Background: Hepatoid carcinoma (HC) is a rare extrahepatic malignancy that shares many morphological and serological features with hepatocellular carcinoma. HC has been reported to arise from several organs that are derived from the foregut endoderm, including the stomach, gallbladder, and pancreas. We present a case of an elderly man with hepatoid adenocarcinoma of the pancreatic head with duodenal invasion, presenting with pancreatitis and a gastrointestinal bleed. With only 23 reported cases at the time of our literature search, we discuss the presentation, histopathology, and management of such a rare disease.Case presentation: A 71-year-old man presented initially with abdominal pain and was treated conservatively for pancreatitis. Four months later, he presented with melena and anemia. His examination was noncontributory. Esophagogastroduodenoscopy revealed a friable ampulla of Vater, and a CT scan of the abdomen showed a 4.5 cm pancreatic head mass. Fine needle aspirate revealed an epithelioid neoplasm with hepatoid morphology. Serum α-fetoprotein was normal. Surgical resection confirmed hepatoid adenocarcinoma of the pancreas with positive lymphadenopathy and negative margins. There was no radiographical or gross evidence of distant spread. Observation and adjuvant gemcitabine were discussed as possible options. The patient elected to receive care closer to home and will continue surveillance imaging.Conclusion: With only 23 reported cases, pancreatic HC represents a rare entity within gastrointestinal oncology. There is no clear postoperative adjuvant standard therapy for this likely heterogeneous group of tumors. Although surgical resection is the mainstay of upfront treatment, metastatic disease to the lymph nodes or liver portends a poor prognosis and may warrant treatment such as transarterial embolization, chemotherapy, or radiotherapy. The first case of a pancreatic HC was described by Hruban et al.4 The terminology “hepatoid” refers to an ectopic “liver-like” tumor having morphological features similar to those of hepatocellular carcinoma (HCC).1 Although this report focuses on pancreatic HC, variations of this tumor can be located in several organs that share their derivation from the foregut endoderm, including the stomach, gallbladder, and pancreas.2 Given the location, patients typically present with symptoms of epigastric pain or obstruction.Hepatoid carcinoma (HC) of the pancreas is a rare extrahepatic neoplasm.1 Histology often reveals “pure form” morphology, which can exist in association with other morphologies such as neuroendocrine carcinomas. Like HCC, HC has historically been associated with elevated serum α-fetoprotein (AFP) levels.1–3 Immunohistochemistry (IHC) also reveals similarities to HCC with positive cytokeratin (CK), keratin, albumin, α-1-antitrypsin protease inhibitor, and transferrin among others.2Microscopically, pancreatic HC is composed of polygonal sheets of cells with abundant cytoplasm and centrally located nuclei. Occasionally, bile canaliculi and actual bile formation are visualized. Tumor cells are organized into glandular, medullary, or trabecular patterns. Many tumors, however, demonstrate mixed architectural patterns.3Although no specific diagnostic criteria have been developed for the diagnosis of pancreatic HC, morphology and IHC similar to HCC are required. With no distinguishable primary, the CK panel  can be useful at distinguishing primary HCC from pancreatic HC. Normal or neoplastic hepatocytes (in the case of HCC) are typically CK18+, CK19−, and CK 20−. HC differs from CK19 positivity and CK20+/−.We present the case of an elderly man with primary HC of the pancreatic head with duodenal invasion presenting with pancreatitis and a gastrointestinal bleed. We also discuss the presentation, histopathology, and management of such a rare disease.A 71-year-old man presented to an outside hospital with abdominal pain and was treated conservatively for pancreatitis. His comorbidities included hypertension and hyperlipidemia. He had no previous abdominal surgeries and denied a history of liver disease, gallstones, or alcohol consumption. He was discharged there after conservative management and presented to our hospital 4 months later with several days of melena and lightheadedness. His hemoglobin was 9 g/dL from a baseline of 14 g/dL and he had melena on digital rectal examination. His vital signs were stable. His abdomen was nontender with no palpable hepatosplenomegaly or masses.He was initially evaluated with an esophagogastroduodenoscopy (EGD) that revealed a prominent, edematous, and friable ampulla of Vater. There was a small oozing ulcer at the ampulla, but with no active bleeding. Colonoscopy was negative for additional bleeding masses. A CT scan of the abdomen with contrast showed a 4.5 cm enhancing mass along the anterior pancreatic head indenting the second portion of the duodenum.Further contrast MRI scan of the abdomen  revealedPertinent laboratory values include an AFP of 0.6 ng/mL , cancer antigen 19-9 of 13 U/mL (<35 U/mL), and carcinoembryonic antigen of 3.4 ng/mL (<4.7 ng/mL). Chromogranin A was not measured. Liver function tests were within normal limits. A repeat EGD with endoscopic ultrasound and fine needle aspirate revealed discohesive, large pleomorphic cells in monolayers resembling an epithelioid neoplasm with hepatoid morphology. The cells were positive for CK AE1/AE3, hepatocyte-specific antigen, and C-KIT. The cells stained negative for synaptophysin and chromogranin.Pylorus-preserving pancreaticoduodenectomy and cholecystectomy were performed. Exploration grossly revealed a well-circumscribed, elliptical shaped mass sitting in the pancreaticoduodenal groove. The mass involved the pancreatic head that bulged into the duodenum. There was no evidence of carcinomatosis, omental implants, or ascites. Grossly, the liver was free from metastatic disease. After meticulous dissection of the pancreatic head and duodenum from the surrounding vasculature, complete resection and successful reconstruction were achieved.Histopathological examination of the Whipple resection specimen revealed a moderately differentiated pancreatic hepatoid adenocarcinoma with involvement of the duodenal muscularis propria. The tumor was 5 cm in greatest dimension with lymphovascular and perineural invasion present. The patient recovered without complication and was evaluated by both medical oncology and radiation oncology. Options presented included observation and gemcitabine, given the adenocarcinoma component. Adjuvant radiotherapy was deferred given the complete local resection with negative margins. The patient elected to receive care closer to home and will continue serial surveillance imaging.1 The pancreatic body/tail region accounted for 61% of the reported cases and the median tumor size was 6 cm. Of the reported cases, hepatitis B and C were serologically negative in all cases. The median age was 56 years (range 21–80 years) and 70% of patients were male. AFP was elevated in 60% of patients and CA 19-9 and CEA were abnormal in 18% of patients. Pure histology represented 59% of the cases, with the remainder showing mixed histology. The majority of patients (85%) underwent surgical resection upfront and 32% were found to have liver metastases, whereas 21% had lymph node metastases. Overall, 1- and 5-year survival rates were 71% and 40%, respectively (median 13 months).1The largest combined analysis of pancreatic HC cases was reported by Kuo et al. and included 23 cases.1Embryologically, two theories may explain the origin of this rare tumor. The “ectopic liver” theory assumes that since the liver and the pancreas are both derived from the foregut endoderm, there is a possibility of liver rests in the pancreas. Liver-specific carcinogenesis can then activate ectopic tissue neoplastic transformation. Ectopic liver tissue has also been found in the stomach, gallbladder, and thorax, where HC has similarly been described. A contrast to this theory is the “pancreas-to-liver trans-differentiation” theory. Pancreatic stem cells typically act as suppressors to hepatocyte differentiation genes. Following exposure to carcinogens, these genes can undergo activation, as has been shown in animal models. Proponents of this theory cite the pathologically mixed features of many HCs.2 Gemcitabine has shown benefit in pure adenocarcinoma, and these results have been extrapolated to other mixed histologies, including adenosquamous and hepatoid adenocarcinoma. Doxorubicin-based chemotherapy has shown efficacy in unresectable HCC in previous reports, but no improvement in survival.2 Petrelli et al. describe a case of metastatic pancreatic HC treated with sorafenib, resulting in more than 7 months of progression-free survival.3 Studies investigating adjuvant sorafenib in the treatment of HCC have failed to reveal a survival advantage. Transarterial chemoembolization has also been explored for treatment of liver metastases in several cases.2 In the series by Kuo et al., two patients received radiotherapy, but it is unclear at what time interval this therapy was administered and whether these patients were initially unresectable.1Regarding adjuvant chemotherapy, similar to HCC, there is no standard recommendation for systemic therapy. Adjuvant therapy combinations reported in the literature include gemcitabine, cisplatin combined with irinotecan, and 5-fluorouracil with streptozocin alternating with dacarbazine and adriamycin.2 Analysis of the reported cases demonstrates the importance of surgical excision. Patients who underwent surgical resection had significantly improved survival. Furthermore, all patients who did not undergo resection died within 1 year of diagnosis.1Given the rarity of this tumor and the reports described, complete surgical resection remains the treatment of choice for pancreatic HC.This case illustrates an unusual presentation of gastrointestinal bleeding from duodenal invasion of a pancreatic head HC in an elderly male. HC of the pancreas is rare and histopathologically represents a heterogeneous group. Complete surgical resection is paramount and postoperative management is not standardized. Chemotherapy and/or radiation therapy are typically reserved for unresectable, recurrent, or residual disease.


