Intracranial epidermoid cysts are generally located in the cerebellopontine and parasellar areas and appear hypo-dense on computed tomography and hypo-intense on T1-weighted magnetic resonance imaging. We report a case of an unusual epidermoid cyst of the cerebellopontine angle extending into the upper cervical canal that appeared hyper-dense on computed tomography scanning, hyper-intense on T1-weighted magnetic resonance (MR) images, and hypo-intense on T2-weighted MR images. Epidermoid tumors represent remnants of ectodermal tissues misplaced during embryogenesis and account for approximately 0.3 to 1.8 % of intracranial tumors. They are most common in the cerebellopontine angle and suprasellar and parasellar regions . IntracrA 46-year-old man presented with tingling sensation and numbness on both lower extremities. CT showed hyper-dense masses in both cerebellopontine areas. In the preoperative MR image, a well-defined, lobulated mass with a size of about 5.6 × 2.7 × 6.1 cm is seen in the pons to the medulla area with several internal septae. The mass showed hyper-intensity on T1-weighted, hypo-intensity on T2-weighted, and hypo-intensity on diffusion-weighted images. This lesion shows no gadolinium contrast enhancement. The superior portion of this cystic lesion compressed the lower portion of the pons. The inferior portion of the lesion extended to the posterior and was surrounding the medulla, extending to the C1-C2 intervertebral level Fig. . A grossEpidermoid tumors are rare congenital lesions originating from the ectoderm that constitute 0.3 to 1.8 % of all intracranial neoplasms . IntracrPathologically, epidermoid cysts have well-circumscribed, irregular, thin walls with squamous epithelium lining. The epithelium undergoes progressive desquamation and keratin breakdown; therefore, the cystic contents include tissue debris, keratin, water, and solid cholesterol .The typical imaging appearance is homogenous non-enhanced hypo-dense on CT, hypo-intense on T1-weighted MR imaging, and hyper-intense on T2-weighted and diffusion-weighted imaging. The CT density of the epidermoid cyst is usually between −2 and +10 Hounsfield units (HU) , 4. ThesThe treatment of choice for epidermoid cyst is a total resection of the tumor. But sometimes total resection is impossible because of anatomical complexity. This tumor was fortunately dissected easily from the adjacent structures. But we could not remove all of the tumor because of the long distance from the surgical field, not due to tumor adhesion. So some part of the tumor was left behind at the right upper cervical level. We periodically followed up the patient, and fortunately, the remnant lesion completely disappeared.The 46-year-old man in our report suffered tingling sensation and numbness on both lower extremities. CT showed hyper-dense masses in both cerebellopontine areas which extended to the C1-C2 intervertebral level. The mass was hyper-intense on T1-weighted and hypo-intense on T2-weighted images.The cerebellopontine angle is the most common site of the epidermoid tumors, followed by the prepontine, suprasellar, and parasellar cisterns in order. It is less frequently located in the parasellar region and the medial cranial fossa . To the We report a case of epidermoid cyst that displayed hyper-intensity on T1-weighted MR images with upper cervical extension. We think the reason for the unusual downward extension into the upper cervical canal is related to the anatomical structure of the cistern and flow of the cerebrospinal fluid.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Neurofibromatosis type 1 (NF1) is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO) and extraosseous (EO) neurofibromas is rare. The present case highlights the importance of imaging findings in the diagnosis and management of multifocal jaw, infratemporal, and parotid neurofibromas with muscular hypoplasia in an eight-year-old girl with NF1. Apart from orthopantomograms (OPG), three-dimensional computed tomography (3D CT) and cross-sectional reformations were valuable in delineating the extent of the lytic lesion and identifying additional bony deformities of the mandible. Magnetic resonance imaging (MRI) helped to identify the solid nature of the lesion and true extent of the soft tissue mass. NF1 or von Recklinghausen's disease is a neurocristopathy presenting with certain cutaneous, ophthalmologic, and skeletal manifestations . IntraorNeurofibroma (NF) extending to the maxilla and associated with multifocal lesions in the infratemporal fossa, temporomandibular joint, parotid, and external auditory canal is extremely rare. Only one such case has been stated in literature showing multifocal jaw and soft tissue tumors . ManagemAn 8-year-old girl referred to the Dental Department presented with right facial asymmetry due to a slow growing painless swelling of 6-month duration. There was periauricular soft tissue swelling and intraoral bony hard enlargement of right mandibular alveolar ridge Figures . A histo S-100  confirmed the diagnosis of simple neurofibroma , uncommonly the facial nerve, glossopharyngeal nerve, or upper cervical nerves , 10. LocD'Ambrosio et al. in a systematic clinicoradiographic study of 38 NF patients found head and neck manifestations in 92% of the subjects. They objectively defined the radiographic findings and highlighted the periodic monitoring of patients for oral and radiographic development . SigilloPlexiform NF is highly suggestive but not pathognomonic of NF1 . PresentManagement of NF1 requires periodic monitoring after baseline evaluation and carefully noticing the changes in consecutive visits to rule out onset of complications like malignant transformation that occurs in 6–29% of cases . The indPeriodic close surveillance is of utmost importance in management of a disease with unpredictable natural history and outcome as NF1. Present report of this rare case with multispectral radiological manifestations underlines the importance of imaging surveillance for the same.


Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumor with distinctive morphological features and malignant clinical behavior. Only a few such cases have been described in the literature. We report here a case of unusual pulmonary EIMS with multiple bone metastases. A 21-year-old Chinese male patient presented with complaints of general fatigue and rapid weight loss, and a huge tumor arising in the left lower lobe of lung was detected by chest computed tomography. The mass of lung was totally resected. Microscopically, the tumor cells were rounded and epithelioid in shape. Myxoid stroma and inflammatory infiltration was also present. The tumor cells were immunopositive to anaplastic lymphoma kinase (ALK) in smooth cytoplasmic pattern. Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene. Three months after lung surgery, there were multiple bone metastases and intraspinal mass found by positron emission tomography. The second surgical treatment was performed to remove the intraspinal lesion. The histological and immunohistochemical features of intraspinal mass were similar to those of pulmonary tumor. The diagnosis of pulmonary EIMS with multiple bone metastases was made. To the best of our knowledge, it may be the first case of an EIMS arising in lung. Awareness of EIMS in respiratory tract and its distinctive features is important for pathologists to avoid a diagnostic pitfall caused by histologic similarities to other ALK-positive tumors. ALK inhibitor is a promising treatment for this aggressive tumor regardless of its potential acquired resistance. Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate biological potential, which frequently occur in the lung or abdominal cavity of children and young adults. Its clinical course is relatively indolent, but may recur and rarely metastasize . RecentlA 21-year-old non-smoking young man presented with complaints of general fatigue and rapid weight loss in the past 3 months. During this period, he was referred to a local hospital for radiological examination. Computed tomographic (CT) scans revealed a huge mass in the left lung. A fibreoptic bronchoscopy was performed in the local hospital, but biopsy examination was negative because only inflammatory mucosa was observed under the microscopy. As a result, the patient was referred to our hospital for examination and treatment. Physical examination results were normal. The laboratory results, including blood count, serum tumor markers and liver and renal function, were within the normal range. The CT images acquired at the local hospital showed a 10.0 × 8.0 cm well-circumscribed mass in the left lower lobe of lung without signs of neighboring pleura invasion Fig.  and b. TThe surgical specimens was received and routinely fixed in 10 % neutral buffered formalin after tumor resection. Four micrometer thick sections were cut and stained with H&E. On microscopic examination, the pulmonary and intraspinal masses exhibited similar histological appearance. Both tumors were non-encapsulated and had an infiltrative margin. The pulmonary tumor was composed of two areas with distinct histological appearance. In one area, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. The microscopical appearance of this area was consistent with the histological features of conventional IMT Fig. -c. HowevImmunohistochemically, the tumor cells in both messes were positive for vimentin and desmin diffusely. They were negative for Pan-CK (AE1/AE3), smooth muscle actin (SMA), HHF35, myogenin, Myo D1, S100, HMB45, Melan A, synaptophysin, CD34, CD68 and CD30. In addition, the tumor cells were positive for ALK, exhibiting a cytoplasmic pattern Fig. -e. The rOn the basis of gross, histopathological features and immunohistochemical phenotypes, its epithelioid morphology and myxoid background, the presence of inflammatory infiltrating and ALK cytoplasmic positive signal pattern, a diagnosis of epithelioid inflammatory myofibroblastic sarcoma (EIMS) with multiple bone metastases was made.EIMS is proposed as a rare variant of IMT and has firstly been designated by Marino-Enriquez et al. in 2011 because of its malignant clinical behavior [Since its first description by Marino-Enriquez et al. so far only 13 such cases have been described in the literature -ALK fusion occurring ALCLs has been reported to be detected in approximately 50 % of IMTs . BesidesThe EIMS appears to have a poor prognosis with short survival time after surgical treatment and following chemotherapy and /or radiotherapy. Among those reported cases, 5 patients died within 3 to 36 months after the initial diagnosis, and 9 patients occurs local tumor recurrence and 3 patients had distant metastasis –4. Our pIn conclusion, we encountered an unusual case of pulmonary EIMS with rapidly lethal clinical course. To the best of our knowledge, it may be the first case of EIMS originating in the lung with multiple bone metastases. Due to its rarity, the diagnosis of EIMS, especially occurring in unusual sites, is difficult and should be made cautiously. Awareness of EIMS in respiratory tract and its distinctive features is important for pathologists to avoid a diagnostic pitfall caused by histologic similarities to other ALK-positive tumors. RANBP2-ALK fusion likely plays the essential role in the carcinogenesis of this tumor, and ALK inhibitor is a promising treatment for this aggressive tumor regardless of its potential acquired resistance.Written informed consent was obtained from the patient’s family for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Inflammatory myofibroblastic tumor (IMT) is a distinct pseudosarcomatous lesion arising in the soft tissues and interior organs of children and young adults. It is rarely seen in adults. It was first described in lungs. IMT can occur in any location in the body. However, it is seen most commonly in lungs, intestinal mesentery and liver. Non-mesenteric alimentary tract IMT’s are quite rare. The presented case is an ileal IMT that caused small bowel invagination. A 38 year-old male patient presented to the emergency department with the complaint of diffuse abdominal pain, distension and no passage of gas or stools for two days. An abdominal examination revealed distension and tenderness in the abdomen with no guarding or rebound tenderness. Computerized tomography (CT) of the abdomen was ordered. CT revealed an image compatible with invagination of the right lower quadrant of the abdomen and a mass inside the lumen measuring 4x3x3cm. The mass causing invagination was detected during the surgical operation. A segmentary small bowel resection and ileoileal anastomosis was performed. The patient was discharged uneventfully on the postoperative sixth day. The diagnosis of IMT was confirmed histologically and immunochemically. Inflammatory myofibroblastic tumor (IMT) is a distinct pseudosarcomatous lesion arising in the soft tissues and interior organs of children and young adults. It is rarely seen in adults. Although it was first described in lungs,A 38 year-old male patient with no previous medical problem presented to the emergency department with the complaint of diffuse abdominal pain, distension, and no passage of gas or stools for two days. Abdominal examination revealed distension and tenderness in the abdomen with no guarding or rebound tenderness. His laboratory tests were as follows: WBC:12.1x10ˆ3/ml, urea:92.0 mg/dl, creatinine:1.15 mg/dl, chloride:111.0 mmol/L and glucose:127 mg/dl. Hemogram and other biochemical tests were within normal ranges. Upright direct roentgenogram of the abdomen revealed air-fluid levels of the small bowel type. Abdominal ultrasonography (US) disclosed a mass in the right lower quadrant of the abdomen and a computerized tomography (CT) of the abdomen was ordered. CT revealed an image compatible with invagination of the right lower quadrant of the abdomen and a mass inside the lumen measuring 4x3x3 cm. An ileo-ileal invagination was observed during laparotomy at a location 220 cm distal to the Treitz ligament. Following the correction of the invagination a solid mass with regular contours was noticed inside the lumen. A segmentary small bowel resection and ileo-ileal anastomosis was performed. The patient passed gas on the postoperative first day and he was fed on the third day. The patient was discharged uneventfully on the postoperative sixth day. The diagnosis of IMT was confirmed histologically and immunochemically.This was a 38 year-old male patient who was diagnosed to have a small bowel IMT. IMT is seen more frequently in children and young adults and is more common in the female gender.6IMT has typically been considered as a benign lesion. However, some variants of the tumor may be locally destructive. Biselli et al. reported that chromosomal aberrations may be present in IMT.7Atypical intestinal gas distribution in the upright abdominal X-ray, and intratumoral calcifications, might be observed in cases with small bowel IMT. A vague solid mass with regular contours can generally be seen in abdominal ultrasonography and CT imaging. In this present case, a solid mass measuring 4 x 3 cm in the left lower quadrant of the abdomen, and an image compatible with invagination in the same area, was detected using abdominal tomography.Microscopically, IMT resembles gastrointestinal stromal tumors. Tumor cells may invade the muscularis propria and even the adventitia without the presence of atypia or hyperchromatism.Immunohistochemical analysis plays a major role in the definitive diagnosis of IMT. Tumor cells, characteristically vimentin positive and with no expression of CD117 and CD34, are present.Surgical excision is the primary choice of treatment in cases with IMT.11


