To present the most important characteristics of inflammatory myofibroblastic tumours (IMTs) arising in different locations of the body with histological correlation.To review the symptoms and main radiological findings of IMTs. On ultrasonography (US), these tumours can appear as hypoechoic or hyperechoic masses and a variable Doppler appearance with increased vascularity. Computed tomography (CT) and magnetic resonance (MR) are the most used imaging tools in their evaluation. On contrast-enhanced CT, IMTs can appear as homogeneous or heterogeneous lesions, with variable enhancement on delayed acquisitions due to fibrosis. These findings are also present on gadolinium contrast-enhanced MR. On T1-weighted and T2-weighted sequences, IMTs usually show low signal intensity reflecting also the presence of fibrotic tissue.To show the main clinical symptoms and radiological features of IMTs in different locations: head and neck, lung, genitourinary, hepatic, splenic, gastrointestinal tract, mesenteric, muskuloskeletal.Although IMTs in some organs are not uncommon, they are not usually included in the differential diagnosis of masses. Their radiological features suggest malignant neoplasms, whereas they are not. Consequently, this is an underdiagnosed entity and only after an histological exam could a definitive diagnosis be achieved.Their radiological features suggest malignant neoplasms, whereas they are not• CT and MR imaging are the most used tools in their evaluation• IMT is an underdiagnosed entity• The definitive diagnosis is only after histological exam•  Inflammatory myofibroblastic tumours (IMTs) constitute a rare group of neoplasms composed of a mixture of spindle-shaped myofibroblasts or fibroblasts and a variable amount of inflammatory cells  . Many diDifferent aetiologies have been proposed for IMT , being dClinical presentation of IMTs depends on the organ in which they arise, but they frequently associate general inflammatory symptoms as fever or malaise.Radiological appearance of IMTs is unspecific and they are often misdiagnosed as malignant neoplasms. Many of them are incidentally discovered when an imaging technique  is performed for any other reason. Their most common radiological presentation is as solid, irregular, well-defined masses.Histological studies are critical to properly diagnose them. Immunohistochemical studies of T- and B-cell subpopulations, CD34, CD117, S-100 and c-Kit may be helpful in distinguishing IMTs from other soft-tissue neoplasms , 9. At tAccording to an update based on the new World Health Organisation (WHO) classification, IMTs are considered as neoplasms which may recur or metastasise in as many as 5 % of cases .The objective of this pictorial review is to present the most important characteristics of IMTs arising in different locations of the body with their histological correlation.The radiological findings of IMTs are non-specific. On US, these tumours can appear as hypoechoic or hyperechoic masses with ill-defined or well-circumscribed edges and a variable Doppler appearance with increased vascularity. US is usually the first imaging technique when IMTs appear in specific locations, such as testicles or neck. In other locations its role is limited except for the guidance of percutaneous biopsies.CT and MRI are the most used imaging tools in the evaluation of IMTs. On contrast-enhanced CT, IMTs can appear as homogeneous or heterogeneous lesions, with variable enhancement on delayed acquisitions due to the presence of fibrosis. These findings are also present on gadolinium contrast-enhanced MRI. On T1-weighted and T2-weighted sequences, IMTs usually show low signal intensity reflecting also the presence of fibrotic tissue   Fig. 7)7). On MRSurgery is the most accepted treatment. If there is a biopsy-proven diagnosis of IMTs that excludes malignancy, medical treatment with no steroidal anti-inflammatory drugs in patients with peripheral hepatic IMTs could be prescribed .Splenic IMTs are extremely rare. As in hepatic IMTs, Epstein-Barr virus has been proposed as a possible aetiological agent.Patients with spleen IMTs frequently associate unspecific symptoms as left upper quadrant or epigastric pain. Splenomegaly is common and some patients can present fever, anaemia and signs of hypersplenism .Different radiological imaging modalities have been proposed for their evaluation. On all of them, the most common radiological finding is a well-delimited mass. On US, they look like echogenic or hypoechoic masses . On CT, The differential diagnosis includes splenic haemangioma and other primary splenic neoplasms such as lymphoma or splenic angiosarcoma .When a suspicious spleen mass is found, the treatment of choice is splenectomy due to the high risk of bleeding associated to splenic biopsy , 31. TheIMTs of the gastrointestinal tract are infrequent. They can arise anywhere, the stomach and small intestine being the most common locations , 9.Clinical symptoms may vary from none to abdominal pain, intestinal obstruction, dysphagia or anaemia, depending on the location and size of the mass. They also may present fever and malaise.Contrast-enhanced CT is the imaging technique, which give us more information about of IMTs of the gastrointestinal tract. They are usually found as hypodense, heterogeneous and well-delimited masses Figs.  and 12.FThe differential diagnosis of these tumours includes GIST, inflammatory fibroid polyp, smooth muscle neoplasm, peripheral nerve sheath tumour, solitary fibrous tumour, fibromatosis, the follicular dendritic cell sarcoma, lymphoma and adenocarcinomas . In mostSurgery is the most accepted treatment. IMTs of the gastrointestinal tract present a higher recurrence rate than in other locations of the body . Other tIMTs of the appendix are extremely rare, with few cases reported in the literature , 33, 34.Their radiological appearance is similar to the IMTs of other gastrointestinal locations. Histological confirmation is needed to achieve the diagnosis.They usually have a benign clinical course with complete resolution after appendicectomy. Extremely rare cases of IMTs of the appendix showing local aggressiveness and metastases have been reported  Fig. 1212.Mesentery is one of the most common locations of extrapulmonary IMTs. They are more frequent in children and young adults .Many different aetiological factors have been proposed for mesenteric IMTs , 38, sucManagement of mesenteric IMTs is variable. Imaging, usually CT. Rapidly growing tumours or symptomatic ones needing treatment may follow from small tumours which are not encroaching any nearby structures. Different treatments have been proposed: non-steroidal anti-inflammatories, anti-oestrogens, chemotherapeutic agents and surgery. Surgery remains as a useful modality although quite a high local recurrence rate has been described for tumours , 37, 38.Few cases have been described of IMTs arising from the muscles . ClinicaAlthough IMTs in some organs are not uncommon, they are not usually included in the differential diagnosis of nodules and masses. Their radiological features suggest malignant neoplasms, whereas they are not.Consequently, this is an underdiagnosed entity and only after an histological exam could definitive diagnosis be achieved.It is fundamental for radiologists to know about the existence of IMTs, as they frequently have better treatment options and prognosis than the malignant neoplasms they are confused with.


Introduction. External auditory canal polyps usually reflect an inflammatory process. Rarely, they may reflect a serious condition that warrants urgent intervention. Case Report. A 19-year-old deaf mute female presented to our department with persistent left ear discharge and a reddish mass in the ear. After surgery, the cause was identified as a neglected foreign body. Tympanic membrane was intact. Conclusion. Aural polyp that is resistant to medical treatment should raise the suspicion of an inflammatory polyp with underlying chronic suppurative otitis media or foreign body. Rarer neoplastic and immunological causes should also be considered. Aural polyp or otic polyp is a proliferation of granulation tissue with chronic inflammatory cells in response to a long standing inflammatory process , 2. It iThe most common symptoms of an aural polyp are otorhhoea, diminished hearing, and a visible mass in the ear. Otalgia and bleeding or sensation of mass are far less common .Grossly, aural polyp is usually solitary, polypoidal with reddish surface, and often friable .The commonest causes of aural masses are inflammation, cholesteatoma, abscess, benign tumors such as osteomas, and malignant tumors like rhabdomyosarcoma and squamous cell carcinoma .Patients with foreign body might present with a history of foreign body insertion, pain, hearing loss, or otorrhoea or as an incidental finding on clinical examination . SeveralForeign body presenting as a granuloma or polyp is rare and is found uncommonly in the literature. The present case report emphasizes the need to keep an underlying foreign body in mind when one encounters an aural polyp in clinical practice. This will prevent unnecessary interventions.A 19-year-old female presented to our department with a history of persistent purulent discharge from the left ear of three-month duration associated with a reddish mass for two months. She is congenitally profoundly deaf with no intelligible speech. She did not have any past history to suggest chronic suppurative otitis media. She denied any ear pain, giddiness, facial weakness, or other symptoms to suggest complications. There was no preceding history of rhinitis. There was no history of dermatitis or seborrhea.Otoscopy revealed a pinkish polyp completely filling the left ear canal and nonfoul smelling purulent discharge. The exact site of origin could not be established with a probe test. The polyp looked smooth, nonulcerative, and nontender. The right ear, nose, throat, neck, and systemic examination were normal.A one-week course of amoxicillin clavulanate and topical antibiotic steroid drops was prescribed after cauterization with silver nitrate. This reduced the size of the polyp and the discharge. Two further attempts at cautery failed to give any benefit and the polyp started to grow to its pretreatment size and the discharge resumed. A computed tomography (CT) scan of the temporal bones was requested.The CT scan revealed a soft tissue mass completely filling the left external auditory canal and pushing the left tympanic membrane medially towards the middle ear cavity with normal pneumatization of the mastoid air cells and the rest of the middle ear cavity Figures  and 2.Informed consent was taken from the patient and patient's guardian after explanation. Left complete aural polypectomy was done under general anesthesia using the operating microscope. The polyp had a broad attachment to the superior and posterior meatal walls and the ear canal skin was excoriated. Dark black material, firm in consistency and friable, was found filling the meats up to the tympanic membrane deep to the polyp. Tympanic membrane was found to be intact. The removed foreign body pieces  appearedHistopathological assessment of the specimen revealed an inflammatory epithelial polyp which was polypoidal and covered by squamous epithelium. The epithelium showed hyperkeratosis, parakeratosis, and acanthosis. Stroma showed proliferated capillaries, hemorrhage, and inflammatory cell infiltration. Another area showed ulcerated inflammatory granulation tissue with a focus of foreign body giant cell reaction.Aural polyps are often attributed only to chronic suppurative otitis media. This case illustrates that other causes should be borne in mind and it is prudent to actively rule out a foreign body by detailed history. When the CT scan rules out middle ear and mastoid involvement, other primary external auditory canal diseases need to be considered .Usually, aural polyps are of inflammatory origin composed of nonspecific inflammatory granulation tissue. These simple aural polyps can offer microscopical clues to some underlying complications of chronic otitis media. Others may reveal clinically unsuspected diseases .In a study done by Gliklich et al., in 1993, they mentioned that 15/35 of their patients with aural polyps were found to have chronic suppurative otitis media and 10/35 had cholesteatoma, while 8 patients had aural polyps on top of foreign body (ventilation tubes) .In his review in 1990, Friedmann stated that the possible causes of aural polyp in the ear might include pyogenic granuloma, relapsing polychondritis, Langerhans cell histiocytosis, neoplastic conditions like osteoma, tumours of the ceruminous glands, rhabdomyosarcoma, and progressive necrotizing otitis externa . TubercuGentle aural cleansing and application of antibiotic-steroid drops can be helpful in reducing the size of the polyp. Also, application of silver nitrate can be helpful in the initial therapy of the aural polyp . In our CT plays an important role in the diagnosis and in specifically the determination of the extent of the disease. It also provides a tool for better evaluation of the surrounding structures and it is considered as the best method to visualize the middle ear when complete occlusion of the external auditory canal (EAC) is the case .In the study case, complete evaluation of the tympanic membrane and extent of the aural polyp was not possible even with microscopic examination in the outpatient clinic. We employed CT scan to assess the extent of the polyp and the condition of the middle ear and mastoid air cells.Law and Watters in 1997 described a case of a penetrating oral foreign body presenting with an aural polyp. The possibility of a penetrating oral injury should be considered whenever a child's fall is unwitnessed. They documented that an underlying foreign body should be considered in cases where an aural polyp fails to respond to standard therapy .Harris et al. in 2004 described a case of a child with chronic otorrhea for 4 months not responding to topical and systemic antibiotics. When the case was referred to them, a large external canal polyp covering an electrical cap foreign body was discovered. They stated that external auditory canal polyps that fail to respond promptly to conservative medical therapy warrant a computed tomography scan and surgical exploration with biopsy .In our case, history of the patient's condition did not help to know the actual cause of the aural polyp. The CT scan done after failure of medical treatment did not give any indication of an underlying foreign body, though it ruled out chronic suppurative otitis media. Only an aural polypectomy under general anesthesia elucidated the diagnosis when multiple pieces of foreign body were found between the site of the polyp and the tympanic membrane.Any aural polyp should be evaluated thoroughly by imaging techniques. Polypectomy should be considered to be mindful that it may uncover a serious disease process or even a surprising one like in our presented case.


