The co-incidence of synchronous intraepithelial neoplasia and early stage invasive lung cancer is not a rare phenomenon. The need for curative treatment and the invasive potential of squamous cell pulmonary carcinoma in situ have been a topic of controversy. Surgical resection still remains the treatment of choice. Varieties of endoscopic techniques such as brachytherapy were developed as an alternative to surgery in selected patients. External beam radiation therapy has been used traditionally in combination with endobronchial brachytherapy in the treatment of roentgenographically occult lung cancer, and can be offered for all patients, but is handicapped, because these tumors are radiographically invisible. We report the first case of a pulmonary carcinoma in situ that was successfully treated with stereotactic body radiation therapy. The co-incidence of synchronous intraepithelial neoplasia and early stage lung cancer is not a rare phenomenon. In-situ and early invasive lesions detectable only by bronchoscopy have been referred to as roentgenologically occult lung cancer. A significant prevalence of synchronous roentgenographically occult lung cancer as well as metaplasia and dysplasia was reported in surgical series of patients with resectable roentgenographically visible lung cancer and estimated at 9% .The need for curative treatment and the invasive potential of squamous cell pulmonary carcinoma in situ have been a topic of controversy ,3. WithoExternal beam radiation therapy  has tradWe report on a case of synchronous pulmonary carcinoma in situ and roentgenographically visible early-stage primary non-small-cell lung cancer in a medically inoperable patient, where both lesions were treated simultaneously with hypofractionated stereotactic body radiation therapy.A 78-year-old Caucasian male patient was admitted to our hospital in February 2011 for evaluation of an incidental left lower lung mass, which was detected on chest X-ray performed due to exacerbation of known GOLD stage IV chronic obstructive pulmonary disease (COPD).Chest computed tomography (CT) showed a 2.1 × 2.6 cm sized mass in the left S6 segment with contact to the pleura.Flurdesoxyglucose whole-body positron emission tomography (FDG-PET) demonstrated a pathologic intensive glucose metabolism in the left lower lobe with standardized uptake value (SUV) of 14.1; and much lower metabolism in the subcarinal, left mediastinal and right hilar lymph nodes (SUV=2.7-3.3). No distant metastases were observed. Magnetic resonance imaging of the skull revealed no signs of brain metastasis. Transthoracic fine needle aspiration was performed to confirm the malignancy of the left lower lobe mass. This revealed moderately differentiated, not otherwise specified, non-small cell lung cancer.Fiberrotic bronchoscopy demonstrated no abnormal finding in the trachea and main bronchi. However, the longitudinal mucosal folds were thickened at the orifice of the right upper lobe bronchus, from which a tissue sample was obtained. The pathology reports indicated a squamous cell carcinoma in situ as well as fragments of squamous dysplasia and metaplasia. To exclude the malignancy in mediastinal and hilary lymph nodes, transoesophageal endosonography was performed and revealed several and enlarged lymph nodes in positions 7 and 4 L (up to 1.6 cm), from which specimens were obtained. Cytologically as well as pathologically there was no indication for malignancy in these lymph nodes. The tumor was classified as cT2a cN0 cM0 with synchronous carcinoma in situ (CIS).Because of medical co-morbidities, poor performance status, and compromised lunge function, stereotactic body radiation therapy (SBRT) of the left lower lobe mass and cryotherapy of the CIS disease in the right upper main bronchus were recommended as the most appropriate curative option by the multidisciplinary team. In March 2011, the patient underwent a second bronchoscopy to deliver cryotherapy of the known lesions in the orifice of the right upper lobe main bronchus with 3 freeze cycles of 25 seconds each. There was no post-procedural complication. However, during bronchoscopy, a newly manifested thickness of surrounding mucosa of the right segmental bronchus B1 was recognized. To prevent airways obstruction by the necrotic tissue, second bronchoscopy was performed a week later. It demonstrated further regression of the granulation in the floor of right upper lobe bronchus, but a widened bifurcation between the right B1 and B2 bronchi. The pathological reports confirmed the persistence and multifocality of carcinoma in situ.After a second discussion of the case by the multidisciplinary team, simultaneous SBRT of the carcinoma in situ was planned at the same time with the SBRT of the left lower lobe mass. Before SBRT treatment planning, the patient underwent bronchoscopy to place a Visicoil 0.5×10 mm) fiducial marker per endobronchial ultrasound-transbronchial aspiration (EBUS-TBNA) near the lymph node station 11 R, since the placement of Visicoil via EBUS in the area of the in situ lesions was technically impossible [Figure 0 mm fiduPrior to radiation treatment, a dynamic planning CT with three breathing phases was performed. The internal target volume (ITV) was constructed by union of delineations of the gross tumor volume (GTV) of the left lower lobe mass in all breathing phases, and a margin of 5 mm in all directions was added to create the planning target volume (PTV). A dose of 12.5 Gy × 3 fractions was prescribed to the 65% isodose covering this PTV.For carcinoma in situ disease there was no GTV to be delineated, thus we pragmatically contoured an ellipsoid as CTV that covered the right upper main bronchus and the segmental bronchi B1/B2 cranially, ventrally and to the right of the Visicoil marker. A margin of 5 mm in all directions to account for the respiratory motion, which was observed on planning CT, was added to create the PTV. A dose of 10 Gy × 3 fractions was prescribed to this PTV (65% isodose).Multiple static beams with energies of 6 MV were used Figure  and the At follow up in July 2011, a follow-up CT showed partial remission in the left lower lobe mass, which was then 1.5 cm maximum diameter, and no evidence of pneumonitis. A scar of mucosa in the area of in situ carcinoma, but no signs of new endobronchial manifestations were seen at bronchoscopy. No biopsies were performed. The patient reported no adverse advents of SBRT.The patient refused further follow-up. Nonetheless, he was admitted again to the hospital in April 2012 due to exacerbation of COPD. A chest CT Figure  showed nExternal beam radiation therapy for carcinoma in situ is also problematic, because these tumors are roentgenographically invisible. It is, therefore, a challenging task for the radiation oncologist to localize clinical target volume (CTV) in space and in time, since there is no gross tumor volume (GTV) be delineated. This inherent uncertainty of CTV must be balanced with the utilization of technological sophistication.Due to the high efficacy in the treatment of superficial lesions, high-dose brachytherapy could be a definitive treatment for carcinoma in situ or endobronchial occult lung cancer with limited invasion. Endobronchial ultrasonography has been reported to result in a change in management in 36% of tumors thought to be curable by endobronchial therapy after bronchoscopy .A recent large retrospective study  of 226 pCompared with conventionally planned radiotherapy, brachytherapy has the advantage of application of high-dose irradiation in tumor with rapid dose reduction outside the treatment volume. This favorable dosimetrical characteristic can be emulated by stereotactic guidance of external beam radiotherapy. The idea of use SBRT to deliver high-dose-rate-like hypofractionated radiotherapy came originally from Molla et al. , who sugA major issue for the treatment of these lesions with SBRT is, however, that high-dose delivery precision requires a higher degree of accuracy in target volume delineation and in patient set-up during the radiation treatment course. Radiographic markers are well known in the postoperative setting, where there is actually no GTV to be delineated, and are standard tools for image-guided radiotherapy (IGRT) and robotic radiosurgery, and can be used for making invisible lesions “visible” for guiding high-dose SBRT by target volume delineation and positional verification.In a prospective pilot series conducted by Malfiat et al.  to assesAnderson et al.  reportedEndoscopic Ultrasound-guided fiducials placement as an alternative method has been described by Charabaty-Pishvian et al. . The DopNevertheless, high-dose radiation treatment for centrally located tumors, particularly since the “bronchial exclusion zone” of RTOG 0236 is the main location of roentgenographically occult lung cancer, remains the main issue concerning the toxicity of SBRT. In addition to the fractionation scheme, central location of target lesions also appears to be a predictor of normal tissue toxicity. However, a recent review of 20 studies suggests that safe treatment of central tumors can be achieved with hypofractionated concepts with reduced single dose .The implantation of radiographic marker near or in the roentgenographically occult lung cancer appears to be safe and would allow precise delivery of hypofractionated SBRT to a target that is now radiologically visible and trackable in space and in time.Written informed consent was obtained from patient’s legal guardian 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.COPD: Chronic obstructive pulmonary disease; CT: Computer tomography; CIS: Carcinoma in situ; PET: Positron emission tomography; SBRT: Stereotactic body radiation therapy; GTV: Gross tumor volume; CTV: Clinical target volume; ITV: Internal target volume; PTV: Planning target volumeThe authors declared that they have no competing interests.FO participated in the radiation management of the patient, collected patient’s data, reviewed literature and drafted the manuscript. GK contributed the endoscopic interventions components of the manuscript. CK performed the radiation treatment planning, and dosimetrical measurement and calculations for this patients. DV contributed the radiotherapy components of the manuscript and revised the article. All authors performed critical review of the manuscript an approved the final manuscript.


