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IgG-kappa-type plasmacytoma secreting salivary-type amylase in adult T-cell leukemia.

A IgG-kappa-type plasmacytoma secreting salivary-type amylase ectopically is reported in a patient with smouldering adult T-cell leukemia(ATL). The patient had plasmacytomas in the distal region of the right femur, the proximal region of left tibia, and the left paranasal sinus. Both his serum and urine contained high levels of amylase. The presence of IgG-kappa and S-type amylase in the plasmacytoma cells was confirmed immunocytochemically. In addition, he was also positive for the antibody against the human T-cell leukemia virus type I (HTLV-I), and had abnormal lymphocytes with convoluted nuclei (ATL cells) in the peripheral blood. The monoclonal integration of HTLV-I proviral DNA was demonstrated in the leukemic cells of the peripheral blood, but not in the plasmacytoma cells. Our case suggested that not only can HTLV-I infection play a role in the development of ATL, but may also induce a B-cell malignancy in an indirect manner, and even an ectopic amylase producing plasmacytoma.

Amylases↗

Irradiated IL-2 gene-modified plasmacytoma vaccines are more efficient than live vaccines.

The effect of irradiation on the therapeutic efficacy of IL-2 gene-modified plasmacytoma cells used as a vaccine in the immunotherapy of parental murine plasmacytoma X63-Ag8.653 was examined. Local administration of the IL-2-secreting plasmacytoma irradiated with a dose of 50 Gy inhibited i.p. plasmacytoma growth more effectively than the administration of non-irradiated, live cell vaccines. Whereas the vaccination with the live cell vaccine could substantially prolong the survival of the tumour-bearing mice but did not significantly induce tumour regressions, the irradiated vaccines could substantially increase the number of tumour-free animals. The irradiated vaccines produce higher amounts of IL-2 than the live cell vaccines both in vitro and in vivo. Depletion of CD4+ and CD8+ effector cells with monoclonal antibodies has significantly decreased the effect of the vaccination. It can be concluded that both, CD4+ and CD8+ T lymphocytes are required for effective IL-2 gene therapy of the X63-Ag8.653 plasmacytoma and that the higher effect of the irradiated vaccines is probably due to their higher IL-2 production.

Animals↗

CD117, but not lysozyme, is positive in cutaneous plasmacytoma.

CONTEXT: CD117 (c-Kit) and lysozyme are frequently expressed by myeloblasts and are sensitive markers for the diagnosis of extramedullary myeloid tumor. The diagnosis of cutaneous plasmacytoma presents a degree of difficulty, particularly with the plasmablastic variant, which can mimic hematologic as well as epithelioid malignancies. Approximately 25% of multiple myelomas express CD117 in the bone marrow by flow cytometry. Lysozyme immunoreactivity has been previously shown in 30% of poorly differentiated myelomas, while it is nonreactive in nonmalignant plasma cells. OBJECTIVE: To ascertain whether CD117 and lysozyme can aid in the diagnosis of cutaneous plasmacytomas, particularly the plasmablastic type. DESIGN: Pathology reports of 2357 patients with a diagnosis of multiple myeloma were reviewed to find 13 cutaneous plasmacytomas (8 Bartl grade II, 5 Bartl grade III). Formalin-fixed, paraffin-embedded tissue sections were stained with CD117 and lysozyme on the Dako Autostainer system.Setting.-Patients with the diagnosis of multiple myeloma who developed cutaneous plasmacytoma(s). RESULTS: The cutaneous plasmacytomas uniformly expressed CD117 in a cytoplasmic or membranous and cytoplasmic distribution with varying degrees of staining intensity unrelated to the Bartl grade of the lesion, while they were uniformly negative for lysozyme. CONCLUSIONS: CD117 is a sensitive marker for malignant plasma cells in paraffin-embedded tissue, while lysozyme does not help identify poorly differentiated malignant plasma cells. While CD117 alone does not distinguish extramedullary myeloid tumor from poorly differentiated myeloma, the combination of CD117 and lysozyme may allow their differentiation. The possibility of c-kit inhibitors being used in the treatment of other hematopoietic malignancies allows speculation regarding implications for the treatment of multiple myeloma.

Biomarkers↗

Detection of clonality with kappa and lambda immunohistochemical analysis in cutaneous plasmacytomas.

