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Extramedullary plasmacytoma of the oropharynx producing kappa-type Bence-Jonas protein.

A case of extramedullary plasmacytoma in the oropharynx of a 41 year-old woman is reported. Histologically, the tumor was plasmacytoma consisting mainly of matured plasma cells and showing a deposit of amyloid in the interstitial structure. Immunoelectrophoretic studies of the concentrated urine specimen revealed a kappa-type Bence-Jones protein even though there was no increase in blood M-protein. Lineac electron radiation of 5,100 rad was given over a period of eight weeks but no effect was noted. After the tumor was surgically removed, follow up of the patient was made for 2 years without evidence of recurrence or metastasis.

Adult↗

Monoclonal gammopathy of undetermined significance and solitary plasmacytoma. Implications for progression to overt multiple myeloma.

MGUS is characterized by the presence of a serum M-protein less than 3 g/dL; fewer than 10% plasma cells in the bone marrow; no, or only small amounts of, M-protein in the urine; absence of lytic lesions, anemia, hypercalcemia, and renal insufficiency; and, most importantly, stability of the M-protein and failure of development of other abnormalities, MGUS is found in approximately 3% of persons older than 70 years and in 1% of those 50 years or older. During long-term follow-up, approximately one fourth of patients develop multiple myeloma (MM), amyloidosis, macroglobulinemia, or a similar malignant lymphoproliferative disorder. Actuarial rate of development of serious disease was 16% at 10 years, 33% at 20 years, and 40% at 25 years in our experience. The interval from recognition of the M-protein to the diagnosis of MM ranged from 2 to 29 years (median, 10 years). The size of the M-protein, hemoglobin value, percentage of bone marrow plasma cells, amount of light-chain excretion, presence of hypercalcemia or renal insufficiency, and presence of lytic bone lesions are often helpful in differentiating MGUS from MM and macroglobulinemia. The plasma cell labeling index and the presence of circulating plasma cells in the peripheral blood are indicators of active disease; however, there are no findings at the diagnosis of MGUS that reliably distinguish patients who will remain stable from those in whom a malignant condition will develop. Thus, a physician must perform serial measurements of the M-protein in the serum and periodic evaluation of the pertinent clinical and laboratory features to determine whether MM, macroglobulinemia, systemic amyloidosis, or related disorders have developed. Solitary plasmacytoma is characterized by the presence of a tumor consisting of monoclonal plasma cells identical to those in MM. In addition, skeletal roentgenograms must show no lytic lesions, a bone marrow aspirate must contain no evidence of MM, and immunoelectrophoresis or immunofixation of the serum and concentrated urine should show no M-protein. Exceptions to the presence of an M-protein occur, but therapy of the solitary lesion often results in disappearance of the M-protein. Tumoricidal irradiation (4000 to 5000 cGy) for approximately 4 weeks is the treatment of choice. Overt MM occurs in approximately 50% of patients with solitary plasmacytoma. Progression occurs in most patients within 3 years. The three patterns of failure are (1) development of MM, (2) local recurrence, and (3) development of new bone lesions in the absence of MM.

Adult↗

A case of retroperitoneal extramedullary plasmacytoma with multiple metastases.

We investigated a patient with extramedullary plasmacytoma in the retroperitoneal space with multiple metastases using abdominal CT scan and 67Ga scintigraphy. Abdominal enhanced CT revealed the retroperitoneal tumor mimicking lymphoma. 67Ga scintigraphy showed multiple tumor localization. We could detect retroperitoneal extramedullary plasmacytoma with multiple metastases using 67Ga scintigraphy. This retroperitoneal tumor may appear similar to lymphoma on CT.

Diagnosis, Differential↗

Extramedullary plasmacytoma of maxillary sinus with later involvement of the gall bladder and subcutaneous tissues.

