Osgood-Schlatter disease (OSD) is a condition in which the patellar tendon insertion on the tibial tuberosity becomes inflamed. It is a well-known condition in late childhood characterized by pain and a bony prominence over the tibial tuberosity. The pain is usually exacerbated by physical activities like running, jumping, and climbing stairs. In the acute stage, the margins of the patellar tendon become blurred in radiographs due to the soft tissue swelling. After three to four months, bone fragmentation at the tibial tuberosity is viewed. In the sub-acute stage, soft tissue swelling resolves, but the bony ossicle remains. In the chronic stage, the bone fragment may fuse with the tibial tuberosity which can appear normal.The primary goal in the treatment of OSD is the reduction of pain and swelling over the tibial tuberosity. The patient should limit physical activities until the symptoms are resolved. In some cases, the patient should restrict physical activities for several months. The presence of pain with kneeling because of an ossicle that does not respond to conservative measures is the indication for surgery. In these cases, the removal of the ossicle, surrounding bursa, and the bony prominence is the treatment of choice. Osgood-Schlatter disease  is a condition in which the patellar tendon insertion on the tibial tuberosity becomes inflamed -2. It isPain and swelling are the primary symptoms felt in the lower aspect of the knee, around the patellar attachment to the tibial tuberosity Figure  7-8].-8.7-8].The pain is usually exacerbated by physical activities like running, jumping, and climbing stairs. Knee extension against resistant and other sports-related activities may worsen the symptoms. In 20% to 30% of cases, both knees are affected, but symptom intensity can vary in each knee.In the acute stage, the margins of the patellar tendon become blurred in radiographs due to the soft tissue swelling. After three to four months, bone fragmentation at the tibial tuberosity is viewed. In the sub-acute stage, soft tissue swelling resolves, but the bone ossicle remains. In the chronic stage, the bone fragment may fuse with the tibial tuberosity which can appear normal. However, sometimes the bone fragment is displaced Figure .Ultrasound may be useful as it determines any swelling in the soft tissue, cartilage, bursa, and tendon -10. It aThere are some other diseases that should be considered in the differential diagnosis of OSD, such as Sinding-Larsen-Johansson syndrome, Hoffa’s syndrome, soft tissue or bone tumors, patellar tendon avulsion or rupture, chondromalacia patella, patellar tendinitis, infectious apophysitis, accessory ossification centers, osteomyelitis of the proximal tibia, and tibial tubercle fracture Table  12]..12].The primary goal in the treatment of OSD is the reduction of pain and swelling over the tibial tuberosity. For this reason, the patient should limit physical activities until the symptoms are resolved, which can sometimes take several months. Other conservative treatments include the use of ice packs, NSAIDs like ibuprofen and naproxen (to reduce pain and swelling), protective padding, and physiotherapy.PhysiotherapyExercises for the improvement of the quadriceps, hamstrings, and gastrocnemius muscles are recommended. Immobilizing the knee (using a cast or brace) can reduce strain on the tibial tuberosity ,14.SurgerySurgery is indicated when ossicle pain on kneeling persists despite conservative treatment measures. In these cases, removal of the ossicle, surrounding bursa, and the bony prominence is the treatment of choice . For a gPihlajamäki, et al. studied the outcomes of the patients who did not respond to conservative measures and were surgically treated . In a teThe detailed algorithm for treatment of OSD is presented in Figure RehabilitationIn this period, the focus is on muscle strengthening, gait training, and pain control to restore a functional knee.OSD is usually a self-limiting disease, and symptoms may resolve without any particular treatment or with simple conservative treatment, like activity restriction or immobilization in a cast for three to six weeks. Surgery is rarely indicated for OSD. Krause, et al. studied the natural history of untreated OSD. In 69 affected knees in 50 patients, they found that 76% of patients had no activity restriction, but only 60% of patients [PME1] could kneel without pain . They suIf the symptoms persist, surgery may be indicated. One trial noted, however, that after tibial sequestrectomy , clinical results were no better than those seen with conservative treatments . BoswortOsgood-Schlatter disease (OSD) is usually a self-limiting disease mainly seen in late childhood. The symptoms usually resolve without any specific treatment or with simple conservative treatment such as the restriction of activity or immobilization in a cast for three to six weeks. Surgery is rarely indicated for OSD. If the symptoms persist and become disabling to the patient, surgery may be indicated especially after the fusion of the apophysis. The excision of the ossicle is the common surgical method, and removal of any prominences on the tibial tuberosity is optional.


CT appearance is that of a mixed‐density lesion with solid component peripherally and cystic component centrally. Even larger tumors are well encapsulated with sharp demarcation, amenable to complete resection.Solid pseudopapillary tumor is a rare pancreatic neoplasm that typically affects young women. Characteristic  A 25‐year‐old woman presented with dull‐aching pain in the upper abdomen for 1 year. Per‐abdominal examination revealed a fixed, firm, and nontender lump in the epigastrium. Blood counts, liver function test, and serum amylase–lipase were within normal limit. Computed tomography image and intra‐operative pictures are as below. What is the correct diagnosis?Gastrointestinal stromal tumor of stomachHydatid cyst of pancreasSolid pseudopapillary tumor of pancreasPseudocyst of pancreasSolid pseudopapillary tumor of pancreas is classically seen in young women and commonly present with upper abdominal pain Although hydatid cyst of the pancreas has been reported, it is extremely rare. CT scan in the present case shows a mass with solid–cystic component. This does not correlate with features of hydatid cysts, where lesion is cystic with multiple daughter cysts within it.Pseudocyst is the most common form of cystic lesion in pancreas. However, it more frequently affects men and is associated with a history of pancreatitis or trauma in majority of the cases. CT picture shows a homogenous hypodense lesion, without any solid component. Absence of these clinical and radiological features suggested different diagnosis in the above case.Similarly, GIST most commonly arises from the stomach and presents with epigastric pain, lump, and recurrent vomiting resulting from gastric outlet obstruction. Moreover, it is diagnosed more commonly in elderly men. CT shows predominantly solid tumor, although central necrosis or hemorrhage may produce solid–cystic‐like picture. In the present case, the mass was seen arising from the pancreas, which could be completely isolated from the surrounding structures, making the diagnosis of gastric GIST unlikely.None declared.SK: wrote the manuscript and is the operating surgeon. RR: collected images and data, wrote the manuscript, and reviewed the literature. AC: edited the manuscript, performed critical revision, and reviewed the literature.


Inflammation in ectopic pancreatic tissue can clinically present with pain or obstructive symptoms, depending on the location of the ectopic tissue. We present a rare case of gastric outlet obstruction secondary to pancreatitis of ectopic pancreatic tissue in the pylorus. Heterotopic pancreas is defined as pancreatic tissue that lacks anatomical or vascular communication with the normal body of the pancreas . Most coA thirty-five-year-old male patient presented to the hospital for evaluation of 3 months of nausea, vomiting and a 25 lb weight loss. The patient was initially seen for these symptoms at an another hospital, at which point his laboratory test results were unremarkable with the exception of an elevated lipase at 1900 U/L . The patient had no significant past medical history and he reported alcohol use of up to 18 cans of beer per week over the past 10 years. A computed tomography (CT) scan of the abdomen and pelvis was performed; it did not reveal any evidence of pancreatic or peripancreatic edema but showed wall thickening, edema and abnormal enhancement in the region of the pylorus and gastric antrum . The patThe patient’s symptoms continued to progress and he presented to this hospital for further evaluation. His laboratory results on presentation showed a mildly elevated lipase (471 U/L) , with an unremarkable complete blood count, electrolytes and liver panel. A repeat CT scan of the abdomen and pelvis with intravenous contrast showed thickening and enhancement of the gastric pylorus and proximal first duodenal segment, with a well-defined fluid collection within the wall .The patient underwent an esophago-gastroduodenoscopy (EGD), which showed mild focal pre-pyloric edema and erythema. Biopsies were unremarkable. He continued to be symptomatic and an endoscopic ultrasound (EUS) was performed, which showed diffuse, moderately congested mucosa in the pre-pyloric region of the stomach, the pyloric channel and the proximal portion of the duodenal bulb. There was asymmetrical hypoechoic thickening of the wall of the pylorus, with loss of the normal five-layered echostructure in this region . Fine-neInflammation within ectopic pancreatic tissue was suspected and, given his continued symptoms, weight loss and atypical appearance on CT and EUS, the patient was referred for surgical intervention. He underwent a distal gastrectomy. Given the degree of edema in adjacent duodenal tissue, a gastroduodenostomy was not feasible and, as a result, a Roux en y gastrojujenostomy was carried out. The pathology report was consistent with distal stomach with ectopic pancreas showing moderate chronic pancreatitis .Heterotopic pancreas is a relatively common congenital anomaly that is reported in 1–2% of autopsy cases .Heterotopic pancreas is usually asymptomatic and may be noted incidentally during endoscopy or imaging studies; it may become clinically evident when complicated by pathological changes such as inflammation, bleeding and malignant transformation. Symptoms would depend on the anatomical location of the ectopic tissue and the condition will manifest itself through obstructive symptoms or pain . LesionsHistologically, ectopic pancreas is classified as Type 1, composed of acini, duct and islets, Type 2, composed of ducts only, Type 3 consisting of acini only (exocrine pancreas) and Type 4, which is composed of islets only (endocrine pancreas) [Imaging studies, CT scans and EUS can all be utilized for evaluation of these lesions; however, pre-operative diagnosis can be difficult. On EGD, ectopic pancreas is noted as a firm, round, sub-epithelial lesion with a central depression that is thought to represent the opening of the duct. This last finding is present in less than 50% of cases, making it difficult to differentiate from other submucosal lesions, such as gastrointestinal stromal tumor (GIST) , 5. The Despite the relatively common observation of ectopic pancreatic tissue during EGD, inflammation causing gastric outlet obstruction is exceedingly rare and limited to few case reports . The manOur patient first presented with inflammation in an area of ectopic pancreas; he later developed an acute fluid collection in that area and continued to be symptomatic, requiring further evaluation and eventually surgery: pathology was consistent with Type 1 ectopic pancreas, with features of chronic inflammation. We speculate that heavy alcohol use may have caused his pancreatitis; however, this association cannot be proven. It is not clear at this time whether or not alcohol can cause ectopic pancreatitis without causing pancreatitis of anatomical pancreas. Although the most common cause of gastric outlet obstruction and elevated lipase without associated pancreatitis is penetrating peptic ulcer disease, other less-common etiologies should be considered.Conflict of interest statement: none declared.


