Pancreatic neuroendocrine tumours (pNETs) are rare and surgical resection offers the only possibility of cure for localised disease. The role of surgery in the setting of locally advanced and metastatic disease is more controversial. Emerging data suggests that synchronous surgical resection of pancreas and liver may be associated with increased survival. We report two cases of synchronous, one stage multivisceral resections for pNET and associated reconstruction. We highlight the technical issues involved in such extensive resections and demonstrate that one stage multivisceral operations can be achieved safely. Pancreatic neuroendocrine tumours (pNETs) are relatively rare with an annual incidence of two to three cases per million of population . Such tuTNM staging, the modified WHO classification and Ki-67 proliferative index may predict recurrence, but are less useful in individual cases due to the unpredictable nature of this disease . For a sSurgical options in the presence of locally advanced disease are more controversial however. Current clinical guidelines recommend aggressive surgical treatment . HoweverWhilst there is mounting evidence justifying such a radical approach ,5,6,8, pA 63 year old woman presented with a six month history of progressive upper abdominal discomfort and intermittent vomiting. History was significant only for left breast cancer for which she had undergone a mastectomy seven years earlier.Clinically she possessed a firm right upper quadrant, tender mass. Blood tests showed mildly deranged liver function tests  and an elevated serum chromogranin A level . Computed tomography (CT) of the abdomen showed a well demarcated head of pancreas lesion (4 × 4 cm) and a large heterogeneous right hemi-liver lesion (15 × 15 × 12 cm). Both lesions showed uptake on a subsequent octreotide scan. An endoscopic ultrasound was also performed and fine needle biopsies of both pancreas and liver lesions were shown to be consistent with a diagnosis of neuroendocrine tumour. Laparoscopy was negative for further dissemination of disease and the multi-disciplinary oncology team meeting consensus was in favour of surgical resection.A midline laparotomy was performed and an extended right hepatectomy commenced. Following mobilisation of the right colon, the duodenum was Kocherised. The avascular plane anterior to the inferior vena cava (IVC) was dissected and a nylon tape passed behind the liver. This facilitated ultrasonic dissector division of hepatic parenchyma with the "hanging manoeuvre". Inflow occlusion was not necessary. At this point, the right and middle hepatic veins were divided. The right portal vein was transected and oversewn transversely to avoid main portal vein trunk stenosis. This completed an extended right hepatectomy  and the specimen was removed  assessed. Following confirmation of resectability, the stomach antrum and common hepatic duct were divided sequentially. The neck of pancreas was then transected. The proximal jejunum was divided and the ligament of Treitz dissected to mobilise and remove the specimen from the abdomen. Figure The abdomen was drained and closed. Total operative time was 6.5 hours with an estimated blood loss of 750 ml. The post-operative period was complicated by an intra-abdominal collection which was managed with percutaneous drainage.Histopathological examination showed a well differentiated pancreatic neuroendocrine carcinoma 45 mm in diameter, with a mitotic rate of nine mitoses per 10 high power fields (hpf) and a Ki-67 proliferative index of 15%. All microscopic margins were clear. A completely excised single liver metastasis, 158 mm in diameter, was identified in the hepatectomy specimen with associated cytological atypia and focal coagulative necrosis. None of 33 resected lymph nodes were involved.The patient remains well two years following resection. Serial CT scans  showed no evidence of recurrence and CgA levels are normal.A 60 year old woman presented with a twelve month history of fatigue, anorexia, weight loss and abdominal distension. She had a history of well controlled hypertension and type II diabetes mellitus.Liver function tests were slightly abnormal . CT of the abdomen demonstrated a large pancreatic mass (13 × 9 × 5 cm) compressing the confluence of the portal and superior mesenteric veins Figure . The rigLaparoscopy was performed to exclude additional peritoneal disease and biopsies of the right liver tumour were taken. Biopsy specimens confirmed the diagnosis of a neuroendocrine tumour with a Ki-67 index of 4%.At a multidisciplinary oncology team meeting, consensus of opinion was that the patient should be offered resection. Volumetric analysis demonstrated a 24% future remnant liver volume. A right portal vein embolisation was performed with a view to inducing left lobe hypertrophy. Four weeks later, reassessment of the liver volume confirmed that the future left lateral section remnant volume had increased to 32%.A midline laparotomy was performed. Exploration confirmed that the pancreatic mass had invaded into the greater curvature of the stomach and adjacent transverse colon. Initially, an extended right hepatectomy  was performed including excision of the terminal part of the middle hepatic vein flush with the IVC. The left hepatic duct was divided, and the right hepatic artery was divided 1 cm distal to its confluence with the left hepatic artery. An extended Kocher's manoeuvre was performed and the posterior relations of the mass were assessed. It was evident that while the IVC and aorta were free from disease, the portal vein (PV) and coeliac axis were involved by tumour and would require resection, en-bloc with the mass. The superior mesenteric vein (SMV) and artery (SMA) were identified in the infra-colic compartment and the dissection plane was maintained along the SMA to its aortic origin. The right colon and small bowel were mobilised using the Cattell-Braasch manoeuvre .The involved PV and SMV were then transected above and below the mass, respectively. Continuity was restored by direct end to end anastomosis; facilitated by the extra mobility gained from the preceding hepatic resection and small bowel mesenteric mobilisation. Following this, an interposition saphenous vein graft was placed from the aorta to the junction of the right and left hepatic artery. The common hepatic artery was divided and the coeliac axis was divided and ligated flush with the aorta. The dissection plane was now continued to the left of the aorta along Gerota's fascia. The left adrenal gland was adherent to the tumour and was included in the en-bloc specimen. The terminal ileum, descending colon and gastro-oesophageal junction were all divided, thus completing the resection which consisted of the stomach, spleen, pancreas, duodenum, left adrenal, right colon and transverse colon Figure . ReconstHistopathological examination revealed a well differentiated pancreatic neuroendocrine carcinoma 95 mm in diameter with a mitotic rate of one mitosis per 10 hpf and a Ki-67 proliferative index of 2%  therapy was pursued for local control of hepatic disease. Bony disease was treated with radiotherapy.Disease appeared static until 12 months. Systemic chemotherapy was commenced upon medical oncology advice with everolimus. Unfortunately, she developed severe haematological and renal complications as a consequence and died 15 months after her initial operation.Successful multivisceral resections of this magnitude have not been previously described. The low incidence, variable biological behaviour of pNETs and a reluctance to undertake multi-visceral resections for advanced disease have been significant impediments to publishing large volume, prospective therapeutic studies.Despite this, the current European Neuroendocrine Tumour Society (ENETS) guidelines support "aggressive surgery" where tumours larger than 2 cm and/or locally advanced disease may necessitate en-bloc resection of adjacent organs . Whilst Given potential morbidity, mortality and the lasting impact that such operations may have upon patients, a survival advantage needs to be demonstrated to justify aggressive management. Retrospective analyses have shown some survival benefit following surgery for locally advanced disease ,10,11. HWith regard to liver metastases, a number of studies have shown that combined resection of the primary lesion and small volume metastatic liver disease improves survival outcomes ,8,12. ENManagement of inoperable metastatic liver disease may involve a spectrum of multi-modality therapy. In highly selected cases even liver transplantation may be considered , but mosBoth cases demonstrate that a complex multivisceral resection with synchronous hepatectomy can be performed safely, provided that the surgeon executes the multistep procedure in an appropriate sequence, in order to avoid the many potential pitfalls. For example, a staged approach with initial extended hepatectomy may have made a subsequent laparotomy and dissection in the supracolic compartment more hazardous. More importantly, in Case 2, initial resection of the primary lesion may have rendered subsequent attempts at inducing future remnant liver hypertrophy ineffective, due to the absence of the trophic effect of endogenous insulin. Early liver resection also aided further dissection as it provided increased manoeuvrability and facilitated selective control of the portal vein without further need for a Pringle manoeuvre. Additionally, sequential resection/reconstruction of the portal vein and coeliac axis minimised hepatic ischaemia. Had prolonged portal vein and coeliac axis clamping been required, the obliterated umbilical vein could have been used as a potential bypass conduit.Chylous ascites was a predictable complication in Case 2, given such extensive retroperitoneal dissection. In this situation we favoured a peritoneo-venous shunt over repeated peritoneal taps to lower the risk of infection.The natural history of pNETs continues to be difficult to predict despite advances in staging, grading and classification systems. A lack of consensus within methods of pathology reporting has also been highlighted recently  and servWhilst further histopathological and prognostic criteria such as the Ki-67 proliferative index and mitotic count are included, such markers may still underestimate the unpredictable nature of this disease . ContrasClinical decision making therefore remains difficult in individual cases and deciding which patients should be offered resection continues to challenge experienced clinicians . Beyond Complex multivisceral resections of neuroendocrine tumours can be achieved safely with appropriate preoperative planning and surgical expertise. We advocate resection of primary and secondary liver disease in a one stage procedure where patient co-morbidity and technical expertise allow. Further studies are required to justify and standardise the approach to aggressive surgery for locally advanced disease.Written informed consent was obtained from patients for publication of Case reports 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.JS, TH, RA and JG were involved in the clinical care of patients. JG, JS and NW collected clinical data. AG reported pathological findings and prepared slides for manuscript inclusion. JG, RA, NW, TH, SG and JS drafted the manuscript. All authors were involved in editing and final review.


