The malignant form of atrophic papulosis (Köhlmeier-Degos disease) is a rare thrombo-occlusive vasculopathy that can affect multiple organ systems. Patients typically present with distinctive skin lesions reflective of vascular drop out. The small bowel is the most common internal organ involved, resulting in considerable morbidity and mortality attributable to ischemic microperforations. Determination of the presence of gastrointestinal lesions is critical in distinguishing systemic from the benign, cutaneous only disease and in identifying candidates for treatment.We describe an 18 year old male who first presented with cutaneous atrophic papulosis but became critically ill from small bowel microperforations. He had an almost immediate and dramatic response to treatment. Prior to his presentation with acute abdomen he had upper and lower endoscopy showing areas of nonspecific patchy erythema. At laparotomy, innumerable characteristic lesions with central pearly hue and erythematous border were seen. PubMed was used for a literature search using the keywords malignant atrophic papulosis, Degos disease, endoscopy, laparoscopy and laparotomy. This search yielded 200 articles which were further analyzed for diagnostic procedures and findings.Among the 200 articles we identified only 11 cases in which endoscopy was performed. Results of endoscopy and laparotomy in our patient with malignant atrophic papulosis were compared to those in the literature. Endoscopy of the gastrointestinal tract has shown gastritis and non-specific inflammation whereas laparoscopy shows white plaques with red borders on the serosal surface of the small bowel and the peritoneum. From personal communications with other physicians worldwide, we identified three additional unpublished cases in which endoscopy revealed only minimal changes while laparoscopy showed dramatic lesions. From our experience the endoscopic findings are often subtle and nonspecific, whereas laparascopy or laparotomy will reveal pathognomic lesions on the serosal surface of the intestine.Our report contrasts the endoscopic and laparoscopic findings in malignant atrophic papulosis which suggest laparoscopy is the more powerful means of detecting gastrointestinal involvement. Imaging studies may serve as a key indicator of systemic progression. Based on our experience, laparoscopy should be performed when there is a high index of suspicion for gastrointestinal malignant atrophic papulosis, even if endoscopic examination is non-diagnostic or normal.The online version of this article (doi:10.1186/s12876-015-0387-y) contains supplementary material, which is available to authorized users. Atrophic papulosis (Köhlmeier-Degos disease) is a rare thrombo-occlusive vasculopathy that can affect multiple organ systems. Patients primarily present with distinctive skin lesions reflective of vascular drop out. The small bowel is the most common internal organ involved, resulting in considerable morbidity and mortality attributable to ischemic microperforations –3. Skin New hypotheses to the pathogenesis of MAP suggests two forms of vasculopathy which are mechanistically distinctive and have helped identify new treatment options. One aspect is a thrombotic microangiopathy attributable to endothelial cell injury. Prior studies have shown extensive vascular deposits of C5b-9 as the basis of injury analogous to other catastrophic C5b-9 mediated microvascular injury syndromes such as atypical hemolytic uremic syndrome . EculizuDetermination of the presence of gastrointestinal lesions is critical. Untreated, individuals with such lesions will have progressive disease culminating in repeated bowel perforations and death. At present, there is no consensus on the best means of detecting gastrointestinal lesions in patients with cutaneous atrophic papulosis.We describe an 18 year old male who presented with cutaneous atrophic papulosis but became critically ill from small bowel microperforations. We also describe his sister, who developed similar skin lesions compatible with a familial variant. PubMed was used for a literature search using the keywords malignant atrophic papulosis, Degos disease, endoscopy, laparoscopy and laparotomy. The search yielded 200 articles which were further analyzed for diagnostic procedures and findings. Also personal communications with other physicians caring for individuals with systemic MAP led to evaluation and comparison of gastrointestinal imaging modalities.Patient One is an 18 year old male who presented with cutaneous disease, had rapidly proliferating skin lesions over a course of a year, and then survived small bowel microperforations from MAP Fig. . Upper ePatient Two is the sister of Patient One. She slowly developed a small number of cutaneous lesions that were biopsied and were consistent with atrophic papulosis. She had minor gastrointestinal discomfort. For this reason and due to her brother's history a laparoscopy was performed with normal findings. Her abdominal discomfort spontaneously resolved. Given the lack of GI findings and the highly limited nature of the cutaneous presentation she is less likely to progress to systemic MAP.A 44 year old female with cutaneous lesions of atrophic papulosis developed sudden monocular vision loss and severe eye pain requiring enucleation with pathologic findings of microvascular changes in the orbit consistent with MAP. Colonoscopy in April 2012 showed normal findings but exploratory laparoscopy in October 2012 revealed innumerable MAP lesions  for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


An indirect inguinal hernia containing an incarcerated fallopian tube and ovary is extremely rare in adult females. The current report describes a woman of reproductive years presenting with an irreducible indirect hernia which required the surgical intervention of a general surgeon as well as counseling regarding future fertility by a gynecologist. The diagnosis was made by physical and sonographic examination and was confirmed by CT scan and surgical intervention. We suggest a multimodel and multidisciplinary approach in order to safely and efficiently preserve ovarian and fertility function in young women who present with an inguinal hernia containing reproductive organs. Inguinal hernias occur in less than 5% of women . Even thThese hernias may contain a variety of visceral organs, of which intestines or omentum is most commonly found, resulting in an incarceration or strangulation which would be a surgical emergency. The ovary or fallopian tube may also become entrapped, although this is rarely considered. The patient may present in a nondescript manner and describe a heaviness or dull discomfort in the groin that is most pronounced when intra-abdominal pressure is increased. Typically, hernias can be diagnosed by careful palpation on physical exam if it is considered and confirmed by ultrasound imaging. However, when ultrasound results remain ambiguous and there is concern for entrapped organs, a CT scan can be performed to provide definitive diagnosis and aid in proper counseling, consultation, and timely decision making for urgent surgical management in order to avoid organ necrosis.We present a rare case of a woman with an indirect, nonreducible inguinal hernia containing a normal appearing ovary and fallopian tube which required urgent surgical intervention to prevent strangulation of the adnexa and to ensure fertility preservation.A 25-year-old G2P1011 presented to the Emergency Department with increasing left groin pain that radiated to the mons for the past five days. The patient noticed a small bulge in the groin area over the past five months but denied pain previously. Over the past five days, however, the patient reported having immediate pain after valsalva, for example, after each bowel movement. She denied any urinary symptoms, nausea, or vomiting. She had no significant past medical history or surgical history.Physical exam revealed a 2 cm × 2 cm mass over the left mons abutting the pubic symphysis. The mass was tender to palpation. The abdomen was soft, nontender, and without peritoneal signs. Vital signs were BP 119/67, HR 94, Temp 98.3, and RR 16. Lab studies including chemistry panel, complete blood count, and urine pregnancy test were within normal limits.The patient was initially evaluated by emergency room physicians, and an ultrasound was ordered to further determine the contents of the inguinal mass. The ultrasound report findings suggested “thick walled, complex tubular fluid collection abutting and inseparable from the left ovary and extending into the left groin, possibly representing a dilated and herniated fallopian tube.” Given the concern for possible entrapped organs, a CT scan was ordered, and consultation requests for both General Surgery and OB/GYN were obtained . The CT A bedside hernia reduction was attempted but was unsuccessful. The patient was evaluated by OB/GYN and counseled regarding the possibility of unilateral salpingo-oophorectomy if the adnexa appeared to be necrotic. The patient was taken for exploration and she underwent an open left inguinal hernia repair with mesh placement. Examination revealed an indirect hernia containing an incarcerated, dilated fallopian tube with a healthy, viable ovary sitting within the hernia sac. The contents were easily reduced back into peritoneal cavity without difficulty. The inguinal floor was reinforced with Prolene mesh.Inguinal hernias consist of direct hernias  and indirect hernias  . In adulIn contrast, the occurrence of entrapped adnexa within an indirect hernia in women, as seen in our patient, would be unexpected and uncommon. When discovered in adult females, the majority of hernias reported are found in perimenopausal or postmenopausal women , 7. Howen = 4/7) [n = 4/15) of those who presented with irreducible ovaries were found to have infarcted ovaries at surgery [Timely management must be undertaken to ensure prompt surgical intervention to reduce the risk of ovarian damage and subsequent infertility. This will likely require collaborative efforts by gynecologists and general surgeons and recognition of this condition by both parties in order to ensure preservation of fertility. In past reports where female adnexa were involved, more than half required oophorectomy secondary to strangulation (n = 4/7) . When ex surgery  as reporIn summary, we have described the rarity of a normal fallopian tube and ovary within an indirect inguinal hernia in a premenopausal adult female. Furthermore, diagnosis of entrapped viscera including the adnexa must be considered in the differential diagnosis of hernias in adult women, in order to ensure proper surgical and medical management to ensure preservation of fertility. Surgical intervention in a timely fashion may be necessary in order to prevent and relieve torsion and to return normal perfusion to the adnexa. Multiple imaging studies may be necessary to assist in diagnosis, including ultrasound and/or cross-sectional imaging by computed tomography (CT). By considering a gynecologic etiology when faced with inguinal hernias in women and using a multimodel approach including history, physical examination, imaging, and surgical management, one can safely and efficiently preserve ovarian and fertility function in young women.