Traumatic abdominal hernia is rare and difficult to diagnose from physical symptoms.A 60-year-old woman was admitted to the emergency department with complaints of vomiting after falling off a bicycle and hitting her abdomen against one of the handlebars 2 days earlier. Computed tomography (CT) demonstrated abdominal wall hernia from blunt trauma to the left upper abdomen. The patient underwent exploratory laparotomy, and the herniated bowel loop was not found to be perforated or gangrenous. Primary hernia repair without resection of the bowel loop was performed.Postoperative course was uneventful.Surgical exploration with primary repair of the defect is the definitive treatment in the present case, as the hernia contained an incarcerated loop of small bowel. The use of abdominal CT to confirm the diagnosis before operative repair of the hernia appears to be a safe and efficacious adjunct to physical examination. Blunt traumatic abdominal hernia is defined as a herniation through disrupted musculature and fascia without skin penetration and no evidence of a prior hernial defect at the injury site . Since tHerein, we report the case of a 60-year-old female diagnosed with traumatic abdominal wall hernia with delayed presentation of bowel obstruction, which may have been caused by low-velocity impact with a bicycle handlebar. The present case did not manifest any trauma-related symptoms apart from associated superficial skin injuries until a delayed onset of bowel obstruction. Careful follow-up using computed tomography (CT) enabled early diagnosis and treatment. Considerations in clinical and radiologic diagnosis and surgical management based on our experience are discussed.A 60-year-old woman was admitted to our hospital with complaints of vomiting and abdominal fullness following blunt abdominal trauma. She had fallen on a bicycle handle bar while bicycle riding 2 days before presentation to the emergency department. The patient developed persistent vomiting and a fever the following day. She was clinically stable with no obvious head or skeletal injuries.Superficial contusion was present in her right iliac fossa, which bore the imprint of the bicycle handlebar. There was no edema with local rise of temperature in the overlying skin. An electrocardiogram was normal. While the abdomen appeared more distended, peritoneal signs were absent. The patient denied any previous abdominal wall defects. Her hemoglobin level remained stable, but her white blood cell count was elevated to 12,240 cells/μL. Her blood chemistry was as follows: aspartate aminotransferase, 25 U/L; alanine transferase, 20 U/L; alkaline phosphatase, 335 U/L; lactate dehydrogenase, 319 U/L; creatine kinase, 541 U/L; and C-reactive protein, 3.7 mg/dL.Contrast-enhanced CT demonstrated an anterior abdominal wall hernia. Small bowel loops were identified within the subcutaneous tissue of the upper abdomen Figure . Based oAbdominal wall disruption following blunt trauma is a rare but challenging injury in both the acute and chronic phases. Traumatic abdominal wall hernia is defined as the ‘herniation through disrupted musculature and fascia associated with adequate trauma, without skin penetration, and no evidence of a prior hernia defect at the site of injury’ . The repSince these hernias can go undetected due to preservation of the skin overlying the hernial defect, diagnosis of traumatic abdominal wall hernia is usually made by CT scan and ultrasound to assess the injury and identify the detect in the anterior abdominal wall. CT is a useful adjunct to clinical examination and surgical exploration with primary repair as the definitive treatment in hemodynamically stable patients with traumatic abdominal wall hernia . OccasioWhether such patients require urgent laparotomy remains controversial. Most authors advocate immediate laparotomy with repair of the defect because of the high incidence of associated intra-abdominal injury (up to 30%) and to avoid complications such as incarceration through or strangulation and subsequent morbidity. In addition, mesenteric and bowel injuries that are liable to be missed on CT scan can also be managed well in time . Thus, tOn the other hand, a conservative approach would be appropriate to avoid negative outcomes in cases in which there is no associated intra-abdominal visceral injury requiring operation or bowel incarceration in the hernia . We agreAlthough laparotomy allows other injuries to be assessed and diagnosed at the time of operation and primary closure of defects, disadvantages include a large incision and increased postoperative recovery time. In our case, we avoided a laparoscopic approach to explore other highly suspected organ injuries in the abdominal cavity. In fact, Gutteridge et al. reviewed their cases of traumatic abdominal wall hernia and reported that none of their cases were able to be managed by laparoscopy alone . The lapWe described a case of traumatic abdominal wall hernia requiring surgery for bowel strangulation. Patients who are diagnosed with traumatic abdominal wall hernia should be explored by CT scan as early as possible to look for intra-abdominal injuries, followed by careful examination to detect early manifestations of organ injuries. Physicians must be aware of the chances of early as well as late incarceration of the bowel in the defect leading to subsequent perforation or strangulation.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A 26-year-old woman was admitted with the chief complaint of chest pain. She had delivered her first child 9 months before admission. Computed tomography showed a bulky mass in her left chest, and histopathological analysis revealed it to be dedifferentiated liposarcoma. We initiated doxorubicin chemotherapy, and the tumor mass reduced. After that, we performed vascular embolization along with chemotherapy, but tumor size did not reduce. On the 160th day of illness, the patient died. This is the first report of a primary diaphragmatic dedifferentiated liposarcoma diagnosed after delivery. Establishment of a regimen of chemotherapy for bulky unresectable liposarcoma is necessary. Intrathoracic liposarcoma is a very rare tumor. About 90%–95% of liposarcomas occur in the retroperitoneum and femoral regions. Liposarcoma does not show predominance in any one sex and occurs most commonly in individuals in their 40s and 50s. Liposarcoma occurring in young patients is rare, and no cases of primary diaphragmatic dedifferentiated liposarcoma diagnosed after delivery have been reported. Here, we present the case of a young 26-year-old woman with primary diaphragmatic dedifferentiated liposarcoma diagnosed after delivery.A 26-year-old woman was admitted to our hospital with left-sided chest pain and a 4-month history of exertional dyspnea. She had delivered her first child 9 months before admission. Physical examination showed decreased breath sounds in the left lung field. Blood tests showed elevated serum lactate dehydrogenase (343 IU/L) and C-reactive protein (4.28 mg/dL). Serum tumor markers carcinoembryonic antigen (0.5 ng/mL), alpha-fetoprotein (1.9 ng/mL), and soluble interleukin-2 receptor (401 U/mL) were all within normal range.Contrast-enhanced computed tomography (CT) showed a bulky mass with heterogeneous enhancement . We perf2, days 1, 2, and 3, every 3 weeks) for tumor reduction, which was obtained after 2 cycles of chemotherapy  and doxorubicin , but her pulmonary congestion worsened by excessive fluid transfused to prevent ifosfamide toxicity , and the treatment was stopped. We next performed vascular embolization of the main nutrient vessel. Subsequently, we tried chemotherapy with single agent of docetaxel , doxorubicin , and pazopanib (800 mg/day); however, no treatment effects were observed. On the 160th day of illness, the patient died due to progression of her dedifferentiated liposarcoma.Treatment was begun with doxorubicin , and the next most common type is well-differentiated liposarcoma. Well-differentiated liposarcoma has a good prognosis, with 5-year survival rates of 80%. Meanwhile dedifferentiated liposarcoma has a poor prognosis, with a recurrence rate of 40%–50%, a metastasis rate of 20%, and a 5-year survival rate of 28% , 7. The Small specimens such as those obtained by needle biopsy are insufficient to determine the histologic type of liposarcoma, so tissue diagnosis by surgical resection is preferable. However, in cases where surgical resection is difficult, such as the present case, it is important to biopsy from multiple sites of tumor in order to obtain a correct pathological diagnosis. As a result, we are able to predict a prognosis and a therapeutic effect.The principle treatment for liposarcoma is surgical resection. Klimstra et al. reported that the mean size of liposarcoma at surgery is 157 mm (range 60–0400 mm) and the mean tumor weight is 1500 g . SurgicaThere is no established standard regimen of chemotherapy for dedifferentiated liposarcoma. In myxoid liposarcoma, it was reported that chemotherapy with doxorubicin plus ifosfamide had a response rate of 43.2% . For ourHere, we present a case of dedifferentiated liposarcoma in a young female patient after delivery. Recently, overexpression of the estrogen receptor and androgen receptor in well-differentiated and dedifferentiated liposarcoma was reported . In the We present the first report of a primary diaphragmatic dedifferentiated liposarcoma diagnosed after delivery. The current standard of treatment for liposarcoma is surgical resection, which, however, is not always possible in bulky intrathoracic liposarcomas. Therefore, we propose that the establishment of a multidisciplinary regimen for bulky unresectable intrathoracic liposarcomas is necessary.


