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1.  Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma (AESOP) Syndrome 
Adenopathy and extensive skin patch overlying a plasmacytoma is a very rare syndrome featuring a red-to-brown, violaceous skin patch along with a plasmacytoma. Only 11 case reports exist in the literature. Skin biopsies from the cutaneous patch overlying the plasmacytoma exhibit a dermal vascular hyperplasia with increased surrounding dermal mucin. Radiation therapy is used to treat and cure the plasmacytoma.
PMCID: PMC3509886  PMID: 23198009
2.  Different guises of plasmacytoma--from skin to bone. 
Journal of Clinical Pathology  1994;47(10):951-953.
A case is reported of a patient with plasmacytoma which assumed different clinicopathological features over seven years. The tumour first presented as a subcutaneous mass which was misdiagnosed as anaplastic carcinoma. Complete response to radiotherapy ensued but recurrence took the form of an intracerebral tumour. Further complete response to "palliative" corticosteroids was followed by osseous lesions three years later, when the diagnosis of plasmacytoma was confirmed. This report illustrates the highly variable clinicopathological presentations of plasmacytoma, including the rare primary cutaneous manifestation.
Images
PMCID: PMC502182  PMID: 7962610
3.  Phlegmasia caerulea dolens secondary to pelvic plasmacytoma and left femoral deep vein thrombosis☆ 
INTRODUCTION
Phlegmasia caerulea dolens (PCD) is a clinical syndrome caused by venous obstruction leading to peripheral limb ischaemia. It can ultimately lead to venous gangrene, amputation or death in 25% of cases.
PRESENTATION OF CASE
A 52-year-old man with a background of myeloma developed PCD secondary to an obstructing plasmacytoma and left femoral vein deep vein thrombosis (DVT). These were treated with combined radiotherapy and anticoagulation, with resolution of the patient's symptoms. His recovery was complicated by the development of heparin-induced thrombocytopenia (HIT) and cutaneous vasculitis.
DISCUSSION
Both plasmacytoma and DVT are recognised complications of myeloma. This is, to our knowledge, the first description of these phenomena in combination causing PCD. The combination of venous stasis from the obstructing plasmacytoma and hypercoagulability from the underlying myeloma may have contributed to clot formation. A multifaceted treatment approach was required which aimed at improving venous flow via radiotherapy to the plasmacytoma and dissolving the obstructing clot with anticoagulant therapy.
CONCLUSION
PCD has a high mortality and morbidity. Recognition is important to avoid an incorrect diagnosis of arterial occlusion and inappropriate surgical intervention. Treatment must be focused on removing the offending causes.
doi:10.1016/j.ijscr.2013.07.010
PMCID: PMC3785849  PMID: 23959409
Phlegmasia caerulea dolens; Deep vein thrombosis; Myeloma
4.  Case for diagnosis*  
Anais Brasileiros de Dermatologia  2014;89(1):173-174.
Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.
doi:10.1590/abd1806-4841.20142431
PMCID: PMC3938377  PMID: 24626671
Multiple myeloma; Plasmacytoma; Skin manifestations
5.  Subcutaneous Plasmacytoma Metastasis Precipitated by Tunneled Central Venous Catheter Insertion 
Case Reports in Oncology  2011;4(2):315-322.
Extramedullary plasmacytomas are tumors of monoclonal plasma cells arising within soft tissue that uncommonly occur in multiple myeloma patients. While sporadic development of these tumors at cutaneous trauma sites, including venous catheter access sites, has been reported, interventional radiologists seldom encounter this disease. Herein, we describe a case of metastatic subcutaneous plasmacytoma precipitated by tunneled central venous catheter insertion in a male patient undergoing stem cell therapy for treatment of multiple myeloma. In addition, we review the identification, diagnostic pitfalls, pathogenesis, and treatment of this rare entity.
doi:10.1159/000330044
PMCID: PMC3130982  PMID: 21738502
Central venous catheter; Multiple myeloma; Extramedullary plasmacytoma; Subcutaneous plasmacytoma metastasis
6.  Radiation Therapy for Secondary Cutaneous Plasmacytomas 
Case Reports in Hematology  2013;2013:739230.
