Ameloblastoma is a benign epithelial odontogenic tumor. It is often aggressive and destructive, with the capacity to attain great size, erode bone and invade adjacent structures. Unicystic ameloblastoma is a rare odontogenic lesion, with clinical, radiographic and gross features of jaw cysts. The lesion histologically shows typical ameloblastomatous epithelium lining part of the cyst cavity with or without and/or mural tumor growth. Unicystic ameloblastoma usually presents in posterior mandibular ramus region, while it is rare and atypical in posterior maxillary region. .We report a case of 16 year old Kosovar male, Albanian ethnicity, who presented with a swelling located in right maxillary region. Clinical examination revealed a painless swelling extending from the maxillary right central incisor to the maxillary right first molar tooth. Panoramic radiograph disclosed a well corticated unilocular radiolucent lesion approximately 5 × 5 cm in diameter which was in contact with the roots of the teeth present inferiorly and with the maxillary sinus superiorly. Maxillary right canine impaction was noted and unerupted lateral incisor tooth was present inside the radiolucency. Preoperative diagnosis of the lesion was made as dentigerous cyst based on the age of the patient, location of the swelling, clinical and radiographic findings, but the unicystic ameloblastoma was also taken into consideration. The patient was treated by surgical enucleation of the lesion and extraction of lateral incisor tooth which was present inside the lesion. The histopathological examination of the lesion revealed confirmed finding for unicystic ameloblastoma mural form. No recurrence was observed in 1 year follow-up.Maxillary region is considered a rare and atypical location for unicystic ameloblastoma. We emphasize the importance of differential diagnosis of an odontogenic lesion with common clinical and radiological features that will impact the treatment planning and follow up. As oral health providers we should be aware that the unilocular radiolucencies may be unicystic ameloblastoma. Ameloblastoma is a local invasive tumor which originates from remnants of the dental lamina and odontogenic epithelium and it accounts for only 1 % of all oral tumors . Based oUnicystic ameloblastoma (UA) as a distinct entity was first described by Robinson and Martinez in 1977 . UA refeThe purpose of this article is to present a rare case report of UA in atypical location into the right anterior and premolar maxillary region together with two impacted teeth.A 16-year-old Kosovar male, of Albanian ethnicity referred to our department with the chief complaint of painless swelling on the right cheek. The patient described painless swelling 3 months before visit as the initial observation, but has shown the enlargement in the last few weeks. Medical history data of the patient revealed no systemic disease or other health problems.Facial asymmetry was present on the right side on clinical extraoral examination.The skin overlying the swelling was normal. The extraoral swelling was well circumscribed, painless and approximately 5 × 5 cm in size. The consistency was hard and without fluctuation.Intraoral examination revealed a painless swelling in the right maxillary vestibule extending from the maxillary right central incisor to the maxillary right first molar. The intraoral swelling was firm, non tender, covered with normal mucous membrane.Egg shell cracking was present buccally but not palatally Fig. . AspiratPreoperative diagnosis of the lesion was made as dentigerous cyst based on the age of the patient, location of the swelling, impacted right canine and unerupted lateral incisor located inside the lesion, aspirated thick juicy yellow liquid and visible cholesterol crystals, but the UA was also taken into consideration. The surgical operation including total enucleation of the cystic lesion together with impacted tooth was made Figs. , 4. AfteThe pathological examination revealed UA, mural form. Infiltrating islands of atypical basaloid cells with peripheral palisading were present. Separation artifact of peritumoral stroma was evident Fig. .Fig. 5InThe nature of the tumor was explained to the patient and we advised the patient to regard regular follow-up visits. There were no signs of recurrence since 2 years after the operation.UA is a rare type of ameloblastoma, accounts for about 6 % of all ameloblastomas. It affects mandible more often than maxilla and in about 50 % of the cases occur in the second decade of life . It is pThe lesion is usually found in association with the crowns of mandibular third molar teeth, but can be seen also in interradicular, periapical and edentulous regions as well . In our Ackermann et al. 1988) [88 [9] anRadiographically there are 2 main patterns: Unilocular and multilocular , 11.Based on histological examination, to diagnose a lesion as unicystic ameloblastoma, the minimum criteria is the demonstration of presence of a single cystic sac lined by odontogenic ameloblastomatous epithelium which is seen only in focal areas .I.Luminal UA ;II.Intraluminal/plexiform UA ; andIII.Mural UA  .There are different classifications of unicystic ameloblastoma. Based on the clinicopathologic study of 57 cases of unicystic ameloblastoma, Ackerman’s classification into three histologic groups is as follows:According to this classification, our case study belongs to Group III.There is another grouping by Philipsen and Reichart  which deSubgroup 1. Luminal UA;Subgroup 1.2. Luminal and intraluminal;Subgroup 1.2.3. Luminal, intraluminal and intramural; andSubgroup 1.3. Luminal and intramural.UA is considered to be a less aggressive form of ameloblastomas that can be successfully removed by simple enucleation or other less aggressive surgery .The use of Carnoy’s solution to decrease the risk of recurrence after conservative surgical treatment of UA’s was initially suggested by Stoelinga and Bronkhorst in 1988 . Also itEvery unilocular radiolucency of the jaw should be closely monitored and examined since UA shares significant clinical and radiographic similarities with odontogenic cysts and tumors. Neither the incisional biopsy may be able to reflect the true nature of the lesion nor the aspirational cytology. Long-term follow-up is mandatory because of the recurrence risk of unicystic ameloblastoma, which may occur after a long time.


Historically, local control of recurrent sarcomas has been limited to radiotherapy when surgical re-resection is not feasible. For metastatic carcinomas to the bone or soft tissue, radiotherapy and some interventional radiology treatment along with other systemic therapies have been widely advocated due to the possibility of disseminated disease. These techniques are effective in alleviating pain and achieving local control for some tumor types, but it has not been effective for prolonged local control of most tumors. Recently, cryoablation has been reported to have satisfactory results in lung and liver carcinoma treatment. In this study, we analyzed the clinical outcome of CT-guided cryoablation for malignant bone and soft tissue tumors to elucidate potential problems associated with this procedure.n = 4), dedifferentiated liposarcoma (n = 2), myxofibrosarcoma (n = 2), chordoma (n = 1), hepatocellular carcinoma (n = 1), and thyroid carcinoma (n = 1). Cryoablation methods, clinical outcomes, complications, and oncological outcomes were analyzed.Since 2011, 11 CT-guided cryoablations in 9 patients were performed for locally recurrent or metastatic bone and soft tissue tumors  at our institute. The patients’ average age was 74.8 years (range 61–86) and the median follow up period was 24.1 months (range 5–48). Histological diagnosis included renal cell carcinoma , and the average number of probes was 2.4 (range 2–3). Complications included 1 case of urinary retention in a patient with sacral chordoma who underwent prior carbon ion radiotherapy, 1 transient femoral nerve palsy, and 1 minor wound complication. At the final follow up, 4 patients showed no evidence of disease, 2 were alive with disease, and 3 died of disease.Reports regarding CT-guided cryoablation for musculoskeletal tumors are rare and the clinical outcomes have not been extensively studied. In our case series, CT-guided cryoablation had analgesic efficacy and there were no cases of local recurrence post procedure during the follow-up period. Although collection of further data regarding use of this technique is necessary, our data suggest that cryoablation is a promising option in medically inoperable musculoskeletal tumors. Recurrent and metastatic bone and soft tissue tumors pose significant problems because of their refractory nature. Re-resection of local sarcoma recurrence has the potential to cure the patient, but it is often difficult to treat because it is almost impossible to discern the extent of tumor infiltration after multiple operations . MetastaCryoablation is a therapeutic procedure wherein hollow cryoprobes are inserted into the tumor, causing a decrease in the local temperature below 40° C. Freezing for longer than 1 min causes cell death and subsequent thawing further ensures disruption of the cellular integrity . Its effThe purpose of this study was to assess the feasibility, safety and efficacy of cryosurgical ablation for locally recurrent sarcoma and metastatic carcinomas.n = 5), retroperitoneum (n = 2), thigh (n = 2), rib (n = 1), and sacrum (n = 1). Serial MRI was analyzed post cryoablation after 1, 3, 6, and 12 months. Lesions were defined as locally controlled if there was no focal enlargement according to the Response Evaluation Criteria in Solid Tumors (version 1.1) [The inclusion criteria included limited symptomatic metastasis, soft tissue recurrence or recurrent skeletal disease with either osteolytic or mixed osteolytic - osteoblastic features. All the cases were contraindicated for other treatments, such as surgery or radiotherapy, due to comorbidity or prior irradiation. Specifically, the reason behind cryoablation for sarcoma cases were as follows: chordoma patient had underwent previous carbon ion radiation therapy, dedifferentiated liposarcoma of the retroperitoneal was an 86-year-old male who was determined to be inoperable due to his comorbidity, and myxofibrosarcoma patient had undergone 4 previous surgeries and refused further aggressive treatment such as an amputation. Osteoblastic lesions were excluded because local anesthesia was deemed insufficient to establish a tract for placing the ablation device. All lesions that were adjacent to vital structures such as the nerves and bowels were excluded, unless they could be displaced by injection of air to ensure safety Fig. . Laboration 1.1)  and if tDuring the study period, 11 lesions from 9 patients were treated using CT-guided cryoablation. Five recurrent cases were all due to sarcoma. Three cases  underwent pre - procedural transcatheter arterial embolization for preventing bleeding during cryoablation. Two and 3 cycles of cryoablation were performed for bone and soft tissue tumors, respectively. Technically, in all procedures, the ice ball successfully encompassed the entire tumor, visualized as a low density region under CT. The average procedure length was 101.1 min (range 63–187) and the average number of utilized probes was 2.4 (range 2–3). Inflammation, depicted as a high intensity area on MRI from the procedure, persisted for several months. However, at the final follow - up, only the low-intensity region that was not enhanced after gadolinium application persisted Fig. .Fig. 4GaLocal control is typically achieved in approximately 60–90 % of sarcomas after wide resection, depending on the tumor location , 11. VarThere has been wide variety of image guided thermal ablations, including radiofrequency, microwave, laser, high intensity ultrasound, irreversible electroporation, and cryotherapy. Percutaneous thermal ablation offers minimal invasive procedures, but each technique has their own clinical applications. Radiofrequency was the first reported percutaneous thermal ablation technique, and its efficacy has been widely reported for various tumor types. It is especially effective for small lesions, such as osteoid osteoma, for which the heat is sufficient to eradicate lesions that are usually less than 2 cm in diameter . HoweverCryotherapy was first reported in the mid - 19th century , and itsThe effectiveness of cryoablation for palliating pain from cancer metastasis has been reported by several groups , 24. FurClinically, the disadvantages of cryoablation include its medical cost , the necessity of an argon gas supply, equipment maintenance, and a large storage area . TechnicThere are a few limitations in the present study. Most critically, the sample size is relatively small, as is the case for past reports using a small case series. The wide differences in follow - up duration stems from the lack of insurance coverage for this procedure in Japan which costs approximately 9000 U.S. dollars. This has been the major obstacle in recruiting large number of patients. In addition, the results may have been biased because only relatively small sized lesions were included in the study. Nevertheless, ablation for asymptomatic metastases or local recurrence for local control has not been thoroughly reported, and our data should provide a basis for further clinical studies to clarify the efficacy of this technique in treating such patients.The outcomes from this study suggest that cryoablation is clinically safe and feasible, because no local recurrence was observed in any of the cases. Further prospective randomized studies are warranted to elucidate the optimum protocol for this technique and effectiveness for a variety of histologically different tumors.