Inflammatory myofibroblastic tumors are rare in the pediatric population. Most common localizations were reported in the lungs. A localization in the pancreas needs differentiation from other tumors and chronic pancreatitis. Treatment is surgical resection, although there are reports of treatment with oral steroids and radiation therapy.A 6-month-old child was treated due to a tumor in the head of the pancreas. On admission he was jaundiced with pruritus. US and MRI confirmed pancreatic tumor. Preoperative biopsy wasn’t conclusive regarding the nature of the tumor. Duodenopancreatectomy was performed. Postoperative course was uneventful. Histologic examination confirmed the diagnosis of inflammatory myofibroblastic tumor. On follow up, he remained with no evidence of recurrence.A literature review revealed 10 cases of pancreatic inflammatory myofibroblastic tumors in the pediatric age group. Our patient is the youngest reported. Despite major resection, there were no complications. However, management of this child might be possible with steroids, but conservative treatment might be insufficient, especially in aggressive forms of tumors. Human herpes viruses 3 and 8, Eikenella corrodens and Epstain Barr virus have been also proposed as possible infectious triggers of the IMT.Inflammatory myofibroblastic tumors (IMT’s) are rare solid lesions that occur primarily in visceral and soft tissue. Most frequently they occur in the first two decades of life. The most common localizations of IMTs have been reported in lung, mesentery and omentum.Clinically and radiologically, an IMT can be confused with malignancy. A localization of IMT in the pancreas is very rare and needs differentiation from other tumors and chronic pancreatitis.The macroscopic appearance of IMT is usually well-circumscribed or multinodular, white, firm mass. Histological, IMT is composed of spindle-shaped myofibroblasts or fibroblasts accompanied by a mixed inflammatory infiltrate of eosinophils, plasma cells, and lymphocytes.7We present a case of 6 months old male, who was referred to our department due to an IMT of the pancreatic head, which caused jaundice and pruritus. To the best of our knowledge, this is the youngest child with this type of tumor published so far in the literature.A 6-months old boy was transferred to our hospital with a 4-days history of jaundice and pruritus. On examination he was jaundiced with no organomegaly. There was a rash on the trunk and extremities. Liver function tests revealed a direct bilirubin level of 109 μmol/L , alkaline phosphatas level of 11.62 μkat/L  and γGT level of 3.23 μkat/L .An ultrasound scan (US) of his abdomen identified a 40 mm large mass in the head of the pancreas. The common bile duct was dilated, the gall-bladder was extremely enlarged, but there was no dilatation of intrahepatic bile ducts and pancreatic duct . The resWhipple’s procedure was performed due to biliary obstruction and possible malignancy .Histological examination revealed an infiltrative growth in the pancreatic head, mainly surrounding and destroying pancreatic acini , but alsBy immunohistochemistry, the lesional cells were smooth muscle actin positive proliferation of bland spindle cells forming a storiform and vague fascicular growth pattern, admixed with areas displaying more epithelioid morphology and variably prominent inflammatory cell infiltrate, composed of lymphocytes, plasma cells and eosinophilic granulocytes, while stainings for cytokeratins, S100, desmin, H-caldesmon and ALK were negative, confirming myofibroblastic differentiation of the lesional cells. Although the histological and immunohistochemical features were suggestive of inflammatory myofibroblastic tumour, an unusual form of chronic pancreatitis could not be reliably excluded.The postoperative course was uneventful. The boy was discharged on the 14th postoperative day. Over the next 3.5 years of follow up, he remains well and with no clinical or radiological evidence of recurrence.Pancreatic tumors are rare in childhood, accounting for only 0.2% of childhood malignancies.14Histologically, inflammatory myofibroblastic tumors are characterized by irregular proliferation of myofibroblasts intermixed with inflammatory cells, mainly lymphocytes and plasmacytes. They are subcategorized into fibrohystiocytic type, plasma cell granuloma type, largely sclerosed or fibrosed type, hypocellular fibrous type and myxoid/vascular type.Only 28 cases of pancreatic inflammatory myofibroblastic tumors have been reported so far, 60% being located in the pancreatic head.Compared to this data, our patient is the youngest child with inflammatory myofibroblastic tumor of the pancreas reported in the literature. The main features at presentation were pruritus, jaundice, abdominal mass, lethargy, vomiting, fever and anemia.28Besides surgical resection, alternative therapeutic regimens are still lacking. While systemic immunosuppresive treatment with steroids, chemotherapy and radiation therapy have been reported for unresected or recurrent cases of extrapancreatic inflammatory myofibroblastic tumors.33In the literature there are only two reported cases of pancreatic inflammatory myofibroblastic tumors that were not treated with resection.Chronic pancreatitis could not be completely excluded according to histological examination. Anyway, specific causative factor for chronic pancreatitis was not identified. In addition, pediatric patients present with chronic pancreatitis much later (average age 6 ± 4 years) than it developed in our patient (6 months).We report a case of pancreatic inflammatory myofibroblastic tumors in a six month old male child treated with surgical resection. This is the first case report of an infant with IMT. In addition, the tumor is rarely described in the pancreas. Despite major surgery no complications evolved in long term follow up.


A 46-year-old man, who had visited our hospital complaining of a small intrascrotal nodule ten years ago, returned to us because of the rapid growth of the nodule. Computed tomography revealed a heterogeneously enhanced intrascrotal tumor of approximately 4 × 3 cm. The tumor and the right testis were excised with the adhered right scrotal skin. The pathological diagnosis was pleomorphic leiomyosarcoma with dedifferentiation originating from the dartos muscle. Urological dedifferentiated leiomyosarcomas are rarely reported and the clinical features are mostly unknown. This is the first report to describe the dedifferentiated leiomyosarcoma of the dartos muscle. Dedifferentiation is a well-recognized process in several bone and soft tissue tumors, including liposarcoma, chondrosarcoma, periosteal osteosarcoma, chordoma, and solitary fibrous tumor . HoweverIn 2004, a 46-year-old man visited our hospital with a complaint of a small intrascrotal nodule and was placed under observation. In January 2014, he noticed rapid growth of the nodule and visited us again. Physical examination showed a mass in the scrotum near the right testis. Blood tests yielded no specific results. Contrast-enhanced computed tomography revealed a heterogeneously enhanced tumor of approximately 4 × 3 cm. Magnetic resonance imagingshowed a heterogeneous signal in T2 weighted image and early contrast enhancement and washout . There wMicroscopically, the tumor consisted of two different components: leiomyosarcoma and malignant fibrous histiocytoma-like dedifferentiated sarcoma . ImmunohThe patient had no evidence of recurrence at six months after the operation.Soft tissue sarcomas are a heterogeneous group of nonosseous tumors that arise from the embryonic mesoderm . In thisChen et al. reported that dedifferentiated leiomyosarcoma was seen in 1.4% of all leiomyosarcomas consulted from 1991 to 2007 [As for grading systems of sarcomas, the French Federation of Cancer Centers Sarcoma Group grading system has been shown to be reproducible among pathologists and correlate with the clinical outcome , 8. In tThe optimum local and systemic treatment for these tumors remains controversial, but there is a general consensus that all paratesticular sarcomas in adults should be managed with complete resection, including high ligation of the spermatic cord . Prognos


Appendiceal diverticulosis is a rare condition. Herein reported is a case of appendiceal diverticulosis and diverticulitis clinically masquerading as appendiceal carcinoma. A 62-year-old woman presented with abdominal pain. US and CT showed a tumor measuring 5 × 4 × 4 cm in vermiform appendix. Colon endoscopy showed mucosal elevation and irregularity in the orifice of vermiform appendix. A biopsy of the appendiceal mucosa showed no significant changes. Clinical diagnosis was appendiceal carcinoma and wide excision of terminal ileum, appendix, cecum, and ascending colon was performed. Grossly, the appendix showed a tumor measuring 5 × 3 × 4 cm. The appendiceal lumen was opened, and the appendiceal mucosa was elevated and irregular. The periappendiceal tissue showed thickening. Microscopically, the lesion was multiple appendiceal diverticula. The diverticula were penetrating the muscle layer. The mucosa showed erosions in places. Much fibrosis, abscess formations, and lymphocytic infiltration were seen in the subserosa. Abscesses were also seen in the diverticular lumens. Some diverticula penetrated into the subserosa. The pathologic diagnosis was appendiceal diverticulitis. When they encounter an appendiceal mass, clinicians should consider appendiceal diverticulitis as a differential diagnosis. The diverticular disease of vermiform appendix is a rare condition , 2. It mA 62-year-old Japanese woman with HCV-related cirrhosis and small hepatocellular carcinoma presented with abdominal pain. The small hepatocellular carcinoma had been treated with microwave coagulation therapy and cured. A blood laboratory test revealed elevation of cirrhosis-related enzymes, bilirubin, and AFP. No leukocytosis or increased C-reactive protein was present. CA125 was elevated. US and CT showed a tumor measuring 5 × 4 × 4 cm in the vermiform appendix . Colon eGrossly, the appendix shows a tumor measuring 5 × 3 × 4 cm . The appThe appendiceal diverticulum has been classified into congenital and acquired ones , 2. The It is interesting that the present case was clinically diagnosed as appendiceal carcinoma. In a review of the English literature, the author could not find cases of appendiceal diverticulitis clinically masquerading as appendiceal carcinoma. The lack of blood inflammatory changes and positive mass formation in the imaging modalities of appendix led to the erroneous clinical diagnosis of appendiceal carcinoma in the current case. Therefore, it must be stressed that clinically appendiceal tumor may be due to appendiceal diverticulosis or diverticulitis. In recent years, relationship between appendiceal diverticulosis and appendiceal tumors or tumor-like conditions has been advocated.Dupre et al.  describeIn summary, when they encounter an appendiceal mass, clinicians should consider appendiceal diverticulitis as a differential diagnosis.


