Imaging studies demonstrated intra-abdominal reactionary fluid along with a heterogeneous mass localized in the falciform ligament. Exploratory laparotomy revealed a necrotic mass of the falciform ligament. No other pathology responsible for falciform ligament necrosis was found. We believe that falciform ligament necrosis should be considered a preliminary diagnosis, if any ligament abnormality, tumor, intraligament air density, or the presence of reactionary fluid surrounding the ligament is detected through abdominal imaging studies.The falciform ligament is one of the anatomical structures which attach the liver to the diaphragm and anterior abdominal wall. Primary falciform ligament is very rare. In this article, we present a case of an isolated falciform ligament necrosis, a rare primary pathology of the falciform ligament, who was admitted with acute abdomen. Case presentation: A 64-year-old female patient was admitted with the complaints of pain. Laboratory test results showed a leukocyte count of 17,000/mm The falciform ligament, which is located on the left of the midline of the abdomen, runs through the anterior wall of the abdomen and diaphragm. It is one of the anatomical structures which attaches the liver to the remnants of the umbilical veins. The length of the falciform ligament may vary individually and it contains the ligamentum teres and obliterated umbilical vein. It also contains paraumbilical veins. The falciform ligament artery originating from the liver was reported in several cases in the literature . Further3 . Serum chemistry tests and electrolyte measurements did not indicate any pathology related to acute abdomen. In addition, CA19-9, CEA, CA-125, and AFP tumor biomarkers were normal. The air under diaphragm demonstrated was by posteroanterior X-ray, one of the radiographic diagnostic tools and also abdominal ultrasonography demonstrated intra-abdominal reactionary fluid along with a heterogeneous mass localized in the falciform ligament. Abdominal computed tomography showed a mass lesion originating from the gallbladder extending to the periportal region and surrounding the falciform ligament without opaque-medium enhancement (A 64-year-old female patient was admitted with the complaints of pain in the right upper quadrant and the epigastric region. The patient's history revealed an abdominal pain for 24 hours with an increasing severity, as well as nausea and vomiting. Physical examination suggested peritoneal irritation with a systolic blood pressure of 100/70 mmHg, pulse rate of 140 bpm, and body temperature of 38.5°C. Laboratory test results showed a leukocyte count of 17,000/mmancement . Based oancement . The posAlthough the anatomical structure and variations of the falciform ligament are definitely defined, associated conditions of the falciform ligament remain to be elucidated. The most common pathologies include ligament cysts, tumors, and abnormal vascularization secondary to portal hypertension . Additio E. coli, Enterococcus, and Klebsiella pneumonia in patient's culture with history of prosthetic mesh repair [ in situ or distant cysts, abscess, and tumor was found, either. The diagnosis of primary falciform ligament necrosis was based on the absence of coagulation disorder or bacteremia.In recent years, the number of patients faced with the intraperitoneal fat tissue necrosis including the falciform ligament has been increasing parallel to the development in the field of radiology. The abdominal ultrasonography and computed tomography could provide important clues in the diagnosis of these patients. Coulier reported that the contrast-enhanced abdominal computed tomography is the gold standard for diagnosis of intraperitoneal fat necrosis as well as the follow-up of the disease . Bacterih repair , 10. No The primary falciform ligament necrosis is often diagnosed during surgery. Coulier proposed that patients diagnosed with falciform ligament necrosis could be treated medically after excluding other disorders with a detailed history , whereasIn conclusion, falciform ligament necrosis is an extremely rare cause of acute abdomen. Surgeons should consider falciform ligament necrosis as a preliminary diagnosis if any ligament abnormality, tumor, intraligament air density, or the presence of reactionary fluid surrounding the ligament was detected through abdominal imaging studies. Surgery is the only treatment of choice. Laparoscopy or laparotomy can be performed according to the preference and experience of the surgeon as well as the overall condition of the patient.


Primary lymphomas of the canine female genital tract are uncommon tumours. A 9-year-old intact female Lhasa Apso dog presenting with a closed pyometra underwent an ovariohysterectomy (OHE), and the hyperplastic uterine horn along with multiple follicular cysts on the right ovary was examined by histological analysis. Severe infiltration of medium-sized lymphocytes with strong positive immunoreactivity for CD79a and numerous anaplastic features was detected in the unilateral uterine horn, and the dog was diagnosed as having extranodal marginal zone B-cell lymphoma (MZBCL). The present case reports an extremely rare occurrence of primary lymphoma involving the uterine horn in a dog and describes histological characteristics of the tumour for definite diagnosis. Uterine tumours are rare in dogs and typically include leiomyomas, leiomyosarcomas, adenomas, adenocarcinomas, and fibromas . AlthougUterine mucosa is a type of mucosa-associated lymphoid tissue (MALT), which is a specific form of peripheral lymphoid tissue, and extranodal marginal zone B-cell lymphoma (MZBCL) can be derived from MALT within various locations, including the intestines, uterus, salivary glands, lungs, thyroid, and orbita . In humaThis patient was diagnosed with extranodal MALT-type MZBCL in the uterus by histopathological and immunohistochemical analysis. This report presents a rare case of primary uterine lymphoma in a dog and describes the diagnostic approach for adequate treatment and prognostic evaluation.A 9-year-old intact female Lhasa Apso dog presented to the animal hospital for mild lethargy and anorexia. No significant changes were detected by complete blood count (CBC) and serum chemistry. A hyperplastic uterus was identified on radiologic imaging, and a closed pyometra was suspected; thus, ovariohysterectomy (OHE) was performed. On gross appearance, the right ovary had multiple follicular cysts, and the connected uterine horn showed mild endometrial hyperplasia.a according to the manufacturer’s instructions. Rabbit monoclonal anti-CD3b (1:100) and mouse monoclonal anti-CD79ac (1:200) were used as primary antibodies. The antibody reaction was visualised using diaminobenzidine peroxidase substrate (DAB).d The sections were counterstained with Mayer’s haematoxylin, dehydrated, and mounted.The resected ovary and uterus were fixed in 10% neutral buffered formalin, embedded in paraffin, and processed routinely with haematoxylin and eosin (HE) stain. For immunophenotyping of lymphocytes, immunohistochemical analysis was performed using the Vectastain® Elite ABC-Peroxidase kitIn HE stain, the uterine horn showed massive infiltration of basophilic cells, and the disappearance of endometrial glands with moderate haemorrhage was noted. The basophilic infiltrates partially invaded the muscular layer Figure . Under hThe patient recovered after OHE, and no additional occurrence or systemic metastasis of lymphoma was found.Generally, canine lymphomas occur in 5- to 11-year-old (middle-aged) dogs, and 84% of canine lymphomas are a multicentric form by anatomical classification /centrocytic [CC]) is also common but is more likely to be disseminated and has a rare primary occurrence .Helicobacter pylori infection in human cases, and low-grade MALT-type MZBCL in the stomach is likely to respond to eradication of the H. pylori infection [Extranodal marginal zone B-cell lymphoma tends to occur in patients with a history of autoimmune disease and chronic inflammatory disorders . This chnfection .When gastric MALT-type lymphoma is localised, the patient may be treated with surgery or radiotherapy and antimicrobial therapy for pre-existing chronic inflammation; however, if the disease is disseminated and at a more advanced stage, single- or multi-agent chemotherapy may be required . There iIn the present study, we described a rare uterine tumour identified as primary extranodal MZBCL in a dog, and, to our knowledge, this unusual presentation has not been reported previously in the veterinary literature. Although future studies are required for further pathologic analysis, this report may help improve the diagnosis and clinical approach for rare uterine tumours of dogs.aVector Laboratories, Inc., Burlingame, CA, USAbAbcam, plc., Cambridge, UKcSanta Cruz Biotechnology, Inc., Dallas, Texas, USAdZymed laboratories, Inc., South San Francisco, CA, USAThe authors declare that they have no competing interests.JS evaluated the tissue sample by HE stain and immunohistochemistry and drafted the manuscript. HJ participated in tissue preparation and prepared a paraffin-embedded section. S performed the ovariohysterectomy procedure and reported clinical findings. SH supported the histopathological diagnosis and supervised the manuscript. All authors read and approved the final manuscript.


