The keratocystic odontogenic tumour (KCOT), formerly known as the odontogenic keratocyst (OKC) is a benign intraosseous lesion that derives from remnants of the dental lamina. Due to its characteristics, clinical and histopathological features and various treatment approaches, this pathology is different comparing with other odontogenic cysts. Radiographically the KCOT appears as well-defined unilocular or multilocular radiolucency with thin radiopaque borders. In most cases, conventional radiographic imaging, such as panoramic views and intraoral periapical films, are adequate to determine the location and estimate the size of an KCOT. However, the clinical use for cone-beam computed tomography (CBCT) in oral and maxillofacial surgery increases and provides additional information about the contents and borders of the large lesions. In the present cases, the diagnostic performances of CBCT versus panoramic radiograph for four KCOTs were evaluated. It was concluded that appearance of lesions in the maxillofacial region could be better documented in the correct dimensions by CBCT versus panoramic radiograph. Key words:Odontogenic keratocyst, cone-beam computed tomography, three dimensional, panoramic radiograph. The odontogenic keratocyst (OKC) is designated by the World Health Organization (WHO) as a keratocystic odontogenic tumour (KCOT) and is defined as “a benign uni- or multicystic, intraosseous tumour of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium .The potential for aggressive clinical behavior and local recurrence resulted in its recent classification as a benign odontogenic tumor .A more “innocent” entity ortokeratinized odontogenic keratocyst (OKK) is not included in this novel classification, is still classified in developmental odontogenic cysts .The keratocystic odontogenic tumour, which represents between 4-12% of all odontogenic cysts, is believed to arise from dental lamina remnants and extension of basal cells of overlying oral epithelium . HoweverKCOTs have a predilection for males and occur mainly in the second and third decade of life. It may occur anywhere in the jaws, but is most frequently found in the posterior region of the mandible ,8. MultiA localized asymptomatic swelling associate with mobility of the teeth, and spontaneous drainage of the tumour into the oral cavity, are the most common symptomes of the KCOT occurrence ,8. RadioTraditional radiographic examinations are usually limited to two-dimensional views captured using radiographic film or digital sensors and are adequate to determine the location and estimate the size of the tumour. However, the clinical applications for cone-beam computed tomography (CBCT) imaging in the oral and maxillofacial (OMF) region are increasing. A major advantage of CBCT that has been reported is the three-dimensional geometric accuracy compared with conventional radiographs. Sagittal, coronal and axial CBCT images eliminate the superimposition of anatomical structures .Despite the fact, that the most clinical applications in OMF region are reported as impacted teeth and implantology , it is wThe purpose of this article is to present four clinical cases of KCOTs for which managements, diagnostic accuracy of the CBCT versus panoramic radiographs were compared.Four patients , age range 24 to 56 with solitary or multiple jaw lesions, clinically and histopathologically diagnosed as the ‘‘KCOT’’, treated in the department of oral and maxillofacial surgery, Faculty of Dentistry University of Istanbul, were included in this study case presentation . For dia-Case 1A 56 year-old male patient was referred with a complaint of painless swelling in the front portion of the upper jaw. The panoramic radiographs disclosed a radiolucent mass in the right anterior region of the maxilla Fig. . Due to -Case 2A 38 year-old male patient complained of six months persisting pain of the right upper lateral incisor, canine and first premolar. A fine-needle aspiration revealed a cystic content. Panoramic radiographs revealed unilo-cular undefined radiolucency in the right maxillary antrum Fig. . Under l-Case 3A 24 year-old male patient referred with complaints of pain and swelling in right maxilla. From the patient’s health history it was found out that a cyst operation in the mentioned part of maxilla was performed, a 5 years ago. Clinical examinations revealed a swelling and asymmetry below a lower eye lid from the right side of the face. From the vestibular side the soft expansion in the region of upper right premolars was noted. Panoramic radiographs revealed a radiolucent lesion extending from upper right canine to second premolar involving a maxillary antrum Fig. . A fine--Case 4A 26 year-old female was referred with symptoms of swelling in anterior mandible. Panoramic radiographs showed a multiple well-circumscribed radiolucent formations: in front mandible involving a mandibular incisors and left first premolar, which undergone a root resorptions; bilaterally at mandibular body ramus area; in right maxillary sinus space, associated with impacted third molar Fig. . CBCT exDue to multiple cystic formations, the patient underwent an investigations for Gorlin-Goltz syndrome, which was denied. The patient underwent enucleation of cystic lesions with extractions of impacted tooth under general anesthesia. Histopathological evaluation of the submitted samples in all cases, except maxillary lesion, revealed KCOT. The maxillary lesion was diagnosed as a dentigerous cyst.Clinical occurrence of KCOTs mostly seen in mandible 65–83%) comparing with maxilla ,5. In ou–83% compBasal layer of the tumour epithelium might be budding into a supporting connective tissue, forming so called daughter cysts, which are mostly responsible for tumour reccurrences, that ranges from 5-62% and is more higher for those tumours associated with Gorlin-Goltz syndrome (Radiographically, the KCOT is characterized as well borded radiolucent lesion with smooth and usually sclerotic margins. One of the important characteristics of KCOTs is their association with impacted teeth, with occurrence rate from 25 to 40%, which should not be neglected, due to differential diagnosis to dentigerous cysts, which was present in our described Case 4 . Other dDisadvantages of the panoramic radiography include geometric distortion, lack of fine detail, and numerous artifacts that require care to interpret . BecauseIn the present cases, it was observed that the panoramic radiograph gives more detailed information in the mandible comparing the maxilla. Especially in maxillary sinus, the panoramic radiograph was very week to describe the lesion. So we concluded that the CBCT evaluation loom large in clinical practice especially for the KCOT located in the maxillary sinus, in order to maintain a good preoperative planning.The KCOT as a thin lining of parakeratinized epithelium, and can have thick cheesy contents due to des-quamated keratinizing squamous cells. These contents increase the radiographic attenuation of the lesion at computerized tomography . These fThe present report compared diagnostic performance of CBCT versus panoramic radiograph. Within the limits of this study, it was concluded that CBCT is more useful for the diagnosis of odontogenic keratocysts and also for proper surgical planning of the operation. CBCT provided more detailed information for evaluation of the borders of the lesion and the relation of the lesions with adjacent anatomic structures. Since KCOT has a great potential to recur, a CBCT examination is recommended for presurgical evaluation. Another important point of the present study is to emphasize that maxillofacial radiologists should be aware of the characteristic CBCT findings of the KCOT in order to differentiate the lesion from the other odontogenic lesions.


