Chondrosarcoma (CHS) is a malignant cartilage-forming tumor and usually occurs within the medullary canal of long bones and pelvic bones. Based on the morphologic feature alone, a correct diangosis of CHS may be difficult, Therefore, correlation of radiological and clinicopathological features is mandatory in the diagnosis of CHS. The prognosis of CHS is closely related to histologic grading, however, histologic grading may be subjective with high inter-observer variability. In this paper, we present histologic grading system and clinicopathological and radiological findings of conventional CHS. Subtypes of CHSs, such as dedifferentiated, mesenchymal, and clear cell CHSs are also presented. In addition, we introduce updated cytogenetic and molecular genetic findings to expand our understanding of CHS biology. New markers of cell differentiation, proliferation, and cell signaling might offer important therapeutic and prognostic information in near future. Chondrosarcoma (CHS) is a rare malignant tumor that produces cartilage matrix. The estimated overall incidence of CHSs is 1 in 200,000 per year .CHSs that arise de novo are called primary CHSs, whereas CHSs developing superimposed on preexisting benign cartilage tumors such as an enchondroma or osteochondroma are referred to as secondary CHSs. CHSs are a heterogeneous group of tumors that can be categorized by anatomic location as central when they occur within the medullary canal or peripheral when they occur in the cartilage cap of an exostosis. In addition to conventional CHSs that show hyaline cartilage differentiation, there are other types of CHSs such as dedifferentiated, mesenchymal, or clear cell, subtypes which show distinct genetic and clinicopathologic characteristics  Tables  and 2. MMost (about 85%) of CHSs, however, are of conventional CHSs, and the majority arises in the medullary cavity of long bone. The minority (up to 15%) of conventional CHSs is secondary peripheral CHSs which develop from the surface of bone as a result of malignant transformation within the cartilage cap of a preexisting benign cartilage tumor such as osteochondroma or develop de novo on the bone surface, and these are not uncommonly referred as juxtacortical or periosteal CHSs. Conventional CHS of bone is the most common type of primary CHS. Primary CHS typically affects an old population. The majority of patients are older than 50 years. The peak incidence is in the fifth to seventh decades of life. There is a male predilection of 1.5–2 to 1 [CHS can involve any bone; the incidence of axial and appendicular involvement is very similar. The bones of the pelvis, especially for ilium, are frequently involved. Long tubular bones are frequently affected. The proximal femur is the most preferred site, followed in frequency by proximal humerus, distal femur, and ribs. Other less frequently involved bones are the spine, scapula, and sternum. CHS rarely involves craniofacial bones, neck, forearm, clavicle, and sesamoids (including the patella). CHS in the small tubular bones is extremely rare  , 5, 6.Clinical symptoms are mostly nonspecific. Localized pain is the most frequent presenting symptom (about 80%) after local swelling. The symptoms are usually insidious, progressive, and worse at night and have a long duration . Pathologic fractures are also common at initial presentation (up to 27%). Radiographs of conventional CHS typically reveal a mixed lytic and sclerotic pattern with characteristic small calcifications, often referred as “popcorn” or “ringlets” calcifications. In the long bones, primary CHS most commonly involves the metaphysis 49%), followed by the diaphysis 36%) . The pre6% . The %, follow Conventional intramedullary CHSs are large lesions, usually greater than 4 cm in size . CHSs grThe grading is primarily based on nuclear size, hyperchromasia, cellularity, and mitoses . The nucGrade 1 (low-grade) lesions are poorly cellular with hyperchromatic round nuclei the size of a mature lymphocyte. There are no mitotic figures, or nuclear atypia and the cells retain the lacunar pattern. Myxoid background is not present, but there may be some degenerative myxoid change. Binucleated cells are rare if any. Dense cellularity, presence of significant numbers of moderately sized or larger nuclei, and mitotic figures are not features of low-grade CHS and, if present, indicate a higher grade of CHS . Grade 2More than 90% of conventional CHSs are low- to intermediate-grade tumors and should be distinguished from enchondroma. Permeation of cortical bone and/or preexisting medullary bone is most important to distinguish CHSs from enchondromas , for whiSo far, several attempts have been made to identify reliable molecular markers and therapeutic targets for CHS . Collagep53 in CHS pathobiology remains obscure, the presence of overexpression of the p53 protein, 17p1 alterations, and TP53 mutations mainly in almost all high-grade CHSs suggest that the p53 mutation is a late event involved in CHS progression [ARF. The loss of INK4A/p16 expression was shown to be restricted to high-grade CHS, suggesting the role for CHS progression [There is increase of genetic aberrations as CHSs progress from low to high grade. Although the role of gression , 20. Ampgression . For the prognosis of CHSs, the single most important predictor of local recurrence and/or metastasis is histological grade, although several histological and clinical parameters such as tumor necrosis, mitotic rate, type of surgery, and tumor location have been suggested to be associated with prognosis. Grade 1 tumor has indolent clinical behavior and no metastatic potential. The five-year survival by grade was 89% for patients with grade 1 and 57% for the combined group of patients with grades 2 and 3 tumors. Only high tumor grades (2 and 3) were significantly associated with the probability of metastasis , 23.CHS is considered relatively resistant to chemotherapy or radiotherapy, and the mainstay of treatment is surgical treatment. Wide, en bloc excision is the preferred surgical treatment in grade 2 or grade 3 CHS. In grade 1 CHS confined to the bone, extensive intralesional curettage followed by local adjuvant treatment and filling the cavity with bone graft has promising long-term clinical results and satisfactory local control .The hands and feet are rare sites for central CHSs, whereas enchondromas are extraordinarily common in these sites. The median age of the patients at the time of diagnosis is 67 years , with a slight preference for female in contrast with CHSs located in other sites of the skeleton , 26.Occurrences in the hand are more common than in the foot, with the proximal phalanx affected most often. The fifth digit has the highest incidence of CHS, and the fourth digit is the least common site in the hands . Pain  is usually present at presentation. The median size is approximately 3 cm . Radiologically, CHSs are predominantly lucent lesions, sometimes with areas of punctuate calcification, with irregular cortical destruction and extension into surrounding soft tissue.The distinction between enchondroma and CHS is often difficult on histologic examination because enchondromas of the hands and feet can exhibit increased cellularity, binucleated cells, and hyperchromasia. Therefore, the most important histologic features of CHS in these sites are permeative growth pattern between preexisting bones and extension to soft tissue or joint space. Other histologic features suggesting malignancy are the presence of myxoid change and peripheral spindling of neoplastic chondrocytes.CHSs of the hands and feet are typically low-grade CHSs with a propensity to recur, but a limited tendency to metastasize in contrast to CHSs located elsewhere and complete excision with margins of normal tissue is curative in almost all cases , 26. CHS Craniofacial CHSs account for 2% of all CHSs and have a predilection for the skull base. The mean age of the patients at the time of diagnosis is 39 years  . The temporo-occipital junction is the most preferred site in frequency followed by clivus and sphenoethmoid complex .  Most patients present with symptoms related to the central nervous system. CT and MR imaging reveal bone destruction and associated soft tissue masses usually containing punctuate areas of chondroid mineralization .  The majority of the tumors are conventional CHSs of low to intermediate grade.When the skull base CHS involves the clivus, distinction from chordoma is important because CHS has a much better prognosis than chordoma. Chordoma tends to occur in patients a decade older than do CHSs and grow much more rapidly. Skull base CHS has an excellent prognosis, and the 5- and 10-year disease-specific survival rates are reported to be both 99%. In contrast, the 5- and 10-year survival rates of chordoma have been reported to be approximately 51% and 35%, respectively .Periosteal CHS is a rare malignant hyaline cartilage tumor arising from the external surface of bone and has also been referred to as parosteal CHS.  This tumor accounts for less than 2% of all CHSs and 0.2% of all bone tumors . The tum The clinical signs and symptoms are mostly nonspecific and present with pain or slowly growing mass. The lesion appears to involve the cortex with indistinct margins. On radiographs, the tumor often appears as a radiolucent juxtacortical soft tissue mass with sharply defined borders containing calcifications characteristic of cartilage tumors. The lesion is usually large  and covered by a fibrous pseudocapsule that is continuous with the underlying pseudocapsule. The mass is usually round to oval, lobulated, and gritty white with areas of enchondral ossification and scattered calcification. Whereas periosteal osteosarcoma is commonly fusiform and shows less-constant chondroid features . HistoloThe differential diagnosis includes periosteal osteosarcoma. The peak age of incidence of periosteal osteosarcoma is 10 years younger than CHS. The most common anatomic site is the diaphysis or diaphyseal-metaphyseal area of the proximal tibia, followed by the femur and humerus. Periosteal osteosarcoma presents as small radiolucent lesions on the surface, with formation of spicules of bone perpendicular to the bone shaft. Histologically, periosteal osteosarcoma is intermediate grade, predominantly chondroblastic osteogenic sarcoma , 32. Per The prognosis for patients with periosteal CHS is favorable compared to that of intramedullary CHS. The overall 5-year metastasis-free survival is approximately 83%. The 5-year metastasis-free survival is less for patients with grade 2 tumors (50%) than for patients with grade 1 tumors (94%). Invasion of the medullary cavity is not frequent. Metastasis is exceptional and occurs very late . DediffeSecondary CHS is a CHS arising in a benign