Peritoneal metastases from gastrointestinal cancer can be treated by a combination of surgery to remove visible evidence of disease and perioperative intraperitoneal chemotherapy to eradicate microscopic disease. Complete cytoreduction is necessary for long-term benefit.Two sites for incomplete cytoreduction because tumor is hidden from visual inspection were identified. Patients who developed recurrence at these anatomic sites were studied.A peritoneal tunnel along a patent processus vaginalis allowed tumor to be sequestered along the inguinal canal in a female patient. A peritoneal tunnel beneath a hepatic bridge over the round ligament obscured mucinous tumor in a male patient.Peritoneal tunnels in the inguinal region or at the porta hepatis should be opened by surgical dissection so that all foci of peritoneal metastases are removed at a complete cytoreductive surgery and these sites treated with chemotherapy. The forThere are some structures within the abdomen and pelvis that may be at particularly high risk for persistence and then progression of peritoneal metastases. These sites of recurrence may be observed in patients with low grade mucinous appendiceal neoplasms because the bulk of disease is consistently eradicated by cytoreductive surgery and perioperative chemotherapy . This alwww.researchregistry.com website with UIN 4783.Data on these 2 patients was prospectively recorded and then retrospectively reviewed at an academic institution. This research work has been reported in line with the PROCESS criteria . This st2In September 2013, a 59 year old woman consulted her family physician with abdominal distention increasing over 2 months. Ultrasound of the abdomen showed bilateral ovarian tumors and copious ascites. In October, an exploratory laparotomy was performed by a gynecologic oncologist who performed a hysterectomy and bilateral oophorectomy, omentectomy, appendectomy, and tumor debulking. All specimens showed well-differentiated mucinous adenocarcinoma with the primary site within the appendix. The patient recovered well following surgery and underwent follow-up using CT.In 2015, CT showed progression of disease and the patient was evaluated by a peritoneal surface oncology team. CEA, normal postoperatively in 2013, had increased to 11. CT showed mucinous tumor widely distributed on parietal peritoneal surfaces especially beneath the right hemidiaphragm and in the pelvis. A cytoreductive surgery lasting 7 h was performed. The procedure required right upper quadrant peritonectomy, greater and lesser omentectomy, cholecystectomy (for cholelithiasis), and pelvic peritonectomy. Hyperthermic intraperitoneal chemotherapy (HIPEC) and early postoperative intraperitoneal chemotherapy (EPIC) were used ,5. All sIn 2019, CT of abdomen and pelvis showed a 4 cm cystic collection in the right inguinal canal . Also, a3A 47 year old male noted abdominal distention. A CT and ultrasound of the abdomen led to a diagnosis of pseudomyxoma peritonei. In 2012, the patient underwent a debulking procedure which included peritonectomy, splenectomy, greater omentectomy and appendectomy. HIPEC with cisplatin was performed. Pathology showed well-differentiated peritoneal mucinous adenocarcinoma (PMCA) . The patIn 2016, CT showed progression of disease. In 2018, CT showed multiple sites of disease progression beneath right and left hemidiaphragm, lesser curvature of stomach and porta hepatis, within the full extent of the prior abdominal incision, and several masses associated with small bowel mesentery. There was a tumor mass filling the rectovesical space. The tumors within the porta hepatis were located along the round ligament . SegmentThe patient underwent a 13 -h reoperative cytoreductive surgery that included extensive resection of the abdominal wall along the old abdominal incision, bilateral peritonectomy of the right and left hemidiaphragm, hemigastrectomy with gastrojejunostomy and pelvic peritonectomy.To resect disease in the porta hepatis, a cholecystectomy was first performed. Then with traction on the left lateral segment of the liver tumor along the fissure defined by the ductus venosus was visualized and resected. There was a tunnel formed by the hepatic bridge. It was type 3 with segment 3 of the liver totally fused over both the tumor and round ligament to liver segment 4b . Using e44.1The inguinal canal is an abnormal persistence of the processus vaginalis, a peritoneal tunnel that terminates in the scrotal sac in the male or near the labia majora in the female. It contains the spermatic cord in the male and the round ligament in the female . If the The inguinal canal may remain at least partially patent in 20% of adolescent or adult males. It is patent in only a few females. The open peritoneal tunnel in males accounts for the 25% incidence of hernia as the first sign of pseudomyxoma peritonei in males. It leads to a diagnosis of pseudomyxoma peritonei in only 4% of women . If mucoThe clinical course of patient 1 indicates that a peritonectomy of the pelvis must include an extraction of peritoneum from the inguinal canal. This removal of a potentially patent inguinal canal is more important in the male patient than female patient but can be accomplished in both without damage to cord structures or ilioinguinal nerve. Sugarbaker reported on 17 of 178 patients having new onset or previously repaired inguinal hernias in patients having cytoreductive surgery with HIPEC for pseudomyxoma peritonei. All peritoneal and/or scar from prior hernia repair was resected from the inguinal canal. No repair of the open inguinal canal, which was washed by the chemotherapy solution was attempted. Surprisingly, no recurrent inguinal hernias occurred. Apparently extraction of tumor, peritoneum and scar tissue from the inguinal canal facilitates fibrous closure of the hernia defect so that hernia recurrence was not observed .4.2The first mention of a surgically important tunnel at the hilum of the liver was published in 2009 . SugarbaA definite description of this anatomic structure, the hepatic bridge, was provided in 2018. In 102 patients undergoing cytoreductive surgery, 50 had a bridge of liver parenchyma over the round ligament between liver segments 3 and 4b creating a peritoneal tunnel. In 52 patients the round ligament could be completely visualized from its origin in the falciform ligament to its entrance into the liver parenchyma. In 44% of patients with a hepatic bridge up to 1/3 of the round ligament was within the peritoneal tunnel (Class 1), in 32% 1/3 to 2/3 of the round ligament was obscured (Class 2), and in 24% more than 2/3 of the round ligament was observed by overlying liver parenchyma (Class 3). In our patient number 2, a class 3 hepatic bridge was present. The electrosurgical division of the liver parenchyma was a full 6 cm long and 3 cm deep [In conclusion, two peritoneal tunnels within the abdominal-pelvic space were identified. These cylindrical spaces may be seeded by peritoneal metastases. Unless the surgeon is aware of peritoneal tunnels as a site of treatment failure, these anatomic sites may be recognized in follow-up as sites of recurrent disease. The inguinal canal is a peritoneal tunnel that may develop progressive disease . ExtractFoundation for Applied Research in Gastrointestinal Oncology.Data management and secretarial support provided by Local IRB-approval for this case report was not required:does not meet the DHHS definition of research (45 CFR 46.102(d)(pre-2018)/45 CFR 46.102(l)(1/19/2017)) or the FDA definition of clinical investigation (21 CFR 46.102(c)) and therefore are not subject to IRB review requirements and do not require IRB approval.MedStar Health Institutional Review Board has determined that a case report of less than three (3) patients This case report is of 2 patients.Written and signed consent was obtained from the patients.Paul H. Sugarbaker, MD: study concept or design, data collection, data analysis or interpretation, writing the paper.This study was registered as a case series on the www.researchregistry.com website with UIN 4783.Paul H. Sugarbaker, MD.Not commissioned, externally peer-reviewed.Paul H. Sugarbaker has no conflicts of interest to declare.