Schwannoma is a benign, solitary, and slowly progressive encapsulated tumor originating from the sheath of myelinated nerve fibers. Schwannoma of the salivary gland is a particularly rare form of an extracranial neurogenic tumor. Here, we present an unusual case of a schwannoma of the submandibular gland in a 19-year-old man. Total excision of the submandibular gland resulted in complete resolution of symptoms with no cranial nerve deficits. The details of the histopathologic and immunohistochemical features are presented.A 19-year-old Caucasian man was admitted to our clinic with a painless mass on the right side of his neck that he had had for the past four months. A neck examination revealed a smooth-surfaced, mobile, firm, and painless mass, 6cm in its greatest diameter, on the right side of the submandibular region. Fine-needle aspiration cytology was suggestive of a submandibular gland schwannoma. After the initial evaluation, our patient was prepared for surgical evaluation and resection with a presumptive diagnosis of a neurogenic tumor of the submandibular gland. The final diagnosis of schwannoma was verified by microscopic and immunohistochemical studies. At one-year follow-up of the case, there was no evidence of recurrence.Schwannoma of the salivary gland is a particularly rare form of an extracranial neurogenic tumor. Our findings indicate good prognosis in an unusual case of a submandibular gland schwannoma in a 19-year-old man treated by surgical excision with no recurrence within 12 months of follow-up. Schwannomas are benign, solitary and well-differentiated tumors originating from Schwann cells. Nearly A 19-year-old Caucasian man was admitted to our clinic with a painless mass on the right side of his neck for the past four months Figure . Our patOur patient was free of symptoms at the 12-month follow-up.Schwannoma, first described in 1908 by Verocay, commonly occurs between 30 and 50 years old; howeverClinical evidence of the tumor usually does not present for a long time. The most common symptom is a slow-growing mass,4,7,8. Net al. have reported their 10 years of experience regarding extracranial head and neck schwannomas, and in their report, only 6 percent of patients could have been diagnosed preoperatively on the basis of clinical findings, CT and MRI scans, and FNA[Diagnostic investigations include computed tomography (CT), magnetic resonance imaging (MRI), ultrasound scan and FNA. MRI is the best choice in detecting the extent of the tumor and correlates well with the operative findings. Biswas  and FNA. Schwann and FNA. Diagnos and FNA,12,13. Wet al. reported that more than half of the surgically treated cases exhibited postoperative neural deficits that were primarily caused by iatrogenic injury to either the nerve of origin or adjacent neural ending[The treatment of schwannomas is problematic. Because of resistance to radiotherapy, surgical excision is necessary for optimal treatment,9,14,15.l ending. In our The malignant potential of extracranial schwannomas and risk of recurrence after surgical resection are unclear, while iSchwannoma of the salivary gland is a particularly rare form of an extracranial neurogenic tumor. Our findings indicate good prognosis in an unusual case of a submandibular gland schwannoma in a 19-year-old man treated by surgical excision with no recurrence within 12 months of follow-up.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GA was the primary physician of the patient, interpreted the patient data and was the major contributor to writing the manuscript. FC operated on the patient and contributed to writing the manuscript. FKC performed the histopathological examinations and contributed to writing the manuscript. All authors read and approved the final manuscript.


Klebsiella rhinoscleromatis. The patient was given a six-month regimen of ciprofloxacin, and a dramatic improvement was observed.A 39 year-old man came to our institution because of a five-year history of a progressive painful growing mass on his left nostril, which cause airway obstruction with ulceration. Because of a suspicion of malignancy, surgery  was performed. Histologic samples ruled out malignancy, and tissue cultures for fungus and mycobacteria were negative, but regular aerobic cultures were positive for  A 39 year-old man who had lived his entire life in rural Colombia and who had no significant medical history except for a minor trauma in his left cheek and temporal region more than 10 years ago. He came to our institution with a history of five years of a growing painful mass on his left nostril, which caused airway obstruction. At physical examination, he had an erythematous mass with central ulceration, purulent discharge, adherent to deep tissues, producing partial obstruction of the left eye visual field, and complete obliteration of the left nostril  and B. CA biopsy and culture were not performed. However, because of a suspicion of malignancy, a surgical mass resection was performed that included the left nasal bone, and the orbital and maxillary sinus wall; all orbital structures were preserve. The same day, a subtotal nasal reconstruction with an antebrachial free flap was performed. The patient showed an uneventful postoperative course.Klebsiella rhinoscleromatis.Histologic samples from the mass and borders ruled out malignancy, but showed groups of large vacuolated histiocytes containing gram-negative bacteria (Mikulicz cells), many of which had Russell bodies  was started, and the patient showed dramatic improvement. At the most recent follow-up, there were no signs of relapse of infection, and the patient was scheduled for a definitive nasal reconstruction .


