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Primary pulmonary plasmacytoma with lobar consolidation: an unusual presentation.

Extramedullary plasmacytoma is a rare form of plasma cell tumor that frequently involves the upper respiratory tract. Primary pulmonary plasmacytoma is even more rare. The usual presentation of primary pulmonary plasmacytoma is a solitary pulmonary nodule. We describe the case of a 58-year-old woman who presented with the chief complaints of progressive dyspnea on exertion, cough, and subsequently, hemoptysis. The main finding on chest imaging studies, including plain radiography, sonography, computed tomography, and magnetic resonance imaging, was consolidation of the right middle lobe. Percutaneous transthoracic lung biopsy of the right middle lobe demonstrated sheets of atypical plasma cells. Immunohistochemical study showed IgA lambda monoclonality. A bone marrow study and whole body bone scan showed normal findings. To the best of our knowledge, this is the first reported case of primary pulmonary plasmacytoma presenting with lobar consolidation of the lung but without a well-defined tumor mass.

Biopsy, Needle↗

Extramedullary plasmacytoma invading the bladder: case report and review of the literature.

Multiple myeloma is characterized by neoplastic proliferation of a single clone of plasma cells engaged in the production of a monoclonal protein. This condition affects mainly the bone marrow, but extramedullary manifestations can be seen in any organ. Urinary bladder involvement is extremely rare, with only 14 cases reported in the literature to our knowledge. Herein, we report a large extramedullary bladder plasmacytoma arising in a patient with history of multiple myeloma. A 78-year-old woman with history of multiple myeloma, currently in remission, presented with a large intravesical tumor. Because the tumor was considered to have characteristics of anaplastic neoplasm from transitional cell origin with evidence of deep muscular invasion, a radical cystectomy was performed. A subsequent microscopic evaluation of the cystectomy specimen revealed round cells with an eccentric cartwheel-like nucleus suggestive of plasmacytoma. The diagnosis was further confirmed with immunohistochemical studies. It is difficult, according to the literature, to distinguish bladder plasmacytoma from anaplastic transitional cell tumors. It is important to provide the pathologist with an appropriate history and to have a high index of suspicion for bladder plasmacytoma in patients with previous diagnosis of multiple myeloma and bladder mass.

Abdomen↗

Bortezomib in relapsed multiple myeloma complicated by extramedullary plasmacytomas.

A 58-year-old man suffered relapse of multiple myeloma complicated by large intra-abdominal extramedullary plasmacytomas and severe hepatic impairment secondary to biliary obstruction. Previous treatment had included two types of combination chemotherapy and high-dose therapy with autologous stem cell transplant. Combination therapy with bortezomib and dexamethasone resulted in partial response of the plasmacytomas and complete resolution of his hepatic impairment. Extramedullary plasmacytomas are rare. This report lends support to the efficacy of bortezomib in the treatment of plasmacytoma and describes the safe use of bortezomib despite hepatic upset.

Antineoplastic Combined Chemotherapy Protocols↗

[Subglottic metastasis of multiple myeloma. Case report and review of the literature of laryngeal plasmacytoma].

A total of 111 cases of laryngeal plasmacytoma have now been reported. The disease may present either as a primary extramedullary plasmacytoma (n = 90) or as a metastasis of a multiple myeloma (n = 21). The distinction between these two types is very important in therapy and prognosis. We report a subglottic plasmacytoma in a 48-year-old woman. Investigations showed bone marrow infiltration, osteolysis and light chain expression in serum, indicating generalized disease. Therefore polychemotherapy was given, during which complete macroscopic and microscopic regression of the laryngeal plasmacytoma was observed. However, 14 months later, the patient died of renal failure due to systemic progression of the multiple myeloma.

Airway Obstruction↗

Multiple myeloma first observed as multiple cutaneous plasmacytomas.

