Primary extramedullary plasmacytoma of the sigmoid colon with perforation: a case report
© The Author(s). 2018
Received: 1 August 2017
Accepted: 22 March 2018
Published: 4 April 2018
Extramedullary plasmacytomas account for 4% of all plasma cell tumors and occur mainly in the upper respiratory tract; gastrointestinal system involvement is rare. Extramedullary plasmacytoma of the colon with perforation has not been reported.
A 77-year-old woman with a 1-year history of lower abdominal pain and nausea was admitted to our hospital. An abdominal computed tomography scan revealed a sigmoid tumor with perforation. The patient underwent emergency surgery. Pathological examination led to a diagnosis of plasmacytoma of the colon. The patient did not undergo postoperative adjuvant chemotherapy. She has had no recurrence in 14 months of regular follow-up.
We have herein described a rare case of extramedullary plasmacytoma of the gastrointestinal tract with perforation involving the sigmoid colon.
A plasma cell tumor is an immunoproliferative monoclonal disease of the B cell line that originates from malignant transformed plasma cells. Plasmacytoma includes solitary plasmacytoma of bone and solitary extramedullary plasmacytoma.
Solitary extramedullary plasmacytoma has been rarely reported, and its natural history and diagnosis are unclear. Most such plasmacytomas occur in the nasopharynx or upper respiratory tract; only 10% of reported cases have involved the gastrointestinal tract. The stomach and small intestine are the most commonly involved sites in the gastrointestinal tract [1–3]. Primary isolated extramedullary plasmacytoma of the colon is extremely rare. No previous reports have described plasmacytoma of the colon with perforation. We herein report a rare case of primary isolated extramedullary plasmacytoma of the colon with perforation and describe the patient’s postoperative clinical course.
360 × 104/μL
21.4 × 104/μL
Postoperatively, the patient was discharged without any complications. She did not undergo postoperative adjuvant chemotherapy and has had no recurrence in 14 months of regular follow-up.
Extramedullary plasmacytoma accounts for only 3 to 5% of all plasma cell diseases. These tumors may be solitary or may precede, accompany, or follow the onset of multiple myeloma. Solitary extramedullary plasmacytoma has rarely been reported, and its natural history and diagnosis are unclear. Diagnosis of solitary extramedullary plasmacytoma requires the exclusion of associated multiple myeloma, which is determined by the absence of Bence-Jones protein in the urine, normal serum electrophoresis, and normal bone marrow biopsy . Our present case met these criteria.
Alexiou et al.  reported that extramedullary plasmacytoma most often occurs in the nasopharynx or upper respiratory tract (82.2%). Only 17.8% of cases involve the gastrointestinal tract. The stomach and small intestine are the most commonly involved sites in the gastrointestinal tract. Primary isolated extramedullary plasmacytoma of the colon is extremely rare, occurring in only 0.028% of cases . Therefore, its clinical features and prognosis are not well known.
Well-documented cases of plasmacytoma of the colon
Vasiliu and Popa/1928
Anorexia, epigastric pain, glandular enlargement
Brown and Liber/1939
Hampton and Gandy/1957
Rectal pain and bleeding
Incidental operative finding
Pain, nausea, vomiting
Pain, rectal bleeding
Transverse colon resection
Weight loss, anemia, pain, fecal occult blood
Hepatic flexure of the colon
Extended right hemicolectomy
Pain, rectal bleeding, asthenia
Segmental resection of the left colon
Dysuria, abdominal pain
Sigmoid colon resection
Right hemicolectomy, lymph node dissection, excision of Gerota’s fascia, partial resection of the posterior portion of the liver
Diarrhea, pain, rectal bleeding
Fecal occult blood
Endoscopic submucosal resection
Cecum and rectum
Endoscopic submucosal resection
Extended left hemicolectomy
Pain and weakness
Left hemicolectomy and small intestinal resection
Perforation during diagnostic colonoscopy
Fatigue, light-headedness, dyspnea, dark stool
Right hemicolectomy Distal ileal resection
In the present case, we were unable to determine the cause of the perforation by pathologic examination. We consider that the tumor was necrosed and perforated; otherwise, as the tumor grew, the intestinal internal pressure increased, resulting in perforation of the sigmoid colon.
Postoperative chemotherapy has no effect on the course of extramedullary plasmacytoma. Our patient did not undergo postoperative adjuvant chemotherapy, and she has had no relapse to date. However, careful follow-up is required.
Because primary isolated extramedullary plasmacytoma in the colon is very rare, the clinical course, treatment guidelines, and prognosis remain unclear. Further study of the clinical features of primary isolated extramedullary plasmacytoma of the colon is necessary to ensure that adequate treatment is administered.
We have described a rare case of extramedullary plasmacytoma of the gastrointestinal tract with perforation of the sigmoid colon. In this case, the prognosis was good because of appropriate treatment involving early surgery.
The authors would like to thank Angela Morben, DVM, ELS, from Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.
This study did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
FK wrote the final manuscript and performed the literature search. KD supervised the writing of the manuscript. HI and TO performed the surgery. All authors read and approved the final manuscript.
Consent for publication
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