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[Primary extramedullary plasmacytomas of the upper respiratory tract. A study of four cases with infrequent localization].

OBJECTIVE: Assessment of the incidence, diagnosis and treatment of three patients afflicted of extramedullary plasmacytoma located in the Waldeyer's ring, and one case afflicted of maxillary sinus disease. PATIENTS: The localization of the masses was the palatine amygdala in two cases, and the rhinopharynx and maxillary sinus in the remaining two patients. Major symptoms were dysphagia respiratory failure with closed rhinolalia, and headache. Diagnosis was made by histological study of biopsied tissue. In every case, general illness or other site of localization were ruled out. RESULTS: In our casuistic, the incidence of primary extramedullary plasmacytoma in Waldeyer's ring is 1.2%. The patient's age were 45, 50, 62, and 73 years respectively, and the male/female ratio was 1. In those cases located in the tonsils, a bilateral tonsillectomy was performed. In the case located at the maxillary sinus, a sinus trepanation was performed according the Caldwell-Luc's technique, followed by local radiotherapy at doses of 40 Gy. In the case located at the rhinopharynx, radiotherapy was the only choice. These three different therapeutical procedures led to the disappearance of the disease, and, after a 12 to 53 month-period, patients remain asymptomatic. A patient died due to other causes not related to the illness.

Aged↗

Primary gastric plasmacytoma of rapid growth presenting with upper gastrointestinal bleeding.

We report a new case of primary gastric plasmacytoma. The presenting symptoms was upper gastrointestinal bleeding, and the diagnosis was made preoperatively by means of gastroscopically obtained biopsy material. The demonstration of a single type of IgA-kappa immunoglobulin in plasma cells infiltrating the gastric antrum is considered conclusive evidence of its neoplastic nature. Endoscopically, the gastric plasmacytoma progresses from a benign antral ulcer (0.5 cm in diameter) to an ulcerated mass (7 cm in diameter) in an 8-wk period, suggesting rapid tumoral growth of a kind not described in the revised literature.

Biopsy↗

Metastatic extramedullary plasmacytoma of the colon and rectum in a dog.

Stage-3 extramedullary plasmacytoma of the large intestine was diagnosed in an 8-year-old Labrador Retriever. Three primary tumors were located in the colon and rectum, with metastasis to local lymph nodes and the spleen. The disease was associated with a monoclonal serum protein spike identified as IgG. Treatment consisted of surgical excision followed by chemotherapy, using melphalan and prednisone. The dog remained free from clinical signs of disease and adverse effects of the chemotherapy at 9 months. Findings in this dog indicated that extramedullary plasmacytoma may be an aggressive disease, associated with spread to distant sites and monoclonal gammopathy.

Animals↗

Fine needle aspiration diagnosis of orbital plasmacytoma with amyloidosis. A case report.

Fine needle aspiration (FNA) cytology of the orbit was used to diagnose an unusual case of multiple myeloma with secondary amyloid deposition in a 27-year-old man who presented with bilateral keratoconjunctivitis and left-sided ptosis. The FNA smears revealed immature plasma cells, extracellular eosinophilic material (amyloid) and numerous multinucleate giant cells. Subsequent histologic studies of excised tissue confirmed the cytologic diagnosis of plasmacytoma with secondary amyloidosis. Amyloid was further confirmed by electron microscopy. The patient was subsequently diagnosed as having multiple myeloma. The cytologic features of amyloidosis and plasmacytoma are presented. The differential diagnosis and the diagnostic utility of FNA cytology in the evaluation of these orbital lesions are also discussed. This apparently was the first case of orbital amyloidosis diagnosed by FNA cytology.

Adult↗

Solitary plasmacytoma of bone.