We are reporting a case of a 75-year-old man with multiply recurrent IgA-lambda multiple myeloma status post multiple rounds of chemotherapy, autologous stem cell transplantation, and palliative radiation therapy for diffuse bone lesions. Approximately 15 years after original diagnosis, he developed secondary cutaneous plasmacytomas of the right arm, right chest wall, and right upper back over the course of several months. He underwent palliative involved field 3D conformal photon or en face electron therapy concurrently with various chemotherapy regimens and achieved good to complete response with palliation of pain at the irradiated sites. He died of complications related to his disease approximately 7 months after developing skin lesions. The case presented is unique for dermal involvement of myeloma treated with palliative involved field radiation.
doi:10.1155/2013/739230
PMCID: PMC3821948  PMID: 24260722
7.  Pancreatic and Gastric Plasmacytoma Presenting with Obstructive Jaundice, Diagnosed with Endoscopic Ultrasound-Guided Fine Needle Aspiration 
Case Reports in Gastroenterology  2010;4(3):410-415.
Pancreatic plasmacytoma is a rare disorder which may present with obstructive jaundice. Only eighteen cases have been reported in the English language literature. We present the first case of pancreatic plasmacytoma and gastric plasmacytoma diagnosed with endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). A 75-year-old male with a known history of multiple myeloma presented with obstructive jaundice and a pancreatic mass. A concomitant gastric mass due to gastric plasmacytoma was seen. The diagnosis was established via EUS-FNA of the pancreatic mass. Pancreatic plasmacytoma should be suspected in patients with a history of myeloma. EUS-FNA is a safe and effective modality in the diagnosis of pancreatic plasmacytoma. Radiation therapy should be the first-line of therapy in treating pancreatic plasmacytomas.
doi:10.1159/000321050
PMCID: PMC2975009  PMID: 21060710
Pancreatic plasmacytoma; Jaundice; Endoscopic ultrasound-guided fine needle aspiration
8.  Solitary plasmacytoma of the skull: Two case reports 
Oncology Letters  2012;5(2):479-482.
Solitary plasmacytoma of the skull is rare and few cases have been reported in the English literature. Plasmacytoma of the skull has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. The prognosis for solitary plasmacytoma of the skull appears to be good when it can be diagnosed on strict criteria. The clinical features of solitary plasmacytoma of the skull are complex and not easily identified, resulting in a high misdiagnosis rate. A comprehensive examination and analysis which includes radiological examination, immunoglobulin, biochemistry, test for Bence Jones protein in the urine and bone marrow is needed for correct diagnosis. If the skull lesion is isolated, with accompanying marked swelling in the area and tenderness, plasmacytoma must be considered as a possibility for the cause of solitary skull masses. Two cases of solitary plasmacytoma of the skull lesions were retrospectively reviewed, in which a comprehensive examination was used in order to predict the clinical course of solitary plasmacytoma of the skull. The patients received postoperative radiation and/or chemotherapy. Survival following surgery was longer than 2 years for patient 1, and patient 2 is alive at the 18-month follow-up.
doi:10.3892/ol.2012.1046
PMCID: PMC3573150  PMID: 23420663
plasmacytoma; skull; diagnosis; therapy
9.  Primary isolated extramedullary plasmacytoma of colon 
Background
Extramedullary plasmacytoma is an uncommon entity that most commonly involves nasopharynx or upper respiratory tract. Involvement of the gastrointestinal tract occurs in approximated 10% of cases. Isolated primary plasmacytoma of colon is rare and only 7 well documented cases have been reported in the literature.
Case presentation
We present a case of 42 year male who presented with diarrhea. Multiple colonic strictures were found on investigation. Colonoscopic biopsy was not helpful in making any specific diagnosis. Patient underwent subtotal colectomy. Isolated primary colonic plasmacytoma was found on histopathological examination.
