Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis. Primitive neuroectodermal tumors (PNETs) were first discovered by Hart and Earle in 1973  for evaluation of a mass in the right side of the vulva. Enucleation of the vulvar tumor was performed and the suspected diagnosis was hemangiopericytoma. The tumor exhibited characteristics of borderline malignancy and the patient was referred to Osaka City University Hospital  for additional therapy. Informed consent was obtained from the patient for the use of clinical information for education and research.The tissue excised at the previous hospital was histopathologically examined by the pathologist and the suspected diagnosis was PNET. There were no abnormal findings in the physical examination. Magnetic resonance imaging (MRI) and computed tomography did not reveal any residual tumor between the lungs and the vulva.Simple vulvectomy and resection of the inguinal lymph nodes were performed, showing no microscopic residual tumor presence. The patient underwent outpatient observation.Four years following the initial surgery, a mass in the right side of the vulva was observed. MRI imaging revealed a 3-cm mass in the right side of the vulva. T1-weighted images demonstrated low signal intensity and T2-weighted images demonstrated high signal intensity . The posHematoxylin and eosin-stained sections of the tumor showed a solid growth pattern. The tumor comprised small, round-to-oval nuclei. Homer-Wright rosettes were not observed .The tumor stained positively for MIC-2, synaptophysin, neuron-specific enolase (NSE) and neurofilament antibodies. The tumor cells were negative for periodic-acid Schiff, vimentin, desmin, chromogranin A, CD34, CD45 [leukocyte common antigen (LCA)], S100, α-smooth muscle actin (α-SMA) and CD117 antibodies .The patient was diagnosed with recurrent PNET that had originated in the vulva. Multidisciplinary therapy was administered. Combination chemotherapy, using vincristine, tetrahydropyranyl-adriamycin and cyclophosphamide (VAC), was administered and radiation therapy, ifosfamide chemotherapy and VAC therapy were subsequently performed. The only adverse event observed with this medical treatment was G3 myelosuppression. Following multidisciplinary therapy, the patient underwent further outpatient observation. To date, the patient remains alive with no evidence of disease four years following the final medical treatment.PNETs were first reported in 1918 by Stout  as small-SMA. However, the immunohistochemistry of PNETs is controversial and identification of the chromosomal translocations is effective in determining the diagnosis. The chimeric chromosomes associated with EWS/PNETs are EWS-friend leukemia virus integration 1 (FLI1) , EWS-ETS-related gene (ERG) , EWS-ETS variant 1 , EWS-EIAF  and EWS-FEV . All chimeric chromosomes exhibit EWS at the 5′-end and transcription factors of the ETS family, associated with cell proliferation, at the 3′-end. They are classified as EWS/ETS chimeric chromosomes due to this feature. EWS is present on chromosome 22 and gene expression of EWS is observed in various organs. EWS/ETS chimeric chromosomes take part in cell proliferation, through transcription or transformation activities, and affect cultured fibroblasts, thereby promoting neural and epithelial differentiation  ;22q24;q, EWS-ETSPoor prognostic factors in EWS/PNETs include lesions of the body trunk, patient ages of ≥15 years, tumor volumes of ≥200 ml, the presence of distant metastasis, high levels of serum lactate dehydrogenase, a poor response to chemotherapy and recurrence within two years. The most unfavorable prognostic factor is the presence of distant metastasis. Even with aggressive treatment, patients with metastases exhibit an ~20% chance of long-term survival . DespiteThere are several reports on the management of PNETs. The majority of PNETs are treated as EFTs. Multidisciplinary therapy is often performed for EFTs as recurrence is common. Prior to the advent of modern chemotherapy, <10% of patients with EFT survived beyond five years following diagnosis . The IntThe current case report presented a rare case of PNET originating in the vulva. Although PNETs usually exhibit a poor prognosis, the patient remains alive and with no evidence of disease. It is hypothesized that this may be due to the 3-cm tumor size and the absence of distant metastasis at the time of recurrence.


Introduction: Ewing’s sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4–9% of primary malignant bone tumors and it affects bones of the skull or face in only 1–4% of cases. Hence it rarely affects the head and neck. Subject and Method: In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results: Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion: Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing’s sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here. Primary Ewing's sarcomas occurring in the skull are exceptionally rare.3 The prognosis of Ewing's sarcoma is often poor because of early metastasis to the lungs or to other bones.4 However, a review of articles suggested that metastases are much less frequent and long term survival can be expected in Ewing’s sarcoma occurring in skull.  Ewing's sarcoma is a primary bone cancer that affects mainly children and adolescents. It's one of a group of cancers known collectively as the Ewing sarcoma family of tumors. It's the second most common bone cancer in children, but it's also relatively uncommon. It accounts for only 1% of all childhood cancers. Although it can occur at any age, it rarely occurs in adults over the age of thirty. Ewing's sarcoma represents 4% to 9% of primary malignant bone tumors Parents of a 9-month-old boy noticed a swelling in their infant’s right temporal region in November 2007. After three months he was referred to our hospital for indolent mass of skull, in initial physical examination cranial nerves were intact and there was no sign of lymphadenopathy and hepatosplenomegaly. A three centimeters mass was palpated beneath the surface of the skin in the right temporal region. The mass was solid, immobile, and non-tender. The skull films were not available but skull graphies showed a well-defined bony mass in right fronto-parietal region and recommended performing bone biopsy. Computed tomography (CT) scans revealed an intracranial, well-circumscribed, isodense mass, surrounded by a hypodense area and hyperostosis in the right temporal region adjacent to the tumor. Part of the tumor grew extra-cranially. The tumor was homogeneously enhanced by intravenous infusion of contrast medium; also brain parenchyma was intact . The results of initial paraclinical studies are listed below: WBC: 4400/dl , Hb: 10.4 mg/dl, Plt: 235000/dl, ESR: 18 mm/h. Biochemistry tests were normal.Tumors that show good differentiation are generally easy to diagnose, but when a tumor is poorly differentiated, identification, morphological features is more difficult. Bone needle biopsy was performed but results showed normal specimen, according to the X-ray report, normal needle bone biopsy and to acquire samples for IHC and cytogenetic, the patient underwent surgical removal of the tumor on February 2008. A brown-colored, solid mass was totally removed, along with hyperostotic temporal bone and overlying temporal muscle. The cortical surface was readily separated from the tumor in all parts. The tumor was composed of solid packed lobular round cell pattern of striking uniformity. The individual cells possessed a rounded or ovoid vesicular nucleus. The cytoplasm was ill-defined, scanty and pale staining .Immunostaining for NSE (Neuron Specific Enolase) and CD99 (Mic-2) were positive. RNA was analyzed for t  by reverse transcription followed by RT-PCR method, result was negative for t  , EWS-FLI1 fusion transcript.  These findings were compatible with a diagnosis of Ewing's sarcoma. Other evaluations including chest, abdomen and pelvis computed tomography scan were normal. Whole body bone scan revealed increase uptake in temporal bone. After surgery, chemotherapy was begun by port cat system. The postoperative course was uneventful. Chemotherapy included Vincristine 2 mg/m², Doxorubicin 75 mg/m², Cyclophosphamide 1200 mg/m² followed by MESNA alternating with Ifosfamide 1800 mg/m²/day for five days and Etoposide 100 mg/m²/day for five days (We adjusted 25% doses of drugs till he became one year old). The courses are administered every three weeks for total 17 courses with planned treatment duration of 49 weeks. Doxorubicin is substituted by Actinomycin D 1.25 mg/m²/dose when total Doxorubicin dose is reached to 375 mg/m² (after 5 cycles). Radiotherapy was not done because patient was less than 2 years old. Ten months after surgery, the evidence for re-growth of tumor or distant metastasis was negative. After 18 months of the last therapy, our patient referred to hospital because of pneumonia infection. Due to its infection, antibiotic therapy was started for him but in spite of all efforts he was expired within a few days. The primary location of Ewing's sarcoma is generally the long bones or the trunk bones. These tumors tend to break through the cortex of the bone of origin into the adjacent soft tissues.5The majority of patients with Ewing's sarcoma complain of pain and swelling in the area of the tumor. Compromised function of the affected part, fever, tenderness, local heat, and a palpable mass are also encountered.6 The skull is a rare primary site for Ewing's sarcoma.7 Sixteen cases of primary Ewing's sarcoma of the cranium have been presented in the literature. 6,7,8,9The duration of symptoms before presentation ranges from two weeks to two years.10 Vaccani et al. reported 70 cases of Ewing’s sarcoma between 1986 - 1996, among them five cases suffered from Ewing's sarcoma of head and neck (7.1 %) and the age of presentation ranged from 7.5 to 14 years. An enlarging mass in the mandible is the most frequent presentation, in none of them temporal bone was involved. Three of five patients died of metastatic disease and two are alive with no evidence of disease.5 Kuzeyli et al. reported a primary Ewing sarcoma of temporal bone in a case of ten years-old girl. She presented with palpable mass on the right fronto-temporal region and proptosis of the right eye but six months later she died because of lung metastases. Ewing’s sarcoma is more common in second decade of life and temporal bone involvement is very rare. Ernest et al. reported one case of Ewing’s sarcoma in a seven months-old white girl in right leg. The mass was not mobile and appeared to arise definitely from the tibia, not from the overlying soft tissue. No metastatic lesions were noted in lungs fields. She deteriorated rapidly and expired six month after the first appearance of her symptoms.Our case due to age of the patient and location of the tumor is a very unique one in literature.   Although according to the young age treatment was so hard and radiotherapy was excluded due to its side effect and also chemotherapy has more dangerous side effects, in consequence Ewing’s sarcoma has poor prognosis in less than one year-old children. Our case has no evidence of recurrence and metastasis after ten months and the therapy was suitable for him but unfortunately he was expired after 18 months because of pneumonia that was resistant to antibacterial therapies.


Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. Its trademark is a unique tX;18) chromosomal translocation resulting in SYT-SSX fusion protein. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh [8 for local tumor control. Three weeks after completion of radiotherapy, he had another operation for excision of the residual tumor and cranioplasty. Intraoperatively, multiple lobulated cysts were seen and removed. Gross total resection of the tumor was achieved; a wire mesh was placed over the right parietal bony defect. The patient recovered quickly and went home on postoperative day 3. Two months later, the patient received 3 cycles of adjuvant chemotherapy (AIM regimen) consisting of doxorubicin (adriamycin), ifosfamide, and mesna. Since then, the patient has been followed up closely in the clinic with MRI scans every 3 months. He continued to be neurologically intact without any evidence of tumor recurrence two years after the chemotherapy.Sarcoma is generally categorized into bone and soft tissue sarcoma. Synovial sarcoma is a type of soft tissue sarcoma that occurs mainly in adolescents and young adults between the ages of 15 and 30 years, with a slight male predominance . The neoSimilar to other soft tissue sarcomas, the most common initial symptom of synovial sarcoma is an enlarging soft tissue mass . FrequenThe standard treatment for local synovial sarcoma is surgical resection with wide margins . AdjuvanSynovial sarcoma is a malignant neoplasm. Rare instances of intracranial disease have been attributed to metastasis. Our patient is the first known case of intracranial disease without obvious primary extracranial pathology, suggesting that the primary disease arose from the brain. We also noted the hemorrhagic complication associated with brain lesions. In the case of sudden neurological deterioration, it is important to be cognizant of the tendency for intracranial lesions to hemorrhage, requiring urgent lifesaving decompression.


Leydig cell testicular tumors are a rare cause of precocious pseudopuberty in boys. Surgery is the main therapy and shows good overall prognosis. The physical signs of precocious puberty are expected to disappear shortly after surgical removal of the mass. We report two children, 7.5 and 7.7 year-old boys, who underwent testis-sparing surgery for a Leydig cell testicular tumor causing precocious pseudopuberty. During follow-up, after an immediate clinical and laboratory regression, both boys presented signs of precocious puberty and ultimately developed central precocious puberty. They were successfully treated with gonadotropin-releasing hormone (GnRH) analogs. Only six other cases have been described regarding the development of central precocious puberty after successful treatment of a Leydig cell tumor causing precocious pseudopuberty. Gonadotropin-dependent precocious puberty should be considered in children treated for a Leydig cell tumor presenting persistent or recurrent physical signs of puberty activation. In such cases, therapy with GnRH analogs appears to be the most effective medical treatment. Central precocious puberty is caused by premature activation of the hypothalamic–pituitary–gonadal axis, while precocious pseudopuberty results from peripheral over production of sex steroids –9. Leydileft testis 4 mL, right testis 6 mL) and an accelerated growth velocity. Pubertal stage has been performed according to Tanner-Marshall method on physical examination (left and right testis 8 mL). Recurrent erections and ejaculations were observed. Bone age was accelerated to 12.5 years. Ultrasonographic examinations ruled out testicular tumor recurrence and brain magnetic resonance imaging (MRI) excluded a tumor of the hypothalamus or pituitary gland. The patient showed a pubertal response to GnRH stimulation test and the hormonal values are reported in Table The patient was first evaluated at our Paediatric Endocrinology Department. Aged 7.5 years with a history of progressive appearance of secondary sexual characteristics for 4 months. He had pubic hair (Tanner stage PH2), enlarged testicular volume . Both testes were homogeneous and had no palpable masses. The penis was 12.5 cm long (left and right testis 9 mL). The patient manifested increased erections and ejaculations. X-ray examination revealed a bone age of 12 years. Brain MRI ruled out a tumor of the hypothalamus or pituitary gland and ultrasound excluded a recurrence of the testicular mass. His hormonal values confirmed central precocious puberty (Table The patient (aged 7.7 years) was evaluated because of progressive appearance of pubic hair and an accelerated growth velocity during the previous 6 months. He presented pubic hair (Tanner stage PH2) and an increased testicular volume . A testosterone-producing testicular tumor was identified as the cause of sexual precocity. It is interesting to underline that our patients developed gonadotropin-dependent precocious puberty, associated with accelerated growth and bone maturation, after surgical therapy. Only six other cases of central precocious puberty after initial treatment of a Leydig cell tumor, causing precocious pseudopuberty, have been described. This evolution may be more common than expected and should be considered in children with persistent or recurrent symptoms of precocious puberty after successful therapy. This phenomenon has also been observed in children with congenital adrenal hyperplasia, familial male precocious puberty, and McCune Albright syndrome . AlthougGnRH analog therapy is the most effective treatment for central precocious puberty. Progressive pubertal development should be documented over a 3- to 6-month period before GnRH analog treatment is initiated as this therapy is not always necessary. A surveillance period is useful to demonstrate the persistence of central stimulation and to detect patients that might benefit from treatment (In Tables In 1986, Criscuolo et al. presented a case of a 6-year-old boy with precocious pseudopuberty due to a Leydig cell tumor. After surgery, the hormonal profile progressively returned to the prepuberal range and sexual precocity disappeared. Six months later, the patient developed central precocious puberty, and was treated with cyproterone acetate . The secThe last case described  is an 8-In conclusion, beside our two patients, only six other cases of central precocious puberty after successful treatment of a Leydig cell tumor, causing precocious pseudopuberty, have been described. We would like to underline that, even though it is a rare condition, gonadotropin-dependent precocious puberty should be considered in patients who have been successfully treated for a Leydig cell tumor and who present persistent or recurrent physical signs of sexual precocity. This can be caused by high levels of testosterone and other sex steroids that can trigger gonadotropin-dependent puberty.GnRH analog treatment appears to be the most effective medical therapy available for progressive central precocious puberty.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Vulvar lipoma is a rare tumor localization and only a few cases have been reported. The clinical characteristics of vulvar lipoma are well known. However, it is important to distinguish lipomas from liposarcomas. We report a case of vulvar lipoma and discuss its clinical features, including diagnostic aspects, with emphasis on histopathological evaluation of all excised lesions. We also report and discuss patient management and treatment outcomes.We report the case of a 27-year-old Moroccan woman. Our patient presented with a painless and slow-growing right vulvar mass that had evolved over one year, which had suddenly become uncomfortable when walking. A physical examination revealed a single soft and pasty mass in her left labium majus, which could be mobilized under her skin towards her mons pubis. The largest dimension of the mass measured 6cm. Magnetic resonance imaging showed a homogenous hyperintense mass with a well-defined contour in her left labium majus; a fat-suppressed magnetic resonance image demonstrated a marked signal intensity decrease. The mass was completely removed surgically. A histological examination revealed a circumscribed benign tumor composed of mature adipocytes, confirming the diagnosis of vulvar lipoma.Vulvar lipomas must be differentiated from liposarcomas, which demonstrate very similar clinical and imaging profiles. The final diagnosis should be based on histopathological evaluation. A precise diagnosis should allow for appropriate surgical treatment. Lipomas are widely disseminated benign mesenchymal neoplasms commonly found over the neck and upper back, shoulders, abdomen, buttocks, and proximal portions of the extremities . Vulva lWe report the case of a 27-year-old Moroccan woman, who presented with a painless and slow-growing right vulvar mass that evolved over one year. Our patient reported a sudden, uncomfortable feeling during walking. A physical examination revealed a single soft and pasty mass in her left labium majus. The mass could be mobilized under her skin towards her mons pubis. The largest dimension of the mass measured 6cm. However, there was no visible palpable cough impulse or inguinal lymphadenopathy, while a physical pelvic examination was normal.Magnetic resonance imaging (MRI) was performed Figures  and 2, wLipomas are very common benign tumors in soft tissues derived from mesenchymal cells . They haLipomas usually present as single or multiple painless slowly growing and mobile swelling soft tissue with a pasty characteristic. The tumor shows ill-defined, well-demarcated, or pedunculated aspects that are not adherent to the overlying skin. These characteristics allow correct diagnosis in most cases by clinical examination -6. Howevet al. showed that septal enhancement in contrast-enhanced MRI allows the differentiation of liposarcoma [In developing countries, ultrasound is recommended instead of expensive imaging investigation because of its availability and cost-effectiveness . Vulvar osarcoma ; they foosarcoma . Therefoosarcoma ,9.Treatment for a lipoma without excision includes steroid injection and liposuction and is common. However, complete surgical excision is the treatment of choice for vulvar lipoma . SurgeryVulvar lipomas need to be differentiated from liposarcomas, which demonstrate very similar clinical and imaging profiles. The final diagnosis should be based on histopathological evaluation. A precise diagnosis should allow for appropriate surgical treatment.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SJ was the principal author and major contributor in writing the manuscript. ML reviewed the literature; HEF performed the histological study; AA participated in the histological study; FZF, HB and HC analyzed and interpreted the data from our patient; MAM corrected the manuscript. All authors read and approved the final manuscript.


