Appendiceal mucinous neoplasm, a rare diagnosis within gastrointestinal tumors. Case report.

Appendicular neoplasms are rare tumors, with an incidence of less than 0.05% among all gastrointestinal tumors. This work presents the case of a 52-year-old patient who manifested colicky pain in the right iliac fossa. Laboratory test results with bandemia and hyperbilirubinemia. Abdominal tomography with an acute appendicular inflammatory process, for which the patient was admitted for surgery. A dependent tumor of the cecum and appendicular region is observed, which compromises the ileocecal valve. The histopathological diagnosis was "low-grade appendiceal mucinous neoplasm." Appendiceal tumors are often incidental findings due to their low frequency; however, their possibility should not be dismissed.

Las neoplasias apendiculares son tumores raros, con una incidencia menor al 0.05% de todos los tumores gastrointestinales. Presentamos el caso de paciente de 52 años, quien acude por dolor cólico en fosa iliaca derecha. Estudios de laboratorio con bandemia e hiperbilirrubinemia. Tomografía abdominal con proceso inflamatorio apendicular agudo por lo que se ingresa a cirugía. Se observa tumoración dependiente de ciego y región apendicular que compromete válvula ileocecal. El diagnóstico histopatológico fue "neoplasia mucinosa apendicular de bajo grado. Los tumores de apéndice son a menudo hallazgos incidentales por su baja frecuencia, sin embargo, su posibilidad no debe descartarse.

Abordaje laparoscópico de la neoplasia mucinosa apendicular de bajo grado / Laparoscopic approach of low-grade appendiceal mucinous neoplasm

RESUMEN Las neoplasias mucinosas apendiculares son infrecuentes y se clasifican en neoplasias mucinosas de bajo grado, de alto grado y adenocarcinoma mucinoso. Se consideran precursoras del pseudomixoma peritoneal, caracterizado por la acumulación de mucina dentro de la cavidad abdominal. Informamos tres casos de neoplasia mucinosa apendicular de bajo grado, dos varones y una mujer, que presentaron dolor abdominal agudo, diagnosticados preoperatoriamente mediante tomografía y que fueron manejados con un abordaje laparoscópico (apendicectomía, resección parcial de ciego y colectomía derecha, respectivamente). Los tres casos tuvieron una buena evolución posoperatoria y no hubo complicaciones. Se concluye que las neoplasias mucinosas apendiculares son raras y no tienen una presentación clínica específica. El abordaje laparoscópico es una opción segura y factible. La frecuencia y la duración del seguimiento de estos pacientes es motivo de controversia.

ABSTRACT Appendiceal mucinous neoplasms are rare and are classified in low-grade mucinous neoplasm, high-grade mucinous neoplasm and mucinous adenocarcinoma. They are considered precursors of pseudomyxoma peritonei, characterized by the accumulation of mucin within the abdominal cavity. We report three cases of low-grade appendiceal mucinous neoplasm, two men and one woman, who presented with acute abdominal pain. The diagnoses were made preoperatively by computed tomography scan and the tumors were managed through laparoscopic approach (appendectomy, partial resection of the cecum and right colectomy, respectively). The three patients evolved with favorable postoperative outcome without complications. Appendiceal mucinous neoplasms are rare without specific clinical presentation. The laparoscopic approach is a safe and feasible option. The frequency and duration of surveillance in these patients are still controversial.

Mucinous cystic neoplasm of the liver

A 55-year-old woman was investigated for occasional epigastric pain and weight loss. T2-weighted abdominal magnetic resonance imaging and magnetic resonance cholangiography revealed a multilocular cyst with multiple septa and a solid component in the liver, measuring 6.1 × 4.8 × 6.5 cm. Given the patient's symptoms and malignant potential, a laparoscopic segmentectomy with partial resection of segments IV B and V was performed to completely remove the cystic lesion, associated with cholecystectomy. Histopathology demonstrated a cyst lined by columnar mucinous epithelium. Therefore, the diagnosis was mucinous cystic neoplasm of the liver. This article presents a case report and literature review of this entity.

