Thoracic benign cystic mesothelioma.

BACKGROUND: Benign cystic mesotheliomas (BCMs), also known as multilocular mesothelial inclusion cysts, inflammatory inclusion cysts or multicystic mesothelial proliferation, are frequently observed in females and are localised localised in the pelvic peritoneum. They are rarely present in the thoracic and mediastinal areas; however, these locations have been reported in a few cases in the literature. CASE PRESENTATION: We present the case of a woman with an intrathoracic BCM. A 28-year-old female patient presented with a cystic mass of 8 × 6 × 6 cm in the left hemithorax shown by computed tomography of the thorax. The patient underwent cystic mass excision with video-assisted thoracoscopic surgery (VATS), which was completed without complications. The diagnosis was confirmed histopathologically after the surgical resection. CONCLUSIONS: Due to BCMs' non-specific clinical symptoms and radiological imaging, preoperative diagnosis is difficult, and they are often confused with pericardial cysts. There is no standard treatment protocol; however, VATS and en bloc resection are the most frequently used treatment options for mediastinal localization. Since these lesions slow proliferation rates have the potential for local recurrence and low malignant transformation, close follow-up is recommended. In this case report, we aimed to present a rare BCM case with intrathoracic paracardiac localization was completely excised through VATS. No recurrence has been detected in three years of follow-up.

Benign multicystic peritoneal mesothelioma: literature review and update

Benign multicystic peritoneal mesothelioma (BMPM) is a rare peritoneal tumor diagnosed predominantly in pre-menopausal women. Associated risk factors include endometriosis and pelvic inflammatory disease in women, and prior abdominal surgery in both genders. To date, the pathogenesis of this disease remains controversial with possible etiologies, including a neoplastic versus a reactive process. Given the risk factors, some authors believe that this disease is secondary to a reactive process. However, because some studies describe cases where there is no prior surgical history or inflammatory milieu present, and because of this entity's predilection for recurrence, some authors believe the origin to be neoplastic. Some genetic and familial associations have also been reported. Malignant transformation is extremely rare, with only two cases reported in the literature, despite the recurrence potential. Like the etiology, the name of this entity is also controversial. Some authors prefer the term "peritoneal inclusion cyst (PCM)" instead of "benign cystic mesothelioma" and argue that the term mesothelioma should only be used when there is evidence of atypia. Most cases of BMPM are discovered incidentally. Others reflect sequela of tumor mass effect. It appears intra-operatively as large, multi-focal, cystic lesions in the peritoneal and pelvic cavity. Diagnosis is achieved through surgical sampling with histopathological examination. Immunobiologically, BMPM exhibits multiple small cystic spaces with flattened lining containing calretinin positive cells without atypical features, mitotic figures, or tissue invasion. Treatment includes cytoreductive surgery. Here we present a case of BMPM in a 60-year-old male - a rare disease in an uncommon patient population.

[Benign multicystic peritoneal mesothelioma]. / Benignus multicysticus peritonealis mesothelioma.

Benign multicystic peritoneal mesothelioma is a rare benign tumor originating from the peritoneum, affecting mostly young, fertile women. Its presentation is non-specific, thus the final diagnosis is made after the histological examination. A young female patient presented with incarcerated inguinal hernia of which an emergency surgery was performed. During the operation a cystic mass neighboring the round ligament in the canal of Nuck was removed. No inguinal hernia was found. The histological examination confirmed the diagnosis of benign multicystic mesothelioma. The patient was referred to a center performing hyperthermic intraperitoneal chemotherapy, where laparoscopic exploration was performed. The second surgery revealed no residual tumor or any other pathology. A 41-year-old male patient, 4 years before presenting at our ward, had an elective umbilical hernia repair surgery. During the operation 2 cm big cystic mass was removed from the peritoneum, and the histological examination revealed benign multicystic mesothelioma. In 2018, acute surgery was performed due to a periappendicular abscess, while during the surgery a multicystic mass situated on the distal end of the appendix was also removed. The pathological finding confirmed the recurrence of the first tumor. The radiological examination did not find any signs of residual tumor mass anywhere else. The chances of malignant transformation in cases of benign multicystic peritoneal mesothelioma are low. The suggested treatment is en bloc surgical removal of the mass, however, in these cases recurrence is still 50%. If during follow-ups the recurrence of the tumor is found, a total peritonectomy or hyperthermic intraperitoneal chemotherapy is advisable. Orv Hetil. 2019; 160(21): 839-843.

