The endoscopic endonasal transphenoidal approach has proven to be a very versatile surgical approach for the resection of small midline skull base tumors. This is due to its minimally invasive nature, the potentially fewer neurological complications, and lower morbidity in comparison to traditional craniotomies. This surgical approach has been less commonly utilized for large midline tumors such as suprasellar germ cell tumors, due to numerous reasons including the surgeon’s comfort with the surgical approach, a higher chance of postoperative cerebrospinal fluid (CSF) leak, limited visualization due to arterial/venous bleeding, and limited working space. We present our surgical technique in the case of a large suprasellar and third ventricular mixed germ cell tumor that was resected via an endoscopic endonasal approach with favorable neurological outcome and no postoperative CSF leak. Intracranial germ cell tumors (GCTs) are rare neoplasms usually localized in the pineal and suprasellar regions and comprise approximately 1% of all primary brain tumors in adults . The peaFor sellar and suprasellar germ cell tumors that are best treated with surgical resection, pterional, orbitozygomatic, and bifrontal craniotomies have traditionally been the most utilized surgical approaches. However, the versatility of the endoscopic transnasal transphenoidal approach provides a less invasive option for the surgical resection of germ cell tumors located in the sellar, suprasellar, and parasellar regions. Presented here is a case report of a large suprasellar and third ventricular mixed germ cell tumor that was resected via an endonasal endoscopic transphenoidal approach, with a favorable surgical outcome, highlighting the safety and utility of this approach to the skull base. Informed patient consent was obtained for this study. Based on our review of current literature, there are only two other reported cases in which an endonasal endoscopic transphenoidal approach was employed for the resection of a suprasellar germ cell tumor. A previously healthy 18-year-old female presented with a three-week history of bitemporal hemianopsia and a one-year history of amenorrhea. Her neurological examination and medical history was otherwise not contributory. The presenting brain magnetic resonance imaging (MRI) is shown below in Figure Abnormal laboratory values included an elevated prolactin level and decreased free T4, B-HCG, FSH, ACTH, LH, and AM cortisol.A combined neurosurgery and otolaryngology approach with neuronavigation was utilized. A lumbar drain was placed for CSF diversion. Using a rigid 0-degree endoscope for visualization, a nasoseptal flap was harvested. Bilateral posterior ethmoidectomies and septostomy were performed, followed by a wide opening of the sphenoid sinus from the clivus to planum sphenoidale. The bony septum was flattened with a high-speed drill and the medial opticocarotid recesses were identified bilaterally. A craniectomy was performed encompassing the floor of the sella and the anterior portion of the planum sphenoidale, just posterior to the cribriform plate. The sella and planum dura were opened, as was the medial opticocarotid recesses bilaterally to gain lateral access to the suprasellar tumor. Horizontal incisions were made in the dura above and below the superior intracavernous sinus. The dura was reflected in a cruciate fashion and the pituitary gland was noted adjacent to the optic chiasm due to its superior displacement by the tumor. The optic chiasm was then identified superiorly; the tumor and its capsule were resected sequentially and sharply dissected off the optic chiasm, using rigid 0-degree and 30-degree endoscopes for visualization. The closure/skullbase reconstruction was performed using non-suturable dural allograft inlay, a nasoseptal flap, and a dural sealant .The postoperative brain MRI is shown below in Figure Intermittent lumbar drainage was utilized for four days and the lumbar drain was discontinued after a clamp trial. A spinal imaging obtained did not show any drop metastases. The patient developed hypopituitarism and diabetes insipidus postoperatively, which were medically treated.The pathology slides highlighted a primitive germinoma component with strong expression of placental alkaline phosphatase (PLAP) and the presence of cartilage, adipose tissue, and mature squamous epithelium in the tumor as shown below in Figure The patient received induction chemotherapy (with carboplatin and etoposide) and proton therapy with 3420 cGy fractionated over 18 sessions. She reported subjective improvement of her vision at her three-month postoperative visit and still had hypopituitarism requiring hormone replacement by her six-month postoperative visit. A six-month postoperative MRI showed no spinal drop metastases and no definite residual or recurrence of the suprasellar and pineal region masses. Based on formal visual field testing, there was significant improvement of her vision by her six-month postoperative visit. Her six-month postoperative brain MRI is shown below in Figure Mixed intracranial GCTs are not rare, representing 25–32% of intracranial GCTs -5. The tOver the past decade it has become clear that the entire ventral skullbase is accessible using an endonasal approach. This has been termed the expanded endonasal approach (EEA) and it provides access to the anterior, middle, and posterior cranial fossa . The EEA reported in some case series.However, EEA produces routinely large dural defects, frequently communicating directly with areas of high cerebrospinal fluid (CSF) flow leak, such as the third ventricle . This waOther challenges in endoscopic skull base surgery are venous/arterial bleeding and the impression of a limited workspace, which limits the ability of the surgeon to work bimanually. However, the use of judicious hemostasis  and adequate bony exposure can improve access significantly and reduce the sword fighting .The significant risk of postoperative CSF leak associated with endoscopic resection of large midline tumors was mitigated in the reported case by placing a lumbar drain for CSF diversion and reconstructing the skullbase with a non-suturable dural allograft inlay, a nasoseptal flap, and dural sealant. While there haven’t been many reported cases of large suprasellar teratoma resection via the endoscopic endonasal approach, we present a case in which this approach was employed with favorable surgical and neurological outcome. The endonasal endoscopic transphenoidal approach offers a minimally invasive approach that obviates the need for brain retraction and its associated temporary (and sometimes permanent) neurological injury. The incidence of postoperative CSF leak can also be significantly reduced with the use of a nasoseptal flap and dural allograft. It is a versatile approach, which may be successfully utilized for larger tumors including suprasellar germ cell tumors, as the operating surgeon’s comfort with the technique increases.


We describe a case of metastatic pancreatic adenocarcinoma treated with neoadjuvant FOLFIRINOX chemotherapy and combined pancreatic multi-visceral and metastatic liver resection in a patient currently disease-free four years after diagnosis. Metastatic pancreatic adenocarcinoma is associated with a poor prognosis. Recent advances in chemotherapy have led to some improvement in prognosis; however, reports of long-term survival are rare.We describe the case of a 63-year-old Professor of rheumatology who presented in October 2011 with a single episode of acute, left-sided back pain and microscopic haematuria. As part of these investigations, he underwent a non-contrast CT which showed no evidence of renal tract calculi. A subsequent contrast-enhanced CT demonstrated the presence of a 4.7 cm heterogeneous but predominantly low-attenuation mass arising from the tail of the pancreas and extending towards the splenic hilum. On the cross-sectional imaging there was loss of the fat plane between the mass and the spleen suggesting direct invasion as illustrated in Figure Furthermore, there was a 1.5 cm low-attenuation area within the medial aspect of segment VI of the liver likely representing a metastatic deposit, as seen in Figure Subsequent investigations included a PET scan, which demonstrated a fluorodeoxyglucose (FDG) positive pancreatic tail primary with at least one liver metastatic deposit in segment 6. He was discussed in the regional hepatobiliary multidisciplinary team (MDT) meeting, where at the end of October 2011, a CT-guided biopsy of the pancreatic tail mass was arranged. Histology confirmed an infiltrative tumor comprising malignant glands, composed of medium-sized cells with abundant cytoplasm but no cytoplasmic inclusions. There was an increased nuclear cytoplasmic ratio; the nuclei were pleomorphic with lobulated to smooth outlines but no nuclear grooves. The nuclear chromatin was evenly dispersed, and each nucleus had a single, prominent, peripherally located nucleolus. Numerous atypical mitotic figures were seen, and a mitotic count of 1-5 mitotic figures per high power field was noted. The features were consistent with a poorly differentiated pancreatic ductal adenocarcinoma. Following further discussion in the MDT, he was commenced on a 6 cycle course of FOLFIRINOX chemotherapy starting 23 November 2011. His CA 19-9 fell from 23 to less than 2 on completion of his chemotherapy. His post-chemotherapy imaging (CT and MRI) revealed a reduction in size of both the primary and the contiguous deposit related to the spleen and the liver metastasis of approximately 50%. It was decided he would receive another 3 cycles of FOLFIRONOX with a view to gaining further systemic response.Following a total of nine cycles of chemotherapy, he was again discussed in the regional MDT, where it was noted that radiologically there had been a significant response; the liver lesion was no longer visible, and the splenic hilar disease was reduced. He had an incidental new pulmonary emboli in the right middle and bilateral lower lobe pulmonary arteries and was commenced on therapeutic, low-molecular-weight heparin--although symptomatically he suffered only grade 1 shortness of breath on exertion.On 28 March 2012, he underwent an open distal pancreatectomy, a splenectomy, a transverse colectomy, and a liver resection of segment 5/6. His post-operative recovery was uncomplicated, and he was discharged on day 9.Histology confirmed a poorly differentiated desmoplastic adenoma with some chemotherapeutic regression and areas of central inflammation due to associated pancreatitis and ductal rupture. The residual carcinoma invaded the capsular spleen into the parenchyma with focal angioinvasion. There was intense fibrosis surrounding the tumor which extended to the peritoneal surface, but there was no peritoneal breach by viable tumor. There was invasion of the colonic mesentery, and viable tumor was present in the muscularis propria, although the resection margins were free from tumor. One lymph node was involved by tumor, probably by direct invasion, but seven other lymph nodes were uninvolved. The mid-pancreas and resection margin of the pancreas were free from tumor. The hepatic parenchymal resection contained metastatic poorly differentiated desmoplastic adenocarcinoma abutting the capsule but not penetrating it, with the margins free from tumor. This was staged as pT3N1M1 V1 R0 poorly differentiated desmoplastic ductal adenocarcinoma of the pancreas with chemotherapeutic regression.He underwent 3 cycles of adjuvant FOLFIRINOX, which was completed in June 2012, and he has remained well since his surgery with ongoing but improving grade 1 neuropathy affecting his fingertips and toes. He has undergone regular surveillance imaging, and his latest CT in October 2015, four years following diagnosis, revealed no evidence of disease recurrence or distant metastases. Metastatic pancreatic adenocarcinoma has a dismal prognosis with a median survival of around only 7 months when treated with standard, gemcitabine chemotherapy alone . TraditiThe landmark PRODIGE 4/ACCORD 11 trial [At the Royal Marsden, we have used FOLFIRINOX for the past 5 years for metastatic adenocarcinoma of the pancreas for patients with good performance status and also in the setting of neoadjuvant therapy for tumor downstaging in locally advanced disease [To our knowledge, this is the first reported case of long-term, disease-free survival following combination multi-visceral and metastatic resection of pancreatic adenocarcinoma treated with neoadjuvant and adjuvant FOLFIRINOX chemotherapy. This case highlights not only the potential benefit of surgery in the era of more effective chemotherapeutic regimes but also the dilemma faced by clinicians and MDT’s taking decisions on individual patients with isolated, hepatic metastases from pancreatic adenocarcinoma in the absence of randomized trials to support these treatment pathways. It is crucial that each case is evaluated on its own merits, all management decisions such as this are taken collectively by experienced clinicians from all disciplines, and that patients considered for these complex surgical interventions--often associated with high morbidity--are adequately counseled as to the potential risks in the context of the available evidence.


