Ewing sarcoma (ES) of the mandible is rare and can be mistaken for inflammation of dental origin. We present a 24-year old male patient which underwent radical tumour surgery and primary reconstruction with a microvascular osteoseptocutaneous free fibular flap as well as postoperative adjuvant chemotherapy. Incomplete osseous tumour resection required a second intervention. This case report recapitulates the clinical and histopathological findings in oral ES, demonstrates its sometimes difficult diagnosis and discusses the (dis-)advantages of primary osseous reconstruction in ablative tumour surgery. Primary malignant tumours of the jaws are rare and especially the diagnosis and treatment of Ewing sarcoma (ES) (ICD-0 code 9260/3) can be challenging. Less than 3% of all ES originate in the maxillofacial region, usually involving the mandible ,2. 90% oThe following case-report is presented to recapitulate the clinical and histopathological findings in oral ES, demonstrate its sometimes difficult diagnosis and discuss the (dis-) advantages of primary osseous reconstruction in ablative tumour surgery.Four weeks before admission a 24-year-old man noticed a swelling in the right floor of the mouth. Assuming that the tumour was an acute dental abscess the attending dentist incised the tumour and prescribed antibiotics. A temporary decline of the symptoms was noted and root channel treatment, root amputation and splinting of the teeth 43 – 45 due to negative sensibility and loosening was performed. However, the pathologic process of the right mandible enlarged constantly and the patient was referred to our department three weeks after initial treatment.On admission the patient was in good general condition. Body temperature was normal, blood examination showed elevated CRP 7.92 mg/l) and regular leucocyte count 8.71/nl). Physical examination revealed an asymmetric swelling of the right mandible with inconspicuous overlaying mucosa and skin . Histologically, the tumour was composed of uniform small round cells with indistinct cytoplasm and round nuclei with finely dispersed chromatin fig. . Lack ofThe patient underwent radical tumour surgery with subtotal mandibulectomy and cervical lymph node dissection Fig. . ReconstSwelling, pain, increased CRP, leukocytosis, and elevated temperature may be the first signs of oral ES, occurring also in odontogenic infections -6. In thES is a poorly differentiated neuroectodermal tumour with small, round and blue cells ,6. More Treatment of ES should include wide surgical resection and (neo-)adjuvant chemotherapy .In the presented case a resection with intraoperative histological control of the soft tissue margins and primary reconstruction of the mandible with a microvascular fibular free flap was performed. On the one hand, immediate bony and soft tissue reconstruction plays a key role in minimizing the deformity created by tumour resection and preventing wound contraction and displacement of bony segments. The combination of ablative and reconstructive surgery within a single procedure reduces the overall treatment time and provides the best aesthetic and functional results ,10. On tOral Ewing sarcoma is a rare tumour entity which can mimic odontogenic inflammation. If root amputation surgery is performed due to an extensive radiolucent lesion, histopathological evaluation should be mandatory. Primary bony reconstruction bears the risk of second intervention when dealing with ES in the mandible.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GM and DO analysed the case, operated the patient, reviewed all patient data and drafted the manuscript. BD carried out the histological analysis wrote the histological part of the paper and contributed to the writing of the final version. IS and KJ were involved in revising the article. All authors reviewed the paper for content and contributed to the writing of the manuscript. All authors approved the final report.


Esophageal squamous cell carcinoma is a relative common malignancy with a very poor prognosis, even adopting an integrated and multidisciplinary approach. According to the literature, gastrointestinal stromal tumors (GISTs) rarely originate from the esophagus. Moreover there are not reports of synchronous occurrence of squamous cell carcinoma and GIST at esophageal site.We describe a case of a 74 year old patient who underwent surgery for squamous cell carcinoma of the lower third of the esophagus with an incidental pathologic diagnosis of a concomitant GIST in the thoracic tract.In literature there is no evidence of concomitant squamous carcinoma and GIST of the thoracic esophagus, even if esophageal GISTs are sometimes described. The occasional finding of this neoplastic lesion underlines the importance of a carefully pathological diagnosis for its identification. Surgery, followed by a multidisciplinary approach remains the first-line treatment in both squamous and stromal neoplasm. GISTs are the most frequent non epithelial neoplasms of the gastrointestinal tract, with a preferred gastric localization (about 60% in the stomach and 20–30% in the intestine). The esophageal location is very uncommon and represents approximately 5% of gastrointestinal GISTs. In addition, the majority of esophageal GISTs arise at the gastro-esophageal junction; therefore a GIST located at the level of the thoracic esophagus is extremely rare. Many esophageal GISTs are diagnosed after the onset of clinical symptoms, or sometimes discovered by chance, during routine examinations . The diagnosed incidentally GISTs tend to be smaller in terms of centimetres and they already present the mutation at the KIT level. The elective treatment for these tumors is esophagectomy, paying particular attention to excision of the mucosa, sub mucosa and the muscular areas and of the surrounding periesophageal tissues.We describe a case of a 74 year old male patient, affected by a myeloproliferative syndrome. At the age of 26 he underwent a surgical gastrectomy for a hemorrhage caused by a perforated ulcer. Later, in December 2006, an upper gastrointestinal endoscopy revealed a neoplasm at the level of the thoracic esophagus. Multiple tissue biopsies histologically showed a scarcely differentiated squamous cell carcinoma. Subsequently all the necessary staging exams, including a total body Computed Tomography (CT) were performed. The CT did not show any suspected lymph nodes nor other lesions. In March 2007 the patient underwent a total esophagectomy and an esophago-gastroplasty with colon interposition for replacement of the esophagus.The final histological findings confirmed the diagnosis of infiltrating squamous cell carcinoma; Figure . The lesThe immunohistochemistry of the fused cells showed an intense and diffuse positivity for CD117, CD34 and CD99 and negative results for S100, desmin and actin Figure . The proThe aspects of this lesion reminded palisading-vacuolated spindle cell GISTs. In fact it showed nuclear palisading, resembling peripheral schwannomas and the typical feature of prominent perinuclear vacuolization.In May 2007, a total body CT scan showed the presence of necrotic-hemorrhagic foci, of probable ischemic nature, in the cortical and sub cortical brain regions. At the same time, the thoracic region showed the presence of diffuse micro nodules in the lungs, associated with chronic bronchial and peribronchial wall thickening. We also noticed a modest splenomegaly (22 cm) and, at the aortic-pulmonary window, in the inferior pre-tracheal and right hilum areas, several lymph nodes of 13 mm. Considering the pathological and clinical staging, the patient did not require chemotherapy treatment, and would continue his follow-up with routine examinations.et al [et al. [The term Gastric Stromal Tumors was first coined in 1983 by Mazur and Clark [et al , reveale [et al. .The median age of onset is 60–69 years and the symptoms are usually non-specific such as tiredness, abdominal discomfort and gastro-intestinal bleeding. The main instruments used for the diagnosis of GISTs are CT scans and PET scans.18F-FDG PET seems to have lower sensitivity than CT for gastrointestinal stromal tumors staging. However PET is superior in monitoring therapeutic treatment response to imatinib in patients with malignant GISTs. CT and PET are complementary and PET/CT techniques have been shown to be useful in diagnosis, staging and treatment response in GISTs [in GISTs -7. BasedPositivity for the CD117 is the key feature of GIST, but CD34 and nestin are other commonly expressed but less GIST-specific antigens. Moreover, these tumors can be positive for smooth muscle markers and generally negative for desmine. S-100 protein expression is rare and glial fibrillary acidic protein is not present. Keratine 18 and Keratine 8 are occasionally expressed.CD99 is not required for GIST diagnosis, but as its immunoreactivity is not uncommon in a variety of soft tissue tumors, correlation of expression of this marker with that of other immunomarkers and with morphology is warranted .Gastrointestinal (true) smooth muscle tumors, nerve sheath tumors, desmoids, inflammatory myofibroblastic tumors, inflammatory fibroid polyps, and undifferentiated sarcomas are the most commonly confused with GISTs. Rarely, poorly-differentiated carcinomas and histiocytic sarcoma can also take a part into differential diagnosis which is usually importantly aided by immonohistochemistry. These tumors have been reported as c-KIT negative but with other peculiar markers, generally not expressed in GISTs .The differential diagnosis among GISTs and other epithelial neoplastic lesions of the gastro-intestinal tract is very important. In fact 95% of these tumors express the trans-membrane receptor with tyrosine kinase activity c-kit, and above all this group of lesions tends to show an interesting response (sometimes dramatic) to new target treatments ,10-12.Clinical studies have shown that the elective medical treatment for patients with inoperable lesions is imatinib (400 mg/die) with positive responses above 50% . The useEsophageal GISTs are rarely described in literature, and no cases of both GIST and squamous cell carcinoma have been reported. On the contrary, other kind of tumors have been found together (synchronous or metachronous) with GISTs in the gastrointestinal system, such as low grade malign lymphoma and GIST in stomach, colorectal cancer, gastric cancer, small bowel or mesenterium tumors and carcinoid of pancreas -18.According to our epidemiological knowledge the hypothesis of a common etiopathogenesis at esophageal site, for GIST and esophageal squamous cell carcinoma cannot be supported. However we could hypothesize that development of these tumors may involve common carcinogen antigens, making the synchronous occurrence of GIST and other abdominal malignancy not only a coincidence.Our case report seems to be the only one described in a patient with a myeloproliferative syndrome, esophageal squamous cell carcinoma and GIST. The occasional finding of this latter neoplastic lesion underlines once more the importance of a carefully pathological diagnosis and a multidisciplinary approach. Of course, surgery ,20 remaiWritten consent was obtained from the patient for publication of this case report.The authors declare that they have no competing interests.GPS conceived of the study, partecipated in its design and drafting. EM conceived of the study, partecipated in its design and drafting. FT participated in the design of the study and collect the clinical data. LR participated in the design of the study and collect the clinical data. GP participated in the design of the study and collect the clinical data. AZ participated in the design of the study and collect the clinical data. FZ participated in the design of the study and collect the clinical data. GCP participated in the design of the study and collect the clinical data. JN carried out the histopathological evaluation. ST conceived of the study, participated in its design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.