Cellular angiofibroma represents a rare mesenchymal tumor typically involving the inguinoscrotal area in middle-aged men. Although the origin of this benign tumor is unknown, it is histologically classified as an angiomyxoid tumor. Cellular angiofibroma is characterized by a diversity of pathological and imaging features. An accurate preoperative diagnosis is challenging. Magnetic resonance imaging examination of the scrotum has been reported as a valuable adjunct modality in the investigation of scrotal pathology. The technique by providing both structural and functional information is useful in the differentiation between extratesticular and intratesticular diseases and in the preoperative characterization of the histologic nature of various scrotal lesions. There are few reports in the English literature addressing the magnetic resonance imaging findings of cellular angiofibroma of the scrotum and no reports on functional magnetic resonance imaging data. Here we present the first case of a cellular angiofibroma arising from the tunica vaginalis of the testis and we discuss the value of a multiparametric magnetic resonance protocol, including diffusion-weighted imaging, magnetization transfer imaging and dynamic contrast-enhanced magnetic resonance imaging in the preoperative diagnosis of this rare neoplasm.A 47-year Greek man presented with a painless left scrotal swelling, which had gradually enlarged during the last 6 months. Magnetic resonance imaging of his scrotum displayed a left paratesticular mass, in close proximity to the tunica vaginalis, with heterogeneous high signal intensity on T2-weighted images and no areas of restricted diffusion. The tumor was hypointense on magnetization transfer images, suggestive for the presence of macromolecules. On dynamic contrast-enhanced magnetic resonance imaging the mass showed intense heterogeneous enhancement with a type II curve. Magnetic resonance imaging findings were strongly suggestive of a benign paratesticular tumor, which was confirmed on pathology following lesion excision.Magnetic resonance imaging of the scrotum by combining conventional and functional magnetic resonance data provides useful diagnostic information in the preoperative characterization of scrotal masses. A possible diagnosis of a benign paratesticular tumor based on magnetic resonance imaging features may improve patient care and decrease the number of unnecessary radical surgical explorations. CA was first described by Nucci in women . Althougin women –8.Magnetic resonance imaging (MRI) of the scrotum has been proposed as a valuable alternative imaging technique for the evaluation of scrotal pathology –15. ReceA 47-year-old Greek man presented to our hospital with a painless left scrotal swelling, which he had for 2 years and which had gradually enlarged during the last 6 months. No prior history of trauma or genitourinary tract infection was reported. A physical examination revealed a painless, firm scrotal mass, associated with hydrocele. No abnormal skin changes were observed. Laboratory tests, including serum tumor markers were unremarkable.Sonographic examination of his scrotum showed the presence of a large left extratesticular mass, of heterogeneous echotexture. Significant hydrocele was also detected ipsilaterally. Color Doppler assessment revealed rich lesion vascularity.b-values of 0 and 900 second/mm−2. MTI was performed in the axial plane using a three-dimensional gradient-echo sequence, both with and without an on-resonance binomial prepulse to saturate the broad resonance of immobile macromolecular protons. The magnetization transfer ratio (MTR) was calculated as follows: SIo–SIm/SIo × 100, where SIm and SIo refer to signal intensities with and without the saturation pulse, respectively. Coronal DCE subtraction MR images were also obtained after an intravenous injection of gadopentetate dimeglumine, with the use of a three-dimensional fast field-echo sequence. The patterns of contrast enhancement of both his normal testis and the extratesticular lesion were evaluated and time–signal intensity (TSI) curves were created.An MRI examination was performed on a 1.5-T magnet, with the use of a circular surface coil. The MR protocol included axial spin-echo T1-weighted sequences and fast spin-echo T2-weighted sequences in three orthogonal planes. Transverse diffusion-weighted (DW) images were obtained using a single shot, multi-slice, spin-echo planar sequence with His spermatic cords, epididymis, and right testis were normal. A well-demarcated left paratesticular mass Figs. , and 5 iAt left inguinal exploration a hard mass was seen in his paratesticular region, separated from his testis and enucleation of the lesion was followed. Histopathology revealed a neoplasm confined to the parietal lamina of the tunica vaginalis, of low-to-moderate cellularity with abundant myxoid stroma Fig. . CharactThe patient had an uneventful recovery and remains well, with no evidence of local recurrence, 10 months after surgery.CA or AMF-like tumor is a rare benign mesenchymal tumor, usually seen during the fifth to eighth decades of life –8. The mMost patients are asymptomatic, presenting with a slow-growing, painless scrotal mass, as it was in this case. Pathological features typically include bland spindle-shaped cells, a myxoid stroma rich in collagen fibers and numerous small to medium-sized blood vessels –8. The rAn expanded role for MRI in the evaluation of scrotal pathology has recently been established –15. ConvImaging features of CAs are correlated to their histology, depending on the amounts of spindle cells, myxoid and collagenous stroma and fat within the tumor. A few references have described the MRI findings of CA –7. All let al. concluded that in the presence of a well-circumscribed hypervascular mass containing fat in the inguinal region of a male the diagnosis of CA should be considered [The presence of intratumoral fat has been reported in 24 to 56 % of CAs . Miyajimb-value DWI with conventional MRI findings a high accuracy has been reported in differentiating between normal, benign and malignant scrotal contents [et al. reported a case of a 72-year old man with AMF-like tumor of the scrotum, without areas of restricted diffusion on high b-value DW images [In our case, a multiparametric MR protocol was used to evaluate the paratesticular mass, including DWI, MTI and DCE MRI. DWI is an evolving technique that can be used to improve tissue characterization when interpreted in combination with conventional MRI findings –11. Lesicontents –11. MaruW images . In our MTI provides a different image contrast compared to the conventional MR sequences, which depends mainly on the concentration of macromolecules . ProtonsDCE MRI provides information regarding the characteristics of microvasculature of testicular carcinomas, assessing tumor angiogenesis , 14. DCEMRI by combining conventional and functional data may provide valuable information in the pre-surgical work-up, helping in the characterization of the benign nature of paratesticular tumors. A possible diagnosis of benignity based on MRI features may obviate unnecessary radical orchiectomies. A multiparametric MR protocol, including DWI, MTI and DCE MRI may provide important additional diagnostic information in the interpretation of scrotal pathology.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Hibernomas are rare benign soft-tissue tumours arising from brown fat tissue. Although imaging characteristics are not specific certain imaging features, common locations and patient demographics may suggest hibernoma as a differential diagnosis.18 F-fluorodeoxyglucose positron emission tomography-computed tomography in the second patient. In both cases histological diagnosis was initially based on excisional and needle core biopsy, respectively. Complete surgical resection confirmed the diagnosis of hibernoma thereafter.We report on two 48-year-old male patients with hibernoma. The tumour presented with local swelling of the inguinal region in the first patient and was an incidental imaging finding in the second patient. Imaging included magnetic resonance imaging in both patients and computed tomography as well as In soft tissue tumours with fatty components hibernoma may be included into the differential diagnosis. Because of the risk of sampling errors in hibernoma-like tissue components of myxoid and well-differentiated liposarcoma, complete resection is mandatory. This article also reviews the current imaging literature of hibernomas. Hibernomas are rare benign soft-tissue tumours arising from brown fat tissue -3. The tThe aetiology of hibernomas is unknown. Molecular genetics show deletions respectively reciprocal translocation of chromosome 11 which encodes the tumour suppressor gene MEN 1 ,9. The dAt histopathology, most hibernomas present as typical variant (82%) of which three cellular subtypes are differentiated . Myxoid variants (9%), lipoma-like variants 7%), and spindle cell variants (2%) of hibernoma are less common ,7.%, and spExcept intrathoracic and retroperitoneal hibernomas, the tumours are usually well diagnosed with ultrasound showing smooth borders and hyperechoic signal. Colour duplex sonography and angiography often present a hypervascular tumour, with arteriovenous shunts in some cases. Due to the increased risk of bleeding, biopsy is not recommended in these cases. But often the diagnosis is not considered prior to biopsy in different imaging modalities, as presented in the two following cases ,10,11.18 F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET/CT) high uptake is measured in hibernomas due to the metabolic activity of brown fat. Therefore, differentiation from malignant tumours is difficult [In computed tomography (CT) and magnetic resonance imaging (MRI), hibernomas are well-circumscribed tumours, commonly located in subcutaneous tissue, skeletal muscle or intermuscular fascial planes. Common sites include thigh, peri- and interscapular region, axilla, neck, chest, abdominal cavity and retroperitoneum. The scalp, breast, scrotum and perirectal region, spine and bones are less frequently affected ,7,12. Hiifficult ,10,15,16ifficult .Hibernomas are benign tumours without a risk of malignant transformation or metastases ,18. As sA 48-year-old male patient was admitted to our hospital with a palpable soft-tissue swelling at the left upper leg extending to the groin. The tumour was slowly growing since approximately 6 months. MRI was performed  showing a 4 × 10 × 12 cm measuring mass between the gluteus medius and minimus muscles which was isointense to fat in all sequences. The tumour extended from the greater trochanter of the femur to the anterior superior iliac spine  revealed an intramuscular lipomatous neoplasia, complying with the diagnosis of the pale cell subtype of typical hibernoma  was performed to exclude gallbladder perforation. CT showed increased enhancement of the gallbladder wall and pericholecystitic infiltration of fatty tissue compatible with acalculous cholecystitis. There was no dilatation or wall thickening of the intra- or extrahepatic bile ducts. Endosonography was performed thereafter, showing a single stone in the middle part of the common bile duct, which was treated with endoscopic retrograde cholangiopancreatography.A 48-year-old male patient presented in our emergency department with upper abdominal pain, elevated liver enzymes and laboratory parameters of cholestasis. Acute cholecystitis was suspected and abdominal ultrasound was performed. Due to overlying bowel gas the gallbladder could not be examined completely. Consequently, abdominal CT  with both intravenous  and oral contrast administration (1000 ml H18 F-FDG (5 MBq/kg body weight) was performed which showed an increased accumulation of 18 F-FDG (SUVmax 12.3) within the tumour 60 minutes after injection. 18 F-FDG-PET/CT further supported the working diagnosis of a malignant tumour . Histological analysis yielded the pale cell subtype of a typical hibernoma Figure . After d18 F-FDG-PET may provide imaging findings suggestive of hibernoma. These findings include hypointensity to fatty tissue on T2-weighted images and hyperintense signal intensity on TIRM images. Large intratumoural blood vessels, heterogeneous contrast enhancement and strong FDG uptake are further imaging signs [Hibernomas are benign, well-circumscribed, slowly growing and well vascularised soft-tissue tumours containing brown fat -3,7. MRIng signs ,15,16.The differential diagnosis of lipomatous soft-tissue tumours is broad and comprises benign  and malignant (e. g. liposarcoma) lesions ,14,20. LHowever, despite the use of multimodal imaging, the radiologic diagnosis of hibernomas is difficult and differentiation from well-differentiated liposarcomas remains a challenge even after biopsy. Therefore, complete surgical resection is highly recommended. A summary table of hibernomas is shown in Table Written informed consent was obtained from both patients for publication of this Case report and any accompanying images. A copy of the written consents is available for review by the Editor of this journal.


