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[Maffucci's syndrome associated with primary brain tumor: report of a case].
No Shinkei Geka 1987; 15(9):971-5NS

Abstract

A rare case of Maffucci's syndrome associated with astrocytoma at the right frontal lobe and intracranial chondroma is reported in detail with a review of literature. A 24-year-old male who had diagnosed as multiple chondroma at the age of 14 years old was admitted to the hospital with complaint of double vision. Neurological examination revealed no abnormality except for the left abducens palsy. Multiple subcutaneous blue nodules in the left hand was carried for biopsy as cavernous hemangioma. Craniograph showed expansive radiolucent area in the parasellar region. CT scan demonstrated a round shaped low density area in the right frontal lobe, part of which was iso-density and turned out to be enhanced following contrast infusion. Roentgenogram disclosed many well-demarcated radiolucent area in phalanges of the left hand and leg, and right leg and third rib. The frontal tumor was totally extirpated and was histologically fibrillary astrocytoma. This is the ninth case of the Maffucci's syndrome associated with the primary intracranial tumor in the literature. It is emphasized that the Maffucci's syndrome is associated with a high incidence of malignancy. And the reason for tendency to develop malignant tumors is discussed.

Authors+Show Affiliations

Department of Neurosurgery, Hiraka General Hospital, Akita, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

jpn

PubMed ID

3320804

Citation

Goto, H, et al. "[Maffucci's Syndrome Associated With Primary Brain Tumor: Report of a Case]." No Shinkei Geka. Neurological Surgery, vol. 15, no. 9, 1987, pp. 971-5.
Goto H, Ito Y, Hirayama A, et al. [Maffucci's syndrome associated with primary brain tumor: report of a case]. No Shinkei Geka. 1987;15(9):971-5.
Goto, H., Ito, Y., Hirayama, A., Sakamoto, T., & Kowada, M. (1987). [Maffucci's syndrome associated with primary brain tumor: report of a case]. No Shinkei Geka. Neurological Surgery, 15(9), pp. 971-5.
Goto H, et al. [Maffucci's Syndrome Associated With Primary Brain Tumor: Report of a Case]. No Shinkei Geka. 1987;15(9):971-5. PubMed PMID: 3320804.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Maffucci's syndrome associated with primary brain tumor: report of a case]. AU - Goto,H, AU - Ito,Y, AU - Hirayama,A, AU - Sakamoto,T, AU - Kowada,M, PY - 1987/9/1/pubmed PY - 1987/9/1/medline PY - 1987/9/1/entrez SP - 971 EP - 5 JF - No shinkei geka. Neurological surgery JO - No Shinkei Geka VL - 15 IS - 9 N2 - A rare case of Maffucci's syndrome associated with astrocytoma at the right frontal lobe and intracranial chondroma is reported in detail with a review of literature. A 24-year-old male who had diagnosed as multiple chondroma at the age of 14 years old was admitted to the hospital with complaint of double vision. Neurological examination revealed no abnormality except for the left abducens palsy. Multiple subcutaneous blue nodules in the left hand was carried for biopsy as cavernous hemangioma. Craniograph showed expansive radiolucent area in the parasellar region. CT scan demonstrated a round shaped low density area in the right frontal lobe, part of which was iso-density and turned out to be enhanced following contrast infusion. Roentgenogram disclosed many well-demarcated radiolucent area in phalanges of the left hand and leg, and right leg and third rib. The frontal tumor was totally extirpated and was histologically fibrillary astrocytoma. This is the ninth case of the Maffucci's syndrome associated with the primary intracranial tumor in the literature. It is emphasized that the Maffucci's syndrome is associated with a high incidence of malignancy. And the reason for tendency to develop malignant tumors is discussed. SN - 0301-2603 UR - https://www.unboundmedicine.com/medline/citation/3320804/[Maffucci's_syndrome_associated_with_primary_brain_tumor:_report_of_a_case] L2 - https://medlineplus.gov/braintumors.html DB - PRIME DP - Unbound Medicine ER -