Cancer of unknown primary is associated with unknown biology and dismal prognosis. The most common primary sites of cancer of unknown primary were usually the lungs in autopsy studies, and intrahepatic cholangiocarcinoma is rare. We describe the case of a 57-year-old male patient with systemic lymph node metastasis. Imaging examination failed to reveal primary cancer; however, immunostaining of cytokeratins 7, 19, and 20 of a metastatic axillary lymph node suggested a pancreaticobiliary cancer as a primary lesion. He died of liver abscess and sepsis, and then, autopsy indicated occult intrahepatic cholangiocarcinoma. We discuss the clinical course of this rare cholangiocarcinoma including the diagnostic procedure and also present a review of the English literature regarding patients with cancer of unknown primary. Carcinomas of unknown primary (CUP) represent a group of heterogeneous tumors that has no identifiable origin . DespiteIn this report, we describe the case of a 59-year-old male patient with CUP. The patient was diagnosed with occult intrahepatic cholangiocarcinoma by autopsy. We present a review of the English literature regarding patients with cancer of unknown primary and discuss the clinical course and diagnostic examination for this occult cholangiocarcinoma case.A 57-year-old male was investigated because of elevation of tumor markers (carcinoembryonic antigen (CEA) 12.9 mg/ml, carbohydrate antigen 19-9 (CA19-9) 658.5 U/ml). Enhanced computed tomography (CT) Fig.  and PET-Autopsy was performed to find the primary lesion. Macroscopically, a gray-white colored, ill-defined solid tumor in the lateral segment of the liver was found, invading the diaphragm Fig. . PatholoWe reviewed recent English literature regarding patients with CUP –12 (TablThe reason why we failed to detect this intrahepatic cholangiocarcinoma using many imaging modalities is considered as follows. Because cardiac pulsation can interfere with diagnostic imaging, it may be difficult to detect the solid tumor in the subphrenic area of the lateral segment of the liver. This area should be considered as one of the blind spots of imaging examination. There were no abnormal findings which could indicate the existence of the cancer lesion from the retrospective viewpoints. If an exploratory laparoscopy was performed, we might have found this intrahepatic cholangiocarcinoma. The result of the immunohistochemistry of the axillary lymph node was accurate in this case, so the treatment choice of chemotherapy with gemcitabine and cisplatin was adequate.Despite advances in diagnostic imaging technology, identifying the primary sites in patients with metastatic malignancies is sometimes difficult even now. In the presented case, immunohistochemistry was accurate and useful, and exploratory laparoscopy may play a significant role to detect the primary lesion. Thus, various examinations should be performed for CUP patients to receive sufficient treatment.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Primary adenocarcinoma of the upper urinary tract, particularly of the ureter, is an extremely rare entity. We are reporting on the first case of metachronous appearance in one patient. The 71-year-old man underwent partial ureterectomy (R0 resection) for primary adenocarcinoma of the left distal ureter. 3 years later, nephroureterectomy had to be performed because of metachronous primary adenocarcinoma of the left proximal ureter. Extensive examinations revealed no evidence for further malignancies at both times. Primary adenocarcinoma of the upper urinary tract is rare but should be kept in mind, especially in patients with chronic inflammation and urinary tract obstruction. Due to the low incidence, there is a lack of data regarding its pathogenesis, diagnosis, and optimal treatment. Malignancies of the upper urinary tract are rare. Approximately, 90% of them are transitional cell carcinomas (TCC); squamous cell carcinomas account for less than 10%. Primary adenocarcinomas of the upper urinary tract are extremely rare, representing less than 1% of all tumors [Adenocarcinomas of the urinary tract are subdivided into tubulovillous, mucinous, and papillary nonintestinal categories. Tubulovillous and mucinous tumors represent intestinal adenocarcinomas and constitute 93% of the cases , 2.Mucinous adenocarcinomas are presumed to be originating from intestinal metaplasia of the transitional epithelium and to have a better prognosis, while the papillary nonintestinal variety occurs in younger individuals and is not necessarily associated with inflammation .However, there seems to be a frequent association of upper urinary tract primary adenocarcinoma with chronic irritation, inflammation, infection, hydronephrosis, and urinary calculi. Thus, glandular metaplasia of the urothelium due to injury or inflammation might eventually progress to dysplasia and adenocarcinoma , 4. A noThe 71-year-old was referred to our clinic 3 years ago after an incidental finding of asymptomatic left sided hydronephrosis. None of the above-mentioned risk factors  could be found in the reported case. Moreover, the patient had no smoking history, neither a family history of cancer. While cystoscopy showed no visible tumor, retrograde ureteropyelography demonstrated ureteral stenosis, 4 cm, proximal to the ureteral orifice, not accessible for biopsy. Selective ureteral cytological evaluation of urine indicated high-grade TCC. Staging was performed by thoracoabdominal computed tomography (CT) and revealed localized disease. As the patient refused radical nephroureterectomy, partial ureterectomy with ureteroneocystostomy was performed. Histology disclosed a 10 × 9 × 7 mm lesion of an adenocarcinoma pT3, pN0 (0/11), G2, R0 . ColonosFollowup controls were scheduled on a regular basis (3 and 6 months after operation and every 6 months afterwards) by CT, cystoscopy, and cytology without signs of recurrence. After 2 years, CT scan showed a ureteral wall thickening with enhancement of contrast medium . The patAs before, the lesion was not accessible for biopsy and cytological examination showed atypical cells interpreted as high grade TCC. This time the patient underwent radical nephroureterectomy for pT3, NX, G2, R0 adenocarcinoma of the left proximal ureter  with absFDG-PET/CT and colonoscopy showed no evidence for another primary tumor or metastatic disease and elevation of serum CEA could be excluded. At followup, performed by CT, cystoscopy, and cytology after 6 and 12 weeks and after 6 and 12 months, so far, no signs of recurrence occurred.Primary adenocarcinoma of the upper urinary tract is a rare tumor entity. However, it should be kept in mind, especially in patients with urinary calculi accompanied by chronic inflammation and urinary tract obstruction. In most cases precise diagnosis is not made before surgery, because surgery is often performed for urinary calculi or chronic infection and there is a low concordance of prior cytology subclassification results , 8. Whil


Introduction. Inguinal hernia is a common disorder with an estimated prevalence of 1.2% of the entire population and it is 12 times more common in males. Objective. To describe a case of appendix with signs of inflammation in the hernia sac, condition that is rare and difficult to diagnose, and to perform literature review, describing the most relevant aspects and the main controversies. Method. Report of a case and search in PubMed on June 1, 2015, using the terms “Appendix” [MeSH term] AND “hernia, inguinal” [MeSH term]. Results. The search resulted in 38 articles in total, and after deleting the articles that were not part of the inclusion criteria, there were 26 case reports remaining. Discussion. The search resulted in a total of 38 articles and after deleting the articles that were not part of the inclusion criteria, there were 26 case reports remaining. Conclusion. Amyand's hernia is a rare and difficult to diagnose condition, being commonly found occasionally in surgical procedures. It should be remembered in the presence of cases of incarcerated hernia, due to its possible complications if not diagnosed. Inguinal hernia is a frequent pathology with estimated prevalence of 1.2% of the population, with an incidence 12 times greater in the male gender. It affects both adults and children and the contents of the hernia sac may vary from epiplo, bowel, ovary, and appendix  AND “hernia, inguinal” [MeSH term]. Review articles and articles that reported cases in children were excluded, plus the editorials and images. The inguinal hernia case reports in adults that contemplate the search terms and were published in English were included.Results. The search resulted in 38 articles in total, and after deleting the articles that were not part of the inclusion criteria, there were 26 case reports remaining  days. At the physical examination a mass in the right inguinal region that increased with physical exertion has been found, irreducible to digital maneuvers with the presence of inflammatory signs. Additional tests were requested which demonstrated infectious white blood cells with 12,000 leukocytes, fasting glucose, urea, and creatinine within normal values. The patient underwent surgical treatment under the diagnosis of strangulated right inguinal hernia. Exploratory laparotomy was performed using an oblique incision in the right inguinal region. At the access of the inguinal canal, there was the existence of a hernia sac beside the deep epigastric vessels, which was part of the vermiform appendix. This was found with an increased size, signs of inflammation, infection, and necrosis of its apex, thus showing a necrotic appendicitis. Therefore, this case is the association of Amyand's hernia with an infectious and necrotic process of the appendix. An appendectomy and inguinal hernia surgery using the Shouldice technique were performed. The patient received ciprofloxacin and metronidazole upon the anesthetic induction and remained with this scheme during hospitalization, which lasted three days. Patient recovered well and was discharged on the third day after surgery with the prescription of the above-mentioned antibiotics for another 4 days at home. At the end there was a complete resolution of the case.Amyand's hernia is a rare condition where the vermiform appendix is part of the hernia content and may eventually evolve as appendicitis of difficult diagnosis. Until 1937 there were a total of 228 cases described in the literature .The search resulted in 38 articles in total, and after deleting the articles that were not part of the inclusion criteria, 26 case reports were remaining . UltrasoAs the diagnosis is difficult at the beginning of the clinical condition, the presence of complications is common, often evolving to perforation and peritonitis, which along with older age are the main factors determining the prognosis . In the The treatment comes down basically to the resolution of complications. When the appendicitis is found in Amyand's hernia, the performance of an appendectomy is virtually wise. However, some authors are reluctant when the appendix shows no signs of inflammation, while others suggest the realization of prophylactic appendectomy, citing the possibility of recurrence of hernia and the difficulty of later diagnosis of complications –35. FindAnother common question is about the use of Marlex screen. Some suggest that its use increases the chances of surgical wound infection , 37. HowThe differential diagnosis should be done with orchitis, Richter's hernia, and crural hernia. There are reports in the literature of Amyand's hernia associated with Richter's hernia evolving with appendicitis .Amyand's hernia is a rare and difficult to diagnose condition, being commonly occasionally found in surgical procedure. It should be remembered in the presence of cases of incarcerated hernias, due to its possible complications if not diagnosed. Ultrasound or CT scan can help in the diagnosis and differentiate an incarcerated reducible inguinal hernia from Amyand's hernia evolving with appendicitis.


