Secondary leptomeningeal dissemination of oligodendroglioma is very rare. We report the case of a 38-year-old Caucasian male who presented with acute hydrocephalus. 8 months before, the patient had undergone craniotomy for right frontal anaplastic oligodendroglioma, WHO grade III. By that time, there was no evidence of tumor dissemination. MRI now ruled out local tumor progression but revealed meningeal contrast enhancement along the medulla, the myelon, and the cauda equina. Repeated lumbar puncture revealed increased cerebro-spinal fluid (CSF) pressure and protein content. Malignant cells were not detectable. Surgical treatment consisted in (1) placement of an ommaya reservoir for daily CSF puncture, (2) Spinal dural biopsy confirming leptomeningeal oligodendroglioma metastasis, and (3) ventriculo-peritoneal shunt placement after CSF protein has decreased to 1500–2000 mg/l. Oligodendroglioma represents about 5% of glial tumors. The clinical course is particularly characterized by seizures. The preferred location is in the cortex and white matter of the cerebral hemispheres, most often located in the frontal lobe. Nevertheless, oligodendroglioma has also been reported in the cerebellum, brain stem, or spinal cord .Median postoperative survival is in the range of 3–5 years. They tend to recur locally. Malignant progression from WHO grade II to III or from II/III to IV (glioblastoma) is not infrequent .The tumor usually presents as a solid mass. However, several cases of primary diffuse leptomeningeal oligodendrogliomatosis have been reported –5. Most We report the case of a 38-year-old male with secondary cranial and spinal leptomeningeal tumor dissemination 8 months after surgical resection of an anaplastic oligodendroglioma WHO grade III of the right frontal lobe. The treatment was intended to prove the dissemination histologically and to treat hydrocephalus appropriately.A 38-year-old patient presented to our department with the 4-day history of headache, nausea, vomiting, and visual disturbance. He also complained about progressive back pain radiating into both legs. The patient had undergone subtotal tumor resection for anaplastic oligodendroglioma  of the right frontal lobe 8 months before. At the time of initial surgery, there were no signs of leptomeningeal tumor involvement or hydrocephalus Figures . Surgery2O. CSF examination showed marked elevation of protein (15972 mg/l), slightly elevated cell count (16/μL), and normal glucose and lactate. Cytological CSF examination showed monocytes and lymphocytes but was free of tumor cells.Cranial computed tomography (CCT) on admission now revealed marked ventricular enlargement. Magnetic resonance imaging (MRI) exhibited enlargement of the lateral ventricles and the third ventricle with periventricular high intensity signal on T2WI suggesting transependymal absorption or tumor spreading Figures . There wAn Ommaya reservoir was placed for daily CSF puncture. Herein, CSF protein content was decreasing to an amount of 1500–2000 mg/l. Yet there were no tumor cells detectable in consecutive cytological CSF examinations. MRI had shown a marked dural contrast enhancement on the L 2/3 level. So, we decided to biopsy the dura and the nerve fibers on this level. Intraoperatively the nerve fibers were covered with a grayish layer while the dura appeared normal. Biopsies were taken from the nerve fibers and the dura.Histological examination confirmed tumor cell infiltration of the dura and nerve fibers by the known anaplastic oligodendroglioma . RemarkaPostoperatively, PCV chemotherapy  was planned but refused by the patient. Four months after presentation with hydrocephalus, the patient was clinically stable without any signs of shunt malfunction or tumor progression.Oligodendroglioma mostly arises as a solid mass but can also appear as a diffuse leptomeningeal lesion . PDLG is assigned a fatal diagnosis and is frequently associated with hydrocephalus –5, 7. ItIn contrast, secondary spinal leptomeningeal dissemination from oligodendroglioma is very rare. In 1977, Arseni et al. reported two cases of secondary spinal involvement in patients with oligodendroglioma from the pre-CT era. The WHO grade is not available. In one case of an 8-year-old boy with pubertas praecox, cachexia, and visual disturbance ventriculography revealed a tumor of the third ventricle. Subtotal tumor resection was followed by radiotherapy. 2 years later, the patient presented with a spinal subdural tumor from C1 to C5 which was again treated surgically. The second patient was a 39-year-old male with the history of left-sided Jackson seizures. Angiography revealed a right temporal lesion which was excised and diagnosed as oligodendroglioma. Radiotherapy was applied. 15 months later, the patient presented with spinal pain and progressive paraparesis. Laminectomy from T1 to T3 revealed a spinal subdural oligodendroglioma .Treatment of hydrocephalus in patients with central-nervous system tumors is often a challenge due to the following facts: (A) it remains unclear if raised CSF protein content may cause shunt dysfunction. Most surgeons believe not only that high CSF protein content affects valve function but also that it affects viscosity . AccordiIn our patient, we had to consider all these facts in order to apply adequate treatment. The raised CSF protein content was brought back to acceptable values by daily puncture of an ommaya reservoir. Since cytological examination of repeated lumbar and reservoir puncture was negative for tumor cells, spinal dural biopsy was performed in order to establish the diagnosis. Finally, ventriculo-peritoneal shunting was performed for persistent hydrocephalus in combination with PCV chemotherapy .Secondary cranial and spinal dissemination in patients with anaplastic oligodendroglioma is extremely rare. It should be kept in mind when these patients report with signs of hydrocephalus or back pain. The diagnostic method of choice is MRI before and after contrast administration. Cytological CSF examination might be negative. As a consequence, surgical biopsy might be necessary. Treatment of hydrocephalus can include emergency lumbar puncture (after CT or MRI), ommaya reservoir placement, and shunt insertion. The latter should be accompanied by chemotherapy in order to prevent metastasis to other organs.


Primary neoplastic lesions presenting with a mucocele of the appendix are very rare and can be divided into benign variants of mucinous adenomas or cystadenomas, mucinous tumours of uncertain malignant potential or mucinous cystadenocarcinomas. Most of these tumourous mucoceles are asymptomatic and are found incidentally. The major complication of neoplastic mucinous appendiceal tumours is the development of a pseudomyxoma peritonei due to spreading of mucin-producing cells within the abdominal cavity.A 44-year-old man presented with a history of non-specific symptoms of right upper abdominal pain. Abdominal ultrasound and computed tomography scan identified a cystic mass consistent with the morphological characteristics of an echinococcal hydatid cyst. After completing systemic albendazole therapy, an explorative laparotomy revealed a cystic tumour of the appendix. Ileocaecal resection was performed and pathology reports confirmed the diagnosis of a mucinous cystadenoma of the appendix. The postoperative course was uneventful.Here we present the case of a man with a mucinous cystadenoma of the appendix mimicking cystic hydatid disease. We discuss the importance of re-evaluation and differential diagnostic reflections in cases of appendiceal mucocele. Mucocele of the appendix is a rare cystic dilatation of the appendiceal lumen caused by mucinous secretions and consecutive retention of mucus. It can be caused by either non-neoplastic or primary epithelial neoplastic disease ,2. The nHere we report a case of a man with a mucocele caused by a mucinous cystadenoma mimicking cystic hydatid disease and discuss the importance of differential diagnostic reflections in cases of appendiceal mucocele.A 44-year-old man presented with a history of non-specific symptoms of right upper abdominal pain for 6 years. Abdominal ultrasound (US) revealed a cystic mass containing low level internal echoes and sonic shadowing. Computed tomography (CT) revealed, in the axial and coronal views, a cystic mass with curvilinear calcifications extending from segment six of the right hepatic lobe into the right lower quadrant, consistent with the morphological characteristics of an echinococcal hydatid cyst Figure  and 1B. During exploration of the abdominal cavity the cystic mass, which measured 17.5 cm in length and 4.5 cm in diameter, was identified as a cystic antecaecal appendix with a smooth serosal surface involving the base of the appendix Figure . Due to The incidence of mucoceles of the appendix is reported to be 0.2% to 0.3% . ApproxiCross sectional imaging such as CT is superior to US in the evaluation of mucoceles of the appendix because it demonstrates the topographic anatomical relationship between the caecum and the mucocele. In addition, CT is more sensitive than US in the detection of mural calcifications within the mucinous neoplasm. The appearance at CT scanning is characterized by a well-encapsulated cystic structure with either an enhancing smooth thin or thick wall, with or without mural calcification. The detection of mural curvilinear calcifications is highly suggestive of the diagnosis, but is detectable in less than 50% of cases . Kim et Several diseases including hydrosalpinx, ovarian cysts or renal cysts have been reported as mimicking appendiceal mucocele in their US and CT appearance as well as in their uncharacteristic clinical symptoms ,11. To ochinococcus granulosus, has an uneven geographical distribution in Europe, presenting with well-defined single or multiple cysts on CT scan that may be uniloculated or multiloculated and either thin or thick walled. Calcification, daughter cysts and germinal membrane detachment may also be present. Usually no rim enhancement is evident unless the hydatid cyst is superinfected [Cystic hydatid disease, caused by Einfected . Cystic infected .Although serodiagnosis was negative, in our case the morphological appearance on CT, the localization with topographic relation to the right liver lobe and the medical history made the diagnosis of a cystic hydatid disease most likely. It is worth noting that the sensitivity and specificity of serodiagnostic tests for Echinococcosis have been reported to range between 80% and 90% and therefore results might be negative despite hydatid disease being present .New therapeutic approaches in the treatment of hydatid disease include percutaneous punction of the cystic lesions followed by aspiration of the internal fluid and injection of alcohol containing solutions, and re-aspiration. This so-called PAIR procedure should be repeated several times . In thisThe case presented shows the importance of re-evaluation and differential diagnostic reflections in cases of the very rare entity of mucocele of the appendix. In particular, in cases with high suspicion for hydatid disease but with negative serodiagnosis, surgical exploration is recommended rather than the interventionally performed PAIR procedure.CT: computed tomography; PAIR: puncture aspiration injection re-aspiration; US: ultrasound.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AK and JSAE performed the surgery, conducted the acquisition, analysis and interpretation of data and drafted the manuscript, LWP performed the analysis and interpretation of the computed tomography scans, SB performed the pathological analysis, WTK made substantial contributions to the conception, acquisition and interpretation of data and revised the manuscript critically. All authors read and approved the final manuscript.