A 62-year-old woman had multiple plasmacytomas of the skin with no roentgenographic or bone marrow evidence of multiple myeloma. Serum IgA-lambda level was increased to 1,000 mg/dL (normal range, 90 to 450 mg/dL). The skin lesions regressed completely when the patient was treated with melphalan. She had recurrence of a skin plasmacytoma and lytic bone lesions ten months later and died of bronchopneumonia 11 months after the was first seen. Solitary skin plasmacytomas are rare, and multiple skin plasmacytomas are even rarer. Occasionally, these lesions may precede roentgenographic and bone marrow evidence of multiple myeloma.

Female↗

Extramedullary plasmacytoma of the gingiva.

Extramedullary plasmacytoma is defined as neoplastic proliferation of plasma cells in soft tissue. It accounts for up to 3% of all plasma cell tumors. Approximately, 90% of extramedullary plasmacytomas are found in the head and neck region commonly affecting the nasal cavity, paranasal sinuses, tonsillar fossa, and oral cavity. Radiotherapy is the common modality of treatment with or without adjuvant chemotherapy. We report a case of extramedullary plasmacytoma of the gingiva.

Biopsy↗

Solitary extramedullary plasmacytoma in the retroperitoneum.

Solitary extramedullary plasmacytoma is an uncommon neoplasm and occurs most frequently in the upper respiratory tract. Herein, we reported a solitary extramedullary plasmacytoma in the retroperitoneum. A 28-year-old man presented with obstructive jaundice and a retroperitoneal tumor. Ultrasound-guided biopsy confirmed that the lesion was a plasma cell neoplasm. A detailed investigation showed that no other sites were involved. The tumor got a moderate reduction following local irradiation, and a complete remission was achieved after 12 courses of adjuvant chemotherapy. Therefore, the possibility of a solitary extramedullary plasmacytoma should be considered in the differential diagnosis of obstructive jaundice without a history of multiple myeloma.

Adult↗

Preleukemia in long-term plasmacytoma-regressor mice.

Our previous results have indicated that mice whose plasmacytoma regressed following curative melphalan chemotherapy manifested various persistent immunohematological abnormalities including immunosuppression, myeloproliferation, as well as excessive production of and response to growth factors. Mice not bearing plasmacytoma treated with an identical dose of melphalan chemotherapy did not exhibit such abnormalities. In the present study we show that plasmacytoma-regressor mice (PRM) contain preleukemic cells which do not progress to leukemia in these mice. However, adoptive transfer of splenocytes originating in PRM to preirradiated but otherwise untreated syngeneic recipients resulted in the development of overt leukemia in these recipients. The presence of leukemia in the primary recipient mice was ascertained by blood counts as well as by spleen histology. Furthermore, splenocytes from the irradiated primary recipients adoptively transferred to non-irradiated secondary recipients caused leukemia formation in 100% of the secondary recipients. Sex chromosome analysis of the leukemic cells in the irradiated primary recipients clearly showed that they originated in the PRM donors. Two leukemic lines were established from leukemias developing in the secondary recipients and both expressed surface markers of hematopoietic progenitor cells as well as markers of T cells. We suggest that PRM could serve as an animal model to investigate development of chemotherapy-related leukemia in humans.

Animals↗

Primary extramedullary esophageal plasmacytoma: First case report.

Solitary extramedullary plasmacytomas are rare tumors, reported to occur most commonly in the upper respiratory passages, but which also are rarely seen in the middle and lower gastrointestinal system. This report documents the first primary plasmacytoma of the esophagus. A 67-year-old Caucasian man complained of dysphagia and weight loss. Preoperative diagnosis, based on functional inquiry, radiology, and biopsy, was undifferentiated carcinoma involving the lower third of esophagus. The patient was treated by esophagogastrectomy. The pathology specimen revealed the typical histology of plasmacytoma. The extramedullary nature of the tumor was established postoperatively on the basis of the following criteria: 1) Absence of Bence-Jones proteinurea; 2) normal serum electrophoresis; 3) normal bone marrow biopsy; and 4) absence of distant metastases on liver scan and bone survey.

