The aim of this study is to increase the understanding of pneumatosis cystoides intestinalis (PCI) and its incidents.We report here a case of PCI in an 88-year-old man with a provisional diagnosis of perforated viscus and possible ischaemic bowels based on CT findings of pneumoperitoneum. The patient was found to have extensive PCI on his small bowels. We then systematically search the PubMed database for case reports for articles containing ‘pneumatosis intestinalis’ in their titles or key words.The study group consisted of 52 cases on PCI from the period of 2010–2014 with the focus on the adult population. The youngest patient was 18 years old and the oldest was 91 years old. The mean age was 60.4 years . There were 27 (52 %) females and 25 (48 %) males. The most common symptoms were abdominal pain (79 %) followed by nausea/vomiting (27 %) and abdominal distension (19 %). CT imaging was the most common investigation modality used (94 %). Three (6 %) of the patients had laparoscopic treatment while 20 (38 %) had laparotomy. Thirty-six (69 %) of them recovered uneventfully while 9 (17 %) of the patients died.Although there have been more case reports published on PCI in the recent years, the understanding of this condition remains in the infancy stage. PCI can be difficult to diagnose and can be easily misdiagnosed as pneumoperitoneum in an acute abdomen. Often it is identified incidentally during operation. Asymptomatic PCI should be treated conservatively, while emergency laparotomy should be reserved for life threatening abdominal pathology. Pneumatosis cystoides intestinalis (PCI) is an uncommon condition where intramural gas cysts are distributed in the small bowel, although the large bowel may also be involved. There is no clear etiology with several proposed theories including mechanical, pulmonary, and bacterial. PCI does not usually cause symptoms and is often incidental on radiology imaging or intra-operative finding. We present a case of an 88-year-old man who was transferred from a regional hospital to a tertiary hospital with a probable diagnosis of perforated hollow viscus.An 88-year-old man presented to a regional hospital with 3 days of generalised abdominal pain with nausea, vomiting, and obstipation. His past medical history included prostate carcinoma treated with radiotherapy, Hartman’s procedure, and multiple episodes of small-bowel obstruction requiring bowel resection, atrial fibrillation, aortic stenosis, hypertension, and cerebrovascular disease. A computerised tomography (CT) scan performed at the regional hospital showed diffuse intramural gas with suspected pneumoperitoneum Fig. –d. The pUpon arrival at the tertiary hospital, he was haemodynamically stable. The patient had mild generalised abdominal pain, with a soft distended abdomen. There were no signs of peritonism. The stoma was healthy looking but had no output. The patient was noted to have a nasogastric tube with minimal output and an indwelling catheter with adequate urine output. Based on CT images of pneumoperitoneum, he was taken to the theatre with a provisional diagnosis of perforated viscus and possible ischaemic bowels.At laparotomy, there was 1.5 L of serous peritoneal fluid with no faecal contamination. There were multiple adhesion bands causing small bowel obstruction with transition point in distal ileal segment; numerous segments of air-filled cysts and large jejunal diverticuli were identified on an incidental finding. Volvulus caused by the adhesion bands was released Fig. –d. The bSeven of the cystic lesions from the small bowel were excised for histological analysis. Histology was consistent with PCI. Macroscopically, the outer surface of the cystic masses was multi-lobulated, thin, and shiny. The outer surface also showed dilated blood vessels; sectioning of the cystic masses revealed multiple gas filled cystic spaces 1 to 20 mm in diameter Fig. .Fig. 3ReMicroscopically, the specimens have variable-sized cystic spaces lined by histiocytes and multinucleated foreign body giant cells Fig. –c, on a A search was performed through PubMed database in the last 5 years (2010–2014) on case reports containing ‘pneumatosis intestinalis’ in the titles or key words. Only articles written in English and available in full were included in our analysis.n = 41, 79 %) followed by nausea and vomiting , abdominal distension , per rectal bleeding or bloody stools , diarrhoea , and constipation . During examinations, 6 patients had a soft abdomen with no tenderness while 15 patients were found to have peritonitic signs. CT investigations were performed on 49 (94 %) patients while abdominal X-rays were performed on 17 (33 %). Colonoscopy was performed on 10 patients as a form of investigation. Twenty-seven (52 %) patients were treated conservatively with medical therapy, such as antibiotics, hyperbaric oxygen therapy, bowel rest, and observation. Three (6 %) patients underwent laparoscopy, while 20 (38 %) received laparotomy. Only 1 patient who had laparoscopy needed to be converted to laparotomy. In terms of outcome, 36 (69 %) recovered uneventfully. Nine (17 %) of them died, of whom 2 died prior to surgery, and the remaining 7 died postoperatively. Amongst the 9 deceased patients, 4 died from multi-organ failures, 2 died from sepsis and septic shock, 2 died from pulmonary complications, and 1 died from hypovolemic shock after surgery with autopsy showing intra mural haemorrhage. Both the patients who died from sepsis and septic shock were deceased before surgery.The study group included 52 case reports relating to the adult populations. There were 27 (52 %) females and 25 (48 %) males. The mean age was 60.4 years . Thirteen patients (25 %) fell within the age group of 60–69 years old. Nine patients (17 %) were between 70–79 years old and another 9 patients (17 %) were between 80–89 years old. The most common symptom was abdominal pain (PCI, also known as intramural gas, refers to gas within the bowel wall. Other names include pseudopneumatosis, intestinal emphysema, and bullous emphysema of intestines . In our Based on current studies, there is no definite etiology of PCI. PCI appears to be associated with mechanical, bacterial, and biochemical processes. Some papers suggest an association of PCI with immunosuppressed patients , 3.The mechanical theory proposes that increased intra-luminal pressure forces gas within the bowel lumen to breach the mucosal or serosal layers , 4, 5. OThe pulmonary theory refers to increased intra-luminal pressure due to the respiratory system , 4, 6. IIn the bacterial theory, gas is introduced into the bowel lumen by pathogens. The gas again breaches the bowel wall integrity and seeps into the mucosal or serosal layer , 4, 5. HFinally, the biochemical theory describes the production of hydrogen gas during metabolism of food groups, especially carbohydrate. The increased amount of gaseous by-product raises intra-luminal pressure and the gas is forced into the weakened bowel wall. Trapped extra-luminal gas accumulates to form cysts.Several case reports have postulated a relationship of PCI with patients who are immunosuppressed . PCI hasMany hypotheses have been discussed in the literatures, while none can fully explain the etiology of PCI. At present, the condition is linked to several pathologies as described above. However, it must be stressed that PCI is not a disease but a physiological reaction to several conditions; hence, there is no single etiology , 9.PCI is usually a benign, asymptomatic, and occult condition. Review of the existing literature shows that patients who are symptomatic present in a diverse manner as a result of the underlying cause of PCI or complications from PCI. Due to the vague symptoms, the condition is easily misdiagnosed as bowel ischaemia or infarction . In thisAs illustrated in our case, PCI can easily mimic pneumoperitoneum on radiological imaging. Intramural air may appear as radiolucent shadows along the bowel lumen on X-ray . ExtensiDue to diverse interpretation, imaging should not be used for definitive diagnosis. Up to 27 % of benign PCI was misdiagnosed as surgical abdomen resulting in unnecessary operation . Intra-oMacroscopically, PCI is characterised by submucosal cysts varying from a few millimetres to centimetres in diameter , 10. ThePCI are true pseudocysts with no epithelial lining. Histiocytes and multinucleated giant cells line the submucosal or subserosal spaces and rarely the muscularis layer . These cAn incidental finding of PCI is usually benign and does not require treatment. The important decision is recognising when to perform an emergency laparotomy and when to manage conservatively. Patients who do not require surgery can be managed with medical therapies like high flow oxygen, hyperbaric therapy, antibiotics and special elemental diets . SurgeryPCI is an uncommon and poorly understood condition despite being first described in 1738. Identifying the underlying pathophysiology of PCI could potentially avoid misdiagnosis and mismanagement. The majority of the published literatures are case reports. To date, there has been no randomised controlled trial and very few literature reviews. Wu and his colleagues published a systemic analysis in 2013. They also found no studies on epidemiology or randomised control trials on PCI. They performed a systematic review of 239 PCI cases and found that it is a condition more common in males than females and more frequent in large bowel than small bowel . FurtherPneumatosis cystoides intestinalis refers to intramural gas which often presents as an incidental finding in benign cases. The exact cause of PCI is unknown, but the mechanical theory appears to be the most popular. It has been associated with gastrointestinal and respiratory complications. CT imaging appears to be the most sensitive radiological modality for diagnosis. The condition can easily be misdiagnosed as an acute abdomen leading to unnecessary surgery. From a clinical perspective, it is essential not to confuse the incidental finding of asymptomatic pneumatosis with symptomatic colonic perforation because the treatment is significantly different. As illustrated in our case, clinical presentations are very vague, mimicking several other intra-abdominal pathologies. Benign PCI should always be treated conservatively. Emergency laparotomy is reserved for life-threatening abdominal pathology. The typical microscopic appearance of PCI shows multinucleated giant cells lining the air-filled space. Although PCI was first documented more than 200 years ago, it is still an under researched topic and further studies and literature reviews are justified.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Background. Congenital absence of the submandibular gland (SMG) is a rare condition. Although complaints such as dry mouth, dental problems, or difficulty in swallowing may be seen, the subjects may also be asymptomatic. The absence of the SMG may be associated with hypertrophy of the contralateral SMG. Case Report. We report the case of a 44-year-old woman with incidentally detected left SMG aplasia, with contralateral SMG hypertrophy mimicking a mass, and the case of a 46-year-old woman with incidentally detected bilateral SMG aplasia, demonstrated by computerized tomography (CT) and magnetic resonance imaging (MRI). Conclusion. It is important for the clinician to know that this very rare abnormality may exist. When such a case is encountered, symptoms and findings should be reevaluated and, if necessary, conservative therapy should be initiated. The possibility of observing additional deformities should be kept in mind and an evaluation should be done for other cases in the family. Congenital absence of the submandibular gland (SMG) is a rare condition. The term “aplasia” is described as the total or partial agenesis of the gland . In the Due to an inadequate amount of saliva in these cases, some symptoms such as dry mouth, dental problems, and difficulty swallowing are seen. However, while asymptomatic subjects constitute approximately half of the cases, there are also subjects that are arbitrarily diagnosed.In this study, two cases—a patient with bilateral submandibular aplasia and a patient with unilateral submandibular aplasia—are reported and discussed based on the literature.A 46-year-old female patient was presented to our clinic with a palpable mass. In the physical examination, her thyroid gland was palpated as bilateral hypertrophic. In the palpation, nodular formations were detected in both lobes. The thyroid hormone levels were within normal limits. In the ultrasound (US) examination, the right lobe of the thyroid was measured at 13 × 4 × 4 cm and the left lobe was measured at 9 × 3 × 3 cm. Additionally, the isoechoic nodular structures were observed, the largest of which was on the right with a diameter of 32 mm while the left had a diameter of 26 × 19 mm with retrosternal extension. In the nodular structures, peripheral and intranodular bleeding was observed. A fine-needle aspiration biopsy was performed and the result showed a benign cytology. To determine its relation with the surrounding tissue, a computerized tomography (CT) of the neck was requested. In the CT scan, it was observed that the thyroid gland extended into the submandibular area and the SMG was bilaterally absent  agenesis of the left SMG was consulted in our clinic. On physical examination, no palpable mass was detected in the head and neck region. She had no specific relevant medical history such as dry mouth, dysphagia, teeth and gum problems, or sialadenitis. Agenesis of the left SMG and compensatory hypertrophy in the right SMG were observed in the CT and MRI scans Figures  and 4.In the literature , there iIn aplastic cases, dry mouth, difficulty swallowing, and dental problems may be observed. However, approximately half of the cases are asymptomatic . In the Although, bimanual palpation is absolutely necessary in the diagnosis of SMG aplasia, it does not provide adequate information. Evaluating the presence of the Wharton channel and its orifice is necessary. In total agenesis, the orifice and channel may not be observed. In fact, the diagnosis is determined radiologically. The methods that may be preferred in this area include US, CT, scintigraphy, MRI, and sialography . As someA differential diagnosis of the mass in the submandibular area includes nonmalignant or malignant growths. Nonmalignant swelling may occur as sialadenitis, Sjögren syndrome, cysts, infections, and lymphadenopathy. Neoplastic growths such as the SMG, the tail parotid gland, the Hodgkin's disease, non-Hodgkin's lymphomas, and metastatic disease may be seen . Some raIn summary, it is important for the clinician to know that this very rare abnormality may exist. When such a case is encountered, symptoms and findings should be reevaluated and, if necessary, conservative therapy should be initiated. The possibility of observing additional deformities should be kept in mind and an evaluation should be done for other cases in the family.


Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor typically affecting young to middle-aged adults. Despite its otherwise benign histologic appearance and indolent nature, it can have fully malignant behavior, and recurrence and metastasis may occur even decades later.We report a case of LGFMS in the left lower leg of a 5-year-old Japanese boy. A magnetic resonance imaging (MRI) uncovered a well-demarcated intra-gastrocnemial tumor measuring 27 × 20 mm with a slightly high intensity on T1WI and heterogeneously high intensity on T2WI. Histologically, the tumor was composed of bland spindle-shaped cells with a whorled growth pattern. The tumor stroma was variably hyalinized and fibromyxoid with arcades of curvilinear capillaries and arterioles with associated perivascular fibrosis. Although LGFMS is known to affect children under 18 years of age, it is extremely rare in infants and children under 5 years of age. Despite the young age, this patient was accurately diagnosed by the typical histology and the detection of a FUS-CREB3L2 gene fusion.Although LGFMS in children tends to be located superficially, this case presented with an intramuscular tumor in the region of the gastrocnemius. To the best of our knowledge, this is the first case of deep LGFMS arising in a child younger than 5 years of age. The patient is still alive with no evidence of the disease 4 months after diagnosis.http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_163The virtual slide(s) for this article can be found here:  Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor that typically affects young to middle-aged adults ,2. The mIn spite of its otherwise benign histologic appearance and indolent progression, many cases of the LGFMS may recur or metastasize decades later, mainly to the lung. Therefore, it is important to make a correct diagnosis and provide long-term follow-up for LGFMS patients. However, a diagnosis of LGFMS is often difficult because of a small biopsy specimen that can lead to a misdiagnosis of malignant tumors as benign or as tumor-like lesions including nodular fasciitis, schwannoma, desmoid-type fibromatosis, neurofibroma, and myxofibrosarcoma –8.Although, LGFMS is negative for most immunohistochemical markers, a recent study identified up-regulation of the mucin 4 (MUC4) gene in LGFMS compared with histologically similar tumors and lesions ,9, and MSeveral recent studies demonstrated that greater than 90% of LGFMS have a balanced chromosomal translocation t 7;16) q32-34;p11) leading to the fusion of the FUS and CREB3L2, while a minority of cases have a t  translocation leading to the fusion of the FUS and CREB3L1 [6  from a formalin-fixed, paraffin-embedded (FFPE) biopsy specimen.A 5-year-old Japanese boy was referred to our hospital with a painless mass in the lateral aspect of the left lower leg. An MRI revealed a well-demarcated intra-gastrocnemial tumor measuring 20 × 27 mm with slightly high intensity on T1WI and heterogeneously high intensity on T2WI . Purified RNA was reverse transcribed to cDNA using the Superscript first-strand synthesis system for RT-PCR . The primer sequences used for the amplification in this study were described previously . The PCRThe importance of molecular pathological diagnosis is increasing in the clinical pathology, especially in the field of soft tissue sarcomas, because the definite diagnosis made by the molecular pathology such as RT-PCR and specific genetic testing sometimes leads to the application of the tumor specific therapy –17. ReceLGFMS is extremely rare in children younger than 5 years of age , and we Considering both the smaller size and superficial location of LGFMS arising in correspondingly smaller infants and young adolescents, LGFMS in children might be noticed before becoming larger and invading into the fascia and skeletal muscles. Metastasis/local recurrence rates were 13%/38% in infants and 24%/41% in young adolescents, respectively, in our literature review. These rates are not significantly different from those previously reported in adult LGFMS . In addiFinally, we report a rare case of a 5-year-old Japanese boy with deeply located LGFMS in the lateral aspect of the lower leg. The superficial location of LGFMS in pediatric patients might be a reason for the relatively small tumor size in juvenile populations. Furthermore, superficial tumors are easily palpable and readily recognized by the patients and their parents.Wide resection of the tumor in the limb reduces the recurrence rate, but also affecting the future functional outcome, especially in the childhood. Recent advance in molecular pathology is helpful to distinguish specific soft tissue tumors from their histologic mimics. It is important to avoid misdiagnosis and provide appropriate treatment with a help of molecular diagnosis.Written informed consent was obtained from the parents of the patient for the publication of this report and accompanying images. The copy of the written consent is available for review by the Editor-in-Chief of this Journal.


Pneumocephalus is usually a self-limiting condition commonly associated with neurosurgical interventions, head and facial trauma. In contrast, tension pneumocephalus is extremely rare, and considered a neurosurgical emergency.We present a rare case of post-traumatic epidural tension pneumocephalus in a 30-year-old white man who deteriorated rapidly after a blunt head trauma. Imaging revealed a large, right temporoparietal epidural pneumocephalus with mass effect, most likely arising from a small defect in the mastoid sinus. A pre-existing mucocele was also suspected. Emergency burr hole evacuation was performed and he experienced full recovery, but more invasive treatment was eventually needed to resolve the condition.Epidural tension pneumocephalus is a rare and potentially life-threatening condition, but treatable with the right management. To the best of our knowledge, a post-traumatic tension pneumocephalus caused by a pre-existing mucocele has not been reported in the literature. Intracranial air entrapment  is commonly seen after head and facial trauma, ear infections, tumours of the skull base or neurosurgical interventions . It has We report a rare case of post-traumatic epidural tension pneumocephalus in a man who deteriorated rapidly after a blunt head trauma. Imaging revealed a large, right temporoparietal epidural pneumocephalus with mass effect and midline shift, most likely arising from a small defect in the mastoid sinus. Burr hole evacuation was performed and he experienced full recovery. However, more invasive surgery was needed to resolve the condition.A 30-year-old white man was admitted to his local hospital after he was found unconscious in his home. His past medical history included a history of drug abuse, bipolar disorder and chronic hepatitis C. He had no history of headaches. The family reported that he was last seen when he returned home the same morning about 5 hours prior to admission. Before he went to bed, he had told them that he was assaulted on his way home, and had been beaten and kicked several times to the head. There was no reported loss of consciousness. His only complaint before he went to bed was of a mild headache. Five hours later a family member called for an ambulance, as he was not responding adequately to speech or other stimuli.In the emergency department he had a Glasgow Coma Scale (GCS)  score ofThe following days he deteriorated and consecutive CT scans demonstrated increasing air entrapment. An indication for more invasive surgery was found, and a craniotomy with cranialization and sealing of the mastoid sinus with autologous musculature, an absorbable fibrin patch (Tachosil®) and fibrin glue (Tisseel®) was performed, to prevent further air entrapment. The dura was intact, and there was no leakage of cerebrospinal fluid. During the procedure several bony defects were found in his mastoid bone, and a mucosal membrane arising from the mastoid sinus found adherent to his skull and dura mater  was removed.He recovered quickly from this procedure, and postoperative CT scans were satisfactory, with re-expansion of the dura, resolution of the midline shift, and only minor residuals of the epidural air. He was discharged from the hospital 15 days after admission.Unfortunately, 4 weeks later he deteriorated again, and complained of increasing headaches and diplopia. Renewed CT scans revealed increasing entrapment of the residual air, and a second surgery was performed. The same procedure was repeated, and a recanalization of the defect in the bone was found. Again, thorough sealing was obtained with bone wax, a fibrin patch and fibrin glue, and this time it ultimately turned out to be successful. The latest follow-up CT scan performed 4 months later was still found to be satisfactory.pneumocephalus [Intracranial pneumocephalus was first described in an autopsy report of a trauma patient in 1866 . Some yeA previous review of the underlying causes indicated that trauma was the most common cause of pneumocephalus, associated with nearly 75 % of the cases, whereas neoplasm and infection were found in 13 % and 9 % of the cases, respectively .Pneumocephalus is most easily diagnosed on CT , which cThe treatment depends on the underlying cause of air entrapment, but in most cases it resolves spontaneously with conservative care. In symptomatic postoperative pneumocephalus, treatment with 100 % oxygen has been shown to increase the rate of reabsorption . TensionIn our case we present a patient with a large, temporoparietal pneumocephalus, where air was introduced to the intracranial epidural space through a small defect in the mastoid bone, with a fistula into a mucous compartment. Due to the history of a significant blunt head trauma a few hours prior to admission, it was considered to be post-traumatic. As he experienced rapid clinical deterioration and life-threatening symptoms of herniation, immediate pressure relief was indicated, and obtained with a burr hole evacuation of the entrapped air. Although the patient experienced a short relief of symptoms caused by tension, a more invasive procedure was needed to resolve the condition. A craniotomy with cranialization of the mastoid sinus and thorough sealing of the defect in the mastoid bone was performed to prevent air from escaping through the sinus into the epidural space. Due to inadequate sealing, repeat surgery was needed 4 weeks later to eventually treat the problem. Based on the intraoperative findings of an extensive mucous membrane, it is reasonable to believe that a pre-existing mucocele originating from the mastoid sinus must have been a contributing factor, allowing air to enter the epidural space. In addition, a thinning of the skull bone adjacent to the epidural air suggests that there has been a mass lesion there for a substantial time. A possible mechanism could also be the rupture of this mucocele, most likely caused by the head trauma, allowing air to enter the epidural space from the mastoid sinus and causing tension pneumocephalus.Epidural tension pneumocephalus is a rare and potentially life-threatening condition, but treatable with the right management. To the best of our knowledge, a post-traumatic tension pneumocephalus caused by a pre-existing mucocele has not been reported in the literature. Unfortunately, we do not have any prior imaging of this patient to ultimately prove this theory.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.


