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.
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.
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.
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.
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.
Phlegmasia caerulea dolens; Deep vein thrombosis; Myeloma
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.
Central venous catheter; Multiple myeloma; Extramedullary plasmacytoma; Subcutaneous plasmacytoma metastasis
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.
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.
Pancreatic plasmacytoma; Jaundice; Endoscopic ultrasound-guided fine needle aspiration
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.
plasmacytoma; skull; diagnosis; therapy
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.
V(D)J rearrangement; plasmacytoma; allelic exclusion; polyreactivity; V gene usage
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.
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.
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.
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.
Fine needle aspiration cytology; Solitary plasmacytoma; Skull
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.
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.
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.
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.
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.
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).
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.
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.
Cast nephropathy; disease recurrence; plasmacytoma
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.
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.
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.
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.
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).
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.
Plasmacytoma; tonsil; FNAC
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.
Plasmacytoma; Endoscopic submucosal dissection
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.
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.
solitary plasmacytoma; multiple myeloma; monoclonal; bone function; fracture
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.
Plasmocytoma; Salivary gland; Epstein barr virus; Fluorescence in situ hybridization; CMYC; KRAS
Plasmacytoma is a rare disease, which afflicts 2 to 3 people per every 100,000 of the general population. Solitary plasmacytoma accounts for 5% of the plasma cell neoplasm. Solitary plasmacytoma of the bone appears more vividly in the axial skeleton (25-60%), which has the red marrow and usually affects the thoracic vertebrae. We report a case of 54-year-old man who has a chest pain on the right side. After being treated for the muscle pain, his symptoms of pain were changed into weakness and allesthesia. We checked the MRI and found a mass lesion in the T5 vertebra, but there were no significant laboratory findings, in blood and urine samples. Finally, he got an operation due to the aggravation of the weakness. The result of biopsy indicated that it was a solitary plasmacytoma of the spine. After 5 months later, the weakness and allesthesia had disappeared.
Atypical plasmacytoma; Solitary spinal tumor
Plasmacytoma can present as multiple myeloma, solitary plasmacytoma of the bone (SPB), or extramedullary plasmacytoma. SPB is a rare entity that composes of malignant plasma cells and involves the bone to form only one or two lesions without evidence of disease dissemination. It accounts for only 4% of malignant plasma cell tumors. We report a case of SPB in the skull in a 59-year-old male. CT scan revealed an 8 cm ×9 cm osteolytic lesion on the scalp of skull with a well demarcated margin. A cake-like mass was revealed at CT soft tissue window. The mass was completely excised and histological examination revealed plasmacytoma. The diagnosis of SPB was established after ruling out multiple myeloma.
Plasmacytoma; multiple myeloma; solitary plasmacytoma of the bone; extramedullary plasmacytoma; skull
Extramedullary plasmacytomas are rare. Ovarian plasmacytomas, for which treatment options vary, are more unique and even more rare. We will consider the option of optimal cytoreductive surgery followed by adjuvant chemotherapy consisting of carboplatin (AUC-2) to prevent massive ascites and pleural effusion.
We report a case of ovarian plasmacytoma in a 35-year-old woman presenting with abdominal pain due to the mass. She underwent optimal cytoreductive surgery. A post-operative histopathologic diagnosis of ovarian plasmacytoma was confirmed. She was assigned stage IIC disease. She received three cycles of single agent carboplatin for rapidly refilling ascites and pleural effusion. Her response was dramatic. There is no evidence of recurrence clinically for more than 14 months. The patient is receiving follow-up care.
Multimodality treatment comprising of optimal cytoreductive surgery followed by carboplatin-based chemotherapy is a novel observation and may be an option for the treatment of these rare tumours. This options needs to be further researched.
ovarian plasmacytoma; CA-125; LDH; optimal cytoreductive surgery; carboplatin
A patient with a 3-month history of headache refractory to pain medication was admitted. The CT scan and MRI showed evidence of a posterior fossa mass. This was pathologically confirmed as an extra medullary plasmacytoma (EMP). He had a pathologic fracture of the left humerus 7 years ago while the radiologist was unaware at the time of diagnosis. A solitary bone plasmacytoma (SBP) was the cause of the pathologic fracture. This report includes the first description of MRI findings in a patient with a rare-incidence intracranial solitary extra medullary plasmacytoma (SEP) in Iran. There is a striking similarity between the features of intracranial SEP and meningiomas. Intracranial SEP, although rare, should be included in the differential diagnosis of brain tumors in areas where meningiomas commonly arise. The MRI findings and differential diagnosis of plasmacytoma are reviewed. Before this case report, only few cases have been reported in the literature. Nonetheless, this is the first report of posterior fossa EMP from Iran.
Plasmacytoma; Magnetic Resonance Imaging; Posterior Fossa