Introduction. Glomus tumors (GTs) are benign neoplasm originating from the glomus body, commonly described in subungual region. The involvement abdominal is rare. Our aim is to describe a case of glomus tumor of the stomach that presented upper gastrointestinal bleeding. A 34-year-old woman was admitted with upper gastrointestinal bleeding and underwent an upper endoscopy that showed bleeding arising from an ulcerated lesion, treated by sclerosis therapy. A new endoscopy confirmed a submucosal lesion in upper portion of the stomach. During the laparotomy, a tumor at the upper anterior wall of gastric body was found and resected by a vertical gastrectomy. The pathological exam revealed hyperplastic smooth muscle fibers of the muscularis propria of the stomach wall, surrounded by hyaline stroma. The immunohistochemistry panel was positive for smooth muscle actin and type IV collagen, with low rate of mitosis studied by Ki-67 which allowed the final diagnosis of a gastric glomus tumor. Discussion. The majority of intraperitoneal glomus tumors occur in the stomach, and it is phenotypically similar to those localized in peripheral sites. Gastric GT generally is a benign tumor although it can be malignant and have the potential to metastasize. Conclusion. Even though gastric glomus tumor is rarely described, it should be considered as a possible cause of a major upper gastrointestinal bleeding. Glomus tumor (GT) is a rare mesenchymal neoplasm arising from glomus body a dermal arteriovenous shunt responsible for skin thermoregulation , 2. It iGT usually is a benign neoplasm, and the malignant variant is extremely rare with a few case reports in the literature , 4. SmolThe purpose of this paper is to present one patient who had a severe upper gastrointestinal bleeding due to a gastric GT treated successfully by a vertical gastrectomy, whose diagnosis was confirmed by immunohistochemical panel.A 34-year-old woman was admitted with a massive upper gastrointestinal bleeding. She was hemodynamically unstable with signs of hypovolemic shock for which she required resuscitation with intravenous fluids and blood. Physical examination showed that the patient was confused, pale, and with cyanosis of extremities, tachycardia, and 70 × 40 mmHg of blood pressure. Family members reported that she had been complaining of epigastric pain, nauseas, weakness, and five episodes of melena in the previous three days. On physical examination, the abdomen was soft, nondistended, without palpable masses but slightly painful to deep palpation in the epigastric region. The digital rectal examination confirmed the presence of melena, and the hemoglobin level was 5.8 g/dL. After initial fluid recovery of the patient and the administration of 3U of blood concentrate, she underwent an emergency upper endoscopy. The exam showed an active bleeding arising from an elevated and ulcerated lesion located in the upper portion of the stomach at great curve. The bleeding was successfully controlled by local sclerosis therapy. Four days later, the patient was subjected to another upper gastrointestinal endoscopy that revealed mild, diffuse oesophagitis, and a small sliding hiatal hernia. At the cranial portion of the gastric corpus, a 5 cm, well-circumscribed reddish submucosal mass was observed . The gasWith preoperative diagnosis of gastric leiomyoma, the patient was subsequently referred for elective surgical procedure. During the laparotomy, it was found a tumor measuring about 5 cm at its widest diameter, located in anterior gastric wall between the corpus and gastric fundus. The lesion infiltrated the gastric wall and compromised the serous layer where it was possible to observe large vascular proliferation . The tumThe histopathological findings were characteristic of GT of the stomach. The cut surface of the specimen demonstrated a grayish-white nodular tumor, arising from the submucosa of the stomach and extending throughout all layers of the muscular propria, involving the serous surface. Histologically, the tumor was composed of sheets of glomus cells, without nuclear pleomorphism and no mitotic figures. The cells had eosinophilic and focally clear cytoplasm. The nuclei of these cells are round, with inconspicuous nucleoli. The cells contours were clear, and it was possible to identify dilated blood vessels among the cell blocks and areas of stromal hyalinization . We coulHelicobacter pylori. Ten lymph nodes retrieved from the major omentum near the tumor were free of metastatic cells. The patient recovered uneventfully and was discharged 7 days after surgery. Currently, the patient is well 8 months after surgery, without signs of relapse or gastrointestinal bleeding.The immunohistochemical panel showed that the tumor cells were positive for smooth muscle actin and collagen type IV  and nega, but they have also been described in the bone and joints, skeletal muscle, soft tissue, thimpanus, mediastinum, trachea, kidney, uterus, and vagina [Gastric GT is a rare benign mesenchymal neoplasia arising from the neuromyoarterial glomus . The glod vagina . The majd vagina . Gastricd vagina .The first case of GT located in stomach is credited to Talijeva in 1928 , 14, andGT tumors usually arise in the intramuscular layer and typically occur as a solitary submucosal nodule that most frequently affects the greater curvature, antrum, and pylorus. Rarely, they are multiple lesions compromising the lesser curve, anterior, and posterior wall of the corpus of the stomach , 17, 18.The endoscopic biopsy is usually not helpful due to the intramural nature of the tumors. However, in the patient of this present paper, the removal of multiple fragments from a same site allowed the collection of neoplastic tissue from the interior of the mass leading to preoperative suspicion of a gastric leiomyoma. The endoscopic ultrasonography (EUS) usually show heterogeneous tumors between the submucosal and muscularis propria layer, and these aspects may be confused with malignant GIST or leiomyosarcoma, which is also represented by a heterogeneous tumor on EUS , 21. It Histologically, GT was well circumscribed located in gastric submucosa or muscularis and comprised of glomus cells surrounding capillaries. The glomus cells were small, uniform, and round without nuclear pleomorphism, mitotic figures, or necrosis. The stroma showed hyalinization or myxoid change in some patients, and sporadic mast cells could be seen inside of the stroma. Immunohistochemistry is essential in the differential diagnosis of GT, and the immunohistochemical panel generally showed that GT tumor was strongly and diffusely positive for smooth muscle actin, vimentin and actin, calponin, type IV collagen, and laminin. In the patient of this present paper, the immunohistochemical panel showed positivity for smooth muscle actin, actin, and type IV collagen. Other markers, including desmin, cytokeratin (AE1/AE3b), S-100 protein, creatine kinase, C-KIT (CD-117), CD34, DOG1 protein (K9), chromogranin A, p53 protein (DO-7) and neuron specific enolase, were negative. Although gastric GT is usually benign, malignant behavior cannot be excluded. The following classification criterion was proposed for malignant GT: deep location and size more than 2 cm; presence of atypical mitotic figure; combination of moderate to high nuclear grade and mitotic activity (5 mitoses/50 high-power fields) . NeverthGastrointestinal bleeding with hematemesis/melena and epigastric discomfort, like the patient of this paper, the most two common initial symptoms/signs and can be life-threatening or lead to chronic anemia. The patient currently reported that she had never experienced an episode of gastrointestinal bleeding before, despite reporting dyspeptic symptoms for several years, and needed to continue the use of omeprazole. Operative intervention should be carefully planned in cases of gastric GT. The majority of the patients reported in the literature were operated. As gastric GTs are mesenchymal tumors with potential malignant behavior, wedge resection with negative margins should be the treatment of choice . EnucleaThe choice of vertical gastrectomy was preferred by the team after the placement of surgical linear staplers around the lesion. As the location and extent of tumor resection with stapling wedge (wedge-resection) would result in significant gastric deformity with probable involvement of the organ and because vertical gastrectomy is a technique already realized and with good functional results, it was more appropriate.There are few data in literature on postoperative followup of gastric glomus tumors; as the recurrence of these tumors usually occur at the site of removal of the tumor and are rare, we recommend performing endoscopy annually.In conclusion, gastric GT is rare benign mesenchymal neoplasm and preoperative diagnosis is difficult. Since patients have no specific clinical and imaging findings, it is difficult to diagnose before operation. The differential diagnosis includes gastrointestinal stromal tumor, paraganglioma, and carcinoid tumor. The diagnostic gold standard for such lesions is the histological examination and the immunohistochemical markers. The correct approach of the patient optimizes the chances for an accurate preoperative diagnosis and leads to a targeted surgical or endoscopic intervention.


Congenital granular cell lesion (CGCL) or congenital epulis is an uncommon benign of the oral cavity tumor appearing at birth with typical clinical and pathologic features. It predominately affects females, mainly on the anterior maxillary alveolar ridge, and occurs usually as a single mass, although sometimes as multiple. The left side incisor area is the most common site. The etiology and histogenesis of the lesion remain obscure. Though it is a benign lesion, the tumor may cause feeding and respiratory problems if there are too large or multiple tumors. We report a case of a three-day-old, female newborn, who was referred to our hospital with multiple congenital oral swellings arising from the maxilla and mandible. The tumors caused a feeding problem and, hence, they were resected by surgical excision under general anesthesia. Congenital epulis (CE) is an uncommon benign tumor. This condition clinically appears as a protuberant mass in a round or ovoid shape, pedunculated, or sessile . The CE A female newborn was referred from a pediatric hospital for the treatment of soft tissue masses on the alveolar ridges of the maxillary and mandibular noticed at birth . She wasGrossly, in consistency, the masses were oval, creamy and firm and the external surface was slightly irregular. Microscopically, sections revealed neoplastic cells with round small nuclei and abundant granular cytoplasm. Among the tumoral cells, thin vascular plexus was seen. The external surface was covered by stratified squamous epithelium . This clThis benign tumor which appears rarely has various names such as congenital epulis (CE), congenital gingival granular cell tumor of the newborn , or NeumIn a study, in 2005, Olson, Marcus, and Zuker reported that CE is seen three times more frequently on the anterior alveolar ridge of the maxilla than from the mandible with a female to male ratio of 8 : 1 with a Caucasian predisposition . In anotPrenatal ultrasound evaluations have been used to report congenital epulis in sporadic cases after the 25th week of gestation, but the findings are nonspecific, and ultrasonic differential diagnoses, including congenital malformations and various benign and malignant intraoral tumors; however, it can help in the management of pregnancy, delivery, and postnatal management especially if the lesion is large . In our Treatment consists of CE removal by surgery under local or general anesthesia , 2. In aIt is important to stress that clinicians should know differential diagnoses of growths in the oral cavities of newborns, including hemangioma, lymphangioma, fibroma, granuloma, rhabdomyosarcoma, and osteogenic and chondrogenic sarcomas; schwannoma and heterotopic gastrointestinal cyst as the treatment modalities will be different for each case , 2. In tAlthough benign, it may obstruct the oral passage by its size and induce breathing and feeding problems. Delay in operation may cause airway obstruction and feeding difficulty. The tumor should be removed during the immediate postnatal period, as in our case.