Postoperative intestinal intussusception (POI) is a rare cause of intestinal obstruction with POI after surgical reduction of ileocolic intussusception being an extremely rare variant. POI was reported to follow many abdominal and non-abdominal operations. A late diagnosis can risk ischemia and necrosis. POI also increases the morbidity and mortality, rendering an early diagnosis and prompt management as lifesaving.We reviewed the medical charts retrospectively for the last ten years for patients with POI at Palestine Red Crescent Society Hospital, Hebron, Palestine. We reviewed the literature and presented the characteristics of the most reported cases of POI following surgical reduction of ileocolic intussusception.We presented three cases of ileoileal POI and one case of ileocolic POI followed different primary operations. All but one patient presented in the first two weeks. The delayed presentation came two months after revision of a prolapsed colostomy. All patients managed successfully with operative manual reduction with no postoperative complications. Initially, we had struggles in the diagnosis of POI largely due to a low suspicion for this rare entity, but thereafter we kept POI in mind and managed the after-coming cases in an expeditious manner.Frequently, POI is misdiagnosed as postoperative adhesive obstruction. POI is challenging in diagnosis and needs a very high index of suspicion, mainly due to its rarity and atypical presentation. By keeping the possibility of POI in mind, one can easily diagnose it and prevent its consequences. Less co22.1A two year-old female patient underwent exploratory laparotomy with end sigmoid colostomy after a penetrating rectal injury. The intraoperative and early postoperative periods were uneventful. After ten days following the surgery, the child presented three times with a picture of intestinal obstruction. However, at each time, she was admitted for two days, treated conservatively, and improved. One month following the surgery, she developed diffuse severe colicky abdominal pain with vomiting, diarrhea and abdominal distension. Abdominal standing x-ray showed multiple air fluid levels with dilated loops. She was taken to the operating room and underwent a laparotomy and found an ileoileal intussusception. Manual reduction and resection of the necrotic part were performed. The child’s postoperative course was uneventful.2.2A five-month-old female underwent surgical reduction of idiopathic ileocolic intussusception . Initial2.3A seven month-old male with Hirschsprung’s disease, underwent a loop ileostomy at the age of 12 days due to intestinal perforation. At the age of three months, the infant presented with prolapse of his ileostomy necessitating revision of the ileostomy. Two months following laparotomy, the infant developed a prolonged course of watery diarrhea and malabsorption with poor weight gain. Cow's milk protein allergy was suspected and formula was changed with no improvement. At the age of seven months, the infant underwent pull through procedure. An ileocolic intussusception was identified incidentally and managed by manual reduction. The infant’s stool production and intestinal absorption normalized after the reduction. His postoperative course was uneventful.2.4A six month-old male with Hirschsprung’s disease, underwent laparotomy with leveling colostomy. Three days after the operation, the infant developed abdominal distention with bilious vomiting. Plain abdominal x-ray was performed and showed multiple air fluid levels with dilated bowel loops. The infant failed to improve with conservative therapy. The patient was taken back to the operating room five days after the initial laparotomy and an ileoileal intussusception was identified and reduced manually. The infant progressed well postoperatively and was discharged home at POD5.3Postoperative intestinal obstruction is a common problem encountered in children and is mostly attributed to intestinal adhesions and adynamic ileus ,6. One fThe reported incidence of POI is 0.01–0.25%  and abdoBeing reported after many different primary surgeries, POI can have different underlying mechanisms. Although etiology of POI remains unclear, several theories were proposed to explain its pathophysiology, including early postoperative adhesions, excessive bowel manipulation, altered peristalsis, neurogenic factors, electrolyte disturbances, and medications (anesthetics and opioids) . The mosintussusceptions. Ileoileal POI is frequently reported with abdominal procedures while ileocolic POI being reported more commonly with non-abdominal procedures .The typical presentation of the idiopathic intussusception involves painful abdominal cramps, vomiting, a palpable abdominal mass, and rectal bleeding . LethargUnlike idiopathic intussusception, the POI frequently presents with non-specific “prolonged adynamic ileus’’  symptomsPOI is challenging in diagnosis and needs a high index of suspicion, mainly due to its rarity, atypical presentation, and the abundance of postoperative adynamic ileus ,13,14. FAlthough 90% of POI patients present within the first two weeks following the operation , one of A late diagnosis of POI poses a risk of ischemia and necrosis, and need for subsequent bowel resection. It also increases the morbidity and mortality ,5,17, unPOI is usually managed by operative manual reduction with resection and anastomosis in select cases ,10. Two 4Frequently, POI is misdiagnosed as postoperative adhesive obstruction. POI is a rare cause of intestinal obstruction with POI after surgical reduction of ileocolic intussusception being an extremely rare variant. POI is challenging in diagnosis and needs a very high index of suspicion. Contrast studies and abdominal ultrasonography are diagnostic in the majority of cases.The following authors have no financial disclosures: Sadi A. Abukhalaf, Tareq Z. Alzughayyar, Muath A. Baniowda, Radwan Abukarsh, Ihsan Ghazzawi, Nathan M. Novotny and Ahmad Al Hammouri.No funding or grant support.The study is exempt from ethnical approval in our institution.The patient consents were all obtained by the infants’ parents. And all parents accepted the final edition of the article.Study concept or design: Radwan Abukarsh, Ihsan Ghazzawi.Data collection and data analysis: Sadi A. Abukhalaf, Tareq Z. Alzughayyar, Muath A. Baniowda.Writing the paper: Sadi A. Abukhalaf, Ahmad Al Hammouri, Nathan M. Novotny.http://www.researchregistry.com. with registration number of researchregistry4965 and the primary investigator is Sadi Abukhalaf. Here is the link : https://www.researchregistry.com/register-now#home/registrationdetails/5d0c0452c404ef000afa1e4b/We registered the study at Dr. Sadi A. Abukhalaf.Not commissioned, externally peer-reviewed.


Tractional retinal detachment with or without secondary tear is a rare complication reported in less than 0.5% of in eyes treated for retinoblastoma. Pars plana vitrectomy (PPV) in eyes with history of retinoblastoma has been associated with a significant risk for recurrence, extraocular spread, and systemic metastases. We report here the successful management by PPV under melphalan irrigation of 2 children presenting with tractional retinal detachment after retinoblastoma therapy and scleral buckle surgery.A 7-year-old girl with a history of bilateral retinoblastoma (group D) presented with light perception best-corrected visual acuity (BCVA) and tractional retinal detachment (RD) in her left eye, 3 years after the last intra-arterial chemotherapy (IAC) injection. Moreover, she had history of left eye rhegmatogenous RD treated by scleral buckle 1 month after the last IAC and cataract surgery 12 months later. PPV associated with retinectomy, laser photocoagulation and silicone oil tamponade was performed. Silicone oil was removed 4 months later. Fifteen months after PPV, BCVA had increased to 20/32 without recurrence of RD and no evidence of tumor activity. A 7-year-old boy with a history of unilateral retinoblastoma (group D) in his left eye presented with rhegmatogenous RD 21 months after the last treatment for retinoblastoma. Scleral buckle surgery was performed, but 3 weeks later the patient presented with tractional RD associated with proliferative vitreo-retinopathy. BCVA was counting fingers. PPV associated with membrane peel, laser photocoagulation and silicone oil tamponade was performed. Silicone oil was removed after 5 months followed by cataract surgery 5 months later. Twenty months after PPV, BCVA was 20/20 and there was no sign of tumor recurrence.PPV under melphalan irrigation, with retinectomy, if necessary, and silicone oil tamponade, allows anatomical and functional improvement in eyes with history of retinoblastoma and scleral buckling developing tractional RD. Serous retinal detachment (RD) is a frequent finding in eyes with retinoblastoma at presentation, commonly caused by tumor-related exudation and usually completely resolving after tumor regression. Transient spontaneously resolving RD can also follow or worsen after rapid tumor regression. In some instances, however, eye-preserving retinoblastoma management can be complicated by secondary rhegmatogenous and/or tractional RD. Rhegmatogenous RD has been reported in 1%  to 6% 33, 4 of tIntraocular surgery in the context of retinoblastoma is feared because of the risk of tumor spread. Secondary RD in eyes with retinoblastoma are preferentially managed with an external approach, as scleral buckle surgery –7 or enuWe report here the successful management of tractional retinal detachment with PPV under melphalan irrigation in 2 children treated for retinoblastoma and with previous scleral buckling surgery.A 4-year-old girl with bilateral International Intraocular Retinoblastoma Classification (IIRC)  group D A boy diagnosed with unilateral IIRC group D retinoblastoma at the age of 5 was successfully treated with 3 cycles of intravenous chemotherapy  as well as 8 intravitreal and 6 intracameral melphalan injections. At the age of 7, 21 months after the last tumor treatment, he presented with sudden vision decrease in the affected left eye. BCVA was 20/250. Fundus examination showed a total macula-off retinal detachment due to a large tear next to the tumor scar. Scleral buckling without drainage was performed but the retina failed to reapplicate despite appropriate indentation of the buckle, as proliferative vitreoretinopathy (PVR) developed three weeks later, particularly involving the macula (Fig. We described here 2 unusual cases of tractional retinal detachment after retinoblastoma treatment, and scleral buckle surgery for RRD, that were successfully managed by PPV under melphalan irrigation.Secondary RD occurring during treatment of retinoblastoma is to date a rarely reported complication but this may change in the future as more advanced cases are now tried to be salvaged. Rhegmatogenous RD due to an atrophic hole/or a horsehoe tear have been reported after different treatment modalities including intravenous chemotherapy, external beam radiotherapy or plaque brachytherapy with the role of focal treatments, as laser photocoagulation or cryotherapy, being emphasized , 2, 13. To date there is no consensus on the best technique nor the acceptable disease-free interval to manage secondary RD in cases of retinoblastoma. First concern should be to rule out an active tumor as part of the detachment underlying mechanism or as concomitant feature at time of the complication diagnosis. Whereas scleral buckling is an appropriate option for rhegmatogenous RD , 14 exteRecovery of visual acuity was seen in both patients, even though spectral-domain optical coherence tomography of the macula at the last follow-up showed a slightly atrophic fovea with alterations on photoreceptor and retinal pigment epithelium layers in Case 1.In conclusion, pars plana vitrectomy under melphalan irrigation, with retinectomy, if necessary, and silicone oil tamponade is a reasonable treatment option in eyes with controlled retinoblastoma developing tractional RD after rhegmatogenous RD treated with scleral buckle.