Lumbo-costo-vertebral syndrome (LCVS) is a set of rare abnormalities involving vertebral bodies, ribs, and abdominal wall. We present a case of LCVS in a 2-year-old girl who had a progressive swelling over left lumbar area noted for the last 12 months. Clinical examination revealed a reducible swelling with positive cough impulse. Ultrasonography showed a defect containing bowel loops in the left lumbar region. Chest x-ray showed scoliosis and hemivertebrae with absent lower ribs on left side. Meshplasty was done. LCVS consists of abnormal vertebral bodies with abdominal musculature wall defects. Most commonly they appear through superior lumbar triangle of Grynfeltt, and rarely through inferior lumbar triangle of Petit. It may be present at birth or noticed in older age groups [1]. Lower backache is the most common symptom although small hernias may be asymptomatic or present with avisible swelling which becomes more prominent during crying. We herein report a case of LCVS on account of its rarity.A 2-year-old girl presented with a swelling in left lumbar region noticed by parents one year back. The swelling gradually increased in size. It use to become more prominent on crying. On examination, there was a globular painless swelling (5cm x8cm)which was soft, reducible, and present below the left costal margin lateral to the dorso-lumbar spine . X-rays of the chest showed D8-L1hemivertebrae, scoliosis with convexity to the right and absent 10th, 11th, and 12th ribs .Ultrasound abdomen showed a hernial defect in the left lumbar region measuring 4.5cm x 2.9cmwith presence of bowel loops. Operative findings showed hernial sac containing small intestines with absent lower ribs. Hernial sac reduced and mesh was placed in the defect. Postoperative recovery was uneventful. At 1-year follow-up, there was no recurrence.LCVS was first described by Touloukian in 1972; he believed that these defects are due to a single somatic defect occurring during 3-5 weeks of gestational age, resulting in defective formation of vertebral bodies, ribs, and abdominal wall musculature [2]. Small hernias may remain asymptomatic and rarely present as acute bowel obstruction.[3]Computed tomography (CT) or magnetic resonance imaging (MRI) are investigations of choice to confirm the diagnosis, know hernia contents, and muscular trophies [4]. Contents of hernial sac are usually small or large intestine, mesentery, omentum, appendix, caecum, stomach, ovary, spleen or kidney rarely [1]. In our case, the hernial sac content was small bowel. The goal of repair is to close the defect and built a strong and elastic abdominal wall. Wide variety of materials has been used to close the defect [1]. The simple tissue approximation-closure can be sufficient for small defects while larger defect may need meshplasty as done in the index case. The surgical correction should be done early when the size of the defect is small. Similarly in our patient, the parents reported that the defect had progressively increased in diameter over a year. The defect can be repaired either by open surgery or laparoscopic methods [5].Source of Support: NilConflict of Interest: None declared


Background. Classification of hemangiopericytoma (HPC) has evolved to a mesenchymal, nonmeningothelial grade two or three neoplasm according to the World Health Organization; however its blood supply has always been defined by dual origin, pial and dural contribution. Case Description. We present the case of a patient with an intracranial HPC with only pial vascular supply. Angiography confirmed the lack of dural supply to this bihemispheric intracranial mass. Subsequent histologic examination confirmed the diagnosis of hemangiopericytoma. Angiographic evidence here is atypical of the natural history of hemangiopericytomas with dual vascular supply and was critical in the decision-making towards surgical resection without tumor embolization. Conclusion. Data presented suggests the lack of dural vascular supply alone does not rule out the diagnosis of hemangiopericytoma.  In 1942, Stout and Murray . The natOriginally thought to be a variant of meningioma, HPC only recently has been delineated as its own histological entity and is now classified as mesenchymal and nonmeningothelial . HPC is A 35-year-old right handed male presented with two months of progressively worsening headaches and otherwise unremarkable history. Neurological examination revealed no focal deficits. His evaluation included a negative metastatic workup, a CT scan showing a hyperdense, bifrontal, parasagittal lesion with surrounding vasogenic edema causing significant mass effect  without Cerebral angiography demonstrated only pial blood supply. The arterial and capillary phase from the left internal carotid angiogram demonstrated a number of small “corkscrew” vessels originating from the distal left callosomarginal artery, with no obvious contribution from either anterior falcine artery, as expected from a parasagittal-presumably dural based lesion. The right carotid injection demonstrated a deviated distal ACA territory but no pial supply to the tumor blush . SelectiThe patient underwent a gross total resection of the mass. At surgery, there was no evidence of hypertrophy of any meningeal vessels on both the inner and outer surfaces of the dura which appeared completely normal in color and character. The tumor was subpial and presented as a dark reddish-purplish hypervascular mass. The tumor clearly invaded the inferior aspect of the falx with extension under the free edge toward the contralateral side. After resection of the bilateral tumor, the inferior half of the falx in the region of the mass was resected in totality. Postoperatively, the patient woke up without neurological deficit and progressed with a benign course throughout his inpatient hospital stay. He was discharged home on postoperative day four.Gross examination of the tumor specimen showed a fleshy, multilobulated tumor that was distinguishable from normal brain but almost entirely encapsulated within the parenchyma. The tumor appeared to be growing around the falx cerebri with one area of invasion.Histological examination revealed a malignant neoplasm with marked hypercellularity and nuclear atypia, hypervascularity, necrosis, and intratumoral hemorrhage consistent with a WHO 2007 grade III anaplastic hemangiopericytoma [Hemangiopericytomas present rarely as intracranial tumors and little is known regarding their pathogenesis and development. They are usually low grade malignant tumors that have a tendency to continue growing, even after gross total resection, and can metastasize to numerous extracranial locations , 10. A gThis is the second case of a purely pial based HPC. Knowledge of the anatomical variability of the blood supply is essential to the successful endovascular and surgical treatment of these tumors. Preoperative angiography is mandatory. Evidence presented here further suggests that a lack of dural vascular supply does not exclude HPC diagnosis.


Intrascrotal lipoblastoma is a rare pediatric benign soft tissue neoplasm, and only 11 cases have been reported. The accurate preoperative diagnosis is difficult because of its rarelity and the similarity with the other soft tissue tumors. Among them, accurate preoperative diagnosis had been made in only one case. Thus, almost all of the cases had required inguinal mass excision (and orchidectomy in one case). In this paper, we discuss the accurate preoperative diagnosis of intrascrotal lipoblastoma and subsequent simple tumorectomy via minimal invasive scrotal skin incision, in 1-year-old boy. On physical examination, intrascrotal extra-testicular lobulated mass was palpated on the right scrotum. An ultrasonography revealed the well-circumscribed, iso-echoic, scant blood-flow, and lobulated tumors with each lobules of 1 to 4 cm in diameter, and the tumor located outside of the tunica vaginalis testis. The serum values of alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (b-hCG) were within normal limit. The preoperative diagnosis of intrascrotal lipoblastoma was made, and the mass was excised via minimal scrotal incision. The right testicle and epididymis were normal. The lesion consisted of the distinct two lobulated tumors, and microscopic examination confirmed the diagnosis of intrascrotal lipoblastoma. The postoperative course was uneventful without evidence of recurrence. A rare intrascrotal lipoblastoma is seldom made accurate preoperative diagnosis; however, the accurate preoperative suspicion of this tumor leads to the minimal invasive tumorectomy via scrotal skin incision and favorable postoperative recovery without recurrence. In the first two decades of life, adipose tumors are relatively rare, comprising about 6 % of soft tissue neoplasms. About 60 % of these are simple lipomas or variants, and up to 30 % are lipoblastomas . It is fA 1-year and 7-month-old boy was taken to his pediatrician after his family members noticed a right swollen scrotum. An intrascrotal tumor was suspected, and he was referred to our pediatric surgery department. On physical examination, a 7-cm lobulated mass was palpated on the right scrotum, apart from the normal bilateral testes Fig. , b. The Microscopically, both tumors were multilobulated tumors of adipose tissue and were surrounded by the capsule composed of loose connective tissue. Lobules were separated by fibrous septa and were composed of mature adipocytes and vacuolated lipoblasts showing varying degrees of differentiation. Both tumors were diagnosed as lipoblastoma Fig. .Fig. 5PaThe postoperative course was uneventful, and the patient discharged home on postoperative day 1. The patient has been followed up for 8 months, without evidence of recurrence.Lipoblastoma, a relatively rare tumor of embryonal fat, is characterized by its benign nature, early presentation 90 % <3 years) , male pr years , Regarding the surgical treatment, malignant lesions require the gold-standard surgical approach of radical inguinal orchidectomy . On the A rare intrascrotal lipoblastoma is seldom made accurate preoperative diagnosis; however, the accurate preoperative suspicion of this tumor leads to the minimal invasive tumorectomy via scrotal skin incision and favorable postoperative recovery without recurrence.Written informed consent was obtained from the parents of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


