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MR characteristics of primary extramedullary plasmacytoma in the head and neck.

PURPOSE: To define MR characteristics of primary extramedullary plasmacytoma in the head and neck using a variety of plain and contrast-enhanced MR protocols. METHODS: Six patients with primary extramedullary plasmacytoma in the head and neck were examined clinically and with MR imaging at 1.5 T. The morphologic appearance and signal intensities of the lesions were analyzed and the results of the imaging findings were compared with histopathologic findings. RESULTS: Qualitative analysis showed that five lesions were oval and sharply demarcated without signs of infiltration; the other lesion filled the parapharyngeal space bilaterally. On t2-weighted sequences, the lesions had moderate signal intensity. On plain T1-weighted sequences, the tumors were isointense or slightly hyperintense with respect to surrounding muscles; after administration of contrast medium, four lesions notable enhancement, with distinct central inhomogeneity. CONCLUSION: Although morphologic features or evidence of contrast enhancement do not assure certain diagnosis of extramedullary plasmacytoma, these MR imaging characteristics should prompt the radiologist to include this rare lesion in the differential diagnosis.

Adolescent↗

Primary isolated extramedullary plasmacytoma of the colon: a case report.

A tenth case of extramedullary plasmacytoma (EMP) of the colon is reported. The term 'primary isolated extra-medullary plasmacytoma' is preferred to those terms previously used. A broad classification of plasmacytoma of the gastro-intestinal tract is suggested and a management policy, based on this classification, is outlined.

Humans↗

Plasmacytoma of the sigmoid colon associated with a diverticular stricture: case report and review of the literature.

Plasmacytoma is a malignant tumour composed of plasma cells. Most commonly this takes the form of a plasma cell infiltration of bone marrow-multiple myeloma. This may be seen radiologically as either discrete lytic lesions or diffuse osteoporosis. Plasma cells are seen on bone marrow biopsy, and monoclonal immunoglobulins may occur in plasma and/or urine. Less frequently, plasma cell tumours may present as a solitary myeloma of bone, which often progresses to multiple myeloma, or as a plasma cell leukemia. Primary plasma cell tumours in an extramedullary site are relatively rare. Such soft tissue plasmacytomas usually occur in the nasopharynx or conjectiva, and are seldom located in the lower gastrointestinal tract. We report a case of primary plasmacytoma associated with a diverticular stricture in the sigmoid colon, an occurrence not previously documented, and review the current literature.

Bone Marrow↗

Association of elevated mutagenesis in the spleen with genetic susceptibility to induced plasmacytoma development in mice.

Using the phage lambdaLIZ-based transgenic in vivo mutagenesis assay, mean mutant rates were determined in the spleen of mice exposed to sustained oxidative stress and were found to be increased approximately 3-fold in plasmacytoma-susceptible BALB/c and C.D2-Idh1-Pep3 mice, but not in plasmacytoma-resistant DBA/2N mice. This finding suggests a correlation between the genetic susceptibility to inflammation-induced peritoneal plasmacytomagenesis and the phenotype of increased mutagenesis in lymphoid tissues, raising the possibility that plasmacytoma resistance genes may inhibit tumor development by minimizing oxidative mutagenesis in B cells.

Animals↗

Simultaneous extramedullary plasmacytomas of the vagina and vulva: a case report and review of the literature.

An unusual case of simultaneous plasmacytomas of the vagina and vulva is presented along with a review of the literature on urogenital extramedullary plasmacytoma. The diagnosis in this case was obtained by vaginal cytological smear and later confirmed by histological examination of tumor biopsies. Of the four previously reported cases of malignant plasmacytoma of the vagina, one recurred locally and none was associated with metastases. In the case presented, local recurrence as well as multiple osseous metastases occurred.

Adult↗

Pancreatic plasmacytomas in a patient with multiple myeloma: CT and ultrasound findings.

Extramedullary plasmacytomas are an unusual manifestation of multiple myeloma. They occur most frequently in tissues or organs rich in reticulo-endothelial cells. Pancreatic plasmacytomas are rare, with only a few case reports in the literature. We present the ultrasound (US) and computed tomographic (CT) findings of two pancreatic plasmacytomas in a patient with multiple myeloma.

Aged↗

[Amyloidosis of the tongue as a possible diagnostic manifestation of plasmacytoma].

Plasmocytic non-Hodgkin's lymphoma is the most common tumor of bone and bone marrow, typically diagnosed by symptoms such as monoclonal paraproteinemia, proteinuria, anemia and hypercalcemia. In its progress, deposits of amyloids in almost all organs can be observed. However, plasmacytomas which are diagnosed by macroglossia of primarily unknown etiology are rare. This case report presents a 61-year-old woman who suffered from a persistent swelling of the tongue with painful ulcerations. A biopsy led to the diagnosis of primary systemic amyloidosis of the light-chain type, which subsequently proved to be a plasmacytoma with lambda light-chains stage II after Durie and Salmon. In the course of the disease the patient developed further deposits of amyloids in the whole gastro-enteric system. Macroglossia as a primary manifestation of plasmacytoma is rarely described in medical literature. However, reports on deposits of amyloid in the tongue in advanced stages of disease are well known.

