Warthin’s tumor or cystadenolymphoma (CAL) is a benign salivary gland tumor occurring almost exclusively in the parotid gland. CALs of other locations are rare.We report a laryngeal CAL detected in a positron emission tomography/computed tomography (PET/CT) performed for breast cancer follow-up. The tumor was successfully treated by transoral surgery.Only 14 cases of laryngeal CAL are reported worldwide. These cases confirmed our experience of an uncomplicated and mostly successful transoral resection.CALs of the larynx are very rare. They are characterized by hypermetabolism in PET/CT. The increasing use of PET/CT investigations in cancer patients could give rise to more incidental findings of CALs at unusual locations such as the larynx. Laryngeal tumors are predominantly malignant, with squamous cell carcinoma being the most frequent histopathological entity. Benign tumors represent around 5 % of all laryngeal tumors, and most of these are papillomas (more than 80 %).th decade of life, have a male predominance, and are more common in smokers. In general, salivary gland tumors of the larynx are rare and malignant forms prevail. Benign salivary gland tumors of the larynx are very rare and therefore poorly documented, despite claims that oncocytic lesions occur more frequently than previously thought , and with available cross-linked references from the articles found. We identified 12 eligible articles that reported 14 cases of true laryngeal CAL, histologically defined by the presence of papillary cystic epithelium and lymphoid stroma (Warthin’s tumor). Available clinical data were extracted from these articles  and summMale to female ratio was 2:13, and patients’ mean age was of 64.2 ± 10.99 years (range: 45–80). The clinical presentation was not disease–but organ-specific, reflecting laryngeal involvement, with hoarseness as leading symptom in 7, dyspnea in 3, and dysphagia in 2 patients. One patient presented with neck swelling. The location was exclusively supraglottic, with glottic involvement in only one of the cases. One case showed a bilateral CAL. Two patients presented CAL within a laryngocele. Tumor size ranged between 1 and 4.5 cm. In 2 cases, synchronous CALs of the parotid gland were seen. Transoral resection was performed in 9 out of 11 patients, and 2 underwent open transcervical resection. In most cases (9 out of 11 reported) one surgical procedure was sufficient to eradicate the tumor, whereas in one case 4 operations were needed for recurrent disease and the second case showed tumoral persistence after 4 months. Our case underwent a second operation due to persistent supraglottic edema with no residual CAL during definitive histopathologic examination. Another 2 cases showed persistent laryngeal edema postoperatively.In summary, laryngeal CAL is very rare and can most often be managed with transoral resection. Recurrence seems to be an exception in the larynx and only few complications or side effects of the treatment are reported. The unique feature of our reported case is that the patient’s CAL was detected incidentally due to regular PET/CT follow-up investigation for a previously treated breast cancer. Only retrospective inquiry showed minor laryngeal symptoms consisting of some hoarseness episodes. Accurate diagnosis requires nonetheless histopathologic examination of the resected specimens. Malignant degeneration of CALs, originating from both the epithelial and the lymphoid compartments, is a rare but possible event. In the specific case of laryngeal CALs, no case of malignant degeneration could be identified.Workup with PET/CT has been shown to indicate false positive results in head and neck cancer patients showing a CAL within neck lymph nodes mimicking a metastasis . There wAnother interesting point is the potential multifocal presentation of CALs. We found 2 cases of concomitant involvement of the larynx and the parotid gland Table  9, 12]., 12.9, 1Warthin’s tumors of the larynx are very rare. Transoral resection seems to be feasible for most cases, has little side effects, and is most often curative. As Warthin’s tumors show increased FDG-uptake similar to malignant tumors, it is hypothesized that with increasing PET/CT use, the number of Warthin’s tumor case reports outside the salivary glands may augment.The patient has given her consent for the Case report to be published.


Primary hepatic carcinoid tumors are extremely rare neoplasms derived from hormone-producing neuroendocrine cells. It is difficult to make their diagnosis before biopsy, surgical resection or necropsy. A recent publication described only 94 cases of these tumors. There is no sex predilection and apparently it has no association with cirrhosis or preexisting hepatic disease. The most effective treatment is hepatectomy, and resection is determined by size and location of the lesions. Only 60 cases had been described up to 2008 and 94 cases were reported in a recent publication. Carcinoid tumors were first reported by Lubarsch, in 1888, and are functional neoplasms that can develop in any part of the body. However, approximately 54% to 90% of the cases originate from the gastrointestinal tract,,7 mainly from the appendix, small bowel and rectum.The liver is the organ most often affected by metastases of neuroendocrine tumors, There is no sex predilection and the tumors affect individuals aged between 40 and 50 years. The cases reported present large tumors in the hepatic parenchyma, with non-specific and mild symptoms. Due to fast progression of the tumor and delay in making diagnosis, very few cases are surgically resectable.The primary hepatic carcinoid tumors are neoplasms derived from hormone-producing neuroendocrine cells, especially serotonin. Since they are very rare, it is difficult to make their diagnosis before biopsy, surgical resection or even necropsy. The mutating cells originate from the ectopic pancreatic or adrenal tissue in the hepatic parenchyma, or from neuroendocrine cells located in the intrahepatic biliary epithelium. Moreover, it was proposed that the chronic inflammation of the bile canaliculi can cause intestinal metaplasia, which predisposes the development of neuroendocrine tumors. The primary hepatic carcinoid tumor has no association with cirrhosis or preexisting liver disease and accounts to only 0.3% of the cases of neuroendocrine tumors.The primary carcinoid tumor of the liver was first described by Edmonsdson, in 1958, and its origin remains uncertain.A 34-year-old male patient referred abdominal pain that was intermittent, progressive, related to compression on the right flank and irradiating to the dorsum and ipsilateral shoulder, for three months. He presented postprandial fullness, constipation and weight loss (3% of body weight).The laboratory test results showed normal complete blood count and liver function tests, negative serology for hepatites, slight increase in canalicular enzymes and tumor markers within normalcy. Abdominal ultrasound showed a heterogeneous mass in the right hepatic lobe measuring 12x10cm, with a solid-cystic content. Computed tomography demonstrated enlarged liver due to the right hepatic lobe that presented an extensive infiltrating lesion with poorly-defined contours, a liquefied center and the periphery was partially enhanced by contrast medium. There was no further involvement of other organs  and B.® that specifically demonstrated a primary hepatic carcinoid tumor . This clinical picture is very rare, and there are only two cases described in the medical literature.,14Primary hepatic carcinoid tumor may be an incidental finding or it may present with abdominal pain, jaundice, a palpable mass in the upper right quadrant of the abdomen, weight loss, Cushing syndrome and carcinoid syndrome. Further workup with computed tomography generally corroborates the ultrasonographic findings. It is difficult to make a definite diagnosis primarily due to its similarity to hepatocellular carcinoma. Finally, OctreoScan® is very useful and its specificity achieves up to 83%.The investigation should first include an abdominal ultrasound, which often demonstrates a solid-cystic tumor. In the present study, immunohistochemistry correlated with the literature and showed positive chromogranin in 89.1%, and synaptofisin in 55% of the cases.Histopathology reveals a malignant neoplasm, with neuroendocrine differentiation and eight mitotic figures per high-power field, but it is not specific for diagnosis of primary hepatic carcinoid tumor, according to the literature. Survival rate may increase from 29% to 78%. Systemic chemotherapy or chemoembolization showed uncertain results. The indication of liver transplant remains a dilemma, and some researchers suggest this therapy for patients with multiple lesions or in cases of disease with impaired liver function. Another possible treatment is radiofrequency.The most effective treatment is hepatectomy.,18 According to Schwartz et al., the distant metastases are not described when there is no hepatic involvement.Recent studies reported a five-year survival of 74-78% in patients submitted to hepatectomy, and the recurrence rate was approximately 18%. reported 94 cases and described abdominal pain as the most frequent symptom (44% of cases), followed by abdominal mass (14.3%) and absence of symptoms (13.1%); unilobar location (76.6%) and single lesion . Hepatectomy was performed in 86.8% of their patients, and overall mortality was 25.5%.Up to 2009, in the largest series published in the literature, Lin et al. are the authors of the largest series from a single center, describing 11 cases of primary hepatic carcinoid tumor within a 13-year period of analysis.Huang et al.Carcinoid tumors with liver metastasis are relatively common. However, the primary involvement of this organ is very rare. Better diagnostic methods are required to make a more precise preoperative differentiation between primary hepatic carcinoid tumor and hepatocellular carcinoma.Primary hepatic carcinoid tumor should be suspected in patients with no chronic liver disease, normal alpha-fetoprotein levels and solid-cystic lesion in imaging tests, associated with diarrhea and abdominal pain. -3 os carcinoides primários desse órgão são extremamente raros, com descrição de apenas 60 casos até o ano de 2008 e de 94 casos em publicação mais recente. Inicialmente reportado por Lubarsch em 1888, os tumores carcinoides são neoplasias funcionais que podem se desenvolver em qualquer parte do corpo. Entretanto, cerca de 54 a 90% dos casos têm sua origem no trato gastrintestinal,,7 principalmente no apêndice cecal, intestino delgado e reto.Apesar de o fígado ser o principal órgão acometido por metástases dos tumores carcinoides, Não apresentam predileção por sexo e acometem indivíduos na faixa etária entre 40 e 50 anos. Os casos relatados se apresentam como tumorações de grandes dimensões no parênquima hepático, com sintomas inespecíficos e frustos, tornando ainda mais raros os casos passíveis de ressecção cirúrgica, devido à rápida progressão tumoral e à demora no diagnóstico.Os tumores neuroendócrinos primários do fígado (TNPF) são neoplasias derivadas de células neuroendócrinas produtoras de hormônios, notadamente a serotonina, que, principalmente por sua raridade, os tornam de difícil diagnóstico antes da biópsia, ressecção cirúrgica ou mesmo da necropsia. As células mutantes originam-se de tecido ectópico pancreático ou adrenal encontrado no parênquima hepático ou, então, de células neuroendócrinas localizadas no epitélio biliar intra-hepático. Além disso, também é proposto que a inflamação crônica dos canalículos biliares pode acarretar metaplasia intestinal, a qual predispõe ao desenvolvimento do tumor neuroendócrino. O TNPF não apresenta associação com cirrose ou doença hepática preexistente e corresponde a apenas de 0,3% dos casos de tumores carcinoides.O TNPF foi inicialmente descrito por Edmonsdson no ano de 1958 e sua origem ainda permanece incerta.Paciente masculino de 34 anos de idade, com relato de dor abdominal há 3 meses, intermitente, progressiva, de caráter compressivo, em flanco direito, com irradiação para o dorso e ombro ipsilateral. Apresentava plenitude pós-prandial, constipação intestinal e perda ponderal de 3% do peso corpóreo.ominal evidenciava uma massa heterogênea em lobo hepático direito de 12x10cm, com componente sólido-cístico, e a tomografia computadorizada, fígado com dimensões aumentadas às custas do lobo hepático direito, apresentando extensa lesão infiltrativa com contornos mal definidos, com centro liquefeito e periferia parcialmente realçada ao meio de contraste, além de ausência de comprometimentos de outros órgãos , geralmente há metástase hepática. Essa apresentação clínica é bastante rara, com descrição de apenas dois casos na literatura.,14O TNPF pode ser um achado incidental ou, então, apresentar-se com sintomas de dor abdominal, icterícia, massa palpável em quadrante superior direito do abdome, perda ponderal, síndrome de Cushing e síndrome carcinoide. A propedêutica adicional com tomografia computadorizada de abdome geralmente corrobora os achados ultrassonográficos. Além disso, ressalta-se a dificuldade em se estabelecer o diagnóstico definitivo devido, principalmente, à sua similaridade com o carcinoma hepatocelular. Finalmente, o auxílio do OctreoScan® é bastante benéfico, apresentando especificidade que chega a 83%.A investigação deve se iniciar com ultrassonografia abdominal, que demonstra, na maioria das vezes, um tumor sólido-cístico. A imuno-histoquímica, no presente estudo, demonstrou correlação com a literatura, evidenciando a positividade da cromogranina em 89,1% e sinaptofisina em 55% dos casos.A histopatologia revelava se tratar de neoplasia maligna, com diferenciação neuroendócrina, com oito figuras de mitose por campo de grande aumento e, assim como dados da literatura, não é específica para o diagnóstico de TNPF. Há possibilidade de aumento de sobrevida de 29% para até 78%. Quimioterapia sistêmica ou quimioembolização mostraram resultados incertos. A indicação do transplante hepático ainda é um dilema, sendo que algumas pesquisas sugerem essa terapia em pacientes com múltiplas lesões ou em casos de doença hepática com função prejudicada. Outra possibilidade terapêutica é a utilização de radiofrequência.O tratamento mais eficaz é a hepatectomia.,18 As metástases à distância, de acordo com Schwartz et al., não são descritas sem envolvimento hepático.Recentes trabalhos demonstraram sobrevida de 5 anos em 74 a 78% dos casos de pacientes submetidos a hepatectomia, com uma taxa de recidiva em torno de 18%. na maior casuística da literatura, reportaram 94 casos e evidenciaram dor abdominal como o sintoma mais frequente (presente em 44% dos casos), seguido por massa abdominal  e ausência de sintomas ; localização unilobar  e lesão única . A hepatectomia foi realizada 86,8% de seus pacientes, com uma taxa de mortalidade global de 25,5%.Até o ano de 2009, Lin et al., são os autores com a maior casuística de um único serviço, com relato de 11 casos de TNPF em 13 anos de análise.Huang et al.Os tumores carcinoides metastáticos para o fígado são relativamente comuns. Entretanto, o acometimento primário desse órgão é bastante raro. Necessita-se de melhora nos métodos diagnósticos para que haja uma diferenciação pré-operatória mais precisa entre tumores neuroendócrinos primários do fígado e carcinoma hepatocelular.Pacientes sem um passado de hepatopatia crônica, com níveis normais de alfafetoproteína e imagem sólido-cística nos exames de imagem, combinadas com diarreia e dor abdominal, devem ter aventada a hipótese de um caso de tumor neuroendócrino primário do fígado.


