Rosai-Dorfman disease (RDD) is a rare entity that usually involves the lymph nodes but extranodal involvements have been seen in numerous cases, although RDD with cardiovascular involvement is extremely rare. We describe a case of a young male who presented with intermittent palpitations and was found to have a left atrium mass. Our case not only emphasizes the rarity of the above lesion but also highlights the importance of modern-day imaging like computed tomography, Cardiac Magnetic Resonance Imaging (CMRI), and PET scan in characterizing such nonspecific lesions and directing appropriate line of treatment. RDD should be considered as one of the differentials even for isolated cardiac lesions. Rosai-Dorfman disease (RDD) is a rare, benign histiocytic proliferative disorder with massive cervical lymphadenopathy as the most common clinical presentation. Various extranodal lesions of RDD have been reported in the literature but involvement of the cardiac system is rare. The etiology of RDD is unknown and confirmation of the diagnosis still relies on immunohistochemistry analysis. We report a case of a young male presenting with intermittent palpitations and demonstrating a mass in the left atrium on imaging. Imaging like cardiac MRI, CT, and PET scan has proven to be immensely valuable in outlining such nonspecific lesions.A 27-year-old African American male without any significant past medical history presented with intermittent palpitations and left ventricular hypertrophy on electrocardiogram (ECG). Two-dimensional (2D) transthoracic echocardiography showed an echodense mass in the left atrium. Further evaluation with Cardiac Magnetic Resonance Imaging (CMRI) demonstrated a heterogeneous broad base mass arising from the posterior superior wall and roof of the left atrium. The mass was located approximately at the expected location of coumadin ridge and measured 1.9 × 1.5 cm and demonstrated mild postcontrast enhancement . It arisPatient was further evaluated with Computed Tomography Angiography (CTA) of chest for better anatomical evaluation of the mass, which confirmed a cardiac region mass which is difficult to distinguish from the mediastinum and is located at the roof and posterior superior wall of the left atrium similar to the description on the CMRI . The mas18F-fluorodeoxyglucose positron emission tomography has proven to be a valuable imaging technique for distinguishing neoplastic lesions from benign lesions and evaluating the extent and processes of the disease. It not only demonstrates the complete staging of the disease but also can provide functional information about the disease activity to guide biopsy. FDG PET imaging in our patient demonstrates foci of increased uptake in the mediastinum and right perihilar region, corresponding to soft tissue findings on the previous CT study , an inflammatory myofibroblastic tumor (which has a background proliferation of spindle cells associated with an infiltrate of mononuclear inflammatory cells and ALK-1 positivity), metastatic malignant melanoma (in which cells are positive for Melan-A), Hodgkin's disease (which shows characteristic Reed-Sternberg cells and positivity for CD15 and CD30), and fungal or mycobacterial infections  . LiteratCardiac involvement in RDD is a rare occurrence and can mimic sarcoma, granulomatous disease, lymphoma, or benign atrial myxoma. Difficulty in evaluation of a cardiac mass by imaging is a frequently encountered scenario. Computed tomography (CT) helps us understand anatomical extent while magnetic resonance (MR) imaging and positron emission tomography (PET) scan can analyze morphology and metabolic features of a mass. Imaging helps in better characterization of a cardiac mass and also plays a pivotal role in assessment of its complications by demonstrating involvement of adjacent structures and mass effect or looking for a potential source of thrombus. Many pertinent clinical questions determining further management can be very well answered by appropriate imaging. Hence we illustrate the CT, MR, and PET findings of RDD presenting as a left atrial mass.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare entity with extremely uncommon involvement of the heart as an extranodal lesion. Imaging plays an important role in the diagnosis and management of lymphoproliferative disorders like Rosai-Dorfman disease which commonly present as nonspecific lesions. Although immunohistopathology remains as the main stay for confirmation of the disease, CT, MRI, and PET scan imaging are extremely useful in early detection and localization of the mass. PET scan is also a sensitive indicator for early prediction of treatment response in RDD. Through this case, we not only stress the importance of maintaining a high index of imaging suspicion for RDD but also delineate the significance of imaging in management and follow-up of such cases.


Inflammatory myofibroblastic tumors are uncommon neoplasms; presentation of these tumors in the lower extremities is extremely rare. We present a case of a 47-year-old male with fever, fatigue, and a slow-growing thigh mass. The inflammatory markers were elevated and the MR images showed a well-defined intermuscular lesion with mild heterogeneous enhancement. The lesion was excised and histologic examination was consistent with an inflammatory myofibroblastic tumor. No adjuvant therapy was needed and the patient remained asymptomatic with no evidence of tumor recurrence during the 2 years of follow-up. Inflammatory myofibroblastic tumours (IMT) have emerged from a broad category of inflammatory pseudotumours, with distinctive clinical, pathological, and molecular features . AccordiIn the past, these tumors were referred as plasma cell granulomas, xanthomatous pseudotumors, pseudosarcomatous myofibroblastic proliferations, myofibroblastomas, inflammatory myofibrohistiocytic proliferations, and inflammatory pseudotumors –8. They In this paper we aimed to present the unique clinical and radiographic features of this rare tumor, the differential diagnosis and treatment options through a review of the current literature.A 47-year-old male was referred to our hospital due to persisting fatigue and low-grade fever that lasted for the past 4 months. A slow-growing painful mass located at the left thigh was also noted.μL). The white blood cell count (WBC) was normal. On admission laboratory tests revealed markedly elevated inflammatory markers: C-reactive protein (CRP) was 152 mg/dL and erythrocyte sedimentation rate (ESR) 112 mm/h. Mantoux and Vidal-Wright tests were negative.Blood analysis showed a severe hypochromia, microcytic anemia , and thrombocytosis  of the thigh demonstrated a well-circumscribed mass, located in the intermuscular space between the vastus medialis, the vastus intermedius, and the adductor muscles. The mass was measured 8.5 × 6.6 × 5.8 cm. The lesion was characterized by low signal intensity on the T1-weighted images ; heterogA CT-guided biopsy showed a moderately cellular fibroblastic/myofibroblastic lesion with features suggestive of inflammatory myofibroblastic tumor. The differential diagnosis included a low grade fibroblastic/myofibroblastic sarcoma and a low grade myxofibrosarcoma.Excisional biopsy was performed and the mass was resected en bloc. The gross specimen consisted of an ovoid tumor with a firm, fibroelastic white, tan cut surface see . The micκ- and λ-immunoglobulin light chain expression was consistent with a polyclonal plasma cell population.Immunohistochemical examination showed the spindle cells to be variably positive for CD68 and focally positive for SMA and calponin expression while they were negative for CKAE1/AE3, S-100 protein, desmin, EMA, ALK, CD34 and MDM2 expression . ImmunohPostoperatively, the clinical signs and symptoms regressed, while laboratory tests gradually normalized. No adjuvant treatment was given. One month later an MRI of the thigh was performed showing absence of residual tissue of the pre-existent tumour. Thus, no further treatment was considered necessary. After 2 years of follow-up, the patient remains asymptomatic with negative laboratory and imaging tests and no signs of recurrence or metastatic disease.IMTs have emerged from a broad category of inflammatory pseudotumours, with distinctive clinical, pathological and molecular features. Herein, we presented a very rare case of inflammatory myofibroblastic tumor that was developed in a very uncommon anatomic location with a misleading symptomatology. We show the unique clinical and radiographic features of this rare tumor, the pathology and the differential diagnosis, along with the treatment options.The World Health Organization (WHO) classification of tumors of soft tissue and bone currently defines IMT as a distinctive neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and/or histiocytes , 16. HisThe pathogenesis of IMT remains unclear, although various allergic, immunologic and infectious mechanisms have been postulated . MultiplThe lesion usually occurs in children and young adults although it may occur as late as the eighth decade of life , 22, 25 The lung is the most common site of involvement but the neoplasm has also been reported in other organs including the stomach, mesentery, omentum, and retroperitoneum as well as the kidneys, renal pelvis, liver, spleen, esophagus, and lymph nodes , 27. PelThe biologic potential of IMTs is highly variable. In general, these are classified as tumours of intermediate biological activity with uncertain malignant potential. They may be associated with intermediate incidence of local recurrence, infiltrative growth, vascular invasion, and malignant sarcomatous transformation although these tumors are typically benign . The recThe clinical presentation of IMT varies depending on the anatomic location and they are frequently misinterpreted. Patients generally present with a mass without specific symptomatology. Vague abdominal pain or gastrointestinal complaints for intra-abdominal lesions, and cough, chest pain, or less often haemoptysis for pulmonary tumours consist the most common symptoms . Fever, Laboratory evaluation may reveal microcytic anaemia, a raised ESR, thrombocytosis, and/or polyclonal hypergammaglobulinaemia , 19, 33.Patients with IMTs of the extremities have no systemic symptoms; anemia, fever, or weight loss, and laboratory abnormalities are uncommon . HoweverIdentification of a soft tissue mass is usually incidental as a result of an extensive workup for fever of unknown origin or growth failure . The MR The definite diagnosis relies on histological and immunohistochemical confirmation of the disease. However, tissue samples obtained by CT-guided fine-needle or true-cut biopsies are usually insufficient to establish a definitive histological diagnosis and therefore the final diagnosis relies on the pathologic examination of the surgical resection specimen. Grossly, IMTs may be firm, solid or rubbery, occasionally gelatinous, with a white or tan cut surface. Tumour size ranges from 2 cm to 20 cm, with a mean size of 6 cm. Calcification, haemorrhage and necrosis are identified in a minority of cases , 37.Histologically, IMTs are composed of myofibroblastic and fibroblastic spindle cells with inflammatory infiltrate of lymphocytes, plasma cells, eosinophils, histiocytes in a background of abundant blood vessels. There exists a mixture of three different histopathological patterns. The first resembles a nodular fasciitis with elongated myofibroblasts containing abundant eosinophilic cytoplasm and vesicular nuclei, loose myxoid stroma