Conclusion
Plasmacytoma is known to occur in extra osseous sites. Primary colonic plasmacytoma, however, is a rare clinical entity. Primary colonic plasmacytoma may have varying clinical presentations including multiple colonic strictures that may mimic colonic tuberculosis or inflammatory bowel disease. Although these cases are rare, treating physician as well as radiologist, and pathologist should be aware of this entity.
doi:10.1186/1477-7819-5-47
PMCID: PMC1871591  PMID: 17470287
10.  Restricted Immunoglobulin Variable Region (Ig V) Gene Expression Accompanies Secondary Rearrangements of Light Chain Ig V Genes in Mouse Plasmacytomas 
The Journal of Experimental Medicine  1999;190(10):1405-1416.
The many binding studies of monoclonal immunoglobulin (Ig) produced by plasmacytomas have found no universally common binding properties, but instead, groups of plasmacytomas with specific antigen-binding activities to haptens such as phosphorylcholine, dextrans, fructofuranans, or dinitrophenyl. Subsequently, it was found that plasmacytomas with similar binding chain specificities not only expressed the same idiotype, but rearranged the same light (VL) and heavy (VH) variable region genes to express a characteristic monoclonal antibody. In this study, we have examined by enzyme-linked immunosorbent assay five antibodies secreted by silicone-induced mouse plasmacytomas using a broader panel of antigens including actin, myosin, tubulin, single-stranded DNA, and double-stranded DNA. We have determined the Ig heavy and light chain V gene usage in these same plasmacytomas at the DNA and RNA level. Our studies reveal: (a) antibodies secreted by plasmacytomas bind to different antigens in a manner similar to that observed for natural autoantibodies; (b) the expressed Ig heavy genes are restricted in V gene usage to the VH-J558 family; and (c) secondary rearrangements occur at the light chain level with at least three plasmacytomas expressing both κ and λ light chain genes. These results suggest that plasmacytomas use a restricted population of B cells that may still be undergoing rearrangement, thereby bypassing the allelic exclusion normally associated with expression of antibody genes.
PMCID: PMC2195694  PMID: 10562316
V(D)J rearrangement; plasmacytoma; allelic exclusion; polyreactivity; V gene usage
11.  Primary plasmacytoma of the testicle: a case report 
Introduction
Extramedullary plasmacytoma is a rare plasma cell neoplasm. Plasmacytomas are most commonly found in the head and neck region, but can occur in many other locations. They rarely occur in the testis, and are commonly associated with concurrent multiple myeloma at the time of diagnosis. Isolated plasmacytoma of the testis is exceedingly rare, with few cases reported in the literature.
Case presentation
A 72-year-old Caucasian man presented with a painless testicular mass treated by orchiectomy. The mass was determined to be plasmacytoma on pathological examination. At the time of diagnosis, our patient did not have multiple myeloma, and is currently undergoing chemotherapy for treatment of his disease.
Conclusion
Isolated plasmacytoma of the testicle is a rare cause of testicular mass, and is seldom reported in the literature. Patients with this disease require careful monitoring because of their high risk of progression to multiple myeloma. The diagnosis of testicular plasmacytoma can be challenging for primary care doctors and urologic specialists. This condition should be in the differential diagnosis in elderly men.
doi:10.1186/1752-1947-5-494
PMCID: PMC3198716  PMID: 21968163
12.  Solitary Plasmacytoma of Skull: A Rare Cytological Diagnosis 
Solitary Plasmacytoma of the Skull (SPS) is very rare and only 35 cases have been reported in the English literature. It remains controversial whether a solitary plasmacytoma of the skull is essentially identical with a Solitary Plasmacytoma of Bone (SPB) or not. A solitary plasmacytoma of bone, which includes a solitary plasmacytoma of the skull, is characterized by a radiologically solitary bone lesion, neoplastic plasma cells in the biopsy specimen, fewer than 5% plasma cells in bone marrow, less than 2.0 g/dl monoclonal protein in the serum when it is present and a negative urine test for Bence Jones protein (monoclonal light chain). A solitary plasmacytoma of bone tends to disseminate or progress to multiple myeloma, even as long as 7-23 years after its presentation.