Ovarian torsion in the third trimester of pregnancy leading to a midline laparotomy and caesarean section for the delivery of a preterm baby is an uncommon event. As the woman is likely to present with nonspecific symptoms of lower abdominal pain, nausea, and vomiting, ovarian torsion can often be misdiagnosed as appendicitis or preterm labour. Treatment and the opportunity to preserve the tube and ovary may consequently be delayed. We report the case of a multiparous woman who had undergone two previous caesarean sections at term, presenting at 35 weeks of gestation with a presumptive diagnosis of acute appendicitis. Ultrasonography described a cystic lesion 6 × 3 cm in the right adnexa, potentially a degenerating fibroid or a torted right ovary. MRI of the pelvis was unable to provide further clarity. The patient was managed by midline laparotomy and simultaneous detorsion of the ovarian pedicle and ovarian cystectomy together with caesarean section of a preterm infant. This report describes that prompt recognition and ensuring intraoperative access can achieve a successful maternal and fetal outcome in this rare and difficult scenario. Furthermore, we would like to emphasise that the risk for a pregnant woman and her newborn could be reduced by earlier diagnosis and management of ovarian masses . A 33-year-old woman was booked for hospital care because of two previous caesarean deliveries. The first was an emergency caesarean at 42 weeks of gestation for fetal distress in labour. The second was undertaken for failure to progress in spontaneous labour. In this pregnancy her last ultrasound scan was at 20 weeks of gestation and revealed no fetal abnormalities.The patient presented at 35 + 2 weeks of gestation, with a 4-hour history of sudden onset and severe and constant abdominal pain in the right iliac fossa. She found changing position incredibly painful and examination displayed involuntary guarding and rigidity of the right side of her abdomen. The pain was associated with uncontrollable vomiting. There was no history of vaginal loss or bleeding and normal fetal movements had been felt.On examination, the patient was in obvious distress. She was normotensive and tachycardic; pulse rate was 110 bpm; respiratory rate was 16/min; and oxygen saturations were 100% in air. She was afebrile. Abdominal palpation revealed an exquisitely tender abdomen with rigidity and guarding on the lower right side. Acute appendicitis was suspected and a prompt review by the surgical team was undertaken.Ultrasound assessment on the labour ward demonstrated fetal heart movements, cephalic presentation, and an anterior high lying placenta. Cervical length was 32 mm. Fetal monitoring using cardiotocography was reassuring.The patient was managed conservatively overnight, was nil by mouth, and required high doses of oral morphine and antiemetics. A pelvic ultrasound scan revealed a right sided 6 × 3 cm cystic lesion, consistent with a degenerating fibroid or a torted ovary. A previous USS of the abdomen 3 years earlier commented on a 3 cm right ovarian dermoid cyst. The patient subsequently had a prompt MRI scan, though it was unable to provide further clarification on the aetiology of the pain. With the clinical presentation of an acute abdomen and severe vomiting requiring regular need for analgesia, the decision for a midline laparotomy was made. Given the patient's obstetric history of two previous caesarean sections, the decision for an emergency caesarean section was made by the obstetric and the paediatric consultant. The patient had had one dose of dexamethasone injection a few hours prior to delivery.Differential diagnoses included appendicitis, degenerating fibroid, and ovarian torsion.Consent was taken for a category 2 emergency caesarean section. On opening the abdominal cavity through a midline laparotomy incision, a large purple but not necrotic right sided mass was noted. The caesarean section was performed initially, delivering a female infant. Apgars were 9 and 10 at 5 and 10 minutes and she was transferred to the neonatal unit for assistance with breathing and observation. The placental delivery was by controlled cord traction. Syntocinon infusion was commenced due to uterine atony. The uterus was closed in two layers and a number of haemostatic sutures were required at the midline. The right ovary was then examined and it was torted twice and appeared as a purple enlarged structure of 7 × 4 cm . There wThe patient made a good recovery and histological examination of the ovarian cyst confirmed a dermoid cyst . FurtherWith regard to the natural history of ovarian cysts discovered during pregnancy, it is believed that 10% will be operated on soon after diagnosis whilst a further 2% will require intervention later on in view of painful complications. A further 3% may be removed at caesarean section or in the puerperal period [Benign dermoid cysts/teratomas are the most frequent ovarian tumors, with an incidence ranging from 5% to 25% of all ovarian neoplasms . They arTorsion of the ovary in the third trimester is rare as the compressive effect of the gravid uterus restricts the mobility of the ovarian pedicle. However this case clearly demonstrates that it can occur and needs to be considered as a differential diagnosis when patients present with an acute abdomen. Although conservative treatment has been proposed during pregnancy, surgical intervention is the treatment of choice once ovarian torsion is highly suspected .Additionally this case highlights the difficulty in producing good quality radiological imaging of the pelvic organs in advanced pregnancy. Radiologists often have limited experience of pelvic imaging in the third trimester, so in all but the most experienced hand, a definitive diagnosis may not be forthcoming. This case serves to remind us of the importance of clinical acumen alongside diagnostic test as well as ensure that the correct incision is performed to ensure good surgical access. Furthermore, ultrasound scan examinations in early pregnancy should also address the cervix and the adnexa leading to early diagnosis and management of ovarian masses, thus avoiding later emergency situations and the possibility of preterm deliveries.


Cerebral Cavernous malformations (CCM) occur in less than 1% of the general population, and one-third may have more than one lesion. Headaches alone may accompany CCM in up to 10% patients although micro-haemorrhage with accompanying headache is more common.A 67-year old male presented with a 15-year history of episodes of sudden onset left facial pain with dysesthesia in the fifth cranial nerve V1 distribution and left sided headache. The frequency of facial pain has increased with daily episodes of occurrence, exacerbated by movements. His headache impact score HIT-6 was 71. The patient’s family history is a sister died of a stroke, another from multiple sclerosis and a nephew died at 15-years-old of cerebral haemorrhage. On examination there were signs consistent with a brainstem lesion. Extensive scanning demonstrated no haemorrhage into any lesion and confirming multiple foci including a pontine lesion. Genetic testing confirmed a CCM2 mutation heterozygous deletion of two-nucleotides and 50% risk of inheritance of the c76_77del to family members.Pontine CCM can precipitate in migrainous-type headache and atypical facial nerve distribution symptoms due to disruption of the trigeminovascular system (TGVS). The Periaqueductal Grey area (PAG) is involved in modulating pain through both ascending and descending projections, the latters connections to the TGVS that inhibits nociceptive afferent information. CCM lesion is also disrupting the Nucleus Raphe Magnus (NRM), acting as a migraine generator.The diagnosis for the cause of this intractable facial pain and headache is the large pontine CCM lesion disinhibiting fibres of the TGVS.No conflict of interest.


The presence at CT scan of more retained bullets than expected could be a very difficult interpretation challenge in the early management of gunshot wounds. The modern non operative management of haemodinamically stable patients without peritonitis requires that the trajectory of the bullet is clearly recognized. This clinical case reporting of a gunshot wound without evident entry hole, allows to discuss the diagnostic and therapeutic implications in the management of gunshot wounds cases with atypical entry and/or exit holes. In case of abdominal gunshot wound, exploratory surgery has always been deemed indicated, as the likelihood that the bullet has caused a perforation of the gastrointestinal tract is high; unlike the lesions of solid organs, characterized by haemoperitoneum easily detectable by CT scan, intestinal perforation may not be immediately recognized, especially when the CT is carried out quickly, so that the air contained in the bowel has not the time to go outside and to be recorded as an indirect sign of perforation. However some Authors have recently proposed a more selective indication for surgery, based on serial CTs, in cases where the first CT is negative and the patient is haemodinamically stable, with the aim of avoiding unnecessary laparotomies. Two recently published papers highlight the role of laparoscopy in non-operative management of abdominal gunshot wounds, expecially when clinic is not clear or in presence of peritoneal signs , 2.Obviously a quite different problem arises when entry and/or exit holes of the bullets are not clearly detectable, as highlighted by the reported singular case of unrecognized gunshot entrance hole, due to the fact that the patient was shot through his anus. To our best knowledge in scientific Literature there is any case of undetected entrance hole due to trans-anal gunshot, even if we found out 2 rare cases of unrecognized gastrointestinal bullet embolism –5. We thThe Patient, a 60-years old Caucasian male found unconscious in a trailer park of gypsies, was carried to Emergency Department at night, intubated and haemodynamically stable. At physical examination he presented a head gunshot wound, with an only visible bullet-hole located at the back of the neck. No other lesions were found. The total body contrast-enhanced CT scan performed at the Emergency Department revealed a large subdural haematoma, a retained bullet in the brain Fig.  and anotAbout 14 h later the second CT scan showed the presence of hematoma near the pubis, peritoneal air bubbles outside the bowel Fig. , b and dThe following course of the patient was uneventful; he was estubated after 3 days, having a Glasgow coma scale of 11, and he was discharged to rehabilitation 13 days after the incident; 4 months later the head bullet was removed by anterior approach, while the lung bullet was left in place.From the analysis of this clinical report we concluded that the patient received two gunshot: the first was the only one that could be initially detected and it was fired to the back of the neck, while the second was fired directly throughout the anus; it perforated the rectum, and then it crossed the abdomen causing intestinal, gastric and diaphragmatic perforations, arresting into the left lung. It was not possible to make a correct diagnosis at first observation because a second bullet-hole could not be found.To our best knowledge no similar cases have been reported in Literature. We only find 2 rare cases of unrecognized bullet embolism in the gastrointestinal tract causing colonic perforation , 4. In bMany problems in the interpretation and reconstruction of the trajectory of the bullets have been reported in the Literature , 7; a hiTo our knowledge, this is the first report of the absence of an evident entry wound; since the CT scan showed the presence of a retained bullet in the left lung, the search for an entrance hole had been conducted with great attention by the surgeon, the anesthesiologist and the radiologist together. One possible explanation that was proposed was that the bullet had been swallowed and inhaled and then it had taken the path of a segmental bronchu. A second possible explanation was that the pulmonary bullet was fired many times later, during another gunshot assault. Retrospectively reassessing the information available after the first CT scan, the presence of air bubbles and small bone fragments in the extraperitoneal pelvis should have lead to a rectal examination. This was a major missing in the management of the here reported clinical case, even if the absence of indirect signs of bowel perforation was on contrast with the suspicion that the projectile had been shot from the anus and it reached the lung crossing the abdomen.Performing the rectal examination could have anticipated the understaning of the sequence of the events, but probably the patients management would have not been different, due to his clinical stability and in absence of clinical signs of bowel perforation.So the decision to repeat the CT scan after a few hours in order to monitor the presence of any sign of possible damage in the peritoneal cavity, was correct as allowed the diagnosis of the intestinal injures before the onset of clinical symptoms, avoiding the patient to die for septic shock , 6. As aFirearm wounds are increasingly observed in Emergency Department even in areas with classically low incidence of urban warfare, especially in Europe; they often raise new problems in interpretation both for denial by patients of firearm involvement  and for 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.