CONTEXT: Cutaneous plasmacytomas rarely occur in the setting of multiple myeloma. However, since poorly differentiated lesions may resemble other neoplasms, such as carcinoma, melanoma, and lymphoma, the diagnosis of cutaneous plasmacytoma may be difficult. OBJECTIVE: To demonstrate clonality using kappa and lambda immunohistochemical analysis in cutaneous plasmacytomas and to ascertain whether or not interpretation is hindered by background staining. DESIGN: Pathology reports of all patients with the diagnosis of multiple myeloma were reviewed. Twelve patients had cutaneous lesions diagnosed as plasmacytoma, and these lesions were analyzed for light chain restriction with kappa and lambda immunohistochemical analysis. RESULTS: In most cases (11 of 12), monoclonality was demonstrated. In the remaining case, monoclonality could not be established because most cells did not stain for either kappa or lambda. CONCLUSIONS: Light chain restriction can be demonstrated in most multiple myeloma-related cutaneous plasmacytomas, establishing the neoplastic nature of the infiltrate.

Clone Cells↗

Polyradiculoneuropathy revealing a solitary plasmacytoma of the ilium. A new case-report.

Neurological manifestations are uncommon in myeloma patients, and subacute polyradiculoneuropathy as the inaugural manifestations of solitary plasmacytoma of bone is exceedingly rare. We report the case of a 52-year-old man who was evaluated for a three-month history of flaccid tetraplegia with a gradually ascending onset and for a deterioration in general health. Electromyography findings were consistent with polyradiculoneuropathy. Laboratory tests showed a moderate amount of a monoclonal IgG-lambda antibody. Findings were normal from a radiographic bone survey and a radionuclide bone scan. Computed tomography of the pelvis disclosed a solitary osteolytic lesion in the right iliac crest, which was found upon biopsy to be a malignant plasmacytoma. Radiation therapy and chemotherapy were given. Subacute or chronic polyradiculoneuropathy as the inaugural manifestation of solitary plasmacytoma is exceedingly rare and should be distinguished from the sensorimotor polyneuropathy produced by plasma cell infiltration in some multiple myeloma patients. The polyradiculoneuropathy of solitary plasmacytoma can be likened to the neuropathies seen in some forms of multiple myeloma (sclerotic myeloma and POEMS syndrome). The pathophysiology of these neuropathies remains obscure. The case reported here suggests that patients with unexplained lasting polyradiculoneuropathy should be investigated for a plasma cell proliferation even if they have no serum monoclonal component. Because plasmacytomas are painless, imaging studies are needed for their diagnosis. The management of the neuropathy consists in treatment of the tumor.

Bone Neoplasms↗

[A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma].

We here reported a 54-year-old female patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma. She was found to have scattered tumor in 1990. Although the tumor had slowly grown for the last 10 years, she showed no clinical symptoms. Numbness and weakness of lower extremities began in June 1999, and she was referred to Kyoto University Hospital on Oct. 21 1999 for evaluation of progressive symptoms. She had skin pigmentation, edema of the lower extremities, lymphadenopathy, muscle weakness and sensory disturbance in a glove-and-stocking distribution. Serological examination showed monoclonal IgG-lambda gammopathy. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Microscopic studies on biopsied sural nerve demonstrated mild decrease of myelinated fibers. Immunohistochemically, the pulmonary tumor was defined as an IgG (lambda type) plasmacytoma. After treatment with melphalan-prednisolone therapy, the neurological symptoms improved along with decrease of serum VEGF levels as well as the size of pulmonary plasmacytoma. This is the first report of a patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma. This case suggests that growth of pulmonary plasmacytoma might have played an important role in the overproduction of VEGF and thus development of Crow-Fukase syndrome.

Antineoplastic Agents, Alkylating↗

High dose chemotherapy and allogenic peripheral blood stem cell transplantation for multiple myeloma evolving from intra-abdominal plasmacytoma.