doi:10.7860/JCDR/2013/5555.3259
PMCID: PMC3782939  PMID: 24086882
Fine needle aspiration cytology; Solitary plasmacytoma; Skull
13.  Rare pancreas tumor mimicking adenocarcinoma: Extramedullary plasmacytoma 
Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.
doi:10.4253/wjge.v6.i3.99
PMCID: PMC3952166  PMID: 24634714
Plasmacytoma; Endosonography; Pancreatic mass; Multiple myeloma; Fine needle aspiration
14.  Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast 
Background
Extramedullary plasmacytomas are seldom solitary and usually progress to diffuse myelomatosis. Plasmacytomas of the breast are rare, especially when not associated multiple myeloma. Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast have not previously reported.
Case presentation
A 27-years-old woman with an untreated upper outer quadrant breast mass for 1-year was referred to our cancer hospital for surgical evaluation of increasing breast pain. Postoperatively, microscopic examination revealed an infiltrating ductal carcinoma complicated by an extramedullary plasmacytoma divided by fibrous tissue in one section. Following surgery, the patient received chemotherapy for the carcinoma and radiotherapy for the plasmacytoma.
Conclusion
In this case, careful histopathology examination was essential to make the correct diagnosis and therapy for these synchronous lesions. The patient finished chemotherapy and radiotherapy without significant adverse effects.
doi:10.1186/1477-7819-7-43
PMCID: PMC2680856  PMID: 19393076
15.  Skeletal Plasmacytoma: Progression of disease and impact of local treatment; an analysis of SEER database 
Background
Previous reports suggest an as yet unidentifiable subset of patients with plasmacytoma will progress to myeloma. The current study sought to establish the risk of developing myeloma and determine the prognostic factors affecting the progression of disease.
Methods
Patients with plasmacytoma diagnosed between 1973 and 2005 were identified in the SEER database(1164 patients). Patient demographics and clinical characteristics, treatment(s), cause of death, and survival were extracted. Kaplan-Meier, log-rank, and Cox regression were used to analyze prognostic factors.
Results
The five year survival among patients initially diagnosed with plasmacytoma that later progressed to multiple myeloma and those initially diagnosed with multiple myeloma were almost identical (25% and 23%; respectively). Five year survival for patients with plasmacytoma that did not progress to multiple myeloma was significantly better (72%). Age > 60 years was the only factor that correlated with progression of disease (p = 0.027).
Discussion
Plasmacytoma consists of two cohorts of patients with different overall survival; those patients that do not progress to systemic disease and those that develop myeloma. Age > 60 years is associated with disease progression. Identifying patients with systemic disease early in the treatment will permit aggressive and novel treatment strategies to be implemented.
doi:10.1186/1756-8722-2-41
PMCID: PMC2759950  PMID: 19778427
16.  Disease recurrence in a transplant kidney in a patient with extramedullary plasmacytoma 
Renal transplantation in patients with malignancy is controversial. Renal transplantation is generally not considered for patients with multiple myeloma (MM) because of their extremely poor prognosis. There are few reports of MM recurrence among kidney transplant recipients. We present a case of disease relapse of plasmacytoma in a transplanted kidney. We present a patient with extramedullary plasmacytoma, who responded well to chemotherapy and underwent allogenic renal transplantation. He relapsed after 4 years with progression to extramedullary plasmacytoma. Despite minimal clinical symptoms, the patient had developed myeloma cast nephropathy and acute renal failure. His renal failure settled after excision of tumor. Extramedullary plasmacytoma as a mode of relapse is highly unusual. Experience of renal transplantation in MM is limited. In the literature, the recurrence of MM is mentioned as a severe complication with a poor graft prognosis. Extramedullary plasmacytoma as a mode of relapse is highly unusual. It should not be considered as a contraindication for transplantation. Renal transplantation for patients with end stage renal disease (ESRD) due to MM is possible. But large prospective studies are needed to develop a strategy for preventing multiple myeloma recurrence.