Question A 51-year-old Caucasian female with a past history of hypertension and hypothyroidism presented for a screening colonoscopy. Her surgical history was significant for a total abdominal hysterectomy. Abdominal examination was normal and laboratory findings were unremarkable. CT scan of the abdomen was performed which did not show any abnormalities or pathologies upon read. The patient underwent colonoscopy demonstrating diverticulosis and a 6 mm pedunculated polyp in the sigmoid colon. Polypectomy was performed using hot snare. A polypoid lesion was seen at the base of the appendiceal orifice -2.What is the diagnosis?How should it be managed?AnswerThe colonoscopy demonstrated a pedunculated lesion consistent with an invaginated appendix. The lesion was filiform with a normal appearing mucosa on endoscopic imagining with superficial biopsies of the lesion later displaying no dysplastic changes. An invaginated appendix occurs when the appendix pulls itself into the cecum . The pat


Metastatic tumors of the temporal bone are extremely rare. Collet-Sicard syndrome is an uncommon condition characterized by unilateral palsy of the lower four cranial nerves. The clinical features of temporal bone metastasis are nonspecific and mimic infections such as chronic otitis media and mastoiditis.This report describes a rare case of metastatic adenocarcinoma of the temporal bone causing Collet-Sicard syndrome, presenting with hearing loss, headache and ipsilateral cranial nerve palsies. The patient was a 68-year old woman initially diagnosed with extensive mastoiditis and later confirmed as having metastatic adenocarcinoma of the temporal bone, based on histopathologic findings.Clinical presentation of metastatic carcinoma of the temporal bone can be overshadowed by infective or inflammatory conditions. This case report is to emphasize the point that a high index of clinical suspicion is necessary for the early diagnosis of this aggressive disease which carries relatively poor prognosis. This report highlights that it is crucial to suspect malignant neoplasm in patients with hearing loss, headache and cranial nerve palsies. Metastatic tumors of the temporal bone are relatively uncommon and usually originate from the sites with a tendency to metastasize to bone . The mosA 68-year-old woman presented with headache, hearing loss, facial asymmetry and dysphagia for few months. On examination, the patient had right facial palsy . Otoscopic and nasal endoscopic examinations were found to be normal. Examination of the oropharynx and larynx revealed right-sided 9th, 10th and 12th cranial nerve palsies with loss of the gag reflex, paralysis of the soft palate, paralysis of the right hypoglossal palsy with deviation of tongue to the right side and right-sided vocal cord palsy. Neck examination showed drooping of the right shoulder and there were no palpable neck nodes. Pure-tone audiogram demonstrated right-sided mixed hearing loss.  A computed tomography (CT) scan and contrast enhanced magnetic resonance imaging (MRI) showed soft tissue lesion in the mastoid with sclerosis in the basiocciput extending to the clivus and sphenoid; features suggestive of extensive mastoiditis with cholesteatoma –3. The b18F-FDG PET-CT) tracer demonstrated multiple bony involvement, with the skull base including the right petrous bone with intracranial extension and multilevel axial skeleton involvement including the upper cervical spine (C1 to C4), dorsal vertebral (D11)   and lumbal (D11) . UnfortuPrimary tumors of the temporal bone account for about only 1% of all head and neck neoplasms . MetastaCollet-Sicard syndrome is a rare condition, clinically resulting in paralysis of the vocal cords, palate, and trapezius and sternocleidomastoid muscles . This caA literature search reveals the most common clinical symptoms in patients with secondary malignant tumors of the temporal bone to be facial palsy followed by symptoms such as hearing loss and otalgia –8. In co18F-FDG PET-CT. The findings from the 18F-FDG PET-CT study demonstrated extensive lesions with multilevel involvement of the posterior and anterior elements of the spine, the liver and subdiaphragmatic regions. There were no significant changes in the 18F-FDG PET-CT study involving the lungs, bowel, breasts or kidneys to suggest primary involvement of these organs. The patient in this report demonstrated features suggestive of extensive mastoiditis on high resolution CT scan of the temporal bone. Subsequently, her general condition deteriorated with progressive difficulty in swallowing and hoarseness of the voice. Histopathological diagnosis revealed metastatic adenocarcinoma of the mastoid, after which a search for the primary site was performed using Temporal bone imaging is important in the diagnosis of primary as well as secondary neoplasms ,10. Radi18F-FDG PET-CT in the instant detection of distant metastases, with the potential to modify management strategy.Clinical presentation of the metastatic carcinoma of the temporal bone can be overshadowed by infective or inflammatory conditions. This case report emphasizes the point that a high index of clinical suspicion is necessary for the early diagnosis of this aggressive disease which carries a relatively poor prognosis. This case illustrates the usefulness of


Acquired benign bronchoesophageal fistula (BEF) is rare and develops as a complication of other diseases, mostly of inflammatory processes and traumas of the chest. The treatment of choice is a surgical repair, which is considered definitive and leads to successful outcomes. However, incidence of recurrence after the primary repair based on limited data is up to 10% and its treatment is challenging. We report a surgical case of a patient with recurrent acquired benign BEF after primary resection and ensuing successful definitive repair with esophageal bypass surgery after temporary esophageal stenting.A 46-year-old male was referred to our department with a symptomatic left-sided bronchoesophageal fistula as a complication of severe acute necrotizing mediastinitis that originated from odontogenic abscess. Previously, several cervicotomies and bilateral thoracotomy were performed at an external medical facility to manage the acute condition. We performed resection of the fistula through re-thoracotomy. Postprocedural esophagography demonstrated a recurrence of bronchoesophageal communication. Postinflammatory adhesions excluded further repair through thoracotomy, therefore a stent was introduced in the esophagus for 12 weeks. Thereafter, an esophageal bypass surgery using a substernaly interposed gastric conduit was performed and resulted in an excellent long-term outcome.Esophageal bypass surgery using a substernaly interposed gastric conduit may be considered if the standard surgical repair of acquired benign bronchoesophageal fistula is not successful or feasible. Acquired benign bronchoesophageal fistula (BEF) is a rare pathology, which develops as a complication of other diseases, mostly of inflammatory processes and traumas of the chest. Due to the non-specific symptoms of BEF, the diagnosis is often delayed and hence the treatment is inadequate. Despite progress in endoscopic methods, non-operative treatment of benign BEF is generally unsatisfactory. The treatment of choice is a surgical repair, which is considered definitive and leads to successful outcomes , 2. HoweA 46-year old patient with a history of complicated descendent purulent mediastinitis that originated from odontogenic abscess underwent several surgical interventions  at another medical facility. The patient’s condition was complicated by the development of multiple organ dysfunction syndrome, with the necessity to introduce tracheostomy, percutaneus endoscopic gastrostomy (PEG) and amputation of distal phalanges of the right foot due to septic embolization. After the management of the acute condition, further course was complicated by development of fistula between esophagus and left main bronchus approximately 1 cm from tracheal bifurcation presenting with recurring polyresistant bronchopneumonias  support. The fistula was resected through the right re-thoracotomy and both bronchial and esophageal defects were sutured in one (with soft tissue underlay), and two layers, respectively.One week after the surgery, esophagography demonstrated a leak in the esophageal suture Fig. a. Due toWith the SEMS, the patient well tolerated orally administered fluids. He was discharged 9 days after stent introduction in a stable condition with a defined nutritional regimen. The follow-up esophagography showed a satisfactory position of SEMS sufficiently sealing the recurrent BEF Fig. c. The peEsophagography has not revealed extralumination or stagnation of the contrast agent 1 week after surgery Fig. d. SubseqAcquired benign BEF is a rare pathology representing complex clinical problem. Despite the biologically benign nature of the condition, the course of the disease may be fatal. Successful treatment requires a multidisciplinary approach and specific course of action differs case by case. Although several reports of primary endoscopic treatment of BEF were published, first-line treatment remains surgical repair which results in excellent long-term outcomes and should be performed as soon as possible after diagnosis , 4. The For patients in critical condition or patients that are unable to undergo the surgery immediately, it is appropriate to introduce a SEMS into the esophagus to seal the fistula as a bridge to surgical repair. SEMS is otherwise considered to be treatment of choice in malignant fistulas . In our From the perspective of anaesthesiology, a major problem during the surgery is the management of airways and adequate oxygenation and carbon dioxide elimination. Selective ventilation of the non-affected lung is necessary. A legitimate option is to avail ECMO support .The standard and generally the most recognized surgical treatment of an acquired benign BEF is thoracotomy (mostly right-sided) with identification, exposure and resection of the fistula. Then suture of the esophagus in two layers and suture of the bronchus with interposition of pedicled tissue between the sutured esophagus and bronchus is performed . It is gRecurrence of BEF after primary surgical repair is not frequent but challenging and the type of surgical treatment is an intensely debated subject. Ultimately, the condition can be solved with cervical esophagostomy and nutritive gastrostomy. This approach, however, negatively affects the quality of life. To the best of our knowledge, the case presented herein is the first report describing a recurrent acquired benign BEF in an adult that was resolved by esophageal bypass surgery. This procedure is otherwise reserved and well recognized palliative surgical treatment for fistulas of malignant aetiology .Esophageal bypass surgery may be considered if the standard surgical repair of acquired benign BEF is not successful or feasible. In addition to surgical treatment, the case demonstrates an essential significance of the interdisciplinary approach and use of other supportive methods, such as perioperative ECMO or temporary esophageal stenting in the complex treatment of BEF.


Abdomen cocoon is a rare disease in which a thick peritoneal membrane wraps the intestine causing the bowel loops to adhere to each other. It may be either primary(idiopathic) or secondary to other causes like previous abdominal surgery. Most patients present with abdominal pain and intestinal obstruction. The condition is usually diagnosed intraoperatively.A 30-year-old male patient presented with abdominal pain and bilious vomiting. The patient had similar previous attacks. Examination showed distension abdominal distension with central tenderness. Plain abdominal X-ray showed multiple air fluid levels. During surgery most of ileum was enclosed by thin membrane with dilated proximal jejunum. Release of the bowel loops was done. The patient was well after surgery and was discharged with no post-operative complications.A 35-year old male presented with chronic right lower quadrant abdominal pain, the past medical and surgical histories were non-relevant. Abdominal examination showed tenderness on deep palpation at the right iliac fossa, abdominal ultrasound and abdominal X-ray were normal. During diagnostic laparoscopy the terminal ileum was enclosed with a thick whitish membrane with dilated proximal ileum. Release of the adhesions was done. The patient was well in the post-operative period and he was discharged home with no post-operative complications.In both cases the biopsy from the membranes showed features of chronic inflammatory process.Abdomen cocoon is one of the rare causes of small bowel obstruction. The bowel adhesions should be opened and nonviable segments resected. Most patients have good long term outcome. •Abdomen cocoon is a rare disease in which a thick membrane wraps the intestine.•It is either primary(idiopathic) or secondary to previous abdominal surgery.