Beyond the three P's: adrenal involvement in MEN1.

Adrenal lesions (ALs) are often detected in patients with multiple endocrine neoplasia type 1 (MEN1). However, they are not well described in MEN1, making their clinical management unclear. This study examined the prevalence and outcomes of ALs found in MEN1. We performed a retrospective chart review of patients diagnosed with MEN1 from 1990 to 2021. ALs were diagnosed using abdominal or thoracic imaging and classified as being unilateral or bilateral, having single or multiple nodules, and as having diffuse enlargement or not. Measurable nodular lesions were analyzed for their size and growth over time. Patients' clinical and radiographic characteristics were collected. We identified 382 patients with MEN1, 89 (23.3%) of whom had ALs. The mean age at detection was 47 ± 11.9 years. We documented 101 measurable nodular lesions (mean size, 17.5 mm; range, 3-123 mm). Twenty-seven nodules (26.7%) were smaller than 1 cm. Watchful waiting was indicated in 79 (78.2%) patients, of whom 28 (35.4%) had growing lesions. Functional lesions were diagnosed in 6 (15.8%) of 38 that had functional work-up (diagnoses: pheochromocytoma (n = 2), adrenocorticotropic hormone-dependent hypercortisolism (n = 2), hyperandrogenism (n = 1), hyperaldosteronism (n = 1)); surgery was indicated for 5 (83.3%; n = 12 nodules), 2 of whom had bilateral, diffuse adrenal enlargement. Two patients were diagnosed with adrenocortical carcinoma and two with neoplasms of uncertain malignant potential. Radiographic or clinical progression of ALs is uncommon. Malignancy should be suspected on the basis of a lesion's growth rate and size. A baseline hormonal work-up is recommended, and no further biochemical work-up is suggested when the initial assessment shows nonfunctioning lesions.

Is there a role for liquid biopsy in the surveillance of parathyroid carcinoma?

BACKGROUND: Parathyroid carcinoma is a rare malignancy with high recurrence rates. Liquid biopsy is a stratifying tool in disease recurrence/progression in other malignant processes. This study sought to assess the feasibility and application of liquid biopsy in parathyroid carcinoma and its impact on surveillance. METHODS: Retrospective review of a prospectively maintained database of adults treated for parathyroid carcinoma at a tertiary care center (2017-2023). Demographics, clinical characteristics, and laboratory variables were collected. Circulating cell-free deoxyribonucleic acid enrichment and circulating tumor cell enumeration were obtained from serial blood samples. RESULTS: A total of 25 patients were identified-64% were male patients, with a median age of 56 years (interquartile range 45-63). Fifty blood samples were collected postoperatively. At first, circulating tumor cell enumeration, 56% (14/25) of patients had no evidence of disease, and 32% (8/25) had distant metastasis. Median follow-up was 53 months (interquartile range 23-107). At the last follow-up, 40% (10/25) of patients were found to have distant metastasis. Serial circulating tumor cell enumeration was performed in 52% of patients, median highest circulating tumor cell was (interquartile range 1-22). Circulating cell-free deoxyribonucleic acid was assessed in 64% of patients (16/25). There was no difference in circulating tumor cells or circulating cell-free deoxyribonucleic acid between those with distant metastasis and those without distant metastasis. The most common mutation identified was TP53, present in 88% of circulating cell-free deoxyribonucleic acid samples with a mutation. Circulating cell-free deoxyribonucleic acid and parathyroid hormone levels were not found to have any association (r = -0.27, P = .39), but parathyroid hormone and circulating tumor cell had a linear relationship (r = 0.76, P < .001). CONCLUSION: Liquid biopsy appears to be a feasible tool in parathyroid carcinoma surveillance. The relationship between circulating cell-free deoxyribonucleic acid and parathyroid hormone levels remains unclear, and the association between circulating tumor cell enumeration and parathyroid hormone levels may be impactful. The finding that TP53 mutation is more prevalent in patients with distant metastasis may impact further management.