Desmoid tumors (DTs) are benign tumors which are not seen very often, and most of the radiologists and clinicians do not know the characteristics of them very well. Correct and early diagnosis of DTs is important for decreasing mortality and morbidity. Computed tomography enterography (CTE) is a new modality for small bowel imaging which combines the improved spatial and temporal resolution of multidetector computed tomography (CT) with large volumes of ingested enteric contrast material to permit evaluation of the small bowel wall and lumen and also the entire abdomen. We report a familial adenomatous polyposis (FAP) patient with localized mesentery and abdominal wall DTs. We showed the exact location of the DTs and their relation with the small bowel by CTE. In conclusion, CTE is a useful technique for DT localization, the degree of extension and invasion to local structures, presence of partial and complete small bowel obstruction, and the relationship of the tumors with vasculature and whether ischemia has occurred as a result or not. Desmoid tumors (DTs) are uncommon benign tumors occurring as a result of excessive proliferation of connective tissue which tend to be locally more invasive than metastasis . Thirty In routine clinical practice, conventional abdominal computed tomography (CT) or magnetic resonance imaging (MRI) is used in the diagnosis of mesenteric DT . AbdominCT enterography (CTE) is a new modality for small bowel imaging which combines the improved spatial and temporal resolution of multidetector CT (MDCT) with large volumes of ingested enteric contrast material to permit visualization of the small bowel wall and lumen and also the entire abdomen 7]. Diff. Diff7].We report a FAP patient with mesenteric and abdominal wall DTs. We showed their exact location and relation with the small bowel by CTE. As far as we know, there has not been a report of a patient with DT who was diagnosed with CTE in the literature. Our aim is to discuss the efficacy of CTE in the detection and evaluation of DTs referring to the literature. We think that this report may be useful for early and correct diagnosis of similar cases.The patient, who was diagnosed as FAP and had total colectomy before, admitted to the gastroenterology clinic of our hospital with abdominal pain and anemia. In order to understand the etiology of anemia, upper gastrointestinal endoscopy was performed and neither the origin of the bleeding nor a cause such as malignancy or any other etiology was detected. Therefore, we decided to use CTE in this case to see the possible existence of any intra- or extraintestinal pathologic condition.Before CTE, 1250 mL of oral contrast material solution–775 mL of water, 250 mL of lactulose  and 225 mL of dilute barium sulfate suspension especially designed for abdominal MDCT examinations –was ingested over 45 min at a steady rate. After administration of the oral contrast agent, CTE was performed with 64-detector MDCT machine . After intravenous administration of 100 mL of Iopromide  at a flow rate of 3 mL/s, abdominal contrast-enhanced MDCT images with 1 mm slice thickness were obtained in portal phase. After CTE examination, sagittal and coronal reformatted images (slice thickness: 1 mm) were obtained which are routinely available on workstation inside the MDCT machine.In CTE examination, there was no sign of manifested small bowel obstruction and intraluminal pathology. Intraabdominal solid organs were normal. A 39 × 42 × 36 mm sized contrast material enhancing solid mass was seen between the rectus muscles at the superior region of the umbilicus, invading to the subcutaneous fatty tissue anteriorly and limited by the omentum posteriorly. This mass had a well-defined margin making indentation without invasion to the small bowel loops . AnotherAs a result of these findings, gray scale and Doppler ultrasound were applied. On gray scale US, the masses showed well-circumscribed homogeneous hypoechoic appearance (character of fibrosis). In the anterior abdominal wall, two lesions, which were close to the peritoneum and greater omentum, were localized. On Doppler US imaging of these lesions, no sign of significant vascularization was seen. Mesenteric DT seen in CTE could not be demonstrated by US. As a result of US and CTE findings, all these lesions are considered as DTs. Thus, medical therapy and follow-up was recommended.DTs, also called abdominal fibromatosis are benign tumors which are not seen very often, and most of the radiologists and clinicians do not know the characteristics of them very well 2]. The . The 2].DTs are often asymptomatic, but can represent as abdominal pain, palpable abdominal mass, nausea, anemia, vomiting, diarrhea or fever 2]8]. D[8]. D2][. D[8]. DThe treatment options for DTs include medical therapy , cytotoxic chemotherapy, radiation therapy or surgery 2]. Due . Due 2].The radiologic characteristics of DTs could be changed according to their fibroblastic proliferation, collagen component, vascularity and fibrosis 2]8]. O[8]. O2][. O[8]. OOn contrast-enhanced abdominal CT scans, most DTs appear as homogeneous masses with well- or ill-defined margins that may have iso- or hyperdense appearance relative to muscle planes 2]. Some. Some2].CTE is a relatively new technique which is used generally in patients with Crohn’s disease and other small bowel pathologies 10]. The. The10].The advantages of CTE with MDCT are scanning the whole abdomen with thin slices in one breath time, taking multiplanar reformatted images and showing the small bowel and the surrounding tissues directly 13]. The. The13].As a result of the developments in CT technology, there has been significant improvement in reformatted image quality. We could evaluate the extension of mesenteric DTs, surrounding the small bowel and the vessels with 64 detector MDCT which could not be detected by US or endoscopy. Besides, we could easily determine the relationship between anterior abdominal wall localized DTs and the small bowel or the intraperitoneal space with CTE. New and comprehensive studies are needed to determine the role of CTE in demonstrating the relation of DTs with the small bowel and mesenteric structures. The effect on the prognosis and also the role of the morphological appearance of DTs in enterographic images on the surgical plan should be assessed in detail in those further studies.According to the results of our case, CTE is a useful technique for the evaluation of DT location, degree of extension and invasion to local structures, or the presence of partial/complete small bowel obstruction and the relationship of the tumors with vasculature and whether ischemia has occurred as a result or not. CTE is good also for examining extra-intestinal complications such as fistulas, urinary tract obstruction and abscess formation.


Introduction: Schwannomas are benign and not very frequent tumors of the peripheral nerves, derived from the nerve supporting Schwann cells.Study Design: Data were collected on the clinical manifestations , location, size and symptonts of the lesions as well as the evolution time and the initial (presumption) diagnosis.Results: Twelve patients were documented, with a mean age of 29,5 ± 12,1 years (range 16-50) and a balanced gender distribution. The mean duration of the lesions was 42,17± 45,3 months. The lesion located in the floor of the mouth was the largest tumor, measuring about 4 cm in maximum diameter, while the average size of the 12 schwannomas was 2.04± 1.1 cm.Conclusion: We present 12 oral schwannomas diagnosed and treated over a period of 10 years. Key words:Schwannomas, oral benign tumor, neurilemmoma. Schwannomas or neurilemmomas are benign and not very frequent tumors of the peripheral nerves, derived from the nerve supporting Schwann cells. The first description of this type of tumor was made by Verocay in 1910, and although these are infrequent lesions, between 25-48% are located in the cervicofacial territory -5.Schwannomas can be divided into central or intraosseous and peripheral lesions. In turn, a variant known as ancient schwannoma contains degenerative phenomena such as cystic cavities, hemorrhage, hyalinization or calcifications ,7. A varIntraoral peripheral schwannomas are fundamentally located in the tongue, followed by the palate, floor of the mouth, cheek mucosa and gums ,9-11. InSchwannomas are slow-growing and asymptomatic, and the differential diagnosis must be established with other clinically similar tumors such as neuromas, neurofibromas, granular cell myoblastoma, neuroepitheliomas, fibromas or adenomas . The tumImaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI) can offer valuable information, showing a homogeneous, very well delimited and solid lesion. However, the definitive diagnosis requires biopsy-removal of the lesion  and histTreatment consists of complete removal of the encapsulated lesion. The causal nerve is rarely identified, and relapses or neurosensory defects after the operation are exceptional ,15,23,24The present study describes a series of 8 cases of intraoral schwannoma diagnosed and treated in the Department of Stomatology and Maxillofacial Surgery  during the period 2002-2011 (cases 1-8) and 4 cases reported by the Department of Diagnosis and Oral Surgery , diagnosed and treated during the same period 2003-11 (cases 9-12).Twelve patients were documented, with a mean age of 29,5 ± 12,1 years (range 16-50) and a balanced gender distribution . The meaMagnetic resonance imaging was performed in three cases, and in two of them (corresponding to the largest lesions) fine needle aspiration biopsy (FNAB) and prior biopsy were carried out. The two largest lesions  were completely removed under general anesthesia, while in the remaining 10 cases the tumor was removed under local anesthesia. There were no postoperative complications or relapses in any of the cases.A histopathological diagnosis of schwannoma was established in all cases, and the immunohistochemical study revealed positivity for protein S-100, with a Ki-67 cell proliferation index of 4% and 5% in cases 1 and 4, respectively.Schwannomas located in the maxillofacial territory account for 30-40% of all such tumors ,2. Leu eAlthough schwannoma appears to show a slight male predilection , some auClinically, schwannomas produce few symptoms and are usually recognized from the presence of a slow-growing mass. They only rarely manifest as an ulcerated or infected lesion . As in cA tentative clinical diagnosis of schwannoma is difficult to establish, and identification of the lesion sometimes comes as a surprise . Wang etAmong the complementary diagnostic techniques, mention must be made of the panoramic X-ray study to discard associated dental lesions or the involvement of bone in proximity to the lesion. A routine panoramic X-ray study was carried out in all of our patients. However, the mucous and exophytic nature of schwannomas requires the use of MRI, which is the diagnostic technique offering the largest body of information in such cases. MRI was used in three of our patients. A biopsy may be indicated in very large lesions or cases characterized by important diagnostic uncertainty. In our patients with tumors in the floor of the mouth and in the palate, biopsies were obtained to confirm the diagnosis.The definitive diagnosis of schwannoma is established by the histopathological study of the lesion, with the presence of the two typical tissue patterns referred to as Antoni A and Antoni B. The first of these patterns is characterized by the presence of cells with elongated or fusiform nuclei adopting a palisade distribution Fig. . In contThe treatment of schwannomas consists of surgical removal of the tumor, with preservation of the neighboring structures. Relapses are very rare. These lesions usually cause no sensory defects, while Lacosta et al.  in their


Central Giant Cell Granulomas (CGCGs) may manifest as radiolucencies anywhere in the mandible or maxilla. In rare cases, it can appear as a localized periradicular area and mimic an endodontic lesion. This case report presents an uncommon location of CGCG which was not accurately diagnosed nor timely treated. Periodic follow ups of periapical radiolucencies after RCT are necessary. Dentists should include CGCG in differential diagnosis of lesions that are refractory to endodontic treatment.  Central Giant Cell Granuloma (CGCG) is an uncommon benign lesion  that wasAggressive and non-aggressive forms of CGCGs are similar in their histopathologic features  which deCGCGs that are localized to the periapical region or lateral to tooth roots can be easily confused with inflammatory odontognic lesions such as dental granuloma and radicular cyst. The common occurrence of periapical granulomas and cysts lead the clinician to arrive at a definitive diagnosis without full diagnostic tests and histopathologic examination . This caA 30 year-old woman was seen in a dental surgery complaining of painful swelling on her anterior lower jaw in the parasymphysis area. The history of complaint revealed gradual growth of the swelling during the past year. The medical history was not significant. Oral cavity examination revealed a 2.5-cm fixed painful mass in anterior of mandible. The oral mucosa was intact. Intraorally, anterior lower teeth were intact and not mobile. Panoramic and periapical radiographies revealed well demarcated unilocular periapical radiolucency in region of mandibular incisors and canine . Sensitivity tests of involved teeth were negative for the right mandibular canine.Initially, root canal treatment of right mandibular canine was performed. However, after 6 months, expansion and pain of anterior region of mandible had increased gradually.The patient was referred to an endodontist for re-treatment of right mandibular canine. After re-treatment, signs and symptoms did not subside and the swelling of the anterior mandible persisted. Patient was referred to oral and maxillofacial surgeon. Needle aspiration biopsy was carried out, unfortunately it demonstrated negative results.Clinical examination discovered no neurosensory defects by the oral surgeon. These findings suggested a differential diagnosis of Central Giant Cell Granuloma (CGCG), Aneurysmal Bone Cyst, or Cystic Calcifying Odontogenic Tumor. Excisional biopsy was conducted.Gross examination of lesion revealed many small soft elastic specimens, white-brown in color with size of 2.5 2 0.5 cm. Solid section surface of specimens was seen.Histologically, multinucleated giant cells were distributed in a stroma that was highly cellular compromising both spindle-shaped and round cells which were found mostly in hemorrhagic and reactive bone areas. Ingested RBCs and scant collagen fibers were also seen . These fCGCG is a benign intra-osseous lesion with unknown etiology . A quantet al. researched 1659 periapical radiolucencies over a 10 year period and found that 52% of periapical lesions were granulomas, 42% cysts, 2% periapical scars and 4% other disorders  were localized in periapical areas of the tooth  followed by CGCG (3 cases). No malignancy was seen .A differential diagnosis that includes CGCG’s should always be born in mind when assessing periradicular radiolucencies in the anterior mandible. Clinical examination, non surgical approach and periodic follow up is the recommended route for periradicular lesions. If endodontic therapy was ineffective at follow-up examination, surgical biopsy may be taken for histopathological examinations.


