Primary colorectal lymphoma represents a rare minority among the colonic neoplasms. Early diagnosis is often difficult because of unspecific symptoms, with subsequent delays in diagnosis and management. We describe a rare case of colonic lymphoma presenting with synchronous liver metastasis.A 70-year-old male with a 6-mo history of vague abdominal pain, constipation and melena was referred to our hospital. Computed tomography scan of abdomen revealed the presence of a mass along the proximal ascending colon. Colonoscopy biopsy showed external compression of the cecum with two ulcerations of mucosa, but it was not consistent for a definitive diagnosis. Because the difficulties in the preoperative pathological diagnosis, the high risk of bowel obstruction and the correlated hemorrhagic risk, the patient underwent a right hemicolectomy associated with locoregional lymphadenectomy and liver resection.The surgically resected right colon and liver tumors were all immunohistochemically diagnosed as diffuse large B-cell lymphomas (DLBCL). The patient refused any other antineoplastic treatment; he is alive and free of disease at 3 years after initial diagnosis.Primary colonic lymphomas represent a rare minority among the colonic neoplasms. Their correct pre-operative identification is crucial for the design of treatment. This case highlights the difficulty in diagnosing of primary colonic lymphoma. To our knowledge, this is the first report of a colonic lymphoma presenting with a colonic mass and a synchronous liver metastasis. Primary colonic lymphoma (PCLs) is rare comprising 10–20% of all gastrointestinal lymphomas and less than 1% of large bowel malignancies. It is the third most common large bowel malignancy after adenocarcinoma and carcinoid [A 70-year-old male presented with a 6-mo history of vague abdominal pain. The patient also complained of constipation, fatigue, weight loss and melena.The patient’s history included repaired right sided inguinal hernia, pulmonary fibrosis, hypertension and angina pectoris which was treated using coronary stents 5 months ago and dual antiplatelet therapy. He underwent a colonoscopy 6 years before, and the result was negative for colorectal diseases. The abdomen was soft, tender to palpation over the right and lower quadrant. Clinical examination on admission revealed the existence of a palpable mass in the right iliac fossa. Laboratory data, including tumor markers and leucocitary formula, were all within the normal limits, except for the hemoglobin level (hgb 8.8 g/dl). A colonoscopy revealed external compression of the cecum and mucosa appears edematous with two hemorrhagic ulcerations Figure . BiopsieComputed tomography guided tru-cut biopsy of the liver nodule was not performed because of the rapid deterioration of the overall health status of patient. We didn't have repeat a new colonoscopy because of the patient's refusal.During hospital stay, the patient presented a new episode of rectal bleeding and signs of hypovolemia such as tachycardia, hypotension, and low urine output. So blood transfusions were performed to replace blood lost.Both the rapid health deterioration of patient and the high hemorragic risk associated with the risk of bowel obstruction prompted us to perform a right hemicolectomy associated with loco-regional lymphadenectomy. Under Pringle's maneuver a limited anatomic resection of segment 6 was performed.Gross examination of the resected specimen showed a distinct lesion in the cecum. Microscopic examination revealed diffuse large B cell lymphoma of the cecum and portions of colonic tissue infiltrated by the neoplasm.The cells were monotonous with irregular nuclear membranes and prominent nucleoli with easily found mitotic activity. Immunohistochemical staining was also performed and revealed the tumor to be CD10+, CD3+, CD79+ Figure  and negaThe liver resected specimen revealed a metastasis of non-Hodgkin's lymphoma with central necrosis. Morphologic and immunohistochemical results confirmed the diagnosis of synchronous presentation of diffuse large B-cell lymphomas with mesenteric lymph nodes and a single liver metastasis. He was discharged home 12 days after his operation.Four weeks after surgery, the patient underwent bone marrow biopsy for a further evaluation of the disease. Bone biopsy was negative for neoplastic involvement.At 3-year follow-up, patient is alive and free of disease.The colon is an uncommon site of involvement of Non-Hodgkin’s lymphoma (NHL). The most frequently involved colonic site at diagnosis is the ileocaecal region , followed by the caecum, the sigmoid and the rectum. Clinical presentation of colonic lymphoma is quite vague and variable. The most common symptoms of colonic lymphoma are abdominal pain and weight loss with a palpable mass identified on physical examination. Primary colonic lymphomas usually occurs between the fourth and seventh decade of life.The etiology of DLBCL is unknown, but some risk factors and predisposing conditions have been identified such as immunodeficienty and inflammatory bowel diseases . DespiteIn general, the diagnosis of colonic lymphoma could be based on the CT appearance of extensive abdominal and/or pelvic lymphadenopathy. Even in the absence of lymphadenopathy, characteristic images such as location at the cecum, demarcation from the peri-colonic fat with no invasion of surrounding viscera and the presence of perforation in the absence of desmoplastic reaction should raise the suspicion of a lymphoma. Fisher et al. suggest that without the presence of enlarged lymph nodes it is difficult to distinguish this type of tumor from primary adenocarcinoma . RadioloWang et al. showed that the morphology of primary colonic lymphoma (PCL) is variable, resulting in a wide range of endoscopic features, including mass lesions, narrowing of the lumen, ulceration, mucosal irregularities, and aphthous lesions . Wyatt rFurthermore for B-cell lymphomas, fungating mass is the most common endoscopic type (54.0%) .The different presentations of colonic lymphoma could be better revealed by the use of double contrast barium enema technique. The radiologic changes may be similar to those found in Crohn's disease, amoebiasis or pseudomembranous colitis ; they caThe diagnosis of primary colonic lymphomas (PCL) was initially established in 1961 and included the following diagnostic five criteria :1. No palpable superficial lymphadenopathy;2. No enlargement of the mediastinal lymph nodes on chest X-ray;3. Normal white blood cell count;4. Predominance of the bowel lesion, and adjacent lymph nodes affected at laparotomy;5. Absence of any tumor in the liver or spleen at laparotomy.Our patient's disease met all these criteria preoperatively, except for the liver lesion detected by abdominal CT scan, but the radiologic characteristics of this lesion were similar to those of liver metastases from colorectal cancer. Abdominal computed tomography (CT) images revealed one hypovascular space-occupying lesions with diameters of 3.5 cm with internal heterogeneity due to a relative lack of effect of the contrast medium in the liver , as our case reported . Other hColonic biopsies performed by our endoscopist were non-diagnostic and the diagnosis was made postoperatively after colonic resection. For definitive diagnosis is crucial to define morphology and immunophenotyping. Diffuse large B-cell lymphoma cells generally express pan B cell markers such as CD20, CD19, CD22, CD45 and CD79a; seventy percent of tumor cells express BCL-6 protein; CD10 is expressed in 30 to 60% of cases . In our In our case, the lymphoma presented as a colonic mass on colonoscopy, but it was not obvious on CT examination. We didn't perform double contrast barium enema because of the patient's refuseal. Some authors have reported rare cases of emphysematous colitis  as the fFuture advancements in molecular genetics, tumor analysis, and immunohistochemistry could led to most effective management strategies for patients with colonic lymphoma.Accurate staging of bone marrow status is crucial to optimize the therapeutic strategy. Bone marrow biopsy (BMB) remains the gold standard to determine bone marrow involvement (BMI) but has poor sensitivity because the sample size may be small or the BMI focal .Treatment of colorectal lymphoma usually involves chemotherapy, radiation, surgery or a combination of these. Quayle et al. reported that radiation therapy might not be the preferred option for the treatment of colonic lymphoma, because of a high risk of complications involving the small and large bowels . NowadayThis case is the first report of colonic lymphoma arising from the cecum with synchronous liver metastasis. It was impossible to achieve a correct preoperative diagnosis and the indication for surgery was supported by the high hemorrhagic risk associated with the risk of bowel obstruction and by rapid deterioration of the overall health status. In our case, both tomography and colonscopy showed findings mimicking colonic adenocarcinoma.Colonic lymphoma is extremely rare and the variable imaging tests are non-specific; the diagnosis is rarely made before surgery and usually confirmed by histopathological investigation after surgery. This kind of tumour should be considered in the differential diagnoses of colon mass with with enlarged lymph nodes in the abdomen or pelvis and synchronous liver nodules when the biopsies obtained during the colonoscopy were not diagnostic.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.The authors declare that no conflict of interest to declare.PI and ML performed the surgery and has given final approval of the version to be published; DR participated to surgery and wrote the manuscript; RP conducted a literature search and contributed to drafting the manuscript; AD, and FC participated in the acquisition and interpretation of radiological data; DA and CM carried out the histological and bio-molecular studies; LN and RC supervised the draft and cared the patien’s follow-up. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2482/14/75/prepub


