Müllerian duct cysts result from an abnormality in regression of the Müllerian system. They may occasionally give rise to symptoms. We report an unusual case of acute urinary retention in an old man caused by a giant Müllerian duct cyst.A 77-year-old man presented with of acute urinary retention. After bladder drainage, digital rectal examination found a large soft supraprostatic mass. Transrectal ultrasound and computed tomography scans revealed a large multilocular retrovesical cystic mass. The patient underwent open surgical resection of the cyst. Histologically, the cystic lesion was lined with stratified cubocolumnar cells, consistent with a Müllerian duct cyst.Acute urinary retention in the elderly is not always related to prostatic diseases. Other causes, even congenital ones, may be involved Müllerian duct cysts result from an abnormality in regression of the Müllerian system . This coA 77-year-old Tunisian man of North African origin was admitted to our hospital because of acute urinary retention. He has a 5-month history of urinary frequency and dysuria. Initial physical examination revealed a distended urinary bladder from which 600 ml of clear urine was obtained by catheterization. Digital rectal examination revealed an enlarged, nontender, non-nodular prostate and a large soft supraprostatic mass in the midline. Transrectal ultrasound scan (TRUS) showed a huge retrovesical cyst. TRUS guided needle aspiration of the cyst obtained clear yellowish fluid. The cyst fluid contained no sperm, no malignant cells and was sterile on culture.Computed tomography (CT) revealed a large multilocular retrovesical cystic mass in the pelvis and abdomen, with no enhancement after administration of intravenous contrast medium Figure . The cysSurgical exploration was done through a midline infraumbilical incision with a transperitoneal approach. Intraoperatively, there was an enormous cyst arising from the retrovesical space Figure . The cysPostoperative course was uneventful and the patient regains normal voiding function. Histologically, the cystic lesion was lined with stratified cubocolumnar cells, consistent with a Müllerian duct cyst.Müllerian duct remnants, which include Müllerian duct cysts and enlarged prostatic utricles, are midline prostatic cystic lesions . In the Prostatic utricle cysts represent a distinct entity that results from dilatation of the prostatic utricle. These midline masses are typically smaller and communicate with the posterior urethra. They develop in the first or second decade of life and are associated with various genitourinary abnormalities, including hypospadias ,3.Most Müllerian duct cysts are asymptomatic but they may present with irritative urinary symptoms , obstructive symptoms , hematuria, hematospermia, bloody urethral discharge, ejaculatory pain, urinary tract infection, epidydimitis, infertility or constipation ,3,7. MalAcute urinary retention is also a clinical presentation described in children  or adultLearning from this new case report is that acute urinary retention in the elderly is not always related to prostatic diseases. Other causes, even congenital ones, may be involved.Transrectal ultrasound, CT, and magnetic resonance scanning are the most useful diagnostic tools ,7,9. DifMüllerian cysts are not so rare and they are probably underreported . As theyWritten 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.MJ and AH collected the data and literature review, and wrote the manuscript. AS and WH revised and provided comments on the manuscript. NBS and FM were the attending doctors, carried out the surgical procedure and literature review. All authors read and approved the final manuscript.


The primary mucinous adenocarcinoma of the bladder is an extremely rare urologic entity, which is found in less than 2% of all urinary bladder tumours and is often presented as metastatic.A 69-year old male patient was diagnosed with a primary mucinous adenocarcinoma of the bladder after undergoing a transurethral resection of a bladder tumour and complete examination of the entire gastrointestinal tract to rule out other primary cites. Immunohistochemistry confirmed the nature of the tumour. The patient underwent a radical cystoprostatectomy with en block bilateral pelvic lymphadenectomy and urinary diversion with a Bricker ileostomy.The primary adenocarcinoma creates a diagnostic dilemma, since it cannot be easily differentiated by the adenocarcinoma that originates from the colon and the prostate. We advocate the radical surgical management, after exclusion of any primary malignant sites related to the gastrointestinal tract. The immunohistochemistry has a leading role, assisting with the differential diagnosis. Urinary bladder cancer is the second most frequent tumour of the genitourinary tract . AdenocaA 69-year old male patient was admitted having gross painless hematuria for the last 2 months with no other comorbidities, apart from benign prostatic hyperplasia treated with a-blockers. Ultrasound of the kidneys, the bladder and the prostate showed an exophytic lesion of the bladder and dilatation of the left pelvicaliceal system. Intravenous urography (IVU) showed a radiolucent filling defect in the bladder and a non functioning left kidney Fig. . The nexThe specimen of radical cystoprostatectomy included the urinary bladder with pericystic fatty tissue and the prostate gland. On section, a tumour was identified, measuring in the greatest dimension 3 cm. The tumour was localized in the posterior bladder wall and had an exophytic growth pattern with solid (nodular) appearance. It seemed to invade the wall of the bladder, extending to the proximal urethral margin of the prostate.The grossly described tumour is a primary mucinous adenocarcinoma of the urinary bladder, which invades the wall of the bladder, both lobes of the prostate gland and both seminal vesicles. We did not recognize normal urothelium with intestinal metaplasia. The carcinoma includes glandular configurations, having one cell layer of cuboidal or columnar epithelium with large, dark nuclei, signet-ring cells Fig. , nuclearThe majority of primary adenocarcinomas of the urinary bladder (50-60%) arise at the bladder base and almost all of the remaining are associated with urachal remnants . The malHematuria is the most common presenting sign, manifested in about 90% of patients. Almost half of the patients complain about dysuria, nocturia, frequency and pain. Cystoscopically, bladder adenocarcinomas ordinarily appear as single, nodular tumours that can not be reliably distinguished from urothelial neoplasms.Adenocarcinomas of the urinary bladder, regardless of site, include the following histologic variations: 1) Adenocarcinoma non otherwise specified, 2) Adenocarcinoma of enteric type, 3) Adenocarcinoma with signet-ring cells, 4) Mucinous adenocarcinoma, 5) Clear cell adenocarcinoma, 6) Hepatoid adenocarcinoma, 7) Mixed adenocarcinoma [Mucinous adenocarcinoma of the urinary bladder includes large lakes of extracellular mucin mixed with collections of tumour cells. By definition, these mucinous foci should constitute at least half of the tumour mass. In some cases, there is an admixture of extracellular and intracellular mucin; the latter is resulting in signet ring configuration .Regarding immunohistochemistry, adenocarcinoma of the urinary bladder expresses CEA, CDX-2, MUC-1, MUC-2 and MUC-3, same as colonic adenocarcinoma. Cytokeratins 7 and 20 are positive, in contrast with colonic adenocarcinoma that expresses cytokeratin 20 but not cytokeratin 7 [The differential diagnosis includes metastatic colonic adenocarcinoma, urothelial neoplasms with glandular differentiation, intestinal metaplasia and nephrogenic metaplasia. Metastatic adenocarcinoma is differentiated using the immunophenotype (CK7 negative and CK 20 positive). Urothelial neoplasm with glandular differentiation may contain intracellular and luminal mucins; however, mucins are not abundant. In addition, in this type of carcinoma, signet-ring cells are not prominent and the "glands" are surrounded by pseudostratified epithelium. Intestinal metaplasia may infiltrate the lamina propria or even the bladder wall. Mucinous lakes are not uncommon in these cases and their presence in a tissue sample is diagnostic of adenocarcinoma only with the presence of neoplastic cells. The cells of intestinal metaplasia lack nuclear anaplasia and rarely involve the muscularis propria. Nodular areas of cystitis glandularis rich in goblet cells should be considered benign, even if the nodules extend into the lamina propria.Prognosis varies with stage, with survival approaching 75-100% among patients whose tumours are confined to the urinary bladder. Unfortunately, low-stage cancers account for fewer than 30% of reported cases . PatientWritten 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.KS gathered patient data. SIT gathered patient data, drafted and revised the manuscript. ET performed the immunohistochemical study and drafted the manuscript. IK drafted the manuscript and gathered reference articles. NK performed the immunohistochemical study and supervised the manuscript. KGS performed the surgical operation and supervised the manuscript.All authors read and approved the final manuscript.


Sir,A 35-year-old male patient reported to the surgeon with complaints of repeated attacks of pain in abdomen, associated with nausea and vomiting. He had clinical evidence of acute appendicitis. The patient was operated and an appendicectomy was done. The specimen was sent for histopathological examination.Grossly, the appendix measured 6 × 1.2 × 1 cm and showed a bulbous, globular and enlarged tip. The cut section showed localized thickened wall at the tip.Microscopic examination revealed mucosa with villous and papillary infoldings, mostly covered by a single layer of tall columnar mucous-producing cells Figures  and 2. SAppendiceal tumors account for less than 0.4% of neoplasms in the gastrointestinal tract and are found in less than 1% of appendectomies.1 Adenoma 1Villous adenoma of the appendix is a rare pathological condition.5 AdenomaAppendiceal adenomas are diffuse villous lesions involving large areas of the appendiceal mucosa. They frequently have a villous configuration with papillary fronds covered by large number of mucus-producing cells, which often have a deceptively innocuous appearance. The dysplastic nature of the lesion is best appreciated by examining the epithelial characteristics in the basal half of the mucosa, where crowding and stratification of the cells with nuclear atypia will be appreciated. Areas with a serrated pattern may be seen. Excessive mucous production by the lining epithelial cells may occur, in which case a mucocoele of the appendix may form.Adenomas of the appendix may be cured by appendicectomy,3 provide


