Focal nodular hyperplasia (FNH) and hepatocellular adenomas (HCAs) constitute benign hepatic neoplasms in adults. HCAs are monoclonal neoplasms characterised by an increased predilection to haemorrhage and also malignant transformation. On the other hand, FNH is a polyclonal tumour-like lesion that occurs in response to increased perfusion and has an uneventful clinical course. Recent advances in molecular genetics and genotype-phenotype correlation in these hepatocellular neoplasms have enabled a new classification system. FNHs are classified into the typical and atypical types based on histomorphological and imaging features. HCAs have been categorised into four subtypes: (1) HCAs with HNF-1α mutations are diffusely steatotic, do not undergo malignant transformation, and are associated with familial diabetes or adenomatosis. (2) Inflammatory HCAs are hypervascular with marked peliosis and a tendency to bleed. They are associated with obesity, alcohol and hepatic steatosis. (3) HCAs with β-catenin mutations are associated with male hormone administration and glycogen storage disease, frequently undergo malignant transformation and may simulate hepatocellular carcinoma on imaging. (4) The final type is unclassified HCAs. Each of these except the unclassified subtype has a few distinct imaging features, often enabling reasonably accurate diagnosis. Biopsy with immunohistochemical analysis is helpful in difficult cases and has strong implications for patient management.• FNHs are benign polyclonal neoplasms with no risk of haemorrhage or malignancy.• HCAs are benign monoclonal neoplasms classified into four subtypes based on immunohistochemistry.• Inflammatory HCAs show an atoll sign with a risk of bleeding and malignant transformation.• HNF-1α HCAs are steatotic HCAs with minimal complications and the best prognosis.• β-Catenin HCA shows variable MRI features and a high risk of malignancy. Benign hepatocellular lesions in adults can be divided into two main categories according to their pathogenesis: regenerative lesions, composed mainly of focal nodular hyperplasia (FNH), and neoplastic lesions, corresponding to hepatocellular adenomas (HCAs) . FNH andFNH is defined as a nodule composed of benign-appearing hepatocytes occurring in a liver that is otherwise histologically normal or nearly normal. It is the second most common benign liver tumour after haemangioma with a prevalence of 0.9 % and commonly occurs in young females  . The majFNH is often an incidental finding at imaging. Distinction between FNH and other hypervascular liver lesions such as HCA, hepatocellular carcinoma (HCC) and hypervascular metastases is critical as the management differs considerably. FNH is asymptomatic in most patients, and in such cases no treatment is necessary. One third of patients may present with abdominal pain or a palpable mass. Typically, FNH follows a benign natural course and remains stable or may even decrease in size at follow-up examination .CTNNB1), TP53, APC or HNF1α. FNH typically shows dysregulation of the angiopoietin genes, which are responsible for the maturation of blood vessels. An increase in the angiopoietin expression results in uncontrolled maturation and remodelling of vessels, resulting in dystrophic vascular architecture typical of FNH [Genetic analysis of FNH failed to identify somatic gene mutations in the β-catenin gene CTNNB, TP53, Al of FNH . Wanlessl of FNH , 9. Artel of FNH .The β-catenin pathway is activated, including the downstream target, glutamine synthetase, which explains the polyclonal over-proliferation of hepatocytes. The molecular mechanisms of this activation are uncertain, but do not involve demonstrable mutations in β-catenin or Axin, unlike HCA . ImmunohCurrently, FNH is divided into two types: classic and non-classic or atypical. .Classic FNH is characterised by the presence of abnormal nodular architecture, malformed vessels and cholangiolar proliferation. One or more macroscopic central scars are present in most cases that contain fibrous connective tissue, cholangiolar proliferation with surrounding inflammatory infiltrates and malformed vessels. Arterial blood flows centrifugally from the anomalous central arteries. Approximately 50 % of lesions show some degree of fatty infiltration, as opposed to the surrounding liver, which shows signs of steatosis in less than 20 % of lesions. Both the classic and non-classic types contain variable amounts of Kupffer cells .Classic FNH has typical imaging findings enabling accurate diagnosis. FNH is found as an incidental iso- to hypoechoic focal lesion on ultrasound although the isoechoic nature often makes the detection of lesions difficult. A central vascular scar may be seen. Further evaluation with dynamic contrast-enhanced CT or MRI is warranted in most cases for definitive diagnosis. It appears as a lobulated iso- to hypodense lesion on unenhanced CT and shows homogeneous, intense arterial enhancement becoming isoattenuating in the portal and delayed phases with no washout. The central scar showing delayed enhancement because of myxomatous stroma can be seen in 20 % of cases .Magnetic resonance imaging (MRI) has higher sensitivity (70 %) and specificity (98 %) for FNH than US and CT. MRI features include T1 iso- or hypointensity (94–100 %), T2 slight hyper- or isointensity (94–100 %) and a hyperintense central scar (84 %). A scar is usually seen in FNH >3 cm . LesionsHepatobiliary-specific contrast agents are being increasingly used in differentiating focal liver lesions. FNH is characterised by an increased density of functioning hepatocytes and hence shows persistent enhancement (iso- or hyperintense) on delayed phase 20 min) gadoxetic acid-enhanced images  and MRI (22 %) 0 % and M, 27. TheA pseudo-capsule due to compressive effects on the adjacent hepatic parenchyma as well as the presence of dilated vessels and sinusoids around the lesion.Interval growth is considered an atypical and worrisome finding in FNH.FNH can rarely show intralesional steatosis, often due to extension from the underlying hepatic steatosis Fig. . Other pTelangiectatic FNH is an uncommon entity but the most common among non-classic FNHs. These lesions often show heterogeneity on pre- and post-contrast CT and MRI, and they are strongly hyperintense on T1- and T2-weighted images, often with an absent central scar. Intense arterial enhancement with persistent enhancement during the hepatic venous and equilibrium phases is seen. Recently, a molecular study identified several genetic similarities between telangiectatic FNH and HCA indicating most of these are actually inflammatory hepatic adenomas and have been misclassified as 'telangiectatic FNH' .Development of FNH in childhood and presence of symptoms can also be considered as atypical features.Approximately 20 % of patients have multiple FNHs, which are often of a non-classic subtype with atypical imaging features. Multiple FNH syndrome is defined as consisting of two or more FNHs in combination with hepatic liver haemangioma or vascular malformations or intracranial tumours , 32 Fig. Focal dTypical MRI features of FNH lesions, which include homogeneous iso- to hypointensity on TI-weighted images, homogeneous slight hyper- to isointensity on T2-weighted images and a central hyperintense scar on T2-weighted images, are seen in only 9–50 % of these lesions , 24. VarFNH with typical imaging features is managed conservatively and does not necessitate surgical intervention. Surgical excision is considered for symptomatic lesions likely from compression of adjacent structures or hepatic capsular stretching. FNH with atypical imaging features needs further evaluation in the form of additional imaging, percutaneous guided biopsy and follow-up. Suspicious lesions could be followed up or surgically excised .HCAs are rare benign monoclonal hepatic tumours that commonly occur in females who have been receiving oral contraceptives. The duration of oral contraceptive use and oestrogen content determine the risk of developing HCA. It rarely occurs in children and men  . Other rInitially three MRI patterns were identified corresponding to three pathologic forms, namely the steatotic, peliotic and mixed types . Later, Inflammatory HCAs are the most common subtype and account for about 40–50 % of all HCAs. Inflammatory HCAs include liver tumours previously referred to as “telangiectatic FNH” or “telangiectatic adenomas”. They occur most frequently in young females with a history of oral contraceptive usage and in obese patients. Patients may present with signs of chronic anaemia and/or systemic inflammatory syndrome characterised by fever, leukocytosis and elevated serum C-reactive protein [Sustained activation of the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway (JAK-STAT pathway), with resultant hepatocellular proliferation, is the proposed pathogenesis in the development of inflammatory HCAs. This can happen via two pathways: (1) somatic gain-of-function mutations involving the IL6ST gene, which encodes the oncogene gp130 in 60 % of cases, and (2) STAT3 activation without mutations in gp130 in 40 % of cases . Often tInflammatory HCAs appear heterogeneous with areas of congestion and frank haemorrhage on gross pathology. Intense polymorph nuclear infiltrates, marked sinusoidal dilatation and thick-walled arteries are seen on histopathology. Immunohistochemistry shows homogeneous glutamine synthetase and β-catenin staining .On imaging, inflammatory HCAs manifest as hypervascular liver masses with persistent enhancement on portal venous and delayed phase images. They show diffuse T2 hyperintensity and iso- to mild T1 hyperintensity. Focal areas of microscopic fat may be seen in a small (11 %) subset of patients. Sensitivity, specificity, positive and negative predictive values of marked T2 hyperintense signal with delayed persistent enhancement are 85.2, 87.5, and 88.5 and 84 %, respectively, for the diagnosis of inflammatory HCAs . The atomaturity-onset diabetes of the young (MODY), type 3 and familial hepatic adenomatosis has been reported [HNF-1α-mutated HCAs are the second most common subtype and constitute 30–35 % of all HCAs. These develop exclusively in female patients with a history of oral contraceptive use and the tumours are multiple in about 50 % of patients. Often these are incidentally discovered on CT performed for other reasons and are asymptomatic. An association with reported , 43, 44.TCF1 gene inactivating hepatocyte nuclear factor 1α (HNF-1 α) is the primary event and could be somatic (90–95 %) or germ line (up to 10 %) in origin. The resultant non-functioning HNF-1α protein promotes lipogenesis and hepatocellular proliferation. Also there is suppression of liver fatty acid-binding protein, resulting in impaired fatty acid transport in hepatocytes, leading to intracellular fat deposition. Oestrogens act as endogenous genotoxic agents and cause hepatocyte proliferation by somatic mutations in the TCF1 gene [Biallelic-inactivating mutation of the CF1 gene , 36, 38.Excessive lipid accumulation in the tumour hepatocytes is typical on histopathology. No portal tract elements or cytological abnormalities are seen. Immunohistochemistry shows characteristic absence of liver fatty acid-binding protein .On MRI, these tumours show predominantly hyper- or isointensity on T1 with a diffuse signal drop on chemical shift imaging (CSI) because of intracellular steatosis. T1 hyperintensity could also be due to glycogen and haemorrhage. Iso- to slight hyperintensity is seen on T2. Moderate enhancement in the arterial phase with no persistent enhancement in the portal venous and delayed phases is typical Fig. . Sensitiβ-Catenin-mutated HCAs are the third most common subtype and constitute 10–15 % of all HCAs. These occur more frequently in males and are associated with male hormone administration, glycogen storage disease and familial adenomatosis polyposis . The incβ-Catenin is encoded by the catenin β 1 gene (CTNNB1), located at chromosome 3p21. Mutation results in sustained activation of β-catenin protein, resulting in uncontrolled hepatocyte proliferation. Cytologic abnormalities, such as a high nuclear-cytoplasmic ratio, nuclear atypia and acini formation, are seen on histopathology and mimic well-differentiated hepatocellular carcinoma. Strong and diffuse positivity to glutamine synthase is characteristic of this subtype on immunohistochemical analysis .On MRI, these tumours show heterogeneous T2 signal, which can be iso-, hypo- or hyperintense relative to the liver and shows no intratumoral steatosis. Intense arterial enhancement is seen, which may or may not persist into the delayed phase. Portal venous washout can be seen mimicking HCC  Fig. 9)9). TumouHNF1 a, CTNNB1 or IL6ST mutations. The predominant molecular pathogenesis and clinical and radiological features of this subset of tumours are poorly understood and further research is warranted [This subtype constitutes approximately 10 % and does not show arranted .HNF1A gene and non-alcoholic fatty liver disease. HCAs in patients with hepatic adenomatosis may be of the inflammatory, HNF-1a-mutated or β-catenin-mutated subtypes, and their imaging appearances may vary accordingly [Hepatic adenomatosis has been defined as the presence of multiple adenomas (arbitrarily >10) without any history of steroid therapy or glycogen storage disease . It occuordingly   become hypointense on portal venous and delayed hepatobiliary phases with gadoxetic acid, unlike FNH Fig. . The abs(a) intratumoral bleeding with or without associated rupture and haemoperitoneum and (b) malignant transformation to HCC. Different subtypes of HCA show variable complication rates.The two major complications of HCAs are Haemorrhage can occur in 15–20 % of HCAs . BleedinMalignant transformation of hepatic adenoma to hepatocellular carcinoma has been variably reported to occur in 5–10 %, and a recent meta-analysis showed the risk of malignant transformation to be 4% in females and 47 % in males , 58. TheRecent classification of HCAs into different subtypes has allowed better understanding of the natural history and biological behaviour of these relatively rare tumours. This has great implications for the imaging and management of these tumours. Many of the previously followed treatment strategies such as surgical resection for all hepatic adenomas and liver transplantation for multiple adenomas are now changing as we have better insight into these tumours because of recent advances in molecular genetics and imaging . Early rThe management strategy is broadly based on the presence or absence of symptoms Fig. . IncidenIn symptomatic HCAs treatment depends on the duration and type of symptoms. Patients who are haemodynamically unstable because of intra- or extrahepatic bleeding require immediate treatment with either hepatic artery embolisation or surgery . ConservThere are no established guidelines on the optimal interval and duration of follow-up. Yearly imaging surveillance of HCAs is recommended for both solitary HCA and hepatic adenomatosis, and some authors recommend periodic surveillance until menopause .Management of hepatic adenomatosis should depend on the underlying histological subtype and size of the lesions rather than on their number. Symptomatic lesions and asymptomatic lesions larger than 5 cm require surgical resection. Liver transplantation is recommended if there is progressive liver failure or malignant transformation and is no longer advised for patients with asymptomatic familial adenomatosis . GeneticRecent advances in the molecular genetics and genotype-phenotype correlations in benign hepatocellular tumours have greatly improved our understanding leading to subtype recognition and better differentiation from other hypervascular lesions. Each of these tumour subtypes has distinct genetic mutations, molecular abnormalities, histopathological features, imaging findings, biological behaviour and prognosis. FNH typically does not bleed or undergo malignant change. HCAs with β-catenin mutations frequently undergo malignant change, inflammatory HCAs commonly bleed, and HNF-1α HCAs typically show a favourable prognosis. Many of these have reasonably specific imaging features permitting accurate diagnosis, which has significant implications for their management Tables .Table 1S


