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[Rare intracranial plasmacytoma manifestations. Case reports and review of the literature in diffuse plasmocytoma, in primary solitary extramedullary plasmacytoma in in primary solitary osseous plasmacytoma].

Plasmacytomas can be divided into multiple, solitary osseous and solitary extraosseous/extramedullary plasmacytomas. Intracranial plasmacytomas of the dura, leptomeninx and cerebrum are well known from the literature. They are manifestations of multiple myeloma, intracranial extramedullary plasmacytoma or metastatic disease of extramedullary plasmacytoma in distant locations. We describe a cerebellar manifestation of a solitary plasmacytoma of the bone, and a leptomeningeal carcinomatosis of a multiple plasmacytoma. A summary of the literature concerning intracranial plasmacytomas is given. Dural manifestations of plasmacytoma have the same features as meningiomas in CT or MRI. Cerebral or cerebellar manifestations cannot be differentiated from brain tumors by means of CT or MRI. In CT, plasmacytomas show high-density lesions. T2w-MRI reveals a low-intensity lesion. In T1w-MRI, intense homogeneous contrast enhancement can be demonstrated.

Bone Neoplasms↗

Localised plasmacytomas in Taiwan: comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone.

The clinical features and response to therapy of 32 Chinese patients with localised plasmacytoma are presented, and a comparison between extramedullary plasmacytoma (EMP) and solitary plasmacytoma of bone (SPB) is made. Twenty-two patients had SPB and ten had EMP, accounting for 9% of all of our plasma cell neoplasms. Both groups had a male predominance with a median age of 54 years for SPB and 63 years for EMP. The common sites of SPB included vertebral bodies (15) and the skull (4). Most EMPs occurred in the oronasopharynx (6) and paranasal sinuses (2). An M-protein was detected in eight patients with SPB and in six with EMP. Seventeen patients with SPB and seven with EMP received radiation therapy, and all achieved initial local control. The pattern of failure in 22 patients with SPB manifested as local recurrence in two, multiple bone metastases without bone marrow plasmacytosis in two, multiple EMP progression in two, and development of multiple myeloma (MM) in one. There were two local recurrences, one further solitary bone involvement and one MM conversion in the EMP group. Local recurrence or dissemination was associated with the appearance of M-protein or an increase in the M-protein level in both groups. There was no significant difference in M-protein status or incidence and patterns of failure between the two groups. Patients with EMP had a more favourable overall survival than those with SPB (P = 0.03). The 5 year disease-free survival rate was 79% for EMP and 58% for SPB (P = 0.53). Patients aged less than 60 years had a better overall survival in the SPB group, but location of tumour, presence of M-protein, radiation dose and chemotherapy did not influence prognosis in either group. Our results indicate that adequate local therapy can result in long-term survival with a low frequency of MM progression for patients with localised plasmacytomas, and both EMP and SPB appear to be similar in terms of frequency and patterns of failure.

Adult↗

Metastatic cutaneous plasmacytoma: a case report associated with IgA lambda multiple myeloma and a review of the literature of metastatic cutaneous plasmacytomas associated with multiple myeloma and primary cutaneous plasmacytomas.

We present the case of a 67-year-old Japanese woman with immunoglobulin A lambda (IgA lambda) multiple myeloma (MM). She had firm nodular cutaneous lesions on the trunk and scalp without adjacent bone involvement. The patient was diagnosed as having IgA lambda MM of stage IIIA with 52% plasmacytosis in the bone marrow six months before the appearance of the cutaneous lesions. The abnormal plasma cells showed moderate to marked dysplasia in both the bone marrow and skin lesions. The abnormal plasma cells in the bone marrow exhibited abnormal karyotypes: 41, XX, der (1) t (1p; 1q), -4, -10, -14, -16, -17, 17p+, that differed from the "unfavorable" karyotype reported previously. We reviewed the cases of metastatic cutaneous plasmacytoma in MM and cases of primary cutaneous plasmacytoma that have been reported in English or Japanese and identified the Ig class. Among the 83 cases of metastatic cutaneous plasmacytomas in MM, IgG, IgA, IgD, and Bence-Jones protein were found in 52%, 23%, 16%, and 6%, respectively. A disproportionately high frequency of IgD lambda MM was found to have spread to the skin, compared with the frequency of IgD MM itself, which was present in only around 2% of the MM cases. Among the 18 primary cutaneous plasmacytomas, IgG, IgA, and Bence-Jones protein were found in 56%, 11%, and 17%, respectively, but no IgD was found.

