Collagenous fibroma (desmoplastic fibroblastoma) is a recently described rare benign tumor affecting mainly males in the fifth through seventh decades. This tumor occurs predominantly in the peripheral sites, with predilection for upper and lower extremities. The patients present with a painless mass of involving subcutis, with one quarter of all cases involving skeletal muscle. Both radiographically and histologically these tumors are well-circumscribed small lesions from one to several centimeters in diameter, though lesions as large as 20 cm have been reported. We report a case of collagenous fibroma presenting symptomatically as a 2.4 cm mass within the pedicle and adjacent transverse process of the L5 vertebral segment. Bone erosion by desmoplastic fibroblastoma is very rare event. This tumor should be in the differential diagnosis of the soft tissue lesions presenting with bony erosion. Collagenous fibroma (desmoplastic fibroblastoma) is a recently defined entity first described by Evans in 1995 . CollageThe patient, a 56-year-old woman, presented with right L5 radicular pain, worse at night, which subsequently improved with Neurontin (Gabapentin) therapy. Imaging evaluation including plain films, CT, and MRI showed a lytic, minimally expansile intraosseous soft-tissue mass occupying the right L5 pedicle and extending into the adjacent transverse process . The les The most important information related to the lesions in the differential diagnosis of collagenous fibroma is summarized in  One additional differential diagnosis not included in this table is a “burned-out” phase of nodular fasciitis. Whether one wishes to interpret desmoplastic fibroblastoma as a “burnt-out” phase of a nodular fasciitis is probably irrelevant for all practical purposes. However, the nodular fasciitis can present similarly to the collagenous fibroma. One of the most recent papers  describeOur report is unique for several reasons. Our patient presented with pain, while the vast majority of collagenous fibromas are asymptomatic. In addition, bone erosion is a very rare event with only one case previously described . The imaSince a significant component of the tumor was at least contiguous with the adjacent soft tissue, the intraosseous component in our case probably represents an erosion of the vertebral body by the tumor. Interestingly, a peripheral entrapment of the skeletal muscle and adipose tissue was noted histologically , a featuIn summary, collagenous fibroma should be in the differential diagnosis of a well-circumscribed lesion with intraosseous component. Simple excision is recommended in symptomatic patients.


Complicated small-bowel diverticulosis is a rather uncommon cause of upper abdominal pain. It may lead to symptoms presenting with an acute onset or to chronic and nonspecific complaints. As the presentation is often similar to other pathologies  and in many cases diagnosis is made on basis of surgical findings, careful analysis of the imaging landmarks may be warranted to aid in the early stages of detection. In this report, we present clinical and morphological findings in three patients where small-bowel diverticulitis was surgically proven. The relevant literature is reviewed, and typical imaging properties are discussed. Small-bowel diverticulosis is an uncommon, acquired condition that is sporadically observed during conventional barium small-bowel barium follow-through studies. While frequently seen in duodenum, jejunal and ileal localisations are very rare. Only rarely symptoms occur unlike colonic diverticula, and the clinical significance of uncomplicated diverticula is minimal. Nevertheless, these small mucosal herniations may be the cause of occult upper gastrointestinal bleeding or may become impacted with food residue, become inflamed, and lead to an acute upper abdominal syndrome. The individual may present to the emergency room with a nonspecific inflammatory syndrome. Ultrasound and computed tomography will often be inferred in an attempt to narrow the differential diagnosis and demonstrate a focal inflammatory lesion. A specific diagnosis may not always be at hand, depending on the severity of the imaging findings diagnostic laparoscopy with segmental enterectomy may be required, while symptomatic treatment may suffice for less pronounced cases. The aim of this paper is to provide an overview of imaging findings in a small group of pathologically proven cases and to compare these results to those found in recent literature.Review of our patient database revealed three patients with a final confirmed diagnosis of jejunal diverticulitis. All three patients  had presented to the emergency room for work-up of acute onset upper abdominal pain had undergone an abdominal ultrasound and a multidetector CT exam.  For CT, patients drank a 2.5% solution of hydrosoluble contrast agent  over the course of one hour preceding the CT scan. All exams were performed on either a 16- or 64-slice MDCT scanner using standard preset procedures during the portal venous phase after injection of 120 mL of nonionic intravenous contrast material  at a rate of 2–3 ml/s using a power injector . Transverse and coronal slices of 3 mm were interpreted by the abdominal radiologist and later correlated with the surgical findings.A 83-year-old woman presented to the emergency room with a 3-weeks history of watery diarrhoea up to 5 times a day, dehydration, and a weight loss of 4 kg. No significant changes of the inflammatory parameters were found. The patient had a history of presacral immunocytoma treated by radiotherapy and chemotherapy. A plain radiograph and ultrasound of the abdomen at the emergency were unremarkable. The preliminary diagnosis was radio-enteritis. After 10 days of hospital the patient developed fever and an acute onset of abdominal pain. Computer tomography of the abdomen revealed 3 inflammatory mesenteric masses containing few air bubbles involving several jejunal loops and the colon transversum. Some contrast-filled and air-filled diverticula were present in the jejunum adjacent to the inflammatory mass, and the diagnosis of jejunal diverticulitis was suggested . At surgA 73-year-old woman presented with a 3-day history of left lower quadrant pain. Physical examination revealed left lower quadrant tenderness with rebound pain. There was a weight loss of 10 kg over 5 months. A plain radiograph was nonspecific with a few air-fluid levels. Computer tomography of the abdomen revealed multiple jejunal diverticula, surrounding inflammatory changes in the mesenteric fat adjacent to several jejunal loops with symmetric wall thickening. There were some minimal signs of pneumoperitoneum and some free intra-abdominal fluid . The diaA 74-year-old man presented to the emergency room with an acute onset of epigastric and periumbilical pain, described as being a continous pain more severe at the left side with rebound tenderness. His temperature was 39.3°C. There was no vomiting nor nausea. The patient had a history of stomach ulcer, appendectomy, and a cholecystectomy. A plain radiograph was nonspecific with a few air-fluid levels. The CT of the abdomen revealed the diagnosis of jejunal diverticulitis with limited signs of perforation . The patDiverticulosis of the jejunum and ileum is an uncommon entity, with a reported prevalence on conventional barium studies of 0.3%–1.9% and at autopsy of 0.3%–1.3% . They ar The majority of jejunal diverticula are composed of a thin wall composed of mucosal, submucosal, and serosal layer. These pseudodiverticula occur along the mesenteric border of the small bowel, usually hidden within the leaves of the mesentery. The cause of these diverticula is unclear, although it is likely that an abnormality in peristalsis, intestinal dyskinesia, and high intraluminal pressures plays a role in the pathogenesis .  There are no pathognomonic signs or symptoms of small-bowel diverticulitis. However, they may be associated with obscure gastrointestinal bleeding or bacterial overgrowth and may on occasion become impacted with ingested food, become inflamed, and present with acute abdominal pain. Complications due to jejunal diverticula include pseudoobstruction, blind loop syndrome, jejunal dyskinesia, and chronic diverticulitis complicated by the formation of enterolith. More acute complications include perforation, peritonitis, bleeding, and fistula formation .  At computer tomography small bowel diverticulitis usually presents as a focal area of bowel wall thickening most prominent on the mesenteric side of the bowel with adjacent inflammation and/or abcess formation. When a abcess is present, CT findings may include relatively smooth margins, areas of low attenuation within the mass, rim enhancement after IV contrast administration, gas within the mass, displacement of the surrounding structures, and edema of thickening of the surrounding fat or fascial planes. The differential diagnosis includes perforated neoplasm, foreign body perforation, small-bowel ulceration from nonsteroidal anti-inflammatory drug use, Crohn's disease, and diverticulitis . Perfora Resection of the involved area with primary jejunojejunal anastomosis is the surgical management of choice in the presence of perforated jejunal diverticular disease, hemorrhage, or abscess formation after a failure of a short course of bowel rest and antibiotics .


Many of the abdominal foreign bodies are due to accidental ingestion. Our objective in this case report is to emphasize the importance of the enquiry about the foreign body in the differential diagnosis of acute abdominal pain. According to our knowledge, this is the first report of bowel perforation caused by paper ingestion. A 14-year-old boy with abdominal pain underwent exploratory laparotomy and was found to have abdominal pus and ileal perforation. A crumpled paper was found at the site of perforation. Postoperative enquiry revealed that the patient had ingested 10 crumpled papers. We highlight that recording the history is an important aspect in the management of patients with acute abdominal pain and that foreign bodies should be included in its differential diagnosis. Most abdominal foreign bodies are due to accidental ingestion. Nearly 90% of these pass uneventfully, but few cause obstruction or perforation depending on their structure and size. Soft and pliable bodies are usually not mentioned by patients and sometimes may cause bowel perforation.2 ForeignOur objectives in this case report are to emphasize the importance of recording the history in the differential diagnosis of acute abdominal pain, and in this context, report the first case of bowel perforation caused by paper ingestion. To our knowledge, this is the first such case being reported in literature.A 14-year-old boy presented with right-sided abdominal pain for 3 days. It had started gradually and was aggravated with motion. The patient had a few episodes of nonbilious vomiting and anorexia. He had no history of other medical problems or medication except, a few analgesic tablets that were used for pain relief in the preceding 3 days. Clinically, he was febrile and had rebound tenderness on abdominal examination. Abdominal x-ray showed generalized gaseous distension.Patient underwent exploratory laparotomy after an initial working diagnosis of acute appendicitis. Upon exploration, the abdomen was found to contain pus and a 2×1 cm perforation in the 40 cm of terminal ileum with crumpled paper at the site of perforation. Additional undigested papers (5 pieces) were evacuated from the bowel .Postoperatively, upon enquiry, the patient claimed that he had ingested 10 crumpled papers.Foreign bodies may sometimes reach the alimentary tract through other routes like the anus, urogenital canal, or percutaneously, either deliberately or by mishap. A detail4Whenever one foreign body is known to have been ingested, consideration should be given to the possibility of a second one, and a barium swallow may be required to find these foreign bodies. A barium study is also warranted for follow-up after the acute impaction is resolved, and to detect an underlying stricture that is usually present.–6A foreign body impacted in the pharynx or esophagus is unlikely to pass spontaneously, and warrants immediate removal.4 The forMetal or bony materials are easily seen and lead-containing glass or crystal may be visible in X-ray, but plastic or wood may be challenging to diagnose preoperatively unless there is adherent lead paint. Fish bones and other sharp objects  present the risk of perforating the gut wall.7 SometimIdeally, sharp objects should be removed while still in the stomach, but round and pliable objects can be followed safely while passing through the alimentary tract, although a few cases of perforation have been reported. However,Metallic foreign bodies require special consideration in their management. The acid of the stomach may react chemically with the metal and result in mucosal inflammation, ulceration, and perforation. Similarly, gastric acid may break the seal of an ingested battery, leading to corrosive toxicity.9–11 The 612There are few therapeutic options in the management of some of these patients. A bolus may pass spontaneously during a barium swallow, aided by IV glucagon to relieve sphincter spasm, and effervescent agents. Foley's balloon catheter may be introduced although considerable care must be taken with impactions of more than 24-hour duration because of the potential for esophageal perforation from pressure necrosis.We recommend that the patient's history must be taken precisely to find the cause of acute abdominal pain, and it has considerable impact on the type of management. Foreign body ingestion should be included in the differential diagnosis of abdominal pain and even a soft and pliable object like paper may cause bowel perforation, although it is rarely reported.


