A 55-year-old postmenopausal female presented with genital bleeding and lower abdominal mass. An abdominal MRI revealed a heterogeneously enhanced, 15 × 10 cm mass, completely filling the lumen of the enlarged uterus. The cytologic analysis of the mass showed tumor cells in small clusters and as individual cells showing hyperchromatic round to oval nuclei, and pleomorphic and occasionally unipolar “tadpole”-shaped cytoplasm, in a background of severe necrosis and many degenerated squamous cells. We first interpreted it merely as atypical cells, possibly originated from sarcoma. A total abdominal hysterectomy and salpingo-oophorectomy were performed, and gross examination showed an exophytic polypoid mass with a whitish to white-grayish, necrotic appearance, protruding from the endometrial mucosa. Microscopically, the tumor was composed of a diffuse proliferation of highly atypical spindle-shaped cells, admixed with many characteristic rhabdomyoblasts having abundant densely eosinophilic cytoplasm with sometimes distinct cross-striations, coexisted with cellular primitive small blue round to oval cells foci. However, neither carcinoma nor additional heterologous sarcoma components were completely seen within our thorough investigation. Therefore, we finally made a diagnosis of embryonal rhabdomyosarcoma arising from the uterine corpus. We should be aware that owing to its characteristic features, cytopathologists might be able to determine a genuine diagnosis, based on multiple and adequate cytology samplings. Among all adult sarcomas, rhabdomyosarcoma accounts for between merely 2 and 5 % , 2. AlsoThe patient presented here, a 55-year-old postmenopausal female (G3P3) with an unremarkable previous medical history, except for appendectomy 30 years ago, had a complaint of dysfunctional uterine bleeding and lower abdominal mass. Laboratory data, including blood cell count, chemistry and tumor markers, were within normal limits. The sagittal section of a pelvic MRI revealed a heterogeneously enhanced huge mass, measuring approximately 15 × 10 cm in diameter, arising possibly from the uterine corpus and, projecting into and completely filling the lumen of markedly enlarged uterus  according to the manufacturer’s instructions , 9. ImmuBased on all these features, we finally made a diagnosis of primary embryonal rhabdomyosarcoma arising from the uterine corpus in an adult female. To date, approximately 6 months routine follow-up after the surgery is established, and the patient remains well and no recurrence has been identified.Early diagnosis and aggressive clinical treatment for rhabdomyosarcoma of the uterine corpus especially in an adult female could be the hope for better prognosis, due to a higher-grade malignant tumor –4, even The cytologic features should reflect the histopathological ones resembling the two major rhabdomyosarcoma subtypes, embryonal and alveolar, showing many small clusters of tumor cells or cellular individual tumor cells having hyperchromatic small round to oval nuclei, and pleomorphic and sometimes unipolar “tadpole”-shaped scant cytoplasm, in the background of necrosis and/or hemorrhage, reminiscent of malignant small round cell tumors –7. On thAn immunohistochemical analysis must also be a nice and helpful guide to reach a genuine cytological diagnosis of embryonal rhabdomyosarcoma arising from the uterine corpus in an adult female. Immunohistochemistry can demonstrate that several myogenic markers, such as desmin, pan-muscle actin HHF-35) and myogenin, are specifically expressed in those patients’ samples , 2, 5, a and myogWe herein reported a case of an embryonal rhabdomyosarcoma arising from the uterine corpus in an adult post-menopausal female. The present case was tentatively diagnosed as suspicious of sarcoma, not otherwise specified, based on the relatively small and inadequate cytology examination. All cytopathologists should be aware that its cytopathologically characteristic features, including immunohistochemistry, as well as multiple and adequate cytology specimens, might be able to lead to a genuine diagnosis as an embryonal rhabdomyosarcoma from the uterine corpus.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.


An exemplifying case of a 29-year-old female with progressive complaints of pain and paresthesias in the sacral and perianal regions, constipation, and weight loss for 6 months is presented. The surgical technique for en bloc excision of a large sacral giant cell tumor through a modified Kraske procedure with mid-sacrectomy and coccygectomy is described. Complete resection with wide tumor-free margins was achieved. At 5 years of follow-up the patient is neurologically intact, without evidence of local recurrence on imaging studies. A multidisciplinary surgical procedure is mandatory to completely remove sacral tumors. In the particular case of giant cell tumors, it allows minimizing local recurrence preserving neurovascular function, through a single dorsal and definitive approach.Sacral giant cell tumors are rare neoplasms, histologically benign but potentially very aggressive due to the difficulty in achieving a complete resection, their high recurrence rate, and metastization capability. Although many treatment options have been proposed,  Giant cell tumors (GCT) of bone are rare neoplasms comprising 5% of all primary bone tumors in adults  and 5 toGCT are histologically benign, presenting a slow growth rate and insidious or clinically silent onset, making early diagnosis difficult. Usually they exhibit a very large size when diagnosis is made . They arMagnetic resonance imaging (MRI) and computed tomography (CT) scans are useful for early diagnosis and preoperative planning . Needle Different treatment options have been used for sacral GCT , 14. The En bloc excision with tumor-free margins, although challenging, is the procedure of choice, once this constitutes the most effective method for local disease control and recurrence prevention, improving outcome and providing the best chance for cure [When located in the sacrum, surgical resection is the primary treatment modality, being advocated by most authors , 27–29. for cure , 17, 28. en bloc excision of the tumor, with wide tumor-free margins. A detailed and comprehensive step-by-step surgical technique overview is presented.We present an exemplifying case of a patient harboring a lesion, which was surgically treated through a modified Kraske procedure with mid-sacrectomy and coccygectomy, forA 29-year-old female, without known past medical history, was admitted with progressive complaints of severe pain and paresthesias in the sacral and perianal regionsfor 6 months. In this period of time she also presented constipation and 5 Kg weight loss. These symptoms were refractory to medical therapy. Pain exacerbated in the night and by Valsalva maneuvers, causing severe functional disability.Physical examination revealed severe pain on palpation and percussion of the sacral region, without a visible or palpable lesion or other signs of inflammation. Digital rectal examination revealed a large midline presacral mass, fixed to the sacrum, with firm consistency and irregular surface.The lumbosacral CT and MRI scans showed a large, expansive, and osteolytic lower and mid-sacral lesion, with poorly defined margins, extending up to the inferior half of S2 vertebra. The mass comprised both intra- and extracanalar components, a ventral extension displacing the rectum anteriorly, and dorsal expansion out of the sacral hiatus and dorsal foramina with soft tissue compromise. It was located in the midline, slightly more pronounced on the right side, in between the inferior half of S2 vertebra and the sacrococcygeal junction. S2 nerve roots were spared but all nerve roots distal to that were involved by the tumor. The coccyx was not affected .Further diagnostic workup was performed including laboratory studies with tumor markers, chest, abdomen, and pelvis CT-scan and positron emission tomography. No abnormalities or other lesions were detected.After complete characterization of the boundaries of this sacral solitary lesion, a multidisciplinary elective and definitive surgery was scheduled with the collaboration of general and plastic surgeons. No previous biopsy was performed. En bloc excision of the tumor with wide tumor-free margins through a modified Kraske procedure with mid-sacrectomy and coccygectomy was achieved requiring sacrifice of the nerve roots and thecal sac below the level of S2 nerve roots (The patient was electively operated by one of the senior authors (A.L.).ve roots .Anesthesia and Positioning. Under general anesthesia the patient was intubated. Arterial line was placed for blood pressure monitoring. Intravenous dexamethasone and antibiotic prophylaxis (cefazolin 1 g) were administered preoperatively. The patient was positioned prone. All pressure points were covered with padding. Care was taken to avoid elevated abdominal and airway pressures because this would lead to inconvenient bleeding. The posterior lumbosacral area, low back buttock, and posterior thighs were subsequently sterilized and draped after the skin was dried, giving the plastic surgeons many options for soft tissue reconstruction and wound closure at the final step of the surgery. Extra care was taken at this stage due to the proximity of the anal orifice to the surgical field, increasing the risk of wound contamination.Modified Kraske Procedure. A midline longitudinal skin incision was carried out posteriorly extending from the lumbosacral junction to the coccyx. The sacral fascia was exposed from L5-S1 level to the tip of the coccyx  attachments and the distal ends of the S3, S4, and S5 nerve roots were identified and cut with the monopolar electrocautery. This allowed the specimen to be circumferentially freed and removed from the surgical field, resulting in a satisfactoryThe specimen included the tumor with its presacral component  and the Hemostasis and Closure. A large dead-space cavity resulted from the excision of the specimen. Careful hemostasis of the presacral soft tissue was achieved. Bleeding from the sacral osteotomies was controlled with bone wax.General surgeons helped reapproximating the levator ani muscles. In the final step of the surgery, plastic surgeons provided soft tissue reconstruction to fill the defect and close the wound. At this time, the skin incision was lengthened incorporating the prior midline incision. Adipomuscular mobilization and rotational flaps of the gluteus maximus were used in the reconstruction of the sacral defect . AdequatEn bloc excision with wide tumor-free margins of this large lower and mid-sacral mass was achieved through a modified Kraske procedure with mid-sacrectomy and coccygectomy. It required sacrifice of the nerve roots and thecal sac below the level of S2 nerve roots.As previewed on the preoperative MRI scan, the tumor location at the lower and mid-sacrum and its limits  were corroborated with the intraoperative findings and allowed to precisely define the boundaries of the specimen to be removed, keeping distance from the tumor capsule and sacroiliac joints.There were no procedure-related complications. Histopathological analysis of the resected specimen revealed a benign GCT .The patient was discharged home presenting constipation and urinary retention. Constipation resolved with laxatives. Concerning urinary retention she underwent a suitable rehabilitation program, with intermittent self-catheterization, and perineal muscular tonification workout with voluntary sphincter contraction exercises. This resolved within five months following surgery. At five years of follow-up, the patient still complains of minimal subjective numbness in the perianal region, without any other neurological deficits and performing well in all living activities, being inclusively able to sit for long periods of time without pain. The postoperative MRI scan showed complete resection of the distal sacrum and coccyx with no evidence of residual lesion. Last follow-up MRI was performed five years after surgery showing no recurrence of the tumor .Management of GCT of the sacrum is complex and challenging from diagnosis to treatment. This is due to their rarity and heterogeneous clinical scenarios and because the surgical procedures generally involved are extensive and aggressive, aiming for complete tumor resection and potential cure.These procedures may be extra-demanding not only because of the aggressive nature and behavior of GCT, but also once the majority of them are diagnosed in an advanced stage of the disease, exhibiting large volumes and sometimes, poorly defined margins. This may create technical difficulties in surgical access and tumor resection, due to the surrounding anatomical constraints and nearby noble structures to preserve , 20, 30.A detailed clinical and neurological assessment including digital rectal examination is mandatory for helping to establish an early diagnosis. Investigation for metastatic disease should always be performed in order to decide the best treatment option . en bloc resection with tumor free margins without further neurological deterioration. Having this in mind, the surgical approach would be the same regardless of the biopsy result.Tumors' radiological appearance and their location, accessibility, local extension, and involvement of adjacent neurovascular structures are of paramount importance and should always be kept in mind before considering performing a biopsy, once this is an invasive, noninnocuous procedure, with well-known risks of tissue contamination through the biopsy tract, hemorrhage, and infection , 31. CT- en bloc resection [Preoperatively, it is important to precisely define the goal of surgery and the anatomical boundaries to be respected intraoperatively. It is crucial to accurately assess several imagiological parameters such as the level of sacral involvement, infiltrated structures , and ventral and dorsal extensions. This is essential to establish a preliminary diagnosis and decide the best surgical approach and for surgical planification of theesection .Several treatment options have been proposed: intratumoral curettage plus radiotherapy, possibly aided by preoperative embolization, the use of osteoclast inhibiting drugs (bisphosphonates), and cryosurgery with liquid nitrogen, limited by the risk of injury to adjacent neural structures in sacral tumors. en bloc excision is the gold standard procedure for sacral GCT which present radiological criteria denoting the potential aggressiveness of the tumor: poorly defined margins, cortical bone destruction, and soft tissue extension by expansive tumor growth [According to some authors,r growth , 32–34.The presented surgical technique is an optimal and effective strategy to completely remove lower and mid-sacral tumors. It is indicated for the benign but highly aggressive sacral GCT and can also be applied to primary malignant bone tumors of the sacrum. In the particular case of GCT, the modified Kraske procedure with mid-sacrectomy and coccygectomy allows maximizing local tumor control, minimizing the risk of local recurrence, and providing possible cure, giving the possibility to preserve neurovascular function, by this mean decreasing morbidity and improving the final outcome.During the surgery of lower or mid-sacral tumors, several nuances must be taken into account. Only the affected sacral nerve roots should be sacrificed, and the expected neurological outcome needs to be preoperatively predicted and discussed with the patient , 35–37. Given the complexity of evaluation, treatment, and management of GCT, a coordinated multidisciplinary team approach to the problem, involving neurosurgeons, general surgeons, and plastic surgeons, working together in specialized units, has proved useful . This coThe used modified Kraske procedure  provided an excellent surgical access between the mesorectum and the presacral component of the tumor, allowing a safe and complete tumor removal to be achieved without morbidity , 43. AlsThe major benefits of this surgical technique are the capability to successfully achieve complete surgical resection with wide tumor-free margins, protect the presacral vascular and visceral structures, and accomplish these goals through a single and definitive dorsal approach. Trying to accomplish complete removal of GCT during the initial surgery is very important and should be, if possible, the main goal. This allows reaching a favorable prognosis, minimizing the risk of recurrence . NeverthSurgical treatment of sacral GCT is challenging and technically demanding, due to the complex regional anatomy in this area, and the advanced stage of disease by the time diagnosis is made. A well-coordinated multidisciplinary team approach, working in specialized units, is mandatory.en bloc) surgical resection with tumor-free margins, and a comprehensive treatment are essential for local tumor control, best long-term prognosis, and improved outcome with possible cure.Early diagnosis, complete  and delineate the extension of the area to be resected.


