Diffuse malignant peritoneal mesothelioma and ocular melanoma are both rare tumors. To the best of our knowledge there is only one previous report of three cases in a family with known susceptibility to malignancies associating diffuse malignant peritoneal mesothelioma and ocular melanoma, with no sporadic cases previously reported.We describe the case of a 59-year-old man with a history of diffuse malignant peritoneal mesothelioma, who presented with ocular melanoma 41 months after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. We also briefly review the literature.BAP1 mutations in the pathogenesis of these two neoplasias.Diffuse malignant peritoneal mesothelioma is an uncommon but aggressive disease. As diffuse malignant peritoneal mesothelioma characteristically remains confined to the abdominal cavity, any new extra-abdominal symptom should eventually raise suspicion of another primary tumor. Few cases of diffuse malignant peritoneal mesothelioma associated with other primary tumors have been reported. As ocular melanoma is also infrequent, we suspect a genetic predisposition to these tumors. There is emerging evidence supporting the role of  Diffuse malignant peritoneal mesothelioma (DMPM) is a rare but aggressive tumor arising from the serosal lining of the peritoneal cavity. In industrialized countries, incidence rates of DMPM range between 0.5 and 1.7 cases per million in the general population .We present the case of a patient with DMPM initially treated with this approach, who later developed ocular melanoma (OM). Few other primary tumors have been linked to DMPM. To the best of our knowledge there is only one previous study reporting three patients with DMPM and OM, all in the same family .2 for 30 minutes and systemic 5-FU was simultaneously infused. The findings on the final pathological report were as follows: multifocal and multinodular tumor with numerous psammomatous calcifications, tumor made with polygonal cells showing a tubulopapillary proliferation pattern, cells present on the peritoneal surface and invading the underlying tissue inducing desmoplasia, tumor cells of intermediate size with a moderately abundant eosinophilic cytoplasm, round and slightly irregular nuclei with prominent nucleoli, weak mitotic activity, no intracytoplasmic mucin on periodic acid-Schiff diastase (PAS-D) staining. Immunohistochemical studies have been performed for vimentin, keratin 5/6, calretinin, Wilms' tumor 1 (WT1), carcinoembryonic antigen (CEA), CD15, E cadherin, Ber-EP4 and CD141. Tumor cells were extremely positive for calretinin, keratin 5/6 and focally positive for CD141. In conclusion, the histological aspect and the immunohistochemistry confirmed the diagnosis of DMPM of epithelial subtype.A 59-year-old man sought medical attention in 2005 for vague abdominal discomfort, nausea and belching that had started 6 months earlier. The patient did not have any previous history of cancer in his family or any history of asbestos exposure. He denied any other gastrointestinal or constitutional symptoms. A physical examination was unremarkable except for a slightly distended abdomen. Initial investigations included routine laboratory investigations, an upper gastrointestinal tract endoscopy and a colonoscopy, all within normal limits. However, an abdominal computed tomography (CT) scan revealed omental caking, perihepatic ascites and neoplastic seeding in Morrison’s pouch and the perihepatic space Figure . There wOur patient’s postoperative course was complicated by respiratory failure episodes , a right subphrenic abscess and a lower limb deep venous thrombosis. Recovery was slow but complete and our patient was discharged after 6 weeks. He was then followed with a positron emission tomography-computed tomography (PET-CT) scan every 4 months for 1 year and then every 6 months. At 36 months after treatment (June 2009), a PET-CT scan showed no evidence of recurrence.In November 2009 (41 months after CRS and HIPEC), the patient complained of blurred vision in his left eye and was referred to an ophthalmologist. Fundoscopic examination of the left eye revealed a solid pigmented choroidal mass above the macula measuring 6.9 × 9.2 mm in base and 2.5 mm in height. The patient was then referred to a medical oncology service for systemic staging. MRI of the brain, and a CT scan of the thorax, abdomen and pelvis did not show any evidence of metastatic disease. The medical oncology team assumed that the left eye tumor was metastatic disease from DMPM and external beam radiotherapy of a total dose of 40 Gy (20 fractions of 2 Gy) was administered. Further ophthalmologic assessment showed no treatment response, the size of the tumor increased to 8.1 × 11.3 mm in base and 2.9 mm in height. Two fine needle biopsies were performed but the results were deemed inconclusive. A second ophthalmologic evaluation by a specialized group of ocular oncologists was obtained. On fundus examination, a choroidal melanocytic lesion superior to the macular area was found. The lesion showed different grades of pigmentation, orange pigment on its surface and the presence of subretinal fluid , sarcomatoid and biphasic . Immunohistochemistry is helpful to distinguish DMPM from other peritoneal cancers  . MetastaAlthough there is no commonly accepted standard of care for DMPM, it is well established that CRS and HIPEC therapy has prolonged median survival: 12 months with palliative surgery and/or systemic chemotherapy versus nearly 5 years with CRS and HIPEC [In the investigation of new symptoms in DMPM cases, clinicians must recall that DMPM characteristically tend to recur locally and therefore, extra-abdominal metastases are uncommon. The hypothesis of another primary tumor should be considered despite the few cases reported of DMPM associated with primary malignancies, especially in the context of lacking a history of asbestos exposure as in our case. A series of 500 cases of DMPM reported a 1.8% rate of DMPM associated with other primary tumors . In a muSimilar to DMPM, OM is an infrequent malignancy with an estimated annual incidence of six cases per million. It is the most common primary intraocular malignancy in adults, with the choroid affected in 90% of cases . Known rBAP1 mutation [BAP1 is a tumor suppressor gene located on chromosome 3p21 and the BAP1 protein is involved in DNA damage response, regulation of cell cycle and cell growth. Mutations in BAP1 are infrequent in the general population but high in mesothelioma (20.1%) and uveal melanoma (44.1%) [et al. [BAP1 mutations in 2 of 26 patients diagnosed with mesothelioma and both patients were previously diagnosed with uveal melanoma. Three of their four patients with uveal melanoma and BAP1 mutations subsequently developed mesothelioma. They have suggested a new BAP1-related cancer syndrome characterized by mesothelioma and ocular melanoma, and possibly other malignancies [BAP1 mutations may be at increased risk of developing metachronously both OM and DMPM. Patients found with BAP1 mutations and their families should be closely monitored for the development of any or both of these malignancies for the possibility of early detection and intervention.The specific association between OM and mesothelioma has been previously described in three cases in a family with known susceptibility to malignancies. All cases were found to have a mutation . BAP1 is (44.1%) -15. The Patients with DMPM who undergo CRS and HIPEC can expect a longer life expectancy in comparison to patients treated by other therapeutic modalities. Nevertheless, the association of other primary cancer with DMPM may complicate the management and alter the prognosis for these patients.BAP1-related cancer syndrome since the probability of the occurrence of both malignancies by chance in the same individual is extremely remote. Genetic testing for BAP1 mutations in this patient and his family is currently in progress and the results will be the subject of another publication.To the best of our knowledge, this is the first known case report of DMPM and OM occurring in the same individual with no family history. This article is an important element to support the concept of a clinical correlation between DMPM and OM, probably as a Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.Design and writing: LS, SL. Critical review: VMJP, SER, DP, LG. Data collection: LS, VMJP, SL, DP. All authors read and approved the final manuscript.