Primary signet ring cell carcinoma of urinary bladder is a rare type of bladder tumor and carries a very high mortality rate. It may have a clinical presentation similar to common diseases like Benign Prostatic Hypertrophy (BPH) and the management options are extremely limited. We report a case of 58 year old Caucasian male who presented with a 5 month history of increased frequency of urination, nocturia and weight loss without any fever or hematuria. He was found to have an increased creatinine of 2.8 mg/dl and a prostate specific antigen level of 0.18 ng/ml. His azotemia was thought to be secondary to BPH. A foley catheter was initially placed with a plan for outpatient follow up. On removal of the catheter his problems persisted and he returned to the hospital. Diagnostic work up including abdominal ultrasonography, computed tomography (CT) scan, retrograde pyelogram, cystography and cystoscopic biopsies revealed the diagnosis of primary signet ring cell carcinoma of urinary bladder. Although cystectomy was planned, our patient passed away before this could be done. Primary signet ring cell carcinoma of urinary bladder is a rare type of bladder tumor and carries a very high mortality rate. It may have a clinical presentation and symptomatology similar to common diseases like Benign Prostatic Hypertrophy (BPH). It is diagnosed with bladder biopsy. Unfortunately, due to the rarity of this disease entity and due to the aggressive nature of tumor, the treatment options are extremely limited.A 58 year old previously healthy Caucasian male presented with a five month history of increased frequency of urination, feeling of incomplete emptying and nocturia. He denied any history of fever, hematuria, nausea, vomiting or diarrhea. He complained of approximate 10 pound weight loss over a period of 1 week. He reported a previous diagnosis of enlarged prostate with a normal Prostate Specific Antigen (PSA). He also reported a recent history of "small heart attack" for which he was medically treated at an outlying facility where he was also informed about poor kidney function. He also gave history of recently diagnosed hypertension and a 80 pack year of ongoing smoking. His medications included amlodipine, finasteride, doxazosin, metoprolol, clopidrogel, aspirin and lovastatin. All of these were started 5 days prior to presentation. Physical examination revealed a healthy appearing male with normal vital signs. His rectal exam revealed a slightly enlarged, non-tender prostate. Rest of the physical exam was unremarkable. Laboratory data revealed hemoglobin 10.3 g/dl, blood urea nitrogen 24 mg/dl, creatinine 2.7 mg/dl, and PSA level 0.18 ng/ml. Urinalysis was negative for RBC's, WBC's, bacteria, or nitrates. Retroperitoneal ultrasound showed normal sized kidneys with mild pyelocaliectasis bilaterally, prostate measured 4 × 2.7 × 2.6 cm with homogenous echotexture, otherwise unremarkable. Immediately after placement of foley catheter he had 650 ml of urine output. Blood urea nitrogen decreased to 18 mg/dl and serum creatinine decreased to 1.7 mg/dl. His azotemia was thought to be secondary to BPH and he was discharged with indwelling foley catheter to be followed up as an outpatient. At outpatient clinic, on removal of his foley catheter, his post void residual was found to be 150 ml. Various management options including surgical interventions were discussed with the patient in detail. After discussion patient was discharged home to be followed up as an outpatient but was started on tamsulosin. He returned to the hospital with complaints of urgency and frequency. His creatinine had increased to 5.0 mg/dl. Abdominal CT at this time revealed bilateral hydronephrosis and hydroureter  is a rare variation of adenocarcinoma of the urinary bladder. English literature review on pubmed reveals less than 100 cases reported. Adenocarcinoma constitutes 2% of all bladder cancers with the majority being metastatic rather than primary. Signet-Most patients present in middle-age with symptoms indistinguishable from the much more common transitional cell carcinoma of the bladder. The most common presenting symptoms are hematuria and difficulty in urination which can be mistaken for a urinary tract infection. In cases of rapid growth in the trigone area, oliguria, bladder irritation, and renal failure can be the initial presenting signs . YamamotGrignon et al compared the gross features of 34 cases of primary signet-ring cell carcinoma. They found that 47.1% have no mucosal or mass lesion present on cystoscopy. The most common description is of "edematous mucosa" but descriptions of erythematous or finely granular mucosa are also found. As in our case, the bladder wall is often described as thickened or fibrotic  but in mSignet ring cells are described as crescent shaped cells containing nuclei compressed to one edge of the cell by large amounts of cytoplasmic mucin appearing as a single clear vacuole in some tumors and as a foamy, multivesicular cytoplasmic material in others ,8. MucinThree theories for the histogenesis of this type of carcinoma have been suggested in literature. First is the metaplastic potential of urothelium which may occur along the surface of the bladder or in areas of cystitis cystica within the bladder. The second is that diffuse signet ring cell adenocarcinoma derives from isolated signet ring cells that exist scattered in normal transitional epithelium. The third possibility is that of signet ring cell carcinoma arising from metaplastic transitional cell carcinoma ,10-14.Treatment options for PSRCC are limited. This is a rare disease entity; no specific chemotherapy has been recommended in the literature. Radiotherapy has also not been shown to be successful . Total cPSRCC of urinary bladder is a rare entity with a very poor prognosis. Since patients may present with clinical picture similar to common diseases like BPH it may be difficult to diagnose early in the course of the disease. Treatment options are extremely limited and not well studied. Therefore it is important for the clinicians to be aware of this disease entity.The patient is now deceased. Written informed consent for publication of this manuscript and any accompanying images was obtained from the patient's next of kin. A copy of the written approval is available for review by the Editor-in-Chief of this Journal.The authors declare that they have no competing interests.KG and JF participated in patient management, literature review and helped in conceptualization and drafting of manuscript. MP helped in conceptualization, drafting and reviewing of the manuscript. All authors read and approved the final manuscript


Uterine leiomyosarcomas are relatively uncommon soft tissue neoplasms and rarely metastases to small bowel. In the current case; a patient is suffering from intestinal perforation due to metastatic leiomyosarcoma of the small bowel.A 59-year-old woman underwent a modified radical mastectomy for infiltrating ductal cancer of the breast six years ago and a total abdominal hysterectomy for leiomyosarcoma of the uterus two years ago. About 2400 cGy total dose radiotherapy has also been applied after total hysterectomy for bone metastasis of breast cancer. She admitted to our clinic with the complaints of acute abdomen due to perforated small bowel metastasis of leiomyosarcoma during the radiotherapy. Laparotomy was performed and leiomyosarcoma of the ileum was removed totally. Histopathologic examination of the specimen confirmed the presence of the leiomyosarcoma in intestinal tissue samples.We aimed to present this unusual case which perforated presentation of the intestinal metastasis of uterine leiomyosarcoma. Uterine leiomyosarcomas are relatively uncommon soft tissue neoplasms. They mostly metastases to lung and liver tissue, whereas rarely metastases to small bowel, heart, brain and thyroid ,2.In this current case the patient's admission complaint was acute abdomen  due to leiomyosarcoma of the uterus. Small bowel perforation due to uterine leiomyosarcoma is relatively uncommon. However this is a unique condition, the route of spreading from uterus via small bowel is likely to be related with hematogenous spread.A 59-years-old Turkish woman who had undergone modified radical mastectomy for a stage IIB (T2N1M0) infiltrating ductal carcinoma of left breast. After modified radical mastectomy of the left breast, she had a disease free period of four years. Afterwards disease free survival, the patient suffered from menorrhagia. On pelvic examination, an enlarged uterus, the size of which was similar to that at 12 weeks' gestation, was noted and ultrasonographic examination revealed a leiomyoma of the uterus. The patient had underwent a diagnostic laparotomy for a suspicion of leiomyoma. During the evaluation total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy and appendectomy was performed. The uterus was symmetrically enlarged, weighed 650 gr, and measured 13 cm × 12 cm × 6 cm. Uterine tumor measuring 9 × 7 × 5 cm with central necrosis, and there was no obvious disease involving the bilateral ovaries, lymph nodes or omentum The result of pathology was a leiomyosarcoma of the uterus without any metastatic lesion. There was no evidence of uterine serosal and pelvic lymph node involvement. No adjuvant treatment, such as chemotherapy or radiotherapy, was arranged after the surgery.Sixteen months later the patient had undergone cholecystectomy and incisional hernia repair. During laparotomy there was no evidence of metastasis. Afterwards these operations, during routine controls a metastatic lesion in the lomber vertebraes was found. While the patient was receiving radiotherapy for this metastatic bone lesion. She admitted to the emergency room for abdominal discomfort. Physical examination revealed tenderness and rigidity also labaratory tests were as follows; hemoglobin 9.7 g/dl, white blood count 17 500 mm³, chest and abdominal plain radiography were normal. Abdominal ultrasonography revealed fluid collection in the pelvis.Then the patient had undergone an emergency surgical operation. A solid necrotic tumoral mass 4 × 5 cm in diameter was found 2 inches proximal to the ileocecal valve. There was also a perforation in the small bowel with generalized peritonitis. The tumoral segment was resected with tumor free margin and also with regional lymph node dissection was performed.Microscopically the tumoral invasion was observed all through the intestine wall from mucosa into the serosa. There was areas of necrosis with spindle cells in an interlacing or fascicular patern Figure . HistopaAfter 10 months disease free survival the patient admitted to hospital with low gastrointestinal bleeding. During evaluation; a metastatic lesion was evaluated in transverse colon. The patient rejected any kind of medical assistance and died after a short period of time.Uterine leiomyosarcoma is a rare disease and is difficult to differentiate from benign leiomyoma, even by MRI. Uterine leiomyosarcoma presents symptoms similar to leiomyoma, such as massive uterine bleeding, pollakiuria and abdominal pain, and is frequently asymptomatic. Leibsohn et al. reported that out of 1429 patients who underwent hysterectomies due to presumed benign leiomyoma, leiomyosarcoma was histopathologically diagnosed in seven patients (0.5%) .Leiomyosarcoma of the gastrointestinal tract can either be primary or secondary. Leiomyosarcomas in uteri can invade by direct extension, hematologic route, implantation, or via lymphatic route . SpontanEvans et al. reported that all smooth muscle tumors arise from the muscularis propria layer of the intestinal wall . Wilson In the current case, the leiomyosarcoma of the intestinal wall was accepted as a metastatic lesion because it arises from the mucosal layer of the intestinal wall; contrary to the primary leiomyosarcomas.Secondary involvement of the bowel from uterine sarcomas have been reported and accepted as direct implantation by the transperitoneal route . There iRadiotheapy complications involved in the gastrointestinal system are classified into two distinct groups as: early complications, which occur during or immediately after treatment, and late complications, which can occur months or years after radiotherapy. Histopathologic signs of the tissue damage due to the radiotherapy was defined as atrophic alterations in villi and crypts, teleangiectasiae in the submucosa and inflammatory cell infiltrations in submucosal region . These fIn conclusion, small bowel recurrence following treatment for uterine leiomyosarcoma without other concomitant metastases is rare, and the route of spread remains uncertain. However, bowel metastasis via lymphatic or haematogeneous route from the primary site in somatic soft tissues is not very rare .Leiomyosarcoma of the uteri may result in a metastatic lesion in the mucosal layer of the intestinal wall and cause of an intestinal perforation as an unusual finding during admission.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.BS assisted the surgery, prepared the manuscript. ÖVÖ helped in searching references, assisted drafting the manuscript. BK gave final touch to the manuscript. APD did the surgery. ÖH assisted drafting the manuscript. FC guided during surgery, and in assisted drafting the manuscript. All the authors read and approved the manuscript.


