Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures.Here, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the distal ileum presenting as intussusception. A 53-year-old female patient without pathological antecedent for neurofibromatosis was admitted with pain in the right lower abdomen and multiple episodes of vomiting for 1 week. Preoperative diagnosis was intussusception with a contrast-enhanced computed tomography scan (CECT) of the abdomen showing characteristic target sign. Due to difficulty reducing the ileum-colon intussusception, right hemicolectomy with ileocolostomy was performed. Histopathology was suggestive of low-grade MPNST. The patient received postoperative care and was followed up for 9 months. There is no sign of tumor recurrence and metastatic disease.This case is unique in terms of a rare tumor presenting with unusual complication. Malignant peripheral nerve sheath tumors (MPNSTs) are defined as any tumor arising from a peripheral nerve or exhibiting nerve sheath differentiation. Most MPNSTs are located along major nerve trunks, commonly arising on the body trunk, extremities, head, neck, and paravertebral regions. MPNSTs arising from nerves of the small intestinal wall are extremely rare, with only 10 cases reported worldwide. In this report, to the best of our knowledge, we describe the first Chinese case of a malignant peripheral nerve sheath tumor of distal ileum presenting as intussusception in a patient.A 53-year-old female patient presented to the First Affiliated Hospital of Beilun Branch of Zhejiang University China on September 2015 complaining of pain on the right side of her abdomen associated with abdominal distension and multiple episodes of vomiting for 1-week duration. She denied changes in bowel habits, blood in stools, and past abdominal operations. Her family history was insignificant for neurofibromatosis type 1. On abdominal examination, she exhibited abdominal guarding, but no palpable mass was found. Abdominal CECT demonstrated intussusception in the ascending colon with a smooth enhancing 3.5 cm mass suspicious for neoplasm Fig. , right hThe patient declined to undergo further genetic analysis and chemotherapy. She had a good postoperative course and was discharged 2 weeks after surgery. She presented with diarrhea, fever, and moderate anemia at 3 and 7 months post-operation. Abdominal CECT demonstrated no sign of tumor recurrence and metastatic disease. She underwent symptomatic treatment and was discharged home. In June 2016, she died due to complications of her disease.According to the WHO, MPNSTs are defined as any tumor originating from a peripheral nerve or exhibiting nerve sheath differentiation. MPNST is the sixth most common type of soft tissue sarcoma , 2. ApprMost MPNSTs are located along major nerve trunks, commonly arising on the body trunk, extremities, head, neck, and paravertebral regions . MPNSTs There seems to be no characteristic clinical symptoms of MPNST of the intestine. Most patients experience fatigue, weight loss, emesis, abdominal pain, and intestinal bleed . Our patThe quantitative FDG-PET imaging is used to distinguish between benign PNST and MPNST based on a tumor’s metabolic activity , 13. DueIt is important to recognize that there is still a lack of widely accepted diagnostic criteria for MPNST . These tForty to 65% of MPNST patients experience local recurrence, and 30 to 60% develop metastases within 12 months of initial surgery . FactorsBecause of its rarity, the optimal treatment of the small bowel MPNST is not well established . CurrentAccording to the clinical and pathological characteristics, this patient was diagnosed the MPNST of the small bowel. Unfortunately, further genetic analysis was not obtained, due to patient preference as this could have been helpful for creating further treatment plans. Here, we report a rare case of MPNST of the distal ileum, which, to the best of our knowledge, was also the first Chinese case of this disease. Although MPNST arising from the small bowel is extremely rare, a surgeon should also be aware of the possibility of MPNST when dealing with intestinal obstruction or intussusception. Since patients with MPNST have very poor prognosis, it is necessary to perform a quick frozen section in the operation for a clear diagnosis. We suggest an extended radical operation in case of the fast frozen section displaying spindle cell tumors of the small bowel.To date, little is known regarding MPNST of the small bowel. We report a rare case MPNST presenting as ileum-colon intussusception. Multidisciplinary approach is essential for diagnosis and treatment of these tumors. There is no definitive guideline available for treatment of small bowel MPNST. We recommend wide excision of these tumors with very close postoperative follow-up imaging. However, the role of adjuvant radiotherapy and chemotherapy is still under debate reserved only for positive margins and recurrent tumors or when wide local excision is unfeasible.


A foreign body granuloma of the buccal mucosa induced by honeybee sting was reported. The patient was an 82-year-old female who presented with a submucous mass at the right buccal mucosa. The mass was 20 mm in diameter, elastically firm, partly mobile without pain or tenderness, and covered with almost normal mucosa. MR image did not delineate the lesion clearly. Under clinical diagnosis of a benign tumor, the lesion was excised under local anesthesia. The excised lesion was 14 × 11 × 9 mm in size and solid and yellowish in cut surface. Histologically, the lesion consisted of granulomatous tissue with a few narrow, curved, eosinophilic structures compatible with decomposed fragments of a honeybee sting and was diagnosed as a foreign body granuloma, although the patient did not recall being stung. Diagnosing a submucous mass of the buccal mucosa is a challenge. Clinically, it can be a local inflammatory reaction, a tumor, a cyst, or other conditions. Imaging of the lesion in addition to clinical findings and anamnesis may be helpful for the diagnosis. However, histological examination is inevitably necessary for a definitive diagnosis.In this report, we presented the rare case of a foreign body granuloma of the buccal mucosa induced by a honeybee sting, although the patient did not recall being stung.An 82-year-old female patient was referred to our department for a submucous mass at the right buccal mucosa. The lesion was pointed out at the dental office when she presented for a new prosthesis. The patient had suffered hypertension and senile dementia for several years. The mass was 20 mm in diameter, elastically firm, partly mobile without pain or tenderness . The oveA foreign body granuloma of the oral mucous membrane can be induced by a variety of materials such as teeth, dental materials, oral care goods, tableware, foods, toys, and implants. However, foreign bodies derived from living creatures are rarely found because of the very rare chance for those to enter the oral cavity. A foreign body granuloma developed in the oral mucous membrane after a bee sting is extremely rare and thus is challenging to diagnose. Confirmation of bee stinging is a great help for diagnosis in addition to histological identification of bee sting apparatus.Bee stings can occur accidentally in daily life. Forestry and field workers who work outdoors are at high risk . ReactioA honeybee sting comprises two barbed lancets and one stylet with a venom canal in its center . The honThe honeybee sting apparatus can be identified in the tissue if the sting site was excised shortly after stinging. Histological confirmation of the characteristic structure of honeybee sting apparatus in addition to memory of being stung can lead to a definitive diagnosis. However, the retained materials may be decomposed into small fragments after a long period. Furthermore, the patient might not recall being stung. In such a circumstance, a definitive diagnosis is quite difficult unless the anatomical structure of the sting apparatus is well understood. In the present case, a few narrow, curved, eosinophilic materials were found within the lesion and were decomposed but compatible with the fragments of honeybee sting apparatus. Therefore, the diagnosis of a foreign body granuloma induced by honeybee sting was made.A foreign body granuloma of the buccal mucosa induced by honeybee stinging is rarely encountered. In the present case, histological identification of the decomposed fragments of a honeybee sting led to the definitive diagnosis, although the patient did not recall being stung.


Although ectopic pancreatic tissue is common in the upper gastrointestinal tract, the incidence of ectopic pancreatic tissue in the jejunum is low, and malignant transformation in ectopic pancreatic tissue is rare. Furthermore, pancreatic-type acinar cell carcinoma (ACC) developing in the jejunum and ACC accompanied by tumor thrombus are extremely rare.A 78-year-old-woman presented with melena. Abdominal computed tomography images and endoscopic examination revealed a submucosal jejunal mass with tumor thrombus extending into a jejunal vein. The patient underwent a curative resection combined with a partial jejunectomy and partial pancreatectomy. Histopathological examination of the resected tissue showed tumor cells with a homogeneous acinar architecture identical to pancreatic-type ACC and tumor thrombus. Postoperatively, she was followed for 10 months and had no recurrence.We present an extremely rare case of pancreatic-type ACC in the jejunum with extensive tumor thrombus invading into the mesenteric venous system. This type of cancer has not been reported previously but should be considered in the differential diagnosis of a jejunal mass. Ectopic pancreatic tissue is most frequently found in the upper gastrointestinal tract, and malignant transformation of ectopic pancreatic tissue is rare. In the English literature, approximately 30 cases of adenocarcinoma arising from ectopic pancreatic tissue have been reported, and most occurred in the upper gastrointestinal tract , 2. OnlyAcinar cell carcinoma (ACC) is a rare malignant tumor that accounts for 1–2% of all exocrine pancreatic neoplasms and usually arises in the pancreatic parenchyma . ACC derWe herein report an extremely rare case of pancreatic-type ACC presenting as a submucosal jejunal tumor accompanied by tumor thrombus and review the previously reported cases of heterotopic ACC.4 cells/μL; aspartate transaminase, 25 IU/L; alanine aminotransferase, 16 IU/L; total bilirubin, 0.8 mg/dL; albumin, 3.6 g/dL; and creatinine, 0.48 mg/dL. Tumor markers  were within normal limits.A 78-year-old woman was admitted to another hospital complaining of melena and transferred to our facility for further examination. Her physical examination was unremarkable. The laboratory values were as follows: white blood count, 4220 cells/μL; hemoglobin level, 12.4 g/dL; platelet count, 16.4 × 10Abdominal computed tomography scans revealed an 8.5 × 4.0 cm exophytic submucosal tumor in the jejunum Fig. . The tumDouble-balloon enteroscopy showed an approximately 3 cm submucosal tumor with central ulceration in the jejunum Fig. . EndoscoOur preoperative diagnosis was a jejunal adenocarcinoma. Although there was tumor thrombus in the mesenteric venous system and the possibility of pancreatic invasion, we considered the tumor resectable.At surgery, there was neither peritoneal dissemination nor liver metastasis. The tumor was located mainly in the mesentery of the upper jejunum. No invasion of the superior mesenteric artery or vein was found. We did not observe invasion of the pancreatic uncinate process grossly, but we resected it. Accordingly, the patient underwent a curative resection combined with a partial jejunectomy (approximately 50 cm of the jejunum) and a partial pancreatectomy (uncinate process).The gross examination revealed an 8.5 cm, soft, circumscribed, yellowish-white, submucosal mass in the jejunum Fig. . HistoloThe postoperative course was uneventful. The patient received adjuvant chemotherapy with S-1  after the curative resection. She was alive and without recurrence at her last follow-up visit 10 months postoperatively.To the best of our knowledge, this is the first report of pancreatic-type ACC in the jejunum accompanied by tumor thrombus in the mesenteric venous system. This report highlights the clinicopathological findings of an extremely rare case and reviews the features of previously reported cases of heterotopic ACC.There have been only 13 cases, including our case, reporting heterotopic ACC in a digestive organ (Table Ectopic pancreatic tissue is found in 0.5–13.7% of laparotomy and autopsy cases and is usually located in the upper gastrointestinal tract . MalignaACC is typically relatively circumscribed and large with extensive hemorrhage and necrosis . The chaAlthough ACC has been considered to have a poor prognosis, the surgical resection and 5-year survival rates after resection have been reported as 76.5 and 43.9%, respectively, in a nationwide survey performed in Japan . FurtherPancreatic cancers frequently invade the portal venous system leading to extrinsic portal vein obstruction. However, intrinsic venous obstruction by tumor thrombus, while a common occurrence in hepatocellular carcinoma, rarely occurs in pancreatic cancer . Indeed,The efficacy of neoadjuvant or adjuvant chemotherapy for ACC arising in ectopic pancreatic tissue is still unknown. In patients with unresectable ACC, fluorouracil-based chemotherapy should be considered a neoadjuvant or palliative treatment . HoweverIn our case, the patient had a high risk of recurrence because of extensive tumor thrombus. Therefore, administered S-1 as adjuvant chemotherapy according to the treatment for pancreatic cancer after curative resection . The patIn conclusion, we have presented an extremely rare case of pancreatic-type ACC arising in the jejunum with extensive tumor thrombus into the mesenteric venous system. Although it is difficult to diagnose heterotopic ACC because of its rarity, such lesions may develop in the gastrointestinal tracts as submucosal tumors, sometimes accompanied by tumor thrombus. Curative resection in these cases could be associated with increased survival.


