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[Cutaneous leiomyosarcoma and osteomedullary plasmocytoma with the demonstration of IgA-kappa paraprotein in serum and skin tumor].

A dermal leiomyosarcoma associated with a still asymptomatic osteomedullary plasmocytoma with IgA paraproteinemia develop-d rapidly in a 78-year-old man. The same paraprotein type IgA kappa was identified, with distinct decrease in concentration, in serum, sarcoma-tissue, and in the tumour-surrounding skin area by m-ans of immunoelectrophoresis, radial immunodiffusion, and Ouchterlony test. The immunochemical identity of the paraprotein, the course of the disease as followed clinically and immunochemically, and several histological criteria are in favour of monoclonal origin of the paraprotein from malignant plasmocytoma cells. The accumulation of paraprotein in the sarcoma tissue is primarily explained by the marked blood congestion of the tumour. To our knowledge no report exists in the medical literature of an association of dermal leiomyosarcoma and early paraproteinemic plasmocytoma. This coincidence may be only of chance but we rather suggest an immunopathological relation of both tumors due to a partial immune insufficiency caused by the preceding plasmocytoma, resulting in a diminished immunological "surveillance" of the organism and favouring the development of a new malignant cell population (i.e. leiomyosarcoma).

Aged↗

Primary plasmocytomas of the cranial vault.

Primary intracranial plasmocytomas are rare in neurosurgical practice. The accessible literature indicates that they fall into two groups: 1. Extramedullary plasmocytoma originating from the dura and 2. Plasmocytoma originating from the cranial vault. Four patients with primary lesions of the cranial vault were surgically treated and common characteristic clinical and radiological features discussed. Although the immediate outcome of treatment was excellent in all cases, the dissemination of disease occurred in two patients within the period of one year. It is therefore essential that the patients with so called solitary cranial plasmacytoma were rescreened for systemic myelomatosis at regular intervals.

Cerebral Angiography↗

Extramedullary non-gastrointestinal plasmocytoma. An immunohistochemical study of sixteen cases.

Sixteen cases of extramedullary non-gastrointestinal plasmocytoma were studied for intracytoplasmic immunoglobulins by means of the immunoperoxidase-complex technique. Of the sixteen cases IgG class were observed in 4, IgM in one and IgA in 9 cases. Kappa light chains were found in 9 cases and lambda light chains in 6 cases. In one case a bitypic pattern of k- and lambda-positivity was encountered. In 2 cases only kappa light and no heavy chains were detected. The predominance of the IgA class in extramedullary, non-gastrointestinal plasmocytomas is in contrast to the prevalence of IgG type in multiple myeloma. The better possible behaviour of IgA-plasmocytomas is discussed further.

Eyelid Neoplasms↗

Transverse syndrome as the dominant feature of solitary cervical plasmocytoma with dissemination.

STUDY DESIGN: A case report of transverse syndrome secondary to compression from an intravertebral plasmocytoma. OBJECTIVES: To report a rare case of cervical cord compression. SETTING: Vienna, Austria. CASE REPORT: A 47-year-old man presented with pain over the left scapula and subsequent development of pain and sensory disturbance. Investigations showed that the entire C7 vertebra was affected by a plasmocytoma. Decompression was carried out but no stabilisation of the spinal column was performed. Seven days after surgery the patient became paraplegic. Chemotherapy produced no improvement. CONCLUSION: Transverse syndrome may be a dominant feature of plasmocytoma and may resolve after immediate decompression but may recur if the spinal column is not stabilised.

Cervical Vertebrae↗

[Extramedullary manifestation of plasmocytoma in ENT].

Plasmocytomas are non-Hodgkin's tumors which occur with an incidence of 4/100,000. Malignant plasma cells in bone marrow produce monoclonal antibodies which are typically found in urine and blood samples. In rare instances plasmocytoma occur exclusively in extramedullary locations preferring head and neck region. We report about two patients with extramedullary plasmocytoma, one of them with an extremely rare triple manifestation in the upper airway.

Adult↗

Plasmocytoma of the urethra.

CASE HISTORY AND FINDINGS: A 35-year-old male patient presented with a 1-year history of hesitancy in voiding. An urethrogram revealed urethral stricture, therefore an urethrotomy with biopsies was performed. Histologic results suggested the diagnosis of extramedullary mature plasmocytoma. TREATMENT AND OUTCOME: After external beam radiotherapy with a total dose of 45 Gy the patient is disease-free without any therapy-related late effects after a follow-up of 36 months. CONCLUSION: Primary localization of extramedullar plasmocytomas in the genitourinary tract is very rare. This case report discusses a case of a male patient suffering from plasmocytoma of the urethra, who could be treated successfully by primary external beam radiotherapy.