•Most patients present with abdominal pain and intestinal obstruction which is usually diagnosed intraoperatively. In this condition a thick peritoneal membrane wraps the intestine causing the bowel loops to adhere to each other. A variable length of the bowel may be affected .This condition was first described by Foo et al., in 1978, when he presented a series of 6 girls who presented with intestinal obstruction, the cases were encountered over a period of 6 years, all were young females between 13 and 18 years of age and he described the condition to occur within two years of menarche suggesting a possible link. Foo grouped their medical condition under a clinical entity named as “the abdominal cocoon”, later this presentation drew the attention of surgeons from different parts of the world to similar cases and they started to document them and presented many cases and articles, and many possible cause were described .The etiology of this condition is unclear but histopathological examination shows features of chronic inflammation. It may be either primary(idiopathic) or secondary which is seen in association with some conditions such as previous abdominal surgery, complicated abdominal wall hernia, renal and liver transplantation, peritoneo-venous shunt in liver cirrhosis ,3,6,7.Most patients present with abdominal pain and intestinal obstruction which is usually diagnosed intraoperatively. CT-scan or barium study can help in suspecting the condition before surgery ,2,8.The work of this case report has been reported in line with the PROCESS 2018 criteria .2This is a retrospective case series that includes two patients who were presented with chronic undiagnosed abdominal pain and one with intestinal obstruction were included.In one patient open surgery was done and the other one diagnostic laparoscopy were done.The operations were done by two general surgeons who are specialized in the field of general surgery and minimally invasive surgery. The operations were done Duhok Emergency Teaching Hospital and in Azadi Teaching Hospital, which are two academic institutions. The two cases were encountered within a period of 3 years.This case series is registered according to the World Medical Association's Declaration of Helsinki 2013 at the research registry in the 14th of August 2019; the unique identifying number is: researchregistry5 080.33.1A 30-year-old male patient presented to the emergency hospital with colicky central abdominal pain for 12 hours, the pain was associated with 2 attacks of bilious vomiting. The patient has history of similar attacks of milder intensity of pain before 1 year which were managed conservatively. There was not relevant past surgical history and the medical history was negative for comorbid illnesses.The pulse rate was 100 beats/minute, the blood pressure was 130/70 mm Hg and the patient was a febrile. Examination of the abdomen showed symmetrical distension of the abdomen with central tenderness and exaggerated bowel sound.Plain abdominal X-ray in erect position showed multiple air fluid levels in the center of the abdomen. During surgery through right lower para-median incision, most of ileum was found to be enclosed by thin membrane with dilated proximal jejunum. Release of the bowel loops was done by cutting the membrane in between and all the adhesions were opened, the bowel was viable and no resection was required. A piece of the membrane was sent for histopathological study which showed features of chronic inflammatory process with no evidence of malignancy, The patient was well in the post-operative period and he was discharged home with no post-operative complications.3.2A 35-year-old male presented with chronic abdominal pain mainly in the right lower quadrant, the patient had non-relevant past medical and surgical histories. Abdominal examination showed tenderness on deep palpation at the right iliac fossa, abdominal ultrasound and abdominal X-ray were normal.During diagnostic laparoscopy the terminal ileum was found to be enclosed by a whitish thick membrane with dilated proximal segment of the ileum. Release of the adhesions were done and biopsy was taken from the membranes which showed features of chronic inflammatory process with no evidence of malignancy. The patient was well in the post-operative period and he was discharged home with no post-operative complications.4Abdomen cocoon should be differentiated from peritoneal encapsulation which was first described in 1868 because the etiology and the managements are different. In peritoneal encapsulation the bowel lies behind a secondary but normal peritoneal layer which is attached to the ascending, descending, and transverse colon. This bowel enters this sac from two sites; at the duodeno-jejunal and the ileocecal junctions. This condition is probably embryogenic in origin and the sac derived from the yolk sac, it may be associated with some other mesenteric abnormalities and most patients have no or mild symptoms in the form of abdominal pain and most cases discovered at autopsy. In the contrary cases of abdomen cocoon are symptomatic most of them requires surgical intervention due to intestinal obstruction, and its etiology remains unclear .Tuberculous infection of the peritoneal cavity has been shown to cause a similar clinical picture, but the adhesions and the membrane are thicker and denser, although the manifestations of intra-peritoneal tuberculosis may be seen in some cases like mesenteric abscess formation, mesenteric lymph nodes enlargement and caseation, for this reason some authors advocate that this may be caused by tuberculous peritoneal infection ,10,11.Yip and Lee mentioned four clinical findings which may help in predicting the diagnosis before surgery, these findings are: occurrence of this presentation in young women, history of some similar attacks of the same pain which resolved spontaneously, repeated episodes of vomiting, and the presence of palpable soft and mobile abdominal pain, but these features are not specific and may present in a wide range of intra-abdominal pathologies .CT scan of the abdomen have been shown to be the most valuable radiological investigation, it shows dilated small bowel loops in the center of the abdomen, it also identify the fibrous membrane surrounding the bowel loops forming a sac like structure, there may be little amount of fluid collection in-between the bowel loops, CT scan also exclude other causes of intestinal obstruction .The normal peritoneal layer is composed of a single mesothelial cells that is supported by a basement membrane, while the histopathological examination of the membrane covering the bowel in patients with abdomen cocoon shows proliferation of fibrous and connective tissues, inflammatory cells infiltration, with no evidence of foreign body granuloma or giant cell formation .In most cases the treatment is surgical and is discovered during surgery, a high index of suspicion in needed to diagnose the condition before surgery which is not the usual situation. The surgery involves removing the membrane and releasing the adhesions, sometimes resection of part of the bowel will be done if the viability in questionable, the condition has a very low risk of recurrence if no coexisting condition is present .In the future larger number of patients must be collected and their data analyzed regarding the possible causes, the clinical presentation, the complications, and the long term follow up. This may involve collecting data from many different parts of the world or performing a large meta analyses study.No ethical committee approval was needed; consent have been taken from the patients to report their findings.No source of funding other than the authors.Study design, data collection, and data analysis: Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif.Writing the manuscript: Dr Ayad Ahmad Mohammed.Final approval of the manuscript: Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif.5080 Research registry.https://www.researchregistry.com.N/A.Dr Ayad Ahmad Mohammed.An informed consent is taken from the patients to report the case and the accompanying images.Not commissioned, externally peer reviewed.No conflicts of interest present.


Chondrosarcomas are the second most common primary malignant sarcoma of the bone, though it is overall a rare tumor. Our case presents a 36-year-old Caucasian male who complained of an enlarging mass at his third finger MCP joint. After assessing the full clinical scenario, it was determined that wide excision with ray resection would provide the best result for this patient.  This study explains a rare malignancy presenting in the proximal phalanx of the hand. The following report will review chondrosarcomas involving the hands, help differentiate between low-grade chondrosarcomas and enchondromas, and briefly cover treatment modalities. Chondrosarcomas are malignant tumors made up of cartilage-producing cells. Of all the malignant bone tumors, these make up around 20% . The peaAbout 70-75% of these tumors arise in the pelvis, femur, and humerus, and albeit very rare, it has been found that chondrosarcomas are actually the most common primary malignant tumor in the digits . In a stThe patient was informed that information from the case would be submitted for publication. The patient understood and consented to the use of all data, photographs, and other health information.Our case describes a 36-year-old Caucasian male with no significant medical history who complained of a painful mass over the dorsum of his third digit metacarpophalangeal (MCP) joint and long finger. According to the patient, it had been present since childhood but was recently becoming a limiting factor in his work and activities of daily living. The patient had been unable to fully flex the affected digit for years but recently began to lose mobility of neighboring digits of the hand. Radiographs obtained demonstrated a lytic lesion within the head of the proximal phalanx of the third digit with surrounding periosteal reaction . MRI was47This case report aims at summarizing the intricacies of diagnosis and treatment options with low-grade cartilage lesions. Chondrosarcomas can be classified as either primary or secondary based upon a preexisting lesion. In secondary chondrosarcomas, there are preexisting benign cartilage tumors that have transformed into malignant lesions. According to a review article by Lin et al., the authors were able to find that the majority (about 88%) of cases stemmed from osteochondromas, with solitary more common than hereditary multiple exostosis (HME). Additionally, there were documented cases of secondary chondrosarcomas from patients with Ollier disease, Maffucci syndrome, and more rarely from solitary enchondroma, synovial chondromatosis, and chondromyxoid fibroma . UnfortuThe differentiation between an enchondroma and a low-grade chondrosarcoma has become one of the more challenging scenarios in the field of orthopedic oncology. These cases must involve a multidisciplinary approach between orthopedics, pathology, and oncology because of their similar clinical and histologic presentation. Importance of this distinction is related to determining the proper treatment course. Enchondromas are benign lesions while chondrosarcomas have a risk of metastasis depending solely on the grade of the lesion . In a stTypically, cartilage lesions in the small bones of the hands and feet are found to be enchondromas, so any concern for a chondrosarcoma should be evaluated further. The chance of metastasis with chondrosarcomas involving the axial skeleton is much greater when compared to the appendages . In a stTreatment options for low-grade chondrosarcomas have been a recent topic for debate in the literature. The two treatment options that have been discussed are wide resection and intralesional curettage. Some authors/clinicians argue that wide excision is the only reasonable definitive treatment because of the metastatic potential of these tumors. Others comment that because of the benign likelihood of these lesions, intralesional curettage is the most appropriate option related to the reduced degree of morbidity and loss of function. In a systematic review done by Hickey et al., the authors were able to compare 190 patients from five separate studies, in which 112 patients underwent wide excision and 78 were treated with intralesional curettage. In this study, the risk of recurrence between the two groups was not significant [In a recent study done by González del Pino et al., sixteen patients were evaluated for functional differences after either wide excision or intralesional curettage. Their data showed that of the nine patients (6 with primary lesions and 3 with recurrent disease) treated with curettage and bone grafting, two had recurrence. Of the eight patients treated with wide excision, one patient had a recurrence (patient was being treated for recurrent disease). In terms of functional status, there was no significant difference between groups when comparing grip strength, pinch strength, DASH  scores, and cosmesis. Limitations of the aforementioned study included a small sample size and lack of preoperative functional data. This indicates that there is a need for future studies on the functional outcomes for these treatments.In our specific case, the patient had started to lose function with the adjacent digits and was no longer able to work. In addition to his functional loss, the patient's lesion was intra-articular, which made intralesional curettage a poor option. It was decided that ray resection would provide the patient with the best functional status postoperatively, while also reducing the chance of recurrent disease. As concluded by González del Pino et al., there are instances where intralesional curettage may be appropriate, but they believe that wide excision with disarticulation of the distal phalanges or digit ablation still plays a role in local control of the tumor while preserving function. In patients with obvious involvement of tendons, neurovascular compromise, or substantial deformity of neighboring joints, they call for the employment of amputation to preserve function . We hope


