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Importance: There are significant gaps in the literature pertaining to the locoregional control and survival rates of extramedullary plasmacytoma (EMP) with respect to various treatment approaches., Objective: To systematically evaluate the differences in radiotherapy and surgical outcomes in EMP., Data Sources: Databases including PubMed, Scopus, Web of Science, Embase, and ScienceDirect were systematically searched from their inception up to November 2023., Study Selection: Articles reporting radiotherapy and surgical outcomes of head and neck EMP were included., Data Extraction and Synthesis: A random-effects model for meta-analysis was used to obtain pooled estimates and calculate hazard ratios for survival and odds ratios for recurrence and progression of EMP., Main Outcomes and Measures: Survival, tumor control, and progression rates to multiple myeloma (MM) between radiation therapy and surgery for EMP of the head and neck., Results: Of 742 included patients from 12 studies, 527 (71.0%) were male, and the median (IQR) age was 59.1 (53-62) years. A total of 505 patients (68.1%) received radiotherapy only, while 237 (31.9%) underwent surgery-only treatment for EMP. All included patients had an initial diagnosis of EMP without MM. Comparable trends were observed in overall survival and disease-free survival (DFS) rates at 2, 3, 5, and 10 years between patients with EMP treated with radiotherapy only and surgery only. Notably, there were no significant differences in recurrence rate (odds ratio, 0.65; 95% CI, 0.20-2.06) between radiotherapy-only and surgery-only treatment. However, radiotherapy-only treatment of EMP was associated with decreased odds of progression to MM compared with surgery (odds ratio, 0.4; 95% CI, 0.1-0.9). Sensitivity analysis revealed that the radiotherapy-only population had significantly better 5-year DFS (hazard ratio, 0.55; 95% CI, 0.31-0.96) compared with surgery-only treatment., Conclusions and Relevance: This systematic review and meta-analysis provides evidence that patients with EMP receiving radiotherapy had significantly lower chances of progression to MM compared with surgery-only therapy. Additionally, radiotherapy had better 5-year DFS outcomes compared with surgery. Comparable outcomes in terms of overall survival rates, recurrence, and mortality rates were noted between radiotherapy-only and surgery-only EMP treatment groups.

A 72-year-old male patient, who had been on chemotherapy for the treatment of IgG-λ multiple myeloma, presented an enlargement of the testis 3 years and 5 months after the diagnosis. High orchiectomy was then performed, leading to the diagnosis of plasmacytoma. Due to residual disease, treatment with a combination of isatuximab and dexamethasone was initiated. The patient is currently under follow-up without recurrence. While testicular tumors are difficult to diagnose by imaging studies alone and extramedullary plasmacytomas rarely occur in the testis, pathological assessment is critical for treatment planning.

Rationale: Plasmacytoma is a disease caused by the proliferation of monoclonal immunoglobulin-producing plasma cells and divided into multiple myeloma, solitary bone plasmacytoma, and extramedullary plasmacytoma. We report a case of orbital extramedullary plasmacytoma invading the dura mater in a patient with exophthalmos and diplopia., Patient Concerns: A 35-year-old female patient with exophthalmos in the right eye and diplopia visited the clinic., Diagnoses: The thyroid function tests showed nonspecific results. Orbital computed tomography and magnetic resonance imaging revealed a homogeneously enhancing orbital mass infiltrating the right maxillary sinus and adjacent brain tissue in the middle cranial fossa through the superior orbital fissure., Interventions: To diagnose and alleviate the symptoms, an excisional biopsy was performed, which revealed a plasmacytoma., Outcomes: One month after the surgery, the protruding symptoms and eye movement restriction in the right eye improved, and the visual acuity in the right eye was restored., Lessons: In this case report, we present a case of an extramedullary plasmacytoma that originated in the inferior wall of the orbit and invaded the cranial cavity. To our knowledge, no previous reports have described a solitary plasmacytoma that originated in the orbit, causing exophthalmos and invading the cranial cavity at the same time., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Extramedullary plasmacytoma (EMP) is a benign round cell tumor that is most commonly found in cutaneous locations in dogs and occurs less frequently in the oral cavity. They are highly radiosensitive, are distinct from systemic multiple myeloma syndrome and wide surgical excision is typically curative. This report describes five cases of non-invasive oral EMP in dogs treated with a combination of marginal excision and strontium-90 plesiotherapy. All five cases had narrow or incomplete margins on histopathologic evaluation but experienced no recurrence after combination therapy. Plesiotherapy radiation may offer a potential adjunct treatment for non-invasive oral EMP by providing a superficial dose of radiation that complements a less invasive surgical removal. The combination of plesiotherapy and marginal excision may offer an alternative to wide surgical excision for non-invasive oral EMPs.

