Abdominal Wall Tumor Research Articles

EL13 - Current status of stereotactic radiation therapy (SRT)

Stereotactic radiotherapy (SRT) is a technique developed for intracranial tumors. On the other hand, stereotactic body radiotherapy (SBRT) is a technique introduced in the late 1990s for extracranial tumors. SBRT is a method of using single 10-20Gy of high dose and hypofractionated radiotherapy. Recently, many papers have been published on its clinical results, especially in early stage lung cancer. Therefore, to recognize the current status of SBRT in Japan, a nation-wide survey was conducted by the Japan Conformal External Beam Radiotherapy Group (J-CERG). The questionaire was sent to 227 institutions. One-hundred and forty-nine institutions responded by the end of May 2015.The purpose of this presentation is to evaluate the current status of SBRT. The key issues for SBRT are fixing apparatus, respiratory regulation, treatment planning and verification. For regulation of respiratory movement, abdominal wall compression, breath-holding, respiratory gating and tumor chasing methods were used. For irradiation technique, 6 to 10 non-coplanar beams or multiple arc beams were adopted. Daily verification before radiotherapy is mandatory for SBRT. Frequently used radiation regimens were 48 to 60 Gy in 3 to 5 fractions. The local relapse-free rates were almost always between 85 to 95% for lung cancer. Long term follow-up results have also been reported. The number of serious complications was very low. Several unanswered questions are the indication for the patients with damaged lung and the patients with central tumor. The past results of clinical trial including JCOG0403 and the currently ongoing protocols for lung cancer including JCOG1408 will be reviewed. Most updated status of SBRT will also be reviewed.

Chest wall reconstruction using Latismus Dorsi Flap: our experience

Background: Covering a big chest wall defect is the main clinical issue in cancer resection whether it is due to locally advanced breast cancer or any other carcinomas extending to chest wall or injuries leading to chest wall defect. The main aim of the study was to discuss the role of latissmus dorsi flap in chest wall reconstruction.Methods: Study reviewed 15 patients with chest wall defect within a period of two years January 2015 - December 2016, who underwent chest wall reconstruction after tumor resection or burns.Results: Among 15 patients, 12 patients were having locally advanced breast tumors, only one patient with recurrent desmoids tumor of abdominal tumor of abdominal wall extending to chest wall and 2 cases were of electrical burn injuries. The age range varied from 40-60 years. Among these patients 13 were females and 2 were males. The defect size varied from 12x15 cm to 26 x 20 cm. There were no major complications except for partial graft loss in two patients.Conclusions: Lattissmus Dorsi flap is safe and very good flap for chest wall reconstruction.

Case report: Rapidly growing abdominal wall giant desmoid tumour during pregnancy

Abstract Background Desmoid tumours are one of the rarest tumours worldwide, with an estimated yearly incidence of 2–4 new cases per million people. They are soft tissue monoclonal neoplasms that originate from mesenchymal stem cells. It seems that the hormonal and immunological changes occurring during pregnancy may play a role in the severity and course of the disease. Clinical case The case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound. The patient was followed up under clinical and ultrasonographic surveillance. When she presented with abnormal uterine activity at 38.2 weeks of gestation, she was admitted and obstetrics decided to perform a caesarean section. Tumour biopsy was taken during the procedure. Histopathology reported a desmoid fibromatosis. A contrast enhanced abdominal computed tomography scan was performed, showing a tumour of 26 cm × 20.5 cm × 18 cm, with well-defined borders in contact with the uterus, left adnexa, bladder and abdominal wall, with no evidence of infiltration to adjacent structures. A laparotomy, with tumour resection, hysterectomy and left salpingo-oophorectomy, components separation techniques, polypropylene mesh insertion, and drainage was performed. The final histopathology report was desmoid fibromatosis. There is no evidence of recurrence after 6 months follow-up. Conclusions Desmoid tumours are locally aggressive and surgical resection with clear margins is the basis for the treatment of this disease, using radiotherapy, chemotherapy and hormone therapy as an adjunct in the treatment.

Sporadic giant intra-abdominal desmoid tumor: A radiological case report.