Multicystic Mesothelioma of the Omentum Presenting as an Incidental Finding.

Multicystic mesotheliomas of peritoneal origin (also termed benign multicystic mesotheliomas) are uncommon and are exceptionally rare in the omentum. Lack of familiarity of this entity could lead to overinterpretation as a malignancy or present a challenge at frozen section. We describe a case of multicystic mesothelioma arising in the omentum found incidentally at laparotomy for endometriosis. Frozen section evaluation presented a challenge. Grossly, the mass in the omentum was multicystic and mucoid, raising the concern for a mucinous neoplasm. The cysts, however, were lined by flattened cells without atypia. At permanent section evaluation, calretinin positivity confirmed the mesothelial origin. Awareness of this entity is important, as it may be interpreted as a malignancy, particularly intraoperatively.

An unusual cause of acute surgical abdomen: benign multicystic peritoneal mesothelioma associated with adenomatous tumor.

Benign multicystic peritoneal mesothelioma (BMPM) is a rare disease that primarily affects fertile women with previous abdominal surgery. BMPM associated with adenomatous tumor is a single case report, according to our opinion. The patient had a history of abdominal surgery nine years ago for ovarian cysts. Upon admission, the diagnosis was acute surgical abdomen with acute peritonitis signs. The treatment applied consisted in the removal of peritoneal cysts and partial omentectomy. Only immunohistochemical examination established the diagnosis. The aim is to discuss diagnostic and therapeutic difficulties, underlining that there is no consensus on the use of chemotherapeutics. In conclusion, establishing a preoperative diagnosis is difficult if not impossible. One of the causes of acute surgical abdomen may be BMPM. The malignant transformation of this disease is rare, but the disease recurrence rate is over 50%, and it is often recommended to be monitored through abdominal computed tomography.

Multicystic mesothelioma: Operative and long-term outcomes with cytoreductive surgery and hyperthermic intra peritoneal chemotherapy.

BACKGROUND: Multicystic peritoneal mesothelioma (MCPM) is an extremely rare disease with 40-50% rate of recurrence after surgical debulking. Due to the recurrent nature of the disease, the option of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) was offered for this condition. In the present study we aimed to describe the outcomes of this strategy in a single center cohort. METHODS: We retrospectively reviewed a prospectively collected database of all patients with MCPM that underwent the combined procedure in our center. Clinical presentation, operative procedures and outcomes were reviewed. RESULTS: Between August 1997 and October 2017, 19 patients with MCPM underwent 20 cytoreduction and HIPEC procedures in our center. The majority of the patients were females (n = 17, 89%), and mean age was 42, as reported in other series. Disease extent, as measured by mean peritoneal carcinomatosis index (PCI) was 15.5 ± 9.9 and total number of procedures performed 6.7 ± 2.6. Major complications occurred in 3 (15%) patients, with no perioperative mortality. After a median of follow-up of 69 months (range 4-220) all patients were alive and 4 patients had recurrence (21%). Patients with high PCI (defined by median PCI) had shorter recurrence free survival (RFS) than patient with low PCI (mean RFS = 106.4 ± 6.6 for high PCI vs. 125.6 ± 34.1 for low PCI, p = 0.03). CONCLUSION: Cytoreduction and HIPEC offer low recurrence rate and prolonged mean RFS for patients with MCPM. The combined procedure can be offered with acceptable morbidity in specialized centers.

Benign multicystic mesothelioma and peritoneal inclusion cysts: are they the same clinical and histopathological entities? A systematic review to find an evidence-based management.

PURPOSE: Peritoneal mesothelial cysts (PMC) are a clinical dilemma because of their true pathogenic nature. Many definitions have been associated with PMC, including "benign multicystic mesothelioma", "cystic mesothelioma", "multilocular peritoneal inclusion cysts", ''inflammatory cysts of the peritoneum" or "postoperative peritoneal cyst". METHODS: We herein performed a systematic review of the literature focusing on clinical and histopathological aspects of PMC, diagnosis, and therapies. Moreover, we described our experience with a case of PMC in a young female. RESULTS: Since there is often a history of prior surgery or inflammatory disease, most authors consider PMC of reactive origin. However, in some cases they occur without any documentable signs of disease or injury. A variety of clinical findings can complicate the preoperative assessment and a multitude of histological pictures may potentially lead to a misdiagnosis. The absence of a uniform treatment strategy and lack of long-term follow-up often hinder the accurate definition leading to unnecessary or unnecessarily aggressive therapy. CONCLUSIONS: PMC are more common than had previously been thought. Most authors consider them non-neoplastic; thus the designation of "peritoneal inclusion cyst" is preferable. The term "mesothelioma" should be used only in cases of histological evidences of atypia. The high rates of recurrence suggest that the goal of treatment should not be necessarily complete eradication, but symptomatic relief through individualized treatment. This is a topic of particular importance, especially in young female where recurrence rates could be lower than those reported in adults and where an improperly aggressive treatment could have repercussions on fertility.