Early recognition of VATS-related complications is crucial for early interventions, treatments and better outcomesPatient presented with post-obstructive pneumonia like symptoms 1 week after VATS pulmonary resection.CT scan chest showed evidence of complete consolidation of the lobe where the pulmonary segmentectomy resection took place.Diagnostic bronchoscopy confirmed the erroneous transection of the Superior Segment (SS) of Right Lower Lobe (RLL). Patient was then taken back for completion lobectomy and found with necrotic SS of RLL. This finding potentially could have caused significant complication if not recognized and treated earlyPatient recovered well after completion lobectomy and was discharged home several days laterErroneous bronchial transection should be suspected early in patients presenting with post-obstructive pneumonia after VATS pulmonary resection. CT scan chest and diagnostic bronchoscopy are the 2 main diagnostic tests This minimally invasive approach is currently employed for diagnostic and therapeutic lung surgery, as well as other procedures involving the mediastinum, chest wall, pericardium, esophagus and diaphragm. In general, VATS procedures are well tolerated and the prevalence of major intraoperative complications is around 1.5%.2We present a 65 year old woman, with a history of prior left upper lobectomy for early stage non-small cell lung cancer, who underwent elective VATS for a highly suspicious right lower lobe lung nodule Fig. . The nod3In this case, diagnostic bronchoscopy led to an early recognition of erroneous bronchial transection which ultimately was treated with completion lobectomy and saved this patient significant morbidity. Had the bronchoscopy not been performed, her clinical and radiographic findings would have been blamed on severe pneumonia and broad-spectrum antibiotics would have been administered hoping for clinical improvement.–5 In all cases, the fissure-last technique has been identified as a potential cause of this complication. In this technique, the surgeon dissects and transects the hilar structure first then proceeds to complete the fissure with a stapling device last.,7 The fissure-last lobectomy requires advanced expertise and recognition of anatomical structures prior to attempting transection of the hilar structures. A ventilation test after clamping the targeted bronchus could also be helpful. The fissure-last technique could be challenging especially in right-lower lobectomy and when a ventilation test is neglected or not performed.,7The erroneous bronchial transection has been reported as a major complication of VATS pulmonary resection ranging from 0.9 to 1.8%.Just like in this case, the evaluation of pneumonia early after VATS lung surgery should include a CT scan of the chest. Careful review of the operative report, identification and visualization of ipsilateral remaining proximal airways, and localization of surgical clips on imaging are crucial in diagnosing an erroneous bronchial transection of remaining neighboring lobes or segments to the resected part of the lung. If the CT scan shows an unexplained complete consolidation of any lobe or segment and bronchus cut-off sign with presence of proximal surgical clips, a diagnostic bronchoscopy is warranted with careful examination of the airways looking for erroneous transection. Discussion with the surgical team and aggressive surgical resection of the affected segment or lobe are the mainstay of treatment approach.In summary, in patients presenting early after VATS pulmonary resection with signs and symptoms of pneumonia, CT scan chest with careful focus on airway patency is an important diagnostic test. Suspicious findings should be futher investigated with a diagnostic bronchoscopy to rule out an erroneous bronchial transection followed with surgical intervention if applicablePatient has provided informed consent for publication of the case and accompanying imagesWriting – original draft: Amit Borah, Steven Cocciardi, Ziad Boujaoude, Wissam Abouzgheib.Writing – review & editing: Amit Borah, Steven Cocciardi, Ziad Boujaoude, Wissam Abouzgheib.


Amyloidosis is a syndrome involving amyloid protein deposition in various organs, resulting in organ dysfunction. Symptoms of gastrointestinal amyloidosis are usually nonspecific, such as diarrhea and body weight loss. We, here, report a patient who presented to the hospital with simultaneous hematemesis, melena, and intestinal pseudo-obstruction, leading to a diagnosis of primary gastrointestinal amyloidosis based on computed tomography (CT) and endoscopic findings. CT showed diffuse wall thickening from the duodenum to the jejunum, jejunal dilation, and fluid accumulation throughout the gastrointestinal tract. Upper gastrointestinal endoscopy revealed duodenal mucosal edema, jejunal dilation, and small hemorrhages from jejunal mucosal erosion. The definite diagnosis was done based on biopsy results. This report describes the early diagnosis of gastrointestinal amyloidosis based on CT and endoscopy findings. Amyloidosis is a syndrome that involves amyloid protein deposition in various organs throughout the body, resulting in organ dysfunction . GastroiHere, we report a patient who presented to the hospital with simultaneous hematemesis, melena, and intestinal pseudo-obstruction, leading to a diagnosis of primary gastrointestinal amyloidosis. This report appropriately describes the novel aspects of the study, including the early diagnosis made by CT and endoscopy findings.The patient was an 87-year-old female with a history of hypertension, type 2 diabetes mellitus , angina pectoris (she had medication such as β blocker and nitrate), and prior surgery for appendicitis. In early June 2016, she presented to our hospital emergency department with the primary complaints of hematemesis after eating, repeated vomiting, and melena. She was alert, and her body temperature was 36.7 °C. The blood pressure was 130/68 mmHg, and the regular heart rate was 76 beats/min. Her palpebral conjunctivae were indicative of anemia; there was tenderness in the epigastrium and peristaltic depression. A blood sample was collected (Table We performed lower gastrointestinal endoscopy 2 days later, but no inflammatory findings were observed and no abnormality was shown in biopsy. In addition, we examined small intestine with long scope and confirmed small ulcer and small bleeding in jejunum Fig. . As a caAfter hospital admission, the treatment included refraining from intake of food orally, gastrointestinal pressure reduction by insertion of a stomach tube, and administration of proton pump inhibitors, resulting in the resolution of the patient’s symptoms. We administrated her medications that improve peristalsis and enteral feeding product. Following admission, she was discharged on day 5.After the discharge, we managed the patient by administering intestinal motility improving medications and oral nutrients, and no recurrence of symptoms was observed.Regarding the informed consent, we got the informed consent prior to inclusion in the study.There were two important clinical findings with respect to the patient reported here. She presented to the hospital with gastrointestinal amyloidosis, with the primary complaints of hematemesis, melena, and intestinal obstruction and the differential diagnosis of intestinal amyloidosis was predicted on the basis of characteristic CT findings.First, the usual symptoms of gastrointestinal amyloidosis are body weight loss, diarrhea, constipation and fatigue . In the Second, on the basis of characteristic CT findings, including diffuse wall thickening from the duodenum to the jejunum, jejunal dilation, and fluid accumulation throughout the gastrointestinal tract, we considered the potential diagnosis of intestinal amyloidosis before endoscopy was performed. Patients with AL amyloidosis, as in the present case, show thickening of the submucosal layer from the muscularis mucosa . In contThe present patient had no significant underlying disease; thus, the diagnosis of primary AL amyloidosis was made. She had no symptoms related with amyloidosis such as neurologic abnormality, joint ache, anasarca, purple spot and enlarged lymph nodes. Electrophoresis, urinalysis, and cranial CT were performed, but findings suggestive of multiple myeloma were not identified, and rapid alleviation of symptoms was achieved with symptomatic treatment. Clinicians should consider the potential diagnosis of intestinal amyloidosis based on the presenting symptoms and characteristic CT findings.


Primary retroperitoneal masses constitute a heterogeneous group of uncommonlesions and represent a challenge due to overlapping imaging findings. Most aremalignant lesions. Although they are more prevalent in adults, they can occur atany age. Such lesions are classified as primary when they do not originate froma specific retroperitoneal organ and are divided, according to the imagefindings, into two major groups: solid and cystic. The clinical findings arenonspecific and vary depending on the location of the lesion in relation toadjacent structures, as well as on its behavior. The main imaging methods usedfor staging and surgical planning, as well as for selecting the biopsy site andguiding the biopsy procedure, are computed tomography and magnetic resonanceimaging. In most cases, the treatment is challenging, because of the size of thelesions, vascular involvement, or involvement of adjacent organs. In thisarticle, we present a review of the retroperitoneal anatomy and a practicalapproach to the main imaging features to be evaluated, with a view to thedifferential diagnosis, which can guide the clinical management. The majority of cases are malignant tumors, ofwhich approximately 75% are mesenchymal in origin(2-4). Although such tumorsare more prevalent in adults, they can occur at any age(2).Retroperitoneal masses constitute a heterogeneous group of lesions, originating inthe retroperitoneal spaces, that pose a diagnostic challenge forradiologists. Solid lesions can be divided into four groups, byorigin(3): mesenchymal, neural, germ-cell, andlymphoproliferative. Among the cystic lesions, the most common are lymphangioma andcystic mesothelioma. There are also non-neoplastic processes, primarilyretroperitoneal fibrosis, non-Langerhans histiocytosis (Erdheim-Chester disease),and extramedullary hematopoiesis.When they do not originate from organs such as the kidneys, adrenal glands, pancreas,or bowel loops, retroperitoneal masses are classified as primary and are categorizedas solid or cystic , dependi(9). The main imagingmethods for the evaluation of these lesions are computed tomography (CT) andmagnetic resonance imaging (MRI), imaging features facilitating the differentialdiagnosis, the tumor staging, and the definition of the surgical strategy, as wellas guiding biopsies. Although there is significant overlapping of theimaging findings and the final diagnosis is defined by histopathological analysis,there are characteristics that are specific to certain lesions and can guideclinical practice. The treatment of retroperitoneal masses is challenging, mainlydue to their anatomical location, dimensions, vascular involvement, or involvementof adjacent organs.The clinical manifestations of retroperitoneal masses are nonspecific, depending ontheir location and relation with the adjacent structuresIn this article, we present a review of the retroperitoneal anatomy. We also take apractical approach to the imaging characteristics to be evaluated in, as well as theclinical management of, the main primary retroperitoneal masses in adults., as depicted in The retroperitoneal space extends from the diaphragm to the pelvis, being delimitedposteriorly by the transverse fascia and anteriorly by the posterior parietalperitoneum, and is divided into compartments(3). Findings of anterior displacement ofabdominal structures, such as the aorta or colon, or retroperitoneal organs, such asthe kidneys, help identify the lesion site. However, there are situations in whichit is difficult to determine the exact location, because of the anatomicaldistortion caused by the lesion. In such cases, retroperitoneal involvement shouldbe