Parapharyngeal space (PPS) tumors are very rare, representing about 0.5% of head and neck neoplasms. An external surgical approach is mainly used. Several recent papers show how transoral robotic surgery (TORS) excision could be a prospective tool to remove mainly benign lesions in PPS; no cases of neurogenic tumors from the retrostyloid space treated with TORS have been reported. We present two cases which underwent TORS for schwannomas from the retrostyloid compartment of the parapharyngeal space. Clinical diagnosis of schwannoma was performed by magnetic resonance imaging (MRI). In the first case a 6 cm neurogenic tumor arose from the vagus nerve and in the second case a 5 cm mass from the sympathetic chain was observed. Both cases were treated successfully by the TORS approach using a new “J”-shaped incision through the mucosa and superior pharyngeal constrictor muscle. Left vocal cord palsy and the Claude Bernard Horner syndrome, respectively, were observed as expected postsurgical sequelae. In case 1 the first bite syndrome developed after three months, while no complications were observed in case 2. Both patients regained a normal swallowing function. TORS seems to be a feasible mini-invasive procedure for benign PPS masses including masses in the poststyloid space. Parapharyngeal space (PPS) tumors represent 0.5% of head and neck neoplasms , 2. The Schwannoma is the most common tumor of the PPS, arising more frequently from the cervical sympathetic chain or from the last four pairs of cranial nerves of the retrostyloid compartment –6.About 80% of schwannoma cases are diagnosed by magnetic resonance imaging (MRI). MRI is the most sensitive and specific tool for identifying the origin of PPS masses , 8.Different surgical approaches for PPS neoplasms have been reported  , 9.Recently, TORS has emerged as a prospective tool to remove benign and small-sized lesions in PPS in the light of the restricted working space and the risk of injuries to the structures of the retrostyloid compartment. To our knowledge no author has ever applied TORS for the excision of neurogenic tumors arising from the retrostyloid compartment.In this report we present two schwannomas of the retrostyloid compartment excised with TORS using a new “J”-shaped incision through the mucosa and superior pharyngeal constrictor muscle to obtain a wider vision of the structures of the upper portion of the PPS.We present two consecutive patients affected by schwannoma of the retrostyloid compartment of PPS diagnosed with MRI. Patients underwent TORS at the Head and Neck Division of the European Institute of Oncology, Milan.A 39-year-old man, affected by vertigo, had undergone an MRI scan with an incidental finding of an unknown mass arising from the left parapharyngeal space. No symptoms were reported.Oral examination showed a soft and painless bulge on the left posterior tonsillar pillar. The overlying mucosa was smooth and undamaged. On laryngoscopy, the mass extended downwards into the hypopharynx without involving the larynx. Its inferior edge was palpated just below the left angle of the mandible and presented as firm and nonfixed to the surrounding tissues.Patient 1 referred to our institute with a neck ultrasound which showed an oval, hypoanechoic mass in the left parapharyngeal space, with peripheral vascular spots at US-Doppler evaluation. Ultrasound-guided fine-needle aspiration cytology (FNAC) was not diagnostic. Preoperative MRI scan showed a 48 × 39 × 25 mm well-defined mass located in the left retrostyloid PPS. The internal carotid artery (ICA) and external carotid artery were both displaced anterolaterally while the internal jugular vein (IJV) resulted displaced posterolaterally .A 45-year-old woman presented a left submandibular swelling persisting for the last three years. Three years previously, the patient had undergone an ultrasound-guided FNAC, whose diagnosis was suggestive of a benign neurogenic tumor. She underwent an MRI examination which confirmed the presence of a 29 × 20 × 19 mm ovoid mass occupying the left retrostyloid PPS.She refused surgery and decided with her specialist, upon a radiological followup, to undergo an MRI scan every year.When the patient first referred to our clinic, the lesion had grown by approximately 5 mm. On physical examination the swelling was firm, mobile, nontender, and painless.On videolaryngoscopy the hypopharynx and larynx were normal.The last MRI scan showed an ovoid mass occupying the left parapharyngeal space, 25 × 23 × 35 mm in size (versus 20 × 19 × 29 mm in 2010), with homogeneous contrast enhancement, low signal intensity on T1-weighted imaging, while it demonstrated increased T2-weighted signal intensity. The IJV and the ICA artery were both laterally displaced .Both patients underwent preoperative MRI which showed sharply circumscribed ovoid masses with smooth margins, located in the retrostyloid PPS Figures  and 2.Both lesions showed a low signal on T1-weighted sequences, a high signal on T2-weighted sequences, and intense contrast enhancement, uniform in patient 1, slightly inhomogeneous in patient 2, on T1-weighted sequences after gadolinium injection.The absence of high-velocity flow voids inside the lesions helped in the differential diagnosis with paragagliomas in both cases.In patient 1 the lesion displaced the ICA in the anterolateral direction and the JV in the posterolateral direction resulting in the separation of the two vessels. Its upper pole was adjacent to the jugular foramen .In patient 2 the lesion displaced both the ICA and the JV in the lateral direction with no significant separation between the two vessels .According to the classification proposed by Furukawa et al. the supposed nerve of origin on the basis of MRI findings was the vagus nerve in patient 1 and the sympathetic chain in patient 2 .Patients underwent general anaesthesia via nasotracheal intubation. The Da Vinci system with three arms was used . A bedside assistant was ready to suction and cauterise in case of intraoperative bleeding.Transoral exposure was obtained with a Feyh-Kastenbauer retractor .A 4 cm “J”-shaped incision through the mucosa and superior pharyngeal constrictor muscle was made using the monopolar cautery. Instead of performing the usual vertical incision, we decided to introduce this new modified “J”-shaped incision, to obtain a wider vision of the upper portion of the PPS .After the incision of the mucosa, superior constrictor muscle, and pharyngobasilar fascia the tumour appeared as well-capsuled, hard, and elastic in consistency. The ICA had been anterolaterally displaced in both cases. The mass was gently detached from the surrounding fascia, preserving the capsule and separating it carefully from the ICA, with a spatula-tipped arm .In both cases the nerve of origin was identified and resected. The masses were removed entirely without capsular rupture.A thin haemostatic patch was positioned over the ICA. The surgical wound in both patients was sutured with absorbable suture.In patient 1 a tracheotomy was performed to prevent blood inhalation in case of postoperative bleeding and removed on the third postoperative day, while in patient 2 it was not considered necessary.The mean operative times for patient 1 and patient 2 were 254 min and 120 min, respectively. The time of hospitalization was 8 days for patient 1 and 7 days for patient 2. No nasogastric tube was needed. Liquid diet was possible from the day after surgery.The followup time was 7 months for patient 1 and 5 months for patient 2. Both patients are satisfied with no external visible scars and free from recurrences Figures .As expected, patient 1 developed paralysis of the left vocal cord and patient 2 developed Horner's syndrome.The histological examination confirmed the diagnosis of schwannoma in each patient, originating from the left vagus nerve and the left sympathetic chain, respectively. After three months patient 1 developed first bite syndrome   while inSurgical excision is the treatment of choice for schwannomas of PPS. Slow growth and the noninvasive nature of schwannomas also allow an observational approach . TreatmeAn accurate preoperative identification of the nerve of origin, therefore, allows patients to make an informed decision on whether to undergo operation or observation .The differential diagnosis must consider salivary tumors, metastatic lymph nodes, or soft tissue neoplasms: paragangliomas, aneurysms, branchial cleft cyst, or neurogenic tumors .The surgical approach must guarantee a wide view of the surgical bed for a radical dissection of the tumor with the lowest risk of injuries to the vital structures and with minimal functional/cosmetic impairment , 13.The “TORS in PPS” debate deals with its limited exposure of the tumor and the risk of tumor spillage/fragmentation. O'Malley et al. reported a successful excision of a benign salivary cyst of the PPS and 10 benign salivary neoplasms excised with TORS , 15. ConDucic et al. reported a transoral approach to the poststyloid compartment concluding that TORS may not provide a full intraoperative control of lesions behind the ICA . ConversTwo cases of PPS schwannomas excised with TORS alone have been reported, but none of them arose from the poststyloid compartment. It is yet to be verified whether TORS would be suitable for neurogenic tumors of the poststyloid compartment , 19.To our knowledge this is the first paper reporting the safety and feasibility of TORS applied to neurogenic tumors arising from the poststyloid compartment.In our series both tumors were radically removed and patients expressed complete satisfaction with their cosmetic and functional results. Patients 1 and 2 presented a left vocal cord palsy and Claude Bernard Horner syndrome, respectively.Postsurgical sequelae were expected due to the resection of the X cranial nerve and cervical sympathetic trunk, performed in order to achieve a radical excision of the tumor. These neurologic symptoms are also reported with external approaches and as Lee et al. reported the absence of any neurologic deficit can be related only to schwannomas arising from the prestyloid compartment and originating from peripheral trigeminal branches .Patient 1 also reported “first bite syndrome.”First bite syndrome is described as a sudden onset of intense head and neck pain in the parotid region, sometimes extended to the homolateral neck and ear, with no prodromal symptoms triggered by the first bite and then gradually disappears. It is caused by sympathetic-parasympathetic conflict on the myoepithelial cells of the parotid region; in 95% of cases it follows upper neck surgery .We speculate that in our case the first bite syndrome was subsequent to the loss of sympathetic innervation caused by an injury to the cervical sympathetic trunk. A vertical incision on the anterior tonsillar pillar through the superior pharyngeal constrictor muscle is usually reported , 20. InsOur experience confirmed that TORS may overcome the limitations of visual and instrumental access of the classical transoral approach. The vital structures can be magnified and easily preserved. This technique permitted a clear identification of the tumor capsule and its delicate dissection. The 360-degree motion of the robotic system provided excellent manipulation of tissues to facilitate dissection, especially in the lateral plane .For this reason, TORS is a feasible procedure for neurogenic tumors of the poststyloid compartment. A “J”-shaped incision on the anterior tonsillar pillar allows a wider and more detailed three-dimensional view of the structures in the upper portion of the PPS. Morbidity due to injuries to the neurovascular structures appears to be acceptably similar to external approaches but TORS does enable functional and cosmetic impairments to be avoided and carries a lower risk of adverse wound-healing events.