Objective. The aim of this paper is to present an uncommon clinical and radiographic aspect of odontogenic keratocyst (OKC) mimicking paradental cyst. Methods. A 32-year-old female patient showed a well-delimited radiolucent lesion connected with the root of the left third molar with close anatomical relationship with the mandibular canal. The clinical, radiographic, and anamnestic features lead us to diagnose a paradental cyst that was treated by enucleation after extraction of the partially impacted tooth. Results. Histological analysis showed typical histological features of PKC such as the presence of a lining of stratified squamous epithelium with a well-defined basal layer of palisading columnar of cuboidal cells. Conclusion. Initial X-ray analysis and the position of the lesion related to the third mandibular tooth caused us to mistakenly diagnose a paradental cyst. We were only able to identify the cyst as an PKC rather than a paradental cyst after histological analysis. Odontogenic keratocyst (OKC) was categorised as a form of odontogenic tumor in 2005 by a WHO working group. WHO defined OKC as “a benign multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potential for aggressive, infiltrative behavior” .Malignant transformation into squamous cell carcinoma is unusual. There exists a second variant of OKC, the orthokeratocyst, which is less aggressive and occurs less frequently 12% of OKC) but is still classified as an odontogenic cyst [% of OKC The odontogenic keratocyst is often associated with Gorlin-Goltz syndrome and is characterised by the presence of multiple nevoid basal cell epitheliomas, multiple keratocysts in the jaws, and bifid ribs . The incThe most common clinical symptoms are swelling, discharge, and pain, but in some cases OKC may be asymptomatic: the frequency of a casual diagnosis ranged between 5,5% and 42,5%.The aim of this paper is to present uncommon clinical and radiographic features of OKC mimicking paradental cyst.A 32-year-old female patient visited our clinic for a routine control. Clinical examination of the oral cavity revealed the presence of two partially impacted third mandibular molars. They did not show clinical aspects of inflammation such as swelling or pain and the mucosa around the tooth site appeared clinically normal .A panoramic radiograph (OPG) revealed a well-defined radiolucency associated with the left third molar , promptiComputed tomography evidenced a well-delimited radiolucent lesion connected with the root of the left third molar and confirmed a close anatomical relationship between the cyst and the mandibular canal . The cliThe partially impacted tooth was removed surgically under local anesthesia and the healing suffered no complications. Our surgical approach involved a trapezoidal flap with vestibular ostectomy, and the cyst was treated by enucleation . The wouHistological analysis showed typical histological features of PKC such as the presence of a lining of stratified squamous epithelium with a well-defined basal layer of palisading columnar of cuboidal cells .These histological results determined our choice of follow-up program, which was selected to control the aggressive behaviour of PKC.Our protocol for patients with PKC includes clinical and radiographic examinations every three months for the first year after surgery and once a year from then on with a TC control every two years.The orthopantomography conducted one year after surgery showed no signs of relapse .Differential diagnosis represents an important and complex phase of the clinical process. Diagnosis can often be no more than hypothetical, despite thorough anamnesis, accurate clinical analysis, and instrumental examination.A radiolucent lesion, located in the mandibular area, can be more or less aggressive in nature . Differential diagnosis must therefore be executed in order to accurately identify the lesion.In the clinical case under examination, the lesion was small and unilocular, presented clearly defined edges, was not corrosive, and was located in proximity of the mandibular angle, associated with the third molar in dysodontiasis. The lesion was classified as a paradental cyst.This particular classification was diagnosed due to the close proximity of the lesion to the root of the third molar. Follicular cyst and OKC were therefore excluded. Histological examination of the biopsy, however, contradicted this diagnosis, indicating a case of PKC.Diagnosis of OKC is highly important due to its aggressive behaviour and the frequency of recurrence after surgical treatment, which has been observed to occur at a rate between 2,5% and 62,5% . PossiblDiagnosis of OKC is often very difficult because its clinical and radiographic aspects are aspecific. Common radiographic features are unilocular or multilocular well-circumscribed radiolucent lesions surrounded by a thin sclerotic border. While the multilocular variant is difficult to differentiate from other odontogenic or nonodontogenic neoplasms like ameloblastoma , the uniFrom a clinical perspective, these lesions can demonstrate similar symptoms such as swelling, pain, or, in some cases, suppuration from pericoronal sulcus –21. In cIn our case, initial X-ray analysis and the position of the lesion related to the third mandibular tooth caused us to mistakenly diagnose a paradental cyst. We were only able to identify the cyst as an OKC rather than a paradental cyst after histological analysis. From a histological perspective, there are marked differences between the two types of cyst. OKC is characterised by the presence of a thin bandlike parakeratinized stratified squamous epithelium, with a prominent basal layer composed of palisade-like columnar cells and a connective tissue wall that generally does not demonstrate inflammation. Paradental cysts, like other inflammatory cysts, present chronic inflammatory cell infiltration and are lined by a nonkeratinised stratified squamous epithelium .


Dear Editor,We report the case of a 74-year-old man smoking 80 cigarette packages per year, withhistory of pulmonary tuberculosis for 50 years. Two years ago, the patient underwentchest computed tomography that demonstrated centrilobular and paraseptal emphysema,besides sparse bullae, the largest one located in the right lower lobe, with a smallnodular mass inside, measuring about 0.8 cm in diameter .The patient didn't return for follow-up and after two years presented with progressivedyspnea whose onset had occurred two months ago, in association with cough, weight lossand pain in the lower third of the right hemithorax. A new chest computed tomographydemonstrated a mass with spiculated margins, adjacent to the posterior portion of thelargest bulla, occupying the whole bulla where the nodular mass had been seen at theprevious computed tomography images .Also, iPericardium biopsy and cytological analysis of pleural effusion revealed adenocarcinoma,raising the hypothesis of lung adenocarcinoma with metastasis to the pleura andpericardium. A chemotherapy protocol with gemcitabine and carboplatin was initiated. Thepatient presented worsening of the respiratory condition, progressing to death after twomonths.,2. However, the disease presentation forms are quite variable and sometypical findings may be observed. One of such findings is growth from a preexistingcystic mass, mimicking a fungus ball. Thus, a cystic image showing either focal ordiffuse wall thickening progressing to a nodular mass should include lung tumor in thedifferential diagnosis, particularlyin cases where the nodule is attached to the wall and does not move with change indecubitus.Lung cancer frequently presents like a nodule or solitary lung mass,4.Other conditions which may present the finding of fungus ball include Rasmussenaneurysms, hydatid cysts, abscesses and intracavitary hematomas, besides fungal diseasesthemselves ,2,5. The tumor tends to infiltrate in the adjacent pulmonary parenchymacausing a paracicatricial effect, and may lead to emphysematous or cystic changesadjacent to the neoplastic process.As the neoplasm develops in previous pulmonary lesions, it is found especially infibroatelectatic or granulomatous areas resulting from sequelae, generally associatedwith tuberculosis. The occurrence of lung cancer in cavities mimicking fungus ball orair crescent sign is quite rareIn conclusion, lung cancer must be considered in the differential diagnosis for patientswho present with a fungus ball-like lesion, particularly in cases where the nodule isfixed to the cavity wall.