Carcinoma of the recto-vaginal septum is a quite rare location and related to peritoneal and primary ovarian carcinomas. There are only few reports in the literature with a very poor prognosis.Here we report the case of a 63 years old woman with past medical history of left oophorectomy presenting with a pelvic pain. The magnetic resonance imaging (MRI) demonstrated a 10 cm mass located in the recto-vaginal septum. A block resection was performed allowing the retrieval of a 10 cm solid tumor of the recto-vaginal septum. Peritoneal biopsies and the right ovary were normal the final diagnosis was cystadenocarcinoma of the recto-vaginal septum. The patient received adjuvant chemotherapy and displays no sign of recurrence 36 months after diagnosis.The management of recto-vaginal septum carcinoma with en bloc resection should be performed to avoid peritoneal spread and improve prognosis. Occurrence of a carcinoma of a recto vaginal septum is an exceptional presentation. Few cases of such disease have been described in the literature with a poor prognosis due to local early recurrence , 2. WhilA 63 year old patient presented to the clinic for pelvic pain. Her past medical history included a hypothyroidy and dyslipidemia. The BMI of the patient was normal . Her family history was notable for an aunt (father side) presenting with an ovarian carcinoma.Interestingly the patient had an oophorectomy performed 4 years before for a 6 cm left ovarian cyst by laparoscopy. During the previous surgery the cyst was adherent to the pelvic peritoneum and fallopian tube. There was an exophytic part adhering tightly to the rectum and Douglas Pouch. Rectal examination could not find any rectal or recto-vaginal nodule. After adhesiolysis a left adnexectomy was performed without particular difficulties. The pathology examination was consistent with a benign serous cystadenoma of the ovary. The left fallopian tube was normal. She underwent a CT scanner and a colonoscopy after the surgery which with no particular findings. She underwent regular gynecologic examination as well as yearly pelvic ultrasound.Four years later the pelvic ultrasound demonstrated a retro-uterine solid 11 cm cyst. Clinical examination found an immobile pelvic mass pushing the vagina anteriorly. There was no sign of vaginal or rectal invasion. Concordantly a pelvic MRI Figs.  confirmeA midline laparotomy was performed. On abdominal entry the mass seemed enclaved in the recto-vaginal septum Fig. . The rigShe underwent a hysterectomy, right adnexectomy, a coloprotectomy with a low colo-rectal anastomosis and protective ileostomy, omentectomy and peritoneal biopsies. The vagina could be preserved. Pelvic and para-aortic lymphadenectomy was performed. Postoperative course was unremarkable. Pathology exam revealed an adenocarcinoma of the recto-vaginal septum, grade 3 consistent with a serous ovarian carcinoma or a primary peritoneal carcinoma . A second para-aortic node was metastatic. The right adnexa was normal.The patient received 6 cycles of carboplatinum and taxol and 21 cycles of bezacizumab (AVASTIN®).On follow-up the patient is disease free  at 36 months after diagnosis.Serous carcinoma of the recto-vaginal septum is an exceptional presentation presenting multiple clinical challenges. Extra-ovarian Primary Peritoneal Carcinoma (EOPPC) with no ovarian lesion is a described entity . It dispAmong the differential diagnosis few patients had a mesothelioma which can be easily be identified on morphological and histological findings. These cases are however rare.In our case we could not differentiate between primary peritoneal versus primary ovarian tumor. However, the most likely hypothesis is the peritoneum and recto-vaginal septum dissemination of a left ovarian neoplasic foci resected previously. To reduce this risk, in postmenopausic women with a solid and cystic tumor adhered to the rectum, hysterectomy and double salpingo-ophorectomy might be better than simple left adnexectomy. The treatment of primary peritoneal carcinoma is similar to primary ovarian carcinoma with debulking surgery aiming at no residual disease associated to platinium-based chemotherapy. Our surgical approach of « en bloc » resection seems optimal. Indeed using the retroperitoneal sheath for all dissection leads to resection of such tumor without entry in the tumoral mass. Preserving the integrity of the tumor during surgery is mandatory to reduce local dissemination, residual disease and eventually local and distant recurrences.Most studies report a poor prognosis for primary peritoneal carcinoma related to extensive loco-regional disease with an overall survival around 21 months , 9. In oCystadenocarcinoma of the recto-vaginal septum is a rare entity. The surgery should emphasize a retroperitoneal approach with an en-bloc resection to avoid as much as possible tumor rupture. Staging and adjuvant treatment should be performed as in primary ovarian carcinosis. The description of more cases would lead to a better understanding of the specificity of such location regarding diagnosis, management and prognosis.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.