precursor, either an osteochondroma or enchondroma. Although secondary CHS can be either central or peripheral, peripheral lesions are more common. Malignant transformation to peripheral CHS can be seen in 1% of solitary osteochondroma and 3~5% of patients with osteochondromatosis  , 37. HME HME is caused by mutations in either of two genes: exostosin-1 (EXT1), which is located on chromosome 8q24.11–q24.13, and exostosin-2 (EXT2), which is located on chromosome 11p11-12. Most of the mutations in these two genes are inactivating mutations , causing premature termination of the EXT proteins and the loss of protein function , 39. In −/− cells lose their ability to respond to polarity signals, then grow out of the bone, and then recruit normal cells to form an osteochondroma. IHH/PTHLH and FGF signaling molecules are mostly absent in osteochondromas and reexpressed with the progression of osteochondroma towards peripheral CHSs. Upregulation of PTHLH and Bcl-2 characterizes malignant transformation of osteochondroma [In the growth plate, IHH regulates chondrocyte proliferation and differentiation in a tightly regulated paracrine feedback loop, together with PTHLH, and deregulated IHH signaling has been implicated in the pathogenesis of osteochondromas. The EXT genes encode glycosyltransferases involved in the biosynthesis of heparan sulfate (HS) chains at HS proteoglycans (HSPGs). HSPGs have been shown to play a role in the diffusion of IHH, PTHLH, and fibroblast growth factor (FGF), all of which are involved in chondrocyte proliferation and differentiation. Therefore, EXT inactivation affects hedgehog signaling by defective HS Figures . In addihondroma , 44–46. In osteochondromas, lesions that continue to grow or cause pain after skeletal maturity suggest malignant transformation since osteochondromas only rarely grow after skeletal maturation. A thick hyaline cartilage cap greater than 1.5~2.0 cm thick (in osteochondroma: 6 to 8 mm thick) in a skeletally mature patient has been cited as a sign of possible malignant transformation . HoweverCHS arising in osteochondroma is usually solitary and low grade in type, but multifocality and dedifferentiation have also been reported . On micr Malignant transformation of osteochondroma is usually treated with surgery. Treatment of patients with HME is more complex than that of patients with solitary osteochondroma. Because most of these lesions are low-grade CHS, the overall prognosis is good, with long-term survival in 70~90% of patients. Local recurrence rate varies with adequacy of the tumor margins, from 0–15% in widely resected cases to 57~78% in cases with marginal or intralesional resection , 48 Central secondary CHSs develop as malignant transformation of enchondroma (extremely rare) or enchondromatosis such as Ollier disease or Maffucci syndrome. Patients with Ollier disease and Maffucci syndrome have a 25~30% risk of developing CHS. The exact cause of Ollier disease and Maffucci syndrome remains to be elucidated, although mutation in the PTHR1 gene, c.448C>T p.R150C), has been suggested to cause enchondromatosis , 50. A mC, has be Ollier disease is a nonhereditary developmental abnormality characterized by multiple enchondromas throughout the epiphyses, metaphyses, and diaphyses of the skeleton. The size, number, location, and evolution of enchondromas are quite variable. Clinically, Ollier disease often shows asymmetric, unilateral involvement of the lower extremities, but it is often bilateral in the hands and feet. Any portion of the skeleton formed by endochondral ossification can be affected; however, Ollier disease rarely affects bones formed by membranous ossification, such as the skull and facial bones. Maffucci syndrome is a condition in which enchondromatosis is associated with soft tissue hemangiomas , 52.The development of pain as well as the appearance of soft tissue mass, areas of bone destruction, endosteal scalloping, periosteal reaction, and fracture without significant trauma raises the suspicion of malignant transformation of enchondromatosis. CHS arising in enchondromatosis is usually a low-grade tumor like that of osteochondroma or HME. Therefore, identification of invasion to surrounding tissues or marked myxoid change is helpful to the diagnosis. CHS in enchondromatosis has the same prognosis as conventional CHS and depends on the site and grade of the tumor. Malignant transformation of enchondromatosis is greater in Maffucci syndrome than Ollier disease, and the prognosis is worse than that of Ollier disease.Dedifferentiated CHS is a distinct variant of CHS containing a well-differentiated cartilage tumor, either an enchondroma or a low-grade CHS, with an abrupt transition to foci having high-grade noncartilaginous sarcoma Figures .  Dedifferentiated CHS makes up 10% of CHSs. The average age of presentation is between 50 and 60 years. Men and women are affected equally. The most common affected sites are the femur and pelvis , 54. The The patients present most frequently with pain (90%), followed by pathologic fracture and soft tissue mass. The proportion of noncartilaginous component varies greatly and may be frequently osteosarcoma, fibrosarcoma, or malignant fibrous histiocytoma. Rhabdomyosarcoma, leiomyosarcoma, and angiosarcoma have been reported as the dedifferentiated component. Dedifferentiated CHSs have a wide range of radiological appearance; however, the presence of “tumoral dimorphism” with cartilaginous component and aggressive lytic component invading adjacent soft tissues suggests a diagnosis of dedifferentiated CHS . There are at least three hypotheses explaining the origin of dedifferentiated CHS. One theory is that the high-grade noncartilaginous tumor component arises in a long-standing low-grade cartilaginous tumor, particularly when the tumor is recurrent. The second hypothesis is that noncartilaginous component arises simultaneously with CHS with ability to differentiate. The third theory is that noncartilaginous sarcoma represents malignant transformation of adjacent inflamed but otherwise normal tissue . So far, no specific aberrations seem to be associated with dedifferentiated CHS, although dedifferentiated component tends to show aneuploidy, loss of heterozygosity, and amplification and deletion more frequently . Recentl Because the dedifferentiated component determines the prognosis, its identification is a key for management. In spite of aggressive treatment, the overall survival rate is less than 10% at five years, with a median survival time of 7.5 months. While local control is achieved in the majority of cases, distant disease remains the greatest clinical challenge, developing in 90% of patients .Mesenchymal CHS is a rare highly malignant tumor that arises in bone but can occur in extraskeletal sites and is characterized by highly cellular areas composed of undifferentiated small round or spindle cells admixed with lobules of mature hyaline cartilage. Mesenchymal CHS makes up less than 2~13% of all primary CHSs. Mesenchymal CHS occurs at any age, with peak incidences in the second to third decades of life. There is no significant sex predilection . The skeletal tumors show a widespread distribution. The craniofacial region is the most frequently affected site 15~30%), specifically the mandible and maxilla. Other common sites include femur, ribs, spine, pelvis, and humerus 5~30%, sp. Most patients presented with pain and/or swelling. Duration of symptoms prior to the histologic diagnosis is quite variable, ranging from few days to several years. Oncogenic osteomalacia secondary to mesenchymal CHS has been reported.Roentgenographically, mesenchymal CHSs in bone frequently resemble ordinary CHSs, showing osteolytic and destructive appearances with stippled calcifications. Tumors in extraskeletal sites are almost always identified as a mass with flocculent or stippled calcific densities. Sclerosis or periosteal reaction is uncommon, while expansion of the bone, cortical destruction, or cortical breakthrough with extraosseous extension of soft tissue is common . Grossly, the tumors are gray to tan, firm to soft, and usually well defined and well circumscribed. The size of the tumor ranges from 3 cm to 30 cm in diameter. Lobulation is infrequent. Most lesions contain hard mineralized deposits that vary in amount from scattered foci to prominent areas. Some tumors show a clearly cartilaginous appearance, even in a small area. Necrosis is uncommon but may be prominent . Bony ex Histologically, a bimorphic pattern with cellular zones of undifferentiated small or spindle cells and islands of hyaline cartilage is pathognomonic . The amo Immunohistochemically, the cartilaginous area is strongly positive for S-100 protein, whereas only scattered single cells in the undifferentiated areas stain for this antigen. The undifferentiated small cell component of mesenchymal CHSs is consistently positive for CD99 and may stain for vimentin and Leu7 while negative for osteocalcin, actin, cytokeratin, and epithelial membrane antigen (EMA) . SOX9 is At present, cytogenetic findings of mesenchymal CHS are rarely reported. An identical Robertsonian translocation involving chromosomes 13 and 21 ) has been detected in two cases of mesenchymal CHSs, possibly representing a characteristic rearrangement for this histopathologic entity . The t and has a predilection for the end of long bones (epiphysis) in contrast to conventional CHS which tends to occur in the meta-diaphyseal regions of the bone. Although the proximal femur and humeral head are the sites most commonly affected in about two-thirds of the cases by this lesion, most bones including spine, rib, pelvis, and hands and feet can be involved. The age range is wide with peak incidences in the third to fourth decades of life . Clinically, clear cell CHS presents one or two decades later than chondroblastoma. The clinical symptoms are nonspecific; however, pain is the most common presenting symptom. More than half of patients have pain for longer than a year. Roentgenographically, the lesion is typically located in the epiphysis metaphysis of long bone. The lesion is most often purely lytic and slightly expansile, with a sharp margin between the tumor and the adjacent normal bone . Typical Grossly, the tumors are well circumscribed and may be either firm or soft. Grossly cartilage is not usually present . The les Recent molecular genetic studies show that genetic alterations of p53 are infrequent in clear cell CHS in spite of substantial overexpression of p53 . Tumor-s Clear cell CHS is a low-grade malignancy and usually curable by en bloc resection. About 25% of patients experience local recurrences or metastases. However, tumor-related death is uncommon, particularly when the lesion has been completely resected en bloc .


