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Plasmacytoma of the gastrointestinal tract in Korea: higher incidence than in Japan and Epstein-Barr virus association.

As a result of a systematic review of non-Hodgkin's lymphoma of the gastrointestinal tract (GI) at the Yonsei University, Seoul, Korea and the Osaka University, Osaka, Japan, we found an extramedullary plasmacytoma in 5 of a total of 78 cases (6.4%) in Korea and 1 of 136 cases (0.7%) in Osaka, which represents an approximately 9-fold difference in frequency. The presence of the Epstein-Barr virus (EBV) genome was examined in paraffin-embedded specimens from the 5 cases with GI plasmacytoma from Korea together with 17 patients collected by a nationwide study in Japan. There were no clinical findings suggestive of the presence of immunodeficiency in these Korean and Japanese patients. There were no prominent differences in the age distribution or sex ratio between the patients of the two countries. Histologically, the proliferation of mature plasma cells was almost monomorphous with occasional bi- or multinucleated forms. The immunohistochemistry revealed a restricted cytoplasmic expression of immunoglobulin light chain, kappa type in 8 cases and lambda type in 14. A polymerase chain reaction of preserved DNA from 4 Korean and 16 Japanese patients found that only 2, both Korean, were positive for EBV of subtype A. The difference in the frequency of EBV positivity between Korean and Japanese cases was significant (p < 0.05). In situ hybridization revealed positive signals in the nucleus of the tumor cells. IHC revealed that the tumor cells in these two cases were positive for latent membrane protein-1 and EB nuclear antigen-2, showing latent infection of EBV. These findings suggest a close association of EBV and Korean GI plasmacytoma, and might partly explain the remarkable number of cases in this country.

Adolescent↗

Cytopathology of extramedullary plasmacytoma of the bladder: a case report.

BACKGROUND: Plasmacytoma of the bladder is an extremely rare tumor, with all information concerning this neoplasm derived from case reports. It can be a major diagnostic pitfall on both histology and urine cytology. CASE: A 95-year-old woman presented with gross hematuria and a large bladder mass detected by ultrasound. The case was initially misdiagnosed as a high grade urothelial carcinoma. Since the urine cytology did not show the classical cytologic features of urothelial carcinoma, the histologic sections were reviewed and immunohistochemical staining performed. The final diagnosis was plasmacytoma of the bladder. Subsequently the patient underwent a skeletal survey and bone scan, which did not reveal any lesion suspicious for multiple myeloma. The patient was scheduled for radiotherapy. CONCLUSION: In this case of bladder plasmacytoma, urine cytology provided a clue to the diagnosis. Urine cytology can be a diagnostic tool to help make this diagnosis in the case of poorly differentiated bladder neoplasm, especially in a patient with a known history of multiple myeloma.

Aged, 80 and over↗

Plasmacytoma of the breast. A report of two cases diagnosed by aspiration biopsy.

BACKGROUND: Extramedullary plasmacytoma of the breast is an uncommon neoplasm, occurring either as a solitary tumor or as evidence of disseminated multiple myeloma. CASE: Two cases of plasmacytoma of the breast were diagnosed by fine needle aspiration cytology. Aspiration smears showed a dispersed population of plasmacytoid cells with eccentric nuclei, abundant cytoplasm and the characteristic paranuclear hof. CONCLUSION: The clinical, cytologic and immunophenotypic features of plasmacytoma are characteristic, and the importance of distinguishing these neoplasms from primary mammary tumors is important to avoid unnecessary surgery.

ADP-ribosyl Cyclase↗

Plasmacytoma with involvement of the urinary bladder. Report of a case diagnosed by urine cytology.

BACKGROUND: Plasmacytoma of the bladder is a rare but important entity. We report a case of plasmacytoma of the bladder that was diagnosed by urinary cytology. CASE: A 71-year-old male with a history of multiple myeloma presented in renal failure. Renal ultrasound revealed right-sided, moderate hydronephrosis with a 4 x 4-cm, posterolateral, obstructing mass. Magnetic resonance imaging demonstrated a bladder mass involving the bladder base, right lateral wall and dome with extension into the perivesical tissues on the right. The mass showed a moderate degree of enhancement following intravenous gadolinium administration. Urine cytology was performed to evaluate for bladder carcinoma or other malignancies besides plasmacytoma. The specimen was signed out as multiple myeloma of the bladder. Cystoscopy and biopsy were subsequently performed on the bladder mass. The diagnosis of plasmocytoma was made, confirming the urine cytology diagnosis. CONCLUSION: Urinary cytology can be a diagnostic tool for plasmocytoma involving the bladder.