From 1978 to 1992, 19 patients were diagnosed with solitary plasmacytoma of bone (SPB) accounting for 5.5% of plasma cell neoplasms in Chang Gung Memorial Hospital. Fourteen were male and five were female, with ages ranging from 21 to 71 years (median, 53 years). M-protein was detected at diagnosis in five patients, and during follow-up in three. The most common sites of SPB were the vertebral bodies, with seven involving the thoracic, four involving the lumbar and two involving the cervical spine. The remaining sites included the skull in four, and femur and pelvis in one each. Fifteen of the patients received local radiotherapy, local control was achieved in all; nine patients were alive with no evidence of disease for seven to 77 months, two patients remained in apparent remission at 14 and 92 months, two patients progressed to multiple myeloma at 12 and 84 months, one patient developed extramedullary plasmacytoma at three months, and one patient died of intercurrent disease at four months. The four patients who were not treated with radiation therapy ultimately developed local recurrence or dissemination within four years. In our series, local recurrence or dissemination was always associated with the appearance of, or an increase in, the M-component; thus, measurement of M-protein is helpful in assessing tumor control or progression. Based on our experience and a review of the literature, it is recommended that irradiation of the primary sites is the treatment of choice. Chemotherapy is indicated in those patients with disease progression, recurrence, or dissemination.

Adult↗

Solitary plasmacytoma of the rectum: report of a case treated by endoscopic polypectomy and radiotherapy.

Solitary extramedullary plasmacytoma (EMP) of the rectum is an exceedingly rare entity. We report the fourth case of solitary EMP of the rectum and the first one treated by endoscopic polypectomy and radiotherapy. The patient showed no signs of local recurrence or dissemination of the disease after a 12-month follow-up. This case report is presented along with a review of the literature dealing with solitary plasmacytomas of the rectum.

Aged↗

An unusual case of metastatic intracardiac plasmacytoma.

The authors report the case of a 36-year-old male who, following investigations for dyspnea, othopnea and peripheral cyanosis, was found to have metastatic intracardiac plasmacytoma. Diagnosis was made initially with transesophageal echocardiography and emergent cardiac surgery was performed. Postoperative transthoracic and transesophageal echocardiograms revealed residual masses in the right and left atrium. The patient was subsequently treated with systemic chemotherapy and was symptom-free within three months of treatment, with rapid decline of paraproteins in the serum and urine. This case of intracardiac metastatic plasmacytoma is deemed unique because cardiac involvement with multiple myeloma has not been studied in detail, largely due to the rarity of such a presentation.

Adult↗

The role of glutathione (GSH) in determining sensitivity to platinum drugs in vivo in platinum-sensitive and -resistant murine leukaemia and plasmacytoma and human ovarian carcinoma xenografts.

Numerous studies performed in vitro have suggested a role for glutathione (GSH) in determining the sensitivity/resistance of tumour cells to various platinum-based drugs. Few studies have extended these findings into the in vivo setting. We have measured GSH levels in two murine (ADJ/PC6 plasmacytoma and L1210 leukaemia and their acquired platinum-drug-resistant sublines) and five human ovarian carcinoma (PXN/100, PXN/109T/C, SKOV3, HX/62 and OVCAR-3) tumour models of varying sensitivity to cisplatin. Results showed that relatively high GSH levels may be involved, at least partially, in determining platinum drug resistance in vivo in at least some of the tumour models studied (ADJ/PC6 carboplatin and tetraplatin resistant tumours; L1210 cisplatin and tetraplatin resistant tumours and the HX/62 and OVCAR-3 human ovarian carcinoma xenografts). However, in other tumours (e.g., the acquired cisplatin resistant ADJ/PC6 plasmacytoma) non-GSH mediated mechanisms of resistance (such as enhanced DNA repair) probably account for the resistance. Pretreatment of animals with oral buthionine sulfoximine (BSO), which resulted in approximately 70% depletion in tumour GSH levels, failed to potentiate the antitumour efficacy of either cisplatin (using the ADJ/PC6 and L1210 models) or the 1,2-diaminocyclohexane (DACH) platinum-drug, tetraplatin (using the ADJ/PC6 model). These BSO plus or minus cisplatin data suggest a limited role for such combinations in the clinic.

Animals↗

Solitary bone plasmacytoma terminally developed myeloma with multiple extramedullary lesions and myelomatous pleural effusion and ascites.