Objectives. To present a case series outlining the efficacy and safety of the thoracoabdominal incision in complex oncologic procedures in urology. Methods. Four cases utilizing the thoracoabdominal incision, performed by a single surgeon at our tertiary care center, were reviewed. Case history, preoperative imaging, intraoperative experience, postoperative course, final pathology, and complications were examined. A thorough literature review was performed and comparison made with historical cohorts for estimated blood loss, length of stay, and complications encountered versus other common surgical approaches. The indications, advantages, and disadvantages of the thoracoabdominal approach were outlined. Results. All patients had large retroperitoneal masses of varying complexity, requiring maximal surgical exposure. Surgery was straightforward in all cases, without any significant perioperative or postoperative complications. Postoperative pain, length of hospital stay, estimated blood loss, and analgesia requirements were all similar to open and mini-flank approaches in review of historical case series cohorts. Laparoscopic approaches had lower estimated blood loss and length of stay. Conclusions. The thoracoabdominal approach is rarely utilized in urological surgery, due to the perceived morbidity in violating the thoracic cavity. These cases outline the benefit of the thoracoabdominal approach in select cases requiring maximal surgical exposure, and the generally benign postoperative course that appropriately selected patients may hope to endure. Postoperative pain, length of hospital stay, estimated blood loss, and analgesia requirements can be expected to be similar open and mini-flank approaches. As expected, laparoscopic approaches had lower estimated blood loss and length of stay.The thoracoabdominal incision was first described in 1946 as an approach to concomitant abdominal, retroperitoneal, and thoracic injuries. In urology, this technique was popularized in 1949 for the resection of large renal tumours. Today, it is reserved for complex cases where optimal exposure of the renal hilum and adrenal and superior pole of the kidney is necessary. We present four consecutive cases in which this approach was taken by a single surgeon at our tertiary surgical centre. The outcomes, postoperative course, and pathology are described. We provide a comprehensive literature review and outline the indications, advantages, and disadvantages of this approach. A 66-year-old Aboriginal male presented to his family physician with a 2-month history of early satiety, nausea, and abdominal distension. An abdominal CT scan revealed a 20 cm Bosniak IV left renal mass. This occupied much of the left hemiabdomen and displaced the great vessels laterally. No evidence of metastatic disease was found on further workup .The patient underwent a radical left nephrectomy. A thoracoabdominal approach was selected due to size and superior polar location of the renal mass. No intraoperative complications were encountered, and the procedure was well tolerated. A 28 Fr chest tube was placed prior to the closure of the thoracic cavity and was connected to low suction. A nasogastric tube (NGT) was placed in anticipation of a postoperative ileus. Intraoperative estimated blood loss (EBL) was 400cc.The patient's NGT was clamped on postoperative day 2 and removed on postoperative day 3. The epidural was discontinued on postoperative day 2, and the patient was weaned off intravenous analgesia on postoperative day 4. The following day, on postoperative day 5, the chest tube was removed. The patient was subsequently discharged on postoperative day 6 without incident for a total length of stay (LOS) of 6 days.Final pathological analysis confirmed a type 1 papillary renal cell carcinoma. Surgical margins were negative with no evidence of lymphovascular invasion (LVI), corresponding to pathological stage T2bNxMx. Tumour grade was recorded as Fuhrman nuclear grade 2/4.A 50-year-old Caucasian male with a history of hypertension and benign prostatic hypertrophy was found to have microscopic hematuria on his annual urinalysis. An abdominal MRI found an incidental 12 cm left adrenal mass involving the superior pole of the left kidney, and possibly the splenic hilum and distal pancreas. Imaging findings were concerning for a locally invasive adrenocortical carcinoma .There was no evidence of lymphadenopathy or distant metastases on further workup. The patient had serum DHEAS, 17-ketosteroid, and cortisol functionality tests drawn, which were negative. Urine metanephrines were also negative, confirming a nonfunctional adrenal mass.The patient subsequently underwent left nephroadrenalectomy. A thoracoabdominal approach was favoured due to the size, location, and locally invasive appearance of the mass. Intraoperatively, the spleen and pancreas were found to be uninvolved and did not require resection. No complications were encountered and EBL was 150cc. A 28 Fr chest tube was placed prior to the closure of the thoracic cavity and connected to low suction.The chest tube was removed on postoperative day 3, and a follow-up radiograph confirmed the absence of a pneumothorax. The patient experienced modest difficulty weaning the epidural, which was discontinued on postoperative day 5. He was discharged on postoperative day 6 when pain was well managed with oral analgesia.On pathological analysis, microscopic inspection revealed extensive fibrosis, hyalinization, focal dystrophic calcification, and ossification. Immunohistochemical studies  did not show evidence of neoplastic changes. Final pathological diagnosis confirmed an adrenal pseudocyst. No further follow-up was necessary.A 67-year-old Aboriginal female with a history of hypertension and diabetes presented to her family physician with a 3-month history of 20 pound weight loss, early satiety, and fatigue. A CT scan of her abdomen revealed a 14 cm mass in the superior pole of the left kidney with suspected splenic hilar invasion. There was evidence of an enhancing soft tissue mass in the tail of the pancreas, suspicious for metastasis. Further metastatic workup revealed a small burden of pulmonary disease .After a thorough discussion with medical oncology and a full assessment of her functional status, the patient was enrolled in a tumour vaccine trial, which required cytoreductive nephrectomy. With the assistance of the general surgery team, she underwent a left radical nephrectomy, splenectomy, distal pancreatectomy, and retroperitoneal lymph node dissection (RPLND). A 28 Fr chest tube was placed prior to the closure of the thoracic cavity and connected to low suction. Due to the size and location of the tumour, and the suspected local invasion, a thoracoabdominal approach was pursued. No complications were encountered intraoperatively and EBL was 400cc.The patient's postoperative course was uneventful. The epidural and chest tube were discontinued on postoperative day 4. She was weaned off intravenous analgesia by postoperative day 6 and was discharged on postoperative day 8 when fully mobile.Final pathological analysis confirmed a clear cell renal cell carcinoma. Surgical margins were negative with no evidence of LVI. As suspected, a metastatic lesion in the distal pancreas was confirmed. Two lymph nodes were included in the analysis, both of which were negative for malignancy. Final pathological stage was defined as T3aN0M1. The tumour grade was recorded as Fuhrman nuclear grade 3/4.A 61-year-old Caucasian male had previously seen a urologist for recurrent low-grade bladder cancer, which required multiple resections. Unfortunately, he was lost to follow-up and presented to his family physician several years later with abdominal discomfort and weight loss. An abdominal CT scan was ordered, which found a 10 cm cystic mass in the superior pole of the left kidney, concerning for malignancy with suspected splenic hilar invasion. A full metastatic workup was undertaken. No evidence of metastatic disease was identified .The patient underwent a radical left nephrectomy, splenectomy, distal pancreatectomy, completion nephroureterectomy, and RPLND. In anticipation of a difficult resection, the thoracoabdominal approach was selected to maximize surgical exposure. Intraoperatively, the tumour was found to involve the distal pancreas, which was resected with assistance from the general surgery team. During the kidney dissection, an incidental left upper ureteric mass was identified. Given the patient's history of recurrent bladder cancer, urothelial malignancy was suspected, and a completion nephroureterectomy was performed. A 28 Fr chest tube was placed prior to the closure of the thoracic cavity and connected to low suction. No complications were encountered during the procedure and EBL was 4000cc. Three units of packed red blood cells and 1 L of fresh frozen plasma were administered intraoperatively.The patient's postoperative course was slow, but uneventful. The epidural and chest tube were discontinued on postoperative day 5, and he was discharged on postoperative day 9, once deemed physically fit for independent living by physiotherapy and occupational therapy.Final pathological analysis confirmed high-grade transitional cell carcinoma (TCC) with extensive tumour necrosis. Tumour was found to be invading peripelvic fat, renal parenchyma, perinephric fat, and the tail of the pancreas. The resection margins, including the pancreatic margin and the bladder cuff resection margin, were involved by TCC. Two lymph nodes were included in the specimen, which were negative for malignancy. Final pathological stage was defined as T4N0M1. The patient was referred to medical oncology for consideration of systemic therapy.The thoracoabdominal approach involves incision of the eighth, ninth, or tenth rib, with transpleural, transdiaphragmatic, and transabdominal exposure of retroperitoneal structures and the pleural and peritoneal cavities . This meDue to the violation of the thoracic cavity, some surgeons perceive the added morbidity of this approach as a deterrent to its use. However, there are certain advantages that this approach offers over a standard flank or transabdominal or lumbar incision. The incomparable exposure of the kidney and adrenal and renal hilum allows for early primary ligation of the renal vasculature before tumour manipulation. It also allows for easier resection of Gerota's fascia, the ipsilateral adrenal gland, and surgical extirpation of the lymphatic field . ViolatiA commonly described disadvantage of this approach is the incision of multiple muscle layers when accessing the abdominal and thoracic cavities . InherenA recent study by Yang et al.  compared not placing a chest tube in patients unless there was evidence of a parietal pleural injury. In this study, patients without chest tubes had a significantly shorter postoperative recovery course, despite an increased incidence of asymptomatic pneumothoraces and pleural effusions.Kumar et al.  directlyth rib approach to traditional open and laparoscopic approaches. Average EBL was 103 ± 88cc for the mini-flank, 226 ± 264cc for open, and 119 ± 150cc for laparoscopic approaches. DiBlasio et al. [th rib incision for open partial and radical nephrectomy. Average EBL was 400cc. Stifleman et al. [Our four cases presented had EBL values of 400, 150, 400, and 4000cc, respectively, for a mean of 1237 ± 1598cc . The larth rib incision. Stifleman et al. [Our four cases had LOS values of 6, 6, 8, and 9 days, for a mean of 7.2 ± 1.3 days . In consn et al.  had an ath approach, 13% for the open, and 21% for laparoscopic, with all complications being Clavien I-III. DiBlasio et al. [The four cases presented here had no complications, early or late . Yang eto et al.  had no io et al.  had a too et al.  had a coThese case reports represent clinical scenarios where the thoracoabdominal surgical approach was indicated. All patients had large retroperitoneal masses of varying complexity, requiring maximal surgical exposure. The thoracoabdominal approach is rarely utilized in urological surgery, due to the perceived morbidity in violating the thoracic cavity. However, comparison with several retrospective series examining open and mini-flank approaches suggests no difference between thoracoabdominal and the open flank approaches in terms of postoperative pain, length of hospital stay, EBL, analgesia requirements, return to work, and complication rates. As expected, laparoscopic approaches had lower EBL and LOS. Although a small series, our cases outline the benefit of the thoracoabdominal approach in select cases, and the generally benign postoperative course that appropriately selected patients may hope to endure. In the era of robotic assisted and minimally invasive surgery, urologists should be reminded of this effective and safe approach to address challenging retroperitoneal masses.