A choriocarcinoma is a malignant neoplasm normally arising in the gestational trophoblast, gonads and, less frequently, the retroperitoneum, mediastinum and pineal gland. Primary choriocarcinomas of the renal pelvis are extremely rare.We report a case of primary choriocarcinoma of the renal pelvis in a 38-year-old Greek woman of reproductive age, presenting with a sudden development of intracerebral hemorrhage due to metastatic lesions. The diagnosis was established with a renal biopsy, along with an elevated serum level of beta-human chorionic gonadotropin. An extensive diagnostic work up confirmed the origin of the choriocarcinoma to be the renal pelvis.Extragonadal choriocarcinomas are rare neoplasms that require extensive laboratory and imaging studies to exclude a gonadal origin. Moreover, this is the first case of severe intracerebral hemorrhage as the initial presentation of primary choriocarcinoma of the renal pelvis. Nonetheless, choriocarcinomas should be considered in the differential diagnosis of women of reproductive age. A choriocarcinoma is a malignant neoplasm normally arising in the gestational trophoblast and gonads. Choriocarcinomas of the renal pelvis are extremely rare and they often present in association with transitional cell carcinoma (TCC). Their presenting symptoms are flank pain and macrohematuria. We present a case of primary choriocarcinoma of the renal pelvis in a woman of reproductive age presenting with sudden development of intracerebral hemorrhage.A 38-year-old Greek woman was transferred to the Emergency Department of our hospital in a comatose state. After intubation, an urgent computed tomography (CT) scan of her brain was performed, which revealed a 7.8 × 3.3 × 5.0 cm intracerebral hematoma located at her left occipitoparietal area with surrounding edema and a midline shift.Our patient had a history of two pregnancies that were normal term Cesarean deliveries, the first four years ago and the second 10 months ago. The rest of her medical history was unremarkable.An emergent craniotomy was performed along with evacuation of the hematoma. After surgery, she was transferred to the Intensive Care Unit (ICU). Our patient remained sedated and mechanically ventilated for neuroprotection, while an intraparenchymal monitoring device was inserted for continuous monitoring of her intracranial pressure. Intracranial hypertension was treated with osmotic diuretics, hyperventilation and hypertensive therapy, aiming to maintain a constant cerebral perfusion pressure higher than 60 mmHg.A laboratory workup, including liver and renal function tests, coagulation screening, fibrinogen and full blood count, were normal. Due to the enlargement of the right hilus found on a chest X-ray, extensive CT imaging studies were performed, which revealed a space occupying lesion at the inferior pole of her right kidney (approximately 3 cm), a solitary nodule at the IVa part of her liver consistent with a metastatic lesion, a soft tissue mass situated in the right hilus of her lung enhanced by intravenous contrast and diffusely distributed chest nodules without intrathoracic lymph nodes.Her serum level of β-chorionic gonadotropin (β-hCG) was over 200,000 mIU/mL and this value was repeatedly confirmed. Nonetheless, a pelvic examination was unremarkable and all the imaging studies  revealed the normal appearance of her uterus and bilateral ovaries.During the next two weeks, our patient demonstrated gradual improvement of her general status and she was able to open her eyes spontaneously.Magnetic resonance imaging of her brain showed, apart from postsurgical lesions at the area of the hematoma, a metastatic lesion situated on her left temporal lobe, whereas a magnetic angiography did not reveal any vascular dysplasia.th day of her ICU stay, our patient underwent an ultrasound guided fine needle aspiration of the hepatic lesion in order to establish a histological diagnosis for possible further treatment. Due to consequent intra-abdominal bleeding, she was given surgical radiofrequency ablation of the hepatic lesion. A right nephrectomy was also performed due to the size of the lesion with evidence of imminent bleeding.On the 26th day she had a new episode of massive intracerebral hemorrhage, confirmed by CT, which was considered inoperable by our neurosurgeons. She died 10 days later, due to septic shock.On the 35Macroscopic examination following the right nephrectomy revealed the presence of a grey-red tan encapsulated tumor, which measured 6 × 3.5 × 3 cm, situated at the inferior pole, invading the renal pelvis-pelvicalyceal system and extending to the renal capsule. The histological examination of this mass showed the presence of a malignant neoplasm composed of syncytiotrophoblastic and cytotrophoblastic cells in an extensively hemorrhagic and necrotic background with subsequent cystic degeneration and numerous neoplasmatic vascular emboli Figures  and 2. IChoriocarcinoma is a rare malignant tumor of genital cells that develops usually in the uterus, the ovaries or the placenta of women after molar or, rarely, after normal gestation and in the testis of men. Choriocarcinomas disseminate rapidly through the hematogenous route; the lungs, brain and liver are the distant organs more often involved .Extragonadal choriocarcinomas are scarce neoplasms. They have been described in isolated cases, located in the gastrointestinal system, the lungs, the urinary bladder and the pineal gland ,3.Primary choriocarcinoma of the renal pelvis, as described in this case report, is extremely rare. Slightly more frequent, although rare too, are choriocarcinomas in association with high grade TCC. The cases described in the international literature are very few and their characteristics are highlighted in Table et al. detected the presence of hCG-immunoreactive cells in nine (19%) out of 47 patients with high grade urothelial carcinoma. In only one of those cases was the neoplasm situated in the renal pelvis and the 54-year-old patient had been treated with nephrectomy. Histological examination of this tumor revealed a transitional cell carcinoma with choriocarcinomatous elements [et al. described the case of a TCC with choriocarcinomatous differentiation involving the pelvoureteric junction of the kidney in an 84-year-old man with hematuria [et al. reported β-hCG-positive extragonadal germ cell neoplasia of the renal pelvis in a 56-year-old woman with a history of flank pain on the right side for three years [et al. described the case of a choriocarcinoma of the right kidney in a man presenting with fever, gross hematuria and flank pain [et al. presented the case of a 68-year-old man with hematuria diagnosed with a high grade TCC with choriocarcinomatous component of his left renal pelvis [et al. reported the case of a tumor of the renal pelvis composed of papillary urothelial carcinoma in association with choriocarcinoma, in a 60-year-old male patient presenting with painless macrohematuria. Chromosomal analysis revealed a close genetic relationship between these two carcinomatous components. This led the authors to suggest that the choriocarcinoma may have resulted from clonal evolution of the urothelial carcinoma. As in our case, widespread hepatic and pulmonary metastases were found in this patient, while the β-hCG levels in his serum were elevated [Campo ematuria . Vahlensl pelvis . More reAccording to the predominant theories, extragonadal choriocarcinomas arise from multipotent cells left behind during early embryologic development, from dedifferentiation of neoplastic urothelial transitional cells, or from a metaplastic procedure .Pathologically, the tumors have been described as large and exophytic, often showing hemorrhage and necrosis. In all cases, the presence of choriocarcinoma was indicated by the presence of syncytiotrophoblastic elements. In accordance with previous studies, we observed giant syncytiotrophoblasts with intense β-hCG and placental alkaline phosphatase positive reactivity, while extensive necrosis was a predominant feature.The differential diagnosis between choriocarcinomas of the urothelial system and mixed choriocarcinomas associated with TCC, on histological grounds, is very difficult and it is based on strong expression of β-hCG. Moreover, EMA is positive for TCC but negative for choriocarcinoma .de novo choriocarcinoma of the renal pelvis.Extensive clinical and laboratory research is needed in order for the primary extragonadal localization to be confirmed . In the Interestingly, in our case, there was a marked absence of symptoms from the urothelial system, especially hematuria, which is in contrast with the international literature . Severe Since all choriocarcinomas contain and produce hCG, serum β-hCG values have been used for the diagnosis and follow up of extragonadal localizations . Serum values of β-hCG in our patient were repeatedly over 200,000 mIU/mL.Extragonadal choriocarcinomas are usually diagnosed when they have metastasized. The surgical excision proposed by the literature is performed either for localized disease or for establishing the final diagnosis ,5,7. In We present the case of a 38-year-old woman with primary choriocarcinoma of the renal pelvis and severe intracerebral hemorrhage. To the best of our knowledge, this is the first case of extragonadal choriocarcinoma described that presented as an intracerebral hemorrhage. Clinicians should thus be conscious of this atypical clinical presentation in women of reproductive age.β-hCG: beta-chorionic gonadotropin; CT: computed tomography; EMA: epithelial membrane antigen; ICU: intensive care unit; TCC: transitional cell carcinoma; TTF-1: thyroid transcription factor-1.Written informed consent was obtained from the patient's next-of-kin for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.FK participated in all medical interventions, collected the data and drafted the manuscript. MMV drafted the final version of the manuscript. SB participated in all medical interventions and drafted the final version of the manuscript. MAL participated in the diagnosis. CV participated in the diagnosis. AK participated in all medical interventions and drafted the final version of the manuscript. All authors read and approved the final manuscript.


This is an interesting paper of a 4 cm posttraumatic pseudolipoma on the back of the neck of an adult man who has participated in “tar barrel rolling” since adolescence. To the best of our knowledge, this is the first case of a pseudolipoma to be reported in the literature in association with tar barreling. A 37-year-old healthy man known to have a “tar barreler's hump” in his local community presented to his general practitioner because his hump had become acutely inflamed. The hump has been present for over fifteen years without causing any symptoms. He has participated in “tar barrel rolling”—a family tradition since adolescence. Examination of his back revealed a 4 cm nontender solid soft tissue mass at the posterior aspect of the lower neck in the interscapular region, there was mild erythema of the overlying skin . No otheUltrasound examination  revealed a hyperechoic mass in the subcutaneous tissues of the interscapular region . Due to “Tar barreling” is a world famous tradition native to Ottery St Mary, Devon, Southwest England. The tradition dates back to the 17th century, and it is performed annually on the 5th of November (Guy Fawkes Night). Barrels soaked in tar are set ablaze and carried on the back between the shoulders through the streets. Different categories exist for boys, women, and men depending on the size of the barrels. The event culminates at night with men carrying flaming barrels that weigh up to 30 kg. The festival attracts between 15–20,000 visitors annually. In a few cases, generations of the same family carry these flaming tar barrels annually; men often start “barrel rolling” at a very young age similar to the case presented. Allegedly, there are a few participating residents in the community who have also developed humps at the back of the neck where these barrels are carried over the years. These humps are known in the local community as “tar barreler's hump” and are regarded to be of no serious medical significance. Although there is lack of histological analysis in our case as surgical management was declined, the appearance on ultrasound that is, well-delineated hyperechoic subcutaneous mass with linear echogenic lines perpendicular to the ultrasound, beam and absence of posterior acoustic enhancement or attenuation are typical sonographic appearance of a lipoma or lipoma-like lesion . MRI appPTLs are benign soft tissue tumours that develop in various anatomical regions following acute, chronic or repetitive trauma. The exact pathobiological mechanism of development of PTL is unclear; however various theories via mechanical and inflammatory factors have been postulated. Mechanical factors such as herniation of fatty tissue following traumatic disruption of fascial layers or differentiation of preadipocytes triggered by growth factors and inflammatory mediators such as cytokines released from the preceding haematoma are some of the postulated mechanisms , 4.  TabImaging can be used to differentiate benign lipomatous tumours from liposarcomas –8. The tWe cannot speculate the natural progression of this lesion however malignant transformation into liposarcoma has never been reported as sequelae of PTLs. To the best of our knowledge, this is the first case of PTL in association with tar barrelling to be reported in published the literature.


