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Table of Contents
Year : 2018  |  Volume : 1  |  Issue : 2  |  Page : 48-51

A large ischiorectal fossa soft tissue sarcoma: Surgical technique (combined minimally invasive with open approach) and reconstruction with V-RAM flap

1 Departments of Surgical Oncology, Jupiter Hospital, Pune, Maharashtra, India
2 Departments of Plastic Surgery, Jupiter Hospital, Pune, Maharashtra, India
3 Department of Colorectal Surgery, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication26-Nov-2019

Correspondence Address:
Ashish Pokharkar
Department of Oncology, Jupiter Hospital, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJCS.IJCS_11_18

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Here, we report a rare case of large ischiorectal fossa (IRF) soft-tissue sarcoma with involvement of the rectum. The patient was operated with laparoscopic abdominoperineal resection and wide local excision of a tumor through the posterior approach. Postoperatively, the patient received adjuvant radiotherapy and chemotherapy. Surgical resection is the mainstay of treatment and includes resection of surrounding organs to get adequate surgical margins for locally advanced IRF tumors. Laparoscopic approach causes less morbidity and early recovery.

Keywords: Ischiorectal fossa, laparoscopic rectal surgery, sarcoma, V-RAM flap

How to cite this article:
Pokharkar A, Dhamangaokar M, Athawle S, Patil A, Saklani A. A large ischiorectal fossa soft tissue sarcoma: Surgical technique (combined minimally invasive with open approach) and reconstruction with V-RAM flap. Indian J Colo-Rectal Surg 2018;1:48-51

How to cite this URL:
Pokharkar A, Dhamangaokar M, Athawle S, Patil A, Saklani A. A large ischiorectal fossa soft tissue sarcoma: Surgical technique (combined minimally invasive with open approach) and reconstruction with V-RAM flap. Indian J Colo-Rectal Surg [serial online] 2018 [cited 2023 Jan 28];1:48-51. Available from: https://www.ijcrsonweb.org/text.asp?2018/1/2/48/271746

  Introduction Top

Ischiorectal fossa (IRF) is space bounded anteriorly by anterior perineum, posteriorly by sacrotuberous ligament with gluteus maximus muscle, and laterally by ischial tuberosity with obturator internus muscle. Tumors involving this region are very rare and include congenital lesions and soft-tissue sarcomas (STSs). Surgical excision of these tumors remains a challenge due to complex anatomy and need of different approaches. Here, we describe the technique for large pleomorphic sarcoma of IRF involving rectum. We have adopted a minimally invasive laparoscopic approach for rectal mobilization along with posterior approach. Reconstruction was done with pedicled vertical rectus abdominis (VRAM) flap and unilateral V-Y plasty.

  Case Report Top

A 45-year-old female presented with a history of perineal swelling and pain for 1 year [Figure 1]. She had a history of undergoing surgery previously with inadequate excision considering it as infection. Histopathology showed high-grade malignant tumor. The patient did not take any adjuvant treatment and presented after 3 months with a large perineal mass. Local examination revealed a 14 cm × 14 cm mass involving the right IRF crossing the midline toward the left. On per rectal examination, there was external compression with no mucosal involvement. Pathology review showed high-grade pleomorphic sarcoma. Computed tomography (CT) scan of the thorax and abdomen showed no systemic metastases. Magnetic resonance imaging (MRI) of the pelvis showed a heterogeneous mass in the right IRF (14 cm × 11 cm × 9 cm) with extension to the left IRF [Figure 2]. The tumor was seen displacing rectum and vagina with involvement of the external sphincter muscles. There was no significant pelvic lymphadenopathy. After discussion in the multidisciplinary tumor board meeting, treatment was planned for surgical resection of a tumor with abdominoperineal resection (APR) and permanent end stoma.
Figure 1: Right ischiorectal fossa tumor with the skin involvement

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Figure 2: T2 magnetic resonance imaging images show a tumor with its extent