Neuroendocrine tumors constitute a group of heterogeneous neoplasms, both those that are clinically asymptomatic and those which present with an array of symptoms. This variable clinical manifestation and unsatisfactory detection rate on diagnostic imaging make preoperative diagnosis particularly challenging. Insulinoma is a rare tumor originating from insulin-synthetizing pancreatic beta cells which clinically manifests hypoglycemia. The current study presents the case of a patient with a one month history of diabetes, and a tumor of the pancreatic head diagnosed at the Regional Hospital of Lomza . The patient subsequently underwent surgery. The histological examination indicated insulinoma; islet cell tumor of the pancreas. The patient’s postoperative period was uneventful and during two years of follow-up, the patient has remained in good health with completely controlled diabetes mellitus. The hereby-presented case of pancreatic insulinoma confirms this finding, as a correct diagnosis could only be established on the basis of pathomorphological examination. In addition, radical surgical resection is currently the only available treatment. Insulinoma is a rare tumor of the alimentary tract originating from insulin-synthetizing pancreatic beta cells. Its incidence is estimated at four per million individuals per year worldwide . Althoug2, and laboratory tests also did not reveal any abnormalities aside from high blood glucose levels (up to 16.8 mmol/l) and increased C peptide levels (2.17 ng/ml). A 2-h oral glucose tolerance test revealed the same levels of C peptide (2.14 ng/ml). The concentration of bilirubin was normal. A 5.5-cm tumor of the pancreatic head was shown on computed tomography, compressing the interior vena cava and the right renal vein, and segmentally displacing the duodenal loop. Following normalization of glycemia with insulin, the patient was qualified for scheduled surgery.A 47-year-old female without a history of acute pancreatitis and diagnosed with type 2 diabetes one month previously, was admitted to the Second Department of General and Gastroenterological Surgery, Medical University of Bialystok  due to a tumor of the pancreatic head which had been diagnosed at the Regional Hospital of Lomza  . On admiA lard-like, gray-white tumor of the pancreatic head, measuring 5.5 cm in diameter, was revealed intraoperatively. The tumor was observed to compress the common bile duct and the pancreatic duct, and regression of the pancreatic body and tail parenchyma was evident. No lymph node metastases were documented on intraoperative microscopic examination. The pancreas was resected completely. Due to the frozen section examination which revealed a benign characteristic of the tumor, a pylorus-preserving pancreticoduodenectomy was performed according to the method of Traverso and Longmire . PancreaPathomorphological examination of the surgical specimen revealed a G2 (moderately differentiated) and pT3  tumor according to the TNM Staging for Foregut Neuroendocrine Tumors of the Stomach, Duodenum, and Pancreas . The remThe patient was discharged home in a good general condition and visited the Surgical Outpatient Clinic for two weeks following the surgery. The patient was not hospitalized during the two years of postoperative follow-up. The patient has remains disease-free without any complaints and with complete control of diabetes mellitus.Neuroendocrine pancreatic tumors form a group of heterogenic neoplasms originating from exocrine cells. Although these tumors more commonly occur spontaneously, they can be associated with multiple endocrine neoplasia type 1 syndrome in 10% of cases. Insulinoma is a hormonally active tumor originating from insulin-synthesizing beta cells of the pancreas. Although it is typically benign, it can be malignant in 10% of patients. Most (90%) insulinomas are no larger than 2 cm . Due to Usually, patients are evaluated for potential insulinoma due to hypoglycemic signs, such as hand tremor, excessive sweating, heart palpitations, double vision and sudden loss of consciousness. Moreover, cases in which the initial signs of insulinoma included seizure episodes or behavioral disorders have been reported. Abnormalities in laboratory results include hypoglycemia associated with elevated levels of insulin and high activity of C peptide, corresponding to the overproduction of endogenous insulin. The supervised 72-h fast is the gold standard test for the diagnosis of insulinoma . It is net al (Only one such case was recorded amongst 313 insulinoma-type tumors treated at Mayo Clinic between 1927 and 1993. Moreover, only one case of insulinoma associated with hyperglycemia was observed among 443 Japanese patients treated for this tumor between 1976 and 1990 . The patet al  identifiSurgery is the basic treatment option for both pancreatic adenocarcinoma and neuroendocrine tumors of this organ. However, the recommendations on the optimal extent of resection differ. Radical surgery, including resection of the pancreas and regional lymph nodes, should be performed in adenocarcinoma cases. By contrast, an enucleation without intact tissue margin is sufficient in the case of small isolated neuroendocrine tumors, enabling a 90% five-year survival rate . MoreoveThis case confirms that a correct diagnosis can only be established on the basis of the postoperative pathomorphological examination. In addition, due to the high malignant potential of neuroendocrine tumours, radical surgery with regional lymphadenectomy and intraoperative frozen section evaluation remains the treatment of choice. In conclusion, the differential diagnosis of pancreatic neuroendocrine tumours must include insulinomas with high blood glucose levels, as certain neuroendocrine tumors are biologically inactive.


Clear cell carcinoma is the least common of the malignant transformations reported in nonpelvic sites of endometriosis. Two cases with clear cell carcinoma transformation arising from endometriosis in abdominal wall scars are presented. These patients underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, pelvic washings, and abdominal wall lesion resection. The first case had initial treatment with chemotherapy, while chemotherapy and radiation therapy were given for the second case. A recurrence was noted for the chemotherapy only case for, which she was subsequently given radiation, with further resolution of the lesion. Endometriosis is a common benign diagnosis in women, yet its extraperitoneal presence, while well described, is rare. In the past, encountering endometrial implants in cesarean section scars was an uncommon event . HoweverA 41-year-old African-American female, gravida 4, para 4, presented for evaluation of heavy vaginal bleeding and abdominal pain. Her history was significant for a cesarean section 20 years ago. Physical exam was significant for a 2 cm cystic tender lesion under the Pfannenstiel scar. Pelvic ultrasound demonstrated a 9 cm uterus with a submucosal fibroid and a 3.1 × 3.5 × 3.5 cm simple cyst on the left ovary. Endometrial biopsy demonstrated an endometrial polyp. Diagnostic hysteroscopy with ablation was performed for endometrial polyp evaluation and treatment. CA-125 was 22. The Pfannenstiel mass was evaluated with a CT of the abdomen and pelvis, which showed a 4.5 × 5.6 × 8.1 cm heterogeneous soft tissue mass with a fluid component within the rectus abdominis muscle and subcutaneous fat.The patient was lost to follow up for approximately 1 year, after which she returned with abdominal pain. Abdominal and pelvic CT , along wThe patient underwent total abdominal hysterectomy with bilateral salpingooophorectomy, partial omentectomy, and pelvic washings along with complete excision of the mass. Histopathologic workup yielded the diagnosis of clear cell carcinoma arising in association with endometriosis from the Pfannenstiel scar lesion. Uterine pathology was significant for adenomyosis with subserosal leiomyomas. The left fallopian tube had evidence of hydrosalpinx and chronic salpingitis. The remaining pathology was without evidence of malignancy.The postoperative course was complicated by a partial wound separation on postoperative day #38, which healed by secondary intention. Following surgery, she received adjuvant chemotherapy consisting of carboplatin and paclitaxel for a total of 6 cycles. The patient had regular follow-up visits, and at 6 months a CT was obtained for evaluation of a newly found mass at the same location where the initial lesion was excised. CT revealed a new anterior abdominal wall mass within the rectus muscles. A fine needle aspiration was performed and demonstrated atypical cells present suspicious for malignancy. Targeted radiation of the new mass was performed over 6 sessions. A follow-up CT was performed which showed resolution of the mass.A 57-year-old female, gravida 3, para 3, presented for evaluation of a 9-month history of pain under Pfannenstiel scar. Her history was significant for 3 cesarean sections, 30 years ago, with tubal ligation. Physical exam was significant for a tender, 7 cm mass under the Pfannenstiel scar and bilateral inguinal lymphadenopathy. CT and MRI evaluation confirmed an abdominal wall tumor extending to the bladder without invasion. Needle core biopsy of the mass and a right inguinal lymph node revealed poorly differentiated adenocarcinoma, possibly Müllerian in origin. The patient's initial laboratory evaluation revealed a normal CA 19-9. CEA and CA-125 were elevated at 96.5 and 81, respectively. The patient underwent wide local excision of the abdominal wall mass, total abdominal hysterectomy with bilateral salpingooophorectomy, and bilateral inguinal/internal iliac lymphadenectomy with the removal of nodules from the serosa of the terminal ileum and cecum. Frozen section revealed adenocarcinoma of abdominal wall mass without evidence of malignancy in fallopian tubes, ovaries, or uterus. Plastic surgery was required for the closure of the 35 cm abdominal wall defect with biologic mesh and abdominal wall flap.Final pathology showed the fallopian tubes, ovaries, and uterus without abnormality. The abdominal wall lesion was 19.4 cm in the greatest dimension. Pathology showed poorly differentiated adenocarcinoma with clear cell features and positive margin at pubic symphysis. Findings were consistent with clear cell carcinoma of Müllerian origin, with endometriosis lesions noted in close proximity of the clear cell adenocarcinoma. One inguinal lymph node (6.2 cm) and one internal iliac lymph node (6.1 cm) showed metastasis of clear cell carcinoma.Her postoperative course was complicated by a small area of wound infection one month after surgery, which was treated with antibiotics. Following recovery, the patient was treated with carboplatin and taxol for 3 cycles due to positive nodes found, followed by radiation therapy to the positive pubic symphysis margin followed by 3 more cycles of chemotherapy. She was followed up during treatment with CA-125 levels, which progressively dropped to below 7. Follow-up evaluation with CT revealed no evidence of recurrence.Endometriosis has been proposed as a precursor of clear cell carcinoma of the ovary . The timOf the three subtypes of extragonadal malignant endometrial transformations, endometrioid subtypes represent 69.1%, sarcoma subtypes represent 25%, and clear cell carcinoma subtypes represent 4.5% [Unfortunately, a specific marker for diagnostic purposes has not been identified. Due to rarity, there is no standardized management. Index of suspicion leading to fine needle biopsy is usually diagnostic. In malignant transformations, CA-125 has been reported as normal to mildly elevated  with higFrom the few cases reported in the literature, it has been noted that the rectus muscle may be intimately involved within the lesion  and the Treatment of abdominal wall adenocarcinoma arising from endometriosis is not standardized; therefore, adoption of ovarian cancer management has been routinely performed in these patients. Although there is no consensus or evidence based guidelines for this management, complete surgical resection of the lesion should be performed, with strong consideration for surgical staging similar to ovarian cancer. Of the cases presented here, complete resection of the masses was achieved. To achieve complete resection, it may require radical cytoreductive surgery, which may require reconstruction of the abdominal wall. Groin lymph nodes are the main lymphatic spread from lower abdominal wall disease and may require excision and/or sampling. Cases with bulky disease may warrant neoadjuvant chemotherapy prior to surgery. In the cases described, decision for chemotherapy administration was prophylactic for Case 1 and treatment for lymphatic invasion noted in Case 2. Postoperative radiation therapy to prevent/treat local recurrence may be considered. Recurrences are uncommon but have been reported, including progression to death. The lack of standardized management and treatment of these rare malignant transformations poses the greatest challenge to patient care. The two cases presented will add to the current body of literature. Patients with a remote history of endometriosis may have severe sequelae from their disease. Further investigation is required to understand which patients may be at risk for this transformation and how to best treat them.