Ameloblastomas are tumors of odontogenic epithelial origin. The term unicystic ameloblastoma is used to describe cystic lesions with clinico-radiographic features resembling an odontogenic cyst, but histologically showing the presence of ameloblastomatous epithelium lining part of the cyst cavity. A large majority of lesions are found in the mandible, and usually cause a painless swelling of the jaws. They can be radiographically subdivided into 'dentigerous' and 'non-dentigerous' types. The unicystic ameloblastoma is believed to be less aggressive than a solid/multicystic ameloblastoma, and thus has a more favorable response to enucleation and curettage. This case report presents a case of unicystic ameloblastoma with a multilocular radiographic appearance in the anterior mandible of a 45-year-old female patient, along with a literature review of the topic. Ameloblastomas are thought to represent 1% of all the cysts/tumors of the jaws and 18% of all the odontogenic neoplasms.2 The vast majority of ameloblastomas arise in the mandible, and the majority of these are found in the angle and ramus region.2Conventional ameloblastomas are usually seen at 20-50 years of age, with an average age of discovery of about 40 years, and an equal sex distribution.4Unicystic ameloblastomas represent around 10-15% of all the intraosseous ameloblastomas.5 The few that are not associated with impacted teeth are called as a ‘non-dentigerous’ variant. The ‘dentigerous’ type occurs on average 8 years earlier than the ‘non-dentigerous’ variant. The mean age for unilocular, impaction-associated unicystic ameloblastomas is 22 years, whereas the mean age for the multilocular lesion unrelated to an impacted tooth is 33 years.6 Clinically, lesions are often asymptomatic, and usually cause a painless swelling of the jaws. On radiographic examination, as implied by the term 'unicystic', the common presentation is a unilocular radiolucency; however, occasionally a multilocular appearance may also be observed.Over 90% of lesions are located in the mandible. Between 50% and 80% of cases are associated with an impacted tooth, most commonly a mandibular third molar, and are often called a ‘dentigerous’ variant of unicystic ameloblastoma.This paper reports a distinctive case of a unicystic ameloblastoma in a 45-year-old female patient. The lesion was not associated with an impacted tooth ('non-dentigerous' variant) and presented as a multilocular radiolucency in the anterior mandible crossing the midline which is rather unusual for these lesions.A 45-year-old female patient reported to our outpatient department with a chief complaint of swelling in the lower anterior region for 2 months. There was no associated pain, difficulty in opening the mouth, chewing or speech. On extraoral examination, a diffuse swelling was seen over the chin region in the midline, extending from the left angle of mouth across the midline to involve the right angle of mouth area, measuring around 5×3 cm .The overlying skin was normal with no evidence of any discharge. On palpation, the swelling was firm in consistency, tender, non-pulsatile and non-compressible, with no local rise in temperature. Intraoral examination revealed a single diffuse swelling in the mandibular labial and buccal vestibule, extending from the tooth #36 region crossing the midline up to the tooth #44 region, measuring approximately 7×3 cm, and supero-inferiorly extending from the attached gingiva to the labial and buccal vestibules .The swelling was firm in consistency with a smooth surface and tender on palpation. Expansion of the lingual cortical plate was seen in relation to teeth #31, #32, #33, #34 and #41. No tenderness or mobility of the teeth in the involved area was noted. Teeth #33 and #34 were severely carious and exhibited a painful response to electric pulp testing, which lingered for a few seconds following removal of the stimulus, thus suggesting irreversible pulpitis. No significant lymphadenopathy noted.An intraoral periapical radiograph was taken, which showed root resorption of the involved teeth in the apical one-third. A mandibular true occlusal view revealed expansion of the buccal/labial cortical plate from the tooth #36 region crossing the midline up to the tooth #44 region, with the presence of a very thin corticated boundary .Panoramic radiography showed a well-defined multilocular radiolucency with thin corticated borders extending from the left side of the mandible from the tooth #36 region, crossing the midline up to tooth #44 on the right side. The internal structure was completely radiolucent. Root resorption of teeth #31, #32, #33, #34, #35, #41, #42, #43 and #44 was noted in the apical one-third .CT scan showed an expansile cystic lesion in the mandible, with expansion and thinning of both buccal and lingual cortices Figures  and 6.Based on the clinical and radiographic features, a provisional diagnosis of an ameloblastoma was made, with a differential diagnosis of odontogenic keratocyst, residual cyst, central giant cell granuloma and odontogenic myxoma.An incisional biopsy was carried out, which showed a cystic lining of stratified squamous, non-keratinized epithelium, which was 2-3 cell layers thick and resembled reduced enamel epithelium, with a stroma of collagen fibers, fibroblasts, blood vessels, and areas of extravasated blood. Based on the clinico-radiographic features and findings of the incisional biopsy, the lesion was treated conservatively with careful enucleation and curettage. Histopathological examination of the enucleated tissue showed a cystic epithelial lining with an underlying connective tissue stroma. Areas of the cystic lining showed a basal cell layer composed of tall columnar cells displaying hyperchromatic, palisaded nuclei with reverse polarity and subnuclear vacuole formation. Stellate reticulum-like cells were seen overlying the basal cell layer. The ameloblastic changes were confined to the luminal surface of the cystic lining. The connective tissue stroma showed presence of collagen fibers, fibroblasts and blood vessels with a moderate-to-dense chronic inflammatory cell infiltrate. Thus, based on the histopathology of the enucleated tissue, a final diagnosis of a unicystic ameloblastoma [Ackerman's Group I] was made.Postoperative healing was uneventful. A follow-up radiograph taken after one year showed satisfactory healing of the enucleated area. The patient has been under regular follow-up for the last 2 years, during which no recurrence has been noted.4 The term ‘unicystic ameloblastoma’ refers to those cystic lesions that show clinical and radiographic characteristics of an odontogenic cyst but on histological examination show a typical ameloblastomatous epithelium lining part of the cyst cavity, with or without luminal and/or mural tumor proliferation.5 Lesions may originate de novo (reduced enamel epithelium associated with a developing tooth undergoes ameloblastic proliferation with subsequent cystic development), may arise from epithelial lining of a dentigerous cyst, in which case it is called as a mural  ameloblastoma, or may form from the cystic degeneration of solid ameloblastomas.7The unicystic ameloblastoma was first described in 1977 by Robinson and Martinez.2A large majority (more than 90%) of lesions are located in the mandible, with the posterior mandible being the most commonly involved site.5 On clinical examination, lesions are usually asymptomatic, and patients commonly present with a painless swelling of the jaws. The patient in our case belonged to an older age group  as compared to that described in the literature (second to third decades of life) but showed similar clinical features as reported previously. Also, our case occurred in the anterior mandible which is rather unusual, with most cases of unicystic ameloblastoma (up to 80%) thought to occur in the posterior mandible.8Unicystic ameloblastomas are usually seen in younger patients (2nd-3rd decades of life) as compared to solid/multicystic ameloblastomas, with 50% occurring in the second decade of life.9 Despite the fact that the term unicystic would imply a unilocular radiographic appearance, the lesion can rarely have a multilocular radiographic appearance. In the dentigerous variant, the unilocular to multilocular ratio is 4.3:1 and for the non-dentigerous type, this ratio is 1.1:1.10 Our case was a non-dentigerous variant and also presented as a multilocular radiolucency in the anterior mandible crossing the midline, which is rather uncommon for these lesions. The present case was also diagnosed in an older individual (45 years of age) consistent with Singh et al,6 who stated that the ‘non-dentigerous’ variant of unicystic ameloblastoma occurs in older individuals as compared to the ‘dentigerous’ variant.Although unicystic ameloblastoma commonly occurs as a pericoronal radiolucency resembling a dentigerous cyst, it may also be found in the inter-radicular, periapical, or edentulous regions.6 Ackermann3 classified this entity into the following three histological groups:Microscopically, the lesion appears as a well-defined, often large monocystic cavity with a lining, focally but rarely entirely, composed of odontogenic (ameloblastomatous) epithelium. It is often accompanied by an innocuous epithelium of varying histological appearance that may mimic the lining of a dentigerous or radicular cyst.Group I: Luminal Group II: Intraluminal/plexiform Group III: Mural Our case showed areas of cystic lining with a basal cell layer of tall columnar cells displaying hyperchromatism, palisaded nuclei with reverse polarity and an overlying layer of stellate reticulum-like cells. The ameloblastic changes were confined to the luminal surface [Ackerman's Group I]. There was absence of any nodular proliferation into the lumen or connective tissue wall.11 This was observed in the present case, where the incisional biopsy showed features that resembled a dentigerous cyst but postoperative examination of the enucleated material revealed a unicystic ameloblastoma. Thus, a definitive diagnosis of unicystic ameloblastoma can only be reached by histological examination of the entire lesion and cannot be predicted based on only clinical or radiographic grounds. Since a preoperative incisional biopsy is not representative of the entire lesion it may result in an incorrect classification.3The epithelial lining of a unicystic ameloblastoma is not always uniformly characteristic and is often lined partly by a non-specific thin epithelium that mimics the lining of a dentigerous cyst. The true nature of the lesion may only become evident when the entire specimen is available for histological examination.11 The reason for this better prognosis is that in many examples the ameloblastoma involves only the epithelial lining of the cyst or projects into its lumen.Unicystic ameloblastoma is believed to be less aggressive, and its response to enucleation or curettage is more favorable than the classic solid or multicystic ameloblastomas.In the present case, although the radiographic features (multilocular appearance with root resorption of the involved teeth) were suggestive of an ameloblastoma, the incisional biopsy was reported as a dentigerous cyst. However, after taking into account the location and clinico-radiographic features, besides the findings of the incisional biopsy, simple enucleation and curettage was chosen as the treatment of choice, due to low patient morbidity and minimal effect on the quality of life. Recurrence of these lesions may be long-delayed, and hence a long-term postoperative follow-up is essential to the proper management of these patients.The diagnosis of unicystic ameloblastoma based on clinical and radiographic features may be impossible in many cases due to its similarities with odontogenic cysts and tumors and because an incisional biopsy may not be able to reflect the true nature of the lesion. In the present case, we arrived at a final diagnosis of unicystic ameloblastoma only after histopathological examination of the enucleated material. Therefore, careful postoperative histopathological examination is mandatory for all the lesions, which together with a proper follow-up can play an important role in minimizing recurrences.Dr. Anita Spadigam, Professor & Head, and Dr. Anita Dhupar, Asst. Professor, Department of Oral and Maxillofacial Pathology, Goa Dental College & Hospital, Bambolim, Goa–INDIA, for providing the histopathological analysis, report and photographs.