Streptococcus, the causative pathogen, was administered. The VP shunt tube was replaced. The postoperative course was uneventful, and postoperative CT showed hydrocephalus improvement and no pseudocyst recurrence. Abdominal pseudocysts, which are rare after VP shunt surgeries, usually occur after the subacute postoperative course in younger cerebral hemorrhagic cases. Our case was quite rare because the cyst developed in the chronic phase in an older patient and was caused by streptococcal infection. The cyst components should be examined before cyst drainage when choosing surgical strategies.In 1999, a 50-year-old woman underwent ventriculoperitoneal (VP) shunt surgery for hydrocephalus after subarachnoid hemorrhage. She was hospitalized for fever and recurrent systemic seizures in November 2006. Head computed tomography (CT) showed only old changes. The seizures and fever were controlled by medicinal therapy. However, in December, her consciousness level suddenly decreased, and she showed progressive lower abdominal distension. Head CT showed marked ventriculomegaly, and abdominal CT showed a giant cystic mass at the shunt-tube tip in the lower abdominal cavity. Because thick pus was aspirated from the intra-abdominal mass, we diagnosed the patient with acute obstructive hydrocephalus due to an infected abdominal pseudocyst. Laparotomy and direct cyst drainage were performed, and antibiotic therapy against  Complications after ventriculoperitoneal (VP) shunt operations are observed relatively often. However, among these complications, intraperitoneal pseudocysts (IPP) are rare. We describe a case of an IPP that occurred 7 years after a VP shunt operation in a patient who was infected with pseudomonas and who presented with acute hydrocephalus due to shunt malfunction.A 50-year-old female was transferred to our hospital in November 2006 because of recurrent attacks of epilepsy and fever. In 1999, she had undergone an aneurysm clipping surgery after subarachnoid hemorrhage (SAH) in our institute and had also had a VP shunt operation against SAH-induced hydrocephalus after a month from initial clipping. After that, she received long-term rehabilitation after being transferred to another hospital.μL; C-reactive protein levels: 5.2 mg/dL). Head computed tomographic (CT) scans that were performed upon admission showed mild hydrocephalus and ischemic lesions that were due to damage from the vasospasms that occurred after the SAH , and hemiplegia of the right side due to a cerebral infarction that was triggered by vasospasms after the prior SAH was observed. Frequent clonic epilepsy attacks occurred daily on the left side of the body, including the face. A hematological examination revealed a mild upregulation of inflammation markers  was administered continuously. A week after the first operation, the patient's systemic status was stabilized, and the pus discharge from the cyst drainage stopped. Thus, the VP shunt tube was replaced, and the cyst drainage was removed while the patient was under general anesthesia. The postoperative course was uneventful, and a head CT scan that was performed a month after the surgery revealed improvement of the hydrocephalus  and abdominal distention. Head CT scans revealed worsening of the hydrocephalus , and an cephalus . A postocephalus .The frequency of complications after VP shunt surgery has been reported to be around 25%, and, among these complications, the incidence of IPP is low with rates that have been reported to range from 0.33% to 6.8% since it was first reported in 1954 [Our adult case exhibited onset seven years after the VP shunt surgery. This case is considered a rare case of IPP because IPPs that are associated with VP shunts usually occur 3 weeks to 5 years after VP shunt surgeries in younger patients , 8. ThusIPPs after VP shunts are usually diagnosed with abdominal echograms or abdominal CT scans , 8. HoweStaphylococcus aureus or Staphylococcus epidermidis [Streptococcus pneumoniae, and this is the first reported case of this type of infection, as far as we know. Thus, these findings suggested that not only dermal resident flora but also Streptococcus pneumoniae are important potential candidates as pathogens of IPP infection.The cyst components of IPPs that are associated with VP shunts are often infected, and the pathogens have usually been reported as dermal resident flora, such as dermidis , 10. UnlCyst drainage or cyst opening by laparoscopic or direct surgery is usually chosen as the therapeutic strategy for the treatment of IPPs after VP shunt surgery. In particular in cases with uninfected cysts, only the removal of the tube tip from the cyst has been considered an effective method in recent years , 6, 11.


The biceps brachii muscle is prone to variants but absence of the long head of the biceps (LHB) tendon is an exceptionally rare anomaly. This report concerns the fourth case of bilateral congenital absence of the LHB tendon and presents the ultrasonography (US) and magnetic resonance (MR) findings. Our case has the peculiarity of being the first in which bilateral LHB tendon agenesis is not associated with rotator cuff or labral tears. Absence of the long head of the biceps (LHB) tendon is a very unusual anomaly. We report the fourth case of bilateral congenital absence of the LHB tendon in the English literature. Ultrasonography (US) and magnetic resonance (MR) findings are exposed.A 29-year-old male, Muay Thai amateur fighter, presented with right anterior shoulder pain at rest that exacerbated with overhead activities. The pain was moderate for months but worsened in the last few weeks, specially after sports activity. He had no symptoms in the left shoulder.Although the patient is a sportsman, there was no history of a significant lesion/traumatic event involving the upper limbs, including shoulder dislocation. He also denied orthopedic surgery to both shoulders.The physical exam was almost unremarkable. The uppercut and Speed's tests were negative and the “Popeye” sign was absent. Digital pressure over the bicipital groove did not elicit pain. Although the rotatory stress test and the lateral decubitus load and shift test were negative, the anterior apprehension sign was doubtful. Also there was some limited range of motion when the patient tried to elevate the left arm with the forearm in supination. Overall, there was no clear evidence of biceps pathology or anterior instability.On the ultrasonography of the painful and contralateral shoulder we were not able to demonstrate the LHB tendon in its groove , suggestMagnetic resonance imaging of both shoulders revealed bilateral absence of the LHB tendon and shallow intertubercular sulci Figures  and 3. TThe biceps brachii muscle is one of the most diverse in the human body; however, congenital absence of the long head of the biceps tendon is a rare anomaly –7. FrequIn our case, the patient was healthy and physically active and no other congenital anomalies were evident. As in other reported cases, bilateral agenesis of the LHB tendon is most commonly diagnosed in a young adult patient complaining of nonspecific shoulder pain –3. EvideOur case is the first to describe bilateral absence of the LHB tendon not associated with rotator cuff or labral tears. No other significant abnormality was discernible in both shoulders. These findings support that agenesis of the LHB tendon can, by itself, be a source of symptoms and functional deficit. Based on other cases described, which emphasized the role of the LHB tendon as a shoulder stabilizer , 8, 9, w


Chromoblastomycosis is a chronic subcutaneous fungal infection caused bytraumatic implantation of dematiaceous fungi in the skin. The clinicalpresentation is usually a verrucous plaque lesion and the diagnosis isconfirmed by the visualization of muriform bodies at direct examination orat the histologic study. This report describes a rare case of tumoralchromoblastomycosis confirmed by histologic study and whose agent wasidentified by culture and micromorphology. Cladosporium type fructification,confirming the hypothesis of chromoblastomycosis by agent of the complexFonsecacea pedrosoi (Man, 77 years old, institutionalized for psychiatric history. He was taken todermatological evaluation due to a painless tumor in his left knee that waspresenting a progressive growth for a year, and the patient denied previous trauma.Physical examination revealed a tumor in his left knee, pinkish in color, measuring 3centimeters in major axis . In thepedrosoi  and 4.3 The negative mycologicaldirect examination can be interpreted consistently with the clinical form, sincethe epidermal lesion presented full, making it difficult to collect materialcontaining some fungal structure; at the same time, recent publications on thesubject highlight that in about 6% of cases the examination may benegative.2 Despitethe peculiarities of the case, the identified agent is from the complexFonsecacea pedrosoi, the main agent of chromoblastomycosisin our country.3The described clinical presentation is the rarest of the chromoblastomycosiscondition, however its location in the lower limbs and male involvement remainssimilar to other cases.


Intestinal metastasis of angiosarcoma is extremely rare. We herein report a case of intestinal perforation due to intestinal metastasis of angiosarcoma. The patient was a 72-year-old Japanese man with multiple recurrent angiosarcomas of the scalp. He developed acute abdominal pain with guarding, and we performed an emergency exploratory laparotomy. An intestinal perforation was found 80 cm from the ligament of Treitz, and partial jejunectomy was successfully performed. Macroscopic inspection revealed no obvious injury, ulcer, or tumor at or around the perforation site. Pathological examination revealed angiosarcoma cells penetrating through all layers of the jejunum at the site of intestinal perforation. This is the first reported case of intestinal perforation caused by indistinguishable intestinal metastasis of angiosarcoma. This case emphasizes intestinal metastasis of angiosarcoma as a possible cause of small bowel perforation in patients with advanced angiosarcoma, even when no visible tumor is present during surgery. Angiosarcoma is a very rare tumor that originates from endothelial cells and constitutes approximately 2 % of soft tissue sarcomas . AngiosaThe incidence of malignant tumors has been increasing worldwide, and the management of abdominal oncologic emergencies has thus become more clinically important. Common causes of abdominal oncologic emergencies are obstruction, hemorrhage, and perforation. Among these, intestinal perforation often requires urgent surgical intervention. Lung and gynecologic cancers occasionally cause intestinal perforation by metastatic tumors , 5.We herein report an extremely rare case of intestinal perforation due to intestinal metastasis of angiosarcoma, which was indistinguishable during the operation but pathologically confirmed after surgery. We also provide a brief review of the literature regarding intestinal metastasis of angiosarcoma.3/μL, and C-reactive protein level of 16.64 mg/dL. Contrast-enhanced computed tomography showed focal wall thickening of the small intestine surrounded by ascites and small collections of free air , but peritonitis occurred in only two cases 33 %) Table . Ruffolo3 % TableThe growth pattern of angiosarcoma is usually infiltrative, without the formation of a capsule or clear border distinguishing the tumor from normal tissue. At the primary site  in the present case, a red-brown color was the only clue of demarcation between the tumor and normal tissue Fig. a. Such aIntestinal perforation during chemotherapy can be explained by necrotizing enteritis in the presence of neutropenia, metastatic tumor infiltration, and direct intestinal damage by chemotherapeutic agents characterized by mitotic arrest –14. In tThe prognosis of angiosarcoma is very poor. Lahat et al. reported a median disease-free survival duration of 43 months range, 1–188 months), a 5-year disease-specific survival rate of 35 to 40 %, and a median survival duration of 10 months in patients with metastatic angiosarcoma –18. The 8 months,In summary, intestinal perforation due to intestinal metastasis of angiosarcoma should be taken into account, in abdominal emergency cases with advanced angiosarcoma.When obtaining an informed consent for surgical procedure, a general consent was also obtained from the patient, for publication and presentation, as usual.