Pulmonary arteriovenous malformations (PAVMs) can be associated with life-threatening complications such as paradoxical embolization, cerebral abscess, and hemothorax. Therefore, all adults with PAVMs should be offered treatment. Percutaneous transcatheter embolization is the first-line treatment, but 5–25% of cases require further treatment due to persistence after embolization. Recently, the role of minimally invasive thoracic surgery as a definitive treatment has been evaluated. We describe a case of a small peripheral PAVM causing hemothorax, which was safely treated with video-assisted thoracoscopic surgery (VATS). In our case, the PAVM appeared to protrude into the pleural cavity on chest computed tomography (CT), perhaps explaining why it led to a hemothorax.A 64-year-old man with a history of a brain abscess, for which he underwent surgery 6 months previously, developed a left-sided hemothorax. He had experienced recurrent epistaxis and received anticoagulation therapy for chronic atrial fibrillation. Chest CT after drainage revealed a solitary 15-mm nodule in the periphery of the left lower lobe, and identification of a feeding artery and draining vein on three-dimensional CT suggested that the node was a PAVM. The PAVM was adjacent to the diaphragm and multi-detector CT (MDCT) and three-dimensional CT (3DCT) showed that the nodule slightly displaced the diaphragm and protruded into the pleural cavity. After discussion in a multidisciplinary conference, it was decided that surgical treatment would be preferable to catheter embolization. The patient underwent VATS with three ports, the largest of which was 15 mm. The PAVM protruded from the peripheral lung like a polyp, and wedge resection was performed after simple adhesiolysis. There were no complications, and the patient is asymptomatic after 1-year of follow-up.As in the present case, PAVMs protruding into the pleural cavity can cause hemothorax, and surgical wedge resection of the involved lung as a definitive treatment is feasible and possibly safer than catheter embolization, particularly if the PAVM is localized close to the visceral pleura. Protrusion into the pleural cavity (polypoid appearance) was detected using MDCT and 3DCT preoperatively. Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between pulmonary arteries and veins. Approximately 70% of PAVMs are associated with hereditary hemorrhagic telangiectasia (HHT), and 15–50% of patients with HHT have PAVMs . While tWe describe the case of a small peripheral PAVM associated with HHT and causing a hemothorax, which was safely treated with video-assisted thoracoscopic surgery (VATS). In our case, the PAVM protruded into the pleural cavity and was detected on chest computed tomography (CT) preoperatively.A 64-year-old man was admitted to an outside hospital 6 months prior with a brain abscess and underwent surgery. He experienced recurrent epistaxis and was on anticoagulation therapy for chronic atrial fibrillation. During follow-up, he developed dyspnea, and chest CT showed a left-sided pleural effusion Fig. a. Chest The patient was placed in the right lateral decubitus position, and VATS with three ports was performed. The maximum incision length was 15 mm. The PAVM protruded from the periphery of the lung like a polyp, and wedge resection was easily performed after simple adhesiolysis Fig. . There wIn this report, we described a characteristic case of PAVM with a polypoid appearance  in a patient with a history of a brain abscess. The patient presented with hemothorax, and the PAVM was easily and safely treated with VATS wedge resection. We performed VATS to treat the PAVM instead of electing for transcatheter embolization. The reasons for this were that, firstly, the PAVM was a solitary lesion located in the periphery of the lung; VATS wedge resection could thus be safely and easily undertaken. Secondly, as the patient had already experienced complications including a prior brain abscess and hemothorax, we sought to perform the safest and most definitive procedure. The PAVM protruded from visceral pleura into the pleural cavity, perhaps explaining why it ruptured.In previous reports, the location and morphology of PAVMs have not always been described, so the frequency of PAVMs with a polypoid appearance as in our case is unclear. One review described that approximately 81% of PAVMs involved the pleura . A case In our case, the solitary PAVM was located in the left lower lobe and had a single feeding pulmonary artery and draining vein. According to some reviews, 53 to 70% of PAVMs are found in the lower lobes, 75% are unilateral, and 64% are solitary lesions. Eighty percent to 90% of PAVMs have a single feeding segmental artery and a single draining vein and are defined as simple type , 7. PAVMThe diagnosis of HHT is based on clinical criteria or genetic testing. In our case, the patient was categorized as “definite” HHT with three of the four clinical criteria for diagnosis of HHT . He expeInternational guidelines for HHT suggest that there is no role for the surgical management of PAVMs other than in cases of life-threatening bleeding, except for cases wherein the patient is in a center where catheter embolization is unavailable . HoweverPAVMs protruding into the pleural cavity can cause hemothorax, and surgical resection as a definitive treatment may be safer than catheter embolization and certainly may be more readily available from a resource standpoint. A solitary PAVM located adjacent to the visceral pleura can be removed easily and safely with VATS wedge resection with almost no risk of recurrence. Protrusion into the pleural cavity (creating a polypoid appearance) can be detected with MDCT and 3DCT preoperatively.


Necrotising fasciitis is a rare but potentially lethal condition in obstetrics which usually presents with fulminant tissue destruction and a resultant high mortality. We report a 19-year-old Sub-Saharan female diagnosed with a rapidly erosive necrotising fasciitis on day 5 after caesarean section in a resource-limited setting. Timely diagnosis, aggressive antibiotic therapy, and prompt surgical intervention via an extensive abdominal wall debridement were pivotal to her survival. Staphylococcus aureus (MRSA) has been implicated in up to a third of cases [Necrotising fasciitis (NF) is a severe infection involving the skin, subcutaneous tissue, and superficial fascia, which often has a fulminant course and a potentially lethal outcome –5. It isof cases .Necrotising fasciitis had been earlier described by Hippocrates in the 5th century BC , but itsThough a multidisciplinary team is best suited to handle such cases , 5, therWe report a 19-year-old lady with a previous vaginal delivery who underwent a lower segment caesarean section (CS) for acute foetal distress at 39-week gestation. She delivered a healthy male baby and the surgery lasted 43 minutes. There were no intraoperative complications. She has no known medical condition and had an uncomplicated antenatal course.3, HIV negative, 116 mg/dL, and 8.3 g/dL, respectively. A decision to do an exploratory laparotomy was taken. Upon reopening the lower segment incision (Pfannenstiel), a pungent and offensive greenish seropurulent discharge oozed from the site. Further exploration revealed abundant foul smelling ascites and an extensive spread of greyish necrotic tissues involving the skin, subcutaneous tissue, recti muscles, fascia, and anterior serosa of myometrium. The hysterotomy site was nondraining and hyperaemic. An intraoperative diagnosis of necrotising fasciitis was made and all necrotic tissues were excised; the peritoneal cavity was thoroughly washed out and mopped using an adapted abdominal mop due to unavailability of a suction machine. This “abdominal mop” consisted of a commercially available baby napkin (diaper) made of cloth which was sterilised in an autoclave before it was used ; other vital signs were within normal ranges and the incision site was clean with no evidence of surgical site infection. She was managed with analgesics (tramadol) and ampicillin was continued as part of her routine postoperative care. By day 5 postop, she developed an acute abdomen. The incision site had become very tender and soft tissue crepitation was noted on either side of the incision. Due to limited resources, only a white cell count, HIV status, random blood glucose, and a haemoglobin level could be done whose values were 39.800 cells/mmwas used . Two locHowever, due to a deteriorating clinical state , abundant drainage, and continual progression of necrosis, the wound was extensively debrided approximately 2 cm beyond the bleeding edges to ensure adequate resection on day 6 after laparotomy. The large defect  with nei Glycine max (in undocumented quantities) was provided . Two weeks after extensive debridement, a granulating and healthy looking wound was seen and regular breastfeeding had resumed. By the 15th week, the abdominal defect had closed and intraabdominal organs were nonvisible , also referred to as the “flesh-eating disease,” is a life-threatening infection which is usually polymicrobial in nature and requires timely surgical intervention and aggressive antibiotic therapy. It is a rapidly spreading infection and can involve all the layers of the soft tissue compartments as well as deeper tissues in the pelvis , 14–16. Clinically, most patients present with a dusky looking wound margin, bullae, crepitus, and signs of inflammation including swelling, induration (which may be difficult to recognize in dark skin patients), and severe pain disproportionate to local findings at the affected site , 15–17. Whilst treatment with antibiotics plays an irreplaceable role in the management of NF, surgical intervention is the mainstay of management and should be done promptly as there is no room for “wait and watch policy” , 16, 17.Glycine max: 38–45% protein content) is an effective adjunct to enhance healing. Our case management may not be standard, but it is subject to refinement and sets the platform for improvising in the management of various pathologies in the many health care-deprived resource-disadvantaged communities in Sub-Saharan Africa with little or no access to specialised care.This case underscores the importance of a high index of suspicion and prompt surgical intervention, even in the absence of hallmark features in managing necrotising fasciitis. Whilst not undermining the paramount importance of a highly specialised multidisciplinary team in managing such rare life-threatening conditions in sophisticated units, we have reported a successful management by primary health care physicians  with locally adapted materials in a resource-limited setting where referrals to tertiary centres seems a death sentence due to the associated unbearable financial burden. Though it is recommended that antibiotic treatment be adjusted according to microbiological findings, the role of empiric broad spectrum antibiotics in controlling the spread of the infection in such settings cannot be overemphasized. Nutrition with cost-effective and readily available food supplements like soya bean (


