Glomus tumors of the hand are rare, benign but debilitating neoplasms arising from the neuromyoarterial glomus body. They may present a diagnostic dilemma, and take years with multiple consultations and investigations before an appropriate diagnosis is made, but once a diagnosis is made, surgical excision is curative.This is a case presentation of a 35-year-old African man who presented with complex regional pain syndrome of his left upper extremity, whose genesis was found to be a glomus tumor of the pulp of his left middle finger. Surgical excision resulted in resolution of the chronic regional pain syndrome and a return to a normal lifestyle. Chronic regional pain syndrome is a rare presentation of a glomus tumor, which has only been previously reported in patients with neurofibromatosis type 1, and one patient who did not have neurofibromatosis.Patients with glomus tumors may spend many years in pain and distress because of misdiagnosis. Sensitization and education of both the public and health care workers will help in early diagnosis and treatment of this otherwise potentially disabling pathology for which surgical excision is curative. Glomus tumors are rare benign hamartomas that account for 1 to 5 % of soft tumors of the hand. They arise from normal glomus apparatus located in subcutaneous tissue. The glomus body is a contractile neuromyoarterial receptor that controls blood pressure and temperature by regulating peripheral blood flow. Normal glomus bodies are distributed throughout the body in the reticular dermis, but are concentrated in the tips of digits, especially subungually [The classic symptom presentation is a triad of pinpoint tenderness, paroxysmal pain and hypersensitivity to cold . ChangesWe present the case report of a 35-year-old African man who presented with an 8-year history of severe left upper extremity pain and paresthesia and exquisite middle finger pain; these symptoms presented a diagnostic dilemma. Surgical exploration of his finger revealed a glomus tumor, and surgical excision led to complete resolution of all his symptoms.A 35-year-old African man presented with an 8-year history of left upper limb pain. The pain was so severe that he was unable to sleep at night. He complained of almost incessant pain and paresthesia from the left side of his neck, and along his entire left upper limb. Touching him anywhere on his limb and neck would elicit a burning pain that radiated along his entire upper extremity. There was no antecedent history of trauma, and he did not have any features of neurofibromatosis type 1.On further probing, he reported that the pain had initially started from the tip of his left middle fingertip. Over time, the pain had grown worse, and extended to involve the entire limb.The pain was aggravated by cold weather, lifting of heavy weights with his left hand and direct pressure or trauma to his left middle finger. It was relieved by rubber band tourniquet (self-applied) and by rubbing either side of his finger on a rough cloth or other rough surface. During the 8-year period, he was managed for multiple different diagnoses including neurogenic pain, neuroma, gout and thoracic outlet syndrome. He underwent multiple diagnostic tests including computed tomography (CT) scan and magnetic resonance imaging (MRI) imaging of his spine and brachial plexus. He had a total of 12 stellate ganglion blocks administered over the 8 years without a resolution of symptoms.On examination, he had neck tenderness along the brachial plexus roots, and along his entire left upper limb. His left middle finger was visibly thickened and dry, with exquisite tenderness over the entire pulp, precluding further examination.A diagnosis of chronic regional pain syndrome (CRPS), most likely secondary to a glomus tumor, was made. Previous MRI images were inconclusive, and we did not see the need to get new ones. We proposed an exploration of his left middle fingertip, and informed him that the finding and excision of a glomus tumor would be curative.He consented to surgery, which was performed under a regional block and tourniquet, under loupe magnification. A 10×6 mm tumor was found adjacent to bone and excised  or sudomotor abnormalities, motor symptoms or trophic changes on the affected limb. In our patient, the initial insult was an undiagnosed glomus tumor that was repeatedly stimulated; he maladjusted for the resultant pain, causing him to experience excessive pain and hyperesthesia involving his entire upper limb. His pain was so severe and so extensively distributed over his upper extremity, that he had several MRIs and stellate ganglion blocks in an attempt to control the pain. He had trophic changes over his fingertips, most pronounced on his finger with the glomus tumor.Thus, for most of the 8 years, he was managed for CRPS that progressively worsened. At the time of his initial visit to our institution, deciphering the initial pathology was nearly impossible. However, a careful review of the chronology of the illness pointed to the likelihood of a glomus tumor. Surgical exploration was performed based on clinical suspicion, as accompanying MRIs did not reveal any pathology in his fingertip.We found one case report of CRPS in a patient with a glomus tumor without neurofibromatosis type 1 in the English literature; all other reports were of patients with neurofibromatosis type 1 , 5. PatiThe diagnosis of a glomus tumor may be aided by the use of several imaging modalities including plain radiography, Doppler ultrasonography and MRI. Plain radiographs may detect some concavity in the bone in patients with tumors that are adjacent to bone and present for a significant duration of time. Vandenberghe and De Smet described a technique in which lateral finger radiographs of two opposing fingertips pointing towards each other are taken, allowing for a comparative view of both fingertips; thus, even subtle cortical scalloping of the affected side can be detected. This sign, however, is present in only 22 % of patients .CT scan imaging is not a useful modality for diagnosis. In expert hands, ultrasonography especially with color Doppler may identify these lesions.High-resolution MRI is a sensitive but not specific diagnostic tool; it is capable of detecting lesions as small as 1 mm. MRI is therefore a useful radiological adjunct to clinical examination, especially in those cases with nonspecific symptoms. However, an MRI does not always show a glomus tumor, and a strong clinical suspicion should therefore lead to surgical exploration even in the face of a negative MRI, as was the case in our patient [Histological examination of submitted tissue provides the definitive diagnosis. Glomus tumors are made of three primary cell/tissue types: glomus cells, vasculature and smooth muscle cells. There are three histological subtypes, which are based on the predominant cellular pattern within each tumor. Solid glomus tumors have poor vasculature and a scant smooth muscle component, whereas glomangioma tumors have a prominent vascular component. Glomangiomyoma tumors have prominent vascular and smooth muscle components. Solid glomus tumor is the most common variant 75 %) followed by glomangioma (20 %) and glomangiomyoma (5 %) [5 % folloTreatment of glomus tumors is by surgical excision. The tumor is ideally excised along with its capsule Fig. ; failureRecurrence rates of between 1 % and 8 % have been reported after surgical excision. Recurrence within weeks to months of surgery is thought to be due to inadequate excision, while recurrence occurring more than 2 years after surgery is regarded as the result of new tumors [Patients with glomus tumors may spend many years in pain and distress because of misdiagnosis. Unnecessary and costly investigations add to the delay in the correct diagnosis and appropriate treatment. Sensitization and education of both the public and health care workers will help in early diagnosis and treatment of this otherwise potentially disabling pathology, which can be cured by surgical excision. The treatment of CRPS is complex and difficult. Although rare, this report affirms that clinicians can add glomus tumor to the differential diagnoses of causes of CRPS.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. The care and reporting conform fully to the Helsinki Declaration.


Herein, we report a case of a gastrointestinal stromal tumor (GIST) at the esophagogastric junction (EGJ) that was successfully treated by a laparoscopic wedge resection (LWR) after dissection of the seromuscular layer around the tumor to prevent postoperative deformities and stenosis of the EGJ. Subsequently, the abdominal esophagus was wrapped by the gastric fornix according to Dor’s method in order to prevent reflux esophagitis after surgery.A 71-year-old female patient was admitted with a diagnosis of a GIST (23 × 20 × 20 mm) at the EGJ. We performed the abovementioned operation.Gastroduodenal endoscopic examination revealed no deformity or stenosis of the EGJ at 6 months after the operation. The patient has not experienced any reflux symptoms. Tumor recurrence was not noted 26 months after the operation.This procedure is useful in preventing the deformity and stenosis of the EGJ as well as postoperative reflux esophagitis. Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. About 60 % of all GISTs occur in the stomach, 30 % in the small intestine, and 10 % in the esophagus, colon, and rectum –4. SurgiRecently, laparoscopic wedge resection (LWR) is being performed with greater frequency for the treatment of GIST of the stomach because it is less invasive than other procedures. Furthermore, single incisional laparoscopic local gastrectomy for gastric GIST has been reported . HoweverWe report a case of a gastric GIST at the EGJ that was successfully treated by LWR with Dor’s fundoplication after dissection of the seromuscular layer around the tumor.A 71-year-old female patient was admitted to our hospital with a gastric mass incidentally identified by gastroduodenal endoscopic examination during screening. No physical abnormalities were observed. Laboratory tests including hematologic and biochemical analyses revealed no abnormalities.A gastroduodenal endoscopic examination revealed the presence of a submucosal tumor at the greater curvature of the EGJ Fig. . An enhaWe performed LWR after laparoscopic dissection of the seromuscular layer around the tumor to prevent postoperative deformities and stenosis of the EGJ. Our procedure minimized the area of the gastric wall to be removed and did not expose the peritoneal cavity to any intragastric content Fig. .Fig. 2ScAfter a 12-mm trocar was placed at the umbilicus, the laparoscope was inserted into the abdominal cavity. Under laparoscopic view, two additional ports (10 mm) were placed at both outer edges of the right and left abdominal rectus muscle in the upper abdomen and one additional 5-mm trocar was inserted in the subcostal region. The tumor was easily identified at the EGJ as a protruding mass covered with adipose tissue. Mobilization of the EGJ was accomplished laparoscopically. We exposed the anterior wall of the abdominal esophagus and revealed a tumor attached to its left wall. We isolated the adventitia of the abdominal esophagus and seromuscular layer of the stomach and identified the tumor base Fig. . After dThe LWR for the gastric GIST was performed with an Endo GIA linear stapler  Fig. . Then, tThe resected specimen was extracted through the umbilical trocar site using an Endocatch bag. The operation time was 171 min. Gross findings of the tumor resected by LWR showed that the tumor capsule was neither injured nor ruptured during surgery.By pathologic examination of the resected specimen, the tumor was diagnosed as a gastric GIST with a negative margin. The dimension of the tumor was 26 × 24 × 24 mm. The tumor was found to be composed of spindle-shaped tumor cells. Immunohistochemical examination showed that the tumor cells were strongly positive for CD34, c-kit, and DOG1; a weakly positive for alpha-smooth muscle actin; and negative for S100. The