We report the case of an osseous hemangioma located in the seventh cervical vertebra with reactive osteoid formation and non-typical findings in the radiological and the histopathological examination, mimicking metastasis of a malignant tumor. To our knowledge, this is the first description of such a case in the literature.A 44-year-old otherwise healthy Caucasian German woman presented with a discrete sensorimotor loss of both upper limbs. Radiologically, an osteolysis in the seventh cervical vertebra suggestive of metastasis of a malignant neoplasm was diagnosed. After performing corporectomy and cage implantation of C7 on the patient, the histopathological examination was complicated by marked osteoid formation obscuring the true diagnosis of an osseous hemangioma with reactive osteoid formation.Though hemangioma of the bone is a rare tumorous lesion in the cervical spine, it has to be taken into consideration as a reason for neck pain and sensomotoric loss of the upper limbs. Atypical radiological and histopathological presentations may hinder determination of the correct diagnosis. The treatment of such lesions must follow clinical guidelines but may be difficult to define in some cases when the correct diagnosis is not known at the time when therapy starts. Vertebral hemangiomas are often discovered incidentally on screening radiographs or computed tomography (CT) scans [Hemangioma is one of the most common benign tumors of the spine with a reported prevalence of 10% to 12% in the general population . VertebrT) scans .Typically, osseous hemangiomas appear radiographically as lytic medullary lesions with thickened vertical striations, resembling a "corduroy cloth" on X-ray examination or a polka-dot pattern in CT scan cross section . HistoloThis is a rare case of a seventh cervical vertebra affected by an unusual histological type of hemangioma of the bone with reactive osteoid formation with neither typical radiological nor histopathological findings.We present the case of a 44-year-old, otherwise healthy, Caucasian German woman who had been experiencing paresthesia in both forearms and hands for 3 months. Occasionally, she had pain and paresthesia in her face when moving her head. Physical examination showed normal muscle strength in both upper limbs and a discrete sensory loss. Movement of the cervical spine was almost free but painful at the end of the motion range. X-ray showed osteolysis of C7. Magnetic resonance images (MRI) indicated increased signal intensity on T2-weightened images. The osteolysis was diagnosed as a metastatic osteolysis of C7 Figure . The CT A complete staging including thoracic and abdominal CT scans, skeleton scintigraphy and MRI of the whole spine revealed no further lesions. The osteolysis was regarded as an unstable metastasis from an unknown primary tumour. Therefore we decided to perform corporectomy of the seventh cervical vertebra and implantation of a customary titanium cage via a lateral approach. A biopsy was ruled out because the lesion was already seen as unstable. We did not want to risk a pathologic fracture with further complications for the patient.During corporectomy, an intra-operative consultation was performed. The frozen sections showed a cellular lesion displaying spindle-shaped cells with predominantly small, partly elongated nuclei with moderate chromatin density interspersed with immature, partly calcified osteoid. Thus, the intra-operative diagnosis was a mesenchymal tumor.The postoperative recovery was uneventful and the patient's complaint of neck pain and paresthesia in the forearms disappeared soon. Ten days after surgery she was discharged.Further microscopic examination of the paraffin-embedded tissue revealed vessel-like structures adjacent to and within the dense mesenchymal cells as well as osteoid Figure . TypicalThis atypical presentation represented a severe diagnostic challenge prompting consultation with a specialist pathologist. Intensive immunohistochemical work-up by the pathologist revealed a distinct vascularization of the lesion with partly collapsed sinusoidal and slit-like blood vessels Table . CriteriSince the final histopathological diagnosis rendered was "hemangioma with reactive osteoid formation", we recommended postoperative radiotherapy, which was refused by the patient. Six months later movement of the cervical spine was painless with a loss of rotation of 20 degrees. There was no sensorimotor loss in the upper limbs. X-ray examination affirmed a good and maintained position of the cage and a normal alignment of the cervical spine Figure .Vertebral hemangioma is a relatively frequent benign lesion that accounts for a small percentage of surgical biopsies or resections. The radiographic appearance is usually quite characteristic with lytic medullary lesions displaying vertical striations, which appear as "polka-dot" pattern in CT cross sections . Three hIn our case, the predominant histologic pattern of the patient displayed spindle-shaped cells with interspersed, immature and partly calcified osteoid. Further microscopic examination of the paraffin-embedded tissue revealed vessel-like structures adjacent to and within the dense mesenchymal cells. These partly sinusoidal, partly slit-like, blood vessels are indicative of the capillary type of hemangioma. However, the proper diagnosis was obscured by a marked osteoid formation between the vessels, which has been described only on rare occasions , and waset al. found out that the most valuable neuroimaging factor for predicting capillary type are large soft tissue appearances and resorption of bone on CT scans [et al. describe that in the case of "mixed hemangiomas", radiological findings are often untypical and the diagnosis can not be obtained before operation [Also, the typical radiographic pattern of a vertebral hemangioma, with coarse vertical striations and/or a "polka-dot" pattern on MRI and "honeycomb appearance" on plain films, rather characterizes the cavernous type which may account for the pre-operative diagnostic difficulties and the suspicion of a metastastatic osteolysis of C7. Robin CT scans . The sigperation . We postperation ,9,11.Although hemangiomas of the cervical spine are rare, they have to be considered as a reason for severe neck pain with associated sensorimotor loss of the upper limbs. Vertebral hemangiomas of bone can compromise vertebral stability and lead to pathological fracture with severe consequences for the patient. This case shows that vertebral hemangiomas can mimic vertebral osteolytic metastasis with atypical radiological and histopathological findings. The diagnostic course to reach the correct diagnosis can be complex. We used diverse expensive and time-consuming radiological imaging modalities and histopathological examinations, and emphasise that they must be performed along clinical guidelines to prevent a wrong diagnosis and possibly wrong and dangerous treatment.CD: cluster of differentiation; CT: computed tomography; Ki 67: antibody Ki-67; MRI: magnetic resonance imageWritten 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.LS, WCC, FWS and SMD analyzed and interpreted the patient data regarding the osseous hemangioma. SMD carried out the surgical intervention on the patient, WC performed the histopathological examinations. LS was the main author of the manuscript. All authors read and approved the final manuscript.


Cushing's Syndrome (CS) which is caused by isolated Corticotropin-releasing hormone (CRH) production, rather than adrenocorticotropin (ACTH) production, is extremely rare.We describe the clinical presentation, course, laboratory values and pathologic findings of a patient with isolated ectopic CRH causing CS. We review the literature of the types of tumors associated with this unusual syndrome and the behavior of these tumors by endocrine testing.A 56 year old woman presented with clinical and laboratory features consistent with ACTH-dependent CS. Pituitary imaging was normal and cortisol did not suppress with a high dose dexamethasone test, consistent with a diagnosis of ectopic ACTH. CT imaging did not reveal any discrete lung lesions but there were mediastinal and abdominal lymphadenopathy and multiple liver lesions suspicious for metastatic disease. Laboratory testing was positive for elevated serum carcinoembryonic antigen and the neuroendocrine marker chromogranin A. Serum markers of carcinoid, medullary thyroid carcinoma, and pheochromocytoma were in the normal range. Because the primary tumor could not be identified by imaging, biopsy of the presumed metastatic liver lesions was performed. Immunohistochemistry was consistent with a neuroendocrine tumor, specifically small cell carcinoma. Immunostaining for ACTH was negative but was strongly positive for CRH and laboratory testing revealed a plasma CRH of 10 pg/ml  which should have been suppressed in the presence of high cortisol.This case illustrates the importance of considering the ectopic production of CRH in the differential diagnosis for presentations of ACTH-dependent Cushing's Syndrome. Cushing's syndrome (CS) comprises the clinical manifestations of exposure to elevated glucocorticoid hormones, either from exogenous sources or endogenous overproduction by the adrenal glands . CS from+ 2.1 mEq/l and HCO3 42 mEq/l). She was hyperglycemic (random glucose 339 mg/dl). An MRI of the spine showed multiple endplate compression fractures of the thoracic and lumbar vertebrae.A 56 year old type 2 diabetic patient presented to the emergency room with shortness of breath. Review of systems revealed back pain for two weeks, without history of injury, and progressive generalized weakness and fatigue for many months. She also described constant right upper quadrant abdominal pain. On examination, there was hyperpigmentation of her face and neck in sun exposed areas, thin skin with violacious striae on her lower abdomen, and multiple ecchymoses over the extremities, abdomen and at venipuncture sites. There was prominent fat accumulation in the supraclavicular and dorsocervical regions. There also was objective evidence of proximal muscle weakness which, along with back pain, had caused her to be bed-ridden for two weeks. Her medications included an oral sulfonylurea for diabetes but no exogenous sources of corticosteroids. Laboratory results revealed hypokalemic alkalosis  were elevated consistent with a malignancy with neuroendocrine characteristics Table . Laborat111-In]pentreotide showed no tracer uptake at 4 hours. Specimens from bronchoalveolar lavage did not show any histologic findings of small cell lung carcinoma.With the exception of the liver metastases and enlarged lymph nodes, there were no discrete lung nodules or neck, abdomen or pelvis lesions seen on CT. Scintigraphy with [The patient's course continued to deteriorate, with hypokalemia, hyperglycemia and difficult to control blood pressure. She was started on ketoconazole, an inhibitor of cortisol synthesis, but her cortisol levels continued to escape inhibition after each dose escalation Figure . The patCT guided biopsy of one of the liver lesions was performed to obtain tissue for diagnosis. The biopsy consisted of multiple cores of soft tissue with no liver parenchyma present, although rare glandular structures were seen, suggestive of the residual bile ducts in the portal triad. On hematoxylin and eosin staining, there was extensive infiltration by small blue tumor cells surrounded by slender fibrous tissue Figure . The celCushing's syndrome (CS) is the clinical manifestations of cortisol overproduction from ACTH-independent  or ACTH-dependent mechanisms. Most ACTH-dependent CS is due to corticotroph pituitary adenomas 90%), while ectopic ACTH production only comprises around 10% of cases . A signi0%, whileWe believe our patient had a CRH secreting tumor due to strongly positive CRH staining, negative ACTH staining, and detectable plasma CRH. A simple explanation for the lack of ACTH immunostaining is that it is not expressed in the tumor cells. The polyclonal antibody used for our ACTH immunostaining also reacts with the parent propeptide, pro-opiomelanocortin, so that aberrant prohormone processing of POMC to ACTH is not an explanation. We have not examined whether POMC transcripts were present in the biopsy sample, but there is precedence that POMC RNA can be present without detectable pro-peptide or ACTH in the tissue ,18. In tAn interesting aspect of this case is the lack of significant cortisol suppression by the high dose dexamethasone suppression test , with baseline cortisol at 122.2 μg/dl before the test and 109 μg/dl after 48 hours. With isolated ectopic CRH, it is expected that CS develops through the stimulation of pituitary ACTH secretion with resultant adrenal cortisol production. Because of this, ACTH and cortisol levels potentially could suppress with HDDST, similar to a pituitary adenoma. Young and colleagues  reportedWe present a rare case of isolated ectopic CRH production from metastatic small cell carcinoma of unknown primary. From the literature, the tumors most commonly associated with ectopic CRH production are medullary thyroid carcinoma, pheochromocytoma, and prostate cancer. With ectopic CRH syndrome, it is assumed that the pituitary is the source of ACTH with secretion driven by CRH overproduction. However, these tumors have behavior distinct from pituitary corticotroph adenomas, and the majority appears to lack suppression with high dose dexamethasone. Although the clinical presentation may be similar to ectopic ACTH syndrome, it is important to have ectopic CRH syndrome in the differential diagnosis for Cushing's syndrome.The patient was deceased and attempts to contact the next of kin were not successful. Because of this, the case content was submitted to the Institutional Review Board and was determined to be exempt from IRB review.The authors declare that they have no competing interests.All of the authors have read and approved the final manuscript. SS wrote the initial draft of the manuscript and analyzed the clinical data. HSK and RJN performed the immunohistochemistry on the biopsy samples and provided interpretation of the pathological findings. SLS coordinated the manuscript, edited the entire draft, performed the CRH immunostaining, and wrote the discussion of the case. RN edited the entire draft for final submission.Department of Medicine, Division of Diabetes, Endocrinology, and Metabolism and Department of Pathology, One Baylor Plaza, Mail Stop 285, Baylor College of Medicine, Houston, TX, 77030.