Giant cell reparative granuloma (GCRG) is a rare, benign intra osseous lytic lesion occurring especially in gnathis bone but also seen in feet and hands. It has similar clinical and radiological presentations than giant cell tumor, chondroblastoma, aneurysmal bone cyst, and hyperparathyroidism brown tumors but with specific histological findings We report a case of a GCRG of hallux phalanx in 18 years old patient appearing many years after enchondroma curettage and grafting. Radiographs showed a multiloculated osteolytic lesions involving whole phalanx with cortical thinning and without fluid-fluid levels in CT view. Expected to be an enchondroma recurrence, second biopsy confirmed diagnosis of GCRG with specific histological findings. Although if aetiopathogeny remains unknown, GCRG is reported to be a local non neoplasic reaction to an intraosseous hemorrhage. Our exceptional case claims that this tumor can appear in reaction to cellular disturbance primary or secondary. Giant cell reparative granuloma (GCRG) is a rare, benign intraosseous lytic lesion occurring especially in gnathic bones and also in hands and feet phalanx –3. It isA 13- year-old boy presented in emergency room with left hallux painful evolving for several months without any underlying trauma. The physical examination noted a localized swallowing of the dorsum of the right hallux base with no inflammatory local signs and a pain sensitivity induced by examination. Mobility of the adjacent joint was normal and no biological abnormalities were found. Radiographic examination showed a well-defined lytic lesion of the hallux proximal phalanx. Neither cortex erosion nor soft tissues abnormalities were noticed . An initInitially described by Jaffe  in 1953,This case suggests a potential connection between GCRG and enchondroma and hypothesis that GCRG could appear in reaction of cellular disturbance primary or secondary to enchondroma surgical treatment.


Pheochromocytomas and paragangliomas are neuroendocrine tumors that arise from chromaffin cells of adrenal medulla and extra-adrenal paraganglia, respectively. The recurrence of these neuroendocrine tumors as a jejunal mass causing obstruction in the small intestine is an exceptional entity. The present study reports the case of a 70-year-old male who presented to the Emergency Department of Bezmialem Vakif University Hospital with abdominal pain and vomiting. The patient possessed a history of left nephrectomy due to malignant pheochromocytoma that had invaded into the left kidney eight months prior to presentation. Bowel obstruction was diagnosed and the patient underwent a laparoscopic procedure. Partial resection of the jejunum was performed and immunohistochemical studies revealed the lesion to be malignant paraganglioma. The majority of paragangliomas are chemo- and radioresistant. Surgical excision remains the primary treatment. Metachronous paraganglioma arising from the small intestine is an extremely rare entity and may be a relevant consideration in patients presenting with bowel obstruction. Pheochromocytomas and paragangliomas are neuroendocrine tumors that arise from the chromaffin cells of the adrenal medulla and extra-adrenal paraganglia, respectively. Pheochromocytomas are occasionally termed intra-adrenal paragangliomas . The annThe present study reports the case of a 70-year-old male that presented to the Emergency Department of Bezmialem Vakif University Hospital  complaining of abdominal pain, a lack of defecation for one week and vomiting of two-day duration. The medical history of the patient included left nephrectomy due to malignant pheochromocytoma that had invaded into the left kidney eight months prior to presentation. A coronary artery bypass graft operation two years previously and acetylsalicylic acid use were also reported in the medical history. The vital signs were stable, without hypertension and tachycardia. Physical examination revealed abdominal tenderness in the epigastric region, with mild abdominal distention. No abdominal guarding was present, but rebound tenderness occurred. The physical examination also identified hyperkinetic bowel sounds and empty rectal ampulla upon digital examination. Sternotomy and left subcostal incision scars were clearly visible on the patient. A contrast-enhanced abdominal computed tomography (CT) scan was performed. The CT scan revealed the jejunojejunal invagination of a contrast enhancing mass 20×9 mm in size located at the distal jejunum. The jejunum was dilated, resulting in a proximal diameter ≤3.5 cm. The CT findings featured a hypervascular target sign resembling a neuroendocrine tumor .Informed consent was obtained from the patient prior to undergoing surgery. In the present patient, a laparoscopic approach was preferred. The invaginated jejunal segment was identified at 120 cm distal to the ligament of Treitz . The segPathological examination of the mass revealed a malignant mesenchymal neuroendocrine tumor with vascular invasion. Mitosis was identified at a level of three mitoses per 10 high-power fields and the Ki-67 index was 40%. The surgical margins were tumor-free. Immunohistochemistry (IHC) revealed islets of ganglion-like cells that possessed large eosinophilic cytoplasms and were located in the submucosa under the epithelium. These cells were positive for the expression of neuron-specific enolase and did not express S-100 . The finPheochromocytomas are neuroendocrine tumors that arise from the chromaffin cells of the adrenal medulla. The neuroendocrine tumors that arise from the extra-adrenal paraganglia are termed paraganglioma. Pheochromocytomas are occasionally termed as intra-adrenal paraganglia . The incFor the surgical excision of paragangliomas, either an open or laparoscopic approach can be performed. In a case of suspected malignancy, the open approach is recommended by certain literature in order to protect the capsule of the tumor and avoid seeding, therefore preventing recurrence . However


Synovial sarcomas are a rare entity with predilection for extremities and joints. The literature suggests that these tumours are rare in the head and neck region. Very few authors have reported their origin in head. Among the ones occurring in the head region, most of them are found to originate from the parotid area. According to our extensive review of available literature, there has been no reported case of the primary case of synovial sarcoma originating from the scalp convexity. We hereby report one such case in a young female which, by far to the best of our knowledge, is the first ever reported case of a synovial sarcoma occurring on the scalp. The tumour is also the largest ever reported in the literature and posed a great surgical and diagnostic challenge to our team. Through this paper, we recommend that synovial sarcoma should be included as a very rare yet possible differential diagnosis for the scalp tumours. Soft tissue sarcomas are rare malignancies arising from embryonic mesenchymal cells, thus having varied origin and a wide range of subtypes. They constitute about 1% of all malignancies with an incidence of about 20 per million people and about 8% of cancers in children . We presA 25-year-old woman from a remote village of India presented to the surgery clinic of our hospital with complaint of recurrent swelling on her scalp for the last 6 years. Insidious in onset, painless, and gradually progressive in size, the swelling first occurred on her midscalp and gradually progressed in size over the next 1 year. It was first excised locally by a private practitioner at her hometown. She remained asymptomatic for about two years when she had a recurrence of swelling followed by a second surgical excision at a local hospital. No histopathological diagnoses were made on the excised specimens due to limitation of resources. The patient remained asymptomatic for about a year before she had the second recurrence at the same site but with a more rapid growth and presence of ulceration. She was referred to our tertiary level multispecialty centre for management.The patient presented to us with a huge swelling rapidly increasing in size. The swelling was large and boggy and was associated with heaviness of the head, pustular discharge, and on and off fever for the last 4 months.There were no neurological deficits or metastatic symptoms. The patient was married and nulliparous with no obstetric or gynaecologic complaints. There was no significant family history. The patient was depressed due to associated disfigurement and marital problems due to her disease. Viral markers and toxic profile were negative.Examination revealed a large 25 × 18 cm lobulated, bosselated swelling covering almost the whole scalp with focal ulceration and alopecia. There were multiple pus pockets with foul smelling blood stained pus discharge. Swelling was mildly tender .There was cervical lymphadenopathy with multiple mobile, nontender, firm lymph nodes in the left submandibular region and posterior triangle. The rest of the general physical and systemic examinations were within normal limits. She had a pulse rate of 89 per minute and blood pressure of 112/78 mmHg. She had a low grade fever (99.2°F). Staphylococcus aureus (MSSA). Her blood and urine cultures and further wound swab cultures came sterile. Her fever responded to antibiotics.Multiple cultures were obtained. Pus cultures revealed methicillin sensitiveMultiple trucut biopsies taken from the scalp swelling came inconclusive. Incisional biopsy was thus taken which showed fibrocollagenous tissue with chronic inflammatory infiltrate and extensive areas of necrosis. There was no evidence of malignancy in any of the histopathological reports. Excisional biopsy of posterior cervical lymph nodes suggested reactive changes.The patient underwent contrast-enhanced computed tomography (CECT) of head and neck with 3-dimensional reconstruction Figures . ReportsWith the provisional diagnosis of recurrent scalp tumour with probable malignant or inflammatory pathology, excision under general anaesthesia was planned. Challenges were to achieve negative surgical margins in a highly vascular and huge scalp tumour and to cover the large scalp defect thus created.With the patient in supine position, the tumour was well painted and segregated with sterile drapes. The head was placed flexed for adequate exposure of the tumour tissue. With high vascularity of the tumour, it was dissected off the cranium with haemostatic sutures applied all around on the tumour edge and with a 2 cm margin on the normal scalp tissue. The raw area thus created was covered with split thickness skin graft harvested from the left thigh. The procedure was uneventful with less than 300 mL of blood loss .The patient made uneventful recovery and was discharged on postoperative day seven with almost 90% graft uptake. Two-week follow-up revealed healthy graft tissue .Histopathology of the excised specimen revealed a solid cystic tumour with areas of necrosis. The size of the tumour in excised specimen was 24.5 cm × 12 cm × 10.5 cm . The tumShe received postoperative locoregional radiotherapy after complete take of skin graft and epithelisation of all raw areas. A repeat PET-CT at 24 months showed no focus of recurrence of disease. On a 29-month follow-up after surgery, the patient was asymptomatic with no recurrence . As the Sarcomas of head and neck origin account for <10% of all sarcomas and about 1% of all primary head and neck malignancies , 4, 5. SSynovial sarcoma is a relatively rare subtype of soft tissue sarcoma with histologic resemblance to synovial cells, thus the name. The morphologic subtypes are divided into monophasic and biphasic according to the presence or absence of differentiation into glandular epithelium and a third poorly differentiated subtype. The most characteristic and hallmark genetic alteration is the chromosomal translocation tX;18)p11.2;q11.2) . The SYT(p11.2;q1.2;q11.2 Synovial sarcoma rarely presents on trunk and regions of head and neck. The latter contributes 2.5–3.7% of all synovial sarcoma cases , 13. TheBased on extensive literature search, our case is the largest case of synovial sarcoma of head and neck ever excised , 13, 15.Surgery is the mainstay of treatment . HoweverThe role of adjuvant therapies is debatable. Chemotherapy takes care of the microscopic cancer cells locally and those disseminated systemically. Locoregional radiotherapy is important to reduce the chances of local recurrence and also takes care of the microscopic or sometimes macroscopic positive surgical margins.The outcome of a well differentiated tumour with adequate surgical excision and aggressive adjuvant therapies is fairly good with survival rates in the range of 60% , 16. RecAs per our extensive search of the literature and the unique presentation of our patient, we can affirmatively say that this is, in most likelihood, the first ever reported case of synovial sarcoma of scalp origin and indeed the largest primary synovial sarcoma of head and neck region ever excised. We emphasise the need of including synovial sarcomas as one of the rare differential diagnoses of head and neck sarcomas.