mitotic count was just 1/50 HPF; therefore, according to the Fletcher Risk Table, the GIST was classified as a low-risk malignancy.There were no postoperative complications, and the patient was discharged per her request 10 days after the operation. Gastroduodenal endoscopic examination revealed no deformity or stenosis of the EGJ at 6 months after the operation Fig. . The patGISTs originate from the gastrointestinal wall as submucosal tumors. In GIST, the frequency of lymph node metastasis is low. LWR is commonly used for the treatment of gastric GISTs because it is less invasive than open surgery . DespiteThree issues must be resolved for LWR to become the standard procedure for GIST close to the EGJ: (1) how to prevent the disturbance of food flow after LWR, (2) how to protect the abdominal cavity from exposure to intragastric contents during the procedure, and (3) how to prevent reflux esophagitis after the procedure.With LWR for GISTs close to the EGJ, the resected specimen tends to be large because of limitations in the manipulation range of the stapling device. The primary goal of surgical treatment for GISTs is to achieve resection of the tumor with a negative margin . RecentlBoth combined laparoendoscopic approaches and PEIGS involve the risk of exposing intragastric contents to the abdominal cavity. These intragastric contents may contain malignant cells and bacteria. Waterman et al. reported a case of peritoneal dissemination of GIST during surgery . TherefoThe additional surgical procedure to avoid reflux esophagitis after LWR becomes necessary when the GIST is located adjacent to the EGJ. After resecting the tumor at the EGJ, the cardiac sphincter system and the angle of His may be destroyed. In our case, the muscle layer of the abdominal esophagus was exposed to mobilize the GIST. We wrapped the front surface of abdominal esophagus with the gastric wall of the gastric fornix, and Dor’s method, rather than Nissen’s or Toupet’s method, was used to reinforce the exposed front surface of the abdominal esophagus and to prevent reflux esophagitis. The patient did not experience any reflux symptoms after surgery.Herein, we report the case of a gastric GIST at the EGJ that was successfully treated by LWR after laparoscopic dissection of the seromuscular layer. This procedure is useful for the treatment of GISTs at the EGJ.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The preoperative diagnosis of gallstone ileus is challenging due to the variability of its presentation, often resulting in late diagnosis. Controversy remains regarding the management of gallstone ileus; surgery is the standard treatment, but also less invasive approaches have proven to be successful. We present an unusual case of gallstone ileus and its conservative treatment.We describe the case of a 49-year-old Caucasian woman with a bowel sub-occlusion, treated conservatively. The imaging technique  led to a diagnosis of gallstones ileus. A surgical intervention was not performed. Instead, she underwent extracorporeal shock-wave lithotripsy to fragment the stones, mechanical intestinal dilatation for ileocolic stenosis and endoscopic removal of the gallstone. The presence of an apricot shell contributed to the bowel occlusion and was removed. The intervention was successful and without complications.Given the variability of the gallstone ileus presentation, surgery could not be the only treatment for our patient. In our case report, we show that colonoscopy could be a non-invasive approach that allows for diagnosis and treatment at the same time. The available data do not show a higher rate of recurrent biliary disease in cases where this method has been used, therefore in select patients, a conservative treatment could be an effective solution. Gallstone ileus is a rare mechanical bowel obstruction caused by the transition of a gallstone in the gastrointestinal tract through a biliary-enteric fistula, or following endoscopic retrograde cholangiopancreatography (ERCP), which occurs in 1 to 3% of all cases of mechanical ileus , howeverA 49-year-old Caucasian women presented to our department with a one-day history of nausea, vomiting and abdominal pain. Approximately two months previously, she had presented with symptoms of biliary colic, which was subsequently regressed with non-steroidal anti-inflammatory drugs. Her medical history included caecum and terminal ileum resection for bowel ischemia with ileocolic anastomosis. On examination she did not present with any signs of intestinal obstruction or peritonitis and her rectal examination was normal. The laboratory findings, including her white blood count, C-reactive protein (CRP), aspartate aminotransferase (AST), alanine aminotransferase (ALT), Υ-glutamyl transpeptidase (GGT), alkaline phosphatase (AP) and bilirubin levels, were within the normal range.Her abdominal radiographs showed dilated loops and air-fluid levels, mainly in the terminal ileum, however the rectal ampulla was inhabited by stool and air. Calcified masses were identified in the right upper quadrant Figure .A computed tomography (CT) scan of the abdomen confirmed dilatation of her distal small bowel, which was more marked in the pre-anastomotic loop, with a calcified obstructive intraluminal formation Figure . Her galShe reported a resolution of the occlusive symptoms; the surgeon chose to avoid a cholecystectomy because she was considered a high-risk patient. At six months after discharge she has remained without reoccurrence of symptoms.Gallstone ileus is a rare bowel obstruction caused by gallstones; the most frequent mechanism is the migration of stones through a gallbladder-duodenal fistula –3. OftenA preoperative diagnosis of gallstone ileus is challenging. The clinical presentation can be characterized by non-specific symptoms and it usually depends on the localisation and the nature of the obstruction . However, in cases of a clinical history of gallstones, clinical signs of cholecystitis and bowel obstruction, gallstone ileus can be strongly suspected. The onset can manifest as acute, intermittent or chronic episodes.Plain abdominal X-rays, abdominal ultrasound and CT scans may reveal signs of gallstone ileus and help in the preoperative diagnosis and management of this disease. The Rigler’s triad of radiological features consists of mechanical bowel obstruction, pneumobilia and ectopic stone within the intestinal lumen . The preControversy remains about the management of gallstone ileus. Although spontaneous resolution of gallstone ileus are described , it geneIn our case report, the nuanced presentation and the possibility to access the obstruction endoscopically suggested a conservative treatment approach. This case shows some different features when compared with cases described elsewhere. First of all, the age of our patient was uncommon, since gallstone ileus is more frequent in patients over 65 years of age . The preAn endoscopic dilatation allowed us to remove two stones but a bigger third stone required an ESWL. We did not have to place a radiopaque marker to guide the ESWL, unlike in a previous case , becauseOur case report demonstrates that gallstones may enter the gastrointestinal tract through the Vater papilla and later increase in size. The presence of a narrow tract of intestine can facilitate the incidence of gallstone ileus. Given the variability of gallstone ileus presentation, the treatment may not necessarily require a surgical treatment. In our case report, colonoscopy and ESWL were the non-invasive approaches that allowed for diagnosis and treatment simultaneously. Available data does not show a higher rate of recurrent biliary disease, therefore in selected patients conservative treatment may be a curative therapy.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Ameloblastoma is a benign but locally aggressive tumor of odontogenic epithelial tissue. Reports of radiotherapy treatment modalities are limited in the literature.A thirty-five year old male presented with complaints of headache radiating to his face for about six months and impaired vision. The patient’s Positron Emission Tomography (PET) showed a mass in the left maxillary sinus extending to the nasal cavity and invading the adjacent tissues. An R2  surgical resection performed to debulk the tumor. Due to the recurrence and residual mass, the patient was treated with helical tomotherapy. At 2 months post-radiotherapy, patient’s vision returned to normal. PET scan showed a significant reduction in lesion size 12 months post-radiation.In cases of ameloblastic carcinoma with, post-surgical recurrence or patients not suitable for surgical treatment, helical tomotherapy can be an effective treatment option. Ameloblastoma is a locally aggressive benign tumor derived from the odontogenic epithelial tissues. The tumThe basic form of treatment for localized ameloblastoma is radical surgery. The only treatment option in metastatic disease appears to be chemotherapy, although the outcome is not favorable. Radiotherapy modalities are limited in the literature.Here we report a case of ameloblastic carcinoma with basal cell histology, where helical tomotherapy achieved a prominent response.TM, Slovak republic) contouring workstation. The patient’s eyes, optic nerves, lenses, brain stem, optic chiasm, parotid glands, spinal cord, cochleae, vestibules and skin were contoured as critical organs. Prior to the radiotherapy for ameloblastic carcinoma, two separate clinical target volumes (CTV) were defined. CTV1 was defined by adding a 5 mm margin to the gross tumor volume, while planning target volume (PTV) 1 was obtained by adding a 3 mm margin to the CTV1 volume, and then administration of 60 Gy total dose was planned using simultaneous integrated boost (SIB) technique. PTV2 was defined by adding a 15 mm margin to CTV1 volume, and the prescription dose was 50 Gy to PTV2  and Positron Emission Tomography (PET) images  uptake decreased , while a complete response was achieved in the lesion intracranial extensions , whereas 20% of the all cases it involves the premolar region, and 10% the anterior region,15.According to the histological findings, the tumors are classified as follicular, plexiform, acanthomatous, granular, basal cell and desmoplastic type. The maiIn the diagnosis of ameloblastoma, the radiological tools such as panoramic radiography, CT, MRI and PET-CT can be used. Panoramic radiography is often used in daily practice, while the other methods are better at detecting the presence of metastases, contours, content and soft tissue extension of the lesion,5,17,18.Although considered a benign tumor, ameloblastoma may develop recurrence after resection and become clinically more aggressive, while leading to massive local destruction and metastasis. 15-25% As ameloblastoma is a rare and slow-growing tumor, the use of radiotherapy in the treatment should be discussed. Also there are limited data for detailed radiotherapy field design and dose prescriptions. The information regarding radiosensitivity in the current literature is ambiguous. In incoAmeloblastoma is a slow-growing tumor with no standard chemotherapy treatment options, and primarily treated with curative surgical procedure. In conclusion, we suggest that helical tomotherapy can provide an effective treatment option in ameloblastoma cases where complete resection is not feasible or in patients developing local recurrence.The authors declare that they have no competing interests.TK (Corresponding author): Carried out contouring the patient, drafted, generally evaluated and write the manuscript, HB: Made the tables and carried out coordination between clinics, DA: Carried out the patient interrogation and follow-up, write the manuscript, DS: Evaluated the patients radiographs and made calculations, ZAÇ: Carried out the patient set-up preperation, ÖK: Carried out set-up verifications, SK: Prepared the treatment plans of the case, CİB: Carried out histo-pathological examination, HÖO: Carried out surgery, MD: Carried out PET scans. All authors read and approved the final manuscript.


