A focal adenomatoid-microcystic pattern is not uncommon in peritoneal mesothelioma, but tumors composed almost exclusively of this pattern are distinctly rare and have not been well characterized. A small subset of mesotheliomas (mostly in children and young adults) are characterized by gene fusions including EWSR1/FUS::ATF1, EWSR1::YY1, and NTRK and ALK rearrangements, and often have epithelioid morphology. Herein, we describe five peritoneal mesothelial neoplasms (identified via molecular screening of seven histologically similar tumors) that are pure adenomatoid/microcystic in morphology and unified by the presence of an NR4A3 fusion. Patients were three males and two females aged 31-70 years (median, 40 years). Three presented with multifocal/diffuse and two with a localized disease. The size of the individual lesions ranged from 1.5 to 8 cm (median, 4.7). The unifocal lesions originated in the small bowel mesentery and the mesosigmoid. Treatment included surgery, either alone (three) or combined with hyperthermic intraperitoneal chemotherapy (two), and neoadjuvant or adjuvant chemotherapy (one case each). At the last follow-up (6-13 months), all five patients were alive and disease-free. All tumors were morphologically similar, characterized by extensive sieve-like microcystic growth with bland-looking flattened cells lining variably sized microcystic spaces and lacked a conventional epithelioid or sarcomatoid component. Immunohistochemistry confirmed mesothelial differentiation, but most cases showed limited expression of D2-40 and calretinin. Targeted RNA sequencing revealed an NR4A3 fusion (fusion partners were EWSR1 in three cases and CITED2 and NIPBL in one case each). The nosology and behavior of this morphomolecularly defined novel peritoneal mesothelial neoplasm of uncertain biological potential and its distinction from adenomatoid variants of conventional mesothelioma merit further delineation as more cases become recognized.
- Keywords
- EWSR1, NR4A3, adenomatoid tumor, gene fusion, microcystic, peritoneal mesothelioma,
- MeSH
- Adenoma * MeSH
- DNA-Binding Proteins genetics MeSH
- Adult MeSH
- Gene Fusion MeSH
- Middle Aged MeSH
- Humans MeSH
- Mesentery pathology MeSH
- Mesothelioma * genetics MeSH
- Biomarkers, Tumor genetics MeSH
- Peritoneal Neoplasms * genetics pathology MeSH
- Cell Cycle Proteins genetics MeSH
- Receptors, Thyroid Hormone genetics MeSH
- Repressor Proteins genetics MeSH
- Aged MeSH
- Receptors, Steroid * genetics MeSH
- Trans-Activators genetics MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Names of Substances
- CITED2 protein, human MeSH Browser
- DNA-Binding Proteins MeSH
- Biomarkers, Tumor MeSH
- NIPBL protein, human MeSH Browser
- NR4A3 protein, human MeSH Browser
- Cell Cycle Proteins MeSH
- Receptors, Thyroid Hormone MeSH
- Repressor Proteins MeSH
- Receptors, Steroid * MeSH
- Trans-Activators MeSH
It is well known that the blood supply of the greater omentum and female internal genital organs are not physiologically connected. There is also no mention of such anatomical variation in anatomical, radiological, or surgical textbooks. Here we present a very rare case report of atypical double arterial anastomosis (the first and second variant artery) between the right limb of the omental arcade of Barkow, uterus, and right ovary, which was found during a routine student anatomical dissection course. It is very challenging to find a proper explanation for the presence of the described anatomical variation; however, we hypothesized that it is based on their common embryonic origin - the mesentery. The first and second variant arteries could be remnants of transient anastomoses or collateral circulation, which were present during embryonic development and persisted until adulthood. Moreover, during our literature review, we noticed that the general description of omental blood supply and its possible variations is relatively poor; therefore, we emphasize the need for more precise knowledge regarding these anatomical parts, which could help surgeons who are performing abdominal or pelvic surgeries in preventing avoidable bleeding.