Odontogenic keratocysts (OKCs) are developmental cysts which occur typically in the jawbones. They present more commonly in the posterior mandible of young adults than the maxilla. OKCs have been reclassified under odontogenic tumours in 2005 by the WHO and have since been termed as keratocystic odontogenic tumours (KCOTs). Here we report a case of a recurrent buccal lesion in a 62-year-old man which was provisionally diagnosed as a space infection  but surprisingly turned out to be a soft tissue KCOT in an unusual location on histopathologic examination. Odontogenic keratocysts are developmental odontogenic cysts which were reclassified as tumours in 2005 by the WHO on account of their aggressive behavior and recurrence potential . They arThe histogenesis of intraosseous KCOT can be attributed to the sources of odontogenic epithelium, that is, dental lamina and its remnants and the extensions of basal cells from the overlying epithelium . The invCysts and tumours which arise from the epithelium and its remnants associated with tooth development are termed as “odontogenic.” These lesions typically occur centrally within the bone. However, occasional reports exist concerning those odontogenic lesions having occurred in the soft tissues overlying the alveolar bone. These have been aptly termed “peripheral” or “extragnathic” .Rare cases of parakeratinized cysts in the facial soft tissues, skin, muscles of mastication, and also temporomandibular joint, which histopathologically resemble intraosseous KCOT, have been reported. Owing to the rarity of these loci, variants of KCOT can be described and further discussed as intraosseous, solid-cystic, and peripheral types .A 62-year-old male reported to our hospital with an asymptomatic swelling in the right lower half of the face with reduced mouth opening since two weeks. History revealed that this was the 3rd time that such a swelling had appeared in the buccal region over a span of 8 years. The first episode 5 years before involved a previous history of pus discharge with a diagnosis of buccal space infection treated by incision and drainage. The buccal swelling had regressed on its own upon a second occurrence 2 years back. The patient has been a known diabetic since a year and on regular medication. A detailed medical examination was performed in which the patient was screened for anomalies of the skin, eyes, skeletal system, endocrine system, nervous system, and any other developmental disorders, ruling out any syndromic association.On examination, gross asymmetry on the right side of the face was evident. A large ovoid submucosal swelling (6 × 6 cm in size) was observed extraorally, extending superoinferiorly from the level of the right lateral canthus of the eye to 1 cm below the corner of the mouth and anteroposteriorly from the corner of the mouth to the mandibular ramus . The borIntraorally, the ovoid swelling presented as a slight elevation of the buccal mucosa without any obliteration of the buccal vestibule. It extended from 1 cm from the commissure of lip to 1 cm short of the pterygomandibular raphae. The buccal mucous membrane appeared stretched and glistening without any signs of inflammation. Mouth opening was restricted due to the size of the lesion.Computed tomography revealed a well-defined lesional mass with cystic areas involving the buccal space. Intracystic loculation was visible with a pus-like dense cystic content. Erosion of the posterior buccal cortex was evident with no perforation of the maxillary bone. The maxillary antral wall was compressed Figures .A clinical diagnosis of a nonspecific chronic abscess with spread of infection was made and the lesion was treated under GA through an intraoral approach. An incision was made on the buccal mucosa and the lesion was excised in toto along with a safe margin of a fibrous tissue cuff. The lesion shelled out easily without any involvement of the buccinator muscle nor of any bony component. On surgical exposure, the lesional mass was well circumscribed with a fibrous outer covering. The lesion looked lobulated as the mass had herniated into the pterygomaxillary space Figures .The excised lesion resembling a leathery pouch was present completely within the soft tissue in the buccal space. It was not attached to the buccinator muscle nor was it attached to any bony component of the maxilla or related to the parotid gland or duct.The specimen obtained was 4.8 × 4.5 cm in size, creamish brown in colour with a firm consistency with an irregular surface. Multiple samples were routinely processed and stained with hematoxylin and eosin .A differential of a large dermoid or epidermoid cyst, a parasitic cyst, and a cyst of inflammatory origin was considered.Microscopically, the tissue sections showed a cystic lumen lined by parakeratinized epithelium of uniform thickness. The basal cells were cuboidal to columnar in shape exhibiting nuclear palisading. The spinous layer was thin and the parakeratin layer showed surface corrugations. Some areas were lined by flattened, stretched epithelium whereas other areas exhibited numerous infoldings of the epithelium into the connective tissue wall. The epithelium connective tissue interface was smooth and flat with no rete ridges. Underlying fibrous connective tissue was thin and mature with fibrocytes and fibroblasts with few odontogenic rests. Areas of haemorrhage and blood vessels were evident in the stroma. No daughter cysts, epithelial islands, skeletal muscle tissue, or any appendages were present in the connective tissue wall Figures . extragnathic KCOT of the buccal region was made taking into account mainly the histologic features supported by the clinical and CT (radiologic features). Regular patient follow-up is carried out every 6 months with no evidence of recurrence in 24 months.A diagnosis of anThe lesional tissue was immunostained with Ki-67 (Biogenex) to assess the mitotic activity of the cystic lining to reveal the proliferative potential of the lesion as compared to conventional intraosseous KCOT. An interesting finding was that almost all the basal cells and only few suprabasal cell nuclei stained positive for Ki-67. A converse of this finding is generally observed in most conventional intraosseous KCOTs wherein a larger number of suprabasal cell nuclei stain positive for Ki-67 as opposed to the nuclei of the palisading basal cells Figures .A PubMed search of the English literature of all the cases of KCOT occurring in the buccal region has been compiled in a chronological order in KCOTs are cystic odontogenic lesions arising in the gnathic skeleton. KCOT has been defined by the latest WHO classification of tumours of head and neck as “a benign uni- or multicystic, intraosseous tumour of odontogenic origin, with characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive, infiltrative behaviour” . KCOTs hThough KCOTs conventionally occur in an intraosseous location; reports of odontogenic tumours like ameloblastoma, CEOT, and AOT including KCOT arising in a peripheral location exist in literature . The locHowever, KCOTs of the soft tissue have been reported in other anatomic sites like the buccal mucosa and masticatory muscles apart from the gingiva. A review of the English literature revealed 25 cases of peripheral (soft tissue) KCOTs. Of the 25, 17 of them were found in the gingiva, 6 in the buccal mucosa, and 2 cases intramuscularly .Generally KCOTs of the jawbone do not produce a large expansion of the cortex as the cyst proliferates within the marrow spaces, thus growing in a longitudinal manner. But when it occurs in the buccal soft tissues, it tends to grow in a centrifugal direction, therefore causing the cyst to expand uniformly on all sides, resulting in a large buccal swelling as the main clinical presentation. The cyst also simulated spread of infection as it had extended into the pterygomandibular space and infratemporal space but could be easily dissected during surgery.Plain radiographs play a very limited role in the detection of a soft tissue lesion. However, studies prove that the positive rate of computed tomography (CT) in the diagnosis of PKCOT  is 100%. CT can reveal the relationship between the lesion and the adjacent hard or soft tissues. By measuring the CT value, the lesion can be defined as cystic [Though an organising abscess with buccal space infection can give a similar picture, absence of signs of inflammation ruled out this possibility.KCOT is a distinct lesion, owing to its unique histopathology. The cystic lining is characterized by a parakeratinized stratified squamous epithelium of uniform thickness, with a corrugated surface and a palisaded and polarized basal layer with “picket fence” or “tombstone” appearance, devoid of rete ridges. Numerous infoldings of the epithelium are a common finding. The cystic lumen generally contains a straw colored fluid or a thick creamy material which may represent keratin .Our case also exhibited a cystic epithelium comprising of numerous folds and a benign fibrous connective tissue wall. The lining was characterized by 5-6-cell layer uniformly thick epithelium with parakeratinization and surface corrugation, basal cell palisading and devoid of rete pegs which conforms to the typical descriptive diagnosis of an intraosseous KCOT.The main histopathologic differentials for a soft tissue KCOT include epidermoid cyst, cutaneous keratocyst (CKC) ectopic occurrence of keratocyst of skin adnexa in buccal mucosa , trichilemmal cyst, and steatocystoma.Epidermoid cysts are developmental pathologies occurring in the head and neck region almost always found in the regions of embryonic fusion with an intraoral incidence of less than 0.01% . HistoloCKCs were first reported by Barr et al. as subcutaneous cysts in 1986 . CKCs arThe lining epithelium comprises several layers of squamous epithelial cells without a granular cell layer and skin appendages. The cystic epithelium appears wavy or festooned with normal maturation . PresencSteatocystoma simplex is an uncommon benign cutaneous hamartomatous malformation arising from the pilosebaceous duct junction , 18. It Trichilemmal cysts mainly occur on the scalp and their cystic content comprises a cheesy, foul smelling thick material . The cysThe mechanism of KCOT development in the soft tissues especially the buccal mucosa is yet to be established. It is common knowledge that the KCOT is considered to originate from the remnants of dental lamina. Therefore, to have occurred in the buccal soft tissues, these cells should have been displaced into the buccal mucosa and persisted during embryogenesis . Recent These findings suggest that the remnants of the dental lamina entrapped in the buccal tissues during embryogenesis may be triggered to form a keratocyst with the features of KCOT. Other odontogenic lesions such as ameloblastoma and odontoma developed in the buccal mucosa have also been reported in the English literature , 27.It is also established that KCOTs may originate from the basal cells of the oral epithelium . TherefoIn the statistics of soft tissue KCOT given by Abé et al., the recurrence rate of 21 cases was 13.6%, which is in close proximity to the recurrence rate of jawbone KCOTs , 28.In this case, as the cyst was present in an unusual location, that is, the buccal soft tissue, IHC was applied to compare intraosseous KCOT and extraosseous KCOT in its Ki-67 staining properties.Previous reports and observations reveal that, in conventional intraosseous KCOT, a larger number of suprabasal cells and very few basal cells take up the stain. But, in our case, a larger number of basal cells as compared to suprabasal cells showed positivity for anti-Ki-67 antibody.This finding may question the general assumption that peripheral counterparts of central odontogenic lesions may exhibit less aggressive behaviour and may show lesser rates of recurrence. A larger number of cases need to be included to test this premise. Until then this will remain as a topic of speculation.The diagnosis of KCOT of the jawbone is based purely on its characteristic histologic picture. However, in our case, similar findings were associated with a keratinizing cyst of the buccal mucosa. The locale for the predilection of this site is extremely rare, but the rationale for this topographic location can be explained by a theory of displaced and persistent dental lamina rests in the buccal mucosa during odontogenesis in the embryo. Therefore, though KCOT of the buccal mucosa represents an example of heterotopia, it is to be noted that the frequency of detection and identification of the peripheral variant of this pathology has risen. As our case is the 7th one to be reported till date, we propose that PKCOT deems recognition as a separate entity or as an extragnathic  counterpart of the central (intraosseous) KCOT.


Calcification of the yellow ligament sometimes compresses the spinal cord and can induce myelopathy. Usually, the calcification does not induce acute neck pain. We report a case of a patient with acute neck pain caused by calcium pyrophosphate dihydrate in a calcified cervical yellow ligament.A 70-year-old Japanese woman presented with acute neck pain. She had a moderately high fever (37.5 °C), and her neck pain was so severe that she could not move her neck in any direction. Computed tomography showed a high-density area between the C5 and C6 laminae suspicious for calcification of the yellow ligament. Magnetic resonance imaging showed intermediate-signal intensity on T1-weighted imaging and high-signal intensity on T2-weighted imaging surrounding a low-signal region on both T1- and T2-weighted imaging with cord compression. There was a turbid, yellow fluid collection in the yellow ligament at the time of operation. Histologically, calcium pyrophosphate dihydrate crystals were found in the fluid, and she was diagnosed as having a pseudogout attack of the yellow ligament.Pseudogout attack of the cervical yellow ligament is rare, but this clinical entity should be added to the differential diagnosis of acute neck pain, especially when calcification of the yellow ligament exists. Calcification of the yellow ligament of the cervical spine is not rare and sometimes compresses the spinal cord and induces myelopathy . Total rUsually the calcification does not induce acute neck pain. However, CPPD deposition disease is known to induce acute arthritis in other joints such as the knee. We previously reported a series of patients with acute neck pain caused by lateral atlantoaxial joint CPPD crystal-induced arthritis . However3 , and C-reactive protein was 5.13 mg/dL . Computed tomography (CT) showed a high-density area between the C5 and C6 laminae with suspected calcification of the yellow ligament  showed low signal on both T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI) (Fig. Six months postoperatively she had no further severe neck pain. At a follow-up evaluation 7 years after initial onset, our patient had complete relief of neck pain, and there were no neurological abnormalities.This patient’s course highlights two important clinical issues. First, pseudogout attacks of the cervical yellow ligament can cause acute neck pain. Second, calcification of the cervical yellow ligament raises suspicion for pseudogout.There have been some reports of cervical canal stenosis caused by CPPD crystal deposition . MyelopaFor diagnosis CT and MRI are important. On CT, calcification of the yellow ligament was seen in the present patient. MRI is the preferred imaging method because of the excellent soft tissue contrast that is achievable. In this case, effusion around the calcification was visible. Surgery is needed if myelopathy is present. It is possible that this patient may have been treated without surgery because cord compression was not very severe. Conservative treatment with NSAIDs or cortisone is another treatment option. However, surgical decompression is a safe and effective treatment for calcification of the cervical yellow ligament. In this case, surgical decompression was chosen because the symptoms remained after conservative treatment.Pseudogout attack usually occurs in a joint with radiographically proven calcium deposition. Although pseudogout can occur without radiographic evidence of chondrocalcinosis , 6 radioPseudogout attack of the cervical yellow ligament is rare but this clinical entity should be added to the differential diagnosis of acute neck pain, especially when calcification of the yellow ligament is present.CDS, crowned dens syndrome; CPPD, calcium pyrophosphate dihydrate; CT, computed tomography; MRI, magnetic resonance imaging; NSAIDs, nonsteroidal anti-inflammatory drugs; T1WI, T1-weighted imaging;T2WI, T2-weighted imaging; VAS, visual analog scale.