Convex endobronchial ultrasound (C-EBUS)–guided transbronchial needle aspiration (TBNA) is an effective tool for the diagnosis of hilar, mediastinal, and central parenchymal lung lesions. However, it has a limited utility for pleural-based masses. We report a unique case of a pleural synovial sarcoma recurrence that was diagnosed by C-EBUS.The patient had a history of inguinal synovial sarcoma. He presented with cough and chest pain. Imaging of chest revealed large right pleural mass. Bronchoscopy with EBUS-TBNA diagnosed pleural recurrence of synovial sarcoma. He underwent radical resection and pathological examination confirmed the diagnosis of pleural synovial sarcoma. He experienced complete recovery and resolution of symptoms.Synovial sarcoma should be included in the differential diagnosis of pleural masses. Convex EBUS-guided biopsies can provide adequate diagnosis of large pleural tumors adjacent to the central airways without need for more invasive diagnostic procedures. Ethics committee or institutional review board approval was not obtained. It was not necessary for the case report. Informed consent was obtained from the patient.A 50-year-old man with history of inguinal synovial sarcoma resected 3 years prior presented for right-sided chest pain and a persistent cough of 3 months duration. Chest x-ray showed an almost complete whiteout of the right lung. CT scan of the chest showed a very large, multilobed, pleural-based tumor collapsing most of the right lung as well as small mediastinal and hilar lymphadenopathy Fig. . He unde1 Approximately, 11,000 cases are diagnosed in the United States every year. There are over 100 different histologic subtypes; synovial sarcomas are one of the more common ones. The most frequent sites of initial involvement include lower extremities, head and neck, trunk, upper extremities, mediastinum, and abdomen. These tumors have a high risk of metastases and a high frequency of recurrence.Fibrosarcomas are soft tissue tumors of fibroblastic origin. Once considered to be the most frequent type of sarcomas, they have since then been found to be exceedingly rare and constitute only about 1% of all soft tissue sarcomas.2 The most frequent site of metastasis was by far the lung parenchyma, followed by local recurrence, then bones and lymph nodes. Other smaller series reported similar findings.3 The pleural space has not been reported as a site of metastasis of these tumors. This is the first unique feature of this case. Secondly, and perhaps more interestingly, an adequate diagnosis was made by endobronchial ultrasound. EBUS-TBNA has emerged in the past few years as an effective tool to diagnose and stage a large number of chest malignancies. It allows sampling of mediastinal, hilar, and proximal endobronchial masses,5 but its utility for reaching pleural lesions is limited. This is mainly due to the thickness of the scope which usually limits its ability to enter the subsegmental airways. There have been multiple reports of diagnosis and staging of pleural malignancies with EBUS; however, this was possible only indirectly, through transbronchial aspiration of enlarged mediastinal lymph nodes and not the pleural mass itself.7 Only 1 case of endobronchial sampling and diagnosis of a pleural-based mesothelioma is reported in the literature.8 Although typically transthoracic biopsy would have been the first step in evaluation of our patient's tumor, we elected to start with flexible bronchoscopy for 2 reasons: First, the transthoracic biopsy of pleural sarcomas is known historically to be often difficult with a low yield. Second, and most importantly, we wanted to assess for endobronchial involvement given the size and near total collapse of the lung and evaluate ipsilateral and contralateral lymph nodes for staging purposes. Fortunately in our case the mass was large enough to be at proximity to the airways and was visualized by EBUS. The samples obtained allowed an adequate diagnosis of the histology of the mass and helped guide the next steps of management without the need for more invasive procedures.Several large series have been published looking at sites of recurrence. The largest one comes from the National Cancer institute in 1985.9 Other authors have discussed that it complements and may one day replace the use of blind transbronchial biopsies.10 Our case describes yet another clinical application of EBUS and adds further to the rapidly growing body of the literature supporting its utility in diagnosing both malignant and nonmalignant diseases of the chest.11 Traversing the visceral pleura transbronchially appears to be safe but certainly careful postprocedural monitoring of these patients is warranted because of the added risk of iatrogenic complications.In a recent review article from Great Britain, José et al concluded that since the advent of EBUS, fewer patients needed to undergo mediastinoscopies for diagnosis and staging of lung cancer.Synovial sarcoma should be included in the differential diagnosis of pleural masses. Convex EBUS-guided biopsies can provide adequate diagnosis of large pleural tumors abutting proximal airways without need for more invasive diagnostic procedures.


Mucoceles are mucus-filled, epithelial-lined sacs that slowly develop in the paranasal sinuses when sinus or concha bullosa drainage is obstructed by inflammatory processes, trauma, or prior surgery. They are extremely rare in children. Symptoms usually arise from the nasal obstruction or compression of neighboring structures.This case report describes a 5-year-old Turkish boy with a 3-year history of nasal obstruction. A computed tomography scan showed a well-defined soft tissue density lesion, seemingly originating in the region of the middle concha and was suggestive of a middle concha mucocele. The mass was removed by endoscopic sinus surgery.In the case of a child presenting with nasal obstruction, mucocele should be remembered in the differential diagnosis of intranasal tumors. Computed tomography and magnetic resonance imaging are helpful in making the diagnosis and endoscopic nasal surgery has proven successful in the treatment. Langenback first described mucoceles in 1819, but it was Rollet who introduced the term mucocele in 1896. Onodi described the histological characteristics and Turner differentiated the frontal from the ethmoidal lesion . The eti3/mm3 (range: 4 to 10×103/mm3), hemoglobin level 13.4g/dL (11 to 16g/dL), platelets 361×109/L (range: 150 to 400×109/L), and sedimentation rate 9 (range: 3 to 20mm/hour). A high resolution computed tomography (HRCT) scan showed a well-defined soft tissue density lesion, seemingly originating in the region of the middle concha and was suggestive of a middle concha mucocele , which was aspirated on the tenth postoperative day. The postoperative period was uneventful without any complications or synechia formation. His nasal obstruction improved remarkably in the immediate postoperative period. A postoperative HRCT examination was performed 2 months after surgery. There were no signs of recurrence or inflammation . MRI is helpful in clarifying soft tissue extension to the cranium and orbit. It also helps in differentiating from other soft tissue masses with potentially similar presentation such as meningocele, rhabdomyosarcoma, hemangioma, and neuroblastoma ,10,11. IAll cases of mucocele should be treated surgically. Two methods may be used in the treatment of paranasal sinus mucocele. One of them, conservative minimally invasive surgery, involves endoscopic marsupialization of the mucocele. This approach preserves the nasal anatomy and architecture. The other approach is the more radical transfacial approach and involves a Lynch–Howarth incision that is used mostly in cases with intracranial extension ,13. In oThe cyst is mainly lined by pseudostratified ciliated columnar or cuboidal epithelium, but sometimes it may be lined with areas of squamous epithelium. There is cellular infiltrate comprising neutrophils, lymphocytes, plasma cells, and eosinophils. Subepithelial lymphoid aggregates and vascularity are also seen. Osseous elements are woven bone, lamellar bone, osteoblasts, and osteoclasts . Our hisMucoceles are rare in the pediatric age group. A mucocele may become large enough to make a unilateral or bilateral nasal obstruction. Therefore, in the case of a child presenting with nasal obstruction, mucocele should be remembered in the differential diagnosis of intranasal tumors. HRCT and MRI are helpful in making the diagnosis and endoscopic nasal surgery has proven successful in the treatment.Written informed consent was obtained from the parents of our patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GA performed the surgical procedure and obtained the patient data and was a major contributor in writing the manuscript. MBU was a major contributor in writing the manuscript. NB performed the pathologic examination and was a contributor in writing the manuscript. All authors read and approved the final manuscript.


A 37-year-old woman, previously healthy with no history of trauma, presented with a scalp swelling of the left frontal region. The swelling was present for the last one year and had gradually increased in size with recent localized headache. The patient had no neurologic deficit. Local examination showed a painful local swelling of about 25 mm in diameter in the left frontal area and appeared to arise from the bone. Hematological and biochemical investigation results were within the normal range. Computed tomography scan revealed a left frontal intradiploic mass with bone defect (A). Magnetic resonance imaging showed that the lesion was soft with homogeneous enhancement following gadolinium injection. There was erosion of both inner and outer table of the cranial vault with extra cranial and intracranial extradural extension (B and C). The tumor was excised completely and the surrounding bone was removed (D), followed by cranial reconstruction. There was no intradural extension of the lesion. Histologically, the tumor was diagnosed as meningothelial meningioma. The patient has been well for 1 year following the operation with no evidence of recurrence. Meninigiomas are primary tumors of arachnoids cell layers and the lesions are in purely settled intradural locations. Meningiomas in extradural locations are very rare and should be considered in the differential diagnosis of osteolytic skull vault lesions.


Ischemic pancreatitis is a rare medical entity. The pancreatic tissue is susceptible to ischemia with the possibility of developing acute pancreatitis. The abdominal aortic aneurysm can be one possible cause of pancreatic hypoperfusion.We report the case of a 68-year-old man, Caucasian, with a history of a cluster of severe cardiovascular conditions, who presented epigastric pain of variable intensity for about 2 weeks. The pain occurred after intense physical effort, and was associated with anorexia and asthenia. The palpation revealed epigastric pain and palpable pulsatile mass above the umbilicus. Laboratory tests showed increased serum and urine amylases. The abdominal contrast-enhanced CT scan evidenced acute lesions of the pancreas and a caudal pancreatic pseudocyst of 39x24 mm. An abdominal aortic aneurysm was also described  with a maximum diameter of 60.5 mm and generalized atheromatosis. By corroborating clinical, anamnestic, laboratory and imaging data, the case was diagnosed as moderately severe acute ischemic pancreatitis, pancreatic pseudocyst, abdominal aortic aneurysm, generalized atheromatosis. The pancreatic pseudocyst was resorbed in eight months. Surgery for the abdominal aneurysm was performed after the resorption of the pseudocyst. The patient died after aortic surgery because of a septic complication.Ischemic pancreatitis is a rare condition but should be considered in a patient with upper abdominal pain and elevated amylase in the context of an abdominal aortic aneurysm and generalized atheromatosis. The most frequent etiological factors of acute pancreatitis are gallstone disease and alcohol consumption, followed by hyperlipemia, pancreatic malformations, autoimmunity, etc. . PancreaWe present the case of a 68-year-old male patient, Caucasian, living in an urban environment, who was admitted in emergency for epigastric pain. His personal history included duodenal ulcer 20 years ago, coronary heart disease, heart failure diagnosed 6 years ago, with PTCA and stent implantation in the anterior interventricular branch of the left coronary artery 5 years ago, exertional angina pectoris, dilated cardiomyopathy, hypertension risk grade III, hypertensive cardiomyopathy, type 2 diabetes . The epigastric pain had set on suddenly 2 weeks before, after an intense physical effort (and with inadequate hydration) and radiated to the back. The pain had variable intensity during its evolution, initially described as being of great intensity, later as having an average intensity and was accompanied by anorexia and asthenia. The patient reported associated progressive exertional dyspnea and chest pain with angina characteristics when making intensive efforts.The patient denied any history of gallstone disease and