Non-infectious soft tissue lesions of the foot and ankle are relatively rare clinically. These include benign and malignant neoplasms, as well as non-neoplastic or pseudotumoral lesions such as ganglionic, synovial and epidermoid cysts, intermetatarsal and adventitious bursitis, inflammatory lesions like gouty tophi and rheumatoid nodules, Morton’s neuroma, and granuloma annulare.A 48-year-old male with a history of medically treated tophaceous gout presented with left foot neuropathic pain and paresthesia, in the setting of a well-circumscribed soft tissue lesion of the second intermetatarsal space, suspected to be a Morton’s neuroma. Magnetic resonance imaging (MRI) showed a 4.1 x 2.7 x 2.6 cm heterogeneous soft tissue mass containing multiple cystic areas. Excisional biopsy was performed and histologic examination revealed well-circumscribed nodules of amorphous material containing needle-shaped clefts, rimmed by histiocytes, and multinucleated giant cells consistent with a gouty tophus.This is the first case reported in the literature of an intermetatarsal gouty tophus causing neuropathic pain and paresthesia. While Morton’s neuroma is the most common cause of this presentation, this case illustrates that other pseudotumoral lesions, such as a gouty tophus, may present similarly, and should be considered in the differential diagnosis. While most cases of tophaceous gout can be adequately treated with urate-lowering therapy, surgery may be indicated for tophi that do not resolve with medical treatment based upon symptom severity, compression of nearby structures, and functional impairment. Non-infectious soft tissue lesions of the foot and ankle are relatively rare clinically. These include benign and malignant neoplasms as well as non-neoplastic or pseudotumoural lesions. The pseudotumoural lesions of the foot include ganglionic, synovial and epidermoid cysts, intermetatarsal and adventitious bursitis, inflammatory lesions such as gouty tophi and rheumatoid nodules, Morton’s neuroma, and granuloma annulare.Gout is a disease characterized by high blood uric acid levels and monosodium urate crystal deposits in joints and extra-articular tissues . This chClinical history, laboratory examination, and radiographic findings can help lead the clinician to the correct diagnosis of tophaceous gout. However, a gouty tophus may be mistaken for another pseudotumoral lesion or an infectious process. Gouty lesions most often occur in the foot at the metatarsophalangeal (MTP) or interphalangeal joints . HoweverA 48-year-old male presented with left foot pain which started “a couple of years ago” with no injury. The patient first noticed a “knot” on his foot between the second and third toes approximately one year ago. The patient was referred to the orthopaedics foot and ankle clinic for further evaluation. The patient reported his pain as neuropathic and paresthetic, and radiating to the second interdigital space. His symptoms were worsened by walking, standing, and climbing ladders and stairs. The patient had a known history of gout and was currently being treated with allopurinol and nonsteroidal anti-inflammatory drugs (NSAIDs). Despite treatment with urate-lowering medication, the patient exhibited tophaceous lesions of various sizes on his left elbow, right knee, right foot and bilateral hands, all asymptomatic.Upon evaluation, the patient was afebrile; the other vital signs were within normal limits. On physical examination, the patient had pain with palpation of the interspace between the second and third metatarsal heads, with no metatarsal-phalangeal instability or hyperkeratosis. The rest of the physical examination was unremarkable.X-rays and magnetic resonance imaging (MRI) were ordered to supplement the physical examination. The anterior-posterior and lateral foot X-rays showed small periarticular erosions in the second metatarsophalangeal (MTP) joint, consistent with crystal-induced arthropathy, with no significant degenerative change, fracture, or dislocation Figure . The MRIThe patient was consented for an excisional biopsy of the lesion and decompression of the second intermetatarsal space. The procedure started with a dorsal incision over the second intermetatarsal space. The skin was sharply dissected and the subcutaneous tissue bluntly dissected. Keeping the neurovascular structures protected, the deep tissue was completely exposed. The pseudotumoral lesion was visualized as granular, opaque and whitish, located within the second intermetatarsal space, with no involvement of the adjacent soft tissue. The lesion was excised and removed completely, measured as 2.5 x 1.6 x 1.0 cm, and sent to pathology.Next, attention was made toward decompression of the intermetatarsal space. The intermetatarsal ligament was identified and resected while protecting the nerve underneath. The patient tolerated the procedure well without complications, and was discharged the same day with postoperative pain management medication. Postoperative instructions were to partially bear weight with a wedged shoe for two weeks, then progressively increase weight bearing as tolerated, and return to normal activities and normal shoe wear after six weeks. The patient's postoperative follow-ups were in two weeks for wound check and suture removal in six weeks and three months.The surgical pathology report confirmed the lesion was a gouty tophus. The histologic findings are described in Figure Gout is a metabolic disease, characterized by the deposition of monosodium urate crystals within joints and soft tissues. Typically, patients with this condition have a serum uric acid concentration greater than 7 mg/dL, due to an increase in production or a decrease in excretion of uric acid . Gout isThe cardinal sign of advanced gout is the appearance of gouty tophi. Tophi consist of a mass of monosodium urate crystals, surrounded by cells that typically characterize an inflammatory response, including immature fibroblasts, lymphocytes, plasma cells, macrophages, and foreign body giant cells . They arBased on our literature review, there have been no reported cases of a gouty tophus located in the intermetatarsal space, or causing intermetatarsal pain or neuritis, as in our patient. These findings are typically seen in Morton’s neuroma, a characteristic lesion of the second intermetatarsal space. For this reason, Morton’s neuroma seemed the most likely diagnosis based on the patient’s history and physical exam, despite his known diagnosis of tophaceous gout. However, other differential diagnoses included a gouty tophus, a ganglionic, synovial or epidermoid cyst, intermetatarsal bursitis, granuloma annulare, infection, or a neoplasm. Thus, we ordered imaging with X-ray and MRI to further characterize the lesion. When the imaging results suggested crystal-induced arthropathy, a gouty tophus seemed the most likely diagnosis.Most cases of tophaceous gout can be adequately treated with urate-lowering therapy. However, some tophi do not resolve with medical treatment, and surgery may be indicated based on symptom severity, compression of nearby structures, and functional impairment . In our This case illustrates that while Morton’s neuroma is the most common cause of a second intermetatarsal space lesion causing neuropathic pain and paresthesia, other pseudotumoral lesions such as a gouty tophus, may present similarly, and should be considered in the differential diagnosis. Thus, in the case of a space-occupying lesion, a thorough history, physical exam, and imaging should be performed, and a broad differential diagnosis should be considered. While gouty tophi can usually be resolved with medical treatment, persistent symptomatic tophi may require surgery.While gout most commonly affects the joints, it can potentially involve any area of the body. Thus, the diagnosis of a gouty tophus should be considered for a space-occupying lesion in a patient with risk factors for gout, or a known history of this disease. This is the first case reported of a gouty tophus of the second intermetatarsal interspace, presenting similar to a Morton’s neuroma. MRI helped to narrow the differential, and excisional biopsy with histologic examination definitively diagnosed the gouty tophus. As in our patient, some gouty tophi do not resolve with urate-lowering medication and may necessitate surgery.


We subjected 31 tumors to clinical, imaging, morphological and molecular analyses, including DNA methylation profiling. We identified only one tumor belonging to the MC-AAP (3%), the others exhibiting a methylation profile typical for PA (77%), IDH-wild-type glioblastoma (7%), and diffuse leptomeningeal glioneuronal tumor (3%), while three cases (10%) did not match to a known DNA methylation class. No significant outcome differences were observed between PAAF with necrosis versus no necrosis (p = 0.07), or with 4–6 mitoses versus 7 or more mitoses (p = 0.857). Our findings argue that the diagnostic histomolecular criteria established for anaplasia in adult PA are not of diagnostic or prognostic value in a pediatric setting. Further extensive and comprehensive integrative studies are necessary to accurately define this exceptional entity in children.Pilocytic astrocytoma (PA) is the most common pediatric glioma, arising from a single driver MAPK pathway alteration. Classified as a grade I tumor according to the 2016 WHO classification, prognosis is excellent with a 10-year survival rate > 95% after surgery. However, rare cases present with anaplastic features, including an unexpected high mitotic/proliferative index, thus posing a diagnostic and therapeutic challenge. Based on small histomolecular series and case reports, such tumors arising at the time of diagnosis or recurrence have been designated by many names including pilocytic astrocytoma with anaplastic features (PAAF). Recent DNA methylation-profiling studies performed mainly on adult cases have revealed that PAAF exhibit a specific methylation signature, thus constituting a distinct methylation class from typical PA [methylation class anaplastic astrocytoma with piloid features—(MC-AAP)]. However, the diagnostic and prognostic significance of MC-AAP remains to be determined in children. We performed an integrative work on the largest pediatric cohort of PAAF, defined according to strict criteria: morphology compatible with the diagnosis of PA, with or without necrosis, ≥ 4 mitoses for 2.3 mmThe online version of this article (10.1007/s00401-019-02088-8) contains supplementary material, which is available to authorized users. BRAF gene with KIAA1549 in 66% of cases, resulting from a tandem duplication at chromosome 7p34  and a subtle loss of chromosome 10q, where FISH for PTEN revealed a monosomy . The MIB1 labeling index was estimated at 40%  and microvascular proliferation, but no necrosis. The tumor harbored a double FGFR1 mutation (N546S and K656E) detected by sequencing.The tumor from patient 26 received the DNA methylation-based diagnosis of GBM, 2, and harbored an NTRK2 rearrangement by FISH [ZytoLight SPEC NTRK2 dual color break apart probe ]. Interestingly, 1p FISH was difficult to interpret when first performed. This tumor was classified by DNA methylation as DLGNT , with a subtle loss of chromosome 1 on CNP. Therefore, 1p FISH was redone on multiple tumor fragments and, after reanalysis, was interpreted as a partial deletion due to probable