Glioblastoma is the most common and the most malignant type of gliomas. Cerebral hemispheres are usual locations for gliomas. Isolated third ventricular presentation is very rare for glioblastomas. A new case of isolated third ventricular glioblastoma has been presented in this report.A 36-year-old woman was admitted to outpatient clinic with headache, blurred vision and confusion. A head CT scan and MRI had showed third ventricular mass lesion with obstructive hydrocephalus. Previous to her admission to our clinic, a ventriculo-peritoneal shunt had been inserted and her hydrocephalus had been relieved to some extent in acute settings. In our clinic, stereotactic biopsy was performed and a second ventriculoperitoneal shunt was inserted from the opposite site. Histopathological diagnosis was glioblastoma. Radiotherapy and chemotherapy were started immediately after the surgery. Patient’s hydrocephalus has resolved and she was well at post-operative 6th month.In differential diagnosis list of the tumors presenting in the third ventricle, there are plenty of tumors such as colloid cyst, meningioma, germinoma, craniopharyngioma, lymphoma, choroid plexus papilloma, subependymal giant cell astrocytoma, chiasmatic and hypothalamic benign astrocytoma. Ring enhancement of this region pathology is a peculiar sign for glioblastoma, yet not pathognomonic. Tumor histology is crucial to yield the final diagnosis.Management of obstructive hydrocephalus, making histopathological diagnosis, starting adjuvant radiotherapy and chemotherapy in isolated third ventricular glioblastomas is a safe and effective approach when we consider malignant nature and intractable progress of glioblastomas. Gliomas are primary tumors of central nervous system (CNS). They are pathologically derived from astrocytes (82 %), oligodendrocytes, or both cell types  shunt had been inserted and her hydrocephalus had been relieved to some extent in acute settings. Thereafter, she had been referred to our clinic for management of the third ventricular lesion.In her examination, there was cognitive decline and gait instability. A heterogeneously enhanced multi-cystic isolated third ventricular neoplasm was observed on the brain MRI Fig. a–c. We aGliomas are the most common primary tumors of CNS in adult patients. Glioblastoma is the most malignant and most common type (50–60 %) of all gliomas  should be carefully inspected on MRI (Prieto et al. In differential diagnosis list of the tumors presenting in the third ventricle, there are plenty of tumors such as colloid cyst, meningioma, germinoma, craniopharyngioma, lymphoma, choroid plexus papilloma, subependymal giant cell astrocytoma, chiasmatic and hypothalamic benign astrocytoma (Lee and Manzano Total resection is possible yet hard due to very deep location of the tumor at the center of the brain, within surrounding delicate tissues and fragile vessels. Malignant nature and intractable course of the tumor should be considered while planning treatment. Lee and Manzano  mentionePresentation of glioblastoma as an isolated mass in the third ventricle is very rare. Although MR is very useful in detection of the lesion, definitive diagnosis depends on histopathology due to many other different neoplasms that may present in the third ventricle. Management of obstructive hydrocephalus and giving radiotherapy with chemotherapy after histopathological diagnosis is safe and effective therapeutic approach in isolated third ventricular glioblastoma.


Tumors arising at the lacrimal sac are extremely rare, as a limited number of cases have been reported worldwide. They are commonly primary and the majority of them are malignant and epithelial in origin. Adenocarcinomas account for a small percentage of these tumors. Treatment of local disease mainly includes complete surgical resection. However, metastatic disease has a poor prognosis and the development of new treatment strategies is highly important. Research efforts mainly focus on the identification of molecular targets for therapy. Herein, we describe for the first time a case of a patient with an androgen receptor expressing adenocarcinoma of the lacrimal sac that had an impressive response to abiraterone. The tumors of the lacrimal drainage system are extremely rare as approximately 400 cases have been reported in the literature to date –3. The lTumors arising at the lacrimal sac are usually primary. Most of these tumors are malignant 75%) and of epithelial origin (70%) including squamous cell carcinoma, transitional cell carcinoma, mucoepidermoid, adenoid cystic, poorly differentiated carcinoma, and adenocarcinoma. The non-epithelial histologies include lymphoma, histiocytoma, sarcoma, hemangiopericytoma, malignant melanoma, and neurofibroma . Seconda% and of Lacrimal sac tumors usually present with symptoms such as epiphore, dacryocystitis, and pain with or without associated tumor mass. Differential diagnosis includes infection or inflammatory lesions . CT and MRI of the head and neck are required for the evaluation of the size of the tumor mass, the extent of the invasion into the adjacent structures and assessment of regional lymph node status. Chest imaging is also required at diagnosis as these tumors often metastasize to the lungs .With the exception of lymphomas, lacrimal sac carcinomas should be treated with complete surgical removal of the tumor with wide excision to achieve clear margins. Resection of adjacent structures and lymph node dissection may be required in more extensive disease . For epi2. Baseline CEA level before surgical resection was not evaluated. After surgical resection, CEA level was 13 mg/L.In May 2007, a 65-year-old man was diagnosed with locally advanced lacrimal cyst adenocarcinoma. The patient presented with dacryorrhea and 3 months later, he developed a right parotid mass and multiple enlarged cervical lymph nodes. He underwent a complete surgical resection including right ophthalmus, right parotid gland, and lymph node dissection. Biopsy revealed a lacrimal sac adenocarcinoma with positive surgical margins. Post-operative radiotherapy was administered (50 Gy) with concomitant administration of weekly cisplatin at a dose of 35 mg/mTwo years later, he developed recurrence manifested with extensive superficial cutaneous involvement of the right cervical area. Biopsy confirmed recurrence of a lacrimal sac adenocarcinoma. The tumor cells were positive for EGFR (3+), AR (strongly positive) HER-2 expression (2+) by immunohistochemistry. FISH was positive for HER-2 amplification. Six cycles of carboplatin/docetaxel/trastuzumab were administered every 3 weeks between May and September of 2009. The patient attained a partial response (PR), which was short-lived. Two months following completion of chemotherapy, the patient had disease progression with appearance of new cutaneous lesions. Second line chemotherapy with eight cycles of cisplatin/pemetrexed/bevacizumab every 3 weeks was administered between November 2009 and April 2010. A complete clinical and radiological remission was achieved and maintenance therapy with bevacizumab was given until disease progression.Six months after completion of cisplatin/pemetrexed and bevacizumab triplet and while on bevacizumab maintenance, the patient developed new cutaneous nodules at the right neck area, which were positive for adenocarcinoma on fine needle aspiration. Third line chemotherapy with weekly carboplatin/paclitaxel/cetuximab was initiated. Following the administration of a single dose, the patient developed grade III mucosal toxicity and grade IV diarrhea that led to omission of cetuximab in subsequent treatment cycles. After four cycles of chemotherapy, the patient was deemed to have PR and a decision was made for him to discontinue chemotherapy and proceed to electron beam radiotherapy, which was administered between February and March 2011.In April 2012, a PET–CT identified osteoblastic bone metastasis. Administration of afatinib and zolendronic acid was begun. After 4 months of treatment, afatinib was discontinued due to disease progression, which involved presence of new cutaneous nodules in the right zygomatic area and soft tissue mass in the temporal area. In January 2013, the patient presented to our hospital for the first time for a second opinion. Due to AR positivity, treatment with bicalutamide and LHRH was begun. The patient displayed PR to androgen ablation, which was manifested as size reduction of the temporal mass and decline in CEA level (from 13 to 10 mg/L), which was unfortunately short-lived, as it lasted for only 2 months.In August 2013, bicalutamide was substituted by abiraterone (1000 mg daily) plus prednisolone (10 mg daily), while LHRH analog was continued. Disease evaluation at 3 months revealed a good clinical response with disappearance of cutaneous lesions and a decrease in size of the temporal soft tissue mass, as well as a substantial biochemical response (CEA level dropped from 10 to 4 mg/L). After 6 months of treatment, the patient had a complete response according to RECIST criteria, with resolution of cutaneous lesions and temporal soft tissue mass , an enzyme mainly expressed in testicular, adrenal, and prostatic tumor tissues. More specifically, CYP17 catalyzes two sequential reactions; the conversion of pregnenolone and progesterone to their 17-α-hydroxy derivatives by its hydroxylase activity, and the subsequent formation of dehydroepiandrosterone (DHEA) and androstenedione, respectively, by its lyase activity. DHEA and androstenedione are androgens and precursors of testosterone. As a result, inhibition of CYP17 activity by abiraterone decreases circulating levels of testosterone (Tumors of the lacrimal sac are very rare and heterogeneous. Surgical resection is the backbone of treatment when a total resection of the tumor is feasible. Radiotherapy and chemotherapy may be added. When a tumor recurs locally or distantly and surgery or radiotherapy is not therapeutic options, systemic therapy can be considered. However, administration of chemotherapy is anecdotal and has been disappointing .Adenocarcinomas constitute a small percentage of tumors of the lacrimal sac. These are rare tumors and research efforts focus on identification of molecular targets for therapy. In our case, the tumor was found to be AR positive. This is in concordance with one previous report . Other mThe lacrimal and salivary glands share similar characteristics. Lacrimal sac adenocarcinomas may originate from seromucinous glands in the lacrimal sac. These glands constitute of serous and mucinous units and have been found to be similar in salivary gland