Primary malignant neoplasms of the cecal appendix: unusual findings in acute appendicitis

Introduction: To evaluate the prevalence of appendix neoplasia correlating with patient profile, histological types and frequency. Methods: Data collection was performed in the Pathology Department of a General Hospital, with the objective of identifying patients diagnosed with malignant cecal appendix tumors by histopathologic study of specimens from acute appendicitis. Results: The prevalence of malignant primary epithelial neoplasia of the appendix was 1%. Fifty percent of the cases were neuroendocrine tumors, 35% were mucinous, and 15% were adenocarcinomas. The mean age at diagnosis was 41.3 (SD, 20.4) years (range 16-81), with a women/men ratio of 3:1. Discussion: Appendiceal neoplasms are rare and should be suspected manly in women over 40 years of age with suggestive symptoms of acute appendicitis. The size, location, extent, margins and presence of mucin are essential findings for the treatment of these patients. (AU)

A rare cecal subepithelial tumor in a Crohn´s Disease patient

Appendiceal tumors comprise a variety of histologic types, including appendiceal mucinous neoplasms, which can be grouped as premalignant lesions, tumors of uncertain malignant potential, and malignant lesions. The appendiceal mucinous neoplasms are characterized by mucinous epithelial proliferation with extracellular mucin and pushing tumor margins, commonly an incidental finding during operative exploration. We report the case of a low-grade appendiceal mucinous neoplasm presenting as a subepithelial lesion in Crohn´s Disease patient. The diagnosis was not straightforward, and only surgical resection allowed an accurate diagnosis. Although Inflammatory Bowel Disease is a risk factor for the development of colorectal neoplasms, the absolute risk for appendiceal tumors is uncertain. The frequency of progression to malignancy remains to be determined.

The rare case of a cystic pancreatic neuroendocrine tumor

The pancreatic neuroendocrine tumors (PanNETs) most commonly present as solid neoplasms; however, very rarely, they may present primarily as cystic neoplasms. Most of the cystic PanNETs are non-secreting tumors, and the radiological features are not well defined. Hence pre-operative diagnosis is usually challenging and the tumors are misdiagnosed as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, serous cystic neoplasms, solid pseudopapillary neoplasms, and non-neoplastic cysts. However, the management depends on the accurate diagnosis of these cystic lesions, which poses a dilemma. Herein, we report the case of a cystic PanNET in the tail of the pancreas, which was clinically and radiologically misdiagnosed as a mucinous cystic neoplasm. This case is reported to highlight this issue to the medical community regarding the diagnostic difficulty in such rare non-functioning pancreatic neuroendocrine tumors.

Características de la neoplasia quística mucinosa del páncreas en pacientes atendidos en un Hospital Nacional del Perú / Characteristics of pancreatic mucinous cystic neoplasm in patients treated at a National Hospital in Peru

RESUMEN Con el objetivo de analizar las características clínico, patológicas y quirúrgicas de la Neoplasia Quística Mucinosa de páncreas (NQM), se realizó un análisis de los pacientes del servicio de Cirugía de Páncreas, Bazo y Retroperitoneo del Hospital Nacional Guillermo Almenara Irigoyen en Lima, Perú desde enero del 2009 hasta octubre del 2018. La presencia del estroma ovárico se usó como criterio diagnóstico de NQM. De diez pacientes con edad promedio de 47,8 años, nueve fueron mujeres, las lesiones estuvieron localizadas en el páncreas distal, el tamaño tumoral promedio fue de 88,6 mm. En todos los pacientes se realizó una pancreatectomía distal siendo tres laparoscópicas, no hubo reoperaciones ni fallecidos, dos pacientes tuvieron carcinoma invasor asociado. En conclusión, la presentación de NQM es mayor en mujeres de edad media siendo la localización en el páncreas distal y el porcentaje de malignidad bajo. La cirugía laparoscópica es una alternativa de manejo.