Succinate Dehydrogenase Mutations as Familial Pheochromocytoma Syndromes.

It is recognized that a large portion of pheochromocytoma and paraganglioma cases will have an underlying germline mutation, supporting the recommendation for universal genetic testing in all patients with PPGLs. A mutation in succinate dehydrogenase subunit B is associated with increased rates of developing synchronous and/or metachronous metastatic disease. Patients identified with this mutation require meticulous preoperative evaluation, a personalized surgical plan to minimize the risk of recurrence and tumor spread, and lifelong surveillance.

Pheochromocytoma recurrence in hereditary disease: does a cortical-sparing technique increase recurrence rate?

BACKGROUND: Posterior retroperitoneoscopic adrenalectomy is an appealing approach for patients with hereditary pheochromocytoma and lends well to cortex preservation. We sought to examine pheochromocytoma recurrence in patients with hereditary pheochromocytoma in the era of posterior retroperitoneoscopic adrenalectomy and evaluate the predictors of recurrence. METHODS: Patients with hereditary pheochromocytoma who underwent adrenalectomy for pheochromocytoma between 1995 and 2020 with biochemical cure and follow-up >1 year were identified. Recurrence was defined as plasma metanephrines above the upper limit of normal with radiographic evidence of disease in the ipsilateral adrenal bed. RESULTS: Seventy-eight hereditary pheochromocytoma patients (median age = 32.4 years; 60.3% women) underwent 114 adrenalectomies for pheochromocytoma. Of these patients, 40 had multiple endocrine neoplasia type 2A (51.3%), 10 had multiple endocrine neoplasia type B (12.8%), 17 had von Hippel-Lindau disease (21.8%), and 11 had neurofibromatosis type 1 (14.1%). Thirty-eight adrenalectomies (33.3%) were performed before the introduction of posterior retroperitoneoscopic adrenalectomy and 76 (66.7%) after. Cortical-sparing technique was performed in 62 (54.4%) adrenalectomies, with no difference in its use before and after posterior retroperitoneoscopic adrenalectomy introduction (P > .05). During a median follow-up of 80.7 months (interquartile range 43.4-151.2), 12 ipsilateral recurrences (10.5%) were identified. There was no difference in recurrence before and after the introduction of posterior retroperitoneoscopic adrenalectomy or by surgical technique or approach of the entire cohort (P > .05). Recurrence was more common in those with RET M918T mutation (23.5% vs 8.2%; P = .05). Patients with RET M918T mutations had a shorter recurrence-free survival (P = .013). On multivariate analysis, only RET M918T mutation was independently associated with an increased recurrence risk (hazard ratio = 4.30; 95% confidence interval, 1.26-14.66; P = .019). CONCLUSION: The introduction of posterior retroperitoneoscopic adrenalectomy did not influence the recurrence rate after adrenalectomy for hereditary pheochromocytoma patients. Patients with a RET M918T germline mutation are at increased risk for pheochromocytoma recurrence and may benefit from initial total adrenalectomy.

Parathyroidectomy for Normocalcemic Primary Hyperparathyroidism is Associated with Improved Bone Mineral Density Regardless of Postoperative Parathyroid Hormone Levels.