and lacrimal sac . FurtherAndrogen-deprivation therapy consisting of bicalutamide and LHRH analog has been repeatedly reported to be an effective treatment option for AR+ adenocarcinomas of the salivary glands –13. In 2Of note, potential side effects of abiraterone are concerning, since grade 3 or 4 adverse events have been reported in almost 25% of patients in large randomized trials . CoadminIn the era of molecular targeted therapy, we recommend for all recurrent/metastatic epithelial lacrimal sac tumors to be tested for AR positivity. Potential administration of androgen-deprivation therapy or abiraterone to female patients with AR expressing tumors is intriguing. A randomized study for assessment of effectiveness of abiraterone in lacrimal sac tumors is warranted, albeit extremely difficult to realize due to rarity of the tumor.In conclusion, androgen-deprivation therapy including the new anti-androgens such as abiraterone and enzalutamide may prove effective treatment options for AR+ lacrimal gland tumors.Elena Vagia reviewed the literature and wrote the paper. Panagiota Economopoulou and Ilias Athanasiadis collected the data. Nikolaos Oikonomopoulos prepared the figure. Amanda Psyrri and George Dimitriadis carried out critical interpretations and contributed in the final version of the paper. All authors read and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Urinary bladder hemangiomas are rare, accounting for 0.6% of the urinary bladder tumors. Hemangioma is considered arising from embryonic stem cells of an angioblastic lineage. A 3-year old boy presented with hematuria. He had past operative history of excision of extensive lymphatic malformation involving retroperitoneum, pelvis and upper thigh. Computed tomography scan of abdomen and pelvis with contrast revealed a large soft tissue mass arising from the dome of the bladder. Partial cystectomy was done. Histopathology confirmed the mass as cavernous hemangioma of urinary bladder. A 3-year old male child presented with pain in the lower abdomen of one month duration which was localized and non-radiating along with one episode of gross hematuria. He had past history of extensive lymphatic malformation involving retroperitoneum, pelvis and upper thigh and was operated for the same 18 months back. The lesion was histologically confirmed as lymphangioma. There was no history of trauma, infection or coagulation abnormalities. Physical examination revealed pallor and scar of previous surgery on right upper thigh along with localized tenderness in hypogastric region. Blood work-up was within normal range, however, urinalysis showed 10–20 red blood cells and 2–5 white blood cells per high-power field. Cystoscopy was done from other hospital which revealed a large 6 cm × 5 cm, reddish mass arising from dome of bladder. Computed tomography scan of abdomen and pelvis with contrast also revealed a large 7cm x 6cm x 2cm soft tissue mass arising from the dome of the bladder and hanging in bladder lumen showing homogenous enhancement . There was no evidence of perivesical mesenteric infiltrate.Surgery was performed through a lower midline abdominal incision. Urinary bladder was defined by extraperitoneal approach. The tumor was palpated. Partial cystectomy was done and part of bladder containing tumoral vascular tissues with a safe margin was sent for histopathological examination. Macroscopically it was 5cm x 3cm x 2cm partial cystectomy specimen which on cut-section showed a large soft to firm hemorrhagic tumor mass measuring about 2.5cm x 1.6cm x 1cm . Histopathological examination revealed a tumor occupying the submucosa and muscle layer; it was composed of dilated vascular spaces lined by flattened endothelial cells containing red blood cells over-lined by partially ulcerated transitional epithelium. There was no evidence of atypia .Thus final impression of cavernous hemangioma was given on histopathology. Hematuria resolved after surgery and at 1 year follow-up, the patient was doing fine.Hemangioma is a common benign tumor occurring in various parts of the body, but it is a very rare primary tumor of the bladder representing only 0.6% of all bladder tumors.[1] It occurs in all age groups but is relatively rare in childhood. It has a slight male predominance.[2] In the review of literature most of hemangioma are solitary (66%), varying from few millimetres to 10cm in diameter with a predilection for the dome, posterior wall, and trigone of the bladder.[2] In most cases, the tumors measure 1 to 2 cm and is sessile. In our case it was large in size.In most reported cases, it may coexist with a cutaneous hemangioma or be associated with the Klippel-Trenaunay-Weber syndrome where the hemangioma of the urinary bladder are frequent (3-6%).[3] Hemangioma is also associated with Sturge-Weber syndrome or encephalo-trigeminal-angiomatosis, the Rendu-Osler-Weber disease or hemorrhagic telangiectasia syndrome, and systemic angiomatosis.[3] For this reason systemic evaluation in these patients is highly recommended. In our case except for previous scar of lymphangioma excision surgery, systemic evaluation did not reveal any findings.The predominant clinical symptom of urinary bladder hemangioma is recurrent, isolated hematuria. Other symptoms include suprapubic pain due to vesical irritation and urinary retention.[4] The index case also presented with pain in lower abdomen and gross hematuria.Management of patients with urinary bladder hemangioma is controversial and should depend on their evolution, size and degree of penetration. Treatment vary from observation, transurethral resection, electrocoagulation, radiation, systemic steroids, injection of sclerosing agent, and partial or complete cystectomy.[5] Asymptomatic hemangiomas do not require treatment. Although bladder hemangioma have a benign course, follow up is important to detect recurrence or residual disease. Severe pain abdomen, hematuria resulting in anemia, and suspicion of some malignant lesion are indications of surgery.In our case the patient's progressive anemia and large tender vesicle mass led to the decision of surgical tumor removal with a safe margin. Hematuria resolved after surgery and there was no recurrence of bleeding or mass during 1 year follow-up.Source of Support: NilConflict of Interest: None declared


While paraneoplastic leukocytosis is a common phenomenon in solid tumors, extreme elevations of white blood counts (WBC) in the range of more than 100,000/μl are uncommon in patients with non-hematologic malignancies. Leukocytosis with mature neutrophils due to a granulocyte colony-stimulating factor (G-CSF) producing tumor is only seen on rare occasions.Massive neutrophil leukocytosis of approximately 100,000/μl was diagnosed in a 57-year-old Caucasian woman with metastatic undifferentiated endometrial sarcoma. A bone marrow trephine biopsy revealed massively increased granulopoiesis, but no evidence of monoclonal myeloproliferative disease. After the primary tumor had been resected, white blood count (WBC) plummeted and went back to nearly normal levels within one week. With progressive metastatic disease, granulocyte colony-stimulating factor (G-CSF) plasma levels were found to be increased by 10-fold. White blood count (WBC) strictly correlated with tumor burden and response to chemotherapy. In the final stage of treatment resistent disease, white blood count (WBC) approximated 300,000/μl.We report on a granulocyte colony-stimulating factor (G-CSF) secreting undifferentiated endometrial sarcoma, which was associated with extreme neutrophil counts. White blood count (WBC) were closely correlated with tumor burden and associated with an aggressive clinical course. We suggest that paraneoplastic neutrophilia represents a poor prognostic sign in soft tissue sarcoma. In patients with similar constellations, antitumor therapy must not be delayed. Paraneoplastic leukocytosis is a common phenomenon in several different solid tumors. Affected patients typically have total white blood counts (WBC) between 12,000 and 30,000/μl with mainly mature neutrophil granulocytes [Idiopathic extreme leukocytosis, also known as leukemoid reaction and defined as WBC ≥ 40,000/μl is rare in patients with non-hematologic malignancies: in the largest retrospective analysis of these uncommon patients, only 10% of cases were paraneoplastic in origin. Ninety-six percent of these patients had neutrophilia, and the mean WBC was 53,000/μl . A severBCR/ABL fusion gene. In addition, analysis of JAK2-V617F mutation was performed and showed a negative result.Herein, we report on a 57-year-old Caucasian woman, who presented to our emergency department on July 30th 2011 with severe abdominal pain and a palpable lower abdominal tumor. A computed tomography (CT) scan revealed a 14 × 10 × 12 cm mass with close relation to the uterus (Figure Simultaneously, the general condition of the patient rapidly worsened over night and a further hemoglobin decline was diagnosed. Another CT scan on the following day confirmed abdominal bleeding (white arrows, Figure Only few hours after tumor resection, leukocyte and platelet counts decreased rapidly. Within one week, blood counts returned to near normal levels. Figure Unfortunately, the first postoperative CT scan documented rapid progression of bilateral disseminated pulmonary metastases Figure . In paraIn summary, this case demonstrates severe leukemoid reaction with pathologic left shift caused by a G-CSF secreting advanced undifferentiated endometrial sarcoma imitating hematologic malignancy.Consistent with previously reported cases of paraneoplastic G-CSF secretion, leukemoid reaction was closely correlated with tumor burden and associated with an aggressive clinical course. To our knowledge, neutrophilic leukocytosis up to 200,000/μl and a severe left shift with massively increased G-CSF levels has not been reported before as a paraneoplastic phenomenon in sarcoma. We suggest not to delay antitumor treatment in patients with a similar constellation, but rather aggressively treat the underlying solid malignancy utilizing a response-inducing chemotherapy regimen.Written informed consent was obtained from the patient herself before blood was drawn for G-CSF analysis. The patient agreed to 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.The authors declare that they have no competing interests.CD and HGK wrote the manuscript. SB and EM performed laboratory analyses. MRM, KCW, and LK revised and corrected the manuscript. UV has processed tumor tissue inclusive immunohistochemistry. MH provided CT-imaging. All authors read and approved the final manuscript.


