Desmoplastic ameloblastoma (DA) is a rare variant that accounts for approximately 4–13 % of ameloblastoma, displaying significant differences in anatomical site, imaging, and histologic appearance. It has been included in WHO classification of head and neck tumor (WHO-2005) as a variant of ameloblastoma. The tumor resembles benign fibro-osseous lesion for being frequently occurring in the anterior region of jaws as a mixed radiopaque-radiolucent lesion.We present a case of DA in a 43-year-old female with a painless swelling in the anterior region of mandible. No fluid was evident on fine needle aspiration. A mixed lesion with multilocular appearance was evident on both panoramic radiographs as well as computed tomography scan. An incisional biopsy confirmed it to be a case of desmoplastic ameloblastoma. Segmental mandibulectomy was performed from teeth 35 to 44. The patient is on routine follow-up and is currently free of ailment.The present case deserves emphasis because of its unfamiliar appearance, potentially aggressive nature and deceptive radiologic appearance maximizing the chances of misdiagnosis. So, the clinician should be alert enough to include desmoplastic ameloblastoma in differential diagnosis of any lesion/growth with mixed radiolucent-radiopaque appearance having ill-defined borders and occurring in anterior maxilla or mandible. Ameloblastoma is the second most common tumor, only next to odontoma, has its origin from the odontogenic epithelium. The tumor is considered benign despite of its locally invasive nature. The follicular and plexiform varieties of ameloblastoma are most common, followed by the acanthomatous and granular cell types. Less frequent cellular variants of ameloblastoma are desmoplastic ameloblastoma, clear cell ameloblastoma, basal cell ameloblastoma, keratoameloblastoma, and unicystic ameloblastoma , 2.Ameloblastomas of 0.9–12.1 % have been reported to be desmoplastic ameloblastomas (DA). Desmoplastic ameloblastoma differs from other variants of ameloblastoma in that it is more frequently seen in the anterior region of jaw and its mixed radiolucent radio-opaque appearance is often more representative of a fibro-osseous lesion . HistoloThe true biologic profile of DA is still not well understood due to paucity of adequate samples.Sun et al. presented retrospective analysis evaluating clinicoradiographic features of 115 cases of DA that were reported in literature from 1984 to 2008 [This report is an attempt to help the dental community in developing familiarity with the clinical presentation of desmoplastic ameloblastoma and at the same time emphasizing an index of suspicion in recognizing and treating the peculiar aspect of this unusual lesion.A 43 years old female of Han ethnic group from Northeast China, farmer by occupation, presented to the Department of Oral and Maxillofacial Surgery, Hospital of Stomatology, Jilin University, Changchun, China with an asymptomatic swelling in her anterior mandible. The swelling had started 1 year previously and since then, there had been a gradual increase to its present size. She denied experiencing any bleeding, pain or sensory changes. She also denied any history of trauma and the past medical, dental and family history was insignificant.On physical examination, facial asymmetry due to swelling on the left side of the face was noticed. The swelling was oval in shape crossing the midline thereby obliterating the labiomental sulcus.The swelling had smooth surface with normal overlying skin but stretched. It was non-tender on palpation.The intra-oral examination revealed a large mass approximately 5 × 4 cm in size, extending from lower right canine to left 2nd premolar buccally. Buccal expansion of the mandibular left and right symphyseal and para-symphyseal region was evident. The overlying mucosa appeared normal. There was labial displacement of 32 Fig. .Fig. 1InOn palpation, the swelling was found to be firm, bony hard in consistency, non-tender, non-fluctuant, irreducible, non-compressible and non-pulsatile. The teeth in the vicinity were non-tender to percussion; there was slight mobility of 32 and 33. On electric pulp vitality testing, all teeth in the affected area were vital except 32 and 33. No lymphadenopathy or fistulae were present.Radiographic examination of the mandible revealed a diffuse ill-defined mixed radiolucent radio-opaque lesion extending from mesial surface of lower right canine to the mesial surface of the lower left 2nd premolar with an approximate size of 5 × 4 cm Fig. . Areas oA provisional diagnosis of a fibro-osseous lesion of the anterior mandible was made based on clinical and radiographic appearance. The lesion was non-productive on aspiration. Blood profile showed no abnormality except slightly raised levels of serum alkaline phosphatase, 148 IU/L, suggestive of a bone forming lesion. The final diagnosis was established through incisional biopsy performed under local anesthesia. The histologic features were corroborating with those of desmoplastic ameloblastoma.A segmental resection of mandible from 44 to 35 was done under general anesthesia with proposed incision as shown in Figs. In 1984, Eversole  pioneereThe incidence of desmoplastic ameloblastoma is low. Although DAs are similar to conventional solid ameloblastomas regarding the age and gender distribution, tumors present a strong proclivity for the anterior region with equal incidences on either of the jaw . DA occuThe majority of the cases has been reported particularly in Chinese residing in Malaysia and Hong Kong, Malaysians, Japanese and Afro-Caribbeans . ReportsAccording to Philipsen et al., desmoplastic ameloblastomas originating in the maxilla possess more aggressive behavior than those in the mandible. The insidiousness of maxillary lesions to mandibular tumors are attributed to proximity to vital structures as well as the very thin cortical bone of the maxilla being a weak barrier favours the dissemination of tumors. Therefore, maxillary ameloblastomas have potential to spread earlier and more rapidly than mandibular neoplasms.The extensive presentation of the desmoplastic ameloblastoma may be due to (1) relatively higher incidence in the maxilla leading to an early encroachment of adjacent vital structures (2) the ill-defined diffuse radiographic appearance.Patient usually presents with a chief complain of painless swelling of the jaw bone. The tumor varies in size between 1.0 and 8.5 cm in diameter . Tooth dSo far as the origin of DA is concerned, Kishino et al.  assumed Radiographically, about 50 % of DA show a mottled, mixed radiolucency/radiopacity with ill-defined margins, making it difficult to differentiate from a fibro-osseous lesion. It was hypothesized that this may be due to the infiltrative nature of DA to involve the trabeculae. Three radiological presentations of DA are mentioned in the literature as follows: type I (osteofibrosis type) which has radiolucent as well as radiopaque appearance; type II (radiolucent type) which has a completely radiolucent appearance; and type III (compound type) which has radiolucent as well as radiopaque appearance combined with a large radiolucent change . RadiogrThe final diagnosis of desmoplastic ameloblastoma is based on histopathological evaluation of biopsy specimens. The usual microscopic features are: (1) extensive stromal desmoplasia with abundance of collagen and moderate amount of cellular connective tissue, which is the most consistent and distinguishing feature Fig. ; (2) islHistologically, the possibility of misdiagnosis of DA as another odontogenic tumor is high if biopsy specimen is not sufficient enough to warrant the presence of characteristic palisading layer of ameloblastoma in all the epithelial clusters. DA may resemble odontogenic fibroma if presence of only narrow strands of epithelial cells are evident within desmoplastic stroma. The epithelium-poor type of central odontogenic fibroma (COF) is a non-infiltrating lesion of connective tissue that mimics a dental follicle. It contains little cellular component with scattered delicate collagen fibres. The presence of significant quantity of ground substance gives a fibromyxoid appearance to the background in contrast to myxoid changes in the juxtaepithelial stroma in desmoplastic ameloblastoma. Scattered remnants of inactive-looking odontogenic epithelium appear as small irregular islands and cords. Occasionally, variably formed calcifications occur. The epithelium rich type of odontogenic fibroma is composed of cellular, fibroblastic connective tissue that are intermingled with less cellular but vascularized areas. Foci of calcified collageneous matrix mimicking dysplastic cementum, dentin or osteoid are often present. Islands or strands of inactive-looking odontogenic epithelium forms an essential component which may be sparse but are often obvious in contrast to stromal components compressing odontogenic epithelium making epithelial tumor islands very irregular or bizarre with pointed stellate appearance in case of desmoplastic ameloblastoma. The correct diagnosis of these two tumors signifies the clinical behavior and approaches for their management. Ameloblastoma being potentially aggressive tumor requires en bloc resection whereas odontogenic fibroma being much less aggressive requires enucleation.Squamous odontogenic tumor also comes under differential diagnosis of DA. DA resembles squamous odontogenic tumor if metaplasia of squamous epithelium is evident in some areas but palisading layer of tall columnar cells are not visualized . The islImmunohistochemical studies have suggested that the desmoplasia might be a result of overexpression of transforming growth factor beta (TGF-β), a potent local factor that modulates the formation of extracellular matrix. This is attributed to a new protein synthesized from the extracellular matrix that controls cell growth, proliferation, differentiation and apoptosis. Moreover, it has also been proposed that this again synthesis of extracellular matrix proteins involved in the support, adhesion, proliferation, migration and differentiation of tumoral cells might be related to the phenomenon of desmoplasia .Various immunohistochemical studies have supported the fact that DA tumor cells show variable expression of S-100 protein and desmin. There may be increased expression of caspase-3 and Fas(cell surface receptor protein of tumor necrosis factor receptor family), increased expression of p63, decreased expression of cytokeratin19 .Desmoplastic ameloblastoma exhibited desmoplasia of the stromal connective tissue which is thought to be a maturational change in a solid ameloblastoma, as it has been observed that dense collagenisation may be seen in tumors with a long history. This logic can be applied to cases of hybrid tumors. A combination of DA with any other histological type of multicystic ameloblastoma is called a hybrid ameloblastoma. So, it may be hypothesized that a hybrid tumor may be a transitional phase in the maturation of a solid multicystic ameloblastoma to the desmoplastic variety .The biologic behaviour of DA has been depicted in the WHO classification of odontogenic tumors which states DA to have a lower recurrence rate similar to unicystic ameloblastoma and peripheral ameloblastomas. Philipsen et al. reported a recurrence rate of peripheral ameloblastoma with conservative treatment to be 16–20 % . A metanAs the tumor is without capsule, the cells infiltrate between the trabeculae of the cancellous bone leaving them intact for some time. Thus, the tumor actually penetrates beyond the radiographic margin. It could be the possible reasoning for the inconspicuous radiographic margins and the significant recurrence rate following curettage. An analysis of 34 mandibular ameloblastomas by Marx and others reported data showed that the tumor extended 2.3–8.0 mm beyond the radiographic margin . TherefoIn view of the paucity of such case series and limited understanding of its biological behavior and prognosis, proper treatment strategies are not completely defined so far. Hence, such cases need to be identified and reported. It is still an enigma whether the recurrence is due to the nature of the tumor (lack of capsule and precise limit) or due to the incomplete surgery. Therefore, resection is the most commonly accepted treatment to prevent recurrence . In our The desmoplastic ameloblastoma is characterized by distinctive clinicoradiographic and histologic features. The clinician must be vigilant regarding the rare presentation of this benign tumor and DA should be included in differential diagnosis of any lesion as simple as abscess to any fibro-osseous lesions or any mass/growth occurring in anterior region of either jaw. Moreover, the horizon of differential diagnosis of Desmoplastic ameloblastoma also extends over any mixed radiolucent–radiopaque lesion with inconspicuous radiographic margin presenting in the anterior–premolar region of the maxilla/mandible. There are still ongoing debates regarding the true biologic behavior of the lesion due to paucity of adequate samples. The radiological and histological findings of poor encapsulation and ill-defined borders suggestive of infiltrative nature warrants in depth analysis and a long-term follow-up. With potential for recurrences, a complete resection to its treatment is recommended alike conventional ameloblastoma.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