Gallbladder duplication in association with other GIT anomalies is a rare entity. We report two neonates; one with duodenal atresia and the other newborn with pyloric atresia, ileal atresia and colonic atresia, both were associated with gallbladder duplication which has not been reported earlier. Gallbladder duplication is a rare anomaly occurring in about 1/4000 births. [1] Only one case of gallbladder duplication with associated fetal anomaly has been previously described in the English literature till date; the neonate had gallbladder duplication associated with type 3 duodenal atresia. [2] In our study, one neonate presented with duodenal atresia associated with gallbladder duplication; while the other newborn had gallbladder duplication associated with pyloric atresia, ileal atresia and colonic atresia. We herein, propose that there exists a common etiology for these associations.Case 1: A 12-day-old, preterm male neonate, weighing 1.9 kg, 1st in birth order, born to non-consanguineous parents, presented to paediatric department of our institute with non- bilious vomiting and decreased oral intake. There was no delayed passage of meconium but patient did not pass stool for last 48 hours. Antenatal ultrasounds were not done. On examination, child was hemodynamically stable, anicteric, mildly dehydrated, pulse rate-132/min and respiratory rate-56/min. Abdominal signs included mild epigastric distension, soft on palpation and absent bowel sounds; nasogastric aspirate was non-bilious. Laboratory values revealed an Hb of 19.1gm% and total leukocyte count of 14,300/mm3. Liver function tests, renal functions and serum electrolytes were normal. C-reactive protein was raised, and treatment started on the lines of necrotizing enterocolitis. Abdominal ultrasonography (USG) was not conclusive. Paediatric surgical opinion was sought and upper gastro-intestinal contrast study was performed which suggested duodenal atresia . The child was taken up for laparotomy. Classic type 3 duodenal atresia was noted.The atresia was pre-ampullary. This was associated with a double gallbladder with a common neck as shown in Fig.1. No other anomalies were detected. Kimura’s diamond shaped duodeno-duodenostomy was done. Since there was no problem in biliary drainage, cholecystectomy was not considered. The post operative course was uneventful. Child was started oral feeds on post operative day 7 and discharged on post operative day 10.Case 2:A 2-day-old, preterm male neonate, weighing 1.6 kg, born by vaginal delivery was presented to our department with copious, non-bilious vomiting and epigastric fullness since birth. There was history of polyhydramnios on antenatal ultrasonography. On examination, neonate was hemodynamically stable, pulse rate-152/min and respiratory rate-50/min. The abdomen was soft and non-distended. Laboratory investigations were normal. An abdominal X-ray showed a single gas bubble suggestive of distended stomach with no air distally in intestine; contrast study of abdomen revealed non-passage of contrast beyond stomach. A presumptive diagnosis of pyloric atresia was made taking into consideration the history of antenatal polyhydramnios and X-ray findings. Laparotomy revealed distal ileal atresia associated with a double gallbladder with a common neck and pyloric membrane (Type 1 pyloric atresia) having a small central hole . The patency of the intestines distal to ileal atresia was checked by injecting saline through a no. 6 infant feeding tube which revealed membrane/web in transverse colon (colonic atresia). Excision of the pyloric membrane with Heineke-Mikulicz pyloroplasty, excision of colonic web and ileostomy with distal mucus fistula was performed. Cholecystectomy was not contemplated in view of major surgical procedure. Postoperatively patient had continued downhill course, developed sepsis and sclerema and died on 3rdpost operative day.The first reported human case of gallbladder duplication was noted in a sacrificial victim of Emperor Augustus in 31 BC. [3] Boyden’s classified gallbladder duplication into:(1) vesica fellea divisa ; (2) vesica fellea duplex , (a) Y-shaped type (the two cystic ducts uniting before entering the common bile duct) and (b) H-shaped type . The exact pathogenesis of this condition is unclear and various theories proposed include.  incomplete re-vacuolization theory, resulting in a persistent longitudinal septum that divides the gall bladder lengthwise; and occurrence of two separate cystic buds.Only one case of gallbladder duplication associated with duodenal atresia has been described previously along with the present case. Also we report the index case of gallbladder duplication associated with pyloric atresia along with ileal atresia and colonic atresia which qualifies for Hereditary multiple intestinal atresias of the gastrointestinal tract.[5] Boyden et al. described the second part of duodenum as the “embryological traffic jam”.[6] Epithelial proliferation followed by occlusion (5thto 6th week) and failure of recanalization and re-vacuolization is the proposed embryogenesis of duodenal atresia; with second part of duodenum recanalizes at the end. [7] Boyden also suggested the relation between duodenal atresia and associated biliary ductal anomaly.[6] We also propose that there exists a common etiology  for association of duodenal atresia with gallbladder duplication.Ultrasonographic appearance of gallbladder duplication may be mistaken for other common diagnoses like choledochal cyst, gall bladder diverticulum, Phrygian cap, extrinsic fibrous bands across the gallbladder, and a folded gallbladder of Horattas. [8]However in our case ultrasonography did not pick the anomaly and final diagnosis was made intraoperatively.Though the literature on gall bladder duplications and previous report suggest that cholecystectomy could have been contemplated to prevent future complications, but we disagree with this view, as studies in paediatric patients are lacking and extrapolating the adult data would not be justified. [2]Source of Support: NilConflict of Interest: None


Neurilemmoma commonly occurs from the fourth to sixth decades of life with an incidence of 3 to 10 per 100,000 people, and is rare in adolescence. This case report describes the clinical and radiographic features of 2 rare cases with intraspinal neurilemmoma of the cervical and thoracic spine.A 29-year-old man who experienced middle back pain with prominent right lower limb weakness, and an 11-year-old boy who suffered from sudden onset neck pain with left arm weakness and hand clawing for 2 weeks before admission to our department were included in this case report.Magnetic resonance imaging of both patients revealed an intraspinal mass causing spinal cord compression at the cervical and thoracic spine. The patients subsequently received urgent posterior spinal cord decompression and tumor resection surgery. The histopathology reports revealed neurilemmoma. The 2 patients recovered and resumed their normal lives within 1 year.Intraspinal neurilemmoma is rare but should be considered in the differential diagnosis of spinal cord compression. Advances in imaging techniques and surgical procedures have yielded substantially enhanced clinical outcomes in intraspinal neoplasm cases. Delicate preoperative study and surgical skill with rehabilitation and postoperative observation are critical. The informed consents of the 2 patients were included in the data for the ethics committee review.A 29-year-old man, who is a telecommunications engineer, experienced back soreness and muscle cramping in the abdomen for 8 months. Progressive weakness of both lower limbs with an unstable gait was noted for 1 month before surgery. Numbness in the bilateral lower limbs and lower abdomen (below the umbilicus) occurred simultaneously. Positive myelopathy with prominent right lower limb weakness was noted at the outpatient department (OPD). The preoperative Nurick score was 3 and the visual analog scale (VAS) for upper back pain was 7. T2-weighted magnetic resonance imaging (MRI) of the entire spine revealed a 5.1 × 1.4 × 1.5 cm irregular mass from the vertebral body with a compressed spinal cord at the T10–T12 level. Irregular enhancement was observed in T1-weighted images after gadolinium administration  accompanied by cystic, myxoid, and hemorrhagic degeneration (Antoni type B tissue) Figure A. The maIn Case 2, the tumor tissue was gray and grossly elastic. Microscopically, it exhibited a well-defined spindle cell tumor with moderate-to-high cellularity (Antoni type A tissue) Figure B. Focal 5 An osseous schwannoma affects the bone through 3 possible mechanisms: tumors arising within the nerve canal and forming a dumbbell-shaped configuration producing an enlarged canal, an extraosseous tumor resulting in bone erosion, or a tumor arising centrally within the bone.6 Sherman7 reported the presence of myelinated fibers within the marrow of vertebral bodies in 2 adults. Pederson et al8 noted nerve filaments of the recurrent branch of each spinal nerve root with the accompanying blood vessels entering the vertebral body. The most common site of occurrence is the mandible because of the long course of nerves within this bone.9 Reports of axial skeleton sites  affected by neurilemmoma are rare.12Neurilemmomas are benign neoplasms that account for <0.2% of primary bone tumors.13 Gadolinium-contrast MRI images are generally suggested to help differentiate the neurilemmoma from fluid-filled cysts. Neurilemmoma is typically round or oval with a bright, heterogeneous signal on T2-weighted images, and a moderately bright signal on T1-weighted images. Technical advances in imaging techniques such as MRI and surgical procedures have substantially enhanced clinical results of intradural spinal tumor cases. For our 2 cases, we confirmed diagnosis of myelopathy on the basis of positive long tract sign and degrees of neurologic deficit. We arranged MRI with and without contrast to ensure the margin of tumor and the range of spinal cord compression, and then determined the surgical resection plans.Spinal neurilemmoma often affects the spinal cord and causes radiculopathy or myelopathy symptoms. Detailed recording and thorough physical examination should be performed. Neglected and untreated myelopathy could cause irreversible neurologic deficit and disability. Computed tomography and MRI are the primary diagnostic tools for evaluating neurilemmomas.14 Decompression and surgical resection of tumor are the primary therapy, with or without further fusion. For T-spine neurilemmoma of Case 1, we performed tumor resection with posterior instrumented fusion because the tumor was located at the physiologic kyphotic curve of the T spine, and postoperative progressive kyphosis could happen without instrumentation. For C-spine neurilemmoma of Case 2, we performed posterior decompression with tumor resection without instrumentation because of 2 reasons: the tumor was located at the physiologic lordotic curve and only involved the left side, and hence the right side facet joints could be preserved to maintain structure stability; and unnecessary additional fusion should be avoided to preserve the range of neck motion. The nerve is typically spread over the lesion, which is excised marginally to spare the nerve fibers. Interlesional resection is reasonable when complete resection might cause permanent loss of neurologic function. Most patients undergo resection surgery with complete relief of symptoms and recovery of neurologic functions. A low incidence of recurrence exists following complete resection, and malignant change is extremely rare in isolated lesions.15 After surgery, the removed tissues were pathologically examined and neurilemmoma was diagnosed. Both of our patients were satisfied with the functional recovery and resumed their normal lives well without residue neurologic deficit or local pain at regular annual follow-up.As with most benign neoplasms, neurilemmomas respond favorably to local resection surgery.Spinal neurilemmoma that causes neurologic deficit is rarely reported. The recurrence of neurilemmoma is rare after careful surgical resection. In this article, 2 cases of spinal neurilemmoma with favorable middle-term follow-up functional outcome are reported. By careful physical examination, the level of neurocompression can be determined. MRI and surgical techniques enable resection. By pathologic examination, neurilemmoma can be diagnosed.


