[A 38 years old female with inflammatory myofibroblastic tumor and lactic acidosis]. / Un cas de tumeur myofibroblastique inflammatoire maligne compliqué d'une acidose lactique chez une patiente de 38 ans.

Inflammatory myofibroblastic tumors (IMT) are rare tumors. They were originally described in the lung, but they have been now observed in many others locations, mainly abdominal and pelvic. These tumors are usually benign but their recurrent nature and the presence of an abnormality of chromosome band 2p23 in some of them, suggest that some lesions form a true tumor entity. Surgical excision as complete as possible is the gold standard treatment. We report the case of a 38 years old female, who presented a recurrent metastasizing inflammatory myofibroblastic tumor causing lactic acidosis and other biological abnormalities such as hypercalcemia, hypoalbuminemia, hypoglycemia, disseminated intravascular coagulation and inflammatory syndrome.

Buschke-Löwenstein tumor in a human immunodeficiency virus-positive patient: a case report and short literature review.

Giant condyloma acuminatum, also known as Buschke-Löwenstein tumor, is a rare variant of verrucous carcinoma presenting in the ano-genital region. While its metastatic potential is limited, aggressive local growth is common, with invasion and destruction of the surrounding tissues often causing important therapeutic challenges. Also, data to inform the optimal management approach are scarce and mostly limited to anectodical reports. We present the case of a human immunodeficiency virus-associated locally advanced Buschke Löwenstein tumor that was successfully treated with extensive surgery.

Total neoadjuvant therapy for rectal cancer: Making sense of the results from the RAPIDO and PRODIGE 23 trials.

A few months ago, results from two randomised phase III trials of total neoadjuvant therapy (TNT) in locally advanced rectal cancer were presented (RAPIDO and PRODIGE 23), consistently showing better short- and long-term outcomes with TNT as compared with standard neoadjuvant long-course chemoradiotherapy (CRT) or short-course radiotherapy (SCRT). These results represent corroborating evidence in support of a practice that many centres had already implemented based on promising preliminary data. Also, they provide new, high-level evidence to endorse TNT as a new management option in the treatment algorithm of stage II-III rectal cancer in those centres where CRT and SCRT have long remained the only accepted standard neoadjuvant treatments. Having two consistently positive trials is certainly reassuring regarding the potential of TNT as a general treatment approach. Nevertheless, substantial differences between these trials pose important challenges in relation to the generalisability and applicability of their results, and translation of the same into practical clinical recommendations. In this article, we address a number of key questions that the RAPIDO and PRODIGE 23 trials have raised among the broad community of gastrointestinal oncologists, proposing an interpretation of the data that may help the decision making, and highlighting grey areas that warrant further investigation.

How should we treat mucinous appendiceal neoplasm? By laparoscopy or laparotomy? A case report.

Appendicular mucocele (AM) usually denotes a dilatation of the appendiceal lumen as a result of mucus accumulation that may be related to various neoplastic and non-neoplastic processes. Most of them are discovered incidentally. Treatment consists in complete resection avoiding rupture of the cyst in the peritoneal cavity. Indeed, rupture of such a cystic lesion in the peritoneal cavity can induce a catastrophic complication such as 'pseudomyxoma peritonei' (PMP). Therefore, some authors recommend an open surgical treatment. Currently, the debate concerning the best surgical technique to adopt for AM remains controversial. We report a case of AM found incidentally and treated by laparoscopy. The macroscopic aspect of the appendix suggested the diagnosis intra-operatively and every effort was made to avoid cystic rupture during appendicular resection. The histopathological diagnosis was mucinous cystadenoma. The patient is doing well at 2-year follow-up. The reported case and literature review show us that AM is not a contra-indication for laparoscopic surgery, but major concern resides in the early recognition of such a lesion at laparoscopy and in taking appropriate precautionary measures to avoid rupture in the peritoneal cavity.

A radical approach to achieve complete cytoreductive surgery improve survival of patients with advanced ovarian cancer.

INTRODUCTION: Cytoreductive surgery of locally advanced ovarian cancer has evolved in the last few years from surgery to remove macroscopic residual disease (<1cm; R2b) to macroscopic complete cytoreductive surgery with no gross residual disease (R1). The aim of this study was to evaluate the impact of the adoption of a maximalist surgical approach on postoperative complications, disease recurrence and survival. MATERIALS AND METHODS: This was a retrospective study using prospectively collected data on patients who received either conservative approach (CA) or radical approach (RA) surgical treatment for primary ovarian cancer stage IIIc/IVa/IVb between June 2006 and June 2013. RESULTS: Data for 114 patients were included, 33 patients in the CA group and 68 patients in the RA group were consequently analysed. In the RA group, operative time was longer, in relation to more complex surgical procedures; with more blood losses and a higher rate of compete macroscopic resection. Totally, 77% of the patients had postoperative complications, with more grade I/II complications in the RA group but the same rates of grade III/IV complications in the both groups (P=0.14). For all patient study population, the overall and disease-free survivals were improved in case of no macroscopic residual disease. Overall survival was improved in the RA group (P=0.05), with no difference in terms of disease-free survival (P=0.29) CONCLUSION: A radical approach in advanced ovarian cancer allows a higher rate of complete cytoreductive surgery impacting overall survival. However, a non-significant trend for increased mild complications (grade I/II) rate is observed in this group.