Dear Editor,In the male fetus, Mullerian ducts degenerate leaving behind only remnants in connection with prostatic utricle and rete testis. Cysts of Mullerian origin are rare congenital lesions that are typically present as midline pelvic masses.2] Howev Howev2] A 30-year-old Afghani construction worker man was admitted in urology ward of Imam-Reza Hospital, Mashhad, Iran in November 2008, with a fourmonth history of refractory constipation. No genitourinary problem was reported. The only positive finding in physical examination was a fluctuating perineal mass. Except for normocytic anemia and hematuria, all other tests were unremarkable.Contrast-enhanced computed tomography (CT) scan demonstrated a midline multilocular cyst measuring 13 cm in greatest diameter located between urinary bladder and rectum with extension to prostate gland, perineum, cavernous bodies and scrotum. No obvious communication with prostate was seen. There was a diminished perirectal fat contrast indicating rectal adhesions. The aspirated fluid was spermfree, containing some scattered macrophages and epithelial cells. The culture of the fluid was negative. The patient underwent an operation that resulted in incomplete resection and partial colectomy due to extensive and severe adhesions to adjacent viscera and perineal skin. Microscopically, the cyst wall was lined with one layer of mucinous columnar epithelium. There was a proliferation of mucinous glands with complex branched ducts, similar to prebulbar (Cowper) glands, draining into the cyst . These gThe diagnosis of Mullerian duct cysts was classically made by considering the clinical, imaging and surgical investigations. However, recent studies highlights the importance of proper histological investigations especially the immunostaining for ER and PR.9]10][10]9][10[10]9][10


A 44-year-old man presented with the rare complication of remote cerebellar hemorrhage (RCH) after removal of a supratentorial glioma without the loss of a large volume of cerebrospinal fluid (CSF). He presented with severe headache, nausea, and vomiting for a few days, then he developed neurological deterioration including progressive disturbance of consciousness and left hemiparesis. Magnetic resonance imaging revealed a large tumor with intratumoral hemorrhage in the right frontal lobe that led to subfalcial and transtentorial herniation. The tumor was removed en bloc without excessive loss of CSF throughout the perioperative period. Although the level of consciousness remained unchanged from the preoperative level and no new neurological deficit was detected, routine postoperative computed tomography showed a bilateral RCH. Careful conservative therapy was provided and follow-up computed tomography demonstrated no further progression of hemorrhage. Compensatory acute engorgement of venous sinuses derived from the rapid decrease in intracranial pressure that occurred due to removal of the huge tumor might have caused cerebellar hemorrhagic venous infarction. Most postoperative intracranial hemorrhages develop around the operative site. Hemorrhage at a remote site following intracranial operation is a very rare complication . HoweverA 44-year-old man without notable previous medical history presented with severe headache, nausea, and vomiting that had persisted for a few days. He was admitted to a local hospital, and computed tomography (CT) and magnetic resonance imaging revealed a large tumor with intratumoral hemorrhage in the right frontal lobe. For the management of intracranial hypertension, osmotic diuretics were administrated, leading to dehydration. The third day after admission, he was transferred to our hospital due to neurological deterioration including progressive disturbance of consciousness and left hemiparesis.Magnetic resonance imaging performed at our hospital showed a rapid increase in mass effect, resulting in subfalcial and transtentorial herniation . The comPostoperatively, the level of consciousness remained unchanged from the preoperative level and no new neurological deficits were detected. A routine postoperative CT scan on the day after surgery revealed a bilateral cerebellar hemorrhage along with the cerebellar sulci facing the tentorium . MagnetiRemote cerebellar hemorrhage is an infrequent complication after supratentorial surgery, with an incidence rate of 0.08–0.6% , 6. It hRemote cerebellar hemorrhage commonly presents as decreased level of consciousness, cerebellar dysfunction, and delayed awakening . HoweverA total of 47 cases of RCH after removal of a supratentorial brain tumor have been described in the English literature, and we report the 48th case of RCH that occurred after removal of a supratentorial glioma. We have summarized the type of tumor, treatment, and the outcome of all 48 reported cases in The cause and the risk factors of RCH have been discussed in previous studies. Several authors have pointed out preexisting coagulopathy, arterial hypertension, administration of mannitol, and direct venous obstruction from intraoperative head rotation as causative factors for RCH. Nowadays, most authors agree that loss of a large volume of CSF is strongly related to the occurrence of RCH , 25, 27.There have been few reports that have discussed the pathomechanism of RCH following tumor removal without substantial CSF loss. Friedman et al. asserted that a compensatory reduction in CSF volume occurs to ameliorate the increased intracranial pressure that is caused by the presence of a large supratentorial mass. Therefore, in the hours immediately after the removal of the mass, there is a relative shortage of CSF . HoweverIn conclusion, we emphasize the importance of recognizing the possibility that RCH can occur after removal of a supratentorial tumor without loss of a large volume of CSF, especially in cases with a huge tumor and dehydration due to osmotic diuretics. Even if there is no loss of CSF in the perioperative period, close postoperative observation is still essential after removal of a supratentorial huge tumor.


This report describes a recurrent orbital glomus tumor in an Asian patient.A healthy 50-year-old Korean man had progressive right exophthalmos and a soft mass on his right lower lid for 6 months. We evaluated the mass using CT and MRI, and performed excisional biopsy and pathologic examination. Pathologically, the mass was a glomus tumor. Although proptosis of the right eye decreased, one month after surgery it increased to almost the same level as before surgery.This is the first report of an Asian patient with an orbital glomus tumor that demonstrated rapid re-growth after incision without pain or visual problems. Glomus tumors are rare, benign neoplasms of the glomus body, a specialized thermoregulatory arteriovenous structure. These tumors most commonly present in the dermis of the digits and palms,  and rareWritten informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. A healthy 50-year-old Korean man presented with progressive right exophthalmos and a soft mass on his right lower lid that had persisted for 6 months Figure . He had To confirm the diagnosis, an incisional biopsy was performed under local anesthesia. A subciliary incision exposed the mass to reveal a smooth, round, highly vascularized tumor. After controlling the extensive bleeding that was encountered, two fragments of the tumor were obtained. The fragments were irregular and multi-lobulated reddish masses measuring 0.9x0.7x0.6cm and 0.6x0.5x0.2cm. Pathologically, tumor cells were arranged in organoid and sheet-like patterns with intimately associated, capillary-sized blood vessels. The individual tumor cells were round, oval, polyhedral, or fusiform. Nuclei were round or ovoid and centrally placed in pale or slightly eosinophilic cytoplasm. The cell borders were not clearly delineated. Tumor cells were intermingled with thin fascicles of bland spindle cells Figure  and showTo our knowledge, only 4 other cases of orbital glomus tumors have been reported, and none of them were in Asian patients -5. We reThe authors declare that they have no competing interests.TSL, MC, YL and SB carried out the case report’s study, participated in the sequence alignment and drafted the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/12/62/prepub


This case highlights one of the infrequent complications of a commonly abused substance. A particular high index of suspicion of ischemic bowel is associated with cocaine abuse and should be included in the differential diagnosis of any young adult or middle-aged patient with abdominal pain and/or bloody diarrhea, particularly in the absence of other predisposing factors. To our knowledge, we report a rare case of ischemic small bowel associated with gangrene and pneumatosis intestinalis due to cocaine abuse. This case highlights one of the infrequent complications of a commonly abused substance. A particular high index of suspicion of ischemic bowel is associated with cocaine abuse and should be included in the differential diagnosis of any young adult or middle-aged patient with abdominal pain and/or bloody diarrhea, particularly in the absence of other predisposing factors. Up to our knowledge, we report a rare case of ischemic small bowel associated with gangrene and pneumatosis intestinalis due to cocaine abuse.A 36-year-old Caucasian man presented to the emergency room with one-day history of acute onset right lower quadrant abdominal pain with nausea and vomiting. He denied any diarrhea, constipation, rectal bleed, fever, chills, food intolerance, or change in appetite. His medical and surgical history was unremarkable except for an appendectomy 10 years ago; he had never had a colonoscopy. His social history was significant for smoking (20-pack years) and occasional alcohol use. He denied the use of illicit drugs. On presentation, the patient was afebrile; other vital signs were within normal limits. He appeared well developed, well nourished, and in no acute distress. His abdomen was soft, with increased tenderness to palpation in the right lower quadrant with no rebound tenderness or palpable masses. The remainder of the physical examination, including a rectal examination, was unremarkable, and a test for fecal occult blood was negative. Initial blood workup revealed a slightly elevated white blood cell count of 12,500 cells/mL  with a normal differential. Serum calcium, kidney and liver function, and urinalysis results were normal. Computed tomography (CT scan) of abdomen and pelvis revealed only a 2 mm nonobstructing right lower renal stone with no hydronephrosis. Over the next 24 hours, however, the patient's abdominal pain worsened. He was in obvious distress. Vital signs included tachypnea in the 30 s and a sinus tachycardia to the 130 s. The remainder of the physical exam revealed a diffusely tender, tympanitic, and distended abdomen with guarding and rebound tenderness. Laboratory analyses revealed an elevated white blood cell count of 16,900 cells/mL and an elevated lactic acid of 5.5 mmol/L (0.5–2.2 mmol/L). Repeated CT scan of the abdomen and pelvis showed multiple loops of mid and distal small bowel were dilated with pneumatosis intestinalis . FollowiIntestinal Ischemia is more common in elderly patients but also is seen in younger patients . The cauAlthough the complications of cocaine abuse are generally more likely to be cardiovascular or respiratory in nature than gastrointestinal , cocaineIschemic injury occurs most often in the areas of the intestine that are more vulnerable to a low-flow state, such as the watershed areas (the splenic flexure and rectosigmoid junction), which have limited collateral networks. Although any area of the intestine can be affected, approximately 75% of cases involve the left colon and approximately 25% of these involve the splenic flexure. In recent series, the right colon was found to be involved in 12% to 47% of cases [In a literature search, we found approximately 30 reported cases of cocaine-induced bowel ischemia. Most patients were in their third or fourth decade of life , and theIn the present case of ischemic bowel in a 36-year-old man, the cause was the recreational cocaine use. Our patient had no history of other known predisposing factors for bowel ischemia, although his cigarette smoking might have been a contributory factor. Cigarette smoking is common among those who use cocaine, and the vasoconstrictive effects of cocaine and nicotine, which are probably additive , also miAlthough computed tomography findings can suggest ischemic bowel or demonstrate its extent, colonoscopy, which establishes a definitive diagnosis, is the diagnostic procedure of choice when the large bowel is involved . In caseThe clinical course of ischemic bowel varies. In the majority of patients with ischemic colitis 85%), the ischemia involves the mucosa and submucosa, and the prognosis is good; the ischemia is transient and self-limited and heals uneventfully with conservative management. A minority with the chronic subtype may have sequelae of persistent segmental colitis or colonic strictures, some of which might require surgery. In the most unfortunate patients, delayed diagnosis leads to acute fulminant ischemia with transmural infarction that may progress to necrosis and death; these patients deteriorate with conservative management, and the majority require surgery . Wattoo %, the isCocaine-induced bowel ischemia is a rare reported presentation of cocaine abuse but should be included in the differential diagnosis of young or middle-aged risky patients with abdominal pain and/or bloody diarrhea. Clinicians should recognize the importance of drugs screening in young adults with visceral ischemia.