A total laparoscopic gastrectomy was performed. In the second patient, GISTs were identified at the level of the fundus and the greater curvature of the stomach. A laparoscopic partial sleeve gastrectomy was performed. Both surgeries were successful with no complications or relapses at three to five years following surgery.Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from interstitial cells of Cajal or their stem cell-like precursors. Generally, GISTs have specific c-KIT gene mutations. The incidence of GISTs is estimated to be 10 to 20 cases/one million individuals, and GISTs typically affect people over 50 years of age. The majority of GISTs are solitary. However, multifocal GISTs have been observed, especially in children. We report on two unusual adult cases of double GISTs that were treated by laparoscopic surgery. The first patient presented a polypoid mass of the fundus and a second isolated smaller tumor in the posterior wall of the lesser curvature of the stomach. A histopathological examination confirmed that both tumors were GISTs and were  Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms of the gastrointestinal tract that account for approximately 0.1% to 3% of all gastrointestinal malignancies .c-Kit (CD 117) and less frequently the platelet-derived growth factor receptor-α (PDGFRA)[In most cases, GISTs originate from interstitial cells of Cajal, a complex cellular network that regulates gastrointestinal motility, or mesenchymal stem cells of the mesentery or omentum, which transform into the malignant phenotype that expresses the proto-oncogene  (PDGFRA),4.Small GISTs (less than 2 cm in diameter) are usually asymptomatic and are detected during investigations performed for other unrelated diseases. Conversely, GISTs that are greater than 2 cm are generally associated with clinical signs and symptoms, such as nausea, vomiting, abdominal pain, obstruction, abdominal mass, anemia and melena . DiagnosClinically, GISTs vary from virtually benign diseases to aggressive tumors (20% to 30% of cases) with a five-year survival rate that ranges from 35% to 65% depending on the tumor size, the mitotic index  and the tumor location ,3,6. OveKit tyrosine kinase inhibitor, imatinib mesylate , has resulted in significant improvements in non-resectable patients , and it has been successfully used as adjuvant therapy for tumors with a high risk of relapse [Complete surgical resection is the only curative treatment for GISTs . However relapse .We report two unusual cases of double GISTs of the stomach in two female adult patients.A 59-year-old female patient with a history of hypertension, breast cancer and type II diabetes visited her general practitioner because of a stomachache, dysphagia to solids, weight loss (approximately 13 kg), and general fatigue that began three months earlier. She was referred to a gastroenterologist for further examinations. An esophagogastroscopy was performed and indicated chronic gastritis of the antrum and a polypoid lesion of the fundus, which was suggestive of a GIST  and the lymph nodes were free of neoplasia (0/34). The postoperative course was uneventful, and the patient was discharged on the 14th postoperative day. Adjuvant therapy was proposed with Glivec® 400 mg/day for 12 months. After five years of follow-up, the patient is clinically and radiographically disease-free.The histopathological and immunohistochemical examinations revealed two isolated GISTs (7 × 6 × 6 cm and 2.5 × 1 × 1 cm) that were A 62-year-old female patient visited her general practitioner because of a stomachache, chronic diarrhea, occasional melena and weight loss (approximately 7 kg). An esophagogastroscopy was performed and revealed a polypoid lesion of the fundus, which was suggestive of a GIST. A CT scan was performed for diagnosis and staging. The CT confirmed the intramural lesion at the level of the fundus of the stomach (6 × 5 × 2.5 cm) and showed another extraluminal gastric mass that was located in the middle of the greater curvature (2.5 × 1.5 × 1 cm)  were free of neoplasia. The postoperative course was uneventful, and the patient was discharged on the sixth postoperative day. Adjuvant therapy was proposed with Glivec® 400 mg/day for 12 months. After three years of follow-up, the patient is clinically and radiographically disease-free.The histopathological and immunohistochemical examinations revealed two isolated GISTs (5.5 × 5 × 2.5 cm and 2 × 1 × 1 cm) that were GISTs are rare neoplasms of the gastrointestinal tract, which occur mostly as solitary tumors ,6. We rekit mutations in the individual lesions suggested the presence of distinct subsets of gastric GISTs of an unknown etiology. As reported in the literature, the majority (approximately 79%) of cases of multifocal and closely associated GISTs that present c-kit+ and CD34+ phenotypes are initiated by different types of somatic KIT exon 9 and 11 mutations [Both our case patients had no history of familial GISTs, no diagnoses of GIST-related district syndromes, or any signs of metastatic spreading from a single primary GIST. Moreover, the patterns that were detected in the histopathological and immunohistochemical analyses supported the diagnosis of double sporadic GISTs. Particularly, the synchronous location in the proximal stomach, the close proximity and the demonstration of different Surgical treatment is indicated in the case of a non-metastatic GIST >2 cm, and the standard primary treatment is complete resection with recommended 1-cm negative margins without tumor rupture. Microscopic negative margins are sufficient to ensure R0 resection ,8,12. ThRadiographic assessment is a prerequisite for correct surgical planning. In the initial investigation, esophagogastroscopy and trans-abdominal ultrasonography are undertaken. Then, in patients with gastric resectable GISTs, EUS-FNA and a CT scan with a “stomach protocol”  are perfIn Case Report 1, we performed different diagnostic imaging techniques; however, only the larger GIST was correctly detected. Most likely due to its small size, the second GIST was not identified even by EUS-FNA. During surgery, we performed intra-operative laparoscopic ultrasound to evaluate the relationship between the tumor and the cardias. This procedure indicated the presence of another small lesion in the posterior wall close to the cardias. The discovery of a second tumor and the proximal location (<1 cm) from the gastro-esophageal (GE) junction required a total laparoscopic gastrectomy to guarantee an en-block excision with R0 resection margins and prevent stenosis of the GE junction. Moreover, we performed an extensive lymphadenectomy, even though this procedure is not recommended, because we did not know peri-operatively the nature of the second tumor. In this patient, the anatomical characteristics of the double GISTs determined the surgical choice, which led to a more invasive surgery that was counterbalanced by the achievement of the oncological outcomes.In Case Report 2, the correct detection of both GISTs pre-operatively and their location at the greater curvature allowed us to perform a laparoscopic sleeve gastrectomy, which is less invasive and associated with a shorter recovery.In both patients, the laparoscopic approach was selected regardless of the tumor size and the tumor location. The patient positioning and the location of the trocars were similar to those used in laparoscopic surgeries for early gastric cancers and Toupet fundoplication. The location of the trocars was adjusted to the left and proximally for lesions of the fundus of the stomach and distally when lesions were located toward the antrum. Before initiating the resection, we systematically performed an intra-operative laparoscopic ultrasound to better locate small lesions. If needed, intra-operative endoscopy can be used to support the surgical excision.In conclusion, this case report presents the surgical treatment of two unusual double gastric GISTs in adult patients. The laparoscopic approach was confirmed as a safe and effective technique, regardless of the size and the location of the tumor, when performed by skilled surgeons who work in a high-volume center.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.CT: Computed tomography scanning; EUS-FNA: Echoendoscopy with fine needle aspiration; GE: Gastro-esophageal; GIST: Gastrointestinal stromal tumors; MRI: Magnetic resonance imaging; PDGFRA: Platelet-derived growth factor receptor-α.The authors report no conflicts of interest. This study received no funding or financial support.NdeA, FB and RM contributed to the study conception, the data analysis, the data interpretation, and the drafting and final revisions of the manuscript. DM and VZ contributed to the data collection, the data analysis and the drafting of the manuscript. DA contributed to the study conception and the final revision of the manuscript. All of the authors approved the final version of the manuscript.


Clival chordomas present with headache, commonly VI cranial nerve palsy or sometimes with lower cranial nerve involvement. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying chordoma-induced skull base erosion.A 60-year old Caucasian woman presented with meningitis secondary to cerebrospinal fluid rhinorrhoea. At first, radiological imaging did not reveal a tumoral condition, though intraoperative exploration and tissue histology revealed a chordoma which eroded her clivus and had a transdural extension.Patients who present with meningitis and cerebrospinal fluid rhinorrhoea could have an underlying erosive lesion which can sometimes be missed on initial radiological examination. Surgical exploration allows collecting suspicious tissue for histological diagnosis which is important for the actual treatment. A revision endoscopic excision of a clival chordoma is challenging and has been highlighted in this report. Chordomas are rare, slow-growing malignant bony tumors that arise from remnants of the notocord and account for approximately 1% of intracranial tumors. While tCerebrospinal fluid (CSF) rhinorrhea has been very rarely described as a presenting symptom,10. We pA 60-year-old Caucasian woman, living alone and previously in good health, was found unconscious at home and fortunately helped by her friends and the emergency medical team. One week before this incident, she had infrequent episodes of watery nasal discharge that increased with forced sneezing and cough. On her arrival to our clinic, she was confused and febrile. A cranial nerve examination was normal. A computed tomography (CT) scan Figure  of her ben bloc excision of the chordoma was done, anticipating her basilar artery to be just posterior to the tumor. Reconstruction of the defect was performed using fascia lata, fat and fibrin glue. A lumbar drain was inserted preoperatively and used to drain CSF in the postoperative period. It was removed on the fifth postoperative day. Broad spectrum antibiotics (ceftazidime 2g three times a day) were given for 10 days. Our patient had an excellent recovery with no complications. The follow-up scans showed complete excision of the tumor and no other adjuvant therapy was instituted. Our patient was discharged on the 12th postoperative day and a systematic clinico-radiological follow-up is planned. She was totally asymptomatic at the latest follow-up (three months after surgery) with an MRI  used for the CSF leak closure and skull base reconstruction during the prior surgery. During such revision explorations, it is of paramount importance to define the tumoral soft tissue and bone erosion limits, followed by identification of the important landmarks within the sphenoid sinus. Neuronavigation helps in identifying vital anatomical structures at the anterior skull base and avoids intraoperative complications, thereby facilitating a complete tumor removal. The two nostrils - four hands technique facilitates better transnasal instrumentation allowing a complete tumor excision and efficient reconstruction of the skull base defect.The role of radiation therapy has been extremely controversial in the treatment of clival chordomas. Conventional radiation does not appear to have an effect on survival in the study published by Colli and Al-Mefty. ResultsPatients with a clival chordoma commonly present with headaches, visual changes, cranial nerve palsies and, rarely, with CSF rhinorrhea and epistaxis. Patients who present with meningitis secondary to a CSF leak may have an underlying erosive lesion that can be missed on initial radiological examination. A revision transnasal endoscopic excision of a clival chordoma is challenging and warrants collaborative efforts between the ear, nose and throat surgeons and neurosurgeons.Written informed consent was obtained from the patient for publication of this manuscript 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.JA - collection of data, manuscript writing. PM - analysis and editing. RTD - analysis and editing the manuscript. KS - collection of data, manuscript writing, analysis and editing the manuscript. KS was the major contributor in writing the manuscript. All authors read and approved the final manuscript.