Solitary fibrous tumor of the pleura is a rarely encountered clinical entity which may have different clinical pictures. Although the majority of these neoplasms have a benign course, the malignant form has also been reported.We herein describe a case of 72 year-old man with head, facial, and thoracic traumas caused by neurally-mediated situational syncope when coughing. The diagnostic work-up including chest x-ray, CT and PET, revealed a large solitary mass of the left hemithorax. Radical surgical resection of the mass was performed through a left lateral thoracotomy and completed with a wedge resection of the lingula. Hystological examination of the surgical specimen showed an encapsulated mass measuring 12 × 11.5 × 6 cm consistent with a solitary fibrous tumor of the pleura. It's surgical removal definitively resolved the neurologic manifestations. The patient had no postoperative complications. At two years follow-up the patient is free from recurrence and without clinical manifestations.In our case its resection definitively resolved the episodes of situational syncope due, in our opinion, to the large thoracic mass compressing the phrenic nerve First described by Klemperer and Rabin , the solA 72 year-old man was admitted to the hospital for head injury, facial and left hemithorax contusions. The patient referred to had fainted after coughing; the same symptoms had occurred six and three months earlier. Diverticulosis of the colon was the only disease reported by the patient in his medical history. He denied smoking, drug or alcohol abuse. Physical examination showed dullness to percussion and decreased breath sound in the affected hemithorax. The neurological examination was negative. Blood pressure was 170/80 mmHg, heart rate was 90 beats/minute and rhythmic. Laboratory findings, arterial gas analysis, electrocardiogram, and brain computed tomography were negative.A chest x-ray revealed fractures of three left ribs plus a large medium-basal opacity on the left hemithorax Figure . ComputePathological examination showed a 12 × 11, 5 × 6 cm encapsulated tumor mass Figure , whitishAfter two years of follow-up the patient is in good clinical condition without recurrence of disease or clinical symptoms.et al, [It is well known that solitary fibrous tumors of the pleura are incidentally discovered during chest x-ray examination because these neoplasms often have a silent clinical course for several years ,6. It haet al,  in whichet al, . Suggestet al, . In our et al, . All theGenerally SFTP is a localized, benign tumor which may have different clinical pictures. It is curable using a careful and complete resection, provided that the surgical margins are free from neoplastic cells. In our case its resection definitively resolved the episodes of situational syncope due, in our opinion, to the large thoracic mass compressing the phrenic nerve.Written and 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.LS conceived the idea, did supervision of manuscript preparation and proof reading initiated treatment, did surgical procedures and approved the final version of the paper. MN, AP, LR, DT proof reading, initiated treatment, did surgical procedures. UC, MDS wrote the manuscript and carried out literature review; MMC contributed to data management and preparing of the manuscript. All authors read and approved the final manuscript.


Segmental dilatation of the ileum is one of the uncommon causes of intestinal obstruction in neonates. We present a case of slow transit of bowel contents leading to suspicion of functional bowel obstruction in a new born, which on exploration turned out to be a case of segmental dilatation of the jejuno-ileal region. The clinical and radiological evaluation was suggestive of hypomotility disorder of gut, resulting in diagnostic dilemma and delayed surgical intervention. Neonatal bowel obstruction as a result of segmental dilatation of the gut is a very rare disorder with limited published cases.–3 The coA routine prenatal sonographic examination in a primigravida of 35 weeks gestation revealed a complex thin-walled cystic mass with internal echogenic debris present in right lower abdomen of the fetus. The cystic mass showed peristalsis and changes appearance during scanning, suggestive of dilated bowel loop. It was opined as a case of anorectal malformation  as peristalsis were appreciated in this segment and it was located posterior to bladder. At birth, the patient passed copious amounts of meconium. He was put on oral feeds but developed bilious vomiting and 120 to 140 ml of light green aspirate every day while on IV fluids. The abdomen has remained soft, there was no distension. Postnatal ultrasound studies did not reveal any intraabdominal cyst. There was no suspicion of malrotation in any of the films or Doppler studies. Barium contrast studies have shown a dilated gut till the mid-portion of the small gut . The pasCongenital segmental dilatation of a portion of the small intestine in neonates causing intestinal obstruction is quite uncommon.2 Segment46789


Within a month the platelet count increased and the vascular tumor started regressing. This case signifies the importance of step wise management of KMS.Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area noticed at birth, which suddenly increased in size since 3 days. Hemogram revealed anemia severe thrombocytopenia, prolongation of bleeding, clotting time and increased fibrin degradable products, suggestive of KMS. Coagulopathy was managed by transfusing fresh frozen plasma and platelets. Oral prednisolone up to 5mg/kg/day for four weeks yielded no effect on thrombocytopenia or regression of tumor size. Embolization of feeding artery was attempted but not feasible. We used Interferon –alpha– 2b (IFN α 2b), in a dosage of 3million IU/m Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. It is characterized by a rapidly enlarging vascular anomaly, consumptive coagulopathy, thrombocytopenia, prolonged PT and APTT, hypofibrinogenemia, the presence of D dimer and fibrin split products with or without microangiopathic hemolytic anemia. Kasabach13et al.[A variety of treatments have been used to induce tumor regression, including prednisolone, dipyridamole, pentoxyfylline, ticlopidine, and heparin, interferon (IFN), vincristine, radiotherapy, and embolization of the feeding vessel to the tumor. We followed a step-wise regimen as evaluated by S. Wananukul et al.A 14-month-old female child came with history of sudden increase in size of lesion over left shoulder, back and chest wall associated with severe pain. This lesion was present from birth . On clinA hemogram revealed anemia with severe thrombocytopenia and prolongation of bleeding and clotting time and the presence of increased fibrin degradation products. Skin biopsy of the lesion showed dilated irregular thin walled vascular channels lined by flattened endothelial cells in the dermis. Ultrasonography of the chest revealed large ill defined heterogeneous solid diffuse lesion noted over the left side of the upper chest wall extending from front to back and axilla, involving skin and subcutis. Color Doppler showed feeding arterial supply from the intercostals vessels.2 /day subcutaneously. Within four weeks her platelet counts became normal and the hemangioma started regressing [Figures With a confirmed diagnosis of KMS, the patient was started on transfusions of platelets and fresh frozen plasma as she had oral bleeds. Oral prednisolone was started 2mg/kg body weight to suppress the hemangioma. Although the coagulopathy reverted to normal in a few days, platelets started decreasing and so she was referred to higher centre for thrombosing the feeding vessels of the hemangioma. However, she was referred back to us as it was not feasible. In spite of increasing the dose of steroids to 5mg/kg body weight, the platelet count continued to drop. It was then that we started her on interferon α2b in a dose of 3million IU/m Figures . FigureKMS is a rare syndrome. The objectives of treatment of KMS are to prevent bleeding from thrombocytopenia and consumptive coagulopathy and to induce vascular tumor regression. Thrombocytopenia in KMS is due to peripheral destruction, as platelets are trapped in these vascular tumors. Platelet transfusions can also exacerbate bleeding. PlateletTumor regression can be induced by various treatment modalities, including prednisolone,35 dipyri3Our patient had complications on high dose prednisolone, particularly hypertension and cushingoid appearance. Nevertheless, because of its low cost and ease of administration, steroid may still be most appropriate first line drug for the initial few weeks of therapy.2/day, with favorable results.IFN has been used in the treatment of KMS since 1992 due to its antiproliferative and antiangiogenic effect.[The known adverse effects of IFN therapy include fever, neutropenia, mild anemia, mild elevation of liver function test, and, most deleteriously, spastic diplegia in infancy. In concl