A pseudohernia is an abdominal wall bulge that may be mistaken for a hernia but that lacks the disruption of the abdominal wall that characterizes a hernia. Thus, the natural history and treatment of this condition differ from those of a hernia. This is the first report of a pseudohernia due to cough-associated rib fracture.th and 11th ribs in a 68-year-old white woman is presented. The patient suffered from a major coughing episode 1 year prior to her presentation, after which she noted a progressively enlarging bulge in her left flank. Computed tomography demonstrated a bulge in the abdominal wall containing bowel and spleen but with all muscle and fascial layers intact; in addition, lateral 10th rib and posterior 11th rib fractures were noted.A case of pseudohernia due to fractures of the 10As there was no defect in muscle or fascia, we diagnosed a pseudohernia, likely due to a denervation injury from the fractured ribs. Symptomatic treatment was recommended, including wearing a corset and referral to a pain management clinic. Symptomatic treatment is thought to be the mainstay of therapy for pseudohernias, as surgical intervention is unlikely to be of benefit. A pseudohernia is an abdominal wall bulge that resembles a hernia but differs from a true hernia in that there is no actual muscular disruption and all muscle and fascial layers remain intact. Pseudohernias are rare phenomena, which have been reported in association with a variety of syndromes involving neuropathy or denervation, including zoster infection , diabeteth rib and the posterior aspect of her left 11th rib  presented to the clinic complaining of a bulge in her left flank that had progressed over the prior year. One year prior to presentation, she suffered a “coughing jag,” after which she developed pain in her left flank as well as the progressive flank bulge. The pain was constant, had a burning quality, and was intermittently severe. She denied a history of zoster or other neuropathy syndromes. Prior surgical history included a hysterectomy and an umbilical hernia repair. A computed tomography (CT) scan Fig.  was initib Figs.  and 3. HRecommendations included wearing a corset to manage the bulge, weight loss, and evaluation in a chronic pain clinic to manage the pain associated with her pseudohernia, which was thought to be more likely related to nerve injury rather than to the bulge itself. Surgery was not felt to be indicated as this was not, in fact, a true hernia.The phenomenon of pseudohernia was first described in 1936 by Loewe in the context of local anesthetic injection into the abdominal muscles of guinea pigs . He notePseudohernias have been described as sequelae of herpes zoster infection. A 2013 literature review analyzed 36 cases of pseudohernias related to segmental zoster abdominal paresis [Pseudohernias have also been described as sequelae of diabetic neuropathy. A 1999 case report described a 63-year-old man who experienced a pseudohernia 6 months following the onset of stinging left-sided abdominal pain and was found to have denervation of the left paraspinal muscles in the distribution of the T6-T10 myotomes on electromyography (EMG) . His pseth intercostal space, 6/31 patients developed pseudohernias postoperatively [Postoperative pseudohernias have been noted to occur following procedures involving flank incisions. A 2004 study of 70 patients who underwent radical or partial nephrectomy with flank or thoracoabdominal incisions found that 34 (49 %) of the patients reported durable, often painful, flank bulges after 1 year, thought not to be true hernias. Interestingly, flank bulges were more common on the left side, and the authors speculated that these bulges were related to denervation injuries, though they did not use the term “pseudohernia” [ratively . Other rFinally, two case reports describe “cough-induced intercostal hernias” associated with rib fractures, attributed to rupture of intercostal muscles , 9. BothIn summary, pseudohernia should be considered in the evaluation of flank or abdominal protrusions, particularly when a cause for denervation injury can be ascertained. Causes may be infectious, metabolic, iatrogenic, or traumatic. A CT scan can be a useful imaging modality and demonstrates an abdominal wall bulge with preserved integrity of the musculofascial layers of the abdominal wall, in contradistinction to a true hernia, in which a muscular or fascial disruption should be seen. It has been suggested that EMG may have utility to confirm the diagnosis. As there is no physical defect in the abdominal wall to be corrected, surgical intervention is not generally thought to be efficacious for the treatment of pseudohernias , though Pseudohernias are functional abdominal protrusions that resemble hernias but are not associated with evidence of muscular or fascial disruption. They are associated with neuropathy or neural disruption. In the case presented here, a pseudohernia resulted from an apparent nerve injury related to cough-induced rib fracture. Symptomatic relief, rather than surgical repair, is thought to be the mainstay of therapy for pseudohernias.


Although hilar cholangiocarcinoma (HCCA) has a very poor prognosis, there are cases in which long-term survival is rarely obtained by multidisciplinary treatment.A 61-year-old man diagnosed with HCCA was referred to our hospital. We performed an extended left hemi-hepatectomy and caudate lobectomy with extrahepatic bile duct resection. The tumor stage was T2aN0M0, stage II, based on the TNM classification, seventh edition. R0 resection was successfully performed. Adjuvant chemotherapy was not administered. After 38 months, computed tomography revealed peritoneal dissemination. The patient received chemotherapy with tegafur-gimeracil-oteracil-potassium (S-1) and gemcitabine. The peritoneal dissemination was successfully controlled for more than 50 months. During the treatment, levels of CEA and CA19-9 kept rising slowly, which was followed by bowel obstruction due to peritoneal dissemination of HCCA. The patient underwent resection of transverse colon with tumor nodules, and the tumor was pathologically diagnosed as metastasis of HCCA. Tumor markers decreased to normal levels, and the patient has been free from tumor relapse for 6 months.We here report a rare case of HCCA patient with recurrent peritoneal dissemination 3 years after R0 surgery which was sensitive to chemotherapy. The patient successfully received resection of peritoneal dissemination 50 months after the induction of chemotherapy and survived for 10 years. Hilar cholangiocarcinoma (HCCA) is a type of bile duct cancer that occurs in the extrahepatic biliary tree proximal to the origin of the cystic duct . It is tWe here report a rare case that chemotherapy could control the recurrence of peritoneal dissemination of HCCA over 50 months which was resected successfully and survived more than 10 years after initial resection with normal tumor marker level.A 61-year-old man, who was diagnosed with HCCA after an endoscopic retrograde biliary drainage for obstructive jaundice, was referred to our hospital. Laboratory tests showed that the patient’s total bilirubin, aspartate transaminase, alanine aminotransferase levels were 1.2 mg/dl, 56 U/L, and 161 U/L, respectively. Levels of carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were 3.3 ng/mL and 40.4 U/mL, respectively. Percutaneous transhepatic bile duct drainage for the left branch of intrahepatic bile duct and cholangiography showed that biliary tract was obstructed at the hilum (Fig. + T cells were found in the tumors (Fig. Thirty-eight months after the operation, CEA and CA19-9 levels had elevated, and peritoneal dissemination was detected by enhanced CT Fig. a and posors Fig. d. After ors Fig. . The patAlthough surgical resection is the only curative treatment for HCCA, the recurrence rate after surgery is 50–75% . Therefo+ T cells play an important role in anti-tumor immunity during tumor progression [+ T cell after chemotherapy was a predictor of good response to chemotherapy [+ T cell accumulation in cancers can lead to better clinical outcome [+ T cell status predict prognosis and sensitivity to chemotherapy after curative resection in extrahepatic cholangiocarcinoma [+ T cells within the tumor by immunohistochemistry, which might have contributed to the positive response to chemotherapy and long-survival.Adjuvant chemotherapy has not been standardized for cholangiocarcinoma, although GEM plus cisplatin is effective for advanced cholangiocarcinoma . Sasaki gression , 19. Sevotherapy –22. This outcome . We alsoarcinoma . We deteWe here report a rare case of HCCA patient with recurrent peritoneal dissemination which occurred 38 months after curative surgery and successfully resected 50 months after the induction of chemotherapy. As a result, the patient survived more than 10 years after initial resection maintaining normal tumor marker level. Persistent chemotherapy seemed effective for a long period in this case, and we need to explore predictive factors for such successful chemotherapy based on the pharmaceutical mechanism of each chemotherapeutic agent.