Extramedullary plasmacytoma (EMP) can rarely occur in conjunction with multiple myeloma (MM). EMPs are usually detected in the upper aerodigestive tract (UAD) but can also occur along the digestive tract. However, the involvement of gallbladder is uncommon. Gastrointestinal tract symptoms often lead to the diagnosis of EMP in the gallbladder. An 81-year-old man was referred to our hospital with suspected primary gallbladder carcinoma. He was subsequently operated on, and the pathological findings showed EMP of the gallbladder without MM.

Extramedullary plasma cell tumor (EMP) is a kind of plasma cell tumor, and its pathogenesis is not completely clear. According to whether it is independent of myeloma disease, it can be divided into primary and secondary EMP, which have different biological and clinical characteristics. Primary EMP has low invasion, fewer cytogenetic and molecular genetic abnormalities and good prognosis, and surgery and / or radiotherapy are the mainly treatments. Secondary EMP, as the extramedullary invasive progression of multiple myeloma (MM), is often accompanied by high-risk cellular and molecular genetic abnormalities and poor prognosis, chemotherapy, immunotherapy and hematopoietic stem cell transplantation are the mainly treatment. This paper reviews the latest research progress of EMP in the pathogenesis, cytogenetics molecular genetics and treatment, so as to provide reference for clinical work.

Rationale: Plasmacytoma is a rare plasma cell dyscrasia that grows within the axial skeleton or soft tissue structures as solitary or multiple masses. The primary types are solitary plasmacytoma, including solitary bone plasmacytoma (SBP) and solitary extramedullary plasmacytoma, and multiple solitary plasmacytomas. SBP is characterized by localized proliferation of monoclonal plasma cells and is rare. However, SBP with chronic osteomyelitis is even rarer., Patient Concerns: A 47-year-old man previously diagnosed with chronic osteomyelitis presented with repeated discharge and ulceration in the front of his right tibia., Diagnosis, Interventions and Outcomes: Lower extremity magnetic resonance imaging (MRI) and computed tomography (CT) examinations showed dead bone formation and surrounding inflammatory edema. Thus, the patient underwent dead bone excision and fenestration of the bone marrow cavity. The histopathologic examination results indicated plasmacytoma. Therefore, we administered radiotherapy with satisfactory results., Lessons: Physicians should pay close attention to chronic osteomyelitis because it may be accompanied by plasmacytoma. Postoperative pathological and immunohistochemical examinations are crucial, and surgical resection of the lesion and local radiotherapy are effective treatment methods., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Central airway obstruction due to tracheal tumors presents unique challenges to the anesthesiologist. We present the case of a 44-year-old male taken to the OR for biopsy and resection of an undiagnosed tracheal mass. Intraoperative management was complicated by bleeding and significant hemodynamic instability, necessitating rapid surgical and anesthetic intervention. This ultimately led to abortion of surgical resection. Pathologic examination revealed a primary tracheal plasmacytoma, a rare type of tracheal tumor. Here, we describe anesthetic and hemodynamic considerations for a tracheal plasmacytoma. We discuss the approach to airway management in variable intrathoracic tracheal obstruction and the unpredictability of tracheal tumors.

Nasal extramedullary plasmacytoma (EMP) is a rare plasma cell tumor that occurs in the soft tissue of the nasal cavity, and its imaging characteristics are still unclear. The purpose of this study was to investigate the clinical features, imaging findings, treatment, survival analysis, and prognosis of nasal EMP, and to provide a systematic review of the patients we treated and the published literature. A 45-year-old female patient who presented with epistaxis with nasal obstruction was recommended for magnetic resonance imaging to assess the nature of the lesion. On magnetic resonance imaging, abnormal signal shadow can be seen in the right nasal cavity. Diffusion weighted imaging showed signal of the lesion was significantly limited, presenting high signal, with a low apparent dispersion coefficient, and the lesion was significantly enhanced on contrast-enhanced scan. Combined with the clinical manifestations of the patient, who was initially considered to have a hemangioma. She underwent endoscopic nasal surgery under general anesthesia to remove the mass, and the final pathology confirmed it was EMP. However, the final pathology confirmed EMP. Five months later, the patient came to our hospital for follow-up and underwent fluorine-18-fluorodeoxyglucose/positron emission tomography/computed tomography scan, which showed no recurrence of the lesion and no transformation of multiple myeloma. The nasal EMP imaging findings were mostly soft tissue masses with uniform density or signal, which were significantly enhanced by enhancement scan, high signal on diffusion weighted imaging and low signal on apparent dispersion coefficient. Immunohistochemical staining for CD38, CD138, and CD79a was positive in most of the cases evaluated, while CD20 and CD10 were negative. The absence of dilated features, infiltrative features and the presence of significant contrast enhancement may be relatively specific imaging findings of nasal EMP. The prognosis of nasal EMP is good, and recurrence, metastasis, and transformation into multiple myeloma are rare. Because the lesions are sensitive to radiotherapy, surgical resection combined with radiotherapy is a more effective treatment., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: The authors declare that they have no conflict of interest.