In this case report, we describe the successful treatment of a small-bowel intussusception, which was caused by a 3cm solitary hamartomatous polyp, with single-incision laparoscopic surgery. Single-incision laparoscopic surgery is a minimally invasive surgical procedure with important advantages that allows the reduction of the intussusception and the resection of the polyp. This case report contributes to the medical literature by describing the advantages of this surgical technique that warrant its consideration as a treatment of choice in similar cases.We report a case of a 19-year-old Greek woman who complained about intermittent, non-specific abdominal pain in her left lateral abdomen. She had been admitted to the hospital because of incomplete obstructive ileus. Ultrasound and computed tomography were carried out, which revealed an intussusception of the small bowel. This pathogenic situation was treated by single-incision laparoscopic surgery. Her pathology report revealed a benign, hamartomatous excised polyp of the Peutz-Jeghers type. The patient had a quick recovery without any post-operative complications.We recommend single-incision laparoscopic surgery for the safe excision of solitary hamartomatous polyps and the management of their complications, as it represents a potential advance in minimally invasive approaches. Hamartomatous bowel polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions in combination with their pedunculated nature -5. UsualA 19-year-old Greek woman consulted at the emergency department for recurrent episodes of non-specific cramping and intermittent abdominal pain in her left lateral abdomen of 4 days’ duration. The pain was accompanied by nausea and vomiting. She had already been admitted to the hospital two times in the past with symptoms of incomplete obstructive ileus accompanied by partial retention of gas and feces. The patient had no significant past medical history, reported no tobacco use or alcohol intoxication and was on no medications. Her family history was also not significant, and thus her symptoms were not deemed to be associated with inherited PJS. Her general condition and vital signs were normal. Her physical examination was unremarkable, except for a mild tenderness to the left lateral abdomen. On the day of admission, the results of her routine laboratory investigations were within normal ranges, except for mildly increased leukocytes, which were measured at 15,200/μl . Ultrasound (US) of the abdomen was performed, which revealed a target-like mass that suggested an intussusception of the small bowel to the left abdominal quadrant and very closely to the ligament of Treitz , which we also used to create a pneumoperitoneum. By using the patient’s umbilicus as a single port, first we recognized the pathogenic segment of the small bowel at a distance of 90cm from the ligament of Treitz, and continually we managed to reduce the intussusception by using articulate graspers Figure . Then, wMacroscopically, the excised polyp measured about 3cm Figure  and was The pathology report confirmed the polyp to be a hamartoma with characteristics of a PJP Figure .Figure 6Post-operatively, the patient had an uneventful recovery, and she did not experience any complications. Her recovery was quick, and she was discharged on post-operative day 4. Three months later, an endoscopic examination of the upper and lower gastrointestinal system was performed without confirmation of any polyps.Small bowel intussusceptions induced by polyps can be managed successfully with minimally invasive surgical approaches. Laparoscopy is one of these approaches that deals with the acute problem, is a safe and effective method for reducing an intestinal intussusception and may prevent adhesion formation. Laparoscopy allows adequate access to explore and treat small bowel polyps and avoid the classic laparotomy ,8,9. TheThe laparoscopy-assisted double-balloon enteroscopy is a recently developed technique that can be used as both surveillance and a therapeutic tool for small bowel polyps in patients with hamartomatous polyps ,9,10.A combined endoscopic and laparoscopic approach can be used to treat proximal small bowel intussusception, which could possibly eliminate the need for laparotomy and reduce the post-operative complications associated with multiple reoperations in this patient population .Attempts to further minimize trauma related to surgical procedures and improve their cosmetic effects, especially in young patients, resulted in modification of the laparoscopic technique to a surgical procedure with a single, small incision, most commonly in the umbilicus .SILS or one-port umbilical surgery is a more recent technique in the field of laparoscopic surgery. The first SILS case was reported in 1997. Complex surgical procedures can be performed through a single 2cm incision by using flexible endoscopes and articulating instruments. All surgical instruments are placed through this small incision . In SILSSILS seems also to be a very effective surgical technique specifically with regard to PJPs. PJPs are usually sited in the jejunum and ileum (90% of the cases), colon 42%), stomach 38%) and rectum (28%) %, stomac. They co8% and reThe diagnosis of PJS should be considered in patients who present with a clinical picture of bowel obstruction and mucocutaneous hyperpigmentation ,6. A comIdeally, preservation of intestinal length is important in patients with PJS because recurrence is seen in up to 10% of cases, and multiple resections could lead to the short bowel syndrome. However, recurrence of intussusception due to polyps is common in PJS, and a combined approach of laparoscopy and endoscopy to reduce the need for multiple laparotomies may avoid untoward problems in the future ,9.Despite the fact that we describe above the PJS, we have to mention that our patient had only a solitary bowel polyp, which appeared to be a PJP based on the pathology report, and that we did not diagnose our patient with PJS. Our report aims to present the surgical management of the intussusception caused by this solitary PJP by SILS.To our knowledge, there are at least six other published case reports regarding the laparoscopic management of bowel obstructions in PJS. We searched the medical literature in PubMed by using the keywords “PJS”, “solitary PJP”, “small-bowel intussusception”, “SILS”, and “laparoscopy.” We analyzed this database and found only three articles about small bowel intussusception induced by a solitary PJP treated with laparoscopic management, but we found no reported results about SILS. There are only three reports about laparoscopic treatment of multiple polyps Table .Table 1Surgical procedures have strongly evolved from laparotomy to laparoscopy because of the development of laparoscopic equipment and improvement in surgical skills. Nowadays, SILS is one of the most minimally invasive laparoscopic techniques and has a lot of benefits, even in rare pathogenic situations. We think that this effective surgical approach can be equally used for the treatment of non-acute and acute cases, such as of a bowel intussusception. SILS can be a suitable choice for the treatment of intestinal intussusception induced by a solitary hamartomatous polyp and offers the opportunity to retain the whole length of the bowel as well as the benefits of its minimally invasive nature.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Epidermoid cysts are a rare entity in the oral cavity and are even less frequent in the floor of the mouth, representing less than 0.01% of all the cases. We present the case of a 3-year-old girl with a growth in the floor of the mouth with 2 months of evolution and without changes since it was discovered by her parents. The lesion was asymptomatic; it did not cause dysphagia, dyspnea, or any other alteration. A CT scan with contrast was done which revealed the location and exact size of the lesion, allowing an intraoral approach for its excision. The histological examination confirmed the clinical speculation of an epidermoid cyst. Epidermoid cysts are benign cystic malformations that are derived from the ectoderm . They reHistologically and according to Meyer's classification , they caMany theories have been proposed to explain their development, suggesting a dysontogenetic or traumatic origin, or even due to anomalies of the thyroglossal cyst . Those oAnatomically they are often seen in the midline and we can divide them into three groups based on the relation that they maintain with the muscles of the floor of the mouth: sublingual or medial genioglossus, medial geniohyoid or submental, and lateral, although some authors consider the lateral ones to be medial cysts that expanded laterally, and not a separate entity . In the They are usually slow growing and progressive and always tend to be asymptomatic. When they do not cause displacement or elevation of the tongue due to their size, they can cause dysphagia, dyspnea, and/or the development of a “double chin” if they expand to the submental space –3.They are commonly observed during the 2nd and 3rd decades of life , 2, 4, 5The majority of authors believe that there is no sex predilection , althougThe size is variable, ranging from a few millimetres to a few centimetres, and within the literature there are three reported cases of giant epidermoid cysts, one of 7 cm and two of 13 cm , 13.A 3-year-old girl, with no relevant family or personal medical history, presented a growth in the floor of her mouth with two months of evolution, and without any noted changes since it was discovered by her parents. It was asymptomatic and did not cause dysphagia, dyspnea, or any other alteration. There was no palpable lateral cervical lymphadenopathy or fever. On exploration a well-marked growth was observed in the anterior region of the floor of the mouth in the midline. It was covered by normal oral mucosa and did not show signs of inflammation; it was not attached to deeper planes and fluctuated upon palpation .An initial diagnosis was of a ranula, due to its higher prevalence with respect to the epidermoid cyst, but both its clinical appearance and its location in the midline led us to believe that it could be a dermoid cyst.The CT scan with contrast was done which showed a well-encapsulated cystic mass and confirmed the initial clinical diagnosis Figures .The patient underwent surgical treatment of complete excision via intraoral approach. The surgery was performed under general anaesthesia and tracheal intubation which allowed us to make a vertical incision on the ventral aspect of the tongue and blunt dissection was done until the lesion was completely released . The sizThis pathology is very rare at this location. As cited by Longo et al.  in theirHistologically and in accordance with the classification described by Meyer in 1955 , they arThe occurrence of complications is not frequent, although they may be infected spontaneously or after an FNAC test (fine needle aspiration cytology) . In our The differential diagnosis must include infectious processes, malignant tumours, extravasation of mucous, and anatomic abnormalities which originate during embryonic development , 9. In oSince the clinical appearance was compatible with the ranula and these are much more common than epidermoid cysts, it was our first diagnostic hypothesis. The images of the CT, however, made us suspect a dermoid cyst.Like the rest of the authors, we believe that these growths should be operated on and eliminated in their entirety. This allows for confirming of the diagnosis and avoiding future problems , 16–18. If it is completely eliminated, the recurrence of the cyst is rare , 2, 4. AFinally we can conclude that the dentist should be familiar with the differential diagnosis of the tumour and cystic lesions of the area, in order to be able to perform the appropriate tests for each case and therefore be able to plan a safe surgery.


