We report the multimodality imaging findings of peritoneal inclusion cysts in two adolescent females each with a prior history of abdominal surgery. The few reports of peritoneal inclusion cysts in the pediatric population have largely focused on the clinical and pathological features of this entity. We wish to emphasize the imaging findings of peritoneal inclusion cysts on multiple modalities, the advantage of MRI in confirming the diagnosis, and the need to keep considering this diagnosis in patients who present with a pelvic cystic mass, with a history of surgery, even if remote. Additionally, we review the pathology, pathophysiology, differential diagnosis, and treatment options of peritoneal inclusion cysts. Peritoneal inclusion cysts (PIC), also known as “peritoneal mesothelial cysts,” “peritoneal pseudocysts,” and “inflammatory cysts of the pelvic peritoneum,” have been described in females of reproductive age . ReportsTwo cases of peritoneal inclusion cysts in patients with a history of abdominal surgery are presented. The first patient was born with genitourinary anomalies and underwent surgery three years prior to presentation. The second patient underwent surgery as an infant for necrotizing enterocolitis, and to our knowledge, this association has not been reported previously. The characteristic multimodality imaging findings associated with PIC are illustrated. We also review the pathogenesis, clinicopathological features, and differential diagnosis of PIC.A 16-year-old female presented with Mullerian renal cervical somite (MURCS) syndrome. The patient was born with ambiguous genetalia, multiple vertebral body segmentation anomalies, and unilateral renal agenesis. She presented three years after undergoing a right renal transplant with a painful right inguinal mass and increasing abdominal girth. Sonography demonstrated a large anechoic mass in the right lower quadrant extending into the right inguinal region Figures . The rigA 17-year-old female presented with left lower quadrant pain. She was a former 25-week premature infant and had undergone a partial colectomy during infancy for necrotizing enterocolitis. A radiograph was performed which demonstrated a paucity of bowel in the left lower quadrant and a large mass was suspected ; an ultrDuring the reproductive years, fluid produced by the ovaries is normally absorbed by the peritoneum. When the integrity of the peritoneum is disrupted as a result of surgery, trauma inflammation, or endometriosis the peritoneum has decreased ability to absorb fluid. In addition, postsurgical adhesions can trap ovarian fluid that is no longer being absorbed by a disrupted peritoneum, producing a complex cystic pelvic mass. Functional, active ovaries and adhesions are thus essential for the development of peritoneal inclusion cysts, compatible with the presentation of this lesion upon and after the onset of puberty.The most common presenting symptom in patients with a peritoneal inclusion cyst is lower abdominal or pelvic pain. On physical exam, often there are no palpable abdominal or pelvic masses . Common Peritoneal inclusion cysts are simple or complex cystic adnexal collections consisting of a normal ovary entrapped in multiple fluid filled adhesions. Grossly, the cysts are often multiple with some forming confluent masses. Individual locules may contain serous fluid, gelatinous fluid, or hemorrhagic material .Pathologically, peritoneal inclusion cysts are pseudocysts and are typically lined by hyperplastic mesothelial cells proliferating within inflamed fibrous granulation tissue walls . Often, Most cases of PIC express focal negative immunoreactivity for CEA. This needs to be considered when entertaining a diagnosis of PIC versus an ovarian malignancy. Rarely, however, PIC may exhibit positive CEA immunostaining .As previously mentioned, peritoneal inclusion cysts may not be palpable and may only be detected via imaging . Thus, tUltrasound is the most frequently used modality in the initial workup of pelvic pain, as it does not use a radiation source, it is relatively inexpensive, and it is easily accessible. In addition, sonography may be useful in lesions warranting image-guided aspiration. The most typical sonographic finding of a peritoneal inclusion cyst is a normal ovary surrounded by anechoic fluid containing multiple septations . The fluOn CT, peritoneal inclusion cysts depict a cystic mass with regular or irregular borders, containing material with the attenuation properties of fluid and/or hemorrhage. MR images demonstrate cystic lesions with low T1 signal and high T2 signal consistent with serous fluid. In cases of hemorrhagic material, cysts may demonstrate high T1 signal. The high contrast resolution of soft tissues on MR makes it useful in detecting these pseudocysts. The shape of peritoneal inclusion cysts may be irregular, secondary to mass effect by adjacent organs, as these pseudocysts lack true walls. Contrast-enhanced T1 images typically demonstrate little or no contrast enhancement. Again, the “spider in a web” appearance of the lesion on MRI is typical of PIC. MRI may also be helpful in defining the extraovarian nature of the fluid collection(s), as the ovary may not be clearly defined on sonographic examination, as in The main differential diagnosis for PIC in the pediatric population includes macrocystic lymphatic malformation, hydrosalpinx/pyosalpinx, paraovarian cysts, and ovarian epithelial malignancy. Macrocystic lymphatic malformations appear as multilocular cystic masses that are anechoic or contain internal septations and echogenic debris. There may be enhancement of the septations within the lymphatic malformation, not characteristically seen in PIC. Hydrosalpinx may mimic a peritoneal inclusion cyst, as adhesions can entrap fluid in an oblong loculation adjacent to the uterus, as illustrated in . OccasioEndometrioma may have a similar appearance to PIC on sonographic examination and also should be considered in the differential diagnosis. However, an endometrioma usually has hemorrhagic components, not typically seen in PIC. Again, MRI may be helpful in distinguishing an endometrioma from PIC.Both conservative and surgical management approaches have been described in patients with a peritoneal inclusion cyst. The use of oral contraceptives is thought to decrease the amount of fluid produced by the ovary. As reported by Hoffer et al. , women wIn summary, we emphasize the importance of entertaining the diagnosis of a peritoneal inclusion cyst in pediatric patients with a history of surgery and in whom a simple or complex cystic mass is demonstrated on imaging. Although sonography plays a major role in the diagnosis, MRI is a useful adjunct in demonstrating ovarian tissue entrapped within the cyst. Familiarity with the characteristic imaging findings of PIC and knowledge of its clinical associations is useful to establish this diagnosis and guide appropriate treatment and may avoid aggressive intervention.


Primitive neuroectodermal tumors (PNETs) are presented as rare malignant neoplasms. In unusual cases, those neoplasms may arise in solid organs containing neuroendocrine cells, such as the pancreas. Herein the case of a 28-year-old patient that underwent gastroduodenopancreatectomy after the diagnosis of a huge mass (PNET) located in both head and body of the pancreas is reported. This is the 19th case of pancreatic PNET reported in literature. Primitive neuroectodermal tumors (PNETs) are presented as rare malignant neoplasms, showing different degrees of differentiation. They are often classified as small round cell tumors arising from soft tissues. PNETs also integrate Ewing's sarcoma family of tumors (ES), which represents 1% of all sarcomas .In rare cases, PNETs may arise in solid organs containing neuroendocrine cells, with previous reports on kidneys, urinary bladder, ovaries, uterus, parotid glands, heart, and lungs . BesidesThese neoplasms occur predominantly in pediatric and teenaged populations , in a raA proper integration of the pathological, clinical, immunohistochemical, and cytogenetic findings is necessary for a successful diagnosis of pancreatic PNETs and also for distinguishing this group from other neoplasms with similar presentations . DespiteIn spite of the poor prognosis, surgical treatment followed by chemotherapy or radiotherapy or both is the most widely accepted option, given the aggressiveness of such neoplasms . This isA 28-year-old female patient was admitted to the Emergency Department of Santa Casa Hospital in Porto Alegre, Brazil. She was referring epigastric pain for 14 days, with partial relief due to the use of weak analgesics. The pain was associated with diffuse cutaneous pruritus, jaundice, choluria, and acholia.On physical examination, the patient was in a good general condition, jaundiced, with a hardened palpable mass in the epigastric and right hypochondrium regions. Laboratory tests showed a cholestatic pattern . TumoralInitially, an abdominal ultrasonography was performed, revealing a voluminous heterogeneous hypoechoic mass measuring about 13 cm width, 9 cm length, and 13 cm height. It presented cystic areas inside, between left hepatic lobe and pancreas. It was not possible to determine the origin of the mass. Dilatation of intrahepatic bile ducts and proximal common bile duct was also referred to .Computerized tomography with intravenous contrast unfolds a voluminous expansive lesion in pancreatic head and body, with well delimited borders, measuring about 12.8 × 12.1 × 10.9 cm. A heterogeneous density due to the presence of both liquid and solid areas is also shown in the CT. The image showed intense enhancement of an intravenous contrast agent. The lesion displaced adjacent structures, although no infiltration was detected. A significant ectasia of the intrahepatic and extrahepatic bile ducts could also be seen .The patient was evaluated by the hepatopancreatobiliary team and underwent a gastroduodenopancreatectomy, performed uneventfully. The gross appearance of the lesion was a solid cystic mass, was encapsulated and multiloculated, and was with necrotic aspect areas, measuring a total of 11.5 cm along its longest axis .β-catenin, automated RP , and LCA (leucocyte common antigen).The pathological study revealed a neoplasm of small round blue cells with scant cytoplasm arranged in nests with fibrovascular stroma. Few mitosis pictures and several areas of necrosis were also found. Immunohistochemistry was strongly positive for CD99 , vimentiγ-glutamyl transpeptidase, aspartate aminotransferase, and alanine aminotransferase. She was discharged on the 13th day after surgery and remains well, without signs of recurrence after 3 years. Considering R0 resection and controversies in the literature, adjuvant treatment with chemotherapy and/or radiotherapy was not performed.The patient recovered well postoperatively, presenting normalized levels of bilirubin, alkaline phosphatase, Primitive neuroectodermal tumors (PNETs) represent about 1% of all sarcomas and have a five-year survival rate of approximately 50% , 7. TherHistologically, the ES family of tumors is made of small monomorphic round cells with small nuclei and scant cytoplasm; nonetheless, there is a large group of tumors with the same pattern , 11. WhaAn association of pathological, immunohistochemical, clinical, and cytogenetic features is, therefore, required for diagnosing pancreatic PNETs . FurtherMIC2, which is considered important in cell adhesion [Immunohistochemistry is extremely useful in diagnosing this neoplasm, as peripheral primitive neuroectodermal tumors substantially express a cell surface glycoprotein. This glycoprotein is product of the MIC2 gene , also called CD99 or p30/32adhesion , 15.Regarding the clinical presentation, it can be said that there is no specific set of symptoms for pancreatic PNETs. The onset of the disease seems to be insidious and producing few symptoms, with abdominal pain during palpation of the mass being the most referred to symptom. Mao et al.  indicateBose et al.  pointed Among imaging tests, abdominal CT and MRI are the most used in the detection of these tumors. The diagnosis is not easy, as there is no pattern of radiological findings. The commonly found one, however, consists of masses with clear margins in the head of the pancreas , 12.Discussing again the compilation of 16 patients of Bose and colleagues [The PNETs are characteristic aggressive tumors. Studies have shown that 25–30% of patients presented metastasis at diagnosis of PNET and it is believed to be an even larger number of patients with micrometastasis , 12. TheMoschovi et al.  reportedMovahedi-Lankarani et al.  reportedIn spite of being an extremely rare disease, pancreatic PNETs should be considered in the differential diagnosis of pancreatic mass research, especially in patients younger than 35 years. Cases with undifferentiated small cell carcinoma, pancreatoblastoma, neuroendocrine carcinomas, and pancreatic endocrine tumors should also be investigated for PNETs. The clinical presentation of the tumor is diffuse and its histological findings are not exclusive. Most patients have a rapid growth mass, with nonspecific clinical symptoms and, often, abdominal pain.These tumors present themselves as highly aggressive tumors with almost inevitable recurrence and metastasis, which makes the overall five-year survival rate reach almost 50%. Despite significant advances in treatment, there is still no definitive conclusion on which is the best therapeutic strategy at the moment.