Desmoid tumor (DT) is a locally invasive form of fibromatosis, comprising only 0.03% of all tumors. DTs occur more frequently in patients with familial adenomatous polyposis and Gardner's syndrome, as intra-abdominal or anterior abdominal wall tumors, whereas sporadic DTs are more likely to be extra-abdominal (only 5% of sporadic DTs are intra-abdominal). There is also an association of DTs with prior trauma, surgery, estrogen exposure and childbirth. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) are used for preoperative diagnosis and for the planning of the surgery. Following surgery, CT and MRI are used to detect recurrence and to monitor the tumor's response to radiotherapy or medical therapy for unresectable or recurrent tumors. We herein report a rare case of a sporadic giant intra-abdominal DT in a 28-year-old female patient without any predisposing factors, and highlight the importance of including DT in the differential diagnosis of huge intra-abdominal masses.

Multi-parametric (mp) MRI for the diagnosis of abdominal wall desmoid tumors

IntroductionDesmoid tumors are benign myofibroblastic neoplasms, originating from the muscle aponeurosis and classified as deep fibromatoses. The aim of this study was to evaluate the utility of multi-parametric (mp)-MRI for the diagnosis of abdominal wall desmoid tumor (awdt). Material and methodsThis Institutional review board approved retrospective study compared 10 patients (mean age±SD; 38.2±13years; 9 females and 1 male) with awdt to 14 subjects (mean age±SD; 45.6±14.7years; 9 females and 5 males) with non-desmoid abdominal wall tumors (ndawt). All included subjects underwent mp-MRI, which included conventional, diffusion weighted and dynamic contrast-enhanced (DCE) MRI. Two blinded experienced fellowship trained radiologists (MK and SR) evaluated each lesion characteristics qualitatively and quantitatively which included margin, homogeneity, T2W/T1W signal intensity (SI), T2 dark strands, and fascial tail together with measurements of apparent diffusion coefficient (ADC) and semi-quantitative DCE analysis. Inter-observer agreement was assessed using Cohen’s kappa and data were compared between groups using independent sample t-tests and Chi-square tests. ResultsNo significant differences in age or gender appeared between groups. On qualitative analysis, T2 dark strands were identified in 90% by both radiologist (K=0.82) of awdt, while fascial tail was identified in 70% by radiologist 1 and 80% by second radiologist (k=0.91) of awdt; however no other lesions showed these findings. Other subjective imaging findings did not significantly differ between groups with moderate-to-strong agreements (k=0.7–1.0). On quantitative measurements, diffusion imaging awdt lesions showed higher mean ADC value compared to other lesions, although it did not reached at the level of significance. While on DCE MRI, all awdt lesions showed type 1 (progressive) DCE curve, however no significant difference was observed between groups. ConclusionsT2 dark strands and fascial tail are characteristic features of awdt, whereas other subjective/qualitative findings are not useful. Quantitative findings such as ADC measurements and DCE curve analysis may have additional value to differentiate awdt from ndawt, but will require further analysis.

腹壁の皮下に発生したatypical lipomatous tumorの１例

腹壁の皮下に発生したatypical lipomatous tumorの１例

Impact of fetal counseling on outcome of antenatal congenital surgical anomalies.

To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39weeks (median 24;32weeks). Diagnoses in ACSA;PACSA included urological (26;31), neurological (10;5), congenital diaphragmatic hernia (CDH)(5;1), gastrointestinal (5;5), lung and chest anomalies (5;1), intraabdominal cysts (4;1), abdominal wall defects (4;0), tumors (3;3), limb anomaly (1;1), esophageal atresia (1;1), conjoint twins (1;0), hepatomegaly (1;0), and major cardiac anomalies (2;0). Two antenatal interventions were done for ACSA; vesicoamniotic shunt and amnioinfusion for oligohydramnios. 17;24 ACSA;PACSA required early surgical intervention in post-natal period. Nine ACSA underwent medical termination of pregnancy and 4 had intrauterine demise. Nine ACSA babies died including two CDH, one gastroschisis, one duodenal atresia, one conjoint twins, one megacystitis with motility disorder and three posterior urethral valves. All PACSA babies survived. Fetal counseling for CSA portrays true outcome of ACSA with 32.3% (22/68) mortality versus 0% for PACSA due to selection bias. However, fetal counseling ensures optimal perinatal care.