detailed by describing the spaces involved.In the initial evaluation of a retroperitoneal mass, its location within theretroperitoneal space should be confirmed and the affected compartment  should be identified(1).The collective evaluation of these findings is aimedat narrowing the possible differential diagnoses and guiding the therapeuticplanning.To categorize a retroperitoneal mass as a primary retroperitoneal lesion, its originfrom a larger retroperitoneal organ should be excluded. It should then be classifiedas solid or cystic, its main imaging characteristics  shouldbe evaluated, and its relationship with adjacent structures should be described.There are radiological signs  that aid in the diagnostic assessment; the absence of those signs canconfirm the categorization of a mass as a primary retroperitoneallesion(17).A finding of intralesional fat significantly shortens the list of differentialdiagnoses, narrowing it down to only lesions with distinct biological behavior,such as liposarcoma, teratoma, and extramedullaryhematopoiesisLiposarcoma - Liposarcoma is the most common retroperitoneal sarcoma, accountingfor approximately 30% of all retroperitoneal sarcomas. It affects individuals inthe fifth and sixth decades of life. It can be classified as welldifferentiated, with or without dedifferentiated, myxoid, round cell, orpleomorphic components, which have distinct clinical and radiologicalcharacteristics. It is often located in the perirenal space.. Among the imaging characteristics that favorthe diagnosis, making a benign lesion less likely, is lesion size greater than10 cm, the presence of thick (> 0.2 cm) septa, and foci of nodularenhancement. However, histopathological analysis with themolecular markers (anti-CDK4 and anti-MDM2 antibodies) facilitates thatdistinction. When technically feasible, the treatment of choice is surgicalresection, with wide negative margins to avoid localrecurrence. Currently, some therapies targeting amplifiedoncogenes have shown promise in the treatment of certain liposarcomas,especially the well-differentiated and dedifferentiatedsubtypes.Well-differentiated liposarcoma, which is the most common subtype of liposarcoma,contains mature adipose tissue and is characterized by infiltration of theadjacent structuresRetroperitoneal teratoma - Retroperitoneal teratoma is a germ-celltumor, derived from the embryonic layers, than can present elevated serum levelsof markers, including alpha-fetoprotein, CEA, CA-19-9, andβ-hCG. It is characterized by macroscopic fat, cysticareas, calcifications, and a fat-fluid level, as well as heterogeneous contrastenhancement, as shown in . In male patients, consideration should begiven to the possibility of secondary retroperitoneal lesion of gonadal originand the testes should always be investigated(5).Extramedullary hematopoiesis - Extramedullary hematopoiesis is acompensatory mechanism related to reduced hematopoiesis in the bone marrow andis characterized by deposits of hematopoietic tissue in organs of mesenchymalorigin (the spleen and liver) and within paravertebraltissues(16). Imaging can reveal multiple, homogeneous,bilateral masses, with minimal contrast enhancement, with or without macroscopicfat(5). Secondary findings of chronic anemia, includingclinical and laboratory data, contribute to the diagnosis.Extra-adrenal myelolipoma - Extra-adrenal myelolipoma is a rare,typically unilateral, benign mesenchymal lesion, containing adipose andhematopoietic tissue, that can occur in the retroperitoneum, most commonly inthe presacral region. It usually affects women between 50 and 70 years of age.Most patients with extra-adrenal myelolipoma are asymptomatic, the lesionusually being identified as an incidental finding on an imaging examination,although some patients present with abdominal pain, due to hemorrhage, tumorinfarction, or extrinsic compression. The main differential diagnoses areretroperitoneal teratoma, extramedullary hematopoiesis, and liposarcoma, thelast being practically indistinguishable from extra-adrenal myelolipoma onimaging studies. Biopsy is often required in order to make that distinction, andthe presence of hematopoietic components confirms the diagnosis of extra-adrenalmyelolipoma. Treatment options range from imaging follow-up-for small,asymptomatic lesions-to surgery-for symptomatic, bulky, or progressively growinglesions, as well as for cases in which the nature of the lesion remainsundetermined.Cystic lymphangioma - On imaging studies, cystic lymphangioma canshow lipid content, due to the presence of chylous ascites(8).Key point: There is a specific group of retroperitoneal lesions thatcontain fat and have distinct biological behavior. The identification of fatcontent during the imaging evaluation reduces the number of possibledifferential diagnoses..Among the solid lesions that can contain calcifications are neurogenic tumors,dedifferentiated liposarcomas, teratoma, and undifferentiated pleomorphicsarcomaDedifferentiated liposarcoma - Dedifferentiated liposarcoma presentscalcifications in approximately 30% of cases; when accompanied by nodules withheterogeneous enhancement . In the investigation of liposarcomas,18F-FDG PET/CT can facilitate the evaluation of the tumor grade,as well as the staging and postoperative follow-up, through quantification ofthe FDG uptake of the solid components of the lesion, especially in thededifferentiated and pleomorphic subtypes. Some authors have suggested thatthe tumor grade correlates with the rate of glycolysis(22).ancement , suchcaRetroperitoneal teratoma - Retroperitoneal teratoma is characterizedby a lesion that is predominantly cystic, containing fat and calcifications, afinding that is suggestive of mature teratoma .teratoma . In contExtragastrointestinal stromal tumor - An extragastrointestinalstromal tumor is a mesenchymal neoplasm originating from the interstitial cellsof Cajal, typically located outside the gastrointestinal tract and rarelyinvolving the retroperitoneum. It is characterized by mutations in the c-kitgene, being histologically similar to a gastrointestinal stromal tumor.Extragastrointestinal stromal tumors manifest as homogeneous or heterogeneousmasses, with central necrosis and calcifications. The main differentialdiagnoses include sarcomas(18).Key point: Calcification is a common finding in most retroperitoneallesions and, when accompanied by other imaging features, such as fat, narrowsthe differential diagnosis. Regarding liposarcoma, the presence ofcalcification, nodules with enhancement and FDG uptake in the 18F-FDGPET/CT study suggest a dedifferentiated tumor component.(1). The major lesions that contain a myxoid matrixare neurogenic tumors, myxoid liposarcoma, andmyxofibrosarcoma. We highlight myxoid liposarcoma and neurogenictumors, which can originate from the neural sheath, the paraganglion system, orthe sympathetic ganglia(3).A myxoid stroma consists of a mucopolysaccharide-rich matrix and is characterizedby a hyperintense signal in T2-weighted MRI sequences and by delayed contrastenhancementNeurogenic tumors - Neurogenic tumors originate from the neuralsheath, encompassing schwannomas and neurofibromas.. On imagingexaminations, schwannomas manifest as circumscribed masses with oval orspherical morphology and heterogeneous enhancement, commonly located in theparavertebral region. On MRI, the signal intensity varies dependingon the predominant tissue type; for example, the characteristic hyperintensesignal in T2-weighted sequences is related to a greater quantity of myxoidstroma. Depending on their location and size,schwannomas can also compress adjacent structures . The treatment ofchoice is surgery-mainly for lesions that are large or symptomatic-ormonitoring-for asymptomatic cases or cases with a high risk of surgicalmorbidity.Schwannomas are the most common benign neural sheath tumors, are common in youngadults, and can be accompanied by neurofibromatosis(10). On MRI, they manifest as masses withsoft-tissue attenuation, producing hypointense signals on T1-weighted images andsignals of variable intensity on T2-weighted images(3). Other features ofneurofibromas include the presence of myxoid degeneration, the target sign, andcontrast enhancement. They usually present spindle cell morphologywith longitudinal orientation in relation to the affected nerve trajectory, orenlargement of the neural foramen when they originate from the spinal nerveroots(3). Retroperitoneal plexiform neurofibromas aretypically bilateral, symmetrical lesions that follow the lumbosacral plexusdistribution, being caused almost exclusively by neurofibromatosis typeI. It is of note that malignant peripheralnerve sheath tumors account for 10% of soft-tissue sarcomas and currentlyrepresent a group of neoplasms originating from any cell in the neural sheath,including malignant schwannoma and neurofibrosarcomas. Such tumors affectindividuals between 20 and 50 years of age and are strongly associated withneurofibromatosis type I.Neurofibromas are characterized by proliferation throughout all elements of thenerve structure and tend to cause diffuse nerveenlargement. They are characterized by heterogeneousmasses, with hemorrhage, central necrosis (in 40% of cases), punctatecalcifications, and a fluid-fluid level. On MRI, they present hyperintense orheterogeneous signals in T2-weighted sequences and intense contrastenhancement. In some cases, there is catecholaminehypersecretion. Approximately 40% of retroperitonealparagangliomas are malignant, usually leading to necrosis and distantmetastases. Functional imaging studies are useful forlocalization of the tumor and of distant metastases, the methods including MIBGscintigraphy, 18F-FDG PET/CT . Surgical resection is the treatment of choice,even in cases with metastasis, because it is associated with improvedsurvival.Among the neurogenic tumors originating from the paraganglion system, wehighlight retroperitoneal paragangliomas, which originate from chromaffin cellsremaining at extra-adrenal sites. The retroperitoneum is the most commonextra-adrenal location of paragangliomas, most commonly arising from the organof Zuckerkandl, which is located in the para-aortic region near the origin ofthe inferior mesenteric arteryG PET/CT , and 68G(27). Ganglioneuromas are benign,slow-growing tumors located along the paravertebral sympathetic plexus, usuallyin the posterior mediastinum or retroperitoneum. A ganglioneuroma manifests as awell-defined mass, with lower attenuation than that of muscle, containing myxoidstroma and punctate calcifications, as depicted in . The diagnosis is based on the results of thepathology study, and complete surgical resection is the treatment of choice,especially when the patient is symptomatic(40). However, there are specialistswho advocate for follow-up imaging evaluations, which should be done withcaution because of the possibility that there are also poorly differentiatedcomponents.The group of neurogenic tumors derived from sympathetic ganglia comprisesganglioneuromas, neuroblastomas, and ganglioneuroblastomas, the last two beingmore common in pediatric patientsMyxoid liposarcoma - Myxoid liposarcoma affects individuals in the40- to 60-year age group and is more common in the extremities, especially inthe deep soft tissues of the thigh, although it can occasionally arise in theretroperitoneum. It contains adipocytic and non-adipocyticmyxoid components, which create a pseudocystic appearance on imaging studies, aswell as showing slow, progressive contrast enhancement. The adiposecontent usually corresponds to less than 10% of the tumorvolume(31).Myxofibrosarcoma - Formerly known as myxoid malignant fibroushistiocytoma, myxofibrosarcoma affects individuals with mean age of 65 years,originating in the retroperitoneum in approximately 8% of cases. The imagingfindings are nonspecific, with a variable pattern due to the presence of myxoidstroma, fibrous tissue, cellular tissue, hemorrhage, and necrosis, with orwithout a fluid-fluid level or pseudocapsule(31).Key point: Myxoid stroma is a common finding in a large group ofretroperitoneal tumors and is characterized by the pseudocystic aspect and thecharacteristic hyperintense signal in T2-weighted MRI