Gilton Marques Fonseca

Downstaging and resection after neoadjuvant therapy for fibrolamellar hepatocellular carcinoma.

Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare malignant liver neoplasm, commonly observed in adolescents and young adults of both genders. The disease is more common in Caucasians and in patients without a prior history of liver disease. The best treatment option is a surgical resection associated with liver hilum lymph node dissection. However, there is no established systemic drug treatment for patients with locally advanced or metastatic disease. We report on a patient with advanced FLHCC, initially considered unresectable due to invasion of the right and the middle hepatic veins and circumferential involvement of the left hepatic vein. Following the treatment with gemcitabine-oxaliplatin systemic chemotherapy, the patient exhibited a significant tumor reduction. As a result, a complete resection was performed with an extended right hepatectomy associated with a partial resection of the inferior vena cava, a wedge resection in segment 2, and lymphadenectomy of the hepatic hilum. The case was unusual due to the significant tumor downstaging with gemcitabine-oxaliplatin, potentially enabling curative resection. More studies are needed to confirm the efficacy of the systemic drug treatment for FLHCC.

Prognostic implication of mucinous histology in resected colorectal cancer liver metastases.

BACKGROUND Colorectal mucinous adenocarcinoma (MAC) is a subtype of colorectal adenocarcinoma with prominent mucin production associated with proximal location of tumor, advanced stage at diagnosis, microsatellite instability, and BRAF mutation. The prognostic implication of MAC in colorectal cancer liver metastases (CRCLM) is unknown. The purpose of our study was to determine the frequency and elucidate the prognostic implication of mucinous histology in CRCLM. METHODS The medical records of 118 patients who underwent CRCLM resection between 2000 and 2010 were reviewed. Clinicopathologic variables and outcome parameters were examined. Resected specimens were submitted to routine histologic evaluation. Patients were grouped according to the metastasis mucinous content: >50%, MAC; <50%, adenocarcinoma with intermediated mucinous component (AIM); and without any mucinous component, non-MAC (NMA). RESULTS Mean follow-up after resection was 37 months. Tumor recurrence was observed in 75% of patients. Overall survival and disease-free survival rates after hepatectomy were 61%, 56%, and 26%, 24% at 3 and 5 years, respectively. Tumors with mucinous component (AIM and MAC) were related to proximal location of the primary tumor and were more frequently observed in females. Multivariate analysis revealed that MAC was an independent negative prognostic factor (hazard ratio, 3.13; 95% CI, 1.30-6.68; P = .011) compared with non-MAC (NMA and AIM). CONCLUSION MAC has an adverse prognostic impact compared with NMA, which may influence therapeutic strategy raising an important subject for discussion and future investigation.

Pathological factors and prognosis of resected liver metastases of colorectal carcinoma: implications and proposal for a pathological reporting protocol

Colorectal cancer is a leading cause of death worldwide. The liver is the most common site of distant metastases, and surgery is the only potentially curative treatment, although the recurrence rate following surgery is high. In order to define prognosis after surgery, many histopathological features have been identified in the primary tumour. In turn, pathologists routinely report specific findings to guide oncologists on the decision to recommend adjuvant therapy. In general, the pathological report of resected colorectal liver metastases is limited to confirmation of the malignancy and details regarding the margin status. Most pathological reports of a liver resection for colorectal liver metastasis lack information on other important features that have been reported to be independent prognostic factors. We herein review the evidence to support a more detailed pathological report of the resected liver specimen, with attention to: the number and size of liver metastases; margin size; the presence of lymphatic, vascular, perineural and biliary invasion; mucinous pattern; tumour growth pattern; the presence of a tumour pseudocapsule; and the pathological response to neoadjuvant chemotherapy. In addition, we propose a new protocol for the evaluation of colorectal liver metastasis resection specimens.

Primary liposarcoma of the pancreas: A review illustrated by findings from a recent case.

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in limbs and retroperitoneum, with only rare instances of visceral location reported. Pancreas is a very rare site of primary liposarcoma, with a total of seven cases reported since 1979 and only four of those in the English literature. We review the literature specific for primary liposarcoma of the pancreas and discuss radiological and pathological aspects of this rare tumor type as well as emerging options of treatment. The review is illustrated by findings of a recent case of a dedifferentiated liposarcoma of the pancreas coupled with undifferentiated pleomorphic sarcoma, including the first description of this rare tumor by magnetic resonance imaging. The patient was successfully treated with distal pancreatectomy and splenectomy, followed by adjuvant chemotherapy and radiotherapy. At the 5-year follow-up, the patient showed no signs of recurrence.

Late-Onset Ornithine Carbamoyltransferase Deficiency Accompanying Acute Pancreatitis and Hyperammonemia

Hyperammonemia related to urea cycle disorders is a rare cause of potentially fatal encephalopathy that is encountered in intensive care units (ICUs). Left undiagnosed, this condition may manifest irreversible neuronal damage. However, timely diagnosis and treatment initiation can be facilitated simply by increased awareness of the ICU staff. Here, we describe a patient with acute severe pancreatitis who developed hyperammonemia and encephalopathy without liver disease. Urea cycle disorder was suspected and hemodialysis was initiated. Following reduction of ammonia levels, subsequent treatment included protein restriction and administration of arginine and sodium benzoate. The patient was discharged to home after 47 days with plasma ammonia within normal range and without neurological symptoms. In clinical care settings, patients with neurological symptoms unexplained by the present illness should be assessed for serum ammonia levels to disclose any urea cycle disorders to initiate timely treatment and improve outcome.