Gastric adenomyoma is a rare benign tumor, known to occur in the antrum or pylorus of the stomach. To the best of our knowledge, this is the first case reported in the literature of a gastric adenomyoma in the stomach body, but not in the antrum.We report the case of a 79-year-old Korean woman with a gastric subepithelial lesion in the stomach body. The lesion was observed via endoscopy as a bulging mass in the stomach body with a cystic center. It was confirmed via endoscopic ultrasonography. A laparoscopic wedge resection was performed. A biopsy revealed epithelial and smooth muscle proliferation, and a final diagnosis of gastric adenomyoma was made.Gastric adenomyoma can occur in the stomach body and can be treated completely with surgery. According to the World Health Organization’s histopathologic classification, gastric adenomyoma is a benign tumor of the stomach characterized by a smooth muscle stroma in the glandular structures of the cuboidal epithelium or columnar epithelium . Magnus-A 79-year-old Korean woman was referred to our hospital for an incidentally detected subepithelial lesion in the stomach. Her medical history and alcohol intake, smoking history, and recent drug use were unremarkable. A physical examination did not reveal significant abnormalities. The routine laboratory tests conducted at the time of referral to our hospital were within the normal ranges.Abdominal computed tomography (CT) showed a well-defined, intramural, heterogeneous mass lesion in the gastric body. The mass was oval-shaped and exhibited an enhanced pattern after contrast medium injection. In addition, no enlarged lymph nodes or distant metastasis in other organs around the stomach were observed Figure .In an enMicroscopically, broad interdigitating bundles of smooth muscle were present between the ductal collections. Some of the ducts were dilated and lined with tall columnar epithelium featuring regular, basally oriented nuclei. Also some of them were surrounded by branched glands lined with mucus-secreting cells Figure . AccordiThe patient did not develop any noticeable complications following the operation. Oral intake was initiated on the third post-operative day, and the patient was discharged from the hospital on the sixth post-operative day. Currently, the patient’s progress is being monitored in the outpatient clinic.An adenomyoma is a lesion with observed epithelial and smooth muscle proliferation ,6. The eThe average size of an adenomyoma ranges from 0.6cm to 4.5cm, and these lesions are usually found on the submucosa and sometimes on the muscle layer. This disease can affect patients from 8 months to 82 years of age, and more men are affected than women, albeit this difference is not statistically significant . AlthougChu described the characteristics of a gastric adenomyoma observed by EUS . HoweverAs previously mentioned, although gastric adenomyomas are characterized as benign tumors, there have been reported cases in which these tumors were found alongside adenocarcinomas. Chapple and colleagues were first to report a case of an adenomyoma observed with adenocarcinoma . KanehirWe report that gastric adenomyoma is a very rare disease with differential malignant characteristics that can be observed using EUS. In our patient, we observed that this disease can occur in the stomach body and can be successfully treated with wedge resection of the lesion without a requirement for extensive surgery.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KHY was a major contributor to the writing of the manuscript. DYE collected and interpreted patient data. JHK and SOL were involved with patient care. HSK was involved with formation of the study concept. DWL carried out the endoscopic examination and contributed to revising the manuscript. All authors read and approved the final manuscript.


Lipomas of the larynx are very rare benign lesions; macroscopically, they resemble retention cysts, so their diagnosis is usually made after surgery. A rare case of pediatric paraglottic space Lipoma in an 11-year-old boy is explained. The mass was mobile, soft, without fluctuation or pulsation. CT scan revealed a 5.7 cm cervical fat density with regional lymphadenopathy. After lateral neck incision, a mass located deep in the carotid artery, which was attached to the larynx and which extended to the paraglottic space, was excised completely. Pathologic evaluation revealed Lipoma without any evidence of malignant cells present. This rare differential diagnosis for neck masses in pediatric population should be considered. Benign mesenchymal neoplasms of the larynx include a wide variety of pathologies. One of the rare diagnoses is neoplasm of adipose origin, like Lipoma (0.6%) . Lipoma Lipoma originates from parts of the larynx in which fat tissue is normally located, like the aryepiglottic fold, the epiglottis, and false vocal folds .Though iThe patient, who was presented to the clinic, was an 11-year-old boy with a neck mass he had acquired 10 years earlier. His family explained that this mass enlarged when drinking cold water and when exposed to cold weather. He had no previous major medical illnesses. He had no inflammatory signs, dyspnea, swallowing disorder or hoarseness. The mass was mobile, soft, without fluctuation or pulsation. It was mobile during swallowing. Indirect laryngoscopy was normal. No other mass was observed during the neck examination. CT scan revealed a 5.7 cm cervical fat density with regional lymphadenopathy .th day after surgery. Pathologic evaluation revealed Lipoma without any evidence of malignant cells present.After lateral neck incision, a mass located deep in the carotid artery, which was attached to the larynx and which extended to the paraglottic space, was observed . BecauseIn this case report, a rare case of pediatric paraglottic space Lipoma, that was uncommon regarding its location and the patient’s age, is explained.These types of tumors reveal symptoms such as hoarseness, sensation of a foreign body in the throat, dyspnea, intermittent stridor, dysphagia, nocturnal cough, and dysphonia -9.CT scan is the choice for preoperative imaging. A CT scan or MRI can correctly diagnose the Lipoma before surgery . There are some immunohistochemical markers such as S-100, vimentin, murine double minute 2 (MDM-2), and cyclin-dependent kinase 4 which can also be used for preoperative imaging (CDK4). The treatment recommended is based on complete tumor removal, because it may grow again after incomplete excision. The size of tumor defines the surgical approach . TracheoLong term follow-up of these patients is mandatory, because there is possibility of recurrence after a long period of time. Also, itLipomas of the larynx are very rare benign lesions. A rare case of pediatric paraglottic space Lipoma in an 11-year-old boy is explained. Such a rare differential diagnosis for neck masses in pediatric populations should be considered.


