While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed. The wide varieties of rare intraocular and orbital neoplasms differ in presentation in the pediatric population when compared to these same lesions in adults. While most pediatric ophthalmic tumors are benign, they may have a significant impact on vision and may result in significant morbidity and mortality. We categorize these diseases according to etiologies as neoplastic and vascular. Some congenital tumors may present in the first year of life, while others typically present later in childhood. Clinical examination signs that should raise concern include leukocoria (white pupil), strabismus, restriction of ocular motility, asymmetric eye position within the orbit, decreased vision, high pressure in the eye, inflammation of the eyelids or conjunctiva, pseudohypopyon (inferior whitish layer in the anterior chamber of tumor cells), vitreous hemorrhage or inflammation, and an afferent pupillary defect. However, these are often late findings. Clinical presentation combined with the characteristic imaging features of the disease can narrow differential diagnoses. Imaging modalities most often used to evaluate these lesions include orbital ultrasonography (US), computed tomography (CT), and most importantly magnetic resonance imaging (MRI). Ophthalmic pathology is also critical to come to a diagnosis. In this paper, we review the epidemiology, clinical manifestations, current treatment modalities, and the imaging features that differentiate pediatric ocular and orbital lesions.RB1 tumor suppressor gene likely predispose retinal progenitor cells to tumor growth [Retinoblastoma is the most common intraocular malignancy in the pediatric population. The incidence of retinoblastoma is approximately one case per 15000–20000 live births, which amounts to 9000 new cases each year. There is no gender or racial predilection, and 90% of cases are diagnosed in patients under three years of age [r growth . Very rar growth .The most common initial sign of retinoblastoma is leukocoria, where the light emanating through the pupil is white light reflecting off the tumor instead of red light reflecting off the retina. Other signs of retinoblastoma may include decreased vision, strabismus, redness, pain, high pressure in the eye, cellulitic-like periocular inflammation, pseudohypopyon, and proptosis in late disease . Young cThe fourth pattern, diffuse infiltrating retinoblastoma, accounts for 1-2% of retinoblastomas and usually arises from the anterior retina in older children. This form usually does not form a mass or contain calcium. The resultant pseudohypopyon and floating vitreal tumor cells can be easily mistaken for uveitis or endophthalmitis and has thus been termed a masquerade disease. The absence of pain, conjunctival hyperemia, synechia (anatomic adhesions), cataract, and vitreous fibrosis suggests retinoblastoma rather than inflammation. Diffuse retinoblastomas start anteriorly and may enter the anterior chamber and fill the vitreous cavity but usually do not extend to the optic nerve. Spontaneous regression of tumor results in a shrunken, nonfunctioning globe . The diagnosis of retinoblastoma is made noninvasively by exam under anesthesia with ophthalmoscopy, orbital ultrasound, and fluorescein angiography. Lumbar puncture and bone marrow biopsies are only performed in patients at high risk for having extraocular disease. Biopsies are not performed because of the risk of extraocular spread. Typically, neoplastic cells spread through the optic pathways via the optic nerve and can breach the pia to extend into the subarachnoid space. Direct extension through the choroid and sclera can occur with subsequent orbital extension. Finally, hematogenous metastases can spread to the lungs, bones, brain, and other viscera .http://dx.doi.org/10.1155/2013/975908). More recently, the International Retinoblastoma Staging System (IRSS), proposed by a consensus of clinicians to separate retinoblastomas into stages based on management approach, has become popular [Historically, retinoblastoma was grouped into categories based on the Reese-Ellsworth classification system (groups I–V). The International Intraocular Retinoblastoma Classification System (IIRC) separates retinoblastomas into groups (A–E) based on prognosis, which is related to the extent of intraocular disease at diagnosis  or neurofibromatosis 1 (NF1).The treatment of retinoblastoma is complex and involves a multidisciplinary team, including pediatric oncologists, ophthalmologists, diagnostic and interventional radiologists, radiation oncologists, ocular pathologists, and geneticists. The available treatment options include enucleation , chemoreduction, selective intraarterial and systemic chemotherapy, laser photocoagulation, focal cryotherapy, transpupillary thermotherapy, plaque brachytherapy, and external-beam radiation therapy (EBRT). Enucleation is the treatment of choice for advanced retinoblastoma with no potential for visual salvage. Historically, chemotherapy was reserved for extraocular or metastatic disease with adjuvant chemotherapy after enucleation. Discovery that patients who underwent EBRT are at an increased risk of developing secondary malignancies led to a paradigm shift in retinoblastoma treatment . Now, syMultiagent chemotherapy usually includes a combination of carboplatin and vincristine with or without etoposide. Studies have shown that for retinoblastomas in Reese-Ellsworth groups I–III, systemic chemotherapy in combination with local ophthalmic therapies  can avoid the need for enucleation or EBRT –13. SomeMore aggressive therapy is needed for Reese-Ellsworth groups IV and V, as advanced cases with diffuse vitreous seeding are extremely difficult to treat. Subconjunctival carboplatin and intravitreal carboplatin have been used in cases of diffuse vitreal seeding in Reese-Ellsworth group Vb eyes without evidence of seeding at 37 month followup . This trCryotherapy is effective for small  peripheral tumors, and a triple freeze-thaw technique is used with a tumor control rate of up to 90%. Multiple sessions of focal laser photocoagulation can be used alone for tumors less than four disc diameters. Thermotherapy with infrared radiation may also be used. When the aforementioned local treatments fail, brachytherapy is ideal for small, discrete, and accessible tumors. In a large study, plaques with radioactive ruthenium 106 or iodine 125 had a 79% tumor control rate at 5 years . Proton Infusion of selective intraarterial chemotherapy (SIAC) is currently the new trend in retinoblastoma treatment. SIAC has been used for the primary treatment of unilateral and bilateral retinoblastoma in IIRC groups C and D with reported cures after one and two cycles of chemotherapy without major adverse events , 19. In It has recently been reported that SIAC exposes patients to a significant amount of radiation  during eThe future of ocular drug delivery now is focused on localized sustainable drug delivery directly in the eye. Transscleral depot reservoirs, liposomes, and nanoparticles are being investigated in preclinical models and may play a role in the future of treatment for intraocular malignancies like retinoblastoma. These treatments are being studied extensively for other retinal diseases with possible future applications in intraocular tumor treatment.Medulloepithelioma is a rare congenital neuroepithelial tumor that arises from the nonpigmented ciliary epithelium of the ciliary body. Rarely, it can arise from the retina and optic nerve. It is slow growing and characterized as teratoid or nonteratoid and malignant or benign, based on histologic appearance. Population-based information on incidence is not available; however, most cases are found within the first decade of life and rarely late in adulthood . This raReported medulloepithelioma cases describe a rapidly expanding ciliary body mass that masqueraded as chronic uveitis, cataract, and uncontrolled secondary glaucoma . ClinicaImaging can assist with determining extent of disease, and presence of extraocular spread of tumor with all imaging modalities typically demonstrates a solid mass with multiple characteristic cystic collections. Ultrasound biomicroscopy can be very useful in distinguishing anterior segment masses by providing high resolution images . On B-moOnce the diagnosis is favored based on location, clinical appearance, imaging, staging, and size of the tumor are evaluated. Most medulloepitheliomas are cytologically malignant, but distant metastases are rare. Nodal metastases in the neck can occur with a predilection for the lymph nodes in the parotid gland. For small tumors, local excision via iridocyclectomy in conjunction with radiotherapy has been done with varying success , 28. TheThough typically arising from the ciliary body, medulloepitheliomas have on rare occasion been reported arising from the optic nerve . Most suRetinal astrocytic hamartomas are glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. They are cream-white, elevated, well-circumscribed lesions that may be multiple or solitary. There are two types of retinal astrocytic hamartomas: small, smooth, and flat noncalcified tumors that appear to be thickening of the nerve fiber layer or “mulberry” lesions, which are nodular large yellow-white calcified lesions. They can be found incidentally on exam, often in conjunction with tuberous sclerosis (TS) and rarely neurofibromatosis . The incUsually, these lesions create no clinical symptoms, and treatment is rarely required. They can spontaneously resolve  but may Argon laser photocoagulation has been reported to cause visual stabilization, but choroidal neovascularization is a reported complication. Photodynamic therapy is effective in resorption of subretinal fluid in these cases . Rhabdomyosarcoma of the orbit represents the most common orbital malignancy in childhood, accounting for 10% of all rhabdomyosarcoma cases with mean age of 6–8 years of age , 36. ChiOn imaging, orbital CT and MRI are both helpful in evaluating extent of disease and are often complimentary. Orbital rhabdomyosarcoma appears isoattenuating to muscle on CT and is usually seen as a moderate to markedly enhancing extraconal, homogeneous, and well-circumscribed mass with calcifications only occurring with bony destruction Figures . Other cNuclear medicine bone scintigraphy is currently used as part of the work-up for children with rhabdomyosarcoma and is most accurate in detecting osteoblastic metastases. Some studies have shown that PET-CT may be better at detecting bone and lymph node metastases than conventional anatomic imaging  –43. AddiThe treatment of orbital rhabdomyosarcoma has changed drastically over the last 20 years from primary orbital exenteration to a more conservative approach combining systemic chemotherapy and radiation. Depending on the extent of tumor involvement, treatment may also involve surgical debulking. Patients routinely receive chemotherapy with vincristine and d-actinomycin . PatientNeuroblastoma is the most frequent extracranial solid tumor of childhood and arises from neural crest cells. The prevalence based on the SEER registry from 1973 to 2002 is 0.9 per 100,000 persons. The median age of diagnosis was one year, with 42% of patients presenting at less than one year of age . It is tThe most common clinical presentation of orbital neuroblastoma metastases in patients less than two years old is unilateral or bilateral proptosis and periorbital or eyelid ecchymosis (raccoon eyes). Less common clinical findings include periorbital swelling, hemorrhage, strabismus, restricted ocular motility, ptosis, atrophy of the optic head, and ocular mobility disturbance . OpsocloLike Langerhans cell histiocytosis, orbital neuroblastoma metastases tend to occur in the posterolateral orbital wall with CT and MRI providing better diagnostic information over ultrasound. On CT Figures , metastaMYCN oncogene. The prognosis of disseminated neuroblastoma is better in infants  when compared to children above one year of age . Survival and outcomes have improved substantially over the last 30 years with multimodality modern therapy, including chemotherapy, radiation therapy, surgery, myeloablative therapy with stem cell transplant, immunotherapy, and differentiation therapy [Urine catecholamines have high sensitivity and specificity for neuroblastoma. Treatment of metastatic neuroblastoma is stratified based on clinical features  and specific tumor biological markers that include histopathological analyses, chromosomal abnormalities, and quantification of