Primary cardiac sarcomas are quite uncommon. Among them, sarcomas originating from the mitral valve are exceedingly rare. They are often misdiagnosed due to non-specific symptoms. The prognosis of cardiac sarcomas remains poor, and finding a more effective therapy poses a big challenge for the doctors. Currently, complete surgical resection of the tumor is still the most popular treatment in cases that without metastases.A 38-year-old female was transported to our department with persistent cough and chest pain. The operation of mitral valve replacement was scheduled after a provisional diagnosis of serious mitral stenosis. During the operation, we found that a large polypoid mass infiltrating mainly the mitral valve and part of the left atria. We removed the tumor as completely as possible and replaced the mitral valve. Histology confirmed the diagnosis of undifferentiated sarcoma after operation. The patient was asymptomatic with no recurrence at 3-month follow-up.Primary cardiac sarcomas is a rare and the prognosis is poor. The sarcomas is often found by chance during the operation. Surgical operation need to be carried out directly. Primary cardiac tumors are rare with approximately 25 % of them are malignant. Sarcomas account for 75 % of the cardiac malignant neoplasms . CardiacDue to rapidly proliferating and distant metastases, the prognosis of sarcomas is poor. The median survival of patients without metastasis is 15 months, in contrast to 5 months for those with metastasis . We presA 38-year-old female presented with 4 months history of persistent cough with shortness of breath and chest pain. Laboratory tests and physical examination were unremarkable. Chest × ray suggested the pulmonary congestion. Electrocardiography revealed sinus rhythm. The morphological changes of the mitral valve revealed by echocardiography included leaflet and subvalvular apparatus thickening, shortened chordae tendineae, and commissural fusion. The tricuspid valve was intact and right ventricular and atrial sizes were normal under echocardiography.2 orifice area. In addition, part of the left atrium was also involved with the same lesion. According to the character of the mass, we considered it to be heart sarcoma. After a complete resection of the leaflets and chordae tendineae and a partial resection of papillary muscle [Fig. The operation of mitral valve replacement was scheduled after a provisional diagnosis of serious mitral stenosis. A traditional median longitudinal sternotomy was implemented. After full heparinization, cardiopulmonary bypass (CPB) was routinely applied, and cold cardioplegic solution was instilled via the aortic root after cross clamping the aortic. Upon the opening of the left atrium by longitudinal incision, we observed unexpectedly that a large polypoid mass infiltrated the entire anterior mitral leaflet and part of posterior mitral leaflet, resulting in a 0.5 cmcle Fig. , we exciOn immunohistochemical examination, the tumor cells stained positive for CD34, CD31, vimentin and WT1 but negative for CK(AE1/AE3), Desmin, Calretinin, CK5/6, S-100 and HMB45. Histology confirmed the diagnosis of undifferentiated sarcoma Fig. .Fig. 2HiUpon reviewing of current literature, we discovered that primary sarcomas were occasionally found in the left atrium, left ventricle and right atrium with undifferentiated sarcomas usually found in left atrium. However, the tumor originating from mitral valve and infiltrating the left atrium has not been reported. In this study, we report a case with primary sarcoma infiltrating mainly mitral valve, suggesting that the origin of the tumor was most likely the mitral valve. Considering of the possible origin of the sarcoma, we focused on dealing with the primary lesion during the surgical treatment. Before replacement of mitral valve, electrotome burning of the remains of the mitral valve and part of the left atrium infiltrated by the sarcoma was implemented to avoid recurrence.Although the true diagnosis of primary cardiac sarcoma is often made as an unexpected finding during the operation, the development of non-invasive cardiac imaging such as Transthoracic Echocardiography (TTE) and Transesophageal Echocardiography (TEE) has vital value in the preoperative assessment of cardiac sarcoma . HoweverStandard treatments for primary cardiac sarcomas have not been defined yet due to their low incidence. There are currently many treatment options such as surgical excision, radiotherapy, chemotherapy, heart transplantation and complex treatment. Among these managements, completely surgical excision is the most popular therapy that has shown to prolong the survival . The effThe current study reported a rare case of primary cardiac sarcoma that possibly originating from mitral valve and infiltrating part of left atrium. The tumor was completely excised through surgery and the patient was recovered without complications. Since the tumor infiltrated both the mitral valve and part of left atrium, the true origin of the tumor is still a puzzle, which is the limitation of this study.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.


Introduction. Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare malignant adnexal tumor, which arises from syringocystadenoma papilliferum. To date, less than 30 cases of malignant SCACP have been reported, of which locoregional metastases were found in only four cases. Case Report. A 57-year-old female patient who presented to our Oncology Department with a recurrent malignant SCACP of the left labia along with right inguinal lymphadenopathy. Pathological examination confirmed the diagnosis of malignant SCACP with right inguinal lymph node metastases. Due to the fixity of the right inguinal nodes, neoadjuvant chemotherapy was administered with Cisplatin and 5-Fluorouracil for four cycles, following which the primary tumor and the contralateral inguinal nodes regressed completely. Then definitive chemoradiation was delivered with five cycles of weekly Cisplatin and external beam pelvic irradiation up to a dose of 59.4 Gy. Patient is disease-free 11 months after treatment. Discussion. We here report the fifth case of malignant SCACP with locoregional metastases. This is the first case of malignant SCACP which has been treated with neoadjuvant chemotherapy followed by concurrent chemoradiation. Although surgery has been used most commonly, chemoradiation may also have a role in the treatment of malignant SCACP, especially in cases of locoregional metastases. Syringocystadenocarcinoma papilliferum (SCACP) is a very rare entity of cutaneous adnexal tumor, which is listed in the WHO classification of skin tumors as the malignant counterpart of syringocystadenoma papilliferum (SCAP) , 2. SCAPA 57-year-old woman presented to a private practitioner with a swelling in the left labial region in last three years, which was now associated with bleeding, foul smelling discharge, and pruritus since last one month. There were no enlarged inguinal nodes at the time of presentation. She underwent wide local excision alone and was diagnosed with SCACP. Postoperative margin status was negative. Three months postoperatively, she presented to our institutional Oncology OPD with an ulcerative lesion in the left labial region along with right inguinal lymphadenopathy.Histopathological examination of the ulcerative lesion in the left labia showed epidermis comprising of keratinized squamous epithelium and dermis showing a tumor extending from lower epidermis into deep dermis. The tumor was made up of multiple nodules comprising of duct-like structures, some appearing to be cystically dilated, papillary structures having central fibrovascular cores, solid nests, and islands . Dense i2 and 5-Fluorouracil of 1000 mg/m2 was given for four cycles. Following this, the primary tumor and the contralateral inguinal nodes regressed completely (Figures 2 and external beam pelvic irradiation up to a dose of 59.4 Gy in 33 fractions. After a dose of 50.4 Gy to the whole pelvis, a boost dose of 9 Gy to the primary tumor site and the inguinal lymph nodal site along with a 2 cm margin was delivered. Patient has been followed up regularly and is disease-free 11 months after treatment.Magnetic resonance imaging of the pelvis demonstrated an irregular, intermediate signal intensity lesion in the left side of the labia of size of 10 mm × 10 mm along with multiple oval to round lymph nodal masses in the right superficial inguinal region measuring 5.3 cm × 7.5 cm in size Figures . Owing t Figures . After tWe describe a rare case of SCACP in vulva with locoregional metastases. A total of less than 30 cases of SCACP have been described in literature till date , 8. The The histopathological diagnosis of SCACP is sometimes difficult and may resemble hidradenocarcinoma papilliferum, apocrine ductal adenocarcinoma, and cutaneous metastases of malignancies microscopically . While hSCACP is generally a low-grade malignancy, with only two cases of recurrence being reported , 9. LocoDue to the infrequent occurrence of the tumor, no standard treatment protocol has been advocated , 14. WidIn the current report, the patient had SCACP with locoregional failure postoperatively, which responded to chemotherapy followed by chemoradiation and is without any recurrence or distant metastases for 11 months after treatment.SCACP is an extremely rare adnexal tumor which may occur in unusual sites like vulva. Locoregional lymphatic metastases, though infrequent, should always be investigated for. Though, as of now, wide local excision is the standard treatment, chemotherapy and radiation therapy can also achieve a good tumor control in cases of inoperable SCACP or in patients declining surgery. The role of chemoradiation has to be further investigated, especially in cases of locoregional disease spread. Regular follow-up is utterly important to detect any locoregional failure or recurrence early, as aggressive treatment may achieve good tumor control.