Desmoplastic small round cell tumor (DSRCT) of the abdomen is a recently identified aggressive neoplasm. Very few cases have been reported in the literature. Thus, the treatment guidelines are yet to be defined. The role of chemotherapy, radiotherapy and surgery is evolving. We treated four cases of DSRCT involving the abdomen using combination chemotherapy and/or tumor cytoreductive surgery. There were two men and two women. The chemotherapy drugs consisted of cisplatin, adriamycin, etoposide, ifosphamide, vincristine and cyclophsophamide. All patients achieved meaningful partial response to chemotherapy, which maintained for 6–9 months. There were very minimal chemotherapy-related complications. At the time of reporting, the median survival time was 15 months. Thus, DSRCT is an aggressive intra-abdominal tumor with excellent chemoresponsiveness, but relapse is frequent. Desmoplastic small round cell tumor (DSRCT) is a recently identified histopathological entity. This malignancy was first described by Gerald and Rosai from Memorial Sloan Kettering Cancer Centre, New York. Since thet al. used whole abdominal radiotherapy as a consolidation treatment after chemotherapy in low-volume abdominal disease.[Management of DSRCT includes early surgical removal of the tumor followed by high-dose aggressive chemotherapy. Some authors recommended chemotherapy supplemented with stem cell transplantation. Radiothe disease. In gener2, ifosphamide 2 g/m2 (with mesna rescue), adriamycin 10 mg/m2 and etoposide 100 mg/m2 for 3 days every 4 weeks for six cycles. However, the patient only received two cycles in view of extreme chemotherapy-induced fatigue. Following the above chemotherapy, the patient achieved complete response and survived without symptoms 12 months postdiagnosis of disease.Mr. WRI is a 33-year-old gentleman who presented with complaints of abdominal pain and lump for 2–3 months duration. The pain was ill-defined and confined to the hypochondrium and was associated with distention of the abdomen. There was distended abdomen and dull on percussion. Initially, he was managed with symptomatic medications without any benefit. Subsequently, he was found to have multiple lumps all over the abdomen . ContrasZA, a 27-year-old lady, was seen by a surgeon with history of abdominal mass and pain for 1 month duration. The abdominal mass was arising from the pelvis and was fixed to the underlying viscera. A diagnostic CT scan was ordered and she was found to have a huge pelvic mass of size 13.5×13.9×21.9 cm. There were multiple ill-defined hypodense lesions suggestive of liver metastases. There was also a single lung nodule in the upper lobe of the right lungs. The histopathological examination of the biopsy specimen revealed features of DSRCT. In view of metastatic disease, she was advised VAC/IE combination chemotherapy. However, the patient developed progressive disease and succumbed to disease 3 months later.2, vincristine 2 mg and adriamycin 60 mg/m2 for 1 day followed by a gap of 3 weeks. Phase two chemotherapy consisted of ifosphamide 1.2 g/m2 and etoposide 100 mg/m2 for 5 days. The patient received four cycles of the above chemotherapy and achieved good partial response. Subsequent evaluation with CT scan showed good partial response and hence the patient was subjected to surgical debulking operation. Following surgery, the patient noticed epigastric pain and CT scan revealed multiple liver metastases. In view of painful liver metastases, local radiotherapy was delivered to the hepatic areas. A dose of 30 Gy in 15 fractions was delivered using a 6 MV linear accelerator. The patient achieved good palliation and is alive with metastatic disease at the end of 18 months from the date of diagnosis.SZ, a 33-year-old man, was seen by our surgical service with symptoms of abdominal tenderness and altered bowel habit for 1 month duration. There was also associated loss of appetite and loss of body weight. Clinical examination revealed multiple intra-abdominal lumps all over the abdomen. Colonoscopy was performed and was found to be normal. Contrast-enhanced CT scan of the thorax, abdomen and pelvis showed a bizarre contrast-enhanced mass lesion in the intraperitoneal area in the pelvic cavity, possibly invading the small and large bowels. There was presence of lesions in the liver. Excision biopsy specimen was subjected to histopathological evaluation, which showed islands of tumor cells separated by wide brands of fibrous tissue. The tumor cells were small and round, with enlarged nucleus and scanty cytoplasms. The mitotic figures were abundant, with individual cell necrosis. Immunohistochemistry test showed positivity for cytokeratin, NSE and desmin. The stroma was made uniform spindle-shaped embedded in collagen. The other hematological and chemistry parameters were normal. He was offered alternate VAC/IE combination chemotherapy. The chemotherapy consisted of cyclophosphamide 1.2 g/m2, adriamycin 50 mg/m2 and cisplatinum 50 mg/m2 every three weekly for six cycles. Following the above chemotherapy, she became asymptomatic, with very good partial response. On subsequent follow-up, her follow-up CT scan revealed residual disease in the abdomen. She was subsequently offered chemotherapy consisting of ifosphamide and etoposide for four cycles. However, she did not responded and developed progressive abdominal discomfort and abdominal distension due to disease. She subsequently died of disease 21 months post diagnosis.A 14-year-old girl presented to her gynecologist with a complaint of suprapubic mass for 2-3 months duration. The lump was gradually increasing in size within a short span. The lump was associated with pain. Initial gynecological ultrasound revealed an ovarian mass. Laparotomy was performed. Peroperatively, the patient presented a solid and cystic omental mass, which was highly vascularized, measuring 22 cm×17 cm×13 cm. CT scan evaluation of the abdomen and pelvis revealed multiple seedlings varying in size all over the peritoneum, including the undersurface of the diaphragm. There was also liver nodularity felt and multiple enlarged nodules adhered and infiltrated into the pelvic sidewalls. The superior part of the tumor was adhered to the greater curvature of the stomach from which a wedge resection was made. The histopathological evaluation of the excised specimen showed sheets and clusters of well-demarcated nests of tumor cells with areas of necrosis and hemorrhage. The cells were small and monomorphic with hyperchromatic nuclei. The cells contain scanty cytoplasm and numerous mitotic figures (17/10 hpf). Occasional rosette formation was also seen. The postoperative period was uneventful and she was referred to the oncologist for further management. The preoperative CA125 tumor marker was 290.9 U/ml. She was treated with CAP chemotherapy regimen to a dose of cyclophosphamide 500 mg/mDSRCT of the abdomen is an enigmatic disease whose biological behavior, genetics and morphological characteristics are yet to be elucidated. In view of nonspecific tumor biological understanding, the management of DSRCT is also not straightforward and specific. Further, formal recommendation of any treatment regimen is handicapped due to the rarity of the disease. The current knowledge about its management is generally based on the anecdotal case reports in the literature and the experience from major referral centers like Memorial Sloan Kettering Cancer Center and Italian cancer centers.7 In geneet al. of the Memorial Sloan-Kettering Cancer Centre, New York, USA The above regimen is offered for seven cycles, followed by delayed cytoreductive surgery and consolidated radiotherapy. The European chemotherapy protocols are equieffective, but are prescribed for four cycles, followed by similar management with surgery and radiotherapy.[DSRCT is very responsive to combination chemotherapy. The cytootherapy. The respotherapy. The diseotherapy.et al.[The role of radiotherapy is not consistent throughout the literature. Most studies reported radiotherapy for the palliation of symptomatic metastatic sites or to consolidate the residual disease in the abdomino-pelvic region. Very recently, Goodman et al. presenteet al. to be 101 months.[Survival of DSRCT patients after successful treatment is variable. Very rarely, the patient survives beyond 24 months. The results of combination of optimal cytoreductive surgey followed by multiagent chemotherapy resulted in a 3-year survival rate of 58% compared to inoperable patients. However,1 months.In conclusion, in this presentation, we highlight the management of four cases of DSRCT using multimodality treatment. The chemotherapy response was encouraging in all cases, although none of our patients maintained response beyond 6–7 months. In view of the aggressive nature of the disease, patients should be subjected to primary surgery followed by high-dose combination chemotherapy and stem cell rescue. At present, whole-body radiotherapy cannot be recommended due to the risk of bowel complications. Alternatively, radiotherapy should be reserved for palliation of symptomatic disease and to control residual disease after chemotherapy. Further, molecular characterization of DSRCT should be performed in the future to design targeted therapies in this aggressive neoplasm.


