Treatment of Extramedullary Plasmacytoma of the Rectum with Endoscopic Mucosal Resection: A Case Report and Literature Review

doi:10.21203/rs.3.rs-1668309/v3

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Case Report

Treatment of Extramedullary Plasmacytoma of the Rectum with Endoscopic Mucosal Resection: A Case Report and Literature Review

https://doi.org/10.21203/rs.3.rs-1668309/v3

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Background Extramedullary plasmacytoma (EMP) is an unusual plasma cell disease.We report a case of EMP arising in the rectum, which is fully removed using endoscopic mucosal resection (EMR).

Methods The patient’s clinical, endoscopic, and histologic data were reviewed, as well as the past literature of rectal EMP with a focus on the clinical presentation, treatment and outcome.

Results A 64-year-old woman was referred to our hospital with recurrent diarrhea for longer than half a month. She underwent colonoscopy which revealed a polypoid lesion. The lesion was fully excised using EMR, and histopathological examination coupled with immunohistochemistry of the endoscopic biopsy confirmed a primary EMP of the rectum. She did not receive radiotherapy or chemotherapy after being diagnosed with EMP and experienced no recurrence or progression during the 6-month follow-up period.

Conclusions Rectal EMP is an extremely rare clinical entity, Only 14 cases have been reported in the English-language literature as far as we know. Clinical suspicion is infrequent owing to its location and nonspecific manifestations. The diagnosis of EMP requires biopsy-proven monoclonal plasma cell infiltration of a single lesion, and the exclusion of multiple myeloma. Although EMP is a highly radiosensitive tumor, endoscopic therapy is increasingly becoming a good option for colorectal EMP when complete endoscopic resection of the tumor is possible. Although rectal EMP has an indolent course and favorable prognosis, long-term follow-up is recommended due to the possibility of relapse or progression to multiple myeloma.

Extramedullary plasmacytoma

Rectum

Endoscopic mucosal resection

Case report

Plasma cell neoplasms are mature B-cell malignancies characterized by monoclonal dysproliferation of plasma cells and immunoglobulin production via clonal expansion. Plasma cell tumors are classified into two main groups, namely multiple myeloma (MM) and plasmacytoma. The World Health Organization stipulates that plasmacytoma includes solitary plasmacytoma of the bone and solitary extramedullary plasmacytoma (EMP) [1]. EMP is an uncommon entity that involves organs outside the bone marrow, and is characterized by localized infiltration of neoplastic plasma cells and no evidence of systemic involvement, accounting for approximately 3-4% of all plasma cell tumors [2, 3]. EMP can originate in any site of the body, with about 90% of cases involving the head and neck, but only 5% involve the gastrointestinal (GI) tract [4]. Rectal EMP is extremely rare, herein, we present a case of rectal EMP that was successfully treated using endoscopic mucosal resection (EMR).

The patient was a 64-year-old woman who experienced recurrent diarrhea for longer than half a month, excreting yellow and loose stools three times a day. Colonoscopy was performed at our outpatient department on 25th August, 2021, and a rectal pedicled polypoid lesion measuring approximately 15 mm in size was discovered (Fig. 1a). She was admitted to our department for further management of the rectal lesions. Owing to the small size of the polyp-like tumor and relatively narrow base, the patient underwent EMR after providing informed consent for complete excision of the lesion (Fig. 1b-d). Microscopic biopsy of the resected rectal polyp revealed massive infiltration of tumor plasma cells. Immunohistochemical staining (Fig. 2b-c) revealed the following: CD38 (+), CD79a (+), Mum - 1 (+), IgG (+), kappa (+), Ki - 67 (about 50%), as well as CD20 (-), CD3 (-), CD5 (-), Cyclin D1 (-), CD45 (-), CD56 (-), lambda (-). An in situ hybridization assay showed EBER (-) (Fig. 2d). This finding is consistent with the immunohistochemical profile of plasmacytoma, subsequent normal bone marrow aspiration and whole-body bone scan results further excluded MM. General physical examination and systemic examination was normal, chest computed tomography and laboratory studies, such as those of blood and urine immunofixation electrophoresis, urine light chain assay, immunoglobulins, complete blood counts, and urine examination were all unremarkable. Therefore, the final diagnosis in this case was primary plasmacytoma of the rectum. The patient was treated with simple rectal EMR, no radiotherapy or chemotherapy was administered as complete endoscopic resection had been performed, and no recurrence or progression was observed at the 6-month follow-up.