Solitary plasmacytomas include extramedullary plasmacytomas and those found in the bone. Seventy percent of patients are male and the median age is 50-55 years, younger than that for plasma cell myeloma. Most solitary plasmacytomas of bone eventually evolve to plasma cell myeloma within 2-10 years, while the extramedullary ones do so infrequently. We present an unusual case of intra-abdominal plasmacytoma in a young woman which was misdiagnosed and treated as T cell lymphoma initially. Typical manifestations of plasma cell myeloma appeared one year later. High dose chemotherapy followed by allogeneic peripheral stem cell blood transplantation (allo-PBSCT) was given. Relapse in skin occurred one year after allo-PBSCT, and was treated with wide excision and local irradiation. The patient was well and alive without evidence of disease 4 years after wide excision of the recurrence of chest wall solitary plasmacytoma and local radiotherapy.

Abdominal Neoplasms↗

Up-date on solitary plasmacytoma and its main differences with multiple myeloma.

Solitary plasmacytoma is plasma cell neoplasm. It is a localized bone disease and for this reason it is different from multiple myeloma (systemic plasma cell neoplasm). Sometimes, solitary plasmacytoma precedes a following multiple myeloma. Clinical findings of solitary plasmacytoma are related to the univocal localization on damaged bone, while laboratory findings could be similar to multiple myeloma (i.e. M component, kidney dysfunction, blood calcium alterations, increased beta-2-microglobulin). However, during a solitary plasmacytoma, laboratory findings could not be present contemporaneously such clinical complications (i.e. kidney failure, immunological disorders with a trend toward infectious disease and/or autoimmunity, neurological disorders, haematological disorders, amyloidosis, POEMS syndrome). These raise the reason because solitary plasmacytoma has better prognosis compared to multiple myeloma.

Aged↗

Primary gastric plasmacytoma: a morphological and immunohistochemical study of five cases.

Five cases of primary gastric plasmacytoma were studied histopathologically and immunohistochemically. Plasmacytoid cells proliferated diffusely in the propria mucosa, almost preserving the structure of gastric glands. Occasionally, intranuclear inclusions, giant cells, and needle-shaped crystalline inclusions were observed. The neoplastic nature could be suspected on the basis of these histological findings. Immunohistochemically, three cases were positive for IgM and two for IgA. IgM positivity was more commonly observed in the gastric plasmacytoma than in multiple myeloma. Another immunohistochemical study demonstrated that LN-1 negativity and anti-EMA antibody positivity might be an indicator to differentiate gastric plasmacytoma from other types of gastric lymphoma. Four cases of early-stage gastric plasmacytoma have been followed for 5-12 yr. No recurrence has been observed so far. These cases suggest that gastric plasmacytoma has a relatively good prognosis.

Adult↗

Multiple primary cutaneous plasmacytomas.

BACKGROUND: Cutaneous plasmacytoma is an uncommon tumor and is mostly seen in the context of end-stage multiple myeloma. Only 20 cases of primary cutaneous plasmacytoma have been documented. A significant proportion of these patients went on to develop systemic disease with a poor prognosis. In a number of patients, however, the abnormal clone of plasma cells may arise in the skin and never progress to multiple myeloma involving the bone marrow. OBSERVATIONS: We describe a patient who developed multiple primary cutaneous plasmacytomas after a possible insect bite reaction. The monoclonality of the tumor cells is demonstrated using immunohistochemical techniques. He has been treated vigorously with chemotherapy and local radiotherapy and remains well 3 years after diagnosis. Bone marrow has been harvested for use as an autologous bone marrow transplant in the event of systemic relapse. CONCLUSIONS: Unlike previous reports of this rare entity, this case documents the monoclonality of tissue plasma cells with immunohistochemical techniques. As cutaneous plasmacytomas have been reported with an early significant mortality, unlike extramedullary plasmacytomas elsewhere, we have advocated combination chemotherapy and cryopreservation of uninvolved bone marrow for future autologous bone marrow transplantation should systemic myelomatosis develop in the patient.

Adult↗

Extramedullary plasmacytoma of the thyroid.

Plasmacytomas of the thyroid are uncommon. A 19-year-old female presented with a palpable, 3.5-cm-diameter mass in the right thyroid lobe. Fine-needle aspiration cytology (FNAC) showed that lymphoma cells were likely. The patient underwent a right lobectomy. Final haematoxylin and eosin (HE) staining of the tumour confirmed a diagnosis of thyroid plasmacytoma. Immunohistochemical staining showed that plasma cells were stained strongly with IgA antibody. To date, this is the youngest patient with thyroid plasmacytoma in the literature. Diagnosis of thyroid plasmacytoma by fine-needle aspiration cytology is typically difficult, as it was for this patient. Currently, no treatment standard exists for thyroid plasmacytoma.