with neutrophils, lymphocytes, and eosinophils, but few plasma cells. The second pattern is characterized by the presence of cellular with spindled myofibroblasts and fibroblasts in more compact stroma, arranged as islands surrounded by fibromyxoid stroma with prominent plasma cells and mitotic figures. The third pattern has densely hyalinized stroma with few spindle cells, few plasma cells or lymphocytes. All 3 histological patterns have no nuclear pleomorphism and no atypical mitotic figures –40.The differential diagnosis includes benign or malignant spindle-cell tumors such as fibrous histiocytoma, fibroblastic/myofibroblastic tumor and solitary fibrous tumor. They spindle-cell morphology can be misdiagnosed as both benign reactive processes and malignant spindle-cell neoplasms due to their similar morphology. The presence of nuclear hyperchromasia, atypical mitoses, or more than mild nuclear atypia argues strongly against the diagnosis.From the immunohistochemical stand point, the differential diagnosis includes a variety of neoplastic and reactive lesions. The dedifferentiated liposarcoma can have prominent inflammatory myofibroblastic tumor-like features with expressions of MDM2 and CDK4 for identification , 42. TheThe mainstay of treatment for this tumor is surgical resection with wide margins . RadiothComplete removal of the tumor leads to resolution of all symptoms and laboratory abnormalities. After complete resection, the prognosis of IMT is generally good with a low risk of distant metastasis. Incompletely resected tumors may have local recurrence within 1 year , 43, 46.Because IMT exhibits uncertain malignant potential ranging from local recurrence to malignant transformation, it should be closely followed clinically or radiologically over a long-term period. Recurrence has been seen up to 9 years after resection of the primary tumor . Our pat


Horse shoe shaped lipoma of the upper extremity is a very rare entity. We present a case of 45 years old female who presented with painless progressive swelling over the distal forearm and tingling and numbness over the ulnar nerve territory. MRI and surgical exploration showed a horse shoe shaped multilobulated lipoma encasing the distal ulna. The mass was excised in toto, and the sensory alterations were completely relieved at three months follow up. We would like to highlight this rare occurrence of a horse shoe lipoma and present a detailed history of this case to increase awareness amongst clinicians regarding this condition. Development typically begins with an initial insidious growth period followed by a prolonged and latent maintenance state.2 Rarely, lipoma may present in an unusual fashion, which might result in lesions entangled within neurovascular structures, and may also result in incomplete excision of the deeper part of the lesion.3 In patients with suspected complicated anatomical infiltration or multilocular lesions, or lesions that are deceptively large MR imaging is a useful tool for preoperative evaluation, identification of important structures and planning incisions.3,4 Although rare, abnormal extensions of the lipoma in the upper extremity must be kept in mind while dealing with such lesions. A rare case of multilobulated horse shoe lipoma encasing the distal ulna is highlighted.Lipomas were once believed to be rare in the upper extremity but are now considered common among soft tissue tumors of the hand.A 45 years old lady presented with history of tingling and numbness at palmar aspect of fingertips in the ulnar nerve territory of the right hand since the past four months. She also complained of an asymmetric progressive swelling of her right distal forearm since the past two years. Physical examination revealed a soft tissue swelling localized to the ulnar border of the distal forearm extending volarwards and dorsally. The mass was non-tender and did not transilluminate. The grip strength, adduction and abduction and range of motion were symmetrical in both the hands .MRI showed a well defined horse shoe shaped lobulated mass with homogenous appearance encasing the entire distal ulna . The masSurgical excision was done under brachial block with tourniquet control. Longitudinal incision was made on the ulnar border of the distal forearm. The FCU was stretched, and displaced outwards. On reflecting the FCU, the mass was exposed .Grossly, the mass was yellowish in color, lipomatous and multilobulated. The mass was gradually dissected . It extended from interosseous membrane on volar side to the interosseous membrane on the dorsal side encasing the entire distal ulna. As the ulnar neurovascular pedicle was at some distance from the mass and appeared largely uninvolved, no further exploration or neurolysis was considered.Histology was suggestive of lipoma with presence of well differentiated adipocytes. The post operative course was uneventful, and the tingling numbness gradually improved over a period of one month. At three months follow up, there was no evidence of any sensory alterations, and the patient was satisfied.1 These tumors may be deceptively large, and preoperative evaluation using MRI will be of help in planning of the incisions and identification of important regional structures that are often displaced or obscured. Using careful surgical techniques, complication rates can be minimized.There is adequate literature on compressive neuropathy caused by giant lipoma in the upper extremity.Horseshoe lipoma is a rare entity, and must be kept in mind as a differential diagnosis of soft tissue masses and recurrent tumors of the upper extremity. Thorough clinical evaluation supplemented with MRI gives us precise guidelines to the surgical approach to the management of these tumors.The authors declare no conflict of interest.


Mucocele is a benign lesion characterized by an extravasation or retention of mucous in submucosal tissue from minor salivary glands. Mucoceles are known to occur most commonly on the lower lip, followed by the floor of mouth and buccal mucosa being the next most frequent sites. Trauma and lip biting habits are the main cause for these types of lesions. Mucocele is a common oral mucosal lesion but it is rarely observed in the infant. This paper highlights the successful management of a rare case of mucocele in an 11-month-old child. Diagnosis and management of mucocele are challenging. For this reason we felt it would be interesting to review the clinical characteristics, histological features, differential diagnosis, and their treatment and evolution in order to aid decision-making in daily clinical practice. Oral mucocele represents one of the most common benign lesion of the oral mucosa that means a cavity filled with mucus (muco means mucus and coele means cavity), which is the secretory product of salivary glands. The mechanisms for the development of these lesions are two, mucus extravasation, generally regarded as being of traumatic origin, and mucus retention, resulting from obstruction of the duct of a minor or accessory gland. When located on the floor of the mouth these lesions are called ranulas because the inflammation resembles the cheeks of a frog . The mosAn 11-month-old male patient was referred to our department with the chief complaint of a “little ball” in the lower lip and that he had difficulty in sucking for more than 3 months. The baby was in good general health and no other symptoms were reported. Oral habits or a local trauma was not reported. The clinical examination revealed the presence of a soft tissue nodule on the lower lip mucosa  which waThe baby was reexamined after 15 days and 6 and 12 months. No recurrence was observed after 12 months .trauma,obstruction of salivary gland duct.Mainly physical trauma causes a spillage of salivary secretion into surrounding submucosal tissue. Later inflammation may become obvious due to stagnant mucous. Habit of lip biting and tongue thrusting are also one of the aggravating factors [Yamasoba et al.  highligh factors .In the first phase there will be spillage of mucus from salivary duct into the surrounding tissue in which some leucocytes and histiocytes are seen.In second phase, granulomas will appear due to the presence of histiocytes, macrophages, and giant multinucleated cells associated with foreign body reaction. This second phase is called as resorption phase.Later in the third phase there will be a formation of pseudocapsule without epithelium around the mucosa due to connective cells.The retention type of mucocele is commonly seen in major salivary glands. It is due to the dilatation of duct due to block caused by a sialolith or dense mucosa [The extravasation type will undergo three evolutionary phases .In the fe mucosa . It depeClinically they are characterized by single or multiple, spherical, fluctuant nodules, ranging from normal pink to deep blue in color, and are generally asymptomatic. The tissue cyanosis and vascular congestion associated with stretched overlying tissue and the translucency of the accumulated fluid beneath result in the deep blue color. At times it may rupture leaving slightly painful erosions that usually heal within few days. Para functional habits such as lip biting and Lip sucking and trauma explain the lower lip being the most commonly described location of extravasation mucoceles . They arThe history and clinical findings lead to the diagnosis of a superficial mucocele. The appearance of mucocele is pathognomonic and the following data are crucial: lesion location, history of trauma, rapid appearance, variations in size, bluish color, and the consistency . UsuallyRadiographs are the contributing factors in diagnosis of ranulas. Localization of these lesions is done by computed tomography and magnetic resonance imaging. High amylase and protein content can be revealed by the chemical analysis . A histoMucocele has clinical resemblance with many other swellings and ulcerative lesions of oral cavity and hence needs to be differentiated carefully. Palpation can be helpful for a correct differential diagnosis. Lipomas and tumors of minor salivary glands present no fluctuation while cysts, mucoceles, abscess, and hemangiomas do. A simple technique known as fine needle aspiration biopsy (FNAB) is very helpful, especially when differential diagnosis of angiomatous lesions is involved . Here we2 laser ablation, cryosurgery, intralesional corticosteroid injection, micromarsupialization, marsupialization, and electrocautery [Conventional surgical removal is the most common method used to treat this lesion. Other treatment options include COocautery .2 laser is that it minimizes the recurrences and complications and allows rapid, simple mucocele ablation. It is also indicated for the patients who cannot tolerate long procedures [There is no difference in the treatment of retention and extravasation mucocele. Small sized mucoceles are removed with marginal glandular tissue and in case of large lesions marsupialization will help to avoid damage to vital structures and decrease the risk of damaging the labial branch of mental nerve . Lacrimaocedures . Other tocedures .Mucocele is the most common benign self-limiting condition. Since these lesions are painless, it is the dentists, who usually pick up these lesions when the patient comes for a routine oral check or an unrelated dental problem. Management of mucocele becomes challenging because of their high chances of recurrence. However, surgical excision with dissection of surrounding and contributing minor salivary glands proved to be successful with least recurrence.


