Bladder exstrophy occurs in approximately 1 in 35,000 live births and is associated with an increased incidence of bladder cancer.The primary mucinous adenocarcinoma of the bladder is an extremely rare urologic entity, which is found in less than 2% of all urinary bladder tumours and is often presented as metastatic. This is the first case in literature of a primary mucinous adenocarcinoma of an unreconstructed exstrophic bladder.A 55-year old male patient was diagnosed with a primary mucinous adenocarcinoma of an unreconstructed exstrophic bladder. Examination of the entire gastrointestinal tract shown there were not other primary cites. Immunohistochemistry confirmed the nature of the tumour. The patient underwent a radical cystoprostatectomy with en block bilateral pelvic lymphadenectomy, urinary diversion with a cutaneous ureterostomy and epidpadias repair.Adult bladder exstrophy and epispadia correction is a very rare practice in urology due the fact that this congenital disease is diagnosed and corrected in neonates. We advocate the radical surgical management, after exclusion of any primary malignant sites related to the gastrointestinal tract. Bladder exstrophy is a complex congenital abnormality and its management is normally done in neonates.Reports on exstrophy patients presenting in adulthood are very rare. Bladder exstrophy occurs in approximately 1 in 35,000 live births and is associated with an increased incidence of bladder cancer. Urinary bladder cancer is the second most frequent tumour of the genitourinary tract .Few series have been reported in literature, but most contain small numbers of cases.One of the most common forms of adenocarcinoma of the bladder is the metastatic adenocarcinoma. The primary sites for these tumours include the rectum, stomach, endometrium, breast, prostate, and ovaries.The reported incidence among exstrophy cohorts varies from 3.3% to 7.5%, which is several times greater than in age-matched controls in the general population .In contrast, treatmentt of bladder exstrophy in patients presenting in adulthood is rarely reported in the literature , 6], , , 9]. De. De9]. DThe etiology of this increased incidence remains unclear. The environmental stimuli and chronic infection may cause bladder cells to undergo glandular metaplasia . After eThere are a variety of histological types among adenocarcinomas, including papillary, glandular, adenoid cystic, clear cell, mucinous and signet-ring cell carcinomas. The last of these types is characterized by the presence of intracellular vacuoles filled with mucin that displace the hyperchromatic nuclei . This paA 55-year-old man with classic bladder exstrophy who had never undergone a reconstruction earlier presented with a lesion of all his bladder [Figure 1]. The patient was otherwise healthy. His renal function tests were normal. The intravenous pyelogram showed bilateral normal excretory kidneys with no cystogram phase. The characteristic skeletal defects of exstrophy complex such as widening of the pubic symphysis caused by malrotation of the innominate bones in relation to the sagittal plane of the body along both sacroiliac joints were present. The metastatic work up was negative as seen on the CT scan. No preoperative bladder biopsy were performed.We advocate the radical surgical management, after exclusion of any primary malignant sites related to the gastrointestinal tract.The patient underwent radical cystectomy, closure of the infra-umbelical defect with Cardiff repair with On-lay mesh repair  and episA wide local lymphadenectomy was performed [Figure 5].Abdominal wall closure was done with a fasciocutaneous M-plasty [Figure 6 ].Osteotomy was not done.The orientation of the tumor on final clinical and pathologic examination in our patient showed that the bladder of 11x8 cm was completly occupied by a nodular, grey lesion of a maximum diameter of 10 cm producing mucin [Figure 2], thus strongly suggesting the bladder as the primary source.eactive lymphoid hyperplasia. The skin margins were not involved. The patient was not given any adjuvant therapy and is doing well at six months of follow-up.Histopathological examination revealed a G3 poorly differentiated (High grade) mucin-producing adenocarcinoma, with intracellular and extracellular production of mucin. The lymph nodes examinated were negative showing a rOther primary sites for the tumor had been excluded and, in the absence of digestive tract tumor and for confirmation that it was a primary bladder tumor, an immunohistochemistry study was performed.Regarding immunohistochemistry, adenocarcinoma of the urinary bladder expresses CEA, CDX-2, MUC-1, MUC-2 and MUC-3, same as colonic adenocarcinoma. Cytokeratins 7 and 20 were positive, in contrast with colonic adenocarcinoma that expresses cytokeratin 20 but not cytokeratin 7 .Bladder exstrophy and epispadia is a very rare congenital disease in urology.Because the vast majority of patients with bladder exstrophy now undergo reconstruction at a young age, unreconstructed bladder exstrophy in an adult patient is exceedingly rare.We advocate the radical surgical management, after exclusion of any primary malignant sites related to the gastrointestinal tract.With proper surgical management of late presenting bladder exstrophy patients, an acceptable functional and cosmetic outcome can be achieved.The authors declare that they have no competing interests.GdL: conception and design, interpetration of data, given final approval of the version to be published. AR: acquisition of data, drafting the manuscript, given final approval of the version to be published. LR: acquisition of data, drafting the manuscript, given final approval of the version to be published. SM: acquisition of data, drafting the manuscript, given final approval of the version to be published. UP: critical revision, interpretation of data, given final approval of the version to be published. FI: critical revision, interpretation of data, given final approval of the version to be published. EI: critical revision, interpretation of data, given final approval of the version to be published. DP: critical revision, interpretation of data, given final approval of the version to be published. GR: critical revision, interpretation of data, given final approval of the version to to published. BA :critical revision, interpretation of data, given final approval of the version to be published.GdL : Consultant in Urology at Hospital Santa Maria delle grazie, Pozzuoli, Naples, Italy. AR : Resident in Urology at Federico II University, Naples, Italy. LR : Resident in Urology at Hospital Santa Maria delle grazie, Pozzuoli, Naples, Italy. SM : Resident in Urology at Hospital Santa Maria delle grazie, Pozzuoli, Naples, Italy. UP : Resident at Hospital Santa Maria delle grazie, Pozzuoli, Naples, Italy. FI : Associate Professor of Urology at Federico II University, Naples, Italy. GR : Resident in Urology at Federico II University, Naples, Italy. EI : Resident in Urology at Federico II University, Naples, Italy. DP : Associate Professor of Urology at Federico II University, Naples, Italy. BA : Associate Professor of Surgery at Federico II University, Naples, Italy.


Filariasis is an endemic infection seen in the tropical and subtropical regions of the world, presenting with lymphatic dysfunction in the form of lymphocele, hydrocele, chyluria, or groin lymphadenovarix. We report a rare presentation of filariasis as an intra-abdominal cystic mass. Wuchereria bancrofti, Brugia malayi, or Brugia timori. The threadlike worm resides in the lymphatic channels or lymph nodes. The clinical manifestations are directly related to the occlusion of the lymphatic channels, thereby causing lymphangiectasia.[Filarial infections are common in most tropical and subtropical regions of the world. Lymphatic filariasis is caused by iectasia. The mostiectasia. We reporA 42-year-old male patient was referred for radiological evaluation of a gradually enlarging lump, extending from the right hypochondrium to the infraumbilical region for the last 6 months. He also had occasional low-grade fever.Wuchereria bancrofti on microscopic examination [Ultrasonography (USG) revealed a cystic lesion extending from the subhepatic region to the pelvis. It showed internal septations, debris, and few small solid areas . The patmination . The patClinical examination alone is seldom helpful in the evaluation of an abdominal mass, apart from its ability to localize the lesion in some cases. Imaging in conjunction with clinical evaluation remains essential for more accurate localization, characterization, and for narrowing the differential diagnosis.USG and CT scan help to differentiate between cystic and solid masses. The presence of a cystic retroperitoneal mass may be due to many entities including benign lesions such as lymphocele, urinoma, mesothelial cyst, lymhangioma, traumatic cyst, and parasitic cyst as well as malignant lesions such as mucinous cystadenocarcinoma, pseudomyxoma retroperitonei, and parachordoma. In our patient, we considered the possibilities of lymphangioma, traumatic cyst, or mesothelial cyst.Despite extensive imaging evaluation, the diagnosis very often is only obtained on some form of histopathology. In our case as well, though the presence of an associated scrotal swelling suggested the possibility of a cyst of filarial origin, the diagnosis could be confirmed only after an FNAC.Filarial retroperitoneal cysts have a reported incidence of 1 in 105 000 hospitalized patients.4 There a


Multiple intracranial meningiomas are a condition where there is more than one meningioma in several intracranial locations in the same patient without signs of neurofibromatosis. Incidence varies from 1 to 10%. The prognosis of multiple intracranial meningioma does not differ from benign solitary meningiomas despite the multiplicity. However, the simultaneous occurrence of different grades of malignancy is observed in one-third of multiple meningiomas. Surgery remains the best option for treatment of symptomatic lesions. Our case review aims to present and discuss a 75-year-old female patient diagnosed with multiple intracranial meningiomas, describing their clinical, radiological, histological characteristics. It also highlights the fact that the patient had two tumours, underwent surgery, and so far has a good quality of life. Cushing and Eisenhardt  in 1938 A 75-year-old female patient presented with a six-month history of global headaches, loss of balance, and a change in facial sensation. She had no stigmata of neurofibromatosis. Central nervous system examination revealed reduced sensation on the left side, a GCS of 15/15, and all other cranial nerves were otherwise normal. Contrast enhanced CT scans  revealedMeningiomas are tumors originating from arachnoidal cells, granulations, stroma of the perivascular spaces, and in the choroid plexus, corresponding to 13 to 20% of all intracranial tumors . This isKyoi et al.  encounteOne of the relevant etiological factors  that is The location of the tumours can be varied. There appears to be a tendency towards unilateral hemispheric localization, and the most common locations were the supratentorial convexity and the parasagittal falx, but multiple meningiomas occurring in the posterior fossa are very rare . In our Histologically, multiple meningiomas do not differ from the solitary types ; howeverThe treatment and prognosis of multiple meningiomas do not differ from those of solitary benign tumours. Surgery is the treatment of choice for multiple meningiomas and depends on the following characteristics: symptomatic meningioma, asymptomatic meningioma greater than 3 cm in size and surgically accessible, and symptomatic expanding tumor , 15. EacSince it is well documented that multiple intracranial meningiomas are histologically benign, the prognosis is good and may be the same as for solitary meningiomas. Some studies have shown no recurrence at follow-up evaluation , 7, 13. 3 per year and the tumour doubling time ranges from 1.19 to 6.81 years. This growth varies with age, is minimal, and may thus be observed without surgical intervention unless specific symptoms develop.Nakamura et al.  further  Additionally, an asymptomatic small meningioma should be followed up with MRI every 6 or 12 months if the patient is more than 65 years old. Surgery should be prescribed mainly for an expansive symptomatic tumour with cerebral edema. Our patient had two tumours of which only one was removed surgically, whereas the other was left to be followed up as it was small and asymptomatic.Multiple meningiomas are rare. When they do occur they are more common in females. Their location can be varied, most commonly unihemispheric rarely in the posterior fossa. The histological subtypes are similar to the solitary types with the most common ones being psammomatous, fibroblastic, meningothelial, and transitional types. Definitive treatment is surgical removal of expansive tumours with cerebral edema and subsequent followup of small asymptomatic tumours.