Aged↗

Extramedullary plasmacytoma presenting as a primary mass in the breast. A case report.

BACKGROUND: Extramedullary plasmacytoma (EMP) of the breast is extremely rare, especially that not associated with multiple myeloma. CASE: A case of plasmacytoma of the breast in a 73-year-old man was diagnosed by fine needle aspiration cytology (FNAC). Aspiration smears revealed a dispersed population of plasmacytoid cells at various stages of maturation. The tumor was excised, and the histologic sections confirmed the cytologic diagnosis. CONCLUSION: FNAC diagnosis of plasmacytoma of the breast offers the opportunity to distinguish these neoplasms from primary mammary tumors and avoid unnecessary surgery.

Aged↗

Extramedullary plasmacytoma diagnosed by fine needle aspiration cytology. A report of four cases.

BACKGROUND: Fine needle aspiration cytology (FNAC) diagnosis of plasmacytoma is rare. CASES: Four cases of plasmacytoma were diagnosed by FNAC. The patients had clinically palpable lesions in more than one anatomic site. The sites of aspiration included lymph nodes, soft tissue swellings and a bony mass. The ages of the patients ranged from 42 to 80 years (mean, 62 years), and all the patients were male. All four cases showed a monomorphous population of well-differentiated myeloma cells in aspiration smears. Serum electrophoresis demonstrated an M spike in all cases, and Bence Jones protein was detected in the urine sample in one case. Radiologic and bone marrow findings also substantiated the diagnosis of multiple myeloma. CONCLUSION: FNAC diagnosis of plasmacytoma at extramedullary sites offers the opportunity for noninvasive verification of widespread involvement.

Adult↗

Plasmacytoma with aberrant expression of myeloid markers, T-cell markers, and cytokeratin.

Plasmacytomas are localized neoplastic proliferations of monoclonal plasma cells. When multifocal, the process is referred to as multiple myeloma. These lesions exhibit a pattern of antigen expression and cytomorphology that usually leads to a ready diagnosis. However, potentially troublesome variations in immunophenotype occur. We describe a case of a plasmacytoma from a patient who presented with sudden onset of pain and a lytic lesion of the left proximal humerus. Hematoxylin and eosin-stained sections showed a lymphoproliferative lesion composed of large lymphoid cells, some with plasmacytoid and immunoblastic features. The lesion also showed significant mitotic activity. Immunohistochemical staining was positive for CD45 (LCA), CD56 (N-CAM), CD43 (MT1), and cytokeratin CAM5.2. There was also clonal staining for lambda light chains. In addition, flow cytometric analysis showed positivity for myeloid markers such as CD13, CD33, CD38, and CD138. Significant negative markers include CD20 (L26), CD45RO (UCHL-1), and CD79alpha. The unusual phenotypic features of this plasmacytoma illustrate potential diagnostic pitfalls. It is important to fully study such lesions to correctly classify them, because this has significant impact on prognosis and management.

Antigens, Differentiation, T-Lymphocyte↗

Extramedullary plasmacytomas of the head and neck.

Extramedullary plasmacytomas are solitary tumors consisting of neoplastic plasma cell proliferations that occur in locations other than bone. On initial presentation they must be differentiated from multiple myeloma. This may prove to be difficult because a varying percentage may be associated at a later date with the development of multiple myeloma. Solitary extramedullary plasmacytomas represent up to 4% of nonepithelial lesions of the upper respiratory tract. From 1970 to 1990, at West Virginia University Hospitals, seven patients with solitary extramedullary plasmacytoma were identified. In four of these patients the tumor was located in the head and neck, with one tumor located in each of the following sites: temporoparietal scalp, maxillary sinus, nasopharynx, and cervical region. One patient had extensive destruction of the temporal bone, with extension intracranially to the middle cranial fossa. No patient had multiple myeloma, nor did any develop. Diagnosis was based on a combination of histology along with special immunoperoxidase staining for Ig lambda and kappa light chains. This will be demonstrated and discussed. Treatment consisted of radiotherapy in three cases, with doses ranging from 3175 to 6000 rad. One patient, treated with surgical excision, experienced a relapse at a distant site 6 years later. All patients have maintained local control and have been followed for a minimum of 1 1/2 years, with an average of 3 years. We describe our experience with these tumors and present a pertinent review of the literature. While these tumors may present as aggressive locally destructive lesions, their management should be as organ-sparing as possible because excellent control can be achieved in the majority of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Identification of immunoglobulin light chains in canine extramedullary plasmacytomas by thioflavine T and immunohistochemistry.