Avascular necrosis (AVN) of the bones of the wrist most commonly involves the lunate followed by the proximal pole of the scaphoid and the capitate. Trapezium avascular necrosis is extremely rare with only two cases reported in the literature, both of which were treated surgically. In this article, we report a unique case of trapezium avascular necrosis treated conservatively. A 38-year-old man complaining of a 4-month history of mild pain on the base of his right thumb. MRI scan was performed. The clinical presentation and the imaging findings indicated avascular osteonecrosis of the trapezium. The patient was treated with immobilization of the wrist joint for a period of six weeks. Three months later, the patient was free of symptoms and the MRI scan revealed a normal trapezium. AVN of trapezium is extremely rare. Our case shows that immobilization of an early stage avascular necrosis of the trapezium might be a treatment option. Avascular necrosis (AVN) is a disease where there is cellular necrosis of bone and marrow elements due to an interruption of the blood supply. It is a disease documented in several bones throughout the human body and most commonly affects the femoral head , 2.AVN of the carpal bones is an uncommon condition that most commonly involves the lunate  and the Trapezium AVN is extremely rare with only two cases reported in the literature, both of which were treated with bone excision and arthroplasty or vascularized bone graft , 7.In our case, we report a trapezium AVN that was treated conservatively.A 38-year-old man was presented in the orthopaedic department of our hospital complaining of a 4-month history of mild pain on the base of the right thumb. The patient had no history of trauma, no systemic disease was reported, and he had not taken any corticosteroids. He is a white-collar bank worker and reported continuous mild pain that impaired his ability in everyday activities.There was no inflammation and the mobility of the wrist and fingers was normal. Physical examination revealed tenderness over the trapezium without any swelling.The patient underwent an X-ray examination which proved to be normal. Further investigation with an MRI of the wrist revealed an area of abnormal signal intensity affecting the whole trapezium. On T1-weighted MR images, the bone demonstrated low signal intensity while being hyperintense on PD and STIR sequences . The imaDue to the mild symptoms, the age of the patient, and the blood supply of the trapezium, we decided to proceed with conservative treatment. The patient's wrist and thumb were immobilized with a thumb thermoplastic splint for six weeks and he was prescribed nonsteroidal anti-inflammatory drugs. Right after that period and because most of the symptoms were faded, he applied the splint only during the night for additional four weeks.After three months, the patient's symptoms subsided and an MRI scan revealed a normal trapezium .AVN is a condition defined as bone necrosis due to interruption of blood supply –10. AVN The disruption of blood supply leads to bone oedema as seen on MRI scans.Gelberman et al. described in detail the blood supply of the carpal bones –13. The The role of MR imaging in the early diagnosis of AVN is well established. Findings include signal drop of the whole trapezium on T1WI and increased signal on T2WI and STIR images.Its clinical presentation includes chronic pain, swelling, reduced mobility, and stiffness. An early diagnosis is vital due to the fact that it can lead to bone salvage if the correct treatment is applied.The differential diagnosis of “mild” types of trapezium AVN should include bone oedema secondary to trauma, infection , or basilar thumb arthritis, in which case the Χ-ray and MRI imaging would have been different. In our case, there was also no history of trauma or any clinical or laboratory findings of infection.With this case report, the authors would like to emphasize the excellent results of conservative treatment (immobilization) in early stages of trapezium AVN, especially when the X-ray is diagnosed as “normal.” Actually, there is no specific classification for trapezium AVN as a result of the very few cases reported in the literature. We characterised this incident as a stage I AVN based on Steinberg et al.  and ARCO2 [There is no literature regarding trapezium AVN treated conservatively. However, there are articles documenting conservative treatment of early AVN stages, such as in hip, using teriparatide, alendronate , or pros2 . The imm2 , whereasTo our knowledge, only two cases of AVN of the trapezium were reported so far and both of them were treated surgically either by suspension arthroplasty or by vascularized bone grafts , 7. In tAvascular necrosis of the trapezium is extremely rare with only two cases reported in the literature so far, both of which were treated surgically. In our case, taking into consideration the diffuse blood supply of the trapezium, we decided to treat the patient conservatively resulting in a normal bone after three months. Conservative treatment should be considered as an option before surgery.


Angiosarcoma of the ovary is rare but represents an aggressive type of malignant ovarian neoplasms. The purpose of this report is to describe the features of angiosarcoma arising in mucinous tumor that was misinterpreted as a benign vascular proliferation during the intraoperative consultation. A 45-year-old woman presented with an abdominal mass for 1 month. Exploratory laparotomy was performed. A 35 cm right ovarian mass submitted for intraoperative consultation was a multicystic mucinous tumor with an 8 cm area of hemorrhagic lesion between cystic locules. The frozen section diagnosis was at least mucinous borderline tumor. The hemorrhagic area, which was intraoperatively interpreted as organizing vessels associated with previous hemorrhage, represented angiosarcoma in permanent sections. Angiosarcoma may present a challenge in intraoperative frozen section diagnosis of an ovarian mass. The presence of ectatic anastomosing vessels with dissecting growth appears to be the clue to a suspicion of angiosarcoma. The presence of endothelial atypia provides further support for the diagnosis. A macroscopic hemorrhagic area in an ovarian mucinous tumor should be evaluated with care, and the possibility of angiosarcoma should be borne in mind. Mucinous tumor is a common type of ovarian epithelial tumors. The large majority of mucinous tumors are benign and borderline tumors. Invasive mucinous adenocarcinoma is uncommon and usually arises in the background of benign or borderline tumors. Nonepithelial malignant neoplasms sometimes arise in mucinous tumors, typically as mural nodules.Angiosarcoma of the ovary is rare but represents an aggressive type of malignant ovarian neoplasms. To our knowledge, less than 40 cases of ovarian angiosarcoma have been reported in English literature –3. In twDue to the rarity of angiosarcoma in the ovary, this tumor can be a diagnostic challenge to surgical pathologists, particularly during an intraoperative consultation. A failure to intraoperatively recognize this malignant neoplasm may result in incomplete surgical staging procedure or inappropriate clinical management. The purpose of this report was to describe the features of angiosarcoma arising in mucinous tumor that was initially misinterpreted as a nonneoplastic vascular proliferation in the intraoperative frozen section examination. The findings in this case may be useful for pathologists in the recognition of this uncommon type of ovarian tumors.μg/L . Ultrasonography and abdominal computed tomographic scan revealed a 26 cm right ovarian solid-cystic mass with internal septation. No abnormality in the other intra-abdominal organs was identified. Exploratory laparotomy was performed. Intraoperatively, there was 200 mL of mucous fluid. A 35 cm right ovarian mass was identified, with focal previous rupture of the external surface. The left ovary showed a 6 cm cyst with chocolate-like content. The other gynecologic and intra-abdominal organs were unremarkable.A 45-year-old woman, parity 0, presented with a complaint of a palpable abdominal mass with discomfort for 1 month. The physical examination showed unremarkable finding except for a large pelvic mass. The serologic tumor markers were as follows: CA 125, 110 IU/mL , CA 19-9, 144 IU/mL , and CEA, 1.3 The right ovarian mass was submitted for intraoperative consultation. The sectioned surface of the mass was multicystic and composed of numerous mucus-filled cystic locules and spongy to almost solid tissue. An area of hemorrhagic foci between cystic and spongy locules is noted, with a maximal linear dimension of 8 cm . The froThe right ovarian mass was further sampled in 35 tissue blocks for permanent sections. The tumor represented a mucinous borderline tumor with marked nuclear atypia  and few foci of infiltrative-type stromal invasion of less than 5 mm in diameter (microinvasive mucinous adenocarcinoma). In the hemorrhagic area, proliferation of irregular and dilated anastomosing vascular channels was present around small collections of blood and dissected into the septa between mucinous borderline locules . The linAnother focus of infiltrating undifferentiated carcinoma composed of high-grade pleomorphic spindle and epithelioid cells, measuring 14.0 × 2.5 mm, was present in the wall of a mucinous borderline locule remote to the hemorrhagic area . No connThe left ovary contained a 6.5 cm endometriotic cyst. The uterus and bilateral fallopian tubes were not remarkable. The omentum, pelvic and para-aortic lymph node specimens, and appendix were negative for neoplastic involvement.The immunohistochemical stains showed different staining patterns between angiosarcoma and undifferentiated carcinoma .Ovarian angiosarcoma is an aggressive malignant neoplasm. Approximately two-thirds of cases had stage II–IV tumors, and approximately one-half of patients with known follow-up data died of disease with a median survival of 10.2 months . MetastaTo our knowledge, 37 cases of ovarian angiosarcoma have been previously reported  . Seven oMacroscopic appearance of angiosarcomatous component is typically a hemorrhagic lesion. In 5 previously reported cases of angiosarcoma associated with mucinous tumors, the angiosarcomatous component was macroscopically recognized as mural nodules or hematoma-like areas in 4 cases , 2, 6, 7It should be noted that benign vascular neoplasm arising in ovarian mucinous tumor is less common than angiosarcoma. Among approximately 50 reported cases of ovarian hemangioma, only 2 cases of hemangioma were associated with mucinous tumors: one in mucinous cystadenoma (32-year-old)  and the The histologic recognition of angiosarcomatous component in the frozen section of ovarian mass can be difficult . In the Differential diagnosis of angiosarcoma includes benign vascular proliferation or hemangioma. Anastomosing network of vascular channels with intervening hyalinized stroma may be similarly observed in benign papillary endothelial hyperplasia following organization of hematoma or thrombus , but thiIntraoperative frozen section is important in making decision for surgical management, that is, radical resection with complete staging in malignant ovarian tumors. Sampling error is a major cause of inaccurate frozen section diagnoses, and this type of error occurs more commonly in tumors with high tissue heterogeneity, particularly mucinous tumors . As obseAngiosarcoma may present a challenge in intraoperative frozen section diagnosis of an ovarian mass. A macroscopic hemorrhagic area in an ovarian mucinous tumor should be evaluated with care, and the possibility of angiosarcoma should be borne in mind. The presence of ectatic anastomosing vessels with dissecting growth appears to be the clue to a suspicion of angiosarcoma. The presence of endothelial atypia provides further support for the diagnosis.


