre (Homi Bhabha National Institute), Varanasi, Uttar Pradesh, India

Abstract

Hemangiomas are common tumors, accounting for 4 to 5% of all benign tumors in infancy and childhood. While they typically occur superficially, their presence in bone is extremely rare. This case report involves an 8-year-old female patient with a left scapular hemangioma, thrombocytopenia, and consumptive coagulopathy (Kasabach–Merritt syndrome). The patient presented with a slow-growing osseous lump in the left scapular region over 3 years. Magnetic resonance imaging revealed an expansile lytic lesion originating from the left scapula, with a large soft tissue component, areas of hemorrhage, necrosis, and bony destruction. A preoperative biopsy suggested hemangioma. During evaluation, the patient was found to have a low platelet count and consumptive coagulopathy, leading to a diagnosis of Kasabach–Merritt syndrome. The patient was managed with multimodal drug therapy. Due to the large size and progression of the lesion, a total scapulectomy was performed. Postoperatively, blood parameters normalized. Final histopathology confirmed a cavernous hemangioma. No recurrence or derangement in the coagulation profile was observed at the 6-month follow-up through clinical and X-ray examinations. This report is presented due to its exceptional rarity.

Keywords

hemangioma - scapula - Kasabach–Merritt syndrome - consumptive coagulopathy - angio-embolization

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Referenzen

References
1 Okumura T, Asamura H, Kondo H, Matsuno Y, Tsuchiya R. Hemangioma of the rib: a case report. Jpn J Clin Oncol 2000; 30 (08) 354-357
2 Politi M, Romeike BF, Papanagiotou P. et al. Intraosseous hemangioma of the skull with dural tail sign: radiologic features with pathologic correlation. AJNR Am J Neuroradiol 2005; 26 (08) 2049-2052
3 Ching BC, Wong JS, Tan MH, Jara-Lazaro AR. The many faces of intraosseous haemangioma: a diagnostic headache. Singapore Med J 2009; 50 (05) e195-e198
4 Lewis D, Vaidya R. Kasabach-Merritt Syndrome. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2024
5 Gourgiotis S, Piyis A, Panagiotopoulos N, Panayotopoulos P, Salemis NS. Cavernous hemangioma of the rib: a rare diagnosis. Case Rep Med 2010; 2010: 254098
6 Verbeke SL, Bovée JV. Primary vascular tumors of bone: a spectrum of entities?. Int J Clin Exp Pathol 2011; 4 (06) 541-551
7 Li W, Zou F, Dai M, Zhang B, Nie T. A rare case of pure primary hemangioma of the scapula: a case report. Oncol Lett 2015; 10 (04) 2265-2268
8 Abrão FC, Tamagno M, Canzian M. et al. Hemangioma of the rib. Ann Thorac Surg 2011; 91 (02) 595-596
9 Abass K, Saad H, Kherala M, Abd-Elsayed AA. Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature. Cases J 2008; 1 (01) 9
10 Haisley-Royster C, Enjolras O, Frieden IJ. et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. [published correction appears in J Pediatr Hematol Oncol 2002 Dec;24(9):794] J Pediatr Hematol Oncol 2002; 24 (06) 459-462