Splenic hamartoma is a primary benign tumor of the spleen, which is often found incidentally. Splenic hamartomas are very rare, with approximately 150 cases documented in the literature to date. They represent benign vascular proliferation. Histological findings consist of disorganized stroma and vascular channels of varying width, with or without lymphoid follicles.We present the case of a 39-year-old Greek woman, with no significant medical history, who was diagnosed incidentally with an enormous splenic hamartoma on computed tomography, finally confirmed by surgery and histopathology. Hamartomas are benign lesions, and it is important to differentiate them from malignancy.Hamartoma represents a rare vascular entity characterized by a cluster of differentiation 8-positive immunophenotype. It is usually asymptomatic but large hamartomas may present with symptoms such as hemopoetic disorders, which resolve after splenectomy. It is important for radiologists to be able to differentiate splenic hamartoma from malignant entities. In terms of structure and function, the spleen consists of white and red pulp . White pVascular neoplasms are the most common primary neoplasms of the spleen. Spleen hamartoma was described first in 1861 by Rokitansky , and hasBenign splenic tumor-like masses are generally rare, with an incidence of seven cases in 100,000 autopsy specimens, and most are cysts or hemangiomas. Hamartomas are extremely rare, with an incidence of three cases in 200,000 splenectomies .With the rapid improvement of imaging modalities, the detection of small and asymptomatic lesions is now easy, and a differential diagnosis can be achieved in a timely manner with high accuracy.A 39-year-old Greek woman, with no remarkable medical history, presented to the emergency room of our hospital with diffuse abdominal pain and a mass-like distention of the left side of her abdomen. No weight loss was reported.On physical examination we found a big painless, palpable mass on the left side of her abdomen. She had normal vital signs and laboratory findings were normal, except for mild leukocytosis.An abdominal ultrasound showed a huge, mostly hypoechoic, mass that was impossible to characterize. Its origin was also difficult to define.Abdominal computed tomography (CT) was performed, and images before the administration of contrast material showed a slightly hypodense, well-circumscribed, encapsulated mass on her left abdominal side, measuring 23×17×23cm  showed a high signal intensity mass on T2-weighted images, presenting intense gadolinium enhancement Figures  and 5. THistological examination revealed disorganized red pulp-like stroma with lipocytes and disorganized vessels of variable width, lined by slightly plump endothelial cells without atypia, with or without white pulp Figure .After surgical intervention, additional CT imaging was performed as a follow-up. This showed normal post-surgical evolution, with her other organs in normal positions and only some post-surgical lesions, such as adipose tissue thickening and fibrous remnants Figure .Figure 1The pathogenesis of hamartomas is controversial. Some consider hamartomas to be a congenital malformation of splenic red pulp, others a neoplasm or reaction to trauma , 4. The Splenic hamartomas must be differentiated from other vascular tumors of the spleen depending on the cell of origin, such as hemangioma, littoral cell angioma, lymphangioma, hemangioendothelioma or angiosarcoma . Solid mHemangioma is the most common benign tumor of the spleen, arising from endothelial cells of the sinusoids and usually measuring less than 2cm . LittoraHamartomas are quite rare lesions , which occur in any age group . They seGenerally, hamartomas are asymptomatic. Only large lesions are able to manifest clinical symptoms, such as palpable mass, spontaneous rupture with intra-abdominal bleeding, and hypersplenism  , 6, 9. Tet al.[Diagnosis is enhanced by imaging findings. Recently, Wang et al. describeet al., 10, 11.Finally, diagnosis is confirmed by histopathological examination and is enforced by immunohistopathology, with a splenic hamartoma having a cluster of differentiation 8 (CD8)-positive immunophenotype .Hamartoma represents a rare vascular proliferation characterized by CD8-positive immunophenotype. It is usually asymptomatic, but large tumors may present with symptoms such as hemopoetic disorders, which resolve after splenectomy. It is important for radiologists to be able to differentiate splenic hamartoma from malignant entities.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.


Functioning adrenocortical oncocytoma is very rare neoplasm. It is usually nonfunctional and benign and incidentally detected. Generally, these tumors originate in the kidneys, thyroid, parathyroid, and salivary or pituitary glands; they have also been reported in other sites including choroid plexus, respiratory tract, and larynx. Histologically, they are characterized by cells with eosinophilic granular cytoplasm and numerous packed mitochondria. We reported a case of a 44-year-old female who presented with Cushing syndrome for hypersecretion of cortisol due to adrenocortical oncocytoma. Magnetic resonance of abdomen revealed a right adrenal mass. Laparoscopic adrenalectomy was performed and the tumor was pathologically confirmed as benign adrenocortical oncocytoma. After surgical treatment, Cushing's syndrome resolved. Adrenocortical oncocytoma is a very uncommon neoplasm, usually considered as benign and nonfunctional. Oncocytoma is, in the majority of cases, a benign tumor originating in the kidneys, thyroid, parathyroid, and salivary or pituitary glands , 2; it hβ-blockers, sartans, thiazide-like diuretic, calcium antagonists, α1-blockers, and salicylate drugs. Metabolic disorders were diabetes mellitus type 2 and dyslipidemia treated with metformin, insulin, and statins, respectively. The patient underwent laparoscopic cholecystectomy for lithiasis five years before. Physical examination revealed truncal obesity with moon face and dorsal fat pad  and thin extremities. The skin showed spontaneous ecchymosis and purple striae on the abdomen. Another sign was hirsutism with increased facial hair. Muscles were hypotonic and hypotrophic and bilateral pretibial edema was present. BMI was 36,8 with a waist circumference of 133 cm. On admission, blood analyses were all within the normal values. The hormonal studies showed a normal value of adrenocorticotropic hormone (ACTH) and circadian rhythm of cortisol. The cortisol level was raised after dexamethasone suppression test (DST) with single dose of this steroid medication (1 mg) and the value was 16,6 μg/dL . Dehydroepiandrosterone-sulfate (DHEA-S), androstenedione (ADS), testosterone, progesterone (P4), and 17-hydroxyprogesterone (17-OHP) were normal. Blood and urine tests for adrenal medulla function and viral and tumor markers were within the normal ranges. MR of the abdomen was performed with intravenous paramagnetic contrast medium. It described an expansive lesion in the lodge of the right adrenal gland, measuring about 3 cm in maximum diameter; this mass had close relations with the posterior edge of the adrenal gland, from which it appeared to originate (A 44-year-old woman was admitted to our department presenting with sign and symptoms of Cushing's syndrome since about three years. The patient was a nonsmoker and nonalcoholic. Cardiovascular diseases were high blood pressure and hypertensive cardiopathy treated with riginate . The sigriginate . On cut riginate , vimentiriginate , and calAdrenocortical masses are often detected incidentally by imaging studies during the evaluation for unrelated problem. These “incidentalomas” can be cortical adenomas and carcinomas, cysts, myelolipomas, ganglioneuromas, pheochromocytomas, and adrenal metastases . Adrenal


Fibroepithelial polyps (FEPs) are rare benign upper urinary-tract neoplasms originating from mesodermal components of the ureteral wall covered in normal urothelial epithelium. Historically, these lesions have been treated with endoscopic, laparoscopic, or open means depending on the size, number, and morphology of the polyps. Digital ureteroscopy (DURS) offers many advantages over fiber-optic endoscopy, including superior resolution and potential ergonomic benefits, given the absence of external cameras and light cords. We describe a case involving multiple proximal ureteral FEPs treated with flexible DURS and the holmium (Ho) laser, in which the digital ureteroscope offered exceptional visualization of the FEP stalks allowing for straightforward resection and endoscopic removal. Medical history was positive only for hypercholesterolemia, and surgical history was negative. The patient denied smoking history and had no known family members with cancer or nephrolithiasis. His physical examination demonstrated stable vital signs, there were no palpable masses, but he did have intermittent colicky right flank pain.A 72-Serum creatinine was normal, and the urinalysis demonstrated gross blood without signs of infection. Urine cytology was overtly positive for numerous red blood cells, but no malignant or dysplastic cells were noted. A CT urogram (CTU) showed no urinary tract calculi but showed a 3 cm right proximal ureteral filling defect with mild hydronephrosis .Ureteroscopic evaluation was recommended given the CTU findings of a filling defect in the ureter along with the clinical findings of hematuria in this patient older than 70 years. The patient was counseled on the differential diagnosis, which included upper tract urothelial carcinoma (UTUC) and less likely benign etiologies, such as fibroepithelial polyps (FEPs), sloughed papilla, and blood clot secondary to upper tract hemangiomas or arteriovenous malformations. After general anesthesia was administered, rigid cystoscopy was performed with both 30° and 70° lenses. No bladder mucosal lesions were observed, and the ureteral orifices were in the orthotopic position, draining clear urine at the time. No lateralizing hematuria was noted. A right retrograde pyelogram was performed with an 8F cone-tipped catheter and ∼8 mL of 30% contrast diluted with saline at a 1:2 ratio. The image was created under fluoroscopy as the contrast was slowly injected. Findings were similar to the CTU.Given the absence of obvious distal ureteral pathology, we inserted a flexible digital ureteroscope with constant 8.5F diameter from tip to proximal shaft, per urethra. Using no-touch technique, we carefully entered the right ureteral orifice without an initial guidewire. Inspection of the entire collecting system showed three lesions, visually consistent with FEPs, in the right proximal ureter . One domThe postoperative course was uneventful except for some mild irritative lower urinary tract symptoms secondary to stent placement. The patient returned for stent removal in the office without difficulty. A follow-up visit after 4 weeks revealed a normal renal ultrasound with no residual hydronephrosis. The final pathology report demonstrated benign FEPs with no evidence of malignancy.1 The largest published series reported 22 cases over a 63-year experience.2 In this retrospective series, FEPs were located in the proximal ureter in 59% of cases, representing the most common location in the ureter, in contrast to UTUC, which, when present in the ureter, is found most often in a distal location. Renal colic was the most common presenting symptom in the Mayo series, whereas about 20% of patients with FEPs were found incidentally. The mean polyp length was 2 cm while the range was 0.5 to 4 cm in this group. After complete endoscopic resection, with 2 years of follow-up, less than 10% of patients had recurrence.FEPs are extremely uncommon with just more than 200 cases reported in the literature.3 and therefore, routine pathologic examination is mandatory for complete evaluation. Given the predominantly benign nature of FEPs, it is not surprising that they often are the reported false-positive etiology of upper tract filling defects after radical nephroureterectomy for presumed UTUC, thus strengthening the argument for initial ureteroscopic evaluation in this setting.The majority of FEPs show benign pathology. Descriptions of UTUC originating from FEPs exist in the literature,4 The initial flexible digital ureteroscopes were relatively larger in diameter than their fiber-optic counterparts. Recently, more practical smaller versions of the digital flexible ureteroscopes have been introduced with distal tip and proximal shaft diameters of 8.5F, allowing for more convenient upper tract access. Now with the best of both worlds, in terms of resolution and size, DURS enables the surgeon to clearly see upper tract pathology, including FEP stalks, thus potentially facilitating laser resection.Digital ureteroscopy has been in use for almost 10 years. With an extremely small digital camera at the distal tip of the endoscope along with light-emitting diode-powered light sources, resolution during upper tract endoscopy has arguably never been better.