In the central nervous system, cerebellopontine angle (CPA) lymphomas are rare; few cases have been reported. Lymphoplasmacytic lymphoma (LPL) in the CPA is rarer still, and often misdiagnosed as acoustic neuroma.We report a rare case of CPA LPL—a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological test.A 43-year-old woman presented with headaches. Her magnetic resonance imaging revealed an abnormal homogeneously enhancing mass in the left CPA. We present detailed analysis of her disease and review relevant literature.When surgically treated, her specimen showed a typical LPL histopathology pattern. After surgery, the patient's symptoms improved greatly, and she received chemotherapy.Despite its rarity, LPL should be considered in differential diagnoses of CPA lesions that mimic acoustic neuromas. Cerebellopontine angle (CPA) lymphomas are rare and only a handful of cases have been reported so far. Lymphoplasmacytic (or lymphoplasmacytoid) lymphoma (LPL) is an uncommon mature B-cell lymphoma that usually involves the bone marrow and, less commonly, the lymph nodes and spleen. LPLs in the CNS are uncommon, and rarer still in the CPA.Primary central nervous system (CNS) lymphoma is a very rare aggressive non-Hodgkin disease that originates in CNS  without evident involvement of other systemic locations. They account for less than 1% of all intracranial tumors but have apparently increased over the last 2 decades in both immunocompromised and immunocompetent patients.Here, we report an uncommon solitary primary LPL in the CPA, and discuss its treatment and differential diagnoses. Although a few CPA lymphomas have been reported previously, this is the first reported case of a CPA LPL, to our knowledge.1.1A 43-year-old woman was admitted to our hospital complaining of continuous frontal and occipital headaches for 4 months, and a left hearing loss for 2 months. One week before admission, she began vomiting for about 5 times a day. She had no significant medical background or family history. No lymphadenopathy or organomegaly were found upon general physical examination. Skull, bone, cardiac, chest, and per rectal examinations revealed no abnormalities. She was oriented and conscious. Visual fields and acuity was normal. Neurological examinations revealed normal motor function and sensation in all four limbs. No obvious changes in superficial or deep tendon reflexes were detected. Ataxia and pathological signs were absent. Routine hematological test results were all normal and the serological HIV test was negative. However, a brain magnetic resonance imaging (MRI) showed a homogeneously enhancing mass in the left CPA, measuring 5 cm × 5 cm × 3.5 cm; neuritis or a CPA tumor was suspected Fig. . The tumThe patient underwent a left retromastoid craniectomy and a tumor resection. During surgery, a well-defined left CPA lesion extending to the left internal auditory canal was seen. It was against the petrous bone and tentorium cerebelli, with abundant vascularization and relatively clear boundaries. The tumor was extirpated as multiple pieces of friable, gray, and firm tissues, which were fixed in 10% paraformaldehyde and embedded in paraffin. Microscopically, the tissues were a mixture of cancerous lymphocytes and plasma cells. Immunohistochemical tests showed the tissues to be positive for CD38, CD20, CD38, and CD79α, and negative for AE1/AE3, Bcl-6, CD3, CD10, glial fibrillary acidic protein, neuron-specific enolase, and Syn. The positive rate for Ki-67 was 10%.Pathological examination confirmed the diagnosis of LPL Fig. . The patOne week after the surgery, the patient's headache and hearing loss were much relieved. Postoperative MRI showed that the CPA tumor was totally removed, and adjacent structures were thoroughly decompressed. She was then transferred to the hematology department for chemotherapy. The chemotherapy protocol was the cyclophosphamide, vincristine, and prednisone regimen along with rituximab. After 6 cycles of chemotherapy, her Karnofsky Performance Scale score was 95. An MRI performed half a year after the surgery showed no recurrence Fig. .2 In a series of 32 nonacoustic CPA tumors, hemangiosarcoma was also recorded.A few rare tumors can occur in the CPA. After excluding cranial nerve schwannomas (1.4%), cholesteatoma (2.4%), meningioma (3.1%), and acoustic neuromas (91.3%) from a series of 1354 CPA tumors, 25 uncommon tumors remained, including lipomas, metastatic tumors, gliomas, hemangiomas, hemangioblastomas, arachnoid cysts, dermoids, and a teratoma. They may occur in both immunocompromised and immunocompetent individuals. For immunocompetent patients, the mean age at diagnosis of PCNSL is 55 years; for AIDS patients, the mean age is 31 years. The female: male ratio is 2: 3. PCNSLs often occur in thalami, periventricular region, corpus callosum, and basal ganglia. However, only 15 cases of PCNSLs at the CPA have been reported in the literature. Almost all PCNSLs are aggressive neoplasms of diffuse large B-cell lymphoma type; low-grade malignant lymphomas such as peripheral T-cell, Burkitt lymphoma, marginal mantle cell lymphoma, and LPL are rare.Primary central nervous system lymphomas (PCNSLs) are uncommon tumors that account for approximately 0.8% of lymphomas at all sites and 0.3% to 1.5% of all intracranial tumors. Most patients demonstrate increased monoclonal serum IgM, and a few have elevated IgA or IgG. LPL usually involves the bone marrow and, less commonly, the spleen and lymph nodes. The 2008 World Health Organization (WHO) classification defined Waldenström macroglobulinemia (WM) as a clinicopathologic entity associated with an IgM monoclonal gammopathy and marrow involvement, which is the commonest manifestation of LPL. The pathogenesis of LPL is incompletely understood. However, deletions of 6q21 have been identified as a common site of chromosome loss in patients with WM. These findings suggest that MYD88 mutations that lead to the activation of NF-kB family transcription factors are pivotal role in the pathogenesis of LPL.,12 Chronic infection with HIV and hepatitis C virus (HCV) has also been implicated as risk factors.LPL is a kind of non-Hodgkin lymphoma with low-grade malignancy, and composed of plasma cells, plasmacytoid lymphocytes, and small B cells. Its incidence is approximately 3% to 4.5% per year worldwide.–16 of which 9 as well as the present one included detailed information , meningiomas (2–3%), and epidermoid tumors (1–2%). Other rarer CPA tumors include facial nerve neurilemomas, arachnoid cysts, paraganglioma of the glomus jugulare, lipomas, hemangiomas, angioblastoma, choroid plexus papillomas, teratoma, craniopharyngioma, gliomas, liposarcoma, chondrosarcoma, medulloblastoma, metastatic tumors, primary epidermoid carcinomas, endodermal sinus tumors, and malignant schwannoma with rhabdomyoblastic differentiation. Signs, symptoms, and tests—especially imaging examinations—of some common differential diagnoses are listed in Table  The CPA LPL immunophenotype is sometimes similar to other small B-cell lymphoid neoplasms, such as marginal mental cell lymphoma; thus, testing for serum immunoglobulin and gene mutation can be useful.The average onset age of primary CNS LPL is 43.3 years, and a high proportion of patients are young or middle-aged Table . The braingiomas –3%, and  As the cases are few, no specialized guideline has yet been developed. The chemotherapy regimen is cyclophosphamide + doxorubicin + vincristine + prednison (CHOP) or cyclophosphamide, vincristine, and prednisone, with rituximab—a monoclonal antibody widely used against B cell lymphoma. Patients who received rituximab along with CHOP reportedly had significantly higher rates of overall response than patients who only received the traditional regimen.The predominant treatments for primary CNS LPL are surgery, radiotherapy, chemotherapy, and molecular targeted therapy. No accurate prognostic data are available for primary CNS LPL because of the scarcity of case reports. LPL patients with no special treatment are reported to have a median survival of longer than 5 years; their 10-year survival rate is around 70% to 75%.,21 No deaths were reported among the 7 patients with follow-up data summarized in Table The positive rate of Ki-67 in LPL is generally less than 20% (∼10% in our case), which leads to a benign prognosis. For asymptomatic patients, regular follow-up alone is recommended.3We report here a rare case of LPL initially presenting with CPA compression. LPL rarely involves the CPA. It is difficult to diagnose pre-surgically and is usually misdiagnosed. With this article, we aimed to both present this rare entity and to emphasize the importance of keeping this differential diagnosis in mind when analyzing CPA lesions.


Stone formation within the intestinal lumen is called enterolith. This stone can encroach into the lumen causing obstruction and surgical emergency. Jejunal obstruction by an enterolith is a very rare entity and often missed preoperatively. To our knowledge, most cases of jejunal obstruction, secondary to stone, were associated with biliary disease (cholecystoenteric fistula), bezoar, jejunal diverticulosis, or foreign body. Hereby we present a rare case report of small bowel obstruction in an elderly man who was diagnosed lately to have primary proximal jejunal obstruction by an enterolith without evidence of a cholecystoenteric fistula or jejunal diverticulosis. This patient underwent laparotomy, enterotomy with stone extraction, and subsequent primary repair of the bowel. The term enterolithiasis defines intestinal intraluminal stone. This pathology can also be described as enterolith ileus or pseudogallstone ileus when it causes small bowel obstruction . ExtrinsA 76-year-old male patient presented to our emergency department with a 72-hour history of persistent nausea, vomiting, and generalized fatigue associated with diffuse colicky abdominal pain. He had a long history of intermittent episodes of abdominal pain and distension. He was afebrile and obstipated and did not pass stool for 3 days. His past medical history is significant for hypertension and prostate cancer. He had open prostatectomy 1 year ago. Upon physical exam, the patient was hemodynamically stable and slightly dehydrated. His abdomen was soft with mild diffuse tenderness and distention.9/L (neutrophils 92%), C-reactive protein of 36 mg/L, and Cr of 2 mg/dL. The remaining lab studies were within normal limits. We did not order a plain abdominal film as this would not show valuable information regarding the diagnosis.Blood tests revealed a leukocyte count of 11,500 × 10A computed tomography scan with oral contrast was performed and showed proximal dilated jejunal loop (up to 5 cm in diameter) with a large ring of calcification likely suggestive of ascariasis ; the resThe patient was admitted to the hospital. Conservative management was started with IV hydration, pain management, and a nasogastric tube . He was prepared for exploratory laparotomy through a supraumbilical midline incision.Upon surgical exploration, the proximal jejunum was found to be dilated, whereas the distal jejunum, ileum, and large bowels were collapsed. A mass about 5.5 cm in size was found to be obstructing the proximal jejunum about 30 cm from the ligament of Treitz . An enteProximal jejunal obstructions are typically caused by adhesions or tumors. Less frequently, such cases can be secondary to strictures because of inflammatory bowel disease, gallstone impactions, bezoars, and/or foreign bodies . To our Some authors have found a possible association between primary enteroliths in the jejunum and the presence of small bowel diverticuli. With the usual composition of primary enterolith being choleic acid, an end product of bile salt metabolism, it has been postulated that the formation of these stones is secondary to the acidic pH shift within the small confined bowel diverticulum .Another contributing factor that has been reported is the stasis encountered in patients with bowel hypomotility. Indeed, stasis seems to be necessary to permit the progressive accumulation of particulate matter leading eventually to the formation of a stone . In our Other possible causes of enterolith in the small bowel include Meckel's diverticulum, small bowel anastomosis, metabolic diseases, intussusception, intestinal strictures, and inflammatory or infectious enteritis . CongeniConcerning the management of such cases crushing the enterolith and milking it distally is the first step to do . If thisIn conclusion, jejunal obstruction by a primary enterolith is a very rare entity. This pathology should be expected when other common pathologies have been excluded for the cause of small bowel obstruction in the elderly population. Hence, diagnosis and management are often delayed. Surgical exploration is often necessary as it can result in serious potential complications.


