To report on a female patient diagnosed with Currarino’s triad in adulthood.This case presents an adult patient with a medical history of a congenital anal atresia, a partial sacral agenesis, and a surgically treated ectopic anus. After a coincidentally observed presacral mass by MRI, due to unexplained constipation later in adulthood, Currarino’s triad was suspected in this patient. This triad consists of anorectal malformation(s), sacrococcygeal defects and a presacral mass of various origin. Further investigation confirmed the mass to be a meningocele, and showed a tethered cord and a syrinx.In (young) patients with anorectal malformations, although having no other symptoms, further examination might be required to exclude Currarino’s triad. Importance of early diagnosis and multidisciplinary assessment is recommended to establish adequate treatment if needed. Constipation is a symptom commonly observed in neonates. Few patients suffering from constipation at young infancy, however, are diagnosed with Currarino’s triad, consisting of a malformation in the anorectal region, a  sacral agenesis, and the occurrence of a presacral mass. It is caused by a deletion on chromosome 7q36 . Since tIn this report, we present an adult female with a history of congenital anal atresia and partial sacral agenesis. At a later age she presented with an anterior meningocele, highly suggestive of Currarino’s triad. We discuss the diagnostic and optional treatment aspects of this case, thereby underlining the urgency for early diagnosis.A 22-year-old Caucasian female was seen in the neurosurgical outpatient clinic. The family history was irrelevant. Her medical history revealed a congenital anal atresia and an ectopic anus. At the age of 3 years, the latter was corrected with an anterior sagittal anorectoplasty (ASARP) procedure. Furthermore, she had suffered from grade III vesico-ureteral reflux. X-ray imaging of the lumbar spine confirmed a partial sacral agenesis, sickle-shaped sacrum Fig. . After sDuring the second part of 2010, she experienced transient defecation difficulties. For that reason, the pediatric surgical team performed an MRI without contrast of the pelvic region. This showed the formerly known changes as expected from the ASARP procedure and the partial sacral agenesis. Coincidentally an anterior presacral mass of unknown origin, suggestive of a meningocele, as well as a bicornuate uterus was diagnosed  symptoms, and the radiological findings. We recommend an early and multidisciplinary approach if a patient is suspected to have Currarino’s syndrome.


Objective. To investigate the clinicopathological characteristics of gastrointestinal stromal tumor (GIST) with significant cystic changes and to assess the molecular genetic characteristics. Methods. In a 68-year-old man, a large abdominal tumoral mass was discovered incidentally. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of a large cystic lesion with multiple contrast-enhancing septae and papillary projections. No clear connection with any of the surrounding organs was identified. Malignancy could not be excluded, and surgery was indicated. During surgery, the large mass was found to be attached by a narrow stalk to the large curvature of the stomach. Results. The histological features and immunohistiochemical profile of the tumor cells (positivity for CD117 and CD34) were consistent with a gastrointestinal stromal tumor with a high risk of progressive disease according to the Fletcher classification. Diagnosis was confirmed by mutational analysis; this demonstrated mutation in exon 14 of PDGFRA. During the followup of 97 months, the patient had a cancer-free survival. Conclusions. This case demonstrates that gastrointestinal stromal tumors (GISTs) with extensive cystic degeneration should be considered in the differential diagnosis of a cystic abdominal mass. Gastrointestinal stromal tumors (GISTs) are specific mesenchymal tumors of the gastrointestinal tract. GISTs are rare, accounting for only 0.2% of all gastrointestinal tumors . MorpholIn a 68-year-old man, a large abdominal tumoral mass was discovered incidentally during checkup for vascular