Extramedullary plasmacytoma localized in the rectum is particularly rare, to our knowledge,only 14 reports exist in the English-language literature. These cases are reviewed and patient data analyzed regarding age, gender, clinical presentation, treatment and outcome (Table 1) [5-16].

The clinical symptoms of GI EMP vary with tumor location and depth of invasion and can manifest as nonspecific abdominal pain, abdominal mass, GI bleeding, vomiting, changes in bowel habits, intestinal obstruction, and intussusception [6]. Rectal bleeding is the most common symptom of rectal EMP [7]. In the present case, the patient manifested a change in bowel habits. Symptoms in patients with GI EMP are nonspecific and may be misdiagnosed as other GI conditions, such as cancer, stromal tumor, lymphoma, metastases, or inflammatory bowel disease [17]. Therefore, we cannot make a reliable initial diagnosis based on the clinical presentation.

The initial diagnostic techniques for EMP vary by tumor location. In cases of GI EMP, a definitive histological diagnosis is usually obtained following endoscopy with biopsy or surgical resection of the tumor. Immunohistochemistry confirmed the presence of monoclonal plasma cells expressing CD138 and/or CD38, CD79a, as well as kappa or lambda light chain restriction in EMP. These characteristics are very similar to those of MM in terms of immunophenotype, however, the absence of evidence on peripheral plasmacytosis or clonal myeloid on bone marrow biopsy can facilitate the exclusion of MM and other types of plasma cell neoplasms [18]. The accurate diagnosis of GI EMP relies on histopathological analysis, which can be challenging and requires input from experienced pathologists.

No guidelines for the treatment of GI EMP have been established because of its extreme rarity. Although EMP is a highly radiation-sensitive tumor with a local control rate of approximately 80–100% [19], surgery alone is preferred if EMP is present in local soft tissues and complete surgical resection of the tumor is possible [3]. Patients receiving combined therapy (surgery and radiotherapy) either had tumors in critical areas or had inadequate surgical margins due to unclear diagnosis at the time of surgery [20]. Adjuvant radiotherapy is not recommended for patients who undergo complete surgical resection and have negative margins. However, patients with surgical margin involvement should receive adjuvant radiotherapy, whereas combined treatments are recommended if complete surgical resection of the tumor is not possible and/or the lymph node area is affected [21]. Endoscopic treatments, such as endoscopic submucosal dissection and EMR, are becoming increasingly viable treatment options for colorectal EMP [9, 10, 22].

EMP usually has an indolent course and the prognosis is generally favorable, with a 15-year survival rate of 78%. Approximately 15% of patients progress to MM, especially during the first three years after diagnosis [23]. In contrast, only 4.9% of cases of small-bowel EMP progressed to MM. This also usually occurs 5–10 years after resection; therefore, continuous monitoring is usually recommended for patients with EMP [24].

Here, we report a case of rectal EMP, which is a peculiar clinical entity that presents diagnostic challenge due to its infrequent location and nonspecific symptoms. Furthermore, we discuss the clinical manifestations, diagnosis, treatment and outcome of patients with GI EMP. Early diagnosis and timely intervention are crucial, and surgical resection is the ideal choice for GI EMP. Although EMP is a highly radiosensitive tumor, endoscopic therapy is increasingly becoming a good option for colorectal EMP when complete endoscopic resection of the tumor is possible. Generally, GI EMP has favorable prognoses and outcomes, but occasionally it may progress to MM, or relapse. Therefore, the patients of GI EMP should be followed-up for a long time.We hope that the endoscopic and histopathological findings and treatment of solitary rectal EMP we present will be valuable for the management of similar manifestations of EMP in other patients.

Ethics approval and consent to participate Not applicable.

Consent for publication The authors affirm that the patient has consented to the submission of the case report to the journal. Moreover, the authors affirm that the patient provided informed consent for publication of the images in Fig. 1 to 2.

Conflict of interest The authors declare that they have no competing interests.

Funding None.

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Table 1 is available in the supplementary files.

No competing interests reported.

Table1.xlsx

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Treatment of Extramedullary Plasmacytoma of the Rectum with Endoscopic Mucosal Resection: A Case Report and Literature Review

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Case Report

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