Infantile fibrosarcoma is very rare tumor and seldom occurs in oral cavity. Overall good prognosis is reported in more than 80% cases. We present a 5-month old female patient with swelling of the tongue for four months. This was excised completely. Histopathology and immunohistochemistry revealed it as infantile fibrosarcoma of grade II. A 5-month old female infant presented with progressive swelling of the tongue since one month of age. There was no associated difficulty in swallowing or breathing. On examination, there was a firm, diffuse swelling about 3cm x 4cm in dimension, involving left side of the tongue and extending to midline. Mucosa over the mass was intact and margins were ill-defined . There was no associated cervical lymphadenopathy. Ultrasonography revealed heterogeneous mass in the tongue sparing the floor of mouth. Fine needle aspiration was inconclusive. At operation adrenaline mixed local anesthetic was injected at the incision site to minimize the bleeding. Linear incision was made on the left lateral border of the tongue. The mass was excised and wound stitched with polyglycolic 3/0. Patient was put on injectable antibiotics for 48 hours with mouth cleansing antiseptic oral spray. Specimen was sent for histopathology which was reported as infantile fibrosarcoma of grade II. The margins of the excision were tumor free. Immunohistochemical staining showed positive staining for SMA and CD34 which also supported the diagnosis. Patient was reviewed postoperatively and referred to oncologist for chemo-therapy . At three months follow up patient was disease free after receiving chemotherapy.Fibrosarcomas are malignant tumors predominantly originating from soft tissues.[1] Fibrosarcomas are commonly found in the extremities, trunk, and head and neck regions in descending order of frequency. Among all the fibrosarcomas, only 0.05 % occur in head and neck region, of which 23% occur in oral cavity.[2] Rarely it arises from the tongue.[2-5] There are two types of fibrosarcomas, infantile or congenital (IF) and adult form. The presentation of congenital fibrosarcoma is usually in early age as in our case. Clinical features depend upon the size, site and grade of the tumor. However, it presents as slow growing painless mass.[6] Feeding and breathing difficulties are the main concerns in case of intraoral mass lesions. In our patient the mass was also increasing slowly in size but it was not causing any feeding or breathing difficulties. The exact cause of IF remains obscure, however genetic malformation and radiation may contribute.[3]CT scan or MRI may point a solid mass but the exact diagnosis can only be made on histopathology powered by immunohistochemistry as we diagnosed in our case. Infantile fibrosarcoma can be graded into low, intermediate and high grade depending on severity of cellularity, nuclear pleomorphism and mitosis.[3] Infantile fibrosarcoma must be kept in the differentia diagnosis of other soft tissues tumors like rhabdomyosarcoma and Infantile myofibromatosis and its variants. Our case was of grade II on histopathology. The management of IF is a multidisciplinary which includes surgery, chemotherapy and radiotherapy with rehabilitation if indicated.[4] Surgery is the mainstay of treatment for IF. In our case the mass was excised apparently completely and then patient was referred to oncologist. The success of treatment of IF depends on age of the patient, extent and site, grade and stage of the disease, response to the treatment and associated side effects of the medications. Overall good prognosis of IF is more than 80%, if treated in time.[8] Our patient is doing well till date. In conclusion, although infantile fibrosarcoma is a rare tumour, it must be kept in differential diagnosis of soft tissue tumors presenting in infancy. Surgery is the mainstay of treatment for infantile fibrosarcoma that can be complemented with chemo-radiotherapy depending upon its stage.Source of Support: NilConflict of Interest: None declared


Dear SirThe patient was a 6-hour-old boy, a first twin delivered at the federal medical centre Yola by a 20-year-old house wife per vaginum after 39 weeks of unsupervised pregnancy. The abdomen of the index neonate was immediately noticed to be distended at birth and he did not pass meconium for more than 24 hours. There was an accompanied respiratory difficulty. There was no vomiting. The second twin was doing fine. The child weighted 2.2 kg, and was dyspneic and tachypneic with pulse of 165/min. The abdomen was uniformly distended and shiny. Rectal examination was unremarkable. Plain abdominal x-ray showed pneumoperitoneum with dilated central bowel loops. No calcification was seen. The patient was taken for exploratory laparotomy after resuscitation. The intraoperative findings were meconium ascites, three ileal perforations  on the antimesenteric border, each not less than 2cms apart, and approximately 20cm from the ileocecal junction. There were fibrinous adhesions involving loops of the distal ileum. There was a bolus of large meconium distal to the perforations with collapsed loops of bowel distal to it. The bowel proximal to the perforations was moderately dilated. This segment of the ileum was resected about 15cm from the ileocecal junction and an ileoileal end-to-end anastomosis was performed. A full thickness rectal biopsy was taken to exclude total colonic aganglionosis. The patient did well, except for developing a superficial surgical site infection which was managed with wound dressing. The histology of the respected ileum showed features of nonspecific inflammation with bile pigments. The rectal biopsy was negative for Hirschsprung’s disease. Breast feeding was introduced on the 5th day after surgery and the patient was discharged on the 10th day. In utero intestinal perforation could be due to various causes and can lead to generalized, fibro-adhesive or cystic forms of meconium peritonitis 1]. Generalized meconium peritonitis develops if the perforation occurs late in the prenatal period. . The diffuse nature of the peritonitis initiates the inflammatory process, and calcification is absent because the inflammatory period is short. The typical presentation of abdominal distention at birth and failure to pass meconium are common in the majority of cases . The presence of fever may suggest sepsis because of bacterial colonization in patients with delayed presentation. Delay in presentation increases mortality in these patients [5]. Prenatal diagnosis is vital in finding the predisposing factors of meconium peritonitis [4]. Antenatal diagnostic features include: bowel dilatation, ascites, and polyhydramnios. Calcification is also a vital diagnostic feature, which can easily be seen on a post-natal plain abdominal x-ray, if present [4]. The pathogenesis of calcification in meconium peritonitis has been linked to the precipitation of calcium salts in the meconium, or the inflammatory response of keratin debris in the meconium [5]. In our case, the other twin was normal indicating local factors in the affected fetus played a role in the pathogenesis of meconium disease and maternal systemic factors are less likely involved. Source of Support: NilConflict of Interest: None


We describe in this report a case of successful radiofrequency ablation of an unresectable stage III-type B3 thymoma, and we discuss the role of this novel approach in the management of patients with advanced stage thymoma. The patient, a 59-year-old Caucasian male underwent neoadjuvant chemotherapy with only a slight reduction of the mass. Subsequently, an explorative sternotomy and debulking were performed; before closing the thorax, radiofrequency ablation of the residual tumor was carried out and a partial necrosis of the mass was achieved. A further percutaneous radiofrequency ablation was performed subsequently, obtaining complete necrosis of the lesion. Successively, the patient underwent adjuvant radiotherapy. As a result of this multidisciplinary treatment, complete and stable response was obtained. It is hard to say which of the single treatments had the major impact on cure; nevertheless, the results obtained suggest that radiofrequency ablation must be taken into account for the treatment of advanced stage thymomas, and its effectiveness must be further assessed in future studies. Thymomas are rare tumors of the thymus gland, accounting approximately for 0.15 per 100.000 person-years in the United States , 3. We dA 59-year-old Caucasian male with a stage III-type B3 thymoma was referred to the Businco Oncological Hospital of Cagliari, Italy, in 2006. The patient underwent four cycles of cisplatin, doxorubicin, vincristine, and cyclophosphamide, with only a slight reduction of the mass .The surgeons performed an explorative sternotomy and, once the unresectability of the lesion was confirmed, a debulking. Before closing the thorax, an RFA of the residual tumor was performed with a needle monopolar electrode, as we previously described, positioned in the centre of the lesion, and a partial necrosis of the mass was achieved  4]. A f. A f4]. The patient subsequently underwent adjuvant radiotherapy with a total amount of 50 Gy fractionated in 25 doses. A complete response was obtained, as shown by a nuclear magnetic resonance (NMR) scan performed 36 months later . A positThe current practice in the management of advanced stage thymomas is based on a multidisciplinary approach, including the combination of chemotherapy, surgery, and radiotherapy. Ablative treatments in this setting are only sporadically reported. Yamauchi et al. reported for the first time the use of cryoablation for palliation in a 29-year-old female patient with locally advanced unresectable B3 thymoma [The use of radiofrequency thermal ablation is widely reported in the treatment of several thoracic tumors, especially those of the lung, either primitive or metastatic, with or without involvement of the mediastinal lymph nodes. Nevertheless, no reports on its use in the treatment of advanced thymomas have been published; to our knowledge, the present case is the first to be officially reported, as only sporadic unpublished cases have previously been described .The rationale of the RFA use in the treatment of advanced stage thymomas is based on the biological effects of radiofrequency on the neoplastic tissue and on the possibility to accurately caliber the anatomical extension of the treatment. The tissue effects have been extensively described, especially in lung tumors: the neoplastic cells are heated by ionic agitation to over 60° and this causes an irreversible protein denaturation and cell death. Furthermore, RFA is a minimally invasive technique which can be performed in local anesthesia and may be repeated if necessary. Some authors found that the proximity of target lesions to large vessels has been associated with higher local tumor progression rates in patients with lung malignancy . NeverthRadical resections have been demonstrated to be a significant predictor of survival in patients with thymoma, even when surgery is extended to the adjacent anatomical structures . Unfortunately, these conditions only partially represent Masaoka stage III disease, which comprises also unresectable tumors. In these last cases, the only surgical option is an incomplete resection. In terms of survival, the role of debulking in advanced thymomas is still a matter of debate. Some authors have reported an increased 5-year survival rate in up to 30% of cases submitted to incomplete resection of thymomas . UnfortuThe multidisciplinary approach used in our case produced excellent results, leading to a complete and stable response. It is difficult to assess which of the single treatments employed had a major impact on obtaining such results. Consequently, it is hard to state that RFA was the “golden bullet.” Nevertheless, we believe that RFA must be taken into account for multidisciplinary treatment of advanced stage thymomas, and its effectiveness must be assessed in the context of adequately designed clinical trials.


