Extramedullary plasmacytoma with the uvula as first affected site:A case report

BACKGROUND Extramedullary plasmacytoma(EMP)represents one of the rarer forms of plasma cell malignancies,capable of impacting a variety of tissues and organs throughout the body.The majority of EMP cases are predominantly found in the head and neck region,especially within the laryngopharynx,as well as in the gastrointestinal tract.While there have been documented instances of oropharyngeal involvement in EMP cases in the academic literature,it is important to note that EMP specifically affecting the uvula is exceedingly uncommon.Furthermore,it is noteworthy that over 60% of epithelial carcinomas in the upper respiratory tract and oropharynx tend to metastasize to the cervical lymph nodes,indicating a propensity for regional spread in these types of cancers.In this context,we present a rare case of extramedullary plasmacytoma where the uvula served as the initially affected site.This case emphasizes the need for heightened awareness among clinicians regarding such unusual comorbidities,as early recognition and diagnosis can significantly influence patient management and treatment outcomes.In addition,a review of the relevant literature is included to further educate and inform healthcare professionals about this rare presentation,ultimately aiming to enhance clinical understanding and improve patient care in similar situations.CASE SUMMARY A 51-year-old man was admitted to our hospital because of a slowly enlarging neck mass.A physical examination revealed a palpable left lymph node,and magnetic resonance imaging(MRI)of the oropharynx and the neck showed a soft tissue mass in the oropharynx and enlargement of multiple lymph nodes in the neck.The soft tissue mass was diagnosed as plasmacytoma by immunohistochemical analysis.Monoclonal immunoglobulins and bone marrow biopsy showed normal results.Therefore,we diagnosed that as EMP of the uvula.After four cycles of adjuvant chemotherapy dominated by bortezomib,MRI reexamination showed a significant reduction of the mass in the oropharynx and the cervical lymph nodes.Afterwards,the λ light chain returned to normal levels.There was no evidence of evolution to multiple myeloma.CONCLUSION We have reported a rare case of extramedullary plasmacytoma with the uvula as the first affected site and the relevant literature is reviewed to improve clinicians'awareness of such rare comorbidities.

Role of long non-coding RNAs in non-alcoholic fatty liver disease

Non-alcoholic fatty liver disease(NAFLD)is emerging as a common cause of chronic liver disease in children and adults.NAFLD can progress to steatohepa-titis and potentially even hepatocellular carcinoma.Early identification of pati-ents at risk for progressive disease is crucial for managing NAFLD.Recent studies have identified long noncoding RNAs(lncRNAs),circular RNAs,and microRNAs as playing important roles in the pathogenesis of NAFLD.These noncoding RNAs are involved in modulating several metabolic pathways such as hepatic glucose and lipid metabolism,oxidative stress,and even carcinogenesis.Elevated levels of lncARSR and lncRNA nuclear-enriched abundant transcript 1 have been found in patients with NAFLD.In addition,lncRNAs such as PRYP4-3 and RP11-128N14.5 can distinguish patients with NAFLD from healthy indi-viduals.Increased MEG3 expression has been observed in both NAFLD and non-alcoholic steatohepatitis,suggesting that it may help predict patients at risk for disease progression.With advances in transcriptomics,we may discover additional targets to help in the identification and prognostication of NAFLD.

头颈部髓外浆细胞瘤3例

髓外浆细胞瘤(extramedullry plasmcytoma,EMP)是来源于B淋巴细胞的肿瘤,由单克隆细胞异常增生形成的一种较为罕见的疾病[1]。EMP在临床和影像学表现不典型,早期难以诊断,往往术后经病理检查才得以确诊。由于EMP病例罕见,目前尚无治疗指南,治疗方式仍存在争议。1临床资料病例1。患者,女,60岁,于2009-12-29因右侧鼻塞、流涕就诊,鼻窦CT检查示右侧鼻腔外侧壁软组织块状影(图1A),遂入院治疗并完善相关检查后行鼻内镜下右侧鼻腔肿物切除术,术后病理结果考虑EMP。

Interaction between age and gender on survival outcomes in extramedullary multiple myeloma over the past two decades

