Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. The presence of single or multiple raised cutaneous lesions characterize this self-healing disorder. Extracutaneous sites are rare.We present a rare case of oral juvenile xanthogranuloma in a 49-year-old Caucasian woman. The histopathologic diagnosis of the lingual neoformation was histiocitary proliferation with the presence of giant cells, Touton type, compatible with juvenile xanthogranuloma.To establish an accurate diagnosis, microscopic evaluation and immunohistochemical staining are necessary. Dentists, dermatologists and general practitioners may be the first to recognize this rare condition during the inspection of the oral cavity. This pathological condition has subsequently been reported under various names such as nevoxanthoendothelioma, juvenile xanthoma or xanthoma tuberosum [Juvenile xanthogranuloma is an uncommon non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. This lesion was first reported by Adamson in 1905 [uberosum . Its moduberosum  in 1936 uberosum .The pathogenesis is unknown, although the disease is believed to be a reactive rather than a neoplastic process. It is caused by the proliferation of plasmacytoid monocytes in response to an unknown etiologic agent, possibly either physical or infectious .Juvenile xanthogranuloma is a benign and self-healing disorder which is characterized by the presence of single or multiple raised cutaneous nodules, yellow-brown to reddish in color and usually measuring from a few millimeters to a few centimeters in diameter. Sometimes superficial telangiectasias and an erythematous border may also occur . The moset al. [Juvenile xanthogranuloma is not limited to cutaneous sites, however. The eye is the most common extracutaneous site, with other affected organs including the lung, kidney, pericardium, colon, central nervous system, liver, spleen, eye/orbit, bone, kidneys, adrenal glands, testis and ovary . Treatmeet al. , oral leThe aim of this report is to highlight an additional case of solitary extracutaneous juvenile xanthogranuloma involving the tongue in an adult patient.Approximately five years ago, a 49-year-old Caucasian woman came to our hospital for diagnosis, evaluation and treatment of a lingual lesion.She complained of constant dysphagia and dysgeusia. These symptoms had worsened in the past three months because of growth of the lingual mass Figure .Her medical history was uneventful, except for breast cancer several years earlier, and included no history of smoking or drinking.Clinical examination revealed the presence of a nodular neoformation with a fibrotic aspect on the dorsum of the tongue. Its consistency was hard, and it consisted of nodular, sessile microsurveys.Because of the rapid clinical evolution of the lesion in a patient with previous breast cancer, the pathologist (UM) decided to perform an incisional biopsy immediately to exclude malignancy. Local anesthesia was administered deep around the proposed biopsy site. The specimen was taken with a scalpel to a depth of at least 5 mm, and a 3- to 5-mm margin of clinically normal mucosa was also included. The tissue was submitted in 10% neutral buffered formalin fixative for microscopic examination. A resorbable suture with polyglactin  was used. An axial magnetic resonance imaging (MRI) study was also requested for a diagnostic scope Figure .The histopathologic diagnosis was histiocytic proliferation with the presence of giant cells, Touton type, compatible with juvenile xanthogranuloma Figure .After the diagnosis, the patient was included in a bimestrial follow-up program. During the first two months, we observed a modest volume reduction of the neoformation together with a reduction of its consistency.Afterward the lingual lesion underwent a spontaneous involution. To date, a hyperchromic area and a mild atrophy remain, without any evidence of local recurrence after follow-up of more than four years Figure .Juvenile xanthogranuloma is a benign disorder which characteristically develops as a well-demarcated mass in the dermis of a child. This disease heals spontaneously within one to several years in the majority of cases.In most cases, cutaneous juvenile xanthogranuloma has a favorable prognosis and does not require any treatment at all, except for periodic follow-up until its disappearance . AlthougOf the 31 previously reported biopsy-proven cases of juvenile xanthogranuloma with onset in the oral cavity, only eight occurred on the tongue. After the biopsy, all patients did well. Recurrence after partial or complete excision of lesions has been reported in previous studies .Because juvenile xanthogranuloma in the oral cavity is extremely rare and usually unifocal, it may be difficult to diagnose. Because its clinical appearance may be quite similar to dental abscess giant cell fibroma, gingival cyst, fibroma, peripheral ossifying fibroma, peripheral giant cell granuloma, peripheral odontogenic fibroma, pyogenic granuloma and lipoma, the only way to establish an accurate diagnosis is through microscopic examination, including immunohistochemical staining. A close follow-up after initial biopsy may be preferable to aggressive resection of the lesion when histological diagnosis is unequivocal.General practitioners, dermatologists and dentists may be the first health care professionals to see patients with symptoms and signs of oral disease, which should be referred to an oral pathologist and oral surgeon for diagnosis and treatment.We have presented a rare case of lingual juvenile xanthogranuloma. In our opinion, although gross total resection of lingual xanthogranuloma is regarded as curative, one may decide alternatively to follow up closely for spontaneous regression after initial biopsy.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AV analyzed and interpreted the patient data regarding the oral lesion and the histological examination. UM performed the biopsy. FV reviewed the manuscript and followed the patient together with AV and UM during the more than four years of follow-up. All authors read and approved the final manuscript.


Duodenal perforation is an uncommon complication of endoscopic retrograde cholangio-pancreatography (ERCP) and a rare complication of upper gastrointestinal endoscopy. Most are minor perforations that settle with conservative management. A few perforations however result in life-threatening retroperitoneal necrosis and require surgical intervention. There is a relative paucity of references specifically describing the surgical interventions required for this eventuality.Five cases of iatrogenic duodenal perforation were ascertained between 2002 and 2007 at Cairns Base Hospital. Clinical features were analyzed and compared, with reference to a review of ERCP at that institution for the years 2005/2006.One patient recovered with conservative management. Of the other four, one died after initial laparotomy. The other three survived, undergoing multiple procedures and long inpatient stays.Iatrogenic duodenal perforation with retroperitoneal necrosis is an uncommon complication of endoscopy, but when it does occur it is potentially life-threatening. Early recognition may lead to a better outcome through earlier intervention, although a protracted course with multiple procedures should be anticipated. A number of surgical techniques may need to be employed according to the individual circumstances of the case. Duodenal perforation is an uncommon complication of endoscopic retrograde cholangiopancreatography (ERCP) and a very rare complication of upper gastrointestinal endoscopy. Most series report a majority of non-life-threatening perforations which settle with conservative management ,2. ThereFive cases of iatrogenic duodenal perforation occurring between 2002 and 2007 at Cairns Base Hospital are presented for comparison, with reference to a review of ERCP at Cairns Base Hospital for the years 2005/2006. Further, a focused review of the literature was undertaken to inform discussion of the surgical management of such cases.Cairns Base Hospital is a secondary referral hospital in Far North Queensland, Australia. It serves a catchment population of approximately 250 000, 15% of which identify as Indigenous Australian. Hospital surgical audit and endoscopy records for the period 2002–2008 were searched for cases of duodenal perforation following endoscopy or ERCP. Age, sex, indication for endoscopy/ERCP, timing or delay to diagnosis and definitive management, type of perforation, surgical management, complications, length of stay, and late morbidity were recorded for each case.An audit of ERCP at Cairns Base Hospital for the two year period 2005/2006 was utilized to determine incidence of complications of ERCP and is presented in Tables For the focused literature review, a PubMed search was undertaken using the terms “duodenal perforation”, “endoscopic” and “retroperitoneal necrosis”. Case-based articles cited by reviews were secondarily sourced. Articles with English language abstracts were considered, and excluded if endoscopy was not the cause of the perforation (rather a treatment) or if specific operative details were not reported. Similarly, only cases that underwent some form of surgical management were included.Approval to access and analyze de-identified patient records for this study was given by the Human Research Ethics Committee of the Cairns and Hinterland Health Service District.Five patients sustaining iatrogenic duodenal perforation were identified. The clinical data pertaining to these are presented in Table In cases 1, 2 and 4, the actual duodenal perforation could not be identified at operation. This may have been due to a smaller size of the perforation and/or delay to surgery resulting in difficulty identifying the perforation. Ongoing leakage in Case 2 necessitated subsequent pyloric exclusion and gastrojejunostomy. Case 5, where endoscopy alone was performed, is likely to have perforated through a duodenal diverticulum, which is a known risk factor for perforation both in endoscopy and ERCP . This laTiming of intervention was assisted by serial computerized tomography examination. In the four cases treated surgically, definitive intervention consisted of open surgical drainage with or without subsequent CT-guided percutaneous drainage of amenable collections. While open surgical drainage was immediately effective in all cases, percutaneous drainage as an initial intervention was not effective in Case 1, attributable to the large volumes of semi-solid necrotic material in the retroperitoneum of this patient. This is consistent with experience in pancreatic necrosectomy ,8. In coRetroperitoneal necrosis was progressive and in most cases multiple operations were required due to ongoing symptoms. An oblique right flank to right iliac fossa incision was performed in Cases 1 and 5 giving good access to the upper and lower right retroperitoneal space and to the presacral space. A feature of the three cases in males was involvement of the right inguinoscrotal tract, with Cases 2 and 5 requiring separate drainage of symptomatic inguinoscrotal collections. None had pre-existing hernias.One patient (Case 4) died indirectly as a result of the perforation, from sepsis associated with vascular access. This patient had significant co-morbidities, being steroid-dependent for pulmonary interstitial fibrosis and rheumatoid arthritis. Of the four survivors, one recovered quickly with conservative management alone, but the other three endured long hospital stays, underwent multiple surgical and other procedures, and developed short-term and long-term complications as a result of the original perforation and its treatment.All cases in this series were managed by General Surgeons at a regional hospital, serving a population of 250 000 and geographically remote from larger facilities. The endoscopic procedures were performed by a Gastroenterologist and a General Surgeon, both of whom were formally trained and accredited in these skills. As upper endoscopy and now ERCP are readily available in larger regional centres, an awareness of this serious but fortunately rare complication and its clinical course is useful for General Surgeons faced with its management. Certainly Case 5, undertaken with the benefit of specific experience gained in the management of Case 1, does seem to have had a better quality outcome, with shorter length of stay, fewer procedures, and fewer complications.While duodenal perforation at endoscopy alone is extremely rare, the rate during ERCP is significantly higher, estimated to be between 0.4 and 1% . The ratFour types of duodenal perforation have been described – Type 1: lateral duodenal wall, Type 2: peri-Vaterian duodenum, Type 3: bile duct, and Type 4: tiny retroperitoneal perforations caused by the use of compressed air during endoscopy. Most perforations are Type 2, due to concomitant endoscopic sphincterotomy, and may be suitable for a trial of conservative management -15. In oVarious management algorithms for duodenal injuries have been proposed, largely focusing on early diagnosis and the decision for surgical management -21. IndiPublished case series and reports regarding possible surgical management options for endoscopically induced Type 1 and 2 duodenal injuries are summarized in Table The other important issue to contend with in duodenal injuries is the management of retroperitoneal necrosis or sepsis. In most cases where laparotomy is performed, some degree of debridement and placement of drains is undertaken. This may be all that can be done if primary duodenal repair is not feasible, or the perforation cannot be localized amid the devitalized tissue. As illustrated by our own case series, repeated drainage procedures are often necessary if signs of recurrent sepsis develop. As has been noted by other authors,  males arRetroperitoneal necrosis due to duodenal perforation is a rare but serious complication of ERCP. Early recognition based on risk factors and clinical suspicion may lead to a better outcome, although a protracted course with multiple and various types of procedures should be anticipated. Urgent interventions typically involve debridement and drainage, duodenal repair where feasible, and if indicated, duodenal diversion or other protective procedures. Familiarity with a number of possible surgical strategies is desirable due to the need to adapt to individual circumstances. Surgical management plans should also take into account any underlying pathology that was the initial indication for the endoscopic procedure, although definitive procedures may not be feasible at first operation. The use of ERCP for purely diagnostic purposes should only be considered where less invasive imaging modalities are not possible.CBD: Common bile duct; CVL: Central venous line; CT: Computerized tomography; ERCP: Endoscopic retrograde cholangiopancreatography; EUS: Endoscopic ultrasound; LFTs: Liver function tests; MRCP: Magnetic resonance cholangiopancreatography; SIRS: Systemic inflammatory response syndrome; UTI: Urinary tract infection.The authors declare that they have no competing interests.RT contributed clinical cases to the series, co-wrote the manuscript and attended to reviewer comments. CS contributed clinical cases to the series and co-wrote the manuscript. PB provided the summary data of institutional endoscopy outcomes and edited the first draft of the manuscript. All authors read and approved the final manuscript.


Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon benign intraosseous lesions. Simultaneous occurrence of both lesions is extremely rare. We present an example of concomitant FD and ABC in a 7 year-old with left eye blindness and discharge of one month duration. Physical examination revealed a proptotic left eye and bulging of the hard palate. CT and MRI are consistent with FD and ABC that involved the sphenoid and ethmoidal bones bilaterally. Incomplete combined endonasalcranial resection was performed. The patient presented five months postoperatively with a large recurrence and subsequent follow up was lost. Concomitant FD with ABC may occur in paranasal sinuses and may develop rapidly and exhibit locally aggressive behavior. Fibrous dysplasia (FD) consists of rare and benign osseous lesions of unknown etiology. They represent 2.5% of all bone tumors and 7% of benign bone tumors , in younAneurysmal bone cyst (ABC) is rare, benign vascular lesion, and considered secondary to certain pathological bone lesions . ABC repConcomitant FD and ABC are extremely rare with only 13 cases reported previously -12. The We report a rare example of concomitant FD and ABC of the skull base that presented with blindness and rapid local recurrence in a 7 year-old female patient. Also, we are presenting the multidisciplinary team approach of treating such cases.A 7 year-old female, is presented at a local hospital with left eye blindness and discharge of one-month duration.Physical examination showed a slightly proptotic left eye with no light perception, absence of direct pupil reflex and optic nerve atrophy in the left eye. In addition to, a slight hypertelorism. Otolaryngologic examination showed rhinorhea, bulging hard palate, and a left nasal mass Figure .Preoperative CT findings Figure  were conA combined endonasal-cranial approach to resect this lesion that entailed an extradural bi-coronal craniotomy (pericranium saving technique for closure) and medial bilateral orbitotomy was performed Figure . The preHistological evaluation revealed fibroblastic proliferation comprised of benign fibrous spindle areas with mature irregular bone formation Figure . Cystic Five months after surgery, a recurrent large tumor was noticed, estimated to be 30.5 cc Figure . The patWe present a rare case of FD with ABC in the skull base of a female child with sudden blindness. The radiological and pathological findings were consistent with the diagnosis in this case. In contrast to previous reports, however, the present case progressed rapidly and led to bone destruction and extension to adjacent vital structures. Saito et al  presenteThe presentation of these lesions, as in our case, depends on the location, rate of growth and site of involvement. Symptoms may include painless mass, pressure symptoms, nasal obstruction, headache and loss of vision. Acute hemorrhage into the cyst(s) may causes pain, rapid enlargement and/or rupture and subarachnoid hemorrhage . All repABC in distal tibia has been treated by high-energy, low-dose radiation on one patient and was successful, with no recurrence during the five years follow up . HoweverOur case represents a rapid developing fibrous dysplasia with aneurismal bone cyst at the skull base leading to unilateral blindness. Awareness of such cases may lead to early detection and diagnosis for effective therapy.A multidisciplinary team is needed for both diagnosis  and management  in this kind of diseases.The authors declare that they have no competing interests.AST: writer, alignment and drafted the manuscript. KHQ: surgen, writer. EB: radiological diagnosis, writer. LS: neuronavigation, writer. ABM: pathological diagnosis, writer. AJS: surgen, writer. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A ganglioneuroma is a benign neoplasm arising from neural crest cells of the sympathetic nerve fibers and is most commonly seen in the posterior mediastinum or retroperitoneum. Although very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses. In young adult women, neck incisions made for excision of these benign tumors should be avoided whenever possible.We herein describe the case of a 19-year-old Japanese woman with a ganglioneuroma. The tumor was found in the parapharyngeal space, an unusual location. A fine-needle aspiration biopsy was performed but was considered inadequate to make a definitive diagnosis, so the asymptomatic lesion was surgically excised using a Weerda laryngoscope. The lesion measured 4 × 3 cm in size and was encapsulated. A pathological analysis showed the presence of two distinct cell types, ganglion cells and Schwann cells, embedded in a loose myxoid stroma. The final diagnosis was a ganglioneuroma.A complete excision was made possible by using a transoral approach with a novel use of the Weerda laryngoscope. Although its applicability to specific cases depends on the location, size and nature of the tumor, we believe that the Weerda laryngoscope will continue to be useful for performing transoral surgery for cervical tumors. Ganglioneuroma is the most differentiated benign counterpart of neuroblastoma and originates similarly from neural crest cells that normally migrate into the adrenal medulla and sympathetic ganglia ,2. GanglWe herein describe a case of ganglioneuroma that was found in the parapharyngeal space, an unusual location ,5. ComplA 19-year-old Japanese woman presented to our hospital complaining of a mass in the middle of the pharynx, but without any systemic signs or symptoms. A mass measuring approximately 25 mm in diameter was noted in the middle of the pharynx. The mass was elastic in consistency, tender, and mobile, with a normal overlying oral mucosa. The patient's general condition and family history were unremarkable. A plain computed tomographic (CT) scan of the neck showed a low-density, homogeneous, neoplastic lesion occupying the right parapharyngeal space. A slight partial contrast effect was observed. Magnetic resonance imaging (MRI) showed the tumor to have low signal intensity on T1-weighted images and heterogeneously high signal intensity on T2-weighted images. The tumor exhibited an uneven contrast effect on the T1-weighted coronal images Figure . HoweverA gross pathological examination revealed a well-circumscribed 72 × 33 × 11-mm tumor with a fibrous capsule. The cut surface was whitish and exhibited a whorled pattern. A histopathological examination showed the tumor to be composed predominantly of nodular neurophilic ganglioneuromatous stroma with a minor component that consisted of collections of maturing ganglion cells which were unevenly distributed. The fibrous capsule of the tumor was thinned in areas, but no extracapsular extension was identified. The histological findings were consistent with ganglioneuroma, maturing subtype (Schwannian stroma-dominant neuroblastic tumor) (Figure After surgery, the patient exhibited left palpebral ptosis and anisocoria with ipsilateral mydriasis typical of Horner's syndrome. These symptoms resolved completely within three months.Ultrasonography of the abdomen was performed to exclude any visceral involvement. The patient's postoperative course was satisfactory, and she was discharged with no difficulties. No local recurrence or distant metastases have been observed during the five years since her surgery.Sympathetic nervous system tumors include neuroblastomas, ganglioneuroblastomas and ganglioneuromas . All areGanglioneuromas are composed only of mature elements and rarely if ever metastasize, so the prognosis is usually favorable ,7.Ganglioneuromas are usually found in the posterior mediastinum or in the retroperitoneum ,4. A repAlthough very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses, along with infectious cervical adenitis, branchial cleft cyst and some other malignancies, such as other variants of neuroblastic tumors, sarcoma and malignant lymphoma . SurgicaNumerous approaches for resection of parapharyngeal space neoplasms have been described, including the transcervical approach, the transparotid approach, the transcervical-transpharyngeal approach, the infratemporal fossa approach and combinations of the these ,9. The lIn our present case of a 19-year-old woman, a tumor was observed in the neck and was found to be prominent on the right side of the oropharynx when her mouth was open. To avoid performing an excision through an incision if possible, we attempted an excision using a transoral approach with a Weerda laryngoscope.The Weerda laryngoscope has recently been reported to be useful for the removal of early esophageal lesions  and embeAlthough very uncommon, ganglioneuromas must be included in the differential diagnosis of neck masses. Although its applicability to individual cases may depend on the location, size and nature of the tumor, we believe that the Weerda laryngoscope will continue to be useful for transoral surgery for cervical tumors.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.HY, AI and KI performed surgery on the patient. AA analyzed the pathological findings as a pathologist. HY drafted the manuscript. All authors read and approved the final manuscript.


