Management of rectal inflammatory myofibroblastic tumor... : Journal of Cancer Research and Therapeutics (original) (raw)

INTRODUCTION

Inflammatory myofibroblastic tumor (IMT) is a rare, solid tumor found pre-dominantly in children and adolescents.[1] The treatment of IMT is controversial for its rarity. In the past, talking about IMTs was mainly case reports, commonly seen in lung, mesentery, liver, and spleen. However, there are two case discussions seen in children without recurrence after initial surgery. Here, we report the successful management of rectal IMT recurrence in an adult patient with chemo-radiation and non-steroidal anti-inflammatory drugs (NSAID).

CASE REPORT

A 36-year-old male patient with hematochezia, tenesmus, and constipation presented about 3 years ago. Colonoscopy revealed a soft tissue mass of rectum about 3 cm from anus. Computed tomography (CT) showed no metastasis on radiologic examination.

Procedure

Segmental resection of rectum with the tumor was performed and the bowel was anatomized in an end-end-fashion. Macroscopically, the lesion was rubbery with a size of 5 cm × 5 cm × 4 cm. Histologically, the tumor is composed of spindle-shaped cells with proliferation and intense inflammatory infiltration pre-dominantly of plasma cells and lymphocytes [Figure 1]. The spindle-shaped cells consisted of active karyokinesis and some conspicuous nucleoli. Lymph nodes were free of disease. The immunohistochemical analysis showed positive expression of ALK-1, P53, MSA, SMA, and Ki-67 (20%). Eighteen months after the treatment, the patient experienced symptoms of tenesmus and frequent tools, which revealed a mass on per-rectal digital examination. Abdominal magnetic resonance imaging and CT scan revealed a rectal anastomotic thickening and an irregular mass in the pre-sacral space [Figure 2]. During the surgery, this solid mass measuring 5 cm × 3 cm × 3 cm in size was found invading the pelvic floor muscles, sacrococcyx, pre-sacral fascia, and diffused into the end of the rectum. Hence, the tumor was palliatively removed with complete resection of the coccyx and the fifth sacrum while preserving the anus. Post-operative biopsy was confirmatory for IMT recurrence. The recurrent tumor was treated with a three-dimensional conformal radiotherapy with a total dose of 66 Gy/33f/45d, 2 Gy/f, 5f/w. Pelvic lymphatic drainage area was not included in the radiation field. Chemotherapy was initiated with two courses of Cisplatin (75 mg/m2) and Epirubicin (50 mg/m2) which was administered concomitantly with 3-week intervals. The patient was also on Celecoxib 200 mg/day for 6 months. Patient is being followed up every 6 months and is currently disease-free.

Figure 1:

The inflammatory myofibroblastic tumor is composed of spindle cells with a background of chronic inflammatory infiltrate (H and E, ×400)

Figure 2:

Abdominal computed tomography (a) and magnetic resonance imaging (b) scan in relapse demonstrates an irregular mass in pre-sacral space

DISCUSSION

Histologically, IMT is characterized by a myofibroblastic proliferation, storiform pattern, and a varying degree of inflammatory infiltrates consisting mainly of lymphocytes, plasma cells, and histiocytes. Surgical resection is considered as the primary therapeutic approach of IMT, and most patients respond well. Kovach et al.[1] reviewed forty-four patients with IMT and treated them with surgical resections, who have either responded well or are in complete remission. Out of the follow-up of 24 months, 3 patients (8%) with IMT had a local recurrence and none of the patients had local or recurrent metastasis. Tanaka et al.[2] reported a 79-year-old male with IMT of the ascending colon who had no local or distan metastasis 16 months after hemi-colectomy. Zhou et al.[3] described a 13-month-old girl with rectal IMT, who was also free of recurrence 4½ years after complete resection. Nevertheless, the optional strategies are still unclear for impossible or incomplete resection.

The risk of IMT recurrence varies from 8% to 25%, and that of metastasis is less than 5%.[145] This suggests that some IMT have malignant trend. Therefore, radiotherapy and chemotherapy are often considered for IMT with impossible or incomplete resection. Role of chemotherapy has been proven to be controversial in the treatment of IMT. Most reported composition of chemotherapy referred to the soft tissue tumors, such as ifosfamide, etoposide, adriamycin, and cisplatin. Bertocchini et al.[6] reported pediatric cases of unresectable multifocal omental and peritoneal IMT was followed by chemotherapy and ketorolac. The chemotherapy regimen consisted of 4 cycles of ifosfamide and adriamycin followed by two courses of ifosfomide. Tumor was under control 15 months after chemotherapy. Whereas another case of a 28-year-old patient with pulmonary IMT showed metastasis to the bones and adrenal glands, and was also not responsive to 4 cycles of chemotherapy with bleomycin, etoposide, and cisplatin.[7]

Radiotherapy is rarely discussed in the treatment of IMT. Schutte et al.[8] reported a 44-year-old male patient with unresectable IMT of pancreatic head. The patient was managed with radiotherapy (20GY/10f) and corticosteroid medication, and presented with stable disease after 12 months of palliative treatment. Kovach et al.[1] also reported a 15-year-old patient with extensive lesions in the retro peritoneum and mediastinum who received radiotherapy and chemotherapy. The patient has been disease-free for 2 years. Currently, radiotherapy is advised for incompletely resected or unresectable IMT.

