Medulloblastoma is one of the most common pediatric brain malignancies. The usual presenting clinical features are related to posterior fossa syndrome or/and hydrocephalus. Cauda equina syndrome is a very rare presentation for this disease.We describe the case of a three-year-old boy with cauda equina syndrome as the initial presenting clinical feature for medulloblastoma. He was initially diagnosed as having a spinal tumor by magnetic resonance imaging scan. Subsequently, a cranial magnetic resonance imaging scan revealed a posterior fossa tumor with features of dissemination. He had substantial improvement after treatment. This case report is complemented by a literature review related to this unusual presentation.Medulloblastoma primarily presenting with cauda equina syndrome is very rare. However, spinal drop metastasis should be considered in the pediatric age group to avoid suboptimal management. Medulloblastoma is the second most common posterior fossa tumor in children. It accounts for 40% of posterior fossa tumors in the pediatric age group . The cliOur patient was a three-year-old Arabic boy who presented to a peripheral medical center with a two-month history of lower back pain followed by progressive paraparesis. He had no symptomatology related to posterior fossa syndrome or increased intracranial pressure. Six weeks after the start of his symptoms, he developed urinary and bowel incontinence. During the physical examination, the boy was fully awake and cooperative. All cranial nerves were intact. His pupils were 2 mm, bilaterally equal and normally reacting to light. He had full extra-ocular movement with no nystagmus. There was no papilloedema. An examination of the upper extremities was normal. He had symmetrical lower limb weakness with muscle group power grade 1/5 distally and 3/5 proximally associated with hypotonia, absent reflexes and diminished sensations up to the L1 dermatome. His anal tone was reduced. Cauda equina syndrome was suspected, for which he underwent a magnetic resonance imaging (MRI) scan of the spine that revealed spinal canal filling and lesions in the thoracic and lumbar regions. At that stage, he was referred to our institution as a patient with a spinal tumor.Upon reviewing his spinal MRI, drop metastasis was suspected. A cranio-spinal MRI scan was performed, which showed a posterior fossa tumor associated with radiographic features of intracranial and spinal metastasis, Figures A. This MDuring his treatment course he received rehabilitation therapy. Three months after his initial treatment, he had substantial improvement in his overall neurological functions. He started to walk without assistance and was able to control his bladder and bowel functions. A neuroaxis follow-up MRI after one year showed regression of his cranial and spinal disease Figures B. At theet al., reported the case of a 28-year-old woman with thoracic extra-medullary medulloblastoma metastasis causing her to have progressive weakness as the initial presenting symptoms (Table The usual presenting symptoms of medulloblastoma are headache, vomiting and ataxia ,4. Althoms Table  7]. Mor. Moret aet al. in 1986 [However, spinal intramedullary metastasis of medulloblastoma rarely occurs. To date, there have been a limited number of cases with spinal intramedullary metastasis reported in the literature -14. Zump in 1986 . Recentl in 1986 . Our patCauda equina syndrome as the presenting clinical feature for medulloblastoma is rare. Similar clinical presentations in the pediatric age group should raise the suspicion of cranial neoplastic pathology. Cranial neuroimaging is important to rule out drop metastasis and to optimize patient management.Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.FA and AU analyzed and interpreted the patient clinical and radiological data and prepared the manuscript. HA performed the histopathological examination, prepared the pathology slides, and reviewed the manuscript. AA reviewed the manuscript. EA reviewed the manuscript and gave final input prior to publication. FA wrote the manuscript and prepared the radiology figures. All authors read and approved the final manuscript.


Duplex collecting system is a congenital genitourinary anomaly commonly found incidentally. Our experience with a duplex system associated with giant hydroureter presenting as mobile abdominal swelling that was noticed from birth, constipation, and failure to thrive is described. Ultrasound and IVU did not assist in making the diagnosis, while a barium enema suggested a colonic duplication. Congenital giant hydroureter should be considered as a differential diagnosis in infants with cystic abdominal swelling. A preserved renal moiety attributed to a dilated ureteric cistern was a unique theory in this case. A duplex collecting system can be defined as renal units containing two pelvicaliceal systems associated with single (incomplete) or double (complete) ureters. Autopsy studies indicated an incidence of 0.5–1.25% . Left anCommon complications of duplex system include vesicoureteric reflux, ureterocele, pelviureteric junction obstruction, and recurrent urinary tract infection. Few cases of hydroureter associated with duplex system have been reported. However, much more uncommon is congenital giant hydroureter with a preserved upper moiety. We report a case of giant hydroureter in a duplex systemwhich was noticed immediately after birth as abdominal swelling. There was associated failure to thrive and constipation.A 4-month-old male presented with abdominal swelling that was noticed at birth. There was failure to gain weight since birth. The swelling involved the whole abdomen but was more on the left and was not progressively increasing in size . There wHaemogram, urinalysis, and urine MCS were suggestive of urinary tract infection. Lentiviral screen was negative. Abdominal ultrasound showed huge cystic mass extending from the epigastric region and displacing the bowel loops to the right. An impression of a mesenteric cyst to rule out a gastrointestinal duplication cyst was made. Barium enema showed smooth walled rectum and a soft tissue shadow displacing the sigmoid and descending colon upwards and to the right . IntraveThe child was treated with intravenous antibiotics and nutritionally rehabilitated with dextrose and amino acids preparations. At exploratory laparotomy, a complete duplex system was found on the left side. The left kidney was moderately dilated with normal lower pole ureter. There was a huge hydroureter at the mid-third of the upper pole ureter extending from the left lumbar region to the suprapubic and the contralateral right iliac regions displacing the urinary bladder to the ipsilateral iliac fossa . It measHydroureter associated with duplex system is not common. Even more uncommon is its occurrence in the neonatal age group causing grossly distended abdomen as seen in this patient. Only very few cases have been reported as in the cases of giant ureter in a duplex system that presented as an abdominal mass in neonate , 8. Our Differential diagnoses like mesenteric cyst and enteric duplication cyst were entertained in this infant because of the constipation and radiologic impression. IVU was not helpful because the contrast got diluted in the dilated hydroureteric segment (cistern), hence, the improper outlining of the urinary system. We also think that this cistern also serves as a vent which preserved the upper renal moiety as the majority of the cases of duplex moiety have dysplastic renal poles which necessitates partial or even complete nephrectomy , 11. In Hydroureter associated with duplex system though less common should be entertained as a possible diagnosis in the assessment of infants presenting with recurrent urinary tract infection and soft intra-abdominal mass, especially, where there are limitations to extensive radiological investigations. As seen in this patient, it could be so huge to cause constipation, bladder outlet obstruction, sepsis, and nutritional failure.Duplex collecting systems are usually asymptomatic and in rare instances may present with hydroureter. This patient is lucky to have a preserved upper pole moiety from the cistern, which we suspect vented the ureter and prevented back pressure effect on the kidney.