Lipomas are rare, subserosal, usually solitary, pedunculated small lesions appearing mainly in the large intestine with a minimal malignancy potential. They usually run asymptomatic and become symptomatic when they become enlarged or complicated causing intestinal obstruction, perforation, intusucception or massive bleeding. In rare cases they can be self-detached and expulsed via the rectum as fleshy masses. This event mainly occurs in large, pendunculated lipomas which detach from their pedicle. The reason for this event remains in most of cases unclear although in some cases a predisposing factor does exist. Abdominal pain and obstructive ileus may be observed while in many cases bleeding occurs. The expulsed mass sets the diagnosis and in most of the cases all symptoms subside. Diagnosis is rarely established before surgery with the use of barium enema, computed tomography and colonoscopy which additionally provides measures of treatment and diagnosis. In atypical cases though, in cases where the malignancy can not be excluded or in complicated cases, surgery is recommended. Usually the resection of the affected intestinal part is adequate. If during surgery a lipoma is encountered simple lipomatectomy seems also to be adequate. Intestinal lipomas were firstly described by Bauer in 1757 . They coThey are non-epithelial, mostly solitary, sessile or pedunculated lesions originating from mature lipocyte cells . They caTheir site of appearance starts from the hypopharynx till the rectum, with the ascending colon and the distal ileum alongside with the ileo-cecal valve being the most common sites of appearance ,6,14-16.th decade of age being favored [Lipomas present mainly on the right side of the abdomen with females in their 5 favored ,18. In m favored .Lipomas are long standing and usually run asymptomatic and unnoticed whatsoever for many years . They bePatients complain of symptoms which are usually vague; the most frequent symptom reported is a non-specific abdominal pain with crabby, colic or intermittent character without rebound tenderness. This pain is usually repeated before the patient asks for medical assistance ,3,4,6,7.There is also lack of signs and findings during clinical examination -6. It isHowever, in most of the cases the lipomas are complicated and therefore the presenting symptoms and clinical signs appear according to the presenting manifestation, with hemorrhage being the most common symptom encountered .The size of the lipoma plays key role in bleeding appearance possibility with lesions greater than 4 cm in diameter being presented with bleeding in 10% of cases . BleedinSymptoms and signs of ileal obstruction are also quite often seen. This is the case in masses evolving in the terminal ileus or in "giant" masses that cause lumen obstruction and ileus -6,24. MoIn rare cases the lipoma may be detached from its base and expulsed from the rectum.This rare manifestation was firstly described in 1940 by Backenstoe with 19 cases being reported in the literature since 1942 .Spontaneous expulsion of a lipoma is described only in few cases in literature ,18,25-30Spontaneous expulsion is observed in cases of huge lipomas which are mainly pedunculated with a narrow pedicle . For an The authors have also encountered one such case in a 77-year-old female who was presented with acute abdomen and melena Figure  and who This course of symptoms progression is more or less identical in most cases of spontaneous lipoma expulsion. The main symptom in most of the cases is abdominal pain usually left sided and colicky in character, followed by rectal bleeding ,24,27-30Another possible presentation is obstructive ileus because the detached lipoma obstructs the ileo-ceacal junction and hinders stool passage . In our Eventually, in almost all cases a fleshy mass is passed from the rectum and sets the diagnosis ,27-30 asIn almost all cases the diagnosis was accidental either after examination of the expulsed mass ,27,28,30Diagnosis of intestinal lipoma, if not accidental, is usually established during surgery for possible intestinal cancer or for treatment of lipoma complications ,26.In barium enema, an ovoid, well delineated, smooth and radiolucent mass is usually observed. The size and the shape of the mass may be changed with bowel movements with the elongation of the mass being the foremost appearance ("squeeze sign") . In mostComputed tomography will also show a spherical, ovoid, pear shaped mass with sharp margins with density of -40 to -120 Housfield units in uncomplicated cases ,25. In cIn colonoscopy, a normal lipoma may be visualized and therefore establish the diagnosis . In moreMRI may provide additionally information but is not yet considered as a potential diagnosis indicator ,25,26.Despite all imaging modalities preoperative diagnosis is established in 62% of patients .In histopathology, mature and adult fat cells with lipoblasts surrounded by a fibrous capsule are usually observed . "PseudoThe treatment modality is usually chosen according to the size of the mass, the complications or the suspicion of malignancy.If the lipoma is less than 2 cm in diameter, it can be endoscopically removed, as stated before. For larger lesions more factors may play role apart from the size in choosing the correct modality such as the presence of a stalk (pedunculated lesions are easier removed than sessile lesions), the suspicion of malignancy or the manifestation of symptoms such as hemorrhage or obstruction ,7,25,26.Surgery includes removal of the colon which is affected or more radical procedures such as hemicolectomy ,33-36. HLaparoscopic excision has been proposed to provide less postoperative pain, shorter duration of ileus and quicker recovery. Laparoscopic assisted minimally invasive techniques are also been reported in the treatment of lipomas ,34,35.Recurrence has not been so far documented .Intestinal lipomas are rarely appearing with their diagnosis being established postoperatively despite the imaging modalities available today. Although for small pendunculated lesions endoscopic removal seems adequate in most cases surgery is required to achieve excision, ensure diagnosis or to control manifestations such as obstruction or bleeding. Pedunculated lipomas may rarely detach from their base spontaneously and expulsed via the rectum, an event which although rare should not lead to cessation of further investigations.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.IB, VKK, GK and ME treated and operated the patient. IB and VKK wrote the case report and the review. IM obtained the pictures. ME and DZ edited the paper. All authors have read and approved the final manuscript.


We describe an ovarian borderline tumour that presented as an acute deterioration in an incisional hernia secondary to intraperitoneal mucin accumulation. The differential diagnosis associated with hernial sac contents and options for opportunistic diagnosis are discussed. This case raises awareness of potential serious diagnoses that may be overlooked during emergent hernia repair. Several eponymous hernias describe the variety of organs and their pathologies that can manifest in abdominal wall hernial sacs.A 61-year-old woman, with a 25-year history of a longstanding, lower midline, incisional hernia at the site of a previous appendicectomy, attended the emergency department complaining of a gelatinous discharge through a new skin defect overlying the hernia. The patient had no other significant signs or symptoms and, specifically, no clinical or radiological evidence of bowel obstruction or strangulation. On examination there was a 10cm spherical hernia in the left lower quadrant . Digitalen bloc left oophorectomy. Histology was consistent with a borderline ovarian tumour of low malignant potential. Adjuvant chemotherapy was not recommended at a multidisciplinary team meeting. The patient made a good recovery and has been referred to plastic surgery for definitive management of the hernia.Computed tomography (CT) revealed a 23cm multiloculated mass arising from the left adnexa . Serum CA variety of organs and pathologies that may be encountered at hernia repair have been described including eponymous hernias such as Amyand’s (appendix) and Littre’s (Meckel’s diverticulum).This case illustrates how the presence of unexpected fluid in a hernial sac led to the diagnosis of an ovarian neoplasm. Ascites is a not uncommon finding that, while often indicative of strangulation,Disruption of a hernia has been described previously in massive ascites.This case demonstrates an unusual presentation of an ovarian neoplasm that could easily be overlooked. We urge vigilance and awareness when tackling acute hernias that may conceal more sinister diagnoses.