A cardiac calcified amorphous tumor (CAT) localized to the mitral valve leaflet without mitral annular calcification (MAC) is a rare entity. We report a case of a 69-year-old woman with such a condition, who underwent successful excision of the tumor and mitral valvuloplasty using a glutaraldehyde-treated autologous pericardium. During 38 months of follow-up, no recurrence of a cardiac mass has been recognized. This report addresses questions on the surgical indication for CAT, particularly in cases without MAC, and reviews CATs of the mitral valve. A cardiac calcified amorphous tumor (CAT) is a non-neoplastic cardiac mass that includes nodular calcium deposits, chronic inflammatory cells, hyalinization, and degenerating blood elements . Most caWe discuss a surgical indication for CAT on the mitral valve with specific consideration for the presence and absence of MAC after reviewing the 18 previously reported cases.The patient was a 69-year-old woman whose abnormality was initially identified by chest radiography at a local hospital in 2008. At that time, transthoracic echocardiography revealed a calcified and immobile mass on the posterior mitral valve leaflet. She was referred to our hospital for evaluation of the mass. Her past history was significant with pulmonary tuberculosis at the age of 6 years and systemic hypertension. However, there was no history of infectious endocarditis or chest trauma. Any thrombus formation due to a hypercoagulable state was not recognized. Differential diagnoses included calcified cardiac fibroelastoma and myxoma. We offered her a surgical treatment option because of the potential risk of a thromboembolic event. However, she refused the surgical intervention. She was managed on an outpatient basis and periodically underwent transthoracic echocardiographic evaluations every 6 months.The patient claimed to have chest discomfort in March 2011 and was admitted to our hospital in October 2011. A transthoracic echocardiogram showed that the calcified mass measuring 19 × 8 mm was located between the P2 and P3 areas of the posterior mitral valve leaflet. There was no mitral regurgitation, and her ejection fraction was 67%. There were no findings suggestive of infectious endocarditis (Figure Because there was potential risk of thromboembolism, surgical intervention was indicated for the calcified mass or tumor on the mitral valve. Definitive diagnosis was also expected after surgical excision of the mass.The patient underwent surgical removal of the mass with the aid of cardiopulmonary bypass. The mitral valve was inspected through a right-sided left atrial incision. A round mass was originating from the P2 area and measured 8 mm in diameter. The calcified process was localized to the posterior leaflet itself. The entire mass was excised and submitted for histopathological examination. The defect on the posterior leaflet of the mitral valve was patched with a glutaraldehyde-treated autologous pericardium.No regurgitation was detected after the procedure. Weaning from the cardiopulmonary bypass was uneventful.Grossly, the mass was white and measured 9 × 5 × 2 mm. Histological examination revealed nodular calcification in the fibrous connective tissue with dense fibrin and mixed inflammatory infiltration, predominantly with plasma cells and lymphocytes. There were no identifiable myxoma cells or malignant cells. Consequently, a definitive diagnosis of cardiac CAT was made Figure .Figure 3The patient recovered uneventfully and was discharged without any adverse events. No recurrent cardiac mass has been detected at 38 months after the surgery.Forty-four cases of cardiac CAT have been reported previously. As a significant feature, CATs were originating from the mitral valve in 18 of these cases (Table Our literature review revealed that most patients with MAC-related CATs are on hemodialysis. It is therefore possible to preoperatively determine whether a calcified intracardiac mass of the mitral valve is CAT by the presence of extensive MAC in end-stage renal disease patients. Our patient did not have end-stage renal disease, and there was no history of endocarditis or thrombus formation. It was impossible to distinguish a calcified intracardiac mass of the mitral valve from calcified cardiac myxoma, fibroma, fibroelastoma, and caseous MAC ,17 by diOperative and histological findings of the small portion of the posterior leaflet was quite normal and negative for any sign of previous injury, formation of thrombus, presence of fungus body, and caseous necrosis. The mechanism which the CAT without MAC arose was not defined. Since the lesion was not diffuse but rather discrete, we may speculate that its etiology is not inherent. Based on these preoperative diagnostic findings, we could not exclude a risk of thrombus formation and caseous necrosis occurring during the course of this pathology. Hence, certain level of risk of thromboembolism was not neglected prior to surgical intervention. Regardless of the benign or malignant nature of a cardiac mass, an excision of the lesion is important to avoid the potential risk of embolization ,8-10,18 In terms of the size, we are unable to define the size criteria toward surgical intervention for calcified tumor on the mitral valve. Although it has been known that a risk of thromboembolism increases when the size of vegetation exceeds 10 mm, this size criteria should apply to the case with vegetation associated with infective endocarditis. We presume that it is not easy to decide surgical indication for CAT by the size of the mass. We need to accumulate more number of cases with cardiac mass of suspected CAT.CAT localized to the mitral valve leaflet without MAC in our case was unique, and its etiology has not been determined. In general, CAT is pathologically diagnosed after surgical excision. The prognosis of a patient with a cardiac mass is unknown unless surgical excision is performed. This patient had chest discomfort. However, she had no signs of heart failure or arrhythmia. We offered her a surgical treatment option on an occasion of her first visit considering certain risk of thromboembolism and for the purpose of diagnosis. However, she declined a surgical intervention. She was managed on an outpatient basis. She started to worry about her future health condition especially after the Great East Japan Earthquake. Although the reason for her chest discomfort was not clear, it certainly enhanced her treatment which might help an avoidance of stoke or other thromboembolic event.When a calcified tumor localized to the mitral valve leaflet with MAC is immobile, it can be conservatively observed as CAT. In contrast, as observed in our case, if a calcified mitral tumor without MAC cannot be distinguished from a calcified cardiac myxoma, fibroma, fibroelastoma, and caseous MAC by conventional diagnostic imaging, we should consider surgical intervention. Before deciding a surgical indication for the calcified tumors localized to the mitral valve leaflet, the specific determination of the presence or absence of MAC help to define the surgical indication.We reported a rare case of CAT localized to the mitral valve leaflet without MAC. A literature review revealed that the presence of MAC is suggestive of CAT on the mitral valve. However, even with the absence of MAC, CAT remains a potential diagnosis, and surgical intervention is still considered.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.


Chordomas are low- to intermediate-grade malignant tumors that recapitulate the notochord. Chordomas belong to the dysontogenetic bone tumors and appear primarily in the region of the axial skeleton. Chordomas are divided into conventional, chondroid, sarcomatoid and dedifferentiated subtypes. The different subtypes of chordoma have varied survival periods. According to the literature to date, secondary pulmonary and lymph-node metastases occur most frequently, followed by liver, bone and skin metastases. To the best of our knowledge, there has been no previous report of one subtype of chordoma metastasizing or transforming into another subtype with a different histopathology. This study presents a 24-year-old man with secondary pulmonary conventional chordoma arising from a primary sarcomatoid chordoma of the sacrum. The patient was alive at the end of November, 2009 and the survival time exceeded eight years. This is the first case of a patient with primary sarcomatoid chordoma of the sacrum with complete remission in whom a secondary pulmonary conventional chordoma arose from the primary cancer. Chordomas are low- to intermediate-grade malignant tumors that recapitulate the notochord. Etiologically, chordomas belong to the dysontogenetic bone tumors and occur predominantly in the region of the clivus . CorrespThe histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, occasionally described as physaliferous (having bubbles or vacuoles) .Although chordomas are locally invasive, metastasis has been reported in 10–42% of cases. However, these may be the result of the misdiagnosis of mucus-producing carcinomas of the rectum as chordomas in certain case studies . PreviouAccording to the current literature, no case of a chordoma subtype that has metastasized or transformed into another subtype with a different histopathology and immunoreactivity has been previously reported. This study presents a case of secondary pulmonary conventional chordoma arising from a primary sarcomatoid chordoma of the sacrum.A 24-year-old man was referred to the Tri-Service General Hospital  presenting with a palpable sacral mass and constipation of two months in duration. A magnetic resonance imaging (MRI) examination of the sacral spine revealed a large mass (measuring 13×8×7 cm) with compression of the colon . Based oAfter one year, the patient presented to the Tri-Service General Hospital with dizziness and unstable gait of one week in duration. Computed tomography (CT) of the brain revealed a mass lesion over the right cerebellar vermis. A suboccipital craniotomy was performed, with removal of the mass and the placement of a ventriculoperitoneal shunt. The pathological findings identified a sarcomatoid-type metastatic chordoma. Immunohistochemical staining was positive for CK, EMA, vimentin and S100. Intracranial whole-brain radiotherapy and adjuvant chemotherapy with methotrexate were administered to the patient.After two years, a CT examination of the lungs was performed due to a chronic cough, revealing multiple lesions, enlarged lymph nodes and suspected metastases over the right lower lobe . A thoraIn the present case, the patient suffered from two different subtypes of chordoma. The first was a sarcomatoid chordoma that responded completely to an exploratory laparotomy with debulking of the tumor and postoperative radiotherapy, with no evidence of residual disease. The secondary chordoma was the conventional subtype. To the best of our knowledge, this study is the first to present the case of a different subtype of chordoma arising at a metastatic site.The majority of patients with conventional-type chordoma are 50–70 years old and 50% of these tumors arise in the sacrococcygeal region . The patIn the dedifferentiated type of chordoma, the pathological findings show a sharp demarcation between the conventional chordoma and the high-grade sarcoma, with no transitional characteristics between the two components, and negative CK immunoreactivity in the sarcoma component –16.The primary subtype of the chordoma affects the survival period and the progressive characteristics of the tumor, including local relapses, surgical pathway seeding and progressive distal metastasis  . TherefoStandard treatments for conventional chordoma are maximal surgical resection and subsequent radiotherapy (50 Gy), even proton-beam therapy. These treatments may improve morbidity and mortality . However