Myxoma, a rare type of intracardiac tumor, forms a very small percentage of the cardiac cases. Reports of biatrial myxoma are rarer, with cases of single tumor reaching both atria being more common. Here, we present an unusual case of two independently growing atrial myxoma in a 29-year-old female. We emphasize that early recognition of symptoms, confirmation of diagnosis by transesophageal echocardiography, and prompt surgical excision remain vital in the management of such patients. The patient in the present case was managed successfully with no evidences of recurrence at the last follow-up.  Myxoma is a rare type of intracardiac tumor with an incidence of 0.0017% among the general population . AccordiA 29-year-old female presented to our institute with class III dyspnea and a history of cough for the past 1 year. She denied any past history of cardiac illness. Cardiac auscultation revealed a middiastolic murmur across mitral valve, followed by a tumor plop. Patient's electrocardiogram  showed nTransthoracic echocardiogram performed at the 6-month follow-up in our patient revealed normal cardiac chambers, improved left ventricular function, and no evidence of residual myxoma or myxoma recurrence. The patient was kept on a yearly follow-up and she has remained symptom-free for about 1.5 years after the procedure.Myxoma is the most prevalent primary tumor of the heart. Despite being benign, it may lead to embolic events or even sudden death. Early recognition, diagnosis, and prompt treatment remain vital to prevent life-threatening events in such patients .In general, patients with cardiac myxoma present with symptoms of hemodynamic obstruction, embolization, or constitutional changes. Transesophageal echocardiography is widely accepted as the ideal diagnostic tool for cardiac myxoma. It confirms the location and extension of the tumor as well as the site of attachment of the tumor. Once the diagnosis is established, immediate surgical treatment is indicated in all patients to avoid further tumor embolism and valve obstruction. Prognosis is excellent after surgical excision. After surgery, regular follow-up with serial echocardiography is also very important to detect recurrence , 2.The present case is rare and particularly interesting because there were two different tumors, coexisting in each atrium. Initially, the diagnosis was not clearly established on transthoracic echocardiographic examination and the second tumor in the right atrium was missed. On the other hand, transesophageal echocardiography was effective at detecting both atrial tumors and was particularly helpful in identifying the site of tumor attachment and characteristics of the mass. Therefore, we recommend the use of transesophageal echocardiography as an ideal noninvasive diagnostic modality in cases of cardiac myxoma. It is estimated that the average growth rate of left atrial myxoma ranges from 1.8 to 5.7 cm/year . AccordiAlthough prompt excision with high-risk cardiopulmonary bypass is the only acceptable mode of treatment , we beli


We report a case of a 58-year-old man who presented initially with lumbar pain.According to radiography, computed tomography, magnetic resonance imaging, and bone biopsy results, Ewing sarcoma (ES) was diagnosed. Tumor resection was performed, followed by chemotherapy and radiotherapy; pathology confirmed the diagnosis of ES. After surgery, the tumor recurred twice with progressive symptoms, meriting repeated excisional surgery. At the 4-year follow-up, the patient showed apparent improvement, with return of function and strength and resolution of pain.We discuss its clinical features and treatment in the light of the current knowledge. Here, we report an unusual case of a second lumbar (L2) ES, in which 3 surgeries were performed because of local recurrence. Surgical treatment yielded satisfactory results, with no involvement of other sites or organs.Ewing sarcoma is the second most common malignant bone tumor in children and adolescents.The patient was a 58-year-old man who had experienced low back pain for 3 months. He had been diagnosed and treated for lumbar intercalated disc herniation at other hospitals. However, his symptoms had worsened, with a decrease in lower limb muscle strength and neurogenic bladder and bowel dysfunction. The patient was transferred to our hospital and examined with radiography, computed tomography (CT), magnetic resonance imaging (MRI), 99mTc-methylene diphosphonate bone scan Figure , and bonThe patient visited the hospital again on August 8, 2008, because of progressively worsening pain in his waist and myasthenia of his limbs for 4 weeks. The results of CT and MRI (Figure On February 25, 2010, the patient could not easily move both of his lower extremities because of dysuric syndrome. On physical examination, muscle strength of the right lower limb was scored as 2 and that of the left was scored as 1. Results of other examinations showed that the tumor had recurred locally, mainly around the pedicle Figure . The sur6 ES is thought to arise from neural crest cells.7 MRI8 plays a predominant role in evaluating the features of the lesion and developing a preoperative surgical plan for the ES family of tumors. The signal intensity of spinal peripheral ES is usually homogeneous and is enhanced homogeneously after the administration of contrast agents. Spinal peripheral ES tends to involve paraspinal tissue, and spinal metastases can pathologically cause further paraplegia.9 Thus, we should consider surgical therapy when treating patients with spinal ES in order to improve quality of life by providing neurological improvement and spinal stabilization.10 Surgery and fixation are also good treatments to relieve the pain caused by spinal cord nerve root compression and increase spinal stability. After spinal ES resection, spinal stability is compromised to different degrees and must be restored by internal fixation to effectively protect the spinal cord. A clear diagnosis can be helpful for choosing the best treatment and preserving functionality for paraplegics, particularly as every minute counts.The spine is frequently involved in cases of metastatic ES, but primary involvement of the spine is much less frequent.11 Owing to the complex anatomical structure of the spine and insufficient surgical resection, recurrence is difficult to avoid. It is also difficult to abide by the principle of Enneking while removing the tumor as completely as possible and protecting the nearby nerves and major blood vessels. Assuming the patient's general condition allows for elective surgery, revision surgery is necessary for some patients to avoid the occurrence of paraplegia and prolong survival time.Surgical ablation is a common and effective treatment for primary spinal tumors, especially those with local invasion and metastases.


In February 2007, a 41-year-old Japanese male was admitted to our hospital with increasing upper abdominal pain. A contrast-enhanced computed tomography (CT) scan of the abdomen demonstrated a well-demarcated, hypodense cystic mass with a thickened wall in the mesocolon. The laboratory results were within normal limits, except for increased carcinoembryonic antigen, carbohydrate antigen 19-9, DUPAN-2 and SPAN-1. The patient was diagnosed as having a mesenteric malignant cyst, and during a laparotomy, a right hemicolectomy with mesenteric cystectomy was performed without rupture in March 2007. In the microscopic findings, there was a well-differentiated adenocarcinoma in the inner surface of the cyst and in the fibrous connective tissue of the hypertrophic cystic wall. The tumor cells were immunohistochemically reactive to cytokeratin (CK) 7, CK18 and CK20. No remnant of the malignancy was detected in the resected margin of the colon, cyst, liver or peritoneum nor was an uptake detected in an 18[F]-fluorodeoxyglucose positron emission tomography/CT examination of other organs. Finally, the malignancy was concluded to be a serous cystadenocarcinoma of the mesentery. Nineteen months after the operation, the patient died from peritonitis carcinomatosa due to a small intestine rupture. This report suggests mesenteric cystadenocarcinomas originating in the ovary, oviduct and intestinal mucosa, but these were ruled out in our patient. In this report, we discuss a case of the malignant transformation of a cyst into adenocarcinoma, which to our knowledge has never been previously reported in a male patient. Mesenteric cystadenocarcinomas originating in the ovary, oviduct, and intestinal mucosa have been reported , but theIn February 2007, a 41-year-old Japanese male presented with upper abdominal pain. A computed tomography (CT) showed a large, round unilocular boundary cyst in the mesocolon . The patThe resected specimen measured 7.5 × 8.6 × 9.5 cm in size; the wall of the cyst was 3 mm thick  and the Although a histological diagnosis of this case seems to be difficult, the primary site of this adenocarcinoma was assumed to be the peritoneum, because the primary lesions were found in no other organs during the operation. No remnant of malignancy was detected in the resected margin of the liver. Finally, we diagnosed cystadenocarcinoma of the mesocolon. Post-operative positron emission tomography(PET)-CT scan showed no detectable tumor cells in other organs. At five months after operation, the serum concentration of CEA and carbohydrate antigen 19-9(CA19-9) had decreased to within normal limits. At 19 months after operation, the patient was dead from acute panperitonitis due to small intestine rupture. In autopsy, recurrence of adenocarcinoma was found in the retroperitoneum, involving the hepatic hilum with perineural invasion and the pancreas head. Metastasis to fat tissue around the ureter had caused obstructive hydronephrosis. Adenocarcinoma of the peritoneal cavity had recurred extensively, resulting in lesions occupying the peritoneal cavity, which finally caused the man’s death. Although histological diagnosis of this case seems to be difficult, the primary site of this adenocarcinoma is assumed be the peritoneum, because primary lesions were found in no other organs at operation or autopsy.There is no consensus on the exact origin of cystadenocarcinoma of the mesentery, but it has been attributed to accidental implantations, malformation, the ovary, and the intestine . Only 19de novo, because of the similarities in the pathological immunochemical findings. These findings permit a good guess at the origin, but not a perfect one; none has been reported to date and, notably, this case is the first to be examined with an immunohistochemical approach. The cyst could have metastasized in the mesentery and increased in volume as a cystic formation with an epithelium like a positive pancreatico-biliary marker with a cancerous change of the normal cells. Finally, the benign mesenteric cyst might have transformed into cystadenocarcinoma of the mesocolon after the first or second scenarios described above.Our views on the reason why the pancreatico-biliary epithelia created a mesenteric cyst are as follows. Firstly, it seems possible that an implantation of a pancreatico-biliary fragment from an external injury could have metastasized in the mesentery and increased in volume as a cystic formation with a cancerous change of the normal cells. The cyst was filled with cloudy ‘café-au-lait' fluid; chylous cysts are usually associated with small bowel mesentery and serous cysts with the mesocolon, and hemorrhagic cysts are caused by trauma . TherefoThe recurrence pattern of previously reported cases was unclear, but peritonitis carcinomatosa associated with peritoneal dissemination occurred in our patient. Also, the surgical margin was negative in our patient, but there was a thin margin in the surgical cut-line of the gastrocolic ligament . TherefoConflict of interest: none declared.