Splenosis is a heterotropic implantation of splenic fragments onto exposed vascularised peritoneal and intrathoracic surfaces, following splenic injury or elective splenectomy.A 60 year old cirrhotic patient was referred to us with a hepatic mass, suspected to be HCC in a cirrhotic liver. A computerized tomography scan (CT) demonstrated a cirrhotic liver with a 2 × 2.7 cm focal hypervascular nodule, lying peripherally at the junction of segment 7 and 8. Diagnostic laparoscopy demonstrated a 3 cm exofitic dark brown splenunculus attached to the diaphragm and indenting the surface of segment 7 of the liver. The lesion was easily resected laparoscopically and shaved from the live surface with no need for a liver resection. The histopathological assessment confirmed the diagnosis of splenunculus, with no evidence of neoplasia.Hepatic splenosis is not a rare event and should be suspected in patients with a history of splenic trauma or splenectomy. Correct diagnosis is essential and will determine subsequent management plans. In doubtful cases laparoscopic investigation can offere essential information and should be part of the standard protocol for investigating suspected splenosis. Splenosis is a heterotropic implantation of splenic fragments onto exposed vascularised peritoneal and intrathoracic surfaces, following splenic injury or elective splenectomy.There are a few reported cases of hepatic splenosis in the English literature, which has usually a challenging and difficult differential diagnosis with hepatic adenoma, haemangioma, focal nodulal hyperplasia, lymphoma and hepatocellular carcinoma -4. ExcluLaparoscopic exploration provides a port of minimally invasive entry for the visualisation of suspect masses, and allows access for potential subsequent biopsy or resection. To our knowledge, this is the first case were explorative laparoscopy has been an essential tool to confirm the diagnosis of splenosis and rule out the doubt of malignancy. This had a significant positive impact on this patient's management, avoiding unneccassary laparotomy or'/and surgical resection in a high-risk patient.Hepatic splenosis is not a rare event and should be suspected in patients with a history of splenic trauma or splenectomy. Correct diagnosis is essential and will determine subsequent management plans. In doubtful cases; laparoscopic investigation can offer essential information and should be part of the standard protocol for investigating suspected splenosis.A 60 year old cirrhotic patient was referred to us with a hepatic mass, suspected to be HCC in a cirrhotic liver. The patient was diagnosed with liver cirrhosis in December 2003, secondary to Hepatitis C infection after receiving blood transfusions at splenectomy for a ruptured spleen 46 years ago.Serial blood tests showed sudden derangement in his liver function, and a mild rise in alpha-feto protein levels. Clinically, he complained of non-specific flu-like symptoms and also reported recent weight loss and reduced appetite.On examination he appeared jaundiced, there were signs of clubbing and on examination of his abdomen, there was a mild degree of ascites and the liver edge was palpable.He was a persistent alcoholic and was prone to frequent binging. A computerized tomography scan (CT) demonstrated a cirrhotic liver with a 2 × 2.7 cm focal hypervascular nodule, lying peripherally at the junction of segment 7 and 8 figure . There wA double contrast MR study, using Gadolinium and resovist contrasts, confirmed the presence of a solitary 2 × 2.5 cm mass with features suggestive of hepatoma, lying within segment 7 in a subcapsular position figures . AlthougDiagnostic laparoscopy demonstrated a 3 cm exofitic dark brown splenunculus attached to the diaphragm and indenting the surface of segment 7 of the liver. Multiple other typical looking splenunculi were found. Intraoperative ultrasound was performed and excluded any other lesions within the liver or surrounding tissues.The lesion was easily resected laparoscopically and shaved from the live surface with no need for a liver resection. The histopathological assessment confirmed the diagnosis of splenunculus, with no evidence of neoplasia.The patient finished a 24 weeks course of pegylated interferon α and ribavirin as part of his hepatitis C treatment regime post operatively. At two years his follow up showed no radiologic evidence of HCC, and his LFTs and AFP were within normal limits.Splenosis is the heterotropic implantation of splenic fragments onto exposed vascularised peritoneal and intrathoracic surfaces, following splenic injury or elective splenectomy. This can occur anywhere within the abdominal cavity and the resultant splenunculus will receive its blood by parasitizing the surrounding tissue.There are few previous reports of hepatic splenosis mimicking hepatocellular carcinoma ,12,13. IDistinguishing the nature of a hepatic mass is important because it significantly alters patient management. In this case, if a diagnosis of HCC was confirmed, this patient would be suitable for resection (Child-Pugh class B) or for a liver transplant, satisfying the Milan criteria. Liver cirrhosis, having recent LFT derangement and with the above radiological picture made HCC strongly suspected. However, the pervious history of traumatic splenic rupture and the presence of multiple splenunculi within the abdominal cavity suggested that the best way to proceed would be for a laparoscopic exploration and a secure diffinition of the lesions nature before planning for future management.Laparoscopy was sufficient in confirming diagnosis of splenosis, as well as excluding coexistent malignancy. This had a significant impact of clinical plans and patients management. It is already recognized that laparoscopy provides a port of minimally invasive entry for the visualisation of suspect masses, and allows access for potential subsequent biopsy or resection.The abnormal liver function behavior in this case can be explained by an active hepatitis C process, which have improved following further anti viral treatment. Yet, a low threshold for HCC is a must with any similar scenario of suspicious liver function tests and radiological findings.Laparoscopic resection of symptomatic or suspicious splenosis is a minimally invasive and feasible procedure. This was reported to be a successful diagnostic and interventional tool even in laparoscopically challenging scenarios involving the pancreas ,19.To the best of our knowledge, this is the first case where laparoscopy has been the main tool in difining the correct diagnosis in a case of splenosis, suspected to be an HCC on radiological investingations and strong clinical bases.We therefore propose that laparoscopic investigation should be part of a new approach for investigating suspect intrahepatic masses.Hepatic splenosis is not a rare event and should be considered with the differential diagnosis in case of suspected lesions especially in patients who had previous splenectomy. Correct diagnosis is essential and can significantly influence patient management. We propose that laparoscopic investigation should be part of a new protocol for confirming the diagnosis of suspected intrahepatic masses.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MAH wrote the paper. AH was responsible for literature review, medline search and wrote the first draft. BZ wrote the case history and collected all clinical information. JNP reviewed the article and made suggestions. NWP was the surgeon, reviewed the paper and made suggestions. BS was the radiologist who selected the images and commented on the manuscript.


Lower Spigelian hernia is a very rare entity. The clinical findings are similar to those of inguinal hernias and in many cases may be misdiagnosed. In the literature, only a few references to this entity have been reported in children. To the best of our knowledge, this is the first case report of a lower Spigelian hernia in a child who presented with an acute painful scrotum.We discuss the case of a 6-year-old Greek boy who presented to our emergency department complaining of severe pain in the left inguinal area and scrotum. The acute painful swelling started suddenly, without any obvious cause. The initial diagnosis was incarcerated inguinal hernia which was reduced with difficulty. Five days later, the patient still experienced mild pain during palpation and he was operated on. During the operation, a large lower Spigelian hernia was revealed and reconstructed.Although Spigelian hernias are rare in children and difficult to diagnose, physicians should be aware of them and include them in the differential diagnosis. Lower Spigelian hernia is a rare entity in pediatric surgery, and is often misdiagnosed as an inguinal hernia . Less thA 6-year-old Greek boy presented with a painful intumescence of the left inguinal area. The child was initially referred to a general hospital where the physicians suggested that the problem was an incarcerated left inguinal hernia. The hernia was reduced with difficulty by the local surgeon and the next day, the child was referred to our hospital for further evaluation and treatment. In the physical examination, the left inguinal area appeared normal without signs of intumescence. The only findings were subcutaneous edema and a hematoma, probably due to the manipulations for the reduction. During palpation, the child presented with mild discomfort and pain in the left testicle without clinical evidence of testicular torsion. These symptoms persisted for 3 days and finally we decided to operate on the patient. A lower Spigelian hernia was identified during the operation. The hernia sac was found to penetrate from a small defect (with an estimated diameter of about 1.5 cm) in the Spigelian fascia within Hesselbach's triangle  may contribute to the pre-operative diagnosis in selected cases. A CT scan may be useful in the diagnosis of an incarcerated Spigelian hernia mainly due to its ability to demonstrate the layers of the anterior abdominal wall ,12.Reduction of the content with excision of the sac and closure of the fascia defect with non-absorbable sutures is the most common and widely accepted surgical approach, presenting the lowest recurrence rates ,3,7. RecSpigelian hernia occurs through a congenital or, more often, an acquired defect in the Spigelian fascia, lateral to the rectus muscle sheath. It is very rare in children. The case that we present is unique because it is probably the first report of a lower Spigelian hernia presenting as acute scrotum. No similar cases have been reported in the literature. The aim of the present case report is to alert the physician of this rare entity because, although it is difficult to diagnose pre-operatively, accurate diagnosis and appropriate treatment are essential to avoid future recurrences.SH: Spigelian hernia; CT: computed tomography.The authors declare that they have no competing interests.All authors contributed equally to the treatment of the patient . EC, NP and GF wrote the draft. SR and DF approved it. GF, DS and DF carried out the revision.Written informed consent was obtained from the patient's parents 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.