Prostate cancer is mostly known to metastasize to the bony skeleton. Soft tissue metastasis involving visceral organs such as the liver, lung and brain are unusual and rare manifestations of this cancer. However, with better treatment modalities and increased survival, the incidence of these unusual presentations, seems to have risen in the last few years. Prostate cancer is rarely known to metastasize to the orbit. Although cases of bony metastasis involving the orbit have been reported, soft tissue metastasis involving the orbit is a very rare presentation. Most soft tissue metastasis to the orbit involves the uveal tract, with prostate cancer being the most common primary cancer to metastasize to the iris . Our paper highlights a case of extraconal orbital soft tissue metastasis secondary to prostate cancer, an extremely rare presentation. Patient was started on combined androgen blockade therapy. Three-month repeat MRI orbit showed that the extraconal orbital mass had decreased in size significantly. His clinical symptoms have resolved at the time of this report. To our knowledge, this is the first reported case of its kind. Prostate cancer (PC) is the most common non-skin cancer in men . It is tA 79-year-old male presented to an ophthalmologist’s outpatient office with progressively worsening vision in his right eye over 3 - 4 months. He also complained of diplopia and proptosis in the same eye. Review of systems was positive for increased urinary frequency and occasional nocturia over the last 6 months. However, he denied any incontinence, dysuria, flank pain or fever or chills.Physical examination revealed a well-built man with no acute distress. Vital signs were within normal limits, except for an elevated blood pressure of 146/87 mm Hg. Head and neck exam was positive for mild pallor; however, no icterus or lymphadenopathy was noted. Rest of the systemic exam was unremarkable. His karnofsky performance status scale was 80%. Ophthalmological exam revealed visual acuity of 20/30 and 20/25 in right and left eye, respectively. Intra-ocular pressures were 13 mm Hg and 10 mm Hg in right and left eye, respectively. Proptosis of 5 mm and a 60% reduction in all fields of right eye were noted. Examination of the left eye revealed full motility, no proptosis and 2+ nuclear sclerotic lens changes. Dilated fundus exam was unremarkable in both eyes.Magnetic resonance imaging (MRI) of the head revealed a hypointense, mildly enhancing, multi-compartmental soft tissue mass lesion in the superior right extraconal space measuring approximately 4.3 × 2.3 cm. This mass was seen extending and breaking through the orbital roof into the anterior cranial fossa superiorly, and medially extending into the lamina papyracea, posterior ethmoid sinus and the anterior lateral sphenoid sinus. Lateral extension into the posterior lateral orbital wall and sphenoid wing was noted. This mass was also seen wrapping and compressing the optic nerve sheath complex in the posterior intraconal space at the orbital apex. A 1.2 cm sclerotic lesion in the right parietal bone was also noted . PatientA thorough systemic evaluation was performed to look for a primary source of malignancy. Laboratory work-up revealed a normal complete blood count and comprehensive metabolic panel except for an elevated alkaline phosphatase level of 317 units/L . Whole body PET-CT showed: an FDG avid mass in the right posterior orbit extending into the ethmoidal cells (SUV 4.2), FDG avid focus in the left posterior aspect of the prostate gland suspicious of a malignancy (SUV 8.9), bilateral FDG avid enlarged iliac nodes (SUV 6.1 - 6.3) and multiple FDG avid sclerotic bony lesions, suspicious of metastasis .Further work-up revealed an elevated prostate specific antigen (PSA) and acid phosphatase level of 521.20 ng/mL  and 70.2 ng/mL , respectively. A urological consultation was obtained, and prostate biopsy results revealed a poorly differentiated adenocarcinoma with a Gleason score (GS) of 8 and 9 in 11 out of 12 cores, similar to the orbital lesion. Immunohistochemical stains on the orbital tissue (biopsy) were positive for PSA, PSMA, AE1:AE3 and Cam5.2 cytokeratins, and negative for TTF-1, CDX2, CK7, CK20 and EMA. MRI of the spine confirmed multi-level osseous metastatic infiltration throughout the spinal axis particularly involving the lower thoracic and lumbar spine .This confirmed the diagnosis of adenocarcinoma arising in the prostate gland, with metastasis to the soft tissue of the right eye (stage IV). Patient was started on bicalutamide and degarelix (combined androgen blockade therapy). Three-month repeat MRI orbit showed that the extraconal orbital mass had decreased in size significantly. Eight months later patient continues to follow up as an outpatient, and his vision and diplopia have improved significantly.Adenocarcinoma of the prostate accounts for 3.6-4% of all orbital metastasis . In anotHowever, soft tissue metastasis to the orbit is extremely rare. Most cases of soft tissue orbital metastasis secondary to PC involve the uveal tract -17 Tabl. PC is tExtraconal space is the area outside muscle cone in the eye. Various lesions are known to arise in this space . AlthougTreatment options for orbital metastasis secondary to PC mainly include androgen blockade therapy and cranial irradiation. Cases of complete regression of choroidal metastasis following hormonal manipulation in patients with PC have been reported , 22. RadExtraconal orbital soft tissue metastasis secondary to prostate cancer has not been reported in the past. To our knowledge, this is the first reported case of extraconal soft tissue metastasis secondary to PC.


Introduction. The myoepithelioma is a rare benign tumor, most frequently found in the salivary glands. The extrasalivary gland involvement is even rarer and few cases involving the nasal cavity have been reported in the literature. Case Report. MES, a 54-year-old woman, complaining of progressive nasal obstruction and mild epistaxis through the right nostril which had developed 1 year previously. Computed tomography scan showed tumor with heterogeneous contrast enhancement occupying the right nasal cavity, moving contralaterally in the nasal septum. Excisional biopsy was performed through endoscopic surgery of the mass that was inserted at the nasal septum. Pathological and immunohistochemical exams concluded myoepithelioma. Discussion. The main symptoms of nasal myoepitheliomas are nasal obstruction and epistaxis. Immunohistochemistry is necessary to confirm the diagnosis, typically positive for cytokeratin and S-100, calponin, smooth muscle actin, myosin, vimentin, glial fibrillary acidic protein (GFAP), and carcinoembryonic antigen. The main marker for myoepithelioma is the S-100 protein. In our case, it was positive for cytokeratin, S-100, calponin, actin smooth muscle, and GFAP. In all cases reported in the literature surgical treatment was performed and the recurrence was associated with incomplete tumor resection. Final Comments. The myoepithelioma is a rare differential diagnosis of nasal tumors and its treatment is the total lesion excision. Myoepithelioma is a rare benign tumor, most frequently found in the salivary glands, representing, however, only 1% of all tumors of these glands. In the major salivary glands there is a predilection for the parotid and in the minor salivary glands for the palate and oral cavity . The extMES, a 54-year-old woman, presented at our hospital complaining of progressive nasal obstruction and mild epistaxis through the right nostril which had developed 1 year previously. She denied smoking and had no known toxic exposure or family history of neoplasms. Nasofibroscopy revealed irregular vascular lesion which occupied the right nasal cavity almost completely. Computed tomography (CT) scan showed heterogeneous contrast enhancement by the tumor, which displaced contralaterally the nasal septum . CompletThe material that consisted of several brown tissue fragments measuring together 6,0 × 3,0 × 1,0 cm was sent for pathological examination, which showed morphological features compatible with myoepithelioma, despite the focal presence of chondroid matrix and occasional ductal structures. Immunohistochemistry presented positivity for glial fibrillary acidic protein (GFAP), smooth muscle actin, cytokeratin (AE1AE3 and MNF116), calponin, P63, S100, and CD99. The septal margin was found to be affected by the tumor but was chosen for follow-up, and after two years of follow-up there was no evidence of tumor recurrence or metastasis.Nasal cavity and paranasal sinus tumors are very infrequent, accounting for less than 1% of all head and neck tumors . Nasal mMyoepithelioma is composed of myoepithelial cells and may have pattern of solid growth, myxoid or reticular. The cell types found are fusiform, plasmacytoid, epithelioid, and clear cells. The pattern of growth and cell type does not change the prognosis of the disease –12. ThisImmunohistochemistry is necessary to confirm the diagnosis, typically positive for cytokeratin, S-100, calponin, smooth muscle actin, myosin, vimentin, glial fibrillary acidic protein (GFAP), and carcinoembryonic antigen , 15. TheIn all cases reported the surgical treatment was performed and the recurrence has been associated with incomplete resection of tumor, which proves the aggressive biological behavior of this tumor , 17. HisThere are very few cases of myoepithelioma involving the nasal cavity reported in the literature. However in the last few years the publications of its extrasalivary gland involvement increased substantially, which makes the authors question if it is an underdiagnosed disease. The advances in pathology and immunohistochemistry knowledge and techniques may help us to diagnose it more and more commonly in the future. Therefore, although it is rare, we have to consider it as a differential diagnosis of nasal tumors.


