RESUMO
In front of a primary retroperitoneal tumour, it is necessary to have in mind all possible diagnoses in order to specify the diagnostic strategy and the treatment. According to the World Health Organization (WHO) classification of tumours, mesenchymal benign and malignant tumours (including sarcomas and, currently, neurogenic tumours), parasympathetic tumours, extragonadal germ cell tumours, and lymphoid tumours have been identified. By definition, primary retroperitoneal tumours start independently from the retroperitoneal organs. Secondary lesions, carcinoma metastasis, and adenopathy are excluded from this definition, but they can also develop in the retroperitoneal space and lead to misdiagnoses. In the absence of positive tumour markers or an evocative biology, percutaneous biopsy is necessary. Pathological diagnosis is necessary to decide whether surgery must be done, its timing among the other treatments, and its extension. This paper summarizes all the diagnostic possibilities.
Assuntos
Leiomiossarcoma/cirurgia , Lipossarcoma/cirurgia , Linfoma/cirurgia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Neoplasias Retroperitoneais/cirurgia , Adulto , Biópsia por Agulha , Humanos , Leiomioma/diagnóstico por imagem , Leiomioma/patologia , Leiomioma/cirurgia , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Lipossarcoma/diagnóstico por imagem , Lipossarcoma/patologia , Linfangioma Cístico/diagnóstico por imagem , Linfangioma Cístico/patologia , Linfangioma Cístico/cirurgia , Linfoma/diagnóstico por imagem , Linfoma/patologia , Mixoma/diagnóstico por imagem , Mixoma/patologia , Mixoma/cirurgia , Gradação de Tumores , Inoculação de Neoplasia , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/patologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/cirurgia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neurofibrossarcoma/cirurgia , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Paraganglioma/cirurgia , Cuidados Pré-Operatórios , Neoplasias Retroperitoneais/diagnóstico por imagem , Neoplasias Retroperitoneais/patologia , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/patologia , Rabdomiossarcoma/cirurgia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Sarcoma/cirurgia , Tomografia Computadorizada por Raios X , Carga TumoralRESUMO
The treatment of desmoid tumours (DTs) has greatly evolved in recent years, and surgery is no longer considered a first-line treatment. Percutaneous biopsy with molecular analysis for beta-catenin or APC gene mutation provides a certain diagnosis. After imaging, a specialized multidisciplinary tumour board (MDT) addresses the following therapeutic strategy. As more than half of patients stabilize or regress, despite initial progression, active surveillance is offered to most patients as the first option. Surgery is proposed for complications such as perforation and occlusion, which sometimes are the first manifestations of the disease. In these cases, limiting surgery to the treatment of complications and leaving the tumour in place is possible if significant bowel sacrifice is needed, especially in patients with previous colectomy for polyposis. Medical therapy is discussed by the MDT in cases of functional or life-threatening masses and is preferred to local treatments that could be mutilating and often incomplete. First-line surgery is now contraindicated in cases of incomplete unplanned surgery, recurrence, pregnancy or DTs occurring in familial adenomatous polyposis (FAP). The best indications of second-line surgery are significantly progressing disease when morbidity is acceptable, such as parietal locations. Medical and other locoregional treatments (radiotherapy, isolated limb perfusion and cryotherapy) should be considered by the MTB when surgery might cause sequelae.
Assuntos
Fibromatose Abdominal/patologia , Fibromatose Abdominal/cirurgia , Recidiva Local de Neoplasia/cirurgia , Conduta Expectante , Consenso , Progressão da Doença , Fibromatose Abdominal/diagnóstico , Fibromatose Abdominal/terapia , Humanos , Neoplasia Residual , Seleção de Pacientes , Reoperação , Carga TumoralRESUMO
PURPOSE: To assess the nutritional status and its role in the outcome of patients operated for retroperitoneal liposarcoma (RLS). MATERIAL AND METHODS: Retrospective study on consecutive patients operated with en bloc compartment resection for primary or local recurrence of RLS between 2016 and 2017. Preoperative nutritional and laboratory assessment comprising serum albumin, serum transthyretin, orosomucoid, and CRP was systematically performed. The following preoperative parameters were analysed: weight, body mass index (BMI), significant weight loss (>5% in one month and/or >10% in 6 months), serum albumin, transthyretin, CRP, orosomucoid. PINI (prognostic inflammatory and nutritional index) was calculated. RESULTS: There were 40 patients operated for RLS: 22 women and 18 men with a median age of 61 years (34-90). Median tumour was 280 mm (80-530). Median preoperative BMI was 24.8 (18-42) and median postoperative BMI was 23 (17.8-44). Twenty-one patients (52.5%) were considered to be malnourished: 3 with biological signs of malnutrition and 18 with weight loss. Eleven (47.6%) in the group of malnourished patients and 4 (26.3%) in the group with satisfactory nutritional status developed postoperative complications (p = 0.042). A PINI score>1 was related to significantly longer hospitalisation time 21.8 days (10-58) in comparison with 14.9 [9-30] in patients with PINI < 1, p = 0.003. CONCLUSIONS: The malnourished patients with RLS experienced more postoperative complications and longer hospitalisation. Nutritional status and biological markers contribute to the global management of RLS with improved postoperative behaviour including fewer complications and shorter hospitalisation. A prospective larger study with longer follow-up is necessary to refine these results.