Hyaline ring granuloma (HRG) of the oral cavity is an uncommon disorder considered to be a foreign-body reaction resulting from implantation of food vegetable particles. Microscopically, it is characterized by the presence of structures of hyaline rings in an inflamed fibrous tissue background, which contains multinucleated giant cells.We present the case of a 4-year-old boy diagnosed with a mandible osseous HRG, which showed clinical and tomographic aspects suggestive of an aggressive bone tumor.The patient underwent surgical exploration and histopathologic analysis showed fragments composed predominantly of widespread dense connective tissue with an acute and chronic inflammatory infiltrate containing multinucleated giant cells and scattered areas of eosinophilic material associated with hyaline rings, strongly suggestive of vegetable particles. The eosinophilic material was positive for periodic acid-Schiff (PAS) and resistant to diastase digestion. These features led to diagnosis of osseous HRG. Scanning electron microscopy (SEM) analysis was performed for illustrative purposes and the multiple structures resembling vegetable particles were characterized in more detail.Although rare, this case highlights the importance of the clinician’s awareness regarding the existence of an osseous counterpart of HRG. Key words:Agenesis of the corpus callosum, child, hyaline ring granuloma, intraosseous, mandible, pulse granuloma. Hyaline ring granuloma (HRG) is a rare benign lesion of the oral cavity that affects almost exclusively soft tissues, with unclear and controversial etiology. The histopathological findings are characterized by the presence of a hyaline ring of eosinophilic amorphous material lying in a fibrous connective tissue with an inflammatory background and multinucleated giant cells ,2.Since 1971, HRG has been described in the literature under various terminologies, such as giant-cell hyaline angiopathy ,3, and pTwo opposing theories have been proposed in order to explain the nature of oral HRG . The exoOral HRG is mostly observed in adults, with the majority of cases affecting the soft tissue of edentulous areas . The cliOsseous cases of HRG have only rarely been reported in the English language literature, with a variable radiological appearance. Nevertheless, most of these cases are well-delimited radiolucent areas with only discreet bone erosion . The majA 4-year-old boy was referred for evaluation of a painless swelling in the mandible. According to his mother, 14 days before attending our clinic he presented high fever and edema on the left side of his face. His medical background included congenital agenesis of the corpus callosum causing vision impairment, cognitive low muscle tone and poor ability. He was taking phenobarbital (40 mg/day) to prevent recurrent seizure. No swelling was noted on his face on extraoral clinical examination. However, the patient presented lack of lip sealing. Intraorally, a fibroelastic swelling affecting the left body of the mandible measuring approximately 1.5 x 0.8 cm was noticed. The lesion was covered by normal mucosa without evidence of infection  scan was performed and showed a hypodense osteolytic lesion on the left side of the mandible, extending from the distal surface of the deciduous canine to the molar regions Fig. B. FurtheThe patient underwent surgical exploration under general anesthesia and a yellowish fibrous material was completely removed Fig. C. HistopDespite the extensive discussion regarding the nomenclature and etiopathogenesis of HRG, it is most likely to be a consequence of implantation of leguminous seeds, resulting in a granulomatous reaction against this foreign body. According to the exogeneous theory, food debris may incidentally have been impounded on the mucosa in sockets following tooth extractions or secondary to bone lesions such as odontogenic cysts. These foreign materials then undergo attempted degradation by host phagocytic cells and produce eosinophilic and hyaline ring structures . TalackoWhereas cases of HRG occur in the oral cavity , they haThe most recent review of the English language literature regarding oral HRG found a total of 173 cases and the majority occurred in soft tissue . ClinicaBased on a review of the well-documented cases of oral osseous HRG, the majority of cases were diagnosed in adults (70%) and the most common location was the mandible of edentulous patients, and a large proportion of them occurred in association with odontogenic cysts .Keirby and Soames  (1985) aUnlike the reports found in the literature, the current case represents an unusual oral osseous HRG in a child, with atypical clinical and radiographic features that could lead to misdiagnosis and inadequate treatment, including an excessively aggressive approach. Due to a poor clinical history and limited clinical information, foreign bodies affecting infants may be confused with other lesions, including neoplastic processes .Nuts and seeds are commonly reported as foreign bodies in children due to inhalation, with the risk of suffocation . On rareIn conclusion, the recognition of oral osseous HRG in children is important to enable a correct diagnosis and avoid unnecessary aggressive surgical treatment.


AFLP) is a rare liver disease unique to pregnancy that can lead to acute liver failure. Clinicians must have a high index of suspicion for AFLP because only early diagnosis and prompt delivery improve maternal and fetal prognosis.Acute fatty liver of pregnancy ( LDH was normal (193 UI/L). Ultrasonography (US) of the liver showed one hyperechogenic nodule suggesting a tumor. The patient gave birth spontaneously to a healthy girl 1 day after admission. Computed tomography (CT) after delivery showed a well‐limited zone corresponding to the US hepatic nodule , hypoglycemia (2.20 mmol/L), hyperuricemia (445 Clinicians must have a high index of suspicion for AFLP. Indeed, early diagnosis and prompt delivery have improved maternal and fetal prognosis of this rare liver disease unique to pregnancy BA and YB: drafted the article. BS: participated in critical review and revision of the article, gave the final approval of the article.The authors have no conflict of interest to declare.


Gastric schwannoma (GS) is a rare neoplasm of the stomach. It accounts for 0.2% of all gastric tumors and is mostly benign, slow-growing, and asymptomatic. Due to its rarity, GS is not widely recognized by clinicians, and the precise differential diagnosis between GS and other gastric submucosal tumors remains difficult preoperatively. The present study reports a case of GS misdiagnosed as gastrointestinal stromal tumor and reviews the clinical, imaging, and pathological features, treatment, and follow-up of 221 patients with GS previously reported in the English literature. Although GS is rare, the case reported in the current study highlights the importance of including GS in differential diagnoses of gastric submucosal tumors. Furthermore, the findings of the review suggest that although many cases are asymptomatic, the most common symptoms are abdominal pain or discomfort, not gastrointestinal bleeding, and malignant GSs present with clinical symptoms more commonly. Although large-sample multicenter studies on the efficacy, safety, and oncological outcomes of minimally invasive techniques are required, the findings presented herein may be helpful for clinicians when diagnosing or treating GS. Gastric schwannoma (GS) is a rare submucosal tumor that arises from Schwann cells in the neural plexus of the stomach. It accounts for only 0.2% of all gastric tumors, 6.3% of gastric mesenchymal tumors, and 4% of all benign tumors of the stomach . GS was The present study reports the case of a 61-year-old woman with GS and reviews the current knowledge of GS available based on sporadic case reports and the occasional series of case reports in the literature. We hope that the findings will be useful for clinicians during the diagnosis and treatment of GS.A 61-year-old woman with a 2-year history of nonspecific epigastric abdominal pain underwent esophagogastroduodenoscopy (EGD) to rule out a digestive ulcer. EGD revealed a submucosal bulge on the anterior wall of the gastric body . The patA computed tomography (CT) scan revealed a uniformly enhancing mass located between the left lobe of the liver and the lesser curvature of the gastric body. The mass was partly exophytic and partly projected into the gastric lumen, causing smooth indentation, and measured 4.3 × 3.2 cm . The patThe patient was then subjected to laparoscopic examination, which showed an exophytic tumor (4 × 3 cm size) arising from the anterior wall of the lesser gastric curvature. The exophytic part of the mass appeared off-white in color and rough, with a concavo-convex surface; however, the margin of the mass on the gastric wall was clear . To achiA review of the existing literature identified a total of 221 cases of GS . The meaGS typically grows as a solitary lesion and is commonly located in the body of the stomach. In the current review, we found only 1 case that reported the presence of two GS lesions in the same patient . The mosGSs are usually benign and patients have an excellent prognosis after curative resection. Nevertheless, we identified 10 reported cases of malignant GS in the last several decades , 24, 25,18F]-fluorodeoxyglucose positron emission tomography (FDG-PET), have been clearly described in several isolated case reports and some case series [The features of GS shown by imaging, including CT, magnetic resonance imaging (MRI), and [e series –34. Briee series . FDG-PETe series . Althouge series , 35, 36 e series . TherefoEndoscopic ultrasonography (EUS) is considered to be the most reliable procedure for the assessment of patients with gastrointestinal submucosal lesions –40, and Regarding its gross appearance, GS commonly presents as a yellow-white or off-white, solid, well-circumscribed, and round mural mass. Microscopic examination demonstrates that the typical cytological/morphological features of GS are palisade-arranged spindle cells and peritumoral cuff-like lymphocytic infiltration . On histComplete surgical resection is widely considered to be a curative treatment for GS, and laparoscopic or open approaches for wedge resection, subtotal gastrectomy or near-total resection, and total gastrectomy are the treatments of choice , 47–51. A paper published in 2015 by Hong et al.  reviewedAlthough GS is rare, the case reported in the current study highlights the importance of including GS in differential diagnoses of gastric submucosal tumors. Furthermore, the following points regarding GS should be noted: (i) the magnitude of the gender predilection may be reduced as more cases are reported; (ii) the most common symptom is abdominal pain or discomfort, but not gastrointestinal bleeding; (iii) patients with malignant GS commonly present with some clinical symptoms; (iv) although endoscopic submucosal tunneling resection, endoscopic enucleation, and endoscopic full-thickness resection, with or without laparoscopic assistance, have been actively performed as diagnostic and therapeutic techniques for GS, large-sample multicenter studies on the efficacy, safety, and oncological outcomes are still required.


