Ewing’s Sarcoma of the Adrenal Gland

Pal, Dilip Kumar; Chandra, Vikash; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

doi:10.21699/ajcr.v7i3.406

Cited by 4 publications

(2 citation statements)

References 8 publications

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“…1,[4][5][6] In Indian literature, there are just 5 case reports of Adrenal ES/PNET prior to our case. [5][6][7][8][9] Most patients ES/PNET of adrenal gland present with abdominal pain, abdominal distension, palpable tumor mass and non-specific symptoms like anorexia, weight loss, fatigue and fever. 1 However, patients usually show no specific changes in their routine and biochemical laboratory tests.…”

Section: Discussionmentioning

confidence: 99%

Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

Jagdale

Pol²

2021

IJPO

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Ewing sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) is a malignant small round blue cell tumor of neuroectodermal origin that affects bones and soft tissue in children and young adults. ES/PNET is very uncommon in parenchymal organs. We report a case of primary adrenal PNET in a young female having pregnancy induced persistent hypertension. She underwent right adrenalectomy for a large adrenal mass. On histopathology, it was typical malignant round cell tumor (MRCT). Immunohistochemistry confirmed the diagnosis of ES/PNET with expression of CD99, FLI-1 and NKX2.2. Until now, only 38 cases of primary adrenal ES/PNET have been reported in the English literature with just 5 cases from India. Adrenal ES/PNET can be mistaken with other MRCTs like Non-Hodgkin lymphoma and neuroblastoma. Since the biology, treatment and prognosis of these tumors is entirely different, we emphasize that pathologists must be aware of this possibility and try to differentiate them using ancillary techniques.

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Section: Discussionmentioning

confidence: 99%

Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

Jagdale

Pol²

2021

IJPO

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“…The mean age of patients was 22.8 years, and the oldest patient was 57 years old. Recently, additional reports regarding adrenal Ewing's sarcoma have been published ( 25 - 31 ), and the oldest case among those was 63 years old. Therefore, to our knowledge, the present 74-year-old patient is the oldest reported patient with adrenal Ewing's sarcoma.…”

Section: Discussionmentioning

confidence: 99%

Adrenal Ewing's Sarcoma in an Elderly Man

et al. 2018

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Ewing's sarcoma usually arises in the bones of children and adolescents. We herein report a 74-year-old man with Ewing's sarcoma in the adrenal gland. The diagnosis was confirmed by a genetic test, pathological studies, and several imaging studies. He already had multiple liver metastases when he was transferred to our hospital and died on the 37th day. The diagnosis was further confirmed by autopsy studies. Adrenal Ewing's sarcoma is very rare, and our patient was older than other reported cases. Ewing's sarcoma should be considered even in elderly patients with adrenal tumors.

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Ewing sarcoma of the adrenal gland: a case report and review of the literature

et al. 2018

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BackgroundEwing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy.Case presentationWe report the case of a 23-year-old Moroccan woman presenting with flank pain. An abdominal computed tomography scan showed a large and enhancing left suprarenal mass. After radical nephrectomy, histologic examination revealed a small round cell proliferation. The diagnosis of Ewing sarcoma was confirmed by molecular analysis; time to final diagnosis was 5 months due to financial and coordination issues. Computed tomography (on an asymptomatic patient) revealed a locoregional recurrence, our patient received 12 cycles of the vincristine, doxorubicin and cyclophosphamide/ifosfamide and etoposide protocol used in an alternating schedule, with partial radiologic response (62%) and pathologic complete response, then underwent adjuvant radiotherapy of 45 Gy. The young women is still in remission after 36 months of follow-up.ConclusionsOur patient had an early recurrence due to absence of adjuvant treatment, but did respond well to neoadjuvant chemotherapy with a pathologic complete response. Management of adrenal Ewing sarcoma could be extrapolated from skeletal one with good outcomes even in locoregional recurrence.

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Ewing’s Sarcoma of the Adrenal Gland

Cited by 4 publications

References 8 publications

Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

Adrenal Ewing's Sarcoma in an Elderly Man

Ewing sarcoma of the adrenal gland: a case report and review of the literature

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