Facial swelling is commonly encountered in paediatric patients and is typically related to an underlying infection. The spectrum of possible causes, however, is wide, and includes traumatic, inflammatory, nutritional and neoplastic aetiologies. In this pictorial essay we present 13 examples of rare conditions selected from a total of 136 MRI examinations performed at our institution between April 2007 and May 2013. These include HIV-associated malignancies such as a case of plasmablastic lymphoma, parotid gland tumours including a parotid hamartoma, rare congenital lesions such as a thyroid fetiform teratoma, and infective lesions including tuberculosis of the mandible. In many cases, only minimal information could be gleaned from the literature, particularly with regard to imaging findings. An analysis of the spectrum of masses and specific clinical presentations allowed for the construction of a diagnostic flowchart which may serve to assist in unusual cases.Facial swelling is commonly encountered in paediatrics, with a wide spectrum of possible aetiologies.• MRI is the favoured imaging modality for accurate assessment.• Facial swelling is typically infectious in nature, but includes various benign and malignant causes.• This pictorial essay presents 13 examples of rare conditions with corresponding imaging.•  Facial swelling is a common clinical scenario in the paediatric setting, associated with a diverse range of possible causes. The underlying aetiology ranges from congenital lesions to infective and inflammatory conditions, and various other benign or malignant masses .Advances in imaging techniques have led to the ever-increasing use of computed tomography (CT) and magnetic resonance imaging (MRI) for evaluating the extent of disease and for treatment planning .In the case of facial swelling associated with a systemic illness, poor response to antibiotic treatment, or a clinical suspicion of malignancy, cross-sectional imaging should be considered .At our institution, MRI is favoured for the assessment of paediatric facial masses, with the advantages of superior soft tissue resolution and a lack of ionizing radiation.There are several key clinical features that should be taken into account when interpreting this type of imaging, including the age of the child, the location of the mass, the duration and nature of onset, and any underlying condition associated with the development of neoplasms .The most commonly encountered facial masses in the paediatric population are infective in nature, typically due to lymphadenitis, sinusitis, or a dental infection , 2.Congenital masses tend to be non-progressive; examples include fronto-ethmoidal cephaloceles, nasal gliomas, and nasal dermoid and epidermoid cysts , 2.Rapidly progressive masses include malignancies, with rhabdomyosarcoma and lymphoma being the most common, in addition to osteogenic sarcoma, Langerhans cell histiocytosis (LCH), Ewing’s sarcoma, and metastatic neuroblastoma , 4.Although several reviews of the common causes of facial masses can be found in the literature, there is relatively little on the spectrum of more unusual causes such as salivary gland tumours and teratomas .Our series includes 13 examples of rare, biopsy-proven conditions  is an aggressive malignant soft tissue neoplasm of skeletal muscle origin which accounts for up to 8 % of all malignancies in children under 15 years of age. Neonatal presentation of this tumour, however, is rare .A large cohort analysed by the IRS group showed that only 0.4 % presented before the age of 1 month .The diagnosis of congenital RMS suggests the possible intrauterine development of this tumour, and the head, neck, and trunk are the most commonly affected sites .On MRI, the tumours are typically hypointense to skeletal muscle on T1-weighted (T1-W) and hyperintense on T2-weighted (T2-W) imaging, enhance heterogeneously post-contrast, and demonstrate prominent vascularity .Our MRI, performed when the baby was 5 days old, showed a 10 × 10.5 cm heterogeneous mass centred within the left parapharyngeal soft tissues. The tumour extended from the middle cranial fossa to the level of C4 inferiorly. There was associated destruction of the ramus of the left mandible and left lateral orbital wall. On the post-contrast images, there was avid enhancement with a large area of necrosis centrally Fig. .Hamartomas of the parotid gland are exceedingly rare, with only four cases having been described in the English language literature .These benign tumour-like lesions arise during the development phase of an organ or tissue, and consist of a disorganized growth of differentiated mature tissues indigenous to that particular anatomic location .No description of imaging findings of this rare lesion could be located in the literature.In our example, the left parotid gland was expanded and almost entirely replaced by an avidly enhancing, heterogeneous mixed solid and cystic lesion Fig. .Tuberculosis (TB) is a chronic granulomatous disease caused by Mycobacterium tuberculosis. Although the chest is most frequently affected, any organ system may be involved, particularly in immunocompromised individuals .Musculoskeletal tuberculosis, however, accounts for only 1–3 % of cases , with TBRadiologically, tuberculous osteomyelitis typically appears as a unilocular destructive bone lesion with an associated periosteal reaction .Our MRI showed a destructive bone lesion of the right mandible involving the ramus and coracoid process, with a periosteal reaction and associated heterogeneously enhancing soft tissue mass. Characteristic of TB were large bilateral carotid space nodes which were predominantly T2 hypointense, with rim enhancement and central necrosis Fig. .Pilomatricoma is a benign skin neoplasm derived from hair follicle matrix cells  and is cClinically, pilomatricomas often present as a solitary, superficial, rock-hard mass—a pathognomonic feature—with the overlying skin occasionally demonstrating a bluish discolouration , 17. AltPilomatricomas occur most commonly in the first and second decades of life, predominantly in female patients—as was our patient .Despite a lack of agreement regarding imaging features, pilomatricoma has been described as having a uniform intermediate signal on T1, heterogeneous signal on T2, and internal reticulations on contrast-enhanced T1-W images corresponding to oedematous stroma . PeripheOur MRI showed a 23 × 12 mm T1 and T2 hypointense subcutaneous nodule, with a well-defined T2 hyperintense rim. On the post-contrast images, there was both rim enhancement and diffuse reticular enhancement centrally Fig. .Central giant cell granulomas (CGCGs) have been described as uncommon benign bone lesions, typically affecting the mandible and maxilla . There iThere is a wide age range affected, but the majority occur in patients younger than 30 years, and there is a 2:1 female predilection . Our femThe lesions exhibit a wide spectrum of clinical behaviour, ranging from a painless, slowly-progressive swelling, to a larger, locally-destructive ‘aggressive’ lesion with a higher rate of recurrence following excision and curettage .Similarly, there is considerable variation in the imaging findings. The spectrum ranges from a small unilocular lesion, to a much larger lesion with ill-defined margins, multiple loculations, and associated tooth displacement or root resorption .Our imaging revealed a well-corticated, multi-cystic, expansile mandibular lesion, with solid components which enhanced avidly post-contrast Fig. .A schwannoma (neurilemmoma) is a benign, slow-growing, encapsulated perineural tumour arising from nerve sheath Schwann cells .Schwannomas occurring in the head and neck region are not unusual, but those originating in the maxillary sinuses are rare, with only seven cases to date having been reported in the literature , 21.No case occurring in a child could be found in the literature, with the youngest reported case in a patient 17 years of age [Clinically, the lesions are typically asymptomatic, but may present with pain, an enlarging mass, or proptosis , as was No description of typical MRI features could be found.Our case demonstrated a lesion with heterogeneous hypointense signal on T2, implying high cellularity. The mass was iso-intense to grey matter on T1 and enhanced post-contrast Fig. .Infantile fibrosarcoma is a rare soft tissue neoplasm diagnosed at birth or soon afterwards.These tumours typically affect the distal extremities, with only 16 % occurring in the head and neck region , and areThe lesions may become disproportionately large relative to the size of the child, and tumours as large as 30 cm have been reported .On MRI there is typically a heterogeneously enhancing soft tissue mass containing focal areas of hypo-enhancement due to haemorrhage or necrosis .In our example, MRI showed a left subcutaneous masticator space lesion which was avidly enhancing and contained foci of low signal consistent with central necrosis.There were no significant intralesional flow voids to suggest a vascular malformation Fig. .Rhabdomyosarcomas (RMSs)—which originate from primitive mesenchymal tissue—constitute between 3 and 5 % of childhood malignancies . The regBased on their location in the head and neck region, they may be classified as 1) orbital, 2) parameningeal, or 3) non orbital-non parameningeal . The parThe clinical and biological behaviour of RMSs varies widely with their appearance, ranging from a small cutaneous facial nodule to an extensive rapidly progressive facial swelling , as was Our patient, a 4-year-old child, presented with an apparent history of progressive facial swelling over the previous month. On examination, there was a massive right-sided exophytic tumour with a destroyed right eye.MRI showed a 12 × 10 cm aggressive lobulated heterogeneous right facial mass extending from the base of the skull to the inferior margin of the mandible, with invasion of the infratemporal fossa and destruction of the right globe Fig. .Yolk sac tumours (YSTs) are malignant neoplasms of germ cell origin, usually occurring in the ovary or testes. Extragonadal YSTs are uncommon and most often seen in the sacrococcygeal, mediastinal, intracranial, and retroperitoneal regions. Extracranial head and neck YSTs are exceedingly rare .YSTs primarily affect neonates and infants, and often occur in conjunction with other germ cell tumours, most commonly teratomas .In the head and neck, extracranial YSTs may involve the orbit, maxillary sinus, retroauricular region, oral cavity, nasopharynx, submandibular gland, and parotid gland .YSTs tend to be very aggressive and have early metastatic potential, usually involving the lungs, lymph nodes, liver, and bones .MRI features have been described as a well-defined hyperintense tumour .In our case, MRI revealed a large well-circumscribed left cervical mass centred in the parapharyngeal space, extending from the skull base to the level of the thoracic inlet, with associated right cervical adenopathy. The mass was predominantly hyperintense on T2 and demonstrated heterogeneous post-contrast enhancement Fig. .Plasmablastic lymphoma (PBL) has been classified by the WHO as a new clinical entity, and characterised as an aggressive, invariably fatal, subtype of non-Hodgkin’s lymphoma, typically occurring in HIV-infected patients , 28.Epstein–Barr virus and Kaposi sarcoma-associated human herpesvirus-8 are also thought to play a role in the pathogenesis of PBL .PBL is a rare phenomenon in children, with only 10 cases reported in the literature –31. Our Although originally described as a disease arising in the oral cavity of immune-deficient patients, subsequent cases involving extraoral sites such as the maxillary sinus, nasopharynx, lung, skin, anus, and spermatic cord have been reported .A description of the typical imaging features could not be found in the literature. Our MRI showed multiple bilateral lobulated facial masses which were T2 hypointense with multi-septated rim enhancement.Mortality at 1 year is said to be approximately 60 %, although the combination of highly active antiretroviral therapy (HAART) and chemotherapy may significantly improve the prognosis . At the Burkitt lymphoma—an undifferentiated non-Hodgkin lymphoma—is an AIDS-defining illness and a commonly encountered AIDS-related malignancy .Epstein–Barr virus has been associated with approximately half of AIDS-related Burkitt lymphoma cases .In a study comparing HIV-positive children admitted with malignancy to HIV-negative children with malignancy, Burkitt lymphoma was found to occur 7.2 times as frequently in the HIV-positive group .Although HIV-associated Burkitt lymphoma typically involves the abdominal organs and bone marrow, our patient presented with a left-sided mandibular mass.MRI revealed a T1 and T2 iso-intense subcutaneous mass posterior to the left mandibular angle, with heterogeneous enhancement post-contrast and associated posterior triangle lymphadenopathy Fig. .Malignant parotid gland tumours are very rarely observed in children, particularly those under 10 years of age , 35.A review of 122 paediatric patients with salivary gland tumours found only 17 to be malignant. All 17 occurred between the ages of 11 and 18 , while oAccording to Belghiti et al., fewer than 19 cases of malignant parotid gland tumours in children have been published, with mucoepidermoid carcinoma accounting for approximately one third of these .Although a clear link between mucoepidermoid carcinoma and HIV has yet to be established, Serraino et al. described an increased age-standardised incidence of salivary gland cancer in HIV-positive adult men compared with HIV-negative men .Imaging appearance is linked to histological grade, ranging from a well-circumscribed heterogeneous parotid space mass to a more invasive, ill-defined tumour with associated lymphadenopathy .In our example, the left intra-parotid mass was well-defined, hyperintense to muscle on T2, iso- to hyperintense on T1, and enhanced avidly post-contrast. There were intratumoral cysts and necrosis as well as bilateral cervical and posterior triangle lymphadenopathy Fig. .Although infective causes account for the majority of cases of paediatric facial masses or mass-like lesions, there is a much wider spectrum of more unusual aetiologies, which have been highlighted in this pictorial essay, and to which radiologists should be alerted in order that appropriate treatment is not delayed.