A 47-year-old male with a history of left colon cancer, status post left colon resection for 12 years, presented with rectal bleeding. Colonoscopic examination revealed an 8 mm sessile polyp in the proximal descending colon. Microscopic examination showed that the surface of this polyp was covered with a layer of normal colonic mucosa with focal surface erosion. In the submucosal layer, an intimate admixture of multiple cystically dilated glands and prominent lymphoid aggregates with germinal centers was seen. The glands were lined by columnar epithelium. Immunohistochemical staining showed the glands were positive for CK20 and CDX2 and negative for CK7, with a low proliferative index, mostly consistent with reactive colonic glands. The patient remained asymptomatic after one-year follow-up. A review of the literature shows very rare descriptions of similar lesions, but none fits exactly this pattern. We would designate this inverted lymphoglandular polyp and present this case to raise the awareness of recognizing this unusual histological entity. When glandular epithelium appears in the submucosa or the deeper layers of the intestinal wall, it raises the potential diagnostic confusion with other more serious lesions, such as adenomas with pseudoinvasion or well-differentiated adenocarcinoma. Here, we present a descending colonic sessile polyp, which shows an inverted pattern with an intimate admixture of cystically dilated columnar epithelium lined glands and lymphoid aggregates with germinal centers in the submucosal layer. To date, there have been no reports of the same type of colonic polyp as that reported here.A 47-year-old male with a history of left colon cancer, status post left colon resection for 12 years, presented for surveillance examination and recent rectal bleeding. He had no other symptoms or family history. He underwent esophagogastroduodenoscopy and colonoscopy. The esophagogastroduodenoscopy showed normal vocal cords, esophagus, GE junction, stomach, duodenal bulb, and 2nd portion of the duodenum. On colonoscopy, there was a 3 mm sessile polyp in the cecum, which was ablated with snare tip, an 8 mm sessile polyp in the proximal descending colon, which was snared with electrocautery and retrieved, and a 7 mm sessile polyp in the proximal rectum, which was also snared and retrieved. The patient had an uneventful postoperative course. The patient remained asymptomatic after one-year follow-up.The descending colon polyp was received in formalin and measured 0.6 × 0.2 × 0.2 cm. The specimen was bisected and totally embedded in one cassette. Paraffin embedded tissue was sectioned at 5 microns and multiple levels were evaluated with routine hematoxylin and eosin stain. Microscopic examination showed that most of the surface of this polyp was covered with a layer of normal mucosa with muscularis mucosae, and focal erosion was found on one part of the surface. In the submucosal layer, an intimate admixture of multiple cystically dilated glands and prominent lymphoid aggregates with germinal centers was seen. The glands were lined by columnar epithelium with basally located small nuclei. No goblet cells were present. The lymphoid aggregates surrounded the individual glands Figures . No acutA review of the literature shows very rare descriptions of similar lesions, but none fits exactly this pattern. We would designate this inverted lymphoglandular polyp.Our first impression of the lesion is colitis cystica profunda. Colitis cystica profunda is a rare benign condition characterized by cystic dilation and misplacement of mature crypts through the muscularis mucosa and/or deeper layers of the bowel wall. Colitis cystica profunda is generally localized and only rarely diffuse . The locThe submucosal growth pattern of inverted lymphoglandular polyp is similar to a case report of “inverted colonic mucosal lesion” . HoweverAn intimate admixture of lymphoid aggregates and columnar epithelial glands of inverted lymphoglandular polyp resembles lymphoglandular complex , 6. LympInverted lymphoglandular polyp is also different from inverted hyperplastic polyp. The latter is characterized by proliferative tubules in submucosa with surface mucus hypersecretion .Tanaka et al.  reportedThe mechanism of inverted lymphoglandular polyp is unclear. We propose that this kind of polyp may result from local trauma and subsequent protrusion of mucosal gland into the submucosa through inherently weak areas in the muscularis mucosae, such as that which occurs adjacent to lymphoid aggregates.Lack of cellular atypia and low proliferative index suggests that the neoplastic potential of inverted lymphoglandular polyp is likely to be very low. However, since this is such a rare case, it is uncertain whether it needs to be removed or simply just followed up.


We present the case of a 70-year-old patient admitted with strong abdominal pains and operated in our service for hemorrhagic acute abdomen. Intraoperative hepatic cavernous hemangioma was found with capsular rupture and hemoperitoneum. Due to liver cirrhosis, and no proper technical equipment we chose to perform simple hemostasis. Postoperative evolution was favorable. The complication most feared is rupture; the spontaneous rupture is extremely rare, 21 cases being reported in literature.Cavernous hemangioma is the most common benign tumor of the liver, incidence in different studies ranging from 0.7% to 7%. The discovery is most often incidental on ultrasound examinations for other complaints or at necropsy. The symptoms are polymorphic and can often be attributed to other diseases. More than 4 cm sized giant hemangioma is considered but it may reach sizes of 10-12cm. The therapeutic attitude varies from simple clinical supervision to medication and surgery generally reserved for symptomatic cases  consisting coagulopathy due to coagulation and fibrinolysis inside hemangioma.Hemangiomas are one of the most common liver tumors, second after liver metastases. There are extensive discussions in literature regarding the therapeutic attitude. The symptoms in these conditions are highly variable from pain, anemia, inflammatory manifestations, bleeding, blood disorders and, in adults, rarely Kasebach-Merrit syndrome [4]. Preoperative embolization can be used to lower the blood supply. Surgery is not currently indicated by the size of the hemangiomas but by the complications that may occur: blood disorders, diagnostic uncertainty, hemorrhage, necrosis inside the tumor. Surgical methods are enucleation, liver resection, hepatic artery ligation and liver transplantation [2]. In the case described we could not use these methods due to liver disease and technical equipment limitations. We opted for a minimal solution to remove the patient from the emergency situation.Ultrasound and CT investigations remain elective, MRI being useful for tumors <2cm. Also, there are useful hepatic angiography and biopsy, the last one with high risk of hemorrhage. These patients need extensive investigations to rule out any conditions that can cause the symptoms described by the patients. Treatment is surgical and nonsurgical. Ultrasound follow up and various methods of treatment including interferon α, steroids, arterial embolization, radiation therapy, are used for asymptomatic hemangiomas [We were facing a particular situation of hemorrhagic acute abdomen in a patient with major liver disease due to both toxic-nutritional and viral causes. The etiology of the rupture was still uncertain, the limited degree of cooperation and not very accurate historical data provided could not exclude trauma, even in the absence of traumatic abdominal marks. The minimal solution chosen was beneficial for the patient, long-term prognosis remained limited due to pre-existing liver disease, eating habits and lack of cooperation.Disclosure: none


Background. Xanthogranulomatous inflammation is an uncommon affection of the female genital tract. The youngest case reported of xanthogranulomatous salpingooophoritis in literature was by Shilpa et al. in 2013 in an eighteen-year-old female. Case Report. We report a case of 2-year-old female child with right-sided xanthogranulomatous salpingooophoritis presented as mass in abdomen. This is a case report of the youngest documented case of xanthogranulomatous salpingooophoritis in literature. As per abdominal examination, there was generalized distention of abdomen and a mass was palpable which was arising out of pelvis more on the right side. The ultrasonography (USG) abdomen and pelvis revealed a thick-walled mass measuring 9.2 cm × 6.0 cm × 7.6 cm in pelvis. We did right salpingooophorectomy of the patient. On histopathology, the diagnosis of xanthogranulomatous salpingooophoritis was confirmed. Conclusion. Clinical presentation, radiological appearance, and gross features of xanthogranulomatous lesions of ovary can mimic neoplastic lesions and lead to misdiagnosis. Though, it is very rare in pediatric age group, xanthogranulomatous salpingooophoritis as one of the differential diagnoses should be kept in mind while dealing with tuboovarian masses in this age group. Xanthogranulomatous inflammation is an uncommon cause of chronic destructive process of the normal tissue of the affected organ. It is characterized by accumulation of lipid-laden foamy macrophages  intermixed with lymphocytes, giant cells, and plasma cells . Most coA 2-year-old female child presented with pain in abdomen with abdominal distention and fever since 8 days, as per the history narrated by her mother. She also had history of nausea, vomiting (nonbilious), and constipation since 2 days. As per abdominal examination, there was a generalized distention of abdomen and a mass was palpable which was arising out of pelvis more on the right side. The mass was extending from the right iliac region up to just below umbilicus and crossing the midline towards the left side in the suprapubic region . Mass onThe ultrasonography (USG) abdomen and pelvis revealed a thick-walled mass measuring 9.2 cm × 6.0 cm × 7.6 cm in the pelvis. The right ovary was not seen separately from the mass and it showed irregular solid areas with large cystic changes and thin strands inside. The uterus and left ovary were normal. There was no free fluid in abdomen. Computed tomography (CT) scan showed a large heterogeneous mass arising from of right ovary. X-ray erect abdomen showed air fluid levels .3 with polymorphs 80%, lymphocytes of 20% and platelet count of 2 lakhs/mm3, and ESR 26 mm/hr, serum creatinine was 0.7 mg%, liver function test, urine routine, and microscopy were within normal limits, and urine culture showed no organism. Alpha-fetoprotein value was 11.45 ng/mL . Other tumor markers like CA 125 and CEA were also within normal limits. On exploratory laparotomy, we found a right tuboovarian mass of size approximately 10 cm × 6 cm × 5 cm with distended small bowel loops and no ascites or pus in the abdominal cavity (Hematological investigation showed a hemoglobin of 6.0 gm%, total leucocyte count of 12,000/mml cavity . We did l cavity .On histopathology report, the specimen of the mass was yellowish in color grossly, had smooth outer surface, and measured 9 cm × 6 cm × 5 cm and the fallopian tube of the right side measured 2 cm in diameter and 4 cm in length with exudates on its surface and serous fluid in its lumen. The cut section showed multiple solid and cystic areas with small patchy areas of necrosis too. There was diffuse and dense infiltration of ovarian tissue by sheets of foamy histiocytes, lymphocytes, plasma cells, and polymorphs on microscopic examination. Microscopic examination also showed multiple microabscesses with extensive areas of hemorrhagic and infarctoid necrosis, with areas of marked fibrosis . The spe Escherichia coli, Bacteroides fragilis, and Proteus vulgaris from the affected tissue by culture [Xanthogranulomatous inflammation of the female genital tract is very rare and if involved is confined to the endometrium. However, there are documented cases of involvement of vagina, cervix, fallopian tube, and ovary. The youngest documented case is of 18-year-old female by Shilpa et al. . Kunakem culture . The cas culture , 10. For culture . On the  culture . Histolo culture , 10. RadDifferential diagnosis of xanthogranulomatous oophoritis includes infections like tuberculosis, fungal infections which can be ruled out by culture and special stains for the causative organisms. Malakoplakia is also one of the differential diagnoses. Wather presumed that malakoplakia and xanthogranulomatous inflammation were identical chronic inflammatory diseases . In malaThough it is very rare in pediatric age group, xanthogranulomatous salpingooophoritis as one of the differential diagnoses should be kept in mind while dealing with tuboovarian masses in this age group. This will help in preventing misdiagnosis and radical surgeries if misdiagnosed as neoplasm of ovary. In a proven case of xanthogranulomatous salpingooophoritis, investigations should be done to find the causative factors, such as immunodeficiency disorders, diabetes, inborn errors of lipid metabolism, and pelvic inflammatory disease.