Purpose. To study the clinical, radiological, and pathological characteristics of abdominal desmoplastic small round cell tumor (DSRCT) and investigate the optimal therapy modalities. Patients and Methods. A retrospective cohort study was performed on 12 abdominal DSRCT patients; all pathological, radiological, and prognostic data were analyzed. There were 3 patients (25%) with metastatic disease at presentation. In all 12 cases, 6 cases underwent operation and adjuvant chemotherapy . The other 6 cases were diagnosed by fine needle aspiration or exploratory laparotomy biopsy ; all cases received four to six courses of multiple agents chemotherapy, respectively. Results. All cases were finally diagnosed as DSRCT pathologically. Among group 1, all cases underwent en bloc resection  or tumor debulking  and, following four courses of multiple agents chemotherapy, Kaplan-Meier analysis revealed that 3-year survival was 50% in group 1 versus 16.7% in group 2 (P < 0.05). Gross tumor resection was highly significant in prolonging overall survival; patients with localized solitary lesion have a better prognosis, most likely due to increased feasibility of resection. Conclusions. DSRCT is a rare malignant tumor with poor prognosis. Surgical excision with combination chemotherapy as an adjunct is mandatory for nonmetastatic cases because these modalities used in isolation may have less impact. Desmoplastic small round cell tumor (DSRCT) is an extremely rare, highly aggressive, and malignant neoplasm initially reported by Gerald and Rosai in 1989 . Moreove2, day 1), ifosfamide (3 g/m2/day × 3), and doxorubicin (30 mg/m2/day × 2) or the IVC scheme  for four to ten cycles. Response to chemotherapy based on the degree of tumor volume reduction was defined as follows: complete response (CR), partial response (PR), stable disease (SD), and progressive disease (PD). Survival outcomes were estimated using the Kaplan-Meier method and compared between groups by the use of log-rank test. P value <0.05 was considered to indicate statistical significance; all statistical tests were carried out utilizing SPSS, version 17.Institutional review board approval was obtained for this retrospective cohort study. It was performed on 12 cases of DSRCT between March 2003 and May 2011. The clinical, pathological, and radiological imaging studies were available for review. In our series, the median age was 26.4 ± 8.4 years with a range of 14–39 years and the male-to-female ratio was 2 : 1. All 12 patients were evaluable for radiological characteristics and histological and immunochemical data. All cases underwent abdominal and pelvic CT examination with contrast-enhanced CT scan. According to their treatment, two subgroups were defined: group 1 ; all were treated with multiple agents chemotherapy following four to ten courses of multiple agents chemotherapy until tumor progression; group 2  underwent exploratory laparotomy, followed by en bloc resection in two cases and debulking or cytoreductive surgery in four cases, because the lesions were found as multiple nodes in different sizes and distributed in multiple organs including peritoneum, mesentery, and pelvic cavity. Debulking surgery in our investigation is defined as definitive removal of at least 90% of the tumor burden. Because of multiple lesions or diffuse nature of tumors infiltrating adjacent vital organs, it is often impossible to complete resection of all tumors with negative microscopic margins. Subsequently, all cases were treated with adjuvant chemotherapy. In both groups, the chemotherapy scheme was based on the intensive use of vincristine (1.5 mg/mn = 8), palpable abdominal mass (n = 6), and urinary disorders (n = 2) and no positive findings (n = 1). Serum CA125 was examined in three cases but only one increased significantly about 131 U/ML whose normal value is 1.9~16.3 U/ML. General information of all patients and follow-up data are listed in In our series, the most common primary manifestations were gastrointestinal symptoms including nausea, vomiting or distention  in abdominal and pelvic space  in abdominal-pelvic space (n = 7), located in the omentum and paravesical region (n = 5). CT also showed serosal tumor implants from intraperitoneal spread (n = 3). Two patients had hydronephrosis ; one patient has frequency and dysuria. Areas of central low attenuation within tumors were seen in 4 patients. Scattered amorphous or punctuate tumor calcification was seen in three patients (25%). On contrast enhancement CT scan, it was modest enhancement (n = 4), obvious enhancement (n = 5), without enhancement (n = 3).All patients underwent abdominal and pelvic CT examination: three cases complicated with hydronephrosis, two cases had uterine accessories infringement, and three cases presented with liver or lung metastases. According to striking CT features, the abdominal and pelvic lesions were divided into two groups. The most common imaging finding was multiple nodular peritoneal soft-tissue masses with variable sizes   .The rarity of DSRCT may attribute to the less knowledge of its biological behavior; meanwhile, the pathogenesis of DSRCT is unclear. Histologically, the majority of DSRCTs are distinguished by solid clusters of undifferentiated small round cells embedded in dense desmoplastic stroma –13. ThesDSRCT mainly affects young adolescents with a male-to-female ratio of 4 : 1 and tends to be symptomatic on presentation. In our series, the average age is relatively older than the literature reported. The tumor has a predilection for the omentum and adheres to the hollow viscus, surface of the omentum, mesentery of bowel, or pelvis peritoneum. Digestive organs involvement is secondary; liver and lung are two common sites for metastatic disease beyond the peritoneum. Extra-abdominal primary DSRCTs are relatively uncommon. Clinically, patients may be asymptomatic for long periods of time and diagnosis is made when tumor burden is large. Some abdominal DSRCTs present with persistent, nonspecific abdominal symptoms such as discomfort or distention, constipation or bowel obstruction, nausea or emesis, weight loss, ascites, increasing abdominal girth, and palpable masses and infiltration of urinary organs such as hydronephrosis or urinary disorders. Occasionally, incidental palpable abdominal masses may be the first presentation. Clinical presentation may be related to tumor size, distant metastasis, and infiltration of the surrounding structures. In our series, the median tumor size was 8.4 ± 5.3 cm. Although it can develop at various sites, most cases usually present with widespread abdominal serosal involvement whose growth pattern closely mimics that of mesothelioma; it is speculated that the cell origin of DSRCT may be a primitive mesothelial cell. Typical cases of DSRCT in the intra-abdominal cavity or gastrointestinal tract are accompanied by abdominal mass and/or pain which are similar to gastrointestinal tumors. Ascites and solitary or multiple nodules can also present in some patients. Occasionally, urinary tract symptoms were caused by ureter or bladder involvement, whereas DSRCT originating from the genitourinary tract system was rare. Serum CA-125, which is reported to be increasing significantly among DSRCT patients, may be used as a marker but lack specificity . Only onCT scan is the most widely used diagnostic modality; abdominopelvic site was the commonest presentation and the disease can occur at other nonserosal surfaces also. CT frequently shows multiple bulky, lobulated, heterogeneous, and peritoneal soft-tissue masses with a predilection for intraperitoneal spread without obvious primary organ involvement. In advanced cases, abdominopelvic DSRCT can develop into bulky and multiple masses that displace the neighboring organs. These radiographic findings must be distinguished from peritoneal carcinomatosis. The hallmark imaging feature was from dozens to hundreds of multiple millimeter sized nodules to lobulated or confluent peritoneal masses which lack characteristic features, and preoperative imaging had a low diagnostic utility. Bellah et al. analyzed CT characteristics of 11 patients with DSRCT and found that most characteristic CT features of DSRCT include bulky intra-abdominal soft-tissue masses that involve omental and serosal surfaces, without a distinct organ of origin, and widespread implant of the tumor was also found . In the Proper consensus about treatment has not yet been established, and the treatment of DSRCT remains a clinical challenge and lacks standard treatment modalities. Despite multiple treatment strategies including high-dose chemotherapy regimens active for DSRCT, aggressive debulking surgery, whole abdominal radiation, or even autologous stem cell transplant, the prognosis of DSRCT is poor and most cases die within 3 years .Owing to frequent multiple peritoneal implants or multifocal lesions, complete resection without sacrificing the adjacent organs is usually impossible. The effect of complete resection of disseminated tumors on survival is still unknown because of the rarity of achieving complete resection at operation.For advanced disease, symptom palliation is paramount as these modalities impact survival minimally. Aggressiveness of DSRCT may add a surgical burden and the impact of surgical resection upon survival remains unclear; in our series, patients who underwent complete surgical excision seem to provide a better survival. Nevertheless, larger prospective studies are needed to provide it. Cytoreductive or debulking surgery has been performed before chemotherapy is used for symptomatic relief, especially for those intestinal obstruction cases. When peritoneum was involved, the patient should be performed peritonectomy and re-operation was needed occasionally. In our experience, surgical resection especially total radical resection or even debulking combined with multiagent adjuvant chemotherapy can highly improve overall survival of advanced DSRCT. Although it is difficult to demonstrate, increased survival depends only on combined modality therapy instead of other factors.Many chemotherapy combinations have been tried, but the optimal scheme and generally accepted chemotherapy option have not been determined at present. In previous investigation, DSRCT has been confirmed to be moderately sensitive to intensive chemotherapy; unfortunately, response duration was extremely poor . Some foRadiation treatment in DSRCT is controversial; radiotherapy especially whole abdominal-pelvic radiotherapy (WAPI) in DSRCT has not been used extensively owing to its acute toxicities and low response rate . HoweverInitial data confirmed that the combination with continuous hyperthermic peritoneal perfusion may be a rational approach to improve local control of abdominal DSRCT . RecentlManagement of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments. According to the current series, we would recommend aggressive debulking plus multiple agent chemotherapy for advanced abdominal DSRCT patients. The demerits of this investigation are the absence of randomized and large-scale trials. Our knowledge is based on a small series of patients in whom the outcomes are highly variable depending on the extent of the disease, resectability, and type of therapy.


