Malrotation in children is due to either an incomplete or non-rotation of the foetal mid-gut during perinatal development. Presentation is usually in the first few weeks of life, often with life-threatening volvulus and ischaemia. However, it can be a rare cause of abdominal pain in older children and young adults. We present such a case, as a reminder to emergency physicians that malrotation should be considered in the differential diagnosis of recurrent or chronic abdominal pain not only in children but also in adolescents. A 14-year-old boy presented to the paediatric emergency department (PED) with a 24-h history of intermittent right-sided abdominal pain and bilious (greenish) vomiting that had settled just prior to his arrival to the PED. His haemodynamic parameters were normal and clinical examination including an abdominal examination was unremarkable.H. pylori infection identified on stool antigen test and on hydrogen breath test almost 6 months prior to this presentation. He was awaiting an upper GI endoscopy because of his recurrent symptoms.His past medical history revealed that he had experienced at least four identical previous episodes of abdominal pain with vomiting that necessitated attending three different emergency departments over a 1-year period. On each occasion he had been admitted to a general paediatric or a paediatric surgical ward with provisional diagnoses of evolving appendicitis, but was discharged home as his blood results including inflammatory markers and abdominal ultrasound scans were normal. He had received quadruple therapy  for He was admitted to the paediatric ward on this presentation for evaluation of this recurrent abdominal pain and concerning bilious vomiting. He subsequently underwent an upper GI contrast study as an inpatient. The x-ray Figure  findingsMalrotation is the term used to define a spectrum of developmental abnormalities resulting in incomplete or nonrotation of the mid-gut. During foetal development between the 4th and 12th week of gestation, the mid-gut rotates anticlockwise around the axis of the superior mesenteric artery before assuming its final place in the abdomen and is fixed with the posterior abdominal wall. First described by Ladd in the early 20th century [The incidence of malrotation is reported at 1 in 500 , with moUp to 70% of cases are associated with other congenital abnormalities, such as gastroschisis, omphalocele, intestinal atresias, anorectal malformations and cardiac/hepatic abnormalities. Malrotation is rarely seen in older children, and when it does occur, symptoms may be absent or intermittent.The clinical presentation depends on the age of presentation and the location of the defect. Infants most frequently present with bile-stained emesis. Pain and irritability are not prominent clinical features, and the abdomen is usually soft and non-tender unless progression to ischaemia has occurred. At this point, the abdomen becomes distended and tender, and the emesis may be blood stained.Presentation later in life is vaguer, as our case illustrates. Making a diagnosis is often more difficult, because there is a larger spectrum of differentials to consider including annular pancreas, intussusceptions, duodenal web, etc. Symptoms often include abdominal pain and vomiting, although in a large proportion of case, the emesis is non-bile-stained. Gastro-oesophageal reflux, anaemia secondary to occult bleeding , malnutrPlain abdominal x-ray is normal in a simple malrotation. If there is a mid-gut volvulus then the classic radiographic finding is that of a double-bubble appearance where the first bubble is due to gastric dilatation and the second bubble is due to the dilatation of the first part of the duodenum. Abdominal ultrasound/CT scan  can be aInitial management in an emergency presentation would involve fluid resuscitation, NG tube placement ('drip and suck'), correction of electrolyte abnormalities  and administration of broad-spectrum intravenous antibiotics. Surgery is the treatment of choice as there is a high risk of vascular compromise and intestinal necrosis. A classic Ladd procedure is described as a reduction of the volvulus (if present), division of mesenteric bands, placement of small bowel on the right and large bowel on the left of the abdomen, and appendicectomy. An appendicectomy is carried out, partly because blood supply to the appendiceal vessels can be compromised, but also to prevent future diagnostic confusion as the appendix would lie in the left upper quadrant of the abdomen alongside the repositioned caecum. Ladd's operation is usually an open procedure; however, a modified laparoscopic technique has also been described ,11.Whilst mortality following Ladd's procedure remains low at 2%, this figure appears to be higher in those with intestinal ischaemia and even higher in the presence of intestinal necrosis/perforation and those with other co-morbidities . MorbidiMalrotation can present even in the adolescent age group, and emergency physicians must be aware of this condition. Recurrent/chronic episodes of abdominal pain with bilious vomiting must be thoroughly investigated and less common differentials considered.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.RM wrote the first draft of the paper. ST co-authored the first draft and reviewed all drafts and radiographic images. TJ reviewed and commented on all the drafts of the paper and radiographic images. All authors read and approved the final manuscript.


Hereby we report a case of well-differentiated intraosseous osteosarcoma in the sacrum. A 32-year-old woman was admitted to our hospital with a low echo-level mass in the pelvis searched by ultrasound in a routine physical examination. Radiographically, the mass was misdiagnosed as a benign bony tumor originating from the sacrum. The tumor was completely resected and pathological diagnosis was intraosseous well-differentiated osteosarcoma. Twelve months after operation, the patient was well and there was no evidence of recurrence and distal metastasis. This is a peculiar case of well-differentiated osteosarcoma involving an unusual site of the sacrum. The radiographic appearance and the differential diagnosis are discussed. We consider that dense trabeculated-like bone within an intraosseous solid mass might be suggestive of a well-differentiated osteosarcoma that was valuable in guiding the treatment and prediction of its prognosis. Well-differentiated osteosarcoma, although malignant, may be mistaken for a benign condition. Local excision has almost always been associated with recurrence. For this case, the patient had a wide excision and had no recurrence and metastasis. Therefore, it is very important to identify the radiological features and to distinguish this tumor from benign lesions and high-grade osteosarcomas before operation. Well differentiated osteosarcoma of the bone is rare, accounting for approximately 1.2% of all osteosarcomas . HoweverA 32-year-old woman was admitted to our hospital for a low echo-level mass searched by ultrasound in the routine physical examination 4 days before. The patient was asymptomatic. A full blood examination gave values within the normal limit. Plain film showed cortical thinning and discontinuity at the sacrum without reactive periosteal bone formation . ContrasThe tumor was resected completely and successfully. Pathological diagnosis was well-differentiated intraosseous osteosarcoma. Histologically, the tumor was composed of newly formed irregular bony trabeculae and hypocellular spindle cells with only minimal atypia in the fibrous stroma . Twelve Most osteosarcomas are highly malignant and hence, cytologically obvious. However, well-differentiated osteosarcoma is rare  and has a more benign, indolent course with less potential for metastasis and a higher survival rate compared with classic osteosarcoma , 4. ThisPatients with well-differentiated osteosarcoma tend to be older than those with conventional osteosarcoma. The mean age for well-differentiated osteosarcoma is in the third decade. In thisIn our case, the tumor margin was well defined and no cortical disruption was observed. In 18 patients with well-differentiated osteosarcoma, Unni et al.  reportedIn summary, we reported a case of well-differentiated osteosarcoma of an unusual location of the sacrum. Correct diagnosis of well-differentiated osteosarcoma probably affects the decision of local excision or wide excision and the prediction of the recurrence and the survival rate. We consider that dense trabeculated-like bone within an intraosseous solid mass might be suggestive of well-differentiated osteosarcoma that was valuable in guiding the treatment and prediction of prognosis.


Mayer-Rokitansty-Küster-Hauser (MRKH) syndrome is a Müllerian anomaly that presents with varying degrees of uterovaginal aplasia and is secondarily associated with cervicothoracic, auditory and skeletal anomalies. However, MRKH syndrome patients have normal and functional ovaries. A supernumerary ovary is an extremely rare form of an ectopic ovary and there are no reported cases of MRKH syndrome with cancer of the supernumerary ovary in the current literature. A 31-year-old female with a history of MRKH syndrome that was diagnosed 4 years previously presented with abdominal pain and a suspected malignant pelvic mass was identified. During the staging surgery, both ovaries were separated from the main mass, observed and removed. A third ovary was discovered in the pelvic mass and the diagnosis of primary ovarian cancer from the third ovary was confirmed by immunohistochemistry. We report the first known case of cancer of the supernumerary ovary in a patient with MRKH syndrome. Although both ovaries were confirmed to be normal in the patient with MRKH syndrome, we propose that an ovarian neoplasm should be considered in the diagnosis of a pelvic mass. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper two thirds of the vagina in females demonstrating normal development of secondary sexual characteristics and a normal 46, XX karyotype. MRKH may be isolated but it is more frequently associated with renal, vertebral and, to a lesser extent, auditory and cardiac defects. The first indication of MRKH syndrome is a primary amenorrhea in young females that otherwise present with the normal development of secondary sexual characteristics and normal external genitalia, with normal and functional ovaries .The supernumerary ovary is an ectopic ovary that is not connected to the utero-ovarian, broad or infundibulopelvic ligaments. This is one of the rarest gynecological abnormalities . A superA 31-year-old nullipara was admitted to the Korea University Anam Hospital, Korea University College of Medicine  with a 2-week history of intermittent pain in the lower abdomen and back. On ultrasound examination, an 11.8×8.3-cm cauliflower-like mass was noted on the left side of the pelvic cavity. A computed tomography (CT) scan revealed an ill-defined, irregular soft tissue mass with extensive calcification in the pelvic cavity.The patient had been admitted to our hospital 4 years previously with chief complaints of primary infertility and amenorrhea. On admission, the patient was observed to be age-appropriate with normal development of the breasts, pubic hair and external genitalia. The karyotype was normal 46, XX. A diagnostic laparoscopy revealed an absent upper vagina and two irregularly shaped uterine bodies positioned near each of the infundibulopelvic ligaments. Both ovaries were normal in appearance. A hypoplastic and non-functioning left kidney was diagnosed, as the left kidney was not visualized in the renal scan. Additionally, a scoliotic change in the thoracic spine was observed by a chest X-ray. After two months, the patient underwent an Abbé-McIndoe procedure.At the time of the current admission, the laboratory results were within the normal limits, with the exception of an elevated CA 125 level of 1,870.0 IU/ml. During surgery, a 25-cm pelvic mass was observed to occupy the left pelvic cavity. Multiple peritoneal seeding, omental invasion and rectal wall adhesions were detected. Following adhesiolysis, the bilateral ovaries and salpinges were demonstrated to be slightly enlarged, but normal in their contour and position. The uterus had enlarged since the previous surgery. A pelvic exenteration, omentectomy, sigmoid colon resection en-bloc, bilateral pelvic lymph node dissection and right external iliac node sampling were performed with a peritoneal washing cytology.Both ovaries exhibited normal histological features, but with numerous carcinoma cell implants on their surface. Furthermore, the pelvic mass itself contained areas of well-defined ovarian tissue with cystic follicles. This latter finding and the immunohistochemistry results revealed that a serous tumor had developed from a third ovary, as opposed to the mesothelium.The patient received post-operative chemotherapy. Sixteen months post-surgery, the patient was stable and did not demonstrate signs of recurrence.The en-bloc resected pelvic mass consisted of an ill-defined tumor mass, a segmentallyresected rectum and duplicate uterine bodies with attached bilateral adnexae . On dissHistologically, the main tumor in the peritoneum was a serous papillary carcinoma, characterized by the thin or complex papillary growth of cuboidal-to-columnar serous-type cells. Numerous psammoma bodies were noted . The tumBoth uteri had the proliferative phase of the endometrium, and adenomyosis was present in the right-side of the uterus.MRKH syndrome, first described by Mayer and further studied by Rokitansky, Küster and Hauser, is one spectrum of Müllerian anomalies originally characterized by the congenital absence of a uterus and vagina in genotypic and phenotypic females with a normal endocrine status ,5. MRKH In isolated MRKH syndrome (type I), the Fallopian tubes and ovaries are usually present, as well as variable degrees of uterine or vaginal aplasia. Type II MRKH syndrome includes other non-Müllerian anomalies, such as urinary tract and cervicothoracic disorders, and hearing defects . The evaDuring surgery, the pelvic mass was suggested to be a primary peritoneal carcinoma, as the ovaries had been defined to be morphologically normal. The primary peritoneal serous carcinoma was histologically indistinguishable from a primary ovarian serous tumor. We propose that malignant mesothelioma arising from the peritoneum ought to be distinguished from primary peritoneal Müllerian neoplasms. Immunohistochemical staining for mesothelial markers  excluded the mesothelial origin. The expression of ER and PR, as well as WT-1, favored a serous tumor of Müllerian origin.The distinction between a supernumerary and an accessory ovary is not always clear. In an extensive review of the literature in 1959, Wharton described the supernumerary ovary . The teret al(There are two proposed mechanisms for the formation of a supernumerary ovary . Pearl aet al. Supernuet al. The masFrom these mechanisms, we suggest that the supernumerary ovary tends to be situated in the posterior peritoneum, where it is hard to detect the normal-sized ovary without expectation, compared with the anterior peritoneum. This explains why the third ovary was not identified in the first diagnostic scope operation in the present case.In summary, we report the first case of cancer of the super-numerary ovary in a patient with MRKH syndrome. Although both ovaries were confirmed to be normal in the present patient with MRKH syndrome, an ovarian neoplasm should be considered in pelvic mass diagnosis.