This report describes a rare case of nodular fasciitis (NF) of the oral cavity, discussing the clinical, histological, and immunohistochemical characteristics. Histopathologic diagnosis of this type of lesion can be challenging due to its differential diagnosis, which principally includes sarcoma. The patient presented with a painless, well-defined nodule, reported as increasing in size, located at the apical aspect of the upper left buccal sulcus. Histologically, the lesion revealed spindle cell proliferation arranged in fascicles, while immunohistochemistry demonstrated positivity for smooth muscle actin. Eight months after complete surgical excision, no signs of local recurrence have been observed. Nodular fasciitis (NF) is a benign reactive soft tissue lesion, frequently found in the subcutaneous tissues and muscle fascia, which shows proliferation of myofibroblasts and fibroblasts. While NF has been frequently reported at many anatomical sites, namely, the upper extremities, the trunk, head and neck, and, lastly, the lower extremities, it is extremely rare in the oral cavity , 2. MYH9-USP6, and postulated that NF could be the first example of a self-limiting human lesion that can be characterized by a recurrent somatic gene fusion event.NF was first described by Konwaler et al. as a subcutaneous pseudosarcomatous fibromatosis and has subsequently been nominated as infiltrative fasciitis and pseudosarcomatous fasciitis –5. SeverThe histopathological diagnosis of NF can be challenging, as its differential diagnosis includes other significant soft tissue lesions, such as sarcoma. NF is characterized by myofibroblast and fibroblast proliferation. Immunohistochemical panels are often needed to aid in diagnosis, with NF showing positivity for vimentin, smooth muscle actin, and muscle specific actin –15.The present report describes the clinical and histopathologic features of a rare case of nodular fasciitis of the oral cavity.A 17-year-old white female presented to a private dental clinic complaining of a painless swelling to the apical aspect of the upper left buccal sulcus, which had increased in size over the preceding seven weeks. The medical history was otherwise unremarkable.Initial inspection revealed a gross asymmetry of the face, with no palpable lymph nodes. Intraoral examination demonstrated a well-defined, rubbery, painless, nonulcerated mass at the apical aspect of the upper left buccal sulcus. Ultrasound examination showed a rounded hypoechoic mass . An exciThe specimen was fixed in 10% buffered formalin, with paraffin sections being prepared for light microscopy following standard procedures. Microscopic examination of the Hematoxylin and Eosin (H&E) stained sections revealed spindle cell proliferation arranged in fascicles. In general, the cells were elongated, with oval nuclei and eosinophilic cytoplasm. Rare mitotic figures were observed, yet no atypical mitoses were present. Scarce discrete cell pleomorphism was observed, with myxoid areas also present. A chronic inflammatory process scattered throughout the lesion was observed Figures .Immunohistochemistry revealed spindle cell positivity to the smooth muscle actin (clone 1A4)  antibodyNo sign of local recurrence has been observed after eight months of follow-up .The case reported, herein, fulfills the clinical, histological, and immunohistochemical criteria for the diagnosis of NF, a rare benign tumor of the oral cavity. The challenge in diagnosis of oral NF comes from the fact that the lesion is rare in the oral cavity, as well as the fact that it has a significant histopathologic differential diagnosis .According to the literature, NF is typically diagnosed in young adults between the ages of 20 and 40 years –15. LimaThe clinical features of NF are rather nonspecific, with it usually being described as a well circumscribed nonencapsulated mass, soft and elastic in consistency, which firmly adheres to the underlying tissues , 14. OraNF is characterized by rapid growth, which often leads to tissue distortion, mimicking its malignant counterpart , 12. In Histologically, NF is classified into three subtypes, according to the tissue plane involved: subcutaneous, intramuscular, and fascial. The lesion is usually attached to the fascia from which it arises and extends into the subcutaneous fat in an irregular fashion. Occasionally, it may also arise from the fibrous septa of the subcutaneous fat, only secondarily extending to the fascia , 16. NF α-smooth muscle actin, and muscle specific actin, demonstrating the myofibroblastic differentiation of the spindle cells [Despite the histopathological characteristics of the present case being similar to those of other NF cases, immunohistochemistry was an essential aid for the final diagnosis. In the literature, the immunohistochemical profile of NF includes positivity for vimentin, le cells , 12, 16.le cells , 12, 16.NF is a challenging diagnosis owing to its unusual clinical features, as well as its histological features, which are similar to other lesions , 14, 16.NF should also be distinguished from highly cellular lesions, such as fibrous histiocytoma, which displays dense fascicles of fibroblastic cells, chronic inflammation, lipophages, and giant cells. The myxoid appearance of NF can lead to a misdiagnosis of myxoma, myofibroma, fibrous histiocytoma, and fibromatosis or malignant peripheral nerve sheath tumor . Myxoma,While the treatment of choice for NF is surgical excision , 16, 17,In conclusion, NF is a benign lesion that can occur at any anatomical site, yet it is rare in the oral cavity. Despite its rarity, the dental surgeon and oral pathologist must have an adequate understanding of the lesion in order to establish an accurate diagnosis and offer appropriate treatment.


Massive localized lymphedema (MLL) is a rare pseudosarcomatous lesion due to localized lymphatic obstruction from variable causes. It is most common on medial aspect of thigh and inguinal region. Abdominal localization is rare and may cause clinical diagnostic confusion with other malignant tumors due to its large size. We report a case of abdominal wall MLL of a 56-year-old male patient under clinical suspicion of well differentiated liposarcoma. The literature search and differential diagnosis will be addressed. In doubt cases, immunohistochemical stain or fluorescent in situ hybridization can help to separate this entity from the other mimickers. Massive localized lymphedema (MLL) is a rare pseudosarcomatous lesion that usually develops in morbidly obese individual. The underlying pathophysiology is a lymphatic flow disturbance that may be due to the massive amount of adipose tissue . LymphatMLL most commonly occurs in the medial aspect of proximal thigh. Abdominal wall, scrotum, and upper extremity, although rare, are the other possible areas of occurrence. We present an abdominal MLL clinically mimicking well-differentiated liposarcoma from an obese patient which can be excluded by histological and immunohistochemical characteristics. The literature search and differential diagnosis from other mimickers will also be discussed.A fifty-six-year-old obese male patient (155 kg), with previous medical history of type II diabetes, hypertension, and dilative cardiomyopathy, presented to our surgery department with a 26 × 22 × 9.5 cm sized, polypoid subcutaneous mass below his umbilicus over 4 years. It was soft in consistency and painless. The overlying skin was thicker with “peau d'orange-” like appearance. Neither previous operation nor history of hypothyroidism was claimed. No sign of hypothyroidism was identified during the clinical examination. Due to the large size of the tumor, the patient underwent surgical excision under the impression of sarcoma.µm slides from the formalin-fixed, paraffin-embedded blocks, deparaffinization in xylene and rehydration in a series of decreasing concentration of ethanol were done. Antigen retrieval using either the Bond Epitope Retrieval Solution 1 (pH 6) or the Bond Epitope Retrieval Solution 2 (pH 9)  at 99-100°C for 20–30 min was performed. The slides were treated with S-100 , vimentin , CD31 , MDM2 , and p16 , separately. Immunostaining was performed on Leica BOND-MAX autostainer (Leica Microsystems) and we used peroxidase/DAB Bond Polymer Refine Detection System (Leica Microsystems) for visualization. Cytoplasmic staining for vimentin and CD31 and nuclear staining for MDM2 are regarded as positive stain whereas, for S-100 and p16, both nuclear and cytoplasmic stains are required.After sectioning of 4 During pathological examination, a polypoid skin-covering subcutaneous lesion was received measured 26 × 22 × 9.5 cm in size. The skin was indurated and hyperpigmented with classical “peau d'orange” appearance . On cut Microscopically, the epidermis showed mild acanthosis, elongated rete ridges, basal melanocytic proliferation, and hyperpigmentation . The derImmunohistochemically, the reactive fibroblast cells were stained for vimentin  and theyThe patient underwent follow-up control for 8 months since the operation. No recurrence of the lesion was found and plastic surgery was advised.MLL is a rare clinical entity that has relatively few literature published , 3, 4. IIndeed, clinical pictures and histologically expanded interlobular septae and massive amount of adipose tissue may mimic WDLPS; nevertheless, lymphedema is a superficial process that involves mainly skin and dermo-/epidermochanges related to chronic lymphedema without rapid growth imply a benign process. WDLPS usually forms a discrete lesion, as opposed to the diffuse changes seen in MLL. In microscopic levels, the presence of widened, edematous fibrous septa together with reactive fibroblasts may mimic WDLP.FUS;CHOP) translocation can be used as a confirmation test.In MLL, the adipocytes are evenly sized and lack the characteristic hyperchromatic stromal cells, thick-walled blood vessels containing similar hyperchromatic cells within their walls, and lipoblasts. In challenge cases, immunohistochemical stain for MDM2, CDK4, and p16  or fluorβ-catenin of fibroblast in MLL can also serve as a great diagnostic clue.Desmoid-type fibromatosis may also be one of differential diagnoses due to the reactive fibroblasts and fibroedematous stroma. However, similar to WDLPS, desmoid-type fibromatosis usually presents as a discrete fibrous lesion within the soft tissues with long, sweeping fascicles of bland (myo)fibroblasts together with thin-walled, dilated vasculature and perivascular oedema and peripheral chronic inflammatory cell infiltration which cannot be found in MLL. Negative nuclear stain of Low grade myxofibrosarcoma occurs usually in elderly patient's extremities. It can be differentiated from MLL by its relatively localized nature, atypical hyperchromatic spindle cells, myxoid stroma, and well-developed arcing vasculature.Since the underlying cause of MLL is lymphatic obstruction possibly due to morbid obesity, persistence or even recurrence of these lesions is expected after the surgical resection although recurrence with an aggressive manner has not been documented . RecentlIn conclusion, we present a case of MLL arising from abdominal wall from a morbidly obese patient which is a rare pseudosarcomatous lesion and diagnosis can be challenging for pathologists. Awareness of this rare entity and detailed clinical and radiological information and correlation are required. Differential diagnosis of WDLPS, the most common mimicker, can be ruled out by immunohistochemical stains and fluorescent in situ hybridization. Although rare, secondary sarcomatous transformation had been documented; frequent follow-up is recommended.