Unusual presentation of extramedullary blast crisis in chronic myeloid leukemia:A case report

BACKGROUND Extramedullary blast crisis in chronic myeloid leukemia(CML)is an uncommon occurrence of leukemic blast infiltration in regions other than the bone marrow.Malignant infiltration of the serosal membranes should be considered in cases where CML presents with ascites or pleural effusion.CASE SUMMARY A 23-year-old female with CML presented with progressively worsening ascites and pleural effusion despite first-line tyrosine kinase inhibitor treatment.Her blood work indicated leukocytosis with myelocyte bulge and 2%blasts.Analysis of the patient’s bone marrow confirmed the chronic phase of CML.Abdominal ultrasound revealed hepatosplenomegaly with ascites.The fluid investigation of both ascites and pleural effusion revealed a predominance of neutrophils with exudate.However,no acid-fast bacilli or growth was observed after culturing.Although hydroxyurea reduced cell counts,there was no observed effect on ascites or pleural effusion.Repeat investigation of the ascitic and pleural fluid revealed a polymorphous myeloid cell population consisting of myelocytes,metamyelocytes,band forms,neutrophils and a few myeloblasts.Extramedullary blast crisis was suspected,and mutation analysis was performed.We switched the patient to dasatinib.The patient’s symptoms were relieved,and ascites and pleural effusion diminished.CONCLUSION Serosal membrane involvement in CML is extremely rare.In this case,the patient responded well to dasatinib treatment.

Extramedullary plasmocytoma associated with a massive deposit of amyloid in the duodenum

We report a rare case of extramedullary plasmocytoma associated with a massive deposit of amyloid in the duodenum. A 72-year-old Japanese man was admitted to our hospital presenting with a 3-mo history of epigastric pain, vomiting and weight loss. On computed tomography （CT） a wall thickening of the fourth part of the duodenum was observed. Multiple biopsies obtained from the lesion showed infiltration of plasma cells and lymphocytes, but they were not conclusive. The patient underwent resection of the lesion and, on histopathological examination, the lesion consisted of a dense and diffuse infiltrate of plasma cells and a few admixed lymphocytes with reactive follicles extending to the muscular propria. An extensive deposition of amyloid was also observed. Immunohistochemical stains revealed that a few plasmacytoid cells showed λ light chain staining, though most were κ： light chain positive. These cells also were positive for CD138 and CD56 but negative for CD20 and CD79. The findings were consistent with extramedullary plasmocytoma associated with a massive deposit of amyloid in duodenum. A subsequent workup for multiple myeloma was completely negative. The patient showed no signs of local recurrence or dissemination of the disease after 12 mo follow-up. Because of the association of plasmocytoma and amyloidosis, the patient must be followed up because of the possible systemic involvement of the neoplasm and amyloidosis in future.

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.

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.

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.

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)

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.

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.

Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis

Primary myelofibrosis(PMF) is a clonal hematopoietic stem cell disorder. It is characterized by bone marrow fibrosis, extramedullary hematopoiesis with hepatosplenomegaly and leukoerythroblastosis in the peripheral blood. The main clinical manifestations of PMF are anemia, bleeding, hepatosplenomegaly, fatigue, and fever. Here we report a rare case of PMF with anemia, small bowel obstruction and ascites due to extramedullary hematopoiesis and portal hypertension. The diagnosis was difficult to establish before surgery and the differential diagnosis is discussed.

Paraparesis induced by extramedullary haematopoiesis

We describe a case of worsening paraparesis induced by spinal cord compression at T6-T7 levels associated with compensatory extramedullary haematopoiesis from a compound heterozygote for haemoglobin E and for β-thalassemia.An emergency T3-T9 laminectomy was performed with excision of the masses and complete rehabilitation of the patient.