A rare case of IgG-kappa type multiple myeloma associated with multiple extramedullary tumor-forming lesions and myelomatous pleural effusion and ascites is reported. The patient suffered from sternal solitary plasmacytoma, which 5 years later developed disseminated tumor formation with myeloma cells mostly in the soft tissues. At autopsy, multiple extramedullary lesions particularly in the abdominal cavity and myelomatous pleural effusion and ascites were observed without diffuse infiltration of myeloma cells in the bone marrow. The disseminating change from solitary bone plasmacytoma to extramedullary tumor formation seen in this case seemed to be unusual but suggestive of a feature of an aggressive tendency of multiple myeloma.

Abdominal Neoplasms↗

A malignant nasal polyp. A clinically surprising plasmacytoma.

In conclusion, this is a study of a 52-year-old white male who had the removal of a benign appearing nasal polyp with the surprising diagnosis of extramedullary plasmacytoma. The clinical course has been completely benign since surgery for over two years. This should alert physicians to the possibility that benign appearing polyps, on rare occasion, can be malignant, and particularly those polyps occurring on the septum should be carefully investigated. It happened that this patient had a plasmacytoma, and a search for the presence of multiple myeloma is warranted.

Diagnosis, Differential↗

Murine plasma cells secreting more than one class of immunoglobulin heavy chain. I. Frequency of two or more M-components in ascitic fluids from 788 primary plasmacytomas.

Primary ascites from 778 consecutive plasmacytomas of BALB/c and other mouse strains were tested by immunoelectrophoresis for the presence of paraproteins belonging to different immunoglobulin classes. gammaA was produced by 338 tumors: gamma Gs (gamma 1, gamma 2a, gamma 2b, or gamma 3) by 163 tumors; and gamma M by seven tumors. Free kappa- or lambda-typed light chains were produced by 14 tumors, and no detectable paraprotein was produced by 202 tumors. Two or more paraproteins were detected in the primary ascites of 54 plasmacytomas. Evidence suggests that in most cases, the multiple paraproteins are produced by simultaneously occurring, independent plasma cell tumors. However, two tumors, SAMM 368 and TEPC 609, produced two paraproteins for over 30 generations in transplantation. This finding implies that single clones of plasma cells have the capacity to produce more than one class of immunoglobulin.

Animals↗

[Giant plasmacytoma of the skull which appeared in the clinical course of multiple myeloma--a case report].

A 51-year-old woman with large plasmacytoma occurring from the temporal bone is presented. She has a history of multiple myeloma for 9 years. She manifested marked swelling in the left temporal area with tenderness. Neurological examination revealed no abnormality. She showed monoclonal free light chain (lambda type) in the serum and urine, and had multiple osteolytic lesions in her general bones. T1 WI of MRI exhibited a huge mass showing slightly high intensity in the left middle fossa and infratemporal fossa, and a part of the mass protruded into the extracranial space. The mass was markedly enhanced by Gd-DTPA. Angiography showed a hypervascular mass supplied by the external carotid artery. Biopsy disclosed plasmacytoma. She underwent local irradiation of 30 Gy and chemotherapy of Ranimustine (100 mg) and Cyclophosphamide (400 mg). The tumor reduced its size, and tenderness in her temporal area disappeared.

Cerebral Angiography↗

Alpha-chain disease with localized plasmacytoma of the intestine. Immunoperoxidase study.

An ileocaecal tumour in a patient with alpha-chain disease in remission was studied immunohistochemically by a quantitive immunoperoxidase method. The tumour which was histologically shown to be a plasmacytoma consisted of 68% alpha cells and 15% lambda cells; no kappa cells were found. Away from the tumour the pattern of immunoglobulin-producing cells was normal. It is concluded that the abnormal cell causing alpha-chain disease remains inactive and that the patient developed a plasmacytoma from a different cell clone.

Adult↗

[A case of retroperitoneal extramedullary plasmacytoma].