insufficiency of the lower limbs. The patient complained of vascular insufficiency and had no symptoms of abdominal pain. Physical examination revealed a palpable mass in the right upper quadrant of the abdomen. Results of blood samples were all within normal values.Ultrasonography showed a well-defined large cystic mass with several membranous septa with a diameter of approximately 12 cm in the right hypochondrium . SubsequHowever, no clear connection with any of these organs was identified. The imaging findings demonstrated no vascularity of the lesion. Malignancy could not be excluded, and surgery was indicated.At laparotomy a large exophytic mobile mass was found to be attached by a narrow stalk to the larger curvature of the stomach at the level of the antrum. No infiltration into the surrounding tissues was observed.Surgical resection of the mass along with a wedge resection of the stomach, at the larger curvature, adjoining the stalk and the adherent omentum was performed.The resected tumor measured almost 12 cm in diameter. Grossly, it was a unilocular cystic tumor filled with serous fluid, with a smooth outer surface and several membranous septa projecting in the lumen . The cysGastrointestinal stromal tumors (GISTs) are rare neoplasms, with an annual incidence of approximately 4 per million . HistoriThe cellular origin of GIST recently has been proposed to be the interstitial cell of Cajal, an intestinal pacemaker cell. This postulate is supported by the finding that GISTs display positivity for cell markers similar to those of the normal cell of Cajal –6. The mComplete tumor resection with disease-free resection margins is the treatment of choice for primary nonmetastatic tumors. Lymphadenectomy is not recommended because lymph node involvement is rare. Wedge resection allows full-thickness resection of the stomach wall containing the tumor, with negative resection margins , 6.Grossly, GISTs vary greatly in size and can be more than 30 cm in diameter. These tumors are usually well circumscribed and unencapsulated. GIST can grow in an endophytic or exophytic pattern. They are usually solid. Small cysts are frequently observed, presumably as a consequence of cystic degeneration or necrosis. Larger stromal tumors usually degenerate, and cysts are formed –11.In the present case, the large size of the cyst obscured the origin from the stomach. Imaging showed that the tumor was not originating from the pancreas or any other organ, so the exact origin of the tumor could not be determined preoperatively. Imaging demonstrated no vascularity of the lesion. Since malignancy could not be excluded in our case and the origin of the tumor could not be determined by imaging, surgery was indicated.Complete tumor resection with disease-free resection margins is the treatment of choice for primary nonmetastatic tumors. Lymphadenectomy is not recommended because lymph node involvement is rare. Wedge resection allows full-thickness resection of the stomach wall containing the tumor, with negative resection margins , 6.Lesions that should be considered in the differential diagnosis of a cystic abdominal mass on radiologic imaging (CT and MRI) include gastric or bowel duplication cysts, cystic mesothelioma, cystic lymphangioma, cystic mucinous retroperitoneal tumors, cystic pancreatic tumors, pseudocysts of the pancreas or peritoneum, cystic teratoma, and GIST –14.In this case, tumor cells showed diffuse and strong positivity for CD117 (KIT) and CD34, which was consistent with a diagnosis of GIST. This was confirmed by molecular biology that showed a mutation in exon 14 of the PDGFRA gene .Wang et al. recently published a series of 7 patients with cystic GISTs and analysis of c-kit and PDGFRA gene. Gene mutation of exon 11 of c-kit was identified in 3 cases . PDGFRA In summary, GISTs with cystic appearance clearly should be considered in the differential diagnosis of cystic abdominal tumors. Most GISTs (95%) express Kit (CD117) and CD34 (70%). In case of doubt genmutation analysis is necessary. KIT and PDGFRA genotyping is important for GIST diagnosis and assessment of sensitivity to tyrosine kinase inhibitors.