BACKGROUND Extramedullary multiple myeloma(MM)(EMM)is a rare and aggressive subentity of MM that can be present at diagnosis or develop anytime during the disease course.There is a paucity of data on the clinical characteristics and overall epidemiology of EMM.Furthermore,there is a scarcity of data on how the interaction of age and gender influences the survival of EMM.AIM To evaluate the clinical characteristics of patients with EMM over the past 2 decades and to identify epidemiologic characteristics that may impact overall prognosis.METHODS A total of 858 patients diagnosed with EMM,between 2000 and 2017,were ultimately enrolled in our study by retrieving the Surveillance,Epidemiology,and End Results database.We analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of EMM.Variables with a P value<0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio(HR)of greater than 1 representing adverse prognostic factors.RESULTS From a sample of 858 EMM,the male gender(63.25%),age range 60-79 years(51.05%),and non-Hispanic whites(66.78%)were the most represented.Central Nervous System and the vertebral column was the most affected site(33.10%).Crude analysis revealed higher OM in the age group 80+[HR=6.951,95%confidence interval(95%CI):3.299-14.647,P=0],Non-Hispanic Black population(HR=1.339,95%CI:1.02-1.759,P=0.036),Bones not otherwise specified(NOS)(HR=1.74,95%CI:1.043-2.902,P=0.034),and widowed individuals(HR=2.107,95%CI:1.511-2.938,P=0).Skin involvement(HR=0.241,95%CI:0.06-0.974,P=0.046)and a yearly income of$75000+(HR=0.259,95%CI:0.125-0.538,P=0)had the lowest OM in the crude analysis.Crude analysis revealed higher CSM in the age group 80+,Non-Hispanic Black,Bones NOS,and widowed.Multivariate cox proportional hazard regression analyses only revealed higher OM in the age group 80+(HR=9.792,95%CI:4.403-21.774,P=0)and widowed individuals(HR=1.609,95%CI:1.101-2.35,P=0.014).Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups.Eyes,mouth,and ENT involvement had the lowest CSM in the multivariate analysis.There was no interaction between age and gender in the adjusted analysis for OM and CSM.CONCLUSION EMM is a rare entity.To our knowledge,there is a scarcity of data on the clinical characteristics and prognosis factors of patients with extramedullary multiple myeloma.In this retrospective cohort,using a United States-based population,we found that age,marital status,and tumor site were independent prognostic factors.Furthermore,we found that age and gender did not interact to influence the mortality of patients with EMM.

鼻咽部髓外浆细胞瘤1例

1临床资料患者,女,54岁,主因渐进性鼻塞1年余入院。患者夜间睡眠时张口呼吸伴打鼾,偶有头痛,无发热、血涕、耳鸣及听力下降。鼻内镜检查:右侧鼻咽部淡红色新生物,表面不光滑,质韧,堵塞全部右侧后鼻孔及大部分、左侧后鼻孔,基底位于右侧咽隐窝处(图1A)。耳内镜检查发现右耳鼓膜内陷,入院诊断为鼻咽部肿物。

鼻窦浆细胞瘤1例

1临床资料 患者男,45岁,因右侧鼻塞流涕8个月于2005-10-09入院。入院前8个月无明显诱因出现右鼻塞伴黏涕、嗅觉下降,进行性加重,偶尔头痛,无鼻出血和复视。当地医院以鼻窦炎治疗无效转来我院。专科检查：右侧鼻腔可见淡红色肿物堵塞,表面光滑,质软。查体：一般状况好，无浅表淋巴结肿大，眼球活动自如，视力正常，心、肺、肝、脾、肾未见明显异常。

上颌窦髓外浆细胞瘤1例

1临床资料患者，男，42岁。因左眼溢泪6个月，左侧鼻塞伴左侧头痛1个月于2011年1月人院。患者6个月前无明显诱因出现左眼溢泪及左侧鼻塞，伴阵发性头痛，头痛无规律，以左侧前额部为重，无鼻涕及涕中带血，无视物模糊及复视。曾于外院多次诊断为泪囊炎，全身应用抗生素及眼药水滴眼治疗，溢泪无好转。

Imaging findings of primary gastric plasmacytoma:A case report

Primary gastric plasmacytoma (GP) is a rare extramedullary plasmacytoma with clinical and imaging features that are common among other gastric tumors, such as gastric adenocarcinomas, gastric stromal tumors, and lymphomas. Here, we present a histologically con&#x0fb01;rmed case of primary GP examined with biphasic computed tomography (CT), magnetic resonance imaging (MRI), and endosonography. A well-circumscribed extraluminal mass appearing as homogeneous attenuation/intensity with gradual enhancement was identified on biphasic enhancement CT and MRI. This mass was hyperintense on diffusion-weighted imaging and hypointense on the apparent diffusion coefficient map, implying that water diffusion in the mass was restricted. In addition, endosonography indicated a low echogenic mass in the gastric wall. These imaging findings increase the available knowledge about imaging of this disease and provide valuable information for differentiating primary GP from common gastric tumors.

