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Presented by Ashley Cimino-Mathews, M.D. and prepared by Doreen Nguyen, M.D.
Case 1: A 45 year-old female with a hand mass.
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1. Question
Week 563: Case 1
A 45 year-old female with a hand massimages/D Nguyen/8-19-13/case 1/2x_nodular_450 pixels.jpg
images/D Nguyen/8-19-13/case 1/2x_450 pixels.jpg
images/D Nguyen/8-19-13/case 1/10x_foamcells_450 pixels.jpg
images/D Nguyen/8-19-13/case 1/10x_giantcells_450 pixels.jpg
images/D Nguyen/8-19-13/case 1/20x_spindlecells_450 pixels.jpgCorrect
Answer: Giant cell tumor of tendon sheath
Histology: The tumor is lobulated on low power. The lesion displays nodules of bland cells, some with abundant foamy cytoplasm and some with scant cytoplasm and ovoid nuclei with longitudinal grooves, alternating with fibrous regions. Scattered multinucleated giant cells are present. The mitotic rate is brisk. No nuclear atypia or necrosis is evident.
Discussion: consists of admixed foamy histocytes (CD68 positive) with lymphocytes (CD45 positive) and synovial cells. Mitotic figures may be abundant. They are benign neoplasms but may be locally aggressive with recurrences and erosion or invasion of bone.
Clear cell sarcomas, epithelioid sarcoma and sarcoidosis are also lesions that can have multinucleated giant cells. Clear cell sarcomas (melanoma of soft parts) can also arise in the deep tissues of the extremities (as well as in the trunk), and can be associated with a tendon. They consist of cytologically atypical and pleomorphic epithelioid to spindled cells with macronucleoli resembling melanoma, and they can also show admixed floret-like multinucleated giant cells. The label immunohistochemically like melanoma (positive for S100, HMB45 and MelanA) but carry a translocation t(12;22) in ATF1 and EWS. Epithelioid sarcomas are most common in the distal extremities (e.g., hand) of young adults and carry a poor prognosis, with high incidence of metastasis. Histologically, they consist of cytokeratin-positive epithelioid cells admixed with giant cells and poorly-formed granulomas and regions of necrosis. Sarcoidosis typically shows well-formed granuloma formation.
Reference(s):
– Kosemehmetoglu K, Folpe AL. Clear cell sarcoma of tendons and aponeuroses, and osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: a review and update. J Clin Pathol. 2010 May;63(5):416-23.
– Lucas DR. Tenosynovial giant cell tumor: case report and review. Arch Pathol Lab Med. 2012 Aug;136(8):901-6.
– Spillane AJ, Thomas JM, Fisher C. Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000 Apr;7(3):218-25.Incorrect
Answer: Giant cell tumor of tendon sheath
Histology: The tumor is lobulated on low power. The lesion displays nodules of bland cells, some with abundant foamy cytoplasm and some with scant cytoplasm and ovoid nuclei with longitudinal grooves, alternating with fibrous regions. Scattered multinucleated giant cells are present. The mitotic rate is brisk. No nuclear atypia or necrosis is evident.
Discussion: consists of admixed foamy histocytes (CD68 positive) with lymphocytes (CD45 positive) and synovial cells. Mitotic figures may be abundant. They are benign neoplasms but may be locally aggressive with recurrences and erosion or invasion of bone.
Clear cell sarcomas, epithelioid sarcoma and sarcoidosis are also lesions that can have multinucleated giant cells. Clear cell sarcomas (melanoma of soft parts) can also arise in the deep tissues of the extremities (as well as in the trunk), and can be associated with a tendon. They consist of cytologically atypical and pleomorphic epithelioid to spindled cells with macronucleoli resembling melanoma, and they can also show admixed floret-like multinucleated giant cells. The label immunohistochemically like melanoma (positive for S100, HMB45 and MelanA) but carry a translocation t(12;22) in ATF1 and EWS. Epithelioid sarcomas are most common in the distal extremities (e.g., hand) of young adults and carry a poor prognosis, with high incidence of metastasis. Histologically, they consist of cytokeratin-positive epithelioid cells admixed with giant cells and poorly-formed granulomas and regions of necrosis. Sarcoidosis typically shows well-formed granuloma formation.
Reference(s):
– Kosemehmetoglu K, Folpe AL. Clear cell sarcoma of tendons and aponeuroses, and osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: a review and update. J Clin Pathol. 2010 May;63(5):416-23.
– Lucas DR. Tenosynovial giant cell tumor: case report and review. Arch Pathol Lab Med. 2012 Aug;136(8):901-6.
– Spillane AJ, Thomas JM, Fisher C. Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000 Apr;7(3):218-25.