BACKGROUND: Biochemical cure in normocalcemic primary hyperparathyroidism (nPHPT) is defined as parathyroid hormone (PTH) level normalization 6 months after parathyroidectomy. However, recent studies show that a significant number of nPHPT patients have persistent PTH elevation postoperatively. We sought to correlate changes in PTH levels with skeletal outcomes after parathyroidectomy in nPHPT patients. METHODS: Adult patients who underwent parathyroidectomy at a tertiary referral center for sporadic PHPT between 2010 and 2020 were reviewed. Pre- and postoperative (6 months, 18 months, and last follow-up) laboratory and bone mineral densities (BMD) were recorded. Primary outcome was 18-month postoperative BMD change in the lumbar spine (LS), total hip (TH) and femoral neck (FN) in normocalcemic and hypercalcemic PHPT (hPHPT) patients. RESULTS: Of 661 patients included, 68 had nPHPT. nPHPT patients frequently had multigland disease (31% vs. 18%, p = 0.014), more bilateral cervical explorations (22% vs. 13%, p = 0.042), and fewer achieved biochemical cure (76% vs. 95%, p < 0.001) than hPHPT patients. Twenty-eight nPHPT patients had BMD data for comparison. Overall, nPHPT patients had improvement in the LS (1.84%, p = 0.002) and TH (1.64%, p = 0.014). When stratified by postoperative PTH levels, nPHPT patients with persistent PTH elevation had more BMD improvement at the TH than those who normalized PTH (3.73% vs. - 0.83%, p = 0.017). There was no difference in improvement at the LS or FN (p = NS). CONCLUSION: Parathyroidectomy is associated with improved BMD in nPHPT patients with bone disease. Although one in four nPHPT patients had elevated postoperative PTH levels persisting throughout the study, BMD improvement was still seen regardless of postoperative PTH level normalization.

Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.

BACKGROUND: Neuroendocrine tumors can cause ectopic Cushing syndrome, and most patients have metastatic disease at diagnosis. We identified risk factors for outcome, evaluated ectopic Cushing syndrome management, and explored the role of bilateral adrenalectomy in this population. METHODS: This was a retrospective study including patients with diagnosis of ectopic Cushing Syndrome secondary to neuroendocrine tumors with adrenocorticotropic hormone secretion treated at our quaternary referral center over a 40-year period (1980-2020). RESULTS: Seventy-six patients were included. Mean age at diagnosis was 46.3 ± 15.8 years. Most patients (N = 61, 80%) had metastases at ectopic Cushing syndrome diagnosis. Average follow-up was 2.9 ± 3.7 years (range, 4 months-17.2 years). Patients with neuroendocrine tumors before ectopic Cushing syndrome had more frequent metastatic disease and resistant ectopic Cushing syndrome. Patients with de novo hyperglycemia, poor neuroendocrine tumor differentiation, and metastatic disease had worse survival. Of those with nonmetastatic disease, 8 (53%) had ectopic Cushing syndrome resolution after neuroendocrine tumor resection, 3 (20%) were medically controlled, and 4 (27%) underwent bilateral adrenalectomy. In patients with metastatic neuroendocrine tumors, hypercortisolism was initially medically managed in 92%, 3% underwent immediate bilateral adrenalectomy, 2% had control after primary neuroendocrine tumor debulking, and 2% were lost to follow-up. Medical treatment resulted in hormonal control in 7 (13%) patients. Of the 49 patients with metastatic disease and medically resistant ectopic Cushing syndrome, 23 ultimately had bilateral adrenalectomy with ectopic Cushing syndrome cure in all. CONCLUSION: Patients with neuroendocrine tumors before ectopic Cushing syndrome development were more likely metastatic and had worse survival. De novo hyperglycemia and poor neuroendocrine tumor differentiation were predictive of worse prognosis. Medical control of hypercortisolism is difficult to achieve in patients with neuroendocrine tumors-ectopic Cushing syndrome. Well-selected patients may benefit from bilateral adrenalectomy early in the treatment algorithm, and multidisciplinary management is essential in this complex disease.

Risk Factors Associated to Residual Tumor in Re-excision for Positive Margins in Breast Conservative Surgery.