Adrenal ganglioneuroma is a rare tumor constituting 20–30% of all ganglioneuromas. It is a benign tumor and can present diagnostic problems when confused with other adrenal solid tumors.We herein report a case of adrenal ganglioneuroma in a 28-year-old Arabic patient and emphasize the diagnostic role of cross-sectional imaging modalities (computed tomography and magnetic resonance imaging).Imaging of adrenal ganglioneuromas is diagnostically challenging. Differentiation between adrenal ganglioneuroma and other solid adrenal tumors can be difficult. However, some suggestive features on computed tomography and magnetic resonance imaging are helpful in achieving a correct diagnosis. Adrenal ganglioneuroma (GN) is an uncommon benign neuroblastic tumor comprising Schwann cells and ganglion cells , 2. It iA 28-year-old Arabic man was referred to our unit after the discovery of a right adrenal incidentaloma during a workup for right subcostal pain. The patient had no other symptoms. Serum laboratory values included normal 24-hour urinary native catecholamine and metanephrine levels, normal adrenal corticotrophin and cortisol rhythms, normal 24-hour urinary free cortisol level, normal adrenal plasma aldosterone/plasma renin activity ratio, and normal androgen hormone levels. Computed tomography (CT) showed a well-circumscribed, homogeneous right adrenal tumor measuring 10 × 7 × 8 cm and exhibiting peripheral punctate calcifications. The density of this mass on unenhanced images was lower than that of muscle Figure , the tumGN is an uncommon benign and mature neuroblastic tumor . It is aHistopathologically, GN is entirely composed of ganglion cells and Schwannian stroma and does not contain neuroblasts, intermediate cells, or mitotic figures , 5. The Most GNs are discovered incidentally on imaging studies , 8. UltrT1-weighted MRI shows a homogeneous mass with a signal intensity lower than that of the liver, and T2-weighted MRI shows a heterogeneous mass with a predominant signal intensity higher than that of the liver. T2-weighted MRI also shows no absolute change in signal intensity on chemical shift imaging. The high, heterogeneous signal intensity on T2-weighted images is presumed to be caused by a combination of the myxoid matrix and relatively low numbers of ganglion cells , 11. DelPreoperative diagnosis is difficult because of the lack of specific imaging findings , 12. PerCertain biological and radiological features should lead the clinician to consider a diagnosis of GN: no elevated hormonal secretion; presence of punctuate calcifications, no vascular involvement, and nonenhanced attenuation of <40 Hounsfield units on CT; a homogeneous, hypointense mass on T1-weighted MRI; a heterogeneous, hyperintense adrenal mass on T2-weighted MRI; and poor, delayed enhancement on dynamic MRI.In conclusion, adrenal GN is a diagnostic challenge on imaging studies . This tuWritten 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.


Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary that commonly presents with enlargement of the pituitary gland. Clinically and radiologically, IGH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma.Through such a case, we aim to present this rarity and emphasize the importance to correctly diagnose confusing pituitary lesions comprehensively by clinical presentations, radiological signs, and biopsy.We present an uncommon case of IGH in a 19-year-old man. The patient was admitted to the hospital with severe headache, vomiting, and vision's sharp decline. Magnetic resonance imaging showed a sellar lesion with obvious cystic change and ring enhancement. The disease course including diagnosis and treatment was presented and analyzed in detail. The pertinent literature is reviewed regarding this uncommon entity.The patient underwent surgical exploration and partial resection via the transsphenoidal approach. The pathologic findings suggested IGH giving no significant evidences of systemic granulomatous disease and venereal disease. Large dose methylprednisolone was then used. The pituitary function recovered, but there was no apparent improvement of his vision.IGH is a rarely occurred inflammatory disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Although rare, IGH should be kept in mind in terms of differential diagnosis of sellar region lesions. In absence of any demonstrable causative agent, the process is termed idiopathic granulomatous hypophysitis (IGH). The most common presenting symptom was headache, followed by chronically progressive visual changes. Magnetic resonance imaging (MRI)-specific data for IGH was poorly reported, with pituitary enlargement the most common feature. Contrast enhancement and pituitary stalk thickening may also appear. Clinically and radiologically, IGH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma.Hypophysitis is relatively a rare disorder, with an estimated incidence of 1 case per 9 million people per year and imaging diagnosis is difficult.Herein, we present a case of a 19-year-old man suffering from IGH complaining of vision's sharp decline to blindness within 1 week. Uniquely, the MRI showed a sellar lesion with obvious cystic change and ring enhancement, very much mimicking a pituitary or peripituitary abscess. To the best of our knowledge, this is the first reported case of IGH presenting in this manner giving his unique clinical and image features. Because the rarity of IGH often leads to the misdiagnosis of either another inflammatory and infectious disease or a pituitary tumor, we also review the latest literature on the etiology, diagnosis, and treatment of IGH.2O. The cerebrospinal fluid (CSF) was clear. Total cell count in CSF was 17 × 106/L, and white blood cell was 16 × 106/L, with mononuclear lymphocytes and phagocytes the majority. CSF biochemical analysis yielded no abnormal finding. Endocrinological evaluation showed a normal thyroid and adrenocortical function and a mild hyperprolactinemia . Immunological and rheumatic indicators were all within the normal range.A 19-year-old Chinese man, without particular previous medical history, had complained of a bilateral temporal headache for more than half a year. The pain was diffuse, dull, and paroxysmal, and could be alleviated by rest. He also presented with transient fever (maximum 39.2°C) for several times. He did not seek medical services until nausea and vomiting occasionally occurred especially in the morning 1 month after the onset. He underwent a head computed tomography scan at the local hospital, showing a 15 mm × 10 mm cyst in the pineal region (just a chance finding). No apparent changes were found in other intracranial structures including the sella turcica area. He was treated pharmacologically with analgesics. However, his condition did not improve. After 2 weeks, because of frequent nausea and vomiting, he came to our hospital. The patient was down in spirits with heavy complexion. In addition to the above symptoms, he also complained a mildly decreased bilateral visual acuity (VA) in recent weeks. He denied polyuria, polydipsia, and sexual hypoactivity, and had no symptoms of unconsciousness, convulsion, epilepsy, and cognitive disorder, and no special circumstances regarding his family history or personal history related to his presentation were identified. Neurological examination showed a normal function of both sensation and motor at 4 limbs. No apparent changes in superficial and deep tendon reflexes were observed. Both superficial and deep tendon reflexes were normal. Pathological signs and ataxia were absent. Upon rough ophthalmologic examination, his left palpebral fissure was relatively small with ptosis, and pupillary light reflex was insensitive on the right side. The left VA was 0.8 and the right 0.6. There was suspicious defect in right upper quadrant of his right eye. However, his VA declined sharply. Just within 3 days while waiting for his head MRI scanning, the patient complained he even could not see anything with his right eye, and his vision on the left side blurred further. Upon specialized ophthalmologic examination, relative afferent pupillary defect on the right side was strong positive and fundoscopic examination was unremarkable. Temporal incomplete hemianopia and nasal scotomas of his left eye were observed  treatments have also been widely reported.21Transsphenoidal surgery is a primary diagnostic option, especially for those whose clinical and radiological presentation is not typical and diagnosis is not confirmed.22 All in all, VF recovery may be related to many factors including differences in the patient population studied, differences in preoperative deficits in VF and VA, the lesions’ pathological nature, the extent of lesion resection, the dose of postoperative radiotherapy, and so on.What is noteworthy is that in the present case, there was no apparent improvement of the patient's vision after recovery of normal pituitary function. We guess there are at least 3 possible reasons accounting for that. First, the resection surgery was not total but partial. Although impulsion therapy with large dose methylprednisolone was used after surgery, there might still be some left lesion tissues which could still exert mass compression effect and stimulatory effect on the optic chiasm. Second, the patient's preoperative VF and VA were too poor and deteriorated rapidly. According to a multivariate analysis for pituitary adenomas, the only independent predictor of postoperative recovery of VF was the degree of preoperative VF deficit. Postoperative VF recovery was also related to both the duration of symptoms and the severity of preoperative VA. Third, the pituitary lesion nature in this case is IGH rather than the commonest adenoma, whose pathogenic effect may be not limited to simple compression. So in this circumstance, the optic chiasm could encounter some nonreversible pathologic change such as optic atrophy.We report an uncommon case of suprasellar IGH with cystic and ring-enhanced MRI appearance resulting in a precipitously worsened vision loss. IGH is a rarely occurred inflammatory disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. With this article, we not only aim to present this rare entity, but also to emphasize the importance to correctly diagnose cystic ring-enhancing sellar lesions to ensure the patient receives proper treatment.