INTRODUCTION: Aggressive fibromatosis, also known as desmoid tumour (DT), is a locally invasive soft tissue malignancy originating from fascial planes, connective tissue, and musculoaponeurotic structures of the muscles. The symptoms greatly depend on the location and size of the tumour. CASE REPORT: A 68-year-old male patient without any comorbidities with a large, palpable mass in the abdomen underwent computed tomography (CT) of the abdomen during diagnostic examination in September 2017 in another centre. The CT scan revealed a giant intraperitoneal 30×40cm tumour without signs of infiltrating the surrounding organs and large vessels. The tumour biopsy revealed an aggressive DT. The patient was scheduled for tumour resection. Midline laparotomy was performed in the supine position under general anaesthesia. After gaining access to the abdominal cavity, 8 litres of clear ascites were evacuated. The tumour was not attached to the abdominal wall. Large omentum was freed from the DT. The perioperative finding confirmed the CT images of DT encapsulation of the medial colic artery, part of the small intestine, and transverse colon. The tumour was resected with part of the mesenteric radix, 30 cm of small intestine, and 2/3 of the transverse colon. After the DT was removed entirely, the small intestine was re-anastomosed end to end. The abdominal cavity and the liver were carefully checked for bleeding. The abdominal cavity was closed in a standard manner. RESULTS: The postoperative hospital stay was uneventful. The patient was discharged on the 7th postoperative day with prophylactic low weight molecular heparin for one month. Currently, we have five months of follow-up with no signs of DT recurrence based on CT examination. The histology of the resected tumour confirmed the diagnosis of a desmoid tumour (aggressive abdominal fibromatosis). CONCLUSION: Desmoid tumours are benign neoplasms with no metastatic potential. However, their treatment is challenging due to their aggressive growth, infiltrative behaviour, and a high tendency to recur.
- Keywords
- abdominal, desmoid tumour, fibromatosis,
- MeSH
- Desmoid Tumors * diagnostic imaging surgery MeSH
- Abdominal Wall * surgery MeSH
- Laparotomy MeSH
- Humans MeSH
- Mesentery surgery MeSH
- Tomography, X-Ray Computed MeSH
- Aged MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Aged MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
Umbilical cord hemangioma is a rare tumor that can be associated with significant fetal and perinatal complications. Although usually described as a single anomaly, sometimes these tumors are reported in association with other vascular lesions. We report an unusual case of simultaneous occurrence of two umbilical cord hemangiomas and vascular malformation of the transverse mesocolon in a stillborn fetus with hydrops. To our knowledge, this is the first report of two simultaneously occurring umbilical cord hemangiomas. Moreover, presence of associated vascular malformation of transverse mesocolon could support the hypothesis of underlying predisposition to the development of vascular tumors.
- Keywords
- fetal hydrops, hemangioma, hydrops fetalis, stillbirth, transverse mesocolon, umbilical cord, vascular malformation,
- MeSH
- Vascular Malformations * MeSH
- Hemangioma * complications MeSH
- Hydrops Fetalis etiology MeSH
- Humans MeSH
- Mesocolon * MeSH
- Umbilical Cord MeSH
- Pregnancy MeSH
- Check Tag
- Humans MeSH
- Pregnancy MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
INTRODUCTION: Diverticula of the small bowel are quite rare findings, frequently confirme only by necropsy. The authors describe the signs and symptoms resulting from complications of small bowel diverticula as well as the diagnostic options and treatment modalities. The paper also presents the results of treatment in the Department of Surgery at University Hospital in Pilsen. METHOD: Our retrospective study involved 6 patients who were operated on due to complications of small bowel mesenteric diverticulum at the Department of Surgery at University Hospital in Pilsen between 1 January 2006 and 1 January 2016. We assessed the number of days in hospital, emesis or pathological stools (diarrhoea or bleeding), admission body temperature and abdominal clinical signs, operating time and postoperative complications according to the Clavien-Dindo classification. As for laboratory parameters, leukocytosis and C-reactive protein levels were evaluated. RESULTS: The results of our study are similar to those reported in available literature. After medical history and physical examination which are ne-cessary, laboratory methods, and less frequently also imaging methods, can be used in diagnosis. The treatment of complications of small bowel diverticular disease is only surgical. The postoperative course is burdened by comorbidities of these patients as they are very often elderly. CONCLUSION: Management of complicated small bowel diverticular disease is only surgical and consists in laparotomy with thorough lavage of the abdominal cavity and with drainage. Key words: small bowel acute abdome.
