Cystic Foreign Body Giant Cell Granuloma In Xeroderma Pigmentosum

A case of cystic foreign body giant cell granuloma is presented. The patient, nineteen years of age, known case of Xeroderma Pigmentosum, presented to the maxillofacial unite, Basrah General Hospital, Iraq, with slowly enlarging cervical cystic mass. Examination revealed cystic swelling in the mid-left side of the neck of six months duration. The mass was not tender and was immobile. Aspiration revealed straw colored fluid. The excised mass, showed cystic lesion, the base of which had an extensive soft tissue growth, histologically consisted of foreign body giant cell granulomas. There was no recurrence at a follow-up of 17 years. Introduction eroderma Pigmentosum is a rare hereditary disease transmitted as an autosomal recessive disorder, characterized by extreme sensitivity to sunlight 1 . Patients develop multiple skin and mucosal cancers in the sun light exposed areas often within the first few years of life. There is a deficiency of the enzyme required for the nucleotide excision repair of the ultra-violet light induced mutations. Most persons with Xeroderma Pigmentosum appear normal at birth but within time, an exorable destruction of skin sets in, freckles and solar keratosis usually have formed by late infancy. In minor proportion of affected persons, neurological abnormalities including mental deficiencies are an integral part of the clinical syndrome. Many others suffer serious corneal damage, the consequence being varying degrees of blindness. Angiomas, basel cell carcinomas and squamous cell carcinoma are among the skin tumors. Oral squamous cell carcinomas are frequently found as well 2,3 . The Author had an experience with one family of 13 children, 9 girls and 4 boys, of them four girls were involved with Xeroderma Pigmentosum. A total of 32 operations were conducted by the same surgeon (author) for excision of 7 Angiomas, 7 Basal cell carcinomas, and 18 squamous cell carcinomas . Follow-up for 17 years, none of the patients had recurrence at the original primary site, neither had shown metastasis to cervical lymph nodes. In one case, the patient at the age of nineteen years developed a very rare condition of cystic foreign body granuloma in the neck; this has not been described before. The author presents the first such case. Case Report A 19 year old female patient, a known case of Xeroderma Pigmentosum, presented with a non-painful cystic mass at the mid-left side of the neck. The patient has noticed a small soft swelling six months earlier, since then it slowly increased in size. The patient has nine X Cystic Foreign Body Giant Cell Granuloma In Xeroderma Pigmentosum Ali Abbas Alshawi Bas J Surg, December, 20, 2014 110 sisters and three brothers. Three sisters are involved with the disease. The father and mother are close relatives. None of them or their parents have the disease neither they recall the occurrence of the disease in the family. The patient has nine operations conducted by the same surgeon (author), the first when she was two years old, and the last when she was thirteen years. The operations were to excise six squamous cell carcinomas involving the anterior part of the tongue, the vermilion border of the lower lip, commeasure of the mouth, skin of the face and the scalp; these were presented as erosive or, ulcerative lesions and exophytic lobulated partly infiltrative masses (fig.1). Other tumors were basel cell carcinoma involving the infraorbital region, skin of the nose; others were angiomas involving the medial canthal area and lower eyelid. The patient did not recall any trauma to the face or neck neither she had any previous surgery to the neck.