We report a very unique case of an esophageal metastasis from a pancreatic ductal adenocarcinoma (PDAC) primary.After obtaining consent from the patient, all relevant records of the case were obtained and retrospectively reviewed.At presentation, the patient was diagnosed with synchronous pancreatic and esophageal cancer. He received six months of neoadjuvant therapy including FOLFIRINOX  and stereotactic body radiation therapy (SBRT) to the pancreatic tumor followed by a combined pancreaticoduodenectomy and Ivor Lewis esophagectomy. Review of the final esophageal specimen revealed normal overlying squamous mucosa with an underlying focus of metastatic PDAC. The patient remains alive with no evidence of disease 17 months from surgery and 25 months from diagnosis.Differentiating an esophageal metastasis from a PDAC primary versus a synchronous esophageal carcinoma is very difficult despite state-of-the-art diagnostic techniques performed at a high-volume tertiary cancer center. Extensive evaluation and continued follow-up of PDAC patients presenting with a synchronous esophageal lesion in a multidisciplinary setting may help ensure efficient and accurate management. In our case, neoadjuvant FOLFIRINOX and SBRT to the primary PDAC tumor followed by surgery has been an effective approach for this patient. Pancreatic ductal adenocarcinoma (PDAC) is the third leading cause of death by a solid malignancy in the United States, with a 5-year overall survival rate of 8%.  PDAC is PDAC most commonly metastasizes to lymph nodes, the liver, lung, and peritoneal cavity, while rare locations that have been reported include bone, brain, myocardium, and the umbilicus. , 4 At thnd case of a metastasis to the esophagus arising from a PDAC primary reported in the modern era (since the 1980s). [Not only is a PDAC metastasis to the esophagus extremely rare, but it is also difficult to distinguish an esophageal primary from a metastasis to the esophagus by radiographic imaging or endoscopy. To our knowledge, we report the 2 1980s). -13A 72-year-old non-smoking male presented with a 6-month history of weight loss (9 kg) followed by obstructive jaundice characterized by a 2-month history of acholic stools and dark urine. Past medical history was significant for hypertension and dyslipidemia and an extensive family history of cancer was significant for pancreas, liver, breast, gynecologic, and colon malignancies in 5 siblings and his father. Initial evaluation was conducted by his primary care provider and included laboratory studies and imaging. Computed tomography (CT) scan of the abdomen and pelvis revealed a 2.5 x 1.7 cm mass in the pancreatic head, abutment of the superior mesenteric artery (SMA) and vein (SMV), and marked biliary and pancreatic ductal dilatation consistent with PDAC. Liver function tests (LFTs) were elevated, with an alkaline phosphatase of 515 IU/L, aspartate aminotransferase of 198 IU/L, and total bilirubin of 10.3 mg/dL. Carbohydrate antigen 19-9 (CA 19-9) at this time was 395 U/mL. Upon further workup by a gastroenterologist, endoscopic ultrasound (EUS) with fine needle aspiration (FNA) revealed adenocarcinoma of the pancreatic head in addition to an incidental 2.0 cm distal esophageal exophytic lesion that returned positive for adenocarcinoma. The relationship of these two carcinomas was uncertain. Endoscopic retrograde cholangiopancreatography (ERCP) was also performed for metallic biliary stent placement to relieve high-grade biliary obstruction related to the pancreatic mass.Further imaging with 18-fluorodeoxyglucose positron emission tomography (FDG-PET)/CT demonstrated a large hypodense mass in the head of the pancreas with moderate FDG activity consistent with the patient’s known PDAC in addition to multiple enlarged peripancreatic, aortocaval, and porta hepatic lymph nodes as well as a focal area of mild metabolic activity in the distal esophagus just above the gastroesophageal junction with multiple paraesophageal lymph nodes. At an outside institution, the patient was diagnosed with localized PDAC that was thought to be unresectable along with a separate esophageal adenocarcinoma primary. Therefore, his local oncologist recommended chemotherapy with FOLFIRNOX  and referred the patient to our Pancreatic Multidisciplinary Clinic (PMDC) for additional recommendations. nd and 3rd portions of the duodenum and demonstrating proximal main pancreatic duct dilation. Vessel involvement included encasement of the SMV/portal vein (PV) confluence and 180° abutment of the SMA, thereby conferring a diagnosis of borderline resectable PDAC  or stereotactic body radiation therapy (SBRT) with re-evaluation for potential surgical resection. FOLIFIRINOX was the recommended chemotherapy such that the platinum agent would have activity in both primary pancreatic and esophageal tumors. Depending on the expertise of the thoracic oncologists and tumor response to chemotherapy, standard CRT would be warranted in order to encompass both the esophagus and pancreas in the same field; however, if the esophageal lesion would not require neoadjuvant radiation, SBRT to the pancreas lesion would be preferred. In order to address the suspected esophageal lesion, our thoracic colleagues were consulted and the patient was referred for formal evaluation by a thoracic surgeon.After endoscopy and thoracic surgical consultation, the esophageal lesion was thought to be a synchronous esophageal primary cancer  and the treatment recommendation consisted of neoadjuvant FOLFIRINOX followed by SBRT and evaluation for surgery. It was understood that treatment of the PDAC was of primary significance, with the possibility of delivering definitive CRT to the esophagus later in the treatment course.The following week, FOLFIRINOX was initiated locally and continued for 3 months . Following 6 doses of FOLFIRINOX, the patient presented back to our PMDC for re-evaluation. The patient continued to work 12-hour days throughout therapy, with his only complaint being minor fatigue. His CA 19-9 had decreased to 71.9 U/mL at this time (4 months from diagnosis), with CT demonstrating the pancreatic mass and regional lymphadenopathy to be slightly less bulky, improvement of SMA/SMV involvement Figure , and impThe patient then underwent SBRT to the pancreatic tumor to a total cumulative dose of 30.5 Gy in 5 fractions. Image guidance was performed using 3 gold fiducial markers endoscopically placed around the lesion and active breathing control (ABC) was used to minimize movement of the tumor during respiration. Images of the pancreatic and esophageal lesions at the time of endoscopy can be visualized in Figure Of note, an esophagogastroduodenoscopy (EGD) was performed at the time of endoscopic fiducial placement to re-biopsy the esophageal lesion. The morphology was most consistent with a carcinoma that spread from the pancreaticobiliary system and immunolabeling for SMAD4 demonstrated retention of labeling, which neither confirmed nor refuted an interpretation of spread from a pancreaticobiliary lesion. The patient also experienced a few episodes of hematochezia during chemotherapy. A colonoscopy was performed and reported as negative, with the bleeding resolving spontaneously.Eight months after initial diagnosis and after six months of neoadjuvant therapy, the patient underwent a pylorus-preserving pancreaticoduodenectomy and Ivor Lewis esophagectomy with jejunostomy feeding tube (J-tube) placement. During the operation, the right gastric artery was preserved and the blood supply to the stomach was confirmed both visually and with an intraoperative Doppler ultrasound. The pancreatic specimen revealed numerous microscopic foci of adenocarcinoma with vacuolated cytoplasm and hyperchromatic nuclei scattered within a 5 cm fibrotic tumor bed Figure , otherwiin situ carcinoma. Focally active chronic gastritis and chronic cholecystitis were noted. The normal overlying squamous mucosa as shown in Figure The esophageal specimen revealed a microscopic focus (3 mm) of infiltrating adenocarcinoma involving submucosa of the distal esophagus Figure  along wiThere were no major surgical complications although gram-positive cocci in chains were found in the wound and treated with antibiotics and negative-pressure wound therapy was performed for drainage at the incision site. One month from surgery, the patient was doing well with no major complaints other than intermittent abdominal pain. His J-tube was removed at the time of two month follow-up and adjuvant chemotherapy was recommended; however, numerous appointments were missed and the patient refused adjuvant therapy. Unintentional weight loss secondary to pain during eating and acid reflux led to a nutrition consultation and prescription of a proton pump inhibitor and pancreatic enzyme supplementation. The patient occasionally complains of abdominal pain and nausea but is doing well otherwise and remains with no evidence of disease 17 months from surgery and 25 months from diagnosis, with surveillance laboratory studies and imaging occurring every 3 months.th reported case of an isolated esophageal metastasis from PDAC and the 2nd report on an aggressive approach of a combined pancreaticoduodenectomy and esophagectomy. [This case report summarizes a unique case of a patient with PDAC with an isolated metastasis to the esophagus who underwent successful resection of a pancreatic head and distal esophageal lesion after neoadjuvant therapy. To our knowledge, this is the 12gectomy. -13, 15The patient’s pancreatic primary showed moderate treatment effect among a background of extensive fibrosis, whereas the esophageal lesion showed a more classic appearance of an untreated adenocarcinoma with numerous dilated glands with an infiltrative growth pattern, nuclear atypia, and scattered mitotic figures. While there was focal Barrett’s mucosa elsewhere in the esophagus, it did not show significant dysplasia. Most significantly, the adenocarcinoma in this case had overlying squamous mucosa with no significant histopathologic change. While SMAD4 expression was intact in the esophageal lesion, roughly half of PDACs retain SMAD4 expression, and this result does not exclude spread from a PDAC primary. , 17versus a synchronous esophageal carcinoma. Despite numerous methods of imaging and procedures including CT, PET/CT, EUS, EGD, and immunohistochemistry as well as multidisciplinary review among radiology, pathology, gastroenterology, surgery, medical oncology, and radiation oncology at a high-volume tertiary center, the final diagnosis of metastatic PDAC to the esophagus was not reached until a substantial amount of tissue was reviewed after partial esophagectomy. FDG-PET is commonly used in combination with CT and/or EUS to identify occult metastases in pancreatic and esophageal adenocarcinomas. However, the sensitivity and specificity of detection of metastases range from 50-90% and, as observed in this case report, may not lead to conclusive evidence. [This case report elucidates the difficulty of differentiating an esophageal metastasis from PDAC primary vidence. -21In general, local therapy is not traditionally recommended for metastatic disease, PDAC or otherwise; however, oncologists are becomingly increasingly aggressive by offering radiation therapy and/or surgical resection in this patient population, particularly in the setting of limited oligometastatic disease. , 22-27 Ivs. 6.8 months), progression-free survival (6.4 months vs. 3.3 months), and objective response (31.6% vs. 9.4%). Since then, FOLFIRINOX has been studied in other settings of PDAC as well as other gastrointestinal cancers, with promising response rates in patients who are able to tolerate the regimen. [Neoadjuvant FOLFIRINOX was administered in this patient as a method to provide aggressive systemic therapy and include a platinum agent that may warrant a treatment response in both the PDAC and esophageal lesion. In 2011, Conroy and colleagues published the results of a randomized clinical trial comparing FOLFIRINOX and gemcitabine monotherapy.  FOLFIRINregimen. -35An esophageal metastasis from a pancreatic primary may be more common than traditional belief and patients with a suspicious esophageal lesion should undergo comprehensive evaluation and close follow-up in order to guide management. Although there are limited data to suggest an optimal approach to these cases, neoadjuvant FOLFIRNOX followed by SBRT and surgery has resulted in favorable disease control over two years from diagnosis despite no adjuvant therapy. However, we only recommend aggressive surgery of both lesions if there is no clear evidence of metastatic disease at other locations after an extended period of time ( > 6 months). As future technologies involving novel analytic techniques such as next-generation sequencing, , 37 circAfter obtaining consent from the patient, all relevant records of the case were retrospectively reviewed. The pathologic specimens were reviewed with response to neoadjuvant therapy graded using the criteria described previously by the College of American Pathologists.