Diffuse large B-cell lymphoma (DLBCL) is the most frequent type of non-Hodgkin´s lymphoma found in oral and maxillofacial regions. A large number of cases may be biologically heterogeneous, which are commonly defined as DLBCL, not otherwise specified (NOS) by the World Health Organization (WHO-2008). The present case reports on an ulcer of raised and irregular edges, found on the border between the hard and soft palate, as the first and only manifestation of an extranodal non-Hodgkin lymphoma in an 85-year-old patient. Incisional biopsy was carried out, and the specimen revealed a proliferation of large lymphoid cells suggestive of diffuse large cell lymphoma. An immunohistochemical analysis was performed. EBV-RNA was assessed by in situ hybridization that also proved to be negative. Immunohistochemical and EBV analyses are important to avoid delays and inappropriate treatment strategies. Although advanced age is considered an adverse prognostic factor, early diagnosis did prove to be a key contributory factor in the cure of non-Hodgkin lymphoma. Key words:Diffuse large B-cell lymphoma, elderly, EBV. Diffuse large B-cell lymphoma (DLBCL) is the most frequent type of non-Hodgkin´s lymphoma (NHL) found in the maxillofacial region -4. DLBLCDLBCL is a proliferation of large B lymphoid cells with nuclear sizes equal to or exceeding the macrophage nuclei or more than twice the size of a lymphocyte . AuthorsAn 85-year-old woman was referred to the Oral Medicine Clinic of the School of Dentistry at Universidade Federal de Minas Gerais (UFMG) for evaluation of a lesion on the palate, with a two-weekevolution and complaints of pain. Intraoral examination identified an ulcer of raised and irregular edges, measuring 30 mm at its largest diameter, covered by a yellowish-white membrane, located on the border of the hard and soft palate on the left side Fig. . The patIn the present case report, the patient presented an ulcer of raised and irregular edges, on the border of the hard and soft palate, with a diagnosis of DLBCL-NOS. After the physician’s assessment, the mouth was considered the primary site of the disease, with no other lesions appearing in other regions. The patient, at the time of the biopsy, was 85 years of age. Although DLBCL can occur in a broad age range, the mean age most commonly occurs in the 7th and 8th decade of life ,3,6. TheThe lymphomas are neoplasms that are classified in two groups: in Hodgkin’s lymphomas, by the presence of the Reed–Sternberg cells, and in non-Hodgkin’s lymphomas, by all other lymphoid neoplasms. For the NHL group, up to 40% are observed in an extranodal site, and 2-3% of these extranodal cases may arise primarily in the oral cavity and jaws. Studies of oral NHL demonstrated a major predominance of the DLBCL subtype, which accounts for 50% to 68% of all oral NHL .In 2008, the International Agency for Research on Cancer (IARC) published the 4th edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues . New subImmunohistochemical markers have been reported to be associated with prognostic effects for DLBCL . In the In contrast, Chung et al.  suggest Germinal-center B-cell (GCB) markers, such as CD-10 and Bcl-6, and GCB activation markers, such as MUM-1 by immunohistochemistry can be correlated with the prognosis of patients with DLBCL. This study explained that the discrepancies regarding the prognostic significance of CD-10 or Bcl-6 expression in DLBCL reported in prior literature is related to the evaluation of these two markers (CD-10 and Bcl-6) individually without the simultaneous evaluation of activation markers, such as MUM-1. Bhattacharyya et al. , in a stIn addition to the prognostic factors, more unfavorable results could be observed in older patients, including a poor hematologic tolerance to chemotherapy, a higher risk for treatment-related toxicity due to altered drug metabolism, and a worse immune system. These factors lead to a less effective NHL therapy ,23. OldeIn the current case, although the patient was of an advanced age with the presence of hypertensive disease and diastolic heart dysfunction, he did undergo an excellent follow-up treatment. Although doses of R-CHOP had been adjusted for the age and heart condition of the patient, the early diagnosis did prove to be a key contributory factor to this outcome. The extent of tumors in the early-stages of DLBCL may well contribute to the choice of therapeutic strategy , which c


A 55-year-old man presented with a large tumor in his lateral ventricles. Magnetic resonance imaging revealed disseminated lesions in the third and fourth ventricles at the time of diagnosis. The patient underwent a partial removal of the tumor in the lateral ventricles. Histologically, the surgical specimens showed glioneuronal differentiation with ganglion or ganglioid cells, Rosenthal fibers, oligodendroglia-like honeycomb appearances, a spongy pattern, perivascular pseudorosettes, and many hyalinized blood vessels. Papillary structure was not observed. The neuronal component showed a moderately high labeling index of Ki-67/MIB-1. We diagnosed this tumor as atypical intraventricular glioneuronal tumor. The disseminated lesions disappeared after chemoradiation therapy with temozolomide, and the residual tumors in the lateral ventricles remained stable for 3 years after the surgery. We discuss the pathological diagnosis, therapy and clinical course with review of the literatures. Many types of brain tumors originate in the ventricles. For tumors with neuronal differentiation that occupy the anterior central part of the lateral ventricles in adults, central neurocytoma is typically the diagnosis . However11C-methionine and a moderate uptake of 18F-fluorodeoxyglucose. A carotid angiography showed mild tumor staining. These findings led us to the primary diagnosis of central neurocytoma. The patient underwent a subtotal removal of the tumor by a frontal interhemispheric transcallosal approach. Intraoperatively, the tumor appeared ashy in color. It was soft superficially and elastic and hard internally. The tumor was adhered strongly to the septum pellucidum and moderately to the walls of the anterior horns and the bodies of the lateral ventricles. The choroidal arteries fed the tumor. Incidentally, the body of the right fornix was injured in order to remove the tumor extending into the third ventricle. Postoperative MRI showed that the tumor remained in the tip of the left anterior horn and the fourth ventricle. After the operation, the patient's memory disturbance worsened, and his postoperative HDS-R scale was 10/30.A 55-year-old man had a 6-month history of memory loss and stagger. He was admitted to our hospital with the diagnosis of an intraventricular tumor. Upon admission, his consciousness level was clear without focal neurological deficits. His Hasegawa's dementia scale (HDR) score had deteriorated to 12/30 points due to his recent memory disturbance. He did not show any symptoms of increased intracranial pressure. His hematological tests and physiological function tests were in the normal range. A computed tomography (CT) scan showed a large mass lesion of about 6 cm in diameter in the central part of the lateral ventricles. No calcification was shown in the lesion. Magnetic resonance imaging (MRI) revealed that the lesions had spread to the third and fourth ventricles Figure . MRI didFormalin-fixed, paraffin-embedded tissue sections were examined with hematoxylin-eosin (HE) staining and immunohistochemistry. The primary antibodies and their dilution with buffer were as follows: rabbit polyclonal anti-olig2 antibody , polyclonal anti-vimentin antibody , mouse monoclonal anti-glial fibrillary acidic protein (GFAP) antibody , mouse monoclonal anti-synaptophysin (Syn) antibody , mouse monoclonal anti-tubulin, βIII isoform (TuJ1) antibody , monoclonal anti-neuronal nuclear antigen (Neu-N) antibody , monoclonal anti-microtubule associated protein-2 (MAP-2) antibody , monoclonal anti-neuron-specific enolase (NSE) and monoclonal anti-Ki-67/MIB-1 antibody . Antigen retrieval using an autoclave  was performed for all antibodies. An Envision kit (Dako) was used as a source of secondary antibodies conjugated to dextran polymer and hydrogen peroxidase, and 3,3-diaminobenzidine was used as the chromogen.HE slides exhibited a variety of glioneuronal tumors. Some ganglions or ganglioid cells were observed with a background of neuropil Figure . Spongy The results of the immunohistochemistry are shown in Table 2) was orally administered as a concomitant and adjuvant therapy following radiation therapy. Twelve months after the surgery, MRI with Gd revealed that the disseminated lesions in the lateral ventricles and fourth ventricle had disappeared. A small residual nodule remained in the tip of the left anterior horn. MRI with Gd showed that the residuum had remained stable for 30 months after the surgery. His memory disturbance gradually subsided, and his HDS-R scale improved to 25/30.For the residual tumor, the patient underwent intensity-modulated radiation therapy (IMRT) using helical tomotherapy, which consisted of 30 fractions and a median dose of 30.6 Gy. Temozolomide . However, the tumor of this case had less papillary structures and less ganglion cells than PGNTs, in which tumor cells are typically arranged around vessels, forming pseudopapillary structures .Spongy patterns, Rosenthal fibers, hyalinized vessels, and honeycomb-like components have often been associated with PA . HoweverAlong with PGNTs, rosette-forming glioneuronal tumors of the fourth ventricle (RGNT) were recently listed in the fourth edition of the World Health Organization Classification of Tumours of the Central Nervous System ,5. It isThe moderately high Ki-67 labeling index (LI) was another clue for the diagnosis of this tumor. The Ki-67 LI of ordinary glioneuronal tumors or central neurocytoma is less than 3%. The differential diagnoses were atypical central neurocytoma and PA with atypical features. Considering these histological variabilities of neuronal and glial component and the atypical histological findings, we finally diagnosed this case as an intraventricular tumor with atypical glioneuronal differentiation. According to Ishizawa et al. .To the best of our knowledge, 11 cases of intracranial glioneuronal tumors (1 cases of PGNTs , 9 of maVarlet et al.  reportedFollowing surgery, radiation therapy with concomitant administration of TMZ has recently become the standard therapy for malignant gliomas. As shown by the cases of Javahery et al.  and the This is the rare case of glioneuronal tumor to present intraventricular dissemination at diagnosis, which have a possibility of transitional form between glial and neuronal components. The case was successfully treated wih a possible reductive surgery followed by IMRT with administarion of TMZ.HDR: Hasegawa's dementia scale; CT: computed tomography; MRI: magnetic resonance imaging; WI: weighted images; FLAIR: fluid-attenuated inversion recovery Gd: gadolinium; HE: hematoxylin-eosin; GFAP: glial fibrillary acidic protein; Syn: synaptophysin; TuJ1: tubulin, βIII isoform; Neu-N: neuronal nuclear antigen; MAP-2: microtubule associated protein-2; NSE: neuron-specific enolase; IMRT: intensity-modulated radiation therapy; PGNT: papillary glioneuronal tumors; PA: pilocytic astrocytoma; RGNT: Rosette-forming glioneuronal tumors of the fourth ventricle.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.HY was a major contributor in writing the manuscript. YH and NO participated in the pathological examination of the case. NN and TI participated in the surgery of the case. SY and JS participated in its design and coordination and helped to draft the manuscript. All authors have read and approved the final manuscript.1 Department of Neurosurgery, Gifu University Graduate School of Medicine, Gifu, Japan2 Pathology Division, Gifu University Hospital, Gifu, Japan3 Chubu Medical Center for Prolonged Traumatic Brain Dysfunction, Department of Neurosurgery, Kizawa Memorial Hospital, Minokamo, Japan