A 17-year-old girl presented with bilious vomiting and abdominal pain to the surgery department. The history was positive for trichotillomania and trichophagia. A CT scan showed a mass in the stomach, which was highly suspicious for a gastric bezoar. Drooping parts of the bezoar caused a duodenal obstruction with secondary acute pancreatitis. The bezoar was removed via a laparoscopically performed gastrotomy.  This case describes an unusual genesis for acute pancreatitis especially in young women. Rapunzel syndrome is a very rare cause of pancreatitis but should be considered as a differential diagnosis in patients with a significant psychiatric history. This case report presents a novel minimally invasive surgical approach to the treatment of obstructive bezoars causing pancreatitis.A 17-year-old Caucasian female was referred to the surgery department by her primary care physician with a 3-week history of intermittent bilious vomiting and mid-epigastric to right upper quadrant abdominal pain. The pain had started during traveling in her school holidays. In the previous weeks she was admitted to the emergency department for dehydration twice and was released without a definitive diagnosis. On presentation, she had a weight loss of 10 pounds, a decreased appetite, and a reduced urine output. The remainder of the review of systems was negative.After further extensive inquiry the mother made a side note that the patient had a habit of chewing her hair. Nevertheless she had no signs of alopecia. Furthermore there was a history of swallowing large amounts of gum in her childhood. Otherwise her past medical history was significant for dysmenorrhea which she took oral contraceptives for. The patient was living with her parents and sister and denied any substance abuse. The family history was positive for an unknown clotting disorder, which was noncontributory for the course of this case.The patient's lab studies showed elevated markers for pancreatitis. Amylase was 396 and lipase was 639. Her basic metabolic profile was within normal limits. Her complete blood count showed a mild leukocytosis of 11.6. Her Ranson criteria score on admission was 1 (mild pancreatitis). Her radiographic studies were performed including an abdominal X-ray, HIDA scan, and CT scan. The abdominal X-ray merely showed a paucity of gas. The CT scan of the abdomen however revealed a mass, which was considered as a possible trichobezoar in the stomach and proximal duodenum. This bezoar could strongly be related to the history of trichophagia and would be a cause of duodenal obstruction with secondary pancreatitis, which was detectable on CT imaging Figures .At first the patient was treated conservatively. She was placed on IV fluids and received NG tube, antiemetics, and analgesia. Then the gastroenterologist attempted to do an EGD to evaluate the mass. The EGD revealed a distal esophagitis, gastritis, and trichobezoars occupying and obstructing the lumen of the stomach. The duodenum could not be intubated due to the mass ; therefoSurgery was consulted for operative intervention. The decision to undergo a laparoscopic gastrotomy with exploration and removal of the bezoar in her stomach and duodenum was made. The procedure was performed under general anesthesia without any complications. Four ports were used. After identifying the stomach and feeling a bulge in the distal part of it, an incision for the gastrotomy was made with scissors and electrocautery .™ through the umbilical incision. The gastrotomy site was closed with Endostitches™ vicryl sutures. An endoleak test was performed with air insufflations via an intraoperative EGD. The total operation time was 2.5 hours.A large bezoar was found in one part of the stomach, attached to the pylorus, and another part was extending into the duodenum. After detaching the bezoar from the stomach mould, it was divided into 3 pieces in order to extract it from the port sites . These pThe patient tolerated the procedure well without any complications. Prior to discharge the patient's pain was adequately controlled with p.o. narcotic pain medication. The follow-up radiographic studies showed no evidence of leakage or signs of obstruction or free air. She was discharged with a stool regimen and instructions for a follow-up as an outpatient.One month after surgery the patient was gaining weight and had no nausea or vomiting or any abdominal symptoms. Her wounds had also completely healed up. Furthermore we planned an outpatient follow-up and psychotherapy for prevention of recurrences, which present in 20% of the cases .A trichobezoar in the stomach.A long “tail” of hair strands that extends through the pylorus into the small intestine and sometimes even into the large intestine.Gastrointestinal symptoms.The complications associated are bleeding, perforations with peritonitis, ulcers, enteropathy with protein loss, appendicitis, and, in severe cases like ours, pancreatitis [“Rapunzel syndrome” is a unique manifestation of an advanced gastric trichobezoar, which was first described in literature by Vaughan et al. in 1968 and has three common features , 3:A trireatitis –7.At this point of time approximately 30 cases of Rapunzel syndrome have been published worldwide –10. The It is assumed that the slippery nature of hair and its entrapment within the gastric folds could be the reason for cross-linkage and the formation of bezoars . The symThe objective of the treatment is the mechanical removal of the trichobezoar and the prevention of recurrences with psychotherapy , 24, 25.The treatment of Rapunzel syndrome is still the subject of an ongoing debate, since a gold standard does not exist. A retrospective review of laparoscopic versus open bezoar removal by Yau et al. shows that laparoscopic removal has a lower rate of complications. Of note, these were not Rapunzel syndromes. In our literature review we only found 3 other reports of successful laparoscopic management of Rapunzel syndrome –29. DornReasons for a laparoscopic approach are most notably attributable to the epidemiology of this condition. Since the common presentation of trichobezoars is in young females, attention should inter alia be paid to the aesthetic result, which speaks in favour of a laparoscopy  . Further


An 85-year-old woman was an outpatient treated at Tokyo Rosai Hospital for cirrhosis caused by hepatitis B. She had previously been diagnosed as having common bile duct stones, for which she underwent endoscopic retrograde cholangiopancreatography (ERCP). However, as stone removal was unsuccessful, a plastic stent was placed after endoscopic sphincterotomy. In October 2012, the stent was replaced endoscopically because she developed cholangitis due to stent occlusion. Seven days later, we performed ERCP to treat recurring cholangitis. During the procedure, the stone was successfully removed by a balloon catheter when cleaning the common bile duct. The next day, the patient developed abdominal pain, abdominal distension, and nausea and was diagnosed as having gallstone ileus based on abdominal computed tomography (CT) and abdominal ultrasonography findings of an incarcerated stone in the terminal ileum. Although colonoscopy was performed after inserting an ileus tube, no stone was visible. Subsequent CT imaging verified the disappearance of the incarcerated stone from the ileum, suggesting that the stone had been evacuated naturally via the transanal route. Although it is extremely rare for gallstone ileus to develop as a complication of ERCP, physicians should be aware of gallstone ileus and follow patients carefully, especially after removing huge stones. Ileus is a disease that should be differentiated as a cause of abdominal pain and nausea in elderly individuals. Among the different types of ileus, gallstone ileus is a rare condition associated with bowel obstruction caused by a gallstone passing into the intestinal tract, and this disease accounts for 1–3% of cases of mechanical obstruction of the small intestine and 25% of cases of nonstrangulation ileus in individuals >65 years old [An 85-year-old woman, who had been treated for hepatitis B virus-related cirrhosis at Tokyo Rosai Hospital, was diagnosed as having common bile duct stones during a routine abdominal ultrasonographic examination in January 2009, for which she underwent ERCP in July 2009. However, because the Vater papilla was located inside a diverticulum in the third portion of the duodenum, the use of a stone crusher was difficult. Therefore, a double-pigtail plastic stent  was placed to prevent post-ES cholecystitis, and the stent was replaced usually every 3 to 12 months. An interval was decided according to her general condition. In September 2012, the patient was admitted to the Department of Gastroenterological Medicine for the management of pain due to a lumbar compression fracture and hepatic encephalopathy. Hepatic encephalopathy improved after admission, and the patient was undergoing rehabilitation for lumbar compression fracture when she developed abdominal pain and fever on hospital day 47. Physical examination revealed yellowing of the palpebral conjunctiva and tenderness in the epigastric region. Hematological findings were a high white blood cell count, low level of hemoglobin, low platelet count, and reduced prothrombin time. The patient also had hypoproteinemia, renal dysfunction, and elevated levels of bilirubin, hepatic enzymes, and C-reactive protein . ComputeThe patient developed sudden abdominal pain and nausea on hospital day 58 and was diagnosed as having ileus on the following day based on the CT findings of abdominal distension and the accumulation of intestinal fluid. CT also revealed that the stone, which had been passed into the duodenum in the previous ERCP, was incarcerated in the ileocecal region , and thiFirst reported by Erasmus et al. in 1654, gallstone ileus is a rare disease . Many gaIn addition to stone size, patient characteristics are important factors in gallstone ileus. More than 60% of gallstones are said to be incarcerated in the ileum , largelyDiagnostic imaging is essential for the diagnosis of gallstone ileus. Even though 77.8% of air-fluid levels and 88.9% of abdominal distension cases are diagnosed on plain abdominal radiography, only 37% of pneumobilia cases, a condition unique to gallstone ileus, and 33% of ectopic gallstone cases  are diagIn principle, gallstone ileus requires treatment intervention since the disease condition improves naturally in only 4–8% of cases. The goal of intervention is to cure ileus by removing stones from the intestinal tract, which is achieved mostly by surgical intervention, but the use of an endoscope –27 and eWe recently encountered a rare case of gallstone ileus that developed after endoscopic stone removal. The present findings suggest that when endoscopically removing huge stones, surgeons should pay attention to the risk of gastrointestinal obstruction, as well as stone size, and follow patients carefully.


