A perforation occurring during colonoscopy is an extremely rare complication that may be difficult to diagnose. It can be responsible for acute abdominal compartment syndrome, a potentially lethal complex pathological state in which an acute increase in intra-abdominal pressure may provoke the failure of several organ systems.We report a case of acute abdominal compartment syndrome after perforation of the bowel during a colonoscopy in a 60-year-old North African man with rectal cancer, resulting in respiratory distress, cyanosis and cardiac arrest. Our patient was treated by needle decompression after the failure of cardiopulmonary resuscitation. An emergency laparotomy with anterior resection, including the perforated sigmoid colon, was then performed followed by immediate anastomosis. Our patient remains alive and free of disease three years later.Acute abdominal compartment syndrome is a rare disease that may occasionally occur after a colonoscopic perforation. It should be kept in mind during colonoscopy, especially considering its simple salvage treatment. Colonic perforation due to colonoscopy rarely occurs but still remains a major complication with a high rate of morbidity and mortality and often needs surgical management . The freAbdominal compartment syndrome (ACS) refers to organ dysfunction that may occur as a result of increased intra-abdominal pressure (IAP),8. It maWe report a life threatening case of primary acute abdominal compartment syndrome (AACS) resulting from iatrogenic colonic perforation during a diagnostic colonoscopy.We report the case of a 60-year-old Moroccan man admitted for the surgical management of a high rectal adenocarcinoma. He had no past history of cardiovascular or pulmonary disease, with no recent surgery, and was classified according to the American Society of Anesthesiology (ASA) as ASA II. He was scheduled to undergo a second colonoscopy by an expert endoscopist to eliminate a second colonic tumor before an anterior resection. Preoperative anesthesia investigations, including all blood tests, a chest X-ray and an electrocardiogram, were unremarkable.On the day of the colonoscopy, our patient's blood pressure was 120/90 mmHg, his heart rate was 75 beats/minute, his respiratory rate was 12 breaths/minute, his body temperature was 37°C and he had a blood oxygen saturation level of 99% at ambient air.Our patient had the procedure under conscious sedation with the presence of an anesthetist nurse. During the first five minutes of the procedure, the endoscopist reported some difficulties, but no signs of perforation, that caused a little discomfort and pain to our patient, which was managed with two 1 mg intravenous bolus injections of midazolam. This allowed our patient to remain awake and follow the endoscopist's instructions.At that time his vital signs were unchanged except of a rise in his heart rate. At the end of the examination of his ascending colon, our patient developed progressive bradycardia. The endoscopist noticed a significant abdominal distension followed by respiratory distress that rapidly progressed to cardiac arrest. The procedure was immediately stopped and cardiopulmonary resuscitation begun. The reanimation team was called for intubation while closed chest cardiac massage was initiated by the endoscopic and surgical team.Our patient was intubated and manually ventilated with 100% oxygen and epinephrine was administered. Despite these measures, the cyanosis worsened and his pulse was still not palpable.After five minutes, a 14-gauge needle was used to decompress the abdominal tension as a last procedure to avoid a salvage thoracotomy  results in physiologic derangements in several organ systems ,13. ACS This resulting intra-abdominal hypertension (IAH) leads to both cardiologic and pulmonary symptoms ,13. The The most common causes of increased IAP are abdominal surgery complicated by pre- or postoperative bleeding, acute abdominal trauma, retroperitoneal bleeding after aortic surgery and extended burns to the abdominal wall . HoweverThe unique and specific treatment of this exceptional acute state, when suspected, is abdominal decompression, performed by a decompressive abdominal laparotomy or an air puncture. Although the use of a 14-gauge needle seems to be so simple as to be inadequate, in our case it represented the salvage procedure after the failure of cardiopulmonary resuscitation. It permitted decompression of the IAH, restoring a normal pulse, blood pressure and respiratory rhythm.This can be explained by the high compliance and elasticity of the abdominal wall, similar to the pericardium. The pericardial pressure to volume relationship is curvilinear : the peret al., applied to IAH, that shows that there is a linear relationship between the volume introduced or extracted from the abdominal cavity and intra-abdominal pressure [There is a prediction model described by Papavramidis pressure . This hapressure ,15. Othepressure .The management of iatrogenic colonic perforation is still controversial, with two options-operative and non-operative management. However, given the high risk of mortality (up to 50%) and morbidity of these complications, many authors insist on quick surgical management .The absence of any significant contamination (after colonic preparation for this procedure) makes it possible to perform an initial repair or, like in our case, radical surgery with a hemicolectomy and an immediate colorectal anastomosis.To the best of our knowledge, only two similar cases have previously been reported. In the first case, the patient underwent conservative treatment to manage a colonic perforation resulting from a diagnostic colonoscopic examination of a recently known constructed mucus fistula . In the During a colonoscopy, the endoscopist and anesthesiologists should be aware of this rare complication that may occur due to asymptomatic colonic perforation, and keep in mind the salvage action of fine needle decompression, because failure to recognize and treat AACS is inevitably fatal.AACS: acute abdominal compartment syndrome; ACS: abdominal compartment syndrome; ASA: American Society of Anesthesiologists; IAH: intra-abdominal hypertension; IAP: intra-abdominal pressure.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AS was the author in charge and wrote the manuscript. RM, LI and AB helped in writing the manuscript. HOEM was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


A 61-year-old female underwent right hemihepatectomy and caudate lobectomy for hilar cholangiocarcinoma in 1999. Ten years later, increasing serum carbohydrate 19-9 was detected by routine follow-up. Subsequent positron emission tomography revealed an asymptomatic lesion in the right 11th rib. As the mass steadily grew in size, the lesion was resected en bloc with the affected rib and muscle. The histopathological findings closely resembled those of the primary cholangiocarcinoma. Thus, the tumor was diagnosed as a metastatic recurrence 10 years after resection of the primary tumor. There have been a few reports of cholangiocarcinoma recurrence in long-term survivors at the surgical margins, peritoneum, or transhepatic drainage route. However, there are no reports of solitary extra-abdominal recurrence. This case highlights the need for careful follow-up of patients with cholangiocarcinoma and nodal metastasis, even in the absence of recurrence for >5 years after curative resection. Bile duct cancer has been associated with a poor prognosis; node metastasis is a particularly important poor prognostic factor –6. DespiA 61-year-old female was diagnosed with hilar cholangiocarcinoma in 1999. It mainly affected the right hepatic duct, but also extended to the left hepatic duct and proximal common bile duct. Percutaneous transhepatic cholangio drainage tubes were inserted into the anterior brunch and posterior brunch approached from the 7th intercostal space and into the posterior brunch approached from the 9th intercostal space. The patient underwent right hemihepatectomy and caudate lobectomy after portal vein embolization. The tumor was 2.2 cm in diameter and infiltrated to the liver parenchyma. The cystic node and retroportal nodes were histologically involved. According to the sixth edition of the UICC TNM classification , the tumIn September 2008, a sudden increase of the serum CA19-9 level to 68 U/mL  was noted. The site of recurrence could not be identified on CT. Positron emission tomography (PET) revealed an elevated standard uptake value of 3.4 in the 11th right rib. We chose the wait-and-watch approach because of the low suspicion of metastasis and the patient’s wishes. The wait-and-watch approach was adopted in view of the absence of any bone destruction, mass formation, or symptoms. In February 2009, the serum CA19-9 level increased from 68 to 207 U/mL. PET again revealed a solitary accumulation in the 11th right rib and adjacent tissues, with an increase of the standard uptake value from 3.4 to 5.8 (Fig. In April 2009, a tumor biopsy was performed under general anesthesia by an orthopedic surgeon. Intraoperative frozen section diagnosis revealed adenocarcinoma, and tumor resection was carried out en bloc, along with the 11th rib, adjacent intercostal muscle, and surrounding tissues.Histopathological examination of the resected specimen revealed metastatic adenocarcinoma of the costal bone with invasion of the adjacent intercostal muscle. The tumor morphology closely resembled that of the resected primary bile duct cancer. An immunohistochemical study was performed Fig. , which rOver a period of eighteen months after the second surgery, while the patient was observed without systemic chemotherapy, the serum CA19-9 level decreased. CT and PET showed no evidence of recurrence; therefore, close follow-up has continued.Long-term results of patients with cholangiocarcinoma, especially with nodal metastases, are unsatisfactory. Several investigators have reported a mean survival time of 12–22 months in patients with nodal metastasis , 6. DiseRecurrences many years after the treatment may be related to long-lasting tumor dormancy being turned on, especially in distant organs –21. HolmOtherwise, the immune system is able to effectively mobilize against tumor invasion. Steffen et al.  reportedIn cholangiocarcinoma, CA19-9 is elevated in 50–79 % of patients . The diaIn conclusion, in patients with bile duct cancer, it must be emphasized that long-term surveillance is required even in patients without recurrence for >5 years after curative resection of the primary tumor, even if there are no early signs of recurrence.