Osseous choristoma is a very rare, benign lesion in the maxillofacial region. It appears as a benign mass of normally matured bony tissue covered by the normal epithelium of the tongue. It is usually seen in front of the foramen cecum of the tongue. Surgical excision is the treatment of choice with an excellent prognosis and there have been very few cases of recurrence.Here we present two cases of osseous choristoma on the dorsum of the tongue. Case 1 was a 15-year-old Japanese girl who presented with a painless but gradually growing swelling on the dorsum of her tongue approximately 1 year before her admission. Case 2 was a 21-year-old Japanese woman with a complaint of pain in the lower left, posterior side of her mouth. Histological findings showed that both lesions were composed of well-organized, mature, compact bone beneath the oral mucosal membrane. Subsequent to simple surgical excision, no recurrence of the lesions was observed after the follow-up period. Previous literatures have proposed both malformation and trauma hypotheses as the etiopathologies of osseous choristoma. However, the histopathological findings of the two cases in the present study do not support the trauma hypothesis.Although osseous choristoma is clinically a benign condition, the underlying histopathological processes are important. The outcome of aberrant formation of calcified tissue in the vicinity of vital structures such as nerves and blood vessels may be of clinical significance. Osseous lesion of the tongue is a rare entity and was originally reported as a lingual osteoma . Since tA 15-year-old Japanese girl was referred to our out-patient department (OPD) with a chief complaint of discomfort in her throat. She had noticed a painless but gradually growing swelling on the dorsum of her tongue approximately 1 year before her admission. Her past medical history revealed that she had taken iron-containing medications for a short period of time; however, there was no documentation of her medical condition. An intra-oral clinical examination revealed the presence of a pedunculated well-circumscribed swelling, measuring 5 mm, on the left side of the posterior dorsum of her tongue, near the foramen cecum. It was covered by healthy looking oral mucosa Fig. , and felA 21-year-old Japanese woman visited our OPD with a chief complaint of pain in the lower left, posterior side of her mouth. An intra-oral clinical examination revealed the presence of partially impacted, lower third molars bilaterally, both of which required surgical extraction. She did not have any other abnormal, medical or surgical history. Further clinical examination revealed the presence of a pedunculated well-circumscribed swelling, measuring 5 mm on the right side of the posterior dorsum of her tongue, near the foramen cecum. The lesion was lined by a thin normal-looking mucosa, and was bony hard on palpation. The lesion was symptomless and accidentally discovered during her oral cavity examination. Her chief complaint was addressed first, and the tongue lesion was planned for surgical excision at a later period. An excisional biopsy of the lesion was performed under local anesthesia, and the tissue was immediately placed in 10 % formalin for fixation. She was reviewed on the second and seventh day during which her healing was found to be uneventful. She was kept under observation and recalled 1 month, 6 months, and 2 years after surgery, and was found to remain free of the disease at the end of the follow-up period.Histopathology showed that the lesion was composed of well-organized, mature, compact bone with lamellar structures beneath the mucosal membrane, and was lined by parakeratinized, stratified, squamous epithelium Fig. .Fig. 2HiOsseous choristoma of the tongue is extremely rare with only 67 cases reported in the English literature so far . Here weThe etiopathology of osseous choristoma is still unknown. Two different hypotheses have been proposed; whereas one suggests that this is a type of malformation, the other concludes that it is formed as a result of trauma or chronic irritation . AccordiThe differential diagnosis of a protruding mass in the tongue can also be based on its location. When the lesion is on the dorsum of the tongue near the foramen caecum, it can be considered a benign tumor such as hemangioma, lymphangioma, teratoma, hamartoma, leiomyoma, thyroglossal duct cyst, lingual thyroid, mucocele, and pyogenic granuloma, or a malignant tumor such as rhabdomyosarcoma and epidermoid carcinoma. Lesions found on the margins of the tongue may be diagnosed as a traumatic neuroma, neurofibroma, schwannoma, fibroma, or cartilaginous choristoma. Pyogenic granulomas, mucoceles and cartilaginous choristomas have a greater predilection for the anterior region of the tongue. Other differential diagnoses include vascular malformation, fibroma, papilloma, salivary gland mixed tumor, osteochondroma, and other neoplasms such as osteogenic sarcoma or squamous cell carcinoma . HamartoSurgical resection is the most common treatment for osseous choristomas , 5, althThis study presents two rare cases of osseous choristoma in the posterior part of the dorsum of the tongue, and adds to the existing knowledge of the condition.Written informed consent was obtained from patient 1’s legal guardian and written informed consent was obtained from patient 2 for the publication of this case report and the accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


Testicular germ cell tumors (TGCTs) are the most common malignancy in males aged 20 to 39, and the incidence is increasing. TGCTs have a tendency to grow rapidly with a high risk of metastatic spread. TGCTs generally present with a palpable testicular mass, yet may present less commonly with symptoms arising from metastatic disease. A 24-year-old otherwise healthy male presented with progressive headaches. Initial imaging reported a single mass in the right frontal lobe. Complete surgical resection revealed suspicion for metastatic poorly differentiated carcinoma with an inconclusive immunohistochemical profile. Further staging scans revealed pulmonary and pelvic tumor deposits. Tumor markers with alpha-fetoprotein, beta-human chorionic gonadotropin, and lactate dehydrogenase were not elevated. Follow-up cranial magnetic resonance imaging revealed intracranial disease progression and he underwent whole brain radiation therapy. Additional outside pathology consultation for chromosomal analysis revealed features consistent with a TGCT. A scrotal ultrasound revealed a minimally atrophic right testicle. With evidence supporting the potential for response to chemotherapeutic treatment in TGCT, the patient was started on cisplatin and etoposide. Bleomycin was planned for the second cycle of chemotherapy if his pulmonary function improved. A salient feature of all invasive TGCTs is a gain in material in the short arm of chromosome 12, and is diagnostic if present. Although the initial pathology revealed a non-diagnostic metastatic tumor, further testing revealed amplification of chromosome 12p. The examination of poorly differentiated carcinomas of an unknown primary site using light microscopy and immunohistochemical profiling alone may be inadequate, and should undergo molecular chromosomal analysis.This case is presented for its unconventional presentation and rarity of occurrence. It brings forward the discussion of both the commonality of TGCT in young male adults, as well as the anomaly of a 'burned out' phenomenon. With unreliable tumor markers, nonspecific symptoms, and pathological findings, ‘burned out’ TGCTs may account for a challenging diagnosis in a variety of cases, especially with the presenting symptom arising from a less common metastatic site. This case adds to the increasing literature on a rare entity of the 'burned out' TGCT, and upon literature review, presents itself as the first reported case presenting with brain metastasis. Testicular germ cell tumors (TGCTs) are the most common malignancy diagnosed in males aged 20 to 39, and the incidence is increasing -3. TGCTsThe phenomenon of a primary TGCT outgrowing its blood supply and undergoing auto-infarction has been described as a ‘burned out’ TGCT. The regressed testicular lesion is not appreciable on physical exam, and spontaneous regression occurs without treatment . DespiteImaging can be helpful in making the diagnosis, with scrotal ultrasonography revealing evidence of a regressed tumor. Possible findings consist of a hypoechoic area, atrophic testicle, or microcalcifications -8. MacroOf significance, extra-gonadal germ cell tumors (EGCT) are a known entity that also present with biochemistry and histological findings of a germ cell tumor in the absence of primary testicular or ovarian tumor. However, EGCT are differentiated from ‘burned out’ TGCT by their characteristic midline location, from the pineal gland to the coccyx. Furthermore, in EGCT no radiologic nor pathologic evidence of a primary malignancy is present in the primary reproductive organs .Chemotherapeutic strategies implemented in the 1970s for the treatment of advanced stage TGCTs represents a paradigm shift to a curable disease -15. HereA 24-year-old previously healthy male presented with progressive nausea, vomiting, visual changes, and memory impairment. His only significant finding on history was a strong family history of factor V Leiden mutation. The physical exam was grossly unremarkable. The initial magnetic resonance imaging (MRI) reported a single mass in the right frontal lobe Figure .With high suspicion for primary brain tumor, total resection of the intracranial lesion was performed and revealed a metastatic, poorly differentiated carcinoma with an inconclusive immunohistochemical profile.Staging investigations with computed tomography (CT) and positron emission tomographic (PET) scans revealed pulmonary and pelvic tumor deposits. A scrotal ultrasound revealed a minimally atrophic right testicle with no further abnormalities detected. A follow-up cranial MRI revealed enhancement in the surgical bed and new metastatic foci Figures , 3.Pathological and imaging findings were consistent with metastatic carcinoma with progressive brain lesions from an unestablished primary focus. At this time the brain lesions were increasingly symptomatic. Further treatment options with chemotherapy and whole brain radiation therapy (WBRT) were discussed with the patient. The patient refused palliative intent chemotherapeutic intervention for unknown primary, but agreed to WBRT with a prescribed dose of 30 gray in 10 fractions delivered. Subsequent to this, additional remote pathological consultation with chromosomal analysis revealed isochrome 12p amplications, consistent with a TGCT. Tumor markers with alpha-fetoprotein (aFP), beta-human chorionic gonadotropin (BhCG), and lactate dehydrogenase (LDH) were not elevated. With evidence supporting the potential for response to chemotherapeutic intervention in TGCT, the patient was started on cisplatin and etoposide, with the plan to include bleomycin in subsequent cycles if his pulmonary function improved -16. UnfoHe rapidly deteriorated before receiving a full course of treatment and succumbed to his disease only five months after initial presentation. Informed consent was obtained from the patient initially and from the patient's family after he passed away.English publications of ‘burned out’ TGCT case reports were identified from Medline and EMBASE databases via OVID engine without restrictions on year of publication. The keywords were “germ cell tumor,” “burned out phenomenon,” and “testicular tumor.” Additional studies were identified from reference lists of retrieved papers and review articles. Studies that did not discuss primary testicular origin were excluded. The search yielded 38 results and each abstract was reviewed. A total of 27 articles were thoroughly reviewed and 79 cases of ‘burned out’ TGCTs were identified. The presenting sites, age of patient, tumor markers, histology, treatments employed, and outcomes were tabulated Table  5, 7-8,-40. The sites of symptomatic metastasis identified were retroperitoneal 51.9%), testicular 12.7%), mediastinal 3.8%), pulmonary (3.8%), gastric (3.8%), and others (24.1%) consisting of prostate, supraclavicular, head and neck, and widely disseminated. The average patient age at presentation was 32.7 years old. Tumor markers were not found to be consistently elevated, with only 12.7%, 10.2%, and 5.1% of the cases found to be increased for BhCG, aFP, and LDH respectively. The most common treatment employed was orchiectomy with chemotherapy (57.5%), followed by chemotherapy alone (32.5%). Radiation therapy was utilized in four (10%) cases, all of which were seminoma [1.9%, tes%, medias%, pulmonA salient feature of all invasive TGCTs is a gain in material in the short arm of chromosome 12, and is diagnostic if present . AlthougThe behavior and aggressive nature of the tumor discussed throughout this case combines the complexity of the evolving field of tumor biology and unique patient characteristics. Interestingly, the patient had a confirmed family history of factor V Leiden mutation. It has been suggested that clotting factor polymorphisms such as factor V Leiden are associated with cancer onset and progression. The theoretical mechanism behind such adverse effects stems from the involvement of tissue factor and thrombin in tumor angiogenesis, which is essential for tumor growth and metastasis . FurtherThis case is presented for its unconventional presentation, rarity of occurrence, and difficulty in diagnosis. It brings forward the discussion of both the commonality of TGCT in young male adults, as well as the anomaly of a ‘burned out’ TGCT. With unreliable tumor markers, nonspecific symptoms, and pathological findings, the ‘burned out’ phenomenon accounts for a challenging diagnosis, particularly with the presenting symptom arising from a less common metastatic site. This case adds to the increasing literature on the rare entity of the ‘burned out’ TGCT, and upon literature review, presents itself as the first reported case presenting with brain metastasis. By establishing a strong foundation of ‘burned out’ TGCT in the literature leading to familiarity of the diagnostic process, a deeper understanding into medical management may arise.