Brown tumors are erosive bony lesions caused by rapid osteoclastic activity and peritrabecular fibrosis due to hyperparathyroidism, resulting in a local destructive phenomenon. The classical “brown tumor” is commonly seen in ends of long bones, the pelvis and ribs. Facial involvement is rare and, when present, usually involves the mandible. We report a case of 60-year-old male with a rare initial presentation of primary hyperparathyroidism with bilateral maxillary brown tumors. The present case represents the third report of the bilateral maxillary brown tumors in a patient with primary hyperparathyroidism. Differential diagnosis is important for the right treatment choice. It should exclude other giant cell lesions that affect the maxillae. Brown tumor is an osseous lesion that develops in bones affected by primary or secondary hyperparathyroidism, as a component of a metabolic bone disease known as osteitis fibrosa, cystica generalisata or Recklinghausen’s disease of bone. The nameWe report the case of a 60-year-old male who presented with brown tumors of both maxillae. The present case represents the third report of bilateral maxillary brown tumors in a patient with primary hyperparathyroidism. The repoA 60-year-old male reported to our hospital with progressive swelling in the upper left and right jaw since 6 months. The patient noticed a swelling on the right index finger 7 months back. The patient complained of pain in the jaw swellings since 1 week. The past medical history and family history of the patient was noncontributory. On extraoral examination, a diffuse swelling in the left infraorbital and zygomatic region was observed . A swellSylvanus 1743) was the first to diagnose hyperparathyroidism. Recklinghausen (1891) is credited with the first description of the associated bone changes known as osteitis fibrosa cystica.[3 was thePrimary hyperparathyroidism is the uncontrolled production of PTH, usually as a result of a parathyroid adenoma or hyperplasia of glands. Infrequently, a parathyroid carcinoma may be the cause. The boneThe reported prevalence of brown tumors is 0.1%. Brown tuDental changes reported in association with hyperparathyroidism include abnormally large pulp chambers in developing teeth. In erupted teeth, the pulp chambers may appear abnormally large in young patients; however, in adults, it may be narrower than expected in correlation with the patient’s age. The pulp space narrowing was evident in patients with end-stage renal disease. Amelogenet al. (1991) and Felix Jebasinghin (2008). Most patients with hyperparathyroidism are asymptomatic. Hypercalcemia is often discovered incidentally during routine laboratory testing; hypophosphatemia and increased alkaline phosphatase levels in blood may also be seen.[Bilateral maxillary lesions have been reported earlier by Khochtali, Ach, Jlidi, Bouhaouala nghin 200. Most paRadiographically, brown tumors appear as well-defined marginated expansile lytic lesions and may cause cortical expansion. Concurrent bone changes associated with hyperparathyroidism, such as generalized demineralization of the medullary bones of the jaw and loss of lamina dura around the roots of teeth, can help differentiate brown tumors from other processes.10Grossly, a brown tumor appears as a mass with partly cystic and partly solid areas. Microscopically, brown tumors are characterized by intensely vascular fibroblastic stroma serving as a background for numerous osteoclast-like multinucleated giant cells. Cysts deTreatment of hyperparathyroidism is the first step in the management of brown tumor. After appropriate medical or surgical treatment of the underlying endocrine abnormality, brown tumor may regress, and almost all radiographic changes tend to return to normal. An occasBrown tumors of the jaw may commonly involve the mandible and rarely involve the maxilla in association with primary hyperparathyroidism. The presence of underlying primary hyperparathyroidism should be sought in all unexplained mandibular and maxillary lesions.


Background. With this study we focus on the etiopathogenesis and on the therapy of the simultaneous occurrence of Gastric gastrointestinal stromal tumor (gGIST) and adenocarcinoma of the stomach in a patient with Billroth II gastric resection (BIIGR). We report the first case of this event and a review of the literature. Methods. A 70-year-old man with a BIIGR, affected by adenocarcinoma of the stomach, was successfully treated with total gastrectomy. The histological examination showed a gastric adenocarcinoma with a synchronous GIST sized 2 cm and S-100, CD117, and CD34 positive. The mutation of PDGFR gene was detected. Discussion. This tumor is a rare mesenchymal neoplasm of the gastrointestinal tract. Few cases of synchronous gastric adenocarcinoma and GIST are observed in the literature and no case in patients with BIIGR. Various hypotheses have been proposed to explain this occurrence. It is frequently attributed to Metallothioneins genes mutations or embryological abnormalities, but this has not been proven yet. We suggest a hypothesis about the etiopathogenesis of this event in a BIIGR patient. Conclusion. GIST may occur synchronously with gastric adenocarcinoma. This simultaneous occurrence needs more studies to be proven. The study of Cajal cells' proliferation signalling is crucial to demonstrate our hypotesis. They arA 70-year-old man suffered from dyspepsia, chronic anemia, and epigastric pain for about six months, and he also had a weight loss of 15 kg and melena. The patient underwent BIIGR for a gastric ulcer 35 years before. A gastroscopy was performed, and it showed a “polypoid neoformation that occupies one third of the anastomosis and extends along the greater curvature of the gastric stump for about 3 cm.” Biopsies were performed, and they showed “adenocarcinoma infiltrating the stroma and focally the smooth muscle.” Bioumoral findings  were negative. The abdominal computed tomography (CT), executed for the staging of the neoplasia, showed parietal thickening of the gastric stump without secondary repetitions . The patGIST is a rare cancer that originates from Cajal cells, interstitial pacemaker cells , 3, 4; iJust few cases in the literature report GISTs synchronous to gastric adenocarcinomas –14. In oGIST in combination with other synchronous malignant disease is a rare event. The studies reported in the literature about this tumor are still few. Some authors , 22, 23 Ki-67 labeling index assesses the fraction of Ki-67-positive tumor cells and is often correlated with the clinical course of cancer [However, no study highlights if there is a higher GIST preponderance in gastrectomized subjects. In patients with BIIGR in fact, biles' role in the genesis of gastric adenocarcinoma on the anastomotic mucosa is known; there is no evidence so far for an analogous effect for GIST. A common metabolic pathway may be shown through the study of the proliferation signal. It is known that Cajal cell proliferation persists throughout the postnatal period. KIT and Ki-67 signal are observed in the proliferation and development of this kind of cell . Ki-67 pf cancer .We propose that the genesis of these neoplasms can be the overstimulation of the enteric sympathetic plexus as a result of increased gastric clearance. The absence of a parasympathetic stimulus due to vagotomy can lead to the hypertrophy and hyperplasia of Cajal cells, pacemakers of the enteric sympathetic plexus. The study of Cajal cells' proliferation signal in GISTs and in healthy muscular tunica of resected stomachs could demonstrate the etiopathogenesis of these tumors, justifying the synchronism with adenocarcinomas in gastrectomized subjects. This kind of research could provide useful information on the origins of these tumors, but also they could be the starting point for new therapies. Anyway, because of the GIST's low malignant potential, its probability of recurrence is considerably lower than synchronous gastric adenocarcinoma's one. For this reason, incidental GISTs should not be over treated, in order not to invalidate the outcome of surgical and oncological treatment of the primary disease.gGIST in combination with other synchronous malignant diseases is a rare event. GIST's malignancy is considerably lower than gastric adenocarcinoma's one; in this respect, the surgeon has to pay attention to a possible primary GIST, synchronous to other primary malignancies. The coexistence of these neoplasms is an intriguing oncologic model. There is not a unique interpretation of the biochemical mechanisms behind this synchronism, and further studies are needed. Our hypothesis is that in gastrectomized patients there is an overstimulation on Cajal cells, because an increased clearance is necessary. PGFRA mutation affects the treatment of GIST and therefore its prognosis. The GIST overtreatment in terms of survival however could not be effective in case of synchronous neoplasm, because of the worse prognosis of the other tumor. Further studies on the molecular biology of these neoplasms are necessary to detect their biochemical mechanisms; if we could understand the genesis of these synchronisms we also could be able to produce new therapies, such as the biological one.


