Inter-carpal soft tissue entrapment. A possible explanation for chronic dorsal wrist pain.

This retrospective study evaluates the surgical treatment of a group of patients with unknown chronic dorsal wrist pain. The cause of their symptoms was interpreted as a painful entrapment of fibrous tissue in the radio-carpal and inter-carpal joints during specific movements. Between 1997 and 2001, 30 patients were treated by surgical excision of this tissue and 26 patients were traced for follow-up. Twenty-three patients were symptom free, or experienced major benefit from surgery. Wrist function measurements using the VAS scale showed improvement in 24 patients. Microscopic examination of the removed specimen shows fibrous tissue with non-specific changes. Inter-carpal soft tissue entrapment can explain the typical clinical findings in some patients with unknown chronic dorsal wrist pain. After careful selection, surgical excision of all entrapped tissue in the radio-carpal and mid-carpal joint may give relief of pain and improvement of wrist function.

Microsatellite instability and expression of hMLH-1 and hMSH-2 in sebaceous gland carcinomas as markers for Muir-Torre syndrome.

Sebaceous gland carcinomas (SGCs) are rare malignant skin tumors occurring sporadically or as a phenotypic feature of the Muir-Torre syndrome (MTS). A subset of patients with MTS have a variant of the hereditary nonpolyposis colorectal cancer syndrome caused by mutations in mismatch repair (MMR) genes, which lead to microsatellite instability (MSI). We evaluated the value of MSI and loss of expression of the MMR genes, hMLH-1 and hMSH-2, as a marker to identify and distinguish MTS from sporadic SGC. Using a nationwide pathology report database system, we identified patients with the MTS phenotype. SGCs from 10 MTS patients and the colorectal carcinomas from 3 additional MTS patients were collected. In addition, SGCs from eight patients without a history of visceral neoplasm were collected. MSI was detected in 9 of 13 MTS-associated tumors (69%) versus 0 of 8 sporadic SGCs (P = 0.002). Except for the age of onset of colorectal carcinoma [58 years in the MSI-positive group versus 69.8 years in the MSI-negative group (P = 0.17)], no differences were seen between the MSI-negative and the MSI-positive MTS patients. Loss of expression of hMLH-1 (n = 4) or hMSH-2 (n = 4) was found in MSI-positive patients only. MSI and loss of expression of MMR genes can be used as markers for MTS in patients with SGC. Consequently, MSI and loss of MMR gene expression in a patient presenting with SGC as the initial malignancy have important consequences for the patient and family. There are at least two variants of MTS with different molecular genetic mechanisms because 31% of the patients with the MTS phenotype had no MSI.