Traumatic AVF of the scalp is a rare abnormal vascular disease. It is defined as a communication between the high flow arterial system and the low flow venous network, which directly connects the arterial feeding vessels of the scalp and the draining veins without an intervening capillary bed. The superficial temporal artery (STA) was involved in 90% of the cases, and 71% of the patients only had one dominant feeding STA. Here, we report the case of a rare large traumatic arteriovenous fistula (AVF) of the scalp that is fed by intra- and extracranial blood supply. The clinical and radiological features are presented, and the possible pathogenesis and surgical technique are discussed. Traumatic AVF of the scalp is rare vascular disease , 2 and iIn 90% of the patients, superficial temporal artery (STA) was involved, and in 71% of the cases there was one dominant feeding STA . SeveralHere, we present a case of a large sized fistula fed by a complex network of feeding vessels that involved both intra- and extracranial blood supply, which, to the best of our knowledge, has never been reported previously. According to the angioarchitecture and presentation of the case, and for economic reasons, we chose straight-forward surgical treatment of the AVF over a pure endovascular treatment or in combination with surgery.A 57-year-old man was diagnosed with paranoid schizophrenia at the age of 20. Eight years prior to presentation in the emergency department, the patient sustained a fist blow to the top of the left temporoparietal region, in a conflict with another patient during treatment in a psychiatric institution. Initially, after the injury, a thumbnail-sized mass developed. It increased in size with time. The patient was therefore hospitalized by his guardian. At presentation an irregular hemispherical mass measuring 8 × 5 × 3 cm was noted in the left temporoparietal area ; this maAfter obtaining informed consent from his guardian, the AVF was resected under general anesthesia. In order to decrease the intraoperative bleeding, we first temporarily ligated the proximal branch of the feeding vessels (both STAs) twice, by suturing through the skin with a silk suture; then a 25 cm long horseshoe-shaped scalp flap including the galea was incised around the mass and pedicled towards the temporal region. Subsequently, the feeding arteries and draining veins were isolated and sequentially ligated with the exception of the feeding arteries within the periosteum, which as we suspected arose from intracranially . The periosteal feeding vessels were cut superficially after coagulation. The overlying arteriovenous fistulous mass was then removed. By leaving the periosteum that contained stumps of the feeding vessels which originated from the left ACA, we prevented retraction of the stumps and a possible subsequent intracranial bleeding. After resection of the AVF, we meticulously cauterized minor bleeding sites with the bipolar coagulation forceps, particularly on and around the deliberately left periosteum and then released the previous temporary ligations around the STAs. Lastly, the wound was closed in a multilayered fashion. The surgery was uneventful, with blood loss of less than 50 mL. The pathological report revealed many irregular blood-supplying arteries with large diameters and thickened walls that were interwoven to form a mass and arteriovenous fistula. A postoperative CT scan showed no intracranial hemorrhage. The wounds healed nicely, and the shape of the head appeared normal. Furthermore, there was no recurrence in the 1-year follow-up.Wardrop first reported this disease in 1827 . To our It is generally accepted that scalp arteriovenous fistula may be of either congenital or traumatic lesion. Most congenital lesions become symptomatic in the third decade of life (range: three months to 59 years of age) . MatsushThe disease is characterized by four factors: a pulsating mass on the head, thickening of the vasculature morphologically and on angiography, pulsatile sounds on auscultation that will decrease and eventually disappear when the main feeding arteries are depressed, and draining veins. Angiography is the gold standard diagnostic tool to highlight the angioarchitecture .For economic reasons, we opted for conventional surgical excision, which is cost efficient and does not bear the risk of cosmetic irregularities after interventional injection/placement of embolic material in the subcutaneous tissue , 2, 15.


We aim to illustrate the multimodal imaging spectrum of hepatic involvement in tuberculosis (TB). Whilst disseminated tuberculosis on imaging typically manifests as multiple small nodular lesions scattered in the liver parenchyma, isolated hepatic tuberculosis remains a rare and intriguing entity.Indubitably, imaging is the mainstay for detection of tubercular hepatic lesions which display a broad spectrum of imaging manifestations on different modalities. While sonography and computed tomography (CT) findings have been described in some detail, there is a paucity of literature on magnetic resonance imaging (MRI) features. Due to a significant overlap with other commoner and similar appearing hepatic lesions, hepatic tuberculosis is often either misdiagnosed or labelled as indeterminate lesions. This article is a compendium of cases highlighting the spectrum of imaging patterns that can be encountered in patients with isolated primary hepatic tuberculosis as well as disseminated (secondary) disease. Rare patterns of primary disease such as tubercular cholangitis, hypervascular liver masses, and those with vascular complications are also illustrated and discussed.Imaging plays a valuable role in the detection of tubercular hepatic lesions. Also, imaging can be helpful in their characterisation and for assessing associated complications.Hepatic TB has myriad imaging manifestations and is often confounded with neoplastic lesions.• Imaging patterns include miliary TB, macronodular TB, serohepatic TB and tubercular cholangitis.• Concurrent splenic, nodal or pulmonary involvements are helpful pointers towards the diagnosis.• Miliary calcifications along the bile ducts are characteristic of tubercular cholangitis.• Histological/microbiological confirmation is often necessary to confirm the diagnosis.•  Tubercular infection constitutes one of the foremost causes of death and morbidity across the world, more so in the tropical region. With the emergence of HIV and AIDS, the disease that was thought to be endemic to emerging nations has become pandemic in nature –3. ApproTubercular involvement of the liver is more commonly a part of disseminated disease wherein the hepatic parenchyma shows a diffuse pattern of involvement in the form of multiple small-sized miliary nodules. In contrast, isolated hepatic tuberculosis is seldom encountered in clinical practice with only a few sporadic cases and short series available in the current literature –9.Although there is no standard classification system available for hepatic tuberculosis, Levine  classifiHepatobiliary tuberculosis most commonly affects people in the 11– to 50-year-old age group with the peak incidence of the disease reported in the second decade of life , 12, 13.Generally, the disease remains silent and is often incidentally detected while the patient is being evaluated for a mostly non-specific symptomatology. It may however present in the form of abdominal pain or organomegaly which may or may not be associated with clinical jaundice. Jaundice if present is usually a consequence of extrahepatic biliary obstruction secondary to attendant periportal lymphadenopathy , 15.Laboratory analysis may reveal altered liver function tests in the form of elevated hepatic enzymes. The elevation of alkaline phosphatase can be seen in jaundiced as well as non-jaundiced patients. In the event of isolated elevation of the alkaline phosphatase the possibility of tubercular hepatic parenchymal involvement must be excluded , 13.The imaging manifestation of the tubercular hepatic disease can be wide ranging but can be broadly categorised into miliary pattern, nodular tuberculosis with serohepatic variant and tubercular cholangitis. As imaging pattern is largely non-specific, a histopathological or bacteriological confirmation is often required.It is the most common pattern of hepatic involvement by tuberculosis. Miliary pattern is usually associated with disseminated disease. Accordingly, concurrent involvement of the spleen and other abdominal organs is not uncommon . The milOn computed tomography (CT), miliary lesions appear as microabscesses in the form of multiple small foci with a low attenuation ranging between 30 to 40 HU. The lesions appear nodular or cystic and may exhibit minimal peripheral enhancement following intravenous contrast administration Fig.  or otherCalcification is not uncommon in long-standing chronic disease. In fact, these lesions are better discernible and more frequently discovered at this stage, where they manifest as small nodular foci of calcifications Fig.  9].Fig..Fig.9].FTubercular hepatic lesions that are more than 2 cm in size are referred to as macronodular or pseudotumoral tuberculosis. This form of hepatic tuberculosis is rare compared to the miliary variant and frequently manifests as solitary or multiple variable-sized hepatic masses. Often it may be difficult to distinguish these lesions from the more common neoplastic and other infective lesions. Depending on the stage of the hepatic granuloma, the imaging appearances can be quiet variable –10, 21.Sonographically, these lesions can range from being heterogeneously hypoechoic Fig. , mixed hCT imaging features of macronodular granuloma of the liver depends on the stage of the disease. Non-caseating granulomas appear hypodense on the unenhanced study and usually display no or minimal peripheral rim enhancement following intravenous contrast administration Figs. . UnderstRarely, tubercular liver abscesses can rupture resulting in extra-hepatic complications Fig.  such as On magnetic resonance imaging (MRI), macronodular tubercular lesions appear hypointense on T1-weighted images and hypointense, isointense or hyperintense lesions with a peripheral hypointense rim on T2-weighted images depending upon the stage of the disease Fig. . Post coSerohepatic tuberculosis is the rarest variant reported in the literature characterised by predominant involvement of the subserosal plane of the liver i.e. the connective tissue lying beneath the serous coat of liver which is often inseparable from the fibrous Glisson capsule. The imaging features of this subtype include peripherally positioned lesions centred in the subcapsular plane of the liver. The thickened liver capsule and sub-capsule overlying these hypoattenuating lesions simulates a ‘sugar-coating’, an appearance popularly referred to as “frosted liver” Fig.  9].Fig..Fig.9].FTubercular cholangitis can either be due to primary involvement of the biliary tree or secondary involvement of the biliary tree due to compression by enlarged periportal nodes and/or hepatic granulomas. Biliary tree involvement can be variable i.e. may involve either the  intrahepatic biliary radicles or the larger (juxtahilar or extrahepatic) bile ducts. Imaging findings can vary from bile duct thickening, biliary dilatation and strictures to frank obstructive biliopathy Fig . Allied MRI and magnetic resonance cholangiopancreaticography (MRCP), endoscopic ultrasound, and endoscopic retrograde cholangiopancreaticography (ERCP) also play an important role in the diagnosis and management of patients with biliary tuberculosis. MRCP depicts the biliary anatomy and pathology not only non-invasively Figs.  and 21 bHepatic tuberculosis has many faces and the imaging manifestation can show a considerable overlap with other relatively more frequent primary or secondary lesions of the liver. Isolated hepatic involvement by tuberculosis can especially be challenging to diagnose on imaging alone due its largely non-specific imaging features. Nevertheless, in endemic countries and in appropriate clinical settings an atypical imaging pattern of a hepatic lesion should prompt the radiologist to consider hepatic tuberculosis as one of the differential considerations. Although image-guided biopsy is usually required for a confirmatory diagnosis the presence of calcifications and the concurrent involvement of extrahepatic sites  should prompt the possibility of hepatobiliary tuberculosis.