The extracranial internal carotid artery aneurysm (EICAA) is an uncommon arterial lesion. Patients typically present with neurologic symptoms resulting from impaired cerebral perfusion and compression symptoms of cranial nerves. Often EICAA presents as a pulsatile neck mass, which is otherwise asymptomatic. We present a case of an 84-year-old female, who was initially referred to the Emergency Department for Otolaryngology with suspected peritonsillar abscess. The patient had a history of recent upper airway infection and cardiovascular comorbidities, including hypertension and ischaemic stroke complicated by extensive neurologic deficits. Physical examination revealed a compact, nonpulsatile mass in the lateral parapharyngeal space and local erythema of the mucosa. Duplex Doppler Ultrasonography and Computed Tomography revealed an atherosclerotic aneurysm of the right internal carotid artery, measuring 63 × 55 × 88 mm, stretching from the skull base to the angle of the mandible. Extracranial internal carotid artery aneurysms (EICAA) are rare vascular lesions, most commonly caused by atherosclerosis. Manifestations are usually neurological and result from decreased perfusion of central nervous system; however, patients also present with symptoms of nerve and aerodigestive tract compression. Often the only sign is palpable neck mass , 2. HereAn 84-year-old female with pain in the angle of the mandible and throat was referred to the Emergency Department for Otorhinolaryngology with suspected PTA. The patient had a history of cardiovascular diseases including hypertension and ischaemic stroke, complicated by left-sided hemiplegia, facial nerve palsy, hemispatial neglect, and dysarthria. History of trauma and previous vascular interventions was negative. The patient had dementia and was a nursing home resident, which impeded history taking. Physical examination revealed a compact, nonpulsatile mass in the right parapharyngeal space reaching the body midline. Mucous membrane was erythematous as a manifestation of pharyngitis sicca. Pyrexia, hoarseness of voice, and trismus were absent. Duplex Doppler Ultrasonography and subsequently performed Computed Tomography (CT)  with digInternal carotid artery aneurysm is defined as a local increase in vessel diameter of >50%, compared to reference values for appropriate vessel segment. EICAA are uncommon arterial lesions; the estimated incidence is less than 1% of all arterial aneurysms and exceedingly rarely may be bilateral .Reported etiology of EICAA varies greatly depending on population studied. According to the three largest studies, atherosclerosis is the leading cause of EICAA 43–91%). Other etiologies include fibromuscular dysplasia, aneurysm formation after surgical interventions, trauma, vasculitis, and infections %. Other , 4. PrevManifestations of EICAA depend on size and location and fall into three main categories: neurologic disorders caused by decreased perfusion of the central nervous system, symptoms resulting from compression of surrounding tissues, and hemorrhage. Neurologic disorders are most common and typically include focal symptoms such as strokes, transient ischaemic attacks (TIA), transient amaurosis, and anopsia. Less commonly described are Horner's syndrome, inarticulate speech, facial numbness, and retinal infarction , 3, 5, 6Large aneurysms displace tonsillar fossa medially and trigger compression symptoms, of which dysphagia is the most common and is caused by impaired function of the glossopharyngeal nerve (n. IX). Hoarseness of voice results from compression of recurrent pharyngeal and vagus nerves (n. X); local pain and discomfort are associated with malfunction of cervical branch of facial nerve (n. VII) , 7. FurtDifferential diagnosis of peritonsillar mass comprises carotid artery aneurysm and infectious and neoplastic etiologies. Patients with diabetes, the elderly, and those living in common quarters are at risk of developing infections and their complications, signs of which may be less apparent. The initially suspected PTA is the most common complication of acute tonsillitis. In a series of 15 EICAA, Szopinski et al. mention a patient with recurrent tonsillitis who sustained hemorrhage after an attempt of drainage of suspected PTA . FurtherThe first line of treatment in EICAA is open aneurysmectomy with end-to-end anastomosis or graft interposition , 4. OtheTypical manifestations of EICAA are neurologic; however signs and symptoms observed most commonly by ENT specialists, such as neck mass, mass in the parapharyngeal space, dysphagia, dysphonia, and local pain, may be in the foreground. Thus a mass in the parapharyngeal space requires a careful differential diagnostic procedure, especially in patients with cardiovascular comorbidities. Palpation may reveal pulsation of the mass; however, lack of pulsation does not exclude presence of an aneurysm, which may be atherosclerotic with adherent thrombus.


Extraventricular neurocytomas (EVNs) are rare parenchymal brain tumors, distinct from central neurocytomas that are typically located within the supratentorial ventricular system. Seizures and headache represent the most common symptoms of extraventricular neurocytomas in the cerebral hemisphere both in adult and pediatric population.We describe two cases of pediatric EVN with clinical onset characterized by behavioral and attention deficit/ hyperactivity disorders. The association between behavioral/attention disorders in childhood and the presence of a frontal neurocytoma has never been described before. Furthermore, inappropriate levels of inattention, hyperactivity and impulsivity are common among the neurobehavioral and developmental disorders in childhood. We reviewed 43 pediatric cases of extraventricular neurocytoma included in the PubMed database and their clinical presentation, and we never found this unusual relationship.In childhood, the attention/hyperactivity disorders seem to be often over-diagnosed. When these deficits are more subtle and do not well-fit in a specific neurocognitive disorder, the clinicians should have a suspicion that they might mask the clinical features of a frontal lesion. This paper is focused on the clinical presentation of the extraventricular neurocytoma and the possible organic etiology of an attention and hyperactivity deficit. Extraventricular neurocytoma (EVN) is a parenchymal brain tumor distinct from central neurocytoma. It is most commonly located in the frontal and parietal lobes  and inclAlthough the incidence of EVN in childhood is not known, it is certainly a rare tumor. It is composed by either glial or neuronal cell differentiation. EVNs have a potential aggressive behavior based on the MIB-1 labeling index >3%) and on atypical histological features. They are mostly confused with oligodendrogliomas or ependymomas [% and on Written informed consent was obtained from the patients’ parents for publication of this Case report and any accompanying images. We report on two cases of extraventricular neurocytoma in two children referred to the Neuropsychiatry Unit of Bambino Gesù Children’s Hospital in Rome with the diagnosis of attention deficit/ hyperactivity disorder (ADHD). We further reviewed 43 pediatric cases of EVN included in the PubMed database with particular reference to onset symptoms.A 10-year-old male, with a clinical diagnosis of ADHD since he was 7-year-old, was admitted to our Hospital with persistent headache and vomiting. Physical examination showed bilateral papilloedema. No other neurological signs were evident. Non-enhanced brain CT scan documented a large (4.5 cm), calcified and heterogeneous round mass in the right frontal lobe with vasogenic edema. Brain MRI confirmed an enhancing lobulated mass with micro-cystic and solid components, causing a right-to-left shift and disappearance of the frontal horn of the right ventricle Figure . The patA 10-Year-old male was admitted to our Hospital, suffering from frontal headache and focal seizures with a sudden onset. At 7 years a diagnosis of ADHD was performed, based on clinical evidence of speech and learning delay with attention deficit and anxiety symptoms. Head CT scan revealed a right mesial frontal mass, with calcifications. Brain MRI confirmed the small (12 x 17 mm) parasagittal mass of the right frontal lobe, involving the anterior part of the cingulate gyrus. The lesion demonstrated a heterogeneous post-contrast enhancement on T1-weighted image, while restricted diffusion was observed in the solid component Figure . A videoNeurocytoma is a rare tumor of the central nervous system, mainly recognized within the ventricular system adjacent to the Monro’s foramina in middle-aged patients. It displays both neuronal and glial differentiation. Extraventricular neurocytoma was introduced as a separate entity from central neurocytoma in the 2007 World Health Organization (WHO) classification of CNS tumors . Raised Attention deficit/hyperactivity disorder has to be well defined among the behavioral disorders appearing at school age, in order to avoid misdiagnosis. ADHD defines significant and wide-ranging impairments and affects as many as 3 to 9% of children worldwide. It is based on strict criteria but is often over-diagnosed. The diagnosis of primary ADHD is made by DSM IV criteria when there is no evidence from the history, the physical examination or laboratory findings of any other condition producing a similar clinical picture. Other psychiatric and neurological disorders such as traumatic brain injury, epilepsy and depression can lead to disturbances in attention and/or activity level. In patients with frontal tumor masses and low proliferation index, the attention impairment and behavioral disorders probably result from direct disruption of frontal lobe functioning or disconnection from subcortical afferents.Our patients were evaluated with cognitive and behavioral assessment at the time of admission, before and after surgery, with one-year follow-up intervals. Clinical history reported by parents showed that language and motor delay had already been present since they were 4-year-old. Therefore we hypothesized a delayed global development, overlooked and underestimated when the ADHD was diagnosed. Cognitive assessment was performed by Wechsler Intelligence Scales for Children (WISC III), documenting a normal range and no significant difference between verbal and performance skills. Behavioral symptoms were clinically investigated and standardized by Child Behavior Checklist CBCL 6-18) DSM IV oriented, used for children and teenager . Patient-18 DSM IPatient 2 had focal seizures shortly before diagnosis and they stopped after surgery, with normal EEG pre and post-operatively. Moreover, the progressive improvement after tumor resection suggests a frontal lobe disfunction as the origin of observed neuro-behavioural symptoms.Attention deficit hyperactivity disorder is associated with multiple neuropsychological deficits. Several studies underline how difficult it is to make a diagnosis of ADHD. Our experience suggests caution in cases with significant comorbidity before excluding alternative diagnoses.A recent study, based on electronic records of Emergency Department visits for 87 pediatric patients with brain tumors, retrospectively reviewed from 2002 to 2011, shows that behavior/school change and altered mental status appear with a low frequency, less than 17% . These dOur patients underwent gross total resection. Surgical resection remains the main management strategy for a good outcome. When a subtotal surgical resection is performed, radiotherapy could be considered to ensure better local disease control.Neuropsychological follow-up one year after resection showed progressive behavioral improvement in both patients, suggesting that the initial diagnosis was not ADHD.Attention deficit in childhood, not symptomatic of other injuries, do not tend to improve with age, while an age-related improvement is possible in hyperactivity disorders. We ascribe the improvement of the behavioural performance to the surgical resection, in our patients. This suggests that the attention deficit/hyperactivity disorder was linked to the presence of the EVN. Our report