Characteristics of PVT1 and Its Roles in Diseases

With the development of genome-wide sequencing technology, 195 types of functional, long non-coding RNAs(lnc RNAs) have been identified so far, and their cellular roles are gradually being revealed. Lnc RNAs have now become a hotspot in the field of life sciences. These small molecules exist in almost all higher eukaryotes and play very important regulatory roles in these organisms. This review briefly summarizes the recent progress in research on plasmacytoma variant translocation 1 gene(PVT1), an lnc RNA.

A case of mucosa-associated lymphoid tissue lymphoma of the gastrointestinal tract showing extensive plasma cell differentiation with prominent Russell bodies

A 73-year-old Japanese woman was hospitalized for detailed examination of nausea, diarrhea and loss of appetite. Atypical erosion in the ileum was found on endoscopy. Biopsy of this erosion showed proliferation of cells containing numerous Russell bodies. Differential diagnoses considered were Russell body enteritis, crystal-storing histiocytosis, Mott cell tumor, immunoproliferative small intestinal disease(IPSID) and mucosaassociated lymphoid tissue(MALT) lymphoma. The cells containing prominent Russell bodies showed diffuse positivity for CD79 a and CD138, but negative results for CD20, CD3, UCHL-1, CD38 and CD68. Russell bodies were diffusely positive for lambda light chain, but negative for kappa light chain, and immunoglobulin(Ig)G, Ig A and Ig M. Based on these findings, Russell body enteritis, crystal-storing histiocytosis and IPSID were ruled out. As the tumor formed no mass lesions and was restricted to the gastrointestinal tract, MALT lymphoma with extensive plasma cell differentiation was finally diagnosed. The patient showed an unexpectedly aggressive clinical course. The number of atypical lymphocytes in peripheral blood gradually increased and T-prolymphocytic leukemia(T-PLL) emerged. The patient died of T-PLL 7 mo after admission. Autopsy was not permitted.

Rare pancreas tumor mimicking adenocarcinoma: Extramedullary plasmacytoma

Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.

Coexistence of cervical extramedullary plasmacytoma and squamous cell carcinoma:A case report

BACKGROUND Extramedullary plasmacytoma(EMP),a variant form of myeloma,is a rare solid plasma cell tumor that originates from the bone marrow hematopoietic tissue and accounts for about 3%of all plasma cell tumors.EMP can affect various tissues and organs,about 90%of which is found in the head and neck.However,EMP in the reproductive organs is rare,and is difficult to be distinguished from other primary or metastatic genital tumors according to clinical symptoms and imaging findings.CASE SUMMARY Herein,we report a case with coexistence of EMP and squamous cell carcinoma in the cervix.The first histopathological report of neoplasms on the surface of the cervix and vagina showed an EMP.Both ultrasound and pelvic enhanced magnetic resonance imaging(MRI)indicated that there was a tumor in the cervix.Thus,another cervical biopsy and pathological examination were performed,which indicated EMP combined with squamous cell carcinoma.Then,the patient underwent extensive total hysterectomy(type C1)+systemic lymph node dissection and received 25 external pelvic irradiations with a dose of 50 Gy following surgery.During 2-year follow-up,no recurrence was reported.CONCLUSION In conclusion,EMP involving the reproductive system is relatively rare.In this case,MRI,B-ultrasound,and cervical canal scraping were used to further determine the diagnosis of EMP combined with squamous cell carcinoma.The patient had improved prognosis after appropriate treatments.