Lateral presentation of meningocele and meningomyelocele is extremely rare. Most of the lateral meningocele described are associated with other syndromes. Isolated lateral meningomyelocele cases are rarer still. We herein report a neonate with isolated lateral gluteal meningomyelocele.  A full-term baby boy was referred for a congenital gluteal swelling on the second day of life. The child was otherwise healthy without any neurological deficits. Examination revealed soft, cystic, fluctuant, right upper medial gluteal region . The swelling was reducible on pressure and refilled on release. Transillumination was suggestive of clear fluid. A plain radiograph showed classical spindle-shaped widening of interpeduncular space in the sacral region. CECT performed showed widened interpeduncular distance at S1-S3 areas, and a lateral swelling showing evidence of CSF . Exploration revealed a CSF filled meningomyelocele with some neural elements . Neural placode was reduced and repaired with a gluteal muscle cover . Post-operative period was uneventful. One and a half years later, MRI revealed tethered cord with a cystic component at sacral region. The cyst was excised in toto and de-tethering of the cord was done. Post-operative period was uneventful. Histopathology of excised mass revealed glandular columnar epithelium along with some neural element. On 8 year follow-up child is doing well without any neurological sequel.Meningomyelocele are the most common forms of neural tube defects. Lateral presentations are extremely rare. Perusal of literature revealed only 2 such cases published earlier . Both these cases have been in older children. The index case is a neonate presenting with a compressible gluteal mass. Lateral meningocele have also been reported with other neurocutaneous syndromes and Marfans syndrome. The index case did not have any café au lait spots or multiple neurofibromas. Panil Kumar et al [3] published multiple level lateral meningocele but associated with other syndromes. Neonatal isolated gluteal meningocele has not been published earlier. Shore [4] described lateral cervical meningocele. Many meningomyelocele often are associated with tethered cord. Index case also presented at 2 years of age with tethered cord due to neuro-enteric cyst.Source of Support: NilConflict of Interest: Nil


Introduction. Rhinosporidiosis seeberi causes a chronic granulomatous disease of upper airway, usually involving the nose and nasopharynx, and has a notorious tendency to reoccur. The current line of management is surgical excision of the lesion along with cauterization of the base, which does not prevent reoccurrence of the disease. Case Presentation. A 65-year-old male resident of rural India reported a history of breathing difficulty and change in voice. Patient is a Hindu priest by profession, who according to their rituals has to take bath in local pond or river. Conclusion. Rhinosporidiosis is a difficult to treat pathology due to its tendency to reoccur. Till date the management of the disease is far from satisfactory. Coblation system which has already found its roots in otorhinolaryngology can be used as a novel tool in surgical resection of recurrent rhinosporidiosis and has added advantage of low temperature dissection along with clear surgical field due to constant suctioning.Recurrent rhinosporidiosis is a chronic granulomatous disease with a known tendency to reoccur. Coblation EVAC 70 is a novel surgical tool which seems to provide excellent option in management of this notorious disease. We present an interesting case and the innovative approach in its management, using Coblation system.  Rhinosporidiosis is a rare chronic granulomatous disease of mucocutaneous tissue. This disease is widely endemic in Indian subcontinent and some parts of Africa. The etiological agent rhinosporidiosis seeberi is an aquatic protistan parasite . The disA 65-year-old male presented to us with difficulty in breathing, change in voice, and nasal obstruction. Patient was a Hindu priest by profession, was a resident of Jharkhand, and had a positive family history of rhinosporidiosis in a paternal cousin and maternal uncle. Patient had been operated on 11 times earlier; first surgery was done when he was 16 years of age. The last procedure was done about 1 year back with KTP laser when he had to undergo a tracheostomy because of the extensive nature of the disease. On examination, a fleshy mass was seen filling both nasal cavities with yellowish pinhead sized spots on surface. Note was also made of the presence of septal perforation in the cartilaginous part of the septum. The mass was extending in the oropharynx behind the soft palate. On laryngoscopy, growth was seen involving bilateral vallecula, bilateral glossoepiglottic fold, and lingual surface of epiglottis pushing the epiglottis over the laryngeal inlet. Vocal cords could not be visualized. A contrast enhanced computed tomographic scan was done, which revealed a homogenous mass filling bilateral nasal cavity without any bony invasion, although thinning of bilateral lamina papyracea was noted. Also mass was filling nasopharynx and oropharynx involving the lingual surface of epiglottis, obscuring laryngeal inlet . Taking An elective tracheostomy was done taking into view the difficult airway. Patient was put under general anesthesia. The nasal extension of the mass was first addressed under endoscopic vision. Using Coblation wand EVAC 70, the nasal mass was ablated but there was no attachment in the nasal cavity and mass appeared to arise from the nasopharynx attached to the posterior wall. The patient was then put in tonsillectomy position and mouth gag was applied and then mass was removed from the nasopharynx using Coblation wand at a setting of 8 in ablation mode, and the wand had to be bent to approach the choanal region. Following this the mass was removed from the oropharynx including vallecula and lingual surface of epiglottis and lateral glossoepiglottic folds . To stopOn gross pathology, fleshy mass with white pinhead sized spots on the surface was noted. Microscopic evaluation identified sporangia and endospores demonstrating a granulomatous reaction with eosinophilia. Final diagnosis was consistent with rhinosporidiosis with chronic inflammation and giant cell reaction. Patient was started also on dapsone after assessing the G6PD level on 100 mg twice daily.Postoperatively patient was followed up for 1 year, during which patient was subjected to endoscopic examination twice, with no evidence of reoccurrence at 1 year of follow-up.Rhinosporidiosis is a rare disease which has been known for hundred years since its first description in 1900 by Seeber, an individual from Argentina . It is a Rhinosporidium seeberi in a class, the Mesomycetozoea, along with 10 parasitic and saprobic microbes. The controversial spherical bodies have been shown to comprise both lipid-protein nutritive bodies and other spherical bodies [Dermocystidium, the rosette agent, Ichthyophonus, and Psorospermum) [Rhinosporidiosis seeberi, the causative organism, has a debatable taxonomy as the microorganism is intractable to isolation and microbiological culture, and the morphological features resemble both fungi and protozoa. But recently a study has classifiedl bodies . This isspermum) , 5.Diagnosis is made by biopsy and histological examination of the section. The tumor is covered with columnar epithelium, along with many spores and sporangia in various stages of development. Also there is cellular infiltrate with lymphocytes, plasma cells, polymorphs, and eosinophils .The treatment of choice is wide local excision with cauterization of the base. In addition, systemic dapsone is given for a period of 1 year and is known to prevent reoccurrence which arrests the maturation of sporangia and promotes fibrosis , 7, 8. BIn a case report of recurrent rhinosporidiosis by Nichlani, in which they excised the lesion using diode laser, they followed up the case for 1 year, in which no recurrence was reported . In 2014μm [Coblation has recently been used to treat various otolaryngological conditions: obstructive sleep apnea, tonsillectomy, turbinate reduction, and benign tumors of head and neck . Coblatiμm . As the With the use of Coblation system, we could achieve complete resection of the lesion, with minimum bleeding and less blood contamination, also preserving the integrity of local tissue. Patient is under follow-up for last 1 year and there is no evidence of occurrence .Coblation system can be a promising new tool in the surgical resection of recurrent rhinosporidiosis. This system enables the surgeon to obtain better resection of the disease due to constant suctioning, with minimum lateral thermal damage. This also decreases the chances of reoccurrence due to very little contamination of the surrounding areas by preventing autoinoculation. The novel design of the wand can help to resect “the difficult to reach areas.”