Chordoma is a rare malignant tumour with an incidence of metastasis of less than 10 percent. Usually arising from clivus its posterior extension may involve the brainstem before presenting as nasal mass and obstruction. Surgery is the main mode of treatment with adjuvant radiotherapy. However surgery is rarely possible for a large intracranial lesion.We report the case of an adolescent patient with a chordoma extending posteriorly to the brainstem and anteriorly to the nasopharynx and managed by the combination of resection using a maxillomandibular swing approach and the use of a neurosurgical ultrasonic aspirator.Maxillomandibular swing approach provides good access for large nasopharyngeal tumour extending brainstem area. Chordomas are comparatively slow growing malignant neoplasms derived from notochord. They can present anywhere from skull base to sacrum. In the cranial region the tumours usually arises from the clivus. Clival chordomas usually present in the third and fourth decades of life and there is slight male preponderance . ChordomSkull base chordomas usually extend posteriorly from the clivus to the sella turcica and brain stem . AlthougA 17 year old boy presented with progressively worsening right-sided nasal blockage of one year duration, with two episodes of epistaxis and deterioration of vision. On examination there was a mass in the right nasal cavity extending across the postnasal space on either side. Fundoscopy revealed signs of left eye compression and optic neuropathy. An initial differential diagnosis was of chordoma, angiofibroma, pyogenic granuloma and nasopharyngeal carcinoma.CT (Computed Tomography) scan showed a hypodense mass in the nasopharynx entering into the sellar and parasellar regions with erosion of the pituitary fossa, clivus and middle cranial fossa. An MRI (Magnetic Resonance Imaging) brain sequence showed a large clival based tumour with involvement of the dura at the region of the whole clivus where the tumour had compressed the brain stem anteriorly Fig. . CerebraTumour excision was via left maxillary swing, left mandibular swing and midline tongue split followed by reassembly and filling of the defect utilizing rectus abdominus muscle. First a left maxillary swing approach via a Weber-Fergusson-Longmire incision was performed. The maxilla was swung laterally based on a cheek flap. As the access to the tumour, which was extending from skull base to first cervical vertebra, was not adequate with this approach alone, a paramedian mandibulotomy facilitating a left mandibullar swing was also performed. However a massive tongue occupying the mid part of the dissection did not allow free instrumentation. A temporary midline glossotomy was carried out to provide more exposure. This ultimately gave wide access to the whole clival mass.The chordoma was resected using an ultrasonic aspirator set at a high frequency mode of 100 Hertz which is typically used in meningioma resections. The tip of the Dissectron ultrasonic aspirator  was calibrated with the Omnisight Image Guided System  so that precise removal could be performed without injury of the structures within the dural space i.e. brainstem, basilar artery and pituitary gland. Despite this there was a small dural tear at the level of the upper clivus where invasion was maximum. At the end of the surgical procedure, the defect was filled with a free rectus abdominis muscle transfer. Despite this a CSF  fistula developed and was managed by external ventricular drainage which was converted to a low pressure ventricular peritoneal shunt fourteen days later. The CSF leak stopped two days two days after surgery and acetazolamide was continued for duration of 5 days.Chordomas are lobulated and apparently capsulated tumours which arise from notochord and derive from ectoderm. Mainly seen in the sacrococygeal region, they may arise from the spheno-occipital region and protrude into the nasopharynx. While plain X-rays may show tumour with destruction of the clivus, CT-scan and MRI are essential assessment tools in delineating the gross margins of a chordoma . MorpholWhile radical surgical resection is the treatment of choice, this is rarely possible due to intracranial extension. There have been reports of using an endoscopic approach but in cases with extensive dural invasion, inferior clivus-centred tumours and large tumours extended to the occipital condyle, an open external approach is preferred . RadiothOur patient was an adolescent boy with extensive chordoma and we faced similar problems if we were to approach the tumour transcranially. Furthermore the proximity of vital structures such as internal carotid arteries and cranial nerves made this surgical approach difficult and very challenging. However we succeeded in approaching the tumour via a maxillomandibular swing with a tongue split procedure. This procedure provided adequate exposure for surgical resection of the tumour mass utilizing an ultrasonic aspirator to remove tumour which was abutting brainstem without any residual central nervous system deficit. The advantage of the use of an ultrasonic aspirator is its ability to remove tumour without causing damage to nearby blood vessels. Radiotherapy was subsequently administered and the patient is regularly followed-up and has been in sound health for three years from the time of surgery.Excision of an extensive chordoma with minimal complications and a good prognosis is possible utilizing an upper & lower jaw double swing approach in association with neurosurgical expertise.CSF – cerebrospinal fluidEMA – epithelial membrane antigenCT – Computed TomographyMRI – Magnetic Resonance ImagingThe author(s) declare that they have no competing interests.SH: Drafted the overall design of the manuscript, the otolaryngology management part and collect all the figures and photos. JMA: Drafted the neurosurgical part of the management. SJWD: Drafted the clinical presentation and acquired references. ZI: Help in drafting the overall manuscript and performed final review. All authors have read and approved the final manuscript.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.


Dear Sir,We have read the article on high-resolution USG of the anterior abdominal wall by Sudheer Gokhale. He has g2The first patient, a 28-year-old man, presented with a swelling on the left side of the anterior abdominal wall that had been present for 1 month. Clinically, it was diagnosed as a lipoma or neurofibroma. The patient was sent for a high-resolution USG study, which revealed a well-defined cystic lesion with an echogenic nidus in the subcutaneous tissue in the area of the swelling . Based oThe second patient was a 35-year-old man presenting with a painful swelling on the right side of the anterior abdominal wall for 8-10 days. On examination, the swelling was tender and hard and the skin overlying the swelling was inflamed. There was no history of fever or trauma. Clinically, it was diagnosed as an abscess. On high-resolution USG, there was a small cyst of size 3 mm, with a surrounding 15 × 22-mm hypoechoic area in the left rectus abdominis muscle . Based oIn both these cases no further investigations were done. The patients recovered fully after treatment with albendazole and corticosteroids.High-resolution USG plays an important role in establishing the diagnosis of muscular and subcutaneous cysticercosis. The salient diagnostic feature of a cysticercus granuloma is the presence of an oval or rounded well-defined cystic lesion, with an eccentric echogenic nidus within. If this picture is seen in a subcutaneous or intramuscular swelling, the diagnosis of cysticercosis can be made with great confidence and no further investigations are required;3 biopsy


With the 2005 WHO classification of salivary gland tumours and its increasingly recognized diagnostic entities, the frequency of adenocarcinoma (NOS) has decreased significantly.This paper describes a fast growing adenocarcinoma (NOS), originating from the minor salivary glands of the left buccal mucosa with a rapid onset of multiple local and distant metastases, especially in the lung. A lung primary was unlikely as the tumour was characterized by positivity for cytokeratin 20 and negativity for the thyroid transcription factor-1 protein (TTF-1) in immunohistochemistry.A rare case of an adenocarcinoma (NOS) of the minor salivary glands with a rapid development and an unfavourable clinical course is reported. It shows that additional immunohistochemical analysis can decisively contribute to determine the site of the primary tumour in cases with unknown primary. Epithelial tumours arising in the intra-oral minor salivary glands account for 9–23% of all salivary gland tumours ,2 and ofA 68-year old female patient with a painless swelling of the left buccal mucosa was referred to our department. An initial incisional biopsy of the lesion was inconclusive and magnetic resonance imaging (MRI) of the head and neck diagnosed a benign appearing connective tissue tumour, arising without local invasion.Detailed medical history pointed to a more than three months consisting, rapidly enlarging mass in the patient's left buccal mucosa, which provoked pain while using her dentures. The patient further complained of lack of appetite, sleeping disturbance and weight loss of 11 kilograms (15% of body weight) over the last five months. Tobacco and alcohol abuse was excluded.Intraoral examination revealed an asymptomatic, solid, firm, exophytic and endophytic growing tumour of the left buccal mucosa Fig . The tumHistopathological analysis of the specimen, supported by immunohistochemistry  allowed the diagnosis of a poorly differentiated adenocarcinoma (NOS) of the minor salivary glands Fig .Positron-emission tomography with 'low dose CT' (PET-CT), computerised tomography  and bone scan showed the tumour in the left buccal area and an additional circular mass in the hilum of the left lung, a tumour of the left kidney, as well as multiple pulmonary, cervical lymph nodes and osseous  masses Fig , 4, 5, 6Due to the extent of the disease, palliative chemotherapy was initiated.Data concerning the relative frequency of adenocarcinoma (NOS) vary from 1.2% to 17.8% of all salivary gland carcinomas ,8, sinceIn general there are various differential diagnoses for a buccal swelling comprising both benign and malignant neoplasia. Tumours may originate from the squamous epithelium , the soft tissue  and from salivary glands  ,10. In vMicroscopically, adenocarcinoma (NOS) is characterized by a variable spectrum of different architectural patterns, which may include glandular, papillary, cystic, cribriform or solid structures . TumoursCytokeratins (CK) are distinctive intermediate filaments, which are confined to epithelia and indicate the tissue of origin in malignant transformation and metastasis . They maThe overall prognosis of adenocarcinoma (NOS) depends on clinical stage and malignancy grade. For stage I a 10-year survival rate of 75% has been reported by Spiro et al , droppinAlthough incidence of the adenocarcinoma (NOS) is decreasing with the establishment of new neoplastic entities of the salivary glands, this carcinoma still occurs and should be taken into account in case of intraoral mucosal tumours with indiscernible borders. High-grade malignancies arising in the minor glands may show a rapid growth and early metastases to lymph nodes and distant organs. Additional immunohistochemical analysis can decisively contribute to determine the site of the primary tumour.The authors declare that they have no competing interests.TE drafted the manuscript. JK helped to the critical review of the article. RM helped to the critical review of the article. SS performed the histopathological investigations. TER helped to the critical review of the manuscript. OD performed the surgical procedure, helped to draft the manuscript, helped to the critical review of the manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-In-Chief of this journal.