Dens invaginatus (DI) is a malformation of teeth probably resulting from an infolding of the dental papilla during tooth development. DI is classified as type I, II, and III by Oehlers depending on the severity of malformation. The maxillary lateral incisor is the most commonly affected tooth. Structural defects do exist in the depth of the invagination pits, and as a consequence, the early development of caries and the subsequent necrosis of the dental pulp, as well as abscess and cyst formation are clinical implications associated with DI. Occasionally, we can see more than one developmental anomaly occurring in a single tooth. In such cases it becomes important to identify the anomalies and initiate a proper treatment plan for good prognosis. In this paper, an unusual case of DI which clinically presented as a huge talons cusp affecting a mandibular lateral incisor tooth is described. This case report illustrates grinding of the talons cusp followed by nonsurgical endodontic management of dens invaginatus type II with an immature apex and periapical lesions, in which Mineral Trioxide Aggregate (MTA) shows a complete periapical healing with bone formation at the site of the lesions. Dens invaginatus (DI), commonly known as dens in dente, is a developmental anomaly resulting from invagination in the surface of a tooth crown before calcification has occurred. Coronal invaginations usually originate from an anomalous infolding of the enamel organ into the dental papilla. The most extreme form of this anomaly is referred to as “dilated odontome.” This kind of malformation was first described by “Ploquet” in 1794 in whale's tooth . Hallet The frequency of its occurrence is 0.04 to 10% of all dental malformations .Synonyms for this malformation are dens in dente, invaginated odontome, dilated gestant odontome, dilated composite odontome, deep foramen caecum, tooth inclusion, dentoid in dente, gestant odontome, and dents telescopes—invagination is the common denominator of these lesions , 7.Another such anomaly is talons cusp. Talons cusp is considered a type of dens evaginatus. W.H. Mitchell was the first to describe it. Mellor and Rippa named it as an accessory cusp. The origin of the name is said to be so because this accessory cusp resembles the talon (tail) of an eagle when seen from an occusal aspect .There are case reports of co-occurrence of multidevelopmental anomalies in a single tooth. A few might give rise to clinical symptoms in early life, with a few manifesting later. The identification and treating the co-existing anomaly is important in the treatment plan for a better prognosis. Here we are reporting a case of Oehlers type II DI which clinically presented as a talons cusp, whose occurrence in the mandibular arch is a rare entity. Prophylactic grinding with root canal therapy using MTA apexification was done.A young 14-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry, with a chief complaint of pus discharge from the mandibular left anterior tooth region i.r.t. 31 and 32 for a week. He reported that he sustained an injury while brushing his teeth with a neem stick 4-5 months back. No relevant history of pain was reported. Thorough oral examination revealed a sinus in relation to tooth number 31 and 32 topographically. Tooth 32 had a clinical presentation of Talon's cusp with deep foramen caecum (not interfering with occlusion) [Figures A diagnostic periapical radiograph revealed that 32 presented with dens in dente type II with immature apex and periapical radiolucency of approximately 15 mm × 20 mm extending distal to the root tip of tooth number 31 . AnotherWith emergency treatment the abscess was drained through the vestibular sulcus. Antibiotics and analgesics were prescribed. Six days later the patient was recalled again and Talons cusp in relation to 32 was grinded to expose the pulp. A large central canal was found but there was no communication between the mesial and distal rudimentary canals in the DI. This was confirmed by the radiographic dye. Then complete elimination of invagination was done with the aid of a K-Flex #80 file . In the After establishing optimal working length in the radiograph of 31 and 32, the canal was disinfected with 5.25% sodium hypochlorite . Then caAn apical barrier of 4 mm in thickness was created with Pro Root MTA  in 32 and it was left with a cotton pellet moistened with distilled water for 48 hours . At the The patient was recalled for regular clinical visits at six monthly intervals and there was a complete absence of clinical symptoms.type I—an enamel invagination within the crown and not extending beyond the CEJ,type II—the enamel invagination into the root below the CEJ ending as a blind sac,type III—the extension of enamel-lined invagination through the root to form an additional apical or lateral foramen; usually no direct communication with the pulp.An early diagnosis of dens in dente is crucial and requires a thorough clinical examination of all teeth. Radiographically, this anomaly demonstrates a radio opaque invagination, equal in density to enamel and extending from the cingulum into the root canal. In order to characterize the degree of extent in the crown, root, and up to apex, Oheler classified DI as follows: type I—anThe presumed etiology of this phenomena has been related to focal growth retardation, rapid and aggressive proliferation of a part of the inner enamel epithelium, growth pressure of the dental arch, localized external pressure in certain areas of the tooth bud, distortion of the enamel organ, fusion of the two tooth germs, infection, and trauma .The clinical appearance of dens invaginatus varies considerably. The crown of the affected tooth can be of normal morphology but also can be associated with unusual forms like greater labiolingual diameter, peg shaped, barrel shaped, conical shaped, or talons cusp , 11. As Depending on the degree of malformation of DI and presence of clinical symptoms, there are different treatment modalities to prevent possible complications. Even without symptoms, dental treatment is considered necessary because access of irritants to the invaginatus may result in immediate or eventual contact with pulp. Strict observation is recommended if dental treatment is not done. The invaginatus is usually removed during treatment as was also done in this case.Various techniques of treating DI have been reported including conservative restorative treatment, teeth with deep palatal or incisal invagination or foramen caecum treated with fissure sealants before carious destruction can occur, nonsurgical root canal treatment, endodontic surgery, and intentional replantation and extractions . NonsurgA complete disinfection of the canal is of great importance to promote healing of affected periradicular tissues. In this case, sodium hypochlorite as irrigation and calcium hydroxide and metapex as intracanal medication between appointments were used to obtain this result.One of the major problems in endodontic therapy in teeth with pulp necrosis and open apex is obtaining an adequate closure of the root canal. The clinical procedure required for these teeth is based on the principle of pulp space disinfection allowing the formation of a mineralized tissue barrier at the apex of the tooth. Calcium hydroxide is the most commonly used medication for this purpose.MTA has been proposed as a material for immediate closure of the apical opening without waiting for a natural healing process. This will create an apical barrier in the canal preventing the extrusion of root filling material into the periapical tissues . It has The dens invaginatus type II reported here had a diagnosis of chronic periapical abscess with immature apex. In our case, the root canal was filled apically with an approximate thickness of 4 mm of MTA. At a later appointment the rest of the root canal was coated with glass ionomer as a liner and obturated with gutta percha. This was an attempt to reinforce the root, as there is a high incidence of root fracture after endodontic treatment in these types of teeth with large pulp space .Sathorn and Parashos reported a case of type II DI of maxillary canine in which the dense and infected necrotic pulp were removed and apexification with MTA was done . KristofOur case is unique by itself because the patient was unaware of the presence of the tooth malformation related lesion. DI presented as a talons cusp on the mandibular lateral incisor which is a rare anomaly. Radiographic evidence presented that the tooth was involved in periradicular infection, and hence root canal therapy was advised but at the same time the preservation of resistance of tooth structure was also kept in mind.This anomaly is clinically important because potential complications lead to pulp necrosis and periapical infection. A severe form often causes periapical cyst formation at an early age. As this is a rare phenomenon, it is necessary for clinical and radiographic assessments to identify the defects. Appropriate care has to be taken for the intervention so as to be sure of a favourable prognosis.


Extraskeletal myxoid chondrosarcoma (EMC) is a relatively rare but well-defined neoplasm. This report describes two patients, one with EMC of the buttock and one with EMC of the knee. The two cases presented with large lobed masses and long T1 and T2 signaling identified by magnetic resonance imaging (MRI). An enhanced MRI scan demonstrated enhancement of the tumors. The tumors were composed of strands or cords of oval and spindle cells embedded in abundant myxoid stroma. Pathology results confirmed EMC. Extraskeletal chondrosarcomas were first described by Stout and Verner in 1953 . EMC is This report presents two patients, one with EMC of the buttock and the other with EMC of the knee. Both patients provided written informed consent.A 47-year-old man presented with a five-year history of a painless lump in his left buttock. Six months prior to presentation, the patient had noted that the mass had gradually enlarged and become hard. Magnetic resonance imaging (MRI) revealed an 8×6×6-cm sized lobular mass in the left buttock that had long T1 and T2 signal intensities. The MRI scan also revealed a uniform low signal in the T1 weighted image (WI)  and a hiA 45-year-old woman presented with a painless lump in her right knee for one week. Physical examination demonstrated that the right lower extremities and knee were slightly swollen with a palpable mass, but the patient had no difficulty in mobilization. Computed tomography (CT) revealed an irregular-shaped soft tissue mass measuring 8.0×6.6×3.3 cm in size located in the right knee bursa, and the mass showed clear boundary and uniform density . The adjEMC is a relatively rare neoplasm with no specific findings in the clinic. Patients commonly present with non-specific symptoms, including tenderness and the detection of a palpable mass . The mosThis study describes two patients, one with EMC of the buttock and one with EMC of the knee. EMC of the buttock has rarely been reported; since its first description in 1972 (The differential diagnosis of EMC is broad and includes mucus liposarcomas and soft tissue myxomas. Mucus liposarcomas often present as a large bump situated in the muscles, with a clear boundary, multilocular high signaling in T2WI and without radial low signal separation. Soft tissue myxomas belong to the embryonic mesenchymal benign tumors. The appearance in MRI of an intramuscular lesion with low T1 signal and high signal intensity on fluid-sensitive sequences demonstrating a peripheral rim of fat and edema is highly suggestive of a soft tissue myxoma . The tum


The calcifying epithelial odontogenic tumor (CEOT) is a rare benign epithelial odontogenic neoplasm of slow growth that is locally aggressive and tends to invade bone and adjacent soft tissue. Here is reported the case of a 21-year-old female patient with a CEOT in the left mandibular posterior region. The computerized tomography in coronal plane revealed a hypodense lesion in the posterior region of the left mandibular body with hyperdense areas inside and was associated with element 37. An incisional biopsy of the lesion was performed and the histopathological analysis revealed the presence of layers of epithelial odontogenic cells that formed prominent intercellular bridges. A large quantity of extracellular, eosinophilic, and amyloid-like material and an occasional formation of concentric calcifications (Liesegang rings) were also found. The histopathological diagnosis was a Pindborg tumor. Resection of the tumor with a safety margin was performed and after 6 months of follow-up there has been no sign of recurrence of the lesion. The calcifying epithelial odontogenic tumor (CEOT) or Pindborg tumor is a rare benign epithelial odontogenic neoplasm , 2, withA 21-year-old black female patient sought treatment at the Oral and Maxillofacial Trauma Surgery Unit, with swelling in the mandibular left posterior region. While performing the intraoral physical exam an asymptomatic hardened exophytic nodule was found in region 37 .The patient reported a clinical evolution of only five months of the lesion. A radiographic exam revealed a diffuse radiolucent, unilocular lesion, associated with region 37, yet including the distal root of region 36 up to the crown of region 38 . Using aAn incisional biopsy ensued and the specimen was sent to the Laboratory of Oral Pathology. The histopathological analysis revealed layers of odontogenic epithelial cells that formed prominent intercellular bridges. Areas of extracellular, eosinophilic, and amyloid-like material with an occasional formation of concentric calcifications (Liesegang rings) were also present . Based oSurgical resection of the tumor was performed, including a marginal portion of apparently healthy bone, while attaching a 2.4 mm reconstruction plate of titanium, in search of the patient's rehabilitation. After 6 months of follow-up there has been no sign of recurrence of the lesion.CEOT is an uncommon lesion which generally affects the posterior maxillary region. A frequent radiographic finding in these tumors has been the presence of calcifying structures of varying sizes inside the lesions. Besides this, the cases reported in the literature have mostly been painless, of a slow evolution, and in an intraosseous area –9. SuppoThe histological criteria of the Pindborg tumor are layers of polyhedral epithelial cells with well-defined borders that oftentimes show prominent intercellular bridges. Figures of mitosis are rarely seen. In the layer of epithelial cells, circles, full of a homogenous amyloid-like substance, were observed. Some of those cells were also filled with a calcifying matter in the form of Liesegang rings, which are pathognomonic of this tumor –5. The hGenerally its treatment is undergone beginning with the surgical excision of the tumor. As the lesion was not encapsulated, the majority of authors agree that resection should include a safety margin of bone that is considered clinically and radiographically healthy , 10. The