Introduction. Hepatic metastases of gastric adenocarcinomas are frequently observed due to the drainage into portal vein. Intestinal metastases disseminate from gastrocolic and mesenteric ligaments but they are seen very rarely and in most cases detected in postmortem studies. Case Report. A 74-year-old female patient with no known history of disease. Her complaints on application were epigastric pain, burning, and constipation. Gastroscopy showed a submucosal mass in the greater curvature of fundus and in colonoscopy, a mass with polypoid appearance that narrows the lumen at the rectum was detected. No far metastases or pathology were detected. Pathology report from gastric biopsy material demonstrated well-differentiated adenocarcinoma. Cytokeratin 7 (CK7) was found to be extensively strongly positive, Cytokeratin 20 (CK20) was negative in the immunohistochemical staining of the biopsy obtained from rectosigmoid area. Conclusion. Gastric cancer is among the frequent cancers today, most of which are adenocarcinomas. Although most of the metastases are observed in the liver, lungs, lymph nodes, and peritoneum, it should be remembered that intestinal metastases may be seen without the presence of any other metastatic focus. Our case is the first in literature reporting a rectum metastasis without any other organ metastasis. Hepatic metastases of gastric adenocarcinomas are frequently observed due to the drainage into portal vein. In addition to peritoneal metastases while it is an intraperitoneal organ, other frequent areas of dissemination include lungs due to lymphatic drainage for the tumors adjacent to esophagocardiac junction and intra-abdominal lymph nodes. Intestinal metastases disseminate from gastrocolic and mesenteric ligaments but they are seen very rarely and in most cases detected in postmortem studies .In our case, a rectal metastasis of an intestinal-type gastric adenocarcinoma was detected without any hepatic or lymph node metastases, the first of such case in literature. We aimed in our paper to present this case of rectal metastasis from primary gastric adenocarcinoma without any other metastatic foci by assessment from clinical, radiological, pathological, and genetic perspectives in light of up-to-date literature evidence.The patient, who had epigastric pain and constipation was examined with endoscopy and colonoscopy. In pathological evaluation there was carcinoma both in stomach and rectum. Primary diagnosis was based on two different primary tumors and metachronous metastases. Thorax and abdominal computerized tomography (CT) was performed for other organ metastases. Differential diagnosis was made by the evaluation of the morphology and immunohistochemical staining of the carcinoma in stomach and rectum.A 74-year-old female patient with no known history of disease. Her complaints on application were epigastric pain, burning, and constipation. Gastroscopy showed a 2-cm submucosal mass in the greater curvature of fundus and in colonoscopy, a 2-3-cm mass with polypoid appearance that narrows the lumen at 10 cm in the rectum was detected. No far metastases or pathology were detected in the computer tomography of thorax/abdomen/pelvis, except thickening in the distal rectum wall and gastric wall. Biopsies were taken from the masses in the gastric and rectal areas. Pathology report from gastric biopsy material demonstrated well-differentiated adenocarcinoma .Cytokeratin 7 (CK7)  and musiThe rectosigmoid biopsy material was considered to be an infiltration from the gastric tumor, when assessed in light of morphologic findings and the results of immunohistochemical staining. No positive staining was detected in the cytoplasmic membrane in C-erb B2 immunohistochemical staining (Score 2). There was no amplification in C-erb B2 (Her 2-neu) gene with FISH method. No findings were detected in favor of additional metastatic foci in CT scans of thorax or abdomen other than thickening in the gastric wall and asymmetric thickening in the rectal wall .2, docetaxel 75 mg/m2, and 5-fluorouracil 1000 mg/m2 from day 1 to 5; each cycle performed every 21 days), which is administered to patients with metastatic gastric cancer in accordance with NCCN 2012 guideline. Total neutrophil count was 398 following the 1st chemotherapy. This was considered as neutropenia grade 4 as per Common Terminology Criteria for Adverse Events (CTCAE) Version 4.0, and therapy was resumed decreasing the chemotherapy doses by 25%. No metastatic findings were present and there was regression in constipation in the interim assessment after 3 cycles, and CA 19-9 marker level was decreased to 380 ng/dL. Followup and treatment of the patient is still continuing.Tumor markers CA 19-9 and CEA were found to be 3083 U/mg and 2 ng/dL, respectively. Chemotherapy was initiated at TCF protocol (cisplatin 75 mg/mWhile the frequency of gastric cancers is gradually increased, the estimated incidence of gastric cancer in America was reported to be 21,260, and the number of deaths due to this cause was 11,210 in 2007 . GastricHepatic metastases of gastric adenocarcinomas are frequently observed due to the drainage into portal vein. In addition to peritoneal metastases while it is an intraperitoneal organ, other frequent areas of dissemination include lungs due to lymphatic drainage for the tumors adjacent to esophagocardiac junction and intra-abdominal lymph nodes. Intestinal metastases disseminate from gastrocolic and mesenteric ligaments but they are seen very rarely and in most cases detected in postmortem studies . Rectal Gastroscopy is the most effective method for establishing the diagnosis of gastric cancer. It is highly valuable in diagnosis because it allows direct visualization of the tumor, cytological tests and histological biopsy. In a study issued in 1998, sensitivity, specificity, positive predictive value, and negative prognostic value of gastroscopy was found to be 82%, 100%, 99.1%, and 99.6%, respectively. Taking into consideration all the data, it was found to be 99.6% diagnostic . In a st


Sarcoma of the gallbladder is a rare entity. This report presents an extremely rare clinical case of a neurofibrosarcoma of the gallbladder. On examination, a mass was felt in the right hypochondrium. An ultrasound of the abdomen showed a mass in the gallbladder. Computed tomography (CT) scan of the abdomen showed a grossly distended gallbladder with soft tissue mass in the gallbladder. The mass was diagnosed as carcinoma of the gallbladder and an extended cholecystectomy was performed. Histopathological examination revealed spindle-cell proliferation and the possibility of a malignant tumor of mesenchymal origin. Neurofibrosarcoma of the gallbladder is a rare form of tumor compared to adenocarcinoma and other sarcomatous tumors of the gallbladder . Here, wA 72-year-old female patient had been hospitalized for three days with pain in the right upper quadrant of the abdominal region. History reveals that the patient had experienced spontaneous pain, which increased after eating. The patient did not feel abdominal distension, nausea, vomiting and fever. Physical examination revealed body temperature (TPR) was 36.5 to 37.3C°; the heart rate (HR) of the patient was 78 beats/min and blood pressure (BP) was 120/90 mmHg. In addition, skin and tunica dura of the patient were not stained yellow; superficial lymphoid node was not swollen; abdomen was flat; GI form and peristaltic wave(−) subcutaneous varicose vein of abdominal wall(−) and abdominal muscle was soft. The patient felt light tenderness in the right upper quadrant without rebound tenderness and muscle tonus. Tumescent gallbladder could be palpable and was about 6×6 cm with Murphy sign(+). Liver and spleen were not touched below costal margin. Bowel sound (BS) was 2 to 3 min.Blood counts and liver function were normal. Carbohydrate antigen (CA)19-9, carcinoembryonic antigen (CEA) and α-fetoprotein (AFP) were normal. Ultrasonography B (Ultra-B) showed increased volume of gallbladder, thickening of wall (0.6 cm) and a 6.4×4.9 cm of tumor (6.4×4.9 cm) could be viewed Figure . Soft tiThe liver of the patient showed chronic liver disease alteration but no metabasis nodus. The size of the gallbladder was 12×10×6 cm. The color was gray-red. The capsule wall of the gallbladder was thickened without being touched by the tumor on the plasma membrane. The tumor extended up to the visceral surface of the bottom of the gallbladder. The size was about 8×6 cm with hard texture and less movement. There were no metabasis nodus and swelling of lymphoid nodes on ligament hepatoduodenale, stomach, small intestine, pelvic cavity, abdominal membrane and root of mesentery. The surgical intervention included resection of the gallbladder, partial resection of the fifth segment of the liver and skeletonized dissection of the ligament hepatoduodenale.A gray-red tumor 7 × 4.5 cm in size was removed from the gallbladder. Microscopic examination revealed that neoplastic cells were makr-fusiform shape, few short-fusiform shapes and similar-round shape. The karyotin of the cells was well-distributed, contained few nucleoli, and had light-red cytoplasm with slender cellosilk. The cells were arranged with a fasciculated and weaving appearance. The nucleo-heterology of the cells was quiet, but neoplastic cells were abundant and intensive. The nuclear fission could be found 1 per 2 to 3 high power field , S-100 (+), CD34 (+), Neuron specific enolase (+), CK (−), CD68 (−), CD163 (−), CD31 (−), SMA (−), Masson dyed:blue Figures , 6 and 7Among malignant tumors of the gallbladder, adenocarcinoma accounts for 82%, followed by undifferentiated carcinoma, which accounts for 7%, squamous cell carcinoma for 3%, gland scale mixed cancer for 1% and other uncommon malignant tumors such as lymphatic sarcoma, rhabdomyosarcoma, reticulum cell sarcoma, fibrosarcoma, carcinoid tumor and carcinoma sarcoma [The mass assessed by abdominal ultrasound, CT and/or magnetic resonance imaging (MRI) revealed a huge soft tissue tumor. Laboratory detection of tumor markers, such as AFP, CA19-9 and CEA were usually negative. Patients were treated using multimodal approaches including surgery, chemotherapy and radiotherapy, based on the time of diagnosis. Surgical intervention is the main treatment strategy. Radiotherapy was considered in patients with risk of local recurrence due to micro- or macroscopically incomplete resection. Adjuvant chemotherapy was not administered routinely, but only in some cases with metastasis. Commonly used drugs were doxorubicin, vincristine and cyclophosphamide. The disease could present with lymphatic and hematogenous metastasis. Pretreatment investigations included chest x-ray or chest CT scan, and whole-body bone scan. A 5-year survival rate was about 30%. In this case, the patient has not received any adjuvant therapy so far (after over 6months of follow-up) and there is no evidence of local recurrence and distant metastasis.This report presents for the first time, an extremely rare case of a patient with neurofibrosarcoma of the gallbladder. The condition was managed by an extended cholecystectomy. The case described was a clinical entity that could have been easily misdiagnosed as gallbladder cancer. We think competent imaging and predictive immunological studies could improve the diagnosis of this disease. Moreover, improved surgery and newer methods of adjunctive and neoadjunctive treatment would result in better prognosis for patients with this tumor.Written informed consent was obtained from the patient for publication of this report and any accompanying images.AFP: α-Fetoprotein; BP: Blood pressure; BS: Bowel sound; CA19-9: Carbohydrate antigen (CA)19-9; CEA: Carcinoembryonic antigen; CT: Computed tomography; HR: Heart rate; MRI: Magnetic resonance imaging; TPR: Body temperature; Ultra-B: Ultrasonography B.The authors declare that they have no competing interests.LXF performed the literature review. TK did the immunohistochemistry. All authors read and approved the final version of the manuscript.