Ectopic tooth eruption may result owing to one of 3 processes: developmentalDisturbance, iatrogenic activity, or pathologic process, such as a tumor or a cyst. In rare cases, occlusion of the sinus ostia may predispose a patient to develop a maxillary sinus mucocele. When the maxillary sinus is invaded, symptoms usually occur late in the process.A 17 years old boy referred to department of Otolaryngology, Head and Neck Surgery of university of medical sciences, Tabriz_Iran in 2010 with chronic recurrent mucoprulent discharge from retromollar trigone , posterior to right superior alveolar ridge. CT scan revealed a dense mass resembling tooth, obstructing sinus ostium with homogenous opacity with ring enhancement, occupying whole sinus and expanding all walls. A Caldwell Luke approach in combination with endoscopy was selected.In the present patient, removal of ectopic tooth resolved the symptoms completely, the fistula obstructed and discharges discontinued. An ectopic tooth is a rare entity obstructing sinus ostium. The etiology of ectopic eruption has not yet been completely clarified, but many theories have been suggested,including trauma, infection, developmental anomalies and pathologic conditions, such as dentigerous cysts. In summary, although the ectopic teeth is rare but it would be assumed in presence of unilateral symptoms of sinonasal cavity. Therefore in peristant unilateral sinonasal symptoms we should complete examining of this site to rule out rare causes of these symptoms. Ectopic tooth eruption may result from one of the 3 processes:developmental disturbance, iatrogenic activity, or pathologic process, such as a tumor or a cyst ,2. DentiIn rare cases, occlusion of the sinus ostia may predispose a patient to develop a maxillary sinus mucocele . When thEctopic teeth obstructing sinus ostium is a rare condition. It causes morbidities sometimes differentiated with the most common causes of sinus ostium obstruction like foreign bodies, sinonasal tumors of any origin, sinonasal polyposis and etc. Trauma, infection, genetic, teeth crowding, dense alveolar bone, iatrogenic causes like dentistry and developmental disorders are some causes of ectopic teeth in sinonasal cavity ,15.Here we report a patient who was referred because of chronic mucoprulent discharge from retromollar trigone. This condition was due to dentigerous cyst caused by an ectopic tooth near the sinus ostium, that was compelling with its drainage.A 17 years old boy referred to department of Otolaryngology, Head and Neck Surgery of Tabriz University of Medical Sciences in Iran in 2010 with chronic recurrent mucoprulent discharge from retromollar trigone, posterior to right and to superior alveolar ridge. The patient also had fullness sensation on his right cheek, halitosis, posterior nasal discharge, pain and swelling around a fistulous site where drainage occurred. Office examination revealed a 2 mm point with mucoprulent discharge in retromolar trigone. A 4 mm 0 degree rigid rod endoscope introduced to nasal cavity to rule out any obstructing mass in nasal cavity and middle meatus. Mucoprulent discharge from middle meatus was apparent in nasal endoscopy. Multiple courses of medical treatment with various generations of antibiotics had been used for 1years. Imaging like waters and panoramic X-ray revealed no definite diagnosis until he referred to our institute. He had no previous history of trauma, dentistry, infection and no evidence of developmental disorders.Previous imaging was studied again and in panorex X-Ray an opaque mass was seen near the right maxillary sinus antrum, which was missed during previous visits. CT scan revealed a dense mass resembling tooth,near the accessory sinus ostium with near total opacification of maxillary sinus. Anterior and lateral walls had been eroded in lower cuts .A Caldwell Luke approach in combination with endoscopy was selected. A sub labial incision made, preserving 2 mm of mucosa on gingival side. Dissection was made in sub periosteal plane, up to infra orbital bundle. Erosion was seen on anterior wall of maxillary sinus. The anterior wall was fenestrated with a 4 mm direct osteotome. A cystic mass in grayish color filled with mucoid secretions dissected from sinus walls, erosion in lateral and posterior wall was seen. The ectopic tooth has been placed near the sinus ostium, adjacent to floor of the orbit, compelling with sinus drainage. Using a 4 mm rod lens endoscope, the ectopic tooth extracted and uncinectomy and antrostomy was completed.A fistulous tract was found in floor of sinus draining to retromolar trigone. It was sealed off with muscle & adipose tissue .The ectopic tooth had four roots and it was consistency lesser than normal teeth.Because of negative history of tooth extraction and location of fistula in retromolar trigone, it could not be due to tooth eruption. Pathologic examination revealed dentigerous cyst and no evidence of dental origin mass lesions was found.An ectopic tooth is a rare entity obstructing sinus ostium. The etiology of ectopic eruption has not yet been completely explained, but many theories have been suggested, including trauma, infection, developmental anomalies and pathologic conditions, such as dentigerous cysts 16). The etiology of ectopic teeth in the maxillary sinus in our patient is unknown. Ectopic molars and supernumerary teeth are usually discovered on routine radiographic examinations, because most of the cases are asymptomatic . The den6. The etTimur saieem reported a 45 years old man with 2-month history of haemoptysis. Morton Litwin in 2008 reported a 57 years old black woman with dentigerous cyst and an impacted tooth in orbital rim that presented with facial swelling ,13. In 2Water view, panoramic radiography and plain skull radiography are simple and inexpensive methods that can be used in daily practice . CT scanThe standard treatment for a dentigerous cyst is enucleation and extraction of the cyst-associated impacted or unerupted tooth ,21,28,29Marsupialization is another advisable treatment to preserve the cyst-associated tooth and promote its eruption -36. TakaIn summary, although the ectopic tooth is rare, it would be assumed in presence of unilateral symptoms of sinonasal cavity. Therefore in persistent unilateral sinonasal symptoms we should completely examine this site to rule out rare causes of these symptoms.


A 71-year-old man was referred to us for investigation of a liver mass and adenomyomatosis of gallbladder. Findings on ethoxybenzyl diethylenetriamine-enhanced MRI (EOB-MRI) led to a presumptive diagnosis of a 1.5 cm hepatocellular carcinoma (HCC) in the right posterior lobe of the liver. Transcatheter arterial chemoembolization and radiofrequency ablation of the tumor were attempted. After 2 months, CT scan and EOB-MRI showed that the tumor had enlarged to 3 cm. Positron emission tomography (PET) confirmed abnormal metabolic activity with a high standardized uptake value of 7.3 in the lesion. These findings could indicate malignancy such as well-differentiated HCC or cholangiocarcinoma or a benign lesion such as hepatic abscess. Histopathological examination of a liver biopsy revealed a granuloma with many inflammatory cells, leading to a diagnosis of inflammatory pseudotumor of the liver. We report a rare case of hepatic inflammatory pseudotumor with enhancement on EOB-MRI and increased uptake on PET, mimicking HCC. Inflammatory pseudotumor (IPT) is a rare benign tumor that may develop in various organs . HoweverWe herein report a case of IPT of the liver in a patient with cirrhosis, with imaging features mimicking HCC.A 71-year-old Japanese man was referred to our out-patient clinic in 2012 for investigation of a 1.5-cm liver mass and adenomyomatosis of gallbladder, which were detected incidentally by ultrasonography (US). γ-guanosine triphosphate, 242 IU/L , CEA, 4.1 ng/mL ; CA19-9, 23.2 U/mL ; alpha fetoprotein (AFP), 11.3 ng/mL ; protein induced by vitamin K absence or antagonists-II (PIVKA-II), 11 ng/mL . His daily alcohol intake was over 80 g for 40 years. Hepatitis B surface antigen, hepatitis B e-antigen, and hepatitis C virus antibody were negative. The patient had no history of medication. Physical examination was normal. No stigmata for chronic liver disease was identified. Superficial lymph nodes were not palpable. The lungs were normal on auscultation. A chest radiograph and an electrocardiogram were normal. The results of biochemical analysis of the blood were total bilirubin, 0.8 mg/dL ; direct bilirubin, 0.1 mg/dL ; aspartate aminotransferase (AST), 45 IU/L ; alanine aminotransferase (ALT), 50 IU/L ; alkaline phosphatase, 419 IU/L ; Ultrasonography reviewed a lesion in the liver, which was isoechoic to surrounding parenchyma. Computed tomography (CT) showed a 1.5-cm mass in the right posterior lobe of the liver, with contrast enhancement in early phase and washout with rim enhancement in delayed phase, together with definite findings of cirrhosis such as loss of hepatic volume or hypertrophy of the left liver and caudate lobe. The imaging findings thus indicate cirrhosis with IPT. The etiology might be alcoholic liver injury judging from his alcohol intake (over 80 g daily for 40 years). In MRI examination, the tumor was isodense on T1-weighted image and partially round and hyperintense on T2-weighted image. Dynamic contrast-enhanced MRI with gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid (EOB-MRI) was performed. In the arterial phase following contrast injection, the center of the tumor was enhanced intensely and homogeneously. In the delayed phase and hepatobiliary phase, the tumor was completely washed out , similarAfter 3 months, CT revealed a 3 cm mass located in the right posterior lobe of the liver showing early phase contrast enhancement and delayed phase washout with rim enhancement. MRI revealed that the mass was entirely hypointense on a plain T1-weighted image and hyperintense on plain T2-weighted image. On EOB-MRI, the tumor was enhanced intensely and homogeneously in arterial phase and entirely hypointense in the delayed phase and hepatobiliary phase . An FDP-A histopathological diagnosis of IPT was established. After the diagnosis, antibiotics were administered for one week. Two months after the diagnosis, the IPT was reduced spontaneously from 30 mm to 15 mm . Escherichia coli [Staphylococcus aureus [Certain bacteria including hia coli , Staphyls aureus , and Gras aureus  have bees aureus , 14. It The IPT in our case was histologically composed of neutrophils, lymphocytes, plasma cells, and fibroblastic cells. Someren  classifiRecently, IPT can be classified pathologically into two types: fibrohistiocytic and lymphoplasmacytic, from IgG4-related disease . FibrohiIn the present case, findings observed in the liver biopsy, especially the focal CD10 positivity and neutrophilic infiltration, may suggest changes secondary to previous treatment. Heat injuries by RFA involving adjacent organs and Glisson's sheaths have been reported to cause complications such as cholecystitis . In our In conclusion, this paper reports for the first time the EOB-MRI and FDP-PET findings of IPT of the liver, which is difficult to differentiate from malignant tumor. In addition, our case may be a rare case of enlargement of hepatic IPT induced by TACE or/and RFA. IPT should be suspected when treatment for a suspected liver lesion, whether it is HCC or abscess, does not produce expected response.