Basal cell carcinoma (BCC) is the most common cutaneous malignancy among Caucasians. Rare examples of aggressive and neglected BCC have been reported. Here we report a unique case of a neglected BCC with significant jaw involvement.A 50-year-old female, referred by an otorhinologist, presented with a large ulcer on her chin, which was extended to her mandibular vestibule. The ulcer was 9×5.5 cm in size, and tissue destruction, necrosis was observed in the central portion, and the mandibular bone was exposed. On intraoral examination, tooth mobility and severe bone loss were evident. Due to the primary cutaneous origin of the lesion, BCC was considered as preliminary diagnosis. Biopsy was performed and diagnosis of BCC was confirmed. The diseased mandibular bone was resected and reconstructed with a surgical plate. The soft tissue defect was reconstructed with deltopectoral flap. The patient refused secondary stage plastic surgery.Although BCC is not a lethal malignancy, if left untreated and neglected, it can result in severe destruction, disfigurement, and even mortality.  The nevoid BCC syndrome or Gorlin‘s syndrome is a condition with significant oral, head and neck involvement that occurs at a younger age (under 30 years) and should be recognized by dentists. The syndrome consists of multiple BCCs, odontogenic keratocysts of jaws, palmer, or planter pits, anomalies of the ribs and spine  and calcification of the falx cerebri.2,8 Here we report a unique case of a neglected BCC with significant jaw involvement. The patient underwent mandibulectomy and reconstructive surgery was performed.BCC is mainly located on sun-exposed sites; being the head and neck , are the areas of more incidences.A 50-year-old female was referred by an otorhinologist with a large chin ulcer and toothache. The medical history was unremarkable, having never had a medical consultation due to the poor socioeconomic situation. There was a 20-year history of gradually enlarging black papule on her chin. The patient complained of unpleasant facial appearance and a dull pain in the chin region. On extra oral examination, there was a large ulcer (10×6.5 cm) with a firm and indurated margins on the chin extending to the mandibular vestibule and necrosis was evident in surrounding bone . On palp5 Here we report a unique neglected case of BCC with extensive mandibular and intraoral involvement. At the first visit, one might consider lesions with mandibular origin, but further examination and accurate history taking led to the diagnosis of BCC. BCC has a gradual growth without any pain or complaint, which may lead to negligence. Occasionally, BCC behaves aggressively and invades deep structures, recurs after treatment and metastasizes to regional and distant lymph nodes.5 BCC has several types due to clinical and histopathological characteristics as nodular, superficial, micronodular, morphemic form, infiltrating, pigmented, metatypic and fibroepithelioma of pinkus.3 Nodular variant is the most frequent form of BCC and, if left untreated, may result in a large eroding tumor with central depression, necrosis of underlying tissue and raised and rolled borders.2,4 There are several reports about aggressive and neglected BCC of the head and neck with terrible clinical outcomes. Asillian et al. reported on a 58-year-old man with an extensive BCC and signs of cranial nerve involvement with a large, infected ulcer (15×15 cm) on his scalp. The patient had skull bone destruction, osteomyelitis, mastoiditis, cranial nerve paralysis, and radiographic features of the skull base and upper cervical soft tissue involvement. Pathological studies revealed an infiltrating form of BCC. The patient provided a history of receiving an unknown amount of radiation to the scalp for the treatment of tinea capitis during childhood. The patient died because of cranial involvement.9 Rishiraj et al. reported on three cases of BCC with maxillofacial involvement. One case had extensive ulcer affecting right facial structures such as antrum, orbit, and hard palate. A purulent discharge and necrosis was evident. The patient refused any treatment. Other cases were treated by radiotherapy.3,9Most BCCs involve the head and neck region (80%) and the reminders involve the trunk and limbs.7 Hatano et al. reported on two cases of BCC that developed and invaded the preorbital and lacrimal region.8 Nagler and Laufer reported on three patients with BCC lesions invading their jaws, as a consequence of which either their mandible or maxilla had to be partially resected.10Sebastian reported on a 77-year-old with infiltrating ulcer from the eyelid to ocular globe. The biopsy confirmed BCC. Cranial tomography showed destruction of the orbital inferior wall and infiltration from papyraceous lamina to the ipsilateral maxillary antrum. A superstructure maxillectomy with left orbital exertion was performed. Eight months after the procedure, the patient remained without symptoms of tumoral activity.5,6 The goal of BCC treatment is complete excision of the tumor with the preservation of surrounding structures and careful follow-up to detect recurrence or new primary tumors if BCC is operable.9 In some areas such as orbit, nose, and external ear, surgery is complicated and other forms of treatment must be considered. The rate of recurrence is about 1% with Mohs surgery and up to 10% for other forms of treatment. Smaller basal cell carcinomas are less likely to recur than larger ones. BCC rarely spread to other parts of the body, but if involves vital structures such as calvarium, it can be fatal. Our case presented with a destructive ulcer in the chin with mandibular involvement. Preliminary diagnosis was BCC due to the prolonged clinical course, enrolled borders, and patient’s negligence. Differential diagnosis consisted of necrotizing ulcerative gingivitis with facial involvement (noma), leishmaniasis, Wegener granulomatosis, deep fungal infection, and amelanotic melanoma. Amelanotic melanoma was ruled out because of a long clinical course of the lesion. Leishmaniasis cannot cause bone necrosis and has a moderate clinical course. Noma was excluded, because the lesion was initiated from the skin and the patient reported bone involvement as a secondary complaint. In addition, cytological evaluation did not reveal spirochetes. The absence of respiratory symptoms and the location of the lesion, ruled out deep fungal infection and Wegener granulomatosis. The preliminary diagnosis was confirmed by biopsy and the patient underwent surgery. No other treatment such as radiotherapy and chemotherapy was necessary. The patient refused second stage plastic surgery. The final outcome was an acceptable reconstruction, but the lips remained incompetent.There are many treated modalities for BCC such as surgical treatment, microscopic controlled surgery (Mohs surgery), electric cauterization and curettage, cryotherapy, roentgen therapy, laser treatment, 5-flourouracil, imiquimod, interferon alpha, and photodynamic therapy.BCC is a dermatologic disease, with a high prevalence in the head and neck but rarely can involve maxillofacial bones and oral cavity. Therefore, dentists should be familiar with this disease and possible unpleasant outcomes.