Extramedullary plasmacytomas were studied in 29 dogs. The site at which tumors occurred and the age and sex of the dogs were similar to those in previous reports. The skin of the digits, chin, ear, and lip represented the most common (17/29) tumor sites. Males and females were equally represented, and tumors occurred in middle-aged to old dogs (mean age, 9.0 years). A breed predilection was seen in the Cocker Spaniel (n = 7; 24%); Cocker Spaniels represented only 4% (210/4,725) of the submissions during the same period. Tumors were stained with immunohistochemical markers (lambda light chain, K light chain) and thioflavine T. Immunoreactivity was limited to either lambda or K light chains, consistent with a monoclonal plasma cell population. The majority of tumors expressed lambda light chains, consistent with previously reported canine plasma cell dyscrasias. Thioflavine T cytoplasmic fluorescence was seen in the majority (18/29) of plasmacytomas and with inflammatory plasma cells present in control specimens. Other round cell neoplasms (lymphosarcoma, histiocytoma, and mastocytoma) were negative with thioflavine T, indicating that positive staining with thioflavine T was specific for plasma cells (neoplastic and inflammatory). This study confirms by immunohistochemistry that canine extramedullary plasmacytomas disproportionately express lambda light chains and establishes thioflavine T staining as a rapid histochemical method for diagnosis of these tumors.

Animals↗

Solitary plasmacytoma of the cuboid.

Solitary plasmacytoma is an isolated malignant lesion of plasma cell origin. More than half of the reported cases of solitary plasmacytomas occur in the spine. Lesions occurring in the appendicular skeleton have a lower local recurrence rate and a favorable long-term survival rate. This is the first report of a solitary plasmacytoma involving a bone of the foot. With accurate diagnosis and effective medical treatment, long-term remission can be anticipated.

Adult↗

A case of extramedullary plasmacytoma arising from the nasal septum.

A rare case of extramedullary plasmacytoma arising from the nasal septum with localized amyloid deposition is reported. A 75-year-old woman presented with a history of post-nasal discharge and nasal obstruction for several months. Endonasal endoscopic observation revealed the presence of a mass arising from her nasal septum with an extension to the nasopharynx. No cervical lymph nodes were palpable. The biopsy specimen was diagnosed as a plasmacytoma (IgG, lambda-light chain type). Both serum myeloma-protein and urine Bence-Jones protein were negative. Bone marrow biopsy, a chest radiograph, total body skeletal survey and 67Ga- and 99mTc-scintigrams showed no other systemic lesions. These findings confirmed the diagnosis of extramedullary plasmacytoma in the nasal septum. The patient received irradiation of 40 Gy without clinically detectable reduction of tumour size. The patient eventually underwent complete resection of the tumour by KTP/532 laser under endonasal endoscopic control. Pathologically, the tumour mass was composed mainly of amyloid deposition with a marked reduction of tumour cells. This indicated the radiosensitiveness of tumour cells, which was clinically masked by the increased amyloid deposition. The clinical presentation, pathological features and surgical procedures are described with a review of the literature.

Aged↗

Plasmacytoma of the mastoid bone: solitary and systemic.

Plasma cell tumours of the mastoid are rare malignancies of haematological origin. Two patients are described with a plasma cell tumour in the mastoid bone. In one patient it concerned a solitary plasmacytoma of the bone and in the other a focus in the mastoid of a recently diagnosed multiple myeloma. Symptoms in these patients were non-specific. Computed tomography (CT) and magnetic resonance image (MRI) scanning showed a non-specific space-occupying lesion in the mastoid. The diagnosis was made on immunohistological examination, that showed diffuse sheets of monoclonal plasma cells in a clear matrix. When a plasmacytoma lesion is found multiple myeloma should always be excluded. Treatment in case of solitary plasmacytoma of the mastoid bone consists of radiotherapy. In case of localization in the mastoid of multiple myeloma the treatment consists of palliative chemotherapy.

Aged↗

Laser treatment of endobronchial extramedullary plasmacytoma.