Urethral caruncles are the most frequent benign tumors of the female urethra. Most of them are found in post-menopausal women, and they are rare in childhood. Only a few pediatric cases have been published in the literature. In this report, we present an unusual case of a pediatric patient with a urethral caruncle, along with a review of the literature.A 9-year-old Mongolian girl was referred to our hospital with a 2-week history of frequent adherence of a small amount of blood to her underwear. We found a sessile smooth margin, a clear boundary and an elastic, soft red tumor over the entire circumference of the urethral meatus. At the beginning, because of the child’s age, urethral prolapse was suspected. There was no response after 3 weeks of conservative treatment with steroid ointment. With the patient under general anesthesia, a partial tumor resection was performed for the purpose of histological examination. The tumor excision was limited to about 1/2 laps of the urethral meatus to prevent the development of urethral stricture. On the basis of clinical and histopathological examinations, a diagnosis of a urethral caruncle was made. Post-operatively, steroid ointment application to residual masses was continued, and these disappeared about 6 months later. Our patient was free of recurrence and had had no complications after 3 years of follow-up.Urethral caruncles are rare in children, and the possibility of malignancy is slight during this period. Biopsy of the mass is not required for diagnosis. It should be indicated only if the mass has other characteristics that raise suspicion of malignancy. In previously reported cases, all of the tumor was removed. However, the trigger of the caruncle in childhood is chronic inflammation. Conservative therapy with steroid ointment should be the core treatment. However, it may be necessary to proceed to treatment because caruncles take a long time to heal. The case that we describe in this report will serve as an example for similar cases in the future. Urethral caruncles are benign and pedunculated and appear as sessile polypoid lesions of the posterior lip of the urethral meatus. They represent the most common lesion of the female urethra and occur primarily in post-menopausal women ,2. Only A 9-year-old Mongolian girl was referred to our hospital with a 2-week history of frequent adherence of a small amount of blood to her underwear. The girl’s mother’s pregnancy and the girl’s birth and neonatal history were uneventful. She had no history of hospitalization or surgery. We found a sessile smooth margin, a clear boundary and an elastic, soft red tumor over the entire circumference of the urethral meatus Figure a. The paUrethral caruncles are usually confined to the female urethra. They most often occur in middle-aged and elderly women and are rarely found in young females ,2. A totThe clinical differential diagnosis for a urethral or peri-urethral mass in pediatrics is urethral prolapse and peri-urethral gland abscess. Although uncommon, a spectrum of neoplasms may mimic urethral caruncle clinically, including sarcoma . Biopsy The management of such patients can be divided into conservative treatment and surgical resection. Conservative therapy with application of estrogen ointment is common in adults , and steUrethral caruncles are rare in children, and the possibility of malignancy is slight during this period. Biopsy of the mass is not required for diagnosis. Biopsy should be indicated only if the mass has other characteristics that raise suspicion of malignancy. In previously reported cases, all of the tumor was removed. However, the trigger of caruncles in childhood is chronic inflammation. Conservative therapy with steroid ointment should be the core treatment. However, it may be necessary to proceed to treatment because these lesions take a long time to heal. Because this disease is rare in children, the treatments employed for our patient and in the other cases reported in the literature should be borne in mind when such patients are encountered. The case that we describe in this report will serve as an example for similar cases in the future.Written informed consent was obtained from the patient’s legal guardian 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.


Castleman's disease is a benign lymphoproliferative condition with three distinct histological subtypes. Clinically it presents in either a unicentric or multicentric manner and can affect various anatomic regions, the mediastinum being the most frequent location. We herein present a rare case of unifocal retroperitoneal mass proved to be hyaline vascular Castleman's disease. We perform a review of the current literature pertaining to such lesions, focusing on the management of the various clinical and histological variants of the disease. Surgical excision is the treatment of choice for unifocal Castleman's disease. Castleman's disease is a benign lymphoproliferative condition with three distinct histological subtypes: hyaline vascular, plasma cell and mixed. Clinically it presents in either a unifocal or multifocal (multicentric) manner and can affect various anatomic regions, the mediastinum being the most frequent location. We herein present a rare case of unifocal pararenal mass proved to be Castleman's disease. We also perform a review of the current literature pertaining to such lesions, focusing on the management of the various clinical and histological variants of the disease.A 40-year-old female was admitted to our Surgical Department due to an asymptomatic left pararenal mass. The lesion was incidentally discovered during routine gynecological ultrasound workup. Past medical history was unremarkable. The patient did not report any abdominal discomfort, flank pain, back pain, weight loss, sweating, fatigue, or fever. On physical examination, she was afebrile with normal vital signs. Superficial lymphadenopathy or organomegaly was absent. Complete blood count, serum biochemistry, and tumor markers  were all within normal range.Further investigation with thoracic and abdominal computed tomography revealed a 7.6 × 5.3 × 12 centimeters' heterogeneous lesion adjacent to the left kidney. The mass was hypervascular with regular margins and did not invade other structures. Imaging of the thorax, mediastinum, and the rest of the abdominal cavity was unremarkable . On abdoDue to diagnostic uncertainty, we opted for a surgical exploration. The lesion was entirely removed via a left flank approach under general anesthesia. Postoperative course was uneventful and the patient was discharged on the 5th postoperative day.Histology revealed a lymph node specimen constituted by small-sized follicles with atrophic germinal centers and hyperplastic follicular mantle regions. The germinal centers were almost totally replaced by endothelial and dendritic cells. Mantle cells were arranged in layers surrounding the atrophic germinal centers. The interfollicular area abounded in numerous postcapillary venules, small vessels, with concentric fibrosis or hyalinization of their wall, and multiclonal plasma cells, while the number of the lymphocytes was reduced. No normal lymphoid tissue or lymph node sinus was recognized circumferentially. The immunohistochemical assays highlighted the CD20 expression of the mantle cells surrounding the hyaline vascular germinal center follicles, the dendritic cell hyperplasia, prominent vascularity of the interfollicular area, and reduced expression of Ki67 in the atrophic germinal centers. HHV-8 staining was negative. Histology was conclusive for Castleman's disease, hyaline/vascular variant.One year after the operation, the patient remains asymptomatic and in perfect general condition. Yearly abdominal computed tomography was negative for local recurrence or any other intra-abdominal pathology .The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumors and malignant neoplasms that can be either primary or metastatic lesions. Retroperitoneal tumors can cause a diagnostic dilemma and present several therapeutic challenges because of their rarity, relative late presentation, and anatomical location, often in close relationship with several vital structures in the retroperitoneal space.Sarcomas comprise a third of retroperitoneal tumors. Other retroperitoneal neoplasms include primary lymphoproliferative tumors (Hodgkin's and non-Hodgkin lymphoma) and epithelial tumors  or might represent metastatic disease from known or unknown primary sites .In order to investigate such tumors, various common tumor markers could be used preoperatively. In our case we used CA 19-9, CA-125, CEA, and AFP.β-human chorionic gonadotropin [In men with a retroperitoneal mass, the determination of germ cell tumor markers occasionally enables preoperative distinguishing of primary retroperitoneal germ cell tumors with considerable consequences for management. In this setting, a retroperitoneal tumor should be investigated by specific tumor markers such as alpha-fetoprotein and dotropin .In some cases of retroperitoneal tumor, neuron-specific enolase (NSE) can be used as a marker for neuronal tissue and thus offers a diagnostic criterion differing neuroblastoma from, for example, Wilms' tumor .Benign tumors can cause concern and are often an incidental finding during an investigation for unrelated symptoms. The most common benign pathologies encountered in the retroperitoneum include benign neurogenic tumors , paragangliomas , fibromatosis, renal angiomyolipomas, and benign retroperitoneal lipomas .Castleman's disease (hereafter depicted as CD) should also be included in the differential diagnosis of any hypervascular and heterogeneous tumor mass in the retroperitoneum .Castleman's disease, also known as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, or lymphoid hamartoma, is a nonclonal, nonneoplastic lymph node proliferation first described by Castleman and Town in 1954. Castleman and Town reported 13 cases of unicentric hyaline vascular lesions of the chest and described the classic pathologic features of hypervascular lymph nodes with hyalinization of vessels, which form concentric arrangements somewhat reminiscent of Hassall corpuscles in the thymus . CD has Despite its rarity, CD has recently sparked major research interest because of its association with human herpes virus 8 (HHV-8) and HIV infection, providing a model of initially benign viral disease with cytokine-driven propagation into expanding infected cell pools and potent malignant transformation . In HIV Clinically, there are two distinct types of the disease: the unicentric and multicentric subtype.It is of pivotal importance to identify unicentric as opposed to multicentric disease at a clinical level in a stepwise approach . UnicentHistologically, CD consists of three variants: hyaline vascular (HV), plasma cell (PC), or mixed (Mixed). The HV variant is described in 90 per cent of CD cases and is characterized by the proliferation of capillary vessels in germinal centers of lymphatic follicles ; germinaThe distribution of localized CD has been reported to be 65 per cent in the mediastinum , 16 per Ultrasonography usually reveals a hypoechogenic and homogenous mass. Computed tomography shows a solid homogenous hypervascular mass when the tumor diameter is less than 5 centimeters, whereas larger tumors (>5 centimeters), because of necrosis or fibrosis, tend to have more heterogeneous enhancement with a central low-attenuation area. Three patterns of involvement have been described, including a solitary noninvasive mass (most common: 50 per cent of cases), a dominant infiltrative mass with associated lymphadenopathy (40 per cent of cases), and matted lymphadenopathy without a dominant mass (10 per cent of cases) . At magnCurative resection, radiotherapy, steroids, immunotherapy such as interferon-alpha or anti-IL-6 antibodies, and combination chemotherapy such as cyclophosphamide, vincristine, and doxorubicin have all been used to manage the disease. Recently, neoadjuvant therapy with rituximab has been suggested . CompletFaced with the diagnosis of the multicentric subtype of CD, there is no curative indication for surgery because outcomes are at best similar to those obtained with various forms of immunochemotherapy. At present, the role of the surgeon in cases of multicentric CD should be limited to gaining tissue by an appropriate biopsy and to debulking of dominant foci of multicentric disease in presence of specific organ-related indications .The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumors and malignant neoplasms that can be either primary or metastatic lesions. Castleman's disease should always be included in the differential diagnosis of any hypervascular and heterogeneous tumor mass in the retroperitoneum. The localized hyaline vascular form presents as a solitary lesion which can be located mainly in the mediastinum, but also in the abdomen, in retroperitoneum, or in many other locations. Complete surgical excision is curative; recurrences have only been described after incomplete resection.


Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist's report. Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later. Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist's report diagnosed a middle ear adenoma. Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour's natural course and risk of recurrence is lacking. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Their cell line of origin, natural history, and progression if left untreated are unknown. Patients generally present with conductive hearing loss and most surgeons advocate surgical excision with or without immediate or delayed reconstruction of hearing.A 52-year-old patient was referred by a private ENT doctor to the University Hospital Zurich on suspicion of a right sided cholesteatoma. The patient described a loss of hearing and pressure sensation in the right ear which had been present for two months. The patient reported no pain, otorrhoea, dizziness, or systemic signs of infection. Past medical history was unremarkable. The patient took no medications and had no allergies.Otoscopy showed an intact eardrum but with the suggestion of white mass behind the eardrum in the posterior superior/inferior quadrants. A pure tone audiogram  showed nThe MRI showed a 6 × 10 mm soft tissue mass, isointense on T1 and T2 sequences, with inhomogeneous gadolinium contrast uptake in the meso- and hypotympanum. Posterior to this was a 5 mm lesion, hyperintense signal on T2, without contrast uptake and without reduced apparent diffusion coefficient. All the inner ear structures were intact. These findings were felt to be more consistent with a glomus tumour than a cholesteatoma and the second lesion was felt to be artifact from a shine-through effect from mucosal swelling.The patient underwent an explorative mastoidectomy, epitympanectomy, and tympanoplasty.Intraoperatively , the whoPathological examination revealed a glandular and neuroendocrine tumor with areas of pleomorphic nuclei Figures . ImmunohA postoperative MRI scan performed 3 months later showed a discrete mucosal swelling in the hypotympanum with contrast uptake, possibly consistent with residual tumour. A further MRI scan 6 months later showed regression of this finding.A full year after the initial excision, a second look was performed. No signs of persistence or recurrence were found, and a partial ossicular reconstruction prosthesis (PORP) was performed with good results see .Middle ear glandular neoplasms are rare tumours thought to arise from the epithelial lining of the middle ear or a stromal precursor derived from the neural crest . MEAs weMEAs are grossly vascular tumors which are well circumscribed despite having no capsule. The most common presenting symptom is conductive hearing loss, though otalgia or a sensation of aural fullness is also commonly described. The mass tends to surround the ossicles without erosion, though in 8 other case reports, as with our case, bony erosion has been described. Facial paresis , 6 due tIn 2009, Saliba and Evrard performed a thorough analysis of the literature and proposed a 3-tier classification according to immunohistochemistry (+/−) and metastasis (+/−). When both are negative, the tumor is described as an MEA. The most common type however shows positive immunohistochemistry and no metastasis and is described as a neuroendocrine adenoma of the middle ear. The rarest finding is of a tumor with both positive immunohistochemistry and metastasis/carcinoid syndrome and is described as a carcinoid tumor of the middle ear [Pathologically MEAs are composed of exocrine and neuroendocrine cell types, sometimes with neuropeptides such as chromogranin, synaptophysin, serotonin, and pancreatic polypeptide . They arTreatment is surgical excision with or without the ossicles. Local recurrences rates as high as 12.7% are reported  and requMiddle ear glandular neoplasms are rare tumors of the middle ear. Controversy still exists as to the most appropriate description and classification of these tumors and whether they should be subclassified in order to aid treatment and prognostication. Clinical suspicion of such a tumor mandates a thorough radiological workup, and patient fitness/consent aside, operative excision. This should be aggressive in order to keep down local recurrence rates and simultaneous or delayed reconstruction of the hearing may then be performed. Long term followup is recommended to rule out late recurrence and metastatic disease.Preoperative radiological workup does not always correlate with intraoperative findings or with the ultimate diagnosis. Clinicians should keep an open mind and be prepared to change their operative plan according to intraoperative findings.Middle ear glandular neoplasms are rare and controversy exists as to their appropriate classification.Currently, the classification by Saliba et al. is the most widespread and includes  neuroendocrine adenomas, adenomas, and carcinoid tumours according to immunohistochemical markers and metastasis.Adjuvant radiotherapy of chemotherapy is not recommended. Instead, a surgical second look can be performed, especially if interval imaging is suggestive of persistent disease.