Meningiomas are the most common brain tumor in the adult population. This casereport describes the epidemiology, the clinical presentation as well as thecurrent treatment options for this condition.A 49 year-old man attended a chiropractic clinic with non-specific chroniclow back pain. Upon the history taking and the systems review, he reported aloss of both smell and taste for which investigations conducted by twodifferent otolaryngologists did not yield a specific diagnosis. The patient wasreferred to a neurologist who ordered a computer tomography scan thateventually revealed a compression brain tumor.Brain tumors can produce a large variety of clinical presentations, such asupper motor neuron lesion symptoms, altered consciousness or vital functionswhich are easy to identify. However, subtle signs, such as those presented inthis case, can be neglected.Clinicians should be aware of uncommon clinical presentations including cranialnerve or neurological dysfunction and refer their patient to a specialist whendetected. Meningiomas arise from arachnoidal cells of the leptomeninges and are the most commonprimary tumors of the central nervous system in adults . Over thA 49 year-old male attended a chiropractic clinic with a history of nonspecificchronic low back pain. Upon further questioning, the patient mentioned a progressivedecrease in sense of smell and taste which was reported as complete since the lasttwo and a half years. Following medical consultation for this matter since a yearago, two otolaryngologists had been consulted and both conducted nasal andoesophageal endoscopies that were considered normal. Lifestyle review revealed anaverage consumption of four alcoholic beverages daily and sometimes up to forty-eightto seventy-two beverages weekly. The result of the CAGE questionnaire  which yiUpper and lower limb neurological examination was bilaterally present, symmetric andrated as unremarkable for deep tendon reflexes, sensation and motor strength.Pathological reflexes were absent bilaterally. A cranial nerve exam revealed anosmia.In accordance with diagnostic imaging practice guidelines  cervicalA computer tomography (CT) scan ordered by the neurologist upon initial assessmentrevealed a 6 cm planum sphenoidale mass located in the frontal lobe, suggestinga benign meningioma Figures  and 2. TAt one year follow-up, the patient did not report any neurological symptoms, exceptanosmia. No further surgical complications were reported. He had no limitations tohis daily living activities and he returned back to work full time. Of interest, thepatient’s problem with alcohol resolved without treatment after thesurgery.Meningiomas of the midline anterior skull base such as the olfactory groove meningiomapresented in this case report are a rare clinical entity and represent about 10% of allintracranial meningiomas . OlfactoAlthough there are several meningioma subtypes,  their identification has little prognostic value. Only clear cellmeningiomas are thought to have clinical significance due to their aggressive behaviour . HistoloSeveral risk factors for the development of meningiomas have been hypothesized, anduntil now, the literature does not reveal any clear association. Environmental riskfactors such as exposure to ionizing radiation are currently acknowledged as the leadingcause underlying meningioma formation . As suchAlthough they usually remain clinically quiescent during the early phase of growth,meningiomas localized to the frontal lobe result in a variety of alterations incognitive and functioning behaviours . The mosWithin the primary care setting, the suspicion of olfactory groove meningioma may bebased solely on information gathered in the history. Although anosmia is thought tobe an early symptom, surprisingly few patients complain of olfactory dysfunction,making its detection extremely difficult during routine clinical examination.Proposed explanations for the lack of suspicion include natural gradual decline inolfactory function and lateralised anosmia in which case olfactory function ismaintained by the contralateral side . When paDiagnosis and decision on the best surgical approach are usually made with the use ofmagnetic resonance imaging, due to its ability to show the tumor’s duralorigin, along with perifocal edema and location or encasement of major vessels . In addiTreatment options for OGMs follow those in other skull base tumors. It is generallyaccepted that a small tumor, stable in size over time and asymptomatic, should bemonitored through serial imaging. However, for meningiomas occurring in patientsyounger than 65 years of age, surgery is usually recommended. Curative treatmentof meningiomas can usually be achieved by surgery alone when the tumor, its duralattachment and infiltrated bone can be completely resected. Even large size tumorscan be totally removed due to the arachnoid membrane creating a separation fromnearly all surrounding critical neurovascular structures . DespiteRadiation therapy comes into play in the management of meningioma when gross totalresection cannot be safely achieved, such as in optic nerve sheath meningiomas, whentumors recur after surgery and in radiographically diagnosed tumors when biopsy isnot possible. Radiotherapy is also provided as adjuvant therapy following resectionof atypical and malignant meningiomas ,9. The rWhen OGM resection results were initially reported, mortality ranged from 17.3% to22.7% . Recent Although meningioma is the most common primary tumor in the nervous system in adults, itis a rare condition. In the presence of subtle clinical signs such as those presented inthis case report, clinicians should keep a high index of suspicion for brain tumor. Inthe presence of uncommon neurological signs and symptoms, cranial nerve dysfunction orfailed conservative therapy, clinicians should not hesitate to refer the patient forfurther evaluation as part of a complete management program.Written informed consent was obtained from the patient for publication of this Casereport and any accompanying images. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AAM and JO contributed to the literature review and writing of the manuscript. Allauthors read and approved the final manuscript.


Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM. Vascular malformations are related to an abnormality in embryonic development and are composed of ectatic vessels . They are classified according to the vessel affected and the amount of flow through the vessel: high flow  and low flow . Vascular malformations are present from birth, but they may be asymptomatic and go undetected at that time. Most of these lesions appear in the head, neck, and trunk regions [The classification of benign vascular lesions and the related terminology was confusing until Mulliken and Glowacki  differen regions , and theOf all vascular anomalies, AVMs are the most dangerous because they can be associated with life-threatening complications. Despite their benign histology, deep lesions can produce serious systemic signs and symptoms due to extensive arteriovenous shunting and soft tissue hypertrophy. However, AVMs usually produce more subtle signs as they grow. Mandibular AVM shows a wide variety of signs and symptoms, such as dental mobility, otalgia, secondary pain due to thrombosis, facial asymmetry, and cosmetic distress –6. AVMs In this paper, we report a case of AVM of the mandible manifesting in the pediatric age, with a description of the clinical and imaging findings leading to the diagnosis and the strategy used for treatment.A 9-year-old girl was received for consultation at the Maxillofacial Unit of Hospital de Nens de Barcelona. She had been referred because of bleeding around the permanent mandibular left first molar. The child had no relevant medical history. Her mother reported gingival bleeding in the left mandible for two months. The patient presented facial asymmetry, with increased size of the lower facial third on the left side. Intraoral examination showed gingival bleeding and an ecchymotic area extending from the canine to the retromolar area . On palpCT confirmed the multiloculated appearance, bone expansion, and preservation of the dental structures within the affected area  . GadolinInterventional angiography through a femoral approach was decided as a diagnostic and therapeutic option. Angiography showed a high-flow vascular malformation with branches emerging from the facial artery, the lingual artery, and the inferior alveolar nerve artery. During the procedure, several branches were sclerosed with a 40 : 60 mixture of cyanoacrylate tissue adhesive  + Lipiodol.As only an angiographic embolization was not enough to solve the problem, a second embolization procedure with femoral access was scheduled 8 weeks later. This time in combination with an intraoral approach, transcortical puncture was used to access the interior of the mandible with a trocar under radioscopic control and angiographic support . An alcoPostoperative imaging showed control of the lesions with ablation of all branches. Follow-up consisted of physical examination, panoramic radiography, and MRI and CT studies. There has been no evidence of relapse to date, at 8 years of follow-up. The patient has functional occlusion with no deviation of the mandible or the occlusal plane. There are no aesthetic sequelae, and her permanent mandibular left second molar has erupted correctly; although radiographically they show some root resorption they have no significant mobility Figures –7.A high-flow AVM involving the mandible and surrounding soft tissue is extremely rare. The literature contains few cases and they are quite diverse; hence, the overall approach to take and therapeutic algorithm to follow in these patients remain uncertain. As reported by Corsten et al. , mandibuSuspicion of a maxillary or mandibular AVM should arise when the first clinical signs appear, including facial asymmetry, pain, swelling, local heat, tooth mobility, bluish discoloration of the mucosa and gums, or gingival or floor mouth bleeding in a patient with no history of predisposing factors, such as trauma or a known diagnosis of blood dyscrasia or oropharyngeal neoplasm ; LambergThe diagnosis is usually made in conjunction with clinical and radiographic investigations. The presence of a mandibular radiolucency on panoramic radiography may suggest AVM as a differential diagnosis. CT angiography shows the lesion and the feeding vessels. MRI can additionally help to differentiate between tumors and malformations. Nonetheless, angiography is the reference standard diagnostic method, although it is more invasive . RegardiHigh-flow lesions are a challenge for the attending team of professionals. The standard treatment for AVM has been endovascular embolization with subsequent surgical removal of the lesion , but nowRecently, minimally invasive surgical management proposals have emerged, such as cleaning the cavity through the alveolar process reported by Azzolini et al. , the bucThe case presented of a young patient with a mandibular mass consistent with a large and complex AVM was successfully resolved with two sclerotherapy approaches: intra-arterial and a transcortical approach. Clinicians must be mindful not to consider cessation of hemorrhage as an indicator of complete cure, because vascular reconstitution can lead to regrowth of the AVM and subsequent hemorrhage. Careful follow-up of patients treated is needed before the procedure's long-term success can be assessed.It is important to be aware of AVMs of the mandible so that the medical community and particularly dentists and pediatricians will take them into account in the differential diagnosis. The diagnosis is easily achieved with physical examination and imaging studies. Patients are usually diagnosed when the first clinical signs appear: pain, swelling, gingival bleeding, local heat, tooth mobility, or bluish discoloration of the mucosa and gums. A correct diagnosis is essential because treatment depends on it, and the proper choice of treatment is also a key factor. Patients with extensive or complex vascular anomalies should be treated by a multidisciplinary team that can provide appropriate, up-to-date treatment. Grouping of cases will facilitate clinical research in this line, with the aim of improving the therapy for this rare condition.