Adult↗

[Familial plasmocytoma in mother and son].

Familial plasmocytoma in mother and son is documented in three cases, we can add one more. The review of the pertinent literature and our case disclose 40 reports of familial plasmocytoma. It seems that genetic factors may play a role in the pathogenesis of plasmocytoma.

Aged↗

[Extramedullary plasmocytoma of the nasal cavity: a case report].

PURPOSE OF STUDY: To establish a diagnosis and therapeutic management in patients with extramedullar plasmocytomas. METHOD: There is no consensus concerning extramedullar plasmocytoma treatment. One patient with a nasal cavity tumor location and a review of literature are reported. RESULTS: Extramedullar plasmocytoma is a rare tumor that occurs most frequently in the upper respiratory tract. The most common location is the nasal cavity. Diagnosis can only be made after histological and immunohistochemical examinations. Localized tumors are treated by radiation therapy, combined or not with surgery. Disseminated diseases are treated by chemotherapy. CONCLUSION: Diagnosis can only be confirmed after the exclusion of a systemic disease (multiple myeloma).

Adult↗

Primary lymph node plasmocytoma of IgD-class.

An extramedullary plasmocytoma in a right inguinal lymph node was observed in a 62-year-old woman. The tumor showed monoclonal proliferation of plasma cells which revealed highly positive stainings of both IgD and lambda light chain using the PAP-technique. Monoclonal IgD-lambda was also secreted in serum and urine. Clinical data, lymphography and bone biopsy showed a tumor spreading in the right inguinal, left iliacal and lumbar regions without systemic involvement of bone. In contrast to extramedullary plasmocytomas of other immunoglobulin classes the disease progressed rapidly and the patient died 8 months after the onset. The present case is the first reported IgD-plasmocytoma in lymph nodes.

Female↗

[Sternal plasmocytoma with monoclonal dysglobulinemia 28 months after a trauma. Pathogenic discussion (author's transl)].

A case of sternal plasmocytoma discovered 28 months after an occupational trauma is reported. At the first examination a small quantity of monoclonal IgG was present, and persisted in spite of a rather heavy treatment by irradiation and chemotherapy. To-day, more than eight years after the trauma, there is not any clinical or radiological sign of generalization, but bone marrow plasmocytosis is notably increased. Thus the long-dated prognosis is reserved. We discuss the frequency of solitary plasmocytomas of the sternum, the possibility of generalization sometimes after a very long delay, the relationship between the trauma and the plasmocytoma, the lack of pejorative significance of associated monoclonal dysglobulinemia. We suppose that in our case the dysglobulinemia antedated the trauma, this hypothesis is corresponding to the present concepts on oncogenesis, the trauma being only a cocarcinogenic agent.

Clone Cells↗

Spinal cord compression by solitary plasmocytoma.

A rare case of spinal cord compression caused by a solitary plasmocytoma situated at the level of the 9th and 10th thoracic vertebrae, is described. The solitary plasmocytoma should be included in the differential diagnosis of various types of spinal cord compression and considered as something completely separate from multiple myeloma (Willis, 1941). If operated on adequately, the solitary plasmocytoma can be considered a lesion with benign characteristics.

Adult↗

[Transformation of a rib plasmocytoma into an amyloid tumor (author's transl)].

The appearance of amyloidosis during the course of multiple myeloma is a well known fact and has an overall incidence of 6 to 15%. However, the total transformation of a plasmocytoma into a voluminous amyloid tumor is a very rare event. A female patient was diagnosed of lambda light chain disease after developing a conspicuous rib plasmocytoma over the same region where a pathological fracture had appeared three years before. She was treated with discontinuous courses of melphalan and methyl-prednisolone, and developed a reversible nephrotic syndrome and a pathological fracture of the right clavicle. At necropsy there was generalized amyloidosis and complete substitution of the rib plasmocytoma by amyloid substance, with another important accumulation of amyloid in the region of the clavicular fracture. The present concepts on amyloidogenesis in multiple myeloma are reviewed, and the peculiarities of the present case together with the possible role of initiating factors and the effects of therapy are discussed. The case herein reported appears to represent a human model of focal amyloidogenesis in myeloma.