Dear Editor,A 32-year-old male sought treatment, complaining of headache. Computed tomography (CT) ofthe brain revealed hyperdense intraventricular nodule to the right of the foramen ofMonro, highly suggestive of a colloid cyst . The pat-3. Primary central nervous system (CNS) lymphomas account for upto 6% of brain neoplasms and 1-6% of extranodal lymphomas; approximately 90% of primaryCNS lymphomas are non-Hodgkin lymphomas of the diffuse large B-cell subtype-6. The incidence of CNS lymphoma is higher in the presence of certainimmunodeficiencies, especially human immunodeficiency virus (HIV) infection. Among immunocompetent individuals, theprevalence of CNS lymphoma is highest (60-67%) in men 45-75 years of age. In that group,CNS lymphomas present as a single homogeneous mass (in 62%), often in the supratentorialcompartment (in 83%) and notably in the deep white matter (in 57%). The corpus callosumand regions surrounding the ventricles are typically affected. Perilesional edema isalso common, being seen in 77-90% of cases,3-6. On CT scans, CNS lymphomas are typically hyperdense, becausethey are hypercellular and have a high nucleus-cytoplasm ratio,3. On MRI, they often demonstrate a hypointense or isointense signal inT1-weighted sequences and an isointense or hyperintense signal in T2-weighted sequences.After intravenous administration of contrast medium, they show homogeneous (90%) or, inrare cases, annular enhancement. They also exhibit signs of restricted water diffusion.Perfusion-weighted imaging shows less vascularization than that seen in other malignantbrain tumors. On magnetic resonance spectroscopy, CNS lymphomas show elevated lipid andcholine peaks, as well as a reduction in N-acetyl-aspartate levels,3-5. The definitive diagnosis is made bybiopsy,2,4,6. Such lymphomas respond to chemotherapy andradiotherapy, the surgical option being used for tumor mass reduction,3-5. Overall survival ranges from 15% to80%, depending on the age of the patient, as well as on the characteristics and stage ofthe disease,4.Lymphomas are designated primary when they originate at and are confined to a givensite,3-5,7-11. When suchlesions appear in an intraventricular location and are hyperdense on CT, they can beconfused with colloid cysts, which are common at that site and exhibit similardensity.The list of differential diagnoses of expansile CNS lesions in imaging studies isextensive, including glioma, acute ischemia, inflammatory processes, and infectiousdiseases-14. Burkitt-like lymphomas are typically associated withinfection-HIV or the Epstein-Barr virus. They account for 2-3% of non-Hodgkin lymphomasin immunocompetent adults, being most common among the elderly-14. Burkitt-like lymphomas can affect the brain, intestines, skin,ovaries, kidneys, liver, and bone marrow. Chemotherapy is the most widely used treatment, although, evenwith treatment, survival is less than one year,14.Burkitt-like lymphomas are highly malignant, with cellular characteristics intermediatebetween those of diffuse non-Hodgkin large B-cell lymphoma and those of Burkittlymphoma,4,15,16. In the brain, lymphomas often occur after corticosteroid therapy,demyelinating diseases, or inflammatory disorders,16.The term "vanishing tumor" refers to a tumor that shows marked regression or disappears,with or without nonspecific therapy, and can recur or progress to new forms


Fast-growing congenital hepatic cysts with intracystic hemorrhage are rare in clinical practice. Additionally, the clinical manifestations of and laboratory and imaging findings for this condition are often nonspecific and are particularly difficult to differentiate from those of hepatobiliary cystadenoma and cystadenocarcinoma, thus posing great challenges for diagnosis and treatment. The 2 case reports presented here aim to analyze the diagnosis and treatment of 2 rare cases of congenital hepatic cysts with intracystic hemorrhage in the Chinese Han population to provide an important reference for the clinical diagnosis and treatment of this condition.These 2 case reports present 2 rare cases of congenital hepatic cysts with intracystic hemorrhage. Case 1 involved a 31-year-old patient with a very large, fast-growing hepatic cyst with intracystic hemorrhage and elevated carbohydrate antigen 199. Case 2 involved a patient with intense, paroxysmal right upper abdominal pain; computed tomography suggested a hepatic cyst with intracystic hemorrhage and possibly hepatobiliary cystadenoma.Both patients underwent liver resection. Postoperative follow-up showed that for both patients, the symptoms improved, the laboratory findings returned to normal levels, and the surgical outcomes were satisfactory.Liver resection is an ideal treatment for patients with congenital hepatic cysts with intracystic hemorrhage, and especially those with fast-growing, symptomatic hepatic cysts or hepatic cysts that are difficult to differentiate from hepatobiliary cystadenoma and cystadenocarcinoma. Congenital hepatic cysts may or may not be solitary. Hepatic cysts usually have a single compartment with a thin wall and clear intracystic fluid and without local thickening, nodules, or septa.Generally speaking, “hepatic cyst” refers to various hepatic cystic diseases, which may be parasitic or nonparasitic, depending on the cause. Parasitic hepatic cysts are mainly observed in hydatid liver disease, whereas nonparasitic hepatic cysts may be congenital, inflammatory, traumatic, or neoplastic. Congenital hepatic cysts are the most common, so the term “hepatic cyst” usually refers to these cysts, also known as simple hepatic cysts, which is a condition that may be related to congenital biliary developmental aberrations. Congenital hepatic cysts grow slowly, and most such cysts are asymptomatic and are detected during routine physical check-up. Congenital hepatic cysts are easy to diagnose and usually require no specific treatment except in cases of complications, such as intracystic hemorrhage, infection, cyst rupture, jaundice, or portal hypertension. Cases of fast-growing congenital hepatic cysts with intracystic hemorrhage are rare in clinical practice, accounting for approximately 10% of all cases of congenital hepatic cysts. The clinical manifestations and laboratory and imaging findings are often nonspecific and are particularly difficult to differentiate from those of hepatobiliary cystadenoma and cystadenocarcinoma, posing great challenges for diagnosis and treatment.,5The incidence of congenital hepatic cysts is approximately 2.5%, and these are more common in women and tend to occur in the right lobe.Recently, our center admitted 2 Chinese Han patients with rare cases of congenital hepatic cysts with intracystic hemorrhage and successfully performed liver resection in these patients. The 2 case reports below aim to provide an important reference for the clinical diagnosis and treatment of hepatic cysts with intracystic hemorrhage.2.2.1.A 31-year-old female Chinese Han patient was admitted to the hospital “6 years after surgery for hepatic cyst rupture, showing abdominal distension with yellowing of the skin and sclera for 1 month.” Six years earlier, the patient had sought medical attention for “abdominal pain after abdominal impact.” Examination suggested “liver rupture, ascites,” and laparotomy showed “hepatic cyst rupture”; hence, the patient underwent “hepatic cyst resection,” and postoperative pathology suggested a “hepatic cyst.” During the operation, the surgeon found 2 additional “hepatic cysts” of approximately 2 to 3 cm in size but decided to leave them untouched. One month before presentation, the patient had developed abdominal distension with yellowing of the skin and sclera. Computed tomography (CT) performed at a local hospital suggested an extremely large hepatic cyst, and magnetic resonance imaging (MRI) suggested possibly cancerous hepatic cystadenoma.The patient visited our hospital for further examination. Liver function tests showed total bilirubin 30.2 μmol/L, direct bilirubin 22.4 μmol/L, total bile acid 43.5 μmol/L, albumin 37 g/L, prealbumin 92 mg/L, and alanine aminotransferase 83 U/L. Hepatitis B surface antigen and antihepatitis C antibodies were negative. Tests for tumor markers showed that alpha-fetoprotein and carcinoembryonic antigen were negative. The carbohydrate antigen 199 (CA19-9) level was 298 U/mL. CT showed 2 masses (approximately 13.2 × 19.9 cm and 8.3 × 10.4 cm in size) of slightly low density. Septa were visible in the larger cyst, with enhancement of the cystic wall and septa and no significant enhancement inside the cyst  and the pathological process of hemoglobin destruction or absorption after intracystic hemorrhage, we made a diagnosis of a hepatic cyst with intracystic hemorrhage. We invite discussion of other reasonable diagnostic hypotheses that might explain the pathological changes observed in these 2 cases.In hepatic cysts, intracystic hemorrhage may be caused by excessive intracystic pressure and the ensuing necrosis of vascular epithelial tissue of the cystic wall.The clinical manifestations of hepatic cysts with intracystic hemorrhage are nonspecific, and patients with this condition may seek medical attention for a series of clinical manifestations and complications related to the size of the cyst, such as acute or chronic abdominal pain, a fast-growing mass during a regular check-up, and jaundice. The patient in Case 1 visited the hospital due to abdominal distension, mass, and yellowing of the skin and sclera. Fast-growing cysts must be differentiated from hepatobiliary cystadenoma and cystadenocarcinoma. The patient in Case 2 visited the hospital due to intense paroxysmal right upper abdominal pain, which should be differentiated from acute cholecystitis. In addition, a congenital hepatic cyst with intracystic hemorrhage should be differentiated from hydatid liver disease and liver abscess. reported clinical data for 4 patients with congenital hepatic cysts with intracystic hemorrhage, for whom MRI showed high signals on both T1 and T2 images, similar to what was observed in Case 2. Hagiwara et al reported 1 case of a cystic lesion, 8 cm in size, that was detected by an ultrasound B-mode scan. The cystic wall was thin and smooth, with scattered calcifications and small nodules. Enhanced CT and MRI showed mild enhancement, and MRI showed high T1 and T2 signals in the cystic fluid. The patient was considered to have a malignant tumor and underwent liver resection. During the operation, the cystic fluid was bloody, and postoperative pathological examination confirmed that the nodules were organized hematomas.The imaging and laboratory findings for a hepatic cyst with intracystic hemorrhage are nonspecific. T1-weighted MRI images may show high signals for hemorrhagic cysts and low signals for nonhemorrhagic cysts, and T2-weighted images may show high signals for both hemorrhagic cysts and nonhemorrhagic cysts. CT usually shows low density for nonhemorrhagic cysts and may show high density for hemorrhagic cysts in the acute stage and low density or mixed density after the acute stage. Vilgrain et al reported 1 case of a huge hepatic cyst with elevated serum CA19-9; the authors believed that the elevated CA19-9 may have been related to compression of the surrounding organs by the huge hepatic cyst and excessive secretion by the epithelial tissue in the thickened cystic wall. Horsmans determined CA19-9 levels in the serum and the cystic fluid in 1 case of cystadenoma and 1 case of cystadenocarcinoma and found that both serum CA19-9 and CA19-9 in the cystic fluid were elevated in cystadenoma and cystadenocarcinoma, whereas only CA19-9 in the cystic fluid was elevated in a congenital hepatic cyst. CA19-9 may not contribute to the differential diagnosis of congenital hepatic cysts with intracystic hemorrhage. Choi et al showed that CA19-9 and carcinoembryonic antigen levels in the cystic fluid