Breast fibromatosis: Making the case for primary vs secondary subtypes

Fibromatosis of the breast is a rare condition that can be locally aggressive. The mainstay of treatment remains wide local excision, with varied adjuvant therapy as needed. The authors describe their experience in the treatment of a series of patients and propose the classification of primary and secondary breast fibromatosis. A single-institution retrospective analysis of patients treated for breast fibromatosis from 2003 to 2017 was completed. Primary breast fibromatosis was defined as arising in the absence of previous surgery or radiation therapy to the ipsilateral breast. Secondary breast fibromatosis was defined as arising in the setting of previous surgery or radiation therapy to the ipsilateral breast. A total of 16 patients were included with the median age 40 (28-64) years. The average size of the lesion was 6.37 cm (1.5-15 cm). Mean follow-up time was 65 months. Surgical excision was completed in 14 patients, with two recurrences. There were no recurrences in patients with surgical margins >1 cm. Two patients were treated nonsurgically. There were seven patients with primary fibromatosis of the breast and nine patients with secondary fibromatosis of the breast. We advocate for the multi-disciplinary treatment of this disease process with an aggressive surgical approach to achieve margins >1 cm.

We present a patient with an intra-abdominal desmoid tumor diagnosed 20?month after laparoscopic resection of rectal cancer. A 70-year-old woman with hematochezia was diagnosed with advanced rectal cancer. Preoperative chemoradiotherapy followed by laparoscopic low anterior resection was performed. During follow-up, a nodular soft-tissue density measuring 28?mm was detected in the presacral region. Metastasis from rectal cancer was diagnosed and four courses of chemotherapy were given, including capecitabine, oxaliplatin and bevacizumab. Computed tomography scan showed that the mass slightly decreased in size and surgical resection was performed. Histopathological examination revealed a proliferation of spindle-shaped cells and collagenous stroma diagnosed as a desmoid tumor. This report highlights the possibility of a desmoid tumor in the differential diagnosis of an intra-abdominal mass found during follow-up after resection of colorectal cancer including following laparoscopic resection.

Papillary thyroid cancer with desmoid-type fibromatosis (PTC-DTF) is an uncommon tumor characterized by extensive stromal proliferation of fibroblasts and myofibroblasts with a small component of PTC. We report a case of PTC-DTF with infiltration of the mesenchymal component of tumor into perithyroidal muscle and early recurrence of desmoid after thyroidectomy, an outcome previously not reported. A 20-year-old man underwent left hemithyroidectomy for a thyroid nodule. Pathology demonstrated a 4.2?cm tumor with PTC-DTF with the PTC comprising <10% of the tumor. The stromal component extended into adjacent skeletal muscle. After completion thyroidectomy, histopathology of the right thyroid lobe revealed no malignancy or fibromatosis. Neck MRI 16 months after the initial operation revealed a 10.5?cm tumor in the left thyroid bed. Core biopsy and open excisional biopsy showed desmoid-type fibromatosis without PTC. The patient is undergoing chemotherapy of his recurrent desmoid-type fibromatosis. In patients with PTC-DTF there is a risk of recurrence of the benign component of the tumor. In recent reports, the role of less aggressive surgery, or even non-surgical management, of patients with recurrent DTF has been emphasized, in particular when extensive surgery may be associated with high risk of functional loss. The management of our patient adheres to modern recommendations for the treatment of DTF. Patients with PTC-DTF should be carefully monitored after thyroidectomy for both recurrent PTC and local recurrence of the fibrous component of the tumor.

We review the role of hormonal therapy in the management of different conjunctive tumors. Progestin and aromatase inhibitors seem active in low-grade endometrial stromal sarcoma, but larger case-series are needed. The use of aromatase inhibitor in estrogen-receptor-positive uterine leiomyosarcoma requires further clinical investigation. There is no evidence supporting the use of hormonal therapy in desmoid-type fibromatosis.

Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies.This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

There is currently no standard treatment for desmoid tumors (DTs) associated with familial polyposis coli (FAP). Familial adenomatous polyposis in DT patients is sometimes a life-threatening condition. We enrolled all consecutive patients with FAP treated at Unit of General Surgery and Transplant, University of Naples Federico II and evaluated the incidence of DTs on FAP between 1996 and 2016. We observed 45 consecutive patients with FAP; of these 5 were DT-FAP-associated. All 5 cases with FAP were young women, age 25 to 65 years, previously treated by colectomy. Of these, 4 patients presented a parietal localization and had been treated with a wide surgical exeresis; one patient had an intra-abdominal, mesenteric tumor that was unresectable at laparotomy. We performed CT-guided drainage, ureteral stenting, medical therapy (sulindac + tamoxifene), and chemotherapy (dacarba-zine+doxorubicine). All patients were alive and underwent follow-ups for 5 years post-surgery; only 1 patient with parietal localization showed a local relapse after 2 years. We propose a modulated approach to the single patient with FAP, with surgery as treatment of choice for parietal localization disease and integrating different kinds of therapies (surgery alone or associated with RT, CT) for the intra-abdominal tumor.