Extramedullary plasmacytoma (EMP) is a very rare disease and mainly arises in the head and neck area. We herein reported a case of EMP arising in the retroperitoneal space. A 46-year-old man was referred to our outpatient clinic in November 1989 with the complaint of flank pain on the left side. Radiological examinations showed a tumor formation in the retroperitoneal space, which involved the left kidney, spleen and pancreas. Immunoelectrophoresis showed an elevation of serum IgG level and a spike of M-protein was detected in the serum protein electrophoresis. No bone lesions were detected, and bone marrow aspiration showed no abnormal cells. US-guided needle biopsy of the tumor led to the histological diagnosis as plasmacytoma of the IgG-kappa type. Following three cycles of preoperative chemotherapy (a THP-COP regimen), which resulted in a size reduction of the tumor by 40%, extensive resection of the tumor including extirpation of the left kidney, spleen, and tail of pancreas was performed. Because of tumor extension into the posterior wall of the stomach, however, the surgery resulted in incomplete resection. A total of 11 cycles of postoperative chemotherapy (THP-COP) was performed periodically for the residual tumor in the stomach. Rapid tumor spreading in addition to re-elevation of the serum IgG level, however, developed after the 11th postoperative chemotherapy, which extensively involved the stomach and intestines. The patient died of the disease 33 months after the initiation of treatment.

Humans↗

Establishment of a CD4-positive plasmacytoma cell line (AMO1).

A human plasmacytoma cell line (AMO1) was established. The AMO1 cells had the light and electron microscopic characteristics typical of plasmacytoma cells and did not harbor Epstein-Barr virus. These cells expressed cytoplasmic immunoglobulin A kappa and the immunoglobulin heavy-chain gene (JH) and kappa light-chain gene (C kappa) were rearranged. Coexpression of a CD4 antigen and plasma cell antigens (CD38 and PCA-1) was an unusual and sustained feature. Neither the T-cell receptor beta nor the gamma chain gene displayed the rearranged form. Other lineage-specific surface antigens, namely T, B, monocytoid, and myeloid antigens, were all negative in AMO1. In accordance with the surface CD4 expression, polymerase chain reaction analysis indicated constitutive expression of CD4 mRNA, and the cytogenetic findings revealed that AMO1 cells had a derivative chromosome 12, which had a structural abnormality of the short arm carrying the CD4 gene locus. These findings provide strong evidence for the presence of CD4-positive malignant plasma cells and raise the possibility that the CD4 expression in the AMO1 cell line is closely associated with the derivative chromosome.

Biomarkers, Tumor↗

[A case of solitary plasmacytoma that originated in a rib].

A 52-year-old woman was admitted to our hospital with the chief complaint of left anterior chest pain. A chest X-ray showed a tumor shadow in the left 4th rib. No abnormalities were detected in peripheral blood and aspirated bone marrow samples. The tumor was surgically resected with the involved bone, the intercostal muscle and the parietal pleura. The resected specimen contained a 3 x 6 x 3 cm lesion, and histological investigation of the tumor revealed a plasmacytoma of the rib. It relapsed as a multiple myeloma within two months after surgery. Chemotherapy was administered and the bone lesions were observed to disappear. We believe that the best way to treat solitary plasmacytoma is to first resect the solitary tumor, and then to add cobalt irradiation and chemotherapy after surgery.

Antineoplastic Combined Chemotherapy Protocols↗

[Pulmonary plasmacytoma. Apropos of a case. Review of the literature].

A 65 old man had a solitary primary pulmonary plasmacytoma. Serum immunoelectrophoresis revealed an immunoglobulin G Lambda M-protein. Screening for multiple myeloma with bone marrow biopsy and skeletal survey were negative. The patient underwent left lower lobectomy associated with resection of mediastinal lymph nodes. Immunoperoxydase staining of the mass revealed a monoclonal plasma cell population producing IgG, Lambda chains. No adjuvant chemotherapy or radiation therapy was performed. In few weeks, the paraprotein durably disappeared. Four years later, the patient is well and has no evidence of recurrence or myelomatosis. Authors emphasize the rarity of this tumor. The best treatment of pulmonary plasmacytoma is discussed.

Aged↗