A case of gallstone ileus of the colon with illustrative pictures is presented, making the physicians more aware of this rare entity. Furthermore, the use of imaging modalities for diagnosis and decision making in management strategy is discussed. In 1941 L.G. Rigler described his famous triad of roentgen manifestations indicating gallstone ileus (GSI), that is, the association of an ectopic gallstone, bowel distension, and pneumobilia . HoweverGSI is a rare disease only accounting for 1–4% of all cases of mechanical intestinal obstruction . Its incA case of GSI of the colon with illustrative pictures is presented, making the physician more aware of this rare entity. Furthermore, the use of imaging modalities for diagnosis and decision making in management strategy is discussed.μmol/L; gamma-glutamyl transpeptidase (ϒGT): 67 U/L; alkaline phosphatase (AP): 97 U/L.A 78-year-old woman with a medical history of appendectomy, transient ischemic attack, cognitive dysfunction, type II diabetes mellitus, urinary and faecal incontinence, obesity, and hypercholesterolemia was admitted to the emergency department of our hospital because of abdominal pain, nausea, and vomiting. She had been constipated for three days. Physical examination revealed a mechanical ileus and a palpable mass in the left hemiabdomen. Laboratory studies were as follows: white blood count (WBC): 20.5 10∗9/L; C-reactive protein (CRP): 176 mg/L; bilirubin: 21.6 A plain abdominal radiograph  showed aA conservative management strategy including nasogastric tube, intravenous antibiotics, and colon lavage was chosen because of the extensive comorbidities of the patient and because the gallstone was already in the descending colon. Spontaneous passage of gallstones was awaited as this has been reported in the literature , 13, 14.μmol/L; ϒGT: 86 U/L; AP: 190 U/L. A new abdominal CT scan showed that the gallstone was still in situ but had passed onto the sigmoid colon and that the gallbladder was now containing faecal material (However, seven days after discharge the patient was readmitted experiencing the same complaints as during the first admission. Laboratory studies had worsened: WBC: 27.2 10∗9/L; CRP: 338 mg/L; bilirubin: 17.3 material . A colonmaterial . AttemptIn the subsequent hours the clinical situation of the patient deteriorated. An exploratory laparotomy showed a diffuse purulent peritonitis, dilatation of bowel, and a concrement in an ischemic sigmoid colon. It was decided to perform a cholecystectomy, a cholecystocolonic fistulectomy, and a sigmoidostomy to extract the stone Figures  and 6 anGSI is a rare disease not usually considered by the physician. However, delayed or missed diagnosis may have severe consequences.Plain abdominal radiographs have for long been the fundamental method to recognize the pathology. The main signs to acknowledge are Rigler's triad and Balthazar's sign, that is, air in the gallbladder , 15. HowBesides diagnosis and decision making in management strategy, CT scan has a role in the followup of conservative treatment of GSI of the colon. With regard to the case presented, it was assumed that the gallstone had passed causing essential delay in surgery. Therefore we suggest that if a conservative management strategy is chosen, passing of the gallstone should be proven by CT scan.Surgical relief of intestinal obstruction remains the mainstay of treatment. Recently, laparoscopy-guided enterolithotomy has become the preferred surgical approach in treating GSI . Debate In conclusion, a case of GSI of the colon with illustrative pictures is presented, hopefully making the physicians more aware of this rare entity. Our case illustrates that abdominal CT is the most appropriate noninvasive technique for diagnosis, treatment planning, and evaluation of success of conservative treatment.


We report an interesting case of asymptomatic retinal involvement in an encephalopathic patient enabling early identification of Susac’s syndrome.A 39-year-old Caucasian lady with hearing loss and encephalopathy was referred for ophthalmic assessment, including screening for branch retinal artery occlusions characteristic of Susac’s syndrome. Clinical features included severe headaches, right-sided hypoacusis, dysphasia and poor memory. Routine blood tests were normal. MRI brain showed numerous hyperintense lesions mainly in corpus callosum. Although she was visually asymptomatic, dilated funduscopy detected bilateral multiple peripheral branch retinal artery occlusions which were confirmed on fluorescein angiography. She was subsequently started on intravenous steroids and pulsed cyclophosphamide which improved her symptoms within 48 hours. Full recovery was made with no new arterial occlusions on four months follow-up.The case further establishes the crucial role of a detailed ophthalmic examination supported by fluorescein angiography in the assessment of these patients, who are at risk of being misdiagnosed and undertreated. Sucas’s syndrome (SS) is a retinocochleocerebral vasculopathy. It is characterized by the typical triad of encephalopathy, retinopathy and sensorineural hearing loss, which was first described by J.O Susac in 1979 and since when around 300 cases have been reported [et al. noted that capillaries with thickened walls and narrow lumens around the optic nerve head were surrounded by corpora amylacea formations which they suggested were likely to be the result of microinfarcts arising from a capillary angiopathy [SS manifests more often in women between their third and fourth decade . The patgiopathy .It has been suggested that retinopathy with branch retinal artery occlusions (BRAO) and hearing loss are not always essential in order to have the diagnosis of SS as long as encephalopathy and pathognomonic radiological findings are present . MultiplCareful interpretation of radiographic findings may establish the correct diagnosis. In SS the corpus callosum is affected with multifocal lesions of size 3–7 mm, centrally located and with a ‘punched-out’ appearance. Leptomeningeal enhancement may also be positive but is not always present. Lesions in patients with MS and ADEM typically involve the undersurface of the corpus callosum and leptomemingeal involvement is not typical .et al. recommend hyperbaric oxygen to attempt to reverse visual loss, although this is not generally practiced [Due to its underlying immunopathogenesis, SS is being widely treated with high dose intravenous corticosteroids plus a second immunosuppressant. Although the rarity of the disease means that there are no randomized controlled treatment trials, the literature provides reports of cases and case series treated with agents such as intravenous immunoglobulin, mycophenolate mofetil, cyclophosphamide, plasma exchange, azathioprine, rituximab and methotrexate have been used ,9,10. Furacticed . It woulWe present here a case of a 39-year-old female who presented acutely to the neurology team with rapidly progressive encephalopathy and hearing loss. The possibility of SS was raised following investigation with brain imaging which showed suggestive corpus callosal lesions. A referral to the Ophthalmology department was made and the diagnosis was confirmed by the presence of multiple branch retinal artery occlusions.A 39-year-old Caucasian lady presented with five weeks of severe vertigo, headaches and rapidly progressive confusion, following a trip to Tunisia. She reported no visual symptoms. She had a past medical history of bipolar disorder diagnosed two years previously. Neurological examination revealed right-sided hypoacusis, dysphasia, ataxia and poor memory. Haematological and biochemical profile was normal. Serological screening for infectious agents was negative. Lumbar puncture revealed marginally elevated opening pressure, raised protein with no cells and no oligoclonal bands. A T1-weighted brain MRI revealed inflammatory changes, with multiple ‘punched-out’ lesions in the corpus callosum  sequences, most (92%) white matter lesions in MS were centred on a small vein and were often (41%) characterized by a hypointense rim, whereas white matter lesions in SS were less commonly (54%) surrounding an identifiable vessel and rarely (4%) exhibited a hypointense rim. The authors also noted callosal atrophy and CSF-isointense lesions within the central part of the corpus callosum [SS-associated retinal artery occlusions are of importance both because they may cause severe visual loss (depending on the arteriole involved) but also because they may hold the key to rapid diagnosis and early initiation of immunosuppression. In SS, retinal artery occlusions have a characteristic pattern on fluorescein angiography with arterial wall hyperfluorescence due to atheromatous plaques, also called “Gass plaques” and are usually distally located from the bifurcation . Indocyapatients . Seven-Tcallosum .Through this case we want to make physicians more aware of the possibility of visually asymptomatic patients with retinal involvement in SS and suggest detailed fundoscopic and angiographic assessment by a neuro-ophthalmologist or retinal specialist in cases where neurological and radiological investigations raise the possibility of SS.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 of this journal.SS: Sucas’s syndrome; MS: Multiple sclerosis; ADEM: Acute disseminated encephalomyelitis; BRAO: Branch retinal artery occlusion; OCT: Optical coherence tomography; FLASH: Fast low angle shot.The authors declare that they have no competing interests.SB carried out information gathering, literature search, drafting of case report. SJ involved in diagnosis of condition, approved the final manuscript. ML case information gathering, literature search. AD performed tests on patient, carried out literature review, helped to draft the case, and approved the final manuscript. MAB confirmed diagnosis, proposed publication of case, and approved the final manuscript. AKD and MAB share the role of senior author. All authors have read and approved the final manuscript.Alastair K Denniston and Michael A Burdon share the role of senior author.