Here we present our clinical experience in a case of esophagus perforation due to the swallowing of a bone piece causing acute angina pectoris and leading to misdiagnosis of acute coronary syndrome.A 73-year-old Caucasian man underwent urgent coronary angiography with possible diagnosis of acute coronary syndrome. His coronary arteries were found to be normal. A computed tomography examination revealed esophagus perforation by a foreign body (a piece of bone), and he underwent urgent left thoracotomy and the foreign body was removed.Sometimes, even a piece of bone within a meal can lead to esophagus perforation, and injure the pericardium and myocardium. The symptoms of esophagus perforation may be confused with acute coronary syndrome due to their similarities and lack of knowledge about the detailed clinical history as shown in our case. Thus, careful consideration of detailed clinical history as well as choosing an appropriate medical imaging modality, such as computed tomography, should always be kept in mind in order to promptly diagnose and start early treatment to reduce mortality. Esophagus foreign bodies are mostly encountered in childhood. Although they are rarely seen in adults, the rate of mortality and morbidity is high in cases of perforation. Most esophagus perforations are iatrogenic . Perfora3 (4.8 to 10.8mm3), urea 65mg/dL (5 to 23mg/dL), creatinine 4.2mg/dL (0.6 to 1.3mg/dL), total bilirubin 3.5mg/dL (0.2 to 1.2mg/dL), gamma-glutamyl transpeptidase 78U/L (12 to 64U/L), aspartate transaminase 105U/L (5 to 34U/L), alanine transaminase 118U/L (0 to 55U/L), and C-reactive protein 314mg/dL (0.0 to 5.0 mg/dL). A CT examination was performed to exclude aortic aneurysm or dissection. There was pleural fluid in both sides; an air bubble containing pericardial fluid reaching to 2cm thickness was noted. There was minimal free fluid in perihepatic, perisplenic and retrovesical spaces. With these findings and clinical situation, severe mediastinitis was considered, and his detailed clinical history was taken into consideration once again. The patient later told that he had eaten chopsteak (meat on the bone) in his dinner just before the onset of his symptoms. Then the CT images were checked again, and a dense 2cm-long linear foreign body was found in the posterior aspect of his left atrium . A hemogram and biochemical test results were as follows: white blood cell count 17,280/mmThese findings revealed a perforation of esophagus by a foreign body, and the patient underwent urgent left thoracotomy. At surgery, a sharp contoured piece of bone was found, perforating the 1/3 distal aspect of the posterior wall of his esophagus. It reached to the pericardium, penetrating it and eroding his left atrium. The adventitia of the aorta was affected on the left. There was pus in his pericardium. The foreign body was removed Figure . After aEsophagus perforations due to foreign bodies have high mortality and morbidity rates. Diagnosis can be made by detailed clinical history, physical examination and CT. Immediate diagnosis and early treatment are important in preventing high mortality and morbidity in a case of esophagus perforation. The treatment depends on preventing and controlling sepsis and infection, and maintaining the continuity of digestive tract and nutrition. While early intervention performed within the first 24 hours of injury offers favorable outcomes, delayed surgery has increased mortality and morbidity rates . MediastSometimes, a piece of bone can cause esophagus perforation and injure the pericardium and myocardium. Lack of knowledge of detailed clinical history may cause confusion because of the similarity between the symptoms of acute coronary syndrome and those of esophagus perforation. Because of this, the patient’s detailed clinical history should always be checked and an appropriate medical imaging technique  chosen. Early proper diagnosis and treatment may reduce the mortality rate.Written informed consent was obtained from the patient’s next of kin 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.


Heterotopic pancreas is a rare congenital condition characterised by pancreatic tissue lacking vascular or anatomic communication with the normal pancreas. Most cases of ectopic pancreas are asymptomatic. The preoperative diagnosis of this condition is difficult.A 50-year-old woman presented with dyspeptic symptoms of 4 years duration. Contrast enhanced CT (computed tomography) scan of abdomen suggested a gastrointestinal stromal tumour in the fundus of the stomach. The patient underwent laparoscopy assisted resection and subsequent histology revealed ectopic pancreatic tissue.Although heterotopic pancreas is a rare lesion diagnosed on histology, it should be considered in the differential diagnosis of gastric mass lesions and in patients presenting with vague upper gastrointestinal symptoms. Heterotopic pancreas is a rare developmental anomaly with a reported incidence of 0.55–14 % at autopsy , 3. HeteHeterotopic pancreas is usually found incidentally and is generally asymptomatic. However it may become symptomatic when complicated by inflammation, bleeding, obstruction or malignant transformation , 11. TheA 50-year-old woman presented with burning epigastric pain, loss of appetite and associated GORD  symptoms for 4 years duration. She had a history of worsening symptoms of severe dyspeptic symptoms. There was no history of loss of appetite, post prandial vomiting or gastrointestinal bleeding. Previously she had undergone several upper GI  endoscopic examinations at a local hospital for epigastric pain, dyspeptic symptoms and found to have a hyperplastic polyp on biopsy. She gave a history of diabetes mellitus with satisfactory glycaemic control on oral hypoglycaemic drugs. She was on long standing proton pump inhibitors and antacids to relieve symptoms. Physical examination was unremarkable. Her routine laboratory investigations were normal. Upper gastrointestinal endoscopy showed an elevated area of mucosa resembling a sessile polyp in the gastric fundus and an adjacent diverticulum Fig. . On uppeAlthough several theories have been proposed to explain the pathogenesis, the exact causes of heterotopic pancreas are still not recognized. Embryological basis for heterotopic pancreas is believed to arise during rotation of the foregut, when fragments of the pancreas become separated from the main body and are deposited at ectopic sites , 13. GasHeterotopic pancreatic tissue is an incidental rare lesion and gastric fundal site makes it more peculiar and rare. Despite the improvements in the diagnostic endoscopy and cross sectional imaging, it still remains a challenge to differentiate ectopic pancreatic tissue from neoplasms such as GIST. Therefore it prudent to consider it as a differential diagnosis of gastric mass lesions and in patients presented with vague upper gastrointestinal symptoms.We have obtained informed written consent from the patient for publication of this case report and accompanying images.