Cotyledonoid dissecting leiomyoma is a rare variant of uterine smooth muscle tumor with an unusual growth pattern that shows intramural dissection within uterine myometrium and often a placenta-like appearance in its extrauterine components.We present a unique case of cotyledonoid dissecting leiomyoma with adenomyosis. A 40-year-old Japanese female presented with prolonged menorrhagia and severe anemia. She had a pelvic mass followed-up for 6 years with a diagnosis of leiomyoma. However, increase in tumor size and cystic changes with hemorrhage were found by magnetic resonance imaging, and total abdominal hysterectomy with bilateral salpingectomy was performed. Macroscopically, the placenta-like exophytic mass protruding from the posterior uterine wall was composed of multiple nodules containing numerous hemorrhagic cysts. The mass showed continuity as a white multinodular dissecting mass infiltrating the posterolateral myometrium. Microscopically, both extra–and intrauterine portions of the mass were composed of nodules that contained swirled neoplastic smooth muscle cells with marked hyalinized degeneration, as observed in cotyledonoid dissecting leiomyomas of conventional type. In addition, numerous non–neoplastic glands of endometrial type surrounded by abundant endometrium–like stromal cells and non–neoplastic smooth muscle cells were found in the tumor, suggesting that it involved a part of concomitant adenomyosis originating from the nontumoral myometrium.Thus far, over 30 cases of cotyledonoid dissecting leiomyoma have been reported, none of which have described the presence of adenomyosis within the tumor. The present case suggested that cotyledonoid dissecting leiomyoma might have a unique clinical presentation involving concomitant uterine adenomyosis. It is critical for pathologists, gynecologists, and radiologists to be cognizant of cotyledonoid dissecting leiomyoma variants for timely and appropriate diagnosis and treatment. Uterine leiomyomas are the most common type of uterine tumor , with itA 40-year-old Japanese female with gravidity 2 and parity 2 presented with prolonged menorrhagia and severe anemia. The patient had a history of cervical carcinoma in situ and underwent cervical conization 10 years ago. During postoperative follow-up, carcinoma did not recur, but menorrhagia and anemia persisted. Six years before admission, a uterine leiomyoma 3.5 cm in diameter was confirmed by transvaginal ultrasound examination; the tumor increased in size to 5 cm in diameter during evaluation 2 years before admission. During the last year, an extrauterine mass 5 cm in diameter protruding from the posterior uterine wall was discovered. For further assessment and treatment, the patient was admitted to the gynecology department at our hospital. Magnetic resonance imaging showed a large pelvic mass measuring 10 × 9 × 7 cm Fig.  containiMacroscopically, the placenta-like extrauterine mass measuring 10 × 9 × 9 cm was protruding from the posterior uterine wall Fig. . This maHistologically, both extra–and intrauterine components of the mass consisted of numerous ill–defined nodules, with nodules of the intrauterine component dissecting the myometrium Fig. . All nodIn the present case, the clinical manifestation, gross appearance, and histological findings were in good agreement with typical cotyledonoid dissecting leiomyoma. Leiomyomas of the uterus are the most common uterine tumor and usually affect women in their fourth and fifth decades . Most utInterestingly, several reports alluded to cotyledonoid dissecting leiomyomas with non-neoplastic cystic lesions Table . ConventWe suppose the endometrial component in this tumor may be a part of adenomyosis, not endometriosis. It is because localization of the endometrial component is not superficial and endometriosis is not found in any part of the pelvic cavity. However, adenomyosis is distributed both in the tumor and tumor-free myometrium Fig. . FurtherOn the other hand, adenomyoma should be an important differential diagnosis in the extrauterine part of the tumor. Adenomyoma is one of the mixed epithelial and mesenchymal tumors of the uterine corpus and is described as a well-circumscribed tumor composed of endometrial glands and endometrial-type stroma surrounded by abundant smooth muscle component . We cannThe mechanism of tumor development in this patient is interesting. In this case, leiomyoma was first found in the uterine wall by transvaginal ultrasonography and was later noted to extend into the pelvic cavity. It is possible that during tumor growth from the myometrium, endometrial glands and stromal cells might have been captured by the intramural component of tumor during its dissection through the surrounding adenomyosis, to be located together with the extrauterine component. In this case, glandular components and stromal cells were also recognized in the intramural component, supporting this possibility. The cystic expansion of endometrial glands outside the uterus might be correlated with loose connective tissue with congestion and hydropic change.The cotyledonoid dissecting leiomyoma in our case included a large number of non-neoplastic cystic lesions compared with previously reported three cases. This case indicated that cotyledonoid dissecting leiomyoma could occur with concomitant adenomyosis, depending on the pattern of tumor growth. These four cases, including ours, suggested that cotyledonoid dissecting leiomyoma might have a unique presentation with benign non-neoplastic cystic lesions, such as endometriosis, endosalpingiosis, adenoleiomyomatous component, and adenomyosis.The case presented herein is the first documented cotyledonoid dissecting leiomyoma with diffuse adenomyosis and reveals a unique variant of cotyledonoid dissecting leiomyoma involving benign cystic lesions. Accumulation of similar case reports is expected and important. While cotyledonoid dissecting leiomyoma has alarming gross appearance with hemorrhagic cysts of adenomyosis or other benign cystic lesions within the tumor mass, it has a completely benign clinical course. Gynecologists, radiologists, and pathologists should be cognizant of the variance and nature of cotyledonoid dissecting leiomyomas to avoid misdiagnosis and overtreatment.Bcl-2, B-cell lymphoma 2; CD10, Cluster of differentiation 10


Lipoma is a relatively frequent, benign soft-tissue tumor rarely located in the hand. A lipoma of the hand causing a carpal tunnel syndrome by compression of the median nerve is exceptional. We report the case of a 70-year-old female presenting with a carpal tunnel syndrome. A compression of the median nerve by a lipoma was discovered during surgery. Transverse carpal ligament release with lipoma excision and neurolysis of the median nerve were performed. Histopathological study of the resected mass was consistent with a lipoma. Two-month postoperatively, the patient recovered full hand function with entire disappearance of acroparesthesia. Carpal tunnel syndrome caused by space occupying lesions is rare. Diagnosis is difficult, usually based on the clinical study, electrophysiology and magnetic resonance imaging (MRI). Transverse carpal ligament release and excision of lipoma provides excellent functional recovery. Soft tissue lipoma is the most common benign tumor found in limbs, although the occurrence of lipoma in the hand remains rare, between 1 and 3.8% of benign tumors in the hand [°, flexion 75°). There was a decrease in sensitivity in the territory of the median nerve as compared with the contralateral hand without evidence of motricity impairment. The Tinel′s sign was positive at the wrist. Electophysiological study confirmed the diagnosis of CTS. Standard X-rays did not found any abnormalities. Surgery was performed under locoregional anesthesia with axillary block and with tourniquet controlled hemostasis maintained during the procedure. The patient was arranged in a dorsal decubitus position. A palmar conventional approach of the carpal tunnel was chosen. Transverse carpal ligament was incised. Intra-operatively, a lipomatous mass occupying the carpal tunnel space was discovered. The median nerve was carefully identified, It was flattened, suffering and compressed by the mass (A 70-year-old left-handed woman, without any significant pathological history presented with numbness and tingling sensation in the left hand affecting thumb, index and middle finger, with a decreased grip strength, appeared 6 months ago. Interrogation revealed a progressive evolution of nocturnal acroparesthesia over several months. Physical examination did not reveal any soft tissue mass on palpation, with a normal range of motion of the wrist (extension 70the mass . The tumthe mass  and neurLipomas are tumors developed from mature adipocyte cells, usually encapsulated and sometimes infiltrating , they usMRI is a useful tool for the diagnosis of soft tissue tumors, because of its high sensitivity, it specifies the nature of the lesion. It also allows a preoperative planning by studying the dimensions of the tumor, its local extension and its relationship with the neuro-vascular elements. Usually, lipomas appear as an homogeneous mass, with a sharp border and spontaneous T1 and T2 hyper signals with reduction of signal on fat suppressed sequences. In some cases, the image contains fibrous septa or calcifications. After gadolinium injection, the fibrous septa signal moderately raises, but fat keeps the same signal . In a seLipoma is uncommon in hand and wrist, even rarer as a cause of secondary CTS, however it should be suspected. A thorough clinical examination, Functional exploration with electromyography, morphological study with MRI are necessary. Conventional open approach is recommended with contraindication for endoscopic procedures. Complete and successful surgical resection provides excellent functional recovery. Preoperative assessment and patient information are necessary for proper management. Histology after complete removal of the tumor remains necessary to confirm the diagnosis and eliminate malignant tumor, in particular a liposarcoma.