Currarino syndrome (Currarino triad) was described in 1981 as a triad syndrome with a common embryogenesis in infants and with three characteristics: anorectal stenosis, a defect in the sacral bone, and a presacral mass. We describe here an unusual case of Currarino syndrome in an adult presenting with a presacral abscess but no meningitis.A 32-year-old Japanese man presented with fever, arthralgia and buttock pain. A digital rectal examination showed mild rectal stenosis with local warmth and tenderness in the posterior wall of his rectum. Computed tomography showed a scimitar-shaped deformity of his sacrum and an 8cm presacral mass, which continued to a pedicle of his deformed sacrum. This was diagnosed as Currarino syndrome with a presacral abscess. The abscess was drained by a perianal approach with our patient treated with antibiotics. His symptoms soon disappeared. After three months, an excision was performed through a posterior sagittal approach. His postoperative course was uneventful and he was discharged 10 days after surgery. A histopathological examination revealed an infected epidermoid cyst. He has been free from recurrence as of four years and six months after surgery.We report a case of Currarino syndrome in an adult who presented with a presacral abscess but no meningitis. Abscess drainage followed by radical surgery resulted in a successful outcome. Thesemosome 7 . The premosome 7 . The preWe report an unusual case of CS in an adult presenting with a presacral abscess. There was an early suspicion of CS, and a multidisciplinary assessment resulted in successful treatment.A 32-year-old Japanese man was referred to our hospital complaining of fever, arthralgia and buttock pain. His birth history was unremarkable. He had a past history of bronchial asthma and gastric ulcer without constipation. On admission, his temperature was 40.0°C. An abdominal examination showed no tenderness, no distention and no palpable mass. A neurological examination showed no contributory factors. The anal location and tonus were normal. A digital rectal examination demonstrated mild rectal stenosis with local warmth and tenderness in the posterior wall of his rectum.Laboratory studies revealed a white blood cell count of 18,000 cells/μL and C-reactive protein of 26.11mg/dL. Results of a urine analysis were normal. Computed tomography (CT) showed a scimitar-shaped deformity of his sacrum. An 8cm presacral mass containing air displaced his rectum ventrally and appeared to continue to his spinal canal through the anterior wall of his sacrum  showed the presacral mass with the same imaging characteristics as an infected epidermoid cyst Figure . MyelogrHLXB9 mutation was not evaluated because he declined a gene test. On a follow-up appointment at four years and six months, our patient was doing well without any neurological deficit and with normal defecation.Histopathology confirmed our diagnosis of an infected epidermoid cyst. The cystic wall consisted of stratified squamous epithelium. Our patient’s postoperative course was uneventful, and he was discharged 10 days after surgery. The HLXB9, which encodes the nuclear protein HB9. However, a genotype-phenotype correlation has not been reported [et al. reported variable clinical and imaging features of CS in three siblings with a HLXB9 gene mutation [To date, approximately 300 cases of CS have been reported in the English literature ,7,8. Thereported . Kim et mutation . The conmutation . Other cmutation ,7. In pamutation . We founmutation -12. Two It is easy to detect a sacral defect or anomaly by pelvic X-ray. If CS is suspected, imaging of the full spine including the presacral mass is needed. CT and MRI are useful examinations for a presacral mass, as well as for spinal cord tethering. Although the reported incidence of tethered cord with CS is variable 14% to 57%) % to 57% ,13,14, iManagement of CS depends on the existence of a presacral mass, an anorectal malformation, and a fistula between the colon and spinal canal. Various therapeutic strategies have been reported -16. SurgThe phenotypic variability and complexity of CS requires a multidisciplinary treatment. Because the Currarino triad is often missed, its diagnosis tends to be delayed. CS has a risk of serious complications resulting in morbidity and mortality. A precise preoperative diagnosis should lead to appropriate surgery and help to optimize the long-term outcome of CS.We have reported a case of CS in an adult who presented with a presacral abscess but no meningitis. Abscess drainage followed by radical surgery resulted in a successful outcome. CS consists of various phenotypes and clinical presentations, and precise multidisciplinary assessment can lead to suitable and successful treatment.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.CS: Currarino syndrome; CT: computed tomography; MRI: magnetic resonance imaging.The authors declare that they have no competing interests.MS was a major contributor in writing the manuscript. NN performed the first surgery. NN and NK performed the second surgery. AY, WF, NK, HH and RI carried out the several examinations and contributed to patient management. NN followed the patient up for four years and six months and helped to draft the manuscript. All authors read and approved the final manuscript.


Oral lesions show a wide range of biologic behaviours. There are various lesions which may mimic others and present in such an unusual manner thus making them very difficult to diagnose clinico-pathologically. An accurate diagnosis is not only important for correct treatment planning but also for determination of prognosis. Thus, it is very important for a surgical pathologist to be aware of the various atypical presentations of the lesions. The present unusual case report of basal cell adenoma occurring on upper lip with frank areas of calcifications and abundant inspissated mucoid secretions is an example of one such case.BCA is an uncommon benign epithelial salivary gland neoplasm. It is one of the nine subcategories of salivary gland epithelial tumours according to WHO 2005 classification of salivary gland tumors. It is composed of basaloid cells organized with a prominent basal cell layer and distinct basement membrane-like structure and no myxochondroid stromal component as seen in pleomorphic adenomas.To our best knowledge, no case in English literature has been reported BCA with exuberant inspissated mucoid secretions and frank areas of calcifications to such a large extent and this is the first case to report the same. Key words: Basal cell adenoma, calcifications, diagnosis, inspissated mucoid secretions, surgical pathologist. Basal cell adenoma [BCA] is an uncommon benign epithelial salivary gland neoplasm 1-2%] of all salivary gland tumours -2% of al. BCAs haThe categorization of any neoplasm whether benign or malignant, is crucial in terms of treatment planning, evaluation, and determination of prognosis. This relies upon the histopathological diagnosis which is made after the macroscopic and microscopic examination of the biopsy obtained. It is the responsibility of the surgical pathologists to diagnose as accurately as possible using all the appropriate diagnostic tools available.Thus, intention of this case report is to report an atypical case of BCA on upper lip with frank areas of calcification and exuberant inspissated mucoid secretions which has never been reported by any author till now. At the first glance, these areas of calcifications and exuberant mucoid secretions were thought to be a tissue which has been burned due to technical errors. Later on, series of investigations and a careful examination led to the diagnosis of BCA. The aim of this paper is to make pathologists worldwide, aware of difficulties which may be encountered during the diagnosis of such an unusual case.A 42 year old male reported to outpatient department in Sudha Rustagi College of Dental Sciences and Research, Faridabad, India; with a chief complain of a painful swelling on inner side of upper lip since six months. The history revealed an insidious, slow growing painless swelling since 3-4 years which became painful since 6 months. Pain was continuous, diffuse, dull and non- radiating in nature. There was no history of any associated trauma/ discharge/ any other swelling/ any dental treatment. The past medical/ dental/ personal history was non contributory. On extraoral examination, no evidence of swelling/ asymmetry or any other abnormality was detected. Intraoral examination demonstrated a well circumscribed, symmetric, dome-shaped sessile swelling was present on inner surface of upper labial mucosa. It was approximately 1.0 cm in diameter crossing midline, pale pink in colour with few red areas and smooth in surface texture. There was no blood/ pus discharge and surrounding mucosa was normal. On palpation, swelling was multinodular, firm in consistency, tender, non-fluctuant, non-compressible, non-mobile and had no localised raised temperature.The swelling was provisionally diagnosed as pleomorphic adenoma and an excisional biopsy was performed under LA and was examined histopathologically.Macroscopically, the specimen was 1.0x 0.8x 0.7 cm in size, creamish red in color, firm in consistency and smooth in texture. Microscopically, H&E stained soft tissue sections showed well circumscribed and encapsulated tumour. In the centre of the tumour, exuberant, faintly basophilic mucoid secretions with numerous globular and highly basophilic areas of calcifications were seen. Mucoid secretions were surrounded by the isomorphic basaloid tumour cells, which were arranged in patterns of varying sizes and shapes such as glandular, ductal, nests and cords Figs. . BasaloiHistopathologically the case was diagnosed as basal cell adenoma with atypical findings of presence of inspissated mucoid secretions and calcification foci.The highlight of the present case report is the exuberant inspissated mucoid secretions and prominent calcification foci which were very misleading. For the first glance, the inspissated mucoid secretions resembled the burnt tissue which has been burnt during tissue processing due to negligence of laboratory technician. It was interpreted that probably during impregnation and embedding, the paraffin wax was kept at extremely high temperature, which could have caused the tissue to burn. The sections microscopically appeared to be degenerated with myxoid appearance and loss of cellular details as seen in a burnt tissue. Later, on interrogating the technician and enquiring about the processing technique used, it was found that no processing error was made. Further, it was revealed that the tissue posed a difficulty for the technician as well during microtomy as the tissue was firm. Since it was clear that tissue was not burnt, many serial sections were made and examined. Along with routine staining, PAS staining was also performed which confirmed the presence of inspissated mucoid secretions superimposed on the cellular structures.Conventionally, inspissation is the process of thickening due to dehydration . In laboet al. (Antoniades D. et al.  presenteet al.  and the Another obstacle encountered during the diagnosis of the present case was the presence of frank areas of calcifications. To our best knowledge, there are only three cases in English literature  which haIt may be hypothesized that inspissated mucoid secretions present in long standing mucocele, may have under-gone dystrophic calcifications leading to the frank areas of calcification. The mucoid secretions contains water [95%], most of which is bound in a viscoelastic gel containing mucins . The gelSince no such case with presence of such exuberant inspissated mucoid secretions and calcification foci in BCA have been reported in literature, our case having both is the first case reporting the same, and is thought to be extremely rare.