Nodular lymphoid hyperplasia (NLH) of intestine is an extremely rare lymphoproliferative disorder of uncertain etiology. Usually, polyps benign lymphoid tissue are present in the intestinal mucosa. It may present as an asymptomatic or manifest with gastrointestinal symptoms such as abdominal pain, chronic diarrhea, occult bleeding or intestinal obstruction. NLH found in association with common variable immunodeficiency (CVI) constitutes an increased risk for malignant transformation.Report a patient with common variable immunodeficiency and diffuse nodular lymphoid hyperplasia of the small and large intestine.Giardia lamblia in feces. The dosage of immunoglobulins IgG showed 279mg/dl, and IgA and IgM decreased, the diagnosis of CVI being taken. Received intravenous gammaglobulin 400mg/kg. Duodenal biopsy revealed nonspecific chronic inflammation with immunohistochemistry and search for Giardia lamblia negative. The revision blade colectomy reiterated the diagnosis of follicular lymphoid hyperplasia. The patient follow-up in outpatients receiving monthly intravenous infusion of gammaglobulin.RG, 32, male, valued at outpatient consultation in Gastroenterology, Hospital do Servidor Público Estadual de São Paulo in January 2014 due to heartburn and reflux. As personal history, had undergone total colectomy in 2009 for multiple polyps at colonoscopy and diagnosis of familial adenomatous polyposis. Pathology revealed reactive follicular lymphoid hyperplasia. Evolved with loose and frequent stools without blood or mucus. Upper Digestive Endoscopy in 2009: enanthematic pangastritis and light bulboduodenite. A sigmoidoscopy conducted in 2010 showed ileal polyps and ileo-rectal anastomosis with inflammatory pseudo-polyps. Was admitted for further investigation with diagnoses of Inflammatory Bowel Disease and Lymphoproliferative Disease. The abdominal CT revealed lymph node enlargement in several chains and chest CT without evidence of lymph node enlargement. Colonoscopy showed sessile polyps in the ileum and colon mucosa with micronodular pattern. Metronidazole received due to We have reported a case of a patient with CVI that presented NLH. Due to increased risk for intestinal lymphoma, should be kept under constant surveillance by tumor tracking.Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Synovial lipomatosis  is a rare and benign lesion affecting synovium-lined cavities. It is characterized by hyperplasia of mature fat tissue in the subsynovial layer. Although the most commonly affected site is the knee joint, rarely additional locations such as tendon sheath and other joints are involved. We present a case of synovial lipomatosis of the glenohumeral joint in a 44-year-old man. The clinical data radiological studies and histopathologic results are described, as well as a review of the current literature. Synovial lipomatosis is a rare and benign lesion affecting synovium-lined cavities . It mostA 44-year-old right hand dominant chef referred to the clinic complaining of recurrent episodes of right shoulder pain with prolonged asymptomatic periods between them. He described an episode that occurred four years earlier in which he was hospitalized for suspected glenohumeral joint infection. The suspected diagnosis was then based on a combination of severe shoulder pain, globally limited range of motion, fever of 38°C, and elevated ESR and CRP values . At that time an ultrasound examination revealed a large amount of fluid in the glenohumeral joint and the bicipital tendon sheath. A synovial fluid sample was aspirated and a Gram stain showed only a few white blood cells but was negative for the presence of bacteria. The cultures were also negative. The patient's condition improved under nonsteroidal anti-inflammatory treatment and he was discharged. Three years later he had a similar episode for which he was treated as before.He came to our outpatient clinic a few weeks later. His physical examination at that point was unremarkable. There was normal active range of motion as well as normal cuff strength and no signs of instability or impingement. Tests for subacromial and bicep irritation were negative.Plain radiographs as well as a CT scan which were performed a few years earlier demonstrated a medium size Hill-Sachs lesion, without blunting of the anterior inferior glenoid . Only whAn MRI examination revealed large joint effusion and heterogeneous signal intensity within the periphery of the joint, suggestive of synovial hyperplasia .Due to the persistence of the symptoms the patient underwent arthroscopy of his right shoulder which demonstrated abundant yellowish villous synovial tissue in the glenohumeral region. The rotator cuff tendons as well as the articular cartilage were intact. The subacromial space was not involved. After samples were obtained, arthroscopic synovectomy was performed.Histopathological examination of the retrieved tissue showed fragments of synovial villi with no acute inflammation or infection, with evidence of lipomatous metaplasia .The patient's postoperative course was uneventful. Twelve months after surgery his pain has diminished with no recurrence of inflammatory episodes to date.Synovial lipomatosis is a rare benign lesion affecting synovium-lined cavities. It most commonly affects the knee  but rareSince it was first described by Hoffa  in 1904,In 1995 Laorr et al. published the first description of synovial lipomatosis of the shoulder in a 90-year-old patient. The diagnosis was based on clinical findings and imaging, and the patient was treated nonoperatively . In et aSynovial lipomatosis may equally affect males and females, between the second and the ninth decades of life. It has been suggested that adolescent patients present an idiopathic primary form of the disease, whereas older individuals, young and older adults, exhibit a secondary process. Less than a handful of multifocal cases were described in young patients. The etiology was suggested to be linked to systemic conditions in some reports, specifically to fat metabolism due to coexisting short bowel syndrome in one case  and to cClinically, synovial lipomatosis usually presents an inflammatory condition of the joint. Manifestations include painless swelling of the involved joint due to accumulation of synovial fluid. In addition, this condition of unknown etiology is marked by villous proliferation consisting of replacement of the subsynovial tissue by adipose tissue with mature fat cells . Lack ofDiagnosis of synovial lipomatosis is confirmed by histology. Although plain radiography and CT scan may not be very helpful, magnetic resonance imaging criteria have been published as an aid to diagnosis. These may include high signal intensity villous or nodular foci on both T1- and T2-weighted images that are suppressed on short tau inversion recovery (STIR) or fat saturation sequences, while the remaining nonfatty component of the hypertrophied synovium displays heterogeneous high signal intensity on T2 or STIR sequences and intermediate-to-low signal intensity on T1-weighted sequences. Degenerative or posttraumatic changes may be seen in the secondary (but not the primary) type of synovial lipomatosis. The subsynovial fatty proliferation usually presents in the diffuse villous form or in the focal nodular frond-like form. A mixed form of the two patterns may also be seen , 19, 21.In 2003 Vilanova et al. reported a series of 33 cases of synovial lipomatosis in 32 knees and one shoulder in which the lesion was located in the subacromial bursa. Only 12 lesions were confirmed by histology, diagnosis of the remaining 21 joints based on MRI findings only . These fDifferential diagnosis of synovial lipomatosis of the shoulder should include pigmented villonodular synovitis, rheumatoid arthritis, tuberculous arthritis gouty arthropathy and synovial osteochondromatosis. Although clinically these may be indistinguishable from synovial lipomatosis, correct diagnosis can be made based on detailed history, laboratory tests of blood, and synovial fluid as well as imaging, particularly by magnetic resonance.Asymptomatic and therefore undiagnosed cases of synovial lipomatosis should be assumed to exist. When symptomatic, synovial lipomatosis is commonly treated nonoperatively by physical therapy and anti-inflammatory medications aimed at controlling the reactive episode. Failure of these modalities may indicate the need for invasive measures.Treatment of synovial lipomatosis by radionuclide therapy in one case yielded significant reduction of lesion volume at six months . No clinSynovial lipomatosis (lipoma arborescens) is an uncommon intra-articular condition of unknown etiology, with less than a handful of cases reported in the glenohumeral joint. The etiology is unclear, but synovial lipomatosis was suggested to represent a late reaction of the synovium to injury in young and older adults. Although it commonly presents insidiously with local soft tissue swelling and joint effusion, on rare occasions it may manifest with an acute local and systemic inflammatory response that is difficult to differentiate from septic shoulder. Clinical and radiological evaluation may facilitate diagnosis. Arthroscopic synovectomy may result in prolonged remission of symptoms.


The melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign neoplasm of neural crest origin most commonly found in theanterior region of the maxilla. The tumor almost always develops during the first year of life, although in some cases it can be presentat birth. MNTI’s present as a rapidly growing, painless expansile, partly pigmented mass. They are usually unencapsulated, with atendency to occur as a single lesion. Local excision is the treatment of choice and is usually curative. Main sites for recurrences are themaxilla (57%) and the skull/brain (28.6%). Malignant transformation has been noted in approximately 6.5% of all cases and in 2% ofmaxillary tumors. The case of a 3-month old boy who presented with a loose primary maxillary left central incisor is discussed. Thediagnostic and clinico-pathological features as well as tumor management and importance of a timely diagnosis are reviewed. First described by Krompecher in 1918, the melanoticneuroectodermal tumor of infancy (MNTI) is a rare benignneoplasm of neural crest origin.1345The case of a 3 months old boy who presented with acomplaint of a loose primary maxillary left central incisor isdiscussed. The diagnostic and clinico-pathological featuresas well as tumor management and importance of a timelydiagnosis are reviewed.A 3-month old caucasian boy was referred to the dentalclinic at a major children’s hospital by a physician in theemergency department (ED). His young parents had severalconcerns: (1) "Swollen gums" in the upper anterior area;(2) A loose upper left incisor (#F) that had erupted quicklyin just a few weeks; (3) Feeding disturbances caused bythe tooth; (4) Fear that it might be aspirated.The boy was healthy and had no known drug allergies.He was afebrile, playful and active. The extraoral exam wasnot remarkable. The intraoral exam revealed the primarymaxillary left central incisor erupted, very mobile(grade 3), with edematous gingiva surrounding it. No otherteeth or swelling were present although the incisal edge ofthe primary mandibular right central incisor could be felt onpalpation. His parents wanted the tooth removed and declineda radiograph. After a written consent was obtained, apediatric dental resident infiltrated 0.5 ml 2% lidocaine withepinephrine 1:100,000 around the primary maxillary leftcentral incisor which was extracted uneventfully viaforceps. Hemostasis was easily obtained with a resorbablegelatin sponge and gauze pressure. Postoperative instructionswere reviewed with the parents and all questions wereanswered. The tooth presented a fully developed crownbut no root structure.Eleven days later, the pediatric dental resident on callwas summoned to the ED at 11 pm. The parents had broughtthe patient back with complaints of gingival and nasalbleeding, and facial swelling which increased in size steadilyafter the extraction. The patient was not feeding well, hadlost weight and was dehydrated. The extraoral exam showedleft facial asymmetry with the nostril and upper lip beingdisplaced upward with evident bulging of the area .The intraoral exam revealed expansion of the upper left arch,involving both the vestibular and palatal cortices, extendingfrom the anterior region to the first primary molar area,filling the upper gingivobuccal sulcus . The areawas firm, nontender, blue/black in color with purulentexudate present in the extraction site. A computerizedtomography (CT) scan of the facial bones revealed anexpansile, bilobed mass with soft tissue attenuation centeredalong the left upper anterior area, measuring 14 mmmediolaterally at the region of the incisors and 8 mmmediolaterally in the hard palate . Cortical thinningand destruction of the maxilla and hard palate were evident.The orbits were intact and there was no sign oflymphadenopathy.The patient was admitted to the hospital and the oraland maxillofacial surgery and oral pathology services werecontacted for a consult. A radical excisional biopsy wasperformed under general anesthesia by the oral surgeon thefollowing day and two specimens were sent to the lab forexamination. The buds of the primary maxillary left lateralincisor and canine as well as the permanent maxillary leftcentral incisor, lateral incisor and canine were removed withthe mass. The sections studied showed a large number ofnests with lesional cells within a fibrous stroma. Two typesof cells were described: (1) A large cell population that washighlighted by cytokeratin and immunostains which wasconsistent with neural, epithelial and melanotic markers;(2) A population of small, round blue cells that were "veryneuroblastic" in appearance. The final diagnosis was MNTI.Following the surgery, the facial asymmetry disappearedand the boy was able to feed well. The postoperative coursewas uneventful, and he was scheduled to follow-up withthe oral surgeon 2 weeks later and at regular intervalsthroughout the first year to monitor for recurrences. Atdischarge, the pediatric dental service reviewed oral hygiene,diet and caries prevention with the parents as well as futureorofacial rehabilitation the patient may need. He will returnto the pediatric dental clinic at 1 year of age to establish adental home and will then be referred to the hospitalcraniofacial team for follow-up on his craniofacial growth.Early diagnosis and treatment of any pathological conditionis the key to avoid serious sequelae. Late diagnosis of tumorswith rapid invasive growth, such as in the case of TMNI,may compromise a radical resection because of the possibleinvolvement of the adjacent critical anatomic structures.Although our patient did not present a clear bony expansionat the time of the extraction, a high index of suspicion wouldhave led to a quicker identification and resolution of theproblem. Firstly, the gingival tissue surrounding the primarymaxillary left central incisor did not have a healthypresentation. Secondly, the initial thought of the eruptedtooth being a neonatal one was erroneous given that thoseteeth erupt within 30 days of birth. In this case, the patient’stooth erupted around 80-90 days of age. Furthermore, thefully developed crown should have led to the suspicion of anormal primary tooth being displaced and forced to an earlyeruption by a pathological entity. Neonatal teeth rarely presentfully developed crowns. A radiograph of the upper anteriorarea would have shown the lytic lesion and toothdisplacement which would have generated an immediateconsult with the other dental services before the dentalextraction.MNTI’s present as a painless, expansile, partlypigmented mass. They are usually unencapsulated and tendto occur as a single lesion.66356Since its first description, MNTI has been assigned morethan 20 other names, such as pigmented ameloblastoma,retinal anlage tumor and melanotic progonoma, because ofthe variable theories of its origin. The current nomenclaturewas arrived at in 1966 when Borello and Gorlin10Radiographic examination of MNTI’s usually reveals aradiolucency with or without regular margins.6While clinical and radiographic findings may besuggestive of the tumor, histopathological examination isrequired for a definitive diagnosis. MNTI consists of 2different cell populations that form nests, tubules and alveolarstructures within a dense, collagenous stroma. Thesestructures are lined by cuboidal epithelial cells that havegranules of dark brown melanin pigment. The other celltype is neuroblastic in appearance, consisting of small roundcells.Local excision is the treatment of choice and is usuallycurative.612In summary, the case of a 3-month old male with MNTIassociated with premature eruption of a primary tooth ispresented. Despite a short delay in diagnosing the entity, itscomplete surgical removal without involvement of vitalstructures was possible. The patient will be followed by thehospital’s transdisciplinary craniofacial team to treat resultingeruption aberrances and to monitor for recurrences, giventhe potential serious nature of this entity.


Dear Sir,Rhabdomyosarcoma (RMS) is the most common soft tissuesarcoma in infants and children. RMS can occur in any age but approximately 87% of patients are younger than 15 years.Approximately25% of these tumors arise from the genitourinary (GU) tract and another 10% from pelvic or retroperitoneal sites.[1]A 10 year old boy presented with straining, weak stream, sense of incomplete voiding, suprapubic discomfort and occasional dribbling of urine for 6 months. General and systemic examinations revealed no abnormality. Urine examination showed plenty of red blood cells (RBCs) and few pus cells; however the culture was sterile. Hematological and biochemical investigations were normal.Ultrasonography of abdomen suggested a bladder mass of size 5x3x3 cm arising from the left posterolateral wall and impinging on the bladder neck. Kidneys,ureters, retroperitoneum and pelvic cavity were normal. Contrast enhanced CT (CECT) scanreconfirmed the large heterogeneous bladder mass arising fromleft lateral wall of the urinary bladder. Perivesical fat planes were maintained . Metastatic workup was negative.Urine cytology did not reveal malignant cells.On cystoscopy a large faint pinkish polypoidal proliferative growth was seen arising from left posterolateral wall that was extending upto the bladder neck and encroaching almost half of bladder lumen. Left half of the trigone was infiltrated by the mass and left ureteric orifice could not be located.Bladder neck was obliterated by the mass and rest of the bladder was trabeculated. Trans urethral resection of the bladder tumor(TURBT) was done. Histopathological examination showed the presence of elongated cigar-shaped cells with pleomorphic nuclei and skeletal muscle like cross-striations in the cytoplasm, characteristic of rhabdomayoblasts along with abundant population of small,pleomorphic round cells, numerous giant cells and spindle cells; suggestive of RMS.Partial cystectomy was not feasible due to posterior location of the tumor.Patient was treated with 4 cycles of vincristine and actinomycin D based chemotherapy followed by external beam radiation.Patient remained on regular follow up and became tumor free after 18 months. At 28 months of follow up, there is no evidence of tumor recurrence and patient is doing well.Bladder and prostatic primaries account for 50%of genitourinary sarcomas. The tumour originates mainly in the submucosa of posterior bladderwall with particular preference for the regions of bladder neck and trigone [2]. The outcome of children with RMS has improved dramatically over the past 30 years. According to IRS V there are three primary protocols that are based on the risk of disease recurrence with the overall aims of reducing cyclophosphamide and radiation in low-risk groups, evaluating new regimens for unfavourable disease, and applying molecular studies for occult metastasis. The low-risk protocol is recommended for localized embryonal and botryoid histology patients and only vincristine and actinomycin D based chemotherapy is used for node-negative tumors at favorable-site (GU non-bladder/ prostate) and small(≤ 5 cm) node-negative tumors at unfavourable-sites. The three drug VAC regimen is reserved for large(>5 cm) tumor at unfavorable sites with microscopic residual, or positive nodes and for tumor at favorable sites with gross residual or positive nodes.[1] According to IRS V, index patient belongs to low-risk RMS; therefore was treated with two drug chemotherapy regimen  followed by external beam radiation as the standard treatment  Because of the rarity of tumor, no guideline yet has been set for follow up of bladder RMS. We followed up this case with history, physical examination, urine cytology and ultrasongraphy of abdomen and pelvis (3 monthly),CECT (6 and 12 months and then yearly), and cystoctoscopy. Though the chance of recurrence is more in first two years, these patients should be followed for long time as delayed recurrence has been reported.Source of Support: NilConflict of Interest: None declared


Prostate cancer is a commonly diagnosed and treated malignancy, although it rarely presents with cutaneous metastases. In this case presentation, we describe the diagnosis and treatment with radiotherapy of a patient who presented with cutaneous metastases on his chest wall secondary to prostate cancer.In 2006, a 73-year-old Caucasian gentleman with metastatic castration resistant prostate cancer treated with mitoxantrone and prednisolone presented with cutaneous nodules on his chest wall. A punch biopsy diagnosed cutaneous metastases, with histological confirmation with positive staining for cytokeratin, PSA (prostate specific antigen) and PAP (prostatic acid phosphatise). Systemic treatment was ceased due to progressive disease; radiotherapy was used to treat these nodules with a durable clinical response. The patient died five months after initial diagnosis of cutaneous metastases.In this report, a rare metastatic manifestation of a common malignancy is presented. Whilst dermal metastases carries a poor prognosis from reported literature, this is the first report of radiotherapy providing a durable clinical response with relief from bleeding and pain. Prostate cancer is the most commonly diagnosed cancer in Australia, with an estimated incidence of 18,560 in 2012  - when iMetastasis to the chest wall is uncommon, although there is one case report published of cutaneous metastasis to the neck and upper chest . The mecWe report herin a patient with metastatic castration resistant prostate cancer, who presented with cutaneous nodules confirmed to be metastases from his prostate cancer. This was subsequently treated by radiotherapy, with a clinically durable response. It is of interest for the rarity of its presentation, and potential treatment with radiotherapy.In 2006, a 73-year-old Caucasian man presented with nodular lesions on the left chest wall while receiving mitoxantrone and prednisolone for metastatic hormone refractory prostate cancer with skeletal metastases. At the time, mitoxantrone was the standard systemic chemotherapy for this condition, this gentleman was first diagnosed with prostate cancer 9 years previously. Past treatments included androgen deprivation, strontium, carboplatin–etoposide, radiotherapy to the chest wall for pain in the manubrium and sternum; and also with cisplatinum–phenoxidiol . He initially responded well to mitoxantrone with a fall in the serum prostate specific antigen (PSA) and resolution of bone pain.After the eighth cycle of mitoxantrone, the patient reported having skin lesions on his chest. The lesions were non-tender, pink, raised and occurred in irregular clusters just lateral to the nipple towards the mid axillary line, there were no epidermal changes. A clinical suspicion of cutaneous metastases was considered along with the differential diagnoses of a drug eruption and other dermatoses.A punch biopsy was performed on a sample lesion. The pathology showed metastatic adenocarcinoma in the dermis that stained for cytokeratin, PSA and prostatic acid phosphatase (PAP) consistent with prostate cancer Figures  and 2. HChemotherapy was ceased due to progressive disease, thereafter, serum PSA levels continued to rise. The PSA increase coincided with increasing extent, size and pain in the nodules on the chest wall. The lesions close to a previous sternal ulcer became friable and bled easily Figure . Two monThe PSA continued to rise, however, and further symptoms developed including fatigue, leg swelling and dyspnoea. A computed tomography (CT) scan revealed bilateral pleural effusions, the larger effusion on the left was drained. The cytology of the fluid revealed adenocarcinoma cells staining with anti-cytokeratin antibody CAM 5.2, PSA and PAP consistent with metastatic disease.The treated cutaneous lesions did not cause further problems but new lesions were found out of the field of radiotherapy treatment Figure . He contIn prostate cancer, dermal involvement generally presents late in the disease and is associated with poor prognosis , 6. One The mechanism of spread is uncertain – in our case, the finding of pleural effusions with cytology positive for prostate cancer cells in this case raises the possibility of a transmural infiltration of metastases in the chest wall. Histologically, there are multiple reported morphological patterns, and it typically shows immunohistochemistry positivity for PSA and PAP. However, expected morphology and staining may not be present in a poorly differentiated specimen , 11, 12.This patient also had a significant and durable local response to radiotherapy. The symptoms of bleeding and pain completely subsided after treatment with 18 Gy in 3 fractions; although this did not lower serum PSA or stop systemic progression.Treatment options for cutaneous metastases include local excision, intralesional chemotherapy  or radioWritten informed consent was obtained from the patient, prior to his death, for publication of this case report and accompanying images. Furthermore, written informed consent from the patient’s next-of-kin was also sought post mortem, and a copy of the written consent is available for review by the Editor-in-Chief of this journal.