BACKGROUND: Breast conservative surgery (BCS) is an adequate treatment for patients with early breast cancer. Local recurrence is associated with diverse factors. Our objective was to evaluate risk factors associated with finding residual tumor in patients with positive margins in BCS. METHODS: Observational retrospective study, including patients diagnosed with breast cancer undergoing BCS between 2000 and 2016. Clinicopathological and treatment variables were collected. Main outcome was the finding of residual tumor on re-excision. Positive margins were defined as tumor present on ink. RESULTS: Three hundred and six patients underwent BCS. Mean age was 57 ± 12.2 years. Positive margins were found in 84 (27.4%) patients, 15 (4.9%) had unknown margin status, and 207 (67.6%) had negative margins. Seventy-eight patients from the positive margin group and 23 patients from the unknown/negative margin group were reintervened. Residual tumor was present in 41% of patients with positive margins and in 45% of patients with negative margins (P = .192). In univariate analysis, overweight (P = .04) and positive axillary lymph nodes (P = .02) were associated with residual tumor on re-excision. In multivariate analysis, postmenopausal status was a protective factor (HR .047, P = .30). Mean follow-up was 58.4 months and mean local recurrence-free survival (LRFS) was 56.4 months (.1-203.2), with no difference regarding margin status or residual tumor. DISCUSSION: Postmenopausal status was associated with a decreased rate of residual tumor in patients with positive margins. The presence of residual tumor on re-excision was not associated with a lower LRFS. These factors must be considered when positive margins are present in BCS.

Evaluation of risk factors, long-term outcomes, and immediate and delayed autotransplantation to minimize postsurgical hypoparathyroidism in multiple endocrine neoplasia type 1 (MEN1): A retrospective cohort study.

BACKGROUND: Postoperative hypoparathyroidism from inadequate parathyroid hormone is of concern after multigland resections in multiple endocrine neoplasia type 1-related primary hyperparathyroidism. We evaluated risk factors, long-term outcomes, and roles of autotransplantation and cryopreservation in postoperative hypoparathyroidism in multiple endocrine neoplasia type 1. METHODS: Retrospective cohort study of patients with multiple endocrine neoplasia type 1 and parathyroidectomy who were evaluated at MD Anderson from 1990 to 2020. RESULTS: We included 206 patients. Median follow-up after the last operation (index 65%, reoperation 35%) was 8 years. Index parathyroidectomy was subtotal in 47%, less than subtotal in 42%, and total in 12%; hypoparathyroidism was more frequent after total parathyroidectomy. Forty-seven patients (23%) had hypoparathyroidism ≥6 months; odds were significantly higher when cumulative ≥4 glands were resected (odds ratio 6 [2.96-12.24]) or when immediate postoperative parathyroid hormone was <15 pg/mL (odds ratio 13.10 [3.61-47.47]). After median 26 months postoperatively, 30% recovered parathyroid function spontaneously; this was less likely when ≥4 glands were resected (odds ratio 0.19 [0.05-0.72]). None of the 4 patients who were aparathyroid (parathyroid hormone undetectable or ≤3 pg/mL) at 6 months postoperatively recovered parathyroid function. Immediate autotransplantation success rate was 72%. Cryopreservation was performed in 96 operations with delayed autotransplantation in 10 patients (10% utilization), of whom 5 recovered parathyroid function (time to recovery 12-93 months). CONCLUSION: Odds of prolonged hypoparathyroidism are higher when cumulative ≥4 glands are resected or postoperative parathyroid hormone is <15 pg/mL. Spontaneous recovery occurred but was less likely when ≥4 glands were resected or patients were aparathyroid at 6 months postoperatively. Cryopreservation should be sparingly used, but there is value in select high-risk patients such as reoperative parathyroidectomy/cervical surgery.

Parathyroid Surgery: What Radiologists Need to Know.

Surgical intervention remains the mainstay of treatment of hyperparathyroidism and provides the highest chance at cure. After the disease is confirmed by biochemical testing, surgeons must use a combination of patient clinical history and radiographic imaging to determine the most appropriate surgical strategy. Through either minimally invasive parathyroidectomy or bilateral cervical exploration, surgeons provide high rates of cure for hyperparathyroidism with low rates of persistence or recurrence.

Endocrine hypertension secondary to adrenal tumors: clinical course and predictive factors of clinical remission.