Ewing sarcoma of the abdomen is a rare entity in pediatric oncology and represents a technical challenge both for surgeons and radiation oncologists. We document the case of a young female patient with primary disseminated, intraperitoneal Ewing sarcoma who after an excellent response to chemotherapy received preoperative whole abdominal-pelvic radiotherapy with good tolerance. Ewing sarcoma is most commonly a bone tumor and involvement of the peritoneum has been rarely described. Peritoneal dissemination may occur via metastatic spread from the primary tumor, tumor spillage during surgical resection, or primary peritoneal disease with local spread . In osseA 16-year-old female patient presented in May 2015 with dyspnea and a mass in the periumbilical area. An ultrasound-guided biopsy of a peritoneal implant confirmed a diagnosis of Ewing sarcoma, with a FLI-EWSR1 translocation. Computed tomography (CT) and Positron Emission Tomography (PET) staging identified multiple areas of increased metabolic activity in the abdomen and pelvis (maximum SUV 11.6) along with bilateral pleural effusions  alternating with Ifosfamide / VP16 chemotherapy (IE) on a two-week interval schedule, and completed her 8th cycle of treatment with good tolerance apart from expected hematologic toxicity, weight loss (<10% body weight), mild neuropathy, GERD, and constipation. She did not require unscheduled hospitalizations.Repeat CT and PET scans after six cycles of chemotherapy in September 2015 showed an excellent radiologic and metabolic response. There were only two residual masses: one in the right flank that measured 4.5 cm in greatest dimension with an SUV of 2.3, and a second in the pelvis that measured 10.7 cm in greatest dimension with an SUV of < 2  and that her abdominal craniocaudal extent was 53 cm, her treatment plan was a technical challenge. It became apparent that field junctioning with a linear-accelerator based treatment would be required. Another challenge was to protect her kidneys while maintaining coverage to the entire intraperitoneal cavity since renal tolerance is a known limiting constraint [The patient underwent WAP-RT with concurrent and subsequent boosts to the two residual, metabolically active lesions. Given that the patient measured 1.82 m  daily kilovoltage cone-beam computer tomography scans (CBCT) were performed for image guidance on a TrueBeam linear accelerator  Figure . Target Toxicity was analyzed at least once weekly during RT. Treatment was well tolerated and delivered without interruptions. As per the National Cancer Institute Common Terminology Criteria for Adverse Events v4.3 (CTCAE) [All the residual masses were removed. The peritoneal lavage cytology was negative for malignant cells and revealed only chronic inflammatory and reactive cells.There are few studies reporting the treatment of peritoneal Ewing sarcoma ,5-7. In For example, Ahmad et al., based on their review of twenty-four patients with ESE, concluded that wide resection was a major determinant factor that impacted overall survival . The quaKinsella et al.  analyzedIn our patient, we used preoperative chemoradiotherapy with the goal of maximizing the chance of achieving a complete resection. An IG-IMRT technique confirmed on a daily basis the accuracy of treatment.We present a possible approach for disseminated, intra-abdominal Ewing sarcoma that includes whole abdominal-pelvic radiotherapy. Our patient was treated to a dose of 24 Gy plus a boost to residual masses; the acute toxicity was acceptable and was followed by complete resection without complication.


A 10 year old girl, without any particular pathological history, presented with complaints of swelling in the left side of abdomen increasing very slowly in size since the age of 3 years. Physical examination objectified left parietal bulk, compressible, by 5 cm long axis without signs of acute inflammation. Abdominal US showed a left paramedian parietal mass sub umbilical developed at the expense of the rectus abdominis muscle. This mass was well limited, measuring 57×41 mm, hypoechoic, micro cystic with many thin septa. She arrived deeply in contact with bowel loops and iliac vessels.Cystic lymphangioma is a rare malformative benign tumor of the lymphatic vessels. It occurs most commonly in the neck. Rarely they are known to involve the axilla, groin, mediastinum, retroperitoneum, pelvis, mesentery, omentum and spleen Further exploration by MRI showed a polylobed mass, developed at the expense of the rectus abdominis muscle. It extended to the large muscles of the ipsilateral side, with a hypo-signal T1 and hyper-signal T2. This formation was responsible for ascension of small bowel and came in contact with the external iliac pedicle, the left psoas muscle and the sigmoid without invasion. The MRI appearance was compatible with a cystic lymphangioma. Per operatively, the cystic mass was attached at the rectus abdominis muscle . Entire  Histological examination revealed an intramuscular cystic lymphangioma extending to the peritoneum. The postoperative course was uneventful. Subsequent control after 10 months showed a child in remission.[2]. Most of the cystic lymphangiomas are discovered during childhood; however, their nascent presentation in adults is also reported[3]. Cystic lymphangioma is a rare congenital malformation. The failure of connection or separation between the lymphatic and venous system and the proliferation of abnormal lymphatic tissue are two theories most implicated in its developmentlocation, extremely scarse, was described both in adults[3] and children[4] but not in the rectus abdominis muscle. The clinical presentation of cystic lymphangioma is polymorphic, unspecific and variable depending on the location and extent. It can manifest anywhere in the body. The common locations are cervico-facial regions. Muscle [3]. Imaging in our case was highly suggestive of the diagnosis. US[5] and MRI are excellent ways for diagnosis of cystic lymphangiomas in several locations. US usually shows multicystic lesion with internal septations and no blood flow is detected on color doppler ultrasonographs. Other modalities like CT scan and MRI can be employed to delineate the lesion, in a better way. A CT scan demonstrates multicystic, homogeneous, non-invasive density with low attenuation. These modalities are usually helpful in ascertaining the extent of the lesion and their association with nerves and vessels and are particularly useful, when surgical management of the lesion is contemplated[2]. In front of a cystic mass localized in the muscle several diagnostics can be evoked, such as hematoma or cystic muscle remodeling especially following trauma or tumor lesions or muscle abscess[5]. MRI of lymphangioma usually shows a multiloculated heterogeneous mass with low signal intensity on T1-weighted images, and high signal intensity on T2-weighted images because of its content. The authors' experience suggests that most lymphangiomas have a characteristic appearance on MRI. The information obtained with MRI can help in providing a preoperative diagnosis, in planning surgical resection, and in defining recurrence. Sclerotherapy presents an interesting therapeutic method in invasive forms[1]. In our patient complete resection was performed. The anatomopathologic study confirmed the diagnosis. Postoperative course was simple. A total surgical excision, if feasible without a major sacrifice of adjacent organs, can be the best therapeutic option The presentation of this case draws attention to the possibility of occurrence of such an etiologic lesion in rectus abdominis muscle. We stress the necessity of imaging as a diagnostic tool for this location.