Urachal diseases are rare and may develop from a congenital anomaly in which a persistent or partial reopening of the fetal communication between the bladder and the umbilicus persists. The most frequently reported urachal anomalies in adults are infected urachal cyst and urachal carcinoma. The diagnosis of this entity is not always easy because of the rarity of these diseases and the atypical symptoms at presentation. Imaging techniques, such as ultrasonography and computed tomography have a significant role in recognizing the presence of urachus-derived lesions.Case presentation 1: A 25-year-old Arab-Berber man presented with a 10-day history of progressive lower abdominal pain accompanied by fever, vomiting, and low urinary tract symptoms to our emergency department. Laboratory data revealed leucocytosis. The diagnosis of an acute peritonitis was made initially. Abdominal ultrasonography revealed a hypoechoic tract from the umbilicus to the abdominal wall, and the diagnosis was rectified . The patient was initially treated with intravenous antibiotics in combination with a percutaneous drainage. Afterwards an extraperitoneal excision of the urachal remnant including a cuff of bladder was performed. The histological analysis did not reveal a tumor of the urachal remnant. Follow-up examinations a few months later showed no abnormality.Case presentation 2: A 35-year-old Arab-Berber man, without prior medical history with one week of abdominal pain, nausea and vomiting, associated with fever but without lower urinary tract symptoms visited our emergency department. Laboratory data revealed leucocytosis. Abdominal ultrasonography was not conclusive. Computed tomography of the abdomen was the key to the investigation and the diagnosis of an abscess of urachal remnants was made. The patient underwent the same choice of medical-surgical treatment as previously described for case one, with a good follow-up result.Case presentation 3: A 22-year-old Arab-Berber man, with no relevant past medical history, presented to our emergency department because of suspected acute surgical abdomen. Physical examination revealed umbilical discharge with erythema and a tender umbilical mass. Abdominal ultrasonography and computed tomography scan confirmed the diagnosis of infected urachal sinus. Initial management was intravenous antibiotics associated with a percutaneous drainage with a good post-operative result, but a few days later, he was readmitted with the same complaint and the decision was made for surgical treatment consisting of excision of the infected urachal sinus. The clinical course was uneventful. Histological examination did not reveal any signs of malignancy.We describe our clinical observations and an analysis of the existing literature to present the various clinical, radiological, pathological and therapeutic aspects of an abscess of urachal remnants. To the best of our knowledge, this manuscript is an original case report because this atypical presentation is rarely reported in the literature and only a few cases have been described. The urachus or median umbilical ligament is a fibrous cord that originates from the involution of the allantoic canal. It extends from the bladder dome to the posterior umbilicus. A partial or total defect of obliteration of the urachus channel after the fifth month of gestation can be the origin of urachal abnormalities. The first description was in 1550 by Cabriolus -5. This Proteus mirabilis infection for which he was treated with ciprofloxacin. At 18 months post-operatively, he was asymptomatic and no abnormalities of the abdominal wall were seen.A 25-year-old Arab-Berber man, without prior relevant medical history, with lower abdominal pain which had persisted for 10 days presented to our emergency department. The character of the pain was intense and persistent, accompanied with fever, vomiting, and low urinary tract symptoms . On physical examination, he looked tired and his body temperature was 39.5C. Abdominal examination revealed a diffuse tender lower abdomen and midline suprapubic mass measuring 5cm in length. The umbilicus looked normal and no peritoneal signs were elicited. A rectal examination revealed no tenderness and no blood. Laboratory data revealed leucocytosis with 82% neutrophil predominance and a white blood cell count of 13,000/L. Blood biochemistry was normal. Urinalysis was normal and urine culture showed no bacterial growth. A standing abdomen radiography was normal. Abdominal ultrasonography (US) revealed a hypoechoic tract from the umbilicus to the abdominal wall . Blood biochemistry was normal. Urinalysis was normal and non bacteriuric. His standing abdomen x-ray film was normal. Abdominal US revealed a small quantity of fluid in the peritoneal cavity. A computed tomography (CT) of the abdomen was the key to the investigation and showed inflammation with localized abcess formation (65 × 36 × 42mm) from the umbilicus to the dome of bladder, and the surrounding fatty tissue was infiltrated. There was no free intraperitoneal fluid or lymphadenopathy , the laparoscopic approach for resection of the urachus was first introduced by Trondsen et al.  Since thet al. ,31,34-36et al. [Each case was technically feasible with acceptable operative time but no quantitative assessment of outcome and morbidity was provided ,37. Usinet al.  have sugIn summary, urachal abscess may only present with abdominal pain without obvious erythematous periumbilical tissue or exudates in adults. Despite the low incidence in adult patients, it should not be ignored in the differential diagnosis of abdominal pain. History taking and detailed physical examination may help in early diagnosis. US and CT scan are the gold standard diagnostic tools for suspected cases of urachal lesions.Treatment of the infected urachal remnants in adulthood should consist of antibiotic treatment combined with adequate drainage and later followed by total excision of the remnant including resection of the bladder cuff. This strategy seems to be the most effective treatment option.The laparoscopic approach appears to be a safe and effective alternative to open surgery in the management of urachal remnants with recurrent infections in both infants and adults. It provides easier access to the bladder dome in contrast to classical open surgical incisions. In addition it permits an acceptable excision of the lesion without producing marked scarring. It yields good long-term cosmetic results.Written informed consent was obtained from the patients for publication of this manuscript 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.FT and MA are the principal authors and major contributors in writing the manuscript. AK and SM analyzed and interpreted the patient data and reviews of the literature. MJE and MHF read and corrected the manuscript. RSW contributed to the writing and correction of this paper. All authors read and approved the final manuscript.


Eccrine porocarcinoma (EPC) is a rare tumor. It develops from the intraepidermal ductal portion of the eccrine sweat gland. Metastatic disease is rare. We report a new case of metastatic eccrine porocarcinoma with a successful management and a good response to docetaxel. A 54-year-old man was admitted with a mass in the breast. Biopsy specimen found carcinomatous tumor proliferation with large anastomosing ducts. Cellular atypia were noted, with eosinophilic cytoplasm and round to oval nuclei. The tumor showed positive immunoreactivity for ACE and negative to anti-PS-100. Resection was performed. One year later, he presented with local and metastatic recurrences. The patient had received 3 cycles of cisplatin and 5-fluorouracil; he progressed with increase in mass size and number of lung lesions. He has been undergoing three cycles of docetaxel with complete response in the lung and regression of the breast mass. The mass was excised. Porocarcinoma is a very rare entity and poorly understood. In the metastatic phase, it has modest or no sensitivity to anticancer treatment. Docetaxel should be considered in the metastatic eccrine porocarcinoma. Eccrine porocarcinoma (EPC) is a rare tumor . It deveA 54-year-old patient was admitted with a mass in the breast, the biopsy specimen found carcinomatous tumor proliferation with ulcerated surface, large anastomosing ducts that contained clear cell nests . CellulaEccrine porocarcinoma is a rare tumor . It accoThe differential diagnosis with other malignant tumors of the skin is very complex, especially with seborrheic keratosis, Bowen tumor, multifocal basal cell carcinoma, lymphoma, achromic melanoma, pyogenic granuloma, wart, and nevus.The classic histological description of eccrine porocarcinoma is an acanthotic epithelial proliferation that contained clear cell nests with radial extension of polygonal nuclei, eosinophilic cytoplasm, and rudimentary ductal structures with many intraepidermal atypia . The diaMetastasis occurs in about 20% of cases with a very poor outlook and high mortality. They occurred preferentially in lymph nodes, lung, retroperitoneum, and liver . The main treatment for localized form is surgical excision with histologically clear margins, , 10; it'Single agent docetaxel have been used in metastatic eccrine porocarcinoma with symptomatic and radiological response lasting several months. It was well tolerated .Our patient had complete response in the lung and regression of the breast mass after three cycles of docetaxel. Docetaxel should be considered in platinum resistant or refractory patients with metastatic eccrine porocarcinoma.Porocarcinoma is a very rare entity and poorly understood. The clinical diagnosis is very evocative. The standard pathological diagnosis establishes the diagnosis easily. Immunohistochemistry is useful in difficult cases. Treated early, eccrine porocarcinoma is curable by wide excision. In the metastatic phase, it has little or no sensitivity to anticancer treatment. Docetaxel should be considered in platinum resistant or refractory patients with metastatic eccrine porocarcinoma.


