Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There are 4 types of plasma-cell neoplasia: classic multiple myeloma (MM), extramedullary plasmacytoma without MM, solitary plasmacytoma of bone, and plasma-cell leukemia. Cutaneous involvement may be seen in all 4 types of plasma-cell neoplasia. Specific cutaneous involvement in patients with MM is very uncommon. It usually occurs in late stages of MM as a reflection of increased tumor cell burden. Extramedullary plasmacytoma (EMP) of the skin is a well-recognized, extremely rare, occurrence in MM. Extramedullary plasmacytomas of the skin can be divided into primary cutaneous plasmacytoma (PCP) and metastatic cutaneous plasmacytoma (MCP). Primary cutaneous plasmacytoma is defined as monoclonal proliferation of plasma-cells that arises primarily in the skin without evidence of systemic disease. In contrast to PCP, MCP arises from lymphatic or vascular spread of tumour or, more frequently, by direct extension from underlying bone lesions. Chest, back and abdomen are the most frequently involved areas followed by face, scalp, neck and extremities. Unusual localizations have been described related to MCP, including scrotum, eyelid and tongue. In literature, no cases of MCP presenting as a perianal giant mass have ever been reported. To our knowledge, we describe the first case of MCP located on the perianal area.
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PMID:Metastatic cutaneous plasmacytoma presenting as a perianal giant mass. 1906 99

The authors report a very rare case of a vestibular schwannoma with an infiltrate of monoclonal plasma cells. A 45-year-old woman underwent routine excision of a presumed vestibular schwannoma. Histological analysis revealed the presence of a distinct lambda light chain restricted plasma cell population within the schwannoma. The light chain restriction and polymerase chain reaction-demonstrated monoclonality of the plasma cell population suggested the co-occurrence of a plasma cell neoplasm within a schwannoma. A search for systemic disease of plasma cell origin was unremarkable. A search of the literature suggests that this is the first report of such an occurrence.
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PMID:Schwannoma with monoclonal plasma cell infiltration. 1937 3

We report a case of multiple myeloma that presented as a fluctuating sixth cranial nerve palsy in the absence of widespread signs of systemic disease. A 63-year-old woman presented with horizontal diplopia of two weeks duration that subjectively changed over time. Ocular examination showed a fluctuating sixth nerve palsy. A computed tomography (CT) scan of the brain showed multiple, enhancing, soft tissue, mass-like lesions involving the left cavernous sinus and the apex of both petrous bones. Based on bone marrow biopsy and hematologic findings, she was diagnosed with multiple myeloma. Multiple myeloma may be included in the differential diagnosis of a fluctuating sixth nerve palsy, and although ophthalmic signs are rare and generally occur late in the course of multiple myeloma, they can still be its first signs.
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PMID:Multiple myeloma manifesting as a fluctuating sixth nerve palsy. 1979 56

Involvement of the nervous system is not uncommon in patients with multiple myeloma, with polyneuropathy and myelopathy predominating. Intracranial involvement producing neurological symptoms, however, is distinctly uncommon. Massive intraparenchymal hemorrhage from a previously unrecognized intracranial plasmacytoma is exceedingly rare. The authors report the case of a 57-year-old male who presented with sudden onset of severe headache, rapid onset of right-sided weakness and deterioration in level of consciousness while at work. Two years earlier the patient had completed treatment for multiple myeloma and was considered to be in remission, with a recent bone marrow biopsy that was negative, and complete normalization of serum protein electrophoresis. Imaging studies revealed a massive intracerebral hemorrhage with the possibility of an underlying lesion, and the patient was taken for emergent hematoma evacuation and tumor resection. The patient made an excellent recovery and was treated with intracranial radiation. Even in patients with multiple myeloma without evidence of systemic disease following successful treatment, the possibility of unrecognized lesions lingers. The onset of new symptoms referable to potential intracranial pathology in this setting should prompt consideration of intracranial plasmacytoma in the differential diagnosis.
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PMID:Intracranial plasmacytoma with apoplectic presentation and spontaneous intracerebral hemorrhage: Case report and review of the literature. 2003 3