Schwannomas (or neurilemmomas) of the tongue are benign, usually solitary, encapsulated masses derived from Schwann cells. Clinical evidence indicates that schwannoma is painless and slow growing. In general, schwannoma is treated by surgical excision.Here, we describe a case of schwannoma of the tongue, include a review of the literature from 1955 to 2016, and provide data on age, gender, location, presenting symptoms, size, and treatment methods.A 71-year-old female patient presented with a swelling at the base of the tongue of unknown duration. Magnetic resonance images (MRI) showed a large well-circumscribed solid mass and no significant lymph node enlargement. The mass was excised without removing overlying mucosa.The authors report a case of lingual schwannoma that was completely removed intraorally without preoperative biopsy. No sign or symptoms of recurrence were observed at 12 months postoperatively. Around 25–40% of schwannomas occur in the head and neck region, and of these, 1–12% affect the intraoral area .Clinically, schwannomas are benign, usually solitary, encapsulated masses that originate from Schwann cells without pain or ulceration.Here, we report a case of schwannoma of the tongue base and review the literature. A Google search of the terms “schwannoma (neurilemmoma) of the tongue” and “lingual Schwannoma” was performed from 1955 to 2016. Age, gender, location , presenting symptoms, size, and treatment methods were extracted from case reports.X years) and thyroid grand tumor. A well-encapsulated nodular mass was evident at physical examination, but without any neurologic symptom or lymphadenopathy in the submandibular area. The mass was 3 × 2 cm sized without ulceration , pain , dysphonia (6 cases), voice change (5 cases), paresthesia (3 cases), snoring (2 cases), bleeding (2 cases), ulceration (2 cases), and abscess (1 case). Masses were located in any part of the tongue. Average size at removal was 2.4 cm , and all were treated by transoral excision except 3 cases. The submandibular approach was used in 2 cases and lip splint and mandibulectomy in 1 case. In all three of these cases, masses were located in posterolateral bases.Although the etiology of schwannoma is not clear, it is known to be derived from nerve sheath Schwann cells, which surround cranial, peripheral, and autonomic nerves , 7. The Computed tomography (CT) usually shows well-defined homologous lesions. When a heterogeneous lesion is observed by CT, malignant change may be suspected . HoweverIn our case, MRI ruled out the possibility of malignancy and invasion. Enoz et al.  reportedHistologically, all schwannomas are encapsulated, and beneath capsules, two main patterns are observed, that is, Antoni type A, which is highly cellular and is composed of elongated Schwann cells, which exhibit a palisading nuclear pattern, and Antoni type B, which is also composed of elongated Schwann cells, but cells are arranged in a less dense myxoid manner and are more disorganized than Antoni type A Fig. .2 laser excision has also been used to treat base of tongue Schwannomas [Schwannomas are usually treated by surgical excision with involved originating nerve . In the wannomas , 17. On wannomas , 19. Schwannomas . BecauseLingual schwannoma is a relatively rare tumor of the head and neck and may occur anywhere in the tongue. At presentation, the majority of patients complain an asymptomatic mass and slight ulceration. Transoral resection preserving overlying mucosa allowed us to remove the tumor in a manner that precluded recurrence and prevented tongue dysfunction.


Neural tube defects result from failure of neural tube fusion during early embryogenesis, the fourth week after conception. The spectrum of severity is not uniform across the various forms of this congenital anomaly as certain presentations are not compatible with extrauterine life  while, on the other hand, other defects may remain undiagnosed as they are entirely asymptomatic (occult spina bifida). We report a child with previously normal neurological development, a devastating clinical course following superinfection of a subtle spina bifida defect which resulted in a flaccid paralysis below the level of the lesion and permanent neurological deficits following resolution of the acute infection and a back closure surgery.  Spina bifida occulta is the most benign presentation of a neural tube defect in newborns. Based on CT scan findings of 228 paediatric patients and 235 adults who underwent abdominal and pelvic imaging for reasons unrelated to the spine, spina bifida occulta seems to be subclinically present in a sizeable proportion of screened children 41.2%) with a much lower figure reported for adults (7.7%) [.2% with This clinical case study was approved of by the Medical Ethics Committee of Kilimanjaro Christian Medical Centre, Moshi, United Republic of Tanzania. Informed written consent was obtained from the child's mother.A two-year-old Tanzanian girl presented with a 1-week history of fevers up to 38.5°C, flaccid lower limb paralysis, and loss of sphincter control. Prior to admission, neurological development had been normal with good sphincter control. Her mother had noticed a small reddish pimple on her back since birth, which had never discharged fluid or grown in size.On examination, the child was febrile with normal level of consciousness, meeting cognitive milestones, normal neurological examination above the waist, and flaccid paralysis of the legs. There appeared to be decreased sensation roughly from the umbilicus down. No structural abnormalities of the lower extremities were appreciated: leg lengths were equal, without atrophy or foot deformity. A soft lipomatous midline swelling of 2 by 2 cm was observed at a low-thoracic level, which had a minute central pore without discharge. Spinous processes could not be palpated in the region surrounding the lesion. Mild thoracolumbar scoliosis and mildly increased lumbar lordosis were observed. However, the child was unable to sit unassisted. Over the course of the admission, she had a single generalised seizure with quick recovery of consciousness and transiently complained of a painful feeling in her right arm. Due to the young age, the postulated meningitis with the present sinus, and seizures in the course of admission to the hospital, we assumed that the pain which the child consistently indicated in the right arm may have a neuropathic origin, for which we prescribed low-dosage amitriptyline.The working diagnosis was superinfection of a dermal sinus from a spina bifida occulta defect in a previously neurologically normal child, leading to spinal cord and/or nerve root compromise and bacterial meningitis. The lower extremity paralysis and apparent sensory level were evidence of spinal cord and/or nerve root compromise, either by direct myelitis/polyradiculitis or by compression from epidural abscess formation. The episode of seizures and the unilateral cervical radiculitis was evidence of a component of meningitis. Alternative diagnoses on the differential included: common bacterial meningitis with secondary involvement of the spinal cord, an inflammatory or infectious polyradiculitis, or thoracic spine Pott's disease with secondary paraplegia.A lumbar puncture could not be performed because of the midline lumbar defect and the suspected infected dermal sinus. Initial lumbar X-ray (limited by underexposure) showed no evidence of vertebral displacement or collapse. An ultrasound examination of the swelling identified a superficial cyst, without clear connection to the spinal column. A Full Blood Picture (FBP) demonstrated an elevated white cell count with a predominance of lymphocytes see .The child was treated with broad-spectrum intravenous antibiotics, ceftriaxone, cloxacillin, and metronidazole, to cover for aerobic and anaerobic causes of bacterial meningitis and abscess. Unfortunately, blood cultures had not been done prior to starting empirical antibiotic therapy. Five weeks after admission, the child and her mother absconded from the hospital due to inability to afford the cost of care. The family was also unable to afford a spinal CT scan (75 American Dollars) during admission.The child and her mother returned to the paediatric neurology clinic for a follow-up examination 10 months later, having managed to afford CT spine. The two-centimetre midline nodule had subsided; only a two-millimetre papule with a small central invagination remained in its place. Over two years, the child's legs had become spastic, left more than right, and she developed persistent urinary and faecal incontinence. She maintained a sense of bladder fullness which facilitated bladder catheterisation. Progressive scoliosis with marked lumbar lordosis was noted see .The CT scan demonstrated multilevel spinal dysraphism and deformity above and below the level of the now-resolved swelling. Axial CT sections revealed a dorsal dermal sinus at the level of T12 connecting the epidermis to the epidural space. (See Figures The dermal sinus caused recurrent bouts of fever with purulent discharge from the lesion. She was enrolled in a NGO-funded surgery program performing back closure operations for newborns with spina bifida. Since the surgical intervention was a free treatment option offered to the mother in the framework of a spina bifida NGO care program, extensive surgical reports were not made available for review. Following the surgery, the local discharge and bouts of fever resolved and, subsequently, her general condition improved. However, her neurological deficits remained unchanged.We present a child with previously normal neurological development and a devastating clinical course following rare superinfection of a subtle spina bifida defect. The diagnosis was evident upon visual inspection of the lesion and knowledge of spinal embryology. However, nearly one year passed before the family could afford imaging to confirm the clinical suspicion and even longer before the defect could be surgically repaired. Unfortunately, delay in diagnosis and treatment is common in rural Tanzania where the vast majority of individuals lack health insurance. The child's mother ran away from the hospital because she was unable to pay the bills. As a result, there had been progressive episodes of clinical infection and irreversible neurological damage. Moreover, it is important to note that spina bifida incidence is significantly reduced by adequate maternal prenatal folate intake. It is possible the defect could have been prevented entirely if the mother had access to prenatal care and adequate nutrition or dietary supplementation.In this case study, the child had a dorsal dermal sinus, which is an epithelium-lined tract beginning at the epidermis and extending to variable depths into underlying structures . ReporteThis manifestation of a neural tube defect is most likely to present at the lumbar and lumbosacral levels of the vertebrae with the cervical region involved in less than 1% of cases . Rarely,Patients with this inconspicuous malformation often come to medical attention with clinical complaints concerning sites of the body below the level of the lesion. Systemic reviews note that the presenting complaints primarily involve neurological or cutaneous abnormalities, signs of infection, urological or bowel abnormalities, and skeletal abnormalities; examples include scoliosis, limb weakness, muscle atrophy, gait disturbances, and congenital talipes equinovarus , 6. In rSince a dermal sinus tract which terminates intrathecally is high-risk for complications of CNS infection, medical consensus is that lesions above the sacrococcygeal level be explored to the terminus and excised . Prompt In summary, the resulting neurological deficits which resulted from an acute infection of the dermal sinus illustrate the potential risks of a spina bifida occulta, a condition generally accepted as a benign abnormality. Additionally, the delayed imaging and surgical treatment represent the arduous circumstances of lacking financial capability to afford medical care in low-income countries.


