Ewing's sarcoma (EWS) is an uncommon primary malignant tumor of the bone, mostly affects 5–25 years of age with male predominance. Diaphysis of the long bone is usual site of origin with occasional extraskeletal extension. However, it is extremely rare (3%–5%) in the small bones of the hand and feet, hence often misinterpreted as osteomyelitis, aneurysmal bone cyst, giant cell tumor, cartilagenous tumor and osteosarcoma; delaying definitive treatment. Metastasis of calcaneal EWS is common, and prognosis is dismal. Multimodality treatment approach is the standard care rendered for it. Herein, we report such a case of EWS in a 12-year-old girl child at an unusual site of calcaneum with skip metastasis for which the diagnosis and treatment was deferred by 5 months. Only after histopathology and immunohistochemistry confirmation, the definitive chemotherapy was provided and surgery was scheduled after five cycles of chemotherapy. The patient is now on chemotherapy and followed up till today.
Background:
One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping histomorphology and immunohistochemistry (IHC) findings. Limited, small biopsy of these not completely excisable tumors adds to the diagnostic confusion.
Materials and Methods:
A cross-sectional study was done for 2 years (January 2018–December 2020) in a tertiary care institute, which included 70 cases of tumors of which 49 cases were malignant. All paraffin-embedded blocks were subjected to hematoxylin and eosin stain and IHC followed by molecular study wherever needed.
Results:
Of the total cases, small-blue-round-cell tumor constituted the major category comprising 20 rare and interesting cases which included sinonasal undifferentiated carcinoma (4 cases), malignant lymphoma (2 cases of diffuse large B-cell lymphoma and 2 cases of extranodal natural killer/T-cell lymphoma), rhabdomyosarcoma (2 cases), olfactory neuroblastoma (2 cases), malignant melanoma (2 cases), plasmacytoma (2 cases), atypical Ewing’s sarcoma (EWS) (1 case), EWS (1 case), nuclear protein in testis (NUT) carcinoma (1 case), and small-cell neuroendocrine carcinoma (1 case).
Conclusion:
Tumors of the sinonasal tract are very diverse, more so in small-round-cell tumor which present with a undifferentiated morphology. Thus, accurate diagnosis needs clinicoradiological parameters and special ancillary techniques such as IHC and molecular study in addition to histopathology for early diagnosis and therapy to prevent significant morbidity and mortality caused in these tumors.
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