Carcinomas of the appendix are exceedingly rare tumors and have an annual age-adjusted incidence of around 0.4 cases per 100,000. Appendiceal adenocarcinoma accounts for < 0.5% of all gastrointestinal neoplasms and, of these, mucinous adenocarcinomas account for the majority. Published accounts of familial instances of primary appendiceal tumors are strikingly rare. We report two siblings who both developed primary mucinous adenocarcinomas. A genetics evaluation was conducted to determine if there was a recognizable underlying single gene disorder; no DNA mismatch repair defect was evident, and no other diagnosis was apparent. A review of appendiceal cancers seen at Mayo Clinic from l997 to the present was conducted to search for additional familial cases. Among 316 cases of primary appendiceal cancer of any histologic type, this sib pair was the only family reporting a second affected family member. The occurrence of appendiceal cancer in siblings may represent a random occurrence. An exceedingly rare predisposition syndrome cannot be ruled out. Carcinomas of the appendix are rare tumors and have an annual age-adjusted incidence of 0.4 cases per 100,000 ,3. The dVery few reports have been published with familial appendiceal tumors. Anderson et al., presented a father-daughter case report in 1966. In this report, the ages at diagnoses were 42 and 15 respectively, and the histologies were reported as carcinoids of the appendix . BecauseA 69-year-old white male of Greek descent presented to his local hospital with abdominal pain and nausea and was diagnosed with acute appendicitis. During appendectomy, the surgeon observed an appendiceal tumor. The pathology report confirmed a grade II mucinous adenocarcinoma involving the mucosa and extending up to the serosa. The patient developed a postoperative infection leading to his transfer to our tertiary care facility. Colonoscopy was conducted and no polyps or other tumors were found. A laparoscopic right hemicolectomy was performed and a follow up examination two years later showed no evidence of recurrent disease.Two years later, the proband's sister, age 77 years old, presented with abdominal bloating and lower extremity edema. Pelvic ultrasound and CT scan revealed a pelvic mass, more than 23 centimeters in diameter. The CA-125 was elevated to 83.4 U/mL (reference range is < 35 U/mL) and the CEA level was 151.9 ng/mL (reference range < 3 ng/mL in adult nonsmokers such as this patient). Her brother, himself a physician, transferred her to our facility for gynecologic surgery consult. At the time of consultation, abdominal distention was so pronounced that she was unable to bend over and tie her shoes. Bimanual examination revealed a palpable pelvic mass which shifted uniformly across the abdominal cavity. A distinct adnexal mass was not palpable. Colonoscopy revealed only a 4 mm polyp in the transverse colon; pathology of the polyp indicated a tubular adenoma with low grade dysplasia. The patient elected to proceed to laparotomy. At laparotomy, the surgeons noted copious mucinous deposits as well as presence of a primary mucinous appendiceal adenocarcinoma that essentially replaced the entire right ovary. Due to the appendiceal tumor metastases, right salpingo-oophorectomy, ileoascending colon resection with side-to-side reanastomosis, and placement of intraperitoneal catheter were performed. A week postoperatively, a two-day course of 5-fluorouracil intraperitoneal chemotherapy was administered and completed.Gross pathology was notable for a well-differentiated multiloculated mass 19 cm at the greatest diameter. Histologic evaluation revealed a well-differentiated mucinous adenocarcinoma arising in the background of a serrated adenoma with mucocele formation. The appendix contained a 6.0 X 2.5 X 2.0 cm mass that had invaded into submucosa, and involved the subserosal and periappendiceal soft tissue. The ileum and cecum contained no diagnostic abnormality while the sigmoid colon revealed acellular mucin. Immunostaining was performed on the paraffin-embedded tissue using antibodies directed against CK7, CK20, CDX-2, and estrogen receptor. The neoplastic cells showed strong cytoplasmic positive staining for CK20 with nuclear positive staining for CDX-2 and no significant staining for CK7 or estrogen receptor. These data support the diagnosis and indicate a colorectal  primary.Because of the familial occurrence of these rare tumors in siblings, and because appendiceal cancer has been reported previously in Lynch Syndrome, this diagnosis was considered . From thPKD1, PDK2). A novel variant of unknown significance was found in PKD1, an A > G transition at nucleotide position 2427 (codon position 739) which causes an amino acid change from glutamine to arginine in PKD1. DNA mismatch repair genes  were sequenced and large rearrangement testing was conducted by multiple ligation probe assay (MLPA). The DNA repair genes were all normal . The summary of that data extraction is shown in Table In light of this sib pair concordant for appendiceal carcinomas and the paucity of literature on the subject, we conducted a retrospective chart review using the medical records at Mayo Clinic Rochester. Between l997 and July 2010, we identified 316 individuals with documented cancers of the appendix (314 plus the sib pair reported). We recorded the histologic type of cancer, the age at diagnosis, and looked for a family history of appendiceal cancer. The results of this chart review are shown in Table Very few case reports can be found in the literature regarding familial primary adenocarcinomas of the appendix. To our knowledge, the siblings reported here represent the first report of first degree relatives with appendiceal adenocarcinomas and the third report of familial appendiceal tumors ,7. The fOur efforts to find even a single additional familial case of appendiceal cancer among 314 Mayo cases were unsuccessful. While this was based only on a retrospective chart review, it suggests familial aggregation of appendiceal cancer is uncommon if not truly rare. We did document in the Mayo experience that adenocarcinomas are more common than neuroendocrine/carcinoid tumors of the appendix, in both men and women. This is in agreement with the SEER data. We also observed that the ages at diagnosis were older for the adenocarcinomas, and, for all appendiceal carcinomas, women were diagnosed on average about 5 years younger than men. We acknowledge the occurrence of these rare tumors within the siblings could be a result of chance alone but given the rarity of these tumors in the general population, the issue of familial predisposition seems still to remain a possibility. We were not able to systematically assess for cosegregation with any other type of cancer, given the nature of the chart review. Further investigation is warranted to explore potential hereditary predisposition.Written informed consent for use of the medical record was obtained from the patients for publication of this case report. A copy of the IRB documentation of authorization for use of the medical records for these two individuals is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AR did the primary literature research and drafted the manuscript. AP and MW provided clinical consultations, encouraged writing this report and provided final editing. NML consulted on the writing of the manuscript, audited some charts, reviewed the content, and edited the final product. KR performed statistical analysis and extracted the SEER data. All authors read and approved the final manuscript.