Minimal invasive thoracic surgery is growing rapidly and may become the standard of care for certain diseases. Its benefits over traditional surgery, including reduced morbidities and hospital stay, have been well established in several reports. We herein report a case of midesophageal leiomyoma in a patient with MEN I syndrome successfully enucleated by thoracoscopy highlighting the technical details of the procedure. Benign tumors of the esophagus constitute less than 10% of all esophageal neoplasm, On the oLeiomyomas usually present as single lesion and one-half of the cases are smaller than five cm in greatest dimension. It may remain asymptomatic, but when symptoms arise they are usually in the form of dysphagia, pain and weight loss. The preferred methods for diagnosis are barium swallow and endoscopy. In barium swallow the lesion appears as a smooth rounded filling defect with sharp demarcation and on eWe herein present the first case of thoracoscopic excision of esophageal leiomyoma in Saudi Arabia.A 32-year-old Saudi male patient who is a known case of Multiple Endocrine Neoplasia type 1 (MEN1) status post parathyroidectomy, distal panreatectomy and spleenectomy in 2006 was found, on CT screening, to have extra luminal midesophageal mass about five cm in greatest dimension at the level of the carina, compatible with leiomyoma . FurtherUnder general anesthesia utilizing a double lumen tube and in left lateral position, right thoracoscopy was performed after right lung isolation using four ports: 11-mm port is inserted through the sixth intercostal space by blunt dissection at the midaxillary line to provide access for a 10-mm thoracoscope. Three more ports are placed bluntly to provide access for surgery: Five mm port is placed in the posterior axillary line at the fifth intercostal space, 10 mm scope 30 degree is placed in the posterior axillary line at the eighth intercostals space, and a five-mm trocar is placed in the anterior axillary line at the ninth intercostals space for lung retraction.The mediastinal pleura over the esophagus were divided to expose the tumor and the adjacent esophagus. About five-cm of the esophagus below the azygus vein was mobilized and the tumor was located. A four-cm myotomy was performed using combination of hook and endoscopic scissor avoiding injury to the mucosa. After that blunt dissection was performed separating the tumor from the mucosa, followed by applying traction suture to the tumor to aid in tumor elevation as well as in the dissection which was done mostly by blunt dissection and occasionally using sharp dissection. After tumor enucleation, the specimen was placed in a retrieval bag introduced through anterior 10-mm trocar and was delivered through this trocar wound .To ensure that the underlying mucosa is intact, the right thoracic cavity was instilled with normal saline. Size 14F nasogastric tube was inserted proximal to the area of the myotomy and 50-cc of air injected by the anesthetist gently which did not show any leak. The esophageal muscle fibers were approximated using interrupted 2-0 absorbable suture. Size 24F chest tube was introduced through the anterior 10-mm port and directed towards the apex, the trocars were removed under vision, the intercostal muscle fibers were approximated with 2-0 absorbable suture and the skin closed in subcuticular pattern.The patient was kept nil by mouth till next day when contrast swallow confirmed mucosal integrity, after which he was started on oral fluid sand his drain was removed. On the second postoperative day he was on a soft diet and discharged on the same day. He was to be on soft diet for two weeks. Final histology confirmed the diagnosis of leiomyoma.Review of literature showed no reports of simultaneous occurrence of MEN syndrome and gastrointestinal leiomyomas. We think this was an incidental finding rather than true association.Surgical excision of esophageal leiomyoma is advised for symptomatic cases and for asymptomatic cases if the tumor is larger than five cm, if the tumor is increasing in size and if there is mucosal ulceration.6 Esophag2Right thoracotomy is the most common procedure used for excision of midesophageal leiomyoma, while distal third lesions are classically approached through left thoracotomy. Upper midline laparotomy may also be used for tumors near the gastro esophageal junction. Muscle fibers are gently separated and the tumor is enucleated by blunt dissection. There is general agreement on approximation of muscle fibers after enucleation to prevent the formation of pseudo diverticulum; however This approach has been increasingly used after Everitt reported the first successful thoracoscopic enucleation for esophageal leiomyoma in 1992.[Peroperative endoscopy is an important adjunct to the surgery. It helps in locating the tumor, ensuring complete tumor excision and integrity of the esophageal mucosa. Unfortunately we could not use it in our case.A review of literature on thoracoscopic excision of leiomyoma found the outcome excellent with majority reporting complete resolution of symptoms. There was no mortality but morbidities included mucosal tear (13.3%), postoperative mucosal bulging 10%) and pleural effusion (3.3%).10 It off% and pleAlthough some surgeons do not routinely perform contrast swallow postoperatively, we prefer to do it in order to ensure mucosal integrity as well as the adequacy of contrast flow at the enucleation site.Thoracoscopic enucleation for esophageal leiomyoma is safe and feasible. It should be the procedure of choice for lesions less than five cm since it is less invasive and provides shorter recovery with same functional result to thoracotomy.


Primary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.One patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour. Primary liver sarcoma is a rare tumor associated with rapid growth and poor prognosis. The majority of hepatic malignancies are carcinomas with sarcomas representing only 0.1% to 2% of primary hepatic cancers [Our institution is a leading tertiary referral centre for the treatment of hepatic malignancies in the region. We reviewed our cases of primary liver sarcoma and found three patients presenting with leiomyosarcomas (LMS) with an unusually young age distribution compared with previous reports of this disease. We present our cases and discuss the current literature including controversial management strategies such as liver transplantation.From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; with an age range of 20-39 years.The first patient was a 25 year old female who presented with abdominal pain and a palpable right abdominal mass. A computed tomography (CT) scan revealed a 14 × 10 cm hepatic mass occupying segments 5,6,7,8 and compressing the right and middle hepatic veins. A right hepatectomy was subsequently performed. Histopathology revealed a primary hepatic leiomyosarcoma; immunohistochemistry showed positive staining for vimentin, desmin, smooth muscle actin (SMA) and muscle specific actin (MSA). The surgical margins were negative and the tumour was classified as stage I sarcoma under the American Joint Committee on Cancer (AJCC) classification system. The patient received adjuvant chemotherapy however the tumor recurred with distant metastasis and she passed away 22 months post resection.The second patient was a 39 year old male who presented with abdominal pain associated with anorexia, fever and weight loss. A palpable abdominal mass was found on clinical examination. CT scan revealed a 27 × 22 × 17 cm mass in the right hepatic lobe. The lesion showed multiple internal hypodense areas compatible with necrosis. Fine needle aspiration was consistent with leiomyosarcoma. Immunohistochemistry showed positive staining for actin, desmin, and was strongly positive for vimentin. An extended right hepatectomy was performed and histopathological examination revealed a tumor size of 33 × 23 × 20 cm with positive surgical margins. One week post surgery, the patient developed fulminant liver failure secondary to small for size syndrome and passed away 19 days after surgery.The third patient was a 30-year old male who presented with abdominal pain associated with anorexia, weight loss, nausea and vomiting. A CT scan showed a large multi-lobulated lesion measuring 18 × 17.8 × 11.8 cm occupying almost the entire right hepatic lobe  and Epstein-Barr virus (EBV) ,3. We haMatthaei et al reported that survival in most patients with primary sarcoma of the liver is poor. However a long-term survival for more than 10 years after curative surgery (R0 resection) is possible [Diagnosis of hepatic LMS is challenging due to the non-specific nature of symptoms and lack of serological markers. Pre-operative histological diagnosis of liver tumors, particularly hepatocellular carcinomas is controversial due to the risk of needle-track seeding. Only one of our three patients had a pre-operative diagnosis of LMS made by FNA. Hepatocellular carcinoma (HCC) is the commonest primary live tumour and many patients who are diagnosed with hepatic sarcoma are initially treated for presumed HCC. In one of our cases the initial diagnosis was of hydatid disease of the liver which is the commonest cause of cystic liver lesions in this age group in our region.The characteristics of hepatic LMS on CT or magnetic resonance imaging (MRI) are non-specific. CT imaging will invariably show a well-defined hypodense mass with peripheral enhancement with evidence of central necrosis or in some cases a cystic appearance. MRI may show homogeneous hypointensity on T-1 weighted images and heterogeneous hyperintensity on T-2 weighted images .Histological examination of LMS reveals a tumour composed of intersecting bundles of spindle-shaped cells. Immunohistochemistry will show positivity for desmin, vimentin and smooth muscle actin but negative for keratin, S-100 protein and neuron-specific enolase.The standard treatment for liver sarcomas is surgical resection followed by adjuvant chemotherapy however due to the rarity of the disease, there is very little data regarding optimal management and this remains empirical.The efficacy of chemotherapy for LMS in general is unclear, particularly since older series include chemo-resistant gastrointestinal stromal tumors (GISTs) that are now treated with the c-kit inhibitor imatinib. The cytotoxic agents doxorubicin and ifosfamide are commonly used for many subtypes of soft tissue sarcoma both in the adjuvant and palliative setting. In a retrospective study of first-line chemotherapy for unresectable or metastatic LMS from different primary sites the overall response rate was 18%; median progression-free survival and overall survival were 3.8 months and 9.7 months respectively . There iComplete resection of the primary tumour (R0 status) is only potentially curative treatment for primary liver sarcoma. The largest case series of 22 primary hepatic sarcomas reported by Matthei et al found that no patient survived longer than 3 years after incomplete tumour resection .Liver transplantation for primary hepatic sarcoma is controversial. Orthotopic liver transplantation (OLT) is now accepted as a valuable therapeutic option for early, unresectable HCC. Initial results showed high levels of tumour recurrence and disappointing short and long term survival rates however the seminal study by Mazzaferro et al in 1996 culminating in the development of the "Milan Criteria" established OLT as a viable treatment for HCC. These criteria stipulated that patients with a single tumour less than 5 cm or up to three tumors with the largest less than 3 cm with no evidence of vascular invasion, nodal or distant metastasis could be transplanted with acceptable rates of recurrence and overall survival comparable to OLT for benign disease. Transplantation for HCC within the Milan Criteria can achieve 4-year survival rates of 85% to 92% .Liver transplantation for primary hepatic sarcoma has not been as successful as OLT for HCC. Due to concerns regarding needle-track seeding, most patients do not have a pre-operative histological diagnosis and the diagnosis of sarcoma is made on histological examination of the explanted liver. Husted et al reported the outcomes for 19 patients identified as undergoing OLT for primary or metastatic liver sarcoma. The 6 patients transplanted for primary hepatic sarcoma had primary hepatic angiosarcoma and had recurrence of disease at a median interval of 2 months (range 2 -10 months). Median survival was 5.7 months after transplantation (range 2.6-15.4 months) with all patients dying of disease recurrence. For the 13 patients receiving OLT for metastatic sarcoma to the liver, all patients had prior control of their primary malignancy before consideration of OLT and were free of extra-hepatic disease at the time of transplantation. Twelve patients had recurrence of disease after a median of 11.7 months (range 2-90 months), ten patients died of recurrent disease. The three long-term survivors have what is now termed GIST and are maintained on imatinib [There are four reported cases of OLT for primary hepatic LMS. Saint-Paul et al. reported a case of primary hepatic LMS diagnosed post-transplant for presumed HCC who died 15 days post-operatively . In the We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour. We have reviewed the literature and discussed treatment strategies including adjuvant chemotherapy and liver transplantation. Curative resection is the mainstay of treatment for hepatic leiomyosarcoma. In cases primary liver tumors with unusual features we would advocate pre-operative histological diagnosis in order to optimize management. Liver transplantation for hepatic LMS cannot be recommended due to high rates of tumour recurrence however in cases of LMS diagnosed on histopatholgical examination of the explanted liver or LMS arising post-transplant associated with immunosuppression, immune system modulation is an important strategy to consider. The role of adjuvant chemotherapy for hepatic LMS is unclear and remains empirical. In view of treatment advances in the treatment of uterine LMS, patients with non-uterine LMS should be offered treatment within clinical trials whenever possible to improve our understanding of this rare disease and improve outcomes for patients.The authors declare that they have no competing interests.AcS drafted the manuscript, WF and DM participated in the design of the study, NEM and AyS assisted with the collection of data and conceived of the study, MK and AlS participated in the design and coordination of the study. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