The aim of our study is to enhance the awareness of blue rubber bleb nevus syndrome (BRBNS) through the patients in our hospital and introduced a new measure of endoscopic intervention.A retrospective review of 5 patients, who were diagnosed as BRBNS in our hospital from January 2013 to January 2017, was conducted. Data were collected with regard to demographics, clinical presentation, endoscopic and imaging findings, management, and follow-up data.In total of 5 patients, the mean age was 28.8 years, range 16 to 44 years  with the average initial age of onset 15.4 years. No family history was identified in our group. Physical examination showed multiple cutaneous lesions in 2 patients . All the 5 patients had gastrointestinal tract vascular malformations; stomach involved in 2 cases, large intestine in 2 cases, and small intestine involved in 3 cases. Lesions in the visceral organs and tissue were found in 1 patient. Gastrointestinal bleeding was its main symptom . Laboratory investigations revealed anemia in 4 patients and abnormality of coagulopathy in 2 patients with severe anemia. Conservative approach was recommended in 3 cases that included iron supplementation, drug hemostasis, and/or blood transfusion. An innovatively therapeutic approach with endoscopic submucosal dissection (ESD) procedure was used successfully in 1 patient with 2 polypoid BRBNS lesions in rectum.BRBNS is a very rare vascular malformation syndrome with unclear etiopathogenesis and noncurative treatments. ESD procedure was a feasible approach to remove the partial gastrointestinal lesions. To the clinicians, gastrointestinal bleeding and secondary iron deficiency anemia were the most common symptoms ascribed to the GI tract lesions. The onset of the disease could be mostly traced from birth, infancy, or early childhood; only few patients started after adulthood, and the lesions increased in size and number with the advancing age. However, due to its low incidence and atypical clinical symptoms, it was easily misdiagnosed in the early stage. To date, only few hundreds of cases have been reported since William Bennett Bean brought the BRBNS to our attention.Blue rubber bleb nevus syndrome (BRBNS), also known as the Bean syndrome, is a very rare disorder characterized by multiple vascular malformations of the skin, gastrointestinal (GI) tract, and, less often, other visceral organs.Up to now, more and more literatures across the world have described this rare syndrome; nevertheless, the majority of the articles were case reports. In our manuscript, we retrospectively reviewed 5 patients admitted to our hospital who had been diagnosed with BRBNS in the 4 years between 2013 and 2017. We mainly analyzed their clinical characteristics, diagnosis, endoscopic and imaging findings, and treatment to enhance the awareness of the physicians. In addition, we reported a new therapeutic approach through endoscopic submucosal dissection (ESD), which was first to be reported to our knowledge.2From January 2013 to January 2017, a total of 5 BRBNS patients were found in our hospital at a mean age of 28.8 years, range 16 to 44 years . The symptoms included the following: melena in 1 case, hematochezia in 1 case, abdominal pain and nausea in 1 case, fatigue in 1 case, and positive of fecal occult blood in 1 case. The initial ages of onset in the 5 patients were 7, 9, 14, 15, and 32 years, respectively, with the average age of 15.4 years. In the past, 2 patients had the surgical operation for removal of venous malformations: in one patient it was in colon, and in the other, it was located in lung. The long-term iron supplementation and occasional blood transfusion had been tried in 2 patients for many years before hospitalization. Multiple painless bluish nodular cutaneous lesions were found in 2 patients; physical examination revealed multihemangiomas appeared in the skin of trunk, limbs, tongue, etc. The other 3 patients had no skin lesions. There was no family history identified in our 5 cases. Table We obtained human subjects approval from Ethics Committee of The First Affiliated Hospital, College of Medicine, Zhejiang University (Reference Number 2017-389). The record/information of patients were anonymized and de-identified prior to analysis. Our institutional review board approved this study with a waiver of informed consent.33.1Both gastroscopy and colonoscopy were performed in all 5 patients: 3 patients finished the whole GI screening with capsule endoscopy (CE) examination. The locations of hemangiomas were listed as follows: 1 case only in stomach; 1 case in duodenum, jejunum, and rectum; and 1 case in jejunum and ileum. The other 2 patients, whose lesions were detected in stomach and in colon, respectively, were not examined by CE or double balloon enteroscopy (DBE), so their small bowel could not be evaluated endoscopically. However, abdomen computed tomography (CT) showed the small bowel was involved in 1 of these 2 patients.Generally, endoscopic examination would show multiple, congested, bluish, tender, nodular hemangiomas in the GI tract. Clinically, various appearances may be present: sometimes they appear as strawberrylike mucosal polypoid, but have a submucosal protrusive lesion at the other times. The typical endoscopic images of BRBNS were shown in Figure 3.2d-dimer in the 2 patients with severe anemia, whereas the concentration of fibrinogen decreased. The hemoglobin level was normal in 1 patient with abdominal pain and nausea, the fecal occult blood was also negative. No abnormality of coagulopathy was found in this patient.Laboratory investigations revealed anemia in 4 patients, whose hemoglobin levels at the time of admission were 48, 55, 89, and 96 g/L, respectively. The red blood cells were microcytic and hypochromic, which indicated iron deficiency anemia. Laboratory tests also showed abnormality of coagulopathy, presented as the elongation of prothrombin time (PT), activated partial thromboplastin time (APTT), and the raised All of these 5 patients received the CT scan of the abdomen, which may be characterized by the thickening of bowel wall, nodular or irregular elevated lesions, soft-tissue masses in the GI, or abdominal cavity or viscera; and calcification of the hemangioma could be seen in partial cases. Enhancement in venous phase was sometimes observed through the inspection of the contrast-enhanced CT images. CT may help to identify extraintestinal lesions also. In our group, 1 patient revealed multiple vascular malformations on the liver, mediastinum, pelvic cavity, abdominal cavity, muscles of buttocks, and iliacus. However, all of the lesions were low density with no enhancement but multipunctate calcifications Fig. . CT scan3.3In our group, in 1 case admitted for repeated hematochezia, 2 polypoid BRBNS lesions, sized 0.6 × 0.8 and 0.5 × 0.6 cm, respectively, were detected in the rectum. To resect completely, we adopted innovative endoscopic measures—ESD in BRBNS, which was extensively used in the resection of early gastric cancer (EGC), gastrointestinal stromal tumors (GIST), etc. The ESD procedure was similar to other operations of GI tumors, which contained lesion marker, submucosal injection, circumferential incision, submucosal dissection, wound treatment, etc Fig. . LiftingConservative approach was recommended in the other 3 cases, which included iron supplementation, drug hemostasis, and/or blood transfusion. One patient with multigastric BRBNS, who chiefly complained of abdominal pain and nausea, showed negative fecal occult blood in laboratory tests, and had normal hemoglobin level, accepted the drug of proton pump inhibitors (PPI) and gastroprotective agents. All of the 5 patients were told to eat soft foods for rest of their lives. No antiangiogenic agents were used in our group because of the uncertain response. No massive sudden hemorrhage occurred, so surgery was not available.3.4After a minimum follow-up of 6 to 12 months in the 4 patients with anemia, 2 patients demonstrated a favorable response to iron supplementation; the hemoglobin level increased obviously . One patient with multigastric and small bowel lesions remained melena repeatedly after her discharge from the hospital; the hemoglobin level was still in lower level, laparotomy was refused by surgeon because of its extensive involvement in GI. So thalidomide treatment was administered to the patient after her informed consent. The case with ESD in the rectum had no GI bleeding again during the follow-up time within half a year; the hemoglobin level increased from 55 to 70 g/L. Another patient with multigastric BRBNS showed no melena or positive fecal occult blood.4 The etiology and pathogenesis of this syndrome remained unknown; locus on chromosome 9P, elevated c-kit expression may be involved on molecular and genetic level. Although an autosomal dominant inheritance had been described in several familial cases, the majority of the cases appeared to be sporadic; no family history of BRBNS was found in our group also. The venous malformations were primarily located on the skin and GI tract. Xue-Li Jin reviewed 120 cases from the literature; cutaneous angiomas were observed in 112 cases, which accounted for 93%, and GI lesions were observed in 76% cases. In our group, skin lesion appeared in 2 patient (40%), GI lesions were detected in all the 5 patients (100%). There seemed to be a positive correlation between the number of GI lesions and the number of cutaneous hemangiomas. In the reviewed cases, other parts of the body may have been affected, such as liver, spleen, pancreas, kidney, bladder, eye, brain, heart, lung, muscle, peritoneal cavity, thyroid, thymus, submandibular and parotid regions, adrenal gland, and joints.,10William Bennett Bean brought the BRBNS to our attention and named the condition in 1958, although Gascoyen had described it almost 100 years previously. Skin lesions were seldom treated unless they were cosmetically unacceptable or functionally troublesome. Several studies have shown effective methods for the treatment of cutaneous lesions like laser, surgical removal, electrodesiccation, cryotherapy, and sclerotherapy. No treatment was intervened to the skin lesions in the 2 patients in our hospital because of the absent symptoms.Skin lesions were usually observed through physical examination that could appear anywhere on the body but predominantly occurred on the trunk and upper limbs. The characteristic cutaneous lesions consisted of deep blue or purple, soft, rubbery blebs, easily compressible and turned to white; they may also lie deep in the skin and appear as bluish macules. Cutaneous lesions were presented in 2 patients in our study, multihemangiomas appeared in the skin of trunk, limbs, tongue, buttocks, etc. Complaint of painful lesion was reported in few patients because of the contraction of smooth muscle fibers surrounding the angioma.,14 On the morphological aspects, just like Kang Kook Choi's report, GI BRBNS could arise in diverse forms, including polylobulated, nodular, sessile, pedunculated, or ulcerated, and submucosal protrusive was also its appearance in our patients. Venous malformations could distribute throughout any segment of GI tract from the oral mucosa to the anal canal and mostly located in small intestine and distal colon. Some literatures also reported small intestine was the most frequently involved extracutaneous organ. In our 5 cases, gastric hemangioma was found in 2 cases (40%) and large intestine hemangioma in 2 cases (40%). In 3 cases (60%), small intestine was involved through CE and abdomen CT. With the examination of EUS, we could detect the typical hypoechoic lesions with luminal structure predominantly involving the mucosa and submucosa. Muscularis propria, even the whole layer could be affected. The transmural nature of lesion was reported in partial case reports based on the operation results, which might reveal the infeasibility and high-risk of the endoscopic intervention in partial BRBNS lesions.For BRBNS-involved GI tract, endoscopy was the first-line diagnostic tool and it enabled immediate therapeutic measures, especially in bleeding patients. Gastroscopy, colonoscopy, and CE were recommended for every BRBNS to screen the whole GI tract. Usually, the GI lesions presented at a later age but continued throughout life; the GI bleeding mainly manifested as intermittent melena, and sudden massive hemorrhage rarely occurred. Rarely, abdominal pain, intussusception, volvulus, infarction were its complications.D-dimer, and decrease in the concentration of fibrinogen, was found in our patient with severe anemia. In addition, thrombocytopenia, or chronic lymphocytic leukemia may occur in literature. CT examination especially contrast-enhanced CT was the most valuable noninvasive screening procedure in BRBNS which could