Osteochondroma is a common benign bone tumor, protruding from the underlying normal bone. Osteochondromas can cause pain depending on their location and size. The pes anserinus is located at the proximal medial side of the tibia, where the tendinous insertions of the sartorius, gracilis and semitendinosus muscles collectively attach. Pes anserinus syndrome, or anserine bursitis, is a painful condition of the pes anserinus, and is more common in adults typically with overweight females. The occurrence of pes anserinus syndrome is rare in the paediatric population.In the current case series, five patients with pes anserinus syndrome due to proximal tibial osteochondroma are reported. Pain was present in all cases, with snapping in one case. The average age of the patients was 13 ± 1.2 years, ranging from 12 to 15 years. Three patients had a single osteochondroma, and two patients had hereditary multiple exostoses. The sizes of the osteochondromas on plain radiographs varied from 0.5 to 2.5 cm, with an average of 1.46 ± 0.83 cm. All lesions characteristically were located at the medial-posterior edge of the proximal tibia. The symptoms resolved in four cases with surgical resection, and persisted in one non-resected patient.The characteristic location of the osteochondroma causes pes anserinus syndrome, even though the lesion is small. The diagnosis of osteochondroma or pes anserinus syndrome may be overlooked when it occurs in a paediatric population. The symptoms seem to be consistent, and resection of the osteochondroma is necessary for treatment. Osteochondroma is the most common benign tumor of bone, and is characterized by an exophytic lesion composed of cortical and medullary bone with an overlying hyaline cartilage cap . Two cliThe pes anserinus is located at the proximal medial side of the tibia, where the tendinous insertions of the sartorius, gracilis and semitendinosus muscles collectively attach , 8. ThisIn the current report, five paediatric cases of pes anserinus syndrome due to osteochondroma are described. The article focuses on the clinical character and diagnosis with an emphasis on the paediatric population because the diagnosis of pes anserinus syndrome can be overlooked in this population.1.A total of five cases of pes anserinus syndrome due to osteochondroma were diagnosed in one boy and four girls with a mean age of 13 ± 1.2 years (range from 12 to 15 years). Pain was present in all patients, and snapping was present in one patient. The sizes of the osteochondromas on plain radiographs varied from 0.5 to 2.5 cm, with an average of 1.46 ± 0.83 cm. The lesions were on the right side in four cases, and on the left side in one case. All lesions characteristically were located at the medial-posterior edge of the proximal tibia. Four lesions were surgically resected, and the symptoms disappeared. One patient did not undergo surgical resection and continued to experience persistent pain at the two-year follow-up. We describe the details of each case below. Clinical data are summarized in Table 1a). CT depicted a protuberant bone lesion with continuity to normal bone , but an overlying cartilaginous cap was not obvious. The MRI showed the lesion had continuity to the host bone marrow. There was no obvious inflammation characterized by high signal intensity on a T2 weighted fat-suppression image around the tip of the osteochondroma . For surgical resection, the lesions were approached posterior to the conjoint tendon . While retracting the attachment of the pes anserinus, the lesion was resected using a curved chisel. Histologically, the specimen showed partially sclerotic lamellar bone trabeculae covered by an attenuated hyaline cartilaginous cap undergoing endochondral ossification with fatty marrow, compatible with osteochondroma. There was no evidence of malignancy. Six months after surgery, the symptoms disappeared.A 12-year-old boy presented with pain and a mass on the medial aspect of the right proximal leg. He first noticed symptoms at around 10 years of age. The pain appeared on knee flexion, and was absent when he walked or ran. His height and body weight were 162 cm and 59 kg, respectively. He had no past medical history related to the lesion. There was no history of trauma or injury at the knee. On examination, a non-mobile hard mass was palpable with tenderness at the medical side of the proximal tibia. Swelling was not obvious. Plain radiographs showed a lesion protruding from the bone with continuity to the underlying bone. The size of the lesion was 0.8 cm on a plain radiograph . The size of the lesion was 2.0 cm on a plain radiograph. MRI depicted a protuberant bone lesion with continuity to normal bone. However, an overlying cartilaginous cap or surrounding inflammation was not apparent . Surgically, the lesions were approached posterior to the conjoint tendon, and resected using a curved chisel . The lesion was composed of lamellar bone trabeculae with fatty marrow and a covering cartilaginous component, compatible with osteochondroma. There was no evidence of malignancy. Six months after surgery, the preoperative symptoms had disappeared.A 13-year-old girl presented with pain and swelling on the medial aspect of the proximal leg, which she had noticed for several years. Her height and body weight were 161 cm and 64 kg, respectively. She had no past medical history related to the lesion. There was no history of trauma or injury. On physical examination, the non-mobile hard lesion was palpable with slight tenderness on the medial side of the proximal tibia. Plain radiographs showed a lesion protruding from the bone with continuity to the underlying bone . CT depicted a small protuberant bone lesion with continuity to normal bone . Surgically, the lesions were approached posterior to the conjoint tendon, and resected with a curved chisel while retracting the attachment of the pes anserinus. The lesion was composed of a hyaline cartilaginous cap and underlying lamellar bone trabeculae and fatty marrow . There was no evidence of malignancy. Six months after the surgery, the symptoms had disappeared.A 13-year-old girl had noticed pain for six months when she ran, but no pain while she walked. She also noticed snapping at the medial site of the proximal leg on knee flexion. Her height and body weight were 150cm and 42kg, respectively. She had no past medical history related to the lesion. On physical examination, a small hard lesion was palpable. Tenderness at the medial side of the proximal tibia was observed. The snapping phenomenon was reproduced on knee flexion. Plain radiographs showed a small 0.5 cm osseous lesion on the antero-posterior radiographic view . The size of the lesion on the medial-posterior proximal tibia was 2.5 cm on a plain radiograph. The resection was performed using a postero-medial approach. The conjoint tendon was retracted anteriorly, and the protuberant lesion was resected using a curved chisel. The lesion was composed of a hyaline cartilage cap and underlying lamellar bone trabeculae with fatty marrow . There was no evidence of malignancy. The medial collateral ligament at the proximal tibia was partially detached. Pain persisted after the operation for about two weeks. Six months after surgery, the preoperative symptoms had disappeared.The patient was diagnosed with multiple hereditary exostoses in early elementary school. She had difficulty in fully flexing the right knee because of pain on the medial aspect of the right proximal tibia when she was 12 years old. The symptoms lasted for 2-3 years. She did not have pain while walking. Her height and body weight were 150 cm and 39 kg, respectively. On examination, multiple bony exostoses were palpable on the distal thigh and proximal leg. Tenderness was found only on the lesion of the proximal medial tibia, not at the other lesions. Plain radiographs showed a bony lesion protruding with continuity to the underlying bone in the distal femur and proximal tibia and fibula. The plain radiographic findings permitted a diagnosis of multiple hereditary exostoses . The size of the protruding lesion on the medial proximal tibia was 1.5 cm on a plain radiograph. Pain appeared occasionally, especially on flexing the knee, but did not disturb activities of daily living. The pain did not require medication, but the symptoms still persisted at the time of the two-year follow-up.The patient had multiple hereditary exostoses from her first decade. When she was 15 years old, she presented with pain on the medial aspect of the right leg. The pain appeared when she changed clothes while sitting on her buttocks and flexing the knee. The pain had been present for six months prior to her visiting a hospital. Her height and body weight were 154 cm and 45 kg, respectively. She had no other specific past history. On examination, multiple bony protuberances from multiple exostoses were palpable at the distal thigh and proximal leg. Tenderness was only found at the medial proximal lesion. Plain radiographs showed a bony protruding lesion on the distal femur and proximal fibula and tibia (Fig. Osteochondromas can cause pain depending on their location and size. Symptomatic lesions usually occur in younger patients, and 75%-80% of such cases are discovered before the age of 20 years , 3, 4. MA diagnosis of pes anserinus syndrome should be considered when there is spontaneous pain infero-medial to the knee joint , 8, 11. Pes anserinus syndrome is rare in childhood , and canPlain radiographs generally are not useful in the diagnosis of pes anserinus syndrome. In the majority of the cases, the diagnosis of pes anserinus syndrome was made clinically . The curA series of five patients with pes anserinus syndrome due to proximal tibial osteochondromas is described. The lesion is always located at the medial-posterior edge of the proximal tibia. Osteochondromas at this location cause pes anserinus syndrome, even though the lesion is small. All symptoms resolved following surgical excision of the osteochondromas. Pes anserinus syndrome could be overlooked in a paediatric population with osteochondroma, because pes anserinus syndrome is rare in this population.


Polyarteritis nodosa (PAN) presents mostly as a systemic disease characterized by necrotizing vasculitis affecting small- and medium-sized arteries. Rarely, the inflammatory process is isolated and involves a single organ without systemic manifestations. We described the case of a 57-year-old patient with isolated testicular PAN who presented with a testicular mass mimicking a primary testicular tumor.


