髓外浆细胞瘤11例临床分析

髓外浆细胞瘤(Extramedullary plasma-cytoma,EMP)是指原发于骨髓以外的器官和组织的恶性浆细胞病。近年来国外仅有些零星报告。我院10余年经手术后病理证实 EMP11例。就临床表现、诊断、分期进行了讨论,现报告如下:     

鼻及鼻咽部髓外浆细胞瘤6例

<正>浆细胞瘤是一组来源于B淋巴细胞,以单克隆性浆细胞异常增生并分泌大量单克隆免疫球蛋白为特点的罕见恶性肿瘤,包括多发性骨髓瘤(MM)、孤立性骨髓瘤(SPB)和髓外浆细胞瘤(EMP)〔1〕。其中EMP约占所有浆细胞肿瘤4%,可发生于全身任何髓外的组织和器官,但90%发生于头颈部,尤其是上呼吸道〔2〕。为提高对本病的认识,本文对我院2008年3月至2013年12月收治的6例鼻及鼻咽部EMP患者的临床及病理     

气管髓外浆细胞瘤一例

髓外浆细胞瘤(extramedullary plasmacytoma,EMP)是骨髓瘤的一种变异形式,是原发于骨髓造血组织以外的浆细胞实体肿瘤,占浆细胞肿瘤的3%~5%,多发生在头颈部或上呼吸道,但也可发生在肺、消化道和颅内[1],在气管内很罕见。临床症状及影像学检查结果与其他原发性或转移性气管肿瘤难以鉴别,易误诊,确诊主要依靠组织病理学检查。两年半前我科诊断1例气管髓外浆细胞瘤患者,经支气管镜下介入局部切除术后复发,现报告如下。     

直肠孤立性髓外浆细胞瘤及文献回顾

孤立性髓外浆细胞瘤（solitary extramedullary plasmacytoma，SEP）由Schridde在1905年首先报道，临床上非常少见。多发生于淋巴组织较丰富的头颈部。而在直肠的SEP极为罕见。我院1995年2月至2005年10月共收治4例直肠SEP，结合文献回顾，探讨直肠SEP的临床特点、诊断和治疗。     

多发孤立性浆细胞瘤伴维生素D缺乏

多发孤立性浆细胞瘤(MSP)发病率低,相关报道罕见。本文报道1例63岁女性患者,右下肢针刺样疼痛2月余,伴麻木、乏力。实验室检查提示高甲状旁腺激素血症,血清骨源性碱性磷酸酶升高,维生素D缺乏。神经肌电图显示右下肢神经源性损害。骶椎、骨盆CT增强提示骶1椎体右侧见软组织团片灶,呈膨胀性骨质破坏,骨皮质破裂。18 F-FDF PET/CT显示胸骨、胸、腰椎椎体、左侧髂骨及右侧骶骨骨质破坏影。CT引导下经皮穿刺活检免疫组化显示浆细胞骨髓瘤、CD138(+)、MUM-1(+)、Lambda(+)。结合骨髓涂片等结果该病例诊断为多发孤立性浆细胞瘤。     

成人髓母细胞瘤多发骨转移1例

1 病历摘要 患者,男,因髓母细胞瘤(MB)术后1年,放疗后7个月余,右侧臀部疼痛2个月入院.该患2011年8月因头晕、恶心及呕吐1个月就诊于我院,入院时意识清楚,查体配合,颈软,双侧瞳孔等大等圆,对光反射灵敏,指鼻试验左侧欠稳准,小脑性步态,左上肢不自主抖动.核磁提示小脑上蚓部见2.92 cm×3.46 cm×4.31 cm团块样高信号,轻中度强化,中心可见斑点状无强化区.遂于2011年8月11日行腰大池引流术+幕下开颅肿瘤切除术.术中见肿瘤位于小脑蚓都,约3.0cm ×3.5 crn ×3.5 cm,质软,血运丰富,边界欠清,将肿瘤完整切除.     

多发性骨髓瘤伴肺内浆细胞瘤1例

患者 ,男 ,70岁。因浮肿、尿少伴头晕、乏力半个月于 2 0 0 0年 3月 2 4日入院。半个月前双下肢浮肿 ,尿量减少 ,每日约 80 0～ 1 0 0 0 ml,伴头晕、乏力、食欲减退 ,并逐渐加剧 ,曾在当地卫生院对症治疗 ,无好转。体格检查 :中度贫血貌 ,巩膜无黄染 ,全身浅表淋巴结无肿大 ,皮肤未见皮疹及出血点。桶状胸 ,两肺呼吸音低 ,肺底可闻及湿音 ,心率 1 0 0次 /min,律齐 ,未闻及杂音。腹部检查无特殊。四肢关节无肿大 ,无活动障碍。双下肢明显凹陷性浮肿。实验室检查 :Hb88g/L,WBC1 .9× 1 0 6/L,尿蛋白定性 ( ) ,血清总蛋白 80 .8g/L,白蛋…     

多发性骨髓瘤并双侧卵巢浆细胞瘤1例

多发性骨髓瘤(multiple myeloma,MM)是浆细胞的恶性肿瘤,骨髓瘤细胞在骨髓内克隆性增殖,引起溶骨性破坏,骨髓外肿瘤细胞形成实体肿瘤组织较少见,本文报道1例MM并发卵巢浆细胞瘤。患者女,39岁,2011年1月因劳累后出现腰痛、左下肢疼痛,行CT检查提示腰椎间盘突出,经对症治疗后症状缓解不明显。2011年2月无明显诱因     

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.     

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.     