Mycobacterium avium complex (MAC) lung disease presenting as a solitary pulmonary nodule (MAC-SPN) is often asymptomatic, is more common in middle to old age, and mimics lung cancer or tuberculoma. We report herein a case of MAC-SPN in an immunocompetent young adult patient, presenting with persistent chest pain and a subacutely progressive nodule with high intense 18F-fluorodeoxyglucose uptake. Histological examination of resected specimens revealed pleurisy, which is a rare finding of MAC-SPN.18F-fluorodeoxyglucose uptake in the nodule. Owing to his continuous chest pain and subacutely progressive nodules, wedge resection was performed using video-assisted thoracoscopic surgery. Histological examination revealed an epithelioid granuloma and pleurisy, and the lung tissue culture was positive for mycobacteria identified as M. avium.A 36-year-old Japanese male presented with chest pain and a subacutely progressive pulmonary nodule. Positron emission tomography-computed tomography showed high intense This is the first report of MAC-SPN occurring with persistent chest pain, suggesting that MAC should be considered in the differential diagnosis of a solitary pulmonary nodule, even for patients who experience persistent chest pain. As in the present case, surgical resection with video-assisted thoracoscopic surgery is a reasonable approach to the diagnosis and treatment of MAC-SPN with possible malignancy, especially as MAC can be diagnosed using resected lung tissue culture with histological confirmation. Mycobacterium avium complex (MAC) lung disease commonly presents as upper lobe fibrocavitary or nodular bronchiectatic forms of pulmonary disease and occasionally presents as a solitary pulmonary nodule (MAC-SPN) [18F-fluorodeoxyglucose (FDG) uptake. Histological examination of resected specimens revealed pleurisy, which is a rare finding of MAC-SPN.MAC-SPN) . MAC-SPNMAC-SPN) –4. We reA 36-year-old Japanese male was referred to our hospital because of persistent chest pain and a subacutely progressive pulmonary nodule. Four weeks prior to his referral to our hospital, he began to experience chest pain that increased in severity on inspiration. He visited a private hospital the following day. He had no family or personal history of nontuberculous or pulmonary disease. Chest computed tomography (CT) showed an irregularly shaped solitary nodule in the periphery of the left lower lobe with microcalcifications and pleural indentation. On CT at a follow-up appointment 2 weeks after the initial consultation, the nodule was enlarged Fig. , b. To dParagonimus westermani or P. miyazaki, were unremarkable. Two weeks after the referral, positron emission tomography (PET)-CT showed intense FDG uptake in the nodules, with a maximum standard uptake value (SUV) of 13.94 , and antibody to .94 Fig. .Fig. 2PoMycobacterium avium via DNA-DNA hybridization. His chest pain disappeared almost simultaneously. Anti-interferon-γ autoantibodies were negative. We plan to initiate anti-MAC chemotherapy  once these adverse effects have abated, for which the patient is being closely monitored.Owing to his continuous chest pain and subacutely progressive nodules, which could not be diagnosed using CT-guided lung biopsy, wedge resection via video-assisted thoracoscopic surgery was performed in the left lower lobe. The histological examination revealed an epithelioid granuloma, with caseating necrosis and calcification, and pleurisy over the visceral pleurae Fig. –d. Althoet al. reported the first case series in 1981 [M. avium. A report of MAC (not MAC-SPN) pleurisy in 16 cases with pleural effusion included a patient who was younger than the patient in the present case [To our knowledge, this is the first report regarding MAC-SPN presenting with persistent chest pain and induced by histologically confirmed pleurisy. Since Gribetz  in 1981 –10 and 1ent case . The facent case , 13, furIn the present case, lung cancer and tuberculoma were included in the differential diagnosis of SPN prior to the definitive diagnosis of MAC-SPN. Distinguishing MAC from tuberculoma in cases involving an SPN is very difficult. In a report of 26 patients with tuberculoma and 15 patients with MAC-SPN , 10 of tIn the present case, surgical resection of MAC-SPN was performed, and we plan to initiate anti-MAC chemotherapy once the adverse effects have abated, although the significance of chemotherapy for MAC-SPN following surgical resection remains controversial. In the literature, of 45 patients with MAC lung disease presenting as an SPN, surgical resection was performed in 16 patients. Of the 14 patients who were administered anti-MAC chemotherapy after the surgical resection, none experienced recurrence; however, this was also true for the remaining 2 patients who were not administered anti-MAC chemotherapy . ChemothWe report a case with MAC-SPN with high-intensity FDG uptake and histologically confirmed pleurisy. MAC should be considered in the differential diagnosis of an SPN, even for patients who experience persistent chest pain. Surgical resection with video-assisted thoracoscopic surgery is a reasonable approach to the diagnosis and treatment of MAC-SPN with possible malignancy, as MAC can be diagnosed using resected lung tissue culture with histological confirmation.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.


Congenital granular cell tumor is a rare benign neoplastic growth affecting the gingival mucosa of neonates. Prenatal ultrasound diagnosis has recently come to focus and in spite of several reports on immune-histochemical and other advanced marker studies, the cause and origin of the lesion remains debatable till date. Review of literature on prenatal diagnosis and histopathology along with immunohistochemistry is discussed.  Introduction:Congenital Epulis (CE), or congenital granular cell tumour (CGCT), is a congenital benign rare tumour of the newborn. The original first description of the lesion was dated in 1871 by Neumann [1]; approximately 250 cases have been reported since then [2]. Other terminologies for CGCT described in literature are congenital epulis, granular cell rabdomyoma, congenital myoblastoma, or Neumann’s tumour. The term 'epulis' has been used, but this simply means swelling on the gingival. It was suggested to be discontinued and congenital granular cell tumour to be used in the literature. Currently “congenital epulis of newborn” is universally accepted and frequently employed terminology in the literature [3]. The congenital gingival granular cell tumour is most frequently seen on the alveolar median ridge of the maxilla than alveolar ridge of mandible (1:3). Incisor-canine area is commonly affected. It has a female preponderance. Incidence rate of the CGCT is unknown but suggested to be 6 per million [4]. The clinical diagnosis of CGCT is almost obligatory when a solitary tumour of the gingiva is present at birth. The presentation is unique at birth as fibrous mass arising from the gingival mucosa or alveolar ridge of the maxilla or mandible. CGCT appears sporadically and has no familial tendency [5]. It most commonly originates from the anterior alveolar ridge, with the maxilla being involved twice as much as the mandible [6], though the lesion can arise from the tongue, palate, skin, the subcutaneous tissue, skeleton muscles, the vocal chords, and in small number cases from the rest of the body. This pedicular tumor has a smooth or lobular surface and a firm, rubbery consistency [7]. Ultrasonography confirms diagnosis as early as at 26 weeks of gestation or in third trimester of pregnancy [8]. The histogenesis of CGCT has been long debatable, as various authors suggested different source of origin of the tumour. The proposed source of origin includes undifferentiated mesenchymal cells [9], odontogenic epithelial, pericytic, and fibroblastic, histiocytes, nerve-related, smooth muscle, and primitive mesenchymal cells [10]. The histogenesis remains still unclear despite several studies. Though spontaneous regression of the lesion has been reported , surgical excision is the only valuable therapeutic option which can be beneficial to both mother and newborn. Recurrence or malignant transformation is not yet mentioned in available literature.SiteTypically, a single tumour is present (90%), ranging in size from several millimetres to several centimetres [13]. Multiple lesions (10%) has been reported involving either or both jaws and have been described with associated abnormalities of the nasal bridges and septum [13-15]. Classically, it arises from the pre-incisor-canine area [11]. Literature survey has suggested that maxilla as most common site of involvement [Fig. 1].Prenatal DiagnosisPrenatal imaging of congenital lesions of oral cavity is possible by ultrasound (USG) or Magnetic resonance Imaging (MRI) and can be helpful in planning the delivery as well as postnatally in demonstrating the congenital lesion and treatment planning. With the help of Ultrasonography or MRI, tumor mass can be identified, mainly in last weeks of pregnancy [16]. Ultrasonography or even MRI can only show the presence of tumor mass suggestive of epulis, but the diagnosis could not be conclusive. However, few studies indicated in past has state successful prenatal diagnosis of congenital epulis using ultrasonography [5]. Postnatally, it may interfere with feeding [14], or respiration. Prenatal diagnosis of CGCT can aid in counselling the parents as to the nature and treatment of the abnormality, as well as the potential risk of airway obstruction and intraoperative complications. Prenatal ultrasound investigation is important, since large tumours may interfere with vaginal delivery and a caesarean may be required . The discovery of a tumor mass on a foetus may help the intervention planning as well as to prepare the parents mentally for eventual measures that may occur in the newborn. Prenatal diagnosis of the lesion on ultrasonography was reported in the literature as early as 26 to 38 weeks [Table 1] which is mostly coincide with third trimester of the pregnancy. It’s very striking that lesion is not detectable till that period, reason for this are unclear. Kusukawa et al. have demonstrated a positive correlation between tumor size and estimated fetal weight [17].PathogenesisIt has unique female preponderance . Based on the theory supported by the experimental production of CGCT in a mouse following injection of endogenous hormone, this characteristic finding was suggested to be attributable to presence of endogenous hormones and sudden regression of mass after birth [14]. This was subsequently failed proved as there was no detectable presence of these endogenous hormone receptors with the lesion tissue. Trauma, due to finger sucking in utero, was suggested not considered significant . Recently, it was suggested that it may be a local metabolic or reactive change [19]. Table 1Supplementary PDF file supplied by authors.Click here for additional data file.Table 2Supplementary PDF file supplied by authors.Click here for additional data file.It most commonly presents as an isolated finding with no known association with other congenital anomalies though reported to be associated with neurofibromatosis, polydactyly, binder syndrome, congenital goiter [20], and bilateral transverse facial cleft [21]. Rarely, shows association with absence of underlying tooth/germ. Majority prenatal associated complications reported are polyhydramnios, obstructed deglutition of amniotic fluid whereas postnatally causes midface hypoplasia, incisor hypoplasia, severe feeding and respiratory/aesthetic problems. As there is potential risk of neonatal respiratory distress, it was suggested that prenatal diagnosis becomes prerequisite for uneventful pregnancy. Ultrasonography is crucial in such cases.Differential DiagnosisDiagnosis of congenital epulis can usually be made on characteristic clinical findings. Clinical differential diagnosis (D/D) is broad based and is depends upon site of involvement, size velocity of growth and possible accompanying lesion or other developmental anomalies like Epstein pearls, granular cell tumour, vascular malformations and neuroectodermal tumours of infancy.Predominance of female patients, tumor location on maxillary anterior region, presence at birth and absence of growth potential and the possibility of spontaneous remission without any intervention could rule out majority of the D/D. Various lesions that can be considered prenatally are congenital malformations, dermoid cyst, haemangioma, lymphatic malformations, melanotic pigmentation neuroectodermal tumours of infancy, rhabdomyosarcoma, granular cell tumor, oral teratoma-epignathus as well as other possible diagnoses such as fibroma, lipoma, leiomyoma, rhabdomyoma, peripheral giant cell granuloma, pyogenic granuloma, neurofibroma, myxoma, hemangioma, lymphangioma, and alveolar lymphangioma [10]. The location and sonographic appearance aid in differentiating these masses. Teratomas often contain calcifications. Hemangiomas arise externally from subcutaneous tissues and may be solid or cystic in appearance. The accurate diagnosis has been further complicated by its similarity, histologically, with the Granular cell tumor/ myoblastoma (GCT), which occurs in adults at a number of intraoral sites, such as tongue. CGCT can be separated from Granular cell tumor (GCT) by location, patients age, absence of cytoplasmic hyaline granules, solid growth pattern, pericytic proliferation, attenuated overlying epithelium, negativity of lesion tissue to S- 100 . d. ImmunohistochemistryThe immunohistochemistry of the tumor is diverse in newborns and adults. Congenital granular cell tumor/epulis is S-100 negative and does not show differentiation to specific cell type . Possible histological origins of the epulis may include epithelial and undifferentiated mesenchymal cells, pericytes, fibroblasts, smooth muscle, nerve-related cells, and myofibroblasts [22]. It has been suggested to be a nonneoplastic, degenerative, or reactive lesion [23].HistopathologyCharacteristic histological findings shown by these congenital epulides include large round cells with granular, eosinophilic cytoplasm and small eccentric nuclei and a delicate fibrovascular network separating the cells. Histopathologically, it known that congenital epulis consist of granular cell and is similar to the adult granular cell tumor, but there are some differences such as pseudo-epithelialomatous hyperplasia, lesser vascularity, more conspicuous nerve bundles than congenital epulis [Table-3] [24]. Pseudoepitheliomatous hyperplasia of the oral mucosa on the tumor surface is seen in 50 % of adult GCT cases. but is usually absent in CGCT cases. Spindle cell variant of granular cell Cases with accompanying pseudo-epitheliomatous hyperplasia or ulceration may mimic squamous cell carcinoma [25]. TreatmentTreatment is with surgical excision; with few cases of spontaneous regression has been reported [26]. After surgical removal, no recurrence or malignant changes have been reported even after incomplete excision, and spontaneous regression may occur [12]. Any Delay in operation can lead to airway obstruction and feeding difficulty. The tumour should be removed during the immediate postnatal period and is usually achieved without serious consequences. If the lesion is smaller conservative approach can be taken to avoid unwanted surgery. Prognosis in CGCT is good as there no reported case of recurrence even after the incomplete removal of lesion [10]. ConclusionThe main areas of controversy surrounding the congenital epulis remain with the exact aetiology, growth, and progression of the lesion in the foetus. The lesion still poses scope for further research in exact etiopathogenesis. Prenatal Diagnosis is an important aspect to be considered specifically in this entity. Treatment planning should be based on the prenatal diagnosis, size of the lesion etc. Source of Support: NilConflict of Interest: None