were of little significance for differentiating between biliary cystadenoma and a simple hepatic cyst. Bertino et al,13 showed that serum CA19-9 levels were frequently elevated in chronic viral hepatitis and hepatic cirrhosis. Serum CA19-9 levels may be elevated due to compression of the surrounding organs by the huge hepatic cyst and the excessive secretion by epithelial tissue in the thickened cystic wall. Elevated cystic fluid CA19-9 levels may be detected in patients with elevated serum CA19-9 levels and intracystic hemorrhage, which may be caused by excessive intracystic pressure and ensuing necrosis of vascular epithelial tissue of the cystic wall. Thus, in Case 1, CA19-9 in the cystic fluid may be significantly elevated, and in Case 2, CA19-9 in the cystic fluid may be not high.Serum and cystic fluid CA19-9 levels may not contribute to distinguishing between congenital hepatic cysts with intracystic hemorrhage and cystadenoma or cystadenocarcinoma. In Case 1 in the present study, serum CA19-9 was significantly elevated (CA19-9 in the cystic fluid: NA), and in Case 2, serum CA19-9 was not high (CA19-9 in the cystic fluid: NA). Dinc et al Surgical approaches include laparoscopy and open cyst fenestration, liver resection, and liver transplantation. Hotta et al and Poggi et al each reported 1 case of spontaneous rupture of a huge hepatic cyst. In the present study, the 2 patients had obvious symptoms and were at risk of cyst rupture. The cysts could have been hepatobiliary cystadenoma or a malignancy such as cystadenocarcinoma, and the cysts were large. Thus, the patients underwent open resection. After the operations, the patients recovered well, with satisfactory outcomes. Martin et al showed that no patient requires a second surgery after liver resection of a symptomatic hepatic cyst. We believe that liver resection should be considered if cystadenoma or cystadenocarcinoma cannot be ruled out and in cases with turbid cystic fluid if it is not clear whether the cyst communicates with the bile duct or whether intracystic hemorrhage or infection is present. Liver transplantation is indicated in patients with poor liver function who cannot tolerate surgery and in patients whose FLV is too small.A congenital hepatic cyst with intracystic hemorrhage may be treated with a nonsurgical or surgical approach. Nonsurgical approaches mainly include percutaneous drainage, which is primarily used in confirmed cases in the elderly and in patients with a poor general condition. The relapse rate for nonsurgical approaches is relatively high; studies have reported that the relapse rate for percutaneous drainage is up to 100%.0.425 × height [cm]0.725 × 0.007184 m2) was first calculated, after which the SLV was calculated (formula: [706.2 × body surface area (m2) + 2.4] mL) and the SFLVR was calculated. In Asian populations, the safety threshold for donors for live liver transplantation is 30% of the SLV. For Case 1, the FLV of the right liver was 455 mL, and the SFLVR was 37.0%, whereas the FLV of the left liver was 575 mL, and the SFLVR was 46.7%. Thus, either the left or the right liver could have been preserved, but it was obviously safer to preserve the left liver. During the operation, the 3 right lobes were resected while effectively preserving the FLV. The patient recovered well after the operation.In Case 1, both cysts were large and located in the middle lobe, posing great challenges for liver resection. In addition, the patient had a history of surgery; before the operation, she had experienced liver damage , and her left hepatic vein was unclear on radiographic images. Due to these unfavorable surgical factors, it was extremely difficult to preserve her left hepatic lobe. In terms of preserving the FLV, cystectomy retained the maximum amount of liver tissue, which ensured postoperative recovery. However, the operation was associated with a long operation time, a large liver wound, and high blood loss, all of which increase surgical risk. In contrast, resection of 3 lobes simplified the operation. Thus, before the operation, an accurate assessment was performed, and the FLV was calculated to determine the surgical approach. According to Urata's method, the patient's body surface area (body weight [kg]4.For congenital hepatic cysts with intracystic hemorrhage, in patients with a poor general condition and elderly patients with a confirmed preoperative diagnosis, nonsurgical approaches can be used. In addition, for fast-growing, symptomatic, and huge hepatic cysts as well as cysts that are difficult to differentiate from hepatobiliary cystadenoma and cystadenocarcinoma, liver resection may be an ideal treatment if the benefits and risks are thoroughly evaluated before the operation.


Epidermoid cyst of the presacral space is a rare congenital lesion of ectodermal origin. Presacral epidermoid cysts have been previously reported in women, however are extremely rare in males. We report a case of presacral epidermoid cyst in a 55-year-old male who presented to our emergency department with acute urinary retention and history of chronic constipation. A non-contrast computed tomography scan was performed with suspicion of urolithiasis, which revealed a well circumscribed low attenuation presacral mass. Magnetic resonance imaging (MRI) of the pelvis was subsequently performed to further characterize the lesion. The mass was returning hypointense T1 and hyperintense T2 signals with few foci of T2 hypointensities. There was no post-contrast enhancement; however the lesion was showing diffusion restriction, appearing hyperintense on diffusion weighted imaging (DWI) and hypointense on the corresponding apparent diffusion coefficient map. These imaging features were consistent with an epidermoid cyst. Laparotomy with complete surgical excision of the cyst and preservation of the adjacent structures was performed. The histopathology confirmed the diagnosis. This case highlights the importance of MRI with additional sequences of diffusion weighted imaging which can be helpful to differentiate, to a good degree of confidence, among different pelvic tumors, therefore obviating the need of biopsy before surgery. Presacral epidermoid cyst is a rare congenital lesion of ectodermal origin. It develops from an ectodermal tissue remnant which is misplaced during embryogenesis due to faulty development of adjacent structures . PresacrWe present a case of a presacral epidermoid cyst in a male patient who presented with difficulty in passing urine and chronic constipation. The following case, to the best of our knowledge, is the third globally reported case of a presacral epidermoid cyst occurring in a male patient and an extremely unusual cause of chronic constipation and urinary retention , 5.A 55-year-old male patient came to the emergency room (ER) with complaints of difficulty in passing urine for the past eight hours. He also gave history of chronic constipation on and off for the past ten years. A non-contrast computed tomography scan of the kidneys, ureter and bladder (CT-KUB) was performed on a multi-detector scanner which revealed a large well-defined low attenuation presacral lesion measuring 13 x 11 cm, which was displacing the rectum anteriorly and urinary bladder superiorly with compression over its base, resulting in pressure effects over these viscera  of the abdomen and pelvis with additional diffusion weighted sequences was performed on a 1.5-Tesla scanner for further characterization of the lesion. The scan revealed a well-defined mass in the presacral space returning hypointense T1 and hyperintense T2 signals with few foci of T2 hypointensities  and hypointense on the corresponding apparent diffusion coefficient (ADC) map. There was an additional rare feature which we noticed in the diffusion weighted sequences in which differential restriction was seen in its dependent contents Figure .These MRI features were pathognomonic of an epidermoid cyst. Features did not favor a pelvic abscess, which also shows diffusion restriction on MRI, because the surrounding fat was clear, the adjacent viscera were normal in appearance, and there was no thickened, irregular ring-enhancement of the cyst wall or heterogenous signals within it in any of the sequences. There was also a lack of clinical history of fever, systemic illness, or previous surgery.The patient subsequently underwent an exploratory laparotomy and complete surgical excision of the cyst with preservation of the adjacent structures. On gross pathological examination, the cyst was filled with keratinous material. Histological sections examined revealed a benign cystic lesion lined by stratified squamous epithelium with keratinization on the luminal surface Figure .The underlying wall did not show any skin appendages or other teratomatous elements. There was no evidence of malignancy. Overall, the features were in accordance with those of a typical epidermoid cyst. No pus cells were noted to suggest secondary infection.Developmental cystic lesions arising in the presacral location include a multitude of tumors, including dermoid cysts, epidermoid cysts, chordomas, adrenal rest tumors, anterior sacral meningoceles, cystic hamartomas, tailgut or rectal duplication cysts . Bony dePresacral epidermoid cysts, on the other hand, are unique in the sense that they are the only developmental lesions that show diffusion restriction. Previous reports suggest that presacral epidermoid cysts are generally found in females of reproductive age group as incidental findings during gynecologic or obstetric related imaging . BecauseA previous study reported that presacral epidermoid cyst was preoperatively diagnosed using endorectal ultrasound, CT and conventional MRI without mentioning diffusion weighted imaging characteristic. They reported that fine needle aspiration was performed for diagnosis . GeneralAnother recent case report from Japan reported that presacral epidermoid cysts can be characterized using diffusion weighted imaging on MRI of the pelvis . There wThe closest differential of an epidermoid cyst on basis of diffusion restriction is a retrorectal pyogenic abscess. In such cases, there is often a clinical history of previous surgery, systemic illness or high-grade fever. Additionally, on imaging an abscess is usually ill-defined or loculated with heterogenous signals and presence of a thickened enhancing rim, intracavity fluid debris level or air specks. The surrounding fat may be infiltrated, the rectal wall may be thickened or there may be evidence of diverticulosis depending on the etiology. On DWI, there may be heterogenous diffusion restriction in an abscess which is frequently central. Reports have also suggested that pelvic abscesses generally have lower ADC values than pelvic cystic tumors .Presacral epidermoid cyst is a rare entity, and use of diffusion restriction to characterize them is propitious for preoperative planning. It is recommended that these lesions be surgically excised because of the risk of malignancy or secondary infection. Therefore, correct diagnosis and proper treatment for a presacral lesion is very important as inadequate primary surgery can lead to increased morbidity. It can also increase the risk of recurrence and can even cause complications such as fecal incontinence .With the advent and availability of higher magnetic strength MRI scanners, the use of diffusion weighted imaging in MRI for pelvic lesion characterization should be performed routinely. Epidermoid cysts in the presacral/retrorectal location are a rare entity which can be accurately diagnosed using diffusion weighted imaging. Therefore, DWI is a useful diagnostic tool in this regard as it obviates the need of biopsy. The characteristic appearance of an epidermoid cyst on MRI with diffusion weighted imaging warrants its use in diagnosis of pelvic cysts and should be made part of the standard protocol for pelvic imaging.