Objective. The brown tumour of hyperparathyroidism is a result of a metabolic disorder caused by primary hyperparathyroidism. Report. We described a case of a 37-year-old female patient presenting bimaxillary intraoral lesions and swelling in the neck. Incisional biopsy of the oral lesion was performed and histopathological examination revealed a central giant cell lesion composed by intense haemorrhagic exudate, abundant presence of giant cells, and areas with hemosiderin pigment. The patient also presented high levels of serum calcium and parathyroid hormone, hyperfunctioning parathyroid tissue, bilateral parenchymal nephropathy, and densitometry lower than expected, showing an advanced stage of osteitis fibrosa cystica. Synchronous parathyroid adenoma and papillary thyroid carcinoma were confirmed by imaging exams and histopathologically. Conclusion. The composition of all the clinical, pathological, and imaging findings led to the final diagnosis of brown tumour of hyperparathyroidism. The occurrence of parathyroid adenoma, papillary thyroid carcinoma, and brown tumours of hyperparathyroidism in their late stage (osteitis fibrosa cystica) associated with oral brown tumours involving the mandible and maxilla is extremely rare.  Osteitis fibrosa cystica is the end-stage manifestation of primary hyperparathyroidism. The bone lesions are the result of a metabolic disorder caused by the altered parathyroid, mainly due to adenomas in 85% of the patients [Severe parathyroid bone disease is rare.patients ; the ovepatients . Histolopatients .Brown tumours of hyperparathyroidism are infrequent today due to improved medical technology that enables early diagnoses and correction of PTH levels . The cla osteitis fibrosa cystica.We report herein a very rare case of concomitant maxilla and mandible brown tumours, thyroid carcinoma, parathyroid adenoma, andA 37-year-old woman presented to the Clinics of the Dental School, University of São Paulo, complaining of oral pain and facial deformity. The patient reported bleeding and painful oral lesions over the last 6 months.The physical examination revealed a consistent cervical mass on the left side of the neck. Intraoral inspection showed an exophytic mass extending from the right mandibular lateral incisor to the molars. The associated teeth were mobile and enclosed by the tissue growth. In the maxilla, a swelling was seen across the palatal midline, extending from anterior region to posterior right side. Both maxilla and mandible lesions were soft in consistency and tender on palpation Figures .The patient's past medical and family history were noncontributory. Due to the severity of the case, the patient was referred to the Clinics Hospital, Medical School, University of São Paulo (HCFMUSP), for systemic investigation and medical management.The X-rays images revealed an ill-defined osteolytic lesion causing expansion of the affected mandible, loss of lamina dura, and roots resorption of the anterior teeth Figures . ComputeThe bone densitometry values were below the expected limits for the patient's age and parathyroid scintigraphy showed hyperfunctioning parathyroid tissue on the left side.An incisional biopsy was performed in the mandible lesion and histopathological examination was consistent with central giant cell lesions, composed of intense hemorrhagic exudate, abundant presence of multinucleated giant cells, and areas with haemosiderin pigment Figures .The biochemical investigations were carried out and showed marked hypercalcaemia  and an elevated parathyroid hormone (PTH) concentration  in the blood.The patient had partial thyroidectomy and parathyroidectomy on the left side. The PTH concentration taken 1 hour after the adenoma had been removed confirmed a level within the normal range. The diagnosis of papillary thyroid carcinoma and parathyroid adenoma was confirmed histologically .The serum calcium and inorganic phosphorus levels were estimated daily after surgery. The postoperative hypocalcemia was administered by calcium carbonate orally in addition to vitamin D until stable levels. Thyroid hormone replacement therapy was instituted.At 6-month follow-up, there was only a modest improvement of oral pain, but the oral lesions had remained. The patient interrupted her clinical follow-up despite the efforts of the medical team.The occurrence of brown tumours in both jaws concomitant with the presence of hyperparathyroidism, parathyroid adenoma, and synchronous papillary thyroid carcinoma is very rare according to the literature , 8. The There is a great variation in the clinical expression of hyperparathyroidism. Hyperparathyroidism may be caused primarily by excessive secretion of PTH or secondarily due to kidney disease. The resultant hypercalcemia causes various metabolic problems, such as kidney stones, gastrointestinal disturbances, and muscular weakness , 2. Our Radiographically, the present case was similar to those previously reported by other authors . The proThe destructive bone lesions of brown tumour show similar histological features to those viewed in central giant cell lesions, aneurysmal bone cyst, and cherubism and these are considered as differential diagnosis , 7. The The pathogenesis of this disease is caused by the marked increase in osteoclastic bone resorption followed by replacement of marrow spaces with fibrovascular tissue and increased formation of osteoid. The cystic spaces are populated with clusters of giant cells as well as with hemosiderin-filled macrophages and plump fibroblasts. Clinically, these areas appear as red-brown friable mass, hence the term brown tumour , 10. osteitis fibrosa cystica. Occasionally, osteitis fibrosa cystica can be mistaken for a malignant lesion, even though it is reactive and clearly nonneoplastic [Brown tumours are the late manifestation of severe hyperparathyroidism; this clinical situation is also known asoplastic .Primary hyperparathyroidism is characterized by hypersecretion of PTH, which is caused by adenomas in 85% of all cases. The presence of brown tumours is recognized in less than 5% of these cases .According to the literature, concomitant thyroid disease is present in 3.1% of patients with primary hyperparathyroidism , but theIt was not possible to evaluate whether surgical excision of parathyroid adenoma and papillary thyroid carcinoma allowed the regression of oral lesions in our patient. After 6-month follow-up, she showed discrete improvement in pain, but she did not perform imaging tests that were previously requested for bone evaluation, and after this appointment she interrupted the treatment.Some authors report that the excision of parathyroid lesions results in controlled serum levels leading to a gradual decrease in the maxillary tumours; therefore, treatment is directed to the correction of hyperparathyroidism instead of any local intervention in bone affected by brown tumours. Once hormonal dysfunction is corrected, the bones tend to mineralize completely, resulting in the regression of the osteolytic lesions , 14. Broosteitis fibrosa cystica) with oral manifestation involving the mandible and maxilla is extremely rare. The appropriate resection of the affected glands leads to the metabolic control of serum levels of calcium and PTH. Exuberant cases, as the one reported, still occur in developing countries due to poor and inefficient governmental health and education policies.The composition of all the clinical, pathological, and imaging findings led to the final diagnosis of brown tumour of hyperparathyroidism. The occurrence of parathyroid adenoma, papillary thyroid carcinoma, and brown tumours of hyperparathyroidism in the last stage (


Massive arterivenous malformations (AVM) in the cervico-facial area are rare but potentially life-threatening. Treatment protocols are not well-established. A 41-year old man presented large painless rubber-like mass within the entire neck, which also extended intraorally through the floor of the mouth, showing a slow growing pattern for 5 years. Angiography diagnosed it as cervicofacial AVM. Treatment approach consisted on the embolization of the right upper thyroid, lingual and facial arteries under intravenous sedation. Three days later, bilateral radical neck dissection and subtotal glossectomy was performed. A musculo-cutaneous pectoralis major pedicled flap was harvested to reconstruct the floor of the mouth. Treatment of massive AVMs in the cervico-facial area is challenging due to the associated disfigurement and frequent recurrence rate due to incomplete resection. Also, massive bleeding may be present despite pre-operative super-selective embolization. A new case is presented with focus on surgical treatment considerations. Key words:Arteriovenous malformation, high-flow vascular malformation, cervical region, tongue, surgical resection Vascular malformations are collections of abnormal vessels displaying a flat non-proliferative endothelium that most frequently develop in the adulthood, although are always present at birth and grow progressively . They caThe case presented here clearly illustrates the aggressiveness of a giant AVM involving the whole anterolateral cervical region bilaterally from the infrahyoid muscles up to the mobile tongue, and also gives some recommendations for a safer complete surgical resection.A 41-year old man presented with a large cervical mass involving both sides of the neck, with associated macroglossia for 5 years. No personal history of disease was reported. Physical examination revealed a large painless rubber-like mass within the entire cervical region, which also extended intraorally through the floor of the mouth, pushing it upwards. The tongue was largely involved with lingual mandibular imprints and also marked protrusion and displacement of the teeth over the vestibular region both in the upper maxilla and mandible. Progressive limitation of breathing, swallowing and speech was observed. A cervicofacial CT-scan showed a large mass in the floor of the mouth with involvement of the tongue, mylohyoid and geniohyoid muscles and anterior cervical soft tissues, formed by multiple tortuous small and large-size vessels, with preservation of the hyoid bone and both submaxillary glands. No pathologic lymph neck nodes were observed. Angiography of both external carotid arteries confirmed the vascular nature of the mass, this particular case being an arteriovenous malformation [AVM] mainly supported by the right lingual artery, but also by the facial and upper thyroid arteries. The mass presented large draining veins crossing the midline that finished at the left jugular vein (Fig. Treatment approach consisted on the embolization of the right upper thyroid, lingual and facial arteries under intravenous sedation followed by surgical resection 3 days later. Embolization was tried with rounded beads [microspheres] of polymers and collagen. Unfortunately, embolization was scarcely successful, as it was checked during open surgery. Tracheostomy was performed under local anaesthesia. Under general anaesthesia and intubation by the tracheostome, a bilateral cervicotomy form the right to left mastoid apophyses was performed to gain access to all the cervical levels. A visor flap was elevated for a facial degloving, followed by a median mandibulotomy and mandibular swing. This approach allowed wide access to the entire mass communicating the neck and the oral cavity. Both internal and external carotid arteries and jugular veins were exposed. The right upper thyroid, lingual and facial arteries were dissected and transected at their exit from the external carotid artery. This maneuver allowed a safer dissection of the mass both through a lateral and inferior approach. The thyroid gland was preserved, while resection was performed until the pre-laryngeal, infrahyoid and suprahyoid muscles were exposed. Further dissection along this layer was performed up to the submental region Fig. . An V-Y Failure of conservative treatment has been reported in the management of high-flow vascular malformations . This coSince AVMs are rare in the head and neck, limited experience is available concerning the surgical treatment of cervicofacial massive AVMs. The rationale for an adequate management of these lesions must be clearly established pre-operatively Fig. , while aet al. (Jackson et al.  reportedet al. ,5.This is why resective surgery is advisable for most of cervico-facial AVMs. While most authors recommend surgery within the first 48-72 hours post-embolization, others  suggest et al. (Surgery of massive giant AVMs, such as the one presented here, must be focused on entire resection of the specimen and adjacent involved soft tissues. Koshima et al.  also notet al. (As presented here, reconstruction should be performed to cover the generated defect in the cervical region and also the floor of the mouth. A pectoralis major myocutaneous flap is bulky enough to cover the excised cervical region, to protect the cervical vascular axis, and to provide a reliable support for the remnant tongue. Indeed, the use of myocutaneous flap reconstruction has been reported to prevent the recurrence of AVMs . Yamamotet al.  when usiet al.  reportedet al. . RegardiIn summary, surgeons must be aware of the severity of this type of AVMs in terms of pre-operative and intra-operative life-threatening conditions. For massive cervico-facial AVMs, bleeding is not the exception, but the rule, as pre-operative embolization is frequently unsuccessful. Whenever achievable, reconstruction with vascularized pedicled or free flaps must be performed to cover the defect or the surgical field.


A 55 year old man, with no history, referred by his doctor for headache lasting for 3 months, a week before the patient had two complex partial seizures became secondary generalized. Physical examination was unremarkable. EEG and laboratory data were normal. The CT scan showed a highly hypodense (-73 to -86 H.U) mass of the quadrigeminal region (A). The cranial MRI with and without contrast administration, revealed a non-enhancing 3×2,7cm quadrigeminal cistern mass lesion, hyperintense on T1 (B)and T2 (C) weighted sequences, On fat suppression pulse sequence intensity of the lesion is homogeneously decreased (D). These signal intensities were consistent with fat. The patient was put under valproic acid with a very good control without any seizure activity for 12 months. Intracranial symptomatic lipomas located in the quadrigeminal cistern are extremely rare. These lesions are usually managed conservatively as surgical removal is difficult owing to their deep localization and close contiguity with adjacent neurovascular structures.