Our patient was a 76-year-old female who has been operated on 2 times in 8 years for pelvic chondrosarcoma. The patient came to our clinic with a large mass in left iliac region which extended into the paravertebral area. Physical examination and preoperative imagining studies revealed a mass at the left iliac area that infiltrated sciatic notch and extended from posterior iliac region towards the anterior side of iliac bone through the sciatic notch and an incisional hernia including descending colon. The mass was also penetrating the abdominal cavity through the hernia. Surgical intervention was planned. Since the tumor infiltrated the sciatic nerve, hemipelvectomy was indicated. Patient refused hemipelvectomy. Therefore, palliative debulking surgery was considered. We treated the case with marginal excision and abdominal wall reconstruction employing prolene and vicryl suture materials in order to prevent a postoperative visceral herniation and local invasion. At the latest follow-up appointment in 2 years, the patient still had no signs of tumor recurrence. This case showed us that an incisional hernia can serve as a pathway for the recurrence invasion of the chondrosarcoma. Chondrosarcomas constitute a heterogeneous group of neoplasms that have the production of cartilage matrix by tumor cells in common . ChondroThe pelvis is the most common site of occurrence with the ilium being the most frequently involved bone, followed by the pubis and ischium . AlthougA 76-year-old woman came to our clinic with a large mass of the left iliac region.According to the patient's history, she had a pelvic tumor removal surgery in 2006 and she was reoperated on for local recurrence in 2009. During admission to our oncosurgical clinic, an incisional hernia and a large mass of left iliac region were diagnosed. The patient was neurovascularly intact.Radiological studies showed a mass at the left iliac area that infiltrated sciatic notch and extended from posterior iliac region towards the anterior side of iliac bone through the sciatic notch and incisional hernia. The mass was also penetrating the abdominal cavity through the fascial defect and infiltrating the mesosigmoid that herniated through the fascial defect out of abdominal cavity Figures  and 2. SThe tumor was approached through the utilitarian pelvic incision that extended into the inner side of thigh and lateral border of the abdomen .The tumor in the pelvic and paravertebral area was marginally excised . AbdominWide surgical tumor excision with adequate margins appears to be the procedure of choice in chondrosarcoma treatment, since this is the most effective way of reducing tumor recurrence rate –9. AlthoThe key point of our paper was to define an unusual way of local recurrence. To the best of our knowledge, no case of clearly defined pelvic chondrosarcoma developing local recurrence through the abdominal hernia into the abdomen was reported previously. Regarding the originality and rarity of our case, we performed a literature scan by employing PubMed database. The research criteria were as follows: (1) the research had to be in English, (2) papers examining pelvic chondrosarcoma related with colon and small intestines, (3) cases involving chondrosarcoma and abdominal wall repair, (4) cases involving bone resection that resulted abdominal hernia, and (5) cases involving chondrosarcoma, bone resection, and abdominal hernia altogether. Out of 252 articles, only 14 studies fulfilled the criteria described above. There was no case related with local recurrence and invasion into mesocolon area through the abdominal hernia. Considering this fact, we can say that our paper is unique in terms of focus on a local recurrence and invasion into mesocolon through the abdominal hernia.Two articles  docExtraskeletal myxoid chondrosarcoma is differentiated by a biological structure that is distinct from the genetic heterogeneity observed in other forms of chondrosarcoma. Despite its high tendency to local recurrences and distant metastases, it has a relatively slow growth and a lazy clinical course , 13.Bustinza-Linares et al.  presenteHull  reportedWe searched the literature for the link between bone resection (the resected bone was used for bone grafting) and abdominal hernia  and 7 articles were found –20.Petsatodis et al. performed radical excision of the tumor (chondrosarcoma) at symphysis pubis and used synthetic mesh in order to reduce the possibility of a postoperative visceral herniation . They emJuan-García et al.  reportedIn our case we repaired abdominal wall carefully with prolene and vicryl suture materials to prevent future herniation and local invasion of pelvic chondrosarcoma.This case showed us that an incisional hernia can serve as a pathway for the recurrence invasion of the chondrosarcoma.In conclusion, surgeons should separate compartments by materials like mesh or sutures to minimize the ratio of intercompartmental recurrence.


Single-site laparoscopic colectomy (SLC) is a promising minimally invasive and safe treatment for colorectal cancer. Improvements of the working instruments and procedures for SLC have helped to overcome challenges regarding the difficulty of operation, supporting the gradual acceptance of this technique. In contrast, narrow working space of the abdominal cavity sometimes prevents securing an adequate surgical view. To obtain precise anatomical information and enable complete mesocolic excision (CME), we routinely perform three-dimensional computed tomography prior to SLC.A 69-year-old Japanese woman was clinically diagnosed with rectosigmoid cancer. Unexpectedly, preoperative examination revealed asymptomatic stenosis of the great artery, which was diagnosed as middle aortic syndrome. Because radical colectomy requires dissection of vessels that supply blood flow to the legs, a vascular stent was inserted prior to operation. We chose SLC due to the reduced risk of damaging epigastric arteries, which may eventually become collaterals in the event of stent re-stenosis. We accomplished SLC with CME, and the patient was discharged on the tenth day after operation without complications.The present case is the first to proceed by SLC for colorectal cancer complicated by vascular obstructive disease. Preoperative imaging enabled us to identify an unexpected rare disease and to still accomplish SLC with CME, thus reinforcing the importance of preoperative imaging to optimize the use of SLC. In addition, SLC may become one of the most adequate procedures for patients complicated by vascular obstructive disease. Single-site laparoscopic colectomy (SLC) for colorectal cancer is a minimally invasive surgical procedure that satisfies patients’ demands regarding pain reduction and cosmetic improvement, as well as reduces risks of complications —such as Here, we report a case in which we discovered an unexpected occlusive disease of a great vessel during computed tomography angiography (CTA) prior to SLC. Using this information, we successfully performed stent placement and then achieved SLC. To the best of our knowledge, this is the first literature of laparoscopic surgery for colon cancer with occlusive disease of great vessels. The present case study highlights the importance of preoperative imaging and the utility of SLC.A 69-year-old female Japanese patient suffered from narrow stool and was determined to have a 15-mm superficial lesion with central depression in the rectosigmoid colon Fig. .Fig. 1PrWe observed no blood vessel wall thickness at the narrow segment and only a little calcification, then we diagnosed middle aortic syndrome (MAS). Although we did not detect the overswelling of the inferior epigastric artery that is usually seen in this condition as the collaterals to the legs, the IMA communicated with the collateral circulation branched from the superior mesenteric artery and gastroduodenal artery, such that blood flow to below the narrow segment was expected to be due to IMA reflex. Furthermore, deficiency of left colic artery (LCA) meant that the bloodstream to below the narrow segment depended only on collateral circulation, namely, curative colectomy with vascular ligation blocked blood flow to the legs.The patient exhibited no hypertension, leg numbness, coldness, or intermittent claudication—but further examinations revealed the following low ankle brachial index (ABI) values: 0.69 in the right and 0.66 in the left. From this information, we anticipated a low ability to preserve blood flow to the legs and decided that vascular reconstruction was needed. Angiography revealed that the collateral circulation flowed to the IMA, as expected from the CTA Fig. a. We choEight days after vascular reconstruction, we performed single-incision laparoscopic anterior resection. We started SLC with a 2.5-cm vertical incision in the umbilicus Fig. a. We ligMany reports describe the minimal invasiveness and safety of SLC for colorectal cancer. SLC has gradually been accepted for its improved patient satisfaction, while also ensuring adequate oncologic clearance and safety . CME witIn the present case, CTA was performed as a preoperative examination prior to laparoscopic surgery, and it unexpectedly revealed stenosis of the abdominal aorta and development of collateral circulations. We diagnosed MAS, which is a rare disease of unknown cause that involves locoregional stenosis between the arch and the terminal bifurcation of the aorta . AlthougAlthough vascular reconstruction secured the blood flow to the legs, it was also important to avoid damaging the epigastric artery so that it would still be available to serve as collateral circulation in the event of future stent re-stenosis. From the various possible procedures, we chose SLC for several reasons. While laparotomy with midline incision is unlikely to directly damage the epigastric artery, this procedure is highly invasive, and the operator must be careful not to compress the epigastric artery with the wound retractor as this has been reported to obstruct blood flow . On the There are only four previously reported cases of operation for colorectal cancer with stenosis of the abdominal aorta and collateral circulation Table . All fouIn the presently described case, preoperative imaging enabled the discovery of an unexpected rare vascular obstructive disease. The information obtained from this preoperative examination was sufficient to enable use to take adequate safety precautions and to still perform curative resection with SLC, without any complications. The present case reaffirms the importance of preoperative diagnosis and indicates the merit of SLC for patients complicated by vascular obstructive disease.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Papillary fibroelastomas are cardiac benign tumours. Among the benign cardiac tumor, papillary fibroelastomas are reported second after myxomas. Most often diagnosed incidentally, papillary fibroelastomas may embolize to cerebral circulation. Valvular locations are predominant; location in left atrium is rare. In this paper, we present a case of papillary fibroelastoma located in left atrium with symptoms of cerebral embolization. Transoesophageal echocardiography diagnosed a mobile mass. The patient was treated with surgical resection without further embolic complication. Primary cardiac tumors are rare. In autopsy series their incidence varies between 0.0017 and 0.28%. Papillary fibrolelastoma (PFE) is the third most common benign cardiac tumor after atrial myxomas and lipomas. PFE constitutes 10% of primary cardiac tumors [A sixty-four-year-old woman was referred to the department of cardiovascular surgery for the management of ischemic cerebral stroke suspected of cardiac etiology. During the preceding four months, she experienced one episode of transient ischemic cerebral attack. Physical examination was consistent with a monoparesis of left upper extremity and left facial paralysis. No more sign occurred. Cerebral CT scan revealed embolic lesion in the right sylvian artery area. Medical history showed a high blood pressure and hypercholesterolemia. The patient had no prior history of arrhythmia. Electrocardiogram (EKG) and Holter EKG were in sinusal rhythm. Investigations in search of a potential cause of cerebral embolism included a Doppler ultrasound of the carotid arteries which was normal. Transthoracic echocardiography (TTE)  showed aThe tumors were excised under normothermic cardiopulmonary bypass using ascending aorta and bicaval cannulation. Anterograde blood cardioplegia was used. The left atrium was exposed by extended vertical transatrial septal incision (Guiraudon). Two tumors were found: a 10 mm friable translucide mass was attached between the two pulmonary veins and a 6 mm tumor located in left atrium between right inferior pulmonary vein and the base of anterior mitral leaflet. The left atrial appendage was closed. Mitral leaflet and subvalvular apparatus were inspected and were free of tumors. After bypass arrest a TEE confirmed a left atrium cavity with no tumor. Histological examination showed a papillary fibroelastoma including few fibroblasts, collagenous tissue, and elastic fibers surrounded by mucopolysaccharide acid. The patient had uncomplicated perioperative course and was discharged from hospital on postoperative day 8. At the 6-month followup, the patient was well. She has not experienced any neurological event. Transthoracic and transesophageal echocardiography did not find any recurrent tumour at 6-month followup. PFE is a rare primary cardiac tumor. PFEs are predominantly found on the valvular endocardium (80%). Although they may be present anywhere in the heart, but the location in left atrium is rare . Most ofTumor's location in the heart determines symptoms. Left side location is associated with embolism in systemic circulation which induces cerebral ischemia, myocardial infarction, retinal artery obstruction, renal infarction, and mesenteric or limb ischemia. High potential embolism and high risk of recurrent cerebral ischemia of PFE justify surgical resection , 7. Righ Papillary fibroelastoma is a rare cause of cerebral emboli and should be suspected and ruled out in such clinical presentation.