expression of the  therapy , 52. In Optic pathway gliomas can involve any portion of the visual pathway, including the hypothalamus, optic disc, nerve, chiasm, geniculate nucleus, and optic radiations. Categorized as juvenile pilocytic astrocytomas, they account for 4–6% of all brain tumors in children, and the median age of diagnosis is 5–9 years , 54. AppThe vast majority of optic pathway gliomas is slow growing and can go unrecognized clinically for a long time. Patients typically present with decreased visual acuity, which may worsen with growth of the glioma within the optic pathway nerves. Other signs include optic disc edema, pallor, atrophy, relative afferent pupillary defect, decreased color vision, pupil dysfunction, visual field defects, ocular motility problems, and proptosis. If the tumor arises within the hypothalamus, endocrinopathies such as accelerated growth and precocious puberty may manifest . HypothaThe appearance of optic pathway gliomas on CT and MR is characteristic and specific for the disease process; however, MR is more sensitive for detection of smaller lesions. The gliomas typically show fusiform enlargement of the optic nerve . AdditioAdvanced MR techniques including magnetic resonance diffusion tensor imaging (MRDTI), diffusion-weighted imaging (DWI), and dynamic contrast enhancement (DCE) have been employed to further evaluate optic pathway gliomas. An early study in 2008 used DW and DCE imaging to calculate the diffusivity and permeability of optic pathway gliomas and found that clinically aggressive gliomas had significantly higher permeability than clinically stable gliomas and may warrant closer followup . A separSome gliomas enter a stage of stable or slow growth, and some have even spontaneously improved. Tumors confined to the optic nerve at presentation rarely extend into the chiasm or develop extradural extension or metastases. Given the slow growing nature of most of these tumors, active surveillance can be a reasonable option. In patients with progressive disease, chemotherapy is the mainstay of treatment, usually with carboplatin and vincristine. Radiation therapy with fractionated gamma knife radiosurgery can be used after chemotherapy failure and retards or reverses progress in many cases. However, long term-data does not suggest that this specifically reduces mortality or vision loss . RadiatiPlexiform neurofibroma (PNF) is a hamartoma of neuroectodermal origin representing 1-2% of all orbital tumors and typically arises in the first decade of life . PresencEyelid PNFs have a characteristic S shape due to thickening, fat deposition, and horizontal redundancy. Orbital involvement can cause globe proptosis, bony expansion, and sphenoid dysplasia that can lead to temporal lobe herniation and pulsatile exophthalmos , 58. ComImaging with CT or MR can help determine extent of tumor involvement, amount of bony dysplasia, and can aid in surgical planning. PNFs appear as diffuse, irregular masses that cross multiple tissue planes with other features including thickening of the soft tissues, irregular intraconal fat, irregular nodularity of the optic nerve sheath, and thickening of the sclera/choroid . Tumor cPNFs show considerable enlargement during childhood and adolescence and can result in severe disfigurement. Treatment is directed at the relief of specific symptoms. Generally, complete surgical excision of the plexiform neurofibroma is not possible. Surgical debulking and frontalis suspension procedures can reduce the ptosis and allow for binocular vision. However, the condition is often progressive. Chronically inflamed conjunctiva occasionally requires resection.Leukemia is the most common malignancy of childhood in the USA, with acute lymphoblastic leukemia accounting for 80% of all cases and acute myeloid leukemia (AML) accounting for 20% of cases , 67. In The most common manifestation is leukemic retinopathy, which presents with flame-shaped retinal hemorrhages, sometimes with white centers, in the nerve fiber layer. Additionally, perivascular infiltrations, microinfarctions, and discrete tumor infiltrations can be seen. Central scotomas and serous retinal detachments of the macula have also been reported. Anterior chamber hyphemas, pseudohypopyons, and iris masses can also be presenting signs of intraocular leukemia. Optic nerve infiltration needs to be distinguished from papilledema secondary to CNS leukemia. The less common orbital deposition of leukemic cells can present as a rapidly enlarging orbital mass, which can cause pain, eyelid swelling, ecchymosis, diplopia, and proptosis , 57. ThiGranulocytic sarcomas are somewhat irregular homogenous masses with lateral orbital predilection that tend to encase rather than invade the adjacent lacrimal gland and extraocular muscles . CT may Treatment, usually systemic chemotherapy and bone marrow transplantation, is guided by the pediatric oncologist and customized based on specific genetic features of the malignancy. A leukemic infiltrate of the optic nerve can cause optic nerve elevation, and this is a medical emergency because of potential permanent loss of central vision when left untreated. The treatment is low-dose radiation therapy often combined with intrathecal chemotherapy as soon as possible. Lymphoproliferative disease is rare in children, representing less than 5–10% of orbital lesions. Lymphoid hyperplasia accounts for 10–40%, and non-Hodgkin's lymphoma accounts for 60–90% of the lesions. Lymphoproliferative disease can be categorized into reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and ocular adnexal lymphoma. Isolated orbital lymphoma is usually a low grade B-cell lymphoma, a subtype of non-Hodgkin's lymphoma. Diffuse large B-cell lymphomas are usually systemic.These lesions are most commonly seen in the superotemporal quadrant with a predilection for the lacrimal gland; however, they may present with diffuse extraocular muscle involvement that can mimic thyroid ophthalmopathy . Patient−3 mm2/sec, and OIS cases typically have ADC values greater than 1.0 × 10−3 mm2/sec; however, overlap does exist. Lymphoid hyperplasia and lymphoma have overlapping imaging characteristics as well. Hyperplasia  is a disease characterized by abnormal proliferation of Langerhans cells and was previously thought to encompasses three clinical syndromes: Letterer-Siwe disease, which involves the vital organs of infants with often fatal outcomes; Hand-Schuller-Christian disease, seen in young children with diabetes insipidus, osteolytic calvarial defects, and exophthalmos; and eosinophilic granuloma, an indolent disease limited to the bones of older children or adults , 71. LCHImaging can help define the extent of disease and osseous destruction. CT and MRI provide more information than ultrasound. On CT, LCH manifests as a soft-tissue lesion that replaces/destroys osseous structures, can be well-defined or diffusely homogeneous, and shows moderate to marked enhancement after contrast administration Figures . The sofIsolated asymptomatic lesions may be observed. Painful lesions with favorable access can be excised or directly injected with steroids. Patients can be medically managed with systemic corticosteroids or chemotherapy, depending on the severity of the disease and location of involvement. Lesions are also treated with low-dose radiation. These patients must be followed because they can develop juxtaneural or intracranial extension and can progress to multifocal disease , 72.Infantile hemangiomas are hamartomas of capillary endothelial cells. They are the most common vascular tumor of childhood in the orbit and are classified into preseptal, intraorbital , and compound/mixed, involving both . Most cases are diagnosed within the first weeks to months of life, after which the hemangioma begins a proliferative phase for up to 10 months. During this phase, the lesion may be complicated by hemorrhage, ulceration, cause amblyopia by obstructing the visual axis or inducing astigmatism, cause glaucoma by compression of the ocular outflow channels, stretch the optic nerve, and cause corneal ulceration . After t2 with variable echogenicity. Spectral waveforms show a high-flow system with low resistance; however, no arteriovenous shunting should be identified. Imaging is indicated to assess for the depth of the lesion and its relationship with adjacent structures for any lesions with atypical features on physical exam or presentation, or an associated underlying syndrome. US is useful as an inexpensive initial exam and typically demonstrates a well-circumscribed mass with a vessel density of greater than 5 vessels/cmIf further characterization is needed, MRI offers more sensitive evaluation of deeper lesions and their relationship to adjacent structures while avoiding the ionizing radiation of CT. Additionally, MR angiography (MRA) can be used to assess flow characteristics within the vascular lesions. Typical hemangiomas are well-circumscribed solid masses with arterial flow voids, intermediate signal on T1WI, increased signal on T2WI, marked enhancement on post-contrast images, and high flow on time-resolved MRA . During If complications of the capillary hemangioma arise, intralesional corticosteroid injections into the eyelid can be safely performed but should be done with caution. Injection pressures routinely exceed the systolic blood pressure during these procedures , making Venous-lymphatic malformations (VLMs) encompass a spectrum of complex congenital lesions of the orbit that are the result of maldevelopment of lymphatic and vascular structures during embryologic life. VLMs are composed of a mixture of venous and lymphatic vessels that vary in composition based on location. Deep lesions tend to be more venous in nature, while superficial lesions contain more lymphatic components. This corresponds to the normal vessel and lymphatic distribution of the orbit. They account for 4% of all expanding pediatric orbital masses and are usually evident by two years of age .Periorbital malformations may cause major morbidity, including intralesional bleeding, intermittent swelling, blepharoptosis, fluctuating proptosis, pain, amblyopia, chemosis, astigmatism, and strabismus. Forty percent of children have diminished vision in the affected eye . SequelaThe imaging characteristics of VLMs reflect their gross appearance with the mass appearing irregular, lobulated, infiltrating with ill-defined margins (due to lack of a capsule), and involving both the pre- and postseptal as well as the intra- and extraconal portions of the orbit , 80. MacStreptococcus pyogenes. This treatment, however, generates a significant local inflammatory reaction, which can include bleeding of the lesion and an increase in size [Treatment of periorbital VLMs requires an interdisciplinary team that includes interventional and diagnostic radiologists, craniofacial surgeons, and ophthalmologists. Several interventions are usually required. Percutaneous sclerotherapy with 3% sodium tetradecyl sulphate (STD)  is an ef in size , which r in size . A coron in size .A wide assortment of intraocular neoplasms and orbital masses involves the pediatric orbit and can present with various clinical manifestations. Given the complexity of their location and considerable morbidity that may be associated with biopsy at this site, a systematic and multidisciplinary approach is needed to help facilitate an accurate diagnosis. By understanding the clinical presentation and the characteristic imaging features of the disease, a narrow differential diagnosis can be formulated, and thus timely treatment can be initiated. The treatment of pediatric orbital and intraocular neoplasms, especially of retinoblastoma, has changed drastically over the last 10 years. During this same period, wider use of 3 T magnets with newer coils has improved signal quality of MR imaging, while new 3D pulse sequences now allow slice thicknesses of less than 1 mm. Furthermore, DWI imaging has recently emerged as an important diagnostic tool used to differentiate malignant and benign neoplasms. As imaging hardware and advanced imaging techniques become more refined, diagnostic accuracy should continue to improve. Advancements in drug delivery systems and therapies will hopefully translate into decreased morbidity and mortality in this young patient population. Supplementary Table 1: The International Intraocular Retinoblastoma Classification system divides intraocular retinoblastomas into 5 groups (A through E), based on the chance that the eye can be salvaged using current treatments.Click here for additional data file.