Background: Extramedullary (extraosseous) plasmacytoma (EMP) of the small intestine is a rare plasma cell neoplasm (PCN) that presents with abdominal pain, nausea, vomiting, etc. We describe 2 cases of EMP of the small intestine that presented with ileus., Methods: A 78-year-old woman and 68-year-old man visited our clinic with complaints of abdominal discomfort and obstruction. Abdominal computed tomography (CT) revealed a thickened lesion with multiple enlarged mesenteric lymph nodes (LNs) in the ileum and duodenum. The female patient underwent segmental resection in the ileum. The male patient underwent Whipple's operation in the duodenum., Results: Histopathological examination and immunohistochemical staining of resected specimens from the 2 patients confirmed a PCN. In the surgical specimens of 2 cases, immunoglobulin heavy-chain rearrangement was confirmed by polymerase chain reaction amplification, but no Epstein-Barr virus (EBV)-infected cells were found by EBV-in situ hybridization. Bone marrow aspirate and trephine biopsies were negative for the type of PCN. Bone marrow cytogenetics and fluorescence in situ hybridization revealed no abnormalities. Serum β2-microglobulin levels were within normal limits. Additionally, none of the patients showed an M-spike in serum or urine protein electrophoresis. Therefore, the patients were diagnosed with a solitary EMP of the small intestine. The female patient refused treatment. At follow-up 3 months postoperatively, her disease progressed and she newly developed multiple LNs and nodular lesions in the right pelvic side wall. She was treated with dexamethasone. The male patient experienced back pain 25 days after Whipple's operation. Spine series magnetic resonance imaging revealed an intermediate signal intensity mass in the posterior epidural space from T8/9 to T10. The mass was removed, and the same histologic features were identified as duodenal masses. He was treated with dexamethasone and radiotherapy., Conclusions: EMPs of the small intestine are easy to overlook because they rarely occur in the small intestine. Although surgery is not required for diagnosis, surgical resection can be a good option for EMPs of the small intestine, instead of local radiation therapy. However, close follow-up is required due to the possibility of relapse or progression to plasma cell myeloma., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: While extramedullary plasmacytomas are infrequently encountered plasma cell malignancies, most cases occur in the head and neck, with a predilection for the sinonasal cavity. Due to the rarity of this disease, the majority of studies on sinonasal extramedullary plasmacytoma (SN-EMP) are case reports or small retrospective case series., Objective: To investigate the impact of patient, disease, and treatment factors on the survival of patients with SN-EMP., Methods: The National Cancer Database was queried for all patients with SN-EMP between 2004-2016 (N = 381 cases). Univariate and multivariate analyses were used to examine patient demographics, tumor characteristics, and survival., Results: The majority of SN-EMP patients were over 60 years old (57.0%), male (69.8%), and white (86.2%). The most common treatment modality was radiotherapy alone (38.6%), followed by surgery plus radiotherapy (37.8%). Five-year overall survival was 74.0% and median survival was 9.1 years. Accounting for patient demographics and tumor characteristics in a multivariate model, the following groups had worse prognosis: 60 and older (HR 1.99, p = 0.031) and frontal sinus primary site (HR 11.56, p = 0.001). Patients who received no treatment (HR 3.89, p = 0.013), chemotherapy alone (HR 5.57, p = 0.008) or radiotherapy plus chemotherapy (HR 2.82, p = 0.005) had significantly lower survival than patients who received radiotherapy alone. Patients who received surgery with radiotherapy (HR 0.57, p = 0.039) had significantly higher survival than patients who received radiotherapy alone., Conclusion: In patients with SN-EMP five-year overall survival was found to be 74.0% with decreased survival associated with a frontal sinus primary site and being aged 60 or older. Patients receiving no treatment, chemotherapy alone, or radiotherapy with chemotherapy was associated with lower survival. Receiving surgery plus radiotherapy was associated with the highest five-year overall survival.

(1) Background: Plasma cell neoplasia can be separated into independent subtypes including multiple myeloma (MM) and solitary plasmacytoma of the bone (SBP). The first clinical signs patients present with are skeletal pain, most commonly involving ribs and vertebrae. (2) Methods: Retrospective analysis of 114 patients (38 female, 76 male) receiving spinal surgery from March 2006 until April 2020. Neurological impairments and surgical instability were the criteria for intervention in this cohort. Analysis was based on demographic data, Spinal Instability Neoplastic Score (SINS), location of the lesion, spinal levels of tumor involvement, surgical treatment, histopathological workup, adjuvant therapy, functional outcome, and overall survival (OS). (3) Results: The following surgical procedures were performed: posterior stabilization only in 9 patients, posterior stabilization and decompression without vertebral body replacement in 56 patients, tumor debulking and decompression only in 8 patients, anterior approach in combined approach without vertebral body replacement and without biopsy and/or without kyphoplasty in 33 patients, 3 patients received biopsies only, and 5 patients received kyphoplasty only. The histopathology diagnoses were MM in 94 cases and SBP in 20 cases. Median OS was 72 months (53.4-90.6 months). Preoperative KPSS was 80% (range 40-100%), the postoperative KPSS was 80% (range 50-100%). (4) Conclusions: Surgery for patients with plasma cell neoplasia is beneficial in case of neurological impairment and spinal instability. Moreover, we were able to show that patients with MM and a low number of spinal levels to be supplied have a better prognosis as well as a younger age at the time of the surgical intervention.