Ewing’s sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing’s sarcoma is very rare. Here we report a case of Ewing’s sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery. An 8-year-old girl presented with progressive pain and palpable lump in right flank for the last one month. Ultrasonography (USG) showed right retroperitoneal tumor, suprarenal in location. Contrast enhanced computed tomography (CECT) demonstrated 12.7 cm x 11.8 cm large variegated mass in right adrenal gland with retroperitoneal lymphadenopathy . A 24-hour urinary catecholamines, plasma metanephrine, aldosterone, and plasma renin levels were within normal range. CT-guided core biopsy revealed malignant small round cell tumor. Bone-marrow aspiration was unremarkable. Tumor was large and appeared unresectable thus neoadjuvant chemotherapy was planned and given for seven cycles using vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide/etoposide with granulocyte colony stimulating factor (GCSF). Tumor regressed to one-fourth of its original size and lymph nodes appeared insignificant size . Patient also improved symptomatically. At operation the tumor was found densely adherent to surrounding tissues especially the upper pole of right kidney and inferior vena cava . The tumor was ultimately resected. Grossly the specimen was 8 cm x 6 cm with tan-yellow cut surface and areas of hemorrhage and necrosis. Histopathology showed adrenal medulla and cortex completely replaced by a small round blue cell tumor . Immunohistochemical (IHC) studies showed that tumor cells were positive for CD99 (membranous), synaptophysin and chromogranin . Postoperatively the patient was lost to follow up and admitted after five months with metastasis in lungs and liver and died within few days.Ewing’s sarcoma/PNET of the adrenal gland is extremely rare.[1-3] Usually the patients present with flank pain and rapidly growing mass. USG should be done as an initial screening tool but CECT is essential to find out the relation with the surrounding structures. CECT imaging helps in distinguishing between benign and malignant adrenal tumors but exact nature.[4] Hormonal studies should be done to differentiate it from the functional adrenal tumors. It is difficult to make a definitive diagnosis of ES/PNET even on histopathology without IHC.[5] Our patient had very large tumor and considered non-resectable, so needle biopsy was done to find out the nature of the tumor. Final diagnosis was made after IHC. CD 99 is a highly sensitive marker for PNET/ES. Patients of ES/PNET show reciprocal translocation of t  involving the EWSR1 gene on chromosome 22 and the FLI1 gene on chromosome 11 in more than 90% of cases but this is not routinely assessed.[2] As per available literature these tumors are very rapidly growing with aggressive nature. As this is a rare tumor, there is no definite guideline for management but surgical resection is considered to be the mainstay for local disease control.[5-7] This tumor is considered as radio and chemo sensitive so adjuvant chemotherapy and radiotherapy are advocated for better disease control but there is no consensus on standard chemo-radiation regimen.[8] We initially tried chemotherapy which worked well in our patient. The outcome seems good in case of complete excision but the prognosis was poor in our case. Proper postoperative surveillance with imaging modalities and adjuvant chemotherapy might alter the outcome in index case.Source of Support: NilConflict of Interest: None declared


Desmoid-type fibromatosis is a rare mesenchymal neoplasm with local aggressiveness. The incidence of desmoid-type fibromatosis is 2–5/million/year with intra-abdominal fibromatosis, such as that which is reported in this clinical case, occurring only in 12–18% of cases. After having analyzed the pathogenetic hypotheses of desmoid-type fibromatosis, the authors point out that the diagnosis of this disease, especially in the intra-abdominal form, is often late, specifically when highly demolitive interventions are needed or when the limits of radical surgery have been exceeded. In the clinical case reported, the tumor was infiltrating both ileus and sigma. The authors consider the differential diagnosis of desmoid-type fibromatosis, especially with GISTs, with regard to both the radiological preoperative diagnostic and histological studies on the surgical specimen. Radical surgical excision is not always, for this disease, a sign of healing; in fact, even when the resection margins are negative, the incidence of recurrence is between 13 and 68%. The average time of recurrence is between 15 and 24 months; in this case report, the patient, who has not been subjected to complementary therapies, is tumor-free for over 30 months since surgery; his prognosis may be satisfactory if we consider the negativity of resection margins, which in any case remains the most important prognostic factor.  Desmoid-type fibromatosis , also knAccording to the anatomical site, desmoid-type fibromatosis can be divided into (i) extra-abdominal fibromatosis 60%); (ii) fibromatosis of the abdominal wall (25%); (iii) intra-abdominal fibromatosis 8–15%). The latter, usually arising in the mesentery, in the retroperitoneum or in the omentum, is the most biologically aggressive type owing to its capability of infiltration of both pelvic and abdominal organs 5%. The l, 4, 6, 70%; (ii) This report deals with a rare case of sporadic mesenteric desmoid-type fibromatosis, emphasizing clinicopathological features and differential diagnostic problems. Therapeutic strategies are also discussed on the basis of the results available in the literature.A 47-year-old man was referred to our colorectal surgical division in 2011. He presented a 4-month history of intermittent pain at his left flank and abdominal cramps at his left iliac region. In the couple of days prior to being admitted, he had also been suffering from a subocclusive symptomatology. Physical examination revealed the presence of a 10 cm mass in the left abdominal region with a smooth surface and mobility (from the hypogastrium to the left hypochondrium). His past medical history was unremarkable.The laboratory results revealed no abnormalities except for a mild microcytic anemia. Tumor markers, such as CEA, Ca 19.9, Ca 125, and AFP, were within normal limits.Abdominal computed tomography (CT)  showed aColonoscopy was not performed. en bloc with the adjacent infiltrated colon and a tract of small intestine (20 cm) was performed. A side-to-side ileoileal anastomosis and an end-to-end colorectal anastomosis were subsequently performed.The patient, after having signed his informed consent, was submitted to laparotomy which revealed, at the base of the mesentery of the fourth to last intestinal loop, a well-circumscribed mass, firm in consistency, measuring 15 cm at its greatest diameter . It was The postoperative course of the patient has been uneventful, and the patient was discharged healthy on the 9th postoperative day.We did not suggest any adjuvant therapies to the patient but only recommended a follow-up.The patient is doing well and is without any recurrence 2 years after surgery.Pathological Findings. Tumor mass, measuring 15 × 10 × 6.5 cm, exhibited well-circumscribed borders and was firm in consistency. The cut section revealed a glistening whitish surface with fascicular appearance and extensive myxoid changes. Surgical specimen was submitted for histological examination in neutral-buffered 10% formalin, dehydrated using standard techniques, and embedded in paraffin; 5 μm sections were cut and stained with hematoxylin and eosin (H&E), alcian blue at pH 2.5, and periodic acid-Schiff (PAS). Immunohistochemical studies were performed with the labeled streptavidin-biotin peroxidase detection system using the Ventana automated immunostainer . The antibodies tested were vimentin (dilution 1 : 100); α-SMA (dilution 1 : 200); desmin (dilution 1 : 100); myogenin (dilution 1 : 100); S-100 protein (dilution 1 : 500); CD99 (dilution 1 : 100); CD34 (dilution 1 : 50); B-cell lymphoma 2 (Bcl-2) protein (dilution 1 : 100); CD10 (dilution 1 : 200); CD117 (dilution 1 : 400); cytokeratins ; epithelial membrane antigen (EMA) (dilution 1 : 100); anti-human melanosome (HMB45) (dilution 1 : 300), all from Dako, Glostrup, Denmark. Appropriate positive and negative controls were included.Histologically, the typical morphological features of desmoid-type fibromatosis were identified: proliferation of bland-looking spindle-shaped cells arranged in long sweeping fascicles and set in a finely collagenous stroma Figures . Interesα-smooth muscle actin. Nuclear immunoreactivity for β-catenin was observed in 70–80% of neoplastic cells  for ic cells . No immuThe incidence of desmoid-type fibromatoses is 2–5/million/year. They represent about 0.03% of all tumors and only 3% of all soft tissue tumors. 12–18% of the desmoid-type fibromatoses are intra-abdominal; 80% of them are located in the mesentery of the small bowel, but some may originate from the ileocolic mesentery. Only rarely desmoid-type fibromatosis can occur in the pancreas, gastroesophageal junction, diaphragm, or the appendix . KreuzbeIts pathogenesis is not completely understood, even if some cases have been associated with antecedent abdominal trauma, including previous surgery 25%) [5% 2, 6,,6, 9, 11Apart from sporadic cases, desmoid-type fibromatosis may arise as the result of genetic disorders such as familial adenomatous polyposis (FAP) or Gardner syndrome (2–32% of cases) , 12, 13.Mutations of the APC gene on the long arm of chromosome 5 have been documented in inherited cases, namely, both FAP and familial non-FAP tumors . InteresAs far as clinical signs are concerned, most patients are asymptomatic over time due to the slow growth of tumor mass, but clinical presentation is quite variable depending on tumor site and size. In fact, the desmoid-type fibromatosis, due to mechanical phenomena, may be responsible for intestinal obstruction or, more rarely, compression of the ureter or splenic vein , 17. In An abdominal ultrasound can often be used at a first level of investigation since it, often occasionally, raises the suspicion of abdominal mass, but CT and MRI, also used in a complementary manner, are the type of imaging which the study of desmoid-type fibromatoses mainly relies on.The purpose of the TC is both staging the tumor  and, mainly, evaluating the possible infiltration with organ or adjacent vascular structure in order to assist in planning the best therapeutic strategy , 13. ItsAlthough MRI may be an alternative diagnostic tool when patients are allergic to an iodinated contrast agent, it is a method of choice, especially with regard to extra-abdominal desmoid-type fibromatosis since it permits a high tissue differentiation. It is indicated for intra-abdominal desmoid-type fibromatosis especially in the case of recurrence , 13.In our case, an abdominal CT scan was crucial in identifying a mesenteric tumor mass involving a tract of the small intestine and sigmoid colon.Instead, we deemed it not possible to perform a colonoscopy (i) due to the urgency of the disease (bowel obstruction), (ii) due to the fact that, in our estimation, it would not have provided us with new information, and (iii) due to the difficulty of performing on the patient the bowel preparation in order to evaluate the colon above the stenosis. Even in the literature, however, in similar situations, colonoscopy is neither mentioned , 12, 18 We postulated the differential diagnosis between a desmoid-type fibromatosis and GIST while the possibility of a primary intestinal lymphoma  or sarcoma (extreme mobility of the tumor itself) seemed less likely.In our case, we did not perform preoperative biopsy for frozen section diagnosis since the surgical treatment of the lesion could not have been postponed due to symptoms of intestinal obstruction determined by the desmoid-type fibromatosis. Several cases have been reported in the literature where preoperative biopsies were not performed mainly on account of the need for emergency treatment. Few authors report a preoperative CT-guided biopsy or an endoscopic ultrasound-guided biopsy , 7 but, α-smooth muscle actin expression and nuclear β-catenin expression, which are consistent with the diagnosis of desmoid-type fibromatosis [Even in our case, the final diagnosis of desmoid-type fibromatosis was obtained by a histological