Section: Approach and Reconstruction Strategy

189

Sporadic Abdominal Wall Desmoid Tumor after Caesarean Section: Approach and Reconstruction Strategy Nai-Jen Chang,1,4 Shao-Chih Hsu,1,4 Chih-Wei Wang,2,4 Wei-Fan Chen,1,4 Ming-Mo Hou,3,4 Chih-Hung Lin1,4 1

Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital

2

Department of Pathology, Chang Gung Memorial Hospital

3

Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital

4

Chang Gung Medical College and Chang Gung University, Taoyuan, Taiwan

Background: Desmoid tumor is the most common abdominal wall tumor. It is frequently associated with either surgical trauma, such as abdominal or pelvic surgery, or physiologic trauma, such as pregnancy. So, Caesarean sections (C/S) is thought to be an important risk factor because it contains both factors. Aim and Objectives: Review our experience of sporadic abdominal desmoid tumors relate to C/S and analyze it. Materials and Methods: Between 1983 and 2010, 9 cases of abdominal wall desmoid tumor with prior history of C/S were included in the study. All of the 9 patients were female with the mean age of 30.2 (ranging from 27 to 35-year-old). The demographic data including sex, age, history of previous C/S, and outcomes were analyzed. Results: Between all the patients, one patient had three prior C/S; three patients had two and five patients had one. All of the patients received surgical excision with primary closure with clear margin. The postoperative courses were all smooth except one subsequently developed recurrence and underwent re-excision. Conclusion: Pre-operative biopsy to confirm the pathology and rule out the possibility of systemic disease is mandatory when making the diagnosis of desmoid tumor. Intervene the tumor concurrent with C/S or at postpartum period are both acceptable choices. One stage excision with wider margin and optional reconstructive modalities including mesh closure and autologous tissue reconstruction is the goal to achieve abdominal wall closure. (J Taiwan Soc of Plast Surg 2011;20:189∼195) Key words: desmoid tumor, caesarean sections, reconstruction

190

Sporadic Abdominal Wall Desmoid Tumor after Caesarean Section

Background Desmoid tumor is the most common abdominal wall tumor.1,2 At this area, it commonly develops from the rectus abdominis muscle or connective tissue. It infiltrates adjacent muscles and fascial planes, frequently entrapping them, and results in their degeneration. Although desmoid tumor has a high rate of local recurrence, it rarely metastasize.3 Desmoid tumor can occur sporadically or as part of familial adenomatous polyposis (FAP) caused by adenomatous polyposis coli (APC) gene mutation. The incidence of sporadic desmoid tumor is estimated to be 2 to 4 per million per year.4 It is associated with either surgical trauma, such as abdominal or pelvic surgery, or physiologic trauma, such as pregnancy.5 Given these associations, it unveils that desmoid tumors peak at reproductive age, and prone to occurred after Caesarean sections (C/S). In this paper, we review our experience of sporadic abdominal desmoid tumors after C/S.

Material and Method Between 1983 and 2010, 11 cases of abdominal wall desmoid tumor with prior history of C/S were identified but two patients were excluded from the study – one had incomplete data and the other was found to have underlying familial adenomatous polyposis (FAP). The remaining 9 cases were all female with the mean age of 30.2 (ranging from 27 to 35-year-old), all of the diagnoses were confirmed with both conventional hematoxylin and eosin stain and immunohistochemical (IHC) stain. The demographic data including sex, age, history of previous C/S, and outcomes were analyzed in the study.

Result Amongst all the patients, one patient had three prior C/S; three patients had two; and five patients had one. In three patients the diagnosis of desmoid tumor was made during the pregnancy, and the remaining patients had their diagnoses established after the C/S. As for the location, the tumors were located at precious 臺灣整形外科醫誌：民國 100 年／20 卷／3 期

C/S scar in four patients and the other five were not, including one at para-umbilical area, two at left lower quadrant, and two at right lower quadrant of abdomen. The diameters of tumor were between 3 and 14 cm. One patient subsequently developed recurrence and underwent re-excision. Regarding the three cases diagnosed during pregnancy, two patients were intervened during C/S and another one intervened after C/S. All of the patients received surgical excision with primarily closed clear margin, and all had uncomplicated postoperative course.