A common indication for laparoscopic mesenteric lymph node biopsy is to provide a tissue diagnosis in the absence of palpable peripheral nodes via a minimally invasive approach.  There are no reports to date of ischaemia to the appendix as a complication of this procedure.   We report the case of a 34-year-old lady who underwent a mesenteric biopsy for a lesion found incidentally on CT to investigate longstanding abdominal pain, and 2 days later required an appendicectomy for ischaemic appendicitis. Appendicitis arising as a complication of a surgical procedure has not been reported in the literature.Appendicitis is the most common intra-abdominal surgical emergency. The classical pathophysiology of appendicitis is that of obstruction of the lumen and subsequent bacterial overgrowth5. Where this cannot by achieved by percutaneous techniques, laparoscopic mesenteric biopsy has been shown to be a safe and effective alternative, when compared with open techniques5. As laparoscopy is a minimally invasive technique, the reduction in wound healing time decreases the delay to therapeutic interventions such as chemotherapy2. Reasons for conversion to laparotomy include adhesions, poor intra-operative exposure and bleeding1. There are no reports to date of ischaemia to the appendix as a complication of this procedure.A common indication for mesenteric lymph node biopsy, in the setting of enlarged mesenteric nodes or a mesenteric mass on imaging, is to provide a tissue diagnosis in the absence of palpable peripheral nodes via a minimally invasive approachWe describe a case of appendicitis in the post operative period in a 34-year-old lady who underwent a mesenteric biopsy of a lesion found incidentally on CT.A 34-year-old Caucasian lady, with no significant medical history, presented to her local GP with persistent epigastric pain and nausea. Investigation of the pain included an abdominal CT, which revealed an incidental mass in the right iliac fossa measuring 17mm suspicious for a carcinoid tumour, and no other features of concern or metastatic disease. She underwent a diagnostic laparoscopy and excisional biopsy of this mesenteric mass near the junction of the ileum and caecum, with a Harmonic scalpel. She had an uneventful recovery, and was discharged day 1 postoperatively. She later presented to the emergency department on the same day of discharge, with worsening abdominal pain and fevers. She was taken back to theatre the following day and was found to have a necrotic appendiceal tip, and underwent an appendicectomy.The histology from the mesenteric biopsy showed a 10×15mm nodule of carcinoid tumour, as well as 2 of 4 lymph nodes containing metastases. The histology from the appendicectomy showed extensive mucosal necrosis, but no evidence of malignancy.The patient has since undergone an open, uncomplicated right hemicolectomy which showed an 8mm grade 1 neuroendocrine tumour at the region of the ileocaecal valve with clear margins and 11 lymph nodes negative for metastatic disease. She is currently under the care of an oncologist and receiving lanreotide 60–120mg monthly, the duration of which is currently ongoing.4. The appendiceal artery is the terminal branch of the ileocolic artery, and runs in the free edge of the mesoappendix. Varying descriptions of anatomical locations and incidence of accessory appendiceal arteries are described in the literature6.Appendicitis due to primary ischaemia, or as a complication of mesenteric biopsy, is not described in the literature. Acute torsion of the appendix and subsequent appendicitis has been described infrequently in the paediatric population7.We hypothesise that this presentation of acute appendicitis on the second post-operative day following mesenteric biopsy was secondary to inadvertent vascular injury, and subsequent ischaemia, given the proximity of the initial operative field to the ileocaecal junction. One possible mechanism is that appendiceal artery, or a possible aberrant course of the appendiceal artery or accessory appendiceal artery, was not identified during initial laparoscopic survey and was divided during dissection. Another possible mechanism is that the appendiceal artery was indirectly affected by secondary thermal injury during harmonic dissection of the mesenteric lesion. Whilst uncommon, unexpected thermal injuries to surrounding structures have been reported during use of Harmonic dissectionLaparoscopic mesenteric biopsies are becoming increasingly more common as histological diagnosis is required to confirm disease processes. This case illustrates an unusual complication of a procedure often viewed as straightforward and highlights the diligence required during mesenteric dissection to prevent inadvertent injury to nearby structures, as well as the possibility of unusual circumstances resulting in unexpected injuries when using the Harmonic scalpel as an energy source for dissection.Consent for publication of information from this clinical case was sought and obtained from the patient. The authors present a case report of a complication from a laparoscopic mesenteric excisional biopsy. Following an excision of an suspected carcinoid node in the mesentery, the patient suffered an ischemic event to the appendix.The authors describe that the indications for the laparoscopic excision are for those areas not amenable to percutaneous biopsy. This should also include lesions that have failed image guided biopsy. To that end, please comment on the patient's course in that regard. Did they have an attempted biopsy? Did they have a biopsy that failed to yield the diagnosis?Did the patient have a colonoscopy? With a suspicious carcinoid, an endoscopic evaluation should be considered to assess for primary tumor.Also, please comment on the intraoperative findings in more detail. Carcinoid tumors, and specifically its associated lymphadenopathy, have a very distinctive appearance that can clue in the operative surgeon to the diagnosis. Did you consider intraoperative pathologic assessment? With the possibility of completing the hemicolectomy in the first operation?Lastly, I agree with the other review that additional information regarding recommendations/suggestions to avoid mesenteric vascular injury during these procedures would be helpful.My comments/suggestions for the article are as follows:I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. Can they be more descriptive in how the actual excisional biopsy was done - was there an attempt to identify the vascular supply to the nearby structures?Can they suggest ways of preventing ischemic injuries while performing similar procedure?Drs. Zukiwsky, Tun and Desai has described ischemia as a potential complication of mesenteric biopsy.  This certainly highlights the need to be cautious in performing what might be considered a minor procedure. I do have 2 requests:I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Inverted Schneiderian papilloma is an uncommon benign tumor that presents tendency to recur and propensity to be associated with malignancy in approximately 10% of the cases. Some of these lesions are isolated in the maxillary sinus, and predominantly affect white males with mean age of 50 years. We report a case of squamous cell carcinoma arising from inverted Schneiderian papilloma in the maxillary sinus extending to the mouth. The patient was submitted to extraction of a maxillary molar tooth four months before the exacerbation of the symptoms of nasal airway obstruction and facial enlargement. Computed tomography scan revealed a sinonasal mass causing opacification of the right maxillary sinus with destruction of the lateral nasal wall and maxillary sinus floor. The patient was referred to an oncology center for treatment and died from tumor progression one year after the cancer was diagnosed. The intention of this report is to alert dentists to include the inverted Schneiderian papilloma, either associated with squamous cell carcinoma, or not, in the differential diagnosis of maxillary sinus tumors with aggressive behavior, which may extend to the oral cavity or involve roots of teeth. Papillomas are uncommon benign tumors of Schneiderian mucosal origin , 2. ThreDespite its benign nature, the inverted Schneiderian papilloma has a high propensity for local recurrence and may undergo malignant transformation into squamous cell carcinomas , 3, 7. AThe etiology of inverted Schneiderian papillomas remains controversial, but factors such as chronic inflammation, allergy, occupational pollutants, and mainly infection with human papilloma virus have been suggested , 4. CompWe report a case of invasive squamous cell carcinoma that arose from an inverted Schneiderian papilloma, causing maxillary sinus destruction with extension to and involvement of the oral cavity.A 53-year-old man presented with a painful mass in the posterior maxillary alveolar ridge, symptoms of nasal airway obstruction, and facial enlargement. The patient reported a history of tooth extraction (tooth 17) due to a periapical lesion, four months previously. The rest of his medical history was unremarkable. Radiographic evaluation including a computed tomography scan revealed a sinonasal mass causing opacification of the right maxillary sinus with destruction of the lateral nasal wall and maxillary sinus floor . The preThe inverted Schneiderian papilloma is a benign tumor that presents a tendency to recur and propensity to be associated with malignancy. However, its potential malignant transformation has been described in approximately 10% of the cases , 3, 9. TSome symptoms such as nasal obstruction, epistaxis, rhinorrhea, and facial pain or pressure are frequently associated with the occurrence of the inverted Schneiderian papilloma but other inflammatory or neoplastic lesions that affect the maxillary sinus may present similar clinical findings , 4, 7. OThe presumptive clinical diagnosis was aggressive mucocele of maxillary sinus or malignant tumor, based in the imaginologic exams. In fact, the radiographic characteristics including opacification and/or bony destruction of the paranasal sinuses are also found in other benign lesions, such as mucoceles of the maxillary sinuses, nasal polyps, or cholesterol granuloma , 13, 14.In the present case the histopathological features were typical of inverted Schneiderian papilloma , 9, 11, The most accepted treatment for inverted Schneiderian papilloma is complete surgical resection and due to the aggressiveness, possibility of recurrence, and malignant transformation, long-time followup is recommended , 11, 12.Although our patient presented clinical symptoms such as nasal airway obstruction and facial enlargement, he sought a dentist and not a physician. Probably, this occurred because he associated the exacerbation of the symptoms with the extraction of a maxillary molar tooth that had been performed four months earlier. Therefore, in conclusion, the intention of this case reported is to alert dentists to include the inverted Schneiderian papilloma, either associated with squamous cell carcinoma or not, in the differential diagnosis of maxillary sinus tumors with aggressive behavior, which may extend to the oral cavity or involve the roots of teeth.