should stimulate clinicians to be careful to the behavioral disorders in childhood not connectable to a clear neuropsychological diagnosis, such as a poor academic achievement and problematic peer relationship, since they could have an organic etiology.Written informed consent was obtained from the patients’ parents for publication of these Case reports and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Lipoma arborescens is a rare cause of chronic monoarticular arthritis, with only a few cases reported in the literature. It is most commonly seen in the knee, but cases in other joints such as the wrist, shoulder, and elbow have also been described. It is a benign condition, in which the subsynovial tissue is replaced diffusely by mature fat cells. We describe a case involving the knee and discuss the symptoms, diagnosis, and treatment. Lipoma arborescens, also referred to as villous lipomatous proliferation of synovial membrane, diffuse lipoma of the joint, or diffuse synovial lipoma, is a rare benign intra-articular lesion characterized by villous proliferation of the synovial membrane . The etiLipoma arborescens typically affects adults. It most commonly involves the knee, but other locations have also been described. People present with joint pain, swelling, and effusion. The diagnosis is based on the typical appearance on MRI, and the recommended treatment is open or arthroscopic synovectomy. Recurrence is uncommon.A 31-year-old Dutch man of Turkish origin presented to the physical and rehabilitation medicine department with low back pain. On physical examination, a marked effusion in the right knee joint was noted. According to the patient, the swelling had been present since two years in a varying degree, with intermittent episodes of pain. There was no history of trauma. The patient's father had type-2 diabetes mellitus, but our patient was not diagnosed with it. The patient was known to have psoriasis.During the consultation, an aspiration was performed yielding 113 cc of citrine-colored fluid. The patient was prescribed rest and nonsteroidal anti-inflammatory drugs. Because the patient did not respond to this treatment, radiographs were obtained demonstrating effusion in the right knee and osteoarthritic changes .One week later, the patient again presented with recurrent swelling. An ultrasonographic examination disclosed a large amount of fluid in the suprapatellar recess, with synovial villi, but no hyperemia. A bony spur was seen on the medial femoral condyle. Laboratory tests showed no elevated inflammatory parameters, and negative rheumatoid factor, anti-CCP, and HLA-B27 typing. A bone scan combined with SPECT revealed mildly-to-moderately increased remodeling at both knees, but mainly on the right. MRI demonstrated moderate knee osteoarthritis, a region of chondropathy in the medial tibial cartilage, and marked effusion with multiple fatty synovial proliferations, which is pathognomonic of lipoma arborescens Figures .Six weeks later, an arthrotomy was performed with a total synovectomy and resection of hypertrophic multilobulated synovial tissue. The diagnosis of lipoma arborescens was confirmed on histological examination . lipoma is misleading because the lesion does not show any macroscopic or histological features of a neoplasm. Therefore, Hallel et al. suggested that lipomatous proliferation of the synovial membrane would be a more appropriate name [ arborescens comes from the word arbor (Latin for tree) and describes the macroscopically treelike morphology of this lipomatous villous synovial proliferation [Lipoma arborescens is a rare, mainly intra-articular lesion characterized by diffuse replacement of subsynovial tissue by mature fat cells, giving rise to a prominent villous transformation of the synovium . It was ate name . The terferation .Although the lesion is usually located within a joint, involvement of the subdeltoid and bicipital bursa  and the Lipoma arborescens usually affects the knee, preferentially the suprapatellar pouch, but has also been reported to occur in other joints such as the hip , wrist , elbow , and shoLess than 100 cases of lipoma arborescens have been reported in the literature. The peak incidence is between the third and fifth decades, with a predilection for male . There aThe etiology is still unknown, and although it can occur without antecedents, it is associated in the literature with trauma, inflammation, or inflammatory joint diseases, neoplasm, and degenerative conditions (osteoarthritis) , 15. TheA synovial reaction triggered by a trauma has been postulated, but most patients with lipoma arborescens do not have a history of trauma and nor did our patient.The hypothesis of lipoma arborescens being a reaction to chronic inflammation is supported by the histological finding of a mononuclear cell infiltrate in the underlying synovial membrane . FurtherA causal relationship between lipoma arborescens and degenerative joint disease is suspected but has yet to be confirmed. It has been suggested that the lesion can be divided into a primary and a secondary type. The primary type is rare and has been described as a form of synovial lipomatosis with hypertrophy as the cardinal feature; it is rarely considered a cause of degenerative knee joint changes. The secondary type has been defined as lipomatosis resulting from chronic irritation of the synovium (as seen with degenerative joint disease or arthritis) rather than a true neoplasm and is by far the most common form of lipoma arborescens. Most authors have accepted the last hypothesis , 19, 20;Patients with lipoma arborescens typically present with one or more of the following symptoms: slowly progressive swelling, pain, intermittent episodes of joint effusion, limited range of motion, and locking. Only rarely can a soft-tissue mass be identified on palpation . Most paThe diagnosis of lipoma arborescens is based on the typical findings on MRI, but because of the clinical appearance in this case other more common pathologies had to be excluded by using laboratory tests, joint fluid aspiration, and other imaging studies. In general the laboratory findings are nonspecific. The joint fluid aspirate is negative for crystals and microorganisms and is mainly used to exclude other causes of joint swelling , 15.Plain radiographs sometimes show a soft-tissue density, nonspecific bone erosion, or other osteoarthritic changes  , 10.On ultrasonography, a frondlike hyperechoic mass is seen that waves during manipulation of the joint .CT scanning shows a villous synovial mass of a density similar to fat. The signal is not enhanced with intravenous contrast administration .On MRI it has a pathognomonic aspect, which makes MRI the diagnostic imaging modality of choice . The finThese findings firstly include a large frondlike mass arising from the synovium. Secondly, the mass has a subcutaneous fat-equivalent signal on all pulse sequences. The subsynovial component has a high signal intensity on T1- and T2-weighted images and signal suppression on short T1 inversion recovery sequences (STIR) or after presaturation of the fat , 24, 25.Other features are bone erosions, synovial cyst, degenerative changes, and chondromatosis. On MRI, our patient presented both the typical signal characteristics and a marked effusion. Furthermore, some degenerative changes such as chondropathy, osteophyte formation, and other signs of knee osteoarthritis were also seen.The most common presentation of lipoma arborescens is a diffuse villous proliferation, but in a minority of cases it may also present as a focal pseudomass. According to Soler et al., the pseudomass type presumably is associated with primary lipoma arborescens, that is, without degenerative changes, but this was later refuted by Vilanova et al. In his retrospective series of thirty-two cases he found that 87% of the patients showed associated degenerative changes of the joint and 72% had a meniscal tear. We have to note that his study group mainly consisted of elderly patients , 25.In the past, a biopsy was considered essential for making a final diagnosis, but now the typical MRI findings are sufficient to permit a reliable diagnosis .Macroscopically, the lesion has a white-yellowish aspect and shows villous proliferation. Histologically, the villi are filled with mature fat cells and enlarged hyperaemic capillaries may be present. The underlying synovial membrane may contain mononuclear chronic inflammatory cells and the synovial cells may appear to be enlarged and reactive, with abundant eosinophilic cytoplasm , 11, 25.Lipoma arborescens forms part of the differential diagnosis of a chronic joint swelling; including pigmented villonodular synovitis, synovial osteochondromatosis, rheumatoid arthritis, intra-articular or synovial lipoma, synovial hemangioma, amyloid arthropathy, and xanthoma. Intra-articular lipoma and synovial osteochondromatosis are the only among these entities that can demonstrate similar MRI signal characteristics .Intra-articular lipoma can be differentiated from lipoma arborescens based on its macroscopic and microscopic features. It appears as a solitary round or oval mass, as opposed to the multiple villous lipomatous proliferations and the frondlike morphology of lipoma arborescens. Intra-articular synovial lipoma (IASL) is composed of mature fat cells covered by a thin fibrous layer, usually with no synovial lining. Histologically, lipoma arborescens is characterized by diffuse replacement of the subsynovial layer by mature fat cells with a moderate infiltration of mononuclear cells. Intra-articular lipoma does not arise from the synovial layer, nor does it replace it.Synovial osteochondromatosis is characterized by a nodular proliferation and metaplasia of the synovial membrane. On MRI, the signal intensity of it is similar to that of lipoma arborescens, but because of the cartilaginous nature of the lesion and the extent of the ossified or calcified regions, the signal intensity usually varies. In addition, the calcified or ossified lesions usually are already visible on radiographs .Synovial hemangioma is a benign vascular malformation of the synovium which mainly occurs in children and adolescents. MRI demonstrates a lobulated intra-articular mass with a hyperintense signal due to pooling of blood in vascular spaces. Furthermore, lipoma arborescens usually arises in the suprapatellar pouch, whereas a synovial hemangioma is primarily found in the infrapatellar pad , 28.The differential diagnosis of pigmented villonodular synovitis can be made by the absence of hemosiderin .Joints affected with chronic rheumatoid arthritis show diffuse joint space loss, periarticular osteopenia, soft-tissue swelling, and marginal erosions on plain radiographs. Chronic rheumatoid arthritis has intermediate to low signal intensity on T1- and T2-weighted MRI-images associated with the formation of fibrous pannus. When affecting multiple joints, lipoma arborescens can mimic rheumatoid arthritis; but history, physical examination, laboratory tests, and radiography allow us to distinguish between rheumatoid arthritis and lipoma arborescens .Temporary relief of lipoma arborescens symptoms may be achieved with an intra-articular injection of corticosteroids, but the recommended treatment for symptomatic lipoma is surgical resection (synovectomy) by open arthrotomy or arthroscopy . Using aErselcan et al. described a case of lipoma arborescens that was successfully treated by yttrium-90 radiosynovectomy. But, there have been no additional reports of cases treated in this manner .Nevertheless, when patients do not have pain or any functional limitations, a conservative treatment is indicated.Lipoma arborescens is a rare intra-articular condition, characterized by villonodular proliferation of the synovium. The typical MRI appearance of fatty synovial proliferation with no other signal intensities is pathognomonic for lipoma arborescens. Although rare and benign, the condition should be considered in the differential diagnosis of a chronic joint swelling. The recommended treatment, if it causes pain and discomfort, is synovectomy. This can be performed arthroscopically or open, depending on the location and size.