Chondroid syringoma (CS) of the orbit is an extremely rare benign neoplasm. To the best of our knowledege, this is the second case reported in the english litérature.We report a case of a 41-year-old woman with orbital CS. This tumor developed slowly over 8 years causing indolor, no axil, exophtalmos of the left eye. Computed tomography demonstrated an isodense intraorbital tumor with homogeneous enhancement without bony erosion. On Magnetic resonance imaging the tumor was isointense on T1-weighted imaging, slightly hyper intense on T2-weighted imaging, and enhanced after Gadolinium administration. The patient was operated via left lateral orbitotomy. At surgery the mass was well circumscribed, extraconal, very firm and did not invade or adhere to other structures. The tumor was removed in toto. The diagnosis was confirmed by histopathological examination, the lesion was nodular, and there was differentiation toward the adnexal ductal epithelium with chondromyxoid and adipocytic differentiation in the stroma. No recurrence was seen with one year follow-up.CS should be included in the differential diagnosis of intra-orbital tumors. Complete resection remains the best therapeutic option to prevent recurrence. Close followup is recommended because malignant transformation, although rare, is possible. Chondroid syringoma (CS) are rare mixed tumours of sweat-gland which were first described by Billroth in 1859, that have both a bening and malignant form. Hirsch and Helwig in 1961 [CS of the orbit are extremely rare. In the better of our knowledege, only one case has been reported in the english litérature . Here weA 41-year-old woman presented with a history of left exophtalmos for 8 years and deterioration of visual acuity in the last 8 months. His medical and ocular histories were unremarkable. Physical examination disclosed no system abnormality except ocular findings. There was an indolor left exophtalmos, no axil with downward displacement of the globe. Abduction of the left eye was slightly restricted. Partial visual field defect was noted. Visual acuity was 8/10 in the left eye and 10/10 in the right eye. Cranial CT scan demontrated a superolaterally solid tumor, well circumscribed with homogeneous enhancement without bone erosion Figure . MRI shoChondroid syringoma (CS) is a rare, benign, skin appendagal tumor, first described by Billorth in 1859 for a group of tumors of the salivary gland that contained varying amounts of mucoid and cartilaginous material . VirchowIncidence of CS has been reported as less than 0.01 percent of primary tumors of the skin . CS usuaClinically, CS presents typically as a slow growing, painless, firm, non-ulcerated subcutaneous or intracutaneous nodule. The lesion commonly measures 0.5-3 cm in diameter [The sites of predilection for CS are on the head and neck region, particularly cheek, nose, or skin above the lip ,5. Less Kitazawa T and al  have repHistologically, CS consists of mixed epithelial and mesenchymal elements, with epithelial cells arranged in cords and forming tubules with a myoepithelial layer, set in a myxoid or chondroid stroma ,9. OsteoRadiological features of CS are not as suggestive as the histological findings. The MRI features are non-specific, but can accurately depict the anatomic extent and identify tissue of origin, depth of invasion and relation to adjacent structures, such as muscles and bones .The differential diagnosis of CS is made with other benign orbital lesions include pleomorphic adenoma, dermoids, lipomas, neurofibromas, meningioma, lymphangiomas, Cavernous hemangiomas and histiocytic tumors. . We descThe treatment of choice is complete excision without esthetic or functional disruption of surrounding structures . LateralChondroid syringoma is a benign tumor. However, rare cases of malignant CS have been reported ,13. ThesChondroid syringoma should be included in the differential diagnosis of intraorbital tumors, especially in middle-aged patients. Although malignant forms and recurrence are extremely rare, wide excision of the tumor and long-term followup are the most effective ways of management.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.The authors declare that they have no competing interests.HB, AA Conception and Design. HB Acquisition of Data. AA, BE, MO Analysis and Interpretation of Data. HB Drafting the Manuscript. AA Revising It for Intellectual Content. MB, BE Final Approval of the Completed Article. All authors read and approved the final manuscript.


The mandible is an infrequent localisation of primary osseous non-Hodgkin’s lymphomas. Few cases of mandibular non-Hodgkin’s lymphomas (NHL) have been reported.A rare condition of primary malignant non-Hodgkin’s lymphoma of the mandible in 53-year-old man, was reported at the Department of Maxillofacial and Plastic Surgery in Charles Nicolle Hospital .Histologic and Immunohistochemical (IHC) examination Confirmed a B-Cell lymphoma.The purpose of this report is to describe this rare case of NHL of the mandible, explore the diagnosis and workup, and discuss treatment strategies.In this localisation, neither the clinical features nor the radiologic appearances are often pathognomonic.Particular care must be taken to consider lymphoma in the differential diagnosis because this uncommon lesion can pose significant diagnostic problems and is frequently misdiagnosed. Non-Hodgkin’s lymphoma (NHL) arises primarily within the lymph nodes, but approximately 24% affect extranodal locations.3The mandible accounts for only 0.6% of isolated malignant non-Hodgkin’s lymphomas.5We present a rare case of primary lymphoma of the mandible. To our knowledge, there are only a few reports on this condition.A 53-year-old male with a past history of vague pain along his left mandible was referred to the department of Maxillofacial and Plastic Surgery at Charles Nicolle Hospital.Six months previously he had undergone the extraction of the sixth lower left tooth.He then began noting pain in the vicinity of the previous extraction site and left lower lip numbness developed.The panoramic radiograph showed a large radiolucent lesion with irregular margins in the left part of the mandible .This was thought to be “osteomyelitis” or an”abscess” and he was treated with a course of antibiotics.Then, the patient presented a gradually swelling on the left mandibular in molar area, with paresthesia of the ipsilateral lower lip.Extraoral examination showed a left mandibular body swelling, that measured 3*3 cm, expanding both laterally and inferiorly with no lymphadenopathy.The intraoral examination showed the presence of an expansive lesion of the mandible with normal appearing overlying mucosa. It was tender and painful.At the time of presentation, the patient did not report any systemic symptoms and had no fever, weight loss, or dysphagia.Computed tomography (CT) of the head and neck performed with i.v. ioded medium showed a wide osteolytic area in the left part of the mandible , 3; no pIncisional biopsy was done and showed dense infiltration of cells with small round hyper chromatic nucleolus and scant cytoplasm resembled lymphocyte .Immunohistochemical (IHC) examination revealed CD20 and PanB positive cells . This fiThe total body CT scan with i.v.ioded medium and bone marrow aspiration biopsy confirmed the mandible as the only localisation of the pathology. He therefore was categorized as stage IAE, primary mandibular NHL.The patient refuses all treatment by radio and chemotherapy. He has remained free of disease 10 months after surgery. A follow-up panoramic radiograph showed new bone formation after successful treatment.The lymphomas are a group of neoplasms arising in the reticuloendothelial and lymphatic system.8Malignant non-Hodgkin’s lymphomas may involve the medullar part of the bone during their evolution but only rarely arise primarily there.11Primary mandibular involvement makes up about 0.6% of all malignant non-Hodgkin’s lymphomas,8The most frequent site of occurrence in the mandible is the body.12Owing to their low frequency and to their not specific symptoms, primary malignant non-Hodgkin’s lymphomas of the mandible are often misdiagnosed.7The clinical presentation of mandibular NHL usually involves localized bone swelling, teeth mobility,11In this patient, Lymphoma was presented as mandibular intraosseous lesion. Paresthesia in mental region indicate compression or infiltration of mandibular nervous tissue.Despite extensive local destruction, systemic manifestations of malignant disease, such as weight loss, fever, and malaise, were very minimal.7This patient’s clinical presentation of a painful mass was consistent with reports of localized pain present at presentation. This mandibular swelling was initially thought to be due to an inflammatory odontogenic/periodontal process and was treated as such with antibiotic and dental extraction.In the head and neck region, lymphoma should be considered when there is unexplained dental pain, numbness, tooth mobility, swelling, ulceration, a mass in an extraction socket, or any ill-defined lytic osseous changes.1Histopathologic evaluation, together with immunophenotypic and cytogenetic studies, elucidate the histologic type.Diffuse large B-cell lymphoma (formerly known as diffuse histiocytic lymphoma) is the most common subtype of NHL, including primary mandibular NHL.3The work up should assess the extent of disease and allow for accurate staging.The current staging procedures for extranodal oral lymphoma include a panoramic radiograph, abdominal and chest CT scan, bone scans, routine laboratory tests , and bone marrow aspiration biopsy.13There are no radiographic pathognomonic findings.9To be classified as a primary NHL of bone there must be no evidence of visceral or lymphatic involvement and no distant metastases for at least 6 months following diagnosis.16Stage I NHL has a 5-year survival rate of 70% and the median survival time for IE is 10 years.10Treatment for primary lymphoma of the mandible typically consists of a combination of chemotherapy and radiotherapy9The role of surgery is for biopsy purposes and for the control of persistent or recurrent local disease.3For this patient the follow-up was extremely short; the treatment consisted of only surgical excision, he showed complete remission with the disappearance of all clinical evidence of disease and the normalization radiologic abnormalities related to the disease. In spite of this clinical remission, a complement of radio and chemotherapy is discussed to improve prognosis but the patient refused that treatment.Lymphomas are the most frequent nonepithelial malignant tumors in the oral cavity and maxillofacial region and represent the third most common group of malignant lesions in this site, following squamous cell carcinoma and salivary gland neoplasms.11We present a case of isolated mandibular NHL, stage IE. This report discusses this rare malignancy, including clinical presentation, histopathologic features, immunologic profile, treatment, and prognosis.