intra-tumoral heterogeneity . Reexamination of the initial imaging found no leptomeningeal dissemination of the tumor (no MRI follow-up available). Based on the spinal location, NTRK2 rearrangement, and heterogeneous 1p loss in tumor cells, we retained the diagnosis of DLGNT.The tumor from patient 30 received the DNA methylation-based diagnosis of DLGNT. Patient 30 was a 7-year-old girl who presented with headaches and neck pain. On MRI, she had a large purely intramedullary spinal lesion ranging from the medulla oblongata to T7, with strong contrast enhancement and necrotic parts. This oligo-like tumor presented with microvascular proliferation, without necrosis, and 6 mitoses for 2.3 mmBRAF showed a typical rearrangement. The rearrangement pattern evoked a duplication as frequently seen in the case of a KIAA1549:BRAF fusion. A sensitive secondary technique (ddPCR) confirmed the KIAA1549:BRAF fusion in all three cases. Therefore, the diagnosis of classical PA was retained.In all “no match cases”, FISH for σ = 26.1 months). Eighteen (58%) patients had tumor recurrence, with a mean PFS of 16.6 months . Four patients died of their disease, and one during surgery, with a mean OS of 41.5 months . Multivariate analyses using the Cox proportional hazards model did not find any statistically significant predictors of OS .In a univariate analysis, using the Log-rank test, no clinical or histopathological features were significantly associated with a worse OS , midline location compared to posterior fossa (p = 0.0293), and partial tumor resection (p = 0.0357). Outcome analyses dependent on histopathological criteria were carried out. No significant outcome differences between cases of PA with anaplasia with necrosis versus no necrosis (p = 0.07), or with 4–6 mitoses versus 7 or more mitoses were evidenced (p = 0.857). For cases with available ASL values (n = 17), no significant association was found between ASL hyper-perfusion and PFS (p = 0.197).Univariate PFS analyses using the log-rank test put forward the following clinical features as significantly associated with a shorter PFS: age at diagnosis ≤ 6 years (p < 0.001) and partial surgical resection  as independent risk factors which significantly impacted progression free survival  7.08 [95% CI 2.03–31.05], www.ebi.ac.uk/arrayexpress) under accession number E-MTAB-8390.An excel sheet detailing all the data from this publication can be found in supplementary Table 1, online resource. Methylation array data have been deposited in the ArrayExpress database at EMBL-EBI  and/or clustered with one of the three conventional PA methylation groups. t-SNE analysis reinforced that a majority of these tumors clustered with classical PA. These findings led us to consider the diagnosis of PAAF based on histological criteria in children with caution since these criteria can also be observed in bona fide PA.We described the largest series of pediatric PAAF cases selected on the basis of the 2016 update of the WHO classification. Anaplasia in PA is defined by detailed criteria: ≥ 4 mitoses for 10 HPFs, with or without necrosis , 19, 26.KIAA1549:BRAF alteration (n = 3). One harbored a double MAPK and H3K27M mutation, whose biological significance in this setting is still uncertain, but which must clearly be distinguished from both single MAPK altered tumors and single H3K27M mutated tumors [CDKN2A/B deletion, and only one also harbored a loss of ATRX protein expression. These findings have also been mentioned by Mistry et al. as alterations defining a subgroup of low-grade glioma with a tendency towards progression, including a single case with PA morphology[CDKN2A deletion, but was nonetheless classified as PA by DNA methylation profiling. Thus, the loss of ATRX and/or CDKN2A deletion, considered as hallmarks of MC-AAP in adults, does not seem to be strong indicators of MC-AAP in children. Overall, of the 7 pediatric cases (including the one from our cohort) with a diagnosis of MC-AAP confirmed by DNA methylation profiling, none presented with the most common anomalies described by Reinhardt et al.: NF1 mutation, CDKN2A deletion, and ATRX loss. Therefore, we consider that the diagnostic criteria established for adult MC-AAP are not accurately applicable to the pediatric population. This reinforces the importance of comparing molecular anomalies of pediatric tumors between themselves and separately from adult tumors.When focusing on the rare pediatric cases of PAAF , and it featured necrosis and microvascular proliferation. The CNP also revealed an amplification of MDM2 gene, confirmed by immunohistochemistry, as previously described in GBM [IDH-WT in our series had 16 and 32 mitoses for 2.3mm2 and microvascular proliferation, with or without necrosis. The diagnosis of supratentorial pediatric GBM can in itself be complex if IDH1/2 and histone gene mutations are not found [In our case of MC-AAP, the main differential diagnosis radiologically was ependymoma, d in GBM . The 2 Got found .IDH-WT all harbored FGFR1 alterations. Alterations of FGFR1 have been described in the form of somatic mutations, gene fusions, and intragenic tyrosine kinase domain (TKD) duplications both in pediatric low-grade gliomas and glioblastomas [FGFR1 mutations have been described in PA and GBM alike, and were found in our cohort in both a classical PA (N546K) (patient 25) and the spinal GBM, IDH-WT (N546S and K656E) (patient 26) [FGFR1 K687E mutation. The other GBM, IDH-WT (patient 28), harbored both an FGFR1 TKD-duplication and an E454K PIK3CA mutation. Activation of the PIK3CA pathway is considered an important genetic event in the development of GBM and can be associated with MAPK pathway activation[The distinction is further complicated by the fact that the MC-AAP and GBMs, lastomas , 25, 32.ient 26) , 25. OurFGFR1 are not a diagnostic or grading argument in pediatric glioma, as they have been described in benign glioneuronal , glial (PA) and malignant neoplasms alike  [FGFR1 alterations. We suggest that in cases of pediatric tumors with piloid features and > 10 mitoses for 10 HPFs the diagnoses of GBM and MC-AAP can be considered, but should be confirmed by DNA methylation profiling.This reinforces the notion that isolated anomalies of -mutant) , 10. PedCDKN2A deletion and ATRX loss) described by Reinhardt et al. have not been found in this cohort, one can conclude that the MC-AAP is rare in children, justifying further cooperative studies to define this entity in the pediatric population through the integration of multiple layers of information.Therefore, based on our cohort and on the few published pediatric cases, the histological and molecular criteria that define anaplasia in adult PA cannot be applied for diagnosis in children. In this setting specifically, these tumors correspond either to PA or various other diagnoses including DLGNT and GBM. As the specific molecular alterations Supplementary material 2. (TIFF 12 mb)Below is the link to the electronic supplementary material.


Intramuscular hemangiomas can be an infrequent but important cause of musculoskeletal pain. This report describes a 25-year-old male who presented with pain in the right forearm and contracture of the right hand for 4 years. Physical examination revealed severe tenderness of the midforearm with contracture of the flexor tendons in the index, middle, ring, and little fingers. Conventional radiography of the forearm revealed a soft tissue phlebolith. Magnetic resonance imaging showed a well-defined lobulated mass partially involving the FDP tendon. An intramuscular hemangioma within the deep anterior compartment of the forearm was suspected. Following surgical excision of the hemangioma, the patient's symptoms resolved completely. In conclusion, intramuscular hemangioma can be a rare cause of flexion contracture of the hands and should be considered as a cause of a flexion contracture that fails to respond to conservative treatment. Hemangiomas are abnormal proliferations of blood vessels, making up 7% of all benign soft tissue tumors , 2. IntrA 25-year-old man presented with a painful flexion contracture of his right hand for four years. 10 years earlier, he had a motorcycle accident, injuring the right forearm. Following the injury, he had pain in his right forearm for several days, which improved with nonsteroidal anti-inflammatory drugs (NSAID). He was asymptomatic and had no movement deficits after the medication. Over the next few years, he gradually developed stiffness in his right index, middle, ring, and little fingers with pain in his right forearm and increasing difficulty in using the affected hand. He was unable to extend his metacarpophalangeal (MCP) joints, proximal interphalangeal (PIP) joints, and the distal interphalangeal (DIP) joints of the index, middle, ring, and little fingers. There were no constitutional symptoms like fever or weight loss.On local examination, his right midforearm was tender, and no mass or swelling could be palpated. His right index, middle, ring, and little fingers were held in flexion, and passive extension of the PIP and DIP joints was grossly limited . The paiA clinical diagnosis of myositis flexion contracture was made. Conventional radiography revealed a soft tissue mass with calcific spots. Magnetic resonance imaging showed a well-defined lobulated mass with a bright signal intensity (SI) on T2W. Internal septations and dark internal SI spots, consistent with calcifications and/or hemorrhage, were present midway on the lateral aspect of the flexor digitorum profundus (FDP) tendon and partially involved the FDP muscle and extended to the intermuscular plane between the FDP and flexor pollicis longus (FPL). It was fed by an artery from the anterior interosseous artery, but it was not dilated .The patient underwent an excision of the mass. Surgery revealed a multiloculated red-yellowish mass, measuring 2 × 2 cm, which involved the FDP, but there was no neuromuscular involvement . After eHemangiomas are one of the most common benign soft tissue tumors , 15. LesPlain radiography, ultrasound, and MRI may be helpful in the evaluation of intramuscular hemangiomas. The plain radiography may show the abnormal soft tissue shadows or associated bony erosion when lesions increased in size . The rouMultiple treatment options are available for symptomatic intramuscular hemangiomas. Some of the intramuscular hemangiomas may involute with time but do not undergo spontaneous regression , 21. SurFew of the literatures have reported intramuscular hemangioma with contracture in the upper extremity. Kamath et al.  reportedIn our case, it took four years to establish the correct diagnosis of the intramuscular hemangioma. Physical examination revealed pain with stiffness of the index, middle, ring, and little fingers but no palpable mass. Such vague symptoms and signs can lead to a misdiagnosis of trigger finger and inappropriate treatments by many health care providers.However, upon correct diagnosis by MRI and successful excision, the patient's symptoms and signs resolved fully, and he had normal hand function.In conclusion, this case demonstrates that when a patient presents with flexion contracture of the forearm with or without pain and fails to respond to conservative treatment, intramuscular hemangioma or other soft tissue tumors should be included in the differential diagnosis. Appropriate imaging is essential to establish the diagnosis.