Histopathology revealed a type III gastric carcinoid. Patient was treated with laparoscopic distal subtotal gastrectomy with Roux-en-Y reconstruction and partial omentectomy. Although there was no evidence of metastasis on initial presentation, eighteen months later, patient was found to have multiple metastatic lesions in her liver. Patient's lesions were treated with intra-arterial (hepatic artery) Yttrium-90.We report a case of a 40-year-old female who presented with dyspeptic symptoms for six months. Upper GI endoscopy revealed a submucosal nodule in gastric antrum. Using “ Gastric submucosal tumors are a common incidental finding occurring on routine upper GI endoscopies. Although different modalities for diagnosing these tumors are available, definitive diagnosis requires tissue analysis. Tissue acquisition for gastric submucosal tumors can be challenging. We report a case of a 40-year-old female who presented with dyspeptic symptoms. Upper GI endoscopy revealed a submucosal nodule in gastric antrum. Histopathology was compatible with a type III gastric carcinoid. Although there was no evidence of metastasis on initial presentation, eighteen months later, patient was found to have multiple metastatic lesions in her liver. Our case highlights the malignant potential of a gastric submucosal nodule, which may otherwise present as an incidental finding on upper GI endoscopy.A 40-year-old female presented with dyspeptic symptoms for six months. She denied any nausea, vomiting, or change in bowel habits. There was no history of hematemesis, melena, or weight loss. Physical examination revealed an obese female with normal vital signs. Head and neck exam was positive for mild pallor but no icterus, thyromegaly, or lymph node enlargement was noted. Abdomen was soft, nontender with no hepatosplenomegaly.μL, and a platelet count of 2.26 × 103/μL. Blood urea nitrogen, creatinine, and electrolytes were normal.Workup revealed a Hb of 11 gm/dL, white blood count of 11000/Helicobacter pylori related chronic active gastritis. Concurrently, a submucosal mass measuring about 2.0 cm was noted in the gastric antrum  consistent with a gastric carcinoid Figures  and 3. SSubsequently, an endoscopic ultrasound (EUS) was done that confirmed a submucosal mass slightly smaller in size than initially anticipated, arising from the third layer, and no lymphadenopathy. Biomarkers for carcinoid such as serotonin, chromogranin A, and 5-HIAA were also negative. Computer tomography of the abdomen revealed gastric antral thickening secondary to carcinoid and no evidence of extragastric extension, liver, or adrenal metastasis.Patient was treated with laparoscopic distal subtotal gastrectomy with Roux-en-Y reconstruction and partial omentectomy. Biopsy results confirmed a well-differentiated neuroendocrine tumor (NET) consistent with type III gastric carcinoid, restricted to submucosa, without involvement of the muscularis propria . Twenty-Postoperatively, patient did well but complained of some nausea. An upper gastrointestinal series was performed, which ruled out leakage from the anastomotic site . PatientEighteen months later, patient presented to the hospital with progressively worsening generalized abdominal pain for one month. She complained of occasional nausea but denied any change in her appetite or weight. Physical examination was unremarkable. Computer tomography of the abdomen showed multiple, small, ill-defined, and low attenuating lesions in the left lobe of liver and a 1.7 cm mass in small bowel mesentery .Magnetic resonance imaging revealed several hepatic lesions, with the largest lesion measuring 2.1 × 1.4 cm in left lobe of liver. Multiple lymph nodes in small bowel mesentery and porta hepatis were also enlarged .A computer tomography guided core biopsy of hepatic lesions was performed, and results confirmed metastatic lesions, secondary to gastric carcinoid . PatientPatient has been following with us for over two years, and her lesions have been stable so far.Gastric submucosal tumors (SMTs) are a common incidental finding occurring on routine upper GI endoscopies. The exact prevalence of these lesions is uncertain, although one retrospective study reported an incidence of 0.36% .The differential ranges from benign lesions such as fibroma, lipoma, leiomyoma, varices, and heterotopic pancreas to malignant or potentially malignant lesions like lymphoma, gastrointestinal stromal tumors (GISTs), carcinoid, neurofibroma, schwannoma, and so forth. Extraluminal compression secondary to visceral structures can also appear as a submucosal nodule on endoscopy. The most common source of extraluminal compression is from spleen and splenic vessels . AlthougCertain risk criteria for malignancy in a submucosal nodule have been established on EUS. These include size > 3 cm, inhomogeneous echo pattern, irregular margins, and presence of lymph nodes . LesionsCross-sectional imaging like CT and MRI can be used to evaluate the extent of metastasis for malignant tumors like GIST . HoweverGastric carcinoids are rare lesions that constitute less than 1% of all stomach neoplasms. They arise from enterochromaffin-like cells lining the gastric mucosa. Carcinoids can be subtyped into three categories. Type I and type II carcinoid account for 80% of these lesions and are associated with atrophic gastritis and Zollinger Ellison syndrome respectively. Management includes endoscopic resection followed by endoscopic surveillance. Type III carcinoid accounts for 15–20% of these tumors and has the highest rate of metastases (>50%). Most of these tumors are metastatic at the time of presentation. Treatment of this subtype requires surgical resection. On endoscopy, carcinoids appear as polyploidal masses with a normal overlying mucosa . They usGastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the GI tract. The annual incidence is estimated to be at least 10 to 20 cases per million . Most GITissue acquisition for a gastric SMT can be challenging. The yield of standard endoscopy is usually poor . EUS guiAlthough cytological examination is usually sufficient to make a diagnosis of GIST, differentiation between benign and malignant stromal tumors requires histopathological and immunochemical analysis. Pathological assessment of GIST requires immunohistochemical staining for c-KIT CD 117). 95% of GISTs are positive for C-KIT. Immunohistochemical stains can also be used to differentiate GISTs from endoscopic differentials like lymphoma and inflammatory polyp. However, there are some GISTs that are negative for C-KIT. In these tumors, DOG 1 gene expression can be used. DOG 1 has a greater sensitivity as compared to C-KIT , 15. Ki-. 95% of Various factors such as size of lesion, site (lesions in lower third of stomach are difficult to sample), number of needle passes, on-site cytopathologist, and cytological versus histopathological assessment can affect the outcome of EUS-FNA. Side effects of EUS-FNA include bleeding and infections. EUS Doppler before EUS-FNA can prevent rupture of a varix, which otherwise might be mistaken for a submucosal nodule .EUS guided trucut needle biopsy (TCB) has been used for acquisition of core tissue specimens. Procedural difficulties such as needle stiffness and lesions in distal stomach can pose challenge for an endoscopist. The combination of both EUS-FNA and EUS TCB has been found to be superior to either technique alone . CombiniJumbo biopsy forceps can be used for obtaining tissue from deeper layers of the gastric wall. In a retrospective study by Buscaglia et al., out of the 129 patients with subepithelial lesions of the upper and lower GI tract that underwent EUS with biopsy using jumbo forceps, 58.9% of patients had a definitive diagnosis . The resTissue diagnosis of a gastric SMT can be challenging. Use of jumbo biopsy “unroofing technique” seems to be an attractive option for diagnosing these tumors. On-site “touch preparation cytology” has shown to further increase the accuracy.