Neonatal diaphragmatic abscesses are extremely rare and they usually develop by direct extension from a liver abscess. The first case of primary diaphragmatic abscess in a neonate is reported and the difficulties of diagnosing this rare entity are discussed. Intra-abdominal abscesses are rare in neonates, and they could occur spontaneously or as a complication of abdominal pathologies and surgical procedures [1]. Neonatal diaphragmatic abscesses are extremely rare and they usually develop by direct extension from a liver abscess [1-4]. We report a case of primary diaphragmatic abscess in a neonate. To the best of our knowledge, such localization has not been previously reported for neonatal intra-abdominal abscesses.We describe a male newborn at 29 weeks gestation with a birth weight of 1100 g and delivered by primary cesarean section due to eclampsia. He was admitted to the neonatal intensive care unit for neonatal respiratory distress syndrome. Umbilical venous catheter and Broviac catheter were placed for three days and twelve days, respectively. The evolution was initially favorable.On day 32, the baby also developed fever in addition to respiratory distress suspecting a nosocomial infection. He required intubation and initiation of mechanical ventilation. Blood cultures were drawn, and ampicillin and cefotaxime were started. Laboratory investigations revealed a white cell count of 11.2 × 109/l, neutrophils 9.3 × 109/l, platelets 126 × 109 /l, C-reactive protein 110 mg/l and hemoglobin of 11.9 g/dl. Blood cultures, analysis of the cerebrospinal fluid, and urinalysis were negative. The chest X-ray showed a right basilar opacity. Ultrasound scan showed 2 collections in the liver suggesting a hematoma. Thoraco-abdominal computed tomography scan showed a 5.5 X 4.2 X 3.2 cm abscess in the liver dome with trans-diaphragmatic spread  but the origin of the abscess could not be identified (pulmonary or hepatic). We retained the diagnosis of liver abscess and a right subcostal laparotomy was performed. No abscess in the liver was found. The bare area of the liver was opened and exposed but there was no liver lesion. Then, a right posterolateral thoracotomy was done; the right lung had normal appearance without pleural effusion. We found a 5x5 cm fluctuant swelling in the right diaphragmatic dome. Six ml of yellowish pus was drained by puncture-aspiration. Surgical drainage was performed; a drain was left in abscess cavity for five days. Culture of pus was positive for staphylococcus aureus. The baby remained hospitalized for 3 weeks postoperatively because of a diaphragmatic hypokinesis. Follow-up ultrasound before discharge showed resolution of the diaphragmatic abscess.Intra-abdominal abscesses are uncommon in neonates. The literature review shows that liver abscesses were the most common location for intra-abdominal abscesses. Risk factors include blood sepsis, umbilical catheterization, central parenteral nutrition catheters, necrotizing enterocolitis, surgery, and prematurity [1-4]. Neonatal diaphragmatic abscesses are extremely rare and they usually develop by direct extension from a liver abscess [1-3]. We reported the first case of a primary diaphragmatic abscess in a neonate.The diaphragm harbors a rich vascular network including arteries forming a branch-like pattern on its superior and inferior surfaces, veins and lymphatic plexuses [5]. Therefore, we suggest that the diaphragmatic abscess was caused from hematogeneous spread via the diaphragmatic arteries. It can also be argued that it could have been a congenital lesion of the diaphragm that acted as a nidus for the infection to lodge and fester. Such lesions are extremely rare in children and only few cases have been reported in the literature .In our case, the diaphragmatic location of the abscess was not detected on CT scan. Diaphragmatic lesions are very difficult to detect on CT scan and, unless a clear splitting of the diaphragm is identified, the correct location of the lesion can be recognized only at surgical exploration [7]. We believe that magnetic resonance imaging (MRI) could have possibly better defined the exact site of this exceptional localization of thoraco-abdominal abscesses.Source of Support: NilConflict of Interest: None


Early bowel obstruction is not a rare complication of gastrectomy, and it may require re-operation in some cases.We report the case of a 71-year-old woman who underwent a total gastrectomy with Roux-en-Y reconstruction for a massive gastrointestinal stromal tumor. Postoperatively, she was making good progress and started consuming meals on postoperative day 3. However, on postoperative day 10, she complained of upper abdominal discomfort and nausea. Blood tests showed a mild inflammatory reaction. An upper gastrointestinal series showed obstruction of the elevated jejunum. An abdominal computed tomography scan suggested upper bowel obstruction. Endoscopic observation and repositioning was selected as an effective approach for treatment considering the patient’s clinical condition and background. Upper gastrointestinal endoscopy showed kinking of the elevated jejunum, easy passage through to the anal intestine, and no evidence of mucosal edema, stenosis of the Roux-en-Y anastomosis, bowel ischemia, or necrosis. After endoscopic repositioning, upper gastrointestinal series showed good passage of the jejunum and no evidence of bowel obstruction. At the 6-month follow-up examination, the patient was in good condition and had no complaints.We concluded that early endoscopic management should be the effective procedure considered for diagnosis and treatment of early bowel obstruction after gastrectomy in some cases. Gastrectomy is associated with several postoperative complications, and early gastrointestinal obstruction is not a rare complication and generally occurs 2 weeks after surgery –3. Most A 71-year-old woman was referred to our hospital to undergo surgery for a gastrointestinal stromal tumor . She suffered from deep vein thrombosis (DVT) after undergoing total hip arthroplasty 6 months previously and was receiving oral anticoagulant therapy. We performed total gastrectomy because it was the massive GIST, which was 9.0 × 9.5 × 5.0 cm in size and was located in the anterior side of the stomach and 2 cm from the gastroesophageal junction. Roux-en-Y reconstruction with the retrocolic route was conducted. The jejunojejunostomy was located 40 cm from esophagojejunostomy, and petersen defect and jejunojejunal mesenteric defect were closed during the surgical procedure. The patient was making good progress after the operation and started consuming meals on postoperative day 3. However, on postoperative day 10, she complained of upper abdominal discomfort and nausea. Physical examination showed partial abdominal tenderness and no rebound tenderness, with no rigidity, and the patient had a fever (38.2 °C). The white blood cell count was 9100/μL , and the C-reactive protein level was 3.0 mg/L , indicating mild inflammation; no remarkable abnormalities were found in other biochemical tests including renal, hepatic function, and blood gas tests. An upper gastrointestinal series (UGIS) and abdominal X-ray picture showed decreased peristalsis and distension of the jejunum with no visible passage to the anal intestine Fig. , b. CompRecent studies have reported that endoscopic repositioning should be the first procedure considered for patients with lower gastrointestinal conditions like sigmoid colon volvulus . HoweverBowel obstruction after gastrectomy can be an early or late complication, with the 2-week period after surgery as the threshold , 7. LateIn the present case, we could not ascertain the cause of bowel obstruction, although imaging findings suggested the possibility of internal herniation or bowel obstruction due to banding or adhesion of the elevated jejunum. We closed possible hernia sites during the surgical procedure to prevent internal herniation. Therefore, we could not arrive at a conclusion whether possible hernia sites should be closed during operation. In our cases, we should have shortened the elevated jejunum in surgical procedure.There was no evidence of strangulation, bowel ischemia, or perforation in the images. Nasogastric decompression and bowel rest did not improve her situation. Her condition was stable with no abdominal tenderness, and further, she had several surgical risk factors and needed to avoid re-operation. We selected endoscopy for observation and treatment because it has several merits: endoscopy ensures (1) viability of the bowel, shows the exact location of the damage and provides information regarding the mucosal appearance; (2) allows mechanical release of bowel obstruction and decompression of the dilated proximal bowel; and (3) is less invasive and more convenient than surgery . In factWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Patients with primary multiple malignancies are progressively increasing due to prolonged survival of cancer patients and to the advances in diagnostic techniques and therapeutic options.Here we present a 66 year-old caucasianpatient with four synchronous primary malignant tumors affecting the lung, oropharynx, large bowel and prostate gland, respectively, treated with multidisciplinary approach.The increased incidence of multiple malignant tumors is a real challenge to the clinician and clinical attention should be made to avoid a misdiagnosis. In addition an early diagnosis is essential to achieve a radical treatment. We believe that the treatment modality should be carefully made and tailored on the individual patient suffering from this disease. Patients with multiple primary malignancies (MPMs) are progressively increasing; these tumors may be metachronous or synchronous. This distinction implies important diagnostic and therapeutic challenges. From a diagnostic point of view the different patterns of MPMs should be considered. Therapeutically, a multi-disciplinary and patient-oriented approach should be considered. Hereby, we present a case of four primary malignant synchronous tumors affecting the lung, oropharynx, large bowel and prostate gland, respectively.18F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed avid uptake of the pulmonary nodule as well as oropharyngeal, sigmoid colon, and prostate gland uptake  scan confirmed the presence of a pulmonary malignant-looking nodule without hilar lymphadenopathy. Since the first report of Billroth and the definition of Warren and Gates , the incWritten informed consent was obtained from the patient for publication of this case report and accompanying images.


