Squamous-cell cancer of the skin in pigmented xeroderma

Abstract

Squamous-cell carcinoma of the skin in patients with pigmented xeroderma is characterized by early onset, aggressive course, and rapid metastatic growth. The development of malignant tumors can be prevented by early diagnosis and stringent limitation ofphotoexposure of the skin. A clinical case is presented: an 8-year-old child with multiple tumors on the face with destruction of the sublying bone and cartilage tissues and metastases in the regional lymph nodes. The first tumor emerged at the age of 4 years, dissemination started at 5. Surgery, close-focus X-ray therapy, roaccutan, and cyclophosphamide were ineffective.

Keywords

pigmented xeroderma / squamous-cell carcinoma of the skin / therapy / prevention

References

[1] Литвиненко В.М. Рак кожи - причины возникновения, классификация, способы лечения и профилактики. Наука, техника и образование. 2014; 1: 115-9.

[2] Mareddy S., Reddy J., Babu S., Balan P. Xeroderma pigmentosum: man deprived of his right to light. Sci. World J. 2013; 2013: 534752. doi: 10.1155/2013/534752. eCollection 2013.

[3] Дворников А.С., Рыбко В.А., Ковалева О.В., Книжник А.В., Архипова К.А., Каинов Я.А. и др. К вопросу о патогенезе и ранней диагностике паранеопластических процессов в дерматологии. Вестник РГМУ. 2007; 6: 50-4.

[4] Kraemer K.H., Lee M.M., Andrews A.D., Lambert W.C. The role of sunlight and DNA repair in melanoma and nonmelanoma skin cancer: the xeroderma pigmentosum paradigm. Arch. Dermatol. 1994; 130(8): 1018-21.

[5] Grampurohit V.U., Dinesh U.S., Rao R. Multiple cutaneous malignancies in a patient of xeroderma pigmentosum. J. Canceer Res. Ther. 2011; 7(2): 205-7. doi: 10.4103/0973-1482.82932.

[6] Mohanty P., Mohanty L., Devi B.P. Multiple cutaneous malignancies in xeroderma pigmentosum. Indian J. Dermatol. Venereol. Leprol. 2001; 67(2): 96-7.

[7] Masutani C., Kusumoto R., Yamada A., Dohmae N., Yokoi M., Yuasa M., et al. The XPV (xeroderma pigmentosum variant) gene encodes human DNA polymerase eta. Nature. 1999; 399(6737): 700-4.

[8] Bradford P.T., Goldstein A.M., Tamura D., Khan S.G., Ueda T., Boyle J., et al. Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair. J. Med. Genet. 2011; 48(3): 168-76. doi: 10.1136/jmg.2010.083022.

[9] Ramkumar H.L., Brooks B.P., Cao X., Tamura D., Digiovanna J.J., Kraemer K.H., Chan C.C. Ophthalmic manifestations and histopathology of xeroderma pigmentosum: two clinicopathological cases and a review of the literature. Surv. Ophthalmol. 2011; 56(4): 348-61. doi: 10.1016/j.survophthal.2011.03.001.

[10] Kraemer K.H., Lee M.M., Scotto J. Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases. Arch. Dermatol. 1987; 123(2): 241-50.

[11] Ганцев Ш.Х., Юсупов А.С. Плоскоклеточный рак кожи. Практическая онкология. 2012; 2: 80-91.

[12] DiGiovanna J.J. Retinoid chemoprevention in patients at high risk for skin cancer. Med. Pediatr. Oncol. 2001; 36(5): 564-7.

[13] Lehmann A.R., McGibbon D., Stefanini M. Xeroderma pigmentosum. Orphanet J. Rare Dis. 2011; 6: 70. doi: 10.1186/1750-1172-6-70.

[14] Lehmann J., Schubert S., Emmert S. Xeroderma pigmentosum: diagnostic procedures, interdisciplinary patient care, and novel therapeutic approaches. J. Dtsch. Dermatol. Ges. 2014; 12(10): 867-72. doi: 10.1111/ddg.12419.

RIGHTS & PERMISSIONS

Eco-Vector