Intracranial epidermoid cysts are uncommon benign tumors of developmental origin; malignant transformation of benign epidermoid cysts is rare, and their prognosis remains poor. We report a case of squamous cell carcinoma arising in the cerebellopontine angle. A 52-year-old man presented with left facial paralysis and cerebellar ataxia. He had undergone total removal of a benign epidermoid cyst six months previously. Postoperative magnetic resonance imaging of the brain revealed a heterogeneous and cystic lesion in the left cerebellopontine angle with hydrocephalus. The cyst wall was enhanced by gadolinium. He underwent ventricle-peritoneal shunt and removal again; the histopathological examination revealed a squamous cell carcinoma possibly arising from an underlying epidermoid cyst. This entity is being reported for its rarity. The presence of contrast enhancement at the site of an epidermoid cyst combined with an acute, progressive neurological deficit should alert the neurosurgeon to the possibility of a malignant transformation. Intracranial epidermoid cysts (ECs) are rare, histologically benign, slow-growing, congenital neoplasms of the central nervous system that may arise from retained ectodermal implants . MalignaA 52-year-old man had first presented with dizziness, cerebellar ataxia, and facial paresis (grade 3 House-Brackmann) (HB3). Magnetic resonance imaging (MRI) revealed a large cystic lesion of CPA, hypointense on T1-weighted image, and hyperintense on T2-weighted image, extending to the left parapontine, with partial enhancement of the capsule after intravenous administration of gadolinium .The tumor was removed via the suboccipital retrosigmoid approach in the sitting position, and surgery revealed a large cyst with a thin layer of white capsule containing yellowish-white, soft, and cheesy material. The cyst including the capsule and contents was removed completely. The patient recovered without incident, and the histological diagnosis was EC.Six months after the first surgery, he again presented severe facial paresis (HB5), gait disturbance, and intracranial pressure (ICP). MRI demonstrated a large heterogeneous mass isointense on T1-weighted image, hyperintense on T2-weighted image, strongly enhanced after gadolinium, extending to the upper medulla oblongata with hydrocephalus and unusual edema . Rapid dLocal radiation therapy (50 Gy) was administrated after surgery, with good followup, one month later his facial paralysis was well (HB2) without ICP.Epidermoid or “pearly” tumors were described by Cruveilhier and designated the “most beautiful tumors of the body” by Dandy. Although the formation of epidermoid lesions develop from aberrant ectodermal embryonic tissue in the neural groove at 4 or 5 weeks of fetal development, the mechanical introduction of such skin elements can also occur by skin puncture .Malignant transformation of EC into carcinoma is quite rare and was first reported by Ernst in 1912 . Most prRapid progression of symptoms and signs is the most important clinical indication of malignant transformation of EC. When transformation does occur, the clinical and radiological course is quite aggressive as compared with the indolent growth of EC . The typIn the present case, the rapid deterioration of symptoms was concomitant with predominant enhancement of the tumor on MRI, thus suggesting extremely rare malignant transformation of an IEC. The mechanisms of malignant transformation of EC are controversial and may involve inflammation caused by reaction to foreign bodies or in situ carcinoma. Chronic inflammatory response to repeated cyst rupture and subtotal resection of the cyst wall may cause the malignant transformation , 14. So,Treatment options include surgery with adjuvant chemotherapy or radiotherapy. Radiation therapy for intracranial SCC has been effective over a short period and stereotactic radiation therapy following the surgery is also effective in some cases, with disease-free survival of more than 5 or 8 years or local tumor control for 29 months . RecentlRapid onset of symptoms, recurrence, leptomeningeal carcinomatosis, and tumor enhancement in MRI suggests malignant degeneration of the EC. Surgery resection and adjuvant radiation therapy are highly recommended.


Epidermoid cyst of the quadrigeminal cistern is uncommon, and its presentation as mutism as the main clinical finding with no other neurological finding is very rare. We report a case where the epidermoid cyst presented with progressive symptoms of absolute mutism, which improved significantly following surgery. The possible causes and pathophysiological mechanism of mutism in the lesions of this region are discussed in this paper. Epidermoid cyst/tumor is a benign lesion that may arise in the spine or may arise intracranially. It may be intradural  or extradural . Intracranial epidermoid cyst accounts for 1.8 -2 % of all brain tumors. The mostThe term “mutism” describes patients who lack spontaneous speech despite the appearance of alertness. AccordinMutism is more commonly related to surgery in the posterior fossa tumor. It may be immediate or delayed. Virtually all cases of mutism occur within the first week of surgery, with 50% occurring within the first 2 days. Overwhelmingly, mutism of cerebellar origin has been reported following surgical interventions in the posterior fossa for tumors. However, there are other etiologies reported in the literature, including posterior fossa trauma, cerebellitis (inflammation of the cerebellum), cerebellar hemorrhage, embolic event and arterio-venous malformations. However, epidermoid cysts of the quadrigeminal cistern presenting with mutism are not known. We report a rare case of quadrigeminal cistern epidermoid with mutism. The causes, mechanism, pathophysiology, and management of such cases are discussed here.th postoperative day. The patient was continued on steroids for the next 2 weeks in order to take care of chemical meningitis. At the follow-up of 6 months, the girl was perfectly well.A 16-year-old female presented to us with a 10-month history of headache of mild to moderate severity. She gave history of occasional vomiting during the previous months. Her parents revealed that their daughter was failing to speak anything for the last 6 months. On examination, the child was fully conscious, following simple commands but not verbalizing at all, but she was able to gesticulate for most of the queries. She had upward gaze paresis with normal insight and reaction. Otherwise, the rest of the ocular movements remained unrestricted. Fundi showed mild papilloedema. Her routine investigations remained within normal limits. A magnetic resonance imaging image revealed a large homogeneously hypointense mass on the T1-weighted images, which was hyperintense on the T2-weighted image to occupy an entire quadrigeminal cistern . It was rd ventricle manifested mutism. The cyst was tapped three times and, after each tapping, the patient improved, but after a few days the symptoms recurred.[There are numerous case reports of mutism in the literature. In a report, a 14-year-old girl with epidermoid cyst in the 3recurred. ThereforTransient mutism has been reported in three cases following removal of the lateral and third ventricular mass following a transcallosal approach. Mutism mTransient mutism had also been reported in a 65-year-old man following resection of anterior falx meningioma; the lesion being at the supplementary motor area may be responsible for postoperative mutism in this area. A 13-yeaJonathan reported two patients, each of whom developed cerebellar mutism after tumor resection, using a posterior fossa approach. The first patient underwent gross total resection of a pineal region tumor via a supracerebellar approach. The secoForty-six cases of cerebellar mutism with mean age ±10.4 years following posterior cranial fossa surgery were studied. The pathological lesions were medulloblastomas in 33, astrocytomas in seven, ependymomas in four, metastases tumor in one and arteriovenous malformation in one. All mass lesions were considered to be large or very large. The lateIt is apparent from all the above reports that neither the occurrence of mutism was a sole manifestation in different cases nor the improvement was very dramatic following surgery. But, in our case report, mutism was the only presenting complaint with epidermoid cyst in the quadrigeminal cistern, and its improvement immediately after surgery denotes that some anatomical structure in this region is directly responsible for such a severe mutism, if compromised significantly. Although this epidermoid was not confined only to the quadrigeminal cistern, it was sizeable enough to cause a significant compression over the surrounding structures. It denotes that either one anatomical structure or bilateral involvement of one or more than one structure was responsible for such a severe mutism. The mutism in our case could be due to pressure at the reticular formation of the mesencephalon, posterior corpus callosum, bilateral fornices, bilateral thalami or a combination of all these structures.Mutism is a well known event after surgical intervention of tumors of the posterior fossa. This event is thought to be more evident in cases of tumors in which the brain stem is in some way mistreated during surgery, but it is clear that this type of mutism is speech disturbance, a motor disorder in which the cerebellum temporarily loses its capacity to poke the phonic nuclei function efficaciously to the point of anorthia and mutism. However,It seems probable that the anatomical substrate for mutism lies in close vicinity to the quadrigeminal cistern, which may be either reticular formation of midbrain, fornices, thalami or corpus callosum. However, the possibility of bilateral involvement of these paired anatomical structures cannot be denied. Compression of the midbrain may also be responsible for such an event.


Human herpes virus 8-unrelated primary effusion lymphoma (PEL)-like lymphoma is a rare type of large B cell lymphoma. This report presents the case of a male with abdominal pain and distension who was found to have massive ascites and enhanced peritoneum, mesenterium and greater omentum on enhanced computer tomography (CT) scan with negative ascitic cytology. The diagnosis of PEL-like lymphoma was established by fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT and laparoscopic biopsy of the greater omentum. To the best of our knowledge, this is only the second case report to describe FDG PET/CT presentations of PEL-like lymphoma, and the first case report to use laparoscopy for diagnosis. Primary effusion lymphoma (PEL) is a rare type of large B-cell lymphoma characterized by lymphomatous effusion of body cavities without lymphadenopathy or organomegaly. PEL often occurs in patients with human immunodeficiency virus (HIV) and/or human herpes virus type 8 (HHV-8) infections . However9 cells/l and hemoglobin levels of 12.8 g/dl. Aspartate aminotransferase levels were 14 U/l and alanine aminotransferase levels were 70 U/l. Tests for hepatitis markers, Epstein-Barr virus (EBV), HHV-8, HIV and tumor markers were negative.A 39-year-old male was referred to the First Affiliated Hospital of Wenzhou Medical College  with a one-week history of abdominal pain and distension. Laboratory tests revealed a white blood cell (WBC) count of 10.1×10The WBC count of the ascitic fluid was 640,000 cells/ml: 5% polymorphonuclear leukocytes, 71% lymphocytes and 24% abdominal cells. Bacterial cultures were negative. The ascitic effusion test for HHV-8 using polymerase chain reaction was also negative. Ascitic cytology was performed three times but no malignant cells were found. Gastroscopy and colonoscopy were also normal.Enhanced abdominal CT scan showed massive ascites and enhanced peritoneum, mesenterium and greater omentum but no detectable mass or lymphadenopathy. Therefore, the patient underwent FDG PET/CT examination. FDG PET/CT showed FDG uptake in the peritoneum, mesenterium and greater omentum . No massA laparoscopic biopsy of the greater omentum was performed, revealing lymphoma cells with large nuclei and abundant cytoplasm which exhibited a B-cell phenotype . ImmunohThe patient was diagnosed with HHV-8-unrelated HIV-negative PEL-like lymphoma (indeterminate phenotype). The patient and his relatives refused chemotherapy and the patient succumbed to PEL-like lymphoma one month later.PEL is often associated with HHV-8 and occurs most frequently in immunodeficient states . Howeveret al or rituximab-containing regimen (2) have frequently been administered in these cases. Although the prognosis of HIV-negative HHV-8-unrelated PEL-like lymphoma patients is better than the HIV-positive PEL group ( or rituxIn conclusion, PET/CT and laparoscopic biopsy may be useful diagnostic tools for PEL-like lymphoma when the origins of ascites cannot be determined by general ascitic examination or conventional imaging tests, such as CT scans.