PWE-099 Desmoid Tumours in Familial Adenomatous Polyposis: Our Experience

Introduction Desmoid tumours are rare, benign, non-metastasising fibromatoses that affect 8–23% of patients with FAP. Desmoid disease poses significant morbidity through local compression and obstruction. There is an increased risk in females, pregnancy, those with germline mutation of the APC gene beyond codon 1399 and previous abdominal surgery. Management requires a balance of chemoprophylaxis and careful surgical intervention. We aimed to evaluate our local investigation and management of patients with FAP and desmoid tumours with respect to published guidance. Methods Patients with FAP who are on the colorectal database who were known to have desmoid disease were identified. Data was collected retrospectively from case notes, ICE and PACS systems. Management of each patient was compared to the St Mark’s Polyposis Registry guidance and published literature. Results There are 41 patients with FAP in our colorectal database (25 female, 16 male). Eleven patients from eight families were identified as having desmoid disease (six female, five male). Ten of these had desmoid tumours; one only postoperative desmoplastic reaction. The median age at diagnosis was 33 (range 8–49). Of the 10, all had undergone colectomy (five restorative proctocolectomy, three ileorectal anastomosis, two panproctocolectomy (eight prophylactically, two for malignancy). Three patients have had excision of desmoid tumours; all abdominal wall tumours. Six patients have been managed conservatively with observation, chemoprophylaxis and ureteric stenting for managing complications of compression. All patients have been kept under surveillance. One patient died following bowel perforation, one following recurrent bowel obstruction with inoperable mesenteric desmoid disease. Genetic testing was performed on the majority but specific mutational analysis was available for only 67% of patients. 6 of 8 of the patients with mutations in the desmoid region have developed desmoid tumours. Conclusion Mortality in our patient group from desmoid disease is higher than that from colorectal cancer and periampullary tumours, in line with national reported data. Prophylactic colectomy has been suggested to increase risk of developing desmoid disease especially with mutations in the desmoid region. Our results show that our patients are being managed appropriately but knowledge of location of germline mutation is strongly advised prior to offering prophylactic colectomy and management of solid desmoid tumours. In those with a higher risk of developing desmoid tumours, there may be an argument to continue endoscopic surveillance for as long as possible. Reference 1 Speake, et al . Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations. Br J Surg 2006; 93 (Suppl 1):21. Disclosure of Interest None Declared

Pulmonary benign metastasizing leiomyoma

Pulmonary benign metastasizing leiomyoma (BML) is a rare disorder affecting women with a history of uterine leiomyoma metastasizing to lung. These lesions present either as a multiple benign synchronous or metachronous nodules. We report a case of 53-year-old postmenopausal women who presented with multiple bilateral pulmonary nodules on a chest X-ray during an evaluation for dyspnea, 11 years after a hysterectomy due to uterine fibroids and excision of abdominal wall tumor. Chest computed tomography (CT) revealed multiple well-defined soft tissue enhancing mass lesions in both lungs, with the largest one in the right lower lobe. She underwent right side bilobectomy by thoracotomy. Pathologically, the resected lesion consisted of benign spindle cells and was diagnosed as smooth muscle tumor of unknown potential which includes BML [1]. Other lung nodules have been meticulously monitored at follow-up, and repeat X-ray after 1 year later showed that these nodules had not increased at all in size and that no new lobe nodules had appeared. She did not receive any adjuvant hormonal therapy because of her postmenopausal status.