Intra-abdominal lymphangiomas usually present by 2 years of age and are uncommon in adults. Cystic lymphagiomas arising from the lesser sac are even more uncommon. We report an unusual case of a lesser sac cystic lymphangioma presenting as acute appendicitis. A 21 year old female was admitted with pyrexia, right iliac fossa tenderness and an elevated C-reactive protein (CRP). At laparotomy, a large fluid filled cystic lesion was observed occupying the right side of the abdominal cavity. The lesion was excised in its entirety and histological diagnosis confirmed lymphangioma. The patient remains well with no evidence of recurrence 1 year post resection. Lymphangiomas are rare, benign tumours of the lymphatic tissue, most commonly found in the neck. It usually presents in childhood, and most cases are seen within the first two years of life. Intra-abdominal lymphangiomas are not common . LymphanA 21 year old female student was referred by her general practitioner with a three day history of central abdominal pain which moved to the right iliac fossa after two days. On examination, she was pyrexial with a temperature of 38.2°C and acutely tender in the right iliac fossa. Blood inflammatory markers revealed normal white cell counts but an elevated C-reactive protein (CRP) of 342 mg/l. She was admitted with the working diagnosis of acute appendicitis and a decision was made to carry out an emergency appendectomy.Upon induction of anaesthesia, it was noted that there was a large mass within the abdomen. The mass extended from the epigastrium to the hypochondrium and was fluctuant. A midline laparotomy was subsequently performed.Upon entry into the abdominal cavity, it was noted that a large multi-loculated cyst was occupying most of the right side of the abdomen. On closer inspection, the cyst originted from the lesser sac and extended to the hypochondrium and right iliac fossa. The cyst had an extensive vascular supply and was filled with a foul smelling yellowish fluid. The cyst was completely excised and measured roughly 25 cm × 15 cm (Figure Histopathological examination of the cyst wall demonstrated loose fibrocellular connective tissue with prominent vasculature with areas of severe inflammation. On the basis of these findings, the cystic lesion was diagnosed as a benign cystic lymphangioma with inflammatory changes.The patient remains well with no evidence of recurrence 1 year post resection.Lymphangiomas are benign lesions of the lymphatic system whose exact aetiology remains uncertain. 90% of lymphangiomas occur in patients less than two years old, most commonly in the neck or axilla, in locations of primitive lymph sacs and are often referred to as 'cystic hygromas'. Intra-abdominal lymphangiomas are uncommon . LymphanThere have been no previous cases recorded in the literature of cystic lymphangiomas arising from the lesser sac presenting as acute appendicitis in an adult. The cause of this unusual presentation is likely infection of the contents of the cyst with subsequent inflammation.Surgical excision is the optimal treatment for symptomatic lymphangiomas and often total excision of the lesion is readily possible with cystic lymphangiomas . ExcelleIn conclusion, cystic lymphangiomas arising from the lesser sac are rare tumours in adults with the potential to present acutely.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BT was involved in the patient care, acquisition of data and writing of the manuscript. TL contributed to the writing of the manuscript. All authors read and approved the final manuscript.


Gorham's disease is a rare bone disease, is characterized by the proliferation of thin-walled vascular channels associated with regional osteolysis. There have been fewer than 150 cases reported in the literature. Shoulder and pelvic region is common site. Skull is the least common site of involvement.We describe a case of a 35-year-old female of Indian origin presented with this rare condition involving her left parietal bone. She was treated successfully with excision of diseased bone and cranioplasty.This is the first reported case in India. We provide a review of the clinical, radiological and pathological diagnosis of this rare condition and describe treatment options. Gorham disease  is a rare disorder characterized by a nonfamiliar, histologically benign vascular proliferation originating in bone and producing progressive resorption of all or a portion of the bone . This unA 35-year-old female of Indian origin presented with complaint of depression over left parietal area noticed accidentally about four years back. At that time the size is about a coin. It is slowly and gradually progressive in nature. At present it is about 4 × 4 cm in size. There were no other symptoms at this time duration. There was no history of any trauma. Her neurological examination was normal. Local examination shows a well-defined nontender rounded left parietal depression of 4 × 4 cm in size without any soft tissue mass with normal overlying scalp. Plain x-ray skull was showing a well-demarcated rounded lytic area in parietal region with normal margin. Figure  There isth postoperative day.A left parietal craniectomy was performed, a bony defect of 4 × 4 cm with a very thin remainder of the inner table was found. There is involvement of overlying galea and subgaleal tissue in form of thickening and increased vascularity was found. Figure  The inneJackson first reported a case of massive osteolysis of the humerus in a 12-year-old boy . In 1955The proposed pathogenesis of Gorham's disease is nonmalignant, neoplastic proliferation of hemangiomatous or lymphangiomatous tissue -8. Some Clinical manifestations include pain, swelling, or a pathologic fracture, whereas others may be asymptomatic or have an insidious onset of soft tissue atrophy -9. ThereThe osseous deformity or pathologic fracture in Gorham disease is common, but more serious complications are infrequent ,8,9. ParThe initial radiologic feature of Gorham disease may reveal radiolucent foci in the intramedullary or subcortical regions that resemble osteoporosis ,10. SubsThe decreased uptake over the left parietal area seen in our case may indicate decreased in bone in that region. CT with bone window setting provides delineation of the extent of osteolysis, and 3D imaging is useful for surgical planning for skull reconstruction. MR imaging, however, allows superior delineation of bone marrow and soft tissue involvement. Although other pathologic conditions in the skull such as juvenile Paget disease, eosinophilic granuloma, Brown tumor, and osteolytic metastases may have similar imaging findings, the long asymptomatic clinical course and typical CT and MR imaging findings may allow differentiation of Gorham disease.The diagnosis of Gorham's disease is one of exclusion, based mainly on clinico-radiological findings, evolution and compatible histological findings. The aim of this case report is to emphasize the Gorham's disease as a rare differential diagnosis for skull lesions.Written informed consent was obtained from the patient for publication of this case report and accompanying images. Patient has given their informed consent for the case report to be published. The copies of the consent documentation available with corresponding author on request at any time.The authors declare that they have no competing interests.PV analyzed and interpreted the patient data regarding the Gorhams disease and performed the surgical procedure. YYR was a major contributor in writing the manuscript. SD made correction and approved the final manuscript.


Cantrell's pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken. We discuss the case and present a brief review of literature and of embryogenesis Of all congenital anomalies, ectopia cordis, i.e., the presence of a live, beating heart outside the thorax, is perhaps the most distressing; it gives a grotesque appearance to neonate. This condition is extremely rare and is usually associated with Cantrell's pentalogy or a variant of Cantrell's pentalogy with sternal defect. We discuA 20-hour-old full-term male neonate presented to us with complaints of an externally visible, beating heart over the chest wall and difficulty in respiration. The neonate was delivered at home by a normal vaginal delivery and had a birth weight of 2.5 kg. He was the first issue of his 28-year-old mother. The antenatal history was unremarkable and there was no history of exposure to any unusual infection or drug. Antenatal ultrasonography had been done by a local practitioner but the deformity was not detected, probably due to lack of experience. There was no family history of any congenital abnormality. At the time of presentation, the neonate had peripheral cyanosis and there was history of difficulty in breathing and of vomiting since birth. The beating heart, covered with a serous membrane, was visible in the middle of the chest wall. The lower half of the sternum and the upper abdominal wall was deficient and was covered with a thin membrane. The umbilical cord was attached at the lower part of the abdominal wall defect near the epigastric region and the abdomen was scaphoid . Other tThe pentalogy of Cantrell is an extremely rare congenital anomaly. The complexity of this syndrome is usually incompatible with life, so the exact incidence could not be found in the literature; however, an incidence of 1:100000 births, with a male preponderance M : F = 2 : 1.2), has been described in literature in developed countries. : 1.2, h DisplaceThe entire group of anomalies would appear to be closely related in embryologic development, arising as the result of defective formation and differentiation of the ventral mesoderm at about 14–18 days of embryonic life.14; these vClass 1: Exact diagnosis, with the five defects presentClass 2: Probable diagnosis, with four defects  presentClass 3: Incomplete diagnosis, with combination in the defects The occurrence of congenital intracardiac anomalies is a constant element of this syndrome; an interventricular septal defect was present in every instance in which a description of the heart was available, and was also found in our case.3 Other i21et al.[Although most cases of the ectopia cordis appear as isolated, sporadic defects, other associated anomalies, including chromosomal abnormalities (trisomy 18), are reported in literature. Carmi etet al. describeet al. In our cet al.[In our case, the neonate had thoracoabdominal ectopia cordis with Cantrell's pentalogy and, therefore, he belongs to class 1 of the embryological classification. In developing countries like ours, there is a lack of adequate health services in remote rural areas. In the absence of antenatal diagnosis, the neonate was delivered at home without qualified medical assistance. He presented late with severe respiratory acidosis and dyselectrolytemia which resulted in the fatal outcome. Ghidini et al. describeet al. To concl