Epithelioid hemangioendothelioma (EH) is a rare, vascular neoplasm that can affect any age group and has been reported previously in sites including bone, liver, lung, breast, and brain. We describe a case of EH located in the mastoid, which appears to be the first report of EH in this site. The patient was 62 years old when they presented with dizziness and nausea. A suboccipital surgical approach was utilized to resect the tumor. After 18-month followup, the patient was symptom-free; however, imaging demonstrated a recurrence and the patient was taken back to the operating room for a resection. There is no evidence of recurrence after 8 years of followup. This paper aims to reinforce the need for a timely radical excision and aggressive clinical followup as the best hope for cure. Here, we describe an illustrative case and review the pertinent literature. Weiss and Enzinger first defined a unique soft tissue vascular tumor as an epithelioid hemangioendothelioma (EH) in 1982 . These tA 62-year-old patient presented with a one-month history of progressive dizziness and nausea. The patient complained of persistent positional vertigo and increasing hearing loss in the left ear. The past medical history was nonsignificant. Neurological examination revealed mild auricular tenderness and decreased hearing, however; the rest of the exam was unremarkable.Bone windows on computed tomography (CT) demonstrated an osteolytic mass expanding into the epidural space . The masThe patient underwent tumor resection through a left retromastoid approach utilizing motor evoked potentials, cranial nerve monitoring, and neuronavigation. After careful dissection into the suboccipital triangle, a suboccipital craniectomy extending into the occipital condyle was performed along the dural margin. Special precautions were taken to avoid injuring the underlying dura. The tumor was moderately vascular, but soft, and was resected carefully off the dura and surrounding neural elements. Once we identified the sigmoid sinus, it was carefully skeletonized and we proceeded to the presigmoid region. The VII cranial nerve was identified and skeletonized. We skeletonized the jugular foramen and identified the IXth, Xth, and XIth nerves. The involved mastoid air cells were resected. The semicircular canals were visualized and were uninvolved with tumor. Multiple specimens were sent for frozen section as well as permanent. Once all visible tumor was resected and hemostasis was obtained, an abdominal fat graft was placed and the incision was closed primarily. The patient tolerated the procedure well without any complications.Both specimens showed similar histopathology. There were cords and sheets of epitheloid cells in the background of fibromyxoid stroma . The celPostoperatively, the patient's dizziness resolved with no change in his hearing difficulties or any other cranial nerve deficits. Immediate post-operative imaging demonstrated gross total resection Figures . The patEH is a rare vascular tumor that has been reclassified as a fully malignant tumor by the World Health Organization . They haThe most common reported location is intra-axial with evidence of mass effect causing headache, seizure, or acute neurological deterioration . The varThe pathomorphology of EH is very characteristic with cords of epithelioid cells in myxohyaline stroma forming primitive lumina that points towards the endothelial differentiation. The differential diagnosis includes metastatic carcinoma, melanoma, and epithelioid angiosarcoma. The cytoplasmic vacuoles can be confused with gland formation by adenocarcinoma or signet ring cell morphology. However, metastatic neoplasms usually present pronounced nuclear atypia and mitotic activity. Although EH can show some expression of epithelial markers, which is usually focal, adenocarcinoma would be diffusely positive for cytokeratin, as well as mucicarmine . MelanomAbout 25% of EH demonstrates “atypical features” of cellular atypia, >1 mitotic figure per 10 hpf, necrosis and spindle morphology; such tumors were historically defined as malignant EH  in a recThe risk of recurrence and metastasis makes treatment complex. Including the current report, there have been six cases of local recurrence, progression, and seeding of the neuraxis , 20, 21.The present case describes the clinical and pathologic features of a epithelioid hemangioendothelioma involving the mastoid in a 62-year-old male. To our knowledge, this is the first reported case of EH in this site. Complete surgical resection is the initial treatment of choice, but timely and aggressive clinical followup is necessary to monitor for recurrence.