Extragonadal localization of germ cell tumors (GCTs) is rare; to the best of our knowledge, a location in the soft tissue of the arm has never been previously reported in the literature.We report the case of a 37-year-old man who presented with a primary malignant mixed non-seminomatous GCT (teratocarcinoma variety) in the right arm, treated by a combination of cisplatin-based chemotherapy and surgery. After 18 months of close follow-up, no locoregional recurrence or distant metastases have been detected.A combination of chemotherapy and surgery is the most appropriate treatment strategy for extragonadal GCTs, to ensure both local and systemic control. Germ cell tumors (GCTs) are classified as extragonadal if there is no evidence of a primary tumor in either the testes or ovaries ; they tyThe aim of the present work was not only to report what is to the best of our knowledge the first observation of a mixed non-seminomatous GCT of the arm, but to also increase exposure of the various hypotheses that could explain this unusual location. We also discuss the diagnosis, treatment, and prognosis of this entity.2 intravenously, days 1 through 5; cisplatin 20 mg/m2 intravenously, days 1 through 5). A clinical evaluation of response at the end of chemotherapy showed a stable disease. Then, 1 month later, a wide excision with axillary dissection was performed; the excised tumor was partially well circumscribed and measured 57 × 42 × 38 mm and had a uniform, yellowish, solid, and partially nodular appearance on the cut surface. Final pathology revealed the same histological aspect as observed in the biopsy but did not revealed tumoral necrotic patterns. All surgical margins were free, and one of six lymph nodes identified was involved without extracapsular spread (Figures A 37-year-old man presented with a 2-year history of painless swelling of the right arm with a gradual increase in size. A physical examination was normal except for a well circumscribed non-tender mass in the upper two-thirds of the right arm and multiple lymph nodes in the right axilla. Imaging revealed a 53 × 40 mm diameter soft tissue mass that was hyperintense on T2-weighted MRI in the posterior compartment of the right arm, with no bone or vascular invasion Figures  and 2. Set al. [Primary GCTs of extragonadal origin comprise 3% to 5% of all germ cell tumors. Extragonadal GCTs arise from midline structures ,3. A caset al. . Herein,et al. . This hyet al. ,8. The eet al. -12. A biet al. . Howeveret al. . It may et al. -17. Extret al. .Because an extragonadal GCT may be curable with cisplatin-based chemotherapy, many recommend that the diagnostic evaluation in such cases should include measurement of the serum tumor markers AFP and β-HCG, immunohistochemical assessment of the biopsy , and cytRegardless of the location, the therapeutic approach of extragonadal GCTs should be multidisciplinary, combining systemic chemotherapy and surgery.The decision of the number and timing of cycles of chemotherapy depends on the disease stage and histological analysis of the surgical specimen.Written informed consent was obtained from the patient for publication of this report. A copy of the written consent is available for review from the Editor-in-Chief of this journal.Hicham Ait Benali and lssam Lalya are equally contributed.The authors declare that they have no competing interests.IL and HA performed the literature review, composed the case report and wrote the manuscript. MA and AB were involved with conception and design and collection and assembly of data. BEK and MA analyzed and interpreted the anatomopathology findings. SB, NB and ME approved the final manuscript. All authors read and approved the final manuscript.


With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas). We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed. Leiomyomas are common uterine neoplasms, but other, benign or malignant, soft tissue tumors are very rare . EpithelWe present herewith a case of uterine epithelioid hemangioendothelioma of low-grade malignancy, the first reported in the English language literature, hoping that it will contribute to the general knowledge within the spectrum of epithelial vascular neoplasms.A twenty-nine-year-old pregnant woman, at 32-month of gestation, was admitted to the Department of Obstetrics and Gynecology, because of premature rupture of fetal membranes, which led to urgent cesarean delivery. Physical examination did not reveal other pathological findings. Patient's personal history was unremarkable, having no previous surgery or use of hormonal drugs. The patient was nulliparous and had no abortions. During surgery, a submucosal endometrial mass was detected in the region of isthmus, which was taken as a leiomyoma. Excision of the tumor was performed and was sent to the Department of Pathology for histopathologic evaluation.Macroscopic examination of the excised specimen showed a relatively circumscribed tumor, 1.5 cm in diameter, of soft consistency. Formalin-fixed, paraffin-embedded tissue sections were stained with hematoxylin and eosin and assessed immunohistochemically for the following markers: CD31 , Vimentin , CD34 , SMA , Myosin , Desmin , Cytokeratin , CD10 , EMA , S-100 protein , and Ki-67 .On microscopic examination, the tumor was composed almost exclusively of well-formed vascular channels embedded in a myxoid stroma . There wClinical and laboratory investigation for extrauterine spread or evidence of distant metastases was negative. No further therapy was administered. The patient is free of disease ten months postoperatively.Epithelioid hemangioendotheliomas are soft tissue tumors of uncertain biological behavior; they are generally considered as being borderline tumors lying within the spectrum of vascular tumors, at one end of which are hemangiomas and at the other end are high-grade epithelioid hemangioendotheliomas, also called hemangiosarcomas , 7. SuchAlthough epithelioid hemangioendotheliomas are usually presented with vaginal bleeding, anaemia, weight loss, and enlargement of the uterus –3, 11, aThe differential diagnosis should always include capillary and epithelioid hemangiomas, primary or metastatic high-grade hemangioendothelioma (angiosarcoma), carcinosarcoma , vascular leiomyosarcoma, and hemangiopericytoma , 10. In The pathogenesis of the tumor is obscure, although Morrel et al.  presenteThe management of the uterine epithelioid hemangioendothelioma remains under consideration, mainly because of its rarity . Olawaiy


The purpose of our study was to retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. We reviewed the CT/MRI findings of six lesions of Epi-HAML and found absence of adipose tissue in the lesions. In addition, recognizing the imaging features of no capsule, and hypervasularity with central punctiform or filiform vessels as a characteristic enhancement may distinguish Epi-HAML from other hepatic tumors. Angiomyolipoma (AML) of the liver is an uncommon benign tumor, with abundant fatty component that can be easily diagnosed by CT or MRI. However, epithelioid AML of the liver (Epi-HAML) is a rare tumor of unpredictable behavior, that contains no or only a minimal amount of adipose tissue and is difficult to distinguish from other hypervascular tumors –3. Rare n = 1), suspected gallbladder stone (n = 1) or on routine health examination (n = 4). Of the six patients, none had a history of cirrhosis or viral hepatitis and none had tuberous sclerosis. All patients were negative for HbsAg and a-fetoprotein (AFP) levels were within normal ranges.All the six patients who had Epi-HAML were women, aged 32–64 years with a median of 47 years. Epi-HAML lesions had been examinated by CT/MRI because of abdominal pain  or Gd-DTPA (gadolinium diethylene triamine penta-acetic acid) of 15 mL were injected intravenously as a bolus, a triphasic contrast-enhanced dynamic exploration during the arterial, portal venous, and delayed phases was performed.All patients received liver biopsy or partial hepatic resection. CT images of the four patients and MRI images of the two patients were available for retrospective analysis and were compared with pathological findings by three experienced radiologists.Helical CT and MR imaging demonstrated an obvious tumor in all patients. The location, size, fat-containing, capsule, non-enhanced images as well as the enhanced images are listed in Table arrows)  Figs. B, 2B. Ths) Figs. C, D. Ones) Figs. C.Fig. 16C). On portal venous and delayed phase T1-weighted MR images, the two tumors were hypointense to liver  and discrete capsule enhancement was evident in one lesion (white arrows)  Fig. B–E.Fig. Histologically, the tumor was almost exclusively composed of epithelioid cells, with little fat and thick-walled blood vessels Figs. D, 4F. AlAML is an uncommon tumor that is composed of blood vessels and mature fat cells as well as smooth muscle cells. However, there are atypical AMLs composed almost exclusively of epithelioid cells with pronounced abnormal blood vessels and with less or no lipocytes. These atypical AMLs have been referred to as Epi-AML . The appThe imaging characteristics of HAML are correlated with its histological components. The fat content of typical HAMLs produces a characteristic appearance on imaging studies, thereby enabling the preoperative differentiation of typical HAMLs from other hepatic tumors . PreviouOn dynamic CT or MRI studies, all the tumors were markedly enhanced in the arterial phase, suggesting that Epi-AML is a hypervascular tumor. Most lesions (four) decreased obviously in the portal venous/delayed phase. These findings are compatible with hepatocellular carcinoma (HCC) . The disIn our study, discrete capsule enhancement was observed within one lesion on contrast MRI. According to the pathology specimens and the literature , there iAlthough typical AMLs were regarded as universally benign tumors and often grow slowly, it is becoming increasingly clear that Epi-AML should be regarded as tumors of uncertain malignant potential. Recently, rare cases of Epi-AML with tumor recurrence, and vascular invasion were reported , 13. SurIn summary, Epi-AML usually presents as a well-defined, low fat content and unencapsulated mass in an asymptomatic woman. In addition, hypervascularity with central punctiform or filiform vessels is a characteristic radiographic features for Epi-HAML. The enhancement pattern is divided into two types, lesions with abundant central vessels rapidly decreased and lesions with small or no vessels demonstrated prolonged enhancement in the portal venous/delayed phase.