A 27-year-old man with a 2-year history of recurrent hospitalizations for various neurologic and cardiologic emergencies was admitted to our hospital presenting with left hemiparesis, which gradually progressed to quadriparesis, bilateral hemianopsia, intracranial hypertension syndrome, and seizures. A diagnosis of echinococcosis was made, based on the radiologic findings of multiple cerebral hydatid cysts and a sizable cyst of the heart. The hydatid cyst of the heart was treated first with a thoracotomy, and after a month he underwent three consecutive surgical operations for the removal of six cerebral cysts. The patient was on albendazole treatment throughout the entire hospitalization, and this led to the shrinkage and finally to the disappearance of two other cerebral cysts. The patient's focal neurologic signs eventually disappeared, with the exception of a slight unilateral hemianopsia. Ten years after his discharge, he remains in a good condition, with no signs of clinical or radiologic relapse. Echinococcus granulosus, alveolar echinococcosis caused byEchinococcus multilocularis, and polycystic echinococcosis caused byEchinococcus vogeliand/orEchinococcus oligarthrus. The liver, lungs, and brain are predominantly involved.Hydatid disease (echinococcosis) is a common parasitic infection, mainly in sheep-raising areas of the world .The diagnosis of cerebral parasitoses depends on the causative agent. Adults or larvae of helminths or protozoa enter the central nervous system and cause meningitis, encephalitis, ventriculitis, myelitis, ischemic stroke, bleeding, venous thrombosis, or cerebral abscess, clinically manifesting as headache, epilepsy, weakness, cognitive decline, impaired consciousness, confusion, coma, or focal neurologic deficits. Headache and vomiting have been reported as the most common initial symptoms.E. multilocularis, the parasite may disseminate from there to other organs and tissues, such as the lung, heart, brain, bones, and ligaments. Cross-sectional imaging is crucial for differentiating echinococcosis from malignant processes; computed tomography (CT) is most useful for depicting the peripheral calcifications surrounding established echinococcal cysts, and magnetic resonance imaging (MRI) is most helpful for identifying echinococcosis of the central nervous system.The diagnosis is usually based on findings at radiologic imaging and in serologic analyses. Because echinococcal lesions can occur almost anywhere in the body, familiarity with the spectrum of cross-sectional imaging appearances is advantageous. Echinococcal lesions may produce widely varied imaging appearances depending on the parasite's growth stage, the tissues or organs affected, and the presence of associated complications. Although the liver is the initial site of mass infestation byAngiostrongylus costaricensis) or whether they remain undetected or asymptomatic, like 25% of neurocysticercosis cases.Treatment relies on drugs and sometimes surgery. The outcome of cerebral parasitoses is highly variable, depending on the effect of drugs, whether they are self-limiting  was based on clinical presentation, characteristics  affecting the morbidity of the disease, and the risk of potential major intracranial events . We therefore decided to perform two separate procedures, the first in the right hemisphere where the largest cyst was located and the second in the left hemisphere, because the multiplicity of the cysts in both hemispheres would render their surgical removal in one setting extremely lengthy and toilsome.However, a sudden clinical deterioration of the patient 2 weeks after the first operation made an emergent surgical procedure necessary before the planned contralateral side approach. Τherefore, neurosurgeons attempting surgical removal of such multiple space-occupying lesions should always bear in mind that by doing so they may disturb an established pressure balance inside the cranial vault and thus contribute to an imminent herniation.It is noteworthy that the cyst fluid in all cases was crystal clear, and microbiological tests of both cyst fluid and cyst wall were negative for parasites. This points to the importance of antiparasitic treatment. Albendazole in this case proved extremely efficient because it led to the shrinkage and finally to the disappearance of two other small cerebral hydatid cysts.Cerebral hydatid cyst disease should be kept in mind in the differential diagnosis of increased intracranial pressure syndrome, especially among patients in areas endemic for echinococcosis.


Spontaneous hepatic rupture in pregnancy is a rare and life-threatening event during the perinatal period.We report a case of a 33-year-old woman with 36 + 6 weeks’ gestation that present with elevated blood pressure before delivery, who was admitted to our hospital due to irregular abdominal pain. Diagnosed with abdominal paracentesis, the emergent caesarean section and laparotomy were performed. Postoperatively, the patient experienced 22-day intensive therapy in ICU and was transferred to the General Surgery Department in good physical condition without post-operative complications.This case indicates that making an accurate and timely diagnosis and taking multidisciplinary approach contribute to a successful clinical outcome. Spontaneous hepatic rupture in pregnancy is a rare condition and carries a high maternal and perinatal mortality . It is uWe reported a successfully treated case of spontaneous hepatic rupture in a pregnant woman. It emphasizes the need for the early diagnosis as well as the timely surgical management and the postoperative monitoring of patients in the intensive care unit. The relevant literature is also reviewed.9/L, reticulocyte count of 0.0525 × 1012/L, liver enzymes with an alanine aminotransferase of 281 U/L, aspartate aminotransferase of 392 U/L, lactate dehydrogenase of 525 U/L, total bilirubin levels of 19 μmol/L, and normal alpha fetoprotein. Serological markers of hepatitis B and hepatitis C virus were negative. Ultrasound scan showed that fetal heart beats could not be found and free abdominal fluid was present. There were no images suggesting placental abruption. Ultrasound-guided intra-abdominal puncture revealed active intraperitoneal bleeding.A 33-year-old primigravida woman, a body mass index of 31 kg/cm2, was admitted to the emergency department at36 + 6 weeks gestation with abdominal pain that had lasted for 6 h. She did not have a history of trauma. Her antenatal period had been uneventful except for one episode of hypertension (141/84 mmHg). Physical examination revealed a blood pressure of 102/62 mm/Hg, a heart rate of 80 beats/min and a temperature of 36.6 °C. Blood tests showed hemoglobin of 95 g/L,Hematocrit of 28.9%, platelet count of 111 × 10In less than 3 h of admission, an emergency laparotomy was performed and a dead fetus was delivered by cesarean section. No evidence of placental abruption or active intrauterine bleeding was detected. Exploration of the ovarian tubes and ovaries revealed no bleeding or neoplasm. Two liters of hemoperitoneum was removed from abdominal cavity. Active hemorrhage came from rupture of the right lobe of the liver which appeared fatty liver. Direct hemostasis was not achievable because the liver was edematous and was ruptured during the performance of sutures. Thus perihepatic packing was done by seven large gauze packs and hemostatic sponges were used to compress the bleeding lesions. Throughout the laparotomy, intraoperative bleeding was 2000 mL and the patient received 8 units of RBC and 1200 ml of plasma.The patient was transferred to the ICU after surgery to continue resuscitation with intravenous fluids and vasoactive support. The following day in ICU, she had high fever and developed the acute kidney injury and severe acute respiratory distress syndrome. She was managed by massive blood transfusion, mechanical ventilation, renal replacement therapy and antibiotic treatment. Intra-abdominal pressure was maintained between 7.5 and 11.3 mmHg (1–1.5 KPa) by abdominal binder.A second laparotomy was performed on the 6th postoperative day. A large hematoma and rupture of the right liver lobe were visible after removing the gauze packs Fig. , which wSpontaneous hepatic rupture in pregnancy, which was first described by Abercrombie in 1984, is a rare condition associated with high maternal and perinatal mortality . A recenHepatic rupture requires immediate laparotomy to stop bleeding, therapeutic options involves perihepatic packing, placement of sponges or absorbable mesh, fibrin glue, argon laser coagulation and hepatic artery ligation. Partial liver resection or even liver transplantation has been described in the literatures. The outcome of patients with spontaneous hepatic rupturegreatly depends on the etiology and severity of the hemorrhage , 12.In this case, the patient had no history of trauma, excessive alcohol consumption, or use of hepatotoxic medications, moreover, she did not have pre-eclampsia or eclampsia during this pregnancy, tumor biomarkers as well as clinical tests of hepatitis B and C virus infection were negative. Liver lesions were not visible at operation or with imaging. There was insufficient evidence for the diagnosis of HELLP syndrome: the platelet count was marginally low even with massive hemorrhage, tests of hemolysis were negative , and liver enzymes before acute hemorrhage were mildly elevated. Nevertheless, HELLP syndrome cannot be totally excluded due to the presence of hypertension. HELLP syndrome is considered as a risk factor for hepatic rupture in pregnancy. Vigil-De et al. reported that it was present in 92.8% of cases in their series during the past two decades . With thA large hematoma was on the right lobe of the liver in this patient. Perihepatic packing was carried out to control the bleeding in this case, however, she remained at risk for complications, such as intraperitoneal infection. For patient with continued bleeding after the first surgical operation, ICU assistance played a valuable role on the recovery during the post-operative course. The right hepatic lobectomy was another choice for the surgeon when hemorrhage remained uncontrolled, which probably led to postoperative liver dysfunction. Hence, an effort should be made to gain hemostatic control while avoiding large resections as much as possible.In conclusion, spontaneous hepatic rupture in pregnancy is rare but life-threatening. A high level of alertness for the diagnosis aided by timely ultrasonography and CT scan is critical to prompt intervention, which requires multidisciplinary approach to achieve a successful outcome.


How to cite this article: Karadeli E, Parlakgumus A, Tok S, Erbay G. Long Segment Intestinal Invagination in an Adult Case. Euroasian J Hepato-Gastroenterol 2017;7(1):99-100. Dear Editor,1 This is the intertwining of two consequent segments of one gastrointestinal segment. It is rare among adults. It is frequently observed in the small intestines.2 Our case was a 21-year-old female, who was admitted to our hospital with complaints of abdominal pain, nausea, and vomiting for 5 days and nondefecating problem for 1 day. The history included appendectomy 3 years previously and severe abdominal pain experienced from time to time for 3 years. The physical examination revealed rebound and diffuse sensitivity in the abdomen, being prominent in the upper right and lower right quadrants of the abdomen. The laboratory findings were as follows: C-reactive protein 17.40 mg/L, sedimentation 32 mm/hour, fibrinogen: 4.85 gm/L. Intravenous contrasted computed tomography (CT) imaging was performed due to the abdominal pain. In the CT imaging, long segment invaginated intestinal segment was observed, starting in the ascending colon level in the lower right quadrant, fitting the transverse colon level, and partially extending to the descending colon level (In pediatric ages, invagination is one of the major causes of acute abdominal disorders.on level . In the 3In 70 to 90% of the adults, the pathology may be defined for invagination. The etiology includes benign reasons, such as hamartoma, lipoma, leiomyoma, inflammatory adenoma, or Meckel’s diverticulum. Malignancy is common among colon invaginations.1 The most common complaint is abdominal pain. Nausea and vomiting are the second common complaint. Palpable mass may be observed in the abdomen.4 False kidney appearance in the sonographic longitudinal plan and target appearance in the transverse plan are diagnostic. The golden standard is CT imaging in the diagnosis, and it may also define the etiology. The treatment of the invagination is surgical in adult cases.2The clinical presentation in adults is generally chronic or nonspecific. Invagination frequently presents with acute abdominal pain, nausea, vomiting, and high white blood cell count in adults.