Background. A malignant mixed Müllerian tumor (MMMT) is a malignant neoplasm found in the uterus, the ovaries, the fallopian tubes, and other parts of the body that contains both carcinomatous  and sarcomatous (connective tissue) components. Outcome of MMMTs is determined primarily by depth of invasion and stage. The metastatic background of these lesions is controversial and unknown. Case Report. A 75-year-old woman was admitted to the hospital with anorexia, weakness, and persistent coughing. The imaging exams revealed a solid, promiscuous lesion of 16 × 14 cm in dimensions located into the small pelvis, surrounding the uterus and the ovaries. The patient underwent exploratory laparotomy. The mass was removed and the histological examination of the specimen revealed an advanced mesodermal adenocarcinoma of the ovary (MMMT). Nine days after the operation the patient presented with metastatic lesions in the mouth as well as the lungs. Within a month after the discharge from the hospital metastatic lesions of the MMMT were also depicted in the CT brain scan. Conclusion. Despite the fact that sarcomas have a long-term metastatic potential, to our knowledge this is the first case of Müllerian adenosarcoma presenting with such extraperitoneal metastases.  Ovarian malignancies are very common and may develop in the form of either epithelial tumors accounting for about 85–90% of all ovarian malignancies, germ cell tumors, sex cord-stromal tumors, or mixed tumors. Single-port prophylactic laparoscopic bilateral salpingo-oophorectomy is a very promising procedure offering the benefits of minimizing the risk of ovarian cancer in patients with mutant base as well as less postoperative pain, best esthetic outcome, and faster and easier extraction of the surgical specimens. One particularly rare type of ovarian malignancies is the Müllerian adenosarcoma. To our knowledge this is the first report on such a type of malignancy, presenting with metastases to the lungs, mouth, and brain.A 75-year-old woman was admitted to our department with a 4-day history of anorexia, weakness, and persistent coughing. She had a clear medical history without cardiac, pulmonary, or any other problems. During the clinical examination a large mass was palpated in the lower abdomen. A simple chest X-ray  was condIn 1974 the term “Müllerian adenosarcoma” was used for a distinctive uterine tumor characterized by a mostly low-grade malignant stromal component and a benign, although occasionally atypical, glandular epithelial component . More thIn 2010 Elnemr et al. presented a case of primary retroperitoneal Müllerian adenocarcinoma. They presented a case of a 46-year-old woman who presented with an eight-year history of lower abdominal mass. Ultrasonography (US) and computed tomography (CT) demonstrated a 15 × 10 cm cystic mass in the left lower retroperitoneum. As serial percutaneous needle aspiration cytology was negative for malignancy, she was observed for seven years. Eleven months ago, the mass was excised. The histopathology was reported as mucinous adenocarcinoma of the retroperitoneum. Six cycles of intraperitoneal (IP) chemotherapy were administered during the last six months after diagnosis of recurrence by aspiration cytology and high serum tumor markers . A few days ago, positron emission tomographic (PET) scanning showed evidence of local recurrence and single vertebral metastasis, so she was admitted again for systemic chemotherapy. Meticulous revision of additional sections of the tumor revealed papillary, serous, mucinous, and endometrioid subtypes of the Müllerian adenocarcinoma .Quite recently in 2015 Rosas-Guerra et al. presented a case of a metastatic collision tumor in the intestine. The case was about a 71-year-old female with history of a carcinoid tumor removed 20 years before without any recurrence. The patient was admitted with intestinal occlusion symptoms secondary to a right flank abdominal tumor. An exploratory laparotomy was performed, removing the tumor and applying optimal debulking. The histopathological study reported bilateral ovary adenocarcinoma, as well as metastatic collision tumor of two histological types: well differentiated adenocarcinoma and a mixed malignant mesodermic Müllerian tumor. The patient was treated with adjuvant chemotherapy with poor results (death in 24 months) [Last but not least in 2014 Mukonoweshuro P presented a case of endocervicosis involving axillary lymph nodes. The patient was a case of benign Müllerian inclusions of mucinous endocervical type (endocervicosis) coexistent with metastatic breast-infiltrating ductal carcinoma in 2 axillary lymph nodes. The inclusions exhibited diffuse positive staining with CK7, PAX8, CA125, and estrogen receptor and were WT1 negative .Bilateral prophylactic salpingo-oophorectomy is a very promising procedure offering the benefits of minimizing the risk of ovarian cancer in patients with mutant base. Recent studies demonstrated the benefits of single-port prophylactic laparoscopic bilateral salpingo-oophorectomy versus conventional multiport access in high-risk patients for ovarian cancer. Comparing the results, single-port incision was offering the benefits of less postoperative pain, best esthetic outcome, and faster and easier extraction of the surgical specimens .The patient of the present case was considered as having a stage IV tumor according to the FIGO classification system because of the radiological findings on her CT scan of the thorax and brain even though they were not histologically confirmed as metastases. This confirmation came with the results of the biopsies of the mouth lesions, which were considered metastases from the known sarcoma.Despite the fact that sarcomas have a long-term metastatic potential, to our knowledge this is the first case of Müllerian adenosarcoma presenting with such extraperitoneal metastases.


Purpose: To evaluate the etiopathogenesis, clinical features and surgical approaches for removal of ectopic third molars in the mandible.Methods: We report a case of an impacted mandibular third molar dislocated on mandibular sigmoid notch. 20 cases of ectopic mandibular third molars reported in the English-language literature, identified from Pubmed and Medline databases are also reviewed.Results: Among the 20 article reported in the presented study, ectopic third molars were generally located in the condylar region. The common symptoms of the clinical examination were pain, trismus, swelling, temporomandibular joint syndroms or no symptoms.Conclusions: Ectopic third molar may be asymptomatic initially with clinical manifestations, later on as adjacent structures are affected. The surgical approach must be carefully planned for the aim of choosing the more conservative technique that produces the minimum trauma to patients. Key words:Ectopic third molar, sigmoid notch, cyst. Ectopic teeth often impact in unusual positions or at a distance from their normal anatomic location. Ectopic eruption of a tooth into dental structures is a common entity, while ectopic eruption of a tooth in other sites is infrequent . ReportePermanent mandibular third-molar impaction is a common condition in populations, with a frequency of 20% to 30%. Ectopic mandibular third molars, however, are unusual, with their heterotopic positions reported in the condylar area, in the ascending ramus of the mandible, or in the coronoid process. Most cases of ectopic third molars are asymptomatic and are usually found during routine clinical and radiographic investigations. In the present study, we report a case of an ectopic third molar on the right sigmoid notch with multiple cysts and compare it with the literature reports of this anomaly.We performed a literature search using the PubMed database. We used ectopic third molar and mandible as key words. Of the 45 articles found, we only considered the English literature and well-documented cases of ectopic third molars in the mandible. Our inclusion criteria were as follows: English-language articles generated by the Pubmed database search containing at least one case of an ectopic third molar in the mandible; and articles containing cases of patients with ectopic third molars located in the mandibular condyle, coronoid, and sigmoid regions . We compA 45-year-old man was referred to the Department of Oral and Maxillofacial Surgery, Faculty of Dentistry Hospital , in August 2011 for the evaluation and assessment of an impacted lower right third molar in the mandibular sigmoid notch associated with a radiolucent lesion and multiple cysts located in the jaws. His medical history also did not give any significant information that could associated with the presenting symptoms. On intra oral examination, there were no signs of any significant pathology in relation to any tooth or tissue. Panoramic radiograph examination revealed a radiolucent area surrounding the crown of the fully formed, ectopic mandibular third molar in right mandibular sigmoid notch region , intraoral, and recently, endoscopic approaches. The most commonly used extraoral approaches were submandibular access and preauricular approach. These external approaches have the advantage of good exposure of the surgical site but may result in complications such as extraoral scar formation, damage of joint components, facial nerve injury in the case of preauricular access, or damage of the marginal branch of the seventh cranial nerve in the case of submandibular access .Surgeons have use endoscopy because it magnifies the surgical field. Especially in intraoral approaches, this is an important property. However, the cost of endoscopes and the lack of training facilities prohibit the routine use of endoscopy.The selection of the surgical approach is basically linked to the experience and preference of surgeons. In one study, surgeons presented an endoscopically assisted surgical technique for the removal of an ectopic lower third molar in the condylar process associated with a dentigerous cyst . In a caIn our literature review, the authors agreed that the treatment should be carefully planned according to the location and position of the ectopic tooth and morbidity associated with surgery, with the aim of choosing the most conservative technique that produces the least possible trauma to the patient.In conclusion, occurrence of an ectopic tooth in the coronoid or condylar regions and association of a dentigerous cyst with it is a rare phenomenon. Its presence may be the asymptomatic initially with later clinical manifestations as adjacent structures are affected. The surgical approach must be carefully planned for the aim of choosing the most conservative technique that produces the minimum trauma to patients. Postoperative follow-up with radiographic examination at regular intervals is mandatory to rule out any recurrence.


