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The management of myeloid and lymphoid disease is essentially based on chemotherapy and targeted therapies. Since radiotherapy could be responsible for severe late toxicities, essentially due to conventional bidimensional irradiation techniques, many trials have attempted to omit radiotherapy or to scale down the dose in their therapeutic strategy. Nevertheless, radiotherapy still plays a role for curative or symptomatic purposes., (Copyright © 2021 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Objective: To investigate the outcomes of radiotherapy with or without surgery during treatment of patients with solitary plasmacytoma of bone (SBP) of the spine., Methods: Patients diagnosed with SBP of the spine treated with radiotherapy with or without surgery were identified and extracted from the SEER database. Propensity score matched (PSM) analysis was performed to balance patient characteristics between radiotherapy alone and radiotherapy with surgery groups. Patients in different age-groups were stratified and analyzed., Results: A total of 1275 patients with SBP of the spine treated with radiotherapy with or without surgery were extracted from the SEER database. Before PSM, the unadjusted Kaplan-Meier curve showed that the radiotherapy with surgery group had worse overall survival than did the radiotherapy without surgery group (both P < 0.05), whereas the difference of overall survival was attenuated after PSM. Stratified analysis found that the radiotherapy with surgery group had less progression to multiple myeloma for young patients (age <45 years) with SBP of the spine than did the radiotherapy without surgery group., Conclusions: The results of our study suggest that radiotherapy with surgery may show less progression to multiple myeloma for younger patients with SBP of the spine., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: The primary objective was to determine in our department the progression-free survival rate of patients with solitary bone plasmocytoma and secondarily to evaluate its diagnostic, therapeutic and evolutionary aspects., Patients and Methods: This is a retrospective review of 12 patients monitored and treated in the radiotherapy department of the Mohammed-V military medical teaching hospital in Rabat for a solitary bone plasmocytoma between January 2012 and December 2018. The average age of our patients were 53.8 years old (range: 31-72 years old). Pain was the most common telltale sign. The site of the lesions was spinal in four cases, iliac in four cases, mandibular, ribal, humeral and at the level of the astragalus in one case respectively. All patients received radiotherapy. This irradiation was delivered alone in 60% of cases or associated with surgery in 40% of cases. The average dose of radiotherapy was 47.3Gy (range: 45 to 50.4Gy) and this was delivered by a modulated volumetric arc therapy technique in ten patients and conformal tridimensional radiotherapy in two patients., Results: Local control, defined by stability or radiological regression, was obtained in ten patients and four patients progressed to multiple myeloma, two of whom died. The average duration of follow-up was 51 months., Conclusion: Radiation therapy is the standard treatment for solitary bone plasmocytoma. It ensures good local control in 90% of cases. The prognosis is affected by progression to multiple myeloma, which justifies rigorous monitoring after treatment and suggests a reflection on the exact place of chemotherapy., (Copyright © 2019 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Purpose: To characterize local relapse after surgical fixation and postoperative radiotherapy (RT) for multiple myeloma (MM) with cortical involvement of long bones., Patients and Methods: We retrospectively identified patients with MM involving cortical long bones treated with surgical fixation followed by postoperative RT at our institution. Local failures, defined as radiographic recurrence along the surgical hardware, were documented, and potential associations of independent variables (RT dose, fractionation, and extent of hardware coverage) with local failure were assessed by univariate Cox regression., Results: We identified 33 patients with 40 treated sites with a median follow-up of 25.7 months; 68% of treatments were for pathologic fracture, and 32% were for impending fracture. The most common dose and fractionation were 20 to 25 Gy in 8 to 12 fractions. On average, 76% of the surgical hardware was covered by the postoperative RT field (median, 80%; range, 28%-100%). Local failure was observed in 5 cases (12.5%), 2 within the RT field and 3 out of field. None of the relapses resulted in hardware failure, and 2 were retreated with RT. The extent of hardware coverage predicted disease relapse along the hardware (hazard ratio = 6.44; 95% confidence interval, 1.09-37.97; P = .04); however, total RT dose, biologically effective dose, and number of fractions did not., Conclusion: After internal fixation of long bones with MM, full hardware coverage with the RT field could reduce the risk, though small, of disease developing in the future in the proximate hardware. Postoperative RT doses of 20 to 25 Gy in 8 to 10 fractions can achieve excellent local control., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Extramedullary plasmacytomas (EMP) are rare plasma cell neoplasms that are mostly located in the mucosal surfaces of the upper aerodigestive tract. The standard treatment is radiotherapy (RT), with surgical resection reserved as salvage procedure. In this report a patient with a EMP, located in the nasopharynx and refractory to curative RT. Because of the technical difficulty of a radical resection in the nasopharynx area the EMP is managed by endoscopic debulking and temoporfin mediated photodynamic therapy. The patient remains disease free since six years., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Crystal-storing histiocytosis (CSH) is a rare consequence of abnormal accumulation of immunoglobulins which may arise in a number of different clinical scenarios. In this report, we describe the case of a male patient who presented with an apparently innocuous lesion on the dorsum of tongue which showed the typical features of CSH. Subsequent investigations revealed an associated plasmacytoma, and the patient developed further systemic lesions. The rarity of such lesions presents diagnostic difficulties, yet accurate diagnosis underpins the timely implementation of appropriate therapy., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Purpose: To assess the outcome and the management of solitary extramedullary plasmocytoma in the head and neck region., Patients and Methods: From 1997 to 2008, five cases of solitary extramedullary plasmocytoma were treated in the department of radiotherapy at Salah-Azaiz Institute. Three patients had a solitary plasmocytoma of the nasal fossa; the others were ethmoidal and submandibular node. All patients received irradiation of 40 to 45 Gy in the primary site associated to surgery in four cases. Among the five patients, two had radiotherapy after recurrence., Results: Four complete responses were noted with a follow-up of 12, 36, 52 and 72 months. Multiple myeloma occurred in one patient 8 years after treatment., Conclusion: Radiotherapy is the best effective local treatment. Local control of extramedullary plasmocytoma in the head and neck region seems to be improved when the dose is at least 45 Gy. Predictive parameters of unfavourable outcome and conversion of extramedullary plasmocytoma to multiple myeloma should be better defined., (Copyright © 2010 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Plasmacytoma of the bronchus is a very rare plasma cell neoplasm affecting the bronchus. Here we report a case of plasmacytoma of the bronchus treated by radical radiotherapy in July 2002. The tumor responded very well to treatment and showed a slow but sustained regression in the size over two years. Presently, he has completed four and a half years of follow-up and is free of disease.