Paraduodenal hernia is a rare congenital anomaly that arises from an error of rotation of the midgut. The duodenum and the small intestine become trapped in a sac which is lined by the peritoneum, behind the mesentery of the colon, either to the right or left of the midline. It is therfore a rare and potentially life-threatening condition that can cause intestinal obstruction progressing to strangulation and perforation. We report a case of a 55-year-old patient presenting a left paraduodenal hernia diagnosed intraoperatively after being operated on in the emergency setting for acute abdomen. The small bowel was twisted upon its mesentery and was entrapped in a large left paraduodenal space. Fortunately, once the bowel was reduced from the paraduodenal space, the blood flow was reestablished and the small bowel resumed a proper functioning. The mouth of the sac was obliterated by suture opposition to the posterior wall. The patient's subsequent hospital course was uneventful, and he was discharged in satisfactory condition 4 days postoperatively. Paraduodenal hernias are rare types of hernias which result from incomplete rotation of the midgut. They may cause acute abdominal pain, chronic digestive disorders, and nonspecific or mild symptoms such as nausea and vomiting. Therefore, because of its highly variable symptoms and signs, preoperative diagnosis of paraduodenal hernia is not always possible. This may be an incidental discovery at laparotomy or a rare cause of small bowel obstruction progressing to strangulation and perforation. Timely surgical intervention minimizes the mortality and morbidity associated with the acute presentation of this hernia.A 55-year-old patient was presented to the emergency department of our hospital after several hours of severe and constant diffuse abdominal pain. There were no relieving or instigating factors. The pain was so sharp and was followed by non bilious emesis consistent with gastric contents. The pqtient denied any history of weight loss, chronic abdominal pain, or other gastrointestinal symptoms. He was previously asymptomatic with no history of abdominal surgery. On physical examination, the patient was moderately dehydrated with mild tachycardia (96 pulse / min) but had normal blood pressure. The abdominal examination showed signs of peritoneal irritation. The patient had generalized tenderness with epigastric and periumbelical predominance. Laboratory studies were significant for an elevated white blood cell count of 16,400 with a left shift on differential smear. Plain abdominal radiograph showed small fluid levels within bowel walls. The patient was thus taken to the operating room urgently for exploratory laparotomy, which showed dusky but viable small bowel, twisted upon its mesentery and entrapped in a large left paraduodenal space Internal hernias are uncommon cause of intestinal obstruction and occur when abdominal contents are trapped within a compartment of the abdominal cavity. It is a rare condition with an incidence of < 1% of all cases of bowel obstruction and up to 5.8% of all cases of small bowel obstruction . The ovePatients with a left paraduodenal hernia usually present during the fourth to sixth decade of life (which is the case of our patient), the mean age of presentation is 38.5 years . On X-raParaduodenal hernia is a relatively rare cause of acute abdomen. Therefore, its diagnosis is often incorrect or delayed owing to its variable clinical manifestations. Although this congenital anomaly is uncommon, it should be taken into consideration in the differential diagnosis of any patient with small bowel obstruction in the absence of previous abdominal surgery. It is thus important for a medical practitioner to recognize it in order to start appropriate treatment without a delay.


Transomental hernia is the rarest form of internal hernias. Clinical expression of this pathology is ambiguous and diagnosis is often made at complication phase, after irreversible strangulation of the herniated loop. Radiological diagnosis is still difficult and intraoperative exploration usually allows discovering this pathology when patient is operated for acute intestinal obstruction. Treatment is surgical and aims to treat intestinal obstruction and prevent recurrence. We describe the case of a 65 years old male operated for a preoperatively suspected internal hernia; surgical exploration found a transomental hernia trough the greater omentum. Adult's Internal hernias (IH) are rare , 2, they3, blood urea at 0.60 g/l and sodium at 131 meq/l. A standard radiography of the abdomen showed many small and central air-fluid levels, with an abnormal projection of an air-fluid level near to the liver area (We report the case of a 65 years old Algerian male, without any surgical or medical history, except the antecedent of recurrent paroxystic abdominal pain, and constipation ceding quickly and spontaneously without any treatment, explored by colonoscopy and abdominal CT but no organic pathology was found. Patient was admitted this time for abdominal pain, vomiting and permanent constipation evolving since less than 24 hours. At admission time the patient was in good conditions, conscious and cooperating, blood pressure was 100/60 mmhg, physical examination showed an average central abdominal distension with no more abnormality. Biology tests founds, leukocytosis at 12,000 elements/mmver area . An abdover area . The diaver area . To prevIH are rare, the most common form of IH is the para-duodenal hernia, while the TOH is the rarest form and represents 1-4% of all IH, and the TOH has a collet but no hernia sac . DiagnosInternal hernia should be suspected in any patient with antecedent of paroxysmal abdominal pain and hospitalized for acute intestinal obstruction. Despite the advances in radiology, diagnosis is still made during operative time, this is due to the diversity of these hernias, to their misconception and especially to the urgent character of the acute intestinal obstruction forcing surgeons to operate as soon as possible and not having time to ask for modern radiology and appreciate its contribution.


Background. Alveolar soft part sarcomas (ASPS) of the head and neck are rare, aggressive soft-tissue malignancies. This study describes the clinical course and management of two patients presenting with ASPS in very rare head and neck locations, the larynx and parotid gland. Methods. We identified two patients presenting with ASPS of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1987 to 2013. Results. Patient ages at diagnosis were 27 and 39 with presenting symptoms of hoarseness and parotid swelling, respectively. Mean follow-up was 87 months. All patients received surgical resection and adjuvant radiotherapy. There were no recurrences or evidence of distant metastatic spread during the series. Disease-free survival time for the patients was 4 months and 168 months, respectively. Conclusions. Our study suggests that a combined-modality approach is important in the treatment of ASPS of the head and neck even in these rare locations. Continued research into new therapies is necessary to improve historically poor outcomes. Alveolar soft part sarcoma (ASPS) is a rare soft-tissue malignancy constituting less than 1% of soft-tissue sarcomas with only about 25% of those occurring in the head and neck . In the This IRB-approved retrospective case series describes two patients with ASPS of the head and neck treated at the University of North Carolina. Both patients present with very rare locations for head and neck ASPS (the larynx and parotid gland) with only a few known cases reported in the literature , 9–11. TA 27-year-old male presented to our hospital with a 3-year history of hoarseness. He reported that the hoarseness had been worsening over the past few months and was newly associated with increased throat clearing and an occasional eating-associated cough. The past history was otherwise unremarkable.Head and neck physical examination was unremarkable without evidence of masses, lesions, or cervical adenopathy. Endoscopy was then performed for further assessment. Flexible fiberoptic laryngoscopy revealed a left-sided submucosal mass of the false vocal cord obstructing the view of the underlying left true vocal cord. Normal mobility of the true vocal cords was noted and there was good glottis closure with pronation. The rest of the laryngeal exam was unremarkable.Computed tomography (CT) revealed a 1.5 × 1.2 × 1.5 cm avidly enhancing mass in the anterior left vocal cord . The lesMicroscopic examination of the biopsy specimen revealed a multilobulated spindle cell lesion. Many of the lobules were composed of nests of large polygonal cells containing finely granular eosinophilic cytoplasm and separated by delicate vessels. Based on the structure, the differential diagnosis included ASPS, perivascular epithelioid cell tumor (PEComa), or possibly metastatic carcinoma.Immunohistochemistry then revealed negative staining results for epithelial membrane antigen (EMA), cytokeratin AE1/AE3, Cam 5.2, S-100, HMB-45, Melan-A, and smooth muscle actin, with rare cells positive for desmin. TFE-3 then showed diffuse nuclear positivity. These results narrowed the differential to ASPS or PEComa. To confirm the diagnosis, FISH analysis was then performed to evaluate for TFE-3 gene rearrangement. A characteristic feature of ASPS is the unbalanced TFE-3 translocation; however, in this case, the translocation was determined to be balanced, even on repeated analysis. Given the observed morphology and gene rearrangement results in the limited biopsy sample, PEComa was the preliminary diagnosis.The patient was consented for excision of the mass via an endoscopic left vertical hemilaryngectomy excising the left true and false vocal cord. Intraoperatively, all frozen section margins were reported to be clear of tumor; however, on final pathologic analysis, two of the frozen section margins contained residual tumor. Also, now evaluating a larger sample, pathology concluded that the characteristic microscopic appearance and the immunohistochemical results should override the molecular results and made the final diagnosis of ASPS.Laser reexcision was then performed to manage the initial positive margins, resulting in negative margins, and it was determined that no residual tumor was present in the reexcised specimen. Considering the initial positive margins, the narrow surgical field, and the inability to obtain wide margins while sparing the larynx, the multidisciplinary tumor board decided to include adjuvant radiotherapy in the treatment plan to reduce the risk of local recurrence. The patient received radiotherapy (59.4 Gy) at an outside hospital closer to home due to additional family support. He responded well to the therapy, had no complications, and is disease-free 4 months after the completion of treatment.A 37-year-old female presented to our institution with a 2-year history of left cheek swelling. The swelling was located in the region of the parotid gland and was not associated with pain. The past history was otherwise unremarkable.Head and neck physical examination revealed a 2 × 2 cm firm, nontender mass in the left parotid area. The mass did not appear to be fixed to the underlying tissues. No adenopathy was appreciated and the remainder of the exam was unremarkable. Flexible fiberoptic nasal laryngoscopy was then performed for further evaluation, also determined to be unremarkable. CT scan was previously obtained at an outside institution and was not available to report.Fine-needle aspiration was then performed to evaluate the mass. The initial assessment reported the presence of oncocytic cells consistent with an oncocytoma. The patient was then consented for excision of the mass via a left superficial parotidectomy, which was completed with close surgical margins.Microscopic examination of the excised mass revealed a 2.1 cm discrete, possibly encapsulated, tumor consisting of nests and sheets of large, moderately pleomorphic, and polygonal cells bounded by broad fibrous septae with abundant small vessels. The cells showed minimal nuclear pleomorphism, prominent single central nucleoli, and abundant clear to eosinophilic granular cytoplasm. Rare mitotic figures were also noted. The differential diagnosis included oncocytoma, myoepithelioma, melanoma, adult rhabdomyoma, and ASPS.Immunohistochemistry then revealed focal reactivity to desmin with no reactivity for actin, S100, HMB45, keratin, synaptophysin, or chromogranin. This immunophenotype supported the diagnoses of adult type rhabdomyoma and alveolar soft part sarcoma and excluded the others. Periodic acid Schiff (PAS) staining was then performed revealing focal reactivity with some cells showing an intracytoplasmic crystalline material. It was then determined that the PAS positive structure combined with the desmin reactivity was most consistent with ASPS.Since ASPS has a high metastatic rate, especially to the lung, chest CT was performed for staging and ruled out pulmonary metastases. Considering the close surgical margins, she then underwent adjuvant radiotherapy to a total dose of 63.4 Gy. There were no major complications from treatment. Since completion of radiotherapy she has had regular follow-up and has remained disease-free 168 months after the completion of her treatment.Alveolar soft part sarcomas are extremely rare soft-tissue sarcomas, typically occurring in the deep soft tissues of the lower extremities, and are especially rare in the head and neck , 9. ClinFor the diagnosis of ASPS, imaging combined with analysis of the histologic, immunochemical, and molecular genetic features is beneficial. On imaging, the tumor demonstrates low attenuation on noncontrast CT and strong tumor enhancement with contrast administration. TI- and T2-weighted MRI images typically show higher signal intensity than muscle while demonstrating tubular areas of flow voids, representative of rapid blood flow within the tumor , 17. MicDue to the rarity of ASPS of the head and neck, the optimal treatment plan has not been clearly elucidated. Currently, the main treatment of primary ASPS, like most soft-tissue sarcomas, is surgical removal using a wide-local excision with the goal of obtaining negative margins , 7, 9. TThe role of radiotherapy has been controversial. Early studies reported no significant benefit with the addition of radiotherapy , 25. ForConventional cytotoxic chemotherapy has been widely reported to have little benefit in the treatment of ASPS , 25, 27.Despite current treatments, the long-term prognosis for ASPS has remained poor due to the high rate of metastatic disease. Lieberman et al. cited 2-year, 5-year, 10-year, and 20-year survival rates of 77%, 60%, 38%, and only 15%, respectively . SimilarIn conclusion, our study reports two cases of head and neck ASPS in extremely rare locations, the larynx and parotid gland. When presenting in rare locations such as these, diagnosis can be difficult and analyses of the histopathologic, immunohistochemical, and molecular genetic features are beneficial for confirmation. Due to its rarity in the literature, the optimal treatment for ASPS has yet to be clearly elucidated. The most evidence seems to conclude that wide-local excision with adjuvant radiotherapy is the best current therapy, though long-term survival remains poor. Hopefully, continued research into new therapies such as antiangiogenic agents, as well as stringent follow-up for late-metastatic detection, will improve the poor outcomes of ASPS.