Amputation neuromas typically arise in injured peripheral nerves; rarely, however, they arise in the spinal cord. We report a rare case of intramedullary amputation neuroma associated with ependymoma in the cervical spinal cord. A 73-year-old woman presented with a 5-year history of progressive gait disturbance. Neurological examination revealed complete motor deficit of her hands and legs. Magnetic resonance imaging of the cervical spine revealed an enhancing mass within the spinal cord at the C6/7 level. The patient underwent C5–C7 laminectomy surgery. During resection of the spinal tumor, we found a whitish string resembling an aberrant nerve root or schwannoma with adhesion to the tumor on the ventral side of the spinal cord. After resecting the tumor, the surgical specimen was cut and separated into a soft greyish tumor  and the tough whitish string. Histopathological and immunohistochemical examination revealed the former was a spinal ependymoma and the latter was a neuroma. An intramedullary amputation neuroma associated with a spinal ependymoma is rare, and this is the first known case in which intraoprerative findings were clearly shown. Neurosurgeons should be aware that spinal ependymomas might coexist with neuromas. Amputation neuromas are non-neoplasm tangles consisting of myelinated axons and Schwann cells that sprout from the end of an injured nerve. They typically arise in the peripheral nerves; rarely, however, they arise in the central nervous system, from the spinal cord or brain stem. For example, most of those previously described were discovered in association with or without a history of trauma –9 and diAlthough microscopic examinations and findings of neuromas in the spinal cord have been reported , 7, 9–13A 73-year-old woman presenting with a 5-year history of progressive gait disturbance was referred to our hospital. She had no past history of trauma or particular disease.Neurological and physical examination revealed complete motor deficit of the hands and legs with severe muscle atrophy. She had apparent sensory disturbance of her hands, trunk, and legs. She could not stand up by herself.Magnetic resonance (MR) imaging of the cervical spine revealed an intramedullary lesion as a hypointense mass on T1-weighted images and a hyperintense mass on T2-weighted images at the C6/7 level. T2*-weighted images detected intramedullary microhemorrhage around the mass. After gadolinium injection, peripheral enhancement of the intramedullary lesion was visualized (Fig. The patient underwent surgery in the prone position. A C5–C7 laminectomy was performed to expose the lesion. After dural opening, we found spinal cord swelling. A midline myelotomy was performed and a grayish tumor immediately appeared. The tumor was sharply circumscribed and could be easily resected. When we dissected the ventral side of the tumor near the anterior median sulcus, a whitish string resembling an aberrant nerve root appeared with adhesion to the tumor Fig. a. It wasThe specimen was cut and separated into the soft grayish tumor and the tough whitish string. Histopathological examination of the soft grayish tumor revealed perivascular pseudorosettes of small cells with round nuclei and fibrillary elements Fig. a. True eHistopathological examination of the tough whitish string revealed the proliferation of interlacing large and small bundles of spindle cells with elongated nuclei Fig. a, which Although her postoperative course was uneventful, the patient did not completely recover from the motor deficit of her hands and legs until 6 months after the operation. Postoperative MR imaging revealed no residual tumor.Extramedullary neuromas arising from spinal nerve roots in the CNS have been described in the literature , 3, 8, aIntramedullary neuromas might arise from errant axonal migration during embryogenesis , and canAs far as we know, only one report has described amputation neuromas associated with spinal ependymomas, and 5 of these cases were microscopic . They alTo the best of our knowledge, no reports have shown operative findings of intramedullary macroneuromas associated with spinal ependymomas. This is the first report with images showing operative findings of the intramedullary neuroma. Our operative findings were that the intramedullary neuroma existed on the ventral side of a ependymoma and passed rostrocaudally, probably close to the anterior median sulcus. These findings seem to support the hypothesis that intramedullary amputation neuromas might derive from intramedullary peripheral nerve twigs around the anterior median sulcus . In our During surgery, we found a whitish string in spinal cord passing rostrocaudally and attached to the soft tumor; we could not, however, understand what it was. Although histopathological examination enabled easy diagnosis of the primary spinal tumor as spinal ependymoma, another specimen attached to the spinal tumor, part of the whitish string, had features quite different from those of ependymoma. It comprised a proliferation of interlacing bundles of spindle cells with elongated nuclei. At first we believed this specimen was a small schwannoma coexisting with an ependymoma. Immunohistochemical examination, especially immunoreactivity for neurofilament protein, finally led to the exact histological diagnosis of the whitish string in the spinal cord as a neuroma.Intramedullary neuromas might occur not only with ependymoma but also with other slow-growing tumors, for example subependymomas, pilocytic astrocytomas, and hemangioblastomas. Neurosurgeons should be aware that these tumors might coexist with neuromas, which can be found rarely during surgery. Moreover, if the resected tumor has two different components on microscopic examination, immunohistochemical examination is needed to diagnose an intramedullary “non-neoplastic” neuroma associated with the primary tumor. This should be distinguished from an intramedullary schwannoma, which is a neoplasma without aberrant axons. Pathologists should also be aware of this distinctive intramedullary tissue, so it is not confused with a neoplasm.


A 71-year-old man was diagnosed with an aggressive mantle cell lymphoma and was started on six cycles of R-CHOP chemotherapy. Approximately two weeks after starting his first cycle of chemotherapy, he complained of severe right lower quadrant abdominal pain, and an abdominal CT scan demonstrated an enlarged appendix with evidence of contained perforation. The man underwent open appendectomy for acute appendicitis and recovered. The appendectomy specimen was submitted for routine pathological analysis. There was histologic evidence of perforation in association with an inflammatory infiltrate with fibrin adhered to the serosal surface; scattered small lymphoid aggregates were present on the mucosal surface. Although the lymphoid aggregates in the submucosa and lamina propria were rather unremarkable by routine histologic examination, immunohistochemistry revealed the lymphocytes to be predominantly Cyclin D1-overexpressing B cells. To our knowledge, this is the first reported case of acute appendicitis in association with appendiceal involvement by mantle cell lymphoma. An appendectomy specimen collected in a clinical scenario supportive of acute appendicitis is a common sample received in gross rooms for routine pathology analysis. Indeed, the relative simplicity of the specimen makes it a rite of passage for medical students and new residents first approaching the pathology gross examination. While the pathologic examination of the appendix typically supports the clinical impression of acute appendicitis, routine histopathological analysis plays a crucial role in evaluating for occult pathologic conditions, including neoplasia. Carcinomas are the most frequent neoplasms encountered in the appendix; however, other less frequently encountered neoplasms include carcinoid tumors and lymphomas . LymphomA 71-year-old man complained of a two week history of right-sided abdominal pain that started after he began his first round of chemotherapy with R-CHOP with pegfilgrastim for mantle cell lymphoma. He presented to the emergency department (ED) after he developed severe (9 out of 10) right lower quadrant abdominal pain, approximately 17 days after starting chemotherapy. A physical examination in the ED revealed tenderness most pronounced in the right lower quadrant, with guarding and rebound pain. Lymphadenopathy was also noted in the cervical and supraclavicular regions. Imaging by CT scan of the abdomen/pelvis revealed an enlarged appendix with disruption of the appendiceal wall and small foci of gas and fluid consistent with a contained perforation. No associated mass or tumor was observed in the imaging studies. Peripheral blood was collected and sent for complete blood count, which demonstrated a normocytic anemia with a relative neutrophilia.The patient's mantle cell lymphoma had been recently diagnosed on the basis of involvement of bone marrow and cervical lymph nodes. Imaging studies revealed widespread lymphadenopathy and splenomegaly. The mantle cell lymphoma expressed CD5 and kappa light chain restriction by flow cytometry and expressed Cyclin D1 by immunohistochemistry. The mantle cell lymphoma was noted to have a high Ki-67-defined proliferative rate of 60–80%, a finding that suggested an aggressive clinical behavior. There was no prior documented involvement of the gastrointestinal system. Based on these findings, the patient was diagnosed with an aggressive mantle cell lymphoma, stage IVA. The treatment plan for the lymphoma included 6 cycles of R-CHOP  chemotherapy with pegfilgrastim support for induction, which was to be followed by high-dose consolidation and stem cell collection  in anticipation of potential future autologous bone marrow transplantation.Several days before the patient was to receive the second cycle of R-CHOP; however, he presented with acute appendicitis and was referred urgently to general surgery. He was taken to the operating room on the same day for laparoscopic appendectomy, which was converted to open appendectomy with washout when it was directly observed that the appendix had perforated and had dense adhesions. Two small abscess cavities were identified, each of which drained purulent material on opening. His recovery from surgery was generally unremarkable other than mildly prolonged postoperative ileus and the presence of two small persistent abscesses by imaging postoperatively. He was discharged 9 days after his surgery on antibiotics and was continued on rituximab alone until he was able to return to his second cycle of CHOP roughly 1 month after his presentation with acute appendicitis.cyclin D1 gene product   translocation. [Mantle cell lymphoma is an intermediate-grade B-cell neoplasm composed of monomorphic small-to medium-sized lymphocytes with irregular nuclear contours and is characterized by overexpression of ocation. , 17 Mostocation.  Additionocation. . In many Lymphoma involving an appendectomy specimen has been previously described, albeit rarely –15. We fWe are uncertain of the role that chemotherapy played in this patient's presentation. While prior reports have described appendicitis in leukemic and other patients with malignant neoplasms undergoing chemotherapy, these cases are usually in association with neutropenia. This case is unusual, in that while the patient was undergoing therapy he was also receiving growth factor therapy (pegfilgrastim) and was not neutropenic. At the time of the surgery the degree of appendiceal involvement by lymphoma was not extensive; however, the possibility that the tissue had been more extensively involved prior to chemotherapy and that some component of cytoreduction contributed to the perforation of the appendix cannot be excluded. The majority of the gross and histologic features were otherwise typical of a ruptured suppurative appendicitis, however. It is also noted that acute appendicitis has been previously associated with benign lymphoid hyperplasia that may occlude the lumen of the appendix and, therefore, contribute to the development of the infection . HoweverIn summary, we describe an unusual case of partially treated mantle cell lymphoma presenting as an acute appendicitis. In addition to supporting the practice of routine histologic evaluation of all appendectomy specimens, this case emphasizes the potential clinical complications of chemotherapeutically treating a patient whose gastrointestinal system is involved by lymphoma. Given the frequency of involvement of the gastrointestinal tract in mantle cell lymphoma patients, this case emphasizes the importance of incorporating Cyclin D1 immunostaining into the evaluation of even morphologically unremarkable aggregates of lymphocytes in tissue samples from these patients. Furthermore, we suggest that immunohistochemical evaluation (including cyclin D1) may be indicated in all adult patients with appendicitis whose lymphoid tissue appears unusually prominent or exhibits atypical histologic features.