This report shows a multi-compartmental lipoma that occupied a vast area of the hand volar compartment, the distal aspect of the Parona’s space and infiltrated the dorsal compartment between the IV and V metacarpal bones. The mass was entirely removed through a dorsal approach, minimizing risks of neurovascular and tendinous lesions. Lipoma is one of the most common soft tissue tumors of the body , but it The informed consent was obtained from the patient involved in this study according to the Institutional Review Board and all procedures were in accordance with the institutional and national ethical standards. The patient was a retired 70-year-old female with a past medical history of hypertension, hypercholesterolemia and cutaneous psoriasis that presented a prominent swelling in her left hand. The woman had a more than 10-year history of slow increasing painless mass involving both the dorsal and volar aspects of her left non-dominant hand. In 2006, she initially noticed the swelling in the central area of the palm, and then progressively enlarged in dimensions over a period of ten years. On physical examination, the mass occupied the deep palmar space between tendons and metacarpal bones and protruded into the IV inter-metacarpal space, the third finger web and the IV and V finger, respectively. The superficial palpation of the protruding lesion revealed an elastic mass while no deeper palpation was possible. The mass itself did not cause any functional impairment or neurovascular symptoms. The overlying skin was stretched and normal. The surgical indication was given on the basis of the recent growth of the mass and on the patient’s concerns upon the potential development of neurovascular symptoms or malignant transformation of the mass.On July 2010, ultrasounds showed a well-defined avascular hyperechoic mass of 19 × 10 × 8.5 mm within the superficial soft tissues of the dorsal left hand. The lesion lay superficially to the extensor tendon of the IV finger that presented a volar tenosynovitis sized 18 mm (data not shown). On February 2016, ultrasounds showed a well-defined iso-hyperechoic avascular lesion of 40 × 20 × 40 mm. It bridged the anterior and posterior compartments of the left hand . In partThe surgery was carried out under axillary block and by tourniquet hemostasis. The mass was dorsally approached through a curved skin incision over the IV inter-metacarpal space . The masHistopathology reported a well-encapsulated tumor composed of mature adipose cells consistent with a benign lipoma. No post-operative complications were experienced by the patients, only a mild swelling and hematoma occurred both in the palmar region and in the III, IV and V fingers. About 15 days after surgery, the stitches were removed and the patient presented only a mild functional impairment of the movements of the V finger due to the muscular atrophy induced by the lesion compression. After 10 months of follow-up the lesion did not present any sign of recurrence and any functional impairment has been shown by the patient.This kind of lesions is commonly asymptomatic after the first diagnosis, however, it has been recommended to remove these masses despite asymptomatic in order to prevent complications, such as loss of strength or sensory damage . The int


Multicystic dysplastic kidney (MCDK) is the most common form of renal cystic disease in children and is one of the most common causes of abdominal mass in infancy. Here in we are reporting a rare case of a large MCKD that caused respiratory compromise and the infant presented with respiratory distress. A primigravida with supervised pregnancy delivered a full term 3.7 kg male baby at PGIMS Rohtak. The baby was born of non-consanguineous marriage, with no history of exposure to any teratogens. Baby cried immediately at birth and Apgar score was normal. On examination, baby appeared healthy but had a huge distended abdomen. The infant was in respiratory distress with respiratory rate of 70/min and mild subcostal retractions. Baby was admitted to NICU and started on oxygen via nasal prongs and other supportive management. Nasogastric tube was inserted and anal patency was checked. The Mean arterial pressure was 45 mmHg. The abdomen was distended and a huge cystic abdominal mass occupying whole of the abdomen was palpable. X-ray abdomen showed a huge homogenous soft tissue opacity occupying almost whole abdomen pushing gut to the left side.  CT scan abdomen showed a large multicystic kidney lesion measuring 14x 11.5x 8.5 mm arising from right renal fossa. The lesion was crossing the midline and displacing bowel loops with no invasion in solid viscera. Explorative laparotomy revealed a huge multicystic mass in right retroperitoneum arising from right kidney, occupying the whole abdomen. The multicystic mass was excised in toto. There were multiple cysts of variable sizes and the largest cyst measured 16cmx 16cm . On histopathological examination, the diagnosis of right multicystic dysplastic kidney was confirmed. Postoperative period was uneventful. Postoperatively, the respiratory distress settled and the patient was discharged on 10th postop day on full oral feeds. On follow up, he is doing well.MCDK is the most common form of renal cystic disease in children and is one of the most common causes of abdominal mass in infancy. It consists of cysts of varying number and size with small intervening islands of dysplastic parenchymal tissue, including immature glomeruli, primitive tubules and cysts derived from tubular and glomerular structures. The pathogenesis of MCDK is unknown, possibly results from abnormal metanephros differentiation, probably due to disturbed connection of ureteric bud with renal blastema and abnormal division at the stage of metanephros.[1] The left kidney is involved more commonly (55%) than the right kidney (45%). But our index neonate had MCDK on the right side. Also as per literature, males are affected more frequently than females .[2] Similarly, our index case was also male. Bilateral MCDK, which is often incompatible with life, contributes to 20% of prenatally diagnosed cases. Dysplastic kidney may persist without any change, increase in size, or might undergo spontaneous involution. The initial MCDK length is the most important factor for predicting complete involution.[3] In prenatally diagnosed cases, the abnormal kidney is palpable in only 13–22% of patients. It is usually asymptomatic and can remain undetected into adulthood, if not detected antenatally. But our case presented with huge abdominal mass presenting with respiratory distress which has been reported occasionally.[4] It is important to distinguish MCDK from poorly functioning hydronephrotic kidneys and multilocular cystic renal tumor.[5] ‘Prophylactic’ nephrectomy of MCDK in the 1980s was recommended by some authors although precise indications for removing these kidneys were unclear.[6] According to CUA update 2014, nephrectomy continues to be suitable treatment option for MCDKs that do not involute, or are unlikely to involute.[7] Currently, the indications for resecting the MCDK are restricted to: (i) very large MCDKs that interfere with respiratory or intestinal function in the neonatal phase; and (ii) that contain solid parts that seem to grow during the follow-up on ultrasonography; and (iii) Hypertension. Using these indications, there was a need for surgery in our case. The respiratory distress in the index case settled after the mass was removed.Source of Support: NilConflict of Interest: Nil


Gastric hepatoid adenocarcinoma (GHAC) is an atypical form of gastric cancer (GC) that has similar tissue morphology to hepatocellular carcinoma and frequently produces alpha-fetoprotein. We present an exceedingly rare case of GHAC resulting in a spontaneous gastric perforation.A 61-year-old man presented at our institution complaining of abdominal and back pain. A computed tomography scan revealed a spontaneous gastric perforation with a solitary liver tumor and lymph node swelling. Following a diagnosis of advanced-stage GC with a gastric perforation, perforative peritonitis, multiple lymph node metastases, and a solitary metastasis of the lateral segment of the liver, the patient underwent distal gastrectomy. Histopathology of the resected specimen revealed that the tumor cells were arranged in a hepatoid pattern. On immunohistochemical staining, the tumor cells were positive for alpha-fetoprotein and Sal-like protein 4. Thus, the patient was diagnosed with GHAC. Hepatic resection of the solitary liver metastasis was performed. However, recurrence occurred and the patient achieved complete response following tegafur/gimeracil/oteracil-based chemotherapy.GHAC is a highly malignant histological subtype of GC. We reported on an extremely rare case of GHAC resulting in a spontaneous gastric perforation and reviewed the literature, including epidemiological data, treatment regimens, and the association between GHAC and alpha-fetoprotein-producing GC. Hepatoid adenocarcinoma is a malignant cancer manifesting outside the liver that most frequently arises in the stomach, with gastric hepatoid adenocarcinoma (GHAC) accounting for 63% of cases. Hepatoid adenocarcinoma also arises in the ovaries 10%), lungs 5%), bladder 4%), pancreas (4%), and uterus (4%) 0%, lungs–4. GHAC %, pancre%, bladdeA 61-year-old man experienced upper abdominal and lower left back pain 1 month and 1 week prior to examination, respectively. He was referred to our hospital after the pain had worsened. The patient experienced spontaneous lumbar and epigastric pain with muscular defense of the epigastrium and accompanying tenderness. Blood test results indicated a white blood cell count of 12,430 /μL, a C-reactive protein level of 0.6 mg/dL, and mild but increasing inflammation. No abnormal findings were reported from the other blood counts, biochemical examinations, and coagulation tests.Abdominal contrast-enhanced computed tomography (CT) revealed disruption and thickening of the anterior wall of the gastric antrum. CT also revealed the presence of ascites and free air at the ventral side of the stomach and on the surface of the liver Fig. . SeveralGross findings of the resected specimen included an ulcerative and infiltrative type 3) tumor (approximately 100 × 50 mm) with an infiltrative serosal surface and 7-mm puncture sites  was recorded 6 weeks after hepatic resection and recurrence of LN metastasis was detected on CT. Combination chemotherapy with tegafur/gimeracil/oteracil (S-1) was administered at a dose of 120 mg/day for 2 weeks with a 1-week rest. Three months later, serum AFP levels had normalized and shrinkage of the resected LN was observed. Elevated serum AFP levels were not detected 15 months post-recurrence and complete response of the LN metastasis was achieved.Boureille et al.  first reIn 1985, Ishikura et al.  introducInagawa et al.  investigGHAC is not associated with specific symptoms and many common symptoms of GC are observed  . In our Histopathologically, GHAC resembles HCC. The tumor cells grow, proliferate, and invade surrounding tissues with significant accompanying venous infiltration , 16. GHAAFP and glypican-3 are oncofetal proteins that are produced by the fetal liver, yolk sac tumors, hepatoblastomas, and HCCs . BecauseAlthough GC resulting in a gastric perforation is rare , theCurrently, a standard treatment regimen for GHAC is lacking and treatments designed for GC are being administered. In instances where GHAC is accompanied by liver metastases, radical resection should be considered, provided the number of liver metastases is limited and liver resection is achievable , 27. HowAlthough the cause is unknown, LN and liver metastases could easily develop given the extent of LN and venous invasion accompanying GHACs. GHACs also progress relatively rapidly. Therefore, GHAC is considered to be highly malignant . Nagai eTreatment for GHAC patients with unresectable metastases, including S-1 monotherapy and paclitaxel/cisplatin-based, oxaliplatin/capecitabine-based, and fluoropyrimidine/platinum-based chemotherapy has been effective in some instances , 30. HowTo improve the prognoses of patients with GHAC and AFP-producing GC, as well as, diagnostic accuracy and incorporation of radical resections, multimodality therapy (including supplementary chemotherapy) and the establishment of a standard chemotherapy regimen are required to address instances of recurrent tumors. Even when radical resections are performed, recurrences and additional metastases frequently occur. Therefore, closer monitoring is needed.We describe a rare case of GHAC resulting in a spontaneous gastric perforation. In our patient, the GHAC was accompanied by multiple LN metastases and a solitary liver metastasis. Distal gastrectomy and lateral liver lobe resection were performed. However, the patient developed postoperative recurrence of LN metastasis. S-1 monotherapy was administered and complete response of the LN metastases was achieved.GHACs progress rapidly and are characterized by a high frequency of LN and liver metastases. Therefore, GHACs are considered highly malignant tumors with poor prognoses. We anticipate that both clinical and basic research will continue to advance with the accumulation of future cases.


