Volume 67 - 2004 - Fasc.2 - Case reports
Perianal Paget's disease : case report and review of the literature
Cutaneous Paget's disease (PD) is a rare entity, predominantly involving the breasts. Anal involvement is rather exceptional, and can be associated with underlying malignancies, among which prostate and rectal adenocarcinoma. We report the case of a 71- year-old man suffering from a long history of anal itching, associ- ated with an erythematosquamous lesion of the right buttock extending up to the anus. The diagnosis of perianal PD (PAPD) was confirmed by histopathological demonstration of Paget's cells from a biopsy performed after ineffective topical treatment. Radiological and further clinical inspections allowed us to exclude any synchronous malignancy. A first-step surgery consisted in coe- lioscopic diverting sigmoid colostomy, along with multiple peri- anal, anal and rectal biopsies revealing an anal canal involvement. Coelioscopic abdominoperineal surgery and a wide cutaneous excision were then performed. Histopathological analysis revealed positive posterior margin, requiring further excision. No adjuvant therapy was prescribed, and to this day, after a one-year and a half follow-up, the patient remains disease-free.
Our case report and review of PAPD stress that appropriate management is required to improve the poor prognosis of this rare affection. (Acta gastroenterol. belg., 2004, 67, 228-231).
MR imaging findings in a patient with hepatic veno-occlusive disease
We report the MRI findings in a 31-year-old woman with veno- occlusive disease. MRI demonstrated patent hepatic veins and patchy signal enhancement of the liver after gadolinium chelate injection. This enhancement was compatible with sinusoidal con- gestion. The diagnosis of veno-occlusive disease was confirmed by histological examination of liver biopsy. The diagnosis of veno- occlusive disease should be evoked when patchy liver enhancement suggestive of sinusoidal congestion is observed in the absence of hepatic vein thrombosis and congestive heart failure. (Acta gastro- enterol. belg., 2004, 67, 236-238).
Idiopathic eosinophilic oesophagitis : atypical presentation of a rare disease
A 72 year-old man presented severe dysphagia and weight loss of recent onset. Repeated oesophageal endoscopy and biopsies with macroforceps were normal. oesophageal manometry dis- closed features compatible with achalasia. oesophageal EUS endoscopy localized an infiltrating process between muscular layers of the oesophageal wall and CT scan delimited a circular thickening in the inferior part of the oesophagus. Because of severe clinical presentation mimicking a possible oesophageal neoplasm like a lymphoma, partial oesophagectomy was performed and revealed eosinophilic oesophagitis. This unusual presentation emphasizes that idiopathic eosinophilic oesophagitis must be pro- posed in the differential diagnosis of dysphagia, even in old patient without apparent oesophageal lesion at endoscopy. (Acta gastroen- terol. belg., 2004, 67, 232-235).
Metastatic follicular dendritic cell sarcoma of the stomach : A case report and review of the literature
Follicular dendritic cell (FDC) sarcomas are rare tumours, typ- ically seen in lymph nodes. However, in about one third of the reported cases, a FDC sarcoma presents as an extranodal mass. Involvement of the gastrointestinal tract is extremely rare, and only 3 cases have been described to date.
We report on a 40-year-old female patient with a follicular den- dritic cell sarcoma located in the stomach and the presence of a metastasis in the liver at the time of diagnosis. Severe asthenia, nausea, back pain and loss of weight were the presenting symp- toms. A CT scan of the abdomen and an upper gastrointestinal endoscopy revealed a tumour mass in the stomach.
The diagnosis of a FDC sarcoma was made on histological and immunohistochemical findings. We report the second case of a FDC sarcoma presenting in the stomach.
Due to its rarity, a FDC sarcoma seldom enters the differential diagnosis of spindle cells neoplasms of the gastrointestinal tract. Complete surgical resection is the treatment of choice for FDC sarcoma. (Acta gastroenterol. belg., 2004, 67, 223-227).