Retroperitoneal enterogenous cysts are uncommon and adenocarcinoma within such cysts is a rare complication.We present the third described case of a retroperitoneal enterogenous cyst with adenocarcinomatous changes and only the second reported case whereby the cyst was not arising from any anatomical structure.This case demonstrates the difficulties in making a diagnosis as well as the importance of a multi-disciplinary approach, and raises further questions regarding post-operative treatment with chemotherapy. We present a patient with a retroperitoneal enterogenous cyst that contained adenocarcinoma cells presenting in adulthood. To date, only 3 cases have been reported of retroperitoneal enterogenous cysts -3 and onIn 2002, a 54 year old man presented with mild heart-burn. Incidental "hepatomegaly" was found on examination accompanied by a slightly elevated bilirubin but otherwise normal liver function. A computerized tomography (CT) scan reported a well defined cyst arising from the right lower pole of the liver, measuring 14 × 12 × 10 cm  are rare. Their presence have been described in the pericardium , centralet al., [Dardik et al.,  describeet al., ,10. The The embryogenesis of enterogenous cysts have included intra-uterine volvulus resulting in ischaemia and infarction, persistence of an intra-uterine diverticulum and failure of complete vacuolisation of the solid alimentary tract -4. This The two CT scans  initially reported an intimate relationship of the cyst with the liver. Initially, radiographic and clinical signs suggested a simple benign liver cyst resulting in the patient's discharge. However, at surgery 4 years later, the cyst did not appear to be arising from any organ and retrospective review of the CT scans concurred with this finding. Previously, enterogenous cysts have been defined as arising from a gastro-intestinal organ, which mDuplication cysts of the gastro-intestinal tract, specifically arising from the rectum, have been described with in-situ adenocarcinoma -14. In tThis case illustrates the difficulties in making the diagnosis and the dangers of leaving such cysts untreated.Histology revealed invasive in-situ adenocarcinoma, which has been previously described . ImmunohIn view of the differential cytokeratin profile in this case (CK 7 and 20 positive) suggesting a fore gut origin, an adjuvant chemotherapy regimen comprising a fluoropyrimidine and oxaliplatin was recommended. Due to the rarity of such cases no direct evidence regarding adjuvant systemic therapy could be applied. Unfortunately, the condition of this patient deteriorated rapidly and he did not receive chemotherapy.The aggressive nature of this type of cyst has not previously been described. The cyst had remained dormant for at least 4 years. The high levels of CA19.9 related to the cyst may be a marker of its aggressive nature. The authors do not know if tumour markers should be measured routinely in cases similar to this.Following surgical excision the patient developed rapidly progressive metastatic disease. Although the patient died before receiving chemotherapy, the decision to treat with adjuvant therapy was made since the cyst had ruptured during surgery. Although its role remains to be defined the aggressive malignant potential demonstrated in this article suggests that post-surgical adjuvant therapy should be considered.This represents the third case in the literature of adenocarcinoma arising within a retroperitoneal enterogenous cyst. It is also the second case of such a cyst not associated with any anatomical structures and resulted in death due to aggressive uncontrollable malignant disease following excision. Cases like these must be reported in order to build a profile of clinical and pathological presentations of these rare cysts. This case demonstrates the difficulties in make a diagnosis, the importance of a multi-disciplinary approach and raises further questions regarding post-operative treatment with chemotherapy.The author(s) declare that they have no competing interests.JTLprepared the draft manuscript. RLJ, NDK, SdS and JGW helped in preparation of the manuscriptGM and NM edited the final versionAll authors read and approved the final manuscript.


Herein, we report a 66 year old woman who was diagnosed to have intrabiliary rupture of liver hydatid cyst with demonstrative computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography findings, with a review of the literature. The hydatid cyst is a parasitic disease mainly caused by Echinococcus granulosus. Hydatid cysts are common in the Mediterranean countries, the Middle and Far East, Europe, Asia, South America and Australia. In humans, the most commonly infested organs are the liver and lung .Anaphylactic shock, cyst infection of the biliary tree, and rupture into the peritoneum are the most common complications. Intrabiliary rupture is reported to be seen in a range of 6,1-17% -4]. In . In 4]. Abdominal ultrasound, CT and magnetic resonance imaging (MRI) are used to diagnose the complications. Endoscopic retrograde cholangiopancreatography (ERCP) is used extensively nowadays both in the diagnosis and treatment of biliary complications of hepatic hydatid cysts . We repoSixty-six years old Turkish woman living in eastern Turkey presented to our hospital with complaints of a continuous epigastric and right upper quadrant pain which had a duration of one month. The patient also had nausea, vomitting, loss of appetite, and jaundice. The patient had no previous disease, she is non-alcoholic, and her family history was not significant. On physical examination, diffuse tenderness was noticed at the epigastric and right upper quadrant regions. Laboratory findings revealed increase in serum aspartate transaminase (420 U/L) and alanine aminotransferase levels (180 U/L) , elevated bilirubin (5.17 mg/dl), amilase (1292.55 U/L), and gamma glutamyl transferase (154.3 U/L). All the other laboratory examinations were normal.On sonography, multiloculated hepatic cystic lesions maximum of 6 cm in diameter and dilatation of the bile ducts were detected. The gallbladder was enlarged, containing stones and echogenic material. The common bile duct was dilated and contained echogenic material inside without an acoustic shadow. The CT scan revealed dilated bile ducts which contained linear hyperdense material corresponding to germinative membranes together with other cyst contents Figure . The MRILaparotomy, cystectomy, cyst drainage, cholecystectomy, common bile duct exploration, and T-tube drainage were performed surgically. At the operation it was found that the hydatid cyst was ruptured into the bile ducts and there were daughter vesicles in the common bile duct. The gallbladder was hydropic containing multiple stones and cyst material inside.All the laboratory findings returned to normal after the operation. Control MRI examinations were performed 6 months after the operation which showed hepatic hydatid cysts and changes secondary to operation Figure .contained rupture when the endocyst is torn, but the cyst content is confined within the pericyst; communicating rupture consists of tear of the endocyst with loss of the cyst content via small biliary ducts and direct rupture when a tear of both endocyst and pericyst occurs, allowing the cyst content to spill into the peritoneal or pleural spaces . Di. DiocculAlthough communication between biliary system and cyst occurs in 80%-90% of patients with hepatid hydatic cyst, the incidence of clinical CBC is only 13%-37%. The incidence of frank intrabiliary rupture ranges between 5% and 17%. Although its mortality and morbidity are high, there are no significant problems with the management of CBC. However, occult CBC, which constitutes 10%-37% of cases, is difficult to diagnose, because the symptoms and preoperative radiological findings are unremarkable. CBC is the most common complication of hepatic hydatid cyst, occuring in 14%-25% of cases of postoperative biliary leakage [Before the introduction of US or CT, preoperative diagnosis of the complications of hepatic hydatidosis was difficult and based on clinical manifestations and results of laboratory studies. US and CT may suggest the diagnosis of a frank intrabiliary rupture in most of the cases, whereas MRI provides additional multiplanar images. MRCP provides a characteristic intense rim, daughter cysts, detachment of the membranes, and dilated biliary tree containing hydatid material ,12.Potential sites of rupture are peritoneum, bile ducts, pleural space, thorax, and visceral organs such as stomach or duodenum .Surgical management of hepatic hydatid disease has ranged from radical procedures like hepatic resection and total cystopericystectomy to conservative ones like cyst evacuation followed by capsulorrhaphy or external drainage. The aim is to remove the entire disease while minimizing complications. Conservative procedures are safe and technically simple, and are useful in the management of uncomplicated hydatid cysts. However, their main disadvantage is the high frequency of postoperative complications, the most common being bile leak from a cyst-biliary communication and its sequelae like bilio-cutaneous fistulae, bilomas and bile peritonitis (4%-28%) .External biliary fistulae following surgery for liver hydatid disease tend to close spontaneously. In a review of 304 cases, all the 10 external biliary fistulae closed spontaneously over a period of 2-4 months . In anotMost series on hepatic hydatid disease report on a small number of patients with postoperative external biliary fistulae; it is generally accepted that endoscopic management in the form of endoscopic sphincterotomy, with or without stenting or naso-biliary drainage, plays a key role in the management of such patients. Endoscopic sphincterotomy is believed to reduce the high intra-biliary pressure, and promote early closure of these fistulae even in the absence of distal biliary obstruction .As a conclusion, sonographic findings of intrabiliary rupture of hepatic hydatid cysts have been discussed in the literature before, but as far as we know, CT, MRI and MRCP findings are not well demonstrated until now, which made us report our case.TOD: Transomental Defect; CT: Computerised tomography; MRI: Magnetic resonance imaging; MRCP: Magnetic resonance cholangiopancreatography; US: Ultrasonography; ERCP: Endoscopic retrograde cholangiopancreatography; CBC: Complete blood count.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.SA, ÖÜ, and HA analyzed and interpreted the patient data regarding the clinical and radiological findings of the patient. All authors were a major contributor in writing the manuscript. All authors read and approved the final manuscript.


Antecedent trauma has been implicated in the causation of soft tissue tumors. Several criteria have been established to define a cause-and-effect relationship. We postulate possible mechanisms in the genesis of soft tissue tumors following antecedent traumatic injury.We present a 27-year-old woman with a paraspinal desmoid tumor, diagnosed 3-years following a motor vehicle accident. Literature is reviewed.Soft tissue tumors arising at the site of previous trauma may be desmoids, pseudolipomas or rarely, other soft tissue growths. The cause-and-effect issue of desmoid or other soft tissue tumors goes beyond their diagnosis and treatment. Surgeons should be acquainted with this diagnostic entity as it may also involve questions of longer follow-up and compensation and disability privileges. The etiology of most soft tissue tumors is unknown. Our search of the English literature revealed a few case reports of soft tissue tumors developing at the site of a previous traumatic injury -17. DesmWe describe a young woman with a left paraspinal desmoid tumor at the site of a recent trauma, possibly associated with a cause-and-effect mechanism. We hope this study will shed more light on this phenomenon.A 27-year-old woman presented with a large subcutaneous mass in the upper back Figure  of 8 monFamily history and past medical history were unremarkable. The patient reported that she had been involved in a motor vehicle accident 3 years previously, in which she sustained a brain concussion, fracture of the right lamina of the C-6 vertebra, and comminuted fractures of the left radius, ulna and femur.2 weighted images and enhanced with the injection of contrast material, demonstrating its vascularity. Findings on core needle biopsy were compatible with desmoid tumor. Colonoscopy revealed no abnormalities.Physical examination revealed a firm mass measuring 15 × 10 cm, adherent to its surroundings, with no apparent pathological vasculature or satellite lesions. Cytological examination was inconclusive. Magnetic resonance imaging (MRI) demonstrated a solid space-occupying lesion measuring 12 × 4.8 × 7.6 cm, located in the left paraspinal region beneath the trapezium muscle (asterisk), compressing the paraspinal muscles medially  scan, 4 months later demonstrated tumor growth. There was no evidence of infiltration of adjacent bony structures or pulmonary metastases. The patient was offered surgery.The tumor was surgically excised. It measured 9 × 12 × 22 cm and weighed 1970 grams. It was relatively well circumscribed, with a fibrous consistency, and no areas of hemorrhage or necrosis. Microscopic study revealed relatively low up to 2–3/10HPF) mitotic activity  have a 1000-fold increased risk of developing desmoid tumors compared to the general population. The abdomen is the most common site of the tumors in this patient group, many times following a surgical insult.et al., -catenin or adenomatous polyposis coli (APC) gene mutation leading to intranuclear accumulation of [beta]-catenin . The expThe pathogenesis of desmoid tumor may involve genetic abnormalities, sex hormones, and trauma , includiet al., [et al., [et al., [Gebhart et al.,  reportedet al., . Skhiri [et al.,  reported[et al.,  describeet al., [Traumatic injury has been implicated as a causative factor in the genesis of other soft tissues as well. Radhi et al.,  reportedIn 1969, Brooke and MacGregor  suggesteThe largest series of 24 pseudolipomas was reported by Rozner and Isaacs  describeet al., [In 1988, Dodenhoff  describeWarren  listed sIn our case, the wounded part (upper back) was previously tumor-free, the authenticity of the trauma was confirmed by MRI, the tumor originated within the boundary of the injury, and the latency period was reasonable. Furthermore, the desmoid histology was compatible with a scar or other reparative process. Thus, the tumor met the criteria of both Warren  and EwinThe cause-and-effect issue of desmoid or other soft tissue tumors goes beyond their diagnosis and treatment. It may also involve questions of longer follow-up and compensation and disability privileges.Pseudolipomas are not real neoplasia, but they seem to account for the reports of the so-called post-traumatic lipomas. The post-injury local reparatory mechanisms better explain the creation of desmoid tumors, which, in these rare cases, seem to have lost control of cell growth, giving rise to a soft tissue tumor. The rarity of desmoid tumor, its specific biology, the well-documented association between abdominal wall desmoids and pregnancy, and even the tendency of surgery to induce new desmoid tumors in patients with FAP support the notion that trauma/tissue injury is a likely cause of at least, some of these tumors, including the one described here.CT-computerized tomography; FAP-familial adenomatous polyposis; MRI-magnetic resonance imagingThe author(s) declare that they have no competing interests.CS participated in drafting the manuscript, interpretation of data and conceptual design, AD conceived the study and participated in drafting the manuscript, BO carried out the imaging analysis and interpretation of data, GH carried out the surgical procedure, conceptual design, participated in drafting the manuscript and revised it critically for important intellectual content.All authors read and approved the final manuscript.


