Abscess or Tumor? When a Retroperitoneal Mass on Computerized Tomography Turns Out to Be a Rare Soft Tissue Growth

A retroperitoneal finding on a computerized tomography scan, in a symptomatic patient, can harbor the clinician to many differential diagnoses from infectious to malignancy. Desmoid fibromatosis (DF), a relatively innocuous mass that can spread locally, can be found in that anatomical region. Even for a rare tumor such as DF, our patient did not meet the usual benchmarks of this cancer, being an elderly female and having it appear as an abscess radiologically. Timely clinical response with radiotherapy and tamoxifen allowed our patientÂ’s DF to regress and resolved her symptoms.

Successful resection of intra-abdominal tumors using indocyanine green (ICG) fluorescence imaging has not been reported. Here, we report a rare case of an intra-abdominal desmoid-type fibromatosis successfully resected using this technique after intersphincteric resection (ISR) for rectal cancer. One year after ISR for rectal cancer in a 47-year-old man, computed tomography showed a 50-mm intra-abdominal tumor near the left common iliac vein. Surgical resection was performed. The tumor was located in the mesentery of the remnant rectum after ISR. ICG fluorescence imaging confirmed the blood supply to the mesentery of the distal remnant rectum after tumor excision. The anal canal was successfully preserved without creating a permanent colostomy. The tumor was safely resected with resection margins, diagnosed as desmoid-type fibromatosis. No tumor recurrence was observed 6 months post-resection. This was the first case report to demonstrate the utility of this technique for an intra-abdominal tumor resection.

Desmoid tumours are rare and locally invasive neoplasms that originate from the muscles and their aponeurosis. Incomplete excision causes recurrences; therefore, patients require aggressive resection that essentially entails tumour excision with a clear surgical margin. After radical resection, the resultant wide defect may lead to difficulty in closure of the anterior abdominal wall. Here, we report a case having surgery for large desmoid tumour of the anterior abdominal wall through an abdominoplasty incision followed by an abdominal wall reconstruction with a dual-sided composite mesh.

Introduction: Desmoid-type fibromatosis (DF) is a rare tumor that develops in the limbs, abdominal wall, and abdominal cavity. It is accounting for less than 3% of soft-tissue sarcomas and less than 0.03% of all neoplasms. Presentation of case: A 57-year-old man was diagnosed as acute peritonitis due to intra-abdominal tumor rupture. Since his systematic symptoms were relatively stable, gastrointestinal perforation was ruled out, the differential diagnosis of the tumor itself was difficult, and it was unclear resectable by emergency surgery, we started conservative treatment. After examinations, ileocolectomy was performed. Histopathological examination revealed spindle cells with collagenous fiber hyperplasia and immunohistochemical staining for ?-catenin was positive, so we made a diagnosis of mesenteric desmoid-type fibromatosis (MDF). Discussion: The mechanism of DF development is suggested to be associated with hereditary diseases, mechanical stimuli, and a history of exposure to radiation appear to be involved as pathogenetic factors in sporadic development. Surgical resection is the first-line treatment for MDF, but the postoperative high local recurrence rate is problematic. Drug therapy and radiation therapy are selected for cases in which radical resection is not possible or for recurrent cases. However, the number of examined cases is small and sufficient evidence has not been accumulated for most treatment strategies, it is expected that the optimal treatment at the time of recurrence will be further verified by the accumulation of MDF. Conclusion: There are few reports of peritonitis caused by MDF rupture; emergency surgery can be avoided.

Objective: The objective of this study was to retrospectively review clinical data, management protocols, and clinical outcomes of patients with fibromatoses of head and neck region treated at our tertiary care center. Methods: We retrospectively reviewed the medical records of 11 patients with confirmed histopathological diagnosis of fibromatosis registered in the Department of Head and Neck Surgery at Tata Memorial Centre, India, between 2009 and 2019. Various clinical and pathological features and treatment modalities were evaluated. Results: Age at diagnosis ranged between 18 and 74 years, with a median age of 36 years. The female-to-male ratio was 5:6. Supraclavicular fossa (n=4) was the most common subsite of origin in the neck (n=8). The lateral (n=2) and posterior cervical regions (n=2) were other common neck subsites. Less commonly involved sites were the mandible (n=1), maxilla (n=1), and thyroid (n=1). A total of eight patients underwent surgery at other centers before being referred to us for further management. Out of a total 11 patients, nine patients had unresectable disease at presentation. Six of the patients with unresectable disease received a combination of weekly doses of vinblastine 6 mg/m2 and methotrexate 30 mg/m2 for a median duration of 6 months (range 6-18 months) followed by hormonal therapy with tamoxifen. Three patients received metronomic chemotherapy followed by hormonal therapy. One treatment-naive patient with fibromatosis of posterior cervical (suboccipital) region underwent R2 resection (excision of bulk of the tumor with preservation of critical structures) at our center along with adjuvant radiotherapy. One pregnant patient reported to us after undergoing surgery outside and defaulting radiotherapy. During median follow-up of 29 months (range 1-77 months), six patients had stable disease, and four patients had disease reduction. Disease progression was seen in one patient. The two-year progression-free survival (PFS) was 90% (95% CI 70%-100%). Conclusion: Gross residual resection (R2) was the mainstay of surgical treatment in our series, as obtaining clear surgical margins is seldom possible in these locally aggressive tumors. Radiotherapy, chemotherapy, and hormonal therapy are the other preferred and more conservative treatment modalities. The goal of surgery should be preserving function with minimal or no morbidity. As fibromatoses in the head and neck region are extremely rare, their treatment awaits the development of standard treatment protocols.

Purpose: The aim of this study was to determine outcomes and prognostic factors for patients with primary and locally recurrent extra-abdominal desmoid tumors who underwent percutaneous cryoablation, and to compare with patients treated with surgery. Methods: Group characteristics were compared using Fisher’s exact test, and propensity score matching was performed using the nearest-neighbor approach. Kaplan-Meier and log-rank analyses were used to evaluate the variation in first local recurrence and disease control, while multivariate Cox regression was used to identify factors associated with first local recurrence. All statistical tests were two-sided and a p-value of 0.05 was considered statistically significant. Results: Twenty-two cryoablation patients were matched with 33 surgical patients (n = 55). Median follow-up after cryoablation was 16.3 months versus 14.9 months after surgery. Two-year local recurrence-free survival (LRFS) was 59% after cryoablation and 71% after surgery, and median LRFS was 26.6 months after cryoablation but was not reached after surgery. Two-year disease control for all patients was 85%, however median disease control was not reached in either the cryoablation or surgery groups. There was no significant difference in LRFS or disease control between matched cryoablation and surgical patients. No local recurrences occurred after the first cryoablation in patients with zero or one of the following risk factors: tumor size > 5 cm, age ? 25 years, or locally recurrent disease. All patients with two or more of these risk factors recurred locally after the first cryoablation. Conclusion: Percutaneous cryoablation of primary and locally recurrent extra-abdominal desmoid tumors provides freedom from first local recurrence and long-term disease control comparable with surgery.