Extramedullary plasmacytoma (EMP) is a tumor characterized by plasma cell proliferation in organs or tissues outside the bone marrow. It originates from B lymphocytes and can occur in all extramedullary tissues and organs of the body. Primary EMPs of the nasal cavity are relatively rare and mostly presented as case reports. Nasal EMP usually manifests as nasal obstruction, epistaxis, and progressive dyspnea. A 64-year-old man was admitted to our hospital because of rhinorrhagia with nasal obstruction. Computed tomography (CT) revealed a mass in the right nasal passage, which we resected under nasal endoscopy. At the 2-year follow-up, positron emission tomography/CT showed osteolytic bone destruction in the third cervical vertebra and its accessories, accompanied by increased metabolism. We considered the possibility of progression to myeloma, but the patient refused another puncture biopsy. We reviewed the literature for the clinical characteristics and CT findings of 14 patients pathologically confirmed with EMPs of the nasal cavity. All patients had nonspecific clinical manifestations, such as nasal obstruction and epistaxis. Computed tomography mostly showed a uniform-density polypoid mass in the nasal cavity. Therefore, nasal EMP should be included in the differential diagnosis of nasal polyps and other nasal tumors.

Objective: The decision to perform surgery in cases of spinal plasmacytoma (SP) is controversial. This study aimed to evaluate the reliability of the Spinal Instability Neoplastic Score (SINS) in evaluation of spinal instability in patients with SP., Methods: Clinical and radiological characteristics of 10 patients with SP were retrospectively evaluated. Age, sex, preoperative symptoms, duration of symptoms, pain score, American Spinal Injury Association score, and SINS were analyzed., Results: The 10 patients included 6 men and 4 women. Plasmacytoma was located in the sacrum in 1 patient, in the lumbar spine in 2 patients, in the thoracic spine in 6 patients, and in the cervical spine in 1 patient. Biopsy was performed in 2 patients, biopsy and vertebroplasty were performed in 2 patients, and biopsy and acute decompression and stabilization surgery were performed in 6 patients. SINS was <7 in 1 patient, 7-12 in 5 patients, and >12 in 4 patients. Two patients with a low SINS (<13) underwent only biopsy, and 2 patients underwent biopsy and vertebroplasty. Decompression and stabilization surgery was performed in 2 patients with SINS 7-12 and 4 patients with SINS >12., Conclusions: Decision making regarding augmentation, decompression, and stabilization in patients with SP is controversial. SINS may play a role during the decision-making process. Augmentation can be performed in patients with painful SPs with osteolytic changes with or without fracture (SINS <13). Decompression and stabilization surgery is the first-choice treatment in patients with SINS >12., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. Methods: A retrospective analysis was conducted on 10 cases with extramedullary plasmacytoma of the head and neck who were admitted to Henan Tumor Hospital from January 2005 to January 2020. Among the 10 patients, 6 were male and 4 were female. The average age at diagnosis was 56.3 years old (34-74 years old). Among them, 3 cases were located in the nasal cavity, 2 cases in the nasopharynx, 1 case in the sinuses, 2 cases in the larynx, 1 case in the oropharynx, and 1 case in the cervical lymph nodes. Treatments were administered according to tumor size and resection extent. Complete surgical excision (negative margins) was preferred, followed by adjuvant radiotherapy or radiotherapy alone. The clinical characteristics, diagnosis, treatment and prognosis of EMP were analyzed. Results: The patients' symptoms were not specific, frequently with local obstruction symptom and localized masses. All patients were confirmed pathologically as suffering from monoclonal plasmacytoma, with negative bone marrow biopsy and negative skeletal survey. Five patients received surgery, 3 received radiotherapy, and 2 received surgery with additional radiation. The follow-up time was 16-125 months, with a median of 92 months. Two patients developed into multiple myeloma. One patient who received radiotherapy after surgery relapsed after 7 years of follow-up and again received surgical treatment, with no evidence of second recurrence. The remaining patients had no recurrence or progression. Conclusion: Extramedullary plasmacytoma of the head and neck has a good prognosis. Surgical treatment can be considered for completely resectable lesions.