Pancreatic arteriovenous malformation (PAVM) is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented. Pancreatic arteriovenous malformation (PAVM) is a rare condition with various clinical manifestations. In most cases, the condition is congenital and the diagnosis is accidental since most of the patients are asymptomatic and the tumorous mass is revealed in an imaging investigation performed for a different reason. The widespread use of imaging techniques such as color Doppler sonography, contrast-enhanced computed tomography, magnetic resonance imaging, and angiography is the exact reason why an increase in the number of pancreatic AVM cases has been reported in recent years. Nevertheless, some present with symptoms varying from abdominal pain, gastrointestinal bleeding, jaundice, and more. We report a case of a symptomatic pancreatic arteriovenous malformation in a 64-year-old woman, which was successfully treated by transarterial embolisation.Helicobacter pylori. The patient received treatment for H. pylori eradication, improving her overall condition but failing to completely control the epigastric pain. Abdominal sonography was performed—for possible cholelithiasis—revealing a mass in the body-tail of the pancreas and thus the patient was referred to our institution for further investigation and management. She was diagnosed 6 years ago with multiple sclerosis complicated by cardiomyopathy for which she was under treatment. She had no history of trauma, pancreatitis, or other congenital abnormalities, and the only surgery she had undergone was right inguinal hernia repair. On physical examination, intense blepharoptosis was noted with generalized muscle weakness. Mild epigastric tenderness but with no definite mass was as well noted. Laboratory data were grossly normal apart from slightly elevated liver aminotransferases and γ-glutamyl transpeptidase, which were compatible with the sonographic finding of fatty liver infiltration. Serum amylase levels were within normal limits, as were serum Ca19-9 and CEA levels. The patient was tested negative for hepatitis B and C virus. She underwent abdominal CT and MRI scans, which in conjunction with the angiography that followed and revealed vascular distribution from the splenic artery, led to the diagnosis of pancreatic arteriovenous malformation (PAVM)  with specific image-processing techniques including Maximum Intensity Projection (MIP), Multi-Planar Reformation (MPR), and Volume Rendering (VR), which enable effective use of the large amount of the data obtained by MDCT . MDCT whManagement of PAVM consists of surgical therapy and conservative therapy . Last but not least, a watch and wait policy with “no treatment at all” has been described in the literature. Nishiyama et al. suggested that complete cure is accomplished only by total extirpation of the affected organ or at least its involved part . Koito eWritten consent of the patient was obtained before the information of her case was collected and she declared that it could be published or announced. All possible measures were taken that her identity is not revealed.


Placental mesenchymal dysplasia (PMD) rarely complicates with pregnancy. A 30-year-old woman, gravida 3, para 3, presenting with placentomegaly, was referred to our department at 18 weeks of gestation. An ultrasonography revealed a normal fetus with a large multicystic placenta, measuring 125 × 42 × 80 mm. The border between the lesion and normal region was not clear. Color doppler revealed little blood flow in the lesion. Magnetic resonance imaging revealed normal fetus and a large multicystic placenta. Serum human chorionic gonadotropin level was 20124.97 U/L, which was normal at 20 weeks of gestation. Thus, placental mesenchymal dysplasia rather than hydatidiform mole with coexistent fetus was suspected. Then, routine checkup was continued. Because she had the history of Cesarean section, an elective Cesarean section was performed at 37 weeks of gestation, and 2520 g female infant with apgar score 8/9 was delivered. The baby was normal with no evidence of Beckwith-Wiedemann syndrome. Placenta of 20 × 16 × 2 cm, weighing 720 g, was bulky with grape like vesicles involving whole placenta. Microscopic examination revealed dilated villi and vessels with thick wall which was lacking trophoblast proliferation. Large hydropic stem villi with myxomatous struma and cistern formation were seen. PMD was histopathologically confirmed. Placental mesenchymal dysplasia (PMD) is a rare benign entity characterized by placentomegaly and grape like vesicles resembling molar pregnancy on ultrasonography. We diagnosed a case of PMD at the second trimester and obtained healthy baby. Herein, we report the case and review the literature.A 30-year old woman, gravida 3, para 3, with a previous Caserean delivery presenting with placentomegaly was referred to our department at 18 weeks of gestation. Her medical history was significant for myomectomy at the age of 18. An ultrasonography revealed a normal appearing fetus and a large multicystic placenta measuring 125 × 42 × 80 mm. The border between the lesion and normal region was not clear . A chorioangioma was suspected first, but color Doppler imaging revealed little blood flow. Then the magnetic resonance imaging was performed at 20 weeks of gestation. A normal fetus and a large multicystic placenta were seen but normal part of the placenta was not clearly identified .Omphalocele was not identified. Serum human chorionic gonadotropin level was 20124.97 U/L, which is normal at 20th weeks of gestation, and we concluded that PMD was more probable rather than hydatidiform mole with coexistent fetus. The karyotype was not examined. Then routine checkup was continued. Because she had the history of Cesarean section, Cesarean section was performed at 37 weeks of gestation. Under spinal anesthesia, a 2520 g female infant with Apgar score of 8 and 9 at 1 and 5 min, respectively, was delivered. The infant had no evidence of Beckwith-Wiedemann syndrome. Placenta of 20 × 16 × 2 cm, weighing 720 g was bulky with grape like vesicles involving whole placenta with no border .The cord was 56 cm in length and 1 cm in diameter and appeared normal. Microscopic examination revealed enlarged villi and vessels with thick wall which was lacking trophoblast proliferation. Large hydropic stem villi with myxomatous struma forming cistern were observed . The diagnosis of PMD was confirmed. Her postoperative course was uneventful. PMD is a rare benign entity characterized by placentomegaly and grape like vesicles resembling molar pregnancy on ultrasonography . PMD wasApproximately 23% of the cases of PMD are associated with Beckwith-Wiedermann syndrome, which is characterized by macrosomia, exomphalos, macroglossia, omphalocele, internal visceromegaly, placentomegaly, and increased childhood tumors. Qichang et al. reported a case of PMD concomitant with chorioangioma . Nayeri β-hCG slightly increases. On ultrasonography, the placenta of complete molar pregnancy with cotwin and partial molar pregnancy look heterogeneous, with partially solid and cystic areas [Placentomegaly is usually presented and chorionic vessels were aneurysmatic and dilated. Many pathologists are unfamiliar with PMD, which may lead to underdiagnosis of this entity. Differential diagnosis from gestational trophoblastic disease is important because management and outcomes are different. Histopathologically, trophoblast proliferation is revealed in molar pregnancy but not in PMD. Partial molar pregnancy is accompanied by an abnormal triploid fetus and was not strongly suspected in this case because fetal development was normal. In case of partial molar pregnancy, pregnancy termination is indicated. A triploid fetus usually has several morphological anomalies and tends to die in the first trimester. When there is the combination of a molar appearance to the placenta and a sonographically normal fetus, suspicion for PMD may be particularly increased. In PMD, blood test reveals elevated AFP while ic areas , 8. Coheic areas  reportedic areas  reportedPMD is rare and resembles molar pregnancy, but the prognosis is different. The fetuses are normal in the majority of cases. This clinical entity should be kept in mind to avoid unnecessary termination of pregnancy.