Mesenteric panniculitis is a rare chronic fibrosing inflammatory disease that typically affects the adipose tissue and mesentery of the small intestine but may also affect the mesosigmoid and the mesocolon. The pathology of this disease remains unclear despite association with some malignancies or inflammatory disorders. We report a case of mesocolic panniculitis and a literature review of its clinical presentation, imaging findings, associated conditions and treatment options.A 64 year-old Caucasian man was admitted to the gastroenterology department for severe weakness, left lower quadrant abdominal pain, weight loss and diarrhoea. Physical examination revealed a palpable firm mass occupying the entire left part of the abdomen. Abdominal CT-scan showed fatty infiltration of the mesosigmoid and left mesocolic fat which was strongly suggestive of panniculitis. Laparoscopic surgery revealed an inflamed and edematous mesocolon and mesosigmoid; the sigmoid mucosa appeared petechial which was suggestive of venous ischemia. Histological examination of surgical biopsies revealed mesocolic panniculitis. Despite exhaustive investigation, no associated conditions were found and the cause was classified as idiopathic. Surprisingly, the patient clinically improved without therapeutic intervention other than supportive care.Although mesenteric panniculitis is most often a radiographic diagnosis without clinical symptomatology, it can also present with significant general status alteration. We report a case of mesocolic panniculitis complicated by development of an inflammatory mass associated with ischemic colitis. Mesenteric panniculitis is a difficult diagnosis to make which typically requires histologic confirmation. The overall prognosis is good with supportive treatment. Mesenteric panniculitis (MP) is a benign fibro-inflammatory process involving adipose tissue of the mesentery which is characterized by the presence of fat necrosis, chronic inflammation and fibrosis. This term is also improperly used to characterize the involvement of the mesosigmoid and the mesocolon, peripancreatic and omental fat and less frequently retroperitoneal or pelvic fat. We report here an unusual case of MP located in the mesentery of the sigmoid and left colon presenting with ischemic colitis. This rare observation of mesocolic panniculitis (MCP) was notable for the reversible ischemic colitis and abdominal pseudotumoral mass related to this condition. Here we highlight the differential diagnosis of this rare disease and review the published literature.A 64 year-old Caucasian man was admitted to the gastroenterology department for severe general weakness, left lower quadrant abdominal pain, weight loss  and diarrhoea for four weeks prior to admission. There was no history of melena, bloody diarrhea or fever. His past medical history included only type 2 diabetes for which he received oral antidiabetic agents. He had no cardiovascular risk factors and did not consume alcohol. He had travelled to Tunisia one year previously. Physical examination revealed a palpable firm mass occupying the entire left part of the abdomen without evidence of bowel obstruction. Blood tests showed an elevated C-reactive protein level  without leucocytosis and a decreased potassium level. Hepatic and pancreatic enzyme levels and renal function tests were normal. The hepatitis B, C and HIV serological tests were negative. Blood and stool bacterial cultures as well as Clostridium difficile toxin A and B were all negative. Contrast-enhanced thoraco-abdominal CT-scan showed a thickening of the wall of the sigmoid and ascending colon without contrast enhancement, an increased density of the thickened mesosigmoid and mesocolon and ascites, all of which suggested the diagnosis of MCP Figures  and 1B. To definitely eliminate infectious or neoplastic disorders, a laparoscopic surgery was performed. The mesosigmoid and mesocolon were markedly thickened and retractile. The normal architecture was lost and was replaced with an irregular nodular mass which was difficult to move. The adjacent bowel wall was thickened, edematous and congested, with a petechial aspect. There was a moderate amount of serous ascites in the peritoneal cavity. No neoplasia, diverticula, abscesses or enlarged lymph nodes were identified. Histological examination of surgical mesocolon biopsies showed scattered areas of steatonecrosis with lipid-laden macrophages, lymphocytes and congested capillaries Figure  and 2C. MP is a benign, tumor-like mesenteric lesion, formed as result of chronic inflammation of adipose tissue; the etiology is unknown. In the literature, the disease is named with a number of synonyms: mesenteric lipodystrophy and sclerosing or retractile mesenteritis, each of which is a histologic variant based on the relative amounts of inflammation, fat necrosis and fibrosis ,2. WhileMP has a slight male predominance and commonly occurs in late adulthood ,3,4. TheAbdominal CT and magnetic resonance imaging (MRI) play important roles in suggesting the accurate diagnosis and can be used to distinguish MP from other diseases with similar imaging features such as carcinomatosis, carcinoid tumor, lymphoma or desmoid tumor. The “fat ring sign”  and the finding of “tumor pseudocapsule” (hyper-attenuated stripe partly surrounding the mass) are considered to be suggestive of mesenteric involvement . HoweverA recent study by Zissin et al.  suggesteThus, a biopsy should be performed in symptomatic patients to confirm the diagnosis of MP, because imaging specificity is limited since a broad differential diagnosis exists for mass lesions of the mesentery, particularly mesenteric tumour involvement .The pathological mechanism of MP is not clearly known but would appear to involve a nonspecific response to a wide variety of stimuli. Daskalogiannaki et al.  reportedMP has been included in the field of fibrotic disorders such as retroperitoneal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis and orbital pseudo tumour . The patSeveral therapies have been proposed to treat MP but no consensus has been established since no one therapy has been shown to be superior to the others. Treatment may include steroids ,16,30,31The surgical approach should be limited to biopsy of the mass; resection or bypass procedures are proposed only in cases of bowel obstruction or perforation . AlthougMP is a rare entity that occurs independently or in association with other disorders. This process mainly affects the mesentery of the small intestine but involvement of the colon is not uncommon. Diagnosis is a challenge despite the help of imaging tools, and histological analysis is necessary in some symptomatic cases in order to rule out treatable alternate etiologies. Overall the prognosis is usually good with supportive treatment, even in symptomatic cases like the one reported here.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.MP: Mesenteric panniculitis; MPC: Mesocolic panniculitis.The authors declare that they have no competing interest.AD for design and drafting of the manuscript. SA for his expertise on imaging of the patient and his help in drafting the manuscript. JV for the histological diagnosis, the histological figures and the revision of the manuscript. PA-S for acquisition, analysis, interpretation of data and tight surveillance of the patient. OE for the macroscopic description of the mass, the surgical treatment and the revision of the manuscript. BG took in charge the patient until the final diagnosis, has been involved in drafting the manuscript and its revision, and gave final approval of the version to be published. All authors read and approved the final manuscript form.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/12/59/prepub


Introduction. Although pleomorphic adenoma is the most common neoplasm of the salivary glands, this tumor most commonly involves the minor salivary glands of palatal and rarely occurs in cervical region. Case Report. A 21-year-old female referred to our clinic due to painless mass of right upper region of neck. After paraclinical and pathologic evaluation, it was diagnosed as cervical pleomorphic adenoma. Conclusion. Pleomorphic adenoma may be rarely involving the neck. Although the prognosis is good, the choice treatment is the complete resection of the tumor. There are nearly 450 to 750 minor salivary glands throughout the upper aerodigestive tract . AberranOne type of these neoplastic lesions most commonly involving the salivary glands is Pleomorphic adenoma. This tumor also has been called benign mixed tumor. Pleomorphic adenoma constitutes about 75% of all benign tumors of the major salivary glands. This tumor most commonly occurs in the minor salivary glands of palatal area –3.A 21-year-old female patient was referred to our clinic in Imam Reza hospital with painless mass of right upper part of her neck from seven years ago. No significant changes had developed in its size during this period. Physical examination revealed a mass in the upper border of thyroid cartilage next to bifurcation of common carotid. The mass appeared with no tenderness. It was also mobile, firm, and nonpulsatile. There was no bruit in auscultation. The examination of the head and neck including nasopharynx, oral cavity, hypopharynx, and larynx was normal. All of the routine blood chemistry tests were normal.CT scan of the neck showed a 36 × 48 mm soft tissue mass on the right side of the neck, in association with extra sheath of carotid on the sternocleidomastoid muscle with medial displacement of carotid vessels . The masThe patient underwent total resection of the mass with diagnosis of primary cervical tumor. Intraoperatively, the tumor was completely placed in the cervical region far from the tail of parotid.Histopathological investigation confirmed the pleomorphic adenoma diagnosis .The case report was approved by Ethics Committee of Tabriz University of Medical Sciences and the patient gave informed consent for this case presentation.Pleomorphic adenoma (benign mixed tumor) is the most common neoplasm of the salivary glands. They originate from an uncommitted reserve cell of the intercalated duct which has the potential to differentiate into epithelial and myoepithelial cells –5. PleomClinically, pleomorphic adenoma is characterized as a painless, slow-growing mass. Multiple primary pleomorphic adenomas are extremely rare. Pathologically, they are solitary, firm, round tumors. The cut surface is characteristically solid and can be hard, rubbery, or soft in consistent with whitish gray to pale yellow color. In contrast of major solitary glands, they are usually noncapsulated in minor solitary glands .Common sites that minor salivary gland tumors arising are the palate, followed by the maxillary sinus, lip, cheek, tongue, and so forth .Ishikawa et al. in Sao Paulo Medical University reported a 55-year-old man with nasal obstruction of many years ago that finally diagnosed as pleomorphic adenoma of maxillary sinus . KhademiDuring recent years, some other cases have been reported in rare regions such as oropharynx including tongue base , buccal  Pleomorphic adenoma may be rarely involving the neck. Although the prognosis is good, the choice treatment is the complete resection of the tumor. But, because of recurrence potential of this tumors, long-term followup is necessary.