Background. Primary pulmonary synovial sarcoma is a rare malignancy with a poor prognosis. Surgical resection and postoperative management of these tumors has not been previously described in pregnancy. Case. A 38-year-old pregnant woman was admitted for evaluation of a right thoracic mass found on chest radiography at 26 weeks of gestation. A computed tomography-guided biopsy was subsequently completed and demonstrated a high-grade neoplasm. A right pneumonectomy was performed at 28 weeks of gestation due to pulmonary decompensation, and pathological examination revealed a pulmonary synovial sarcoma. The patient developed a postpartum pulmonary embolism and expired 6 weeks after delivery. Conclusion. Aggressive intervention for pulmonary malignancies during pregnancy may be necessary. Complete tumor resection is the most important prognostic factor in primary pulmonary synovial sarcoma. Soft-tissue synovial sarcomas commonly arise in paraarticular locations of the extremities but are rarely found in the lungs. Primary pulmonary synovial sarcoma is an aggressive tumor accounting for approximately 0.05% of all primary lung malignancies , 2. The A 38-year-old African American woman at 26 weeks' gestation presented with a 3-week history of dyspnea, upper right back pain, and orthopnea. Her pregnancy was complicated by the finding of a small fetal ventricular septal defect on ultrasound. The remainder of her medical history was noncontributory. Physical examination revealed a patient in moderate distress with a heart rate of 126, blood pressure 140/68, and respiratory rate of 25. Auscultation was notable for crackles in the base of the right lung. Chest roentgenography demonstrated complete opacification of the right lower lobe with an adjacent effusion. A computed tomography (CT) angiogram was performed revealing a large, 12 cm partially calcified mass in the right chest with broad-based apposition to the mediastinum. This was followed by a CT-guided needle biopsy demonstrating a high-grade neoplasm . The patThe patient was followed closely with antepartum fetal testing and serial growth sonography. Intrauterine growth restriction was diagnosed at 30 weeks' gestation with an estimated fetal weight at the 6-percentile. Due to subsequent poor interval growth, a repeat cesarean section was performed at 32 weeks' gestation. The patient had an uncomplicated postoperative course and was discharged home on hospital day 3. Following delivery the newborn developed cardiac decompensation and was transferred for a pulmonary artery banding procedure. The infant was discharged to home on day of life 50. Four weeks after delivery, the patient re-presented with acute shortness of breath and was diagnosed with a pulmonary embolus. A repeat CT scan revealed continued tumor growth and near complete occlusion of the superior vena cava by extrinsic mass effect and tumor invasion . The patPrimary pulmonary synovial sarcoma (PPSS) is a rare malignancy with few reported cases and only one other case in pregnancy . PrimarySYT gene on chromosome 18 to one of two homologous genes on chromosome x, SSX1 or SSX2. The fusion transcripts are detected by reverse transcriptase-polymerase chain reaction (RT-PCR) or fluorescence in situ hybridization (FISH) and are thought to function as aberrant transcription regulators [Diagnosis is made by histologic appearance, immunohistochemistry, and more recently cytogenetic analysis. Cytogenetic analysis has become an increasingly important tool in diagnosing pulmonary synovial sarcoma. Primary pulmonary synovial sarcoma is characterized by a reciprocal chromosomal translocation t which fuses the gulators . These dgulators , 7. The radiologic features of PPSS vary and overlap with many other pulmonary and pleural lesions. On chest radiograph various descriptions have been reported to include focal pleural thickening, opacification of a hemithorax, consolidation, and uniform or homogeneous masses with well-circumscribed borders. An ipsilateral pleural effusion may be present; however, pneumothorax, cavitation, and calcification are not typically described. In our patient, the initial appearance was that of a pleural effusion.On CT, PPSS is often well-defined heterogeneous masses containing areas of fluid attenuation compatible with hemorrhage or necrosis. Tumor calcification, infiltration of the chest wall, and cortical bone destruction may be demonstrated with synovial sarcomas that arise in the chest wall , 8. MagnGiven the rarity of this malignancy and the dearth of data regarding the natural history of the disease, there are no guidelines directing optimal treatment, particularly in pregnancy. Furthermore, no controlled studies investigating adjuvant chemotherapy have been undertaken for such reasons. There is no standardized therapy; however, current standards of treatment involve multimodal therapy including surgical resection followed by adjuvant chemotherapy, radiotherapy, or both , 6. In gThe overall reported 5-year survival rate for this type of malignancy is approximately 50%. Predictors of poor prognosis include male gender, age over 20 years, tumor size >5 cm, large number of mitotic figures (>10 per high-powered field), poorly differentiated tumor cells, high grade, and neurovascular invasion. The most important prognostic factor is complete tumor resection. Metastases are common with tumors greater than 10 cm in diameter . In our This case highlights not only the diagnostic and treatment dilemma for PPSS in pregnancy, but also the poor prognosis associated with PPSS. Malignancies, even those as rare as PPSS, must be considered in the differential diagnosis of a pulmonary mass in pregnancy. Characteristic findings on computed tomography or magnetic radiography may help further to clarify the diagnosis. Given the aggressive nature of this tumor, early intervention during pregnancy with tumor resection and addition of chemotherapy treatment should be considered to optimize maternal outcomes. However, the gestational age and fetal condition must also be evaluated when clarifying palliative options.