Hepatic hemangioma, adenoma and focal nodular hyperplasia are the most frequent benign lesions of the liver, but they are all infrequent among pediatric population. The reports of focal nodular hyperplasia in children have recently increased in number, with many cases associated to drug intake, particularly to chemotherapy. We here describe, to our knowledge, the first case of focal nodular hyperplasia in association with diabetes mellitus in childhood. The focal nodular hyperplasia (FNH) first described by Edmondson in 1956, accounts for 8% of primary hepatic tumors in adults and less than 2% in children. Nonetheless, its evidence in pediatric population is increasing with more than eighty cases described in the last 5 years. The pathogenesis of FNH is largely unknown. Evidences of polyclonality in different DNA studies exclude a neoplastic nature of the lesion and further support the hypothesis of a possible reactive hyperplastic response of liver cells to local vessel abnormality. Chemotherapy and/or radiotherapy may be associated with development of FNH and, as clarified by a case-control study, also smoke may play a pathogenetic role [A.L., male, suffering from type 1 diabetes mellitus since he was 7 years old; the disease was metabolically compensated with stable insulin doses. At the age of 12 he was admitted for abdominal pain and a voluminous mass in right hypochondria was found; he was in good general conditions (32 kg of weight) and remaining physical examination was normal. His insulin requests were: regular insulin 5 IU before breakfast, 10 IU before lunch and 6 IU before dinner; NPH insulin 16 IU at bedtime. The liver ultrasound scan showed, at the VI liver segment, two hypo-isoechogenous oval lesions whose diameters were, respectively, 83 × 43 mm and 77 × 38 mm. CT scan Fig.  confirmeRoutine blood tests were all normal and the detection of serological markers of HAV, HBV, HCV, HIV and CMV was negative: CEA and αFP were also negative. On the basis of the mild abdominal pain, and also for a formal request of parents, the child underwent surgical intervention: the two masses were completely removed. The gross appearance was that of lobulated, well circumscribed masses, lacking a fibrous pseudocapsule and showing a central scar with fibrous septa running to the periphery and partially demarcating nodular structures. At histologic analysis, both lesions were classic FNH showing a nodular hyperplastic parenchyma with a typical central fibrous scar, containing a proliferation of small bile ducts, irregular tortuous arteries with thickened walls, veins and capillaries. A discrete inflammatory infiltrate filled the fibrous septa surrounding the hepatocytic nodules Fig. . The chiFNH is very rare in pediatric population with an age prevalence in children 7-8 years old, although some cases are diagnosed in early childhood or even in the prenatal period . The femThe majority (70-90%) of FNH at presentation is asymptomatic and the most common way that the disease is discovered is when, during an occasional physical examination, hepatomegaly or a palpatory abdominal mass are detected. More rarely the disease is diagnosed after the occurrence of abdominal pain, even though this event appears not exceptional in pediatric age. Almost ever the liver function tests are normal. The lesion is more often unique, but about 8% of cases may show multiple nodules, up to 30. The diameter of lesions is extremely variable, from less than 1 cm to more than 15 cm but usually is less than 5 cm.Some cases of associations with specific disorders or therapies, have been published:1. Glycogen storage disease type I: described up to now about 10 cases.2. Infrequent cases reported in association to adrenal cancer, adrenal pseudocyst or Cushing's syndrome.3. Rare and doubtful cases in association with drepanocytosis.4. Three cases reported after portoenterostomy for biliary atresia.5. Some cases reported after intake of drugs such as clomifene, busulfan and especially androgens.6. A plenty of pediatric cases detected after chemotherapy as well as after Hematopoietic Stem Cell Transplantation (HSCT) .7. Only five cases of association with diabetes mellitus have been reported until now: three cases were mentioned into a series of 28 Swedish patients, another one was a German 62-year old woman with long-term diabetes and the fifth was an Australian patient -13. TheyDifferent histological variants of FNH are recognized: the classic, the most frequent and corresponding to the present case, the not classic, including FNH with cytologic atypia, and mixed hyperplastic and adenomatous FNH. The so-called telangiectatic FNH is now considered a variant of adenoma .Classic FNH is characterized by hepatocellular trabeculae forming nodules separated by fibrous septae radiating from a central fibrous scar. The lesion is generally well circumscribed, without a capsule but surrounded by a rim of compressed parenchyma. The central scar may be absent or inconspicuous in many cases. The evidence of a typical vascular component is an important feature, probably related to the vascular malformative pathogenesis of the lesion.With modern equipment, the diagnosis is easy in the majority of cases:1. Ultrasound. Typical FNH is a mass with variable echogenicity and the typical hyperechogenic central scar. Moreover, with the doppler, the presence of areas of hypervascularization is appreciated in about 50% of the cases. The flow is predominantly arteriosus type, with high-speed and low resistance and irradiation from the center to the periphery. With the contrast enhanced ultrasound the typical pattern is characterized by an high arterial and portal venous enhancement.2. CT scan. Hyperintense lesion after administration of contrast; there is often an immediate contrast enhancement, mostly isodense, with high initial attenuation. There is also the ability to detect, when it is present, the hypodense area of the central scar. So it is possible to say that typical FNH lesions have homogeneous enhancement in hepatic arterial phase, presence of central scar, and delayed enhancement, while the atypical FNH, more frequent in male sex, may show heterogeneous immediate enhancement, absence of central scar, or presence of pseudocapsule .3. MRI. The enhancement after injection of contrast tends to be early, intense and homogeneous and the lesion appears more frequently as isointense or hypointense in T1 and isointense or slightly hyperintense in T2, even if atypical presentations are possible.Needle-biopsy or open air biopsy are necessary when the radiological investigations are doubtful, above all in case of absence of the central scar, and not rarely the differential diagnosis from other nodular lesions of liver may be difficult. The differential diagnosis includes different nodular lesions of the liver.Nodular regenerative hyperplasia (NRH) is a disease characterized by multiple nodules composed by hepatocytes, without a fibrous tissue or central scar . It is oThe typical imaging showing anechogenic and regular profile of the mass at ultrasound, easily recognize cystic lesions: however CT and MRI may be necessary in selected cases.In haemangiomas a doppler ultrasound test is diagnostic almost ever. At CT scan the contrast enhancement proceeds typically from center to periphery. Nonetheless in adults and, exceptionally, in children, it is possible to have a concomitance of FNH and haemangioma.Radiological findings play a key role also in the diagnosis of Adenoma (HCA). At ultrasound there is not the central scar and the doppler effect is predominantly venous type with either continuous or pulsatile peripheral flow: typically absent is the portal venous enhancement at the contrast enhanced ultrasound. MRI and CT may also highlight spots of hemorrhage and necrosis. The differential diagnosis is crucial, because the adenoma is at risk of breaking, haemorrhage, and malignant transformation. Surprisingly there are uncommon cases of simultaneous detection of adenoma and FNH. Moreover, the ductular proliferation may be minimal in some FNH at histological examination, making the differential diagnosis difficult .Hepatoblastoma, the most frequent liver tumor in childhood, is almost always associated to α-fetoprotein (α-FP) increase and there is not the central scar at CT scan. Similarly to what happen in adenoma, there are exceptional cases of concomitant presence of hepatoblastoma and FNH. The hepatocellular carcinoma, exceptional in pediatric population and often associated with α-FP increase, at the CT scan shows an hypodense lesion with a less prompt contrast enhancement than FNH and, at MRI, an heterogeneous initial enhancement after contrast injection.The natural evolution of FNH is unpredictable even if the mass, in about 2/3 of cases, remains stable and in about 1/3-1/4 of cases shows a gradual spontaneous improvement as far as a complete remission; it is rare an increase in number as well as in size .The recent studies in molecular biology have confirmed that FNH is not a pre-neoplastic lesion: the tissue parenchimal organization is pretty the same of usual liver tissue and, moreover, even though in some cases a clonal origin of FNH nodules have been demonstrated, until now no somatic mutation in the β-catenin gene or in the other genes implicated in the hepatocellular adenoma  have been discovered ,16. MoreAbout the management the first step is, of course, the stop of oral contraceptive. Considering the body of evidence that FNH doesn't undergo malignant transformation and that there are only sporadic cases followed by spontaneous rupture and consequent abdominal bleeding, we agree with the opinion that in asymptomatic cases it is opportune a careful follow-up with an ultrasound scan every 6-12 months, and that elective surgery has probably to be limited to the patients suffering of abdominal pain or with a voluminous or growing mass . It is wWe think that reporting children with FNH may be helpful to provide to clinicians data useful for the differential diagnosis process and for the therapeutic decisions: remarkably this is the sixth case of FNH associated to diabetes, the first, at our knowledge, in a child.Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PF, FC, FF and PD revised the clinical history and drafted the manuscript. RA carried out histological examination, provided the pictures of pathologic findings and contributed in preparing the manuscript. ST and AT provided useful contribution in the revision of literature and participated in writing discussion. All authors read and approved the final manuscript.