De Garengeot first described the presence of the appendix within a femoral hernia in 1731.We report the case of a 66-year-old Caucasian woman who presented with acute appendicitis within an incarcerated femoral hernia. This is the first reported case of de Garengeot's hernia in the Balkan area.Appropriate management without incurring any delay for radiological imaging can be promising for an uneventful postoperative course. The treatment of choice of this disease entity is emergency surgery and consists in simultaneous appendectomy through the hernia incision and primary hernia repair. In patients with large hernia defects or in older people the use of mesh for repairing the hernia defect can be an excellent choice. From 1731, when Rene Jacques Croissant de Garengeot first described the presence of the appendix within a femoral hernia , and appA previously healthy 66-year-old Caucasian woman presented with a 24-hour history of sudden onset painful right-sided groin swelling. On clinical examination, there was a fixed, round, tender mass about 5 × 3 cm in size in the right groin, above the inguinal crease. Her temperature was 38.7°C and she did not appear to be in distress. She did not have any bowel obstruction revealed by clinical examination or on the abdominal X-ray. Her past medical history was insignificant. Her laboratory findings were within normal limits except an increased WBC count (13.00 K/μL) with 80% neutrophils.A presumptive diagnosis of a chronically incarcerated femoral or inguinal hernia versus a strangulated hernia or an inguinal abscess was made with plans for a right groin exploration using a more curved low inguinal incision under general anesthesia Figure . When thAlthough femoral hernias account for 4% of all groin hernias, a hernia sac can contain any of the intraabdominal contents such as omentum. A pelvic appendix has the highest risk of entering a femoral hernial sac .De Garengeot's hernia is often misdiagnosed as an incarcerated or strangulated femoral hernia. The incidence of an appendix in a femoral hernia is reported to be 0.5-5% ,6-8; theAbdominal X-ray does not aid in the diagnosis of de Garengeot's hernia. Computed tomography (CT) and ultrasound have been successfully used for preoperative evaluation . The preThe treatment of choice of this disease entity is emergency surgery  and consChoice of repair in a femoral hernia containing a pathological appendix is debatable. Generally prosthetic material is not preferred in a contaminated field due to the risk of infection , but a fEven though there is at least one report of infection with the use of mesh, even in the absence of acute appendicitis , this reIn this case, the operation was performed immediately and no abscess was found in the hernial sac. There was no evidence of perforation and the patient was more than 60-years-old.The most common complication of the de Garengeot's hernia repair is wound infection with a rate reaching 29%. Some cases of necrotizing fasciitis and even death have been reported , probablAlthough the incidence of de Garengeot's hernia is extremely low, the surgeon has always to keep it in mind in cases with femoral hernias and regional symptoms of inflammation due to the lack of abdominal signs of appendicitis. Appropriate management without incurring any delay for radiological imaging can be promising of an uneventful postoperative course. In patients with large hernia defects or in older patients the use of mesh for repairing the hernia defect can be an excellent choice.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.All authors read and approved the final manuscript. PK was a major contributor in writing the manuscript. ES was involved in acquisition of data and review of the literature. AS was involved in acquisition of data and review of the literature. KK was involved in drafting the manuscript and revising it critically for important intellectual content. GK was involved in drafting the manuscript, revising it critically for important intellectual content and gave final approval of the version to be published.


Skull base inverted papilloma (IP) is an unusual entity for many neurosurgeons. IP is renowned for its high rate of recurrence, its ability to cause local destruction, and its association with malignancy. This paper is a comprehensive review of the reports, studies, and reviews published in the current biomedical literature from 1947 to September 2010 and synthesize this information to focus on its potential invasion to the base of the skull and possible intradural extension. The objective is to familiarize the clinician with the different aspects of this unusual disease. The role of modern diagnostic tools in medical imaging in order to assess clearly the limits of the tumors and to enhance the efficiency and the safety in the choice of a surgical approach is pointed out. The treatment guidelines for IP have undergone a complex evolution that continues today. Radical excision of the tumour is technically difficult and often incomplete. Successful management of IP requires resection of the affected mucosa which could be achieved with open surgery, endoscopic, or combined approach. Radio and chemotherapy were used for certain indications. More optimally research would be a multicenter randomized trials with large size cohorts. Inverted papillomas generate considerable interest because they are locally aggressive, have a propensity to recur, and are associated with malignancy , 2. Recupapilloma means neoplasia with epithelial growth. The US National Cancer Institute's [The term titute's  has defititute's . It may In 1600s, C. Victor Schneider demonstrated that nasal mucosa produces catarrh and not CSF and identified its origin from the ectoderm. The first report of this type of tumour in the nasal cavity was made by Ward in 1854 . RingertThe lining of the nasal cavity and paranasal sinuses is unique in the upper aerodigestive tract in that it is ectodermal in origin. It is formed of ciliated, pseudostratified columnar epithelium, the Schneiderian membrane, with a thin submucosa containing seromucous glands. The Schneiderian membrane is of ectodermal origin from the nasal placode . The subSchneiderian papillomas most often arise from the ectodermally derived mucosa of the nasal cavity and sinuses (Schneiderian epithelium).Inverted papilloma arises from the outlining Schneiderian respiratory membrane . The behfungiform (everted) papillomas  (50%): arise from the nasal septum and have an exophytic growth pattern, oncocytic Schneiderian papillomas: formerly known as “cylindrical cell papillomas,” arise from the lateral sinus wall or paranasal sinuses, and lined with a distinctive stratified lining of bright pink granular columnar cells with microcysts, and more often mixed with typical inverted papilloma, rather than presenting in its pure form [ure form –34, inverted papillomas: arise from the lateral nasal or sinus wall or paranasal sinuses and show an endophytic or mixed exophytic/endophytic growth pattern [ pattern . The histomorphologically based classification formulated by Hyams (1971) , 22 diviThese tumors all are biologically similar . The conThe World Health Organization (WHO) classification basically followed this classification . HoweverIP can be found in the lateral wall of the nose, nasal septum –44, the Grossly, inverted papilloma looks like a polyp with a narrow or broad-based stalk, but is usually firmer with significant bulk and has more of a granular mulberry type appearance. They are a variety of colors from red to pale pink. IPs have an irregular, friable appearance. They are usually more vascular than the average polyp and bleed easily. IP must be distinguished from squamous papillomas, which consist of an arborizing fibrovascular core with a bland squamous epithelial lining and from sinonasal polyps, which represent localized outpouchings of the sinonasal mucosa due to edema , 82, 83.Microscopic features are that of digitiform proliferation of squamous epithelium with extensive invasion of the hyperplastic epithelium into the underlying stroma. The tumor has crypts which are subepithelial and maintain connection to the surface epithelium at all times; a finding which lead to the name inverted papilloma. Microcysts containing mucus are often trapped within the neoplastic epithelium. The lining consists of multilayered nonkeratinized squamous epithelium with scattered mucous cells and microcysts filled with neutrophils. The covering epithelium can be squamous, respiratory, or transitional cell epithelium or a combination of all three . The celOn the bases of electron density, cells from inverted papilloma specimens are characterizable as light, intermediate, and dark cells. This variation reflects variations in the amount of granular endoplasmic reticulum and non-membrane-bound polysomes. Cytological characteristics include filament formation, filament concentration into bundles, and changes in nuclear structure leading to pyknosis .Metaplastic change might play an important role in the malignant transformation of IP. Evidence suggests that IP originates in submucosal glands, specifically exocrine glands in the sinonasal region. In these glands, metaplastic change was commonly seen and occasionally revealed malignant features such as SCC in the sinonasal cavity . FeatureAs many as 75% of patients with IP have evidence of various degrees of bone destruction. These may include thinning, remodeling, erosion, and (less commonly) sclerotic bony changes. Benign sinonasal masses and slow-growing neoplasms tend to remodel the nasal vault and facial bones, and this is particularly true of nasal polyps and inverted papillomas. However, when such benign masses press against the floor of the anterior cranial fossa and the walls of the sphenoid sinuses, simulated aggressive bone destruction rather than bone remodeling usually occurs . The preThis type of bone destruction implies to the radiologist that a carcinoma may also be present , and thiBenign and malignant IP can displace neighbouring structures, causing extension into the intracranial cavity with or without dural involvement . Intracr Extension to intracranial temporal fossa and middle ear has been reported in few cases in the literature . MassiveMost tumors extended into the nasal cavity or ethmoid sinus combined frequently with erosion of the lateral wall or intersinus septum. Simultaneous attachment to multiple walls including both lateral wall and floor attachment was noted . The comIntracranial extradural inverted papilloma can be effectively controlled with craniofacial resection. Intracranial intradural involvement of inverted papilloma has a poor prognosis regardless of treatment. Aggressive treatment of intranasal inverted papilloma may be the most important factor in preventing intracranial presentation , 107.The etiology of inverted papillomas is still unclear. There have been many causes suggested such as allergy, chronic sinusitis , viral iHistological analysis suggests that IP tumorigenesis may occur through a stepwise series of discrete events graded according to a four-stage histological grading system . Viral infection and genetic insults may be required to progress –112. IndViruses have been implicated due to the well described finding in recurrent respiratory papillomatosis. There is little consensus regarding the incidence or role of Epstein-Barr virus (EBV) in IP , 120 whiThe observation that IP tends to recur after incomplete surgical removal supports investigations suggested that IP is a true neoplasm arising from a single progenitor cell and that recurrence represents growth of the residual clone . AlthougCIP/KIP family proteins entitled p21(WAF1/CIP1) and p27(KIP1) have key positions in cell cycle regulation leading to an arrest of cell proliferation. They are supposed to enable a repair process of DNA damage. In several human tumors, a loss of these proteins is associated with poor clinical outcome . IP contInverting papilloma is a relatively uncommon neoplasm of the nasal cavity, constituting 0.5% to 4% of all primary nasal tumors , 210–216Since the nasal cavity and sinuses are air-filled cavities, tumors must reach a significant size before they cause symptoms. Symptoms usually do not develop unless the involved sinus cavity is obstructed or the tumor has extended beyond the confines of the sinus, producing symptoms secondary to invasion of adjacent structures  test for possible Wegener granulomatosis should be considered. This condition often mimics a neoplasm. Preoperative laboratory studies include a complete blood cell count, electrolyte evaluation, and bleeding and coagulation