Case Report | DOI: https://doi.org/10.31579/2690-1897/059
*Corresponding Author: arveen Kumar, Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalya, New Delhi, India.Kumar P., Singh L., Mathur G. (2021) The Diagnostic Pitfall for Angiosarcoma in one year old child with Extremity Tumor. J Surg. Cas Repo and Imag 4(1); DOI:10.3
Citation: Kumar P., Singh L., Mathur G. (2021) The Diagnostic Pitfall for Angiosarcoma in one year old child with Extremity Tumor. J Surg. Cas Repo and Imag 4(1); DOI:10.31579/2690-1897/059
Copyright: © 2021 Parveen Kumar, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 20 January 2021 | Accepted: 23 January 2021 | Published: 19 February 2021
Keywords: pediatric; infant; rhabdomyosarcoma; endothelium; vascular tumor
Pediatric angiosarcomas are very rare and less studied. There exists a significant degree of confusion in histologically differentiating angiosarcomas from other endothelium derived tumors. We present here a case of right forearm mass in an infant with diagnostic dilemma, which later turned out to be angiosarcoma.
Angiosarcoma is an aggressive malignant endothelial cell tumor of lymphatic or vascular origin. They are rare tumors that usually affects adults/elderly patient. Pediatric angiosarcomas are exceedingly rare, poorly studied and a very little is known about its line of treatment. The classic presentation is an enlarging, painful mass of several weeks’ duration and usually associated with anemia and/or thrombocytopenia.[1] Due to the variability in the appearance of angiosarcoma, the correct diagnosis can often be delayed. It is characterized by polymorphic and non-specific clinical and radiological features. These lesions may have a destructive pattern on radiology. Immunohistochemistry support is often needed as vasoformative architecture usually get missed on light microscopy. Treatment modalities are variable depending on tumor extent, resectability and metastasis.[1] The prognosis is often dismal.
A 1-year-old male child presented with right forearm mass for 6 months. It was progressively increasing in size associated with foul smelling watery discharge [Fig.1a]. The incisional biopsy had more of necrotic tumor, causing interpretation of IHC difficult. The tissue had spindle cells and desmin positivity and was reported as suspected case of sarcoma possibly embryonal RMS. Contrast enhanced computer tomography (CECT) showed soft tissue density mass lesion in muscular layer of lateral and posterior compartment of right forearm, with encasement of neurovascular bundle and elbow joint, multiple enlarged supra-trochlear lymph nodes. FDG PET CT showed heterogenous enhancing mass with involvement of skin. FDG uptake was positive in supra-trochlear lymph nodes, right axillary lymph nodes, occipital and sphenoid bones.
Figure 1a: Right forearm mass.
He received 12 weeks of VAC (Vincristine, Actinomycin D and Cyclophosphamide) neo-adjuvant chemotherapy and had partial response (>50% size reduction) [Fig.1b]. He underwent above elbow amputation for mass and histo-pathological examination showed tumor with ramifying vascular spaces, showing mild to moderate atypia [Fig. 2a, 2b]. The vessels were diffusely infiltrating into dermis, extending into subcutaneous tissue and skeletal muscle. The tumor cells were immune- positive for CD31, CD34 and D2-40 and final reporting was angiosarcoma [Fig.2c]. Post-operatively he developed edema of amputated stump and later child succumbed to sepsis secondary to neutropenia.
Figure 1b: Partial response status post neo-adjuvant chemotherapy.
Figure 2a: Photomicrograph showing tumor comprising of ramifying vascular spaces which are lined by hobnail endothelial cell (H&E;100).
Figure 2b: Endothelial cell lining the vascular spaces shows mild to
moderate atypia.
Figure 2c: Endothelial cell show positivity for CD 34.
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