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The patient was placed in modified Lloyd–Davies position under general anesthesia. Laparoscopic ports were placed as shown in [Figure 3]. The right side ports were placed a little bit more laterally to avoid future VRAM donor area. Abdominal and pelvic dissection was done with complete laparoscopic approach till the levator ani was reached bilaterally [Figure 4]. The sigmoid colon was cut with Echelon 60 Green Stapler (Ethicon Inc., Ethicon, Johnson and Johnson Inc.,) [Figure 5]. The sigmoid colon was taken out as end stoma. The patient was then placed in a prone position. The tumor was resected with 2 cm margin [Figure 6]. Posteriorly, the resection involved S4–5 vertebrae along with coccyx. Laterally, on the right sides, some part of gluteus maximus was resected. The tissue between both ischial tuberosities was completely excised. The patient was again placed in a supine position to harvest VRAM flap. A 25 cm × 15 cm flap was harvested from the anterior abdominal wall based on distal inferior epigastric vessels [Figure 7]. The flap was delivered to the pelvis through mini laparotomy in suprapubic region and abdominal wound sutured primarily along with suction drain [Figure 8]. Then, the patient was placed in prone position and the defect was closed with VRAM flap and left V-Y plasty along with suction drain [Figure 9]. The total blood loss was 300 ml.
Figure 3: laparoscopic ports' placement

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Figure 4: Laparoscopic part of dissection

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Figure 5: The sigmoid colon cut with Echelon Stapler

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Figure 6: The tumor was resected with adequate margins

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Figure 7: V-RAM flap was marked and harvested

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Figure 8: The flap was delivered through wound defect

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Figure 9: The defect was closed with V-RAM flap and V-Y local flap

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The levator ani was cut on both sides at origin on the lateral pelvic wall. Anteriorly, the vagina and uterus were preserved. The specimen was retrieved along with the rectum and sent for histopathological examination.

Postsurgery, histopathology showed high-grade pleomorphic sarcoma with clear surgical margins. The patient was discharged on postoperative day 8. Stitches were removed on postoperative day 12. The patient developed urinary retention after the removal of Foley's catheter which was managed with self-intermittent catheterization. Pudendal nerve injury could be the cause of urinary retention, which was sacrificed during surgery. Postoperatively, the patient received adjuvant chemotherapy and radiotherapy.

  Discussion Top

STSs can involve any body site, and IRF is not the exception to this. Very few reports of IRF sarcoma have been reported in the literature.[1],[2],[3],[4],[5] IRF is a subsite of perineum which holds many important functions of the body, e.g., reproductive and excretory. Being complex anatomical site, tumors involving perineum pose a great challenge for the surgeons. Here, we report a case of large locally advanced pleomorphic sarcoma of the right IRF in a 45-year-old female.

Many benign and malignant pathological masses involve perineum. Congenital lesions include tailgut cyst, rectal duplication, giant epidermal cyst, chordomas, and meningomyelocele. Neoplastic lesion includes STS, lipoma, and anorectal sarcomas.[6],[7] There are many types of sarcoma, the most common being liposarcoma, malignant fibrocystic histiocytoma, and leiomyosarcoma. These STSs are very aggressive tumors with poor prognosis. These STSs are either superficial or deep tumors. Often, these tumors are diagnosed late and treated as more common pathologies like ischiorectal abscess. Deep lesions have abundant space to expand and involve deeper structures such as the rectum and vagina. Like in our case, these tumors spread in both superficial and deep directions and compress rectum, vagina, and urinary bladder. MRI pelvis is an investigation of choice because of better soft-tissue differentiation as compared to CT. Furthermore, MRI can better delineate relationship with surrounding structures.[8] Tissue diagnosis is generally obtained with percutaneous image-guided biopsy or incisional biopsy. Metastatic workup includes CT thorax and abdomen or positron emission tomography-CT scan.

Treatment of IRF sarcoma remains the same as other STSs. Surgical wide local excision is mainstay of treatment. Resection margin of 1–2 cm is adequate and further wider margin does not give added benefit.[9] Often, the margins are limited by surrounding important structures. In locally advanced sarcomas, wide local excision often entails APR, sacrum excision, and pelvic exenteration. In our case, we have excised sacrum below S4 to get adequate margin. We planned APR due to the sphincter and rectal involvement. We did laparoscopic APR and believe that laparoscopic approach in these complex surgeries definitely benefits the patient with less blood loss and early postoperative recovery. Reconstruction of postoperative defect can be done with primary closure or local flaps like V-Y plasty if the tumor is small. However, large defect like in our case requires more tissue, and this can be done with V-RAM, Gracilis flap, etc.