Case Presentation 31-year-old female (case 9) has a history of C/S 13 months ago with prominent transverse scar at low abdomen. She palpated a left flank smooth and movable mass 2 months ago and the mass grew gradually (figure 1A). The computed tomography (CT) revealed an enhanced mass (*) over the left lateral abdominal wall beneath the left external oblique muscle measuring 7 X 4 cm. The CT diagnosis was leiomyoma (figure 2). The operation was performed through the previous C/S incision and the tumor was located at external oblique muscle layer. Tumor was well encapsulated, not related to the previous incision (figure 1B). The tumor did not involve the peritoneum and removed entirely with the wound closed primarily. The size of the tumor was 7.5 x 5.5 x 4.5 cm (figure 1C). Histopathologically, the tumor was composed of slender spindle cells with bland nuclei in a collagenous stroma under hematoxylin and eosin (H&E) stain. It was poorly circumscribed and showed infiltrative borders with adjacent soft tissue. Immunohistochemical (IHC) stain showed negative for smooth muscle actin, thus excluding the possibility of leiomyoma. (figure 3A and 3B). The outcome of the patient was smooth without tumor recurrence or abdominal hernia after 4 months follow up.

Nai-Jen Chang, Shao-Chih Hsu, Chih-Wei Wang, Wei-Fan Chen, Ming-Mo Hou, Chih-Hung Lin

191

Fig. 1. (A) 31-year-old female has a history of C/S 13 months ago with prominent transverse scar at low abdomen. She palpated a left flank smooth and movable mass 2 months ago and the mass grew gradually. (B) The operation was performed through the previous C/S incision and the tumor was located at external transverse muscle layer with well encapsulation. The tumor was removed entirely without involvement of the peritoneum. The wound was closed primarily. (C) The size of the tumor was 7.5 x 5.5 x 4.5 cm. The outcome of the patient was smooth without tumor recurrence or abdominal hernia after 4 months follow up.

*

Fig. 2. The computed tomography showed an enhanced mass ( ) over left lateral abdominal wall beneath the left external oblique muscle measured 7 X 4 cm. The histological diagnosis revealed a desmoid tumor despite CT diagnosis of a leiomyoma.

JTSPS 2011. Vol 20‧No.3

192


Fig. 3. The histological examination of the above case. (A) The tumor is composed of slender spindle cells. It shows an infiltrative border with the adjacent skeletal muscles (hematoxylin and eosin stain, 200X). (B) The spindle cells only very focally positive for smooth muscle actin, the diagnosis was desmoid tumor (immunohistochemical stain, 200X).

Table 1.

The report case of desmoids tumor at abdominal wall after Caesarean section.

Case

Sex

Age

Previous C/S times

Time interval of previous C/S

Occurred during pregnancy

Tumor location at C/S scar

Tumor size (cm)