This is the case report of a 67-year-old man who developed a solitary lytic lesion of the proximal humerus. Through further studies that included MR imaging of the affected upper extremity and biopsy, a final diagnosis of plasmacytoma was given. We present the clinical, imaging, and histologic findings and review the general characteristics of this neoplastic condition.

Purpose: To present experience with high dose-rate endobronchial brachytherapy in the treatment of primary tracheal tumors., Patients and Methods: Four patients with nonresected primary tracheal tumors are presented: 2 cases of squamous cell carcinoma of the trachea, 1 of recurrent adenoid cystic carcinoma, and 1 with recurrent plasmacytoma. All received brachytherapy, alone or as a boost for primary irradiation, in 3 or 4 fractions of 7.5 Gy, calculated at a depth of 1 cm. Follow-up was considered to start from the end of brachytherapy., Results: Local control was achieved in all cases at the time of first bronchoscopic evaluation. Two patients with squamous cell carcinoma died at 6th and 33rd months after brachytherapy, respectively. The first had no evidence of disease, and the latter had local recurrence. The other 2 patients were alive after 64 and 110 months of follow-up, respectively, both with no evidence of disease. Tracheal stenosis developed in these 2 cases, 22 and 69 months after brachytherapy. Tracheal stent placement was needed only for the patient with an adenoid cystic carcinoma., Conclusions: Endobronchial high dose-rate brachytherapy may be used for tracheal tumors, even as a boost for external beam irradiation, or in recurrences. Local control in 3 out of 4 patients indicates that individual cases may benefit from the treatment. Long-term survival may also be expected, mainly for tumors with adenoid cystic histology.