Multiple pelvic cysts in a patient with familial Mediterranean fever: Benign cystic mesothelioma.

Benign cystic mesothelioma (BCM) is a rare tumor arising from endothelial cells of the pelvic visceral or parietal peritoneum. It is a clinically and histopathologically benign disease. Etiology and pathogenesis of BCM remain unclear. Familial Mediterranean fever (FMF) is an inherited disorder characterized by episodes of fever, and abdominal, chest and/or joint inflammation. Association between malignant mesothelioma and FMF has been reported previously; however, co-existence of FMF and BCM is rare. Here, we report a case of BCM in a 43-year-old male patient with FMF.

Radiological predictors of complete cytoreduction in 59 patients with peritoneal mesothelioma treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy at a UK referral centre.

OBJECTIVE: To assess the imaging features of peritoneal mesothelioma and identify key anatomical sites that aid patient selection for complete cytoreduction. METHODS: Pre-operative imaging of 59 (32 males, 27 females) patients who underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) for histologically proven peritoneal mesothelioma [36 malignant peritoneal mesothelioma, 23 cystic mesothelioma were reviewed. Imaging findings were correlated with surgical outcome. Best imaging predictors of complete cytoreduction, n = 22 and major tumour debulking, n = 12 were assessed. RESULTS: Most patients (88.9%) had diffuse peritoneal disease with mean radiological peritoneal cancer index of 18 ± 12 (range 2-39). Disease in the lesser omentum (n = 10), porta hepatis (n = 8), perigastric area (n = 5), mesentery (n = 25), small bowel (n = 17), hydronephrosis (n = 1), concurrent pleural disease (n = 2), lymph nodes (n = 1) and abdominal wall disease (n = 4) was considered unfavourable. While 78.9% of patients who underwent complete cytoreduction had no disease at unfavourable sites, 75% of those who underwent MTD did have disease at these sites. There was significant difference in the radiological peritoneal cancer index, severity of upper abdominal disease, small bowel and mesenteric involvement between patients who underwent complete cytoreduction and MTD for malignant peritoneal mesothelioma. Complete cytoreduction was not achieved in the presence of a rind of soft tissue around the small bowel (p = 0.016) and was unlikely in the presence of large volume upper abdominal disease (p = 0.06). CONCLUSION: Involvement of key anatomical sites such as small bowel serosa and large volume upper abdominal disease reduced the likelihood of achieving complete cytoreduction in patients with malignant peritoneal mesothelioma. Advances in knowledge: Demonstration of small bowel disease and large volume upper abdominal disease on imaging in patients with malignant peritoneal mesothelioma can be used to identify patients who may not benefit from cytoreductive surgery.

Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma.

BACKGROUND: Benign peritoneal cystic mesothelioma (BPCM) is a rare disease entity that arises from mesothelioma cells. We describe a rare case of BPCM recurrence 36 years after its initial presentation. CASE: A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. She had a preceding history of known BPCM treated with extensive debulking surgery. She presented after 36 years of clinical remission. A repeat laparotomy for a debulking surgical procedure confirmed a recurrence of BPCM. CONCLUSION: Our current case represents a woman with a remote recurrence of BPCM after initial optimal debulking surgery. Her clinical presentation of recurrence after 36 years illustrates the need for long-term follow-up and clinical suspicion in symptomatic patients with previously diagnosed BPCM.

Multicystic peritoneal mesothelioma: A short review.