Ameloblastic fibroma (AF) is a benign tumor of mixed odontogenic origin, which affects predominantly young individuals. AF appearing as a mixed radiographic image is very rare. This report describes a case of AF in a 12-year-old male identified during a routine radiographic exam for orthodontic treatment planning. The panoramic radiography revealed a well-defined multilocular mixed image located in the mandible between the roots of the left mandibular second premolar and first molar. The lesion was excised under local anesthesia. Histopathological analysis revealed islands of epithelial cells and columnar peripheral cells showing a nucleus in inverted polarization, interspersed with spindle-shaped cells and abundant extracellular matrix deposition. No atypia was observed. The diagnosis of AF was established. No tumor recurred up to 30 months after treatment. Although rare, AF should be also considered in the differential diagnosis of mixed radiographic images of the jaws in young patients. Key words:Ameloblastic fibroma, differential diagnosis, incidental finding, mixed image, radiographic features. Ameloblastic fibroma [AF] is a rare tumor, accounting for 2% of all odontogenic tumors, characterized by simultaneous neoplastic proliferation of mesenchymal and epithelial components, with no formation of hard dental tissues . The tumAF exhibits slow growth and most cases present with a painless swelling or are discovered during routine radiographic exams -5. DelayMicroscopically, the lesion shows cords and islands of neoplastic odontogenic epithelium interspersed by neoplastic mesenchymal tissue that resembles the dental papilla. Microscopic differential diagnoses include odontogenic myxoma and odontogenic fibroma . AlthougA 12-year-old male was referred for evaluation of a lesion in the left body of the mandible identified in a radiographic exam for orthodontic treatment planning. The medical history revealed no prior trauma or episodes of pain in the affected region. The extraoral examination revealed a painless slight swelling in the inferior portion of the face, corresponding to the region of the left mandibular second premolar and first molar. The lesion presented a firm consistency and no signs of inflammation were observed. On the intraoral examination, the same features were observed, with the discrete swelling showing a hard consistency and recovered by mucosa of normal appearance. There were no carious or periodontal lesions in the left mandibular teeth. However, a diastema was found between the left mandibular second premolar and first molar.The panoramic radiography revealed a well-defined multilocular mixed image, with sclerotic borders, located in the mandible, laterally and between the roots of the left mandibular second premolar and first molar, measuring approximately 1.5 cm in extension. Thin septa were observed within lesion. The radiopaque portion was restricted to the peripheral area of the lesion. Moreover, divergence of the roots was also observed (Fig. As the lesion was well circumscribed, under local anesthesia, the patient was submitted to excision of the lesion. Enucleation of the tumor was performed, followed by curettage of the surrounding bone. The surgical specimen was fixed in formalin and sent for histopathological analysis, which revealed an odontogenic tumor formed by islands of epithelial cells, with the columnar peripheral cells showing nucleus in inverted polarization. Numerous spindle-shaped cells and abundant extracellular matrix deposition were found between the islands of neoplastic cells Fig. . No atypAF predominantly affects young patients, with a mean age of 15 years -4. Howevet al. (Although few cases have been reported, the majority of studies indicate a slight predilection for the male gender -4. The pRadiographically, AF appears as a well-defined unilocular or multilocular radiolucent image with a mean size of 4.0 cm ,9. In thParticularly in this case, mixed radiographic lesions, such as ossifying fibroma, extra-follicular variant of adenomatoid odontogenic tumor, calcifying cystic odontogenic tumor and calcifying epithelial odontogenic tumor were also considered as hypotheses of diagnosis -14. HoweBecause of AF is often associated with non-erupted teeth, it may initially be interpreted as a dentigerous cyst ,2. BesidMicroscopically, AF is characterized by the proliferation of odontogenic epithelium immersed in a mixoid cellularized tissue similar to a dental papilla with different degrees of inductive changes and no formation of hard dental tissue ,5. The eWhile the malignant transformation of AF is rare, attention should be paid to the occurrence of mitosis, which is a finding to consider the possibility of an ameloblastic fibrosarcoma . MesenchThe appropriate treatment for AF remains matter of debate. A conservative approach, with excision followed by curettage, as performed in the present case, appears to be the most adequate treatment . HoweverIn summary, AF is a rare tumor and presents a good prognosis. However, patients must be followed up carefully after treatment and special care should be given to recurring tumors. Radiographic exams should always be requested to complement the periodic clinical exam because several mandibular lesions may present as an incidental radiographic finding. Although rare, AF should be considered in the differential diagnosis of mixed lesions of the jaws in patients during childhood and adolescence.


Benign multicystic peritoneal mesothelioma: a rare tumor of the abdomen, is a diagnostic dilemma. This report emphasizes the importance of diagnostic laparoscopy in the diagnosis of the tumour.  Benign multicystic peritoneal mesothelioma (BMPM), a rare tumor, occurs mainly in women in their reproductive age . The patA 40-year-old gentleman with no previous medical or surgical history presented with chief complains of vague abdominal pain in right lumbar and iliac fossa. Pain was nonradiating and there was no relation with food intake or with defecation. There was no history of fever or vomiting or similar pain in the past. On examination there was a diffuse nontender lump felt on the right side of the abdomen extending from the right hypochondrium to iliac fossa.Ultrasonography (USG)/CT scan guided needle puncture and aspirate and sending fluid for cytology.Diagnostic laparoscopy.Colonoscopy and biopsy.Ultrasonography of abdomen showed 14.5 × 7.5 × 15.9 cm multiseptated lesion with no definite solid component with septations showing minimal vascularity which was mainly venous. Lesion was intraperitoneal and anterior to caecum and terminal ileum. Surface of the liver showed no scalloping. Contrast enhanced computed tomography (CECT) abdomen and pelvis showed similar size irregular multiseptated multicystic lesion engulfing the ascending colon and caecum. Bowel lumen showed no growth . CEA andThis case was discussed with the senior surgical team and a differential diagnosis of appendicular abscess or mucinous cyst adenoma/adenocarcinoma of the appendix was made. Colonoscopic biopsy was not done as there was no growth in bowel wall on CECT. CT scan guided biopsy was not done in view of question of yield of the procedure and tracking of malignant cells in the path. In view of these two differentials and after ruling out other options, diagnostic laparoscopy was planned.Diagnostic laparoscopy revealed multicystic lesion over the whole of the ascending colon and caecum. As the cyst look very fragile, we converted to laparotomy and did a right hemicolectomy with side-to-side ileotransverse anastomosis without causing any rupture of the cyst. Patient had an uneventful postoperative period and was discharged in 8 days .On first follow-up after 1 month patient was well and ultrasonography of abdomen was normal. Histopathology on gross examination revealed a cystic mass of 21 × 13 × 8 cm attached to the serosa of the ceacum without any infiltration into the bowel wall. Cut section showed a multiloculated cyst, each cyst ranging from 0.5 cm to 2.5 cm filled with mucinous material. Lining was smooth and paper thin with few solid areas. Appendix and ascending colon were normal. Proximal and distal resection margins were uninvolved. Microscopy showed cuboidal epithelium lined tumor filled with eosinophilic proteinaceous material. Intervening septae showed fibrosis and dense lymphoplasmacytic infiltrate admixed with eosinophils and neutrophils. No infiltration was seen into the serosa of the caecum. No cytologic atypia or proliferation of the lining epithelium was seen Figures .Immunohistochemistry. Lining epithelium was positive for calretinin and CD 34 negative.BMPM was first described in 1979 by Menemeyer and Smith  our caseBMPM requires high index of suspicion for diagnosis, so the authors would recommend doing a diagnostic laparoscopy, for all tumors of suspicious etiology on imaging. Diagnosis can only be made on histopathology. Although its recurrence is high after surgical resection, it does not present a tendency to transform into malignancy. Finally, a prolonged systematic follow-up of these patients, perhaps for life, is required since the lesion usually reappears and further resection or another therapy may be indicated.


Central giant cell granuloma (CGCG) is a benign intraosseous lesion of the jaws that is found predominantly in children and young adults. Although benign, it may be locally aggressive, causing extensive bone destruction, tooth displacement and root resorption. The common therapy is aggressive curettage, peripheral ostectomy or resection, which may be associated with loss of teeth and in younger patient's loss of dental germs. In this article, a 6-year-old girl with CGCG is successfully treated surgically and this treatment is discussed along with review of the literature.How to cite this article: Patloth T, Reddy SJ. Surgically Treated Central Giant Cell Granuloma in Six-Year-Old Child: A Case Report. Int J Clin Pediatr Dent 2013;6(2):146-149. The anterior portion of the mandible has been identified as a more common location for CGCG development with the lesion frequency crossing the midline. Radiographically, the majority of CGCGs present as an expansile radiolucency, either unilocular or multilocular, which is generally traversed by bony spicules.Based on the clinical behavior and radiographic features, CGCG is classified as following;Aggressive lesion: They are found in young patient characterized by rapid growth, pain, expansion and/or perforation of cortical bone, induce root resorption and high recurrence rate.4Nonaggressive lesion: It is characterized by slow growth that does not perforate the cortical bone or induce root resorption and has a low recurrence rate.56depending on following factors: Aggressive vs nonaggressive behavior, location, size and radiographic appearance. Other treatments have included radiation and systemic injections of calcitonin and interferon.The traditional treatment of CGCG of the jaws has been surgical excision either by curettage or enbloc resection,The purpose of this case report is to highlight the surgical approach used for the treatment of CGCG affecting the maxilla in a 6-year-old girl.A 6-year-old female patient reported to the Department of Pedodontics and Preventive Dentistry, Government Dental College and Hospital, Hyderabad, with a complaint of swelling in the right side of the upper jaw. It was first noticed 6 months before the initial consultation. It was smaller in size and then suddenly increased to present size within 10 days. There was no history of fever, pain, sensory disturbance, bad taste or traumatic injury except discomfort and difficulty in mastication due to large swelling.Extraoral examination revealed an enormous swelling on the right side of the face involving the right maxilla causing obliteration of nasolabial fold and crossing the midline resulting in facial asymmetry . The sweIntraoral examination revealed diffuse swelling on the right side of maxilla obliterating the right buccal vestibule and crossing the midline extending up to deciduous lateral incisor of contralateral side. No teeth were visible intraorally on the effected side presumably due to pathological resorption caused by the lesion leading to loss of teeth.The serial axial and coronal sections of computed tomography revealed lytic lesion causing destruction of cortex in right maxilla along with overlying large soft tissue component which was benign in nature .Laboratory values for serum calcium, phosphorous, alkaline phosphatase and parathyroid hormone were within normal limits.Incisional biopsy obtained showed highly cellular tumor with nodular surface folds. The ulcer base revealed granulation tissue and dense chronic inflammatory infiltrate. The cellular lesion consisted of polygonal to spindle cells arranged in sheets and bundles admixed uniformly with many osteoclastic giant cells. The giant cells showed moderate amount of fibrillary pale vacuolated cytoplasm and oval to plump elongated vesicular nuclei. Mild to moderate nuclear pleomorphism and small nucleoli with increased mitotic activity in focal areas was suggestive of CGCG .Surgical approach was preferred because of the size of the lesion . Partialin toto with the associated tissues and was removed completely (After reflection of the soft tissue a fissure bur was used to cut the cortical bone around the lesion approximately 0.5 cm from its margin. The lesion was reflected mpletely . Suturesmpletely . There ampletely  followin7The CGCG may occur at any age but it is most commonly seen in the first 3 decades. The anterior part of the mandible has been identified as the most common location for CGCGs, with some crossing the midline. CGCGs have been thought to arise in regions of the jaw originally housing deciduous teeth.8 considered that these lesions should be defined as aggressive giant cell granuloma of the jaws.CGCG of the jaw is usually unifocal. Multifocal lesions should alert the clinician to the possibility of hyperthyroidism or if bilateral cherubism. CGCG should also be distinguished from giant cells tumor of long bones. The later is locally aggressive with a high recurrence rate and a potential for malignant transformation. Some CGCGs of the jaws, despite an innocent histologic appearance, show an aggressive behavior and a tendency to recur. Ficarra et al9Aggressive CGCG has a tendency to recur if inadequately removed and high recurrence rates have been reported. It has been shown that recurrence usually happens when the lesion perforates the cortical plates to involve the surrounding soft tissue.4advocated the technique of curettage or curettage plus peripheral ostectomy for the treatment of CGCG. Chuong et al5 suggested that aggressive tumors that present with pain, rapid growth and facial swelling or cortical perforation be treated with enbloc resection.CGCGs have traditionally been treated surgically. The common therapy is curettage or resection. Eisenbud et alIn an attempt to distinguish aggressive and nonaggressive subtypes of CGCG and to predict the prognosis of newly diagnosed CGCGs, numerous studies have been conducted using cytometric and immuno-cytochemical methods. It has been shown that aggressive types have a higher number and relative size index of giant cells and a greater fractional surface area occupied by giant cells.10by administration of intralesional corticosteroids. Other nonsurgical treatments include calcitonin treatment and use of alpha interferences. However, in this case nonsurgical approach was not recommended due to aggressive behavior, location, size of lesion and rapid expansile destructive growth hence surgical resection, i.e. partial maxillectomy was preformed.Surgical treatment of CGCGs can be associated with recurrence and serious facial mutilation and loss of teeth and tooth germs are also unavoidable. For these reasons a nonsurgical approach was suggested by Jacoway et alThe CGCG is usually asymptomatic and is most often discovered on a routine radiographic examination and its diagnosis is confirmed by histopathological examination. The lesion can be accompanied by pain, resorption or root displacement. This aggressive lesion is usually found in young children. Our case report along with the review of literature highlights an unusual presentation that it occured in maxilla which is an uncommon site. Due to its extreme aggressiveness, among the various treatment modalities the surgical approach became essential and hence partial maxillectomy was performed.