sequences. The anatomicalrelationships of the lesion, such as with nerve structures or with the organ ofZuckerkandl, help narrow the differential diagnosis, as does the presence ofgenetic syndromes.. Such tumors are described below.Necrosis is a characteristic finding of high-grade neoplasms, generallyindicating a poor prognosis and occurring in the most common malignantretroperitoneal tumorsLeiomyosarcoma - Leiomyosarcoma is the second most common primaryretroperitoneal tumor in adults between 50 and 60 years of age and originatesfrom retroperitoneal smooth muscle tissue, from the vascular wall, or fromembryonic remnants of the Wolffian ducts. On imaging, it is characterizedby a bulky mass with necrosis, with or without hemorrhage, and contiguousinvolvement of the vascular structure . On imaging studies, three vascular growthpatterns have been described: extraluminal, intraluminal, and mixed.Although surgical treatment is complex, given the difficulty related to the sizeand extent of the tumor, surgery remains the treatment of choice. Tumorrecurrence is common. Chemotherapy is also a therapeutic option,even if it is only palliative.tructure , calcifiUndifferentiated pleomorphic sarcoma - The disease formerly known asmalignant fibrous histiocytoma was reclassified as undifferentiated pleomorphicsarcoma by the World Health Organization in 2013(31). Undifferentiatedpleomorphic sarcoma is considered the third most common retroperitoneal tumorand usually affects individuals between 50 and 70 years ofage. The imaging findings are characterized bybulky, infiltrative masses with heterogeneous enhancement and areas of necrosis,hemorrhage, fibrosis, and calcifications (in 20% of cases), with or without apseudocapsule. The treatment of choice is surgical resection,and the prognosis is directly related to tumor grade, lesion size, andmetastatic status(19).Pleomorphic liposarcoma - Pleomorphic liposarcoma is an aggressive,heterogeneous tumor that is the least common liposarcoma subtype, characterizedon imaging by areas of necrosis and the absence of adipose components, making itindistinguishable from other solid retroperitoneal tumors.Key point: Liposarcoma, leiomyosarcoma, and undifferentiatedpleomorphic sarcoma are the most common malignant mesenchymal tumors of theretroperitoneum, necrosis being a characteristic common to this group anddenoting aggression.Some retroperitoneal tumors have a cystic appearance. In the differentialdiagnosis of such tumors, lymphangioma is the main lesion to be considered.Cystic lymphangioma - Cystic lymphangioma is a benign, slow-growinglymphatic malformation caused by failure of the communication between theretroperitoneal lymphatic channels and the main lymphaticsystem(33). It can present serous, chylous, hemorrhagic,or mixed contents and is characterized by a thin-walled, unilocular ormultilocular cystic mass, with variable attenuation depending on its content. content. In patiCystic mesothelioma - Cystic mesothelioma is a rare benign neoplasmof mesothelial origin and is characterized by thin-walled cystic lesions ofvarying dimensions that are indistinguishable from other cystic masses of theretroperitoneum. Although cases of malignant transformationhave been reported, some authors argue that cystic mesothelioma has no malignantpotential(44). Therefore, periodic imaging follow-up isrecommended for patients who are not candidates for surgery. Other less commoncystic lesions include Müllerian cysts, epidermoid cysts, nonpancreaticpseudocysts, cystadenoma, and cystadenocarcinoma. It should beborne in mind that some schwannomas and paragangliomas can be completely cysticand should therefore be included in the differentialdiagnosis.Key point: The management of retroperitoneal cystic lesions is aclinical dilemma, the main options being percutaneous biopsy, clinicalfollow-up, and even (in symptomatic cases) surgicalexcision(33).(1).Tumor vascularization facilitates the characterization of retroperitoneal masses.Among the hypervascular lesions, we highlight the paragangliomas. Moderatelyhypervascular tumors include leiomyosarcomas, myxofibrosarcoma, and othersarcomas. In contrast, hypovascular tumors comprise low-grade liposarcomas,lymphomas, and most of the benign tumorsThe behavior of a lesion, especially its growth pattern, extent, and relationshipwith the adjacent structures, facilitates the differential diagnosis. The termmantle growth pattern applies to lesions involving adjacent structures with noobvious signs of infiltration.Lymphoma - Lymphoma is the most common malignant retroperitonealneoplasm, as well as being the most common small round cell tumor, and typicallypresents as a para-aortic or pelvic mass, involving the adjacent structures,with a homogeneous and hypovascular aspect. Necrosis and calcifications are uncommon priorto treatment(3). The standard examination for the diagnosis,staging, and in-treatment evaluation of lymphoma is 18F-FDG PET/CT.However, FDG avidity depends on factors such as the histologicalcharacteristics, grade, and proliferation of the tumor. Some studies haveshown that low-grade (indolent) lymphomas have less FDG avidity than dohigh-grade (aggressive) lymphomas, such as large B-cell lymphoma and nodularsclerosing Hodgkin lymphoma. The treatment consists of chemotherapycombined with radiotherapy or immunotherapy, depending on the histologicalsubtype(46). One main differential diagnosis is metastaticlymph node enlargement, which has a similar appearance on imagingstudies.Erdheim-Chester disease - A rare non-Langerhans cell histiocytosisof unknown origin, Erdheim-Chester disease is characterized by multiorganinflammation composed of histiocytes and commonly affects males between 40 and60 years of age. In 29% of the cases, there is retroperitoneal involvement,characterized by homogeneous soft-tissue attenuation on CT, as well as by ahypointense to isointense signal in T1-weighted MRI sequences and a hyperintensesignal in T2-weighted MRI sequences. Circumferential involvement of the aortaand the bilateral symmetrical perirenal space is characteristic of the disease.As shown in . The diagnosis is based on the identificationof specific histopathological findings in an appropriate clinical andradiological context. Bone changes are common; biopsy is usually necessary inorder to confirm the diagnosis, even in cases in which there is a high level ofclinical suspicion(48). Due to the rarity of the disease and thescarcity of randomized studies of the topic, there is little knowledge about thespecific treatment, which is based on the use of corticosteroids,immunomodulatory agents, and cytotoxic agents, as well as otherdrugs.Retroperitoneal fibrosis - Retroperitoneal fibrosis is a rarecondition that is more common in males and is classified as idiopathic orsecondary. The idiopathic form accounts for two thirds ofall cases. The secondary form is related to neoplasms, infections, trauma, andother conditions. On imaging examinations, retroperitoneal fibrosis ischaracterized by homogeneous attenuation of the soft tissue surrounding theabdominal aorta and iliac arteries, with or without involvement of the adjacentstructures, such as the ureters and the inferior vena cava, which would resultin ureteral obstruction and venous thrombosis,respectively. On MRI, it shows an intense signal inT2-weighted sequences and a enhancement pattern that varies according to thelevel of disease activity(3). Treatment of the idiopathic form consistsof corticosteroid therapy, and treatment with tamoxifen has been quite effectivein some cases. In cases of secondary retroperitoneal fibrosis, the treatment isbased on management of the underlying disease.Key point: Retroperitoneal lesions with a mantle growth pattern canbe benign or malignant  and are typically treated withmedications or chemotherapy, suggesting that percutaneous biopsy is an importanttool in their initial clinical management.We present an algorithm with the objective of facilitating the differential diagnosisof the main primary retroperitoneal masses by analyzing the imaging characteristicspreviously mentioned . Figure Primary retroperitoneal lesions are rare and constitute a diagnostic challenge due tooverlapping imaging findings. Knowledge of imaging characteristics helps narrow thedifferential diagnosis and guide clinical decision making.


Deep vein thrombosis (DVT) is a condition classically associated with blood stasis, hypercoagulability or injury to the vasculature. As blood stasis is usually associated with patient immobility, DVT occurrence in young active patients with no underlying haematological conditions is a rarity. An exostosis, also known as osteochondroma, is a cartilage capped lesion. If solitary, they represent low malignant potential and unless symptomatic, they are rarely excised. A 23-year-old, active male, presented to hospital with pain and swelling in the left lower leg. It was a deep, non-radiating pain, exacerbated by exercise. Wells' criteria score for DVT was 2. An ultrasound was performed which identified thrombosis in the superficial femoral, and popliteal veins. Haematological causes of thrombosis were ruled out. X-ray showed a posterior femoral exostosis. It was determined that compression by the exostosis was the cause of the thrombosis. We present a case of a DVT secondary to osteochondroma formation in a young male. Isolated DVT in this setting is uncommon with fewer than five previously reported cases identified in the literature. We also discuss the current literature and management of this rare entity. Deep Vein Thrombosis (DVT) is a common condition which occurs due to the presence of thrombus in a vein. Due to obstruction of venous pathways, patients frequently complain of symptoms such as pain, redness, warmth and swelling. DVT is therapeutically managed in a hospital setting due to the risk of pulmonary embolism, which is a potentially life-threatening condition.Historically, three broad categories of factors are thought to contribute to thrombosis: venous stasis, hypercoagulability and endothelial damage. These factors are known as Virchow’s triad.A 23-year-old male presented to the emergency department complaining of pain and swelling of the left lower leg. He described a constant deep pain that was exacerbated by exercise that had been intermittently troubling him for over 1 month. On this presentation, however, the pain and swelling had occurred acutely over 12 h and persisted despite terminating exercise.On examination, the leg was noted to be inflamed and the patient described tenderness throughout the lower limb. There was associated swelling, which was non-pitting in nature. The limb was significantly (4 cm) larger in size than the contralateral leg. With a Well’s score of 2, coupled with no history of immobility, it appeared highly unlikely that the young patient was suffering from deep venous thrombosis.OsteochondromaBakers cystVenous insufficiencyVenous stasisCancerAntithrombin deficiencyProtein C deficiencyProtein S deficiencyFactor V LeidenHypercoagulabilitySmokingEndothelial damageDeep vein thrombosisVenous InsufficiencyAn ultrasound of the left leg was performed to investigate for deep vein thrombosis. Both the distal superficial femoral vein and the popliteal vein were noted to be occluded. The patient was subsequently treated with the therapeutic dose of low molecular weight heparin  and thromboembolic deterrent stockings, and the patient’s care was managed further by the haematological service. A thorough thrombophilia screen was performed. There was no haematological abnormality identified that could account for a deep vein thrombosis.With no improvement of clinical findings following prolonged therapeutic thrombolytic treatment, a repeat of the Doppler ultrasound was performed. Ultrasonography revealed that the thrombosed veins had not recanalized. A plain film radiograph of the knee, however, indicated that the patient had a small femoral bony exostosis in the popliteal fossa . A multiThe patient underwent a 3.5-h operation by a team of vascular and orthopaedic surgeons who initially performed a popliteal exploration and preservation of vessels such as the popliteal artery and vein, and the common peroneal