We recently encountered a patient with extramedullary plasmacytoma (EMP) originating in the maxillary air sinus, who developed subcutaneous tissue and gall bladder involvement 2 years after initial local radiotherapy. Treatment with melphalan, prednisone and interferon led to a rapid and sustained regression of the gall bladder tumour. Complete remission was obtained by subsequent regional radiotherapy to the subcutaneous tumours in the extremities. Progression of plasmacytoma to the hepatobiliary system and subcutaneous tissues is quite rare and the effects of chemo- and radiotherapy on these lesions have not been fully elucidated. This study demonstrated that EMP can be well controlled and complete remission obtained by various therapeutic strategies, even though this tumour develops widespread dissemination.

Gallbladder Neoplasms↗

Spontaneous development of plasmacytomas in a selected subline of BALB/cJ mice.

Sixty per cent of BALB/cAnPt mice injected intraperitoneally (i.p.) with tetramethylpentadecane (pristane) develop plasmacytomas (PCs), whereas less than 10% of BALB/cJ develop such tumours. Most other mouse strains are completely resistant. Resistance is dominant over susceptibility in F1 hybrids between BALB/cAnPt and the resistant non-BALB/c strains, suggesting that susceptibility may be due to some genetic defect. (BALB/cAnPtxBALB/cJ)F1 hybrids have a PC incidence of 36-42%. Previously, BALB/cJ has been shown to harbour at least one resistance gene (Potter et al., Genomics 1988, Vol. 2, pp. 257-262). On the assumption that BALB/cJ may contain a segregating resistance gene, we cross BALB/cJ females with pristane-pretreated BALB/cJ males that were found to be carrying PC cells intraperitoneally 5-7 months after pristane treatment. After two selective crosses, 62% of the BALB/cJ subline BALB/cM2/22 developed PC after pristane and 52% after pristane followed by Abelson virus, while unselected controls had an incidence of 11% and 0%, respectively. Moreover, six spontaneous plasmacytomas developed in untreated females of the selected colony. Five of these carried T(12; 15) (F2; D2/3) translocations. The sixth had a T(1; 10) (G; C1) translocation and an interstitial duplication of segment (C1/E3) on one chromosome 5. It may be concluded that pristane treatment is not a prerequisite for the induction of the PC associated Ig/myc translocations.

Animals↗

Solitary plasmacytoma with intracranial intraorbital and, paranasal sinus extension.

Intracranial solitary plasmacytomas (ICSPs) are extremely rare tumours in neurosurgical practice, and are often misdiagnosed preoperatively. Here we present a solitary intracranial plasmacytoma with orbital, nasal and paranasal sinus extension. A subtotal excision of the tumour was performed and the complete response was seen after postoperative radio-chemotherapy. The neuroradiological and neurosurgical features of the case are discussed with the pertinent literature.

Adult↗

Primary extramedullary plasmacytoma in the atria of the heart.

A rare case of primary extramedullary plasmacytoma of the heart is described. The large space-occupying tumor in the atrial wall and its associated significant pericardial effusion caused marked impairment of cardiovascular function. The diagnosis was made via intravenous biopsy. Immunohistochemistry demonstrated CD45 and kappa light chain expression. Other B-cell- and T-cell-specific lymphoid markers were negative. Elevated kappa light chain was detected in serum by immunofixation electrophoresis. The differential diagnosis of plasmacytoma should be considered in cardiac tumors when routine screening is negative with lymphoid (CD20, CD3) and soft tissue immunohistochemical markers.

Aged↗

Cardiac plasmacytoma.

A 69-year-old white woman presented with a 3-month history of progressive dyspnea, orthopnea, fatigue and weakness. Clinical, diagnostic imaging and echocardiographic investigations suggested an occult primary cancer with metastasis to the heart. The patient's condition deteriorated gradually, and she died 2 months later. At autopsy, a malignant tumor encasing the heart and a 1-cm solitary tumor nodule in the lower lobe of the left lung were found. Histologic and electron microscopic studies revealed a plasmacytoma predominantly involving the epicardium and a small solitary plasmacytoma located in the left lung. The two tumors were further confirmed by immunohistochemical studies that showed monoclonal IgG expression and kappa light chain restriction.

Aged↗

Primary extramedullary plasmacytoma of the esophagus.