Dermoid and epidermoid cysts in the oral cavity frequently develop in the midline or sublingual region of the floor of the mouth. Here, we report a rare case of an epidermoid cyst in the submandibular region. The patient was a 69-year-old man with a chief complaint of a mass in the right submandibular region. A mobile, elastic, relatively soft mass without tenderness was palpable in this region. The skin covering the mass was normal. MRI showed a cystic lesion measuring 3.5 × 3.0 cm under the platysma in the right submandibular region. Cystectomy was performed under general anesthesia. There was no adhesion to surrounding tissue and the right submandibular gland was preserved. The surgical specimen was cystic and contained soybean cord-like materials. Histopathologically, the cyst wall was lined by stratified squamous epithelium with no skin appendage, suggesting an epidermoid cyst. The postoperative course was uneventful and without recurrence after 28 months. Dermoid and epidermoid cysts in the oral cavity frequently develop in the midline or sublingual region of the mouth floor –6. In thHere, we present a rare case of an epidermoid cyst in the submandibular region.A 69-year-old man presented with a 2-year history of a mass in the right submandibular region. He had no symptoms, but had recently noticed the slow enlargement of the mass. He was referred to our hospital in January 2011. He had no significant medical history. Initial examination showed a mobile, elastic, relatively soft mass measuring 4.0 × 3.5 cm in the submandibular region. The mass was not tender and the skin moved freely over the mass . There wContrast-enhanced CT showed a mass measuring 3.5 × 3.0 cm with a clear boundary and regular margin positioned under the platysma anterior to the right submandibular gland . The marSurgical excision was performed under general anesthesia in March 2011. A skin incision parallel to the lower border of the mandible was made above the mass, and then the division was advanced to the lower layer of the platysma and the mass was excised with ease. There was no adhesion with the submandibular gland and it was possible to preserve the gland, including the capsule. The postoperative course was uneventful with no recurrence after 28 months.Macroscopically, the surgical specimen was a cyst of 4.0 × 3.0 × 2.5 cm with a smooth surface  and a reDermoid and epidermoid cysts can develop in any region of the body. For congenital cases, the mechanism of development involves embryonal aberration of residual ectodermal tissues, while for acquired cases, the cyst is thought to be derived from epithelial tissue aberration in the dermis due to trauma, inflammation, or surgery . The midA dermoid or epidermoid cyst arising in the lateral side of the submandibular gland, as in our case, is extremely rare. There has been one previous report of a dermoid cyst , which wSubmandibular gland tumor, ranula, malignant lymphoma, metastatic lymph node, hemangioma, and branchial cleft cyst require differentiation from dermoid and epidermoid cysts in the submandibular region. In our case, CT and MRI revealed a cystic lesion located anterior to the submandibular gland. A branchial cleft cyst was suspected based on the frequency of this cyst in the neck although development is usually along the anterior border of the sternocleidomastoid muscle. Diagnosis is difficult using imaging and clinical findings alone, and total excision is required for definite diagnosis. The recurrence rate of an epidermoid cyst is low, but malignant transformation may occur . In our


We report a case of a 50-year old female presenting with a giant tumor on the volar aspect of the third finger of the left hand, a thorough clinical and paraclinical evaluation followed by surgical resection resulted in a benign lipoma with an uneventful postoperative course. We present this case due to its rare location and repercussion in the decision making process when other more common similar pathologies with varying prognosis are conceived. Excluding cutaneous malignancy, 95% of tumors of the hand are of benign origin. Non-neoplastic ganglions are probably the most common on hand and wrist. After these tumors, inclusion cysts, warts, giant cell tumors, granulomas and hemangiomas follow in frequency. Lipomas are benign mesenchymal neoplasms occurring in areas of abundant adipose tissue. They are not very common in the hand, when present, they predominate in the thenar and hypothenar regions (Al Qattan et al. A 50-year-old otherwise healthy female arrived at our Plastic Surgery Department, with a one year history of a painless slow growing tumor on the volar aspect of the third finger of the left hand Figure  that proThe presenting lesion was a 50 × 20 mm subcutaneous mass in the proximal and middle phalanx, with a firm consistency but mobile over the underlying structures; she had discrete erythema and bluish skin color without palpable pulse, thrill or bruit. She presented limited movement in flexion of the interphalangeal joint and fingertip paresthesia. There was no local hyperthermia or manifestations of systemic disease.The Roentgenogram showed a soft tissue swelling of low density over the middle phalanx, with no bone alteration.An ultrasound scan showed a well circumscribed isoechoic well defined ovoid mass, with a thin capsule and scarce septations, without alteration of the surrounding tissues Figure .Figure 2It was decided to perform an excisional biopsy with a zealous and careful dissection, with clear identification of the neurovascular structures and tendons, the tumor was nourished by two pedicles that emerged from ulnar and radial digital bundle branches Figure .Figure 3Pathology result reported as a lipoma that measured 53×25 mm with no neural component or malignant transformation Figure .Figure 4She had an uneventful postoperative course, followed by complete movement and function of the finger. The reported fingertip paresthesia disappeared.There was no evidence of recurrence of the tumor during the 12 months of postoperative follow-up with excellent range of motion and sensivity Figure .Figure 5Lipomas account for approximately 16% of soft tissue mesenchymal tumors. They usually develop as a well-circumscribed, encapsulated mass with a doughy feel that is freely mobile underneath the skin. According to the 2002 World Health Organization’s committee for the Classification of Soft Tissue Tumors, they are categorized into 9 entities, including lipoma, lipomatosis, lipomatosis of nerve, lipoblastoma, angiolipoma, myolipoma of soft tissue, chondroid lipoma, spindle cell/pleomorphic lipoma and hibernoma. Benign lipomatous lesions affecting bone include intraosseous lipoma, parosteal lipoma and liposclerosing myxofibrous tumor. Benign lipomatous lesions may also affect joints and tendon sheaths, in a focal, or more commonly, a diffuse pattern. They are rarely encountered in the hand and with very less frequency in the digits. Its etiology is unknown, but multiple causative factors have been proposed which include genetic, traumatic and metabolic triggers. They appear mostly in the fifth and sixth decade. Presenting superficially or arising from the subfascial deep plane, in rare cases they can originate from juxta-articular regions or adjacent to the periostium as a parosteal lipoma or erode into the bone. Previous studies have defined a giant lipoma of the upper extremity as a mass larger than 5 cm (Cribb et al. Lipoma in the hand typically presents with painless swelling and usually attains a large size by the time patients seek medical attention. Clinically, it has been described with grasping difficulties, decreased digital flexion and deviation of the fingers (Brand & Gelberman Imaging studies are diagnostic in 71% of the cases, (Murphey et al. Surgical resection is the treatment of choice, it requires extensive dissection and mobilization of the neurovascular structures to accomplish success. Physician-patient relationship is key for the understanding of potential loss of function and other sequela. The main indication for their removal is the disturbance caused in hand functionality and cosmetic appearance (Higgs et al. In this case, the patient sought for medical attention when movement limitation appeared with no other systemic association. It is clear that other neoplastic lesions and non-neoplastic lesions appear with clinical characteristics similar to those of a lipoma and should be considered as a differential diagnosis. When evaluating a tumor in the upper extremities the main concern is to rule out malignancy, which changes decision making and prognosis dramatically. Although lipoma is considered a rare entity in the hand, the surgeon should be aware of their existence due to its benign nature and favorable outcome before making radical decisions; as in this case, in which complete tumor resection lead to cure of the pathology with excellent cosmetic and function result.In conclusion, lipomas of the digits are rare but benign tumors with a very limited risk of malignant transformation, they are associated with an excellent prognosis after successful excision; however, the preoperative evaluation requires a careful diagnostic workup due to its shared similarities to other pathologies.Written informed consent was obtained from patient for publication of this report and any accompanying images.


Eosinophilic esophagitis (EE) is an increasingly recognized primary clinicopathologic disorder of the esophagus which lacks a specific etiology. Most reports on EE have been limited to the esophagus mucosa. We present a 56-year-old man with the mural form of EE and superficial squamous cell carcinoma in the esophagus. The eosinophils diffusely invaded the full-thickness of the esophagus, mainly infiltrating the muscularis, including the skeletal and smooth muscles. The lesions in the mucosa, submucosa, and adventitia were slight. Although the superficial squamous cell carcinoma was excited by an endoscopic biopsy, there were some changes in the architecture and size of the squamous epithelial cells. The changed cells also expressed the p53 protein. It appears that the eosinophils stimulated cell proliferation, followed by genetic mutations and cancer development. The patient survived with resection of the esophagus and inhaled corticosteroids. Eosinophilic esophagitis (EE) was first described by Furuta et al. and deemed a variant of eosinophilic gastroenteritis . Since tA 56-year-old man presented as having abdominal distention with no reason for six years. Chinese medicine helped to relieve his symptoms. He had no other complaints, although six months prior, his skin had started itching, and three weeks prior he felt unable to successfully swallow hard food but could swallow semifluid food. He also had no history of asthma or allergies. The man went to the local hospital and had a gastroscopic examination, which showed a mass of about 1.3 cm × 1.2 cm in size , locatedThe esophagus was resected, which was 20 cm in length and 3 cm in circumference. The entire esophagus was very hardened and thick. There was mucosal erosion of about 1.2 cm × 1.1 cm in size , locatedFive tissues were biopsied . Two tisAfter esophageal resection, there were mucosal coloboma and no remnants of squamous cell carcinoma . The str9). A subsequent blood smear examination showed 50% (absolute number was 3.70 × 109) eosinophils (9)  . After tEosinophilic esophagitis (EE) is defined as a primary clinicopathological disorder of the esophagus. It is characterized by esophageal and/or upper gastrointestinal tract symptoms associated with esophageal mucosal biopsy specimens containing ≥15 intraepithelial eosinophils/HPF (high-power field) in one or more biopsy specimens. It is also characterized by the absence of pathologic gastrointestinal reflux disease, as evidenced by normal pH monitoring of the distal esophagus, or lack of response to high-dose proton pump inhibitor medications . In our EE should be distinguished from gastroesophageal reflux disease (GERD). In our case, the patient had no symptoms of heartburn and regurgitation. Eosinophils diffusely invaded the middle and lower esophagus, generally exceeding 15/HPF. The blood eosinophils returned to normal when the patient was treated with inhaled corticosteroids, so GERD was excluded. Eosinophilic gastroenteritis (EGE) was also a possibility as this condition involves the esophagus. However, although the patient had peripheral blood eosinophilia, he had no complaints of abdominal pain, nausea, weight loss, or diarrhea. In addition, there were no eosinophils in the epiploon, and the surgeon found that the stomach was soft, so the diagnosis of EGE was removed as well. Eosinophilic fasciitis also involves the esophagus. However, the antinuclear antibody, anti-DNA antibody, and rheumatoid factor were normal, so a diagnosis of eosinophilic fasciitis was not made.The cause of EE is still uncertain. Fifty to eighty percent of EE patients suffer from other allergic conditions , and antEosinophils can be present in malignant tumors. As we know, malignant tumor cells can release the cytokines, eotaxin, IL-5, and eosinophil chemotactic factor of anaphylaxis (ECF-A), which stimulates the recruitment of eosinophils from circulation into the tumor site. However, there is no case report about EE-associated superficial squamous cell carcinoma of the esophagus in English literature. In our case, the patient's clinical history showed that EE existed first, and squamous cell carcinoma confined to the mucosa developed later. The relationship between EE and esophageal squamous cell carcinoma is unclear. In EE, basal cell hyperplasia correlates with the density of intraepithelial eosinophils . In the In our case, the patient could not successfully swallow hard food. This symptom may be related to injury of the muscle and nerve plexus of the muscularis. After the surgery was complete and inhaled corticosteroids were taken, there were no further complaints of abdominal distention and itching skin. The patient survived with no recurrence of his condition.