Cardiac fibromas are benign primary tumors composed of connective tissue and fibroblasts. These uncommon tumors are primarily found in the pediatric population, and their prevalence among the adult population is exceedingly rare. We report a case of an adult with nonspecific symptoms, who was subsequently found to have a solitary mass located in the left ventricle. This case highlights an unusual finding in an adult who through various imaging modalities, surgical excision, and immunohistological analysis was found to have a cardiac fibroma. Cardiac fibromas are benign primary tumors composed of connective tissue and fibroblasts. These uncommon tumors are primarily found in the pediatric population , and theA 60-year-old woman presented with an acute onset of fatigue, shortness of breath, chest pain, and palpitations, following an episode of bronchitis. The patient also had a 7-pound weight gain over the past 1-2 weeks. She was found to have moderate pericardial effusion, and during preliminary imaging for echocardiographic guided pericardiocentesis a mass was noted on the epicardial surface of the left ventricle. The pericardiocentesis was deferred, and the patient underwent further diagnostic imaging.An echocardiogram conclusion was a large, well-circumscribed epicardial mass on the left ventricular surface of the heart, not consistent with a left ventricular pseudoaneurysm. The mass was solid, and it appeared to sit on top of the normal myocardium. Cardiac MRI with and without contrast showed an approximate 4 cm broad based attachment to the inferolateral myocardium of the left ventricle apex Figures . The masComplete surgical resection was carried out under cardiopulmonary bypass. The operative finding was 5.5 × 4.0 × 3.5 cm tan-brown colored mass at the posterior lateral portion of the heart near the apex . The tumSectioning revealed tan-white, rubbery, whorled, calcified cut surfaces . NeitherThis case highlights an extremely unusual finding in an adult who through various imaging modalities, surgical excision, and immunohistological analysis was found to have a cardiac fibroma in the left ventricle. While cardiac fibromas are rare solitary lesions that lack metastatic potential, they are the most common neoplastic cause of life-threatening arrhythmias and even sudden death. Largely dependent on the size and location of the tumor, patients may present with clinical manifestations relating to conduction defects, ventricular arrhythmias, congestive heart failure, and hemodynamic compromise , 4.In our patient, the presenting symptoms were very nonspecific, and there were no signs of any conduction abnormalities, congestive heart failure, or hemodynamic instability. Incidental findings of a pericardial effusion and a mass on an echocardiogram prompted further investigation by cardiac MRI and CT. Surgical resection appears to be a safe and effective method for treatment.


Dysmetropsia  most commonly results from retinal pathologies, epileptic seizure, neoplastic lesions, viral infection, or psychoactive drugs. Vascular lesions are an uncommon cause of dysmetropsia. Vascular hemimicropsia, although rare, has been more frequently described in the literature, whereas hemimacropsia from acute ischemic injury is exceedingly rare. We describe a patient presenting in the emergency room (ER) with visual perception disturbances characterized by a distorted perception of the size of objects, compatible with left hemimacropsia. Magnetic resonance imaging (MRI) of the brain showed an acute occipitotemporal ischemic injury corresponding to the posterior cerebral artery (PCA) territory. The location of the lesion is consistent with previous case reports that suggest that hemimacropsia is associated with the occipitotemporal projection, which plays a decisive role in the visual identification of objects by interconnecting the striate, prestriate, and inferior temporal areas. The difference of our case as compared to previous case reports is that the lesion in our patient spared Brodmann area 17  and therefore did not present symptoms of quadrantanopsia. Instead, the patient presented isolated hemimacropsia, therefore suggesting that the anatomical lesion causing hemimacropsia is located in the ventral portion of the occipitotemporal projection, more specifically Brodmann areas 18 (parastriate) and 19 (peristriate). Dysmetropsia refers to the collective group of perceptual alterations including macropsia, micropsia, teleopsia, and pelopsia , 2. ThesA 58-year-old male patient with history of hypertension and diabetes mellitus presented to the ER with 2 days history of visual perception disturbances characterized by distortion of the size and shape of objects, which he described as perceiving a basketball player's hand as larger than normal when visualized in the left visual field. The altered visual perception lasted 2 days. He also complained of bifrontal headache and transient horizontal diplopia, which had subsequently resolved. In the ER the patient did not present with headache, scintillating scotomas, vision loss, nausea, or vomiting. He did not refer to twitching of face or extremities and had no loss of consciousness.Neurological examination revealed full visual fields. Visual acuity was 20/20 in both eyes. Pupils are equal, round, and reactive to light and accommodation, and extra ocular movements are intact. There was no hemianopia or diplopia. EEG showed slowing to 6 Hz in the right occipital region but no spike wave discharges suggesting an interictal epileptic focus.Magnetic resonance imaging of the brain showed an acute occipitotemporal ischemic injury corresponding to the PCA territory, affecting Brodmann areas 18 and 19 involving the cuneus . An angiMicropsia is more frequently reported than macropsia, but both are rather infrequent phenomena, even more so if occurring as isolated symptoms . It has The difference between our case and previous case reports presenting hemimacropsia is that the lesion in our patient spared Brodmann area 17 , which was consistent with symptoms because the patient did not present quadrantanopsia (would be expected with a lesion in this location). Instead, the patient presented isolated hemimacropsia, therefore suggesting that the anatomical lesion causing hemimacropsia is located in the ventral portion of the occipitotemporal projection, more specifically Brodmann areas 18 (parastriate) and 19 (peristriate).


Root canal system is complex to understand because of its unpredictable nature. It differs for different teeth and for the same teeth in different individuals. Successful endodontic therapy thus depends on the clinician's ability to anticipate and look for these variations. A mandibular first molar with six root canals represents a rare anatomical variant, particularly when four canals are found in distal root. This case report discusses successful nonsurgical endodontic management of two-root mandibular first molar with four distal canals and two mesial canals reported for the first time in Indian population. Cone beam computed tomography was used as a diagnostic method to confirm the position and presence of 4 root canals in the distal root. Blayney in 1932 very aptly said that “probably the first requisite for successful operation is that the operator should be thoroughly familiar with the anatomy of the region” . This coComplete disinfection of the entire coronoradicular space harbouring microorganisms and infected or necrotic pulp is essential for successful endodontic treatment. This necessitates clinicians to have a thorough working knowledge of radicular tooth anatomy along with a three-dimensional appreciation of internal anatomy of root canals with its possible variations . AdvanceThe most common root canal morphology of MFM is the presence of 2 roots with either 3 or 4 root canals . There aA female patient aged 18-year reported to the Department of Conservative Dentistry and Endodontics of Jodhpur Dental College General Hospital, Rajasthan, India, with the chief complaint of severe pain and swelling in the lower right back tooth region since 2 days. She also complained of food lodgement in the same tooth since 6–8 months. Patient's medical history was noncontributory and vital signs were within normal limit.Extra-oral examination showed a soft diffuse swelling on the right lower border of the mandible extending up to the angle of the mandible. Clinical examination revealed a deep proximoocclusal carious lesion in relation to right MFM. The tooth was severely tender on vertical percussion and palpation. There was no sinus tract present in the involved tooth. The periodontal status was normal (probing depth < 3 mm) with no mobility. The tooth gave a negative response to all three pulp vitality tests .Radiographic examination  revealedCBCT was advised to further substantiate this rare root canal anatomy and for the better understanding of the root canal configuration. An informed consent was obtained from the patient before proceeding with the CBCT of the mandible. The examination was performed with a CBCT Unit Kodak 9300 3D Imaging system with a voxel size of 0.20 mm × 0.20 mm × 0.20 mm and 5.5 cm field of view. The images were reconstructed using C S 3D Imaging Software v 3.2.9 . Complete morphology of the root canal system was obtained in coronal, axial, and sagittal sections of 0.2 mm thickness. CBCT  revealed Gutta percha  and AH plus sealer . Owing to the close proximity the distal canals to each other, they were not separately appreciable in the obturation radiograph but they could be clearly visualized in the CBCT and preoperative radiograph. Core buildup was done with composite  and the patient was advised a full coverage crown  for 2-3 minutes in each canal. Final irrigation was done with 17% ethylenediaminetetraacetic acid (EDTA) solution and dried with absorbent paper points . Master cone radiograph  was takege crown . Patientge crown . PatientLiterature has innumerable studies reporting consistently teeth with aberrant root canal morphologies –12. InteMartínez-Berná and Badanelli reported the first case of a third canal in the distal root of MFM and termed it as distocentral root canal . Its preVariation in the distal root of MFM has been reported to be higher in younger age group (less than 31 years) which is in accordance with the present case report. Pattanshetti et al. concluded that the frequency of finding second canal in the distal root of MFM reduces as the age advances. He presumed that the tooth exposure to insults like caries, attrition, and erosion with age leads to calcification of the orifice or canal itself thus making it difficult to locate the canals , 16. ConEarly eruption of MFM makes it the most frequent tooth requiring endodontic or restorative procedures. Presence of iatrogenic and pathological problems and heavy occlusal forces lead to frequent pulp chamber calcification associated with it . SimilarMFM was reported to have a canal configuration 1-1 (62.7%), followed by 2-1 (14.5%) and 2-2 (12.4%). Furthermore 77.2% of the distal roots had one foramen while 22.2% had two foramina . 74.8% oThe third canal if present is frequently located centrally between the buccal and lingual roots with a smaller diameter compared to the other two canals . The maxVarious methods advocated for additional canal location include knowledge of law of symmetry and canal location, use of endodontic explorers like DG16 and microopener, Champagne test, fibre-optic illumination dental endoscopy and orascopy, use of magnification, visualisation endograph using Ruddle's solution, magnetic resonance microscopy . Use of Conventional radiography is of limited significance in cases with complex root anatomy as it produces images in only two dimensions, usually in the mesiodistal direction. They can aid in the identification of these aberrancies but are of not much significance for the exact location of the canals . An ex vThe present case reports four root canal orifices in the distal root of MFM with two apical terminations which could be classified as type XIV canal configuration according to Sert and Bayirli . This caExcellence in endodontics can be achieved by comprehensively understanding the key elements essential for successful treatment and by critically evaluating the etiology of failures. Endodontic treatment of multirooted teeth is always challenging to the clinicians due to the enormous variations associated with them. Inability to locate and access aberrant root canals results in failures. Clinician with good biological rationale, thorough knowledge of dental anatomy, careful interpretation, and utilisation of diagnostic tools integrated with skill and experience can deliver successful treatment to the patient. The use of US and CBCT in the above case illustrates that endodontic research and technology are continually evolving and to provide best results to our patients it is our duty as clinicians to be a part of this evolution and to apply and utilize these technological advancements to efficiently identify, disinfect, and obturate root canal.