In the era of double balloon enteroscopy, capsule endoscopy, CT, and MRI enterography is transabdominal ultrasonography (TUS) underestimated method for evaluation of small bowel pathology. As often initial imagine method in abdominal complaints, nowadays has TUS much better diagnostic potential than two decades ago. High-resolution ultrasound probes with harmonic imaging significantly improve resolution of bowel wall in real time, with possibility to asses bowel peristalsis. Color flow doppler enables evaluation of intramural bowel vascularisation, pulse wave doppler helps to quantificate flow in coeliac and superior mesenteric arteries. Small intestine contrast ultrasonography with oral contrast fluid, as well as contrast enhanced ultrasonography with intravenous microbubble contrast also improves small bowel imaging. We present a review of small intestine pathology that should be detected during ultrasound examinations, discuss technical requirements, advantages and limitations of TUS, typical ultrasound signs of Crohn's disease, ileus, celiac disease, intussusception, infectious enteritis, tumours, ischemic and haemorrhagic conditions of small bowel. In the hands of experienced investigator, despite some significant limitations, is transabdominal ultrasonography reliable, noninvasive and inexpensive alternative method to computerised tomography (CT) and magnetic resonance imaging (MRI) in small bowel examination. The reference diagnostic standard for all mucosal bowel diseases is endoscopy with histology, but some of small bowel diseases, despite introducing double balloon enteroscopy and capsule endoscopy, still need cross-sectional imaging, where nowadays dominate radiologic methods—CT enterography/enteroclysis and MRI enterography/enteroclysis. Whereas 20 years ago was diagnostic yield of bowel ultrasonography limited to detection of large tumours, ileus and extensive Crohn's disease, nowadays as one of the cross-sectional imaging methods transabdominal sonography has become established and relatively reliable method for examination of SB, thereby offers to gastroenterologists good possibility and reasons to amplify their diagnostic arsenal also in small bowel examination. Modern ultrasound devices with high-frequency (high resolution) probes and harmonic imaging significantly improve examination of SB by offering better overall image quality, better visualization of bowel pathology and associated changes in real time  , and ileus. In some conditions of SB  can TUS contribute to correct diagnosis.Using peroral (SICUS) and intravenous contrast (CEUS) offers images of SB pathology similar to the ones acquired by CT and MRI enterography, but reliable evaluation of entire small intestine by ultrasound is possible usually only in non-obese patients. However, advantage of high resolution sonography consist in high spatial resolution in pathological segment of SB, where focused TUS can provide additional information to CT and MRI imaging .In a meta-analysis of prospective studies comparing accuracy of CT, MRI, scintigraphy, PET, and TUS in inflammatory bowel disease (IBD) no significant differences were observed among these techniques—mean per-patient sensitivity (89.7%) and specificity (95.6%) and mean per-bowel segment sensitivity (92.9%) and specificity (92.9%) of TUS did not significantly differ from other evaluated methods .High-resolution ultrasound probes (frequencies >7.5 Mhz) exhibit stratification of SB wall—with five different concentric layers—the first from the lumen is echogenic interface between lumen content and mucosa, then hypoechogenic mucosa, echogenic submucosa in the middle of wall, next hypoechogenic muscularis propria and the fifth—outer echogenic layer represents serosa and interface with perienteric structures. These sonographic layers practically correspond to histological layers . ThickneHigh resolution (high-frequency) probes still have disadvantage of unsatisfactory penetration, so cannot be used in evaluating of deep abdominal structures, especially in obese patients, in addition, in some cases of initial forms of SB diseases false negative results are possible.Reliability of sonographic examination depends on good-class ultrasound device with standard abdominal 2.5–6 Mhz) convex and high resolution linear or convex probe7.5–14 MHz), both with harmonic mode, pulse wave doppler (PWD) for quantitative evaluation of celiac and superior mesentery flows, color flow doppler (CFD) and contrast enhanced ultrasonography (CEUS) software for detection and quantification of intramural vascularisation in thickened bowel wall and perienteric structures. Isosmotic polyethylene glycol (PEG) solution 1000 mL is required for small intestine contrast sonography (SICUS) [–6 Mhz co.5–14 MHzExperienced sonographer with practice in abdominal and bowel ultrasonography, enough time (at least 30 min) for examination, and information about results of other imaging methods or surgery are also necessary for reliability of small bowel examination.Examination should be performed after overnight fasting, in supine position. In bowel examination we should use both standard (2.5–6 MHz) abdominal convex probe and high resolution (7.5–14 Mhz) probe .Every examination of small bowel should be preceded by standard abdominal sonography with convex abdominal (2.5–6 Mhz) probe. This probe offers along with imaging of parenchymal abdominal organs also overall evaluation/panoramic view/of large and small bowel as well as flow parameters in coeliac (CA) and superior mesenteric (SMA) arteries.Then examination with high resolution probe (7.5–14 Mhz) should be focused on the suspect pathological (thickened) segments of SB. This probe provides high spatial resolution but only in superficial structures (higher frequency = worse depth penetration). If a pathology is detected, wall thickness, stratification, luminal patency, degree of stenosis or dilatation, and motility pattern should be determined .All parts of small bowel—duodenum, jejunum and ileum are accessible to TUS examination.Relatively stabile localisation of duodenum and terminal ileum Figures  makes thSmall Intestine Contrast Ultrasonography (SICUS) or Hydrosonography. TUS with using oral contrast solution (iso-osmolar nonabsorbable polyethylene glycol solution (PEG). The amount of PEG solution used in different studies varies between 200 and 2000 mL [ 2000 mL , 7, 11.  2000 mL  SICUS im 2000 mL , but ext 2000 mL  diagnostColor Flow (Power) Doppler—CFD. It is used to estimate presence, density or absence of vascular signals in thickened segments of bowel wall, in intraluminal or extraluminal pathological structures and for imaging flow in big abdominal vessels—SMA, coeliac trunk, portal vein. CFD is part of standard abdominal and bowel sonography.Duplex Scanning (TUS + PWD). B-mode assisted Pulse Wave Doppler can estimate flow parameters of coeliac trunk and SMA, usually with measurement of peak systolic velocity (PSV), end diastolic velocity (EDV), RI (resistance index = (PSV − EDV)/PSV), pulsatility index (PI) and minute flow volume (MFV) [me (MFV) –16. QuanIn gastroenterological practice usually uses only PSV, EDV, RI, and MVF in SMA and CA.Triplex Scanning or Color Assisted Duplex Scanning (TUS + CFD + PWD). Enables evaluation of SMA/CA flow and intramural flow in thickened bowel segments.CEUS-Contrast Enhanced Ultrasonography. is by EFSUMB recommendations [ndations  indicate After standard TUS in CEUS specific harmonic mode we apply sulfur-hexafluoride based second-generation echo-signal enhancer (SonoVue) injected as a bolus 1.2–5 mL, folowed by 10 mL of isotonic saline, with watching enhancement of bowel wall in examined segment. Amount of 1.2 mL is usually sufficient with using standard abdominal probe in harmonic mode, high-frequency probes usually need higher amount of contrast. Every other examined segment needs another intravenous bolus of contrast. All CEUS examination shoud be videograbbed for analysis of enhancement patterns of each evaluated bowel segment, then by ultrasound device dedicated or PC software can be assessed the vascularisation of the examined bowel loop , 9, 17. Using CEUS can significantly extend time of examination, not only in real time, but also in analysing of videosequences of examination.Crohn's disease (CD) of small bowel is usually suspected during initial TUS performed by experienced examiner. The basic sonographic feature of small bowel CD is segmentally thickened bowel wall >3 mm) with or without preserved wall stratification, intramural vascularisation evaluated by CFD in active inflammation is usually high  Figure , longitu mm with CEUS has potential of better intramural vascularisation imaging than CFD, so can be used to set the inflammatory activity in thickened bowel segments, to differentiate between inflammatory and fibrotic strictures, and between abscesses and infiltrates , 18, 24,TUS has also significant limitations in deep (pelvic) localised CD and in obese patients (insufficient penetration of high-frequency probes). Sufficient evaluation of TUS contribution in setting the diagnosis and evaluating stenosis, abscess, fistula, postoperative recurrence and activity of Crohn disease was recently documented by Calabrese et al. . Need foDespite the fact, that gold standard for the diagnosis of celiac disease is histologic confirmation of the intestinal damage in serologically positive individuals, in patients with untreated celiac disease we can regularly find out several sonographic signs that raise suspicion of this chronic disease also in clinically asymptomatic persons. Increased fluid content in moderately dilated bowel loops 25 to 35 mm) with hyperperistalsis in fasting state  to 35 mm, lightlyNone of the signs are specific, but combination of above mentioned signs is characteristic and indicates a suspicion of the disease .The most frequently visualised tumors of SB are localised in duodenum and terminal ileum. Tumors in other parts of SB can be viewed after gaining significant volume and are causing clinical symptomatology. Among the malignant tumors are more frequent adenocarcinoma localised prevalently in duodenum, then carcinoids with prevalent localisation in terminal ileum, followed by lymphomas in ileum and jejunum, and less frequent mesenchymal tumors, predominantly in jejunum . Most ofThe substantial part of SB is arterially supplied by superior mesenteric artery (SMA) except duodenum (part of celiac trunk). Imaging of celiac trunk and especially SMA should be done by all SB examinations, as well as evaluating of portal venous flow in accessible parts of portal vein. Absence of flow in SMA indicates occlusion Figures  and in aIn a patient with typical symptomatology of ileus TUS shows dilated bowel loops with diameter usually above 35 mm, with stagnation of intraluminal fluid. In initial phase of this condition we can see hyperperistalsis of bowel loops, small amount of free peritoneal fluid between dilated bowel loops. In about 50% of cases we can find out cause of ileus .Truong et al.  in a retUltrasound may detect the cause of ileus with specific sonographic findings such as external hernias, intestinal intussusception, tumors, ascariasis, superior mesenteric artery syndrome, bezoars, foreign bodies, and Crohn's disease.Sonographic findings suggesting a need for surgery include intraperitoneal free fluid, bowel wall thickness of more than 4 mm, and decreased or absent peristalsis in previously documented mechanically obstructed bowel. Bowel wall perfusion can be assessed by color doppler sonography, and the presence of free intraperitoneal air indicates bowel perforation . CT scanHaematomas of SB are usually sporadic complication of hypocoagulation states—especially caused by anticoagulation pharmacotherapy. In ultrasound view are small bowel haematomas typical with segmentally concentrically thickened bowel wall with or without preserved stratification Figures  and withIntussusception (invagination) of SB is in TUS typical by multilayered structure with onion or donut appearance in transverse view . In adulSonography in acute enteritis shows thickened inflamed bowel wall, usually with preserved stratification and with intramural hypervascularisation (in colour doppler) and hyperperistalsis. In some cases, especially caused by Yersinia enterocolitica, Campylobacter jejuni and Salmonella enteritidis  significn = 2), Mycobacterium avium intracellulare infection (n = 3), T-cell lymphoma (n = 2) and in carcinoma of small and large intestine (n = 3), and most patient had enlarged lymph nodes and so this phenomenon was very probably caused by lymph oedema of small bowel wall.Transabdominal ultrasonography in 66 patients with abdominal tuberculosis  revealed Transcutaneous ultrasound elasticity imaging (UEI) is a promising, noninvasive approach for measuring tissue mechanical properties, that can differentiate inflammatory from fibrotic intestine in rat models of IBD and can differentiate between fibrotic and unaffected intestine in humans with CD . PromisiTUS as, usually, the first diagnostic procedure in abdominal complaints reveals the most of Crohn's SB inflammations, ileus, and intussusceptions enable to express suspicion of celiac disease, significant stenosis, or SMA occlusion. In the known Crohn's disease transabdominal ultrasonography with oral contrast, color doppler, and in some cases intravenous contrast can reliably evaluate segmental inflammatory activity, local, and distal complications of a disease. Thanks to noninvasiveness and lack of radiation, TUS is a relatively good alternative to CT or MRI enterography, particularly in young patients and pregnant women. In the duodenum and terminal ileum, TUS can detect the most of benign and malignant tumors. TUS is patient and doctor friendly, noninvasive, and low-cost diagnostic procedure, and despite some significant limitations , in the hands of experienced examiner offers reliable tool for SB diseases examination.


The vast majority of paratesticular masses are benign. Magnetic resonance imaging of the scrotum may provide valuable information in the pre-operative work-up of scrotal masses, by allowing the precise localization of the lesion and helping in characterizing its nature. Diffusion-weighted magnetic resonance imaging is an evolving technique that can be used to improve tissue characterization, when interpreted with the findings of conventional magnetic resonance sequences. We present the case of an adenomatoid tumor of the tunica albuginea, with abundant fibrosis evaluated by magnetic resonance imaging of the scrotum, including both conventional and diffusion-weighted sequences. To the best of our knowledge, there are very few reports in the English literature regarding the magnetic resonance imaging features of this rare benign paratesticular tumor and no report on the diffusion-weighted magnetic resonance findings. We discuss the value of magnetic resonance imaging in the pre-operative diagnosis of benign fibromatous paratesticular tumors and differential diagnosis.A 45-year-old Caucasian man was referred to us with a palpable left scrotal mass. Magnetic resonance imaging of his scrotum revealed the presence of a multilobular left paratesticular mass, mainly detected with very low signal intensity on T2-weighted images and restricted diffusion on apparent diffusion coefficient maps. These findings were suggestive of a fibrous component, and were confirmed on histology following lesion excision.Magnetic resonance imaging of the scrotum, by using both conventional and diffusion-weighted sequences, could have a potential role in the evaluation of scrotal masses. Determining the accurate location of a scrotal mass, whether intratesticular or paratesticular is extremely important pre-operatively, to ensure adequate treatment planning. Most paratesticular masses are benign, therefore radical orchiectomy may be obviated ,2. MagneAdenomatoid tumors are benign mesothelial neoplasms, accounting for approximately 30% of all paratesticular neoplasms -4. The mA 45-year-old Caucasian man presented to our Urology department with a palpable left scrotal mass, known for two years, which had progressively enlarged during the last three months. He reported no history of epididymitis, torsion or trauma. On clinical examination the mass was painless, firm and mobile. His serum tumor markers, including alpha-fetoprotein, beta-human chorionic gonadotropin and lactate dehydrogenase, were normal.b-value was 900 s/mm2. A multilobular left paratesticular mass (Figures -3mm2/s, and others of very low T2 signal and ADC value of 0.86 × 10-3 mm2/s (Figures -3mm2/s was also revealed. Both of his testicles, his epididymis and his spermatic cords were normal. The mean ADC value of his testicular parenchyma was 0.94 × 10-3 mm2/s and that of the epididymis 1.37 × 10-3mm2/s. His left testicular tunicae were intact. Based on MRI findings, the diagnosis of a benign fibromatous paratesticular tumor was suggested. Therefore, our patient underwent local excision of the mass. Histopathology reported an adenomatoid tumor of the tunica albuginea, with abundant fibrosis. Our patient is now well, without signs of disease on clinical and sonographic examination, one year after surgery.Sonographic examination showed a sharply-demarcated hypoechoic, vascular left paratesticular mass, located close to the head of his epididymis. A large left hydrocele, with low level echoes was also found. MRI evaluation of the scrotum was done on a 1.5-T magnet unit, using a pelvic phased-array coil. The study included fast spin-echo axial, sagittal and coronal T2-weighted sequences and spin-echo axial T1-weighted sequences. Diffusion imaging was performed in the axial plane, using a single shot, multi-slice spin-echo planar diffusion pulse sequence. The maximum  Figures , in clos Figures . A largeSolid neoplasms of the paratesticular tissues are rare ,2. They MRI of the scrotum as an alternative imaging modality has been proven with satisfactory results in the evaluation of scrotal contents ,2,5-7. Tet al. reported a case of an adenomatoid tumor of the tunica albuginea evaluated by MRI [MR examination of the scrotum in our patient revealed the presence of a sharply-demarcated multilobular paratesticular mass, isointense on T1-weighted images, heterogeneous, but predominantly of very low signal intensity on T2-weighted images. One limitation of the MR protocol used in this study was that it did not include post-contrast images, although the lesion was reported with vascularity on sonographic examination. The hypointensity of the mass on T2-weighted images and the restricted diffusion on ADC maps was suggestive for the presence of fibrous tissue, proved through histology to correspond to the abundant fibrotic component of an adenomatoid tumor of the tunica albuginea. The presence of abundant collagen-producing fibroblastic cells and a dense network of collagen fibres cause restriction in the diffusion of the water molecules in fibrotic lesions, as also proved in our patient ,9. Pateld by MRI . The tumd by MRI .Differential diagnosis of benign fibromatous paratesticular masses, as in our case, should include fibrous pseudotumor. This rare tumor is not a true neoplasm, but a reactive fibrous proliferation of the extratesticular tissues ,2,10,11.MRI evaluation in our patient provided valuable information in the pre-operative work-up, by allowing the precise localization of the mass and helping in characterizing the benign nature of fibrous paratesticular tumor, by using both the conventional and diffusion MRI. Confirmation of the diagnostic efficacy of MRI examination with prospective studies in unselected scrotal masses is required.ADC: apparent diffusion coefficient; DW: diffusion-weighted; MRI: magnetic resonance imaging.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.ACT, MIA and KT were major contributors in writing the manuscript. PG and NS had contribution to conception and data acquisition, and also in writing this manuscript. All authors read and approved the final manuscript.