Glomus tumors are rare neoplasms that usually occur on the hands in a subungual location, or sometimes in palms, wrists or soles of the feet. They are described as purple/pink tiny painful lesions with a triad of pain, local point tenderness, and cold hypersensitivity. They are almost always benign, but rare malignant variants have been reported. They have also been reported to be present at unusual locations, like the lung, stomach, or liver. Gastrointestinal glomus tumors are extremely rare tumors and very few cases have been reported in the literature. Most that have been reported were usually benign in nature. A rare esophageal glomangioma, mimicking a papilloma, was reported in 2006. We report a case of glomangiosarcoma  in a 49-year-old female, who presented with symptoms of dysphagia including some spasm and hoarseness and subjective unintentional weight loss. On endoscopic exam, she was found to have a distal esophageal mass with malignant features. Radiologically, the mass had a size of about 8 cm on the CT scan without evidence of metastases. Pathology and immunostaining of the biopsy showed features resembling a malignant glomus tumor. She underwent an endoscopic and laparoscopic staging of the tumor along with ultrasound. Based on the laparoscopic findings, which were consistent with the preoperative diagnosis, she was scheduled for an esophagectomy. Histopathology and immunophenotypic features of the excised mass were consistent with a diagnosis of malignant glomus tumor. Glomus tumors are rare, mostly benign, vascular hamartomatous derivatives of glomus bodies, which are present at arteriovenous anastomoses (without a capillary bed in between), normally located in the dermis. The glomus bodies play a role in thermoregulation of the skin. Glomus tumors usually arise in subungual areas, palms, wrist, and soles of feet as these areas are rich in glomus bodies. They usually present clinically as a triad of severe subungual pain, tenderness localized over a point, and cold hypersensitivity , 2. GlomOur patient, a 49-year old female, presented with a history of dysphagia for seven months with subjective unintentional weight loss. Endoscopy with esophageal ultrasound revealed a mass in the distal third of the esophagus with malignant features including ulceration, thick size, and granulation tissue but no mediastinal, celiac, or periportal lymphadenopathy. She also underwent an EUS-guided cold-forceps biopsy of the mass. The initial impression was that of a gastrointestinal stromal tumor (GIST). However, an outside pathology report of the biopsied mass showed that the tumor comprised of smooth muscle cells and was C-Kit (CD117) negative. The final diagnosis of the mass was inconclusive about the nature of the tumor, although a smooth muscle component in the mass was noted. Her original diagnostic material from an outside institution was reviewed. The neoplastic cells were epithelioid with focal spindle cells and necrosis and showed Bcl-2, and CD138 positivity, positivity for Vimentin and Actin, and negative for AE1/3, CAM5.2, CK20, CD56, Desmin, S 100, MPO, CD20, CD21, CD99, CD30, DOG1, and PLAP by immunostains see . ImmunosCT scan of the chest revealed a large heterogenous mass located in the distal third of the esophagus, arising from the posterior wall, measuring 7.6 × 4.9 × 4.2 cm. There were multiple areas of decreased attenuation within the mass. No pulmonary nodules were identified. CT scan of the abdomen and pelvis revealed multiple liver lesions which were hyper-vascular, largest measuring 4 cm, in the left lobe, showing a benign central scar, suggestive of focal nodular hyperplasia, which is a common benign tumor of the liver. No distal para-esophageal or gastrohepatic lymphadenopathy was noticed.She was then scheduled for a laparoscopic staging with possible biopsy of the mass and US of the liver with biopsy of the nodular lesion the following week. An esophagectomy with regional lymph node dissection was recommended. During the laparoscopic procedure, she underwent an intraoperative biopsy of the esophageal mass and ultrasound (US) of the liver with biopsy of the nodular lesion. During her exploratory laparoscopy, she had no evidence of peritoneal, omental, or hepatic metastasis. EGD showed a nonobstructing esophageal tumor, 35–40 cm from the incisors. Lymph node biopsy of the left gastric node and perigastric node showed no features of malignancy on frozen section. Pathology of the esophageal tumor showed reactive squamous epithelium with no neoplasia at 36 cm of the esophagus. Liver biopsy also turned out to be benign.Surgical excision of the tumor was planned. On a preoperative chest X-ray, the tumor appeared as a 6 × 3 cm oblong mass with smooth margins in the lower left middle-posterior mediastinum. She underwent minimally invasive esophagectomy with partial gastrectomy with lymph node dissection. An esophagogastrectomy specimen containing a 5.0 × 4.5 × 3 cm mass lesion in the distal esophagus, 0.4 cm proximal to the gastroesophageal junction and involving 60% of the circumference of the lumen . The cutAfter-surgery, the patient recovered very well and had no complications. Moreover, the tumor appeared to be somewhat of low grade given that there was no perineural and vascular invasion. Surgical excision was carried out with clear margins. Adjuvant radiotherapy or chemotherapy was considered after the surgery, but the risks and complications of radiotherapy like stricture and aspiration, nausea, vomiting, and cramping outweighed the benefits and there was no evidence to prove that adjuvant chemotherapy was linked to improvement in survival. The patient was to be followed up with scans every three months. On her follow-up visit at six months after surgery, her appetite had improved, dysphagia subsided, and she has also gained weight. The patient is doing remarkably well. There is no evidence of recurrence or metastatic disease on her latest CT scan.This variety of tumor was a surprising diagnosis in this female, who was being followed up for history of dysphagia and subjective weight loss, found to have a distal esophageal mass eroding into the lumen with possible granulation tissue on an EGD.Glomus bodies are mostly found in the dermis or subcutis of fingers or palms, or toes and soles of the feet. These play a role in regulating blood flow and temperature. Benign glomus tumors resemble the normal glomus body, as vascular, hamartomatous derivatives of it. Glomus tumors are usually found in the extremities, which is also the most common location of Glomus body. They account for about 1% of hand tumors in adults, usually in females. Glomus tumors are almost always tumors of adults and apparently have no association with other diseases. They may, as a rare exception, present as glomangiosarcomas, which is a malignant variant. It is supposed to arise de novo, as a low-grade malignancy, and presents with same features but is characterized by local recurrence after resection with or without metastasis.Extradigital sites are seldom seen with glomus tumors, and a malignant glomus tumor of the esophagus, moreover, is an exceptional occurrence. There have been some small case series describing glomus tumors of the antrum of the stomach. Malignant glomus tumor of the esophagus is a rare entity and our current case represents a unique case due to the lack of lymph node metastases. This 49-year-old female, who presented with 6–8 months of dysphagia, upon endoscopic findings, was diagnosed with a tumor invading and eroding into the lumen with possible granulation tissue around it. The differential diagnosis was that of a gastrointestinal stromal tumor , a smooth muscle tumor (leiomyoma), sarcoma, neuroendocrine tumor, but the possibility of each one of these was ruled out by histologic features, immunohistochemical staining, and FISH studies. Based on the histologic features of plasmacytoid and spindle cells with occasional blood filled spaces and immunophenotype by actin and collagen Type 4, a diagnosis of malignant glomus tumor was made.The management of this localized mass without metastasis was surgical resection with follow-up with scans. No adjuvant chemotherapy/radiotherapy was given in this case. From a clinical and radiological standpoint, a differential diagnosis of a rare glomus tumor must be considered if the neoplasm is solitary, vascular, and localized , without lymph node or distant metastasis. The cornerstone of diagnosis is pathological features and immunostaining. The prognosis is not bad if the disease is localized. However, a close watch with regular scans should be kept for the possibility of recurrence, which is a feature of malignant glomus tumors. Postsurgical resection, adjuvant radiotherapy, or chemotherapy has a limited proven documented role, as the tumor is one of the rare kinds of gastrointestinal malignancies. Risks and complications of radiotherapy are more concerning and disabling than any proven benefits.


PEComas are a collection of generally rare tumors, defined by the World Health Organization as ‘mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epitheloid cells’. We describe the case of retroperitoneal PEComa with a liposarcoma-like appearance on cross-sectional imaging, but distinctive immunohistochemistry revealing the correct diagnosis. Perivascular Epitheloid Cell tumors (PEComas) are rare mesenchymal tumors that can appear at various sites in the body and can be associated with the tuberous sclerosis complex. They can possess a heterogeneous appearance, ranging from poorly differentiated soft-tissue tumors to sclerosing masses. Lipid-rich PEComas are uncommon. We present the case of a large lipid-rich PEComa primarily misdiagnosed as a liposarcoma on computed tomography and magnetic resonance imaging. Histology, however, revealed a lipid-rich PEComa without any signs of malignancy, which required no further treatment following surgical resection.A 75-year-old man was referred for abdominal computed tomography (CT) due to a poorly defined liver mass, detected by the primary care physician on ultrasound screening, which turned out to be a hemangioma. As an accidental finding, the contrast-enhanced CT scan additionally showed an 8 × 11 × 15 cm large retroperitoneal mass in the lower left abdomen adjacent to the sigmoid colon and the left psoas muscle  for MDM2 gene amplification was negative, thus a liposarcoma was ruled out and the final diagnosis was lipid-rich perivascular epitheloid cell tumor (PEComa) without any signs of malignancy. The patient recovered well from surgery and did not require any further adjuvant treatment.Macroscopic evaluation of the surgical specimen confirmed the predominance of fat within the thinly encapsulated tumor Figure A. HistolPEComas are a rare mesenchymal tumor entity incorporating angiomyolipomas, clear ‘sugar’ cell tumors, lymphangioleiomyomas and histologically and immunophenotypically similar tumors occurring at various soft-tissue and visceral sites, and can be associated with the tuberous sclerosis complex ,2. DistiPEComas have been described at various extrarenal locations including the mesentery, urinary bladder, liver, pancreas, rectum, heart and lung as well as in the bone -11. RetrThe imaging signature of PEComas is highly variable, although a few common features have been described. Mostly, PEComas have well defined borders and are of regular shape. The enhancement on cross-sectional imaging after contrast agent administration is usually inhomogeneous due to the heterogeneous composition of the tumor .et al. have recently established a suggestion for risk stratification and treatment strategies [The degree of malignancy is highly variable in PEComas, but as with other sarcomas, the size of the primary tumor as well as the mitotic rate seem to be the most reliable prognostic factors associated with recurrence after surgical resection. Bleeker rategies . If possrategies .With a size > 5 cm but absent further risk factors such as infiltrative growth pattern, high nuclear grade and cellularity, mitotic rate > 1/50 HPF, necrosis or vascular invasion, the tumor in our patient was stratified as ‘uncertain malignant potential’ according to the modified Folpe criteria . AccordiAlthough a rare tumor entity, PEComas are occasionally found as usually well-circumscribed tumors, often located in the abdomen, retroperitoneum or the pelvis. Besides surgical resection as the treatment option of choice, clinicians should be aware of new treatment strategies using inhibitors of the mTOR signaling pathway, which is commonly activated in PEComas.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: Computed tomography; HPF: High-power field; MRI: Magnetic resonance imaging; mTOR: Mamallian target of rapamycin; PEComa: Perivascular epitheloid cell tumor.The authors declare that they have no competing interests.All authors we involved in the clinical care of the patient described in this case and contributed similarly to preparation and review of the manuscript. All authors read and approved the final manuscript.


We present the unusual case of a 74 year-old female with a history of breast cancer who presented with acute painless orbital swelling and vertical diplopia. MRI revealed a focal enhancing mass within the superior rectus muscle. As the concern for metastatic disease was high, surgical biopsy was performed and revealed an unusual mimicker of metastatic disease, the parasitic infection dirofilariasis. Orbital metastases can occur in approximately 8 to 10% of patients with metastatic breast cancer [Dirofilariasis is a parasitic disease that rarely affects humans. The life cycle of this parasite normally involves dogs as a primary host and mosquitoes as potential vectors. While intraocular infections have been reported with dirofilariasis, infections involving the orbit and, in particular, the extraocular muscles are exceedingly uncommon –6. A 74-year-old woman was initially aware of intermittent vertical diplopia which then became constant over the next 24 hours. This was followed by painless left orbital swelling with mild erythema and swelling of the left upper lid. The globe itself was normal. Visual acuity was 20/20 right eye and 20/25 left eye. There was mild erythema and swelling of the left upper eyelid. Eyelid fissures measured 10 mm on the right and 6 mm on the left. The left globe was 1 mm proptotic. Ductions of the left eye were mildly deficient in upgaze. Initial CT and MRI imaging, along with the clinical history were suggestive of a varix. She returned 1 month later with improvement of the lid swelling and diplopia however repeat MRI was unchanged raising concern for a metastasis. At surgery, a firm yellow tan nodule was seen within the superior rectus/levator muscles. Approximately 70% of the mass was removed since it seemed to involve most of the muscle. Intraoperative frozen section evaluation revealed intense necrotizing granulomatous inflammation. Dirofilaria sp.  and transmitted to humans through inoculation. The parasite has been reported to survive in human tissues for years without causing symptoms. In most human cases, the infection is discovered due to the associated immune response causing a granulomatous reaction and mass [Dirofilarial infections are dead-end parasitic infections in humans, normally occurring without risk of dissemination. Canines are a common primary host of and mass –7.Dirofilaria infection in humans can occur in various tissues. Cases of orbital , 6, 8, 9Our case demonstrates how the MRI appearance of ocular dirofilariasis can mimic the appearance of metastatic disease, especially in patients with a history of systemic cancer. As the MRI imaging features are nonspecific, the tumor-like appearance of the affected tissues typically leads to surgical resection , 7, 10.


