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Extramedullary plasmacytoma of the cavernous sinus.

We present the case of an 80-year-old male with an history of multiple myeloma (MM) stage I with extramedullary plasmacytoma of the neck, diagnosed 18 months before and in complete remission after radiation therapy and melphalan-prednisone therapy. He was admitted with signs and symptoms characteristic for cavernous sinus syndrome, including diplopia, exophthalmia, ptosis and orbital pain. Magnetic resonance imaging showed a mass lesion in the cavernous sinus, consistent with relapsing extramedullary plasmacytoma. The patient received palliative radiation therapy and high dose dexamethasone, but treatment failed and the patient died. This case represents one of the few reports of extramedullary plasmacytoma of the cavernous sinus. The development of a clinical presentation of cavernous sinus syndrome in a patient with a history of MM or extramedullary plasmacytoma should raise the suspicion of a plasmacytic involvement of the cavernous sinus.

Aged↗

Vascular endothelial growth factor (VEGF) expression in plasmacytoma.

Vascular endothelial growth factor (VEGF) plays an important role in angiogenesis. Although the role and importance of angiogenic factors such as VEGF have been established in various solid tumors, this has not been widely evaluated in hemopoietic neoplasias. In this trial, VEGF was studied in plasmacytoma and VEGF expression was compared with histopathologic grade. Forty-seven samples have taken from cases with plasmacytoma (Pm) (33 bones and 14 soft tissue plasmacytomas) were used as study material. Pm was the initial presentation in all cases, and bone marrow (BM) involvement was detected in 19 cases with systemic evaluation. Twenty-seven of the cases were male (age range was between 19 and 81 years). Histopathologically 27 cases had mature and 20 cases had immature morphology. Immunohistochemical analysis was used to detect VEGF expression and this was scored according to the percentage of the VEGF stained cells. VEGF expression was detected in 32 cases and in eight cases this expression was strong. In 11 cases expression was moderate and 13 cases showed mild expression. When we compared VEGF expression with pathologic grade, 17 of 20 immature samples showed VEGF expression while 15 of 27 mature samples showed VEGF expression. There was a statistically significant association between immature morphology and VEGF expression (p < 0.0264). Additionally all the samples, except one, with strong VEGF expression showed immature morphology. In conclusion two thirds of the cases with Pm showed VEGF expression and this was associated with immature morphology. Increased expression of VEGF was seen in plasmacytomas, and additional studies are needed to determine whether this translates to increased microvasculature or increased risk of progression to myeloma.

Adult↗

Chromosomal translocations activating myc sequences and transduction of v-abl are critical events in the rapid induction of plasmacytomas by pristane and abelson virus.

Plasmacytomas with short latent periods can be induced in BALB/c mice by a single intraperitoneal (i.p.) injection of 0.5 ml pristane followed 20-40 d later by an injection of Abelson virus. The karyotypes of 18 such tumors were determined; 10 of these had rcpt 12;15, 5 had rcpt 6;15 and 3 had no translocations, but two of these have been shown to have interstitial deletions of chromosome 15. The specific breakpoints were the same as described in pristane-induced plasmacytomas, i.e., at 15D2 /3, 6C2 , and 12F2 . Near diploid karyotypes and trisomy of chromosome 11 were frequently seen. All of the Abelson-plus-pristane-induced plasmacytomas (ABPC) were studied as transplanted tumors, contained integrated v- abl sequences, and actively transcribed v- abl mRNA. All but one of these tumors contained abundant myc RNA transcripts. The shortness of the latent periods of the ABPC suggests that the rcpt 12;15 and rcpt 6;15 occur soon after pristane administration and are present at the time Abelson virus is introduced. In this form of plasmacytomagenesis , activated v- abl genes appear to bypass other genetic changes that require a much longer period of time in pristane plasmacytomagenesis . Nonetheless, the consistent finding of chromosome-15 alterations and abundant myc expression in these plasmacytomas emphasize the apparent need for multiple events even in the genesis of some tumors induced by rapid transforming viruses.

Abelson murine leukemia virus↗

Solitary extramedullary plasmacytoma of the breast with serum monoclonal protein: a case report and review of the literature.

A case of a solitary extramedullary plasmacytoma of the breast in a 73-year-old woman is reported. This is the second case of solitary breast plasmacytoma reported in the world literature. Plasmacytomas of the breast are extremely rare, especially those that are not associated with myelomatosis or disseminated disease. An associated monoclonal protein was present in the serum and was immunologically identical to the immunoprotein found in the tumor cells. The subject of extramedullary plasmacytomas in the breast is reviewed and discussed.