Combined small cell carcinoma (SmCC) and squamous cell carcinoma (SqCC) is a rare malignant neoplasm in the head and neck. This study presents the first reported case of combined SmCC and SqCC originating from the sinonasal tract accompanied by syndrome of inappropriate secretion of antidiuretic hormone (SIADH). An 80-year-old female presented with a four-week history of right nasal discharge, nasal obstruction and left neck swelling. Imaging studies revealed a tumorous lesion in the maxillary sinus encroaching upon the right nasal cavity and left cervical lymph node (LN) swelling. An incisional biopsy carried out from the right maxillary sinus and LNs resulted in a diagnosis of combined SmCC with SqCC, staged as T4aN2cM0. Clinical examination revealed a sustained increase of antidiuretic hormone, hyponatremia with urinary sodium increase, and serum hypo-osmosis, resulting in SIADH. Water restriction to <1,000 ml/day was effective in improving sodium and osmotic imbalance. Curative treatment for the tumor was not prescribed due to the poor condition of the patient. Palliative treatment was administered and the patient succumbed to cachexia five months after histological diagnosis. The presence of SIADH may have marked implications for the treatment and prognosis of this disease. Neuroendocrine tumors, which cause syndrome of inappropriate secretion of antidiuretic hormone (SIADH), are potentially detectable in small-cell carcinoma (SmCC) of the lung. Up to 10% of patients with pulmonary neuroendocrine SmCC  develop Combined SmCC, which consists of SmCC and another histological type of carcinoma, accounts for only 5% of all SmCCs in the lungs . The comAn 80-year-old Japanese female presented with a four-week history of right nasal discharge, nasal obstruction and left neck swelling. The patient was a non-smoker with a history including left traumatic blindness, chronic renal disorder , hypertension and diabetes mellitus. Physical examination demonstrated an easy-bleeding tumor in the right nasal cavity  and swelmax) of 9.63  with low plasma osmolarity (271 mOsm/l), high plasma ADH levels , high urine sodium (39 mEq/l) and high urine osmolarity (301 mOsm/l)without dehydration. After water restriction to <1,000 ml/day, the sodium imbalance and osmotic status improved. To confirm the neuroendocrine characteristics of this tumor, immunohistochemistry using the rabbit polyclonal anti-human ADH antibody  was performed; however, the tumor was negative for ADH immunostaining (data not shown). Two weeks after the biopsy, the patient complained of progressive loss of vision in the right eye, induced by tumor extension to the right orbit. Curative treatments, including surgery, irradiation and/or chemotherapy, were not prescribed due to high performance status (grade 3) and severe complicated diseases. Instead, palliative treatments were administered. The patient succumbed to cachexia five months after diagnosis.Carcinoma of the nasal cavity and paranasal sinus is relatively rare, reported to comprise only 3% of all malignant head and neck diseases . Among sCombined SmCC is a histological group of carcinomas in which SmCC and non-SmCC, including SqCC or adenocarcinoma, are mixed. In contrast to head and neck cancers, the neuroendocrine type of combined SmCC has been frequently reported in SmCC of the lung. To the best of our knowledge, 17 cases of combined SmCC with SqCC in the larynx  and fourAccording to the 2005 WHO pathological classification of tumors of the head and neck , neuroenHyponatremia due to SIADH is a well known paraneoplastic syndrome and occurs in a variety of malignancies, particularly small-cell lung cancer 11–15%) , male gender and age >70 years (et al revealed that the development of SIADH does not correlate with the clinical stage or distribution of metastatic sites of SmCC (Notably, several reports have described that SIADH correlates with clinical factors, including prognosis and the development of metastases. Hansen 70 years . Further70 years . By cont of SmCC ; thus, tIn conclusion, this report presents the first known case of combined SmCC and SqCC in the sinonasal tract accompanied by SIADH. The presence of SIADH may have marked implications for the treatment and prognosis of this disease. More studies are required to establish the exact cause and appropriate treatments.


Pelvic and intra-abdominal Actinomycosis can be difficult to diagnose preoperatively and it may also mimic many other diseases, including malignancies. We present a patient with pelvic Actinomycosis probably caused by a long-standing intrauterine device (IUD). We emphasize the challenges in diagnostic process and stress that though a rare disease, intra-abdominal Actinomycosis should be suspected in cases with intra-abdominal mass of uncertain etiology. The early recognition may spare the patient from extensive surgical operation. Actinomyces are Gram-positive anaerobic bacteria that are normal inhabitants of the mouth and bowel. The organism only penetrates mucosal barrier after a tissue injury [Pelvic Actinomycosis is a rare disease with poorly understood pathogenesis. It commonly occurs in women with an intrauterine device of prolonged duration. e injury .Early diagnosis of abdominal Actinomycosis is of importance, as timely recognition enables conservative treatment with antibiotics, avoiding surgery . There iOur own case is a 45-year-old woman, who was referred to surgical ward due to an intestinal occlusion and tumor-like mass in the region of flexura lienalis. The patient had no preceding chronic disease or constant medication. She complained of increasing lower abdominal discomfort for 6 months with diarrhea and constipation. 9/L) and a mild decrease of hemoglobin value (107 g/L). Other laboratory parameters including C-reactive protein, leukocytes, CEA, CA 12-5, and CA 19-9 were normal. She had no weight loss or fever. A contrast-enhanced (i.v.) 16-detector row helical pelvic CT scan was performed  and peroral metronidazole (1.5 g/day) for 7 weeks. After that, peroral amoxicillin (1.5 g/day) was conducted for 6 months. Papanicolaou test revealed ASC-US, but after 6 months the results were normal.Macroscopic gross pathology examination of the formalin-fixed tissues revealed inflamed right ovary and tuba, focal damage of colonic mucosa, and wide peritoneal fibrosis and adhesions with pus. Histology showed purulent salpingo-oophoritis, necrosis of colonic mucosa, chronic peritoneal inflammation with abscess formation, granulation tissue, and diffuse fibrosis. Multiple Gram-positive cotton ball-like bacterial aggregates consistent with observed . Postope species . The patThe most difficult task in the management of abdominal Actinomycosis is to reach diagnosis before surgical operation takes place. Actinomycosis has been shown to mimic appendiceal tumor and abdominopelvic actinomycosis is associated with abdominal surgery and bowel perforation or occlusion . If diagWe report a case of pelvic Actinomycosisin a patient using the same IUD for over 20 years. The Actinomycosiscaused occlusion and appeared as a pelvic mass simulating malignancy and was surgically removed. Subsequently, the patient was treated with appropriate antibiotics with good result. Penicillin is the antibiotic of choice for treatment of Actinomycosis infection, whereas metronidazole appears to be most effective in treating associated infection of Gram-negative organisms. A prolonged treatment is required because of the poor penetration of antibiotics into the inflamed and fibrotic tissue .Actinomyces infection is 37 years old with IUD usage lasting longer than 8 years. A long-standing IUD can also cause perforation of the uterus [According to the literature, the typical patient at risk for IUD-associated e uterus . The cliLaboratory parameters commonly revealed anemia and leukocytosis. Imaging findings are nonspecific. Typically, a neoplastic or inflammatory process is suggested. CT is the most useful imaging study as it reveals the extent of the disease. Most common CT features at abdominopelvic  region iEven though intra-abdominal Actinomycosis is very rare, it should be included in a list of differential diagnoses, especially in any women using IUDs with abdominal pain and pelvic mass. This may spare the patient from unnecessary surgery.


Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder, characterized by painful swelling of muscles and connective tissue in the early years of life, consequently leading to ossification at a mean age of 4-5 years. We report FOP in a 2-year-old boy with palpable masses in the frontal and lower cervical paraspinal and left periscapular muscles.He was born with hallux valgus. Despite this hallmark, he was referred to the hospital with the primary diagnosis of hematoma, but further investigation indicated FOP. The patient was discharged from the hospital with non steroidal anti-inflammatory drugs (NSAID) and education of the parents. The importance of this case was that in spite of the early occurrence of the typical presentation of FOP for more than one year and the fact that the patient's mother was a physician who had consulted with many specialists, the diagnosis had been missed.This indicates that the general physicians, radiologists and other specialists' awareness and knowledge of FOP is insufficient. Fibrodysplasia ossificans progressiva (FOP) or myositis ossificans progressiva also known as Munchmeyer disease is a rare autosomal dominant condition that begins in childhood, but most patients have a new mutation. Genetic analysis revealed that the FOP gene located on chromosome 4 and mutation in this gene causes an over expression of a bone morphogenetic protein (BMP4). This is characterized by proliferation of connective tissue in voluntary muscles, fascias, tendons and ligaments resulting clinically as painful swelling of the muscles and connective tissue. This swelling subsides, then after approximately 6 months or more, ossification starts at some sites at the mean age of 4-5 years. Eventually, heterotrophic bone formation interferes with normal movement of the patient and most of them are confined to a wheel chair by the third decade. Mortality is related to restrictive lung disease (inability to expand the chest). Congenital malformations which are characteristically observed in the great toes at birth in almost all cases of FOP are the diagnostic hallmark.A 2-year-old boy presented with left periscapular and upper back paraspinal tender masses. He was referred from pediatricians to our hospital (Imam Reza) and subsequently admitted. He was the third child of 37-year-old mother delivered by cesarean section with good Apgar score, normal birth weight (4100gr), head circumference (HC) of 35 cm and length of 53 cm. His parents were non consanguineous and the similar disease was not observed in the family. The mother was a general physician and the father was an engineer. The patient’s sister and brother were healthy. In the past medical history, atopic dermatitis and lacrimal duct obstruction were seen at infancy. Growth and development was normal in the past two years.Present disease began one year ago with painful swelling of the forehead after a mild trauma. Similar swelling in the arm and shoulders started later subsiding spontaneously. At this admission, he had paraspinal (upper thoracic and lower neck) and periscapular muscle swelling which was painful, tender and indurate, without redness and inflammation over the skin . The shoIn physical examination of the limbs, bilateral hallux valgus was observed .He was referred with the primary diagnosis of multiple hematomas due to coagulation disorders, in which routine blood tests were normal twice and only hypochrome microcytic anemia was present. Ultrasonography showed solid hypoechoic masses with the largest diameter of 85 mm and thickness of 26 mm adhered to the scapula and spine. Sonographic differential diagnoses were mesenchymal tumor, bone tumor and aneurysmal bone cyst.The first CT scan indicated soft tissue masses, 25 Hounsfield Unit in density without bone destruction or calcification propounding mesenchymal or vascular masses .In spite of typical clinical and imaging findings for the early stage of FOP such as the congenital malformation of bilateral hallux valgus as a hallmark in this step, the diagnosis was not made and an unnecessary biopsy was performed on the periscapular mass. It may have lead to catastrophic disability. The pathology report showed proliferation of fibro-connective tissue with large islets of compact cartilage cells and lacunars cells with blades and specula of bone and osteoblastic activation, compatible with fibrodysplasia ossificans progressiva. The patient was discharged with non steroid anti-inflammatory drugs. Almost one year later, extensive ossification was detected in foot and neck radiographies , chest CGay patin first described this entity (FOP) in 1648 as a case who “turned to wood”. Prevalence is approximately 1/2.000.000 and over 700 cases have been reported in the world literature to date.7]The radiological evidence of ectopic ossification is not usually detected until 6 months (sometimes up to one year), after the appearance of masses so the mean age at onset of ossification has been reported as 5 years. Bony bridge causes skeletal contracture  3]3]In our case, the onset of symptoms with the typical anatomic pattern occurred at one year of age and spread at the second year. Ossification in the radiologic exam and CT scan was detected. In addition, ossification of thoracic soft tissue causes scoliosis and restrictive pulmonary disease. As a result of this event, the patient will be predisposed to chest infection and consequently death in the third or fourth decade prevalently. 8]8]FOP should be diagnosed as early as possible based on history, clinical and imaging findings. The guide of diagnosis is bilateral anomaly of the great toes present from birth, reported almost in 100% 6]9] of[9] of6][ of[9] ofRoentgenogram aid demonstrates minor osseous dysmorphism such as hallux valgus, clinodactyly, microdactyly, phalangeal shortening, metacarpal and metatarsal shortening, shallow acetabulum, short widened femoral neck, medial cortex thickening of the tibia, long small vertebra, fusion and segmentation of the spine, increased incidence of exostosis and long bone metaphyseal widening.When the ectopic ossification occurs, in plane radiography, bony bridges running from the bones to the muscles spreading also along the fascial planes are seen 9]..9]. FUS of early lesions may show sonolucent soft tissue mass and then when ossification occurs, echogenic and shadowing mass is detected.2]2] In early disease before ossification, CT demonstrates fascia plane edema and swelling and mesenchymal mass like lesion in the muscle that moderately enhances with IV contrast. In late disease after ossification and calcification, these findings could be seen in the muscles, fascia and connective tissue, especially near the bones.3]3] Although the diagnosis of FOP is more difficult when ectopic ossification is not present, MRI finding of a preosseous lesion is sufficient for accurate diagnosis of FOP in early stage 9]. In T. In T9].Although other soft tissue tumors mimic this MRI finding, MRI appearance of spreaded masses along the fascial planes is the single feature that confirms the diagnosis. Late MRI shows low T1 and T2 signal intensity associated with fibrous or calcified tissue.Bone scintigraphy with TC-MDP demonstrates heterotrophic ossification in the early stage and helps in the assessment of the extent and progression of the disease. In our patient, plain radiography, US and CT scan were performed. US showed hypoechoic soft tissue masses near the left scapula and spine corresponding to mesenchymal tumor or bone tumor and CT scan showed soft tissue masses with 25 HU in density that adhered to the bone without bone distraction or obvious calcification, propounded mesenchymal or vascular massesIn the early phase, these patients may be misdiagnosed with malignant tumors especially sarcoma, rhabdomyosarcoma, aggressive fibromatosis, post traumatic myositis, lymphadenopathy, TB, scleroderma, dermatomyositis of childhood, RA, nodular fasciitis and Klippel-Feil syndrome. 5]8]9][8][9]5][[9][8][9][8][9]5]In the late phase, the differential diagnoses are osteosarcoma, progressive osseous heteroplasia (POH), Albright hereditary osteodystrophy (AHO), osteoma cutis, ankylosing spondylitis, Still's disease, calcinosis interstitialis ossificans, Weber-Christian disease, pseudohypoparathyroidism, hypervitaminosis D, traumatic myositis ossificans and multiple exostosis. 8]10][10]8][10[10]8][10FOP has many differential diagnoses. This disease may be misdiagnosed in the early stage most importantly and most commonly as sarcoma and other cancers, aggressive fibromatosis and vascular masses.In summary, in review of case series and case reports and articles of diagnostic errors, and our case, we conclude that FOP is often misdiagnosed and the correct diagnosis happens very late. Early diagnosis prevents catastrophic harmful diagnostic and treatment procedures. For early correct diagnosis we propose:1. Family physicians, pediatricians and radiologists should be aware of the early feature of FOP before development of heterotrophic ossification. The most important and best key is great toe malformation which must be noticed clinically and radiologically as an early pattern of involvement.2. Early clinical and radiological diagnosis must lead to an MRI study or molecular genetic study, which depends on the accessibility and cost. Both of them proved the diagnosis.


Case Description. A 56-year-old male teacher presented with incomplete transverse syndrome due to several intradural extramedullary tumors at the level of lumbar vertebrae 1–3. The histological diagnosis at the time was atypical ependymoma. One year later, two additional tumors were removed at the L5-S1 vertebral level. For 12 years, the patient remained tumor free on followup. Fourteen years after the initial diagnosis, the patient presented with thoracic paresthesias due to two new extramedullary tumors in the C7-T1 and the T8-T9 vertebral levels. After complete removal of the tumors, a radiological survey revealed an intracranial lesion in the third ventricle. Five months later, an additional lesion recurrence was removed surgically. The most recent histological diagnosis revealed an atypical central neurocytoma. In retrospect, the previous tumors were reclassified as neurocytoma according to the additional immunohistochemistry evidence. Discussion. There is no standard adjuvant treatment regimen for atypical neurocytoma; therefore, the patient is currently under close followup. Modern histopathological diagnosis is essential in these cases. Potential routes for dissemination of the tumor should be considered upon first recurrence.We present an unusual case of a late recurrent central neurocytoma that was rediagnosed as an ependymoma and neurocytoma in accordance with changes in histological classifications.  Central neurocytomas are rare benign tumors of the central nervous system, characterized by their intraventricular localization. They are considered to arise from precursor cells of the septum pellucidum. They predominantly occur in young adults and generally have a favorable outcome, although cases with an aggressive clinical course and recurrences have been described. Historically, many of these lesions were regarded as either intraventricular oligodendroglioma or as ependymoma until detailed immunohistological clarification of their neuronal phenotypes was established. Neurocytoma was first described by Hassoun et al.  and is nIn the literature, only a few neurocytomas were reported with an extraventricular location, including cerebral hemispheres , thalamuHerein, we report a case of an atypical central neurocytoma with recurrent spinal dissemination over a period of 20 years.A 56-year-old Caucasian male teacher was first seen by his general practitioner in September 1991 complaining of back pain that was treated symptomatically. No imaging studies were arranged at that time. In April 1992, the patient developed sudden paraplegia of both legs, necessitating emergency surgery in an outside hospital. After a myelography and post-myelography-CT, three tumors located in the L1-3 vertebral level were removed via laminectomy L1-3. The paraplegia was caused by bleeding within the biggest tumor at the L1 level. Investigation of the removed tumors showed an “atypical” ependymoma. Immunohistochemical tests were not performed at that time. After removal, the neurological symptoms persisted, including saddle anesthesia, sphincter disturbance , and weakness of both legs. In December 1992, a second operation  was necessary to remove two newly appeared tumors which were located in the L5-S1 vertebral level. The histological study of the removed tumors also demonstrated an atypical ependymoma. Since that time, the patient has had no new clinical presentations. However, the symptoms mentioned previously persisted.In December 2006, the patient was admitted to our neurosurgical department because of numbness and pain radiating into the upper part of the body and into both arms. The spinal MR imaging showed several intramedullary spaces occupying lesions in the C7-T1 and the T8-T9 vertebral levels Figures . The larDue to repeated presentations of multiple ependymoma, a cranial MRI was obtained for the first time. This revealed a contrast enhancing intracerebral mass in the third ventricle . The craIn the MR imaging studies of the cranium and spinal axis in September 2010 and 2012, no new tumors were seen and progression of the cranial tumor was indiscernible.βIII Tubulin  or within the brain parenchyma (extraventricular neurocytoma). Atypical neurocytomas, which are neurocytomas with a MIB-1 LI >2% with/without anaplastic features , 16, havDespite the usually benign nature of this tumor, eight cases with ventricular or spinal dissemination have been reported in the literature . Sharma Pitfalls of the pathological diagnosis consist of morphology very similar to ependymoma, which also contains perivascular acellular areas, but subtly fewer monomorphic nuclear shapes and a more fibrillar matrix. In this case, the first diagnosis of an ependymoma which is much more likely in this location was based only upon morphology. However, using immunohistochemistry, it was possible to reach the correct diagnosis of neurocytoma. Paraganglioma represents a further differential diagnosis of spinal neurocytoma. Immunohistochemical differentiation between these two entities is considerably more difficult since both tumors show expression of neuronal markers; however, paraganglioma should also show expression of chromogranin and at least some more mature neural cells. Furthermore, paraganglioma usually contains a morphological “Zellballen” pattern and sustentacular cells at regular intervals with positive staining of S100 . These sIn summary, the case is of interest to the readership due to its unusual course, intracerebral spread, and long duration with only incidental pathology, as well as its ambiguous histological interpretation over the years and neuropathological classification. Early cranial imaging studies are considered upon repeated spinal recurrences to exclude a primary “seeding” tumor.