Benign mesenteric lymphangiomas are rare intra-abdominal cysts which may be asymptomatic or present with a variety of abdominal symptoms including an acute abdomen. We are however not aware of any reports in the literature linking mesenteric lymphangioma to acute pancreatitis. We present the case of a 62-year-old man who was admitted with signs and symptoms of acute pancreatitis and a palpable abdominal mass. Computerised tomography (CT) of his abdomen confirmed the presence of a mesenteric cystic mass. He underwent a laparotomy at which a large thin walled mass filled with a chylous fluid was resected. Histological analysis of this cyst showed it to be a benign mesenteric lymphangioma. A working diagnosis of acute pancreatitis and a palpable mass of unknown significance was made. CT scan of the abdomen revealed a large multi-lobulated cyst within the small bowel mesentery  and had a soft, distended, abdomen with palpable diffuse soft mass in the central abdominal area which was moderately tender. His chest X-ray was unremarkable, his abdominal X-ray in hindsight shows some peripheral displacement of small bowel, but was otherwise also unremarkable. His blood biochemistry showed an elevated serum amylase (762 u/dL) and an elevated alanine aminotransferase 191 IU/L (3-35). Other liver function test results were, total bilirubin 24 u/L (3 - 25), alkaline phosphatise 139 u/L (38 - 149) Gamma-glutamyl transferase was also elevated at 508 u/l (12-64). Serum lipase was not checked. His haemoglobin was 14.5 g/dl and white cell count was 13 × 10At laparotomy, a large thin walled multi-loculated mass measuring 25 × 15 × 10 cm Figure . These fEscherichia coli was cultured from it. He settled symptomatically with drainage of the collection and antibiotic treatment, and was subsequently discharged three weeks later. He represented one month after discharge with a recurrent intra-abdominal collection which also necessitated CT guided drain. There was no further recurrence of a collection after this. No recurrence of the cyst has been noted after a year of follow-up.In the early post-operative period, he made good progress but began showing signs of infection, with rising infection markers, pyrexia and abdominal discomfort. A CT scan of the abdomen revealed a pelvic collection requiring a CT-guided drain. This collection was purulent in appearance, and A diagnosis of acute pancreatitis should be considered in a patient presenting with acute abdominal pain, nausea and vomiting. The cause of pancreatitis is diverse but is often associated with gallstone disease or excess alcohol and can be discerned from the history and initial laboratory investigations. Other more obscure causes require further investigation. Approximately 10% are said to have idiopathic pancreatitis, where no obvious case is found [Our patient presented with typical symptoms of acute pancreatitis. He had evidence of peripancreatic inflammation on his CT scan and elevated serum amylase. Abdominal ultrasonography and CT also did not show demonstrate gallstones. Other liver function tests were not consistent with gallstone disease and he did not have a history of alcohol excess. Intra-operatively, the mass was found to be adherent to the head of the pancreas.Benign mesenteric lymphangiomas are rare intra-abdominal cysts thought to account for between 1 in 100,000 to 1 in 250,000 of hospital admissions . LymphanThe aetiology of lymphangiomas remains unclear. Congenital developmental defects of the lymphatics have been proposed, such as the proliferation and dilatation of blind ended lymphatic sacs which lack proper connections with the venous system . The theClinically, intra-abdominal lymphangiomas may be asymptomatic; however a gradual increase in abdominal girth, sensation of fullness and vague abdominal pain should alert the clinician of this rare condition. Pain may range from chronic background abdominal pain to more acute pain due to torsion or haemorrhage into the cyst, compression of surrounding structures or intestinal obstruction. They can also infiltrate into surrounding viscera and lead to organ dysfunction . AlthougThe diagnosis of mesenteric lymphangioma is usually made histologically following surgical resection of the cyst. There are no blood tests which will confirm the diagnosis. Radiological investigations using ultrasound or computerised tomography (CT) usually confirms the presence of a mass and may help to exclude other causes of intra-abdominal masses but is usually insufficient to provide a definite diagnosis. MRI has also been proposed as a suitable investigation for defining the origins of the mass and thus aid diagnosis . HoweverTreatment of mesenteric cysts is primarily by surgical resection. This has traditionally been through open surgery, however laparoscopic resection has been reported . OccasioOur case illustrates an unusual presentation of benign lymphangioma and options for treatment. Although rare, clinicians should consider mesenteric lymphangioma in the differential diagnoses of patients found to have intra-abdominal cystic masses on abdominal CT scans.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.SA did literature review and writing of manuscript. NB involved in literature review and contributed to writing and amending the manuscript. PDM supervised writing of the manuscript. All authors were also involved in the care of the patient and have approved the manuscript.