Metastatic phyllodes tumor has very few treatment options. Phyllodes tumor in metastatic setting has limited role of surgery, radiotherapy and chemotherapy or combined treatment. Most of the patients receive symptomatic management only. We present a case of metastatic phyllodes tumor managed with aggressive margin negative resection of primary tumor leading to palliation of almost all the symptoms, which eventually led to improved quality of life and probably to improved survival. The improved quality of life was objectively assessed with Hamilton depression rating scale. Surgery may be the only mode of palliation in selected patients that provides a better quality of life and directly or indirectly may lead to improved survival. The phyllodes tumor includes a group of lesions of varying malignant potential, ranging from completely benign tumors to fully malignant sarcomas. They are rare with an incidence of 0.3-1.0% of all breast neoplasms. Surgery 357A 57-year-old postmenopausal woman presented with a 3 month history of recurrent lump  in left breast of 30 × 25 × 15 cm in size with bossalated surface and profuse foul smelling discharge from necrotic surface ulcer and off and on fever with occasional chills . PatientOn examination, patient was anemic with average general condition. Her core biopsy was suggestive of malignant phyllodes tumor. CECT of thorax was suggestive of solitary pulmonary metastasis in lower zone of right lung. She was planned for a staged surgery . As per planning, she underwent radical wide excision with 2 cm margin which included total mastectomy with excision of pectoralis major and lateral border of latissimus dorsi muscle. Medial based thoraco-abdominal rotation advancement flap used for local wound cover . Post opet al. showed that four features  were predictors for the development of metastases. Of these, stromal overgrowth is the most reliable predictor. Tumor size was also an important prognostic variable in determining the overall survival. Fou et al. suggested a 75% mortality in metastatic phyllodes with mitotic index >10 per hpf.[et al. from Japan have shown just 11% 2 year survival in their review of 15 patients of metastatic phyllodes tumor, and in their case report, the patient survived just 5 months.[et al. in 2006 reported that, no clinical trials were available to assess the best treatment regimen for metastatic phyllodes, experience with systemic therapy for metastatic phyllodes tumor is anecdotal.[et al. to even cause complete remission but there are a few case report.[11et al. was effective in improving local control after surgery in malignant phyllodes, but its role in metastatic setting has not been described.[The present patient is a case of metastatic phyllodes with all the features of poor prognosis. Hawkins 5 months. Asogolu report.11 Ifosfamireport.11 but its Metastatic phyllodes tumor with such dismal prognosis, the tendency is to offer only symptomatic and supportive care with no significant role of surgery in such setting. But in the present case surgical resection relieved the patient of pain . Surgical resection has lead to -Improvement in psychological state of patient as shown by HDRS score which decreased to 3 from 15, also there was a generalized mood upliftment.Patient was relieved of her physical pain and her chronic inflammatory state due to the presence of tumor, which was necrotic with infection and discharging foul smelling purulent sanguinous fluid. She became afebrile with increased appetite and improved her nutritional status and gained weight.Improvement also occurred in social and spiritual aspects. Patient on her follow up visit described the joy of being back to her social life and she felt herself being freed of a long standing curse.et al. have improved survival with radical resection in metastatic breast cancer,[Surgery till date is considered to be futile in metastatic malignancies, but the impact of local control achieved by surgery in metastatic phyllodes tumor has lead to a better quality of life and is hypothesized to improve survival. The present case also highlights the fact that surgery should aggressive, the patient had underwent surgery twice before she presented to us which seemed to be marginal excision with futile results, it was only after radical surgery that adequate local control was achieved and quality of life improved. Steyaert t cancer, these faHypothesis: Aggressive surgical resection in the patient discussed may lead to improved survival by improvement in physical and mental well being leading to improved nutritional and immunological status to be eligible for chemotherapy. The role of chemotherapy in improving survival in metastatic phyllodes is still to be defined but drugs such as ifosfamide and doxorubicin may have a role.With the advent of cancer stem cells and their role in metastasis, the remoSurgical resection may not have a proven role in metastatic phyllodes tumor treatment. But in some situations, it may be the only modality for palliation and provide a better quality life to the patient and directly or indirectly may lead to improved survival."Everyone has a right to good quality of life, however short it may be."


Papillary seromucinous adenocarcinoma of the sinonasal tract is exceedingly rare. The objectives of this case report are to describe a case of papillary seromucinous adenocarcinoma presenting in the nasopharynx and to review the literature pertaining to other similar cases.A review of the patient's chart and a review of the English literature were conducted.We describe the case of a 64 year-old woman who presented with a 3-year history of epistaxis and right-sided otitis media with effusion. The patient had been followed for a known nasopharyngeal mass that had twice been biopsied and in both cases was considered a benign mass pathologically. A third biopsy was diagnosed as a low-grade papillary seromucinous adenocarcinoma. The patient was otherwise asymptomatic. The patient was referred to a multidisciplinary cancer clinic at which endoscopic resection was determined to be the preferred treatment modality. A literature review and approach to patients with nasopharyngeal masses will be presented.Papillary seromucinous adenocarcinoma is a rare tumor that can present in the nasopharynx. We describe the endoscopic surgical management of one such patient that presented to our care. The nasopharynx accounts for less than 1% of malignancies in the United States. The most common malignancy in the nasopharynx is Nasopharyngeal Carcinoma (NPC), which is a form of squamous cell carcinoma (SCC) with a histopathology and behavior unique from SCC originating elsewhere in the head and neck [A common problem with nasopharyngeal malignancies is that patients can often present without local symptoms; rather their primary tumor will be detected during the workup of a metastasis to a cervical lymph node . Tumors We present the case of a 64-year-old woman with a rare nasopharyngeal malignancy of salivary origin, that being papillary serous adenocarcinoma.A 64 year-old woman presented with a 3-year history of epistaxis and right-sided otitis media with effusion. She had no history of nasal obstruction, dysphagia, odynophagia, voice change, vision change, diploplia, weight loss, or B-symptoms. There was no prior history of smoking or radiation and she had minimal alcohol consumption. Prior to referral she had twice undergone biopsy of the lesion and this was first reported as a benign growth in the nasopharynx. A third biopsy was then diagnosed as a low-grade seromucinous papillary carcinoma, for which she was referred to our center for definitive management.Physical examination of the patient was remarkable for a right-sided tympanostomy tube and 0.5-1 cm lesion derived from the right Eustachian tube. The lesion had a fleshy papillomatous appearance. Examination of cranial nerves III-VII and IX-XII was unremarkable as was examination of the oral cavity. The patient had no palpable lymphadenopathy (Figure A CT scan of the head and neck had been performed. The scan was normal in appearance with notable absence of bony erosion or involvement of adjacent structures. There was no cervical adenopathy. MRI demonstrated thickening of the right Eustachian tube orifice and confirmed that it did not involve adjacent structures such as the parapharyngeal space Figure .The patient was assessed by a Multidisciplinary Head & Neck Oncology team, wherein primary surgery via endoscopic resection was deemed the best first-line treatment. This decision was made in consideration of the small size and minimal growth of the tumor over a 3-year period, its pedunculated nature, the absence of involvement of surrounding structures and patient preference to avoid chemoradiation or more invasive open surgical approaches. For access to the lesion, a corridor was created in the nasal cavity, including development of binasal access via posterior septectomy. The tumor was visualized at its origin at the Eustachian tube orifice. The resection began with dissection of the mucosa covering the face of the sphenoid. This developed a superior plane to provide access to the tumor and expose the pterygoid wedge. The wedge was followed inferolaterally to expose the skull base, pterygoid wedge and medial pterygoid plate. The plate mucosa was stripped for an anterior tumor margin.A linear cut was made in the posterior aspect of the torus tubarius anterior to the fossa of Rosenmuller to establish a posterior margin. The levator palatini muscle was incised inferiorly where it joined into the torus tubarius for an inferior margin. All soft tissue over the medial pterygoid plate was removed, exposing the medial pterygoid muscle, forming the lateral resection margin boundary. We then used careful cautery to form deep cuts, peeling tumor off the underlying surface of the skull base until we came to our posterior cut. The depth of dissection was approximately to the bony isthmus of the Eustachian tube. The specimen was friable and so it was removed as 2 large pieces. Frozen sections were deemed to be impractical. The surgical margins were at the level of the petrous carotid and pterygoid plates, and the tumor appeared to be completely removed at a gross level.Final pathology was performed on the two resected fragments. The medial fragment consisted of reactive respiratory muscosa with chronic inflammation, abundant fibrinous hemorrhage and necrosis; no viable tumor was seen. The deeper specimen contained fragments of a well-differentiated papillary neoplasm consistent with low grade papillary seromucinous adenocarcinoma, as previously diagnosed, the histology having been compared to that of the previous biopsy Figure .An MRI was performed 6 months post-operatively to reevaluate the operative site. There was no evidence of a lesion in the right Eustachian tube or nasopharynx. She was seen in clinic the following week. She had no symptoms of recurrence and the nasopharyngeal mucosa and Eustachian tube orifice were unremarkable on nasopharyngoscope. There was no cervical lymphadenopathy. The patient will be followed with serial physical examination and MRI to monitor for recurrence.Salivary gland tumors account for less than 5% of head and neck neoplasms and typically present in the oral cavity . The likPapillary serous adenocarcinoma is a histologically unique form of adenocarcinoma. This tumor is considered to be derived from nasopharyngeal surface epithelium rather than from underlying minor salivary glands, based on histological appearance and immunohistochemical staining profile. It is uncommon, occurring over a wide age range with no sex predilection. The commonest site is lateral or posterior wall or roof of the nasopharynx, and it presents as a soft to gritty mass with a nodular or papillary appearance. Despite a benign histological appearance, this tumor is infiltrative and tends to recur if not completely removed.While major salivary gland tumors have their own staging system, minor salivary gland tumors are staged based on their anatomic subsite. The AJCC Cancer Staging Manual classifies the T stage of nasopharyngeal tumors based on the presence or absence of involvement of surrounding structures such as the parapharyngeal space, skull base, paranasal sinus, cranial nerves, hypoharynx, orbit, infratemporal fossa or masticator space . Like otMultiple treatment modalities exist for the treatment of salivary gland tumors of the nasopharynx . GeneralTo our knowledge endoscopic resection of nasopharyngeal salivary gland tumors is not yet a common practice. Since the majority of patients with nasopharyngeal malignancies present with spread to cervical lymph nodes and/or the parapharyngeal space, less invasive techniques may not be considered. Primary endoscopic approaches to other nasopharyngeal neoplasms have been described with increasing frequency. Localized benign lesions such as juvenile nasal angiofibromas can be endoscopically resected, typically after angioembolization ,12. FurtThere is a paucity of literature that specifically pertains to the endoscopic resection of exocrine gland neoplasms of the nasopharynx, particularly as a primary therapy modality. Al-Sheibani et al. described their 4-handed endoscopic endonasal transpterygoid nasopharyngectomy in a retrospective study of twenty patients. Most patients in this study had advanced or recurrent disease. Among other tumors, 5 adenoid cystic and 2 adenocarcinomas were described with disease-related mortality rate of 20% and 50% respectively at the time of publication .The low staging of our patient’s disease factored into our decision to pursue endoscopic resection. The absence of parapharyngeal extension or invasion of adjacent structures made the approach possible. Furthermore, the pedunculated nature of the lesion facilitated resection in that it made the tumor readily distinguishable from surrounding tissue. We were confident based on imaging and our exam findings that we would not only be able to resect the tumor but too achieve sufficient normal tissue margins with an endoscopic approach. Another factor favoring this approach was the indolent nature of this tumor, progressing minimally over a 3-year period. Our patient will require close surveillance and while we are confident that our resection was adequate, if the tumor recurs it should be detectable by physical examination.This case is interesting because it represents a rare pathological variant of an uncommon nasopharyngeal tumor. Additionally, from our review of the literature, minor salivary gland tumors of the nasopharynx appear to be derived from the mucosa of the nasopharynx itself. However, in the case of our patient, the lesion in question was in fact a pedunculated lesion that appeared to originate from within the Eustachian tube. Finally, the management of patients such as ours remains controversial. Traditionally surgical resection of nasopharyngeal malignancies has been performed by an open approach, but this paradigm appears to be evolving. In this case, a minimally invasive resection was achieved with no evidence of residual disease. This was made possible by the small size of the lesion and the lack of invasion of surrounding structures.Written informed consent was obtained from the patient for publication of this report and any accompanying images.The authors declare that they have no competing interests.All authors read and approved the final manuscript.