Fibroids are the commonest uterine neoplasms, occurring in 20% - 30% of women of reproductive age. In women who have pelvic masses of unknown cause, unusual manifestations of fibroids such as necrosis or degeneration may simulate a carcinoma or hydrometra resulting in problems with image interpretation. We report a case of an unsuspected large degenerated uterine fibroid in a lady mistakenly diagnosed as hydrometra on computed tomography scanning. A 34-year-old nulliparous woman presented with sudden onset of severe abdominal pain and a six months history of progressive abdominal swelling. Her menstruation cycle was normal and regular. 5 years prior to admission she had undergone a right oophorectomy for a benign mucinous cystadenoma. On admission she was pale (Hb 9.4g/dl) with a large firm, tender and fixed pelvic mass corresponding to 36-week gravid uterus. Urine pregnancy test and serum tumour markers were negative. A clinical diagnosis of twisted ovarian cyst was made.Contrast enhanced spiral CT scan of the thorax, abdomen and pelvis revealed an enlarged lobulated uterus with a large (17.1 x 16.0 x 20 cm) fairly oval central hypodense lesion (30 – 70 HU) stretching the myometrium . In addiMagnetic resonance (MR) imaging of the pelvis was performed to determine the cause of endocervical canal obstruction. It showed an enlarged uterine outline with 2 masses; a large left mass occupying almost the entire anterior wall of the uterus with compression and displacement of the endometrium posteriorly , and a sRetrospective 3D multiplanar reformation (MPR) of the CT dataset, confirmed the MRI findings. In addition numerous blood vessels were noted in the posterior uterine wall supplying the lesions .Patient had total abdominal hysterectomy following failure of a trial of gonadotropin-releasing hormone (GnRH) analogues. Laparotomy confirmed multiple large uterine fibroids. Histopathological examination showed well-circumscribed fibroids with large areas of necrosis and hyalinisation with surrounding inflammation. The endocervical glands and the endometrium were normal. A diagnosis of multiple fibroids with a large degenerated component was made. Patient recovered well.Fibroids are classified as submucosal, intramural or subserosal based on their location. Intramural fibroids are within the substance of the myometrium and are the most common type but often asymptomatic. It has been estimated that 20%-50% of women with fibroids present with symptoms such as menorrhagia, dysmenorrhea, pressure, urinary frequency, pain, infertility, or a palpable abdominal / pelvic mass . Pain ocEven though fibroids are often found incidentally on CT, it is not the primary modality for diagnosing or evaluating fibroids. The common CT features include; uterine enlargement with a lobulated outer contour, focal myometrial thickening, deformed endometrial cavity, foci of punctate or amorphous coarse calcifications, and abnormal density within the soft tissue mass . A fibroOther differential diagnoses of a hypodense lesion in the uterus on CT include leiomyosarcoma and hydrometra. Leiomyosarcoma occur in less than 1% of all cases and is impossible to distinguish it from a benign degenerating fibroid on imaging . HydromeIn conclusion, fibroid degeneration with varying degree of liquefaction may simulate a hydrometra on CT. Recognizing the CT features on both hyaline degenerated fibroid and hydrometra are important. The application of 3D MPR, we believe can improve interpretation and diagnostic accuracy of CT.


Intestinal stomas are commonly used in a temporary or permanent fashion in gastrointestinal surgeries. The complication rate of stomas has been reported to vary between 23 and 50%. There is only one case in the literature involving transstomal small bowel evisceration following colonic perforation. In this paper, we aimed to present a patient with a perforated colon secondary to ischemic colitis, which resulted in small bowel evisceration through this perforation site. Intestinal stomas are commonly used in gastrointestinal surgeries. Common complications of stomas on the abdominal wall include herniation, retraction, stenosis, necrosis, and prolapse. There is only one case in the literature involving transstomal small bowel evisceration following colonic perforation. However, this is the first report after perforation due to ischemic colitis. In this paper, we aimed to present a patient with a perforated colon secondary to ischemic colitis, which resulted in small bowel evisceration through this perforation site.A 76-year-old man with an end colostomy was admitted to the emergency department due to evisceration of the small bowel through the stoma. The patient had received surgery by Hartmann procedure (sigmoid colon resection and end colostomy) 11 months previously because of sigmoid colon necrosis, and he had been discharged 10 days after the operation without any complications. The histopathologic material obtained from the patient had been found to be consistent with ischemic gangrenous colitis. Clinical history of the patient revealed diabetes mellitus and smoking, but he mentioned no symptoms for this period until the abrupt prolapse of the intestines through the intestinal stoma during walking.The initial examination revealed that the small bowel loops were eviscerated through the stoma, and the intestinal loops were ischemic and edematous while also being irreducible into the abdomen . The patCommon complications of stomas on the abdominal wall include herniation, retraction, stenosis, necrosis, and prolapse . ComplicIn the literature, there is only one case of transstomal small bowel evisceration secondary to events such as acute intestinal obstruction, sudden increase of abdominal pressure, and prolapsing distal colonic lumen . MoreoveIschemic colitis is the most common form of ischemic injury to the gastrointestinal tract. This disease has various clinical subtypes which can present in a wide range of pathologies from transient segmental colopathy to fulminant gangrenous colitis . The pat


Pheochromocytoma is a rare cathecolamine tumor that presents with hypertension and the triad of headaches, palpitations, and sweating. We present a case with shock and tachycardia as the manifestation of pheochromocytoma.12 year old female sought consult at the ER due to loss of consciouness. In the past, other symptoms included bouts of fatigue, headache, pallor, palpitations and profuse sweating of two years duration. The patient had history of body weakness, abdominal pain and vomiting associated with dyspnea hours prior to her admission. She had history of pheochromocytoma for which a left adrenal mass was excised three years prior her present admission. She then lost to follow-up after her operation. On clinical examination, the patient was drowsy, incoherent, diaphoretic, cyanotic, tachypneic (30 beats/min), tachycardic (200 beats/min) and no blood pressure appreciated. Her weight was 26 kg and height of 140 cm (-2SD), BMI: 13.2 (-2SD) Eye exam noted a grade III retinopathy. Initial blood work-ups showed anemia, hypoglycemia and hypocalcemia. Echocardiography showed concentric LV enlargement, systolic dysfunction, tricuspid regurgitation with LVEF of 31%. The CKMB was elevated but the troponin I was normal. Fluid resuscitation was initiated and intravenous inotropes agents were administered. Thereafter, her hemodynamic condition improved gradually and later found to have hypertensive episodes (range: from 120/100-180/120) even after discontinuing the inotropes. In view of hypertension and history of left adrenal tumor, work up for pheochromocytoma was done. Biochemical evaluation for pheochromocytoma revealed elevated 24-hour urine free metanephrines of 16.61mg . The computed tomography (CT) of the abdomen revealed a right suprarenal mass measuring 5.7 x 4.7 x 4.7cm. Patient was started on Terasozin and Carvedilol with normalization of blood pressure and resolution of other symptoms. She underwent laparoscopic mass resection. During tumor manipulation, the patient had several hypertensive crises (247/147mm Hg) which was treated with nitroglycerin and nicardipine drip. Post resection of the tumor, there was hypotension (60/40mmHg) which was given dopamine drip. The surgical specimen weighed 37.5grams, measured 5.5 x 4 x 2.3cm and histopathological examination confirmed the diagnosis of pheochromocytoma. At OPD, patient has no BP elevations and repeat 24 hour urine free metanephrines was normal at 0.06mg . Currently she is maintained with Hydrocortisone at (8mg/m2/day).This is a rare manifestation of pheochromocytoma and can be a challenge to the clinicians. Recurrent pheochromocytomas are unlikely in children but recurrent tumors may appear years after initial diagnosis.