Aged↗

Solitary plasmacytoma and extramedullary plasmacytoma of the paranasal sinuses and soft palate.

Solitary plasmacytoma of the paranasal sinuses are uncommon neoplasms of B lymphocyte origin. They comprise one per cent of all head and neck tumours of the upper respiratory tract. They can be solitary plasmacytomas of the bone (SPB), an extramedullary plasmacytoma or a local manifestation of multiple myeloma. Conversion to multiple myeloma happens more frequently in SPB. Radiotherapy is the common modality of treatment with, or without, adjuvant chemotherapy. Extramedullary plasmacytoma carries a better prognosis than a solitary plasmacytoma of the bone. We report four cases of solitary plasmacytoma of the bone and an extramedullary plasmacytoma of the paranasal sinuses and soft palate.

Female↗

Solitary phalanx plasmacytoma relapse with disseminated extramedullary plasmacytomas and myeloma after short duration of remission.

Solitary bone plasmacytomas account for 5-7% of multiple myeloma cases and are assumed to have a fairly good prognosis, with a long duration of relapse-free survival after primary local treatment. Isolated phalanx plasmacytoma is a very rare entity, because involvement of extremities is seen in less than 1% of all solitary bone plasmacytomas, where they are usually localized centripedally, often in the axial skeleton. A 68 year old patient with a lytic lesion involving 5th phalanx was diagnosed as having a biopsy-proven solitary plasmacytoma, with a negative work-up for coexisting plasma cell dyscrasia. Three and a half months after completion of radiotherapy of the involved phalanx, the patient was readmitted with hypercalcemia, renal insufficiency and subsequently diagnosed as having atypical plasma cell infiltration of marrow, and plasmacytomas involving the right vocal cord and the premaxillary region, as well as pathological ulna fracture. Plasmacytoma of the phalanx, with extreme short duration of remission and an aggressive type of clinical relapse, is in sharp contrast with the natural stable course of a solitary plasmacytoma where the use of systemic treatment is subject to intense debate.

Aged↗

[Solitary plasmacytomas of bone and extramedullary plasmacytomas].

Among plasma cell disorders, solitary plasmacytoma (solitary plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with multiple myeloma (MM). Furthermore, the relationship between solitary plasmacytoma and MM remains unclear. Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagnosis were: (1) no evidence of other lesions based on clinical and radiologic examinations; (2) biopsy evidence of a plasma cell neoplasm; (3) bone marrow biopsy specimen with negative findings (less than 10% plasma cells); (4) no anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SBP and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P > 0.05). We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytoma. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cementation. All patients with apparently isolated plasmacytoma should receive local radiotherapy after operation. Adjuvant chemotherapy should be given if the tumour turns out to be poorly differentiated, in order to delay their progression to MM.

Adult↗

Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different nosological entities?

BACKGROUND: The relationship between solitary plasmacytoma and multiple myeloma is still unclear, but they can be distinguished by their different clinical course. Indicators of disease activity and extension, and of a possible evolution to multiple myeloma, have not been identified as yet. METHODS: Two cases of solitary plasmacytoma are described: one of the mandible and one extramedullary plasmacytoma (EMP) of the rhinopharynx. Pathologic data included immunohistochemical staining for heavy and light Ig chains, and for the proliferating cell nuclear antigen (PCNA). Analysis of the peripheral immunological status and serum parameters (beta 2 microglobulin, thymidine kinase, IL-2, IL-6 and soluble IL-2 receptor) was performed and correlation was made with the clinical status. Flow cytometry analysis of nuclear DNA content and S-phase cell fraction were also studied in both neoplasms. RESULTS: Solitary plasmacytoma of bone (SPB) showed important basal immunologic alterations and a marked increase in all serum parameters considered with respect to EMP. Ploidy analysis demonstrated an almost complete aneuploidy cell population for the SPB patient (80%), whereas in the EMP patient only 2% of the cells were aneuploid. The S-phase cells were 16% and 4%, respectively. PCNA index was 60% in SPB and 10% in EMP. CONCLUSIONS: Solitary plasmacytoma of the bone appeared to be a more aggressive form of plasmacellular neoplasia, distinct from EMP and similar to multiple myeloma. The study of serum parameters, together with analysis of PCNA, ploidy and S-phase fraction, can aid in better understanding disease activity, and in the choice of more adequate treatment. Moreover, serial analysis of some serum factors might be useful markers for monitoring the disease.