Giant pheochromocytomas (Pheo) are rare entities requiring clinical suspicion coupled with strategic diagnostic evaluation to confirm the diagnosis. The majority of cases are discovered incidentally. The diagnosis consists of biochemical evaluation and imaging study to localize the mass. Pathological examination confirms the diagnosis. The female patient in this case report presented with chest pain, palpitation of three weeks duration and was found on evaluation to have an abdominal mass concerning for pheochromocytoma. She was treated with surgical resection. The pheo measured 20.5 x 18 x 10 cm and weighed 2,582 grams. Pathological examination confirmed the diagnosis of pheochromocytoma. Pheo is a rare catecholamine-secreting tumor that arises from chromaffin cells in the adrenal medulla. The annual incidence of pheo is about two million and prevalence in the population is 1:6500 . Pheo is2. Initial laboratory investigations revealed white blood cell (WBC) count of 12.7 K/uL, serum glucose 248 mg/dL. The patient has a history of diabetes mellitus type II and hypertension.A 55-year-old woman with a history of abdominal pain of three weeks duration, chest pain, palpitation, dyspnea on exertion and dizziness was referred to our surgical outpatient clinic upon detection of large abdominal and pelvic masses on computed tomography (CT) concerning for adrenal and uterine in origin respectively. Physical examination revealed systolic blood pressure in 170 and sinus tachycardia with pulse rate of 105, body mass index (BMI) 54 kg/mCT revealed a 19 x 14.5 x 15.8 cm mass involving the right adrenal gland . An open right adrenalectomy was performed through a right subcostal incision with extension up the midline to the xiphoid. The mass was completely resected en bloc with portion of the right hemidiaphragm  of the abdomen. The sensitivity of CT scan reaches 85–94% and is approximately 90% for extra-adrenal localizations. The sensitivity of MRI reaches 93–100% . 123I-meGenetic testing is selectively performed as part of the diagnostic evaluation for pheo. Pheo is classically associated with three syndromes: von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN2) and neurofibromatosis type 1 (NF1) . Study sThe management of pheochromocytoma consists of both pharmacological and surgical therapy. The goal of pharmacologic therapy is to control hypertension and associated cardiovascular abnormality in both the preoperative and postoperative settings . Tumor mOther pharmacologic alternatives include the use of specific α1 receptor blockers, such as prazosin or terazosin, as this would avoid the reflex tachycardia that is seen with phenoxybenzamine use ,10. In aThe ultimate treatment for pheochromocytoma is surgical intervention. According to current guidelines, an open resection is recommended for large adrenal adenomas measuring greater than 6 cm or invasive tumors . LaparosIn this report, we presented a case of a 55-year-old super obese female with a giant 20.5 cm pheo with an incidental finding of uterine leiomyoma. Diagnosis of pheo was made by plasma and urine metanephrine levels while imaging was used to localize the mass. Management consisted of providing alpha blocker followed by surgical resection of the mass. Our patient, 11 months after resection, remained stable and disease free. In a patient with multiple surgical indications including pheochromocytoma, as in our case, pheochromocytoma should always be managed first to prevent the risk of adrenergic crisis which can result in increased morbidity and mortality. Multidisciplinary team including cardiologist, endocrinologist, anesthesiologist and the surgeon are needed in the successful management of patients with pheo.


Leiomyoma is a form of benign tumor originated in hypertrophy of the smooth muscles, which is most prevalent in the uterus and gastrointestinal tract. However, Leiomyoma originating from smooth muscle at the vessels lying on deep soft tissue is very rare.Our case was a rare case of leiomyoma originating from the axillary region, which was initially diagnosed as a fibroadenoma on radiological examination. The mass was separated from surrounding tissues and totally resected. Pathologically, hematoxylin–eosin-stained biopsy tissue showed the typical findings of leiomyoma. Postoperative follow-up observation was done for 1 year, without any complications or recurrence.Notably, a leiomyoma in the axillary region is difficult to differentiate from other benign or malignant tumors on preoperative radiological examinations such as ultrasonography or computed tomgraphy. Therefore, when an indefinite asymptomatic mass that is not lymphadenopathy or common benign tumor is identified in the axillary region, leiomyoma can be considered as one of the differential diagnoses. Its presence in the small intestines can cause digestive disorders and bleeding, with some cases of transformation into malignant tumors, such as sarcoma. Leiomyoma development in deep soft tissues is very rare, with most cases typically originating from the blood vessels.,4 Until now, cases involving leiomyoma developing in deep soft tissues have rarely been reported.Leiomyoma is a form of benign tumor originated in hypertrophy of the smooth muscles, which is most prevalent in the uterus and gastrointestinal tract.Our case involved a leiomyoma that developed in the left axillary region. We hereby report this case because it is rare, as well as an examination of its differential diagnoses through literature review.2A 29-year-old man noticed a hard, palpable mass of approximately 5 × 5 cm in the left axillary region, which was found by patients 1 week earlier. Although protrusion of the mass was nonvisible, there was no change in the skin texture. The mass showed a pattern of continued growth; however, there were no other complaints of specific discomfort from the patient. It was difficult to determine the characteristics or diagnose the mass through physical examination alone. On preoperative contrast-enhanced computed tomography (CT), a well-demarcated mass of approximately 3.5 × 3.4 cm, with features consistent with those of a fibroadenoma, was observed at low attenuation in the left axillary region , is essential. Among such tests, ultrasonography is the first choice, since it is not only very useful in distinguishing whether the phase of the mass is solid or cystic but also has a low cost, can identify most anatomical structures, and identify the phase of blood flow through the use of a Doppler image.Leiomyoma in the axillary region is extremely rare, with almost no reported cases.Although accurate initial diagnosis of axillary leiomyomas is difficult, complete resection is required considering the possibility of malignant lesions and differentiating them from breast cancer or lymph node metastasis. In cases of difficult or ambiguous diagnosis of a deep mass in the axillary region, it can be considered one of the differential diagnoses.


Dear Sir,Echinococcus multilocularis, a cyclophyllid tapeworm, is a disease in certain terrestrial mammals, including wolves, foxes, jackals, coyotes, domestic dogs, and humans.Echinococcus granulosus,E. multilocularisproduces many small cysts within the internal organs of the infected animal.We read with great interest the article by Kvascevicius et al entitled “Fatal liver and lung alveolar echinococcosis with newly developed neurologic symptoms due to the brain involvement.”Radiologically, what is most crucial for the follow-up of such a case is that the evaluation of the course of the disease should preferably be made by comparing the studies performed by the “same” imaging tool. Therefore, the comparison between preoperative magnetic resonance imaging (MRI) and postoperative computed tomography (CT) scans poses as a limitation in the current report.There is no doubt that it is important to differentiate AE from cerebral malignancies, such as glial and metastatic tumors, and other infective cerebral diseases involving the cerebrum, including tuberculoma, in patients from areas endemic for hydatidosis. In spite of the fact that CT and MRI are the most accurate diagnostic tools, the presented cystic radiological appearance of the lesion in this patient is unfortunately not pathognomonic for AE.Histopathologically, necrosis with granulomatous reaction composed of multinucleated giant cells and epithelioid cells and inflammatory cells surrounding multiple periodic acid-Schiff-positive cysts are key findings of AE, as seen in the surgical specimen of the current case. Nevertheless, total magnification rate in light microscopes which were not given in Fig. 5 is a significant drawback for the present report because the thoroughness of the presentation of a scientific contribution is as important as a proper content, which apparently falls to the authors of the article.Finally, Schmid et al suggested that gamma knife radiosurgery may also be considered in the treatment of AE.


We present a rare case of intraoral nerve sheath myxoma. Clinically, the neoplasm mimics many other oral mucosal pathosis, underscoring the importance of histopathology in ensuring accurate diagnosis of oral mucosal lesions. Reports of intraoral nerve sheath myxomas are essential to enhance our understanding of this rare intraoral entity. Nerve sheath myxoma (NSM), also referred to as myxoid neurothekeoma, is an uncommon benign peripheral nerve sheath neoplasm which usually arises within the dermis and subcutaneous tissues of the head and neck and upper extremities A 43‐year‐old female was referred to an oral medicine clinic with regard to a small swelling involving the anterior dorsal surface of her tongue, just left of the midline Fig. . The patOn examination, no extraoral abnormalities were identified and there was no palpable cervical lymphadenopathy. Intraorally, a small sessile mass was noted on the anterior dorsal surface of the tongue, just left of the midline. The lesion was nonulcerated and not tender to palpation. No other oral mucosal abnormalities were identified. Based on the history and clinical examination, a provisional diagnosis of a fibroepithelial polyp was made and an excision was recommended and undertaken.An ellipse of oral mucosa measuring 10 × 8 × 8 mm was subsequently submitted for histopathological examination. The sections showed a semipedunculated nonencapsulated, but circumscribed mass covered by stratified squamous epithelium Fig. A. CentraIntraoral NSM is a rare benign tumor, first reported in 1974The 2013 systematic review identified the mean age at diagnosis to be 36 years, although NSMs have been reported to occur across the entire age spectrum, from newborns to the elderly (84 years), with a male to female ratio of 1:1.5 n = 8), smooth muscle actin (n = 5), epithelial membrane antigen (n = 4), or AE1/AE3 (n = 3) The etiology of NSM is unknown, although as many occur in areas subject to trauma, it has been proposed that traumatic injury may play a role Nerve sheath myxomas are not known to be associated with any hereditary conditions and have not been reported to undergo malignant transformation Intraoral NSM represents an unusual and extremely rare, benign neoplasm of peripheral nerve sheath origin. The neoplasm mimics other oral mucosal pathosis underscoring the importance of histopathological examination of lesional tissue. Accurate disease description and appropriate use of terminology are essential in ensuring better understanding of rare entities.AF: was involved in the clinical management and follow‐up of the patient. NF: was responsible for the histopathology. AF and NF: were conjointly responsible for the literature search and preparation of the manuscript. AF: acquired the clinical images, while NF provided the photomicrographs.None declared.


A 29-year-old female patient was admitted after a computed tomography (CT) scan showing a neoplasm measuring 4.5×4.0×4.5cm located in the middle and dorsal part of the left kidney . In the MAF (metanephric adenofibroma), initially reported in 1992 . GalluzzHerein, we describe the CT manifestations of MAF to help make the accurate preoperative diagnosis of MAF, the diagnostic methods of which have not been sufficiently reported. Currently, its confirmed diagnosis mainly depends on postoperative pathology. As for the best treatment modality, surgery is preferred, among which laparoscopic partial nephrectomy is ideal when conditions permit. Although no postoperative recurrence is reported, long-term follow-up is still needed. More clinical cases are required to facilitate formulations of standard treatment and follow-up for MAF.