Castleman's disease is an uncommon lymphoproliferative disorder of unknown etiology, most often involving the mediastinum. It has 2 distinct clinical forms: unicentric and multicentric. Unicentric Castleman's disease arising from an intrapulmonary lymph node is rare, and establishing a preoperative diagnosis of this disease is very difficult mainly due to a lack of specific imaging features. We report a case of intrapulmonary unicentric Castleman's disease in an asymptomatic 19-year-old male patient who was accurately diagnosed by preoperative computed tomography (CT). The mass was incidentally found on a routine chest X-ray. A subsequent dynamic CT showed a well-defined, hypervascular, soft-tissue mass with small calcifications located in the perihilar area of the right lower lung. Three-dimensional CT (3D-CT) angiography indicated that the mass was receiving its blood supply through a vascular network at its surface that originated from 2 right bronchial arteries. The clinical history and CT findings were consistent with a diagnosis of unicentric Castleman's disease, and we safely and successfully removed the tumor via video-assisted thoracoscopic surgical lobectomy. This case shows that the imaging characteristics of these rare tumors on contrast-enhanced CT combined with 3D-CT angiography can be helpful in reliably establishing a correct preoperative diagnosis. Castleman's disease, also known as giant lymph node hyperplasia and angiofollicular lymphoid hyperplasia, is an uncommon lymphoproliferative disorder of unknown etiology that was first reported by Castleman and Towne in 1954 , 3. MostA 19-year-old, nonsmoking, male patient was referred to our department for further evaluation of an abnormal shadow on a routine chest radiograph. The patient was asymptomatic with no past medical history, and physical examination findings were unremarkable. His hematological and biochemical parameters were within normal limits. Plain chest radiograph showed a tumor mass in the right infrahilar region . ComputeThe patient's history and the findings of hypervascularity with small calcifications in the intrapulmonary soft-tissue mass supported a diagnosis of unicentric Castleman's disease. We performed video-assisted thoracoscopic surgery and found an elastic hard tumor that was tightly adhered to the intermediate-to-common basal bronchi. The tumor had abundant arterial supply from the 2 right bronchial arteries. The tumor was totally removed via a right middle-lower lobectomy. Macroscopically, the cut surface of the lesion was yellow-white, well-circumscribed, and encapsulated and measured 5.2 × 5.0 × 3.8 cm . HistoloUnicentric Castleman's disease is a localized form of hypervascular lymphoid hyperplasia. In cases of intrapulmonary disease, it arises from a lobar or segmental lymph node of the lung –10. EstaThe unicentric and multicentric forms of Castleman's disease have 3 histologic subtypes: hyaline vascular, plasma cell, and mixed. Unicentric Castleman's disease is usually of the hyaline vascular type and multicentric disease of the plasma cell or mixed type. Unicentric Castleman's disease presents as a slow-growing mass that is usually found in asymptomatic young adults, without gender or race predominance , and it The most common site of involvement of unicentric Castleman's disease is the mediastinum (70%) , but it The difficulty of diagnosis of intrapulmonary unicentric Castleman's disease without a tissue specimen is also a concern with these hypervascular masses because of the risk of massive hemorrhage during biopsy by transbronchial needle aspiration or during excision by thoracotomy. Nontissue diagnosis is very difficult mainly because of the lack of reliable specific findings on most imaging studies –10. It iThe imaging characteristics found in our patient were almost the same as those described previously, which enabled us to establish a correct preoperative diagnosis, despite the unusual tumor location of involvement, and to our knowledge, this is the first report to describe the 3D-CT angiographic features of a unicentric Castleman's disease arising from an intrapulmonary lymph node. Although 3D-CT angiography did not clearly demonstrate a homogeneous capillary blush in our case, we believe that, combined with the other findings described herein, it did provide a less invasive, more efficient, and safer alternative to conventional angiography in formulating a preoperative diagnosis and an appropriate surgical plan. We found that the angiographic features of 3D-CT angiography were useful for diagnosing and successfully treating this rare case of intrapulmonary unicentric Castleman's disease. We report a rare case of unicentric Castleman's disease arising from an intrapulmonary lymph node. The findings of hypervascularity at the surface of a well-delineated homogenous mass accompanied by small calcifications and located in the perihilar area of the lung proved to be reliable imaging characteristics in guiding our diagnosis of this rare tumor. Castleman's disease should be part of the differential diagnosis when an intrapulmonary mass with the features described in this report is found in a young patient. Complete surgical resection guided by an accurate preoperative diagnosis will achieve the best results.


TP53 gene mutation (exons 4–10) was not detected in either specimen. The patient had leptomeningeal dissemination and died 26 months later.We report a case of a 20-month-old girl with a large choroid plexus carcinoma arising in the left lateral ventricle and an adrenocortical tumor. Following brain tumor resection, the patient was treated with radiation and chemotherapy. The adrenocortical tumor was found with the manifestation of precocious puberty.  Choroid plexus tumors are relatively uncommon intraventricular neoplasms of neuro-ectodermal origin, accounting for less than 1 % of all intracranial tumors. Most cases occur in children under 2 years of age –7. Some A 20-month-old girl was admitted because of a 3-week history of gradual progression of occasional vomiting and right hemiparesis. Magnetic resonance imaging (MRI) revealed a 7-cm-diameter mass with contrast enhancement in the atrium of the left lateral ventricle Fig. a and a 1Histologically, the surgical specimens in the initial operation were characterized by papillary and tubular structures lined by single or multiple layered epithelia Fig. a. NucleaOne month after the last operation, the patient developed signs of premature puberty such as pubic and axillary hair growth, hypertrophy of the clitoris and acne. Endocrinological studies revealed exceedingly high values of plasma testosterone at 3.3 ng/ml, the normal level being less than 0.1 ng/ml, and dehydroepiandrosterone-sulfate (DHEA-S) at 21,200 μg/dl, the normal range being 20–119 μg/dl. Abdominal MRI disclosed a large right adrenal mass 5.0 cm in diameter Fig. a. The adTP53 gene within exons 4–10 was carried out with hot-start PCR polymerase . Sequencing reaction  was analyzed using a capillary sequencer . No mutation was detected in any of the DNA samples (exons 4–10) extracted from the specimens , and also from her white blood cells. Amplification of the MRI obtained 5 months after the last operation for the brain tumor disclosed leptomeningeal metastases. After additional radiation therapy and five cycles of chemotherapy including etoposide and carboplatin, tumor regression was observed. Nevertheless, she eventually succumbed to disseminated disease 26 months after the initial surgery.CPC is the malignant counterpart of choroid plexus tumors and histologically corresponds to WHO grade III . This caCPC in children generally follows an aggressive course , and theChildhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low. According to the International Pediatric Adrenocortical Tumor Registry, they typically present during the first 5 years of life and have female predominance. Hormonal hyperproduction is almost universal, and most patients (84.2 %) present with virilization . PediatrTP53 mutation [Sandrini et al.  mentionemutation . Thus, tTP53 germline mutation in our case, this case is likely to be a rare coexistence of CPC and adrenocortical adenoma in an infant. However, both tumors are exceedingly rare; it is possible that the patient had other unknown genetic predispositions toward malignancy.Since we failed to show In conclusion, we should know this type of coincidence of tumors in infants.


Pneumatosis intestinalis (PI) is an uncommon condition and can be associated with a wide spectrum of diseases, ranging from life-threatening to innocuous conditions. We report the case of a 46-year-old women coming to our attention for an acute abdominal pain, nausea, vomiting, constipation, and increased inflammatory marks, with a CT showing pneumoperitoneum and pneumatosis intestinalis. The previous diagnosis was advanced neoplasia of unknown origin. Despite the surgical intervention, which excluded an ischemic colitis, the patient died in the early postoperative period. The postmortem diagnosis was carcinoma of thymus gland, and the presence of pneumatosis was put down to metastasis nodes in the pulmonary parenchima. This case demonstrates the wide spectrum of presentation of pneumatosis intestinalis, the importance of a careful radiologic evaluation beside the clinical history, since the identification of correct pathogenesis and treatment can be very difficult. Pneumatosis intestinalis (PI) is an uncommon condition characterized by the presence of gas within the wall of the gastrointestinal tract. PI is a physical or radiographic finding, associated with a wide spectrum of diseases, ranging from life-threatening to innocuous conditions. For this reason, the PI management can vary from urgent surgical procedure to nonoperative management. In only 15% of the cases, PI is defined idiopathically, since it is not related to a specific disease , 5, 6.We report the case of a 46-year-old women hospitalized at the Department of Oncology with a diagnosis of advanced neoplasia of unknown origin. The diagnostic workup by means of total body CT scans followed from a PET-CT had shown disseminated neoplasia in the lungs, right kidney, and adrenal gland and in the 8th hepatic segment. In addition, a prominent lymphadenopathy, compatible with metastasis, was described in the cervical area, the mediastinal space, and along the aortic caval space, without interruption. A cervical lymph node biopsy revealed a metastasis from a poorly differentiated carcinoma with some aspects of sarcoma probably of kidney origin. Awaiting a systemic chemotherapy, the patient was transferred in our surgical unit because of an acute abdominal pain with signs of peritonitis on physical examination. Nausea, vomiting, and constipation were associated. Inflammatory marks were increased: white blood cells 26380/ul, C-reactive protein 243 mg/dL, and erythrocyte sedimentation rate 50 mm/h. A new abdominal CT scanning showed pneumoperitoneum, intramural air in the colon, and gas collection in the mesentery . On thesPI is an uncommon condition which has recently arisen to clinical attention thanks to improved radiographic identification, with an incidence of 0.37% in patients submitted to abdominal CT scans . The act The conditions underlying secondary PI can be divided into six groups: traumatic and mechanical, inflammatory and autoimmune, infectious, pulmonary, drug induced, and others (as the necrotizing enterocolitis) . MultiplThis radiological finding can be caused by a number of conditions, and the extent of the PI does not correlate with the severity of the underlying disease but with its localization and the surgical treatment in many cases is not required, while it can be mandatory if the underlying cause of PI is perforation or bowel ischemia. Therefore the most effort should be made in identifying the underlying cause, searching for pneumoperitoneum or portal venous gas. The decision for surgical intervention should be secondary to a very accurate study of the patient's history beside the clinical presentation, since there are so many conditions which can cause this radiological finding but do not require any surgical treatment , 7.Khalil et al. proposed a very interesting management algorithm for pneumatosis in which the presence of critical CT findings, critical laboratory findings, conspicuous physical conditions, or examination all lead to the surgical exploration . OtherwiWe completely agree with this suggestion, even if in our case, possibly we might have not performed surgical treatment without any difference in the clinical outcome. The clinical conditions on admission and the clinical laboratory findings were so poor, that the radiological finding of gas simply confirmed a strong preoperative suspicious. Moreover, our preoperative CT detected, along with the presence of gas on the colon wall, the presence of pneumoperitoneum, which in absence of previous trauma or iatrogenic endoscopic trauma makes the surgical approach absolutely mandatory.The presence of unknown neoplasia, even considering lung metastasis, made it more difficult to hypothesize the underlying etiology of pneumoperitoneum.In this case autopsy revealed a very strange origin of pneumatosis highlighting the complex wide spectrum of presentation of PI. We believe that our study confirms as mandatory steps in the management of such cases the most meticulous radiological evaluation through CT scanning together with careful evaluation of the medical history and the clinical presentation of the patient.