Astroblastoma is a controversial and an extremely rare central nervous system neoplasm. Although its histogenesis has been clarified recently, controversies exist regarding its cellular origin and validity as a distinct entity. Because of its extreme rarity and because its common features are shared with other glial neoplasms, this tumor is prone to misdiagnosis and remains challenging not only in terms of diagnosis and classification but also in the subsequent management. This case report describes a new case of astroblastoma. It discusses clinical, radiologic, pathological, and therapeutic features and differential diagnosis of this rare neoplasm, with a review of the recent literature.We report the case of an 8-year-old Moroccan girl who presented with a 1-year history of epileptic seizure, headache, and decreased visual acuity. Cranial magnetic resonance imaging revealed a right occipito-temporal mass. A tumor resection was performed and histological examination combined with immunohistochemical study confirmed the diagnosis of low-grade astroblastoma.Astroblastoma is a very rare primary brain tumor. Its diagnosis is often challenging because of the astroblastic aspects that can be found in astrocytic tumors, in ependymomas, and in non-neuroepithelial tumors. Considerable confusion surrounds its histogenesis and classification. The low incidence rate makes it difficult to conduct studies to examine tumor characteristics. WHO Classification of Tumours of the Central Nervous System [Astroblastoma is a controversial and an extremely rare central nervous system neoplasm –4. It acs System . Astrobls System , 17. Becs System , 4, 9.This case report describes a new case of a young girl with astroblastoma. It discusses clinical, radiologic, pathological, and therapeutic features and the differential diagnosis of this rare neoplasm, with a large review of the literature.IDH)1/2 gene analysis by polymerase chain reaction (PCR) sequencing did not reveal mutation. Based on these data, the diagnosis of low-grade astroblastoma was confirmed.We report the case of an 8-year-old Moroccan girl who presented with a 1-year history of epileptic seizure, headache, and decreased visual acuity. Cranial magnetic resonance imaging (MRI) revealed a right, occipito-temporal, voluminous, well-demarcated mass with a multicystic component and a characteristic bubbly appearance on T2-weighted images and hypointense heterogenous on T1-weighted images. After contrast injection, a strong contrast enhancement was observed. There was a peritumoral edema and a monoventricular left hydrocephaly Fig. . The tumAstroblastoma is one of the rarest central nervous system gliomas. It can occur in persons of any age, with a bimodal age distribution, with one peak in infancy (between 5 and 10 years) and the other one in young adults (between 21 and 30 years). The studies performed to date show a striking female preponderance with a male to female ratio of 1:11 , 18.The tumor usually presents as a well-circumscribed and superficial mass, usually supratentorial with occipital and frontal lobes the most frequently affected sites. However, tumor invasion has also been reported into corpus callosum, cerebellum, brain stem, and optic nerve , 2, 6, 8Clinical signs and symptoms are dependent on the location and size of the tumor and primarily consist of those associated with elevated intracranial pressure. Headache, seizures, vomiting, and focal neurologic deficits are the most commonly mentioned symptoms , 4, 7.Considerable confusion has surrounded the diagnosis, the histogenesis, and the classification of astroblastoma. Controversy still exists in the literature of the cell of origin of this neoplasm. Bailey and Bucy  believedOn radiographic examination, the lesions show a characteristic appearance that may aid the pathologist in making the diagnosis of astroblastoma. On MRI, it is almost exclusively seen supratentorially and is peripheral in location. It typically appears as a large, well-demarcated, lobulated mass. It often has solid and cystic components with a characteristic bubbly appearance in the solid component, which was believed to result from the tumor vascular architecture, with inhomogeneous contract enhancement and little vasogenic edema , 9, 18. On macroscopic examination, astroblastomas were described as superficial, well-demarcated, lobulated, solid, or cystic masses .On histologic examination, an astroblastoma is defined by the presence of perivascular pseudorosettes and prominent perivascular hyalinization. The perivascular pseudorosettes give the characteristic “cartwheel” appearance. They exhibit characteristic epithelioid cells with cytoplasmic processes having blunt-ended foot plates attached to the basal lamina of blood vessels , 6, 8, 9Because astroblastoma exhibits a highly variable biological behavior, a WHO grade has not been established yet. Based on morphology, Bonnin and Rubinstein  reportedImmunohistochemical features of astroblastoma have some variability throughout the literature. Immunostaining for GFAP is positive, lending support to the theory that the tumor cell is derived from an astrocyte cell line. Astroblasts also consistently stain positive with vimentin, suggesting derivation from a more primitive astroblast, and for S-100 protein. Other immunostains, such as neuron-specific enolase (NSE), EMA, cytokeratin (CK), and CAM 5.2, have had highly variable results in the current literature , 9.Apart from our case, 53 reported cases of astroblastoma are reviewed in this work. Epidemiological, clinical, radiologic, and immunohistochemical characteristics and grading are summarized in Table The diagnosis of astroblastoma is often difficult. In fact, astroblastic features are not unique to astroblastoma and can also be found in other tumors. Therefore, the combination of the radiologic and the histopathologic characteristics is necessary for making a correct diagnosis. The main differential diagnoses are ependymoma and angiocentric glioma , 9, 18. et al. [et al. [IDH 1/2 and TP53 mutations, which are known to be involved in the development of low-grade gliomas, was shown in astroblastomas [IDH1 mutation. P53 immunostaining was negative.Data on the molecular genetics of astroblastoma are rare and only recently available from the literature. A study by Brat et al.  demonstret al. , 4. Otheet al. . Shuangs [et al.  found lolastomas , 25. In Since astroblastomas are rare and tumor descriptions in the literature concern only individual cases or small collections of cases, optimal treatment protocols have not been established. Total resection is the best treatment. It provides excellent tumor control rates , 2. Subtet al. [n = 239). They found that older age, supratentorial location, and treatment prior to 1990 were poor prognostic factors. In addition, they thought the reason why patients with cerebellar tumors had better prognosis was that they might have earlier signs of increased intracranial pressure, leading to a quicker diagnosis and subsequently more timely treatment than their supratentorial counterparts. Similarly, better prognosis for patients diagnosed after 1990 might be related to multiple factors like advances in diagnostics and therapeutic modalities, such as MRI [Several investigators have found that astroblastoma prognosis may be predicted by the histology and extent of resection. The low-grade astroblastomas are thought to have a better prognosis than the high-grade ones. Their prognosis is similar to that of low-grade gliomas. High-grade astroblastoma prognosis corresponds to that of anaplastic astrocytomas and has been associated with recurrence and progression , 8. Ahmeet al.  presenteh as MRI .Astroblastoma is a very rare primary brain tumor. Its diagnosis is often challenging because of the astroblastic aspects that can be found in astrocytic tumors, in ependymomas, and in non-neuroepithelial tumors. Considerable confusion surrounds its histogenesis and classification. The low incidence rate makes it difficult to conduct studies to examine tumor characteristics.