Synchronic occurrence of benign and malignant tumors is extremely rare. Fibrolamellar hepatocellular carcinoma represents 1% to 2% of all hepatocarcinomas, while myxomas represent about half of all the cases of primary tumors of the heart. We present the case of a 53-year-old woman with a left atrial myxoma that was surgically removed. Several weeks later, the patient returned to the hospital with abdominal pain. CT scan showed a mass in the left lobe of the liver that was resected and diagnosed as fibrolamellar hepatocellular carcinoma. As of this writing, the patient is healthy. Hepatocellular carcinoma (HCC) represents 80% to 90% of all liver cancers . AccordiPrimary cardiac tumors are rare, representing 0.0017% to 0.19% of autopsy cases . MyxomasHere we describe the case of a 53-year-old woman who was diagnosed with fibrolamellar hepatocellular carcinoma synchronous with left atrial myxoma.A 53-year-old female with no known medical conditions reported the start of symptoms 4 months before consultation. The patient reported palpitations that exacerbated by cardiovascular exercise and attenuated with rest. The cardiologist requested an ECG and diagnosed sinus tachycardia. Subsequently, a transthoracic echocardiography was performed, and a mass dependent on the left atrium compatible with auricular myxoma was detected. The patient was scheduled for surgery, a median sternotomy for a transseptal approach to the right atrium was performed, and the patient began treatment with IMMUNEPOTENT CRP  which was administered orally to promote postsurgical recovery.The patient underwent surgery without complications and was discharged from the hospital. The mass was diagnosed by pathology as left atrial myxoma with mural thrombus . Three mA CT scan of the abdomen was performed, which showed 8 × 8 × 7.3 cm mass in the left lobe of the liver . The lesAfter postoperative analysis from pathology, the diagnosis was of a 9 cm, Grade III moderately differentiated fibrolamellar hepatocellular carcinoma with clean resection borders  Figures . The patMultiple primary malignant neoplasms (MPMN) are defined as a diagnosis of two or more independent primary malignancies of different histologies/origins in an individual . They arFibrolamellar hepatocellular carcinoma and cardiac myxoma are a rare presentation of synchronous tumors. Ochoa et al. 2011) described one case of hepatocarcinoma synchronous with atrial myxoma . There a1 describFLHCC is usually asymptomatic, and most patients visit a doctor when they note the presence of an abdominal mass. Those patients with symptomatology report vague symptoms such as weight loss and abdominal pain or discomfort . ImagingLiver function tests for patients with FLHCC are usually normal, although sometimes alpha-fetoprotein and aminotransferases show slightly increased levels , 12.FHLCC prefers the left lobe of the liver, and, grossly, it is usually yellow to pale tan and firm, with an average size of 6–19 cm at its longest diameter. About 75% of tumors present a central scar; some tumors present foci of necrosis, hemorrhage, and calcification , 11.Histopathologically, the tumor is composed of large polygonal cells with large vesicular nuclei containing marginalized chromatin and prominent nucleoli with eosinophilic (oncocytic) cytoplasm surrounded by distinctly lamellar stroma. Calcifications and pale bodies are seen in more than 50% of cases , 13. StaMyxomas represent 50% of the very rare cases of primary cardiac tumors. The most frequent location for myxomas is the left atrium, and they usually originate from the endocardium of the atrial septum , 15. GenSurgery is the election treatment for myxoma; it must be performed without delay because of the high risk of sudden death from thromboembolism or valvular obstruction; resection of the tumor must be total , 17.Macroscopically, myxomas are pedunculated and gelatinous, and their surface can be smooth, villous, or friable and, in some cases, they show areas of hemorrhage. Their diameter ranges from 1 to 15 cm, and their weight ranges between 15 and 180 grams [Histologically, myxomas are made up of stellate and polyhedral (lepidic) cells. These tumor cells have small round or oval nuclei and variable amounts of cytoplasm. Myxoma cells can appear singly or in small clusters or chords, and when grouped around small spaces they can simulate glandular structures; they rarely show mitotic activity. Myxomas have abundant, fibrillary, slightly basophilic, mucoid matrix . These tMyxomas show a diffuse positive reaction for the immunohistochemical marker Vimentin and focal expression of CD34, CD68, and SMA . They alThe IMMUNEPOTENT CRP  is a mixture of low molecular weight substances and is a drug capable of modulating the immune response. It has been used for years in clinical trials of multiple types of diseases such as cancer, toxic shock, and postoperative recovery, as in the clinical case presented here, increasing the quality of life of the patients , 22.Synchronous tumors are rare and the coexistence of malignant and benign tumors is rarer still. Additionally, the association of fibrolamellar hepatocellular carcinoma and auricular myxoma is scarcely reported in the literature. In our case, our patient was treated in accordance with the established surgical procedures, received oral doses of IMMUNEPOTENT CRP, and, as of this writing, is completely asymptomatic.


En bloc resection is the only well-established predictor of progression-free survival. Optimal surgical management requires a complex multi-disciplinary approach. Here, we describe two cases of sacral chordoma and review current management paradigms.Sacral chordomas represent more than 50% of all sacral tumors. These slow-growing, malignant lesions present insidiously and are often large and intimately involved with sacral neurovascular and pelvic structures.  Median survival is estimated to be 6.29 years. Five, 10 and 20 year survivals are approximately 68%, 40%, and 13%, respectively [Chordomas are slow-growing malignant neoplasms that represent approximately 1-4% of all primary bone tumors [These tumors almost always arise in the midline axial skeleton suggesting an embryological origin from vestigial elements of the notochord . Recent Radiological workup usually reveals a destructive bone lesion involving the vertebral body on CT, with a corresponding soft tissue mass on MRI that is T2 hyperintense and heterogeneously contrast-enhancing. There is often local invasion into the adjacent disc spaces. From a pathological perspective, chordomas are considered low to intermediate grade lesions. Due to extensive disease progression at the time of presentation and the high rate of local recurrence, these lesions are considered malignant and require multi-disciplinary management.en bloc resection and stereotactic radiosurgery and review current management paradigms for this challenging clinical entity.Here, we report two recent cases of sacral chordoma treated with Informed patient consent was obtained for all participants in this report. No identifying patient information was included,en bloc resection of the lesion. The patient tolerated the procedure well and was discharged home on postoperative day 1. Pathology was consistent with a chordoma with negative surgical margins. A postoperative MRI demonstrated no residual tumor. Given that there was en bloc resection of the tumor with negative margins, adjuvant radiotherapy was deferred. At his three-month follow-up, the patient was doing extremely well with resolved pain, a well-healed incision, and no neurological deficits nor bowel or bladder deficits.The patient is a 34-year-old previously healthy male who presented with three years of progressive sacrococcygeal pain, worse with sitting. He denied lower extremity weakness or sensory changes and did not have any bowel or bladder incontinence. On examination, he had no neurological deficits and had tenderness to palpation over the distal sacrum and coccyx. MRI revealed a T2 intense well-circumscribed sacrococcygeal mass approximately 3 x 2 x 2 cm in size involving the S4 nerve roots Figure . The paten bloc resection and radiosurgery were discussed with the patient. Given her advanced age and invasion of the tumor into the S2 nerve roots, the decision was made to treat the tumor with radiosurgery alone. She was treated with CyberKnife radiosurgery with a dose of 40 Gy in five sessions . Options of Sacral chordomas represent a challenging clinical entity due to often large tumor size and advanced disease progression at the time of presentation. Although treatment can require multiple disciplines and incurs a risk of morbidity and decreased quality of life, surgical resection remains the definitive method of preventing local recurrence and minimizing overall mortality -5.en bloc resection using a posterior approach in sacrococcygeal chordoma. Complete excision of the tumor without contamination of the surgical wound resulted in a 28% recurrence rate compared to 64% with subtotal resection [Kaiser, et al. first demonstrated the superiority of esection .The operative approach to sacral chordomas is tailored to lesion size and relationship to the sacrum, sacroiliac joints, and sacral nerve roots. Combined anterior-posterior approaches may be required in some circumstances. Lumbopelvic reconstruction with instrumented fusion is recommended in cases involving the majority of the sacroiliac joint, or when a total or high sacrectomy is performed and can be achieved using the modified Galveston technique -9. For mWith the goal of total resection, patients with sacral chordomas can experience postoperative morbidity related to motor, bowel and bladder function. The most significant predictors of postoperative function are preoperative function and level of sacrectomy . The sacChordomas are considered to be poorly responsive to traditional radiation therapy techniques . Developen bloc resection is achieved [en bloc resection and initial radiotherapy [The role of adjuvant radiation therapy remains controversial. Several studies have failed to show a benefit of adjuvant therapy when achieved . One grootherapy . There hotherapy . Proton otherapy . Radiothotherapy -22. When recurrence does occur, it commonly involves the soft tissues around the sacrum, including the piriformis and gluteus maximus muscles. There may be a role for reoperation after recurrence but only with complete resection . These cen bloc resection with a multi-disciplinary team of colorectal and wound specialists is the gold standard for limiting recurrence and maximizing survival. The role of radiotherapy in an adjuvant role or in recurrence remains unclear. For patients who cannot tolerate an operation, radiation may provide a less optimal option for disease control in a limited fashion. Sacral chordoma is a complex clinical entity which often presents in a delayed fashion leading to large tumor size and involvement of critical neural elements in the sacrococcygeal region. For those patients who can tolerate the operation,