Tumor embolisms (TE) are an underappreciated source of pulmonary embolisms in sarcoma. Most evidence in the literature is limited to case reports and none have described the presence of TE secondary to myxofibrosarcoma. We report the first case of myxofibrosarcoma TE and perform a review of the literature for TE secondary to bone and soft tissue sarcomas (STS).A 36-year-old female presented with debilitating pain of the right upper extremity secondary to a recurrent soft tissue sarcoma. She had distant metastasis to the lung. An MRI revealed a 25-cm shoulder mass involving the proximal arm muscles with encasement of the axillary artery, vein, and brachial plexus. A palliative forequarter amputation was performed and tumor thrombus was evident within the axillary artery and vein. Postoperatively, she developed an acute onset of dyspnea and hypoxia. A computed tomography scan revealed a pulmonary saddle embolism. A bilateral lower extremity venous duplex was negative. She became hemodynamically unstable despite resuscitation and was placed on vasopressor support. A transthoracic echocardiogram revealed elevated pulmonary artery pressure, tricuspid regurgitation, right heart dilation, and reduced right heart systolic function consistent with acute cor pulmonale. The patient did not want to pursue a median sternotomy with pulmonary artery embolectomy and expired from cardiopulmonary arrest within 24 h of the operation. The final pathology revealed a 25 × 16 × 13 cm high-grade myxofibrosarcoma with invasion into the bone, skin, and neurovascular bundle as well as evidence of tumor thrombus.TE is a rare but deadly cause of pulmonary embolism in sarcoma. A high index of suspicion is necessary in individuals who present with respiratory-related symptoms, especially dyspnea. Diagnostic confirmation with a computed tomography scan of the chest and echocardiogram should be rapid. Unlike venous thromboembolism, pulmonary embolectomy remains the preferred therapeutic approach.The online version of this article (doi:10.1186/s12957-017-1223-3) contains supplementary material, which is available to authorized users. Sarcomas are solid tumors originating from mesenchymal tissue that account for less than 1% of all adult malignancies , 3. NotaPulmonary embolism (PE) is a major cause of sarcoma-related death. An investigation of 252 individuals with STS and bone sarcomas revealed 1.2% had PE and 0.4% had a fatal PE . Non-venTumor embolism (TE) has been reported in 0.3–26% of patients with a solid malignancy at autopsy and is associated with a mortality rate of 8% , 11. TE A 36-year-old female presented with a recurrent right upper extremity STS. Two years prior to initial presentation at our facility, she was diagnosed with an 11-cm right axillary, intermediate-grade pleomorphic spindle cell neoplasm. She underwent neoadjuvant chemoradiation therapy followed by a resection of the tumor. Microscopic margins were positive following the surgical resection and adjuvant chemotherapy was initiated. She developed a local recurrence 3 months after resection which ultimately progressed to invasion of the brachial plexus with paralysis of the right upper extremity as well as a pathologic humeral fracture. She was placed on low molecular weight heparin after developing a right subclavian vein thrombosis. Additionally, she developed distant metastatic disease in the left upper lobe of the lung. She perused several experimental modalities that were unsuccessful at reducing the tumor burden.She presented to our institution 2 years after the initial diagnosis with debilitating pain of the right upper extremity. An MRI revealed a 25-cm shoulder mass involving the proximal arm muscles with encasement of the axillary artery, vein, and brachial plexus Fig. . After aTen hours after completion of the operation, she developed an acute onset of dyspnea with a resultant increase in her supplemental oxygen requirement. A chest x-ray was obtained which revealed bilateral atelectasis. Suspicion of a pulmonary embolism remained high and confirmatory testing was performed with a computed tomography (CT) chest Fig. . A pulmoThe final pathology of the primary tumor revealed a 25 × 16 × 13 cm high-grade myxofibrosarcoma. The tumor invaded the bone, skin, and neurovascular bundle and contained tumor thrombus.n = 14) or bone sarcoma (n = 31) . Seven patients had a subacute (2–8 weeks) onset of symptoms, and one patient had a chronic (> 8 weeks) duration of symptoms before seeking medical care.Including our case report, 40 publications that met criteria were included in this series. Together, these studies included 45 patients with TE secondary to STS n = 4 or bonen = 7)  followed by pleomorphic, rhabdomyosarcoma, and synovial sarcoma (n = 2 each)  and pelvis (n = 9) were the most common sources of TE in the setting of bone sarcoma  was the most common bone sarcoma while the remainder of cases were comprised of osteosarcoma (n = 14)  Fig. . The mosch) Fig. . Conversoma Fig. . Chrondr 14) Fig .Fig. 3Sin = 36) was the most common modality utilized followed by chest roentgenography (n = 30), lung perfusion scans (n = 16), angiography (n = 10), and transthoracic echocardiography (n = 10) . Additionally, evidence of cor pulmonale was found in 19 cases.The diagnosis of TE was not established until postmortem evaluation in 22.2% of cases. A variety of diagnostic modalities were used. CT scan (10) Fig. . Chest rn = 12), lung resection (n = 7), and chemotherapy alone (n = 6)  of cases and most commonly ensued within 3 months (n = 21) . Death occurred in three of four cases where an IVC filter (n = 3) or IVC plication (n = 1) was performed as the sole management of PE (follow-up duration not specified for the sole survivor). Of those patients that received definitive intervention for TE (n = 25), 6/12 patients with embolectomy survived compared to 4/7 that underwent a lung resection and 4/6 who received chemotherapy alone.Venous duplex of extremity/inferior vena cava (IVC) was performed in 11 cases and diagnostic of a deep vein thrombosis in 5/11 cases. Direct tumor venous invasion at the primary tumor site was noted in eight cases  Fig. . Death o21) Fig. . NotablyIndividuals with TE have a range of clinical findings from asymptomatic to hemodynamic instability resulting in death . Within TE can develop from several mechanisms. Pulmonary artery embolism may occur but lymphatic invasion with mediastinal and pulmonary lymphatic extension is more common . DiffuseThe risks factors underlying TE development are not well characterized . This seWithin the pulmonary artery, TE may resolve, remain latent, or progress . ProgresSimilar to other studies, evidence of cor pulmonale was present in less than 50% of cases within this series . Most paThe diagnosis of TE is challenging and may not be established until post-mortem evaluation as evidenced by 22.2% of cases within this series. CT is considered the gold standard for the diagnosis of TE , 25. A bTE is frequently mistaken for VTE and anticoagulation is improperly initiated. Anticoagulation and thrombolysis are not useful for TE and considered an absolute contraindication as it may result in hemoptysis , 29, 30.Limitations of this study include the limited reports of sarcoma TE. The rarity of TE in sarcoma and underappreciation of TE as a source of PE have led to misdiagnoses. Additionally, several cases did not have sufficient information to make comparisons across all categories that were assessed. Several reports were carried out before the advent and widespread availability of newer technologies . Thus, their merit may not be fully appreciated and the rarity of this phenomenon in conjunction with a multitude of histologic sarcoma subtypes makes research investigations challenging.Sarcomas have an increased propensity for the development of emboli . TE accoAdditional file 1:Clinical data: Presentation and diagnosis. Clinical presentation and diagnosis of cases containing tumor embolism secondary to soft tissue and bone sarcoma. (DOCX 32 kb)Additional file 2:Clinical data: Venous extension and management. Presence of local venous tumor extension and clinical management of cases containing tumor embolism secondary to soft tissue and bone sarcoma. (DOCX 25 kb)


Objective: To report a case of iatrogenic central retinal artery occlusion after embolization and surgical resection of carotid body paraganglioma.Methods: Case report Results: One adult female patient presented with persistent unilateral visual loss after embolization with Embosphere® and Contour® microparticles of carotid body tumor. Fluorescein angiography revealed intraluminal microspheres in the central retinal artery ramifications. OCT revealed intraretinal spherical, hyporeflective particles with posterior shadowing. Conclusions: Central retinal artery occlusion should be assessed as a possible complication after surgical repair of head and neck paragangliomas. It functions as a chemoreceptor to changes in arterial pOpiration . Carotidpiration . The morpiration . Paraganpiration , mainly piration . Duplex piration . We desc® Polyvynil alcohol embolization particles  of 150–250 microns, and then by injection of Embospheres®  of 300–500 microns until vessel occlusion was achieved. The same procedure was performed for the occipital artery. The superior thyroid artery could not be embolized because of severe vessel tortuosity. A post-embolization angiography revealed adequate permeability of intracranial and extracraneal vessels. An average decrease of 80% of the tumor’s blood flow was documented. After the procedure was completed, the patient woke up without neurological or visual deficits. The surgical resection was then performed under general anesthesia. A transverse cervical incision and dissection to complete exposure of the tumor was done. The surrounding vascular structures  with extension to the skull base were identified. The hypoglossal nerve, the hypoglossal loop and vagus nerve, were released without severing. The tumor resection was performed with the subadventitial technique [A 45-year-old female presented with a history of syncope, headache, and dyspnea for three years. A pulsatile mass was discovered on her left side of the neck. CT angiography revealed a mass located on the left carotid bifurcation classified as Shamblin II Figure 1 . PreoperOn the first postoperative day, the patient complained of poor vision in her left eye with no other associated symptoms. Brain MRI was performed to rule out intracerebral ischemia. Ophthalmology examination found no light perception, afferent pupillary defect, and a pale retina with arteriolar thinning and segmentation of macular vessels in the left eye Figure 2 . FluoresIt is well known that paragangliomas may be hereditary and may be part of genetic syndromes. The sporadic form of carotid body paraganglioma is more common than the inherited variety and tends to occur slightly more often in women. Due to the proximity of the tumor to major vessels and nerves, the risk of associated morbidity and mortality is around 3–9% . CT is o® and Contour® microparticles. Due to the late onset of symptoms we believe this was a complication of the surgical procedure per se, in which, the surgical manipulation could have eased the liberation of particles to the circulation. It remains an interrogant as to why the particles embolized directly to the central retinal artery instead of other high-flow intracerebral branches. The patient’s OCT was conclusive on the presence of hyporeflective spherical particles that confirmed the nature of the embolization. Although a rare complication, we think it must be assessed with the patient as one of the possible complications prior to embolization and surgical resection procedures. To our knowledge this is the first report of acute central retinal arterial occlusion secondary to paraganglioma embolization and excision with EmbosphereCentral retinal artery occlusion should be assessed as a possible complication after embolization and surgical repair of head and neck paragangliomas.The authors declare that they have no competing interests.