Despite huge advances in medicine, glioblastoma multiforme (GBM) remains a highly lethal, fast-growing tumour that cannot be cured by currently available therapies. However, extracranial and extraneural dissemination of GBM is extremely rare, but is being recognised in different imaging studies. To date, the cause of the GBM metastatic spread still remains under discussion. It probably develops at the time of intracranial progression following a surgical procedure. According to other hypothesis, the metastases are a consequence of spontaneous tumour transdural extension or haematogenous dissemination. We present a case of a 59-year-old woman with symptomatic leptomeningeal and intramedullary metastases of GBM who has been previously surgically treated with primary subtotal resection and underwent a repeated surgery during adjuvant radiotherapy and chemotherapy with temozolomide. Today, the main goal of surgery and chemoradiotherapy is to prevent neurologic deterioration and improve health-related quality of life. With this paper, we want to present this rare entity and emphasise the importance of a multidisciplinary approach, a key function in the management of brain tumour patients. The prognosis is still very poor although prolongation of survival can be obtained. Finally, although rare, our case strongly suggests that clinicians should be familiar with the possibility of the extracranial spread of GBM because as treatment improvements provide better control of the primary tumour and improving survival, metastatic disease will be increasingly encountered. Glioblastoma multiforme (GBM) is the most common primary malignancy of the central nervous system (CNS) in adults. Macroscopically evident and symptomatic spinal metastases occur rarely, in up to 2% to 5% of patients ,2. Sprea2 per day) with regular daily administration of dexamethason 12 mg i.m. During chemoradiotherapy (after 24 of 30 fractions), she underwent an MRI examination due to exacerbation of the neurological signs and tumour progression was noted  continued with 16 Gy in eight fractions . Sequential chemotherapy with temozolomide (150 to 200 mg/m2 for 5 days during each 28-day cycle) was discontinued after two cycles because the patient complained of back pain and proximal weakness in both lower limbs with paraesthesia. MRI of the cervical spine demonstrated enhancing cervical leptomeningeal metastases on a level of C3 to C7. Additionally, on a T8 to T10 level, MRI also revealed intramedullary metastases with extensive contrast enhancement, central necrosis and vasogenic oedema  of the brain showed a lesion in the right frontal area. A right frontotemporal craniotomy for the mass resection was performed. Intraoperatively, a quite irregular and partially necrotic tumour mass was noted. The pathohistology analysis demonstrated marked nuclear pleomorphism, scattered mitoses and vascular endothelial proliferation with necrosis, consistent with the diagnosis of GBM  grade IV)  [Gliomas comprise a heterogeneous group of tumours that differ in location within the CNS, in age and sex distribution, in morphological features, in tendency to progression, and in response to surgical and oncological treatment. GBM remains the most aggressive of gliomas, a collection of tumours developing from glial tissue or their precursors, accounting for 12% to 15% of all intracranial neoplasms . Evidencary GBM) ,11. They2/day, given 7 days per week from the first day of radiotherapy up to the last day of radiotherapy, but for no longer than 49 days). After a 4-week break, patients were then to receive up to six cycles of temozolomide according to the standard 5-day schedule every 28 days. [With the exception of brainstem gliomas, GBM has the worst prognosis of any CNS malignancy, despite multimodality treatment. The current standard of care for glioblastoma is surgical resection of as much of the tumour as is safe and possible, followed by postoperative radiation therapy  and chemotherapy (temozolomide at a dose of 75 mg/m28 days. . To date28 days. ,14. Howe28 days. -23. Such28 days. ,25. The Notwithstanding all this, the treatment of GBM still remains one of the most disappointing challenges in modern oncology. A surgical cure for these brain tumours is virtually impossible to provide. The clinical course is determined by the biology of the tumour and response to surgery, radiation and chemotherapy . In our As mentioned earlier, spinal or systemic GBM metastases are very rare ,2. This The prognosis in cases of leptomeningeal metastasis is very poor, regardless of the treatment modality used. The survival of patients is approximately 2 to 3 months ,38. RadiAlthough merely a theoretical possibility in most cases, reoperation of GBM during adjuvant radiochemotherapy was still feasible in our patient. Today, the main objective of chemoradiotherapy in patients with metastatic GBM is to prevent neurologic deterioration and improve health-related quality of life . ResearcWritten informed consent was obtained from the patient to the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.3D-CRT: Three-dimensional conformal external beam radiotherapy; CNS: Central nervous system; GBM: Glioblastoma multiforme; Gy: Gray; KPS: Karnofsky performance status; MRI: Magnetic resonance imaging; WHO: World Health Organization.Each co-author certifies that she/he has no commercial interest that might constitute a conflict of interest in connection with the submitted article.JJG, FS and RSP were responsible for the writing. JP, MS and KZ participated in data collection. DK, AJ and SP participated in literature searching and helped draft the manuscript. All authors have read and approved the final manuscript.


Adrenal angiomyolipoma is rare. Only four cases have been reported so far. These are commonly found in Kidney but extrarenal sites are also mentioned. Angiomyolipoma arising in adrenal is very rare entity, usually asymptomatic, diagnosed incidentally on radiological investigation of abdomen for other conditions. We report our experience with a 45-year-old woman who presented with epigastric discomfort. A computerised tomography (CT) scan showed an adrenal mass. Laparoscopic adrenalectomy was performed and the histopathological features confirmed the diagnosis of adrenal angiomyolipoma. The patient recovered without any complications following surgery. Adrenal angiomyolipoma is rare. Only four cases have been reported so far. We report our experience with a 45-year-old female who presented with right subcostal pain. A computerised tomography (CT) scan showed a right adrenal mass with features of angiomyolipoma. Laparoscopic adrenalectomy was performed, and the histopathological features confirmed the diagnosis of adrenal angiomyolipoma. The patient recovered without any complications following surgery.Angiomyolipoma is apparently a part of a family of neoplasms that derive from perivascular epithelioid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon, and the most common extrarenal site is the liver .A 45-year-old female presented with epigastric discomfort off and on. Upper G.I Endoscopy was normal. Sonography for hepatobiliary system was normal but revealed a well-defined 5 × 6 cm mass in the retroperitoneum  . CT abdoLaboratory investigations, that is, Serum catecholamine, cortisol, and urinary, VMA were within normal limits. Exploratory laparoscopy revealed 5 × 5 × 4 cm mass, firm in consistency, quite separate from right kidney with no definable right adrenal gland. Right laparoscopic adrenalectomy was performed.On cut section mass was grey-white and non-homogeneous in texture. Histopathological examination revealed mature fat cells, smooth muscle fibres, and thin-walled blood vessels with peripherally compressed adrenal cortical tissue suggestive of angiomyolipoma of adrenal . The patBecause up to 52% of patients with angiomyolipomas larger than 4 cm are symptomatic and have an increased risk of bleeding, surgery or selective arterial embolization has been suggested in large angiomyolipomas [ In the last years, laparoscopic adrenalectomy has been recommended because it is less invasive, with lower mortality compared with open surgery. Management should be the same as that for any adrenal mass. Assessment of functional status of the tumor should be done. Surgery is indicated if the patient is symptomatic or the tumor is more than 5 cm since the risk of malignancy increases with size. Also, the risk of spontaneous rupture increases with size, owing to the presence of abundant and abnormal elastin-poor vascularity in the tumor. Laparoscopic adrenalectomy is an option and had been successfully done for a 5 cm adrenal angiomyolipoma , 4.