Meningioangiomatosis (MA) is a rare meningiovascular malformation or hamartomatous lesion in the central nervous system. Radiographic findings of MA may show a variety of characteristics according to different histological components. We present three cases of sporadic MA with different imaging appearances in an attempt to identify specific imaging characteristics.b = 1,000 s/mm2). The preoperative diagnoses of the above three cases were meningioma, hemangioblastoma, and ganglioglioma. However, all were pathologically diagnosed as MA.In case 1, an irregular hyperdense solid mass was localized in the left middle cranial fossa, demonstrating low and equal signal intensity on T1-weighted imaging , high signal intensity with multiple flow void effect on T2-weighted imaging , and significant and homogeneous enhancement on post-contrast magnetic resonance imaging (MRI). In case 2, the lesion in the right insular lobe showed a cystic-mural nodule pattern. The cystic content demonstrated similar density or signal intensity as cerebrospinal fluid, while the mural nodule demonstrated equal density or signal intensity on computed tomography (CT) and MRI. On post-contrast MRI, the mural nodule showed significant enhancement, but the cystic wall and content showed no enhancement. In case 3, a remarkably enhanced solid nodule was found in the cortex of the left parietal lobe with multiple small cysts surrounding it. This nodule showed low signal intensity on T2WI and diffusion-weighted imaging , gyriform hyperintensity on T2-fluid attenuated inversion recovery (FLAIR) sequence, and susceptibility artifacts on T2 gradient echo (GRE) sequences (as found in the literature). MA may A 73-year-old female patient had a history of binocular diplopia for 1 week. Physical examination showed a limitation in abduction movement in the left eye. A head computed tomography (CT) scan showed an irregular mixed hyperdense mass in the left middle cranial fossa   followed by the frontal lobe (25%), but MA accompanied by NF has been shown to mostly occur in the frontal lobe (35%) ,7. Atypiet al. [The pathogenesis of MA remains unclear. Proposed hypotheses include hamartoma with degenerative changes, leptomeningeal meningioma with invasion in adjacent brain tissue, and cortical vascular malformation. MA was formerly classified as a neurocutaneous syndrome, and some cases associated with NF supported the theory of a hamartoma . Althouget al.  found thHistopathologically, MA has been regarded as a hamartomatous, reactive, or neoplasic lesion originating from multivariate cells, such as meningothelial, fibroblastic, myofibroblastic, smooth muscle, or pluripotent cells . The chaImmunohistochemical staining of MA was relatively unhelpful for diagnosis. Positive vimentin expression reflected the proliferation of blood vessels and perivascular fibroblast-like cells in almost all cases. However, the results of other immunohistochemical markers, such as epithelial membrane antigen (EMA), S-100 protein, and glial fibrillary acidic protein (GFAP), were variable in the literature [According to different percentages of perivascular cell proliferation and cortical vascular proliferation, MA may be broadly classified into predominantly cellular or vascular types . PredomiIt has been observed that there are no specific MA characteristics upon MRI or CT; thus, correct preoperative MA diagnoses were difficult. MA has always been misdiagnosed as meningioma, oligodendroglioma, ganglioglioma, metastasis, or arteriovenous malformation. With regards to the location of lesions, they may manifest as intra-axial or extra-axial lesions with well- or ill-defined margins because of the cortex invasion. CT scans show that lesions exhibit different densities or even normal appearance, but calcification may be a specific indication for MA. The probability of calcification is variable, and the highest observed was 89.6% . The siget al. [N-acetyl aspartate (NAA) peak on MRS, which suggested a proliferating tumor of presumed non-neuronal origin. The high Cho peak could have been a reflection of the proliferation of meningothelial cells and/or fibroblasts that formed the concentric cuffs surrounding blood vessels in the cortex. However, the high Cho peak could have also been observed under other conditions, for example, high-grade brain tumors [Some specific signs on MRI may be helpful for MA imaging diagnoses. Gyriform hyperintense on T2-fluid attenuated inversion recovery (FLAIR) sequence has been reported to be the most prominent characteristic for sporadic MA on MR imaging, which can be attributed to a thickened cortex with proliferating leptomeningeal vessels interwoven with bands of fibrous connective tissue . Calcifiet al.  have repn tumors .et al. [We presumed that the MA imaging appearances could be divided into two patterns: solid and cystic. Solid MA was more common than cystic MA and demonstrated a higher probability of calcification and remarkable enhancement in most cases. Less than ten cases with cystic MA have been reported -23, inclet al.  speculatet al. . AnotherDifferential diagnoses for MA are not easy due to variable radiological appearances. Extra-axial tumors, such as meningiomas, are the most common differential diagnosis. Intra-axial tumors in the superficial cortex, such as oligodendroglioma, ganglioglioma, and dysembryoplastic neuroepithelial tumor (DNET), are also frequently considered . HoweverSurgical resection is important not only for seizure control but also for pathological diagnosis. Most MAs are completely removed without recurrence. However, long-term seizures disappeared in only 43% of patients after operation, and antiepileptic drug administration was required in greater than 70% of patients ,25.The cases in this study demonstrated that MA may present with solid or cystic imaging patterns. Imaging diagnoses for MA were difficult due to non-specific characteristics. Several signs on MRI may be helpful, such as gyriform hyperintensity on T2-FLAIR sequences, susceptibility artifacts on T2 GRE sequence, and flow void effects on T2WI as in our case. Cysts in cystic MA demonstrate variable appearances in location, number, and size, but separation from solid masses or nodules may be one of its specific characteristics.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Laryngeal schwannomas are rare, benign neurogenic tumors. They normally present as a slow-growing, encapsulated, submucosal mass in the supraglottic region. We describe a 20-year-old female presenting with a 2-year history of hoarseness and progressive worsening dyspnea. Fiberoptic laryngoscopy and computed tomography revealed a round, low-density submucosal mass at right false cord and arytenoepiglottic regions with glottic extension. Microlaryngoscopic biopsy and debulking for this solid tumor were performed without tracheostomy. Schwannoma was confirmed by histopathological study. However, rapidly worsening stridor occurred 2 weeks after the surgery. Fiberoptic laryngoscopy showed an exophytic tumor occupying the right hemilarynx with airway compromise. Definite complete excision of the tumor was performed by right vertical hemilaryngectomy. At 5-month follow-up, the laryngeal wound was clear without signs of recurrence. Rapid occurrence of airway obstruction after debulking and biopsy was demonstrated in this case. Vertical hemilaryngectomy was inevitable to cure this potentially life-threatening laryngeal schwannoma in this young female with postoperative serviceable voice. Schwannomas were first described in 1908 by Verocay ,3.A 20-year-old Taiwanese female presented with a 2-year history of hoarseness and progressive dyspnea. Submucosal bulging in the right supraglottic area was found by her ear, nose and throat physician 1 year previously, but the patient did not wish to undergo any treatment. She had never smoked and had no throat symptoms before hoarseness developed. She denied any known systemic disease. General physical examinations were unremarkable except for moderate obesity.Fiberoptic laryngoscopy revealed a round, submucosal bulging at the right false and true vocal folds Figure A. The ri2 laser were performed , but they can develop in the subglottic region in extremely rare cases ,7. SchwaThe clinical symptoms are related to the mass effect of a slowly growing lesion in the larynx: the patient gradually develops hoarseness, globus sensation and dysphagia over years rather than weeks or months . As the The diagnostic work-up in such cases should include fiberoptic laryngoscopy, image study, and histological biopsy. On laryngoscopy, the characteristic finding is a round submucosal bulge confined to the aryepiglottic fold or false vocal cord. This was in concordance with our patient. It may obstruct the view of the laryngeal inlet or result in reduced mobility of the vocal cord.  Image stSince endoscopic assessment and imaging studies cannot rule out malignancy, the definite diagnosis can only be made histologically. Enger and Weiss established three histological criteria for the diagnosis of schwannoma: the presence of a capsule, the presence of a stromal Antoni A  and/or Antoni B (loosely arranged spindle cells within a myxoid matrix) histological pattern, and positive staining with S-100 (characteristic of Schwann cells) . All of To prevent recurrence, treatment of laryngeal schwannomas is based on complete surgical resection. In addition, preservation of laryngeal function and protection of the laryngeal mucosa from surgical injury are the keys to better surgical outcome.2 laser microsurgery can be a reasonable treatment option [Surgical intervention should be planned according to the symptoms of each patient, as well as the location and extent of the tumor. For smaller tumors with adequate endolaryngeal exposure, transoral COt option ,12. Moret option . Larger t option .2 laser microsurgery or median thyrotomy [2 laser and biopsy could only be used initially due to the location and size of the tumor. The reasons for the rapid enlargement and return of obstructive symptom within 2 weeks in our particular patient were considered to be tumor inflammation and infection. Besides, malignancy needed to be ruled out in this aggressive clinical presentation. Hence, vertical hemilaryngectomy was inevitable as the definitive surgery. Extreme care was used to preserve as much normal endolaryngeal mucosa as possible in order to facilitate favorable clinical outcome in our patient.Rapid return of symptoms after initial biopsy or surgery has been described for supraglottic laryngeal schwannomas, and these patients were eventually managed with transoral COhyrotomy ,11. In tLaryngeal schwannomas are rare, benign neurogenic tumors. In this young female, vertical hemilaryngectomy was inevitable for her potential life-threatening, infected, large supraglottic schwannoma with glottic and subglottic involvement.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.


Purpose. Implant therapy plays an important role in contemporary dentistry with high rates of long-term success. However, in recent years, the incidence of peri-implantitis and implant failures has significantly increased. The peripheral giant cell granuloma (PGCG) rarely occurs in peri-implant tissues and it is clinically comparable to the lesions associated with natural teeth. Therefore, the study of possible diseases associated with dental implants plays an important role in order to be able to diagnose and treat these conditions. Materials and Methods. This report described a 60-year-old Caucasian male who presented a reddish-purple pedunculated mass, of about 2 cm in diameter, associated with a dental implant and the adjacent natural tooth. Results. An excisional biopsy was performed and the dental implant was not removed. Histological examination provided the diagnosis of PGCG. After 19-month follow-up, there were no signs of recurrence of peri-implantitis around the implant. Conclusion. The correct diagnosis and appropriate surgical treatment of peri-implant giant cell granuloma are very important for a proper management of the lesion in order to preserve the implant prosthetic rehabilitation and prevent recurrences. Peripheral giant cell granuloma (PGCG) is an exophytic lesion of the gingiva, arising from the periosteum or from the periodontal membrane in response to local irritating factors or a chronic trauma –3. It clPGCG mainly affects female patients (60% of cases) and, although it can occur at any age, has a peak incidence between 40 and 60 years , 4. It iThe maximum capacity of expansion of PGCG is unknown because it is usually surgically removed in early proliferative stages, but lesions of maximum 5 cm diameter in patients with poor oral hygiene or xerostomia have been reported . The PGCThe etiopathogenesis of PGCG is still uncertain: the lesion is thought to be a reactive localized tissue response towards several possible etiologic factors as dental plaque, calculus, tooth extractions, denture irritation, periodontitis, food impaction, orthodontic therapy, inadequate restorations, ill-fitting dentures, and hormonal disturbances, like hyperparathyroidism –10.The treatment of PGCG includes surgical excision and elimination of the possible etiologic factors , 12.Histologically, PGCGs show the presence of nonencapsulated highly cellular mass with abundant multinucleated giant cells, inflammatory cells, interstitial haemorrhage, hemosiderin deposits, and mature bone . Some auPGCGs associated with dental implants are very rare. The first case was described by Hanselaer et al. in 1984  and onlyThe aim of this paper is to report a successful clinical and surgical management of PGCG associated with a dental implant and the adjacent natural tooth.A 60-year-old Caucasian man referred to the Department of Oral and Maxillofacial Science, “Sapienza” University of Rome, with a chief complain of swelling in the anterior zone of the palate. His medical history was negative and he was not taking drugs at the time.Intraoral examination revealed a nonulcerated red-purple pedunculated mass, of about 2 cm in diameter, with elastic consistency and smooth erythematosus surface, arising from the gingival margin of the anterior right palatal region . The lesThe radiographic evaluation  revealedThe diagnostic hypotheses of peri-implant infection, granuloma, and trauma reaction were carried out and an excisional biopsy was programmed, with the decision of maintaining dental implant.Vestibular and palatal excisional scalpel biopsy was performed in local anaesthesia Figures , with anThe specimens were fixed in 10% formalin solution for histologic examination .There were no complications in the postoperative period and the clinical examination of the surgical site showed good wound healing after 7 days Figures  and compAt 19-month follow-up, there were no clinical or radiological signs of infections or recurrence Figures . A soft Histological examination showed the presence of focal clusters of multinucleate giant cells dipped in a richly vascularized connective tissue containing spindle or ovoid mononuclear cells. Giant cells present numerous variations of size, shape, and number of nuclei. A mixed inflammatory infiltrate is also observable in the vascular stroma. The histological section was compatible with the diagnosis of PGCG .PGCG infrequently arises from peri-implant tissues: only other 15 cases have been described before this one in the English literature –29. The The etiopathogenesis of PGCG associated with dental implants is not yet totally clear because the cases reported in the literature are still too few. Since PGCG is a reactive lesion, it is conceivable that some kinds of irritating factors play a determining role in the manifestation of this disease , 8–10. IClinical difficulties associated with this disease result from the necessity of being able to perform the differential diagnosis of PGCG. Other gingival reactive lesions such as pyogenic granuloma and peripheral ossifying fibroma clinically appear very similar to PGCG and thus can be distinguished after histological examination. Pyogenic granuloma's histological appearance is characterized by the presence of a mass of angiomatous tissue with a variable amount of collagen in the connective tissue. Peripheral ossifying fibroma is a reactive lesion arising from the periodontal ligament and microscopically shows a well represented mineralized bony tissue immersed into a stromal fibroblastic proliferation; some authors reported a possible case of PGCG and peripheral ossifying fibroma hybrid lesion, with distinct areas having histological characteristics of both diseases .Central giant cell lesions in advanced stages may erode the bone surface and reach the lining soft tissue; in these cases, radiological evidence of bone resorption is diriment.In this specific case, having the authors hypothesized the diagnostic phase of the reactive nature of the lesion, they considered that preserving the implant by performing excisional biopsy and accurate curettage of the surrounding bone tissue is possible.The correct diagnosis and appropriate surgical treatment of peri-implant giant cell granuloma are very important for proper management of the lesion in order to focus on a correct differential diagnosis with other soft tissues peri-implant diseases and with the aim of preserving the implant prosthetic rehabilitation and preventing recurrences.