S. stercoralis.Intestinal obstruction is a poorly recognized and probably underreported complication of strongyloidiasis. We present herein an unusual case, of complete duodenal obstruction caused by A systematic review of the literature examining the clinical course, diagnostic methods, and outcome of this rare complication of strongyloidiasis was performed.Strongyloides stercoralis infestation. Duodenal obstruction is a rare complication of S. stercoralis infection, with only 8 cases described in the literature since 1970. Most of the patients are males, middle-aged, and the diagnosis was made by duodenal aspirate/biopsy, or analysis of surgical specimen.A 42-year-old woman presented with a 5-month history of abdominal pain, vomit, and weight loss. An abdominal CT scan showed an obstruction of the third part of the duodenum. Segmental intestinal resection was carried out and histopathology examination revealed heavy S. stercoralis infection. The large spectrum of clinical manifestation and lack of classic clinical syndrome make the final diagnosis of strongyloidiasis extremely difficult. A high index of suspicion, mainly in patients from endemic areas, is needed for correct and early diagnosis of this uncommon presentation of Strogyloides stercoralis enteritis.Duodenal obstruction is an unusual, but potential fatal, complication of  Strongyloides stercoralis. The true prevalence of S. stercoralis is likely underestimated because infection is often subclinical [Strongyloidiasis is a parasitic disease, caused by a nematode helminth, clinical -3. Curreclinical . Typicalclinical .S. stercoralis. Additionally, we performed a systematic review of the literature examining the clinical course, diagnostic methods, management and outcome of this rare, but potential fatal complication of S. stercoralis infection.Intestinal obstruction is a poorly recognized and probably underreported complication of strongyloidiasis. Herein, we report an unusual case, of complete duodenal obstruction caused by Strongyloides stercolaris. Inclusion was limited to cases reported in adults, and published in the English language since 1970. All the articles were systematically reviewed and only cases of confirmed duodenal obstruction were included in this report.A review of literature was performed using the MEDLINE database in order to identify articles of duodenal obstruction caused by A 42-year-old woman presented with a 5-month history of recurrent abdominal pain, nausea, post-prandial vomiting, intermittent diarrhea, and a 20 Kg (44 lb) weight loss. Her past medical history was unremarkable, except for an admission for pneumonia in the past year. On physical examination the patient was in poor clinical condition, malnourished, afebrile, with a blood pressure of 100/40 mmHg, pulse of 100 beats per minute and a respiratory rate of 24 breaths per minute. No lymphadenophaty was found. The lungs were clear and the heart was normal on auscultation. Abdominal examination revealed epigastric distention, without guarding or rebound tenderness. The spleen and liver were not palpated and a mild pedal edema was observed.3/L , and platelet count of 600 × 103/μL. Total serum protein and albumin levels were 2.9 g/dL and 1.2 g/dL, respectively. Serum creatinine was 2.5 mg/dL, BUN 118 mg/dL, and potassium 2.8 mMol/L. Liver function tests, amylase and lipase were within normal limits. She had a positive serology for toxoplasmosis (IgM antibody), but negative for HIV, and HTLV-1.Stools tested for occult blood were positive, and negative for ova and parasites. Laboratory evaluation revealed a hematocrit of 39%, white blood cell count of 14.9 × 10A central line was established and fluid replacement was started. Broad-spectrum antibiotics were initiated for a possible intraabdominal infection/sepsis. An abdominal computed tomographic scan showed a marked gastric and duodenal distension, with a possible point of obstruction of the third part of the duodenum Figure . A nasogAt this point we decided to start the patient on total parenteral nutrition and repeat the upper endoscopy in 48 hours. Despite clinical support, 24 hours after admission, the patient presented a significant worsening of the abdominal pain, fever, increasing white blood cell count, and intermittent hypotension requiring additional intravenous fluid bolus. Based on the abdominal CT findings, we suspected of the presence of a complicated submucosal duodenal tumor, such as a primary intestinal lymphoma or gastrointestinal stromal tumor, and decided to take the patient to the operating room.Strongyloides stercoralis . Once these eggs hatch, rhabditiform larvae are released. These larvae can either passed in the stools, continuing the soil based cycle, or can cause autoinfection. The autoinfection occurs when the rhabditiform larvae prematurely become the infective filariform larvae in the intestinal lumen, and penetrate in the intestinal mucosa or perianal skin . In either case the infective larvae migrate to the lungs and restart the cycle previously described [S. stercoralis to persist and replicate within a host for decades, with the longest reported period being 65 years [The complete elucidation of the parasite's life cycle occurred 50 years after its identification. This fact is probably related to the unique and complex life cycle of the parasite. escribed ,3,7. The65 years .The term "disseminated disease" is used to define when the infective larvae migrate, from the intestine, in massive numbers not only to the lungs but to other organs not involved in the normal helminthic life cycle. In disseminated strongyloidiasis, the mortality rate can be as high as 70-90% . SeveralStrongyloides infections are asymptomatic [Strongyloidis infection that usually occurs during an external autoinfection episode. The serpiginous urticarial rash is caused by rapid (approximatelly 15 cm/h) moving of Strongyloides stercoralis larvae from the anal area down the upper thighs [Approximately, half of ptomatic ,3. Cliniptomatic -3. Pulmoptomatic ,2,6. Larr thighs .Strongyloides stercolaris since 1970 [S. stercoralis. First, the obstruction would be related to a severe mucosal edema and inflammation with significant narrowing of duodenal lumen. Second, an extrinsic compression of the duodenum by the superior mesenteric neurovascular bundle could be responsible for the obstructive symptoms. Several mechanisms are proposed to explicate the extrinsic duodenal compression (i.e. superior mesenteric artery/Wilkie's Syndrome) in patients with strongyloidiasis, including severe weight loss, duodenal distention, mesenteric lymphatic dilation, and increase in the diameter of superior mesenteric vessels [Duodenal obstruction is an extremely rare complication of strongyloidiasis, with eight cases reported in the medical literature. Table nce 1970 -18. Two  vessels ,16,19.S. stercolaris hyperinfection [Strongyloides-related ileus [Paralytic ileus is also a potential complication of nfection ,11,20-23Strongyloidiasis is usually associated with anemia, hypocholesterolemia and hypoalbuminemia. Eosinophilia is an inconsistent finding, present in up to 35% during the acute phase, and less frequent in patients with chronic or disseminated disease. Most patients with duodenal obstruction presented low eosinophil count indicating a chronic infection. Eosinopenia and low IgE level have been associated with a poor prognosis, in patients with disseminated disease .Strongyloides-related duodenal obstruction. The diagnosis of strongyloidiasis may be confirmed by the presence of the larvae in the stools. This is an easy performed, broadly available and inexpensive method for detection of the parasite. However, stool examination is relatively insensitive, and diagnostic yield of a single specimen is approximately 30%. The sensitivity of fecal smear could be increased to up to 60%, if five or more stool samples are examined [S. stercoralis is the only helminth that secretes larvae in the stools. Thus, the presence of eggs in the fecal smear is unlikely.Duodenal obstruction may be caused by different diseases, including tuberculosis, primary intestinal lymphoma, Crohn's disease, eosinophilic gastroenteritis and gastrointestinal stromal tumor. Despite extensive preoperative work-up, three out of the nine cases presented in Table examined . Of noteS. stercoralis larvae identification in duodenal biopsies is feasible in 71% of cases [Other methods such as duodenal aspirate or biopsy are more invasive therefore less desirable. Nevertheless, it has been shown that the examination of a duodenal aspirate for ova and larvae is the most sensitive diagnostic procedure, with a false-negative frequency of less than 10% ,25. Endoof cases . In eighS. stercoralis cannot be demonstrated by the standard diagnostic evaluation. Although, indirect hemmagglutination (IHA) and indirect fluorescent antibody (IFA) test have been used, enzyme-linked immunosorbent assay (ELISA) is currently recommended because of its greater sensitivity [In cases of disseminated infection, the parasite can be also identified in sputum, broncho-alveolar lavage, cerebrospinal fluid, skin, urine, and ascites . Serologsitivity ,28,29. Dsitivity ,8.Imaging studies are nonspecific. However, radiological abnormalities restricted to the duodenum and proximal jejunum, on CT scans and upper gastrointestinal series, should alert the surgeon to the possibility of strongyloidiasis. A unique radiographic feature of strongyloidiasis is the reflux of oral contrast into the biliary tree, possibly due to an incompetent sphincter of Oddi caused by severe inflammation of the duodenal wall .Medical treatment should be achieved even in the absence of symptoms, in order to avoid the dissemination of the parasite and minimize the risk of development hyperinfection syndrome. The drug of choice for treatment of strongyloidiasis is ivermectin given at a dose of 200 mcg/kg of body weight daily for at least 2 days ,8,31. InStrongyloides stercoralis hyperinfection.In patients who are not able to tolerate oral treatment, rectal administration of ivermectin or thiabendazole has been suggested ,33. HoweS. stercoralis infection. The large spectrum of clinical manifestation and lack of classic clinical syndrome make the final diagnosis of strongyloidiasis extremely difficult. Therefore a high index of suspicion, mainly in patients from endemic areas, is needed for correct and early diagnosis of this uncommon complication of Strogyloides stercoralis infection.In summary, duodenal obstruction is a rare, but potential fatal, complication of The authors declare that they have no competing interests.All the authors participated in the admission and the care of this patient, the conception, manuscript preparation and literature search. In addition, all authors read and approved the final manuscript.Written informed consent was obtained from the patient's family 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.