Clinical features and prognosis of multiple myeloma and orbital extramedullary disease:Seven cases report and review of literature

BACKGROUND Multiple myeloma(MM)complicated with extramedullary disease(EMD)has a poor prognosis and is a limiting factor in the treatment of MM,and no standard treatment is recommended in international guidelines.Few studies have reported MM with periorbital EMD.CASE SUMMARY In this paper,the clinical characteristics and survival of seven patients with multiple myeloma and orbital are described and analyzed.The common ocular symptoms were blurred vision,proptosis and/or eye movement disorders,IgG type MM may be a risk factor for orbital involvement.Of them,six patients were treated with bortezomib-based regimens.The median overall survival(OS)and progression free survival for the entire cohort were 48 and 33 mo,respectively,which was much worse than the OS reported for MM patients without orbital EMD.CONCLUSION Orbital MM may have significantly shortened survival for the entire cohort,so multidisciplinary collaboration is emphasized and recommended in the diagnosis and treatment of these difficult cases.

Clinical features of multiple myeloma patients with isolated extramedullary relapse

This study sought to analyze the clinical features and prognosis of multiple myeloma with isolated extramedullary relapse and with the absence of systemic progression.The clinical features and outcome were retrospectively analyzed in six multiple myeloma patients.These patients had secretory multiple myeloma at diagnosis.When relapsed,the dissociation between medullary and extramedullary response was detected.The serum or urine monoclonal component was extremely low or absent.The plasma cells in bone marrow were 〈5%.All patients received new targeted therapies（thalidomide or bortezomib） before extramedullary relapse.It is difficult to achieve second remission for them.Even in those showing response,the duration of response was extremely short.The median of overall survival from diagnosis and from extramedullary relapse was 19 months and 6 months,respectively.The overall survival was significantly shorter compared to the patients without extramedullary involvement（84 months,P= 0.001）.These patients exhibited a special and rare relapse pattern.Patients with this relapse pattern were resistant to current therapies,including novel targeted agents and associated with poor prognosis.

Intracranial Extramedullary Hematopoiesis in β-thalassemia Major

A case of β-thalassemia major with a huge mass of hernatopoictic tissuc firmly attached tothe dura mater was reported This is the first case reported in China.

Surgical resection of intradural extramedullary tumors in the atlantoaxial spine via a posterior approach

BACKGROUND The anatomical features of the atlantoaxial spine increase the difficulty of complete and safe removal of atlantoaxial intradural extramedullary(IDEM)tumors.Studies concerning surgical interventions via a posterior approach are limited.AIM To investigate the safety and efficacy of atlantoaxial IDEM tumor resection using a one-stage posterior approach.METHODS We retrospectively analyzed clinical databases for one-stage atlantoaxial IDEM tumor resection via a posterior approach between January 2008 and January 2018.The analyzed data included tumor position,histopathological type,pre-and postoperative Japanese Orthopedic Association(JOA)scores and Nurick grades,postoperative complication and recurrence status.RESULTS A total of 13 patients who underwent C1-C2 Laminectomy and/or unilateral facetectomy via the posterior approach were enrolled in the study.In all cases reviewed,total tumor resection and concomitant C1-C2 fusion were achieved.The average follow-up was 35.3±6.9 mo(range,26-49 mo).A statistically significant difference was noted between the preoperative JOA score(11.2±1.1)and the score at the last final follow-up(15.6±1.0)(P<0.05).A statistically significant difference was noted between the preoperative Nurick grade(2.3±0.9)and that at the last follow-up(1.2±0.4)(P<0.05).However,no statistically significant difference was noted between the preoperative and last follow-up C1-2 Cobb angle and C2-7 Cobb angle(P>0.05).No mortalities,severe complications or tumor recurrence were observed during the follow-up period.CONCLUSION Total resection of atlantoaxial IDEM tumors is feasible and effective via a posterior approach.Surgical reconstruction should be considered to avoid iatrogenic kyphosis and improve spinal stability and overall clinical outcomes.