Aged↗

Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. IX. Plasmacytomas.

Plasmacytomas occasionally may arise in extraosseous sites, of which the upper respiratory tract is the most common. In our series of 256 nonepithelial tumors involving the nasal cavity, paranasal sinuses and nasopharynx, 10 (4%) were apparently primary extramedullary plasmacytomas. Of our patients with adequate follow-up information, four died of disseminated disease 2, 4, 5 and 6 years after the initial diagnosis. One was alive 6 years after diagnosis with locally persistent tumor. Another was living without recurrence 12 years later. Based on our findings and on the cases reported in the literature, extramedullary plasmacytomas arising in the head and neck area may evolve into one of several different patterns. 1) Some patients have localized disease which is apparently controlled (by surgery, radiotherapy or both) and which never recurs locally or becomes disseminated. 2) In some cases, the tumor recurs locally and is controlled by further therapy. 3) Other patients have a locally persistent and aggressive lesion which cannot be eradicated and which eventually leads to the patient's death by uncontrolled local growth. 4) Still other patients eventually develop evidence of plasma cell neoplasms elsewhere in the body and/or multiple myeloma.

Adult↗

Plasmacytoma of lymph nodes.

A case of primary plasmacytoma in a lymph node accompanied by IgG Kappa paraproteinemia is described. Eight months after the plasmacytoma's removal, the paraprotein was undetectable in the serum and the patient has remained well for a year. Abundant crystalline inclusions were present in the tumor and the immunoperoxidase technique was used to show that these consisted of monotypic immunoglobulin are were contained predominantly within macrophages. Reported cases of plasmacytoma in lymph nodes are reviewed and the natural history of this group of tumor is discussed.

Adult↗

Plasmacytoma of the colon: case report with lambda light chain, demonstrated by immunoperoxidase studies.

A case of solitary extramedullary plasmacytoma in the transverse colon of a 20-year-old woman is reported. This case is unusual in that the plasmacytomas are only rarely observed in the gastrointestinal tract and are especially uncommon in the colon and in young women. The lesion was composed of pleomorphic plasma cells and contained areas of extracellular amyloid deposition. Its neoplastic nature was verified by the use of immunoperoxidase studies which are positive only for lambda light chain. The subject of gastrointestinal plasmacytomas and the use of immunoperoxidase studies on plasmacytic lesions are discussed.

Adult↗

IgM-producing gastric plasmacytoma.

A case of gastric plasmacytoma is reported in an 81-year-old woman. Immunofluorescent and immunoelectrophoretic studies showed that the tumor cells produced IgM-lambda molecules, whereas no monoclonal immunoglobulin could be detected both in the serum and in the urine. Of more than 60 reported cases of gastric plasmacytoma, only a few reports referred to the production of monoclonal immunoglobulin by the tumor cells. This case is the first one of IgM-producing gastric plasmacytoma. Moreover, the produced IgM was found to be 7S-IgM. It is extremely rare that 7S-IgM alone is detected in the absence of 19S-IgM.

Aged↗

Plasmacytoma of the thyroid gland.

Primary plasmacytoma of the thyroid gland is a rare disease, and clinicopathologic features of this disease are not fully understood. Six cases of primary plasmacytoma of the thyroid, in which immunoperoxidase procedures confirmed a monoclonal nature of proliferating plasma cells are presented. All patients had antithyroid antibodies in their serum together with histologic evidence of chronic lymphocytic thyroiditis, suggesting an intimate relationship of these diseases. Review of the literature revealed that 18 cases, 14 cases from Western countries and 4 cases from Japan, described primary thyroid plasmacytoma. The authors summarized the clinicopathologic features in the current and previously reported cases.

Adult↗

Esthesioneuroblastoma masquerading as extramedullary plasmacytoma.