Aged↗

Regulation of murine plasmacytoma transferrin receptor expression and G1 traversal by plasmacytoma cell growth factor.

Many plasmacytomas arising in BALB/c mice require a specific, macrophage-derived growth factor in order to proliferate in vitro. Since transferrin receptor expression is normally regulated by tissue-specific growth factors and because expression of these receptors is required for cell proliferation, we examined the interaction of plasmacytoma growth factor (PCT-GF) on transferrin receptor expression and cell cycle progression in several PCT-GF-dependent and independent plasmacytoma cell lines maintained in vitro. We found that removal of PCT-GF results in a rapid and specific loss of transferrin receptor expression with concomitant G1 arrest in early G1. The time required for G1 arrest to become maximal correlates closely to the initial level of surface transferrin receptor expression and the rate of decay following removal of PCT-GF. The calcium channel blocker diltiazem interferes with the ability of PCT-GF to maintain transferrin receptor expression in PCT-GF-dependent cell lines and causes a G1 arrest of the cell population. When added to a PCT-GF-independent cell line, diltiazem also inhibited transferrin receptor expression and caused G1 arrest. Thus, both PCT-GF-dependent and -independent plasmacytoma cell lines require transferrin receptor expression for growth. In factor dependent cell lines, transferrin receptor expression requires exogenous PCT-GF, while in factor-independent cells, transferrin receptor expression is constitutive. In both cell types, intracellular calcium levels may play a role in receptor expression.

Animals↗

Solitary plasmacytoma of bone and extramedullary plasmacytoma.

A small proportion of patients with plasma cell myeloma have a solitary plasmacytoma of bone. Strict staging criteria, including normal MR imaging studies of the axial skeleton and the long bones and absence of monoclonal plasma cells detected by flow cytometry or PCR, are required for diagnosis. Radiotherapy at a dose of 4500 cGy is required to eradicate the local tumor. Many patients enjoy prolonged disease-free survival, but the incidence of systemic relapse is high. It is expected, however, that if strict diagnostic criteria are applied some patients may be cured. Extramedullary plasmacytoma is an even rarer plasma cell disease which usually occurs in the head and neck area. Careful microscopic and immunohistochemical studies are required for the correct diagnosis, because this disease can be confused with other malignancies, particularly lymphomas. The treatment of choice is radiotherapy which, in cases of head and neck plasmacytomas, should encompass the adjacent lymph nodes. Most patients with extramedullary plasmacytoma can be cured, and fewer than 30% develop a distant failure in the form of multiple myeloma or multiple extramedullary tumors.

Bone Neoplasms↗

Surface immunoglobulins of lymphocytes in mouse plasmacytoma. IV. Evidence for the persistence of the effect of plasmacytoma-RNA on the surface immunoglobulins of normal lymphocytes in vivo and in vitro.

The previous findings were confirmed that RNA extracted from murine plasmacytoma alters the character of the lymphocyte surface immunoglobulins (SIg) to express the idiotypic specificity of the Ig of the plasmacytoma from which the RNA was derived (cell conversion). RNA extracted from spleens of plasmacytoma-RNA-injected BALB/c mice also had convering activity, and if injected into other mice, caused the appearance of RNA active in cell conversion in spleens of the second set of mice. This activity was lost only after two additional transfers. When splenic cells from animals 1 hr after injection with RNA extracted from MOPC 300, LPC-1 or MOPC 104E, were cultured for 7 days, the proportion of cells with the SIg specific for these tumours increased. The cell-converting activity of the RNA extracted from the cultured cells after 7 days incubation ('7-day' RNA) was higher than that of RNA extracted from cells after 1 hr incubation ('immediate' RNA). 'Seven-day' RNA could be used for sequential transfers without marked loss of activity in cell conversion for at least five transfers. The repetitive transferability of this phenomenon by the injection of plasmacytoma-RNA suggests the possibility of RNA replication in the recipient cells.

Animals↗

Solitary plasmacytoma of bone vs. extramedullary plasmacytoma and their relationship to multiple myeloma.

Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMPC) are unusual solitary tumors of plasma cell origin. The clinical differences between these tumors and multiple myeloma are the subject of continued study. This review examines the radiotherapy experience at M. D. Anderson Hospital. Between 1948 and 1977, 12 patients with SPB and 12 patients with EMPC were treated with radiotherapy. Radiotherapeutic doses were most often (22/24) greater than 4,000 rads, at 200 rads per day. In order to qualify as solitary plasmacytoma a disease-free interval of 3 years was required. In the group of SPB, 5 patients of 9 were alive with no evidence of disease (NED) at 3 years, 4 of 5 at 5 years, and none of 4 at 10 years. In the EMPC group, 8 patients of 11 were NED at 3 and 5 years, and 6 of 9 at 10 years. Six patients with SPB developed multiple myeloma compared with only two with EMPC. The results confirm the better prognosis of EMPC and support the theory that SPB and EMPC are two different entities.

Adolescent↗

Solitary plasmacytoma. I: Extramedullary soft tissue plasmacytoma.

The details of 16 patients with extramedullary plasmacytoma are presented. All but one presented with localized disease, and all were treated successfully by radiotherapy. Only one patient developed local recurrence and only one has developed disseminated disease. Radiotherapy is recommended as the primary treatment of choice for these localized radiosensitive tumors. It is suggested that the good prognosis of plasmacytomas of the head and neck, in contrast to plasmacytomas arising at other sites, is related to the observation that a high proportion of these tumors are localized at the time of diagnosis and that very large primary tumors are uncommon.

Adult↗

Solitary plasmacytomas of bone and extramedullary plasmacytomas. A clinicopathologic and immunohistochemical study.

Twenty-two patients with solitary plasmacytoma of bone (SPB) and 13 with extramedullary plasmacytomas (EMP) were studied. The average follow-up period for SPB was 90 months and 86 months for EMP. Thirty-six percent of patients with SPB developed multiple myeloma (MM) in an average of 39 months, and 23% of patients with EMP developed MM in an average of 23 months. No significant differences in survival, incidence of MM, or interval to the development of MM were found between the two groups. The 11 cases of EMP with evaluable tissue for immunohistochemical study were either monotypic kappa or lambda, as were 9 of 10 SPB. Presence of monoclonality did not predict the development of MM. The histologic parameters of nuclear immaturity and presence of prominent nucleoli seem to be the best indicators of which patients will develop MM. Solitary plasmacytoma of bone and EMP appear to be more closely related than has been previously recognized.

Adult↗

Solitary bone plasmacytoma and extramedullary plasmacytoma.

Solitary bone and extramedullary plasmacytomas are rare plasma cell proliferative disorders. Their diagnosis is based on histologic confirmation of monoclonal plasma cell infiltration of a single disease site and on the exclusion of systemic myeloma. For both entities, the treatment of choice is localized radiotherapy. With modern radiotherapy and with a total dose of at least 4000 cGy, the risk for local recurrence is less than 5%. There is no role for systemic chemotherapy in the management of these disorders. Approximately 30% of patients with solitary bone plasmacytoma (SBP) remain disease-free for several years; some of these patients may be cured. Patients with the best prognosis are those in whom the monoclonal protein disappears by 1 year after radiotherapy. The prognosis of patients with solitary extramedullary plasmacytoma (SEP) appears to be better than for patients with SBP because approximately 70% of patients with SEP remain disease-free at 10 years. With more sensitive staging procedures, the diagnosis of SBP and SEP may become less common, but the number of patients with prolonged stability and cure may increase.

Bone Neoplasms↗

Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different entities?

BACKGROUND: The objective of this study was to evaluate the similarities between solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EP). PATIENTS AND METHODS: The clinical features, treatment and survival of 54 patients with localized plasmacytoma (LP) 1990 were carefully reviewed. Follow-up was expected to continue until June 1993. RESULTS: Thirty-two patients were classified as having SPB and 22 EP. Most of the patients were males. particularly those in the SPB group. Their median age at diagnosis was 54 years and no significant difference between the two groups was observed. SPB occurred most frequently in the vertebral column (42%) and EP in the upper respiratory tract (73%). Fifteen patients with SPB and 2 with EP had paraproteinemia at diagnosis, multiple myeloma (MM) became evident in 75% of the patients with persistent paraprotein after therapy, and in only 22% of those in whom it disappeared. Four patients in the SPB group had immunoparesis, and 3 developed MM. Disease progression toward MM was significantly different (p=0.003) in the two groups, while overall survival differences were not significant (p=0.07) unless unrelated causes were excluded (p=0.02). Adjuvant chemotherapy did not seem to limit diffusion of the disease. CONCLUSIONS: Although EP and SPB are both localized forms of plasma cell dyscrasias, SPB seems to have a greater tendency to progression MM. It appears, however, that the apparent stronger propensity of SPB to progress is actually due to the great number of cases that at diagnosis conceal an occult MM.