recurrence

1

F

27

2

1 month

Yes

No

7

No

2

F

29

2

Unknown

No

No

3

No

3

F

35

2

Unknown

No

No

6

No

4

F

26

1

Unknown

No

Yes

5

No

5

F

31

3

84, 60, and 6 months

No

No

14

No

6

F

33

1

24 months

Yes

Yes

11

Once

7

F

31

1

3 months

Yes

Yes

5

No

8

F

29

1

18 months

No

No

5

No

9

F

31

1

13 months

No

Yes

8

No

Gardner's syndrome

臺灣整形外科醫誌：民國 100 年／20 卷／3 期


Discussion Desmoid tumor is the most common abdominal wall tumors but we should be careful during the treatment because of certain pitfalls. First, desmoid tumor is easily confused with other diseases, so it is important to confirm a diagnosis histopathologically pre-operatively. Sometimes immunohistochemical (IHC) staining is necessary to differentiate from other diseases. For instance, abdominal wall leiomyoma is easily confused with desmoid tumor clinically, radiologically and histopathologically.6,7 Only IHC stain can differentiate these two diseases clearly because desmoid tumor would demonstrate absence of smooth muscle actin, unequivocally rule out leiomyoma. Second, systemic evaluation is required because sometimes desmoid tumor is associate with FAP, which may lead to colorectal cancer in the young adult.8 Third, compared with other benign tumors, the excision of desmoid tumors need to include adjacent tissue, muscle and fascia because it usually infiltrates into them, otherwise the recurrent rate is high.8 Interestingly, most of the previously published series did not pursue a definitive histopathological confirmation with IHC staining, and the systemic review of FAP is usually absent, which may overlook the malignant changes of the gastrointestinal tract. Function-sparing resection is appropriate treatment for desmoids tumor because adjuvant radiation therapy can offset the adverse impact of positive margins, and unresectable disease should be treated with definitive radiation therapy.4,9 Compared with other sites, abdominal wall desmoid tumor has a significantly lower recurrence rate because it can be removed with a generous negative margin without affecting vital organs, especially in patients after C/S, in whom the abdominal wall is usually lax. The series presented by Sutton and Thomas10 (n=7) and Lahat8 et al (n=16) found no recurrence.5 Shao et al.11 reported a recurrence rate of 5.5% in a large series in China of 42 abdominal wall desmoids. In our series, the recurrent rate was 11.1 % (1/9 cases). Although the series is small, we still have to pay attention to it because the approach to desmoid tumor is actually different from

193

other tumors. The patients with young age, large tumor size, recurrent disease have higher rate of failed local control because of relative difficulty to achieve generous negative margins. The patients without biopsy pre-operatively may also lead to the inadequate resection of the tumor. In the majority of cases of demoid tumors, the resulting defect can be closed primarily. Especially for patients after C/S, the abdominal wall is usually lax.12 If primary closure is not feasible, a variety of reconstructive modalities can be used to achieve abdominal wall closure. Alloplastic meshes have been used successfully for this purpose with a low risk of hernia.11 Acellular dermal allograft (AlloDerm) is an excellent choice due to its fascia-like characteristics to keep the strength and also it can resistant infection.14 Autologous modalities include component separation,14 pedicle or free anterolateral thigh (ALT) flap,15 vascularized fascia lata,15 latismus dorsi flap,16 rectus abdominis myocutaneous flap,17 or propeller deep inferior epigastric perforator flap.18 Pedicled flaps from lateral circumflex femoral artery territory are versatile modalities for reconstruction. It can be performed quickly without the need of microscopic anastomosis as in free tissue transfer, and it provides an abundance of skin, soft tissue, and fascia for abdominal wall reconstruction.15 Regardless of the kind of reconstruction, single-stage operation combining wide excision to achieve negative margin and restoration of the abdominal wall is recommended.12 In conclusion, when the diagnosis of abdominal wall desmoid tumor is suspected, it is crucial to perform fine needle biopsy preoperatively to achieve unequivocal diagnosis histopathologically. If there are any doubts, IHC staining can help to differentiate with other diseases. Wide excision of the tumor with adjacent muscle, fascia and tissue can help to achieve negative margins. If the resulting defect is too large for primary closure, artificial or autologous tissue reconstruction can be considered. As for the patients with previous C/S, the tumor is not necessarily related to the incision. The intervention can be during C/S or after delivery, and the laxity of the abdominal wall usually allows the wound to be closed primarily. JTSPS 2011. Vol 20‧No.3

194


Reference

abdominal wall. Eur J Surg Oncol 1999; 25: 398-400. 11. Shao YF, Yu HT, Hu JQ et al. Abdominal wall desmoid

1. Stewart EA: Uterine fibroids. Review Lancet 2001, 357 (9252):293-8. 2. Stojadinovic A, Hoos A, Karpoff HM et al. Soft tissue tumors of the abdominal wall: analysis of disease patterns and treatment. Arch Surg. 2001 Jan;136(1):70-9. 3. Enzinger FM, Weiss SW. Fibromatosis. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. St Lewis, MO: Mosby, 1995: 201-29.

tumor – analysis of 42 patients. Zhonghua Zhong Liu Za Zhi 1988; 10: 63-4. 12. Khorsand J, Karakousis CP. Desmoid tumors and their management. Am J Surg 1985; 145: 215-18. 13. Goyal N, Khurana N. Leiomyoma of rectus sheath: an uncommon entity: report of two cases. Indian J Pathol Microbiol 2010;53:597-8. 14. Kolker AR, Brown DJ, Redstone JS et al. Multilayer

4. de Bree E, Keus R, Melissas J et al. Desmoid tumors: need

reconstruction of abdominal wall defects with acellular

for an individualized approach. Expert Rev Anticancer

dermal allograft (AlloDerm) and component separation.