This patient had an 18-month history of painless inflammatory lesions and erythema on the left forehead and right upper eyelid. The clinical and pathologic characteristics of the CHE were evaluated in the present study.The evidence of the coexistence of variable components of the CHE in our study emphasized the importance of distinctive morphology and biology, and should be considered in the differential diagnosis of other vascular lesions. The hemangioendothelioma was initially considered a type of vascular neoplasm with different combinations and scopes, varying from hemangiomas to highly malignant angiosarcomas. Owing to the morphologic heterogeneity of CHE, however, it was introduced as a new diagnosis. This tumor has different types of grading, including benign, low-grade malignant, and malignant. The differential diagnosis of CHE is crucial due to the morphologic heterogeneity of CHE and it plays an important role in the careful evaluation of CHE. A biopsy test result is very important to determine the combination of vascular components and the features of individual vascular patterns [Composite hemangioendothelioma (CHE) is a type of recently described vascular tumor named hemangioendothelioma (HE). This may be benign or malignant depending on the particular group member’s function. This lesion is known as a low-grade malignant neoplasm. In most cases, the CHE are solitary lesions that occur as dermal papules or nodules; however, they may be multiple. The colors of these lesions are commonly red, purple, or black violaceous, and they can be scaly or hemorrhagic , 2. Accopatterns . This stThis study presented a case of a 78-year-old female with an 18-month history of painless progressive swelling and erythema on the left forehead and right upper eyelid. The lesions were slightly developed and involved all of the scalp surface, eyelids, and face; however, there was no other abnormality. The chief complaint of the patient was the mass on the right eyelid. She had a proptosis of the right eye and an orbital mass; however, there were no signs of headache, nausea, vomiting, or double vision. The medical history of the patient demonstrated that she had hypertension and coronary artery disease, and had undergone angioplasty. However, she had no history of undergoing radiotherapy or chemotherapy. The result of the brain MRI in May 2015 demonstrated a mucosal thickening in the right maxillary sinus. A soft tissue with a slightly lobulated surface was observed using magnetic resonance imaging (MRI). The ventricles, subarachnoid space, and brain stem demonstrated normal appearance, and there was no evidence of mass lesion or midline shift. The MRI with and without contrast, performed 2 months later, revealed a tissue measuring 5 × 3 × 1 cm and weighing 12 g in the right orbital mass. The microscopic findings demonstrated a benign lesion composed of a mixture of angiomatous tissue and lobulated fatty tissue connected with skeletal muscle tissue, which was suggestive of hemangioma. The brain computed tomography (CT) findings showed that there were no abnormalities in the brain structure. There was diffused skin and subcutaneous soft tissue edema in the cranial area, which was more pronounced in the radiation therapy site. The ultrasound reviewed an inflammatory soft tissue in the right frontotemporal region progressing to the ear. The patient used atenolol 50 mg (twice daily), triamterene-h , acetylsalicylic acid (daily), and atorvastatin 20 mg (daily). In July 2016, she was hospitalized in the infectious diseases ward with a diagnosis of cellulitis and received oral antibiotic therapy  for 2 weeks. Owing to the lack of response to the treatment, she was then referred to the dermatology department. Swelling and slightly warm soft tissue along with erythema was observed throughout the facial surface and scalp. The swelling of the eyelids resulted in complete closure of the eyelids on both sides, which impaired the patient’s vision; however, the patient’s eye exam was normal Fig. . FurtherComposite Hemangioendothelioma (CHE) is a new entity classified under the Heman gioendothelioma (HE) group of tumours. It is an extremely rare vascular neoplasm of low to moderate malignancy. Based on the previous studies, the CHE may present at any age and its size ranges from 0.7–30 cm. These lesions are usually composed of circumscribed nodules, plaques, or ulcerated tumors. The risk of CHE was reported to be higher in females than males , 6. It cIn our study, there were various components of the CHE and it was unique due to site and extent of the lesion. Therefore, the diagnosis of the CHE features is very important and should be considered in the differential diagnosis of other vascular lesions.


Juvenile nasopharyngeal angiofibroma is the most common benign tumor of the nasopharynx with complaints of unilateral nasal obstruction and recurrent nosebleeds in the young male population. Despite being a benign tumor, it can be aggressively destructive in surrounding tissues and bones by acting locally. The gold standard treatment method is the surgical excision of the tumor. This case report is a case of angiofibroma, a 32-year-old asymptomatic male patient with no evidence of clinical signs and endoscopic examination, which is recognized as a localized vascular mass lesion in the right sphenoid sinus on the cranial MR imaging. We prepared this case report that may represent an angiofibroma localized only within the sphenoid sinus which is very rare in the literature. Juvenile nasopharyngeal angiofibroma (JNA) usually destroys adolescent male patients aged 14–25 years and accounts for 0.5% of head and neck tumor . It is iA 32-year-old asymptomatic male patient who was followed up for multiple sclerosis in the neurology clinic was referred to ENT clinic. The cranial MR imaging revealed an intensely vascular lesion which contained hypointense areas with an expansile growth pattern within the right sphenoid sinus . Nasal eJNA is a histologically benign, nonencapsulated, locally aggressive, and vascular-originated neoplasm and a rare tumor in adolescents , 4. AlthDespite the use of sclerosing agents, cryotherapy, hormonal therapy, and preoperative embolization in JNA treatment, the most effective treatment methods are nonsurgical techniques combined with radiotherapy and preoperative angiography. The gold standard method is the surgical excision of tumor . JNA canThe nature of our case which is very rare in the literature, the JNA is completely localized within the sphenoid sinus. Therefore, it is diagnosed incidentally by imaging methods taken for other reasons as mass of the sphenoid sinus without any clinical symptoms and examination findings.


Hypercalcemia and severe osteolytic lesions are rare complications of acute lymphoblastic leukemia (ALL) in childhood. We report a case of a 3 years old boy who presented with prolonged fever, nausea, vomiting and increasing lower limbs pain. Skeletal X-rays and CT scan showed severe osteolytic lesions of the skull and extremities. Her physical examination showed multiple cervical lymph nodes. In laboratory tests, he had severe hypercalcemia. Parathyroid hormone (PTH) was not elevated. Despite the absence of circulating blasts, bone marrow biopsy revealed B-precursor (ALL). Hypercalcemia was initially treated with intravenous isotonic sodium chloride solution and diuretics but the serum calcium level normalized only after the beginning of corticosteroids and chemotherapy. The child responded initially to chemotherapy and eventually relapsed and died of septic shock. Acute leukemia must be considered in differential diagnosis in patients with hypercalcemia. A detailed examination even when there no circulating blasts in their peripheral blood smear, and if in doubt bone marrow aspiration should must be taken into consideration. Acute lymphoblastic leukemia (ALL) is the most common form of acute leukemia in children . It usuaAcute lymphoblastic leukemia (ALL) is the most common form of acute leukemia in children . It usuaHypercalcemia is a rare metabolic complication and occurs in less than 1% of children with cancer . In the Our case as well as the review of the literature confirms that in childhood ALL presenting with hypercalcemia, the main symptoms are non-specific: anorexia, vomiting, abdominal pain, constipation, confusion, bradycardia and these signs may precede those related to intramedullary invasion by blast cells . When hyThe hypercalcemia is a metabolic emergency in children and it may be complicate acute leukemia at diagnosis. Therefore, a bone marrow examination should not be delayed in these cases even if the physical examination is normal and there are no circulating blasts in the peripheral smear. Once identified it can be rapidly treated with a combination of intravenous isotonic saline, diuretics, calcitonin and bisphosphonates. Early treatment of leukemia helps to correct quickly hypercalcemia along with other therapies.


We report a rare case of an elderly woman presented with right renal mass with invasion of renal vein and several small lymphadenopathy in the hilar area. The diagnosis of kidney cancer is suspected and the patient underwent open radical nephrectomy, surrenalectomy and lymphadenectomy dissection. The pathologic examinations find a rosai dorfman disease. This unusual benign entity is uncommon in the kidney, but in medical imaging, it may simulate an infiltrative renal neoplasm, especially a lymphoma or leukemia or even renal cell carcinoma. A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder. Rosai-Dorfman disease (RDD) or sinus histiocytosis is a rare benign disease of unknown etiology. Classically, it is characterized by lymphadenopathy mainly in the cervical area associated with fever, leukocytosis, polyclonal gammopathy, and an elevated erythrocyte sedimentation rate. Histopathologically an inflammatory proliferation of S 100 protein-positive histiocytes characterized by lymphophagocytosis is seen. Most cases involve patients in their first and second decades, but all age groups can be affected . Extrano68-year-old woman presented with hematuria and right flank pain since 3 years. The patient had no history of smoking and no other medical problems. The physical examination revealed a sensitivity of a lumbar fossa and had not revealed any adenopathy, fever or weight loss. Laboratory examinations were normal. The abdominal and pelvic Computed tomography scan showed the presence of a large tissue process at the upper pole of the right kidney measuring 8 cm of diameter, demonstrating heterogeneous contrast enhancement.this process extends over the hilar area with invasion of the right renal vein, while the lower vena cava is permeable. Several small lymphadenopathy in the right hilar area . There iRosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. It is classified as a non-neoplastic histiocytic disorder and is classically characterized by massive lymphadenopathy secondary to infiltration and dilation of the lymph node sinuses by large histiocytes, mainly in the cervical area.Generally manifests in children or young adults with massive cervical lymphadenopathy, fever, leukocytosis, an increased erythrocyte sedimentation rate and hypergammaglobulinemia. Other lymphatic groups such as mediastinal, axillary and inguinal lymph nodes can also be affected . The etiTo our knowledge there only four cases of RDD in kidney cases have been reported with two of them diagnosed with adenocarcinoma of the prostate. In a rare case reported by Buchino et al, the kidney was only focally involved with a single small mass in the lower pole that contained an admixture of histiocytes, lymphocyte and plasma cells . In anotThe differential diagnosis of RDD in kidney includes malignant fibrous histiocytomas and histiocytic proliferations of infectious etiology (the presence of S100 is useful in discriminating these lesions), leukemia or lymphoma, especially when accompanied by lymphadenopathy (absent of emperipolesis and Immunohistochemistry profiles help to correct diagnosis). Other possible differential diagnoses include storage disease, tuberculosis or even renal cell carcinoma, a metastatic tumor such as malignant melanoma. On the basis of the sonographic and CT appearances, we could not differentiate this entity and biopsy was recommended to exclude those diagnosis. The clinical course varies widely from spontaneous remission to death from vital organ infiltration. Patients with renal involvement have been associated with poorer outcomes, with 40% of patients dying of the disease and the remainder having persistent involvement , 8. A deRosai-Dorfman disease of the kidney, although rare, should be considered in the differential diagnosis of renal mass especially when it is bilateral.in computed tomography scan The appearance can resemble renal cell carcinoma and lead to radical nephrectomy.