Thus, a diagnosis of PNET of the prostate was established. Systemic chemotherapy was the selected treatment, however, a poor response was obtained. The patient succumbed approximately four months after the initial onset of symptoms. PNET of the prostate has been reported in eight cases worldwide; in comparison, the present case exhibited the most unsatisfactory outcome.The present study reports a rare case of primitive neuroectodermal tumor (PNET) of the prostate. A 23-year-old male presented to Oita Medical Center  with the complaint of dysuria and anal pain. A large mass in the prostate and a number of swollen lymph nodes in the pelvic region were identified by a computed tomography scan and magnetic resonance imaging. The patient was, thus, admitted to Oita University Hospital , where a biopsy of the prostate gland was performed. Histological analysis revealed small round cells that were positive for MIC-2 expression and fluorescent  Primitive neuroectodermal tumor (PNET) is included in the Ewing family of tumors (ESFTs) as they exhibit identical cytogenetic changes. The specific translocation t11;22)q24;q12) was identified in Ewing’s sarcoma and PNET, however, various other translocation patterns may also be involved  analysis using a Ewing sarcoma breakpoint region 1 (EWSR1) probe  the patient exhibited various side-effects, including dizziness, headache and nausea. MRI revealed multiple metastases to the intracranial meninges and a CT scan indicated a poor chemotherapy response. Therefore, best supportive care was administered and the patient succumbed approximately four months after the initial onset of symptoms.A 23-year-old male presented to Oita Medical Center  with the complaint of dysuria and anal pain. A computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed a large solid tumor in the pelvic region replacing the prostate  and multA multimodal therapeutic regimen that includes a combination of chemotherapy, surgery and radiotherapy is the current treatment strategy for the Ewing sarcoma family of tumors. Additionally, PNET of the prostate is commonly treated by combination therapy  9,12–18–1812–18.The present case lacked the specific Ewing’s sarcoma/PNET translocation t, however, EWSR1 gene rearrangement on chromosome 22 was detected by performing FISH analysis. Overall, ~15% of histopathologically defined MIC-2-positive Ewing sarcomas lack the classical specific translocation. However, in the majority of the remaining cases, variant translocations involve chromosome 22q12. Chromosome 21q22 (10% of Ewing sarcoma), and chromosomes 7q22, 17q12 and 2q36 (<1% of Ewing sarcoma) were reported as translocation partners  present to hospital with the complaint of dysuria, to improve the rate of early diagnosis. Non-invasive examinations, including ultrasound or digital rectal examination, are sufficient to identify the presence of this disease in the prostatic region.


We report a patient who had an inflammatory pseudotumor (IPT) that invaded to the maxillary sinus and orbital cavity, with the left pterygopalatine fossa as the principal site; this is a very rare case. The patient was an 83-year-old woman who suddenly became aware of impairment in the eyesight and visual field of the left eye. CT images showed a neoplastic lesion that invaded to the maxillary sinus and orbital cavity, with the left pterygopalatine fossa as the principal site, and also showed contrast effects. To obtain a definitive diagnosis from histopathological analysis, the lesion was biopsied, and she was diagnosed as the inflammatory pseudotumor with the immunohistochemical study and multiplex polymerase chain reaction-based clonality assays. The patient had a lymphoid-predominant lesion that responded to radiotherapy but corticosteroids were not effective. It is important to scrutinize the pathology to avoid unnecessary and mutilating surgery. An inflammatory pseudotumor (IPT) is a space-occupying lesion that causes neoplastic proliferation clinically but is a histopathologically benign disease involving nonspecific chronic inflammation .IPTs are commonly found in the lungs  and insiAn 83-year-old woman suddenly became aware of impairment in her eyesight and visual field of the left eye. She was admitted to the ophthalmology department in our hospital because she did not experience any improvement with observation. During an eyesight examination, abnormal vision was confirmed in her left eye.The patient had a history of lung cancer for which she had undergone surgery 10 years ago and arrhythmia for which she had a pacemaker implanted 5 years ago.Computed tomography (CT) images showed a neoplastic lesion with contrast enhancement and an indistinct boundary, which had invaded to the maxillary sinus and orbital cavity. The principal site of the lesion was the left pterygopalatine fossa. In addition, the images also showed bone destruction of the lateral wall of the maxillary sinus because of exclusion and invasion of the lesion at its outer side . MagnetiEyesight examination test showed that the best corrected visual acuity (BCVA) of the left eye was 0.2. The visual field test showed enlargement of the blind spot. The soluble interleukin-2 receptor (sIL-2R) level was a little high at 576 U/mL ; however, abnormalities pertaining to inflammation markers, tumor markers, and collagen disease markers were not noted during another blood test.We performed a biopsy of the lesion to obtain a definitive histopathological diagnosis. First, the uncinate process was removed, and then the maxillary ostium was opened. The tumor mass was removed from a part of the maxillary sinus and the pterygopalatine fossa.Hematoxylin-eosin (HE) staining of the biopsied specimen showed dense infiltration of small lymphocytes, which possessed equally sized round nuclei with a fine chromatin pattern. There was also an admixture of small numbers of plasma cells and eosinophils. The lymphocytic infiltration did not reveal nodularity or lymphoepithelial lesions of the sinonasal gland . Immunohγ) were conducted using genomic DNA extracted from the formalin-fixed paraffin-embedded tissue samples and BIOMED-2 primer sets [To examine the possibility of lymphoproliferative disorders, multiplex PCR-based clonality assays as to VH-JH region of immunoglobulin heavy chain (IgH) and T-cell receptor gamma (TCRmer sets . As showThe clinical course after biopsy included steroid pulse therapy according to the following protocol: 1 g of methylprednisolone sodium succinate for 3 days, followed by prednisolone, for which the dosage was gradually reduced from 40 mg to 10 mg and finally to 5 mg. However, no apparent vision recovery was observed during eyesight examination. Subsequently, we decided to administer radiotherapy. The patient received a total of 40 Gy radiotherapy, which was very effective, and her left eye vision recovered with a BCVA of 0.7.The CT images after steroid therapy showed no change in the lesion ; howeverThe patient is well and free of disease after the therapy for three years.IPT was initially reported as a nonneoplastic mass occurring in the lungs . HoweverThe nasal sinuses, particularly the maxillary sinus, can be the site of not only carcinomas, such as squamous-cell epithelial carcinoma, but also hematopoietic malignant tumors, such as malignant lymphoma and extramedullary plasmacytoma, and autoimmune diseases, such as Wegener's granuloma, making histopathological differential diagnosis problematic.Although pseudotumor with the pterygopalatine fossa as the principal site is rare, a few cases have been reported, and in all these cases, the IPTs were difficult to differentiate from malignant tumors , which iThe histology results of our study were unlike those of chronic conditions in which epithelioid and giant cells, such as sarcoidosis and tuberculosis, are present. Further, we did not observe necrotic granuloma or vasculitis, thereby, differed from Wegener's granuloma and periarteritis nodosa. It was evident that no tumor cells were present during histological analysis in our case, monoclonality could not be shown by immunostaining, and the proliferative potential of the tumor was not particularly high. Thus, considering the overall clinical findings, we diagnosed the patient as IPT.In this nonfocal case, CT image showed that the principal site was the left pterygopalatine fossa, causing displacement of the inferior lateral wall bone into the maxillary sinus of that side and its pervasive destruction; further, the tumor had advanced into the orbital floor. The fact that the only subjective symptom was visual impairment suggested that the tumor had invaded the ophthalmological region via soft tissue, which was in turn invaded via the inferior orbital fissure from the pterygopalatine fossa.Previous studies have shown the effectiveness of therapy, such as high-dose corticosteroids, surgical excision, or radiotherapy, for IPTs . A literHere, we describe the rare case of a patient having IPT with the left pterygopalatine fossa as the principal site that invaded the maxillary sinus and orbital cavity. Previous reports have shown an association between IPT, chromosomal abnormalities, and P80 and ALK1 expressions . Althoug