Extramedullary plasmacytoma: report of two cases with uncommon presentation

Lymph node infiltration by monoclonal plasma cells can occur either in aggressive forms of myeloma or may represent regional extension of extramedullary plasmacytomas, whereas lymph node plasmacytoma presenting as a solitary extramedullary plasmacytoma is very unusual. We report two cases of lymph node plasmacytomas without systemic disease diagnosed after surgical excision. Clinical remission was achieved after local radiotherapy although one patient relapsed with multifocal extramedullary plasmacytomas 20 months after radiotherapy.     

Response of the extramedullary lung plasmacytoma with pleural effusion to chemotherapy

 An elderly patient with an extramedullary lung plasmacytoma and subsequent pleural effusion is described. The presence of abnormal plasma cells in the pleural fluid led to diagnosis. Histologically similar conditions such as multiple myeloma and solitary myeloma of bone were ruled out by clinical evaluation. These neoplasms usually occur in the head and neck area and are not characterized by paraprotein accumulation. Few cases in the lung have been reported. We describe a case of extramedullary plasmacytoma of the lung with plasmacytoma-induced pleural effusion and the presence of monoclonal paraprotein in both the serum and urine. Chemotherapy with melphalan was effective in reducing the size of the plasmacytoma, and pleurodesis was used to manage the pleural effusion. Received: 16 December 1996 / Accepted: 16 April 1997     

Secondary extramedullary plasmacytoma involving the pancreas

Extramedullary plasmacytoma is a rare variant of plasma cell tumor involving organs outside the bone marrow. The vast majority of extramedullary plasmacytomas present as a secondary tumor of systemic myelomatosis of the bone marrow. We experienced a patient with extramedullary plasmacytomas of the head and tail of the pancreas presenting as secondary masses from extramedullary plasmacytoma of the maxillary sinus that had been treated 5 years previously. A 38‐year‐old Japanese man had undergone radiation therapy for an extramedullary plasmacytoma of the maxillary sinus 5 years before the current presentation. He experienced severe upper abdominal pain in November 1999, when laboratory data showed elevation of the serum amylase level. Computed tomography showed two isodensity masses, in the head and tail of the pancreas. Angiography showed two hypervascular masses, one in the head and the other in the tail of the pancreas, and encasement of the portal vein trunk junction. Laparotomy was performed, with the tentative diagnosis of extramedullary plasmacytoma of the pancreas, in order to obtain a definite diagnosis. Intraoperative biopsy revealed that the two pancreatic masses were extramedullary plasmacytomas. External radiation therapy was performed after the operation. When a pancreatic mass is noticed in patients with a history of plasmacytoma, secondary extramedullary plasmacytoma of the pancreas should be considered as a differential diagnosis.     

Extramedullary plasmacytoma of the small intestine: First case report of ileocolic fistula and review of the literature

Extramedullary plasmacytoma (EMP) of the small bowel is a rare entity previously reported as a cause of intestinal obstruction or bleeding. A case report of this disease entity presenting as an ileocolic fistula is reported. EMP is diagnosed by the following critiera: 1) absence of paraproteinemia; 2) absence of Bence Jones proteinuiria; 3) normal skeletal survey; and 4) normal bone marrow biopsy specimen. Gastrointestinal plasmacytoma often occurs as a manifestation of multiple myeloma. EMP of the gastrointestinal tract is a rare cases manifestation of the disease, accounting for 13 per cent of all cases of EMP. It is a slow-spreading, radiosensitive tumor with a high tendency toward local recurrence. Surgical excision combined with radiotherapy is the treatment of choice for EMP of the gastrointestinal tract.     

Multiple myeloma with primary amyloidosis presenting with digestive symptoms: A case report and literature review

We present a case of multiple myeloma with primary systemic amyloidosis presenting with digestive symptoms in a 32-year-old male. Initial symptoms included upper abdominal discomfort for 4 months, and stool with mucous and blood for 1 month. Erosive gastritis, Helicobacter pylori infection, haematochezia, and weight loss were noted, but without bone pain, anaemia, or hypercalcaemia. Bone marrow examination showed 18.5% mature monoclonal plasma cells that were λ light chain protein and CD38 positive. Three courses of 28-day PTD therapy (i.e., bortezomib, dexamethasone, and thalidomide) were administered. Gastrointestinal symptoms and laboratory parameters improved. Post-treatment follow-up showed 0.5% plasma cells with normal morphology in bone marrow, urine λ light chain 10.1 mg/L, and negative M protein. Nevertheless, the patient died of multiple organ system failure 8 months after treatment.ConclusionsAmyloidosis is an uncommon finding in patients with multiple myeloma, especially in younger individuals.     

Solitary extramedullary plasmacytoma in the retroperitoneum

Solitary extramedullary plasmacytoma is an uncommon neoplasm and occurs most frequently in the upper respiratory tract. Herein, we reported a solitary extramedullary plasmacytoma in the retroperitoneum. A 28-year-old man presented with obstructive jaundice and a retroperitoneal tumor. Ultrasound-guided biopsy confirmed that the lesion was a plasma cell neoplasm. A detailed investigation showed that no other sites were involved. The tumor got a moderate reduction following local irradiation, and a complete remission was achieved after 12 courses of adjuvant chemotherapy. Therefore, the possibility of a solitary extramedullary plasmacytoma should be considered in the differential diagnosis of obstructive jaundice without a history of multiple myeloma. Am. J. Hematol. 58:235–238, 1998. © 1998 Wiley-Liss, Inc.     

消化道多发黄色瘤1例

胃是黄色瘤高发部位,近年来,食管、十二指肠、结直肠甚至咽喉部等单一部位黄色瘤有见报道,但食管、胃、十二指肠、结肠同时受累较为罕见。本文就1例食管胃肠多发、胃部呈连续性大片状、全胃受累的黄色瘤病例进行报道。