any high-fat, high-calorie intake or alcohol consumption. He was on a therapeutic regimen containing angiotensin-converting enzyme inhibitor (Prestarium 5 mg once daily), beta blocker , antiplatelet (Aspirin 75 mg once daily), nitrate (Isosorbide mononitrate 40 mg once daily), statin (Rosuvastatin 10 mg once daily) and biguanide (Metformin 800 mg once daily); was compliant to this therapy. As a risk factor he was an ex-smoker, but did not present any familial risk factor.Clinical findings on hospital admission: apyrexia; asthenia; no pulmonary findings; rhythmic heart sounds, heart rate = 56 beats/min, blood pressure = 140/85 mmHg; no peripheral swelling; abdomen with tenderness in the epigastric area and palpable pulsatile mass above the umbilicus, no defense or muscle stiffness, Murphy and Blumberg signs were negative; normal bowel movements; the diuresis was normal.The blood tests showed: ESR = 20 mm/h, normal blood count, increased serum  and urinary amylase , serum glucose = 119 mg/dl, hepatic and renal functions unaltered, normal triglycerides and cholesterol.The chest x-ray showed no pathological changes.The abdominal ultrasound followed by abdominal contrast-enhanced ultrasound (CEUS) revealed a 40 mm pancreatic pseudocyst with sediment Figure  and the The CT examination with contrast substance (Ioperamidum) described the spindle-shaped aneurysm of the abdominal aorta dilation beginning from the renal level, extending to 169 mm, until the iliac bifurcation and continuing on both iliac arteries, 40 mm left and 20 mm right respectively. The maximum diameter of the aortic aneurysm was 60.5 mm, with calcified circumferential atheromatosis, diffuse on its whole length; a parietal circumferential thrombosis with a maximum thickness of 27 mm and a minimum of 7 mm was also present; the diameter of the circulated lumen was 19 mm in the distal portion of the aorta, and 29/32 mm respectively below the kidneys. At the level of the renal arteries the aorta had an antero-posterior caliber of 36 mm and a latero-lateral diameter of 42 mm, with thrombosis of 5 mm postero-laterally. No signs of dissection or rupture of the aneurysm were evidenced. Calcified atheromas were described at the level of the splenic artery, and also at the level of the superior mesenteric artery ostium and bilateral renal arteries  and oral antidiabetic drugs. The evolution was favorable with slow normalization of pancreatic enzymes.After 2 months, the patient returned for follow-up examination. His clinical condition was improved, he did not present abdominal tenderness. Biological findings: ESR = 20 mm/h; normal pancreatic enzymes. The repeated abdominal contrast CT scan showed no significant changes in size for the pancreatic pseudocyst and the abdominal aortic aneurysm Figure .Figure 5This time gastroscopy was performed, showing no pathological changes.A cardiovascular surgery consultation was performed again, and the patient was scheduled for aortobifemoral endoluminal prosthesis. Meanwhile the follow–up showed that the pancreatic pseudocyst had resorbed in eight months. Figure . SurgerySurgery for the abdominal aneurysm was performed after the resorption of the pseudocyst. The cure of the aortic aneurysm was performed by bypass grafting with dacron aorto-bifemoral prosthesis. After surgery, a complication appeared, namely a periprosthetic retroperitoneal abscess. Another surgical intervention was therefore necessary, but severe septic shock occurred, leading to the patient’s death.The arterial vascularization of the pancreas is ensured by branches of the hepatic artery and superior mesenteric artery for the head of the pancreas, and branches from the splenic artery for the body and the tail of the pancreas. It is known that the pancreatic tissue is susceptible to ischemia with the possibility of developing acute pancreatitis . The appCases of acute pancreatitis after cardiogenic shock determined by cardiac tamponade have been described , supportGeneralized atheromatosis with significant impairment of splanchnic circulation was also considered a cause, which determines the appearance of pancreatic necrosis of an ischemic type . A case Our patient – in the context of the partially thrombosed aneurysm of the abdominal aorta  and generalized atheromatosis , he had undertaken intense physical effort, with an increased need for oxygen at the level of the muscles, to the detriment of the splanchnic territory, i.e. pancreatic, causing the onset of ischemic acute pancreatitis. We have also excluded any other possible causes of pancreatitis. In the case described, the pathogenetic mechanism of pancreatitis is explained by the arterial steal phenomenon, aneurysm of the aorta causing pancreatic hypoperfusion, especially in the presence of generalized atheromatosis.Because the surgical treatment of an aortic aneurysm itself can suffer complications with the appearance of pancreatitis of an ischemic type, the intervention was delayed until the normalization of pancreatic enzymes and the limitation of the lesions. Surgery for the abdominal aneurysm was performed after the resorption of the pseudocyst. But postoperative complications occurred - periprosthetic retroperitoneal abscess and severe septic shock, which led to the patient’s death.This case has several particular features. The diagnosis of a partially thrombosed aneurysm of the abdominal aorta could be established only after the presentation for abdominal pain caused by ischemic recurrent acute pancreatitis, i.e. after an unusual complication. Both the thrombosed aneurysm of the abdominal aorta and the generalized atheromatosis were simultaneously detected, which significantly affected the pancreatic circulation, each of these entities being able to cause ischemic pancreatitis. It should be mentioned that even the surgery for abdominal aortic aneurysm itself may cause pancreatic ischemia and an ischemic episode of pancreatitis; therefore, aorto-bifemoral endoluminal prosthesis was delayed until the normalization of serum pancreatic enzymes.Ischemic pancreatitis is a rare condition but should be taken into consideration for patients with upper abdominal pain, elevated amylases and significant cardiovascular diseases with extended atheromatosis. The diagnosis relies on the exclusion of other more common causes of pancreatitis.Written informed consent was obtained from the patient’s family for the publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Lipoblastoma and lipoblastomatosis are rare benign soft-tissue tumoral lesions resembling fetal adipose tissue. A total of 16 cases of lipoblastoma of the neck were reported in the literature, and only 3 were described in the posterior side of the neck. Hibernoma is a rare benign adipose tumor composed of brown fat cells and only about ten cases occurring in the cervical area have been reported. We reported two rare cases of adipose tissue tumors. The first case was a male infant aged 12 months who had a cervical mass on the posterior side of the neck. He underwent a complete resection of the lesion and the pathologic study revealed lipoblastomatosis. The second case was a 36-year-old man with an anterior cervical mass, which moved with swallowing. A resection was made and the histological analysis showed hibernoma. Lipoblastoma is a benign tumor of embryonic fat cells that occurs mainly before the age of 3 years, are more prevalent in men, and arise primarily in the trunk and extremities. Only 16 cases of lipoblastoma in the neck have been reported in the literature, and only 3 cases were described on the posterior side of the neck. Cervical hibernoma is a benign tumor that could be considered a differential diagnosis and is opposed to lipoblastoma, and occurs mainly in adults. Only ten cases have been described in the neck. The symptoms are: compression of cervical structures, respiratory compromise, Horner's syndrome and hemiparesis.Lipoblastomas and their diffuse, multicentric and infiltrative forms, the lipoblastomatosis, are rare benign soft tissue tumors that resemble the fetal adipose tissue. Adipose tumors comprise about 6% of soft tissue neoplasms and are developed in the first two decades of life. In general, 94% are lipomas, 4.7% lipoblastomas and 1.3% liposarcomasWe reported two cases of these rare lesions. The first one was a cervical hibernoma and the other a cervical lipoblastomatosis; both were found on the posterior neck.Male infant aged 12 months who had a cervical mass on the posterior side of the neck measuring 60×40mm. He underwent diagnostic imaging studies that showed a density fat tumor. After, a complete resection of the lesion was performed. The histological analysis showed an admixture of mature and immature adipocytes with muscle fibers entrapped and foci with a mixoyd stroma, which confirmed the presence of lipoblastomatosis .A 36-year-old man with anterior cervical mass for 40 days that measured 50mm, had fibroelastic consistency and mobile with swallowing. Ultrasonography examination showed a thyroid nodule. The patient underwent a fine-needle aspiration. The cytological diagnosis was adipocytic tumor which confirmed the presence of hibernoma. After the complete resection, the histological exam confirmed the diagnosis of hibernoma.. Hibernoma is a rare benign tumor of embryonal brown fat, and only about ten cases of it are reported in the neck.Adipose tumors comprise about 6% of soft tissue neoplasms and are developed in the first two decades of life. In general, 94% are lipomas, 4.7% lipoblastomas, and 1.3% liposarcomas. Cervical lipoblastomas are rare and constitute 10 to 15% of all lipoblastoma cases. It grows rapidly and often is asymptomatic, except when causing mass effect. On the other hand, hibernoma presents firm and mobile tumor, grows slowly and occurs chiefly in adults; both hibernoma and lipoblastoma can cause mass effect.Lipoblastoma are characterized as single subcutaneous nodule or by multiple lesions. Plain X-rays and computed tomography scan can suggest the fat density of the tumor. Results of ultrasonography examinations can be confusing. The MRI is the most reliable method showing location, size, extent and mass characteristics of the tumor, suggesting the histological components.Diagnostic imaging studies are invaluable in the preliminary diagnosis of fatty tumors. The lipoma is the most common soft tissue tumor and may show cytologic similarities to lipoblastoma. Although vacuoles may be seen in the adipocytes of conventional lipomas, the extent of vacuolated adipocytes is greater in number and smaller in lipoblastoma. Hibernoma has been well cytologically described. Hibernoma cells differ from those of lipoblastoma, and they usually show a central nucleus and abundant finely-granular or microvacuolated cytoplasm. In addition, smears show no myxoid matrix and less capillaries. Furthermore, hibernoma shows a lobular pattern, but it is entirely composed of brown fat with eosinophilic granular cytoplasm characteristic, that is not found in white fat of lipoblastoma. Liposarcoma is histologically different from lipoblastoma. It is important to recognize that both tumors may contain mitotic figures, and also that lipoblastoma is a mass of fetal fat cells varying in different degrees. Despite both tumors could contain a plexiform capillary network, this feature tends to be more prominent in myxoid liposarcoma. It is particularly important to differentiate myxoid liposarcoma from lipoblastoma because both tumors have a myxoid background. The characteristic lobulation of lipoblastoma is a feature that is often absent in liposarcoma, but can be seen in the myxoid variant. Hyperchromasia and nuclear atypia are seen in liposarcoma, but absent in lipoblastoma, and may be the most distinguishing feature.The differential diagnosis of lipomatous tumors should include lipoma, lipoblastoma, hibernoma, and liposarcoma2 and lipoblastomas show rearrangement in chromosome 8q11-13.Different lipomatous tumors tend to have characteristics of chromosomal abnormalities. Lipomas have abnormalities of chromosomes 12, 6, or 13, whereas myxoid liposarcoma tends to have the translocations t or t. Hibernoma shows rearrangement in chromosome 11q13. Its prognosis is good. By far the a recurrence rate of 15% has been reported for cervical lipoblastoma. Lipoblastomatosis has a high propensity for recurrence.Despite well-localized and nonmalignant nature of lipoblastomas, their rapidly growing might cause compressive symptoms. The standard therapy for cervical lipoblastoma is the complete surgical excision. This procedure must be made without technical difficulties since hibernoma usually presented as a well-circumscribed mass. Unlike lipomas, the hibernomas has an extensive vascularity that should be carefully treated to avoid postoperative bleeding or hematoma. No case of recurrence or metastasis has been reported,5. Additional treatment is not necessary, except when liposarcoma is suspected.Curative treatment to hibernomas is the complete excision with preservation of vital structuresWhen dealing with a cervical mass, lipoblatomatosis and hibernoma should be considered in the differential diagnosis. The surgical treatment must be considered.