Giant cell tumors (GCTs) mainly occur in metaphyses of long bones and are generally considered histologically benign; however, GCTs may be locally aggressive with a high rate of local recurrence and exhibit the potential for distant metastasis. Primary GCT of the clivus is extremely rare and is easily misdiagnosed and, thus, treatment remains controversial. The present report describes the case of a 22-year-old male with GCT located in the skull base originating from the clivus, with the involvement of multiple cranial nerves, which was successfully treated with transnasal transsphenoidal surgery following adjuvant radiotherapy and intravenous bisphosphonate administration. The patient remains symptom free at two years of follow-up. This report contributes to the limited literature regarding GCTs of the skull. Giant cell tumors (GCTs) are considered to be a locally aggressive benign tumors, also known as osteoclastoma, which typically occur in the epiphyses of long bones, particularly the distal femur, proximal tibia, distal radius and proximal humerus. GCT rarely manifests in the skull, accounting for <1% of all GCTs of the bone, primarily involving the sphenoid and temporal bones in the middle of the cranial fossa . At presA 22-year-old male with a six month history of dull frontal headache did not receive any medical treatment or examination as the symptoms were tolerable. However, three days prior to admission, the headache worsened, and was localized to the right side. The patient developed secondary diplopia and facial numbness in the right maxilla area. On ophthalmological examination, the diplopia secondary to the left abducens nerve (CN VI) palsy was observed in the left eye. Examination of the cranial nerves revealed facial paresthesia along the distribution of maxillary (V2) divisions of the right trigeminal nerve (CN V). No abnormalities in vision, visual field, corneal reflexes, hearing or the power of masseters were identified and no papilledema was observed. The remaining motor and sensory neurological examinations, including cerebellar tests, were normal with full cooperation and orientation. Endoscopic nasal examination revealed a soft, friable mass, which bled when palpated in the posterior wall of the nasopharynx top. The laboratory evaluations including, complete blood count, biochemistry, analysis of tumor markers, thyroid and pituitary function tests and endocrinology examinations were normal, and the patient’s medical history was noncontributory. Magnetic resonance imaging (MRI) of the brain demonstrated an extensive soft-tissue density mass with an irregular shape and a clear boundary measuring 4.0×4.68×3.7 cm, involving the clivus, surrounding the cavernous sinuses on both sides and compressing the front of optic chiasm in the sphenoid sinus area of middle fossa. The posterior wall of the nasopharynx top was not involved. The tumor tissue was isointense on T1-weighted imaging (WI), T2WI and fluid-attenuated inversion recovery, and moderate homogenous enhancement was identified on the post contrast scan . Due to The tumor was removed using an endoscopic transnasal transsphenoidal method under general anesthesia. Intraoperative findings revealed a gray, soft, friable, hypervascular mass arising from the clivus and involving the sphenoid and posterior ethmoid sinuses. Consequently, almost total resection included the tumor together with the slopes and bone surrounding the optic canal and all cranial nerves were preserved. Postoperatively, the patient exhibited transient diabetes insipidus.From the postoperative histopathology, the patient was diagnosed with a giant cell tumor. The histopathology revealed the tumor was composed of flaky oval or spindle-shaped mononuclear stromal cells and evenly distributed osteoclast-like multinucleated giant cells. The giant cells contained a variable number of nuclei with an median of 25 . The nuclear features of stromal cells were similar to multinucleated giant cells, with open chromatin, one to two nucleoli and numerous mitotic figures (≤15 per 10 high-power fields). No pathological mitosis was identified .Postoperatively, the patient was administered three courses of intravenous zoledronate  and radiotherapy to reduce the local recurrence caused by subtotal resection of the tumor. The patient received three-dimensional conformal radiotherapy using five coplanar fields and two non-coplanar fields to deliver a total dose of 45 Gy in 25 fractions over five weeks. The dose volume histogram revealed that 92% of the planning treatment volume was receiving 100% of the dose. All critical structures received doses within their limits of tolerance. The patient completed therapy without any significant acute toxicity. Complete regression of the tumor was later confirmed on MRI following treatment.Follow-up has been performed for two years, the patient is clinically asymptomatic and no evidence of recurrence or metastases has been identified by computed tomography (CT) and MRI examination .GCTs are generally considered histologically benign; however, they may exhibits locally aggressive behavior with a high rate of local recurrence of up to 60% if treated purely by intralesional curettage. In adiditon, GCTs exhibit the potential for distant metastasis, mostly commonly to the lung, which occurs in 4% of patients with GCT . The incClinical manifestations are usually in accordance with the site of the tumor. Skull-base GCTs generally present with headache, decreased vision, visual field defect, diplopia, ophthalmoplegia, deafness, endocrinopathy and dysfunction of cranial nerves, most commonly the sixth followed by the third cranial nerve . HoweverX-ray and CT scan of skull GCTs frequently demonstrate an expansive and occasional lytic bone lesions usually without the classical ‘soap bubble’ appearance. MRI clearly demonstrates the soft-tissue extension and association with the surrounding structures. On MRI, GCTs are usually hypointense or isointense on T1-weighted images (WI) and T2WI with contrast enhancement ,11. A siGCTs of the bone originate from the primary mesenchymal stromal cells in the connective tissue of the bone marrow, which expresses the receptor activator of NF-κB ligand that stimulates osteoclast maturation from mononuclear precursors. Histologically, GCTs are primarily composed of mononuclear stromal cells and giant cells. However, histogenesis is controversial as the terms GCT and osetoclastoma imply that the giant cells are responsible for the proliferative capacity of the tumor, however, the mononuclear cells present the true neoplastic component and the multinucleated giant cells exhibit an osetoclast-like phenotype and express histocytic lineage markers. The mononuclear cell presents the true neoplastic component while the multinucleated giant cells exhibit an osteoclast-like phenotype and express histocytic lineage markers . MultiplThe clinical behavior of GCT is unpredictable and, thus, treatment remains controversial. Radical surgical extirpation is the treatment of choice for cranial GCT, which requires complete removal of the diseased bone. However, this may not be possible due to anatomical location or the involvement of vital structures, as observed in the present case, and thus the patient was treated using a minimally intralesional approach. Therefore, the recurrence rate is very high, and the use of adjuvant therapy is invaluable ,15.GCTs were previously considered to be radio-resistant with a potential for sarcomatous transformation following radiotherapy. However, along with the development of modern megavoltage irradiation and precise image guided system, the tumor control rate has significantly improved and the frequency of malignant transformation has reduced. Therefore, radiotherapy is recommended as a postoperative adjunctive therapy particularly for incomplete resection in skull base, with a dose of 45–50 Gy in order to gain a long-term healing . The rolRadiographical and histological grading systems do not predict clinical outcome; however, the extent of surgical resection has been shown to affect prognosis. The majority of recurrences occur within the first two years following treatment, although late recurrences have also been reported and, thus, long-term surveillance is recommended .Giant cell tumors are generally benign, locally aggressive lesions with a potential to metastasize. Primary GCTs of the clivus are extremely rare and only five cases have been reported during the past decade. Imaging examination alone is insufficient for the diagnosis of GCT in the skull-base, and the final diagnosis is dependent on histopathology. Surgical extirpation is the standard treatment for skull-base GCT, and adjuvant radiation must be applied in all cases due to the high rate of local recurrence and since complete resection cannot be achieved. Bisphosphonate administration is also recommended. The present case indicated that the use of subtotal excision via minimally invasive surgery following the administration of intravenous bisphosphonate and adjuvant radiotherapy (45 Gy) results in excellent tumor control after a period of two years’ follow-up. Recurrences usually occur within the first two years following treatment, however, long-term surveillance is proposed.