Amyloidosis↗

Skull Base Plasmacytoma in a Patient with Light Chain Myeloma.

Skull base involvement of plasmacytoma is reported in a patient with light chain myeloma. A 39-year-old man was admitted after experiencing paresthesia on the left side of the face and left arm, intermittent diplopia, and hoarseness for 2 years. Cranial magnetic resonance imaging revealed a large midline mass extending from the middle and posterior skull base into the upper two cervical vertebrae. An extramedullary plasmacytoma associated with light chain multiple myeloma was diagnosed after biopsy of the mass and laboratory investigations. The imaging findings and clinical features associated with this rare site of extramedullary plasmacytoma involvement are reported.

Case Reports↗

Primary cutaneous plasmacytoma: treatment with intralesional tumour necrosis factor-alpha.

We report a case of primary multiple cutaneous plasmacytomas, without underlying multiple myeloma, treated with intralesional tumour necrosis factor-alpha (TNF-alpha). A marked reduction in tumour size, with no significant adverse effects, suggests that intralesional TNF-alpha is an effective treatment for non-solid malignant tumours. Extramedullary plasmacytoma, especially involving the skin as a primary site, has rarely been reported in the English literature. We describe the ninth reported case of multiple cutaneous plasmacytomas without underlying multiple myeloma. Intralesional tumour necrosis factor-alpha (TNF-alpha) was used to treat this disease and its anti-tumour effect quantitatively evaluated by comparing the tumour size and histological change before and after treatment.

Aged↗

Solitary Plasmacytoma of the Parotid Gland with Crystalline Inclusions: A Case Report.

BACKGROUND: Solitary extramedullary plasmacytoma of the parotid gland is a rare condition. Intracytoplasmic Crystalline inclusions in the tumor are even rarer and have been reported only once in the parotid gland. CASE PRESENTATION: We report here, a case of plasmacytoma of the parotid gland with intracellular crystalline inclusions in a 73-year-old woman CONCLUSION: Solitary extramedullary plasmacytoma of the parotid gland and crystalline inclusions in the tumor is of rare occurrence. The importance of such a finding with relation to tumor progression, clinical course of the disease or prognosis in general remains to be understood.

Journal Article↗

Recurrence of primary extramedullary plasmacytoma in breast both simulating primary breast carcinoma.

BACKGROUND: Extramedullary myelomas (plasmacytoma) are malignant proliferations of plasma cells in the absence of bone involvement. When they occur in the soft tissue they usually involve the upper respiratory tract and oral cavity. Extramedullary plasmacytomas of breast are uncommon. CASE PRESENTATION: A 70 year-old woman with bilateral breast masses underwent excisional biopsy for suspected primary carcinoma that subsequently proved to be a recurrence from extramedullary plasmacytoma of the mediastinum. This was diagnosed and treated 5-years prior to appearance of breast lumps. CONCLUSION: Though uncommon, considering the possibility of metastatic carcinoma and primary, secondary or recurrent lymphoproliferative disease presenting as a breast mass may avoid unnecessary surgeries.

Journal Article↗

Epstein-Barr virus-associated high-grade anaplastic plasmacytoma in a renal transplant patient.

Allograft transplant patients have an increased risk of developing polyclonal or monoclonal lymphoproliferative disorders, but high-grade anaplastic plasmacytomas are extremely rare in these patients. We present a renal transplant patient who developed multiple extramedullary high-grade anaplastic plasmacytomas in the oral cavity, the left maxillary antrum, the scalp, the thigh and the upper abdominal wall with no evidence of diffuse bone marrow infiltration. Epstein-Barr virus (EBV) mRNA transcripts were detected within the myeloma cells by in situ hybridization using EBER1-2 probes. Following discontinuation of immunosuppression applied, the patient was treated with a cyclophosphamide-prednisone regimen followed by local irradiation, and a complete remission was achieved within four weeks. We concluded that EBV-associated high-grade anaplastic plasmacytomas constitute one more type of post-transplant lymphoproliferative disorder, and that despite their characterization as highly malignant neoplasms, their clinical behavior is not always aggressive.

Herpesvirus 4, Human↗

Altered myc gene transcription and intron-induced stabilization of myc RNAs in two mouse plasmacytomas.

