|Year : 2015 | Volume
| Issue : 3 | Page : 662
Plasmacytoma rectum extending to para-rectal region
Mehul Hamjibhai Gohil, Devang C Bhavsar, U Suryanarayana, Dhaval H Jetly
Department of Radiotherapy, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
|Date of Web Publication||9-Oct-2015|
Mehul Hamjibhai Gohil
Department of Radiotherapy, Gujarat Cancer and Research Institute, Civil campus, Asarwa, Ahmedabad - 380 016, Gujarat
Source of Support: None, Conflict of Interest: None
Solitary plasmacytoma most commonly presents in bone (80%), while extramedullary plasmacytoma (EMP) is an uncommon (20%) entity that most commonly involves nasopharynx or upper respiratory tract. Involvement of the gastrointestinal tract occurs in approximated 10% of EMP cases, in that also solitary EMP of the rectum is an exceedingly rare entity. Here we are presenting a case of 55-year-old male patient who was diagnosed to have EMP of rectum in September 2012 who was initially operated in which de-bulking surgery with abdominoperineal resection was done with large residual lesion postoperatively. Then patient was treated with postoperative radiotherapy (RT) with conventional portals 40 Gy in 20 fractions with complete response at 3 months. Patient was followed-up until now (1 year and 2 months post-RT), without any symptom or recurrence.
Keywords: Extramedullary plasmacytoma, radiotherapy, rectum
|How to cite this article:|
Gohil MH, Bhavsar DC, Suryanarayana U, Jetly DH. Plasmacytoma rectum extending to para-rectal region. J Can Res Ther 2015;11:662
| > Introduction|| |
Plasma cell neoplasms account for 22% of all mature B-cell neoplasms. The majority of plasma cell neoplasms are multiple myeloma, with solitary plasmacytoma accounting for ≤6% of cases.  Whereas extramedullary plasmacytoma (EMP) is even rare. Involvement of the gastrointestinal tract occurs in approximated 10% of EMP cases, while solitary EMP of the rectum is an exceedingly rare entity. Here, we present a rare case of plasmacytoma of rectum.  Only six cases have been reported in the literature till now and last case report was published in 1994 by Pais et al.
| > Case report|| |
A 55-year-old male patient initially presented with pain in the perineal region while sitting or driving, which was followed by altered bowel habit and difficulty in defecation with no any significant past medical or surgical history. General physical examination and systemic examination was unremarkable. Digital per rectal examination revealed indurated posterior wall of rectum and upper part of the anal canal with vague mass felt at the tip of the finger. Preoperative magnetic resonance imaging of pelvis revealed 11 cm × 9 cm × 14 cm lesion posterior in pelvis involving the posterior wall of rectum and anal canal without any lymphadenopathy [Figure 1]. Computed tomography (CT) guided biopsy showed leiomyoma/leiomyosarcoma? (immunohistochemistry [IHC] was difficult due to scanty tissue).
|Figure 1: Pretreatment magnetic resonance imaging showing large mass in the rectum|
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Preoperative complete blood counts, renal and liver function tests, serum carcinoembryonic antigen level, urine examination and chest X-ray were normal. The patient was operated outside in the private hospital where abdominoperineal resection was done, gross examination of which shown a huge pararectal fleshy mass in the lower segment of bowel in rectum measuring 13 cm × 8 cm size. Histopathology was suggestive of plasmacytoma with CD138 (strong), and vimentin were positive markers, while cytokeratin, leucocyte common antigen, desmin, s-100 and CD117/c-kit were negative. After this, patient was referred to Gujarat Cancer Research Centre, Ahmedabad, where slides were reviewed with IHC, which also confirmed the disease as plasma cell myeloma anaplastic. Skeletal survey, bone marrow biopsy, urine examination for bence-jones proteins were done, which were normal/negative and ruled out multiple myeloma. Serum free kappa, serum free lamda and serum beta-2 microglobulin were marginally elevated. All these reports confirmed that the patient had plasmacytoma rectum extending to pararectal region. The postoperative CT scan [Figure 2] showed 60 mm × 48 mm × 58 mm sized residual lesion seen on the right side in pelvis.
|Figure 2: Postoperative computed tomography scan showing large residual lesion in the rectum|
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The patient then received adjuvant postoperative radiotherapy (RT) (40 Gy in number 20) to pelvis by anterior posterior/posterior anterior portals with 6 MV photons using a linear accelerator with 2 Gy per fraction for 5 days a week for 1 month. Follow-up ultrasonography on 1 st month was normal with no evidence of residual disease, while follow-up CT scan on the third month also shown no evidence of disease with post-RT changes [Figure 3].
|Figure 3: Follow-up computed tomography scan showing postradiotherapy changes without evidence of disease|
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Review of reported case in literature
To our knowledge, only six cases of primary EMP of rectum has been reported till now. First reported case was a postmortem report of a male with multiple lesions in the ilium, colon and rectum by Brown and Liber in 1939.  The second patient was 41-year-old female who previously had a gastric EMP, treated with RT with concurrent nitrogen mustard. The rectal lesion occurred 28 months later and was treated by segmental resection and no recurrence on 12 months follow-up.  Sharma and Shrivastav had reported the third patient of plasmacytoma of rectum in 1961 who was treated by abdominoperineal resection of the rectum.  The fourth and fifth cases were reported by Price et al. in 1987, and the patients were treated by RT and they showed good response to therapy.  The sixth case was an 87-year-old female who was treated by endoscopic polypectomy and RT, who was followed-up for 12 months without any evidence of recurrence and was reported by Pais et al. in 1994. 
| > Conclusion|| |
Extramedullary solitary plasmacytoma is a radiosensitive tumor and can be treated with curative RT. As in this case, surgery followed by adjuvant RT can be advocated. However, longer follow-up and larger number of patients is needed to interpret the result and survival and to determine the proper line of management.
| > References|| |
Hodgson DC, Mikhael J, Tsang RW. Plasma cell myeloma and plasmacytoma. In: Halperin EC, Perez CA, Brady LW, editors. Perez and Brady's Principles and Practice of Radiation Oncology. 5 th
ed. 530 Walnut Street Philadelphia, PA 19106 USA: Lippincott Williams and Wilkins; 2008. p. 1790-800.
Gupta V, Nahak B, Sakhuja P, Agarwal AK, Kumar N, Mishra PK. Primary isolated extramedullary plasmacytoma of colon. World J Surg Oncol 2007;5:47.
Brown CR, Liber AF. Multiple plasmoma of ileum and colon. Arch Pathol 1939;28:112-3.
Hampton JM, Gandy JR. Plasmacytoma of the gastro-intestinal tract. Ann Surg 1957;145:415-22.
Sharma KD, Shrivastava JD. Extramedullary plasmacytoma of the gastrointestinal tract. Arch Pathol 1961;71:229-33.
Price A, Quilty PM, Ludgate SM. Extramedullary plasmacytoma of the rectum: Two cases treated by radiotherapy. Clin Radiol 1987;38:283-5.
Pais JR, García-Segovia J, Rodríguez-Garcia JL, Alvarez-Baleriola I, García-González M. Solitary plasmacytoma of the rectum: Report of a case treated by endoscopic polypectomy and radiotherapy. Eur J Surg Oncol 1994;20:592-4.
[Figure 1], [Figure 2], [Figure 3]