The term odontogenic keratocyst was first used by Philipsen in the year 1956. The lesion was renamed by him as keratocystic odontogenic tumor (KCOT) and reclassified as odontogenic neoplasm in the World Health Organization's 2005 edition that occurs commonly in the jaws having a predilection for the angle and ascending ramus of mandible. In contrast, KCOTs arising in the maxillary premolar region are relatively rare. Here, we discuss a rare case of keratocystic odontogenic tumor occurring in the maxilla with an ectopic tooth position. Keratocystic odontogenic tumor (KCOT) is defined as “a benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potential for aggressive, infiltrative behavior.”In 2005, the World Health Organization redefined the odontogenic keratocyst as a result of its biological behavior as a benign tumor of odontogenic origin and named it as keratocystic odontogenic tumor.KCOTs comprise approximately 11% of all cysts of the jaws. They occur most commonly in the mandible, especially in the posterior body and ramus regions. They almost always occur within bone, although a small number of cases of peripheral KCOT have been reported.A 17-year-old male patient came to the department with a chief complain of pus discharge from the right upper back teeth region 3-4 months ago with pain and swelling 15−20 days ago. Pain was gradual in onset, throbbing type, continuous, nonradiating, aggravates on mastication, and relieved on taking medication. Swelling was initially smaller in size and gradually increased to the present size associated with pus discharge from right upper back region 2-3 months ago. There was no history of trauma or fever. Patient also gave history of exfoliation of a tooth from right upper back tooth region on its own .On general physical examination, patient was found to be moderately built and nourished. He was conscious, cooperative, and well oriented with time, place, and person. Extraoral examination showed no abnormality.On intraoral examination, a localized, solitary swelling was present in the right upper back vestibular region measuring approximately 1–1.5 cm in diameter in relation to teeth 15, 16 with overlying mucosa slightly erythematous in appearance; however the surface appeared to be smooth and the surrounding area appeared normal. On palpations all inspectory findings were confirmed. The swelling was nontender, bony hard in consistency, nonfluctuant, nonpulsatile, noncompressible, and nonreducible. Hard tissue examination revealed missing 13, 14 and over retained 53. On percussions 15, 16 were tender. All other teeth were present in the oral cavity with no abnormality. On the basis of history and clinical examination, a provisional diagnosis of dentigerous cyst was given.Patient was further subjected to radiographic investigation where intraoral periapical radiograph , occlusaPanoramic radiograph revealed multilocular radiolucency separated with intermittent septa involving the right maxilla with impacted canine and first premolar. Displacement of second premolar is also seen.Fine needle aspiration of the cyst was done which revealed straw colored fluid intermixed with blood . The samSurgical excision of the lesion was done and was sent for histopathological confirmation. Histopathologic features reveal cystic lining with parakeratinized, corrugated stratified squamous epithelium exhibiting palisading basal cells, nuclear hyperchromatism, and flattened epithelial connective tissue junction at places. The underlying connective tissue capsule is fibrillar with admixed population of acute and chronic inflammatory cells at places, blood vessels, and peripheral bone. Features are suggestive of keratocystic odontogenic tumor Figures .Keratocystic odontogenic tumor (KCOT), formerly known as OKC, is a benign unicystic or multicystic intraosseous neoplasm of odontogenic origin which arises from the remnants of the dental lamina both in mandible and maxilla .The discovery of increased mitotic activity in the cyst epithelium, the potential for epithelial budding from basal layer or daughter cysts in the cyst wall, the presence of chromosomal abnormalities, and the role of mutation of the PTCH gene in the etiology of KCOT resulted in its reclassification and renaming as keratocystic odontogenic tumor –6.Keratocystic odontogenic tumors are sometimes related to nevoid basal cell carcinoma syndrome, which is a rare inheritance disorder caused by mutations in the PTCH gene on chromosome 9 causing multiple odontogenic keratocyst of the jaws, basal cell carcinoma (BCC) of the skin, and vertebral anomalies .According to Madras and Lapointe three factors led to the recharacterization of the keratocyst as KCOT. The KCOT exhibits locally destructive and highly recurrent behavior; the histopathology of the KCOT reveals budding of the basal layer into the connective tissue and frequent mitotic figures, and, finally, the KCOTs are associated with an inactivation of PCHT, the tumor suppressor gene .KCOT has a predilection for occurring in the mandible (75.58%) as compared to maxilla –12. In mKCOT when involving the maxilla sinus must be carefully assessed because the orbital damage and the spreading of associated infections could lead to local and systemic compromise to the patient, present with pain. Displacement of tooth and destruction of the floor of the orbit and proptosis of eyeball when it involves maxilla are most common features .The ectopic eruption of teeth in the regions other than the oral cavity is rare, although there have been reports of teeth in unusual locations, one of them being the maxillary sinus. The etiology of ectopic eruption has not yet been completely clarified but may occur as a result of trauma, infection, developmental anomalies, and pathologic condition such as odontogenic cysts. As the growth of an odontogenic cyst continues, the cyst encroaches on the space of the sinus and displaces its borders: it may be that the displacement of teeth buds by this expansion of a cyst results in the ectopic eruption of a tooth .KCOT is more common in males than females and occurs over a wide age range and is typically diagnosed during second to fourth decade .On radiographic examination, KCOT cannot be distinguished from other intrabony cysts. In mandible, the epicenter is commonly located superior to the inferior alveolar nerve canal. It usually shows evidence of a cortical border with a scalloped outline which represents variation in the growth pattern of the cyst . An impoa parakeratinized surface which is typically corrugated, rippled, or wrinkled;uniformity of thickness of epithelium ranging from 6 to 10 cells thick;a prominent palisaded, polarized basal cell layer of cells having “picket fence” or “tomb stone” appearance.Numerous surgical modalities have been suggested for the treatment of KCOTs, including enucleation with primary closure, enucleation with open packing, and resection with or without loss of jaw continuity. The treatment depends on several factors, such as age, location, and size of lesion and whether the lesion is primary or recurrent. Total enucleation with or without “peripheral ostectomy” is the treatment of choice for most KCOTs unless lesion is recurrent or has significantly invaded soft tissue [The keratocystic odontogenic tumor wall is usually thin unless there has been a superimposed inflammation , 13, 14.t tissue , 17.The destructive, high recurrence potential of KCOTs and their ability to resemble other jaw cysts make it important to consider them in differential diagnosis of radiolucent lesions occurring in the maxilla. Also due to aggressive behavior and high recurrence rate of KCOT, all pathologic tissue should be properly excised and histopathologic confirmation should be made for a definitive diagnosis. The followup is advised for every six months for the next two years.


The incidence of ureteral and bladder lesions after laparoscopic hysterectomy is the most encountered urinary complication in gynaecological surgery. We report the unusual case of 42-year-old woman who had a delayed diagnosis of bilateral ureteral injury associated with bladder lesion and loose of vaginal suture after undergoing laparoscopic hysterectomy for uterine adenomyosis. The incidence of ureteral and bladder lesions after hysterectomy is 0.3% to 4.3% , 2. SuchA 42-year-old woman, with no medical history, was admitted to department of gynaecology after suffering from chronic pelvic pain, metrorrhagia, and dyspareunia for several months. Uterine adenomyosis was diagnosed on magnetic resonance imaging (MRI) of the pelvis. A laparoscopic hysterectomy was performed with no intraoperative complications or surgery-related technical problems. At the close of the procedure, the urinary catheter drained clear urine. It was removed on postoperative day 2. The patient recovered spontaneous micturition but complained of urinary incontinence on day 3. She was discharged on day 4 . On postoperative day 8, the patient was readmitted with abdominal pain, inability to pass stools and gas, and vomiting. She complained of vaginal loss and persistent urinary incontinence but had no fever. The clinical examination was suggestive of bowel obstruction, with muscle contraction on abdominal palpation. The surgical scars were healthy. Laboratory findings were leucocytosis, 11.5 g/L; C reactive protein, 12 mg/L; and creatinine, 65 micromoles/L. A computed tomography (CT) scan of the abdomen and pelvis, with injection of contrast agent, revealed bilateral hypotonia of the pyelocaliceal cavities, dilatation of the right lumbar ureter, a full bladder, peritoneal effusion (fluid collection of 45 and 35 mm), and small bowel dilatation with no mechanical obstruction . The obsInitial management was conservative on the gastrointestinal surgeon's advice. A gastric catheter was placed in the fasting patient; 1.5 L were collected in 24 hours. The patient had dysuria. Kidney function was impaired . Fever and persistent contraction on abdominal palpation prompted an exploratory examination on day 17, which revealed peritonitis; diffuse adhesions of the ileum, and intraperitoneal effusion of a fluid resembling urine. A 1.5 × 4 cm inflammatory lesion of the Douglas pouch was excised and, on pathological examination, proved to be due to endometriosis and an abscess. The vaginal vault was open and had required closure by 2 stitches. The abdominal cavity was drained with a Shirley sump drain. The urinary catheter drained no urine during the first hours following the intervention, but increasing the intravenous infusion and injection of diuretic led to the appearance of urine in the Shirley drain. The urologist on duty requested a methylene blue test, which proved positive for the vagina and Shirley drain. An abdomen and pelvis CT scan confirmed bilateral ureteral injury .In the absence of pyelocaliceal cavity dilatation, we did not perform percutaneous bilateral nephrostomy but attempted insertion of ureteral endoprostheses after bilateral ureteropyelography. Cystoscopy revealed that the posterior bladder wall was perforated 3 cm above the intertrigonal ridge. Retrograde ureteropyelography confirmed bilateral ureteral injury . We wereThe patient's clinical condition warranted a second laparotomy. Surgical exploration revealed partial ligature and iatrogenic transection of the ureters at the level of the uterine arteries. The ureters leaked. The discovery of adnexal ischemia and ovarian necrosis prompted a left ovariectomy. A Politano-Leadbetter bilateral ureter reimplantation was performed after suture of the bladder breach. Hyperdiuresis (6 L/24 hours) on postoperative day 1 was followed by a favourable postoperative clinical course. Normal intestinal transit was recovered on day 5. Creatinine level was 70 micromoles/L. Cystography showed minimal posterior leakage on day 15 and none on day 25. The ureteral endoprostheses were removed during postoperative month 2. Pathology revealed a normal urothelium with acute inflammation and necrosis at the ureteral margins, and inflammation and acute ischemia of the left ovary. Two years after surgery, the patient was symptom-free. The abdomen and pelvis CT scan and urologic parameters were normal. Creatinine was 68 micromoles/L.To our knowledge, late diagnosis of bilateral iatrogenic ureteral injury and injury to the bladder during laparoscopic hysterectomy has not been reported. Diagnosis was delayed in our patient because of leakage leading to partial conservation of micturition. Urine loss via the vagina was due to a loose vaginal suture. A urinoma had formed because the bladder breach and vaginal opening did not allow complete continuous urine evacuation; the urethrocutaneous fistula was formed after vaginal wound closure and catheter placement.Such dual injury should be routinely sought when faced with an acute abdominal syndrome after laparoscopic hysterectomy. The diagnosis should be established by the methylene blue test, a CT scan, and/or retrograde urography, and the placement of a double-J catheter should be attempted. In the event of pyelocaliceal cavity dilatation, nephrostomy will be preferred. Bilateral ureteral reimplantation with bladder suture is feasible and represents standard first-line management after failure of a minimally invasive procedure or after renal drainage. Omental wrapping can also be used to effectively treat ureteral injury , 8. DuriA recent published study by Janssen in 37 patients during a 20-year period  have shoThe urinary tract injury should be investigated in case of acute abdominal syndrome after laparoscopic hysterectomy even occurring late.