Collagenous gastritis is an extremely rare disease, both in children and adults. Symptoms vary depending on the extent of collagenous changes in the bowel. In most of the children, iron deficiency anemia and abdominal pain are the presenting symptoms. We present a 15-year-old boy with acute abdomen due to gastric perforation the cause of which was collagenous gastritis. A 15-year-old, previously healthy boy without a history of smoking or drug abuse, admitted to the hospital with acute epigastric pain without dyspnea or heartburn. On physical examination the boy was pale with tachycardia (110/min), but normal blood pressure (110/70 mmHg) and normal capillary oxygen saturation in room air (SPO2 99%). Tenderness in the upper abdomen was present. Laboratory investigations showed mild iron deficiency anemia  with normal infection parameters. X-ray revealed free air under the right diaphragm. Abdominal ultrasound and CT scan were performed in search of underlying abnormalities, but none could be detected. At laparotomy, gastric perforation was found, which was repaired after refreshing the margins and tissue was sent for histopathology. Histopathology of the fragments showed ulceration without demonstrable Helicobacter pylori; recognizable mucosa was lacking. Subsequent gastro-duodenoscopy showed macroscopically a nodular aspect of the corpus with remarkably coarse gastric folds and pseudo-polyps . Histological examination of gastric biopsies showed active chronic inflammation with lympho-plasma-cellular infiltration and a thickened sub-epithelial collagenous band, up to 23 μm, consistent with the diagnosis collagenous gastritis; the duodenal mucosa was normal.The patient was treated with iron supplementation because of persistent anemia. Repeated gastroscopies over the years yielded similar results with collagen bands of up to 22-29 m ; colonoscopy revealed normal colonic mucosa, both macroscopically and on histological examination. At the age of 18 year, the patient is asymptomatic, but needed frequent courses of iron replacement therapy.The first report on collagenous gastritis was published in 1989.[1-3] Over 300 patients with collagenous gastritis (40 pediatric) have been reported, the youngest being 9 months old.[4] It is twice as common in females.[5-8]. Collagenous gastritis in adults is often accompanied by collagenous sprue and (or) collagenous colitis; in children and adolescents it is usually an isolated phenomenon.[7] The etiology has not been completely known yet; an autoimmune background is suggested.[4] The presentation is variable in children including upper abdominal pain, anemia, vomiting, upper gastrointestinal bleeding.. Perforation of the stomach as the presenting symptom of collagenous gastritis is unusual. Leung et al presented a 20-year-old man with perforated gastric ulcers preceding the occurrence of collagenous gastritis by four years.[3] Collagenous gastritis was also reported in literature, in a 15-year-old boy with a bleeding gastric ulcer, but without perforation.[6]The diagnosis of collagenous gastritis is based on characteristic endoscopic and histological findings. At endoscopy, especially in children and adolescents, the gastric mucosa usually looks macroscopically inflamed, with thickened folds and a nodular or pseudopolypoid as was also seen in our patient.[7] Histologically the sub-epithelial layer is irregularly thickened, not extending around the foveolae and glandular tubes. It consists of collagen; typically, entrapped capillaries are present within this layer and the lamina propria contains an increase in intraepithelial inflammatory cells.[4] The collagen layer is normally is 0.30-2.81 μm  and does not seem to change significantly in other forms of chronic gastritis but it is grossly increased to between 15 and 94 μm with a diffuse or patchy distribution in collagenous gastritis.  This phenomenon is pathognomonic; it is found in no other gastrointestinal condition.There is very limited data on the treatment of collagenous gastritis in children and adults . Both acid reduction and corticosteroid treatment seem to be mostly ineffective, although many children become symptom-free independent of the type of treatment. Obviously, iron deficiency anemia can successfully be treated with iron supplementation, a treatment that has to be continued for prolonged period. Collagenous gastritis is easily overlooked on account of non-specific features and can lead to gastric perforation. Histological specimens should always be taken during surgical repair, since the diagnosis of collagenous gastritis can only be established on histological findings.Source of Support: NilConflict of Interest: None declared


Ectomesenchymal chondromyxoid tumours (ECTs) are rare mesenchymal soft tissue neoplasms that typically present as a slow-growing asymptomatic mass on the anterior dorsum of the tongue. Our patient presented with impaired speech articulation and pain associated with upper respiratory tract infections when the lesion on his dorsal tongue would swell, and he would accidentally bite down on it. Microscopically, ECTs appear as unencapsulated, well-circumscribed proliferations of uniform round to fusiform cells embedded within chondromyxoid matrices. Most cases of ECT have been detected in the third to the sixth decades of life, with no sex preference. ECT may cause a range of symptoms that negatively impact patients' quality of life, including pain, dysphagia, odynophagia, bleeding, and, in the case of our patient, impairment of speech. We provide a unique preoperative clinical photograph and case description that should help readers in recognizing this neoplasm. Considering the rarity of ECT presenting clinically as well as in the literature, we believe this report will add to our growing understanding of ECT and its management. We report a case of ECT presenting on the anterior dorsal tongue that was successfully surgically resected under local anesthesia with clear margins, accompanied by a review of the pertinent literature. Ectomesenchymal chondromyxoid tumours (ECTs) are rare mesenchymal soft tissue neoplasms that typically present as a slow-growing asymptomatic mass on the anterior dorsum of the tongue, or, much less frequently, on the posterior tongue . MicroscThe patient is a 51-year-old male with a 15-year history of a slow-growing midline dorsal tongue lesion that was not causing any compressive symptoms. He had not experienced dysphagia or any impairment to his airway; however, the tongue lesion more recently resulted in a change in his speech articulation. He noted that there was only pain associated with upper respiratory tract infections, when the lesion would swell and he would occasionally bite down on it, resulting in mild bleeding. The patient was otherwise healthy. His previous history included diverticulitis in the descending colon, a hernia repair, colonoscopy, and chronic knee pain for which he was awaiting arthroscopy. His medications included ibuprofen and celecoxib and he had no known allergies.On oral examination, a 2.5 cm high × 1.5 cm wide dorsal tongue lesion with normal tongue papillae on the dorsal surface and mucosa on the undersurface was present . The masThe patient was offered excision for definitive management and to establish a definitive diagnosis. This was done under local anesthesia with 5 mm margins. A midline glossectomy was performed with dissection into the midportion of the deep substance of the tongue. The patient tolerated the procedure well and on follow-up the tongue had healed nicely with no evidence of recurrent disease. The final pathology was in keeping with an ectomesenchymal chondromyxoid tumour (ECT) with negative margins.Microscopically, the tumour was lobulated but generally well-circumscribed, extending from just beneath the surface epithelium into the underlying skeletal muscle. It consisted of ovoid to spindle-shaped cells with ill-defined cell borders and uniform nuclei . The celECT is a rare and benign neoplasm of the oral cavity, classified by the World Health Organization (WHO) in the pathologic spectrum of soft tissue myoepithelioma. The entity was first described by Smith et al. in 1995 . In thisSimilarly to Smith et al.'s original findings, the vast majority of subsequently described ECTs have been documented on the anterior tongue surface, although there are a handful of cases in which tumours have been located on the posterior aspect of the tongue , 5 and oIn Smith et al.'s original report, the ECTs had been documented as occurring in men and women aged 9 to 78 years, with a median age of 32 years. The tumour's presence and growth ranged in duration from a few months to 10 years, and tumour size ranged from 0.3 cm to 2.0 cm . A more Along with their original clinical review of ECT in 1995, to help with diagnosis, Smith et al. conducted thorough immunohistochemistry on the 19 cases presented. Results of this demonstrated strong positivity for glial fibrillary acidic protein (GFAP) in nearly all cases (18 of 19) and variable reactivity to CD-57/Leu-7 (8 of 9 cases were positive), occasionally positive for S100 (with these ranging from faintly to intensely positive), nonreactive to intensely reactive for cytokeratins AE1/AE3, and negative for epithelial membrane antigen (EMA) and desmin . In the The immunohistochemistry performed for our patient's ECT showed diffuse positivity for S100 and glial fibrillary acidic protein (GFAP), focal positivity (of variable intensity and extent) for keratin AE1/AE3, neuron specific enolase, smooth muscle myosin heavy chain, and muscle specific actin and desmin. Immunohistochemistry was negative for EMA, CK8/18, CK5/6, CD34, and p63. The presence of staining for markers of both epithelial (AE1/AE3) and muscle differentiation  supports the concept that ECT is related to soft tissue myoepithelioma.A recent case by Shogo and Koda from 2015 described the potential usefulness of CD56 as an adjunct marker for diagnosis of ECT, arguing that its inclusion may result in more frequent encounters and resulting diagnoses of ECT . HistoriDiagnosis of ECT takes into consideration the clinical, light microscopic, and immunohistochemical findings. In particular, the clinical, immunohistochemical, and histopathologic features of ECT still remain to be definitively established, in large part due to the scarcity of ECT in the literature. However, this lack of definition of ECT's features likely also results from it being confused with or mistaken for other entities that share some of its histopathological features. Entities to include in the differential diagnosis include myoepithelioma, nerve sheath myxoma, mucocele, pleomorphic adenoma, oral focal mucinosis, glial choristoma, ossifying fibromyxoid tumour, chondroid choristoma, soft tissue myxoma, and cellular neurothekeoma , 10, 11.Smith et al. originally offered several hypotheses in 1995 regarding the histogenesis of ECT, which is still a subject of dispute and remains to be established. Considering the tumour's nearly exclusive location on the dorsal anterior segment of the tongue, which is known to be derived from the first branchial arch during embryogenesis, it is widely held that ECTs arise from uncommitted ectomesenchymal cells that migrated from the neural crest. This was one of Smith et al.'s hypotheses and remains widely held at this point in time, although theories of muscle cell and myoepithelial cell origins have also been considered , 12, 13.Interestingly, a group of authors (as yet unpublished) have recently demonstrated EWSR1 rearrangement in a subset of 2 of 9 tumours . There wECT is a benign entity, though it can impact important functions such as swallowing and speech, thus affecting patients' quality of life. Surgical excision remains the mainstay of treatment. Smith et al., in their original article, made note of rare recurrences of ECT following excision; in one case, the tumour was reexcised with repeat surgery and did not subsequently reappear, while follow-up information regarding the other recurrent ECT was not provided . More reEctomesenchymal chondromyxoid tumour (ECT) is an uncommon, benign tumour of the oral cavity that typically presents on the dorsum of the anterior tongue. The recommended treatment is conservative surgical removal. Further studies will need to be carried out to better delineate the histologic and genetic origins of this interesting and rare neoplasm.