Nine years after biopsy and radiation therapy for an extramedullary plasmacytoma of the nasal cavity, a 64-year-old man developed locally recurrent tumor. Repeat biopsy revealed tumor identical in hematoxylin and eosin stain (H & E) appearance to that found originally, leading to the diagnosis of recurrent extramedullary plasmacytoma. Neither the original tumor or its recurrence demonstrated features characteristic of esthesioneuroblastoma (e.g., nests, rosettes, ganglion cells, or fibrillary background). However, Gremilus staining and neuron specific enolase tests (immunoperoxidase method), done strictly as a teaching exercise, were strongly positive and eventually resulted in an altered diagnosis to esthesioneuroblastoma. This case underscores the difficulty that may be encountered in distinguishing these two pathologic entities of the nasal cavity solely on the basis of H & E staining. In view of differences in staging, evaluation, prognosis, and management, accurate differentiation of these two relatively uncommon tumors of the nasal cavity is of immediate clinical importance. These observations suggest that care should be taken to exclude the possibility of esthesioneuroblastoma before making the histologic diagnosis of extramedullary plasmacytoma of the nasal cavity.

Diagnosis, Differential↗

Primary pulmonary plasmacytoma.

BACKGROUND: A patient was diagnosed with an extramedullary plasmacytoma of the lung after complete resection of the mass at thoracotomy. Immunoperoxidase staining of the mass revealed monoclonal lambda chains. Screening for multiple myeloma identified a small amount of M-protein in the blood, but no other evidence of multiple myeloma was found. METHODS: A literature search was conducted to determine the prognosis and the best way to manage the patient. RESULTS: Nineteen cases of primary pulmonary plasmacytoma were found in the literature. The age range was 3-79 years. Most of these cases were diagnosed at thoracotomy and treated by surgical excision. Immunohistochemical evaluation of the lesion is essential for diagnosis but was done in only three cases. CONCLUSIONS: Surgery and radiation therapy seem to be equally effective forms of treatment. The role of adjuvant chemotherapy is unknown. Local recurrences are rare. Follow-up data were inadequate to determine disease-free survival, progression to multiple myeloma, and overall survival in primary pulmonary plasmacytoma. Close follow-up is needed to detect progression.

Adolescent↗

Translocation remodeling in the primary BALB/c plasmacytoma TEPC 3610.

Myc-activating chromosomal 12;15 translocations, the hallmark mutations of inflammation-induced BALB/c plasmacytomas, have recently been shown to undergo remodeling by isotype switch-like genetic recombinations that remove approximately 180 kb of immunoglobulin heavy-chain sequence in the vicinity of the rearranged, expressed Myc gene. Here we combine cytogenetic data on the 12;15 translocation (SKY and FISH) with the molecular analysis of key junction sites (long-range PCR followed by DNA sequencing) to demonstrate that translocation remodeling occurred as an infrequent, stepwise, and disomic tumor progression event in the tetraploid, fully transformed, and transplantable plasmacytoma TEPC 3610. This result was used, in conjunction with previously obtained molecular data on five other primary plasmacytomas, to devise a hypothesis that predicts that the selective pressure to undergo translocation remodeling may be predetermined by the location of the break site in Myc. The pressure may be low if the break occurs 5' of the normal promoter region of Myc, but it may be considerably stronger if the break occurs 3' of the Myc promoter. Published 2001 Wiley-Liss, Inc.

Animals↗

Solitary intracranial plasmacytoma: two patients with extended follow-up.

Solitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow-Up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, neither showed involvement of the adjacent calvarium or evidence of a systemic plasma cell dyscrasia. The first patient underwent partial excision of a mass in the right occipital lobe that showed no gross or microscopic involvement of the dura. The second patient had partial resection of a mass arising from dura and invading the left temporal lobe. Postoperative irradiation was given to both patients. No clinical or radiological sign of recurrent brain tumor has developed in either patient during the extended follow-up period. Neither patient has had laboratory or clinical evidence of a systemic plasma cell dyscrasia. Solitary intracranial plasmacytoma may be a "curable" brain tumor.

Adult↗