examination. Interestingly, the tumor showed such extensive myxoid stromal changes that it posed differential diagnostic problems with other several benign and malignant myxoid lesions. However, morphological and immunohistochemical findings were consistent with a fibroblastic/myofibroblastic tumor that fit within the spectrum of intra-abdominal desmoid-type fibromatosis, therefore, our case represents an uncommon myxoid variant. The following morphological and immunohistochemical features, typically described in most cases of desmoid-type fibromatosis, supported our diagnosis: (i) identification of a minority component with morphological features typical of conventional desmoid-type fibromatosis including the coexistence of the three different morphological phases, namely, proliferative, involutional, and residual phases , 21;  [Differential diagnosis of mesenteric desmoid-type fibromatosis mainly includes GIST, leiomyoma, leiomyosarcoma, solitary fibrous tumor, and neurofibroma. As in our case, all these tumors may variably exhibit myxoid stromal changes. GIST differs from desmoid-type fibromatosis in that it is stained for CD34 and CD117 stains, not usually expressed in desmoid-type fibromatosis. Leiomyoma and leiomyosarcoma are tumors which express smooth muscle cell markers, including desmin, esentery . This tuesentery . Althougesentery . Unlike  desmin) .en bloc.” Histological examination revealed tumor infiltration of the subserosa and smooth muscle wall of both the small intestine and colonic bowel, while the submucosa and mucosa were tumor-free.Even though we reported herein cases of spontaneous regression , 12, theIn our case, we did not consider it necessary to have the patient undergo complementary therapies as the tumor was a primary desmoid-type fibromatosis, surgery was R0 (complete resection) and the margins of resection were free. In the literature, Walczak and Rose  and de BWhen intra-abdominal desmoid-type fibromatosis may involve celiac and/or mesenteric vascular structures, demolitive surgical resections are needed, including bowel transplantation . In otheThe poor results obtained by surgery on the treatment of relapse, also because of the hyperplasiogenous stimulus that such treatment determines, have made this approach a secondary choice or at least one which follows complementary treatments such as radiation therapy, which seems to achieve a control of tumor growth in 40 to 80% of cases , 29.The average time of recurrence is between 15 and 24 months, although relapses may occur within the first 5 years . Our pat


Subtalar coalition is an uncommon condition that usuallymanifests in early adolescence(1). Frequently, this conditionis missed. Delayed diagnosis may result in osteoarthritisrequiring triple arthrodesis. Here, we report two patients withsubtalar coalition. The first patient is a 12 year old boy whopresented with right ankle pain for one year and was treatedwith excision of the coalition and bone wax insertion at theexcision site. We followed up the patient for two years andthe result was excellent with full range of movement of hisright ankle and subtalar joint attained within two months. Hereturned to athletic activity by six months and wasdischarged with no complications after two years. Thesecond patient is a 15 year old girl who presented withbilateral ankle pain and swelling for three years and wastreated with excision of the coalition and subtalarinterpositional arthroplasty bilaterally. She defaulted followup after seven months as she was very satisfied with theresult. We wish to highlight this condition which may bemisdiagnosed as flexible flat foot or ankle sprain.Subtalar coalition, excision of coalition, bone wax insertion, subtalar interpositional arthroplasty Talo-calcaneal coalition is an abnormal bridge between thetalus and calcaneum, causing hindfoot pain, bony swellingand restriction of subtalar movement. The abnormalconnection can be bony, cartilaginous or fibrous in naturewith reported incidence ranging from 1 to 12%A 12 year old Siamese boy who was active in sportspresented to our clinic with the complaint of pain andswelling over the medial aspect of his right ankle for a year.The swelling gradually increased in size and the painworsened during walking and running. The pain wasdescribed as throbbing in nature and located at the posterioraspect of the right ankle. Patient also claimed that he haddifficulty in moving the ankle sideways. He had no previoushistory of trauma to his right ankle.Physical examination revealed a 3 x 4 cm swelling below theright medial malleolus, tender on palpation, bony hard inconsistency, surface, non mobile and the skin overlying itwas normal. There waRadiographs revealed a subtle C sign. He was tWe proceeded with the medial approach, just below themedial malleolus. Intra-operatively, we noted the right talocalcanealjoint space was immobile. The coalition wasresected and bone wax was inserted at the site of resection toprevent further bone formation. He was discharged on postopday 3 and allowed weight bearing as tolerated.We followed up the patient by using the AOFAS scoringscale and the result was excellent. Pre-operatively, it was 76and at six months post-op, it was 100. At two months postop,patient was able to fully bear weight without difficulty.Examination showed no pain on eversion and inversion ofright ankle and the range of motion was full. After sixmonths, he was already able to start playing football asbefore with no further swelling or pain. The last follow upwas at two years post-op; he was asymptomatic and carryingout normal daily activities and sports with no difficulty. The radiograph film showed no osteoarthritic changes of his rightsubtalar joint.The second patient was a 16-year old girl who presented withbilateral ankle pain and swelling for 4 years .The pain wasdull in nature, aggravated by walking, relieved by rest,radiating to the heel and gradually worsening and thuslimiting her daily activities. She also complained aboutdifficulty walking on uneven surfaces. She had no previoushistory of trauma.On examination, there were bilateral bony swellings at thepostero-inferior aspect of medial malleolus which weretender. Skin overlying it was normal. The range ofmovements of ankles was full. Subtalar movement ofinversion and eversion were associated with pain. CT scanThe both revealed prominent bony overgrowth projectingmedially from the sustentaculum tali and the medial part ofthe talus, appear to be impinging upon the adjacent flexortendons. She was Subtalar coalition can be congenital, as a consequence ofautosomal dominant inheritanceA large case series by Takakura et al demonstrated that abony prominence inferior and posterior to the medialmalleolus may be palpated in cases of talo-calcanealcoalition. These patients may also exhibit symptoms of tarsaltunnel syndrome, such as sensory disturbance in the sole ofthe foot and a positive Tinel’s test There is lack of evidence to support the use of conservativemanagement for subtalar coalition. Generally there are twooptions of surgical treatment, which is resection or fusion,with or without adjunctive reconstruction procedures3.Historically, tarsal coalitions have been treated with isolatedor triple arthrodesis . However, as these patients were youngwith no evidence of arthritis, we proceeded with surgicalexcision of the coalition. Younger patients tend to fare betterwith resection. Yu G et al concluded that to achievesatisfactory outcomes for talo-calcaneal coalitions, areasonable surgical procedure should be chosen according tothe specific facet and complicationIn conclusion, clinical suspicion is very important in thediagnosis of talo-calcaneal coalition. With newer imagingmodalities like CT and MRI, the diagnosis of subtalarcoalition should not be missed as the outcome of earlytreatment is good. We recommend the use of surgicalintervention in these young, active and symptomatic patients.


Pseudoachalasia is a rare disease that accounts for only a small percentage of patients with dysphagia. Neuroendocrine tumors are rare malignancies that most commonly originate within the gastrointestinal tract, with the next most common site being the lungs. Esophageal neuroendocrine tumors are the least common site within the gastrointestinal tract. Pseudoachalasia can be secondary to a malignant process within the body. Its typical characteristic in elderly patients is a short duration of symptoms with substantial weight loss.A 68-year-old woman presented with worsening dysphagia that had started six months after the resection of a large cell neuroendocrine carcinoma of the lung in 2011. An extensive work-up in 2012, including esophagogastroduodenoscopy, chest computed tomography and positron emission tomography, was unremarkable. Esophageal manometries revealed findings characteristic of achalasia. A repeat esophagogastroduodenoscopy in January of 2014 revealed a nearly circumferential ulcerated, fungating mass in her distal esophagus. Biopsy results confirming a recurrence of her large cell neuroendocrine carcinoma.We report a case of pseudoachalasia due to metastatic large cell neuroendocrine carcinoma of the lung. Our patient had an exceptionally prolonged duration of symptoms preceding the local esophageal recurrence, which was eventually revealed via endoscopy. Neuroendocrine neoplasms may arise in a number of organs and come from cells containing vasoactive substances within secretory granules located in the cytoplasm . Neuroen. Malignancy-associated pseudoachalasia can occur via one of three ways: the cancer can be located at the gastroesophageal junction and inhibit swallowing by mass effect; esophageal neuronal invasion by the malignancy can lead to a disruption of neuronal transmission, resulting in dysmotility of the esophagus and consequent dysphagia; or a pseudoachalasia can be associated with a paraneoplastic process [Achalasia is a condition in which there is a loss of inhibitory neurons of the myenteric plexus within the wall of the esophagus. In pseudoachalasia, the patient presents with clinical and manometric findings consistent with achalasia, but the symptoms are caused by a secondary organic entity. Pseudoachalasia needs to be excluded in older patients >60 years) who have a short duration of symptoms (<one year) and substantial weight loss . Maligna process . The lat0 years wWe present the case of a patient with pseudoachalsia due to metastatic LCNEC, who had a negative extensive workup preceding luminal manifestation for over two years.Our patient was a 68-year-old woman with chronic obstructive pulmonary disease. She had no history of gastroesophageal reflux disease or esophageal disorders but was found to have a progressive solitary pulmonary nodule (1×1.3cm). A right lower wedge resection was performed in December 2011 and the University of Iowa pathology department identified the tumor as a LCNEC.Approximately six months after her operation, our patient started having slowly progressing dysphagia for both solids and liquids. A positron emission tomography (PET) scan performed in 2012, one year post surgery, was unremarkable for recurrence or metastasis. Repeat computed tomography (CT) in June 2013 (18 months post surgery) did not show any recurrence , followed by metastases (mainly to the lung and breast) of paraneoplastic syndromes in the context of small-cell carcinoma, bronchial carcinoma, gastric carcinoma and pleural mesothelioma .We report a case of pseudoachalasia due to metastatic LCNEC of the lung in a 68 year-old woman, with mild onset of symptoms for two years prior to substantial weight loss. Over one year after her surgery, our patient had normal findings on an EGD, chest CT and PET scan. Her manometric and radiological findings were consistent with progressive achalasia. Although it might be difficult to diagnose in an early phase, it is very uncommon for a patient with metastatic pseudoachalasia to maintain weight and clinical stability for a period as long as over two years before establishment of luminal findings.We would like to suggest here that in an older patient newly diagnosed with achalasia, regardless of normal CT and PET scan results over one year after tumor resection, persistence or worsening of symptoms with extensive weight loss should indicate thorough and repeat work-up until a diagnosis of metastatic pseudoachalasia can be ruled out.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.