Pituitary adenoma may present with neuro-ophthalmic manifestations and, typically, rapid tumor expansion is the result of apoplexy. Herein, we present the first case of an isolated sixth cranial nerve palsy as initial feature of a rapidly expanding ACTH positive silent tumor without apoplexy.A 44 year old female with a history of sarcoidosis presented with an isolated sixth cranial nerve palsy as the initial clinical feature of a rapidly expanding ACTH positive silent pituitary adenoma. The patient underwent emergent transsphenoidal hypophysectomy for this rapidly progressive tumor and subsequently regained complete vision and ocular motility. Despite tumor extension into the cavernous sinus, the other cranial nerves were spared during the initial presentation.This case illustrates the need to consider a rapidly growing pituitary tumor as a possibility when presented with a rapidly progressive ophthalmoplegia. Pituitary adenoma, the most common cause of sellar masses after the third decade of life, may present with neuro-ophthalmic manifestations. Visual impairment is the most common presenting symptom in patients with a non-secreting adenoma as a result of chiasmal compression -3. Less A 44-year-old female with past medical history significant for sarcoidosis, not currently receiving treatment, and hypertension presented to the clinic with a chief complaint of vague headaches and horizontal diplopia that worsened with left gaze of one days' duration. Neuro-ophthalmologic exam revealed an isolated, incomplete, left cranial nerve six palsy. Visual acuity was 20/20 bilaterally, Humphrey visual field testing with 24-2 testing strategy was full bilaterally, and dilated fundus examination revealed the absence of papilledema and a normal retinal periphery. There was no clinical evidence of either myasthenia gravis or thyroid ophthalmopathy.An MRI of the orbits and brain Figures , 2 reveaThe patient was immediately admitted to the neurosurgical service for preoperative studies and scheduled for urgent transsphenoidal hypophysectomy. Preoperative and systemic work up was initiated, inclusive of complete blood count, metabolic profile, coagulation studies, sedimentation rate, thyroid function tests, acetylcholine receptor antibodies both binding and blocking, & angiotensin converting enzyme. All tests were within normal limits aside from an elevated ACE, which was unchanged from previous examination. Review of history and physical exam revealed no clinical evidence of hypercortisolemia, hyperprolactinemia, or menstrual dysfunction. Evaluation of the pituitary hormone status of tumor, inclusive of ACTH, cortisol, prolactin, IGF-1, LH, and FSH, was scheduled but could not be completed due to rapid clinical deterioration.The patient remained stable over the next 36 hours, at which point she developed a left-sided, severe, retro-orbital headache. Over the next four hours the patient developed acute left sided ptosis and proptosis with complete ophthalmoplegia and decreased visual acuity, findings consistent with complete cavernous sinus involvement. An emergent head CT was then obtained which demonstrated an enlarging mass in the sella without evidence of intra- or extra-lesional hemorrhage. Final dimensions of the tumor on this non-contrast head CT were 1.5 × 1.3 × 2.4 cm, overall a significant increase in size from the measured dimensions of 1.5 × 1.9 × 1.4 cm found on initial MRI especially given that MRI has been shown to provide better evaluation of pituitary macroadenomas .The patient underwent emergent transsphenoidal hypophysectomy for this rapidly progressive tumor. Intraoperatively, the tumor was found to have clinical extension into the cavernous sinus. Pathologic examination of the mass Figures , 4, 5 deThe cavernous sinus contains the carotid artery as well as the oculomotor, trochlear, ophthalmic and maxillary divisions of the trigeminal, and abducens nerves. Extraocular palsy generally indicates compression of the cavernous sinus wall or direct extension of the pituitary adenoma into the cavernous sinus ,8. The iDespite the patients past medical history being positive for sarcoidosis, there was no evidence of neurosarcoidosis in this case. Overall, 25-60% of patients with systemic sarcoidosis will develop ocular symptoms, while 5% will develop neurosarcoidosis. Cranial nerve neuropathies are the most frequent neurological manifestation; cranial nerves II, III, IV, V, VI, and VII may be affected individually or in combination -17.The rapid expansion of the tumor secondary to tumor growth and not apoplexy, as evidenced by the lack of acute hemorrhage observed on imaging and subsequently on pathological examination, in this ACTH positive silent tumor is highly unusual. In a series of non-operated patients, the mean increase in tumor diameter was 0.6 mm per year . FurtherThe authors declare that they have no competing interests; there was no grant support or research funding and no proprietary interests in the materials describedAK, NS, JJ, and BR treated the patient and in doing so acquired the case data; they were also involved with drafting of the manuscript. IM interpreted the pathological specimens and was involved with drafting of the manuscript. EK assisted in data acquisition and was involved with drafting the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/11/4/prepub


Blue nevi that arise from the Müllerian tract are rare melanocytic lesions. Several histopathologic variants of cellular blue nevi have been described. The angiomatoid variant is characterized by a vascular component, and is considered to be a rare variant. Few studies have explored the influence of pregnancy on melanocytic lesions.A 29-year-old woman was presented with a pigmented vaginal lesion that increased gradually during pregnancy. A full term gynecologic examination showed a tumor mass protruding into the vaginal canal. The mass was resected during cesarean-section under the clinical impression of vaginal hemangioma.Gross examination revealed a cystic mass measuring 6.0 × 4.3 × 3.5 cm, which was filled with dark friable material. Histologically, the mass showed a subepithelial cellular proliferation of heavily pigmented dendritic melanocytes with prominent vascular stroma. Cytologic pleomorphism, junctional activity, atypical mitosis, and necrosis were not found. The proliferation was immunoreactive for HMB-45, S-100 and melan-A, and non-immunoreactive for CD34, smooth muscle actin, and AE1/AE3. The MIB-1 proliferative index was less than 1%. The patient had a postoperative course without complication.Angiomatoid giant cellular blue nevus arising from the vagina during pregnancy is extremely rare. The low proliferative index and absence of cytologic pleomorphism, or necrosis, supports a benign biological behavior. Clinical follow-up showed no evidence of recurrence at one year after the resection of the mass. Blue nevi are rare benign pigmented lesions that are derived from dermal melanocytes. They may develop on anatomical regions such as hands or feet, and, less commonly, on the head, neck, trunk and buttocks . Their oThe classification of blue nevi is complex, with biological behavior being benign, borderline, or malignant . Only a th week of pregnancy, and showed a 1.9 × 0.9 cm oval hypoechoic bilobed area overlaying introitus  Figure  ImmunohiBlue nevi are distinct dendritic melanocytic proliferations that may arise in the skin  or MülleBlue nevi have a wide spectrum of morphological variants. The common blue nevus and cellular blue nevus are the most frequent types . Other mThe angiomatoid variant is characterized by a vascular component and is described as an extremely rare variant .The presence of a rich vascular component was described as a distinct variant of other tumors, including melanocytic and non-melanocytic lesions. The angiomatoid melanoma is a rare morphologic variant . AngiomaThe occurrence of a cellular blue nevus in the vagina is extremely rare and, to the author's knowledge, only three cases have been published in the literature . Giant cFew studies have explored the influence of pregnancy on melanocytic lesions  or theirThe angiomatoid giant cellular blue nevus of the vagina is extremely rare, and may clinically mimic hemangioma. The low proliferative index as shown by MIB-1 immunohistochemical marker and the absence of nuclear pleomorphism, or necrosis indicated benign histological features. This observation was supported by the absence of any recurrence at the clinical follow up after one year.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.GCBN: Giant cellular blue nevusThe authors declare that they have no competing interests.The author prepared, read and approved the final manuscript