Sigmoid volvulus is a rare surgical complication occurring in pregnancy and puerperium. Only 84 cases of sigmoid volvulus in pregnancy have been reported in the English literature so far. We have reviewed the available literature on this subject and present another case recently managed at our institution. The available literature suggests that over the years, there has been an improvement in the maternal and fetal outcome for this critical condition, but delay in presentation and a further delay in diagnosis remain a challenge for the treating physicians. Our patient was a 30-week pregnant lady, who presented late with 6 days history of abdominal pain, distension and absolute constipation. She had evidence of multi-organ dysfunction at presentation due to complicated sigmoid volvulus. She was resuscitated and surgical exploration revealed gangrenous large bowel. Bowel resection with diverting ileostomy was performed, but she succumbed to the septic shock due to late presentation. Acute surgical pathology may be overlooked in pregnant patients due to reluctance in radiological workup and a high index of suspicion is essential for enhanced outcome. There is a need to increase the awareness amongst the obstetricians and general practitioners. Early diagnosis and referral and timely surgical intervention could significantly improve the outcome of this surgical and obstetric catastrophe. Sigmoid volvulus in pregnancy is a rare but serious complication associated with a significant maternal and fetal mortality . The funA 25-year-old pregnant lady, gravida 2 and para 1, presented to the emergency room with 6 days history of lower abdominal pain, abdominal distension, bilious vomiting and constipation. She had no significant past medical or surgical history and her menstrual and antenatal history were uneventful. She was being managed conservatively for these complaints at a local hospital before presenting to us in the emergency. On examination, she was dehydrated, tachycardiac with a heart rate of 136 beats per minute and tachypneic with a respiratory rate of 28 per minute. Her initial blood pressure was 102/69 mmHg and she was drowsy. Her abdomen was distended and tense, with generalized tenderness and a tympanitic note on percussion. The uterine size was of 30 weeks gestation. Rectal examination revealed no stool and cervical os was closed on per vaginal examination. She was clinically suspected to have peritonitis with evidence of septic shock. She was resuscitated with intravenous fluids and antibiotics.Plain radiographs of the chest and abdomen showed pneumoperitoneum, a single fetus and a distended gas filled transverse colon. Ultrasound scan of the abdomen revealed moderate amount of free fluid with mobile internal echoes representing bowel content and intra-uterine death of the fetus. Her laboratory workup revealed severe electrolyte imbalance with serum potassium of 6.3 mmol/L  and blood glucose level of 39 mg/dL . She had evidence of severe metabolic acidosis with serum pH of 7.18 , hypoxia with pO2 of 39 mmHg  and deranged coagulation. The surgical and obstetric teams in the emergency room evaluated the patient. While being resuscitated in the emergency room, the conscious level of the patient dropped further and she was intubated and put on the mechanical ventilator.th post-operative day due to overwhelming sepsis and organ dysfunction.With the clinical diagnosis of bowel perforation and peritonitis, the patient was taken up for emergency laparotomy. Intra-operatively findings were of sigmoid volvulus resulting in closed loop obstruction leading to distension and ischemia of whole large bowel. The whole of the colon was dilated, friable, and gangrenous. Multiple perforations were identified in the colon with around 800 ml of feculent material aspirated on opening the abdomen. Whole colon was mobilized & resected and diverting ileostomy with a Hartman’s procedure was done. A lower segment caesarean section was done for delivering the dead fetus and modified B-lynch sutures applied to the uterus. Post-operatively, she was continued on broad-spectrum antibiotics and shifted to the intensive care unit. She had an initial period of recovery for a couple of days, but subsequently, her pulmonary function deteriorated with development of pneumonia and adult respiratory distress syndrome. In addition to high ventilator support, she also needed increasing dose of inotropes and eventually expired on the 8The incidence of intestinal obstruction in pregnancy ranges from 1 in 1500 to 1 in 66431 deliveries . IntestiTo date, 84 cases of sigmoid volvulus have been reported occurring in the pregnancy and puerperium Table . LambertThe diagnosis of sigmoid volvulus is suspected when a pregnant female presents with a clinical triad of abdominal pain, distention, and absolute constipation. The average time from the onset of obstructive symptoms until presentation has been reported to be 48 hours . This isThe maternal and fetal outcome in sigmoid volvulus has been directly related to the degree of bowel ischemia and subsequent systemic sepsis. In our analysis of recent 20 cases, there were 4 (20 %) maternal and 8 (40 %) fetal deaths, including one ectopic pregnancy. It is important to note that all the maternal deaths occurred in the group of patients where delay in presentation and surgical intervention was more than 2 days.  SimilarlAnother important area of concern is the reluctance in the utilization of modern radiological diagnostic tools in pregnant patients. There have always been concerns about the radiation exposure of the fetus during pregnancy. Significant radiation exposure may lead to chromosomal mutations, neurologic abnormalities, mental retardation, and increased risk of childhood leukemia. Cumulating radiation dosage is the primary risk factor for adverse fetal effects, but fetal age at exposure is also important -24. ExpoThe management of intestinal obstruction and perforation in pregnant women is pretty much similar to that of non-pregnant women. The basis of therapy is early surgical intervention . SurgerySigmoid volvulus complicating pregnancy is very rare condition with significant maternal and fetal morbidity and mortality. Timely diagnosis mandates high index of clinical suspicion in patients presenting with abdominal pain, distension and absolute constipation. Hesitancy in getting X-rays in view of pregnant situation must be avoided and appropriate management must be defined. Delay in diagnosis and treatment beyond 48 hours results in increased fetal and maternal morbidity and mortality. Review of the available literature emphasizes the importance of early diagnosis and timely intervention to minimize maternal and fetal morbidity and mortality.A written informed consent was obtained from the next of kin 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.The author declares that they have no competing interest.MRK conceived the study. SR collected the data and prepared the initial manuscript. The manuscript was critically reviewed and approved by all the authors.


Inflammatory pseudotumor (IP) is a clinically aggressive but histologically benign condition of unknown cause. Its appearance in the temporal bone is uncommon. We present clinical, radiological, and histopathologic findings of three cases originating in the temporal bone. In the first case, a simultaneous IP of the temporal bone and parotid gland was found with histopathologic confirmation. In the second case, an enlarged cervical node, which was also believed to be related to IP, was observed accompanied with the temporal lesion. While the third case presented with chronic suppurative otitis media. Two of them were treated by surgery alone with complete resolve of the diseases. Another one underwent tympanomastoidectomy in combination with oral steroids, radiation, and chemotherapy, but the IP still recurred. A comprehensive review of the literature on clinical features of the temporal pseudotumor was conducted. Inflammatory pseudotumor (IP), first described by Brich-Hirshfield in 1905 and named by Umiker et al. in 1954, is a clinically invasive, yet histologically benign mass lesion of unknown etiology. It has a variety of histologic presentations and has been recognized under various names like plasma cell granuloma, inflammatory myofibroblastic tumor, histiocytoma complex, xanthomatous pseudotumor, fibrous xanthoma, and inflammatory myofibrohistiocytic proliferation . . 24]. ThIPs is usually isolated. There is only one case had a concomitant IP of the temporal bone and lung , and thrIP most commonly involves the lung and orbit, but it has been described in almost any location, in both sexes, at all ages , 10. It The diagnosis of temporal pseudotumor is one of exclusion based on history, clinical examination, radiological tests, and especially histopathology. Clinical manifestations vary widely among cases as indicated by our and previous reports. Laboratory abnormalities include anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and elevated ESR, CRP, or IgG4 , 28. CT Treatments for temporal pseudotumor are controversial with excision, corticosteroid, radiotherapy, chemotherapy, or any combinations of the four depending on tumor location, behavior, and the condition of the patient –24. SurgThe prognosis of IP is usually good. As far as our three patients are concerned, the outcomes are much different. In two cases treated by surgery alone, the diseases resolved completely. While the IP of the first case experienced several recurrences during followup, even though a combined modality therapy of surgery, steroid, radiotherapy and chemotherapy was chosen. This may due to the variable natural history of IP, the infiltrative and recurrent nature of focal lesion, or because of the insufficient steroid course used.IP of the temporal bone is rare. It has no distinctive characteristics both clinically and radiologically, and the presenting symptoms and biological behaviors vary widely among patients. In this paper, we present three new cases of temporal pseudotumor with variable clinical manifestations and treatment outcomes. In two of them, a possible involvement of cervical lymph node and/or concomitant IP of the parotid gland were found. To the authors' knowledge, our first patient represents the only reported case so far to have a simultaneous appearance of IPs both in the temporal bone and in the parotid gland.


Intramuscular hemangioma is a relatively rare, benign tumor of vascular origin, accounting for less than 1% of all hemangiomas. This paper reports a case of a 48-year-old female patient with intramuscular hemangioma in the upper lip, treated with sclerotherapy and subsequent complementary surgery. Hemangiomas are benign vascular alterations characterized by the proliferation of blood vessels, commonly occurring in subcutaneous and submucous tissue –6. When When the proliferation of blood vessels is found between skeletal muscle fibers, such tumors are denominated intramuscular hemangioma (IMH), which accounts for less than 1% of all hemangiomas . IMHs ocGiven the rarity, deep location, and variable clinical presentation of these tumors , the aimA 48-year-old female patient came to the stomatology service complaining of swelling in the upper lip. The patient history revealed that the lesion was asymptomatic, with evolution of approximately four years without treatment. The extraoral exam revealed asymmetry between the upper and lower lips. The intraoral exam revealed a submucous nodule coated with normal mucous, with firm consistency to the touch as well as clear limits and mobility. The initial diagnostic hypotheses were fibroma, pleomorphic adenoma, and neurilemmoma. Following incisional biopsy, the histopathologic exam revealed the proliferation of vascular capillaries invading the muscle tissue , leadingClinically, the differential diagnosis of submucous hemangioma includes cyst, mucocele, and Kaposi's sarcoma. The vitropressure maneuver makes a hemangioma acquire a pale coloration and reduce in size due to the emptying of its blood vessels –14. ThisThe slow growth with an evolution of four years indicated a likely benign nature of the tumor. Kanaya et al.  reportedIn the present case, the patient had not undergone any type of treatment since the perception of the first indications of the nodule and only sought treatment for esthetic reasons. Affected patients generally seek treatment due to esthetic or functional problems . SurgicaIn the case reported here, the tumor was located in the orbicular muscle of the upper lip, with a nodular appearance and absence of pain symptoms. However, such tumors may be accompanied by pain , 17–19. In the present case, the initial conduct was incisional biopsy of the tumor for diagnostic purposes. Bleeding was easily controlled during the procedure. The histopathologic analysis revealed the proliferation of capillaries invading the muscle tissue. There was also the proliferation of endothelial cells due to increased mitotic activity, with the accumulation of thin-walled capillaries separated by sparse conjunctive tissue stroma. The diagnosis was determined as capillary hemangioma , 20, 21.The treatment selected was sclerotherapy. However, before choosing the adequate type of treatment for a hemangioma, a number of characteristics should be considered, such as duration, size, location and number of tumors, patient age, and the hemodynamics of the tumor. Moreover, the viability of the intended technique must also be assessed .Sclerotherapy with monoethanolamine oleate was chosen due to its safety and ease of application and the fact that the treatment could be repeated without risk in the case of recurrence of the nodule . ThroughMonoethanolamine oleate initially irritates the venous or capillary epithelium, thereby producing an extra-vascular inflammatory response, which results in fibrosis and the occlusion of the blood vessels . This agIt should be borne in mind that an injection of a volume greater than the recommended amount can cause extensive tissue necrosis and trigger an anaphylactic reaction in patients who are sensitive to the drug . MoreoveSclerotherapy and surgery are the most often employed techniques in the treatment of hemangiomas of the mouth . SurgeryAfter three sessions of sclerotherapy, the patient did not appear for followup and only returned three years after the biopsy. Due to the residual presence of the tumor, the decision was made for the surgical removal of the submucous nodule, establishing the diagnosis of capillary IMH, with the proliferation of capillaries and the presence of hyaline perivascular material intercalated in the muscle tissue. The surgery was used as a complementary treatment to sclerotherapy. However, if the patient had shown up for the remaining scheduled sessions for the administration of the sclerosing agent, there may have been no need for surgery.The patient is currently in followup, with no clinical signs of recurrence or any other condition associated to the tumor. Although local recurrence may occur in more than 50% of cases, it has no correlation with the type of predominant vessel or anatomic location of the tumor and there are no reports of malignant transformation in cases of incomplete removal , 30. KanIt is important for dentists to know that diascopy is of considerable assistance to the correct diagnosis of superficial vascular lesions. However, when such tumors are located more deeply in the tissue, a biopsy is essential to the diagnosis. Moreover, one must take into consideration the correct indication of sclerosing agents for treatment.


Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. It may be suspected based on severe clinical signs, significant laboratory findings, and the tumor size. High-resolution ultrasonography with Doppler imaging has become the principal imaging method in the preoperative diagnosis of primary hyperparathyroidism. The ultrasound finding is not specific, but three of the described pictures are different from the typical appearance of benign adenoma of the thyroid gland and were suspicious in the context of clinical findings. According to the ultrasound criteria, one finding was benign and only the histological examination revealed carcinoma. Parathyroid carcinoma (PCa) is a rare cause of primary hyperparathyroidism (PHPT). It is mostly reported to account for 1% of cases; however, the proportion may be as high as 5% in some countries  . On rarePCa may be suspected based on the severity of clinical signs of PHPT, size of the parathyroid gland, and laboratory findings. PHPT due to a benign lesion is usually characterized by mild symptoms and course. The clinical signs and laboratory findings in PCa are usually marked , 5.99mTc-MIBI) scintigraphy are basic approaches to the preoperative diagnosis of PHPT. The two methods, each having its strengths, complement each other and are both characterized by high specificity and a positive predictive value (PPV). If used simultaneously, the PPV ranges from 85% to 90% [High-resolution ultrasonography (US) with Doppler imaging and technetium-99m sestamibi  and hypervascularization confirmed by Doppler imaging. It is usually localized in the superior parathyroid, posterior to the upper third of the thyroid lobe, and in the inferior parathyroid, posterior or inferior to the lower pole of the thyroid lobe. The shape is oval, ellipsoidal, or bean-like , 9.On a US scan, PCa may be distinguished from PAd by its size, usually of more than 3 cm, nonhomogeneous structure, lower echogenicity, and frequent degenerative changes—cystic cavities and calcifications and irregular borders .The case is interesting in that malignancy was doubled. A search for the etiology of severe hypercalcemia in a patient with relapsed B-cell non-Hodgkin's lymphoma revealed PCa. The 66-year-old male had laboratory-confirmed hypercalcemia 3.41 mmol/L . When looking for its cause, PHPT was confirmed, with an intact parathyroid hormone (iPTH) level of 616 pg/mL . The clinical signs of PHPT were dominated by recurrent left-sided nephrolithiasis . The nec99mTc-MIBI and 99mTcO4−) with two-phase technetium-99m sestamibi (99mTc-MIBI) confirmed the presence of hyperplastic parathyroid tissue in the right inferior parathyroid region.Imaging methods showed a large tumor in the right inferior parathyroid gland. Ultrasound examination revealed the nonenlarged thyroid gland with a normal structure. Under the lower pole of the right lobe, a large cystic tumor was found, sized approximately 5.5 × 4.0 × 4.0 cm, 47 mL in volume, and propagating dorsally and retrosternally. The tumor had a complex echogenic structure, with a hyperechoic solid part and several centrally located anechoic cavities. A CT scan of the neck and upper mediastinum revealed a cystic tumor in the region, sized 5.3 × 4.1 × 3.8 cm, which was well differentiated from the lower pole of the thyroid, propagating into the upper mediastinum up to the aortic arch and causing tracheal deviation . Along tThe tumor was completely removed by a surgeon. Histologically, PCa was confirmed .μmol/L; normal range: 44–104 μmol/L). In her history, no standard manifestations of PHPT were found  with two-phase technetium-99m sestamibi (99mTc-MIBI) revealed only low accumulation of the radiopharmaceutical at the caudal pole of the right thyroid lobe which was considered nonspecific (probably caused by degenerative and neoplastic changes in the parathyroid).Combining subtraction scintigraphy . Subsequently, PHPT was confirmed (iPTH 317 pg/mL). Apart from osteoporosis, no other clinical signs were present . There wA US scan revealed thyroid atrophy with autoimmune inflammation (4 mL in volume). Inferior to the lower pole of the right lobe, large hypoechoic PAd was found, with homogeneous structure, increased vascularization, a size of 3.5 × 1.7 × 1.1 cm, and 3 mL in volume. Since the finding was not suspicious according to US criteria, US-FNAB was not performed . ParathyA 61-year-old female with a long history of a bipolar disorder was admitted to a psychiatric ward for increasing apathy. Laboratory screening tests showed hypercalcemia (s-Ca 3.47 mmol/L). PHPT was confirmed, with an iPTH level of 2,070 pg/mL. The clinical signs of PHPT in the patient's history included renal colic recurring for about 5 years and acute pancreatitis . There wA US scan showed no thyroid abnormalities but in the left inferior parathyroid gland, there was a large tumor with cystic changes and calcifications, sized 4.4 × 2.5 × 1.8 cm and with a volume of 10 mL . US-FNABParathyroidectomy of the tumor was performed. Histological examination showed PCa with bleeding and necroses.99mTc-MIBI scintigraphy are the basic methods used for a preoperative diagnosis of PHPT. The two methods, each having its strengths, complement each other.It is difficult to diagnose PCa preoperatively as the clinical signs and laboratory findings are nonspecific. High-resolution US with Doppler imaging and 99mTc-MIBI sensitivity was 67–92% and its PPV was 86–91% [99mTc-MIBI scintigraphy proves beneficial [In the current era of minimally invasive surgery, high-resolution US with Doppler imaging has become the principal imaging method. In recently studied large groups of patients  operated for PHPT, the US sensitivity was 67–96% and its PPV was 82–98%; the s 86–91% . US examneficial .Generally, the advantages of US are low costs, repeatability and no radiation load. Although there is a risk of subjective bias, it may be of a high value if performed by an experienced sonographer. It must be realized that US is not meant to diagnose PHPT  but to localize the source of PTH overproduction. Therefore, the examining sonographer  needs to have precise clinical information available. US enables a simultaneous morphological assessment of the thyroid gland and an investigation of a concomitant thyroid disease and the surrounding area, in particular enlarged lymph nodes. US may be used for precise measurements of the PAd size (volume) and the description of its structure. Abnormal findings may be suggestive of parathyroid tumors or cysts.Parathyroid carcinomas are generally larger than adenomas, occasionally with a palpable mass in the neck. But as they affect mainly the inferior parathyroid gland and propagate into the mediastinum, they may be impalpable. According to the National Cancer Data Base 2000 review, in a group of 286 cases of PC reported over an 11-year period, the average size of tumors was 3.3 cm; regional nodes were enlarged in 36.7% of cases but only in 15% of those metastases were found . In anotThe conclusions of large studies of patients operated for PHPT are consistent in that the basic signs of suspected PCa include markedly increased serum levels of calcium and iPTH, severe clinical manifestation of PHPT, and often palpable tumor mass in the neck , 16. Som99mTc-MIBI scintigraphy was successful in confirming the presence of hypertrophic parathyroid tissue in a large tumor despite its cystic degeneration. However, the finding was not equally clear in a small fibrotic PCa, probably due to the fact that fibrosis limited adequate accumulation of the radiopharmaceutical. 99mTc-MIBI scintigraphy certainly does not allow to suspect potential neoplasia. Other imaging methods such as computed tomography or magnetic resonance imaging need to be used for preoperative diagnosis in case of large tumors involving the upper mediastinum (Case report 1).Three cases reported in this paper demonstrate the major advantage of US, that is, its ability to assess the enlarged parathyroid gland morphologically and suggest neoplasia in the context of clinical findings. As far as the tumor size as a sign of malignancy is concerned, both a large tumor (Case reports 1 and 4) and small PCa of less than 3 cm were recorded (Case report 2). Of great benefit was US-FNAB to determine iPTH levels in the aspirate. However, one case (Case report 3), probably that of early PCa, showed that, in the US examination, even the established “benign” criteria are not 100 percent reliable for PAd. Moreover, the laboratory findings were not suggestive of malignancy and only the histological examination confirmed malignant involvement of the parathyroid gland. It must be realized that, in some cases, PCa may only be diagnosed retrospectively, following local recurrence or metastatic involvement, and, that for a pathologist, it may be difficult to distinguish benign degenerated adenoma from carcinoma histologically .Clinical suspicion of PCa results from severe clinical symptoms and marked progressively increasing levels of serum calcium and iPTH. The US finding is nonspecific, but, more importantly, the appearance of the tumor is not typical for benign PAd. In most cases, a large tumor in the parathyroid region is revealed , occasionally propagating to the upper mediastinum and often with degenerative changes. In atypical or borderline cases, US-FNAB enables the iPTH level assay in the aspirate and cytologic examination.