identify GI hemangiomas and extra intestinal lesions. On the contrast-enhanced CT images, the lesions were demonstrated by the thickening of bowel wall, nodular or irregular elevated lesions, soft-tissue masses, and enhancement in venous phase. Systemic complications, such as thrombosis and the calcification of vascular lesions could also be observed through CT. In one of our patient, low-density lesions with no obvious enhancement but multipunctate calcifications were located in liver, mediastinum, pelvic cavity, abdominal cavity, muscles of buttocks, and iliacus. Like in Vig EK's report, it was important to examine the sites of possible vascular malformations as a routine measure, especially in the central nervous system. We suggested CT or magnetic resonance imaging (MRI) of brain was necessary to exclude brain hemangiomas that involved fatal cerebral hemorrhage. CT could also be helpful in evaluating the appeared abdominal complications and made some preliminary location judgments in active GI bleeding of BRBNS.A diagnosis of BRBNS was made on the basis of the typical skin lesions or GI lesions. Ancillary examination was helpful in severity evaluation. The laboratory test may reveal anemia, abnormality of coagulopathy, and positive fecal occult blood. The anemia was characterized by hypochromic microcytic anemia with iron deficiency, all that indicated iron deficiency anemia. Abnormality of coagulopathy, which presented as the rise of PT, APTT,  drug hemostasis and iron supplementation had good clinical outcomes in our partial patients with mild bleeding. Antiangiogenic agents, such as corticosteroids, propranolol, thalidomide, and interferon-a, and immunosuppressive agents like sirolimus had been described in treating vascular lesions and lessening the frequency of bleeding. The majority of therapies were only limited to case reports which lacked long-term prospective data. In our study, 1 case adopted the pharmacological therapeutic strategies of thalidomide because of the recurrent gastrointestinal bleeding in the period of follow-up. Progress in endoscopic technology had advanced medical practice, endoscopic interventions such as argon plasma coagulation, band ligation, endoloops, or lauromacrogol injection (sclerotherapy) had been suggested for the treatment of vascular lesions, and good clinical outcomes had been reported. Lesions located in small intestine and transmural lesions were relatively formidable and had high risk of perforation that should be viewed with great caution. To date, the role of surgical resection in the management of GI BRBNS was deemed controversial. In Hasan Yuksekkaya's literature, surgical resection had been condemned as overly aggressive and unhelpful because of the belief that lesions would recur after removal; the risk of short bowel syndrome should be considered if the GI lesions were extensive. Conversely, Zhao-Hui Deng's review described surgical wedge excision of angiomas or segmental resection of involved bowels was recommended in case of severe or recurrent hemorrhage, and surgery combined with intraoperative endoscopy intervention was proposed. We also thought surgical resection was essential in patients with recurrent bleeding, and complete visualization of the entire GI was necessary before operation through gastrointestinal endoscope. Staged operative procedures may require in patients with an overwhelming number of lesions. In the postoperative period, antiangiogenic agents or immunosuppressive agents could also be tried to prevent the recurrence of bleeding.Nowadays, although a wide range of therapeutic options had been described in the literature, there was no consensus on the treatment of the GI tract vascular malformations. The prognosis and treatment depended on the extent of GI tract involvement and the symptoms at presentation. Diet was important to the GI BRBNS patients; soft food was therefore required particularly for patients with the history of GI bleeding. In accordance with reports, histologically microscopic examination showed dilated and hyperplasia capillaries in the lamina propria of mucosa consistent with cavernous hemangiomas. The complete resection of lesions may reduce the recurrence of new angiomas compared with other endoscopic managements and increased the quality of life. However, ESD for BRBNS also had its limitations like small bowel lesion, transmural lesion, lesions with widely involvement. So we suggested the gastric and colonic BRBNS located above the submucosa, polypoid lesion, and single or less than 5 lesions were the main indications. Lesions located in the duodenal bulb and the descending part of the duodenum could also be tried.As we all know, ESD had launched a new era of broadened indication for endoscopic resection especially in early GI cancer, GIST, leiomyoma, laterally spreading tumor (LST), etc. Here we reported a new therapeutic approach using ESD in BRBNS lesions, which had not been reported because of its low incidence and high risk. Two rectum vascular malformations were resected completely in our center without intraoperative and postoperative complications. The ESD procedure was carried out in a standardized traditional way. EUS combined with intraoperative lifting-sign was helpful in pointing out the involved layer of the lesions to avoid transmural BRBNS. Because of its hemangioma characteristics, like the skin lesions, the lesion's color would turn lighter after the submucosal injection of the mixture with glycerol fructose solution, indigo carmine, and 0.001% epinephrine. In accordance with literature,In summary, BRBNS is a very rare vascular malformation with unclear etiopathogenesis and noncurative treatments. The diagnosis relied to the typical skin lesions and/or GI lesions. A wide range of therapeutic options including medical management, endoscopic intervention, and surgical resection had been proposed. However, sufficient evidence, long-term prospective data, and new available therapies remain to be searched in the future. Meanwhile, we deduced that ESD procedure is a feasible approach to remove the partial GI lesions. Because of the recurrences and incurable characteristics of GI hemangioma, every patient should be counseled about the chronicity of this disease and regular follow-up throughout their life.


Dear SirRectal atresia is categorized in rare variants of anorectal malformations (ARM) and comprised of only 1-2% of all cases of ARM. Similarly, congenital pouch colon (CPC) is placed under the rare malformations category. Although it is scarce in prevalence in most part of the world, however, it forms 10-20% cases of ARM in south Asian countries like India, Pakistan, and Nepal. Concurrent presence of rectal atresia and CPC is exceedingly rare and only 4 cases have been described so far.[1-4] We are reporting another case and first from Pakistan, of this concurrence. A 2-day-old male neonate, weighing 2.5 kg, presented with failure to pass meconium since birth. On examination abdomen was distended but not tense or tender. Perineal examination showed normal buttocks and normally placed anus. A rectal examination with thermometer could not negotiate beyond 2-3 cm from anal verge. The invertogram, with contrast filled nelaton tube in the anus, showed a distance of about 2-3cm between proximal end of nelaton tube and the distal most dilated gas shadow . Supine x-ray abdomen depicted air vesicogram besides dilated bowel shadows. A preliminary diagnosis of rectal atresia with rectourinary fistula was made. At operation, type IV congenital pouch colon was encountered  which was excised with repair of colovesical fistula; and end colostomy was made. At the age of 3 years, abdomino-PSARP was done to anastomose mobilized end colon with the rectal pouch. Patient is doing fine on follow-up.Mathur et al, reported first case of this concurrence in 2002 followed by Kazez et al in 2009, Parelkar et al in 2010, and Singh et al in 2011. The diagnosis is suspected when a in a normally placed anus, rectal examination cannot negotiate thermometer beyond 2-3 cm from anal verge; and abdominal radiograph shows a big gas shadow in the abdomen with or without air vesicogram. Except the patients without colovesical fistula, there is meconuria may be present which may at times decompress the pouch colon and precludes its detection on abdominal roentgenogram. Majority cases of rectal atresia can eventually be managed with PSARP approach, however, in case of concomitant CPC, abdominal approach is always added to address CPC and associated colovesical fistula. The reported cases have been managed by primary abdomino-transanal pull through and staged abdomino-PSARP procedures. In case of staged approach, the pouch must be excised with ligation or repair of colovesical fistula during first stage with formation of end stoma as we did during initial surgery in neonatal life. In case of retention of pouch with big fistula various complications can arise; Singh et al reported hyperchloremic metabolic acidosis in their case where urine started collecting in the retained CPC through a big fistula.Source of Support: NilConflict of Interest: None


Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extracardiac sites. Even though the head and neck region is the most common area of extracardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia, and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult-type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin, and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A, and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment. Rhabdomyoma (RM) is an uncommon benign mesenchymal tumor showing skeletal muscle differentiation that may occur in cardiac and extracardiac locations . CardiacEven though the head and neck region is the most common area of extracardiac RM –3, this An 85-year-old woman was referred to our institution with a 10-day history of breathing difficulties, dysphagia for solids and liquids, and dysphonia. The patient was a former smoker (20 cigarettes/day for 45 years until the age of 60), while no alcohol intake was reported. The patient's medical history was positive for chronic obstructive pulmonary disease, hypertension, anaemia, osteoporosis, goitre, and chronic renal failure.General otolaryngological examination was normal. Endoscopy revealed a submucosal lesion in the right arytenoid, covered with normal mucosa, extending towards the right pyriform sinus. The right vocal cord was fixed while the left was hypomobile, thus reducing breathing space. There was no clinical evidence of cervical lymphadenopathy. A computed tomography (CT) scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins, originating from the right pyriform sinus with partial contralateral extension. The mass measured 18 × 22 × 12 mm and extended caudally to the right arytenoid and the upper margin of the cricoid cartilage, causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. Other laryngeal structures were preserved .The patient underwent complete endoscopic removal of the lesion and temporary tracheostomy for respiratory distress; tracheostomy was closed 32 days later. Neither postoperative complications nor signs of recurrence during an 18-month follow-up were observed.Histologic examination of the neoplasm revealed a well-circumscribed, not encapsulated, proliferation of large closely packed neoplastic round to polygonal cells in a scant stroma Figures . The celAbout 90% of extracardiac RMs occur in the head and neck region –3,17. EvIn the larynx, adult-type RM usually occurs as a solitary lesion in adulthood, is more frequent in men than in women, and more frequently involves the glottic and supraglottic regions –38. In oDefinitive diagnosis is possible only after histologic examination and immunohistochemical analysis. It is recommended to obtain the neoplastic tissue by excisional rather than incisional biopsy because in the latter the small amount of tumor tissue may contribute to misdiagnosis and inappropriate treatment , 37. IndThe treatment of adult-type RM is surgical and can be done either by endoscopic or external approach, but always preserving the adjacent structures such as the vocal folds and the swallowing apparatus. Complete surgical excision is curative. Adult-type RMs may recur , 13, 18,In conclusion, the adult-type RM of the larynx is a very rare benign tumor that must be distinguished from other submucosal laryngeal neoplasms. The present case fulfills the clinicopathologic criteria for the diagnosis of adult-type RM of the larynx. Since clinical and imaging features are not specific for adult-type RM, histology and immunohistochemistry are mandatory for a definitive diagnosis. Radical endoscopic excision is the recommended procedure to obtain tissue for histologic evaluation and for patient treatment.