Huge abdominal wall reconstruction with island pedicle anterolateral thigh flap with tensor fascia lata plus vastus lateralis muscle

Full-thickness abdominal wall defects may result from trauma, tumor resection, or infection. Management of these defects poses a significant challenge. Few studies have been conducted on using the pedicle anterolateral thigh (ALT) flap to reconstruct a defect of over half the anterior abdominal wall. We report a large abdominal wall reconstruction with island pedicle ALT flap with tensor fascia lata plus vastus lateralis muscle. An 80-year-old man with a large recurrent abdominal wall tumor had undergone two tumor resections after 2010. The pathology report demonstrated fibromatosis in 2010. Recently, computed tomography revealed a large, lobulated mass measuring approximately 11.8 cm × 5.3 cm × 8.8 cm, with heterogeneous enhancement, located at the left abdominal wall with cutaneous and subcutaneous involvement and adjacent omentum thickening. Wide excision and subsequent reconstruction of the abdominal wall defect with a pedicle ALT flap was conducted. The permanent histology report confirmed fibrosarcoma. Partial skin necrosis was noted after reconstruction surgery, and a second operation was performed to debride the necrotic skin. Wound healing was optimal, without any recurrence. Large primary abdominal fibrosarcoma is rare; thus, reconstruction of the postoperative defect is particularly challenging for plastic surgeons. In this case, we successfully used the pedicle ALT flap with tensor fascia lata plus vastus lateralis muscle to reconstruct the abdominal wall defect in the patient. This is an effective yet simple single-stage reconstructive procedure for defect repair.

Desmoid Tumours in Familial Adenomatous Polyposis: Review of 17 Patients from a Portuguese Tertiary Center.

Desmoid Tumours (DT) are benign tumours with an estimated incidence of 2-4 per million per year. Between 7-16% of them are associated with Familial Adenomatous Polyposis (FAP) and are mostly parietal or intra-abdominal. They are a challenge in relation to their unpredictable natural course, associated complications and difficult treatment. The aim of the present study was to review the occurrence, management and follow-up of DT on FAP patients treated consecutively at a tertiary care center. A retrospective review of clinical data from patients treated consecutively between 1993 and 2014. Patients' data was gathered from clinical records. Data collection included the following variables: demographic data, genotype, FAP phenotype, data on FAP related surgery, DT diagnosis, location, size and number, DT treatment, patients' status and follow-up data. The study population consisted of 17 patients from 9 families; with a mean age of 41 years, mostly women (59%) and most with a mutation either on codon 232 or 554. Most tumours had an intra-abdominal component (59%) with a mean size of 5cm. Fifteen patients were first treated with pharmacotherapy (Non-steroidal Anti-inflammatory Drugs (NSAIDs) and Tamoxifen). Five patients (29%) underwent surgery, 4 of them for complications of intra-abdominal tumours and 1 patient for abdominal wall tumours. Two patients underwent chemotherapy in relation to aggressive intra-abdominal disease. The mean follow-up time since diagnosis of DT was 123 months. Overall, 2 patients had remission, 11 patients had regression or stabilized disease, and 2 patients had progression. One patient died due to surgical complications. Diagnosis of DT is based on clinical symptoms, without the need for screening, although imaging plays an important role once diagnosis is suspected. The treatment approach is conservative on most patients, leaving surgery for DT related complications. The follow-up of patients with DT is also based on clinical symptoms.

Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo

Tumors involving the abdominal wall arise from a large number of etiologies. This review article describe a diagnostic approach with macroscopic, histopathological and immunohistochemical features of the most common causes of abdominal wall masses. We propose a diagnostic algorithm for abdominal wall tumors which combines clinical data and morphologic appearances to classify each lesion.

Advanced endocervical adenocarcinoma metastatic to the ovary presenting as primary ovarian cancer