The purpose of this paper is to show an unusual case of meningioma simulating arachnoid cyst on CT scan and MRI, diagnosed in a 63-year-old woman evaluated for headache and vision disorders. The meningioma shown is predominantly cystic with a small mural nodule enhancing after gadolinium and exhibiting diffusion restriction. Cystic portion of the tumor is hypodense on CT, and evidences fluid signal intensity on T1- and T2-weighted MR imaging. The objective of this paper is to show an unusual case of meningioma simulating an arachnoid cyst on CT scan and conventional MRI.Meningiomas are the most common benign intracranial tumors, representing 13–18% of all intracranial neoplasms. They are more common in women (2-3 : 1), especially middle-aged women (40–60 years old) [We present the case of a 63-year-old female patient, evaluated for intermittent headaches in the right temporoparietal region associated with blurred vision for the past two years, which had increased in the last month. As a relevant antecedent, she referred to a cranioencephalic trauma 9 years ago in a traffic accident, with impact on the right temporal lobe, with no associated lesions. At that time, she underwent a CT scan, reporting no intracranial hematic collections.Unenhanced CT scan was performed, identifying a hypodense image in the right frontoparietal region, with clear borders. A small mural hyperdense nodule was also identified . Such leThen, an MRI study was conducted using a high-field (1.5T) resonator, without and with gadolinium enhancement, applying diffusion technique and spectroscopy. Conventional sequences showed the lesion to be hypointense on T1-weighted sequences and hyperintense on T2-weighted and FLAIR sequences, with a dominant cystic component and a small mural nodule . After iMR spectroscopy showed an elevated choline peak and a decreased N-acetylaspartate peak at the mural nodule level. Furthermore, a double peak at 1.3 ppm and 1.5 ppm was observed, probably corresponding to an increase in the lactic and alanine peaks . With thThe patient underwent surgery, the lesion was completely resected, and macroscopic features were the presence of a cystic lesion containing a dense proteinaceous liquid, with a peripheral nodule of 8 mm in maximum diameter. Anatomic pathology revealed monomorphous cell proliferation, formed by medium sized cells with slightly hyperchromatic nuclei and moderate cytoplasm in a solid pattern, associated with the presence of numerous blood vessels with prominent walls. Immunohistochemistry revealed the following monoclonal antibodies: anti-EMA (+), Anti-Vimentin V9 (+), Anti-Cytokeratin Ae1/Ae3 , and Anti-Ki-67 (+). Definitive diagnosis was cystic meningioma. Three months after surgery, brain MRI without and with gadolinium enhancement showed neither persistence nor meningioma recurrence .Meningiomas are usually benign tumors which originate from the meningothelial cells, extra-axial, frequently solid lesions, presenting typical imagenologic features both at CT scan and MRI in most cases 85%) . Associa% . AssocLarge eccentric cyst walls are associated with reactive gliosis or with collagen. Neoplastic cells are rarely found in the distal cyst wall; therefore, the entire cyst wall should be resected to prevent tumor recurrence –8.Several authors have described the relation between meningioma and traumatic brain injury (TBI), especially Cushing, who cited 24 cases of a strong association between location of TBI and that of meningioma. In our case, the patient had a history of TBI with frontal and temporal lobe damage which coincided with the location of the meningioma, in keeping with Cushing's theory. However, the association between meningioma and TBI with frontal and temporal lobe damage as risk factor is currently controversial.The literature describes some theories on the mechanism of cyst formation in this entity. Penfield believes that this is due to central degeneration within the tumor. Cushing suggested that cyst formation is due to the build-up of xanthochromic fluid at the periphery and that its coalescence leads to the formation of large cavities .Intratumoral cysts may be the result of a degenerative process, ischemic necrosis, or hemorrhage . They maPreoperative differentiation between cystic meningioma and other brain neoplasms such as gliomas, hemangioblastomas, and metastases with cystic component is difficult and frequently carried out after pathological examination , 12. BraIn diffusion techniques, meningiomas have different behavior according to the degree of cellularity, usually showing restriction to water molecules. In our case, the cystic component showed no restriction, presenting facilitated diffusion, but to a lesser extent than cerebrospinal fluid. The mural nodule showed restriction.Spectroscopy reveals lower NAA, increased choline peak, and decrease in the NAA/Cho ratio. In addition, increase in lipid peaks and alanine can be observed at 1.5 ppm . This wa


Histopathology and immuno-histochemistry clinched the diagnosis of Pericardial Angiosarcoma. He refused further treatment.To augment our understanding of Cardiac and Pericardial Angiosarcoma and formulate a proper investigation workup plan including imaging modality of choice and multi-modality management plan.PubMed and Google were searched for primary references using the terms "Cardiac Angiosarcoma" "Pericardial Angiosarcoma" "Treatment of Angiosarcoma" and "Newer Therapies in Angiosarcoma". The Case Reports, Case Series and Review articles obtained were thoroughly reviewed.Shortness of Breath remains the most common symptom with Trans Thoracic Echocardiography as Investigation of Choice, but Computed Tomography(CT) and Magnetic Resonance (MRI) being of more yield.The disease shows a distinct male preponderance with 64.2% being male. Mean age of presentation being 44.15 ± 19.65 years. Surgical Resection - alone or in conjunction, was done in 85.7% (reviewed in Table 1), 1 patient was managed conservatively, 1 patient refused treatment. Novel treatments have shown promise demanding further researchCardiac and Pericardial angiosarcoma are rare, aggressive neoplasm often presenting late with loco regional metastasis, thus jeopardising operability and prognosis. Echocardiography is used most commonly, in addition HRCT(High Resolution CT) and MRI have been used for better cross sectional imaging. And 18 FDG PET-CT(Positron Emission Tomography - Computed Tomography) apart from being an useful adjunct, possibly would become the imaging modality of choice in coming decades. Surgical Resection - alone or in conjunction, remains the most commonly used treatment modality. Novel treatments with monoclonal antibodies and chemotherapeutic agents have shown excellent results and merit further research.Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal


Hilar cholangiocarcinoma is the most common malignant tumor affecting the extrahepatic bile duct. Surgical treatment offers the only possibility of cure, and it requires removal of all tumoral tissues with adequate resection margins. The aims of this review are to summarize the findings and to discuss the controversies on the extent of surgical resection aiming at cure for hilar cholangiocarcinoma.The English medical literatures on hilar cholangiocarcinoma were studied to review on the relevance of adequate resection margins, routine caudate lobe resection, extent of liver resection, and combined vascular resection on perioperative and long-term survival outcomes of patients with resectable hilar cholangiocarcinoma.Complete resection of tumor represents the most important prognostic factor of long-term survival for hilar cholangiocarcinoma. The primary aim of surgery is to achieve R0 resection. When R1 resection is shown intraoperatively, further resection is recommended. Combined hepatic resection is now generally accepted as a standard procedure even for Bismuth type I/II tumors. Routine caudate lobe resection is also advocated for cure. The extent of hepatic resection remains controversial. Most surgeons recommend major hepatic resection. However, minor hepatic resection has also been advocated in most patients. The decision to carry out right- or left-sided hepatectomy is made according to the predominant site of the lesion. Portal vein resection should be considered when its involvement by tumor is suspected.The curative treatment of hilar cholangiocarcinoma remains challenging. Advances in hepatobiliary techniques have improved the perioperative and long-term survival outcomes of this tumor. Hilar cholangiocarcinoma, or Klatskin tumor, is the most common malignant tumor affecting the extrahepatic bile duct. It is relatively slow growing and is usually small at clinical presentation. Only a very few patients with unresectable, indolent, and slow-growing hilar cholangiocarcinoma can have long-term survival . The proP value of <0.05 was considered statistically significant.English medical literatures were extensively searched via online pubmed using the search strategy “hilar cholangiocarcinoma OR Klatskin tumor”. Studies focusing on curative surgical resection for hilar cholangiocarcinoma were included. Case report and studies with less than 40 resections were excluded. Outcomes of surgical resection from 73 studies published during the period 1990–2014 were extracted and summarized in Table The most important factor affecting long-term survival in the surgical treatment of hilar cholangiocarcinoma is whether the tumor has been completely resected on histological examination (R0 resection). The margin status include R0 margin , R1 margin , and R2 margin . Patients with R1 margin or R2 margin have a dismal survival , 12. Of Several reports have suggested that the long-term survivals after R0 and R1 were not significantly different –16. TherIt is frequently difficult to achieve a wide and long resectional margin for curative treatment. First, hilar cholangiocarcinoma is located in the liver hilum surrounded by vital structures. Second, it is difficult to determine the exact length and width of microscopic tumor extension preoperatively and intraoperatively. The biological nature of cholangiocarcinoma involves proximal microscopic spread of the disease along the bile duct extending beyond the palpable macroscopic boundaries of the primary hilar mass. Sakamoto et al. histologically examined serial sections of 62 specimens of resected hilar cholangiocarcinoma. They found that anastomotic recurrences never occurred in patients who had a proximal tumor-free resection margin greater than 5 mm, suggesting that a 5 mm tumor-free margin was adequate for curative intent . HoweverNonetheless, to achieve real R0 resection, transection of the proximal bile duct above the macroscopic border of the primary tumor should be carried out as high as technically feasible with careful consideration of the potential morbidity, and resection of hilar cholangiocarcinoma combined with major hepatectomy has the potential to provide wide and long resection margins on the ipsilateral side of the combined liver resection .The recent reported incidences of positive resection margins in patients who had undergone surgical resection with curative intent ranged from 64.6 to 88.2 % at high-volume centers , 21–23. In the old days, hilar cholangiocarcinoma had been considered to be unresectable and palliative decompression of the obstructed biliary tract using bypass surgery or tube-drainage was popularly employed. Surgical resections with curative intent were later attempted to achieve better survival –31. BothIn 1990, a review on 581 resections for hilar bile duct cancer was published: 245 patients (42.2 %) received local bile duct confluence resection while 224 (38.6 %) patients received resection of the bile duct confluence combined with major liver resection . Tumor cOver the last two decades, complex and aggressive surgical resections were made possible with acceptable morbidity and mortality rates by major advances in patient selection, radiologic assessment, surgical techniques, and perioperative care , 36–41. Even though combined major hepatic resection is widely accepted for Bismuth type III and IV hilar cholangiocarcinoma, whether it is necessary for type I or II tumor is still controversial. A number of researchers consider that tumor resection with an adjacent cone of liver parenchyma is sufficient for patients with type I or II tumor , 52–55 eThe caudate lobe is located deep in the liver between the inferior vena cava and the hepatic hilum, thus isolation and resection of the caudate lobe remain a challenge for surgeons. The importance of tumor involvement of the caudate lobe in the treatment of hilar cholangiocarcinoma had not been fully recognized two decades ago. Some surgeons do not adopt a strategy of routine caudate lobe resection even now , 35, maiThe close anatomic relationship between the caudate lobe and the hilar cholangiocarcinoma was well studied by Mizumoto et al. in 1986 . As hilaNimura et al. reported in 1990 that 98 % of caudate lobe resections were histologically confirmed to be tumor positive . HoweverThere are still controversies on the optimal extent of hepatic resection to achieve a high percentage of R0 resection for hilar cholangiocarcinoma.Combined major liver resection represents an aggressive surgical approach to remove a large volume of hepatic parenchyma, including the use of right trisectionectomy , right hemihepatectomy , left trisectionectomy , or left hemihepatectomy (S1–4). This approach has widely been advocated as a prime choice of surgical treatment for hilar cholangiocarcinoma, especially in patients with advanced tumors –71. It iIn the late twentieth century, Nimura and Miyazaki advocated using minor central hepatic resection in carefully selected patients to preserve as much as possible the functional liver volume , 64. LimThe lack of consensus on the extent of liver resection seems to arise mainly from the difficulty in precisely determining the extent of the proximal tumor preoperatively and intraoperatively. Kawasaki et al. argued against minor central liver resection and claimed that major hepatectomy should be performed for all patients with hilar cholangiocarcinoma because of the limitations of the currently available preoperative diagnostic modalities . Some suThe decision of whether right- or left-sided hemihepatectomy is indicated is made according to the predominant site of the lesion. In general, right hemihepatectomy can be applied to type IIIa tumors and IV tumors when the lesion is predominantly located in the right hepatic duct; whereas left hemihepatectomy can be applied to type IIIb tumors and IV tumors with left-sided predominance , 84, 85.Right or extended right hemihepatectomy, the most radical surgical procedure, is routinely adopted by many surgeons on the basis of several anatomical considerations for patients with centrally situated hilar cholangiocarcinoma which can be treated by either combined right or left hemihepatectomy , 60, 86.The major drawback of the right-sided hepatectomy is loss of a large volume of liver mass. Farges et al. reported a higher mortality rate after right-sided hepatectomy than left-sided hepatectomy . Some suEven though right-sided hepatectomy is the preferred approach for hilar cholangiocarcinoma , if therShimuzu et al. performed left-sided hepatectomy for Bismuth type IIIb tumors in 88 patients and right-sided hepatectomy for type IIIa and IV tumors in 84 patients and showed equivalent operative curability and postoperative long-term survival between patients undergoing left-sided hepatectomy and right-sided hepatectomy . FurtherThe anatomical location of hilar cholangiocarcinoma is close to the portal vein bifurcation and the hepatic artery. These vascular structures are often invaded by tumor. The involvement of these vascular structures calls for combined vascular resection to achieve R0 resection. The indications for combined vascular resection include intraoperative suspicion of gross tumor invasion to the vessels , 93, 94,Concomitant hepatic arterial resection and reconstruction should be performed with caution because it may result in higher morbidity and mortality rates but without any proven survival benefit . Recent The histological involved margin status seems more important than the presence of direct invasion/involvement of portal vein for long-term overall survival . Even thThe reported rates of actual tumor invasion into the resected vessels varied from 22 to 100 % , 98–100.Treatment for hilar cholangiocarcinoma remains challenging. In order to improve surgical outcomes for patients with hilar cholangiocarcinoma, strategy of surgical resection with curative intent has improved over the years. Some consensuses have been reached, including R0 margin achievement, routine caudate lobe resection, combination of partial hepatectomy, and portal vein resection when involved by tumor. However, there are still several controversial issues need to be further clarified. Improvement of hepatobiliary imageological technique that can provide more accurate information of the tumor extent preoperatively will offer help. Due to the rarity of hilar cholangiocarcinoma, prospective randomized studies can only be carried out in multiple centers.