Dear Editor,A 25-year-old woman with neither history of trauma nor other previous medical historyreported a 2-month history of low back pain. Physical examination revealed no significantabnormality. Conventional abdominal radiography showed the presence of a large,heterogeneous, calcified 10-cm mass in her left lower pelvis  and 1B. The patient underwent pelvic surgery with left adnexectomy. Macroscopically, the lesionmeasured 10.3 ×9.2 × 8.6 cm and was filled with a yellowish viscous material, hair, andseveral tooth fragments . Analysi-8.A series of recent publications in the Brazilian radiological literature have evaluated therole of radiology in the study of abdominal tumors-11. Mature teratoma is the most common benign ovarian tumor in womenaged < 45 years. The clinical manifestations of ovarian teratoma range from anincidentally detected small mass to a malignantly transformed tumor associated with highmortality. Most maturecystic teratomas are asymptomatic. Abdominal pain or other nonspecific symptoms occur in aminority of patients.The term “teratoma” comprises several histological types of tumor containing mature orimmature tissue of the three germ cell layers: the ectoderm , mesoderm, and endoderm (mucinous or ciliated epithelium)-11.At gross pathological examination, mature cystic teratomas are unilocular and frequentlyfilled with sebaceous material and lined by squamous epithelium. Hair follicles, skinglands, muscle, and other tissues lie within the wall. A raised protuberance (Rokitanskynodule) usually projects into the cyst cavity. At any imaging modality, mature teratomasdemonstrate a broad spectrum of findings ranging from purely cystic to mixed masses withcomponents of all three germ cell layers, to noncystic masses composed predominantly offat. Adipose tissue is present in 67– 75% of cases, and teeth are seen in31%. Atconventional radiography, a typical mature teratoma appears as a large mass with fatopacity and/or multiple toothlike calcifications. The most common US finding of an ovarian teratoma is a cysticmass with intratumoral fat and a densely echogenic tubercle (Rokitansky nodule) projectinginto the cystic lumen.Most mature cystic teratomas can be diagnosed by US, but such diagnosis is complicated bytheir diversity in appearance. The diagnosis of mature cystic teratoma by computed tomography(CT) and magnetic resonance imaging (MRI) is fairly straightforward as such modalities aremore fat sensitive. At CT, fat attenuation within a cyst, either with or withoutcalcification in the wall, is diagnostic of mature cystic teratoma. Presence of fat isreported in 93% of cases and teeth or other calcifications in 56%. At MRI, the signalintensity of the sebaceous component of a teratoma is similar to that of retroperitonealfat. Some hemorrhagic lesions may mimic this MRI appearance.Ovarian teratomas may cause various complications  with a wide spectrum of clinicaland imaging features


Endomyocardial fibrosis is a neglected tropical disease of unknown etiology and poor prognosis. It is endemic of tropical climates where it is the most common cause of restrictive cardiomyopathy in the second and fourth decades of life. A forme fruste of the disease is thought to be present in temperate climates where the diagnosis remains exceedingly rare.We describe a case of isolated right ventricular endomyocardial fibrosis in a 27-year-old Caucasian man from a temperate climate who presented initially with frank hemoptysis and pulmonary thromboembolic disease. We further describe the approach utilized in the diagnosis, the surgical treatment and the outcome of the disease.We conclude that endomyocardial fibrosis should be included in the differential diagnosis of apical cardiomyopathies in patients from temperate climates. Endomyocardial fibrosis is the most common cause of restrictive cardiomyopathy in tropical climates . It is cA 27-year-old Caucasian man presented with frank hemoptysis preceded by a two-month history of shortness of breath and non-productive cough. Prior to this subacute illness, he was healthy, had no travel history, and was a long-distance endurance athlete. An intravenously-contrasted computerized tomogram of the chest revealed bilateral pulmonary emboli and a filling defect within his right ventricle. Duplex lower extremity ultrasonography was negative for deep vein thrombosis. Transesophageal echocardiography revealed a mass at his right ventricular apex spanning proximally as pedunculated extensions through the tricuspid valve. Gadolinium-enhanced cardiac magnetic resonance imaging (MRI) confirmed a complex mass obliterating his right ventricular apex, insinuated between the chordae tendineae of the tricuspid valve and terminating in lobular stalks (Additional file Anticoagulation therapy was initiated post-operatively. Extensive work-up for coagulopathy was negative. Peripheral blood and bone marrow biopsy were normal; in particular, the percentage of eosinophils was normal at 2%. Cytogenetic study of the bone marrow showed no evidence of a malignant process. Other causes of endomyocardial fibrosis, including parasitic infections, carcinoid tumor and systemic diseases, such as Churg Strauss syndrome, periarteritis nodosa and Behçet syndrome, were excluded.Toxoplasma gondii in affected patients, suggesting a role for inflammation [The etiology and pathogenesis of endomyocardial fibrosis remain poorly understood. Several mechanisms have been postulated, including environmental and genetic factors, nutritional deficiencies, parasitic infections and eosinophilia. Recent studies have demonstrated antibodies against myocardial proteins and a high incidence of cardiotropic infective agents like cytomegalovirus, Epstein-Barr virus and ammation . The preammation .The diagnosis of endomyocardial fibrosis relies on echocardiographic criteria. The most salient features are a mass associated with one or both ventricles, typically the apices, and mural thrombus on the endocardial surface ,4. CardiEndomyocardial fibrosis is rare in temperate climates and should be included in the differential diagnosis of apical cardiomyopathies. Echocardiography and cardiac MRI each provide important information in the diagnosis of endomyocardial fibrosis; histopathological examination remains essential for the final diagnosis.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.MRI: Cardiac magnetic resonance imaging.The authors declare that they have no competing interests.MB was a major contributor in writing the manuscript. KL obtained the surgical specimen and was a major contributor in writing the manuscript. JCM performed the histological examination of the surgical specimen. EM performed the transesophageal examination, image analysis and interpretation, and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.Cine magnetic resonance imaging findings. Cine cardiac magnetic resonance imaging study in the four-chamber view shows a large intracavitary space occupying mass in the right ventricle. The mass is polypoid and lobulated, and marginally mobile. Note the concordance with the surgical findings in Figure Click here for file


A 56-year-old man presents with a 1.5-cm lesion over his right eyebrow. The mass appears cystic and is affixed to the overlying skin. It has been present for 1 year and has not changed in size for several months. Patient denies pain or changes in vision.What is the differential diagnosis for lesions of the eyebrow and upper eyelid?What clinical presentation is associated with this lesion?What is the recommended diagnostic and treatment paradigm for such lesions?What is the risk of progression to malignancy?-2Common lesions of the upper eyelid and eyebrow include chalazion, inclusion cyst, pilomatricoma, dermoid cyst, sebaceous cyst, and basal cell carcinoma. These lesions are often excised and diagnosis is confirmed by gross and histologic examination. A pleomorphic adenoma is a benign biphasic tumor comprising epithelial and stromal components. Though commonly seen in salivary or parotid glands, a pleomorphic adenoma found in this region is rare and is usually derived from either a lacrimal or a cutaneous sweat gland. Histological findings for both derivations are very similar  and 2.1-1When derived from a cutaneous sweat gland, the pleomorphic adenoma presents as a firm, nontender mass of the lid margin, central lid, or brow. It is typically a well-circumscribed nodule that may be mobile or fixed to the tarsus or overlying skin.,6Diagnostic modalities for the lesions are controversial. A mass anterior to the orbital rim can often be appreciated on high-resolution computed tomography.3en bloc resection followed by radiotherapy or chemotherapy for small and circumscribed lesions, with consideration for orbital exenteration if mass has infiltrated beyond the capsule. Regular long-term follow-up is imperative, as the mean survival following diagnosis of carcinoma ex pleomorphic adenoma is 3 years.3 and involved the subcutis.It is important that the tumor be excised with an intact pseudocapsule and clean margins as recurrence rates are as high as 30%, and progression to malignancy has been reported to be 30% after 15 years.Pleomorphic adenomas are benign glandular lesions. Though often found in parotid or salivary glands, they may rarely be seen originating from a lacrimal gland or cutaneous sweat gland. These lesions often present as well-circumscribed, firm, nontender masses. Complete excision with intact pseudocapsule and negative margins is important to prevent recurrence and malignant transformation.


Teratomas are neoplasms derived from the germ cell with components of all the three embryonic layers. These are rare neoplasms in head and neck region which can occur in any age group but are more prevalent in children. The present case is an 11-year-old girl who was brought with history of painless and progressive swelling in the oropharynx for 3 years with the associated left sided nasal blockage and nasal discharge. CT scan was suggestive of benign nasopharyngeal mass highly suspicious for lipoma. Excision of the mass was done under general anaesthesia. Peroperatively, it was a smooth, pedunculated mass arising from the left lateral wall in the nasopharynx. On cut section, it was solid to cystic mass similar to fatty tissue. Her HPE report came out to be mature teratoma of nasopharynx. Teratomas are congenital neoplasms derived from pluripotent cells from all the three germinal layers . The incThe clinical presentation depends on the size and site of the lesion. The most frequent symptoms of head and neck teratomas include aerodigestive tract obstruction such as nasal blockage, difficulty in breathing and breast feeding, rhinorrhea, and snoring. Occasionally there can be history of cyanosis suggestive of respiratory obstruction and respiratory distress; similarly there may be associated dysphagia if the pedicle is long enough. The head and neck teratomas pose a unique challenge in the surgical management . The aimAn 11-year-old girl presented with the complaint of nasal blockage, recurrent nasal discharge, and open mouth breathing for 3 years. Recently her parents noticed an abnormal swelling inside mouth, which was insidious in onset, painless, and gradually progressive. There were no other ear and throat complaints. Physical examination revealed a smooth pinkish mass hanging in the oropharynx posterior to the left palatine tonsil . FlexiblComputed tomography (CT) demonstrated a well circumscribed, heterogenous mass without intracranial extension originating from the nasopharynx suggestive of lipoma . The masTeratomas are rare congenital neoplasms composed of elements from all three germinal layers. The teratomas are mostly found in the sacrococcygeal region and the head and neck region only accounts for 5% of all teratomas. In the head and neck region, the common sites of origin include the neck, oropharynx, nasopharynx, orbit, and paranasal sinuses , 6, 7. TTeratomas are usually benign neoplasms which have been classified into 4 histological types such as dermoid, teratoid, true teratoma, and epignathi. Of these teratomas, dermoids comprise the vast majority; true teratomas occur less frequently. The exact etiology of these lesions is unknown. There are various theories regarding the origin of teratomas. For the head and neck teratomas, theory postulates that they arise from uncontrolled growth of a pluripotent cell originating in the region of the embryonic notochord .Imaging investigations such as CT scan and MRI (magnetic resonance imaging) are useful to determine the site, size, and extent of lesions, to differentiate between solid and cystic mass, and to rule out the intracranial extension of the nasopharyngeal neoplasm, thus aiding in the surgical management . In our Surgical excision is the treatment of choice for the mature nasopharyngeal teratoma, and the relapses are rare following complete excision of the mass . In the Nasopharyngeal teratoma is a rare neoplasm which usually presents at or soon after birth with features of upper aerodigestive obstruction. The treatment of choice for mature teratoma is complete surgical excision. We present a first case of nasopharyngeal teratoma in child in Nepal along with the review of relevant literature.