parameters. Masses located around the sella turcica merit serum endocrine studies.Cytologic examination is a useful initial approach in the diagnosis of IP, and in the differential diagnosis of other tumors that occur in the same sites , 272. BiSurgical approaches may be necessary, although open biopsies should be avoided if possible to minimize the risk of contaminating otherwise noninvolved tissue planes. The maxilla can be approached through an anterior antrostomy through an upper gingivobuccal sublabial incision  . The ethImaging of the sinuses is essential before a treatment plan can be proposed to provide additional information for staging and treatment planning. The true extent of locally aggressive IPs cannot be accurately assessed on clinical examination because of their location within the bony craniofacial skeleton. There is currently not enough evidence to suggest one sole modality as providing optimum imaging for IP. The use of plain radiographic films and tomograms has given way to computed tomography (CT) and magnetic resonance imaging (MRI), which offer far more detailed information about the size and behavior of tumors , 275. CT Several radiologic patterns could be seen, therefore it is difficult to categorize any as specific for inverted papilloma , 281. A CT scanning is the ideal screening examination when a sinonasal tumor is suspected and the initial investigation of choice for assessment of a confirmed neoplasm , 79, 285Although using multiple CT diagnostic signs provides a reasonable assessment of tumor origin and extent, accurate tumor mapping was still impossible because of inadequate differentiation of tumor from inflammatory pathologies . This drNot only MRI is especially good for detecting soft tissue changes, but also MRI is superior to CT scanning in distinguishing papillomas from inflammation. The main utility of MR imaging is in defining the extent of the lesion, providing better delineation of the lesions in contrast to surrounding soft tissue , 302. InOjiri et al.  discusseExperience is growing with positron emission tomography (PET) using such agents as 18-F fluorodeoxyglucose and C-ll methionine to image the metabolic activity of a range of head and neck tumors, including those of the nasal cavity and paranasal sinuses. However, their routine clinical application is still under investigation . The SUVThe combined use of imaging techniques and diagnostic nasal endoscopy allows for an accurate diagnosis and enables minimally invasive techniques to be tailored to the patient's disease . IntraopAbsence of neurological symptoms at the time of diagnosis and first treatment of the disease does not eliminate a later on intracranial extension. Consequently, radical surgery and proper followup might prevent this unusual complication . SelectiStaging allows the assessment of outcome following different approaches. Also, having standardized grouping of IP is necessary to allow valid studies or meta-analysis . DiffereKrouse  introducIn 2007, Cannady et al.  developeRegional nodal metastases are uncommon . Lymph nThe treatment algorithm for this tumor has undergone a complex evolution that continues today . The rarAs with other types of skull base tumors, a multimodality interdisciplinary team approach should be considered, with a tumor board consultation being recommended, including otorhinolaryngologist, head and neck surgeon and a neurosurgeon, and when indicated a neuroradiologist, pathologist, radiation oncologist, oncologist/neurooncologist, radiation oncologist, neurologist, neurophysiologist, ophthalmologist/neuroophthalmologist, oral/maxillofacial surgeon, and plastic surgeon as active members. Neurosurgery is needed for skull base involvement, intracranial extension, and potential dural involvement. Although multiple surgical and adjuvant approaches are available, a balance should be found in attempting to preserve cosmetic, oral, and nasal function. When possible, the orbit should be preserved and reconstructed. Visual acuity, evaluation of any extraocular motility disturbances, and proptosis should be documented through an ophthalmology consult. Postoperatively, the ophthalmologist may assist with treatment of epiphora or dry eye syndrome, with dental consultation to evaluate for dental extraction in preparation for radiotherapy. Consult a prosthodontist if maxillectomy is expected, and consult speech pathology as needed. Despite these seemingly competing needs, first and foremost should be the goal of a safe and complete eradication of disease, when possible .Interferon and other antiviral agents like intralesional therapy with Cidofovir has been suggested for the use of a patient with multiple recurrences, advanced disease, or spread to the orbit and skull base. This is still investigational [The role of medical therapy is limited; it is used mainly as an adjunct to specific complications such as sinusitis. With some of the data suggesting the human papilloma virus may be a potential etiologic factor in the development of inverted papilloma, the use of antiviral agents may be beneficial. gational , 335.Mitomycin C was tried as an adjuvant therapy for dacryocystorhinostomy (DCR) approach in the treatment of Schneiderian papilloma of the nasolacrimal tract, and it showed preservation of function, compared with the conventional treatment of dacryocystectomy alone [my alone .Preoperative medical therapy can help normalize inflamed mucosa and minimize intraoperative bleeding . AdjunctRadiation therapy is generally not a recommended option to surgery in the treatment of benign IP. Although surgery is generally the primary treatment modality for this disease, radiation therapy should be considered for patients with early or multiple recurrence, advanced stage lesions, biologically aggressive tumors , incomplThe primary and preferred treatment of inverted papilloma is surgery. Advances in the surgical approach to the anterior skull base have resulted in increased opportunity for the cure of tumors involving these structures. Traditionally, surgical resection has been the primary treatment modality for the management of sinonasal tumors involving the maxillary or ethmoid sinuses that are centrally located in the nose and sinuses , 348; hoPrecise determination of the sites of tumor origin and attachment during the operation, strict application of selection criteria, proper preoperative evaluations, intraoperative determination of extent and attachment of the tumor, close endoscopic followup, expert application of endoscopic techniques, meticulous use of subperiosteal dissection in the involved areas, wide removal of the tumour origin along the subperiosteal plane as well as drilling the underlying bone , completTraditional surgery is reserved for more extensive lesions, recurrent lesions, or patients who have developed squamous cell carcinoma arising from an inverted papilloma –381. MasEarly and aggressive surgery is usually recommended , 397–401Growth in experience with the endoscopic management of inverted papillomas has led to evolving technical improvements and innovations. The recent literature has further refined the technique of endoscopic resection of inverted papillomas by delineating essential principles and applying new technologies, such as image guidance and angled endoscopic drills. Modified approaches and methodology have been described to address tumors originating in particular anatomic locations. In most tumor locations, the completeness of resection achieved by a skilled endoscopic surgeon , 416 is A treatment based on endoscopic resection with image guidance appears to offer a safe treatment modality of inverted papilloma with insignificant morbidity . The imaExternal approach has been the golden standard for sinonasal tumor  removal limited conservative surgery,lateral rhinotomy,transfacial with midface degloving,craniofacial resection ,endoscopic approaches.The available surgical techniques are as follows:Conservative surgery  is successful in controlling some cases, but may requires two or three operations. The surgical exposure in inverted papilloma should be adequate to ensure complete excision. Selected patients with limited size localized disease can be satisfactorily managed by conservative procedures if they are carefully followed , 430. LiThe traditional lateral rhinotomy incision described originally by Moure in 1902 has proved a versatile approach to the midfacial skeleton . Most ofMidfacial degloving is an extended sublabial rhinotomy, degloving constitutes a hidden route of access to the middle third of the face. It combines a bilateral sublabial incision with a rhinoplastic approach, whereby all the soft tissues of the face can be undermined subperiosteally, which permits good exposure of the midface, extended bilateral route of access to the nasal cavities and ethmoid/sphenoid/maxillary paranasal sinuses, nasopharynx, the base of the skull, and the clivus. It is an excellent alternative to the lateral rhinotomy in exposure to the nasal cavity, paranasal sinuses, and the nasopharynx. The exposure obtained using the degloving approach is superb. By lifting the soft tissue from the midportion of the face, many extensive benign inverted papilloma and some limited malignant lesions could be safely removed. This procedure may be associated with other neurosurgical approaches in the treatment of extended lesions of the base of the skull. Its use is particularly recommended in the treatment of inverted papillomas, especially in cases requiring bilateral surgery, although it applies as well to malignant tumors of the sinuses under certain conditions. It can be enlarged by supplementary incisions to meet the demands of tumour surgery –443. ThiThe postoperative functional outcome is good with minimal complications and low recurrence . Common Denker rhinotomy and medial maxillectomy through a sublabial technique, with dislocation of the nasal septum into the opposite nasal passage, provide excellent surgical exposure for these same lesions, while external facial incisions are avoided , 453. A IP of the paranasal sinuses can be removed thoroughly with a craniofacial approach that provides for good visualization. With this approach, some tumours previously considered inoperable may be removed and in some cases cured large extensive inverting papilloma of the ethmoid sinus that invaded the orbit and coursed over the globe around the optic nerve . Skull bA modification technique of frontal craniotomy allows excellent access to the anterior cranial fossa and orbit. It is the approach of choice for orbital tumors. This technique results in en bloc removal of a bone flap which incorporates the orbital roof, superior orbital rim, and frontal bone. Its advantages are excellent exposure, clear visibility in the placement of chisel cuts, and less brain retraction than conventional techniques . Endosco The advances in endonasal micro-endoscopic surgery allow both safe and effective removal of IP with low morbidity, and therefore it could be the approach of the first choice . FunctioEndoscopic sinus surgery is a gold standard in the diagnosis and treatment of paranasal sinus inflammatory disease since 1970s –463. MorThe availability of different endoscopic techniques allows the entity of the dissection to be modulated in relation to the extent of disease . TailoreCases with invasive disease or previous medial maxillectomy are treated via the open approach. Otherwise, the extended endoscopic approach is used . The tumin situ and to invasive carcinoma [There are two main clinical problems that require particular attention in the management of skull base IP: the tendency for recurrence, and the risk of malignancy , 210. Rearcinoma , 497.The magnitude of the recurrences is directly proportional to the completeness of removal with the best results obtained by techniques that afford the best operative exposure . The incwith IP (synchronous carcinoma) or at a later time (metachronous carcinoma). Why carcinomas in inverted papillomas arise meta—or synchronous is also still unknown. The majority of cases in the literature are synchronous carcinomas [There is also a variable wide range in the frequency of reported carcinoma in patients with IP, ranging somewhere between 1–50% , 519. Carcinomas , 520. Sircinomas . The avercinomas , 505. Malignancy was found to be associated with bilateral inverted papilloma, histologic multicentricity , a predoPatients with associated squamous cell carcinoma were found to be in older age groups, were more likely to manifest epistaxis than the more common unilateral nasal obstructive symptoms, and had less time between the onset of symptoms