An uncommon manifestation of plasma cell neoplasia occurs outside the bone marrow and is designated "extramedullary plasmacytoma." These are usually encountered in mucosal sites of the head and neck region. The gastrointestinal tract may be secondarily involved in multiple myeloma or be the site of primary extramedullary plasmacytomas (PEMPs). The esophagus is the least common site of gastrointestinal PEMP. A 58-year-old man presented with dysphagia for solids over a period of 2 months. Otherwise, he was well and systemic examination did not reveal anything of note. After a nondiagnostic biopsy, the patient was subjected to esophago-gastrectomy. Gross examination of the esophagus revealed a large polypoid tumor. Histologically, it was composed of mature plasma cells, plasmablasts (some of which appeared anaplastic), and a minor admixture of lymphoid cells. Focally, the infiltrate permeated the squamous epithelium simulating lymphoepithelial lesions. The neoplastic cells were positive for epithelial membrane antigen, CD79a, IgG, and kappa, while the lymphoid cells were predominantly B cells. The patient did not have a monoclonal gammopathy. Skeletal x-rays and bone trephine examination were both normal. PEMP is biologically and prognostically different to other plasma cell neoplasms. Although rare, esophageal PEMP should be considered in the differential diagnosis of so-called undifferentiated malignant tumors of the esophagus.

Biomarkers, Tumor↗

Plasmacytoma of middle ear and upper respiratory tract.

The first case of extramedullary plasmacytoma of middle ear is reported. One case of plasmacytoma of the nasal cavity alone and another of the nasal cavity and maxillary sinus have also been described. Some of the problems in diagnosis and management have been discussed.

Adult↗

Detection of plasmacytoma of the nasal cavity by immunoelectrophoresis of nasal washing fluid.

A patient with a solitary IgG-lambda plasmacytoma of the nasal cavity is described. Immunoelectrophoresis of the nasal washing fluid showed the presence of the same paraprotein. After radiotherapy the tumour and the paraprotein disappeared. Recurrence of the tumour after 6 months was again associated with the presence of the paraprotein in the nasal washing fluid. Immunoelectrophoresis of nasal washings may offer an easy method in detection and follow-up of localized plasmacytomas of the upper respiratory tract.

Aged↗

Plasmacytoma of the larynx.

Plasmacytoma of the larynx is rare; thirty-one cases are reported in the literature and in this paper a further case is presented. The difficulty in diagnosing plasmacytoma is mentioned. Investigations to exclude multiple myeloma are mandatory to be sure that the disease is a localized lesion. Treatment of a localized lesion is essentially by radiotherapy but in the case of widespread or systemic disease, chemotherapy is recommended. A scoring system utilizing different parameters is suggested to decide whether the case has passed the borderline between local and systemic disease, a line which is invariably difficult to define.

Diagnosis, Differential↗

Extramedullary plasmacytoma.

Extramedullary plasmacytomas of the head and neck are very uncommon tumours. Three rare cases of extramedullary plasmacytoma are presented-one each in the nose, larynx and maxilla.

Adult↗

Extramedullary plasmacytoma of the larynx.

A case of IgD myeloma in a 54-year-old male with a long-standing history of extramedullary plasmacytoma involving the larynx is reported. The patient was treated with radiation therapy and laryngectomy. Twelve years later, he complained of nasal bleeding. On examination he was found to have large masses in the left nasal cavity and in the left supraclavicular region. Histological examination of both lesions showed plasmacytoma. Serum immunoglobulin studies revealed an IgD monoclonal spike of the lambda type. Bence-Jones protein was present. Using the immunoperoxidase staining technique, cytoplasmic monoclonal IgD was detected.

Bone Neoplasms↗

Plasmacytoma of the hyoid.

A case of plasmacytoma arising from the hyoid bone is described, this is a distinctly unusual site, as the marrow-containing flat bones are more commonly affected. On a review of the literature this appears to be the first report of plasmacytoma occurring in the hyoid bone. The patient underwent a CT scan and plain radiographs. Histopathology was diagnostic.

Bone Neoplasms↗

Nodal marginal zone B-cell lymphoma resembling plasmacytoma arising from a plasma cell variant of localized Castleman's disease: a case report.

