Calcium pyrophosphate dihydrate crystal deposition disease: a clinicopathologic analysis of 20 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features of calcium pyrophosphate dihydrate crystal deposition disease (CPPD-CDD).</p><p><b>METHODS</b>The clinical and pathologic profiles were retrospectively analysed in 20 cases of CPPD-CDD.</p><p><b>RESULTS</b>CPPD-CDD was far more common in women, most frequently involving joints, especially the knees and presenting with various arthrisis. Abnormally calcified and the articular damages were characteristic features by imageing. Histologically, multifocal indigo granular calcinosis was seen in synovium and sometimes appeared as needle-shaped or rhomboid crystals, which characterized the CPPD.</p><p><b>CONCLUSIONS</b>Though clinical symptoms of CPPD are quite variable, the definite diagnosis can be made by the abnormal calcification and joint damage radiographically and the indigo CPPD crystals histopathologically.</p>

Clinicopathological features of multiple mucosal neuroma without multiple endocrine neoplasia type IIB / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathological features of multiple mucosal neuromas without multiple endocrine neoplasia type IIB (non-MEN-IIB MMN).</p><p><b>METHODS</b>Three cases of non-MEN-IIB MMNs were analyzed for the clinical manifestations and histopathological characteristics.</p><p><b>RESULTS</b>All the 3 cases were females, age ranging from 30 to 45 years. Two cases of them involved in the laryngopharyngeal mucosa and another one located in the left margin of the tongue. Clinically, non-MEN-IIB MMNs presented with uncertain foreign body sensation, itching, vomiting and causalgia in the laryngopharyngeal areas. Mucosal papular lesions were treated by laser ablation or local surgical excision. The cases were respectively followed up for 6 to 20 months and found nothing. Histological examination showed the lesions were not encapsulated and contained irregular tortuous nerve bundles with undefined perineurium in the lamina propria. There were no nuclear palisade. Immunophenotype showed tumor cells strongly positive for vimentin, S-100, myelin specific enolase, CD56, neurofilament and neuron specific enolase, uniformly negative to CD34, CD117 and epithelial membrane antigen.</p><p><b>CONCLUSIONS</b>Non-MEN-IIB MMN is a very rare disease and the possibility of MEN-IIB should be excluded before making diagnosis. The lesions located in the mucosal tissue with polyp-like or papular appearance, so they should be differentiated from other neoplasms or non-neoplastic lesions.</p>

Clinicopathologic features of calcifying fibrous tumor with reappraisal of its histogenesis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and histogenesis of calcifying fibrous tumor (CFT).</p><p><b>METHODS</b>The clinical manifestations, histopathologic characteristics and immunophenotype were analyzed in 11 cases of CFT.</p><p><b>RESULTS</b>The male-to-female ratio was 5:6, with a mean age of 38 years and age range of 25 to 52 years. The sites of involvement included abdominopelvic cavity (n=6), soft tissue (n=4) and scrotum (n=1). Most patients presented with a gradually enlarging and painless mass. Nearly half of the cases were associated with other diseases or history of inflammation, trauma or surgical intervention. One third of the tumors represented incidental findings and showed no recurrence after resection. Imaging revealed a solitary solid soft tissue mass or multiple nodules with clear borders and associated high-density calcifications. Macroscopically, the tumors were well-circumscribed but non-encapsulated. They ranged from 0.5 to 20.0 cm in diameter and were tan-greyish, round to oval, lobulated or irregular and solid with rubbery consistency. The cut surface was whitish to tan-yellowish, gritty and showed scattered spotty yellowish discoloration corresponding to the foci of dystrophic calcifications. Histologically, CFT was composed of hyalinized fibrous tissue and thickened vessel walls with interspersed bland spindly fibroblastic cells, scattered psammomatous calcifications, dystrophic calcification and lymphoplasmacytic infiltration. In addition, focal cloak-like polymorph infiltration at the tumor periphery and entrapment of adipocytes and nerves were demonstrated in some cases. Foci resembling solitary fibrous tumor, fibromatosis, keloid or inflammatory myofibroblastic tumor were observed. Immunohistochemical study showed that the tumor cells were diffusely positive for vimentin and focally positive for CD34, factor VIII-related antigen and beta-catenin. The admixed plasma cells were notably IgG positive, with more than 50% being IgG4 positive.</p><p><b>CONCLUSIONS</b>CFT has characteristic histopathologic manifestations and shows morphologic and immunohistochemical overlaps with known IgG4-related sclerosing diseases. It is possible that CFT may represent another example of IgG4-related diseases. It often runs a benign clinical course, with rare recurrence after surgical resection. Previous inflammation and trauma may be the precipitating factors of CFT.</p>