One year later, the patient remained symptom-free and direct examination of the larynx showed no signs of recurrence. The aim of the present study is to highlight this rare disease and its management options to the otolaryngological community.Laryngeal schwannoma is a rare benign tumor of the larynx, with a high recurrence rate. The present study reports the case of a 36-year-old male patient with a two-week history of hoarseness. Electronic laryngoscopy revealed that there was a submucosal mass at the level of the right supraglottic area. A computed tomography scan of the larynx showed an 8×11-mm expansile mass in the right supraglottic area. Histopathological examination diagnosed a schwannoma of the larynx. The tumor was removed by CO According to the patient, other symptoms, such as a sore throat, globus sensation and difficulty in swallowing and breathing, were not present. Electronic laryngoscopy showed a submucosal mass at the level of the right supraglottic area . A compu2 laser through an endoscopic transoral approach under general anesthesia. During the surgery, the tumor was completely resected along the tumor boundary. No nerves were connected caudally to the tumor. Histopathological examination of the frozen section demonstrated that the tumor was mainly composed of active spindle cells. Next, ~2 mm of tissue was resected along the edge of the tumor by CO2 laser. Histopathological examination subsequent to the surgery demonstrated benign spindle cell lesions (Resection of the tumor was performed by CO lesions . Immunoh lesions , but neg lesions .Schwannomas are slow-growing, benign tumors of the nerve sheath, first described by Verocay in 1908 . A studyThe majority of laryngeal schwannomas occur in the supraglottic area and are present at any age. The etiology of schwannomas is not well understood. Surgical excision is the main strategy for treatment. Furthermore, the preservation of laryngeal function and protection of the laryngeal mucosa from injury during surgery must also be guaranteed. However, recurrences have been reported by long-term follow-up subsequent to conventional surgery .2 lasers are widely used to remove thin layers from the surface of the mucosa without undermining the deeper layers. This procedure can be performed with fairly little bleeding, swelling, pain or scarring. The lasers are more precise than scalpels and the high temperature generated by the lasers aids in cleaning the edges of the body tissue that it is cutting, reducing the risk of infection and recurrence. Using this approach, the surgery time may be reduced and the healing time may be shortened. CO2 lasers now play an increasingly important role as a minimally invasive alternative to conventional surgical interventions for patients in a number of oncology services (COservices .2 laser via a transoral approach can be applied in patients with early-stage laryngeal carcinoma and atlanto-axial vertebral chronic dislocation (2 laser, demonstrating that safe removal of the tumor is possible using this approach, without severe injury to the laryngeal mucosa and with low recurrence.Although differing surgical approaches for removing laryngeal schwannomas have been discussed in the literature, including endoscopic removal and a laryngofissure approach ,14. The location  In the p


Any mass on the chest wall may not always be the primary local pathology. A case of lymphoma with an aggressive course may involve the sternum through local invasion and can mimic a chest wall tumour. A 15-year-old boy with mediastinal lymphoma presented with a sternal mass. Partial sternectomy with replacement by methyl methacrylate prosthesis was performed. Majority of sternal tumours are malignant regardless of being primary or secondary. A slowly enlarging lump of the sternum may mimic as a primary pathology. A CT scan can also support it as sternal tumour spread to the adjacent tissues. We managed a patient with a suspected primary sternal tumour; it was later found to be an extension of mediastinal lymphoma.A 15-year-old boy was referred to thoracic surgery clinic with a slowly growing non-tender mass on the anterior chest wall for 2 months. It was initially considered as a subcutaneous abscess because of slow growth and fluctuation on palpation. Ultrasound of the lesion reported as subcutaneous abscess. Needle aspiration of the lesion did not reveal any pus thus infection was ruled out. At the one-month follow-up, the overlying skin of the mass became whitish and its periphery turned reddish and inflamed. Patient also developed pain and tenderness locally. The size of the mass increased from 2x2 cm to 5x5 cm and involved the middle part of the sternum.Laboratory tests were within the normal range. CT scan revealed a mass of 8x5 cm occupying the anterior mediastinum, eroding the sternum, extending subcutaneously as an expanding necrotic area. A thymic tissue of 4x2 cm and conglomerated lymph nodes without definite borders were also noted . The second percutaneous needle biopsy of the lesion gave no aspirate. Pathological examination of the trucut needle biopsy could not prove malignancy. The patient was considered as having a sternal mass. For both the diagnosis and treatment, the anterior mediastinum was explored. Frozen section examination showed malignancy. Skin flaps were elevated over the tumour mass. The pectoral muscles were dissected from the chest wall laterally and superiorly. Total gross excision of the lesion was performed. The resection included the two thirds of sternal body, the anterior aspects of the third to sixth ribs bilaterally to the costochondral junctions. A discrete mediastinal mass of 8x10 cm and the attached thymus were also excised. The resultant bony defect of the anterior chest wall (about 10 cm) was covered with sandwich prosthesis with propylene mesh and methyl methacrylate. Medial compression of the chest from either side enabled the graft to be sutured without tension. The pectoral muscles were then re-approximated in the midline. Chest drain was left. The postoperative recovery was uneventful.Pathological examination of the specimen revealed primary mediastinal B-cell lymphoma invading thymus, sternum and skin. Immunohistochemically, the malignant cells were positive for CD20, Bcl-2, LCA, CD43, EMA and negative for CD3, CD10, SMA, desmin, myoglobin, suggestive of B-cell lymphoma. Following surgery and adjuvant chemotherapy, the patient was clinically without evidence of recurrent disease and had an acceptable cosmetic appearance.Tumours of the sternum are uncommon lesions. They present with localized pain. The masses in anterior mediastinum are more malignant compared to the other locations. These include thymoma, germ cell tumours, and lymphomas. Primary mediastinal lymphomas compose 10-20% of primary mediastinal tumours.[2-4] Primary non-Hodgkin disease in the sternum hardly occurs and believed as an extension of the disease from the anterior mediastinal lymph nodes. The cortex and sponges were not intact in our case. The disease, however, involved both the mediastinal and anterior layers of the periosteum and exhibited marked reactive membranous bone formation. The disease involved the anterior mediastinal and parasternal nodes first, and then secondarily invaded the sternum by extension through the peristernal lymphatics, which were connected to the sternal periosteum and intramedullary spaces.[5]The index case was an example of localized lymphoma. The exploration was done to determine resectability of a primary sternal tumour. The treatment of choice for a primary sternal tumour is total sternectomy when the deeper anterior mediastinal nodes are uninvolved. Even some localized lymphomas are amenable to aggressive surgical therapy.To conclude, anterior mediastinal lymphomas may mimic as primary sternal mass. Fine needle aspiration and trucut biopsy were non-contributory to the final diagnosis. Sternectomy with complete apparent surgical resection of the tumour and adjuvant chemotherapy worked well for our patient.Source of Support: NilConflict of Interest: None declared


Dysphagia represents a difficulty in passage of solid or liquid foods from the oral cavity into the stomach and is considered as an alarm symptom of gastrointestinal system. It often indicates an organic disease and needs to be explained. In this paper, a case of 61-year-old man with posterior tongue abscess is presented. Dysphagia represents a difficulty in passage of solid or liquid foods from the oral cavity into the stomach and is considered as an alarm symptom of gastrointestinal system. It often indicates an organic disease and needs to be explained. Dysphagia is classified into two major types: oropharyngeal (high) dysphagia and esophageal dysphagia based on the localization , 2.Oropharyngeal dysphagia is characterized by difficulty in initiating swallowing and usually indicates a problem on the oral or pharyngeal localization. Although the most frequent causes of oropharyngeal dysphagia are central nervous system disorders in older people and inflammatory muscle diseases in young, the infections of oral cavity and the neck should be remembered. Although tongue abscesses are rarely seen, they should be kept in mind in differential diagnosis of dysphagia. If the early diagnosis and treatment of posterior tongue abscesses is not achieved, it may lead to sudden life-threatening respiratory complications.In this paper, a case of 61-year-old man with posterior tongue abscess is presented with clinical, radiological, and microbiological features. Taking a careful history, the appropriate diagnostic tests, and consultations with other departments and planning an immediate surgical and/or medical treatment will follow.A 61-year-old man was admitted to the department of the gastroenterology with complaints of odynophagia and dysphagia to solid foods for one month. He experienced worsening of dysphagia and he also reported otalgia for the last two days. His oral intake had decreased and he had preferred to consume liquids. As a result, he had lost 10 kg in a month. Fifteen days before the admission, the patient was assessed by several ENT outpatient clinics and was treated for pharyngitis with two different antibiotics. He had no history of smoking, alcohol consumption, or any other systemic diseases. During the questioning of the patient, he pointed out his neck region as the site of obstruction. On physical examination, he was afebrile with normal vital signs. However, the palpation of the neck was painful. The white blood cell count was 6,940/mm³, C-reactive protein was 51 mg/L, and erythrocyte sedimentation rate was 64 mm/hour. The standard barium swallow study was normal. The upper gastrointestinal endoscopy revealed normal findings except for erosive bulbitis. The etiology of dysphagia had not been identified by these tests and a computed tomography (CT) scan of the neck was performed. The CT scan demonstrated a 4 × 2.5 cm sized cystic lesion with minimal irregular border at the base of the tongue . The patOropharyngeal dysphagia, cervical dysphagia, and transfer dysphagia are synonyms of each other. The cricopharyngeal region and the upper 1/4 of esophagus are under the direct control of the central nervous system. The pharyngeal phase of swallowing is under the management of brain stem and controlled reflexively.The causes of oropharyngeal dysphagia can be classified as either the neuromuscular or the structural. The most commonly seen neuromuscular pathologies are cerebrovascular accidents, Parkinson's disease, Myasthenia gravis (affecting nerve-muscle junction), and polymyositis (affecting muscles). Carcinomas, cervical osteophytes, esophageal webs, radiation and surgical therapies, Zenker's diverticulum, Schatzki's rings, peptic strictures, goiter, infections of the neck, and pharynx are the structural causes , 3.Although the tongue is exposed to many potential pathogens, it is resistant to infections. Thus, tongue abscesses are rarely seen infections due to the strong protective mechanism. The protective mechanisms are the increased vascularization and lymphatic drainage, thick keratinized mucosa of the tongue, the immunological properties of saliva, and the constant mobility of the tongue which reveals cleaning effect of saliva . The antThe symptoms of the tongue abscess are sudden painful swelling of the tongue within hours or days, pain radiating toward the ears, and voluntary fixation of tongue due to pain, fever, dyspnea, dysphagia, and odynophagia. Dysphagia and dyspnea are the alarm symptoms that the lesion should be decompressed urgently and airway maintenance is necessary. Therefore, ENT surgeons must be consulted in time to prevent life-threatening results. Patients with oropharyngeal dysphagia usually report discomfort in the cervical region , 9. Our The differential diagnosis for the swellings of the tongue includes carcinomas, anaphylaxis, acute epiglottitis, dermoid cysts, lipoma, lingual artery aneurysm, arteriovenous malformation, infarction, hemorrhage, lingual tonsillitis, thyroglossal cysts, tuberculosis, and actinomycosis . The diaThe successful treatment of tongue abscess begins with an accurate diagnosis and consists of airway maintenance, abscess drainage, and antibiotic treatment. It has been reported in the literature that the surgical drainage of the posterior tongue abscess may be technically difficult. Drainage may cause edema and respiratory involvement; thus the general anesthesia and endotracheal intubation may be required. Vellin et al. and Balatsouras et al. preferred needle aspiration technique in their case reports , 8. To aThe posterior tongue abscesses are rare but potentially life-threatening pathologies. Trauma, foreign bodies, dental infections, tonsillitis, and surgical history are the most common etiological factors. If there is dysphagia to solids, which is unexplained by barium swallow pharyngoesophagography and upper endoscopic examinations, tongue abscesses should be considered and it should be noted that the neck CT may also help in the diagnosis.