True hermaphroditism represents only 5% cases of all of disorders of sexual differentiation (DSD) and usually present in early childhood with ambiguous genitalia. Occasionally, cases might present later on in adolescence with problems of sexual maturation. Our case report presents a true hermaphrodite with normal male phenotype that presented as a left testicular mass, two years after being diagnosed with Sertoli cell only syndrome in the contralateral testis. Histological examination of the left testis showed ovarian, fallopian tube, myometrial, endometrial, and epididymal tissue. This combination of findings is found in approximately one-third of true hermaphrodites, but it is very rare to present clinically as an inguinoscrotal mass. The term disorder of sexual differentiation (DSD) refers to a child born without clear male or female phenotype . The MetTrue hermaphrodites usually present in early childhood with ambiguous genitalia, although presentation in early adulthood is not rare. Late diagnosis of true hermaphrodites may have a severe psychological impact on the patient, hence making early detection and treatment essential . Our casA 22-year-old gentleman presented in 2012 with right testicular pain. On examination the patient was a phenotypically developed male with normal external male genitalia. His past medical history included recurrent left hypospadias and bilateral mammoplasty. He underwent bilateral testicular exploration during which the right testicle was found to have abnormal features and was biopsied. Histologically there was no malignancy but the seminiferous tubules showed Sertoli cell only syndrome .Two years later the patient presented with a 2-day history of left dull hemiscrotal ache. On examination a rubbery hard large left testicle was palpated. In view of suspected neoplastic growth an ultrasound was done. This showed an atrophic right testicle and an avascular left testicle. The ultrasound was repeated and confirmed a necrotic left testicle. In view of these findings and the probability of malignancy the patient underwent radical left orchidectomy.The histology of the left gonad showed proper cycling ovarian parenchymal tissue with primary and secondary follicles, a corpus luteum, and several corpora albicantia. Other sections showed mature fallopian tube tissue. A second solid nodular mass attached to the gonad was in fact a functional small uterus Figures –5. A ruA karyotype was conducted and this revealed 46 XX/XY chimera with 11% of the population of cells being XX and 89% of the cell population being XY. Hormonal tests revealed an FSH level of 26.3 U/L (0.711.1 U/L), LH level of 9.8 U/L (0.8–7.6 U/L), testosterone level of 15.50 nmol/L (5.55–25.2 nmol/L), and an oestradiol level of 135 pmol/L (0–206 pmol/L).The diagnosis of a true hermaphrodite depends on the histologic confirmation of testicular and ovarian tissue in the same individual , 4. 90% Lateral: testis and a contralateral ovary (30% of cases).Bilateral: both testicular and ovarian tissue, usually represented by an ovotestis that is identified on both sides (50% of cases).Unilateral: ovotestis on one side and a testis or ovary on the other side (20% of cases) .The descent and position of the gonad depend on the amount of testicular tissue present [The most common gonad variant found in a true hermaphrodite is an ovotestis, with 50% being found in ovarian position on the right hand side. Ovaries are present in 33% of cases while testicles are found in 22% . The mosA vas deferens and epididymis are formed beside a testicle, while a fallopian tube accompanies an ovary in the majority of cases. Ductal development is variable in the case of an ovotestis. 65% of the ovotestes are accompanied by a fallopian tube while a vas deferens accompanies the rest. The development of a uterus can occur in the presence of an ovotestis/ovary combination .70% of cases of true hermaphrodites have 46 XX karyotype . Only 10The risk of malignancy ranges from 2.6% to 4.6%, although in true hermaphrodites it is lower than in other types of DSD . The mosImaging modalities that may aid in the diagnosis of true hermaphrodites include ultrasound, MRI, and genitography . UltrasoUltrasound is a noninvasive, cheap procedure that should be used as initial screening for assessment of developmental sexual disorders, especially when different approaches  are used . MRI exaLaparoscopy is also helpful in diagnosing patients with DSD and in the planning of corrective surgery. Biopsy and gonadectomy may also be performed with this technique .Diagnosis of DSD in a patient should ideally be made before the age of 2 years, since psychological problems are more likely after this age . The aimTreatment in cases of DSD may involve medical treatment, surgical correction of ambiguous genitalia and removal of dysgenic gonads or mullerian components, and psychological counselling, especially in patients who have presented during their adolescence or adulthood . If surg


Although a thyroglossal duct cyst is a congenital anomaly, it can also appear in adults. Despite the presence of embryological remnants, it is still unclear why the cyst should suddenly develop later in life. We report a case of a 46-year-old male with an extravasation mucocele arising from a long-standing lingual thyroglossal duct remnant. MRI demonstrated a lingual cystic lesion near the hyoid bone associated with a suprahyoid tract-like structure masquerading as a thyroglossal duct cyst. However, histopathological examination demonstrated a mucocele secondary to a rupture of a thyroglossal duct remnant with numerous intramural heterotopic salivary glands. We propose a new mechanism of an acquired cystic formation of this congenital disease that excessive production of mucus from heterotopic salivary glands and a physical trauma such as swallowing may lead to extravasation of mucus from the thyroglossal duct. A cyst of the thyroglossal duct is the most common congenital neck mass . AlthougA 46-year-old male with a two-year history of globus sensation was referred to our hospital because of a tumor at the base of his tongue. He did not complain of sore throat or dysphagia. His voice sounded normal and no tumor was found in the neck. His symptoms gradually worsened, especially in the supine position. Flexible fiber-optic examination revealed a smooth round mass in the midline of the base of the tongue. MRI of the neck revealed a well-circumscribed cystic lesion coupled with an ascending tract from the hyoid bone towards the base of the tongue Figures . The thyThis patient explains the phenomenon of delayed presentation of a cyst from an embryological remnant after having been dormant in the neck for years. The thyroglossal duct is an embryological remnant of the pathway taken by the thyroid anlage as it descends from the region of the foramen cecum in the back of the tongue to its final position low in the neck. This duct normally disappears by fifth to tenth week of life . If a poA cyst may develop anywhere along the course of the thyroglossal duct; however, only 2% are found at the base of the tongue . There aSalivary gland tissue is reported within the thyroglossal duct remnant in 1.6–60% , 8 of paIn conclusion, we report a case of a 46-year-old male with an extravasation mucocele arising from a long-standing lingual thyroglossal duct remnant, which was masquerading as a thyroglossal duct cyst on MRI. This finding suggests a possible mechanism of late-onset cystic development from the persistent thyroglossal duct remnant in the tongue.


We report a case of retroperitoneal mature cystic teratoma in a 2-day-old neonate. Diagnostic and surgical procedure including its complexity and relevant literature review has been discussed.  A 2-day-old female neonate, weighing 2.7 kg, antenatally diagnosed at 30 weeks of gestation as a case of abdominal mass with polyhydramnios in primipara mother referred to our department for further management. Baby was delivered normally. Abdominal examination revealed a lump in left side of abdomen extending from left hypochondriac region to left iliac fossa and crossing midline in umbilical region. Lump had variegated consistency, nodular surface, clearly defined margins and restricted mobility. Abdominal X-ray showed bowel loops pushed to right side of abdomen by a space occupying lesion in left side with foci of calcifications in it. Post natal abdominal ultrasound revealed a large heterogeneous solid cystic lesion with few echogenic areas and calcifications. Computed tomography scan of abdomen revealed 10.8 x 8.3 x 8.4 cm heterogeneously enhancing cystic/solid mass lesion with multiple foci of discrete calcifications in retroperitoneum . Lesion was partially encasing aorta, and displaced major vessels around. Serum alpha-fetoprotein (AFP) level was 28675 IU/ml. Tru-cut biopsy was taken under anesthesia which came out as mature teratoma. Baby underwent laparotomy at day 24 of life. A large retroperitoneal tumor (12 x 8 cm) consisting of solid and cystic areas occupying mainly left flank and central abdomen. The tumor was adherent to the surrounding structures namely the root of mesentery, superior mesenteric vessels, inferior mesenteric vessel, duodenum, colon, and left kidney and renal vessels. Tumor was displacing aorta and inferior vena cava to right side. However, there was no infiltration into any vessels or organs neither was any significant lymphadenopathy noted. Left kidney was found to be pushed down into pelvis by tumor. Tumor (800 gm) was excised in toto . Post-operatively, the patient was kept on ventilator and extubated on 2nd post-op day. Baby was discharged on 11th post-op day on full oral feeds and followed up monthly. Serum AFP at 2 months and 6 months were normal. Baby has gained adequate weight and doing well. Histopathological examination of tumor revealed presence of neural tissue, spindle cells, cartilage, fibroblasts, hypoplastic bone.Teratoma is the commonest congenital tumor. The usual site for congenital teratoma is sacrococcygeal, the other sites being the mediastinum, head and neck, oropharynx, pericardium, and the retroperitoneum [1-3]. Retroperitoneal teratoma comprises 3.5-4% of all germ cell tumors in children and 1-11% of primary retroperitoneal neoplasms [4-6]. Patients usually present with abdominal distension or a palpable mass. An accurate diagnosis of a teratoma cannot be made on clinical basis. Radiological features include presence of calcification, teeth and fat, however calcification cannot be considered an indicator of a benign tumor since 12.5% of calcified tumor are malignant. Ultrasound of abdomen is usually the first imaging modality used in pediatric abdominal mass. CT scan is useful to delineate the extent of the disease in retro-peritoneum and its relationship to major vessels. Serum alpha-feto protein level is good indicator for diagnosis and assessing the recurrence of tumor. Malignancy is uncommon in retro-peritoneal teratoma hence non mutilating excision is possible and should be attempted . The prognosis of neonatal teratoma is favorable with an 80-100% survival reported after surgical excision of the tumor and treatment of any recurrence . In our case, the patient was a neonate and retroperitoneal teratoma is very unusual in this age group and at this site. Postoperative recovery was uneventful. At present there is no sign of recurrence of malignancy at follow up of 10 months. To conclude, retroperitoneal teratoma can present in neonates however early diagnosis and management like done in the index case can give good outcome.Source of Support: NilConflict of Interest: Nil