Hemangiopericytoma is a rare neoplasm that can occur in any part of the human body, but it rarely develops in the greater omentum. We report a case of a patient who presented with a huge abdominal lump. At laparotomy, a huge vascular tumor, which was observed originating from the greater omentum, was resected. Histopathology investigation revealed this tumor as a benign hemangiopericytoma with a malignant potential. Hemangiopericytoma is a rare tumor featuring Zimmermann's pericytes, which was first described by Stout and Murry. Pericyte3 was detected arising from the greater omentum [A 41-year-old male patient was admitted to the with a huge abdominal lump. Abdominal ultrasonography diagnosed it as a retroperitoneal tumor. Fine needle aspiration cytology (FNAC) described it as being mesenchymal in origin. During laparotomy, a huge, dark brown, lobulated mass weighing 470 g measuring 12.5 × 8.5 × 7.5 cm omentum . This ma omentum . The tumHemangiopericytoma is a rare tumor featuring Zimmerman's pericytes, which was first described by Stout and Murry. Hemangio4


Collecting duct carcinoma is a rare type of renal cell carcinoma. The primary is difficult to diagnose on imaging, and metastases are often present on initial presentation. Extensive multiorgan metastases can result in complex presentations that can be difficult to diagnose.We present two case reports of multiorgan metastases of collecting duct carcinoma that were autopsy confirmed. The first case was a 55-year-old man who presented with fever and abdominal pain. Abdominal computed tomography showed enlargement of the right kidney. Pyelonephritis was considered on the basis of laboratory test results and imaging findings. However, multiple cavitary lesions were found on routine chest radiography. These lesions were biopsied, resulting in a histological diagnosis of metastatic adenocarcinoma. A renal tumor was considered. Transitional cell carcinoma was suspected, which proved to be misdiagnosed and chemotherapy was given accordingly. However, this was not effective and the patient died after 2 months. Autopsy demonstrated the primary tumor to be collecting duct carcinoma, with metastases to lung, liver, spleen, bone marrow, right adrenal gland, and para-aortic lymph node. Computed tomography done while the patient was alive detected lung, liver, and para-aortic lymph node metastases. The second case was a 77-year-old man who presented with fever. Pyelonephritis was considered on the basis of the laboratory test results and imaging findings. Antibiotic therapy improved his symptoms and laboratory indicators of inflammation. One year later, he developed backache. Computed tomography revealed a progressively enlarging right renal lesion, multiple liver masses, enlargement of the para-aortic lymph nodes, and multiple osteoblastic and osteoclastic lesions. A renal tumor with multiple metastases was diagnosed. Chemotherapy was given without effect, and the patient died of cardiac failure 1 year later. Autopsy revealed a primary tumor of collecting duct carcinoma with metastases to the liver, right adrenal gland, right upper ureter, bone marrow, para-aortic and mediastinal lymph nodes, and bone.We present the radiological findings of lung, liver, lymph node, and bone metastases in two patients with collecting duct carcinoma. Collecting duct carcinoma (CDC) is a rare type of renal cell carcinoma (RCC), accounting for 0.4% to 1.8% of all RCCs . In geneA 55-year-old man presented with fever and abdominal pain. Abdominal CT showed swelling of the right kidney and low attenuation areas Figure . LaboratA 77-year-old man was admitted to our department after developing a fever and backache. Based on CT findings Figure  and laboCDC is an uncommon yet distinct epithelial neoplasm of the kidney . Unlike et al. [et al. [Characteristic imaging findings of CDC are not well delineated because only case reports or studies involving small numbers of patients have been published to date. Fukuya et al.  reported [et al.  reportedUp to 40% of CDC patients have metastatic disease at the time of presentation . In caseOne of our cases had bone metastases that exhibited both osteolytic and osteoblastic features. This pattern of bony metastases was also observed in a recent report .When extensive multiorgan metastases with necrotic changes are seen along with aggressive involvement of the kidney, the differential diagnosis of the primary tumor should include collecting duct carcinoma.Written informed consent for publication of these case reports and any accompanying images was obtained from the patients in both cases. 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.HN conception and acquisition of data. YK design and analysis of data and drafting the manuscript. MK acquisition and interpretation of data. AS interpretation of data and drafting the manuscript. TY drafting and revising the manuscript. YN revising and final approval of the manuscript


It arises from smooth muscle of the uterus and is a rare tumor that accounts for 2% to 5% of all uterine malignancies. Very few cases are reported in the literature. Our patient did not have any history of genital bleeding, which is the usual presentation in uterine sarcoma. We report an original case report of an unusual presentation of this rare tumor arising from the uterus.Malignant change in a leiomyoma or uterine fibroid is termed A 40-year-old nulliparous woman of South Indian origin presented with a mass in her abdomen for one year with a rapid increase in size over the previous three months. Tumor marker CA-125 was raised, and a computed tomography scan showed a mass arising from the pelvis. An exploratory laparotomy was performed and the histopathology report confirmed the diagnosis of uterine leiomyosarcoma.Because of their rarity, uterine sarcomas are not suitable for screening. Diagnosis is by histopathologic examination and surgery is the only treatment. The prognosis for women with uterine sarcoma primarily depends on the extent of disease at the time of diagnosis and the mitotic index. It arises from smooth muscle of the uterus and is a rare tumor that accounts for 2% to 5% of all uterine malignancies [Malignant change in a leiomyoma or uterine fibroid is termed gnancies .A 40-year-old nulliparous woman of South Indian origin reported to our outpatient clinic with complaints of a mass in the lower abdomen for one year and lower abdominal pain for three months. The patient was apparently asymptomatic one year previously, and then she noticed a mass in the lower abdomen that gradually increased in size. She provided a history of a rapid increase in size for the past three months. She also had associated lower abdominal pain, which was dull and aching in type, dragging in nature and continuous with no aggravating or relieving factors. Her menstrual cycles were regular and normal. She had no history of genital bleeding.On examination, pallor was present, and the patient's vital signs were normal. She was thinly built. On abdominal examination, an irregular midline mass arising from the pelvis was present. The upper and lateral borders of the mass could be made out; the lower margin could not be ascertained. The mass was firm to hard in consistency with restricted mobility and nontender with no free fluid. There was no hepatosplenomegaly.On vaginal examination, the patient's uterus was enlarged to 20 weeks' gestational size and nodular, occupying the whole pelvis. No mass could be appreciated separate from uterus. Computed tomography (CT) scan findings were suggestive of a large, multiloculated, multiseptate growth containing both solid and cystic elements arising from the pelvis and extending into the abdomen, measuring 25 × 17 cm, adherent to both the uterus and bladder. CA125 was 94.80 ng/mL (0-35 ng/mL). Other results were normal, and the patient was posted for exploratory laparotomy.Intraoperatively, omental adhesions to the mass were noticed. A mass of 20 weeks' size was arising from the fundus of the uterus Figure . It was Total abdominal hysterectomy  with bilateral salpingo oophorectomy was done Figure . The uteUterine sarcomas are rare and constitute about 2% to 5% of all uterine malignancies . Prior pBecause of their rarity, uterine sarcomas are not suitable for screening. Surgery is the only treatment. The prognosis for women with uterine sarcoma primarily depends on the extent of disease at the time of diagnosis and the mitotic index .Women wiWritten informed consent was obtained from the patient for publication of this case report. 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.The case was managed by CNR, VVS, MR, KS and SCB. All authors have read and approved the final manuscript.


