Gastric aggressive fibromatosis: report of a case and review of the literature

A 47-year-old man presented with nonspecific abdominal pain. Gastroscopy revealed stomach wall swelling. An antral gastrectomy was performed. Histological examination revealed spindle-shaped cells and morphology typical of aggressive fibromatosis. We performed a literature search to identify conditions with features similar to those of aggressive fibromatosis. Aggressive fibromatosis does not metastasize, but it is locally invasive and has a tendency to relapse; however, our patient has not had recurrence > 1 year after surgery. Aggressive fibromatosis of the stomach may be confused with an inflammatory fibroid polyp, a gastrointestinal stromal tumor, schwannoma, leiomyoma, inflammatory myofibroblastic tumor, scirrhous carcinoma of the stomach, follicular dendritic cell sarcoma, inflammatory malignant fibrous histiocytoma, myofibroma/myofibromatosis, and solitary fibrous tumor of the stomach. Aggressive fibromatosis of the stomach is a rare spindle cell tumor that must be differentiated from a variety of conditions.

We have presented the case report of a 13 year old boy with aggressive fibromatosis in the left gluteal region involving the supra acetabular portion of ilium and a part of ischium. Clinical examination revealed a firm swelling about 6x5x1 cm in the upper outer quadrant of left gluteal region. X-ray pelvis with both hips revealed an osteolytic lesion in the supra acetabular portion of left ilium. Computed Tomography showed geographic, lytic lesions involving the supra acetabular region of left ilium, quadrilateral plate and a part of ischium. MRI showed a soft tissue mass adjacent to the left ilium, infiltrating in to supra acetabular region of left ilium, and a part of ischium. With these findings our differential diagnoses were chronic osteomyelitis, eosinophlic granuloma and cystic type of fibrous dysplsia. CT scan guided FNAC showed degenerated osteocytes with occasional osteoclastic giant cells and inflammatory mononuclear cells. Then we did a wide local excision of the tumour with curettage of the surrounding bone. Histopathological examination of the specimen was suggestive of aggressive fibromatosis. Considering the hypercellularity and local aggressive behaviour of the tumour we diagnosed it as aggressive fibromatosis. In our two years of follow up the patient did not show any signs of recurrence. This case is being reported for its rarity since fibromatosis involving the bone is an unusual event.

A 25-year-old female patient was admitted to the hospital with complaint of right breast swelling. Physical examination revealed a hard palpable mass in the right breast that was affixed to the chest wall. The patient had undergone right mastectomy for a 2×3.5 cm mass with a histopathological diagnosis of Aggressive fibromatosis (AF) approximately 3 years ago. Contrast enhanced and non-contrast-enhanced MRI of the breast and thorax were performed for the new palpable breast mass and they revealed a 6×9 cm irregular-shaped mass with irregular margins extending into the right breast. The mass was isointense on T1-weighted images (T1-WI) and hyperintense on fat-suppressed T2-WI. MRI demonstrated invasion of the pectoral and intercostal muscles and the ribs. Fine needle aspiration biopsy was performed. Microscopically, the tumor was composed of fibroblastic spindle-shaped cells embedded in abundant collagenous tissue without epithelial components. The tumor cells were immunoreactive for smooth muscle actin and negative for desmin, CD34, and S100. This phenotype was consistent with the diagnosis of fibromatosis. Based on these findings, radiotherapy was recommended first and then a wide surgical excision was decided.

A 2-year-old male presented to the emergency department with a rapidly growing right facial mass. Imaging revealed a 6 × 4 cm enhancing mass centered within the right parapharyngeal space with extensive local tissue compression and osseous remodeling. The patient underwent transoral biopsy that demonstrated both spindle and rhabdoid cells with mitotic figures. Immunostaining was positive for desmin, actin, and beta-catenin, suggesting a desmoid tumor. Given the extent of the tumor and its location, the patient was initiated on a weekly regimen of vinblastine and methotrexate due to concerns for serious cosmetic and functional morbidity with surgery. Over the next 4 weeks, there was an interval increase in size by ?25%, primarily at the biopsy site, and the patient became increasingly symptomatic with intermittent bleeding and episodes of upper airway obstruction. This prompted surgical intervention by a multidisciplinary team comprised of oral-maxillofacial surgery, otolaryngology—head and neck surgery, and microvascular reconstructive surgery. Reconstruction was discussed at length, and a decision was made to defer definitive bony reconstruction because of growth concerns and lack of an adequate anatomic replacement for the mandibular ramus at this age. At completion of surgery, the patient’s face was symmetric with complete oral competence. The postoperative course was uncomplicated with no evidence of recurrence at 1 year. His maximal incisal opening and speech are excellent. He continues to do well, is taking an oral diet, and is pending decannulation in the near future.

We assessed the success rate of MR-HIFU for the treatment of extra-abdominal desmoids at our institute. Five patients with relapsing desmoid tumors (three males, two females; age range 40-79 years) were treated using the Sonalleve system in an outpatient setting without general anesthesia. MR-HIFU was successful in all patients without severe side effects. Follow up ranged from 13 to 60 months. Three patients required more than one treatment session. In 3 patients with small lesions (mean = 9.7 mL), complete ablation was achieved with no evidence of viable tumor on follow up MRI at an average of 35.7 months, while in two patients with larger lesions (mean = 46 mL) the targeted tumor volumes decreased by 73% at 14 months. Skin injuries comprised first- and second-degree burns and were observed with short distance to skin (mean = 0.9 cm) and proximity to bone (i.e. ribs). Skin burns healed within weeks with topical treatment. MR-HIFU shows good mid-term result for extra-abdominal desmoids with complete response for small lesions and stabilization of larger lesions. MR-HIFU for desmoids can be performed under regional anesthesia/sedation as outpatients.

Case 1: A 52-year-old man underwent laparoscopic total gastrectomy for gastric cancer. After 2 years, a follow-up computed tomography(CT) scan showed a large, solid 10 cm-sized mass in his left upper abdomen. Under the diagnosis of a suspected mesenchymal tumor, a tumor resection with a partial resection of the upper jejunum and transverse colon was performed. Case 2: A 61-year-old man underwent laparoscopic pylorus-preserving gastrectomy for gastric cancer. After 1.5 years, follow-up CT showed a tumor of 2 cm in diameter near the greater curvature side of the upper stomach. Under the diagnosis of a suspected gastrointestinal stromal tumor(GIST), a laparoscopic partial resection of the stomach was performed. Histologically, spindle-shaped cells without atypia and rich collagen fibers were observed, and the sample was positive for b-catenin by immunostaining in both cases; from this evidence, the patients were diagnosed with desmoid tumors. Desmoid tumors have invasive proliferation characteristics, and treatment requires consideration of the balance between securing a surgical margin and increasing surgical stress.