Synchronous spinal intramedullary ependymal cysts and intramedullary schwannomas are rare. To the best of our knowledge, the present study is the first report of a case of intramedullary schwannoma coexisting with an ependymal cyst. A 35-year-old male presented with lower back pain and weakness in the left leg. Magnetic resonance imaging identified an intramedullary cystic-solid lesion at the thoracolumbar junction of T11-L2; based on the clinical presentation and radiological features, a pre-operative diagnosis of ependymoma was formed. Subsequently, the patient underwent a T11-12 laminectomy via a posterior approach, with intraoperative monitoring of somatosensory and motor-evoked potentials, achieving a gross total resection of the tumor with a well-demarcated dissection plane. Post-operative histopathological examination demonstrated a schwannoma coexisting with the ependymal cyst, and the neurological status of the patient markedly improved compared with the pre-operatively observed neurological deficit. Spinal schwannomas are usually intradural extramedullary tumors. As Schwann cells are not typically found in the parenchyma of the central nervous system (CNS), these tumors are rarely observed within the spinal cord . IntrameOn March 4, 2013, a 35-year-old male was admitted to Beijing Tiantan Hospital  with a two-year history of lower back pain and weakness in the left leg. In addition, the patient had experienced sphincter dysfunction for two months. The patient had no history of spinal cord injury or previous back surgery. Neurological examination revealed the power in the left leg to be 3/5, as classified by the Medical Research Council grading system . SuperfiThe patient initially underwent pre-operative magnetic resonance imaging MRI; . SagittaThe patient underwent a T11-12 laminectomy via the posterior approach with intraoperative monitoring of somatosensory and motor-evoked potentials. Intradural exploration revealed a bulging conus medullaris. Subsequently, classic median myelotomy was performed in the conus medullaris for intramedullary exploration. The solid mass was nodular in shape, with a poor blood supply. The mass was well demarcated from the spinal cord parenchyma and not attached by any nerve root, which facilitated its exposure and dissection. Following GTR of the solid mass, the cranial syringomyelia was examined. A cyst filled with clear fluid was identified; the cyst was in close proximity to the solid mass, however, it was separated by neural tissue. The cyst appeared yellow in color, was filled with clear fluid and did not adhere to the spinal cord, therefore, a GTR of the cyst was performed.Pathological examination of the solid mass identified spindle-shaped cells with features of a schwannoma . SurgicaIn the immediate post-operative period, although the weakness in the left leg and the sphincter dysfunction did not improve, the lower back pain disappeared. The patient was discharged two weeks later; the weakness in the left leg had improved to grade 5/5 and sphincter dysfunction gradually improved after three months. No recurrence of the schwannoma or the cyst were observed on the follow-up MRI . The preSchwannomas and ependymal cysts represent two distinct disease entities. To the best of our knowledge, intramedullary ependymal cysts have not previously been reported in association with schwannomas. Intramedullary schwannomas are predominantly located in the cervical region, followed by the thoracic region and the lumbar cord ,12; the et al  based solely on MRI is not viable pre-operatively. In the present study, the solid mass did not demonstrate the significant enhancement observed in hemangioblastoma and it presented with cranial syringomyelia or cystic degeneration, therefore, a pre-operative diagnosis of ependymoma was considered. Certain studies have diagnosed cranial or caudal cystic degeneration as simply non-neoplastic peritumoral syringomyelia ,27. EnteIn conclusion, the present study reports a rare condition in which the association between the two different congenital neoplasms may indicate the presence of dysembryogenesis as the causative mechanism. Owing to the rarity of concurrent intramedullary schwannoma and ependymal cysts, as well as a lack of clinical and imaging characteristics, intramedullary schwannoma is easily misdiagnosed as ependymoma. Therefore, tumor excision with exploration of the syringomyelia adjacent to the tumor is the recommended surgical strategy.


Splenic artery aneurysm rupture is rare and potentially fatal. It has largely been reported in pregnant patients and typically not diagnosed until laparotomy. This case reports a constellation of clinical and sonographic findings that may lead clinicians to rapidly diagnose ruptured splenic artery aneurysm at the bedside. We also propose a rapid, but systematic sonographic approach to patients with atraumatic hemoperitoneum causing shock. It is yet another demonstration of the utility of bedside ultrasound in critically ill patients, specifically with undifferentiated shock. Ruptured splenic artery aneurysm (SAA) is a rare condition that is challenging to diagnose given the nonspecific presentation. Non-specific abdominal pain is common in the emergency department (ED) representing 4–5% of complaints.A 41-year-old woman presented to the ED with sharp, stabbing chest pain radiating into the abdomen and the back with nausea and diaphoresis. She reported diffuse abdominal pain for several months, and admitted to only occasional alcohol use. Cholecystectomy was her only surgical history.Initial vital signs were BP 82/60 and pulse 110. Physical examination showed a diffusely tender abdomen with increased pain in the left upper quadrant and epigastric regions. Vital signs improved initially with an intravenous (IV) fluid bolus.The initial differential included upper gastrointestinal bleeding, sepsis, myocardial infarction, aortic emergencies, pregnancy complications including ectopic, and perforated viscus.Chest radiograph and electrocardiogram were normal. Despite initial stabilization, the patient again became hypotensive with signs of profound shock including an ashen appearance, decreased mental status, and weak, thready pulses.A bedside ultrasound was performed to evaluate the patient’s physiology and potential etiology of shock. The cardiac views were limited but showed no effusion or obvious right ventricular dilation, and left ventricular function appeared vigorous , Frame 1At this point the differential was modified and included spontaneous splenic rupture, but from previous clinician experience, this was felt less likely due to the normal appearance of the spleen on ultrasound. Hemorrhagic pancreatitis was considered, but the extent of intraperitoneal hemorrhage and clinical presentation did not appear consistent. Ruptured ectopic pregnancy and hemorrhagic ovarian cyst were also felt unlikely given the lack of adnexal mass and negative HCG. SAA was felt the most likely diagnosis given the overall clinical and sonographic findings, specifically diffuse atraumatic hemoperitoneum, the localized clot formation in the epigastrium and left upper quadrant and lack of findings to support other differential considerations.Adequate IV access was assured and resuscitation with blood was initiated while the patient was taken immediately to radiology for computed tomography (CT) angiography, which showed multiple SAAs and ongoing hemorrhage. Interventional radiology and surgery were consulted. The patient was taken to a dual angiography/operating room suite where splenic artery embolization was performed, followed by open evacuation of hematoma, splenectomy, distal pancreatectomy and further hemorrhage control. Resuscitation followed a massive transfusion protocol, resulting in total administration of seven units of packed red blood cells, four units of fresh frozen plasma, one unit each of platelets and cryoprecipitate, in addition to autotransfusion during surgery. She did well postoperatively.Ruptured SAAs are an uncommon cause of hemorrhagic shock but the splenic artery accounts for 60% of visceral aneurysms.After rupture, SAAs cause significant blood loss with hemodynamic instability typically occurring in 6–96 hours, giving time for repair if diagnosed. The mortality ranges from 10–36% in non-pregnant patientsInitial presentation of rupture is chest pain followed by hemodynamic instability 6–96 hours later. The delayed blood loss is caused by the “double rupture phenomenon,” where blood is initially contained within the lesser omental sac, delaying the onset of intraperitoneal hemorrhage.Ruptured SAA is most frequently reported in pregnancy. Only a few reported cases described the use of bedside ultrasound to identify hemoperitoneum prior to open laparotomy. Jackson et al.The diagnosis of SAA primarily occurs when a CT with contrast is ordered as part of the work up of abdominal pain or during exploratory surgery for non-traumatic hemoperitoneum.Etiologies of non-traumatic hemoperitoneum with hemodynamic instability include ruptured vascular neoplasm in a solid organ, spontaneous splenic rupture, ruptured ectopic pregnancy, uterine rupture during pregnancy, uterine artery rupture, or intraperitoneal abdominal aortic aneurysm rupture. A ruptured hemorrhagic ovarian cyst may cause hemoperitoneum, but hypotension is atypical.8When using ultrasound to assess cases of non-traumatic shock with hemoperitoneum, a careful consideration of the differential diagnosis with a rapid but systematic sonographic evaluation may suggest the most likely etiology. In this case, the absence of clot or fluid around the spleen implied spontaneous spleen rupture was unlikely. This belief was based mostly on clinician experience, but there have also been reports of spontaneous spleen rupture that report splenomegaly, perisplenic hematoma and/or fluid collections as common sonographic findings.We report a patient who presented with nonspecific complaints and undifferentiated hypotension where bedside ultrasound assisted in drastically altering the differential. Identifying the rare diagnosis of ruptured splenic artery aneurysm early led to rapid intervention and a more favorable outcome for the patient. This case further illustrates the utility of bedside ultrasound in the evaluation of critically ill patients, specifically in undifferentiated shock. We suggest a rapid but systematic sonographic evaluation to assist in determining the etiology of nontraumatic hemoperitoneum causing shock. The absence of sonographic signs of other etiologies combined with the finding of extensive clot formation in the epigastrium and left upper quadrant may suggest ruptured splenic artery aneurysm earlier in the patient’s course, expediting diagnosis and management, and potentially improving outcome.