Russell body gastritis with Dutcher bodies evaluated using magnification endoscopy

Russell body gastritis(RBG) is an unusual type of chronic gastritis characterized by marked infiltration of Mott cells,which are plasma cells filled with spherical eosinophilic bodies referred to as Russell bodies.It was initially thought that Helicobacter pylori(H.pylori) infection was a major cause of RBG and that the infiltrating Mott cells were polyphenotypic;however,a number of cases of RBG without H.pylori infection or with monoclonal Mott cells have been reported.Thus,diagnostic difficulty exists in distinguishing RBG with monoclonal Mott cells from malignant lymphoma.Here,we report an unusual case of an 86-year-old-Japanese man with H.pylori-positive RBG.During the examination of melena,endoscopic evaluation confirmed a 13-mm whitish,flat lesion in the gastric antrum.Magnification endoscopy with narrowband imaging suggested that the lesion was most likely a poorly differentiated adenocarcinoma.Biopsy findings were consistent with chronic gastritis with many Mott cells with intranuclear inclusions referred to as Dutcher bodies.Endoscopic submucosal dissection confirmed the diagnosis of RBG with kappa-restricted monoclonal Mott cells.Malignant lymphoma was unlikely given the paucity of cytological atypia and Ki-67 immunoreactivity of monoclonal Mott cells.This is the first reported case of RBG with endoscopic diagnosis of malignant tumor and the presence of Dutcher bodies.

Non-secretory multiple myeloma expressed as multiple extramedullary plasmacytoma with an endobronchial lesion mimicking metastatic cancer:A case report

BACKGROUND Non-secretory multiple myeloma(MM)is a rare condition that accounts for only 3%of MM cases and is defined by normal serum and urine immunofixation and a normal serum free light chain ratio.Non-secretory MM with multiple extramedullary plasmacytomas derived from endobronchial lesions is extremely rare and can be misdiagnosed as metastasis of solid cancer.CASE SUMMARY A 36-year-old man presented with progressive facial swelling and nasal congestion with cough.Various imaging studies revealed an endobronchial mass in the left bronchus and a large left maxillary mass with multiple destructive bone metastatic lesions.He initially presented with lung cancer and multiple metastases.However,pathologic reports showed multiple extramedullary plasmacytomas in the left maxilla and the left bronchus.There was no change in the serum and urine monoclonal protein levels,and no abnormalities were observed in laboratory examinations,including hemoglobin,calcium,and creatinine levels.The bone marrow was hypercellular,with 13.49%plasma cells.The patient was diagnosed with non-secretory MM expressed as multiple extramedullary plasmacytomas with endobronchial lesions in a rare location.Radiation therapy for symptomatic lesions with high-dose dexamethasone was started,and the size of the left maxillary sinus lesion dramatically decreased.In the future,chemotherapy will be administered to control lesions in other areas.CONCLUSION We present a rare case of non-secretory MM with multiple extramedullary plasmacytoma with an endobronchial lesion.

Difficult endoscopic diagnosis of a pancreatic plasmacytoma: Case report and review of literature

A 71-year-old man, with history of plasmacytoma in relapse since one year, was hospitalized for a initial presentation of acute pancreatitis and hepatitis.Although there was a heterogeneous infiltration around the pancreas head, the diagnosis of an extramedullary localization of his plasmacytoma was not made until later.This delayed diagnosis was due to the lack of specific radiologic features and the lack of dilatation of biliary ducts at the admission.A diagnosis was made with a simple ultrasound guided paracentesis of the low abundance ascites after a transjugular hepatic biopsy, an endoscopic ultrasound-guided fine needle aspiration of the pancreatic mass, and a failed attempt of biliary drainage through endoscopic retrograde cholangiopancreatography.In order to document the difficulty of this diagnosis, characteristics of 63 patients suffering from this condition and diagnosis were identified and discussed through a systematic literature search.

Bortezomib in treatment of extramedullary plasmacytoma of the pancreas

BACKGROUND: Extramedullary pancreatic plasmacytoma treated with bortezomib is rarely reported. METHODS: We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months, then an asymptomatic swelling of the pancreas was found. A biopsy on the mass and a fine needle aspiration of the pancreas were performed. The diagnosis of extramedullary plasmacytoma (EMP) was made. The patient was initially treated with combination chemotherapy consisting of vincristine, doxorubicin and dexamethasone (VAD regimen). She progressed to painless jaundice during the chemotherapy. Then she was treated with bortezomib and hyper-dose dexamethasone. As a result, she had a near complete remission. RESULTS: The data demonstrated that the diagnosis was EMP of the pancreas. The patient responded very well to bortezomib, while failing to respond to the traditional chemotherapy regimen of VAD. CONCLUSION: EMP of the pancreas is rare. This case gives evidence for an excellent response of EMP of the pancreas to bortezomib. (Hepatobiliary Pancreat Dis Int 2009; 8: 329-331)

Gastrointestinal bleeding as initial presentation of extramedullary plasma cell neoplasms: A case report and review of the literature

BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.