Carcinoid tumors are the most common type of small bowel tumor, and the incidence is rising. The majority of small bowel carcinoid tumors arise within 60cm of the ileocecal valve. The addition of ileoscopy to screening colonoscopy can detect asymptomatic small bowel carcinoid tumors and improve long-term prognosis through early surgical resection. Ileoscopy is a brief procedure with a high success rate and minimal complications beyond those of colonoscopy. The use of ileoscopy during screening colonoscopy has led to an increase in the early-stage detection of locoregional small bowel carcinoid tumors that can be completely treated with surgery alone, and as such has improved long-term prognosis in these patients.Five asymptomatic Caucasian patients, 3 males and 2 females, from 53 to 70 years old , were diagnosed with locoregional ileal carcinoids during routine colonoscopy with ileoscopy. Since having an ileocolectomy and without adjuvant treatment, no patient has developed tumor recurrence over a follow-up period of one and half to 12 and a half years.The early detection of carcinoid tumors by ileoscopy during screening colonoscopy can lead to increased long-term survival in patients with locally invasive disease. The high success rate and brief duration of the procedure, in addition to the lack of associated morbidity and mortality suggest that with further studies, routine ileoscopy during colonoscopy may be promising in the diagnosis of small bowel carcinoid tumors. Carcinoid tumor is the most common tumor of the small bowel, and its incidence is rising . Patientet al.[et al.[Recent case series by Yarze et al. and Lee [et al. have shoWe document the cases of five patients aged 53 to 70 years old  diagnosed with SBCTs during screening colonoscopy with ileoscopy. All patients demonstrated localized lymph node metastases on ileocolectomy, but no distant metastases by computed tomography. No patient received adjuvant therapy. To date, no patient has experienced a recurrence of the carcinoid tumor or metastatic disease.A 65-year-old asymptomatic Caucasian man presented for screening colonoscopy with ileoscopy that revealed a terminal ileum lesion, which proved to be a SBCT. He underwent an open ileocolectomy, which revealed a 2.7 cm by 2.0cm malignant carcinoid tumor with non-contiguous mesenteric metastasis. No lymphadenopathy greater than 1 cm was found, however four out of ten mesenteric lymph nodes were positive for metastases. His cecum and Gerota’s fascia were also involved in the tumor and removed. On long-term follow-up, our patient remains asymptomatic with no evidence of disease 12.5 years after surgery.A 53-year-old asymptomatic Caucasian man was diagnosed with SBCTs during screening colonoscopy with ileoscopy that revealed a protuberant ileocecal valve mass. He underwent an open ileocolectomy that revealed a 2.5 cm by 2.2 cm ulcerated carcinoid tumor of the distal ileum prolapsing through the ileocecal valve, with four mesenteric lymph nodes positive for metastases. On long-term follow-up, our patient remains asymptomatic and free of disease 11 years after surgery.A 50-year-old asymptomatic Caucasian woman presented for screening colonoscopy because of a family history of colon cancer. A submucosal tumor of greater than 2 cm was noted in her ileum, found to be a carcinoid tumor. An open ileocolectomy revealed a 0.7cm carcinoid tumor with submucosal involvement and three out twenty four lymph nodes positive for metastases. On long-term follow-up, our patient remains asymptomatic 11 years after surgery.A 65-year-old asymptomatic Caucasian man presented for screening colonoscopy with ileoscopy that revealed a SBCT in his terminal ileum. He underwent an open ileocolectomy, which demonstrated a 2 cm SBCT of the ileum with three out of thirty eight mesenteric lymph nodes positive for metastases. Our patient demonstrated no evidence of carcinoid tumor disease until his death 7 years after surgery, from small cell lung cancer.A 62-year-old asymptomatic Caucasian woman presented for screening colonoscopy with ileoscopy. The ileoscopy revealed a biopsy-proven ileal carcinoid tumor (8mm), which was partially resected by endoscopy. A laparoscopic ileocolectomy was performed to completely excise the tumor, and revealed a 3.8 mm by 2 mm SBCT with tumor invasion into the submucosa. five out of fourteen mesenteric lymph nodes were positive for metastases. On follow-up our patient remains asymptomatic one and a half years after surgery.Our case series supports routine ileal intubation at the time of screening colonoscopy to facilitate early diagnosis of SBCTs involving the terminal ileum, which ultimately will improve long-term prognosis. Our five patients diagnosed with regional ileal carcinoids all underwent successful surgical treatment as the sole therapy. All patients showed no evidence of disease one and a half to twelve and a half years after surgery, with one patient who died of an unrelated malignancy.et al. found that as small bowel carcinoids spread from local to regional to distant metastases, the five-year survival rates decrease from 100% to 86% to 51% respectively [As SBCTs spread undetected, patient prognosis worsens. Landerholm ectively . The oveectively , thus ouBecause the diagnostic yield of ileoscopy in asymptomatic patients undergoing screening colonoscopy is low, some authors argue against its routine use . These det al.[et al.[Similar previously mentioned case series by Yarze et al. and Lee [et al. also supEarly detection through screening colonoscopy with ileoscopy, and subsequent early surgical intervention of SBCTs in this case series likely contributed to improved long-term morbidity and mortality. Given the increasing incidence of SBCTs in populations undergoing screening colonoscopy, and the high success rate with minimal increase in procedural time or complication rate, ileoscopy shows promise as a worthwhile addition to standard screening colonoscopy practice. While further research is warranted, the post-surgical asymptomatic survival seen in the patients of this case series suggests that there may be long-term benefits to routine ileoscopy during screening colonoscopy.Written informed consent was obtained from the patients, and for the next of kin in case 4, for publication of this case series and any accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


Retroperitoneal fibrosis is a rare fibrosing reactive process that may be confused with mesenteric fibromatosis. Abdominal aorta aneurysm is rare too and mostly develops secondary to Behcet's disease, trauma, and infection or connective tissue diseases. Incidence of aneurysms occurring as a result of atherosclerotic changes increases in postmenopausal period. Diagnosis can be established with arteriography, tomography, or magnetic resonance imaging associated with clinical findings. Tumors and cysts should be considered in differential diagnosis. Abdominal ultrasound and contrast-enhanced computerized tomography revealed an infrarenal abdominal aorta aneurysm in a 41-year-old woman, but, on surgery, retroperitoneal fibrosis surrounding the aorta was detected. We present this interesting case because retroperitoneal fibrosis encircling the abdominal aorta can mimic abdominal aorta aneurysm radiologically. Retroperitoneal fibrosis is a rare reactive process. It is two to three times more common in men, and most patients present with it during the fifth or sixth decade of life . It is uAbdominal aorta aneurysms (AAAs) are vascular pathologies that mostly develop in advanced-age group due to atherosclerosis. AAAs have a male predominance. But following menopause, female/male ratio becomes equal. Aneurysms become symptomatic after they reach a certain diameter. The diagnosis is established radiologically. But sometimes, benign or malignant lesions may mimic AAAs by surrounding the aorta. Herein, we report a 41-year-old woman who had complaints of abdominal and back pain and was diagnosed as AAA on CT and USG. But on surgery, it was seen that the periaortic thickening was due to retroperitoneal fibrosis. Therefore, we aimed to present this rare case and stress that retroperitoneal fibromatosis should be kept in mind in the differential diagnosis of AAA.3, hematocrit: 42.4%, glucose: 96 mg/dL, and BUN: 17 mg/dL. Her family history was unremarkable.A 41-year-old woman was admitted to the cardiology clinic because of abdominal and back pain. Physical examination revealed a mass in the subxyphoideal region. An abdominal aorta aneurysm with thrombosis was detected on abdominal ultrasound. CT was obtained for showing the extent of the lesion. CT showed an infrarenal aneurysm  that extSurgical operation was planned. A midline incision was made to enter abdomen. Intra-abdominal organs were closed by towel dressings to provide the exposure needed. A mass encircling the aorta was encountered. Posterior peritoneum and neighboring structures were firmly adhered to the mass. Complete resection was impossible because the mass was surrounding the aorta and neighboring structures. A diagnostic biopsy was taken from the mass. Intraoperative pathological evaluation of the specimen was considered as fibromatosis, and the operation was terminated. Histopathology of the specimen was also reported as fibromatosis . The patRetroperitoneal fibrosis is quite uncommon, with an incidence estimated to be less than 1 per 10.000 patients . It is cRadiologic imaging methods such as ultrasound, computerized tomography, magnetic resonance imaging, and arteriography decreased the probability of misdiagnosis of vascular pathologies. But still misdiagnosis may occur. Fibromatosis surrounding the aorta is one of the most important pathologies that should be included in the differential diagnosis of AAA cases. In our case, retroperitoneal fibromatosis was mimicking abdominal aorta aneurysm in localization, and symptoms and abdominal CT and ultrasonographic appearance were consistent with abdominal aneurysm. However, when we think retrospectively, being 41 years old, having no exact etiologic cause, and having a homogenous appearance all around could be important clues to exclude aorta aneurysm. Arteriography can provide important information in assessing such equivocal conditions.Retroperitoneal fibromatosis is a rare reactive-benign process . It growFibromatosis is divided into two major groups: superficial and profound. Superficial forms  typically have small volumes and grow up slowly , 5. NineMost of the patients are asymptomatic until the mass reaches great volumes. Depending on localization of intestinal obstruction, abdominal pain, gastrointestinal bleeding, or fever unknown origin can be seen. Symptomatic patients present with vague, nonspecific abdominal symptoms, including dull, poorly localized back or flank pain. In this case, retroperitoneal fibromatosis caused back pain.Despite the great probability of recurrence, surgery takes great part in treatment of fibromatosis. Complete resection has been suggested as a first choice, and if not, mass reduction has been suggested. Large excision of tumor decreases recurrence risk . HoweverIn conclusion, it is necessary to consider fibromatosis with retroperitoneal localization that was found to be rare in differential diagnosis of abdominal aorta aneurysm. Abdominal MRI and/or CT before surgical procedure can provide important clues to exclude abdominal aorta aneurysm.


A 9-week-old female was referred with a 7-week history of increasing abdominal distension and non-bilious vomiting. Antenatal and postnatal history was unremarkable. Physical examination revealed a large palpable mass filling most of abdomen, with no jaundice. Results of laboratory and tumour markers (serum β-HCG and α-fetoprotein) were within normal range. Abdominal ultrasound and CT-scan confirmed a homogenous cystic mass that filled most of the abdomen displacing solid viscera and bowel superiorly and posteriorly .At surgery, a large cyst attached to the inferior part of the accessory hepatic lobe was found. The cyst was removed together with the accessory lobe. The postoperative course was uneventful and the patient was discharged seven days after the procedure. Histopathological examination revealed a non-complicated hepatic angiomyxoma. No recurrence of tumour was seen on follow-up CT scan.Angiomyxoma is a rare , slowly growing, and benign proliferative mesenchymal tumor arising primarily in the soft tissue of the pelvis and perineum of adults. There is lack of agreement among pathologist regarding its pathogenesis; however, a fibroblastic/myofibroblastic origin seems most likely. Histologically, it consists of spindle-shaped or stellate cells widely separated by loose, myxoid stroma, and a prominent vascular component. Most patients are asymptomatic however others may complain of pain or abdominal distension and pressure symptoms. The diagnosis can be difficult. Ultrasound, CT-scan, and MRI can be useful tests to help in diagnosis. The main treatment is surgery with the aim to remove the whole tumour. Despite local recurrence reaching up to 47%, the prognosis is considered good.[1] Ectopic liver and hepatic accessory lobes are extremely rare congenital anomalies (0.09-0.7%), which can be associated with benign tumours.[2-4]Source of Support: NilConflict of Interest: None declared