Dear Editor,Cervical squamous cell carcinoma (SCC) and adenocarcinomamay involve the ovary. Although it is not difficult to diagnose inmost cases, on very rare occasions the presentation may showsymptoms related to an ovarian mass, with the cervical cancerbeing undetected, especially endocervical adenocarcinoma [1,2].This is important because the challenge of diagnosing primaryendocervical adenocarcinoma occurs especially in women whoshow no symptoms, and have a grossly normal cervix and anegative Pap smear [3]. The spread of cervical malignancy (bothSCC and adenocarcinoma) is often through lymphatic drainage ordirect invasion [4]; except some reports show that endocervicaladenocarcinoma carries a higher risk of ovarian metastases thanSCC of the cervix [5]. There is an apparent difference in the treat-ment strategy for advanced cervical cancers and ovarian cancers[6,7]. Therefore, making an accurate diagnosis before planningtherapy is of paramount importance. The following is a case reportof advanced cervical adenocarcinoma metastatic to the ovarymimicking primary ovarian cancer.A 54-year-old menopausal woman (G3P3) visited the emer-gency room due to acute sudden onset of abdominal pain afterseveral weeks of abdominal fullness. Her past medical history wasunremarkable. She did not have any Pap smears since the birth ofherlastchild(28yearspreviously).Physicalexaminationrevealedaprotuberant and tense abdomen, but the cervix was essentiallynormal. Transvaginal ultrasound revealed a 15 cm complex cysticmass lesion located at the right adnexal area accompanied withmassive ascites, but the uterus and the left ovary seemed to benormal.Computedtomographyidentifiedandconfirmed theabovefinding (Fig. 1). Serum tumor markers, including CA 125, CA 199,and CEA were 286.0 U/mL, 209.0 U/mL, and 226.0 ng/mL, respec-tively. Other investigations, including hematological andbiochemical tests, a chest X-ray, and upper and lower gastrointes-tinal (GI) tract evaluations were within normal limits.Under the diagnosis of ovarian malignancy, the patient under-went a laparotomy. During surgery, a right ovarian cystic complexmass with a mucinous component (Fig. 2) accompanied withmassive ascites (7000 mL) and peritoneal carcinomatosis wasfound. The immediate frozen pathology report favored a diagnosisof adenocarcinoma-mucinous type. Therefore, the patient under-went an optimal debulking surgery, including total hysterectomy(Fig. 3), bilateral salpingo-oophorectomy, infracolic omentectomyand retroperitoneal lymph node sampling, and multiple biopsies.However, the final pathologic report was primary uterine endo-cervical adenocarcinoma.Pathologic features included hyperchromatic dysplasia of themucinous glandular cells of the uterine endocervix; the mucinoustumor occupied the whole layer of the endocervix and extended tothe lower segment of the uterine body. Other sections of the rightovary, appendix, omentum, abdominal wall, and mesentery allshowed tumor metastases with floating mucinous tumor cellswithin an extensive mucin pool.The patient was treated with adjuvant systemic chemotherapy(8 cycles of topotecan [Hycamtin Injection, GlaxoSmithKline, San

Buschke Löwenstein Tumor of the Right Lower Abdominal Wall: Case Report and Review of the Literature

Buschke-Löwenstein tumor (BLT), known as giant condyloma acuminatum (GCA), is a very rare disease that typically appears as a penile lesion but can although appear in the anogenital region, bladder, vulva, scrotum and sacrococcygeal area as well. Despite of its histologically benign signs, a high recurrence rate, invasiveness and destructive growth characterizes this rare disease as clinically malignant. Malignant transformation into verrucous carcinoma (VC) and squamous-cell carcinoma (SCC) have been described as well. Many treatment modalities inclusive neoadjuvant radio-and chemotherapy and topical treatment have been reported but due to lack of controlled studies no treatment can be recommended. We present a case of Buschke-Löwenstein tumor involving the right lower abdominal wall of the colostomy region at a 71 years old male. To our knowledge, we first describe a case report of GCA involving abdominal wall at the colostomy region, successfully treated by wide radical excision and plastic reconstruction.

Symptomatic schwannoma of the abdominal wall: A case report and review of the literature.

Schwannoma is a rare, benign tumor that arises from the nerve sheath. This tumor usually involves the extremities, but can also be found in the head and neck, trunk, pelvis, retroperitoneum, mediastinum and gastrointestinal tract. In numerous cases, the tumors are asymptomatic and are identified incidentally on physical examination or imaging. Occasionally, schwannoma is symptomatic due to compression of surrounding large nerves. In the present study, a 57-year-old female presented to the surgical outpatient’s department due to a well-localized parietal pain in the left lower quadrant. The onset of the pain occurred three years prior to presentation, without apparent cause and in the absence of other symptoms. Ultrasound and a computed tomography scan revealed a small solid tumor in the anterior abdominal wall, which was dimensionally stable over time, but was not noted in a preliminary analysis by a radiologist. The lesion was surgically removed using an anterior surgical approach. Histopathology revealed the tumor to be benign schwannoma. The painful symptoms completely disappeared. To the best of our knowledge, this is the third case of an abdominal wall benign schwannoma in the medical literature, and the first symptomatic case.