Pituitary adenomas represent the most frequently observed type of sellar masses; however, the presence of a rapidly growing sellar tumor, diabetes insipidus, ophthalmoplegia and headaches in an older patient strongly suggests metastasis to the pituitary. Since the anterior pituitary has a great reserve capacity, metastasis to the pituitary and pituitary involvement in lymphoma are usually asymptomatic. Whereas diabetes insipidus is the most frequent symptom, patients can present with headaches, ophthalmoplegia and bilateral hemianopsia.A 70-year-old woman with no previous history of malignancy presented with headaches, right oculomotor nerve palsy and diabetes insipidus. As magnetic resonance imaging revealed a sellar mass involving the pituitary gland and infundibular stalk, which also extended into the right cavernous sinus and sphenoid sinus, the patient underwent an immediate transsphenoidal decompression surgery. Her prolactin was 102.4 ng/ml, whereas her gonadotropic hormone levels were low. A low level of urine osmolality after overnight water deprivation, along with normal plasma osmolality suggested diabetes insipidus. Histological examination revealed that the mass had been the infiltration of a high grade B-cell non-Hodgkin's lymphoma involving respiratory system epithelial cells. Paranasal sinus computed tomography scanning and magnetic resonance imaging of the thorax and abdomen were performed. Since magnetic resonance imaging did not reveal any abnormality, after paranasal sinus computed tomography was performed, we concluded that the primary lymphoma originated from the sphenoid sinus and infiltrated the pituitary. Chemotherapy and radiotherapy to the sellar area were planned, but the patient died and her family did not permit an autopsy.Lymphoma infiltration to the pituitary is difficult to differentiate from pituitary adenoma, meningioma and other sellar lesions. To plan the treatment of lymphoma infiltration of the pituitary gland, it must be differentiated from other sellar lesions. The most frequent tumor of the pituitary gland is pituitary adenoma, but craniopharyngioma, Rathke cleft cyst, dermoid, epidermoid germinoma, metastasis, meningioma, arachnoid cyst, sarcoidosis, tuberculosis, histiocytosis, lymphocytic hypophysitis, schwannoma, infundibular glioma, cavernous carotid artery aneurysm and pituitary involvement in lymphoma or leukemia are included in the differential diagnosis of sellar and parasellar diseases ,2.Primary sellar lymphoma is a very rare disease accompanied by acquired immunodeficiency syndromes. The mean age of immunocompetent patients with sellar lymphoma is 60 to 70 years old. Sellar lymphoma is an exceedingly rare disease and is asymptomatic in most cases. Since the pituitary gland has a great reserve capacity, pituitary involvement in lymphoma also appears to be asymptomatic in most cases. In many cases, diabetes insipidus (DI) seems to be the most common clinical presentation as the posterior lobe of the pituitary is supplied with blood directly from the systemic circulation while the anterior lobe is not. In patients with lymphoma, it is essential to differentiate pituitary involvement in lymphoma from benign lesions for the appropriate therapeutic plan. Local therapy aiming at symptom relief may be beneficial -3.A 70-year-old woman presented with headache, weakness, fatigue, right palpebral ptosis and diplopia. Magnetic resonance imaging (MRI) of the sella revealed a sellar mass involving the pituitary gland and infundibular stalk, which also extended into the right cavernous sinus and the sphenoid sinus. The neurologists demonstrated infiltrations of the right II, III, IV, V and VI cranial nerves figure .A week after occurrence of right palpebral ptosis, the patient received immediate transsphenoidal decompression surgery. The histological examination revealed that the mass was the infiltration of a high-grade B-cell non-Hodgkin's lymphoma involving respiratory system epithelial cells.After the transsphenoidal operation, the patient was transported to our endocrinology clinic. Her skin was pale and she had right palpebral ptosis. No lymphadenopathy or goiter was found. She did not have galactorrhea. Her axillary and pubic hair was scant. Her blood pressure was 120/70 mmHg with a pulse rate of 80 beats/per minute . Examination of the chest, abdomen and cardiovascular system was normal.3, lymphocytes were 1400/mm3, neutrophils were 9660/mm3 and platelet count was 123,000/mm3. The biochemical analysis results of the blood were as follows: glucose: 90 mg/dl, blood urea nitrogen (BUN): 22 mg/dl, creatinine: 0.7 mg/dl, sodium: 141 mEq/L, potassium: 4 mEq/L, serum osmolarity: 295 mOsm/kg H2O, with a paired urine osmolality of 173 mOsm/kg, free T4: 20.4 pmol/L, thyroid stimulating hormone (TSH): 0.705 mIU/L, cortisol at 8 am, 16.7 mcg/dl, adrenocorticotrophic hormone (ACTH) at 8 am, 41 ng/L, luteinizing hormone (LH): 0.1 mIU/ml, follicle-stimulating hormone (FSH): 1.8 mIU/ml, estradiol: 8.1 pg/ml, prolactin (PRL): 102.4 ng/dl. Low levels of urine osmolality after overnight water deprivation along with normal plasma osmolality suggested DI. Desmopressin treatment was not administered because the daily urine volume did not exceed 4 liters (her weight was 56 kg).As per whole blood count, hemoglobin was 12.3 g/dl, hematocrit was 38%, white blood cell count (WBC) was 11,760/mmHyperprolactinemia was attributed to the stalk compression which interrupted dopamine transport to lactotrophs. The low levels of FSH and LH observed in this post-menopausal woman suggested infiltration of the anterior pituitary by lymphoma. Because primary sellar lymphoma is very rare and histological examination of our patient revealed that the mass was due to infiltration of a high-grade B-cell non-Hodgkin's lymphoma involving respiratory system epithelial cells, computed tomography (CT) of the paranasal sinus and MRI of the thorax and abdomen were performed.Although MRI of the thorax and abdomen did not show any abnormalities, when the paranasal sinus CT scan was performed, we concluded that the primary lymphoma originated from the sphenoid sinus and infiltrated the pituitary gland. When bone marrow aspiration was performed, lymphoma infiltration to the bone marrow was detected and the thrombocytopenia was thought to be resulting from lymphoma infiltration of the bone marrow. Chemotherapy and radiotherapy to the sellar area were planned, but the patient died and her family did not permit an autopsy.We have described a patient with non-Hodgkin's lymphoma infiltration of the pituitary gland, who presented with ophthalmoplegia and DI.Sudden onset of DI, ophthalmoplegia and headaches in a patient over 50 years of age should always raise suspicion for lymphoma or leukemia of the pituitary gland or metastasis to the pituitary. The vast majority of central nervous system (CNS) lymphomas are non-Hodgkin's B-cell lymphomas as in our patient, and CNS lymphomas are usually observed in patients with acquired immunodeficiency syndromes .As the pituitary gland has a great reserve capacity, pituitary metastasis and pituitary involvement in lymphoma or leukemia appear to be asymptomatic in most cases . MetastaOccasionally DI is transient, because regeneration of neuro-hypophyseal fibers may occur or intermittent corticotrophin cell insufficiency may obliterate the presence of DI until corticosteroid treatment is instituted. Hypothyroidism and hypoadrenalism are the most frequent types of symptomatic hypopituitarism. Bilateral hemianopsia is the most common type of visual field impairment. Infiltration of the adjacent cavernous sinus usually induces cranial nerve III palsy or less frequently nerve IV palsy; compression of nerve VI is relatively uncommon because it is well sheltered within the cavernous sinus. Facial numbness due to cranial nerve V dysfunction is also rare. Tumor extension to the septum pellucidum or the frontal lobes may result in cognitive deficit or psychiatric symptoms and anosmia. If cranial nerve I is affected, stretching of the diaphragm sella or ventricular distention can give rise to headaches or intracranial hypertension . In our Hyperprolactinemia is attributed to stalk compression; the degree of hyperprolactinemia is important for the differential diagnosis, because PRL levels above 200 ng/ml (9.0 nmol/liter) are generally considered to be indicative of a prolactinoma. The prolactin level of our case was consistent with the prolactin levels observed in the presence of stalk compression ,7.The differential diagnosis must be established with pituitary adenoma and meningioma which are the most frequent tumors of the pituitary gland. Although symptoms of lymphoma or leukemia infiltration of the pituitary gland and metastasis to the pituitary at presentation may resemble pituitary adenomas, DI is reported in only 1% of adenomas ,8.Schubiger and Haller suggested that DI is the most important criterion to differentiate pituitary adenomas from tumor metastasis and infiltration of lymphoma or leukemia to the pituitary .Presentation with hypopituitarism, headaches or visual disturbances is less helpful in the differential diagnosis, being described in infiltration of the pituitary gland with lymphoma, leukemia and metastasis to the pituitary. Ophthalmoplegia may be more helpful in the differential diagnosis, without being pathognomonic for metastasis either ,9.In contrast, presentation with abducens nerve palsy should always raise the suspicion for malignancy. The rapidity of symptom development may also contribute to the diagnosis. Consequently, a rapidly growing sella tumor, especially in patients treated with dopamine agonists or a sudden onset of DI, ophthalmoplegia and headaches in a patient of more than 50 years of age as reported here, strongly suggest lymphoma or leukemia, infiltration of pituitary or metastasis to the pituitary gland [1 and T2 weighted images. The absence of T2 prolongation results from the dense cellularity and high nucleus to cytoplasm ratio of lymphoma, which may help in the differentiation of CNS lymphoma from other brain tumors [Differential diagnosis must be established for the frequent tumors of the sellar area such as pituitary adenoma and meningioma. High resolution CT and MRI are more sensitive for the differential diagnosis. Typical MR findings of CNS lymphoma are mass lesions that are iso-hypointense on Tn tumors ,11.Typically, contrast enhancement is intense and homogeneous in immunocompetent patients, but it is more likely to be non-homogeneous or ring-like in immunocompromised patients . Pituita1 and T2 weighted images of an older patient with immunodeficiency, lymphoma infiltration of the pituitary must be considered. To plan the treatment of lymphoma infiltration of the pituitary gland, it must be differentiated from other sellar lesions.Lymphoma infiltration to the pituitary is difficult to differentiate from pituitary adenoma, meningioma and other sellar lesions. But when iso-hypointense mass lesions are present on TACTH: adrenocorticotrophic hormone; CNS: central nervous system; DI: diabetes insipidus; MRI: magnetic resonance imaging; CT: computed tomography; FSH: follicle-stimulating hormone; LH: luteinizing hormone; PRL: prolactin; TSH: thyroid stimulating hormone; BUN: blood urea nitrogen; WBC: white blood cell count.The authors declare that they have no competing interests.GT was the attending physician of the case and the diagnosis was established by her under the supervision of FA. This case report was written by GT and partly by IK and overviewed by FA.Written informed consent was obtained from the patient's next of kin 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.


Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases. Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. These arA two-year-old male child presented with history of progressive prominence of the right eye for the past three months. On external ocular examination there was an upward and lateral deviation of the right eye with eccentric proptosis. The child was not cooperative for visual assessment. Anterior segment examination of the right eye was unremarkable except for a sluggish pupillary response; however, there was no relative apparent pupillary defect. Fundus evaluation of the right eye showed choroidal folds with normal optic disc. The patient had undergone magnetic resonance imaging (MRI) three months earlier, suggestive of an ovoid-shaped, contrast enhancing lesion measuring 2.59 × 2.06 × 1.86 mm, involving the inferomedial quadrant of the right orbit pushing the globe upward and laterally. The lesion was indistinguishable from the inferior rectus, inferior oblique and medial rectus muscles, however, the optic nerve was normal. Left orbit and brain scans were normal .Subsequent imaging by computed tomography (CT) scan showed increase in the size of the lesion that involved both extraconal and intraconal compartments of the right orbit. The lesion was now engulfing the optic nerve sheath complex .An incision biopsy of the mass was done via inferior transconjunctival approach. Paraffin sections were stained with Hematoxylin-Eosin and Periodic Acid-Schiff stains. Histopathologic examination of the tumor revealed diffuse collection of small malignant round to oval cells . Cells hThe child was subsequently treated with 12 cycles of chemotherapy which included six cycles of vincristine, adriamycin and cyclophosamide alternating with six cycles of ifosfamide and etoposide. This was followed with external beam radiotherapy of 44 Gy dose over 22 fractions. Serial orbital imaging was done every four months, showing a gradual reduction in the mass. Last CT scan done at the end of 30 months of follow-up showed no evidence of local recurrence or residual disease . As a knPNET is a malignancy which usually peaks in adolescents and young adults with no gender bias. They are4Microscopically, primary peripheral PNET is cellular tumor with characteristic small round cells with hyperchromatic nuclei, and a high nuclear–cytoplasmic ratio. Ultrastructural studies by electron microscopy show cytoplasmic filaments and neurosecretory granules. This may2The differential diagnosis of primary peripheral PNET of orbit includes Ewing's sarcoma, lymphoma, neuroblastoma, hemangioblastoma and small cell osteogenic sarcoma. ImmunohiOut of nine previous reported cases of isolated orbital PNET,310 age g3Primary peripheral PNET of the orbit is a rare entity that poses a diagnostic challenge. Imaging and histopathology though supportive do not confirm the diagnosis. Immunocytology helps in the confirmation of diagnosis. Management should be aggressive using multimodality treatment approach given at the appropriate time. These patients should be followed up for life to rule out recurrence, metastasis and treatment-related malignancies.