Carcinosarcoma is a rare type of renal pelvis malignancy, the diagnosis of which requires the presence of malignant epithelial and mesenchymal components. The prognosis of this disease is extremely poor due to its rapid progression and widespread metastases. The present study describes a case of carcinosarcoma involving the right renal pelvis in a 73-year-old female who presented with intermittent hematuria and right-flank pain that had persisted for one month. Computed tomography revealed a 2.4×2.5 cm mass in the right renal pelvis, which was diagnosed as a right renal pelvic tumor. Laparoscopic radical resection of the right kidney and ureter was performed. Following surgery, immunohistochemical analysis showed positive reactions for epithelial and mesenchymal markers. Based on these findings, the patient was diagnosed with carcinosarcoma. Thus, immunohistochemical analysis is a critical method for the accurate diagnosis of carcinosarcoma. Carcinosarcoma is a rare neoplasm that shows an admixture of epithelial and mesenchymal components ,2. The cA 73-year-old female was admitted to the Department of Urology, First Hospital of Jilin University  presenting with intermittent hematuria and right-flank pain for one month. The patient had a history of hysterectomy for uterine fibroids 10 years previously. The patient had no other voiding complaints or any significant urological history. The patient also denied past tobacco use or analgesic abuse, and the results of the physical examination were normal, except for mild percussion pain in the right kidney area. Urinalysis revealed increased numbers of red blood cells, leukocytes and suspicious malignant cells. Furthermore, protein was found to be excreted in the urine  and the analysis of the blood biochemistry revealed an elevated erythrocyte sedimentation rate (90 ml/h).Abdominal ultrasonography showed a solid, relatively well-demarcated tumor, 3.0×3.1 cm, occupying the right renal pelvis. Computed tomography (CT) showed a 2.4×2.5-cm heterogeneous and poorly-enhanced mass in the right renal pelvis . The CT Macroscopic examination of the 13×8×6-cm nephrectomy specimen revealed a 8×5×4-cm tumoral mass in the renal pelvis. The cut section of the mass was gray-white in color, with a hard consistency. Areas of extensive necrosis were also present. The tumor had invaded the full-thickness of the renal pelvic wall and peripelvic adipose tissue. Furthermore, invasion into the renal parenchyma was observed. Histological examination of the tumor showed a malignant neoplasm comprising of epithelial and mesenchymal components, which were largely separated from each other . Howeveret al (et al (Carcinosarcoma is a rare, malignant neoplasm that shows histological evidence of intimately mixed epithelial and mesenchymal elements . The hiset al  proposedl (et al  proposedCarcinosarcoma has been described in various organs, including the uterus, breast, stomach, lung, salivary glands, thyroid gland and gallbladder. However, carcinosarcoma of the urogenital organs has rarely been reported ,6,10–15.Carcinosarcoma of the renal pelvis has been shown to be aggressive and often has a poor prognosis –18, thusDue to the similar microscopic appearance of carcinosarcomas and sarcomatoid carcinomas, immunohistochemistry may be a useful diagnostic adjunct for differentiating between these tumors. In the present case, antigenic determinants that were specific for epithelial cells, such as cytokeratin, were identified. Furthermore, the sarcomatous component was characterized by strong staining for vimentin. In addition, the lack of expression of keratin markers in the mesenchymal component further confirmed the diagnosis of carcinosarcoma.et al (No clinical trials have been specifically designed for carcinosarcoma, thus an optimal treatment strategy has yet to be established. At present, radical resection is the only curative treatment. Nephrectomy, radiation therapy and chemotherapy have been used alone or in combination ,19. In tet al  reportedIn conclusion, carcinosarcoma of the renal pelvis is a rare, aggressive tumor, with a low survival rate. Although rare, carcinosarcoma should be included in the differential diagnosis. Further investigations into the natural history and prognostic factors of this disease and specific guidelines regarding therapeutic approaches for this tumor are urgently required.


Mesenteric panniculitis is a chronic inflammatory process involving the adipose tissue of the mesentery. The etiology is unknown, and it is rare in children. We report a 5 year old girl who presented with abdominal symptoms and was misdiagnosed as appendicular mass. The correct diagnosis was established after surgical resection. Mesenteric panniculitis is a rare disease characterized by chronic inflammation of the adipose tissue of the mesentery. The specific etiology is unknown, but surgery, trauma, infection, autoimmunity and malignancy have been suggested as possible factors.Most of the cases described in the literature are adults. The disease is exceptional in children probably because of less mesenteric fat. We report here a case of mesenteric panniculitis in a child who presented with abdominal pain following a minor trauma, and was misdiagnosed initially as appendicular mass.A five year old female child was referred for evaluation of an abdominal mass. She has had a history of minor abdominal trauma one month prior when she fell down while playing. Her abdominal ultrasound at that time showed minimal intraabdominal free fluid. She was observed for one day at another hospital and discharged home the following day without any symptoms. Ten days later she started to have severe right sided abdominal pain, associated with fever (up to 38.5°C) and poor oral intake, but no vomiting or diarrhea. She had leukocytosis , and abdominal ultrasound showed a non specific abdominal mass on the right side. A laparotomy was done for suspicion of an appendicular mass. The mass was not excised but a biopsy and culture were taken and a drain left in place. Her fever and abdominal pain persisted postoperatively with new onset of vomiting.On arrival to our hospital she was still in pain and febrile 38.5-39°C). She was irritable with right sided abdominal tenderness. Her labs showed leukocytosis with neutrophilia , ESR 38, kidney and liver function tests were within normal. Abdominal X-ray showed a right sided mass with calcifications  is a chronic fibrosing inflammation of the mesentery of the small intestines and colon  (Delgado Plasencia et al. The presentation of the disease varies considerably. It could be asymptomatic in 30-50% of cases (Ferrari et al. MP usually has a benign slow course. Nevertheless, significant morbidity and mortality have been related to MP including small bowel obstruction, perforation and pneumoperitoneum (Chawla et al. Because of this wide variety of non-specific presentations, MP should be in the differential diagnosis of chronic abdominal pain especially when more common causes have been excluded. Physical examination is also non-specific, abdominal tenderness and feeling of an ill defined mass may be the only findings. Signs of intestinal obstruction may also be present (Delgado Plasencia et al. MP is rare in children. Viswanathan et al. reported a 6 year old girl with idiopathic sclerosing mesenteritis . They alSimilar to adults, the most common presenting feature in children was abdominal pain. Some presented with features of acute abdomen and required surgery (Cakmak et al. The differential diagnosis of MP is broad. It could easily be missed as mesenteric neoplasm  or any oBlood tests are usually unremarkable. Non specific elevation of inflammatory markers is common (Issa & Baydoun CT scan has been proposed as the imaging modality of choice. Some suggested that changes are classical that the diagnosis can be made by CT alone . The appMP has been reported to regress spontaneously in most patients  and may Surgical resection is generally not recommended except in presence of complications like intestinal obstruction and ischemia (Ferrari et al. The prognosis is good with a benign course and favorable outcome in most cases (Ferrari et al. MP is a benign condition with no definitive causal relationships, though many conditions as surgery, trauma, malignancy, autoimmunity and infection have been proposed as predisposing factors. It has various presentations with a wide range of clinical and radiological manifestations, posing a diagnostic challenge. It is important especially for the surgeons to keep this entity in the differential diagnosis of chronic ill defined abdominal pain and/or mass. Very few cases have been reported in children. Our patient was initially misdiagnosed as appendicular mass. Indeed the accurate diagnosis was difficult before surgery given the rarity of the condition in children and the lack of awareness among surgeons and radiologists about this disease. MP could be more common than we think, but rather under-diagnosed. It is possible with the wide availability of CT scans and their use in evaluating chronic ill defined abdominal pain in all age groups that more and more cases will be reported in children.Written informed consent was obtained from the patient’s parents for the publication of this report and accompanying images.


Prepubertal gynecomastia due to testis tumors is a very rare condition. Nearly 5% of the patients with testicular mass present with gynecomastia. Sertoli cell tumors are sporadic in 60% of the reported cases, while the remaining is a component of multiple neoplasia syndromes such as Peutz-Jeghers syndrome and Carney complex. We present a 4-year-old boy with gynecomastia due to Sertoli cell tumor with no evidence of Peutz-Jeghers syndrome or Carney complex. Prepubertal gynecomastia is characterized by the presence of palpable unilateral or bilateral breast tissue in boys, without other signs of sexual maturation. Gynecomastia is common in normal males in the neonatal period, at early puberty, and with increasing age . PrepubeSertoli cell tumors (SCTs) account for 2% of prepubertal testicular tumors and very few have occurred in the first decade of life. The most common presenting symptom of a testicular tumor is painless scrotal mass. Gynecomastia can be seen in approximately 5% of patients with testicular mass. Most of the SCTs in prepubertal boys, which are generally bilateral and diffuse, are in the content of Peutz-Jeghers Syndrome (PJS) or Carney complex [A 4-year-old boy was referred to pediatric endocrinology because of bilateral breast enlargement. There was no history of a chronic disease, medication, or a familial disorder. Height was 114 cm (+1.2 SDS), weight was 20 kg (+0.7 SDS), and physical examination revealed bilateral gynecomastia. Breast development appeared as Tanner Stage-2 , axillarGynecomastia is common in boys at early puberty, while prepubertal gynecomastia is a rare condition. It is generally considered a pathological sign of a possible endocrinopathy, requiring a detailed history, physical examination, and laboratory work-up . Most ofThe records of Prepubertal Testicular Tumor Registry include forty-two patients with stromal tumors, of whom only 10 patients had SCT with an overall mean age of 52.5 months at presentation . They arThere is no specific differential immunoprofile for this tumor. Vimentin, inhibin, and calretinin seem more widely expressed and may help the diagnosis of SCTs . MalignaIn conclusion, our patient had unilateral testicular solid-cystic mass with no evidence of perioral lesions, intestinal symptoms, or family history for syndromes. Although unilateral testicular mass is very rarely reported, this patient should be followed up carefully in terms of other clinical findings of PJS as well as Carney Complex. This case was reported to remind of a very rare condition in the etiology of prepubertal gynecomastia and emphasize on the importance of careful physical examination and scrotal USG.


