Basaloid Follicular Hamartoma of the Eyelid: A Case Report and Literature Review about an Unusual Lesion in the Ocular Region

Basaloid follicular hamartoma (BFH) is a normally benign, uncommon, malformative lesion involving the hair follicles, which usually poses challenges in the differential diagnosis with other benign and malignant tumours, especially basal cell carcinoma, due to significant clinical and morphological overlap. Here, we report the case of a 53-year-old male who presented with a mass in the upper left eyelid evolving for one year. The patient had a previous history of total colectomy and an abdominal desmoid tumour within the context of Familial Adenomatous Polyposis (FAP), with a documented germline mutation in the Adenomatous Polyposis Coli (APC) gene. The eyelid lesion was biopsied and the histological analysis of the three small tissue fragments received revealed fragments with cutaneous–conjunctival lining displaying a subepithelial proliferation of basaloid nests with peripheral palisading, compatible with primitive hair follicles. There were images of anastomosis between different basaloid nests, which had their connection to the epithelial lining preserved. The stroma had high cellularity and sometimes primitive mesenchymal papillae were evident. Pleomorphism was absent, mitotic figures were barely identified, and no necrosis was seen. The basaloid nests did not have epithelial–stromal retraction nor mucin deposits. A diagnosis of BFH was proposed, which was later confirmed after surgical excision of the whole eyelid lesion. No evidence of carcinoma was present. This case illustrates the main features of the rare benign eyelid BFH. The standard medical or surgical approach of these lesions remains to be firmly established. Nearly nine months after surgical excision our patient remains well without signs of disease recurrence.

Surgical management of a giant abdominal wall desmoid tumour

Desmoid tumours are clonal fibroblastic proliferations in soft tissues, characterised by infiltrative growth and local recurrence, but not metastasis. Various treatment strategies for desmoid tumours exist, varying from observation, medical and systemic therapy to radiotherapy and surgery. A 25-year-old woman with a background of familial adenomatous polyposis was referred with an enlarging abdominal desmoid tumour measuring 40×40×40 cm despite repeated radiofrequency ablation, surgical debulking and hormone therapy. The patient had a two-stage operation. The first stage involved excision of the desmoid tumour with full-thickness abdominal wall. The abdominal wall was not closed, and a topical negative pressure seal was applied. After 2 days, she underwent the second stage: reconstruction of the abdominal wall defect with a large porcine mesh which was covered with anterolateral thigh flaps. Postoperative complications included ileus and a fall which required further surgery. The patient was discharged 1 month after the first operation. Abdominal MRI scans were performed at 3 and 7 months postdischarge and showed no recurrence of diseaseBackground

Caecal desmoid tumour – A rare tumour at uncommon location and review of literature

A 49-year female presented to surgery outpatient clinic with the awareness of a right lower abdomen asymptomatic lump for one week. Contrast enhanced computed tomography of the abdomen demonstrated a 10x11x15cm heterogeneous lesion anterior to the ileocecal junction with the possibility of leiomyosarcoma. Patient was prepared for laparotomy and intraoperative there was a large tumour arising from the medial wall of cecum. Right hemicolectomy was performed, and histopathology came as a desmoid tumour of the cecum. The patient had an uneventful postoperative course and is well on one year follow up.

Giant abdominopelvic desmoid tumour herniated trough perineum: a case report

Desmoid tumours are deep aggressive fibromatoses that usually arise in the soft tissues of the limbs or the abdominal wall. Intra-abdominal localisation, rarely occurs and their treatment may be challenging. When necessary, surgery must be personalized to what is achievable in terms of margins while preserving functional outcomes. This condition is illustrated herein with the case of a 40-year-old female presenting an unusually large sporadic desmoid tumour with abdominal, pelvic and perineal involvement. Resection was performed without organ involvement through a combined perineal approach. Tumour resection was macroscopically completed except in the perineum, where the tumour was left (R2 resection) to preserve anal sphincter. Adjuvant treatment with tamoxifen was given to achieve local control. The hormonal treatment was well tolerated, and no recurrence was observed after 36 months of follow-up.

Intra-abdominal fibroid lesion of colonic involvement

Fibroid is a benign lump of growth, occurring within or outside the uterus. It is a rarity for such growths to occur intra-abdominally. Such cases are of aggressive fibromatosis which are marked by the presence of desmoid tumours. It is most commonly seen in patients with a history of familial adenomatous polyposis (FAP) or past surgical procedures. As FAP has a wide fibroepithelial growth spectrum, the symptomatology and therefore the prognosis varies. Unpredictable clinical behaviour, varied location and non-specific presentation are the factors accounting to diagnostic difficulty. Therefore, misdiagnosing the disease is not uncommon. Here we report a case ofa26-year-old pregnant female presenting with lower abdominal pain and mass. Preoperative diagnosis was of uterine fibroids as per radiological evidence, but on exploration it turned out to be a large right colonic mass. The patient underwent right hemicolectomy. Histopathological reports confirmed morphological and immuno-histochemical features indicating fibromatosis. This fibromatous mass though intra-abdominal was not growing from the mesentery/mesocolon or the pelvis. It appeared to grow from the wall of the colon. This is a confirmatory presentation of true colonic wall fibromatosis which is deep and isolated form of intra-abdominal desmoid tumours. As this lesion was isolated in nature, the treatment of choice was surgical resection. It is essential to have a multi-disciplinary team approach in the management of such a patient. This improves the treatment and the prognostic outcomes. Keywords: Fibroid, Colon, Fibromatosis, Hemicolectomy, Desmoid Tumour

A rare case of desmoid fibromatosis of the transverse colon mimicking a perforated malignancy

ABSTRACT Desmoid tumour of the colon is a very rare and aggressive type of intra-abdominal desmoid fibromatosis. Patients can present with a range of symptoms from a mild chronic abdominal pain to those of an acute abdomen. We present a rare case of abdominal fibromatosis that presented as a rapidly growing mass with free intraperitoneal gas. Intraoperatively however, we found a large tumour arising from the wall of the transverse colon and local necrosis. No bowel perforation was noted. The tumour was removed with a wide resection of transverse colon’s wall instead of colectomy. The histopathology reported benign fibromatosis and excluded malignancy.