Echinococcus infestation is endemic.A hydatid cyst of the head and neck is a very rare condition, even in areas where We report a rare case of primary hydatid cyst of the right maxillary sinus in a 40-year-old man. The initial diagnosis of the presence of a cystic mass was the result of physical examination and computed tomography (CT) scan. We resected the cystic mass using the Caldwell-Luc procedure. A definitive diagnosis was confirmed by postoperative histopathologic examination. Hydatid cyst of the maxillary sinus is an extremely rare presentation. However, this condition should be considered in differential diagnosis of cystic lesions of the maxillary sinus. Echinococcus granulosus(E. granulosus), for which humans are an intermediate host  is usually caused by the cestode ate host ,2. The mate host . Hydatid disease occurs throughout the world and is especially common in sheep- and cattle-raising regions of Africa, Australia, New Zealand, India, the Middle East, South America, and the Mediterranean. The incidence of humans infected with hydatid disease is approximately 1–2:1,000, although it may be higher in rural areas of regions that are affected . It mainEchinococcus infestation is endemic  of the face showed a loculated soft tissue density in the right maxillary sinus with erosion of the lateral and medial walls, as well as the floor of the orbit. The soft tissue density involved the orbit and compressed the globe . The appA Caldwell-Luc approach was adopted for excision of the cystic mass .The mass in the maxillary sinus was yellow-white in color and elastic in nature, suggesting parasitic disease. We carefully removed the cystic mass and irrigated the sinus with hypertonic saline a total of three times. The macroscopic appearance of the extracted mass suggested a hydatid cyst. The cyst was enucleated easily, and multiple daughter cysts were found inside the capsule . E. granulosus  with a 2-week interval after the first month was prescribed to the patient for prophylaxis against recurrence. The patient did not show any evidence of recurrence at follow-up 12 months after the operation.Primary hydatid cysts of the maxillofacial region are relatively rare. Of the reported cases in the current literature review, only a few have involved the maxillary sinus ,4,7-11. Most hydatid cysts in the head and neck are asymptomatic, with symptoms depending on the location and size of the cyst. Lesions are characteristically slow growing ,12. A coPlain radiography, ultrasound, CT, and magnetic resonance imaging (MRI) are the most useful diagnostic tools for detection of hydatid cystic lesions of the maxillofacial region . CT and Besides the excellent diagnostic value of imaging techniques in the detection of hydatid cysts, it is sometimes difficult to determine the cystic nature of a radiologically-visualized mass .In addition, it is sometimes difficult to distinguish a hydatid cystic lesion from a simple cystic structure . This isWe also visualized a loculated mass in the right maxillary sinus in the present case, which was suggestive of a cystic structure. In our case, the diagnosis was made postoperatively through histopathologic examination. A hydatid cyst was not considered in our preoperative differential diagnosis. FNA did not cause an anaphylactic reaction or spreading of the cyst. Thus, a hydatid cyst should be considered in the differential diagnosis of cystic masses of the head and neck in, and FNA should be avoided unless hydatidosis is ruled out.Surgery is generally the treatment of choice for hydatid disease unless resection is not possible  ,17. DuriCombination of surgical therapy with imidazole derivatives has been used for the prevention of recurrences ,6. AlthoAlthough maxillary sinus is an extremely rare location for a hydatid cyst, this possibility should be considered in the differential diagnosis of a maxillofacial mass. Imaging techniques are highly beneficial in the primary diagnosis; however, the definitive diagnosis is usually made by postoperative histopathologic examinations. The principal treatment of maxillary sinus hydatidosis is surgery and postoperative medical treatment to prevent recurrence. FNA carries the risk of anaphylactic reactions and it should be avoided when the diagnosis of hydatid cyst is considered.


We report on an extremely rare case of a giant solitary fibrous tumor (SFT) of the mesentery in a 65-year-old male who was admitted to our hospital because of lower abdominal pain and abdominal fullness. Computed tomography demonstrated a well-defined solid mass of 25 × 11 cm located in the lower abdomen, which was completely resected during surgery. Histopathologically, this lesion had a heterogeneous cell population, mainly comprising spindle cells with fibrous collagen proliferation, and various other cell populations exhibiting patternless growth. Immunohistochemically, the tumor revealed strong and diffuse staining for CD34, bcl-2, and vimentin, and a high mitotic index (seven mitoses per 10 high-power fields). We diagnosed this case as an SFT of the mesentery, which is unusual according to a PubMed search that reported only nine such cases. Our case may be the largest tumor reported to date, and only one retrieved case reported recurrence, although the lesion was exceptionally large with deep invasion. Nonetheless, the lesion in our case was larger than that in the reported case of recurrence and invasive to the ileum. Since surgery, there has been no evidence of recurrence. Hence, we propose that a large SFT and high mitotic index may present risk factors for recurrence. Therefore, long-term careful follow-up is necessary in such cases, although our case exhibited few risk factors for recurrence. A follow-up at 12 months after surgery found no indications of recurrence. A solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm which often involves the thoracic cavity , and less frequently soft tissues and other visceral and parenchymal organs, including the central nervous system -3. AlthoA 65-year-old male with no significant personal or familial medical history was admitted to our hospital 12 months after a previous visit for symptoms of abdominal fullness and lower abdominal pain. On clinical examination, routine blood results were normal, although the tumor marker cancer antigen 19-9 levels  were slightly elevated, whereas those of carcinoembryonic antigen were not . Contrast-enhanced computed tomography demonstrated a 25 × 11 cm, well-circumscribed, lobulated, heterogeneous mass in the abdominal cavity, which contained several areas of necrosis and calcification  to ensure complete removal.Macroscopic examination of the tumor demonstrated a 25.5 × 13 × 10 cm, well-defined, firm mass with necrotic foci and a pale cut surface Figure . FollowiA SFT is a rare mesenchymal spindle cell neoplasm, first described by Klemperer and Rabin in 1931 . Howeverhttp://www.ncbi.nlm.nih.gov/pubmed/) shows reports of only nine cases  and the histologic features of malignant behavior are marked by hypercellularity, necrosis, cellular atypia, and a high mitotic index (>4 mitoses per 10 HPF) -19. All Although the natural history of extrapleural SFTs remains unknown, long-term and careful follow-up may be necessary, especially in cases with a large tumor and a high mitotic index . We follHere, we reported an extremely rare case of a mesenteric SFT in which the tumor may be the largest reported to date. The tumor was completely removed during surgery; however, careful follow-up is necessary because cases with a large tumor and a high mitotic index may have malignant potential.Written informed consent was obtained from the patient for publication of this case report and all accompanying images. A copy of the written consent form is available for review by the Editor-in-Chief of the journal.


Mucin-producing urothelial-type adenocarcinoma of the prostatic urethra is extremely rare. These lesions must be differentiated from other mucinous tumors including mucin-producing prostatic adenocarcinoma and metastases from either colonic or bladder primaries.We report here a case of urothelial-type adenocarcinoma arising from the prostatic urethra. The patient is an 81 year-old man with a history of pT1 urothelial cell carcinoma of the bladder status post trans-urethral resection of bladder tumor (TURBT) who initially presented with irritative lower urinary tract symptoms and mucosuria refractory to Flomax and finasteride. A shared decision was made for the patient to undergo trans-urethral resection of prostate (TURP). At the time of surgery, a papillary tumor emanating from the prostatic urethra was found and no urothelial lesions were noted in the bladder. Pathology of the resected prostatic chips revealed an invasive adenocarcinoma with intestinal-type differentiation that stained positive for CK7, CK20, and villin, but negative for PSA, PSAP, uroplakin, and CDX-2. Colonoscopy was normal and CT scan did not show any evidence of colonic lesions nor visceral or lymph node metastases. Thus, the patient was diagnosed with a primary urothelial-type adenocarcinoma of the prostatic urethra.Herein we review the literature regarding this unusual entity, and discuss the differential diagnosis, immunohistochemistry, and the importance of correctly identifying this rare tumor. The most common mucin-producing adenocarcinoma of the prostate gland is a primary prostatic adenocarcinoma with mucin production. The difAn 81 year-old Caucasian man with past medical history of COPD, on home oxygen, presented with mucosuria, urinary frequency, and nocturia. Digital rectal exam demonstrated a 60 cc benign-feeling gland and the patient’s PSA was 0.38. The patient has a history of a 2.5 cm pT1 urothelial cell carcinoma at the bladder dome status post TURBT 19 months prior . He underwent an oncology evaluation including PET CT scan demonstrating no evidence of metastatic disease. Based on this evaluation, the patient declined to undergo exenterative surgery due to concerns for decreased quality of life. A decision was thus made for him to undergo local aggressive trans-urethral resection with adjuvant multimodal therapy including 42 days of Intensity Modulated Radiotherapy (IMRT) and radiosensitizing oral capecitabine chemotherapy (900 mg po qd × 6 weeks). Capeitabine versus a platinum-based chemotherapy was chosen as the patient had several contraindications to platihum-based chemotherapy including hearing loss, CKD, and peripheral neuropathy. His PSA remains low at 0.074.Mucin-producing urothelial-type adenocarcinoma of the prostate is an exceedingly rare diagnosis, and one that is poorly understood thus far. It is thought to arise from precursor lesions (urethritis glandularis and glandular metaplasia) found in the prostatic urethra or prostatic ducts,5. The pIn 1996, Tran and Epstein reported the first two cases of mucinous adenocarcinoma arising from the prostatic urethra. In both cases, the patient presented with obstructive urinary symptoms. The gross pathology of the tumors revealed mucin lakes lined by tall columnar epithelium, one specimen of which contained mucin-positive signet ring cells. Both patients had a negative colonoscopy and cystoscopy to rule out bladder and colon primaries, and both had low PSA values. The patients remained without evidence of disease with short-term follow-up following radical retropubic prostatectomy in the first patient and external beam radiotherapy in the second.Curtis, et al. in 2005 described an additional two cases. One man presented with urinary retention, bilateral flank pain, microhematuria, and renal insufficiency and the other with a nodule on digital rectal exam. Again the patients had a low PSA and a negative cystoscopy and colonoscopy work-up. The first patient underwent TURP for bladder outlet obstruction, and shortly after was found have liver metastases and died from disseminated intravascular coagulation. The second patient was treated with radical prostatectomy with bilateral lymph node dissections, and remained with no evidence of disease for 16 months of follow-up.Osunkoya, et al. in 2007 published 15 cases identified between 1990 and 2006, the largest collection to date. All patients presented with an element of urinary obstructive symptoms, however other symptoms included mucosuria (20%), hematuria (13.3%), and urinary tract caliculi (20%). The PSA remained <1.5 in 9/10 patients throughout follow-up. Treatment included TURP in 7/15 and radical pelvic resection in 8/15, including 5 radical prostatectomies (3 of which underwent adjuvant therapy), 2 cystoprostatecomies, and 1 pelvic exenteration. Among these men, 53% died of their disease at an average of 49.2 months from presentation, and all 8 men undergoing radical resection had evidence of extraprostatic extension, 4 of whom developed metastatic disease to the lungs, liver, pelvic side wall, and/or testes. The two men initially described by Tran and Epstein as having no evidence of disease at short-term follow-up both eventually died of their disease at 13 and 63 months[This large collection of cases encompassed only 0.02% of their consult service for prostate cancer. In addition, this description of cases was the first to truly highlight the aggressiveness of the disease with a clinical course that differs drastically from the common adenocarcinoma of the prostate. In these cases, bone metastases were not present at time of progression, and progression of disease to death was more common and rapid.All case reports thus far have helped to identify the histologic features and the immunohistochemical staining that can help differentiate this disease on pathology. Precursor lesions identified in men include glandular metaplasia of the prostatic urethra (53%) and villous features 47%). All specimens contained mucin pools lined by pseudostratified columnar mucinous epithelium with varying degrees of cytologic atypia. Other less commonly demonstrated patterns include necrosis, signet ring cells, and perineural invasion-4.7%. All sCurtis, et al. identified that in comparison to prostatic adenocarcinoma with mucin production, both urothelial-type adenocarcinoma arising in the prostatic urethra and secondary adenocarcinoma of colonic origin involving the prostate were PSA and PSAP negative and CK20 positive. In addition they proposed that a colonic primary would stain negative for CK7 and 34βE12 (high molecular weight cytokeratin) while a prostatic primary would stain positive. HoweverUsing the work of our predecessors, we were able to diagnose the case reported here with primary mucin-producing urothelial-type adenocarcinoma of the prostatic urethra. We obtained an immunohistochemical profile including PSA, PSAP, CK7, CK20, CDX-2, and villin. This in addition to a negative colonoscopy and CT urogram helped to differentiate between prostatic adenocarcinoma with mucin production from secondary lesions from colon or bladder to suggest a diagnosis of primary urothelial-type adenocarcinoma of the prostatic urethra. As evidenced by previous case reports, making the correct diagnosis in this disease early is crucial. First, it may save the patient multiple invasive procedures and work-up to exclude other primaries. In addition, the distinction from prostatic adenocarcinoma with mucin production is critical, as the disease described here is more aggressive and deadly. It cannot be treated with hormonal therapy and assigning a Gleason score is rendered useless. Continuing to gather data and case reports will be crucial to help determine the clinical behavior and to identify the optimal treatment for this rare and aggressive disease.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 Series Editor of this journal.TURP: Trans-urethral resection of prostate; TURBT: Trans-urethral resection of bladder tumor; PSA: Prostate-specific antigen; PSAP: Prostatic-specific acid phosphatase; CK7: Cytokeratin 7; CK20: Cytokeratin 20; CDX-2: Caudal type homeobox 2.The authors declare that they have no competing interests.EMS wrote the manuscript and made the revisions. JWR performed the pathological evaluation and provided the slides for pathology. HAM, JKP, and AKK treated the patient. HAM obtained written informed consent from the patient for submission of this case report. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/14/39/prepub