Skeletal muscle neoplasms, in contrast to other groups of tumors, are almost malignant. The benign variant, rhabdomyoma, is distinctly rare. Rhabdomyomas can be classified generally into two types: cardiac and extracardiac. Extracardiac rhabdomyoma can be further divided into three subtypes: adult, fetal, and genital type. Adult rhabdomyoma is the most common subtype of rhabdomyoma even though it remains relatively rare. Fetal rhabdomyomas are less common than the adult type. In this paper we report a rare case of a fetal rhabdomyoma with polyp-like appearance originating from right tonsil. Punch biopsy and then right tonsillectomy were performed for complete excision. There was no obvious recurrence. The term “rhabdomyoma” was introduced by Zenker in 1864. It was used to describe a benign tumor showing muscle cells with different degrees of differentiation and maturity. Rhabdomyomas are rare benign tumor consisting of striated muscle. They can be classified generally into two types: cardiac and extracardiac .Extracardiac rhabdomyoma can be further divided into three subtypes  with regard to clinical and morphological differences, although some overlapping exists. Genital rhabdomyoma is seen most frequently in the vagina or vulva of young and middle-aged women. Fetal and adult rhabdomyoma are located predominantly in the head and neck region; the former occurs primarily in the subcutaneous tissue of the head and neck of male infants usually younger than 3 years, and the latter is characterized by a slowly growing mass typically seen in the head and neck of elderly patient, with a male predominance. Seventy-seven percent of all extracardiac rhabdomyomas occur in the head and neck and 14% occur in the genital region [3 over right tonsil without bleeding or ulceration (A 57-year-old patient presented to our otolaryngology department on August 17, 2009, with a small painless polyp which he has had over right tonsil for one month. This patient had past history of diabetes mellitus under regular oral hypoglycemic agents control for more than 5 years. He denied any other medical or surgical history. Physical examination revealed a smooth surface polyp about 1.4 × 1.0 × 0.5 cmceration ; there wceration . IHC forceration . The difDeep resection margin could not be completely evaluated due to fragmentation of specimen. Final diagnosis was fetal rhabdomyoma. Surgical excision is the imperative treatment for rhabdomyoma, so we arranged right tonsillectomy under general anesthesia on August 31, 2009. Pathologic report showed focal granulation with chronic inflammation and reactive lymphoid hyperplasia. There was no rhabdomyoma noted on this specimen. The result indicated that polyp-appearance rhabdomyoma was excised completely when we performed punch biopsy and there was no residual rhabdomyoma on the tonsil. After operation, the patient did not come back to our department for follow-up. We contacted this patient by phone and he said he had no discomfort or abnormal lesion over right oropharynx. There was no obvious recurrence.Rhabdomyoma is an exceedingly rare soft tissue benign tumor derived from skeletal muscle. The pathogenesis is unclear. As a general rule, benign soft tissue tumors occur more frequently than their malignant counterparts, but this does not hold true for striated muscle tumors because rhabdomyomas account for only 2% of skeletal muscle tumors . Fetal rRegarding therapeutic options for this tumor, the gold standard of treatment is surgery. Rhabdomyoma may recur, but no cases of malignant transformation have been described in the literature. Recurrence is probably due to incomplete excision or multifocality. When total excision cannot be accomplished, reoperation or narrow follow-up is indicated to prevent advanced revision surgeries. In this presented case, complete excision was done, and there was no obvious recurrence.Skeletal muscle neoplasms, in contrast to other groups of tumors, are almost always malignant. The benign variant, rhabdomyoma, is distinctly rare. Our patient was a rare case of fetal rhabdomyoma located in right tonsil. Initially we thought it was a polyp over right tonsil and performed punch biopsy. Pathology report showed fetal rhabdomyoma, and curative treatment was obtained by surgery. There was no obvious recurrence.Although rhabdomyoma of tonsil is a rare disease, we should keep in mind this differential diagnosis when we meet patient with polyp over tonsil or other H&N areas. Be sure to take photodocumentation routinely before punch biopsy or excision even though it looks like benign lesion. If you meet patient with rhabdomyoma in the future, surgical excision is an imperative treatment. Recurrence rate is extremely low when complete excision is done.


Adenomatoid tumors (AT) are the most common paratesticular neoplasms and account for approximately 30% of all paratesticular masses. Most of them occur in the third or fourth decade and present as well-defined firm and painless masses. We report here a case of adenomatoid tumor from tunica albuginea. This patient is a 12-year-old boy with left testicular pain for 6 months. Scrotal ultrasonography revealed a solid mass of paratesticular origin. The histology and immunohistochemistry confirmed the final diagnosis. A right tumor resection was performed. Because of its rarity, the clinical and histopathologic appearance is seldom illustrated. Here we present a case report and a comprehensive literature review with the objective of providing useful information on this entity. Adenomatoid tumors (AT) are benign tumors most commonly occurring in the male and female genital tracts. Extragenital AT are rare and have been found in the adrenal glands, heart, mesentery, lymph nodes, and pleura . When thA 12-year-old boy presented to the andrology department in June 2013 with left testicular pain that he had had for 6 months. There was no history of recent trauma, infection, hydrocele, or undescended testis. He denied having any urinary or constitutional symptoms. Physical examination revealed a hard, tender, 10 mm × 10 mm × 8 mm testicular nodule in the superior aspect of the left testis. The contralateral testicle was normal.Scrotal ultrasonography  revealed 8 mm × 10 mm, hypoechoic homogeneous solid mass with unclear margin at the junction of the epididymis and left testis . Serum tThe patient was then referred to our institution for surgical treatment. The left inguinal approach was established and the left spermatic cord was identified. On visual inspection, the tumor arose from the tunica albuginea protruding into the testis parenchyma. Intraoperative frozen-section biopsy showed benign tumor from tunica albuginea. Subsequently, a right tumor resection including removing a portion of tunica albuginea was performed.Final histological examination confirmed the diagnosis of AT from the tunica albuginea . ImmunohAT are the most common paratesticular neoplasms and account for approximately 30% of all paratesticular masses . It was In our review, AT from tunica albuginea usually occur in the third or fourth decade and present as well-defined firm, painless masses that have been noted for several months to 1 year or discovered incidentally on physical examination. Most of them are asymptomatic and only two patients complain of dull pain of testis or scrotal discomfort . Right-sUltrasound of the scrotum reveals a well-defined interface between the tunical mass and the displaced testicular parenchyma . KolgesiThe imprint cytology and fine needle aspiration cytology (FNAC) have been applied to confirm the diagnosis . MicroscOrchiectomy has traditionally been the gold standard for the treatment of testicular tumors in children. Tumor resection appears to be the treatment of choice only when a benign lesion confirmed. But in our review, we found only one case underwent partial orchiectomy and one case performed tumor resection. The rest of cases underwent orchiectomy. Both Garg and Monappa established the diagnosis of seminoma preoperatively by FNAC , 14, whiThis case highlights the difficulty of accurately diagnosing a paratesticular mass preoperatively when the origin of the mass cannot be established before excision. We report this case for its rarity of origin and occurrence in a young boy. So this benign tumor should be borne in mind and testis should be spared as possible as we can.