A 77-year-old male with a history of metastatic scalp angiosarcoma presented with intractable gastrointestinal bleeding from a jejunal mass detected on capsule endoscopy. He underwent laparoscopic-assisted resection of the mass. Intraoperatively, an isolated small bowel mass with bulky lymphadenopathy was seen and resected en bloc. Pathology showed a 6.8cm high-grade metastatic angiosarcoma with nodal involvement and negative margins. Angiosarcoma is a sarcoma with a grim prognosis. The incidence is 2% of all soft tissue sarcomas; cutaneous lesions comprise 27% of manifestations and usually appear on head and neck. Risk factors include lymphedema, neurofibromatosis, vinyl chloride, arsenic, and anabolic steroids. Overall 5-year survival is 30-35% and is higher in patients younger than 60, those without metastasis, tumors less than 5 cm, and favorable histology. Angiosarcoma metastasis to small bowel is rare but nodal involvement is even more unusual, reported only three times in the literature. This case is the first with nodal involvement to present at a resectable stage. To diagnose disease when still at a resectable stage, a high index of suspicion must be maintained with any gastrointestinal symptoms in a patient with a history of angiosarcoma. Laparoscopic-assisted resection is safe for the resection of small bowel angiosarcoma.  A 77-year-old male presented to our clinic with gastrointestinal bleeding. He had recently been admitted for melena and iron deficiency anemia, requiring multiple transfusions. Workup was benign, with EGD and colonoscopy significant only for non-bleeding internal hemorrhoids. His medical history was significant for angiosarcoma of the scalp which had been excised and radiated; he had had metastases to his neck and lungs resected and had undergone radical neck dissection and radiation. He had refused systemic chemotherapy. Laboratory results on presentation were significant for hemoglobin of 7.3 g/dL  and were otherwise unremarkable. Abdominal exam was benign with no masses or tenderness. His endoscopy showed no culprit lesions. He was referred for video capsule study, which revealed a large mass in the proximal small bowel with small amount of blood obscuring lesion . With otAfter entering abdomen, the small bowel was run from the ligament of Treitz until the lesion was identified. The mass was found in mid-jejunum, protruding from small bowel lumen and adherent to the small bowel mesentery . Involved jejunum was exteriorized for resection. Upon inspection, gross lymphadenopathy was noted to the root of the mesentery; this was divided without vascular compromise. The small bowel was resected for 10cm gross margins. The intestine was examined to rule out satellite lesions and the operation was concluded. Postoperative course was notable only for a prolonged ileus; no further bleeding episodes were noted during the hospital stay.  Pathology returned noting 6.8cm mass positive for high-grade metastatic angiosarcoma. Margins were microscopically negative. Three of four resected nodes were positive for angiosarcoma.  The patient has not experienced any bleeding episodes since resection. Angiosarcoma is a vascular sarcoma characterized by endothelial origin, local aggression, distant metastases, and a grim prognosis . The ove Gastrointestinal angiosarcoma, primary or metastatic, is exceedingly rare. In a literature review by Qingqiang et al.,  a total Rates of metastasis to the small bowel are even more unusual; our literature review  revealed only six cases in the English literature -6. Five  The nodal metastasis we saw intraoperatively is atypical of angiosarcoma. Our review revealed only three cases with gastrointestinal nodal metastasis. All cases presented with an advanced stage of disease, including: multiple unresectable lesions , chylousThere is insufficient information, either in cutaneous or visceral angiosarcoma, to support the role of surgical nodal debulking; however, the presence of nodal metastasis certainly warrants a referral to medical oncology and the serious consideration of systemic treatment if the patient survives the initial operation. Although rare, the presence of these lesions in the literature and their late detection and poor survival suggest that a high index of suspicion must be maintained in patients with a primary angiosarcoma who present with gastrointestinal symptoms, especially those of bleeding or pain.The presence of GI metastasis in patients with angiosarcoma is rare, but has been defined in the literature. Any new presentation of GI bleeding in a patient with known angiosarcoma or with suspicious skin lesions should be worked up aggressively and early, as most GI metastases present at an advanced stage. We suggest a full evaluation, including EGD and colonoscopy early in the clinical course with VCE included regardless of findings, given the incidence of these tumors in the small intestine and the relatively high rates of multifocal disease. Any patient who presents with isolated angiosarcoma of the intestine should also undergo a full skin exam at the time of presentation to rule out a cutaneous source. With vigilance, we can detect these lesions earlier in their course, at a time when they are more amenable to resection and ultimately, to survival.


Desmoid-type fibromatoses are rare benign and fibrous tumors that account for approximately 0.03% of total neoplasms. Within this category of neoplasms, pancreatic desmoid-type fibromatosis is an extremely rare subgroup, accounting for approximately 5% of desmoid-type fibromatoses. Although the etiology is unknown, some risk factors include trauma, surgery, family history of desmoid tumor, pregnancy, use of contraceptives, genetic mutation, and familial adenomatous polyposis or Gardner syndrome. Desmoid-type tumors are primarily diagnosed by pathological and immunohistochemical studies. The treatment of choice is surgical resection of the tumor. Systemic chemotherapy and radiotherapy are optional treatment approaches for patients with high risk for surgery.The following is a report of an unusual case of a pancreatic desmoid-type fibromatosis in which a very rare variation in the immunohistochemistry findings was demonstrated: slides were immunopositive for S100 protein and not immunonegative. Most desmoid tumors are immunonegative for S100 protein with just a few cases being positive for this protein.We describe the case of a 15-year-old boy, a Chinese national, who was diagnosed as having a pancreatic desmoid-type fibromatosis. He was a healthy individual who was incidentally diagnosed with an abdominal mass. His chief complaints were mild generalized abdominal pain for 1 week, nausea, vomiting, and a low-grade fever. An enhanced computed tomography scan of his abdomen showed a large cystic mass in the anterior surface of the body of his pancreas. He underwent a radical resection of the pancreatic mass, partial pancreatectomy, splenectomy, segmental resection of transverse colon and distal jejunum, and subsequently a one-stage jejunojejunostomy and colonic anastomosis. Postoperatively, he was diagnosed as having a desmoid-type fibromatosis of the pancreas by pathological and immunohistochemical studies.The diagnosis of desmoid-type fibromatosis is usually incidental and challenging. Pathological and immunohistochemical testing are essential for confirming diagnosis where demonstration of β-catenin nuclear staining is probably the single most important characteristic. Other markers, such as vimentin, are usually positive, while S100 protein is usually negative. Our case however, confirms that there are rare cases that can be S100 positive. This is the first diagnosed case of pancreatic desmoid-type fibromatosis with S100 protein positivity. The risk factors are history of trauma, irradiation, positive family history for DT, surgery, pregnancy, use of contraceptives, genetic mutation, and FAP or Gardner syndrome [APC) gene or β-catenin (CTNNB1) gene. The protein APC regulates cellular β-catenin which is involved in wound healing and fibroprofileration. APC and β-catenin are both members of the Wnt signaling pathway, and both can be aberrantly expressed in DTs irrespective of familial or sporadic origin. The consequence of this is a translocation of cytoplasmic β-catenin into the nucleus that activates the expression of T-cell factor and this upregulates the expression of downstream genes such as cytochrome c oxidase 2 (COX2), platelet-derived growth factor (PDGF), matrix metallopeptidase (MMP), hyaluronan-mediated motility receptor (RHAMM), and retinoblastoma 1 (RB1) [Desmoid-type fibromatosis or aggressive fibromatosis is also known as a desmoid tumor (DT). It is a rare group of benign neoplasms that result from the proliferation of well-differentiated mesenchymal fibroblasts. These tumors are known to be locally invasive with no evidence of distant metastatic potential. The advanced stages of the disease, however, can still be life-threatening due to local tissue invasion , 2. Althsyndrome –4. The g 1 (RB1) –7.The following is an unusual case of an isolated and sporadic desmoid-type fibromatosis located in the body and tail of the pancreas. In this case we discovered a rare immunohistochemistry presentation of a pancreatic DT; therefore, our objective is to highlight the current pathological and immunohistochemical diagnostic criteria of this entity while demonstrating this rare finding. This is