A long-term follow up case of hepatobiliary cystadenoma originating from simple hepatic cyst is rare.We report a case of progressive morphologic changes from simple hepatic cyst to hepatobiliary cystadenoma by 11 – year follow up imaging. A 25-year-old man visited our hospital in 1993 for a simple hepatic cyst. The cyst was located in the left lobe of the liver, was 6 cm in diameter, and did not exhibit calcification, septa or papillary projections. No surgical treatment was performed, although the cyst was observed to gradually enlarge upon subsequent examination. The patient was admitted to our hospital in 2004 due to epigastralgia. Re-examination of the simple hepatic cyst revealed mounting calcification and septa. Abdominal CT on admission revealed a hepatic cyst over 10 cm in diameter and a high-density area within the thickened wall. MRI revealed a mass of low intensity and partly high intensity on a T1-weighted image. Abdominal angiography revealed hypovascular tumor. The serum levels of AST and ALT were elevated slightly, but tumor markers were within normal ranges. Left lobectomy of the liver was performed with diagnosis of hepatobiliary cystadenoma or hepatobiliary cystadenocarcinoma. The resected specimen had a solid component with papillary projections and the cyst was filled with liquid-like muddy bile. Histologically, the inner layer of the cyst was lined with columnar epithelium showing mild grade dysplasia. On the basis of these findings, hepatobiliary cystadenoma was diagnosed.We believe this case provides evidence of a simple hepatic cyst gradually changing into hepatobiliary cystadenoma. Hepatobiliary cystadenoma is a rare benign tumor arising from the liver, or less frequently from the extrahepatic biliary tree. Edmondson et al . reporteA simple hepatic cyst was detected in the left lobe of the liver of a 25-year-old man in 1993 . The patient was followed in our hospital, and no surgical treatment was performed although the cyst showed gradual enlargement. The patient was admitted to our hospital due to epigastralgia and for re-examination of simple hepatic cyst in 2004. In 1996 the cyst was unilocular, 6 cm in diameter without calcification, septa or papillary projections as observed by CT Fig. . In 2001Hepatobiliary cystadenoma is a rare benign tumor arising from the epithelium . HepatobIn general, hepatobiliary cystadenoma was described as multilobular cyst with smooth surfaces and the vasculature externally . The tumAs to microscopic features in details, hepatobiliary cystadenoma consisted of following three layers; 1) the epithelial layer of mucin producing columnar to cuboidal cells lining within the cysts; 2) the layer, less than 3 mm in thickness of undifferentiated mesenchmal cells; 3) the outer layer, which was the dense layer with collagenous connective tissue.Tumor size varies from 8 to 20 cm, with a mean of 13 cm . SymptomWe report a case of hepatobiliary cystadenoma with morphologic changes from simple hepatic cyst shown by 11-year follow up imagings. Fortunately, complete radical resection was performed and no recurrence has been observed to date. Complete resection is mandatory surgical procedure, when hepatobiliary cystadenoma showing atypical imaging findings is suspected, or the malignancy cannot be denied.Written informed consent was obtained from the for publication of this case report and any accompany images. A copy of written consent is available for review by the Editor-Chief of this journal.The authors declare that they have no competing interests.NF wrote this manuscript and revised it. MI performed the operation. He conceptualize and recommended me to write this case and advised me to revise it. HI performed the operation and conceptualize and recommended me to write this case. TI performed the operation and conceptualize and recommended me to write this case. SK performed the operation and conceptualize and recommended me to write this case. AS performed the operation and conceptualize and recommended me to write this case. KS participated in the design of this case. JN participated in the design. YF made a diagnosis of this case histologically and participated in the design. All authors read and approved the final manuscript.


Splenic pseudocysts are nonparasitic cyst without epithelial lining. We report this case especially by its way of revelation, its large size and its per operative presentation which needed total splenectomy. To this opportunity, we discuss the diagnostic procedure and therapeutic indications.A twenty-year old Senegalese woman, was admitted with a three-month history of spontaneous abdominal mass associated with a pain. Ultrasonography and CT scan found the giant splenic pseudocyst with a diameter of 20 cm which needed a total splenectomy by median laparotomy.Usually, symptomless splenic cysts are untreated. When surgical treatment is indicated, recommendations are to preserve splenic parenchyma by partial splenectomy or fenestration especially by laparoscopy. Total splenectomy retains some guidance. Splenic pseudocysts are nonparasitic cyst without epithelial lining . Those aA twenty-year-old Senegalese black woman, was admitted with a three-months history of spontaneous abdominal mass associated with a pain. There were no histories of trauma or infection. On examination, there were an abdominal mass in the left hypochondrium and epigastric area. This mass was steady, with a diameter of 20 cm. Hydatid serology was negative and blood formula was normal. Ultrasonography found a voluminous mixed fluid mass without specifying its origin. CT scan found a splenic cyst mass with net lining, regular, without calcifications, without enhancement after contrast injection Figure . Its conManagement of these splenic cyst is threefold: What is the nature of the cyst? Should we operate and what surgery do? Cysts are classified as congenital cyst, pseudocyst, parasitic and tumor cysts. Our case was nonparasitic splenic cysts in the absence of infectious syndrome and non-tumoral on imaging. Martin Fowler , have maSplenic pseudo cysts are benign formations often secondary to the resorption of a hematoma. Asymptomatic forms do not belong to any treatment. When they are symptomatic, partial splenectomy is superior to laparoscopic fenestration in terms of recurrence. Total splenectomy retains some guidance.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MC performed the surgical procedure and reported the case. IK and OK interpreted and analysed the tomodensitometry findings. MD participate to diagnostic and therapeutic decisions. AD and CT have major contribution in writing the manuscript. All authors read and approved the final manuscript.


Gastropleural fistula is a rare condition. We report a case where fistula developed iatrogenically during repeated intercostal drainage tube insertions for empyema. Gastropleural fistula has been reported after intrathoracic gastric perforation in hiatal hernia, traumatic diaphragmatic hernia with later gastric perforation, perforated malignant gastric ulcer at fundus, extension of subphrenic abscess with gastric perforation, pulmonary resection and gastric bypass operations.–5Because of the corrosive actions of gastric juice and with nutritional debility associated with this pathology, early diagnosis and prompt surgical treatment of gastropleural fistula are necessary for satisfactory results.A 35-year-old male developed left-sided tubercular empyema thoracis 9 years back. He was maintained on antituberculous drug coverage. A left intercostal tube thoracostomy was done, but no improvement followed. After 3 months, left-sided decortication surgery was done. The lung did not expand completely and he was advised thoracoplasty, which he refused. For residual empyema, intercostal tube was changed repeatedly, nearly once every 6 months. After changing intercostal tube 2 months back, he suddenly noticed the presence of ingesta and food particles in the drainage bottle. He was afebrile but started losing weight. With the suspicion of esophago or gastropleural fistula, barium study was done, which highlighted the opacified pleural cavity with a fistulous tract from fundus of stomach to intercostal tube via left pleural space Figures  and 2. EHe was advised thoracoplasty and gastropleural fistula closure. At left thoracotomy under general anesthesia, there were dense pleural adhesions; and left pulmonary parenchyma was dystrophic with multiple bronchopleural fistulas. The left hemidiaphragm was raised and after lysis of the adhesions, the gastric fistulous communication of about 2 × 2 cm in left posterolateral part of diaphragm was visualized. The stomach was repaired by continuous monofilament polypropylene suture, and the diaphragm was repaired with continuous monofilament polypropylene suture. Thoracoplasty with resection of four ribs was done. In postoperative phase, minor leak persisted; hence midline laparotomy under general anesthesia was done. Complete separation of gastric fundus from the diaphragm was done, and the fundus rent was repaired by double layer of polygalactin sutures. The diaphragm was repaired with single layer of continuous monofilament polypropylene suture. Feeding jejunostomy was also done. Patient recovered from fistula. Further, rib resection for completion of thoracoplasty is planned.Markowitz and Herter first described gastropleural fistula in 1960. They described causes of gastropleural fistula as intrathoracic perforation of stomach in hiatal hernia, traumatic diaphragmatic hernia with perforation of stomach and intraperitoneal gastric perforation with erosion of subphrenic abscess via diaphragm.1 Other ca34Diaphragm is an effective barrier to spread of infection. Transdiaphragmatic spread of infection occurs by means of spontaneous diaphragmatic perforation. The diagnosis is usually made with contrast radiology, upper GI endoscopy and by testing of pleural fluid. For surgical approaches, both laparotomy and thoracotomy have been described depending on factors as etiology and site of fistula.478 Previ4734Our case is one of this rare diagnosis. Fistula developed likely due to straight and inadvertent forceful insertion of intercostal tube in previous insertion site in seventh left intercostal space. Other significant medical history was decortication surgery for left empyema thoracis 9 years back. To our knowledge, there is no other reported similar case due to this etiology.This case emphasizes that not only subphrenic infection or below-diaphragmatic pathology can erode and lead to gastropleural fistula, but supradiaphragmatic infection, intrathoracic surgery and strong forceful intercostal tube insertion can also result in this condition.This case gives us a word of caution for such a rare entity.