The aim of this paper is to present clinicopathologic features and immunohistochemical findings of a primary leiomyosarcoma of bone occurring in the proximal femur of a 46-year-old Caucasian male patient. Each case report on this exceedingly rare entity contributes to the notion of this disease. Sarcomas comprise a heterogeneous group of mesenchymal neoplasm. The vast majority of so-called smooth-muscle tumors arise in the uterus, gastrointestinal tract, mesentery, and omentum . LeiomyoFor nonretroperitoneal soft tissue leiomyosarcomas, local recurrences and metastases usually occur within the first few years after initial diagnosis and commonly metastasize to the lungs. Prognosis depends on the location, stage, and grade of the primary tumor as well as the presence of metastatic disease .Herein we present clinicopathologic features and immunohistochemical findings of a primary leiomyosarcoma of bone occurring in the proximal femur of a 46-year-old Caucasian male patient.A 46-year-old Caucasian man was admitted to our department due to pain in his right proximal thigh. Medical history revealed no contributory family and past histories, and no neurological deficits were detected by physical examination.X-rays and MRI presented an expanding 10 cm lytic lesion with an inhomogeneous contrast medium enhancement occupying the right proximal femur and extended into the proximal shaft . The lesBiopsy specimen revealed a malignant mesenchymal fascicular spindle cell proliferation with smooth-muscle differentiation reflected by the expression of a-SMA (a-smooth-muscle-actin), desmin, and caldesmon by immunohistochemistry. The patient underwent limb salvage surgery followed by endoprosthetic replacement . Wide suThe explant consisted of a 25 cm long proximal femur  with a 1 The histological evaluation revealed a malignant fascicular spindle cell proliferation with an infiltrative growth pattern composed of interweaving fascicles. The tumor cells were spindle shaped, elongated, blunt ended, hyperchromatic, focally pleomorphic, and surrounded by a prominent eosinophilic cytoplasm . With an After one year of followup, a successfully performed one-stage revision surgery became indispensable due to a late-onset infection of the primary prosthesis. For more than 9 months of followup after first surgery no evidence of metastatic disease or local recurrence was seen. Although two 2 mm lesions were detected in both lungs by soft tissue scans the lesions did not show any progression during the following 16 month of followup.Primary leiomyosarcoma of bone is exceedingly rare, accounting for less than 0.7% of all primary malignant bone tumors . It mostAs primary leiomyosarcoma of bone is exceptionally rare we considered the following differential diagnoses. First of all skeletal metastases from a primary leiomyosarcoma located in the gastrointestinal tract or soft tissues were excluded by CT scans and scintigraphy using the diagnostic guidelines described by Antonescu et al. .In addition low-grade intramedullary osteosarcoma, fibrosarcoma of bone, and metastases of a spindle cell carcinoma were excluded by means of morphological analysis and immunohistochemistry.Based on a 0.2 mm area of cartilaginous differentiation a dedifferentiated chondrosarcoma (DCS) was considered as another differential diagnosis. DCS is defined by the presence of a well-differentiated cartilaginous component with abrupt transition in a dedifferentiated tumor component, which may have features of pleomorphic sarcoma, NOS , and exceptional rarely leiomyosarcoma . TypicalApproximately 90% of all patients with DCS have a poor prognosis and die thus due to metastatic disease within the first two years after diagnosis , 12–14. The age of our patient, the radiologic appearance  as well as the fact that the 0.2 mm area with cartilaginous differentiation corresponded to the area of the previous biopsy side prompted us to consider the cartilaginous component as reactive, and therefore, the differential diagnosis DCS was rejected.We herein report clinicopathologic features and immunohistochemical findings of a primary leiomyosarcoma of bone located in the proximal femur of a 46-year-old Caucasian male patient. Each case report on this exceedingly rare entity contributes to the notion of this disease. As the reported case shows, clinicians should be aware of leiomyosarcoma of bone as differential diagnosis.


Balo's concentric multiple scleroses (MS) is a rare demyelinating disease and a variant of multiple sclerosis. We report a case with interesting misleading clinical history and typical RI findings of Balo disease. A 19-year-old girl presented with fever and left hemiparesis following dental procedure 15 days ago. On physical examination fever and left limbs forces loss were noted. On CT scan a hypodense mass like area, and in MRI a low T1 and high T2, and flair concentric onion-like partial enhancement of mass in parieto-frontal periventricular white matter was detected. Regarding the findings tumefactive MS, Low grade glioma, lymphoma, and abscess and regarding the history of abscess formation were in differential diagnosis. After therapy and no improvement in clinical condition and MRI findings during 2 months, the patient underwent stereotactic biopsy and tumefactive balo MS was revealed. After 4 months of intensive therapy, the patient was discharged with normal condition. Balo's concentric sclerosis (BCS) is a tumefactive demyelinating lesion of the central nervous system (CNS) and considered a rare variant of multiple sclerosis (MS).1The clinical course is characterized by an acute onset and steady progression to major disability within a few months. Age of onset is between 20–50 years, and patients predominantly present with cerebral symptoms. Classical manifestations of the disease are headache, aphasia, cognitive or behavioral dysfunction, and/or seizures. Most patients are young adults in their second to fifth decades of life, and it is relatively more common in males.2BCS is delineated pathologically by huge, tumor-like brain lesions showing concentric rings of alternating demyelination and preserved myelin layers which involve the cerebral hemispheres, cerebellum, brain stem, spinal cord, and optic chiasm.3 Diagnosis is based on clinical signs and symptoms consistent with BCS, exclusion of other neurologic diseases, and the characteristic concentric rings on MR images. In the present case report, we report the case of a 19-year-old female with BCS involving right parieto-frontal periventricular white matter, with typical MRI appearance but interesting misleading clinical history.Magnetic resonance imaging (MRI) associated with BCS demonstrates concentric rings on T2-weighted and contrast-enhanced T1-weighted images, and thus helps differentiation from other pathology. T2-weighted MRI scans sometimes reveal a distinct pattern of hypo-/isointense and hyperintense rings corresponding to bands of preserved and destroyed myelin.A 19-year-old girl referred with a history of decrease in left limbs forces, muscular tones, and fever following dental procedure from 15 days ago. Urgent plain computed tomography of brain revealed a hypodense mass-like area in the right parieto-frontal periventricular white matter. Brain MRI revealed a tumefactive lesion in the right parieto-frontal periventricular white matter with alternating hypo-isointense and hyperintense concentric onion-like bands on T1- and T2-weighted and FLAIR, and partial enhancement in post-contrast images in parieto-frontal periventricular white matter with mild edema and no hemorrhage and restriction .According to history and imaging findings tumefactive MS, Low grade glioma, and lymphoma was our differential diagnosis; however, only regarding the patient's history, abscess formation was the most possible diagnosis. The patient received intravenous antibiotic and modified steroid therapy. No improvement was observed in clinical conditions or MRI findings during 2 months. The patient underwent stereotactic biopsy to differentiate probable tumoral lesion from abscess or tumefactive MS. Biopsy revealed Tumefactive Balo MS. After 4 months of intensive therapy, the patient was discharged in a good condition. Our patient did not experience any relapse, and at the time of the last follow-up examination a year after starting treatment, she had only residual mild left hemiparesis.4 In our case, concentric pattern on all MR sequences and partial enhancement of mass lesion on post-gadolinium scan were seen. The exact reason for BCS lesion formation is not clear. Recently, it has been proposed that demyelination in these lesions are similar to hypoxic-like tissue injury.BCS is considered as a rare variant of inflammatory demyelinating disorder that presents the characteristic concentric area of alternating demyelination and myelination on pathologic examination with an apparent basic pathologic similarity to multiple sclerosis. However, the exception of an alternating lamellar pattern of demyelinated and well-preserved white matter which encompass alternating bands of low and high signal in white matter were seen on T2-weighted images. The presence of concentric ring enhancement was also observed on post contrast T1-weighted images. Edema and mass effect may also be seen.2Clinical differential diagnosis includes acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), neoplasms, and infections such as abscesses. However, the typical concentric pattern on MR images is highly suggestive of BCS. The concentric pattern is not always observed if the MR imaging is not performed early in the course of the disease.4A brain biopsy should then be considered to exclude other disorders. In histopathological investigation, it is important to stain for myelin to determine alternating concentric zones of demyelination.In our patient, in regard to history, Imaging findings, and the clinical process following primary therapy (no response in our case), tumefactive MS, neoplasia lesion, and infection were in differential diagnosis.2 In conclusion, BCS disease can mimic space-occupying lesions in the central nervous system with similar clinical and radiological features.Corticosteroids at high dosage are the first line of therapy and have been shown to be effective in recovery from neurological deficits related to BCS. Our patient had a good response to intensive steroid therapy during the 4 months after starting of treatment. Often, steroids do not alter the acute attacks of CNS demyelination, but cycles of plasmapheresis have been reported to be useful. Some cases have been treated successfully with immunosuppressive drugs, such as mitoxantrone.


