• case report
• extramedullary plasmacytoma
• multiple myeloma
• radiotherapy
• surgery

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.

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.

EMP of the small intestine may have spontaneous perforation, which requires emergency surgery. Surgical resection can obtain good therapeutic effects.

• Extramedullary plasmacytoma
• Perforation
• Small intestine
• Gastrointestinal tract
• Treatment
• Case report

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.

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 (PubMed 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.

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.

• Gastrointestinal bleeding
• Extramedullary plasma cell neoplasm
• Plasmablastic myeloma
• Multiple myeloma
• Extramedullary plasmacytoma
• Case report

• case report
• extramedullary plasmacytomas
• trachea

• Humans
• Male
• Middle Aged
• Plasmacytoma/diagnosis
• Plasmacytoma/therapy
• Tracheal Neoplasms/diagnosis
• Tracheal Neoplasms/therapy

•

No general treatment guidelines have been established for gastricplasmacytoma.

Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm that presents as a solitary tumor. EMP in the gastrointestinal organs are extremely uncommon.

A 36-year-old man was admitted to our hospital with advanced anemia. He had no specific medical history. Gastroendoscopic findings showed an 8.0-cm submucosal tumor with ulcer on the greater curvature of the gastric body. Fine-needle aspiration was performed, and the pathologic diagnosis of the submucosal tumor was a plasmacytoma. Therefore, the patient was diagnosed with gastric plasmacytoma. A total gastrectomy was performed with lymphadenectomy. The result of intraoperative peritoneal lavage cytology was positive. Histological examination revealed serosa-exposed plasmacytoma of the stomach with lymph nodes metastasis. Additionaly the patient received a three-drug chemotherapy regimen (bortezomib, cyclophosphamide, and dexamethasone [VCD]) from 3 weeks after the operation. After 4 cycles of chemotherapy, the patient received autologous peripheral blood stem-cell transplantation (auto-PBSCT). Eighteen months after diagnosis, the patient is in complete remission with no evidence of local relapse or evolution to multiple myeloma.

This is the first reported case of advanced gastric plasmacytoma using adjuvant chemotherapy involving bortezomib and auto-PBSCT after the resection, and the patient has maintained a good course over a year. This protocol could be a new way to treat these tumors.

• Chemotherapy
• Plasmacytoma
• Stomach
• Surgical resection

• Extramedullary plasmacytoma (EP)
• Multiple myeloma (MM)
• Solitary bone plasmacytoma (SBP)

Extramedullary plasmacytoma is a plasma cell neoplasm that presents as a solitary lesion in soft tissue. Most extramedullary plasmacytomas involve the nasopharynx or upper respiratory tract. Primary plasmacytoma of the stomach is extremely rare.

A 78-year-old Korean woman presented with epigastric pain for 3 months. She had a history of an intractable gastric ulcer despite repeated endoscopic biopsies and appropriate medical therapy for the ulcer. She underwent another endoscopy and a biopsy was performed for multiple large and deep specimens. Ultimately, primary gastric plasmacytoma was confirmed. However, she and her attendant refused standard local radiotherapy or surgical resection. She came to our emergency room 3 months later with hematemesis due to a large gastric ulcer, despite management with medication for over 3 months at a local clinic. We again recommended local radiation or surgical resection. However, as she was willing to undergo only medical therapy, she was prescribed high-dose dexamethasone. Surprisingly, her ulcer completely regressed and remission was maintained for over 1 year.

We report successful treatment of a rare primary gastric plasmacytoma mimicking intractable ulcer by using high-dose dexamethasone. To the best of our knowledge, this is the first reported case successfully treated with only high-dose dexamethasone.

• Plasmacytoma
• dexamethasone
• gastric cancer
• gastric ulcer

Gastrointestinal lymphomas represent up to 10% of gastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies are mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma. However, the gastrointestinal tract can be the site of rarer lymphoma subtypes as a primary or secondary localization. Due to their rarity and the multifaceted histology, an endoscopic classification has not been validated yet. This review aims to analyze the endoscopic presentation of rare gastrointestinal lymphomas from disease diagnosis to follow-up, according to the involved site and lymphoma subtype. Existing, new and emerging endoscopic technologies have been examined. In particular, we investigated the diagnostic, prognostic and follow-up endoscopic features of T-cell and natural killer lymphomas, lymphomatous polyposis and mantle cell lymphoma, follicular lymphoma, plasma cell related disease, gastrointestinal lymphomas in immunodeficiency and Hodgkin’s lymphoma of the gastrointestinal tract. Contrarily to more frequent gastrointestinal lymphomas, data about rare lymphomas are mostly extracted from case series and case reports. Due to the data paucity, a synergism between gastroenterologists and hematologists is required in order to better manage the disease. Indeed, clinical and prognostic features are different from nodal and extranodal or the bone marrow (in case of plasma cell disease) counterpart. Therefore, the approach should be based on the knowledge of the peculiar behavior and natural history of disease.

• Endoscopy
• Lymphoma
• Endosonography
• Stomach
• Intestine

• Endoscopic submucosal dissection
• Plasmacytoma

• Endoscopic submucosal dissection
• Extramedullary
• Plasmacytoma

• Aged
• Aged, 80 and over
• Female
• Humans
• Immunosuppressive Agents/administration & dosage
• Immunosuppressive Agents/adverse effects
• Male
• Middle Aged
• Multiple Myeloma/drug therapy
• Multiple Myeloma/metabolism
• Multiple Myeloma/pathology
• Recurrence
• Thalidomide/administration & dosage
• Thalidomide/adverse effects
• Treatment Outcome

• Adult
• Aged
• Diagnosis, Differential
• Endoscopy, Gastrointestinal
• Female
• Gastrointestinal Neoplasms/diagnosis
• Gastrointestinal Neoplasms/epidemiology
• Humans
• Male
• Middle Aged
• Multiple Myeloma/complications
• Plasmacytoma/diagnosis
• Plasmacytoma/epidemiology