Heterotopic pancreas is defined as pancreatic tissue without a real anatomical or vascular connection to the pancreas. It can be found in the stomach, duodenum, jejunum, ileum, Meckel’s diverticulum, colon gall bladder, umbilicus, fallopian tube, mediastinum, spleen and liver. Complications of heterotopic pancreas are inflammation, bleeding, obstruction, malignant transformation, carcinoid syndrome, jejunojejunal intussusception and ileus, but it is usually asymptomatic and diagnosed only during examinations for other diseases.An 81-year-old Lithuanian woman was diagnosed with caecal cancer and had undergone elective surgery. A right hemicolectomy was performed and a Meckel’s diverticulum was observed and excised. Histological results showed a poorly differentiated G3 adenocarcinoma of her large intestine and heterotopic pancreas tissue in the Meckel’s diverticulum and mesenteric adipose tissue.Asymptomatic heterotopic pancreas is rarely diagnosed, and usually found incidentally during surgical or diagnostic interventions. Although it has no symptoms, heterotopic pancreas found during surgical procedures should be excised. Heterotopic pancreas (HP) is defined as pancreatic tissue without real anatomical or vascular connection to the pancreas and was first reported by Jean-Schultz in 1729 . The mosAn 81-year-old Lithuanian woman was tested for faecal occult blood during preventive colorectal cancer screening. Test results were positive and she underwent a colonoscopy. The colonoscopy revealed a tumour in her caecum. Abdominal and thoracic computed tomography (CT) scanning revealed no metastasis. She then underwent elective surgery. During the operation, a 6×5cm tumour was found in her caecum. Her MD was found 40cm from the hepatoduodenal ligament [99mtechnetium pertechnetate scintigraphy, which detects ectopic gastric mucosa in MD. However, the diagnostic accuracy of this method is 46% in adults. Abdominal ultrasound, X-ray and CT usually give nonspecific findings. MD can be detected by capsule endoscopy or double-balloon enteroscopy. The diagnosis of MD with HP is difficult [The aetiology of HP, otherwise known as “aberrant”, “ectopic”, or “accessory” pancreas, is still unclear, but it is believed to arise embryonically during rotation of the foregut and fusion of the dorsal and ventral pancreatic buds. Some tissue separates from the pancreas and develops into HP in any portion of the alimentary system. Another best known theory is based on the pancreatic metaplasia of endodermal tissue ,5. Becauifficult . Managemifficult . In our If HP is asymptomatic, as in our case, diagnosis is unlikely without surgical intervention. Surgical interventions are usually performed in other diseases. Because of its complications and chance of malignant transformation, HP found during surgical procedure should be excised.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Ileosigmoid and ileoileal knotting are two rare entities. They usually present as acute abdomen and the diagnosis is established intraoperatively. The treatment is surgical and should be performed as soon as possible to decrease the incidence of perioperative mortality and morbidity.We report an unusual case of a 26-year-old Argentine man with ischemic necrosis in parts of his small and large intestine due to combined ileosigmoid and ileoileal knotting. He had an ileal loop of ileum concurrently wrapped around the neck of a sigmoid volvulus and other loops of ileum strangulating them, forming two different tangles of tying. This very rare and unusual entity was diagnosed and managed intraoperatively during a diagnostic laparotomy performed on an emergency basis. Both the gangrenous small bowel loops and the affected sigmoid colon area were resected. The continuation of the intestinal tract was restored by primary end-to-end anastomoses. The present case is unusual and to the best of our knowledge no similar cases of simultaneous ileosigmoid and ileoileal knotting have been described in the literature. The postoperative course of our patient was uneventful and he was discharged from the hospital on the 15th postoperative day. One year later he still remains without symptoms from his intestinal tract.Simultaneous ileosigmoid and ileoileal knotting is a very rare entity that should be diagnosed and treated surgically on an emergency basis to minimize the high postoperative morbidity and mortality. Intestinal knotting is the obstruction of an intestinal segment with closed loop phenomenon secondary to knotting of the mesentery. Three types of intestinal knotting have been described in the literature. They are characterized by the segments of the large and small intestine that are affected in this rare entity. Intestinal knotting can be ileosigmoid, ileoileal, or include Meckel’s diverticulum or appendix . In ileoWe report the unusual case of a patient with synchronous ileosigmoid and ileoileal knotting causing ischemic necrosis in both small and large intestine. The diagnosis was established intraoperatively. Resection of the gangrenous small bowel and sigmoid colon was performed and the intestinal and colon continuation restored by primary end-to-end anastomoses.A 26-year-old Argentine man presented to the emergency department of our hospital complaining of acute severe abdominal pain. The pain started about 6 hours previously and was worsening progressively. A few hours before he visited the hospital he complained of proctalgia and rectal bleeding, and an abdominal discomfort of mild pain, which had started 2 to 4 hours ago; he had been examined by the on-duty physicians who failed to establish an accurate diagnosis. In the physical examination, he seemed to be severely ill and was presenting bradycardia (47 beats per minute) and mild tachypnea, while his body temperature and blood pressure were within the normal values. His abdomen was moderately distended and he presented tenderness with rebound during palpation and rigidity of his abdominal wall. Bowel sounds were absent. In a digital examination, although his rectum was found free of content the pouch of Douglas was very painful. Blood analysis revealed only a mild leucocytosis with neutrophil predominance. His arterial blood gas values were within the normal range. An abdominal radiography showed some rare small air-fluid levels of his small bowel and a large air-fluid level of his sigmoid colon as seen in Figures The initial description of intestinal knotting is found on the 16th century by Riverius, while a more detailed description of this rare entity achieved in 1836 by Rokitansky. Ileosigmoid and ileoileal knotting are two very rare entities, particularly in Western countries, that can rapidly evolve to gangrene of the affected bowel segments . The preSimultaneous ileosigmoid and ileoileal knotting is a rare entity that should be diagnosed and treated surgically on an emergency basis. An emergency laparotomy as soon as possible and the correct procedure may contribute to a decrease in the high perioperative mortality, which accompanies this terrible pathology. The saving treatment of this rare entity is urgent surgical intervention.I would like to remain anonymous.I write the following to provide assistance to the case report written about my operation. I have no medical knowledge or background. So I write from my own perspective and experience. The problem with my health started as a low diffuse pain in my abdomen and after 2 to 4 hours a quite severe pain appeared in my rectum with a little blood which led me to the hospital “Polykliniki”. I was examined clinically and endoscopically by a gastroenterologist without pathological findings. Afterwards, I came back to my house. Then the abdomen pain was worsening and it forced me to visit again the hospital at the emergency department. The surgeon on-duty, who examined me, diagnosed that I was presenting a severe condition of acute abdomen and then quickly after laboratories and radiological examinations I was led to an operating room. My operation was performed after midnight. The next day I was not feeling as much pain as when I entered hospital. However, I had a tube in my nose and a urocatheter. They were removed after a few days and I slowly started taking drinks and then soups and small meals. The nursing staff attended me with kindness and periodically administered antibiotics, analgesics and serums, according to the doctors’ guidelines. My surgeon, in his morning visit, examined my abdomen, my legs, my chest, my blood pressure and my temperature and then he changed my surgical wound. All the days after my operation were without fever and trouble. I stayed in the hospital 15 days. The stitches of my wound were taken out the day that I was leaving. No problems existed in my recovery and I have been well since.Written informed consent was obtained from our patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.Our patient was admitted under the care of NA during this episode and was followed up in the outpatient clinic. NA was major contributor in writing the manuscript. DF, SP and AK contributed significantly to the editing of the manuscript and the review of the literature. All authors read and approved the final manuscript.