Absence of specific clinical symptoms and a good prognosis after wide resection are the characteristics of this entity. Chemotherapy is not indicated in this highly differentiated tumour.Low-grade central osteosarcoma is a very rare subtype of osteosarcoma with a predilection for the metaphysis of long bones and a peak incidence in the 3 A 12-year old girl presented with limping, swelling and pain in the mid of the left femur. Radiography showed a 12 cm long intraosseous expansion with lamellated periosteal reaction and contrast medium enhancement in MRI. Although radiology led to the differential diagnoses of Ewing’s sarcoma, osteomyelitis and fibrous dysplasia, the histological specimen showed a hyopocellular spindle-cell proliferation arranged in fascicles with mild cytologic atypia and only single mitotic figures. In synopsis with radiology the diagnosis of low-grade central osteosarcoma was made and confirmed by reference pathology. The tumour was resected with wide margins and reconstruction was performed with a vascularized fibula, a homologous allograft and a plate. Staging was negative for recurrence and metastasis at a follow-up of 16 months.rd decade. The diaphyseal localization and the young age make this case special. To achieve the correct diagnosis of this rare low-grade entity and thereby the adequate treatment, despite a wide range of differential diagnoses, a multidisciplinary approach is essential.Low-grade osteosarcoma accounts for only 1% of all osteosarcomas with a peak incidence in the 3 The pain started 3 days prior to the presentation and did not persist at night or at rest. Additionally the patient herself detected a mass in the left femur. The clinical examination revealed a visible and palpable swelling of the femur. On exertion of pressure it was painful. The surrounding skin and the remaining examination were normal. There was no history of weight loss, fever, chills, fatigue or exhaustion. The patient had no relevant past medical history. Routine laboratory tests were within normal range.Radiography showed an osseous expansion with lamellated periosteal reaction in the diaphysis of the left femur . The MR The treatment included a resection with wide margins and a reconstruction of the femur with a contralateral vascularized fibula, a homologous allograft and a plate . Fifteenet al. in 1977.7LGCOS was first described by Unni LGCOS is typically localized in the long bones with a predilection for the lower limb, especially the femur is most frequently affected.5Macroscopically, LGCOS is a well demarcated and large tumour with 2–25 cm in greatest dimension. The appearance of the tumour is variable from firm and gritty to white- fibrous and rubbery tissue. Hemorrhagic areas may be present as well as extension into the adjacent soft tissue.9Radiographs show a variable appearance in LGCOS, mimicking benign lesions, such as fibrous dysplasia (FD). Andresen et al. have described four different radiographic patterns of LGCOS: lytic with varying amounts of thick and coarse trabeculation, predominantly lytic with few thin, incomplete trabecula, densly sclerotic and mixed sclerotic and lytic. MRI or CT scans are mandatory to detect features of aggressiveness, such as cortical disruption and intramedullary or soft tissue expansion. Thus MRI and CT scans are the modality of choice, to differentiate between benign lesions and low-grade malignancies.10As differential diagnoses benign and low- grade malignant lesions have to be considered. LGCOS is initially often misdiagnosed as FD, due to similar histological and radiological findings.12Desmoplastic fibromas, low-grade fibrosarcomas and parosteal osteosarcoma are differential diagnoses for low-grade malignant tumours. Parosteal osteosarcoma can be ruled out by its location, since this tumour does not infiltrate into the medullary cavity. Both desmoplastic and low- grade fibrosarcoma can radiologically resemble LGCOS, but there’s a lack of bone formation in these entities.7Considering the patient’s age and the diaphyseal location in the presented case, these features are very uncommon within an already extremely rare tumour entity. A constellation like this makes it difficult to obtain a correct diagnosis, which can only be achieved by a multidisciplinary approach, including radiology, pathology and orthopaedics. When misdiagnosed or mistreated the patient is set at risk to develop high-grade osteosarcoma and metastasis. Unnecessary exposition to chemo- or radiotherapy has to be avoided. Therefore, one should be aware of this rare subtype of osteosarcoma and LGCOS should be considered as a differential diagnosis.


To highlight unusual presentations of Churg Strauss Syndrome (CSS) in children.Raising clinical awareness of atypical presentations.Case note review and literature search.9/l, ESR normal, no proteinuria). On needle biopsy, dilated ducts contained necrotic eosinophilic debris, intense infiltrate of eosinophils and plasma cells without granulomatous infiltration or vasculitis. Despite withdrawing Montelukast the mass persisted but treatment with steroids was successful.A 15 year old girl with worsening asthma and rhinitis presented with a persistent 10x6 cm breast lump 6 months after starting Montelukast. Chest X-rays and CT scan were normal, maxillary sinuses congested, with absolute eosinophilia of 2.4 x 109), anaemia, ESR 114 and positive pANCA (non PR3 / MPO). A good clinical response to treatment with steroids, cyclophosphamide (IV) and immunoglobulin is currently maintained on MMF and low dose steroid with anticoagulation (warfarin). Her cardiac mass has not regressed, but remained static, most likely representing chronic endocardial fibrosis.A 14 year old girl presented with worsening asthma associated with malaise, weight loss, an intermittent vasculitic rash and abdominal pain. She had a large right ventricular mass with pericardial effusion and endocardial impairment, pulmonary infiltrates, microscopic haematuria, vasculitic changes on cerebral MRI, a raised eosinophil count (6.16x10These cases illustrate atypical presentations of CSS in children. Eosinophilic mastitis may be idiopathic, parasitic or part of CSS - reported following treatment with Montelukast but never with eosinophilic mastitis. Cardiac masses in CSS have been reported occasionally in adults, but not previously seen in children.


Heterotopic pancreas is a rare condition and its diagnosis is often difficult. Traditionally the condition is treated by open surgery. We report two young women with symptomatic heterotopic pancreas located in the prepyloric region. In the first patient, upper gastrointestinal endoscopy identified a round sessile lesion with a central umbilication of the mucosa without bleeding and in the second endoscopy showed a lesion with intraluminal protrusion. In both cases, a diagnostic laparoscopy identified masses amenable to laparoscopic excision. Intraoperative histology confirmed ectopic pancreatic tissue in both. In the treatment of heterotopic pancreas, laparoscopic excision provides a feasible, safe and effective treatment option. Heterotopic pancreas (HP) is defined as pancreatic tissue found outside the ectopic pancreas without any anatomic or vascular connections between them. Gastric We report two patients with chronic recurrent abdominal pain and upper gastrointestinal bleeding caused by HP and their successful laparoscopic management.A 28-year-old woman with past history of recurrent attacks of epigastric pain and progressive dyspeptic symptoms was admitted with a bout of hematemesis. General and abdominal examinations were unremarkable. Blood count and liver function tests were normal. An upper gastrointestinal endoscopy revealed a nonbleeding, firm, round sessile nodular lesion with central umbilication in the gastric antrum . A surfaA 32-year-old female with a 2 year history of recurrent upper abdominal pain was admitted with progressively increasing epigastric pain radiating laterally. She was in good health and her vital signs, cardiovascular and respiratory systems were normal. Abdominal palpation revealed epigastric tenderness. Blood count, liver function tests, amylase and lipase tests were normal. Abdominal ultrasound and computerized tomography were unremarkable. An upper gastrointestinal endoscopy revealed an intraluminal protrusion in the prepyloric area ; the mucThe estimated incidence of HP is 1 per 500 upper abdominal operations and between 0.6 and 14.0% in autopsy cases.1 More freHP often remains asymptomatic throughout life but may sometimes cause symptoms including recurrent epigastric pain, upper gastrointestinal bleeding, gastric ulceration, gastric outlet obstruction or pancreatitis. In a few cases, complications such as pancreatic pseudocyst or cyst formation supervene. Association with insulinoma, adenoma and malignant transformation has been reported. All beniThe differential diagnosis includes a number of benign and malignant gastric wall tumors, intramural vessels and extrinsic compression from extramural structures. The clasAs can be seen from the case reports of both our patients, HP are eminently suitable for laparoscopic excision if situated in a suitable location. An intraoperative biopsy is recommended in order to prevent unnecessary extensive surgery. The application of laparoscopic surgery to HP in the stomach appears to be a technically feasible, safe and effective treatment for symptomatic patients. It should be considered a viable alternative to open surgery.