nerve and tibial nerve .The osteochondroma was then identified and excised. Histological findings confirmed the lesion to be an osteochondroma . No mali1 This is 10 times lower than the overall population incidence.2 DVT has both acquired and genetic aetiologies.3 In this case hypercoagulability phenomena and genetic disorders were ruled out. We therefore had to consider acquired causes that may lead to venous stasis and cause recurrent DVTs in a fit young man.DVT in a young, otherwise healthy mobile person is extremely uncommon. Among 20–40 year olds in Western population, as few as 1/100,000 will develop a DVT.4 Due to their irregularity, optimal imaging for characterization and evaluation is by MRI  or pulsatile mass (38.6%) due to an underlying pseudoaneurysm, distal ischaemia (24.4%) and phlebitis (2.2%) have been reported.6 These conditions are more commonly found in male adolescents and young adults, corresponding to the age at which growth ceases and ossification of the cartilaginous spike occurs, and may be related to strenuous physical exercise.8Vascular complications are rare and are predominantly arterial,12 An isolated DVT with no arterial involvement, however, is far less common. To our knowledge, there are fewer than five cases identifiable in the literature. In this case, a multidisciplinary approach to diagnosis and management of the patient was crucial. Radiological reports indicating that there was bowing of vessels over the exostosis signalled the complicated surgery required to safely resect the bony tumour while maintaining the integrity of surrounding structures.As this case demonstrates, vascular complications are not limited to the arterial network. Pseudoaneurysm from an adjacent artery and mass effect of a large volume exostosis can lead to venous occlusion and ultimately deep venous thrombosis.6 Surgery, in the absence of symptoms, is not recommended, unless there is growth disturbances or suggestion of the lesion harbouring malignant transformation potential, as is often the case with multiple hereditary osteochondromas.Vasseur et al highlighted that vascular interruption occurs secondary to a osteochondroma should be managed early with operative excision to prevent vascular complications including distal vessel disease if arterial, or phlebitis and pulmonary emboli if venous.To date, there are very few cases of an isolated DVT secondary to osteochondroma documented in literature. Osteochondroma is usually an incidental finding on a radiograph that rarely requires intervention. This case reiterates the value of imaging in the diagnosis and management of a surgical rarity.This case report provides an overview of how DVT secondary to osteochondroma formation presents and how it is appropriately diagnosed and managed.Appropriate imaging throughout the management of a surgical rarity can provide guidance for a structured management pathway, pre –, peri – and post-operatively.The importance of a multidisciplinary approach is crucial. The vast range of possible causes of deep vein thrombosis encouraged a multifocal approach which assisted in identifying the osteochondroma as the predisposing agent to the venous occlusion.Timely and appropriate imaging can be the key to diagnosis.Follow up interval radiographs can help rule out recurrence of osteochondromas.Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Spindle cell lipoma (SCL) is a rare variety of lipoma that mostly arises in male patients and rarely affects the oral cavity. The floor of the mouth is an uncommon site for SCL, and very few cases have been reported in this location. A case of SCL is reported in a 70-year-old woman who had noticed a swelling of the floor of the mouth without any functional consequence. Both ultrasonography and RMI suggested a diagnosis of ranula, whereas clinical palpation showed a nonfluctuant mass. The lesion was excised under local anesthesia. A 37 × 32 mm encapsulated yellow mass was removed. Histological features (mature adipocytes and CD34+ spindle cells) led to a diagnosis of SCL. Medical imaging assessment of this lesion could have been influenced by the high frequency of the ranulas in the floor of the mouth. This case appears to be quite infrequent because of its location (floor of the mouth), its size (over 3.5 cm), and the patient's gender . Lipomas are not frequent in the oral cavity. They only represent 0.1 to 5% of oral tumors [Several histopathological types of lipomas have been described: fibrolipoma, sialolipoma, myelolipoma, osteolipoma, chondrolipoma, chondroid lipoma, angiolipoma, angiomyolipoma, myolipoma, intramuscular lipoma, pleomorphic lipoma, and spindle cell lipoma (SCL) . EnzingeIn 2003, Fregnani et al. reported 46 cases of lipomas of the oral cavity . Five ofWe are reporting a new case of SCL in the floor of the mouth in a 70-year-old woman.A 70-year-old Caucasian woman was referred to the hospital by her dentist for a swelling noticed 3 weeks before in the floor of the mouth. It had no functional consequence on her speech or mastication and neither on tongue mobility. An echography was performed, which lead to the conclusion of a ranula. The patient's medical conditions of osteoporosis, hypothyroidism, and mild depression were treated by alendronic acid, levothyroxine, and fluoxetine. Physical examination revealed a tender mass, in the right sublingual region, though its posterior limit could not be clearly defined .The swelling was not attached to the mandible bone or to other surrounding tissues. The mucosa covering the mass had a normal appearance and color. No inflammation, erosion, or ulceration were noted. Salivary flow from the right submandibular gland was not affected by the swelling. Palpation revealed a nonfluctuant mass without cervical lymphadenopathy.An MRI scan was prescribed, which showed a 37 mm well-defined lesion with both T1 and T2 hypersignals ; the conAn encapsulated yellow mass was removed .Histopathological examination established a diagnosis of spindle cell lipoma .A proliferation of adipocytes in a myxoid background was seen, associated to small bland spindle cells with small chromatic nuclei without mitosis or atypia, low cellular density, and without wavy collagen fibers. Adipocytes size was regular, without significant anisocytosis unlike the recently described anisometric cell lipoma . ImmunohLike in the case reported here, oral lipomas are usually asymptomatic. However, large lipomas have been reported to cause functional impairment: a lipoma measuring 5 cm disturbed lower denture stability . An 8.5 In the case series reported by Manor et al. , only onIn this case, ultrasonography could not eliminate a diagnosis of the ranula. Yet, a ranula typically appears as an anechoic or hypoechoic cystic component, whereas a lipoma has an isoechoic appearance with multiple internal fine echogenic lines . ClinicaFrom a histologic point of view, SCLs are made up of mature adipocytes and CD34+ spindle cells in a myxoid matrix with bands of birefringent collagen. Their histological differential diagnosis includes benign lesions such as classic lipoma, schwannoma, neurofibroma, leiomyoma, solitary fibrous tumor (whose prognosis is regarded as uncertain) and truly malignant ones such as atypical lipomatous tumors (well-differentiated liposarcoma), and dermatofibrosarcoma protuberans . In partThis case appears to be very infrequent because of its location (floor of the mouth), its size (over 3.5 cm), and the patient's gender . It also reminds the clinicians to prudently consider data from imaging interpretation of tumors.


TWIST1 (p.Gly86dup), along with the potentially pathogenic non-synonymous variants in NF1 , RAF1 (p.Leu445Arg), and WHSC1 (p.Ser4Asn) genes. Currently, 6 months after completion of treatment according to the SIOPEL-4 protocol, the patient is in good general condition, without any signs, and symptoms of relapse of both neoplasms. The coexistence of two different primary childhood malignancies is rarely seen. So far, only one case of synchronous HBL and NBL has been reported. However, for the first time therapeutic process was successful. A specific signature of rare germline mutations can be proposed as a predisposing factor to synchronous HBL and NBL occurrence.The aim of the following case report is to provide a description of the coexistence of two independent tumors in a child. A 9-month-old male was referred to Department of Pediatric Oncology and Hematology with hepatic tumor present on ultrasound imaging and symptoms of enlarged abdominal circumference. Physical examination revealed a palpable epigastric mass and the imaging techniques showed a tumor of the left hepatic lobe measuring 11 × 6.5 × 8.9 cm with pancreas infiltration, distant metastases in both lungs and abnormal lesion in the left adrenal gland. Basing on histopathological examination, after a core-needle biopsy, hepatoblastoma (HBL)  was diagnosed. The α-fetoprotein level was 112 993 ng/ml. Elevated values of normetanephrine, 3-methoxytyramine as well as neuron-specific enolase were observed. Due to the clinical picture and diagnosis, the patient was qualified to preoperative chemotherapy according to the SIOPEL-3 protocol, followed by SIOPEL-4 protocol for the high-risk patients. After undergoing preoperative chemotherapy, imaging tests revealed regression of hepatic tumor and no focal pulmonary masses, while regression of adrenal gland mass was not completed. The patient was qualified for left hemihepatectomy with left adrenalectomy. Histopathological examination of liver specimen confirmed the HBL diagnosis. However, in left adrenal gland and paraaortic lymph nodes the residual neuroblastoma (NBL) cells were detected. Whole exome sequencing (WES) was utilized to identify disease-associated germline mutations. WES revealed a novel germline insertion variant in  Sporadic or multifactorial etiology is considered as a cause in most cases of cancer development in children. However, as has been shown, up to 10% of cancer cases that occur in children are related to known predisposing genetic syndromes . High-thThe presence of significant genetic disorders is extremely suspected in the rare coexistence of two independent solid tumors. Both hepatoblastoma (HBL), the most common primary malignant neoplasm of the liver in children and neuroblastoma (NBL), the most frequent extracranial solid tumor, belong to the same group of tumors consisting of immature cells which often resemble individual stages of embryonic organs development. The incidence of HBL according to US registers is 1.6 cases per million, while NBL−10.54 cases per million . BecauseTo our knowledge, in the available literature only one case of the coexistence of HBL and NBL in a child has been already described .We report a case of a 9-month-old male referred to Department of Pediatric Oncology and Hematology with suspicion of hepatic tumor measuring 12.5 × 7 cm on ultrasound imaging. His medical history was remarkable for enlarged abdominal circumference lasting for 2 months, with no abdominal pain or gastrointestinal disorders. Physical examination revealed an extensive palpable epigastric mass. All kinds of dysmorphic features were excluded.Computed tomography (CT) imaging was performed as a part of routine surveillance and showed: (1) tumor of the left hepatic lobe measuring 11 × 6.5 × 8.9 cm, infiltrating pancreas and adhering to falciform ligament of the liver, neck of gallbladder, main trunk of portal vein, common hepatic artery and inferior vena cava  ; (2) hypA core-needle biopsy of segment IV and V of the liver revealed HBL . The initial α-fetoprotein (AFP) level was 112 993 ng/ml (age/sex-adjusted reference ranges: 7–53 ng/ml). Patient presented no viral hepatitis infections. Due to the presence of abnormal mass in left adrenal gland, urinary excretion of catecholamines and their metabolites as well as serum neuron-specific enolase (NSE) levels were measured. Increased values of normetanephrine , 3-methoxytyramine  and NSE  were noted. Fluctuations of NSE in the perioperative period are presented in Due to the clinical picture and diagnosis, the patient was qualified for preoperative chemotherapy according to the SIOPEL-3 protocol  . TherapyMYCN amplification.The patient was qualified for surgical treatment: left hemihepatectomy and left adrenalectomy. Elective surgical procedure was performed 4 months after the beginning of oncological treatment. The histopathological examination of the removed liver tumor confirmed the initial diagnosis of HBL  . In turnIn the early postoperative period, the bile leakage was observed without the formation of fluid collections. In the next days of hospitalization symptoms of transient gastrointestinal obstruction resolved and parenteral nutrition was gradually reduced. Wound healing proceeded properly.Currently the patient is in good general condition, without any signs and symptoms of relapse of both neoplasms, 6 months after completion of treatment according to the SIOPEL-4 protocol for HR Super PLADO.Due to the clinical course of the disease and histopathologically confirmed coexistence of two independent solid tumors, genetic tests were carried out. Genomic DNA was extracted from the peripheral blood samples according to the standard protocols. Whole exome sequencing (WES) was performed on Illumina HiSeq X Ten platform, using sequencing libraries generated with Agilent SureSelect Human All Exon V6 Kit  and TruSeq DNA Library Preparation Kit. The raw reads from FASTQ files were aligned to hs38DH reference genome using BWA MEM algorithm (version 0.7.15). Resulting BAM files were post-processed with Sentieon software implementing the equivalent GATK 3.5 Best Practices protocol and the GVCFs files were generated.The personalized panel of genes was created based on Human Phenotype Ontology (HPO) . The panel consists of genes associated with the phenotype: “HP:0003006 Neuroblastoma”; “HP:0002884 Hepatoblastoma”, and “HP:0002664 Neoplasm” . In order to calculate the coverage of obtained genes across samples, the custom script was employed. SNVs (Single Nucleotide Variants) and INDELs (short INsertions and DELetions) were discovered via joint genotyping of GVCFs.The resulting VCF was annotated and filtered using an analysis of designed workflow. After discarding variants with QUAL <30, remaining variants were annotated on variant and gene level. The variant level annotations consisted of: flags designating the presence of variant , names of diseases associated with a given variant and their clinical significance [ClinVar  and MITOMAP  databases] and their frequencies in 1000 Genomes, ExAC, gnomAD, MITOMAP and local database. Variants were also annotated with impact prediction scores, such as SnpEff annotations , SIFT—deleteriousness score , GERP and PhastCons—evolutionary conservation scores. Variant-level filtering removed variants more frequent than 1% in 1000 Genomes, ExAC, or MITOMAP populations and variants with SnpEff impact LOW or MODIFIER. Gene-level filtering retained variants located in genes present in the panel. All variants that have passed filters defined above were additionally inspected using Integrative Genomics Viewer (IGV) to detect and exclude from further analysis false-positive calls.RAF1 (p.Leu445Arg) and WHSC1 (p.Ser4Asn) were found. Both mutations have high SIFT deleteriousness score (<0.11) and high evolutionary conservation (GERP >4.7 and PhastCons = 1). A novel insertion variant in neoplasm-related gene TWIST1 (p.Gly86dup) was identified, however, insertion did not produce a translational frameshift and occurred in the poorly evolutionary conserved region. There were also two variants in neoplasm-related NF1 gene, including ultra-rare non-synonymous variant  in a highly conserved region (GERP > 4.0 and PhastCons = 1) though with poor SIFT score (0.552) and synonymous likely benign variant (p.Lys1745 =). WES also detected known pathogenic variant in MYOC (p.Gln368*). The mutation was reported in ClinVar as pathogenic with autosomal dominant inheritance for primary open angle glaucoma. The obtained results of the analysis are presented in abbreviated form in Among genes associated in HPO database with autosomal dominant model of disease inheritance, there were 4 potentially pathogenic variants identified. The rare non-synonymous variants in The above case report concerns a very rare clinical situation in pediatric oncology. To date, few cases of coexistence of various neoplasms in children have been described in the literature. Detailed statistical data defining the actual frequency of this phenomenon are missing. Due to the very low prevalence, there are no original papers about specific treatment strategies. The sequence of treatment and further outcomes depend on the type of coexisting tumors and their aggressiveness. It is also worth emphasizing that due to the similar and often nonspecific symptoms of neoplastic disease in the pediatric population, synchronous tumors constitute a diagnostic problem even for an experienced physician.in vitro fertilization-assisted pregnancy. The treatment outcomes of both patients are also different. The infant described by Ozawa et al. died immediately after admission due to a ruptured liver which was caused by the developing HBL. In turn, our patient despite of the advanced initial stage of disease is 6 months after completion of treatment and remains in close follow-up. In both cases different genetic techniques were used and various results of the analyzes were also obtained. Ozawa et al. performed their testing on the material from the twin brother of the deceased patient and showed only Gaucher disease carrier. In our case, an advanced WES was carried out using NGS technology from material taken directly from the patient. Numerous potentially significant pathogenic variants were found  mutation in patients with NBL is a rare occurrence and, to our knowledge, has not been reported previously in children with HBL. Martinsson et al.  in a highly conserved region (GERP >4.0 and PhastCons = 1) related with tumor predisposition has been reported.n et al.  gave thee et al.  reportedRAF1 mutations (OMIM 164760) and have been associated with increased risk of developing NBL (RAF1 gene (p.Leu445Arg), not yet previously described in cancer-related conditions, has been found.NBL has been associated with several dominant disorders caused by germline mutations in RAS signaling pathway, involved in carcinogenesis. Noonan syndrome and LEOPARD syndrome (known as Noonan syndrome with multiple lentigines) are both related with germline ping NBL . In our TWIST1 gene (p.Gly86dup) in patient with synchronous NBL and HBL. To date, TWIST1 (OMIM 601622) has been associated with craniosynostosis 1, Robinow-Sorauf syndrome and Sweeney-Cox syndrome. The impact of TWIST1 mutations on NBL has been rarely reported and remains unclear. Selmi et al. , is a rare occurrence with only one case available in the medical literature (WHSC1 (p.Ser4Asn) gene with high SIFT deleteriousness score (<0.11) and high evolutionary conservation (GERP >4.7 and PhastCons = 1).NBL in a child with Wolf-Hirschhorn syndrome, caused by mutation in terature . Our patMYOC (p.Gln368*) reported as pathogenic with autosomal dominant inheritance for primary open angle glaucoma.WES also detected known pathogenic variant in The issue of follow-up and prognosis in this type of patients remains unresolved. Complex genetic background has already led to the development of two independent tumors in the infancy. Undoubtedly, such unique genetic disorders may predispose to the further neoplasms in the future . In addiThe coexistence of HBL and NBL represents extremely rare clinical issue in pediatric oncology. To our knowledge, presented case report is the second such description in the literature. The use of combined oncological treatment in our patient led to remission of both independent tumors. Based on performed testing, a specific signature of rare germline mutations can be proposed as a novel predisposing factor to synchronous HBL and NBL occurrence.Written informed consent was obtained from the patient's parents for the treatment according therapeutic protocols, genetic testing as well as for the publication of the case report and potentially-identifying information and images.SS, KS, MaK, and WB contributed to case report concept and design. SS, KS, MaK, MiK, and MP wrote sections of the manuscript. TL, MH-K, MiK, MP, KK, LW, KW, and WG performed diagnostic tests and collected relevant clinical data. SS and WB critically revised the article. All authors were responsible for the integrity and accuracy of the data and approved the submitted version.Authors MiK, MP and KK are employed by company ‘Intelliseq sp. z o.o.’. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Spontaneous splenic rupture is an atraumatic event that represents a rare and life-threatening acute complication in which the spleen is damaged producing internal hemorrhage in the abdominal cavity. Its association with hematologic malignancies, although a rare occurrence, has been previously described. Among this subset of patients, chronic myeloid leukemia is one of the main causes.A 26-year-old male with history of chronic myeloid leukemia presented with acute intense abdominal right lower quadrant pain. Computed tomography showed a wedge in the lower third of the spleen (probably associated with infarction), active bleeding, and hemoperitoneum. Laparotomy and splenectomy were performed.The most common symptom of spontaneous splenic rupture is acute abdominal pain, sometimes radiating to the left shoulder. It can also be associated with nausea, emesis and signs of hypovolemia or shock. Splenomegaly may be absent. Diagnostic methods of choice are computed tomography and ultrasound.Management of splenic rupture is divided in surgical and conservative. The former is reserved for patients with extensive splenic injury that is accompanied by hemodynamic instability or other trauma that warrants surgical treatment. Patients who do not meet these criteria and respond to initial stabilization strategies can be offered clinical and laboratory monitoring. Stable patients with moderate to severe splenic injuries can be offered angioembolization.It is important to include splenic rupture as a differential diagnosis for acute abdominal pain, especially in patients with hematologic malignancy, since early recognition and treatment increases patient survival and improves prognosis. Spontan2A 26-year-old male patient with history of CML was admitted to our hospital’s emergency department for acute abdominal pain. The pain was intense, had initiated 2 h before presentation, and was located in the right lower quadrant and right iliac region. Nausea without emesis and dysuria were also present. Diagnosis of CML had been made 2 months prior to the index event and he had rejected chemotherapy due to personal reasons; further, he denied taking other medications and there was no relevant family history. At admission, the patient had the following vital signs: heart rate 80 beats/min, respiratory rate 18 breaths/min, arterial pressure 123/71 mmHg, body temperature 36.0 °C, and oxygen saturation 87 % in ambient air. Physical examination was notable for mucocutaneous pallor and a distended abdomen; abdominal guarding and rebound tenderness were absent. The patient reported no history of trauma. Differential diagnoses initially considered included blast crisis, urolithiasis, pneumoperitoneum or abdominal viscera infarction.After 2 h in the emergency department, the patient reported an increase in pain which was unresponsive to high doses of opioids.3), low hemoglobin (11.2 g/dL) and hematocrit (33.3 %) values, and thrombocytosis (925.000 platelets). Hypokalemia was also detected . Other blood tests were unremarkable . Abdominal computed tomography (CT) scan was also performed, which reported “important hepatosplenomegaly, an ill-defined lesion with irregular infiltrating edges and vascular tracts, and free fluid in the abdominal cavity” ; infectious diseases, such as malaria, mononucleosis or other viral infections 27.3 %); and inflammatory or neoplastic disorders like acute or chronic pancreatitis (20.0 %). Other described causes include drugs and chemotherapy such as treatment with filgrastim (9.2 %) and mechanical disorders 6.8 %). A small number of cases have been described in patients