Background. Duodenal gastrointestinal stromal tumours (GISTs) are an uncommon malignancy of the gastrointestinal (GI) tract. We present a case of life-threatening haemorrhage caused by a large ulcerating duodenal GIST arising from the third part of the duodenum managed by a limited duodenal wedge resection. Case Presentation. A 61-year-old patient presented with acute life-threatening gastrointestinal bleeding. After oesophagogastroduodenoscopy failed to demonstrate the source of bleeding, a 5 cm ulcerating exophytic mass originating from the third part of the duodenum was identified at laparotomy. A successful limited wedge resection of the tumour mass was performed. Histopathology subsequently confirmed a duodenal GIST. The patient remained well at 12-month followup with no evidence of local recurrence or metastatic spread. Conclusion. Duodenal GISTs can present with life-threatening upper GI haemorrhage. In the context of acute haemorrhage, even relatively large duodenal GISTs can be treated by limited wedge resection. This is a preferable alternative to duodenopancreatectomy with lower morbidity and mortality but comparable oncological outcome. Gastrointestinal stromal tumours (GISTs) are generally considered to be an uncommon soft tissue malignancy of the gastrointestinal tract. They predominantly occur in patients over the age of 50. They constitute 0.1% to 3% of all gastrointestinal tumours with an incidence equivalent to 10–20 cases per million . Of all GISTs tend to present with vague nonspecific abdominal symptoms such as pain, a mass, or an occult gastrointestinal bleed. Occasionally patients will present with more severe symptoms such as obstruction, perforation, or severe acute bleeding as in the case described below . The ideA 61-year-old man presented to the emergency department with hypovolaemic shock following a sudden episode of upper gastrointestinal bleeding associated with melaena, haematemesis, and a temporary loss of consciousness. There was no prior history of gastrointestinal disturbance, and his past medical history included essential hypertension controlled by antihypertensives. On admission to accident and emergency, he was haemodynamically unstable with refractory hypotension (89/60 mmHg) and a sinus tachycardia of 110 bpm. Physical examination revealed melaena on rectal examination and a Glasgow coma score of 14/15 but was otherwise unremarkable. He remained haemodynamically unstable despite aggressive fluid resuscitation with Hartmann's and packed red blood cells. His Rockall score was 3 out of 7, scoring one for age and two for shock.Initial blood tests revealed a haemoglobin of 9.0 g/dL, and emergency oesophagogastroduodenoscopy (OGD) demonstrated fresh ongoing bleeding with no identifiable source. His haemoglobin continued to drop despite being transfused a total of five units of blood, and inotropic support was needed to maintain his blood pressure and so arrangements were made for emergency surgery.Emergency laparotomy identified a mass lesion on the anterior duodenal wall between the 3rd and 4th part of the duodenum  . ContinuIn total, he was transfused 12 units of packed red blood cells and 6 units of fresh frozen plasma with one unit of cryoprecipitate and pooled platelets and after surgery he was transferred to intensive therapy unit (ITU). He was extubated after one day and remained haemodynamically stable, not requiring any further transfusions. He was discharged to the ward after 3 days. Histopathological analysis of the resected specimen showed a well-demarcated small-bowel tumour measuring 50 × 29 × 28 mm. Clear margins were demonstrated over 2 mm from the stapled resection edge with no mucosal infiltration. Microscopically , it showStaging computerised tomography (CT) revealed no evidence of metastases and he was discharged from hospital after twelve days with 6 monthly followup.In 1983, Mazur and Clark first described gastrointestinal stromal tumours (GIST) as neoplasms neither showing immunohistochemical or ultrastructural characteristics of neuronal Schwann cells or smooth muscle cells . Histolocharacteristic expression of CD34 and CD117 (c-kit) proteins has been demonstrated [Macroscopically, these tumours are usually grey-white in appearance arising from the muscularis propria and grow either into the peritoneum  like in this case or into the lumen of the gut  . Hirota nstrated . Unlike carcinomas, GISTs do not widely infiltrate at the microscopic level and rarely metastasize to lymph nodes or liver , 7. BeniFletcher et al. proposed a risk stratification system for GISTs which classified them according to tumour size and mitotic count which is summarised in  Chiarugi et el. reviewed 156 duodenal GISTs in adult and paediatric patients finding that 86% of those with a tumour >5 cm with >5 mitoses per 50 HPF died of the disease, whereas no recurrence or metastases as seen in patients with tumour <2 cm with <5 mitoses per 50 HPF [Metastases outside the abdominal cavity, such as the lung, bone, or brain, are quite rare especially as an initial presentation . However The results of these studies would suggest that the patient in the case reported herein was at low risk for local recurrence and disease progression.As a result of the introduction of anti-KIT tyrosine kinase inhibitors in their treatment, these tumours have become better known. Imatinib mesylate (Gleevec) is one such inhibitor that is particularly effective against the KIT protein becoming second-line treatment or neoadjuvant treatment. Up to 50% of patients with advanced disease show a response to imatinib, and for large poorly positioned tumours that are difficult to resect or unresectable, neoadjuvant treatment is recommended , 11. There is ongoing controversy regarding optimal surgical treatment with some arguing that pancreaticoduodenectomy provides better oncological control for those located near the ampulla of Vater, and others support the selective use of a limited resection of the duodenum . It is kFor duodenal GIST, the limited wedge resection may be difficult to achieve due to proximity of adjacent structures, particularly when the tumour has extended through the serosa, and as these are uncommon locations, optimal surgical treatment is even less defined. Similar to our patient, the recent Chung et al. series support the use of wedge resections and primary closure as their most commonly performed operation . A case series of 15 patients by Goh et al. showed comparable oncological results in patients treated with limited resection versus those treated by pancreaticoduodenectomy thus suggesting that a more extensive resection is not indicated . Two series by Miettinen et al. reported 20% and 40%, respectively, of patients undergoing pancreatico-duodenectomy. These should be considered when localised to the second portion of the duodenum and involving complex anatomy like the ampulla .As discussed, surgical resection may be achieved through several means but we suggest that limited resections should be considered even for relatively large tumours—up to 5 cm as in the present case. Segmental resection and local wedge resection were performed in the Miettinen series in 45% and 20%, respectively, knowing that only 30% of duodenal GISTs show a malignant appearance [This case highlights the successful emergency surgical management of a rare tumour with an emphasis on bowel-preservation surgery. GISTs are a rare type of gastrointestinal tumours that are most commonly located in the small bowel or stomach but can also be found in the duodenum. They tend to present with vague non-specific abdominal symptoms but occasionally will present with severe acute bleeding. We present a case of a patient with a duodenal GIST that presented with a life-threatening upper-GI bleed requiring extensive intravascular volume replacement and emergency surgery.The most definitive treatment for GISTs is complete surgical resection which is normally achieved by segmental resection or wedge resection. Tumours of the periampullary region may require a more extensive surgical resection. The patient described herein was found to have a GIST of 5 cm in diameter on the anterior aspect of the duodenum at the junction of D3 and D4 that showed no evidence of local spread and was successfully treated with wedge resection preserving the ampulla of Vater. This is a preferable alternative to duodenopancreatectomy with lower morbidity and mortality but comparable oncological outcome.


We present a case of nonseminomatous germ cell tumor of the testes with acute urinary retention secondary to urethral metastasis. This presentation, and similar cases of urethral metastasis from this tumor, have not been reported previously.A 35-year-old Caucasian man presented to hospital with a history of acute urinary retention. On examination he was found to have right testicular enlargement with raised β-human chorionic gonadotrophin, serum α-fetoprotein and lactate dehydrogenase levels. He underwent radical left inguinal orchidectomy and histology confirmed a nonseminomatous germ cell tumor of the testes. Cystoscopy carried out due to urinary retention showed penile metastasis and the biopsy confirmed metastatic malignant undifferentiated teratoma. Staging computed tomography scan and magnetic resonance imaging of the pelvis showed pulmonary, pelvic nodal, ischial and penile metastasis. The diagnosis of the International Germ Cell Cancer Collaborative Group of poor prognosis metastatic nonseminomatous germ cell tumor was made, following which he received four cycles of bleomycin, etoposide and cisplatin chemotherapy with curative intent. He had a complete marker and an excellent radiological response. He is currently under follow up.The unusual presentation of lymphovascular spread in this case of nonseminomatous germ cell tumor highlights the need to include routine pelvic imaging in the assessment and follow up of testicular cancer. We report the case of a Caucasian man who presented with acute urinary retention and was found to have urethral metastasis from a nonseminomatous germ cell tumor. Urethral metastasis from a nonseminomatous germ cell tumor is very unusual, as it does not lie in the lymphatic drainage pathway of the testis.A 35-year-old Caucasian man presented to his local general hospital emergency department with a history of acute urinary retention. He had a background history of grade 1 astrocytoma in the posterior fossa at the age of six that was resected in 1979, and required re-excision in 1982 followed by radiotherapy resulting in residual hydrocephalus, epilepsy and mild learning difficulties. There was no history of testicular maldescent, orchidopexy or other inguino-scrotal surgery. On examination he was found to have no new neurological deficits, but right testicular enlargement was noted. An ultrasound study confirmed a 9×8 cm heterogeneous mass replacing the body of the right testis. His serum β-human chorionic gonadotrophin level was 5047 U/L , serum α-fetoprotein level was 7413 kU/L  and lactate dehydrogenase level was 1452 IU/L . His liver and renal biochemistry was normal.A urethral catheter was placed, and drained normal urine. He underwent a radical left inguinal orchidectomy five days later. Histology indicated a non-seminomatous germ cell tumor, with predominantly yolk sac tumor and small amounts of undifferentiated teratoma. The overall histological diagnosis was malignant teratoma intermediate (British Testicular Tumour Panel (BTTP) classification) or embryonal carcinoma  classification). Lymphovascular involvement was noted, with widespread blood vessel involvement seen Figure . The speA staging computed tomography (CT) scan revealed extensive disease within the thorax with multiple bilateral pulmonary metastases measuring up to 3 cm in both lungs Figure . In addiHistology of the urethral mass confirmed a metastatic malignant undifferentiated teratoma Figure . ImmunocPrimary urethral tumors are rare, accounting for less than 1% of urological cancers in the USA . AccordiThe spread of nonseminomatous germ cell tumors may be local, via the lymphatics or via vascular channels. Review of our patient's primary tumor revealed widespread blood vessel involvement. As the spermatic cord was not involved, it is unlikely that dissemination to the urethra represented local invasion. Seeding through urine or seminal fluid also seems unlikely. The involvement of ischium and inguinal lymph nodes is also unusual and may be attributed to lymphovascular dissemination.In the absence of previous inguino-scrotal surgery, the usual lymphatic drainage of the right testis is through the interaortocaval nodes. Secondary lymphatic spread may cross the midline, and may occur in a retrograde fashion to the common external and inguinal nodes involving the pelvic structures. As the distal urethra drains into superficial and deep inguinal lymph nodes, it could have been involved in a retrograde manner. Spread via hematogenous dissemination is also a likely possibility.We believe this rare presentation of testicular cancer relates to atypical lymphovascular dissemination within the pelvis, with no history of surgical interventions that would predispose our patient to this route of dissemination. While the presentation in this case is highly unusual, it confirms the need to include pelvic imaging in the initial investigation of patients with testicular cancer. Furthermore, patients who have demonstrated intrapelvic dissemination of disease need to have continued pelvic imaging at every reassessment. Clinicians should consider further dedicated investigation such as penile MRI in patients presenting with urinary tract obstruction and testicular germ cell tumor.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.VA, TW, JJ and DS were involved in conception, design, interpretation and writing of the manuscript. VA, JJ and DS were directly involved in looking after our patient. TW created and provided the radiological images. SC performed the histological examination and provided the histopathological images. All authors have been involved in critically revising the manuscript for intellectual content and they have read and approved the final version submitted.