Sertoli-Leydig Cell Tumors (SLCTs) make up <1% of all ovarian tumors and are benign or malignant, androgen-secreting tumors. Rhabdomyosarcoma (RMS) is a heterogeneous group of malignant tumors that resemble developing skeletal muscle. There have been case reports of patients with concurrent SLCT and RMS with limited treatment options. We aim to demonstrate treatment strategies used in our patients, which seemed to have prolonged survival when compared to prior case reports of patients not cured by surgical resection. Herein we describe 22 cases of SLCT with RMS elements as discussed in prior case reports and three cases from the authors' institution. Of the 19 cases from prior case reports, five were lost to follow-up and two had NED after surgical intervention. Eleven patients had recurrence and were deceased within one year. Of those patients not surgically cured, only three patients were documented as living beyond two years, all of whom received chemotherapy. The three patients presented from our institution had clinical evidence of response to chemotherapy that is traditionally used for RMS. In conclusion, chemotherapy with doxorubicin and ifosfamide has activity in patients with SLCT and RMS as does salvage chemotherapy with vincristine, irinotecan, and temozolomide. There aith SLTC . SLTC imith SLTC , 7. The ith SLTC . The TMNith SLTC . Variousith SLTC . Malignaith SLTC .Rhabdomyosarcoma (RMS) is a heterogeneous group of malignant tumors that resemble developing skeletal muscle –12. ThesThe association between cervical sarcoma botryoides and ovarian SCLT has been described in at least eight patients from prior case reports , 17–22. Herein, we report three cases of patients with ovarian SLCT with RMS components. To our knowledge, there have been cases reported in literature with limited treatment options. We aim to demonstrate treatment strategies used in our patients, which seemed to have prolonged their lives when compared to prior case reports of patients not cured by surgical resection, in order to provide treatment options for clinicians treating patients with these concurrent rare malignancies.Patient 1 was a 32-year-old Caucasian female noted to have a left sided ovarian mass on CT scan after presenting for left lower quadrant abdominal pain. She was status post resection and pathology was consistent with stage Ia poorly differentiated SLCT. Five months later, recurrent pains lead to a trans-vaginal US that showed a right pelvic mass. She underwent radical hysterectomy and debulking procedure that showed pathology consistent with recurrent poorly differentiated SLCT with heterologous RMS. A follow-up PET scan showed uptake bilaterally in the pelvis. A month later, she was started on doxorubicin, ifosfamide, and vincristine. She was hospitalized for a small bowel obstruction with stable metastatic abdominal nodules on CT. She received cycle 2 doxorubicin, ifosfamide, and vincristine while hospitalized and had a palliative PEG placed. Her chemotherapy was changed to ifosfamide and etoposide because of mucositis and palmar-plantar erythrodysesthesia. After a total of 6 cycles of chemotherapy, she was found to be in complete clinical remission by CT. Approximately 10 months later, CT showed a liver lesion and pelvic mass consistent with recurrent poorly differentiated SLCT on repeat biopsy with no RMS elements seen. She was given 3 cycles of bleomycin, etoposide, and cisplatin (BEP). Bleomycin was held for cycles 4 through 8 due to side effects. A follow-up PET showed stable disease. Three months later, she was found to have a left lower quadrant mass consistent with recurrent SLCT at which point radiation therapy was given with good response with only a small amount of residual disease. Eight months later, a biopsy of a liver lesion was consistent with recurrent SLCT with no definitive RMS identified. She underwent further radiation with stable disease for approximately five months before repeat imaging showed marked disease progression with symptoms that lead to hospice care. The time from diagnosis until death was approximately four years.Patient 2 was a 30-year-old, black female with a left sided ovarian mass found incidentally during a cesarean section that was initially called a granulosa tumor on pathology but later deemed SLCT with focal poor differentiation when reviewed. At the time of initial diagnosis she was thought to have stage IA disease that was cured surgically. She was lost to follow-up for 13 months at which time a follow-up CT showed a right sided ovarian mass that increased on serial imaging. Surgery for recurrence revealed a left adnexal mass, left pelvic sidewall, cul-de-sac, and anterior abdominal wall nodules with pathology consistent with high-grade sarcoma ultimately deemed embryonal RMS consistent with recurrence of the poorly differentiated SLCT. Two months later, she was started on doxorubicin, ifosfamide, and vincristine for a total of 6 cycles with follow-up CT showing NED. A repeat CT performed 7 months after chemotherapy completion showed an increasing pelvic mass that was resected. Pathology was consistent with recurrent SLCT with RMS, and a liver mass was discovered during surgery that was not seen on CT scan. She was started on salvage chemotherapy for RMS with vincristine, irinotecan, and temozolomide for a total of 6 cycles. Follow-up CT after cycle 4 showed NED. The patient developed massive progression 5 months after her last cycle of chemotherapy with small bowel obstruction requiring debulking surgery with colostomy placement and ultimately hospice care. The time span from diagnosis until death was approximately 3.5 years.Patient 3 was a 23-year-old female who presented with nausea and vomiting with an US showing a right ovarian mass. She underwent exploratory laparotomy with debulking and removal of a large abdominal pelvic mass with a right salpingo-oophorectomy (RSO) and appendectomy. Pathology was consistent with stage IA poorly differentiated SLCT with some elements of RMS, and she was found to have a DICER1 mutation. She underwent 6 cycles of doxorubicin, ifosfamide, and vincristine and is currently NED on follow-up imaging approximately 10 months after chemotherapy was initiated.In one case report, a patient with SLCT of intermediate differentiation and RMS of FIGO stage 1C was treated with surgery alone and remained with no evidence of disease (NED) at four years after surgery . These aAnother case report describes a patient who after a unilateral oophorectomy was found to have an SLCT with focal rhabdomyoblastic differentiation, and she developed an omental mass with features consistent with high-grade sarcoma one year later. She underwent further surgery with a plan for adjuvant chemotherapy but was lost to follow-up . ChougulZaloudek and Norris described a 16-year-old patient with stage Ia poorly differentiated SLCT with RMS elements who was treated with USO and later thiotepa and 5-fluorouracil after recurrence at 1.4 years. She was found to have peritoneal metastases on autopsy after her death four months later .The most recent case report of SLCT with RMS elements describes a 70-year-old patient who was diagnosed with stage Ia disease and underwent hysterectomy with left salpingo-oophorectomy (LSO), appendectomy, and omentectomy without evidence of macroscopic disease after the procedures. At 7 months postoperatively, she represented with a large abdominal mass, ascites, unilateral hydronephrosis, and a massive pulmonary embolism. She was treated palliatively and died 15 days later without subsequent autopsy performance .The largest number of case reports was presented by Prat et al. who document nine cases of SLCT with skeletal muscle elements. With one exception, all of these subjects were initially treated with surgery alone. Of those with available information, all subjects had recurrence within one year of initial treatment. Most subjects had recurrence surgically treated and most were deceased within one year. For full information on these subjects, see Discussion. Herein we describe 19 cases of SLCT with RMS elements as discussed in prior case reports as well as three cases from the authors' institution. Of these 19 cases, five were lost to follow-up and two were documented as being NED after surgical intervention alone. Of the remaining cases, eleven patients had recurrence within one year and five of these patients were deceased within that year. Of those patients not surgically cured, only three patients were documented as living beyond two years, and all three of these patients received chemotherapy.In patients with RMS, chemotherapy regimens considered in recurrent disease include vincristine, doxorubicin, and cyclophosphamide; ifosfamide and etoposide; and vincristine, irinotecan, and temozolomide , 33. Sta