Purpose: - To describe natural history of solitary bone plasmocytomas (SBO) after treatment., Patients and Methods: - Between 1975 and 1998, we retrospectively identified 13 patients with SBO treated in the department of radiotherapy at Salah-Azaïz institute of Tunis. To be included in this study, three criteria were needed: histologic confirmation and only one bone lesion without medullary infiltration (or less than 10%). Mean age was 43 years (22-64) with sex-ratio of 3,3. Tumor sites were vertebra (6), flat bones (6) and tibia (1). Megavoltage radiotherapy was given to all patients, associated to surgery in eight cases (5 excisions and 3 laminectomies) and to chemotherapy in three., Results: - With a mean follow-up of 63 months, two local failures were noted at 24 and 48 months. Two patients developed multifocal lesions and one patient had an extramedullary lesion. Multiple myeloma occurred in four patients (30%) after a median follow-up 3,5 years. Unfavorable outcome could not be predicted by age, gender or site of disease but by myeloma protein., Conclusion: - Radiotherapy is an effective local treatment for solitary bone plasmocytoma. Prospective studies are needed to better define predictive parameters of unfavourable outcome and indications of combined chemotherapy.

Castleman's disease is a rare disorder characterized by lymphoid hyperplasia. It may present as asymptomatic involvement of one lymph node group or as a multicentric disease with systemic features. We report a patient with Castleman's disease who presented with axillary lymphadenopathy associated with a solitary plasmacytoma originating from a rib. The affected rib was surgically resected and radical radiotherapy was subsequently administered to the axillary lymph nodes. In this particular case, a joint surgical and oncologic approach resulted in a successful outcome.

Angiogenesis plays an important role in the biology of multiple myeloma (MM) and has prognostic importance in this disease. Solitary plasmacytoma is a localized plasma cell malignancy that progresses to MM in a significant number of patients. We examined if angiogenesis is increased in solitary plasmacytoma and if it can help identify patients likely to progress to myeloma. We studied angiogenesis in plasmacytoma biopsy samples and bone marrow biopsies from 25 patients. High-grade angiogenesis was present in 64% of plasmacytomas. In contrast, bone marrow angiogenesis was low in all patients. Patients with high-grade angiogenesis in the plasmacytoma sample were more likely to progress to myeloma and had a shorter progression-free survival compared with patients with low-grade angiogenesis (P =.02). Angiogenesis is increased in solitary plasmacytoma and is a significant predictor of progression to myeloma and provides further evidence of its importance in the pathogenesis of myeloma.

Most patients with multiple myeloma (MM) present with symptoms, have evidence of generalized disease, and require chemotherapy promptly to reduce the malignant clone. Some patients present with a local symptom from a single plasmacytoma but no myeloma elsewhere. Such patients usually become free of symptoms after local radiotherapy. In patients with MM without symptoms, the diagnosis is made on the basis of screening laboratory tests. In patients with either solitary plasmacytoma of bone or asymptomatic MM, systemic treatment should be deferred until there is evidence of disease progression.

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.

We report two cases of solitary plasmacytoma of the skull, a very unusual presentation and location of this tumor. There seems to be no difference in prognosis between the tumor originating from the skull (osseous form) and from the dura mater (non-osseous form), differently from the data of other parts of the body. The risk of secondary multiple myeloma appears to be low but the mean follow-up is too short for any conclusion on this point.

A case of kappa light chain multiple myeloma with space-occupying hepatic plasmacytoma is presented. Magnetic resonance (MR) imaging shows an eccentric multiple-layer pattern of the liver mass and suggests malignancy. Differential diagnosis concerning the MR appearance of this rare hepatic tumor is discussed.