Two cases of extramedullary plasmacytoma (EMP) of the head and neck, an uncommon plasma cell neoplasm, were studied. Both patients had good outcomes and have been followed for 17 and 12 years without recurrence or systemic disease. In these two cases, the tumours measured were 4 cm and 0.3 cm, and both cases were positive for lambda light chain restriction on immunohistochemistry. Prognostic factors were considered, based on a review of EMP of the head and neck, including published reports and the authors' clinical experience. Of all plasma cell tumours, EMP has the best prognosis, especially when located in the head and neck region. Variation in the biological behaviour of EMP and the limited number of documented cases make it difficult to determine prognostic factors. Some series have found that age, tumour size, site of origin (extramedullary versus bone), grade, M-protein, light chains and radiotherapy dose all influence the outcome in EMP patients.
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PMID:Prognosis and expression of lambda light chains in solitary extramedullary plasmacytoma of the head and neck: two case reports and a literature review. 2023 40

We describe the case of a 70-year-old woman who presented with slowly progressive vision deterioration in both eyes and an increase in glare sensitivity. A slit-lamp examination showed stromal corneal deposits that were atypical for a dystrophy. Protein electrophoresis with immunofixation and bone marrow aspiration led to the diagnosis of an IgG-kappa myeloma. Chemotherapy relieved systemic disease and improved the patient's visual symptoms.
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PMID:[Unusual stromal corneal deposits]. 2037 57

Extramedullary (extraosseous) plasmacytomas are localized, plasma cell neoplasms that arise in tissues other than bone and bone marrow, and constitute about 4% of all plasma cell neoplasms. Extramedullary (extraosseous) plasmacytomas rarely affects the female lower genital tract; only 6 cases of primary cervix plasmacytomas have been reported to date. Here we describe the case of an otherwise healthy 21-year-old woman who presented for a routine examination with no symptoms. A Pap smear showed an intense inflammatory process with some atypical cells. This was confirmed by microscopic examination of a biopsy, which revealed a metaplastic process of the cervix with a massive infiltration of plasma cells with mild atypia. The atypical plasma cells showed cytoplasmic lambda immunoglobulin light chain restriction with the absence of kappa light chains, indicative of monoclonality. The patient was extensively screened for systemic disease, including bone marrow biopsy, but the disease was restricted to the cervix.
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PMID:Primary plasmacytoma of the cervix in a 21-year-old female patient. 2040 32

We present a case of bilateral otitis externa that did not respond to local treatment. Cutaneous biopsies revealed bilateral amyloid depositions secondary to multiple myeloma. Persistent, identical bilateral canal lesions may be the only manifestation of treatable systemic disease and should be biopsied, even though their bilaterality argues against malignancy.
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PMID:Amyloidosis of the external auditory canal. 2046 81

Intracranial plasma cell tumors are extremely rare and can either be solitary lesions or part of systemic multiple myeloma. We report a 42-year-old woman who presented with a posterior fossa mass and successfully underwent surgical resection, leading to the diagnosis of multiple myeloma. To our knowledge, this is the first reported case of multiple myeloma presenting as a posterior fossa mass lesion. This report highlights the importance of maintaining plasma cell tumor in the differential of intracranial mass with bony involvement. Furthermore, once the diagnosis is established, further work up is critical to evaluate for systemic disease.
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PMID:Multiple myeloma presenting as solitary mass in the posterior fossa. 2072 69

Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins. Identification of the amyloidogenic protein determines the type of amyloidosis. In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia. Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients. The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death). The authors wish to draw attention to an entity that is rarely encountered in clinical practice and that requires a high index of suspicion.
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PMID:Behind heart failure syndrome: remember AL amyloidosis. Two case-reports. 2130 62


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