This is a case report of a 56-year-old male with hypertension who presented with urinary retention and bowel incontinence. CT and MRI of the abdomen and pelvis showed a large complex cystic and solid enhancing mass in the right presacral space. Pathology biopsy result showed malignant peripheral nerve sheath tumor (MPNST) with extensive necrosis. The diagnosis of MPNST is extremely difficult due to the lack of (1) conclusive immunohistochemistry or unique chromosomal anomaly, (2) universal distinctive histopathology, and (3) clinical criteria. The clinical, radiologic, and histologic presentation of MPNST isimportant in its diagnosis. A rare case of MPNST that produced urinary retention and bowel incontinence is presented that may aid clinicians in the diagnosis of this rare clinical entity. Motor weakness, central enhancement, and immunohistochemistry may assist in the diagnosis of MPNST and differentiation between benign peripheral nerve sheath tumor (BPNST) and MPNST.  This is a 56-year-old male with history of hypertension and neurofibromatosis who presented with sudden onset of urinary retention and bowel incontinence.MRI of the lumbar spine showed a large mass extending anteriorly from the right S2 nerve root and in the posterior right lower back musculature at the level of L4-L5 and mildly enlarged left L2 ganglion and multiple skin neurofibromas. CT of the abdomen and pelvis showed a large complex pelvic mass measuring greater than 10 cm displaying mass effect on the posterior surface of the rectum arising from the right S2 neuroforamen. MRI of the abdomen and pelvis showed 9.6 × 9.8 cm complex cystic and solid enhancing mass in the right presacral space, extending from the right S2 neural foramen, displaces the rectum anteriorly and to the left Figures .Pathology biopsy result showed malignant peripheral nerve sheath tumor (MPNST) with extensive necrosis.MPNST is a term that replaces malignant schwannoma, malignant neurofibroma, neurosarcoma, and neurofibrosarcoma. MPNSTs which account for 3–10% of all soft tissue sarcomas are rare and arise in proximity to large peripheral nerves. The most common location of these tumors is the trunk, extremities, or head and neck, being extremely rare in abdomen. The majority of MPNSTs are high grade sarcomas with high probability of recurrence and metastases. About half occur in patients with neurofibromatosis type 1 which have a poorer prognosis than MPNST in patients without neurofibromatosis type 1. Approximately two-thirds of MPNSTs result from malignant transformation of tumor associated with neurofibromatosis type 1. The overall lifetime risk of developing MPNST in neurofibromatosis type 1 patients is 8–13%. Neurofibromatosis type 1 is autosomal dominant disorder with highly variable phenotypic expression and natural history. The involvement of the abdomen and pelvis by neurofibromatosis type 1 is rare. Neurofibromatosis type 1 associated MPNST occurs in young adults (20–35 years of age) whereas sporadic MPNST occurs in middle age and older adults (peak: 5th and 6th decades). Pelvic peripheral nerve sheath tumors are very uncommon. Differential between benign peripheral nerve sheath tumor (BPNST) and MPNST is difficult because the former may be large and have marked atypical nuclei. The age range and size of BPNST and MPNST are 10–76 years  and 4–20 cm (median size 9 cm) and 16–61 years  and 9–17 cm , respectively. MPNSTs occurring in central locations such as the paraspinal region of the retroperitoneum have lower 5-year survival rates, higher recurrence rates, and higher frequency of metastasis compared to tumors in other parts of the body. MPNST may occur as solitary or multiple enlarged masses associated with major nerve trunks such as the brachial plexus, sacral plexus, and sciatic nerve and may be asymptomatic or present with various sensory and motor symptoms, including projected pain or compressing and infiltrating surrounding tissues and structures. About 10% of MPNSTs occur due to therapeutic or occupational radiation with a latent period of greater than 15 years [BPNST is frequently asymptomatic whereas MPNST produces pain or neurological deficit. Pain at rest, tumor size, and duration of symptoms are of little value in differentiation between BPNST and MPNST. Faint motor weakness may be present in BPNST whereas severe motor weakness is seen exclusively in MPNST. MPNST may occur as solitary or multiple enlarged masses associated with major nerve trunks such as the brachial plexus, sacral plexus, and sciatic nerve and may be asymptomatic or present with various sensory and motor symptoms, including projected pain or compressing and infiltrating surrounding tissues and structures. The involvement of the abdomen and pelvis by neurofibromatosis type 1 is rare presenting with dysfunction and obstruction of genitourinary and gastrointestinal tracts. Patients with BPNST present with pain at rest (79%), sensory disturbance (53%), and faint-to-mild motor weakness (32%). The duration of the symptoms is 2–120 months . All patients with MPNST have pain at rest (100%), some patients have sensory disturbance (73%), and other patients have faint-to-severe motor weakness 64%). Symptom duration was 3–96 months  [%. SymptoThe correct histological diagnosis of MPNST is made in about 17–41% of new patients, whereas about 52% were diagnosed as having another type of sarcoma, about 4% were classified as suspicious, and 4% represented false negatives due primarily to profound cytological overlap with other sarcomas. The diagnosis of MPNST is extremely difficult due to the lack of (1) conclusive immunohistochemistry or unique chromosomal anomaly, (2) universal distinctive histopathology, and (3) clinical criteria. The clinical, radiologic, and histologic presentation of MPNST is important in its diagnosis [In the present case report, MPNST has the following: (1) MRI signal characteristics: high signal intensity: STIR, T2, and STIR > T2; intermediate signal intensity: PD FS, T1 FS, and PD FS > T1 FS; low signal intensity: T1, isointense to muscle; heterogeneous enhancement, whereas skin neurofibromas have high signal intensity: STIR, T2, and STIR > T2; intermediate signal intensity: T1; uniform enhancement; and (2) CT with contrast attenuation characteristics: heterogeneous attenuation; small lesions: attenuation lower than muscle; skin neurofibromas: attenuation same as muscle, with no significant enhancement. MRI is better than CT at detecting small lesions. CT did not show the smallest lesions but it is adequate for diagnosis if the patient cannot have MRI. The most sensitive MRI sequence for detection of MPNST is STIR. Heterogeneous hypointense signal on T1WI and heterogeneous hyperintense signal on T2WI are due to high water content of myxoid matrix or cystic or necrotic degeneration. Central areas of low signal intensity may be seen due to fibrosis. Post-IV contrast CT may show no contrast enhancement due to areas of necrosis, hemorrhage, or cystic degeneration. About 40–50% of BPNSTs may have central enhancement on CT corresponding to central zone of tightly packed cellular components (Antoni A) surrounded by hypocellular myxoid material (Antoni B) corresponding to target sign on MRI T-2 weighted images . The central enhancement and target sign are rarely seen in MPNST. In large tumor, there is central necrosis or degeneration, with peripheral tumor enhancement. The borders in MPNST are frequently irregular and infiltrative borders may invade adjacent structures or destroy adjacent bones. CT and MRI cannot accurately differentiate between MPNST and BPNST because tumor heterogeneity, irregular or infiltrative borders, and bone erosion may be seen in both MPNST and BPNST .MPNST is high grade tumor with high mitotic rate. However, histological diagnosis of MPNST is very difficult due to the lack of 1) histological and immunohistochemical markers specific for malignant peripheral nerve sheath tumor and (2) standardized diagnostic criteria. However, S-100 protein is seen in 50–90%, Leu-7 in 50%, and myelin basic protein in 40% and Ki-67 indices are seen in 5–65% of MPNSTs. Coinactivation of TP53 and Rb pathways is seen in 75% of MPNSTs [ histologThe most common metastatic site of MPNST is the lung, followed by the bone, pleura, retroperitoneum, and subarachnoid space of the spine . The patSurvival of MPNST is associated with complete tumor resection. The overall 5-year survival rate is about 44–50% which is affected by the patient's age, size of tumor, location of tumor, and margins affecting survival. Tumor size is the most reliable independent prognostic factor, with larger tumors having worse prognosis. Tumors occurring in central locations such as the paraspinal region of the retroperitoneum have lower 5-year survival rates, higher recurrence rates, and higher frequency of metastasis compared to tumors in other parts of the body. Longer survival is associated with early diagnosis (due to improved imaging), aggressive treatment with complete surgical resection and neoadjuvant therapy, small tumor size (<5 cm), and presence of low grade components. Negative staining for S-100 is associated with better prognosis for completely resected tumors .A rare case of MPNST that produced urinary retention and bowel incontinence is presented that may aid clinicians in the diagnosis of this rare clinical entity. Motor weakness, central enhancement, and immunohistochemistry may assist in the diagnosis of MPNST and differentiation between BPNST and MPNST.