Retroperitoneal haematoma is a rare clinical entity with variable etiology. It can happen spontaneously, without any obvious precipitating factors, the so-called spontaneous retroperitoneal haematoma. There is no general consensus as to the best management plan for patients with retroperitoneal haematoma. Polyarteritis nodosa (PAN) is a rare cause of retroperitoneal haematoma. Here we report relationship between PAN and retroperitoneal haematoma and treatment approaches. However, an accepted and clearly defined treatment has not been established due to its rarity. PAN is a systemic, necrotizing vasculitis affecting medium-sized and small arteries. It is characterized by segmental necrosis leading to aneurysm formation. Kidney involvement in PAN is seen in 70–80% of the patients and it may rarely be complicated by aneurismal rupture leading to subcapsular and perirenal haematomas . PerirenA 24-year-old man was referred to emergency service because of nontraumatic excruciating abdominal pain. Assessment of patient's history revealed that there have been intermittent abdominal pain, fever, loss of appetite, 10 kg weight loss, weakness, myalgia, and arthralgia of small joints in the last few months. He had no history of bleeding diathesis, or anticoagulant therapy. On physical examination, the patient appeared uncomfortable. His temperature was 37.4°C, his pulse was regular at 80 beats per minute, and his blood pressure was 110/60 mmHg. He had left costovertebral angle tenderness. There were no rushes and skin lesions, the testes were nontender, and genital system looked normal. The results of laboratory examinations revealed leukocytosis of 23,9 , anemia with hemoglobin level of 9,6 , and elevated sedimentation rate of 70 (0–15). Renal function tests and other biochemical parameters were unremarkable. The urinalysis showed proteinuria (+++) and haematuria (+++). Hepatitis B surface antigen, antinuclear antibody, and antineutrophil cytoplasmic antibodies were negative. A contrast enhanced computed tomography of the abdomen showed multiple small wedge-shaped less-enhanced areas  with susSpontaneous retroperitoneal hemorrhage also known as Wunderlich syndrome (WS) is a rare but potentially life threatening entity characterized by acute onset of nontraumatic subcapsular and perirenal haematomas. Spontaneous perirenal bleeding secondary to PAN was first described by Schmidt in 1908. It is classically characterized by Lenk's triad: acute flank pain, abdominal tenderness, and signs of internal bleeding. A wide spectrum of neoplastic and nonneoplastic renal pathologies may result in WS. Zhang et al. reported on 135 such cases in their meta-analysis. Benign and malignant neoplasms accounted for the majority of cases (61%), with the most common being angiomyolipoma and renal cell carcinoma. Vascular causes were the next major cause with PAN accounting for 28 cases (17%) . With apSometimes urgent nephrectomy is life-saving . But sel


A 40-year-old mentally retarded Japanese man was admitted at rehabilitation facility for handicapped persons and found dead in his bed. His neonatal period was complicated by seizures, and he had a medical history of schizophrenia. A postmortem computed tomography scan suggested an intestinal obstruction, but the cause was unknown. To clarify the cause of death, a medicolegal autopsy was carried out. The gastrointestinal tract was found to contain copious amounts of cloth pieces. A diagnosis of intestinal obstruction secondary to pica of clothes was made. Despite still being an essentially neglect condition; mental retardation is cause to significant burden to the patient, his relatives and caregivers and the whole society. Moreover, people with mental retardation may be at increased risk for potentially self-injury due to ingestion of non-eating substance or incongruent intake of eating substances, which may on turn lead to severe or even life-threatening medical and surgical complications as herein reported. Specific attention also to pica in mentally-retarded patients with sudden, severe, gastrointestinal events, should therefore be placed in order to prevent potential death or otherwise severe chronic consequences, ideally aiming at enhancing the early recognition and multi-disciplinary management of those psychological stressors or triggers potentially responsible for pica too. Pica is the craving or ingestion of non-food items. The cravings found in patients diagnosed with pica may be associated with a nutritional deficiency state such asiron-deficiency anemia, with pregnancy, or with mental retardation or mental illness . Pica isWe present a case of bowel obstruction secondary to pica. The implication and need for an early multi-disciplinary approach are discussed in the present case-report. In addition, we reviewed previous reports of pica associated with mental retardation in adults. Pica should be suspected in mentally retarded patients with bowel obstruction of an unknown cause.A 40-year-old mentally retarded Japanese man was admitted at rehabilitation facility for handicapped persons and found dead in his bed. His neonatal period was complicated by seizures, and he had a medical history of schizophrenia. Intelligence quotient (IQ) level was 60. He spent in facility more than 20 years. Because the etiology of his death was unknown, a postmortem computed tomography (PMCT) scan was performed. The PMCT showed a massively dilated loop of bowel with stasis of fecal material in the small intestine and colon. Moreover, the trachea was filled with residue Fig. . The PMCPica is the compulsive consumption of nonfood and/or non-staple food, often resulting in iron deficiency anemia . It is iThe cause of pica is unknown, but a multi-factorial etiology has been suggested. Causes include iron deficiency, psychological factors such as poverty, maternal neglect and abuse, lack of parental supervision, or a disorganized family situation, mental retardation, autism, and brain behavior disorders . The varPica can result in intestinal obstruction. Anderson et al. reported a case of intestinal obstruction caused by talcum powder pica. Their literature review identified 43 previously reported cases of surgical complications caused by various forms of pica. Intestinal obstruction was the most common clinical presentation and the ileum was the site most commonly reported at surgery. Perforation with peritonitis was the next most common presentation. Clues pointing to pica as the underlying cause of abdominal complaints should not be overlooked, especially in patients who are known to be at a higher risk for pica .Though potentially leading to severe medical complications or even life-threatening outcomes, relatively little evidence has been made available on the matter to date, especially among people with mental retardation, essentially due to stigma issues and lack of inclusion of people with mental disabilities in most controlled psychiatric and general medical trials. Therefore, the aim of the present case report is to provide a synthetic report of one of such cases in order to shed further light on the matter.The prevalence of pica is difficult to establish because of differences in definition and the reluctance of patients to admit to abnormal cravings and ingestion. Various prevalence estimates have been reported in the literature. We reviewed the prevalence of pica associated with mental retardation in adults Table , which sThere are some cases of pica leading to death via intestinal obstruction. However, PMCT imaging findings of pica have not been reported. In recent years, computed tomography (CT) has proven to be an excellent diagnostic tool for the assessment of intestinal obstruction. It can accurately reveal the site and cause of obstruction, and, most importantly, distinguish between an extrinsic and an intraluminal cause. However, the diagnosis of pica is often difficult, even more so if the pica material is of low radiopacity. In our case, the obstructing material was cloth, and it was not recognized on CT. To confirm reproducibility, a piece of cloth located in the intestinal tract was scanned by using CT. However, this was not similar to the imaging findings of PMCT.Pica is often observed in mental retardation. Pica should be suspected in mentally retarded patients with bowel obstruction of an unknown cause. If pica is suspected in the mentally retarded or in institutionalized patients, autopsy examinations should carefully check for the potential side effects of foreign material ingestion, which can range from upper airway obstruction to intestinal obstruction.Despite still being an essentially neglect condition; mental retardation is cause to significant burden to the patient, his relatives and caregivers and the whole society. Moreover, people with mental retardation may be at increased risk for potentially self-injury due to ingestion of non-eating substance or incongruent intake of eating substances, which may on turn lead to severe or even life-threatening medical and surgical complications as herein reported. Specific attention also to pica in mentally-retarded patients with sudden, severe, gastrointestinal (G.I.) events, should therefore be placed in order to prevent potential death or otherwise severe chronic consequences, ideally aiming at enhancing the early recognition and multi-disciplinary management of those psychological stressors or triggers potentially responsible for pica too.Written informed consent was obtained from the patient’s legal guardians for publication of this case report and any accompanying images. A case report is not required institutional review board (IRB) in our institution.