A 32-year-old woman presented with a slow-growing, painless, subcutaneous lesion in the right inguinal and perineum regions. The mass was 24.0 cm × 10.5 cm × 5.0 cm in size, well circumscribed, mobile, and rubbery. Microscopically, the resected mass was mainly composed by a proliferation of small spindle or stellate cells, variably admixed with mature adipose tissue, embedded within an abundant myxoid and collagenized stroma. Immunohistochemically, the spindle and stellate cells were strongly positive for vimentin, CD34, and bcl-2 antibodies but not for smooth muscle actin and desmin. The tumor was diagnosed as dendritic fibromyxolipoma based on the typical findings of histology and immunohistochemistry. Clinical follow-up of 9 months after surgery revealed no evidence of recurrence. We report the first case of dendritic fibromyxolipoma in the right inguinal and perineum regions and discuss the different diagnosis.http://www.diagnosticpathology.diagnomx.eu/vs/1313680868103019.The virtual slide(s) for this article can be found here:  Dendritic fibromyxolipoma (DFML) is a rare benign soft tissue lesion that most commonly arises in the subcutis or muscular fascia of the head and neck, shoulders, calf, foot, or back in adult male patients. The characteristic histologic picture is described as an admixture of mature adipose tissue, spindle and stellate cells, and abundant myxoid stroma with prominent collagenization. These neoplasms typically show positive immunoreactivity for CD-34, bcl-2 and Vimentim. We describe a case of DFML in the right inguinal and perineum regions. To our best knowledge, female DFML in inguinal including the perineum region has not been reported.A 32-year-old woman was first seen by us 3 years after becoming aware of a painless subcutaneous mass in her right inguinal and perineum regions. Initially, the mass was in the right inguinal region, grew up slowly towards the perineum region. The mass was unreducible and could not become more prominent when coughed, strained, or stood up. The mass was grew fast with no obvious incentive in the past three weeks. On physical examination, the mass was subcutaneous, 20 cm × 10 cm × 7 cm in size, mobile, rubbery, unreducible. The transillumination test and auscultation for bowel sounds on the mass were negative. Her past and family histories were non-contributory. Laboratory data as well as tumor markers, such as CEA, CA19-9, were normal. Ultrasonography (US) revealed a well circumscribed, inhomogeneous mass with prominent vascularity, measuring about 19.7 cm × 9.2 cm × 6.8 cm, in the right inguinal and perineum regions. The mass originated from the right labium majus pudendi and extended to the low abdominal wall. No evidence was shown for a connection between the abdominal cavity and the mass. Also, computed tomography (CT) demonstrated a mixed density, well circumscribed mass in the same region. CT value of the mass ranged from −9 to 25 Hu, which revealed a soft tissue. Enhanced CT scanning revealed a heterogeneous, medium enhanced with prominent collagenization in the mass Figure . ImmunohDendritic fibromyxolipoma is an uncommon benign soft tissue tumor that first reported by Suster et al. in 1998 in twelve patients -4.The most striking histologic feature of DFML is an admixture of mature adipose tissue, spindle and stellate cells, and abundant myxoid stroma with prominent collagenization. Immunohistochemically, the vimentin and CD34 immunohistochemical stains accentuated the cell’s dendritic nature by revealing slender, complex cytoplasmic prolongations which are the main reason of it’s named . The curDFML should be differentiated from some benign lesions: spindle cell lipoma (SCL), solitary fibrous tumor(SFT), lipoblastoma, lipoblastomatosis, and nodular fascitis. Of the other tumor-like lesions, SCL is most likely to be confused with DFML. SCL is composed of a mixture of mature adipocytes and uniform spindle cells within a matrix of mucinous material traversed by a varying number of birefringent collagen fibers. It shares many features with DFML including age, male predilection, location, gross features. The signally similar clinical and histological feature of the lesions makes it difficult for distinguishing DFML from SCL . Suster Other benign spindle cell tumor that should be distinguished from DFML is solitary fibrous tumor (SFT). SFTs which have a predilection for the thoracic cavity are rare fibrous neoplasms. Histologically, the tumor is characterized by a “patternless pattern” of short spindle cells with scant cytoplasm and bland cytologic appearance separated by strands of rope-like collagen, and a “hemangiopericytoma-like” pattern where the lesional cells are densest around small and medium ectatic and branching vessels . The “heThe distinction between DFML and malignancy, both of which can have a plentiful myxoid matrix, proliferation of capillaries and relatively large size, is more subtle and difficult. Among the malignant tumors, the possibility of a myxoid liposarcoma (MLS) should be seriously considered. MLS, a low grade malignancies, is the most common subtype of liposarcoma. MLS shares many features with DFML including a delicate plexiform vascular pattern, large size, and a myxoid matrix. But MLS is distinguished from DFML by the lower extremities fascial planes predilection, the infiltration of surrounding structures, and the presence of lipoblasts on higher magnification ,9. FurthDFML has never been described in the perineum region. DFML in the perineum region of females should be differentiated from perianal sepsis, lesions of anogenital mammary-like glands (AGMLG) and some relatively site-specific stromal tumours. Perianal abscess/sepsis is the most common cause of a mass in the perineum region and can be easily differentiated from DFML by its clinical presentation . AGMLG aEven though DFML has been found for more than 14 years, the nosological position of DFML is still indeterminate. Suster et al.  on the mDendritic fibromyxolipoma is very rare benign tumor. We report the first example of DFML in the right inguinal and perineum region. A diagnosis of DFML should be made by their microscopical and immunohistochemical features. DFML should be considered in the differenial diagnosis of lesions with spindle cell lipoma, solitary fibrous tumor, lipoblastoma, lipoblastomatosis, nodular fasciitis, and myxoid liposarcoma.Written informed consent was obtained from patient's parents for publication of this case report and any accompanying images.The author’s declared no potential competing interest with respect to the research, authorship, and/or publication of this article.XJZ, KN and GJK performed the histological examination of the tumor and were major contributors to the writing of the manuscript. SZ, DFC and XHZ are the surgeons who operated on the patient and interpreted the patient data. All authors read and approved the final manuscript.


Youssef's syndrome is characterized by cyclic hematuria (menouria), absence of vaginal bleeding (amenorrhea), and urinary incontinence due to vesicouterine fistula (VUF), the least common of the urogynecological fistulas. Youssef's syndrome has a variable clinical presentation. A vesicouterine fistula is an abnormal pathway between the bladder and the uterus. The most common cause is lower segment Cesarean section. Conservative treatment may be appropriate in some cases, but surgery is the definitive treatment. Vesicouterine fistula should be suspected in cases presenting with urinary incontinence even years after Cesarean section. Diagnostic tests as well as necessary appropriate surgery should be performed on cases with suspected vesicouterine fistula. We present a 40-year-old multiparous woman with vesicouterine fistula after primary Cesarean section; she presented with urinary incontinence, hematuria, and amenorrhea 1 year after the birth. Here, we discuss our case with the help of previously published studies found in the literature. A vesicouterine fistula is an abnormal pathway between the bladder and the uterus. The first case was reported by Knipe and colleagues in 1908. Vesicouterine fistula is the least common of all the urogenital fistulas, representing 1–4% of all cases . The lesA 40-year-old multiparous female patient presented to our clinic with urinary incontinence, hematuria, and amenorrhea a year after giving birth by Cesarean section due to fetal distress in our clinic. Patient history had no highlights except a lower segment Cesarean section by Pfannenstiel incision due to fetal distress in previous year. Transvaginal USG (ultrasonography) showed normal uterine and ovary structures. Noticeable urine smell showed that the case had moist vulvar area and speculum examination showed a leakage consistent with hematuria that flowed from cervical os . After aIn 1957, Youssef described a syndrome comprising of cyclic hematuria, amenorrhea, menouria, and complete urinary incontinence in a patient who had lower segment Cesarean section (LSCS) . The VUFThe diagnosis of VUF is often confirmed by imaging studies and cystoscopy. Cystoscopy, even when repeated, can fail to confirm the fistula . MethyleAlthough VUFs are difficult to diagnose using USG, Park et al. reported that sonography can demonstrate the fistulous tract as double echogenic lines between the endometrium of the anterior wall of the uterine body and the mucosal layer of the posterior wall of the bladder . HoweverTreatment methods include expectant management with long-term bladder catheterization, medical treatment, and surgery. Spontaneous closure of vesicouterine fistula has been reported . MedicalTransabdominal repair can be performed by extraperitoneal or retrovesical (O'Connor) technique . DisadvaIn our case, fistula focus between the bladder and lower segment of uterus was found using O'Connor technique through extraperitoneal abdominal incision and mucosa of uterus and bladder were repaired using long-term absorbable suture materials. Extraperitoneal drainage catheter placed in abdomen was removed after 5 days and urethral Foley catheter was removed after 14 days following operation. No urine leakage was detected and spontaneous urination was normal.Vesicouterine fistulas are uncommon, yet they are becoming more prevalent due to changes in modern obstetrical care. They should always be kept in mind in patients with a history of Cesarean section or who have experienced a gynecological procedure associated with signs of hematuria and/or urinary leakage.