Aged↗

Specificity of the deletion of chromosome No. 15 in mouse plasmacytoma: brief communication.

Q-banding studies in two independent hyperdiploid mouse plasmacytomas, 63-1 and the NP-38-ABCD subline of MSPC-1, revealed that deletion of a No. 15 chromosome was common. The deletion of No. 15 appeared to result from a translocation between No. 12 and 15 in the NP-38-ABCD, as in most plasmacytomas. In 63-1, however, No. 10 was probably a recipient chromosome of the missing segment from the deleted No. 15, and both chromosomes No. 12 remained intact. These karyotypic features suggest that the deleted No. 15 is a tumor-specific marker chromosome in mouse plasmacytoma and that an abnormality of No. 12 is not essential for development of the tumor. The constitution of chromosomes in the two plasmacytomas remained remarkably stable in their homogeneous modal population.

Animals↗

c-myc Gene rearrangements involving gamma immunoglobulin heavy chain gene switch regions in murine plasmacytomas.

In murine plasmacytomas, the c-myc gene has frequently been found to undergo rearrangement by virtue of a T(12;15) chromosome translocation. The immunoglobulin heavy chain gene switch region (S alpha) constitutes the target for most of these recombinations particularly in IgA producing plasmacytomas. We sought to identify non-S alpha myc target sites in several IgG producing tumors. The c-myc target in MPC-11 (a BALB/c IgG2b producing plasmacytoma) has been cloned, localized to the Igh-C locus and identified as the gamma 2a heavy chain gene switch region (S gamma 2a). Furthermore, by Southern blot hybridization, we have determined that the S gamma 2b region is the c-myc target in two NZB IgG2b producing plasmacytomas. The potential relation between Ig class expressed and c-myc translocation target is discussed.

Animals↗

Primary cutaneous plasmacytoma--report of two cases and review of the literature.

Primary cutaneous plasmacytomas are very rare. In this report, we describe two such cases and review the literature on this entity. Both patients presented with a slowly growing, painless, and solitary mass on the chest wall. Histologically, one case was composed of mature-looking plasma cells, while the other was composed of immature and anaplastic plasma cells, infiltrating the dermis. The epidermis was spared. Kappa light-chain restriction was demonstrated by immunohistochemical techniques in both cases. There was no evidence of marrow disease even on repeated marrow biopsies, although extracutaneous lesions were detected in one patient. One remained in complete remission 6 years following local irradiation. The other patient was treated with local irradiation and systemic chemotherapy, with a complete response, but this was followed by multiple cutaneous recurrences and further remissions by treatment with cytotoxic agents. The present cases and those reported in the literature illustrate well the variable clinical course of primary cutaneous plasmacytoma. Although cure can apparently be achieved in some patients by local radiation therapy, more than half of the cases relapse or progress to myelomatosis. The disease-related mortality is at least 40%. Thus cutaneous plasmacytoma appears to be more aggressive than noncutaneous extramedullary plasmacytomas and should be separately categorized from them in future studies.

Aged↗

Ga-67 and Tl-201 scintigraphy in extramedullary plasmacytoma: a case report.

Extramedullary plasmacytoma is a rare form of plasma cell tumor occurring in a wide variety of organs and tissues. Most tumors occur in the head and neck, involving the nasal cavity, paranasal sinuses, and upper airway. Tl-201 and Ga-67 scan findings in a patient with extramedullary plasmacytoma in the right side of the chest wall are presented. Only a few publications have described Tl-201 or Ga-67 uptake in extramedullary plasmacytoma. This is the first report of both Tl-201 and Ga-67 uptake in an extramedullary (or intramedullary) plasmacytoma.

Female↗

The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage.

We describe an easily recognizable and previously not individualized clinical syndrome that can reveal solitary plasmacytoma of bone. We report 4 patients with a slowly extending violaceous skin patch overlying a solitary plasmacytoma of bone, associated with enlarged regional lymph nodes. Biopsies of the cutaneous lesion and the lymph nodes were not specific, although increased dermal mucin deposition and vascular proliferation were present in all skin specimens. Three patients had associated polyneuropathy. One patient had POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes) syndrome at the time the plasmacytoma was diagnosed. Another patient developed POEMS syndrome, from which he died, 4 years after excision of the plasmacytoma. The 3 other patients were treated either with irradiation or with a combination of irradiation and surgery, and recovered completely, including from the associated neuropathy and/or POEMS syndrome. We suggest calling this unique and distinctive clinical presentation the for

Aged↗

Bronchial plasmacytoma identified by immunoperoxidase technique on paraffin embedded section.