Autogenous tooth transplantation (ATT) can be considered when there is a hopeless molar tooth and suitable donor present. This report presents an unconventional case of successful ATT of a third molar replacing the adjacent fractured second molar in a 33 year old woman. This wisdom tooth had completely developed roots. Root-end filling with Calcium Enriched Mixture (CEM) cement was performed in the third molar. The second molar was extracted non-traumatically without any bone removal; the wisdom tooth was immediately transplanted into the recipient socket. No endodontic treatment was carried out either during or after the ATT. At six-month and 2-year clinical examination the patient was asymptomatic; the transplanted tooth was still functional, with no evidence of marginal periodontal pathosis. At the same follow ups, radiographic evaluation illustrated bone regeneration, normal PDL, and absence of external root resorption. Transplantation of mature third molar seems to be a promising method for replacing a lost permanent molar tooth and restoring aesthetics and function.  Dental autotransplantation or autogenous tooth transplantation (ATT) was first reported in 1951 . The reci.e. untreatable large decay, severe periodontal disease, failed root canal treatment and root fractures/perforations  named calcium enriched mixture (CEM) cement has been developed as root-end filling material . Althougstandard -15, and d humans -18 revead humans . This ded humans  and doesd humans . AdditioThe aim of this case report is to demonstrate a successful replacement of an untreatable second mandibular molar with vertical root fractured with its adjacent completely-developed third mandibular molar.A 33-year-old Caucasian woman was referred to an endodontic practice for extraction of her mandibular left second molar. The medical history was non-contributory. Clinical examination revealed good oral hygiene but the second molar had vertical fracture. Periodontal probing depths did not exceed 3 mm, however, the involved tooth was tender to percussion and palpation. The radiographic examination showed previous endodontic treatment with poor instrumentation and unsatisfactory obturation. A large extended periapical lesion was observed. The patient was informed about the fractured tooth; and extraction was recommended to her.The examinations also showed that adjacent third molar was healthy, favourably developed, completely erupted and well positioned within the dental arch, and therefore it was an appropriate candidate for ATT . The treAt the treatment session, oral disinfection was carried out with 0.2% chlorhexidine gluconate solution. Surgery was performed under local anaesthesia with a mandibular block . First, the fractured second molar and then the wisdom tooth were extracted. The third molar was then positioned into the recipient socket to assess its adaptation. As a result of the small size of single donor root, adequate adaptation was achieved with ease.Root-end resection was made by removing 3mm of the tooth root apex. Three millimetres deep class I root-end preparations were made using an ultrasonic power unit  with ultrasonic retrotips  and irrigated with sterile normal saline solution. The root-end cavity were dried with absorbent paper points and filled with CEM cement. The tooth was rinsed in sterile saline to remove all debris and was then replaced in recipient socket with favourable distance (1 mm) to the adjacent teeth and no interference with opposing teeth . The traThe patient was recalled for clinical and radiographic follow-ups after 1 day (post-operative check-up), 1 week , 1 month , 6 and 24 months . The postoperative period was uneventful. At the 2-year follow up visit the tooth had normal occlusion, with physiological mobility and masticatory function. Periodontal probing revealed no pockets or other pathological signs, and the patient was symptom-free. The periodontal ligament appeared intact; periradicular area had normal status and no evidence of root resorption or periapical lesion was observed.Teeth with vertical root fractured and poor prognosis are classified as a genuine indication for ATT . In the It is obvious that ATT requires size compatibility between the transplanted tooth and recipient socket. In the present case, the third molar was single-rooted which was smaller than recipient site. Thus, extraoral modification of the donor root was not required and the transplanted tooth was simply inserted, well positioned and easily stabilized in the new site. It was reported that close contact of the transplanted tooth with the alveolar bone of the recipient site may provide better blood supply for the PDL cells . AlthougThe critical factor for inflammatory root resorption after ATT is the infection of root-canal system. ATT of closed-apices teeth, therefore, necessitates pulp extirpation within 1 to 2 weeks to avoid pulpal infection followed by periradicular inflammation and subsequent inflammatory root resorption . This seThe favourable results obtained in this rare case of ATT may be attributed to distinct factors, such as maintaining asepsis during surgical procedure, atraumatic surgical extraction and replacement, preservation of the PDL cellular vitality, minimal extraoral time, good occlusal interrelationship, adequate fixation as well as filling and sealing the apex with a biocompatible root-end filling material.ATT of third molars with closed apices may be considered as a viable treatment option compared to the usual prosthetic and implant treatments for both restorative and financial reasons.