We report a case of a Calcium pyrophosphate dihydrate deposition disease (CPPD) presenting as a mass in the parotid and temporomandibular joint (TMJ) that simulated a parotid tumor. A 35 year-old man presented with pain in the left ear area. A CT Scan of the area showed a large, calcified mass surrounding the left condylar head, and extending into the infratemporal fossa. FNA of the mass showed birefringent crystals, most of which were rhomboid with occasional ones being needle shaped, embedded in an amorphous pink substance. Scanning electron microscopy (SEM) with energy dispersive x-ray spectroscopy (EDS) of these crystals showed peaks corresponding to calcium and phosphorus. SEM/EDS is a rapid method of diagnosing calcium pyrophosphate dihydrate deposition disease (CPPD) and an alternative to more commonly used method of special staining of cell block sections coupled with polarizing microscopy. Calcium pyrophosphate dihydrate deposition disease is characterized by the accumulation of calcium pyrophosphate dihydrate crystals in intra-articular and periarticular tissues. CPPD of the temporomandibular joint is an uncommon entity. It is characterized by the presence of crystal deposits that can lead to the formation of a tumor/mass.We report a case of TMJ CPPD diagnosed by fine needle aspiration and microanalysis using SEM/EDS. To the best of our knowledge, the cytologic description of CPPD on FNA in TMJ has not previously been reported.A 35-year-old man presented to his physician with severe pain in his left ear. Examination showed a possible pustule within the external auditory canal along with TMJ discomfort. He was treated for ear infection but the lesion in the external auditory canal failed to resolve. A subsequent computed tomographic scan revealed a mass in the TMJ area. A more detailed history later revealed that the patient had been having discomfort/pain in the TMJ area for the past 4 years. The patient developed imbalance and tinnitus in the last few months along with hearing loss. An examination of the left parotid showed an area of obvious fullness in the preauricular area. External ears on both sides were normal. The left external auditory canal was tender and showed a small prominence in the anterior part of the canal. The left tympanic membrane was abnormal and showed mild erythema. The CT scans showed a calcified mass surrounding the left mandibular condyle, and extending medially into the infra-temporal fossa. The mass was eroding the floor of the middle fossa and the anterior wall of the epitympanum. A fine needle aspiration (FNA) of the mass was performed in the clinic. Subsequently, the patient underwent resection of the mass and TMJ condylectomy. The mass eroded the zygomatic process of the temporal bone superiorly and extended anterior and medial to the mandibular condyle.A fine needle aspiration of the mass was performed using a 23-gauge needle after anesthetizing the left parotid area with 2% lidocaine. Smears were made from the aspirate and stained with Diff-Quik and Papanicolaou stain. Multiple aspirations were obtained from different areas of the mass. The aspirate showed abundant extra-cellular crystals, which at places seem to be forming tophi Figure . The cryThe resected mass consisted of multiple fragments of tan-brown soft tissue from 0.5 to 3.0 cm (in aggregate 5.0 × 5.5 × 0.7 cm) with a chalky white covering. The resected portion of the condyle was 1.5 × 1.5 × 1.2 cm. The H&E stained section of the fragments showed nodular aggregates of crystals, surrounded by dense collagenous fibrosis Figure . Areas oThe freshly cut surface of the paraffin block from the FNA was directly examined with SEM/EDS using the variable pressure mode to allow examination of such non-conductive samples. The crystals demonstrated peaks for calcium and phosphorus. The crystals were present in groups/tophi and were mostly rhomboid with few needle shaped ones intermingled Figure . The cryCPPD is a rare, benign crystalline arthropathy of unknown cause. It is presumed to result from a disturbance of phosphate metabolism. Pritzker et al in 1976 first described a case of pseudogout in the TMJ but the term CPPD was first proposed by Ryan and McCarty in 1985 reportedTwo main forms of CPPD have been described – common/diffuse and tumoral. The common type of CPPD (pseudogout) usually affects larger joints and often follows trauma, surgery, or ischemic heart disease. The tumoral type mainly affects the TMJ, cervical spine and hand. The main differential diagnosis includes tophaceous gout, tumoral calcinosis, synovial chondromatosis, and benThe crystals of uric acid in gout are water-soluble ; needle shaped and demonstrates negative birefringence with polarized light, whereas the crystals of pseudogout have blunt/squared ends with weak birefringence. The crystals of CPPD are 2–40 micrometer, typically rhomboid shaped but small-long rods and even squares may be seen. Areas oThe role of FNA in diagnosis of CPPD has been described in the neck,8 vertebThe cell block can be used for histopathological findings and also for SEM/EDS. Our findings of aggregates of crystals with basophilic appearance on cell block was similar to that previously reported by Pakzad et al. Our hisCT scan is considered to be the best imaging modality for the diagnosis of CPPD. The CT A detailed literature search revealed that SEM/EDS has been used in characterizing the crystals of CPPD in a few cases. Dijkgraaf et al examinedIn summary, FNA findings coupled with SEM/EDS when available can provide a rapid and accurate diagnosis of CPPD of TMJ and an alternative to other ancillary techniques including polarizing microscopy along with special stains like NAES.The cytology diagnosis was made by Dr. Asghar H Naqvi and Dr. Kamal K Khurana. Dr. Jerrold L Abraham rendered the Scanning Electron Microscopy report. Dr. Robert M Kellman clinically evaluated the patient. Dr Asghar H Naqvi, Dr Jerrold L Abraham and Dr Kamal K Khurana equally contributed in drafting and designing the manuscript. All the authors read and approved the final manuscript.


Double Antibody Glucagon compared to DakoCytomation's Polyclonal Rabbit Anti-Human Glucagon allow for pathologic distinction of enteral versus pancreatic glucagonoma.This case report demonstrates that the differential immunohistochemical reactivities of Siemens' Double Antibody Glucagon, a clinical syndrome consistent with glucagon hypersecretion. A periampullary mass biopsy proved to be a neuroendocrine tumor, with positive immunohistochemical reactivity to DakoCytomation's Polyclonal Rabbit Anti-Human Glucagon.A 64-year-old Caucasian man was diagnosed with a duodenal enteroglucagonoma following presentation with obstructive jaundice. He had a low serum glucagon level using Siemens' Double Antibody Glucagon and DakoCytomation's Polyclonal Rabbit Anti-Human Glucagon discerns enteroglucagon from pancreatic glucagon.Differential comparison of the immunohistochemical reactivities of Siemens'  Pancreatic glucagonomas are rare neuroendocrine tumors with an estimated incidence of approximately 1 in 20 million ,4.Neuroendocrine tumors are slow-growing neoplasms . Both chTreatment of glucagonomas depends on location, size and lymph node involvement. Simple excision with resection of peripancreatic lymph nodes is the treatment of choice for isolated confined lesions . Periamp"Enteroglucagon" applies to a group of proglucagon peptide cleavage fragments. The proglucagon moiety forms the precursor molecule for both pancreatic and enteral glucagon. Differential proteolytic processing occurs in these tissue spaces. Enteral cleavage results in the major products glucagon-like peptide 1 (GLP-1), glucagon-like peptide 2 (GLP-2) and glicentin. Glicentin is cleaved to oxyntomodulin and glicentin-related pancreatic product (GRPP). Oxyntomodulin closely resembles glucagon with the only difference being an eight amino acid C-terminus extension . The gluet al. specifically reported assay positivity for an N-terminal reactive antibody measuring both pancreatic and gut glucagon-like immunoreactivity with negative C-terminal reactive antibody more specific for pancreatic glucagon and demonstrating only a 2% cross-reactivity with gut extracts [Enteroglucagon secreting tumors have been reported but detection of enteroglucagon was indirect ,9,10. StOur patient was a 64-year-old Caucasian man referred from an outside hospital for evaluation and treatment of obstructive jaundice secondary to a periampullary mass. Patient informed consent for publication of findings was obtained during admission. Prior admission to the outlying hospital had occurred 5 days before transfer following presentation with jaundice, fatigue and bilateral lower extremity edema.Double Glucagon Antibody and found to be depressed . The serum serotonin level (< 25 ng/mL) was low  and the vasoactive intestinal polypeptide (VIP) level (< 30 pg/mL) was within normal limits . Carcinoembryonic antigen (CEA) level was normal at 1.1 ng/mL. Carbohydrate antigen 19-9 (CA 19-9) was markedly elevated at 233.5 U/mL. Computed tomography revealed a 3.2 cm by 1.9 cm hypervascular mass in the medial duodenal wall juxtaposing the pancreas. Three hepatic lesions appeared consistent with metastasis.Hypoalbuminemia, refractory severe hypokalemia, uncontrolled new onset diabetes mellitus, and anemia were associated findings. New onset diabetes mellitus was diagnosed 3 weeks before developing jaundice. His medical history was significant for gout, hypertension, hyperlipidemia, osteoarthritis, degenerative disk disease, erectile dysfunction, and two episodes of idiopathic pancreatitis 15 years earlier. Glycosylated hemoglobin measured an elevated 7.5%. Endoscopic retrograde cholangiopancreatography (ERCP) was performed at the referring institution before transfer. Biopsies returned a diagnosis of neuroendocrine tumor, not otherwise specified. The patient was then transferred to our hospital. His serum potassium remained low despite all replacement efforts. Hypoalbuminemia worsened to 1.6 gm/dL. Serum glucagon was measured using Siemens' Exploratory laparotomy was performed. Five perihepatic lymph nodes appeared suspicious for metastatic disease; they were surgically excised and examined by frozen section. Three of these five lymph nodes were positive for metastatic neuroendocrine carcinoma. Two liver masses each less than 1 cm were identified by intra-operative ultrasound. Each lesion was wedge resected and pathologic examination confirmed metastases. Pancreaticoduodenectomy was indicated and performed due to lymph node and hepatic metastases. Pathology specimens stained positive for chromogranin, synaptophysin, and glucagon. The tumor mass was 4.5 cm and infiltrated through the duodenal wall into the pancreas. Extensive angiolymphatic invasion was noted.The patient's post-operative course was complicated by respiratory aspiration, disseminated candidiasis with candidal sepsis, wound infection, disseminated varicella, atrial fibrillation, acute renal failure and a subhepatic intra-abdominal candidal abscess. Discharge to a rehabilitation center occurred on post-operative day 53. Results of the multidisciplinary gastrointestinal tumor board recommended close monitoring with long-acting octreotide.The patient presented clinically with evidence supporting a glucagonoma. The classic glucagonoma syndrome consists of necrotizing migratory erythema, hyperglycemia without ketosis, weight loss, and anemia ,4,11. UnPolyclonal Rabbit Anti-Human Glucagon antibody reacts with all moieties containing the glucagon molecule . Carpinteria, California: DakoCytomation, Inc.) We sought to clarify the inconsistencies in the serum and tissue glucagon reactivities.The Whipple resection specimen consisted of a segment of distal stomach, duodenum, and proximal pancreas. Located in the immediate periampullary region was a 4.5 × 2.0 × 1.5 cm well-circumscribed solid firm tan-yellow submucosal nodule covered by intact mucosa. Light microscopy demonstrated the tumor infiltrating through the duodenal wall into the superficial pancreas. On immunohistochemistry, tumor cells were positive for synaptophysin, chromogranin and glucagon. The manufacturer reported that the Double Antibody Glucagon I125 radioimmunoassay used for serum glucagon level determination failed to detect any glucagon reactivity with the addition of Glucagon-like Peptide 1 (GLP-1), Glucagon-like Peptide 2 (GLP-2), or Oxyntomodulin (Glucagon 37/Enteroglucagon) to a level of 1,000,000 pg/ml . Sensitivity for human pancreatic glucagon, however, was excellent. The Siemens' Double Antibody Glucagon I 125 radioimmunoassay then has particular specificity for pancreatic glucagon but not enteral glucagon. Consequently, analysis of the patient's serum only indicated the level of pancreatic glucagon while tissue reactivity with Polyclonal Rabbit Anti-Human Glucagon reagent was capable of detecting both pancreatic and enteral glucagon. To summarize, the laboratory blood and solid tissue results revealed an intestinal neuroendocrine tumor producing a non-pancreatic form of glucagon: enteroglucagon. GLP-1 inhibition of glucagon secretion may have resulted in the depressed serum level of detectable pancreatic glucagon [The analyzing laboratory was contacted and no compromise of the patient's submitted specimen was suspected. Prior studies performed by Siemens  showed that the glucagon .Double Antibody Glucagon reflect only pancreatic glucagon production and do not indicate enteroglucagon production. Tissue analysis using Polyclonal Rabbit Anti-Human Glucagon detects any glucagon sequence containing moieties including enteroglucagon. Patients clinically exhibiting a glucagonoma syndrome with paradoxical low serum glucagon levels and tissue glucagon immunohistochemical positivity may possess an underlying enteroglucagonoma.Serum glucagon levels measured with Written informed consent was obtained from the patient for publication of this case report. 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.WV directly assembled the patient information, formulated in depth analyses of the known literature, performed the operative interventions and managed the recovery of the patient, constructed and edited the manuscript, was responsible for the final construction of the manuscript for submission to the senior staff/primary investigator, and served as the corresponding author. ZZ processed, evaluated and diagnosed the submitted surgical specimens. She also constructed the pathology narrative for the final manuscript. MA served as the senior staff reviewer/primary investigator. He directly supervised every element of the patient's course and was responsible for final approval of the submitted manuscript following his directed revisions.