We report the case of a patient who developed a vesicoovarian fistula on an endometriosis abscessed cyst. The patient presented with an advanced endometriosis stage IV complicated with a right ovarian abscessed cyst of 10 cm. A first coelioscopy with cystectomy was realized. After surgery, a voiding cystography highlighted a fistula between the ovarian abscess and the bladder. A second surgery by median laparotomy was realized with the resection of the right ovarian abscess and the resection of vesical fistula. Numerous complications of the endometriosis exist. The vesicoovarian fistula is a very rare case; only two cases have been documented. We are going to present the case of a patient who developed a vesicoovarian fistula on an endometriosis abscessed cyst. in vitro fertilization.A 31-year-old patient was treated by the gynecology obstetrics department for a 10 cm right ovarian endometriosis cyst before going intoShe presented with in her medical history a uterine malformation with a complete compartmentalized uterus and a complete vaginal partition, an ankylosis spondylitis (AS), and endometriosis. She had two laparoscopies in 2007 as part of her infertility check-up. The first one in June showed a severe endometriosis stage IV with ice-cold pelvis and a lot of adhesions. Nothing was done during this surgery, and a second surgery was subsequently performed in December after six months of treatment by LHRH analogs.This second surgery resulted in adhesiolysis and an incomplete resection of bilateral endometriosis cysts, confirmed by histopathology. in vitro fertilization (IVF). She had an urgent C-section in 2009 at 36GW + 3 days due to a premature rupture of membranes with beginning of a maternofoetal infection. In February 2012, she had a second trial of IVF treatment hoping for a second child.The patient was in the medical assisted procreation (MAP) program. She was expecting at the end of the second attempt ofDuring the fertilization check-up, a 10 cm right ovarian endometriosis cyst was discovered. The patient was sent for cystectomy before IVF. She had an asymptomatic recurrent urinary infection and turbid urines for several months. She did not have abdominal pain or fever. She denies dysmenorrhea or dyspareunia. Elevated C-reactive protein (CRP) was attributed to her periodic inflammatory reactions of AS.The preoperative magnetic resonance imaging (MRI) showed a 11 × 9 cms endometriosis cyst of the right ovary, with a doubtful fistula in the bladder on its left side .A right hydrosalpinx of 6 mm was also identified below the voluminous cyst. Numerous adhesions and a deep endometriosis invading the two-uterosacral ligaments had been found. A cystoscopy was performed before surgery but no fistula was found. E. coli.The patient was treated with LHRH analogs and a coelioscopy was scheduled in January 2013. The patient had no fever. Her blood test was normal with 7880 white blood cells but the culture of the CBEU turned out to beUpon catheterization, the patient still had cloudy urines. During the laparoscopy, numerous adhesions were identified, preventing a complete exploration. During the right ovary cystectomy, there was a leakage of fluid.The cyst was partially resected and the abscess was drained. Escherichia coli which was sensitive to ofloxacin. An appendectomy was performed at the same time. No fistula in the bladder was visualized. The patient was initially put under quadriantibiotic treatment . The postoperative blood test found 9300 white blood cells of which 83% were neutrophils and her CRP was 114. The patient was discharged after three days with a protocol of antibiotics. Twenty-two days after being released, she came back for her follow-up. Her follow-up CRP, 22 days after discharge, was 380 and she still had cloudy urines. No symptoms of fever were found.Samples were sent to bacteriology. Her peritoneal fluid hadA retrograde cystography highlighted a fistula between the abscess, which had been reconstituted (measuring 8.5 cm), and the bladder .The control CBEU did not find bacteria in the direct examination or in the culture. A new intervention by median laparotomy was scheduled in April 2013. At this time, she did not undergo hormonal treatment before the surgery.Upon exploration numerous adhesions were found between the sigmoid colon and the cyst. It completely covers the intestinal loops, the small intestine, and cecum. There were also severe adhesions from sigmoid area up to the posterior pelvic wall due to severe persistent endometriosis; Douglas' pouch appears shielded by these adhesions.After a difficult adhesiolysis, the uterus, the sigmoid, the right ovary, and the bladder were identified and visualized. An injection of a blue dye confirmed the presence of a very small caliber fistula about 1-2 mm, located at the top left quadrant of the bladder. It is a fistula of very small caliber to about 1-2 mm oblique pinhead. The intervention allowed the resection of the right ovarian abscess and the debridement of the vesical fistula. A mobilization of the bladder was done after the opening of the retropubic space. Finally, a withdrawal of a 1 cm bladder patch and suture of the bladder in 2 plans were performed. Suction and drainage had been implemented.Histopathological findings were performed on the right ovarian endometriosis cyst and the bladder fistula.The surgery was uneventful with no complications. The patient was discharged after removal of the Foley catheter, eight days after surgery. The uroscan was normal. Her urine evidently became clear. No abscess or infection was noted.The vesicoovarian fistula is an exceptional case. Only two cases have been reported. The first case was a fistula caused by an ovarian abscess linked to salpingitis in 1990 [The second case was described in Japan in 1997  wherein Acystoscopy revealed a mass at the posterior wall of the bladder and the MRI showed a fistula between this mass and the bladder.The anatomopathology reported that the fistula was caused by endometriosis.The case that we reported is an atypical case due to its poor symptomatology. Indeed, the patient had no fever and did not present any pelvic pain.There was only an isolated increase of CRP which had been attributed to inflammatory response of AS.Furthermore, the diagnosis of fistula was difficult to make. The preoperative cystoscopy did not demonstrate the presence of the fistula, neither did the laparoscopy. The MRI was suspicious for the presence of a fistula but the diagnosis was confirmed by a voiding cystography. Fistulas are difficult to diagnose, especially when they are very small. The voiding cystography remains the gold standard of diagnosis. This procedure gives pressure to the bladder and highlights fistulas of very small caliber.The cause of the fistula is uncertain in our case. Was the fistula due to ovarian abscess draining of the surrounding organs, in this case, the bladder? Or was the fistula due to endometriosis complications by an abscess as a consequence of the urine contamination?Furthermore, the origin of the abscess remains unclear: was it connected to the draining of the cyst for the IVF two years previously? Or did it result from a spontaneous secondary infection of the endometriosis cyst, without any iatrogenic intervention associated?The vesical fistulas in the bladder in female patients are mostly iatrogenic, as a result of a medical history of pelvic surgery, in particular C-sections for vesicouterine fistulas or radiotherapy . InfectiEndometriosis fistulas are hormone dependent. It was reported that some rare cases of vesicouterine fistulas close after a medical treatment by LHRH analogs . Indeed,Surgical treatment with the abscess resection and the repair of the fistula was the only treatment that allowed complete recovery for the patient. Surgery remains the treatment of choice for endometriosis fistulas. If the laparoscopy did not seem appropriate in our case because of the very small size of the fistula, this surgical technique is recommended as a first intervention for the treatment of endometriosis fistulas to avoid the development and the extension of the fibrosis . HoweverSome risk factors of recurrence of fistulas after surgical treatment had been identified: advanced endometriosis (stage III or IV), advanced age patients, presence of many pelvic adhesions, larger endometriomas or deep nodules, a failure of previous medical treatment, or an incomplete surgical resection –10.We reported a very rare case of endometriosis complications with a vesicoovarian fistula. Although fistulas are rare, it is necessary to remain attentive to symptoms that make the diagnosis possible, to be able to ensure adequate treatment. To avoid recurrences and complications, a surgical complete excision is recommended in these cases.


Solitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. While they frequently arise from the pleura, they can occur at any soft tissue site in the body. We present a case of a large (28 × 21cm) malignant solitary fibrous tumor arising from the bladder serosa. In addition, the clinicopathologic features, differential diagnosis, cytogenetics and management of this rare disease are discussed, along with a review of the existing literature on this topic.An otherwise healthy 41-year-old Caucasian man presented with weight loss and progressive abdominal bloating. A subsequent computed tomography scan of his chest, abdomen and pelvis revealed a 26.8 × 21cm intra-abdominal mass occupying most of his abdominal cavity. The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder. He underwent operative resection and the resected mass measured 28 × 21 × 18cm and weighed 4.8kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern, with randomly assorted tan fleshy nodules. A histologic evaluation revealed variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma. In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity. The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder. Our patient is currently eight months post-surgery without evidence of recurrence.Extrapleural occurrences of solitary fibrosis tumors are being increasingly observed. Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare. As there are no pathognomonic features of malignancy, surgical resection is often both diagnostic and therapeutic, as was the case in our report. In 1942 Stout and Murray first described and defined the hemangiopericytoma (HPC). They described a vascular tumor made of contractile spindle cells surrounding the capillaries . SecondlIn fact, extrapleural SFTs are now more commonly found than intrathoracic SFTs . SpecifiA 41-year-old otherwise healthy male presented to the emergency department at Hakensack University Medical Center, NJ with abdominal pain and abdominal fullness that had progressively worsened over the course of the last year. He also reported constipation, urinary frequency, dyspnea on exertion and a 25-pound weight loss. A subsequent computed tomography (CT) scan of his chest, abdomen and pelvis revealed a 26.8 × 21cm intra-abdominal mass occupying most of his abdominal cavity . The midline laparotomy was closed in routine fashion.The abdomen was entered through a midline incision. The liver and peritoneal surfaces were uninvolved. The mass did not appear to be arising from the retroperitoneum and was noted to be quite mobile, except in the pelvis where it was tethered to the superior dome of the bladder. The mass was freed from the lateral pelvic side walls and a partial cystectomy was performed On gross inspection, the mass measured 28 × 21 × 18cm and weighed 4.8kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern with randomly assorted tan fleshy nodules Figure a-b. In tHe had an uneventful post-operative course and was discharged from the hospital on post-operative day five. He was seen in the clinic at one, three and eight months following discharge and is recovering well without evidence of recurrence.Extrapleural SFTs are most commonly diagnosed between the fifth and seventh decades of life. They are typically slow growing and asymptomatic. Symptomatic tumors are typically secondary to the locally invasive nature of the tumor and compression and/or impingement of nearby structures. Common symptoms seen with intra-abdominal and pelvic SFTs include pain, palpable mass, abdominal distention, urinary retention, hematuria, constipation and bowel obstruction . OverallBecause of the wide variety in clinical presentation, the work-up and diagnosis of SFT can be challenging. Although non-specific, certain imaging characteristics have been observed. A sonography can reveal a hypo- or heterogeneous echogenic mass with well-defined margins. A CT scan typically shows a well-enhanced, circumscribed heterogeneous mass. Prominent vasculature due to mass effect and calcification can be observed in larger lesions, as seen in our case . RegionsNAB2 gene which indirectly represses transforming growth factor (TGF) - β, and the STAT6 gene which is a transcriptional factor that modulates signaling through interleukin-4 and interleukin-13, have both been described as oncogenic. A recent study of 53 patients undergoing whole exome tumor sequencing revealed that when fused, these two genes (located on chromosome 12) represent a distinct molecular feature in SFT. This characteristic fusion transcript occurred in 55% of tumors, creating the opportunity to develop drugs that specifically target the fusion gene product. Interestingly, a cytogenetic analysis did not reveal the NAB2-STAT6 fusion in our patient [STAT6 and GRIA2 was also negative. An analysis of 44 SFTs by Mohajeri et al. revealed that in addition to the NAB2-STAT6 fusion transcript, GRIA2 was the top up-regulated protein found by immunohistochemistry using tissue microarrays [Macroscopic examination of solitary fibrous tumors typically reveals a well-circumscribed, tan-colored rubbery mass with a white whorled appearance on cut sections. The mass is often tethered by a pedicle and encapsulated. Most cases of bladder SFT are described as being intravesicular, growing from the submucosa, however there have been isolated cases of pelvic SFT growing from the serosal surface of the bladder, as was seen with our patient. Microscopically, SFT is classically described as having a ‘patternless pattern’, with juxtaposed hypercellular (spindle to ovoid cells) and hypocellular zones  and a prominent branching vasculature ,7. Mitos patient . Moreoveroarrays .Malignant criteria for SFTs include large tumor size (>10cm), hypercellularity, nuclear atypia, tumor necrosis, more than four mitoses per 10 high power fields and infiltrative margins . HoweverBecause of the variable clinical behavior and unpredictable nature of SFTs, surgery is considered the treatment of choice. Furthermore, long-term follow-up is strongly recommended for all cases of SFT, although no specific surveillance strategy has proved to be superior. Surgical resectability is the most important prognostic factor, and the five-year survival rate is high, with some authors claiming close to 100% with complete surgical excision (R0 resection) . For unrWe report the case of a 41-year-old man who presented with the largest documented case of malignant SFT arising from the urinary bladder. While there are characteristic findings suggestive of SFT, no gold standard diagnostic test currently exists. As such, surgical excision is both therapeutic and diagnostic when combined with subsequent histological, immunohistochemical and genomic testing.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.