This report is aimed at giving emphasis on the use of simple dynamic TVS for accurate diagnosis of rare spontaneous ruptured pyometra causing peritonitis. Pyometra is collection of pus within the uterine cavity and is usually associated with underlying gynaecological malignancy or other benign causes. Spontaneous rupture of pyometra is a rare complication. We report a case of a 65-year-old female who presented with acute abdomen and was diagnosed with a ruptured uterus secondary to pyometra and consequent peritonitis on dynamic transvaginal sonography (TVS) which was later confirmed on contrast enhanced computed tomography (CECT). An emergency laparotomy was performed and about 800 cc of pus was drained from the peritoneal cavity. A rent was found in the anterior uterine wall and hence hysterectomy was performed. Histopathology revealed mixed inflammatory cell infiltrate with no evidence of malignancy. There are only 31 cases of ruptured pyometra reported till date, most of which were definitively diagnosed only on laparotomy. In only two of these cases the preoperative diagnosis was made on CECT. We report this case, as the correct and definitive diagnosis was made preoperatively on dynamic TVS. To our knowledge, Pyometra is collection of pus in the uterine cavity with several aetiologies like malignancy of genital tract more commonly and sometimes benign lesions and other benign conditions. Clinical presentation of pyometra is a whitish discharge per vaginum and sometimes lower abdominal pain and bleeding per vaginum also. Spontaneous rupture of pyometra is a rare complication. Usually a definite diagnosis of spontaneous rupture pyometra was made only by laparotomy. This report is aimed at giving emphasis on the use of simple dynamic TVS for accurate diagnosis of rare spontaneous ruptured pyometra causing peritonitis.A 65-year-old postmenopausal diabetic female presented to the emergency department with complaints of abdominal pain, fever, and distension along with white discharge per vaginum for seven days. Her vital parameters were stable except for mild tachycardia (pulse rate being 110 beats/min). Blood pressure was 110/70 mm Hg. Temperature was 100 degree F. Diffuse guarding, rigidity, and tenderness were present on abdominal palpation. On per speculum cervix was stenosed with minimal white discharge through external os. Per vaginal examination revealed retroverted normal sized uterus with free fornices.Laboratory investigations revealed neutrophilic leukocytosis , elevated ESR of 32 mm/hour, and a low haemoglobin 10.7 gm/dL.http://dx.doi.org/10.1155/2016/1738521). Based on these findings a diagnosis of spontaneous rupture of pyometra into peritoneal cavity was suggested. Emergency CECT abdomen and pelvis was performed which confirmed the pyometra, defect of 2.0 × 2.0 cm in the anterior wall of the uterus, and moderate peritoneal collection with multiple air pockets and smooth peritoneal enhancement suggesting pyeopneumoperitoneum with peritonitis  was performed which revealed moderate free fluid with thick echoes within the peritoneal cavity and mild collection within endometrial cavity. TVS was performed to look for a cause of endometrial collection, which revealed a collection with thick echoes within the uterine cavity suggestive of pyometra and a defect of size 2.0 × 2.0 cm in the anterior wall near fundus . Using a Figures .An emergency exploratory laparotomy was done. At laparotomy a defect of 2 × 2 cm was found in the anterior wall of the uterus and hence abdominal hysterectomy was performed . No evidPyometra is defined as the collection of pus in the uterine cavity. The main cause of pyometra is cervical canal occlusion usually secondary to carcinoma cervix; however, other benign causes are endometrial polyp, leiomyoma, infection especially senile cervicitis, a forgotten intrauterine device, cervical occlusion after surgery, and radiation –3. The uSpontaneous rupture of pyometra is a rare complication, the incidence being 0.01–0.5% of all gynaecological patients and more common in postmenopausal females –8.Spontaneously perforated pyometra is difficult to diagnose preoperatively. Clinically it commonly mimics the symptoms of gastrointestinal tract diseases. Misdiagnoses are common and most frequent preoperative diagnosis is generalised peritonitis secondary to gastrointestinal perforation . It is m There is no case report suggesting perforated pyometra being diagnosed preoperatively on dynamic TVS till date. This report is aimed at giving emphasis on the use of simple dynamic TVS for accurate diagnosis of rare spontaneous ruptured pyometra causing peritonitis. The advantage of USG over CT is accessibility, affordability, and no risk of radiation. USG is readily available and cheaper compared to CT and hence helpful to patients of smaller institutions especially in developing countries.Preoperative diagnosis of perforated pyometra on CECT was made in only two cases in which CT suggested the diagnosis and surgical intervention was performed , 11. Abd To our knowledge this is the first such case report revealing spontaneous ruptured pyometra causing peritonitis being diagnosed preoperatively on dynamic TVS.In our case, the patient presented with symptoms of generalised peritonitis and TVS demonstrated the pyometra and a defect in the anterior wall of the uterus near fundus. Dynamic TVS showed real time movement of endometrial collection and peritoneal collection through defect in either direction further confirming diagnosis of perforated pyometra. The findings were confirmed on subsequent CECT abdomen, which revealed pyometra, uterine perforation in anterior wall near fundus, and pyeopneumoperitoneum.The treatment of spontaneously ruptured pyometra is emergency laparotomy, peritoneal lavage and drainage, and hysterectomy.Although spontaneous rupture of pyometra is rare, it should be kept in mind as a differential diagnosis in postmenopausal women presenting with acute abdomen and dynamic transvaginal sonography should be advised prior to laparotomy for definitive diagnosis. USG is more accessible and cheaper compared to CT and hence helpful in diagnosis of ruptured pyometra in patients from smaller institutions especially in developing countries. A carefully performed dynamic transvaginal sonography will give the accurate diagnosis so as to help with early intervention and proper treatment to reduce the associated morbidity and mortality.Video of dynamic transvaginal ultrasound reveals real time movement of endometrial collection through defect into peritoneal cavity on increasing transvaginal probe pressure on cervix and refilling of endometrial cavity by reverse movement of peritoneal collection into endometrial cavity after release of pressure, confirming diagnosis of spontaneous rupture of pyometra.


The present study retrospectively examined 24 cases of pathologically confirmed synovial sarcoma and analyzed the clinical presentation and imaging findings in order to explore the imaging features of synovial sarcoma. The majority of the lesions were large , rounded or lobulated, relatively well-defined tumor masses in the extremities near the joints or deeply located. On computed tomography (CT) scans, the lesions demonstrated intensity signals similar to those of muscle. Six cases exhibited punctate calcification in the periphery of the tumor. On T1-weighted images, the largest lesions of >5 cm, were usually heterogeneous, with a signal intensity similar to or slightly higher than that of muscle. On T2-weighted images, heterogeneous high-intensity or slightly high-intensity signals were observed, with 12 cases exhibiting a high signal consistent with hemorrhage and 12 presenting signals that indicated internal septations. Contrast-enhanced scanning revealed heterogeneous enhancement in the majority of the lesions and no enhancement in areas of cystic necrosis or internal septations. Synovial sarcoma has specific imaging features, and comprehensive analysis of CT and magnetic resonance imaging can improve the accuracy of the pre-operative diagnosis. Synovial sarcoma originates from mesenchymal tissue that undergoes sufficient differentiation to exhibit the histological appearance of the synovium. Synovial sarcomas constitute 10% of all primary soft-tissue malignant tumors  and occuThe lack of characteristic clinical manifestations and specific imaging features leads to a high rate of misdiagnosis for synovial sarcoma . The preIn total, 24 cases of pathologically proven synovial sarcoma treated at The First Affiliated Hospital of Xinxiang Medical University  between 2004 and 2011 were included in the present study. The subjects consisted of 15 females and nine males, with a median age of 35 years . The patients typically presented with a palpable mass or pain that had persisted for three weeks up to six years. In total, 12 patients presented with local tenderness upon physical examination and six patients possessed distant metastasis at the time of diagnosis. No redness or swelling was observed in any patient.In total, 18 patients underwent MRI, including 15 who underwent contrast-enhanced MRI. Nine patients underwent CT and another nine patients underwent contrast-enhanced CT scans. MRI fast-spin echo T2-weighted imaging , T1WI  and short T1 inversion recovery  were performed with the GE Signa 1.5T or 3.0T MR scanner . The contrast-enhanced T1WI MR imaging was performed with 0.2 ml/kg meglumine gadopentetate . Scans on the horizontal, coronal and sagittal planes were recorded.CT scanning was performed with the GE LightSpeed RT 16 CT Scanner . Contrast-enhanced CT scans were performed in the arterial phase using non-ionic iodine  and contrast medium , with 1.5 ml/kg being administered intravenously at a rate of 3.0 ml/sec.Written informed consent was obtained from the families of patients. The study was approved by the ethics committee of The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaOut of the 24 cases of synovial sarcoma, nine were located in the spine, consisting of three sarcomas located in the cervical spine and six in the thoracic spine. Three tumors were located in the ankle, three in the knee, three in the subclavian area around the shoulder joint, three in the groin area and three at the upper thigh. All the lesions were deeply located, with nine lesions located near the joints of the extremities. The lesions observed were between 6.2 and 15.0 cm in size. Six patients exhibited signs of recurrence at one to two years post-surgery.The images of nine tumors in the shoulder, groin and upper thigh revealed lobulated masses deep in the intramuscular space, with a density slightly lower than that of muscle. Six lesions were unclearly defined and three were well-defined. The masses were heterogeneous, with six lesions exhibiting punctate or lobular calcification  and six Images of the 18 lesions located on the spine, knee, ankle and subclavian area close to the shoulder joint revealed that 15 lesions were lobulated masses  and 4 anSynovial sarcoma is a mesenchymal spindle cell neoplasm, arising from mesenchymal tissues, with the histological appearance of the synovium. Classic biphasic synovial sarcoma exhibits differentiation of the tumor cells into epithelial cells and fibroblasts, and is categorized into three subtypes, including fibroblastic, epithelial and mixed-differentiation tumors . The curSynovial sarcoma is more common in young adults, but can occur at any age, with half of the lesions occurring in individuals between 20 and 40 years old. Males and females are equally affected. Synovial sarcoma is considered the most common malignant non-rhabdomyosarcomatous soft-tissue sarcoma in children and adolescents –8. The lIn the current study, nine lesions were located on the spine, which is a rarely affected site according to the literature, indicating that synovial sarcoma may easily be misdiagnosed as another type of tumor, if it is not located in the extremities. Additionally, six patients presented with signs of recurrence within one to two years post-surgery, and six other patients presented with metastasis involving the lung and other sites.Based on the present results and those reported in the literature, the imaging features of synovial sarcoma can be summarized as follows: Firstly, the lesions are usually in periarticular locations or deeply located within other sites, with a well-defined, round or lobulated soft-tissue mass. Certain lesions present with poorly-defined margins, with a tendency to grow as a diffuse tumor mass along the tendon, tendon sheath and interstitial space, enveloping neighboring tissues and joints, and leading to osseous destruction of the neighboring bones. In general, the size of the lesions tends to be large, with 85% of tumors being >5 cm in size . In the Secondly, images obtained from CT scans demonstrate areas of isointensity relative to muscle and areas of hypointensity, indicating necrosis and cysts. Areas of calcification are observed in 20–30% of synovial sarcomas , with plet al studied the T2WI results of synovial sarcoma in 34 patients and reported that synovial sarcomas were frequently heterogeneous, with a triple-signal intensity that depicted areas of high signal intensity as fluid, isointensity or hyperintensity relative to fat and hypointensity relative to fibrous tissue  A lobulated or round soft-tissue mass that develops near the joints of extremities, particularly in the lower limbs; ii) the tumor can be well-defined or poorly-defined; iii) the neighboring bones are affected due to infiltration or compression by the tumor; iv) calcification or bone structure can be observed in the periphery of the tumor; v) a high-intensity signal exhibited by the tumor on T1WI, suggesting hemorrhage, and a triple signal demonstrated on T2WI; vi) contrast-enhanced imaging reveals significant heterogeneous enhancement, and areas of cystic necrosis and internal septations can be observed on enhancement; and vii) tumors often occur in young or middle-aged adults. However, due to the rarity of synovial sarcoma in sites other than the extremities, pre-operative diagnosis must rely on other pathological examinations apart from imaging examinations. CT and MRI each have their own advantages in evaluating synovial sarcoma. The combination of the two approaches can improve the accuracy of the pre-operative diagnosis. However, the final diagnosis relies on pathological investigation.