Tufted angioma (TA) is a benign vascular tumor with endothelial origin. It is extremely rare in oral mucosa; only seven cases have been reported in the literature so far. Here, we describe two cases of tufted angioma observed in children and we also present a review of the literature about this pathology, concerning the differential diagnosis and management of this lesion in children. Tufted angioma (TA) is a rare benign vascular tumor with endothelial origin characterized by slow and indolent growths –3. This The TA most common form of presentation is an erythematous macula or plaque with angiomatous appearance , 7, 8. IThe aim of this paper is to report two cases of tufted angioma affecting children and to review the literature about this pathology, discussing the differential diagnosis and management of these lesions. A bibliographic search was performed up to October of 2014 on online databases , using the key words “tufted angioma,” “oral tufted angioma,” “oral angioma,” “lip OR tongue OR buccal mucosa OR oral mucosa OR mouth AND tufted angioma.” The literature search retrieved 6 papers that reported a total of 7 cases. The clinical features of the oral mucosa cases are reviewed in A 10-year-old male presented to the Center of Dental Specialties of the Federal University of Rio Grande do Sul, Porto Alegre, Brazil, with a lesion localized in the left labial commissure of two months of duration. The lesion was asymptomatic and has rapidly grown in size since it was first noticed.The clinical examination revealed a firm, red to purple nodule with a sessile base, measuring approximately 1 cm in diameter. The lesion was diagnosed clinically as pyogenic granuloma due to its clinical appearance and because patient reported trauma in the region before the appearance of the lesion .Based on the clinical diagnosis of pyogenic granuloma an excisional biopsy was performed. The microscopic examination revealed an endothelial cell proliferation in nodular arrangements with a “cannon ball” pattern covered by oral mucosa. The formation of irregular capillary blood vessels with a slit-shaped lumen and presence of discrete inflammatory infiltrate in the underlying connective tissue were also observed. Based on the histological features of the lesion, the final diagnosis was tufted angioma Figures .After 2 weeks, patient returned with recurrence of the lesion in the same anatomic site and reported trauma in the wound area. The clinical examination showed a red nodule with 0.5 cm in diameter . The recA 12-year-old male presented to the Center of Dental Specialties of the Federal University of Rio Grande do Sul, Porto Alegre, Brazil, with a nodule localized in upper lip with one month of duration. The patient related a previous lesion with 4-month-period lesion at the same region, which was removed in another service; however no histopathological analysis was performed. After one month of the procedure, the lesion relapsed, and the patient was referred to our service. The clinical examination revealed a firm nodule with reddish color, being asymptomatic, measuring approximately 1 cm in diameter . The lesThe histopathological features were similar to the first case and the final diagnosis was tufted angioma Figures . The patThe tufted angioma is a rare benign lesion, though its reactive or neoplastic nature is unclear. Some authors associate this disease with endocrine stimuli, such as the loads of hormonal release that occur at puberty suggesting that it may represent a reactive vascular proliferation secondary to hormonal stimulation . However2. However, the oral mucosa tufted angiomas reported so far, including the ones here reported, have not been associated with neither hyperhidrosis nor KMS [Most frequently, the cutaneous TA is clinically characterized as a solitary tumor with erythematous, macula, or plaque appearance located in the skin of neck, upper trunk, and extremities. Sometimes it can be associated with hyperhidrosis or hypertrichosis and Kasabach-Merritt syndrome (KMS), which is a rare coagulopathy resulting in thrombocytopenia, anemia, and hypofibrinogenemia nor KMS , 14.The oral mucosal involvement by TA is extremely rare. These lesions usually are asymptomatic and display a papular or nodular aspect. They may grow rapidly and their color ranges from red-brown to purple-blue. Most of cases are localized in lip , 10, 11.Clinical differential diagnosis of the tufted angioma in lip should include reactive lesions, such as pyogenic granuloma and mucocele and benign neoplasms from endothelial or salivary gland origin. However, the histopathological findings are specific and these lesions exhibit individual characteristics by which they can be distinguished from TA .The histopathology analysis of the herein reported cases revealed intense endothelial cell proliferation in nodular arrangements in a “cannon ball” fashion and numerous irregular capillary blood vessels with a slit-shaped lumen, consistent with TA diagnosis. Based on the histologic features it is important to distinguish TA from other vascular lesions such as pyogenic granuloma, haemangioma, and kaposiform hemangioendothelioma. The pyogenic granuloma shows a high vascular proliferation as capillaries and the presence of intense inflammatory infiltrate containing young fibroblasts that resemble granulation tissue, while tufted angioma is characterized by proliferation of spindled and polygonal cells surrounding vascular channels, organized as tufts . The capThe treatment of TA is mostly performed by complete excision of the lesion. Furthermore, other methods were used previously by different authors, including compression therapy, surgery, cryotherapy, laser, topical or systemic corticosteroids, interferon, and chemotherapy , 9, 11. In conclusion, dentists should be aware of recurrent tufted angioma diagnosis in children and the reported cases may contribute to databases of the literature to provide a better understanding of the biological behavior and treatment of this lesion in children.


Endobronchial lipomas are rare benign tumors of the respiratory tract. Bronchial occlusion may cause parenchymal damage and lead to a misdiagnosis of chronic obstructive pulmonary disease or malignancy. Therefore, both accurate diagnosis and radical treatment of endobronchial lipomas are essential. We describe the case of a 61-year-old man with a history of smoking (40 pack years), dyspnea in exertion, and cough during the past 6 months due to an endobronchial lipoma. Chest computed tomographic (CT) scan revealed a circumscribed polypoid lesion partially obstructing the left lower lobe. The endobronchial lipoma was removed by flexible bronchoscopy, and the patient had complete resolution of symptoms following the procedure. Flexible bronchoscopy was normal at the 3-month follow-up. In addition, clinical characteristics, diagnosis, and treatment of endobronchial lipomas are discussed. Endobronchial lipomas are rare tumors with incidence reported at 0.1–0.5% of all lung tumors . The sizA 61-year-old man presented with shortness of breath, cough symptoms during 6 months and atelectasis of the left lower lobe on chest radiograph. Chest radiograph from 2012 was normal. He had a medical history of smoking (40 pack years), COPD, and had been previously operated for acute myocardial infarct and ventricular septum defect. He disconfirmed weight loss, loss of appetite, chest pain, fevers, and night sweats.Chest CT scan showed the left lower bronchus occupied by fatty content material, size 0.8×0.7×1.1 cm, with atelectasis of the medial segment of the left lower lobe . PositroThe patient had complete resolution of his symptoms following the procedure. Flexible bronchoscopy was normal at the 3-month follow-up.Tumors in the tracheobronchial tree are often malignant. Benign lung tumors are rare and comprise 2–5% of all lung tumors . LipomasBronchoscopic resection should be considered as the first line of management of endobronchial lipoma. The advantage of bronchoscopic resection of endobronchial lipoma is that it is a less invasive method, preserves lung tissue, resulting in lower morbidity compared with surgical resection. Not only is it diagnostic and therapeutic, it is also associated with good symptomatic control and local control , 11.Both flexible and rigid bronchoscopy have been used successfully , 7, althThe endoscopic techniques usually include mechanical debulking . AdditioThe recurrence rates of endobronchial lipomas are low after complete surgical resection and the risk of malignant transformation is virtually non-existent , 11.To our knowledge, this unique entity is not previously described in the Danish literature. Clinicians should be aware of this rare condition which may be misinterpreted with symptoms of COPD, asthma, pneumonia, or malignancy. Early diagnosis and intervention should be planned on a case-by-case basis by a multidisciplinary team in centers experienced in complex airway disorders , 11.The identification and diagnosis of endobronchial lipomas can be challenging as it can simulate obstructive lung diseases. However, early identification and intervention are essential as endobronchial lipomas can cause irreversible damages to the lung parenchyma. Bronchoscopic resection is the first line management of endobronchial lipomas, but surgical resection should be considered as an option in certain complex cases.