Intraosseous epidermoid inclusion cysts are rare benign epithelial inclusion cysts in the bone. They are usually found in the cranium and hand phalanges. They are slow growing lesions, and it is difficult to differentiate them from other inflammatory and cystic lesions. Only a few cases of epidermoid inclusion cyst in the jaw have been reported in the literature. This is the fourth case reported as intraosseous epidermoid cyst of the maxilla in the English literature.An asymptomatic 59-year-old Caucasian man was referred to our Oral and Maxillofacial Surgery clinic for a unilocular radiolucent area at his anterior maxilla shown on an orthopantomograph. He was scheduled for surgery and underwent cyst extraction surgery. A pathological examination revealed epidermoid cyst. The diagnostic dilemma in this case report in opposition to the presented intraosseous epidermoid cysts in the literature is that there was no trauma history to his upper jaw. Treatment for this cyst is conservative surgical excision and recurrence is uncommon.This report presents an unusual case of an intraosseous epidermoid cyst that occurred with no trauma history to the upper jaw. Although only three cases of epidermoid inclusion cyst have been reported in the maxilla, epidermoid inclusion cyst should be considered in the differential diagnosis of radiolucent lesions of the jaws. An epidermoid cyst is a benign soft tissue neoplasm that can be seen anywhere in the body. Males are affected more frequently than females. This lesion is unusual before puberty unless associated with Gardner syndrome. EpidermAn asymptomatic 59-year-old Caucasian man was referred to the clinic for routine dental evaluation. A radiologic examination revealed a unilocular radiolucent area at his anterior right maxilla with well-circumscribed sclerotic border Figures  and2.All his maxillary teeth with the exception of his left maxillary cuspid had been removed over the years, uneventfully; the most recent extractions were performed 7 years previously. There was no history of trauma to his maxilla or of any other surgery. He was a non-cigarette smoker, had no further illness and was otherwise healthy. On examination, the alveolus was of normal consistency with expansion or swelling; there was no pain or tenderness; there was no change in the color of the soft tissues around the lesion. There was no lymphadenopathy.Based on the clinical and radiographic examinations, differential diagnosis should be performed with other radiolucent lesions such as residual, radicular or lateral periodontal cyst, odontogenic keratocyst, globulomaxillary cyst, traumatic bone cyst and ameloblastoma.Residual cyst is the first pathology that should be considered depending on the similarity of radiologic appearance, clinical evaluation and location, unless the lesion is located around the tooth root region and the lesion is unilocular and asymptomatic as in the presented case.On the one hand residual lateral periodontal cysts occur along remnants of the lateral root surface of a tooth and 80% occur in the mandibular premolar, canine, lateral incisor area. On the other hand, polycystic appearance may be seen radiologically.A unilocular clinical appearance can also be seen with odontogenic keratocysts. The usual localization of keratocysts is the posterior mandible and ascending ramus and for large keratocysts their radiologic appearance can be multilocular. In addition, in up to 40% of odontogenic keratocysts an unerupted tooth is involved in the lesion.Globulomaxillary cysts are one of the pathologic lesions seen in this region of the maxilla. On clinical examination this cyst is localized between the maxillary lateral and canine tooth; radiological evaluation shows unilocular radiolucency. Although recently most so-called globulomaxillary cysts are usually found to be odontogenic cysts of various types, it should be also considered for differential diagnosis.Traumatic bone cyst should be discussed in the diagnosis. A generally agreed etiologic factor for this cyst is bleeding into the bone after trauma. Organized hematoma will develop the cystic defect. Similarly, according to the literature, the most probable etiologic factor for intraosseous epidermoid cysts is traumatic implantation of epidermoid cells into the bone. This lesion is usually seen at the first and second decades of life primarily at the premolar and molar area of the mandible. The lesion is usually asymptomatic but sometimes pain or paresthesia occurs. The radiologic appearance is well-defined unilocular radiolucency.Ameloblastomas generally occur at the posterior mandible and are mostly seen in multicystic (86%) form. Unilocular lesions are mostly localized at the pericoronal area of an unerupted mandibular third molar.The patient was scheduled for surgery. Under aseptic conditions, his anterior superior alveolar nerve was blocked with local infiltration using 4% articaine hydrochloride and 1:100,000 epinephrine. A full thickness mucoperiosteal envelope flap with two oblique release incisions was carried out to expose the lesion and the surrounding alveolar bone was removed by round and fissure burs under irrigation with sterile saline solution. On clinical examination the lesion appeared well demarcated from the surrounding bone. The unilocular mass was removed totally Figure . Sharp eThe gross pathologic specimen was a well-circumscribed cystic lesion containing soft, tannish yellow material in cross-section. Grossly the extracted cystic lesion measured 1.0×1.0×0.7cm.Microscopic examination of the specimen revealed a cavity that was lined with stratified squamous epithelium resembling epidermis Figure . A well-The usual etiologic factor for epidermoid cyst generation is localized inflammation of hair follicles. Other etiologic factors are non-neoplastic proliferation of infundibular epithelium during healing process or traumatic implantation of dermis epithelium-7.Histological differential diagnosis should eliminate dermoid cysts. To be designated as a dermoid cyst, skin appendages, such as hair follicles, sebaceous and sweat glands, and arrector pili muscles must be identified in the wall of the cyst. As the specimen presented here did not contain these dermal tissues it was defined as an epidermoid cyst.et al. reported that head injuries caused the inclusion of epidermal cells into the calvarium, and epidermoid cysts occurred[The development of epidermoid cysts can be due to congenital or acquired factors. The former is thought to develop from congenital inclusion of ectodermal tissue during embryological development. Althougoccurred.et al. presented the first fully documented example of a totally intraosseous dermoid cyst of the mandible in 1980[Craig  in 1980,10. The Although intraosseous epidermoid cysts are extremely rare in the maxilla, they should be considered in the differential diagnosis of radiolucent lesions of the jaws.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SYE was the principal author and major contributor in writing the manuscript. SU collaborated in writing the manuscript. HO performed the histological examination of the epidermoid cyst. All authors reviewed and approved the final manuscript.


Hemangiopericytomas are vascular tumors with a susceptibility to arise anywhere in the human body. We present a case of a 68-year-old female with primary omental hemangiopericytoma and a two-time recurrence managed with surgery and close follow-up. The first recurrence was at 52 months and the second at 37 months following the prior presentation. No adjuvant chemotherapy or radiation therapy was administered. Given the widespread nature of the cell of origin, routine follow-up postoperatively with interval imaging in order to detect recurrences is imperative. Pathologic tumor characteristics may determine potential for recurrence and may also assist in determining whether adjuvant treatment modalities should be included in the management plan. Review of the English literature reveals a total of 24 cases of omental hemangiopericytomas inclusive of the current report. Hemangiopericytomas are a rare form of vascular tumors that arise from the pericytes of Zimmerman that surround capillaries and function to regulate blood flow. They were first recognized by Schmidt in 1937 and fully described and named by Stout and Murray in 1942 [βHCG (beta-human chorionic gonadotropin). She underwent an exploratory laparotomy with lysis of adhesions and resection of the abdominal tumor with partial omentectomy because of concern for a primary ovarian carcinoma. Intraoperative findings included numerous dilated blood vessels in the omentum measuring up to 1.5 cm in diameter with a 25 × 20 cm soft, friable mass. The mass occupied most of the midabdomen, encasing the omentum, and was attached to the sigmoid mesentery and bladder peritoneum. The ovaries, uterus, and remaining pelvic structures were normal in appearance. There was no evidence of metastatic disease. The estimated blood loss (EBL) at the time of surgery was 1 liter and the patient received 2 units of packed red blood cells (PRBCs). Final pathology revealed “hemangiopericytoma of the abdomen with low malignant potential.” Histological findings showed a staghorn vascular pattern with solid perivascular areas formed by plump, comma-shaped cells with rare mitoses (less than 2 per 10 high power fields (HPF))  was elevated at 77 U/mL with normal CEA (carcinoembryonic antigen), AFP , and s (HPF)) . On immus (HPF)) . S-100, She presented again to the gynecology oncology clinic in early 2011, 52 months after her primary surgery with symptoms of a “pinching sensation” and concerns for a palpable mass in the left lower quadrant of her abdomen of few months' duration. Computed tomography (CT) imaging of the abdomen and pelvis revealed interval development of a 2 × 1 cm hyperdense soft tissue lesion with a midline pelvic ventral hernia. Follow-up magnetic resonance imaging (MRI) showed a 2.1 × 1.5 cm ovoid-shaped mass probably originating in the peritoneal surface interposed between the lower uterine segment and sigmoid colon which demonstrated slightly hyperintense T2 signal in comparison to muscle with isointense T1 signal and uniform postcontrast enhancement. She underwent a second-exploratory laparotomy, tumor reductive surgery, total abdominal hysterectomy, bilateral salpingooophorectomy, segmental ileal resection with side-to-side reanastomosis, and repair of the ventral hernia. Intraoperative findings revealed rectosigmoid nodules as well as small nodules involving the ileum. Pathology was again consistent with hemangiopericytoma involving the resected rectosigmoid nodules, small bowel nodules with microscopic small bowel serosal residual disease. Histologic examination revealed uniform spindled to round cells with small amounts of pale or eosinophilic cytoplasm, indistinct margins, bland vesicular nuclei, and low mitotic activity. Numerous staghorn vascular spaces of varying sizes lined by flattened endothelium were also observed. Within the mass were alternating hypercellular perivascular areas formed by plump cells as well as hypocellular areas with increased collagen surrounding individual cells and occasional multinucleated giant cells. Immunohistochemical stains revealed CD 34 and focal CD 10 positivity. CD31, calretinin, HBME-1 (anti-mesothelioma antibody), C-kit, keratin, and S-100 all stained negative. Given findings consistent with recurrent hemangiopericytoma, the plan of care at the time included routine surveillance with no adjuvant chemotherapy/radiation therapy. She was followed up with imaging of the abdomen/pelvis.Imaging performed 37 months after the last surgery showed a 2 cm enhancing solid mass in the left hemipelvis interposed between the proximal sigmoid serosa and adjacent small bowel and subjacent to the medial left urinary bladder. Another adjacent enlarging 1.7 × 1.9 cm enhancing mass with central necrosis or hypoattenuation was also seen just above the subjacent bladder and vaginal cuff. The patient elected to proceed forward with surgical resection and underwent a third-exploratory laparotomy, extensive lysis of adhesions, enterolysis, and tumor debulking. Intraoperative findings included a 3 cm ileal mesenteric mass with additional tumor nodules . Pathology was again consistent with recurrent hemangiopericytoma. No adjuvant therapy was recommended at the time except close surveillance and imaging.Hemangiopericytomas have a distinct set of clinical, histopathological, and immunohistochemical features. These tumors are characterized by a unique staghorn appearance composed of thin walled vessels surrounded by ovoid and short spindle shaped cells , 6. ImmuHemangiopericytomas in general have been found to have a favorable prognosis, despite the potential for recurrence and/or metastasis even after surgical management , 9. EnziOmental hemangiopericytomas represent around 10% of all primary solid omental tumors . A revieSurgery with curative intent remains the primary mode of management of hemangiopericytomas arising from the omentum . PerformHemangiopericytomas have a high five-year overall survival; however, given the cellular origin of these tumors, long-term follow-up is essential as recurrences have been reported even after extended disease-free intervals. Predictors of recurrence and metastasis include histological appearance and behavior of the primary tumor. Surgery remains the chief method of therapy, with the potential for utilization of chemotherapy and/or radiation therapy as adjuvants in the more aggressive malignant cases with an unclear benefit to its use given the nature of the disease.