Nodal marginal zone B-cell lymphoma (NMZBL) occasionally represents prominent plasma cell differentiation. Recently, primary lymph node plasmacytoma has been suggested to represent an extremely plasmacytic differentiation of NMZBL. We here report a case of NMZBL showing histological features resembling plasmacytoma arising from a plasma cell variant of localized Castleman's disease (PCLCD). The patient was a 69-year-old Japanese female with a 20-year history of a right inguinal mass. Histologically, a prominent proliferation of plasma cells occupied the interfollicular area of the central portion of the lymph node, whereas centrocyte-like (CCL) cells were the main cellular component in the peripheral portion of the lymph node. Although most of the plasma cells were mature 'Marshalko-type', occasional atypical forms with enlarged nuclei were also present. The majority of the lymphoid follicles had atrophic or regressive germinal centers. A few lymphoid follicles were colonized by CCL cells. Immunohistochemistry study revealed that both plasma cells and some CCL cells had a monotypic intracytoplasmic lambda light chain. When monoclonal plasma cell infiltration is observed in PCLCD, the light chains are mostly restricted to the lambda chain. This case suggests that some plasma cell-containing tumors arising from PCLCD may represent a variant of NMZBL.

Aged↗

Aberrant rearrangement of the kappa light-chain locus involving the heavy-chain locus and chromosome 15 in a mouse plasmacytoma.

The creation of a functional antibody gene requires the precise recombination of gene segments initially separated on the chromosome. Frequently errors occur in the process, resulting in the formation of a non-functional gene. The non-functional genes can be generated by incomplete rearrangements, frameshifts, or the use of pseudo V or J joining segments. It is likely that these aberrant rearrangements arise by the same mechanism as is used in generating functional genes, a process which we have suggested may involve unequal sister chromatid exchange. Aberrant rearrangements of immunoglobulin genes occur in normal lymphocytes and play a major part in allelic exclusion. However, it has recently been suggested that aberrant rearrangements involving immunoglobulin and non-immunoglobulin genes may be involved in tumorigenesis. This suggestion has been stimulated by the frequent occurrence of translocations involving chromosomes known to carry immunoglobulin genes in B-cell malignancies. The rearrangement of non-immunoglobulin DNA to the heavy-chain locus has recently been reported. Some aberrant rearrangements of the kappa locus appear to be due to rearrangements to sites that do not include the conventional sequence for V gene segment joining. Here we describe an aberrant kappa rearrangement that has led to the joining of DNA from chromosomes 15, 6 and 12, and so appears to be the result of chromosomal translocations or transpositions. As 15/6 or 15/12 translocations have frequently been found in mouse plasmacytomas (as have analogous translocations in human lymphocyte tumours) this aberrant kappa rearrangement may be unique to the plasmacytoma from which it was isolated.

Animals↗

A conserved sequence at c-myc oncogene chromosomal translocation breakpoints in plasmacytomas.

Many recent studies have shown that chromosomal translocation breakpoints frequently occur near cellular proto-oncogenes (reviewed in ref. 1). In both mouse plasmacytomas and Burkitt lymphomas, the c-myc oncogene becomes joined to an immunoglobulin heavy-chain gene in a head-to-head configuration. Within c-myc, the breaks frequently occur near the first exon-intron boundary, while within the immunoglobulin gene the breaks usually involve sequences directing heavy-chain switching. It has been assumed that the translocations represent abortive immunoglobulin switching events which have activated the c-myc gene for a role in tumour formation. However, sequence analysis of the c-myc gene does not reveal any apparent similarity to the immunoglobulin switch signals. With these results in mind, we have determined the precise breakpoints within c-myc for two plasmacytoma lines in order to search for any common features that may shed some light on the mechanism of chromosomal translocation. We report here that the tetranucleotide sequence GAGG occurs close to the breakpoint in five out of six translocations, and so may be a sequence recognized by either the enzymes that catalyse immunoglobulin heavy-chain switching, or some other DNA-cleaving activity.

Animals↗