What follows is a case report of a 12 years old child whocomplained of gaps between teeth in the lower front region.OPG showed tooth 43 impacted and malformed withenlarged pulp chamber and a unilocular radiolucencysurrounding the crown. Surgical enucleation was done underLA. Biopsy was sent for histopathological examinationrevealing the miracle diagnosis of "Central OdontogenicFibroma" being a rare tumor of odontogenic tumor familyand also a rare finding regard to age, site and clinical behaviorof tumor is reported. Central odontogenic fibroma is a rare and benign neoplasmof jaw. It is derived from mesenchymal component ofodontogenic apparatus that is dental papillae, dental follicleand periodontal ligament. Clinically it is more frequentlyseen in children and young adult. Commonly found inmandible but in posterior regions. Radiographically, majorityof COF are radiolucent with multilocular radiolucencyand rarely unilocular. Lesion often contains small radiopaqueflecks of varying density.Histologically WHO type COF consists of maturecellular fibrous connective tissue with many islands ofodontogenic epithelium. Osteoids, dysplastic dentin andcementum can be seen.Case of central odontogenic fibromaA 12 years old male child presented to Jaipur Dental College,Department of Pedodontics and Preventive Dentistry. Thechief complaint of patient was gaps between teeth in lowerfront region. Patient gave history of crown fracture of 42which he had got endodontically treated.Neem datun to maintain his oral hygieneand behaviorally the patient was cooperative.The patient used Extraoral examination revealed that patient was ofnormal built with facial symmetry bilaterally symmetrical,oval facial form and a straight facial profile.On Intraoral examination soft tissue appeared normal.On hard tissue examination all permanent teeth were presentexcept 41, 43 and 45 .42 showed unsatisfactory restoration and was driftingdistally. Molar relation on left side was class I and on rightside class III.The patient was advised for OPG to see the missing teeth.Radiographic examination revealed:41: congenitally missing42: endodontically treated45: impacted, and an unusual finding was seen in relationto 43 which was impacted and malformed with enlargedpulp chamber and radiolucency completely surrounding thecrown.At this juncture, the patient was referred to orthodonticdepartment for further opinion regarding definitiveorthodontic treatment plan and it was decided to firstundergo surgical extraction of malformed 43 followed byfixed orthodontic therapy. Therefore, the treatment plan was,first the oral prophylaxis was done, then surgical extractionof malformed 43 followed by restoration of 42 followed byfixed orthodontic therapy.The surgery was planned and consent of parents was taken.The inferior alveolar nerve block was given on right side.The incision extending from mesial of 42 to 44 was madeand the envelop flap was raised and the lesion was exposed.Then, we surgically extracted 43 along with the soft tissuelesion . The flap was repositioned and interruptedsutures were given.Then, the specimen  was sent to oral pathologydepartment for histopathological examination.Soft tissue section consisted of soft connective tissue stromawith collagen fibers arranged in whorl pattern. The fibrouscomponent varied from fibrous to myxoid. Island ofodontogenic epithelium  were visible all over theconnective tissue stroma. Some islands appeared ameloblastlike cells and stellate reticulum and some solid lackingfeatures of odontogenic epithelium. Few islands weresurrounded by eosinophilic material . Numerouscalcification in form of dentids and cementum visible.Fibrous capsule with strands and islands of odontogenicepithelium was seen. Dentin hypocalcified at places andshowed interglobular dentin. Regular enamel space wasseen. Cementum appeared normal. Pulp showed collagenfiber, blood vessels and pulp stones. Pulp stones did notresemble dentin."Central odontogenic fibroma-WHO type".Seeing the histological findings the diagnosis came asCentral odontogenic fibroma is a rare and a benign neoplasmof jaw. It is derived from mesenchymal component ofodontogenic apparatus that is dental papillae, dental follicleand periodontal ligament.Clinically, it is found in maxilla and mandible. Morefrequently seen in mandible in the posterior region but, inthis case it was seen in anterior region of mandible.It is asymptomatic and displacement of teeth can be seen.It is more commonly seen in children and young adults andis more prediculent in females.Radiographically, majority of COF are radiolucent withmultilocular radiolucency and rarely unilocular.Histologically, the WHO type consist of mature cellularfibrous connective tissue with many islands of odontogenicepithelium. Osteoids, dysplastic dentin and cementum canbe seen.


A 26-year-old woman presented with symptomatic recurrent desmoid tumor to her anterior abdominal wall. The patient underwent radical resection of the desmoid tumor. She was left with only 30% of her anterior abdominal wall intact, creating a large defect requiring composite reconstruction.What is a desmoid tumor?What are treatment options for desmoid tumor?What are surgical options for composite abdominal wall reconstruction?What specific considerations should be made when deciding a particular reconstructive surgery?Desmoid tumors are derived from the proliferation of spindle-shaped mesenchymal fibroblast-like cells. Desmoid tumors often present as an aggressive fibroproliferative process, with a variable clinical course. Metastatic disease and dedifferentiation into high-grade malignancy are not typical features.,Treatment options for desmoid tumors include surgical excision, radiotherapy, systemic medical treatment, or combinations of these.1This is a case of a 26-year-old woman who had presented with symptomatic recurrence of desmoid tumor to her anterior abdominal wall . The pat,Composite full-thickness abdominal wall defects continue to be a challenge to the reconstructive surgeon, and special considerations should be made when deciding a reconstructive surgery. The surgeon is required to address the structural, functional, and aesthetic features when considering surgical options. Pedicled ALT flaps are an option for abdominal wall reconstruction. However, the surgeon needs to consider that the pedicled variant is restricted by the pedicle length and therefore is more suitable for reconstruction of the lower abdominal quadrants.4