PURPOSE: Endocrinopathies constitute ~ 10% of secondary hypertension (SH) etiologies. Primary aldosteronism, pheochromocytoma (PHEO), and Cushing's syndrome are common causes. Early identification and treatment result in resolution/improvement of SH. The aim of this study was to characterize the clinical course, outcomes, and remission-associated prognostic factors of SH related to adrenal tumors. METHODS: Retrospective cohort study including patients with SH who underwent adrenalectomy from 2000 to 2019. Postoperative outcomes were analyzed. Remission was defined as normalization of blood pressure without drug use. RESULTS: Eighty-three patients with SH were included. Mean ± SD age was 38.8 ± 14.2 years and 75.9% were women. Diagnosis was PHEO in 35 patients (42.2%), aldosteronoma (APA) in 28 (33.7%), cortisol producing adenoma (CPA) in 16 (19.3%), and ACTH-dependent Cushing's in 4 (4.8%). Laparoscopic adrenalectomy was performed in 81 (97.6%) patients. Mean ± SD follow-up was 57.4 ± 49.6 months (range 1-232). Surgical morbidity occurred in 7.2% of patients and there was no mortality. Remission of SH occurred in 61(73.5%): 100% of ACTH-dependent Cushing's, 85.7% of PHEO, 68.8% of CPA, and 57.1% of APA. Biochemical phenotype and the combination of larger tumor size, number of antihypertensive drugs, male gender, older age, obesity, and preoperative SH for more than 5 years were associated with less likely clinical remission in patients with APA (p = 0.004), CPA (p < 0.0001), and PHEO (p < 0.0001). CONCLUSION: SH remission rates are 57-100% after adrenalectomy. Several prognostic factors could be used to predict SH control. Adrenalectomy provides good clinical outcome and must be considered a treatment option in all surgical candidates.

Does laparoscopic reoperation yield symptomatic improvements similar to those of primary laparoscopic Heller myotomy in achalasia patients?

BACKGROUND: Laparoscopic Heller myotomy fails in approximately 3.5% to 15% of patients. Evidence of successful laparoscopic reoperation is limited to a few studies. METHODS: This case-control study was conducted in patients who underwent laparoscopic Heller myotomy reoperation (LHM-R) from 2008 to 2016. The operative outcomes, preoperative and last follow-up manometric parameters, and symptom questionnaire results, including the Eckardt, Gastroesophageal Reflux Disease-Health Related Quality of Life (GERD-HRQL) and eating assessment tool (EAT-10) scores, were obtained. The data were compared with those of patients who underwent primary laparoscopic Heller myotomy (LHM-1). RESULTS: Thirty-five patients who underwent LHM-R and 35 patients who underwent LHM-1 were included. The reasons for failure in the LHM-R patient group included incomplete myotomy (71.4%), myotomy fibrosis (25.7%) and structural alterations in fundoplication (2.9%). The follow-up duration was 34 months for the LHM-R group and 24 months for the LHM-1 group (p = 0.557). The procedure was performed by laparoscopy in 100% of the patients in the two groups. No differences were found regarding surgical morbidity (11.4% LHM-R vs. 2.9% LHM-1, p = 0.164). The symptomatic outcomes were equivalent between groups (Eckardt p = 0.063, EAT-10 p = 0.166, GERD-HRQL p = 0.075). An IRP < 15 mmHg was achieved in 100% of the LHM-R and LHM-1 patients. At the last follow-up, 82.1% of the LHM-R patients and 91.4% of the LHM-1 patients were in symptomatic remission (p = 0.271). CONCLUSION: The results achieved with LHM-R are similar to those achieved with LHM-1. Laparoscopic reoperation should be considered an effective and safe treatment after a failed Heller myotomy.

Clinical characteristics and surgical treatment of schwannomas of the esophagus and stomach: A case series and systematic review.