Adjuvant chemotherapy for rectal cancer: Current evidence and recommendations for clinical practice.

While adjuvant chemotherapy is an established treatment for pathological stage II and especially stage III colon cancer, its role in the multimodal management of rectal cancer remains controversial. As a result, there is substantial variation in the use of this treatment in clinical practice. Even among centres and physicians who consider adjuvant chemotherapy as a standard treatment, notable heterogeneity exists with regard to patient selection criteria and chemotherapy regimens. The controversy around this topic is confirmed by the lack of full consensus among national and international clinical guidelines. While most of the clinical trials do not support the contention that adjuvant chemotherapy may improve survival outcomes if pre-operative (chemo)radiotherapy is also given, these suffer from many limitations that preclude drawing definitive conclusions. Nevertheless, in the era of evidence-based medicine, physicians should be guided by the available data and refrain from extrapolating results of adjuvant colon cancer trials to inform treatment decisions for rectal cancer. Patients should be informed of the evidence gap, be given the opportunity to carefully discuss pros and cons of all the possible management options and be empowered in the decision making. In this article we review the available evidence on adjuvant chemotherapy for rectal cancer and propose a risk-adapted decisional algorithm that largely relies on informed patient preferences.

Grey areas and evidence gaps in the management of rectal cancer as revealed by comparing recommendations from clinical guidelines.

BACKGROUND: While the management of nonmetastatic and oligometastatic rectal cancer has rapidly evolved over the last few decades, many grey areas and highly debated topics remain that foster significant variation in clinical practice. We aimed to identify controversial points and evidence gaps in this disease setting by systematically comparing recommendations from national and international clinical guidelines. METHODS: Twenty-six clinical questions reflecting practical challenges in the routine management of nonmetastatic and oligometastatic rectal cancer patients were selected. Recommendations from the ESMO, NCCN, JSCCR, Australian and Ontario guidelines were extrapolated and compared using a 4-tier classification system (i.e., identical/very similar, similar, slightly different, different). Overall agreement between guidelines (i.e., substantial/complete disagreement, partial disagreement, partial agreement, substantial/complete agreement) was assessed for each clinical question and compared against the highest level of available evidence by using the χ2 statistic test. RESULTS: Guidelines were in substantial/complete agreement, partial agreement, partial disagreement, and substantial/complete disagreement for 8 (30.8%), 2 (7.7%), 7 (26.9%), and 9 (34.6%) clinical questions, respectively. High level of evidence supported clinical recommendations in 3/10 cases (30%) where guidelines were in agreement and in 10/16 cases (62.5%) where guidelines were in disagreement (χ2 = 2.6, p = 0.106). Agreement was frequently reached for questions regarding diagnosis, staging, and radiology/pathology pro-forma reporting, while disagreement characterised most of the treatment-related topics. CONCLUSIONS: Substantial variation exists across clinical guidelines in the recommendations for the management of nonmetastatic and oligometastatic rectal cancer. This variation is only partly explained by the lack of supporting, high-level evidence.

Should ruptured liver haemangioma be treated by surgery or by conservative means? A case report.

Spontaneous rupture of a liver haemangioma is a rare but life-threatening acute clinical situation following haemorrhage within the liver, the subcapsular space and the peritoneal cavity in cases of capsular rupture. Rupture of a liver haemangioma has been reported to occur spontaneously in the majority of cases. In the past, prompt surgical treatment was recommended but was associated with high morbidity and mortality. Currently, conservative management and, in cases of recurrent haemorrhage, delayed surgery may be proposed. We report a case of spontaneous rupture of hepatic haemangioma treated by arterial embolisation and conservative means. The literature is also reviewed.

Acute pancreatitis following segmental pancreatic resection associated with portal vein thrombosis and aneurysm of the spleno-mesenteric venous confluence.

Vascular abnormalities associated with acute pancreatitis are well-known and reported in the literature in up to 50% of the patients with acute necrotizing pancreatitis. Most reported vascular abnormalities are superior mesenteric and/or portal vein thromboses and arterial pseudo-aneurysms. Portal vein aneurysm and/or spleno-mesenteric venous aneurysm are rare entities. Furthermore, portal vein aneurysm can be complicated by portal vein thrombosis. We report a case of spleno-mesenteric vein aneurysm that unusually followed portal vein thrombosis and was secondary to postoperative pancreatitis following segmental pancreatic resection for a well-differentiated endocrine carcinoma. The patient was treated successfully with conservative treatment, although he developed a hepatic abscess two months after his treatment.