Hemangiopericytoma is a rare primary tumor originating from Zimmerman's pericytes, with significant metastatic potential. Hepatic metastatic disease requires an aggressive approach by a multidisciplinary team of dedicated oncology specialists, to prolong survival in selected patients. We report on a patient with recurrent hepatic metastases of grade II intracranial hemangiopericytoma 5 years after initial treatment, managed by a stepwise combination of liver resection, radiofrequency ablation, and transarterial embolization. Although metastatic disease implies hematogenous dissemination, long-term survival after liver resection has been reported and major hepatectomies are justified in patients with adequate local control. Liver resections combined with transarterial embolization are highly recommended, due to hypervascularity of the tumor. Noncolorectal, nonneuroendocrine hepatic metastases are a diverse group of secondary liver neoplasias, exhibiting variable clinical behavior and characteristics. Particularly in cases of rare primary tumors, where limited datasets are available and no official guidelines are published, a multidisciplinary team (MDT) approach is mandated, to provide comprehensive assessment, consultation, and treatment.We present our experience with a case of recurrent hepatic metastases from an intracranial hemangiopericytoma (HPC), managed by an MDT of oncologic surgeons, medical oncologists, interventional radiologists, and dedicated pathologists.A 23-year-old Caucasian male underwent a right temporal lobe glomus tumor excision. Seven years later he was diagnosed with local recurrence and underwent two craniotomies to achieve radical resection . HistopaαFP, and Ca19-9) were within normal limits.Five years after the last intervention, he was admitted to hospital due to diverticulitis and an incidental finding of a small lesion in the body of T10 vertebra was demonstrated on CT scans, as well as multiple, atypical, hypervascular lesions in both liver lobes, the larger being 8 cm in diameter, initially considered hemangiomas Figures  and 3. MA right hepatectomy plus radiofrequency ablation of one lesion in the left lobe was decided and the patient had an uneventful postoperative course. Histopathology revealed two tumors (8.7 cm and 3.8 cm) consisting of relatively bland mesenchymal cells, with no unique characteristics, packed around an elaborate network of vessels Figures  and 5, cOne year after liver resection, follow-up CT scan revealed no local recurrence in the brain cavity, but three metastatic lesions in the liver parenchyma (segments II and IV) and slow progression of the T10 lesion. The MDT proposed stereotactic radiosurgery for the bone metastasis and elective transarterial embolization (TAE) of the liver lesions, as bridging therapy to a second hepatectomy. Two of the liver lesions were hypervascular, although the caudate lobe metastasis was only slightly vascular with a capillary network. Transarterial embolization was successful, due to hyperselective arterial catheterization Figures  and 8.Hemangiopericytoma is a rare mesenchymal tumor, originating from Zimmerman's pericytes, which are contractile spindle cells, surrounding capillaries and postcapillary venules, and regulate capillary flow and permeability . Most coGenerally HPCs are aggressive tumors, with 5-year local recurrence rate of 65% and 5-year distant metastasis rate of 33%, with lung, liver, and bones being the most frequent metastatic locations [Clinical symptoms are vague and nonspecific, depending on tumor location and size . Pain isHypervascularity is the prominent imaging characteristic and preoperative differential diagnosis from other vascular lesions is necessary, due to differences in treatment and prognosis. Unenhanced liver ultrasound reveals hypoechoic lesions, which become markedly hyperechoic during the arterial phase of contrast medium administration . On CT sMagnetic resonance imaging reveals well-defined, lobular lesions, with an isointense signal in T1WI and slightly long signal in T2WI unenhanced scans. Following gadolinium administration, a heterogeneous enhancement is observed, due to cystic degeneration, focal tumor necrosis, or flow voids , 6.Unlike meningioma, which has a hyperplastic effect on adjacent bone, HPCs are shown to exert an osteolytic effect. Moreover, no dural tail sign is observed , 6.Fluorodeoxyglucose (FDG) PET may be helpful in revealing multiple distant metastases. As in our case, HPCs show avid enhancement on CT, but low FDG uptake on PET, indicative of low glucose metabolism .Accurate preoperative diagnosis may still be challenging, despite high-tech imaging modalities, and histopathology and immunohistochemistry confirm the diagnosis. Hemangiopericytomas are highly cellular and vascularized tumors, consisting of tightly packed, round to fusiform cells, around a well-developed, elaborate branching “staghorn” vasculature , 8.According to the World Health Organization classification, HPCs are graded as being differentiated (grade II) and anaplastic (grade III) . Signs oImmunohistochemically tumor cells usually express CD34 and vimentin but not EMA and S100 [Despite advances in diagnostic and therapeutic modalities, management of HPCs remains challenging and problematic. Due to the tumor's rarity, most published studies are retrospective cohorts with small numbers of patients. Consequently, there are no concrete treatment recommendations based on level I evidence.Surgery is the mainstay of treatment. Indeed, all authors agree that gross total resection increases overall survival (OS) and recurrence-free interval , 9–12. HThe role of adjuvant radiotherapy is controversial. Safe conclusions cannot be drawn, due to small sample sizes and retrospective character of available studies. Zweckberger et al. propose radiotherapy to grade II patients with subtotal resections and to all grade III patients . SchiariStereotactic radiosurgery  has been used in cases of recurrent or residual disease, with promising results . Ecker eSystemic chemotherapy has shown only disappointing results and its role is purely palliative , 12. On Although HPC has been described as borderline malignant, its clinical behavior is difficult to predict and long-term follow-up is indicated, since recurrence and metastasis have been reported even after prolonged disease-free intervals , 9, 18. Comparison between low- and high-grade tumors has shown that even grade II tumors have significant metastatic potential and often relapse . High-grThe survival benefit of gross total resection applies to both CNS and extra-CNS HPCs, although extra-CNS tumors tend to be larger and more advanced and with shorter OS .In their study, Damodaran et al. reported OS at 5, 10, 15, and 20 years at 79%, 56%, 44%, and 22%, respectively . For graAlthough liver metastatic disease is a surrogate marker of tumor hematogenous dissemination, long-term survival after hepatectomy is reported. Extrahepatic disease is not a contraindication to liver resection in case of local control, as reported in our case. In essence, if the time interval between the primary lesion and the liver metastatic disease is prolonged, major hepatectomy is justified in young individuals. In case of liver recurrence, re-resection combined with TAE is highly recommended, due to the hypervascular nature of the tumor .


The diagnosis of hepatocellular carcinoma (HCC) relies on imaging tools and biopsy. It usually does not present to be a challenge.Here we report the case of a 69-year-old patient with HCC, initially mistaken for a gastric hepatoid adenocarcinoma (HAC), with a favorable outcome after neoadjuvant chemotherapy.The initial presentation  led to the diagnosis of a gastric hepatoid adenocarcinoma. Neoadjuvant chemotherapy by epirubicin, oxaliplatin, and capecitabine protocol was administered. Biological , radiological (−35% RECIST), and histological (20% of necrosis) responses were observed. Complete surgical resection was then performed. The final pathological diagnosis was a well-differentiated HCC, staged pT4 N0 (0/24) R0.There are no guidelines as to how such tumors should be managed. Nonetheless, neoadjuvant chemotherapy yielded a good outcome. This observation stresses the importance of the final pathological findings and addresses the issue of neoadjuvant therapy in some cases of HCC. Prognosis of HCC depends on the initial tumor staging, which subsequently determines the therapeutic management.2Hepatocellular carcinoma (HCC) accounts for over 90% of all common liver primary cancers. It often occurs in the setting of an underlying liver disease.3 Alpha-fetoprotein (AFP) plasma level is often misleadingly elevated,4 especially in the setting underlying liver disease such as cirrhosis. Furthermore, histological assessment of HAC specimen shows similar morphological features to those of HCC.3 Immunohistochemical staining may be helpful for the diagnosis of HAC showing positive rates of AFP, Hep Par 1, and carcinoembryonic antigen (CEA) stains of 91.6%, 38.1%, and 78.7%, respectively.3 The prognosis of HAC is poor, with a life expectancy shorter than that of HCC.5Clinicians may be confronted with differential diagnosis including benign and malignant lesions. For instance, hepatoid adenocarcinoma (HAC) is a rare variant of extrahepatic adenocarcinoma that frequently originates from the stomach. The distinction between HCC and HAC may be challenging, because of their similar clinical and pathological features.Herein, we report the case of an unusual presentation of HCC, located in the left lobe of the liver and extending to the anterior gastric wall. The tumor was initially mistaken for a gastric HAC. The patient received neoadjuvant chemotherapy followed by radical surgery yielding a prolonged survival.2.A 69-year-old male presented in February 2012 with epigastric pain, nausea, and melena. He had been treated with nonsteroidal anti-inflammatory drugs (NSAIDS) for the pain. Past medical history included hypertension treated with FORZAAR (hydrochlorothiazide and losartan) and ALPRESS (prazosin hydrochloride). There was no family history, allergies, and alcohol or tobacco intoxication. Clinical findings were a good general condition (Performance Status Score: 0), no lymph nodes, ascites, or other signs of portal hypertension. The body mass index was 28 kg/mAn upper gastrointestinal endoscopy was performed given the digestive symptoms and showed a 4 to 5-cm-long lesion located in the anterior part of the greater curvature of the stomach. This lesion looked necrotic and bled easily upon contact. Multiple biopsies were performed, and analysis showed an unusual aspect, made of large cells, with an eosinophilic cytoplasm, lacking mucosecretion, presenting a moderate anisokaryosis and many mitoses. These findings were consistent with a gastric HAC. Immunohistochemical analysis showed that the tumor was negative for CK7, CK20, antihepatocyte antibody, chromogranin synaptophysin, CD56, and positive for pankeratin  and Ki67 (30%–40%). A computed tomography (CT) scan was performed to evaluate tumor extension and showed a large gastric tumor measuring 10 × 8 mm, extending to the diaphragm, the splenic hilum, and lymph nodes in the lateroaortic region . Liver function tests were unremarkable.2 (day 1), oxaliplatin 130 mg/m2 (day 1), and capecitabine 625 mg/m2 b.i.d (from day 1 to day 21). Tolerance was good overall, except an episode of diarrhea (grade 1) and thrombocytopenia (grade 2). The latter led to a 25% reduction of the capecitabine dosage after the first 2 cycles.A multidisciplinary consultation meeting was held, and neoadjuvant chemotherapy by epirubicin, oxaliplatin, and capecitabine (EOX) protocol was decided. The patient was informed and gave his oral consent for the treatment strategy. The aim was to perform surgery afterward provided the response be sufficient. Treatment consisted of epirubicin 50 mg/mThe response to chemotherapy was evaluated by CT scan after 3 cycles of chemotherapy, which showed a moderate response, based on RESIST criteria, with a size diminution of 35%. The residual tumor was located in the splenic hilum, the diaphragm, and the left liver lobe Figure . No lesiThe patient was operated after the fourth cycle of chemotherapy. Gastrectomy with oesojejunal anastomosis, splenectomy, left hepatectomy, and partial phrenicotomy was performed by laparotomy. Macroscopically, the resection was judged complete (R0).Pathology analysis of the surgical specimen Figure  turned oThe resection was judged complete, and the final tumor staging was pT4 N0 (0/24) R0. From June 2012 to December 2013, the patient was under simple surveillance. In December 2013, he presented a pleural effusion and increased AFP serum levels. Imaging did not show macroscopically detectable lesions at that time. A pleuroscopy and upper gastrointestinal endoscopy with biopsies were performed, showing no signs of recurrence of the malignancy. In spite of that, the context, the rising AFP, and the otherwise unexplained pleural effusion lead to the conclusion of an HCC recurrence, with a time to progression after surgery of 19 months. Chemotherapy by EOX protocol was initiated in March 2014, 2 years after the initial diagnosis. The patient died of uncontrolled sepsis in September 2014. At that time, he was still treated by EOX protocol, and recent CT scan had shown stability based on RESIST criteria. Postsurgery survival was of 31 months.2 Diagnosis of HCC is usually based on imaging