An extramedullary plasmacytoma (EMP) is a rare neoplasm characterized by monoclonal proliferation of plasma cells without features of multiple myeloma. Most EMPs occur in the head and neck region, especially in the aerodigestive tract. We herein report a case of an EMP arising from the nasal inferior turbinate. The mass was surgically removed, and a short cycle of radiotherapy was performed after the surgery. There was no recurrence of the tumor after 1 year of follow-up. These result may be useful for physicians who encounter similar situations in clinical practice.

Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm, with the majority (80-90% of cases) occurring in the upper aerodigestive tract. To our best knowledge, primary EMP from renal tissues is extremely rare. Herein, the diagnosis and treatment of a refractory primary EMP with renal involvement in a 53-year-old male patient is reported. The patient received radical nephrectomy followed by radiotherapy, and showed relapse 3 months after treatment. The cancer cells were sensitive to subsequent chemotherapy, however, the patient died of infection associated with the disease after almost 3.5 years following first presentation.

A 10-year-old, intact male Siberian husky dog was presented for a suspected left renal cyst. Computed tomography (CT) identified a large, left kidney mass with retroperitoneal hemorrhage. A left-sided nephrectomy was performed, and histopathology confirmed a renal plasmacytoma. Perioperative screening for multiple myeloma was negative. The dog was lost to follow-up and was euthanized 11 months after surgery. A necropsy was not performed. To the authors' knowledge, this is the first case of renal extramedullary plasmacytoma in a dog. Key clinical message: This report describes the clinical presentation, and laboratory, diagnostic imaging, and surgery findings of a case of renal extramedullary plasmacytoma in a dog., (Copyright and/or publishing rights held by the Canadian Veterinary Medical Association.)

We present an extreme rare case of extramedullary nasal plasmacitoma that arise from nasal septum. The mass surgically removed was analyzed by a pathologist who diagnosed an extramedullary nasal plasmacytoma. The patient did not present systemic involvement. A short cycle of radiotherapy was performed after the surgery. At 9-month follow-up, the patient is recurrence free.

Extramedullary plasmacytoma of the iris and ciliary body is extremely rare. We present a case which was misdiagnosed as granulomatous uveitis with neovascular glaucoma, and underwent a trabeculectomy with mitomycin-c along with iris biopsy. The post-operative period showed early bleb failure and catastrophic growth of the suspected mass. Histopathological examination revealed a diagnosis iris plasmacytoma. Subsequent ultrasound biomicroscopy showed involvement of the iris and ciliary body. A prompt systemic workup was done, and an associated systemic plasma cell dyscrasia was ruled out. The affected eye was enucleated, and the patient remains disease free at the end of 3-year follow-up.

A 63-year-old man presented with imbalance when coughing due to a respiratory tract infection. He had a history of multiple myeloma with a plasmacytoma of the left temporal bone. Examination revealed a positive leftward head impulse test, no spontaneous nystagmus, left-beating positional nystagmus, and left-beating Valsalva-induced nystagmus. Videonystagmography, audiology, and comprehensive vestibular function tests revealed a subtotal left peripheral audio-vestibular loss. Temporal bone computed tomography showed an unchanged bony erosion of the left labyrinth from 2 years prior. Vertigo subsided after treatment of the respiratory tract infection. Although no tumor progression was evident, coughing had triggered a preexisting third mobile window to declare itself. Laryngoscope, 131:E966-E969, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Solitary plasmacytoma of bone (SPB) is a single, isolated plasmacytoma originated from the bone. The survival trends of patients with SPB in recent years remain unknown. And the prognostic system of SPB may also need to be refined. The 18 Surveillance, Epidemiology, and End Results (SEER) databases of the National Cancer Institute in the United States were used to extract data for this study. The third edition of the International Classification of Disease for Oncology (ICD-O-3) code 9731 was used to identify cases of SPB. For each case, factors including age at the time of diagnosis, sex, race, marital status, insurance status, primary sites of tumors, and the use of surgery were collected. The outcomes of patients with SPB were compared between two groups. And the prognostic impacts of baseline characteristics and use of surgery was studied. A total of 4103 (from 1976 to 2016) cases of SPB were identified. The median age was 65 years old. Patients in time period-2 (2008-2016) show better survival as compared to those in time period-1(1976-2007) (median overall survival: 88 months vs. 73 months, p = 0.0332). Age ≤ 65 years and being male were associated with better outcomes. The widowed individuals had significantly inferior survival and myeloma-specific survival than the single, married, or divorced individuals (p values all <0.0001). Patients with lesions in bones of skull and face and associated joints had longer survival as compared with those with bone lesions in other sites (median overall survival: 107 months vs. 79 months, p = 0.0694). The use of surgery was significantly associated with improved survival (median survival: surgery performed 98 months vs. not performed 73 months, hazards ratio [HR]: 0.7623, 95% CI: 0.7009-0.8472; p < 0.0001) and myeloma-specific survival (median myeloma-specific survival: surgery 160.0 months vs. no surgery 143.0 months, HR: 0.8469, 95% CI: 0.7493-0.9572; p = 0.0078). Multivariable analysis revealed that surgery, marital status, and age were independent prognostic factors for overall survival in patients with SPB. The improvement in the survival of patients with SPB has been observed in recent years. And several potential prognostic factors were identified. Future prospective studies are warranted to explore the roles of novel agents, surgery, and systemic chemotherapy in the treatment of SPB., (© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Solitary plasmacytoma (SP) of the skull is an uncommon clinical entity that is characterized by a localized proliferation of neoplastic monoclonal plasma cells. This case report describes a 50-year-old male that presented with a headache and an exophytic soft mass on the occiput. The diagnosis of SP was based on the pathological results and imaging examinations. The patient underwent occipital craniotomy, skull reconstruction and lower trapezius myocutaneous flap (LTMF) transplantation under general anaesthesia. The tumour was capsulized and extended to the subcutaneous and the subdural space through the dura mater with skull defects. The neoplasm of the occipital bone involved large areas of scalp and subcutaneous tissue, which resulted in a large postoperative scalp defect that was repaired using LTMF transplantation. All of the tumour was removed and the transplanted flap grew well. Follow-up at 5 months identified an aggressive mass lesion on the right frontal lobe. The patient received six cycles of the PAD chemotherapy regimen (bortezomib, doxorubicin and dexamethasone) and the lesion was significantly reduced. This case demonstrates that LTMF is an alternative approach for the repair of scalp and subcutaneous soft tissue defects caused by the excision of a large malignant tumour of the occipital region. Chemotherapy is the choice of treatment for neoplastic recurrence.