doi:10.4103/0971-5851.73600
PMCID: PMC3009442  PMID: 21206716
Cast nephropathy; disease recurrence; plasmacytoma
17.  Cranial Plasmacytoma: A Case Series and Review of the Literature 
Plasmacytomas are malignant proliferations of plasma cells which can occur with different plasma cell dyscrasias. Solitary plasmacytomas of bone or extraosseous plasmacytomas, depending on the tissue of origin, develop in isolation without systemic manifestations of multiple myeloma. Three cases of cranial plasmacytomas are described, two in patients with multiple myeloma and one extraosseous plasmacytoma. Management options are discussed.
doi:10.1007/s12288-011-0126-7
PMCID: PMC3572258  PMID: 24426333
Cranial; Plasmacytoma; Multiple myeloma; Treatment
18.  Mediastinal plasmacytoma with multiple myeloma presenting as a diagnostic dilemma 
Cases Journal  2008;1:116.
Plasmacytoma are extramedullary accumulations of plasma cells. Most extramedullary Plasmacytomas are associated with the upper respiratory tract. The mediastinum is rarely involved. We report a rare case of mediastinal plasmacytoma with multiple myeloma. The patient is 66 year old woman presented with bone pains and mediastinal mass on CT scan and MRI. The preliminary diagnosis of occult lung cancer with mediastinal involvement, and widespread skeletal metastasis was made, although lymphoproliferative disorder along with germ cell tumor was also kept in differentials.
The diagnosis of mediastinal plasmacytoma with multiple myeloma was made after extensive investigations
doi:10.1186/1757-1626-1-116
PMCID: PMC2533298  PMID: 18717993
19.  Inhibition of plasmacytoma development in BALB/c mice by indomethacin 
The Journal of Experimental Medicine  1985;161(5):996-1012.
Indomethacin given continuously in the drinking water (20 micrograms/ml) to BALB/cAn pi mice during the latent period of pristane- induced plasmacytoma development dramatically reduced the plasmacytoma incidence from 34.9 to 2.2%. Additionally, indomethacin given from day 0 to 120 or begun as late as 60 d after a single injection of 1.0 ml pristane was also highly effective in reducing the development of plasmacytomas. Indomethacin treatment did not prevent the formation of a peritoneal inflammatory exudate or peritoneal oil granulomatous tissue, although it had a mild inhibitory effect on the intensity of the cellular inflammation, particularly after extensive treatment of greater than 100 d. Indomethacin treatment reduced the incidence of arthritis by 50%. A major effect of indomethacin treatment was a reduction in the appearance of microscopic plasmacytomas that appear in the oil granuloma before plasmacytomas can be detected by routine sampling of the peritoneal exudate. Between days 116 and 181, 16 of 20 mice given 0.5 ml pristane were found to have foci of plasmacytoma cells, while only 2 of 20 indomethacin-treated mice had foci-containing plasmacytoma cells. The number of mice with microscopic foci in the pristane-treated group greatly exceeded the expected incidence of plasmacytomas (22%) at this dose of pristane. The growth of primary plasmacytomas in transplant that is dependent on the pristane- conditioned peritoneal environment was not inhibited by indomethacin treatment. The role of indomethacin in inhibiting plasmacytoma development was not established; two possibilities are that it inhibits production of mutagenic and tissue destructive oxidants by inflammatory cells, and it inhibits prostaglandin synthesis and intracellular production of oxidant biproducts.