Oligodendrogliomas are usually located in the frontal, parietal and the temporal lobe, with the ones in the fourth ventricle quite rare. Hence we want to introduce a case about the rare disease.An eight-year old boy complained of progressive headache, dizziness and vomit for about 2 months. Then the slight ataxia was found by the physical examinations, with no sensory disturbances and other motor disturbances.Abnormal signals on the fourth ventricle were found by the preoperative brain computed tomography (CT) scan and magnetic resonance imaging (MRI) scan. So the patient accepted a gross total resection of the lesion with pathologically confirmed oligodendroglioma.Radiotherapy was then delivered in 27 fractions at 2Gy per fraction after the operation, with one fraction daily for five days weekly. No other therapies were used for the patient.The brain MRI was used for follow-up every three months until now when he has finished all therapies for more than one year. No progressive behaviors  or images have been presented. And the follow-up will be continued.Although oligodendrogliomas are usually located in the frontal lobe, with the ones of fourth ventricle extremely rare, they must be kept in mind all times. Treatments applied to our case may be provided as a reference for clinicians. Furthermore, the maximal range of resection, histologically proved oligodendroglioma and the 1p/19q loss of heterozygosity are associated with favorable prognosis. They are usually located in or under cerebral cortex, with most of them found in the frontal, parietal and temporal lobe. But primary oligodendroglioma in the ventricle, especially in the fourth ventricle, is quite rare. Herein we would like to introduce a case about the rare disease based on clinical symptoms, treatment, and prognosis.Oligodendroglioma derives from the oligodendrocyte. It accounts for approximately 1.3% to 4.4% of the brain tumor and about 5% to 10% of the gliocytoma.2An 8-year-old boy complained of progressive headache, dizziness and vomit for about 2 months. Then the slight ataxia was found by the physical examinations, with no sensory disturbances and other motor disturbances. And he has no history of seizure or other illnesses.b = 1000)  scan showed that there was a cystic or solid lump with plaque calcification in the fourth ventricle Fig. . The bra00) Fig. D. Mildly00) Fig. E. In addRequired by his parents, the boy accepted the external ventricular drainage and brain tumor exploratory resection. The range of the operation has reached to the point of gross total resection. Operatively, it was easy to see that the tumor was soft with rich vascular circulation, closely adhering to the floor of the fourth ventricle and extending down to the vermis. The postoperative MRI displayed that structures of surgical area were disordered and partial occipital bone was incontinuous. Moreover, the cerebellar tonsillar was 0.5 cm below the foramen magnum, which signified slight cerebellar tonsillar hernia Fig. .The histological examination  of the surgical specimen Fig. A revealeIn consideration of characteristics of the low-grade gliocytoma and the patient's age, radiotherapy alone was given as the adjuvant therapy after the operation. Intensity modified radiotherapy (IMRT) targeted on the surgical regions was delivered in 27 fractions at 2 Gy per fraction. And the patient was treated with one fraction daily for five days weekly. During the radiation, diplopia and blurred vision occurred. But the symptoms relieved apparently after the utilization of mannitol and dexamethasone. Then brain MRI was used for follow-up every 3 months until now when he has finished all therapies for more than one year. No progressive behaviors  or images have been presented. And the follow-up will be continued.The parents of the patient have consented for the publication of the case report.3 Only 7% among them are found in the posterior fossa, with one-quarter found in the fourth ventricle,,4 to say nothing of the percentage of those happened in children and adolescent. In 1969, Greenwood et al reported 2 cases of adult oligodendrogliomas in the fourth ventricle. Then only another case about a 16-year-old boy was reported by Pitt in 1992.The overwhelming proportion of primary oligodendrogliomas are located in the cerebral hemisphere, with mostly in the frontal lobes.,5 Similarly, examinations were performed due to the patient's progressive headache and vomit in our case. Generally, seizure is common with oligodendroglial histology, and it is because the cortex is invaded. No signals of seizure were observed in our case. And obstructive hydrocephalus was verified by the preoperative MRI. Moreover, the postoperative MRI also displayed slight cerebellar tonsillar hernia, mostly for the reason of high intracranial pressure.Anatomically, the fourth ventricle lies between the cerebellum and pons, medulla oblongata. It is connected with mesencephalic aqueduct above and cavum subarachnoidale below through the median aperture and 2 lateral apertures of the fourth ventricle. Therefore, tumors growing in the fourth ventricle can affect the flow of cerebrospinal fluid. Further, high intracranial pressure  and hydrocephaly are common initial symptoms. They can be attributed to the location. Patients in previous cases showed headache, nausea or blurring of vision, and even blindness. reporting their research on the comparison between surgery and biopsy, surgical resection has become the chief choice for LGGs. For those located in nonfunctional areas, the operation principle of maximum range is of consensus. In addition, the extent of resection is correlated significantly with overall survival (OS).,8 As tumors growing in the fourth ventricle, a nonfunctional area, partial resection may be relatively easy, but total resection in this region is hard to be realized. It is perhaps followed by secondary changes in adjoining neurological structures, which may be disabled and will not recover. Operation on the boy in our case has reached the principle of maximum resection, a total removal. Although the cerebellar tonsillar hernia after surgery is difficult to relieve, it only need to be observed intently and prevented from progression.Oligodendroglioma of the fourth ventricle belongs to the low-grade  gliocytoma (LGG). It is a very benign lesion, particularly when symptoms arise after the age of 21. After Jakola et al So the long-term surveillance imaging is required. Once radiation is elected, a dose of 50 to 54 Gy in 1.8 to 2 Gy daily fraction over 5 to 6 weeks has become standard, with the targets delineated by the tumor area and margin. Significant tumor shrinkage happens to approximately 30% of patients. A random trial (EORTC 22845) has showed that early radiation (54 Gy) could increase the progress-free survival (PFS) rate and decrease the incidence of epilepsy significantly (P < .01), but no statistics difference in OS rate was observed. In addition, much of decline in cognitive status has been attributed to radiation therapy. But recent studies have proposed that most cognitive decline in the first few years after radiation is blamed on the tumor characteristic or concomitant medications, such as anticonvulsants. In our case, the lesion is located in a nonfunctional area, less cognitive behavior may appear. Although no reports about it have been showed, the boy was performed with early conventional radiotherapy with a dose of 54 Gy at 2 Gy per once-daily fraction. Apart from diplopia and blurred vision during radiation, which was relieved by mannitol and dexamethasone, no typical decline in cognitive status has been observed till now. Furthermore, the symptom of ataxia was relieved slowly with the radiation going on.Whether patients with an excellent resection need additional treatment is one of the most contentious areas in neuro-oncology. A prospective study found that more than 50% of patients younger than 40 with gross total resection of LGG recurred within 5 years after operation. It included newly diagnosed patients with LGGs over age 40 or performed with a subtotal resection. With the emergency of temozolomide (TMZ), a second generation of alkylating agent with low molecular mass, attention has been shifted to this agent. It is a drug of excellent oral bioavailability with plasma concentration up to the peak in one hour and easy to penetrate the blood brain barrier. Whether TMZ can replace radiotherapy and the therapeutic effect combining TMZ with radiotherapy are now research focuses. The RTOG 0424 trial, a single-arm phase II study, has recently reported that for the patients with TMZ and radiotherapy with 3 or more recurrence-related risk factors , the 3-year OS rate is higher than that of radiation alone controls. Whether the friendly TMZ can replace PCV remains unknown. A prospective randomized trial may be required to address. Because concurrent chemoradiotherapy has never been reported in patients with total resection, TMZ was not taken orally in our case. Meanwhile, it was not applicable when considering the age of the boy. All examinations demonstrated stable disease till now.The effect of chemotherapy in patients with LGGs has not been clear until the results of Radiation Therapy Oncology Group (RTOG) 9802 became available. The RTOG 9802 phase III randomized trial demonstrated an increase in PFS rate and a categorical OS benefit (13.3 years vs 7.8 years) for the group of RT armed with PCV compared to those of RT alone.P = .0027) and PFS (P = .0339). In addition, the prognosis of oligodendroglomas is better than that of the same grade of astrocytomas, and gliomas containing oligodendrocytes are also better in prognosis. Furthermore, the proportion of low-grade oligodendrogliomas turned into high-grade ones (grade III or IV) is about 45% while astrocytomas 74% and oligoastrocytomas 70%, and the median survival time of oliogodendrogliomas is about 8.8 years while astrocytomas 3.1 years and oligoastrocytomas 4.4 years. Meanwhile, the 1p/19q loss of heterozygosity, as the most commonly biomolecular change, is also rewarding for the prognosis of oligodendrogliomas with the incidence about 70%.,18 Although there was no test for the heterozygote of the 1p/19q loss in this case, with other favorable factors, presumably the boy will be with a favorable prognosis, and follow-up need be continued.Besides of the range of resection mentioned above, some factors are also connected with prognosis. The RTOG 0424 trial showed that among the risk factors mentioned above only histology was significantly associated with OS (4Although oligodendrogliomas are usually located in the frontal lobe, with the ones of fourth ventricle extremely rare, they must be kept in mind all times. No reports about them have ever been displayed, but researches on treatments are necessary to focus on. As known from our case, surgery with the maximum range is indispensable. Although additional treatments for patients with an excellent resection have been unresolved, conventional radiotherapy with a dose of 54 Gy at 2 Gy per once-daily fraction without chemotherapy could been considered. Maybe it can be considered as a reference for clinicians. But follow-up and further studies will be required to expand the reach of our observations. In terms of prognosis, the maximal range of resection, histologically proved oligodendroglioma and the 1p/19q loss of heterozygosity are associated with favorable prognosis.Investigation: Shaomei Li.Writing – original draft: Xiujuan Gai.Writing – review & editing: Yumei Wei, Shuhua Yu.


We report an unprecedented case of ectopic thymoma in a young adult. A33-year-old male presented with a 10-day history of non-productive cough andfever. Investigation revealed mediastinal widening without pulmonaryinvolvement. Computed tomography showed a large mass-14.8 × 10.8 ×8.4 cm-in the mid-posterior mediastinum, and a biopsy obtained by video-assistedthoracoscopy indicated that the mass was a tumor. Immunohistochemistry showedcombined thymoma type AB1. Because of the considerable proportions of the tumorand its close proximity to major structures, the patient was treated withchemotherapy. Theinvolvement of middle and posterior mediastinum is rare, only 16 cases of thymoma inthe middle mediastinum having been reported-10. The incidence ofthymoma peaks between 50 and 60 years of age. Here, we describe the challenge ofdiagnosing this rare neoplasm in a mildly symptomatic young adult.Although thymoma is the most common primary tumor of the anterior mediastinum, itaccounts for less than 1% of all neoplasms in adults3); and leukocytosiswithout a shift . The patient evolved todaily fever spikes and tachycardia. A chest X-ray revealed mediastinal widening; inversionof the albumin/globulin ratio (A/G ratio = 0.65); a C-reactive protein level of 33.1mg/dL; thrombocytosis  or tothe middle mediastinum. Most patients areasymptomatic, being diagnosed on the basis of incidental findings on imagingstudies. Approximately 40% of symptomatic patients present with myasthenia gravis,the paraneoplastic syndrome most often associated with thymoma,9. Our patient reported having a normal diet and showing no signsof dysphagia.The annual incidence of thymoma is 0.15 cases/100,000 population, with no differencebetween genders, and its incidence increases in adulthood, peaking at 50-60 years ofage. The most widely used staging system is that devised byMasaoka et al., which involvespostoperative pathological evaluation of capsular invasion of the thymus.Thymomas originate from the epithelial cells of the thymus. Histologically, thethymus has two regions: the cortex, which is rich in lymphocytes, and the medulla,which is composed of epithelial cells. The histological classification is based onthe morphology of the epithelial cells and the proportional relationship betweenthose cells and lymphocytes. Most of the thymomas that are determined to be type A,AB, or B1 have a benign course. Thymoma types B2 and B3 are considered to bemalignant, with metastatic potential,15.Because the tumor could not be resected, radiology played a key role in the casepresented here. The initial diagnosis and staging were based on imaging studies,with an emphasis on the detection of locoregional or distant invasion. Approximately45-80% of thymomas are visible on conventional chest X-rays, which initiated thediagnostic investigation in our patient. For the evaluation of mediastinal masses,CT is the tool of choice,16.Complete surgical resection is the main therapy for invasive and noninvasivethymomas, being the most important predictor of long-term survival. However,radiation therapy and chemotherapy, alone or in combination, produce favorableresults, increasing survival and improving the prognosisThymoma can mimic a variety of diseases, including those with compressive symptomsand paraneoplastic diseases, as well as mediastinal widening. Hence the importanceof this case-to expand diagnostic reasoning for a tumor in the middle mediastinum,because it is a rare differential diagnosis to be considered, especially in youngpatients.