Leiomyomas are common benign gynecologic tumors occurring in up to 30% of women. Round ligament leiomyomas however are very rare and, if symptomatic, can present as an inguinal hernia. We report the case of a 47-year-old woman who presented with an irreducible inguinal mass consistent with an incarcerated hernia. Intraoperatively, the mass was found to be a round ligament leiomyoma, a diagnosis that was confirmed by histopathology following excision of the mass. Although rare, round ligament leiomyomas should be part of the differential diagnosis of an inguinal hernia in females. The round ligament (RL), the embryological equivalent of the gubernaculum testis in females, is composed mainly of smooth muscle fibers and extends from the uterus to the labia majora passing through the inguinal canal. RL tumors are rare but whenever they occur, leiomyomas are the most common lesions , 2. LeioA 47-year-old G1P1 African American woman presented with a right groin mass. The mass was noticed around four months ago and has been intermittently painful. The patient stated that the mass has grown in size recently. She denied any associated gastrointestinal or urinary symptoms and denied any recent weight loss, fever, or chills. She had no past medical history, no history of uterine leiomyomas, and no long-term estrogen use. Her past surgical history was significant for a Cesarean section followed by an abdominoplasty and a laparoscopic appendectomy. On physical exam, the patient's groins were asymmetric in the standing position; a bulge was visible in the right inguinal area. Upon palpation, the mass was round and firm measuring around 4 cm. Tenderness was only elicited with deep palpation. The mass was only partially reducible with pressure and with the patient in the supine position. A working diagnosis of incarcerated right inguinal hernia was made and the patient was scheduled for an elective surgical repair. Blood workup was within normal limits and no imaging was obtained. Under general anesthesia, a transverse groin incision was made and the external oblique fascia opened. A 4 × 3 × 2 cm firm well-circumscribed egg-shaped mass adherent to the RL was encountered; no hernia sac was seen . The masFollowing complete excision of the round ligament and lesion, it was noted that the internal ring was vacant, as it no longer had anything to obturate it. Additionally, the floor of the inguinal canal was weakened from the dissection. Therefore, a formal hernia repair was performed which included closure of the ring and placement of a lightweight mesh patch.The RL, the embryological equivalent of the gubernaculum testis in females, is composed mainly of smooth muscle fibers and extends from the uterus to the labia majora passing through the inguinal canal. Leiomyomas are benign smooth muscle tumors found in 20–30% of women older than 35 . Risk faInguinal masses in women have a long differential diagnosis list including inguinal and femoral hernias, lymphadenopathy, psoas abscess, femoral artery aneurysm, hydrocele of canal of Nuck, leiomyoma, leiomyosarcoma, endometrioma, and other round ligament lesions. Preoperative imaging can be helpful in diagnosing rare causes of inguinal masses but are very rarely performed. However, when obtained, a CT scan can be useful in diagnosing a RL leiomyoma which appears as a well-circumscribed lesion with bright and heterogeneous enhancement, an appearance typical of but not specific to leiomyomas , 16. DefIn cases of RL leiomyomas, a fine needle or a core needle biopsy could potentially confirm the diagnosis preoperatively ; howeverAs described above, we felt it necessary to perform a formal hernia repair following the excision as the internal ring was vacated and the floor was weakened. It is our recommendation that whenever a lesion is excised from the inguinal canal and the round ligament removed, a formal inguinal hernia repair should be performed, preferably with mesh, in order to complete the procedure and prevent a hernia from occurring. However, other types of hernia repair without mesh could also be considered depending on surgeons' preference.


Cardiac lipomatous hypertrophy is a rare benign condition that usually involves the interatrial septum. Due to its benign nature it rarely requires intervention. Its presence outside the interatrial septum is reported infrequently. We present a case of lipomatous hypertrophy in the intraventricular septum that was complicated by a severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction. The symptoms significantly improved following the excision of the mass. In our case transthoracic echocardiogram was used to visualize the mass and measure the severity of the obstruction; Cardiac Magnetic Resonance Imaging was used to characterize the mass and histopathology confirmed the diagnosis. Cardiac lipomatous hypertrophy is a rare condition, which was first described in 1964 involving the interatrial septum (IAS). It is rarely diagnosed because of lack of specific clinical symptoms. The reported incidence on autopsy series was 1–2.2%. Histologically, cardiac lipomatous hypertrophy differs from a lipoma by its location in the IAS, absence of a capsule, and the presence of fetal fatty tissue. Its presence in the myocardium outside of the IAS has been reported very infrequently. Furthermore, it is typically a benign finding requiring no intervention. We present a case of lipomatous hypertrophy in the intraventricular septum complicated by severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction.A 54-year-old Caucasian male presented with eight-month history of progressively worsening shortness of breath. Five years before, on a routine physical examination, he was found to have a harsh systolic murmur along the left upper sternal border for which he underwent a transthoracic echocardiogram (TTE). The TTE showed mild systolic anterior motion of the mitral valve with significant left ventricular outflow tract gradient. A definite septal abnormality could not be excluded based on the TTE. Subsequently, he underwent a cardiovascular magnetic resonance imaging (CMR) to characterize the lesion and was found to have a basal anterior septal mass measuring 15.2 × 5.9 mm. The signal intensity by T1 and T2 was consistent with fatty tissue. Moderate LVOT acceleration was also noticed in the vicinity of the septal mass. The LVOT gradient was further evaluated by an exercise stress echocardiography which demonstrated a resting LVOT gradient of 10 mmHg that significantly increased to 69 mmHg at 79% of maximal predicted heart rate and returned to baseline at a heart rate of 88 bpm. It also showed a significant bileaflet systolic anterior motion of mitral valve at peak heart rate as compared to the baseline without any significant mitral regurgitation. No intervention was performed due to lack symptoms and good functional capacity. He was followed up with a CMR every other year, which showed small increments in size but he remained clinically asymptomatic.Eight months prior to this report, he started experiencing dyspnea with exertion which progressively worsened. During this presentation his cardiac examination demonstrated a loud crescendo-decrescendo 5/6 systolic ejection murmur along left sternal border that worsened with Valsalva. A TTE was performed which demonstrated the previously visualized nonmobile septal mass  and a trThe patient was referred for surgical evaluation and he underwent a surgical resection of the mass via transaortic approach. Intraoperatively the mass was identified as a fibrofatty tissue, which was resected without any complications. His postoperative course was uneventful. His symptoms significantly improved after the surgery permitted him to resume work.Histopathological examination of the excised specimen showed benign hypertrophic myocardial muscle cells which revealed variation in the size and shapes of the nuclei. These myocytes were dispersed in between unencapsulated mature adipocytes with vacuolated cytoplasm. There were no mitotic figures or signet ring structures and the adipocyte nuclei were not hyperchromatic or indented, which ruled out liposarcoma , it has never been reported to be caused by a lipomatous hypertrophy. An LVOT blood outflow acceleration due to the presence of an obstruction causing lift and drag resulting in the systolic anterior motion of the mitral valve (Venturi Effect) can explain the high peak aortic pressure gradient as seen in our case . Surgica


Published reports concerning internal hernias after extraperitoneal stoma construction are scarce. In our present report, we describe the case of a 56-year-old man who was referred to our hospital for the treatment of rectal cancer. He underwent abdominoperineal resection of the rectum with sigmoidostomy using an extraperitoneal route. On the ninth postoperative day, the patient experienced sudden and intense abdominal pain and was diagnosed with strangulation of the small intestine due to a stoma-associated internal hernia. Therefore, an emergency laparotomy was performed. The surgical findings showed that the small intestine protruded through the space between the sigmoid colon loop and the abdominal wall in a cranial-to-caudal direction. The strangulated portion of the small intestine was recovered, and the orifice of herniation was closed. No recurrence of internal herniation was observed during the follow-up period. Abdominoperineal resection (APR) was first described in 1908 by Miles -4. In 1918 F]-fluorodeoxyglucose positron emission tomography scans. The patient underwent APR of the rectum with lateral lymph node dissection on both sides (Figure A 56-year-old man who had a positive result on a fecal occult blood test was referred to our hospital. Advanced rectal cancer 2 cm from the anal verge was detected by colonoscopy and barium enema Figures A and 1B.Although the patient’s postoperative course was uneventful, he experienced a sudden onset of intense abdominal pain on the ninth postoperative day. Emergency CT showed the loop of a poorly enhanced edematous small intestine that was strangulated by the sigmoid colon and abdominal wall Figures A and 2B.Although intestinal obstruction has previously been reported to occur in 3.5% to 7.2% of cases after colostomy , the majIn the present case, because metastasis to the lymph nodes surrounding the internal iliac artery was suspected, we performed lateral lymph node dissection, which shortened the pelvic wall peritoneum. Although we managed to close the retroperitoneum, the closure was under strong tension, and the closure was disrupted at the time of the second surgery Figures A and 3B.The development of laparoscopic surgery in recent years has contributed to the difficulty in closing the space lateral to the sigmoid colon. Laparoscopic colectomy, including APR, has recently become very popular, and sigmoidostomy is constructed through an intraperitoneal route in many institutions. Although the laparoscopic extraperitoneal colostomy technique has been developed to avoid parastomal herniation -9, it reInternal hernia associated with extraperitoneal stoma is a rare but possible complication that should be considered when treating a patient with ileus caused by small-intestine obstruction after APR.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.APR: Abdominoperineal resection; CT: Computed tomography.The authors declare that they have no competing interests.YY conceived of the study, collected data and drafted the manuscript. KK conceived of the study and corrected and revised the manuscript. SK, SY, JT, TT, TK, HN, TK and HY helped in drafting the manuscript. SI, ES, JK and TW corrected and revised the manuscript. All authors read and approved the final manuscript.