Conjunctival melanoma is a rare malignant tumour of the eye. Its diagnosis represents a challenge for general pathologists due to low exposure to ocular biopsies and a broad differential diagnosis. In addition, conjunctival samples are often small and are associated with a high frequency of artefacts due to their processing. Here, we present the first case to date of a traumatic iridial extrusion masquerading as a conjunctival melanocytic neoplasm. An 83-year-old Asian man presented with a conjunctival-pigmented nodule surrounded by an area of diffuse pigmentation. Histopathology revealed in the nodule a well-demarcated lesion composed of spindle shaped melanocytes with thick-walled blood vessels. At higher magnification, the blood vessels were composed of thick walls with collagen fibres in an onion-skin-like arrangement. The histological findings were consistent with extruded iridial tissue. The map biopsies of the flat, pigmented lesion showed melanocytic cell proliferation with dendritic processes restricted to the lamina propria without any epithelial involvement, consistent with ocular melanocytosis. The diagnosis of conjunctival melanocytic lesions is challenging, and non-neoplastic conditions should always be included in the differential diagnosis. Pathologists should correlate clinicopathological findings and be familiar with the normal histology in order to achieve the correct diagnosis. Conjunctival melanocytic neoplasms can be classified into benign neoplasms, such as nevi and primary acquired melanosis (PAM) without atypia; premalignant tumours such as primary acquired melanosis (PAM) with atypia, or malignant neoplasms such as conjunctival melanoma . About 7As with other melanocytic lesions in different parts of the body, the diagnosis of conjunctival melanoma (CM) is a challenge. The main architectural features indicating a malignancy in a conjunctival melanocytic lesion are the same as those found in skin melanoma: asymmetry and the lack of maturation. However, the lack of an epithelial inclusion cyst within the melanoma is specific, but not sensitive, to conjunctival malignancy; in contrast, the presence of an epithelial cyst favours the diagnosis of a nevus. Cytological features are pleomorphism, the presence of nucleoli, and mitotic figures deep in the stroma. In addition, several non-melanocytic tumours are known to masquerade as conjunctival melanoma, which further obscures the diagnosis. Herein, we present a case of a traumatic iridial extrusion mimicking clinically a conjunctival melanoma and histopathologically a blue nevus.An 83-year-old Asian man was referred to the ophthalmology department by an optometrist for the evaluation of an asymptomatic pigmented lesion on the conjunctiva of the left eye. The patient’s medical history included ocular melanocytosis and ocular trauma that resolved spontaneously six months prior to presentation. On examination, a well-defined 10 × 5 mm pigmented nodule was seen on the superior medial conjunctiva of the left eye. The surrounding conjunctiva was also diffusely pigmented superficially. A clinical diagnosis of CM within an area of PAM was made. Excisional biopsy of the nodule and map biopsies from the flat-pigmented lesion were obtained in order to rule out any malignancy.Haematoxylin and eosin (H & E)-stained sections from the nodule and map biopsies from the flat surrounding lesion were examined. Immunohistochemical studies in formalin-fixed-paraffin-embedded tissue were performed against HMB45, Melan-A, and Ki-67.An excisional biopsy from the nodule revealed a well-demarcated melanocytic lesion with thick-walled blood vessels . In someBased on the histopathological and immunohistochemical findings along with the clinical information obtained, the patient was diagnosed with extruded iridial tissue and ocular melanocytosis.It is important to be familiar with the peculiarities in ocular histology when ophthalmic specimens are being reviewed. Exposure to ocular histology is sometimes limited among general pathologists and dermatopathologists. In the present case, a biopsy was requested to rule out conjunctival melanoma; however, the histopathological picture was typical of an iridial tissue and guided us towards the correct diagnosis. The iris is characterised by the presence of dendritic melanocytes within a loose stroma, with surrounding characteristic thick-walled blood vessels . In thisThe differential diagnosis for a pigmented conjunctival lesion is broad, encompassing benign and malignant melanocytic proliferations, as well as non-melanocytic entities. Examples of benign melanocytic lesions are nevi (including blue nevus in this case), ocular melanosis, and PAM without atypia. Conjunctival nevi, similar to their skin counterpart, are circumscribed melanocytic proliferations in the epithelium or stroma of the conjunctiva and tend to occur in the interpalpebral area as yellow-brown nodules with associated epithelial pseudocysts . Blue neIn the map biopsies, the presence of spindle melanocytic cells led us to the diagnosis of spindle cell melanocytic lesion of the conjunctiva, as summarised in There is a group of reactive non-neoplastic conditions, which are clinically referred to as pseudomelanosis and should be considered by the pathologist when reviewing a case with the clinical diagnosis of conjunctival melanocytic lesion. These include infections, foreign bodies, and chronic inflammation. Mycosis has been reported to mimic clinically conjunctival melanoma in a number of cases of dematiaceous fungi due to the presence of melanin in the wall of the fungal elements . ForeignThe differential diagnosis for a pigmented conjunctival lesion is broad. In spindle melanocytic lesions of the conjunctiva, a multidisciplinary teamwork to obtain detailed medical history, perform clinical examination, and interpret tissue biopsies in light of the clinicopathological findings is essential to achieve the correct diagnosis. To the best of our knowledge, this is the first reported case of a traumatic iridial extrusion with associated ocular melanosis, clinically masquerading as conjunctival melanoma arising in a primary acquired melanosis.None.This case was presented at the 55th International Academy of Pathology, October 2014 Bangkok, Thailand.