Linear epidermal nevus is an uncommon diagnosis of benign lesions of the oral cavity. It is characterized by a congenital malformation arising from the ectoderm cells, which are arranged according to a typical linear configuration known as Blaschko's lines. We report a case of linear epidermal nevus of oral cavity in a 51-year-old lady or woman. The linear epidermal nevus of the oral cavity, although rare, can be considered a differential diagnosis of oral papillomatosis (OP). The histopathological studies and detailed description are the center of the diagnostic and clinical evolution. Linearepidermalnevus(LEN) is hamartomas from the embrionary ectoderm and is classified according to its major tissue composition  . InflammOral papillomatosis (OP) are hyperplastic verrucous lesions which differential diagnosis includes squamous lesions, vulgar verruca, condyloma, and even congenital lesions as LEN. The diagnosis is mainly according to a histological study –9. OP onA 51-year-old female patient was attended at the Otorhinolaryngology Clinic of the University of Campinas Teaching Hospital  on November, 2009, for an oral cavity verrucous lesions evaluation . PatientAt the age of 16, patient had a left parotid gland myxoid tumor, being submitted to a partial parotidectomy surgery. After two years, the tumor returned, and she had another surgery. During the new operation, the left facial nerve was injured, resulting in an ipsilateral peripheral facial paralysis. After the procedure, patient received adjuvant radiotherapy.There are no data regarding the palate lesion pattern before or after these treatments. However, the patient and her family did not reported any lesion change during the subsequently years.Candida spp, HPV). The final diagnosis was oral linearepidermalnevus.After 11 years since the first procedure, the patient attended at the Otorhinolaryngology Clinic for a palate lesion evaluation. An incisional biopsy was made and the histopathological result reported presence of both gingival e palate tissues with stratified and cornified epithelium, severe hyperplasia, and mild chronic inflammatory infiltration Figures  and 3. TThe follow-up decision was to do lesions evaluations every six months. During the first period, the lesions remained the same. Patient is currently under the Otorhinolaryngology Clinic care.Solomon et al. described in 1968 epidermal nevus lesions combined with noncutaneous organs injuries, which he called epidermal nevus syndrome (ENS) , 11. TheOral LEN is a rare disease with only a few cases described in literature . The diaLEN wounds usually affect the vertebral column in a radial pattern injury and the limbs in a longitudinal way. Happle described linear typical manifestations including mucous exclusively involvement of the head and neck regions , 4.Differential diagnosis for LEN is oral papillomatosis . The OP Cutaneous LEN treatment is performed due to an unwanted smell or aesthetic reasons. Bad smell plates can be managed with benzoyl peroxide solutions . For minIn this particular case, lesions characteristics were multiple papules restricted to a hyperplastic mucosa, with facial midline preservation, unilateral, congenital, and without evidence of aggressive behavior. The association among this information and the anatomopathological analysis was pivotal for the final LEN diagnosis.Despite LEN rarity, it should always be considered as a differential diagnosis for oral cavity pathologies. The anatomopathological lesions analysis represents a crucial stage in the diagnostic process. Clinical evaluation should always be reported to the pathologist for a better diagnosis.


Soft tissue sarcomas represent less than 1% of all human neoplasms. One-third of malignant tumors that arise in the retroperitoneum are sarcomas and liposarcoma is the most common retroperitoneal sarcoma. More often than not, patients report late to the hospital due to the slow progress and few late symptoms. Thus, the tumor is known to grow to enormous sizes. Here, we report a case of giant retroperitoneal liposarcoma weighing more than 7 kgs. Liposarcomas  generallA 65-year old lady presented with complaints of painless distension of abdomen for the last 2 years, which was insidious in onset and gradually progressive in nature. There was history of weight loss for the past 6 months. There was no history of hemoptysis, malena, and bleeding PR. Past History was not suggestive of any chronic illness. On examination she was: afebrile; pulse: 88/min and regular; blood pressure—136/80 mm Hg.Her abdomen was distended; flanks were full, soft, and nontender. A palpable retroperitoneal lump of size approximately 26 × 18 cm occupying left hypochondrium, left lumbar, and left iliac fossa, involving umbilical region and crossing midline was felt with no evidence of ascitis. Rest of systemic examination was within normal limits and a provisional diagnosis of a retroperitoneal lump was made.Lab investigations were as follows: hemoglobin: 11.4 gm%; total leucocyte count: 4,600/cu mm of blood; polymorphs: 60%, lymphocytes: 30%; ESR: 18 mm/hour; random blood sugar: 88 mg/dl; liver and renal profile: WNL.Chest X-Ray PA view was WNL and USG-guided fine needle aspiration cytology revealed it to be a lipoma.Ultrasonography of abdomen was suggestive of “a large echogenic lesion having anechoic areas within it suggestive of necrosis, occupying the entire abdominal cavity, causing displacement of the adjacent bowel loops.” All other vital organs appeared normal and there was no evidence of ascites.Contrast Enhanced Computed Tomography of abdomen revealed “a large heterogeneous lesion having multiple linear septation and HU value ranging from −97 to −105 HU suggestive of fat containing lesion causing compression and displacement of adjacent bowel loops, left kidney, and aorta towards the right side. This most likely suggests a malignant tumor most likely to be liposarcoma.”Mass was approached by a midline incision and descending colon was seen to be pushed across the midline entire left side of abdominal cavity was occupied by the mass. Extensions of the mass were seen in the entire abdominal cavity. Careful and complete resection of the mass was done and no organ resection was required. The resected out specimen weighed 7 kilograms.Histopathological examination was indicative of a “well-differentiated liposarcoma.” Postoperative course was largely uneventful and the patient was discharged on the 5th postoperative day and is on regular followup since 2 years with no recurrence of tumor till date. Radiotherapy was not given considering the age of the patient and tumor extent.Liposarcoma is the most frequent histological type of retroperitoneal sarcoma, corresponding to 41% of these tumors . LiposarThis case report is amongst the heaviest reported in the Indian subcontinent.


Desmoplastic small round cell tumors (DSRCTs) are extremely rare and mainly affect adolescents and young adults. The tumors are usually involved with the abdominal area and/or the pelvic peritoneum. Only a small number of cases have been reported concerning DSRCTs of the testicular region. The present study reports a case of DSRCT of the testis with radical orchectomy and systemic chemotherapy, leaving the patient disease-free for 14 months. However, the patient died of multiple metastasis 12 months later. Furthermore there is a review of the English literature to analyze the incidence, site of origin, imaging and pathological characteristics of DSRCT. A DSRCT is an aggressively malignant tumor that predominantly occurs in adolescents and young adults. The condition was first described by Gerald and Rosai in 1989 . The preA 27-year-old male presented with gradual swelling and intermittent testicular pain that had lasted for approximately four months. There was no specific infection or a history of trauma. The patient was initially diagnosed with epididymitis in a clinic and treated with antibiotics for two weeks. However, no significant improvement in the condition was observed. A physical examination revealed a solid mass located in the right scrotum, with no tenderness. Laboratory studies did not reveal any abnormalities. A mass measuring ∼5×6 cm  was iden2 vincristine on the first day, 60 mg/m2 doxorubicin on the second day and 2 g/m2 ifosfamide for five days. The patient appeared to be disease-free at 14 months. No evidence of recurrence was identified on the clinical or imaging examinations during the 14-month follow-up period. However, the patient succumbed to multi-organ metastases 12 months later.The patient was diagnosed with a malignant tumor and a radical orchectomy was performed. The post-operative course was smooth. A formalin-fixed, paraffin-embedded tissue section was obtained for a routine microscopic examination. The specimen was stained with hematoxylin and eosin. Microscopically, the tumors consisted of nests of ‘small cells’, with scant cytoplasm embedded in a densely fibrotic stroma and focal tubule formation. Numerous mitotic figures were observed within the tissues. Certain figures were arranged in well-defined cell nests, which were delimited by a cellular desmoplastic stroma  and 4. IDSRCT is a rare and aggressive, malignant tumor. The disease most commonly presents with a multinodular growth on the serosal surfaces, including the peritoneum  and the Morphologically, DSRCT is characterized by nests of mitotically active, small, round, blue cells that are proliferating in a cellular fibrous stroma. Immunoreactivity indicates a blastomatous cell of origin with a polyphenotypic appearance. The immunohistochemical characteristics of DSRCT exhibit the typical immunophenotype, consisting of positivity for keratin, vimentin, desmin and NSE. The differential diagnosis of DSRCT is fairly broad and includes tumors such as Ewing sarcoma, neuroblastoma, Wilms tumor, rhabdomyosarcoma, small cell carcinoma and lymphoma. A definite diagnosis may only be achieved with a demonstrated multidirectional differentiation and coexpression of epithelial, mesenchymal and neural antigens in the same cell . The preClinically, laboratory test results are non-contributory. Several studies have described DSRCT with elevated serum CA 125 and lactic dehydrogenase levels in certain cases ,9. TheseA DSRCT is characterized by a growth on the serosal surface. Certain studies have described an extra-abdominal location as the primary site of the tumor . To the The most common CT feature that has been reported for DSRCT is the synchronous presence of multiple abdominal masses and scrotal nodules without a clear organ of origin . Large hDespite aggressive treatment, the survival rate of patients with DSRCT remains poor. The optimum treatment remains to be determined. According to a literature review and our experience, a surgical resection, followed by aggressive chemotherapy is recommended as a treatment for DSRCT. Aggressive multimodality therapies with immunotherapy or bone marrow ablation, and dose-intensive chemotherapy with autologous peripheral blood stem cell support, may provide potential benefits for patients with DSRCT and be promising new treatment approaches . The surIn conclusion, primary DSRCTs of the testis are rare. In the present case, there were no specific characteristics in the clinical symptoms or the imaging studies, which resulted in a difficulty in diagnosing DSRCT. To date, no standard treatment has been established and the prognosis of affected patients is poor. Further investigation is required to identify the optimum treatment approach.