Gallbladder carcinoma (GBC) is an uncommon malignancy with a high mortality rate. Detecting gallbladder carcinoma in its early stages can be difficult, despite improvements in ultrasound and computed tomography (CT) imaging. Most diagnoses of GBC are made at advanced stages, with the majority being found incidentally during surgery for cholelithiasis. The presented case demonstrates the difficulty of diagnosing GBC preoperatively in its early stages. Salmonella, Helicobacter), and medications  [Gallbladder carcinoma (GBC) is a rare malignancy, but the most common malignancy of the biliary tract. The incidence of GBC in the US is 1.2 per 100,000. The majority of gallbladder carcinomas are diagnosed incidentally, usually when exploring for cholelithiasis [strogen) -5. HereiA 65-year-old obese, African-American female with a history of hyperlipidemia presented to her primary care physician (PCP) in May 2013 for right neck pain radiating to her right shoulder for several weeks. The patient's initial blood work revealed an elevation in her liver enzymes; otherwise, all other lab results were normal. Additionally, a right upper quadrant (RUQ) ultrasound was performed which demonstrated probable gallbladder sludge and stones, with a low likelihood of being polyps or gallbladder neoplasm given the absence of discrete vascularity , RUQ ultrasound, and nuclear medicine hepatobiliary (HIDA) study. The ultrasound demonstrated cholelithiasis and echogenic sludge within the gallbladder with hypoechoic and hyperemic adjacent liver parenchyma. A mildly enlarged common duct was also observed. These findings were concerning for chronic cholecystitis, xanthogranulomatous cholecystitis, or early acute cholecystitis Figure . The HIDThe patient was taken to surgery for a laparoscopic cholecystectomy for suspected cholecystitis. The laparoscopic approach was converted to an open procedure after the discovery of a mass within the lumen of the gallbladder, a second mass near the hepatic flexure of the colon, and enlarged portal vein lymph nodes. During the operation, the patient underwent a radical cholecystectomy, right hemicolectomy with primary anastomosis, and a portal vein lymph node dissection (LND). Pathology of the gallbladder mass demonstrated infiltrating papillary adenocarcinoma invading the perimuscular connective tissue. The pathology also revealed one portocaval lymph node that was positive for adenocarcinoma. The patient's stage of disease was determined to be IVB (pT2N2). After surgery, the patient underwent radiotherapy to 4500 cGy, targeting the gallbladder fossa and the involved lymph nodes, with concurrent chemotherapy (capecitabine 625 mg/m2 twice daily). Abdominal CT and MRI scans were performed eight months after the completion of chemoradiation. At that time, there was no radiologic evidence of disease recurrence. Currently, the patient is fourteen months post treatment. Overall, she is doing well and continues to receive close follow-up care.GBC is an infrequent neoplasm that is associated with a high rate of regional lymph node metastasis and mortality ,6,9. AdjEarly diagnosis can be difficult because symptoms can mimic or be caused by coexisting cholecystitis, which is a common condition . TherefoImaging with ultrasound and CT has improved preoperative diagnosis of GBC. Despite these advancements, only 50% of gallbladder cancers are recognized before surgery . UltrasoThe most common ultrasound findings include calcified and echogenic mucosal masses, which can be associated with cholelithiasis or porcelain gallbladder . High-ri7), but can also be performed under CT guidance, endoscopic ultrasound guidance, endoscopic retrograde cholangiopancreatography (ERCP), or by laparoscopy.The most useful, non-invasive imaging studies for evaluating GBC preoperatively include CT, magnetic resonance imaging (MRI), and/or magnetic resonance cholangiopancreatography (MRCP) Figures , 6. CT hRegarding further procedures and tests for GBC, endoscopic ultrasound (EUS) is a minimally invasive procedure that has been shown to be more accurate than RUQ ultrasound at imaging the gallbladder. EUS is also helpful in the differential diagnosis of gallbladder polyps and excellent in staging tumor depth -20. ERCPOur case illustrates the challenge of preoperative, early-stage GBC diagnosis. Despite multiple imaging studies, GBC was not diagnosed in the described patient until laparoscopy for acute cholecystitis. In the companion case, GBC was advanced at the time of imaging, which may have been due to patient denial of symptoms. Given that early detection is important in both decreasing the morbidity and mortality of GBC, with potential for surgical cure in cases limited to the gallbladder, patients with RUQ ultrasound findings such as gallstones or polyps should be offered surgical consultation. Further, consideration of GBC within the differential diagnosis by primary care clinicians, radiologists, and surgeons may serve to maximize discovery before the time of surgery."The views expressed in this case report are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense, or U.S. Government."


Pituitary adenomas are the third most common primary intracranial tumor; however, those with postoperative metastases are very rare and are classically considered as pituitary carcinomas. The field of neurosurgery has struggled with diagnosing and treating these unusual lesions. In this report, we retrospectively analyze the clinical features, imaging findings, pathological characteristics and prognosis of one patient with non-hormone-secreting pituitary adenoma who had multiple intracranial and spinal metastases and underwent four surgeries in a 16-year follow-up period. In addition, on the basis of the existing literature, we explore the underlying mechanisms of, as well as the preventive and therapeutic strategies used to treat, pituitary carcinomas and postoperative metastasis of pituitary tumors. Pituitary adenomas are very common and rank third in terms of incidence among all primary intracranial tumors. However, primary pituitary adenomas with intracranial or extracranial metastases are very rare and are commonly defined as malignant pituitary tumors or pituitary carcinomas –7. The fA 40-year-old man was hospitalized (the fourth admission) with a major complaint of declining visual acuity during the preceding 6 months. He underwent his first craniotomy in 1998 for removal of a large pituitary adenoma  showed that his serum hormone levels, including prolactin (PRL) , adrenocorticotropic hormone (ACTH) , human growth hormone (hGH) , insulin-like growth factor 1  and thyroid-stimulating hormone (TSH) , were all normal. His visual field examination indicated bitemporal hemianopsia. Contrast-enhanced magnetic resonance imaging (MRI) of the patient’s brain indicated the presence of three intracranial lesions, with their locations being in the sella turcica , the right frontal lobe  and the anterior cerebral falx , respectively. All lesions demonstrated long T1 and T2 heterogeneous signals and high signal intensity on fluid-attenuated inversion recovery images with significant enhancement , hGH (low +), PRL (low +) and Ki67  Figure .Figure 4The patient was followed from December 1998 (after the first surgery) to December 2014. He achieved a satisfactory recovery after each surgery. No tumor recurrences or metastatic lesions were observed in the sellar region or other regions during the 19-month follow-up period from May 2013 (after the fourth surgery) to December 2014.de novo and whether the hormonal subtype does affect tumor aggressiveness, treatment outcome and prognosis [Pituitary tumors are very common, and most are benign. The World Health Organization (WHO) classification of tumors of the central nervous system 2000) categorizes pituitary tumors into three grades: benign, intermediate or atypical, and malignant . It is w000 categrognosis . Althougrognosis , 14, 15,A literature review revealed that pituitary carcinoma is very uncommon and accounts for only approximately 0.1% to 0.2% of all pituitary tumors , 15–17. et al., the development of metastases followed surgery performed for primary pituitary tumors in 21 (70%) of 30 cases [The spread pathway of tumor cells from a pituitary carcinoma has not been determined, but has been proposed to be varied, including cerebrospinal fluid circulation and lymphatic or hematogenous metastasis to extracranial sites , 20. The30 cases , 22–27. The treatment for pituitary carcinoma should be comprehensive, including neurosurgery, radiotherapy, chemotherapy and medical therapy –3, 15–17In our patient, three intracranial lesions were found in the MRI examination. Because all the lesions were located supratentorially, a one-stage transcranial operation was performed to remove all three lesions simultaneously. Furthermore, although the lesions were intracranial, they were all located at sites outside the brain; therefore, it was feasible to remove the lesions with minimal invasion of the brain tissue. The outcome of the surgery was satisfactory, with no serious postoperative complications. After surgery, radiation therapy, such as fractionated radiation therapy, stereotactic radiosurgery and whole-brain or spinal radiation, should be administered in most cases to prevent tumor relapse , 15, 29.Chemotherapy is commonly used in the treatment of many types of carcinomas; however, its application in the treatment of pituitary carcinoma is just now being tested. The most commonly used drugs are temozolomide and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea in combination with 5-fluorouracil . Other aPituitary carcinoma is very rare. The recommended treatment regimens are comprehensive, including neurosurgery, radiotherapy, chemotherapy and medical therapy. Scientific and professional follow-up is conducive to the early detection of tumor recurrence or postoperative pituitary tumor metastasis. To date, the effectiveness of treatments has not been satisfactory. Future progress in targeted therapy and immunotherapy may provide more effective treatments for this rare malignant lesion.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Background. Primary leiomyosarcoma of the vagina is an exceedingly rare diagnosis. Current estimates are that this tumor could at most represent a mere 0.062% of malignant neoplasms in the female genital tract, although in actuality it is likely far less common. Case Presentation. A 70-year-old female gravida 3 para 2 with new onset palpable vaginal mass and pink vaginal discharge is diagnosed with primary leiomyosarcoma of the vagina. Chemotherapy is complicated by acute Lyme disease, and the patient requires a robotic-assisted total hysterectomy with bilateral salpingo-oophorectomy and partial vaginectomy. The patient remains without recurrence 18 months after surgery. Conclusion. Vaginal leiomyosarcoma is exceedingly rare with an aggressive course, high recurrence, and undetermined ideal treatment regimen. Its diagnosis can be delayed and its presentation varied. Information on this rare tumor type is predominantly through rare case reports with collective consensus on management lacking. The gynecologic oncologist must exercise prudence in individualizing treatment regimens for this rare yet aggressive malignancy. Primary leiomyosarcoma of the vagina is exceedingly rare. The preponderance of carcinomas of the vagina are secondary, with primary tumors representing a much smaller entity . For insA 70-year-old gravida 3 para 2 presented with a palpable vaginal mass and complaints of pink vaginal discharge and perineal discomfort when she sat down over the previous three months. Her past medical history was significant for morbid obesity, arthritis, fibromyalgia, hypertension, depression, and anxiety. She had two previous full term vaginal deliveries and one spontaneous miscarriage and underwent menopause at age 51. Family history was significant for a maternal grandmother, paternal grandmother, and maternal aunt with breast cancer all diagnosed in their seventies and eighties. She denied tobacco, alcohol, or illicit drug use and was retired.On physical examination there was a palpable mass in the posterior lower one-third of the vagina. Transvaginal ultrasound demonstrated 3.0 × 2.5 × 2.2 cm, 1.8 × 1.7 × 1.7 cm, and 0.8 × 0.6 × 0.8 cm vascular lesions within the lower posterior vaginal wall. CT scan of the chest did not demonstrate any evidence of metastatic disease and MRI of the abdomen