Adult↗

The role of plasmacytoma growth factor in the in vitro responses of murine plasmacytoma cells.

Many plasmacytomas arising in BALB/c mice are dependent upon a specific, macrophage-derived plasmacytoma growth factor for survival and proliferation in vitro. Adherent cells taken from the peritoneal oil granuloma in which the early plasmacytomas arise and proliferate produce 50 times more PCT-GF activity in vitro than do normal peritoneal cells, thus suggesting a possible in vivo role for PCT-GF. Purification and amino acid sequencing of PCT-GF derived from the murine macrophage cell line, P388D1, have identified a 23 kDa protein with a unique NH2-terminal sequence. This molecule is now known as murine IL6. As part of the characterization of murine Il-6, genomic sequences have been localized to the proximal end of mouse chromosome 5 via Southern analysis of restriction fragment length polymorphisms. The removal of IL6 from IL6-dependent PCT cell lines results in a growth arrest in early G1. This is accompanied by a rapid and specific loss of transferrin receptor expression and results in eventual cell death. It appears that the response to IL6 is at least partially dependent on Ca++ because functional Ca++ channels are necessary for the PCT cells to pass through G1 and to maintain transferrin receptor expression.

Animals↗

Surface immunoglobulins of lymphocytes in plasmacytoma. V. The effect of RNA-rich extract from mouse plasmacytoma MOPC 104E on the immune response.

BALB/c mice with the plasmacytoma MOPC 104E producing monoclonal IgM-lambda with antibody activity to alpha-1,3 dextran were found to have B lymphocytes with surface immunoglobulins with the immunochemical characteristics of 104E IgM capable of binding alpha-1,3 dextran. RNA extracted from this plasmacytoma induced the synthesis of such surface immunoglobulins on normal B lymphocytes in vitro and in vivo. Injection of 200 mug of MOPC 104E RNA into normal mice 72 hr prior to the administration of the antigen kept the immune response to dextran-S intact, but suppressed that to other antigens, such as DNP-Ficoll and LPS, T cell-independent antigens, and SRBC and BSA which are T cell-dependent. The effect of the RNA was abolished by RNase but not by pronase and DNase. RNA extracted from LPC-1 tumour (gamma2a-k without known antibody activity) significantly suppressed the immune response to dextran-S and to other antigens in normal mice. Thus, opposite effects of MOPC 104E RNA on the response to specific and non-specific antigens strengthen the hypothesis that the immune deficiency in plasmacytoma bearing mice is due to the conversion of normal surface immunoglobulin of a population of B lymphocytes to the idiotype of the respective myeloma globulin.

Animals↗

Regulation of immune responses aginst the syngeneic ADJ-PC-5 plasmacytoma in BALB-c mice. III. Induction of specific T suppressor cells to the BALB/c plasmacytoma ADJ-PC-5 during early stages of tumorigenesis.

Initial stages of tumour growth are not easily accessible to investigation. Therefore an experimental procedure was developed to mimic tumorigenesis as closely as possible. BALB/c mice received intraperitoneally exponentially increasing numbers of irradiated syngeneic ADJ-PC-5 plasmacytoma cells. The initial injection began with two cells per mouse and according to the generation time of this tumour, subsequent doses were doubled until mice had received up to 10(5) tumour cells. At various stages of treatment, peritoneal exudate cells (PEC) and spleen cells (SC) were tested for either cytotoxicity or specific suppression of induction of a primary in vitro T-cell cytotoxic response (CTL) of BALB/c spleen cells against ADJ-PC-5 plasmacytoma cells. No cytotoxic PEC were found. Instead, PEC from mice in which the final tumour cells number had reached or exceeded 10(3) irradiated ADJ-PC-5 cells, induced complete suppression of this primary in vitro CTL. Specificity was found both in the induction and effector phase of suppression. Specific suppression was mediated by Thy-1.2+ cells and amplified by non-specific suppression through adherent cells. The data arae discussed in context with previous findings on the in vivo immunogenicity and tolerogenicity of the ADJ-PC-5 plasmacytoma. They suggest that induction of T suppressor (Ts) cells might be an early event in tumorigenesis.

Animals↗