Upper oesophageal adenocarcinoma is a rare malignancy that occurs in the ectopic gastric mucosa, in the upper part of the oesophagus. Only 29 cases so far have been reported. We report the 30th case in a 50-year-old Asian female presenting with difficulty in swallowing. Upper oesophageal malignancies  are usually of the squamous cell type. Although glandular heterotropia of the upper digestive tract is frequently observed during upper gastrointestinal endoscopy (in 0.26%–4.9% of cases), it has rare malignant potential. Adenocarcinoma of the upper oesophagus is not commonly observed and only 29 cases have been reported so far –8. We arThe 50-year-old patient, complained of progressive difficulty in swallowing for four months, having had only liquid intake at the time of our workup. There was no history of tuberculosis, hypertension, chronic obstructive pulmonary disease, diabetes, any other chronic ailment, or significant family history. Blood biochemistry, including liver and kidney functions, was within normal limits. Ultrasound of the patient’s abdomen was normal. A barium swallow  revealedOccurrence of adenocarcinoma is rare and has been explained in the literature on the basis of ectopic gastric type mucosa in the upper part of the oesophagus. The incidence of ectopic gastric mucosa is 4.0% in males and 2.9% in females [The age at presentation varies from 37 to 90 years with a mean age of 63 years. Out of 30 cases, only three are female with a male to female ratio of 9:1, which is a little higher than the ratio for overall oesophageal malignancies (3.5:1) . In gene


Pediatric GISTs differ from CT-associated GISTs solely by the absence of other components of the triad and are completely different from sporadic GISTs of the adult. Both, pediatric and CT-GISTs, metastasize frequently to regional lymph nodes (29%) and are usually wild type (WT) for common KIT-/PDGFRA mutations.We compare one new CT GIST with two pediatric/young adult multifocal gastric GISTs presenting with lymph node metastases. We put special focus on histomorphological growth pattern in the primary tumors and in the metastases.The two cases of pediatric/young adult GIST without the other components of CT showed all the features of the triad: female gender, young age, multifocal antral-based gastric GIST with biphasic histological growth pattern, lymph node metastases, hypercellularity and WT status for common KIT-, PDGFRA- and B-RAF mutations.Pediatric/CT-associated GISTs and sporadic GISTs of the adults differ significantly from each other with regard to patients' age, gender, tumor localisation, histomorphological growth pattern, mutational status and risk for metastasis. Our cases of pediatric/young adult GISTs show all characteristics of CT except for the absence of other components of the triad.forme fruste of the CT. Thus, these patients need to be regularly examined for the development of extraadrenal paraganglioma or pulmonary chondroma.Therefore these GISTs are probably not sporadic, but may represent either early manifestation or  Howevevalence -8 and thevalence ,9,10. GIevalence .In 1977 J. Aidan Carney first described the association of gastric epithelioid leiomyosarcoma  with pulmonary chondroma and functioning extraadrenal paraganglioma of unknown origin, which is today known as CT ,13.nd and 3rd decades [CT is rare with approximately 120 published cases worldwide to date, usually affects females (88%) in their 2 decades ,15 and o decades ,15. The  decades . The gas decades ,18 and t decades ,20 contr decades -22. Ther decades . Despite decades .In this study, we describe two new cases of multifocal gastric GIST with lymph node metastases in pediatric/young adult females and compare the findings with that of a new CT-GIST with special focus on histomorphological growth pattern, mutational status and the pattern of metastasis.The first patient, a 15-year-old girl was admitted to the hospital with anaemia caused by upper gastrointestinal bleeding. An antral-based multifocal gastric tumor, suspicious for GIST, was detected in the greater curvature of the stomach. The largest tumor nodule measured 7 cm in diameter and showed mucosal ulceration. Furthermore multiple liver metastases (maximum diameter: 4 cm), were detected preferentially in the left lobe. After confirmation of the diagnosis GIST by open biopsy the patient underwent therapy with imatinib (400 mg once a day × 9 weeks followed by 2 × 400 mg/d × 2 weeks). No change in tumor size was observed and the patient underwent gastrectomy and regional lymphadenectomy. The patient has been since then (for now 6 years) on different tyrosine kinase inhibitors including motesanib, sunitinib and nilotinib with only slight progression in the size of liver metastases .2). In the liver metastases the tumor cells demonstrated a paraganglioma-like growth pattern with small compact nests, whorls and "Zellballen"-like aggregates of epithelioid tumor cells. Furthermore, histological examination of a 2.5 × 1.8 × 1.2 cm intrathoracic paraaortal nodule thought to represent lymph node metastasis revealed a paraganglioma leading to the diagnosis of incomplete CT with synchronous gastric GIST and extraadrenal paraganglioma.The primary tumor showed biphasic histomorphological growth pattern with both, spindled and epithelioid cells. Additionally, hypercellularity as well as plasmacytoid character of the tumor cells was seen. Mitotic count was 5/50 high power fields  and PDGFRA  as well as for B-RAF exon 15 V600E mutations. However, further examination disclosed a rare PDGFRA exon 10 polymorphism  that is present on the DNA level but does not lead to alterations on the protein level.The second patient, a 13-year-old girl presented with long-lasting anaemia caused by upper gastrointestinal bleeding and worsening general condition. As the initial therapy with ferrous sulphate showed no benefit, ultrasound of the abdomen and magnetic resonance tomography were performed. During these examinations, an 8.1 × 7.1 × 5.2 cm bulky mass attached to the lesser curvature of the stomach as well as multiple hyperdense areas in both lobes of the liver (max. 2.2 cm) were discovered. Subsequent gastric endoscopy revealed an exophytic bleeding multilobular gastric tumor with mucosal ulceration. By positron emission tomography the hyperdense areas in the liver were confirmed as metastases of the gastric tumor. The patient then underwent neoadjuvant imatinib therapy followed by 4/5 resection of the stomach with gastrojejunostomy and regional lymphadenectomy of the lesser curvature. Because of the multiplicity of the liver metastases surgeons decided against resection. The patient has been since then for 2 years on imatinib (400 mg/d) with no major change in size of liver metastases .Histological examination of the gastric specimen revealed an 8 cm multifocal gastric GIST with mitotic count of 4/50 HPF . Furthermore multiple regional lymph node metastases with epithelioid growth pattern were verified. The primary tumor showed biphasic histological growth pattern. Immunohistochemistry showed distinct cytoplasmic and membranous expression of CD117 and CD34 figure .The tumor was WT for KIT exons 9, 11, 13 and 17, PDGFRA exons 10, 12, 14 and 18 as well as for B-RAF exon 15 mutations.The third patient, a 40-year-old woman currently presented with liver and lymph node metastases (celiac trunk).Exploration of her medical history uncovered that the patient underwent partial gastrectomy 17 years ago (23 years old at that time) because of a gastric mesenchymal tumor with hepatic and peritoneal metastasis. At that time the tumor was classified as gastric leiomyosarcoma or malignant stromal tumor of the stomach and the patient was treated with high-dose chemotherapy and autologous stem-cell transplantation . leadingUnfortunately slides or paraffin blocks of the primary gastric tumor were not available any more for revision, but the histological description found in the medical report was consistent with a GIST. The patients young age, female gender, tumor localisation, mutational status , (histo)morphological growth pattern , immunohistochemistry findings and the pattern of metastasis (lymph node and liver metastases) are consistent with the previous two cases figure . Up to nth and 7th decade of life [Sporadic GISTs, usually affecting adults in the 6 of life -8, are r of life ,9,10. Cu of life  and lackHowever, current understanding suggests that sporadic pediatric GISTs represent a tumor entity distinct from common GISTs in adults but with striking similarity to CT-GISTs.WT pediatric GISTs are distinguished from CT-GISTs merely by the absence of other triad components in the latter. In contrast to most of the sporadic GISTs of the adults, both CT-GISTs and pediatric GISTs are WT for common mutations of the receptor tyrosine kinase genes KIT and PDGFRA ,18. PediSimilarly, our two cases of pediatric/young adult GIST without other components of CT (case 2 and 3) showed all the features of the CT-GISTs: young age, female gender, antral-based gastric GIST, multifocal tumor growth, biphasic histological growth pattern, hypercellularity, WT status for common KIT-, PDGFRA- and B-RAF mutations and presence of lymph node metastases table .forme fruste of the CT. The time interval from diagnosis of GIST until the appearance of other component/s of the CT averages ≥ 8 years but may be as long as three decades [Thus, patients diagnosed with WT pediatric GISTs could either represent an early manifestation of CT or a  decades ,27. TherThe authors declare that they have no competing interests.Written informed consent was obtained from the patient/the patients parents for publication of this report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.All authors read and approved the final manuscript.