An intestinal obstruction occurs when either the small or large intestine is partly or completely blocked so it prevents passing the food or fluid through the small/large bowel. This blockage is due to the existence of a mechanical obstruction such as foreign material, mass, hernia, or volvulus. Common symptoms include cramping pain, nausea and vomiting, changes in bowel habits, inability to pass stool, and lack of gas. We present a case of an 83-year-old man who had been referred to Taleghani Hospital with symptoms of bowel obstruction. He underwent the surgery. The findings of exploration of the entire abdomen showed two types of mass separately in two different organs. In postoperative workup, pathology reported two types of tumors (adenocarcinoma and neuroendocrine tumors). An Intestinal obstruction occurs when either the small or large intestine is partly or completely blocked so it prevents passing the food or fluid through the small/large bowel. This condition would cause inability to pass food, fluid, and gas in the digestive tract. The obstacle could be a foreign material or the result of volvulus, hernia, or tumor growth .Bowel obstruction could also be a result of abdominal infection or inflammation caused by conditions such as diverticulitis. In patients with Crohn's disease or peritoneal carcinomatosis, the risk of bowel obstruction may arise .Some symptoms of this disease include abdominal cramping, abdominal distension, diarrhea, or inability to have bowel habits . Cancer Intravenous hydration and correcting electrolyte levels are the first treatment for such patients and then, according to the nature of bowel obstruction, the curative treatment will be begun .This is reportage of an 83-year-old man who had experienced changes in bowel habits with feeling of diffuse abdominal pain aggravated by eating and relieved by passing stool from 5 months before. Due to these symptoms, colonoscopy was planned for him, but because of the lack of consent, CT scan of the abdomen/pelvis was performed. As you can see in Due to increasing abdominal pain, nausea, vomiting, and inability to pass gas and stool for six days, the patient was referred to Taleghani Hospital of Tehran. In Intraoperative findings indicated bowel obstruction due to a mass that was located at a distance of 20 cm from the rectosigmoid junction in the sigmoid region, dilation of the colon and small intestine, and another mass with dimensions of 2 × 2 cm that was located at a distance of 80 cm from the ileocecal valve in the ileum region.The patient underwent the small bowel segmental resection and primary anastomosis and total abdominal colectomy because of the severe dilatation of the proximal part of the colon and ischemic changes of the cecum with ileorectal anastomosis (end-to-end) was done for him. In postoperative workup, there was no sign of metastasis and he was discharged from the hospital after six days with good general conditions. The pathologic findings showed existence of two tumors as the following.(1) The small bowel wall was infiltrated by a neoplasm composed of monotonous small round cells with small nucleoli, salt and pepper chromatin, and moderated finely granular cytoplasm arranged in nesting and trabecular structure.Well-differentiated neuroendocrine tumor with lymphovascular invasion and size of 2 cm with greatest dimension and mitotic rate of less than 1/10 hpf was seen in the ileum site. Tumor invades the subserosa tissue without involvement of the visceral peritoneum (pT3).(2) There was a well-differentiated adenocarcinoma with lymphovascular invasion and size of 5.5 cm in the greatest dimension in the sigmoid-colon region with microsatellite instability and mucinous production (20% of tumor). Tumor invades the subserosal tissue and 21 lymph nodes were removed (T3 N0).Due to the existence of an obstructive tumor and lymphovascular invasion, the patient was a candidate for chemotherapy. But because of some factors such as age and his disability to tolerate the conditions of chemotherapy, chemotherapy was not done for him.More than 575.000 people die of cancer and more than 1.5 million people are diagnosed with cancer every year in the USA. According to the World Health Organization (WHO), the numbers of new cancer cases are expected to be raised by about 70% over the next 20 years . SurgeryAccording to studies in the USA, each year an estimated 8.000 people in the USA are diagnosed with a neuroendocrine tumor that starts in the gastrointestinal tract which includes the stomach, intestine, appendix, colon, or rectum .Well-differentiated neuroendocrine tumors have cells that do not look very abnormal and are not multiplying rapidly and sometimes the only way to know that a mass is a neuroendocrine cancer is when it spreads to the other organs or tissues .In some cases, there is a possibility of existence of some other benign tumor in the other organs. Accordingly the surgeon must check the possibility of other masses in the abdomen . StudiesThe synchronous occurrence of two differentiated tumors in a single patient is rare. So far, there are several reported cases that had two differentiated tumors in two or several other organs simultaneously , 13–15.Ferrando Marco and his colleagues reported the case of a 64-year-old man with periampullary collision tumor, in which a duodenal-wall carcinoid and an adenocarcinoma of the head of the pancreas coexisted .Akiba and his colleagues reported a case of a 64-year-old man who was found to have EEBV-associated gastric carcinoma with primary gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue .Kleist and his colleagues reported two collision tumors containing a gastrointestinal stromal tumor with intermingling elements of gastric adenocarcinoma .Jang and his colleagues reported a case of a 70-year-old man found to have two separated masses which were observed in the proximal ascending colon containing two separated well-differentiated neuroendocrine tumors with necrosis and increased mitosis .Singh and his colleagues reported a case of a 52-year-old found to have a mass at the base of the appendix. On microscopic examination of the tumor, mixed adenocarcinoma and carcinoid were identified .Van Kerkhóve and his colleagues reported a case of a 72-year-old female found to have a rare ileal collision tumor consisting of an adenocarcinoma and a small cell neuroendocrine tumor with peritoneal metastasis of neuroendocrine origin and coincidental benign lesions on both ovaries .According to the studies, 15–35% of patients with neuroendocrine cancer in the ileum had more than one tumor and 50–60% of them survived about 5 years after surgery  .In 15–30% of cases with ileum neuroendocrine tumor, there is more than one tumor, and 15–29% of tumors are associated with other noncarcinoid malignancies .Neuroendocrine tumors are neoplasms that are raised from cells of endocrine and nervous system. They could be malignant or benign and most commonly occur in the intestine and will spread to other organs. Ileal neuroendocrine tumors often grow in the distal part of the ileum. In 15–30% of cases, there is more than one tumor, and 15–29% of tumors are associated with the other noncarcinoid malignancies.Diagnosis must be done by using laboratory tests, X-ray, CT scan, and endoscopy. Surgery is a definitive treatment in the early stages. Patient's age, the exact location, dimension and differentiation of tumor, and lymphovascular invasion are parameters which affect the progression of the disease.In this case report, the patient with diagnosis of the bowel obstruction underwent surgery and we found a mass in the sigmoid region with pathologic diagnosis of adenocarcinoma and another mass in the ileum region with pathologic diagnosis of a neuroendocrine tumor.Due to the increased prevalence of malignant tumors, checking the entire of the digestive system according to existing protocols in similar cases and not forgetting to consider the possibility of coexisting malignant tumors to find and treat them at an early stage are recommended.


We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully via an intracranial approach. An enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. After a 22-month follow-up period the patient remains symptom free and no recurrence is noted. Impaction of cerebral tissue into the temporal bone (mastoid cavity and/or middle ear) is known as temporal encephalocele. The condition was first documented as early as 1902 by Caboche .Iatrogenic etiology was very common during the early part of the twentieth century and prior to modern antibiotic era, since trephination and draining through the infected mastoid and dura were the mainstay management of brain abscesses, after otitis media. However, encephalocele after mastoid surgery remains today a rare, yet existing occurrence.We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully through an intracranial approach.A 36-year-old male was referred to the Otolaryngology-Head Neck Surgery Department, due to a right sided chronic otitis media with cholesteatoma. The patient underwent a T-tube placement 4 years prior to referral, to treat chronic otitis media with effusion and tube placement resulted to a residual tympanic perforation and cholesteatoma.After a thorough clinical and radiological evaluation a typical canal wall down mastoidectomy was performed. Cholesteatoma debris was meticulously removed along with granulation tissue and infected mucosa. Only an egg cell bone was left intact at the tegmen area and although careful drilling removed all mucosa, no apparent iatrogenic dural injury was noted. Yet part of the roof of the external auditory meatus appeared intraoperatively to be eroded by infection and a “fragile” dura could not be surely excluded.On typical follow-up after 12 months otoscopy revealed an opalescent mass protruding from the roof of the external auditory canal . ImagingPatient was placed on the supine position and the operation was completed in three sequential stages under general anaesthesia. The first stage consisted of the preparation for the autograft of the cartilaginous nasal septum. The second consisted of the repair of the encephalocele and the reconstruction of the skull base and was completed in two substages, one extradural and one intradural. Patient's head was rotated 30° towards the left side and the skin was incised in a semilunar fashion immediately over the right ear . A tempoAfter a 22-month follow-up the patient remains symptom free and no recurrence is noted .Temporal bone encephalocele is a rare entity that occurs mostly as a complication of a middle ear infection, especially with cholesteatoma, or as a complication of surgical intervention aimed at the eradication of that disease. It is important to remember that a bony defect of the skull base alone does not necessary lead to cerebral protrusion because the dura mater is structurally strong and capable of supporting the brain even when large bone defects occur during otologic surgery. It appears that dural injury rather than simple exposure is a prerequisite for iatrogenic encephalocele .Diagnosis of temporal bone encephalocele may be particularly obscure and challenging due to the nonspecificity of findings. Clinical symptomatology may encompass conductive or mixed hearing loss, cerebrospinal fluid (CSF) otorrhea and rarely seizure disorders, recurrent meningitis or aphasia. The typical otoscopic finding is the presence of an opalescent pulsating mass in the external auditory canal or in the mastoid cavity. Yet definitive diagnosis may be ultimately based on radiographic or even intraoperative findings.Radiological evaluation is useful for diagnosing temporal bone encephalocele. High-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) are the preferred means in establishing the diagnosis and excluding other potential congenital communications or otologic infection. The location, number, and size of the bony defects are well demonstrated with HRCT. Direct coronal slices with bone algorithm are required to reliably reveal such bone defects. Yet computed tomography (CT) is nonspecific in the diagnosis of encephalocele since it has a limited ability to resolve subtle distinctions in density between cerebral tissue, cholesteatoma, cholesterol granuloma granulation tissue, or other soft-tissue masses inside the middle ear cavity. MRI, with its affinity for soft-tissue definition, is the ideal method to differentiate these conditions and complements HRCT study in patients suffering from encephalocele.Surgical correction remains the treatment of choice, since complications of encephalocele such as meningitis or a brain abscess are fatal. Surgical management of temporal lobe protrusion goes back to 1913, when Canfield  reportedFurthermore restoration of dural integrity is imperative in successful management of encephalocele. Careful dural closure and firm support of intracranial contents are of crucial importance to prevent recurrence. Different materials can be used to repair both the bone and dural layer. Autogenous graft material is preferred and temporalis fascia remains the dominant choice for use as dural graft because of its accessibility. Yet simple repair with only a single layer of temporalis fascia is associated with a high rate of recurrence ; thus riIn our case an enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. To our opinion, such a technique ensures adequately firm closure and prevents recurrence. Protruded cerebral tissue was cauterized and resected according to literature common practice and no postoperative neurological deficits were noted.We report the case of a temporal bone encephalocele after mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was successfully treated via an intracranial approach and no recurrence has been noted after 22 months of follow-up.