Peutz-Jeghers syndrome (PJS) is an hereditary syndrome characterized by gastrointestinal polyposis and mucocutaneous pigmentation. PJS patients are at increased risk of developing various cancers, especially of the gastrointestinal and gynaecological tracts. Colonic adenocarcinoma is one of the more common tumours that occur in PJS. We report a young lady presenting with a large ovarian tumour, later diagnosed to have PJS with colonic signet cell adenocarcinoma and synchronous ovarian metastasis. Peutz-Jeghers syndrome (PJS) was first described by Peutz in 1921. It is characterized by mucocutanous melaninosis and gastrointestinal polyposis. Melaninosis occurs on the lips, buccal mucosa, fingers, nose, and perioral areas. Gastrointestinal polyps occur anywhere from the stomach to the colon in varying numbers. The clinical presentation of PJS is generally recurrent abdominal pain due to intussusception and anaemia due to occult or overt gastrointestinal bleeding . We desc3/L, platelets 362 × 103/L, normal electrolytes and renal, hepatic and thyroid function tests. Tumour marker values were as follows: Beta hCG <0.10 IU/mL, AFP 1.01 IU/mL, CEA 25.25 ng/mL, and CA125 19.5 U/mL. Stool occult blood was positive.This 32-year-old female presented with history of recurrent bouts of dyspepsia over the preceding two years. There was no history of associated upper or lower gastrointestinal bleeding, abdominal pain, jaundice or menstrual irregularities. She had undergone right oophorectomy 12 years previously for an ovarian mucinous cystadenoma, the details of which could not be traced. Her brother had had history of surgery for intussusception. Physical examination revealed mucocutaneous pigmentation over the lips, buccal mucosa A and finWith a high clinical suspicion of PJS, we proceeded with an upper gastrointestinal endoscopy, which revealed multiple small polyps in the stomach B and larPJS is an autosomal dominant disorder caused by germ-line mutations in the STK11/LKB1 on chromosome 19p13.3 . ClinicaOur patient presented in her third decade with symptoms of dyspepsia for two years. Because she lived in a backward, tribal belt of Kerala, frequent commuting to hospital was difficult and hence her symptoms were never fully evaluated at a major hospital or an institution. Ovarian mass was the striking feature on clinical examination. Characteristic pigmentation, family history of intussusception and evidence of gastrointestinal polyposis led to the diagnosis of PJS. Elevated CEA levels and CT findings were suggestive of colonic malignancy. Left ovarian mass lesion and left ureteric involvement were thought to be due to metastasis. There was diffuse peritoneal involvement and peritoneal cancer index before and after surgery was 24 and 0, respectively. As there was evidence of obstructing colonic lesion, extended left hemicolectomy was performed, followed by cytoreductive surgery. Even though the resected margins of the colon and ureter were free from tumour, the ascitic fluid cytology was positive for malignancy and microscopic disease could not be completely excluded in grossly normal areas, hence R0 resection could not be achieved. However, optimal cytoreduction to no macroscopically residual disease was performed.Abdominal distension with dyspeptic symptoms was the presenting feature in 46% of patients with non-genital cancers metastasizing to the ovaries . The mosPathology was consistent with Peutz-Jeghers polyp and signet cell adenocarcinoma of the colon and ovary. Krukenberg tumour is a metastatic signet-cell adenocarcinoma of the ovary with the primary being stomach 70%), colon or the appendix. A preceding diagnosis of primary tumour is obtained in only 25% of cases. These tumours are bilateral in 80% of cases and the surface of the ovary is usually free from adhesion and deposits . Our pat0%, colonPJS usually presents with benign complications like intussusception and gastrointestinal bleeding. Colorectal malignancy is one of the most common and serious complications in these patients. As in our case, patients can rarely present in the late stages of malignancy and seek the care of the gynaecologist, rather than the gastroenterologist. Two years of delay in seeking appropriate care resulted in our patient presenting with an uncommon initial presentation of a common malignancy affecting patients with a rare underlying disorder. General practitioners need to be aware and refer patients with refractory dyspeptic symptoms—even in young individuals—for timely intervention.Conflict of interest: none declared.


We report a rare case of primary vulval rhabdoid tumor in an adult. The diagnosis was confirmed using the recently emerging INI1/BAF47 immunostain. We also demonstrate the expression of ER and PR hormonal receptors by the tumor cells. Extrarenal malignant rhabdoid tumors (EMRTs) are rare and highly aggressive neoplasms. Although they are known to occur during infancy and childhood, their occurrence in adults is reported. EMRTs were described in various anatomical sites, and the use of INI1 immunostain has recently emerged as an important adjunct for identifying these tumors. An extensive online search of the literature revealed very few reported cases of EMRT of the vulva . We hereA 25-year-old Saudi female, who is medically free, presented to our emergency department complaining of a mass in the mons pubis. The mass was occasionally tender and had rapidly increased in size in two-week duration. No other complains or masses at other sites were reported. On clinical examination, an ill-defined soft mass was identified in the mons pubis, extending to the left inguinal area with a hyperemic overlying skin. Laboratory tests were within normal limits except for a mild leukocytosis. The clinical impression was of an abscess, and the patient was scheduled for surgery. A local excision of the mass was done, and the specimen was sent to the histopathology department. The patient was discharged on the next day of surgery. Grossly, the specimen consisted of two irregular pieces of soft tissue. Both masses were partially encapsulated and measured 6 × 5 × 2 cm and 6.5 × 6 × 2.5 cm, respectively. The outer surface of both masses was irregular. Serial slicing of the masses revealed a heterogeneous cut surface with pale white and dark tan areas. Random sections from both masses were taken for histopathological examination.H&E slides showed a poorly differentiated neoplasm. The tumor consisted exclusively of cells retaining the classical “rhabdoid” morphology . The celAn extended panel of antibodies was performed on Ventana using the iVIEW DAB detection kit and showed focal cytoplasmic immunoreactivity to vimentin  with the characteristic globular cytoplasmic configuration indenting the nucleus. The cytoplasm of tumor cells also coexpressed cytokeratin cocktail , CK 8 , and CK 19 . The tumor cells, in comparison to the endothelial lining of the capillaries, showed loss of INI1  nuclear staining . Anti-KiA diagnosis of malignant rhabdoid tumor of the vulva was established. The patient was recalled once the histopathology report was signed out. A computed tomography scan (CT) of the head, chest, abdomen, and pelvis was performed and revealed no other tumor masses. Unfortunately, the patient failed to show for followup.We report a case of EMRT of the vulva in a 25-year-old female. The age of the patient, the site of the tumor, the clinical presentation, and the microscopic findings in our case were compatible with the 12 previously  reported cases  1–10]. . 10]. ThMalignant rhabdoid tumor was originally described in the kidney as a variant of Wilms' tumor. In 1982, the first extrarenal malignant rhabdoid tumor was reported, and since then this entity was recognized at various anatomical sites including soft tissues, liver, prostate, thymus, skin, pelvis, heart, and central nervous system , 15. TheIn summary, vulval EMRTs are rare and aggressive neoplasms and should be considered in the differential diagnosis of any high-grade undifferentiated tumor at this site. Given that IHC is much more widely available than molecular tests, INI1/BAF47 immunostain represents the most useful, up to date ancillary technique to confirm this diagnosis. Although these tumors express ER and PR receptors, their response to antihormonal therapy is a remote possibility .


Ewing sarcoma (ES) is a high-grade malignant primary round cell tumour of bone in which there is commonly extension into extraosseous soft tissues at the time of diagnosis. This report details the clinical, radiological and pathological features of a case of ES of the tibia in which there was extensive osseous involvement but no infiltration beyond the periosteum into surrounding soft tissue. We also record the findings of one other ES case that exhibited similar behaviour. Both cases were male, involved the tibia and had the characteristic t   translocation. No recurrence of tumour or metastasis has been seen in these two cases, both of which have had 6 years follow-up. Our findings indicate that there is heterogeneity in the behaviour of ES and show that localized ES is associated with a good prognosis. EWS gene and the FLI-1 gene [Ewing sarcoma (ES) is a malignant tumour of bone which is composed of small round tumour cells ,2. It acI-1 gene .ES is highly aggressive tumour which grows rapidly, causing extensive destruction of cancellous and cortical bone. In long bones, the tumour commonly involves the diaphysis and metaphysis with the epiphysis affected in only 2% of cases; radiologically, there is extensive permeative or moth eaten bone destruction and a soft tissue mass is seen in approximately 90% of cases at the time of diagnosis . This reA 36-year old male presented with a 5 year history of left sided shin pain to his general practitioner. A plain radiograph taken at the time showed no bone or soft tissue abnormality. He presented again a few times with recurrent left leg and forefoot discomfort, particularly on exercise, before, 4 years later, complaining of more persistent severe shin pain, including at night. On clinical examination there was no bone or soft tissue swelling of the left leg. There was no other significant medical history and the patient was otherwise well. Haematological and biochemical investigations were normal, including white cell count, ESR and CRP.Plain radiographs taken at this time showed a large expansile permeative lytic lesion involving the proximal half of tumour of the left tibial diaphysis Figure . MRI demHistopathology of a biopsy of the lesion showed a solid proliferation of tumour cells with plump cytoplasm and round vesicular or hyperchromatic nuclei Figure C. ScatteRadiological and histological features indicated that this was an aggressive small round cell tumour that appeared to be confined to bone. The presence of glycogen-containing CD99+ cells pointed to a diagnosis of ES . MoleculThe resected segment of tibia was grossly expanded and had a thickened cortex. Cut surface revealed a large haemorrhagic tumour 18x4x4cm which filled the medulla of the tibial shaft and metaphysis Figure A; the tuTaken together, the morphological, immunophenotypic and molecular genetic findings indicated this was a case of ES which unusually appeared to be effectively confined to the bone with no periosteal or extraosseous soft tissue involvement. The patient recovered uneventfully from the operation. Following surgery he underwent adjuvant chemotherapy includes six cycles of VIDT . In addition, he had radiotherapy to the left leg, 45 Gy in 25 fractions with boost to the superior and inferior margins to 54.5 Gy (9 Gy in 5 fractions). He made a good functional recovery but developed a proximal non-union of the vascularised fibular graft 18 months after initial surgery, requiring re-excision of the segment of non-union and refixation with reconstruction using an Ilizarov frame. He has been regularly followed-up for 6 years and has shown no evidence of recurrence or development of metastasis.The above case of ES was widely discussed with pathology, oncology and surgical colleagues, one of whom (UD) informed us of one other case of ES of the tibia arising in a 16 year old male that had somewhat similar radiological features with extensive bone but limited soft tissue involvement. Radiological features of this case are shown in Figure The two cases of ES documented in this report are unusual in that they show extensive tumour involvement of the tibial bone but limited (Case 2) or no (Case 1) spread of tumour into surrounding soft tissues. Both case 1, which was not treated prior to surgical resection, and case 2, which did receive preoperative chemotherapy, have not shown evidence of recurrence or metastasis after 6 years follow-up; the localized nature of ES in these cases, particularly the limited involvement of extraosseous soft tissue is likely to be significant in regard to this good prognostic outcome.Most patients with ES present with pain and swelling of the affected bone and surrounding soft tissues. In case 1, there was no clinical evidence of swelling but bone pain had been noted several years prior to diagnosis. No tumour was identified on a plain X-ray when the patient first complained of bone pain. It was only 4 years later when the patient was again investigated that ES was identified. Biochemical and haematology investigations were normal over this 4 year period. Imaging studies showed a large permeative tumour in which there was erosion of cortical bone but no evidence of a significant soft tissue mass or prominent periosteal reaction; the latter is classically onion skinning in type in ES . Case 2 There are a few reports of relatively low-grade behaviour of ES, most of which represent rare examples of ES where the tumour is localized to bone and exhibits radiological appearances suggestive of a benign lesion, such as fibrous dysplasia or a simple cyst of bone -8. In onSeveral clinical and pathological features have been shown to have prognostic value in ES -13. In gThe previously reported cases of ES developing slowly over a period of years, taken with the two cases presented in this report, suggest that there is heterogeneity in the growth pattern of ES with some tumours exhibiting a relatively long intraosseous phase; several of these reported cases were in the tibia ,7. EarlyThe authors declare that they have no competing interests.All authors read and approved the final manuscript.