Ewing sarcoma-primitive neuroectodermal tumors (ES/PNETs) constitute a family of neoplasms characterized by a continuum of neuroectodermal differentiations. ES/PNET of the uterus is rare. There are 48 cases of ES/PNET of the uterus published in the literature as far as we know. We describe a case of Ewing sarcoma of the uterus occurring in a 17-year-old woman presenting with a two-month history of pelvic pain. After surgical excision and microscopic, immunohistochemical, and electron microscopy examination, the diagnosis of Ewing sarcoma of the uterus was suggested. This report will discuss the diagnosis and surgical and clinical management of Ewing uterine sarcoma in young women, according to the available literature. In spite of the rarity of ES/PNETs, they should be taken into account in the differential diagnosis of uterine neoplasms in young women. Ewing sarcoma-primitive neuroectodermal tumor (ES/PNET) constitutes a family of neoplasms characterized by a continuum of neuroectodermal differentiations and by translocations involving EWS-FLI1 genes in approximately 85% of all cases. The term Ewing sarcoma has been used for undifferentiated tumors without evidence of neuroectodermal differentiation, as assessed by light microscopy and immunohistochemistry. The term pPNET has been used for tumors with apparent neuroectodermal features .Embryonal rhabdomyosarcoma (20%), Ewing sarcoma/primitive neuroectodermal tumor (17%), angiosarcoma (14%), and pleomorphic rhabdomyosarcoma (13%) appeared to be more common than the other sarcomas, although there was no single overwhelmingly prevalent histotype in the group. Almost all tumors of the Ewing sarcoma/PNET group have some form of EWSR1 (Ewing sarcoma breakpoint region 1) gene rearrangement, which is specific for this group of tumors.Ewing sarcoma rarely occurs in the female genital tract and, until now, only 48 cases have been reported in the literature. Therefore, there is still a lack of accumulated experience with these histotypes due to different diagnostic and therapeutic approach.Those pending problems are mainly important in cases occurring in very young women, when fertility sparing surgery is mandatory, in absence of life threatening conditions. This strategy has not been described until now.A seventeen-year-old nulliparous unmarried patient, referring menarche at 12 years, was admitted to our Institution with a diagnosis of abdominal mass and pelvic pain. Personal history evidenced irregular menstrual cycles and family history of breast and bladder cancer.Transabdominal ultrasound revealed the presence of the right parauterine mass with a complex echogenicity, 12 cm in diameter, deviating the uterus to the left side . Serum tThe abdominal pelvis CT scan evidenced a parauterine right mass approximately 11 cm in diameter with a complex morphology, which contained some large, fluid filled, and hypodense areas, delimitated by dense septa and a pseudocapsule.The right ureter appeared compressed by the mass, resulting in right ipsilateral hydronephrosis. Lymphadenopathies were absent.Vaginoscopy and hysteroscopy excluded any vaginal wall or uterine cavity involvement, while the right fornix appeared convexed, due to overwhelming compression.A preliminary laparoscopy evidenced a 10 cm tumor arising from the anterior wall and right lateral border of the uterus, resembling a subserous fibroma, extending for 2 cm to the cervix and anterior vaginal wall .At laparotomy, a clear cleavage plane allowed a dissection of the uterine mass from underlying uterine myometrium. Anteriorly, the mass was dissected from the bladder and the right ureter, to the vaginal wall of the lateral right fornix.Intraoperatory histological examination was suggestive of mesenchymal neoplasm characterized by leiomuscular structures, with infiltration in the serosal layer by rounded cellular elements with little eosinophilic cytoplasm and dysmorphic large nucleus.At the end of the excision, the lateral margin of the uterus was reconstructed along with the same technique of myomectomy.Final histological examination diagnosed a poorly differentiated malignant neoplasm. The cells were large, with scanty cytoplasm, hyperchromatic nuclei , uncohesImmunostaining revealed a positive reaction for vimentin (+++) and CD99 (+++)  and an epSSG RMS2005 (European Soft Tissue Sarcoma Committee) in the Pediatric Unit and is still PFS at two-year distance.The patient underwent chemotherapy (IVADo) and radiotherapy in the Protocol EFollow-up at the end of the second year revealed a normal PET-CT and ultrasound evaluation without evidence of any abnormality in uterine and ovarian morphology.Primitive neuroectodermal tumors of uterus (PNETs) are extremely rare, mainly in female genital system. Few data are available concerning diagnostic procedures, prognosis, treatment, and follow-up modalities.Ewing's sarcoma-primitive may occur rarely in the ovary, vulva, vagina, cervix, and uterus –5.Until now about 48 uterine cases have been reported, though referred to as different terminology, such as Ewing sarcoma, peripheral PNET (pPNET), PNET (not otherwise specified), and uterine tumors with neuroendocrine differentiation.Therefore, first at all, there is a need for common nosological standardization.Ewing's sarcoma family tumour is a group of small round cell tumors, characterized by the specific translocation t and the specific transcript FLI1/EWS .The two most common entities are classic Ewing's sarcoma and the peripheral primitive neuroectodermal tumour (pPNET): most of the cases arise from the skeleton (and are often characterized by large soft part involvement); however in some cases (less than 20%) these tumors do not involve bone structures, arising from soft tissues.Extraskeletal tumors are most commonly found along the central axis in the paravertebral location, particularly in the chest wall. However, these tumors can arise in any soft tissues and rarely in some organs including kidneys and lungs .In female genital tract, these tumors can be found in vulva, vagina, cervix, and, most commonly, the ovary, while the uterine localization is very rare.Uterine ES-PNET has a bimodal age distribution occurring in postmenopausal age or in adolescence, where fertility sparing surgery could be extremely important.Since most cases (over 75%) of Ewing sarcoma occur in the postmenopausal period , a very Clinically, abnormal vaginal bleeding was the most common presenting complaint. Some patients had an enlarged uterus or a palpable pelvic mass, as in our case.Histologically, most cases of Ewing sarcoma/PNET consisted of uniform small round cells with round nuclei containing fine chromatin, scanty cytoplasm, and indistinct cellular borders. However, some tumors show nuclear atypias or are composed of larger cells with vesicular nuclei and prominent nucleoli. In some cases, Flexner-Wintersteiner and Homer-Wright rosettes can be found and rare tumors show glial or ependymal differentiation .Although well delimited, histological examination of our Ewing sarcoma revealed poorly differentiated malignant neoplasm, with only occasional rosettes.The diagnosis of ES/PNET is based on histopathologic characteristics supported by the immunohistochemical features.The differential diagnosis of Ewing's sarcoma/PNET requires confirmation by ancillary methods including immunohistochemistry and analysis of EWSR1 gene rearrangement.Immunohistochemically, these tumors usually exhibit positivity for CD99, vimentin, and FLI-1 and in some cases focal positivity for cytokeratins .Therefore diagnosis of Ewing sarcoma, pPNET or PNET (NOS), should be based on analysis of EWSR1 gene rearrangement or immunohistochemical analysis of FLI-1 not only on histological findings, associated with a limited immunophenotype usually represented by expression of CD99 .Diagnosis of Ewing sarcoma should be based on positive immunohistochemical reactions for vimentin (+++) and CD99 (+++) and negative reaction for desmin, actin, ML, and CD10, associated as in our experience with a high (90%) proliferative index Ki67.In fact, membranous expression of CD99 is sensitive but not specific for Ewing sarcoma since it can be found in lymphoblastic lymphoma, alveolar rhabdomyosarcoma, synovial sarcoma, some carcinomas (including endometrioid and serous adenocarcinoma), and many others .As clinical point of view, serum markers are not pathognomonic, while fine needle aspiration cytology has been successfully adopted in few cases.Imaging techniques are emerging diagnostic tool, although they have no well-established role, mainly due to the rarity of the disease.Computed tomography imaging reports are not specific for the uterine Ewing sarcoma, as in other uterine sarcomas.In a similar way, ultrasound still does not provide definitive diagnostic imaging. In our case, Ewing sarcoma appeared as a right large dishomogeneous parauterine mass, consisting of various cystic structures filled by hypodense fluid and delimitated by septa, extending downward to the right paravaginal space. These unusual findings, in a very young girl, could be suggestive of botryoid sarcoma, vaginal clear cells carcinoma, and yolk sac tumor. Hysteroscopy and CT may have a role in preoperative staging of advanced cases.Up to date, established surgical approach has been simple or radical total hysterectomy. Moreover, sixteen patients underwent salpingo-oophorectomy and nine patients underwent lymphadenectomy .In all three cases of very young girls, aged less than 18 years, simple or radical hysterectomy has been performed, on the basis of huge extension of the disease.The present case is the first fertility sparing surgery, with tumor exeresis and myometrial reconstruction, in spite of an intraoperative histological frozen section diagnosis of FIGO stage I “mesenchymal tumor,” extending downwards to cervix and expanding in abdominal cavity, without any involvement of nearest structures.However, cases with tumor confined to the uterine corpus had a significantly better result than counterparts and long-term survival has been reported in four cases diagnosed at an early stage, although in all cases total hysterectomy has been performed. Exceptionally, one patient with stage IIIc disease survived 10 years after treatment without evidence of disease recurrence.Undoubtedly, presence of tumor pseudocapsule favoring a bloodless surgical exeresis and normal appearing of surrounding tissues is important for fertility sparing surgery in Ewing sarcoma occurring in a very young patient, mainly at initial stage.Although only a better comprehension of the biology of uterine ES/PNETs could enlighten many aspects of clinical behavior , any aggMoreover, conservative surgical option in early invasive Ewing sarcoma is mandatory in absence of massive local invasion or lymph node metastasis , 7 and iConservative modality treatment is also encouraged by the multimodality therapy which has improved the prognosis of Ewing's sarcoma family tumors, allowing a 5-year survival in 60% of cases.Response to chemotherapy is observed in more than 80% of cases, representing an important prediction factor of outcome.Intensive chemotherapy regimens include alkylating agents (cyclophosphamide or ifosfamide), vincristine, actinomycin-D, and in most cases doxorubicin.Recent studies suggest that doxorubicin, etoposide, and ifosfamide should be added to the classic cyclophosphamide-vincristine-actinomycin regimen; in detail, the North American randomized trial on Ewing's sarcoma and pPNET of the bone, reported by Grier et al. in 2003, described 5-year event-free survival and overall survival of 54% and 61% for patients treated with VACA regimen versus 69% and 72% for patients treated with VACA plus etoposide/ifosfamide, respectively .Other findings would suggest a possible benefit for high-dose chemotherapy (with stem-cell rescue) in localized Ewing's sarcoma with unfavorable features .On the other hand, other data would suggest that the behavior of extraosseous Ewing's sarcoma might be more similar to that of rhabdomyosarcomas: the historical series from the Intergroup Rhabdomyosarcoma Study (IRS) showed satisfying results with VAC regimen and no advantage with the addition of anthracyclines.In conclusion, primary uterine ES-PNET is a rare and aggressive malignancy that requires early diagnosis and multimodal treatment.In the context of the rarity of primary uterine Ewing sarcoma, we report the first case of fertility sparing surgery in an adolescent patient, completed with adjuvant chemotherapy, after comprehensive diagnosis, with a complete progression free survival at two-year distance.In spite of the rarity, ES/PNET should be considered in the differential diagnosis of small cells neoplasms of the uterus.Of course, to clarify the biological behavior of ES/PNETs in uterus, identification of more cases may prove to be beneficial.Whenever possible, fertility sparing surgery is mandatory in young women, if it is possible to restore, after surgery, a normally functioning uterus, able to cope with a future pregnancy.