Ileal perforation due to fish bone is a rare event. The condition is difficult to diagnose due to lack of specific clinical features and low sensitivity of imaging techniques. We report a case of ileal perforation by a fish bone that was detected laparoscopically and managed nonsurgically.A 45-year-old Sinhalese man presented with acute onset right iliac fossa pain and fever for three days. On examination, he had significant right iliac fossa tenderness and guarding. His white cell count and C-reactive protein level were elevated and an ultrasound scan was indicative of a bowel mass formation. A clinical diagnosis of acute appendicitis was made and laparoscopic appendicectomy was scheduled. At initial survey, a thin spike-like structure was retrieved from the bowel mass, which was revealed to be a fish bone. Our patient was managed with antibiotics only and did not develop any complications.Ileal perforation due to fish bone is a rare condition that can mimic common conditions like appendicitis. Preoperative diagnosis is rarely made. The slow process of fish bone migration results in concomitant sealing of the perforation, reducing contamination. Use of laparoscopy may be useful in diagnosing this condition and preventing the morbidity of laparotomy in these patients. Perforation of the gastrointestinal (GI) tract due to an ingested fish bone is a rare event occurring in less than 1 percent of patients . DiagnosA 45-year-old Sinhalese man presented with a history of right iliac fossa (RIF) pain and fever for three days. He did not have nausea or vomiting and was having normal bowel opening. Our patient had undergone coronary stenting for ischemic heart disease and was on clopidogrel. He was not diabetic. On examination, he was afebrile (37.8ºC) and hemodynamically stable. There was localized tenderness, guarding and rebound tenderness in the right iliac fossa. Clinically, there was no free fluid in the peritoneal cavity. A clinical diagnosis of acute appendicitis was made. His white cell count was 10,800/mm3 with 75% granulocytes and the C-reactive protein level was 45.7mg/L . An ultrasound scan of his abdomen revealed a soft tissue mass formation and localized fluid collection in the RIF suggestive of an appendicular mass. It was decided to proceed with a laparoscopic appendicectomy. Pneumoperitoneum was achieved using the open Hassan technique. A 5mm port was inserted supraumbilically and a 5mm telescope was inserted. On initial exploration of the RIF, a mass formation by ileal loops with purulent exudative membrane around the bowel wall and greater omentum was seen Figure . The appet al. have reported a case of fish bone migration to the liver diagnosed with typical ultrasonic features [The majority of ingested foreign bodies (FBs) pass through the GI tract uneventfully . Fish bofeatures . This phfeatures ,10. Thisfeatures . The incfeatures . This paFish bone perforation of the ileum is a rare condition that may mimic other common inflammatory conditions. It is difficult to diagnose clinically or with available imaging modalities. The slow process of migration of the bone through the intestine prevents gross contamination of the peritoneal cavity. Increasing use of laparoscopy in managing acute abdominal conditions may help in managing this condition nonsurgically.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.


Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above. Malignant triton tumors (MTTs) are a histological subgroup of the malignant peripheral nerve sheath tumors (MPNSTs) with focal component of heterologous skeletal muscle differentiation . MPNSTs MTTs account for 5–10% of MPNSTs  and occuOnce diagnosed, physicians face the difficulty of determining the proper therapeutic regimen. Due to the rarity of this type of tumor; there is no treatment consensus available yet. Wide surgical excision or debulking of the tumor with negative margins and successive radiotherapy is currently often employed . The rolHere we discuss two unique cases describing two sisters who were diagnosed with MTTs only a few years apart. Their presentation stands out from the literature available in their interpatient relationship, gender, and age of presentation. Although MTT is described as an exceedingly rare tumor, the fact that it occurred in two siblings in conjunction with clinical NF-1 is of interest. To our knowledge, no prior cases of MTTs occurring in siblings have been described in the literature. Additionally, MTTs in the setting of NF-1 have been described in a patient population of primarily young males . Our pat2 with mesna 2 g/m2 alternating with adriamycin 75 g/m2 every other week for first two cycles four weeks apart and then continued with ifosfamide and mesna for the rest of the two cycles without adriamycin. Concomitant radiation was given after the 2nd cycle and continued till the 3rd chemotherapy cycle. The radiation therapy was applied to the left abdominal wall, with a total dose of 65.4 Gy, with initial photon fields, followed by shrinking fields with electrons with 33 dose fractions during the course of two months. She currently follows up in our cancer center and has no evidence of disease recurrence four years after her treatment.We describe the presentation of malignant triton tumors in two siblings who are familiar to our cancer center. The first patient is a 36-year-old female with past medical history of asthma who presented to us with complaints of left sided chest wall swelling and mild pain that progressed over a few days. She denied the history of smoking, alcohol, or drugs. Family history was significant for colon cancer in father and maternal aunt. She was not sexually active. On examination, she was found to have axillary freckling, café au lait spots, and mild cognitive impairment. The findings were consistent with NF-1. There was no associated hepatosplenomegaly noted on examination. On imaging, a very large soft tissue mass measuring 15 cm by 15 cm by 13 cm in the left lower anterior lateral chest wall with possible ulceration and axillary lymphadenopathy was seen. The mass infiltrated the adjacent chest wall musculature; however, the ribs and lungs were commented as being intact and clear, respectively . At the Unfortunately, her younger sister presented to us a few years later at the age of 42 with complaints of pain in the left lateral chest wall with radiation to the left flank that was worsening over 3-4 months. There was no trauma preceding the event. She had no past medical history and was a nonsmoker and nonalcoholic. She was not sexually active. On examination, café au lait spots were also noted on her along with mild cognitive impairment. There was fullness on the left side of the chest underneath the left ribs with some tenderness to palpation but no axillary lymphadenopathy or splenomegaly. On imaging, a multicompartmental retroperitoneal cystic mass in the posterior pararenal space was observed. It extended through the posterior abdominal wall, measuring 10 cm by 7.7 cm by 10.4 cm, and exerted a compressive effect on the left psoas muscle while simultaneously displacing the left kidney anteriorly. Bulky mediastinal and paratracheal lymphadenopathy was additionally observed Figures . The tumMPNSTs with elements of rhabdomyoblastic differentiation are termed as malignant triton tumors (MTTs). MTTs are referred to as a mosaic tumor due to their muscular and neurogenic components . Masson The incidence of MPNSTs is 1 per 100,000 cases . MTTs acThe most established theory regarding the histopathogenesis of MTTs is that multipotent neural crest cells of ectomesenchymal origin are capable of divergent differentiation to both nerve and muscle components . It has In the majority of reported cases, triton tumors are located across peripheral nerves, usually close to the spine, commonly in the head and neck region . Few casWoodruff et al. established the original histological criteria for the diagnosis of the malignant triton tumors. He stated that the tumors must arise along the course of a peripheral nerve or in a preexisting neurofibroma in patients with NF-1. Additionally, the tumors must display identical growth characteristics of Schwann cells. Finally, they must contain “bona fide” rhabdomyoblasts originating from the body of peripheral nerve tumor and not from extension or metastasis of an extrinsic rhabdomyosarcoma . DaimaruToday, the diagnosis of MTT is generally made according to these criteria as well as immunohistochemical findings for cell origin tracing. Nerve sheath differentiation is confirmed by S-100 protein and Leu-7 (CD57) positivity. Rhabdomyoblastic differentiation is confirmed by immunohistochemical positivity for desmin, actin, and myogenin , 12. ThrHowever, one major area of concern for clinicians is the rate of tumor occurrence and regrowth after appropriate treatment. Local recurrence rate varies from 22% to 43% , 7, 18 aLymphatic invasion and lymph node involvement has not been reported in patients with MTT . The mosα [Currently, there is no consensus regarding the treatment guidelines for malignant triton tumors. Several authors have proposed recommendations, including radical excision, radical excision followed by radiotherapy and chemotherapy, and excision with high dose radiotherapy. However, as for any other sarcoma, resection of the tumor with wide margins followed by radiotherapy is the recommended treatment. The need for chemotherapy has not been clearly defined . Some reα . In concα , 11, 19.In our case reports, we treated the first sister with four cycles of adjuvant ifosfamide and adriamycin along with radiotherapy after wide surgical excision and reresection of the tumor. She responded well to it and remains disease-free even after 4 years of initial diagnosis. We are treating the second sister with the same chemotherapy regimen along with concurrent radiotherapy. She is currently responding well to treatment with few side effects. We will continue to monitor her and observe her treatment response.We present the cases of MTTs in two sisters with clinical neurofibromatosis. Our cases are unique due to their sisterly presentation within a decade of each other. This is in contrast to MPNSTs, which have been previously described in siblings. Additionally, MTTs in the setting of NF-1 have not been seen in our patient demographics: middle-aged females. Given the aggressive nature of this tumor subtype, one of the sisters has remained disease-free for four years after initiation of adjuvant chemotherapeutic therapy. We are hopeful of the treatment potential for her sister. Additionally, the information learned regarding this disease type may help shed light on the role of chemotherapy in care of patients diagnosed with MTTs.