- Keywords
- small bowel acute abdome,
- MeSH
- Abdomen, Acute * etiology surgery MeSH
- Diverticulum * complications surgery MeSH
- Humans MeSH
- Mesentery MeSH
- Intestinal Diseases * complications surgery MeSH
- Retrospective Studies MeSH
- Aged MeSH
- Check Tag
- Humans MeSH
- Aged MeSH
- Publication type
- Journal Article MeSH
Sclerosing mesenteritis is a rare non-cancerous disease affecting the small bowel mesentery, which occurs predominantly in older age. The exact etiology is not known. Clinical symptoms are very variable and they include abdominal pain and distention, nausea, and vomiting. We present a case of a 4-month-old boy, who died suddenly at home. Based on the investigation of the circumstances of death, results of toxicology tests and histological examination the case was concluded as sudden infant death syndrome. During life, the child had repeated abdominal pain with distention. Secondary autopsy finding was a significant thickening of terminal ileum mesentery the length of 2,5 cm, with thickness reaching up to 1,5 cm and affecting almost half the circumference of the small intestine. According to the current literature, the case discussed in this article is the youngest recorded.
- Keywords
- SIDS, child, fibrinoid necrosis, sclerosing mesenteritis,
- MeSH
- Abdominal Pain MeSH
- Infant MeSH
- Humans MeSH
- Mesentery MeSH
- Panniculitis, Peritoneal * MeSH
- Autopsy MeSH
- Check Tag
- Infant MeSH
- Humans MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
OBJECTIVES: Immune checkpoints inhibitors (ICI) represent a new therapy option for the treatment of several advanced tumors. However, this therapy has been linked to a spectrum of ICI related autoimmune (AI) adverse events. Some may be life threatening and their diagnosis is tricky. The aim of our study was to describe various imaging appearances of ICI related secondary hypophysitis and other coincidental AI diseases. MATERIAL AND METHODS: We included 28 patients (19 females, 9 men, mean aged 58±13 years), who were consecutively treated mostly for advanced stage melanoma by different ICI. All their CT/MRI records and clinical data were reviewed. RESULTS: We found 5 (18%) cases of endocrinology proven secondary hypophysitis; 2 cases of panhypopituitarism and 3 cases of central hypocortisolism. Four cases were MRI positive, 1 case was MRI negative. Three cases were accompanied by other AI diseases: 1 by hemorrhagic colitis and mesenterial lymphadenitis, 1 by AI pancreatitis and 1 by pneumonitis. On MRI pituitary gland was swollen in 3 cases, twice enhanced non-homogenously, once homogenously; infundibular enlargement was present in 2 cases. Those 3 cases reacted to glucocorticoid therapy by hypophyseal shrinkage. In 1 case of MRI positive hypophysitis, the pituitary gland was not enlarged, slightly nonhomogeneous with peripheral contour enhancement; no reaction to glucocorticoids was mentioned. CONCLUSION: Secondary hypophysitis is probably more common ICI related adverse event than reported in the literature. Its MRI appearance is variable. Most of our cases were in coincidence with other AI ICI related events that affected their clinical manifestations.