Pediatric pituitary adenomas are rare, accounting for <3 % of all childhood intracranial tumors, the majority of which are prolactinomas. Consequently, they are often misdiagnosed as other suprasellar masses such as craniopharyngiomas in this age group. Whilst guidelines exist for the treatment of adult prolactinomas, the management of childhood presentations of these benign tumors is less clear, particularly when dopamine agonist therapy fails. Given their rarity, childhood-onset pituitary adenomas are more likely to be associated with a variety of genetic syndromes, the commonest being multiple endocrine neoplasia type 1 (MEN-1).MEN1 gene, potentially explaining the difficulties in management of this tumor. Genetic counseling and screening has now been offered to the wider family.We present a case of an early-onset, treatment-resistant giant prolactinoma occurring in an 11-year-old peripubertal boy that was initially sensitive, but subsequently highly resistant to dopamine agonist therapy, ultimately requiring multiple surgical debulking procedures and proton beam irradiation. Our patient is now left with long-term tumor- and treatment-related neuroendocrine morbidities including blindness and panhypopituitarism. Only after multiple consultations and clinical data gained from 20-year-old medical records was a complex, intergenerationally consanguineous family history revealed, compatible with MEN-1, with a splice site mutation (c.784-9G > A) being eventually identified in intron 4 of the MEN1 and AIP mutations in all cases of pediatric pituitary adenoma.This case emphasizes the need to consider pituitary adenomas in the differential diagnosis of all pediatric suprasellar tumors by careful endocrine assessment and measurement of at least a serum prolactin concentration. It also highlights the lack of evidence for the optimal management of pediatric drug-resistant prolactinomas. Finally, the case we describe demonstrates the importance of a detailed family history and the role of genetic testing for  Pituitary adenomas account for <3 % of all childhood intracranial tumors with an estimated incidence of 0.1 cases/million/year . ProlactWe report a patient presenting to our quaternary pediatric neuroendocrine and oncology units with a treatment-resistant giant prolactinoma. His complex genetic history and challenging management illustrates the necessity for thorough history-taking, specialist multidisciplinary management and the potential for long-term neuroendocrine morbidity in this rare tumor.An 11-year-old Middle Eastern boy was referred to Great Ormond Street Hospital for Children with four years of headaches and 18 months of visual deterioration, previously investigated with a reportedly normal brain CT scan in his country of origin. There was no galactorrhea. At presentation he had a right-sided relative afferent pupillary defect, optic atrophy and could only perceive light with his right eye and count fingers with his left eye. Auxology was normal and he was in early puberty with testicular volumes of 4 mls bilaterally. Initial family history revealed an inter-generational multiply consanguineous family Fig. , with on4) 10.3 pmol/l, normal range 10.8-19.0) but normal hypothalamo-pituitary-adrenal (peak cortisol 516 nmol/l to synacthen stimulation) and -gonadal function .Magnetic resonance imaging revealed a large, avidly-enhancing heterogeneous sellar and suprasellar mass merging with the pituitary gland, compressing the optic chiasm and encasing both internal carotid arteries with a maximal transverse diameter of 5.5 cm Fig. . A proviOur patient was commenced on 250 μg/week cabergoline and levothyroxine supplementation. Despite an initial excellent PRL reduction in response to therapy, his tumor became extremely drug-resistant despite cabergoline dose escalation to 3.5 mg/week Fig. . MultiplMEN1 and AIP mutations revealed a heterozygous MEN1 intronic splice site mutation (c.784-9G > A). This has previously been shown to result in alternative splicing, a premature stop codon and a truncated, inactive MENIN protein [His complex family history alerted clinicians to the likelihood of an underlying genetic diagnosis but despite repeated consultations with multiple specialists  no common ancestor linking all three macroprolactinoma cases could be found. Genetic testing of our patient for both  protein –13.Subsequent questioning revealed a history of renal stones and primary hyperparathyroidism in our patient’s mother and two maternal aunts, as well as a history consistent with MEN-1  in his deceased maternal grandmother Fig.  who had The rarity of pediatric pituitary adenomas means that they are often not considered in the differential diagnosis of suprasellar tumors in this age group, leading to the potential misdiagnoses of craniopharyngioma or low-grade glioma, for which primary treatments are predominantly surgical, chemotherapeutic and/ or radiotherapeutic in nature. This case highlights the importance of a routine endocrine evaluation in all such cases, not only to document potential tumor-related pituitary dysfunction but also to identify functioning pituitary adenomas by measuring serum PRL, GH and adrenocorticotropic hormone, particularly as non-functioning pituitary adenomas are rare in children , 15, 5. Medical monotherapy with dopamine agonists is recommended as first-line treatment for all prolactinomas due to their excellent efficacy, likely preservation of residual pituitary function and low side-effect profile , 22. Cab2 receptor downregulation [The mechanism for development of drug-resistance is not well understood, and is thought to relate to dopamine Dgulation . MEN-1 igulation , 9. Secogulation , 30, 31.gulation , 6, 3.AIP mutations are a subset), multiple endocrine neoplasia type 4 (CDKN1B), Carney complex (PRKAR1A), McCune-Albright syndrome (GNAS), SDH-related pituitary adenoma syndrome , neurofibromatosis type 1 (NF1) and DICER1 syndrome [9-22 % of pediatric pituitary adenomas are associated with genetic tumor-predisposing syndromes, the most well-known of which is MEN-1 with an estimated prevalence of 0.02-0.2 per 1000 . Whilst syndrome –37.MEN1 and AIP germline mutations in all pediatric patients with PRL- and GH-secreting pituitary adenomas as they may be index cases for a given family [et al. (2012). This is particularly important given that current guidelines recommend testing first-degree relatives of index cases and performing annual biochemical screening in carriers for insulinomas, PRL- and GH-secreting pituitary adenomas from age 5 years and for hyperparathyroidism from age 8 years [Several authors strongly suggest testing for both n family , 33, 29.MEN1 and AIP mutation testing, with subsequent screening of first-degree relatives if found positive.Pituitary adenomas must be considered in the differential diagnosis of all pediatric suprasellar masses, therefore mandating a detailed endocrine review and an accurate serum PRL measurement as management differs markedly from that of other tumors. Optimal management of dopamine agonist-resistant tumors in childhood remains unclear. A detailed family history of brain tumors and MEN-1-associated symptoms such as renal stones must be obtained and may require several consultations before being fully elucidated. We recommend that all children with PRL- or GH-secreting pituitary adenomas undergo Written informed consent was obtained from the parent of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Retiform hemangioendothelioma is a rare intermediate or borderline neoplasm of the blood vessels that mostly occurs in extremities. Here we report a unique case of retiform hemangioendothelioma presented in the external auditory canal. 58-year-old male patient presented with the complaint of right ear swelling for 4 years. On examination, a spherical swelling in the right ear was found occluding the view of external auditory canal. The tumor was removed surgically. Intraoperatively, the mass was found attached to the outer part of the right external auditory canal near the root of helix. Histopathology of the resected tumor showed typical features of retiform hemangioendothelioma. In addition, immunohistochemical analysis revealed that tumor was positive for endothelial cell marker CD34 and occasionally positive for cell proliferative marker Ki-67. Retiform hemangioendothelioma (RH) is a rare distinctive variant of low-grade cutaneous angiosarcoma typically present on the extremities –4. Till A 58-year-old male patient visited our outpatient department with the complaint of swelling in the right ear for the past 4 years. He reported that he did not feel any pain or difficulty in hearing. There was no relevant family history. He did not undergo any kind of treatment till his day of visit. On preliminary examination, we have found a large spherical shaped swelling with the size of 4 × 4 cm in the right ear obscuring the view of external auditory canal . The sweHistopathological examination of the resected tumor showed epidermis with a well-demarcated neoplasm in the dermis composed of numerous elongated, tortuous, intercommunicating blood vessels; a few appeared ectatic and some with papillary infoldings . Many arRetiform hemangioendothelioma is a rare intermediate or borderline malignancy of the blood vessels , 11, 13.The retiform hemangioendothelioma can be differentiated from the closely related diseases like Dabska tumor, well-differentiated angiosarcoma, and hobnail hemangioma by its histological appearance and morphological features. Dabska tumor shows blood vessels with numerous papillary tufts, with a central hyaline core lined by hobnail-like endothelial cells protruding into the lumina , whereasThe treatment of choice for RH would be surgical excision with histopathologically proven tumor-free margins. Half of the RH tumors have local or regional nodal recurrence and these tumors can be successfully treated with radiation therapy. We have treated our patient surgically, and the patient was under regular follow up for the past 16 months. During this period our patient does not show any recurrence from the site of surgery or any new swelling or mass from other parts of the body.RH tumors are rare and of low-grade angiosarcoma of the skin, so far less than 35 cases were reported worldwide. The case reported here shows typical features of retiform hemangioendothelioma. RH tumors usually occur in extremities and rarely in other sites. However, according to our knowledge, the case we have reported here is the unique one having a tumor in right external auditory canal. Thus, the presentation of rare cases like this will expand the current knowledge about this rare type of tumor and its sites of involvement which will be of immense help for future references.


Peripheral odontogenic lesions are considered to be rare within the classification of odontogenic tumors. They share the same microscopic characteristics of their central counterparts. Here, we report an ulcerated mass of the maxillary gingiva that on histopathological examination was diagnosed as peripheral developing odontoma or peripheral ameloblastic fibroodontoma. The diagnosis of this tumor is challenging and may lead to unnecessary treatment. An 8-year-old healthy girl was referred to the pathology department for examination of an isolated and ulcerated soft tissue mass of the palatal gingiva in the region of right canine and deciduous first molar, measuring 0.8 × 0.8 cm with unknown duration. There was no history of trauma to the area. The periapical radiograph did not show any intrabony lesion. The mass was excised under local anesthesia with clinical diagnosis of reactive soft tissue lesion most probably pyogenic granuloma (PG) or peripheral ossifying fibroma (POF). The cut surface of the lesion was creamy solid . On histOdontogenic neoplasms are categorized into peripheral and central. The relative frequency of peripheral odontogenic tumors is rare and there is no valid information of their frequency in the literature . SaghravOdontoma and ameloblastic fibroodontoma belong to mixed odontogenic tumors group and are composed of odontogenic epithelium and ectomesenchyme . Some auThe etiology of odontogenic tumors is indefinite and their source is the remnants of dental lamina that reside in the gingiva (rests of Serres) and develop lingually to those of the primary teeth . TherefoThe clinical presentation of this case was similar to PG and POF because of an ulcerated surface and the gingival involvement. In the case described by Kintarak et al. , focal fThe presence of dental tissue outside the alveolar process may be associated with ectopic neural crest cells that are still able to differentiate into tooth germ . It is dThe treatment choice is conservative surgical excision . RecurrePeripheral odontoma and peripheral ameloblastic fibroodontoma are exceedingly rare benign odontogenic lesions that are treated by conservative excision. The proper diagnosis of these lesions is necessary to avoid confusion with true neoplasms especially odontogenic tumors and prevent extensive surgery. Therefore, it is essential for oral pathologists to be familiar with clinical and microscopic characteristics of peripheral odontoma.