Granular cell tumors (GCTs) were firstly described by Weber in 1854 and 70 years later by Abrikossoff and classified as benign tumors. Originally considered muscle tumors, they have been identified as neural lesions, due to their close association with nerve and to their immunohystochemical characteristics.GCTs are uncommon tumors and they may arise in any part of the body; they have been mainly observed in tongue, chest wall and upper extremities; less frequent sites are larynx, gastrointestinal tract, breast, pituitary stalk and the female anogenital region.Here we report a case of GCT showing an uncommon localization such as the upper third of the right rectus muscle of the abdominal wall.A 45 year-old woman of Caucasian origin presented to the surgeon with a 6-month history of light pain in the upper third of the abdominal wall.Radiological exams  showed a localized in the right rectus abdominis muscle.After excision, histological and immunohystochemical analysis, with the support of electron microscopy, allowed making diagnosis of granular cell tumor.After fist description by Abrikosoff in 1926 of GCT like mesenchymal tumor of unknown origin, in recent years immunohystochemical techniques definitely demonstrated the histogenetic derivation of GCT from Schwann cells.Granular cell tumors are rare, small, slow-growing, solitary and painless subcutaneous nodules which behave in a benign fashion, but can have a tendency to recur; in rare cases they can metastasize, when they became malignant; there are some clinical and histological criteria to suspect the malignance of this tumor.It is important that clinicians, radiologists and pathologists are aware of the clinical presentation and histopathology of GCT for appropriate management, counselling and follow-up. In our case we had a complete radiological, morphological and immunohystochemical characterization of the lesion and a definitive diagnosis of benignity confirmed by electron microscopy. Granular cell tumors (GCTs) were firstly described by Weber in 1854  and clasThe highest incidence of GCTs is in the fourth to sixth decades of live and a female preponderance has been reported. Clinically, GCTs are asymptomatic slow-growing tumors, normally present as solitary nodules, smaller than 3 cm; only 10–15 % of patients show multiple synchronous lesions; increased familiar incidence is uncommon, however it has been reported [GCTs are uncommon tumors and they may arise in any part of the body; they have been mainly observed in tongue, chest wall and upper extremities , 5; lessGCTs occurring in deep soft tissues are extremely rare, especially those of intramuscular origin , 6–11. DThe differential diagnosis between benign and malignant lesions can be very difficult, because they can display similar histological phenotypes. In order to discriminate malignant from benign forms, six histological criteria have been established : necrosiHere we report a case of GCT showing an uncommon localization such as the upper third of the right rectus muscle of the abdominal wall.A 45 year-old woman of Caucasian origin presented to the surgeon with a 6-month history of light pain in the upper third of the abdominal wall. In her medical history, the patient had undergone surgery for tonsillectomy, appendectomy and carpal tunnel syndrome and was suffering from arterial hypertension and thyroid disease, treated with ACE inhibitor and levothyroxine sodium.Ultrasonography (10 MHz probe), performed in May 2013, showed a 20 mm oval formation localized in the right rectus abdominis muscle with inhomogeneous echogenicity and internal vascular signals.Computed Tomography (CT) scan with contrast, performed in August 2013, showed inhomogeneous solid nodule with maximum transverse diameter of about 16 mm and a longitudinal extension of 30 mm localized in the right rectus abdominis muscle and characterized by moderate contrast enhancement, without clear demarcation on the profile and light anterior extension in the intra-abdominal adipose tissue.Contrast magnetic resonance imaging, performed in October 2013, displayed a 20 mm oval formation, localized in the right rectus abdominis muscle, hypointense in sequences T1-T2-weighted, homogeneously hyperdense in the late phase with subcutaneous outer profile and intra-abdominal inner profile, without signs of infiltration of the underlying peritoneum , S-100 protein (clone S1/61/69 Novocastra Leica Biosystems) and CD68 (clone 514H12 Novocastra Leica Biosystems), negativity for alpha-Smooth Muscle Actin (clone asm-1 Novocastra Leica Biosystems), Muscle Specific Actin (clone HHF35 Novocastra Leica Biosystems), Desmin (clone DE-R-11 Novocastra Leica Biosystems) and CD34 (clone QBEND/10 Novocastra Leica Biosystems); the proliferative index  was less than 1 %  and the histiocytic marker CD68, definitely demonstrated the histogenetic derivation of GCT from Schwann cells .Most of our knowledge regarding GCTs derives from case reports or small case series. Since then, many authors reviewed the reported cases and correlated them with clinical presentations. To date, several controversies about prognostic criteria, treatment strategies and follow-up approaches still exist.Granular cell tumors are rare and account for approximately 0.5 % of all soft tissue tumors. Females are twice as likely to develop GCT, with African-Americans more often affected than Whites . They ocIn rare cases they can metastasize, particularly when they arise in deep to fascia or are over 4 cm in diameter . They caThe best radiological modality for the characterization of GCTs is Magnetic Resonance Imaging (MRI) . Benign We report the case of a patient presenting with a slow-growing, firm nodule in the abdominal wall. After ruling out metastatic origin of the mass by CT scan and MRI, the lesion was surgically removed through an en-block excision with simultaneous removal of subcutaneous tissue, muscle fascia and parietal peritoneum.Pathologic findings were congruent with a benign granular cell tumour of the abdominal wall.Although a GCT usually appears as a solitary small nodular growth and follows a benign course, a malignant potential has been described in a few cases, particularly for large lesions, >4 cm in diameter. Comparisons between malignant transformation and de novo primary malignant tumour are based on primary histologic findings and on the clinical course of the patient, especially in recurrent cases during the follow-up period . MalignaFor descriptive purposes these tumours can be classified into two categories: histologically and clinically malignant type, and histologically benign but clinically malignant type.Although metastasis remains the most important criteria for defining malignancy, not all malignant GCTs truly metastasize. Specific histologic features to predict malignant behaviour include spindling of tumour cells, the presence of vesicular nuclei with large nucleoli, increased mitotic rate, a high nuclear to-cytoplasmic ratio, pleomorphism, and necrosis.Nevertheless, as malignancy can be definitely proven only by clinical findings, especially metastasis, clinical features such as large size, rapid growth, and invasion into adjacent tissue are reported to be more important criteria of malignancy rather than the histologic features of a histologically potentially malignant tumour.The malignant form of granular cell tumours is highly aggressive, responds poorly to radiation or chemotherapy and may sometimes have fatal outcomes, if lesions are present in organs such as lung or liver .Local surgical excision, if complete, is curative for benign GCT. The resection margins should be adequate to prevent a misdiagnosis. If resection margins are involved, wider local excision may be recommended to decrease the risk of recurrence.Recurrence is more likely if the edge of a GCT had an infiltrative and ill-defined pattern, as compared to one with nodular and distinct edges, even with negative margins.However, in a case series of GCTs in the musculoskeletal system by Rose et al., resection margins or depth of tumour had no correlation with the risk of malignancy or recurrence .Although GCTs are uncommon and mostly benign, they have a tendency for local recurrence. Some of these cases may be multicentric at presentation. In rare cases metastases or malignant transformation can occur. Wide local excision is the treatment of choice. Hence it is important that clinicians, radiologists and pathologists are aware of the clinical presentation and histopathology of this condition for appropriate management, counselling and follow-up.In our case we had a complete radiological, morphological and immunohystochemical characterization of the lesion and a definitive diagnosis of benignity confirmed by electron microscopy.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 of this journal.


Haemangioblastomas are uncommon tumours that usually occur in the cerebellum and, less commonly, in the intramedullary spinal cord. The extramedullary spinal canal is an uncommon location for these tumours. Also haemangioblastoma at this site is not known to be associated with polycythemia. We present the clinical, imaging, and histological findings of an adult patient with extramedullary spinal haemangioblastoma and reactive polycythemia. Radiography and computed tomography (CT) revealed a medium-sized tumour that most likely arose from an extramedullary spinal nerve root. This tumour appeared to be slow growing as evidenced by the accompanying well-defined bony resorption with a sclerotic rim and mild neural foraminal widening. Magnetic resonance imaging revealed prominent flow voids consistent with tumoural hypervascularity. CT-guided biopsy was performed. Although preoperative angiographic embolisation was technically successful, excessive intraoperative tumour bleeding necessitated tumour debulking rather than complete tumour resection. Histology of the resected specimen revealed haemangioblastoma. Seven months postoperatively, the patients back pain and polycythemia have resolved. Haemangioblastoma is a benign hypervascular neoplasm that can occur anywhere in the neuroaxis, most commonly in the posterior cranial fossa and within the spinal cord . Up to oA 59-year-old man with type II diabetes mellitus and hypertension was admitted for nontraumatic low back pain of one month's duration. The pain was progressive in nature and associated with numbness and weakness of the right foot. There were no bowel or urinary symptoms. He was a nonsmoker and nondrinker and worked as a construction site worker. There was no significant family history. Physical examination revealed decreased pin-prick sensation in the L4 and L5 dermatomes. The rest of the neurological examination was normal.Full blood count revealed polycythemia with a haemoglobin of 23.8 g/dL . All other laboratory investigations, including the 24-hour urine catecholamine level, were normal. Bone marrow examination revealed no evidence of a myeloproliferative malignancy, indicating that this was a reactive polycythemia.Lumbosacral spine radiograph showed erosion of the right L4 pedicle and the right lateral vertebral body wall of L4 . ComputePreoperative angiography revealed that the hypervascular tumour was supplied by moderately hypertrophied L3 and L4 lumbar arteries. Particle and coil embolisation of these feeder arteries was performed 16 hours prior to surgical resection . SurgeryBack pain, numbness, and weakness were significantly improved on clinical follow-up seven months after surgery while the polycythemia had completely resolved. The haemoglobin level also normalised one month after surgery (12.6 g/dL) and remained normal on further follow-up (seven months after operation), demonstrating how the polycythemia was secondary to the spinal haemangioblastoma. Follow-up MRI lumbar spine six months after operation showed a marked reduction in the size of the right paraspinal tumour . The curSpinal haemangioblastoma occurs less frequently than its intracranial counterpart and comprises about 1.6–5.8% of all spinal cord tumours . Within Histologically, the surgical specimen demonstrated histologic features similar to those found in cerebellar and intramedullary spinal cord haemangioblastomas with characteristic clear stromal cells admixed with a rich capillary network , 9. AlthHaemangioblastomas have a propensity for the central nervous system with immunohistochemical studies demonstrating markers of vascular, glial, neural, fibrohistiocytic, and smooth muscle cell lineages , 9. TheiWe present a rare case of extramedullary haemangioblastoma associated with reactive polycythemia. Preoperative imaging indicated features which should prompt consideration of this tumor when similar cases are encountered in future. The clinical symptoms and polycythemia resolved with tumor debulking surgery.