We report an unusual case of duodenal diaphragm (DD) with “wind-sock” anomaly in a 6-year-old male. The child presented with an acute duodenal obstruction precipitated by multiple pigmented stones completely blocking the duodenum. Congenital duodenal obstruction (CDO) is a common cause of intestinal obstruction in the newborn. In delayed presentations, the duodenum is uninterrupted and the intrinsic obstruction is a membranous diaphragm web with a central aperture. Intra-operatively, duodenum appears normal in cases where the diaphragm has progressed on to a “wind-sock” abnormality.A 6-year-old male child was admitted with a history of bilious vomiting of 3 days duration. The past history was that of an intermittent vomiting (both bilious and non-bilious) since the age of 1 year. The episodes of vomiting usually followed upper abdominal distention with a frequency of 2–3 times per week; also, there was poor weight gain.duodenal ileus that lasted for 8 days. The patient is on regular follow-up and continues to gain weight. Chemical analysis showed stones to be of pigment variety.On examination, the child was of normal intelligence but malnourished, dehydrated, and with deranged serum electrolytes. The patient was evaluated after correcting his dehydration and electrolyte imbalance. Plain x-ray of the abdomen revealed typical “double-bubble sign” with distal intestinal gas in the abdomen. Barium meal follow-through study showed both stomach and duodenum dilated and a streak of contrast going down the duodenum into the small bowel. The size of the duodenum was twice the size of the stomach . Blood sThe diagnosis in CDO is usually easy and, in the majority of cases, this can be established with plain abdominal radiograph and upper gastrointestinal contrast studies. The clasA variety of intrinsic and extrinsic congenital lesions may cause CDO.–5 It mayet al. reported a case of duodenal obstruction secondary to a duodenal diaphragm with a central hole that was obstructed by date seeds.[Nawaz te seeds. In our cThe important lesson to learn from this child's case is that, clinically, low frequency of bilious vomiting, lack of abdominal distention, and atypical abdominal x-ray may contribute to delayed diagnosis. However, better understanding of congenital duodenal membrane behavior may prevent such delay and its sequelae like growth retardation and prolonged hospitalization.


Primary retroperitoneal mucinous cystadenoma is a rare tumor prevailing specifically in female gender. Its histogenesis is still unclear and its diagnosis is mainly based on morphological characteristics.the subject is a 44 years old man presenting an abdominal pain on the right side, with a palpable mass which appeared four months ago. Abdominal ultrasound (echography) revealed a retroperitoneal cystic process, which was successfully resected through laparotomy.Histopathological examination concluded to a mucinous cystadenoma with borderline malignancy foci.After a year of follow-up, no relapse was noticed in this patient.Retroperitoneal mucinous cystadenoma is a rare tumor that should be considered in front of a retroperitoneal cystic process. Several hypotheses may explain the histogenesis of this pathological process.The interest in publishing this case report on primary retroperitoneal mucinous cystadenoma in a male patient lies in the rarity of occurrence of this syndrom in males as compared to females. Primary retroperitoneal mucinous cystadenoma is a benign and rare tumor. Only 47 cases have been reported in the literature worldwide . RetropeThe patient is a moroccan man of North African origin aged 44, with no significant pathological history. He had never drunk alcohol and had smoked only for a while when he was still at school.He was presenting a torsion-like pain on the right side of his abdomen over the last four months and had lost weight in the last few weeks.The clinical examination revealed palpable process, measuring 15 cm (large axis) on the right side. There was no additional noticeable abnormality such as testicular ectopia during the examination.CT scan and abdominal echography showed several retroperitoneal masses, located in the pre-aortic and inter aortocave areas, and measuring between 2 and 5 cm. They had cystic aspect with some heterogenic focuses. Figure Biological analysis  were normal.A tumoral exeresis has been performed.The macroscopic analysis of the resection fragment showed 4 portions, the biggest of them measured 5 × 4 × 3 cm in its wide axis. It was polycyclic, with an elastic consistency, and surrounded by an intact, thin capsule.At the opening: a multilocular cystic aspect with solid zones and endokystic vegetation was disclosed. The inside of the cysts was gelatinous. Figure .Microscopically, the cyst was lined by a single layer of tall columnar epithelium with clear cytoplasm and small nuclei that were basely located in the cells. In another site, the mass was a borderline mucinous tumor similar to that generally observed in the ovary.The cyst was lined by mucinous epithelium with mild to moderate stratification, with mild to moderate cytological atypia; the cystic wall was fairly thin Figure . AlthougThe diagnosis was a primary retroperitoneal mucinous cystadenoma with borderline focuses. There is no relapse throughout a twelve months follow-up period.Retroperitoneal mucinous cystadenoma with borderline focuses is an extremely rare tumor. So far, only 14 cases have been reported in the literature [Radiologically, retroperitoneal mucinous cystadenoma presents a cystic formation, uni or bilocular repressing the organs around .The differential diagnosis with retroperitoneal mucinous cystadenoma is made with lymphangioma, kystic teratoma, lymphocela, urinoma, kystic mesothelioma . The tumMacroscopically, we notice several cysts with different sizes from 6 to 33 cm (mean of 14), surrounded by a thin capsule, with no communication with the neighbouring organs. When opened, the cysts have a gelatinous content, they may be uni or multilocular and contain vegetations.A correct sampling of the resection piece is necessary in order to search for a borderline or a carcinomatous focus .Microscopically, the tumor resembles ovarian mucinous cystic neoplasms. The cyst locules are lined mainly by a single row of mucin-secreting columnar epithelium. The lining epithelium show mild to moderate stratification (not more than 4 layers thick) with mild to moderate cytologic atypia with no evidence of stromal invasion.The immunohistochemitry analysis shows a positive match to CK7 and CK20 antibodies. This is the same profile encounted in ovary mucinous tumors .The histogenesis of this tumor is still unkown. Several theories do exist. For some authors , retropeThe latest hypothesis concerns a metaplasic origin of the cysts: they may develop from a coelomic epithelium . During The management of this tumor requires a large surgical exeresis by a complete enucleation ,8,9, witThe postoperative evolution is generally well . One casRetroperitoneal mucinous cystadenoma is a rare tumor that should be evocated in front of a retroperitoneal cystic process. Several hypotheses may explain its histogenesis. The complete exeresis is recommended, to avoid relapses or a possible evolution to malignant process. Its diagnosis lays on the anatomopathologic examination, requiring a correct sampling, in order not to miss an associated carcinoma.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal.The authors declare that they have no competing interests.All authors were involved in patient's care. AB, AM analyzed and interpreted the patient data regarding the pathological findings of the patient and prepared the manuscript. AB, AM and AJ edit and coordinated the manuscript. All authors read and approved the final manuscript.


Isolated abdominal wall actinomycosis in the presence of an intrauterine contraceptive device (IUCD) is extremely rare and only six such cases have been reported in the literature. We report a case where clinical and radiological examinations revealed a pseudotumor within the anterior abdominal wall. After being lost to follow-up, the patient presented two years later with the enlargement of the mass. The mass including the affected anterior abdominal wall was completely excised. The diagnosis of actinomycosis was established postoperatively by histopathological examination. Further questioning concerning her gynecological history revealed long-term use of the same IUCD. Surgical excision of the actinomycotic pseudotumour and removal of the IUCD followed by antibiotic therapy resulted in the full recovery of the patient. Actinomyces species [Actinomyces is a part of normal flora of the oral cavity, gastrointestinal tract, and the genital tract. Healthy mucosa acts as a barrier to the spread of the organism, but tissues damaged by neoplasm, surgery, trauma, or foreign body allow multiplication and spread of actinomycosis [Actinomycosis is a rare subacute or chronic infectious disease resulting in suppurative and granulomatous inflammation. The disease is caused by anaerobic or facultatively anaerobic, acid-resistant Gram-positive bacteria of  species . In healomycosis , 3. Prolomycosis  and poseomycosis . In thisSurgical exploration was decided on the basis of her medical history, previous histopathological, and the radiological findings. Following an abdominal transverse incision, intraoperative findings revealed a fibrotic mass of 12 × 13 cm within all layers of the anterior abdominal wall  showed an inhomogeneous mass composed of fibrous bands in the subcutaneous tissue in the periumbilical region, 6 × 5 cm in size, with no visceral involvement. An incisional biopsy was performed twice within a month under local anesthesia, where diffuse fibrosis and exudated suppurations were encountered. The histopathological examination indicated active chronic inflammatory changes, fat necrosis, and diffuse myofibroblastic activity corresponding to the diagnosis of an inflammatory pseudotumor. Microbiological examination and bacterial culture did not reveal any convincing findings. After initiating empirical antibiotic therapy with amoxicillin-clavulanic acid per oral, the patients was discharged, however, she was lost to medical follow up. In March 2005, she was readmitted to our clinic with the deterioration of her symptoms. Repeat MRI of the abdomen demonstrated the progression of the pseudotumor which reached a size of approximately 13 cm in diameter within the abdominal wall. nal wall . The omenal wall . Once thActinomyces israelii. Pathologic presentations of actinomycosis include cervicofacial (50% of cases), abdominal (20%), thoracic (20%), and pelvic involvement (15%) [Actinomyces bacteria are considered to be saprophytes in the oral cavity, throughout the gastrointestinal tract and the female genital tract. Actinomycosis is a chronic abscess-forming disease predominantly caused by nt (15%) . The desnt (15%) . There hnt (15%) . Lunca ent (15%)  reportedA. israelii is present in the reproductive tract of 1.6–44% of IUCD users and the incidence rises slightly after two years of IUCD use [ IUCDs have a traumatizing effect on endometrium, causing erosions that, in the presence of preexisting pelvic inflammatory disease or anaerobic infection, create a favorable environment for the development of actinomyces . The preIUCD use . There iIUCD use , 3, 10. Histologically, suppurating abscesses with subsequent necrosis and dense fibrosis are usually seen in actinomycosis. The abscess cavities grow in size and have avascular thick walls. It initially creates dense adhesions with contiguous structures due to extensive fibrosis and in the late stages, can produce internal or external fistulae . BesidesActinomycosis has been called “the most misdiagnosed disease”, and it has been said that “no disease is so often missed by experienced physicians” . ClinicaIt is critical to repair abdominal wall defects with nonabsorbable meshes in the presence of infection. A study by van't Riet et al.  shows thSurgical treatment without antibiotic therapy is not always sufficient to achieve a cure of actinomycosis. When antibiotic therapy is combined with surgery, it is relatively simple to treat, and the cure rate is more than 90% , 10, 21.In conclusion, isolated actinomycosis of the abdominal wall in IUCD users is an extremely rare clinical entity. Preoperative diagnosis is difficult and rarely established. Long-term use of an IUCD represents a risk factor and should be removed in such clinical presentations. The combination of surgery and antibiotic therapy is curative in most of the cases.