Recidivant desmoide fibromatosis of anterior abdominal wall et desmoide fibromatosis of retroperitoneum: Case report

Desmoide or aggressive fibromatosis belong to the group of benign tumor characterized by a locally infiltrative growth and tendency to recur and which do not metastasize. Here is shown 46 years old woman who is coming for examination because of recurrent tumors of the anterior abdominal wall, PH earlier verified as desmoide fibromatosis. With a tumor of the anterior abdominal wall measuring 14.7 x 7.0 x 12.5 cm, CT diagnostics discovered a tumor size of 5.8 x 8.8 cm 6,2x in the lodge earlier removed right kidney, which is in close communication with v. cava inferior (VCI) and A. Illiaca comunis dex. (AIC dex.). Compression of VCI and AIC has resulted in swelling of the right leg as a whole who retired after excision of the tumor. The postoperative course was uneventful.

腹壁に発生した悪性脂肪形成性孤在性線維性腫瘍の 1 例

腹壁に発生した悪性脂肪形成性孤在性線維性腫瘍の 1 例

Desmoid tumor in pregnancy: A case report

Desmoid tumors (DTs) constitute a rare fibroblastic proliferative disease. In a female patient presenting a tumor of the lower abdominal wall especially after cesarean section, an endometriotic tumor as well as an aggressive DT should be considered. Here we report a case of pregnancy associated with an anterior abdominal wall DT in a second gravida with previous cesarean, being the first such case from the northeastern region. DTs are histologically benign neoplasms arising from the connective tissue sheath of striated muscles but may exhibit locally aggressive growth patterns and a high rate of recurrence. Large percentages of DTs in females arise in and around pregnancy, perhaps related to trauma from abdominal stretching during fetal growth and due to estrogen levels. It is not clear from currently available data whether pregnancy-associated desmoids are molecularly distinct from other desmoids. Currently, surgery is the most favored treatment for DTs.

A retrospective analysis of surgical treatment of mesh infection after repair of ventral hernia or defect.

This study aimed to summarize our experience in surgical treatment of mesh infection after repair of ventral hernia or defect. A retrospective analysis was conducted on clinical data of 22 patients who accepted surgical treatment of mesh infection after ventral hernia or defect repair. Included were 16 cases of infection after incisional hernia repair, 5 cases of infection after abdominal wall defect repair following abdominal wall tumor resection, and 1 case of infection with fistula caused by a parastomal hernia of an ileal neobladder repair with a prosthetic patch. All patients had received local dressing treatment for 2 to 24 months but were not healed. The affected mesh was removed successfully in all patients. Six patients had abdominal wall repair using the component separation technique; 4 patients were treated by strengthened repair with polypropylene mesh; 10 patients were repaired with human acellular dermal matrix; 1 patient received local dressing changes and vacuum sealing drain treatment without repair; and 1 patient received wound closure without strengthened repair. The postoperative hospital stay was 9-29 days (mean 16 days). After treatment, 19 patients recovered with primary wound healing and 3 patients recovered with secondary healing. All patients were followed up for 6-38 months (mean 26 months), and no ventral hernia or defect recurred except 1 case of lower abdominal bulge. Mesh infections after ventral hernia or defect repair are difficult to treat using prosthetic materials. For satisfactory results, surgery should be performed according to the specific condition of the individual.

Modern reconstructive techniques for abdominal wall defects after oncologic resection.

Resection of abdominal wall tumors often leaves patients with debilitating soft tissue defects. Modern reconstructive techniques can be used to restore abdominal wall integrity. In this article, we present an overview of preoperative patient evaluation, analysis of the defect, surgical planning, and the spectrum of available surgical techniques, ranging from simple to complex. The established clinical evidence in the field of abdominal wall reconstruction is summarized and a case example is provided.