BACKGROUND: Gastrointestinal schwannomas are slow-growing benign mesenchymal neoplasms that originate from Schwann cells of the nerve sheath of Auerbach´s plexus or less frequently from Meissner´s plexus. The main differential diagnosis of gastric schwannomas are the gastrointestinal stromal tumors (GISTs), which are classified by their immunohistochemistry. The treatment of choice for gastric schwannomas is surgery where laparoscopy plays an important role. Wedge resection, subtotal or total gastrectomy can be done. In its counterpart, esophageal schwannomas are benign tumors of the esophagus that are very uncommon since they comprise less than 2% of all esophageal tumors. The main differential diagnosis is the leiomyoma which corresponds to the most common benign esophageal tumor, followed by GIST. The treatment consists on tumoral enucleation or esophagectomy. AIM: To review the available literature about gastrointestinal schwannomas; especially lesions from de stomach and esophagus, including diagnosis, treatment, and follow up, as well as, reporting our institutional experience. METHODS: A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes guidelines. The following databases were used for reviewing process: PubMed, Ovid, MEDLINE, and Scopus. Only English language manuscripts were included. All gastrointestinal schwannomas specifically located in the esophagus and stomach were included. Cases that did not report long-term follow-up were excluded. RESULTS: Gastric localization showed a higher prevalence in both, the literature review and our institution: 94.95% (n = 317) and 83% (n = 5) respectively. With a follow-up with disease-free survival greater than 36 mo in most cases: 62.01% (n = 80) vs 66.66% (n = 4). In both groups, the median size was > 4.1 cm. Surgical treatment is curative in most cases. CONCLUSION: Schwannoma must be taken into account in the differential diagnosis of gastrointestinal mesenchymal tumors. It has a good prognosis, and most are benign. A disease-free survival of more than 36 mo can be achieved by surgery.

Acute cholecystitis in neutropenic patients.

BACKGROUNDS/AIMS: The frequency of acute cholecystitis reported in neutropenic patients is between 0.4-1.65%. Clinical manifestations differ from general population as well as clinical approach, diagnosis and treatment. The aim of this work is to describe clinical characteristics, diagnostic approach, and outcomes of patients with hematological diseases that presented with neutropenia and fever associated with acute cholecystitis in a tertiary referral hospital. METHODS: We performed a retrospective analysis of patients with diagnosis of neutropenia and fever associated with acute cholecystitis in the period between January 2000 and January 2017. Quantitative variables were analyzed with mean and standard deviation, and qualitative variables with frequency and percentage. RESULTS: During the study period, 2007 patients presented with neutropenia and fever. Twelve of them (0.59%) had associated acute cholecystitis. The most common hematologic disease among these patients was lymphoblastic leukemia. Acute acalculous cholecystitis was diagnosed in 6 cases (50%). Eleven patients (91.6%) had a severe presentation and cholecystostomy was performed in 9 (75%) cases. The main cause of mortality was septic shock (33.3%). CONCLUSIONS: Treatment of acute cholecystitis in patients with neutropenia must be individualized. Cholecystostomy should be considered as a bridge therapy for an interval cholecystectomy.

Undifferentiated embryonal sarcoma of the liver in adult patient: A report of two cases.

Primary sarcomas of the liver are unusual neoplasms developing in adults. They constitute a heterogeneous group of neoplasms including undifferentiated embryonal sarcoma. Patients usually present with an abdominal mass and abdominal pain. Case 1: A 53-year-old woman presented with abdominal pain. Computed tomography showed an occupying mass in the right lobule and an intra-auricular multi-lobulated mass suggestive of a secondary deposit. Biopsy of the hepatic lesion revealed undifferentiated embryonal sarcoma. Despite radiotherapy and supportive measures, her overall status progressively worsened until cardiac arrest. Case 2: A 41-year-old woman presented with hepatomegaly. Abdominal imaging showed cystic lesions in the right hepatic lobule with multiple septae. The patient underwent extended right hepatectomy and a histopathological study reported high-grade undifferentiated embryonal sarcoma. Two years after surgery, a new cystic lesion in the surgical site was recorded and chemotherapy was scheduled. The lesion remained stable for three years when disease progression was observed and second-line chemotherapy was initiated. Although undifferentiated embryonal sarcoma of the liver has poor prognosis, early diagnosis is essential to increase the chances of survival. Currently, surgical resection and chemotherapy are the primary treatment modalities.