The improper fusion of the postero-lateral foramen of the diaphragm was first described by Bochdalek in 1848. The incidence of congenital diaphragmatic hernia varies from1:2000 to 1:5000 live births and Bochdalek hernias (BH) account for 75 to 85% of these. Although it is a well-known entity in neonates, it is occasionally discovered incidentally in adult patients. Until now, a total of around 100 cases of occult asymptomatic Bochdalek hernia have been reported. The symptomatic cases are encountered more rarely. Colon necrosis among the symptomatic cases was reported in some reports. We discuss the present case since we believe it to be, to the best of our knowledge, the first case of a Bochdalek hernia in an adult presenting with caecal perforation and faecal peritonitis and review the published literature about this rare condition. A diaphragmatic hernia may be congenital or secondary to a traumatic rupture of the diaphragm. The incidence of congenital diaphragmatic hernia (CDH) varies from1:2000 to 1:5000 live births . AccordiA 46-year-old male patient presented to our emergency department with a history of generalized abdominal pain of 7 days' duration. The pain had become more localized to the right lower abdomen for the last 2 days. There was a history of constipation lasting for 3 days. There was no vomiting and he did not have any chest or abdominal complaints in the past. There were no known co-morbidities. There was no history of recent trauma or surgery. On physical examination, he was febrile (101 Fahrenheit) and had tachycardia. Abdomen was distended and the liver dullness was obliterated. There was generalized abdominal tenderness in addition to rebound tenderness in the right iliac fossa. The bowel sounds were absent. The haemogram showed leucocytosis (11000/Cu mm). Chest X-ray showed free air under the diaphragm (Fig Although the initial records of diaphragmatic hernia date back as far as the 1690s ,8. The tBochdalek hernia is an uncommon variant of diaphragmatic hernias in adults and symptomatic cases are even rarer. Often, these cases present with chronic chest or abdominal symptoms and rarely present as an emergency like the present case with hollow viscus perforation and peritonitis. We want to emphasize the point that, though rare, diaphragmatic hernias should be kept in mind while considering all possibilities of the differential diagnosis of acute surgical abdomen in an adult, especially when a conventional plain X-ray reveals some abnormal findings.The authors declare that they have no competing interests.AK carried out the surgery, researched the article and drafted the manuscript.VM assisted in the surgery, researched the article and drafted the manuscript.TSR assisted in the surgery, edited and revised the manuscript.SS carried interpreted the imaging studies, edited and revised the manuscript.All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available with the corresponding author for review by Editor-in Chief.


Mediastinal paraganglioms are rare, highly vascularized tumors arising from chromaffin tissue located in the para-aortic ganglia. Tumors tend to invade bordering structures and may also form metastasis. Up to 50% of patients are asymptomatic and diagnosis is incidental. Presenting symptoms are related to catecholamine hypersecretion or to a mass effect. Complete surgical resection remains the standard of care due to malignant potential of the tumor and poor response to chemotherapy or radiation. Strategic location of the tumor in proximity to great vessels, trachea, and recurrent laryngeal nerve poses challenge for the surgeon. We report a case of a 59-year old asymptomatic female who was incidentally diagnosed with a middle mediastinal mass on a positron-emission tomography (PET-CT) scan performed as part of breast cancer surveillance. Complete resection of the tumor was achieved using cardiopulmonary bypass. The patient recovered uneventfully and in a ten-month follow up there is no evidence of recurrence. Ninety percent of chromaffin-cell-originating tumors are located in the adrenal gland and termed pheochromocytomas. The remaining ten percent are extra adrenal and are termed paragangliomas. Paragangliomas appear in the abdomen, pelvis, neck and mediastimun. Mediastinal paraganglioma originate from para-aortic (middle mediatsinum) and para-vertebral (posterior mediatsinum) sympathetic chain ganglia ,2. SimilA 54-year-old woman was referred for resection of an asymptomatic middle mediastinal mass. Her past medical history was remarkable for right breast carcinoma treated with lumpectomy, axillary lymph node dissection, adjuvant chemotherapy and irradiation sixteen years prior to this admission. Six years later a right para-vertebral desmoid tumor was completely resected. One year ago a second right breast carcinoma was diagnosed and completion mastectomy was performed. A positron emission tomography - computerized tomography (PET-CT scan) performed prior to her admission as part of her oncological follow-up revealed a 5-cm middle mediastinal mass with a standardized uptake value (SUV) of 20 Fig . With a Upon admission, the patient denied any symptoms related neither to catecholamine hyper-secretion nor to carcinoid syndrome. Physical examination and routine laboratory results were unremarkable.The operation was performed via a median sternotomy. A soft, highly vascularized, 1.5 × 2.5 × 5.5 cm mass located between the aorta, the superior vena cava and the right atrium was identified. The tumor compressed the right pulmonary artery, and was densely adherent to the aorta and the anterior wall of the trachea with a very rich network of small blood vessels  scintigraphy and PET-CT scan with 18-FDG (18-fluorodeoxyglucose) are used for localization and staging of these tumors when appropriate. CT-guided needle biopsy is not mandatory and, in fact, can be hazardous due to the proximity of the tumor to the great vessels and its intense vascularity. Our patient's history suggested a differential diagnosis for the current mass of either tumor recurrence or a third primary tumor. Therefore preoperative tissue diagnosis was indicated. Needle-biopsy-based tissue diagnosis of paraganglioma is difficult and complete pathological examination including tumor morphology and structure and specific stains are necessary to achieve accurate diagnosis.Paragangliomas have typical imaging characteristics on CT and magnetic resonance imaging (MRI) scans. Paragnagliomas are usually located in the bifurcation of great vessels and show intense and homogeneous enhancement except for necrotic areas that enhanced poorly. MRI images show intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images [Complete resection is the standard of care of paraganglioma, affording the patient with the best chance of cure since these tumors are relatively resistant to chemotherapy and irradiation . The higThe two major concerns involving resection of mediastinal paragngliomas include intraoperative bleeding and catecholamine crises in patients with metabolically active tumors. The highly vascular nature of the tumor, the proximity and invasion to the great arteries and the systemic anticoagulation necessary for cardiopulmonary bypass - all contribute to a high risk of bleeding. . HormonaPrognosis after complete resection is favorable. Lamy et al reported a follow up of 79 patients with middle mediastinal paragangliomas over a period of 180 months. Among these patients overall survival was 62.0%, mean survival time was 98.2 +/- 11.7 months (mean +/- standard error). For patient undergoing complete resection survival was 84.6%, mean survival time was 125.7 +/- 18.7 months (mean +/- standard error). For patient undergoing incomplete resection survival was 50.0% mean survival time was 71.5 +/- 13.8 months (mean +/- standard error) .In summary, paragnagliomas should be included in the differential diagnosis of a middle mediastinal mass. Complete surgical resection remains the standard of care and is associated with excellent survival. Life-long surveillance for local recurrence and metastatic spread is mandatory.The authors declare that they have no competing interests.OW reviewed the literature and wrote the manuscript, UI and OMS operated on the patient and edited the manuscript. IM took part in operation, and prepared the figures. All authors read and approved the final manuscript.


Primary neoplasms of the mesentery are very rare. They are usually of mesenchymal origin and include desmoid tumor, lipoma, liposarcoma, and fibrosarcoma. Metastatic carcinomas and lymphoma are more common. We report a rare case of localized mesenteric Castleman's disease, presenting as intestinal obstruction. Clinical and radiological findings were suspicious for lymphoma. Localized mesenteric Castleman's disease, though rare, has to be considered in the differential diagnosis of mesenteric tumors, particularly in the young and in the absence of history for other tumor, an abnormal blood picture, or splenomegaly. Primary neoplasms of the mesentery are very rare. They are usually of mesenchymal origin and include desmoid tumor, lipoma, liposarcoma, and fibrosarcoma. Metastatic carcinomas and lymphoma are more common. We report a rare case of localized mesenteric Castleman's disease, presenting as intestinal obstruction. Clinical and radiological findings were suspicious for lymphoma. Localized mesenteric Castleman's disease, though rare, has to be considered in the differential diagnosis of mesenteric tumors, particularly in the young and in the absence of history for other tumor, an abnormal blood picture, or splenomegaly.A thirty-three year old female presented with recurrent symptoms of small intestinal obstruction of chronic onset and progressive course. The patient's medical history was non contributory. Her blood picture was unremarkable. CT scan showed intussusception associated with multiple mesenteric lymphadenopathies, measuring 1.8 cm in maximum dimension. Mediastinal lymphadenopathy and organomegaly were absent. The clinical impression was suspicious for lymphoma.Laporotomy was performed which showed increased caliber and thickening of the small intestinal loops, particularly at the ileum over an area of about 90-100 cm. In addition, several enlarged mesenteric lymph nodes were present. An excisional biopsy from the nodes and a full thickness wedge excision from the abnormal area in the ileum were performed.A piece of adipose tissue containing two lymph nodes measuring 2.1 × 1.5 × 1 cm and 1 × 1 × 1 cm and a small intestinal wedge measuring 4.5 cm long are received fresh. The lymph nodes are submitted according to the lymphoma protocol in our institute. Two touch preparations are prepared from the cut surface. The larger node is trisected and the smaller is bisected, with one section of each is submitted for flow cytometry assay. A tiny piece from the larger node is sent to microbiology. The rest of the tissue is submitted for formalin fixation. The intestinal wedge is opened and showed an intact, unremarkable wall. Representative sections are taken.Microscopic examination shows the lymph nodes to have preserved architecture, but the lymphoid follicles are atrophic and possess a thickened mantle with prominent onion skin arrangement. Few follicles are traversed by a thick hyalinised blood vessel imparting a lollipop like appearance Fig. . The intA diagnosis of early mesenteric Castleman's disease is made. The patient received no further treatment, and during the one year follow-up she is well.Castleman's disease (giant lymph node hyperplasia or angiofollicular lymphoid hyperplasia) is a rare lymphoproliferative disease of unknown origin, first described by Castleman's in 1954 [Castleman's disease can be divided into two further forms: the most common localized-solitary form and the less usual is the multicentric form. Approximately 80% of the cases of the solitary form belong to the hyaline-vascular type and the remaining 20% to the plasma cell type.The usual location of the solitary mass is the mediastinum (70%), whereas the mesentery is very rarely involved. The hyaline vascular type predominates in the thorax and is rare in the mesentery . The enlMost Castleman's disease lesions appear as nonspecific, well-defined hypoechoic masses on sonography. Sonography remains useful for the evaluation of cervical and axillary Castleman's diseases, in which the depiction of prominent peripheral vessels and penetrating feeding vessels on Doppler sonograms can suggest the diagnosis of this uncommon disease ,8. On CTCastleman's disease has to be included in the differential diagnosis of every mass located in the mesentery, especially when the lesion shows the imaging features mentioned above, and if the patient is young, and if relevant medical history, an abnormal blood picture, and organomegaly are absent.The authors declare that they have no competing interests.DE: wrote the manuscript.CH: made the corrections as for the reviewer's comments.FT: the case was originally diagnosed by FT and reviewed by DE through intra-departmental review.AN: took the pictures and wrote legends.SA: revised and provided the publication fees.