Magnetic resonance imaging features of intrahepatic extramedullary hematopoiesis: Three case reports

BACKGROUND Extramedullary hematopoiesis rarely occurs within the liver alone,and is easily misdiagnosed.The radiological literature on this disease is exclusively case reports.There is a paucity of literature on the role of magnetic resonance imaging(MRI).The most common imaging modalities used are computed tomography and ultrasound.This report aims to provide more data on the appearance of extramedullary hematopoiesis using MRI to help radiologists establish the diagnosis.CASE SUMMARY Three patients(one male and two females)were incidentally found to have a hepatic mass or nodule,without hepatomegaly or splenomegaly.Laboratory tests including liver function,serum hepatic tumor markers,and hepatitis serologic markers were normal.On MRI scans,all lesions showed lower signal intensity on in-phase images than on out-phase images.One case showed changes in signal intensity on T2 weighted images(WI)and diffusion WI,which shifted from hyperintensity to hypointensity with size enlargement between two rounds of imaging examination.These lesions exhibited different enhancement patterns on dynamic contrast enhancement series.CONCLUSION The MRI signal change and in-/out-phase image might provide useful information and help radiologists establish the diagnosis of intrahepatic extramedullary hematopoiesis.

Surgical treatment of tracheal extramedullary plasmocytoma and pertinent literature review

Objective: To study the diagnostic method, surgical management and complications of the tracheal malignant tumor, as well as the characteristics of plasmocytoma. Methods: One patient with tracheal plasmocytoma and pertinent literature were analyzed retrospectively. Results: Resection of the tracheal tumor and reconstruction of the trachea were performed successfully in this case, and the pathological diagnosis is plasmocytoma. Conclusion: The nature, location and extent of tracheal tumor are precisely determined from radiologic studies before resection, as well as tracheal intubation and tracheal anastomosis without tension in operative procedures, are very important for triumphal operation. Even extramedullary plasmocytoma may occur in a lot of organs, but the incidence of it is rare. Both surgery and radiotherapy are cardinal methods for extramedullary plasmocytoma.

Intestinal perforation with abdominal abscess caused by extramedullary plasmacytoma of small intestine: A case report and literature review

BACKGROUND Extramedullary plasmacytoma(EMP)of the gastrointestinal tract is an extremely rare disease.Clinical manifestations of EMPs are varied and depend on the location and progression of the tumor.CASE SUMMARY Here,we firstly report a case of intestinal perforation with abdominal abscess caused by EMP of the small intestine in a 55-year-old female patient.The patient received emergency surgery immediately after the necessary preoperative procedures.During the operation,EMP was found to have caused the perforation of the small intestine and the formation of multiple abscesses in the abdominal cavity.Partial resection of the small intestine with peritoneal irrigation and drainage was performed.EMP was finally confirmed by postoperative histopathology and laboratory tests.Additionally,we performed a literature review of gastrointestinal EMP to obtain a deeper understanding of this disease.CONCLUSION EMP of the small intestine may have spontaneous perforation,which requires emergency surgery.Surgical resection can obtain good therapeutic effects.

Renal Extramedullary Plasmacytoma--One Case Report

Extramedullary plasmacytoma （EMP） is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.

Extramedullary Hematopoiesis Mimicking a Neoplasm in a Goeldi＇s Monkey （Callimico goeldii）

Extramedullary hematopoiesis consists in the appearance and proliferation of hematopoietic cells outside the bone marrow. In this article, the authors describe a case of hepatosplenic hematopoiesis in a 9-year-old, male Goeldi＇s monkey concurrent with a Calodium hepaticum infestation, belonging to the Lisbon＇s Zoo primate collection （Portugal）. Lesions were identified upon necropsy after euthanasia due to the presence of an apparently non-excisable, metastatic aortic mass. Histopathological analysis of samples taken was carried out and immunohistochemical staining was used to characterize the cellular population involved, confirming the diagnosis of extramedullary hematopoiesis. To the best of the authors＇ knowledge, this is the first report of hepatosplenic extramedullary hematopoiesis in a Goeldi＇s monkey.

Bone-marrow derived cells participate in extramedullary hematopoiesis in a model of acetaminophen-induced acute liver failure in rats

Adult derived mononuclear bone marrow cells are a good alternative as cell therapy. These cells are capable of significantly improve survival rate of Wistar rats with acetaminophen (APAP) induced acute liver failure in ten days. However, long term of cell therapy is not deeply studied in the literature. Here, we report an extramedullary hematopoiesis process derived from transplanted mononuclear bone marrow cells in the liver of rats 10 days after APAP injection. This result indicates that liver maintains an adequate microenvironment for the occurrence of extramedullary hematopoiesis process. The consequence of this finding deserves more studies.