Malignant fibrous histiocytomas have been periodically reported as the primary tumor in various organs including the urinary bladder, and is the second most frequent sarcoma of the urinary tract in adults. This report discusses a case of the well established diagnosis of a malignant fibrous histiocytoma of the bladder occurring as a post-radiation cancer after the treatment of a cervical carcinoma. Our findings support those of many previous studies and make the view of the nature of the disease clearer.We report the case of a 54-year-old Thai woman who had been treated with radiation therapy for cervical cancer, who presented to our facility with urinary incontinence. Initially, our patient was diagnosed as having a high-grade urothelial carcinoma. Subsequent radical surgery rendered the final pathological diagnosis, confirmed histologically and immunohistochemically as malignant fibrous histiocytoma, with clinical and pathological staging of T4b N0 M0. Adjuvant chemotherapy was provided for our patient.This type of malignancy is very aggressive and easily misdiagnosed due to its rarity. Therefore, in a patient with a prior history of irradiation in the pelvic area, this should be considered as a differential diagnosis to ensure early correct diagnosis and treatment. While commonly found as a soft tissue tumor and considered as the most common soft tissue sarcoma in adults over the age of 40 years , malignaA 54-year old Thai woman presented to our facility with a two-month history of urinary incontinence induced by coughing.She had a history of cervical squamous cell carcinoma, stage IIb, which was treated 15 years previously with radiation therapy. Her six-month interval follow-up pelvic examination and cervicovaginal PAP smears had revealed negative findings and the disease had been considered as in complete remission by the attending radiologist. She was first referred to our gynecology department to find out the cause of her incontinence. On pelvic examination by a gynecologist, her uterine cervix was found to be atrophic with an extraluminal compression at the anterior vaginal wall, covered by an intact vaginal mucosa. Laboratory investigation results revealed mild hematuria (3 to 5 red blood cells per high-power field) and mild anemia . Eventually, our patient was referred to our urology department for further management.During the process of the Q-tip test, the applicator was passed with difficulty and deviation to the right. Cystoscopy was performed. Her urethra showed no meatal stenosis and unremarkable mucosa, but the device was passed with difficultly due to external compression from the left side of the proximal urethra. Her bladder had a large sessile mass on the left lateral wall with extension to the bladder neck. Both ureteral orifices could be identified clearly with unremarkable appearance and efflux. Clinically, the differential diagnosis included recurrent cervical cancer, bladder cancer and retroperitonal sarcoma. Urine cytology and transurethral resection of the bladder tumor were performed, with a negative cytology report and a pathological diagnosis of high-grade urothelial carcinoma. A computed tomography scan demonstrated a heterogenous enhancing mass, 5.3 × 6.5 × 6.0 cm, at the left inferior-lateral wall of the bladder with invasion of the uterus and attachment to the left pelvic side wall, as shown in Figures Gross pathological examination revealed a large mass on the left lateral wall that measured 7.0 cm across its greatest dimension. The surface of the tumor on the mucosal side was smooth. The mass obviously protruded from the serosal surface of the bladder and was attached to the uterus, but could still be separated from the organ with ease.Microscopically, the overlying urothelium was intact with no dysplastic change Figure . The undPathological findings from other organs, including the uterine cervix, were unremarkable. No residual cervical dysplasia or neoplasm was detected. All 12 lymph nodes showed no neoplasm. The disease was clinically and pathologically staged as T4b N0 M0.After surgery, our radiation therapist advised not providing additional radiation therapy in the pelvic area. Our patient, therefore, received a course of adjuvant chemotherapy (doxorubicin and ifosfamide). However, two months after the surgery, a bony metastasis was detected at the L5 spine level, and palliative radiation therapy in that area was suggested. Despite aggressive surgical treatment along with adjuvant chemotherapy and palliative radiation therapy, our patient developed further locally recurrent disease with invasion to the right pubic bone and rectum. At this time, a multidisciplinary approach for palliation of our patient's symptoms is still in process.Our patient had received a full course of radiation therapy in her pelvic area and, hypothetically, the bladder sarcoma developed from radiation-induced genetic alterations. The mass developed rapidly and behaved aggressively.From her clinical history, for purposes of retrospective study, our patient's presentation was not typical for a conventional urothelial carcinoma, as the mass was large while producing negative urine analysis and negative urine cytology results. Even though squamous cell carcinoma of the bladder is strongly associated with chronic irritation or inflammation, it is still unlikely due to the laboratory results and no strong association with radiation has been reported. The history of radiation exposure produced a suspicion of mesenchymal tumor origin. The reported incidence of post-irradiation sarcoma ranges from some few per thousand to nearly 1% . HoweverFew cases of malignant fibrous histiocytoma of the urinary bladder have been reported so far . After aMany authors have reported that this type of malignancy is very aggressive, with a high local recurrence rate and a requirement for aggressive treatment with a combination of radical surgery and systemic chemotherapy ,7,8, whiIn summary, malignant fibrous histiocytoma is considered as a rare malignancy of the urinary bladder. However, some of the previously diagnosed high-grade carcinomas might actually be this type of sarcoma. We suspect that the radiation exposure might be a contributing factor to this rare mesenchymal neoplasm. Correct diagnosis requires immunohistochemical studies with a large panel of antibodies in close correlation with histological and cytological features .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.The authors declare that they have no competing interests.PR analyzed and interpreted the data from our patient regarding the neoplasm, and performed the operation. TN performed the histological examination and interpretation of immunohistochemical study results, and was a major contributor to writing the manuscript. All authors read and approved the final manuscript.


The incidence of hernias is increased in patients with alcoholic liver disease with ascites. To the best of our knowledge, this is the first report of an acute rise in intra-abdominal pressure from straining for stool as the cause of a ruptured umbilical hernia.An 81-year-old Caucasian man with a history of alcoholic liver disease presented to our emergency department with an erythematous umbilical hernia and clear, yellow discharge from the umbilicus. On straining for stool, after initial clinical assessment, our patient noted a gush of fluid and evisceration of omentum from the umbilical hernia. An urgent laparotomy was performed with excision of the umbilicus and devitalized omentum.We report the case of a patient with a history of alcoholic liver disease with ascites. Ascites causes a chronic increase in intra-abdominal pressure. A sudden increase in intra-abdominal pressure, such as coughing, vomiting, gastroscopy or, as in this case, straining for stool can cause rupture of an umbilical hernia. The presence of discoloration, ulceration or a rapid increase in size of the umbilical hernia signals impending rupture and should prompt the physician to reduce the intra-abdominal pressure. The anterior abdominal wall has multiple areas of potential weakness  which, when exposed to acute or chronically elevated intra-abdominal pressure, are prone to weaken and allow the formation of various hernias . The umbIntra-abdominal pressure varies in both an acute and a chronic manner. During normal physiology acute variations in intra-abdominal pressure mainly follow changes in body position and patient activities -4. In heAn 81-year-old Caucasian man, with a background history of alcoholic liver disease, presented acutely via our emergency department, with an erythematous umbilical hernia and clear, yellow discharge from the umbilicus. Clinical examination showed signs of decompensated liver disease, including asterixis, spider naevi, a distended abdomen with shifting dullness, fluid thrill and an erythematous umbilical hernia. On straining for stool, after initial clinical assessment, our patient noted a gush of fluid and evisceration of omentum from the umbilical hernia Figures .An urgent laparotomy was performed, using povidone-iodine solution for skin preparation via a midline incision, with excision of the umbilicus and devitalized omentum. Of note, there was evidence of recanalization of the umbilical vein. A full examination of the abdominal viscera was performed, and samples of ascitic fluid sent for cytological, biochemical and microbiological analysis. The liver was noted to be nodular, shrunken and sclerotic with generalized fibrinous exudate lining the coelomic cavity. His post-operative α-fetoprotein was 798 IU/mL. The abdominal fascial edges were re-apposed with interrupted 1/0 polypropylene sutures, with clips to the skin. The ascitic fluid serum-ascites albumin gradient was >1.1 g/dL, and showed increased ascitic protein level (>2.5 g/dl). Cytology was negative for malignant cells.The incidence of hernias is increased in patients with alcoholic liver disease with ascites . The firCurrent thinking suggests that there is a dynamic adaptive change which takes place in all organisms in response to a chronically elevated intra-abdominal pressure, principally as adaptations to the constitutional properties of the abdominal cavity. This occurs in order to maintain normal functioning ,14-16. TThere has been considerable debate in the literature as to the timing of umbilical hernia repair in patients with alcoholic liver disease and ascites. Older studies, in particular by Baron , describUltimately, more evidence is required, and cases should be considered individually, to determine the most effective timing of umbilical hernia repair.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.The authors declare that they have no competing interests.DWG conceived the manuscript, collected the data, took the photographs, wrote and revised the manuscript. JER collected data and reviewed the manuscript. MS conceived and reviewed the manuscript. PCN wrote the manuscript and performed a final review. EE performed a final review. All authors read and approved the final manuscript.


