CASE REPORT Open Access

Fibromatosis in vertical rectus abdominismyocutaneous flap imitating tumorrecurrence after surgery for locallyadvanced rectal cancer: case reportMariusz Adam Goscinski1, Knut Håkon Hole3, Elin Tønne4, Truls Ryder1, Krystyna Kotanska Grøholt2

and Kjersti Flatmark1,5*

Abstract

Background: Abdominoperineal excision is performed in patients with locally advanced, low rectal carcinoma.Reconstruction of the dorsal vagina and perineum using the vertical rectus abdominis myocutaneous flap followingextensive surgery results in favorable surgical outcome and quality of life. However, the rectus abdominis muscle, aspart of the anterior abdominal wall, may develop fibrous lesions also as a transplant.

Case presentation: A 39-year-old female patient with low rectal cancer and extensive colorectal polyposis was treatedwith neoadjuvant chemoradiotherapy followed by colectomy and abdominoperineal excision with resection of thedorsal vaginal wall and subsequent reconstruction of the perineum using the vertical rectus abdominis myocutaneousflap. At the 6-month follow-up, a suspected 2 × 2 cm tumor recurrence was detected in the transposed tissue and wassubsequently surgically removed. Histologic examination concluded with fibromatosis. Genetic testing revealed a knowndisease-causing mutation in the adenomatous polyposis coli gene, confirming the diagnosis of familial adenomatouspolyposis.

Conclusions: Fibromatosis may affect the anterior abdominal wall, that is the rectus abdominis muscle, at the primarysite or may develop in the muscle after its transposition into the perineum at pelvic reconstruction. Fibromatosis in themuscle flap after pelvic reconstruction may present a difficult diagnostic challenge for the multidisciplinary team.

Keywords: Locally advanced rectal cancer, Familial adenomatous polyposis, Abdominoperineal excision, Vertical rectusabdominis myocutaneous flap, Fibromatosis, APC

BackgroundFor low rectal cancer, abdominoperineal excision (APE) isusually the procedure of choice, and in locally advancedcases, the majority of patients require extralevatoric exci-sion to achieve adequate circumferential resection mar-gins [1]. Tumor involvement of the posterior vaginal wallimplicates not only the necessity for resection but also thetransposition of tissue to reconstruct the vaginal continu-ity and promote wound healing [2, 3]. Commonly, a

vertical rectus abdominis myocutaneous (VRAM) flap isused for this purpose, as it has been shown to decreasewound complications after surgery [4, 5].

Case presentationA 39-year-old, healthy, non-smoking female patient withno family history of cancer was referred on suspicion ofrectal cancer. Digital rectal examination and subsequentrigid proctoscopy revealed a low rectal tumor and mul-tiple polyps in the rectum. Subsequent colonoscopyshowed extensive polyposis of the entire colon. Thetumor biopsy confirmed the presence of a rectal adeno-carcinoma, while dysplastic changes were found in thepolyp biopsies. No distant metastases were detected on

* Correspondence: kjersti.flatmark@rr-research.no1Departments of Gastroenterological Surgery, The Norwegian RadiumHospital, Oslo University Hospital, Oslo, Norway5Faculty of Medicine, Institute for Clinical Medicine, University of Oslo, Oslo,NorwayFull list of author information is available at the end of the article

© 2016 Goscinski et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, andreproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link tothe Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Goscinski et al. World Journal of Surgical Oncology (2016) 14:63 DOI 10.1186/s12957-016-0818-4

the thoracoabdominal computed tomography (CT) scan,and baseline carcinoembryonic antigen (CEA) was 8 μg/L.Pelvic magnetic resonance imaging (MRI) revealed a lo-cally advanced rectal cancer with extra-mesorectal growthinto the rectovaginal septum and the lower posterior vagi-nal wall as well as a suspected growth into the anterioraspect of the coccygeal bone with multiple suspected ma-lignant lymph nodes within the mesorectum (T4N2).Upon evaluation by our multidisciplinary team (MDT),she was scheduled for neoadjuvant chemoradiotherapy(CRT) followed by surgery. Neoadjuvant treatment wasgiven as CT planned radiotherapy (daily 2-Gy fractions,5 days per week; the initial 23 fractions to the macro-scopic tumor and areas at risk, and the two final fractionsadapted to the macroscopic tumor) with concomitant cap-ecitabine (825 mg/m2) on days of RT. The response evalu-ation showed excellent tumor volume response, but withsimilar organ involvement as at baseline, and she pro-ceeded to surgery 8 weeks after CRT completion. Surgeryinvolved total colectomy, APE with resection of the pos-terior vaginal wall and coccygeal bone with subsequentreconstruction of the perineum with right-sided VRAMflap, and a terminal ileostomy. After an uneventful recov-ery, she was discharged 15 days postoperatively. Histologicexamination of the specimen showed a 35-mm large ade-nosquamous carcinoma removed with free resection mar-gins and metastasis in one of four local lymph nodes

(pT2N1). In addition, multiple dysplastic adenomas werepresent in the entire colon.At the routine follow-up 6 months postoperatively, clin-

ical examination revealed a 2 × 2 cm tumor located in themuscle tissue of the VRAM flap, and an early local recur-rence was suspected. Pelvic MRI showed a 10 × 16 mmrich vascularized and cell dense lesion corresponding tothe clinically detected tumor (Fig. 1). No other patho-logical findings were made on CT or MRI, and CEA was1 μg/L. The lesion was surgically excised with a wide localresection of the tumor. Interestingly, histologic examin-ation of the removed lesion described a mass composed offatty tissue, fibrocytes, and fibroblasts arranged in broad,sweeping fascicles infiltrating the adjacent striatedmuscle tissue. No dysplasia was observed, but mitoseswere present. Immunohistochemistry showed positivestaining for β-catenin (ABCAM, Cambridge, USA), andthe tumor was diagnosed as a fibromatosis (Fig. 2). Themedical geneticists suspected familial adenomatous polyp-osis (FAP), and testing of the APC gene, revealed a knowndisease-causing mutation c.3317dupG (p.Ala1107Serfs*12),confirming this diagnosis.