Head and neck paragangliomas are rare tumours and can arise as a part of inherited syndromes. Their association with thymic tumour is not well known.This report describes a female patient who presented with right sided neck paragangliomas. The histology of the tumour was consistent with paraganlioma. Few years later her MRI scan of the chest revealed presence of an anterior mediastinal mass that corresponded to the location of the thymus. Review of her previous scans showed that the mass was present all along and had gradually increased in size. Patient developed symptoms including fatigue, dyspnoea, migratory polyarthritis, Raynaud’s phenomenon and erythema nodosum. She had sternotomy and excision of mediastinal mass. The histology was consistent with cortical thymoma (WHO type B2) and she had radiotherapy. After treatment her constitutional symptoms improved. Her paraganglioma susceptibility genes are negative.To our knowledge this is only the second case report in the literature of coexistence of carotid body tumour and thymoma. The first case reported was bilateral carotid body tumour, thyroid gland adenoma and thymoma. This case also highlights the importance of long term surveillance, multidisciplinary management and being aware of associated pathologies in patients with isolated paraganglioma. Head and neck paragangliomas (HNPGLs) are tumors of the autonomic system. They arise from specialised neural crest chromaffin cells of the parasympathetic paraganglia of the skull base and neck, and are also called glomus tumors. HNPGLs account for approximately 3 % of all paragangliomas (PGLs). Most often, HNPGLs progress slowly are benign and nonsecreting with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. They can be widely distributed and prominent locations are the carotid body tumour (CBT) along with the vagal, jugular, and tympanic glomus. Symptoms depend on the specific locations . We also present a brief review the literature regarding the coexisting neck and mediastinal masses. To our knowledge this appears to be only the second case of paraganglioma associated with thymoma described in the literature.The patient is a 49 year old lady who initially presented with right sided earache and right sided neck swelling. Following ear, nose and throat (ENT) assessment, surgical excision of the mass together with an adjacent lymph node was performed. At operation the mass was consistent with a glomus vagale tumour. The histology revealed organised growth pattern in which tumour cells formed characteristic nests  separated by fibrovascular connective tissue septa. The tumour cells were polygonal with large nuclei, many with prominent nucleoli and plentiful eosinophilic granular cytoplasm. The tumour stained positively for synaptophysin, NSE and chromogranin A. No obvious metastatic features were found. The lymph node histology revealed reactive changes. The histology was consistent with paraganglioma Figs. , 2. PostFive years later she was seen in Genetic Endocrine clinic and had an ultrasound scan of the neck, MRI scan of the abdomen and thorax as a part of screening for paraganglioma follow up. MRI scan revealed the presence of a 4 × 2 cm anterior mediastinal mass Fig. . This maTwo years later her annual scan revealed an increase in the size of mediastinal mass to 5.8 by 3.1 cm Fig.  Whole boIn view of the enlargement of the mass and constitutional symptoms patient was discussed in a multidisciplinary meeting and the decision was made to operate. She underwent median sternotomy and excision of mediastinal mass. The mass was completely excised. The histology showed intermediate-sized lymphocytes with admixed epithelial cells with cytoplasmic processes. Immunohistochemistry revealed the presence of reactive T lymphocytes; TdT, CD1a and CD5 positive Fig. . The hisRET, VHL, SDHB, SDHC, SDHD, SDHAF2 and TMEM127 was negative. Analysis of the Paraganglioma/Phaeochromocytoma gene panel was undertaken in the West Midlands Regional Genetics Laboratory. The panel uses an Illumina MiSeq platform to capture the coding regions of the SDHAF2, SDHB, SDHC, SDHD, RET , MAX, TMEM127 and VHL genes using the TruSight Cancer Panel target enrichment system . Sanger sequencing is used to confirm any variants identified. MLPA analysis of all exons of the VHL gene (MRC-Holland Kit: P016-C2) and SDHB/C/D (MRC-Holland Kit: P226-C1) is undertaken.Patient signed a written informed consent for the test. Screening for germline mutations of the HPGLs overall are rare tumours and are known to have hereditary-familial tendency. Their association with thymic tumour is not well known. To our knowledge thymoma associated with paraganglioma has been reported only once before in the literature  and medullary thymic epithelial cells (mTECs) or stromal cells. These thymic stromal cells provide signals for T cell differentiation. During the late stages of the development of the thymic epithelium, hematopoietic bone marrow precursors migrate into the thymus. After this stage the normal thymic development is dependent on the interaction between the thymic epithelium and the hematopoietic thymocytes  are found mostly in the neck and abdomen, less commonly in the pelvic sympathetic plexus of the urinary bladder, and rarely in the mediastinum. PGLs may occur sporadically or as part of a hereditary syndrome. 60 % can develop metastatic disease, indicating that these tumors are often aggressive and need follow up (Ghayee et al. The common neuroectodermal origin is thought to be the reason for coexistence of carotid body tumour and a thymic tumour as in our patient. This was proposed in the first case who presented with bilateral carotid body tumor, thyroid adenoma and a thymoma (Refior and Mees The treatment options for HNPGLs comprise surgical resection, as well as irradiation therapy, stereotactic radiosurgery and permanent embolization. If necessary combined treatment strategy could be used. For carotid body tumours the principal is complete tumor resection. With complete surgical resection, the tumor is controlled locally in 89–100 % of cases. However, there is a possibility of postoperative cranial nerve dysfunction even in cases of successful surgical removal of CBT and complication rates are directly related to tumor size. Postoperatively our patient had vagal nerve palsy and patient is left with hoarse voice (Boedeker et al. Complete surgical excision is the treatment of choice for non metastatic thymoma and thymic carcinoma, even when the tumor is locally advanced. This is followed by postoperative radiotherapy to decrease the incidence of local recurrence. In an advanced disease surgical debulking, radiotherapy, and chemotherapy are recommended (Berman et al. In conclusion this is the second case where carotid body tumour and associated thymoma has been reported. This case highlights the importance long term follow up of patients with HNPGLs that should be mandatory because of their association with other tumours.Such cases should be seen in multidisciplinary clinic and should include geneticists as some of these cases are hereditary. Genetic tests should be offered to all of these patients so that proper surveillance by biochemical tests and imaging can be arranged and family members can be screened.


Neuroendocrine tumors that arise in an extragonadal teratoma are extremely rare. Somatic-type malignancy, defined as any sarcoma, carcinoma, leukemia, or lymphoma developing in a germ cell tumor, occurs in approximately 2% of all germ cell tumors. Our case represents a mediastinal mass that was incidentally found in a patient with syncope. Surgical resection confirmed mature teratoma with neuroendocrine features. Neuroendocrine tumors that arise in an extragonadal teratoma are extremely rare . To the A 34-year-old man with a past medical history of anxiety and hypothyroidism presented to emergency department complaining of nausea, tremors, and syncope. The symptoms had been persistent for the last two weeks. On the day of presentation, the patient had 3 syncopal episodes that occurred while laying down, each lasting for 6 or 7 seconds. The patient denied any history of seizure activity or recent head trauma.Physical examinations were unremarkable. Cardiac enzymes and alcohol level were within normal range. Radiology studies with a CT-scan of the head and CT-angiography were unremarkable. Chest X-ray showed abnormal cardiac shadow with rounded left cardiac border, prominent right cardiac border, and elevated left hemidiaphragm Figures . SubsequAfter excluding any cardiac or neurological reasons for his symptoms, the patient was sent for diagnostic chest fluoroscopy, which showed bilateral diaphragms moving symmetrically with no paradoxical motion and with mild elevation of the left hemidiaphragm. Again seen is a 5.8 × 5.4 cm fat containing mass arising from the left side of the mediastinum abutting the left cardiac border. The lungs are clear without focal consolidation or pleural effusion.It was decided to proceed with surgical resection without needle aspiration or biopsy, and the patient underwent left video-assisted thoracoscopic surgery (VATS) and resection of the mediastinal mass. The tumor was found to be adherent to the lateral border of the pericardium, where it was dissected off without resection of the pericardium Figures . The patPathologic evaluation weighted the tumor to be 165 g and 10 × 6 × 4.5 cm in size with smooth outer surface. On pathologic dissection, the interior was filled with light yellow thick cheese-like material mixed with gray white hair . There wTeratoma is the most common germ cell tumor and usually contains tissue derived from the ectodermal layer, whereas mesodermal and endodermal derived structures are more infrequent . STM is Patients with a mediastinal tumor can have different presentations. A mediastinal mass is often an incidental diagnosis when patients undergo evaluation for an unrelated condition or symptom like in our case in which the patient presented with nausea and syncope. Clinical symptoms are observed in 62% of patients and include chest pain (30%), dyspnea (16%), fever and chills (20%), and cough (16%) . Of noteA mediastinal mass is typically diagnosed or suspected based upon abnormalities detected accidently on a posterior-anterior and lateral chest X-ray. Findings can vary from subtle mediastinal findings to the obvious presence of a large mass in the mediastinum. A chest CT-scan with intravenous contrast is typically used to evaluate chest X-ray abnormalities. The CT can confirm the presence of a mediastinal mass; occasionally an abnormality that was thought to be a mediastinal mass is found to be something else, such as a diaphragmatic hernia or an aortic aneurysm. It can also provide detailed information on the mediastinal abnormality, including its size, location, relationship to other structures, tissue characteristics, and presence of calcifications, fluid or fat.Detailed knowledge of any involvement or compression of other mediastinal structures is critical in planning treatment and preparing a patient for possible surgery. Unnecessary morbidity can be avoided if involvement of unresectable structures is identified prior to resection. No imaging beyond the CT-scan is needed in many cases to evaluate the mediastinal mass.Tumor markers can support the diagnostic work-up in some instances of anterior mediastinal masses when thymoma or germ cell tumor is suspected. Although teratomas comprising areas of immature or neuroendocrine differentiation are generally considered malignant, in localized tumors the prognosis seems favorable particularly in young patients and complete surgical resection is the treatment of choice , 9, 10.The novelty of our case originates from the symptomatic presentation of syncope which initially delineated the differential diagnosis towards neurologic and cardiac causes. The mass effect was not elucidated on history and physical, as patient had long standing history of hypothyroidism and an anxiety disorder. The symptoms and the final diagnosis pointed to a carcinoid syndrome, which could explain the syncope due to an abrupt drop in blood pressure from release of large amounts of hormone or chemical substrate by the teratoma. Although teratomas comprise areas of immature or neuroendocrine differentiated cells, these are generally considered malignant, whereas in localized tumors the prognosis seems favorable particularly in the young and with complete surgical resection confers a positive outlook.