A 36-year-old female was hospitalized with symptoms suggesting intestinal occlusion. She was diagnosed with adenocarcinoma of the ampulla of Vater (pT4N0 stage) and underwent cephalic duodenopancreatectomy 8 months ago. Five cycles of postoperative chemotherapy were administrated using capecitabine and oxaliplatin (CAPOX or XELOX), the last one being completed 1 month ago. During the present hospitalization, because of normal computed tomography and ultrasound abdominal examination, rehydration and antibiotherapy were administrated. However, 4 days after hospital admission, the patient died. At autopsy and histological examination, we found a severe myocardial sclerosis with large scarring areas, severe steatohepatitis, chronic pancreatitis with large fibrotic areas, and acute enteritis. Alcohol consumption was denied. The patient died due to associated heart, liver and pancreatic failure. This multiorgan toxicity and death following CAPOX regimen had not yet been reported in the literature.http://www.diagnosticpathology.diagnomx.eu/vs/6472150549833105The virtual slide(s) for this article can be found here:  In advanced gastrointestinal (GI) cancers, an association between cytotoxic drugs and biological agents is usually used. The biological agents such as bevacizumab, cetuximab, and panitumumab seem to be well tolerated and exhibit promising results, but their cost-effectiveness has been seriously evaluated in the literature. Bevacizumab, an antiangiogenic drug, was approved by the Food and Drug Administration (FDA) in 2004 for the treatment of colorectal carcinoma. Cetuximab and panitumumab, the anti-EGFR  agents, can also be used in case of GI tumors that do not display K-ras mutation. HoweverIn most of the GI locally advanced or metastatic tumors, cytotoxic drugs such as irinotecan, oxaliplatin, 5-fluorouracil (5-FU), and capecitabine are used as first-line therapy,2. OxaliIn locally advanced cases, at least two problems should be taken into account. On the one hand, the predictive criteria are not well defined, with the microsatellite status or immunostaining being more valuable as prognostic than predictive factors. On the 2 of oxaliplatin (diluted in a 5% glucose solution) intravenously over 120 min on Day 1, and then 1250 mg/m2 of oral capecitabine administered twice daily from Days 1 to 14, followed by 1-week drug holiday, in a 21-day cycle. The last (fifth) cycle was stopped 1 month before the present admission. The reason for choosing this aggressive regimen was the patients’ age and good pre-chemotherapy general status. There were no significant acute toxicity-related disorders, except slight diarrhea and vomiting, either of which recurred before this episode.A 36-year-old cachectic female (body weight was 41.2 kg and height was 1.62 m), previously diagnosed with adenocarcinoma of the ampulla of Vater that invades the pancreas (pT4N0 stage), which was surgically removed  8 months ago, was admitted to our hospital with pale skin, scleral jaundice, sudden onset of severe abdominal pain and cramping, vomiting, diarrhea, and subfebrility. No alcohol, tobacco, or other drug use was declared. Before and after surgery and also before adjuvant therapy, no comorbidities were diagnosed, and the general status was carefully assessed without modification. Two months following surgery, she underwent five chemotherapy cycles with a combined regimen that included oral capecitabine and intravenous oxaliplatin (CAPOX or XELOX). CAPOX regimen comprised administration of 150 mg/mDuring the present admission, on physical examination, when palpating the abdomen, generalized abdominal tenderness with voluntary guarding was observed, with hypoactive bowel sounds. Considering her symptoms that suggested a tumor relapse, we decided to perform an emergency GI endoscopy that did not evidence tumor relapse or intestinal obstruction. The abdominal-computed tomography and ultrasound were normal. Her serology showed slight anemia , thrombocytopenia , leukocytosis (86.8% neutrophils and 11.5% lymphocytes), and elevated levels of amylase (147 U/L), total bilirubin (6.26 mg/dl), transaminases , and lactate dehydrogenase (484 U/L). Her glycemia was in normal limits (78 mg/dl). Electrocardiogram showed a slightly sinusal tachycardia and T-wave inversion. Adapted diet, bowel rest, antibiotherapy, and intravenous hydration were administrated, but the patient died 4 days after admission.At necropsy, gross and histopathological examination revealed acute non-specific enteritis, chronic pancreatitis with large fibrotic areas and atrophy of the pancreatic parenchyma, and severe steatohepatitis Figure . Most ofA particular histopathological aspect was also observed in the myocardium. Severe diffuse myocardial fibrosis was present in both right and left ventricles, in subendocardial and subpericardial areas Figure . No coroBased on the macro- and microscopic features, it was decided that the death was caused by multiorgan failure syndrome, with the main lesion being the chemotherapy-induced cardiotoxicity. The patient’s age, absence of coronarosclerosis or myocardium hypertrophy, and the associated-hepatic and pancreatic lesions proved that the myocardial disorders had toxic and non-ischemic etiology.Adenocarcinomas of the ampulla of Vater are very rare malignant tumors of the GI tract. They are usually diagnosed in locally advanced or metastatic stages. Being rare tumors, the optimal first-line therapy has not yet been elucidated. One of the commonest first-line chemotherapeutics association is capecitabine in combination with oxaliplatin (CAPOX or XELOX).Capecitabine is a pro-drug of the cytotoxic agent 5-FU which can be orally administrated and can release FU directly at the tumor site due to increased activity of thymidine phosphorylase in tumor cells, when compared with normal healthy ones, and most of the researchers admit that it presents a favorable side-effect profile. HoweverOxaliplatin is a third-generation platinum-based alkylating agent that inhibits DNA synthesis in cancer cells. Its sid3), thrombocytopenia (<100.000 platelets/mm3), and severe anemia (hemoglobin<10 mg/dl) are not eligible, and irinotecan; FOLFOX: Therapetic regimen that associates 5-FU, leucovorin, and oxaliplatin; GEMOX: Therapetic regimen that associates gemcitabine and oxaliplatin; GI: Gastrointestinal.The authors declare that they have no competing interests.SG wrote the manuscript and carried out the study design. IJ coordinated the study design and the draft the manuscript and carried out the interpretation of the data from literature. MC carried out the histological examination and immunoassays. ZK carried out the oncological management and interpretation of oncological data. LA participated in the interpretation of clinical data. CM carried out the surgical intervention and follow-up and supervised the study design. All authors read and approved the final manuscript.


Introduction. Follicular thyroid carcinoma (FTC) frequently metastasizes to the lungs and bones. However, metastasis to the skeletal muscles is an extremely rare manifestation of FTC. To date, only seven cases of FTC have been reported in the literature. Skeletal muscle metastases from FTC usually remain asymptomatic or manifest as swelling and are associated with dismal prognosis. Case Presentation. A 45-year-old Saudi woman presented with right buttock swelling since 8 months. Physical examination revealed right gluteal mass of size 13 × 10 cm and right thyroid lobe nodule. The rest of examination was unremarkable. Magnetic resonance imaging (MRI) showed 13 × 11.7 × 6.8 cm lobulated mass arising from the gluteus medius muscle, and tru-cut biopsy confirmed the metastatic papillary carcinoma of thyroid origin. The patient subsequently underwent palliative radiotherapy followed by total thyroidectomy and radioactive iodine ablation. At the time of publication, the patient was alive with partial response in gluteal mass. Conclusion. Skeletal muscles metastases are a rare manifestation of FTC, and searching for the primary focus in a patient with skeletal muscle metastasis, thyroid cancer should be considered as differential diagnosis. Thyroid cancer is the commonest endocrine malignancy, presenting with 23 500 and 19 000 new cases per year in the United States and the European Union, respectively [Follicular thyroid cancer (FTC) is the second most common histologic type of DTC, and it commonly metastasizes to the lungs and bones. However, metastasis to the skeletal muscles is an extremely rare manifestation of FTC, only few related case reports have been reported in literature the . PrognosHerein, we report a 45-year-old Saudi woman with a solitary metastasis to gluteus medius muscle as an initial manifestation of follicular variant of FTC. A 45-year-old Saudi woman presented in our clinic with painful right buttock swelling and lethargy. She had noticed this swelling for 8 months, and it had been rapidly increasing in size over two months causing pain in sitting posture. Her previous medical history revealed type I diabetes mellitus since last 11 years and hypothyroidism since last 4 years; for those problems, she was taking thyroxin 50 micrograms daily and regular insulin. She had no history of trauma, smoking, and weight loss.On physical examination, her vitals were stable. A fixed hard mass of size 13 × 10 cm was palpable in the right gluteal region. There was a moderate tenderness in the mass without any inflammatory surface, and there was no palpable inguinal lymphadenopathy. Neck examination revealed enlarged nontender right lobe of thyroid gland; however, no palpable cervical lymph nodes were noticed. Examination of chest, heart, nervous system, and abdomen was unremarkable. Differential diagnosis was soft tissue sarcoma or bone tumor. Hematology, serum electrolytes, and liver and renal function tests were within normal limits. Magnetic resonance imaging (MRI) of pelvis showed 13 × 11.7 × 6.8 cm lobulated heterogeneous mass in the right gluteus medius muscle also involving the right gluteus maximus, piriformis muscles extending to the right iliac bone and the right sacroiliac joint. The anteroposterior center of lesion was found within the right gluteus medius muscle; thus, the origin of the lesion was muscular rather than bony . Tru-cutIn multidisciplinary meeting, gluteal mass was labeled unresectable, and patient was referred to us for palliative radiation therapy. Patient received 30 Gy in 10 fractions to the right gluteal mass . After rSkeletal muscle metastasis is a rare entity and differentiation between a primary soft tissue sarcoma and metastatic carcinoma is difficult without biopsy . Most coMetastases of skeletal muscles may manifest as either isolated or with other metastatic sites and usually arise several years from initial diagnosis of FTC; however, in our patient the initial presentation was gluteal muscle metastasis. conventional iodine scintigraphy may remain fail to localize the foci of distant metastasis, because of the lack of anatomic landmarks. MRI, single photon emission computed tomography (SPECT), and positron emission tomography (PET) allow better location , 12. Treatment is surgical resection followed by systemic therapy by RAI ablation or tyrosine kinase inhibitors (sorafenib or sunitinib) in noniodine avid metastasis . PrognosIn conclusion, we have reported a case of FTC with gluteus medius muscle metastasis, which is extremely rare. MRI, SPECT, and PET are helpful to localize the site for metastatic deposits, and immunostaining, whenever possible, shall be incorporated to reach the final diagnosis and prompt treatment.