ABSTRACT In order to analyze the clinical, pathological, and surgical characteristics of pancreatic mucinous cystic neoplasm (MCN), an analysis of the patients from the Pancreas, Spleen, and Retroperitoneal Surgery Service of the Guillermo Almenara Irigoyen National Hospital in Lima, Peru, was performed from January 2009 to October 2018. The presence of ovarian stroma was used as a diagnostic criterion for MCN. From ten patients with an average age of 47.8 years, nine were women; the lesions were located in the distal pancreas, and the average tumor size was 88.6 mm. All patients underwent a distal pancreatectomy, three of which were laparoscopic; there were no reoperations or deaths; two patients had associated invasive carcinoma. In conclusion, the frequency of MCN is higher in middle-aged women, being the location in the distal pancreas and the percentage of malignancy is low. Laparoscopic surgery is a disease management option.

Laparoscopic Distal Pancreatectomy With Peritoneal Staple-line Reinforcement: A Novel Technique (With Video).

INTRODUCTION: Postoperative pancreatic fistula (PPF) is still the major source of morbidity in distal pancreatectomy (DP). Despite the many surgical techniques and technology devices developed for the closure of the pancreatic stump, the PPF rate remains high, and there is no consensus with regard to the most appropriate stump closure technique. We herein present, for the first time, an innovative approach for staple-line reinforcement in DP using an autologous peritoneal patch. MATERIALS AND METHODS: The results of 2 consecutive patients who underwent laparoscopic DP due to mucinous cystic neoplasms were included in this report. The pancreas was transected with a reinforced stapler using a peritoneal patch obtained from the anterolateral wall of the abdomen, divided into 2 pieces, and sutured around each stapler jaw. The embedded video (Supplemental Digital Content 1, http://links.lww.com/SLE/A209) reports our standardized technique. RESULTS: Both patients had a favorable recovery without PPF or any complication and were discharged fully recovered 4 and 5 days after surgery without drains. Currently, both patients are alive and free of disease 1 year and 8 months after the procedure. CONCLUSIONS: The use of an autologous parietal peritoneal patch for staple-line reinforcement in DP was feasible and safe in experienced hands. Further studies are needed to confirm our findings and elucidate whether this readily available, inexpensive, rapid, and versatile alternative could reduce the PPF rate.

Neoplasia quística mucinosa en hígado. Presentación de un caso / Mucinous cystic neoplasm of the liver: A case report

Anteriormente la neoplasia quística mucinosa del hígado (NQM-H) se había clasificado como cistoadenoma biliar o cistoadenocarcinoma biliar. Sin embargo, la Organización Mundial de la Salud en la clasificación del 2010 definió la NQM-H como contrapartida de la NQM del páncreas (NQM-P). En ambos casos se requiere la presencia de estroma ovárico para establecer el diagnóstico. La NQM-H es una rara enfermedad que se produce en una frecuencia mucho menor que la pancreática y sus características biológicas han sido poco esclarecidas. Presentamos un caso de NQM-H en una paciente de 33 años que fue tratada con resección quirúrgica.(AU)

[Characteristics of pancreatic mucinous cystic neoplasm in patients treated at a National Hospital in Peru]. / Características de la neoplasia quística mucinosa del páncreas en pacientes atendidos en un Hospital Nacional del Perú.

In order to analyze the clinical, pathological, and surgical characteristics of pancreatic mucinous cystic neoplasm (MCN), an analysis of the patients from the Pancreas, Spleen, and Retroperitoneal Surgery Service of the Guillermo Almenara Irigoyen National Hospital in Lima, Peru, was performed from January 2009 to October 2018. The presence of ovarian stroma was used as a diagnostic criterion for MCN. From ten patients with an average age of 47.8 years, nine were women; the lesions were located in the distal pancreas, and the average tumor size was 88.6 mm. All patients underwent a distal pancreatectomy, three of which were laparoscopic; there were no reoperations or deaths; two patients had associated invasive carcinoma. In conclusion, the frequency of MCN is higher in middle-aged women, being the location in the distal pancreas and the percentage of malignancy is low. Laparoscopic surgery is a disease management option.