Adult↗

Mucocutaneous plasmacytomas in dogs: 75 cases (1980-1987).

Mucocutaneous plasmacytomas were diagnosed in 75 dogs. Medical records and communication with owners and referring veterinarians provided information regarding location and description of the tumor, clinicopathologic data, treatment, and postoperative status of the dogs. Males and females were equally represented, and most dogs were aged adults (mean, 9.7 years). Tumors developed most commonly in the mouth, on the feet or trunk, and in the ears. Plasmacytomas were confined to the skin and mucosa in 70 dogs. Two dogs had disseminated lymphoid neoplasia, and 1 dog developed cutaneous plasmacytoma during clinical remission of lymphosarcoma. Two dogs had multiple myeloma, which was diagnosed concurrently or within a few weeks of diagnosis of the cutaneous tumor. Multiple plasmacytomas were found in 10 dogs but were not associated with clinical signs of generalized disease. Tumors did not recur after surgical excision. Clinicopathologic values, when determined, were normal in all dogs with localized plasmacytomas.

Animals↗

Effect of plasmacytoma cells on the production of granulocyte-macrophage colony-stimulating activity (GM-CSA) in the spleen of tumor-bearing mice.

Mice bearing syngeneic plasma cell tumors are characterized by elevated numbers of granulocyte-macrophage progenitors (GM-CFU) in the spleen. We investigated the role of syngeneic plasmacytomas in the hematologic response to tumor cell transplantation by assaying the production of granulocyte-macrophage colony-stimulating activity (GM-CSA) by cultured spleen cells of tumor-bearing mice and by plasmacytoma cells, alone and in coculture with spleen cells. Elevated levels of GM-CSA were detected in 7-day culture supernatants of spleen cells from Balb/c mice transplanted 2 weeks previously with syngeneic 4T00.1 plasmacytoma cells. Colony assays of spleen cells from tumor-bearing mice demonstrated the presence of both granulocyte-macrophage and tumor cell colonies. A high frequency of GM-CFU was detected in cultures which had not been supplemented with an exogenous source of GM-CSA. Significant levels of GM-CSA were detected in media conditioned by 4T00.1 plasmacytoma cells. 4T00.1-conditioned medium did not stimulate the growth of primative erythroid (BFU-E) and multilineage (CFU-GEMM) colonies, but stimulated the growth of FDC-P1 cells, thereby establishing the activity produced by 4T00.1 cells as GM-CSF. The levels of GM-CSA in media conditioned by coculturing control spleen and 4T00.1 cells were significantly higher than those detected in media conditioned by spleen cells alone. The colony frequency induced by the coculture supernatants, however, did not exceed the sum of the colonies detected in marrow cell cultures stimulated with media conditioned by control spleen and 4T00.1 cells alone. Our findings demonstrate that murine plasmacytoma cells are capable of secreting GM-CSF. They further suggest a key role for GM-CSA production by tumor cells in the hemopoietic response of mice bearing syngeneic plasma cell tumors.

Animals↗

[Sonography of 2 unusual extramedullary plasmacytomas].

The author describes two unusual extramedullary plasmacytomas. In the first patient, a 48-year old man, a mass was found sonographically in a retroperitoneal space. A discontinuity of the left iliac bone bordering on the tumour was prominent. Two years earlier, an epidural plasmacytoma had been removed. Subsequently, multiple osteolysis also developed in the left iliac bone. It was concluded that after destruction of the compact bone the plasmacytoma had infiltrated directly into the retroperitoneal space. An excretion of light chains of the kappa type was found in the urine. Six months after the tumour in the left iliac fossa had been confirmed, a second mass was seen in the abdomen. The patient died a few days later. Autopsy revealed plasmocytic infiltration of the retroperitoneal lymph nodes. In the second case, a man of 29 years of age, multiple tumours developed in the skin after removal of a meningeal plasmacytoma. Two months later, a recurrent intracranial tumour and a tumour in the left sinus maxillaris were found. Sonography of the "skin tumours" revealed intramuscular location and a cyst-like aspect; their consistency, however, was considerably increased. Histologically, plasmacytoma was present. Paraproteinaemia IgA of the kappa type was found. No lesions of the bone were seen. 11 months after the diagnosis of an intracranial tumour the patient died from local recurrence of the disease.