Angiosarcomas of the small intestine are rare and present non-specifically. They usually manifest with abdominal discomfort, altered bowel habits, anaemia and gastrointestinal bleeding. Diagnosis is often challenging and occurs at an advanced tumour stage. We describe a case of a terminal ileum angiosarcoma masquerading as an appendiceal abscess, and discuss salient clinicopathological features in diagnosing and managing this disease. Intestinal angiosarcomas are rare high grade vascular neoplasms.9/l). C-reactive protein was elevated at 102mg/l. Abdominal computed tomography (CT) demonstrated a 10cm × 6cm × 4cm thick-walled mass adjacent to the caecal pole with a proximally dilated small bowel  and tachycardic (110bpm) on examination, and her abdomen was distended with tenderness and guarding localised to the right iliac fossa. Full blood examination revealed microcytic anaemia  and leukocytosis (white cell count 13.1 × 10ll bowel . No lympThe clinical picture was thought to be consistent with an appendiceal abscess and associated small bowel obstruction. Although CT guided percutaneous drainage of the abscess was proposed, it was decided that an exploratory laparotomy was more appropriate given the increased risk of bowel injury caused by navigating a wide bore needle through the abdominal cavity with distended bowel. On exploration, the appendix appeared uninflamed. However, a large obstructing mural tumour arising from the terminal ileum was identified. Consequently, a 17cm segment of ileum containing the tumour was resected  and a stMicroscopic examination of the resected specimen revealed an infiltrative malignancy involving the bowel wall and mesentery with evidence of venous invasion. Tumour morphology was suggestive of high grade sarcoma , and conet al,Angiosarcomas are high grade vascular neoplasms that account for 1–2% of all soft tissue sarcomas.The clinical presentation of intestinal angiosarcoma is non-specific. Common symptoms include abdominal discomfort, nausea, vomiting and altered bowel habits.Due to the anatomical location of the tumour, CT has limited diagnostic utility. Occasionally, direct tumour visualisation can be achieved endoscopically. However, conventional endoscopy is only useful for tumours located in the stomach, duodenum and colon. For jejunum and ileum tumours, some authors have advocated the use of capsule endoscopy and barium studies but with limited diagnostic success.Our report of a case of intestinal angiosarcoma masquerading as an appendiceal abscess reiterates the variable nature in which this tumour may manifest. Our patient had no history of lymphoedema, toxic exposure or radiation therapy. Her three-week history of right iliac fossa pain, systemic symptoms and signs of sepsis, with localised guarding, and CT evidence of a thick-walled collection adjacent to the caecum supported the provisional diagnosis of an appendiceal abscess. However, this would not explain her microcytic anaemia.Establishing the correct diagnosis in this case was of paramount importance because the management approach, disease progression and prognosis differs vastly between a tumour and an abscess. The initial management of an appendiceal abscess is usually conservative with percutaneous drainage and intravenous antibiotics, followed by a limited right hemicolectomy if the sepsis does not resolve. The outcome of an appendiceal abscess is typically favourable with rapid recovery of the patient. In contrast, early surgical resection and adjuvant chemotherapy are the definitive treatments for angiosarcomas. Unfortunately, despite this, angiosarcomas are universally fatal with an overall survival of less than one year.The pathological identification of an intestinal angiosarcoma can be challenging. Morphologically, it may appear similar to a leiomyosarcoma, a gastrointestinal stromal tumour, a metastatic melanoma, a lymphoma or a poorly differentiated carcinoma. Immunohistological profiling is mandatory to achieve a diagnosis.Even though intestinal angiosarcomas are rare, they should be considered when protracted abdominal pain and bowel obstruction presents in the setting of unexplained anaemia or gastrointestinal bleeding, especially in the absence of previous abdominal surgery. Immunohistochemistry is mandatory to establish a diagnosis of angiosarcoma.


The objective of the present paper is to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of 2 patients with first branchial cleft anomaly. The first case was an 8-year-old girl presented with an elastic lesion located in the left infra-auricular area, in close relation with the lobule, duplicating the external auditory canal. The magnetic resonance imaging revealed a lesion, appearing as a rather well-circumscribed mass within the left parotid gland and duplicating the ear canal. A superficial parotidectomy was subsequently performed, with total excision of the cyst. The second patient was a 15-year-old girl presented with a congenital fistula of the right lateral neck. At superficial parotidectomy, a total excision of the fistula was performed. During the operation the tract was recorded to lay between the branches of the facial nerve, extending with a blind ending canal parallel to the external acoustic meatus. Conclusively, first branchial cleft anomalies are rare malformations with cervical, parotid, or auricular clinical manifestations. Diagnosis of first branchial cleft lesions is achieved mainly through careful physical examination. Complete surgical excision with wide exposure of the lesion is essential in order to achieve permanent cure and avoid recurrence. First branchial cleft anomalies are relatively uncommon congenital malformations of the head and neck, accounting for less than 10% of all branchial anomalies, and can be roughly classified as cysts, sinuses, and fistulae [Clinically they may appear as chronic purulent ear drainage, periauricular swelling in the parotid area, or persistent fistula of the neck and may be present in both adults and children. First branchial cleft cysts of the parotid area can be a significant problem of differential diagnosis. Misdiagnosis has often led to inadequate management of the lesions and to a high percentage of recurrence. Clinical presentation, imaging characteristics, and knowledge of the anatomy and embryology of the area are important in limiting differential diagnosis.The purpose of our paper is to report two cases of first branchial cleft anomalies, diagnosed and treated successfully in our clinic. Additionally, a literature review of the characteristics, the diagnostic evaluation, and treatment options of first branchial cleft anomalies is presented. An 8-year-old girl was admitted to our department with a mass in her left parotid area, which was present for one month and has increased in size over the last 7-day period. On examination a tender mass was palpated in the left parotid area. The lesion was elastic, moderately movable, and mildly painful, located in the left infra-auricular area, in close relation with the lobule and external auditory canal. The patient underwent magnetic resonance imaging (MRI) of the head and neck, demonstrating the anomaly Figures . The lesA superficial parotidectomy was subsequently performed, with total excision of the cyst. During the operation it was confirmed that the cystic mass extended upwards, being in close contact with the cartilaginous part of the external auditory canal, but no communication with the canal or the middle ear was identified . The lesPathological examination of the surgical specimen showed skin, squamous epithelium, and cartilage, confirming the diagnosis of first branchial cleft cyst (type II).No sign of recurrence was noted after a follow-up period of 15 months. The second patient was a 15 year-old girl presented in our Department with a congenital fistula of the lateral neck with recurrent episodes of drainage during the last years . PatientAt superficial parotidectomy, a total excision of the fistula was performed. During the operation the tract was recorded to lay between the branches of the facial nerve, extending with a blind ending canal parallel to the external acoustic meatus . As in oPathological examination of the surgical specimen revealed that fistula has lined by squamous epithelium and contained cartilage and skin adnexa, indicating first branchial cleft anomaly. Granulomatous and giant cell reaction was also focally found.No sign of recurrence was noted after a follow-up period of 12 months. First branchial cleft anomalies are rare malformations which account for less than 10% of all branchial clefts anomalies and appear in both adults and children . As paro First branchial cleft anomalies are the result of abnormal embryogenesis and arise from incomplete closure of the ventral portion of the first branchial cleft. Whether the defect is a fistula, sinus, or cyst depends on the degree of closure . The anoThe classification by Work is perhaps the most widely quoted. He classified these anomalies into two types based on clinical and histological features . Type I  Depending on the type of the anomaly, clinical manifestations can be roughly divided in cervical, parotid, and auricular symptoms . CervicaDiagnosis of first branchial cleft lesions is achieved mainly through careful physical examination of both the neck and the external auditory canal. According to Triglia et al. physical examination of the external auditory canal allowed discovery of a fistula in 44% of the patients, while an asymptomatic membranous attachment between the floor of the external auditory canal and the tympanic membrane was found in 10% of the cases . Such my Radiological examinations can be helpful diagnostically, in order to determine the typical anatomical localisation, the extent, the size, and the relationship of the lesions to surrounding structures. MRI allows assessment of the extent of the anomaly, especially in the parotid area, and high-resolution-computed tomography imaging shows its exact relationship with the external auditory canal and the middle ear. Fistulas and sinuses are not always obvious on CT and a fistulogram can be useful .  We cannot stress enough the importance of clinical suspicion on initial presentation of such lesions. Misdiagnosis and subsequent inappropriate or inadequate treatment are unfortunately still the rule. The delay between initial presentation and adequate treatment is reported to reach 3.5 to 4 years . Differential diagnosis of first branchial cleft anomalies must be made mainly with second branchial cleft anomalies. Parotid branchial cleft cysts, in particular, must be differentiated from any cystic mass of the parotid , 12. RetComplete surgical excision with wide exposure of the lesion is essential in order to achieve permanent cure and avoid recurrence. Parotidectomy and exposure of the facial nerve are required in the vast majority of cases . Any sin The overall recurrence rate after operation for branchial cysts is 3 to 5%, with a slight increase for sinuses and fistulae , 14. Rec


Osteoblastoma is a rare benign, but locally aggressive bone tumor with rare malignant transformation. It mostly affects the vertebral column and long bones. Radiographically, it is seen as an expansile, oval, sclerotic or lytic mass-like lesion with well-defined borders, although sometimes it may mimic a malignant tumor such as osteogenic sarcoma by its irregular borders. Herein, we report a case of osteoblastoma in a 22 year-old man with a long history of back and neck pain accompanied with neck stiffness. On the routine chest X-ray, the salient lesion appeared as an expansile, oval, sclerotic mass with well-defined borders and speckled calcification without any internal lucency and periosteal reaction, involving the posterolateral aspect of the first left thoracic rib, a rare anatomical site. Despite the unusual location, osteoblastoma should be considered in the differential diagnosis of a solitary rib lesion. Osteoblastoma is a rare benign bone tumor that affects males more than females with a ratio of 2.5-1. Its peak incidence is in the second decade, and it is rare before 10 years and after 30 years of age . It accoA 22-year-old man came with a long history of neck stiffness, back and neck pain radiating to his left upper extremity. On physical examination, no swelling was found, but there was a mild tenderness around the left side of his neck and shoulder. The symptoms had begun since 2 years ago and did not alleviate by pain killers such as salicylates. On a routine chest X-ray, the significant lesion involved the posterolateral aspect of the first left rib. Lordotic view chest X-ray demonstrated an expansile, oval, sclerotic mass with well-defined borders and speckled calcification without any internal lucency and periosteal reaction . In compOsteoblastoma is a rare osteoblastic benign bone tumor that most frequently affects the vertebral column and sacrum, but it may virtually affect any bone in the body , 2. Seve