techniques such as contrast US, MRI, and CT scan showing typical morphological features.4 Pathology analysis of biopsy or resected specimen may be useful especially when the diagnosis is difficult, for instance in the case of an atypical clinical and/or imaging presentation.2 Differential diagnosis includes benign lesions and other malignant liver tumors such as metastatic lesions and cholangiocarcinoma.Here we report a patient with an unusual presentation of advanced HCC, most of the tumor being extrahepatic, to the point where the first CT scan had not shown liver-localized tumor. The metastatic extension was also uncommon . The absence of underlying liver disease and signs of cirrhosis added to the rare presentation. Furthermore, initial histological findings were consistent with gastric HAC. In most cases, HCC develops in the setting of cirrhosis (>85%). HCC may also occur in the setting of a chronic liver disease but rarely on a normal liver.3 Differential diagnosis with HCC may be complicated, given their similarity in terms of histological presentation.3 Immunohistochemistry staining provides specific findings that may be helpful in this setting.3 Positive AFP and CEA stains are found in the majority of HACs (91.6% and 78.7%). AFP is a well-known tumor marker of HCC. Positive AFP stain is found in 8% to 37% of HCCs that may cause confusion. CEA stain may also be positive in HCC. Hep Par 1 (hepatocyte paraffin 1) is a highly specific marker of HCC, but some gastric HACs may present with positive staining (38.1%).3In our case, pathology assessment was challenging. A gastric HAC was diagnosed upon initial assessment of multiple biopsies. Few cases of gastric HAC had been reported so far.3 The positive rate of CK19 in HCC is very low and confers a poor prognosis.6In terms of epithelial markers, all gastric HAC are positive for CK18 and CK19 stains (100%), and most are positive for AE1 and AE3 (92.3%).3 The study by Su et al3 suggests that new immunohistochemistry markers may be useful to differentiate HAC from HCC. Previous studies showed that palate, lung, and nasal epithelium carcinoma-associated protein (PLUNC) seem promising to that effect whereas CD10 and CDX2 are equivocal.8All in all, there is no single marker able to differentiate gastric HAC apparently from HCC. CK19, Hep-Par 1, and CEA are nonetheless essential to establish a diagnosis.10Neither neoadjuvant chemotherapy nor surgery would have been the first treatment approach if the diagnosis of HCC had initially been made. This HCC was stage C according to the Barcelona Clinic Liver Cancer staging classification. Current guidelines do not recommend surgery or neoadjuvant chemotherapy in this setting. Our patient would probably have been treated with sorafenib. The use of sorafenib at this stage is based on the positive results of a large phase III clinical trials and the supposed chemoresistance of HCC.11 Recently, a randomized phase III Asian clinical trial compared FOLFOX  and doxorubicin; 371 cirrhotic patients (Child A or B) presenting a stage B or C HCC were included. Median overall survival was longer in the FOLFOX-treated group . The progression-free survival was also longer . The difference in overall survival was not significant although there was a trend.12 A French phase II prospective clinical trial evaluated the efficacy of GEMOX ; 34 patients presenting a stage B or C HCC were included. Chemotherapy was administered every 2 weeks until progression or severe toxicity. The response rate was of 18% . Disease stabilization rate was 58% (comprising 5 minor responses). Altogether, disease control was achieved in 76% of all patients. Median progression-free survival was 6.3 months (95% CI: 4.3–10.1), and median overall survival was 11.5 months (95% CI: 8.5–14.3).13Several conventional chemotherapies have been evaluated in advanced HCC with disappointing results in terms of improvement in overall survival, despite varied response rates.Our patient had a satisfactory response to chemotherapy, allowing a surgical resection. The chemosensitivity was also an uncommon feature of our case. We believe that chemotherapy could be of interest in highly selected cases of HCC. Unfortunately, there has yet to be markers that could help distinguish these particular tumors. Studies are warranted to identify these markers.We present an unusual case of a mostly extrahepatic HCC with a challenging diagnosis given histological findings mimicking an HAC. This case highlights that HCC may present in an atypical way and that the differential diagnosis with HAC may be difficult. This case shows the importance of the final histological analysis. This case also suggests that HCC may benefit from conventional chemotherapy. Consequently, among the different subtypes of HCC, some may respond to chemotherapy. New markers are needed to identify tumors susceptible of being sensitive to chemotherapy.avec” that funded all costs associated with the publishing of the present manuscript.The authors would like to acknowledge “la Foundation


Dental traumatic injuries may affect the teeth and alveolar bone directly or indirectly. Pulpal necrosis and chronic and apical periodontitis with cystic changes are the most common sequelae of the dental traumatic injuries, if the teeth are not treated immediately. This case report focuses on the conventional and surgical management of mandibular central incisors. A twenty-four-year-old male patient presented with pain in the mandibular central incisors. Radiographic examination revealed mandibular central incisors with dumbbell shaped periapical lesion. After root canal treatment, parendodontic surgery was performed for mandibular central incisors. After one-year recall examination, the teeth were asymptomatic and periapical lesion had healed. Dental traumatic injuries usually occur in 7- to 12-year-old age group and mostly due to falls and accidents near home or school. Anterior region of the mouth is most commonly affected in dental trauma  paste  was given as an intracanal medicament for one week. Patient was prescribed antibiotics and analgesics to manage pain and swelling. Patient was prescribed 500 mg of amoxicillin thrice a day for five days and combination of 100 mg of aceclofenac and 15 mg of serratiopeptidase twice a day for five days. One week later, the patient was asymptomatic, obturation was done with cold lateral compaction technique, and the access cavity was restored with composite resin. Before performing the endodontic surgery, the patient was advised to undergo blood investigations to rule out bleeding disorders. Complete blood picture and coagulation studies report were normal. The general health condition of the patient before the surgery was good and he fell under ASA I, according to “ASA” physical status classification system.Local anaesthesia was administered, under rubber dam isolation, and access cavity was done using a number 2 high speed round diamond bur. Working length was determined by apex locator and canal patency was checked with number 15 K-file. Cleaning and shaping of the root canals were performed by hand instruments with step-back technique up to 45 ISO size K-file with alternate irrigation of 3% sodium hypochlorite (NaOCl) solution and saline. Two percent chlorhexidine (CHX) was used as a final irrigating solution. Calcium hydroxide [Ca(OH)Under local anaesthesia, a full thickness mucoperiosteal flap was elevated. A large soft lesion was seen involving the root apices of 31, 41. The lesion was circumferentially separated from the bony crypt and the teeth. Using gracey curettes, the granulation tissue in the apical and lateral root surfaces of the mandibular central incisors was curetted. For the histopathological examination, the granulation tissue was fixed in 10% buffered formalin. The surgical site was washed with sterile saline solution after the complete removal of the lesion. Apical 3 mm of the roots was resected for 31, 41 and the retrograde filling was done with mineral trioxide aggregate (MTA). As the extension of the defect was large, bone graft (Perioglas) was placed. The mucoperiosteal flap was sutured in place and the periapical radiograph was taken for the confirmation of accuracy of retrograde filling for 31, 41. The granulation tissue was sent for histopathological examination, the findings were suggestive of cystic capsule. The patient was periodically reviewed after 3 months, 6 months, and one year. Patient was asymptomatic during one-year follow-up. At one-year follow-up, a radiograph was taken in relation to mandibular central incisors, which confirmed the satisfactory healing of periapical lesion .The endodontic management of traumatized mandibular central incisors of a 20-year-old patient was described in this case report. After dental injury, if immediate and appropriate treatment is provided, then it results in successful endodontic outcome. Pulpal response to dental trauma is variable. In some cases, the pulp remains normal, whereas in some cases it becomes necrotic. As a consequence to dental trauma, the pulp loses its ability to protect itself from bacterial invasion and the bacteria penetrates through the dentinal tubules, colonizes in the necrotic pulp, and leads to the development of periapical lesion [2 or larger, then the incidence of cysts was almost 100% and they have analyzed the data of different studies relating the radiographic lesion size to histology [Persistent chronic infection can lead to formation of a periapical cyst. Periapical cysts commonly occur in the mandible and may appear as unilocular or multilocular radiolucencies on radiographs. Cystic lesions of the mandible can result in bone remodeling which weakens the bone, leading to functional changes and predisposing the patient to infection and pathologic fracture . Natkin istology .The possible dumbbell shaped radiographic appearance of the present lesion might be due to the chronicity of the lesion and its extension into the lingual aspect through a possible least resistant path . The dumThe differentiation of periapical cysts and granulomas is very difficult by the traditional radiographs. By the radiographs, only the mesiodistal extent of the pathology can be determined but not the buccolingual extent. With the advent of advanced imaging modalities like computed tomography (CT), cone-beam computed tomography (CBCT), ultrasound with power Doppler flowmetry, and magnetic resonance imaging (MRI), differences in density may permit more accurate preoperative diagnosis . Final dWhen compared with conventional radiography, CT scan allows visualization of teeth and their surrounding structures in three dimensions. CT is a valuable tool for the presurgical assessment of the teeth, periodontal structures, and proximity of the teeth to adjacent vital structures. With the CT, the true size, location, and extension of the periapical lesion are known and also they can be detected and localized in relation to critical anatomic structures. Velvart et al. conducted a study on 50 patients to detect the apical lesion and its relation to important neighbouring anatomic structures and concluded that CT provides additional 3D information during treatment of apical surgery of mandibular premolars and molars when compared to conventional 2D radiographs .Trope et al. used CT scans for the differentiation of radicular cyst and granulomas and they concluded that cyst can be differentiated from granuloma by density . In case2 therapy is one of the options for treating large cystic periapical lesions. If this treatment fails, then periapical surgery is considered.The main indications of periradicular surgery includes obstructed canals, failed endodontically treated cases, extruded root filling materials, and lesions after traumatic injuries. Apicoectomy, periradicular curettage, and root resection are performed during periapical surgery, for achieving successful outcome , 17. Dur2 was used as an intracanal medicament for a period of one week. Sjogren et al. reported that Ca(OH)2 as an intracanal medicament for a period of one week efficiently eliminates the bacteria in the root canals and also causes healing of periapical lesion [The main objective of endodontic treatment is complete eradication of microorganisms from the root canal system. Ca(OH)l lesion .In the present case, MTA was used as a root end filling material during periapical surgery of 31, 41. Parirokh and Torabinejad reported that MTA produced cementum formation in 23% of the specimens after 2–5 weeks of periapical surgery and more than 80% of root-end filled cavities with MTA showed deposition of cementum 10–18 weeks after surgery. MTA produces favourable results in terms of absence of inflammation, formation of hard tissue and cementum . PerioglDuring surgical endodontic therapy, necrotic cells, tissue debris, and bacteria in the periapical lesions are completely removed. However, the endodontic surgery is an invasive procedure; if case selection is proper, then surgical debridement is very effective and quite rapid. Compared to nonsurgical endodontic therapy, healing of periapical wound is much faster after endodontic surgery . RadiogrThis case report illustrates the “successful management of large dumbbell shaped periapical lesion of mandibular central incisors with endodontic treatment followed by periapical surgery.” The results confirmed satisfactory healing of the large periapical lesion which responded favourably to successful parendodontic surgery.