Indocyanine green fluorescence-guided surgery after IV injection in metastatic colorectal cancer: A systematic review.

OBJECTIVE: Indocyanine green fluorescence-guided surgery (ICG-FGS) has emerged as a potential new imaging modality for improving the detection of hepatic, lymph node (LN), and peritoneal metastases in colorectal cancer (CRC) patients. The aim of this paper is to review the available literature in the clinical setting of ICG-FGS for tumoral detection in various fields of metastatic colorectal disease. METHODS: PubMed and Medline literature databases were searched for original articles on the use of ICG in the setting of clinical studies on colorectal cancer. The search terms used were "near-infrared fluorescence", "intraoperative imaging", "indocyanine green", "human" and "colorectal cancer". RESULTS: ICG fluorescence imaging (ICG-FI) is clearly supported as an intraoperative technique that allows the detection of additional superficial hepatic metastases of CRC. Data on the role of ICG-FI in the intraoperative detection of peritoneal metastases and LN metastases are scarce but encouraging and ICG-FI could potentially improve the staging and treatment of these patients. CONCLUSION: ICG-FI is a promising imaging technique in the detection of small infraclinic LN, hepatic, and peritoneal metastatic deposits that may allow better staging and more complete surgical resection with a potential prognostic benefit for patients.

Hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis of digestive and peritoneal origin: rationale.

Up to a few years ago peritoneal carcinomatosis was considered as an "incurable" disease. The aim of this paper is to review the surgical approach with curative intent to carcinomatosis: it consists of complete resection of macroscopic disease (R1), associated with hyperthermic intraperitoneal chemotherapy (HIPEC) to treat residual microscopic disease, and to evaluate its indications. Overall 5-year survival of patients with peritoneal carcinomatosis treated by HIPEC is similar to that of patients with hepatic metastases treated with curative intent. Those patients should no longer be considered as patients with a terminal disease but as patients with a potentially treatable localized disease.

Pituitary apoplexy after aortic abdominal aneurysm surgery: a case report.

Pituitary apoplexy (PA) occurring after surgery is a rare but life-threatening acute clinical situation following extensive haemorrhage or necrosis within a pituitary adenoma. Pituitary apoplexy has been reported to occur spontaneously in the majority of cases or in association with various inducing factors. One of the pathophysiological mechanism that has been postulated is the fall of arterial blood pressure inducing ischaemia followed by infarction of the pituitary gland. We report a case of pituitary apoplexy following aortic abdominal surgery. To our knowledge, this has not been previously reported. A 73-year old man complained of headache and diplopia. At clinical examination, he presented a right oculomotor nerve palsy. Magnetic resonance imaging (MRI) showed a haemorrhagic sellar mass. In our case, intraoperative hypotension could have been the precipitating factor. Diagnostic and therapeutic measures are discussed.

[Verrucous carcinoma of the esophagus. A case report]. / Su di un caso di carcinoma verrucoso dell'esofago.

Verrucous carcinoma (VC) of the esophagus is a rare variant of squamous cell carcinoma and only 20 cases have so far been reported in the international literature. The neoplasm is usually highly differentiated, presents a slow growth pattern with invasion of surrounding organs rather than blood-borne metastases. Recently, a causative role of human papillomavirus (HPV) has been hypothesized. The case of a patient affected with locally advanced VC of the esophagus and treated by means of local antiviral therapy is reported. A 41-year-old male patient was admitted to our institution for persistent atypical thoracic pain. The imaging techniques (thoracic and abdominal CT scans; upper GI endoscopy; hydrosoluble contrast swallow; endoscopic US) revealed a cauliflower-like protruding esophageal mass, active mucosal mycosis, multiple ulcerations of the distal esophagus, as well as 2 broncho-esophageal fistulas. The neoplasm extended beyond the esophageal wall, infiltrating surrounding cervical and mediastinal organs and the patient presented with secondary esophageal achalasia and right bilobar pneumonia. The histologic specimen was consistent with VC of the esophagus and the presence of HPV infection was detected by means of qualitative PCR assay. The patient was deemed not fit for surgery and a local antiviral treatment with hydroxy-phosphonyl-methoxypropyl-cytosine 5 mg/kg a week was started. After initial response to treatment, the patient presented with sudden progression leading to further broncho-esophageal fistula treated with endoscopic stent placement and ultimate death 6 months after referral to our center. In keeping with international data, our case confirms that esophageal VC has a highly unfavorable outcome, despite its high degree of differentiation and slow growth pattern. The long natural history, the lack of specific symptoms and the presence of coexisting esophageal diseases delay the diagnosis and account for the local advancement of this malignancy. Surgery is the option of choice for early stage lesions and advanced VC does not seem to benefit from current chemotherapeutic regimens. The causative role of HPV and the advancements of molecular pharmacology might allow for effective treatment in high-risk patients.