and pelvis demonstrated only a uterine fibroid, which was not concerning for malignancy. Given the unknown origin of the 3 vaginal masses, resection in the operating room was performed.Two specimens were sent to pathology both showing a smooth muscle neoplasm with increased mitotic activity and moderate to severe cytologic atypia, consistent with leiomyosarcoma. Sections revealed spindle cell neoplasm with moderate to severe cytologic atypia and increased mitotic activity  with focal coagulative necrosis. Immunohistochemical stains were positive for vimentin, desmin, and SMA in tumor cells and were negative for S100, Melan-A, myogenin, and cytokeratin AE1/3. An immunohistochemical stain for Ki-67 was positive and suggested an increased proliferative index (10–15%).According to current classification criteria, it is recommended that smooth muscle tumors of the vagina that are greater than 3.0 cm in diameter (both specimens #1 and #2 measure 3.5 cm in aggregate), with five or more mitotic figures per ten high power fields (present in both specimens #1 and #2), and with infiltrating margins be classified as leiomyosarcoma . MarginsThe patient was discussed at local tumor board and a regimen of chemotherapy with gemcitabine/docetaxel ×4 cycles with plans for surgery afterward was decided upon. During gemcitabine/docetaxel therapy the patient's course was complicated by acute Lyme disease with a visible target lesion on the patient's right arm. She was consequently treated with doxycycline in the midst of her first cycle. After her third cycle the patient had severe fatigue and left lower extremity cellulitis. She was hospitalized for 5 days in the setting of pancytopenia and given IV antibiotics. The 4th cycle of gemcitabine/docetaxel was not given per patient request as she felt unable to tolerate the regimen. Consequently, plans were made to take the patient to the OR instead of receiving additional chemotherapy. She underwent a robotic-assisted hysterectomy, bilateral salpingo-oophorectomy, cystoscopy, and resection of residual vaginal tumor. Surgical pathology showed spindle cell neoplasia from the vaginal tissue consistent with the previous diagnosis of leiomyosarcoma of the vagina. Her uterus, cervix, fallopian tubes, and ovaries showed leiomyomas, but no malignancy. Her pelvic washings were negative. The patient was then treated with standard field pelvic radiation with vaginal high dose radiation for a total dose of 6600 cGy. The patient has remained cancer-free to date, 18 months after surgery.Leiomyosarcomas are largely considered to be a uterine neoplasm and typically arise de novo from uterine smooth muscle, with a rare few originating from preexisting leiomyomas. Despite this rare transformation from a leiomyoma to a leiomyosarcoma, it is not uncommon to find both leiomyomas and leiomyosarcomas in the same specimen. For instance, approximately 0.5% of women who have hysterectomies for leiomyomas are found to also have leiomyosarcomas . NuclearWhen arising from the uterus, these smooth muscle tumors are most commonly seen between the ages of 45 and 55 and can be quite devastating with potential early metastases often by hematogenous spread , 7. ThesLeiomyosarcomas of the vulva and vagina are much rarer entities. For instance, if primary vaginal carcinomas represent 2-3% of malignant neoplasms in the female genital tract, with primary vaginal sarcomas accounting for about 3.1% of these, primary vaginal sarcomas would represent a mere 0.062% of malignant neoplasms in the female genital tract . In otheThere is little literature available on vaginal sarcomas, most of which are case reports . For insWhile surgery is considered the mainstay of treatment, this study also showed that there was no difference in survival between patients who had surgery alone and those who had surgery followed by adjuvant radiotherapy or chemotherapy . AlternaThis case illustrates an example of how a multimodality treatment regimen resulting in a cancer-free patient 18 months after surgery can be utilized in the treatment of vaginal leiomyosarcoma. Specifically, in our case, chemotherapy was initiated after the initial resection of the vaginal leiomyosarcoma was complete. When pathology showed that the tumor was a leiomyosarcoma, chemotherapy had already been begun. Given the rarity of vaginal leiomyosarcoma, it was thought that this was of a likely uterine source and consequently at that time a resection of residual vaginal tumor, a robotic-assisted hysterectomy, and bilateral salpingo-oophorectomy with cystoscopy were performed. After this was complete, standard field pelvic radiation with vaginal high dose radiation was performed. This multimodality method of treatment in our case appears to be highly effective to date, suggesting a potential role for both chemotherapy and radiotherapy in treatment of leiomyosarcoma of the vagina. Interestingly, a similar case of leiomyosarcoma of the vagina, in which hysterectomy, upper vaginectomy, and pelvic radiation alone were utilized without initial chemotherapy, resulted in pulmonary recurrences after merely three months . Only thIn conclusion, vaginal leiomyosarcoma is an exceedingly rare diagnosis with an aggressive course, high recurrence, and undetermined ideal treatment regimen. Its diagnosis can be delayed and its presentation varied. Information on this rare tumor type is predominantly through rare case reports, and collective consensus on ideal management is lacking. As such, the gynecologic oncologist must exercise prudence in individualizing treatment regimens for this rare, yet aggressive, malignancy.


Insulinoma is the most frequently seen functional pancreatic neuroendocrine tumor. The incidence of multifocal insulinoma is lower than 10%. Its treatment is direct or laparoscopic excision. The present case was examined with the findings of hypoglycemia and hypercalcemia, and as there was high insulin and C-peptide levels the initial diagnosis was insulinoma. The case was investigated in terms of MEN 1. During preoperative screening for localization, there was one focus in the head of the pancreas in the abdominal tomography and two foci in endoscopic ultrasonography. No other focus was detected through intraoperative visual or manual palpation. However, five foci were detected during operation by intraoperative ultrasonography. The relation of masses with the main pancreatic canal was evaluated and they were excised by enucleation method. There was no recurrence during the postoperative 18-month follow-up of the patient. As a result, during treatment for insulinoma, it should be kept in mind that there might be multifocal foci. In all insulinomas, the whole pancreas should be evaluated with intraoperative ultrasonography because none of the current preoperative diagnostic methods are as sensitive as manual palpation of pancreas and intraoperative ultrasonography. The intraoperative detection of synchronous five foci in pancreas is quite a rare condition. Insulinoma is the most common cause of endogenous hyperinsulinism related hypoglycemia and it is the most frequent functional pancreatic neuroendocrine tumor (NET). The incidence is presumably between one and three in one million in a year . As it iAbout 5%–10% of patients with insulinoma have MEN 1, which should be excluded or included based on history, symptoms, and radiological and biochemical findings . MEN 1 iCurative treatment could be achieved by total excision. However, at the present time, there are even cases in which localization could not be precisely determined, in spite of several preoperative tests. This condition increases recurrence, morbidity, and mortality.The present case is a very rare case in which five synchronous foci were detected in the pancreas. Additionally, the present case emphasizes the fact that intraoperative ultrasonography (IOUS) still plays an important role in insulinoma and multifocal localization should be kept in mind in all patients.The case was admitted to the hospital with complaints of crying and seizures that had persisted for one year. The intellectually disabled, 20-year-old woman had hypoglycemia and hypercalcemia in the biochemical analysis. Her complaints were thought to be related to neuroglycopenic symptoms. As Whipple's triad was clinically detected, further tests were performed with the initial diagnosis of insulinoma. Furthermore, investigations related to the parathyroid and hypophysis glands were performed to exclude MEN 1.The insulin and C-peptide levels were high . In the Surgery was planned for insulinoma. Following a midline laparotomy under general anesthesia, the pancreas was explored. On palpation, 1 cm nodular lesions were detected in the head and body of the pancreas . In addiThere were a total of five lesions, one in the head of the pancreas, three in the body of the pancreas, and one in the tail of the pancreas, all of which were hypoechoic and with homogenous smooth borders, with the largest one being 1 cm in diameter Figures -5. The sμIU/mL), and the C-peptide level  were normal, and the patient was discharged on the third day. Pancreatic fistulas did not develop in the patient.The postoperative glucose levels (~85 mg/dL), the insulin level (10,36 The pathologies of all lesions were grade I, well-differentiated, pancreatic NET. The number of mitosis was 1, Ki67 proliferation index was 1%, and there were no vascular or perineural invasions.During the 18-month follow-up, the patient was in remission. Intermittent endocrinological follow-up of the patient continues in terms of parathyroid adenoma.Insulinoma is a pancreatic NET, which is generally benign and rarely malignant and can be cured with accurate diagnosis and treatment. It is commonly present in the pancreas; however, its localization within the pancreas might occasionally produce problems. When the tumor is smaller than 1 cm, preoperative and intraoperative localization of the tumor might be difficult . In smalIn our clinic, imaging methods such as abdominal CT, MR, and endoscopic US are preferred in the preoperative screening of insulinoma for localization. Among these, the sensitivity and specificity of endoscopic US are slightly higher. However, its sensitivity also decreases towards the tail. Different methods such as radionuclide scintigraphy, transhepatic portal venous sampling, and selective angiography could be chosen in cases in which localization could not be detected. There is no exact opinion related to the ideal imaging method in the preoperative radiological localization of pancreatic NET. The sensitivity of multislice CT in the detection of pancreatic NET ranges between 71% and 82%. At the present time, recommended CT type by most authors is biphasic or triphasic pancreas visualization . At the In insulinoma cases, the experience in pancreatic surgery and bimanual palpation of the whole pancreas are important. The use of intraoperative manual palpation together with IOUS increases the sensitivity for the localization of insulinoma , 7. FurtIt is important to use IOUS for both the possibility of multiple lesions and also for investigating the relationship of the mass with the Wirsung canal to choose the surgical method. This method is both an easily applied method and of low cost. Especially in cases with MEN syndrome, the risk of multiple foci and malignancy should be considered.Correnti et al.  reportedIn the present case, the preoperative localization of the two lesions was detected with abdominal CT and endoscopic US. However, the three lesions that were found during the intraoperative period have not been detected preoperatively. These lesions could not have been detected visually or by manual palpation. However, it is obvious that the detection of these unseen foci by imaging with IOUS prevents possible recurrences and complications. If IOUS had not been used, these three foci would not have been recognized.The IOUS is also successful in a false nodular adenoma. Thus, unnecessary resection and the risk of fistula could be decreased.One of the important factors that should be mentioned here is the surgical treatment technique. According to the Japanese GEPNET clinical guideline, in lesions that are 2 cm or smaller and in lesions that are more than 3 mm away from the main pancreatic duct, enucleation is recommended rather than pancreatic resection . In the In conclusion, curative surgical treatment with IOUS was performed in the present case and her follow-up continues without any problems.