Pancreatic dermoid cysts are a rare entity. Preoperative diagnosis is difficult. The diagnosis is generally taking intraoperative. A 20-year-old female presented with epigastric pain without nausea, vomiting, diarrhea, fever, jaundice, and weight loss of one-month duration. Ultrasonography and computed tomography showed a smooth borders, solid, hyperechoic tumor within midline abdomen, without any connection to the stomach or spleen. At surgery, the entire mass was excised off of the head and inferior part of pancreas. Histopathologic evaluation revealed the rare diagnosis of a dermoid cyst. The diagnosis is difficult preoperatively in evaluating cystic pancreatic lesions by imaging. Therefore, we want to summarize the literature on this rare entity knowledge. Dermoid cysts are congenital developmental abnormalities of germ cell origin derived from any of the three germinal layers, that is, ectoderm, entoderm, and mesoderm. Teratomas usually occur along the midline of the body, which is the route of germ cell migration during embryogenesis. These migrating germ cells may become “misplaced” en route to their appropriate organs, leading to the development of tumors later in life. The pancreas is extremely rare as a primary site. As true cysts, dermoid cysts are usually benign, well differentiated lesions. Pancreatic teratomas probably originate from aberrant germ cells arrested in migration to the gonads early in embryonic life. They are composed of tissue derived from any of the three germinal layers and may produce a wide variety of structures with different degrees of differentiation, including hair, teeth, cartilage, bone, and dermal appendages, such as hair follicles, sweat glands, and sebaceous material , 2. TheyA case of a 20-year-old woman was admitted to our hospital due to vague epigastric pain without nausea, vomiting, diarrhea, fever, jaundice, and weight loss of one-month duration. The patient had no significant medical history. A physical examination demonstrated mild tenderness in the epigastrium but no evidence of an acute abdomen. Laboratory studies including functional tests of the pancreas, and the liver and kidneys showed normal values. The levels of serum carbohydrate antigenic determinant (CA19-9) and carcinoembryonic antigen (CEA) were not elevated. A CT scan revealed a lobular contoured solid mass with a 13 cm long axis, 6 cm transverse diameter, and 5 cm anterior to posterior diameter, localized between the head and inferior part of the pancreas, compressing the pancreas and surrounding tissues. Low density was dominant, but the lesion also included patchy high dense regions and an approximately 2 × 5 cm sized calcification. Ultrasonographic (USG) examination reveals that solid and hyperechoic mass lesion was observed in the midline of the abdomen. This mass is approximately 5 × 5 cm in size with smooth borders and does not invade the intra-abdominal organs . No defiAt surgery, the entire mass was excised off of the head and inferior part of pancreas and sent for histopathologic examinations.Macroscopically, a gray-brown colored, 10 × 7 × 5 cm sized mass with irregular surface was found. There was a glazing compound and a 9 cm sized hair ball on the upper surface of this mass. The cross-section was yellow-white in color. In some regions, there was cystic lumination (multiloculation). The different parts of the lesion were observed in seven cassettes. On histological examination, it was revealed that the cyst wall was lined by mature stratified squamous epithelium and surrounded by lymphoid tissue containing germinal centers and sebaceous glands. In the lumen of the cyst, masses of keratinous debris were detected .The patient died from postoperative complication, that is, massive intra-abdominal hemorrhage and hypovolemic shock.Teratomas are widely believed to arise from embryonic inclusions of skin at the time of neural groove closure, hence their characteristic midline localization. Two subtypes of teratomas have been described: mature and immature. Mature teratomas are further classified as either solid or cystic, with the latter also known as “dermoid cyst.” Although dermoid cysts most commonly develop within the ovaries, they have been shown to occur anywhere along the route of ectodermal cell migration, usually in the midline. Localizations in the testis, cranium, brain, mediastinum, omentum, retroperitoneum, and sacrococcygeal region have all been described. Pancreatic dermoid cysts are extremely rare , 3.Following the description of a case of a mature cystic teratoma (MCT) of the pancreas by Kerr in 1918 , 4, the While most patients with pancreatic MCTs are asymptomatic, lesions are usually discovered incidentally. Patients may complain of nonspecific gastrointestinal symptoms such as nausea and vomiting, weight loss, anorexia, fatigue, abdominal pain, back pain, and fever –5. LaborThe radiologic appearance of pancreatic dermoid cysts, as with other MCTs, depends on their composition. A combination of radiologically detectable fat, fat-fluid levels, and calcium within the same lesion is considered to be highly suggestive of a mature teratoma , 7. On uOn computerized tomography scans, an MCT of the retroperitoneum manifests as a complex mass containing a well-circumscribed fluid component, adipose tissue, and calcifications. The presence of hypoattenuated fat areas within the cyst is considered highly suggestive of cystic teratoma, particularly if calcifications are detected in the cyst wall . On further evaluation with magnetic resonance imaging (MRI), T1-weighted images would show cystic lesions with distinct margins and fat-fluid levels of low intensity. Findings on both CT and MRI are highly specific, providing an accurate diagnosis in most cases , 4, 7.A definite diagnosis needs to be made by excisional biopsy or fine needle aspiration cytology (FNAC). In 1991, Markovsky and Russin . KoomalsDermoid cysts of the pancreas are true cysts. The cyst wall, which surrounds the lesion, is lined by a single layer of keratinizing stratified squamous epithelium, and the underlying connective tissue may contain adnexal tissue, sebaceous glands, lymphoid tissue, and even inflammatory cells. Dermoid cysts often contain thick, pasty, doughy sebaceous secretions. Fully differentiated tissues from one or more germ cell layers, most commonly the ectoderm, including hair, teeth, calcium, cartilage, and dermal appendages, such as hair follicles, sweat glands, and sebaceous material, are also usually encountered –4, 11. The differential diagnoses of pancreatic cystic lesions include pseudocyst, neoplastic cysts such as mucinous and serous cystadenoma, intraductal papillary mucinous neoplasm (IPMN), and solid pseudopapillary tumor. Although US, CT, and MRI are undoubtedly helpful, findings are far from being pathognomonic , 13. As Although malignant degeneration of pancreatic dermoid cysts has yet to be described, it is essential that histopathological evaluation includes complete sampling of the cyst wall in order to exclude the presence of immature foci , since 7–10% of other retroperitoneal teratomas have been reported to be malignant . Despite their benign nature, ambiguity regarding a diagnosis often leads to surgical resection, which remains the gold standard treatment for MCTs. In a recent review, it was established that more than 70% of excised pancreatic cystic lesions reported were either malignant or premalignant, and based on these findings it is recommended that surgical excision be undertaken for any symptomatic cystic lesion as well as any lesion larger than 2-3 cm in size, particularly if discovered in an elderly patient . Not onlIntraoperative frozen examination is a useful tool that may help prevent overtreatment of pancreatic masses in young individuals.


Post-stereotactic radiation-induced neoplasms, although relatively rare, have raised the question of benefit regarding CyberKnife® treatments versus the risk of a secondary malignancy. The incidence of such neoplasms arising in the nervous system is thought to be low, given the paucity of case reports regarding such secondary lesions.Here we describe a case of a 43-year-old Middle Eastern woman with primary clear cell renal cell carcinoma and a metastatic focus to the left brain parenchyma who presented with focal neurologic deficits. Following post-surgical stereotactic radiation in the region of the brain metastasis, the patient developed a secondary high-grade astrocytoma nearly 5 years after the initial treatment.Although the benefit of CyberKnife® radiotherapy treatments continues to outweigh the relatively low risk of a radiation-induced secondary malignancy, knowledge of such risks and a review of the literature are warranted. Given the advent of new stereotactic radiosurgery techniques, important questions have arisen regarding the risk of secondary malignancy following such treatments. On the basis of previous case reports, the incidence of such a secondary malignancy following CyberKnife® therapy has been estimated at between 0.7% and 1.9% . SpecifiA 43-year-old Middle Eastern woman originally presented complaining of a sudden onset of right upper extremity weakness and numbness. A magnetic resonance imaging (MRI) scan of her brain demonstrated a lesion in the left frontal lobe consistent with a possible metastasis, and an abdominal computed tomography showed an 8cm mass in the right kidney. She underwent a radical laparoscopic nephrectomy a month later, and surgical pathology revealed clear cell renal cell carcinoma Figure  of FuhrmLater that same month, the patient also had a left frontal craniotomy performed for metastatic focus resection. Microscopically, the specimen revealed nests of large pleomorphic cells with prominent eosinophilic nuclei that resembled malignant ganglion cells. However, clear cytoplasm was observed in some areas Figure A. ImmunoA year later, the patient began developing neurological symptoms including right arm tremor, weakness, dizziness, and abnormal sensation on her right cheek. An MRI scan of her brain revealed a large left frontal mass stemming from the insular that subsequently enlarged and grew upwards, creating a midline shift. After 5 months, she underwent resection of the mass. Pathology showed radiation necrosis with sheets of foamy macrophages and gliosis of the surrounding brain tissue with focal perivascular chronic inflammation. IHC staining was negative for keratin, with no evidence of viable tumor cells. IHC staining was positive for CD68 indicating the histiocytic nature of many of the foamy cells.The patient once again presented with right-sided hemiparesis. Imaging studies showed a left occipital-parietal mass, and surgical resection was completed approximately 4.5 years after her initial craniotomy and brain irradiation. The pathologic appearance of the tissue was consistent with a high-grade astrocytoma, probably glioblastoma multiforme, with areas of necrosis and vascular proliferation Figures A and 3B.et al. in 1948 [The criteria for a radiation-induced neoplasm as originally outlined by Cahan  in 1948  include:The risk of developing a secondary nervous system cancer, particularly meningiomas, following conventional fractionated radiation exposure has been well established. Studies of the survivors of the atomic bombings of Hiroshima and Nagasaki indicate an increased incidence of meningiomas in this population -6. EpideThe risk of oncogenesis due to stereotactic radiosurgery has generally been believed to be lower than that of conventional radiotherapy. Although traditional practices involve low-dose radiation delivered to a high volume of tissue, stereotactic methods allow for high-dose, low-volume radiation with a steep drop in dosage outside the targeted zone. The perception of increased safety in radiosurgery has been supported by an analysis of nearly 5000 English patients who underwent Gamma Knife therapy . This stAs yet, relatively few case reports of post-stereotactic radiation-induced nervous system tumors have been documented in the literature Table , and somWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MA analyzed and interpreted the patient data regarding the histological and immunohistochemical tests, and was a major contributor in writing the manuscript. AB helped in analyzing patient data and follow-up history. Both authors read and approved the final manuscript.