Acute abdomen in advanced pregnancy is one of the most challenging surgical situations. In life-threatening situations, despite optimal management, foetus distress and preterm delivery may occur. Although laparostomy is a useful treatment of abdominal sepsis, its successful management has not been reported previously in pregnant women.30-year-old woman at 23 week of pregnancy was investigated for non-specific abdominal pain. Surgical exploration revealed extensive ischemic bowel necrosis. Multiple segmental resections were performed and abdomen was left open with vacuum assisted dressing, maintained for 48 hours. At the third surgical look the continuity was restored and abdominal wall closed. The foetal condition stayed unperturbed under pharmacologic tocolysis. Pregnancy was carried to full term delivery.Open abdomen strategy can be successfully applied in pregnant woman. Acute abdominal pain in advanced pregnancy remains a diagnostic and management challenge. During pregnancy the usual clinical presentation is masked by gravid uterus and physiological changes. Imaging procedures can rarely help to resolve a diagnostic dilemma because of modified abdominal anatomy and limits in x-ray techniques use . For theFor the most frequent acute abdomen causes including acute appendicitis, cholecystitis, mechanical obstruction and gastric ulcer perforation standard surgical management gives relatively good outcomes with overall 6% of miscarriage, 2.5% of foetus lost and less then 4% of premature labour rate . The matHowever, the decision to operate is often delayed during pregnancy. This is probably the first reason of high foetal or mother morbidity. The recommendation in these situations is to manage the surgical problem regardless of the pregnancy using the same surgical strategy as for non-pregnant patient .A 30-year-old woman was admitted to the emergency department at 23 week of her second pregnancy for non-specific abdominal pain. She was known for previous minor abdominal surgery including mesenteric cyst excision and vesicoureteral reflux surgery in childhood followed by laparoscopic adhesiolysis 10 years later. She had no fever and no vomiting or constipation history. Biological tests including RBC, WBC, C-reactive protein, bilirubin, pancreatic enzymes and serum lactates were also still normal during 48 hours of observation. The initial imaging investigations by abdominal and pelvic ultrasound showed no intra-abdominal abnormalities and the plain abdominal x-ray at 48 hours revealed only some very slightly dilated small bowel loops. The foetus status in ultrasound was normal.® Therapy™, KCI Inc.) with 125 mmHg continuous negative pressure. At the second and third surgical look some intestinal segments required subsequent additional resections. Eventually, after 48 hours of open abdomen management, the intestinal continuity was restored leaving 110 cm of viable small bowel. Abdominal wall was primary closed without aponeurotic defect (figure Persistence of pain not relieved with strong analgesics conducted to laparoscopic exploration despite the absence of biological or radiological abnormality. Laparoscopy revealed massive necrotic lesions of the small bowel with rare viable segments in discontinuity. After conversion to laparotomy multiple segmental resections were performed, potentially viable bowel segments were closed by stapling and abdomen was left open with vacuum assisted dressing in the aim to asses the viability of remaining bowel after 24 and 48 hours figures , 2. The t figure .During the two days where the abdomen was left open, optimal foetal and mother conditions were maintained by intensive care procedures including sedation, mechanical ventilation, liquid resuscitation, adapted parenteral nutrition and pharmacologic tocolysis by hexoprenaline.th postoperative day. Complete recovery requires in-hospital and ambulatory nutritional support for short bowel syndrome. Pregnancy was uneventfully carried to full term vaginal delivery.The patient left the intensive care unit on 9Open abdomen management has become a commonly adopted strategy in severe surgical conditions. Critical intra-abdominal infection, blunt or open trauma, intestinal ischemia and abdominal hypertension are typical indications to leave the abdomen open. It is also the treatment of abdominal compartment syndrome. The rare cases of laparostomy use in pregnant women previously reported haven't been successful in terms of foetus or mother issue . The autIn the present case leaving the abdomen open was a deliberate intraoperative decision. We adopted the principles of damage control surgery consisting of planned subsequent delayed explorations after the primary debridement and necrotic bowel resections.It was shown that temporary dressing with vacuum pack is a safe, well tolerated technique . The disThe present case report contributes to the rational that decision making in severe abdominal surgical emergency in pregnant women should respect the same principles and use the same techniques as in non-pregnant patient. The decision process should not be delayed by pregnancy. The management of acute abdomen by laparostomy during pregnancy is feasible, and may be associated with a good outcome for both the mother and the child.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.The authors declare that they have no competing interests.WS and MC contributed equally to this work; WS and MC drafted the paper; WS wrote, FM critically revised and VB critically revised the paper with an important conceptual and editorial input. All authors read and approved the final manuscript.


Myxofibrosarcoma is one of the most common sarcomas in elderly patients showing a slight male prevalence. The tumor is mainly located in lower and upper extremities and rarely in trunk, neck and feet. We present a case of a 84-year-old man referred to our tumour center with a giant and neglected high-grade tibial myxofibrosarcoma in the anteromedial side of tibial mid-diaphysis. Large size lesions in association with older age may jeopardise the maintenance of limb vitality, vascularity and stability.Authors performed a complete tumour resection, followed by reconstruction of bone and soft tissue defects with cement, plate and a musculocutaneous gastrocnemius flap, in order to cover the underlying bone and promote uneventful healing and perfusion of the operated extremity.At 2 years postoperatively, limb salvage, good functional outcome and no tumour recurrence were reported while the patient was able to effectively perform the majority of the daily activities. Myxofibrosarcoma is one of the most common sarcomas in elderly patients showing a slight male prevalence . Tumor iWide resection of the malignant tumour constitutes the preferred treatment choice . HoweverAn 84-year-old man was referred to our tumour center with a large mass (8 × 5 cm) in the anteromedial side of tibial mid-diaphysis Fig. . The lesThe next step was fine needle aspiration biopsy of the mass. Histological examination revealed that the tumour was a malignant myxofibrosarcoma. Chest and abdominal Computed Tomography (CT) scans as well as radionuclide total bone scan excluded the presence of systemic lesions or metastasis. Therefore, the patient was scheduled to undergo surgical resection of the lesion with additional removal of the adjacent soft tissue structures.en block wide excision of the mass was performed. As the tumour was in close proximity to mid-diaphyseal tibial bone, part of the tibial anterior-medial cortex was also removed to facilitate at least 2 cm tumour-free resection margins circumferentially. The tibial defect of 9 cm was filled with acrylic cement and fixed with a 4.5 mm locking plate and screws. Tumour removal was followed by coverage of the large remaining soft tissue and skin defect (25 cm) with a microvascular musculocutaneous flap from the contralateral thigh.Under general anaesthesia and after tourniquet inflation, Histological analysis of the excised tumour showed a high-grade myxofibrosarcoma with solid sheets and areas of necrosis. At higher magnification, multiple spindle cell cells of mesenchymal origin surrounded by many polygonal epithelioid cells with pleomorphism and elevated mitotic index were found. Surgical margins were disease free. In addition, immunohistochemical staining was negative for CD34, smooth muscular actin, for desmin, keratins, caldesmon, and S-100, which confirmed the high-grade character of the lesion.The postoperative period was uneventful. Partial weight bearing for 6 weeks was advised. After wound healing, about 3 weeks, the patient was referred to the Oncology Department for local Radioterapy. At 2 years follow up evaluation, plain X-Rays and MRI of the operated site didn't reveal any signs of local recurrence (Fig. Myxofibrosarcoma includes a group of mesenchymal neoplasms of fibrous-histiocytic malignant line that mainly is appeared in extremities and has a high rate of local recurrence. The tumour shows a wide variability of presentation and prognosis and is classified as low or high grade . Low-graAbout 2/3 of lesions are superficial and developed in dermal or subcutaneous tissue . SuperfiLocal recurrence occurs in approximately 50-60% of cases regardless the histological grade and location of the lesion ,2. Howeven block wide excision of the mass with tumor-free margins was achieved by partial removal of the anteromedial tibial cortex. Bone reconstruction was performed with plate, screws and acrylic cement allowing early mobilisation and weight-bearing. In our clinic we usually use this solution for the reconstruction of bone defects, because of stability and strength regained to the bone.Wide resection of the mass is mandatory to decrease the risk of local recurrence and increase the overall survival rate.  This mayHowever, the large residual soft tissue defect in association with patient's age threatened the integrity and function of the limb. Coverage of the large soft tissue defect was performed with a musculo-cutaneous vascular flap that was harvested from the contralateral thigh . AlthougPostoperative adjuvant radiation therapy is usually applied even if surgical margins are tumour-free in order to optimise the benefits obtained by the surgery, as universally accepted in literature,3,5,8. MGiant myxofibrosarcomas may severely compromise limb integrity and function. Wide resection and reconstruction of the remaining soft-tissue defect with a micro-vascular flap can be an effective limb-sparing procedure even in elderly with concomitant medical co morbidities.Written consent was obtained from the patient for publication of this case report.The authors declare that they have no competing interests.DM prepared the draft of case report. GM conceived the idea of the case report and helped with the draft of it. BC, MA, MS and MLR helped the draft of the case report. All authors read and approved the final manuscript.