Splenoma or splenic hamartoma is a rare primary splenic tumor most often discovered radiologically and incidentally. Splenic hamartomas have a strong association with solid and hematological malignancies and, in rare cases, with tuberous sclerosis, but to the best of our knowledge no reports of splenic hamartomas associated with familial adenomatous polyposis have been documented, although it is recognized that familial adenomatous polyposis presents a variety of extracolonic manifestations.We report on a very rare case of multiple splenic hamartomas in a 46-year-old white woman who had previously undergone surgery for restorative proctocolectomy for familial adenomatous polyposis. A computed tomography scan of her spleen revealed multiple small lesions which measured less than 1cm in diameter. A splenectomy was performed and a histologic examination of the splenectomy specimen revealed the presence of multiple hamartomas.APC gene. At this time it is not possible to correlate with certainty our multiple splenic hamartomas and familial adenomatous polyposis case as a clinical manifestation of the mutation of APC gene; however, we believe that this case report could be important for further observation of similar cases in the future.Incidence, differential diagnosis, diagnostic procedures, pathologic findings and treatment of splenic hamartomas are discussed here and hamartomas are considered in a differential diagnosis of splenic tumors. A splenectomy is indicated in cases where malignancy cannot be excluded and in cases of associated hematologic disorders. To the best of our knowledge our patient is the first reported case to have splenic hamartomas identified in a familial adenomatous polyposis-affected patient with mutation in exon 15 of the The online version of this article (doi:10.1186/s13256-015-0627-3) contains supplementary material, which is available to authorized users. Splenic hamartomas (SHs), also known as splenomas, accessory spleens in the spleen, congenital malformation and hyperplastic nodules, are rare benign vascular proliferative tumors with an anomalous mixture of normal splenic elements such as red and white pulp, and are characterized by cluster of differentiation (CD) 8 immunopositivity of the vascular endothelial lining cells –3.Their diagnosis is often incidental at autopsy or when evaluating images for other reasons .Although benign, it is imperative that a differential diagnosis be made between splenic primary and secondary malignancies , 4.Here we report on a rare case of multiple SHs incidentally found in a woman who had previously been operated on for familial adenomatous polyposis (FAP) coli.A 46-year-old white woman was admitted for the presence of small multiple splenic lesions which were incidentally found by a routine abdominal computed tomography (CT) scan. At the scan she was found to have an incisional hernia at the midline incision of the previous colectomy. She was asymptomatic with unremarkable laboratory findings. On physical examination, no palpable mass in her abdomen was found. Upper and lower endoscopy and abdominal ultrasonography (US) did not show primary malignancies, while the CT scan Fig.  revealedAPC) gene in fragment C of exon 15, deletion of an A nucleotide (c.2638delA) was found which created a premature stop codon 105 nucleotides downstream and caused a truncated protein (p.Ile880SerfsX36).She had a 20-year history of FAP of a classic phenotype with profuse colorectal polyposis. She had successively undergone a restorative proctocolectomy (1995), exeresis of cranial osteomas (2003), hysteroannessectomy for a benign ovarian cyst and uterine myomatosis (2007), and has been regularly followed up. She also presented congenital hypertrophy of retinal pigment epithelium (CHRPE) and dental abnormalities. On mutational analysis, a mutation of the adenomatous polyposis coli  . RokitanThe origin of SH is not clear with some hypotheses including congenital malformations of the splenic red pulp, neoplasms of the splenic red pulp, post-traumatic reactive lesions , 8 or acSHs may be associated with solid and hematological malignancies  and in rare cases with tuberous sclerosis, an autosomal dominant disorder characterized by the development of benign tumors (hamartomas) in multiple organs  , 2.APC gene. In addition, bi-allelic mutations in the MYH gene may account for up to 30 % of families with multiple adenomas (15–100) which do not exhibit an autosomal dominant pattern of inheritance nor a germline mutation in the APC gene , SHSTK11)  and manySTK11)  were repPTEN) on chromosome ten [Nor was SH reported among numerous clinical manifestations  of hamarsome ten , 16.APC gene [APC gene was not present and was consistent with the benign nature of the hamartomatous tumor [In our patient a profuse FAP was diagnosed back in 1995 and a restorative proctocolectomy was performed. She presented several ECM: osteoma, dental anomalies, CHRPE, ovarian cysts and uterine myomatosis. At molecular analysis a mutation of the APC gene  was founus tumor .Moreover, all carriers in her family presented a classic FAP phenotype with profuse polyposis of the colorectum and had undergone restorative proctocolectomies in the third decade of their lives. Mutations in exon 15 are common in affected patients with FAP and are associated with profuse polyposis and some ECM.A correlation between the mutation site and clinical phenotype encompassing both colonic and repeatedly extracolonic features has been reported , 20. MutSHs vary in size and sometimes can reach 20cm in diameter. Two histological types exist according to the tumor components : a whiteAs in our case, the majority of patients (80 %) with hamartomas do not have any symptoms and an incidental diagnosis is made during autopsy or an image evaluation . In the The diagnosis of hamartomas can be a challenge as it is difficult to distinguish them from splenic primary malignancies with imaging modalities . CT scanThe main differential diagnosis includes other vascular tumors and solid mass-forming lesions  , 23. OncOnce a splenic lesion is discovered, a malignant tumor cannot be ruled out without performing a splenectomy. A splenectomy may be curative even for hematological disorders associated with hamartomas. Although recently laparoscopic surgery has become the standard technique for splenic disorders, due to our patient’s previous colectomy, we adopted a laparotomic approach due to the presence of several tenacious intraperitoneal adherences.APC gene. We therefore feel that the topic of this report could be relevant for further observation of similar cases in the future.SHs are benign vascular proliferative lesions with a characteristic CD8-positive immunophenotype of the lining endothelial cells. A splenectomy is mandatory to exclude primary or secondary malignancies. To the best of our knowledge, this is the first reported SH case identified in a FAP-affected patient with mutation in exon 15 of the Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Although diaphragmatic injuries caused by blunt or penetrating trauma are rare entities, they are the most commonly misdiagnosed injuries in trauma patients and occur in approximately 3–7% of all abdominal or thoracic traumas. Acute pancreatitis secondary to late presenting diaphragmatic hernia is very rare. Here we present two separate cases: one with acute bowel obstruction and the other with acute pancreatitis secondary to late onset traumatic diaphragmatic hernia . Although diaphragmatic injuries caused by blunt or penetrating trauma are rare entities, they are the most commonly misdiagnosed injuries in trauma patients and occur in approximately 3–7% of all abdominal or thoracic traumas . When thHere we present two separate cases: one with acute bowel obstruction and the other with acute pancreatitis secondary to late onset TDH .Case 1. A 33-year-old male patient presented with symptoms of abdominal pain, vomiting, and obstipation to our emergency surgery department.3/μL). Abdominal plain X-ray showed elevation of left diaphragm and infiltration in the left lung. This leaded to a computed tomography (CT) of chest which demonstrated herniation of small bowel, transverse, and descending colonic segments into left hemithorax through a 4 cm defect on diaphragm.His vital signs at admission were normal besides the pulse rate of 110/min. His physical examination revealed decreased breathing sounds and a healed scar on the left hemithorax. Patient had a history of penetrating thoracic trauma through the intersection of left anterior axillary line and costal arch, three years ago. The patient rejected any surgical intervention at that time and left the emergency clinic. In his current presentation initial evaluation was quite helpful in identification of the source of ileus. Laboratory levels were within normal ranges besides elevated white cell count of 18.3  abnormal traction on the pancreas, total incarceration of the gland in hernia without pancreatic volvulus , (2) migOperative intervention is the main management for delayed traumatic diaphragmatic hernias, especially if there is an acute complication like strangulation as in our first case. Although the thoracic approach has been recommended for chronic diaphragmatic hernias due to dense intrathoracic adhesions , abdominThese two cases point out that the diagnosis of TDH can be overlooked at the initial trauma evaluation, thus stressing out the need to actively examine the diaphragm on all trauma laparotomies. The clinicians need to be vigilant, to maintain a high index of suspicion for the diaphragmatic injuries, whether they are immediately after the trauma or even decades after the trauma. Our case seems to be the second case in medical literature depicting acute pancreatitis after TDH.