the first case of a pancreatic DT, diagnosed on 12 January 2017, at the First Affiliated Hospital of Chongqing Medical University, Chongqing, People’s Republic of China. We also present an updated literature review on desmoid-type fibromatosis of the pancreas.Our patient is a 15-year-old boy, a Chinese national, with no significant medical history. His personal and family history were both negative for FAP and Gardner syndrome. He had a normal infancy and early childhood and was fully vaccinated. Significantly, he has a family history of pulmonary tuberculosis, hemophilia, and he is an offspring of parents who are first-generation cousins. He was referred to our institution after visiting his local hospital for a routine physical examination where he was diagnosed as having an upper abdominal mass. A computed tomography (CT) scan at his local hospital revealed an abdominal cystic mass adjacent to his pancreas measuring 11.5 × 9.0 cm.He was initially seen at our Surgical Out-patient Department. His chief complaints were a 1-week history of mild abdominal pain (mainly in the epigastric region), nausea, vomiting, and a low-grade fever. On physical examination his abdomen was slightly distended, with dullness on percussion, and generalized tenderness on palpation. A large mass measuring approximately 7 × 10 cm was palpable in his left upper abdomen extending from the epigastric to left hypochondriac region; it was of a hard consistency with well-defined surface and borders and poor mobility. In our hospital, an enhanced CT scan of his abdomen was done and this revealed a large cystic mass in the anterior aspect of the body of his pancreas measuring 9.3 × 8.5 cm with characteristic of pancreatic cancer (of pancreatic origin). Other radiological differentials were a solid pseudopapillary tumor or cystadenoma. A large splenomegaly with compression of splenic vein was also noted. He was subsequently admitted for further investigation and management  was also done and this confirmed a huge cystic mass measuring approximately 8.1 × 9.3 × 11cm closely adherent to the body and tail of his pancreas. Our patient was scheduled for an elective exploratory laparotomy with a preoperative diagnosis of pancreatic cancer of unknown origin. Intraoperatively, a decision was made to perform a radical resection of the pancreatic mass. Due to the large size of the tumor and extensive local adhesions encountered, a partial pancreatectomy, total splenectomy, segmental resection of transverse colon and distal jejunum, and subsequently a one-stage jejunojejunostomy and colonic anastomosis were also performed.Preoperative laboratory investigations  were all unremarkable. Intraoperative findings were a grossly enlarged spleen measuring approximately 13 × 9 cm and a mass approximately 20 × 14 cm at the body and tail of his pancreas with adhesion to the posterior wall of his stomach, transverse colon, and distal portion of jejunum. There were also multiple enlarged lymph nodes. The duration of the surgery was 5 hours and 5 minutes and it was completed without any incidents. There was a total blood loss of 1700 ml; therefore, 700 ml of packed red blood cells and 400 ml of plasma were transfused intraoperatively. Postoperatively, our patient progressed satisfactorily and was discharged from hospital in a clinically improved and stable condition. He was seen in follow-up clinic 2-months post-operation with a repeat abdominal CT scan which revealed no evidence of recurrence of the abdominal mass. He is currently not being considered for adjuvant chemotherapy or radiotherapy but will continue to attend out-patient clinics for close monitoring and care.Pathology and immunohistochemistry studies of his pancreas, jejunum, and spleen showed transitional hyperplasia and spindle cells atypia with invasion of his pancreas and muscular layer of his intestinal wall. There was evidence of reactive hyperplasia of lymph nodes and mild congestion of his spleen.Individual immunohistochemistry results were β-catenin (+), vimentin (+), S100 (+), Ki67 < 1% (+), DOG1 (−), CD117 (−), smooth muscle actin (SMA) (−), desmin (−), ALK (−), CD34 (−), calretinin (−), WT1 (−), CK5/6 (−), EMA (−), D2-40 (−), and MC (−). The combined pathology and immunohistochemistry results were consistent with aggressive fibromatosis or desmoid-type fibromatosis of the pancreas.Desmoid-type fibromatosis in general, has an annual incidence of approximately 2 to 5 cases per 1 million in USA and European populations and accounts for approximately 0.03% of all tumors and 3% of fibrous tumors –10. AlthThe above-mentioned case of pancreatic DT is to the best of our knowledge the 27th case documented in the English literature since 1980. This is the fourth documented case in English in the People’s Republic of China , 13, 14.The clinical presentation of DTs is usually asymptomatic or they can present with nonspecific signs and symptoms. These symptoms are usually related to the location of growth of the tumor and invasion of adjacent structures. Intra-abdominal DT can present nonspecific abdominal discomfort or pain and weight loss. If located in the gastrointestinal tract or urinary tract, symptoms of compression or obstruction may be the predominant clinical manifestations. DT of pancreatic origin is similar to pancreatic cancer and therefore usually silent, but occasionally DT of pancreatic origin and pancreatic cancer can cause epigastric pain that seldom radiates to the back. A painless jaundice can be seen if DT is located in the head of the pancreas. Weight loss is, however, often seen in pancreatic DT due to chronic aversion to food , 5. In oPossible complications are also determined by the location of the tumor. For instance, a tumor located in the extremities can result in neuropathic pain and debilitating sequelae. Intra-abdominal DTs can result in intestinal obstruction and fistulation, ischemia, hemorrhage, and perforation or ureteric obstruction or recurrence .A clinical diagnosis of pancreatic DT is almost impossible due to its nonspecific clinical presentation. It is always essential to commence with a thorough clinical history and physical examination. The clinical history must specify if there is personal and/or family history of genetic diseases. CT scans and MRI are valuable diagnostic tools. Cross-sectional imaging of the affected area with CT or MRI is needed to define the relationship of the tumor to adjacent structures in order to assess resectability. The definitive diagnosis of DT however, is confirmed by pathological and immunohistochemical findings , 4, 5.Although not pathognomonic to DTs, spindle cells are common histopathological findings. Immunohistochemistry findings for intra-abdominal DT are: vimentin (+) and β-catenin (+) and immunonegative for SMA such as S100, CD117, and CD34 . Our patIn general, desmoid-type fibromatoses are usually immunonegative for S100 protein, although a few rare cells can present S100 immunopositive slides . In the Nuclear staining for β-catenin is a consistent finding in more than 80% of cases. Although it greatly aids in the diagnosis, it is not specific for this type of tumor since it is also seen in a variety of other tumors. The diagnosis can further be confirmed by screening for mutations (mainly in exon 3) of the β-catenin gene, which are found in 85% of sporadic cases , 13.et al. have reported a favorable outcome with 100% overall survival and > 80% disease-free survival [The prognosis is not well known but Jia survival . There isurvival , 16.The current first-line treatment for DT is complete resection  with free margins. Better outcomes are achieved by performance of a resection with wide, free margins for the prevention of tumor recurrence considering the locally invasive behavior of this tumor. Experts are now recommending a wait-and-see policy (observation) especially in cases of static tumor, given the morbidity associated with surgical resection and the relatively high recurrence rate.Adjuvant therapies such as systemic chemotherapy , radiotherapy, and molecular target therapy (tamoxifen) have been proposed particularly for patients with high risk for surgery. To date however, there are no established clinical treatment guidelines for DTs. A few successes have been reported with the use of NSAIDs and celecoxib as COX2 inhibitors, but their mechanism of action is not well documented and verified , 16, 19.The diagnosis of desmoid-type fibromatosis can be challenging because of two main factors: (1) the clinical presentation is nonspecific and thus results frequently in incidental diagnosis; and (2) there is no specific clinical investigation for this tumor. Pathological and immunohistochemical testing are important for confirmatory diagnosis, where β-catenin nuclear staining must be demonstrated.Another important characteristic of desmoid-type fibromatosis is their expression or not of S100 protein. Most DTs are negative for S100 protein but a small percentage of cases can demonstrate S100 protein immunopositivity. This is an important diagnostic criterion that must be taken into consideration when confronting this type of tumor.DT can present as either a solid, cystic, or solid-cystic mass and the treatment of choice remains radical surgical resection of the tumor. Adjuvant chemotherapy and/or radiotherapy are recommended for patients with high risk for surgery. In static tumors a watch and wait policy may be the best option.