Primary lung cancer is extremely rare in children, while secondary malignancies reportedly develop in 2% to 3% of pediatric osteosarcoma survivors.A 14-year-old girl was found to have two pulmonary lesions on computed tomography. These tumors had developed 1 year after osteosarcoma surgery. Segmentectomy of right segment 1 and wedge resection of right segment 9 were performed. Both lesions were completely resected and postoperative histopathological examination revealed metastasis of osteosarcoma and bronchioloalveolar carcinoma, respectively.Bronchioloalveolar carcinoma may present as a solitary pulmonary lesion indistinguishable from a metastatic lesion and should be included in the differential diagnosis of pulmonary lesions in survivors of pediatric cancer. Thus, pulmonary lesions identified in these patients should be biopsied or resected to establish a histological diagnosis. Osteosarcoma accounts for approximately 3% of all pediatric malignancies. With current multi-modal approaches, 70% of patients can achieve long-term survival . The lunA 14-year-old girl was found to have an abnormal shadow on a chest X-ray during postoperative follow-up for osteosarcoma of the upper end of the left tibia Figure B. PositrAs to the past history of this patient, from January 2010 to June 2010, under a diagnosis of osteosarcoma of the left tibia, she had received chemotherapy, including cisplatin + pirarubicin, high dose methotrexate, and high dose ifosfamide. Then, wide resection of the upper end of the left tibia was performed in July 2010. Although chest CT at that time showed no tumor in the right S1, a tiny and extremely faint shadow was observed in the right S9  [et al. reviewed 509 patients with osteosarcoma and documented second malignant neoplasms in 14 (2.7%) [et al. described 26 (2.2%) who had developed a second malignant neoplasm [Although the overall incidence of second malignant neoplasms in survivors of pediatric osteosarcomas is relatively low, it is higher compared with the expected incidence of malignant neoplasm in the general population -14. Inst4 (2.7%) . No case4 (2.7%) ,14. In aneoplasm . Two of neoplasm ,15.Case reports on primary lung cancer arising in survivors of pediatric osteosarcoma are shown in Table et al. reported the 5-year survival rate for stage I pulmonary lung cancer in young patients to be 70% which is not quite different from that for lung cancer in elderly patients, and that the survival rate depends on disease stage rather than age [Icard than age . Thus, sIn conclusion, we have described a very rare BAC in a 14-year-old girl with recurrent osteosarcoma. It is interesting that most of these rare pediatric BAC have occurred in children who had another malignant tumor, raising the possibility of a link between these entities. Additionally, our present patient illustrates the importance of establishing a histopathological diagnosis for suspected pulmonary metastases in children with known primary malignant tumors.Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.BAC: Bronchioloalveolar carcinoma; CT: Computed tomography; FDG: Fluorodeoxyglucose; GGO: Ground glass opacity; PET: Positron emission tomography; S1: Segment 1; S9: Segment 9.The authors declare that they have no competing interests.MO and TG wrote the manuscript. MO and TG performed surgery. YH carried out the pathological examination. RN and MK were involved in the final editing. All authors approved the final manuscript.


Umbilical metastasis, also called Sister Mary Joseph’s nodule (SMJN), is defined as the umbilical nodule associated with advanced metastatic intra-abdominal and pelvic malignancies. A patient with umbilical metastasis has been deemed to have a poor prognosis. Rectal cancer presenting with a SMJN is a rare phenomenon, especially in the early stage and in middle-low rectal cancer.We report a case of a 70-year-old male presenting with umbilical metastasis derived from rectal cancer .For rectal cancer with umbilical metastasis, the exact metastatic routes as well as the criterion of diagnosis and treatments are not very clear. Here we review the literature on rectal cancer and SMJN to deepen the understanding of this disease. Umbilical metastasis, also called Sister Mary Joseph’s nodule (SMJN), is mostly associated with advanced metastatic intra-abdominal and pelvic malignancies . In receIn October 2012, a 70-year-old male was admitted to our department complaining of a 5-month history of abdominal pain, weight loss and constipation. Physical examination revealed a hard, craggy, painful, subcutaneous lump, measuring 4.0 × 4.0 cm in the umbilical area of the abdomen. His blood tests are normal except for a markedly elevated carcinoembryonic antigen level (5.4 ng/ml). Epigastrium computed tomography scan showed an ill-defined mass measuring 3.0 × 4.0 cm in diameter in the umbilical area Figure  and a chRectal cancer is one of the most common malignancies around the world, and has an improved prognosis due to the development of diagnostic and therapeutic procedures . HoweverUmbilical metastasis is also called SMJN, which is defined as a firm umbilical nodule associated with advanced metastatic intra-abdominal and pelvic malignancies. In 1949, Sir Hamilton Bailey first used the name SMJN to describe this umbilical metastatic nodule    could be the best alternative for diagnosis of umbilical nodules, as it has been proved a simple, inexpensive, and reliable technique in making the diagnosis of this mass . The treAnomalous metastasis is not rare in some malignancies, such as colorectal cancer. Umbilical metastasis from gastrointestinal adenocarcinoma has been reported in many cases, but it is very rare for middle-low rectal cancer. Our report described a case of umbilical metastasis from the early stage rectal cancer , and we felt regret that the patient did not accept our treatment recommendations. The differential diagnosis of an umbilical lesion should always include metastatic disease apart from benign lesions and primary neoplasms, and FNAC could give us great help to diagnose the lesion. Surgical treatment in umbilical metastasis from gastrointestinal adenocarcinoma is still controversial, but we suggest that surgery and systemic chemotherapy are both necessary for the treatment. Bear in mind that, before surgery, laparoscopic exploration can avoid unnecessary damage.Written informed consent for publication of this case report and the associated images were obtained from the patient.FNAC: fine needle aspiration cytology; SMJN: Sister Mary Joseph’s nodule.The authors declare that they have no competing interests.ZZ is the first author; XY and JH are the corresponding author of the manuscript. XY and JW collected the patient’s data and provided figures. ZZ, XY and JW were involved in drafting and revising the manuscript. All authors read and approved the final manuscript.


Epididymal leiomyosarcoma is very uncommon. Only 16 cases have been reported in the literature. There has never been a reported case of a patient with an African origin with this tumor. We report the medical history of a 70-year-old man who presented an enormous mass located in his epididymis. A tricut biopsy was performed which allowed histological confirmation of epididymal leiomyosarcoma after which the patient underwent excision of the mass. The patient died after the first round of chemotherapy. Leiomyosarcoma is a tumor that can arise from any organ that contains smooth muscles. Its development in the epididymus is rare. Early diagnostics of this tumor have proven to be of good prognosis as many studies have showed. Surgical resection of the tumor associated with adjuvant chemotherapy seems efficient in its treatment. We report a case of a 70-year-old man who was diagnosed of leiomyosarcoma of the epididymis treated by excision of the mass followed by the 1st round of chemotherapy before his death.A 70-year-old Moroccan male with no medical history, presented with a scrotal mass located in the left testicle and along the spermatic cord which increased in volume gradually. Medical examination showed a patient with an altered general state and an ulcerovegetant mass with its largest diameter measuring 25 cm associated with an enlarged fistulised inguinal lymph node . The dosage of tumor markers  was negative. A tricut biopsy was realized followed by histological study of the masss which confirmed an epididymal leiomyosarcoma . The patient underwent excision of the mass followed by the first round of chemotherapy, and he passed away after a month.Leiomyosarcoma of paratesticular tissues is very uncommon. It arises from smooth muscles which form the muscles of the scrotal sac, the seminiferous tubules, and the vascular tissues of the spermatic cord. Its principal location is in the testicular walls and the spermatic cord .Epididymal leiomyosarcoma occurs in men with ages between 50 and 80 years. It may also occur in children as there has been a reported case in a 6-year-old child . Certain predisposing factors have been described in the literature leading to testicular leiomyosarcoma such as exposure to large doses of anabolising steroids , chronicThere are no reported predisposing factors leading to epididymal leiomyosarcoma with respect to its prognosis in the literature. It is difficult to define aggressive forms of this tumor due to its relatively rare occurrence; however, its aggressivity seems less important with respect to its location, as there has only been a single case reported of recurrence and of metastasis. The lack of a universal histological classification contributes to the difficulty in determining histological characters that influence the prognosis of this tumor. The only character described frequently is the mitotic index which is often elevated. Nevertheless, it seems not to help predict the agressivity of the tumor as well as its prognosis as it is not strictly related to the recurrence of the tumor nor the occurrence of metastasis .Histological studies of the mass biopsied showed elevated mitotic index.Immunohistochemical studies showed differentiated smooth muscle cells that tested positive for actin, desmin, and caldesmon. The diagnosis of leiomyosarcoma was made upon these findings.Most authors prefer treatment by orchidectomy only . An adjuSome authors reported that an adjuvant therapy may be performed in cases where there exist elevated mitotic index. However, it is difficult to determine the benefits of this complementary treatment as few cases have been reported in the literature .The epididymis represents a rare location for leiomyosarcoma although its histological characteristics are the same as that found in other organs.There is no morphological criteria that determine its prognosis (relating to its aggressivity) as well as an anatomo-pathological classification that will help to grade this tumor.In these present times, information from the literature shows that radical orchidectomy seems to be the underlying treatment for this tumor. There is no proven efficacy for any adjuvant therapy.