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.)

Background Context: Solitary plasmacytoma of bone (SPB) can progress to multiple myeloma (MM). Little attention has been paid to the reossification findings on computed tomography (CT) and their correlation with prognosis after radiotherapy with/without surgery., Purpose: To evaluate reossification after radiotherapy and prognostic factors of spinal SPB using single-center data., Study Design: Retrospective observational study., Patient Sample: Patients who had spinal SPB and received radiotherapy with/without surgery, without chemotherapy, denosumab or zoledronic acid., Outcome Measures: MM progression rate, mortality rate, and reossification rate at 12 months., Methods: This retrospective clinical review included 39 patients who underwent radiotherapy as first-line treatment for SPB in the spine. External radiation was divided into 20-25 fractions with a total dose of 35-46 Gy. At the 12-month follow-up after the index radiotherapy, significant and mild reossification, defined as bone formation with ≥30% or 0%-30% increase, respectively, in bony area based on increase in CT values were documented, along with progressive disease, which was a decrease in bony area with lesion enlargement. This study was funded by AO Foundation, AOSpine (AOSDIA2019-026) (CHF45,000), Peking University Medicine Seed Fund for Interdisciplinary Research (BMU2018MX022) (¥40,000), and Peking University Third Hospital (No. Y71508-01) (¥400,000)., Results: Twenty-six men and 13 women (mean age, 51.5 years) were included. Solitary plasmacytomas were located in the cervical, thoracic, and lumbar vertebrae in 16, 17, and 6 patients, respectively. The mean clinical follow-up period after treatment was 72 (range 12-216) months. Sixteen patients (41.0%) had significant reossification after radiotherapy, 21 (53.8%) showed mild reossification, and 2 (5.2%) had progressive bony destruction (after 7 and 23 months, respectively). There were no significant differences in age among the three groups (p=.127). At a mean follow-up of 37 (range 6-90) months after radiosurgery, 14 (35.8%) patients developed MM, including 9 patients who died at a mean duration of 55 (range 19-102) months. In the significant reossification group, only 1 patient (6.3%, 1 of 16) had MM progression 82 months after treatment (p=.044). In the mild reossification group, 56.5% (13 of 23) of patients had MM progression. The significant reossification rates of the radiotherapy dose groups of <40 Gy and ≥40 Gy were 35.7% and 44% (p=.614), respectively. In the univariate analysis, age ≥65 years (p<.001), tumor ≥5 cm (p=.009), Spinal Instability Neoplastic Score scores ≥11.5 (p=.040), radiotherapy (RT) combined with surgery (p<.001), and progression to MM (p=.007) were the independent prognostic factors for overall survival; whereas, age >44 years (p=.045) and RT combined with surgery (p<.001) were for multiple myeloma-free survival. In the multivariate analyses, age >65 years (p=.004) and progression to MM (p=.007) were the unfavorable independent factors for overall survival, whereas RT combined with surgery (p=.004) was the only factor for multiple myeloma-free survival., Conclusions: In patients with spinal SPB, 41.0% lesions showed significant reossification after radiotherapy. Patients with significant reossification had a better prognosis with less possibility of MM progression. Radiotherapy may be a safe and effective treatment choice for spinal SPB; more attention should be paid to reossification., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: To demonstrate the use of 3-dimensional (3D)-printed intracranial lesion models for complex neurosurgery to increase the success rate of clinical surgeries via practice in simulated surgeries., Methods: We collected computed tomographic, magnetic resonance (MR), and computed tomographic images from patients with intracranial tumor or aneurysm, conducted multimodal image reconstruction, and then constructed a 3D-printed model with the skull base, cerebral arteries, and brain tumor or aneurysm. Forty-nine simulated surgeries were carried out on the model under a microscope, and actual surgery was carried out after validation and accumulation of experience., Results: The 3D-printed brain tumor models were used to design the surgical route, to simulate piecemeal resection of tumors through keyhole approach, and to verify the extent of tumor resection. A drill was used for bone flap removal and milling of bony structures such as the anterior clinoid process, tuberculum sellae, petrous apex, and internal acoustic meatus. The tumors were removed by laser knife and cavitron ultrasonic aspiration. The 3D-printed aneurysm models were used to assess the feasibility of different keyhole approaches and to select the aneurysm clip. Actual surgery was based on the results of the simulated surgery. Postoperative MR image review showed that 84% (21/25) of patients had total tumor resection and 16% (4/25) subtotal resection. Digital subtraction angiography confirmed complete clipping of all aneurysms (24 cases/39 aneurysms)., Conclusions: 3D-printed craniocerebral models provide effective simulated surgery conditions for keyhole surgeries of complex brain tumors or aneurysms and aid in preoperative surgical design, accumulation of surgical experience, and validation of surgical outcomes., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Most plasmacytomas arise in the bone marrow (intramedullary), as part of multiple myeloma (MM). In contrast, extramedullary plasmacytoma without MM is rare, and plasmacytoma primarily occurring in the brain parenchyma is extremely rare. Clinical behaviors of primary plasmacytoma in the brain have remained unclear. We report a case of primary plasmacytoma in the cerebellum and review the literature., Case Description: The patient was a 33-year-old woman, displaying vertigo and peripheral facial nerve palsy. A tumor was identified in the subcortical white matter of the middle-upper cerebellum. Magnetic resonance imaging showed no specific findings for this lesion. Tumor was surgically resected because of aggressive tumor growth. Pathologic diagnosis of the tumor was plasmacytoma. The patient was treated with irradiation to the tumor bed after surgery. Although histology of the bone marrow showed a few atypical plasma cells (1%-2%), below the threshold of the diagnostic criterion for MM, we started chemotherapy to prevent occurrence of MM. Neither tumor recurrence nor development of MM was found for 16 months after surgery., Conclusions: Histology is essential for diagnosis of primary plasmacytoma in the brain because of the lack of specific findings on neuroimaging. A finding of a few atypical plasma cells in the bone marrow might support the assumption that extramedullary plasmacytoma represents a harbinger of subsequent development of MM. In addition to appropriate therapies combining maximum tumor removal and radiotherapy to the brain, rigorous hematological management might have contributed to favorable outcomes., (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.)