Fibrous dysplasia (FD) is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. Female patient, white, 20 years old, attended the dental clinic reporting a slow increase in volume in the right mandible region over the last 5 years. She was examined by imaging: the panoramic X-ray revealed a lesion with the appearance of ground glass while the cone-beam computed tomography showed an extensive lesion in the region of the right hemimandible. The histopathological examination was compatible with fibrous dysplasia. Bone gammagraphy was indicated, plus an endocrinological study to eliminate polyostotic forms, which produced a negative result. Monostotic fibrous dysplasia in the right hemimandible was diagnosed. Conservative surgery was carried out and after 1 year recurrence of the tumour was observed. We may conclude that conservative surgery might not be the best choice for treatment for monostotic fibrous dysplasia in the mandible and that other options must be considered, such as radical surgery or the use of bisphosphonates. In our study, we may also conclude that it is very important to explain to the patient the possibility of recurrence of the lesion and the need for monitoring with periodic imaging studies. Fibrous dysplasia (FD) is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue , 2.α-subunit of the stimulatory G protein (GSα) activates the adenylyl cyclase, which in turn catalyzes the formation of cyclic adenosine monophosphate from ATP [Sα (GNAS1), when it suffers a mutation, is associated with the FD disorder spectrum. This mutation occurs in a somatic cell producing somatic mosaicism, in which the cells descending from the mutated cell produce abnormal characteristics, while the cells descending from the unaffected cells produce normal characteristics [S) in osteoblast progenitor cells is thought to be the result of an increase in their proliferation and abnormal differentiation. Studies have established a link between the GSα mutation and the increased production of interleukin-6 stromal cells, which promotes osteoclast activity [FD is caused by the genetic mutation of the cell-surface receptor guanine nucleotide protein (G protein) . The α-sfrom ATP . The generistics . The incactivity .It generally appears in the first or second decade of life; it is asymptomatic, progresses slowly, and affects women twice as frequently as men , 2. It aThe object of this paper is to present a case of recurrent monostotic fibrous dysplasia in the mandible region, which was studied by imaging, with incisional biopsy for definitive diagnosis. Bone remodelling was carried out to improve the patient's facial contour.Female patient, white, 20 years old, with no systemic history, attended the dental clinic in 2005 reporting a slow, painless increase in volume in the mandible region (right side) over the last 5 years, producing facial deformity. During physical examination facial asymmetry was observed due to an increase in the size of the mandibular body on the right side, of solid consistency, pain-free, and without adenomegalies. Intraoral examination showed a hard-textured increase in the alveolar ridge on the right side; the adjacent mucous tissue was normal. A panoramic X-ray was done  in whichWhen the patient returned for control four years later, in 2009, she expressed interest in treating the facial deformity produced by the pathology. She reported that she had received surgical treatment to remove two teeth which were retained in the mandible on the right side. After clinical examination, a panoramic X-ray was done  in whichCraniofacial bones are affected in approximately 30% of cases of FD . In a reAsymmetry and oedema are the most common complaints when FD is found in the bones of the facial skeleton . Other sTumours in young patients or recent tumours present a cyst-like appearance. The smallest mandibular tumour was 1.5 cm in diameter . In geneThe preferred imaging examination is cone-beam computed tomography, since it offers a broader view of the extent and location of the lesion, which helps in planning the surgical procedure . The mosHart et al. , in a stSome authors report that growth of this lesion tends to stop around puberty ; howeverPrimary treatment of mandibular lesions always includes correction of asymmetries and deformities and/or prevention of functional problems by conservative surgery which can be carried out regardless of the size of the lesion; this is the technique indicated by many authors , 9, 20. Considering that there was recurrence in our case and that similar cases are reported in the literature, we may conclude that conservative surgery may not be the best choice for treatment for monostotic fibrous dysplasia in the mandible and that other options must be considered, such as radical surgery or the use of bisphosphonates. It is important for the dentist to analyze each case of FD, taking aspects like gender, age, recurrence, extent of the lesion, and the involvement of adjacent structures into consideration in order to choose the best treatment for each patient. In our study we may also conclude that it is very important to explain to the patient the possibility of recurrence of the lesion and the need for monitoring with periodic imaging studies.


Polyorchidism is a very rare anomaly that is defined by the presence of more than two testes. Although its presentation is primarily as triorchidism, cases of four testes have also been reported in the literature.In this report, we describe color Doppler ultrasound findings in two cases. Patient 1 was a 37-year-old Arabic man with a scrotal mass and a double testicle in the right hemiscrotum visualized by ultrasound. Patient 2 was an 11-year-old Arabic boy with an inguinal mass resulted to be an additional testicle in the inguinal canal. The echogenic texture and vascular flow of supernumerary testicles in question were similar to those of the normal testicles; however, their size was smaller. After 3 years of follow-up, the tertiary testes in the two patients remained stable in both size and echogenicity.Ultrasound plays a crucial role in the evaluation of masses. Notably, inguinal or scrotal masses should not always be considered as lymph nodes or tumors. Indeed, a radiologist should always keep polyorchidism in mind when such masses are encountered. Polyorchidism or supernumerary testicles are defined by the presence of more than two testicles. It is a very rare anomaly. Only about 200 cases have been reported in the literature so far. In this report, we report the color Doppler ultrasound findings in two patients with polyorchidism that presented as asymptomatic masses, and we review the pertinent literature.A 37-year-old Arabic man was referred by his family physician to our ultrasound unit for evaluation of a palpable, painless mass in the right scrotum that he had had for at least 3 years at least. Color Doppler ultrasound Figure  showed aIt was therefore presumed to be a third testicle, and the patient was managed with follow-up only, initially every 3 months and then after one year every 6 months. The sonographic findings were stable during this follow-up period.An 11-year-old Arabic boy was referred electively to our ultrasound unit for evaluation of a painless mass in the right inguinal area that was incidentally detected by his father while they were bathing. A grayscale ultrasound showed a normal testicle on each side of the scrotum Figure .An ultrasound of the boy’s right inguinal canal showed a 0.7cm oval mass resembling a normal testicle in its echotexture Figure . It was A power Doppler ultrasound showed a normal flow in this mass Figure .Figure 2This mass was diagnosed as a retractile third testicle, and no change in size or echogenicity has been observed during more than 3 years of follow-up.The first histological description of polyorchidism was published in 1880 by Ahlfeld. The first clinical case was reported by Lane in 1895 . UsuallyThe color Doppler ultrasound appearance of polyorchidism is a scrotal oval mass with a flow similar to that of the normal testicle. Spermatogenesis in the supernumerary testicle is normal in about 50% of cases. This fact explains the persistent fertility of these patients after bilateral vasectomy. Indeed, when bilateral vasectomy is performed, fertility remains normal.Most patients with polyorchidism have a normal 46,XY karyotype. However, chromosomal abnormalities such as a 46,XX karyotype with XY mosaicism and deletion of the long arm of chromosome 21 have been reported. In polyorchidism, secondary sexual characteristics are the same as in typical individuals of similar age .Leung  and SingPolyorchidism is usually diagnosed on the basis of color Doppler ultrasound, further supported by magnetic resonance imaging (MRI) . In bothThe management of polyorchidism should usually be resection of the dysplastic testicle without a duct; otherwise, the supernumerary testis should be retained and followed up by ultrasound yearly because of its contribution to spermatogenesis. However, any suspected malignancy of the supernumerary testicle should be managed by orchiectomy. If malignancy is not suspected, the patient can be followed up conservatively, as we did in our two patients.In one study, Bergholz and Wenke reviewed 140 histologically confirmed cases of polyorchidism . TriorchThe most common location of the supernumerary testis is within the scrotum. However, it may be present with a maldescended testis, or it may itself be maldescended in patients with two testicles in the scrotum.Anomalies associated with polyorchidism generally include inguinal hernia in cryptorchidism, testicular torsion and microlithiasis .Polyorchidism in general is an incidental finding associated with hydrocele, epididymitis, varicocele, infertility, retractile testes and hypospadias. Reports in the literature also have described an increased risk of testicular malignancy (6.4%) in the presence of polyorchidism . MalignaLeung  recommenAnother condition that mimics polyorchidism is splenogonadal fusion, which is essentially a very rare congenital anomaly. In this condition, the spleen, gonad, epididymis and vas deferens are fused. Sonography reveals a mass with a testicle of similar echogenicity, and it may mimic the appearance of polyorchidism. Hence, when a splenogonadal fusion is suspected, a technetium sulfur colloid scan should be performed to confirm the presence of ectopic splenic tissue .Polyorchidism is a very rare anomaly. Doppler ultrasound is the preferred method for evaluation, diagnosis and follow-up of this condition, and it can help avoid the need for surgery. Radiologists and urologists must be aware of this condition when evaluating any scrotal mass. These patients should be followed up yearly by ultrasound for life, but young patients with a third testicle that does not contribute to spermatogenesis, as in our 11-year-old patient, should undergo resection. MRI is rarely needed nowadays. Furthermore, excision is recommended when any change in echogenicity, shape and focal masses occur in the supernumerary testicle.Written informed consent was obtained from patient 1 and the legal guardian of patient 2 for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Sarcoptes Scabiei. About 300 million cases of scabies are reported annually. Scabies usually presents clinically with an erythematous excoriated papulovesicular rash, burrows, nodules and hyperkeratotic lesions in specific body areas.Scabies is a common parasitic infection caused by the mite A rare presentation of scabies is the bullous pemphigoid-like bullous scabies. So far, to the best of our knowledge, only 32 cases of bullous scabies have been reported in medical literature, of which only 11 were under 60 years of age at the time of initial presentation. This is the first case of bullous scabies being reported from Pakistan.Herein we discuss, with reference to the