Introduction. Intravenous leiomyomatosis with cardiac extension is an extremely rare uterine tumor. We report here a case of intravenous leiomyoma extending to the right atrium, diagnosed in a patient having leiomyoma. Case Presentation. A 39-year-old woman with no symptoms and a past medical history of two myomectomy operations (7 and 3 years previously) was admitted to our clinic for routine control. We detected a uterine fibroid of 8 centimeters and 4 small solid masses of 1-2 centimeters near the uterus and ovaries at vaginal ultrasonography. Computed tomography (CT) was performed to investigate the abdominal cavity. It revealed a mass originating from the left common iliac vein, which invaded the inferior vena cava (IVC) and extended to the right atrium in addition to the uterine fibroids and pelvic masses. The operation was performed with a combined team of gynecologists and cardiac surgeons and a one-stage operation was accomplished. The postoperative course was uneventful. Conclusion. Abdominal CT is a useful imaging technique for the diagnosis of unusual pathology in a patient with uterine fibroid having suspicious pelvic masses. Also, when a right atrial mass is identified in a female with a prior history of hysterectomy because of leiomyoma or in whom there is a uterine myoma, then intravenous leiomyomatosis should be considered. Intravenous leiomyomatosis (IVL) is a histologically benign, rare smooth muscle tumor derived from either a uterine venous wall or uterine leiomyoma . Althoug We present an unusual case of intravenous leiomyoma arising from the left common iliac vein and extending to the inferior vena cava and right atrium with extensive intracaval attachment. We include a brief review of the literature. A 39-year-old woman with no symptoms and a past medical history of two myomectomy operations (7 and 3 years previously) was admitted to our clinic for routine control. She had 2 normal deliveries. We detected a uterine fibroid of 8 centimeters and 4 small solid masses of 1-2 centimeters near the uterus and ovaries at vaginal ultrasonography.  Computed tomography (CT) was performed to investigate the abdominal cavity. It revealed a mass originating from the left common iliac vein, which invaded the inferior vena cava (IVC) and extended to the right atrium in addition to the uterine fibroids and pelvic masses . Tumor m The operation was performed with a combined team of gynecologists and cardiac surgeons, and a one-stage operation was accomplished. The surgical approach was made through median sternotomy and laparotomy. The abdomen was explored via a median laparotomy, and a large soft intraligamentary fibroid was seen in the left site of the uterus. There are small retroperitoneal solid masses of different sizes. Also a solid mass were easily palpated within the left common iliac vein and the inferior vena cava. At first, hysterectomy and bilateral salpingo-oophorectomy was performed and all pelvic masses removed out easily. Then, external circulation by cardiopulmonary bypass was instituted to permit cardiac access. Following the institution of deep hypothermia and total circulatory arrest, intracardiac and intravenous masses were removed out by cardiac surgeons .The postoperative course was uneventful. The patient was discharged on the fourth postoperative day. Pathological examination of all the masses revealed to be composed of benign smooth muscle cells with fibrous tissue consistent with leiomyoma. There were no sarcomatous changes. Followup of the patient in the 4th month revealed an excellent patency of vascular structures and no recurrence of tumor at the control computed tomography scan . IVL is a condition that only affects women, the majority of whom have undergone a previous hysterectomy due to uterine leiomyoma. A review of the cases reported in the literature demonstrates that 64% of the women had undergone a previous hysterectomy, with a range of 6 months to 20 years before presentation with the intravenous portion of the tumor . Our pat Two theories have been proposed to explain the origin of IVL , 11. OneUsually, right atrial mass is detected firstly in patients with cardiac symptoms. Then IVL is diagnosed by echocardiography, CT, and MRI . In mostA successful therapy for IVL is dependent on the total surgical excision of the tumor. In the first total resection, reported in 1982 by Ariza et al., there was a delayed laparotomy after resection of the intracardiac portion of the tumor . RecentlAbdominal CT is a useful imaging technique for the diagnosis of unusual pathology in a patient with uterine fibroid having suspicious pelvic masses. Also, when a right atrial mass is detected in a female with a prior history of hysterectomy because of leiomyoma or in whom there is a uterine myoma, then IVL should be considered.


Vascular abnormality of the intestine is rare, except angiodysplasia. We report on an unusual case of atypical florid vascular proliferations of the appendix. A 41-year old male presented with melena. Adhesioned blood clots in the appendiceal orifice were observed by colonoscopy. He underwent laparoscopic appendectomy. Microscopically, a tiny exophytic polypoid mass was observed. The mass showed pyogenic granuloma-like features in the superficial portion and infiltrative florid vascular proliferations in the deeper portion. Endothelial cells showed minimal nuclear atypia, and mitotic figures were observed infrequently and showed positivity for CD31 and CD34 and negativity for HHV-8. Differential diagnoses include from benign vascular tumor to angiosarcoma or Kaposi’s sarcoma, but this lesion does not fit the description of any defined vascular entity. We diagnosed atypical florid vascular proliferations and the patient has been well during the five-month postoperative follow-up.http://www.diagnosticpathology.diagnomx.eu/vs/1386921325843104The virtual slide(s) for this article can be found here:  Vascular proliferative lesions occurring in the gastrointestinal tract include tumors and tumor-like lesions of the vessels. With the exception of angiodysplasia, vascular abnormalities of the gastrointestinal tract are uncommon . Seven cA 41-year old male presented with melena. He had no other symptoms, such as migratory pain, right lower quadrant abdominal tenderness with guarding, or leukocytosis. Colonoscopy showed non-specific findings, except for adhesioned blood clots in the appendiceal orifice. He underwent laparoscopic appendectomy. No characteristic features were observed on the external surface. Cut sections showed a tiny polypoid mucosa with easy contact bleeding and the appendiceal lumen was filled with blood. Microscopically, inflammatory cells infiltration, diverticulum, parasite, and fecalith were not observed. An exophytic polypoid mass with extensive surface ulceration was observed. The superficial portion of the polyp showed pyogenic granuloma-like features and the deeper portion was composed of vaguely lobulated proliferation of closely packed small capillary-sized vessels and showed infiltrative growth and extension into muscularis propria (Figure In our review of the English literature, vascular tumors or vascular lesions occurring in the appendix have been rarely reported -9. This Differential diagnoses include from reactive response to preceding appendicitis, reactive vascular lesion, benign to malignant vascular tumor. However, this lesion did not fit the description of any defined benign vascular tumor entity. For reactive response to preceding appendicitis, he had no previous history of appendicitis and there were no microscopic features of acute or chronic appendicitis. The florid benign vascular proliferations occurring in the gastrointestinal tract have been reported and may be confused with angiosarcoma -3. The lAngiosarcomas account for less than 1% of all soft tissue sarcomas ,14. PrimIn summary, based on the histologic and clinical findings, we think that this was case of atypical florid vascular proliferatons, rather than angiosarcoma. Consideration of this lesion may helpful in to prevention of misdiagnosis.Written informed consent was obtained from the patient described in this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in Chief of this journal.The authors declare that they have no competing interests.MJG designed the study, drafted the manuscript and approved of the version to be published. SHK collected clinical data. JHC revised for important intellectual content. All authors read and approved the final manuscript.