Musculoskeletal tumours of the lower limbs especially malignant tumours are not common. The fibula is the site of primary bone tumours as reported in 2.4% of lower limb tumours with the proximal third being more frequently involved than the distal segment. Osteosarcoma is the most common primary malignant bone tumour of nonhaematopoietic origin, with distal fibular involvement in 0.47% of patients. The advances in imaging techniques and neo-adjuvant chemotherapy have now made it possible to accurately define the extent of tumour and plan limb salvage with tumour resection. The purpose of this case report is to highlight the successful outcome of limb salvage procedure with a five year follow up in an 11-year old boy with distal fibular osteosarcoma. Limb salvage surgery with distal fibulectomy and retention of the foot are a good alternative to radical amputation. This is a rare presentation and the management presents unique challenges.Musculoskeletal tumours of the lower limbs especially malignant tumours are not common. They are considered to exhibit lower mortality rates as compared to other sites. Synovial cell sarcoma, osteosarcoma and Ewing’s sarcoma are reported as the more common mesenchymal cancers of the lower limbs. The fibula is the site of primary bone tumours as reported in 2.4%, the proximal third being more frequently involved than the distal segment. It has been observed that malignancies involving the distal third of the fibula carry a better prognosis than proximal lesions. Osteosarcoma is the most common primary malignant bone tumour of nonhaematopoietic origin with the distal fibular localizations in 0.47 % of patients2. The advances in imaging techniques and adjuvant therapy have allowed the surgeon to accurately define the extent and therefore plan tumour resection rendering limb-salvage surgery as a feasible option. The purpose of this case report is to record the successful outcome of limb salvage procedure at five year follow up in an 11 year-old boy with distal fibular osteosarcoma.The natural history of aggressiveness of primary osteosarcomas has led to the consideration of amputation as the choice for local control of the tumour in the limb. With the advances in imaging techniques and neo-adjuvant chemotherapy, it is now possible to accurately define the extent and therefore plan limb salvage with tumour resection. However, the wider resections of the entire distal fibula can result in potential loss of ankle stability and therefore the need for reconstruction of a stable ankle joint and sufficient skin coverage of the area. Furthermore, the incidence of inadequate excision margins has been reported to be higher after limb-salvage surgery for fibular osteosarcomas compared with other appendicular lesions that are responsible for poor outcomes related to high rate of local recurrence and metastasis. Recent literature has challenged this view and reported no recurrence and no impact on survival in patients with fibular osteosarcomas who had undergone intentional marginal resection followed by adjuvant therapyAn 11 year-old boy was first seen with the chief compliant of pain and swelling at the lateral aspect of the left ankle of two months’ duration. There was no history of injury, fever, loss of appetite or weight loss. Local examination revealed an illdefined swelling of 5cm x 4cm at the lateral part of left ankle which was warm and tender to touch. There was terminal restriction of movement at the ankle joint with no evidence of distal neuro-vascular deficit. Radiographs revealed an expansile lytic lesion involving the distal third of fibula with epiphysis with some patchy sclerosis and cortical breach. The proximal extension of the marrow and cortex is about 22.5cm from the tip of the fibula .MRI showed an aggressive lesion involving the distal third of the right fibula with involvement of distal epiphysis, and with cortical breach and soft tissue extension . Chest CPatient underwent distal fibulectomy through a direct lateral longitudinal incision. At operation, the lesion was about 11 cm in diameter with involvement of flexor hallucis longus, peroneus brevis and the peroneal artery . About 1Sutures were removed after two weeks and patient was mobilised with non-weight axillary crutches. At 12 weeks, the external fixator was removed and fibreglass cast was applied and full weight bearing started. Post-operatively, chemotherapy with Doxorubicin and Cisplatin was administered every three months for four cycles. Patient was followed up at intervals of three weeks for the first three months, three monthly for one year, six monthly for two years and every year for five years. During his last follow up in December 2016, there is no pain or swelling and the ankle joint was stable. Radiograph showed valgus and talar shift but no sign of joint degeneration.  However,3. This mandates reconstruction of a stable ankle joint and the need for skin grafting or flap.The 1970’s witnessed the management of high grade osteosarcomas solely with amputation. This was attributed to the limitations in the imaging modalities that precluded accurate assessment of the real size of the tumour and its extension beyond the cortices. There is recent evidence in literature supporting limb salvage procedures as the main stay of treatment for high grade osteosarcomas. However, the wide resections of the entire distal fibula results in loss of ankle stability and inadequate soft tissue conditions such as insufficient skin coverage of the areaet al reported the outcome in 11 patients who underwent distal fibular resection for benign or malignant tumours with limb salvage surgery and different techniques of reconstruction. In this case series, seven patients recovered normal function, while four had reduced mobility. One patient developed lateral subluxation of the talus. However, all patients were free of pain and the functional and clinical outcomes were independent of the nature of the lesion2.A PubMed literature review on the surgical treatment of distal fibular tumours showed paucity of results with only case reports and small case series. This is related to the rarity of distal fibular tumours. A review by Capanna 4.Various surgical techniques for different distal fibular pathologies have been proposed. Recent advances in surgical techniques, chemotherapy and bioengineering technology for mechanical prosthesis have led to the introduction of alternatives to amputation. One of the frequently performed technique is distal fibular resection without reconstruction of the lateral side of the ankle. In these situations, ankle stability is obtained by either soft tissue and ligament reconstruction or tibiotalar arthrodesis. If reconstruction is followed after resection, the choice is using allografts, autografts or pedicled vascularised epiphyseal transfers using the ipsilateral proximal fibula. A prosthetic ankle joint replacement is an option5. However there are no specific guiding principles in the choice of the technique and there is need for long term studies and randomised controlled trials to develop protocols on this subject.All these surgical techniques however pose common complications such as varus instability, valgus collapse and limited ROM. Furthermore, the unique associated complications include donor site morbidity, lateral knee instability, peroneal nerve damage, incongruity between the articular surfaces of the ankle joint, and non-union with reconstruction with fibular head and occurrence of talar collapse which can occur after ankle joint replacement2. In the presence of the malignant tumour involving the lateral malleolus, fibular resection with or without reconstruction or prosthetic ankle replacement may be recommended. The lateral malleolus can be removed with remarkably little functional deficit. Post-operative chemotherapy plays an important role in the management of osteosarcoma to prevent micro-metastasis and local tumour recurrence with improved disease-free survival rates.With the given evidence, the authors conclude that sufficient evidence has emerged for limb salving surgeries for distal fibular tumours including the rare primary osteosarcoma with improved quality of life. Distal fibular osteosarcoma carries a good prognosis if sound oncological margins can be achieved during the initial surgery. In the presence of the malignant tumour not involving the lateral malleolus and located at least 2 cm proximal to the tibiotalar joint, meta-diaphyseal fibular resection and periosteal tibial stripping is performed, followed by arthrodesis between the remaining lateral malleolus and the distal tibia described as Capanna’s techniqueThe authors declare no conflicts of interest in the preparation of the manuscript.


Retinoblastoma is the most frequent childhood intraocular tumor. The aim of our study is to evaluate the clinical features and management of extra-ocular retinoblastoma in the Mohamed VI university hospital of Marrakech. Retrospective case series, the patient's records were reviewed for patient and tumor features, ocular management, histopathological findings, and patient survival. Over a period of three years, 35 eyes were diagnosed with retinoblastoma; 12 children (16 eyes) (46%) had extra-ocular retinoblastoma. Mean age was 27 months, 60% were males. Six cases had unilateral tumor, five bilateral and one case of trilateral retinoblastoma. There was no positive family history, proptosis was the mean mode of presentation  followed by staphyloma (25%) orbital cellulitis (25%) and hyphema. The median lag period was 18 months. On imaging and histopathological analysis, there was extrascleral involvement in 41.6%, involvement of orbital part of optic nerve (75%), of orbital muscles (50%) and eyelids in 16.6%. the surgical treatment included according to the degree of extension enucleation (75%) or exenteration (25%) associated to chemotherapy in all cases and one case of external beam radiation. There were 2 cases of orbital recurrence, one death and no metastases at 30 months follow-up.Orbital retinoblastoma still stands as a tall challenge requiring multi-modal and multi-disciplinary approach. Although the survival has increased over the last few years, lack of access to medical facilities, lack of education about the need for early medical attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world. Retinoblastoma is the most frequent childhood intraocular tumor with an approximately incidence of 1/15000-20000 birth in the world . In neglA retrospective case series of all children seen at the eye unit and the pediatric oncology unit of the university hospital Mohamed VI of Marrakech with the diagnosis of orbital retinoblastoma from November 2011 to June 2015 was determined from clinical records of the hospital. Data was compiled on epidemiological characteristics, clinical examination, investigations, histopathology findings, treatment given and their outcomes. The information collected included demographic features, laterality, family history of retinoblastoma, the initial sign noticed, the duration of lag period (the time between the onset of symptoms and diagnosis), any consultation taken in this period and the reason for the delayed presentation. Clinical examination was performed with complete ocular examination including dilated ophtalmoscopy with scleral indentation under general anesthesia. The posterior segment findings included tumor size and location, number of quadrants involved with tumor and extent of retinal detachment. The detail of imaging studies including eye ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) of the orbit and brain were recorded. The recorded details included tumor thickness, intra tumoral calcification and evidence of optic nerve, sclera and orbital invasion by the tumor. Ultrasound abdomen and X-ray chest view was done for metastasis analysis for all patients. The Reese Ellsworth classification and international classification of retinoblastoma were used for staging the tumors. The histopathological variables were documented: endophytic and exophytic growth, the size of the tumor, tumor necrosis, the degree of calcification, tumor differentiation, and the presence or absence of fibrosis. The extent of a tumor was established by assessing the involvement of different intraocular structures. Choroidal extension was expressed in progressive grades, optic nerve involvement was expressed in terms of tumor cell invasion of either prelaminar, laminar, or postlaminar optic nerve and involvement of the cut surgical end the optic nerve. We included in cases of extra-ocular retinoblastoma: patients presenting clinical or radiological detected orbital extension of an intraocular tumor, choroidal or extrascleral involvement, invasion of the optic nerve on histopathological evaluation of enuceated eyes and cases of orbital recurrence. Treatment given included surgery by enucleation or exenteration  or combined surgery chemotherapy and external beam radiation. Patients with suspected clinical and/or radiological extraocular retinoblastoma received neoadjuvant chemotherapy, defined as chemotherapy administered before enucleation to reduce tumor size. The patients who did not receive any treatment before enucleation were those who had only a histopathological involvement of the choroid or the optic nerve detected after surgery. The chemotherapy used included the recommended protocol of VEC  or VC (vincristine+ciclophosphamide), external beam radiation was performed in case of orbital recurrence. All patients were monitored for the control of the contra lateral eye and for orbital recurrence and metastatic disease by clinical examination, brain and orbit MRI every 3-6 months. Statistical analysis was performed using the statistics software SPSS for windows. Data are represented as means/SD when the distribution was normal and median with range when the distribution was not normal.During the study period a total of 35 eyes (26 patient) were diagnosed with retinoblastoma in our center, 12 patient (16eye) had an extra-ocular extension (46%). There were 60% males; none of our patients had positive family history. The disease was unilateral in 6 cases and bilateral in 5 patients, with one case of trilateral retinoblastoma. The average age of diagnosis was 27 months (range: 8months to 4years), however the age at presentation was higher in unilateral cases than bilateral. The initial mode of presentation was varied, there was proptosis  staphyloma (25%) orbital cellulitis (25%)  and hyphIn developed countries, retinoblastoma is usually diagnosed in its early intraocular stages leading to high chances for preservation of vision, globe and disease free survival of the patient. However, in developing countries, retinoblastoma is often diagnosed at a later stage with extraocular dissemination, thus leading to much lower rates of ocular salvage and patient survival –13. We pLeucocoria is the most common presenting sign of retinoblastoma followed by strabismus all over the world . The freWe do acknowledge the limitations of this study. The small numbers analyzed makes it difficult comparing our results with those from centers with larger series; similarly, it is impossible to make inferences for the whole population from this series. However the importance of the disease, being both sight and life threatening, renders the findings significant. We confirmed that intra orbital invasion is strongly associated with a delayed diagnosis due to various reasons. Many methods have been suggested for education of the public thus leading to early detection and a better prognosis for vision and life. Use of media campaigns to increase public awareness, incorporation of education program linked with national vaccination campaign, appropriate counseling of parents as not to abandon treatment and education programs for pediatricians and general ophthalmologists have all been suggested to avoid delays in diagnosis and management.In developing countries, retinoblastoma is often diagnosed at a later stage with extra-ocular dissemination;Longer lag time and denial of management by parents are responsible for the delayed diagnosis.Intra orbital invasion is strongly associated with a delayed diagnosis due to the unawareness about the seriousness of the disease, fear of enucleation and limited access to specialized center;Education of the public leads to early detection and a better prognosis for vision and life.