PMCID: PMC2187607  PMID: 3989472
20.  High resolution banding analysis of the involvement of strain BALB/c- and AKR-derived chromosomes No. 15 in plasmacytoma-specific translocations 
Plasmacytomas were induced in (BALB/c X AKR 6;15) X BALB/c backcross mice where one of the BALB/c-derived chromosomes No. 15 was replaced by the AKR(6;15)-derived Robertsonian 6;15 chromosome. (BALB/c X AKR 6;15)F2 mice that were homozygous for Rb 6;15 were mated to BALB/c mice. Plasmacytomas were induced in the progeny by intraperitoneal injection of pristane. The cytogenetic marker permitted the distinctive identification of the two chromosome 15 homologues, including the distal segment involved in the plasmacytoma-specific translocations. 7 of the 10 plasmacytomas contained the typical t(12;15) translocation. The BALB/c-derived 15 chromosome served as the donor of the translocated segment in six of them. In the seventh, the Rb 6;15 chromosome of the AKR strain was the donor. The remaining three tumors contained the same type of intrachromosomal rearrangement. It arose by the pericentric inversion of the Rb 6;15 chromosome, leading to a variant plasmacytoma-associated rcpt (6;15) translocation. Unlike the usual 6;15 variant that arises by a reciprocal exchange between two separate chromosomes, it was generated by an exchange of the distal segments of a single chromosomal element. High resolution banding analysis of the tumors showed that all translocated breakpoints on chromosomes 15, 12, and 6 were identical with the previously described breakpoints characteristic for the typical 12;15 and the variant 6;15 translocation in murine plasmacytomas. It is known that the distal segment of chromosome 15 carries the c-myc oncogene (23). The PC- associated translocations cut across the 5'-exon of c-myc in the majority of the cases (24,26). The severed oncogene is transposed to the Ig-region on the recipient chromosome. Since the BALB/c strain is highly sensitive to PC-induction, we were interested to examine the question whether its chromosome 15 is preferred as the oncogene donor in AKR X BALB/c backcross mice that carry cytogenetically distinguishable 15 chromosomes. Our results show that this is not the case, since the same segment of the AKR-derived chromosome 15 could also serve in the same capacity. This is in contrast with T cell leukemogenesis where we have previously found that the trisomization- associated duplication of chromosome 15 occurred in a highly asymmetrical fashion, depending on the donor strain of No. 15 (9-11).
PMCID: PMC2187207  PMID: 6607313
21.  Plasmacytoma of tonsil diagnosed by fine-needle aspiration cytology 
Extramedullary plasmacytoma of tonsil is rare. Even though biopsy is necessary for final diagnosis, fine-needle aspiration cytology (FNAC) can provide useful information in the management of such cases. We report a case of plasmacytoma of tonsil diagnosed by FNAC in a 43-year-old man who presented with a swelling in the right tonsillar area. FNAC smears revealed sheets of plasma cells at various stages of maturation. Subsequent histopathological and immunohistochemical studies confirmed the diagnosis of plasmacytoma. This case is reported for the rarity of site for extramedullary plasmacytoma and to highlight the usefulness of FNAC in lesions of tonsil.
doi:10.4103/0970-9371.71875
PMCID: PMC2983074  PMID: 21187876
Plasmacytoma; tonsil; FNAC
22.  Complete Endoscopic Resection of Very Early Stage Gastric Plasmacytoma 
Gut and Liver  2010;4(4):547-550.
Gastric plasmacytomas are very rare, and most are not detected until the disease has progressed to an advanced stage. However, there have been recent reports of cases of early-stage gastric plasmacytoma, in which neoplastic cells are confined to the mucosa or submucosa. Here we report a case of a very early stage gastric plasmacytoma that was confined to the lamina propria of the gastric mucosa. The lesion was successfully and completely removed by endoscopic submucosal dissection, and the surveillance endoscopy showed no recurrence during the follow-up of 40 months. This report appears to be the first documented case of complete endoscopic removal of a primary gastric plasmacytoma.
doi:10.5009/gnl.2010.4.4.547
PMCID: PMC3021614  PMID: 21253307
Plasmacytoma; Endoscopic submucosal dissection
23.  POEMS Syndrome Presentation with an Abscess within the Plasmacytoma—A Rare Case Report 
ISRN Hematology  2011;2011:173164.