Primary carcinoid tumors of the nasal cavity and sinuses are exceedingly rare. An accurate histopathological diagnosis is crucial to optimal investigation and management. We present a case of a primary atypical carcinoid tumor arising from the sphenoid rostrum without evidence of associated carcinoid syndrome. This rare but important differential diagnosis of a nasal tumor is discussed and important unique management issues are highlighted. Carcinoid tumours are not well known to otorhinolaryngologists; these neuroendocrine tumours are found almost exclusively below the level of the clavicle. Arising from enterochromaffin cells, they may synthesize a variety of vasoactive substances and hormones. Carcinoids are typically diagnosed in the fifth or sixth decade of life and approximately half of patients may be asymptomatic at diagnosis.The incidence of carcinoid tumours has been estimated to be 1 to 2 per 100,000 of population. The true incidence is likely to be higher due to the slow-growing, subclinical nature of a large proportion of these lesions. The surveillance, epidemiology, and end result (SEER) program of the National Cancer Institute of the United States of America has analyzed 10,878 carcinoid tumours; 64% were of gastrointestinal origin and 28% originated in the lower respiratory tract [Carcinoids may be classified histologically into “typical” and “atypical” tumours . TypicalWe present a case of an atypical carcinoid of the sphenoid sinus rostrum and posterior nasal septum without evidence of carcinoid syndrome. Whilst very few cases of ethmoid and frontal sinus carcinoids have been described, only one carcinoid of the nasal septum has previously been reported –5. The cA 48-year-old male presented with a 3-month history of mild watery rhinorrhea and pain on nasal blowing, particularly on the left. No epistaxis was reported and there was no history of systemic upset. He was a nonsmoker and had no significant past medical history of note.A left-sided nasal polyp emanating from the left sphenoid sinus ostium was identified on initial nasendoscopic examination. High resolution computed tomography (HRCT) showed this soft tissue mass to be situated between the middle turbinate and the nasal septum, extending posteriorly to the level of the posterior choana with evidence of fluid within the left sphenoid sinus . NeitherHistopathological evaluation revealed a polypoid lesion measuring 35 × 20 × 10 mm without atypia of the surface epithelium. Lobules of fairly uniform tumour cells with stippled chromatin and a moderate amount of pale eosinophilic cytoplasm within the lesion were shown on sectioning . CentralImmunohistochemical staining demonstrated strong and diffuse uptake of synaptophysin. Synaptophysin is a membrane glycoprotein of neuroendocrine cells and, like chromogranin A, is a valuable specific neuroendocrine marker . CytokerFurther investigation was undertaken to further evaluate the nature of the tumour; carcinoid syndrome was not evident. Serum serotonin and 24-hour urine 5-hydroxy-indole acetic acid (5-HIAA) levels were within normal limits. Full radiological evaluation and gastrointestinal endoscopy did not reveal distant disease.Endoscopic resection using navigation technology was then performed. The remaining tumour was found to be pedicled upon the sphenoid rostrum and posterior septum and specifically seemed to arise from the intersinus septum of the sphenoid. An en bloc resection included complete removal of the anterior sphenoid face and removal of all sphenoid sinus mucosa, a posterior septectomy and ipsilateral ethmoidectomy. Histology revealed a small nodular focus of residual tumour with exact pathological correlation with the initial specimen. Tumour involvement of bone was not detected.A policy of close clinical follow-up was agreed on. A whole body octreotide (radiolabelled indium-111-tegnesium-octreotide) scan performed at an interval of 3 months following surgery showed no uptake in the nasal area and no other identifiable lesions. A positron emission tomography (PET) scan at one year postoperatively did not reveal evidence of disease and repeat lower gastrointestinal endoscopy was normal. At 3 years following surgery the patient is asymptomatic without evidence of local recurrence on detailed nasendoscopic examination.Once a diagnosis of a sinonasal carcinoid tumour has been established, optimal initial management relies upon appropriate systemic investigation. Presence of a carcinoid tumour must be distinguished from the “carcinoid syndrome.” This describes systemic effects that occur as a result of release of compounds synthesized by the tumour. Symptoms include episodic flushing and diarrhea and the development of respiratory symptoms, such as wheezing. Carcinoid heart disease, characterized by endocardial thickening and valvular fixation predominantly of the right side of the heart, occurs in approximately two-thirds of patients with carcinoid syndrome. This may require surgical management and lead to significant morbidity and mortality.Carcinoid crisis is a life-threatening form of carcinoid syndrome. Flushing, diarrhea, tachycardia, arrhythmias, hypertension or hypotension, bronchospasm, and altered mental status may occur. Anesthesia, surgery, or chemotherapy administration may precipitate a crisis, thought to be due to the release of compounds secreted by the tumour. Failure to identify either carcinoid syndrome or carcinoid heart disease could therefore result in management-related morbidity and mortality.Treatment of localized disease is relatively well defined and typically comprises surgical resection, as was performed in our case. Metastatic disease poses a complex therapeutic challenge and several modalities of treatment have been investigated. Carcinoid syndrome may be controlled with somatostatin analogues such as octreotide and in such cases multidisciplinary input is recommended. Beneficial additional use of interferon alpha has been described. Liver metastases may be resected whilst liver transplantation and hepatic arterial embolization have also been explored .Given the relative rarity of primary sinonasal carcinoid disease, little is known of the prognosis. Five-year survival of patients with primary laryngeal carcinoids has been estimated to be 46.7% . An unexThis case of a sinonasal atypical carcinoid highlights the importance of accurate histopathological evaluation of any polypoid mass within the nasal cavity and paranasal sinuses. A carcinoid tumour is an important differential diagnosis, albeit rare. It is associated with unique management concerns and a need for long-term surveillance with respect to both recurrent local and distant disease.


Tibialis anterior muscle hernia is a challenging diagnosis. Ultrasound findings generally are negative because patients come to ultrasound study from home when they are at rest. When the operator of ultrasound suspects a muscle hernia, he has to scan the affected limb or the affected organ dynamically at rest and after stressing the limb. Here, we present a case of a 19-year-old dancer with anterior leg mass with negative ultrasonographic findings at rest but was diagnosed with tibialis anterior hernia after stress dynamic ultrasound. The tibialis anterior muscle is the most common site of muscle herniation. It presents clinically as soft tissue mass, usually indolent, but some patients have pain during limb movements. The mass can disappear during rest. This behavior of the mass is the clue for suspecting this entity. Imaging plays a crucial role in diagnosis. MRI can be of help but because of its on-off appearing, this makes dynamic ultrasound the gold standard of examination.A 19-year-old female dancer was referred to our hospital after being evaluated as having a 1-cm nonpainful mass in her right anterior leg that appeared during the day and disappeared the next morning. The ultrasound result of her leg was negative for any pathology (Figure A muscular hernia through an overlying fascial defect, such as loosening or tearing, is most commonly reported in athletes or individuals with occupations that cause excessive strain on the legs like dancers such like our case. They occur usually in the lower limb, and usually, they affect the tibialis anterior muscle because its fascia is the most vulnerable to trauma, although other muscles in the lower leg, such as muscle of the extensor digitorum longus, peroneus longus, peroneus brevis, and gastrocnemius, have also been reported . This raClinical findings include the presence of soft, compressible nodules that vary in size depending on their position. This mass is characterized by herniation during leg dorsiflexion also known as ‘fencer's lunge position’ .Although this condition is typically diagnosed by clinical observation, imaging specially ultrasound should confirm the diagnosis to avoid unnecessary biopsy. The clinical differential diagnoses are soft tissue tumors, such as lipoma, leiomyoma, Schwannoma, and dermatofibroma. However, these tumors do not change when a subject's position changes. The dynamic ultrasound study as shown in our case is essential in diagnosing anterior tibialis hernia. Usually, they are asymptomatic, but some person reported vague and fatigued pain aggravated by exercise and by work –4]..4].The treatment is controversial; surgery is an option in symptomatic patients. Dynamic ultrasound should be done in every patient suffering from leg pain or suffering from a strange vanishing behavior of leg masses. Static ultrasound could be negative as in our first ultrasound scan which was done during rest . UltrasoAnterior tibialis muscle hernia is a rare disease. Dynamic ultrasound is essential for determining the diagnosis. Ultrasound should be done after exercise of the affected limb.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editor in chief of this journal.The authors declare that they have no competing interests.SA made the diagnosis and wrote the first draft. GH revised and approved the final draft. Both authors read and approved the final manuscript.Dynamic ultrasound video clip of the leg after exercise shows the mass appearing and disappearing during dorsoflexion and extension of the foot.Click here for file


Solitary fibrous tumours (SFTs) of the nose and paranasal sinuses are extremely rare. These were originally described as neoplasms of the pleura originating from spindle cells. It is further sub-classified as a benign type of mesothelial tumour. Its occurrence in many extra pleural sites have been reported earlier, mainly in the liver, parapharyngeal space, sublingual glands, tongue, parotid gland, thyroid, periorbital region, and very occasionally in the nose and paranasal sinus area.A 28-year-old man with a 6 month history of persistent progressive left nasal obstruction and watering of the left eye is reported. Further imaging by CT and MRI revealed a large, left-sided, highly vascular, nasal cavity mass  pushing laterally on the medial wall of the maxilla. The patient underwent a lateral rhinotomy, which proceeded with the excision of the mass. Histopathological analysis of the specimen was consistent with SFT.This case is reported to develop insights regarding diagnosis and management of such rare tumours. Solitary fibrous tumour (SFT), also known as benign fibrous mesothelioma or submesothelial fibroma , is sub-Various extra pleural sites have also been reported in literature; but its occurrence in the nose and paranasal sinuses is very rare. Adequate surgical excision with disease free tumour margin is usually curative. Around 27 such cases have been reported in world literature; however, the first case report of SFT found in the nose and paranasal sinuses seems to be from India.A 28 year old man, with a 6 month history of progressive unilateral left-sided persistent nasal obstruction, watering of left eye, and intermittent epistaxis was presented. He had no other known co-morbidities or history of bleeding diathesis. Rigid nasal endoscopy suggested a large mucosa covered mass filling the entire left nasal cavity. It was further evaluated with the help of Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and paranasal sinuses with computed tomography (CT) of the paranasal sinuses, which revealed a huge expansile enhancing mass, with multiple flow voids ,2 in theDue to the highly vascular nature and extent of the tumour, the patient underwent a lateral rhinotomy , which pIntraoperative findings showed a soft to firm, vascular mass filling the left nasal cavity  and pushMicroscopic Examination revealed a hypo cellular neoplasm consisting of fusiform cells within a dense collagen stroma . There wSFT are rare tumors of mesothelial origin which generally occur in adults in the third to fourth decade of life; but the age of onset has been reported ranging from 9 to 86 years . Classically, these tumors were described to originate from the spindle cells of the pleura. However, various extra pleural sites have been reported, namely the liver, parapharyngeal space, sublingual gland, tongue, orbit, eyelids, nose, paranasal sinuses, orbit, parotid gland, thyroid, and laryngopharynx -8. Extrathoracic head and neck SFTs also tend to behave in a benign fashion, in a pattern similar to their intrathoracic counterparts . Nasal aSinonasal SFTs present themselves as a slow-growing painless mass.If symptomatic, the most common presentation of SFT is usually nasal obstruction, rhinorrhea, intermittent epistaxis, and exophthalmos. As per available literature, the size of the tumour usually ranges from 2.8 to 8cm in its major axis ,12. These tumours are usually encapsulated, red, and fibrous. Here a soft to firm, well-encapsulated mass was observed. Non-contrast CT shows homogeneous isoattenuation compared with gray matter, and the tumours usually have marked enhancement after the administration of contrast material . Classically, on T2-weighted MRI, the soft-tissue component of SFTs is iso- to hypo-intense . These fDifferential diagnosis of sinonasal SFT should include inverted papilloma, hemangioma, schwannomas, juvenile angiofibroma, angiomatous polyps, and hemangiopericytoma . Morphologically, SFT has a number of specific macroscopic, histologic and immunophenotypic characteristics that are pathognomonic and help the pathologist to make the correct diagnosis . MacroscThe presence of CD34 and Vimentin positivity on immunohistochemical analysis is a typical finding and over half of all tumours are also CD99 positive (In addition, 75% of the tumours examined by Hasegawa and colleagues were positive for bcl-2 protein . Ultrastructural and immunohistochemical studies have suggested that SFTs primarily originate from mesenchymal fibroblast-like cells . The defFor SFTs of the nasal cavity endoscopic excision is the preferred surgical approach, although lateral rhinotomy, medial maxillectomy, external ethmoidectomy, and transfacial endoscopic approaches have been described -19. In t5 to 10% of all extra pleural SFTs have shown recurrence ,20. ResePrognosis mainly depends on the completeness of the surgical resection. However, the small number of SFT cases of the upper respiratory tract limits an estimation of accurate prognosis of extra pleural tumours and their clinical behaviour. In conclusion, a rare case of extensive SFT of the nasal cavity removed using lateral rhinotomy approach was presented. This case highlights the importance of diagnosing SFTs of the nasal cavity and paranasal sinuses, as their management differs from other tumours. This case also stresses the importance of immuno-histochemical and histopathological features in the diagnosis of SFT.