The P.O.E.M.S. syndrome is a rare clinical entity that has been described mainly in Japan. It is characterized by a progressive polyneuropathy with raised CSF protein content, organomegaly, endocrinopathy, skin changes and plasma cell dyscrasia. We report a new documented case associated with a solitary plasmocytoma of the 12th thoracic vertebra. Immunopathological and ultrastructural studies of the peripheral nerve did not disclose any immune-specific changes. Surgery and radiation therapy of the plasmocytoma allowed a complete recovery, with a 5-year follow-up. This case report illustrates the need for serial full skeletal survey, including scintigraphy, in middle-aged patients with progressive polyneuropathy of obscure origin.

An 81-year-old man presented with bilateral orbital tumors that were found to be extramedullary plasmacytomas. Immunoperoxidase staining of the tissue was positive for IgG-kappa; no lambda chains were seen. Results of serum immunoelectrophoresis were positive for monoclonal IgG-kappa. Test results of the urine were positive for Bence Jones protein; only kappa chains were seen on radial diffusion. Systemic evaluation failed to show tumor elsewhere. The patient was treated with radiation and low-dose melphalan/prednisone, with good response. Five years later, the results of immunoelectrophoresis are normal and there is no clinical evidence of tumor; however, a small amount of kappa light chains remains in the urine. This case supports the important therapeutic and prognostic differences among orbital involvement in extramedullary plasmacytoma, solitary myeloma of bone, and multiple myeloma.

A 58-year-old man with multiple myeloma presented with a 3-month history of a sensation of occipital pressure, transient blurring of vision and increased bone pain. Funduscopic examination revealed disc swelling, tortuous veins and superficial retinal hemorrhages simulating early hyperviscosity syndrome. Clinical investigation and computerized tomography, however, indicated that an intracranial plasmacytoma was the cause of his papilledema. Cobalt 60 beam therapy and vincristine were added to his treatment regimen. Seven weeks later the intracranial tumour had almost totally disappeared. Symptomatic cerebral compression from an intracranial plasmacytoma associated with systemic disease is uncommon. The diagnosis and management are reviewed.

A 48-year-old man developed a plasmacytoma in the subcutaneous pocket of a titanium covered pulse generator that had been inserted sixteen months previously. The tumor represented a cutaneous manifestation of myelomatosis. The presence of a pacemaker may affect the detection and treatment of tumors arising in the region of pulse generator pockets. Possible cause-and-effect relationships are explored.

We describe a 68-year-old patient with a plasma cell leukaemia in haematological remission presenting with massive intracranial leptomeningeal plasmocytic infiltration (LPI) and hydrocephalus. He was treated with skull irradiation and a combination of intraventricular and lumbar intrathecal therapy with methotrexate. Neurologic improvement and clearance of plasma cells from the cerebrospinal fluid was reached after 2 weeks of treatment but prolonged follow-up was interrupted by a lethal gastro-intestinal haemorrhage, 6 weeks after starting the therapy. From previously reported cases it is known that LPI almost always occurs in either high-grade plasmocytomas or plasma cell leukaemia. These data suggest that therapy of LPI should be the same as in other leukaemias with leptomeningeal infiltration.

A polysaccharide preparation isolated from Coriolus versicolor (Fr.) Quél. of Basidiomycetes (PSK) predominantly consists of glucan and approximately 25% tightly bound protein. PSK was effective against various allogeneic and syngeneic animal tumors and has been given orally to cancer patients. Various suppressed or enhanced immune responses of tumor-bearing animals were restored to normal levels by the administration of PSK in the tumor models tested. The killer T cell activity was augmented in tumor-bearing mice by intraperitoneal or oral administration of PSK, and there was correlation between the PSK associated antitumor effect and the killer T cell activity. It was found that PSK competed with immunosuppressive substances isolated from tumor-bearing mice and that the intestinal immune system appeared to be modulated by oral administration of PSK. After oral administration of 14C- or 35S-labeled PSK to normal rats, it was found that small or large molecular substances appeared in the serum depending on the time elapsed after administration, an indication that large molecular size products were from the digestive tract.