Retrorectal tumors in adults are very rare and little known condition. These tumors, often misdiagnosed or mistreated, should be completely excised because of the potential for malignancy or infection. A suitable operative approach is the key to the successful surgical management.We report the case of a 45-year-old Arab male who presented with chronic pelvic pain accompanied by straining to defecate and dysuria. The clinical examination showed a painless mass in the left perineal area. Pelvic magnetic resonance imaging and computed tomography scan demonstrated a huge and well-limited pelvic mass causing displacement and compression of the rectum and bladder. Although the large size of the mass (>7 cm in the greater diameter), it was successfully and completely excised through only perineal approach without undertaking coccygectomy or sacrectomy. The histopathological study revealed a low-grade leiomyosarcoma. The patient is currently in 4-years follow-up with no signs of recurrence or metastasis.Even large retro-rectal tumors may be successfully excised by the perineal approach especially in carefully selected patients, but require extensive knowledge of pelvic anatomy and expertise in pelvic surgery. Retro-rectal tumors are a very rare entity in the adult population with an incidence of about 1 in 40,000 patients [With this case report we attempt to describe the main features related to this disease and highlight key points to improve its safe and successful surgical management particularly with perineal approach.A 45-year-old Arab male presented with a 4-years history of chronic pelvic pain accompanied by straining to defecate and dysuria, but without perineal discharge, rectal bleeding, headaches or weight loss. The patient reported to be operated for anal fistula 10 years ago with satisfactory clinical evolution. His family history was unremarkable and no smoking or chronic alcoholism were noticed. In the proctologic (knee-chest) position, physical examination revealed a 4–5 cm, soft and painless mass in the left perineal area. This mass seemed freely mobile with lower edge extending to the pectinate line but the upper limit was not perceived. No inflammatory signs, fistulous tracts or postanal skin dimple were observed. The mucosa was normal on digital rectal examination and exterior pressure was detected in the posterior region.. Moreover, rectoscopy detected a bulging mass with intact mucosa in the posterior wall of the rectum. Routine blood tests and serum tumor markers including carcinoembryonic antigen, cancer antigen 19–9, cancer antigen 125, prostatic specific antigen and α-fetoprotein were within normal range.Abdominal ultrasound detected a hypoechoic solid pelvic mass with regular contours and heterogeneous echostructure, but relationship with the surrounding structures was not clear. On computed tomography (CT) scan the lesion appeared as a large heterogeneous mass measuring approximately 7 cm in diameter. This mass, developed in the latero-rectal space, extended to the left side of the pelvis causing displacement and compression of the rectum and urinary bladder. There was no pelvic effusion, deep lymph nodes or bone destruction Fig. . AdditioFig. . Moreove and completely excised, with special attention to avoiding injuries to the sphincter complex and rectal perforation Figs. , 4. AftePathological examination of the resected specimen revealed an encapsulated mass measuring 82 × 70 × 60 mm. Microscopically, the tumor was composed of spindle shaped cells arranged in interlacing fascicles with focal myxoid change and areas of necrosis. Number of mitoses was 4 per 10 high power fields (HPF) and all surgical margins were free of disease. In addition, tumor cells exhibited positive staining for smooth muscle actin and desmin, and negative staining for CD-117, CD-34 and S-100 protein. With these findings, final diagnosis was low-grade leiomyosarcoma. According to the low grade of the tumor and the complete surgical excision, no adjuvant treatment was decided and the patient was discharged after an uneventful postoperative period. He is currently in 4-years follow-up with no evidence of recurrence or metastasis. He underwent periodically physical examination with abdomen/pelvic CT every 3 months for 2 years, then every 6 months for the next 2 years.The retro-rectal or pre-sacral space is the virtual anatomic area limited anteriorly by the fascia propria of the rectum, posteriorly by the presacral fascia, superiorly by the peritoneal reflection, and inferiorly by the recto-sacral fascia  and the supralevator space. The lateral boundaries are the lateral stalks of the rectum, the ureters and the iliac vessels , 3. ThisRetro-rectal tumors, typically slow growing, may be completely asymptomatic over prolonged periods of time. Consequently, they are often only discovered incidentally during examination for unrelated physical complaints. The symptoms are usually nonspecific and attributed to compression or invasion of surrounding structures, as was observed in our patient. Symptomatic patients may present vague pain in the perineal area, chronic constipation, rectal or urinary incontinence or sexual dysfunction. Some tumors can lead to perineal discharge with midline dimpling in the sacrococcygeal area, and may be confused with perianal suppurations and complicated fistulas , 6. NeveAlthough pelvic plain films can reveal indirect signs such as the bone destruction typical of malignancy and the presence of calcifications in mature teratomas, diagnosis requires modern imaging techniques for preoperative evaluation of retro-rectal tumors. Providing excellent morphological description of these tumors, CT and MRI have become the best diagnostic modalities to date. CT scan is used to distinguish whether tumors are cystic, solid or mixed and to assess for sacral involvement or invasion to pelvic structures. Whilst MRI, with his higher resolution in soft tissue, is particularly useful in delineating soft-tissue planes and precisely evaluating the relationships of the mass with bones, muscles and nerves . These pIn appropriate surgical candidates, complete surgical excision with clear resection margins remains the cornerstone option for surgical management of retro-rectal tumors, even if asymptomatic and apparently benign. The possibility of malignancy and the risk of eventual dystocia in women of childbearing age, of degeneration in teratomas or infection in cystic tumors have been reported as the reasons for necessity of operative extirpation . DefinitGenerally, three different surgical approaches are commonly used in the resection of retrorectal tumors: anterior, posterior, and combined approaches. The anterior or abdominal approach is recommended for larger (more than 5 cm) and highlying (the lower margin of the lesion is above the level of S4) retro-rectal tumors, because it allows an excellent exposure of pelvic structures, bleeding control, and easier mobilization of rectum , 10. AltAbdominoperineal approach seemed to be the most appropriate procedure in our patient, because the tumor extended below the level of S4 and its upper limit was not reachable on digital rectal exploration. However, despite the large size of the mass and its intrapelvic extension, considered as not suitable for the only perineal approach, we chose it to avoid laparotomy and its potential complications. We obtained optimal exposure with good access to both the tumor and the retrorectal space through a longitudinal para-anal incision centered on the lesion without the necessity of abdominal access. We believe that the perineal approach was particularly feasible in our patient because of the absence of rectal and sacral invasion. This enabled us to achieve an easy dissection of the intrapelvic component of the tumor using retrorectal fat tissue as a cleavage line between tumor and rectum.Identification and transaction of the anococcygeal ligament facilitates exposure of the mass and dissection of the plane between the lesion and the mesorectum. In addition, many authors advocate routine coccygectomy in order to obtain sufficient exposure allowing the complete excision of the mass. Teratomas and all cystic lesions are known to often arise from the coccyx, thus “en bloc” coccygeal resection with these tumors seems necessary for lower rate of recurrence. Currently, this attitude is not recommended unless the coccyx is frankly invaded by a malignant or highly suspected malignant lesion . If sacrFinally, it is important to emphasize that the successful management of retro-rectal tumors requires extensive knowledge of pelvic anatomy and expertise in pelvic surgery. Consequently, optimal treatment can be achieved only by a multidisciplinary team involving colorectal surgeons, neurosurgeons, orthopedic and plastic surgeons especially with tumors involving the sacrum and sacral nerve roots.Retro-rectal tumors are an uncommon disease with often nonspecific clinical presentation which makes diagnosis late and difficult. Therefore, diagnosis of these lesions requires a high index of suspicion, and should be considered in any patient with palpable mass on rectal examination. Complete surgical resection with negative margins is the best treatment even in asymptomatic patients. The perineal approach is feasible, minimally invasive and safe option, providing complete recovery for carefully selected patients with low-lying retro-rectal tumors. This approach should be included in the therapeutic arsenal of colorectal surgery. Finally, a multidisciplinary team is likely to improve the rate of successful treatment of these specific lesions.Written informed consent was obtained from the patient for publication of this Case Report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Primary sinonasal and middle ear neuroendocrine carcinomas are rare malignancies of the head and neck. Owing to the rarity of these tumors, the clinical behavior and optimal management of these tumors are not well defined. We present a case of an incidentally discovered sinonasal neuroendocrine carcinoma that was found to originate from the Eustachian tube, which has not previously been described in the literature. This patient was treated with primary surgical resection using a combination of transnasal and transaural approaches and achieved an incomplete resection. Follow-up imaging demonstrated continued tumor growth in the Eustachian tube as well as a new growth in the ipsilateral cerebellopontine angle and findings suspicious of perineural invasion. However, the tumor exhibited a benign growth pattern and despite continued growth the patient did not receive additional treatment and he remains asymptomatic 35 months following his original surgery.  Primary sinonasal neuroendocrine tumors are rare . Four hiNECs are further classified as well-differentiated , moderately differentiated , and poorly differentiated  , 3. OwinA 68-year-old man presented with an incidentally discovered 2 × 1.6 cm polypoid, exophytic mass in his right posterior nasopharynx found on imaging done as part of a stroke workup . He had Two months later, clinical examination revealed a violaceous, vascular, benign-appearing lesion pedicled to the right Eustachian tube orifice. Aural examination revealed evidence of previous mastoid surgery but no other abnormalities. Owing to concern of aspiration, biopsy was not initially attempted and he was referred for single stage excision. The lesion was removed endoscopically; however, the origin of the tumor could not be easily resected transnasally and given the mass' benign appearance, this was not aggressively pursued. Consequently, a small amount of the tumor pedicle was left. Pathology revealed moderately differentiated NEC Figures  and 3 anExamination two months following demonstrated no evidence of recurrence. Due to the unusual location of this tumor and to rule out another primary, an octreotide scan as well as CT scans of the head, neck, chest, abdomen, and pelvis was done. These demonstrated no other focus of disease; however, they confirmed residual disease in the Eustachian tube. With residual tumor, it was felt that there was, as yet, no role for chemotherapy. Repeat examination five months following surgery demonstrated a 5 mm nodule of tumor at the introitus of the Eustachian tube and he underwent transnasal resection of the residual mass 1 year following his original surgery . Most ofFour months following this surgery, he underwent a microscopic transmastoid right subtotal petrosectomy with microdebridement of his tumor. Tumor was found in the epitympanum and sinus tympani and partially covered the anterior wall and completely covered the medial wall of the middle ear. Most of the tumor in the middle ear space was removed and dissection was carried to the limits of instrumentation. There was, however, some residual tumor in the Eustachian tube and along the stapes footplate that could not be removed. Pathology demonstrated the aforementioned NEC.Follow-up MRI done three and eight months following this surgery demonstrated an area of enhancement anterior to the right Eustachian tube that increased from 14 × 6 mm to 14 mm × 8 mm in the intervening time. The second MRI also demonstrated a 17 × 3 mm enhancing lesion in the right internal auditory canal with an extracanalicular cerebellopontine angle component in addition to perineural invasion . Three wSinonasal NEC was first proposed as a distinct entity in 1982 when it was appreciated that certain small-cell tumors behaved distinctly from other olfactory neuroblastomas . These tAverage age of presentation of sinonasal NECs is 50 years –10. MoreThe most frequent presenting symptoms are nasal obstruction and epistaxis. Two case series summarize the most frequent presenting complaints for sinonasal NECs  2, 4]. , 4. 2, 4Most often, the precise origin of sinonasal NECs cannot be ascertained and, in one series, the tumor origin could only be identified in 30% of cases . HoweverThe unique site of origin of this tumor implies surgical challenges that are not encountered with previously reported sinonasal and middle ear NECs and distinguishes this case from previously reported NECs of sinonasal or middle ear origin. NECs of sinonasal origins may potentially be addressed using endoscopic skull base resections while middle ear tumors may be resected using microscopic techniques, possibly including lateral temporal bone resection. By contrast, the anatomic considerations of resecting a tumor originating from the Eustachian tube are unique and would require a much more invasive middle fossa approach, if an aggressive surgical resection was desired.Most patients with sinonasal NEC present with advanced local disease but rarely with locoregional or distant metastases. One series reported that nearly 80% of patients presented with moderately advanced or very advanced local disease . Only 10Owing to limited published data regarding the behavior and treatment of sinonasal NEC, treatment decisions have often been adapted from NECs of other anatomical sites, such as lung and larynx . TreatmeNeoadjuvant treatment of sinonasal NEC varies. In one series, 15% of patients received preoperative radiation while in another, none received preoperative radiation , 4. By cMost patients receive surgery as part of their treatment, with reported rates between 75% and 80%  2, 4]. , 4. 2, 4Patients treated initially with surgery with or without neoadjuvant chemotherapy frequently receive adjuvant chemotherapy and/or radiation, most commonly adjuvant radiation , 4. FollCurrent combined modality treatment of NECs has led to good disease control and survival rates . In one Recurrence following successful primary treatment of NEC is common, with locoregional more common than distant metastatic recurrence. Reported rates of recurrence for NEC are between 41% and 53% with approximately 75% developing locoregional and the remainder distant metastases , 4. MeanIn our patient, gross residual disease remained following initial treatment which has been associated with a poorer prognosis. One series reported that most patients with residual disease died of their disease within 18 months of initial treatment . Our patThis is the first reported case of sinonasal NEC originating from the Eustachian tube. Although NECs originating from the sinonasal tract and middle ear space have previously been reported, the anatomic confines and surgical considerations of this case of NEC which originates from the Eustachian tube render this a unique, previously unreported case. Pretreatment considerations should include tumor differentiation and surgical resectability. A multimodal treatment approach including surgery, chemotherapy, and radiation should be considered with these tumors.