Liposarcoma occurs most commonly in the extremities and retroperitoneum, however, it has been rarely observed in the colon.A case is reported a 41-year-old man with liposarcoma of ascending colon which was presented as hemoperitoneum and combined with a different histological type of retroperitoneal liposarcoma. He visited hospital with right lower abdominal pain and palpable mass. Laboratory data including tumor markers were within normal limits, and computed tomography revealed a 15 × 10 cm sized enhancing soft mass. Right hemicolectomy was performed, and after that, a further large retroperitoneal mass was revealed and this was also radically excised. Mixed-type colon liposarcoma and well differentiated type of retroperitoneal liposarcoma were diagnosed in pathologic report. The patient has remained free of disease for 24 months.No standardized guidelines have been established for its treatment because too small a number of cases have been reported, but surgical resection was considered the treatment of choice. Liposarcoma is a common sarcoma of the soft tissues in adults, and it occurs most commonly in the extremities and retroperitoneum. HoweverA 41-year-old man visited the emergency room with abdominal pain and a palpable abdominal mass for 1 day. He had no history of disease apart from an appendectomy performed 1 year ago, and histological examination of the appendix showed features of appendicitis only. A large, soft, tender mass was palpable in the right lower abdomen. Laboratory data upon admission were within normal limits, and tumor markers such as carbohydrate antigen (CA) 19-9, carcinoembryonic antigen (CEA), and alpha-fetoprotein (AFP) were also normal. A few hours later, he complained of severe abdominal pain, and the abdominal mass was enlarged. Computed tomography (CT) revealed a 15 × 10 cm slightly enhancing solid mass, with a cystic lesion that was located in the ascending colon, without any enlarged lymph nodes. Some high-density fluid collections were observed in the pelvic cavity  was revealed and this was also radically excised.Macroscopic examination of the ascending colon revealed a large, lobulated, dumbbell-shaped soft mass that measured 20 × 13 × 10 cm, along the mesenteric border Figure . A portiAfter surgery, the patient was discharged without any complications, and has been well, without any evidence of recurrence or metastasis for 24 months with outpatient follow up every 6 months.Liposarcoma is one of the most common soft tissue sarcomas, and represents 20% of mesenchymal malignancies. It tends to occur in the retroperitoneum and deep soft tissues of the trunk and extremities in adults. It is uLiposarcomas rarely involve the gastrointestinal tract-16, and Clinical features of primary liposarcoma of colon are variable and nonspecific: abdominal pain, diarrhea, weight loss, anemia, and hematochezia, constipation and sometimes, an abdominal mass may be palpable Table 5-13]. H-13. H5-1Most studies have treated primary liposarcoma of the colon with complete wide excision-13; in oThe prognosis of primary liposarcoma of the colon is not known. In the first case reported by Wood and Morgenstern, tumor rThe present case is extremely rare because it is mixed type, presenting as hemoperitoneum and was combined with a different histological type of retroperitoneal liposarcoma. No standardized guidelines have been established for its treatment because too small a number of cases have been reported, but surgical resection was considered the treatment of choice in our case. A larger number of cases will be necessary to determine whether additional treatment such as chemotherapy and radiotherapy is necessary or effective in liposarcoma of colon.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journalThe authors declare that they have no competing interests.YYC participated in data collection and conceived the design. SYJ, pathologist, assembled pathologic data and reviewed pathology. YJK is the operating surgeon, collected data and participated in critical review. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/10/239/prepub


Adrenal cortex oncocytic carcinoma (AOC) represents an exceptional pathological entity, since only 22 cases have been documented in the literature so far.Our case concerns a 54-year-old man with past medical history of right adrenal excision with partial hepatectomy, due to an adrenocortical carcinoma. The patient was admitted in our hospital to undergo surgical resection of a left lung mass newly detected on chest Computed Tomography scan. The histological and immunohistochemical study revealed a metastatic AOC. Although the patient was given mitotane orally in adjuvant basis, he experienced relapse with multiple metastases in the thorax twice in the next year and was treated with consecutive resections. Two and a half years later, a right hip joint metastasis was found and concurrent chemoradiation was given. Finally, approximately five years post disease onset, the patient died due to massive metastatic disease. A thorough review of AOC and particularly all diagnostic difficulties are extensively stated.Histological classification of adrenocortical oncocytic tumours has been so far a matter of debate. There is no officially established histological scoring system regarding these rare neoplasms and therefore many diagnostic difficulties occur for pathologists. Hamperl introduced the term "oncocyte" in 1931 referring to a cell with abundant, granular, eosinophilic cytoplasm . NeoplasA 54-year-old man was admitted in the Thoracic and Vascular Surgery Department of our hospital with a 2 cm mass at the upper lobe of the left lung detected on Computed Tomography (CT) scan to undergo complete surgical resection. He had a past medical history of adrenocortical carcinoma (AC) treated surgically with right adrenalectomy and partial hepatectomy en block 2 years ago Figure . He was S 20.8, 1:20, DAKO), EMA , CEAm , CEAp , TTF-1 , Chromogranin  and S-100  were negative. Based on the morphological and immunohistochemical features of the neoplasm and the patient's past medical history, other oncocytic tumours were excluded and the diagnosis of a metastatic AOC was supported. Mitotane oral medication was given in adjuvant setting (2 g/d).Overall physical examination showed neither specific abnormality, nor any signs of endocrinopathy. All laboratory tests including cortisol, 17-ketosteroids and 17-hydrocorticosteroids serum levels and dexamethasone test, full blood count and complete biochemical hepatic plus renal function tests were in normal rates. The patient was subjected to wedge resection. Histological examination revealed a tumour with an oxyphilic cell population, moderate nuclear atypia, diffuse, rosette-like and papillary growth pattern and focal necroses Figure . A numbeSeven months later, a new right lower lobe mass of 1.5 cm diameter was found on follow-up CT scan. A second wedge resection was performed including an excision of a nodule infiltrating the diaphragm. The histopathological examination confirmed diagnosis of AOC.A new CT scan, six months later, demonstrated a lobulated mass, 2.8 cm in diameter, at the lingula and a lymph node block, measuring 11 × 5.5 cm at the preaortic space extending to the aortopulmonary window Figure . The pat2 q 21 days) followed by three cycles of epirubicin (50 mg/m2 q 21 days) and etoposide  combined chemotherapeutic regiments, concurrently with radiotherapy of right hip. No severe toxicity was stated. The therapeutic schedule combination with ongoing, orally given, mitotane was completed uneventfully. The patient remained in a good performance status (PS: 0) for 16 months and finally died, approximately 5 years post his disease onset, due to massive recurrence.Three and a half years post first surgery, a right hip joint metastasis was revealed on CT scan Figure . The patAdrenocortical tumours are usually solitary lesions and in their vast majority occur in adults without sex predilection . SeveralOncocytic variants of adrenocortical neoplasms are a special subgroup and whether the Weiss system can be used to evaluate their clinical behaviour is under consideration by several authors ,24,25,36More recently, Bisceglia et al. proposed new Weiss modified criteria and clearly determined the terms AO, AONUMP and AOC. More specifically, they suggested the following: a) if one of the criteria defined as major  is present in an AON, the latter should be considered malignant, b) if one to four of the criteria defined as minor  is found, the tumour should be deemed of uncertain malignant potential, and finally c) lack of both major and minor criteria indicates a benign lesion .The role of the proliferative index (Ki-67) and oncoprotein p53 has also been a controversial issue in the past years. Some authors have suggested that these markers could be used as potential indicators of the benign or malignant nature of ACs -39. BiscLiterature review revealed 22 cases of AOC so far -29. All In our case, based on the exclusively oncocytic cell features of the neoplasm, a differential diagnosis among oncocytic tumours, either primary of the lung or metastatic, was needed. The patient's medical history and the neoplasm's immunohistochemical profile clarified its adrenocortical origin, its local infiltrative presentation and its malignant metastatic behaviour.There is a wide discussion about the multimodality therapeutic approach which is needed apart from the radical surgical excision of the primary AC tumour, its local recurrences and relevant metastatic involved sites ,43. RadiIt is the first time, in our knowledge, that an AOC was submitted to consecutive resections due to metastatic infiltration of both lungs and mediastinal lymph nodes, as if it was a primary lung cancer. In our case, neoplasm spreading may originate in carcinomatous emboli that entered the inferior vena cava; however, lymph node invasion has not been previously described at such a distant site. It is noteworthy that although this neoplasm had aggressive behaviour with constant relapse, the patient's performance status remained well. This fact dictated the aggressive surgical practice.Histological classification of adrenocortical oncocytic tumours has been so far a matter of debate. There is no officially established histological scoring system regarding these rare neoplasms and therefore many diagnostic difficulties occur for pathologists. Metastatic disease is the only definite criterion of malignancy. Molecular biology and large clinical studies may probably provide in the future more precise criteria for the classification, clinical behaviour and therapeutic approach of AOCs.AC: Adrenocortical Carcinoma; AO: Adrenocortical Oncocytoma; AOC: Adrenocortical Oncocytic Carcinoma; AON: Adrenocortical Oncocytic Neoplasm; AONUMP: Adrenocortical Oncocytic Neoplasm of Uncertain Malignant Potential; CT scan: Computed Tomography scan; HPF: High Power Field.Written informed consent was obtained from the patient's relatives for publication of this case report and the 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.PA did the macroscopic and microscopic examination of the specimens, collected and reviewed the literature data, prepared the figures, drafted, wrote, typed, formatted and revised the manuscript. CDP did the macroscopic and microscopic examination of the specimens, put the diagnosis, made the design, revised and supervised the manuscript. CZ operated on the patient, participated in the literature review and drafted the case presentation apart from the histopathological part. NA elaborated and revised the manuscript critically for stylistic imperfections, participated in the literature review and wrote the part of the manuscript regarding therapy approach. EMK operated on the patient, collected clinical data and participated in the literature review. CG was the attending oncologist, provided relevant clinical information and participated in the literature review.