Vulvar neoplasms represent four percent of all gynecological cancers. While most cases of vulvar neoplasms are benign, two percent of patients present with malignant disease. We present the case of a 37-year-old premenopausal female who presented to an outside institution with a lump in her left vulva, which had progressively enlarged to the size of an egg. A wide local excision of the left vulva was performed, and the pathology revealed a high-grade sarcoma, not otherwise specified (NOS), with negative margins. Imaging showed enlarged bilateral external iliac lymph nodes, likely metastatic. After discussion at a multidisciplinary gynecology oncology tumor board, she was treated with gemcitabine/docetaxel chemotherapy, followed by a left inguinal lymph node dissection and a left radical vulvectomy after being referred to our centre. The final pathology at that time showed a residual sarcoma of 3.5 mm in the left vulva with no lympho-vascular invasion (LVI) and negative margins, with the closest, laterally, at 2 mm. A total of three lymph nodes were negative. She received additional chemotherapy postoperatively. Approximately one year later, she returned to her gynecologist with a 1 cm mass on the left vulva. She underwent a left hemi-vulvectomy and lymph node dissection, and pathology confirmed the presence of a high-grade sarcoma with close margins. She received adjuvant radiotherapy. Three months later, she presented with persistent cough and pneumonia. Imaging revealed a 10 cm lung mass, which was believed to be metastasis from the vulva. This was confirmed with biopsy and was completely resected.Any mass in the Bartholin gland area should be investigated carefully. Poorly differentiated vulvar leiomyosarcoma in the Bartholin gland can recur locally but may also lead to distant metastasis. Despite surgical and systemic treatment, as well as adjuvant radiation, the tumor recurred. Due to the rarity of this condition, there are no clear recommendations for treatment of this disease. To our knowledge, this is the first report of vulvar leiomyosarcoma of the Bartholin gland with metastasis to the lung. Sarcomas are uncommon malignant tumors of mesenchymal origin that comprise two percent of all cancers in humans . Ninety There are very few cases of vulvar leiomyosarcomas reported as small case series or as case reports. This case is unique in that there were no distant metastases in previously reported cases of leiomyosarcoma of the Bartholin gland . InformeA 37-year-old premenopausal female , ex-smoker whose past medical history was only significant for hyperlipidemia, presented to a community hospital in August of 2012 with a lump in her left vulva. What began as a pea-sized mass had rapidly enlarged to the size of an egg. She had a wide local excision of the left vulva with the pathology revealing a high-grade sarcoma, NOS. The tumor measured 5.0 x 3.9 x 2.9 cm, and it displayed ulceration of the surface epithelium, with no definite lymphovascular invasion (LVI), strong expression of estrogen and progesterone receptors, and focal positivity for smooth muscle markers compatible with smooth muscle differentiation  with negative margins. Endometrial biopsy and Pap test were also performed at that time and showed no evidence of malignancy. She was then referred to our institution where staging computed tomography (CT) scan of the chest, abdomen, and pelvis revealed enlarged bilateral external iliac lymph nodes that were likely metastatic. A positron emission tomography (PET)/CT scan performed at that time only showed some nonspecific activity in the vaginal/vulvar region likely related to her recent surgery. After further discussion at the tumor board, she received three cycles of gemcitabine/docetaxel chemotherapy prior to undergoing completion left radical vulvectomy and left inguinal lymph node dissection. The pathology from this second surgery showed residual sarcoma measuring 3.5 mm in the left vulva, no LVI and negative margins, with the closest laterally at 2 mm. Three lymph nodes were found to be negative. She received three more cycles of gemcitabine/docetaxel postoperatively.Fifteen months later, a 1 cm mass on her left vulva was discovered in the same location as the original tumor at a follow-up visit. A magnetic resonance imaging (MRI) of the pelvis showed an abnormal enhancement in the left vulvar area measuring 3.7 cm in greatest dimension  of external beam radiotherapy using intensity modulated radiation therapy. The patient required a four-week treatment break between the brachytherapy and external beam treatment due to development of an ulcer on the left lateral vaginal wall. The clinical target volume included the scar and entire tumor bed with a 1 cm margin and the remainder of the left hemivulva. The planning target volume (PTV) included the clinical target volume (CTV) with a 1 cm margin.On the first follow-up after treatment completion, the patient was very well, with no pain or local irritation. Upon examination, the 1 cm healing ulcer was still visible. There was no evidence of disease.Three months later, the patient was asymptomatic in the vulva, with the ulcer on the left vulva still present and stable in size. She did admit to having a cough and to have suffered from pneumonia several weeks prior, which was treated with antibiotics. She also experienced pain in the lower thoracic spine region when lying on her right side, and in the upper abdomen or lower chest when coughing. She was otherwise well, with no constitutional symptoms. Her appetite and energy were normal, and her weight was stable. The patient’s general physical examination was normal. On pelvic MRI, only postoperative changes were visible at the level of the vulva. However, on CT scan of the chest, a large, 10 cm paravertebral, subpleural mass , and the average age of clinical presentation is approximately 30–40 years old . The youLeiomyoma and leiomyosarcoma of the Bartholin gland are often clinically misdiagnosed as Bartholin gland cysts or abscesses . LeiomyoIn 1996, Nielsen et al. proposed criteria to differentiate between leiomyosarcoma and leiomyoma: 1) The mass should be more than 5 cm in diameter; 2) There should be the presence of an infiltrative margin; 3) There are more than five mitotic figures per 10 HPF; and 4) There is moderate to severe cytologic atypia. Tumors with three or more of these features should be considered sarcomas and those that have only one can be diagnosed as leiomyoma, while two of these characteristics can be considered as atypical leiomyoma . In the In 2003, Ulutin et al. reviewed 453 cases with vulvar malignancy from 1977-1997 [Aartsen and Albus-Lutter reviewed the history of 47 patients with vulvar sarcoma, which included 25 cases of leiomyosarcoma . SeventeIn 2009, Gonzalez-Bugatto et al. published a review of seven cases of vulvar leiomyosarcoma of the Bartholin gland [These tumors have a very low incidence, and the literature shows a wide variety of treatment plans limited to a small number of patients . Wide loIn this particular case, the patient was treated first with wide local excision. After the incidental finding of leiomyosarcoma, referral to our centre prompted staging studies which showed enlargement of the bilateral external iliac lymph nodes. She had received preoperative chemotherapy before undergoing left radical vulvectomy and left inguinal lymph node dissection, followed by additional chemotherapy. Fifteen months later, the tumor recurred locally and the patient was treated with left hemivulvectomy and lymph node dissection followed by interstitial brachytherapy and external beam radiotherapy. Three months later, the patient developed lung metastasis, treated by lobectomy and adjuvant chemotherapy. The patient remains disease free two months from completion of therapy.To our knowledge, this is the first case of vulvar leiomyosarcoma of the Bartholin gland with lung metastasis to be reported. A review of the literature shows few cases of leiomyosarcoma of the Bartholin gland, and none of these cases have distant metastases. Cases with multiple local recurrences and high-grade tumors generally have a poor prognosis . Early d


Torsion of the omentum is a rare cause of abdominal pain. It is clinically similar to common causes of acute surgical abdomen and is often diagnosed during surgery. Inguinal hernia is a common condition but not frequently related with torsion of the omentum.A 40-year-old Caucasian man came to our emergency department with abdominal pain of the left quadrant and abdominal distension for 2 days. His medical history included an untreated left inguinal hernia in the last year. Computed tomography revealed densification of mesocolon with left omentum “whirl” component and other signs of omental torsion. During an exploratory laparoscopy, a wide twist of his omentum with necrotic alterations that extended to the bilateral inguinal hernial content was observed. Omentectomy and surgical repair of bilateral inguinal hernia were performed.Torsion of the omentum is a rare entity and usually presents a diagnostic challenge. The use of abdominal computed tomography can help diagnosing torsion of the omentum preoperatively and, thus, prevents a surgical approach. Nonetheless, some cases of torsion of the omentum require surgical repair. Accordingly, a laparoscopic approach is minimally invasive and efficient in performing omentectomy. Torsion of the omentum is a clinical condition in which the organ rotates on its long axis compromising its vascularity. Segmental torsion of the greater omentum is more common; it was first described by Bush in 1896 ,3. The cTorsion of the omentum presents nonspecific clinical symptoms, namely moderate abdominal pain that may be similar to acute appendicitis. Thus, it is difficult to obtain a preoperative clinical diagnosis. According to the literature, ultrasound and computed tomography (CT) are useful tools for establishing a preoperative diagnosis ,8.The purpose of this publication is to present a case report of a patient with a rare condition of unusual clinical presentation. We report a case of torsion of the greater omentum diagnosed preoperatively associated with bilateral inguinal hernias. Moreover, we also show that minimally invasive surgery can help to confirm the diagnosis, while providing treatment and a better postoperative recovery.A 40-year-old Caucasian man was admitted to our emergency department with moderate pain of his left abdomen for 2 days. He also complained of abdominal distension but presented no other signs and symptoms like fever, nausea, vomiting, diarrhea or intestinal obstruction. His medical history included an untreated left inguinal hernia in the last year. A physical examination revealed pain in his left abdomen associated with a palpable mass on his left flank, without rebound tenderness or guarding.Two bilateral reducible inguinal hernias were found. Laboratory results revealed a C-reactive protein of 124mg/L. Given this unspecific clinical presentation, an abdominal and pelvic CT scan were performed, revealing an intra-abdominal abnormality, suggestive of pathologic infiltration of his omentum and the presence of the whirl sign suggesting torsion of his greater omentum  should render close vigilance of the patient as the situation can worsen. It is still not clear whether treatment of the precipitating factor should be the initial approach. Nonetheless, the use of a laparoscopic approach seems to be successful in such a benign situation.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.ES and PL evaluated the patient. AFC, AJR, AMR and PL were involved in drafting the manuscript and revising it critically for important intellectual content. DR was involved in CT scanning and imaging evaluation. PL, RP and ES performed the laparoscopic omentectomy and hernia repair. PL and AMR were involved in revising it critically and have given final approval of the version to be published. All authors read and approved the final manuscript