Carcinoids are neuroendocrine tumours which may secrete hormones like gastrin, insulin, ACTH, etc. Liver is a common site for metastasis of carcinoid origin and an unusual site for a primary carcinoid tumour to arise.We present the case of a 51-year-old Caucasian man with diarrhoea, weight loss, duodenum ulcers and a liver mass in ultrasonography. A primary hepatic carcinoid tumour with a Zollinger Ellison syndrome was diagnosed. Surgery resection was performed and the patient remained free of symptoms two years after, with normalisation of gastrin levels.Primary hepatic carcinoid tumour represents an uncommon diagnosis, based on radiological and pathological features. The exclusion of different primary locations is necessary. Once associated with a Zollinger Ellison syndrome, diagnose may be more complicated and challenging since only 7 cases of hepatic carcinoids with gastrin secretion were reported in medical literature.A review of medical literature is performed and diagnoses tools that should be used for an accurate diagnosis and available treatment approaches are commented here. Gastrin is predominantly produced by G cells of the gastric antrum and plays two major roles in the gastrointestinal physiology: (i) stimulation of gastric acid secretion and (ii) secondly as a trophic hormone for epithelial and enterochromaffin cells growth ,2. ThereCarcinoid tumours are neoplasms derived from the neuroendocrine system and usually secret hormones like gastrin, chromogranin A, serotonin, ACTH, insulin, etc. . The majA 51-year-old Caucasian man of Spanish nationality was admitted to our department for diarrhoea. The symptoms began 11 years ago and were intermittent and mostly nocturnal. He worsened 3 months before admission with exacerbation of number of deposition, abdominal pain and weight loss. He was on PPI therapy on demand and at high doses for pyrosis. He consumed no toxics. His medical history was irrelevant and his physical examination was unremarkable except for the presence of a mass in his left upper quadrant of abdomen. No palpable lymph nodes and no splenomegaly were found.A complete blood count revealed mild leukocytosis . Coagulation study and biochemistry tests including liver, kidney and thyroid gland function, iron metabolism and tumour markers presented no remarkable alterations. The reactive C protein was of 42.7 mg/dl . Gastrin level was of 114 ng/ml . Secretin induced test at 15 min was of 322 ng/ml. VIP and 5-HIAA levels were normal. The stool exam was negative for bacterial and parasites.nd and 3rd segment of a 10 x 8 cm diameter and small bowel distension. A helical computed tomography scan and a magnetic resonance imaging confirmed the liver mass with contrast uptake  esophagitis and gastritis as well as hypertrophy of the gastric folds and multiple duodenum ulcers with unspecific biopsy and H. Pylori negative. Barium intestinal study showed the same findings, without any small bowel lesions.The gammagraphy with 111 In-DTPA octreotide showed pathologic uptake in the upper abdomen, especially at the jejunum wall, with high expression of serotonin receptors. A PET study was performed which showed high abnormal uptake in the liver mass as well as a diffuse uptake into the axils, small bowel wall and spleen. The study was completed by endoscopic capsule examination which did not reveal alterations at that level and with a complete colonoscopy without relevant findings. The echocardiography was normal.A left hepatectomy was performed and the pathologic diagnosis pointed out to a primary neuroendocrine tumour (carcinoid tumour) with poor mitotic activity and important necrosis. Inmunohistochemistry, on paraffin-embedded formalin fixed sections of the tumour, showed positive staining for synaptophysin, chromogranine, enolase, CK7  and negaDuring the follow up, the patient referred total disappearance of the initial clinical symptoms. Upper endoscopy revealed no duodenum ulcers and his serum gastrin levels decreased till 19 pg/ml two months after surgery.The patient remained free of symptoms two years after surgery.We report here a case of a primary hepatic carcinoid tumour (PHCT) associated with a Zollinger Ellison syndrome. The primary hepatic carcinoid tumours are uncommon  and reprCarcinoid tumours have their origin in neuroendocrine stem cells (known as enterochromaffin cells or APUD cells (Amine Precursor Uptake Decarboxilase) derived from the embrionary neuronal crest. Cells that migrated from the neuronal crest can give rise to gastrointestinal carcinoid tumours to foregut, midgut and hindgut, being the most common localization the appendix and the small bowel . Liver hSome theories for the origin of PHCTs have been proposed. The most accepted thories are: (i) ectopic cells of pancreatic or adrenal tissue, (ii) neuroectodermal origin cells, (iii) neuroendocrine programmed ectoblasts . It is bIn general, PHCT may vary in size, ranging from 1 cm to 20 cm  and theiThe clinical presentation is atypical, with symptoms like upper abdominal pain or discomfort like fullness, as well as diarrhoea or weight loss . Only a The radiology diagnosis has poor specificity as the appearance on ultrasound, CT or MRI is of a solid mass with cystic areas and hyperechoic or mixed pattern, with central or peripheral calcifications and fibrous scars. The majority are intense, hypervascularised tumours, which explains the contrast enhancement in ultrasound and CT images, in a similar fashion as the hepatocarcinoma. . When diThe gold standard for diagnosis is the liver biopsy since it may differentiate the lesion from other types of tumours. Cautions must be taken in order to avoid complications like carcinoid crisis as a recent case report shows . From thThe treatment is the surgical resection, when possible. Almost 85% of primary hepatic carcinoid tumour patients may have a resectable disease ,12. SurvIn our patient’s case, the immunohistochemistry result corresponded to a type A carcinoid tumour (APUDOMA). The moderate staining for gastrin pointed to gastrin secreting PHCT, associated thus with a Zollinger Ellison syndrome. In a recent review, gastrin was the most common hormone secreted of all cases (7/69) of primary hepatic carcinoid published till date .Primary hepatic carcinoid tumours are rare entities that sometimes may be associated with syndromes like ZES, carcinoid, etc. induced by neuroendocrine hormones secretion . An early and pertinent diagnosis offers the possibility of surgery resection, with a good 5 early survival rate and a poor relapse rate.


Primary pulmonary mucinous cystadenocarcinoma is a rare variety of lung cancer. It is characterized pathologically by copious mucin production predominantly in the extracellular space. This tumour has a remarkably favorable prognosis.We present imaging and histopathological findings of primary pulmonary mucinous cystadenocarcinoma presenting as a complex bronchocele in a 67-year-old Caucasian woman.Diagnosis of pulmonary mucinous cystadenocarcinoma should be considered in patients presenting with bronchocele that has suspicious imaging features, because the results of fine needle aspiration cytology and bronchoscopy are frequently inconclusive in these tumours. Positive emission tomography has an important role in helping to identify these tumours. Primary pulmonary mucinous cystadenocarcinoma (PMC) is a rare variety of lung cancer. It is characterized pathologically by copious mucin production predominantly in the extracellular space. This tumour has a remarkably favorable prognosis.We present imaging, which includes positive emission tomography (PET-CT), and histopathological findings of a primary PMC presenting as a complex bronchocele. Diagnosis of pulmonary mucinous cystadenocarcinoma should be considered in patients presenting with a bronchocele that has suspicious imaging features, since the results of fine needle aspiration cytology and bronchoscopy are frequently inconclusive.A 67-year-old Caucasian woman presented to our emergency department after she had collapsed. She was found to be hyponatraemic. Her medical history consisted of hypertension, previous fractured left neck of femur and bilateral breast implants for cosmetic reasons. She had a 50 pack year smoking history but had given up six months previously. She described a more insidious history of tiredness and weight loss of 2 kg over the preceding few months, although she denied having coughs or haemoptysis. Physical examination of all systems including her chest was unremarkable.Her chest X-ray showed a large soft tissue density lesion in the right hemithorax, which included her bilateral breast implants Figure . A compuA PET-CT scan was subsequently performed, which demonstrated fludeoxyglucose uptake within the medial side of the mass with an SUV of 4 to 5 Figure . No otheCurative surgery was not attempted in view of extensive multilobar involvement of the tumour and poor pre-operative lung reserve. The patient was referred to oncologists for radical radiotherapy with the possibility of chemotherapy.Pulmonary mucinous cystadenocarcinoma (PMC) now represents a distinct but rare clinico-pathological entity. It was first described in 1978 by Gowar -5]. The. The5]. PMCs are described as well-demarcated peripheral cystic tumours and are microscopically characterized by copious mucus production that is mainly extracellular and exhibiting only a small number of cancer cells in the periphery of the lesion.CT characteristics of PMC described in the literature include a uniform low attenuation cystic lesion with focal thickening and enhancement of the walls and septa . The radPMCs, as with most lung cancers, predominantly occur in older age , with a positive smoking history and presentation similar to bronchogenic carcinoma. However, the biologic behaviour of these tumours is remarkably favorable with excellent prognosis described after curative surgery , hence tThe pathogenesis of the bronchocele in this patient could have been due to a neoplastic lesion causing focal obstruction of the central bronchi leading to failure of the mucus drainage. Moreover, the tumour itself may cause an absolute increase in mucus production leading to stretching and destruction of airspaces and consequent cyst formation . This alIn summary, we presented a rare but recognized case of primary lung cancer. Diagnosis of primary PMC should be kept in mind when investigating a bronchocele with suspicious features, especially if bronchoscopy and fine aspiration cytology results are inconclusive. Moreover, this case illustrates the important role of PET-CT in helping to differentiate and identify areas that may be malignant within a tumour.PET-CT: Positive Emission Tomography - Computed Tomography; PMC: Pulmonary Mucinous Cystadenocarcinoma; SUV: Standardized Uptake Value.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.SAR and MC were involved in the radiological assessment of the patient. CA, DRL and MW were involved in clinical care. SAR wrote the radiological discussion contained in the manuscript. CA wrote the clinical presentation. MC, DRL and MW reviewed and edited the manuscript. All authors read and approved the final manuscript.