Florid cemento-osseous dysplasia (FCOD) is one of the uncommon dysplasias affecting the maxillofacial region. The age group may vary from 19 to 76 years and typically presents in the 4th and 5th decades. In most cases patients do not have hereditary basis of disease, and only a few familial cases have been documented. As far as we know this is the 1st reported case of familial FCOD in an Indian family. The mother and son exhibited multiple sclerotic masses in both jaws. The mode of transmission appeared to be autosomal dominant with variable phenotypic expression. FCOD is a type of sclerosing lesion characterized by multiple exuberant lobulated densely opaque masses restricted to alveolar process in either or both jaws . The terFCOD is rare in Indian population, and less than 10 cases have been reported in literature, but no cases of familial FCOD in Indian family have been reported till now , 5.A 18-year-old male patient  came to Biochemical analysis of serum alkaline phosphatase, calcium and phosphorus was carried out to differentiate from Paget's disease and was found within normal limits.Patient reported that her mother also had similar problem. She had undergone a lot of treatment for the same problem . All theShe was also subjected to CT scan which showed radio-opaque masses scattered throughout mandible and maxilla . RadiogrBased on the history, clinical feature, radiographic findings, and histological report a final diagnosis of familial FCOD was made. Both patients were educated about their disease and its course. As the son was asymptomatic, a periodic followup was advised. Both were advised to keep good oral hygiene. In mother's case surgical curettage was advised in the areas where she was experiencing pain as it would have been impractical to resect the lesion as it was occupying almost the whole of mandible and maxilla. Surgical intervention may lead to infection in the area which would have been very difficult to treat.Surgical remodeling with curettage was done in mother's case. Antibiotics were also prescribed. Excised specimen was sent for histopathological confirmation, which confirmed our diagnosis. She became symptomless after one week. Both patients are under regular followup.The classification of cemento-osseous lesions of the jaws has long been matter of discussion for clinicians as well as pathologists. The current classification released by WHO in 1992 is based on age, sex, and histopathologic, radiographic, and clinical characteristics as well as location of lesion .FCOD is the sclerotic lesion of the jaw characterized by diffused opaque masses limited to the alveolar process. In the past these calcifications have been interpreted as chronic diffused sclerosing osteomyelitis . ChronicPaget's disease of bone may also mimic this condition, only difference being that FCOD is centered above the inferior alveolar canal whereas Paget's involve the entire mandible .FCOD is more commonly seen in middle-aged black women although it may also occur in Caucasians and Asians . Though Radiographically, the lesion appears as multiple sclerotic masses located in two or more quadrants usually in the tooth-bearing area. They are often confined within the alveolar bone .Histologically, these lesions are composed of anastomosing bone trabeculae and layers of cementum like calcifications embedded in a fibroblastic background , 5, 16.Treatment of FCOD varies from case to case. Complete resection is generally impractical as lesion almost always occupied the entire mandible and maxilla. In asymptomatic patient it is wise to keep the patient under observation without surgical intervention. If required a remodeling resection is recommended for esthetic reasons .The management of symptomatic patients is more difficult because chronic inflammation and infection develop within this densely mineralized tissues .Antibiotics also have poor penetration in these areas and are not generally effective. In our case mother was having pain in the right upper back teeth region where the bone was exposed to the oral cavity .Although clinical and radiographic findings were sufficient to establish the diagnosis in our case, histopathological examination was done in mother's case from the symptomatic area from where remodeling resection with curettage was done to relieve her pain. Familial cases are quite rare, and ours is the first familial FCOD to be reported in Indian literature.