Aged↗

Pancreatic and hepatic plasmocytomas: an exceptional case.

Extra medullary plasmocytoma (EMP) is a plasma cell proliferation of low frequency as compared to multiple myeloma which develops in the upper digestive and respiratory tracts and sometimes in the digestive tract. Other sites are possible but pancreatic or hepatic localizations are unusual with few cases being reported in the literature. This study describes an exceptional observation of pancreatic followed by hepatic plasmocytomas occurring after a long evolution of recurrent EMP. A review of the literature shows that the usual forms of treatment are surgery and radiotherapy. In our patient, this was possible for the pancreatic plasmocytoma, whereas the hepatic lesion was first treated with general polychemotherapy because of the tumour size and adverse prognostic factors. Surgery was then followed by recombinant interferon alpha therapy to obtain complete and hopefully prolonged remission.

Combined Modality Therapy↗

c-myc gene in a murine plasmocytoma without visible chromosomal translocations moves to chromosome 12F1 with Pvt-1 and rearranges with IgH enhancer-S mu sequences.

The DCPC 21 plasmocytoma lacks any of the MPC-associated chromosomal translocations. However, the c-myc gene has been transposed to the IgH locus on chromosome 12 by an Ig switch-region-mediated recombination mechanism. DNA sequencing analysis, further, revealed that this recombination is consistent with an insertion of the IgH enhancer (E mu)-S mu sequences, 2341 bp in length, into the c-myc 5'-flanking region, resulting in 5': c-myc 5'-flanking-E mu-S mu-c-myc 5'-flanking-c-myc exon-1: 3' segment. In situ molecular hybridization of DCPC 21 metaphase chromosome spreads using a Pvt-1 probe demonstrated that Pvt-1 has also moved to the F1 sub-band region of chromosome 12 where the IgH genes are located. These results indicate that the c-myc gene has been inserted into the IgH locus together with the Pvt-1, regardless of whether plasmocytoma has cytogenetically identifiable translocations. The possible interaction between c-myc activation and Pvt-1 in the development of MPCs is discussed.

Animals↗

Solitary bone plasmocytoma of the spine in an adolescent.

Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.

Adolescent↗

IgD/lambda plasmocytoma with immunoglobulin kappa light-chain genes in the germ-line configuration.

Human immunoglobulin (Ig) genes are rearranged in an ordered sequence of events during B-cell differentiation: starting at the IgH locus, a productive VHDJH rearrangement leads to the expression of mu chains. Light-chain gene rearrangements have been found in pre-B cells which express mu chains. In these cells rearrangements of Ig kappa light-chain genes precede that of lambda genes. In an IgD/lambda-producing plasmocytoma, however, we found an apparent exception to this rule: the kappa genes were not rearranged. Together with the observation that roughly 90% of human IgD plasmocytomas produce lambda light-chain proteins, the finding reported here leads us to suggest that lambda light-chain genes are rearranged preferentially in IgD-producing plasma cells. Ig gene rearrangement, isotype switch, and the phenomenon of isotypic and allelic exclusion are discussed with special reference to our findings.

Gene Rearrangement↗

Spinal cord compression caused by plasmocytomas. A retrospective review of 14 cases.

Plasmocytoma is a plasma cell tumor, which occurs in various structures of the body. When the spinal column is involved, it may cause cord compression. In this study, 14 cases of spinal plasmocytomas are presented. Seven of them were male and seven female. The major complaints were pain in twelve cases, motor weakness in eight cases, and bladder disturbance in six cases. On neurological examination, twelve of the patients had impairment of extremity movements (85.7%), and eight had sensory losses (57.1%). Compression was more frequent in the thoracic region. There was a total block in nine and partial block in five cases. All patients underwent surgery. In thirteen cases laminectomy was performed, in one thoracotomy. In this report, complaints, clinical and laboratory findings, neurological examination, and histopathology of our cases are reviewed and the results discussed.

Adult↗

[Extramedullary plasmocytoma of the bronchial system].

We present a case of primary plasmocytoma of the left upper bronchus. Occlusion led to atelectasis of the left upper lobe and subsequent poststenotic pneumonia. Therapy consisted of local excision of the bronchus and postoperative radio-therapy. This type of lung-conserving therapy in a case of primary plasmocytoma has not been described before.

Bronchi↗