existing literature, the case of a 23-year-old Punjabi male who presented with a 3 day history of a tense, non-erythematous, non-tender bulla measuring approximately 0.5 cm x 0.8 cm on the right foot near the interdigital cleft. He was diagnosed to have bullous scabies.Sarcoptes Scabiei mites and eggs should be taken.The diagnosis of scabies should be considered in all patients who present with tense bullous lesions accompanied by pruritus and a maculopapular rash. This is particularly relevant if these lesions do not resolve with steroid treatment. In such patients, in order to prevent a misdiagnosis of bullous pemphigoid, scrapings for  Sarcoptes Scabiei [Scabies is a common parasitic infection caused by the mite  Scabiei . It spre Scabiei .In adults, scabies presents as a small papulovesicular rash with a clinical picture comprising of pruritus, papules and nodules associated with inflammation . SubcutaPatients with scabies can develop various secondary lesions, such as folliculitis, eczema, pseudolymphoma and impetigo. Atypical presentations of scabies include urticarial rash, dermatitis herpetiformis and Darier’s disease .Bean et al. [A rare subtype of scabies is bullous scabies which occurs concurrently with, or after, the appearance of scabietic lesions. It presents as bullous pemphigoid-like bullae, and at times can even induce the formation of true bullous pemphigoid lesions . The firn et al.  and to tn et al. ,4,8. Outn et al. ,4,8.Herein we report the case of a 23-year-old male who presented with a bullous lesion on the right foot. He was diagnosed to have bullous scabies.A 23-year-old male belonging to the Punjabi ethnic group presented with a 3 day history of a large, tense, non-erythematous, non-tender blister on the right foot. The patient also complained of lesions on the hands for the preceding week. The lesions were pruritic (more so at night) and there was no history of fever, trauma or burns. The patient denied any history of unprotected sexual contact. His past medical and surgical history was unremarkable, as was his drug history. Family history was positive for diabetes mellitus and asthma. He had no known allergies, was a nonsmoker and did not have any addictions. Review of systems was also unremarkable.On examination, the patient was hemodynamically stable. Cutaneous examination revealed erythematous, excoriated maculopapular lesions and burrows on the wrists and the interdigital areas of both the hands. A tense bulla measuring approximately 0.5 cm x 0.8 cm was noted on the plantar surface of the right foot near the interdigital cleft Figs.  and 2. IThe differential diagnosis based on this initial encounter included bullous scabies, bullous impetigo, frictional bulla and bullous pemphigoid.Sarcoptes Scabiei in scrapings from both the points. Direct swabs from the hand lesions and the bulla did not show any bacterial or fungal growth upon culture.Scrapings were taken from the maculopapular lesions on the hands and the bulla on the foot; direct microscopy, prepared from 10 % potassium hydroxide (KOH), revealed mites and eggs of Based on the history, examination and investigations’ findings, the final diagnosis of bullous scabies was made.The patient was treated with whole body applications of 5 % topical permethrin and local application of anti-histamine lotion. Complete remission of the lesions was noted after 3 weeks. The patient remained disease free for upto 18 months of follow-up.Bullous scabies usually occurs in the elderly population. The cases that we came across during our review had a predominantly male predilection . Out of the 32 cases reviewed, most (21 out of 32) of the patients were older than 60 years. It usually presents as intensely pruritic bullae, which may be tense or flaccid and at times may have associated hemorrhage. They are usually present in scabies prone areas and may or may not have associated classic scabietic lesions .Staphylococcus Aureus superinfection of mite lesions is one mechanism that can result in vesicle formation; this is similar to the bullae formation in bullous impetigo [The pathogenesis of bullae formation in scabies is not entirely clear and a number of theories have been proposed to explain their pathogenesis. impetigo . The secVarious mechanisms have been proposed for the production of these antibodies. According to one hypothesis, antibodies could be produced due to lytic destruction of the basement membrane by mites’ enzymes (leading to antigenic changes) . The resAnother theory suggests that the mite may elicit antibodies that cross-react with the basement membrane zone (BMZ) antigens (antigen mimicry) ; these aBrawan, et al. [On direct immunofluorescence, complement component 3 (C3) and Immunoglobulin G deposition is often seen in bullous scabies. Out of the 25 cases in which direct immunoflouresence was performed, 9 had IgG deposition and C3 deposition was present in 13 cases. The pattern of IgG deposition was linear in 8 cases and granular in 3; the 2 cases reported by , et al.  showed bThe various signs that have been used to confirm the diagnosis of bullous scabies include bullous eruptions over sites of the body that are usually prone to scabies, involvement of genital organs, nocturnal itch, improvement after application of antiscabietic medications and detection of the scabies mite on histopathology sections of lesions .Bullous scabies is usually treated in the same way as the classical form of scabies . AccordiIn some cases of bullous scabies, a short course of oral steroids may also be required . In moreOut of the 32 cases reported so far, 8 patients were treated with gamma benzene hexachloride, 7 with benzyl benzoate, 5 with permethrin, and 3 with oral ivermectin; combinations of permethrin with oral ivermectin, oral and topical steroids, gamma benzene hexachloride or sulfur were also used in some cases ,4,8.Sarcoptes Scabiei mites and eggs.It is suggested that the diagnosis of scabies be considered in all patients who present with tense bullous lesions accompanied by pruritis and a papular rash. This is particularly relevant if these lesions do not resolve with steroid treatment. Suspecting scabies in such patients is therefore of paramount importance, as it may not only co-exist with bullous pemphigoid, but is also an important possible precipitant for this condition. In such patients, consequently, it is important to test the lesions for Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Gangliogliomas are uncommon tumors of the central nervous system and rarely occur in the lateral ventricle or present with drop metastasis. We report a 49-year-old male who presented with a six-week history of left leg pain and numbness. Clinical examination revealed no focal neurological deficits. Magnetic resonance imaging (MRI) demonstrated enhancing nodular lesions in the sacral spine abutting the S2 nerve root. Further imaging of the neuroaxis demonstrated a cystic lesion in the left frontal horn of the lateral ventricle. Gross total surgical resection of the ventricular lesion was performed through a transcortical approach, followed by resection of the sacral spinal drop metastasis in a staged manner. A histopathological analysis revealed the diagnosis of low-grade ganglioglioma. To our knowledge, this is the first reported case of a low-grade intraventricular ganglioglioma presenting with symptoms associated with drop metastasis in an adult patient. Gangliogliomas are slow-growing, generally benign tumors of the central nervous system (CNS) that consist of both glial and ganglion cells. The incidence is 0.4% to 0.7% of all CNS tumors , 0.4% toA 49-year-old male presented in January 2013 with a six-week history of excruciating left leg pain that radiated down the back of his thigh and anterior portion of the leg. The pain was sharp, constant, and refractory to pain medications. The patient also endorsed numbness over the left shin but denied any focal weakness in his lower extremities, or bowel or bladder dysfunction. He required a wheelchair secondary to severe pain for 48 hours prior to presentation in the emergency department. His past medical history was significant for four vessel coronary artery bypass graft surgery in 2000, diabetes, and carpel tunnel release surgery. On physical exam, the patient was neurologically intact with full strength in bilateral upper and lower extremities.A contrasted MRI of the lumbar spine revealed enhancing nodular lesions located intradurally at S1-S2 and S2  was placed through the corticectomy tract into the left frontal horn. Hemostasis was achieved, and the craniotomy was closed in standard fashion.Postoperatively the patient was taken to the neurosurgical intensive care unit. The EVD was gradually weaned, and the patient was transferred to a floor bed. He was eventually discharged home with scheduled follow-up for resection of the sacral intradural tumors.The patient returned to the hospital two weeks following discharge for resection of S1 and S2 intradural tumors. An incision was performed in the midline at the level of the sacrum. Intraoperative fluoroscopy was used to localize the appropriate level. Laminectomies were performed at S1 and S2 to expose the thecal sac. Intraoperative ultrasound was then employed to visualize the tumors. The dura was opened and two densely calcified nodules were visualized; one on the left at the level of S1, and a smaller nodule on the right at the level of S2. Fragments of the nodules were sent for an intraoperative consultation, and the results revealed characteristics of a spindle cell tumor with prominent calcification and ganglion cells. Gross total resection of the left S1 nodule was achieved. However, because the right S2 nodule was completely blended in with a number of rootlets, this tumor was debulked but not completely resected. The surgical site was closed in standard fashion. The patient was admitted to a floor bed and was monitored for cerebrospinal fluid (CSF) leak for 48 hours. He was then allowed to ambulate. He remained neurologically intact with full strength and was discharged home with scheduled follow-up.The permanent sections of the intraventricular mass revealed a glial tumor containing numerous ganglion and glial cells, some with clear cytoplasm but without significant atypia. No necrosis, or vascular proliferation was apparent. Immunohistochemical analyses were performed which showed the glial cells to be positive for chromogranin, neurofilament, and weakly positive for calretinin. Other markers like glial fibrillary acidic protein (GFAP) and S100 highlighted few spindled cells. The MIB-1 proliferative index using Ki-67 immunohistochemical stain was low at a value of 3% Figure . InteresThis report describes the rare occurrence of a ganglioglioma of the left lateral ventricle and is the first report of such an intracranial mass exhibiting features of metastasis to the sacral spine in a 49-year-old male. Review of literature found only seven reported cases of lateral ventricle ganglioglioma  and one While the review of literature reveals several reports describing intramedullary spinal gangliogliomas of the cervical, thoracic, and conus medularis regions ,5,19, onGangliogliomas are rare low-grade neoplasms defined by the presence of dysplastic neuronal and glial cells that were first described by O.C. Perkins in 1926 . They arThe most common presenting symptom of intracranial ganglioglioma is a seizure, with an incidence rate of 65-92% ,7,19. GaWe report the first case of intraventricular ganglioglioma with sacral spinal metastasis in an adult patient. While the patient’s symptoms were only located in the lower extremity, radiological images were suspicious for drop metastasis. In this case, cranial imaging demonstrated an intraventricular lesion. The authors emphasize the importance of a complete evaluation in such cases where suspicion for drop metastasis exists.