Gallbladder agenesis (GA) is a rare congenital anomaly of the biliary system often associated with other congenital abnormalities. Patients become symptomatic in 23% of cases. GA is often misinterpreted as other diseases, therefore, leading to unnecessary surgery. We report a case of congenital GA associated to cystic duct absence and a biliary tract abnormality diagnosed by Magnetic Resonance with Cholangiopancreatography. Gallbladder agenesis (GA) is a rare congenital anomaly of the biliary system. Reported for the first time in human beings by Bergman back in 1702 [The etiology of GA is unknown, it is often a sporadic occurrence with no clear causes. However, there are families in which the condition has occurred in several members, suggesting that there are familial hereditary forms of GA , 3.The agenesis is attributed to an abnormality in the embryonic development, so most cases of gallbladder agenesis are associated with other congenital abnormalities, including those of the bile system . It is pPatients become symptomatic in 23% of cases, and AG will almost always be misinterpreted as cholecystitis with cystic duct obstruction or as sclero-atrophic gallbladder, therefore, leading to unnecessary surgery . It is difficult to establish a correct preoperative diagnosis of GA in symptomatic patients because of the nonspecific nature of the symptoms.We report a case of congenital GA in an adult patient, associated to cystic duct absence and a biliary tract abnormality diagnosed by Magnetic Resonance with Cholangiopancreatography (MRCP). We also discuss the etiology and physiopathology of this abnormality and diagnostic tools employed.In our knowledge this is the first case of GA preoperatively correctly diagnosed by radiological imaging, in particular MRCP, described in the literature.A 44-year-old man was admitted to our attention for upper abdominal pain, bloating, and dyspepsia for the last 6 months. His blood pressure and pulse rate were regular and his body temperature was 36.7°C. Results of all hematological and biochemical investigations were within normal limits. Ultrasonography (US) examination did not visualize the gallbladder clearly. However, it demonstrated a hyperechoic area with an acoustic shadow in the gallbladder fossa, and this finding was suspected for sclero-atrophic to be contracted lithiasic gallbladder .MRCP was subsequently performed with a 1.5 Tesla magnet , equipped with a Master dynamic gradient system (30 mTm maximum power and 150 mTm/msec slew rate) using a phasedarray body coil. The patient underwent the MRCP study after fasting for 8 hours.The examination protocol consisted of an axial T1-weighted 2D FLASH and axial T2-weighted TSE sequences and axial SPIR to localize the acquisition volume for the MR Cholangiography sequences. We performed BALANCE sequences to obtain an accurate anatomical resolution and gallbladder fossa visualization. The MRCP study consisted of a 3D-MIP breath-hold acquisition of a single slice in the coronal plane, positioned so as to obtain complete visualization of the intra- and extrahepatic biliary tract, with a single-slab RARE sequence Figures and 3.The MRCP showed the absence of both the gallbladder and the cystic duct. There were no images of cystic lesions in the intrahepatic area, in the lesser omentum, in the retroperitoneal and retrohepatic areas, within the falciform ligament, or in the retroduodenal area compatible with condition of ectopic gallbladder . The intrahepatic bile ducts appeared normal with no images of stenosis or repletion defects. Moreover, MRCP demonstrated an anatomical variation of choledochopancreatic duct junction. In fact the bile duct was visualized up until the second part of the duodenum, in correspondence of the Major papilla. The Wirsung duct, on the other hand, was visualized until the initial part of the duodenum, in correspondence of the Minor papilla. Clinical history revealed familial gallbladder agenesis diagnosticated into 2 paternal aunts during laparoscopic surgery. The patient underwent medical treatment with complete control of symptoms.Anatomic anomalies of the biliary tract are not uncommon, but gallbladder and cystic duct agenesis is rare ; it is oThe prevalence range is 0.007–0.13%. The incidence of this malformation is slightly lower in surgical cholecystectomy series (0.007–0.027%) than that in autopsy reports .GA diagnosed during surgery has a female predominance of 3 : 1, while cases found in autopsies have an equal sex ratio . The gallbladder develops from the caudal part of the hepatic diverticulum in the fourth week of prenatal life. There are two theories regarding nondevelopment of the gallbladder , 14. AccGA occurs alone in 70–82% of cases (31.6% asymptomatic cases and 55.6% symptomatic cases). It occurs in association with additional malformations in the remaining 12.8–30% of cases, that fall into two subgroups: one with atresia of the bile ducts or choledochal cyst (9%), and the other with normal bile ducts but with distant multiple fetal anomalies (12.8–21%) –19.In newborns, AG is associated with one or more defects, sometimes incompatible with life. GA has been reported to be associated with many other gastrointestinal, skeletal, cardiovascular, and genito-urinary malformations, such as ventricular septal defect, imperforate anus, duodenal atresia, malrotation of the gut, pancreas divisum, hypoplasia of the right hepatic lobe, duplication cysts of the hepatic flexure, renal agenesis, undescended testes, and syndactyly .GA has no characteristic symptomatology, patients become symptomatic in about 23% of cases. Right upper quadrant abdominal pain is present in 90% of the cases, nausea and vomiting in 66%, fatty food intolerance in 37%, dyspepsia in 30%, and jaundice in 35% . The jauReviewing the literature, we noticed that, with the exception of two cases of GA, symptomatic patients are still unnecessarily operated on because the preoperative investigations carried out failed to demonstrate the exact diagnosis , 24. GA represents a difficulty for the surgeon , 26: durIf the diagnosis of GA is made during operation, the surgeon must prove GA by thoroughly examining the most common sites for ectopic gallbladder, which are intrahepatic, retrohepatic, on the left side, or within the leaves of the lesser omentum or within the falciform ligament, retroduodenal, retropancreatic, and retroperitoneal  . The abUltrasonography is actually the investigation method of choice for the diagnosis of common bile duct stones, with a sensitivity of 95–98%. Crade et al., defined three categories of abnormal ultrasounds of the gallbladder: shadowy gravity-dependent opacities within the gallbladder, nonvisualization of the gallbladder lumen, and nonshadowy opacities within the gallbladder lumen. The accuracy of US in these three different categories is 100%, 96%, and 61%, respectively . The greHowever, the examination conditions as well as the examiner's experience do not always permit such accurate appreciation. Shadowy opacities misdiagnosed as stones can be due to intestinal gas artifacts or to other structures in close proximity, such as a calcified hepatic lesion or a surgical clip .In our patient, the duodenum was probably misdiagnosed as sclero-atrophic or contracted lithiasic gallbladder.Endoscopic retrograde cholangiopancreatography (ERCP) has been used in addition to other diagnostic methods . HoweverMRCP is a noninvasive and well-demonstrated imaging method in the evaluation of the biliary tract –35. As iIn our case, MRCP allowed to make the correct preoperatively diagnosis with a noninvasive examination, avoiding unnecessary surgical exploration, and minimizing the risk of complications. Moreover, it provided accurate anatomical details about the bile tree conformation excluding the condition of ectopic gallbladder too.In conclusions, GA should be kept in mind whenever the gallbladder is improperly visualized in routine imaging methods in patients with biliary-type pain. MRCP technique may not yet replace ultrasound as the gold standard of acute gallbladder imaging but it revealed an ideal complementary study to inconclusive ultrasonographic studies. The correct preoperative diagnosis of GA is fundamental to avoid a needless surgical exploration, which might be risky.


Gallstone ileus following cholecystostomy has been reported once, in a patient with acute cholecystitis, where symptoms of small intestinal obstruction had developed one day after surgery. We report a case of gallstone ileus eight months following a cholecystostomy, which might deter the diagnosis. This is the only such reported case in medical literature according to our knowledge.A 54-year-old Sri Lankan female with a past history of a cholecystostomy presented with symptoms suggestive of small intestinal obstruction. Evidence of ileal obstruction with pneumobilia in the supine radiograph of the abdomen and cholecyto-duodenal fistula in the water soluble contrast study was suggestive of the diagnosis of gallstone ileus. An enterolithotomy was performed with no attempt of closure of the cholecysto-duodenal fistula.This case demonstrates the value of the supine radiograph of the abdomen and the barium follow-through in diagnosis. A cholecystogram, preferably preoperative, is the mainstay of prevention and identification of this clinical scenario. Gallstone ileus following cholecystostomy has been reported only once, in which the patient developed symptoms of intestinal obstruction in the same admission . The morA 54-year-old Sri Lankan female, with a past history suggestive of several episodes of acute cholecystitis, presented with central abdominal pain, distension and bilious vomiting for one day. She had a cholecystostomy during an attempt of an open cholecystectomy, due to obscured anatomy, eight months prior to presentation. The cholecystostomy tube was removed in ten days. However, a tube cholecystogram had not been performed.On examination, she was moderately dehydrated, afebrile and anicteric. Her pulse rate was 90 per minute. She was haemodynamically stable. Abdominal examination revealed distension. However, it was soft and non tender. Bowel sounds were exaggerated. Digital examination revealed an empty rectum with no palpable mucosal lesions. A provisional diagnosis of adhesive intestinal obstruction was made and initial management was planned accordingly.Haematological investigations did not have any evidence of sepsis, anaemia or electrolyte imbalance. A supine abdominal x ray demonstrated features suggestive of upper small bowel obstruction and pneumobilia. A water soluble contrast study was performed to determine the cause of pneumobilia and intestinal obstruction, which revealed evidence of a cholecysto-duodenal fistula and mechanical obstruction of the small bowel. However, there was no radio opaque mass within the small bowel. Pneumobilia and evidence of the cholecysto-duodenal fistula in the presence of small intestinal obstruction was suggestive of gallstone ileus .Following initial resuscitation with intravenous fluid, pain relief, nasogastric suction and monitoring, an exploratory laparotomy was performed. Peritoneal survey revealed multiple adhesions not amounting to intestinal obstruction with obscured anatomy in the right hypochondrium. A 6 × 4 × 4 cm stone was found impacted in the terminal ileum about 15 cm proximal to the ileo-caecal valve . EnterolCholecystostomy may be a life saving procedure for high-risk patients with acute cholecystitis or peri-cholecystic abscess. Occasionally it is indicated for cholelithiasis when cholecystectomy proves unduly difficult and potentially hazardous even in otherwise healthy patients ,4. A posGallstone ileus is mechanical intestinal obstruction due to impaction of one or more large gallstones. It is a potentially serious complication of cholelithiasis , which oDiagnosis of gallstone ileus is difficult and often delayed with 50% of cases detected only at laparotomy . The claThe mortality rate is in the order of 7.5%-15%, largely due to delayed diagnosis ,9. HenceThis article contributes to medical literature as the second reported case according to our knowledge, where gallstone ileus has presented in a patient following cholecystostomy. In contrast to the previous case, where symptoms appeared in the same setting one day after surgery, this patient presented eight months after cholecystostomy, which is unique. This report illustrates the importance of a postoperative cholecystogram, preferably per-operative. The cholecystogram would have revealed the cholecysto-duodenal fistula. Hence, we recommend exploration for a cholecysto-duodenal fistula by an on table cholecystogram or by examining the interior of the gallbladder during cholecystostomy. The presence of a fistula is an indication for careful examination of the intestine for a gallstone. In the absence of CT facilities, the radiograph and gastrografin study are diagnostic of gallstone ileus.