and presentation than those with inverted papillomas alone . Male geTherefore, this emphasizes the importance of long-term followup with clinical examination and endoscopic. CT scan has been indicated for all patients with inverted papilloma to detect recurrences ad malignancy in all patients , 426. ThComplications of treating sinus malignancies are related to the extent of surgery  and recoBleeding may occur if control of the large vessels is overlooked. This problem may occur if the artery is initially in vasospasm and if no active bleeding is noted until after surgery. The anterior and posterior ethmoid and sphenopalatine arteries may be cauterized, clipped, or ligated to prevent or control bleeding. If needed, interventional radiology may be requested to assist with intra-arterial coiling to control bleeding.Cerebrospinal fluid (CSF) rhinorrhoea and temporary nontension pneumocephalus are rare complications , 529–531Conservative management with bed rest and a lumbar drain can be used for the first 5 days in addition to placement on antibiotics. If resolution has not occurred, surgical intervention should be used, including patching with a dermal allograft, turbinate bone, and nasal mucosa. Mucosal flaps can be elevated and used to close the leaks with interpositioned bone or cartilage. For large leaks, a spinal drain may be necessary to allow grafts and sealing techniques to solidify and integrate. Cerebrospinal fluid leaks can be successfully repaired endoscopically . In patiEpiphora is a common complication of surgery caused by obstruction in the lacrimal outflow tract . This caDiplopia is a known complication in any surgery involving the orbital cone. Proper repair of the orbital floor is a key to prevent this complication, but in some cases it is unavoidable even with meticulous reconstruction. In cases of diplopia, prism lenses are usually the simplest method for correction, as surgical correction by ophthalmology can be complicated by prior scarring from surgery and radiation treatment. Ophthalmology consultation is standard of care.Facial disfiguration is one of the most important patient concerns and can lead to considerable social and psychological stress. This outcome must be dealt with initially and on an ongoing basis. However, the ability to resect and reconstruct has improved significantly . WithoutOne of the most difficult decisions made during surgery is when to take or leave the orbit. Suarez reviewed the literature and had 3 recommendations . First, If the eye is preserved, postoperative radiation is usually recommended. Nonetheless, patients should be counseled that despite surgical orbital preservation, impairment can occur from radiation, including optic atrophy, cataracts, dry eye, and ectropion. In the ideal cases, reconstruction preserves form and function. A free rectus flap or other distant tissue may be required to protect vital structures, or facial prosthetics may be used. Facial prosthesis can be offered to improve cosmetic results, but meticulous maintenance of the prosthesis by the team and patient is imperative.Close endoscopic followup is mandatory to ensure early recognition and treatment of recurrent disease , 537–539Complete examinations are performed by trained surgeon at regular intervals independent of the mode of treatment or the stage of disease. The posttreatment periodic physical examination should be methodical and comprehensive. The anterior nasal cavity can be evaluated with a nasal speculum, but when necessary, complete evaluation of the posterior nasal cavity and nasopharynx requires fiberoptic endoscopy. Aside from mucosal irregularities, other abnormalities that should be specifically searched for are pooling of secretions, tissue asymmetries, or bleeding. Examination of the ears should note serous or acute otitis media which can indicate eustachian tube blockage by an obstructing nasopharyngeal mass. Parameters such as hyperkeratosis, squamous epithelial hyperplasia, and a high mitotic index are negative prognostic indicators, which could be useful in the future followup of patients with IP . An addiThere are no prospective long-term studies with sufficiently large cohorts of equivalently staged patients. Today, there is no single right or wrong method, but a range from which to choose in the individual case. The true population base for reported figures is uncertain, given that many series were reported from tertiary centres, where recurrent and problematic cases are likely to be overrepresented . BecauseEndoscopic nasal and sinus diagnosis and surgery are appropriate for diagnosis, followup, and treatment of both limited and recurrent inverted papilloma, provided all patients are made aware of the possibility of recurrence and need for more extensive surgery as a result . Biopsie


Cases of pulmonary artery masses have only rarely been reported, and the optimal type of the diagnosis and treatment is controversial.An 81-year-old woman was found to have an abnormal shadow on chest X-ray film. Computed tomography showed an irregularly bordered tumor centered in the hilar region extending from segment 6 to the middle lobe of the right lung. Pulmonary angiography showed complete occlusion of the trunk at the periphery proximal to the bifurcation of the posterior ascending branch. Based on bronchoscopic biopsy of the tumor, an adenocarcinoma was diagnosed. Middle and lower lobectomy was performed. Histopathologically, the adenocarcinoma had invaded the tunica intima of the pulmonary artery and also replaced the endothelium in the same region. Although a large thrombus was found at the vessel invasion site of the adenocarcinoma in the pulmonary artery, there were no malignant findings in the thrombus itself.This is the first reported case of radical resection of a lung cancer with invasion along the pulmonary artery wherein a benign thrombus had formed. In general, surgery would be the treatment of choice for a pulmonary artery mass. Tumors growing into the pulmonary arteries include angiosarcoma and tumor embolizations from other organs -5. DespiAn 81-year-old woman was found to have an abnormal shadow in the right lower lung field on chest radiography for a routine health check-up Figure . Her pasIntraoperatively, the tumor was found to be located on the interlobar surface of S6-middle lobe, tending to invade proximally and distally along the pulmonary trunk. Massive induration was felt inside the pulmonary artery at the cancer invasion site Figure . Cancer Gross examination of the specimen disclosed that the tumor was mainly centered in the hilar region at S6, measured 40 mm, and was partially grayish inside Figure . The tumThe patient's postoperative course was uneventful, and she has remained in good health since discharge. In consideration of her advanced age, no adjuvant chemotherapy was performed. For the 14 months to date, since surgery, the patient has remained free of lung cancer recurrence, and will continue to be followed-up on an outpatient basis.We experienced a case with lung cancer directly invading the pulmonary artery and forming a thrombus at the vessel invasion site. To the best of our knowledge, no similar cases have been reported previously. The primary etiologies of pulmonary artery masses include pulmonary thromboembolism, angiosarcoma, tumor embolism from cancers of other organs, and growth of a primary lung cancer into the intravascular lumen -9, but tThe rarity of this tumor growth pattern makes it difficult to define its prognostic impact and pathological implications. For example, polypoidal cancer growth in a pulmonary artery is thought to lead to dissemination into the pulmonary circulation. However, such pathological findings are actually quite infrequent. Pulmonary artery invasion reportedly carries a better prognosis than other forms of mediastinal structural invasion . Thus, eIn conclusion, this is the first reported case of a lung cancer mimicking pulmonary artery sarcoma, but which actually formed a massive thrombus at the vessel invasion site. This mode of cancer extension is quite rare and its prognostic significance remains unclear. Although pulmonary arterial invasion is generally an ominous prognostic sign, presentation as a pulmonary artery mass may warrant open exploration for diagnosis and possible definitive treatment.Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: computed tomography; S: segment.The authors declare that they have no competing interests.TG wrote the manuscript. TG and RK performed surgery. AM carried out the pathological examination. RK was involved in the final editing. All authors approved the final manuscript.


Congenital internal hernia is a rare cause of bowel obstruction in adults and often presents with complications. A high index of suspicion, occasionally aided by appropriate radiological imaging, should lead to early surgical intervention and thus reduce morbidity and mortality. We describe a case of a 27-year-old woman who presented with upper abdominal pain and nonspecific abdominal signs. Computed tomography showed features of bowel ischemia which prompted surgical intervention. On exploration, she was found to have a large mesenteric defect with herniating ileum and ascending colon. A segment of gangrenous small bowel was resected. The mesenteric defect was repaired and the bowel tacked down to prevent volvulus. The patient made an uneventful recovery. Internal hernias are a rare cause of small bowel obstruction. TransmesAn otherwise healthy 27-year-old woman presented with severe upper abdominal pain, multiple episodes of vomiting and absolute constipation for the previous 12 hours. The pain was acute in onset, severe and continuous. It was initially located in the epigastrium but became generalized with progression of time. It was severe enough to warrant a continuous intravenous infusion of meperidine. The pain was associated with multiple episodes of nonbilious vomiting. She had no other comorbid conditions and had not undergone any operations in the past. On examination, she was drowsy with a pulse of 110/minute, blood pressure of 90/50 mm Hg, temperature of 37°C and respiratory rate of 23/minute. She was dehydrated. Abdominal examination revealed diffuse abdominal tenderness and voluntary guarding in the right lower quadrant. Bowel sounds were audible. A reducible paraumbilical hernia with a 2 × 2 cm fascial defect was also noticed. Breath sounds in both lungs were equal and vesicular. Results of laboratory investigations were normal except for a borderline elevated white blood cell count . A CT scIn about 0.2% to 0.9% of cases of small bowel obstruction, the obstruction is due to internal herniation. Herniation can be congenital or acquired. Common sites of internal hernias are paraduodenal (50%), supra- and/ or peri-vesical, intersigmoid, foramen of Winslow, omentum, postoperative mesenteric defects and congenital mesenteric defects.4Mesenteric defects are an established cause of internal herniation in nonoperated abdomens and provide a potential site for intestinal incarceration or strangulation. Congenital mesenteric defects most often occur in the small bowel mesentery and less commonly in the colonic mesentery. The vast majority of these cases have been reported in infants or children, often with an associated intra-abdominal anomaly. Murphy found that of the 11 infants presenting with herniation through a mesenteric defect of the small intestine, 10 had an associated anomaly, the most common being intestinal atresia. In adultThe most common location of mesenteric defects is in the region of the small bowel (70% of cases), with 53% of these being in the ileocecal area of the mesentery. These deMesenteric defects present with a spectrum of clinical features — from being asymptomatic to being a cause of unexpected death. Byaud autopsied two patients for unexpected death, one child of 3 days and a 23-year-old woman, and found bowel herniation through a congenital mesenteric defect to be the cause of death.A high index of suspicion for congenital mesenteric defects is warranted in patients who present with features of intestinal obstruction in the absence of obvious external hernia or previous abdominal surgery. Operative management consists of timely laparotomy, reduction of hernia, resection/ anastomosis of devitalized bowel and closure of the defect. The hernSevere unexplained abdominal pain in adults can be due to transmesenteric hernia. Diagnosis requires high index of suspicion, urgent surgical exploration and correction of the mesenteric defect. The innovation described is suggested for the management of patients with large mesenteric defects.