Fluorescence imaging after intraoperative intravenous injection of indocyanine green for detection of lymph node metastases in colorectal cancer.

PURPOSE: This short communication aims at reporting the potential role of ICG fluorescence imaging after an intraoperative IV injection in the detection of lymph nodes (LNs) of a colorectal cancer origin. PATIENTS AND METHODS: Two patients who were included in a protocol study evaluating the role of ICG in the detection of peritoneal metastases of colorectal origin (Protocol NCT-01995591) also had fluorescent LNs at exploration with a dedicated near-infrared camera system (Photodynamic Eye, PDE; Hamamatsu Photonics, Hamamatsu, Japan). An IV injection of ICG was delivered intraoperatively at 0.25 mg/kg. All LNs were also explored for their fluorescence, and tumor to background ratio (TBR) was calculated with IC-Calc 2.0 program. RESULTS: One patient had two retroperitoneal lymph node metastases and one mesocolic on a pre-operative work-up. The three tumoural lymph nodes at histopathology were hyperfluorescent in comparison to other uninvolved LNs. One patient had no pre-operatively known LN metastases and had one epigastric hyperfluorescent LN discovered at intraoperative exploration. This LN of 6 mm in size was malignant at histopathology. CONCLUSION: This is the first report about tumoural LN of colorectal cancer origin detected by fluorescence imaging with intraoperative IV free-ICG injection. ICG fluorescence imaging by intraoperative IV injection represents an easy method for detecting metastatic LNs in colorectal cancer. This proof of concept should lead to further research in this field.

Intraperitoneal follicular dendritic cell sarcoma: role of chemotherapy and bone marrow allotransplantation in locally advanced disease?

We describe a case of a 44 year-old woman diagnosed with follicular dendritic cell sarcoma (FDCS). FDCS is a very rare disease affecting the dendritic antigen presenting cells and is often misdiagnosed. Surgery is considered the best treatment modality, followed by chemotherapy. In our case, surgical excision was not possible, therefore the patient received two lines of chemotherapy followed by bone marrow allotransplantation, then a third line of chemotherapy with a complete metabolic response seen on PET/computed tomography (CT) follow-up 29 months later. A review of the literature has been performed.

The role of PET scan in the preoperative management of oesophageal cancer.

AIM: The aim of this study was to evaluate the role of the 18F-FDG-PET in the preoperative evaluation of patients with oesophageal or gastro-oesophageal junction (GEJ) cancer and to define its impact on therapeutic management. PATIENTS AND METHODS: This study included 58 patients with biopsy proven oesophageal or GEJ cancer who underwent PET in addition to the conventional diagnostic work-up. The sensitivity, specificity and accuracy of CT and PET were calculated for detection of tumour and distant metastases. RESULTS: The sensitivity, specificity and accuracy of tumour detection were, respectively, 84, 100 and 84% for CT and 87, 100 and 87% for PET (p=ns). PET permitted detection of distant metastases, which were not seen on CT in seven patients. In two patients, a second primary tumour was detected on PET. CONCLUSIONS: The sensitivity of PET for loco regional lymph nodes detection is low. The major clinical impact is the detection of distant metastases.

Biliary pleural fistula as a complication of radiofrequency ablation for liver metastasis.

Radiofrequency ablation (RFA) recently emerged as an efficient and safe local ablative method to treat unresecable liver tumours. Currently however, the full spectrum of potential complications of RFA remains undetermined. We report a case of severe biliary complication, associating main bile duct stricture and biliary pleural fistula, arising after extensive RFA for unresectable liver metastasis of central location. Treatment consisted of external drainage of the pleural effusion and internal endoscopic drainage via a biliary stenting. This description of a life-threatening complication emphazises the need to better knowledge of the contraindications of RFA, particularly for the treatment of large tumours at proximity of main bile ducts.

[Surgical treatment of peritoneal carcinomatosis]. / Traitement chirurgical des carcinoses péritonéales: 2--la chimiohyperthermie.

Surgical treatment of peritoneal carcinomatosis with curative intent involves the resection of all macroscopic malignant tissue and the treatment of microscopic residual tissue with local chemotherapy. Intraperitoneal chemotherapy is efficient only when associated with hyperthermia. This article details the technical aspect of intraperitoneal chemohyperthermia. These techniques involve specific and important features. At these conditions, peritoneal carcinomatosis can be cured in two-thirds of cases.