The topic of this case report is a rare subconjuctival osseous choristoma that corresponded to the left lateral sunconjunctiva and canthus. A 20-year-old man was asymptomatic when he arrived for the examination. His full ophthalmic examination was normal. Orbital computerized tomography was concordant with osseous lesion. Osseous choristomas are the rarest forms of ocular choristomas, they are usually being defined as sporadic, and they are found at the superior temporal region of the episclera. In our case, choristoma was in the lateral canthus of the left eye. We had administered surgical excision by reason of the patient's cosmetic requirement. We had noted that the lesion was adherent to conjunctiva but not to the sclera and the muscles. After surgical treatment, we saw mature heterotrophic osseous tissue in subconjunctival area and Haversian canals in compact bone tissue. Osseous choristomas are solid nodules, composed of mature, compact bone together with pilosebaceous units and hair follicles. The topic of this case report is a rare osseous choristoma that localized to the left lateral subconjunctiva, in which underlying sclera was not involved.A 20-year-old man was asymptomatic when he arrived for the examination. In his medical history, he had a swelling on the left corner of the left eye since his birth. He had no medical problems or trauma. He has never used alcohol; however, he was smoking. His family thought that there was growth in the lesion compared to the past. His visual acuity was 20/20. External examination of the right eye, anterior segment, and fundus examination of both eyes were normal.The swelling was approximately 6.0 mm × 6.0 mm subconjunctival lesion on the left eye . It was Informed consent was obtained for the local anesthesia. Intraoperatively, the lesion was adherent to conjunctiva but not to the sclera and the muscles. The mass and the adherent conjunctiva were removed totally and the remaining conjunctiva tissue was stitched edge to edge. The swelling was sent to histopathology immediately. Its result was episcleral osseous choristoma. Histopathologically, mature heterotrophic osseous tissue was seen in subconjunctival area .The term heterotopia (choristoma) is applied to microscopically normal cells or tissues that are present in abnormal locations. Examples of heterotopias include the rest of pancreatic tissue found in the wall of the stomach or small intestine; a small mass of adrenal cells found in the kidney, lungs, ovaries or elsewhere; or as in our case a mass of mature bone cells found under conjunctiva. It was first described by von Graefe as epibulbar osteoma , 2. OculIn a 302-periorbital-tumor-case series, Elsas and Green reported that 33% were choristomas, 29% were nevi, 11% were epidermal inclusion cysts, and 7% were papillomas in children. In the study, there were approximately 100 choristomas; 58% of the choristomas were dermoid cysts, 30% were dermolipomas, 6% were dermoid choristomas, and 6% were teratomas [The other reported localizations of extraocular choristomas were the oral cavity, usually in the tongue and also at other soft tissues of the head and neck .The differential diagnosis includes epibulbar dermoids, epithelial inclusion cysts, limbal dermoids, prolapsed orbital fat, papillomas, dermolipomas, and complex choristomas.Osseous choristomas are the rarest forms of ocular choristomas, they are usually being defined as sporadic, and they are found at the superior temporal region of the episclera , 7. GayrIn literature, there are very few pieces of information about hair follicles localized in the conjunctiva, overlying the choristoma. In our case there were multiple hair follicles, localized only in the overlying conjunctiva of the choristoma. Intraoperatively, the mass and the adherent overlying conjunctiva were excised and the rest of the conjunctival tissue was stitched edge to edge.The lesions are generally adherent to extraocular muscle sheath, overlying conjunctiva or underlying sclera . We had Surgical excision is needed when choristomas get symptomatic and also for cosmetic reasons, although choristomas are stable lesions and usually grow slowly , 4, 7, 8Histopathological images of the choristomas show mature compact bone, surrounded by fibrous connective tissue , 7. TypiIn our case, the choristoma was localized in the left eye, was not adherent to the underlying sclera and the muscles, and had hair follicles, contrary to general literature articles. As a result, rarely seen episcleral osseous choristoma should be considered when a mass is seen in any eye, gender, and age, especially localized superotemporally.


We report a very rare case of acute abdomen caused by torsion of an accessory spleen that was preoperatively diagnosed and cured by single-port surgery. A 31-year-old woman was admitted to our hospital with severe left abdominal pain. Physical examination revealed a left upper quadrant abdominal tenderness with voluntary guarding. Ultrasound demonstrated a well-defined round mass isoechoic to the spleen, measuring 3.0 cm in diameter in the left upper quadrant adjacent to the spleen. A contrast-enhanced CT scan showed a normally enhanced spleen and a 3.0 × 3.0, hypodense, non-enhancing mass anterior to the spleen with a twisted funicular structure. Torsion of an accessory spleen was suspected, and emergency single-port surgery was performed. During surgery, a rounded violet mass measuring 3.0 cm in diameter, suggestive of an accessory spleen, with a 1800° torsion around a long vascular pedicle along the left side of the greater omentum was discovered. The mass was removed and post-operative recovery was uneventful. A review of the literature revealed 26 cases (including ours) of torsion of an accessory spleen in English. Even with the recent advances in radiologic imaging modalities, making a preoperative diagnosis of this is difficult and most cases are diagnosed during laparotomy. This is the first report preoperatively diagnosed and cured by single-port surgery. We decided to start the operation by using a single port, not only for cosmetic reasons for this young female patient, but also for final confirmation of our diagnosis. We believe that single-port laparoscopy is valuable as a diagnostic tool as long as safety is assured for patients with acute abdomen. Although torsion of an accessory spleen is extremely rare, it should be considered in the differential diagnosis of acute abdomen in children and young adults.The online version of this article (doi:10.1186/s40792-015-0101-x) contains supplementary material, which is available to authorized users. Accessory spleen is a congenital anomaly characterized by ectopic tissue separated from the main body of the spleen. It is a relatively common condition that appears in 10 to 30 % of autopsy findings and is usually asymptomatic . It is dHerein we report a rare case of an acute torsion of an accessory spleen as an emergency acute abdomen case in a young female patient, that was successfully diagnosed preoperatively. She was subsequently operated on using laparoscopic single-port surgery which has recently been developed. To the best of our knowledge, this is the first acute case of treatment by single-port surgery following preoperative diagnosis.A 31-year-old otherwise healthy woman was admitted as an emergency with intense left abdominal pain. At the time of admission, she had pyrexia (38.5 °C). Physical examination revealed a left upper quadrant abdominal tenderness with voluntary guarding. She was only found to have an elevated level of serum C-reactive protein. Complete blood cell count was unremarkable. Ultrasound (US) demonstrated a well-defined round mass isoechoic to the spleen, measuring 3.0 cm in diameter in the left upper quadrant adjacent to the spleen Fig. . This twThe operation was performed by single-port laparoscopy. Surgical glove method with a 1.5-cm umbilical incision was used, as we have previously reported , 5. OperAccessory spleens are found in 10 to 30 % of autopsy findings . They caAn accessory spleen without an underlying disease such as a haematological disorder or cirrhosis of the liver may occasionally present as an enlarged mass and/or acute abdomen. Emergency surgical intervention may be necessary if these events are accompanied by infarction, haemorrhage or rupture. Torsion of an accessory spleen leading to acute abdomen has been reported in literature since Alexander and Romanes first reported it in 1914 . TorsionIn fact, in our case, we made a successful diagnosis using both US and CT findings, which showed an isoechoic mass to the spleen and an avascular mass with twisted pedicle, respectively. In particular, the CT scan clearly showed a twisted pedicle, which was the biggest clue in making our correct diagnosis. Nonetheless, preoperative diagnosis is only hypothetical, even though it seemed correct in our case, and torsion of an accessory spleen is so rare that it is impossible to diagnose with 100 % confidence. We decided to start the operation by using a single port, not only for cosmetic reasons for this young female patient, but for final confirmation of our diagnosis. We are able to say that laparoscopy is a good diagnostic tool for acute abdomen and single-port laparoscopy is an alternative solution for diagnosing acute abdomen because if the diagnosis is different from the preoperative findings, the surgeon can easily convert to conventional laparoscopic surgery by adding as many ports as he/she needs or even switching to open surgery by making an adequate skin incision. In our case, once the diagnosis had been confirmed, we could continue with the accessory splenectomy. As an accessory splenectomy in itself is a simple procedure, the benefits for the patient are clear, i.e. much better cosmetic results and less pain than with open surgery.Torsion of an accessory spleen should be considered in the differential diagnosis of acute abdomen in children and young adults. Awareness of this entity and familiarity with typical imaging findings are important. We believe that single-port laparoscopy is valuable as a diagnostic tool, and as long as safety is assured, this method has clear benefits for patients with acute abdomen.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Adenoid cystic carcinoma (ACC) of the trachea is rare, it represents 1% of all respiratory tract cancers. It's generally considered as a slow-growing, with pronlonged clinical course. Most patients present with dyspnea, and the symptoms often mimic those of asthma or chronic bronchitis Surgical resection is the mainstay of treatment often combined to radiotherapy because of close surgical margins. When surgery isn't possible, most tumors respond to radiotherapy alone wich often results in long periods of remission We report two cases of primary ACC of trachea: a 49 year old male presented a distal unresectable tracheal ACC treated with chemo-radiotherapy who developed a recurrence and died 7 years after the diagnosis. And a 50 years old female with a proximal tracheal tumor treated by surgical resection and end- to- end anastomosis followed by adjuvant radiotherapy. At 10 months follow-up, our patient shows no evidence of disease with negative histological findings. Tumors of tracheal origin are exceedingly rare; the incidence of primary tracheal tumors is less than 0,2 per 100,000 persons per year . The cliA 49 year old male developed since 2 months dyspnea with dry cough and thoracic pain. He had smoked 1 pack/day cigarettes for 18 years and has been ex-smoker for 9 years. In November 2004, he was admitted with almost complete atelectasis of the left lung at the chest X-ray. Chest computed tomography (CT) was performed that showed soft tissue mass surrounding the trachea extending from the left pulmonary hilum through the left main bronchus to the carina. This tumor was in close contact with the descending aorta. Contrast-enhanced CT scans demonstrated no paratracheal adenopathy or pulmonary parenchymal disease . The patA multidisciplinary team of medical, surgical and radiation oncologists made a decision to treat the patient with definitive radiotherapy. The patient received 66 Gray (Gy) of radiotherapy to the primary tumor volume in 33 fractions; 5 fractions per week with exclusion of the spinal cord at 46 Gy. Bronchoscopies performed 6 and 12 months after therapy showed a disappearance of the tumor mass of the left bronchus wich became permeable. The patient was lost sight of for 4 years. In july 2010, he was readmitted with a history of severe respiratory insufficiency at rest. Bronchoscopy revealed an obstructive and hemorrhagic tumor of the left mainstem bronchus invaded the carina, and the right main bronchus, wich prevented us to biopsy. Chest CT showed an extensive tumour regrowth in the mediastinum, with atelectasis of the left upper lobe, and mediastinal retraction to the left side. A palliative chemotherapy (based on carboplatine- paclitaxel) was programmed, but the patient died from tumor progression. The patient has survived 7 years from the first medical assessment.A 50 - year-old female, with no particular history; had complaints of nocturnal dyspnea, intermittent cough for three years and had been treated with various bronchodilators and anti-asthmatic medications without significant results; worsening dyspnea continued without improvement. Her investigations revealed a normal chest X-ray. Chest CT was performed that showed a large polypoid intra-luminal mass arising in the trachea within 1 cm of the larynx, causing obliteration of 70% of the lumen . A flexiPreviously called cylindroma or adenocystic carcinoma, ACC of the trachea is a relatively rare occurrence. The incidence of tracheal tumors is less than 0,2 per 100,000 people per year . ACCs haTreatment options include surgery alone, radiation therapy alone; or a combination. The ideal treatment of ACC is primary resection and end-to-end anastomosis when possible. A surgical resection has been thought to be the most favourable procedure to control localized lesions. The median survival time of resected patients was reported to range from 7,5years to 118 months . BecauseWhen the tumor is considered unresectable due to its local extension, exclusive radiotherapy is given at dose of 66 to 70 Gy in conventional fractionation . ConformThe role of post-operative adjuvant radiotherapy remains uncertain. Some advocate a systematic radiotherapy while others only offer this treatment when margins are invaded , 16. In ACC is a rare primary tracheal malignancy. This disease is commonly misdiagnosed as asthma. Surgical resection followed by radiotherapy is widely recommended protocol for treatment of localized tracheal tumors and provides the best chance of pronlonged survival. Patients with unresectable disease, can benefit long periods of remission due to the use of conformal radiotherapy; wich allows the delivery of high dose with an acceptable level of morbidity.