Solitary fibrous tumors (SFTs) are rare tumors that are mostly found arising from the pleura. SFT of the parotid gland is a rare tumor; only a few cases have been described in the literature. SFTs are benign in most cases. Clinically, SFTs usually manifest as well circumscribed, slow-growing, smooth, and painless masses. CT-Scan and MRI are the most sensitive imaging procedures used. The treatment of choice is complete surgical excision of the lesion. Since recurrence and metastasis can take place after several years, a lifelong clinical and imaging regular follow-up is compulsory. In this paper, we describe the diagnostic and therapeutic challenges of the up-to-now biggest parotid SFT. The clinical presentation, surgical management, and pathological and immunohistochemistry findings are described. Solitary fibrous tumor (SFT) was described by Klemperer and Rabin in 1931 as a tumor derived from the pleura . During Extrapleural SFTs have been reported in nearly all anatomic sites, with approximately 6% developing in the head and neck . SFT of A 67-year-old female presented with a 10-year history of a progressively developing, tender mass in the left parotid region. Her medical history was unremarkable. Physical examination revealed a 16 × 18 cm firm, fixed, immobile, smoothly contoured mass without overlying erythema. There were no facial palsy, no tumor in the pharynx and larynx, and no palpable lymph nodes, and the oral mucosa was intact. On the postcontrast computed tomography scan , a heterHistology revealed alternating hypocellular and high cellular areas demonstrating a population of densely packed, randomly arranged cells . The tumSFT is extremely rare in the parotid –13. A reAccording to the literature –14, paroDiagnostic work up includes CT and/or magnetic resonance (MR) imaging, even if results may not be specific for SFT. On CT scan, SFT appears as a well-defined soft-tissue mass relatively hyperdense with respect to adjacent tissues, demonstrating heterogeneous enhancement after contrast administration. On MRI, SFT has a signal characteristic consistent with any soft tissue tumor, with intermediate signal intensity on T1-weighted images and enhancement on T2-weighted images –14. TherDifferential diagnosis is made with other enhancing lesions within the parotid gland, especially with pleomorphic adenomas and mucoepidermoid carcinomas. Large pleomorphic adenomas may have lobulated or poorly defined margins, while high grade mucoepidermoid carcinomas are scantily defined with heterogeneous internal architecture and may have associated cervical adenopathy –14.Characteristically, SFT is a lobulated or nodular, firm, well-circumscribed, gray mass, surrounded by a pseudo-capsule, often with small satellite nodules separate from the main tumor –14. ReceHistological features associated with malignancy include high cellularity, pleomorphism, necrosis, high mitotic rate , and/or infiltrative margins –14. HoweSFT shows immunoreactivity with vimentin and CD34, the major part of tumors displaying also positive results for bcl-2 and CD99. CD34 is the only consistently expressed and sensitive marker in SFT –14. AbseImmunohistochemistry and histology help in the differential diagnosis from other parotid tumors: pleomorphic adenoma, myoepithelioma, fibrous histiocytoma, spindle cell squamous cell carcinoma, schwannoma, neurofibroma, fibrosarcoma, myofibroblastoma, meningioma, melanoma, Kaposi sarcoma, and synovial sarcoma –14.Traditionally, the treatment of SFT has been surgical resection with negative margins . Preoper


Primary malignant tumours of the chest wall are rare. Chondrosarcoma is the most common malignancy of the sternum. Chondrosarcoma derived from rib is rare. Wide resection treatment is important because it is resistant to chemotherapy and radiotherapy.We report a case of giant chondrosarcoma arising on the 6th rib of a 76-year-old man which is growing slowly for 20 years and is treated with chest wall resection and different reconstruction way.Examination of 76-year-old male patient with anemia revealed a giant mass at anterior side of the right hemithorax. The mass with the story of slowly growing for 20 years which causing pain in the last 15 days revealed no involvement in bone scintigraphy. His chest CT showed erosion and destruction of 6.rib and 11 × 7.8 cm mass with millimetric calcifications and intermediate indistinct borders of the pectoral muscles. True-cut biopsy revealed findings consistent with chondrosarcoma.Tumor was resected together with the left 5th, 6th, and 7th ribs, titanium bars, titanium clips were applied into the area of the ribs; pectoralis major and rectus abdominis advancement flap were replaced under the bar, than polypropylene mesh was placed over the bars and the reconstruction was completed with closing of skin. Patient was discharged on the 6th day, after uneventful postoperative follow-up. The pathologic report revealed low grade chondrosarcoma. He had no complaint of the chest at his 5th month outpatient controlFor chondrosarcoma with wide resection in the treatment 97% 5-year survival is reported. Reconstruction with Titanium bar, polypropylene mesh, and chest wall muscle advancement flap has excellent stability, flexibility, and rigidity and permits a rapid return to baseline pulmonary mechanics.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


AbstractPrimary torsion of the omentus is an extremely unusual cause of acute abdomen in the pediatric population. This condition occurs from twist of the pedicle of the omental apron around its longer axis, leading to edema, ischaemia and necrosis. Here we present a rare case of a 9 year old girl referred by her general practitioner due to severe right lower quadrant abdominal pain with a presumed diagnosis of acute appendiceal inflammation. Surgical operation disclosed primary omental torsion. The infarcted segment was resected and the girl’s clinical recovery was uneventful without any complication. The condition may mimic a variety of other causes of acute abdominal symptoms. In this case report, a presumed diagnosis of acute appendicitis urgently induced the decision of a surgical approach. Physicians involved in the acute pediatric care have to include this rare condition in the differential diagnosis of acute onset right-sided abdominal pain.  It is remarkable that Kimber et al, in a 20 years retrospective study among a paediatric population reported that the ratio of primary omental torsion to appendicitis was found less than 4/1000 respectively [3]. In a similar 10 year study in Greece this ratio was found 1/587 with overweight males to be mainly affected [2]. Primary omental torsion represents an extremely rare etiology of acute abdomen . Ultrasound (US) examination showed a small amount of free fluid in the pouch of Douglas. Visualization of the appendix was not feasible, while ovaries were found normal. Due to the increasing character of the abdominal pain, the girl was transferred to the operation room. Intraoperatively, during exploration of the peritoneal cavity was detected in the sero-sanguineous fluid. Appendix was found normal. Further exploration for Meckel’s diverticulum was negative. Torsion of the right omental part around its long axis (4]. In regard to pathogenesis, torsion of the omentum around a pivotal point impairs its vascular perfusion resulting to severe congestion and oedema . Spontaneous derotation may occur at this stage [6]. However, if the torsion continues, edema progresses to acute hemorrhagic infarction and omental necrosis [7]. Extravasation of a sero-sanguineous liquid into the peritoneal cavity is often detected during surgery [5]. Omental torsion was first described in 1899 by Eitel [1]. Secondary omental torsion occurs more frequently than the primary form and is associated with intra-abdominal inflammatory situations, tumours or cysts [8]. Morris et al reported that the majority of secondary omental torsion cases are presented in patients suffering from inguinal hernia [9]. Regarding the primary or idiopathic form, it occurs in the absence of any intra-abdominal disorder [5].  The condition according to its aetiology is further classified as primary or secondary to certain predisposing risk factors [1]. Intraoperatively, twist of the greater omentum around the right epiploic artery is often observed [10]. Increased mobility and length of the right side of the omentum may explain the increased prevalence of right omental infarction [11]. Factors that predispose a patient to torsion include congenital pathological variations of the omentum. Among these are reported bifid omentum which is an accessory omentum derived from a narrow route, abnormal embryological position of the right omental part with secondary fragile blood vessels [12] and irregular accumulation of omental fat [1]. Precipitating factors are those causing sudden omental displacement and include trauma, coughing, violent exercise, hyperperistalsis and compression between abdominal wall and the liver [1]. A certain causative mechanism for primary omental torsion has not yet been established [1]. Acute appendicitis, acute cholecystitis [5] and diverticulitis are conditions included in the differential diagnosis [1]. Right sided acute abdominal pain, fever, nausea and vomiting are the most frequently encountered symptoms at onset [13]. Physical examination reveals signs of peritoneal irritation with guarding and rebound abdominal tenderness [14]. A palpable abdominal mass has been also reported in rare cases [13]. Remarkably, patients with omental torsion share a more prolonged clinical course with less systematic manifestations compared to acute appendicitis [15]. Patients with omental torsion may present with a variety of non-specific symptoms and can mimic various aetiologies of acute abdomen [14], the pathology is rarely diagnosed preoperatively, making exploratory laparotomy the most optimal diagnostic and therapeutic modality [5]. Surgical management of the primary omental torsion includes resection of the affected omental part with or without appendectomy [16]. Laparoscopic approach has been reported to be a safe and effective option [14]. Although conservative management has been described in some reports, surgery has been recommended as the treatment of choice in order to prevent severe complications related to conservative therapy such as sepsis and intra-abdominal abscess formation [14].  Although, US and Computed Tomography imaging are useful tools that assist the diagnosis by excluding acute appendicitis, cholecystitis and diverticulitis [ In this case report, a presumed diagnosis of acute appendicitis urgently induced the decision of a surgical management. The intraoperative diagnosis was this of omental torsion. Describing, in detail, this episode of care which occurred in a 9 year old child, it is crucial to properly rule out causes of abdominal symptoms.We presented an unusual case of primary omental torsion in a non-obese 9 years old girl. Although accurate preoperative diagnosis is seldom feasible, physicians involved in the pediatric care have to consider this condition in cases of acute onset right-sided abdominal pain.