Primitive Neuroectodermal Tumors of Adrenal Gland

Abstract

Adrenal primitive neuroectodermal tumor is a very rare tumor. It originates in primitive neuroectoderma and is found mainly in 20-30-year-old young populations. It has non-specific clinical or imaging manifestation and its diagnosis is mostly based on pathological examinations. The tumor is fast-developing, highly malignant with poor prognosis.

Latent adrenal Ewing sarcoma family of tumors: A case report

“…In 1973, both tumors were defined as PNETs. In 1979, Askin tumors were 60 defined as PNETs in the thoracic cavity [1]. They are, now, collectively termed as ESFT.…”

“…We therefore diagnosed primary adrenal ESFT on the basis of pathological 71 examination. ESFT of adrenal gland is a very rare disease and only a few cases have been 72 reported so far [1,[3][4][5][6][7][8][9][10][11]. Molecular analysis of ESFT reveals a reciprocal translocation of t 73 (11; 22) (q24; q12) and t (21; 22) (q22; q12) [12].…”

“…ESFT 20 often arises in the bone, chest wall, and soft tissues of the thoracic region. However, ESFT 21 that arises from the adrenal gland is rarer and is often associated with clinical symptoms [1]. 22…”

Primitive neuroectodermal adrenal gland tumour

“…[3][4][5][6][7][8][9][10][11][12] As in our patient, CD99, a highly sensitive marker for PNET, was found in all 16 of these patients. Although not routinely looked for, as in our patient over 90% of PNET cases exhibit a reciprocal translocation of t(11;22)(q24;q12) involving the Abbreviations: chemo = chemotherapy; DVT = deep vein thrombosis; IE = ifosfamide and etoposide; N/A = data not available or not reported; RT = radiotherapy; VAC = vincristine, doxorubicin and cyclophosphamide * All surgeries were curative intent and all chemotherapies were adjuvant intent † The patient had R1 resection of the adrenal tumour and refused chemo-and radio-therapies initially.…”

“…4,7,8,12,13 Nevertheless, in a few patients the tumour behaves indolently or has a relatively quiescent period before rapid growth. A case of incidental PNET at autopsy has been reported in a 26-year-old man who committed suicide by hanging himself; his latent PNET measured 8 cm.…”