Oral melanoacanthoma is rare pigmented mucosal lesion that presents most commonly on the buccal mucosa, characterized by sudden appearance and rapid radial growth, thus clinically mimicking malignant melanoma. It was originally described as a mixed tumor of melanocytes and keratinocytes, but appears to be a reactive process; formed in areas prone to trauma, and regressing after the removal of trauma or incomplete excision. The clinical appearance of oral melanoacanthoma is nondiagnostic, and biopsy is mandatory to rule out malignancy. We report a case of melanoacanthoma of a rarer oral mucosal site in a 12-year-old Asian male. A brief review of the current literature is also presented.How to cite this article: Rohilla K, Ramesh V, Balamurali PD, Singh N. Oral Melanoacanthoma of a Rare Intraoral Site: Case Report and Review of Literature. Int J Clin Pediatr Dent 2013;6(1):40-43. Most patients are adults, beyond 40 years of age. Sex predominance is not known.11 Involvement of labial mucosa14 and alveolar ridge15 has also been reported. Mostly unilateral and solitary,16 these deeply pigmented lesions may have a flat or slightly raised surface. The other end of the spectrum of clinical presentation includes lesions that may be bilateral,17 and even multifocal,181819 as well as those which even have a proliferative or warty surface. These intraoral hypermelanotic macules or papules are typically brown, black or blue-black in color, with possible variation in the intensity of pigmentation.131820On the contrary, intraoral melanoacanthomas tend to affect a much younger population, occurring almost exclusively in blacks, with a female predilection. These lesions show rapid increase in size and may attain dimensions of several centimeters in a few weeks. Buccal mucosa is the most frequently reported intraoral site, although masticatory mucosa subject to chronic trauma  may also be affected.24 Some of the previously reported cases have been summarized in Intraoral melanoacanthoma still continues to be a rare entity.A 12-year-old male patient presented for evaluation of a lesion in the left maxillary gingiva, which was present for the past 6 months. The patient was under medication with valproic acid for the treatment of petit mal seizures, till the age of 8 years, after which it was discontinued. Otherwise, the medical history was noncontributory.Extraoral examination revealed no clinically significant findings. Intraorally, there was a soft tissue growth in maxillary left quadrant , involviThe lesion was excised and sent for histopathological examination. Hematoxylin and eosin (H&E) stained sections revealed surface stratified squamous epithelium and underlying fibrous connective tissue. The epithelium exhibited parakeratosis and acanthosis and the rete ridges were irregular in shape. There was a prominence of melanocytes in the basal layer, in a linear fashion . There wThe patient has been on a regular follow-up , and the14 Since then, there has been an addition of more than 65 cases to the available literature. The pathogenesis still remains obscure, though some authors have ascribed the potential role of chronic trauma in these cases.The credit for the first fully documented case of oral melanoacanthoma goes to Matsouka (1979).15 or elimination of local irritants.18 The histologic picture of subepithelial inflammatory cell infiltrate and slightly increased vascularity further add evidence to this concept. To differentiate these lesions from cutaneous melanoacanthoma and to emphasize their reactive nature, several terms have been suggested, including melanoacanthosis, reactive melanocytic hyperplasia and mucosal melanotic macule.6821The intraoral melanoacanthoma is essentially reactive in nature'a fact supported by the clinical course of the lesion. It is characterized by a tendency to affect the mucosal sites that are exposed to trauma, and typically shows rapid growth and observable regression of the lesion'spontaneously or following incomplete removal2 The observation of any of these features mandates a biopsy, because of potential resemblance to a malignant melanoma. The alarming growth rate of oral melanoacanthoma makes it clinically indistinguishable from oral malignant melanoma, especially the radial growth phase of an in situ melanoma. The biopsy, hence, should be performed invariably to rule out the possibility of a melanoma. Once the diagnosis has been established, no further treatment is indicated, and many cases document spontaneous regression.The clinical picture of this lesion is indistinguishable from many other oral pigmented lesions. All pigmented lesions should be observed for evolution with respect to size, shape, color, surface or symptom overtime.10 The immunohistochemical profile of these lesions is limited to the melanocytic markers, but is not necessary for diagnosis, as strong reactivity to HMB-45 and S100 is seen in both oral melanoacanthoma and malignant melanoma.10The diagnosis of oral melanoacanthoma can be made solely on the basis of histological features and special staining. In order to emphasize the presence of melanin and to demonstrate the dendritic melanocytes, Masson-Fontana silver impregnation technique can be used.68 opine that in contrast to the other pigmented lesions, the melanin in oral melanoacanthoma is restricted mainly to melanocytes, the adjacent keratinocytes being devoid of melanin. Interestingly, in our case, the histopathological picture showed the presence of ‘dusty’ melanin in the basal as well as parabasal keratinocytes.Some authorsMelanoacanthoma is a reparative lesion with no malignant potential. The treatment should be directed toward removing all local causes of trauma and excluding any other causes of oral pigmentation, particularly malignant melanoma.The authors advocate the replacement of the misnomer ‘melanoacanthoma’ with a more appropriate description ‘melanoacanthosis', a term which gives due credit to the clinical behavior and histopathological picture of this rare and interesting lesion.We present a case of a rare entity, oral melanoacanthoma, occurring at a rarer oral mucosal site, that is, gingiva, in a 12-year-old Asian male. The diagnosis was based mainly on the histologic findings with H&E and Masson-Fontana stains. The patient is on a regular follow-up and the lesion has regressed completely after the initial surgery.


Fibrolipoma, a subtype of lipoma is painless, well-circumscribed, slow-growing, submucosal benign adipocyte tumour. It is uncommon in the oral cavity and oropharyngeal region, with rare incidence in the retropharynx even rarest in pediatric age group.A very unusual case of fibrolipoma is presented in a pediatric patient, who had a huge retropharyngeal fibrolipoma and who presented with breathing difficulty and increasing stridor. It was managed by intro-oral approach excision.Although rare, retropharyngeal benign tumours should be kept in mind during the differential diagnosis of a paediatric stridor case. Early diagnosis is the key for a better outcome and to alleviate the worsening morbidity. Lipomas are common benign soft tissue mesenchymal tumours involving tissues containing lipocytes. These are commonly found in middle-aged population with no gender predilection. The oral cavity is an uncommon site of presentation comprising 1-4% of cases only -15 Tabl. Simple lipoma was the most common variant among them. Here, a rare case of huge retropharyngeal fibrolipoma is reported. Most likely, this is the first reported case in a very young patient (2years of age), who showed symptoms of obstructive sleep apnea and stridor.A 2-year-old male baby was referred to our otolaryngology and head & neck surgery outpatient department with a progressively worsening difficulty in swallowing and noisy breathing for the last 3 months. The patient had no history of any fever, recurrent cough or cold, vomiting, seizure, or nasal regurgitation of food particles. He had no history of any foreign body ingestion or any contact history of tuberculosis. He was examined clinically and found to have a smooth mucosa covering a retropharyngeal bulge, which was compromising the oropharyngeal inlet and abutting both tonsils. Palatal movements and bilateral tonsils were normal. The patient showed symptoms of stridor and oxygen saturation was near 84% in room air. The patient was admitted in the paediatric intensive care unit and kept under close watch to monitor any vital deterioration. Fibre-optic laryngoscopy was performed with precaution and results were suggestive of a large retropharyngeal bulge with pooling of saliva in both pyriform sinuses. The endolarynx could not be examined properly. The patient underwent radiological evaluation with X-ray soft tissue neck , which showed increased prevertebral soft tissue shadow with predominant fat lucency from C1 to C7 vertebrae, displacing the larynx and trachea anteriorly without any significant airway narrowing. No calcification or air fluid level was observed . Contrast enhanced computed tomography from the skull base to the diaphragm showed a 3.98 x 4.7 x 7.0 cm well-defined, fat attenuating mass with enhancing septa within, extending from C1 to D2 level in the retropharyngeal region without any calcification or cystic spaces. The mass was displacing the airway anteriorly and bilaterally and displacing the carotids laterally. Fat planes with prevertebral muscles and underlying vertebrae were normal . A proviUnder general anesthesia, direct laryngoscopy guided oral intubation was performed. A longitudinal mucosal incision was made in the retropharynx. A well-circumscribed, capsulated, firm tumor was delineated and separated from surrounding tissue by blunt dissection and was delivered in toto intra-orally. Primary wound closure was done after achieving haemostasis. The excised specimen was sent for histopathological examination after fixing with formalin. Intra-operative and immediate post-operative periods were uneventful and the patient was kept on Ryle’s tube feeding post-operatively for 2 weeks. Histopathological examination showed a pale, firm, smooth, capsulated soft tissue tumor of a 8.0 X 5.0 cm size . MicroscLipomas are the most common mature adipocyte soft tissue tumours, mostly affecting the trunk, shoulder, neck, and axilla  with a dThe retropharyngeal space is a potential space with bucopharyngeal fascia anteriorly and alar fascia posteriorly, separating it from the dangear space. It extends from the skull base to T4 vertebra caudally. It contains fatty tissue and lymph nodes and serves as a potential space for spread of infections and malignancy. Retropharyngeal lipomas usually present in later stages with progressive difficulty in swallowing, breathing difficulty, and change in voice. Diagnosis is made by clinical and radiological investigations and the final diagnosis depends upon the histopatho- logical examination after complete surgical excision. Furthermore, fibrolipomas have a greater proliferative activity in comparison to other variants of lipomas, thus accurate diagnosis is essential . RetrophFibrolipomas are well-circumscribed, slow growing soft tissue adipocyte tumours. Clinically these are asymptomatic unless they increase in size. Retropharyngeal fibrolipomas should be differentiated from other neoplastic or infective pathologies. Complete surgical excision is the choice treatment modality. A well-experienced surgeon needs a well-equipped setup and trained anesthetists for better airway maintenance during intraoral excision.