To investigate the clinicopathological features of six cases of soft tissue recurrent ameloblastoma and explore the role of increased aggressive biological behavior in the recurrences and treatment of this type of ameloblastomas. In this study, we retrospectively reviewed recurrent ameloblastomas during a 15-year period; six cases were diagnosed as soft tissue recurrent ameloblastoma. The clinical, radiographic, cytological and immunohistochemical records of these six cases were investigated and analyzed.All the six soft tissue recurrent ameloblastomas occurred after radical bone resection, and were located in the adjacent soft tissues around the osteotomy regions. In Case 4, the patient developed pulmonary metastasis, extensive skull-base infiltration and cytological malignancy after multiple recurrences and malignant transformation was diagnosed. In the other five cases, although there were no cytological signs are sufficient to justify an ameloblastoma as malignant, some malignant features were observed. In Case 1, the tumor showed moderate atypical hyperplasia and the Ki-67 staining percentage was 40% positive, which are strongly suggestive of potential malignance. In Case 5, the patient developed a second soft tissue recurrence in the parapharyngeal region and later died of tumor-related complications. All the remaining three patients showed cytology atypia of varying degrees and high expression of PCNA or Ki-67, which confirmed active cell proliferation.Increased aggressiveness is an important factor of soft tissue recurrence. An intraoperative rapid pathological examination and more radical treatment are suggested for these cases.Key words:Ameloblastoma, soft tissue recurrence, aggressive biological behaviour. The soft tissue recurrent ameloblastomas which occurred after radical bone resection and located in the adjacent soft tissues and without involvement of the residual jaw, showed clincopathologcial features which were not quite consistent with intraossous recurrent ameloblastomas. The role of increased aggressive biological behavior in the recurrences and treatment are discussed in this paper.We retrospectively reviewed the clinical data of ameloblastoma patients admitted to our hospital who underwent resection and had histopathologically confirmed recurrent ameloblastoma during 1998-2013. Of the 25 recurrent ameloblastomas, six cases were diagnosed as soft tissue recurrent ameloblastomas based on their clinical, radiographic and histological presentations. Of these six cases, three cases had multiple recurrences: Case 4 showed six recurrences during 1998-2013; cases 5, two recurrences during the period of study, and Case 6, had recurrences in 1992 and 1996 respectively. The following clinical data of the six recurrent cases were recorded for the six soft tissue recurrence cases: sex, age at presentation of recurrence, site of recurrence, time interval between primary operation and recurrence, number of recurrences, types of surgery and follow-up reviews.Histopathological sections of the primary and recurrent lesions were collected and evaluated for the histopathologic type and presence of cytology atypia. The histopathologic classifications included follicular, plexiform, acanthomatous, granular, basal cell type, and keratoameloblastoma ,12. The Immunohistochemical examination for the quantitative expression of Ki-67 or semi-quantitative expression  of antigen proliferating cell nuclear antigen (PCNA) was conducted in all the six recurrent cases ,15.All patients gave their written informed consent.This study was approved by the Ethics Committee of the Affiliated Stomatology Hospital of Medical School, Nanjing University.The clinical and pathological data of the six intraosseous ameloblastomas with soft tissue recurrence are shown in - Basal clinical data for recurrencesFour men and two women with soft tissue recurrent ameloblastoma were investigated in this study. The mean age at presentation of recurrence was 52.5 years . The average time interval between the primary operation and recurrence was 22.3 years . With the exception of Case 4, the time interval between the primary operation and the recurrences was ≥10 years in the remaining five cases.- Type of primary surgery In Case 4, enucleation and curettage were performed for the primary maxillary ameloblastoma; further, radical bone resection  was performed for the first recurrence 2 years later. In the other five cases, radical bone resection was performed for all the primary lesions: hemimandibulectomy in three cases, segmental resection in one case and resection of the lesion with the dentoalveolar segment and preservation of the lower border in one case. In two cases, osseous reconstruction was performed for the defects.- Location of the recurrent tumorsIn three cases, the recurrent tumors were located in the submandibular region on the same side of the primary lesion; in one case, it was located in the soft tissue of the osteotomy region; and in two cases, the tumors recurred in the gingival tissue of the molar regions.- Follow-up reviewIn all six cases, extensive tumor excision was performed for the recurrent tumors to prevent further recurrences. The follow-up time was 2-10 years, and in four cases, there were no additional recurrences after that. However, in Case 4, the patient developed pulmonary metastasis and extensive skull-base infiltration after multiple recurrences and died in 2013; in Case 5, the patient developed a second soft tissue recurrence in the parapharyngeal region, but he refused further treatment and died not long after that due to complications caused by the tumor.- Radiographic featuresIn all the six cases, all the primary lesions showed extensive bone destruction involving the cortical plate. With regard to the soft tissue recurrences, all the bone margins were totally intact and without signs of bone recurrence.- Histopathological featuresOf the six cases, four were of the follicular type; one was plexiform type and one, basal cell type. All the six patients presented with cytology atypia of varying degrees for both the primary lesions and recurrent lesions . In Case- Case Reports - Case 1The patient was 22 years old at the time of the initial diagnosis of ameloblastoma in 1994. Resection of the lesion with dentoalveolar segment and preservation of the lower border was performed. Eighteen years later, the patient developed an intraoral swelling in the right mandibular body. CT revealed a soft tissue mass in the osteotomy region with an intact bone defect Fig. . Thereaf- Case 2The female patient was 30 years old at the time of the initial diagnosis of ameloblastoma, and hemimandibulectomy was performed in 1979. Thirty years later, the patient developed a swelling in the submandibular region. CT revealed a soft tissue mass with a totally intact bone defect. Extensive resection of the tumor was performed, and the histological diagnosis was recurrent ameloblastoma. The Ki-67 staining percentage was 32% positive for the recurrent ameloblastoma. Retrospective evaluation of the histopathological sections of both the primary and recurrent lesion showed plexiform ameloblastoma with cytology atypia including hyper cellularity, hyper chromatic nuclei and mitotic figures.- Case 3The patient was 30 years old at the time of the initial diagnosis of ameloblastoma in 1973. In 1992 and 2006, the patient had soft tissue recurrences located in the buccal mucosa and mandibular retromolar pad respectively. Extensive resections were performed for the recurrent tumors and the patient has been followed up with no evidence of recurrence. The histopathological classification was follicular ameloblastoma and cytology atypia was found for the primary lesions and two times of recurrent lesions.- Case 4 The patient was referred to our hospital and underwent total enucleation and curettage for maxillary ameloblastoma in 1999 Fig. . Histolo- Case 5The patient was 33 years old at the time of the initial diagnosis in 1982 and hemimandibulectomy was performed for the primary lesion Fig. . Twenty-- Case 6The patient was 36 years old at the time of the initial diagnosis of ameloblastoma in 1989; hemimandibulectomy and osseous reconstruction were performed. Twelve years later, the patient developed a soft tissue recurrence in the mandibular third molar region. The final histological classification was recurrent follicular ameloblastoma. Similar with the other cases, retrospective cytological evaluation of the histopathological sections for the primary and recurrent lesion showed cytology atypia including hyper cellularity and hyper chromatic nuclei.According to the present classification of ameloblastoma, the benign ameloblastomas are divided into 1) solid/multi cystic, 2) extra-osseous/peripheral, 3) desmoplastic, and 4) unicystic ameloblastoma. The solid/multicystic ameloblastomas are more aggressive than other types because they carry a high risk of local recurrences after inadequate treatment . HoweverAmeloblastoma recurrence has been suggested to be correlated to both the histological features and the type of surgical treatment . It is kConsidering the highly aggressive nature of these atypical ameloblastomas, firstly, a preoperative radiographic evaluation of whether the tumor involves the cortical plate is required, as destruction of the cortical plate suggests possible infiltration into the adjacent soft tissues. Secondly, a frozen rapid pathological examination during the surgery is suggested for these ameloblastomas cases; if cytological atypia indicative of aggressiveness is observed, more radical treatment including clearance of at least one more layer of tissue around such lesions should be performed . MoreoveIt has also been reported that 50–80% of ameloblastoma recurrences are diagnosed within the first 5 years -4. HowevIn this clinical retrospective study of recurrent ameloblastomas during a 15-year database (1998-2013), it was noted that soft tissue recurrence following radical bone resection also showed some malignant features. An intraoperative rapid pathological examination and more radical treatment are suggested for these cases of aggressive tumors.