Angioleiomyoma is an uncommon benign mesenchymal neoplasm that originates from smooth muscle cells and contains numerous thick-walled blood vessels. Here, we are presenting a case report of a huge broad ligament angioleiomyoma because of its rarity. Angioleiomyoma is an uncommon benign mesenchymal neoplasm that originates from smooth muscle cells and contains numerous thick-walled blood vessels. It is usually found in the skin of lower extremities but is extremely rare in broad ligament.A 45-year-old multiparous female presented with abdominal pain and lump since 2 months. No history of menstrual irregularities was noted. Sonography (USG) and Contrast Enhanced Computerized Tomography (CECT) showed a large homogeneous solid mass in the lower abdomen. The mass was hypodense in attenuation and showed mottled heterogeneous enhancement.Pre-operatively, the mass was seen arising from right broad ligament, deriving its blood supply from omental blood vessel. However, uterus and bilateral adnexa were normal and separate from the tumor. Mild ascitis was also seen. Only the tumor excision was performed.We received a single large encapsulated mass measuring 25 × 10 × 10 cm for histopathologic examination. External surface was smooth and congested, and dilated blood vessels seen. Cut surface showed predominantly solid mass, gray white in color, having multiple dilated and congested blood vessels, giving it a multicystic appearance.Hematoxylin and eosin stained section showed well-encapsulated mass comprising spindle-shaped smooth muscle cells arranged in fascicles intermingled with many thick-walled dilated and congested blood vessels. The inner layer of vessel wall was arranged in a circumferential fashion and the outer layer of these smooth muscle cells swirled away from vessel wall merging with surrounding tissue. No mitotic figure and necrosis was observed. Areas of myxoid and hyaline degeneration were also present.Van Gieson’s elastic stain (VGE) showed disruption in internal elastic lamina of thick-walled blood vessels. On immunohistochemistry, these tumor cells were positive for SMA, CD31 and CD34, thus confirming the diagnosis of angiolieomyoma.et al, 375 of 562 occurred in the lower extremities, 125 in the upper extremities, 48 on the head and 14 on the trunk. Most of these cases were less than 2 cm in diameter.[3Angioleiomyoma is a benign mesenchymal neoplasm consisting of smooth muscle cells and thick-walled blood vessels commonly found in the skin of extremities and rarely occurs in uterus. In the sThe tumor on gross appearence is whorled dilated blood vessels, giving it a multicystic appearance, as seen Histologically, angioleiomyomas are described into three subtypes: Capillary or solid, cavernous, venous or mixed. Our case was of capillary subtype.Immunohistochemical markers for smooth muscle cells such as smooth muscle actin (SMA) and vessel marker CD34 and CD31 are essential to differentiate angioleiomyoma from other tumors such as angiofibroma, angiomyolipoma and angiomyfibroblastoma. All these tumors are positive for vimentn and desmin but negative for smooth muscle actin. Our caseDegenerative changes in the angioleiomyomas are due to ischemia and the types of degenerative changes depend on the degree and rapidity of the onset of vascular insufficiency. Hyaline changes are the commonest form of degeneration. We present this case because of its extreme rarity and large size of the tumor.Treatment of choice is tumor excision open or laparoscopic, as none of the cases reported by Duhig and Ayer develope


We report two cases of focal nodular hyperplasia in patients following gadoxetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging confirmed with histopathology. These cases showed an atypical pattern during the delayed-hepatobiliary phase after the injection of gadoxetic acid. One case showed a total defect, and the other showed a peripheral ring-like enhancement without a visible central scar, mimicking hepatocellular carcinoma. The pathologic examination demonstrated that the two lesions were focal nodular hyperplasia. Focal nodular hyperplasia (FNH) is the second most common benign lesion of the liver after hemangioma . BecauseA 29-year-old man with underlying chronic hepatitis B visited our out-patient hospital clinic. His serum alpha-fetoprotein level was within normal limits . An abnormal 1.3 cm mixed echoic hepatic nodule was found in the right lobe incidentally on routine screening ultrasonography. The hepatic nodule had a peripheral hypoechoic halo, mimicking a malignant nodule. Multi-detector computed tomography (MDCT) was performed to characterize the focal liver lesion. The hepatic nodule showed complete enhancement during the early arterial phase of MDCT that then faded to iso-attenuation during the portal venous phase and the delayed phase without definite washout of the contrast enhancement. We proposed several possible diagnoses for this incidental nodule, including a well-differentiated hepatocellular carcinoma (HCC) and a high-grade dysplastic nodule due to the background of chronic hepatitis. FNH and hepatic adenoma were also included in the differential diagnosis. On T2-weighted imaging (T2WI) of MR, the nodule showed a high signal intensity (SI) and mild diffusion restriction on diffusion- weighted imaging  and apparent diffusion coefficient imaging. On gadoxetic acid-enhanced MR imaging, the mass demonstrated early intense homogeneous enhancement and subtle peripheral washout during the portal venous phase and ring-like peripheral enhancement with a central washout during the equilibrium phase and hepatobiliary phase  showing homogeneously intense enhancement during the early phase, subtle washout during the delayed phase and a low signal perfusion defect during the hepatobiliary phase . We consFNH is found in approximately 8% of all primary liver tumors, making it the second most common lesion after hemangioma . The preOn SPIO-enhanced T2WI MR images, FNH is hypointense compared with the normal liver, due to phagocytosis by the reticuloendothelial systems (Kupffer cells), and the central scar is more prominent. Atypical findings of FNH have been reported to include hemorrhage, necrosis, fat accumulation, surface retraction, rapid contrast washout, and the absence of a central scar , 13, 14.