Choristoma is development of a normal tissue in an aberrant location. This report describes jejunal salivary choristoma (JSC) causing recurring episodes of abdominal discomfort in a 5-year-old girl. Exploratory laporatomy revealed a pale yellow subserosal jejunal lesion. Wedge resection of the lesion and repair of the bowel were performed. The child did well postoperatively and has since that time been free of pain at follow-up. Histopathological examination of the resected lesion revealed salivary gland choriostoma. Literature review (PUBMED search engine) revealed no previous report of this rare clinicopathologic entity. We conclude that choriostoma should be considered a possible differential when evaluating abdominal complaint in children. Abdominal pain is a common problem in children . The preChoriostoma is a form of heterotopia in which there is development of a normal tissue in an aberrant location . The tisSalivary choriostoma is defined as a tumor - like growth of otherwise normal salivary gland found in an abnormal location. We present the ordeals and the difficulties of management of a young Nigerian girl who had salivary gland choriostoma in the jejunum presenting as a diagnostic dilemma.A 5-year-old female Nigerian child who was referred to our surgical facility on account of recurring abdominal pain. The pain was localized to the central abdomen, being colicky, aggravated by feeding and relieved occasionally by taking antacids. The episodes were frequent but erratic with the colics occurring mostly within 15 to 30 minutes of feeding; it was usually severe enough to stop her activities during the episode of the pain but she was a normal cheerful child in between the colics. She had occasional postprandial vomiting which sometimes relieved the symptoms. The vomitus contained only recently ingested meal and it did not contain blood. There was neither abdominal distention nor constipation. No history of diarrhoea, passage of worms, or haematochezia was found. There were neither fever, weight loss, nor anorexia. There was no groin swelling but she had a small umbilical protrusion which had been present since birth and had not changed in size even during the episodes of abdominal pain.The frequent episodes either kept her away from school totally or prevented her from participating in the school activities. Before the onset of the episodic abdominal pains, she had never been operated upon, she had not been admitted to the hospital, and she had not been diagnosed of any chronic illness. Her genotype is AA. Both parents were anxious and they had taken her for review by several physicians on separate occasions including general surgeons and a paediatric surgeon.On examination by all the physicians she visited, the only consistent positive abdominal finding was umbilical facial defect measuring 0.5 × 0.5 cm which was considered nonsignificant; otherwise she was a normal, well-nourished child. She was diagnosed as having mesenteric adenitis, gastroenteritis, helminthiasis, and peptic ulcer diseases at one time or another and had treatment for these varying diagnoses to no avail.After several reviews and treatment with no finding of any organic disease, the assessment became non-specific abdominal pain with possibility of a child who is seeking attention. Psychotherapy was employed with no improvement in the clinical condition. Because of persistence of the symptoms, she had repair of the umbilical facial defect without abdominal exploration because this was the only possible diagnosis that had not been treated and could not be treated nonoperatively.About two weeks after the repair of the umbilical defect, the symptoms resumed but now with the addition of hyperactive bowel sounds this led to a consideration of postoperative adhesions which further heightened the anxiety of the parents. Further evaluation with plain abdominal X-ray, abdominal ultrasound, gastrografin meal and follow-through showed no abnormalities. Upper gastrointestinal endoscopy and diagnostic laparoscopy were considered but were not done because there was no paediatric scope and there was no expertise for diagnostic laparoscopy at the time. Abdominal computer tomography was not done because the parents could not afford its cost. Eventually, a diagnostic exploratory laparotomy was done because of the continuing psychosocial and physical trauma suffered by the parents and the child.At surgery, the bowel was examined from the duodeno jejunal junction to ileocecal junction. A pale yellow subserosal lesion was seen in the antimesenteric border of the jejunum about 45 cm from the duodeno-jejunal junction. There were enlarged mesenteric lymph nodes but there were neither adhesion nor other abnormalities. A wedge resection of the lesion was carried out and the bowel was repaired in a single layer using long-term absorbable sutures. The enlarged mesenteric nodes were also biopsied. She did well and was discharged home on the 6th post-operative day. The patient has been followed up serially in the clinic at one month, three months, six months, and a year after the procedure. She has been free of symptoms and has since that time been regular and participating fully in school activities.The specimen was a jejunal specimen measuring 1.9 × 1.2 cm with a grayish white mass measuring 0.9 × 0.6 cm and cut surface shows homogeneous tissue. Also a lymph node is included measuring 1.1 × 0.5 cm with a grayish white cut surface. Microscopic appearance shows a sub-mucosal tissue with lobules of serous glands with central lumen, reminiscent of salivary glands . The glaA choriostoma is a rare congenital tumor-like lesion reported in human beings and other animal species consisting of histologically normal mature tissue located in an anatomic position different from its proper site . EctopicDiagnosis and treatment of childhood acute abdominal pain can be straight forward if it has an organic cause and there is obvious need to intervene surgically. However, the scenario is usually complex when one cannot convincingly diagnose an organic cause clinically and radiologically and when patients have subacute presentation in which case it is referred to as non-specific abdominal pain. If indeed the cause of the pain is organic, the consideration of non-specific pain delays further investigations to reach a definitive diagnosis and treatment. This was clearly demonstrated in the case presented in this report, having consulted many physicians including general surgeons and a paediatric surgeon for a period of one year before presentation in our facility. The postprandial abdominal pain and the hyperactive bowel sound could be due to secretion of saliva by the glands in response to meal ingestion. This may increase the size of the gland, thereby causing some degree of luminal obstruction.Most conditions diagnosed as non-specific pain in children eventually resolve with nonoperative management; however, if the symptoms persist and the psychosocial burden culminates as demonstrated in this case, the diagnosis may need to be reconsidered early and more aggressive steps in investigations or interventions may be required. Diagnostic laparoscopy would have been helpful in the index case because it could have resolved the problems with lesser risk of later adhesions. Theoretically, the diagnosis of a choriostoma might be reached pre-operatively using ultrasonographic techniques, computed tomography, or magnetic resonance imaging, but preoperative diagnosis of choriostoma is rarely possible regardless of improvements in diagnostic tools and techniques . In situAnother important lesson learnt from this presentation is that if the diagnosis is unclear and the decision is taken to operate on the child, then a full and thorough exploration should be carried out. If this had been done on the occasion of repair of the umbilical hernia, the diagnosis would have been reached and appropriate treatment could have been offered with possible complete resolution of the symptoms without a need for a second surgical intervention.A delay in diagnosis and intervention may lead to the progression from partial to complete intestinal obstruction, ulceration with bleeding, or even malignant transformation. The malignant potential of such an ectopic tissue in such an unusual location cannot be overemphasized.Generally, the prognosis of choriostoma depends on the location of the choriostoma. It is favourable in the case of a subcutaneous location because it can easily be removed surgically. A more extensive surgery may be required for lesions in the viscera when it becomes symptomatic or complicated.We do not think that in a child with abdominal symptoms choriostoma will be considered a clinical diagnosis in the early periods of evaluation, neither do we think it is necessary to advocate that it should be at the top or near the top of the differentials. However, clinicians should be aware that this entity exists and it is worthy of consideration in a child presenting with bizarre abdominal complaint especially when all that seems to be common causes of the symptom pattern have been considered and ruled out.


We describe an example of reactive pituitary hyperplasia from primary hypothyroidism that mimicked a pituitary macroadenoma in a child.a 10 year old boy presented with headache and statural growth arrest. MRI revealed an intrasellar and suprasellar pituitary mass. Endocrine evaluation revealed primary hypothyroidism.the patient was started on levothyroxine with resolution of the mass effect.primary hypothyroidism should be considered in the differential diagnosis of solid mass lesions of the pituitary gland. Examination of thyroid function in patients with sellar and suprasellar masses revealed by MRI may avoid unnecessary operations which can cause irreversible complications. There are many causes of sellar and suprasellar mass, and pituitary enlargment secondary to primary hypothyroidism has been previously described -5. It reth percentile to the 25th, while his weight had passed from the 50th to the 75th percentile in the last year. He did not complain of vomiting, tiredness or any other symptoms.A 10 year old boy, born at term in Morocco from non-consanguineous parents after an uncomplicated pregnancy, presented at his local hospital with occipital headache over the last three months and height growth arrest: during the previous 2 years he had grown 1 cm, declining from the 75-90th percentile), weight 42 kg (75th percentile), prepubertal status, blood pressure 120/67 mmHg, pulse rate 72 bpm, and non-palpable thyroid gland. Because of persistent headache he was referred to the neurologist at his local hospital and the examination was normal, as were the fundus oculi and visual fields.His past and familial history was unremarkable and his cognitive development normal. On physical examination, he presented with height 136 cm  was performed to rule out an organic lesion and an intrasellar and suprasellar pituitary mass was detected. The enlargment was isointense to gray matter, extended on the suprasellar cistern with mild compression of the optic chiasm. The pituitary stalk and posterior pituitary were dislocated. After gadolinium the mass homogeneously enhanced Figure .After endocrine consultation, blood tests were done: full blood count, erythrocyte sedimentation rate, renal and liver function and anti-coeliac screening wereth percentile and he was no longer complaining of headache. A follow-up MRI scan 5 months after levothyroxine replacement showed resolution of the mass effect  thyroiditis was made and the pituitary mass was attributed to secondary hyperplasia. The patient was started on levothyroxine at 50 ug/day. IGF-I and thyroid function had returned to the normal range after 3 months Table . Height t Figure .Due to the loss of thyroxine feedback inhibition and the subsequent overproduction of thyrotropin-releasing hormone (TRH), long-standing hypothyroidism results in hyperplasia of the thyrotroph cells and subsequent enlargement of the pituitary gland . The incChildren with pituitary hyperplasia and primary hypothyroidism rarely present with neurological symptoms secondary to sellar expansion 3%). Their complaints at presentation are associated with hypothyroidism and abnormal puberty . Most ch%. Their Despite recent progress in imaging, it is still difficult to distinguish between a pituitary adenoma and hyperplasia, even using MRI with gadolinium injection. On imaging studies, secondary pituitary hyperplasia is often characterized by a homogeneously enhancing lesion that may progress rapidly following the onset of a hypothyroid state . InterprThyroid hormone replacement therapy led to a decrease in the size of the gland in 85% of patients with pituitary enlargement who underwent follow-up MR examinations . In our Primary hypothyroidism should be considered in the differential diagnosis of solid mass lesions of the pituitary gland, particularly in children with short stature. Examination of thyroid function in patients with sellar and suprasellar masses revealed by MR imaging may avoid unnecessary operations.The authors declare that they have no competing interests.RF performed the diagnosis and has been involved in drafting the manuscript. UR carried out the MRI. RFA partecipated in the MRI analysis. MB contributed to the patient endocrine follow-up. ADP has been involved in critically revising the manuscript and has given final approval of the version to be published.All authors read and approved the final manuscript.Written informed consent was obtained from the patient's relatives 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.