Any errors occurring during this process can cause dissemination of aberrant nodules that are responsible for most atypical thymomas.The thymus arises in the ventral portion of the third and fourth pharyngeal pouch. It descends into the anterior mediastinum at 6The authors report a unusual case of type-A thymoma located in D10 and D11 vertebral bodies.The histology showed a uniform growth of short, spindle shaped, mitotically inactive cells. A few small, normal lymphocytes were seen scattered or in small groups. The immunohistochemical investigation for neuroectodermal, neuroendocrine, vascular and muscular markers were negative. It also confirmed the presence of CD3+, CD5+ T lymphocytes and the absence of immature T-lymphocyte markers.The case described shows a thymic hystogenesis for spindle cell tumours. To our knowledge no other cases of vertebral thymomas have been described in international literature. Any errors occurring during this phase can cause dissemination of aberrant nodules that are responsible for most uncommon thymomas [The thymus arises in the ventral portion of the third and fourth pharyngeal pouch. It descends into the anterior mediastinum at 6thymomas . Seldomlthymomas , pulmonathymomas , pleura thymomas , and in thymomas . We descA 59-year-old woman presenting with a tumour mass located in D11 with partial invasion of D10 causing middle back pain one year before.There was no family history of bone lesions or bone tumours. There were no general symptoms, such as an increase in temperature or loss of weight or appetite.Clinical examination results showed localized pain increasing with compression. A systemic examination was performed and tested negative. Laboratory studies were not helpful. X-rays revealed an osteolytic lesion eroding the anterior cortex, the superior endplate involving the upper vertebra fig. . The CT-The mass was angiographically quite avascular suggesting a low grade lesion, according to the FDG-PET which demonstrated low metabolic activity and no signs of dissemination. As a tumour was suspected a CT-guided needle-biopsy was performed under local anesthesia through the left pedicle.Histopathological tests showed a mitotically inactive spindle cell tumour, however, no definite diagnosis could be performed as material was scant and the immunohistochemical findings were inconsistent.Hemangiopericytoma and paraganglioma were excluded because of low vascular grade; a metastasis from renal carcinoma, before hypothesized was excluded because contrasted with angiography and FDG-PER-FDG pattern. Having an uncertain diagnosis, a tumour mass has to be treated as a primitive tumour with therapeutic intent thus an en-block vertebral resection was proposed. The patient was informed about the high surgical and post-surgical risks and would not accept undergoing the procedure, so therefore a less dangerous palliative surgical procedure was chosen. During the first surgical operation a laminectomy and a posterior stabilization were performed without carrying out a biopsy, so that the tumour would not spread to the posterior access.Curettage and stabilization with a titanium cage, cement and anterior plate was performed about two months later by intercostals-lateral approach.Postoperative inpatient time followed without any problems and the patient was discharged after ten days in order to start rehabilitation.Histopathological features were done on the curetted material, and pin-pointed the localization of ectopic thymomas so that a meticulous immunohistochemical test panel was applied to confirm the hypothesis.An indirect immunoperoxidase staining was performed on 2 μm formalin fixed paraffin embedded sections by a streptavidin-biotin enhanced immunoperoxidase technique  in an automated auto-stainer  using monoclonal and polyclonal antibodies directed to the following antigens: CD99 , chromogranin A , Smooth Muscle Actin  without any retrieval pre-treatments, HMB45 (clone HMB45 Dako), Synaptophysin , CD31 , CD34 , pan-cytokeratin , keratin 19 , keratin 7 , Vimentin , CD56 , EMA , Bcl2 , CD20 , CD1a , CD68  after pre-treatment of the sections in a thermostatic bath at 96° C for 40 min in a pH 6 citrate buffer, CD5  after pre-treatment of the sections in a thermostatic bath at 96° C for 40 min in a pH 8 EDTA buffer, S100 , Calponin , pan-cytokeratin , keratin 8 , collagen IV  after enzymatic pre-treatment. Diaminobenzidine (Menarini) was used as chromogenic substrate.Under microscope, the tumour showed a uniform growth of short, spindle shaped, mitotically inactive cells. A few small, typical lymphocytes were seen scattered or in small groups fig. .An hemangiopericytic pattern was formed by the short, spindle-shaped cells with bland vesicular fused nuclei. In some areas a gap was found between the epithelial sheets and the tiny-walled vessels.The immunohistochemical investigations tested negative for neuroectodermal , neuroendocrine , vascular  and muscular  markers, whereas the spindle cells were consistently positive for cytokeratin cocktails MNF116, AE1/AE3, for cytokeratin 19 and cytokeratin 7 , CD56, cytokeratin 8, BCL2 (week), were focally positive. The staining for CD20 showed an extensive multi-focal positive membranous staining of the spindle epithelial cells (fig. In situ hybridization (FISH) with the vysis probe of inter-phase nuclei in formalin-fixed, paraffin embedded sections showing negative results.A search of the SYT translocation t (WHO 2002) as typical for synovial sarcoma, performed by fluorescent In the posterior mediastinum, a variety of tumours occur, however the majority of them are of neurogenic origin, schwannomas and neurofibromas being the most frequently encountered neoplasms . In our The diagnosis of hemangiopericytoma was excluded because there were no typical spider-like radial branching vessels nor collagen IV and CD99 positivity.A melanoma metastasis was excluded due to the bland cytological appearance, the lack of mitotic activity and the S100 and HMB45 negativity. Similarly, the possibility of a solitary fibrous tumour was ruled out on the basis of the CD34 negativity and of the week positivity  of bcl2.The hypothesis that the lesion might have been a renal carcinoma metastasis, which appeared after the first surgery, was highly unlikely both from an angiographical aspect and the metabolic activity showed by FDG-PET. The diagnosis of Chordoma was excluded basing on FDG-PET activity as well as plasmocitoma that should have had no hyperfissation period in bone scan and high activity in FDG-PET.In this case, the possibility of a synovial sarcoma was ruled out not only by the bland appearance of the nuclei, but by the lack of mitotic activity and the absence of the SYT translocation typically associated to Synovial sarcoma, as demonstrated by the FISH investigation.In our case, the widespread positivity for Cytokeratins is in keeping only with epithelial tumours. Thymic epithelial tumours occur in several ectopic sites, such as inside or outside  the thoracic cavity, according to the embryological derivation of the thymus from the IV branchial cleft. In the posterior mediastinum, the occurrence of ectopic thymoma has rarely been reported, but nevertheless the para-vertebral site has to be considered as a possible site for ectopic thymomas emerging. Nevertheless, in our case the lesion is clearly located in vertebral body and not in the posterior mediastinum, furthermore the bland appearance of the fusiform nuclei, the presence of scattered typical lymphocytes, the presence of peri-vascular spaces as typical for thymic epithelial tumours (TET), the strong collagen IV deposits among the spindle cells and the extensive co-expression of cytokeratins and CD20 were considered highly diagnostic for type A thymoma according to the World Class Organization classification system of TET . The hemEstablished the thymic tissue origin of the lesion the metastatic nature was the most probable nevertheless it was excluded and the tumour considered primary because both FDG-PET and total body CT-SCAN didn't detect other lesions or ectopic thymus. The hypothesis is today confirmed by the checks performed at two years of follow-up.The long history of the present case reflects this general tendency, although a local invasive capacity has been well documented. Furthermore, the case described points considering thymic origins for spindle cell tumours with a hemangiopericytic pattern of unknown derivation.Considering the low grade of malignancy, the treatment chosen by the patient was acceptable for her disease. However an en-block vertebral resection would have decreased the risk of recurrence. A further treatment as radiation therapy was temporary excluded; the possibility to perform a second surgery and then radiation therapy was considered preferable according to the patient. In order to guarantee a correct histopathological interpretation as well as treatment, diagnosis has to be done based on patient history and clinical imaging.The authors declare that they have no competing interests.FM, MM and PV carried out the morphological and immunoistochemical studies, and participated in the sequence alignment and drafted the manuscript. CZ and NS participated in the design of the study and performed the statistical analysis. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/10/322/prepub