- MeSH
- Autoimmune Diseases chemically induced MeSH
- Adult MeSH
- Antibodies, Monoclonal, Humanized adverse effects MeSH
- Hydrocortisone deficiency MeSH
- Pituitary Gland diagnostic imaging MeSH
- Hypopituitarism chemically induced diagnostic imaging MeSH
- Ipilimumab adverse effects MeSH
- Colitis chemically induced MeSH
- Middle Aged MeSH
- Humans MeSH
- Lymphadenitis chemically induced MeSH
- Autoimmune Hypophysitis chemically induced diagnostic imaging MeSH
- Magnetic Resonance Imaging MeSH
- Melanoma drug therapy pathology MeSH
- Mesentery MeSH
- Skin Neoplasms drug therapy pathology MeSH
- Pancreatitis chemically induced MeSH
- Pneumonia chemically induced diagnostic imaging MeSH
- Tomography, X-Ray Computed MeSH
- Antineoplastic Agents, Immunological adverse effects MeSH
- Retrospective Studies MeSH
- Aged MeSH
- Neoplasm Staging MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Names of Substances
- Antibodies, Monoclonal, Humanized MeSH
- Hydrocortisone MeSH
- Ipilimumab MeSH
- pembrolizumab MeSH Browser
- Antineoplastic Agents, Immunological MeSH
INTRODUCTION: Low anterior resection with total mesorectal excision (TME) is the gold standard for surgical treatment of rectal carcinoma. The radicality of this procedure is negatively counterbalanced by morbidity, lethality, and numerous other complications. Local excision would appear to be an attractive alternative, but its radicality is disputable due to risk of undetected metastasis to the mesorectum. The study aimed to determine the location of mesorectal metastases with respect to circumferentially - located tumors in patients with tumors involving less than one-third of the rectal circumference. MATERIALS AND METHODS: Resected specimens from patients with tumors smaller than one-third of the circumference were divided into: Sector A - tumorous, and Sector B - nontumorous. Group A was created by the pathologist cutting part of the rectal wall with the adjacent mesorectum, as though imitating a full-thickness excision. RESULTS: The study comprised 35 patients with a mean age of 66 years, of which 23 were men (65.7%) and 12 were women (34.2%). Tumors were predominantly (y)pT1-T2; a total of 799 lymph nodes and 5 tumor satellites were examined. Six patients (17.1%) were identified as stage (y)pN+. A total of 3 positive findings (lymph node metastasis or satellites) were detected in 3 patients (8.5%) in tumorous Sector A; and 8 positive findings were detected in 4 patients (11.4%) in non-tumorous Sector B. CONCLUSION: Rectal carcinoma involving one-third of the rectal circumference metastasizes discontinuously, and spreads into parts of the mesorectum beyond the tumor area.
- Keywords
- Mesorectal metastasis, Rectal cancer, Total mesorectal excision,
- MeSH
- Adult MeSH
- Ultrasonography, Interventional MeSH
- Colectomy methods MeSH
- Colonoscopy MeSH
- Laparoscopy MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Mesocolon MeSH
- Morbidity trends MeSH
- Rectal Neoplasms diagnostic imaging mortality surgery MeSH
- Follow-Up Studies MeSH
- Peritoneal Neoplasms diagnosis secondary surgery MeSH
- Prospective Studies MeSH
- Rectum diagnostic imaging surgery MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Neoplasm Staging * MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Czech Republic epidemiology MeSH
INTRODUCTION: Complete mesocolic excision (CME) ensures the removal of all sentinel and regional lymph nodes during colon cancer surgery. For right-sided hemicolectomy it is essential to dissect the wall of vena mesenterica superior, which increases the risk of injuring surrounding organs. So far, no randomized studies comparing long-term oncological results of standard right hemicolectomy and hemicolectomy with CME have been published. METHOD: 83 patients operated for colon carcinoma in 2014 and 2015 were included in this study, all of them undergoing right-sided hemicolectomy using laparotomy access. The standard procedure was done in 63 cases and hemicolectomy with CME was done in 20 cases. We compared the incidence of complications, and the characteristics and descriptions of obtained specimens evaluated by a pathologist for both groups. RESULTS: The operation times of right-sided hemicolectomies with CME was longer by 20 minutes on average. The incidence of postoperative complications was similar in both groups. Pancreatic fistula in the group of patients undergoing right-sided hemicolectomy with CME was an unusual complication; the fistula was healed through conservative treatment. Comparing the resecate parameters, we found no significant differences in the lengths of the resected terminal ileum. But the length of the resected colon was significantly longer for the CME technique (median 42 cm versus 22 cm). The incidence of lymph node metastases was similar in both groups. However, the total number of removed lymph nodes in the group with CME was significantly higher (median 23). CONCLUSION: Right-sided hemicolectomy with complete mesocolic excision offers the removal of more lymph nodes than the standard technique. The central vascular ligation technique elongates the operation time and may be associated with more intraoperative injuries. Introduction of the concept of complete mesocolic excision is derived from an effort to standardize the surgical technique for colon cancer resection.Key words: right-sided hemicolectomy complete mesocolic excision colon cancer lymphadenectomy.