In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as Balo concentric sclerosis and a glioma. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis.A 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic.After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained.We hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system. Tumefactive demyelinating brain lesions present a diagnostic challenge, because their clinical, radiologic, and even histologic features may complicate the identification of their true nature. This often leads to invasive and costly procedures, which frequently yield non-diagnostic results. We report a patient with a right parietal white matter lesion, who presented a serious diagnostic dilemma, as the lesion was difficult to differentiate between a rare demyelinating disease such as Balo concentric sclerosis (BCS) and a glioma. The characteristic magnetic resonance findings of the case, its acute onset, and its clinical improvement after corticosteroid therapy finally set the diagnosis of BCS. The risks of the stereotactic procedures that led to the misdiagnosis of BCS are discussed.1- and hyperintense on T2-weighted images  demonstrated a large (2.1 cm), well-demarcated hypodense lesion in the right parietal lobe, without perifocal edema. Magnetic resonance imaging (MRI) without contrast showed a hypoisointense concentric mass on Ts Figure . After cs Figure . These MN-acetylaspartate and an increase in choline, lipids, and lactate. The findings were not specific and were consistent either with an acute demyelinating lesion or with a low-grade glioma. Ten days later, the patient showed moderate clinical improvement and continued with oral steroid treatment.Somatosensory evoked potentials (SSEPs), serum, and cerebrospinal fluid analysis were normal. Human immunodeficiency virus (HIV) and antinuclear antibody (ANA) tests were negative. The chest radiograph (CXR) was normal. Under the presumptive diagnosis of BCS, the patient received high-dose intravenously administered methylprednisolone (500 mg/day for ten days). The subsequent proton MR spectroscopy (MRS) revealed reduction in A brain CT-guided stereotactic biopsy was scheduled to establish the diagnosis, as MRS and laboratory findings were not specific. Four specimens within and from the periphery of the lesion were taken. Histologic examination failed to show the presence of a significant number of histiocytes, foamy macrophages, or myelin loss that would otherwise be expected in Balo sclerosis. It showed, however, mild to moderate nuclear atypia, whereas Ki-67 immunostaining was positive in 1% to 2% of the nuclei. The pathologist commented that the findings were suggestive of a grade II astrocytoma.2 images , acute disseminated encephalomyelitis, or other rare demyelinating diseases, such as BCS and Marburg type. BCS is a rare demyelinating disease considered to be an acute variant of MS, appearing in young adults and typically following a fulminant course . It show pattern . This tyin vivo markers of disease progression. MRS in BCS may show reduction of N-acetylaspartate and increase in choline and lipids, reflecting axonal destruction and an elevation of lactate resonance due to local ischemia from the ongoing inflammatory process [Advanced neuroimaging can provide important  process . These r process ,4 are no process ,4,5. TheA stereotactic biopsy and histologic examination of the lesion is the final diagnostic approach in equivocal cases . It is sOccasionally, the biopsy is non-diagnostic or dispensable . In our In our case, histopathologic findings from the biopsy were misleading.However, the characteristic concentric rings of demyelination alternating with myelination on MRI, the patient's considerable clinical improvement after steroid therapy, and the signal differences in follow-up MRI scans established the diagnosis of BCS.In serial MRI scans, the concentric ring enhancement of BCS is expected to fade away centrifugally, until it appears as a low-signal, non-enhancing lesion, typical of a chronic demyelinating plaque , as in oDemyelinating diseases can mimic brain neoplasms clinically, radiologically, and histopathologically. BCS is a rare demyelinating disease, which can manifest as a mass lesion. The typical concentric pattern on MR images, along with clinical features, can lead to accurate diagnosis and treatment. For suspected cases, it is advisable to use steroid therapy or undergo serial MRI examinations. However, in borderline cases, pathologic evidence is beneficial to a final diagnosis.BCS: Balo concentric sclerosis; CT: computed tomography; MRI: magnetic resonance imaging; MRS: proton magnetic spectroscopy; MS: multiple sclerosis.The authors declare that they have no competing interests.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.MG collected and analyzed all patient data, conducted a literature review, and was a major contributor in writing the manuscript. LS collected and analyzed data related to the patient's stay in the neurosurgery department and collected the follow-up information. EB and DS provided clinical details and technical input, revised the manuscript, and performed changes throughout the manuscript. All authors read and approved the final manuscript.


Bevacizumab is a monoclonal antibody against vascular endothelial growth factor. It is widely used in the treatment of metastatic colorectal cancer. It has some specific side effects including severe bleeding, wound healing problems, gastrointestinal perforation, proteinuria and hypertension.We present the case of a 65-year old Asian man with synovial metastasis of the knee who experienced intraarticular hemorrhage after bevacizumab treatment. He presented with monoarthritis of the left knee.Bevacizumab-related hemorrhage can cause serious morbidity and unusual sites of hemorrhage may be seen. Colorectal cancer is the third most common cancer in both men and women. should be: "It is also the third most common cause of cancer-related deaths . SurvivaBevacizumab is the first angiogenesis inhibitor that binds and inactivates all isoforms of vascular endothelial growth factor (VEGF). It has some specific side effects including severe bleeding, wound healing problems, gastrointestinal perforation, proteinuria and hypertension. Hemorrhages are usually related to metastasis or primary tumor itself. No case of intraarticular bleeding due to bevacizumab has been reported previously in the literature. We report the case of a man with knee joint metastasis from colon adenocarcinoma who experienced intraarticular hemorrhage subsequent to bevacizumab treatment.3  and C-reactive protein of 0.45mg/dl . The left knee was aspirated and yielded hemorrhagic fluid. He had no history of a traumatic event or use of warfarin, heparin or acetylsalicylic acid. He had no history of hemorrhagic diathesis. Coagulation parameters were normal. A magnetic resonance image (MRI) revealed synovial metastasis of the knee . Oxaliplatin- or irinotecan-based chemotherapy could not be given due to his poor Eastern Cooperative Oncology Group (ECOG) performance score. The lung lesion was resected after partial response to six cycles of chemotherapy. He discontinued therapy and did not come to control visits. After three months he presented to our clinic with weight loss, weakness and pain in his left knee. His carcinoembryonic antigen was increased and positron emission tomography-computed tomography (PET- CT) revealed multiple liver, lung and bone metastasis. Bevacizumab and capecitabine was restarted at the same dose and frequency as he had received previously. After the first course of this regimen, he presented with monoarthritis in the left knee. Physical examination revealed tenderness, swelling and reduced mobility. Laboratory studies revealed a hemoglobin value of 10.2g/dL; white blood count of 7800/mmThis is the first case of bevacizumab-related intraarticular bleeding reported in the literature. Intraarticular hemorrhage was associated with the synovial metastasis of the left knee. Synovial metastasis is a rare entity; it is usually seen in solid tumors. Only a few reported cases of metastatic adenocarcinoma to the synovium can be found in the literature -6. The dBevacizumab is the first angiogenesis inhibitor that binds and inactivates all isoforms of VEGF. The most common side effects are hypertension, proteinuria, epistaxis, gastrointestinal symptoms and poor wound healing. Infrequent serious adverse events include gastrointestinal perforation, arterial thrombotic events and hemorrhage . Severe The mechanism underlying bevacizumab-related bleeding is not fully understood. It is probably associated with the inhibition of VEGF signaling. VEGF stimulates endothelial cell proliferation, promotes endothelial cell survival and helps to maintain the vascular integrity. Inhibition of VEGF can reduce the regenerative capacity of endothelial cells . HoweverBevacizumab-related hemorrhage can cause serious morbidity even mortality. Unusual sites of hemorrhage may be seen. Clinicians should examine the symptoms of monoarthritis carefully in patients treated with bevacizumab and should not continue the therapy until they rule out intraarticular hemorrhage.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.VEGF, Vascular endothelial growth factor; PET-CT, Positron emission tomography-computed tomography.The authors declare that they have no competing interests.MU and HSC designed the work; MU wrote the paper. MU, HSC, SSG, BS, MO and HB followed up with the patient. MU and HSC reviewed the article. All authors read and approved the final manuscript.