Cementoblastoma is a rare benign odontogenic neoplasm which is characterized by the proliferation of cellular cementum. Diagnosis of cementoblastoma is challenging because of its protracted clinical, radiographic features, and bland histological appearance; most often cementoblastoma is often confused with other cementum and bone originated lesions. The aim of this article is to overview/revisit, approach the diagnosis of cementoblastoma, and also present a unique radiographic appearance of a cementoblastoma lesion associated with an impacted tooth. The cementoblastoma was first described by Dewey in 1927 as an odontogenic tumor of mesenchymal origin .A 19-year-old male patient complained of swelling in the left body of mandible with an increase in size for the past two months. On clinical examination, an extraoral swelling presents in lower one-third of the face which measures about 3 × 3 cm in size. Intraoral swelling was associated with partially impacted 36. The swelling was well defined and firm-to-hard in consistency, with expansion of lingual and buccal cortex. Tenderness on palpation was noticed. On radiographical examination, OPG revealed a large, well-defined periapical radiolucency arising from the lateral root surface of an impacted permanent left mandibular first molar and second premolar. The lesion was surrounded by a thin, uniform radiopaque line as can be seen in Cementoblastomas are slow growing lesions with unlimited growth potential. They are odontogenic tumors and are derived from ectomesenchymal cells of the periodontium including cementoblasts. These tumors are commonly seen in children and young persons; males are more frequently affected than females, with more occurrences in mandible than maxilla. The tumor usually involves an erupted permanent first tooth , 4. Few The present case is an association with impacted mandibular first molar which has rarest occurrence for cementoblastoma in a 19 yrs male. These lesions are usually slow growing benign neoplasms. These lesions with unusual clinical and radiographic presentation can lead to a misdiagnosis. The clinicians as well as oral pathologists must bear in mind several possible differential diagnoses due to its unspecific nature. Surgical removal is the treatment of choice and postoperative follow-up is highly recommended.


The perinephric space, shaped as an inverted cone, sits between the anterior and posterior renal fasciae. It can play host to a variety of clinical conditions encountered daily in the reporting schedule for a radiologist. Lesions may be classified and diagnosed based on their imaging characteristics, location and distribution. A broad range of differential diagnoses can be attributed to pathology sitting within this space, often without clinical signs or symptoms. An understanding of commonly encountered conditions affecting the perinephric space, along with characteristic imaging findings, can illustrate and often narrow the likely diagnosis. The aim of this essay is to describe commonly encountered neoplastic and non-neoplastic entities involving the perinephric space and to describe their key imaging characteristics.Despite often a bulky disease, perinephric lymphoma does not produce obstruction or stenosis.• In primarily fatty masses, defects within the renal capsule likely represent angiomyolipoma.• Consider paraganglioma if biopsy is planned; biopsy may lead to catecholamine crisis.•  A diverse assortment of pathologies can present within the perinephric space. The aetiology of disorders seen on radiological imaging can be attributed to solid tumours, both benign and malignant; fluid collections; inflammatory masses; or proliferative syndromes. Appreciation of the close relationship between the perinephric space and adjacent retroperitoneal organs and planes can assist in localising the site of origin of pathologies and can help narrow the differential diagnosis. An understanding of the imaging characteristics of these pathologies will then enable a more accurate diagnosis and guidance of treatment.This pictorial essay will review and illustrate conditions commonly encountered within the perinephric space, outlining relevant anatomical landmarks, radiological signs and features, along with patterns of disease spread.Basic knowledge and understanding of the retroperitoneal anatomical spaces is essential to understanding disease presentation and patterns of spread. The retroperitoneum is divided into three distinct compartments: the perinephric space, anterior pararenal space and posterior pararenal space  Fig. 1)1).Fig. 1The perinephric space is formed by adjacent bordering pararenal spaces anteriorly and posteriorly. The perinephric space contains the kidneys, proximal ureters, adrenal glands, fat, bridging septa (Kunin’s septa), vessels and lymphatics . The thiThe perinephric space appears as an inverted cone, corresponding to the embryological migration of the renal system from the pelvis  Figs. 22 and 3.FThe pararenal spaces lie superficial to the perinephric space. The anterior pararenal space lies between the parietal peritoneum and Gerota’s fascia, and contains the pancreas, duodenum, and ascending and descending colon. The posterior pararenal space sits between Zuckerkandl’s fascia and the transversalis fascia, and contains no organs, only fat pads. As the inter-fascial plane continues towards the iliac fascia, the space produced between Gerota’s fascia and Zuckerkandl’s fascia is known as the infrarenal space , 3.As the perinephric space lies centrally in the retroperitoneal space, disease manifestation and transmission of pathology, such as that of malignancy, can traverse fascial planes, as can rapidly accumulating fluid collections . Slowly A perinephric haematoma is typically described as fluid that is confined within the dense, collagenous Gerota’s fascia . TraumatAcute haematoma has a higher attenuation value than renal parenchyma on unenhanced CT imaging Fig.  and a loUrine leaks are most frequently secondary to trauma Fig. , either The transition of acute urinary extravasation to chronic urinoma formation is secondary to encapsulated urine that leaks and collects within the boundaries of the anterior and posterior renal fascia, producing a proinflammatory and fibrosing state. A urinoma may present with gross fibrous thickening and an enhancing rim and septa on contrast-enhanced CT .Lymphangiomas are congenital benign cystic tumours that most frequently occur within the neck, although up to 5% occur within the abdominal cavity, with the mesentery and retroperitoneum the most common abdominal sites , 15. WheSonographically, the lesions appear as anechoic fluid divided by thin-walled septa Fig. , with noRenal lymphangiomatosis is a rare condition of unknown aetiology. It is believed to arise secondary to poor renal lymphatic communication during embryological development. It is characterised by small peri-pelvic and capsular cystic changes that may be uni- or multilocular. The imaging appearance is similar to lymphangiomas, apart from the location as a subcapsular pathology rather than arising within the perinephric space. The condition may be unilateral or bilateral. It can occur at any age and has no predilection for sex. Diagnosis tends to focus on histology, as radiological signs are often indeterminate for lymphangioma . TreatmeAcute pancreatitis is accompanied by fluid collections in around 43% of cases , with riIn 40-69% of pseudocysts, a communication will remain with the pancreatic duct . MR cholAbscess within the perinephric space can present a diagnostic challenge. Depending on chronicity, aetiology and complications, it may mimic a tumour radiologically if chronic or may mandate evacuation and immediate treatment if acute. Predisposing factors for perinephric abscess include diabetes, pyelonephritis, urinary tract calculi and immunosuppression. Acute perinephric abscess is accompanied by fever, flank pain and leukocytosis. CT and MRI findings are non-specific and include thickening of the adjacent fascia, obliteration of perinephric fat planes and fat stranding , 30. ConIn cases of emphysematous infection, CT can visualise gas extending from the kidney into the perinephric space. Xanthogranulomatous pyelonephritis, a rare progressively destructive process with lipid-rich macrophages replacing degenerative renal tissue, can also present with abscesses within the perinephric space as the inflammatory process spreads . ImagingPerinephric bridging septa (PBS) are composed of numerous fibrous lamellae which traverse the perinephric fat, extending from the renal capsule to the renal fascia and to other points on the posterolateral aspect of the capsule Fig. . They seRetroperitoneal fibrosis is characterised by the proliferation of fibro-inflammatory tissue, usually surrounding the infrarenal portion of the great vessels  . PerinepRenal lymphoma is rarely seen as a primary renal lesion, with incidence of less than 1% . Renal iAt MRI, the masses have an intermediate signal on both T1- and T2-weighted images, although a high T2 signal may occur occasionally . The enhIt is usually seen as a spectrum of multi-system lymphoproliferative conditions. The genitourinary system is the second most commonly affected anatomic entity for extra-nodal spread of lymphoma, next to the haematopoietic and reticuloendothelial organs , 47. CT Retroperitoneal sarcomas are rare tumours, and a number of subtypes may occur, the most common of which are liposarcoma and Leiomyosarcoma . Due to Liposarcoma remains the most common primary retroperitoneal malignancy and the second most common presentation of soft tissue sarcoma after pleomorphic undifferentiated sarcoma , 54. FouIn well-differentiated primarily fatty masses, it is important to search for a defect within the renal capsule, as when a lesion is present, it most likely represents an angiomyolipoma rather than a sarcoma . The adrLeiomyosarcoma is the second most common primary retroperitoneal malignancy in adults . These tAngiomyolipoma (AML) is a mesenchymal neoplasm composed of blood vessels, smooth muscle and adipose tissue . AML occSolitary fibrous tumour (SFT) of the kidney is a rare entity. Initially believed to be solely a pleural malignancy, referred to as “benign fibrous mesothelioma”, SFTs can be seen anywhere throughout the body , 73. MosDesmoid tumours or aggressive fibromatosis develop from musculoaponeurotic tissue and can be sporadic or familial, such as familial adenomatous polyposis (FAP) or Gardner syndrome . Desmoid123I-MIBG) can achieve specificity of up to 99% and sensitivity of 77–90% in the diagnosis of PCC, and remains the most important nuclear imagining tool currently in service [18F]fluorodeoxy-D-glucose (FDG) when taken in by metabolically active adrenal malignancies, can localise and correlate with structural anatomy yet currently lacks the specificity required as an initial investigation of choice [18F-FDOPA -based PET/CT has shown sensitivity profiles close to 95% in diagnosing PCCs and abdominal paragangliomas, reaching 100% concordance with MRI for the detection of PCCs [Phaeochromocytomas (PCCs) and abdominal paragangliomas are rare catecholamine-producing tumours. Incidence patterns show that men and women are affected equally, in the fourth to fifth decades of life . Most 9. When mo service . It is if choice . 18F-FDONeurofibroma is a benign nerve sheath tumour more often seen as an isolated entity (90%) rather than in conjunction with associated genetic conditions such as neurofibromatosis. They consist of Schwann cells, fibroblasts and rich networks of collagen fibres. More common in men, its incidence occurs in young to middle-aged men, 20 to 40 years of age . On CT iRenal cell carcinoma (RCC) remains the most common presentation of malignancy arising from the kidney, accounting for 2–3% of all adult malignancies, with a median age at diagnosis of 65 years and a significant male predilection , 96. ThePrimary extra-gastrointestinal stromal tumour (EGIST) in perirenal locations is extremely rare. Gastrointestinal stromal tumour (GIST) is a non-epithelial neoplasm arising from the muscularis propria layer  of the GI tract, with characteristic exophytic growth patterns , 105. AmThe perinephric space is an unusual site for secondary spread of malignancy. Imaging findings vary depending on the type of primary tumour. Most perinephric metastases present as multiple discrete soft tissue masses Fig. . PulmonaErdheim-Chester disease (lipoid granulomatosis) is a systemic non-Langerhans cell histiocytosis of unknown aetiology . It affeRosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a proliferative benign disorder characterised by proliferation of histiocytes, with clinical features suggestive of lymphoma-like disease. The disease process tends to affect children and young adults, with extra-nodal disease present in approximately 40% . Common


