Xeroderma pigmentosum: spinal cord astrocytoma with 9-year survival after radiation and isotretinoin therapy.

Abstract

BACKGROUND:

Patients with xeroderma pigmentosum (XP) frequently develop sunlight-induced skin cancer. Infrequently, internal neoplasms may also occur. A 21-year-old patient with XP, who had many skin cancers, developed a rare internal tumour - a grade II diffuse fibrillary spinal cord astrocytoma - during a break in a therapeutic trial of isotretinoin for skin cancer prevention. Treatment of neoplasms in XP patients presents special difficulties because of their defect in DNA repair.

OBJECTIVE:

The study objective was to raise awareness of the cancer surveillance process in XP patients and the concerns involved in choice of therapy.

METHODS:

Since the spinal cord tumour was inoperable, the patient was treated with x-radiation, continued on isotretinoin treatment and was followed closely for tumour response.

RESULTS:

Despite sensitivity to sunlight, the patient had a normal acute response to the x-ray treatment without excessive skin reaction. Serial examinations by magnetic resonance imaging (MRI) starting 8 months after x-ray treatment was initiated, showed a marked gadolinium enhancement followed by regression. This clearing was first seen at 2 years after biopsy and persisted to at least 9 years after treatment.

CONCLUSION:

In contrast to the exaggerated sensitivity to UV radiation, XP patients may tolerate therapeutic doses of x-radiation. Isotretinoin treatment may have contributed to the good response of this spinal cord astrocytoma.

• Authors: J J DiGiovanna, N Patronas, D Katz, D Abangan, K H Kraemer
• Journal: Journal of cutaneous medicine and surgery (J Cutan Med Surg) Vol. 2 Issue 3 Pg. 153-8 (Jan 1998) ISSN: 1203-4754 [Print] United States
• PMID: 9479081 (Publication Type: Case Reports, Journal Article)

Chemical References

• Keratolytic Agents
• Isotretinoin

Topics

• Adult
• Astrocytoma (complications, radiotherapy)
• Carcinoma, Basal Cell (etiology, prevention & control)
• Carcinoma, Squamous Cell (etiology, prevention & control)
• Humans
• Isotretinoin (therapeutic use)
• Keratolytic Agents (therapeutic use)
• Male
• Skin Neoplasms (etiology, prevention & control)
• Spinal Cord Neoplasms (complications, radiotherapy)
• Xeroderma Pigmentosum (complications, drug therapy)