Splenic rupture due to colonoscopy is a rarely reported event in the emergency medicine literature. Patients experiencing such an occurrence are likely to report to the emergency department. This paper documents an 84-year-old female who presented to the emergency department with abdominal pain and nausea less than 24 hours following a colonoscopy. An abdominal ultrasound revealed splenomegaly and free fluid. An abdominal computed tomography was significant for a splenic laceration. She underwent radiologic guided embolization and recovered without incident. Emergency medicine physicians need to consider splenic rupture as a differential in patients presenting after colonoscopy with abdominal pain. Colonoscopy is considered to be a safe procedure to visualize the large intestine for various reasons including, but not limited to, colon cancer screenings, lower gastrointestinal bleeding, and inflammatory bowel disease. The most common complications are bleeding and intestinal perforation with incidence rates of approximately 1-2% and 0.1-0.2%, respectively . Little Despite the rare occurrence of splenic rupture following colonoscopy, this is a potentially life-threatening complication that may be on the rise as more colonoscopies are performed. It is therefore important that emergency physicians be aware that splenic rupture is a complication of colonoscopy and be familiar with its typical clinical presentation. An 84-year-old woman presented to the emergency department (ED) with severe left upper quadrant pain, associated with nausea, which radiated into her back and left shoulder. She had a colonoscopy several hours prior to ED presentation. Her past medical history is significant for hypertension, heart disease, and hyperlipidemia. Her medications included clopidogrel, aspirin, olmesartan, atenolol, nifedipine, pravastatin, levothyroxine, and temazepam. She discontinued the clopidogrel for one week in anticipation of the colonoscopy procedure. This procedure was performed to evaluate recent symptoms of hematochezia. She had no history of prior abdominal surgery.On ED presentation, she was diaphoretic and appeared pale. She had a temperature of 97.2 F, pulse of 50 beats/min, respiratory rate of 18 inspirations/min, and a blood pressure of 82/40 mmHg. Upon examination, there was no abdominal distention. She had normal bowel sounds and moderate tenderness in the left upper quadrant, without guarding or rebound, and no palpable masses. Her laboratory tests revealed slightly decreased sodium (130 mmol/L) and chloride (96 mmol/L) levels in addition to an increased glucose level (243 mmol/L). Her hemoglobin and hematocrit levels were both low at 9.8 g/dL and 29.3%, respectively. A chest X-ray revealed biapical pleural thickening, atherosclerotic changes of the heart, and the presence of a coronary artery stent. An abdominal X-ray did not reveal any obstruction or free air. An ED bedside ultrasound of the abdomen showed splenomegaly and free fluid in the left upper quadrant and Morrison's pouch. Following this, a contrast computed tomography (CT) of the abdomen was performed and indicated the presence of a splenic laceration of grade 3 or 4, splenic hematoma involving both intra- and extracapsular components, and extensive abdominal free fluid.The patient initially was treated with intravenous (IV) fluids, a cardiac monitor, and oxygen. She was observed for approximately one hour. A repeat systolic blood pressure was 115 mmHg. She also received 1 unit of packed red blood cells. After reevaluation of her lab tests, vitals, and radiological results, the patient underwent successfully radiologic guided embolization of the splenic artery. She recovered uneventfully over the next several days and was discharged from the hospital without additional sequelae. A review of the literature appears to indicate that splenic rupture associated with colonoscopy is a rare complication, with few reports available in the emergency medicine literature. This may be due to the fact that most cases of splenic rupture following colonoscopy are recognized immediately during the procedure and bypass the emergency department. On the other hand, many cases, like ours, can present several hours after the completion of a colonoscopy and will likely present to an ED. Being aware of this diagnosis and being able to identify the symptoms of such a patient can lead to a faster, life-saving diagnosis. Most patients with a colonoscopy-induced splenic rupture present with the typical symptoms of a trauma-induced splenic rupture within 24 hours of their colonoscopy –14. Howe Contrast (CT) is the best tool for diagnosing a ruptured spleen after colonoscopy. Ultrasonography (US) is a useful test for diagnosing this condition, is rapid, and does not have radiation exposure and intravenous contrast risks. However, US is the best for identifying the presence of free fluid in the peritoneum and further imaging, such as a CT, is better for delineation of the extent of injury to the spleen , 14. In  Three common treatment options for a ruptured spleen due to colonoscopy are splenectomy, splenic artery embolization, and nonoperative management. The majority of previously reported cases were managed by splenectomy –12, 14.  An understanding of the cause behind colonoscopy-induced splenic ruptures would help to identify high-risk patients, but little information is available. Most agree that direct trauma to and/or increased traction on the spleen and/or the splenocolic ligament is a likely mechanism –8, 10–15Splenic injury or rupture during colonoscopy occurs rarely but will likely present to an emergency department when it occurs. Patients typically present with abdominal pain, hypotension, and anemia within 24 hours of the procedure. ED physicians need to be cognizant of this colonoscopy complication and its presentation.