A male child born at 27 weeks, weighting 1305 g and presenting with a right-sidedabdominal tumor. Computed tomography scan demonstrated the presence of a solidmass compressing the right kidney. Puncture biopsy revealed congenitalmesoblastic nephroma. The patient underwent total right nephroureterectomy, anddied on the second day after surgery. In spite of its low prevalence,congenital mesoblastic nephroma represents the most common renal neoplasm inneonates,3. Such a tumor type is generally diagnosed at birth,or even between three and six months of age, is rarely found during childhood andexceptionally in the adulthood,4. The prognosis is generally good,particularly in cases where total surgical resection  is performed,4.Congenital mesoblastic nephroma is rare renal neoplasia that was initially describedby Bolande in 1967The present article reports a case of a mixed subtype of congenital mesoblasticnephroma with poor evolution, probably resulting from prematurity and postoperativeseptic shock.A male child born at the 27th gestational week, weighting 1305 g and with 36 cm inheight. At physical examination, the child presented with globose abdomen, with atumor at right, besides respiratory failure, and the patient was referred to theneonatal intensive care unit. Abdominal ultrasonography (US) demonstrated a solidtumor measuring 5.0 × 4.7 × 4.0 cm between the renal and hepaticregions . Abdomin. Such tumors probably originate from nephrogenicmesenchymal proliferation and generally present as an incidental finding ofasymptomatic abdominal mass in neonates,3. In some cases,tumor rupture may occur, leading to hemoperitoneum,6. In somecases, the diagnosis may be made early in the prenatal period either by means ofamniocentesis or US,6. Besides tumor mass, sonographicfindings include presence of polyhydramnios, sometimes in association withnon-immune hydrops, which is generally fatal.Congenital mesoblastic nephromas  are most commonlyfound in male individuals, at a ratio of 2:1. Such methodscan preoperatively assess the local invasion by the tumor, besides allowing tofollow-up the patients for possible local recidivation. Usually, US demonstrates a homogeneous, solid,well defined lesion with a gross appearance,9. In some cases,typical small-sized mesoblastic nephromas may show a concentric pattern, alternatingbetween hypo- and hyper-echoic rings. The use of Doppler US allow for detecting intratumoralhypervascularization.Imaging methods play an important role in the post-natal diagnosis of mesoblasticnephromas. The presence of small cysticareas is rarely observed, particularly in cases of typical mesoblasticnephromas,9,10. Magneticresonance imaging (MRI) is useful to identify invasion of local structures by thetumor. At MRI, mesoblastic nephromas present isosignal in relation to the renalcortex and to the skeletal muscle at T1-weighted images, and hypersignal in relationto those structures at T2-weighted images. Excretory urography also is useful in the diagnosis ofcongenital mesoblastic nephromas, allowing for detecting distortion of thepyelocaliceal system caused by the presence of the renal mass.CT allows for visualization of a solid renal mass with variable attenuationcoefficient and possible necrotic and hemorrhagic areas, giving the lesion aheterogeneous appearance, which could indicate a worse prognosis. Wilms' tumor andcongenital mesoblastic nephromas are radiologically indistinguishable, so thedifferential diagnosis between such conditions is only possible by means ofhistopathology.The differential diagnosis of congenital mesoblastic nephromas should be made withother renal neoplasias such as metanephric adenomas, renal clear cell carcinomas andWilms' tumor.Macroscopically, the tumor is generally whitish and solid, with weight and volumeranging from 0.01 kg to 2 kg, and 150 to 200 cm3, respectively,4. Two presentations of this tumor can be observed at themicroscope, namely the classical and cellular patterns. At immunohistochemicalanalysis, congenital mesoblastic nephromas are positive for vimentin and smoothmuscle actin, and negative for desmin, CD34, epithelial membrane antigen andcytokeratin.Generally, mesoblastic nephromas are unilateral tumors, although in some cases theymay be found bilaterally,6. With resection, the prognosis will be generally good,particularly in cases of classical histological subtype.Usually, the treatment of this type of tumor is surgical, either with nephrectomy ortotal nephrouretectomy which, besides reducing the possibility of recurrence, willserve as treatment for the hypertension secondary to hyperreninemia.Factors determining a poor prognosis are related to age, presence of positivesurgical margins, and to a mixed histological typeIn the present report, the case of a male, preterm neonate with a large, solid renaltumor at right is described. Imaging studies demonstrated the tumor withheterogeneous contrast enhancement. Histopathological analysis in association withimmunohistochemical panel suggested the presence of a mixed subtype of congenitalmesoblastic nephromas. The patient was submitted to total nephrouretectomy.Basically, except for the prematurity, all the other characteristics of the presentcase are in agreement with the literature.The authors conclude that mesoblastic nephromas represents a rare renal neoplasia,and the knowledge on its clinical, radiological and histopathologicalcharacteristics is important to facilitate the differential diagnosis with othermost common pediatric renal tumors.


The aim of this report is related to adult primary paratesticular mesenchymal tumors with emphasis on a case presentation and discussion of the spermatic cord leiomyosarcoma. Primary paratesticular tumors are rare, only accounting for 7% to 10% of all intrascrotal tumors. In adults, more than 75% of these lesions arise from the spermatic cord, 20% being leiomyosarcoma. Tumor grade, stage, histologic type, and lymph node involvement are independently predictive of prognosis.The case report concerns a 81-year-old man presented with a 3-year history of painless lump in the right hemiscrotum. Scrotal examination demonstrated a 5.1-cm, firm-to-hard mass attached to the spermatic cord. Scrotal ultrasound scan revealed a heterogeneous mass separate from the testis. He was treated with an radical orchi-funicolectomy. Histologically the lesion is composed of spindled cells with often elongated, blunt-ended nuclei and variably eosinophilic cytoplasm. Areas with pleomorphic morphology are present. The level of mitotic activity is equal to 3/10 HPF in the areas with spindle cell morphology and to 12/10 HPF in the areas with pleomorphic morphology. The final diagnosis was that a leiomyosarcoma of the spermatic cord, with grade 1 and grade 2 areas, stage pT2b cN0 and cM0. The patient has been followed up for 3 months with CT scans and shows no signs of recurrence.Spermatic cord leiomyosarcoma, although rare, should be one of the first differential diagnoses for a firm-to-hard lump in the cord. Apart from radical orchi-funicolectomy, there has been added benefit of adjuvant radiotherapy to prevent any loco-regional lymph node recurrence.http://www.diagnosticpathology.diagnomx.eu/vs/1613030331125632The virtual slide(s) for this article can be found here:  Primary paratesticular tumors are rare, only accounting for 7% to 10% of all intrascrotal tumors. In adults, more than 75% of these lesions arise from the spermatic cord . The mosAmong the malignant tumors, the most common histotype is liposarcoma 46.4%), followed by leiomyosarcoma (LMS) (20%), malignant fibrous histiocytomas (MFH) (13%), and embryonal rhabdomyosarcoma (9%) 6.4%, fol,6.Most scrotal tumors are deep-seated. Benign lesions may present as slowly enlarging, asymptomatic or mildly uncomfortable masses. Benign lesions may present as slowly enlarging, asymptomatic or mildly uncomfortable masses. In general, malignant tumors are more likely to be symptomatic, large, and have a history of rapid growth. Liposarcoma is most common, while LMS and MFH histologic subtypes are observed to be the most aggressive . Tumor gLiposarcomas generally present as large extratesticular masses, which are often solid and hyperechoic by ultrasound. However, the sonographic appearance of these tumors is variable and non-specific. CT and MR imaging are much more specific with fat being easily recognized with both modalities ,12. By CWith the exception of liposarcoma, none of the other sarcomas can be differentiated from one another radiologically. They all tend to be large, complex, solid masses . BecauseHemangiomas are most common within the scrotum. Granular cell tumours of the scrotum may be multifocal and are similar to those elsewhere in the skin. Calcifying fibrous (pseudo)tumor is a densely collagenous, paucicellullar fibroblastic lesion with focal lymphoplasmacytic infiltration and scattered psammomatous calcifications.Great majority of liposarcomas are well-differentiated with various combinations of lipoma-like and sclerosing patterns. Multivacuolated lipoblasts may be present, but are not required for diagnosis. Presence of significant nuclear atypia in adipocytes is decisive. Dedifferentiation to spindle cell “fibrosarcoma-like” or pleomorphic “MFH-like” phenotype occurs in a proportion of paratesticular liposarcomas . This coLeiomyomas are composed of mature smooth muscle cells. Focal nuclear atypia may occur, but the presence of prominent atypia should lead to a careful search for mitotic activity or coagulation necrosis which are features of LMS. LMSs are typically composed of spindled cells with often elongated, blunt-ended nuclei and variably eosinophilic cytoplasm. Areas with round cell or pleomorphic morphology may occur. The level of mitotic activity varies widely, from <1 mitotic figures per 10 high power fields (HPF) to >70 mitotic figures per 10 HPF .A 81-year-old man presented with a 3-year history of painless lump in his right hemiscrotum. A scrotal ultrasound examination performed approximately two years ago showed a 2-cm lesion with cystic appearance , infiltration of the epididymis , origin from the spermatic cord (high position of the lesion within the scrotum), absence of testicular infiltration (definite testicular borders), neoplastic lesions within the spermatic cord (thick and edematous appearance) and infiltration of the vessels (vessel distention within the cord)The morphologic range of LMSs is similar to that reported in other soft tissue sites, including a tumor with prominent myxoid areas , an inflImmunohistochemistry is useful in confirming smooth muscle differentiation. Most cases stain strongly with antibodies to smooth muscle actin, muscle-specific actin, and desmin. Some also stain for CD34. Focal cytokeratin and S-100 protein positivity may occur, but myogenin stains have been negative. Spindle cell rhabdomyosarcomas in children can resemble LMSs. However, immunohistochemical stains for MyoD1 and myogenin are positive in the former and negative in the latter.The NCI grading system identify three grades in LMS ,31. GradSimple excision is suboptimal as repeat wide excision has demonstrated microscopic residual disease in 27% of cases and therefore warrants an additional adjuvant treatment. Radical orchiectomy and funicolectomy  is the cornerstone of treatment in the management of this neoplasm, but the reported survival rates indicate the need for additional treatment .Comprehension of the pattern of spread is essential, but this task is difficult by the rare occurrence of this disease. The most common means of dissemination are by regional lymph nodes spread , haematogenous metastases (most commonly to the lungs) and by local extension . Further series suggested that lymph node dissection  should not be performed unless enlarged lymph nodes are encountered on CT scans or palpated during surgery.The literature concerning benefit of adjuvant therapy after radical surgery is inconclusive because of the small numbers. Recurrence after orchidectomy alone was common in earlier reports, and adjuvant radiation has been recommended to reduce locoregional failure .Due to the high incidence of loco-regional recurrence in the lymph nodes, 2 different treatment alternatives, prophylactic retroperitoneal lymph node dissection (RPLND) and radiotherapy, have been tried. The proponents of RPLND indicate that there is a 29% risk of metastatic potential to regional lymph nodes. A review of 101 patients by Banowsky and Schultz described 29 cases of RPLND with lymph node involvement. Of these, 17 patients had isolated lymphatic dissemination. Despite such a high incidence of lymphatic spread, no report has yet shown a significant survival benefit from the addition of RPLND to radical orchidectomy . A studyIn conclusion, spermatic cord leiomyosarcoma, although rare, should be included in the list of differential diagnoses for a firm-to-hard lump in the cord. Apart from radical orchi-funicolectomy, there has been added benefit of adjuvant radiotherapy to prevent any loco-regional lymph node recurrence.The authors declare that they have no competing interests.Drs ABG and LG: conception and design of the study. Dr RM: acquisition of data. Dr MS: drafting of the manuscript. Drs AL-B and LC: critical revision of the manuscript for important intellectual content. Dr RM: supervision. All authors read and approved the final manuscript.