Eccrine carcinoma is a quite rare malignant tumor that typically arises from a normal sweat gland and that features a rather high recurrence rate subsequent to simple excision. Given its rather poor response to adjuvant therapy, wide excision of the lesion with tumor-free margins may offer a reasonable chance for long-term control of this neoplasm.Herein, we report on an unusual case of perianal eccrine carcinoma, initially presenting as a perianal abscess.Even though eccrine carcinomas would appear to be rare, when dealing with recurrent skin tumors or recurrent perianal fistulas, the possibility of eccrine carcinoma should be considered by consulting clinicians. There are three types of sweat glands that occur over the skin surface: eccrine, apocrine and apoeccrine. Eccrine sweat glands are one of the major sweat glands found in humans. Such sweat glands are distributed over the entire body skin surface, with the greatest density being found on the palms of the hands and soles of the feet . EccrineEccrine carcinoma is a rare, malignant sweat gland tumor, generally featuring a slow growth rate and a rather high local recurrence rate . Sweat gA-57-year-old female patient presented at our institution with an apparently slow growing and painful perianal mass, which had produced some serous discharge for the preceding several years. The woman denied any history of constipation or anal bleeding, and she also denied any history of major disease including malignancy. Digital examination revealed a huge (5 × 5 × 5 cm), well-defined solid tumor associated with a skin ulcer, which was located over the left posterior region of the buttock adjacent to the anus. Tracing back her surgical history, this patient had undergone perianal skin tumor excision twice previously, once in 1981 and again in 1992. On both occasions, this was due to the presence of a painful perianal mass.According to her description of the previous perianal masses, they were firm and slow growing, and featured associated skin ulceration Figure . The patLaboratory data, including complete blood count and relevant blood biochemistry, were unremarkable. Under the initial impression of a perianal fistula or perianal skin tumor, incision biopsy was performed. The biopsy specimen was sent for frozen section examination, which revealed malignant neoplasm with myoepithelial-like proliferation. Following this histopathology report, en-bloc excision was undertaken in order to excise the tumor with a gross margin of 2 cm Figure . The posSubsequent to surgery, metastatic work-ups revealed multiple nodules over both of the patient's lungs, presumably secondary to the primary malignant neoplasm. Other examinations, including immunological tumor markers , sputum cytology and gynecological ultrasonography, were performed and all produced unremarkable results. The final pathology report suggested the presence of an eccrine adenocarcinoma. Microscopically, lesion sections revealed hyperchromatic neoplastic cells arranged in solid nests, anastomosing trabeculae with ductular formation Figure , a syrinThe tumor cells were characterized by the presence of mild to moderate nuclear pleomorphisms, occasional distinct nucleoli, not uncommon mitotic activity, and a variable amount of eosinophilic, basophilic or clear cytoplasm featuring focal tumor necrosis. Perineural invasion and lymphovascular space permeation were also noted Figure . ImmunohSurgery is the first choice for management of such eccrine carcinomas since this type of carcinoma typically features a poor response to chemotherapy and/or radiotherapy . HoweverFor our patient, when dealing with the current tumor, we intended to review the histopathology slides prepared in 1992 from the patient's previous episode in order to compare presentations and determine whether any mistake may have been made previously regards microscopic interpretation of the prepared tissue slides.Unfortunately, the relevant slides had been lost during transportation several years previously and we were denied such an opportunity. Following our review of the literature, as far as we were able to determine, there is one case report about a patient whose eccrine porocarcinoma could possibly have arisen from a long-standing benign eccrine poroma . OtherwiAs for the suspicious nodules present on this patient's chest X-ray, we suggested thoracoscopic biopsy as being appropriate and, subsequent to metastasis having been confirmed, concurrent chemo-radiotherapy. However, this was refused by the patient.Even though eccrine carcinoma is a clinical rarity, when dealing with recurrent skin tumors or recurrent perianal fistulas, the possibility of eccrine carcinoma should be considered by consulting clinicians. Such a tumor can be slow growing but does feature the potential for recurrence, as well as distal metastasis . A high The author(s) declare that they have no competing interests.THL: Primary author, preparation and submission of manuscriptHHCL: The attending surgeon of this case and critically for important intellectual contentSCC: Contributing authors and carried out the surgical procedures with HHCLAll authors have read the final manuscript and agree to its publication.


Crohn's disease (CD) can affect any part of the alimentary tract from the mouth to the anus, with most common site being the terminal ileum.A child suffering from undiagnosed Crohn disease (CD), presented with an acute abdominal obstruction due to a large pseudopolyp in the jejunoileal area. At laparotomy, a jejunoileal segment of 45 cm, containing multiple areas of damage to the small intestine, was excised and a primary end – to – end anastomosis was performed.The coexistence of an intestinal pseudopolyp with undiagnosed Crohn's disease may be the cause of acute abdominal obstruction in children. Crohn's disease (CD) can affect any part of the alimentary tract from the mouth to the anus, with most common site being the terminal ileum. Bowel obstruction is a well-known complication of CD, usually as the result of stricture formation, or more rarely as mechanical obstruction. Intestinal obstruction due to a large pseudopolyp is a rare event in CD ,2.A 12-year-old boy was brought to the emergency department with acute abdominal pain lasting 12 hours, and associated abdominal distension, absolute constipation for two days, vomiting and fever of 38.5°C. When examined he had general abdominal tenderness. White blood cell count was 17.5 k/ml with 85.5% neutrophils, hemoglobin was 10.9 gr/dl, hematocrit 34.7% and platelets 820 k/ml. Abdominal X-rays showed air-fluid levels. Abdominal ultrasound examination revealed a solid intraluminal pattern  declare that they have no competing interests.EC, EM, DF operated on the patient, MC and SK performed the diagnostic and histological examinations, MP, NP and SR participated in the follow up and the diagnostic strategy. All authors participated in writing the case report and revising the draft.Written informed consent was obtained from the patient and his parents for publication of this Case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A case of low-grade intraosseous fibrosarcoma of the mandible in a 9-year-old girl is described. The patient underwent pre-surgical chemotherapy which was abandoned as unsuccessful after two cycles. Radical tumour resection and mandibular reconstruction with a titanium bar were performed 3 months after diagnosis. No adjuvant therapy was given and lymph node dissection was not performed. No signs of recurrences or metastasis have been observed after a follow up time of 3 years so far. This article is presented to document the rarity of fibrosarcomas in the jaws of children and emphasizes the possible changes in the appearance of radiological imaging under tumour progression. Fibrosarcoma is a malignant neoplasm of mesenchymal origin. Its occurrence in the jaw especially in childhood is very rare. An infantile and an adult form are described showing identical histopathological features but differing in survival prognosis. The presented case describes an fibrosarcoma originating in the mandible of a young girl. Of special interest is the change in radiological appearance of the tumour during treatment.3) at the right mandibular angle, which was noticed by the parents 2 months before first presentation. There was no ulceration of the overlying mucosa. Mouth-opening was reduced to 32 mm due to pain. Paraesthesia was evident in the right lower lip. Radiological examinations with panoramic radiography . Pulmonary metastasis appeared to be a possible explanation for this, leading to initial treatment with neoadjuvant chemotherapy with cisplatin/doxorubicin in the first cycle and cisplatin/ifosphamide in the second cycle (according to the protocols Euramos-1/COSS and COSS 04). A computed tomography scan after the chemotherapy revealed no change in the initial pulmonary finding, whilst the primary tumour in the right mandibular angle showed progression and a striking gain of tumoural calcification (Figure. A 9-year-old girl was referred to the own department with a hard, slightly painful, well-circumscribed swelling (4 × 4 × 5 cm Figure.  and compA hemimandibulectomy with exarticulation of the right condyle and temporary reconstruction with titanium osteosynthesis plate and condylar prosthesis was performed Figure. . The his3 Years after the primary operation the patient shows no sign of recurrence or metastasis. The pulmonary finding shows no change or growing.Fibrosarcoma is a very rare malignancy with possible occurrence in the whole head and neck region . It accoth and 6th decade with equal gender distribution [rd decade [rd year of age. Although infantile and adult fibrosarcoma are histologically identical, the infantile form carries a much more favourable prognosis. The infantile form meastasizes rarely and has a natural history similar to that of fibromatosis [The mean age for the occurrence of fibrosarcoma is between the 2d decade . The aduomatosis ,6.Typically, the tumour presents with swelling, associated with pain and paraesthesia ,3,8. RadFibrosarcomas are graded from low to high malignancy after the FNCLCC grading system, depending on the number of mitotic figures, tumour differentiation and the presence of tumour-necrosis ,9,10.The prognosis is highly dependent on the tumour-grading and the success of complete resection ,2,4,6,11The authors declare that they have no competing interests.MG and FGD analysed the case, reviewed all patient data and drafted the manuscript. The operation planning and the operation were performed by WAW and MG. JM-H carried out the histological analysis and contributed to the writing of the final version. MG, FGD, WAW and OD contributed substantially to discussion and in writing of the paper. All authors reviewed the paper for content and contributed to the writing of the manuscript. All authors approved the final report.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Small cell carcinoma of the gallbladder is a rare disease process with approximately 40 cases reported in the literature. It is most often found in elderly female population and is associated with cholelithiasis and cigarette smoking. A multidisciplinary approach to treatment with wide surgical resection and adjuvant chemotherapy is the current standard of care. Notwithstanding prompt medical intervention, it is a disease with a poor prognosis. The pathology is characterized by early metastases and extensive local invasion. Herein, we report two cases of small cell carcinoma addressed at our institution. In both cases, a radical resection was performed with subsequent referral to oncology for additional therapy. Approximately 7000 new cases of gallbladder carcinoma are diagnosed every year . It is aA-47-year-old male presented to an outside hospital with a 48-hour history of epigastric and right-upper quadrant abdominal pain. A review of systems was grossly negative at presentation. An RUQ ultrasound and MRI performed revealed a heterogeneous mass arising from the gallbladder measuring 7.5 × 5.4 cm and extending into segment 4B of the liver . Liver fThe patient was taken to the operating room for a laparoscopic exploration and surgical resection. Exploratory laparoscopy yielded no evidence of metastatic disease. The gallbladder was excised en bloc with a subsegmental liver resection of segment 4B. Pathology was consistent with a poorly differentiated neuroendocrine carcinoma with extensive lymphovascular invasion Figures  and 3. TThe patient recovered from the intervention without complication. He has since received a six-week course of 54 Gy of radiotherapy with a concurrent chemotherapy regimen of cisplatin and etoposide. Currently, this individual is eight months out from his initial operation and is doing well.A 41-year-old female presented to an outside institution with symptoms consistent with biliary dyskinesia for which an uncomplicated laparoscopic cholecystectomy was performed. The pathology returned with an incidental finding of a well-differentiated neuroendocrine carcinoma of the cystic duct. The mass was 0.4 centimeters in largest diameter with the proximal margin of the cystic duct positive for malignancy. An MRI and abdominal CT scan performed at that time were negative for any evidence of residual tumor or metastatic disease. She was subsequently referred to this institution for additional surgical treatment.Following the completion of a negative metastatic work up with laboratory analyses and an Octreoscan, the patient was taken to the operative suite for re-exploration. The cystic duct remnant was resected at its origin, and a wide portal lymphadenectomy was completed. When the frozen pathology returned with no evidence of residual tumor, the operation was concluded.The patient tolerated the procedure well. Pathology findings were negative for any additional tumor. She has since been treated with adjuvant chemotherapy by medical oncology. A repeat octreotide scan two months postoperatively documented no recurrent or metastatic disease.Gallbladder carcinoma is the fifth most common gastrointestinal malignancy with 7000 new cases diagnosed in the United States each year . The preNeuroendocrine carcinomas represent an even smaller percentage of the general surgeon's practice. Carcinoid tumors comprise the majority of this pathology, with as many as 68% originating from the gastrointestinal tract . The varThere have been limited discussions of this pathology in the literature to date, principally in the form of case reports or small case series. It is a disease that most commonly affects an elderly, female population . SimilarDue to the scarcity of this neoplasm, diagnostic criteria in the form of radiologic findings and preoperative tumor markers are poorly described. Furthermore, given the insidious nature of the mass, diagnosis can be difficult without a histologic analysis. For instance, in the first patient, the MRI was notable for a large, infiltrating mass. There were no notable abnormalities in the laboratory work up including CEA and CA19-9. In the second subject, it was not until after the therapeutic cholecystectomy for acute cholecystitis that the mass was incidentally discovered. Even with imaging, it is often not until the histopathologic analysis is complete that the diagnosis of a neuroendocrine tumor is reached.Therapeutic options for this pathology are often limited due to the advanced nature of the disease at diagnosis. Surgical excision remains the best option for a cure. However, the necessity of a radical resection and the extent of surrounding tissue removed are debated in the literature . In thosGallbladder neuroendocrine carcinomas remain an exceptionally rare malignancy with a strikingly poor prognosis. Early diagnosis with prompt surgical intervention provides the patient with the best long-term outcome. Adjuvant chemotherapy provides a definable survival advantage to the patient but is without a well-defined standard of care protocol.