Castleman's disease (CD) is a rare, localized or generalized, lymphoproliferative disorder with a frequent mediastinal location, but possible in any lymph node or extra nodal site. It usually appears in young adults whilst it rarely occurs in childhood. There are only about 100 pediatric cases published, five of them in Italy. We report 3 cases of localized Castleman's disease, investigated in our Department in a 3 years period and reviewed the literature. Castleman's disease (CD) is a rare and not well recognized disease characterized by a massive growth of lymphoid tissue of unknown etiology that was first described in 1954, and subsequently better defined by Castleman in 1956 . The disWe report three cases of localized hyaline-vascular CD in pediatric age observed in our Department in the last 3 years; a complete surgical resection was not feasible in one of them and so various therapeutic attempts were performed.GM, female, was admitted, at the age of 3.3 years, with an asymptomatic left axillary mass showing a slow progressive growth. At presentation she was in good general conditions and the physical examination was normal. Routine blood tests were all normal too. Radiological examinations  confirmed the adenopathy and excluded other locations. After 2 months a complete surgical resection was performed, and a single lymph node of 4 cm maximum diameter was removed. Histological assessments indicated the diagnosis of hyaline-vascular CD Figure . There hLG, male; at the age of 3.8 years an isolated palpable subcutaneous tumefaction, sized 1 cm in maximum diameter, appeared on his right blade shoulder. On admission he was in good physical condition; routine blood tests, abdominal ultrasound and chest X-ray were all normal. After a follow-up period of 3 months, during which the mass enlarged to a diameter of 2.5 cm, a complete surgical resection was performed and two subcutaneous and intramuscular nodules, having maximum diameter 2.5 and 1.2 cm respectively, were removed and examined. Histology revealed a classic picture of hyaline-vascular CD Figure . No recu 99 mTc-MDP total body bone scan were both normal. After two weeks, suspecting a mediastinal Hodgkin Lymphoma, an open biopsy of the mass was performed; during the surgical procedure massive bleeding occurred and the hemoglobin level dropped from 12.3 to 8.7 gr/dl. No other serious events occurred. The histology showed the typical aspect of a hyaline-vascular CD  scan showed that the neck mass, with high contrast-enhancement, originated in the anterior-superior mediastinum and caused a serious right dislocation of the trachea Figure . A fine D Figure . A seconAfter prolonged and troublesome discussion with surgeons, an attempt of surgical excision was planned through a left trap door incision. This approach consisted of a left-anterior cervical access, median sternotomy and left-anterior thoracotomy that could provide adequate exposure of the neurovascular structures . The masFinally, after troubling discussion with the parents, the boy underwent a 45 Gy course of 3D conformal radiation therapy on the upper mediastinal mass, with standard fractionation of 1.8 Gy per day. The treatment was delivered through two antero-posterior, postero-anterior asymmetrically weighted beam pairs of different energies in order to encompass the mass into the 95% isodose. Particular care was taken to avoid undue radiation delivery to the nearby organs: right and left lungs received an average dose of 8 and 6.4 Gy respectively, while the spinal cord maintained a maximum dose less than 45 Gy. A CT scan performed at two months from the end of radiotherapy showed a reduction of the mass by about 60% and a significant improvement of tracheal dislocation: both were more evident in a second CT scan performed 3 months later. The FDG-PET scan that initially revealed a mildly significant uptake of the radiopharmaceutical of the residual disease at three months from the end of radiotherapy, became totally negative after a further 3 months, confirming the effectiveness of our therapy choice. A further reduction of the residual mass sizes and negative total body scan was obtained six months later with neck-chest CT scan and FDG-PET scan respectively. Now the boy is well, on a watchful follow-up, without any side effect of radiotherapy, 12 months after discontinuation of treatment.Histologically all of our cases, diagnosed as hyaline-vascular type of Castleman's disease, showed a mass of lymphoid tissue with scattered abnormal lymphoid follicles, which ranged in size from small to large. The germinal centers were depleted and composed predominantly of follicular dendritic cells and endothelial cells of hyalinized capillaries. Their appearance corresponds to that of "hyaline vascular nodules". Another peculiar finding was the presence of more than one small germinal center within a single follicle. There was a concentric layering of lymphocytes at the periphery of the follicles with an onion-skin appearance.The interfollicular areas showed vascular proliferation with numerous hyperplastic vessels of the postcapillary venule type with hyaline material between vessels and absence of sinuses. A small number of lymphocytes, plasmacells, eosinophils and immunoblasts were present.Immunohistochemically, positive CD21 and CD35 were seen in the large cells with vesicular nuclei (follicular dendritic cells) in the center of follicles, strong positive FVIII-related antigen was seen in the endothelium of the interfollicular vessels, but only weak and focal reaction was found in the hyaline vessels located in the center of the follicles.The immunoglobulin production by plasmacells was polytypic and in the interfollicular areas large numbers of suppressor T cells were found.In summary the diagnostic features were small hyaline germinal centers within an expanded mantle zone, as well as a highly vascularized interfollicular network.CD is a disease of unknown etiology, first described by Castleman in 1956  and currPrevalence is estimated to be less than 1/100,000 . There aIt is postulated that the disease represents a reaction to chronic viral antigenic stimulation with the bulk of evidence pointing toward an interleukin-6 role, above all in the form of the disease associated with systemic manifestations -17. The Finally, there are many reports of association of the multicentric type with HIV infection with a global incidence of 4.3/10,000 patient-years ,22.The majority of HV-CD at presentation is asymptomatic but, in some cases, fever, night sweat or weight loss can be present; in contrast, if the localized disease has a plasma cell histology, constitutional symptoms are very frequent, as in the disseminated form ,8. The sThe plasma cells and/or the multicentric CD present with a variety of constitutional symptoms: peripheral lymphadenopathy, hepatosplenomegaly, weight loss, anemia, asthenia, night sweats, fever, skin rash, lung disorder and kidney dysfunction ,25,27.Less common presentations are polyneuropathy, pleural effusion, ascites etc. ,28. TypiEnhancement on CT images after intravenous administration is universally reported but it seems that the enhancement is to a lesser degree in the plasma cell variant. At MRI the T1 weighted images are isointense or slightly hyperintense relative to the skeletal muscle; the hyperintensity is much more evident on T2 weighted images .About the therapy issue, it is difficult to draw definitive conclusions for both local and disseminated CD, but some broad indications can be found.Since the recurrence of localized CD is very unusual when the resection is complete, surgery is the preferred treatment in these cases -8,29-31.Even though cases of spontaneous remission have been described , the couIn some more recent series, however, the prognosis seems to have been better . The treOur experience also emphasizes the potential hazards of surgical resection depending on the location and vascular characteristics of the Castleman's disease: the second surgical attempt was prematurely stopped due to serious life-threatening bleeding. Probably, a preoperative embolization could be a safety precaution before of a surgical approach on a HV-CD variant . BetweenThus we planned local radiotherapy, choosing a total dose of 45 Gy, that had good results, as previously reported in the literature [ 99 mTc-MDP: technetium-methil-diphosphonate; FDG-PET: phospho-di-glicerato-positron emission tomography; CD21: cluster differentiation 21; CD35: cluster differentiation 35; HHV8: human herpes virus 8; HIV: human immunodeficiency virus; MRI: magnetic resonance imaging; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal antibody, skin changes syndromeCD: Castleman's Disease; CT: computed tomography; LDH: lactato-dehiidrogenase; ERS: eritrosedimentation rate; CRP: c-reactive protein; IL-6: interleukin 6;The authors declare that they have no competing interests.PF has made substantial contributions to conception and design of the manuscript, was involved in clinical management of the patient, drafted the manuscript and gave final approval of its last version. AT has made substantial contributions to conception and design of the manuscript, was involved in clinical management and helped to draft the manuscript. NS and ESGD carried out histology study, suppling all the tissue images and participated in drafting of part of the manuscript. GC was involved in surgical management of the patient, and participated in drafting of part of the manuscript. PG was involved in clinical management of the patient in the phase of radiation therapy and participated in drafting of part of the manuscript. PD has made substantial contributions to conception and design of the manuscript, was involved in clinical management of the patient, helped to draft the manuscript and gave final approval of its last version. All authors read and approved the final manuscript.1PF, AT and PD are physicians of Pediatric Hematology and Oncology Unit, at "G. Di Cristina" Children's Hospital, Palermo. They are AIEOP  members. PF has a peculiar experience in the field of pediatric lymphoma and non oncologic hematology, AT has particularly developed his activity in the field of primary immunodeficiency and histiocytic disorders, while PD is especially involved in the management of children and adolescents with solid tumors.2NS, from Unit of Pathology, "Civico e Benfratelli" Hospital, A.R.N.A.S., Palermo, Italy.3GC, from Pediatric Surgery Department, University of Padua, is chairman of GICOP , SIOP member and co-chairman in various international pediatric oncology trials.4PG, from Unit of Oncological Radiotherapy, "Civico e Benfratelli" Hospital, A.R.N.A.S., Palermo, Italy.5ESGD, from Unit of Pathology, San Bortolo Hospital, Vicenza, Italy. ESGD is one of the AIEOP referring pathologist in the field of Hodgkin and non-Hodgkin Lymphoma.Written informed consent was obtained from the patients' parents for publication of these case reports and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Malignant mesothelioma in the mediastinum is rare and the majority of known cases have been reported as ‘localized mesothelioma’. The present study reports a case of an upper mediastinal tumor, which was diagnosed through thoracoscopic surgery and surgical biopsies of the mass. A computed tomography scan revealed a giant upper mediastinal tumor, adjacent to the aortic arch, trachea, superior vena cava and left pulmonary artery. The vessels in the mediastinum were compressed and were shifted to the lower right. The trachea became stenotic and a small amount of bilateral pleural effusion was observed. The mass was relatively well encapsulated. There was no pleural thickening or clearly swollen lymph nodes in the mediastinum. The histopathological and immunohistochemical examinations of the tumor verified the diagnosis of a malignant mesothelioma. The tumor was demonstrated to be derived from the mediastinal pleural mesothelium cells. The patient received pemetrexed disodium and cisplatin combination chemotherapy for four cycles. At present, the patient is undergoing follow-up. Malignant mesotheliomas are a rare form of cancer derived from pleural mesothelial cells. In contrast to diffuse mesothelioma, localized malignant mesothelioma is uncommon and localized malignant mesothelioma in the mediastinum is extremely rare . MalignaTo the best of our knowledge, only a few cases of localized malignant mesothelioma have been reported in the English-language literature. The present study reports a new case of localized malignant mesothelioma in the upper mediastinum. This study was approved by the ethics committee of Shandong Tumor Hospital, Jinan, China. Written informed consent was obtained from the patient’s family.9/l and the level of carcinoembryonic antigen (CEA) was 1.7 ng/ml. A chest radiograph revealed a projecting abnormal giant upper mediastinal shadow  on July 17, 2012, due to coughing that had lasted for one month and a fever that had been present for seven days. The patient had no history of any remarkable asbestos exposure. No abnormalities were noted upon physical examination and the patient exhibited no heart-involved symptoms. Furthermore, no heart abnormalities were detected by electrocardiogram and color ultrasonography studies. Laboratory tests showed that the leukocyte level was 7.8×10l shadow . A contrl shadow .2) and cisplatin (75 mg/m2) combination chemotherapy for four cycles . At present, the patient, showing no symptoms, is undergoing follow-up.A detailed general examination showed no other metastatic or primary lesions. A CT-guided puncture biopsy of the mediastinal mass failed to provide a pathological diagnosis. Based on the suspicion of a mediastinal tumor, thoracoscopic surgery and surgical biopsies of the mass were performed. During surgery, it was observed that the mass was located in the upper mediastinum under the normal mediastinal pleura and convex to the left chest cavity. The surface of the tumor was smooth and uneven, and the tumor was an elastic-hard mass, measuring 8.0×9.0 cm. Furthermore, the tumor was in a frozen state with the mediastinal tissue, with no metastatic nodules in the pleural membrane and mediastinal pleura, and a small number of light-yellow pleural effusions. Four sections were cut and sent for pathological examination. In the macroscopic analysis, the soft tissue sections were pale and the quality of the material was fine. Through microscopic analysis, it was observed that the large atypical mesothelial tumor cells proliferated in a diffuse or nodular pattern. Frequent mitoses and small foci of tumor necrosis were also observed. Immunohistochemical analysis showed that the tumor cells were positive for cytokeratin CK; , CAM5.2 Pleural mesotheliomas, which usually originate from the mesothelial cells of the pleura and peritoneum, were once rare . HoweverMalignant mediastinal mesotheliomas are uncommon and are generally considered to arise from the mesothelial cells of the pericardium; the majority of cases have been reported as ‘pericardial mesothelioma’ . The laret al , while the markers for adenocarcinoma, CEA and thyroid transcription factor 1 (TTF-1) were negative . CalretiIn the present study, the patient’s mass was initially considered to be a metastatic tumor, although no primary lesions were detected and the mass did not have the typical imaging characteristics of lymphoma. The mass was finally demonstrated to be a primary mediastinal tumor. The differential diagnosis of localized mesothelioma includes lymphoma and thymic carcinoma. However, the imaging and clinical findings are generally unhelpful in the differential diagnosis and tumors may only be differentiated by histopathological and immunohistochemical examinations .There has been significant progress in the treatment of malignant mesothelioma, although it remains extremely difficult to treat. Pemetrexed in combination with cisplatin has been approved as a chemotherapy regimen for the treatment of malignant mesothelioma in America and Europe . This reThe tumor in the present study was finally diagnosed as a malignant mesothelioma occurring in the mediastinum. To the best of our knowledge, only a few cases have been reported in the English-language literature. This type of mesothelioma is extremely difficult to treat at an advanced stage, even if patients receive comprehensive treatment. In addition, it is difficult to reach a final diagnosis at the early stages.


