Disseminated histiocytic sarcoma in a child: a clinicopathological dichotomy with 8 years survival

Abstract

A 3-year-old girl presented with a non-healing ulcer, originating as a pustule over the right anterior chest wall of 1½ month duration associated with high fever. A subcutaneous nodule along with right apical and anterior axillary lymph nodes was palpable. Abdominal ultrasound and chest skiagram were normal. Fine needle aspiration cytology (FNAC) from the axillary lymph node was suggestive of embryonal rhabdomyosarcoma. Bone marrow aspirate was normal. The ulcer and subcutaneous nodule were excised completely with adequate margins. Histopathological examinations were compatible with malignancy of histiocytic origin with clear resected margins. The axillary nodes were free of tumour. Adjuvant chemotherapy was given for 13 months. The patient is doing well at 8 years follow-up.

Figure 1

Clinical photograph showing (A) the ulcer with the excised subcutaneous nodule and anterior axillary lymph node placed on its anatomical location (B) Excised ulcer with its base with a healthy margin of 0.5 cm from depth and 1cm all around. (C) Wound closure with a full thickness flap closure over a suction drain (with kind permission from Publishers Jaypee Brothers, New Delhi). (D) Scar of wound at follow-up.

Figure 2

Photomicrograph shows dense dermal infiltration of a pleomorphic population of cells with focal ulceration of the overlying epidermis (A, H&E×40). The epidermis shows marked spongiosis, pseuo-epitheliomatous hyperplasia and infiltrate by the tumour cells (B, H&E×40). Higher power photomicrograph shows a discrete population of round to epithelioid cells with vesicular nucleus, prominent nucleoli and moderate amount of dense eosinophilic cytoplasm. Frequent mitotic figures are also noted. The tumour cells are interspersed by occasional small mature lymphocytes (C, H&E×400). Focal necrosis is identified in the lesion (D, H&E×40). The tumour cells show distinct peritheliomatous arrangement as well as vascular infiltrate (E and F, H&E; e×100; f×200). The tumour cells are strongly positive for CD 68 immunostain (G, IHC, CD 68×100), while the cells show very occasional positivity for S-100 immunostain (H, IHC, S-100 protein×100).