Primary central nervous system lymphoma (PCNSL) is a rare disease which accounts for 1–2 % of non-Hodgkin lymphoma and 3–5 % of primary brain tumor lesions. PCNSL of an immunocompetent patient is an uncommon disease, it is estimated at 4 % of new diagnoses of CNS tumors. The prognosis of PCNSL is poor compared to other extranodal lymphomas, with a 5-year survival estimated between 20 and 40 %. PCNSL relapse occurs either in the original site but still confined to the CNS or exceptionally outside it. Brain magnetic resonance imaging, although not allowing a clear distinction between primary lesions and secondary brain lymphoma is of paramount importance not only for diagnosis but also for monitoring the patient. This manuscript report the case of a patient in whom the PCNSL has relapsed in the cervical spinal cord and also in the liver. Primary central nervous system lymphomas (PCNSLs) are rare diseases which account 1–2 % of non-Hodgkin lymphoma (NHL) and 3–5 % of primary brain tumor lesions  and cytarabine (2000 mg/m2) was administered.Chemotherapy with high-dose methotrexate (3500 mg/m2), coupled with radiotherapy was made with success but <1 month after the beginning of the treatment, the patient again presented with paresis of the right upper limb while the fronto-parietal brain injury declined. MRI of the whole spinal cord found an extending lesion from C2 to C4  was started but the patient died at the end of the second cycle of treatment.The evolution was firstly favorable but 2 months later neurologicals signs reappeared and a new MRI concluded an increase of the mass, 41 × 24 versus 32 × 18 mm at the last control). PET/CT has not found any systemic lesion. A second line of chemotherapy with ifosfamide  but also it relapse in the cervical spine and liver. PET/CT has a resolution more efficient than conventional CT for the assessment of brain lymphoma as systemic lesions are identified much earlier (Mohil et al. Although they have not been subject to confirmation by a phase III study, we opted for the first-line combination of high-dose MTX/cytarabine. In fact, these two agents have the advantage of crossing the blood–brain barrier in sufficient concentration to act on brain lymphoma lesions (Ferreri et al. PCNSL, of which the usual locations are eye, meningeal and parenchymal, is rare and has a poor prognosis. It can also relapse at a distance from the primary lesion. Systemic relapse, poorly described so far, will receive a similar treatment to that of diffuse large cell NHL.


Gestational trophoblastic tumours are a rare form of malignancy, which in the majority of cases arise from abnormal trophoblast cells formed in a complete molar pregnancy. These tumours are extremely sensitive to chemotherapy and high cure rates approaching 100% can be expected. The disease is usually limited to the uterus where the abnormal trophoblast proliferation and human chorionic production can lead to vascular changes including the formation of arteriovenous malformations.We describe the case of a 28-year-old Caucasian woman who presented to the United Kingdom's Gestational Trophoblast Tumour Service with rising human chorionic gonadotropin levels following a uterine evacuation for a complete molar pregnancy. She was commenced on chemotherapy but subsequently reported two episodes of haemoptysis. Computed tomography imaging demonstrated findings consistent with a pulmonary arteriovenous malformation, probably due to a small pulmonary metastasis, complicated by recent haemorrhage. These findings were confirmed on emergency pulmonary arteriography, and the pulmonary arteriovenous malformation was successfully embolised.Arteriovenous malformations secondary to gestational trophoblastic tumours at metastatic sites have only been reported in a very limited number of cases. When significant bleeding occurs, as in this case of a pulmonary lesion, urgent referral for embolisation is indicated. Gestational trophoblastic tumours (GTT) are characteristically highly vascular and can produce heavy or even life-threatening bleeding from the uterus or sites of metastatic disease . Over 90The genetically abnormal malignant trophoblast cells share many of the characteristics of healthy trophoblast cells, including human chorionic gonadotropin (hCG) production, rapid division, the ability to invade surrounding tissues and to stimulate angiogenesis and other vascular changes. Fortunately gestational trophoblast tumours are extremely sensitive to chemotherapy and modern cure rates approaching 100% can be expected for patients who develop GTT after a molar pregnancy .In the majority of GTT cases the disease is limited to the uterus where the abnormal trophoblast proliferation and localised hCG production may lead to focal vascular changes including the formation of arteriovenous malformations (AVMs) ,5. The dThe most common site of metastasis in GTT are the lungs, but the formation of AVMs at this or any other extra-uterine site of spread has only been recorded rarely ,7. This A 28-year-old Caucasian woman underwent a uterine evacuation for a molar pregnancy at her local hospital and was registered for follow up with the United Kingdom (UK) Gestational Trophoblast Tumour service. The histopathology review confirmed the diagnosis of a complete molar pregnancy and as such she was enrolled in the hCG surveillance programme. After an initial early fall in her serum hCG level, her hCG level rose in two consecutive samples, because of this she was reviewed in clinic 14 weeks post-evacuation prior to consideration of chemotherapy treatment.She had an unremarkable medical history, with two normal pregnancies, no major surgery or illnesses and no regular medications. The routine investigations demonstrated an hCG value of 2070IU/L, a normal chest X-ray and no visible uterine mass on the pelvic Doppler ultrasound but some increased vascularity. These results confirmed the indications for treatment and produced an International Federation of Gynecology and Obstetrics (FIGO) prognostic score of 1. Following the UK’s standard treatment protocols, chemotherapy treatment was commenced with the low-risk regimen of methotrexate and folinic acid .The patiJust prior to the embolisation procedure, her chemotherapy treatment had been changed to the etoposide, methotrexate and dactinomycin alternating with cyclophosphamide and vincristine regime as a result of a slow rate of hCG fall with methotrexate. Subsequent to this change, her hCG levels normalised 3 weeks later and after an additional 6 weeks of treatment chemotherapy was completed as shown in Figure Gestational trophoblast tumours are a rare but highly curable form of malignancy. The majority of cases occur as a result of a complete molar pregnancy and are characterised as hCG producing, highly vascular and chemosensitive tumours. The effect of the tumour-derived hCG on angiogenesis is well documented ,5 and AVGTT metastases at other anatomical sites may also be highly vascular and have the potential to form AVMs that can lead to life-threatening haemorrhage ,7. In thIn uterine AVMs with light to moderate bleeding, the usual management is to manage these expectantly as many resolve spontaneously with successful chemotherapy treatment. However, in this case with a pulmonary lesion and the potential for life-threatening bleeding, we elected to control the bleeding risk promptly by performing the embolisation.In the UK the management of patients with post-molar pregnancy GTT is centralised, which has allowed the development of expertise in these rare cases. From the almost 3000 other patients treated previously, no cases of bleeding from pAVMs have been recorded. As a result the FIGO recommendation of a chest X-ray remains the appropriate investigation for assessment of potential lung metastases in these patients . ClearlyAlthough we appreciate that pAVMs secondary to metastatic GTT are extremely rare, it is clear from this report that they are at risk of rupture causing haemoptysis. When diagnosed, we suggest that urgent referral for embolisation should be considered to reduce the risk of bleeding.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.AVM: arteriovenous malformation; CT: computed tomography; FIGO: International Federation of Gynecology and Obstetrics; GTT: gestational trophoblastic tumour; hCG: human chorionic gonadotropin; pAVM: pulmonary arteriovenous malformation; MTXFA: Methotrexate and Folnic Acid; EMA: Etoposide, Methotrexate and Dactinomycin; CO: Cyclophosphamide and Vincristine.The authors declare that they have no competing interests.All the authors have read and approved the final version of this manuscript. RD, EG, CC and PS were involved in writing the manuscript. RD and ZMB compiled the final draft of the manuscript. PS revised the manuscript critically and was a major contributor in writing the manuscript. JJ provided the radiological input for the case and prepared the images. EG, CC, JJ, MS and PS were involved in the clinical care of the patient. PS is the corresponding author.