Suture granuloma is a mass forming benign lesion that develops at the site of surgery as a foreign body reaction to non-absorbable suture material. We present a case of suture granuloma that developed at the inguinal region after orchiectomy, and define the sonography, color Doppler sonography and real-time ultrasound elastography findings in correlation with the histopathological findings. A 25-year-old male with a history of left orchiectomy due to testis tumor eight months ago, presented with left inguinal palpable mass. The initial histopathological diagnosis was pT1 mixed germ cell tumor  which was 3cm in diameter, with necrosis but without vascular, rete, epididymis, spermatic cord, or tunica albuginea invasion. The surgical margins were free of tumor. Accompanying intratubular germ cell neoplasia was noted in the non-tumor testis tissue. The patient was then evaluated by thorax and abdomen CT which revealed three left para-aortic lymph nodes where the largest was 17mm in maximum transverse diameter. The patient was treated with two cycles of BEP  regimen, the chemotherapy was completed without any complications and he was taken into regular follow-up. At the time of diagnosis of the tumor, alpha-fetoprotein level was 51.5ng/mL  and ß-hCG level was 136.1ng/ mL  which came into the normal limits after surgery and chemotherapy.Eight months after the initial admittance, the patient presented with a palpable, firm, non-painful, nodular mass in the proximal part of orchiectomy area in the left groin. Serum markers were within normal limits . The patient was evaluated with sonographic examination by L5-12 transducer of a Philips iU22 scanner . On gray-scale sonography, the mass at the operation area was in soft tissue echogenicity 13*21*33mm in dimensions with lobulated but well defined margins . The lesSuture granuloma is a rare complication of surgery developing secondary to the use of non-absorbable suture material –9. It isSuture granulomas may occur anywhere in the body. The risk of development has been reported more with silk and polyfilament threads than the absorbable suture materials , 5. SilkClinical presentation of suture granuloma may vary from a classic inflammatory reaction with erythema, swelling, pain and finally rejection of the suture material to a chronic inflammatory reaction with granuloma formation that may present as a solid mass, usually painless and gradually increasing in volume . The timThe diagnosis of suture granuloma is particularly important in patients treated for cancer because the condition may resemble local tumor recurrence. Clinical findings, history and even the imaging features may be misleading. There are reported confusing lesions in the literature mostly detected by PET-CT –6. SuturColor and power Doppler sonography exam in our patient showed that the lesion was hypervascular in nature particularly at the peripheral parts which were found to be inflammatory regions of the mass on histopathological evaluation. In fact, Doppler imaging had a limited role in the diagnosis of our case. Moreover, this hyper-vascular nature might have been misleading to a tumor diagnosis if the primary was a high stage tumor and/or the local recurrence an expected consequence.We also performed real-time ultrasound elastography in our patient and investigated the strain ratio of the mass to evaluate its nature. Our case is the first one that elastography is used for the evaluation of a suture granuloma. Actually, elastography is a relatively new technique and the literature is limited at the moment in the field of characteristics of the granulomatous lesions , 14. HowThe diagnosis of suture granuloma is provided by surgical excision of the lesion and histopathological examination. The foreign body is detectable at the center of the granuloma under polarized light, surrounded by multinucleated giant cells and macrophages. Surgery is not only used to achieve the final diagnosis but also used for eradication of the inflammation. In our patient, the local recurrence was not an expected condition because the local recurrence of stage pT1 testis tumor in a very short time after the completion chemotherapy, and with normal serology, is rare. Additionally, the sonographic findings were consistent with a suture granuloma. However, the patient was operated to confirm diagnosis, to clear up the lesional area and for the psychological relief of the patient.In conclusion, suture granulomas are rare lesions that can be diagnosed by high resolution sonography with the typical features of single or double layer linear or curvilinear echogenicity covered by hypoechogenic layers. Doppler sonography seems to have a limited role to provide additional information for differential diagnosis. Elastography features reported here are the first in the literature and include increased stiffness with high strain ratios.


Giant cell tumor (GCT) of bone is a generally benign, but often locally aggressive, neoplasm of bone, with a propensity for recurrence. Sarcomatous transformation is rare and typically occurs with a history of recurrences and radiation treatment. Denosumab, an inhibitor of the RANK ligand involved in bone resorption in GCT, is increasingly used in treatment of recurrent or unresectable giant cell tumor of bone. We report two cases of sarcomatous transformation of GCT to osteosarcoma in patients receiving denosumab. One was a 59-year-old male with a 12-year history of GCT and multiple recurrences taking denosumab for 2.5 years. The second case was in a 56-year-old male with a seven-year history of GCT taking denosumab for six months. Review of the literature shows one case report of malignant transformation of GCT in a patient being treated with denosumab. As the use of denosumab for treatment of GCT will likely increase, larger, controlled studies are needed to ascertain whether denosumab may play a role in malignant transformation of giant cell tumor of bone. Giant cell tumor (GCT) of bone is a generally benign tumor that is often locally aggressive, causing significant destruction of bone . RecurreTreatment often involves curettage, with or without bone filler or adjuvants such as polymethylmethacrylate (PMMA) or phenol. Less invasive procedures, such as radiotherapy, radiofrequency thermal ablation, or chemoembolization, may be used in cases where surgery is not possible. Wide resection may be reserved for cases in which surgery results in relatively minor functional impairment or for tumors with extensive local destruction , 3, 10. A recent report described a case of high grade sarcoma arising in a giant cell tumor of bone treated with denosumab . We repoA 46-year-old male presented in 2002 to an outside institution with five to six years of right hip pain radiating down the lateral thigh. Initial imaging is unavailable but by report radiographs showed a large mass involving the ischial tuberosity and portions of the adjacent inferior and superior rami of the right pelvis. He subsequently transferred care to our institution. Biopsies performed at the outside institution were reviewed and confirmed giant cell tumor of bone. He subsequently underwent partial internal hemipelvectomy in November 2002. Histopathologic analysis showed GCT .Postoperatively, the patient was started on alendronate (dose unknown). Follow-up MRI in February 2003 showed an enhancing, high T2 signal, 2 cm soft tissue mass within the right adductor musculature, adjacent to the former location of the ischial tuberosity, suspicious of recurrence. CT imaging in August 2003 showed a mass within the surgical bed, within the obturator externus and pectineus muscles, and abutting the root of the penis. CT guided biopsy confirmed recurrent benign GCT. Reexcision of the mass was performed in September 2003. The pathology specimen showed a 4 cm mass embedded within excised soft tissue. Giant cell tumor was present at multiple resection margins.Postoperatively, the patient did well, with intermittent complaints of pain. Follow-up MRI eight months after surgery showed an enhancing multilobulated mass at the margins of the original hemipelvectomy surgical bed, the largest mass measuring up to 2.7 cm. Edema and abnormal enhancement were noted within the obturator externus, obturator internus, and quadratus femoris muscles. Another 1.5 cm nodule was noted in the proximal adductor brevis muscle. He was subsequently followed up with approximately yearly MRI studies. The recurrent tumor showed progressive, interval growth, reaching a measurement of 3.2 × 3.6 cm in December 2005 , 9.2 × 3The patient was started on denosumab (120 mg subcutaneous monthly) in July 2012. Imaging over the next 2 years showed stable disease, with possible slight decrease in size of the tumor. In November 2014, the patient developed osteonecrosis of the left mandible after tooth extraction, and denosumab was held for two doses. Denosumab was restarted in late December 2014 after presenting to clinic with severe right groin pain radiating inferiorly. Follow-up CT scan in February 2015 showed enlargement of the pelvic mass to 13.6 × 13.0 cm with extension into the ischioanal fossa and subsequent mass effect on the rectum, prostate, bladder base, and base of penis  . By immuSubsequent CT scan showed interval increase in size of the pelvic mass, an increase in the number and size of lung nodules, pulmonary lymphadenopathy, and a hepatic lesion suspicious of additional metastasis. The patient was started on doxorubicin and ifosfamide. CT scan showed slightly decreased primary tumor but an increased number of pulmonary nodules, two liver lesions, inguinal lymphadenopathy, and a probable 3.7 cm metastasis to the penis. He was then started on docetaxel and gemcitabine. He transferred care from our institution shortly thereafter.A 49-year-old male presented in July 2007 with a six-month history of left knee pain and swelling. Radiographs from an outside institution showed a cystic lesion in the left femur condyle with sclerotic borders and expansion of surrounding bone. GCT was strongly suspected and the patient underwent curettage with PMMA packing in August 2007. Histologic evaluation confirmed giant cell tumor of bone .The patient was followed postoperatively with radiographs of the knee. One month after surgery, a rim of lucency around the cement packing was noted . There wThe patient was offered, and declined, both denosumab and surgery at that time. With continued knee pain and weakness, however, he opted for denosumab treatment in January 2014. He initially did well, denying pain except with running or other significant impact activities. But, by July 2014, he reported two months of significantly increasing knee pain and swelling. Radiographs showed continued increase in size of the lucency, measuring up to 5.0 cm, with periosteal reaction suggestive of impending pathologic fracture . BecauseThe pathology specimen consisted of a 15 cm length of distal femur with minimal attached soft tissue and muscle and with an up to 4.0 cm ill-defined heterogeneous mass proximal to the PMMA . HistoloThe patient was given four cycles of doxorubicin and cisplatin in September 2014 but developed skeletal metastases. He was then started on high dose methotrexate. He initially had stable disease but again progressed after several months, with development of pleural and additional skeletal metastases. He was then placed on gemcitabine and docetaxel but died soon after.Recently, the first case report of sarcoma arising in the setting of denosumab treatment for giant cell tumor of bone was published . The patTwo Phase II trials have thus far evaluated the safety and efficacy of denosumab in the treatment of giant cell tumor of bone. In a study of 37 patients with recurrent or unresectable GCT who received 120 mg of denosumab monthly (and loading doses on days 8 and 15 of the first month), 30 of 35 patients with available follow-up data had tumor response, defined as elimination of at least 90% of giant cells on biopsy or no radiologic progression. Denosumab was well tolerated, with pain and nausea as the primary complaints. One patient developed