Splenic artery aneurysms account for about 60% of all visceral aneurysms. Pregnancy is a risk factor for splenic artery aneurysms rupture with high maternal mortality and fetal loss. Intrasplenic arterial aneurysms are extremely rare and have not been reported to be associated with pregnancy. This report presents a 34-year-old woman during the second trimester, admitted with severe left upper quadrant and left shoulder pain. She had two uncomplicated intrasplenic aneurysms. Splenectomy was done. She delivered a full term healthy girl. This is the first report of acute abdomen during pregnancy caused by intrasplenic artery aneurysms with maternal and fetal survival. Intrasplenic arterial aneurysms/pseudoaneurysms involving the intrasplenic arterial branches are very rare. They have the potential for life-threatening complications; and they must be diagnosed and treated immediately. True aneurysms inside the spleen are extremely rare. Posttraumatic intrasplenic pseudoaneurysms following nonoperative management of blunt splenic injuries are seen more frequently. The spontaneous nontraumatic pseudoaneurysms are also encountered. We report a 34-year-old woman in her second trimester, who presented with severe abdominal pain. 9/L. Cross section of the spleen disclosed two aneurysms in its lower part. One was totally intrasplenic while the other protruded into the splenic hilum  and colored Doppler US revealed two splenic cystic lesions: one within the splenic parenchyma (18 mm) and the other at the splenic hilum (16 mm). Both lesions showed arterial blood flow and thus were diagnosed as two splenic arterial aneurysms. The patient improved and was discharged. She was readmitted two weeks later with severe left upper quadrant and left shoulder pain. On physical examination, the patient was in good health but in severe pain. Her abdomen was tender on the left side. There was no history of either abdominal trauma or pancreatitis. She was treated for hypertension during her previous pregnancies. Her laboratory investigations including complete blood count were unremarkable. Abdominal MRI with and without intravenous contrast showed two cystic intraparenchymal and hilar splenic lesions . The lesic hilum . Both anic hilum . Her preSplenic artery aneurysm is the third most common intra-abdominal aneurysm. It is also the commonest visceral artery aneurysm –3. Most Intrasplenic arterial aneurysms (ISAAs) are either true or pseudoaneurysms, the latter being a much more common variety. Our patient had two true aneurysms: one was totally intrasplenic while the second was intrasplenic aneurysm that protruded into the hilum of the spleen.The true ISAAs develop in patients with liver cirrhosis, portal hypertension , 6, infeIntrasplenic pseudoaneurysms (ISPs) result from destruction of the full thickness of the arterial wall. They are either posttraumatic (TISPs) or nontraumatic (NTISPs). The nonsurgical management of blunt splenic trauma gained popularity during the last two decades. TISPs have been reported more frequently, following blunt trauma –16, stabNontraumatic intrasplenic pseudoaneurysms (NTISPs) are rare complications of splenic infarction, infiltration by malignant systemic disorders, infectious process, chronic pancreatitis, and arteritis , 20; theISAAs are usually not calcified and are typically unrelated to atherosclerotic disease, while extrasplenic aneurysms often calcify and are caused by atherosclerosis.Most uncomplicated aneurysms are likely to be asymptomatic. However, left upper quadrant or epigastric pain may occur in patients with intact splenic artery aneurysms. In recent reviews, nearly half of patients with aneurysms complained of abdominal pain. Intact ISAAs may produce pain. Our patient did have pain in the left upper abdomen and left flank for over two weeks prior to admission. The abdominal pain in our patient could be multifactorial. She had splenic enlargement and her aneurysms could have been expanding. Also, the rising estrogen may have contributed to deterioration of her pain. In addition, the hypertension during her pregnancy could have been a factor in the pathogenesis of her aneurysm.Most patients with significant pain are experiencing rupture or acute aneurysmal expansions. Patients with ISAAs expand and then rupture into splenic parenchyma in their way to the peritoneal cavity, causing acute left upper abdominal, left shoulder pain, shock, and abdominal distension. Death can potentially result. Patients with TISPs or NTISPs usually present with intrasplenic, subcapsular, perisplenic, or intraperitoneal rupture . In caseIntrasplenic aneurysms and pseudoaneurysms are initially investigated by US which usually show anechoic lesion in the spleen. It may also identify haematoma in and around the spleen in cases of TISPS . Colour ISAAs potentially expand and cause delayed rupture of the spleen. Thus, early diagnosis and treatment are crucial . If diagDelayed splenic rupture secondary to intrasplenic aneurysms/pseudoaneurysms requires laparotomy followed by splenectomy  and onlyNTISPs present with spontaneous splenic rupture or with splenic infarction secondary to aneurysmal or pseudoaneurysmal thrombosis. The vast majority requires splenectomy . We elec


Introduction. Vulvar cyst in adolescent girls is very uncommon. Epidermoid cyst can be seen in many sites including face, trunk, and extremities but its occurrence in vulva is uncommon. This is the first case of epidermoid cyst of vulva reported in an adolescent girl. Case. A 17-year-old, adolescent girl admitted to our gynecology outpatient clinic with a complaint of painful and palpable mass in her vulva. On examination, a giant mass located in left vulva and labia majora with 11 cm in diameter was seen. The magnetic resonance imaging (MRI) showed a well-defined cystic mass without contrast enhancement. The surgery was advised to the patient and the pathologic examination of mass revealed vulvar epidermoid cyst. Discussion. Vulvar cysts generally grow slowly and the main etiologies are vulvar trauma and surgical interventions including episiotomy and female circumcision in some culture. The exact treatment is total surgical excision and pathologic examination. MRI is an important imaging modality for detection of extension to deep perineal tissue and localization of mass in vulva especially in giant ones. Conclusion. Although vulvar mass in adolescents is rare, the epidermoid cyst with benign origin should be kept in mind. The vulvar epidermoid cysts are seen rarely. These cysts usually develop as a result of invagination of superficial squamous epithelium which is an intradermal tumor locating within epidermis , 2. TherA 17-year-old adolescent sexually inactive girl admitted to our gynecology outpatient clinic with complaint of left painful vulvar mass, which had been noticed since the menarche age of 11 years and became painful for last three months. The patient had no history for genital trauma or any surgery. She noticed that the mass was gradually increased in size slowly for a period of approximately 6 years after menarche. Her medical history was unremarkable.Gynecologic examination revealed an 11 cm diameter mobile, regular contoured, and soft but painful mass in left labia majora and vulva. The mass extended to the pubic rami anteriorly, labia majora and clitoris medially, and inner thigh laterally . On suprThe patient underwent surgical operation and the cyst was easily dissected from peripheral tissue without any major bleeding sites and rupture . The hisEpidermal cyst can arise in many sites including face, trunk, and extremities and also in genital region . EpidermMany cases with reported vulvar epidermal cyst in adolescent girls were located in clitoris , 5, 7. TVarious cystic lesions in vulvar region must be considered in the differential diagnosis. These are Bartholin cyst of vulva, epidermoid cyst, Nuck canal cyst, lipoma, endometrioma, posttraumatic hematoma, and inguinal hernia. Also vulvar malignant tumor should be kept in mind but it is very rare in adolescents . A detaiThe treatment option for vulvar epidermal cyst is total surgical resection without any major complication. However, if the mass is extending to clitoris or is deep into perineal membrane to inferior hemorrhoidal branches of pudendal vessels, it has the risk of bleeding. The surgeon must be alert of that risk; the hemostatic suture should be in cases of heavy bleeding . Total sAs far as we know from literature, up to now, those vulvar epidermal cysts reported in literature were seen in adult and the adolescent cases were involved in clitoral cysts. So this is the largest reported primary vulvar epidermoid cyst arising in an adolescent girl. MRI is a valuable imaging tool for differential diagnosis from other benign cystic lesions. The complete surgical resection is appropriate treatment with good cosmetic results.