Patients with cirrhosis-associated hepatocellular carcinoma (HCC) rarely present with acute pancreatitis (AP) and obstructive jaundice as the main clinical features. AP with obstructive jaundice caused by common bile duct embolism (CBDE) is very rare.A 54-year-old man with CBDE was misdiagnosed with common bile duct stones three times over a 7-month period. Investigations during this time did not identify CBDE. Surgical exploration was performed because of AP, obstructive jaundice, and a tumor in the left lobe of the liver. CBDE from the hepatic tumor was diagnosed by intraoperative biopsy and frozen section examination. The patient underwent left hemihepatectomy, cholecystectomy, and bile duct exploration.Preoperative diagnosis of CBDE is difficult because of the rarity of the condition, lack of physician awareness, and easy misdiagnosis on imaging examinations. Early and accurate diagnosis of this condition is important. Patients with cirrhosis-associated hepatocellular carcinoma (HCC) rarely present with acute pancreatitis (AP) and obstructive jaundice as the main clinical features. AP with obstructive jaundice caused by common bile duct embolism (CBDE) is very rare. Preoperative diagnosis of CBDE is difficult because of the rarity of the condition, lack of physician awareness, and easy misdiagnosis on imaging examinations. We report a rare case of CBDE causing AP with obstructive jaundice, to raise awareness of the importance of early and accurate diagnosis.A 54-year-old man with type 2 diabetes mellitus and liver cirrhosis secondary to hepatitis B virus infection was admitted to the Department of Gastroenterology at Clinical Medical College of Yangzhou University, Jiangsu Province, China, on 21 February 2011 because of abdominal pain. The patient had a 3-day history of yellow conjunctivae and urine. On 22 February 2011, abdominal magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) showed a maximum common bile duct (CBD) diameter of 13 mm with an area of heterogeneous intensity signals in the distal portion of the CBD suggestive of CBD stones , with areas of dense, spotty echoes referred to as muddy stones, and the left intrahepatic bile duct was also slightly dilated. Abdominal MRI and MRCP on the day after admission showed cholecystitis, gallbladder stones, a mass in the left lobe of the liver with unclear margins that was isointense on short inversion time inversion recovery sequences , DBIL (136.1 μmol/l), TBIL (141.2 μmol/l), AST (223 U/l), ALT (366 U/l), and CA19-9 (2041 U/ml); a high urinary amylase level ; and a normal serum AFP level (6.36 ng/ml). The patient had acute epigastric pain, fever, and jaundice. The high serum amylase level and abdominal CT findings indicated a diagnosis of AP.Because the pancreatitis was not severe and the patient’s condition gradually improved, emergency endoscopic retrograde cholangiopancreatography (ERCP) was not performed. After the pancreatitis was stabilized, surgical exploration of the abdomen was performed on 10 October 2011. Because of the patient’s history of liver cirrhosis secondary to hepatitis B virus infection, there was a high probability that the liver mass was hepatocellular carcinoma. We planned to perform cholecystectomy and bile duct exploration, with intraoperative diagnosis of the nature of the liver mass. Intraoperative exploration showed nodular cirrhosis of the liver, an edematous gallbladder, a large number of muddy gallbladder stones, and an unencapsulated tumor in the left lobe of the liver measuring 45 × 35 × 25 mm. A soft grayish-white embolus (45 × 11 × 9 mm) was extracted from the distal CBD, and grayish-white tumor debris was observed in the left intrahepatic bile duct. Intraoperative biopsy and frozen section examination showed HCC with tumor CBDE. Left hemihepatectomy, cholecystectomy, and bile duct exploration were performed. Postoperative pathological examination of the surgical specimen confirmed moderately differentiated HCC in the left lobe of the liver and the CBDE. The surgical margins were free of tumor cells.The serum CA19-9 level decreased to 81.20 U/ml by the 13th day after surgery, and the patient was discharged from hospital on the 35th day after surgery. The patient subsequently died of liver failure in another hospital on 12 February 2012.After reviewing the case, we consider that this patient was misdiagnosed prior to surgical exploration. The EPBD report describes extraction of muddy stones from the CBD. The report of the MRCP performed 61 days later also describes stones in the CBD. However, it is unlikely that so many muddy stones would have formed during this short time. Surgical exploration eventually revealed that the structures thought to be muddy stones were actually CBDE from HCC. These malignant emboli may have been misdiagnosed as muddy stones during EPBD because of their grayish-white, friable, loose appearance.The factors that may have contributed to the initial misdiagnosis were: 1) absence of a visible liver mass in the early stage of disease; 2) a normal plasma AFP level; 3) similar morphological characteristics between tumor emboli and muddy stones, because of absence of a blood supply ; 4) the rarity of tumor emboli causing AP, compared with other causes of AP such as metabolic abnormalities, obesity, genetic susceptibility, and the two most common causes: gallstones and alcohol-related disease; and 5) cholecystitis associated with muddy stones in the gallbladder, leading to the assumption that AP was caused by stones in the CBD.To our knowledge, there are very few reported cases of AP with obstructive jaundice caused by CBDE from cirrhosis-associated HCC. The cause of CBD obstruction was misdiagnosed three times, mainly because of the lack of experience of the clinicians with CBDE and the rarity of this condition. Preoperative diagnosis is difficult, because of the rarity of the condition, lack of physician awareness, and easy misdiagnosis on imaging examinations. However, the imaging tools available for diagnosis of HCC have recently improved.et al. reported HCC emboli in the CBD of a patient in whom no primary hepatic tumor was found on preoperative CT or surgical exploration [At the time of the patient’s first visit, there was no sign of the liver mass on MRCP. We speculate that there was probably a small HCC at that time, which may have ruptured into or invaded the intrahepatic bile ducts, resulting in accumulation of tumor debris and emboli in the CBD. Buckmaster loration . If CBDEloration  and CA19Contrast-enhanced CT findings are useful for differentiating between CBDE and a CBD stone. In our case, the contrast density of the CBDE was low in all phases of the scan, ranging from 15 to 22 HU, whereas the contrast density of CBD stones is usually >50 HU. When the contrast density of a filling defect in the CBD is ≤50 HU, CBDE should be considered.The prognosis of this type of HCC is generally poor, but is better than for HCC with hepatocellular jaundice. Jaundice does not necessarily indicate advanced disease, and is not always a contraindication to surgery. Patients with HCC and obstructive jaundice due to CBDE may benefit greatly from surgery. Early and appropriate treatment of such patients may achieve satisfactory palliation, as well as occasional long-term resolution of symptoms and survival -5.In conclusion, we have described a very rare case of HCC presenting as AP with obstructive jaundice. Preoperative diagnosis is difficult, because of the rarity of the condition, lack of physician awareness, and easy misdiagnosis on imaging examinations. It is important to pay attention to the above-mentioned clinical data to obtain early and accurate diagnosis of this condition.Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AFP: Alpha-fetoprotein; ALT: Alanine aminotransferase; AP: Acute pancreatitis; AST: Aspartate aminotransferase; CA19-9: Carbohydrate antigen 19–9; CBD: Common bile duct; CBDE: Common bile duct embolism; CT: Computed tomography; DBIL: Direct bilirubin; EPBD: Endoscopic papillary balloon dilation; ERCP: Endoscopic papillary balloon dilation; HCC: Hepatocellular carcinoma; HU: Hounsfield unit; MRCP: Magnetic resonance cholangiopancreatography; MRI: Magnetic resonance imaging; TBIL: Total bilirubin.The authors declare that they have no competing interests.


A tumor shadow was identified in the chest X-ray of a 40-year-old Korean man and he was referred to our hospital. The computed tomographic (CT) scan of his chest showed a 3-cm rounded pleural-based mass lesion with calcification, which was growing into the intercostal muscles. Thoracoscopic surgery was performed to resect the tumor. From the histological findings, the tumor was diagnosed as an intramuscular lipoma. The patient displayed no evidence of recurrence for more than 18 months. As well-circumscribed type of intramuscular lipoma is a rare tumor, we report this case with a literature review in this paper. Lipoma is the most common form of soft tissue tumors. Intramuscular lipoma accounts for less than 1 percent of all the lipomas diagnosed ,3. We heA 40-year-old Korean man presented with a pulmonary nodule, which was detected by chest radiographs, and visited our hospital. He did not show any subjective symptoms. A 3-cm round mass lesion in the right upper lung field was seen in his chest X-ray Figure , and theLipoma, the most common form of soft tissue tumors, is composed of mature adipose tissue. Benign lipomatous tumors are divided into nine subtypes: lipoma, lipomatosis, lipomatosis of nerve, lipoblastoma/lipoblastomatosis, angiolipoma, myolipoma of soft tissue, chondroid lipoma, spindle-cell lipoma/pleomorphic lipoma, and hibernoma . IntramuThe diagnosis of intramuscular lipoma is suggested when a painless, slow-growing, well-demarcated lesion occurs in the chest, although this type of lesion is rare. The liposarcoma that has a same histologic and clinical appearance must be excluded and surgical excision should be carefully performed. Patient follow-up is of critical importance due to its high recurrence rate.Written informed consent was obtained from the patient for publication of this case report and the 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.JHL, DHD, and JCL wrote the draft of the manuscript and obtained the written consent. JHL and DHD performed the literature review and participated in the manuscript writing and helped to the final writing of the paper and gave final approval of the manuscript. JHL performed the manuscript review and participated in the manuscript revision. All authors have read and approved the final manuscript.


Iatrogenic damage to the seminal tract is one of the causes of obstructive azoospermia, which can be an indication for reconstruction surgery. We present a case of obstructive azoospermia as an unusual complication after neonatal herniorrhaphy of an omphalocele.A 30-year-old Japanese man was diagnosed with obstructive azoospermia. He had undergone herniorrhaphy of an omphalocele immediately after birth. Reconstruction surgery of both seminal tracts was performed to pursue the possibility of naturally achieved pregnancy. Intra-operative findings demonstrated that both vasa deferentia were interrupted at the internal inguinal rings, although the abdominal end of the right vas leading to the seminal vesicle was found in the abdominal cavity. The discharge from the stump of the testicular end had no sperm, although the right epididymal tubules were dilated with motile sperm. Therefore, we performed right-sided vasovasostomy in the internal inguinal ring and ipsilateral epididymovasostomy simultaneously.To the best of our knowledge, this is the first report describing obstructive azoospermia as an unusual complication of herniorrhaphy of an omphalocele. It is important to pay attention to the existence of seminal tracts in such surgery as well as in inguinal herniorrhaphy. Obstructive azoospermia is one cause of male infertility. Obstruction of the seminal tracts can be caused by iatrogenic damage, by a congenital anomaly or by infectious diseases . AlthougA 30-year-old Japanese man who had undergone herniorrhaphy for an omphalocele immediately after birth was referred to our hospital. He presented to our hospital with 1 year of infertility after his marriage. Multiple semen analyses revealed azoospermia, but a scrotal examination revealed no abnormalities in the testes or in the vas deferens or epididymis on both sides. Magnetic resonance imaging of the scrotum revealed no abnormal findings in the seminal vesicles, the prostate or the ejaculatory ducts. An endocrinological examination demonstrated that his serum follicle-stimulating hormone, luteinizing hormone and testosterone levels were within the normal range . The surgical scar from his omphalocele repair was found in the midline of the abdomen Figure . The abdAzoospermia is one of the causes of male infertility. Azoospermic patients whose testes show normal spermatogenesis on the basis of histopathology are diagnosed as having obstructive azoospermia. Cases of obstructive azoospermia are classified into two groups according to the obstruction sites. One is an obstruction in the vas deferens, and the other is one in the epididymis. The causes of epididymal obstruction include infectious diseases and congenital anomalies , and theOmphalocele is a disease of neonates involving the herniation of intra-abdominal organs because of an abdominal wall defect, and emergency operations are generally required in most cases. The reported escaping organs include the small intestine, liver, bladder and ovary . There iin vitro fertilization (IVF) in cases of severe male factor infertility, there has been a trend toward pursuing IVF, including testicular sperm extraction ICSI, as a first-line therapy for obstructive azoospermia [The vasa deferentia are very thin in neonates . This miospermia . This trThe case of our patient indicates that herniorrhaphy of an omphalocele can be an iatrogenic cause of obstructive azoospermia. This is the first report describing obstructive azoospermia as an unusual complication of this surgery. It is important to pay attention to the seminal tracts in herniorrhaphy of an omphalocele, as well as in inguinal herniorrhaphy.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.KT, TS and HN performed the surgical procedure and reported the case. KY and AK interpreted and analyzed the findings. OO participated in the diagnostic and therapeutic decisions. KO and KI made major contributions to the writing of the manuscript. All authors read and approved the final manuscript.