Several reported complications associated with radiofrequency ablation for liver tumors are due to thermal damage of neighboring organs. We herein report a first case of esophageal perforation due to thermal injury of laparoscopic radiofrequency ablation (RFA).A 75-year-old woman was treated repeatedly with RFA (percutaneous and laparoscopic) and transcatheter arterial chemoembolization for hepatocellular carcinoma. One week after laparoscopic RFA for recurrent HCC located in segment 2 of the liver, dysphagia and thoracic pain occurred. Upper gastrointestinal endoscopy revealed a perforated esophageal ulcer at the esophago-gastric junction. Inflammation was localized because of severe intra-abdominal adhesion due to repeat surgery, so we decided to treat the patient conservatively. The perforation of the esophagus gradually scarred, and exacerbation did not occur after restarting oral intake.When patients with a history of abdominal surgery or intra-abdominal inflammation undergo thermal ablation therapy, caution is required, as there is a possibility of thermal injury of unexpected organs. The incidence of hepatocellular carcinoma (HCC) is increasing worldwide, making HCC one of the most common malignant tumors , 2. RadiA 75-year-old woman was admitted to our hospital for the treatment of recurrent HCC located in segment 2 of the liver (S2). The patient had been diagnosed with hepatitis B virus-related liver cirrhosis 17 years earlier and with HCC (S2 and S7) 8 years earlier. Since then, RFA (percutaneous [four times] and laparoscopic [two times]) and transcatheter arterial chemoembolization  had been performed repeatedly for HCC. During laparoscopic RFA for recurrent S2 HCC, the left lateral lobe of the liver was mobilized. For recurrent HCC located in S2 of the liver, TACE was repeatedly performed; however, the therapeutic response was insufficient, and the patient was referred for further treatment.3/μL; serum aspartate aminotransferase, 39 U/L; alanine aminotransferase, 15 U/L; total bilirubin, 1.2 mg/dL; albumin, 3.6 g/dL; and prothrombin time, 13.6 s. The indocyanine green retention rate at 15 min (ICG-R15) was 30.7%, and the ratio of the 99mTc-galactosyl human serum albumin (GSA) scintigraphy taken up by the liver to that taken up by the liver plus heart at 15 min (LHL 15) was 0.86. The Child-Pugh score was Grade A, and liver damage, as defined by the Liver Cancer Study Group of Japan [Abdominal enhanced computed tomography (CT) showed a 1.5-cm mass in the left lateral lobe of the liver (S2) with arterial phase enhancement followed by washout in the portal phase Fig. , b. A laGiven that the liver function was severely impaired and the patient had already undergone RFA and TACE several times, we decided to perform RFA, not liver resection, for the treatment of this TACE-refractory small HCC. Because the tumor was adjacent to the heart Fig. c, percutAt 1 week after the treatment, the patient complained of dysphagia and thoracic pain. Upper gastrointestinal endoscopy revealed a perforated esophageal ulcer at the esophago-gastric junction, and the liver could be directly seen through the perforated ulcer Fig. . ContrasCurrent treatments for HCC include hepatic resection, liver transplantation, and thermal ablation such as RFA, microwave coagulation, cryotherapy, TACE, and sorafenib . Among tThe management of recurrent HCC requires a multidisciplinary approach. Although repeated hepatectomy is the mainstay treatment for recurrent HCC, only a minority (30–35%) of patients have resectable disease because of a limited liver function reserve due to previous surgery, underlying chronic liver disease and multifocality of tumor recurrence , 9. The A laparoscopic approach to thermal ablation has been reported to be safe and effective for small HCC , 13. ThiIn the present case, the left lateral lobe of the liver adhered severely to the diaphragm, stomach, and lesser omentum due to the previous treatment. We peeled away the adhesion, but sufficient mobilization was not obtained because of the severe adhesion and the existence of the left inferior phrenic vein in the adhered diaphragm. Because we were focused on avoiding burn injury to the heart and stomach, we failed to pay appropriate attention to the esophagus. During the procedure, although we always paid attention to the position of the needle and other organs, including the diaphragm, heart, and stomach by ultrasonography, we did not recognize that the esophagus was located close to the ablated area. We should have paid closer attention to neighboring organs, especially those that are not usually considered in such cases, such as the esophagus. Patients with a history of abdominal surgery or intra-abdominal inflammation should be regarded as being at high risk of thermal injury of unexpected organs.Perforation of the digestive tract does not always require surgical intervention. In the present case, CT findings and laboratory data suggested that inflammation was localized due to severe intra-abdominal adhesion. Therefore, we decided to treat the patient conservatively. However, of note, if conservative management is selected, careful observation is required, and surgical intervention should be performed if signs of spreading of inflammation become apparent.We describe a case of esophageal perforation due to thermal injury of laparoscopic RFA. When patients with a history of abdominal surgery or intra-abdominal inflammation undergo thermal ablation therapy, caution is required, as there is a possibility of thermal injury of unexpected organs.


Diagnostic work-ups in transplanted immunosuppressed patients are a challenge as non-specific findings may be interpreted as transplant-related complications. If the disease in question is rare and slowly developing like pseudomyxoma peritonei (PMP), it is even more difficult. Cytoreductive surgery (CRS) and subsequent hyperthermic intraperitoneal chemotherapy (HIPEC) is the recommended treatment for PMP even with extensive peritoneal spread. CRS-HIPEC for PMP after liver transplantation (LTX) has not been described before.A 48-year-old female patient with end-stage primary sclerosing cholangitis (PSC) underwent orthotopic LTX and subsequent pancreaticoduodenectomy after the finding of cholangiocarcinoma in situ in the native common bile duct. Ten years after the transplantation, she developed symptoms and signs suspected to represent graft-related complications. An extensive work-up revealed PMP. Upon reassessment, a cystic mass near the coecum could be seen on computed tomography scan 1 year after transplantation. The multidisiplinary team was hesitant to accept the patient for CRS-HIPEC because of extensive PMP and possible risk to the graft. However, she was eventually accepted and underwent the procedure. The Peritoneal Cancer Index (PCI) was 28 of 39, and surgical debulking was performed followed by HIPEC. The transplant control 2 months after surgery showed no harm to the graft.Previous LTX should not exclude the possibility for CRS-HIPEC in PMP, even with extensive burden of disease. Primary sclerosing cholangitis (PSC) is an inflammatory disease of the bile ducts leading to concentric fibrosis . The etiPseudomyxoma peritonei (PMP) is a rare, slowly progressive mucinous malignant disease confined to the peritoneal cavity, usually originating from the appendix . The preA 48-year-old female patient underwent her first work-up for possible liver disease 30 years ago. Based on findings of chronic active hepatitis in liver biopsy, autoimmune hepatitis was suspected, and treatment with prednisolone and azathioprine initiated. Seven years later, an endoscopic retrograde cholangiopancreatography (ERCP) showed a stricture in the common bile duct which was interpreted as possible extrahepatic PSC or tentatively an inborn anomaly of the common bile duct. After 20 years with fluctuating transaminases between 100 and 300 U/L and no recorded major clinical events, she developed general edema and thoracic spider nevi. A computed tomography (CT) scan revealed liver cirrhosis and portal hypertension with massive ascites. After an initial response to symptomatic treatment, she soon developed uncompensated liver failure with severe hepatic encephalopathy and was transferred to the national transplant unit for work-up and listing for liver transplantation. A few days later, an ABO-identical donor liver was available and LTX was performed with a duct-to-duct biliary anastomosis. The histopathological examination of the liver explant revealed cirrhosis and pathological changes typical for underlying PSC. Cholangiocarcinoma in situ was found in the common hepatic duct, the cystic duct, and the common bile duct, involving the resection margin. It was decided that the patient should be controlled with ERC with brush cytology from the duct-to-duct anastomosis after 6 months due to the histopathological findings. The brush cytology showed mild to moderate epithelial dysplasia in the common bile duct. After discussion in the multidisciplinary team, a pancreaticoduodenectomy (Whipple procedure) was decided due to the risk of development of cholangiocarcinoma. Ten months after LTX, the procedure was performed. Because of a possible increased risk of anastomotic leak due to immunosuppression, an enteropancreatic anastomosis was avoided and the pancreatic duct was occluded. The surgical specimen showed intestinal metaplasia in the common bile duct, but no dysplasia or carcinoma. She was then followed according to standard post-liver transplantation follow-up regimen. The patient developed insulin-dependent diabetes mellitus and received tuberculostatic treatment due to disseminated tuberculosis , but the graft function remained good. At the 5-year post-transplantation control, recurrent PSC in the graft was diagnosed on liver biopsy. In addition, a 10 × 4 cm multicystic mass in the lower right abdominal quadrant was described on the routine abdominal ultrasound (US) exam. Upon reassessment, a CT scan of the abdomen performed before the Whipple procedure also showed a 10 × 5 cm multicystic mass in the same region. The lesion was asymptomatic, and based on imaging, the lesion was considered to be a benign gynecological cyst. Consequently, no further investigations were performed to establish the exact etiology. At 10 years of follow-up, the patient reported increasing abdominal distension, anorexia, weight loss, and fatigue for the past 6 to 8 months. Clinical examination revealed extensive sarcopenia, multiple spider nevi, and tense ascites. Biochemical tests showed an iron deficiency anemia with Hgb of 9.0 g/dL, moderately elevated liver function tests, inflammatory markers, and carcinoembryonic antigen (CEA). Imaging, including liver and abdominal US, abdominal and thoracic CT, CT enterography, and positron emission tomography (PET)-CT unveiled liver graft fibrosis/cirrhosis with features of portal hypertension, massive low-attenuating heterogeneous ascites, a focal wall thickening in the middle part of the jejunum, multiple focal lesions in both lungs, a focal lesion in the thyroid gland, and the previously described cystic mass in the lower right abdominal quadrant. Diagnostic abdominal paracentesis revealed thick gelatinous ascites, which was culture negative. The ascites was rich in mucin, but without neoplastic cells on repeated cytology examinations. The provisional primary diagnosis of graft fibrosis-related portal hypertension with ascites could not be supported. Further extensive diagnostic work-up did not confirm tentative diagnoses such as gastrointestinal tuberculosis, sarcoidosis, mucinous tumors of the ovaries, or other causes of peritoneal carcinomatosis. Bearing in mind the differential diagnosis of PMP as a rare, but relevant tentative cause of mucinous ascites, re-evaluation of the contrast-enhanced CT scan raised the suspicion of PMP and that the cystic lesion actually represented a mucinous cystadenoma of the appendix  for the patient due to concerns of the magnitude of the disease with the risk of harming the graft during the procedure. However, after a renewed assessment, the patient was accepted for explorative laparotomy and potentially CRS-HIPEC. Perioperatively, the peritoneal cancer index (PCI) was estimated to 28 of 39 and it was found that CRS-HIPEC was technically doable and meaningful. The operation time was approximately 11 h. Surgery included removal of considerable amounts of mucinous masses, extensive peritonectomy including the right hemidiaphragm, part of the left hemidiaphragm and the pelvis, omentectomy, hysterectomy, bilateral salpingo-oophorectomy, ileocaecal resection, splenectomy, and removal of the umbilicus. Minimal mucinous masses in relation to the piggyback anastomosis to the inferior caval vein were left due to safety reasons. HIPEC was then performed with mitomycin C for 90 min. The histopathological examination showed perforated low-grade appendiceal mucinous neoplasm and wide-spread mucin without viable tumor cells in all abdominal specimens, compatible with PMP. Forty-two days after surgery, the patient was readmitted with urinary tract infection, anemia, high glucose levels, reversible kidney failure, weight loss, and malnutrition. She recovered on antibiotics and supportive treatment and received follow-up by nutritionists after discharge. Two months after surgery, she had started regaining weight and transplant control displayed satisfactory graft circulation and function  is a term used in CRS and is assessed at the end of surgery by measuring the diameter of the largest remaining tumor deposits . CompletChronic immunosuppression has been associated with poorer prognosis in CRC patients undergoing surgery, in particular in respect to the risk of developing distant metastasis . There iTo our knowledge, this is the first publication on CRS-HIPEC for PMP in an immunosuppressed liver-transplanted patient. One case report on CRS-HIPEC in a liver-transplanted patient with PM-CRC is previously published and concluded with feasibility of the procedure in this patient category . The pre