Extra-cranial meningioma or ectopic meningioma is a rare tumor. This tumor has been reported in various anatomic sites in the head and neck, mediastinum, skin and soft tissues. We report a rare case of ectopic meningioma in the submandibular region detected by using fine-needle aspiration cytology, histopathology and immunohistochemistry. This case represents another unusual site for extra-cranial meningioma, which prompted us to report it.An 18-year-old Dravidian woman presented with swelling in the right submandibular region. The computed tomographic scan findings were suggestive of a neoplastic mass lesion in the right submandibular region. Fine-needle aspiration cytology led to the differential diagnosis of a monomorphic adenoma of a salivary gland or an ectopic meningioma. The patient underwent excision of the submandibular gland and tumor. The histological examination and immunohistochemistry studies confirmed that the lesion was an extra-cranial meningioma. At her two-year follow-up examination, there was no recurrence of the tumor.Our experience with this case indicates that, although rare, meningioma should be entertained in the differential diagnosis of a mass lesion in the head and neck region. Meningiomas are among the most frequently encountered tumors of the central nervous system (CNS). They arise from arachnoid cells of the meninges. Extra-cranial primary meningioma is a tumor of rare occurrence. Ectopic meningiomas have been reported in various anatomic sites in the head and neck region, such as the floor of the mouth , the nosAn 18-year-old Dravidian woman presented to our hospital with right submandibular swelling of three to four months' duration. An examination revealed a firm, non-tender swelling in the right submandibular region measuring 3 cm × 3 cm Figure . The CT Ectopic meningiomas represent a well-known entity and are reported to occur in different locations. Meningiomas are common tumors of the CNS that arise from the arachnoid cells of the meninges. Rarely, these meningiomas appear in extra-cranial and extra-spinal areas, where they are known as ectopic meningiomas. Their reported incidence ranges from 0.9% to 2.0% of all meningiomas . HeterotHowever, primary ectopic meningiomas are very rare and have been reported in the orbit , head anIn our patient, the clinical impression was that of a salivary gland neoplasm because of its location in the submandibular area. On the basis of fine-needle aspiration cytology (FNAC), the differential diagnosis of oncocytoma and extra-cranial meningioma was made. The cells were oval to elongated and were arranged in loose clusters with a whorled appearance in places. Intra-nuclear pseudo-inclusions were also observed. Similar experiences at the time of FNAC-based diagnosis have been reported by others . Under lIHC analyses of the reported cases of primary ectopic meningioma are similar to their intra-cranial counterparts . The tumThe treatment of choice for extra-cranial meningiomas is surgical excision. The patient's prognosis is good after complete resection. All of the previously reported cases have been treated in this way, and given the long evolution and the lack of recurrence in all of them, it may be concluded that this lesion behaves in a non-aggressive fashion .To summarize, ectopic meningiomas can pose a diagnostic difficulty for the clinician as well as for the cytopathologist, as it is a diagnosis which may be easily forgotten in the list of differential diagnoses of neck masses. Although these tumors are unusual, their characteristic histological features establish the diagnosis.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SD had a major role in establishing the diagnosis histologically and in critically evaluating and revising the manuscript. GP performed the histopathological examination of the specimen and was involved in drafting the manuscript. VR treated the patient surgically and read and approved the revised manuscript. SN managed the patient and provided the data. AV participated in the literature review and contributed to the compiling and editing of the data. All authors have read and approved the final manuscript.


Mucocele of the appendix is an uncommon disorder that is often asymptomatic, but can present similarly to acute appendicitis. Timely diagnosis and treatment is imperative due to the many complications that can result from the mucocele, such as perforation. Appendiceal mucoceles (AM) were previously thought to be either benign or malignant; however, a different pathological classification of AM is currently favored. Also, only a few cases of volvulus of a benign AM have been reported. Here, we present the first reported case of a low-grade appendiceal mucinous neoplasm resulting in a volvulus of the cecum. Mucoceles of the appendix are a rare entity, as they represent only 0.2–0.3% of all surgical appendectomy specimens . AlthougThe most common portion of the colon to have a volvulus is in the sigmoid portion, with cecal volvulus occurring less commonly. Cecal volvulus can be divided into three types: axial torsion (Type I), loop type (Type II) and cecal bascule (Type III). It occurs when there is a lack of peritoneal fixation of the cecum, allowing it to be more mobile and resulting in the obstruction. It is extremely rare for a mucocele to present with the complication of a cecal volvulus. Here we present the first case of a low-grade appendiceal mucinous neoplasm causing a volvulus of the cecum.A 77-year-old male presented to our Emergency Department with a one-day history of abdominal pain located in the right lower quadrant. The pain was sharp and of sudden onset; however he denied any fevers, nausea, vomiting or changes in bowel habits. His medical history was significant for hypertension and chronic renal insufficiency. The surgical history included an aortic valve replacement and an open repair of an abdominal aortic aneurysm 10 years previously.On examination, he had a temperature of 99.0°F and a blood pressure of 157/86; the remaining vital signs were normal. The abdominal exam was significant for tenderness in the right lower quadrant, with a palpable mass. There was a small incisional hernia superior to the umbilicus from his prior abdominal aortic aneurysm repair, which was non-incarcerated. There were no peritoneal signs, and the rest of the abdomen was benign.Laboratory work-up revealed a white blood cell count of 8000/µL with 79% neutrophils. The blood urea nitrogen and creatinine were at his baseline of 33 mg/dL and 1.7 mg/dL respectively, consistent with his chronic renal failure. In addition, his carcinoembryonic antigen (CEA) level was mildly elevated at 4 ng/mL. The remainder of the laboratory work-up, including electrolytes, hemoglobin and hepatic profile, were normal. A computed tomography (CT) scan without contrast of the abdomen and pelvis was obtained, which showed a large, thin-walled structure containing hypodense material in the right lower quadrant . The strThe patient was brought to the operating room as an emergency case and a midline incision was made for an exploratory laparotomy. A large appendiceal mass was identified, which extended to the cecal base . The AM The pathology report described a cystically dilated and enlarged appendix measuring 17 cm in length and ranging in diameter from 2.8 cm at the appendiceal orifice to a maximal diameter of 7.5 cm at the tip. The epithelium of the appendix was mucinous, flat, and showed low-grade dysplasia. The histological features are consistent with low-grade appendiceal mucinous neoplasm. Focally acellular mucin dissection into the extensively fibrotic wall of the appendix was seen. In addition, the tumor extended from the appendix and was found in the cecum, at the junction of the appendix and cecum. The resection margins were negative for tumor or dysplasia and 36 lymph nodes were negative for tumor. The patient’s post-operative course was complicated by a seizure on post-op day two. It was treated with medical management and the remainder of the post-operative course was uneventful. The patient was discharged on post-operative day nine, and made a full recovery on outpatient follow-up.Mucocele of the appendix was first described by Rokitansky in 1842 and then definitively named by Feren in 1876 . The latMucocele of the appendix is a rare entity that can present with a variety of symptoms, or be an incidental surgical finding. It is a progressive dilation of the appendix due to mucus accumulation and makes up only 0.2–0.3% of all appendiceal specimens . They haIn addition, it is important to keep in mind that appendiceal mucinous neoplasms are associated with an increased incidence of other tumors. In some reports, the rate of synchronous tumors is as high as 29% . Follow-An AM can be clinically confused with acute appendicitis, which is why pre-operative diagnosis is important to determine the surgical approach. The most common presentation of symptomatic mucocele of the appendix is acute or chronic right lower quadrant pain. Nausea and vomiting, as well as a change in bowel habits, have also been reported. An intra-abdominal mass can be palpated by the physician on examination in about half of these cases . Open suThe classification of appendiceal mucinous neoplasms is controversial, and has recently been altered. The former classification included histological categories including mucinous cystadenoma and mucinous cystadenocarcinoma, commonly distinguished by the presence of tumor extension into the peritoneum . In 1995Appendiceal mucocele is a difficult diagnosis to make clinically. However, its importance lies in the potential complications that can follow. The most common complication is perforation, which has an incidence of up to 20%. Rupture with subsequent pseudomyxoma peritonei is the most feared outcome in this scenario ; this ocOther complications of AMs have also been described. Albeit much less common, these complications include intestinal obstructions, intussusception, intestinal bleeding, fistula formation and volvulus. It is extremely uncommon for an AM to cause a volvulus, with few cases reported since its initial description in 1946 [Conflict of interest: none declared.