Metastatic gastric cancer in the pituitary (MGCP) is rare. Few are known on the clinical and radiological characteristics of MGCP. To date, the coexistence of metastatic pituitary tumors and intracranial aneurysms has not been reported in literatures.We present a case of MGCP with internal carotid aneurysm in a 57-year-old woman, who presented with oculomotor paralysis, postorbital pain, and hypopituitarism as onset symptoms. The patient had a history of the surgical removal of gastric cancer. Magnetic resonance imaging and single-photon emission computed tomography revealed a recurrent sellar mass with intracranial and multiple bone metastases. The patient underwent subtotal removal of the tumor, followed by conformal radiotherapy and chemotherapy. Ten months after surgery, the patient died due to deterioration of her overall condition.We also reviewed and analyzed the clinical data, imaging features, and treatment methods of additional 4 cases with MGCP, which were reported in literatures. This study provides important clinical information for the diagnosis and treatment of MGCP. The pituitary gland may be the only metastatic site of a tumor, and MP may cause the first clinical presentation of tumors with multiple metastases. Breast cancer and lung cancer are the most common primary tumors metastatic to the pituitary gland in women and men, respectively.6 MP from gastric cancer is rarely reported in literatures.8 Gastric cancer commonly metastasizes via the lymph node, peritoneum, blood, or bone marrow, and common metastatic sites are the liver, peritoneum, lung, and bone. Intracranial aneurysm is found in only 0.3% of brain tumor patients,9 and a coexisting aneurysm can trigger or exacerbate the disease course of tumors.11 Tumors metastatic to the pituitary gland and parasellar aneurysms are usually misdiagnosed as pituitary adenoma. To date, the coexistence of MP and intracranial aneurysms has not been reported in literatures. Therefore, a few information is available regarding the clinical and radiological characteristics of metastatic gastric cancer in the pituitary (MGCP) coexisting with internal carotid aneurysm (ICA).Metastasis in the pituitary (MP) is a rare cancer-associated complication, accounting for <1% of all sellar or parasellar tumors and ∼5.1% of all metastatic brain tumors.In this study, we present the first reported case of MGCP with a left ICA in the siphon segment in a 57-year-old woman, presenting with oculomotor paralysis, postorbital pain, and hypopituitarism. In addition, we reviewed 4 other known cases with MGCP and summarize the clinical manifestations and imaging characteristics of MGCP. Our study provides important clinical information for diagnosis and management of MGCP.In September 2014, a 57-year-old woman visited our hospital presenting with diplopia, paroxysmal headache, and postorbital pain without obvious causes. The patient had right severe ptosis 1 week later. Four years earlier, the patient underwent subtotal gastrectomy for gastric antrum carcinoma followed by 1 cycle of FOLFOX  and 4 cycles of XELOX  chemotherapy. She had no family history of gastric cancer.On physical examination, the patient had right oculomotor paralysis with right eyelid drooping and was unable to perform the inward, upward, and downward movement of the right eyeball. She had diplopia without nystagmus. The right pupil was dilated with blunt pupillary reflex. Visual field and visual acuity of both eyes were normal. Computer tomography (CT) scan of the orbital, chest, and abdomen were unremarkable. Brain CT revealed a destroyed occipital base and dorsum sellae surrounded by soft-tissue density shadows. Brain magnetic resonance imaging (MRI) revealed a round mass of 25 mm in diameter in the enlarged sellae Figure A and B. The patient underwent transnasal transsphenoidal surgery to remove the tumor. The tumor was red and solid with rich blood supply, and infiltrated into the clivus with close adhesion to the bone. The upper left of the tumor surrounded the artery aneurysm. The tumor was subtotally resected in pieces. The aneurysm remained unprocessed due to its small size and wide neck. Postoperative pathological examination revealed that the tumor was adenocarcinoma resembling primary gastric carcinoma Figure A and F. One month after the surgery, the patient complained of bone pain, headache, dizziness, fatigue, and nausea with vomiting. MRI revealed a giant round sellar mass of 28 mm in diameter with an unclear boundary. The tumor was isointense or hyperintense on T1-WI, and isointense or hypointense on T2-WI with heterogeneous enhancement. SPECT revealed that multiple bone metastases occurred in the skull, skull base, 3rd thoracic vertebra, and the right sacroiliac joint  is more sensitive with sharper images for differential diagnosis than SPECT, it is not routinely used due to its high cost.28 SPECT can detect multiple metastases early29 and is recommended for differential diagnosis. Although neuroimaging findings are helpful for differential diagnosis, these findings cannot reliably distinguish MP from adenoma. As clinical syndromes are distinctive between MP and adenoma, clinical manifestations are important for differential diagnosis.30 Therefore, these following features favor the diagnosis of MP: positive history of cancer, fast growth of the tumor, presence of diplopia or diabetes insipidus, rapid development of anterior pituitary hormone deficiencies, high level of cancer biomarkers, positive findings in SPECT/PET examinations, and atypical appearance of the tumor. Definitely, pathological diagnosis is conclusive.Neuroimaging is helpful for differential diagnosis between MP and pituitary lesions such as pituitary macroadenoma, pituitary apoplexy, cystic lesions, and chronic inflammation. MRI is not specific and usually shows isointense or hyperintense signals on T1-WI and isointense or hypointense signals on T2-WI.In summary, we describe a rare case, in which a patient had a sellar mass close to the left internal carotid artery aneurysm. MP should be considered in patients with a history of gastric cancer and had rapidly aggravated diplopia, postorbital pain, headache, and hypopituitarism. CEA, CA199, and CA724 serum levels may be useful markers for monitoring disease progression. Combined treatments including surgical treatment, radiotherapy, chemotherapy, and hormone replacement therapy may be important for improving the survival of patients with MP.


Perforation of the colon occurring during or shortly following colonoscopy or barium enema is a rare complication of collagenous colitis (CC). "Spontaneous" perforation in CC, in which no instigating factor is identified, is even less common, with only five cases reported to date. We report herein an additional case of spontaneous perforation in previously undiagnosed CC and review the clinical and pathological features of previously reported cases.An 80 year old woman presented to the emergency department with abdominal pain preceded by approximately one month of frequent non-bloody diarrhea. Abdominal CT showed parietal thickening of the colon at the splenic flexure with pneumatosis and signs of perforation. Segmental resection was performed. Pathologic examination showed the microscopic findings typical of CC complicated by several deep ulcers and perforation. One day following discharge from hospital abdominal pain and frequent non-bloody diarrhea recurred. The patient was managed conservatively and treated with oral budesonide with resulting resolution of symptoms.Spontaneous perforation is a rare and serious complication of CC. All patients to date have been female. In contrast to procedure-related perforation, which favors the right colon, spontaneous perforation in CC has in all cases involved the left colon. Knowledge of spontaneous perforation as a potential complication of previously undiagnosed CC may be helpful in the evaluation and management of patients presenting with colonic perforation, especially those with risk factors for CC. The classical clinical and pathologic features of collagenous colitis (CC) are well known , 2. LessAn 80 year old female came to the emergency department of our hospital complaining of abdominal pain of recent onset. She related a history of frequent episodes of non-bloody diarrhea of approximately one month's duration, unresponsive to loperamide. Her past medical history included surgical correction of rectal prolapse six months previously, implantation of a pacemaker, anticoagulation, osteoporosis, and hypothyroidism treated with levothyroxine.9/L . The patient was admitted to hospital.Her temperature was normal. Physical examination revealed lower abdominal tenderness but was otherwise unremarkable. The white blood cell count was 8.1 ×10Due to chronic renal failure unenhanced abdominal CT scanning was performed Fig. . This sh9/L. In light of this and the CT scan findings, an exploratory laparotomy was performed which revealed a small amount of ascites. A 16 cm segment of thickened bowel from the splenic flexure was resected.The following day the white blood cell count was 20.4 ×10The postoperative course was uneventful. The patient was discharged on the eighth post-operative day. However, the next day she was readmitted to hospital with abdominal pain accompanied by at least ten episodes of non-bloody diarrhea occurring that day. An abdominal CT scan with rectal contrast showed no evidence of anastomotic leak or other abnormality. Stool culture and testing for Clostridium difficile toxin were negative. The patient improved with conservative management. Given the diagnosis of collagenous colitis (see pathologic findings below), treatment with oral budesonide was started and she was discharged on the eleventh day of her hospital stay. One month later she reported feeling well and having one to two stools of normal consistency per day.Macroscopic examination Fig.  showed nMicroscopic examination Fig.  showed tReview of the prior rectal prolapse resection specimen confirmed the microscopic findings characteristic of prolapse. Histologic features of collagenous colitis were absent.Perforation of the colon in CC may occur in two settings: 1) procedure-related (during or shortly following colonoscopy or barium enema), and 2) "spontaneous", that is, unrelated to any intervention.Although the first reported case of perforation was of the spontaneous type , subsequSpontaneous perforation in CC has been described in five previous patients –8 Table. In all Regarding differential diagnosis, as the clinical findings of ischemic colitis (frequent) and CC with perforation (very rare) would seem to significantly overlap, diagnosis of CC will depend upon high clinical suspicion and histologic analysis of a biopsy or surgical resection specimen. Patients in both CC and IC tend to be middle-aged to elderly, with a female predominance in CC. IC classically presents with abdominal pain and blood in the stool, whereas CC is typified by frequent episodes of non-bloody diarrhea.Risk factors for IC include a number of cardiovascular pathologies such as peripheral vascular disease, diabetes, dyslipidemia, heart failure and abdominal aortic aneurysm repair . Risk faColonoscopy and histologic examination both provide findings that will usually allow separation of IC from CC. The colonoscopic appearance of the mucosa in CC is usually normal, whereas IC features a range of abnormalities including erosions, longitudinal ulcerations, petechial hemorrhages, and necrosis, usually involving watershed areas of the splenic flexure and sigmoid.Histologically, CC and IC are distinct entities. CC is characterized by thickening of the subepithelial collagen band, often associated with separation of the epithelium from the lamina propria, and lymphocytic infiltrates in the mucosa. IC at biopsy shows atrophic glandular crypts, hyalinizing fibrosis and often hemorrhage in the lamina propria with acute inflammation, micro-thrombi in the superficial capillaries, and, when severe, necrosis. Inflammatory pseudomembranes similar to those found in Clostridium difficile-associated pseudomembranous colitis may be present.In the present case, the histologic findings typical of collagenous colitis were clearly present. However, as distinction from ischemic colitis is critical, we further examined the histologic sections with a view to eliminating concomitant ischemic colitis contributing to the clinico-pathological picture. A careful examination of the non-ulcerated mucosa showed no evidence of atrophic glandular crypts, hyalinizing fibrosis or hemorrhage in the lamina propria, or intra-capillary micro-thrombi. The absence of these changes allowed for confident exclusion of associated ischemic colitis. In contrast, photomicrographs in five of the six previously reported cases show the characteristic features of CC, but the authors did not explicitly address whether they considered ischemic colitis as a complicating factor . We believe it is worth keeping in mind that CC and IC should not be considered as mutually exclusive diagnoses.Whereas risk factors, as described above, have been identified for iatrogenic perforation in CC, an explanation for spontaneous perforation is not so evident. One factor may be the degree of thickening of the collagen band, which in our case was focally severe, resulting in very tenuous integrity of the overlying epithelium. In this situation normal peristaltic activity or the repeated bowel movements characteristic of CC may have been sufficient to provide enough stress on the mucosa to lead to tearing and ulceration.We conclude that, although decidedly rare, knowledge of spontaneous perforation as a possible complication of CC may be helpful in the evaluation and management of patients presenting with colonic perforation, especially those with risk factors for CC.