Abdomino scrotal hydrocele (ASH) is a condition in which the hydrocele sac is extended beyond the scrotum to the abdomen via the inguinal canal. The treatment is ordinarily surgical. Different approaches have been described like paramedian laparotomy, an inguinal or inguino scrotal approach. We report a case of giant unilateral hydrocele in an 18 year old male, occupying a large part of the abdomen with urinary symptoms. Ultrasonography and CT showed typical cystic mass in hourglass shape that we have approached surgically by scrotal incision and we removed all the cyst. Pathological examination found a hydrocele with no signs of malignancy. Urinary symptoms disappeared postoperatively. This is a rare entity that evolves often painless and little reported in the literature. The etiology and pathogenesis of this disease is discussed. The hydrocele is a collection of fluid between the two layers of the tunica vaginalis that surrounds the testicle. The hydrocele swells the scrotum but the testicle remains normal. Rarely, scrotal hydrocele may extend into the abdominal cavity through the inguinal canal, defining the abdomino scrotal hydrocele (ASH), this condition is originally described by Dupuytren in 1834 [An 18 year old male was referred to our Institution with a history of an asymptomatic palpable mass in the left testis growing into the lower abdomen for 1 year. The patient complained of bladder symptoms. Physical examination revealed a healthy adolescent presented left painless scrotal mass with ipsilateral palpable abdominal mass, there was non-transilluminant. An abdomino scrotal ultrasound performed in another hospital demonstrated left fluid scrotal mass, measuring large diameter 8 centimeters (cm) above a testicle, which measuring large diameter 6 cm with a suprapubic mass hypoechogenic intra-abdominal measuring large diameter 11.5 cm. The bladder was displaced by the mass to the right . The riget al. have described a case of HSA which compresses the right urinary tract, this one has been solved after surgery [et al. described in their series including 29 cases of ASH, twenty out of 29 patients (70%) were initially managed expectantly. Sixteen (80%) had resolution of their abdominal component, twelve (60%) of which went on to have full resolution, so they recommend observation as the first step in the management of uncomplicated ASH [We found more than 100 cases described in the literature. Unilateral ASH was found in 59% of cases with almost even distribution between left and right-sided hydroceles. Ipsilateral undescended testicle, testicular dysmorphism, contralateral pathology  and adjacent organs compression often accompanied ASH . Dharamv surgery . 11-mont surgery . The eti surgery , 5, dive surgery  and incr surgery . However surgery , 6. In o surgery . The dif surgery . SometimASH is a very rare form of hydrocele. The diagnosis is evoked by clinical examination in front of a scrotal hydrocele associated with an ipsilateral abdominal cystic mass. Ultrasound and CT scan show an inguino-scrotal hourglass mass. The abdominal compartment of the ASH can cause loco-regional complications by compression. MRI can be a valuable contribution to surgical curative treatment.The authors declare no conflict of interest.


Emergency surgery is rare in management of breast cancer. We report a case of encapsulated papillary carcinoma (EPC) of the breast where emergency surgery was performed because of unsuccessful control of hemorrhage.A 62-year-old woman visited our hospital complaining of sudden bleeding from the right breast. She had been aware of the tumor for a year, but had left it unattended. It had been increasing in size rapidly for a few days before presentation. Computed tomography showed a hypervascular cystic tumor 9 cm in diameter. Bleeding was observed from a skin breach in the right breast, from which a clot was removed and the wound compressed with gauze. We attempted unsuccessfully to control the hemorrhage over the next 3 days, but the patient became anemic, so emergency surgery was performed to control the bleeding. Histopathology revealed the tumor as estrogen receptor- and progesterone receptor-positive EPC, with a human epidermal growth factor receptor 2 score of 0.in situ lesion, but the lack of a myoepithelial layer at the lesion's periphery may represent a low grade or indolent form of invasive carcinoma and prognosis is usually good. However, if bleeding into the cyst occurs and hemostasis is difficult, emergency surgery should be considered.There have been only 5 reports of breast cancer treated with emergency surgery. EPC was previously considered an Emergency surgery is rare in breast cancer but should always be considered as an option when hemorrhage is not readily controlled. There have been only 5 reports so far . In most2in situ was seen in the surrounding mammary gland tissue. Surgical margins were negative. A fistula was seen with thin and discontinuous surrounding skin; no tumor cells were found  and biochemistry; carcinoembryonic antigen was slightly elevated (9.7 ng/mL). Computed tomography (CT) revealed a clear cystic mass with a diameter of 9 cm on the right breast, with thinning of the overlying skin. Some areas of the tumors were hypervascular . There wre found C and it 3Breast disease is rarely treated with emergency surgery. There are only 10 case reports of breast disease requiring emergency surgery in Japan . We founIn the differential diagnosis, various imaging diagnostics are performed, but the final result is often obtained from histological examination. If the whole lesion is a mass, imaging is useful to distinguish it from a phyllodes tumor. However, it is difficult to distinguish images with cystic lesions as in this case, and imaging tests such as magnetic resonance imaging (MRI) in the presence of persistent bleeding are not practicable.EPC is an entity described in the WHO Classification of Tumours of the Breast (2012) and is considered to be non-invasive carcinoma or a low grade or indolent form of invasive carcinoma. Histologically, EPC is defined as papillary carcinoma with neoplastic epithelial cells exhibiting low core atypia with papillary growth pattern in a cavity surrounded by a macroscopic cystic fibrous capsule .In our case, a sudden increase in the size of the mass was observed and effective hemostasis could not be achieved. This uncontrollable bleeding may have occurred due to a change in the tumor shape and a sudden rapid increase in the cyst size. The progressive bleeding could have led to skin crushing necrosis, resulting in massive hemorrhage.1)The stromal vascular stem ruptured into the cyst and bled, with expansion of the free space in the cyst.2)Enlargement of the free space increased the range of motion of the tumor, which further encouraged stromal vascular stem rupture and bleeding, leading to associated cyst growth.3)With the enlargement of the cyst, skin necrosis occurred with fistula formation, and ensuing bleeding to the exterior.4)via compression.There were likely multiple bleeding points within the cyst and hematoma, and so effective hemostasis could not be achieved We examined these processes pathologically and histologically, and our inferences were as follows:Puncture and drainage of the hematoma in the cyst is also considered an alternative to compression, but it has been reported that the cyst could return to its original size because the puncture would promote rebleeding .For bleeding due to breast cancer, another option is bleeding control by endovascular embolization. Although some success has been reported , identifIn this case, there was no vascular collapse on CT, anemia was absent, and hemorrhage was judged to be slow, so compression hemostasis was attempted repeatedly. However, when anemia was eventually noted, compression hemostasis was judged unlikely to be effective, and we opted for emergency surgery.The purpose of emergency surgery for bleeding is to achieve hemostasis, but mastectomy and tumor resection must also be performed, meaning that surgery can also treat the tumor.For patients, it is natural that an optimal surgery without excess or deficiency is desired, and as much preoperative diagnosis as possible is vital.Diagnosis of breast cancer in daily practice is based on mammography and ultrasonography, and metastasis is detected using contrast-enhanced MRI and positron emission tomography. However, mammography is not feasible with persistent bleeding and a large mass, and even with ultrasonography it would be difficult to visualize the details of an already enlarged tumor in such cases. Therefore, diagnosis with contrast-enhanced CT is the main option.From past case reports, sudden bleeding from the breast is assumed to be mainly phyllodes tumor and breast cancer. Bleeding is presumed to be from rupture due to tumor progression  and ruptTo our knowledge, there are no reports on prognosis in breast cancer patients following emergency surgery due to hemorrhage, and prognosis was therefore determined based on the final pathological diagnosis, with consideration of the postoperative course. In this case the diagnosis was EPC, which usually has a good prognosis.In routine clinical practice, sentinel lymph node biopsy is performed except in cases with obvious metastasis, but this is practically impossible in emergency surgery, and thus was not done in this case. On the other hand, when there is bleeding from the lesion in the lymph node region, axillary lymphadenopathy if present may be due to reactive enlargement. Reassessment of the axillary lymph nodes after surgery may be essential.For distant metastases, preoperative evaluation in cases requiring emergency surgery could be inadequate. In our case, CT was repeated after surgery with careful follow-up using ultrasonography, but no findings suggestive of lymph node metastasis or distant metastasis were seen.Furthermore, there could be tumor cells in the shed blood, and this carries a high risk of contamination during surgery. Therefore, more thorough observation for local recurrence than usual is necessary. If exposure of surrounding tissue to tumor cells is expected, additional radiation therapy may be considered even for cases of total mastectomy.4We have reported a case of EPC that required emergency surgery due to hemorrhage that could not be controlled by compression. Emergency surgery is rare in the management of breast disease, but should always be considered as an option when bleeding cannot be readily controlled. Preoperative evaluation and further postoperative evaluation are vital in performing emergency surgery, particularly in terms of postoperative treatment.Our study has not received any grant of funding.Our institution does not require ethical approval for a case report that are deidentified and collected retrospectively.Written informed consent was obtained from the patient for publication of this case report and accompanying images.Kimiyau Yoneyama contributed to operation and writing the manuscript.Asuka Hara contributed to operation.Motohito Nakagawa reviewed the work.This is a case report, and no database approval was applied.Kimiyasu Yoneyama.Not commissioned, externally peer-reviewed.The authors declare that there is no conflict of interest regarding the publication of this article.