ConclusionsDeep fibromatosis (desmoid tumor) is a benign, myofi-broblastic proliferation that rarely occurs in the generalpopulation but frequently in one of the hereditary cancer

Fig. 1 Transversal MR images (a–c) and hematoxylin and eosin stained tumor section (d); localization outlined in purple squares. a T2-weightedimage. b Dynamic contrast enhanced parameter map (KTRANS). c Heavily diffusion weighted image (b1500). MRI-indicated and rich vascularization(b) and high cellularity (c)

Goscinski et al. World Journal of Surgical Oncology (2016) 14:63 Page 2 of 4

predisposition conditions known as FAP or a variant ofFAP called Gardner syndrome [6, 7]. Nearly all deepfibromatoses have somatic β-catenin or APC gene muta-tions leading to intranuclear accumulation of β-catenin[8]. Nuclear detection of β-catenin by immunohisto-chemistry is reported in 80–98 % of cases and is used indiagnostics [9, 10]. Desmoid tumors in FAP are typicallyassociated with mutations in the APC gene locateddownstream of codon 1400 [11]. However, the genotype-phenotype association is not absolute, as in this case,where the mutation is approximately 300 codons up-stream of codon 1400. The clinical presentation of fibro-matosis is variable, depending on the location and theextent of the lesion. Desmoids may occur in any musculo-aponeurotic tissue structures of the body as well as withinthe abdominal cavity. Extra-abdominal fibromatoses arisetypically from the rectus or internal oblique muscles andfascia predominantly in young women. Previous surgeryand trauma sites and irradiated tissues may also be a char-acteristic place of desmoid expansion [12, 13].Taking into consideration all of these factors, our pa-

tient was at high risk of developing fibromatosis. How-ever, as the standard follow-up procedure for patientsundergoing rectal cancer treatment is to focus on earlydetection of recurrence and metastasis, and furthermoreconsidering the patient’s primary diagnosis, the MRIfindings, the lesion site, and its rapid development, theinitial conclusion was local recurrence. Fibromatosis as adifferential diagnosis was thus not considered, and theMDT opted for direct surgical removal, as the lesion’slocation and size were found to be appropriate for primarysurgical removal. The use of PET-CT was discussed, buthigh-quality CT and MRI analysis did not reveal additionalsuspected malignant lesions and were considered suffi-cient for making treatment decisions. The histologicalexamination, however, revealed fibromatosis in the VRAMflap in the perineum instead of the expected recurrence. Itis noteworthy that no sign of fibromatosis in the abdom-inal rectus muscle was found on the CT prior to the pri-mary surgery.

Currently, the universal approach to management ofdesmoid tumors (both abdominal and extra-abdominal)favors the “watch and wait” approach rather than surgi-cal removal. This is based on the observation that sizestabilization or spontaneous regression may occurwhile surgery in itself may trigger the growth of newlesions [14, 15].In this case, no histological evidence (biopsy) was avail-

able to support such a decision, nor was the genetic evalu-ation implemented. The lesion was considered to be asuspected local relapse without distant metastases at thetime of diagnosis and was dealt with according to nationalguidelines for treatment of recurrent rectal cancer.In this case, fibromatosis in transposed rectus abdom-

inis muscle tissue imitated a rectal cancer recurrence.Although rare, but with pelvic reconstructive surgerybecoming more frequent, this may be a relevant differ-ential diagnosis for the MDT to consider, particularly inpatients with FAP.

ConsentWritten informed consent was obtained from the patientfor publication of the case report and any accompanyingimages. A copy of the written consent is available for re-view at the editor of this journal.

AbbreviationsAPC: adenomatous polyposis coli; APE: abdominoperineal excision;CEA: carcinoembryonic antigen; CRT: neoadjuvant chemoradiotherapy;CT: computed tomography; FAP: familial adenomatous polyposis; Gy: gray;MDT: multidisciplinary team; MRI: magnetic resonance imaging; N: lymphnodes; T: tumor; VRAM: vertical rectus abdominis myocutaneous.

Competing interestsThe authors declare that they have no competing interests.

Authors’ contributionsMAG fully participated in the clinical treatment and the follow-up of thepatient, initiated the study, participated in its design, and helped to draft themanuscript. KHH participated in the diagnostics of the patient, initiatedthe study, participated in its design, and helped to draft the manuscript.ET participated in the diagnostics of the patient. KKG participated in thefollow-up of the patient. KF participated in the follow-up of the patient,initiated the study, participated in its design, and helped to draft themanuscript. All authors read and approved the final manuscript.

Fig. 2 Histologic high power views of the resected tumor. a Hematoxylin and eosin staining. b ß-catenin immunohistochemical staining

Goscinski et al. World Journal of Surgical Oncology (2016) 14:63 Page 3 of 4

Author details1Departments of Gastroenterological Surgery, The Norwegian RadiumHospital, Oslo University Hospital, Oslo, Norway. 2Departments of Pathology,The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway.3Departments of Radiology and Nuclear Medicine, The Norwegian RadiumHospital, Oslo University Hospital, Oslo, Norway. 4Department of MedicalGenetics, The Norwegian Radium Hospital, Oslo University Hospital, Oslo,Norway. 5Faculty of Medicine, Institute for Clinical Medicine, University ofOslo, Oslo, Norway.

Received: 16 December 2015 Accepted: 1 March 2016

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