Ameloblastic fibroma (AF) is an uncommon mixed neoplasm of odontogenic origin frequently seen in the second decade of life. It mainly presents as an intrabony lesion but can even occur peripherally. Histologically, our case showed hypercellular areas, an uncommon feature seen in typical AF. Whether this benign lesion is treated by mode of enucleation and curettage or by extensive surgery is still a topic of debate. An extensive surgical treatment is suggested as the initial approach due to its high recurrence rate (18%) and the greater chances of recurrent AFs transforming into ameloblastic fibrosarcoma (45%), together with a long-term followup. We report a case of recurrent AF with hypercellular ectomesenchyme which developed a year after its conservative removal. We conclude that in recurrent AF sufficient sections of the pathological specimen are to be taken to rule out any malignant changes that might have begun in focal areas. Ameloblastic fibroma (AF) is a rare tumor of odontogenic origin comprising 1.5–4.5% of all odontogenic tumors .Although reported in a wide age range 0.5–62 years), most AFs are seen in the first two decades of life with 77.7% of cases being diagnosed before the age of 20. Males show slightly higher prevalence (M : F = 1.4 : 1), and the posterior mandible is the most common anatomic location [ years, mSmall lesions are asymptomatic, whereas larger ones may cause painless swelling . ApproxiRadiologically, AF is a unilocular  or multilocular (when large) radiolucent lesion often with a smooth, sclerotic border and may or may not produce bulging of bone , 6.Grossly, it appears as a solid, soft tissue mass with a smooth surface. It may or may not be encapsulated . HistoloAF needs to be differentiated from ameloblastoma, odontogenic myxoma, dentigerous cyst, odontogenic keratocyst, central giant cell granuloma, and histiocytosis . PresencTreatment of AF in general is a conservative approach, such as enucleation with curettage of the surrounding bone along with the removal of the affected tooth , 3. LongIn August 2011, an 18-year-old female patient reported with a complaint of gingival overgrowth in the left lower back region, which interfered with chewing. Growth was insidious, slowly progressing, and no history of associated pain. On intraoral examination, a huge exophytic growth measuring roughly 3 × 4 cms was noticed posterior to the mandibular left second molar. It extended anteroposteriorly from 37 to retromolar pad, buccally in the vestibule and inferiorly till the floor of the mouth. On radiographic examination, a well-defined radiolucency was found around impacted 38 . SurgicaThe patient reported again in August 2012 with a recurrent swelling in the same area. It was associated with difficulty in mastication. There was no history of pain, paresthesia, or discharge. Intraoral examination revealed a proliferative growth measuring 5 × 4 × 3 cms and extending from 35 to retromolar area . SubmandThe excised specimen consisted of left half of the body and ramus of the mandible with the tumor mass measuring 7.5 × 6.5 × 6.5 cms . HistopaWorld Health Organization WHO 1992) classifies AF as a true mixed neoplasm of odontogenic origin with both epithelial and ectomesenchymal components, without hard tissue formation [ classifiAlthough AF is the most common in posterior mandible region of young adults, a case occurring in anterior mandible of a 45-year-old man with considerable extension and soft tissue involvement has been reported .The exact pathogenesis is not clear. The tall columnar ameloblast-like cells in the epithelial component are too primitive to induce the cells of the ectomesenchyme, and only little is known about their interactions. It is also unknown why induction of odontoblastic differentiation is lacking in AF . ImmunohHistological variants of AF that have been described are granular cell AF, in which the ectomesenchyme is dominated by granular cells, papilliferous AF showing marked proliferation of the epithelium with plexiform arrangement , ameloblMIB-1, a monoclonal antibody against proliferation-associated nuclear antigen, in AF ranged from 2.9 to 7.5% and from 1.5 to 13.5% in the epithelial and mesenchymal components, respectively. These indices were higher in recurrent AF and AFS . KnowledApproximately, 45% of AFS is reported to arise from a recurrent AF . Hence, Whether AF is to be treated by conservative enucleation or by aggressive modality is still a topic of debate. A recurrence rate of 43.5% and 18% was reported by Trodahl and Zallen, respectively. Gundlach was of the opinion that simple enucleation would not be sufficient for AF . Most of


A 45-year-old uncircumcised married male presented with asymptomatic growth on the glans penis of 8 months duration. There were no complaints or past history suggestive of sexually transmitted diseases (STDs) or pre-existing skin lesion. There was no history of trauma, any other dermatoses, or systemic disease. There was no history of STD in the partner. The patient was initially diagnosed as a case of genital warts and was being treated with podophyllin and cryotherapy, but there was no improvement.On examination, there was hyperkeratotic, hypertrophic, verrucous plaque with mild crusting on glans Figures  and 2. TWhat is your diagnosis?Answer: Cutaneous HornCutaneous horn (Cornu cutaneum) refers to a well-defined cone-shaped lesion with hyperkeratotic features. Approximately 30% of cutaneous horns are found on the face and scalp. Other common locations include sun-exposed areas, such as the ear, lip, chest, neck, and shoulder. They may, however, develop on areas not exposed to sunlight, such as the penis, mucosal lower lip, and nasal vestibule.2Over 60% of lesions are benign; however, malignant and premalignant lesions might be associated with it. These conditions include actinic keratosis, squamous cell carcinoma, seborrheic keratosis, molluscum contagiosum, verruca vulgaris, trichilemmoma, Bowen’s disease, and basal cell carcinoma.They are asymptomatic with variable size and form, such as cylindrical, conical, pointed, corrugated transversely and longitudinally, or curved like a ram’s horn. They are solitary, growing slowly over years to decades if left alone. The base of the horn may be flat, nodular, or crateriform. SurroundThe etiology of penile horns is uncertain, although they are often found in association with warts, phimosis, naevi, and in areas afflicted by trauma if present. As reported by Lowe and McCullogh, the condition may be benign in 42%–56% of cases, premalignant in 22%–37%, or frankly malignant in 20%–22%.[Treatment options include wide surgical excision with careful histologic examination to exclude a focus of malignancy. If malignancy is present in a penile cutaneous horn, the treatment involves partial penectomy with or without regional lymph node dissection. Therapy with carbon dioxide or Neodymium YAG laser is used for patients who refuse surgery. Our patient was advised for surgical excision and its histopathologic examination to rule out any foci of malignant change.


Aggressive fibromatosis is a benign tumor, thought to arise from deep musculoaponeurotic structures, rarely found in the head or neck. However, when it does occur in the head and neck region, it tends to be more aggressive and associated with significant morbidity, which may be attributed to the vital vascular, neurological or anatomical structures in close proximity.We report the case of a 39-year-old Pakistani man who presented with a two-month history of a lump on the right side of his neck. The mass was excised and histopathological analysis revealed a case of aggressive fibromatosis.Due to the rarity of the condition no guidelines are available on the indications and extent of each modality. Due to its aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. On the basis of this case, we suggest that aggressive surgery is a viable management option and may be successfully used as a single modality treatment. Aggressive fibromatosis (AF) is a benign tumor, thought to arise from deep musculoaponeurotic structures , charactDue to the tendency of AF to invade local structures and recur, a multi-modality management strategy is usually employed. However due to its rarity in the head and neck, no guidelines are available on the indications and extent of each modality. Here we report a case of AF of the neck successfully managed by wide surgical excision; with no signs of recurrence 22 months after surgery.A 39-year-old Pakistani man had presented to our General Surgery clinic with a two-month history of a lump on the right side of his neck. There were no associated symptoms. There was no history of tobacco or irritant use. On physical examination a fixed mass, 4 cm in size, was palpated in right level III. It was not associated with any changes in the overlying skin. An examination of his upper aerodigestive tract was unremarkable. The mass was excised by a general surgeon. Histopathological analysis demonstrated spindle-shaped cells with no identifiable nuclear pleomorphism or mitotic activity. The cells stained positive for anti-smooth muscle actin (ASMA), favoring a smooth muscle origin.The mass, however, recurred within a period of one month and continued to increase in size, limiting his neck movement to the right side. On presentation to our Head and Neck Surgery clinic, a hard, well demarcated, non-tender and non-mobile mass of about 5 × 8 cm was noticed in the posterior triangle.A computed tomography (CT) scan of his neck showed a large soft tissue mass in his right posterior neck, measuring about 6.5 × 4.7 cm in its greatest dimension, involving both his anterior and posterior scalene muscles and closely abutting the right lobe of his thyroid gland. On the anterior side it extended up to his sternocleidomastoid muscle (SCM). Splaying of his right internal jugular vein was also noted between the SCM and the mass. Multiple sub-centimeter lymph nodes were also noted at level II. Fine-needle aspiration cytology (FNAC) showed spindle shaped cells with elongated vesicular nuclei and pink cytoplasm admixed with pink fibrocollagenous material suggestive of fibromatosis. Our patient underwent a wide local excision with a right-sided neck dissection.Histopathological analysis of the excised tissue showed spindle cells arranged in sheets and intersecting fascicles with variable collagen deposition. No cytological atypia was noted; however, infiltrative growth was noticed within the entrapped skeletal muscle at the periphery. The lesion showed evidence of vascular proliferation and peripheral lymphocytic infiltration. The above findings correlated with a tissue diagnosis of AF.Our patient once again presented with local recurrence of the mass Figure , not assMagnetic resonance imaging (MRI) showed a large contrast-enhancing mass, hypointense on T1 and hyperintense on T2, lying in the lower part of his neck to the right of the midline. It was deep to the SCM on the anterior side, abutting the larynx medially and the paravertebral muscles and space on the posterior side Figure .The mass was resected and an extended right neck dissection was performed. Analysis of the excised tissue revealed spindle cells arranged in whorls and fascicles, with intervening thin walled vessels and extravasation of red blood cells. There was no evidence of atypical mitosis or necrosis; however, focal infiltration of adjacent muscle was noted, favoring benign fibromatosis Figure .Our patient was followed in the out-patient clinic regularly for 22 months with no evidence of recurrence of disease. No further treatment was given.AF in the head and neck is a rare benign tumor, thought to arise from connective tissue, fascial sheaths, and other musculoaponeurotic structures of the body . It presAF is a slow growing tumor; as such it demonstrates a low level of mitosis and cellular atypia, and distant metastasis has yet to be reported. Its true pathogenic potential lies in its ability to locally invade surrounding structures. This fact is of extreme importance in the head and neck region due to the compact anatomical schematics and close association with vital neurovascular and anatomical structures; however invasion of the neurovascular structures has not been reported .et al. proposed a classification for AF in the head and neck after reviewing cases reported in the last 40 years, based on the site (such as upper and lower compartment), bone involvement and presence of hormone receptors [Kruse eceptors . In theieceptors , but theThe main treatment modality is wide surgical excision of the lesion, however in the head and neck region preservation of vital structures and their function may impede this objective . TherefoStudies have shown that radiation therapy can also be effectively employed independently, leading to a complete response in 20%, a partial response in 20%, and stable disease in 53% of cases; supporting evidence that radiation therapy may decrease indications for surgery . In caseHowever, radiation therapy is associated with a high risk of complications, especially in the head and neck region, and in our opinion should only be considered in cases with residual disease or where surgery may significantly impair the functional capabilities of the patient.et al. report significant tumor shrinkage with low dose methotrexate and vinblastine [et al., in their review on pharmacological management of AF, concluded that although chemotherapy is effective against AF, larger trials are required to validate their results [Although it is argued that chemotherapy is not effective in benign tumors with low mitotic rates, cases of AF successfully treated with cytotoxic and non-cytotoxic chemotherapy are being reported. Sze blastine . Anotherblastine . Meloxicblastine . Keeping results . Sarcoma results .et al., with no recurrence noted after a mean follow-up of 30 months [Recently radio frequency ablation has also been advocated by Ilaslan 0 months . Early d0 months . HoweverThe rarity of AF in the head and neck means that no guidelines are currently available on the indications and extent of each modality. Due to the aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. On the basis of this case, the authors suggest that aggressive surgery is a viable management option for AF in the head and neck, and may be successfully used as a single modality treatment. However where functional preservation and aesthetics need to be taken into account, a multi-modality treatment plan involving chemotherapy and radiation therapy can be considered for its management.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.ZAS and MJ were primarily involved in compiling patient details, reviewing available literature and drafting the primary manuscript with contributions from the other authors. MJK identified the patient and interpreted the patient data along with contributing to and editing the manuscript. All authors read and approved the final manuscript.