Krukenberg tumors accompanied by gestational hypertension are rare and have a poor patient prognosis. A gravida 1, para 0 patient was submitted to Tianjin Medical University General Hospital  at 32 weeks gestation with symptoms of nausea, vomiting and hypertension. Diagnosis from the gastroscopic biopsy was of a gastric ulcer. A unilateral ovarian mass was identified with B-scan ultrasonography and magnetic resonance imaging, but was confirmed pathologically as a bilateral Krukenberg tumor. Positron emission tomography-computed tomography revealed a high radioactive uptake in the lesser curvature wall of the stomach, and postoperative pathology revealed poorly differentiated adenocarcinoma of the stomach. As Krukenberg tumors are difficult to diagnose, exhibit fast progression and have a poor clinical outcome, developing a greater understanding of Krukenberg tumors is crucial. Imaging manifestations combined with serological examination may aid in early detection, which may lead to improved patient management. A Krukenberg tumor is metastatic ovarian mucin-filled signet-ring cell carcinoma , that exA 31-year-old female, gravida 1, para 0, was referred to Tianjin Medical University General Hospital  at 32 weeks gestation due to nausea and vomiting, with a blood pressure of 155/101 mmHg. The patient had experienced epigastric discomfort for two weeks prior to admission, however, did not see a doctor until the vomiting had persisted for three days. The patient had gained 7 kg in the last month and had previously had regular menses . The last menstrual period had been July 2, 2012, and the estimated date of confinement was April 9, 2013. A uric human chorionic gonadotropin (HCG) test was positive following 40 days of amenorrhea. Fetal movements were felt at four months gestation and a four dimensional ultrasound examination was normal at 28 weeks gestation. The patient had previously experienced two episodes of stomach bleeding (one year and two years ago) due to unknown reasons, but the gastroscopy examinations had appeared to be normal. The patient’s mother had a history of hypertension. Serological tests revealed an increased number of white blood cells and mildly abnormal hepatic and renal function, additionally the D-dimer levels were abnormally high . A urineTransabdominal color Doppler examination revealed an encapsulated pedunculated solid and cystic mass (20×18×13 cm) with irregular but clear margins on the front left of the uterus . A main At week 38, the patient underwent an abdominal exploration and a healthy male infant weighing 1,300 g was delivered by cesarean section, with Apgar scores of 6 and 8 at 1 and 5 min, respectively. The left uterine appendages were removed, and subsequent pathological examination of the frozen section of the left ovary revealed metastatic poorly-differentiated adenocarcinoma . SubsequFive days after surgery, the patient underwent a positron emission tomography-computed tomography scan. High levels of radioactive uptake were detected in the lesser curvature wall of the stomach, which indicated gastric carcinoma. The patient underwent a total gastrectomy and regional lymph node dissection combined with intraperitoneal chemotherapy. Postoperative pathology revealed poorly-differentiated adenocarcinoma with signet-ring cell carcinoma of the stomach, which infiltrated the serous membrane. In addition, a cancerous embolus was observed in the blood vessels.Krukenberg tumors are a type of metastatic ovarian tumor. The primary tumor usually originates from the gastrointestinal tract, primarily from the stomach or the colon and rectum, although occasionally the tumors originate from the breast, uterus, biliary tract, pancreas and kidney . The bloThe level of ovarian hormones during pregnancy and a rich blood flow contribute to tumor metastases. The most common clinical manifestations of Krukenberg tumors during pregnancy are nausea and vomiting, which are similar to symptoms often experienced during pregnancy, thus, may be neglected. As the gestational period increases and the circumference of the waist increases, the pelvic neoplasm and ascites are difficult to locate . TherefoExamining the images in the current case only located the ovarian neoplasm on the left side, but pathological diagnosis indicated that the carcinoma had also invaded the right ovary. This is due to tumor metastasis, particularly from the stomach, which is usually via a blood or lymphatic channel and leads to bilateral adnexa involvement. However, the degree of involvement varies. Certain tumors may be located by image examinations, while others cannot. Therefore, the diagnosis of a Krukenberg tumor should depend on pathological confirmation. Once unilateral lesions are located by image examination, it is important to be aware that contralateral metastasis may also exist. Thus, more care should be taken during abdominal exploration, as resecting all metastases is important to improve the patient prognosis.In conclusion, Krukenberg tumors are difficult to diagnose, progress fast and have a poor outcome, thus, improving the understanding of Krukenberg tumors is of particular importance. When a solid and cystic mass is identified in a pregnant woman by ultrasound, with multiple hyperechoic nodules and a main vessel with small branches in the solid portion, that may be a Krukenberg tumor instead of a primary tumor, further examinations should be performed to substantiate the diagnosis and identify the source, this may help doctors to chose the best remedy and gain more survival time for the patient.


A male patient aged 20 years, visited us for routine oral examination. Incidentally, a well-demarcated reddish area of depapillation, of size 6X3 cm, was found on the dorsum of tongue near the midline. It was located 1 cm anterior to the circumvallate papillae and was roughly diamond shaped. Its surface was smooth, raised and fissured; the opposing palatal mucosa was non-erythematous (kissing lesion was absent). History revealed that it was stable since childhood and was occasionally associated with burning sensation. The patient was otherwise healthy, with no history of tobacco usage or diabetes. Based on history and clinical examination, a diagnosis of “median rhomboid glossitis” was arrived. The patient was given fluconazole  to rule out candidiasis, but, the lesion did not regress. Median rhomboid glossitis is also known as “central papillary atrophy of the tongue”. It is a benign lesion that shows a marked male predilection and occurs in less than 1% of adult population. Initially, it was believed to be developmental in origin, but, during the recent years, it has been considered as a variant of candidiasis. The differential diagnosis includes erythroplakia, geographic tongue, granular cell tumor. Patients with this condition should be reassured about its harmless nature and no treatment is required for asymptomatic cases.


Among all the possible complications of aesthetic rhinoplasty, a rare one is the development of cystic masses on the nasal dorsum: several theories suggest that cysts develop commonly by entrapment of nasal mucosa in the subcutaneous space, but they can also originate from foreign body reactions. This report deals with two cases of nasal dorsum cysts with different pathogenesis: both patients had undergone aesthetic rhinoplasty in the past . Both cystic masses were removed via a direct open approach and nasal reconstruction was performed successfully with autologous vomer bone. The pathologic investigations showed a foreign body inclusion cyst associated with latex rubber in the first case and a sequestration of a mucosal-lined nasal bone was not removed at the time of primary rhinoplasty in the second case. A brief review of the literature focuses on the pathophysiology and treatment options for nasal dorsal cysts following aesthetic rhinoplasty. The development of postrhinoplasty nasal dorsal irregularities due to bone or cartilaginous bossae is a common complication, whereas cysts formation is a rare event. Reviewing previously reported cases reveals that mucous cysts are by far most prevalent in the nasal dorsum. Other locations include the nasal tip, the medial canthus, and the paranasal area –3. ProbaWith regard to nonmucosal cysts of the subcutaneous area, a fully different pathophysiology, namely, foreign body reaction, may be considered. Petrolatum jelly migrated from the packing material and foreign body reaction to alloimplants employed for augmentation have been reported to be possible causes . Other rSince the postsurgical cyst development could be in part prevented, particularly by considering all pathogenetic mechanisms, this report aims at underlining the need of further insight into this rare entity.We evaluated a 62-year-old Caucasian male with an asymptomatic nasal radix mass and nasal airflow obstruction. Twenty-six years ago he had undergone reduction rhinoplasty with an uneventful postoperative period; however, nasal obstruction had not improved.A nasal dorsum mass, measuring 5 × 5 mm was noted three months postoperatively and slowly increased in size over the years. On physical examination, the nasal dorsum and tip were firm on palpation and a small deformity was present in the upper nasal dorsum, appearing as a 2.0 × 2.0 cm round, soft, and painless mass located on the midline of nasal radix . A compuIn consideration of clinical and CT-features, a presurgical diagnosis of nasal radix cyst as a consequence of previous augmentation rhinoplasty was made. The mass was successfully removed via a horizontal glabellar incision. After a skin incision, the cyst was dissected down to the nasal bone and removed in en-bloc fashion because of its close relation to the bony dorsum .The removed cyst contained a liquid, clear content surrounded by a slightly fibrous wall. Inside the cyst, a foreign body was evacuated and revealed to be a 5 × 2 cm piece of latex rubber excised from surgical gloves and supposedly folded to fit the underlying bony defect created by hump removal during the primary rhinoplasty procedure. Foreign material was surrounded by foreign body-type, multinucleated giant cells, and infiltration of chronic inflammatory cells inducing granulomatous reactions .The depressed radix residual due to the underling small bony defect was corrected by a bone autograft taken from the vomer bone exposed during concomitant closed approach septoplasty for correction of the residual nasal obstruction. The redundant skin of the radix was trimmed and accurately sutured.The patient's postoperative course was uneventful and no external deformity remained. Nasal airflow was not affected by the procedure and the patient was satisfied with the aesthetic result.A 44-year-old Caucasian female with no significant medical history, who had previously undergone rhinoplasty 14 years ago, reported of a functionally satisfactory postoperative period without complications. However, three years ago, she discovered a gradually increasing 3 × 3 mm mass at the nasal radix. She also complained of persistent obstruction of nasal airflow.Physical examination revealed a spherical nasal mass (1.0 × 1.0 cm), which was on palpation that was found to be firm-elastic, mobile, painless, and covered by normal skin, located on the right nasal radix . A CT scThe patient was admitted for removal of the mass and reconstruction of the nasal dorsum.An open excision of the lesion was performed. The mass was found to be tightly attached to the bony dorsum and it was thus dissected down to the nasal bone and removed in en-bloc fashion. Within the intact capsule, a mucous-lined cavity filled with a thick yellow liquid was found. Histopathologic investigations revealed a benign epithelial mucous cyst containing a fragment of normal cartilage and bone .The lesion was diagnosed as a sequestration of a mucosal-lined nasal bone not removed at the time of hump removal.The resulting deformity of the nasal root was corrected by using autologous bony graft from the vomer. The excessive skin was removed and sutured carefully. The patient's postoperative course was uneventful and no external deformity remained with a satisfactory final result.According to the literature, the different circumstances of the cases presented here suggest different pathways for cysts formation. The most accepted theories about the etiology of mucous cysts after aesthetic rhinoplasty include the presence of ectopic free mucosal graft implantation during surgical treatment, herniation of mucosa through intranasal incision, improper clearing of mucous epithelial remnants, or bony and cartilage parts during the operation , 9. OtheInflammatory reaction to a foreign body has also previously been described as a possible cause , 14, 15.The only valid treatment of postrhinoplasty nasal dorsal cyst is the complete resection and reconstruction. We performed a direct open approach in both cases, using a glabellar horizontal incision over the cyst. We chose the direct transcutaneous route to the lesion in order to assure the adequate exposure of the upper part of the lesion. A conventional open approach to the nasal septum was unnecessary due to the absence of concomitant dorsal or tip further deformities.Moreover, the horizontal glabellar incision seemed well tolerated, without noticeable scar formations. A closed approach using hemitransfix incision was felt sufficient to expose the septum and to avoid any dissection and possible connection between the nasal vestibule and the dorsal area. Augmentation of the residual depressed dorsum and radix with autogenous septal bone was possible.When treating nasal dorsal masses, one first has to exclude various nasal lesions not related to surgery: neoplastic lesions, benign processes, encephaloceles, gliomas, dermoids, osteomas, lipomas, granulomatous diseases , and infections  .In conclusion, according to the current literature, we would point out that postrhinoplasty nasal cysts do occur , 19–21:


Oral lichen planus which is one of the most common oralmucosal diseases in adults, it has been rarely described inchildren. There are very reports in the literature regardingoral lichen planus in children, here we report a case of intraorallesions of lichen planus. Lichen planus should beconsidered in the differential diagnosis of hyperkeratotic orerosive lesions of the oral mucosa in children. Lichen planus is a common chronic inflammatory diseaseof skin and mucous membraness.544Up to six clinical appearances of oral lichen planus havebeen described,6VS Dental College andHospital, Bengaluru, with a chief complaint of burningsensation in her mouth on consuming food for the past 3months and bilateral pigmentation on the inner part of hercheek.A 12 years old girl reported to the Department of Pedodonticsand Preventive Dentistry at Medical history and review of system were noncontributory. A family history failed to reveal the presenceof any similar lesion in the immediate relation.On examination she appeared to be a healthy 12 yearsold with no skin rashes. Oral examination showed bilaterallybluish purple striations in the posterior buccal sulciextending onto the buccal mucosa. This was approximately8 mm × 12 mm in size, flat and nontender on palpation.The dental state was excellent and there were noamalgam restorations. No other mucosal or skin surfacesshowed lesional changes.A provisional diagnosis of reticular lichen planus wasmade based on clinical examination. Routine haematology,biochemistry and immunology screen were normal except,Haemoglobin which was 9 gm% .Pateint was treated with iron supplements and the haematologicaltests were repeated, which was followed by anincisional biopsy of the buccal mucosa, histopathologicalreports confirmed the diagnosis of the lesion to be LichenPlanus.Patient is currently on topical application of 0.05% Tretinoincream and under weekly review for the first month. Basedon the prognosis a decision will be made after a month,whether to start the patient on systemic steroid therapy.Lichen planus was first described in the literature by EramusWilson in 1869,651418521Familial lichen planus has been reported as beinguncommon.8151618It has been documented that childhood lichen planus ismore common in the tropics612In summary, although lichen planus in children is rareand oral mucosal involvement, this diagnosis should beconsidered in children presenting with white lesions of theoral mucosa. Finding from our case report suggest that thecondition may present as classical lichen planus withoutany predisposing medical history nor positive family history.Although oral lichen planus is considered rare in childhood,the presence of often asymptomatic oral lesions should alertthe clinician to such a diagnosis. The case described in thispaper highlight the importance of considering lichen planusin the differential diagnosis of hyperkeratotic and erosivelesions of the oral mucosa in childhood.


Autoimmune pancreatitis represents 2 % of chronic pancreatitis , most often in adult male presentation associated with IgG4. We present a case of a patient 6 years old, with a dependent pancreatic and bile duct obstruction abdominal mass, which is interesting because this presentation in children is more common with autoimmune pancreatitis that malignancies.Show an exceptional case in pediatrics rheumatology.Presentation of case.Female 6 years old, previously healthy, 3-year evolution gastric recurrent vomiting, abdominal pain intermittently mesogastrio , adding hiporexia, jaundice and increased waist circumference, with palpable mass in the right upper quadrant. Cholestatic syndrome, 857 Lipase, amylase 137 Immunoglobulin IgG subclass 4: 23mg/dL (5-6 years: 1-121). CT: dilatation of intra and extra hepatic bile duct. Level space-occupying hepatic hilum extending to pancreatic head and esophagogastric junction mass. Gonadotropin and alpha-fetoprotein: negatives. Cholecystectomy, incisional biopsy of tumor, liver biopsy was performed. He reported: lymphoplasmacytic sclerosing pancreatitis (chronic autoimmune pancreatitis).This is a rare autoimmune disorder that resembles a pancreatic neoplasm with biliary obstruction, occurring primarily in adults, making it an exceptional case in pediatrics. As part of an IgG4 -associated systemic disease, serum level may be normal in up to 40 %, being positive in pancreatic tissue.None declared.


Lipomas are common soft tissue tumors. Intraosseous lipoma is a rare lesion that constitutes not more than 0.1% of bone tumors. It can occur anywhere in the body and there have only been a few cases found in the mandible. Intraosseous fibrolipoma of the jaw is an uncommon histological variant of the classic lipoma and to the best of our knowledge only one case has previously been reported in the literature. The cause of this lesion is uncertain. Clinically the lesion is generally asymptomatic and its radiographic feature is a well-defined radiolucency. Surgery is the treatment of choice. We present a case of an intraosseous fibrolipoma in the right mandibular ramus in a 25-year-old female. Lipoma is a benign mesenchymal soft tissue tumor of mature adipose tissue with no evidence of cellular atypia.Lipomas are uncommon in the oral cavity and the incidence of this neoplasm is between 1% and 4.5% of all benign lesions [Based on the literature, the overall incidence of intraosseous lipoma represents less than 0.1% of all bone tumors –4 and onIntraosseous lipoma is an uncommon disease. Roux  describeFibrolipoma has been rarely described and, according to the WHO classification , is a vaAccording to the latest review done by de Freitas Silva et al. in 2011   scan revealed a well circumscribed radiolucent unilocular image with sclerotic margins involving the mandibular ramus .Magnetic resonance imaging (MRI) showed a hyperintense neoformation in the T1 and T2 sequences; in the sequences with fat suppression the lesion showed a signal reduction Figures  and 4.As the CT scan and MRI strongly indicated a benign lesion, no exploratory biopsy was performed.Under local anesthesia , an intraoral approach was done. Then an incision along the right external oblique line was performed, the periosteum was elevated, and the bone was drilled until the lesion was exposed; therefore the lesion was removed after blunt dissection .The perilesional curettage after the total removal of the tumor was performed.During this procedure, trauma to the inferior alveolar nerve and lingual nerve was avoided.The histopathological diagnosis of the lesion was fibrolipoma .There was no evidence of recurrence at a 1-year follow-up.In agreement with Cakarer et al. (2009), intraosseous lipomas may arise from the soft tissue adjacent to the bone or may occur in an intramedullary location .The aetiology is not clearly understood; theories such as trauma, infarction, inflammation, and nutritional problems have been proposed , 15–17. Histologically, fibrolipoma is a variant of lipoma, such as spindle cell lipoma, intramuscular or infiltrating lipoma, angiolipoma, sialolipoma, pleomorphic lipoma, and myxoid and atypical lipomas , 19.Intraosseous lipomas were classified and subdivided by Milgram  into thrStage I is tumors of viable fat cells.Stage II is transitional cases composed partly of viable fat cells but also demonstrating fat necrosis and calcification.Stage III is lesions demonstrating necrotic fat, calcification of necrotic fat, variable degrees of cyst formation, and reactive woven bone formation.In contrast to the other cases present in literature described by Newman , our patThe lesion was found accidentally during routine radiographs. Hypoesthesia, pain, and swelling can be associated with these lesions and depend on the location and on the size of the tumor.A radiograph of intraosseous lipoma is usually uncharacteristic and presents a cystic lesion with and increased radiolucency surrounded by a sclerotic rim , 15, 17;In our case MRI showed a hyperintense neoformation like fat tissue in the T1 and T2 sequences; in the sequences with fat suppression the lesion showed signal reduction. For these reasons and for the intraoperative macroscopical aspect of the lesion, the total excision of the neoformation was performed.The differential diagnosis includes keratocystic odontogenic tumor, liposarcoma, simple bone cyst, bone marrow defect, early benign fibroosseous lesion, central giant cell granuloma, calcifying epithelial odontogenic tumor, odontogenic myxoma, cartilaginous tumor, and ameloblastoma .Surgery has been proposed as the therapy of choice .No recurrence or malignant changes of intraosseous lipomas in the maxillofacial region have been reported in the literature .As intraosseous lipomas of the mandible are rare, the diagnosis is very important and it is essential that each new case is documented, especially for the fibrolipoma which has an increased growth potential compared to the classic lipoma .