In addition, NSAIDs, especially Cycloxygenase (COX)-2inhibitors are recommended as adjuvant treatment options. Applebaum et al.[9] found substantial presence of COX-2 and vascular endothelial growth factor (VEGF) expression in IMT. NSAIDs were thought to inhibit angiogenesis stimulation of VEGF by COX-2/prostaglandin/VEGF pathway. Several cases have confirmed that NSAIDs could induce regression of the tumors, example: Przkora et al.[10] reported a 63-year-old female patient and a 22-year-old male patient who achieved a complete regression, and stabilization of unresectable IMTs were induced by NSAIDs.

CONCLUSION

Patient with a diagnosis of rectal IMT, with a complete resection and lack of adjuvant treatment showed recurrence after 18 months. However, radical resection could not be delivered for adjacent organs and the patient's will of anal preservation. Based on biological aggressiveness of recurrent IMT, we considered that it was necessary for intense and combined therapy following palliative resection. Some researchers also thought that adjuvant chemotherapy in conjunction with radiation therapy should be considered after incomplete resection for anatomic location or comorbidities.[1] The course of treatment for the recurrent tumor was chemotherapy with cisplatin and epirubicin, concurrent radiotherapy (66 Gy/33f), and NSAID (Celecoxib) were also administered. Two years into follow-up, there was no recurrence or metastasis. This indicates that concomitant chemoradiation combined with NSAID is reasonable for incompletely resected tumors.

In conclusion, this study is the first of its kind with rectal IMT in an adult with recurrence and was successfully managed with concurrent chemoradiation with NSAID after incomplete resection. The complete surgical resection can be considered as a preferred therapeutic option. However, for incompletely resected tumors, concurrent chemoradiation with NSAID may be a reasonable option.

REFERENCES

1. Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R, et al Inflammatory myofibroblastic tumors J Surg Oncol. 2006;94:385–91

2. Tanaka A, Hirabayashi K, Sadahiro S, Maeda Y, Suzuki T, Ogoshi K. Inflammatory myofibroblastic tumor of the ascending colon in adults manifested by positive fecal occult blood test Gastrointest Endosc. 2010;71:214–6

3. Zhou X, Luo C, Lv S, Gan M. Inflammatory myofibroblastic tumor of the rectum in a 13-month-old girl: A case report J Pediatr Surg. 2011;46:E1–4

4. Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor).A clinicopathologic and immunohistochemical study of 84 cases Am J Surg Pathol. 1995;19:859–72

5. Coffin CM, Dehner LP, Meis-Kindblom JM. Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: An historical review with differential diagnostic considerations Semin Diagn Pathol. 1998;15:102–10

6. Bertocchini A, Lo Zupone C, Callea F, Gennari F, Serra A, Monti L, et al Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: Revisiting the role of adjuvant therapy J Pediatr Surg. 2011;46:e17–21

7. Suetsugu S, Yamamoto H, Izumi M, Takayama K, Inoue H, Nakanishi Y. A case of rapidly growing inflammatory myofibroblastic tumor in the lung Nihon Kokyuki Gakkai Zasshi. 2009;47:1156–60

8. Schütte K, Kandulski A, Kuester D, Meyer F, Wieners G, Schulz HU, et al Inflammatory Myofibroblastic Tumor of the Pancreatic Head: An Unusual Cause of Recurrent Acute Pancreatitis-Case Presentation of a Palliative Approach after Failed Resection and Review of the Literature Case Rep Gastroenterol. 2010;4:443–451

9. Applebaum H, Kieran MW, Cripe TP, Coffin CM, Collins MH, Kaipainen A, et al The rationale for nonsteroidal anti-inflammatory drug therapy for inflammatory myofibroblastic tumors: A Children's Oncology Group study J Pediatr Surg. 2005;40:999–1003

10. Przkora R, Bolder U, Schwarz S, Jauch KW, Spes J, Andreesen R, et al Regression of nonresectable inflammatory myofibroblastic tumours after treatment with nonsteroidal anti-inflammatory drugs Eur J Clin Invest. 2004;34:320–1

Source of Support: Nil.

Conflict of Interest: None declared.

Keywords:

Chemoradiation; inflammatory myofibroblastic tumor; non-steroidal anti-inflammatory drugs; recurrence