Teratoma refers to a neoplasm that recapitulates all three germ layers. Teratomas may be histologically mature and oncologically benign. Teratomas may also be histologically immature while being oncologically benign, or they may harbor malignant components and have the potential to exhibit an aggressive biological behavior. Teratomas of the head and neck are extremely rare and usually present in the neonatal period. As a general rule, pediatric teratomas of the head and neck tend to be oncologically benign, whereas adult teratomas tend to be histologically and oncologically malignant. Most of these teratomas are found in the cervical region and nasopharynx. Calcification within the mass is often evident.A 27-year-old Caucasian man complaining of a nasal obstruction was admitted to our clinic in January 2006. A transnasal endoscopic examination revealed a mass arising from the nasal septum which was completely removed using an endoscopic approach. Histologically, it was determined to be a benign teratoma.Herein, we present a rare case, along with a review of the related literature, in order to emphasize that a benign teratoma of the nasal septum should not be ignored. Benign teratomas are solid and cystic tumors composed of a variety of both immature and mature tissues derived from all three germ layers. The epithelial component of a benign teratoma usually consists of mature squamous epithelium and immature intestinal or respiratory epithelium. Primitive neuroepithelium with rosettes, pseudorosettes or neurofibrillary matrix predominates in some tumors. Pigmented retinal epithelium can also be seen. The mesodermal component consists of fibroblasts and embryonic, immature spindle cells embedded in a myxoid matrix. Islands of cartilage, smooth muscle cells, and skeletal muscle cells exhibiting varying degrees of maturity may also be present.Teratomas of the head and neck are extremely rare and usually seen during the neonatal period. While pediatric teratomas of the head and neck tend to be benign tumors, adult teratomas tend to be histologically and oncologically malignant. Calcification within the mass is often evident. Sinonasal masses present with nasal obstruction symptoms. The presenting symptoms are those associated with a sinonasal mass. Radiologically and grossly, these tumors are heterogeneous and composed of solid and cystic components .The following should be considered in the differential diagnosis of sinonasal teratomas: immature teratomas, teratomas with malignant transformation, sinonasal yolk sac tumors, sinonasal teratocarcinosarcomas, dermoid cysts, hamartomas, and hairy polyps. While teratomas can be surgically resected, malignant teratomas also require adjuvant radiotherapy .A 27-year-old Caucasian man who had been suffering from a nasal obstruction for eight years was admitted to the Department of Otolaryngology and Head and Neck Surgery, the Ministry of Health Izmir Tepecik Training and Research Hospital in January 2008. His past medical and family history did not include any abnormal or notable features. A mass with smooth surface, arising from the left posterior side of the nasal septum and filling the whole nasopharyngeal cavity, was seen on his endoscopic nasal examination. Other systemic examinations were normal. Complete blood count (CBC), activated partial thromboplastin time (APTT), and partial thromboplastin time (PTT) were normal. A soft tissue mass with lobulated margins was seen on computed tomography (CT) scan. Calcification was present in the septa. The mass extended from the posterior nasal septum to the pharyngeal space on the right and the nasal turbine on the left Figure A and 1B.The nasopharyngeal mass, approximately 4cmx3cm in size, was completely resected under local anesthesia via an endoscopic approach within our clinic Figure . No compHistopathological examination with hematoxylin and eosin staining revealed a semi-cystic nasal teratoma consisting of mature epithelial and mesenchymal components Figure A and 3B.The patient was followed up by periodic examinations for eight months and no recurrence was seen during this postoperative period.Teratoma refers to a neoplasm that recapitulates all three germ layers. Teratomas of the head and neck are extremely rare and usually seen during the neonatal period. In general, lesions in the head and neck region account for approximately 5% of all benign and malignant germ cell neoplasms. Common sites of involvement include the neck, oropharynx, nasopharynx, orbit, and paranasal sinuses . Mature Teratomas may be histologically mature and oncologically benign. Teratomas may also be histologically immature while being oncologically benign, or they may harbor malignant components and have the potential to exhibit an aggressive biological behavior. As a general rule, while pediatric teratomas of the head and neck tend to be oncologically benign, adult teratomas tend to be histologically and oncologically malignant ,4.The following should be considered in the differential diagnosis of sinonasal teratomas: immature teratomas, teratomas with malignant transformation, sinonasal yolk sac tumors, sinonasal teratocarcinosarcomas, dermoid cysts, hamartomas, and hairy polyps. Immature teratomas and teratomas with malignant transformation are tumors of infancy and early childhood, whereas sinonasal yolk sac tumors and sinonasal teratocarcinosarcomas have only been documented in adults -6. ImmatSinonasal teratocarcinoma has a marked male predominance. These neoplasms almost exclusively arise in the ethmoid sinus and maxillary antrum. Patients usually present with a short history of nasal obstruction and epistaxis. Imaging studies reveal a nasal mass, occasionally accompanied by opacification of the paranasal sinuses. Bone destruction may be seen. Teratocarcinosarcomas are highly malignant. They are locally aggressive, rapidly invading soft tissue and bone as well as the orbit and cranial cavities. They also have the potential to metastasize to regional lymph nodes and distant sites, mainly the lungs. A dermoid cyst is a developmental lesion histogenetically and histologically composed of ectoderm and mesoderm, but not endoderm. Dermoid cysts of the nose account for 3% of all dermoid cysts and about 10% of those of the head and neck region. Dermoid cysts of the head and neck are located more often in the subcutaneous tissue of the lateral supraorbital ridge and nose. In the nose, they occur most commonly in the bridge and always in the midline. A few cases of dermoid cysts arising from the paranasal sinuses have been reported. The term hamartoma refers to an abnormal, oncologically benign proliferation of indigenous tissues. The sinonasal tract, particularly the area of the nasal septum, is the most common site for hamartomas of the head and neck region. At the histological level, benign disorganized epithelium, salivary glands, muscle, and cartilaginous and vascular tissues are seen -7.The ideal treatment for mature teratomas is total surgical excision. Sinonasal teratomas are associated with high mortality rates, despite the utilization of different modalities in the treatment.In our case, histopathological examination revealed epithelial and mesenchymal components, and the diagnosis of benign teratoma was established based on these findings. In the literature, benign teratomas arising from the nasal septum have only been described once in a young adult .The ideal treatment for mature teratomas is total surgical excision. Sinonasal teratomas are associated with high mortality rates, despite the utilization of different modalities in the treatment. We present a rare case, along with a review of the related literature, in order to emphasize that a benign teratoma of the nasal septum should not be ignored.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.IC performed the diagnosis of the patient and performed the operation. MG and AY collected data and performed statistical analysis. ÜB performed the histopathological examination. OGY had a major contribution in writing the manuscript. All authors read and approved the final manuscript.


Elastofibroma dorsi (ED) is an uncommon, slow-growing, benign, soft tissue tumor of unclear pathogenesis, typically located at the subscapular region of elderly people. It may be unilateral or bilateral. Though many patients are asymptomatic, ED can cause local deformity and symptoms such as periscapular pain or discomfort. Herein we report a case of a 65-year-old woman with painful ED. Clinical features, radiodiagnostic, intraoperative, and pathologic findings, and a brief review of the literature are performed. Elastofibroma dorsi (ED) is a benign, soft tissue tumor found in the subscapular and infrascapular region between the thoracic wall, serratus anterior, and latissimus dorsi muscle. It was first described in 1961 by Jarvi and Saxen . We hereA 65-year-old woman with no significant medical history arrived at the outpatient facilities referring a painful shoulder mass, which had been slowly growing during the last five years. The mass was slightly visible in the neutral position. However, upon physical examination a firm and easily palpable mass at the inferior region of the right shoulder was found. Laboratory findings were within normal limits. She underwent magnetic resonance imaging (MRI) demonstrating a poorly circumscribed lesion with dimensions 67 × 29 × 45 mm located between the right lateral-posterior thoracic wall, the lower corner of the scapula, and serratus anterior muscle. The mass consisted of elements with signal intensity equal to muscles in T1 and T2, situated linearly and forming bundles. Linear areas with a signal intensity equal to fat were also dispersed among them. The lesion was nonhomogenously enhanced after contrast administration. No signs of malignancy were observed Figures . No intrElastofibroma dorsi is a rare, benign, slow-growing soft tissue tumour with typical localization in the subscapular and infrascapular region, between the thoracic wall, serratus anterior, and latissimus dorsi muscle, often connected to the thoracic wall periosteum . UnusualAetiology is unclear. It is strongly supported that ED is more common in people with high physical activity involving the shoulder , 28. ThuElastofibroma dorsi affects primarily the elderly, over 55 years of age, with a mean age of about 60 years at diagnosis . HoweverWhen symptomatic, depending on the site and size of the lesion, ED may present as progressive swelling and discomfort or shoulder/back pain. Other symptoms include snapping of the scapula, stiffness of the shoulder, and restriction of shoulder movements. The physical examination may reveal a palpable, firm mass at the lower corner of the scapula, more prominent on abduction of the arm, usually immobile and probably due to its adherence to the surrounding tissue. Another mass may be present on the opposite shoulder, often smaller and clinically silent , 33, 34.Simple radiographs of the region cannot propose a diagnosis. The imaging modality of choice is MRI, which typically shows a well-defined and heterogeneous mass , 34. TheElderly patients or patients with bilateral tumours of typical localization and radiographic findings do not require biopsy, and the diagnosis of ED can be presumed. If the case is less typical an open biopsy or core needle biopsy must be performed. A fine needle biopsy is inadequate to get a representative tissue specimen. The differential diagnosis includes sarcoma, lipoma, fibroma, liposarcoma fibromuscular tumour, desmoid tumour, hemangioma, and hematoma, aggressive fibromatosis , 34. HisWhen the diagnosis of ED is definitive, its treatment depends on the symptoms it causes to the patient. Due to its benign nature, for the asymptomatic patient with ED no excision is needed, and clinical followup proves to be sufficient , 29. In Elastofibroma dorsi should be taken into consideration in the differential diagnosis of divers shoulder pathologies and seems to be more frequent than reported. It is strongly supported that MRI is imaging modality of choice to identify ED. Knowledge of this entity could avoid useless procedures specially in elderly and asymptomatic patients where a simple followup could be sufficient. In symptomatic patients, marginal resection is recommended.