Turner syndrome is characterized by complete or partial X-chromosome monosomy and has various clinical features, including horseshoe kidney. Leiomyosarcoma is an extremely rare tumor that accounts for only 0.1% of all invasive renal tumors.A 50-year-old Japanese woman presented at a community hospital with a chief complaint of abdominal pain. Computed tomography revealed a horseshoe kidney with a hypovascular tumor  showing calcification in the upper pole of the right kidney. Open right heminephrectomy and division of the isthmus were performed. Histological examination revealed alternating fascicles of spindle cells with blunt ended non-tapering nuclei and eosinophilic cytoplasm. The tumor had high mitotic activity with a mitotic count of 8 mitoses/10 high-power fields. On the basis of these findings, we diagnosed the patient as having leiomyosarcoma.Primary leiomyosarcoma of the horseshoe kidney in a patient with Turner syndrome is a very rare occurrence. Turner syndrome (TS) affects approximately 1 in 2,000 live-born girls. Here, wA 50-year-old Japanese woman presented at a community hospital with a chief complaint of abdominal pain. Computed tomography revealed a horseshoe kidney Figure  with a hThe patient was asymptomatic and disease free at 6 months after the diagnosis.To the best of our knowledge, this is the first reported case of primary leiomyosarcoma arising in a horseshoe kidney in a patient with TS.The risk of cancer in patients with TS has been established. The overall risk of cancer is similar to that in the general population. HoweverThe phenotype of TS is highly variable, with a wide variety of clinical conditions. Renal and/or collecting system malformations, including horseshoe kidney, renal malrotation, and collecting system malformations, have been observed in 30–40% of patients with TS. AlthougPrimary renal sarcomas are rare in adults and account for approximately 1% of all primary renal malignancies, whereas leiomyosarcoma constitute approximately 50–60% of all cases of renal sarcoma. LeiomyoA few large series on primary leiomyosarcoma of the kidney with an analysis of the prognosis have been reported. Kendal analyzed the population-based surveillance, epidemiology, and end results (SEER) registry and reported on the survival of 112 patients with renal leiomyosarcomas. On the In conclusion, primary leiomyosarcoma of the horseshoe kidney in a patient with TS is a very rare occurrence. It is important to remember that this unusual variant of renal leiomyosarcoma has the potential for aggressive behavior, which can lead to metastasis.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.TS: Turner syndrome; RCC: Renal cell carcinoma; SEER: Surveillance, epidemiology, and end results; OS: Overall survival.The authors declare that they have no competing interests.YT and TK were involved in drafting the manuscript. TK performed the clinical follow-up and contributed to manuscript preparation. KO and II performed the surgery. TK and CO were responsible for the concept and design of this study, interpretation of the data, and critical revision of the manuscript. All authors read and approved the final manuscript.


We report a case of adrenal angiomyolipoma showing a significantly high uptake of 18F-fluorodeoxyglucose on PET/CT study. The maximal standardized uptake value (SUV) of the lesion was 18.8.Adrenal angiomyolipoma is an extremely rare tumor, although computed tomography (CT) or magnetic resonance imaging findings of adrenal angiomyolipoma have been reported, there are no reports regarding integrated fluorine-18-fluorodeoxyglucose positron emission tomography and computed tomography (Adrenal angiomyolipoma can show an intense uptake in FDG-PET/CT, and this can easily be confused with a malignant disease. Adrenal angiomyolipoma should be considered as one of the differential diagnoses in cases of adrenal incidentaloma with intense FDG uptake. Adrenal masses are seen in a wide variety of conditions, including benign lesions such as pheochromocytoma adrenal tuberculosis granulomatous infections and malignant diseases, and primary adrenocortical carcinoma and especially adrenal metastasis. Since adrenal metastasis may be encountered in a patient with either a known or an unknown primary malignancy, high uptake in an adrenal mass on fluorine-18-fluorodeoxyglucose positron emission tomography and computed tomography (18F-FDG PET/CT) is thought to represent malignancy. We present a case of adrenal angiomyolipoma that showed intense FDG uptake, and a review of the literature.Specifically, a 53-year-old male patient was admitted to our institution on December 03, 2013, with a 6 months history of abdominal pain in the upper left abdomen.The patient's vital signs were stable at admission, and physical examination showed weakness and tenderness in the upper left abdominal quadrant.Abdominal ultrasonography revealed a well-defined 9 × 6 cm mass in the left adrenal gland. Baseline hematological, and biochemical investigations and urinalysis were normal. Laboratory investigations of serum cortisol, aldosterone, catecholamine, plasma adrenocorticotropic hormone, vanilmandelic acid, 24 hours urinary free cortisol, and a test for low-dose dexamethasone suppression were also within normal limits. On enhanced CT imaging, a low density mass was noted in the left adrenal gland with loss of fat plane between the mass and the left kidney Figure A. On magLaparoscopic adrenalectomy with adrenal mass resection was performed after having provided informed consent with the patient. The histopathological features Figure  confirme8 In our specific reported case, the tumor size is 9 × 6 cm, and is the first reported case using combined 18F-FDG PET/CT imaging. The typical lesion of angiomyolipoma is composed of an admixture of thick-walled blood vessels, bundles of smooth muscle, and mature adipose tissues. Largely through the research of a group of pathologists, in recent years, it has become evident that the most distinctive component of this tumor is immunoreactivity for HMB-45.9 Up to 52% of patients with angiomyolipomas larger than 4 cm were symptomatic and had an increased risk of spontaneous rupture and bleeding. In addition, it has been suggested that in large angiomyolipomas, the risk of malignancy increases with the size of the tumor, following surgery or selective arterial embolization.8 In recent years, laparoscopic adrenalectomy has been recommended with lower mortality as compared with open surgery.Angiomyolipomas belong to the group of tumors that exhibit a diverse appearance known as tumors of perivascular epitheloid cell origin. Angiomyolipomas are rare lesions, and most occur in the kidney. The next common site is the liver. Adrenalangiomyolipoma is an extremely rare tumor, and to our knowledge only 10 cases have been reported, including the present case.In conclusion, adrenal angiomyolipomas can show an intense uptake in FDG-PET/CT, and can easily be confused with a malignant disease. Adrenal angiomyolipoma should be considered as one of the differential diagnoses in cases of adrenal incidentaloma with intense FDG uptake.


We share our experience with serial PET/CT imaging on a patient with extranodal Rosai-Dorfman disease (RDD) with hepatopancreatic involvement. RDD is a benign proliferative disorder of histiocytes mainly involving the lymph nodes. It typically presents with fever and painless cervical lymphadenopathy in young adults and less than half of RDS cases demonstrate extranodal involvement. RDD involvement of the liver and pancreas is extremely rare, and this case highlights the role of PET/CT in its management. Rosai-Dorfman syndrome (RDS) is a rare histiocytic proliferative disorder commonly involving the lymph nodes and presenting with non-specific symptoms, such as fever and elevated erythrocyte sedimentation rate (ESR). Less than half of the cases demonstrate extranodal involvement, with symptomology reflecting the afflicted organ-systems. Hepatopancreatic involvement of RDD is extremely rare, and our case highlights the role of FDG PET/CT in its detection of management.A 59-year-old female presented with abdominal pain, several episodes of syncope, and uncontrolled hyperglycemia. Her initial workup revealed findings suggestive of acute pancreatitis, and initial imaging was suggestive of a pancreatic mass. Informed patient consent was obtained for her treatment. She was taken to the operating room for a Whipple's procedure, and the surgical pathology revealed Rosai-Dorfman disease.A postoperative computed tomography (CT) scan was performed, which demonstrated no evidence of extrapancreatic involvement or lymphadenopathy Figure . At thatHigh-dose intravenous steroid therapy was initiated, and the patient was brought back for follow-up. A subsequent PET/CT was performed, which demonstrated a new focus of increased FDG uptake in the left hepatic lobe, in addition to the known pancreatic body/tail lesion Figure . This waRosai-Dorfman disease (RDD) is a rare, benign histiocytic proliferative disorder of unknown etiology . It commCutaneous RDD typically presents with papules and plaques that can grow to form nodules with satellite lesions that resolve into fibrotic plaques before spontaneous regression . HistoloGastrointestinal localization of RDD, especially in the liver and pancreas, is extremely rare. Typically, the liver is affected as a part of the systematic spread of RDD with nodal and wide extranodal involvement as demonstrated by Lauwers et al. -8. PancrBecause the etiology of RDD is not well understood, the treatment is still nonspecific and controversial. Treatment is recommended only in patients who are symptomatic or have vital organ or systemic involvement, as RDD is reported to be self-limited in approximately 20% of the cases . Complet The findings of the latter were suggestive of failure of the steroid therapy to regress or halt the progression of the disease.Like various other lymphoproliferative disorders, benign and malignant alike, RDD lesions have been shown to be FDG-avid . There hThis case demonstrates the role of PET-CT imaging in the detection of RDD with visceral involvement based on the FDG avidity attributable to the infiltrative and inflammatory component of the disease process. Furthermore, it suggests the usefulness of this modality in the assessment for disease progression to other extranodal sites when used for therapy response assessment.


Lipomas are the slow growing soft tissue tumors of benign nature. They commonly grow on torso and extremities but may also develop in head and neck region. Rarely lipomas can grow to acquire gigantic proportions, turning into an entity termed as giant lipoma. Such lipomas are entitled to immediate attention as they have a relatively high malignant potential. We report a rare case of giant cervical lipoma in an elderly gentleman, followed by a brief discussion on diagnosis and management of the disorder. Lipoma is a slow growing benign tumor of adipose tissue and can occur anywhere in the body. Hence, it is also known as universal or ubiquitous tumor . UsuallyA 62-years-old gentleman reported in surgical OPD with a giant lump hanging from the left side of neck. The swelling was present for the past 20 years. It was insidious in onset and was of small size when he noticed it first. It gradually increased in size to attain the present gigantic proportions. There was no history of pain and sudden change in size of swelling. There was not any history of fever, loss of appetite, loss of weight, difficulty in swallowing, difficulty in breathing, upper limb weakness, or voice change. Personal and family histories were unremarkable. General and systemic examinations were unremarkable. Upon local examination, a huge lump was evident hanging upon anterior aspect of the left side of chest. It was approximately 38 cm × 18 cm × 15 cm in dimensions and was extending from the left ramus of mandible to the left hypochondrium . Its rooRoutine investigations were unremarkable. FNAC from the swelling was suggestive of a benign fibrolipoma. A contrast enhanced CT scan of neck and thorax region was obtained to rule out any suspicious foci of malignant transformation in the mass. The mass however turned out to be essentially benign and showed no infiltration of surrounding vital structures in the neck. The patient was taken up for surgery after proper preanesthetic checkup. The mass was excised completely through an elliptical incision given below the left mandibular ramus and sent for histopathological examination . PostopeLipomas are typically slow growing tumors and only a few grow into the massive lump known as giant lipoma . MechaniThe treatment of choice for the lipoma remains open surgical excision . It is a