Background. High-grade gliomas of the brainstem are rare in adults and are particularly rare in the anterolateral medulla. We describe an illustrative case and discuss the diagnostic and treatment issues associated with a tumor in this location, including differential diagnosis, anatomical considerations for options for surgical management, multimodality treatment, and prognosis. Case Description. A 69-year-old woman presented with a 3-week history of progressive right lower extremity weakness. She underwent an open biopsy via a far lateral approach with partial condylectomy, which revealed a glioblastoma. Concurrent temozolomide and radiation were completed; however, she elected to stop her chemotherapy after 5.5 weeks of treatment. She succumbed to her disease 11 months after diagnosis. Conclusions. Biopsy can be performed relatively safely to provide definitive diagnosis to guide treatment, but long-term prognosis is poor. Glioblastomas (GBM) account for 54% of CNS gliomas; however the incidence of glioblastoma in the brainstem is not well defined . In a seDue to the rarity of medullary brainstem glioblastomas, their diagnosis and management are complex and controversial. We report the case of a 69-year-old woman with a glioblastoma of the ventrolateral medulla to highlight differential diagnosis, anatomical considerations relating to options for surgical management, and multimodality treatment.A 69-year-old Caucasian woman presented to an outside hospital with a 3-week history of progressive right lower extremity weakness. An initial MRI demonstrated a 1.9 × 0.8 × 1.0 cm contrast-enhancing mass in the left ventrolateral aspect of the medulla . She wasA repeat MRI revealed that the mass was larger, now 3.2 × 1.1 × 1.4 cm . The difSurgical intervention was planned to establish a definitive diagnosis and achieve brainstem decompression if possible. The medullary lesion was biopsied using neuronavigation with BrainLAB via a far lateral approach with partial condylectomy. Intraoperative monitoring was utilized to observe the integrity of neural pathways and included somatosensory evoked potentials (SSEPs), brainstem auditory evoked potentials (BAEPs), motor evoked potentials (MEPs), and electromyography (EMG) for the lower cranial nerves. The lesion was quite firm and rubbery but blended in with surrounding tissues. Based on an intraoperative frozen section report of high-grade glioma, the texture of the lesion, and a temporary loss of MEPs during the procedure, resection was limited. A diagnosis of glioblastoma (WHO grade IV) was subsequently confirmed by the final pathology examination Figures .The patient's postsurgical course was complicated by aspiration pneumonia requiring a period of reintubation. Postoperative hoarseness led to a diagnosis of left vocal cord paralysis. Her right-sided weakness remained unchanged from her preoperative baseline. On postoperative day five, she was transferred to a hospital closer to home for continued acute care and adjuvant therapy.Treatment was initiated with temozolomide 100 mg/day. A concurrent course of radiation therapy  was completed. She achieved some improvement in lower extremity function so that she was able to stand and pivot. She was hospitalized multiple times during radiotherapy for recurrent UTIs and required pharmacologic treatment for depression. She elected to stop her chemotherapy after 5.5 weeks of daily temozolomide. Recurrent headaches 10 months after surgery prompted a repeat MRI which demonstrated disease progression . The patGlioblastoma of the medulla is rare; thus the ability to draw definitive conclusions from the literature can be a challenge. Most larger series combine all gliomas of the midbrain, pons, and medulla together making it difficult to draw specific conclusions with regard to GBM of the medulla. Additionally tumors of the medulla vary with regard to location  which affects surgical decision-making and multimodality therapies. The signs and symptoms of a brainstem high-grade glioma overlap with those of many other CNS diseases and are dependent on the location of the lesion. Clinical features observed at diagnosis may include gait disorders, visual disturbances, limb weakness, and cranial nerve deficits , 13, 14.MRI is currently an essential, noninvasive tool for evaluating brainstem lesions. While imaging characteristics can help narrow the differential diagnosis, multiple studies , 21–24 dThe medulla oblongata extends from the inferior pontine sulcus to the roots of C1. Anteriorly, the medulla has three longitudinal fissures. The pyramids are elevated structures on either side of the anterior median fissure and comprise the descending corticospinal tracts. The paramedian sulci, also known as the anterolateral sulci or preolivary sulci, are situated medial to the olives. The rootlets of the hypoglossal nerves exit from the preolivary sulci while the rootlets of the accessory, vagus, and glossopharyngeal nerves exit from the postolivary sulci .Internal structures at this level consist of the dorsal vagus, hypoglossal, ambiguus, and inferior olivary nuclei and the nuclei of the solitary tracts. The autonomic centers participating in swallowing, respiratory, and cardiovascular functions are located in and around the reticular formation in the upper half of the dorsal medulla. The medial lemniscus originates near the caudal edge of the fourth ventricle floor and runs upward on either side of the midline, eventually coursing posterior to the pyramidal tract as it enters the pons. The pyramidal tract is found in the most anterior part of the medulla while the medial longitudinal fasciculus (MLF) runs under the posterior (ventricular) surface just lateral to the median sulcus.Suggested safe neurosurgical entry points for open procedures into the ventrolateral medulla include the preolivary  sulcus located between the caudal roots of the hypoglossal and the rostral C1 rootlets . Some neWe opted to create a corridor between several rootlets of the accessory nerve and a bulbous region in the ventrolateral medulla near the postolivary sulcus . The patBiopsy is recommended and allows the advantage of histopathologic diagnosis and molecular genetic analysis. Generally, stereotactic biopsies can be performed safely with high diagnostic success rates. Kickingereder et al.  completeA number of other approaches, including ipsilateral transfrontal, contralateral transfrontal, and transcerebellar, are available for the stereotactic biopsy of medullary brainstem lesions. Each approach has advantages and disadvantages and the trajectory must be tailored to the lesion. The contralateral or ipsilateral transfrontal approaches have been shown to be effective in the biopsy of medullary lesions , 30–32. Recent advances in neuroimaging, navigation technology, microsurgery, and anatomic knowledge have changed how we classify brainstem gliomas –39 and hp = 0.055).Dey et al.  examinedIn our patient, we considered a stereotactic biopsy but the distinct delineation and ventrolateral location of the mass guided our decision to proceed with open biopsy, anticipating the potential for decompression and thus postoperative functional improvement, which would not be possible with a stereotactic biopsy. During the case, we decided that aggressive resection was not in the patient's best interest for multiple reasons. The firm rubbery texture of the lesion contributed to a temporary loss of MEPs intraoperatively during the debulking of the lesion. Additionally, the frozen section diagnosis of high-grade glioma reduced the enthusiasm for a potentially harmful large resection. Given the infiltrative nature typical of such gliomas, aggressive resection would not be curative and carried a high risk of causing additional morbidity.As a group, adult malignant brainstem gliomas have an overall poor prognosis with a mean overall survival ranging from 11 to 17 months , 13, notRadiochemotherapy recommendations for patients with high-grade brainstem gliomas are less clear and study cohorts are typically small. Hundsberger et al.  describe2 administered on days 1–5); all eventually recurred, received a variety of salvage therapies, and died. Median overall survival was 13.5 months (range 1.9–45.7 months).Primary malignant brain tumors in the elderly (age > 65 years) carry a dismal prognosis compared to younger patients , 47. TheSome patients may obtain clinical improvement after adjuvant therapy. In a study of 38 patients with brainstem glioma who underwent radiation treatment , 50% experienced clinical improvement at 6 weeks . GuillamModern evaluation and classification of gliomas are increasingly driven by molecular genetic analyses. Patients whose gliomas, even high-grade gliomas, have methylation of the promoter region of the gene for MGMT (which thereby suppresses expression of this DNA repair enzyme) have better response to radiation and alkylator chemotherapy and have longer survival. The presence in the tumor of a mutation of the IDH1 or, less commonly, IDH2 genes similarly is associated with better response to therapy and longer survival. On the other hand brainstem gliomas and other midline gliomas, mostly found in children, frequently are found to harbor mutations in the H3F3A gene , and this is associated with a particularly poor prognosis and short survival. In this case the small amount of viable tumor tissue obtained for pathological analysis limited the testing done .Glioblastoma of the medulla is rare. Biopsy can be performed relatively safely to provide definitive diagnosis to guide treatment, but long-term prognosis is poor.


Bile duct adenoma (BDA) is a comparatively rare disease clinically, therefore, there are relatively few reported cases about it both in China and abroad.Herein, we present a 51-year-old man, diagnosed preoperatively with enhanced-contrast abdominal computed tomography, as having a nodule in the left hepatic. The patient underwent a liver tumor resection, and the histological examination revealed bile duct adenoma (BDA).BDA is an extremely rare benign tumor, which is difficult to distinguish BDA from hepatocellular carcinoma definitely preoperatively, surgical resection is needed as a way of treatment. Bile duct adenoma (BDA) is a comparatively rare disease clinically, therefore, there are relatively few reported cases about it both in China and abroad. Herein, we report a case of BDA as well as a literature review, thus presenting a preliminary analysis on BDA.The patient, a 51-year-old male, was transferred to receive surgical treatment after he was found to have low density node on the top of his right liver when receiving a chest CT examination during his treatment period on lung infection and cerebral hemorrhage sequelae in the Department of Neurology. The result of his physical examination showed no yellow sclerae or positive sign in his abdomen. The liver function tests were within normal limits. The serum levels of tumor markers, including carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, and α-fetoprotein (AFP), were not elevated and the hepatitis virology examination was negative. Enhanced CT scanning showed low density nodes in his left hepatic lobe near the top of the liver and an obvious enhancement of the arterial phase, along with which there were also the feeding arteries and perifocal hypertransfusion with an enhanced drop of venous phase and delayed phase. Therefore, the left hepatic nodule was clinically diagnosed with the possibility of liver cancer. When he was receiving an exploratory laparotomy under general anesthesia, the patient was also found to have a medium hard mass measuring about 1.4 cm close to diaphragmatic dome in his left hepatic lobe, but the mass had not infiltrated the liver capsule. The lump was then completely removed together with the margins at a periphery of 2 cm. The pathological report showed bile duct adenoma (BDA) and fatty degeneration of hepatic cells, with the immunohistochemical analysis revealing p53 (−), cytokeratin (CK) 7 (+), CK19 (+), CEA (−), AFP (+), and epithelial membrane antigen (EMA) (+) (Figures BDA is a rare case of benign intrahepatic tumor which is mostly found in patients between the age of 20 to 70 years old, averaging 55 years old, with no significant gender difference in the incidence of this disease . As the 2WI was considered as hypointense; it was proposed that the difference is caused by the number of the fibrous tissues in the tumor and whether there is calcification in the center of the tumor [As the imaging features of BDA are lacking in specificity, it is very hard to distinguish BDA from primary liver cancer. In this case, a plain CT scan showed that there was a nodular hypodense shadow of 1.5 cm with a hazy border close to the liver capsule in the juncture of segment 4 and 8 Figure . The hetTraditionally speaking, BDA is a type of proliferative lesion originating from the damaged intrahepatic bile duct epithelium . The maiAlthough BDA is defined as a benign tumor, it still has the possibility of carcinogenesis. Hasebe et al. reported a case in which BDA developed into cholangiocarcinoma . ThroughBDA is an extremely rare benign tumor, which is difficult to distinguish BDA from hepatocellular carcinoma through imaging examination .Its main character of pathological is a large quantity of proliferated bile ducts, which can be observed in the lesion,They are CK7, CK19, and EMA immunoreactive, and are p53 and Ki67 negative. Surgical resection is needed as a way of treatment.Written informed consent was obtained from the patient for publication of this case and for the accompanying images.All authors have made substantive contributions to the study, and are in agreement with the conclusions of the study. Furthermore, there are no financial competing interests.LC and FC treated the patient. LC wrote the manuscript. MYX performed the literature search. All authors read and approved the final manuscript.None.