Primitive neuroectodermal tumors of adrenal gland

Jpn J Clin Oncol. 2010 Aug;40(8):800-4. doi: 10.1093/jjco/hyq050. Epub 2010 Apr 29.

Abstract

Objectives: To analyze the clinical and pathological characteristics of adrenal primitive neuroectodermal tumors for a better understanding of the disease.

Methods: A retrospective analysis of four cases of adrenal primitive neuroectodermal tumors (two male, two female; age 21-30, average 24) was made. All patients went through necessary endocrinological exams, computer tomography scans (for site-specific diagnoses) and pathological tests.

Results: No positive result was reported in routine laboratory tests and endocrinological exams. Computer tomography scans showed bulk soft tissue masses with rough borders. The masses were 8-17 cm in diameter with solid-cystic changes. Among the four patients, one refused to receive treatment after definitiver diagnosis through needle biopsy, three received surgical treatments and their post-operative pathological exams all confirmed the diagnoses of primitive neuroectodermal tumors. During the follow-ups, the untreated patient died 6 months afterwards, one patient died 8 months after palliative treatment; one patient showed distant metastasis 13 months after surgery and did not respond well to both radio- and chemotherapy; one patient had local recurrence 1 month after surgery and is presently undergoing chemotherapy.

Conclusions: 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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Gland Neoplasms / diagnosis*
  • Adrenal Gland Neoplasms / pathology*
  • Adrenal Gland Neoplasms / therapy
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Chemotherapy, Adjuvant
  • Fatal Outcome
  • Female
  • Follow-Up Studies
  • Humans
  • Laparotomy
  • Lymphatic Metastasis
  • Male
  • Neoplastic Cells, Circulating / pathology
  • Neoplastic Cells, Circulating / ultrastructure
  • Neuroectodermal Tumors / diagnosis*
  • Neuroectodermal Tumors / pathology*
  • Neuroectodermal Tumors / secondary
  • Neuroectodermal Tumors / therapy
  • Radiographic Image Enhancement
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Vena Cava, Inferior / diagnostic imaging