Kaposi sarcoma is a neoplastic vascular disorder. It usually present on the skin of the upper and lower extremities, rarely in the mucosa of the head and neck. The most common sites reported are within the oral cavity, particularly on the palate. Other mucosal sites are rare. We present an unusual case where the primary manifestation of the Kaposi’s sarcoma was in the nasal mucosa.A 56-year-old female of Mediterranean descent presented with a 1 year history of swelling on the left side of her nose, nasal obstruction and occasional minor epistaxes. Physical examination showed a firm and bulging polypoid mass which filled the left nasal cavity without cutaneous lesions.Computed tomography (CT) demonstrated a tumor, measuring 77 mm in diameter, occupying the left nasal cavity causing erosion of nasal septum and extending posteriorly to the left choana and nasopharynx. There was bilateral cervical lymphadenopathy. Patient treated with chemotherapy alone. She was in a complete response after the first cycle. The patient received no further treatment. She needs a regular medical checkups that include a review of a patient’s medical history and a complete physical exam. She is in excellent local control over 12 months.A review of the literature revealed that only seven cases of primary Kaposi sarcoma of the nasal cavity have previously been published and only two of them presented in a patient not associated with the acquired immunodeficiency syndrome. Here, we report the third case where the primary manifestation of the Kaposi sarcoma was in the nasal cavity in a patient with an adequate immune system. Kaposi’s sarcoma (KS) is a low-grade vascular tumor associated with infection with human herpesvirus 8 (HHV-8), also known as the KS-associated herpesvirus (KSHV) , 2.The disease is named after Moritz Kaposi, a Hungarian dermatologist on the faculty of the University of Vienna, who first described the entity in 1872 as “idiopathic multiple pigmented sarcoma of the skin” . The mosThe KS of the head and neck usually involves the mucous membranes of the oral cavity, but it can be found in the pharynx, larynx, and tonsillar –18. IsolA review of the literature revealed that there are a few reported cases of KS involving the head and neck region. In fact, only seven had primary presentation in the nasal cavity and two of them were not AIDS related , 24–26.Here, we present a case of KS of the left nasal cavity in HIV negative patient, treated by chemotherapy only.A 56-year-old female of Mediterranean descent presented with a 1 year history of swelling on the left side of her nose, nasal obstruction and occasional minor epistaxes. Physical examination showed a firm and bulging polypoid mass which filled the left nasal cavity without cutaneous lesions.Computed tomography (CT) Fig.  demonstrA biopsy specimen was obtained in the nasal cavity. Initially, the diagnosis of angiosarcoma was made. After multidisciplinary consultation meeting, a second pathological opinion was required. Original slides examination revealed a submucosal proliferation composed of intersecting ill-defined fascicles, of spindled cells with mild to moderate nuclear atypia. Slit-like spaces containing red blood cells were noted. Intracytoplasmic and variably sized hyaline globules were seen in some tumor cells. Immunohistochemical study revealed a strong expression of CD31, CD34, and vimentin. Tumor cells showed negative staining for AE1/AE3, CK5/6, HMB-45, and melan-A. As tissue was depleted in the paraffin block, we were unable to perform further immunohistochemical testing, using HHV 8 antibody. A final diagnosis of Kaposi’s sarcoma was rendered.A chest CT did not showed metastases in the lungs.Decision in multidisciplinary consultation meeting is to instaure neoadjuvant chemotherapy because surgical excision is not feasible. Then an evaluation must be performed. Chemotherapy drugs ifosfamide (IFO) and doxorubicin (DOXO) was started on December 22, 2014. The detailed treatment regimen was as follows: IFO 1800 mg/m2 i.v. d1-5; mesna dose is 100 % of the IFO dose (3 g) i.v. d1-5 and DOXO 60 mg/m2 d1.The cycle was repeated every 21 days. Physical examination after the first cycle of treatment showed a complete response. To keep the clinical response, the patient continued chemotherapy until four cycles. The fourth cycle date April 27, 2015.Computed tomography (CT) of evaluation showed a near complete response in the nasal cavity Fig. .Fig. 2AxThe patient received no further treatment. She needs a regular medical checkups that include a review of a patient’s medical history and a complete physical exam. She is in excellent local control over 12 months.Sinonasal cancers, which account for 3 % of all head and neck cancers, have their origin in the nasal cavity in 20–30 % of cases . It is rKS is an important opportunistic disease, which frequently occurs in HIV-positive patients. The incidence of KS involving head and neck was reported to be as high as 40–67 %, in which the cutaneous lesions predominate , 26.However, the involvement of this region is only 14 % of patients with HIV-negative , 35–41.Here, we reported a case de Kaposi’s sarcoma of the nasal cavity in patient with HIV-negative.KS involvement has been observed in almost all visceral sites, including lymph nodes, liver, pancreas, heart, the testes, bone marrow, bone, skeletal muscle and mucosa of head and neck , 43.The most frequent sites of noncutaneous disease are the oral cavity, gastrointestinal tract, and respiratory system –48.There is a few cases of the Kaposi’s sarcoma in the nasal mucosa , 26, 49.In a review of the literature, seven cases of the Kaposi’s sarcoma in the nasal mucosa were reported.Gnnep et al. discussed nine cases of KS arising in the head and neck region. One of them was in the nose-mucocutaneous junction with HIV-negative, treated by surgical excision and relapsed at 9 months .The second case of the KS in the nasal cavity with an adequate immune system was reported by Ioannis Venizelos et al. It was a case of a 59 year-old woman who presented a tumor arising from the left nasal septum which was excised. The patient received no further treatment and 4 years later, she is in excellent condition .Three patients with HIV-positive who had KS of the nasal cavity, were treated by surgical excision. One of them relapsed at 2 months , 25, 26.Patients with tumors of the nasal cavity usually present with a history of chronic sinus congestion and recurrent nasal obstruction. In our case, there was no ulceration of the overlying squamous epithelium and the patient presented with swelling on the left side of her nose, nasal obstruction and occasional minor epistaxes.Various clinicopathologic forms of Kaposi’s sarcoma have been described, which often have overlapping features . In the earliest stage of Kaposi sarcoma, a flat lesion made of miniature vessels surrounding larger ectatic vessels is noted , 51.In the more advanced stage of the disease, a discernible but relatively bland spindle cell component, centered around the proliferating vascular channels, can be described. Coalescent foci of spindle cells produce the classic nodular lesions of Kaposi sarcoma .The spindle cells are separated by slit and sieve-like spaces containing erythrocytes and vascular channels. Lymphocytes and plasma cells, hemosiderin deposits, and dilated vessels are commonly seen at the periphery. Both intracellular and extracellular hyaline globules may be identified .The tumor cells are strongly immunoreactive for CD31, CD34, factor VIII–related antigen, and Latent nuclear antigen-1 of HHV-8 (Kaposi sarcoma-associated herpes virus. They are variably immunoreactive for smooth muscle actin and are negative for cytokeratins (AE1/AE3 and CAM 5.2). The nuclear positivity using the LANA-1 antibody confirms the diagnosis , 51, 53.The histologic differential diagnoses of Kaposi’s sarcoma include angiosarcoma, fibrosarcoma, arteriovenous malformations, and spindle cell hemangioendothelioma.Clinicopathological features, mainly immunohistochemical studies, help by excluding these differential diagnoses, but some cases with unusual findings can pose a challenge to general pathologists , 52, 53.The major goals of treatment are symptom palliation, prevention of disease progression, and shrinkage of tumor to alleviate edema, organ compromise, and psychological stress .For HIV-positive patients who develop either limited or advanced Kaposi sarcoma (KS), it is recommended that treatment includes highly active antiretroviral therapy (ART).Local treatment modalities are useful for cosmesis or the management of symptomatic bulky KS lesions, but they do not prevent the development of new lesions in untreated areas. The most widely used local treatment approaches include:Intralesional chemotherapy with vinblastine, radiation therapy and topical alitretinoin.Indications for systemic chemotherapy include extensive cutaneous disease, symptomatic visceral involvement, and cutaneous KS that is unresponsive to local therapy.When chemotherapy is indicated, treatment with pegylated liposomal doxorubicin or liposomal daunorubicin is generally recommended as the first-line treatment for KS . Other aIn our case, chemotherapy seems to be effective, since 8 months later the patient is in excellent condition without evidence of local recurrence or metastasis both clinically and radiologically.Primary Kaposi sarcoma of the nasal cavity is a very rare entity. Our case represents the third reported case not associated with AIDS. Chemotherapy with doxorubicin seems to be effective with in excellent response.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Prenatal ultrasound showing a double bubble is considered to be pathognomonic of duodenal atresia. We recently encountered an infant with prenatal findings suggestive of duodenal atresia with a normal karyotype who actually had a jejunal duplication cyst on exploration. A finding of an antenatal double bubble should lead to a thorough evaluation of the gastrointestinal tract and appropriate prenatal/neonatal testing and management as many cystic lesions within the abdomen can present with this prenatal finding. Duodenal atresia has long been associated with the antenatal “double bubble” sign on ultrasound. This appearance results from a distended stomach and duodenal bulb that are separated by a hypoechoic gastric antrum. It is often associated with a collapsed small bowel and colon and maternal polyhydramnios. [1] While these findings are considered specific for obstruction at the level of the duodenum, specific signs such as a hyper-echogenic band in annular pancreas or anatomic location of the angle of Treitz in malrotation are not found in many instances to facilitate an exact diagnosis. [2] The “double bubble” monicker is mainly associated with duodenal atresia; however differential diagnoses in the literature clearly describe intestinal malrotation, midgut volvulus, annular pancreas and other right upper quadrant cystic lesions consistently. [1] We recently treated a neonate with an antenatal “double bubble” who on further evaluation was found to have a jejunal duplication cyst. The finding of an antenatal “double bubble” should include a thorough work up of the gastro intestinal (GI) tract beyond the ligament of Treitz as the causative pathology may not be restricted to the peri-ampullary area. A full-term 2610g male neonate was admitted to the intensive care unit (NICU) with an antenatal history significant for a “double bubble” detected during the second trimester ultra sound examination . A presumed diagnosis of duodenal stenosis/atresia was made with further karyotyping and anomaly scan showing no abnormalities. The antenatal imaging performed in the third trimester re-confirmed the findings of a double-bubble with no other anomalies being identified. Born at 38 +2/7 weeks, with APGAR scores of 7 and 9, the neonate had mild fullness of the right upper quadrant on clinical exam but no palpable mass. A supine abdominal x-ray revealed a dilated small bowel loop in the right upper quadrant with distal bowel gas, which was interpreted as a possible mega duodenum due to obstruction at that site consistent with the antenatal “double bubble” finding . Patient then underwent an upper gastrointestinal series (UGI), which initially revealed no evidence of gastric