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Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Kevan Salimian
30 year-old female with a dural lesion
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1. Question
Clinical history: 30 year-old female with a dural lesion
Choose the correct diagnosis:
Correct
Diagnosis: D. Plasmacytoma
Histology: The lesion consists of sheets of fairly monomorphic, discohesive cells with eccentrically located nuclei and eosinophilic cytoplasm. Some cells have “clock face” chromatin with clumps of chromatin arranged circumferentially around the periphery of the nucleus, and some nuclei have punctate nucleoli. There is no necrosis or atypia. The cells are immunoreactive for CD138 and show kappa light chain restriction by immunohistochemistry, and flow analysis confirms a monoclonal, kappa positive plasma cell population.
Discussion: The pre-operative clinical impression was of a meningioma, so the actual pathologic findings were unexpected. The features seen here, in the absence of additional lesions throughout the body, are in keeping with a solitary, extramedullary plasmacytoma. However, clinical, radiographic, and laboratory studies are needed to exclude a diagnosis of plasma cell myeloma. Indeed, subsequent clinical evaluation of this patient confirmed a diagnosis of multiple myeloma. The morphologic differential diagnosis includes metastatic carcinoma (such as lobular carcinoma or signet ring carcinoma), melanoma, lymphoma, and possibly a variant meningioma such as rhabdoid meningioma. In a patient with a known history of plasma cell myeloma, confirmatory immunostains are not necessary on a histologically classic plasmacytoma. However, in this patient with a new brain lesion, a targeted panel of immunostains can be helpful in confirming the diagnosis. This lesion is EMA negative (excluding a carcinoma and meningioma) but immunoreactive for CD138 with kappa light chain restriction, supporting the diagnosis of a monoclonal plasma cell population. A potential diagnostic pitfall is that CD138 can label carcinomas, but the constellation of histologic features is sufficient in this setting to ensure the correct diagnosis.
References
1. Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014 Nov;15(12):e538-48.
2. Palumbo A, Anderson K. Multiple Myeloma. N Engl J Med 2011; 364:1046-10Incorrect
Diagnosis: D. Plasmacytoma
Histology: The lesion consists of sheets of fairly monomorphic, discohesive cells with eccentrically located nuclei and eosinophilic cytoplasm. Some cells have “clock face” chromatin with clumps of chromatin arranged circumferentially around the periphery of the nucleus, and some nuclei have punctate nucleoli. There is no necrosis or atypia. The cells are immunoreactive for CD138 and show kappa light chain restriction by immunohistochemistry, and flow analysis confirms a monoclonal, kappa positive plasma cell population.
Discussion: The pre-operative clinical impression was of a meningioma, so the actual pathologic findings were unexpected. The features seen here, in the absence of additional lesions throughout the body, are in keeping with a solitary, extramedullary plasmacytoma. However, clinical, radiographic, and laboratory studies are needed to exclude a diagnosis of plasma cell myeloma. Indeed, subsequent clinical evaluation of this patient confirmed a diagnosis of multiple myeloma. The morphologic differential diagnosis includes metastatic carcinoma (such as lobular carcinoma or signet ring carcinoma), melanoma, lymphoma, and possibly a variant meningioma such as rhabdoid meningioma. In a patient with a known history of plasma cell myeloma, confirmatory immunostains are not necessary on a histologically classic plasmacytoma. However, in this patient with a new brain lesion, a targeted panel of immunostains can be helpful in confirming the diagnosis. This lesion is EMA negative (excluding a carcinoma and meningioma) but immunoreactive for CD138 with kappa light chain restriction, supporting the diagnosis of a monoclonal plasma cell population. A potential diagnostic pitfall is that CD138 can label carcinomas, but the constellation of histologic features is sufficient in this setting to ensure the correct diagnosis.
References
1. Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014 Nov;15(12):e538-48.
2. Palumbo A, Anderson K. Multiple Myeloma. N Engl J Med 2011; 364:1046-10