high grade sarcoma arising in a GCT, found after an abnormal elevated human chorionic gonadotropin level and subsequent tumor resection. Another patient with recurrent GCT metastatic to lung developed malignant GCT eight months after discontinuing treatment .n = 170); those with salvageable disease with planned surgery (n = 101); and a third cohort (n = 11) that included patients from the original Phase II study of 37 patients. Ninety-six percent of patients in the surgically unsalvageable disease cohort showed no progression. Only 26 patients in the planned surgery cohort ultimately required surgery, 16 of these with a less morbid surgery than originally planned. Again, denosumab was well tolerated, with pain, nausea, and fatigue as the primary complaints. Hypophosphatemia, hypocalcemia, infection, and osteonecrosis of the jaw were noted in a handful of patients. Two cases of sarcoma arising in GCT were recorded, though it is unclear in which cohort(s) these patients were. One case was suspected to have been present but unrecognized prior to treatment. The other case arose during the study. The investigators did not believe in a connection between denosumab and sarcomatous transformation of GCT. Interestingly, a case of thyroid carcinoma with high grade sarcoma was also reported, arising in an area of previous radiation [A second Phase II trial utilizing the same doses and scheduling of denosumab as the first trial enrolled 282 patients with GCT into three cohorts: those with surgically unsalvageable disease (adiation . At our The potential relationship between, and mechanism of, sarcomatous transformations of GCT during denosumab therapy is unclear due at least in part to the limited published data on this population. In vivo and in vitro studies of the effect of denosumab on GCT at the cellular level show loss of giant cells, usually a reduction in the neoplastic stromal cells with reduced RANKL expression and proliferation, and reactive and woven bone and/or osteoid formation , 15, 16.Patients taking denosumab for GCT likely represent a subset that are at higher baseline risk for sarcomatous transformation, often having a long-standing history of disease with multiple recurrences and treatments. More extensive studies of the side effects of denosumab in patients with osteoporosis  and canc


Solitary neurofibromas are rare, benign tumours of nonodontogenic origin. The presentation of a solitary neurofibroma on the tongue is an uncommon occurrence and we present such a case here which was discovered in concomitance with multiple neurofibromatosis type 1 (von Recklinghausen's disease). Such a rare presentation seen in this case is a diagnostic challenge and often clinched only with the aid of histopathological and immunohistochemical examination. This work also discusses the various differential diagnoses that can be considered in similar cases. The presence of a hypertrichotic “giant” nevus spilus tardivus (Becker's nevus) is also a rare finding in this particular case. We present such a case which will be of interest to the budding dental practitioner. The lesion was excised and the patient followed up without any evidence of malignant transformation. Neurofibromas are benign nerve sheath tumors originating from the peripheral nerves and are the hallmark presentation in von Recklinghausen's disease or neurofibromatosis type 1 .–13.7–13]The differentiation between neurofibroma and the plexiform variety is usually clinched with histopathological and immunohistochemical diagnosis. This case report presents with a solitary plexiform neurofibroma involving the tongue which is not a very common presentation with sporadic neurofibromatosis type 1. It is also interesting because the case presents with a hypertrichotic giant nevus spilus tardivus in von Recklinghausen's disease involving the right arm which has very rarely been reported in literature.A 39-year-old Asian male presented to our department with a chief complaint of a painless swelling involving the left lateral border of the tongue. History revealed that the growth began as a pea-sized nodule and gradually increased in size to its present state over a period of 4 months.The past medical history did not reveal any cardiovascular, respiratory, genitourinary, gastrointestinal, endocrine, haematological, neurological, or any other medical history of relevance. The past dental or familial history was not of any consequence to our particular case. A complete blood count was performed and no abnormality was detected. No history of trauma, bleeding, pain, or paresthesia was present. There were no signs of any cervical lymphadenopathy noted. Orthopantomographic examination did not reveal any bony abnormalities. Extraoral examination revealed multiple soft cutaneous nodules involving either side of the face, back, trunk, and the lower and upper extremities. They were round to oval in shape and were of various sizes ranging from a few millimetres to centimetres across. On palpation they were found to be sessile and some pedunculated also, soft to firm in consistency, nontender, and noncompressible and showed no signs of fixity. Around 15 café au lait (coffee in milk) macules were present over 15 mm in diameter throughout the body with increased prevalence in the back and trunk. The largest one among these was located over the right arm measuring a whopping 22 × 13 cm across with smooth borders. It was roughly ovoid in shape and there was a brownish macule with long thick dark hair involving the surface of the lesion and it extended from the acromioclavicular joint all the way down to the upper arm . The disIntraoral examination revealed a sessile lesion with a lobulated appearance and measured 2 × 1.5 cm in greatest dimensions. It was roughly ovoid in shape with irregular borders and exhibited a smooth surface. The periphery was non-erythematous in appearance. On palpation it was found to be nontender, firm in consistency, and fixed to the underlying tissue. It was noncompressible, nonreducible, and nonpulsatile in nature . The sloConsidering the clinical presentation and localization of the lesion, we included a neurofibroma, schwannoma, neurilemmoma, granular cell tumour, reactive lesions like a giant cell fibroma or focal fibrous hyperplasia, leiomyoma, rhabdomyoma, hemangioma, lymphangioma, lipoma, and benign salivary gland tumours among differential diagnosis taking into account the peripheral exophytic nature of the lesion.Routine haematological examination revealed a normal blood profile and no other imaging findings for soft tissue analysis like ultrasonography or MRI were performed considering the miniscule proportions of the lesion and its benign presentation. Patient was also referred to the adjacent medical college to rule out the possibility of any internal lesions. Excisional biopsy of the tumour mass on the tongue was performed in toto and primary closure achieved with a single interrupted Vicryl suture. The patient was prescribed an NSAID medication and asked to use povidone-iodine mouth rinse.Histopathological examination which is the investigation of choice in peripheral exophytic lesions involving the tongue revealed a section with densely collagenous stroma with proliferation of spindle cells as fascicles with thin wavy nuclei in irregular pattern. Numerous plump fibroblasts were present and there were many vascular channels. Connective tissue is lined by stratified squamous epithelium . The speThe postoperative healing was uneventful and patient was followed up after two weeks and subsequently after 6 months. The patient was also advised to report for periodic follow-up visits due to the potential for neurofibromas to undergo malignant transformation.A neurofibroma is a benign tumour arising from the cells of neural sheath origin. Although an uncommon benign tumour, we found it prudent to place the diagnosis of a neurofibroma with a higher ranking in our list of differential diagnoses considering the fact that our patient exhibited other extraoral features of neurofibromatosis type 1. It usually presents on the tongue and also occasionally on the buccal mucosa, gingiva and involves the lips very rarely . The expA neurilemmoma or a schwannoma is an encapsulated tumour mass that is present submucosally. They are not very common in the oral cavity, but if present, the tongue is the most common location for the lesion. They appear nodular and sometimes may grow to fantastic sizes. Confirmatory diagnosis is by conducting a biopsy .Granular cell tumour, also called the Abrikossoff's, may occur anywhere in the body but has a marked predilection for the tongue when it occurs in the oral cavity. Usually it presents in the fourth to sixth decades of life. In over 50% of the cases tongue shows pseudoepitheliomatous hyperplasia, but, IHC can help in diagnosis with the aid of S-100 immunoreactivity in the mesenchymal tissues .Another reactive lesion, namely, the focal fibrous hyperplasia (irritation fibroma), typically presents as a smooth nodule. It is usually 1.5 cm or smaller in diameter. Prevalence is greater in individuals above 40 yrs of age .Oral leiomyomas are benign smooth muscle tumours that are rarely noticed in the oral cavity and most of them, if present, arise from the smooth muscles of the underlying vasculature. They generally present as tiny, slow growing, solitary nodular masses located principally on the tongue, lips, palate, and buccal mucosa. Although asymptomatic, they may present with symptoms such as pain, tooth mobility, or difficulty in chewing , 18.Rhabdomyomas are striated muscle tumours which may occur on the mucosal surfaces as well. They are usually very rare and diagnosis can be confirmed by normal H&E staining. However, they have to be biopsied to evaluate the presence of its extremely malignant counterpart, the rhabdomyosarcoma .Hemangioma of the tongue is highly unusual occurrences and is more common in the 1st decade of life. Intramuscular tumours are nonmetastasizing benign congenital tumours. Most of them also increase in size in 2nd-3rd decades of life. They are usually not life threatening entities .Lymphangioma is a benign proliferation of lymphatic vessels and is often hamartomatous transformations of malformed lymphatics. They may present as localised growths and their limited extension facilitates easy surgical removal. They are difficult to diagnose based on the clinical appearance alone and are mainly caused because of the difficulty in drainage of the lymph which causes a localised collection in the area .Lipoma is a painless benign mesenchymal tumor that is well circumscribed. It may be present in any site but is rare in the oral cavity. Possible causes include infection, chronic irritation, hormonal imbalance, and trauma. Histopathological analysis is the gold standard for diagnosis and they are composed of mature far cells with fibrous connective tissue which is often hyalinised with or without the capsule and/or fibrous septa .The majority of salivary gland tumours are benign in nature, with pleomorphic adenoma accounting for 60% of them. The involvement of the tongue is very rare for a pleomorphic adenoma and is hence ranked the last , 24.There is still some controversy regarding the presence of Lisch nodules associated with neurofibromatosis type 1, which was not present in our case, which certain authors think are asymptomatic and not correlated with visual impairment or severe optic nerve involvement . Our casAs was in our case, most of the time, neurofibromas are usually not with significant clinical consequences; a feared complication of neurofibromas is the probability for malignant transformation which is the reason why it is most prudent to perform biopsies of the tumour masses, as we have done. The incidence of sarcomatous transformation has been placed at 15% of all cases by Preston and coworkers. Fibrosarcoma, spindle cell sarcoma, and neurogenic sarcoma are some of these. In addition, Preston and coworkers have also mentioned other pathological lesions, mental disorders, osseous defects, and congenital defects which were not seen in our case . In the