The dorsal patellar defect is a relatively rare entity that involves the superolateral quadrant of the patella. It is usually considered to represent a delayed ossification process, although its exact origin remains unclear. Because of its usually innocuous nature and clinical course, invasive interventions are generally deemed unnecessary, although curretage has been successfully performed on symptomatic cases. This case report presents a rather unusual case of symptomatic bilateral dorsal patellar defects with cartilage involvement and widespread surrounding bone marrow edema as demonstrated by magnetic resonance imaging (MRI). Both cartilage involvement and bone marrow edema should be considered part of the spectrum of associated MRI findings that can be encountered in this entity. Furthermore, the presented case shows that symptomatic dorsal patellar defects can be treated conservatively with success and that (decrease of) pain symptoms are likely related to (decrease of) bone marrow edema. The patella is the largest sesamoid bone of the skeleton and is formed and located in between the quadriceps femoris and patellar tendons . Its priA 16-year-old otherwise healthy male  was referred to the orthopaedic department because of bilateral knee pain and clunking. These symptoms occurred during running and other forceful movements, were less but not absent with rest, and had been present for more than 1 year. There was no history of trauma. Physiotherapy was reported to be not effective. Other than pain on palpation of the inferior pole of the patella on both sides, the physical examination was unremarkable . Radiographs of both knees showed a lytic and round lesion with well-defined margins in the subchondral regions of the superolateral quadrant of both patellae, in keeping with bilateral dorsal patellar defects Fig. . MRI of The dorsal patellar defect was first reported by Caffey and Keats in the early 1970s , 11. A sDespite the fact that the phenomenon of the dorsal patellar defect has been known for over 40 years, its origin still remains unclear. Different hypotheses have been postulated. Since the dorsal patellar defect is located in the superolateral quadrant of the patella, similarly to the multipartite patella, and previous studies have reported cases in which both entities coexist , 7, it iThe diagnosis of dorsal patellar defect is generally straightforward. Because of its typical imaging features and location, it can usually be differentiated from pathologic conditions such as giant cell tumor, chondroblastoma, aneurysmal bone cyst, osteomyelitis, osteoid osteoma, solitary bone cyst, intraosseous gout, metastasis, intraosseous ganglion, and brown tumor in hyperparathyroidism . In addiIn conclusion, this report described a rather unusual case of bilateral dorsal patellar defects with cartilage involvement and extensive surrounding bone marrow edema as demonstrated by MRI. The latter two should be considered as part of the spectrum of associated MRI findings that can be encountered in this entity. Furthermore, the presented case shows that symptomatic dorsal patellar defects can be treated conservatively with success and that (decrease of) pain symptoms are likely related to (decrease of) bone marrow edema.


Partial segmental thrombosis of the corpus cavernosum (PSTCC) is a rare disease predominantly occurring in young men. Cardinal symptoms are pain and perineal swelling. Although several risk factors are described in the literature, the exact etiology of penile thrombosis remains unclear in most cases. MRI or ultrasound (US) is usually used for diagnosing this condition.We report a case of penile thrombosis after left-sided varicocele ligature in a young patient. The diagnosis was established using contrast-enhanced ultrasound (CEUS) and was confirmed by contrast-enhanced magnetic resonance imaging (ceMRI). Successful conservative treatment consisted of systemic anticoagulation using low molecular weight heparin and acetylsalicylic acid.PSTCC is a rare condition in young men and appears with massive pain and perineal swelling. In case of suspected PSTCC utilization of CEUS may be of diagnostic benefit. Partial segmental thrombosis of the corpus cavernosum (PSTCC) is a rare disease predominantly occurring in young men . PriapisMRI or ultrasound (US) are usually used for diagnosis . This reA 23-year-old male was admitted to our university hospital with severe pain and a history of progressive perineal swelling over a period of two weeks. His medical history was non-contributory except a left-sided varicocele ligature performed six years ago. No sexual trauma, dysuria or urethral discharge was reported by the patient. Excessive bicycle riding, masturbation, marijuana or cocaine consumption as well as recent long distance flights were negated.Physical examination confirmed the absence of priapism. A firm, fixed and extremely painful mass was palpable at the proximal penis. The complete blood count, electrolytes, coagulation profile, CRP and urine analysis were unremarkable.Gray-scaled ultrasound  combined with color Doppler of the penis using a 6 MHz transducer  revealed an elongated hypoechogenic mass  revealed a lack of contrast enhancement in the hypoechogenic mass. This was interpreted as segmental thrombosis of the corpus cavernosum Figure , arrow.For confirmation of this diagnosis a MRI study  of the pelvis was performed. Transversal T2-weighted images of the pelvis showed a hypointense thrombus of the right proximal corpus cavernosum  and acetylsalicylic acid (100 mg once daily).Partial segmental thrombosis of the corpus cavernosum (PSTCC) is a rare urological disease which primarily occurs in young men with a mean age of 30 years (range 18–59 years) .PSTCC´s etiology is poorly understood. Although several risk factors have been described, the exact etiology still remains unclear. Recently Ilicki et al.  postulatRisk factors for development of PSTCC include haematological abnormalities , abuse of alcohol or drugs , hypercoagulability associated with malignancy and long distance flights as well as microtrauma after coitus or extended cycling –6.Standard imaging modalities include color coded duplex sonography, MRI and CT imaging. The benefits of contrast enhanced MRI have been demonstrated by several authors , 6, 11. This case report outlines the utility of CEUS as an additional cost-saving diagnostic tool for assessment of PSTCC, especially in patients with contraindications for MRI.The first cases of PSTCC were described in 1976 . At thatPSTCC is a rare condition that predominantly occurs in young men and is accompanied with massive pain and perineal swelling. There are several risk factors described, including abuse of alcohol or drugs, hypercoagulability associated with malignancy and long distance flights as well as microtrauma after coitus or extended bicycle riding. CEUS may be of diagnostic benefit if one suspects PSTCC. Larger studies are warranted to verify this hypothesis. Conservative treatment with systemic anticoagulation has shown excellent results concerning pain relief and erectile dysfunction.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Endodermal sinus tumors are rare malignant germ cell tumors that usually originate from the gonads and are rarely observed extragonadally. Pure primary endodermal sinus tumors of the cerebellar hemisphere are extremely rare and patients diagnosed with the disease often have a poor prognosis. The symptoms of YSTs are unspecific and associated with the location of tumors. Intracranial YSTs (such as cerebellar hemispheres) always present with symptoms including headache and poor vision. The present study reports the case of a three-year-old male who presented to The First Affiliated Hospital of Nanchang University  with a headache that had persisted for one month, and then worsened for the last 10 days. This was accompanied by vomiting and gait disturbance. An abnormal signal mass was identified in the left cerebellar hemisphere on brain magnetic resonance imaging. The case initially presented as a medulloblastoma and the patient was followed up for six months. The final pathology report revealed an endodermal sinus tumor, also known as a yolk sac tumor. Six months following resection of the left cerebellar tumor, the patient succumbed to recurrence of the disease, due to acute vomiting and severe headache. At present, no studies have analyzed the total incidence of yolk sac tumors (YSTs), however, it has been reported that YSTs most commonly occur in the pediatric testis . PediatrA three-year-old male presented to The First Affiliated Hospital of Nanchang University  with a headache that had persisted for one month, and then worsened for the last 10 days. This was accompanied by vomiting and gait disturbance. The remainder of the patient’s physical examination and medical, family and surgical histories were unremarkable. At the time of presentation, routine laboratory tests, including a routine blood examination and coagulation indices, were within the normal ranges. Serum tumor markers, including β-human chorionic gonadotropin and α-fetoprotein (AFP), were not measured, as a diagnosis of GCT was not suspected at this stage.For further investigation, the patient was referred to the Department of Radiology for brain magnetic resonance imaging (MRI). The imaging revealed an abnormal signal mass in the left cerebellar hemisphere , but no A resection of the left cerebellar tumor was performed. The intraoperative findings revealed a well-defined 4.0×3.0×2.5-cm tumor, with a red and white appearance, an inconsistently soft texture and a rich blood supply. The resected tumor was a solid mass, and the cut surface exhibiteda friable, white-to-tan appearance. The resected specimen was characterized histologically by diffuse, malignant, neoplastic cells proliferating in a microcystic or reticular pattern of growth around the blood vessels or cavity. The neoplastic cells, forming Schiller-Duval bodies, exhibited highly atypical, large nuclei, with evident karyokinesis and eosinophilic bodies that partly existed in the cytoplasm and partly in the extracellular matrix . ImmunohFollowing surgery, the patient’s symptoms were relieved for a while. However, subsequent to one month, MRI of the brain revealed tumor recurrence in the same region . After aEndodermal sinus tumors are rare malignant GCTs that usually originate from the gonads and are rarely observed extragonadally . SeveralThe histogenesis of extragonadal YST remains controversial. There are currently two theories that may explain the occurrence of primary GCT at extragonadal sites; the first is the aberrant differentiation of somatic cells, while the second is the malignant transformation of remnant of germ cells along the pathway of migration ,7. The lThe typical morphological structure of the tumor, known as the Schiller-Duval body, is a glomerulus-like structure composed of a monolayer of cubic or columnar neoplastic cells wrapping around the capillaries, thin-walled blood sinus or small venous blood vessels. This forms vessel-centered, sleeve-shaped structures, similar to glomerulus-like structures ,13.In the present case, the patient had initially been diagnosed with a medulloblastoma, however, the post-operative pathology confirmed the lesion to be a YST, indicating that caution must be taken when conducting initial examinations in order to avoid misdiagnosis. Initially, the case was consistent with a medulloblastoma with regard to the age distribution and predilection site, and due to its rarity in the cerebellar hemisphere, primary YST was not considered. Furthermore, the clinical and imaging manifestations of YST are not specific. The clinical symptoms are associated with the location of the tumor, whereas the final diagnosis mainly depends on the pathology. Notably, the present patient did not receive the pre-operative tests associated with YST, particularly the test for serum AFP. To the best of our knowledge, the serum AFP levels in YST patients are likely to significantly increase when these types of tumors contain YST elements. It has also been confirmed that an increased AFP level in the serum and cerebrospinal fluid correlates with the presence of a YST tumor . In the Patients diagnosed with YST generally have a poor prognosis ,16, and In conclusion, the current study presents an extremely rare case of primary YST originating in the left cerebellar hemisphere. Extragonadal YST is aggressive and difficult to diagnose, and further investigations are required to define its pre-operative diagnosis and to further optimize the treatment regimen.