outlet and duodenal obstruction or malrotation. However, subsequent delayed films showed of a space-occupying lesion in the right upper quadrant with a characteristic displacement of small bowel loops. Its subsequent opacification on delayed films was suggestive of a duplication cyst . No evidence of bowel obstruction was seen. A post-natal ultrasound was not considered necessary in view of the findings on the UGI series.On day of life three, the patient underwent a diagnostic laparoscopy. Intra-operative findings included a 4x3 cm cystic mass in the right upper quadrant consistent with a duplication cyst , as well as a freely mobile cecum in the left abdomen consistent with malrotation. A directed laparotomy  with resection of duplication cyst and anastomosis of the small bowel along with a Ladd’s procedure were performed. No classical Ladd’s bands could be identified although there were numerous abnormal mesenteric bands compressing the distal small bowel that were divided. The patient was discharged home uneventfully after a two-week stay in the neonatal intensive care unit. From the onset of the prenatal diagnosis of a “double bubble”, which is mainly a second trimester finding, exclusion of Down’s syndrome becomes an integral part of the management. An antenatal karyotype or a triple test  must be completed to rule out Down’s syndrome. If, as has been rarely reported, the double bubble is detected in the first trimester, it may help facilitate further management of the pregnancy including counseling of the family with regards to their options including termination of pregnancy. The finding of a “double bubble” is non-specific and all differential diagnoses must be ruled out prior to the diagnosis of isolated duodenal atresia. Therefore additional antenatal ultrasonography should be performed to better visualize potential causes as well as neonatal tests to rule out conditions that require urgent operative intervention. Starting at fourteen to fifteen weeks’ gestation, antenatal ultrasound can be used to visualize intra-abdominal organs. While accurate diagnosis of obstructions or abnormalities occur closer to twenty-two weeks gestation, at fourteen weeks a distended fluid-filled stomach appears in the left upper quadrant along with a normally under-distended small bowel. Small bowel distention is therefore indicative of distal GI pathology, either distal small bowel or colonic obstruction. [4] Duplication cysts are rare congenital malformations with an incidence of one in forty-five hundred live births. [3] These cysts always require resection either urgently because of obstructive symptoms or electively for potential malignant degeneration. Fifteen percent of the time there are synchronous cyst along the gastrointestinal tract; reports have documented occurrences from esophagus to rectum. [5] Duplication cysts are more common in males and have a predilection to the ileum. [3] Duplication cysts also have ultrasonographic criteria however they are not present in all occurrences.  Studies show that duplication cysts detected on antenatal ultrasound must include a double wall rim consisting of an inner hyperechoic rim of mucosa-submucosa, and an outer encompassing hypoechoic layer of muscularis propria. [8] Contrast radiography may show a mass lesion that communicates with the GI tract or an extra-intestinal mass displacing adjacent bowel loops.The classic “double bubble” sign represents an over-distended stomach with an abnormally distended duodenal bulb. On transverse plane of an ultrasound this appears as two large echogenic lumens separated by a hypoechoic gastric antrum. [4] No dimensional requirements or criteria for the size of the lumens or the antrum exist as the non-specific finding of a double bubble is visualized with antenatal ultrasound. With that being said, ultrasonographic findings of a polyhydramnios (of variable severity) and double bubble are still historically pathognomonic for duodenal atresia, which occurs in one in every ten thousand live births. [4] The majority of duodenal atresias are isolated developmental vascular accidents, however thirty percent of cases are associated with trisomy 21 Down’s syndrome. [6] Other anomalies responsible for the visualization of an antenatal double bubble include, malrotation, volvulus, annular pancreas, duplication cysts, internal hernia, and cystic lesions of the right upper abdomen such as a choledochal cyst.  An UGI study or other postnatal imaging can be obtained to confirm the exact diagnosis. A double bubble detected on antenatal ultrasound is a serious finding that usually implies an obstruction proximal to the ligament of Treitz. It is well documented that there are many anomalies that mimic a double bubble sign so additional testing is mandatory. We herein report the first case of a large jejunal duplication cyst mimicking a double bubble sign: a “pseudo double bubble”. We believe based on our recent experience that in some neonates the causative pathology of an antenatal “double bubble” may lie far distal to the ligament of Treitz and a thorough evaluation of the entire GI tract is necessary to come to a correct diagnosis and perform the appropriate intervention. Source of Support: NilConflict of Interest: None


Epithelioid hemangioma, also known as angiolymphoid hyperplasia with eosinophilia, is a cutaneous angioproliferative lesion that follows a benign clinical course. It is most frequently localized in the skin of the head and neck region; although it may sometimes arise deeper in soft tissues, orbital involvement is rare. Here we describe a patient who developed multiple epithelioid hemangiomas, including an intraorbital lesion. The histopathological parallels with other reactive and neoplastic lesions as well as therapeutic options are discussed. Epithelioid hemangioma (EH) is an uncommon vasoproliferative process of the skin and less frequently soft tissues that usually affects middle-aged adults . EH typiA definitive diagnosis requires histological evaluation: EH has distinctive morphological features consisting of proliferation of blood vessels lined by abnormal endothelial cells, accompanied by lymphocytic and eosinophilic inflammatory infiltrate , 3. ImpoA 71-year-old woman with a history of sarcoidosis and non-Hodgkin lymphoma of the gastrointestinal tract was admitted to the neurosurgery department for crushing pain in the right eye, headaches, and double vision. A year before, a tumour of the right orbit was partially excised. Histopathological examination at that time showed fibrous tissue with vascular proliferation . The natAt the time of admission to the hospital, exophthalmos of the right eye was evident with paresis of the third and fourth cranial nerves. Visual acuity was 0.8 and had not changed since previous hospitalization. The patient complained of double vision, which was exaggerated in the gaze directions of the affected orbital muscles. No other cranial nerve abnormalities were noted.MRI of the head revealed a mass of the right orbit, about two centimetres in diameter . Some coSurgery was performed through right pterional approach with retrepanation of the cranial bone and complete orbitotomy. A fibrous tumour was found in the periorbital adipose tissue, embedded in scar tissue and extending to the fibrous coverings of the globe, partially adherent to the extraocular muscles. The tumour was completely resected.After surgery, exophthalmos and ocular pain slowly subsided, as did paresis of the third and fourth cranial nerves, although some minor deficit of ocular motion persisted . HistopaIn addition, we retrieved two older biopsies of the same patient from our archives. Both were performed two years earlier; the first one was a skin biopsy of the scalp and the second was a biopsy of the right orbital adipose tissue and lacrimal gland. The lesion of the scalp also displayed histopathological features of EH, while interestingly in the lacrimal gland granulomatous inflammation turned out to be consistent with sarcoidosis.Also, a new tumour localized on the eyelid has developed a few months after surgery. This lesion has not been histologically verified yet, but according to its clinical features, it could represent another EH.EH was first described in 1969 as angiolymphoid hyperplasia with eosinophilia , 8 and, The orbital tumour exhibited convincing histopathological elements of EH, evident in particular in the second biopsy and closely resembling the lesion previously excised from the scalp. Nevertheless, a differential diagnosis in particular with diseases that more commonly affect the orbit had to be considered. This included reactive processes, lymphoproliferative disorders, and some vascular neoplasms.Several reactive processes had to be ruled out. In this category, the main differential diagnosis was with Kimura's disease (KD), since EH shares with KD some clinical and histologic features. Both KD and EH usually manifest as nodules or plaques in the head and neck region. KD probably represents an allergic or autoimmune response and, unlike EH, it often develops systemic features such as blood eosinophilia and nephrotic syndrome, asthma, tuberculosis, or Loeffler syndrome –11. KD dBased on tumour location, the second major reactive process to be excluded was idiopathic orbital inflammation (IOI). IOI is characterized histologically by a mixed inflammatory infiltrate and in later stages fibrosis, but again abnormal endothelial proliferation is not seen .Since the cellular infiltrate was rich in lymphoid cells, the second group of diseases to enter into the differential diagnosis was lymphoproliferative disorders. This diagnosis was excluded considering the reactive appearance of the infiltrate, which was composed of a mixed population of well-differentiated lymphatic cells.Vascular tumours represent the third family of lesions that had to be considered. Among benign vascular tumours, especially arteriovenous hemangioma (cirsoid aneurism) had to be taken into account. Cirsoid aneurisms also present as a nodule or papule in the head and neck region of middle-aged or elderly adults. Histologically, they are characterised by a combination of thin and thick-walled blood vessels with no epithelioid endothelium. Malignant vascular neoplasms, in particular epithelioid hemangioendothelioma and angiosarcoma, also had to be excluded ; accordiThe aetiology of EH remains unknown. It is either a benign neoplastic proliferation of blood vessels or a reactive hyperplasia of vascular structures, which develops in response to trauma, and possibly other triggers such as hyperestrogenic states  or infection –15. It iThere are many treatment options for EH depending on the extent, location, and size of the lesions . The mosIn conclusion EH, although rare at this site, enters into the differential diagnosis of orbital masses. Histopathological examination is mandatory in order to exclude other tumours and tumour-like lesions. Despite being a benign process, its disrupting growth may cause severe symptoms, spontaneous regression is uncommon, and lesions often recur, so complete removal is the therapy of choice.


We report the case of two-year-old girl with hypovolemic shock caused by bleeding from an abdominal cystic lymphangioma. The whole blood was contained within a large omental bag that could be completely removed. There were no associated anomalies. The child progressed satisfactorily. Abdominal cystic lymphangioma is not a common disorder during childhood. It usually presents with abdominal pain or acute abdomen but also presents as an asymptomatic mass.Two-year-old girl, whose father died of an aortic aneurysm in Marfan's disease, showed progressive enlargement of the abdomen, as well as pallor and fatigue of at least two weeks later. She was brought to the emergency department with weakness, apathy, and pallor. AP abdominal radiograph  shows meLymphangiomas are benign tumors of the lymphatic vessels usually located in subcutaneous tissue of head, neck and armpit, and more rarely in abdomen –5, beingThe pathological examination of the masses located in abdomen usually shows thin-walled cavities of different sizes and may contain lymph, pus when infected, or blood as a result of bleeding . Our casCystic Lymphangioma should be considered as a possible cause of massive abdominal hemorrhage.