A case of bronchial plasmacytoma occurring in a 57-year-old housewife is reported. She had had the productive cough and the "abnormal shadow" in the right lower lobe for three years before admission. On bronchoscopy, a tumor was found in the right main bronchus, large enough to obstruct the air way. The tumor was resected through rigid bronchoscope. Histological impression was "plasmacytoma with local amyloid deposit." M-protein was never detected in the serum or urine. Applying the immunoperoxidase technique for the paraffin section, the plasma cells were found to contain only a single type of immunoglobulin, Ig G-L. The differential diagnosis between plasmacytoma and plasma cell granuloma was made, and plasmacytoma was considered to be one type of extranodal malignant lymphoma.

Antibodies, Monoclonal↗

Clinical course and survival in 16 patients with localized plasmacytoma.

The clinical course of 16 patients with localized plasmacytoma, 9 with solitary plasmacytoma of bone (SPB), and 7 with extramedullary plasmacytoma (EMP) are presented. Median follow up of SPB was 77 months and of EMP 74 months. The EMPs were localized in the upper respiratory tract (4 cases), the gastro-intestinal tract (2 cases) and the skin (1 case). The SPBs were localized in the ribs (3 cases), the spine (3 cases), the humoral bone (1 case), the skull (1 case) and the mandibular bone (1 case). In 3 patients with EMP and in 5 patients with SPB, the monoclonal protein could be determined at presentation. Local recurrences following surgical removal or irradiation occurred in 2 patients with EMP and in 1 patient with SPB after 12-60 months. Classical multiple myeloma developed in 3 patients with EMP and in 4 patients with SPB, 9-130 months after diagnosis. The monoclonal protein level proved to be a useful disease marker which (re)appeared at local recurrence or at dissemination. The median survival of the 16 patients with solitary plasmacytoma was 138 months, which is considerably longer than the median survival of 42 months in 14 stage-I patients with classical multiple myeloma who were observed during the same follow-up period.

Adult↗

Solitary plasmacytoma of bone and soft tissues.

Between 1956 and 1978, nine patients with solitary plasmacytoma of bone (SPB) and seven with extramedullary plasmacytoma (EMP) were treated at the University of Washington Hospital and Swedish Tumor Institute. All but one patient had local radiotherapy. In the SPB group, six of nine patients progressed to multiple myeloma (MM) and five died of disease within three years after dissemination. Three of the nine patients are alive at 5, 8, and 16 years, respectively. In the EMP group, none of the seven patients progressed to MM. Five are alive 16 months to 23 years after radiotherapy. Since there are no reliable criteria for prospectively distinguishing true solitary plasmacytoma from occult MM, all patients with apparently isolated plasmacytoma should receive local radiotherapy with curative intent.

Adult↗

Primary plasmacytoma of lymph nodes. A case report.

Most plasmacytomas of lymph nodes are secondary metastases from myeloma or from primary plasmacytoma of the upper air passages. Primary plasmacytomas of lymph nodes are very rare. A case of primary plasmacytoma of mediastinal, cervical and para-aortic lymph nodes is reported. Bence Jones proteinuria of 8 g/24 h was present. Repeated bone marrow examinations were normal. When the patient died, 17 months after the first symptoms, the bone marrow was still not involved.

Adult↗

Extramedullary solitary plasmacytoma of the head and neck. A clinicopathological study.

Monoclonal extramedullary plasmacytoma (EMP) is a rare, low-grade lymphoma found predominantly in the head and neck region. Only since the introduction of immunophenotyping techniques 2 decades ago has it been possible to differentiate EMP from benign polyclonal plasma cell proliferation. The purpose of this study was to trace the evolutionary profile of the disease under consideration of monoclonality assessment. The records of 24 patients with morphologically diagnosed EMP treated in a single institution underwent clinical and pathological review. Only 14 patients had true monoclonal plasmacytoma. No EMP-related deaths occurred. Two patients had local recurrence, and 2 patients developed multiple myeloma. Review of the literature confirms the low-grade malignancy of EMP. Diagnostic procedures must exclude benign polyclonal plasmacytoma, multiple myeloma, and solitary bone plasmacytoma. The slow natural progression of the disease and the rarity of secondary multiple myeloma favor nonmutilating local surgery whenever possible to avoid the long-term sequelae of radiotherapy.

Adult↗

Solitary plasmacytoma of the jaw occurring in an elderly woman affected by hepatitis C virus infection: a case report.