Con el objetivo de analizar las características clínico, patológicas y quirúrgicas de la Neoplasia Quística Mucinosa de páncreas (NQM), se realizó un análisis de los pacientes del servicio de Cirugía de Páncreas, Bazo y Retroperitoneo del Hospital Nacional Guillermo Almenara Irigoyen en Lima, Perú desde enero del 2009 hasta octubre del 2018. La presencia del estroma ovárico se usó como criterio diagnóstico de NQM. De diez pacientes con edad promedio de 47,8 años, nueve fueron mujeres, las lesiones estuvieron localizadas en el páncreas distal, el tamaño tumoral promedio fue de 88,6 mm. En todos los pacientes se realizó una pancreatectomía distal siendo tres laparoscópicas, no hubo reoperaciones ni fallecidos, dos pacientes tuvieron carcinoma invasor asociado. En conclusión, la presentación de NQM es mayor en mujeres de edad media siendo la localización en el páncreas distal y el porcentaje de malignidad bajo. La cirugía laparoscópica es una alternativa de manejo.

Mucocele apendicular / Appendiceal mucocele

El mucocele apendicular es una dilatación quística de la luz del apéndice ileocecal de etiología obstructiva que produce un acúmulo retrógrado de sustancia mucoide. Representa un 8-10% de todos los tumores apendiculares, con una incidencia del 0,2-0,3% de todas las apendicectomías. La etiología hace referencia a procesos no neoplásicos o tumorales (cistoadenoma o cistoadenocarcinoma mucinoso). Un incorrecto abordaje terapéutico o un diagnóstico histológico poco definido o incompleto, pueden provocar un síndrome de pseudomixoma peritoneal. El objetivo de este trabajo es describir un caso de mucocele apendicular. Se trata de una paciente de sexo femenino de 68 años de edad, que consultó en el servicio de emergencia por dolor abdominal a nivel de epigastrio, irradiado a la fosa iliaca derecha, con clínica sugerente de proceso inflamatorio apendicular, y tomográficamente se evidenció imagen probable de mucocele apendicular. Luego del tratamiento quirúrgico, la paciente mejoró significativamente. La TC con contraste es más comúnmente utilizada para el diagnóstico prequirúrgico. El objetivo final de la terapéutica es la de evitar la ruptura del mucocele apendicular y el síndrome de pseudomixoma peritoneal, por lo cual es importante contar con la TC simple y contrastada de abdomen y pelvis para la adecuada planeación quirúrgica en los casos sospechosos de mucocele apendicular.

The appendiceal mucocele is cystic dilation light ileocecal appendix obstructive etiology which causes a retrograde accumulation of mucoid substance. A 8-10% of all appendiceal tumors, with an incidence of 0.2 to 0.3% of all appendectomies. The etiology refers to non-neoplastic or tumor (cystadenoma or mucinous cystadenocarcinoma) processes. Incorrect therapeutic approach or poorly defined or incomplete histological diagnosis, can cause peritoneal pseudomixoma syndrome. The aim of this paper is to describe a case of appendiceal mucocele. It is a female patient of 68 years old who consulted in the emergency department for abdominal pain at epigastrium, radiating to the right iliac fossa, with suggestive clinical appendicular inflammatory process, and tomographic image likely was evident appendiceal mucocele. After surgery, the patient improved significantly. Contrast CT is most commonly used for presurgical diagnosis. The ultimate goal of therapeutic is to prevent rupture of the appendiceal mucocele syndrome peritoneal pseudomixoma, so it is important to have the simple and contrast CT of the abdomen and pelvis for proper surgical planning in suspected cases of appendiceal mucocele.

Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum.

Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73%) or 2 (27%) for MCRNLMP; for pc-CCRCC were 1 (31%), 2 (60%), and 3 (9%); and for non-cystic CCRCC were 1 (9%), 2 (52%), 3 (26%), and 4 (13%). MCRNLMP were either pT stage 1 (91%) or 2 (9%), pT stages for pc-CCRCC were 1 (92.5%), 2 (1.5%), and 3 (6%) and for non-cystic CCRCC were 1 (58.7%), 2 (6.5%), and 3 (34.8%). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58%, CI 38.3-73.5%), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report.

Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are diagnoses that were classified by the World Health Organization in 2010 as mucin-producing bile duct tumors of the hepatobiliary system. The preoperative differential diagnosis between these two entities is difficult; the presence of a communication with the bile duct is usually considered as a typical sign of IPMN-B. However, the presence of an ovarian-like stroma (OLS) has been established to define the diagnosis of MCN-L. We present the case of a 33-year-old woman with a rapid progression of a cystic tumor of the liver. In 2 years, the lesion increased from 27 to 64 mm and a dilation of the left hepatic duct appeared. Percutaneous transhepatic drainage with a biopsy was performed. No malignant cells were found on biopsy. Because of the rapid progression of the cystic tumor and unclear malignant potential, left hemihepatectomy was performed. Even though tumor masses were present in the biliary duct, on the basis of the presence of OLS, histology finally confirmed MCN-L with intermediate-grade intraepithelial dysplasia to high-grade intraepithelial dysplasia. The patient is currently under oncologic follow-up with no signs of recurrence of the disease. We present a rare case where MCN-L caused a dilation of the left hepatic duct, a sign that is usually a characteristic of IPMN-B.

Oligodendroglial cell proliferation arising in an ovarian mature cystic teratoma. Clinicopathological, inmunohistochemical, and ultrastructural study of a case that may represent an oligodendroglioma.

Ovarian mature cystic teratoma (OMCT) is an ovarian benign neoplasm with excellent prognosis presenting components of the three germinal layers. However, transformation into a malignant neoplasm is a rare event (so-called somatic transformation). In most of the cases, the malignant component expresses as epidermoid carcinoma, but occasionally central nervous system tumors occur. Some of the previously reported tumors are astrocytoma, glioblastoma, and ependymoma. Somatic transformation of OMCT into an oligodendroglioma is exceptional. We report a 19-year-old female with a left OMCT with an area of oligonedroglial cells proliferation characterized by immunohistochemical studies with positivity for GFAP and S100, with a low Ki67 index (5%). Additionally, electron microscopy revealed oligodendrocytes with parallel bundles of cytoplasmic intermediate filaments, confirming the oligodendroglial nature of the proliferation. The patient was treated only with left oophorectomy, and three and half years after surgery, there is no evidence of disease.

Cirugía mínimamente invasiva de tumoración quística abdominal gigante en paciente puérpera / Minimally invasive surgery of a giant abdominal cyst tumor in a puerperal patient

La incidencia de masas anexiales diagnosticadas durante el embarazo varía según las diferentes series consultadas; sin embargo, masas de tamaño superior a 6 cm suponen casos excepcionales en la literatura. El manejo de este tipo de pacientes supone un reto para el equipo multidisciplinario, ya que se debe tener en cuenta el riesgo de cáncer de ovario en este tipo de pacientes. Se trata de una gestante de 34 años a la que se le diagnostica una masa anexial gigante durante el control ecográfico del primer trimestre. Ante la ausencia de sufrimiento fetal y teniendo en cuenta las características radiológicas de la lesión, se decide mantener una actitud expectante a la espera de un parto vía vaginal y poder realizar una cirugía electiva para la exéresis de la lesión. Tras el parto, la paciente sufre una anemización progresiva, por lo que se decide priorizar la intervención quirúrgica. Se llevó a cabo una exéresis de la masa mediante un abordaje mínimamente invasivo. Tanto la paciente como el recién nacido, tuvieron una evolución favorable. La paciente fue dada de alta tres días después de la cirugía(AU)

The incidence of adnexal masses diagnosed in pregnancy varies by the different consulted series; however, over 6cm long masses represent exceptional cases in literature. The management of this type of patients is a challenge for the multidisciplinary team since the risk of ovarian cancer should be taken into consideration in this type of patients. This is a 34 years-old pregnant patient who was diagnosed with giant adnexal mass during the echographic control of the first trimester. Due to the lack of fetal distress and the radiological characteristics of the lesion, it was decided to keep surveillance to wait for the vaginal delivery and to perform an elective surgery to remove the lesion. After the childbirth, the patient suffered a process of anemia, so it was decided to prioritize surgery. The mass was excised through a minimally invasive approach. Both the mother and the newborn had favorable recovery and the patient was discharged from hospital three days after surgery(AU)

