A 34-year-old left-handed British male was referred with a slightly painful "recurrent ganglion" over the dorsum of the right wrist of six months' duration. One month prior to presentation the patient noticed the appearance of another small lump over the dorsum of the right hand which was asymptomatic. The "ganglion" was curetted ten months prior to admission in an outside clinic under local anesthesia. There was no report of the original specimen being sent for pathology.The patient underwent exploration and excision of both masses under regional block with a double tourniquet (Biers block). The dorsal wrist mass was found to originate from the sheath of the extensor carpi radialis brevis and adhering to the extensor retinaculum and dorsal capsule of the wrist. The dorsal wrist synovium was intact. The small mass over the dorsum of the right hand was originating from the sheath of the extensor tendon of the ring finger. Both were excised completely. Pathologic FindingsThe material presented consisted of: (1) an irregular piece of white rubbery tissue measuring 1.0 × 0.5 × 0.3 cm, all blocked; (2) a globular mass of pale gray rubbery tissue measuring 3.0 × 2.0 × 1.2 cm with a small segment of tendon tissue. On cut surface the mass was adherent to the tendon and was composed of pale gray soft tissue with areas of hemorrhage. Microscopically, the tumor was composed of nests and fascicles of polygonal rounded or spindle-shaped cells (Figure 1). They presented vesicular nuclei with very prominent single central nucleolei. The cytoplasm was abundant and often clear. Mitoses were frequent, climaxing to 4 per 10 high-power field, with an occasional atypical figure (Figure 2). In a few scattered fields there were multinucleated giant cells. Mucin and alcian blue stains were negative. Periodic acid-Schiff (PAS) stain gave a faintly positive response. Silver impregnation demonstrated a loose network of reticulin fibers that surrounded the tumor cells individually and in small nests. Fontana and iron stains did not react with tumor cells. Inconspicuous iron deposits were noted in the interstitium. The absence of demonstrable melanin in sections stained by the Fontana technique does not rule out the diagnosis of clear cell sarcoma (CCS) of the tendon sheath because melanin is demonstrable in only 50% of the cases [1,2].This tumor is readily differentiated from synovial sarcoma because of the absence of biphasic cellular pattern and intracellular mucin formation. The presence of clear cells and the nesting of the tumor cells on silver impregnation speak against a fibrosarcoma. The differentiation of epithelioid malignant schwannoma or a spindlecell melanoma is more difficult. The absence of peripheral nerves within the vicinity and of any history of neurofibromatosis are arguments against the former. The total absence of skin involvement and the partial clear cell pattern speaks against spindle-cell melanoma [3].
The main purpose of this report is to describe nine cases of xeroderma pigmentosum with primary spontaneous corneal scarring in the absence of history of corneal ulcers or infectious keratitis. The authors studied nine patients with xeroderma pigmentosum. Each patient underwent complete ophthalmologic and dermatologic examinations. Three patients underwent excision of squamous cell carcinoma of the conjunctiva. There were five male and four female patients, with a mean age of 14 years and a range of 5 to 21 years. Seven had conjunctival squamous cell carcinoma. All patients had spontaneous diffuse corneal stromal scars without clinical evidence of keratitis and no previous history of corneal ulcer. The corneal sensation was intact. None of the patients had ulceration of the cornea. One patient had dry eye syndrome. The clinical and histopathologic changes of the cornea and conjunctiva in patients with xeroderma pigmentosum appear to be similar to the changes occurring in sun-exposed skin. This suggests that direct exposure of corneal and conjunctival tissue to ultraviolet light in patients with xeroderma pigmentosum is the primary cause of scarring in the cornea and conjunctiva, which leads to loss of vision. Ann Saudi Med 1996;16(3):296-299.KF Tabbara, WS Tabbara, Spontaneous Corneal Scarring in Xeroderma Pigmentosum. 1996; 16(3): 296-299Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder that was first described by Hebra and Kaposi in 1874. The disease, caused by defects in DNA synthesis, is characterized by skin and ocular tumors and changes that occur following exposure to sunlight. [1][2][3][4][5] The skin manifestations may vary, but generally consist of dryness of the skin with areas of hypo-and hyperpigmentation. Patients with XP manifest a high prevalence of cancer of the skin and the eye. The eyelids, conjunctiva, and cornea are often involved, with tumors of these structures often causing blindness. The tumors encountered most frequently include squamous cell and basal cell carcinoma. Malignant melanoma, keratoacanthoma, angiomas, fibromas, hemangiomas, and sebaceous carcinomas of the meibomian gland are encountered less frequently. The main objective of this report is to describe nine cases of XP with primary spontaneous scarring of the cornea in the absence of corneal ulcers or infectious keratitis. To our knowledge, primary spontaneous corneal scarring has not been previously emphasized in the literature. Report of CasesCase 1. A 20-year-old black female presented with decrease in vision in both eyes of two years' duration. The patient had been examined previously by a dermatologist, who had diagnosed xeroderma pigmentosum. There was no history of previous keratitis or corneal ulcers. On examination, the patient was found to have a visual acuity of counting fingers at 1 foot in the right eye and hand motion vision in the left eye. Tension by applanation was 15 mmHg in the right eye and 18 mmHg in the left eye. Biomicroscopy of the right eye revealed diffuse corneal stromal scarring wi...
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