We present a case of a 57 year old woman who suffered from massive hemoptysis; she was sent to our Department for a suspect neoformant lesion. We assumed it might be a Dielafoy’s disease and proceeded with an imaging study that confirmed the diagnosis. After embolization the patient no longer showed signs of bleeding. In brief, we concluded that whenever there is a suspect of Dielafoy’s disease, the biopsy has to be avoided. Dieulafoy’s disease of the bronchus is supposed to be a very rare disease. In fact only few cases are reported in literature . This condition should be clinically suspected in heavy smokers with recurring and unexplained episodes of massive hemoptysis. The bleeding can occur immediately after the biopsy or delayed up to 12 days. The diagnosis can be made through imaging. Angiographic images document that this vascular malformation is based on a left-to-right shunt, with a bronchial artery draining into a pulmonary artery. Endobronchial ultrasound may be helpful in detecting the vascular nature of the lesion[A 57 year old woman, non-smoker, non-atopic, was sent to our Department and we detected a neoformant lesion at the beginning of the superior right bronchus Figure. PreviouDieulafoy’s disease is an extremely rare vascular anomaly, characterized by the presence of a dysplastic artery in the sub-mucosa. At present, there are few proven cases reported in literature-4. The pIn brief, Dieulafoy’s disease of the bronchus is more frequent than thought, so this diagnostic option should be considered when there is a patient with recurring massive hemoptysis, which cannot be explained otherwise. Obviously, in this case the biopsy has to be avoided even when no active bleeding is evident.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.The authors declare that they have no competing interests.EB wrote the case report and revised the literature, GF participated to choose the images, SR participated to revise the literature, MS chose the images and performed the flexible bronchoscopy; All authors read and approved the final manuscript.


A 41-year-old male was admitted to Nagoya City University Hospital subsequent to experiencing a cough with bloody sputum for a few days. The patient had a 4-year history of lymphoplasmacytic lymphoma (LPL) and had achieved a good partial response to anticancer chemotherapy. Chest computed tomography (CT) showed an endobronchial tumor of the main carina. A bronchoscopy revealed an exophytic tumor at the main carina, and autofluorescence imaging bronchovideoscopy showed that the tumor and surrounding area were magenta in color. The biopsy specimens demonstrated that the endobronchial tumor was composed of large atypical lymphoid cells. The patient was diagnosed with a high-grade transformation of LPL. In addition to describing a rare case of transformed LPL involving the main carina, the present study also summarizes and discusses endobronchial lymphomas, with a brief review of a number of published studies. Malignant lymphomas (ML) frequently involve intrathoracic organs, but endobronchial involvement (EBI) is relatively rare –3. The pA 41-year-old male was referred to the Respiratory Division of Nagoya City University Hospital subsequent to experiencing a cough with bloody sputum for a few days. The patient had a 4-year history of a low-grade non-Hodgkin’s lymphoma (NHL) with IgM paraproteinemia, diagnosed as LPL. The primary LPL lesion was in the bone marrow, with no lesions detected on computed tomography (CT) or 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET). The patient had been treated with chemotherapy, including 8 courses of R-COP therapy , 12 courses of fludarabine and 5 courses of bendamustine, and had attained a good partial response (PR). The cough with bloody sputum was first noticed ∼1 month after the final dose of chemotherapy.μl ], and the concentration of circulating interleukin-2 receptor (IL-2R) was slightly elevated [538 U/ml ]. The lactate dehydrogenase (LDH) level was within the normal range. The IgG, IgA and IgM concentrations were 408 mg/dl , 11 mg/dl  and 187 mg/dl , respectively. A bronchoscopic examination showed an exophytic tumor with rough surfaces and a white, moss-like appearance at the main carina , and achieved a complete response (CR), as confirmed by FDG-PET. Thereafter, the patient underwent an allogeneic stem cell transplantation and is currently (15 months post-treatment) alive without recurrence.The present study describes a rare case of transformed LPL with carinal involvement. Although ML frequently involves the intrathoracic organs, endobronchial involvement (EBI) in NHL is rare, even in patients with advanced disease ,4. To thLPL is classified as a low-grade lymphoma and constitutes <5% of all NHLs. LPL occurs in older adults, usually involving the bone marrow, lymph nodes and spleen, while extranodal involvement and a leukemic phase are rare , and central airway involvement due to a solitary mass in the absence of clinically-apparent systemic lymphoma (type II) ,7. In JaFive mechanisms of endobronchial metastasis have been proposed: i) direct bronchial invasion of a parenchymal mass; ii) direct bronchial invasion of a mediastinal mass; iii) lymphatic spread to peribronchial connective tissues; iv) transbronchial aspiration of tumor emboli; and v) direct hematogenous metastasis . The mosThe majority of EBIs occur in patients with systemic or relapsed/refractory disease, and are more frequent in patients with Hodgkin’s disease than in those with NHL ,7. Althoin situ and early hilar lung carcinoma (Autofluorescence bronchoscopy is an important tool in the early detection of preinvasive bronchial lesions, including squamous dysplasia, carcinoma arcinoma . A new tarcinoma . The AFIarcinoma ,12, furtIn conclusion, the present study describes a rare case of LPL involving the main carina. Bronchoscopic examinations with TBB and AFI led to a diagnosis of a transformation to large cell lymphoma. These findings may provide further insight into endobronchial lymphoma and, particularly in patients with refractory low-grade lymphoma, emphasize the importance of histological examination of newly developed lesions.


Teratomas are rare germline tumors that originate from one or more embryonic germ cell layers. Teratoma of the kidney is extremely rare, and less than 30 cases of primary intrarenal teratomas have been published to date. We report the main radiologic features of an unusual case of mature cystic teratoma arising from the left kidney in a two-year-old boy. A left-sided abdominal mass was detected on physical examination and B-Mod Ultrasound (US) examination revealed a heterogeneous mass with central cystic component. Computed tomography (CT) demonstrated a lobulated, heterogeneous, hypodense mass extending craniocaudally from the splenic hilum to the level of the left iliac fossa. Nephrectomy was performed and a large, fatty mass arising from the left kidney was excised. The final pathologic diagnosis was confirmed as cystic renal teratoma. Teratomas are neoplasms that originate from one or more embryonic germ cell layers. The primary involvement site is the gonadal tissue; less frequent primary sites of involvement include the anterior mediastinum, retroperitoneum, sacrococcygeal region, brain and the gastrointestinal tract. Teratomas of the kidney are extremely rare, and less than 30 cases of primary intrarenal teratomas have been published to date. In some cases, renal teratomas have been mistaken as teratoid nephroblastomas . In most3 sized, heterogeneous mass consisting of intermediate echogenicity , renal cell carcinoma and angiomyolipoma. In childhood, diagnostic problems frequently occur especially when tumors contain a variety of heterogeneous elements. This is the main reason for misdiagnosis of teratomas. Differentiation of teratoma and other renal neoplasms is often problematic . The preChoi et al.  reported