Benign vascular tumors are frequently found in the head and neck, however, such tumors of the external auditory canal are extremely rare. We report three cases of benign vascular tumors limited to the external auditory canal.A 50-year-old woman was diagnosed during an episode of ear fullness and hearing loss. A 10-year-old boy consulted our department about an episode of recurrent otorrhagia. A 20-year-old man found a bulge of his external auditory canal by chance. Complete surgical resection was performed for the first patient. The second patient underwent electro-coagulation of the lesion. In the third patient, to exclude the possibility of a malignant tumor, a biopsy was performed under local anesthesia. Histopathological analysis demonstrated the characteristic of vascular tumors. The lesion showed remarkable reduction during his treatment with antibiotics and cleaning. He remains under careful observation.In diagnosis, there is sometimes confusion between vascular tumors and malformations. Generally, vascular malformations can be differentiated from vascular tumors since they are present at birth and are generally stable.Decision making about treatment of benign vascular tumors is sometimes confusing because of the difficulty in diagnosis. We performed biopsy for only one of our three cases because we regard that informal biopsy should not be conducted for lesions with difficult hemostic conditions and locations.The online version of this article (doi:10.1186/s40064-015-1113-5) contains supplementary material, which is available to authorized users. In 1996, the International Society for the Study of Vascular Anomalies (ISSVA) advocated that the ISSVA classification  scan demonstrated a soft-tissue mass lateral to the tympanic membrane that filled the right external auditory canal without involvement of the tympanum. There was no evidence of bone erosion. The tympanic membrane and the ossicles appeared normal. Magnetic resonance imaging (MRI) of the temporal bone demonstrated a slightly hypointense external auditory canal mass in T1-weighted images, and hyperintensity in T2-weighted images , more precisely enhanced CT of the temporal bone, and/or magnetic resonance imaging (MRI) are effective to evaluate the extent of the tumor, bone erosions and middle ear involvement. Typical MRI features of vascular lesions are a hypointense or isointense lesion on T1-weighted images, and hyperintensity on T2-weighted images, with intense enhancement on gadolinium administration (Luca et al. It is hard to distinguish Case 2 between vascular tumors and vascular malformations without histopathological examination. Increase of the blood vessels is the findings of vascular tumors in Case 2 and Case 3. It seems that vascular lesions frequently include pyogenic granuloma such as that in Case 3, because histologically the expansion of capillaries was showed and the size of the tumor was decreased by local cleaning and antibiotics administration. Though pyogenic granuloma is benign, it frequently shows rapid enlargement. Pyogenic granuloma in the external auditory canal is also extremely rare. To date, only three cases have been reported in the English literature (Casler et al. Vascular lesions in the external auditory canal might be treated by various manners such as radical resection, laser solidification, electrocoagulation, embolus therapy, sclerotherapy or radiotherapy. In 10 of the 12 reported cases, radical operation was performed for the lesion, whereas the others kept under observation. Zinis reported that there was no need for treatment unless the lesion showed especially rapid growth (Luca et al. We believe that in cases in which malignancy cannot be excluded and antibiotic administration has no effect, we must perform biopsy of the lesion after taking sufficient precautions against bleeding. We reported three cases of vascular tumors limited to the external auditory canal. Such lesions are extremely rare. To date, only 12 other cases of the vascular lesion limited to external auditory canal have been reported in the English literature. Radical resection of the vascular tumors was valuable in improvement of the conductive hearing loss, and electro-coagulation was useful for small vascular lesions at an entrance of an external auditory canal. On the other hand, there is no need to resect some vascular lesions by using antibiotics without some symptoms or malignant disease.


Mixed trophoblastic tumor composed of choriocarcinoma and placental site trophoblastic tumor was diagnosed on histopathology slides review, is a very rare mixed tumor and cured after adjuvant chemotherapy.Twenty-nine years old, Para 2 female presented with 4 months history of irregular vaginal bleeding after an uncomplicated vaginal delivery. Abdominal examination showed 14 weeks uterus with β-hCG level of 14,889 mIU/ml. Slides review confirmed the diagnosis of placental site trophoblastic tumor (PSTT). Patient underwent total abdominal hysterectomy and resection of anterior vaginal wall mass. Post operative (48 h) β-hCG level was 6016 mIU/ml. Final pathology showed mixed trophoblastic tumor composed of choriocarcinoma (CC) and PSTT. Adjuvant chemotherapy started and continued for three cycles after achieving normal β-hCG.PSTT is a rare disease and potentially curable. Differential diagnosis in women presented with vaginal bleeding and a uterine mass in the post partum period must include gestational trophoblastic disease. A rare form of gestational trophoblastic disease (GTD) is placental site trophoblastic tumor (PSTT). It arises from invasive intermediate gestational trophoblasts and constitutes 1–2 % of all gestational trophoblastic neoplasia (GTN) . The incHere we present a rare case of mixed trophoblastic tumor consisting of choriocarcinoma (CC) and PSTT. It was diagnosed after term normal delivery and managed with surgery followed by adjuvant chemotherapy.A 29 years old female, Para 2 presented with irregular vaginal bleeding started 4 months after an uncomplicated vaginal delivery of a term male infant. Her first delivery was also normal without any antenatal or postnatal complication. Otherwise her history was unremarkable. In referring hospital she was diagnosed as a case of retained product of conception on ultrasound. Serum beta human chorionic gonadotrophin (β-hCG) level was 4253 mIU/ml. Dilatation and curettage (D&C) done. Final pathology showed PSTT.She was referred on January 28, 2013 for assessment and management to our Oncology centre at King Abdullah Medical City, Makkah, Saudi Arabia. On examination, a thin lean lady was markedly pale with tachycardia. Abdominal examination showed 14 week palpable uterus. Local examination showed mild bleeding, healthy external genitalia, and healthy cervix/vagina. At that time β-HCG was 14,889 mIU/ml. Her Hemoglobin (Hb) was 7.3 and she received four units of packed red blood cells (RBCs). Review of slides showed a neoplasm composed of proliferation of polyhedral mononuclear intermediate trophoblasts with extensive deposition of fibrinoid material confirming the diagnosis of PSTT.Metastatic workup with CT scan of chest, abdomen, and pelvis done on February 2, 2013 showed innumerable pulmonary nodules scattered in both lungs, largest 8.4 mm in right middle lobe. Seven centimeter (cm) heterogenous mass filled the endometrial canal and extending to the level of cervix. The parametria were slightly hazy but no definite involvement was found with prominent both adenexa. No evidence of ascites nor lymphadenopathy . All other structures were within normal limits.Patient offered surgery  followed by chemotherapy but she refused treatment and left the hospital against medical advice. Four weeks later, she presented to emergency department with heavy vaginal bleeding. Hemoglobin was 6.6 mg/dl and received four units of packed RBCs. Abdominal examination showed 22 week sized uterus. A newly developed anterior vaginal wall mass of 4 × 4 cm was found. CT scan repeated CAP showed interval worsening of pulmonary nodules and enlarged uterus with anterior vaginal mass.Patient agreed for surgery, underwent total abdominal hysterectomy and excision of anterior vaginal mass on March 3, 2013. Intra operatively uterus was found to be enlarged (20 weeks sized) with multiple areas of impending tumor perforation. Both tubes and ovaries were normal with no ascities or any intra-abdominal bleeding. Post-operative course was unremarkable. Repeated β-hCG (48 h post-op) was 6016 mIU/ml. The microscopic examination of uterus showed mixed trophoblastic tumor composed of chroriocarcinma (CC) and PSTT. Invasion of myometrium, and lymph-vascular invasion were identified. The CC component showed typical dimorphic picture consisting of syncytiotrophoblasts and cytotrophoblasts. Immunohistochemical staining for β-hCG revealed positive staining in this component. The PSTT which constitute about 20 % of tumor showed sheets of monomorphic intermediate trophoblasts and fibrinoid material. These cells were strongly positive for human placental lactogen (hPL), and inhibin, and negative for β-hCG, and P-63. Ki-67 proliferation index was high in CC component. . CT scan of the chest showed complete resolution of pulmonary metastasis. Currently she is in remission and under monthly follow up.PSTT is rare disease among the other variants of GTN, i.e., choriocarcinoma (CC) the most common and epithelioid trophoblastic tumor (ETT) that is also relatively rare . It is aPSTT produces significantly lower serum levels of hPL and serum β-hCG than does CC. On the other hand some cases of PSTT with high serum β-hCG have been also reported. PSTT may complicate or follow a normal term pregnancy, abortion or molar pregnancy. It can present months or years after a term gestation with a variety of clinical features like; irregular vaginal bleeding, amenorrhea, nephrotic syndrome, and rarely, polycythemia or virilization. It is usually confined to the uterus at the time of diagnosis, but on later stage it has high risk of metastasis to the lungs and vagina . It is lPSTT is a rare disease and potentially curable. Differential diagnosis in women presented with vaginal bleeding and a uterine mass in the post partum period must include GTD. Gold standards for diagnostic evaluation of GTD is β-hCG and tissue diagnosis. For patients with disease localized to the uterus, surgery remains the mainstay of therapy in the first instance, in contrast to other GTDs, because of it is low chemosensitivity. EMA/CO chemotherapy should be considered for metastatic cases.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 of this journal.