3 December 2014 - Case of the Week #335
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Case of the Week #335
Clinical History:
A 31 year old man had a vascular lesion on the right leg near the knee. Prior medical history was significant only for a nasal sinus biopsy showing severe acute and chronic inflammation.
The mass was excised. It was large and fusiform, 14 x 8 x 6 cm, and focally encapsulated, but in other areas tumor was present on the margin. On sectioning, the tumor peripherally was grey-tan-white, soft and "sarcomatous". Centrally, it had irregular calcified and ossified tissue.
Micro images:
What is your diagnosis?
Diagnosis:
Extraskeletal mesenchymal chondrosarcoma
Discussion:
Mesenchymal chondrosarcoma is a rare cartilaginous tumor with a primitive component composed of mesenchymal cells at the condensation stage. It typically affects teenagers or young adults, with no gender preference. Common sites are the diaphysis of the jaw, pelvis, femur, ribs or spine. However, it often involves extraosseous structures such as the orbit, paraspinal region, meninges or extremity soft tissue (see Soft tissue chapter).
The tumor has a biphasic pattern with a hyaline cartilage component and a small blue cell malignancy, either adjacent or with a gradual interface. The cartilage component is well differentiated and demonstrates areas of endochondral ossification. The small cell component has a "staghorn" pattern of vasculature. It is hypercellular with a high nuclear-to-cytoplasmic ratio. Both components are usually immunoreactive for Sox9 (Hum Pathol 2010;41:653, Hum Pathol 2003;34:263).
The differential diagnosis includes other small blue cell tumors, including Ewing/PNET, lymphoma and small cell osteosarcoma, all of which typically lack chondroid lobules.
The prognosis is unpredictable. Survival may be short or prolonged after metastases are identified (Arch Pathol Lab Med 2012;136:61).
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