Calcified Chondroid Mesenchymal Neoplasm

Abstract

Calcified chondroid mesenchymal neoplasm is a term proposed for tumors with a spectrum of morphologic features, including cartilage/chondroid matrix formation, that frequently harbor FN1 gene fusions. We report a series of 33 cases of putative calcified chondroid mesenchymal neoplasms, mostly referred for expert consultation out of concern for malignancy. Patients included 17 males and 16 females, with a mean age of 51.3 years. Anatomic locations include the hands and fingers, feet and toes, head and neck, and temporomandibular joint; 1 patient presented with multifocal disease. Radiologic review showed soft tissue masses with variable internal calcification, which occasionally scalloped bone but in all cases appeared indolent/benign. Tumors had a mean gross size of 2.1 cm and a homogenous rubbery to fibrous/gritty tan-white cut surface. Histology demonstrated multinodular architecture with a prominent chondroid matrix and increased cellularity towards the periphery of the nodules. The tumor cells were polygonal with eccentric nuclei and bland cytologic features and showed a variable amount of increased spindled / fibroblastic forms in the perinodular septa. The majority of cases had notable grungy and/or lacy calcifications. A subset of cases demonstrated at least focal areas of increased cellularity and osteoclast-like giant cells. Herein, we confirm the distinct morphologic and clinicopathologic features associated with this entity with the largest series to date, with a focus on practical diagnostic separation from similar chondroid neoplasms. Awareness of these features is critical in avoiding pitfalls, including a malignant diagnosis of chondrosarcoma.

Superficial acral calcified chondroid mesenchymal neoplasm harboring an FN1::FGFR2 fusion and review of the literature

“…Calcified chondroid mesenchymal neoplasm represents a distinct and recently recognized entity commonly characterized by FN1 gene fusions involving multiple potential tyrosine kinase partners. [4][5][6][7][8][9] These tumors are often found in the temporomandibular joint and the extremities, consisting of polygonal cells surrounded by a cartilaginous or chondroid matrix and frequently exhibiting calcifications with grungy or lace-like patterns. [4][5][6][7][8][9] Initially, 12 cases of a unique tumor characterized by recurrent FN1 rearrangements were reported, termed FN1-rearranged calcified chondroid mesenchymal neoplasm.…”

“…[4][5][6][7][8][9] These tumors are often found in the temporomandibular joint and the extremities, consisting of polygonal cells surrounded by a cartilaginous or chondroid matrix and frequently exhibiting calcifications with grungy or lace-like patterns. [4][5][6][7][8][9] Initially, 12 cases of a unique tumor characterized by recurrent FN1 rearrangements were reported, termed FN1-rearranged calcified chondroid mesenchymal neoplasm. 4 The tumors examined in their study were primarily located in the temporomandibular joint and the extremities, consisting of polygonal cells surrounded by a cartilaginous or chondroid matrix.…”

“…6 The largest study to date reported 33 cases, confirming the presence of a combination of reproducible morphological and clinicopathological features that overlapped with those previously described. 4,5 A subset of cases in both series, with unequivocal histopathological features, lacked FN1 rearrangements by fluorescence in situ hybridization (FISH) or NGS. 4,5 Overall, this tumor predominantly affects the digits of middle-aged adults and behaves in a benign fashion.…”

“…4,5 A subset of cases in both series, with unequivocal histopathological features, lacked FN1 rearrangements by fluorescence in situ hybridization (FISH) or NGS. 4,5 Overall, this tumor predominantly affects the digits of middle-aged adults and behaves in a benign fashion. In the largest series involving 15 of 33 patients with available follow-up, all remained disease-free except for one patient who experienced a local recurrence in the wrist following incomplete excision.…”

Effect of skinfold thickness on arm venous access port in cancer patients

“…The local manifestations were mainly mass formed by malignant proliferation of tumor cells, pain, ulcer, hemorrhage and obstruction caused by compression and stimulation of peripheral nerves due to tumor growth. Systemic symptoms are mostly dominated by symptoms such as weight loss, loss of appetite, cachexia, and anemia in patients 28 . Therefore, cancer patients should be detected and treated early to avoid cancer cell metastasis through direct spread, lymphatic, hematogenous and implantation.…”