Report case (s) relevant aspects: Man, 27 years old, complaining of acute testicular pain by 2 hours in the remaining left testicle. Denies fever, lower urinary tract symptoms such as dysuria, urinary frequency, concommitant or prior urethral discharge to the painful condition. He underwent right orchiectomy 13 years ago by testicular torsion. He is a chronic user of cocaine for 15 years and during the last three days the drug use was continuous and intense.Proposed premise substantiating case (s) description:Initial diagnostic hypothesis:Syndromic:Main Etiologic (testicular torsion)Secondary Etiologic (acute orchiepididymitis)Acute Scrotum Syndrome (SEA) Briefly delineates what might it add? Lines of research That Could be Addressed: In this challenging clinical case we presented an alternative and new etiologic diangosis for the acute scrotum which the main etiologic factor remains testicular torsion. This new diangosis is acute testicular ischemia as a complication of cocaine abuse. Male patient, 27 years-old, got into the emergency room of the local hospital complaining of severe testicular pain that started 2 hours ago and has worsened since then. Moreover, he reported having made use of large amounts of cocaine in the last three days, with the last dose consumed by nasal aspiration within the last 3 hours. He states, as personal history, that underwent orchiectomy 13 years ago due to testicular torsion.On admission the patient was conscious, alert, oriented and hectic. Pulse=HR=125 beats/minute; Respiratory Rate=22 breaths/minute and blood pressure=150 × 100mm Hg.Urological physical examination: penis with normal characters. Scrotal pouch showing remaining left hard testicle, slightly increased in volume and moderate pain on palpation. There were no signs of testicular tumors on palpation, or swelling in the scrotal wall .After initial approach, the patient was referred immediately to Ultrasonography Unit, whose examination showed reduced blood flow in the left testicle, as well as heterogeneous echo-texture.With these sonographic findings the most likely diagnosis was “asynchronous testicular torsion”, and the patient immediately was referred for surgical exploration.After the left testicle surgical exposure it was noted that there was no testicular torsion or twisting of the spermatic cord, as well as spermatic cord edema. However, the testis was increased in volume and hardened on palpation. The surface was white-pearlescent interspersed with several areas of cyanosis and ischemia, suggesting severe vasoconstriction of the testicular vasculature .Ventilatory support and hydration was started and urological treatment consisted of immersing the testis in warm saline, followed by surgical fixation, avoiding future testicular torsion.After 20 minutes of soaking it in warm saline, its surface seemed less cyanotic and having a softer consistency.Next, scrotal wall was closed in layers with absorbable sutures. Pain improved only after cocaine poisoning has been controlled, about 12 hours later.Drug testing to identify the poisoning of cocaine was not performed. The diagnosis was eminently clinical, since the clinical history and physical examination confirmed the hypothesis of acute drug intoxication.Precepts, clinical and basic reasoning supporting the case (s) and the hypothesis raised scenario. Why is it important and is being Reported?After an extensive review of literature and as far as we know, this is the first published case report in the literature related to Acute Scrotum Syndrome caused by ischemia. Furthermore, its main etiology is cocaine abuse, which presents with clinical features that mimic the twist testis or spermatic cord. In fact, it is related to vasoconstriction by chronic cocaine use, worsened with the intensive and acute drug use in the last three days.It is important to be reported because it can become a differential diagnosis of acute scrotum syndrome. Physicians should consider in the differential diagnosis, since the use of illicit drugs, such as cocaine, is quite prevalent among adolescents and young adults around the world.Discussion and future perspectives: what it might add and how does it relate to the current literature‘Take - home message’ - lessons learnedThe sudden onset of clinical symptoms, within a short period of time, scrotal pain or the elements of its contents, with or without increase in scrotal volume is an urologic emergency. The typical presentation is known as Acute Scrotum Syndrome and usually presents in adolescents and young adults. However, some cases are described in newborns .The spectrum of conditions affecting the scrotum and its content ranges from incidental findings to acute pathologic events that require diagnosis and emergency treatment.The most common differential diagnoses are twisting of the spermatic cord, testicular or appendix torsion, epididymitis, orchitis, inguinal hernia, local trauma, sexual abuse, testicular tumor, scrotal idiopathic edema, cellulitis, vasculitis among others and the ultrasound-Doppler examination is important tool for elucidate the etiologic diagnosis .A careful history and physical examination are of vital importance for the correct diagnosis. In addition, depending on the underlying disease that causes Acute Scrotum Syndrome, time is a critical factor for treatment success.Testicular Torsion is a condition which must always be considered in cases of acute scrotum syndrome and orchiectomy may be the final result of diagnosis and treatment delay.This is a 27 year-old man diagnosed with Acute Scrotum syndrome with typical characteristics presented by the acute onset of the history of scrotal pain. The patient had a similar episode of acute scrotum 13 years ago due to a spermatic cord torsion, which resulted in right orchiectomy when he was only 13 years old.Knowing that the current acute symptom might have the same etiology, we were asked for scrotal ultrasound-doppler, which was made in less than 20 minutes. The sonographic/doppler diagnosis was reduced blood flow to the remaining testicle, strongly suggesting the diagnosis of torsion of the spermatic cord or testis. Thus, the patient was immediately taken to the surgical scrotal exploration  and 2.From the intraoperative clinical findings it was necessary to return to clinical history to discover the true etiology of the present Acute Scrotum Syndrome.During the examination of the patient in the emergency room the patient had altered vital signs such as tachycardia, tachypnea and psychomotor agitation, which improved after the use of sedatives in pre-and intraoperative periods.Testicular pain became more intense when he began to abuse cocaine three hours before seeking medical help. Coincidentally, he said that the pain worsened than ever after cocaine abuse.The action of cocaine in testicular circulation is very little reported in the literature. In 1999, Li and colleagues reported the effects of cocaine on testicular circulation in rats , 4. The Yang and colleagues in 2006 reported that the use of cocaine in rats resulted in prolonged vasoconstriction in testicular vessels and the effects of cocaine in the testis, as infertility, may be due to ischemia or ischemia-reperfusion injury of the testis .In humans, the relationship between testicular pain and ischemia induced by chronic use of cocaine was initially suggested by Neynaber and colleagues in a case report of Wegener’s granulomatosis. However, testicular pain in this case was part of the clinical syndrome, where other organs were affected simultaneously. It was described a case of a young man (22 years old-cocaine addict) who developed vasculitis with involvement of multiple organs such as the upper and lower respiratory tract, kidney, testicles and skin as well as laboratory abnormalities. All these characteristics lead to a diagnosis of Wegener’s granulomatosis. The involvement of multiple organs is typical of this syndrome; however, testicular involvement is very rare; it was believed that cocaine abuse had stimulated the development of systemic granulomatous vasculitis .Doherty and colleagues reported the patient’s history with acute testicular pain that mimicked the clinical condition of torsion of the spermatic cord.Ultrasound examination revealed impaired testicular blood flow compatible with the main hypothesis and the patient underwent emergency scrotal exploration. However, it was not found torsion of the spermatic cord. Reviewing the clinical history, the authors observed that the patient was daily user of metanphetamin and had used the drug one hour before starting pain in the acute scrotum syndrome . The desIn a study that evaluated the anatomical aspects of the epididymis and tunica vaginalis in patients with testicular torsion, Favorite and colleagues  found thIn this case, although we found no evidence of testicular torsion in the surgical approach, we proceeded the fixation as a protective procedure justified by his past history of testicular torsion.As differential diagnosis we remember that Polyarteritis nodosa (PAN) is a vasculitis of immune origin that can cause clinical involvement of testicular pain in 2–18% of cases. Testicular pain is one of 10 criteria to make the diagnosis of PAN according to the American College of Rheumatology (This is the first case described in the literature that presents with clinical features that mimic testicular torsion of the spermatic cord or without other systemic symptoms. Unlike other cases in the literature, this patient had testicular pain as the only symptom of acute scrotum syndrome.The effects of the use and abuse of cocaine are well described in the literature. Patients may present tachycardia, hypertension, and psychomotor agitation. Concerning Central Nervous System symptoms, patients may have seizures, ischemic or hemorrhagic stroke and its clinical consequences. In the cardiovascular system, they may present hypertension and tachycardia, cardiac arrhythmias, myocardial infarction even in young patients without atherosclerosis. The respiratory system may have chronic rhinitis, nasal septum perforation, oropharyngeal ulcers and pulmonary infarction. In the digestive system one can find ulcerations and bowel infarction and ischemic colitis as well. The changes in liver, kidney and skin are also predominantly related to vasoconstriction.The final message this case report teaches us is that we must consider testicular isquemia as a differential diagnosis of acute scrotum syndrome in patients under cocaine intoxication.