Bone pathology can be challenging because the skeleton is a living tissue prone to developing a diverse array of inflammatory, metabolic, genetic, reactive, circulatory, and neoplastic abnormalities. Several areas of bone pathology are particularly difficult or problematic for hematopathologists given the close resemblance of some hematologic entities to primary/metastatic bone lesions; examples include plasmacytic disorders versus osteoblastic tumors and lymphoma/leukemia versus round cell tumors of bone. This article provides a conceptual and practical overview of selective bone disorders commonly encountered in the differential diagnosis of hematologic diseases., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Extramedullary plasmacytomas are rare tumors that typically involve the head and neck region. Only three reports exist in the literature of extramedullary plasmacytomas that involve the lower airways. We report the case of a 54-year-old woman with EMP of the right mainstem bronchus treated with sleeve resection., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. 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.)

Background: Multiple myeloma is a hematological malignancy that classically results in an abnormal clonal proliferation of plasma cells in the bone marrow. Extramedullary disease in the setting of multiple myeloma, referred to as secondary extramedullary plasmacytoma, is found in 7-17% of cases of multiple myeloma at the time of diagnosis and can involve any organ system. Small bowel obstruction is a rare but important gastrointestinal manifestation of multiple myeloma that should be considered in patients with multiple myeloma who present with concerning abdominal symptoms., Case Presentation: We present the case of a 52-year-old African-American man with a history of deep venous thrombosis (he is on anticoagulation) and pathologic fracture secondary to multiple myeloma diagnosed 4 months prior to our encounter. He presented with abdominal pain, constipation, nausea, and vomiting. An abdominal X-ray showed distended bowel loops concerning for bowel obstruction and a contrast-enhanced computed tomography scan of his abdomen and pelvis showed a 5.4 cm soft tissue mass involving a loop of distal ileum. He underwent laparoscopic exploration of his abdomen with small bowel resection and primary anastomosis for a small intussusception. He had an uneventful postoperative course and was discharged on postoperative day 6., Conclusions: Multiple myeloma has myriad presentations. Gastrointestinal involvement, although rare, can manifest as small bowel obstruction for which early recognition and appropriate surgical management are key to improving outcome. Intussusception is the most common mechanism of obstruction from extramedullary plasmacytoma causing small bowel obstruction and this has been seen in five of six case reports, including this case. It is important to recognize and consider the risks of immunosuppression, venous thromboembolism, and malnutrition in the surgical management of gastrointestinal complications of multiple myeloma.