Primary hepatic carcinoid tumor is rare and poses a challenge for diagnosis and management. We presented a case of primary hepatic carcinoid tumor in a 53-year-old female with a complaint of right upper abdominal pain. Computer tomography scans revealed a hypervascular mass in segment 4 of the liver. An ultrasonography-guided biopsy showed a carcinoid tumor. No other lesions were found by the radiological investigations. Surgery resection was performed and histopathological examination revealed a primary hepatic carcinoid tumor. Three years later, recurrence was found and transcatheter arterial chemoembolization was performed. After transcatheter arterial chemoembolization, the patient has been free of symptom and had no radiological disease progression for over 6 months. Surgical resection combination with transcatheter arterial chemoembolization is effective to offer excellent palliation. Carcinoid tumors are neoplasms that originate from the cells of neuroendocrine system, and were first characterized by Oberndorfer in 1907. They are usually low-grade malignant tumors that may cause the carcinoid syndrome by secretion of serotonin and other vasoactive hormones. Most of them occur within the gastrointestinal tract, primarily in the appendix and the terminal ileum . PrimaryA 53-year-old female presented with right upper quadrant abdominal pain and was admitted to our hospital in May 2007. The patient had no history of jaundice, vomiting, flushing or diarrhea. Her past medical history included hypertension and a cholecystectomy due to gallstones 10 years previously. Physical examination showed no abnormality except for the presence of a scar in the right upper quadrant of abdomen. Laboratory investigations, including liver function test, renal function test, blood routine test and tumor markers , were within normal limits. Serum hepatitis B surface antigen and hepatitis C antibody were negative.Computer tomography (CT) revealed a low density nodule in segment 4 of liver. Dynamic CT scans showed enhanced of the nodule in the arterial phase and early washout in the portal phase Figure . Liver uIn May 2007, left hepatectomy was carried out. No asites was noted and the liver was not cirrhotic. Extensive abdominal exploration showed no any other site of primary tumor. The operation was performed successfully and the postoperative recovery was uneventful. The resected liver specimen contained a 4.0 cm tumor. Histological and immunohistochemistry of the tumor confirmed the findings of the biopsy and the resection line was free from tumor invasion.At three years after operation, MRI showed multiple lesions of the liver. No other lesions were found by the radiological investigations which include upper gastrointestinal endoscopy, a small bowel series, colonoscopy, chest CT and abdominal CT scans. The recurrence of carcinoid tumor was highly suspected. Due to the multiple lesion of the liver, resection was not feasible and 4 times of transcatheter arterial chemoembolization  was performed. Six months after the last TACE, the patient was free of symptom and had no radiological disease progression.Carcinoid tumors are defined as neuroendocrine tumor. The origin of primary hepatic carcinoid tumors is not well known. They may arise from scatter neuroendocrine cells in the intrahepatic biliary epithelium. It is also hypothesized that chronic inflammation in biliary system may initiate intestinal metaplasia, which predisposes to the development of neuroendocrine tumors. Another possibility is that they originate from ectopic pancreatic or adrenal tissues found within the liver .PHCT are very rare. There are only about 94 cases reported in the current literature [Ultrasound is an inexpensive and easily available imaging test for evaluation of liver lesions. Solid masses with cystic areas and hyperechoic or mixed pattern are common findings of PHCT detected by ultrasonic. Contrast-enhanced US showed a rapid and dense enhancement without parenchymal stain ,6. ConceThe diagnosis of hepatic carcinoid tumor is mainly based on histological and immunohistochemistry examination. In our case, histological examination of the biopsy specimen and the resected specimen revealed that the tumor cells were round or columnar cells with round nuclei and pale eosinophilic cytoplasm. In immunohistochemistry these cells showed positive for the neuroendocrine markers synaptophysin and chromorgranin A. The differentiation between primary and secondary hepatic carcinoid tumor is impossible identified by histology alone. Because liver is a common site for carcinoid metastasis, the diagnosis of PHCT requires intensive preoperative imaging, operative inspection and long-term follow-up. In our case, the patient was followed up for about 4 years after resection. Any other extrahepatic lesion was not found radiologically during follow-up, except for recurrence in the liver three years later after resection. Therefore, we diagnosed this tumor as a PHCT.Surgical resection is the preferred treatment for PHCT and has provided favorable outcomes. One study showed that postoperative 1-, 5-, and 10-year survival rates were 88%, 80% and 68%, respectively . Liver tIn summary, primary hepatic carcinoid tumor is a rare tumor. The diagnosis of primary hepatic carcinoid tumor is base on the histological examination and the exclusion of metastasis disease. Surgical resection is the preferred treatment. Transcatheter arterial chemoembolization is effective to offer excellent palliation for the multiple recurrence lesions.PHCT: Primary hepatic carcinoid tumors; CT: Computer tomography; TACE: transcatheter arterial chemoembolization.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GJ and CX wrote the manuscript. HZ, WJ and BL collected and analyzed the clinical data. GJ and HZ performed the histological examination. All authors read and approved the final manuscript.


Primary ureteral neoplasms are very rare and its prevalence is less than 1% of all genitourinary neoplasms. We report a symptomatic giant ureteral fibro epithelial polyp in adult women presenting as a bladder mass which was resected ureteroscopically and reported at the first time from Iran. Cystoscopy is growing use in the treatment of urinary tract lesions Cystoscopy can be used in large lesions in centers with experience rather than open surgery. Primary ureteral neoplasms are very rare in adults and are responsible for less than 1% of all genitourinary neoplasms . The UFPA medical center diagnosed dysuria with obscure lower abdominal pain  in a 44-year old female patient two weeks ago. Microscopic hematuria results from urine and blood sample laboratory analysis. She had no remarkable history of renal stone, urinary tract infections or any urinary stigmas. Physical examination has shown mild tenderness in right postvertebral angel without any other significant evidence. In hydronephrosis, a small effect has been diagnosed in the urinary bladder (US) which located on the right ureterovesical junction without any evidence of hydronephrosis. Computed tomography (CT) scan and IVP have revealed a small vegetative mass with 50×18 mm diameter situated near to trigon . CystoscNeoplasms which originated from ureter are rare and commonly malignant. However, UFPs are the most common non-malignant neoplasms; they are still rare clinically and primarily seen in males and on the left side . UFPs grWe report a case of large UFP presenting a vegetative bladder mass excised ureteroscopically. Reviewing the English literature illustrated multiple reports of UFP, but ureteral fibroepithelial polyp elongated into the bladder cavity was rare which mimicked bladder mass resected ureteroscopically , 8-15. U


Inflammatory myofibroblastic tumor is currently considered to be a low-grade neoplasm, and it rarely involves the heart. We reported a rare case of a 59-year-old female who received cardiac surgery for complete resection of inflammatory myofibroblastic tumor in the left atrium. Five months after surgery, the patient presented with acute cardiogenic pulmonary edema and subsequent sudden death due to a left atrial tumor which protruded into the left ventricle through mitral annulus during diastole. The recurrence of inflammatory myofibroblastic tumor in the left atrium was strongly suggested clinically. Dear Editor,Inflammatory myofibroblastic tumor (IMT) occurs mostly in children and young adults  and usuaA 59-year-old Chinese female was hospitalized because of exertional dyspnea for 2 weeks. Transthoracic echocardiography showed a left atrial mass Figure A. CoronaUp to now, the pathogenesis of IMT remains unclear, although it is thought to be an abnormal and exaggerated immunologic response by proliferated spindle cells and primary myofibroblasts to injury, inflammation, and infection. The diagnosis of IMT can be difficult due to the wide morphological spectrum. Coffin et al. have classified the histological findings of extrapulmonary IMT into three patterns: (a) spindle cells in a myxoid background with a vascular and inflammatory component resembling nodular fasciitis (myxoid/vascular pattern); (b) compact spindle cells with intermingled inflammatory cells resembling fibrous histiocytoma (compact spindle cells pattern); and (c) dense plate-like collagen resembling a desmoid or scar (hypocellular fibrosis pattern) . In the IMT is considered a low-grade neoplasm. The recent classification of the World Health Organization recognized the uncertainty of its biological nature, that is, its clinical features might resemble malignant neoplasia. Whenever feasible, complete surgical resection remains the mainstay of treatment. Up to now, the recurrence rate of pulmonary IMT is low. However, local recurrence rate of extrapulmonary IMT is up to 25 % . To the IMT = inflammatory myofibroblastic tumor.YX collected data and drafted the manuscript. XC carried out the literature research. LM was helpful in literature review. WY conceived the study and critically revised the manuscript.The authors declare that they have no competing interests.


Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness.A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit.Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness. Glial heterotopia also known as Nasal glioma represents collections of normal glial tissue in an abnormal location distant to the central nervous system with no intracranial connectivity . The mosA 6-year-old boy presented to the outpatient department of our institution with complaint of a painless mass over the medial aspect of the left eye near the nasal bridge since birth. There was no history of pain, nasal bleeding or nasal obstruction. On examination, the best-corrected visual acuity was 20/20 in the right eye and 20/1200 in the left eye. There was a firm, regular, mobile, non-pulsatile and non- tender large mass involving the lateral part of nose and medial aspect of left orbit using lateral displacement of the left eye ball Figure . The walThis case emphasizes on the appropriate diagnosis of the midline nasal masses and the importance of early diagnosis and treatment for the better visual and cosmetic prognosis. Earlier the diagnosis better is the treatment and the probability of visual recovery is more. They are generally present at birth, or become manifest within the first few years of life, but can be seen in any age group. In our Nasal gliomas may masquerade as encephalocoeles or dermoid cysts due to similar embryological origin. The nasal glioma, however, is ectopic sequestrated tissue and not a herniated structure and careTo the best of our knowledge, the effect in visual and refractive status of the eye caused by these masses has been rarely addressed in previous reports. It is interesting to note that there was presence of -12.00D of against the rule astigmatism in our case. This signifies that the mass had been pressing the globe horizontally leading to the steeper horizontal meridian. The child was unilaterally blind most probably due to meridional amblyopia as suggested by the keratometry and refractive findings. Hence, the amblyogenic effect of the mass on the globe should also be considered while evaluating these types of cases.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor in-Chief of this journal.The authors declare that they have no competing interests'.RS and GBS were involved in medical and surgical management of the patient. RS, NP and SS were involved in literature review, conception, design and preparation of manuscript draft. DNS critically reviewed and provided important intellectual contents. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/11/34/prepub