Extrauterine leiomyomas are encountered occasionally, which can pose a diagnostic dilemma and challenge to the gynaecologist. We report a rare case of urethral leiomyoma. A 31-year-old woman with history of primary subfertility presented with mass at her urethral meatus and lower urinary tract symptoms. She underwent examination under anaesthesia and excision of the urethral mass. Histopathological examination confirmed leiomyoma. Diagnosis and management of this common growth situated at a rare location were reviewed and discussed.  Leiomyomas are benign tumours of smooth muscle, which commonly occurred at the genitourinary tract like uterus. Extrauterine leiomyoma are rare especially in the deep soft tissue like female urethra. Thus, presence of a urethra mass poses a significant diagnostic challenge as its differential diagnosis includes an extensive list of both benign and malignant pathologies of gynaecological or urologic origin. We describe an unusual case of urethral leiomyoma that was mistaken as urethral prolapse.A 31-year-old nulliparous with primary subfertility for 4 years presented with one-week history of painful swelling at the urethral meatus associated with lower urinary tract symptoms. She has dysuria and incomplete voiding but no haematuria. There was no significant medical or surgical history. Physical examination revealed a firm mass measuring 2.0 × 1.5 × 1.5 cm at the ventral wall of urethral meatus from 10 to 2 o'clock . FollowiHistopathological examination (HPE) revealed a firm well-circumscribed mass measuring 20 × 15 × 14 mm. Microscopically, section showed a well-circumscribed tumour lined by stratified squamous epithelium and focal area of urothelial lining. The tumour was comprised of interlacing bundles of smooth muscle fibres displaying uniform spindle-shaped nuclei. There was no mitosis or nuclei atypia seen. Immunohistochemistry study showed that the cells were positive for SMA and desmin. The features confirmed diagnosis of urethral leiomyoma .Postoperatively, she recovered well. The indwelling catheter was kept for 10 days and she was able to void without difficulty upon catheter removal. There was no recurrence observed during follow-up.Urethral masses are rare entity. The differential diagnoses include urethral prolapse, urethral caruncle, urethral diverticulum, Skene's duct cyst, Gartner's duct cyst, urethral carcinoma, and ectopic ureteroceles . UrethraUrethral leiomyomas have a female preponderance. The tumour often found in women at reproductive age in their third and forth decades of life . MoreoveA thorough assessment includes detailed history and physical examination is of utmost importance in the evaluation of a urethral mass. In selected cases, radiological imaging such as ultrasound, pelvic magnetic resonance imaging (MRI), or computed tomography (CT) scan might be helpful. Transperineal ultrasound showed well-defined iso- to hypoechoic homogenous mass with whorled appearance and significant internal vascularity on colour flow Doppler  whereas Due to the rarity of this condition, its management and treatment have not been established yet. Though local excision is usually recommended, hormonal treatment with gonadotrophin releasing hormone analogue had been described . CompletUrethral leiomyoma is rare and poses diagnostic difficulty to a certain degree. Complete surgical excision was recommended for HPE confirmation.


In this study, we present a case of unicystic ameloblastoma (UA) treated by marsupialization followed by surgical enucleation as a conservative approach. UA is a rare, benign, less aggressive, and less invasive variant of ameloblastoma that is observed quite often in younger patients. Radical approaches have effects on the physical and psychological development of a growing young patient; therefore, conservative approaches are widely used for UA management in children.This report described a case of an 11-year-old girl with UA of the mandibular molar–ramus area, which also involved impaction of the second and third molars. The lesion was marsupialized, and 31 months after marsupialization, surgical enucleation was performed with extraction of the impacted third molar. The second molar, which was preserved, spontaneously and completely erupted. No recurrence was observed during a 49-month follow-up.Conservative treatments for UA in young patients have more advantages. Our results provide evidence that marsupialization is effective in regressing the lesion size to ease complete removal, preserving mandibular growth, maintaining involved second molar, and promoting the eruption of the tooth. Ameloblastoma is a rare, benign odontogenic tumor that manifests locally as an aggressive neoplasm of the jaw, developing from the epithelium involved in the formation of teeth: the enamel organ, epithelial cell rests of Malassez, reduced enamel epithelium, and odontogenic cyst lining –3. It ocUA presents some distinguishing and characteristic features because it is observed in a rather younger age group , 8, 9 thVarious treatment modalities such as segmental or marginal resectioning for UA have been used, similar to those normally used for conventional ameloblastoma, and more conservative treatments have also been frequently reported , 13. TheThis study presents a case of mandibular UA in a young patient who was successfully treated by conservative management without the removal of an involved second molar.In January 2013, an 11-year-old female patient with a chief report of swelling in the left mandibular molar region was referred to our medical center. Two weeks before the first visit, the patient was prescribed antibiotics by a local family medical clinic because of swelling in the left side of the jaw and tenderness of the mandibular angle. For these reasons, she was referred from a local dental clinic and consulted our center. The patient had no systemic health conditions. Extraoral examination revealed swelling in the left mandibular angle area, and intraoral examination revealed mucosal swelling that extended from the lower first molar to the retromolar region, mixed dentition, and an unerupted left mandibular second molar.A panoramic radiograph revealed a well-circumscribed, unilocular radiolucency in the region of the left mandibular molars, extending from the distal root of the first molar area to the left ascending ramus, with an unerupted second molar, and the dental follicle of the third molar Fig. . The impThe treatment of UA has been controversial and can be radical or conservative , 17–19. One of the factors that can determine the effectiveness of a treatment is the recurrence rate, which varies with the different types of ameloblastoma . UA is lIt is clear that if an adequate bone margin is removed, the chances of recurrence are expected to be low. However, a balanced judgment is required when selecting this treatment option so that maximum success does not lead to overtreatment . DespiteUA’s biologic behavior is considered to be less invasive, and it responds more favorably to conservative treatment than a multicystic ameloblastoma . TherefoThe aim of marsupialization is to reduce the size of the tumor so that a less extensive surgery is required , 24. TheDuring surgery, the involved teeth are usually extracted together with the tumors to not only completely remove the tumors, but also prevent their recurrence. However, tooth loss can result in functional and esthetic disturbances. Furthermore, prosthodontic treatments are generally difficult in young adolescents because of their dental and skeletal growth. If impacted or involved teeth within ameloblastomas can be preserved and if functional occlusion can be obtained, the patients’ quality of life will significantly improve, particularly in young patients .Marsupialization therapy is usually performed for preserving the involved teeth and promoting eruption of the tooth within the lesion , 26, 27.In our case, the eruption of the mandibular second molar on the affected side was disturbed by the tumor. The impacted second molar naturally erupted after reducing the tumor volume from marsupialization. We eventually decided to keep the second molar, although the third molar had to be extracted.Conservative treatment appears to be preferable in the younger age groups because it offers a better quality of life; however, the recurrence rate remains high . TherefoIn conclusion, conservative treatment for UA in young patients has more advantages. Our young patient who was treated by conservative management did not have any complications, and the condition was well maintained, with no signs of recurrence. Spontaneous eruption occurred after marsupialization without the extraction of the involved second molar. This tooth functions well without the need for a root canal.