There are rare case reports of tracheal diverticula or paratracheal air cysts. These cases, however, were reported mostly as incidental sonographic or radiologic findings without histologic confirmation. Furthermore, the handful of studies that describe this entity histopathologically report only cases in patients with prior respiratory symptoms. Here, we report a rare case of an asymptomatic 60-year-old female with no significant past medical history who presented with primary hyperparathyroidism. She was found to have an incidental right paraesophageal air-filled diverticulum with multiple thin septations on her imaging studies. She was taken to surgery and the histologic examination of the specimen revealed multiloculated cystic cavity lined by respiratory-type columnar epithelium with lymphocytic infiltrate and minor salivary glands within the surrounding stroma, rendering the diagnosis of tracheal diverticula. There are rare case reports of paratracheal air cysts/tracheal diverticula. These are generally incidental sonographic or radiologic findings without subsequent morphologic diagnosis. Even the classic histopathologic description is based only on autopsy studies. Hereby, we report a unique case in which the histopathologic diagnosis of a tracheal diverticulum was considered intraoperatively and confirmed on the final surgical specimen in a 60-year-old woman who underwent surgery for hyperparathyroidism.A 60-year-old female with no significant past medical history presented with primary hyperparathyroidism. She was asymptomatic with slightly elevated serum calcium and parathyroid hormone (PTH) levels. The sestamibi scan did not show any uptake, while the computed tomographic scan (CT) of the neck showed an incidental right paraesophageal air-filled diverticulum with multiple thin septations . During The specimen submitted for pathologic examination consisted of a 2.4 × 1.5 × 0.8 cm tan brown multiloculated/cystic structure with some attached adipose tissue. The cyst wall measured 0.1 cm in thickness. An opening of 0.2 cm in diameter was noted on one aspect of the lesion .Histologic examination of the entire lesion confirmed the presence of cystic spaces lined by respiratory-type ciliated columnar epithelium, minor salivary gland tissue, and lymphoid aggregates Figures . Given tParatracheal air collections are usually found as incidental findings on imaging studies. In these cases, the differential diagnosis includes tracheal diverticulum, laryngocele, pharyngocele, Zenker's diverticulum, apical hernia of the lung, and apical bullae . PharyngCongenital tracheal diverticula are different from the acquired ones and are thought to be malformed supernumerary branches of the trachea . They reBranchial cleft cysts are developmental cysts considered synonymous by some with the cervical lymphoepithelial cyst. They may be lined by columnar epithelium (and squamous epithelium) resting on lymphoid tissue. They most commonly present in the neck; however, those arising from the fourth branchial arch may occur in the mediastinum . HoweverIn summary, we presented a unique case of tracheal diverticulum with intraoperative morphologic assessment. This entity, however rare, needs to be kept in mind when encountering a “paratracheal air cyst” because symptomatic or infected diverticula need surgical resection .


Hemangioma is the most common vascular tumor of infancy; presentation is often as cutaneous infantile hemangioma (IH). Cutaneous hemangioma is a clinical diagnosis. Most IHs follow a benign course, with complete involution without treatment in the majority of cases. Visceral hemangioma often involves the liver and manifests as a life-threatening disorder. Hepatic hemangiomas may be associated with high output cardiac failure, coagulopathy, and hepatomegaly which generally develop between 1 and 16 weeks of age. Mortality has been reportedly high without treatment. We report a rare case of a male infant with neonatal hemangiomatosis with diffuse peritoneal involvement, which mimicked a malignant-looking tumor on imaging, and discuss therapeutic options and efficacy. Propranolol is efficacious for IH but generally not useful for other forms of vascular hemangiomas, tumors, and malformations. In our case of neonatal peritoneal hemangiomatosis, propranolol appears to have halted the growth and possibly expedite the involution of the hemangiomatosis without other treatments.  Hemangiomas are the most common vascular tumors of infancy –5. When A term baby boy, birth weight 4.17 kg, with fetal ascites and abdominal distension was delivered by Cesarean section. Antenatal scan at 20 weeks' gestation showed fetal ascites with normal morphology. Amniocentesis was performed and karyotyping was normal. Viral studies including cytomegalovirus and toxoplasmosis were unyielding. Repeated antenatal ultrasonography scan at 23 weeks showed increased ascites with peritoneal thickness. There was an irregular solid mass arising from left posterolateral peritoneal surface and protruding into the peritoneal cavity. Fetal MRI showed a T1 and T2 isointense soft tissue mass at the left lower quadrant of the abdomen, without cystic or fatty components . The masThe infant was born at term with gross abdominal distension and respiratory distress, requiring mechanical ventilation support at birth. He was successfully extubated on day 2 of life. Postnatal CT scan revealed a large multilobulated enhancing mass, 5.8 × 4.3 × 8.6 cm in size, along the left paracolic gutter, extending down to the left iliac fossa. A large tortuous vein was noted to be draining directly into the inferior vena cava and there were multiple small (up to 1.6 cm) avidly enhancing lesions present in the subphrenic and perihepatic regions. They were compatible with peritoneal metastases on contrast imaging . The larEchocardiogram and ultrasound of the brain were unremarkable. The infant was treated with propranolol at 2 mg/kg/day in three divided doses. Clinically the infant continued to do well and had normal growth and development. He tolerated propranolol well. There was clinically no abdominal distension or palpable abdominal mass. Four months after initial presentation, repeat ultrasound of the abdomen showed a reduction in size of the soft tissue and decrease in numbers of small peritoneal deposits. Latest ultrasound scan at 8 months showed the heterogeneous left flank hemangioma measuring 2.4 × 0.9 cm.This is an unusual case of hemangiomatosis involving the visceral omentum. Infantile hemangioma is the most common vascular tumor of infancy, with an incidence between 1 and 12%, varying by race . Female Imaging is useful for visceral hemangiomas. There are typical radiological features which can help with the diagnosis, such as vascular pooling of contrast within the tumor and a centripetal enhancement pattern on dynamic scan. However, in our reported case, the lack of radiological features in combination with the malignant appearance and rapid growing nature warrants a histological diagnosis to exclude other primary neoplasms.β-blocker, has been used as a first-line treatment of problematic infantile hemangioma. Multiple, small retrospective case series confirm the effect of propranolol [Most cutaneous hemangiomas do not require treatment and only warrant close observation. Indications for active intervention include severe or recurrent hemorrhage unresponsive to treatment, threatening ulceration in areas where serious complications might ensue, interference with vital structures, pedunculated hemangiomas, and significant disfigurement. In recent years, propranolol, a nonselective pranolol . A 2013 pranolol . It has pranolol –13. Our In conclusion, we report an unusual case of congenital omental hemangiomatosis which behaves like a malignant-looking neoplasm radiologically. Yet it responded well to propranolol treatment. Propranolol may be considered as first-line therapy for congenital hemangiomas that are extensive and involve the viscera. Life-threatening and complicated cases of hemangiomatosis are challenging conditions especially in the neonates. Other treatment options include corticosteroid, cyclophosphamide, and laser therapy , 8, 12.