Solitary plasmacytoma accounts for a small percentage of plasma cell neoplasms. The disease often affects older people and is potentially curable. Only a few cases of solitary jaw plasmacytoma have been described in the literature. Here we report the case of a 76-year-old woman affected by chronic hepatitis C infection and isolated plasmacytoma of the left jaw. Plasmacytoma was diagnosed in January 2001, but the swelling of the mandible had been present since 1993. Two different pathologists made the diagnosis on the basis of biopsy material from the mandibular swelling.

Aged↗

Transgenic expression of CD40 ligand produces an in vivo antitumor immune response against both CD40(+) and CD40(-) plasmacytoma cells.

Because tumor-specific antigens have been identified in multiple myeloma (MM), immunotherapy might provide an additional treatment modality for the disease. Expression of CD40 ligand (CD40L) proximate to the MM cells might serve this purpose, either by increasing their capacity to present self-antigens by activation through their CD40 receptor or by the recruitment of professional antigen-presenting cells (APCs) able to take up and present tumor-associated antigens. To distinguish between these possibilities and predict whether human CD40(-) myeloma might respond to this approach, we examined 3 murine plasmacytoma cell lines, 2 (MPC-11 and S107) expressing the CD40 molecule and 1 (X-24) lacking such expression. Syngeneic BALB/CBYJ mice were inoculated subcutaneously with tumor cells mixed with CL7.1 fibroblasts, retrovirally transduced to express either the mCD40L or the neo gene. For all 3 plasmacytoma cell lines, coinjection with CL7.1/mCD40L significantly reduced local tumor growth compared with controls. This effect was mediated by a systemic antitumor immune response, since mice immunized with tumor and CL7.1/mCD40L were resistant to subsequent challenge with tumor, and tumor growth inhibition was abolished when CD8(+) or CD4(+) lymphocytes were depleted. Because expression of CD40L gave equivalent protection from CD40(+) and CD40(-) tumors and transgenic-CD40L failed to up-regulate costimulatory molecules in either tumor, the protective effects of CD40L probably resulted from recruitment/activation of professional APCs rather than from CD40 activation of plasmacytoma cells. As further support of this concept, we found that mice were also well protected if CL7.1 and CD40L were injected together with apoptotic plasmacytoma cells from these tumors. Hence, transgenic CD40L expression may produce an antimyeloma immune response against either CD40(+) or CD40(-) tumors and may be of therapeutic value for both types of myeloma in humans.

Animals↗

Dural plasmacytoma.

Dural plasmacytoma is an unusual form of myeloma. We describe a woman with plasmacytoma of the tentorium cerebelli that was managed successfully with surgical decompression and radiotherapy. Computed tomography, not previously reported in cases of dural plasmacytoma, was useful in her management. Another unique feature was the restoration of a normal immunoglobulin G content in the blood and cerebrospinal fluid after local treatment of the neoplasm. Two previously reported cases showed similar normalization of cerebrospinal fluid immunoglobulin G after local radiotherapy. Dural plasmacytoma presents a characteristic clinical syndrome. The typical patient is a woman (92% of the reported cases) in the 5th decade of life. Clinical findings reflect intracranial hypertension, often with focal neurological signs, consistent with the usual dural or tentorial origin of the tumor. Immunoglobulin abnormalities may be found in serum or cerebrospinal fluid. The prognosis is good after surgical decompression and local radiotherapy.

Adult↗

Management of plasmacytomas of the spine.

Epidural plasmacytoma often reflects systemic myelomatous involvement and usually has a poor prognosis. The isolated spinal plasmacytoma, however, is a lesion with the potential for long term remission or even cure in some cases. We report six patients with isolated plasmacytoma of the low thoracic or lumbar region, all of whom presented with pain and minimal neurological deficits. Our approach to such cases included a complete medical work-up and radiographic definition of the lesion with a bone scan, a skeletal survey, myelography, computed tomography, and, in some instances, spinal angiography. All of these patients underwent laminectomy and spinal fusion with autologous bone and Harrington rods. There was no morbidity associated with the procedure, which was designed to prevent possible collapse during subsequent radiotherapy or chemotherapy, with its attendant potential for neurological catastrophe. Several patients have developed systemic involvement necessitating chemotherapy, but in follow-up extending now to 4 years all patients remain ambulatory and pain-free. We are encouraged by the potential for symptomatic relief and neurological improvement of patients with isolated spinal plasmacytomas who are treated with an aggressive medical and surgical approach.

Adult↗