Bezoars are concretions of undigested material in the gastrointestinal tract, most commonly in the stomach. Duodenojejunal localization of bezoars is exceptional. We report a case of a 27-year-old woman who experienced nausea, vomiting and severe abdominal pain for one week. By palpation a mobile and sensitive mass, 15 × 15 cm, was detected, which filled the upper quadrant. Results of gastric endoscopy were normal. X-ray and ultrasonography suggested a bezoar. A laparotomy revealed that the jejunum was fissured by the trichobezoar ball. This trichobezoar mass was totally excised by intestinal resection.To our knowledge, this is the first reported case of duodenojejunal fissuration caused by trichobezoar in an adult. Among patients with high subocclusif syndrome, duodenojejunal bezoar should remain a possibility in differential diagnosis. Bezoars are collections of indigestible material in the gastrointestinal tract. They are of many forms, and the most common forms are phytobezoars, which consist of plant and fruit material; and trichobezoar, which occur from ingestion of hair.Bezoars have been classified into four types: phytobezoars (caused by vegetables), trichobezoars (caused by hair), lactobezoars (caused by milk curds) and miscellaneous .Trichobezoars are less common than phytobezoars but are more frequently seen in young people and are prevalent in females. Exceptionally they may migrate into the small intestine through the pylorus and become a source of occlusion: this is called Rapunzel syndrome.2The trichobezoar, which is an uncommon condition, should be suspected whenever we confront young girls with psychiatric problems who complain about chronic nonspecific digestive problems. The clinical symptoms vary and consist of nonspecific abdominal pain, nausea, vomiting and abdominal mass. Sometimes the bezoar presents itself with gastrointestinal bleeding or occlusion. The endoscopy remains the examination of choice in the diagnosis of intragastric trichobezoar; but in many cases of Rapunzel syndrome, the endoscopic diagnosis is difficult. In these cases, ultrasonography and CT are very helpful for diagnosis. The treatment of trichobezoar is surgical. EndoscopHereby we report a case of duodenojejunal bezoar in a 27-year-old woman presenting with intestinal occlusion which was treated with bowel resection. We want to attract the attention of clinicians to the diagnostic difficulties in this regard.A 27-year-old woman was referred to Avicenne Hospital with a 7-day history of vague abdominal pain and sustained bilious vomiting. Clinical examination showed an oblong mobile and tender mass in the left flank, measuring 10 cm in its greater axis. There was also evidence of paleness of mucosa and skin. Biological examination showed hypochromic microcytic anemia , hypoproteinemia (30 g/L) and hypocholesterolemia (1.05 g/L); the rest of biological examination results were normal. The oesogastroduodenal endoscopy did not show any specific anomaly. On the other hand, abdominal x-ray revealed a soft tissue density overlying left upper quadrant. Ultrasonography revealed a heterogeneous image in the left upper quadrant and mild ascites . A diagnbezoar is derived from the Persian word panzehar; or the Arabic word badzehar, which means masses in the stomach of sacrificed animals used as antidote or antipoison.[The term tipoison. It is deTrichobezoars are composed of hair, fur or fibers. This pathology is observed almost exclusively in young girls (90%) aged less than 30 years, complaining of trichotillomania and trichophagia. Despite 1et al. in 1968.[Rapunzel syndrome was described for the first time by Vaughan  in 1968. It is a  in 1968. Like in  in 1968.5 SometimIn 85% of patients, clinical examination reveals a well-defined abdominal mass that is smooth, firm and mobile in the epigastric area. The bari4Small bowel bezoars are treated surgically. It is mandatory to explore the whole gastrointestinal tract in order to avoid synchronous bezoar and recurrence of intestinal obstruction due to retained bezoar.The jejunal trichobezoar excision must be done by enterotomy. In our case, resection of the fissured jejunum was necessary.8 VariousExtraction of mass by endoscopy often fails and may lead to severe complications such as pneumomediastinum due to esophageal fissure. Dissolution of trichobezoar by Papain syrup is ineffective and is not proposed except for phytobezoars. The fragmentation of the ineradicable mass by YAG laser described in 1986 constitu38Psychological care of trichotillomania and trichophagia is difficult. The patient, as in our report, usually has a refusal attitude, which complicates the diagnosis and management. In cases of associated psychopathological problems such as compulsion, hyperkinetic and depressive syndromes, resort to behavioral treatment becomes necessary.Rapunzel syndrome is an uncommon trichobezoar, with a tail extending into the small intestine. It has varied presentation and is seldom diagnosed preoperatively. Fissure in duodenojejunal area caused by trichobezoar in an adult patient is uncommon. Duodenojejunal bezoar should be in mind as a possibility in the differential diagnosis of partial intestinal obstruction syndromes.


Most underlying diseases for abdominal pain in children are not dangerous. However some require rapid diagnosis and treatment, such as acute ovarian torsion or appendicitis. Since reaching a diagnosis can be difficult, and delayed treatment of potentially dangerous diseases might have significant consequences, exploratory laparoscopy is a diagnostic and therapeutic option for patients who have unclear and potentially hazardous abdominal diseases. Here we describe a case where the anomaly could not be identified using a laparoscopy in an adolescent girl with acute abdomen.A 13-year old postmenarchal caucasian female presented with an acute abdomen. Emergency sonography could not exclude ovarian torsion. Accurate diagnosis and treatment were achieved only after an initial laparoscopy followed by a laparotomy and after a magnetic resonance imaging scan a further laparotomy. The underlying disease was hematometra of the right uterine horn in a uterus didelphys in conjunction with an imperforate right cervix.This report demonstrates that the usual approach for patients with acute abdominal pain may not be sufficient in emergency situations. Abdominal pain in children is one of the most common reasons for emergency evaluation. Underlying diseases often depend on the child’s age and are self limited minor conditions. However it is important to recognize serious diseases, which might require specific treatment, or invasive procedures such as acute ovarian torsion or appendicitis .Ruling out serious surgical conditions can be difficult, especially in infants. Moreover, delay in the treatment of potentially dangerous surgical abdominal diseases might have significant consequences. Therefore, exploratory laparoscopy is a diagnostic and therapeutic option for patients with unclear and potentially hazardous abdominal diseases . NonetheHere we describe a case where the anomaly could not be identified using a laparoscopy in an adolescent girl with acute abdomen.A 13-year old caucasian female presented in the pediatric emergency unit with acute right-sided lower abdominal pain.There were no signs of infectious, gastrointestinal or urinary problems. Menarche had started nine months previously with regular menstrual bleeding. However, the patient suffered from symptoms of primary dysmenorrhea. Her most recent menstruation had started three days before.On examination the patient presented with severe tenderness in the lower abdomen. External genital inspection showed an intact hymen and menstrual discharge. Blood and urine tests were normal. Transabdominal ultrasound with a low-filled bladder detected an 8cm × 6.5cm hypoechoic mass in the right iliac fossa without perfusion, interpreted as a uterine malformation, a hematocolpos or an ovarian torsion.Due to the risk of an underlying ovarian torsion, the patient was taken to theater for vaginoscopy under anesthesia and, in the absence of any pathologic findings, for exploratory laparoscopy and, if necessary, laparotomy. Vaginoscopy showed a normal vaginal vault and a single cervix. Ovarian torsion could not be excluded by laparoscopy due to right-sided peritubal adhesions. Following a Pfannenstiel laparotomy, a bicornuate uterus with a blood- filled right uterine horn was diagnosed. The ovaries and tubes were normal. The right uterine hematometra was transabdominally drained.Postoperative magnetic resonance imaging (MRI) was performed to clearly visualize the anatomic condition. It confirmed two separate uterocervical cavities without communication of the right cervix to the vagina Figure . Right-sThe continuity between the right uterus and the vagina was reconstructed one week later. The girl recovered fully and had normal menstruation without dysmenorrhea when we saw her at follow-up, six months later.This patient illustrates the challenges of diagnosis and treatment of uterine malformations. The presence of acute abdominal pain required us to intervene immediately.One reason why an internal genital malformation could not be diagnosed earlier were the anamnesis of menarchal onset with regular bleeding for nine months, as well as the physical examination showing severe tenderness in the lower abdomen together with a regular hymen.Since the patient presented with an acute abdomen, and ovarian torsion was a likely possibility following transabdominal ultrasound, we took the patient to theater for vaginoscopy, laparoscopy and finally laparotomy.Could more accurate diagnostic imaging have prevented the emergency intervention? We routinely use transabdominal ultrasound with, if possible, a fully distended urine bladder as first imaging choice for cases of unclear abdominal pain in girls . TransvaMRI of the pelvis is the imaging of choice after ultrasound examination if uterine malformation is a possibility . In our For suspected ovarian torsion immediate care is required and surgical detorsion within four hours after onset of symptoms might reduces tissue damage ,6. TakenHow can renal agenesis be interpreted in the present case? Uterus didelphys is caused by an incomplete fusion of the Mullerian duct system. In 36% of all cases, Mullerian anomalies are associated with other structural abnormalities; most of them being renal malformations because of the concomitant embryologic development .V0 C2a U2 A0 MR[The most widely-accepted classification of uterine malformations from the American Fertility Society, does not take into account the frequently associated vaginal, cervical or urological anomalies -10. The  U2 A0 MR.This report demonstrates the challenges and pitfalls in diagnosis and treatment of postmenarchal girls with acute abdominal pain, and shows that the usual approach to patients with acute abdominal pain may not be sufficient in emergency situations. Nonetheless diagnostic laparoscopy remains the standard emergency approach for patients with potentially hazardous acute abdominal pain. Judicious use of MRI in adolescent girls may help to obviate the need for transvaginal ultrasonography in circumstances where the diagnosis is difficult to establish.Written informed consent was obtained from the patient’s legal guardian for publication of this manuscript 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.All authors analyzed and interpreted the patient history, data, images and clinical findings. PK, MK, and UK wrote the manuscript. All authors read and approved the final manuscript.