Abdominal compartment syndrome is a surgical emergency caused by a raised intra-abdominal pressure, which may lead to respiratory, cardiovascular and renal compromise. It is most commonly seen in post-operative and trauma patients and it has a variety of causes. Tension pneumoperitoneum (TP) is a rare cause of abdominal compartment syndrome most often seen after gastrointestinal endoscopy with perforation.We present the case of a fit 52-year-old experienced female diver who developed TP and shock following a routine training dive to 27m. Following accidental inhalation of water, she had an unstaged ascent and, on reaching the surface, developed severe acute abdominal pain and distension. She was brought to our emergency department by air ambulance for assessment. Clinical and radiological examination revealed a shocked patient with dramatic free intra-abdominal gas and signs of abdominal compartment syndrome, which was treated with needle decompression. Symptoms and signs resolved quickly with no need for further surgical intervention. TP is a surgical emergency where surgery can be avoided with prompt diagnosis and treatment. Tension pneumoperitoneum (TP) is a variant of abdominal compartment syndrome (ACS) with high intra-abdominal pressure caused by trapped gas leading to decreased venous return, decreased visceral perfusion and splinting of the diaphragm with consequent circulatory and respiratory compromise. It is an uncommon condition that most commonly results from gastrointestinal perforation during upper and lower gastrointestinal endoscopy with extravasation of insufflating gas. It has also been described after perforation proximal to an obstructing lesion in the gut, following blunt abdominal trauma, mechanical ventilation, pulmonary blast injuries and tracheal injury as well as following nasopharyngeal oxygen delivery and mouth-to-nose resuscitation in an infant.Gastric barotrauma occurs rarely during diving accidents due to swallowed air and an uncontrolled ascent from depth.A previously fit and well 52-year-old woman was brought to hospital with acute severe abdominal pain. She was an experienced diver undergoing a routine training dive in a quarry lake with an instructor. She had an uneventful dive down to 27m, using a trimix breathing gas. This contained a mixture of oxygen (32%), helium (10%) and nitrogen (58%). She was wearing a dry suit and an under suit below. During the exercise at 27m, she accidentally replaced her mouthpiece or regulator upside down, which resulted in inhalation of a small amount of water and air swallowing. She then had a rapid but controlled ascent without staged stops and developed acute severe abdominal pain on reaching the surface.The patient was brought to hospital by air ambulance and primary survey revealed an intact airway, hypoxia with oxygen saturations of 91% on room air and a respiratory rate of 20 breaths per minute. Her systolic blood pressure was 70mmHg and the pulse rate was 90 beats per minute. Abdominal examination revealed a grossly distended peritonitic abdomen.The differential diagnosis was gastric barotrauma due to air swallowing resulting in free perforation and peritonitis. Following intravenous fluid administration and placement of a Ryle’s nasogastric tube, an erect chest x-ray was performed that revealed a large amount of free air under the diaphragm but no evidence of pneumomediastinum . Urgent Needle decompression using a 14G cannula percutaneously through the abdominal wall in the left iliac fossa was performed as a temporising procedure prior to an exploratory laparotomy. There was an audible release of gas under pressure and a dramatic improvement in the patient’s physiological parameters with immediate resolution of pain and clinical signs.Given the rapid improvement and lack of any evidence of contrast leakage on CT, surgery was postponed and the patient was admitted for overnight observation. She was monitored closely for symptoms and signs of decompression illness after discussion with the local Diving Diseases Research Centre. She remained stable with no further symptoms. The volume of nasogastric tube drainage was zero and the tube was subsequently removed. There was no residual abdominal tenderness, blood tests were all normal and there was no pain on drinking clear fluids or eating. She was discharged home the next day and remained well at follow-up.>Primary ACS: Intra-abdominal pathology is directly responsible for ACS, eg intraperitoneal haemorrhage from penetrating trauma or a ruptured abdominal aortic aneurysm or pancreatitis.>Secondary ACS: Extra-abdominal injury leads to intra-abdominal fluid collection, eg fluid overload and burns.>Chronic ACS: This is secondary to cirrhosis with ascites, morbid obesity and peritoneal dialysis.ACS is a serious illness characterised by organ dysfunction secondary to intra-abdominal hypertension. The aetiology of ACS can be split into three categories depending on the cause:The aim of management should be prompt diagnosis and to maintain an adequate abdominal perfusion pressure. This can be achieved using a variety of techniques. The intra-abdominal pressure can be reduced by increasing abdominal wall compliance using analgesia, sedation and neuromuscular blockade where appropriate. Intraluminal and extraluminal pressure should be lessened by the placement of a nasogastric tube and urinary catheter and by drainage of compressing fluids where possible by abdominal paracentesis. Mean arterial perfusion should be optimised by cautious fluid replacement and the use of vasopressors if necessary. In a patient with primary ACS, a laparotomy and treatment of the cause may be necessary and should not be delayed. Temporary abdominal closure is essential in some cases if primary closure is impossible or a second look laparotomy is likely to be required.Rapid ascent by divers from depth causes a rapid increase in volume of trapped gas in hollow organs as the environmental pressure decreases (decompression). Decompression illness is a distinct entity and is caused by bubbles in blood or tissue during or after decompression. It includes two pathophysiological syndromes: arterial gas embolism and the more common decompression sickness. Arterial gas embolism occurs when expanding gas ruptures alveolar capillaries, allowing gas to enter the arterial circulation (pulmonary barotrauma). Decompression sickness is caused by the formation of bubbles of dissolved gas  in tissues. The bubbles can manifest as mechanical, embolic and biochemical phenomena ranging from minor to fatal.The exact mechanism of our patient’s TP was not clearly identified. Possible sources of entry were from pulmonary or gastric barotrauma. Breath-holding during ascent from a dive is well recognised to cause pulmonary barotrauma and subsequent passage of air from lung to abdominal cavity via the mediastinum has been described in a diver.Air swallowing during an unstaged ascent during diving can cause rapid expansion of gas and subsequent rupture of the stomach, usually along the lesser curve. TP has been reported in a diver ascending rapidly from a depth of 65m who exhibited other features of decompression illness and who required an emergency laparotomy and hyperbaric oxygen therapy.ACS is a relatively common condition but the causes are not always clear. Barotrauma from diving accidents does not usually present to the general surgeon on call and TP should be considered in this patient group presenting with abdominal pain and distension. Prompt diagnosis and management may make immediate surgery unnecessary but close observation and repeat examination are essential to avoid delaying surgery when indicated. Symptoms and signs of decompression illness should also be looked for and, if suspected, should prompt further discussions with a recompression facility.


We report the case of a 66-year-old man with a cervical neck mass located behind the left sternocleidomastoid muscle. To exclude malignancy, a full workup, including clinical, radiological, and cytological examination, was performed but failed to provide a definitive diagnosis. Histological analysis following excisional biopsy revealed a benign epithelial cyst, consistent with an atypically located branchial cyst. We describe an approach to the management of these neck masses and discuss several theories of the etiology of branchial cysts and how they may come to be abnormally located. A 66-year-old man was referred to our department with a 2-day history of a painless left cervical neck mass. He denied any other symptoms and reported that he only occasionally drank alcohol and had stopped smoking some 30 years ago with a total of 10 pack years. Clinical examination revealed a well-defined, painless neck lump, posterior to the sternocleidomastoid muscle close to the mastoid. The lump was some 5 centimeters in size, roughly oval, and not fixed to adjacent structures. There were no surrounding skin changes or other associated findings. Intraoral examination and transnasal fiber-endoscopy were normal. Cervical ultrasound showed an irregularly walled mass, 5 cm in diameter, with hyperechogenic reflections in an echo-poor center. The other neck structures were sonographically normal. Magnetic resonance imaging (MRI) showed a cystic lesion with an irregular wall lateral and posterior to the sternocleidomastoid muscle  of Waldeyer's ring  and papillary thyroid cancers have a predilection for cystic lymph node metastases , 8, 9. HThe aetiology of benign cervical cysts is also unclear. The most popular, but still controversial, theory is the branchial apparatus theory first described by Von Ascherson in 1832. Unfortunately, atypical locations of branchial cysts are poorly explained by this theory, and alternatives have been proposed such as the cervical sinus theory, the thymopharyngeal theory, and the inclusion theory –13. The Consequently, the thymopharyngeal theory indicates that lateral cervical cysts are a result of an incomplete obliteration of the thymopharyngeal duct .“benign lymphoepithelial cyst” instead of “branchial cyst” [Reporting a similar case to our own, Grignon et al.  proposedal cyst” . Furtheral cyst” .The diagnosis of a branchial cyst should—especially in patients older than 40 years—only be considered once malignancy has been excluded and should not be discounted because of an atypical location. Whilst in older patients metastatic SCC is more likely, in younger patients metastatic papillary thyroid cancer should be considered. Several theories exist as to the aetiology of branchial cyst formation, and some, such as the inclusion theory, are better able to explain abnormal locations.