We report a rare case of solitary Langerhans cell histiocytosis (LCH) involving the clavicle of an adult female. The patient was a 32-year-old female presenting with 1 month history of progressive pain, swelling, and tenderness in the region near the left sternoclavicular joint. Radiograph, computed tomography, and magnetic resonance imaging showed an osteolytic lesion in the clavicle with tumor extension and soft tissue edema. Surgical curettage of the lesion was performed, and the histopathologic diagnosis was LCH. Because of its rarity and possibly variable presentation, LCH should be included and considered in the differential diagnosis when we encounter a clavicle lesion. One of the most frequent presenting imaging features in adults is skeletal involvement with lytic lesions,1 but clavicle lesions are extremely rare, especially in adults.4 Till now, almost no related articles report characteristic imaging of LCH in clavicle in adults. We report 1 female adult patient with solitary clavicle EG, with a literature review.Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology with an estimated annual prevalence of 1 case per 560,000 in adultsA 32-year-old female adult presented with 1 month history of progressive pain, swelling, and tenderness in the region near the left sternoclavicular joint. The patient denied fever, chills, night sweats, weight loss, and fatigue. There was no history of trauma. The posterior–anterior radiograph showed ill-defined osteolytic lesion without sclerotic margin in the left clavicle with soft tissue swelling above the lesion , CD35 (+), CD1a (++), CD68 (+), VIM (++), Ki-67 (30%+), and Langerin (+) Figure . A diagn6 LCH accounts for <1% of tumor-like lesions of bone7 and the most frequent site is the skull and jaw, followed in decreasing order of frequency by long tubular bones, pelvis, ribs, spine, scapula, and clavicle.8 Clavicle lesions are extremely rare.10LCH is an abnormal proliferation of tissue macrophages called Langerhans cells in 1 or more organs, including skin, lymph nodes, lung, liver, spleen, bone, and bone marrow. Patient age ranges from 5 to 15 years in about 90% of the cases with a slight male predominance.14 Ewing sarcoma and plasmacytoma often show permeative bone lesion with large soft tissue mass. Aneurysmal bone cyst can be geographical with sclerotic margin whereas myeloma can be geographical with ill-defined margin. Desmoid tumor is often with spherical bony outgrowth, no soft tissue mass, and no intraosseous extension. Chondrosarcoma may show huge soft tissue mass with permeative lesion.13 Osteomyelitis should be included for differential diagnosis and usually shows ill-defined bone destruction and sclerosis, expansion, and lamellated subperiosteal new bone formation.14 Although radiologic characteristics of the bony lesion indicated a diagnosis of LCH in the current case, we should stress that a diagnosis of LCH can only be finally made on histopathologic findings of a biopsy. An arrangement of histiocytosis in loose mesh-works or clusters and immunoreactivity for S-100 and CD1a antigens are helpful for the diagnosis of LCH.15 Because of its rarity and possibly variable presentation, the diagnosis of LCH may be overlooked or neglected. When we encounter a clavicle lesion, it might be difficult to remember the possibility of LCH.Our patient was a female adult with a solitary lesion in the left clavicle. The plain radiograph showed an osteolytic lesion of the clavicle. CT demonstrated the proximal part extension of the lesion with bone residual and without sclerotic margin confirming disruption of the cortex and extension of lytic lesion. MRI showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. A wide variety of bone lesions may mimic EG. Tumors and tumorous lesions of the clavicle should be excluded, such as Ewing sarcoma, aneurysmal bone cyst, desmoid tumor, myeloma, plasmacytoma, chondrosarcoma, and osteomyelitis.16 In contrast, multifocal and multisystem types of LCH are generally treated with chemotherapy, in combination with other therapeutic modalities. In this patient, surgical curettage and internal steel-plate fixation were performed.Treatment of LCH depends on the extent of the disease. Various forms of treatment for a solitary lytic lesion affecting bones have been attempted, which include curettage, local steroid injection, radiotherapy, and chemotherapy alone or in combination. The results of treatment of solitary lesions are always satisfactory, although recurrence occurs in some patients (11%).


Embryonal rhabdomyosarcoma (ERMS) of the adult urinary bladder is a rare malignant tumour. Inflammatory myofibroblastic tumour (IMT) of the bladder is a benign genitourinary tumour that may appear variable histologically but usually lacks unequivocal malignant traits. Techniques like flow cytometry and immunohistochemistry may be used to differentiate these two tumours. Our patient, a 46-year-old male, had rapidly recurring lower urinary tract symptoms after two transurethral resections of the prostate. He subsequently underwent a transvesical prostatectomy which showed IMT on histology. However, his symptoms did not resolve and an open resection done at our institution revealed a 6 cm tumour arising from the right bladder neck. This time, histology was ERMS with diffuse anaplasia of the bladder. Rapid recurrence of urinary symptoms with prostate regrowth after surgery is unusual. Differential diagnoses of uncommon bladder malignancies should be considered if there is an inconsistent clinical course as treatment approaches are different. Embryonal rhabdomyosarcomas (ERMSs) are malignant soft tissue tumors that are thought to originate from immature cells destined to form striated skeletal muscle. However, these tumors can arise in locations where skeletal muscle is not typically found, for example, in the urinary bladder. In paediatric patients, 20% of rhabdomyosarcomas (RMSs) occur in the genitourinary tract and this accounts for 6% of all childhood malignancies . In adulInflammatory myofibroblastic tumors (IMTs) of the urinary tract, also termed postoperative spindle cell nodule, inflammatory pseudotumor, and pseudosarcomatous fibromyxoid tumor (PSFMT), are uncommon tumors of the genitourinary tract, with the largest series of 46 cases published by Montgomery et al. . AlthougHere we describe a case of an ERMS misdiagnosed as an IMT/PSFMT. Because IMTs may resemble low-grade sarcomas and vice versa from a histologic point of view, a surgeon should always correlate clinical judgment with histopathology to reach an accurate diagnosis. This is crucial in order to prevent inadequate treatment for a malignant tumor or unnecessary radical therapy for a benign lesion.Mr X, a 46-year-old male, was referred from overseas to our institution for recurring lower urinary tract symptoms suggestive of benign prostate enlargement (BPE) after transurethral resection of prostate (TURP).He initially presented at the age of 43 years with voiding symptoms such as poor stream, hesitancy, incomplete bladder emptying, and terminal dribbling. He underwent TURP in May 2010 with good resolution of his symptoms. The pathology was reported as benign nodular hyperplasia of the prostate (BPH). However, the symptoms recurred 9 months later and progressively worsened. He sought treatment from the same urologist and was diagnosed to have recurrence of his BPE. Second TURP was performed. Again, the histopathology was reported as BPH. His urinary symptoms again resolved after the second TURP but recurred just 3 months after surgery. He then underwent a transvesical prostatectomy. The histopathology was reported as PSFMT. His symptoms recurred in a similar fashion after 3 months, after which he decided to seek a second opinion at our institution.A computed tomogram of his urinary tract performed at our institution revealed a tumour arising from the base of the bladder with possible involvement of the prostate Figures  and 2.The patient underwent an open resection of his bladder/prostate tumour. A 6 cm bladder tumour  was founThe patient was advised to commence on chemotherapy with the standard vincristine, actinomycin-D, and cyclophosphamide (VAC) regime followed by radiotherapy, but he decided to return to his home country for further treatment.The histology of IMT and RMS is similar and may be confused with one another . IMT is Rhabdomyosarcoma, on the other hand, is composed of typical rhabdomyoblasts arranged in sheets and large nests, with infrequent intermixed fusiform cells. They contain small primitive undifferentiated cells characterized by a high nuclear-to-cytoplasmic ratio. Ro et al.  describeRhabdomyosarcoma can be differentiated from IMT based on morphological features  and the application of an immunohistochemical panel that includes MyoD1 or myogenin  Figure . MyogeniFlow cytometry can also be used to differentiate between IMT and ERMS as most cases of IMT are diploid with low S-phase fraction features which suggest benign lesions , 5 and EA learning point for this case is that the rapid recurrence of the symptoms and regrowth of the prostate is inconsistent with the benign nature of IMT. To avoid misdiagnosis, a search for associated carcinoma should be taken. For our patient, after 2 surgeries for presumed benign prostatic hyperplasia, a histological diagnosis of pseudosarcomatous fibromyxoid tumour is a little surprising as it does not match the slow growing benign nature of the condition. A high index of suspicion is warranted here.It may be difficult to differentiate these 2 disparate histological entities as presenting symptoms can be similar with gross haematuria being the most common symptom. Lower urinary symptoms are usually present as well. Inflammatory pseudotumors are benign, slow growing lesions that do not metastasize or undergo malignant transformation. Local recurrences are rarely observed after either endoscopic or open excision. In this case, the rapid recurrence of lower urinary tract symptoms after TURP is unusual and one must consider differential diagnoses of primary or metastatic urothelial carcinomas or sarcomatoid carcinoma of the bladder/prostate as well. Clinical context is essential in raising suspicion in order to make an accurate diagnosis.It is believed that the best management for IMTs is complete surgical extirpation with preservation of normal tissues at the surgical margin , 9. OtheAs ERMSs are very rare in adults, most of the current knowledge is extrapolated from Intergroup Rhabdomyosarcoma Study (IRS) results which are case series from the paediatric population. The staging system used in IRS I–III has gained worldwide acceptance and is commonly used . Adult gIn adults, bladder RMS may demonstrate alveolar or unclassified histology, commonly with anaplasia, and there is uniformly aggressive course. In the reported cases, the patients' age ranges from 23 to 85 years and none had associated CIS of the bladder. Morphologically, pure RMS in adult bladders consists of primitive undifferentiated round cells without obvious rhabdomyoblast differentiation and with varying degrees of anaplasia. Only 4 out of the 13 reported cases of RMS are of embryonal variant and the subtype heterogeneity of these 13 cases suggests that pure RMSs of adult bladders are likely different from the paediatric tumors . For IRSAlthough data is limited for adults, complete excision should be considered for localized disease as long as functional and/or cosmetic results are acceptable. Vulvar, vaginal, uterine, bladder, and prostatic RMSs in paediatric patients usually respond well enough to induction chemotherapy to render them locally resectable, often with clear resection margins . In a reRadiotherapy is also a major tool for the treatment of RMS, particularly for achieving local control in patients with residual microscopic or gross disease following surgery and chemotherapy . BecauseRapid recurrence of lower urinary tract symptoms with rapid prostate regrowth after TURP is unusual and differential diagnoses of unusual bladder malignancies should be considered. A histological diagnosis of pseudosarcomatous fibromyxoid tumour should be viewed with caution if the clinical course of the disease is inconsistent.