Gastric necrosis due to acute massive gastric dilatation is relatively rare. Vascular reasons, herniation, volvulus, acute gastric dilatation, anorexia, and bulimia nervosa play a role in the etiology of the disease. Early diagnosis and treatment are highly important as the associated morbidity and mortality rates are high. In this case report, we present a case of gastric necrosis due to acute gastric dilatation accompanied with the relevant literature. The pathogenesis of acute gastric dilatation (AGD) is not fully known, and several theories currently exist. Vascular compression, herniation, volvulus, acute necrotizing gastritis, complications after abdominal surgery, anorexia, bulimia nervosa, trauma, exposure to caustic materials, diabetes, and acute massive gastric dilatation are indicated in the etiology of the disease . Gastric ischemia and necrosis are rare due to the rich collateral blood flow to the stomach. However, if a sudden increase in gastric pressure occurs, gastric ischemia and necrosis can develop due to the impairment of the intramural blood flow .In this case report, we present a case of gastric necrosis due to acute gastric dilatation in terms of the literature.A 26-year-old hemiplegic, mentally and motor-retarded female patient was admitted to the ER with sudden-onset abdominal pain, abdominal swelling, and vomiting. The patient had a temperature of 38.5°C, blood pressure of 80/60 mmHg, heart rate of 112/min, and respiratory rate of 32/min. Physical examination revealed abdominal distention, defense, rebound, and tenderness. Her laboratory tests were normal except for elevated white blood cell count (25.000/mm³). Direct abdominal X-ray images obtained in a standing position showed air almost completely filling the left upper quadrant of the fundus and extending into the right upper quadrant . ImmediaGastric necrosis due to rich blood flow to the stomach is relatively rare. Acute gastric dilatation was first described by Todd et al. in 2000 . Casper Evaluation of psychiatric patients with abdominal pain is difficult. A detailed anamnesis cannot be obtained, and thus, the surgeon must act based on physical examination findings and must be informed of and experienced with surgical diseases seen in psychiatric patients. Physical examination findings can widely differ amongst patients. Diagnosis can be supported by radiological imaging of gastric dilatation and pneumoperitoneum . Resusci Acute gastric dilatation occurs mostly in women (67%) and is frequently found in the lesser curvature of the stomach . AGD is Resection of the gangrenous gastric part is essential in AGD. Usually, a total gastrectomy is required. Resection and esophagojejunostomy are successful under optimal conditions , 9. In cIn conclusion, early diagnosis of gastric necrosis due to AGD is highly important due to the associated high morbidity and mortality rates. Gastric necrosis due to AGD must be considered in differential diagnosis to avoid late diagnosis in mentally retarded patients and patients with eating disorders.


We describe two cases of large solitary luteinized follicle cyst of pregnancy and puerperium (LSLFCPP) with new clinicopathologic findings. The first case occurred in a 40-year old woman who was found to have a left ovarian mass during the third trimester of pregnancy. The patient delivered a full term healthy female infant via caesarean section. The ovarian mass was removed by oophorectomy. The specimen showed a unilocular, thin-walled, clear fluid filled cyst measuring 15 × 12 × 5 cm. Microscopically, the cyst was lined by single to multiple layers of luteinized cells with mainly small, round and regular nuclei and focally enlarged, bizarre, and hyperchromatic nuclei. Occasional mitotic figures were seen. The cyst wall showed marked edema and nests of luteinized cells that were morphologically similar to the cyst lining cells. Groups of lesional cells were surrounded by reticulin fibers. The patient has been healthy without disease after 7 years. The second patient was a 29-year old pregnant woman who was found to have a right ovarian cyst by ultrasound at 14-week gestation. She then presented with preterm labor at 33-week gestation and delivered a healthy female infant via caesarean section. A right salpingo-oophorectomy was performed. Gross inspection of the specimen revealed a unilocular, brown mucoid fluid filled cyst measuring 14 × 11 × 9 cm. The cyst surfaces were smooth, and the cyst wall exhibited marked edema. Microscopic examination showed features similar to the first case: cyst lined by luteinized cells with focal large nuclei, scattered nests of luteinized cells in the edematous fibrous wall, and reticulin fibers surrounding large nests of lesional cells. No mitoses, however, were identified in the second case. The patient has been well without disease 1 year after surgery. These two cases contribute to a better understanding of LSLFCPP. Our case in the 40-year old patient is the first to show mitotic figures in LSLFCPP and suggests that the presence of occasional mitoses should not exclude a diagnosis of LSLFCPP. The lesion in the second patient caused preterm labor. Nevertheless, absence of disease recurrence in our patients demonstrates a benign nature of LSLFCPP. Ovarian tumors and tumor-like masses during pregnancy are uncommon, with an incidence of about 1% in one large study consisting of 8420 patients (1). Most neoplasms are benign, and about 4% are malignant (1). Tumor-like lesions include pregnancy luteoma, hyperreactio luteinalis, intrafollicular granulosa cell proliferation, hilus cell hyperplasia, ectopic decidua, and large solitary luteinized follicle cyst of pregnancy and puerperium (LSLFCPP) (1-2). These lesions can simulate neoplasms by clinical, gross, and microscopic examinations. Of particular interest is LSLFCPP for its enormous size and confusion with neoplasms. LSLFCPP is a rare lesion; only about 10 cases have been reported in the literature. We now describe the clinicopathologic features of LSLFCPP in two patients.This was a 40 year old, premigravida patient who initially presented to the infertility clinic at our institution for desired pregnancy. Her past medical history was significant for primary infertility. A successful intrauterine pregnancy was achieved via intrauterine insemination. Due to advanced maternal age, the patient underwent extensive prenatal testing and monitoring through the course of her pregnancy. All tests were normal, with the exception of a left ovarian mass that was incidentally detected by ultrasound during the third trimester. The patient was closely followed without prenatal surgical intervention. Her pregnancy advanced uneventfully, and labor commenced at 40-week gestation. Due to failure to progress, a caesarean section was performed resulting in the delivery of a healthy female infant. At the time of caesarean section, a left oophorectomy was performed.The specimen was received fresh for intraoperative pathology consultation. On gross examination, it consisted of an intact, unilocular, thin-walled cyst measuring 15 × 12 × 5 cm and filled with clear fluid. Both the outer and the inner surfaces of the cyst were smooth. The cyst wall ranged from 0.1 cm to 0.8 cm in thickness and showed marked edema. Based on the gross findings, an intraoperative interpretation of benign ovarian cyst was made. No frozen section was performed.On subsequent microscopic examination, the cyst was lined by single to multiple layers of large cells with abundant eosinophilic cytoplasm Figure . Most ceThe patient has been free from disease for 7 years after surgery.The patient was a 29-year old pregnant woman who previously delivered through caesarean sections two healthy full term infants. Her past medical history was significant for gastroesophageal reflux disease, asthma, obesity, gallstones, and smoking. Ultrasound at 14-week gestation revealed a right ovarian cyst. At 33-week gestation, the patient presented with preterm labor. With repeat caesarean section, a healthy female infant was delivered. At the time of caesarean section, a right salpingo-oophorectomy was performed after approximately 2 liters of fluid were drained from the ovarian cyst.We received the specimen for intraoperative consultation. Gross inspection revealed a cystic lesion measuring 14 × 11 × 9 cm. The external surface was maroon and smooth with prominent vascular markings. Sectioning showed a unilocular cyst filled with brownish mucoid fluid. The cyst wall measured from 0.5 cm to 1.0 cm in thickness and showed severe edema. The inner surface of the cyst was flat and displayed scattered patches of hemorrhage but no solid or papillary tumor was identified. Attached to the cyst was a residual normal appearing ovary measuring 3 × 2.5 × 1.5 cm and an unremarkable segment of fallopian tube measuring 3 × 0.5 cm. The gross findings indicated a benign ovarian cyst, and thus no frozen section was performed.On microscopic examination, the cyst was lined by one to several layers of luteinized cells that exhibited abundant eosinophilic cytoplasm with focal vacuolization. Most nuclei were small and round but focal nuclei were irregular and enlarged Figure . No mitoThe patient has been well without disease after 1 year since surgery.Our two cases of LSLFCPP demonstrated many clinicopathologic features similar to those described in the literature (3-6), including a unilateral, unilocular cyst and the presence of focal bizarre nuclei. However, our case in the 40-year old patient was the first to show mitotic figures. This finding indicates that the presence of focal mitoses should not exclude a diagnosis of LSLFCPP. Although most patients with LSLFCPP do not show complications (3), our second patient experienced preterm labor at 33-week gestation. As examination of placenta and review of prenatal history did not show other causes of preterm labor in this patient who previously delivered two infants at full term, LSLFCPP was most likely the culprit of the patient's preterm labor. Our cases demonstrate that LSLFCPP is benign as the patients have been free of disease since surgery for 7 and 1 year, respectively.The pathogenesis of LSLFCPP is unclear. Its occurrence during pregnancy suggests a role of human chorionic gonadotropin (hCG). This association is supported by the presence of numerous cystic follicles, which are known to be induced by hCG, in the remnant ovaries of our cases. The literature, however, has described several cases of LSLFCPP occurring late in the puerperium when hCG levels are low (3). Thus hCG may not be the only contributing factor. Although lined by luteinized cells, the cyst is unlikely to originate from a corpus luteum since a separate corpus luteum was seen in the remnant ovaries of our cases and in the contralateral ovary of another case (3). The presence of reticulin fibers around groups of lesional cells supports a granulosa cell origin from a follicle.The major differential diagnoses include the unilocular cystic granulosa cell tumors of both the adult and the juvenile types. Although LSLFCPP is indistinguishable grossly from unilocular cystic granulosa cell tumor, they differ in microscropic features. Unlike LSLFCPP that show luteinized cells with focal bizarre nuclei, adult granulosa cell tumor is composed of a monotonous population of granulosa cells usually without luteinization and bizarre nuclei (7). Furthermore, nuclear grooves and Call-Exner bodies are seen in granulosa cell tumor but not in LSLFCPP. Juvenile granulosa cell tumor may create a greater diagnostic problem as the neoplastic cells often exhibit abundant eosinophilic cytoplasm and occasional bizarre nuclei. However, juvenile granulosa cell tumor is mitotically active while LSLFCPP shows absent to occasional mitotic figures.The findings in our two cases contribute to a better understanding of LSLFCPP. Our case in the 40-year old patient is the first to show mitoses in LSLFCPP and indicates that the presence of occasional mitoses should not exclude the diagnosis of LSLFCPP. The lesion in the second patient caused preterm labor. Nevertheless, absence of disease recurrence in our patients demonstrates that LSLFCPP is benign.The authors declare that they have no competing interests.ML collected case information and participated in manuscript writing. SK collected case information and reviewed manuscript. CZ collected case information and wrote the manuscript. All authors read and approved the final manuscript.