- MeSH
- Operative Time MeSH
- Incidence MeSH
- Carcinoma pathology surgery MeSH
- Colectomy methods MeSH
- Humans MeSH
- Ligation MeSH
- Lymph Node Excision methods MeSH
- Lymphatic Metastasis MeSH
- Lymph Nodes pathology surgery MeSH
- Mesocolon surgery MeSH
- Colonic Neoplasms pathology surgery MeSH
- Pancreatic Fistula epidemiology MeSH
- Postoperative Complications epidemiology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
INTRODUCTION: The aim of the study was to evaluate the results of fluorescence angiography for assessing anastomotic perfusion after resection of the sigmoid colon and rectum since its introduction into clinical practice at the authors´ workplace and to evaluate the incidence of necessary resection line repositioning based on the quality of perfusion, and also to record any complications in anastomotic healing. METHOD: Retrospective unicentric analysis of prospectively collected data from patients with resection of the sigmoid colon and rectum with primary anastomosis. The patient set included 50 patients, 27 males and 23 females; the median age was 64.5 years (33-80). Forty-four patients were indicated for resection for cancer of the sigmoid colon or rectum, while 6 patients had a benign disease. Twenty-nine patients underwent total mesorectal excision with coloanal mechanical or hand-sewn anastomosis and 21 underwent resection of the sigmoid colon or upper rectum with mechanical anastomosis. Prior to the construction of the anastomosis, assessment of perfusion of the anastomotic segments by near infrared (NIR) indocyanine green (ICG) fluorescence angiography was performed in all patients. The quality of perfusion of the mesocolon and bowel wall and its impact on moving the resection line and complications of anastomotic healing 30 days postoperatively were all evaluated. RESULTS: Assessment of perfusion using fluorescence angiography was technically successfully performed in all 50 patients. In 5 cases (10%) the resection line had to be moved for signs of poor perfusion of the bowel wall. Postoperatively, healing of the anastomosis was complicated in four patients (8%). Dehiscence was recorded in 3 patients (10.3%) with total mesorectal excision and in 1 patient (4.8%) after resection of the sigmoid colon and upper rectum. CONCLUSION: The presented results indicate that fluorescence angiography may lead to a decrease in the incidence of anastomotic dehiscence after colorectal resections by mapping in detail the perfusion of the anastomosed segments.Key words: fluorescence angiography - indocyanine green - anastomotic leak - colorectal resection.
- MeSH
- Anastomosis, Surgical methods MeSH
- Digestive System Surgical Procedures MeSH
- Colon, Sigmoid blood supply surgery MeSH
- Surgical Wound Dehiscence epidemiology MeSH
- Adult MeSH
- Fluorescein Angiography MeSH
- Colectomy methods MeSH
- Colorectal Neoplasms surgery MeSH
- Middle Aged MeSH
- Humans MeSH
- Mesocolon blood supply MeSH
- Anastomotic Leak epidemiology MeSH
- Perfusion Imaging MeSH
- Rectum blood supply surgery MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