Typical carcinoid of the trachea presenting as an endoluminal polypoidal mass is a rare occurrence. Herein, we report a case of a 34-year-old female patient who presented with features of central airway obstruction. Flexible bronchoscopy demonstrated a large pedunculated growth arising from the lower end of the trachea near carina which was flopping in and out of the main tracheal lumen and the proximal right bronchus leading to dynamic airway obstruction. Successful electrosurgical excision (using a snare loop) of the polypoidal growth was performed using the flexible bronchoscope itself. The patient had immediate relief of airway obstruction and histopathological examination of the polyp demonstrated features of typical carcinoid (WHO Grade I neuroendocrine tumor). Endobronchial benign tumors of the respiratory tract are rare , 2. CarcA 34-year-old lady presented with history of shortness of breath of four-month duration and shortness of breath used to be worse on lying in the left lateral position (trepopnea). She also complained of dull aching central chest pain and dry cough for the same duration. In addition, she also complained of recurrent seasonal episodes of sneezing and nasal obstruction associated with headache. There was no history of fever, wheeze, and hemoptysis or weight loss. General physical examination revealed expiratory stridor. Respiratory rate was 24/min and oxygen saturation while breathing at room air was 95%. Examination of the respiratory system and rest of the systemic examination was normal.Chest radiograph was normal. In a young female in view of trepopnea and expiratory stridor, intrathoracic  obstruction was suspected and contrast enhanced computed tomography (CT) scan of the thorax was performed. In view of symptoms suggestive rhinosinusitis CT of paranasal sinuses was also performed. CT scan of the thorax showed presence of endoluminal polypoidal growth arising from the posterior wall of the tracheal bifurcation measuring 13 mm in anteroposterior diameter and 13 mm in the transverse diameter . The groFlexible fiberoptic bronchoscopy (FOB) was performed for further assessment. It revealed a large fleshy polypoidal growth with well-defined narrow stalk arising from the posterior wall of the lower trachea . The groIn view of the respiratory distress , a decision to attempt flexible bronchoscopic removal of the polyp was taken. Facility for rigid bronchoscopy was standby, in case there was any difficulty during flexible bronchoscopic removal. Olympus BF-1T-180 video bronchoscope (with a 3 mm working channel) via oral route was used. An electrosurgical snare loop  was used for electrosurgical excision. The snare loop was used to encircle and firmly tighten around the neck of the polypoidal growth and electrosurgical excision was performed. The mass got avulsed from its base completely and stuck on to the snare loop and the same was removed in toto along with the flexible bronchoscope . The proThe histopathology of the tracheal growth was suggestive of carcinoid . A DOTANCarcinoids arise from the Kulchitsky cells disseminated in the bronchopulmonary mucosa . These tTypical carcinoid tumors, which represent 90% of carcinoid lung neoplasms, occur mostly in young patients. Among patients with typical carcinoids metastases to lymph node (5%–15%) and distant sites (3%) are uncommon at presentation. In comparison, atypical carcinoids though rare (0.1%-0.2%) lung tumors, however, lymph node (40%–50%) or distant (20%) metastasis are common at presentation . BagheriCarcinoid tumors, when localized, are primarily treated surgically. With surgical removal the five-year survival is 97% and 78% for typical and atypical carcinoid tumors, respectively . The hisBronchoscopic ablation is a useful and efficacious modality for the removal of endobronchial tumors , 4, 9. TCentral airway tumors are mimickers of bronchial asthma and chest X-ray can be normal; hence, CT should be considered in patients who have symptoms of stridor or trepopnea. Tracheal carcinoid without extension to the mediastinum can be successfully removed using flexible bronchoscopy and electrocautery but needs close follow-up of the excision site for recurrence.


We present a case of a 5th Lumbar Vertebra (L5) spinous process osteochondroma as a rare cause of lumbar pain in an old patient. A 70-year-old male presented with progressive and disabling lower lumbar pain. Tenderness over the central and left paraspinal area of the lower lumbar region and a palpable mass were evident. CT scan showed a mass arising from the spinous process of L5. Marginal resection of the tumor was performed through a posterior approach. The histological study revealed an osteochondroma. After surgery, pain was completely relieved. After one year there was no evidence of local recurrence or symptoms. Osteochondromas rarely involve the spine, but when they do symptoms like pain, radiculopathy/myelopathy, or cosmetic deformity may occur. The imagiologic exam of election for diagnosis is CT scan. When symptomatic the treatment of choice is surgical resection. The most concerning complication of osteochondromas is malignant transformation, a rare event. Osteochondroma is a benign outgrowth of bone and cartilage and is one of the most common bone tumors that usually occurs in long bones but rarely involves the spine , affectiWe have found in the literature one case of a symptomatic lumbar osteochondroma presenting in the 6th decade of life . We repoA 70-year-old male, with history of hypertension, dislipidemia, and hyperuricemia, presented to our institution with a one-year long history of progressive and intense lower lumbar pain causing great limitation of daily activities. Physiotherapy or medication was ineffective. The patient reported a palpable mass on this region for years but with neither symptoms nor size progression. He had no constitutional or neurologic symptoms. On examination, there were tenderness over the central and left paraspinal area and a fixed palpable mass of size approximately 7 × 5 cm, hard in consistency, and no pulse. The pain aggravated with flexion, extension, and rotational trunk movements. Neurologic examination was normal. Radiographs showed a bony mass protruding posteriorly, apparently from the L5 vertebra. CT scan showed a 7 cm long well-limited mass with an apparent cartilage cap arising from the spinous process of L5. It was lateralized to the left with adjacent paraspinal muscle compression . Under gOsteochondroma is a benign outgrowth of bone and cartilage and is one of the most common bone tumors that usually occurs in long bones but rarely involves the spine . Only 1,The tumors are thought to arise through a process of progressive endochondral ossification of aberrant cartilage of a growth plate following surgery or fracture or as a consequence of a congenital perichondrial deficiency and are the most common radiation-induced benign tumors , 4, 6. CThe tumor affects mainly the cervical and dorsal spine, probably related to different durations of the ossification processes that occur in the secondary centers of ossification. It can be speculated that the more rapidly the ossification process of these centers develops, the greater the probability that aberrant cartilage will form is. In adolescence, secondary ossification centers, which lie in the spinous process, transverse process, articular process, and the endplate of vertebral body, complete the growth of the vertebral column. These secondary ossification centers appear in children between the ages of 11 and 18 years. They develop into complete ossification in the cervical spine during adolescence and in the thoracic and the lumbar spine during the end of the second decade of life , 13.In most reported cases we have found in the current literature, involving the lumbar spine, the tumor is included in posterior arch elements, more commonly the lamina –10, 14. The tumor can be asymptomatic or symptomatic, either causing pain by pressure on adjacent soft tissue structures when it grows posteriorly, or, more rarely, causing radicular or spinal compression symptoms, when it grows into the spinal canal , 4, 6–9.Marrow and cortical continuity with the underlying parent bone defines the lesion  and thisWhen symptomatic, the treatment of choice of osteochondromas is surgical resection. However, Gille et al.  recommenThe most concerning complication of osteochondromas is malignant transformation, fortunately a rare complication. Chondrosarcoma of the spine represents 4–10% of all chondrosarcomas and 12% of all malignant tumors of the spine ; the freWe report a case of a lumbar osteochondroma arising from the L5 spinous process, a rare cause of lumbar pain, especially in the 8th decade. Osteochondromas rarely involve the spine, but when they occur they can be asymptomatic or cause symptoms, like pain, radiculopathy or myelopathy, or, even, cosmetic deformation. The imagiologic exam of election for diagnosis is CT scan. When symptomatic the treatment of choice is surgical resection. The most concerning complication of osteochondromas is malignant transformation, fortunately a rare event.


Background. The synchronous development of two primary brain tumors of distinct cell of origin in close proximity or in contact with each other is extremely rare. We present the first case of collision tumor with two histological distinct tumors. Case Presentation. A 54-year-old woman presented with progressive atypical left facial pain and numbness for 8 months. MRI of the brain showed left middle cranial fossa heterogeneous mass extending into the infratemporal fossa. At surgery, a distinct but intermingled intra- and extradural tumor was demonstrated which was completely removed through left orbitozygomatic-temporal craniotomy. Histopathological examination showed that the tumor had two distinct components: malignant nerve sheath tumor of the trigeminal nerve and temporal lobe anaplastic astrocytoma. Proliferative activity and expressed tumor protein 53 (TP53) gene mutations were demonstrated in both tumors. Conclusions. We describe the first case of malignant trigeminal nerve sheath tumor (MTNST) and anaplastic astrocytoma in collision and discuss the possible hypothesis of this rare occurrence. We propose that MTNST, with TP53 mutation, have participated in the formation of anaplastic astrocytoma, or vice versa. Collision tumors are defined as two tumors with discrete histology appearing simultaneously and in proximity to each other at the same anatomic location. Such occurrence in the brain is relatively rare and few case reports of primary collision brain tumors were published in the literature, which are summarized in Pituitary adenoma and craniopharyngioma in collision is the commonest association, which was recently reviewed by Jin et al., of 14 reported cases –7. The aWe describe the first report of malignant nerve sheath tumor of the trigeminal nerve in collision with anaplastic astrocytoma of temporal lobe and discuss the possible theory of this rare association.A 54-year-old woman presented with 8-month history of progressive atypical left facial pain and numbness. She had no previous illness, and her elder brother died few years ago from parietal glioblastoma multiforme. General physical examination was unremarkable; there were no cutaneous stigmata of neurofibromatosis. The neurological examination demonstrated facial hypoesthesia in the distribution of the second and third divisions of the trigeminal nerve. The rest of cranial nerves and neurological examination were within normal.Magnetic resonance imaging (MRI) of the brain revealed a 40 × 25 × 40 mm isointense lesion that enhanced heterogeneously following intravenous Gadolinium administration enhancing lesion in the anterior part of left middle cranial fossa involving the anterior part of the temporal lobe and extends to the infratemporal fossa through foramen ovale Figures . The tum P53 positivity  histologically consists of a mixture of plump spindle cells with large hyperchromatic pleomorphic nuclei . Numerouuggested .Total tumor excision is the preferred treatment option in these cases, and radiosurgery is usually given as adjuvant therapy for malignant or residual or recurrent benign tumors . MTNST cThe patient had an uneventful recovery of her symptoms within 2 months from surgery. She refused to receive any adjuvant therapy due to her brother's death from glioblastoma. She presented with acute confusion, 9 months after surgery, and MRI showed local recurrence from both tumors and distant recurrence at the corpus callosum, which was believed from the anaplastic astrocytoma. She died from progressive raised intracranial pressure due to severe tumoral edema. Autopsy examination was not performed in respect to family decision.We herein for the first time report MTNST and anaplastic astrocytoma in collision with similar proliferative activity and TP53 mutation which may have induced the malignant features of the first and may have played part of the proliferation of the latter, or vice versa.