Ther. 2009 Apr;9(4):525-35.

Ann Plast Surg. 2005 Jul;55(1):36-41.

5. De Cian F, Delay E, Rudigoz RC et al. Desmoid tumor

15. Kuo YR, Yeh MC, Shih HS et al. Versatility of the

arising in a cesarean section scar during pregnancy:

anterolateral thigh flap with vascularized fascia lata for

monitoring and management. Gynecol Oncol. 1999 Oct;75

reconstruction of complex soft-tissue defects: clinical

(1):145-8.

experience and functional assessment of the donor site.

6. Teo HE, Peh WC, Shek TW. Case 84: desmoid tumor of the abdominal wall. Radiology. 2005 Jul;236(1):81-4.

Plast Reconstr Surg. 2009 Jul;124(1):171-80. 16. Kadoch V, Bodin F, Himy S et al. Latissimus dorsi free flap

7. Ozkavukcu E, Aygün S, Erden A, Savaş B. Pelvic retroperitoneal

for reconstruction of extensive full-thickness abdominal

angioleiomyoma mimicking a uterine mass. Diagn Interv

wall defect. A case of desmoid tumor. J Visc Surg. 2010

Radiol. 2009 Dec;15(4):262-5. Epub 2009 Oct 5.

Apr;147(2):e45-8. Epub 2010 Jun 26.

8. Lahat G, Nachmany I, Itzkowitz E et al. Surgery for

17. Kaemmer DA, Conze J, Otto J et al. New technical

sporadic abdominal desmoid tumor: is low/no recurrence

approach for the repair of an abdominal wall defect after

an achievable goal? Isr Med Assoc J. 2009 Jul;11(7):

a transverse rectus abdominis myocutaneous flap: a case

398-402.

report. J Med Case Reports. 2008 Apr 16;2:108.

9. Ballo MT, Zagars GK, Pollack A et al. Desmoid tumor:

18. Ang GG, Rozen WM, Chauhan A et al. The pedicled

prognostic factors and outcome after surgery, radiation

'propeller' deep inferior epigastric perforator (DIEP) flap

therapy, or combined surgery and radiation therapy. J Clin

for a large abdominal wall defect. J Plast Reconstr Aesthet

Oncol. 1999 Jan;17;1:158-67.

Surg. 2011 Jan 64;1:133-5.

10. Sutton RJ, Thomas JM. Desmoid tumours of the anterior

Reprints request from: Chih-Hung Lin, M.D. Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital Address: 5, Fu-Hsing St. Kuei-Shan, Taoyuan, Taiwan Tel: 886-3-3281200 Ext.2946 Fax: 886-3-3289582 E-mail: [email protected]

臺灣整形外科醫誌：民國 100 年／20 卷／3 期


195

剖腹生產後之偶發性結締織維瘤：治療及重建策略張乃仁許韶芝王志偉陳威帆侯明模林志鴻

背

景：結締織維瘤是最常見的腹壁腫瘤。手術創傷如腹部及骨盆腔手術，或生理創傷如懷孕都是容易產生結締纖維瘤的危險因子。因此年輕女性在經歷剖腹產後得到此疾病是合理的推測。目的及目標：報導並分析本院結締纖維瘤與剖腹產相關之臨床病例之治療經驗。材料及方法： 1983 年至 2010 年，九例腹壁結締纖維瘤病曾經接受剖腹產的病患被列入研究。所有的病例為女性，平均年齡為 30.2 歲。統計及分析研究的資料包含性別，年齡，剖腹產的次數和治療成果。結果：在所有九位病人當中，一位病人經歷了三次剖腹產，三位經歷了兩次，五位經歷了一次。所有的患者接受手術切除後邊緣均無腫瘤細胞且沒有術後併發症，只有一個病人後來復發並接受了再切除。結論：診斷結締纖維瘤需要術前病理的確認且排除全身性相關疾病。連帶周圍組織的切除是確保邊緣乾淨及復發的方法。如果傷口不能直接關閉，各種方式包括人工網的重建或自體組織重建都可以達到腹壁關閉的功能。

JTSPS 2011. Vol 20‧No.3