Abdominal pregnancy is a rare event, but one that represents a grave risk to the health of the pregnant woman. An abdominal pregnancy is defined as an ectopic pregnancy that implants in the peritoneal cavity. Early abdominal pregnancy is self-limited by hemorrhage from trophoblastic invasion with complete abortion of the gestational sac that leaves a discrete crater. Advanced abdominal pregnancy is a rare event, with high fetal and maternal morbidity and mortality.This is a case report of a 22-year-old primigravida with an abdominal pregnancy from a ruptured rudimentary horn. She was diagnosed as a case of term pregnancy with placenta previa with a transverse fetal lie and cervical fibroid and was prepared for an elective cesarean section. Intra-operatively, a live term female baby was extracted from the peritoneal cavity and it turned out to be an abdominal pregnancy from a ruptured rudimentary horn of a unicornuate uterus, which is a very rare condition. Mother and baby were in good condition after such a catastrophic event.This case illustrates a rare obstetric condition which can be a severe catastrophic condition leading to maternal mortality and morbidity. It is imperative for every obstetrician to have in mind the possibility of abdominal pregnancy, although rare, especially in pregnant patients with persistent abdominal pain and painful fetal movements. An abdominal pregnancy is defined as an ectopic pregnancy that implants in the peritoneal cavity. Early abdominal pregnancy is self-limited by hemorrhage from trophoblastic invasion with complete abortion of the gestational sac that leaves a discrete crater. Advanced abdominal pregnancy is a rare event, with high fetal and maternal morbidity and mortality. It still remains a diagnostic and therapeutic challenge for every obstetrician and usually occurs after tubal abortion or rupture. Very rarely, it occurs following rupture of a rudimentary horn. We report a rare case of a term viable abdominal pregnancy following rupture of a rudimentary horn.A 22-year-old primigravida presented to the obstetrics department at 22 weeks gestation with a painful abdomen of 10 days duration. Her early pregnancy was uneventful and ultrasound examination had not been performed in the first trimester. On examination, her vital signs were stable and tenderness was present in the right iliac fossa and right lumbar region. The height of the uterus corresponded to 28 weeks gestation. Ultrasound showed a fetus of 22 weeks with placenta previa and cervical fibroid. Amniotic fluid was normal. Surgical causes of a painful abdomen were ruled out. The patient was managed conservatively with analgesics and antibiotics and discharged after her pain had subsided. Repeat ultrasound before discharge revealed the same finding. She was lost to follow-up and presented to the outpatient department at 40 weeks of gestation with no complaints for the rest of the antenatal period except for painful fetal movements. It was planned to perform an elective cesarean section for central placenta previa with transverse lie and cervical fibroid. Intra-operatively, as the abdomen was opened, the fetus along with the placenta were found lying in the abdominal cavity and with the left horn of the uterus seen separately lower down in the pelvis. A live term female baby of 3 kg was delivered with good Apgar score. The placenta was attached in part to the ruptured right rudimentary horn deriving its blood supply from it and part of it was attached to the layers of the peritoneum. As the placenta could not be separated from the right rudimentary horn, the placenta along with rudimentary horn and right fallopian tube were removed. The left tube and both ovaries were normal. One pint of blood was transfused. The postoperative period was uneventful and the mother and child were discharged in good condition. The histopathology report showed chorionic villi attached to bundles of smooth muscle of uterine cornu, as shown in Figure The incidence of abdominal pregnancy is 1 in 10,000 live births, whereas advanced abdominal pregnancy is encountered in 1 in 25,000 births [Diagnosis of advanced abdominal pregnancy requires a high index of suspicion. History and physical examination are often inconclusive. Our patient presented only with complaints of painful fetal movements and physical examination showed a transverse fetal lie and closed uneffaced cervix. She had transient unexplained anemia at the time she was in our hospital at 22 weeks for painful abdomen, probably due to rupture of the rudimentary horn. In spite of considerable improvement in technical abilities, absolute diagnosis by ultrasound is missed in half of the cases ,3. The fIn this patient, the intra-operative findings were indicative of unicornuate uterus with a non-communicating type of rudimentary horn which could have probably ruptured at the time when she presented with painful abdomen, transient anemia and fluid in the right Morrison's pouch. She fortunately continued the pregnancy until term without significant hemorrhage. Maternal deaths associated with abdominal pregnancy result from hemorrhage after inadvertent dislodgement of the placenta. In our patient, part of the placenta was attached to the ruptured rudimentary horn and but most of it lay in the peritoneal cavity attaching itself to the peritoneal layers. It was possible to remove the whole of the placenta along with the rudimentary horn to which it was attached without significant hemorrhage. Removal of the entire placenta has been recommended but if significant hemorrhage occurs, it is safer to leave all or part of the placenta and allow it to reabsorb slowly. If hemorrhage is intractable, ligation of feeding vessels may be attempted. Cases have been reported where hemorrhage was controlled using a medical antigravity suit .et al. [In a case report by Desai et al. , an initIn three cases reported by Sandberg and Pelligra , the diaet al. [In a case report by Harris et al. , the diaThe delay in diagnosis is mainly due to difficulties in the clinical assessment caused by variance in presentation. A careful examination of the uterine contour in every case may help to avoid misdiagnosis of such a rare and potentially catastrophic presentation.in situ and to expect spontaneous resorption.The presentation of a pregnant woman with an unusual clinical picture, especially with persistent or recurrent abdominal pain in association with painful fetal movements or intrauterine fetal death, should alert the obstetrician to the possibility of abdominal pregnancy. Expertly performed and interpreted ultrasonography may be the definitive diagnostic technique. It is imperative to consider this diagnosis in the case of such patients and, once discovered, to initiate prompt treatment. Finally, if the entire placental blood supply cannot be ligated, it appears prudent to leave the abdominal placenta 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.BA and SD were responsible for the concept, TS wrote the paper, and the manuscript was reviewed and edited by BA and BP. Histopathological confirmation was done by RS. All authors approved the final version.


Handlebar hernias are abdominal wall hernias resulting from direct trauma to the anterior abdominal wall. They usually result at weak anatomic locations of the abdominal wall. Such traumatic hernias are rare, requiring a high index of suspicion for a clinical diagnosis. We report the case of a handlebar hernia resulting from an injury sustained during a vehicular injury, and discuss the management of such injuries. We report a case of such hernia associated with significant intraabdominal injury. A 56-year-old male presented with severe blunt abdominal trauma caused by the gear-poke of a car. There was a defect in the anterior abdominal wall associated with mesenteric tear and a gangrenous segment of the ileum. The gangrenous bowel was resected with primary end-to-end anastomosis and the hernia was repaired in layers. A high index of clinical suspicion is required to diagnose such a type of hernia. Traumatic hernia of the anterior abdominal wall is rare; handlebar hernia is an example of traumatic hernia. It was first described by Dimyan et al. in 1980,A 56-year-old male presented to the emergency room 4h after a motor car accident during which his right lower quadrant sustained a direct hit by the gear-poke of a car. There was no history of previous abdominal wall hernias. The vital signs were stable. Abdominal examination revealed tender irreducible soft tissue swelling in the right iliac fossa with superficial ecchymosis and positive cough impulse. Muscular guarding, tenderness and rebound tenderness was observed all over the abdomen. Bowel sounds were absent. There were no associated injuries and the rest of the physical examination did not reveal any abnormality. The laboratory investigations were within the normal limits. The radiological examination of the cervical spine, chest and abdomen were normal. Computed tomography (CT) of the abdomen showed a defect in the anterior abdominal wall muscles in the right iliac fossa with bowel loops herniating through the defect .Exploratory laparotomy was performed. There was about 200 ml of free blood in the peritoneal cavity and transverse tear of the mid-ileal mesentery causing a gangrene of approximately 20 cm of the bowel. There was also a defect of approximately 8 × 5 cm involving all the layers of the anterior abdominal wall muscles in the right iliac fossa with intact skin. Lacerated muscles were present at the edges of the defect. Active mesenteric bleeding vessels were ligated. The devascularized segment of ileum was resected and end-to-end anastomosis was performed. The mesenteric defect was closed. No other intraabdominal injuries were found. The abdominal wall defect was repaired in layers. The muscular and fascial defects were closed in the anatomic layers. The abdominal incision was closed in a standard manner. Postoperative course was uneventful. Follow up after 2 years showed no evidence of recurrence.Traumatic abdominal wall hernias are produced by the direct blunt trauma from an object that has insufficient force to penetrate the skin, yet able to disrupt the deeper tissues of muscles and fascia. This is possible because the skin is more elastic than the rest of the layers. There ar13The diagnosis is usually made on the basis of history and physical examination. However, ultrasonography and CT scans may be helpful in difficult cases.6 In rareWith the exception of the rare case of thoracic handlebar hernia, all othe2The majority of the cases described with handlebar hernias were caused by low-energy mechanisms such as bicycle or motorcycle. Therefore, in most of the cases, there was no significant intraabdominal injury.45 In few43vs. midline exploratory laparotomy to rule out the intraabdominal injuries.[After dealing with all associated intraabdominal injuries, definitive treatment of these hernias mandates surgical exploration and prompt repair to prevent incarceration or strangulation. This repair can be performed with primary closure if the tissue allows or with prosthetic material if the defect is too large.11 Debateinjuries. AssociatIn summary, a high level of clinical suspicion for traumatic hernia is required in cases of severe abdominal wall injury. Such patients should be examined and treated properly for the possibility of significant intraabdominal injury.