Paragangliomas of the head and neck are rare tumors. Moreover, malignant paragangliomas of the cervical spine are extremely rare. Currently, the combination of curative surgical resection and secondary adjuvant radiotherapy is the gold standard for treating symptomatic malignant paragangliomas. However, traditional treatments for malignant paraganglioma remain unsuccessful. The purpose of this study is to report an exceedingly rare case involving cervical metastasis of a malignant paraganglioma.In this case report, we present a case involving a 72-year-old male with a history of paraganglioma of the neck. He had been experiencing bilateral shoulder pain, neck pain and weakness in the upper extremities for more than six months. Magnetic resonance imaging of the cervical spine revealed a metastasis at C4 with severe vertebral body destruction. To avoid serious complications associated with surgical resection, CyberKnife® radiosurgery  was performed on the parapharyngeal and cervical lesions. A secondary surgery, which involved a posterior laminectomy at C3-6 and posterior fusion at C1-T1, was performed two weeks after the radiosurgery. A histological examination of the surgical specimen demonstrated a malignant paraganglioma. The patient regained strength in all extremities in the postoperative field, and his pain was dramatically reduced. A magnetic resonance imaging study performed three months after the surgery showed a reduced tumor size and spinal cord decompression.This case study is the first report of a patient with symptomatic cervical metastasis of a malignant paraganglioma treated with a combination of radiosurgery and posterior spinal surgery. Although the optimal treatment for these conditions remains unclear, concomitant treatment with radiosurgery and reconstructive surgery appeared to be both safe and effective in this challenging case. Paragangliomas are highly vascular tumors that arise from paraganglia. Of these, head and neck paragangliomas are rare, accounting for about 0.03% of all tumors. MalignaWe report a rare case of symptomatic cervical metastasis of a malignant paraganglioma in a 72-year-old male. Five years prior to presentation, the patient visited another hospital presenting with neck swelling and hoarseness. He was diagnosed with paraganglioma of the neck, but an operation was not planned at that time. Six months prior to presentation, the patient’s chief complaints were bilateral shoulder pain, severe neck pain and weakness of the upper extremities. Because of the continual pain, he was referred to us for a second opinion. A physical examination revealed clumsiness in both hands and muscle weakness in the upper extremities. Cervical roentgenograms revealed a lytic lesion of the C3,4,5 vertebral body. Cervical computerized tomography (CT) revealed destruction of the C3,4,5 vertebral body and C4 lamina. Magnetic resonance imaging (MRI) Figure  demonstrAs the tumor was extremely large and close to the artery and nervous tissue, the head and neck surgeons avoided this treatment route. The conventional anterior approach to the vertebral body was also rendered impossible because of the obstruction of the primary neck tumor. Therefore, CyberKnife® radiosurgery  was employed as the primary treatment for the cervical and parapharyngeal lesions. Even after the radiosurgery, the pain and weakness in the upper limbs persisted. We concluded that it would take some time for the effect of radiosurgery to appear, as his severe neck pain was caused by the destruction of vertebrae. Consequently, a C3-6 laminectomy and C1-T1 fusion using pedicle screw fixation was also performed. The highly vascularized tumor on the C4 lamina was removed and sent to the pathology department. A histological examination , a precursor of noradrenaline, scintigraphy has a sensitivity of 77%–90% and a specificity of 95%–100% in detecting paragangliomas[131I-MIBG sensitivity has been demonstrated in some cases, PET has been found to be superior to 131I-MIBG scintigraphy[18 F-fluorodeoxyglucose (FDG) uptake in the neck and in the C4, L1 vertebral bodies on PET.Paraganglioma can be diagnosed by measuring the catecholamines and metanephrines in the plasma or urine. CT or MRI can identify the location of the paraganglioma. Additionally, ngliomas. HoweverKi-67 index <1% is considered a useful parameter for predicting benign potential[Ki-67 index > 1%, polygonal cells with eosinophilic cytoplasm in a prominent vascular component , and lastly positive staining for chromogranin, synaptophysin and CD56.Pathologically, a otential. In our 131-MIBG remains the only other non-surgical treatment that has produced tumor responses >30%[Currently, the combination of curative surgical resection and secondary adjuvant radiotherapy is the gold standard for treating symptomatic malignant paragangliomas,3. Surgises >30%. Howeveret al. have reported that 97% of patients with paraganglioma achieve tumor control through radiosurgery, the results are comparable to that of surgery[Recently, radiosurgery has emerged as an effective tool for managing paraganglioma. Radiosu surgery,15. Howe surgery. Thus, iIn summary, we report a rare case of symptomatic cervical metastasis of a malignant paraganglioma. Radiosurgery followed by dorsal spinal surgery led to rapid improvement of the paresis and pain. Postoperative imaging findings and clinical outcome revealed the safety and effectiveness of combined surgery and radiosurgery. However, additional follow-ups are necessary to detect local recurrence and the spread of other lesions.Written informed consent was obtained from the patient for publication in 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.CT: Computed tomography; MRI: Magnetic resonance imaging; FDG: Fluorodeoxyglucose; PET: Positron emission tomography; MIBG: Metaiodobenzylguanidine.The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.KS analyzed the treatment and contributed to the final draft of the manuscript. HI planned the treatment, performed the operative procedure, and drafted the manuscript. MS carried out final preparation of the manuscript. SK, TY, TK, and AO planned the treatment and contributed to the final draft of the manuscript. All authors read and approved the final manuscript.


Ectopic production of adrenocorticotropic hormone (ACTH) by neuroendocrine tumours (NET) is a rare condition, occult presentations often hampering the diagnosis. Although NET are relatively frequent in the ileon and Meckel diverticulum, we describe the first Cushing’s syndrome due to ectopic adrenocorticotropic syndrome (CS-EAS) arising from a Meckel diverticulum.99mTc-HYNIC-TOC) scintigraphy revealed increased focalized ileum uptake on the right iliac fossa. Pre-operative ketoconazole and sandostatin treatment controlled the hypercortisolism within a month. Pathological analysis of the resected submucosal 1.8 cm tumour of the Meckel diverticulum and a metastatic local lymph node confirmed a well differentiated neuroendocrine tumour (grade I), whereas immunohistochemistry was positive for ACTH, chromogranin A and synaptophysin. Post-operative clinical and biochemical resolution of Cushing’s syndrome was followed by normalization of both CgA and 5HIAA, which were maintained at the 6 month follow-up.A 44-year-old man was admitted with recent onset of diabetes, myopathy, edema and hypokalemic metabolic alkalosis consistent with Cushing’s syndrome. Both basal and dynamic laboratory evaluation suggested CS-EAS. Laboratory testing also showed high serum levels of chromogranin A (CgA) and urinary 5-hydroxyindoleacetic acid (5HIAA). Pituitary and neck/thorax/abdomen/pelvis imaging proved to be normal, while somatostatin analogue (The identification, characterization and follow-up of this rare cause of ectopic ACTH secretion is important in order to assess the long-term prognostic and management. Cushing syndrome (CS), secondary to ectopic adrenocorticotropic hormone secretion (CS-EAS), was reported initially in 1928, while the link between CS and a nonpituitary tumor as a source of EAS was established in 1962 [Among the variety of tumours, mostly of neuroendocrine origin, associated with CS-EAS –4, 7, 8,We present the first case of an ectopic adrenocorticotropic hormone secretion, from a Meckel diverticulum neuroendocrine tumor.2) over 5 months, moon face, buffalo hump, facial rash, dispersed small bruises on the trunk, generalized muscle weakness and muscle hypotrophy of the lower limbs. The remaining physical examination was normal, including blood pressure levels.A 44-year-old Caucasian man was admitted in June 2014 to the hospital with recent (5 months) onset of ankle oedema, high blood glucose and low potassium levels (1.9 mmol/L). Physical examination showed centripetal distribution of the fat tissue with 10 kg (15 %) weight loss  showed no cortisol suppression confirming the CS-EAS diagnosis Table . Tumour 99mTc-HYNIC-Tyr3-Octreotide (Tektrotyd), previously described as a useful method for diagnosis, staging and follow up of the patients with NETs [Pituitary MRI revealed a pituitary 4 mm adenoma. Thorax, abdomen and pelvis CT did not reveal any tumour, (data not shown) while adrenal glands were hyperplastic. In this context, a whole body scintigraphy using ith NETs , was perThe osteodensitometry showed vertebral osteoporosis with a T-score of −2.9 and Z-score of −2.6, consistent with secondary osteoporosis.After the EAS diagnosis, both ketoconazole (400 mg/d) and sandostatin (100 μgx3/d) treatment alleviated hypercortisolism within a month Fig . For theLaparoscopic abdominal exploration revealed a 12 cm long Meckel’s diverticulum with an irregular shape and a 1.8 cm nodular tumor mass Fig. . DivertiOne month as well as 6 months post-operatively, plasmatic cortisol, ACTH and tumoral markers  were within normal limits Fig. , Table 1–50 % of all small bowel neoplasms, with 72 % survival rates at 5 years for tumours with loco-regional spread [Neuroendocrine tumors in Meckel’s diverticulum resemble to ileal NET in their biological behavior more than to appendiceal NET. It was recently reported that 77 % of Meckel diverticulum tumors present histological characteristics for a neuroendocrine origin, but when compared to ileal tumors, the risk of cancer in the Meckel diverticulum was 70 times higher than any other ileal site . In a rel spread , 18. AloEctopic ACTH syndrome produced by a NET from the gastrointestinal tract is extremely rare and only described in isolated case reports. While only five cases of CS-EAS form appendicular NET were reported in the literature , 19–22, Clinical features of ectopic ACTH syndrome depend on the source of production and rate of ACTH synthesis. Characteristically, these patients have severe hypercortisolemia leading to low serum potassium levels, diabetes, generalized infections, hypertension, and psychosis. Whether rapidly growing tumours, such as small cell lung cancer typically produce profound and sudden onset of symptoms, well defined Cushingoid body habitus changes are noticed in slower growing tumours such as jejuno-ileal NET, as was demonstrated in this patient.The current case is the first report of a Meckel diverticulum NET-producing ACTH presenting clinically as Cushing’s syndrome. The diagnosis was assisted by a positive somatostatin analogue whole body scintigraphy.18F-DOPA-PET-CT or 68Ga-labelled peptides-PET-CT) brought promising results in the localization of jejuno-ileal NET [Localization of EAS tumours is often challenging as occult tumours have been described in 12–19 % of adult patients , 23. Modleal NET , 26.There have been described only two cases of Meckel diverticulum carcinoma with uptake of FDOPA-PET, FDG-PET and somatostatin receptor scintigraphy .Metabolic PET scanning using 18-fluorodeoxyglucose (FDG) was not usually recommended since it has a low sensitivity for jejuno-ileal NET, due to low metabolic activity in G1 tumors. Indeed, the sensitivity of CT and combined somatostatin analogues scintigraphy in the localization CS-EAS was found to be higher (53 %) than that of MRI (37 %) or FDG-PET (35 %) .In the presented case, the somatostatin analogue scintigraphy allowed the localization of the EAS tumour source. Surgical resection was the definitive and optimal management of this rare condition and led to a complete resolution of symptoms.Further studies on more cases would be needed in order to evaluate the best follow-up procedure, as well as the long term progression free survival and outcome for patients with this rare localization of ileal neuro-endocrine tumor.Written informed consent was obtained from the patient. A copy of the written consents is available upon request for review by the Journal Editor.