Accumulation of unusually high amounts of larger-than-normal c-myc mRNAs occurs in two mouse plasmacytomas, TEPC 1165 and TEPC 2027. Southern blot and DNA sequence analyses showed that both tumors have undergone translocations of immunoglobulin heavy chain loci to positions 5' of the c-myc gene promotors resulting in removal of DNA sequences encoding a negative transcriptional regulatory element. In contrast to other mouse plasmacytomas, TEPC 1165 and TEPC 2027 rearranged myc genes show increased transcription, partially explaining their abundance of myc RNA. Similar to other mouse plasmacytomas, the abundance of myc RNA in TEPC 1165 and TEPC 2027 is also influenced by increased stability of structurally atypical myc RNAs. Two myc mRNAs are found in TEPC 2027, a 2.4 kb species including all 3 myc exons and a 4.0 kb species with the 3 exons plus the first intron. The two major myc mRNAs in TEPC 1165, 3.0 and 3.9 kb species, also include all three myc exons plus portions of the first intron. S1 nuclease protection analyses show that the 5' initiation and 3' untranslated (UT) regions of the unusual TEPC 1165 RNAs are normal showing that the size differences arise solely from inclusion of first intron sequences in the large myc RNAs. DNA sequence analysis showed that the presence of first intron sequences in the large myc RNAs is due to mutations affecting the splice donor region at the 3' end of exon 1 in both tumors. SDS-PAGE analysis of immunoprecipitated TEPC 1165 and TEPC 2027 myc proteins showed them to be of normal electrophoretic mobility but no more abundant than in a pre-B cell line 18-81 that contains at least 10 fold less myc RNA. The 4.0 kb myc mRNA of TEPC 2027 is atypically stable while the 2.4 kb myc mRNA undergoes normal rapid turnover within the same cell, demonstrating that the presence of first intron sequences in the large myc RNA stabilizes it despite the presence of 3' UT and putative exon 1 destabilizing sequences. These results show that myc intron 1 sequences can counteract the effect of 3' UT region destabilizing sequences in myc RNA and suggest that the increased myc RNA stability noted in TEPC 1165 and TEPC 2027 is largely due to the presence of the intron 1 sequences.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Extramedullary plasmacytoma of the colon.

Multiple myeloma with extramedullary plasmacytoma is unusual, but associated gastrointestinal involvement is even rarer. A case with initial presentation as solitary plasmacytoma of the clavicle and subsequent lesions in the maxillary sinus, skin, and ascending colon is reviewed. The variable radiographic features of plasmacytoma of the colon are discussed, whether occurring as a solitary finding or in a patient with multiple myeloma.

Aged↗

Multiple plasmacytomas with thoracic and biliary involvement.

The course of a patient who had nonsecretory multiple myeloma was characterized by extraosseous plasmacytomas that were initially limited to pleural lesions with effusion and subcutaneous masses. Subsequently, we noted the development of obstructive jaundice caused by a mass at the head of the pancreas, which was diagnosed by abdominal ultrasound and responded to radiation therapy, and bilateral pulmonary nodules, which were visualized by fiberoptic bronchoscopy. Forceps biopsy of an endobronchial lesion showed plasmacytoma similar in histologic features to her original osseous lesions. The pulmonary nodules responded to cyclophosphamide and prednisone. During her course, she had three forms of intrathoracic myeloma: rib lesions extending into pulmonary tissue, pleural disease, and multiple endobronchial masses. The biliary and pulmonary manifestations of plasmacytomas are rarely seen. Diagnosis by noninvasive procedures and rapid response to conservative therapy were important in this patient's care.

Aged↗

Primary cutaneous plasmacytoma. Report of a case and review of the literature.

Clinical, laboratory, and pathologic findings from a case of malignant extramedullary plasmacytoma of the skin are presented. To our knowledge, this is the 11th reported case of extramedullary plasmacytoma of the skin and the second reported case with a complete follow-up and postmortem examination. In these two cases, surgical excision of the tumor mass, radiation therapy, and chemotherapy were ineffective. At necropsy, visceral metastases were found, but there was no bone marrow involvement.

Follow-Up Studies↗

Solitary extramedullary plasmacytoma of the larynx.

Plasmacytomas, indistinguishable histologically from plasma cell tumors arising in bone marrow, occur almost anywhere in the body-usually independently of multiple myeloma. Thus, they represent an isolated area of plasmacytic dyscrasia but occasionally can be the first evidence of multiple myeloma. From 1949 to 1974, six patients with solitary extramedullary plasmacytomas of the larynx were seen at the Mayo Clinic; during this period, approximately 3,100 patients with malignant laryngeal neoplasms were examined. For these six patients, the median age was 53 years (range, 32 to 63 years). The first symptom in five patients was hoarseness (mean duration, nine months). Excision, electrocoagulation, irradiation, or some combination of these constituted effective therapy. Follow-up ranged from 3 to 25 years. The choice of therapy depends on the size, shape, and location of the tumor. Extensive surgery is rarely necessary.

Adult↗

Extramedullary plasmacytoma of the thyroid associated with a serum monoclonal gammopathy.

Extramedullary plasmacytoma is a malignant plasma cell dyscrasia that is most commonly found in the head and neck. Progression to disseminated disease in the form of multiple myeloma occurs in 17% to 32% of cases. Herein, we describe a patient with extramedullary plasmacytoma of the thyroid gland in whom a serum monoclonal gammopathy resolved with surgery and irradiation of the primary. Although rare, this diagnosis should be considered in patients presenting with obstructive thyromegaly and autoimmune thyroiditis.

Aged↗