Medullomyoblastoma: teratoid nature of a quite rare neoplasm


Er U., Yigitkanli K., Kazanci B., Ozturk E., SORAR M., Bavbek M.

Surgical Neurology, cilt.69, sa.4, ss.403-406, 2008 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 69 Sayı: 4
  • Basım Tarihi: 2008
  • Doi Numarası: 10.1016/j.surneu.2007.01.076
  • Dergi Adı: Surgical Neurology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.403-406
  • Anahtar Kelimeler: cerebellar tumor, medulloblastoma, medullomyoblastoma, pediatric brain tumor, rhabdomyosarcoma, teratoma, DIFFERENTIATION, TUMOR
  • Lokman Hekim Üniversitesi Adresli: Hayır

Özet

Background: Medullomyoblastoma is a quite-rarely reported biphasic histologic variant of medulloblastoma since the first published description of a tumor consisting of medulloblastic and myogenic elements. Controversy over its origin still goes on. Here, an additional case of medullomyoblastoma variant is reported, and discussed are the clinicopathologic features and pathophysiologic mechanisms of and treatment options for this neoplasm. Case report: A 7-year-old girl was admitted to our clinic with headache, vomiting, and gait disturbances. An MRI scan on admission showed a solid tumor with a 2.5-cm axial diameter located in cerebellar vermis. The tumor was removed totally. Histologic examination revealed loose mesenchymal structures of the tumor and small muscle strands and isolated cells having large eosinophilic cytoplasm with striations. The muscular strands also demonstrated striations under light microscope. Glial fibrillary acidic protein, synaptophysin, and myogenin positivity are observed. Conclusion: There are some strong evidences that the medullomyoblastoma may be a teratoma. Survival time with the tumor is very short, outcome is poor, and the tumor can spread along cerebrospinal fluid pathways. Total resection, chemotherapy, and craniospinal irradiation are mainstays of the treatment of medullomyoblastomas. © 2008 Elsevier Inc. All rights reserved.