Tooth avulsion is defined as the complete displacement of the tooth out of its alveolar socket. The treatment of choice is immediate replantation or if that is not possible, placement of the tooth in an appropriate storage media. This report presents replantation of an avulsed maxillary central incisor after 12 hours of storage in milk. The tooth was replanted and splinted. One week later, it was treated endodontically and calcium hydroxide dressing was placed for 1 month; subsequently, the tooth was obturated with gutta-percha. During three years of follow-up, no evidence of ankylosis or inflammatory resorption was observed. After three years, the tooth was stable and remained functional and esthetically acceptable. Dental trauma may result in many significantly undesirable functional, esthetics and even psychological effects. The main causative factors for the trauma are fights and sports  .Tooth avulsion (exarticulation) is characterized as total displacement of the tooth out of its alveolar socket with damage to the periodontal ligament, cementum, alveolar bone, as well as gingival and pulpal tissues , 3. Its The most prevalent outcomes of avulsion are pulp necrosis and root resorption ,8. In anAvulsion causes complete rupture in the periodontal ligament (PDL) tissue; a part remains on the alveolar socket walls and other PDL remnants are seen attached to the root surface of the avulsed tooth. The part of the PDL attached to the alveolar wall will preserve its vitality, so there is no need for additional treatment, while the portion attached to the root surface may be at risk of necrosis. The most important factor in determining the final prognosis of an avulsed tooth is the presence of viable periodontal ligament cells on the root surface . ImmediaThis article reports the management of an avulsed maxillary permanent incisor that had been replanted after 12 hours and describes the successful clinical and radiographic findings observed after 3 years of follow-up.A 12 year old boy was referred to the pediatric department of Shiraz University of Medical Sciences  for the treatment of his traumatized teeth. The accident occurred 12 hours before his referral, due to a fall.The patient seemed healthy with no sign and symptom of cerebral involvement  . Extra-oIntra-oral examination demonstrated a permanent dentition, with class I skeletal and dental relationship; the oral hygiene was good. Clinically, dental traumas included complicated crown fracture of the maxillary left central incisor (tooth 21) and avulsion of the maxillary right central incisor (tooth 11) . The timTo complete the examination, periapical radiographs were taken. Radiographs showed a normal socket for tooth 11 and non-fractured root for tooth 21 were observed .The parents were informed about the treatment procedure and the expected prognosis. The treatment consisted of immediate replantation of avulsed tooth and partial pulpotomy of tooth 21 using hard setting calcium hydroxide.The avulsed tooth was soaked in 2% sodium fluoride gel and local anesthesia was administered with lidocaine 2% with 1:100000 epinephrine. First, the alveolar socket was prepared for proper positioning of the avulsed tooth. Then the tooth was rinsed with normal saline and carefully repositioned so that no periodontal ligament was damaged. Finally, we splinted the tooth with round wire (0.028 inch) and light cure resin composite. Another periapical radiograph was taken to confirm correct tooth repositioning. The splint was left in place for 2 weeks.Based on medical history, the patient did not need any anti tetanus booster. Systemic Tetracycline 250 mg and 0.2% chlorhexidine mouth rinse were prescribed for 7 days.One week later, tooth 11 was treated endodontically and calcium hydroxide dressing was placed for 1 month.Subsequently, the tooth was obturated with gutta-percha and restored with composite resin . The patThe patient was followed-up for three years. The tooth remained functional and was esthetically acceptable. The percussion tone and the tooth mobility were normal. Radiographically, no inflammatory or replacement root resorption was detected and the adjacent anterior teeth remained asymptomatic .Treatment of avulsion is often challenging and should be managed as an emergency. The golden time for replantation is 20-30 minutes; if it is not possible, the tooth should be kept in an appropriate storage media for preserving the viability of the periodontal ligament cells. The most preferable storage medias are Viaspan and HBSS  . ViaspanIf the physiologic media  are not available, soaking the tooth in non-physiologic media such as saliva , normal saline and tap water is a superior option compared to keeping it in a dry condition. Although they are detrimental to the periodontal cells, saliva and saline can maintain the cell viability for 2 hours and tap water can preserve them for 20 minutes .Another tissue which may be damaged during avulsion is the pulpal tissue. In closed apex teeth with narrow apical foramen, prophylactic endodontic treatment should be performed after replantation. But in wide open apex teeth, as there is a chance for revascularization, accurate observation is recommended. In these teeth, uninfected necrotic pulp may act as scaffold for later regenerative processes. Kling et al. reported that there is more chance of revascularization, if the tooth is replanted within 45 minutes . TherefoAnkylosis (replacement resorption) is defined as a fusion of the alveolar bone and root surface. It seems that the absence of viable periodontal ligament cells is the major etiological factor. During ankylosis, the tooth root merges with the remodeled normal bone and is gradually replaced with bone. The latter can be recognized within 4-8 weeks after replantation. Clinically, the tooth is immobile and might be infraoccluded and have a metallic percussion sound. Radiographically, the periodontal space disappears .In the present case, although the tooth was stored in milk for the whole extra-alveolar duration, some parts of the periodontal ligament tissues seemed to have become necrotic because of the prolonged extra-alveolar period., The authors chose to soak the avulsed tooth in 2% sodium fluoride gel for 20 minutes in order to reduce ankylosis progression; however, this procedure is not standard protocol. Fortunately, during the three years of follow-up ankylosis was not evident clinically or radiographically.It is important to highlight that avulsion is a severe dental injury; if it is managed immediately and appropriately, the avulsed teeth can be preserved and be functional for some years. The three year follow-up indicated success of this treatment protocol.Although acceptable results were observed in the three-year follow-ups, further observation is required. Moreover, further clinical and in vivo studies are recommended to confirm that replantation after extended extra-oral time is applicable clinically and that fluoride application contributes to success.


Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm arising most commonly from follicular dendritic cells in the lymph nodes. It is exceedingly rare in extranodal sites, particularly in the pharyngeal region. The present study reports 3 cases occurring in the pharyngeal region. Case 1 had tonsil and cervical lymph node involvement, while case 3 also had tonsil involvement. Cases 1 and 3 relapsed locally at 3 and 17 months after surgery, respectively. Case 2 was diagnosed with a tumor in the parapharyngeal space and the patient succumbed to the disease 5 months after treatment with combined surgery and chemotherapy. All 3 cases were misdiagnosed initially. Pathological biopsy examination, including histopathology and immunohistochemistry, was essential for diagnosis. The data for 52 cases, including cases from the literature and the present cases, were analyzed. The results indicated that 57% (26/46) of the initial diagnoses were inaccurate, while the recurrence, metastasis and mortality rates were 40, 16 and 10%, respectively. The statistics supported the theory that FDCS of the pharyngeal region is a low-grade sarcoma. Involvement of the tonsils  and parapharyngeal space  were observed most commonly, while FDCS at various sites showed different prognoses. The various survival rates were calculated in the present study. The large tumors (≥4 cm) had a poorer prognosis than the small tumors . Among the 50 cases with available follow-up data, 46% (23/50) were treated with surgery alone, 52% (26/50) with combination therapy (surgery followed by chemotherapy and/or radiotherapy) and 2% (1/50) with surveillance. There was no statistically significant evidence (P>0.05) that combination therapy improves survival rates, compared with surgery alone. FDCs belong to the dendritic cell family which may be divided into 4 categories based on immunophenotypes: interdigitating dendritic, indeterminate, Langerhans and follicular dendritic cells . FDCs ar in 1997 .FDCS is a rare neoplasm. At present, ∼200 cases have been reported in the English-language literature. The majority of cases were observed in lymph nodes and less than one-third were extranodal . The phaμm-thick sections for hematoxylin and eosin (HE) staining and light microscopy.Tissue specimens from 3 patients, who were all treated at the First Affiliated Hospital of Zhengzhou University over the last decade, were collected and fixed in 10% buffered formalin, then dehydrated, embedded in paraffin and cut into 4-Immunohistochemical reactions were performed on the paraffinized sections using the EnVision method. The diagnostic antibodies used included antibodies against CD21, CD23, CD35, podoplanin (D2-40), CXCL-13, S100 protein, vimentin, epithelial membrane antigen (EMA), cytokeratin , CD163, leukocyte common antigen , CD1a, CD68, CD34, CD3 and CD20. Ki-67 (MIB-1) antigen was detected to show the proliferative activity of each lesion.In situ hybridization for EBV-encoded RNA (EBER) was also performed on the formalin-fixed, paraffin-embedded tissue sections and the probes used were purchased from Beijing Zhongshan Golden Bridge .www.ncbi.nlm.nih.gov/pubmed) with the term ‘follicular dendritic cell sarcoma’ combined with ‘extranodal’ and ‘pharyngeal’. Efforts were made to identify cases that were reported more than once in various settings and only the entry with the most up-to-date information was included in such cases. Only FDCS of the pharyngeal region were included in A literature search was performed using MEDLINE on PubMed  9.2. Disease-free survival (DFS) and overall survival (OS) rates were analyzed using the Kaplan-Meier method. Log-rank tests were used to analyze the equality of survival function over strata. P<0.05 was considered to indicate statistically significant differences.As shown in 2 i.v (day 1), vincristine 1.4 mg/m2 i.v (day 1), doxorubicin 40 mg/m2 i.v (day 1) and prednisone 100 mg p.o. (days 1–5) and 1 course of radiotherapy with a total of 5,600 cGy (200 cGy x 28 times). Subsequently, complete remission (CR) was achieved. The longest disease-free time (DFT) among the 3 present cases was 17 months.There was only 1 patient (case 1) who remained alive in the follow-up 7 to 24 months. Case 2 succumbed to the disease 7 months after initial treatment with combined surgery and chemotherapy without recurrence. Case 3 succumbed 7 months after local recurrence despite a second resection followed by radiotherapy . Case 1 relapsed locally in the left neck lymph nodes 6 months after surgery, then salvage therapy was performed, including 4 courses of the ‘CHOP’ adjuvant chemotherapy regimen according to the following schedule: cyclophosphamide 750 mg/mIn case 1, the tumor was confined by fibrous tissue and showed clear boundaries. The tumor cells were ovoid- to spindle-shaped and arranged in sheet-like or fascicular patterns. In case 2, the tumor cells showed diffusing sheet-like distributions, partially in the storiform pattern, and were separated by collagenous fibers . In casein situ hybridizations for EBER were negative in all cases. Certain antibodies were stained in 1 case but not stained in the others, as follows: in case 3, the immunohistochemical reaction for CD1a was negative, while CD3 and CD20 were positive in the background cells and negative in tumor cells; CD34 was negative in the tumor of case 2.As shown in The following summaries were obtained from a total of 52 cases of FDCS in the pharyngeal region, including the 3 present cases and 49 cases identified through a MEDLINE search .The patients ranged between 14 and 77 years old . The female/male ratio was 1:1.08 (25:27). The tumors tended to occur in young to middle aged adults without any gender difference. The FDCSs occurred in various anatomical parts of the pharyngeal region. The most common site was the tonsils, with 27 cases (52%). Other sites included the parapharyngeal space , nasopharynx , palate , pharynx  and hypopharynx . The tumors in the various anatomical regions showed distinct prognoses  and theiTo assess the prognosis, the size of the initial tumor was analyzed. The sizes varied between 1 and 7 cm, with an average of 3.4 cm, in the longitudinal dimension. The large tumors (≥4 cm) accounted for 34% (12/35) of cases and had recurrence, metastasis and mortality rates of 58.3, 8.3 and 33.3%, respectively. However, the small tumors  exhibited recurrence, metastasis and mortality rates of 17.4, 0 and 0%, respectively. The 2-year DFS and OS rates of the large tumors were 36.8 and 59.7%, respectively, in the follow-up of 6 to 132 months, while those of the small tumors were 81.5 and 100% in the follow-up of 5 to 54 months.Since the longest follow-up of the small tumors was 54 months (<60 months), the 5-year DFS and OS rates were not suitable, while those of the large tumors were 36.8 and 59.7%, respectively. Further statistical analysis of the size was performed among the 35 available tumors and indicated that large tumors (≥4 cm) had a poorer prognosis compared with small tumors (<4 cm) with regard to DFS  and OS  rates  relapsed, including 12  of local recurrence and 8  of distant metastasis. Additionally, 5 patients  succumbed to the disease. The disease-free time varied from 2 to 180 months . The recurrence, metastasis and mortality rates were 40, 16 and 10%, respectively. The 2- and 5-year DFS rates for the entire group were 66.2 and 51.3%, respectively, while the 2- and 5-year OS rates were 88.6 and 88.6%, respectively  underwent surgery to remove the tumor, with the exception of 1 case  where the patient opted to be surveilled. A total of 2 patients were lost to follow-up. Adjuvant treatment (radiotherapy and/or chemotherapy) was administered post-operatively to over half of the cases , among which 12  suffered recurrence , 8  suffered metastasis  and 2  succumbed to the disease in the follow-up of 6 to 180 months. For the group who received surgery and adjuvant therapy, the recurrence, metastasis and mortality rates were 46.2, 30.8 and 7.7%, respectively. The 2- and 5-year DFS rates were 73.8 and 66.4%, respectively, while the 2- and 5-year OS rates were 91.4 and 91.4%, respectively. Among the surgery alone group, recurrence was observed in 8 cases , no cases of metastasis were detected and 2 cases  succumbed to the disease in the follow-up of 5 to 120 months. The recurrence, metastasis and mortality rates were 34.8, 0 and 8.7%, respectively. The 2- and 5-year DFS rates were 67.4 and 56.2%, respectively, while the 2- and 5-year OS rates were 84.7 and 84.7%, respectively . The disFDCS of the pharyngeal region is an extremely rare neoplasm which has been detected in the tonsils, palate, parapharyngeal space, nasopharynx, pharynx and hypopharynx, with the majority of distant metastasis occurring in the cervical or axillary lymph nodes and lungs . Similarin situ hybridizations for EBV-encoded RNA were negative in the study by Duan et al are the choices for the initial treatment. Radical surgery  is essential for localized FDC tumors, as demonstrated by De Pas et al. Chera e al(et al analyzed al(et al. Other r al(et al. General al(et al who anal al(et al suggesteFor accurate identification and effective therapy for FDCS, more attention should be paid to diagnosis and treatment.