Diagnosis forms the backbone of treatment planning. Accurate diagnosis is essential to initiate the appropriate treatment at the apt time. Diagnosis involves eliciting the signs and symptoms of the patient and their accurate interpretations. The subtle signs that can go unnoticed lead to misdiagnosis and subsequent agony to the patient. Alertness on part of the clinician is important to avoid this error. Reported in this paper are four cases that were wrongly diagnosed either due to lack of clinical experience or due to omission of careful clinical, radiographic, and histopathological examinations. A misdiagnosis is, simply put, a wrong diagnosis. An erroneous diagnosis can take a number of forms, from a missed diagnosis in which no medical problem is identified when a problem exists, to a diagnosis which later turns out to be wrong, such as classifying a benign tumour as malignant. Misdiagnosis is a form of medical error, and while it is difficult to get accurate statistics on wrong diagnoses, some statistics place the rate at around one to two percent, with varying consequences.There are a number of reasons for a misdiagnosis to occur. People who have suffered as a result of a misdiagnosis are often tempted to blame lazy doctors or medical personnel, but all kinds of things can be involved, including malfunctioning medical equipment, a patient's decision to conceal information, a language barrier between doctor and patient, inexperience on the part of the doctor, or a situation in which a diagnosis is extremely unusual, making it hard for a doctor to recognize the signs. Sometimes a disease may also manifest in an unusual way, with a doctor excluding a diagnosis because the symptoms do not fit and later realizing that the patient's case was atypical.Four cases which had been referred to the Department of Pedodontics and Preventive Dentistry, Government Dental College, Thiruvananthapuram, Kerala, India, have been described.A 5-year-old girl was referred from a general dental practitioner with a diagnosis of a periapical abscess with swelling, pain, and erythema of the gingiva in relation to teeth numbers 84 and 85  with an A 5-year-old girl was referred with a diagnosis of Ewing's sarcoma. The child presented with a swelling of the right of the face of 2-week duration. On examination a fluctuant swelling was found in the buccal vestibule opposite 84 and 85. Pulpectomy of 85 had been done 2 1/2 years back. IOPA radiograph  revealedA 5-month-old infant was referred for biopsy with a diagnosis of neuroectodermal tumour of infancy. The parent had noticed a swelling on the palate 2 days before reporting to our department. A pigmented lesion was found on the palate on the right side . On careAn 8-year-old girl was referred with a diagnosis of ostetosarcoma of the left side of the mandible. The child presented with a bony hard swelling of the left side of the mandible in relation to teeth 74 and 75 Figures  of 2-monDentists play an important role in the early diagnosis and treatment of oral lesions. Ideally, this involves not only a thorough oral and sometimes a radiographic examination to identify the condition, but also confirmation of the clinical impression by biopsy and subsequent microscopic evaluation. However, treatment based solely on a clinical impression of the diagnosis, without histologic confirmation, can result in serious consequences, particularly when the lesion is precancerous or cancerous.In a study by Kondori, Mottin, and Laskin, of the clinical diagnoses made by the dentists submitting specimen for histopathological examination, 43% were incorrect. General dentists misdiagnosed 45.9%, oral and maxillofacial surgeons 42.8%, endodontists 42.2%, and periodontists 41.2% of the time. The most commonly missed clinical diagnoses were hyperkeratosis (16%), focal inflammatory fibrous hyperplasia (10%), fibroma (8%), periapical granuloma (7%), and radicular cyst (6%). Cancerous lesions were misdiagnosed 5.6% of the time [A mistaken diagnosis or a wrong diagnosis can cause undue agony to the patient and the parent (in case of children) till establishment of the correct diagnosis. In the cases reported attention to detail could have prevented parental distress as well as delay in the treatment procedure.Round cell tumours are rare destructive lesions of bone . ChildreThe second case presented in this paper was diagnosed as Ewing' sarcoma and referred for biopsy. The clinical and radiographic picture was the same as that in case one except that the lesion was fluctuant. The general practitioner had seemed to miss this finding and had concluded that it was an Ewing's sarcoma. The fluctuant nature of the lesion led to an FNAC which suggested that the lesion was suppurative.Melanotic neuroectodermal tumour of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants. There is increased excretion of vanillylmandelic acid in children affected with the tumour due to the neural crestl origin . In the The fourth case was suspected to be an osteosarcoma, again the result of a hasty conclusion. The child had presented with a swelling of the left side of the mandible of 2-month duration. There was no associated pain. Osteosarcoma is a malignant bone tumour that usually develops during the period of rapid growth that occurs in adolescence, as a teenager matures into an adult. The associated signs and symptoms include bone pain, pathological fractures, and pain during function. Plain radiographs of conventional, intramedullary osteosarcoma most commonly show a mixed lytic and sclerotic lesion causing cortical destruction, often with an associated soft tissue mass that may contain calcification . Various The parents of three of the children had been informed that the lesion was something with a malignant potential though the diagnosis had not been established nor had detailed examinations or lab tests been conducted. The parents were very apprehensive and agonised till the establishment of the final diagnosis. This is where reaching a definite diagnosis matters. As health care professionals it is utmost important that dentists give importance not just to the presence or absence of disease but also to the social and mental well-being of the patient and the parent. As a clinician one has to be careful in eliciting the patient's signs and symptoms. An accurate interpretation of these is even more important. Theoretical knowledge and clinical experience too matter in preventing a wrong diagnosis. But most importantly one has to weigh all the possible conditions that can manifest with the patients symptoms and signs before confiding with the patient, so that unnecessary emotional and mental trauma to the patient can be avoided.


Hydrocele of the canal of Nuck in children is rare. It may present as incarcerated inguinal hernia and necessitates emergency exploration. Risk of infection in hydrocele of the canal of nuck is very rare. We present a case report of a 5-year-old girl who presented with a left tender inguinolabial region swelling with fever, tachycardia, and mild dehydration. The clinical features were suggestive of strangulated left inguinal hernia and further imaging and surgical exploration revealed it to be an infected hydrocele of the canal of Nuck. High ligation and hydrocelectomy were performed. Hydrocele of the canal of Nuck in a female child presenting with an inguinal swelling should be considered in differential diagnosis. Contents of the inguinal canal vary between female and male population. During development, in the female fetus, round ligament of the uterus descends into the inguinal canal to the labium major. The peritoneal fold that descends with the round ligament is named the canal of Nuck. If this communication fails to close, it results in an indirect hernia or a hydrocele .Hydrocele of the canal of Nuck is not a common entity as only isolated case reports are published in the literature –6. InfecWe present a case of infected hydrocele of the canal of Nuck in a 5-year-old girl, initially pictured as strangulated inguinal hernia and later diagnosed as a case of infected hydrocele of the canal of Nuck. We emphasize to consider this as a differential diagnosis in girls with tender inguinolabial swelling.3, C-reactive protein 50, normal electrolytes, and coagulation profile. Her urinalysis was normal. Blood culture and sensitivity was sent and was reported normal. An ultrasound of inguinal region performed in the emergency room revealed a cystic mass in left inguinal region containing fluid in the upper part and fluid with internal echoes in the lower part of mass, and there was no free fluid noted in the abdomen. After resuscitation with fluids and administration of IV antibiotics, she was taken to the operating room for exploration. Operative findings included a cystic mass in the left inguinal canal extending down to the ipsilateral labia majora (A 5-year-old girl presented to the emergency with history of painful mass in the left inguinolabial region noticed a week before presentation. The child has a positive history of trivial trauma in school while playing with other children. The inguinal swelling and pain got gradually worse over the week. There was no history of recent illness in near future. Examination revealed temperature of 38°C, heart rate of 140/m, and mild dehydration. Abdominal and chest examinations were unremarkable. Local examination revealed a 6 × 3 cm irreducible tense swelling in the left inguinolabial region, with overlying skin redness and edematous. Blood investigations revealed white blood cell count of 14000/mma majora , cyst coa majora . The cysa majora . PostopeIn the female child, hydrocele of the canal of Nuck usually presents as a painless, translucent, fluctuating, and nonreducible swelling in the inguinal area and labium majora. Counseller and Black classified hydrocele of canal of Nuck into 3 types . The mosThe infection in the hydrocele of the canal of Nuck in female children is not known and has been reported only in boys where it has been considered to happen due to seeding from intraperitoneal source through patent processus , 7. In oHydrocele of the canal of Nuck should be considered in differential diagnosis for inguinal swelling in a girl. Clinically this may mimic indirect inguinal hernia, femoral hernia, abscess, tender adenopathy, Bartholin's cyst, posttraumatic hematoma, and rare entities such as cystic lymphangioma, neuroblastoma metastasis in groin, and ganglion , 9, 10. In conclusion, a hydrocele of the canal of Nuck though rare should be considered in the differential diagnosis in female children presenting with an inguinal swelling. Ascertaining a definitive diagnosis on physical examination may not be possible; hence, further evaluation with ultrasound imaging will be helpful. Surgery is mandatory for final diagnosis and treatment.