Management of extramedullary plasmacytoma: Role of radiotherapy and prognostic factor analysis in 55 patients

Objective： To investigate potential prognostic factors affecting patient outcomes and to evaluate the optimal methods and effects of radiotherapy（RT） in the management of extramedullary plasmacytoma（EMP）.Methods： Data from 55 patients with EMP between November 1999 and August 2015 were collected. The median age was 51（range, 22–77） years. The median tumor size was 3.5（range, 1.0–15.0） cm. The median applied dose was 50.0（range, 30.0–70.0） Gy. Thirty-nine patients（70.9%） presented with disease in the head or neck region. Twelve patients received RT alone, 9 received surgery（S） alone, 3 received chemotherapy（CT） alone, and 3 patients did not receive any treatment. Combination therapies were applied in 28 patients.Results：The median follow-up duration was 56 months.The 5-year local recurrence-free survival（LRFS）,multiple myeloma-free survival（MMFS）,progression-free survival（PFS）and overall survival（OS）rates were 79.8%,78.6%,65.2% and 76.0%,respectively.Univariate analysis revealed that RT was a favourable factor for all examined endpoints.Furthermore,head and neck EMPs were associated with superior LRFS,MMFS and PFS.Tumor size〈4 cm was associated with superior MMFS,PFS and OS;serum M protein negativity was associated with superior MMFS and PFS;age≥50 years and local recurrence were associated with poor MMFS.The dose≥45 Gy group exhibited superior 5-year LRFS,MMFS and PFS rates（94.7%,94.4%,90.0%,respectively）,while the corresponding values for the dose〈45 Gy group were 62.5%（P=0.008）,53.3%（P=0.036）and 41.7%（P〈0.001）.Conclusions：Involved-site RT of at least 45 Gy should be considered for EMP.Furthermore,patients with head and neck EMP,tumor size〈4 cm,age〈50 years and serum M protein negativity had better outcomes.

Primary pulmonary plasmacytoma accompanied by overlap syndrome:A case report and review of the literature

BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of multiple myeloma.Primary pulmonary plasmacytoma(PPP),with no specific clinical manifestations,is an exceedingly rare type of EMP.Because of its complexity,PPP is often difficult to diagnose,and there is no report in the literature on cases accompanied by overlap syndrome(OS).CASE SUMMARY A 61-year-old woman without a familial lung cancer history was admitted to our hospital in 2018,for intermittent cough,expectoration,and a stuffy feeling in the chest for 50 years;these symptoms appeared intermittently,especially occurred after being cold,and had been aggravated for the last 10 d.She was diagnosed with pulmonary fibrosis and emphysema,bronchiectasis,OS,and autoimmune hepatic cirrhosis in 2017.A pulmonary examination revealed rough breath sounds in both lungs;other physical examinations found no obvious abnormalities.A routine laboratory work-up showed decreased haemoglobin,increased ESR,and abnormal GGT,ALT,Ig G,γ-globulin,κ-light chain,λ-light chain,rheumatoid factor,and autoimmune antibodies.Emission computed tomography demonstrated abnormally concentrated 99 m Tc-MDP.Chest computed tomography revealed a soft tissue mass in the middle and lower lobes of the right lung.After right middle and inferior lobe resection with complete mediastinal lymph node dissection,immunohistochemical analysis revealed an isolated pulmonary plasmacytoma.The patient received chemotherapy for more than 1.5 years and remains in good general condition.CONCLUSION PPP is a type of EMP,and we report an exceedingly rare presentation of PPP accompanied by OS.

Solitary extramedullary plasmacytoma in retroperitoneum:A case report and review of the literature

Extramedullary plasmacytoma(EPM) is a plasma cell tumor arising outside of the bone marrow.Solitary EMP is an uncommon neoplasm and rarely occurs in the retroperitoneum and lacks distinctive clinical manifestations.We report a 26-year-old man with a solitary EMP in the retroperitoneum and discuss its clinical features,diagnosis and treatment.