Heterotopic bone is rarely found in the gastrointestinal tract. Here we report a rare case of metaplastic ossification within a benign ulcerating adenoma and review the literature concerning the aetiology.A 63-year-old woman, who presented with a history of melaena, was found at colonoscopy to have a pedunculated ulcerating polyp. Histological examination demonstrated multiple areas of osseous metaplasia within the polyp stroma.Heterotopic ossification in colonic adenomas is a particularly rare phenomenon, with the majority of cases occurring within malignant lesions. The suggested mechanisms for its aetiology still remain unclear. Heterotopic bone is rarely found in the gastrointestinal tract. The majority of reported cases are associated with malignant lesions -6. ThereA 63-year-old woman presented to her general practitioner with a history of intermittent melaena. She was drinking two to three litres of gin per week and was taking ibuprofen for cervical spondylosis. She had a past medical history of alcoholic liver disease and, 6 years previously, a gastroscopy had revealed oesphagitis and duodenitis.A repeat gastroscopy revealed no abnormality. A colonoscopy was performed which revealed a pedunculated polyp in the proximal transverse colon which was subsequently excised, retrieved and sent for histological examination. The patient has subsequently had no further admissions to hospital or episodes of melaena.All sections of the specimen demonstrated an adenomatous polyp with a mostly tubular growth pattern and moderate epithelial dysplasia. Areas of surface ulceration with granulation tissue and slough were also noted Figure . The polOsseous metaplasia occurs outside the skeletal system in fibrodysplasia ossificans . The incHow this ectopic ossification occurs is unknown. It may be that the osteoblasts needed to lay down bone differentiate from fibroblasts or other precursor cells. Local osteogenic factors then stimulate these osteoblasts to incorporate collagen fibres already found at the site into new bone. Bone remodelling dependent on the balance between osteoblasts and osteoclasts occurs in areas of ectopic bone as it does in the normal skeleton .The adenomatous polyp removed from our patient had surface ulceration alongside areas of granulation and slough. It may be that the osteogenic stimulation was a result of the inflammatory process. Inflammation has previously been suggested as a trigger in a case of osseous metaplasia in an ulcer in Barrett's oesophagus  and in aHeterotopic ossification in colonic adenomas is a particularly rare phenomenon, with the majority of cases occurring within malignant lesions. The suggested mechanisms for its aetiology still remain unclear.The authors declare that they have no competing interests.VW and AS wrote the manuscript. GT performed the colonscopy. AS, JL, GT and DS reviewed the literature. All authors contributed intellectual content and have read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Gallstone ileus is a mechanical obstruction caused by the impaction of one or more gallstones within the lumen of any part of the gastrointestinal tract. Although the disorder is a rare cause of small bowel obstruction (1% to 2%), it has been reported to cause up to 25% of cases of non-strangulated small bowel obstruction in patients over 65 years of age.We report a case of a 67-year-old woman who presented with gallstone ileus following endoscopic retrograde cholangiopancreatography and sphincterotomy for choledocholithiasis. She had a history of terminal ileum resection with ileocolic anastomosis for Crohn's disease. A 3 cm gallstone was found to be impacted just proximal to the previous ileocolic anastomosis. A second gallstone was found on digital examination of the proximal small bowel.A gallstone may enter the gastrointestinal tract following endoscopic retrograde cholangiopancreatography and sphincterotomy and impact proximal to an anastomotic stricture as demonstrated here. The radiographic image of small bowel obstruction plus air in the biliary tree is a classic diagnostic finding. After stone extraction, the entire small bowel and colon should be digitally examined for further stones. Gallstone ileus, caused by migration of a large gallstone either from the gallbladder or common bile duct into the terminal ileum, is an unusual but well documented cause of intestinal obstruction in the elderly.A 67-year-old woman had been unwell for 5 days with intermittent nausea and vomiting, abdominal pain, progressive abdominal distension and obstipation. She had been having similar, though less severe, symptoms for the previous 6 months. She had a history of ERCP for common bile duct stones and terminal ileum resection with ileocolic anastomosis for Crohn's disease. The physical examination revealed a distended abdomen, with no palpable masses and no hernias. Numerous distended loops of small intestine, air in the biliary tree, and a calcified intraluminal mass were identified on the abdominal X-ray Figure . These fGallstone ileus, first described by Dr Erasmus Bartholin in 1654, is a mechanical obstruction caused by the impaction of one or more gallstones within the lumen of any part of the gastrointestinal tract. It is a rare disease and accounts for about 1% to 3% of mechanical ileus of the small bowel, but for 25% of all small bowel obstructions in patients older than 65 years [Plain abdominal X-rays may reveal signs of small bowel obstruction, calcified gallstone in the intestinal lumen  or air in the biliary system (pneumobilia). Computed tomography (CT) scans may also provide useful additional information if the diagnosis is in doubt. CT is useful for estimating the size and number of impacted gallstones and the transition point between dilated and collapsed bowel . MagnetiEnterolithotomy is usually curative. The approach to the biliary enteric fistula is arguable and somewhat contingent upon the patient's clinical status . A one-sThis case report demonstrates that gallstones may enter the gastrointestinal tract following ERCP and sphincterotomy and impact proximal to an anastomotic stricture. The radiographic image of small bowel obstruction plus air in the biliary tree is a classic diagnostic finding. After stone extraction, the entire small bowel and colon should be digitally examined for further stones.CT: computed tomography; ERCP: endoscopic retrograde cholangiopancreatography; MRCP: magnetic resonance cholangiopancreatography.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.AG and AS contributed to the writing and editing of the manuscript. Both authors read and approved the final manuscript.


We report a case of localized amyloid tumor of the jejunum which presented with abdominal pain and gastrointestinal bleeding. We reviewed the pathophysiologic process that precipitates bleeding in this rare tumor. We also examined the documented radiologic and endoscopic features of amyloidosis of the small bowel in the light of our reported case. All with a view to add to the growing evidence on this rare tumor which will facilitate accurate diagnosis and management. A 59-year old Caucasian female admitted to medical service for syncope and anemia. Initial evaluation revealed left lower quadrant abdominal pain and passage of maroon-colour stool which had been present for two days prior to the admission.She has a past medical history which is significant for undefined hypercoagulable disorder characterized by 2 episodes of pulmonary embolism, hypothyroidism, Riedel's thyroiditis for which she had thyroidectomy, depression and obsessive-compulsive disorder. She was on the following medications- Coumadin, Synthroid, and Klonopin.Physical examination revealed a middle-aged woman who was afebrile, tachycardic with heart rate of 136 beats per minute and orthostatic hypotension [supine blood pressure - 113/85 mmHg and 80/60 mmHg in sitting position].Pulmonary examination was normalAbdominal examination revealed a mildly but diffusely tender abdomen with grossly heme positive stool on rectal examination.Initial laboratory work-up showed the following:Complete blood count- white cell count 17,300/mm3, hematocrit 27.6%, platelet 167,000/mm3Coagulation profile- PT 51.4 seconds, PTT 46.5 seconds, INR 15.3Biochemical profile was normalIn view of abdominal pain and lower gastrointestinal bleeding, a computerized tomography scan of the abdomen and pelvis was ordered which revealed a mass within the small bowel associated with thickened small bowel wall and mesenteric lymphadenopathy fig .She had the anticoagulation reversed with fresh frozen plasma and her INR quickly corrected to 1.4. She also had 2 units of blood transfused which raised the hematocrit to 28.1. She underwent laparotomy about 36 hours into the admission. Operative findings include a 5 cm mass in the mid jejunum with associated mesenteric lymphadenopathy [fig Pathology showed amyloidoma associated with ulceration and extensive recent and organizing hemorrhage. Congo red staining of the tumor was done and apple-green birefringence under polarized light was demonstrated fig . AdjacenAmyloidosis often present in the systemic form while localized amyloidosis constitutes 10-20% of cases . LocalizThis is a case of localized gastrointestinal amyloidosis presenting as a bleeding tumor which clinically manifested as abdominal pain and lower gastrointestinal bleeding. We report this case with the aim to review the pathologic process responsible for amyloidosis causing gastrointestinal bleeding. The radiologic and endoscopic features of small bowel amyloidosis are also examined.Various concepts have been described in an attempt to explain why amyloidosis produces gastrointestinal hemorrhage. Amyloidosis angiopathy is described as the process by which amyloid deposition in the wall of the intestinal blood vessels causes fragility of the vessel wall and predisposes to bleeding . AnotherComputerized tomography scan of the abdomen in this patient revealed a soft-tissue density tumor in the proximal small bowel. Differential diagnoses considered by the radiologist are lymphoma, intussusception and hematoma. Other reported cases of amyloidosis in the small bowel have documented difficulty with radiologic diagnosis with radiologists opting for differential diagnoses like metastatic tumors, small bowel adenocarcinoma and lymphoma. This is not unexpected as primary small bowel tumors are uncommon and amyloid tumors of the small bowel are rare . CharactEndoscopic features like fine, granular appearance, erosions, mucosal friability, thickening of the valvulae conniventes, multiple polypoid protrusions and shallow ulcers were reported by Tada et al ,10. Our This reported case is an important addition to the growing body of evidence on gastrointestinal amyloidosis. In view of the rarity of focal amyloidosis of the small bowel and the broad range of possible clinical presentations, it becomes judicious to closely review every diagnosed case to establish consistent, common findings that will make future diagnosis of the condition easier and more accurate.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.OT and YS contributed to the review of the patient's chart, collation of the data, images and information needed for writing the case report. Both authors also contributed to writing the case report.


Gallbladder perforation is a rare clinical condition, which mostly occurs following acute cholecystitis associated with cholelithiasis. A tumor of the ampulla of Vater causes gradually progressive symptoms, and is rarely associated with perforation of the gallbladder.A 56-year-old man with carcinoma of the ampulla of Vater presented with spontaneous gallbladder perforation and localized bile peritonitis. He complained of right upper abdominal pain, and laparotomy revealed perforation of the gallbladder with no gallstones. Postoperative upper gastrointestinal endoscopy demonstrated a slightly enlarged duodenal papilla, and biopsy revealed adenocarcinoma of the ampulla. Pylorus-preserving pancreaticoduodenectomy was performed subsequently.Ampullary carcinoma can be associated with gallbladder perforation and present with acute manifestations. Immediate surgical treatment is required for this condition. Gallbladder perforation (GBP) is a rare but life-threatening condition, which usually requires immediate surgical intervention. Most cases are complicated by acute cholecystitis associated with cholelithiasis , althougA tumor of the ampulla of Vater causes gradually progressive symptoms such as jaundice or weight loss, and rarely presents with acute manifestations -6. In th3, C-reactive protein level of 0.08 mg/dl, total bilirubin level of 0.6 mg/dl, aspartate aminotransferase level of 57 IU/l, and alanine aminotransferase level of 67 IU/l. Computed tomography (CT) and echography demonstrated distention of the gallbladder and thickening of its wall and dilatation of the common bile duct, but no gallstones were detected (Fig A 56-year-old man was referred to our hospital with right upper abdominal pain, which had worsened over the previous two days. He had been free of symptoms previously. He had a history of moderate smoking and alcohol consumption, and no appreciable medical or family history. On admission, his body temperature was 37.4°C. Blood examination showed a white blood cell count of 8900/mmcted Fig .He was diagnosed with acute cholecystitis, and initially treated with fluid resuscitation and administration of antibiotics. However, the abdominal pain did not improve, and laparotomy was performed the day after admission, which revealed biliary ascites around the gallbladder and partial necrotic change in the neck and body of the gallbladder. We performed cholecystectomy and intraoperative cholangiography, which revealed no stones in the gallbladder and bile duct. The postoperative course was uneventful. Pathological examination of the resected gallbladder revealed inflammatory change of its wall, and no arterial occlusive change Fig . MicrobiIt has been reported that GBP occurs in about 5% of patients with acute cholecystitis ,7. IschePreoperative diagnosis of GBP is often difficult, which delays surgical intervention and leads to high morbidity and mortality ,7. CT anObstructive jaundice is the most common presentation of carcinoma of the papilla, followed by weight loss, abdominal pain, and nausea -6. The dAlthough extremely rare, carcinoma of the duodenal papilla can be associated with GBP and display acute manifestations. In a case of GBP without cholelithiasis, ampullary tumor should be considered as a possible underlying condition.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AH participated in the treatment of the patient, collection of case details, literature search and drafted the manuscript. MN, KS, and YM participated in the treatment of the patient and data collection, and helped to revise the manuscript. All authors have read and approved the final manuscript.