Subpleural lipomas are very rare pleural lesions and are often discovered incidentally in asymptomatic patients on conventional radiographs.We report two cases of subpleural lipomas and describe their imaging characteristics on chest radiographs and computed tomography. We describe the cases of two Caucasian men, aged 77 and 62 years old.For non-invasive diagnostic investigation, computed tomography enables the identification and quantification of these tumors due to their characteristic fat attenuation. Lipomas in general are encapsulated, homogeneous, mesenchymal, slow growing tumors that arise in adipose tissue. Although they account for the most common benign soft tissue tumors in humans, intra-thoracic lipomas are considered rare. Subpleural lipomas are parenchymal, fat-containing lesions of the chest, where they arise from the submesothelial layers of the pleura parietalis and may extend into the subpleural, pleural or extrapleural space . Most paA 77-year-old Caucasian man was referred to our hospital with a three-month history of recurring chest pain, increasing in intensity over the last few days. He was a smoker and suffered from high blood pressure, however he has refused antihypertensive medication since diagnosis. In 2006, he underwent percutaneous, transluminal angioplasty of his left leg for a high-grade stenosis of the left femoral artery due to peripheral artery occlusive disease. No other significant events were found in his medical history. At a multidisciplinary team meeting, the decision to refer our patient for coronary catheter angiography was made because of his clinical findings and high pre-test probability for coronary artery disease. Before our patient underwent catheterization, upright posterior-anterior and lateral view chest radiographs were taken Figure  and 2 anA 62-year-old Caucasian man was referred to our hospital by his general practitioner for a routine, annual follow-up examination after he had undergone surgery for two plasmacytoma lesions in the epipharynx nine years ago. He had been in complete remission since then, however his general practitioner requested a chest radiograph because of a three-month history of intermittent coughing and mild chest pain. The chest radiograph revealed a well delineated, solid lesion of the left upper lobe Figure  and 6. FBefore the implementation of computed tomography in diagnostic medical imaging, most subpleural lipomas were detected during autopsies or incidentally during thoracic surgery. Today, if a pathological pleural or pulmonal lesion is detected on a chest radiograph, CT is a very elegant method to quantify and characterize these lesions. In cases of subpleural lipoma, which shows typical fat attenuation values of approximately -50 to -100HU (Hounsfield units), the diagnosis of a fat-containing tumor of the pleura can be made. However, making the differentiation between well differentiated, malignant liposarcomas and benign lipomas may be challenging on CT images. The typical characteristics of a malignant tumor include invasive growth, inhomogeneous enhancement after intravenous contrast medium application, poor delineation of the lesion and the occurrence of metastases. In the case of lipomas and liposarcomas, even for experienced histopathologists it is a challenge to differentiate between these two tumor entities since the only key finding might be the mitosis rate of the tumor cells . HoweverCT should be considered the imaging method of choice to elucidate the location and extent of subpleural lipomas.The authors declare that they have no competing interests.CAK had the initial idea for this work and was responsible for writing the manuscript. PS handled the patient data acquisition and the literature research. TF contributed to the editing of the manuscript. OFD was responsible for creating the figure files. SL contributed to the editing and writing of the manuscript. All authors read and approved the final version of the manuscript.Written informed consent was obtained from both patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Malignant myoepithelioma is a relatively rare malignant tumor occurring most frequently in the salivary glands. A few isolated cases have been described in other locations, including soft tissue, bone, lung, bronchus, oral cavity, nasopharynx, larynx, and maxillary sinus. Malignant myoepithelioma, however, is uncommonly involved within the cavernous sinus. To the best of our knowledge, this is the first report of malignant myoepithelioma arising from within the cavernous sinus.Herein, we report a case of a 48-year-old woman who presented a 1-month history of diplopia and blepharoptosis as well as radiological evidence of a rapidly developing cavernous sinus tumor. The patient underwent a trans-sphenoidal biopsy and a histological diagnosis indicated a malignant myoepithelioma. After diagnosis, the tumor grew rapidly and her clinical condition deteriorated progressively. Therefore, a pterional craniotomy with partial tumor removal was performed. The patient’s clinical state was worsened, and she died two months after the initial operation. Because the malignant myoepithelioma could not be traced to an organ of origin, other than the cavernous sinus, this case was diagnosed as a primary intracranial malignant myoepithelioma.The purpose of presenting this case report is to raise awareness among clinicians to consider malignant myoepithelioma as a differential diagnosis when a cavernous sinus mass is identified. Furthermore, an ideal management strategy for malignant myoepithelioma is not known and the prognosis seems to be unfavorable; therefore, more cases are needed to enhance our knowledge of the diagnosis, treatment, and prognosis of this rare intracranial lesion. Malignant myoepithelioma, also called myoepithelial carcinoma, is a malignant tumor that usually occurs in the salivary glands. It contains markedly proliferating myoepithelial cells, which are normally present in the salivary, mammary, and sweat glands ,2. Less A 48-year-old woman was admitted to our hospital with symptoms of diplopia and blepharoptosis for one month. Before being taken to our hospital, the patient had visited the neurology departments in other hospitals with the aforementioned symptoms and subsequent Magnetic Resonance Imaging (MRI) Figure A and endAt the time of admission, physical examination of the patient showed abnormal function in her left eye such as blepharoptosis, a 4 mm-diameter pupil with light reflex retardation, limited movement, and visual field loss of upside, downside, and temporal side, which were considered to be consequences of homo-side oculomotor and abducent nerves paralysis caused by mass pressure. Signs of reduction in pain and touch sensation on the left forehead skin were also observed in the physical examination. No abnormal function from other cranial nerves was observed. The pituitary hormones test showed nothing unusual. A general examination, including chest CT scan, abdominal ultrasound exam, bone X-ray, and nasal endoscopic check did not reveal any primary or metastatic lesions in other areas.The preoperative differential diagnosis included pituitary adenoma invading the cavernous sinus, granulomatous inflammation, metastatic brain tumor, and primary malignant tumor arising in the cavernous sinus. Therapeutic trials of corticosteroid administration are important diagnostic tests to differentiate granulomatous inflammation from other neoplastic lesions in the cavernous sinus . As aforThe patient was then recommended for postoperative radiotherapy and chemotherapy. Due to economic reasons, they refused the adjuvant treatment. After biopsy, her left pupil progressively enlarged to the state of mydriasis, while the diameter of her right pupil also gradually increased to 5 mm with a reduction in light reflex. Physical examination revealed typical symptoms resulting from impacted trigeminal and hypoglossal nerves, including reduced facial pain and touch sensation, diminished corneal reflex, dysphagia, cough, and hoarseness. The general condition of the patient progressively deteriorated. A repeated MRI showed more severe tumor development, which filled the left cavernous sinus and extended into the ipsilateral middle fossa Figure D. Given Malignant myoepithelioma is a rare malignant neoplasm in which the tumor cells show myoepithelial differentiation. They are most often found in salivary glands but also occur in other tissues and organs, including soft tissues where they were recently recognized as a distinct diagnostic entity . MalignaPreoperative diagnosis may be difficult or impossible for malignant myoepithelioma due to nonspecific clinical manifestation and imaging characteristics. Histological examination supplemented with IHC staining studies, is the most reliable and conclusive method of diagnosis. From a pathological point-of-view, malignant myoepithelioma commonly contains marked proliferating myoepithelial cells, and various morphologically, neoplastic myoepithelial cells are presented, including spindle, plasmacytoid, clear, epithelioid and stellate types ,17. The In this case, the tumor commonly consists of spindle cells with moderate to marked nuclear atypia. The tumor malignancy is supported by cellular pleomorphism, nuclear atypia, hemorrhage, necrotic areas, a mitotic activity and a high proliferative MIB-1 index in the neoplasm under the light microscope. There are some tumors that also show similar spindle cell composition, such as parachordoma, chordoma, extraskeletal myxoid chondrosarcoma, unusual carcinoma and even chondrosarcoma if a chondroid matrix is present and the sampling is small. Distinguishing malignant myoepithelioma from other tumors is as difficult radiographically as it is clinically. Therefore, to make the final diagnosis in the present case, immunohistochemical studies were necessary. The expression patterns of the epithelial and myogenic markers are somewhat varied in each case and may reflect the degree of myoepithelial cell differentiation. The combination of SMA, GFAP, S-100, and vimentin positive cells in these lesions aids in the diagnosis of malignant myoepithelioma.Intracranial primary malignant myoepithelioma is extremely rare and has never been noted within the cavernous sinus. In view of this rare site for malignant myoepithelioma, we only found two cases reporting intracranial primary malignant myoepithelioma utilizing the help of Medline and Pubmed. Although some differences exist between the three cases, we can still explore possible explanations for the occurrence of this salivary gland tumor. Carsten Nieder et al.  had repoThe origin of the malignant myoepithelioma in the present case remains obscure. At first, we tried to find some evidence of an extracranial source with salivary gland distribution to explain it as a metastatic tumor. However, with regard to the negative findings in the chest CT scan and nasal endoscopic check, we excluded possible primary sources, such as the bronchus, nasopharynx, etc. Therefore, the primary tumor source in this case is determined to be the cavernous sinus, while taking into consideration the occurrence of salivary gland heterotopia. Histologically, the sellar region is a neighboring site to the oral cavity, nasopharynx, and larynx, where there are salivary glands existing. And some known reports presented some sellar region salivary gland tumors that may be related to salivary gland rests in the sellar region or pituitary ,22. In aSince intracranial malignant myoepitheliomas are such a rare entity, effective management strategies have not been established. Treatment options include surgical resection, chemotherapy, radiotherapy, or a combination of these approaches. Surgical resection is the mainstay therapy for malignant myoepithelioma, but in some circumstances it may not be technically feasible due to the rich vascularity and involvement of the cavernous sinus and carotid arteries. Radiation therapy and chemotherapy can also be used for malignant myoepithelioma. However, there is no consensus whether postoperative adjuvant therapy is even required because the prognosis of this condition is unclear. Some authors reported radiotherapy or concurrent chemoradiotherapy is effective for local recurrence and distant metastasis ,24. But In conclusion, intracranial malignant myoepithelioma is an extremely rare or often misdiagnosed lesion. This is the first reported case of a malignant myoepithelioma arising within the cavernous sinus. Preoperative diagnosis may be difficult or impossible due to nonspecific clinical manifestation and imaging characteristics, thus diagnosis can only be made postoperatively based on histopathological and immunohistochemical analysis. The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a cavernous sinus mass is identified. Furthermore, ideal management strategy is not known. Surgical resection has been the accepted treatment for malignant myoepithelioma. The role of chemotherapy or radiotherapy, however, is controversial. The prognosis of malignant myoepithelioma seems to be unfavorable; therefore, more cases are needed to enhance our knowledge of the diagnosis, treatment, and prognosis of this rare intracranial lesion.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.MRI: Magnetic Resonance Imaging; IHC: Immunohistochemistry; SMA: Smooth muscle actin; GFAP: Glial fibrillary acidic protein.All authors declare no competing interest.Hong drafted the first manuscript and made a contribution to acquisition and interpretation of data. Chen, Guo and Zhang performed the clinical work-up and literature search. Klebe revised the language and grammar of the manuscript. Hong and Wu revised the manuscript that led to the final approval of the current submission. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/13/40/prepub