Neuroendocrine tumours (NETs) are tumours that commonly involve the gastrointestinal system. Common primary sites in the gastrointestinal system include the small intestine, appendix, rectum and pancreas. Mesenteric NETs are extremely rare entities and are sparsely reported in the literature.We report the case of a 62-year-old woman with ectopic Cushing’s syndrome due to excessive adrenocorticotropic hormone secretion by a primary mesenteric tumour in the small intestine and its liver metastases.Although rare, the mesentery can be a primary site for NETs. It can cause similar symptoms and require similar treatment options. Tumour resection and debulking are acceptable ways to improve both the survival and symptoms. Neuroendocrine tumours (NETs) originate from neuroendocrine cells, which are widely distributed throughout the body. They secret different substances such as somatostatin, gastrin and adrenocorticotropic hormone (ACTH). Excess amounts of these substances can lead to various clinical presentations depending on the substances produced by the tumour. NETs most commonly involve the lungs and gastrointestinal system. They have also been occasionally reported in ovaries, the prostate, lymph nodes and the cervix. Common primary sites in the gastrointestinal system include the small intestine, appendix, rectum and pancreas. Mesenteric NETs are extremely rare and are often secondary to another primary tumour located elsewhere.We report the case of a 62-year-old woman with ectopic Cushing’s syndrome that was caused by excessive ACTH production by a tumour located in the small intestinal (ileum) mesentery and its liver metastases.Our patient was admitted to the endocrinology ward due to new onset diabetes, concomitant hypertension and cushingoid appearance. She had a six-year history of rheumatoid arthritis and was being treated with prednisolone. She reported a history of a benign thyroid nodule that had been biopsied 20 years previously. She also had a history of resolved peptic ulcer disease. She was suspected primarily to have exogenous Cushing’s syndrome and her prednisolone was therefore discontinued. Serum ACTH and 24-hour urine cortisol were measured and both were within the normal range. Because the serum ACTH level was not suppressed in response to a high dose dexamethasone suppression test, Cushing’s syndrome due to ectopic ACTH secretion was considered as the diagnosis.In order to localise the source of ACTH secretion, the patient had an octreotide scan and computed tomography (CT) of the chest, abdomen and pelvis. The abdominal CT demonstrated a mass in the small bowel mesentery with speculated margins and some hepatic lesions in the right lobe suggestive of metastasis . In the To exclude other possible primary sites, the patient underwent a small bowel series and colonoscopy. Neither revealed any pathological findings. Meanwhile, she was transferred to the intensive care unit because of decreased consciousness due to uncontrolled hypertension and diabetes mellitus. Owing to being high risk for surgery at the time, she was not scheduled for surgical intervention. She underwent monthly injection of octreotide acetate. Chemoembolisation was performed for her metastatic lesions in the liver while she was preparing for a surgical intervention. As her symptoms could not be controlled with medical treatment, she was scheduled for surgical intervention  one year following diagnosis.On laparotomy, a 5cm × 5cm mass was found in the mesentery of the ileum and multiple small liver metastases in both liver lobes were observed. The mesenteric mass and adjacent small bowel were resected and a bilateral adrenalectomy was performed . The mulThe pathology examination revealed adrenal cortical hyperplasia in both resected adrenals, a well differentiated neuroendocrine carcinoma in the resected mesenteric mass, no tumoural involvement in four resected lymph nodes and metastasis of neuroendocrine carcinoma in the liver lesion. The immunohistochemistry examination on the mesenteric mass was positive for chromogranin A, synaptophysin and cytokeratin, and negative for epithelial membrane antigen, confirming the diagnosis of NET.The patient had an uneventful post-operative period and was referred to the endocrinology ward for adrenal hormone replacement therapy. ACTH and cortisol levels were both reduced after surgery. At the one-year follow-up appointment, she had been in a good general condition with no significant problems. Her follow-up colonoscopy and chest CT were normal. The abdominal CT did not reveal any lesions other than her previous liver metastases, which were stable in size.Ectopic Cushing’s syndrome results from inappropriately high levels of ACTH, secreted by various types of tumours. These tumours consist of NETs, islet cell tumours, small cell lung carcinomas, medullary thyroid cancers, pheochromocytomas, carcinomas of the thymus and pancreas tumours.NETs originate from neuroendocrine cells, which are widely distributed throughout the body. They secrete various substances and hormones including ACTH. These substances result in diverse clinical presentations. NETs most commonly involve the lungs and gastrointestinal system. They have also been reported in other sites such as the ovaries, prostate, lymph nodes and cervix.The clinical presentation of these tumours depends on their location, and the types of hormones and substances they secrete. NETs that produce ACTH can result in ectopic Cushing’s syndrome. In these patients, serum ACTH levels are high and not suppressed in response to a high dose of dexamethasone (dexamethasone suppression test). In ectopic ACTH secretion, an octreotide scan and CT are very helpful in localising the lesions. In the majority of cases, there would be no need for further diagnostic workup although they may be missed if not searched for meticulously.Other diagnostic modalities such as radioguided surgery have been proven helpful in a number of cases.To make the diagnosis of mesenteric NET, one must first rule out other primary sites by the use of CT, colonoscopy, small bowel series and scintigraphy.NETs have specific immunohistochemistry features. Synaptophysin, chromogranin A, cytokeratins and neuron-specific enolase are usually positive.Medical and surgical methods can be used for the management of hypercortisolism and its complications. Medications such as metyrapone and aminoglutethimide are somewhat effective in symptomatic control but surgical interventions such as a bilateral adrenalectomy are preferred in patients who do not respond to medical treatment.Primary mesenteric involvement in NETs is extremely rare and very few cases have been reported worldwide. After a thorough investigation and ruling out other possible primary sites, it should be kept in mind that the mesentery can also be a primary site for NETs, causing similar symptoms and requiring similar treatment options.


The authors present a case of a presacral myelolipoma diagnosed in an 84-year-old male patient with longstanding pelvic pain and past medical history of bladder cancer. Pelvic computed tomography (CT) revealed a well-encapsulated and lobulated presacral mass, with mixed fat and soft-tissue attenuation. Magnetic resonance (MR) imaging provided further confirmation of macroscopic intralesional fat and excluded either adjacent bone invasion or bladder cancer recurrence. A presacral myelolipoma was suspected based on imaging findings, with liposarcoma and teratoma having also been considered for the differential diagnosis. The histological confirmation of the tumor was only attained postoperatively. This case report alerts to the possible presacral location of myelolipomas, which should be considered for every fat-containing lesion detected in this region. The main clinical, imaging, and differential diagnoses of this entity are reviewed in this paper. Myelolipomas are benign tumors composed of both mature adipose and hematopoietic elements, most commonly occurring in the adrenal glands , 2.This case report alerts to the possibility of presacral myelolipomas, which have been only occasionally reported in the literature but should nevertheless be considered in the differential diagnosis of every fat-containing tumor occurring in this region –10.The imaging findings of these tumors completely overlap with those of their adrenal counterpart, as was observed in this case. Notwithstanding, other possible presacral tumors, such as teratomas and liposarcomas, may have similar CT and MRI appearance and therefore the definite diagnosis still requires histological confirmation –12.An 84-year-old male patient was admitted to our hospital with complaints of longstanding pelvic pain with more than six months of duration and significant worsening during the past month. He denied changes in bowel habits and urinary symptoms. The patient had a medical history of bladder cancer treated conservatively. Other medical problems included arterial hypertension and benign prostatic hypertrophy.Pelvic contrast-enhanced CT revealed a well-encapsulated and lobulated solid mass posterior to the rectosigmoid colon, measuring 5 cm in greatest dimension. It abutted the sacrum posteriorly and was located between S3 and S5 . The masSubsequent pelvic MR imaging was performed in order to provide further characterization of the tumor. The presence of macroscopic fat was confirmed by the presence of components with high signal intensity on T1-weighted sequences that demonstrated a drop of signal on corresponding fat-suppressed T1-weighted sequences Figures . The sofBased on the imaging findings, myelolipoma, liposarcoma, and teratoma were considered in the differential diagnosis.Since the patient was symptomatic and surgery could not be precluded, a preoperative biopsy was not performed. The patient was submitted to a concurrent proctectomy (Hartmann type procedure) due to the evidence of extensive peri-rectal tissue involvement. On gross examination, a tumor with 5,5 × 4 × 3 cm of greatest dimensions was found in the perirectal adipose tissue . HistoloThe postoperative course was uneventful and after two years of follow-up there has been no evidence of local recurrence.Extra-adrenal myelolipomas are benign nonfunctioning tumors composed of mature adipose tissue and trilineage hematopoietic cells . They are histologically indistinguishable from their adrenal counterpart and resemble the bone marrow tissue , 2.To our knowledge, less than 50 cases of extra-adrenal myelolipomas have been published. Half of these have been reported in the presacral region, which constitutes their second most common location, only surpassed by an adrenal origin. In a minority of cases, perirenal, mediastinum, liver, and stomach locations have been reported in a minority of cases .The etiology of extra-adrenal myelolipomas is still unknown, though it has been suggested that they might arise from reactivation of peritoneal connective tissue hematopoiesis or embolization of bone marrow tissue –5.Presacral myelolipomas are usually diagnosed in elderly patients, usually between the fifth and seventh decades, as was the case of this patient. A 2 : 1 female to male predominance has been suggested , 7. The As was observed in this patient, presacral myelolipomas are typically round or oval well-circumscribed lesions that may adhere to the sacrum by direct contiguity. However, they do not invade the bone , 2, 9.Identification of macroscopic intralesional fat is a fundamental milestone in the imaging diagnosis of myelolipomas. Even so, their imaging appearance can be quite diverse, depending on the relative proportion of fatty and hematopoietic elements , 9. It hOn ultrasonography, these tumors present a heterogeneous echogenicity and may range from hyperechoic to hypoechoic masses, according to the predominance of fat or hematopoietic elements, respectively . On CT iThe differential diagnosis of presacral myelolipomas mainly includes other retroperitoneal tumors with fatty components, such as liposarcomas or teratomas, as well as extramedullary hematopoiesis. In addition, other non-fat-containing lesions commonly occurring in this region must be ruled out , 9.Liposarcomas represent the most prevalent and aggressive fat-containing retroperitoneal tumors. One must be aware that lipomas seldom occur in the retroperitoneum and therefore the diagnosis must be approached with extreme caution, since many of them are misclassified due to biopsy sampling errors . In termTeratomas may occasionally occur in the presacral region. However, their incidence is very low in older patients; a bimodal age distribution has been reported, one in the first six months of life and one in young adults . FurtherExtramedullary hematopoiesis can be distinguished of myelolipomas based on the distribution, location, and morphology of the lesions. Their involvement is most frequently multifocal and predominantly affects the mediastinum; these lesions are usually poorly circumscribed and present minimal macroscopic fat. Moreover, an association with hepatosplenomegaly and chronic anemia is generally observed , 8, 13.Neurofibromas and chordomas are usually easily distinguished from myelolipomas, since these do not usually contain fat components. Chordomas typically induce pain and neurologic defects due to extensive bone destruction, in opposition to neurofibromas, which cause only slight bone remodeling and may extend into the sacral foramina , 9.The definitive diagnosis of presacral myelolipomas is still based on histology, given the potential overlapping imaging appearance with some of the more menacing fat-containing tumors described above , 13.Given its benign nature, conservative management is indicated in asymptomatic nonhemorrhagic myelolipomas. Nonetheless, a pathological proven diagnosis obtained from percutaneous fine needle aspiration (FNA) and radiologic-pathologic concordance are required in such cases.Surgery remains the treatment of choice for symptomatic myelolipomas, as was the case of our patient. Surgery is also the preferred approach in indeterminate cases , 8 and i


Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood, adolescence and adult ages. We describe a female infant with prenatal diagnosis of unilateral left ventriculomegaly in which early brain MRI and contrast enhanced-MRI angiography, showed cerebral left hemiatrophy associated with reduced caliber of the left middle cerebral artery revealing the characteristic findings of the Dyke-Davidoff-Masson syndrome. Prenatal imaging, cerebral vascular anomaly responsible for the cerebral hemiatrophy and the early clinical evolution have never been described before in such a young child and complete the acquired clinical descriptions in older children. Differential diagnosis, genetic investigations, neurophysiologic assessments, short term clinical and developmental follow up are described. Dyke-Davidoff-Masson syndrome must be ruled out in differential diagnosis of fetal unilateral ventriculomegaly. Early clinical assessment, differential diagnosis and cerebral imaging including cerebral MRI angiography allow the clinicians to diagnose also in early infancy this rare condition. Dyke-Davidoff-Masson syndrome (DDMS) was first reported in 1933 [, her length was 43 cm  and the head circumference 31.2 cm . On admission she showed bilateral frontal bossing (present in the mother), a slight left occipital flattening, shortening of the neck, flat nasal bridge, rhizomelic shortening of four limbs and mild axial hypotonia with abdominal distention. The rest of the physical examination was unremarkable and no asymmetry of the body was present.Our patient is a female second-born child of healthy non-consanguineous italian parents. The mother was short in stature  and reported no exposure to teratogens during pregnancy. No family history of birth defects and neurologic or cognitive impairment was reported. A fetal ultrasonography (US) at 32 + 2 weeks of gestation (GW) showed a left ventriculomegaly with atrial width of 11.8 mm  was 65 (severely delayed). Nystagmus was no more present. Stretching toward objects, grasping and manipulation were present only with the left hand. She was able to sit with little back support, the sideways parachute reflex was absent on the right side. At 9 months of age her weight, length and head circumference were below the 3rd centile, left facial hypotrophy was evident and the Bayley II MDI was 58. Right upper and lower limbs hypotrophy was present. At rest the right arm was maintained in flexed position, early signs of contralateral hemiplegia. An EEG showed a normal background activity for age and a mild asymmetry in amplitude with a lower voltage in the left hemisphere.Our report can be considered original since we describe the earliest clinical presentation and diagnosis of a primary or congenital type of DDM. Early diagnosis in this case could be achieved thanks to a group with experience in pediatric dysmorphology.In our patient isolated unilateral ventriculomegaly, detected on prenatal US, was confirmed to be related to hemispheric hypotrophy on neonatal transcranial US and MRI. Transcranial Doppler US arose suspicion of left MCA anomaly so we performed a contrast enhanced-MRI angiography that confirmed the reduced caliber of the left MCA and its segments concomitant to unilateral brain hypoplasia.Clinically apart from the isolated depression exhibited at birth, our patient did not suffer respiratory distress, encephalopathy or multiorgan dysfunction. She showed transient weak suction and an isolated axial hypotonia of central origin persistent at two months with a normal spontaneous motility. Thus until the second month of age, clinical evidence of hemisyndrome in terms of posture, asymmetry of resting muscular tone or spontaneous motility was not present. The normal latency of the principal fVEP component, in the presence of a left occipital lobe hypotrophy, is compatible with the supposed subcortical generators of fVEP . NystagmThis very early clinical evolution has never been described before and integrates with previous clinical reports of DDMS in children -5 that dIn our patient isolated unilateral ventriculomegaly  was detected on prenatal US at 32 + 2 GW. Clinical series suggest that at between 15 and 40 GW, on prenatal ultrasound evaluation, an atrial width of < 10 mm is indicative of normal cerebral ventricular size. Ventriculomegaly corresponds to an atrial width of ≥ 10 mm and is considered mild when the atrial width is between 10 and 12 mm, moderate if 13-15 mm and severe when ≥ 16 mm . VentricDyke-Davidoff-Masson syndrome must be ruled out in differential diagnosis of fetal unilateral ventriculomegaly. We report the earliest diagnosis of Dyke-Davidoff-Masson syndrome described in infancy. Early clinical assessment, differential diagnosis and cerebral imaging including cerebral MRI angiography allow the clinicians to diagnose also in early infancy this rare condition.Written informed consent was obtained from the patient's parents for publication of this report and any accompanying images.The authors declare that they have no competing interests.EP performed clinical and neurological assessment, formulated the diagnosis, was involved in drafting the manuscript and revising it,.MP performed genetic counseling and was involved in drafting the manuscript, MG performed genetic counseling and was involved in drafting the manuscript, GM performed cerebral MRI and angiography and was involved in drafting the manuscript, GC performed clinical assessment, revising it critically and contributed to the final version of the paper. All authors read and approved the final manuscript.


Leiomyosarcoma arising in the head and neck region is a rare entity. Auricular involvement by the disease is further rarer with few cases reported in the literature. Usually auricular leiomyosarcoma is a disease of middle-old age. We report a case of leiomyosarcoma of the postauricular region in a young adolescent female. Surgery along with adjuvant radiotherapy was used for complete cure. Patient is disease-free for the last eight years and is on regular yearly followup. The aim of reporting this case is to add to the scarce existing literature regarding auricular leiomyosarcoma and its long-term outcome. Also, this is the first case report in young adolescent and second only of the post auricular region. Leiomyosarcoma constitutes a rare group of malignant tumor of the mesenchymal origin constituting 6% of all soft tissue sarcoma. About 3% to 10% of all leiomyosarcoma are found in head and neck region . We repoA 15-year-old female presented with 8-month history of progressive painless nodular swelling in right postauricular region. There was no history of ear discharge, ear pain, ear bleeding, or impairment of hearing. Patient had undergone local excision of swelling twice at local hospital within 1-month interval. Patient again had recurrence of swelling and for which she was referred to our centre. On examination, a 3/4 cm nontender firm nodular swelling with overlying scar mark (previous surgeries) was present in the right postauricular region. There was no palpable lymphadenopathy, and rest of the otolaryngological examination was within normal limit. Tympanic membrane and external auditory meatus was normal. Fine needle aspiration cytology (FNAC) was suggestive for features of low-grade sarcoma. Patient underwent wide local excision of the lesion with adequate margins. Postoperative histopathology showed 3.5 × 2.0 × 0.5 cm lesion with predominant spindle cell with mitotic rate of 6-7 per high power field. On further subjecting to immunohistochemistry (IHC), tumor cells showed immunoreactivity for smooth muscle antigen (SMA) and S-100 Figures . OverallLeiomyosarcoma affecting ear or temporal bone is a very rare entity. In the head and neck region, the most common site reported is sinonasal tract . Rasp etThe mainstay of treatment is usually wide local excision of the lesion with adequate margins. Radical resection has been suggested for more aggressive subcutaneous lesion. Although metastasis is rarely reported, if it occurs, it is generally by haematogenous route . RecurreAuricular leiomyosarcoma is a rare entity. Surgical excision with adequate margins is the treatment modality of choice. Radiotherapy can be used as an adjuvant treatment in aggressive cases.


Background. Osteoblastoma is a rare benign tumor. This tumor is characterized by osteoid and bone formation with the presence of numerous osteoblasts. The lesion is more frequently seen in long bones and rarely involves maxilla and mandible. Due to its clinical and histological similarity with other bone tumors such as osteoid osteoma and fibro-osseous lesions, osteoblastoma presents a diagnostic dilemma. Case Report. Very few cases of osteoblastomas involving maxillofacial region have been reported in the literature. This case report involves osteoblastoma involving right maxilla in an 18-year-old male patient. Following detailed clinical examination, radiological interpretation, and histopathological diagnosis, surgical excision was performed. The patient was followed up for a period of 3 years and was disease free. Summary and Conclusion. Benign osteoblastoma involving jaw bones is a rare tumor. There is a close resemblance of this tumor with other lesions such as fibro-osseous lesions and odontogenic tumors and thus faces a diagnostic challenge. Surgical excision with a long-term follow-up gives good prognosis to this lesion—Benign Osteoblastoma. Benign osteoblastoma is a rare tumor of bone representing less than 1% of all tumors in the maxillofacial region . HistoloDue to a wide spectrum of bone lesions reported in the literature with very close clinical, radiological, and histological interrelations, these tumors pose a diagnostic challenge. This may cause relevant problems with the differential diagnosis in view of the tumor's rarity, ambiguous clinicoradiologic presentation, and histologic features, which sometimes resemble osteosarcoma . A defin A 18-year-old male patient reported to our center with a chief complaint of pain and swelling of the left maxilla since 6 months. On extraoral examination, there was a diffuse bony swelling of the left maxilla which was slightly tender on palpation. Intraoral examination revealed bicortical expansion of the left maxillary alveolar ridge with the swelling extending from left first premolar to the first molar which was roughly elliptical in outline and nontender . The swe Radiographic examination revealed a well-circumscribed, radio-opaque lesion mixed with areas of radiolucency. The lesion was roughly round to oval in outline and measured around 3∗3.5 cm in diameter. The periphery of the lesion was surrounded by a well-defined radiolucent rim . There w Incisional biopsy was performed under local anesthesia. Access to the lesion was gained through a buccal crevicular incision. A diagnosis of benign bone tumor was then made based on the histological, radiological, and clinical findings. Complete excision of the tumor mass was planned under general anesthesia. Under strict aseptic precautions, complete excision of the tumor was performed through a buccal vestibular incision extending from canine to the second molar . Intraop There were no specific complaints postoperatively, and the patient was discharged 5 days postoperatively. The patient was disease free without any recurrence during the follow-up period.Benign osteoblastoma is a rare osteoblastic tumor of bone . This beDue to its rarity and close resemblance with other bony tumors of the jaws, osteoblastoma presents a diagnostic challenge at the first clinical presentation. Differential diagnosis may include fibro-osseous lesions, bone tumors, and odontogenic tumors. Osteoblastoma itself has been subclassified into different variants based on their aggressive nature and histopathological findings. Capodiferro et al., in their clinicopathologic study of four cases involving osteoblastoma of the mandible, elaborated on two variants, namely, the so-called toxic osteoblastoma and the aggressive osteoblastoma with each one having its specific characteristics . OsteosaBenign osteoblastoma clinically presents mainly with slight pain, swelling, and expansion of bone cortex. They have a limited growth potential and typically do not exceed 4 cm in diameter . RadiogrBenign osteoblastoma involving jaw bones is a rare tumor.There is a close resemblance of this tumor with other lesions such as fibro-osseous lesions and odontogenic tumors and thus faces a diagnostic challenge.Surgical excision with a long-term follow-up gives good prognosis to this lesion—Benign Osteoblastoma.