Biliary tract schwannoma: A rare cause of obstructive jaundice in a young patient

Schwannoma is a tumor derived from Schwann cells which usually arises in the upper extremities, trunk, head and neck, retroperitoneum, mediastinum, pelvis, and peritoneum. However, it can arise in the gastrointestinal tract, including biliary tract. We present a 24-year-old male patient with obstructive jaundice, whose investigation with computed tomography abdomen showed focal wall thickening in the common hepatic duct, difficult to differentiate with hilar adenocarcinoma. He was diagnosed intraoperatively schwannoma of common bile duct and treated with local resection. The patient recovered well without signs of recurrence of the lesion after 12 mo. We also reviewed the common bile duct schwannoma related in the literature and evaluated the difficulty in pre and intraoperative differential diagnosis with adenocarcinoma hilar. Resection is the treatment of choice for such cases and the tumor did not recur in any of the resected cases.

INFLUENCE OF HEPATOCELLULAR CARCINOMA ETIOLOGY IN THE SURVIVAL AFTER RESECTION.

ABSTRACT Background: Hepatocellular carcinoma (HCC) is the most frequent type of primary liver cancer and its incidence is increasing around the world in the last decades, making it the third cause of death by cancer in the world. Hepatic resection is one of the most effective treatments for HCC with five-year survival rates from 50-70%, especially for patients with a single nodule and preserved liver function. Some studies have shown a worse prognosis for HCC patients whose etiology is viral. That brings us to the question about the existence of a difference between the various causes of HCC and its prognosis. Aim: To compare the prognosis (overall and disease-free survival at five years) of patients undergoing hepatectomy for the treatment of HCC with respect to various causes of liver disease. Method: Was performed a review of medical records of patients undergoing hepatectomy between 2000 and 2014 for the treatment of HCC. They were divided into groups according to the cause of liver disease, followed by overall and disease-free survival analysis for comparison. Results: There was no statistically significant difference in the outcomes of the groups of patients divided according to the etiology of HCC. Overall and disease-free survival at five years of the patients in this sample were 49.9% and 40.7%, respectively. Conclusion: From the data of this sample, was verified that there was no prognostic differences among the groups of HCC patients of the various etiologies.

CENTRAL HEPATECTOMY FOR BILIARY CYSTADENOMA: PARENCHYMA-SPARING APPROACH FOR BENIGN LESIONS

Sweden. Report of 49 patients. Dis Colon Rectum. 1990 Oct;33(10):874-7. 7. Jorge E, Harvey HA, Simmonds MA, Lipton A, Joehl RJ. Symptomatic malignant melanoma of the gastrointestinal tract. Operative treatment and survival. Ann Surg. 1984 Mar;199(3):328-31. 8. Kantarovsky A, Kaufman Z, Zager M, Lew S, Dinbar A. Anorectal region malignant melanoma. J Surg Oncol. 1988 Jun;38(2):77-9. 9. Knysh VI, Timofeev IuM, Serebriakova ES. [Treatment of melanomas of the anorectal region]. Vopr Onkol. 1987;33(3):74-8. 10. Pyper PC, Parks TG. Melanoma of the anal canal. Br J Surg. 1984 Sep;71(9):671-2. 11. Singh W, Madaan TR. Malignant melanoma of the anal canal. Am J Proctol. 1976 Feb;27(1):49-55. 12. Slingluff CL Jr, Vollmer RT, Seigler HF. Anorectal melanoma: clinical characteristics and results of surgical management in twenty-four patients. Surgery. 1990 Jan;107(1):1-9. 13. Wong JH, Cagle LA, Storm FK, Morton DL. Natural history of surgically treated mucosal melanoma. Am J Surg. 1987 Jul;154(1):54-7.

Prognostic significance of poorly differentiated clusters and tumor budding in colorectal liver metastases

Histomorphological features have been described as prognostic factors after resection of colorectal liver metastases (CLM). The objectives of this study were to assess the prognostic significance of tumor budding (TB) and poorly differentiated clusters (PDC) among CLM, and their association with other prognostic factors.

Surgical treatment of fibrolamellar hepatocellular carcinoma: an underestimated malignant tumor?

BACKGROUND Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare disease with an indolent behavior. Its prognosis is better than that of patients with hepatocellular carcinoma. The authors present their experience with resection of FLHCC. METHODS Twenty-one patients with FLHCC were treated at our institution between 1990 and 2012. Of these patients, 14 were subjected to resection of the tumor. Patient demographics, medical history, results of imaging studies and laboratory tests, surgical data, and pathologic findings were evaluated. RESULTS The median age of the patients at the diagnosis of the tumor was 20 years and 14 patients were female. None of the patients had tumor-associated chronic liver disease or cirrhosis. The mean tumor size was 12.8 cm (range 6-19) and 18 patients had a single liver nodule. Fourteen patients were subjected to hepatectomy and six of them had lymph node metastases resected. Pathologic evaluation revealed that 5 (35.7%) patients had major vascular invasion. Tumor recurrence was seen in 8 patients (66.7%), during a follow-up. The median survival time for patients who were subjected to resection was 36 months. The 5-year overall survival rate and disease free survival rate were 28.0% and 8.5%, respectively. Univariate analysis showed that vascular invasion was the only variable associated with the disease free survival rate. CONCLUSIONS Despite an aggressive treatment, patients with FLHCC presented unexpected low survival rates. It seems that an underestimated malignant behavior is attributed to this disease, and that the forms of adjuvant treatment should be urgently evaluated.