Due to the rarity of these tumors, all patients should be discussed in a multidisciplinary meeting.[10] Some patients might need neoadjuvant chemoradiotherapy, but there is no sufficient evidence for that. Criteria for adjuvant treatment are same as other sarcomas. Adjuvant radiation is recommended (category 1) if margins are positive or <1 cm, large tumors >8 cm, high-grade tumor.[11] Although there is underpowered evidence for adjuvant chemotherapy, anthracycline-based chemotherapy improves disease-free survival in selected patients who are at high risk of recurrence (category 2b).[12],[13],[14]

  Conclusion Top

IRF sarcomas are rare and management should be discussed in a multidisciplinary meeting. Surgical resection is the mainstay of treatment and includes resection of surrounding organs to get adequate surgical margins. IRF sarcomas where rectal resections are required, laparoscopic approach is preferable as it cause less morbidity and early recovery. Often, adjuvant radiation and chemotherapy are required for tumors having high risk of recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Satheshkumar T, Saklani AP, Banerjee D, Jones DR. Angiomyofibrosarcoma: A rare ischiorectal fossa swelling. Hosp Med 2003;64:244-5.  Back to cited text no. 1
Kumar H, Heroor A, Vanan A, Yadukrishna. A rare primary tumor of ischiorectal fossa: Surgical technique & review of literature. IOSR J Dent Med Sci 2018:17:26-9.  Back to cited text no. 2
Seishima R, Ishii Y, Hasegawa H, Endo T, Ochiai H, Okabayashi K, et al. Large liposarcoma developing in the ischiorectal fossa: Report of a rare case. Int J Surg Case Rep 2013;4:51-3.  Back to cited text no. 3
Mehta N, Konarski A, Rooney P, Chandrasekar C. Leiomyosarcoma of the ischiorectal fossa: Report of a novel sphincter and sciatic nerve sparing simultaneous trans-abdominal and trans-gluteal resection and review of the literature. J Surg Case Rep 2015;2015. pii: rjv016.  Back to cited text no. 4
Kim KH, Cha SH, Yeom SK, Lee SH, Chung HH. Solitary fibrous tumor of the ischiorectal fossa: CT and MRI findings. J Korean Soc Magn Reson Med 2011;15:72.  Back to cited text no. 5
Grobmyer SR, Clary B, Lewis JJ, Delgado R, Woodruff JM, Brennan MF. Adult perineal sarcomas. J Surg Oncol 2001;77:101-4.  Back to cited text no. 6
Behranwala KA, Clark MA, Thomas JM. Soft-tissue tumours of the perineum. Eur J Surg Oncol 2002;28:437-42.  Back to cited text no. 7
Aga P, Singh R, Parihar A, Parashari U. Imaging spectrum in soft tissue sarcomas. Indian J Surg Oncol 2011;2:271-9.  Back to cited text no. 8
McKee MD, Liu DF, Brooks JJ, Gibbs JF, Driscoll DL, Kraybill WG. The prognostic significance of margin width for extremity and trunk sarcoma. J Surg Oncol 2004;85:68-76.  Back to cited text no. 9
Rydholm A. Improving the management of soft tissue sarcoma. Diagnosis and treatment should be given in specialist centres. BMJ 1998;317:93-4.  Back to cited text no. 10
Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16:197-203.  Back to cited text no. 11
Woll PJ, van Glabbeke M, Hohenberger P, Le Cesne A, Gronchi A, Hoekstra HJ, et al. EORTC Soft Tissue and Bone Sarcoma Group. Adjuvant chemotherapy (CT) with doxorubicin and ifosfamide in resected soft tissue sarcoma (STS): Interim analysis of a randomised phase III trial. J Clin Oncol 2007;25:10008.  Back to cited text no. 12
Frustaci S, Gherlinzoni F, De Paoli A, Bonetti M, Azzarelli A, Comandone A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: Results of the Italian randomized cooperative trial. J Clin Oncol 2001;19:1238-47.  Back to cited text no. 13
Bramwell V, Rouesse J, Steward W, Santoro A, Schraffordt-Koops H, Buesa J, et al. Adjuvant CYVADIC chemotherapy for adult soft tissue sarcoma – Reduced local recurrence but no improvement in survival: A study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 1994;12:1137-49.  Back to cited text no. 14


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]


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