Ganglioneuroma is a rare tumor in the posterior mediastinum; fat-containing ganglioneuromas are rarely reported. The present case report documents a brown fat-containing, posterior mediastinal ganglioneuroma, which has not been reported previously. Radiological examination, in particular 18F-2-fluoro-2-deoxyglucose-positron emission tomography, suggested that the tumor had low-grade malignant potential. This led to uncertainty at preoperative diagnosis.An asymptomatic 66-year-old Japanese woman with no significant past medical history was referred for the evaluation of a posterior mediastinal mass. Although its size had not changed in the past 5 years, a malignant lipomatous tumor could not be excluded due to the presence of intratumoral fat and increased 18F-2-fluoro-2-deoxyglucose uptake observed by positron emission tomography imaging. A computed tomography-guided core-needle biopsy revealed a mixture of mature adipocytes, spindle-shaped cells, and fibrotic stroma. Definite diagnosis was not possible, and surgical resection was performed. Three years after the surgery, she remains disease-free.Histological diagnosis of the surgically resected mass confirmed ganglioneuroma with substantial amounts of white and brown adipose tissues in peripheral areas. The existence of both ganglion cells and brown fat tissue intensified the accumulation of 18F-2-fluoro-2-deoxyglucose, resulting in a false-positive result by positron emission tomography. Considering this, ganglioneuroma should not be excluded either clinically or pathologically in fat-containing, posterior mediastinal tumors. Some symptomatic cases with hypertension, watery diarrhea, or virilization have been reported[Ganglioneuroma is a rare, benign neurogenic tumor predominantly found in adolescents older than 10 years of age or young adults[reported, althougreported-7. Here A 66-year-old Japanese woman  was referred for the evaluation of a mass in her left posterior mediastinum. The nodule was noted by her primary care physician about 5 years earlier, and she had also been informed about the presence of an abnormal lesion noted in a chest radiograph around 40 years earlier. She showed no symptoms, and had no significant past medical history. The mass had not changed in size, shape, or location until just prior to her referral. She had undergone computed tomography (CT), which could not exclude the possibility of a well-differentiated liposarcoma due to the presence of intratumoral fat tissue. She was therefore referred for additional investigations. Her physical examination was unremarkable except for mild hypertension. Routine laboratory test results were within normal limits, and levels of tumor markers, including squamous cell carcinoma antigen, carcinoembryonic antigen, cytokeratin 19 fragment, neuron-specific enolase, pro-gastrin-releasing peptide, beta human chorionic gonadotropin, and alpha-fetoprotein were also within normal ranges. Catecholamine levels were not tested. Imaging studies by CT Figure A, left. On careful retrospective examination of the biopsy specimen with serial sections, only one ganglion-like cell without satellite cells was seen in the fibrotic background. Considering this, the biopsy specimen appeared to be reflective of the peripheral portion of the tumor, even if the needle had been placed in the central portion.Ganglioneuromas with abundant fat replacement are unusual and only five cases have been reported to date in the English and Japanese literatures-7. Thus,Two hypotheses have been put forward regarding the pathogenesis of intratumoral fat tissue. The first involves fatty replacement of atrophied tumor lesions, in which the tumor tissue is gradually replaced by mature fat tissue over time. The secIn this case, ganglioneuroma was not suspected until after postoperative pathological diagnosis of the surgical specimen. This is because ganglioneuroma is unusual for a fat-containing tumor, and the SUVmax by FDG-PET suggested a low-grade malignancy. Moreover, many diseases may involve fat-containing mediastinal masses, including benign conditions such as extramedullary hematopoiesis and teraWith respect to imaging features, typical CT findings of posterior mediastinal ganglioneuroma include Regarding the value of SUVmax seen here , it was difficult to rule out well-differentiated liposarcoma because the SUVmax of these tumors has been reported to be around 2.3±1.7 (mean±standard deviation. HoweverA rare case of ganglioneuroma with peripheral replacement by white and brown fat tissue has been presented, which yielded a false-positive result in FDG-PET by suggesting that the tumor was of low-malignant potential. To the best of our knowledge, this is the first report of a brown fat-containing ganglioneuroma. Ganglioneuroma should be included in differential diagnosis for posterior mediastinal tumors with abundant fat tissue, and clinicians should be aware that FDG-PET of ganglioneuroma can yield a false-positive result by suggesting low-grade malignant potential.Written informed consent was obtained from the patient for publication of this case report and the use of accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: Computed tomography; FDG: 18F-2-fluoro-2-deoxyglucose; MRI: Magnetic resonance imaging; PET: Positron emission tomography; SUVmax: Maximum standardized uptake value; WI: Weighted image.The authors declare that they have no competing interests.KY carried out pathological diagnosis on the surgically resected sample, analyzed data, and had a major contribution in writing the manuscript. AY and TA performed clinical examination and surgical treatment. HN performed radiological diagnosis. KN performed clinical follow-up and obtained informed consent from the patient. TF and HK performed pathological diagnosis of the biopsy specimen. All authors have read and approved the final version of the manuscript.


We present a 46-year-old female patient who is diagnosed with synchronous postirradiation sarcoma, cutaneous relapse of a previous soft tissue sarcoma, and lung adenocarcinoma. More than one malignant tumor at the same time with an accompanying relapse of a previous malignant tumor is a rare entity. A relatively young patient diagnosed with adenocarcinoma of the urethra before age 40, which is an unusual tumor for that age, later three more different malignant tumors being diagnosed, two of which are synchronous, causes the suspicion of Li-Fraumeni syndrome. Li-Fraumeni syndrome is a rare genetic condition and increases the risk of developing more than one cancer including breast cancer, soft tissue sarcomas, osteosarcoma, and brain tumors , 2. ClinGenerally accepted criteria to diagnose a postirradiation sarcoma (PIS) include treatment with therapeutic irradiation at least two years prior to development of a sarcoma, a sarcoma arising within the field of previous therapeutic irradiation, and different histologic features between the sarcoma and the primary tumor that required radiotherapy , 4.With this report we aimed to present a 46-year-old female patient who is diagnosed with a possible Li-Fraumeni syndrome with four primary malignant tumors, one of which is a postirradiation sarcoma with simultaneous relapse of the previous sarcoma within the same anatomic compartment.We present a 46-year-old female patient. The patient was first diagnosed with urethra adenocarcinoma 8 years ago. Total resection of the urinary bladder with lymphatic dissection was performed followed by combined chemotherapy and radiotherapy. One and a half-year after the first operation the patient was reoperated on for metastasis of the urethral adenocarcinoma and vulvectomy with left inguinal radical lymph node dissection was performed. The pathology report was in compliance with the first report of the urethral adenocarcinoma. Postoperatively, the patient received 54 Gy/30 fraction radiotherapy. At the end of the same year the patient suffered a mass in her right thigh that caused a suspicion of another metastasis. Tru-cut biopsy was performed and pathology specimens revealed malignant mesenchymal tumor. 28 Gy/8 fraction preoperative radiotherapy was applied due to close neighbourhood of the tumor to the femoral artery and the tumor being larger than 5 centimeters in diameter. Following preoperative radiotherapy, wide resection of the tumor was performed. The pathology reports showed a high grade pleomorphic sarcoma. The oncology consultation did not recommend postoperative chemotherapy, but 20 Gy/10 fraction adjuvant radiotherapy was applied.For the next three years the patient was regularly followed and no evidence of local or systemic relapse was present. The patient quit the routine control examinations and could not be followed until 4 months ago when MRI evaluation was performed for a mass in her left thigh. A subcutaneous lesion was detected in the left thigh with typical features of a lipoma. In addition, a large mass in the right femur was coincidentally observed with a satellite cutaneous nodule close to the previous incision scar Figures  and 2. TThe patient was reoperated on. Wide resection for the metastatic nodule of the skin was performed first and the frozen pathology reported it as a malignant mesenchymal tumor. The tumor of the right femur was also removed with wide resection including the involved 10 cm segment of the femoral artery and vein in the Hunter canal . Frozen Throughout the routine radiologic evaluations and PET scan before surgery, a lesion in the right lung was also detected and was reported as a metastatic lesion . Three wLi-Fraumeni syndrome is a rare cancer predisposition disorder that is associated with germline mutations of the p53 tumor suppressor gene. The three clinical criteria for typical Li-Fraumeni are a proband with sarcoma diagnosed before age 45, a first degree relative with any cancer before age 45, and a first or second degree relative with any cancer before age 45 years or a sarcoma at any age. Patients with Li-Fraumeni syndrome are more prone to having more than one primary tumor and are also more prone to have postirradiation sarcomas due to TP53 gene mutation . In a stThe fact that the tumor must arise in the region of a prior irradiation, having a latency period of at least two years, and proof that the sarcoma is histologically different from the radiated primary lesion are the criteria of postradiation sarcoma modified by Laskin et al. , 8. A hiOur patient had four different malignancies at the age of 46 including two different carcinomas and two different sarcomas. Her son also died because of metastatic complications of osteosarcoma. The first lesion that she was operated on for was urethral adenocarcinoma at the age of 37 which is a carcinoma type that is usually seen after 70 years of age and is atypical for her age . The lesIn a study, chemotherapy followed by surgical resection is proposed for PIS and according to the same study the patients who benefited from complete surgical resection had the best prognosis . AlthougReconstruction of diaphyseal bone defects after tumor resection can be performed using different biologic or nonbiologic materials including diaphyseal endoprostheses, allografts, bone transport, and vascularized autograft –13.Although nonbiologic materials provide immediate reconstruction, high implant failure rates that cause need for additional operations are likely. A reconstruction using an endoprosthesis might be a better alternative for patients with poor survival expectation as these allow immediate weight bearing , 14. A bWe performed massive allograft reconstruction using an intramedullary nail. The advantage of this kind of a reconstruction is that it allows mechanical and biologic reconstruction during the same procedure –14. The A patient with an atypical cancer for his/her age and with a first degree relative who had a sarcoma history, as was the situation in our case, should be examined carefully and the possibility of Li-Fraumeni syndrome should be kept in mind in order not to increase the risk of PIS due to treatment with high doses of neoadjuvant and adjuvant radiotherapy. In such a situation, chemotherapy added to surgical resection could also be a choice of treatment.