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11 June 2009 - Case #149

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This case was contributed by Dr. Asmaa Gaber Abdou and Dr. Nancy Youssef Asaad, Menoufiya University, Egypt.



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Case #149

Clinical history:
A 50 year old woman presented with a thigh mass, which was excised.

Microscopic images:




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Diagnosis: Parachordoma

Immunostains:

Cytokeratin

EMA

S100

Vimentin



Discussion:
The mass showed cords of cells floating in myxoid or mucoid material, admixed with occasional physaliferous (multivacuolated) cells. The original diagnosis was liposarcoma, but after review immunostains were performed showing tumor cells that were immunoreactive for cytokeratin, EMA, S100 and vimentin, which confirmed the revised diagnosis of parachordoma.

Parachordoma is a rare peripheral tumor which usually affects deep soft tissues adjacent to bones and tendons. It is characterized by myxoid or hyaline material containing large, rounded eosinophilic cells. The tumor almost always contains a small number of physaliferous cells (Am J Surg Pathol 1999;23:1059). It stains positively for S100, vimentin, CK8 / CK18 and EMA and negative for CK1 / CK10. Type 4 collagen is also present around nests of cells.

The differential diagnosis includes:
  • Liposarcoma: these tumors have variable lipoblasts, which are also multivacuolated but have atypical nuclei. They also have a prominent component indicated by the subtype (well differentiated, dedifferentiated, myxoid / round cell, pleomorphic or sclerosing) and are keratin negative.
  • Extraskeletal myxoid chondrosarcoma: these tumors have a neoplastic cartilaginous component with long chains of cells, are keratin negative and have a characteristic t(9;22).
  • Chordoma (primary or metastatic): primary chordomas occur on the craniospinal axis, and have prominent physaliferous cells. Some authors consider the immunostaining pattern to be similar to parachordoma but some believe that CK1 / CK10 staining is helpful to differentiate these entities (Pathol Int 2004;54:364). Metastatic chordoma must be ruled out clinically.

Parachordomas typically have benign behavior. Wide resection is usually adequate treatment and recurrences are rare (Adv Anat Pathol 2000;7:141). However, to date, 5 cases of metastatic tumor have been reported (J Cutan Pathol 2009;36:270).


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