Spontaneous rupture of hepatocellular carcinoma (HCC) is a rare emergency condition with high mortality rate. Successful management depends on patients' hemodynamic condition upon presentation and comorbidities, correct diagnosis, HCC status, liver function, and future liver remnant, as well as available sources. There is still a debate in the literature concerning the best approach in this devastating complication. Nevertheless, the primary goal should be a definitive bleeding arrest. In most cases, patients with spontaneous rupture of HCC present with hemodynamic instability, due to hemoperitoneum, necessitating an emergency treatment modality. In such cases, transcatheter arterial embolization (TAE) should be the treatment of choice. Emergency liver resection is an option when TAE fails or in cases with preserved liver function and limited tumors. Otherwise, damage control strategies, as in liver trauma, are a reasonable alternative. We report a case of an elderly patient with hemoperitoneum and hypovolemic shock from spontaneous rupture of undiagnosed HCC, who was treated successfully by emergency surgery and damage control approach. Hepatocellular carcinoma (HCC) is an aggressive tumor that often occurs in the setting of chronic liver disease and cirrhosis and it is typically diagnosed late in its course. It is the sixth most common malignancy in the world and the third commonest cause of death from cancer . One of γGT = 70 U/I . Past medical history included coronary artery disease with coronary artery bypass surgery and carotid artery stenting. However, electrocardiogram and cardiac enzymes were within normal values. Abdominal ultrasound showed a hepatic lesion with free intraperitoneal fluid. A contrast enhanced abdominal CT was ordered which demonstrated a heterogenous mass of 7.5 cm diameter occupying the right lobe of the liver, thrombosis of the right portal vein, and free quantity of blood in the peritoneal cavity (A 87-year-old male patient was transferred to the emergency department after an episode of sudden upper abdominal pain and vomiting. On arrival, the patient was pale, tachycardic with a heart rate of 103 beats per minute, and tachypnoeic with a blood pressure of 110/60 mmHg. Physical examination revealed guarding of the right upper quadrant with tenderness. Laboratory examination revealed a hemoglobin level of 10.2 g/dL, normal platelets count, prolonged INR = 1.41, normal liver enzymes, and slightly elevated l cavity . These fHepatocellular cancer is a hypervascular tumor with a high tendency for vascular invasion. However, the mechanism of spontaneous rupture is still not exactly known. Possible causes are rapid growth and necrosis, erosion of a vessel, increased intratumoral pressure caused from the occlusion of hepatic veins by tumor thrombi or invasion, and coagulopathy . SpontanIn summary, the management of spontaneous rupture of HCC is always a troublesome situation for emergency physicians and surgeons. The treatment of this devastating emergency should be closely scrutinized, with stabilization of vital signs and maintenance of hepatic perfusion as soon as possible. Nevertheless, the primary goal should be a definitive bleeding arrest. Although there is no consensus on the most effective treatment, the therapeutic choice must take into account the hemodynamic conditions, functional status of the liver, and stage of the cancer. In general, TAE should be the method of choice as it is less invasive and very efficacious technique and can be done with regional anesthesia. However, for patients with stable vital signs, noncirrhotic liver, and limited tumors, as shown in the abdominal CT scan, immediate hepatectomy can also be considered . For uns


Management of spinal deformities in a patient with arthrogryposis can be challenging for spine surgeons. The literature about the accompaniment of scoliosis; the most common spine deformity reported in arthrogryposis, is still poor. Moreover, the development of cervical kyphosis and thoracic lordoscoliosis in a patient with arthrogryposis is much rare. This paper reports a 26-year-old medical doctor with arthrogryposis who had underwent thoracic lordoscoliosis surgery about ten years ago with T6-L1 internal rods and is now presented with cervical kyphosis and C3-C4 cord stenosis, which made him a candidate for cervical cord decompression surgery. •Arthrogryposis is a non-progressive congenital musculoskeletal disease.•Arthrogryposis may be associated with different spinal deformities.•The intelligence of arthrogrypotic patients should be considered to be normal to above normal. ChildreScoliosis seems to be the most common spinal deformity associated with arthrogryposis. Here we report arthrogryposis in a 26-year-old medical doctor who have been developed cervical kyphosis and thoracic lordoscoliosis of spine in addition to his other musculoskeletal deformities.2This case report is written by the first author who is a 26-year-old medical doctor suffering from arthrogryposis (probably amyoplasia type) and was graduated from medical school last year. He was born with this congenital musculoskeletal disease. Some of his musculoskeletal deformities include left toes fibular deviation, left foot internal rotation, loss of left patella, shortness of left foot (9 cm in comparison to the right foot), flexion contracture of both knees, femoral heads and bilateral hips deformity, bilateral elbows flexion contracture, both wrists deformity, disability in left thumb extension, short stature, spinal lordoscoliosis and cervical kyphosis (with C3-C4 cord compression). This problems made him undergo many reformative surgeries during his life including bilateral Achilles tendons Z-plasty and legs casting for reforming deformities (1989), left hamstring muscle transfer (1998), left dislocated patella removal and left knee reformative surgery (2001), left femoral growth plate closure (2005), lordoscoliosis surgery with internal rods in T6-L1 (2005) . RecentlHe has no mental or psychological problem. He does not smoke, drink alcohol and take no specific medication. He has normal facies with no craniofacial abnormality. There is no one with similar problems in his relatives. His parents are cousins and he has five siblings who are completely healthy. Although his mother reports of a suspicious history of trauma in her pregnancy period.Last year, while studying hard for the residency examination after the graduation, he felt lots of pain in his neck in addition to paresthesia in hands and foots. He had a complaint of paresthesia for about two years but simultaneously with studying, it became very severe and made him stop studying. He consulted a neurosurgeon who found some neurological symptoms like hyperreflexia in addition to Babinski and Hoffman reflexes in the physical examination. He was referred for a total spine MRI, in which C3-C4 cord compression was reported . After t3Arthrogryposis is a not a diagnosis but a clinical finding. There are about twenty subtypes of arthrogryposis multiplex congenita based on clinical and genetic differences. Research has shown that there are more than thirty-five specific genetic sequence disorders associated with arthrogryposis . The cauThere are three major groups of arthrogryposis; amyoplasia, distal arthrogryposis, and syndromic arthrogryposis. Amyoplasia is characterized by severe joint contractures and muscle weakness. Patients have a symmetrical joint or limb involvement with normal sensations. The intelligence is normal to above normal in children with amyoplasia  however The most common reported spinal deformity in arthrogryposis is scoliosis. 4Arthrogryposis as a non-progressive congenital musculoskeletal disease may be associated with different spinal deformities which is reported less common in the literature. The intelligence of arthrogrypotic patients should be considered to be normal to above normal.The patient has consented to the submission of the case report for submission to the journal.


Dear Editor,A 36-year-old female presented with low back pain and was admitted. During the anamnesis,she reported having undergone surgery, in the first days of life, for the repair oflumbosacral myelomeningocele and imperforate anus. She had been a full-term infant, bornto an adolescent mother who had received no prenatal care. There were no reports ofinfection, drug use, or exposure to teratogenic agents during gestation. Computedtomography of the urinary tract showed ectopic kidneys, with a rotational anomaly andbilateral nephrolithiasis. In addition, as an incidental finding, we identified acongenital  alteration of the lumbosacral spine,characterized by a fusion defect and failure of vertebral body segmentation, with theformation of two axes, starting from the third lumbar vertebra. Other imaging tests forthe evaluation of the lumbosacral spine confirmed the findings and demonstrated twomedullas within a single dural sac, separated by a thin fibrous septum . These f3. Given the scarcity of published cases,it is not possible to accurately establish associations with other abnormalities. Anextensive review of the literature showed that approximately 500 cases have beenreported and that only a minority of the affected individuals reach adulthood6.SNS results from a rare congenital malformation defined as a cleft in the spine, forminga double spinal column, due to failed fusion of the lateral ossification centers of thevertebral bodies. It occurs most often in the thoracolumbar region; when it affects thelumbosacral region, it can be accompanied by imperforate anus and meningocele. Otheranomalies, involving the gastrointestinal tract, central nervous system, andgenitourinary tract-manifesting as dorsal enteric fistulas, hydrocephalus, and bladderexstrophy-have been reported7.In most cases of SNS, there are no reports of known exposure to teratogenic agents or afamily history of birth defects. Although the embryogenesis of the anomaly remains underdiscussion, there is evidence that a primary defect in the initial division of thenotochord, neurenteric canal, and paraxial mesoderm-with a persistent connection betweenthe endoderm and ectoderm, causing division or deviation of the notochord-is associatedwith variety of malformations. Recent hypotheses are based on vascular impairment of theinferior neural structure, which would prevent the closure of the neural tube8.Double spines and spinal cords can be observed in SNS and in caudal duplication syndrome,covering a wide spectrum of malformations, ranging from simple fibrous bands dividingthe medulla to complete duplication of the caudal structures. A diagnosis of caudalduplication syndrome should be considered only when there is also duplication ofvascular structures or organs, the genitourinary tract, the gastrointestinal tract, andthe distal neural tube2. Theaim of this case report was to discuss the diagnosis of SNS, a rare condition,associated with congenital anomalies and high mortality. Therefore, we propose that,when spinal defects are detected, a detailed investigation of the associated findings becarried out, thus avoiding diagnostic errors and delays.Among individuals with SNS, the reported survival and prognosis are poor. Including thecase reported here, there have been only five reports in which the patient survived.However, with the advances in surgical techniques and neonatal intensive care, there isa trend toward better outcomes