Multicystic peritoneal mesothelioma (MCPM) is a rare neoplasm, predominantly affecting female patients during their reproductive years. The lesion is usually distributed diffusely in the abdomen and pelvis, but the peritoneum of the pelvic organs is the most common site. MCPM is composed of fluid-filled translucent cysts, connected by varying amounts of fibrous tissue, and lined by a layer of mesothelial cells. Because of the rarity of this disease, the pathogenesis and natural history of MCPM remain poorly understood and continuously debated. Some authors consider it to be a reactive process for its association with prior surgery or abdominal inflammation. But its high rate of local-regional recurrence, as well as its malignant potential, suggests a neoplastic etiology. Preoperative diagnosis is often very difficult. Imaging methods, such as ultrasound, computed tomography, and magnetic resonance imaging, are of little value for an accurate diagnosis of MCPM. The definitive diagnosis relies on histologic examination of target lesions combined with immunohistochemical stains. There is no consensus on the clinical management of MCPM, although surgical removal remains the first-line treatment of choice. But no standards have been reached concerning which surgical options-traditional debulking surgery or more aggressive one-should be chosen. Alternative therapeutic approaches include hand-off treatment, hormonal supplementation, laser vaporization, and sclerotherapy, and they all come with uncertain results. Moreover, the lesions show no response to adjuvant chemotherapy and radiotherapy. This article aimed to focus on those controversial problems in pathogenesis, natural history, diagnosis, and treatment strategies to help medical workers to better understand this rare disease.

Mesotelioma benigno como causa de dolor abdominal inespecífico en un paciente con Enfermedad de Crohn / Benign multicystic peritoneal mesothelioma in a patient with Crohn disease

Benign multicystic peritoneal mesothelioma is an uncommon lesion arising from the peritoneal mesothelium. It is asymptomatic or presents with unspecific symptoms. Imaging techniques may reveal it, however the final diagnosis can only be made by histopathology. Surgery is the only effective treatment considering its high recurrence rate. We report a 19 years old male with Crohn’s disease. Due to persistent abdominal pain, an abdominal magnetic resonance imaging was performed, showing a complex cystic mass in the lower abdomen. The patient underwent surgery and the lesion was completely resected. The pathological study reported a benign multicystic peritoneal mesothelioma.

Diffuse malignant biphasic peritoneal mesothelioma with cystic areas.

UNLABELLED: We report a case of peritoneal biphasic mesothelioma with cystic areas in a patient with professional exposure to asbestos. It showed focal epithelial glandular and papillary proliferations, also presenting fluid filled cysts, whose wall consisted of a proliferation of spindle cells. Atypia and mitoses were very scanty. EMA, vimentin, CK5/6, D2-40, calretinin and P53 were positive and desmin was negative in both epithelial and spindle areas, including the ones surrounding the cystic spaces. These findings gave an essential aid in the differential diagnosis with a benign cystic mesothelioma and with a cystic epithelial mesothelioma with secondary pseudosarcomatous myofibroblastic proliferation. The presence of cystic areas in a malignant mesothelioma could make difficult the diagnosis. A large amount of tumour tissue is necessary for confirming the biphasic histotype, an aggressive histotype, even in the presence of mild histological features and of some others favourable clinical prognostic indices as in this case. To our knowledge this is the first case of malignant peritoneal biphasic mesothelioma with cystic features reported in the literature. KEY WORDS: Cystic Mesothelioma, Immunohistochemistry, Malignant Mesothelioma, Peritoneal Diseases, Mesothelial Neoplasms.

Malignant transformation in non-recurrent peritoneal cystic mesothelioma Our experience and review of the literature.

AIM: Cystic peritoneal mesothelioma is commonly regarded as a benign neoplasm at its first manifestation. It can only seldom show malignant transformation, and only after repeated postoperative recurrences. MATERIAL OF STUDY: We hereby represent a unique case of peritoneal cystic mesothelioma, malignant since its first presentation. We observed a 73 year-old man presenting with intermittent abdominal pain and periumbilical swelling. At surgery, we found an extensive, oval-shaped, multi-lobed cystic formation that was surgically removed. RESULTS: Histopathology was consistent with a malignant peritoneal cystic mesothelioma. In agreement with oncologists, we decided not to give any further therapy because of the few possibilities offered by systemic chemotherapy and the paucity of published data from the literature. We planned periodical follow-up including US scan every six and CTscan every 12 months. DISCUSSION: Several cases of malignant transformation occurring after repeated recurrences of peritoneal mesothelioma have been reported. To our knowledge, this is the first case showing "ab initio" histological features of malignancy, typical of an active, proliferating and infiltrating lesion. In addition, advanced age and male gender of our patient are extremely peculiar CONCLUSION: Our observation suggests the possibility, although very rare, that peritoneal cystic mesothelioma may present as malignant since its first manifestation. KEY WORDS: Peritoneal cystic mesothelioma.

Benign Multicystic Peritoneal Mesothelioma: AIRP Best Cases in Radiologic-Pathologic Correlation.

RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).