Residual cysts are among most common cysts of the jaws. The location of all odontogenic cysts is usually intraosseous. The peripheral (extraosseous) presentations are rare. The peripheral presentation of residual cyst has never been reported in the literature. In this article, the role of CT in diagnosing an unusual peripheral presentation of a residual cyst is discussed.The term  The higher frequency of occurrence of cysts in the oro-facial region could be attributed to complex embryology and development of teeth and due to presence of odontogenic epithelial remnants. Radicular and residual cysts are by far the most common cyst comprising about 52.3% to 60% of all jaw cysts , 2. MostA 30-year-old male patient reported to the Department of Oral Medicine and Radiology, Mahatma Gandhi Dental College & Hospital, Jaipur, India, with a chief complaint of slowly progressing swelling on the right side of the face of 5 months duration. Onset was insidious after extraction of a decayed tooth from the same region. Initially, it was small and soft but gradually it became large and hard in consistency. Occasional mild pain was reported especially on pressure and forward bending of the head. The medical history was noncontributory.On examination, patient was a short-statured, average-built, healthy-looking male. A general survey of the patient did not reveal any abnormality of significance. Face showed an obvious asymmetry caused due to a swelling on the right side of the maxilla. The swelling was approximately 3.5 × 3.5 cm. in size, circular in shape, and extending anteroposteriorly from the ala of nose to one cm behind an imaginary vertical line drawn from the lateral canthus of the eye and superoinferiorly, from infraorbital margin to one cm below the ala-tragus line. The color of overlying skin was normal. The swelling was bony hard in consistency, mildly tender on palpation with no change in local temperature. The overlying skin was smooth, intact, and appeared little stretched Figures  and 2. T Intraoral examination revealed missing first permanent molar in the right maxillary region with healed extraction socket and normal overlying alveolar mucosa. There was a well-defined, localized swelling obliterating the buccal vestibule of right maxilla. The swelling was extending superoinferiorly 0.5 cm away from gingival margin of premolars and first two molars to upper buccal vestibule and anteroposteriorly from distal of first premolar to distal of second molar. It was dome shaped, soft, fluctuant, and nontender on palpation. The overlying mucosa was smooth, elevated, but of the same color as that of adjacent mucosa and presented with no sign of inflammation. A fine-needle aspiration revealed a dark-red-colored, blood-tinged, and highly viscous fluid. Vitality test gave a positive normal response of the involved teeth . After analyzing history and clinical findings, a provisional diagnosis of a residual cyst was arrived at. The following cystic and tumorous lesions were considered as differential diagnosis. These were an odontogenic keratocyst, cystic ameloblastoma, cystic degeneration of adenomatoid odontogenic tumor, and a cyst arising from the maxillary antrum.As the swelling was in relation to right maxilla and its alveolar bone, we first carried out intraoral radiographic examinations. Intraoral periapical radiograph of maxillary right posterior teeth region revealed missing first permanent molar with a partial radiolucency superimposed on the edentulous region. There was no evidence of root stump, cystic radiolucency, or any abnormality in relation to the floor of the maxillary sinus . Occlusa Panoramic radiograph revealed a unilocular radiolucency with haziness surrounded by discontinuous cortication present on the right maxillary antrum region. It was oval in shape, around 3 × 2 cm in size, extending antero-posteriorly from distal of canine to mesial of third molar region. Superoinferiorly, it was not extended to displace teeth or floor of the orbit. The right zygomatic process, anterior wall, and floor of the maxillary sinus were not clearly demarcated indicating lateral, presence of the lesion causing displacement/resorption of these structures. Interestingly, all remaining first permanent molars were grossly decayed and associated with well-defined periapical radiolucencies; probably radicular cysts . Water's CT scan was carried out to rule out the origin of the lesion, sinus, or orbital involvement by the lesion and erosion of the alveolar bone. Coronal section revealed a round soft tissue lesion surrounded by a thin, discontinuous cortication present on the right lateral surface of the maxilla. It is causing pressure and superior displacement of inferiolateral walls of the maxillary sinus, decreasing the size of the sinus without actual perforation or involvement Figures . Axial s Cytological examination of aspirates was suggestive of chronic inflammatory fluid. The surgical enucleation of the cyst was carried out under local anesthesia and strict asepsis through an intraoral approach . The secResidual cyst usually has a typical location and radiographic aspect, allowing an easy diagnosis. These cysts are usually intraosseous with the epicenter is positioned in the former periapical region of an extracted tooth. Occasionally, radicular cysts are present on the lateral aspect of the root in relation to accessory canal of the devitalized tooth. To our knowledge, peripheral (extraosseous) residual cyst has never been reported to date in the English-language literature. Nevertheless, the location does not seem to have any relation to either behavioral or histological features of the cyst .In a study carried out on five hundred ninety four patients with 621 cysts, most of the cysts were inflammatory: 435 cysts (70.1%) and odontogenic: 603 97%) in their origin . Out of 7% in theCT scan examination revealed a subperiosteal cyst/benign tumor present peripherally on the surface of the maxilla causing superior and posterior displacement of inferiolateral and anterolateral walls of the maxillary sinus, respectively, but not occupying the sinus cavity. In this case, conventional radiographs just revealed a unilocular radiolucency with an incomplete surrounding cortex, present on the surface of the maxilla. However, the origin and location, whether it is intraosseous or extraosseous or from alveolus or maxillary sinus, and its extension and effect on the surrounding structures were not clearly defined. Even histopathological and other additional investigations did not focus much on these aspects of the lesion.The epithelium of residual cyst originates from the epithelial rests of Malassez, although, in some instances, it arises from the respiratory epithelium of the maxillary sinus when the periapical lesion communicates with the sinus wall. It may also come from oral epithelium from a fistulus tract or oral epithelium proliferating apically from a periodontal pocket. The epithelium may be derived from the surface epithelium or from the epithelium of adjacent glands or hair follicles . IntraneP < 0.001) [in situ following extraction of that tooth. Many residual cysts are slowly resolving especially the asymptomatic one [Radicular cyst arising from deciduous teeth is also one of the possibilities considered. There are cyst-prone individuals who show a particular susceptibility to develop radicular cysts . They ha< 0.001) . Many raatic one , 16; somatic one .No statistically significant differences were found between fluid from apical periodontal and residual cysts on the one hand and autologous serum on the other, or between the different types of cyst fluids . A data In conclusion, this case report clearly demonstrated the importance of the advanced imaging like CT scan in detecting exact location, extension of the peripheral (extraosseous) residual cyst, and its effect on the adjacent structures especially maxillary sinus. This cyst should be included in the differential diagnosis of peripheral lesions of the jaws.


Malignant peripheral nerve sheath tumour (MPNST) is extremely rare malignancy in the general population, occurring more frequently in patients with Neurofibromatosis type 1 (NF1). In the literature five cases of MPNST arising from the parapharyngeal space (PPS) in patients without neurofibromatosis have been reported. We report imaging techniques in a patient with MPNST in the PPS, who had neither a family history nor sign of NF1. Computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed for a correct therapeutic planning. CT and MRI findings were correlated with hystopathological diagnosis. Malignant peripheral nerve sheath tumour (MPNST) is an extremely rare malignancy. It usually encountered in patients with Neurofibromatosis type 1 (NF1) with an incidence of 2–5%, while in the general population it has an incidence of 0,001% –5. We reA 71-year-old man was admitted to our observation with a five months history of a gradually enlarging pulsanting painful neck mass, localized in the left laterocervical side. He reported dysphagia and breathing difficulty since about four weeks. He did not report symptoms associated with cranial nerve deficit. Intraoral inspection demonstrated a swelling in the tonsillar region without erosion of the mucosa. Computed tomography (CT) scan, performed with a 64-row scanner , showed a well-defined isodense expansive lesion in the left PPS, which determined bulging of the oropharyngeal wall. After the injection of contrast medium organo-iodized , the mass showed inhomogeneous contrast enhancement  because Different diagnostic hypotheses were formulated: pleomorphic adenoma, neurogenic tumour, and lymphnode pathology. A subsequent dynamic contrast-enhanced MRI with a 1.5 T unit  was performed with the head-neck coil to better characterize the mass and to assess the topographical relationship of the tumour with vessels and with neighbouring structures. MRI protocol included axial and coronal T2 (T2WI), axial and coronal fat-saturated T2, axial e coronal T1 (T1WI), and axial T1W fat-saturated postcontrast agent .MRI confirmed the presence of a well-defined rounded lesion localized in the left PPS. The lesion compressed the pharynx, and it had a cranial extension almost up to the Eustachian tube. The mass presented a signal hyperintensity in T2WI and in T2WI fat-saturated sequences and a signal hypointensity in T1WI sequences.Post-Gadolinium T1WI fat-saturated sequences showed an early inhomogeneous contrast enhancement of the lesion . The mulThe fat tissue thickness appeared reduced. Multiple increased in size lymph nodes were detected behind the left mandibular angle and in the bilateral laterocervical spaces.The patient underwent an incisional biopsy with multiple Tru-Cut needle biopsy which revealed a neoplasm composed of fusiform cells with small necrotic foci. Immunohistochemistry demonstrated strongly positive staining for neurone specific enolase (NSE), slightly positive staining for a smooth, actine, and positive staining for S-100 protein, consistent with malignant neurosarcoma or MPNST . CT total body examination showed no evidence of metastases. The patient underwent a transcervical surgical excision of the tumour without any subsequent complication, followed by radiation therapy.MPNST is a rare sarcomas arising from the Schwann cells of peripheral or exceptionally from the cranial nerves –7. It caThus only few of these tumors occur in the head and neck regions . In thesIn the literature five cases of MPNST arising from the PPS in patients without Neurofibromatosis have been reported , 11. TumThe spectrum of differential diagnosis of a neoplastic mass in the PPS commonly includes salivary gland tumour, neurogenic tumour, skull base, and vertebral tumour such as meningioma or chordoma, rhabdomyosarcoma, and more rarely, Castleman's disease, which arises from adjacent nodes and extends into the space .The PPS is a complex anatomical area, and it is difficult to be examined; it is described as an inverted pyramid divided into two spaces: the prestyloid or “true parapharyngeal” space and the poststyloid or carotid or retrostyloid space. The first one includes fat, a small portion of the retromandibular parotid gland, minor or ectopic salivary glands, branches of the mandibular division of the trigeminal nerve, and some lymph nodes. The poststyloid space includes the internal carotid artery, the internal jugular vein, cranial nerves (IX–XII), the sympathetic chain, and lymph nodes .CT and MRI images are fundamental to evaluate tumours of this anatomical area, while there is not a reliable sonographic window to allow an ultrasound examination. CT is useful to evaluate the PPS in but MRI results in the preferred technique for a better contrast resolution. While CT is useful to assess the tumour extension and eventual metastasis (the more frequent are bone and lung metastasis), MRI can reveal the nerve of origin, and it is more accurate to evaluate the topographical relationship of the tumour with neighbouring structures, especially vascular, muscular structures and fat planes involvement. In particular MRI distinguishes the lesion from the fat tissue better than CT, whose dislocation and thinning of the fat tissue thickness have a critical importance in localizing the space of origin of a neck lesion .Histologically MPNST is a spindle-cell sarcoma. The common type of malignant schwannoma consists of rounded or fusiform cells arranged in sweeping fascicles and recapitulating the features of normal Schwann cells. Other hystological types can be encountered, such as glandular malignant schwannoma, malignant epithelioid schwannoma, or malignant Triton tumour, where both malignant schwannoma and rhabdomyosarcoma are associated. The rhabdomyoblastic type is more often encountered in the head and neck . If some authors consider the fine needle aspiration biopsy useful diagnostically, the definitive diagnosis is immunohistological. In particular, histology can allow differential diagnosis between MPNST and other types of fusicellular sarcoma on the basis of immunohistochemical markers, including S-100 protein and NSE , 18.Histopathological characterization can be performed by tru-cut biopsy, open incisional biopsy, or excisional biopsy. Although there is an established management protocol, surgical resection using different techniques  is the mainstay of treatment of MPNST. Surgical treatment is usually followed by radiation therapy, with or without adjuvant chemotherapy , 19.Surgical candidates include patients with primary malignant neoplasms for which surgery is appropriate therapy, patients with select metastatic malignancies , benign salivary gland neoplasms, neurogenic neoplasms with existing cranial nerve deficits, and neoplasms with mass effect symptoms.If the transcervical approach remains the most commonly used surgical approach to the parapharyngeal space, the transoral approach is now considered for selecting small and accessible neoplasms. Recently, transoral endoscopic and robotic approaches to the parapharyngeal space have been described .MPNST is a very rare disease, especially in patients without neurofibromatosis and in the PPS. Neoplasms developed from the PPS represent a diagnostic issue because of the complexity of this anatomical area. Imaging tools are fundamental to characterize and localize the tumour and for a correct therapeutic planning. MRI represents the technique of choice, because it presents a better contrast resolution than CT scan.