Although blunt trauma to a hernia-containing bowel is known to cause bowel perforation, this report documents the first incident of a small bowel transection following a non-traumatic event.We report the case of a 49-year-old African American man with a chronic incarcerated inguinal hernia awaiting elective repair. He presented to the Emergency Department with abdominal pain following an episode of coughing. On examination, he was found to have peritonitis. He underwent exploratory laparotomy, and had a complete small bowel transection. A bowel resection with primary anastomosis was performed, as well an inguinal hernia repair.Chronic hernia incarceration can lead to weakening and ischemia of the bowel, and minimal trauma can lead to perforation of the weakened segment. In such presentations, bowel resection and repair of the defect with a biological material is safe and feasible. The lifetime risk for development of inguinal hernias is 27% and 3% for men and women, respectively -3. AlthoA prospective study inclusive of 669 subjects demonstrated a 0.3% rate of bowel or omentum resections at elective repair of inguinal hernias . This raIn the present report, we describe a patient with a large inguinal hernia who developed bowel transection following a coughing episode. The rarity of this event is discussed along with the elective repair during the same setting.A 49-year-old African American man presented to a clinic with an 18-month history of a painful, enlarging right groin bulge. On physical examination, he had a large right inguinal hernia containing chronically incarcerated bowel with a scrotal component. He denied any history consistent with obstructive symptoms. However, he expressed interest in surgical intervention as the hernia continued to increase in size and started causing symptoms.While awaiting elective surgical repair, he presented to the Emergency Department (ED) with acute onset of right groin and abdominal pain. Upon arrival to the ED, he indicated that the symptoms began after he had gone to the bathroom for a bowel movement. Following his attempted continuation of bed rest, he began an aggressive bout of coughing. There was no history of trauma to his groin or abdomen. Following the coughing episode, he developed sharp, intense right groin pain and an episode of non-bloody, non-bilious vomiting.In the ED, his physical examination demonstrated hypertension (182/124 mmHg) and normocardia (99 beats per minute). Further examination demonstrated that his abdomen was distended and rigid as well as diffusely tender to mild palpation.Laboratory evaluation showed hypokalemia (3.6 mmol/L), but no other abnormalities. His white blood cell count was 8000 K/cu mm and his lactic acid was 1.3 mEq/L. An upright chest radiograph showed no free air.Given that his abdominal examination was consistent with peritonitis, he was taken to the operating room for an exploratory laparotomy. At celiotomy, there was a large amount of blood (approximately 400 cc) mixed with a small amount of feculent material. A segment of small bowel was found within the inguinal canal. Following lysis of adhesions and extraction of the bowel from the inguinal canal, a complete circumferential segment of jejunum was identified Figure . No otheA small bowel resection was performed on both sides of the transection with a gastrointestinal anastomosis device followed by a side-to-side two layer hand-sewn anastomosis.The patient had excellent hemodynamics and attention was directed at the inguinal canal through a separate groin incision. The inguinal canal demonstrated an obliterated transversalis fascia. The hernia sac was addressed via the transabdominal portion of the operation. We then proceeded with repair of the inguinal floor from the anterior incision. This was undertaken with biologic mesh (AlloDerm®) using the Lichtenstein technique. The remainder of his hospital course prior to discharge was uneventful. There was no surgical site infection at discharge or during clinic follow up. No recurrence had been noted up to 18 months following repair.Inguinal hernia repairs comprise a large portion of general surgical procedures. There are more than 20 million hernias estimated to be repaired annually around the world . In the The vast majority of hernias are repaired electively to prevent complications. Although complications are rare, they might be severe if not promptly addressed. Incarceration, strangulation and bowel perforation are known complications of inguinal hernias. Expectedly, intestinal perforation is higher in patients with inguinal hernias compared with the general population . This isNon-traumatic events leading to intestinal transection are rare events. Experimental models demonstrated that blunt trauma to an inguinal hernia could produce enough force (300mmHg) to lead to intestinal pressures of 260mmHg that can cause intestinal disruption . By contIn the present case, it is possible that the chronic incarceration of the hernia might have led to bowel edema, weakening of the bowel wall and the observed transection. There were no other identifiable risk factors in this patient that could have led to this outcome.In the present report, we elected to proceed with the repair of the inguinal hernia during the same operation. Although the timing of hernia repair in regards to the index operation is controversial, the status of the patient is a universal issue of concern in determining whether to proceed with a repair or not. However, if hemodynamic stability is well established, some authors favor a delayed repair ,16 and oThis operation would be classified as a contaminated case based on wound infection classification. Thus, the use of synthetic mesh was considered a poor option as this leads to high rates of infection, bleeding and fistula formation . A tissuThe present report describes an unusual complication of an incarcerated hernia in a young man. It is unclear what led to the weakening of the bowel wall causing a transection with a small increase in intraluminal and intra-abdominal pressure such as that produced by coughing. Repair of the floor with a biologic mesh in the same setting was feasible in this case and has produced good results to date.Informed consent was obtained from the patient for publication of this manuscript and accompanying image. A copy of the written consent is available for review by the Editor-in-Chief if this journal.The authors declare that they have no competing interest.HH, JD and SH were involved in the initial writing of the manuscript. SH provided major editing changes. HH, JD, TP were primarily involved in the care of the patient. TP and SD provided intellectual contributions to the content of the manuscript as well as editorial assistance. All authors have read and approved the final version of the manuscript.


Primary bone lymphoma (PBL) is a relatively uncommon entity. However, involvement of the cranial vault is an unusual manifestation of aggressive non-Hodgkin’s lymphoma. We report the case of a 42-year old immunocompetent woman who presented with an enlarging mass involving the right parietal bone. Magnetic resonance imaging (MRI) of the brain revealed an expansive tumor that affects the right parietal bone. Computed tomographic (CT) scans of the abdomen, chest and pelvis were negative for lymphadenopathy or organomegaly. Biopsy of the mass showed diffuse large B-cell non-Hodgkin’s lymphoma confirmed by immunohistochemical study. The patient had a complete response after 4 cycles of chemotherapy followed by external beam radiotherapy. After a follow-up of more than 9 months the patient is still in good local control without distant metastasis. The aim of our work is to report a case of Primary bone lymphoma of the cranial vault with good response to treatment combining sequential chemotherapy and radiotherapy. Primary bone lymphoma (PBL) represents about 5% of all non-Hodgkin lymphomas (NHL) and 3% of all bone malignancies . The femA 42-year old immunocompetent woman presented with a 10-month history of enlarging mass involving the right parietal bone without history of trauma. Physical examination showed a localized mass of 8 × 6 cm in diameter, in the right parietal region. There was no erythema and it was firm and slightly tender. The remainder of the examination was unremarkable; in particular no lymphadenopathy and no abdominal masses were felt.Neurological examination was normal. MRI of the brain revealed an expansive tumor (11 × 5 cm) affecting the right parietal bone, including the scalp . There wBiopsy of the mass with histopathology analysis showed diffuse architecture with mixture of large cleaved (centroblastic/centrocytic) and large noncleaved (centroblastic monomorphous) cells. The cytoplasm was scanty. The tumor cells did stain for CD20, CD79a and bcl2 protein. The proliferating fraction, as detected by Ki67 was high (more than 80% of neoplastic cells). The CD3 T-cell marker was negative. The immunohistochemical tests confirmed the diagnosis of large B-cell lymphoma . Based uThe diagnosis of PBL is more definite in patients who present with isolated bone localisations of lymphoma without other sites of involvement. Most series show a male preponderance, higher likelihood of early-stage disease presentation, and a median age of presentation in the fifth or sixth decade. Involvement of the cranial vault is an unusual manifestation of non-Hodgkin’s lymphoma in patients without HIV infection. Only fourteen patients with non-HIV related primary cranial vault lymphoma have been reported in the literature -15. Our Despite the increasing number of primary bone lymphoma and recent therapeutic advances, several questions still remain unanswered about the optimal management of these tumors. We believe that four cycles of a chemotherapeutic regimen (RCHOP) followed by involved field radiotherapy is the appropriate regimen for patients with primary localized cranial vault lymphoma. Furthermore, exclusive irradiation should be proposed for isolated cases. The prognosis is uncertain, but either direct cerebral invasion or leptomeningeal seeding should indicate a less favorable prognosis .This case demonstrates that PBL must be kept in mind in the differential diagnosis of primary lesions in the skull. Chemotherapy followed by involved field irradiation, appears to be an adapted therapy.The authors declare that they have no competing interests.ZF: performed literature review, the composition of this case report and manuscript writing. IL-MA: conception and design collection and assembly of data. BK: analyse and interpretation of anatomo-pathology findings. SB-HM- HE: analyse and interpretation of data, manuscript writing. All authors read and approved the final version of the manuscript.