Leiomyosarcomas typically originate within smooth muscle cells. Leiomyosarcomas arising from the adrenal vein are rare malignancies associated with delayed diagnosis and poor prognosis. The most common vascular site of origin is the inferior vena cava.This is a 64-year old woman who presented with a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the inferior vena cava (IVC) and the right atrium. Biochemical evaluation excluded a functional tumor of the adrenal gland, and multiple tumor markers were negative. We present the novel use of deep hypothermic circulatory arrest (DHCA) in the resection of an adrenal vein leiomyosarcoma extending into the right atrium. The patient remains free of disease ten months after surgery. DHCA afforded a bloodless operative field for optimal resection of disease from within the IVC.The diagnosis of leiomyosarcomas of the adrenal vein is one of exclusion and involves preoperative radiological imaging and biochemical evaluation to exclude other functional tumors of the adrenal gland. Aggressive surgical resection is associated with improved survival and may be best achieved via collaboration among different surgical subspecialties. Leiomyosarcoma is a soft-tissue tumor that differentiates from smooth muscle. Primary leiomyosarcomas of vascular origin are relatively rare and frequently arise within the IVC -3. RarelA 64-year old woman who presented with a 9-month history of persistent, non-productive cough, bilateral lower extremity edema, and multiple spider angiomata extending from umbilicus to ankles. The patient was otherwise healthy with no significant past medical, surgical, or family history. She denied recent weight loss, fevers, chills, nausea, vomiting, abdominal or back pain, and she had no difficulty with ambulation. Physical exam also was significant for hepatomegaly extending 4 fingerbreadths below the costal margin. There was no jaundice or virilization. Routine laboratory studies were unremarkable. Computed tomography (CT) scan revealed a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the IVC and the right atrium  revealed evidence of a filling defect within the most inferior portion of the right atrium at the confluence with the IVC. Twenty-four hour urine collections for cortisol and catecholamines were normal, as were serum aldosterone, renin, potassium, and ACTH levels. Multiple tumor markers also were normal, including dehydropepiandrosterone sulfate (DHEA-S), lactate dehydrogenase (LDH), carcinomembryonic antigen (CEA), alpha-feto protein (AFP), CA-125, and CA-19-9; only an elevated CA-125 was identified . There was no evidence of metastases on chest CT, brain MRI, and bone scan.The differential diagnosis included adrenocortical carcinoma, renal cell carcinoma, retroperitoneal sarcoma, or hepatoma. The patient was brought to the operating room for a planned adrenalectomy, possible right nephrectomy, cholecystectomy (for cholelithiasis), and tumor resection via IVC cavotomy under DHCA. Diagnostic laparoscopy revealed no evidence of peritoneal or hepatic metastases. Exploratory laparotomy was performed through a midline incision, followed by a Köcher maneuver. The adrenal lesion was noted to be separate from the right kidney. Intraoperative ultrasound confirmed that the tumor did not involve the caudate lobe of the liver.A median sternotomy was performed, and the diaphragm was divided in the midline anteriorly. The adrenal gland was mobilized from the liver, and the IVC was fully exposed. The patient was then placed on cardiopulmonary bypass with DHCA, and her core body temperature was lowered to 19°C. The IVC was incised, and intramural tumor was dissected free from the walls of the IVC and excised en bloc with the adrenal gland Figure . There wThe patient required pharmacologic cardiovascular support for the first 12 hours postoperatively, but was awake within 24 hours. She was extubated on the morning of first postoperated day and displayed no neurocognitive deficit. The balance of her postoperative course was complicated by the development of a symptomatic pulmonary embolus, diagnosed by spiral CT of the chest. An ultrasound of the IVC also was performed to exclude tumor embolus; no evidence of thrombus was seen. She was discharged on 12th postoperative day on oral anticoagulation. Pursuant PET-CT showed no evidence of uptake in the lungs, providing further confirmation that this was a pulmonary embolus, and not tumor thrombus.®), but showed strong reactivity for desmin and smooth muscle actin (Dako®)  Figure . This fi) Figure .A leiomyosarcoma of the adrenal vein was first reported in 1981, in a 50-year old patient with a 12 cm leiomyosarcoma arising from the left adrenal vein . Since tThe preoperative diagnosis of a leiomyosarcoma was not established in any of the previously reported cases. However, while the exact etiology of an adrenal tumor may not be known, biochemical evaluation is warranted to exclude a functional tumor that may require preoperative medical management, i.e. alpha blockade. Excluding two historical cases identified at the time of autopsy, all tumors were ≥ 10 cm and diagnosed at the time of surgical resection, underscoring that there are no tumor markers or imaging characteristics specific to this diagnosis -10. Outcet al reported the use of combination chemotherapy (including adriamycin) and external beam radiation to palliate bone pain in a patient with metastatic disease arising from an adrenal vein leiomyosarcoma [Complete surgical resection is the mainstay of treatment for adrenal vein leiomyosarcomas, as there is little evidence to support the role of chemotherapy and/or radiation. Lack osarcoma . Patientosarcoma ,10.et al reported a right adrenal leiomyosarcoma that extended via IVC into right atrium. Tumor resection, including adrenalectomy, nephrectomy, IVC thrombectomy, and pulmonary artery thrombectomy, was approached via a midline abdominal incision, median sternotomy, and cardiopulmonary bypass [Leiomyosarcoma of the adrenal vein can extend into the IVC or the right atrium ,10. In aTo our knowledge, this is the first reported case of the use of DHCA for the resection of an adrenal vein leiomyosarcoma. The use of DHCA for resection of other retroperitoneal tumors, including renal cell carcinomas and adrenocortical carcinomas, has previously been reported -13. TotaA comprehensive review of the medical literature reveals that the predominant subtype of leiomyosarcoma arises within the IVC. Still, there have been fewer than 300 reported cases since the first description by Pearl in 1871. In 1992, the International Registry of Inferior Vena Cava Leiomyosarcomas was instituted, with 218 registered patients . LeiomyoFirst-line treatment for leiomyosarcomas of the adrenal vein is radical resection with the goal of negative margins. Preoperative evaluation should include imaging to delineate the extent of disease and exclude metastases and biochemical evaluation to rule out catecholamine excess from a functional adrenocortical carcinoma or malignant pheochromocytoma requiring preoperative alpha-blockade.  Collaboration among multiple surgical subspecialties might be beneficial, and the novel role of DHCA described here should be considered in the surgical management of these tumors.The author(s) declare that they have no competing interests.TSW and JAS made substantial contributions to the drafting of the manuscript. RRS, JE, and TO contributed significantly to patient care. JAS was the surgeon-of-record.All authors read and approved the final manuscript.


A fit, 57-year-old man admitted with chest pain and haemoptysis turned out to have a primary tumour in the left lung.In this 57-year-old Caucasian man, the diagnosis followed a computed tomography scan and histopathological evidence gained post-resection. The biopsy showed a uniform, spindle shape with focal pleomorphism which was suggestive of lung carcinosarcoma.The authors report this case in literature and discuss how a rare malignant tumour can be found in those presenting with trivial chest symptoms. It is a rare tumour, accounting for 0.3% of primary lung malignancy. The diagnosis is made through histopathological evidence. The patients presenting with this commonly undergo resection.The description of lung carcinosarcoma was developed in the early 20In this report, we present a rare case of a fit, 57-year-old Caucasian man who was admitted with a history of exposure to asbestosis. He presented with chest pain and frank red haemoptysis. For 2-3 weeks he had been complaining of a mild, dry cough and breathlessness on exertion. His other medical history included hypertension, gastritis and migraine. Physical examination revealed decreased air entry in the left upper lobe. The rest of the examination was normal. His chest X-ray  showed ath to the 7th decade of life [This is a rare malignant tumour which has a mixture of carcinoma and sarcoma consisting of differentiated mesenchymal elements . Regiona of life  and therLung carcinosarcoma is a rare malignancy with a poor prognosis. It accounts for 0.3 % of pulmonary malignancies ,5 and itFor patients with pulmonary carcinosarcoma, the treatment of choice with a clear margin is complete resection . The rat