Glial Cyst inside the Cerebellar Parenchyma: Case Report and Literature Review

Cystic lesions inside the brain parenchyma are a common pathological finding in the investigation of patients with suspicion of cerebral tumor. Histological diagnosis is important to guide the patient's treatment and follow-up, and to determine prognosis. Among patients diagnosed with cerebral cysts, most are located in the parenchyma above the tentorium. The authors describe the case of a patient who had been suffering from dizziness and balance disturbance for 4 months; the investigation identified a cyst inside the cerebellar right hemisphere. A surgical procedure was performed, and the biopsy microscopic analysis diagnosis was glial cyst.

Patologias intraparenquimatosas de características císticas são comumente identificadas em pacientes que estão sob investigação de neoplasias cranianas. Lesões císticas são mais prevalentes acima da tenda cerebelar, e o diagnóstico histológico é fundamental para determinar o tratamento, seguimento e prognóstico do paciente. Os autores relatam o caso de um paciente com sintomas de vertigem e alteração no equilíbrio dinämico de 4 meses de evolução, cuja investigação diagnosticou cisto intraparenquimatoso no hemisfério cerebelar direito. Procedeu-se com intervenção cirúrgica cujo diagnóstico histopatológico foi compatível com Cisto Glial

Cystic angiosarcoma of the liver. A previously undescribed neoplasm.

 We report an example of a cystic hepatic angiosarcoma that to our knowledge has not been previously described. The patient was a 70 year old woman who was admitted to the emergency room because of hypovolemic shock. A computed tomography showed four heterogeneous hepatic cystic masses varying from 2.5 to 11.2 cm; one of these with rupture and formation of a subcapsular hematoma. The cyst wall was lined by several layers of neoplastic epithelioid and spindle shaped endothelial cells that in some areas extended to the underlying stroma. They expressed CD31 and CD34, and were negative for cytokeratin. The patient is alive with residual hepatic cystic angiosarcoma. However, follow up is too short to be significant.

Clinical outcomes in patients with isolated serous tubal intraepithelial carcinoma (STIC): A comprehensive review.

OBJECTIVE. Serous tubal intraepithelial carcinoma (STIC) is currently considered the precursor lesion of pelvic (i.e., ovarian or peritoneal) high-grade serous carcinoma. The incidence of STIC has been reported to range from 0.6% to 7% in BRCA mutations carriers. However, the clinical outcome of patients with 'isolated' STIC remains elusive. The aim of this study is to review the published literature on isolated STIC to determine outcomes of these ients and present a summary of management strategies. METHODS. A systematic English-language literature search was conducted in PubMed, MEDLINE-Ovid, Scopus, EBSCO host, Cochrane Library of articles published from February 2006 to April 2015. Study inclusion criteria for review were the following: risk-reducing salpingo-oophorectomy (RRSO), BRCA mutation carriers, non-BRCA mutation carriers, and benign surgical indication. Exclusion criteria were as follows: the presence of synchronous gynecological cancers, concurrent non-gynecological malignancies, the presence of ovarian intraepithelial lesions, and articles that did not include any clinical information and were restricted to pathology information only. RESULTS. A total of 78 patients with isolated STIC were included in our analysis. The median age for all patients was 53.7 years (range; 37-83). Surgical indication was RRSO in 67 patients with BRCA mutations or high-risk personal or family history. In the other 11 patients, an incidental STIC was detected after surgery for non-cancerous indications. Eleven (16.4%) patients received chemotherapy after the diagnosis of STIC. The follow-up time ranged from 2 to 150 months. Three (4.5%) patients with BRCA mutations were diagnosed with primary peritoneal carcinoma (PPC) during the follow-up at 43, 48 and 72 months after RRSO. CONCLUSIONS. The rate of primary peritoneal carcinoma in patients with BRCA mutations and isolated STIC is 4.5%. The role of adjuvant therapy remains elusive and routine surveillance with tumor markers and imaging is not warranted.