Solitary extramedullary plasmacytoma (EMP) arising in the rectum is an extremely rare clinical entity. Only ten cases have been reported in the English-language literature. We experienced a case of an EMP in the rectum of a 55-year-old man with an 8-year history of proctitis-type ulcerative colitis (UC). The plasmacytoma appeared as an 8-mm semipedunculated polypoid lesion in the actively inflamed rectal mucosa when the remittent UC flared. The tumor was treated using endoscopic mucosal resection. This is the second case of rectal EMP associated with UC after a similar report was published in 2004. Both patients had a chronic history of proctitis-type UC and were taking no immunosuppressive agents that could cause Epstein-Barr virus-associated plasmacytoma, such as thiopurines. The UC activity seemed to correspond well with the development of the rectal EMP. Therefore, we herein discuss a possible association between rectal EMP and UC and review the past literature of rectal EMP.

Purpose: We aim to delineate the clinical characteristics of patients with primary intracranial solitary plasmacytoma (PISPC) and prognostic factors for their outcomes., Methods: This study retrospectively reviewed 17 patients with PISPC from our center and an additional 70 cases of PISPC published previously to analyze outcome predictors., Results: The entire cohort included 38 (43.7%) males and 49 (56.3%) females with a mean age of 54 years. Skull base tumors were found in 49 (56.3%) patients. Gross total resection (GTR) was achieved in 31 (35.6%) patients. Postoperative adjuvant treatments, including radiotherapy (RT) alone, chemotherapy (CMT) alone, and RT + CMT were administered in 49 (56.3%) patients, 3 (3.5%) patients, and 16 (18.4%) patients, respectively. After a median follow-up of 24 (mean 42.4) months, the 5-year disease progression-free survival (PFS), recurrence-free survival (RFS), multiple myeloma (MM)-free survival (MMFS), and overall survival (OS) were 52.9%, 76.2%, 69.6%, and 76.1%, respectively. Multivariate analysis unveiled that a skull base tumor location (HR 2.395, p = 0.040) and no RT (HR 3.115, p = 0.004) were negative prognostic factors for PFS, no RT (HR 10.526, p = 0.003) for RFS, each 1-year increase in age (HR 1.039, p = 0.049) for MMFS, and increasing age (HR 1.052, p = 0.043) and CMT (HR 6.022, p = 0.005) were risk factors for OS. However, GTR did not benefit the aforementioned outcomes., Conclusion: For patients with presumed PISPC, a biopsy followed by RT is recommended for skull base PISPC. However, the role of CMT is still not clear. Our findings need to be verified in a larger prospective cohort in the future. Systematic review registration number CRD42018098782.

Extramedullary plasmacytoma (EMP) is the localized plasma cell neoplasm, that arise in tissues other than bone. The upper respiratory tract and the oral cavity are the most common sites for EMP location. EMPs of the thyroid gland are extremely rare and thus little is known about their behavior compared to other EMPs., Case Report: 68-year-old female patient was admitted to the Department of General, Gastroenterological and Endocrine Surgery due to suspicion of neoplastic proliferation of unknown character within the thyroid gland. Computed tomography (CT) of the neck and chest showed tumor modeling adjacent anatomical structures. Cytological presentation obtained by fine needle aspiration biopsy was classified as category V according to the Bethesda system ("suspicious for malignancy"). The recognition of the lymphoma was suggested. The histopathological examination result of the specimens obtained from surgical biopsy was ambiguous. The patient was transferred to the Neurosurgical Department to continue the diagnostic process due to a presence of tumor within Th5 vertebral body. Percutaneous biopsy and magnetic resonance imaging (MRI) was performed, revealing metastatic character of the lesion and presence of plasma cells. After transferring patient back to surgical ward thyroidectomy was performed. Postoperative histopathological and immunohistochemisty analysis revealed EMP with highly immature morphology. Afterwards patient was admitted to Hematology Clinic in order to establish an adjuvant therapy., Conclusions: EMP is a very rare form of the primary thyroid malignancy, what makes it difficult to recognize. However, in the differential diagnosis of the thyroid tumors, EMP should be always considered., (© 2019 MEDPRESS.)