Inferior vena cava (IVC) leiomyosarcoma represents an extremely rare disease that commonly involves the segment between the inflow of the renal veins and the inflow of the hepatic veins (46% of cases). We report the case of a patient affected by an IVC leiomyosarcoma, treated with surgical resection, caval reconstruction with polytetrafluoroethylene (PTFE), and right nephrectomy, followed by external beam radiotherapy. Oncological follow-up was negative for 17 years after this combined treatment, since the patient developed a duodenocaval fistula (DCF). Inferior vena cava (IVC) leiomyosarcoma represents an extremely rare mesenchymal tumor, with approximately 300 cases reported during the last years .This neoplasm can occur in three different segments of the IVC: segments I , II , which is the most commonly affected location (46%), and III  , 3.Being a slow-growing malignancy, clinical manifestations of leiomyosarcoma typically have a delayed appearance and are commonly represented by abdominal pain (66%) , palpablHowever, even with extensive caval involvement, severe venous obstructive symptoms are not often present due to the development of extensive venous collaterals, which maintain adequate flow around the level of obstruction , 6. en bloc with the affected portion of the IVC; this should attain to the goals of complete excision of the tumor and preservation of venous return. The prosthetic substitution of the involved IVC tract is generally performed using a polytetrafluoroethylene (PTFE) prosthesis [Therapy of IVC leiomyosarcoma consists of standard wide surgical resection of tumorosthesis .Additional right nephrectomy is frequently required for cases involving IVC segment II, even if the kidney is not directly involved .The prognosis of patients affected by IVC leiomyosarcoma is not invariably poor, with 3- and 5-year disease-specific survival rates of approximately 75 and 35%, respectively. Up to 50% of patients show recurrence, typically within 30 months after resection [Duodenocaval fistula (DCF) is a rare  but lethDiagnosis of DCF is challenging and rarely obtained before laparotomy or autopsy (50%) , becauseIn the current case report, we present a patient with leiomyosarcoma, treated with IVC resection and right nephrectomy, followed by reconstruction with PTFE and external beam radiotherapy, who developed a DCF 17 years later. The patient was admitted to our emergency department for abdominal pain and small bowel obstruction, but no signs of sepsis or massive bleeding were found because the prosthesis collapsed.We aimed at reporting our experience in the management of DCF, describing clinical presentation, possible etiologies, and therapeutic implications, and focusing on the diagnostic challenges in our case.Our institutional review board approved the preparation of this case report. In August 2013, an 82-year-old woman presented to our emergency department after 1-week persistent vomiting, with a concomitant diagnosis of duodenitis and gastrectasia by esophagogastroduodenoscopy (EGDS) and contrast radiograms performed in another hospital which did not report any signs of ulcer. Moreover, no signs or symptoms of infection were reported by the clinicians.Her past medical history was positive for colic diverticulosis, cholecystectomy, bilateral hysteroannessiectomy, and excision of a retroperitoneal leiomyosarcoma of the IVC performed in 1996 along with right nephrectomy, IVC reconstruction followed by radiotherapy. Oncological follow-up was negative. She reported multiple episodes of deep venous thrombosis of the right limb due to chronic venous obstruction, treated with ticlopidine (250 mg/day). Additionally, she underwent ileal resection extended for 38 cm from the ileocecal valve, not involving the duodenum, and ileoileal anastomosis for bowel obstruction due to postsurgical adhesive bands in December 2012; however, a late effect of external beam radiotherapy could not be excluded.At our emergency department plain radiography was initially performed, revealing dilation of the first part of the duodenum and the presence of gastroduodenal air-fluid levels . Hence, Three months later, a surveillance follow-up CT study was performed on a 256-slice multidetector scanner . Images were acquired before and after  the intravenous injection of 100 mL of nonionic iodinated contrast material  at a flow rate of 3.5 mL/s, using an 18-gauge catheter positioned into an antecubital vein. This study clearly showed a fistula connecting the second part of the duodenum and the prosthetic IVC, along with an incomplete small bowel obstruction and a dilated stomach . MoreoveSeveral aspects of our observations are noteworthy. First of all, DCF represents a relatively rare clinical manifestation and only 37 cases have been described . The occDifferent factors could have contributed to the development of DCF in this case.The frequent association of duodenal ulcer with these fistulae suggests that the fistula itself may be related to postirradiation fibrosis and mucosal damage. The adherence of duodenum and IVC portions within an irradiated field, coupled with mucosal damage and fibrosis due to irradiation, might lead to ulceration and fistula formation . As descDCF represents a life-threatening condition, associated with a 40% mortality rate in 37 patients, as reported in a previous review . BecauseFinally, it should be highlighted that the occurrence of DCF represents a lethal event and prompt laparotomy is mandatory , 15. SinOur investigation reports the first case of a DCF related to a previously treated leiomyosarcoma of the IVC. This complication was properly highlighted by contrast-enhanced MDCT, which represents a conventional imaging technique extensively applied in clinical practice. Moreover, although DCF usually represents a lethal event, in our case it had a protracted and indolent natural history due to the chronic obstruction of IVC.


Mucinous cystadenocarcinoma is the second most common etiology of appendiceal mucocele. We report a relatively rare case of a giant appendiceal mucocele caused by mucinous cystadenocarcinoma, which occupied the entire abdomen of an adult woman.A 63-year-old woman presented with a chief complaint of abdominal distention. Imaging studies showed a giant cystic mass occupying her entire abdomen. Laparotomy confirmed a giant appendiceal mucocele, and the patient underwent ileocecal resection. A mucinous deposit was not found in her abdominal cavity, and the ovaries were grossly normal bilaterally. The pathological diagnosis was mucinous adenocarcinoma with a low-grade mucinous neoplasm that invaded the subserosa. Regional lymph node metastasis was not found. She has had recurrence-free survival for 5 years.The present case is the largest appendiceal cystadenocarcinoma ever reported. The optimal treatment of an appendiceal neoplasm requires further research based on consensus terminology of an appendiceal mucocele. Mucinous cystadenocarcinoma is the second most common etiology of appendiceal mucocele , 2, and A previously healthy 63-year-old woman presented with a chief complaint of abdominal distention. She denied abdominal pain, nausea, or constipation. She also did not have any relevant medical history. Her abdomen was remarkably distended but was soft and nontender. Blood test indicated slight anemia of hemoglobin  and increased levels of the carcinoembryonic antigen (CEA) and cancer antigen 19-9 (CA19-9) to 406.1 ng/mL  and 66 U/mL , respectively. The CA-125 level was normal.Contrast-enhanced computed tomography (CT) showed a well-encapsulated, unilocular cystic mass with focal mural calcification. It occupied her entire abdomen from just below the diaphragm to the pelvis, and it measured 40.2 cm in length Fig. . MagnetiLaparotomy showed the enlarged appendix, which occupied the entire abdomen. A mucinous deposit was not found in her abdominal cavity, and the ovaries were grossly normal bilaterally. Even though the cyst adhered strongly to the abdominal wall due to inflammation, ileocecal resection was performed uneventfully.The cystic mass contained about 7000 mL of serous fluid. The levels of CEA and CA19-9 in the fluid were 57,905 ng/mL and 1380 U/mL, respectively. Macroscopically, a cross-section of the mass showed a cystically dilated lumen containing mucin, and the portion protruding from the appendiceal lumen looked like a villous tumor Fig. . RegionaWe report a rare case of a giant appendiceal mucocele caused by mucinous cystadenocarcinoma, which occupied the entire abdomen of an adult woman.An appendiceal mucocele is a morphologic entity referring to the distention of the appendicular lumen due to the accumulation of mucus. It has been a long time since it was first described by Rokitansky , but theAlthough luminal dilatation is mild (up to 1 cm) in retention mucocele and hyperplasia, mucinous cystadenoma and cystadenocarcinoma often exhibit marked distention . We searA mucocele is usually diagnosed by an abdominal CT scan. The typical finding of an appendiceal mucocele is a low attenuated, well-encapsulated, thin-walled cystic mass in the right lower quadrant. Mural calcification is seen in less than 50 % of cases . MRI demHowever, the precise preoperative distinction between a cystadenoma and cystadenocarcinoma is often difficult , 18. TheThe boundary between cystadenomas and cystadenocarcinomas is ambiguous even in a pathological study. The term “mucinous tumor of low malignant potential”  or “low-The Peritoneal Surface Oncology Group International recently reported the consensus for classification and pathologic reporting . It arguThe operative strategy is also changing. It was the standard concept, and it still is in many facilities, that if the histological diagnosis is hyperplasia or cystadenoma, appendectomy is the definite treatment, but if it is a cystadenocarcinoma, resection should be combined with right colectomy , 26. HowFormerly, an appendiceal mucocele was considered an indication for open surgery, but now, many surgeons think that laparoscopic appendectomy is a reasonable choice when treating a mucocele of the appendix . HoweverWe describe the case of an adult woman with the largest mucinous cystadenocarcinoma of the appendix that occupied the entire abdomen. The optimal treatment of appendiceal neoplasm requires further research based on consensus terminology of an appendiceal mucocele.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.