Epithelioid angiosarcoma (EAS) is a rare disease which presents a great diagnostic challenge. The present study reports a case of EAS in the kidney in a 75-year-old male who presented with gross hematuria. An abdominal computed tomography scan revealed space-occupying lesions of the right kidney and renal cell carcinoma was suspected. Histological examination of the resected specimens showed pleomorphic epithelioid cells with vesicular nuclei, prominent nucleoli and eosinophilic cytoplasm that lined irregular vascular spaces. Immunohistochemical staining revealed that the tumor cells were positive for AE1/AE3, cytokeratin (CK) 7, vimentin, cluster of differentiation (CD) 31 and E-cadherin, but showed no staining for CD10, CD34, factor VIII, CK20, carcinoembryonic antigen or desmin. Based on the histopathological and immunohistochemical findings, the patient was diagnosed with epithelioid angiosarcoma. Postoperative radiation therapy was administered and no recurrence was observed six months after surgery. Angiosarcomas are rare, high-grade malignancies of endothelial origin. The rarity of angiosarcomas makes clinical diagnosis difficult. Histologically, angiosarcomas range from well-differentiated tumors with variable endothelial atypia to high-grade spindle cell neoplasms. Unlike the conventional appearance, a particular morphological subtype of angiosarcoma, in which the neoplastic endothelial cells have a predominantly epithelioid character, has been termed epithelioid angiosarcoma (EAS) . EAS mosA 75-year-old male presented at the Municipal Hospital  with the chief complaint of recurrent gross hematuria for approximately one month. An abdominal computed tomography (CT) scan revealed space-occupying lesions in the upper-mid section of the right kidney with mixed density . No lesiGrossly, the tumors presented as a large, necrotic, hemorrhagic mass in the upper-mid section of the right kidney and measured 4×4 cm in diameter . The pooEAS has a male predilection and generally occurs in adults, with the highest incidence in individuals in their seventh decade . A numbeet al (The diagnosis of EAS primarily relies on pathological examination. Brown et al  reportedet al . The preet al . CD34 poet al . In the et al –12.In conclusion, the present study has reported a case of EAS in the kidney and has reviewed findings from previous studies. Although these soft tissue lesions most commonly occur at other sites within the body, it is important that pathologists are aware that they may also occur in the kidney. In the present case, follow-up data suggested that early diagnosis may enhance survival.


Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is no established standard treatment and limited knowledge about the prognosis and biologic behavior of vaginal mesonephric adenocarcinoma.This report documents a new case of vaginal mesonephric adenocarcinoma diagnosed in a 54-year-old woman, and analyzes this in the context of all previously published cases.MRI demonstrated that the 2.5 × 1.8 cm tumor of the vaginal wall was invading urethra and bladder. Following surgical excision, histologic analysis determined mesonephric adenocarcinoma of the vagina, stage pT2 R1. In order to avoid the mutilating extended surgery which would be required to reach R0 and considerable impairment of quality of life, adjuvant radiochemotherapy was administered with external radiation and brachytherapy, including 5 cycles of cisplatin (40 mg/m²) for radiosensitization. After 4 years of continuous oncologic follow-up, the patient is alive and clinically free of disease.In this case it was shown that adjuvant radiochemotherapy with radiation and brachytherapy was effective to manage the surgical R1 situation and maintain the patient’s life quality. More published cases reports are needed to gradually substantiate optimal treatment strategies. Mesonephric adenocarcinoma (MA) of the vagina is a rare tumor of the female genital tract, with only six cases listed in MEDLINE as of March 2016 (Table The first link between adenocarcinoma of the vagina and intrauterine exposure to diethylstilbestrol (DES) was described in 1971 . DES is We present the case report of a 54-year-old woman with MA of the vaginal wall invading urethra and bladder and describe the course of treatment.A 54-year-old Caucasian woman  was referred to the Obstetrics/Gynecology Department because of vaginal bleeding after cohabitation. During clinical examination, a tumor of size 2.5 × 1.8 cm located underneath the urethra was visible. In pelvic MRI, invasion of the urethra and the urinary bladder by the tumor was described, without any evidence of suspect lymph nodes. A cystoscopy showed no pathologic findings. Biopsy of the tumor revealed vaginal adenosis with microglandular hyperplasia. However, the presence of an adenocarcinoma could not be excluded. Local excision of the lesion was performed in general anesthesia (ITN). Histopathologic workup confirmed infiltration of the urethra and of parts of the muscularis of the urinary bladder. Microscopically, the tumor showed a tubulocystic and papillary pattern, with scanty pale to eosinophilic cytoplasm with low nuclear atypia. The immunohistochemical profile of the tumor cells was positive for pancytokeratin and CK7, but for calretinin and vimentin only focal positive. Furthermore estrogen and progesterone receptor were negative and CD 10 was slightly positive.The final diagnosis was MA of the vagina, stage pT2 R1. Achievement of an R0 resection would only have been possible by extended, mutilating surgery resulting in a severely impaired quality of life. Hence, in the interdisciplinary tumor board the consecutive decision was made to treat in a conservative approach by adjuvant radiotherapy including sensitization with cisplatin in analogy to treatment strategies for gynecological adenocarcinoma .The planning target volume comprised the vulva, vagina, and the whole uterus, as well as bilateral inguinal, external, and internal iliac lymph nodes up to the pelvic inlet as the cranial field border. CT-based 3D-treatment planning was performed in supine position. External beam radiotherapy (EBRT) was delivered as intensity-modulated radiation therapy  with 15-MV photons on a linear accelerator  , in singMesonephric adenocarcinoma of the vagina is a rare entity and any knowledge about is it therefore very limited. Prognosis, biologic behavior, and treatment strategies are controversially discussed and due to the rareness of the disease, there is no established standard therapy. Moreover, the histopathologic findings are complex and immunohistochemical studies show different results , 12. TheThe Müllerian and Wolffian ducts originate from mesodermal tissue. These ducts develop in both male and female embryos. In females the Müllerian ducts develop to form the Fallopian tubes, uterus, cervix, and upper two-thirds of the vagina; whereas in males they regress. Conversely, the Wolffian ducts form the male internal genitals but atrophy in females due to the absence of testosterone . MAs mayCompared to the malignant mixed Müllerian tumor, it seems that MAs located in the female genital tract have a better prognosis .However, the optimal management strategy for MA remains uncertain. Treatment recommendations can evolve only gradually, based on episodic reports and, in principle, derived in analogy to allegedly comparable oncologic constellations. Radical surgery is suggested as a potentially curative option, particularly when malignant lesions appear well capsulated . In thisDespite a limited current follow-up time of 4 years, this favorable disease course supports the hypothesis that these rare tumors are sensitive to adjuvant chemoradiation. Adjuvant treatment should be considered in cases with a higher probability of microscopic remnants, thus potentially also avoiding mutilation without compromising oncologic outcome.


Clitromegaly can be congenital or acquired with the former type being more prevalent. The main etiology behind the acquired type is hormonal imbalance affecting mainly adult women. This type is seen mostly in association with polycystic ovarian syndrome, hyperthecosis, ovarian tumors, and clitoral cysts or it can be drug-induced. Clitoral leiomyoma is a rare benign tumor of female genitalia and is an uncommon cause of clitromegaly.We reported a 42-year-old premenopausal woman with a progressively increasing mass since five years ago, who had attended our clinic with urinary retention. It was a fungating mass with ulceration arising from the crus of the clitoris with the size was 11 × 9 cm. After detailed laboratory investigations, she had normal karyotyping. Hormonal assay for testosterone, dehydroepiandrosterone sulphate, and follicle stimulating hormone, luteinizing hormone, parathormone, and prolactin levels revealed values within the normal range. Twenty-four hours urinary excretion levels of free cortisol and ketosteroids were within normal limits. Beta-hCG level was also in normal range. Thyroid function tests and X-ray chest results were normal. Contrast enhanced computed tomography (CECT) of the abdomen showed no abnormality in adrenals and there was no pituitary enlargement on brain MRI. Pelvis MRI showed a large 11 × 9 × 8 cm clitoral mass. Diagnostic biopsy done from ulcer margin was suggestive of leiomyoma. The mass was completely excised preserving the tip of clitoris. The histopathology showed spindle-shaped cells arranged in palisading form. On immunohistochemistry, tumor cells were positive for smooth muscle actin (SMA) as well as for estrogen and progesterone receptor (ER/PR), confirming the diagnosis of leiomyoma. The patient was regularly followed, and was doing well with no voiding difficulty.We reported the world largest clitoral leiomyoma presenting with symptoms of acute urinary retention. MRI has important role in diagnosis and biopsy is confirmative with spindle-shaped cells arranged in palisading pattern and simple excision would be curative. Clitromegaly is defined as an unusual increase in the size of clitoris, which may be congenital or be acquired. In comparison to congenital types, acquired types are rare. the acquired types are commonly seen in association with polycystic ovarian syndrome (PCOS), cystic lesions, hyperthecosis, and neurofibromatosis or they can be related to intake of certain drugs such as Danazol . Herein,A 42-year-old premenopausal woman attended our emergency department with acute urinary retention. She also had complaint of swelling in the vulval region since five years ago, which was painless and gradually progressive. On local examination, there was an 11 × 9 cm fungating, nontender, solid mass arising from the clitoris . Pelvis 2, which is almost twice the normal size. The origin of the clitoral leiomyoma, unlike the vaginal leiomyoma, is not clearly determined and it is not related to mullerian tract. Although it is a matter of dispute, few studies suggested the surrounding mesenchyme of the urogenital sinus as a presumptive origin of clitoral leiomyoma  agonists or antagonists may be used as an adjuvant therapy . MRI has