with normal spleens and no underlying condition (6.4 %)5. SSR has an approximate 12 % mortality [.8 %. A s.3 %; andSSR secondary to hematological neoplasms is rare. According to the literature reviewed, approximately 200 cases have been reported; chronic myeloid leukemia appears to be one of the main causes 15.8 %) along with Hodgkin lymphoma (36.2 %). Other causes are myeloproliferative disorders (15.8 %), acute myeloid leukemia (13.8 %), acute lymphoblastic leukemia (7.9 %), and myelodysplastic disorders (7.9 %) [.8 % alonSeveral mechanisms through which rupture occurs have been postulated. Among these the most important is congestion of splenic parenchyma and infiltration by blast cells. Nevertheless, concomitant coagulation disorders are likely present leading to sub-capsular hemorrhage and splenic infarction .The most common symptom observed in SSR is acute abdominal pain of variable location and intensity, sometimes radiating to the left shoulder (Kehr's sign). It can also be associated with hypotension, fever, tachycardia, tachypnea, nausea, vomiting and low hematocrit values, reflecting active hemorrhage inside the abdominal cavity. Splenomegaly may be absent; however, this does not rule out the diagnosis ,10. AcutDiagnostic methods of choice are CT scan and ultrasound. Nevertheless, only 19 % of SSR cases reported in the literature were diagnosed correctly . UsuallyIn this patient, a grade IV splenic rupture was found, according to the spleen damage scale of the American Association for the Surgery of Trauma (AAST) , indicatManagement of splenic rupture has been classically divided into surgical (splenectomy) and conservative . According to the 2017 splenic trauma guidelines, the former is reserved for patients with extensive splenic injury that is often accompanied by hemodynamic instability or other concurrent trauma that warrants surgical treatment. This means that patients who do not meet these criteria and respond to initial stabilization strategies can be offered close clinical and laboratory monitoring. Nevertheless, another group of patients has been recently identified that would benefit from angioembolization techniques. Such patients typically are hemodynamically stable at the time of procedure, have moderate to severe splenic injuries and show associated vascular abnormalities on CT scan. It has also been proposed as a salvage therapy for monitored patients that show signs of persistent hemorrhage. In any case, if angioembolization fails to control hemorrhage surgical treatment must be offered .Splenectomy has a morbidity rate of 14–61% and a mortality rate of 0–28%. These values are presumed to be higher in patients with hematologic malignancies due to an increased risk of bleeding and infection. The most commonly associated risk factors are spleen weight over 2 kg, male gender and increased surgical time. Moreover, a variety of postoperative complications exist, such as subphrenic abscess, hemoperitoneum, respiratory complications, postsplenectomy fever, sepsis, thrombocytosis, among others. Splenectomy performed by advanced laparoscopic surgery reduces the incidence of these complications but is usually not recommended in the trauma setting ,13,14,16Other surgical approaches  are usually recommended for localized diseases of the spleen, which is not the case for most causes of SSR ,18.Importantly, current evidence-based guidelines have been proposed for the management of splenic traumatic injury. On the contrary, SSR remains a somewhat uncommon pathology and its management is largely based on local experience, case reports and extrapolated data from trauma cases.4SSR is an important differential diagnosis for acute abdominal pain, especially in patients with underlying hematologic malignancy. Timely diagnosis and treatment is associated with a high survival rate and less complications.The study had no sponsors and no funding was required.In our institution case reports do not require ethical committee approval.Written informed consent was obtained from the patient for publication of this case report and accompanying imagesRoberto Rueda-Esteban: Conceptualization, Methodology, Investigation, Writing – Review & Editing, Visualization, Supervision. Nicolás Stozitzky Muñoz: Investigation, Writing – Original Draft, Writing – Review & Editing, Visualization. Mónica Barrios Díaz: Investigation, Writing – Original Draft, Visualization. Andrés García Sierra: Investigation, Writing – Original Draft, Visualization. Carlos Felipe Perdomo: Conceptualization, Methodology, Writing – Review & Editing, Supervision.This case report does not require registration.Roberto Rueda-EstebanNot commissioned, externally peer-reviewedNothing to declare.


A 51 year old man presented with progressive swelling in the upper arm. MRI revealed a solitary mass extending from the median nerve. Intraoperative finding was a tumour extending within the nerve in its proximal fibres. The histological result showed a Castleman disease. They can be subdivided into two categories according to their intrinsic or extrinsic growth: Peripheral nerve sheath tumors (PNST) and Peripheral non-neural sheath tumors (PNNST). Each entity includes benign and malignant tumours ,2 Table.Diseases arising from lymphoid cells which are associated to the peripheral nerve system are extremely rare. A few cases of primary lymphoma , neuroly2.A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.MRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters . The priSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened . In its The postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.The final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules . ImmunohTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm .In a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography.3.Retrospectively one can discuss what would have been the best neurosurgical management for our patient. Would he have benefit from a more radical tumour resection? Or contrariwise, would a frozen section have been helpful in such a case and a biopsy been the consequence? Should staging have had priority?According to the literature unicentric Castleman disease has a good prognosis concerning the overall survival if treated by either complete surgical removal or a combination of surgery and radiation . In thisThe histological dignity and growth behavior of a peripheral nerve tumour is irremissible information in this benefit-risk-assessment between complete removal on the one side and preservation of sensorimotor function on the other side. The features from Castleman disease distinguish clearly from the characteristic of tumours originating from the nerve sheath which is neoplastic proliferation of Schwann cell differentiation . NeverthIn our opinion the fact of its rareness, the challenging MRI, the malignant histological features, the necessity of interdisciplinary treatment and the lack of experience in the question of radicality justify that unicentric Castleman disease is registered in the itemization of peripheral nerve-associated malignant neoplasms.4.Castleman disease is a group of lymphoproliferative disorders including special histological characteristics of lymph node. It is rare and may affect any body region. A manifestation at a solitary peripheral nerve has not been described so far. We present a case in which an unicentric Castleman disease mimicked a MPNST if dealing with the radiological and neurosurgical features. The pathological examination resolved the challenging case. An interdisciplinary treatment approach seems to be essential.


The application of post-mortem computed tomography (PMCT) and PMCT angiography (PMCTA) is becoming increasingly common in forensic investigations. One of the most frequently applied techniques today is PMCTA. However, few studies have focused on the application of contrast-enhanced PMCT of hollow organs such as the gastrointestinal tract. The intestine is a special digestive organ with a complicated anatomical structure; it is difficult to separate in medicolegal investigations, during which new rupture may occur, affecting the examiner's judgment. Moreover, the formalin-fixed intestine is more difficult to separate because of its increased brittleness. In the present case, the authors applied contrast-enhanced PMCT to the diagnosis of intestinal perforation caused by a medical accident.A 67-year-old woman with cholecystitis underwent laparoscopic cholecystectomy in the hospital. The gallbladder was successfully removed, but the doctor was suspected to have accidentally perforated her intestinal tract with the laparoscopic machinery. The patient developed severe peritonitis and died after the operation.Contrast-enhanced PMCT with isolation of the intestinal tract was performed after dissection of the body. The results suggested that the contrast agent flowed out through the rupture. The autopsy and histological examination revealed a perforated crevasse, confirming the cause of peritonitis while excluding other probabilities despite the doctor's denial.Contrast-enhanced PMCT was an effective technique with which to interpret this gastrointestinal tract rupture and served as a non-invasive tool for identifying the injury. The use of post-mortem computed tomography (PMCT) combined with PMCT angiography (PMCTA) is also applied in forensic investigations. Contrast agents are most frequently applied to the central nervous system, heart, and circulation. However, few reports have described the application of contrast to the gastrointestinal tract. We herein report a case involving the use of contrast-enhanced PMCT for the diagnosis of intestinal perforation.Medical contrast media is conventionally used to enhance the contrast of structures within the body during medical imaging.22.1A 67-year-old woman with cholecystitis was hospitalized for upper abdominal cramps. Laparoscopic cholecystectomy was performed, and her abdominal cavity was explored under monitoring. Her gallbladder was then removed and packed in a specimen bag to be taken out for examination. The doctor inadvertently perforated her intestinal tract with the laparoscopic machinery. The doctor attempted to conceal the accident from the patient's family and secretly suture closed the crevasse to escape responsibility. As a result, the patient developed severe peritonitis and died about 2 weeks after the operation. An autopsy and contrast-enhanced PMCT were performed. External forensic examination and conventional autopsy were carried out. The isolated intestine was then prepared for the examinations described below.This study was approved by the Academic Committee of the Institute of Forensic Science, Ministry of Justice, People's Republic of China. Written informed consents were obtained from the victim's family to publish these case details.2.2Contrast-enhanced PMCT of the isolated intestinal tract was carried out after the autopsy. First, we inserted a urinary catheter into the upper end and ligated the 2 ends of the intestine. The whole process was divided into 5 steps: CT scanning, air-enhanced CT scanning, water-enhanced CT scanning, formalin-unfixed intestinal CT scanning, and formalin-fixed intestinal CT scanning. The isolated intestinal tract was supported by a handmade device Fig.  and scan2.3The body was 154 cm tall and of medium build. Observations included about 400 ml of yellow turbid liquid within the abdominal cavity, omental adhesion, and a 2-cm crevasse 24 cm from the ileocecal section that was sewn with 6 stitches. No leakage of intestinal contents was detected in the crevasse. Another 0.9-cm × 0.3-cm crevasse was observed next to the stitches. The edge of the crevasse was regular, dark brown, and deep within the abdominal cavity. Squeezing the intestinal tract resulted in overflow of a small amount of intestinal contents from the crevasse , for editing the English text of a draft of this manuscript.We thank Angela Morben, DVM, ELS, from Liwen Bianji, Edanz Editing China (Funding acquisition: Kaijun Ma.Methodology: Lei Wan.Project administration: Kaijun Ma.Resources: Donghua Zou.Supervision: Yijiu Chen.Writing – Original Draft: Zhuoqun Wang.Writing – Review & Editing: Ningguo Liu.