We wish to report on a rare cause of dysphagia; oculopharyngeal muscular dystrophy (OPMD). It is a late adult onset autosomal dominant form of muscular dystrophy that constitutes as a rare diagnosis for any place outside of Canada and first case in southern Germany.We report the medical odyssey of a 57-year old male Caucasian patient. He was referred at our hospital for further clarification of a progressive dysphagia, which, at first view, was thought to be tumor related due to the patient's typical anamnesis.The present report outlines the importance of considering this rare disease for general medicine practitioners as well as head and neck specialists as a differential diagnosis for swallowing disorders with, even at second view, uncertain cause. A 57-year old Caucasian male patient with acute pneumonia and a progressive dysphagia was referred to our department. He reported episodes of painless swallowing difficulties, associated with xerostomia, hoarseness and an uncomfortable sensation of a mass in the upper esophagus. His general medical condition includes type II diabetes, chronic bronchitis, chronic gastritis, urate nephropathy with a history of nephrolithiasis and coronary artery disease. The patient reported daily alcohol and nicotine abuse over a period of 40 years.On physical examination he showed a distinct bilateral eyelid ptosis combined with a mild facial weakness. Wet voice and an insufficient cough reflex after solid and liquid intake followed by consecutive aspiration were observed. Laryngoscopy showed an incomplete glottic closure and impaired vocal cord mobility. Video-radiological barium examination revealed intra- and- postdeglutive aspiration Figure . ContrasWe performed a panendoscopy under general anesthesia and esophagus-gastroscopy, suspecting a malignant process. Endoscopic findings neither confirmed a tumorous process nor revealed any other obvious cause for the progressive dysphagia. Therefore a deep incision biopsy of the preepiglottic space was performed, but no suspect structures other than fat tissue were noted. Histopathological evaluation of the preepiglottic mass confirmed solely fatty tissue without malignant pathology.Family history revealed that he has eight siblings, four of which developed similar symptoms at around 50 years of age. One brother has only mild dysphagia. Furthermore the patient's father, one paternal uncle, one paternal aunt and her son suffer from dysphagia and eyelid ptosis. Figure  The patiConsidering the family history of swallowing disorders, we suspected a hereditary cause of the dysphagia and initiated genetic counseling. DNA testing revealed a heterozygous (GCG)10 expansion of the (GCG)-mini-repeat, located in exon 1 of the poly(A) binding protein, nuclear 1gene (PABPN1), thus verifying oculopharyngeal muscular dystrophy Figure .After the diagnosis was confirmed and therapeutic options were discussed, the patient refused a cricopharyngeal myotomy. Therefore a percutaneous endoscopic gastrostomy (PEG) placement was carried out to improve the overall nutritional situation. After a period of five months, he reported on unsteady functional improvements of his swallowing. Other neurological functions remained unchanged. The PEG probe was left in place since sufficient oral deglutition did not occur.Patients suffering from multiple ailments are common in ENT hospitals and many exhibit dysphagia at some point. Especially in patients with long periods of alcohol and nicotine abuse, dysphagia is mostly related to malignant processes of the hypopharynx, larynx or esophagus. This patients underwent a series of hospitalizations simply because physicians would not think of a disease as rare as OPMD as a cause for dysphagia in Germany.Most cases and families suffering from OPMD are located in Canada  among thth to 6th decade with slowly progressive eyelid ptosis and dysphagia due to impaired levator palpebrae and pharyngeal muscle function. In most cases the eyelid ptosis is the first symptom and is almost always closely followed by a progressive dysphagia. At a later stage and as the disease evolves, extraocular and facial muscles may also become affected. However, eyelid ptosis is generally recognized as the first and cardinal symptom. In a minor percentage, patients develop weakness and atrophy of the tongue, pelvic girdle muscles and to a lesser extent the shoulder girdle muscles [OPMD usually manifests in the 4 muscles .The diagnosis can only be finalized by DNA testing of CGC triplets. To date there is no causal treatment for this disease. A high-protein diet is recommended for optimal nutritional balance. Aspiration pneumonia, as in the reported case, is a frequent complication; therefore, early medical supervision should be sought. At this time, only surgical treatments to correct the functional aspects of OPMD is available . SurgicaOPMD: Oculopharyngeal Muscular Dystrophy; ENT: Ear Nose Throat; PEG: Percutaneous Endoscopic Gastrostomy; GCG: Nucleotide Sequences; PABPN1: Gene name: poly(A) binding protein, nuclear 1.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BK as the patient's physician analyzed and interpreted the patient data, ZM performed the genetic screening, BA performed radiological analysis.


Sir,We describe a case of Burkitt’s lymphoma (BL) of the small intestine diagnosed on ultrasound-guided fine needle aspiration cytology (FNAC). BL is a high-grade lymphoma usually involving the mandible and the intestines. An early diagnosis is paramount for prompt and effective management. FNAC forms a rapid and effective tool towards an early detection of these lymphomas. A 5-year-old boy presented with abdominal discomfort and progressively increasing abdominal mass. The computed tomography (CT) scan revealed markedly thickened bowel loops with multiple enlarged lymph nodes. There were well-defined hypoechoic nodular lesions in both the kidneys. A suspicion of lymphomatous origin was raised. Ultrasound-guided FNAC was performed through the thickened bowel. Smears were stained with Giemsa and Papanicolaou stains. Many monomorphic, round to oval single cells were identified. These cells displayed a high nucleo-cytoplasmic ratio, vesicular chromatin and prominent cytoplasmic vacuolization . Many lyet al.[et al.[The small intestine is a common site of BL in children and is associated with multisystem lesions. Abdominal mass, bone marrow and central nervous system involvement are poor prognostic markers. Total tuet al. used FNAl.[et al. studied


Sclerosing mesenteritis is a rare disease resembling a mesenteric tumour. We present here a case of sclerosing mesenteritis that affected both the large and the small intestine of the patient. Therapeutic and diagnostic issues are discussed.A 62-year-old man with a history of non-Hodgkin lymphoma presented with fatigue, a palpable tender abdominal mass and clinical signs of progressing intestinal obstruction. The preoperative evaluation failed to prove recurrence of the lymphoma or any other definite diagnosis. A laparotomy was performed through a midline incision. The mesentery resembled a tumour-like thickened and fibrotic mass. Abundant, rigid intestinal loop adhesions were observed. Diffuse fibrotic infiltration of the ileum and of the sigmoid colon, which obviously affected the intestinal vascular supply, were identified. A right colectomy and partial sigmoidectomy were performed. Pathological evaluation revealed extensive myofibroblastic reaction of the mesentery with accompanying loci of fat necrosis and areas of inflammation. A diffuse fibrotic infiltration that focally showed a ground-glass appearance was observed. The post-operative course was complicated by respiratory insufficiency and infections and the patient died 2 months after the operation.Sclerosing mesenteritis that affects both the small and the large intestine is extremely rare. The disease is characterized by myofibroblastic reaction, fat necrosis and diffuse fibrosis of the mesentery. Pathological confirmation may be required for definite diagnosis. If the disease is characterized by severe and diffuse fibrosis, then the application of surgical therapy may be problematic. Sclerosing mesenteritis (SM) is a rare inflammatory mesenteric disease characterized by chronic fat degeneration and fibrosis; a disease that has become of clinical interest during recent years ,2. Some A 62-year-old Caucasian man of Greek origin was admitted to our hospital with a 1-month history of progressive fatigue, intermittent abdominal pain, weight loss, nausea and vomiting. He was diagnosed with a moderately malignant non-Hodgkin B-cell lymphoma 7 years previously. The patient had undergone chemotherapy for a 5-year period followed by a complete remission of the disease. His regular follow-up showed no evidence of recurrence.Physical examination revealed a palpable tender mass in the left abdomen and scarce abdominal sounds. The rectal examination was negative for blood and laboratory findings were normal. The abdominal X-ray revealed dilated intestinal loops and air-fluid levels Figure . ComputePathological evaluation revealed an extensive myofibroblastic reaction of the mesentery with accompanying loci of fat necrosis and areas of inflammation Figure . Also, iPost-operatively, the patient exhibited respiratory insufficiency, thus he was admitted to the intensive care unit. His clinical picture gradually deteriorated and he became septic. Despite prompt antibiotic administration, he died 2-months after the operation.MP represents an idiopathic inflammatory mesenteric disorder. The disease is known in the literature with many synonyms: mesenteric manifestation of Weber-Christian disease, mesenteric lipodystrophy, mesenteric lipogranuloma and retractile mesenteritis -8. An inThere are fewer than 300 cases of SM reported in the literature since the condition was first described in 1924 . The majThe pathogenesis of SM remains unclear. Autoimmune disorders were suggested as a possible mechanism, while other studies suggested that infection, trauma, ischaemia, cold, different drugs, vasculitis, vitamin deficiency or prior surgery could be involved in the pathogenesis ,7,14. AnThe diagnosis of SM is usually made by biopsy at laparotomy. The presence of a single, multiple or diffuse mass-like inflammatory lesion in the mesentery, together with a histological confirmation of fat necrosis and inflammatory reaction or fibrotic infiltration in the mesenteric lesions, strongly suggests the diagnosis of SM ,3,6,9. MSM is treated largely empirically. Corticosteroids, colchicine, azathioprine, thalidomide, cyclophosphamide and anti-inflammatory agents were suggested as possible therapeutic agents; however, no definite results were documented ,3,4. RadSM that affects both the small and the large intestine is extremely rare. The disease might resemble a mesenteric tumour based on clinical, radiological and gross characteristics. Histological confirmation is almost always required. Medical therapy is empiric and surgical therapy remains controversial. If the disease is characterized by severe and diffuse fibrotic infiltration, then the surgical results may be obscure.MP: Mesenteric panniculitis; SM: Sclerosing mesenteritis; CT: Computed tomographyWritten informed consent was obtained from the next of kin of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SH and RH both participated in the surgical interventions, contributed to the review of the histological section of the case and drafted the manuscript. DK participated in the medical interventions, took the photographs, undertook the literature review and helped draft the final version of the manuscript. AK participated in all medical interventions and helped draft the final version of this manuscript.All authors read and approved the final manuscript.