POEMS Syndrome is a rare cause of demyelinating and axonal mixed neuropathy. Plasmacytomas are usually seen in POEMS syndrome and can be osseous or extramedullary. Plasmacytomas presenting as an abscess has not been noted earlier. Our patient presented with localized hyperpigmented patch on the back and later developed progressive weakness in upper and lower limbs. Initially serum and urine protein electrophoresis were normal. The patient was thought to have Chronic Inflammatory Demyelinating Polyneuropathy and was treated accordingly without any improvement. Repeat serum protein electrophoresis showed monoclonal gammopathy. MRI of the back revealed an abscess in the paravertebral soft tissues reaching up to the skin. Needle biopsy was consistent with plasmacytoma. Later, he developed a purulent fungating lesion in the lower midback. Antibiotics were started and local resection was done followed by radiation. Pathology of the resected mass showed plasmacytoma extensively involving subcutaneous soft tissue and bone. The patient improved with the treatment. Cystic plasmacytomas and abscess within the plasmacytoma has not been reported earlier. Whether abscess formation is part of the disease spectrum due to infiltration of overlying tissue or is secondary to localized immunosuppression is unknown. Local treatment of a single plasmacytoma is useful in ameliorating systemic symptoms.
doi:10.5402/2011/173164
PMCID: PMC3195345  PMID: 22084694
24.  Solitary plasmacytoma of the jaw 
Solitary plasmacytoma may be considered as a rare neoplasm of head and neck and is a different disease compared to multiple myeloma. The main difference is related to the better clinical prognosis of solitary plasmacytoma, which may be clinically silent for several years but several local recurrences may be possible once diagnosed and treated. Clinical signs and symptoms of solitary plasmacytoma are related to bone pain and possible bone fractures. Partial local impairment of local bone function may be present. Bone swelling and local involvement of mucosa and local soft tissue may be revealed. Systemic findings related to the production of monoclonal protein are usually not present and a monoclonal spike in serum electrophoresis may be absent as the monoclonal Bence–Jones protein in the urine. Other systemic dysfunctions as systemic bone marrow involvement with related anemia and absent thrombocytopenia. However, although very rare, solitary plasmacytoma of the jaw may have several clinical presentations and here we review clinical differences reported in the literature.
PMCID: PMC3262320  PMID: 22282681
solitary plasmacytoma; multiple myeloma; monoclonal; bone function; fracture
25.  EBV-Positive Plasmacytoma of the Submandibular Gland—Report of a Rare Case with Molecular Genetic Characterization 
Head and Neck Pathology  2011;5(4):389-394.
Plasmacytomas are differentiated plasma cell tumors that present as a mass lesion in osseous or extraosseous sites. Although the most common site for extramedullary plasmacytomas (EMP) is in the upper respiratory tract, plasmacytomas initially presenting as salivary gland masses are very uncommon. We describe a case of an EBV-positive plasmacytoma presenting as a 7.7 cm submandibular mass in an elderly immunocompetent man which displayed an abundance of “naked nuclei” on fine needle aspiration cytology. The tumor showed lambda light chain restriction and positive expression for CD38, MUM1 and EBER. Subsequent investigation for myeloma revealed absence of M-protein and end-organ damage, except for a lytic lesion in the radial bone. An extensive fluorescent in situ hybridization analysis showed the tumor to be negative for the t(4;14) FGFR3/IGH translocation as well as translocations involving the IGH,IGL, IGK, CCND1, BCL2, BCL6 and C-MYC genes. KRAS genetic analysis did not reveal any mutations of codons 12, 13 and 61.
doi:10.1007/s12105-011-0257-z
PMCID: PMC3210227  PMID: 21442194
Plasmocytoma; Salivary gland; Epstein barr virus; Fluorescence in situ hybridization; CMYC; KRAS

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