Mucosal atrophy as a potential cause of impaired colonic compliance has not yet been described as a complication in Collagenous Colitis (CC).We present a 51-year-old female patient with a 20-year history of diarrhea and diagnosed with CC ten years prior to her presentation. We reviewed reports from three colonoscopies performed after the diagnosis. Overall 12 biopsies obtained in the last two colonoscopies were re-analyzed by two pathologists blinded to the aim of the study. Besides the typical histological findings of CC, the endoscopic appearance was normal, and no histological signs of atrophy were found during the first colonoscopy. Surprisingly, the second and third colonoscopy revealed a region of advanced segmental mucosal atrophy in the cecum with the mucosal height normalizing toward the transverse colon. This pattern of atrophy was inversely related to the pattern of sub-epithelial collagen deposition, which increased toward the rectum.If no chance occurrence, our observation supports the idea that additional factors, probably luminal in nature, may be co-responsible for the mucosal atrophy in this case. Thus, mucosal atrophy in the proximal colon appears to be a new candidate among the growing list of rare complications associated with long standing CC. Collagenous colitis (CC) is a chronic inflammatory bowel disease characterized by chronic watery, non-bloody diarrhea in the context of macroscopically normal appearing colonic mucosa on endoscopic examination. CC was first described in 1976 . The dis2) had remained constant during the prior months. The physical examination revealed no further specific findings. Routine laboratory tests were within normal values with the exception of decreased serum ferritin levels measured at 9.1 ng/ml . The diagnosis of CC had been established 10 years prior in 1998 at our center. At the time of diagnosis, the patient's weight was 43 kg (BMI: 16.2 kg/m2). She had a history of radioiodine therapy for hyperthyroidism. Three years prior, invasive tubulo-ductal breast cancer  was diagnosed in her left breast, and a breast saving R0 resection was performed. Postoperative radiotherapy followed by anti-estrogen therapy with leuporelin and tamoxifen was delivered. Family history was notable for one of her three sisters who had developed Crohn's disease. After the diagnosis of CC was made, she was initially treated with Plantago ovata. In 2002, the patient's weight decreased again by 4 kg to 44 kg (BMI: 16.6 kg/m2). One year later, in 2003, a diagnosis CC was corroborated by endoscopic findings at another center and treated with budesonide for two years, resulting in a weight gain of approximately 4 kg. Budesonide was then gradually switched to mesalamine due to the development of osteoporosis. The presence of celiac disease was excluded with normal duodenal biopsies and negative serologic testing. During her inpatient rehabilitation, the third colonoscopy was performed. Parts of the endoscopic and histologic results are shown in Figures A 51-year-old female with a 20-year history of chronic diarrhea was admitted to our gastroenterological rehabilitation center in 2008. Her stool consistency was described as pulpy, and neither blood nor mucus was reported. Her weight (body mass index (BMI): 19 kg/mWe have described a patient with greater than a 10-year history of CC who developed segmental mucosal atrophy involving the ascending colon while sparing the terminal ileum and the remaining segments of the colon. Although, we were not able to obtain a clear-cut and internationally accepted definition of mucosal atrophy, the presence of advanced atrophy in the cecal region of our patient is beyond any reasonable doubt Figure . HoweverTo our knowledge, such a development of advanced colonic mucosal atrophy has not been documented in the CC literature thus far . Prior tAs expected the number of intraepithelial lymphocytes (IELs) was increased in all parts of the colon Figure . NotablyA chance occurrence would be another explanation for the presence of mucosal atrophy with CC in our patient. This type of bias is always relevant for case reports, especially if findings that display a high prevalence are reported in patients with rare diseases. However, after excluding patients with inflammatory bowel disease, the population based prevalences of CC and advanced mucosal atrophy of the proximal colon are comparatively low . Thus, tConsidering that the histopathological abnormalities in CC are believed to be most prevalent in the proximal colon and that the sigmoid colon and rectum may be spared in up to 73% of cases,  it has bOur observation introduces the mucosal atrophy of the proximal colon as a novel candidate complication that may be associated with long-standing CC.Written informed consent was obtained from the patient to publish this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CC: collagenous colitis; BMI: body mass index; IELs: intraepithelial lymphocytesThe authors declare that they have no competing interests.MM wrote the pathology aspects of the manuscript, provided important input for the interpretation of the histological findings and helped select the histologic specimens that were appropriate for presentation. SW cared for the patient, performed the third colonoscopy, collected the clinical data and prepared the initial presentation of the case. BB prepared the histologic samples and the histologic photographs in Figure The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/11/114/prepub


Mixed adenoneuroendocrine carcinoma (MANEC) carcinomas rarely occur in the gallbladder. Here we reported a case of giant gallbladder unresectable mass with local liver invasion and omentum metastasis, which proved to be neuroendocrine carcinoma (NEC) by biopsy, received successful radical operation after neo-adjuvant chemotherapy plus somatostatin treatment. The patient showed good response as the neoplasm diminished dramatically and showed clear margin after 6 courses of treatment. A radical operation including cholecystectomy, hepatic wedge resection of the gallbladder fossa segment and lymph node of group 8a and 8p resection was performed successfully. Postoperative histopathological examination revealed neuroendocrine carcinoma mixed with adenocarcinoma in the gallbladder wall. Followed up showed no evidence of recurrence after 7 months of the operation. We suggest that neo-adjuvant chemotherapy may be beneficial to gallbladder mixed neuroendocrine carcinomas in an advanced stage which could also be advantageous to NEC of other organs.http://www.diagnosticpathology.diagnomx.eu/vs/2731892837743787 Neuroendocrine tumors are uncommon diseases which mostly occur in the gastrointestinal tract, pancreas and lung. Epidemiology data from several medical centers in western countries indicate the incidence of NETs is about 2.5-5 /100,000 people. NETs occur in the gallbladder are rather rare and only account for 0.5% of all NETs  as well A 55-year-old woman complained of mild epigastric discomfort was found to have a large impalpable mass in the gallbladder area with ultrasound examination. She was then admitted to the first affiliated hospital of Sun Yat-sen University. She had history of chronic cholecystitis for five years and no operation or other chronic diseases were found. Physical examination was negative. A slight impairment of liver function was found with AST 57 unit/L and ALT 68 unit/L  while albumin and bilirubin were within the normal range. Blood and urine analysis were normal. Her serum tumor markers CEA was 43 ug/L , CA 125 465 U/ml  and CA 19–9 100 U/ml , all of which showed a significant elevation. Blood hormone test showed her serum CgA increased to 220 ng/ml , while 5-HIAA, 5-HT and cortisol level were normal. Abdominal ultrasound examination revealed massive ascites together with a mass measuring 14 cm×8.4 cm in the gallbladder bed area closely adhering to the pancreatic head and liver. CT scan suggested that the gallbladder neoplasm invaded the neighboring liver, pancreatic head, peritoneum and omentum . Tumor cells tend to be located around the small vessels in some area forming pseudorosettes  based discussion. The chemotherapy regiment is combination of Carboplatin 300 mg/mIntra-operative findings showed a hard mass measuring 7 cm×3 cm×2 cm in the gallbladder fossa, with clear margin to the pancreas. Palpable lymph nodes were found in the hepatodudenal ligament (group 12B). No metastasis was found in the liver and no para-aortic lymph node enlargement was detected. Cholecystectomy, hepatic wedge resection of the gallbladder fossa segment, lymph node dissection in hepatodudenal ligament and common hepatic artery (group 8a and 8p) were then performed. Postoperative pathological findings showed diffuse small round-shaped tumor cells in the gallbladder wall with marked interstitial fibrosis  can provide useful information of NETs, but confirmed diagnosis can only be made by pathological examination. Endoscopic ultrasonography with fine needle aspiration can increase the diagnostic sensitivity from 74% to 90%, compare to the endoscopic ultrasonography only . DefinitPersonalized and comprehensive therapy is preferred in treatment of NETs. Surgery, radiotherapy, peptide receptor mediated radionuclide therapy, chemotherapy and biological target treatment are all optional in NETs. Different surgical procedures such as mere cholecystectomy and cholecystectomy plus liver segment resection and lymph node dissections were reported according to different cases . It is eThe prognosis of GB-NETs is related to the tumor size, invasion depth, differentiation degree and metastasis state, with a 60.4% 5-year overall survival rate . Poor diGB-NECs are rare malignancies with aggressive biological behavior. Most of the cases are non hormone-producing and often asymptomatic leading to missed diagnosis in early stages. Preoperational pathological diagnosis and identification of clinical stage are of vital importance. Neo-adjuvant chemotherapy and somatostatin treatment got satisfying effects in this unresectable case which could also be instructive in NEC of other places. We suggest that for those NETs of advanced stage, patients may benefit from preoperative pathological diagnosis and standard neo-adjuvant chemotherapy which may also make complete removal of the tumor by surgery possible.A written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.MANEC: mixed adenoneuroendocrine carcinoma; NEC: neuroendocrine carcinoma; NET: neuroendocrine tumors; GB-NEC: gallbladder neuroendocrine carcinoma; EWSR1: Ewing sarcoma breakpoint region 1; SRS: somatostatin receptor scintigraphy; EP: VP16 and cis-platinum.The authors declare that they have no competing interests.WS and WFC contributed equally to this work. WS drafted the paper; WFC made analysis of the pathological examination and revised the paper carefully; SZ made contributions to acquisition of clinical data; JJP carried out the immunohistochemical staining; YLH designed and guided the research. All authors read and approved the final manuscript.