A 68-year-old man presented with an endobronchial lesion and was subsequently found to have a plasmacytoma. After systemic involvement with multiple myeloma was ruled out, a diagnosis of extramedullary plasmacytoma was made. The diagnosis and treatment of extramedullary plasmacytoma is discussed with specific attention to the new role of laser therapy in this disease.

Aged↗

Plasmacytoma as a cause of obstructive sleep apnea.

Solitary extramedullary plasmacytomas are uncommon neoplasms. They occur most frequently in the upper aerodigestive tract and account for 4% of the nonepithelial tumors in this site. The evolution of a plasmacytoma is unsteady and symptoms at presentation have included dystonia, dysphagia, oral pain, cough, and dyspnea on exertion. Plasmacytoma of the upper aerodigestive tract has not been previously reported as a cause of obstructive sleep apnea.

Humans↗

Primary cutaneous plasmacytoma after rejection of a transplanted kidney: case report and review of the literature.

Immunosuppressed organ allograft recipients are at risk of developing lymphomas and lymphoproliferative disorders as a consequence of immunosuppressive therapy and long-term antigenic stimulation from both the graft and possible viral infections. No more than 4% of the malignant tumors detected in organ recipients are plasmacytomas. Primary cutaneous plasmacytoma is a rare type of cutaneous B-cell lymphoma arising primarily in the skin. It is derived from clonally expanded plasma cells with various degrees of maturation and atypia. We report the occurrence of a solitary cutaneous plasmacytoma in a 56-year-old male patient undergoing hemodialysis after rejection of a grafted kidney. The diagnosis was made a few months after the kidney had been surgically removed. A thorough examination showed no evidence of systemic disease. Skin lesions were successfully treated with local radiotherapy. After 2 years of follow-up there were no local or systemic recurrences.

Graft Rejection↗

Primary testicular plasmacytoma: a case report.

A 73-year-old man with a left testicular tumor is presented. Orchiectomy was performed and the tumor was diagnosed as a plasmacytoma. There has been no bone lesion or immunoglobulin abnormality during a follow-up period more than one year. For several reasons, the tumor was regarded as a primary plasmacytoma of the testicle. The literature concerning testicular plasmacytoma is reviewed briefly.

Aged↗

Extramedullary plasmacytoma producing biclonal gammopathy.

A case of extramedullary plasmacytoma presenting with biclonal gammopathy is reported. The patient was an 82-year-old man in whom monoclonal-protein (M-protein) was not noted during his first hospitalization. On his second hospitalization generalized lymph node swelling and biclonal gammopathy (IgG-kappa, IgA-kappa) were observed. Histological findings were compatible with those of extramedullary plasmacytoma. In the terminal stages, the patient became cachexic. The autopsy disclosed dissemination of the plasmacytoma. Only two other cases have been reported in the literature, and a review of these previous reports is included.

Aged↗

Solitary plasmacytoma of the skull: immunohistochemical study of angiogenic factors and syndecan-1--two case reports.

Two cases of solitary plasmacytomas of the skull are presented, and some biological aspects of the tumor examined. A 75-year-old woman presented with a tumor in the right parietal region. The serum level of immunoglobulin G (IgG) was high and a urine test for Bence Jones protein was negative. A reddish vascular mass was totally removed at surgery. The serum level of IgG was within normal limits after the operation. Postoperative radiotherapy was not performed. A 58-year-old woman presented with a tumor in the occipital region. Serum levels of Igs were within normal limits. A urine test for Bence Jones protein was positive for Ig kappa chain. Bone marrow aspiration revealed no evidence of systemic myelomatosis. The tumor mass was totally removed at surgery and she received local radiation therapy (total 50 Gy). Three months after the surgery, Bence Jones protein (kappa chain) was detected in both the urine and serum and bone scintigraphy showed a weak hot spot in the iliac bone, suggesting development to multiple myeloma. Immunohistochemical studies showed that most tumor cells were positive for vascular endothelial growth factor and syndecan-1, and some tumor cells were strongly positive for basic fibroblast growth factor in both cases. The Ki-67 staining indices were 11.3% and 15.6%. Tumor tissues were negative for p53. These results suggest that solitary plasmacytoma of the skull expresses the angiogenic factors, vascular endothelial growth factor, and basic fibroblast growth factor, in accordance with the high vascularity of the tumors, and syndecan-1 may be an immunohistochemical marker of solitary plasmacytoma of the skull.

Aged↗