Neurilemmoma (NL), also termed schwannoma, presents as a well-circumscribed and encapsulated mass in the human body and is almost always solitary. CT scan of a patient with NL shows a round, ovoid, or lobulated well-demarcated solid mass of soft tissue density. Primary intrathoracic neurogenic tumors location varies. However, the development of such tumors is by far more common in the costovertebral angle of the posterior mediastinum. Here, we report a rare case of a 43-year-old patient, never smoker and previously healthy, who presented with a mass adjacent to the right pulmonary hilum. This was an incidental finding on a chest X-ray after annual checkup at his workplace. The diagnosis was primary intrapulmonary NL. Primary intrapulmonary NL is an extremely rare tumor. However, based on the above, chest CT findings of a well-defined solid mass in an asymptomatic patient should raise the suspicion of NL, irrespective of the tumor localization. Neurilemmoma (NL), also termed schwannoma, presents as a well-circumscribed and encapsulated mass in the human body and is almost always solitary. Primary nerve cell tumors of the lung are extremely rare, and they accounted for 0.2% of all pulmonary neoplasms . We here2 21%, lung auscultation without additional sounds). There were no palpable lymph nodes. Complete blood count and metabolic panel, urinalysis, and electrocardiography were normal. The tuberculin skin test was negative. Chest CT showed a small, well-defined, round, and solid lesion (32 mm × 27 mm), in the superior segment of right lower lobe  of the lesion, which exceeded that of vascular structure of the mediastinum (SUVmax 1.7) , PANK(−), CD68(−), SMA(−), Desmine(−), CD31(−)  of NL ranged from 1.3 to 6 (mean 3.2), while malignant neurogenic tumors (MPNST) had an uptake between 4.5 and 9.7 (mean 7.0)  , 9. SUV ean 7.0) , 9.Most patients with intrapulmonary NL present with no symptoms and mass is identified incidentally on the chest X-ray, as in our case. However, a considerable percentage of patients presents with symptoms mimicking asthma, bronchitis, or poststenotic pneumonia due to airway obstruction secondary to tumor .Histologically, NL is typically sharply surrounded by a fibrous capsule and is associated with two microscopic patterns—the Antoni A (cellular) and Antoni B (less cellular) , 4, 7. TWith regards to treatment of primary intrapulmonary NL, successful surgical treatment using limited airway/sleeve resection, lobectomy, or rarely pneumonectomy for large or proximal lesions has been reported. The prognosis of intrapulmonary NL is very good, and to this end there are no reports of recurrence , 4, 5, 7Primary intrapulmonary NL is an extremely rare tumor; yet, chest CT findings of a well-defined solid mass in an asymptomatic patient should raise the suspicion of NL. There is no pathognomonic radiological feature, and PET scan usually shows low uptake values; yet, variable uptake values could be shown. Due to the low malignant potential of a primary intrapulmonary NL, tumor enucleation or partial lobectomy (if possible) is sufficient unless the tumor is more proximal, which may require lobectomy. Histologic examination with positive S-100 protein immunochemistry staining is required for the diagnosis.This study was carried out in accordance with the recommendations of the journal, with written informed consent from the patient. The patient gave written informed consent in accordance with the Declaration of Helsinki. The protocol was approved by the Institutional Board of Chest Hospital Sotiria, Athens, Greece.SC wrote the majority of the manuscript. TK wrote part of the manuscript and offered intellectual contribution. All the authors contributed to the results provided and approved the manuscript. AT, DB, and KD offered also intellectual contribution.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


The dog achieved a complete response, and one year later the dog is still alive and well with no evidence of tumor recurrence. Radiotherapy in combination with carboplatin chemotherapy was effective in the long-term control of this rare disease.Anal sac squamous cell carcinoma (SCC) is a rare tumor in dogs. Only eight cases have been described in the literature, and previous reports of treatment only describe surgery or palliative treatment with non-steroidal anti-inflammatory drugs. We report a case of a 12-year-old female neutered Labrador with locally advanced anal sac SCC. The dog was treated with four cycles of carboplatin 300 mg/m A 12-year-old female neutered Labrador retriever was presented for a mass in the left anal sac. The mass was removed by the referring veterinary surgeon, and a histological diagnosis of anal sac SCC (incompletely resected) was made by a board-certified pathologist. The tumor rapidly recurred less than one month later and the dog was referred for further investigation and treatment. On presentation, the owner reported tenesmus and discomfort in the anal region. A firm, ulcerated mass, approximately 5 cm in diameter, extended over the entire left perianal region, involving slightly more than 50% of the anal circumference . On rectHematology and biochemistry were within normal limits. Abdominal ultrasound and thoracic radiography revealed no evidence of local or distant metastases. Advanced imaging, computerised tomography (CT) scan was declined by the client for financial reasons. Due to its rapid recurrence following surgery, its location, and extent, the tumor was considered inoperable. The prognosis was considered to be very poor.2 every three weeks . Complete blood counts were performed prior to each carboplatin administration and were within normal limits. The patient was also continued on 0.1 mg/kg of oral meloxicam  once a day as previously prescribed by the referring veterinary surgeon. Amoxicillin/clavulanate  15 mg × kg was administered twice a day for two weeks for the treatment of secondary infection of the ulcerated tumor mass. The anal mass reduced in size during the first four weeks of radiotherapy/chemotherapy treatment and no acute side effects from the chemotherapy and radiotherapy were observed.A palliative course of radiotherapy was prescribed and the dog received four, once weekly 8.5 Gy fractions of 12 MeV electrons  delivered by a Varian Clinac 2100 IX DMX . The patient was placed in right lateral recumbency with the beam perpendicular to the tumor in an “en face” position . The radTwo weeks following the final radiotherapy treatment, the tumor had completely regressed with no further signs of the tumor on rectal palpation . The dogSCC is a relatively common tumor arising from the oral mucosa of the mouth and the subungual epithelium ,2,3. LesAnal sac squamous cell carcinoma (SCC) is a rare tumor in dogs. Only eight cases have been previously published in the literature, with no reports describing response to chemotherapy and radiotherapy. In the five cases reported by Esplin et al., 2003  it was sIn dogs, the combination of radiotherapy with carboplatin chemotherapy has been reported to have some benefit in the treatment of tonsillar and oral SCC ,7,8,9,10For definitive or curative intent radiotherapy of pelvic tumors, a fractionated photon protocol is indicated to achieve a dose at depth and minimize the risk of late toxicity to the rectum . PalliatA CT scan of the abdomen was not performed, so it could be possible that a potential enlargement of the pelvic lymph nodes were missed at the abdominal ultrasound examination. The same could be said for metastatic screening of the lungs by thoracic radiography rather than by CT. However, due to the lack of lymphadenopathy and/or metastases at the following rechecks, this appears unlikely.Carboplatin is a radiation sensitizer and combined therapy has been reported to improve outcomes in people with head and neck SCC , but thiLate side effects of chemo-radiation combinations are reported in around 40% of human patients treated for head and neck cancer . Late efOur patient did not develop any late side effects apart from complete alopecia remaining one year following the treatment. It is still possible that more serious late side effects could manifest in the future.It is our hypothesis that the durable response achieved in this case resulted from the combination of radiation and carboplatin through the radiation-sensitizing actions of the latter. However, it is not possible to know which treatment the tumor actually responded to.Complete and durable response of gross disease to carboplatin in combination with piroxicam has been previously reported for oral non-tonsillar SCC . HoweverWe report a case of complete response with long-term tumor control and survival in a dog with locally recurrent, advanced, and inoperable anal sac SCC following treatment with hypofractioned palliative radiotherapy and carboplatin chemotherapy. The treatment was successful and the patient is alive and well, without signs of recurrence of the disease one year later. Combinations of carboplatin and radiotherapy could be useful in the treatment of anal sac SCC, but the risks of late side effects need to be evaluated, particularly in view of the possible long-term control of the disease. A well-designed, highly powered study would be required to investigate the efficacy of carboplatin and radiotherapy for this uncommon tumor.


Osteoid osteoma in periarticular lesions tends to have an unusual presentation that likely leads to a delayed or missed diagnosis compared with a typical osteoid osteoma in the metaphysis or diaphysis of the long bone. In cases that are unresponsive to conservative treatment, surgical interventions including en bloc resection, computed tomography-guided percutaneous treatment, and arthroscopic resection have been performed; however, these methods frequently result in inadequate tumor resection and recurrence. Here we present a case of a 16-year-old girl with osteoid osteoma in the talar neck presenting as anterior impingement syndrome due to marked synovitis in the ankle joint which was successfully treated without complications by arthroscopic synovectomy and tumor resection followed by intraoperative 3D C-arm-based imaging confirming complete tumor lesion removal. Her pain was relieved immediately after the surgery, and there was no recurrence at 12 months of follow-up. This is the first case report of the surgical treatment of the osteoid osteoma in the talar neck with the combination methods of arthroscopy and 3D C-arm-based imaging. Osteoid osteoma (OO) is a common benign osteoblastic bone tumor that affects mainly children and young adults . AlthougA 16-year-old Japanese girl with no past medical history or previous injuries presented to a nearby clinic complaining of chronic anterior left ankle pain for the past 2 years. She was diagnosed with anterior ankle impingement syndrome by ultrasonographic findings of synovial hyperplasia in the anterior aspect of the ankle joint and referred to our hospital for further treatment. A physical examination revealed tenderness and swelling across the anterior aspect of the left ankle but no local heat or redness of the overlying skin. She played badminton three times a week and complained of increasing pain with sports activities. Careful interviewing revealed that she also had pain at rest which increased with motion in the morning which was strong especially immediately after awakening.The range of motion of the left ankle was normal, and marked pain was observed with forced dorsiflexion. There were no signs of ankle instability. A blood test showed that her white blood cell count, C-reactive protein level, and matrix metalloproteinase-3 level were within normal limits. Rheumatoid factor and anticyclic citrullinated peptide antibody tests were negative. Plain radiographs showed a small exostotic bony bulge on the talar neck which resembled a traction spur and a recess on the talar neck . ComputeWe initially considered two pathologies as differential diagnoses: one was anterior ankle impingement syndrome considering the bone lesion as a recess, flake, and spur caused by impaction of the distal tibia against the talar neck; and the other was OO in the talar neck considering the bone lesion in CT and MRI as a nidus with secondary synovitis in the ankle joint. Activity modification and daily oral aspirin therapy slightly reduced but did not eliminate her symptoms. An intra-articular steroid injection provided some pain relief; however, the effect lasted only 1 week. Persistent synovitis unresponsive to conservative treatments for several months prompted us to narrow down the differential diagnosis to OO. The patient opted for conservative treatment consisting of oral aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs); however, her symptoms persisted and she finally decided to undergo surgical resection 14 months after the first visit.We performed arthroscopic surgery using anteromedial and anterolateral portals. The patient was placed in the supine position on a radiolucent carbon fiber table with the ankle manually distracted. Arthroscopy revealed capillary hyperemia and synovial hyperplasia in the anterior aspect of the ankle joint . We perfPostoperatively, the patient reported immediate relief of her ankle pain. She was followed up for 12 months without lesion recurrence. Symptoms of anterior ankle impingement disappeared with no functional disability at the latest follow-up.OO in the foot typically involves a subperiosteal lesion of the talar neck . The diaProstaglandins produced in OO via the strong expression of cyclooxygenase-1 (COX-1) and COX-2 are considered to cause characteristic nocturnal pain that is responsive to nonsteroidal anti-inflammatory drugs (NSAIDs) in typical OO and synovitis in OO located within periarticular lesions . The natOO is treated by several invasive methods including open curettage, en bloc resection, CT-guided percutaneous local ablation, and arthroscopic resection . Open reThe open surgical excision of OO was recently replaced by less invasive methods. CT-guided percutaneous therapies including core drill excision , laser aArthroscopic excision is another minimally invasive treatment option for intra-articular OO which has shown good clinical results , 22–26. Several studies have reported that minimally invasive surgery of OO with 3D C-arm-based imaging is a highly effective and safe procedure –31. ThisIntraoperative CT imaging  is another option for intraoperative imaging. O-arm delivers higher image quality and quicker 3D reconstruction time than 3D C-arm; however, O-arm has some disadvantages . First, To our knowledge, this is the first report to describe a combination method of arthroscopy and 3D C-arm-based imaging. Based on the previous studies and our experience in the present case, we believe that this combination method is a useful option for the treatment of intra-articular OO. The advantages of this method against arthroscopy alone are capabilities to access the nidus easily and less invasively and to confirm the complete resection. The disadvantages are its cost and radiation exposure to patients, though the amount is of acceptable level.We described a case of talar neck OO showing an unusual presentation of anterior ankle impingement syndrome. Using arthroscopy and 3D C-arm-based imaging, we performed a synovectomy, clearly exposed the nidus, and intraoperatively confirmed complete lesion removal without complications.