Adult↗

[Solitary plasmacytoma of the rib--a case report and review of Japanese literatures].

A 72-year-old woman with solitary plasmacytoma of the right fifth rib was surgically treated. She underwent radical resection of the bony chest wall including the right fifth rib, the ribs above and below the involved rib, the intercostal muscle, and the parietal pleura. Histological finding of the tumor was plasmacytoma of the rib. The type of monoclonal protein was IgG and lamda. She is doing well one year and eleven months after surgery without any signs of recurrence. Solitary plasmacytoma is rare as compared with multiple myeloma. Patients with solitary plasmacytoma originating in the rib have a feasibility of operative indication, and radical treatment is expected to be by adequate surgical resection. Seventeen cases of solitary chest wall plasmacytoma described in the Japanese literature are reviewed.

Adult↗

[A case of solitary plasmacytoma of the chest wall].

A 58-year-old man with a solitary plasmacytoma of the chest wall is reported. He was admitted because of a painless tumor on the right lateral chest wall. The chest CT scan showed a chest wall tumor surrounding the 7th and 8th ribs without rib destruction. A transcutaneous needle biopsy of the tumor with a Sure-Cut-Needle revealed a plasmacytoma. The bone marrow biopsy findings were normal. Under a diagnosis of solitary plasmacytoma, the chest wall tumor was resected. The tumor was an extramedullary plasmacytoma. Southern blot analysis of the immunoglobulin light chain gene of the tumor cells detected a rearrangement of genes in the lambda chain, while no rearrangements of immunoglobulin genes were detected in the bone marrow specimen. Seven months later another solitary plasmacytoma was found at the left radius, and was resected. Southern blot analysis of the immunoglobulin light chain gene was useful in the differential diagnosis from systemic myeloma and in determining the monoclonality of the tumor.

Blotting, Southern↗

Plasmacytoma of the middle ear and mastoid.

Extramedullary plasmacytomas are rare plasma cell tumors of the soft tissue that predominantly occur in the head and neck. They are most commonly seen in the upper respiratory passages and oral cavity. There have been only a few reports in the world literature of plasmacytomas occurring within the temporal bone. This report presents a case of plasmacytoma of the middle ear and mastoid that presented as a middle ear mass. Work-ups for systemic dissemination and multiple myeloma were negative, classifying this as a localized extramedullary plasmacytoma. This is the first report in the English literature of this malignant tumor occurring as an isolated lesion within the middle ear and mastoid. The patient was treated with surgical debulking and radiotherapy with complete resolution of the tumor. Although extremely rare, plasmacytoma should to be included in the differential for soft tissue tumors of the middle ear and mastoid.

Biopsy↗

[Plasmacytomas of the head and neck].

Neoplastic proliferation of plasma cells results in a population of immunologically homogeneous cells that can produce diffuse (multiple myeloma) or localized (extramedullary plasmacytomas and solitary plasmacytoma of bone) disease. In otorhinolaryngologic literature these neoplasms are rarely described and their nosological arrangement is often confused. The presence of a plasma cell neoplasm can be a surprise and sometimes a diagnostic challenge to the head and neck surgeon. Proper management of such lesions needs to be individualized according to their expected biologic behaviour. The recent observation of a case of maxillary sinus plasmacytoma suggested the Authors to carefully review the literature, drawing their attention mainly on the current histogenetic hypotheses and their consequences in therapeutic strategy. The correct diagnostic procedure is also explained, highlighting the difficulties due to both the protean nature of the disease and the still existing nosological confusion. The possibility of a plasma cell tumour should be never forgotten in presence of an head and neck neoplasm. Because these neoplasms may signal the presence of multiple mieloma, full evaluation is required to exclude disseminated disease. In light of recent histogenetic acquisitions it is suggested that extramedullary plasmacytomas can be classified among the so-called "mucosa-associated" lymphomas. Possible following differences in therapeutic approach and long-term follow-up are also indicated, stressing the role of surgery in managing these disorders. Surgical excision of extramedullary plasmacytomas followed by complementary radiotherapy on the site of tumour is proposed as the best treatment for these kind of neoplasms. This is in opposition with "classical" statement considering radiotherapy the only treatment for this kind of disorders.

Combined Modality Therapy↗