Alpha-fetoprotein (AFP) is an oncofetal protein produced by hepatocellular carcinoma (HCC). AFP level can also be elevated in other neoplastic or non-neoplastic conditions. An elevated AFP level has high diagnostic significance for HCC; at a level of >200 ng/mL, the probability of HCC is >90%. The aim of the present paper is to report a patient who underwent curative resection of HCC, who had a persistently elevated AFP level postoperatively but did not develop recurrence during a 2-year follow-up period. A review of the literature is also presented.An 82-year-old male was referred following a computed tomography scan showing a 160 mm diameter mass in the left lobe of the liver. This huge mass was diagnosed as HCC, arising in the absence of cirrhosis or viral hepatitis. After tumor removal, the patient’s high AFP level persisted for 2 years.As steatosis was the only pathological change in the remnant liver, this may have caused the persistently elevated AFP level in this patient. Hepatocellular carcinoma (HCC) is commonly associated with hepatitis B or C virus infection, and is particularly frequent in patients with cirrhosis . HCC ariHCC causes 1 million deaths every year, making it the fifth most common malignancy worldwide, and the incidence is increasing -8. The cIf the tumor is resectable, surgery is considered to be the curative treatment choice ,13. PostThe aim of the present paper is to report a patient with a normal liver who underwent curative resection of HCC, followed by a persistently elevated AFP level during two years of follow-up without recurrence. A review of the literature is also presented.An 82-year-old male was referred to us after CT scanning showed a 160 mm diameter mass in the left lobe of the liver . He underwent open resection in April 2009. The tumor occupied all of liver segments 2 and 3, and was adherent to the stomach. The right lobe of the liver was normal in appearance. A left hepatic lobectomy was performed. Histological examination of the surgical specimen showed hepatocellular carcinoma with a solid trabecular pattern, and thick fibrotic septa which extended into the normal hepatic tissue at the resection margin  scans showed no evidence of tumor recurrence. Serum AFP levels were 15.7 ng/mL in November 2009 and 8.6 ng/mL in February 2010. A CT scan in March 2010 and PET scan in April 2010 did not show any evidence of recurrence. AFP levels were 17.2 ng/mL in May 2010; 18.4 ng/mL in September 2010; 15.4 ng/mL in December 2010; 16.2 ng/mL in March 2011; 3.69 ng/mL in June 2011; 3.58 ng/mL in September 2011; and 3.4 ng/ml in December 2011  ,3,23. ItThe frequency of postoperative recurrence is similar in patients with hepatitis B or C virus infection and those without, but patients without chronic hepatitis have better liver function and tend to get fewer nodules in the remnant liver, which enables easier resection of recurrences .Extensive resection with a wide tumor margin is technically easier in HCC arising in a non-cirrhotic liver than a cirrhotic liver, resulting in a lower probability of recurrence and a better prognosis -26.Elevated AFP level is not always associated with liver disease. Other conditions (neoplastic or non-neoplastic) can also cause a transient or persistent elevation in APF level, and should be considered in the differential diagnosis. Non-seminoma testis cancer can cause an elevated AFP level in association with elevated levels of other tumor markers such as beta-human chorionic gonadotropin and lactate dehydrogenase ,28. A puPregnancy may cause an elevated AFP level, especially when the pregnancy is complicated by an abnormality such as a spinal cord defect .Obesity and diabetes seem to be associated with HCC. These conditions are risk factors for cirrhosis, but their role in the non-cirrhotic liver is more controversial .Hepatic steatosis can also cause a high AFP level. Steatosis can be divided into two phases; during the first phase, lipids accumulate in the hepatocytes, and in the second phase, factors such as oxidative stress, proinflammatory cytokines, mitochondrial dysfunction, and lipid peroxidation damage the hepatocytes and cause inflammation and fibrosis. In 2009, a study by Babali et al.  found thIn our patient, steatosis was the only abnormality of the hepatic parenchyma detected on pathological examination, and was the only condition that could have caused the persistently elevated AFP level, as laboratory tests and imaging results eliminated all other possible causes.Non-alcoholic steatohepatitis (NASH) can induce HCC by unknown mechanisms, in which insulin resistance seems to play an important role. Insulin resistance causes inhibition of hepatic mitochondrial fatty acid oxidation and stimulation of microsomal peroxidases, resulting in increased intracellular concentrations of fatty acids, which may cause oxidative DNA damage .NASH can evolve into cirrhosis before giving rise to HCC, but the cirrhosis stage is not necessary, as there are many reported cases in the literature of HCC arising directly from NASH. NASH therefore has to be considered as a possible cause when HCC arises in a non-cirrhotic liver ,35. HCC Another rare condition that may cause an elevated AFP level in a patient with a normal liver is hereditary persistence of AFP (HPAFP), which is asymptomatic. Only 19 families with HPAFP have been described over the past 28 years, many of whom also had urological disorders . HPAFP can, therefore, also be considered in patients with an unexplained high AFP level ,38.In conclusion, we present a case in which hepatic steatosis is the only possible cause of the persistently elevated AFP level after curative resection of HCC.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.AFP, Alpha-fetoprotein; CT, Computed tomography; HCC, Hepatocellular carcinoma; NASH, Non-alcoholic steatohepatitis; PET, Positron emission tomography; HPAFP, Hereditary persistence of alpha-fetoprotein.All authors declare that they have no competing interests.IDC was the principle investigator who prepared, organized, and edited all aspects of the manuscript, and the surgeon who performed the operation. MM supported the work of the principle investigator in writing and editing the manuscript. AT supported the work of the principle investigator in writing and editing the manuscript. AA supported the work of the principle investigator in preparing the manuscript. AG was the pathologist who performed the pathological examination of the surgical specimen. GC supported the work of the principle investigator in preparing the manuscript. GB supported the work of the principle investigator in preparing the manuscript. All authors read and approved the final manuscript.


Rhinoliths are uncommon clinical entities reported in clinical practice as unusual cause of unilateral nasal obstruction and foul smell nasal discharge. Rhinolith is calcified material found in the nasal cavity incidentally or due to patient complaint. It should be suspected when patient presents with nasal symptoms and found to have stony mass showed radiologically. We reported a 28-year-old Saudi male with left sided (LT) nasal obstruction and foul smell discharge for 5 years suspected as being due to foreign body presence since childhood due to head trauma following car accident in sandy area. Rhinoliths are calcified material around intranasal foreign body. They can be endogenous if occur around body tissues as tooth or exogenous if they occur around foreign subject as stones, cotton, or beads. They are found usually in anterior nasal cavity commonly associated with narrowing due to deviated septum, spurs, and/or turbinate hypertrophy. Endoscopic appearance is the main step in diagnosis which can be supported by radiology. Complete resolution of symptoms occurs after endoscopic surgical removal –3.A 28-year-old Saudi male presented to ORL HNS clinic referred from another hospital for his complaint of left (LT) nasal obstruction and foul smell discharge for five years. Symptoms were progressively noticed and disturb the patient's life in the last 3 years. He received multiple courses of antibiotics and nasal steroids with no benefit. He had no history of foreign body introduced into nasal cavity. He had history of head trauma after car accident at childhood in sandy area. Anterior rhinoscopy showed irregular hard material with crustations and thick secretions around, stuck between septal spur and inferior turbinate at LT anterior nasal cavity . Trials Bartholin first described rhinolith in 1654. Then more than 600 cases had been reported –5.Rhinolith was found frequently as incidental finding during rhinoscopy as irregular, hard dark mass with greenish foul smelling crustations around. Radiological investigations such as plain X-ray and CT scan can support diagnosis and direct the management. Maclntyre was the first to describe rhinolith radiographically in 1900 . Rhinoli To our knowledge this case is the first case reported here in Saudi Arabia and the most interesting is the long standing time it takes to present, and ultimately the final diagnosis was reached by clinical suspicion and endoscopic management is the standard modality of treatment.


Inflammatory myofibroblastic tumors (IMTs) are rare benign clinical and pathological entities. IMTs have been described in the lungs, abdomen, retroperitoneum, and extremities but rarely in the head and neck region. A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of the right sphenoid and clivus. MR imaging showed a large tumor of the skull base which was invading the sella turcica, right cavernous sinus, and sphenoidal sinus. A biopsy was performed and revealed an IMT. Corticosteroids were given for 3 months but were inefficient. In the framework of our pluridisciplinary consultation, fractionated conformal radiotherapy (FRT) was indicated at a low dose; 20 Gy in 10 fractions of 2 Gy over 12 days were delivered. Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT. Two years later, the patient is well. Inflammatory myofibroblastic tumors (IMTs), also called inflammatory pseudo tumors or plasma cell granuloma, have been defined in the classification of soft tissue tumors as a lesion composed of myofibroblasts with inflammatory infiltrate . This tuThis tumor has been primarily described in the soft tissues and viscera of children and young adults , with eq A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of right sphenoid and clivus . MR imag Three months treatment with corticosteroids was inefficient. In the framework of our neurooncological plurisdisciplinary consultation, fractionated conformal radiotherapy (FRT) was indicated at a low dose; 20 Gy in 10 fractions of 2 Gy over 12 days were delivered as corticosteroids were decreased until definitive arrest in 30 days.Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT . Two yeaOur literature review was performed with three key words: inflammatory myofibroblastic tumors (IMTs), inflammatory pseudotumors (IPTs), and Tolosa-Hunt syndrome (THS). IMTs are histologically characterized by dominant myofibroblastic invasion and variable inflammatory infiltration. They are generally benign but sometimes can be locally aggressive. Definition of the 1994 WHO classification of soft-tissue tumors refers to “a tumor composed of differentiated myofibroblastic spindle cells usually accompanied by numerous plasma cells, and/or lymphocytes” . The feaThe clinical presentation depends on the location of the tumor. Patients can present with fever, pain, swelling, otorrhea, cranial nerve palsy, and gait disturbance. Radiologically, computed tomography and MRI are both required to assess the extent of bone destruction and infiltration of adjacent structures. Common MRI findings in most reported cases of IMT are low signal intensity on T2-weighted imaging and homogeneous contrast enhancement. They can be multiple . Prompt Low doses of oral corticosteroids are often prescribed and are effective in the management of these tumors , 22, 23.Radiotherapy has usually proved to be ineffective , 26, 28.There have been a few reports regarding high-dose radiation therapy for IPT –31. In tSome reports showed 66% to 100% complete remission rates in orbital IPT patients with radiotherapy . SasagawIn our case, FRT was indicated since we had previous experience in a case of TSH successfully treated with it . FRT wasThe pathogenesis of IMT remains unknown. It has been associated with a number of diseases or agents including Epstein–Barr virus  and is tIMT of the skull base is very uncommon and may mimic malignant tumors, so it is important to recognize this entity. The recommended treatment is complete surgical resection with adjuvant corticosteroid treatment. Considering the tumor location, radiotherapy may be a good indication as in our case, even though the efficacy of such treatment is debatable. Further case reports are needed to ascertain the optimal therapeutic regimen.