Cavitating mesenteric lymph node syndrome (CMLNS) is a rare and poorly understood complication of coeliac disease (CD), with only 37 cases reported in the literature. CD is an immune-mediated enteropathy, with alterations seen in the small bowel architecture on exposure to ingested gluten. Those who fail to respond to a strict gluten-free diet are termed to have refractory coeliac disease (RCD). This is associated with serious complications such as enteropathy-associated T-cell lymphoma (EATL). We present the case of a 71-year-old female investigated for weight loss and a palpable intraabdominal mass. Abdominal computed tomographic (CT) scan showed multiple necrotic mesenteric lymph nodes. At operation, multiple cavitating mesenteric lymph nodes, containing milky fluid, were found. An incidental EATL was found at the terminal ileum, which was resected. The patient subsequently tested positive for CD. This is the second case report to document an association between CMLNS and EATL. This paper highlights the varied presentation of CD. In this case, the diagnosis of CD was made retrospectively after the complications were dealt with. This paper is followed by a review of relevant literature. Cavitating mesenteric lymph node syndrome (CMLNS) is a rare and poorly understood complication of coeliac disease (CD) with only 37 cases reported in the literature and is associated with a very poor prognosis. Some studies estimate a 50% mortality, mainly related to the complications of severe malnutrition, intestinal haemorrhage secondary to ulceration, and overwhelming sepsis as a result of a combination of hyposplenism and malnutrition. CD is an immune-mediated enteropathy, with alterations seen in the small bowel architecture on exposure to ingested gluten. Those who fail to respond to a strict gluten-free diet are termed to have refractory coeliac disease (RCD), which is associated with an increased risk of malignant neoplasms. Lymphoma accounts for approximately 50% of these, and the most common lymphoma is enteropathy-associated T-cell lymphoma (EATL). A 71-year-old female with a past medical history of bronchiectasis was investigated by respiratory physicians for a lobulated opacity seen at the right lung base on computed tomographic (CT) scan of the thorax. A repeat CT scan, 2 months later, which included the upper abdomen, showed that the lung opacity had virtually resolved. However, several lymph nodes lying along the mesenteric vessels had now become more prominent, the largest measuring 1.7 cm in short axis. These nodes had necrotic low-density centres and peripheral enhancement. Due to concerns that the patient may have lymphoma or intraabdominal malignancy , a CT abdomen and pelvis was performed. This demonstrated a left ovarian mass with intraabdominal lymph nodes consistent with a potential diagnosis of ovarian cancer with lymphatic and omental metastases. There were no radiological or haematological features of hyposplenism. A pelvic ultrasound scan (USS) confirmed a cystic mass with a solid component lying within the left adnexa, measuring 33 × 25 × 59 mm. In light of these findings, total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were performed by the gynaecologists. However, pathological examination of the resection specimen was normal. Six months later, a follow-up USS showed several large intraabdominal lymph nodes with necrotic centres lying along the midline, the largest measuring 53 mm in diameter. An USS-guided percutaneous biopsy of one of these lesions withdrew 20 mls of thick white material. Two further needle passes only withdrew fragments of material, which were later proven to be skeletal muscle and fibroadipose tissue. The patient was then referred to the General Surgical service for minilaparotomy and biopsy of mesenteric lymph nodes. Her only clinical symptom continued to be weight loss. At operation, multiple, large cystic masses within the root of the small bowel mesentery were noted, the largest measuring approximately 15 cm in diameter. Due to the multiplicity of these masses and the fear of the devascularisation of the small bowel with attempted removal, a decision was made against this. Instead, one of the cysts was opened up, draining milky-white fluid, and part of the wall of this cystic mass was excised for histological purposes. This revealed a paucicellular wall containing plasma cells, lymphocytes and fibrous tissue, which was devoid of an epithelial lining and was consistent with a cavitating mesenteric lymph node see . An incidental localized small bowel tumour was found in the terminal ileum, and this was removed by means of an ileocaecal resection. Pathological examination and immunohistochemistry revealed this to be an enteropathy-associated high grade T-cell lymphoma. The rest of the small bowel mucosa showed changes of gluten-associated enteropathy with a marked increase in intra-epithelial T cells, villous blunting, and expansion of the lamina propria by small lymphocytes, in keeping with CD. The resection margins were clear of tumour.The patient made an uneventful recovery and was referred for oncological treatment of her lymphoma (chemotherapy). The diagnosis of CD was further confirmed by positive serum endomysial and tissue transglutaminase IgA antibody levels. At 17-month follow-up she remained free of any disease recurrence and asymptomatic with a repeat CT scan showing a reduction in size of the mesenteric lesions.This is the second case report in the literature to document an association between CMLNS and EATL. It is well recognised that CMLNS is a rare and poorly understood complication of coeliac disease –3.CD is an inflammatory condition, characterised by an intolerance to ingested grain glutens found in wheat, barley, and rye. If those with the condition are exposed to the gliadin protein component of gluten this leads to an inflammatory process that damages the small bowel mucosa and ultimately leads to malabsorption , 5. CD iAt one end of the spectrum are patients who fail to respond to a strict gluten-free diet, and this is termed refractory coeliac disease (RCD). As well as persisting symptoms, they are at risk of life, threatening complications, which include the development of intestinal T-cell lymphoma and severe malnutrition. At the other end of the spectrum, patients can be almost asymptomatic. There are a number of extraintestinal manifestations that are commonly associated with CD , 7.The pathogenesis of CMLNS is unknown. Characteristically, patients present with refractory symptoms of CD—weight loss, diarrhoea, and fatigue. Clinical signs of hyposplenism may be evident with increased susceptibility to infection. Peripheral blood smear often shows target cells and Howell-Jolly bodies. Villous atrophy is almost always present at small bowel biopsy, as in our case , 8. MultClassic CT and sonography findings of CMLNS have been described. Masses are variable in size, ranging from 2 to 8 cm in diameter, and the majority are multiple lesions . On CT,  The pathogenesis of CMLNS is unclear but several theories have been proposed. The small bowel is drained by mesenteric lymphatics termed lacteals, which absorb and transfer emulsified dietary fat via specialised lymph called chyle. It is these specialised mesenteric lymphatics that undergo cystic changes and cavitate. These changes are unique to the mesenteric nodal chain supplying the small bowel. It has been hypothesised that this may be attributed to excessive antigenic exposure of the immune system as a result of damaged intestinal mucosa. This then leads to depletion of cellular lymphoid tissue in the nodes and spleen. Alternatively, the cystic changes may actually reflect necrosis of the mesenteric nodes triggered either by a localised immune-mediated complement cascade and intravascular coagulation, or by trauma , 8.Management of CMLNS is complex. There is no convincing evidence to suggest that regression of the cysts occurs on a strict gluten-free diet as long-term follow-up is limited . Simple CMLNS has been associated with a very poor prognosis. Some studies estimate a 50% mortality, mainly related to the complications of severe malnutrition, intestinal haemorrhage secondary to ulceration, overwhelming sepsis as a result of a combination of hyposplenism, and malnutrition , 8, 11.There is an increased risk of malignant neoplasms in patients with CD, the incidence of which is reported as high as 14% . LymphomEATL is a rare, aggressive malignancy with an incidence of less than 1% of all Non-Hodgkin's lymphoma . WhetherThere is a lack of recent data on symptom duration of CD prior to a diagnosis of EATL. In 1967, Harris et al.  suggesteThis case highlights the complex presentation of CD, especially when it presents with its more serious complications of CMLNS and EATL, and the diagnosis can be challenging. Expedient diagnosis will probably help to decrease the morbidity and mortality associated with untreated coeliac disease.