Mujer joven con hepatocarcinoma fibrolamelar metastásico; citorreducción y HIPEC: a propósito de un caso / Young woman with metastatic fibrolamellar hepatocarcinoma; cytoreductive surgery and HIPEC: a case report

Objetivo: Reportar un caso clínico de hepatocarcinoma fibrolamelar metastásico y su manejo multidisciplinario. Caso clínico: Paciente de 24 años de edad con dolor abdominal, distensión abdominal y fiebre. Se le realizó tomografía computarizada de abdomen donde se encontró tumoración hepática irregular. Se realizó laparotomía con evidencia de múltiples implantes en cavidad abdominal y se diagnosticó mediante estudio histopatológico hepatocarcinoma fibrolamelar metastásico. Se decidió realizar citorreducción más quimioterapia hipertérmica intraperitoneal (HIPEC). La sobrevida de la paciente fue de 11 meses. Discusión: El hepatocarcinoma fibrolamelar es un tumor raro. Aún no hay consenso sobre el mejor tratamiento en pacientes con metástasis que tengan buena funcionalidad. El manejo actual se basa en la quimioterapia sistémica y la resección quirúrgica en casos localizados. En el caso de nuestra paciente, la cirugía citorreductora más HIPEC se realizó con la intención de mejorar la supervivencia. Se necesita más evidencia para definir esta estrategia como tratamiento estándar.

Aim: To report a clinical case of metastatic fibrolamellar hepatocarcinoma and its multidisciplinary management. Case report: 24 year-old patient with abdominal pain, bloating and fever. A computed tomography of the abdomen was performed; an irregular hepatic tumor was found. A laparotomy was performed with evidence of multiple implants in the abdominal cavity and the histopathology report was metastatic fibrolamellar hepatocarcinoma. It was decided to perform cytoreductive surgery plus HIPEC. The patient's survival was 11 months. Discussion: Fibrolamellar hepatocarcinoma is a rare tumor. There is still no consensus on the treatment of choice in patients with metastases with good functionality status. Current management is based on systemic chemotherapy and surgical resection in localized cases. In the case of our patient, cytoreductive surgery plus HIPEC was performed with the intention of improving survival. More evidence is needed to define this strategy as standard treatment.

Morbidity and mortality following cytoreductive surgery with hyperthermic intraperitoneal chemotherapy at a tertiary care center: initial experience.

BACKGROUND: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy increases progression-free and overall survival in patients with peritoneal carcinomatosis of appendicular or colorectal origin. The morbidity associated with this procedure is significant (30-52%). This modality is also routinely used in other peritoneal diseases with improvement of outcome. The aim of this study was to analyze the morbidity and mortality associated with this procedure. MATERIAL & METHODS: Thirteen patients had cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in a period from May 2011 to March 2013 and were followed up prospectively. Demographic, pathologic, and surgical variables were recorded. The Clavien-Dindo classification was used to assess surgical complications. The main outcome variable was 30-day morbidity and mortality. Descriptive statics were used. RESULTS: The mean patient age was 52.4 ± 11.1 years. The most common diagnosis was epithelial ovarian cancer (46.2%). Most patients had an adequate preoperative functional status (77% with ECOG 0). Mean hospital stay was 13.5 ± 11.2 days and 2.7 ± 4.2 days in the intensive care unit. Major morbidity (Clavien-Dindo III or IV) observed in this series was 23%, with 0% mortality. CONCLUSION: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy is a feasible option with acceptable morbidity and mortality for selected patients with peritoneal carcinomatosis in Mexico.