Ocular lymphomas account for five to 10 percent of all extra-nodal lymphomas. Primary uveal lymphoma is quite a rare entity and usually unilateral. We present a case of a primary uveal lymphoma with conjunctival and orbital extension, successfully managed with oral chlorambucil.A 71-year-old Caucasian man presented to our facility with visual loss in his only functioning eye (left). On clinical examination, we found a conjunctival lesion with a choroidal infiltration and a secondary retinal detachment. Ultrasound and magnetic resonance imaging studies revealed a choroidal tumour mass and two other lesions around the optic nerve. Results from an incisional biopsy revealed a low-grade B-cell lymphoma . A diagnosis of primary uveal lymphoma was made. Our patient was started on a chemotherapy regime with no effect, and then oral chlorambucil was administered, with a relatively good result. At 10 months after the start of chlorambucil treatment, a best-corrected visual acuity of 0.4 was recorded, the choroidal mass had practically disappeared and the extra-ocular lesions had shrunk.In all, 61 to 80 cases of primary uveal lymphoma have already been described in the literature. Generally, it is an indolent tumor with a good prognosis. However, there are some reports of aggressive tumor behavior a few years after initial diagnosis (about eight percent of cases). Other treatment options are orbital irradiation at low doses (20 to 40 Gy) or steroid administration. This is the first documented report of the efficacy of oral chlorambucil in the treatment of primary uveal lymphoma. Ophthalmic lymphomas account for five to 10 percent of all extra-nodal lymphomas . The majHere, we present a case of a primary uveal lymphoma with typical conjunctival and orbital extension successfully managed with oral chlorambucil.A 71-year-old Caucasian man was referred to our facility due to sudden visual deterioration in his only functional eye (left). His vision was 0.2 in the affected eye and hand movement only with the right eye, which had been amblyopic since his childhood.Anterior segment biomicroscopy results revealed a left conjunctival lesion in the superotemporal quadrant. On fundoscopy we noted a choroidal infiltration with exudative retinal detachment Figure A,B,D,F.Ocular and orbital imaging results revealed a choroidal mass  and two retrobulbar lesions around the optic nerve (6.6×4.4mm and 5.8×3.3mm) Figure C,D.Incisional conjunctival biopsy results revealed a B-cell lymphoma . Additional examinations  scans of the abdomen and thorax, and bone marrow biopsy) excluded other systemic locations of disease.Due to the clinical presentation in the absence of any systemic extension of the lymphoma, we made a final diagnosis of a primary uveal lymphoma, which is generally an indolent tumor with a good life prognosis.Our patient was referred for hemato-oncologic counseling and was initially treated with a systemic chemotherapy regime , prednisone’) with no effect; oral chlorambucil was then administered, with a good response Figure .At 10 months after the onset of chlorambucil therapy, the best-corrected visual acuity in his left eye had improved to 0.4, the choroidal mass had practically disappeared and the retrobulbar lesions had regressed. At 12-month follow-up no systemic extension was noted.Primary uveal lymphoma is a rare pathology; there have only been about 61 to 80 cases reported in the literature . The firThis condition was initially considered as an inflammatory process rather than a proliferation of lymphocytes and histiocytes of low malignancy that starts in the choroidal tissue, as it is recognized as nowadays. Conjunctival or orbital involvement in these cases is secondary . HoweverDifferentiating between these two types of lymphoma can be sometimes difficult since the immunohistochemical or histopathological characteristics of intra-ocular and extra-ocular lesions can differ in the same patient. This limits the reliability of excisional biopsy results . DiagnosILU is generally believed to be an indolent tumor with a good life expectancy. However, it can become aggressive and some systemic extension can appear a couple of years after initial diagnosis (this occurs in about eight percent of cases) . Other tChlorambucil (Leukeran®) is a nitrogen mustard alkylating agent mainly used in chronic lymphocytic leukemia . Its useIn summary, to the best of our knowledge this is the first documented report of efficacy of oral chlorambucil in treatment of a case of ILU.Written informed consent was obtained from the patient for publication of this report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.IRK was responsible for the clinical management of our patient and preparation of the first draft of the manuscript. JK supervised the treatment of our patient and revised the manuscript. MS performed the literature search and revised the manuscript. The manuscript was prepared without the help of professional medical writers. All authors approved the final version of the manuscript.


The occurrence of benign nerve sheath tumors within the neuroaxis is uncommon. Even rarer is the finding within brain parenchyma, termed malignant intracerebral nerve sheath tumors (MINST). We present a case of MINST which occurred in the frontal lobe of an 18-year-old male that recurred almost 4 years later. Imaging demonstrated a 4.0 cm lesion with an associated mass effect. He underwent a right fronto-parietal craniotomy for gross total resection. Pathology was inconclusive with a Glioblastoma Multiforme (GBM) as the most likely diagnosis, though gliosarcoma and MINST were also highly considered. Postoperatively, he was treated with chemotherapy and radiation and followed for almost 4 years, when an MRI indicated a recurrence. Resection of the recurrence was highly suggestive of MINST. Surgery was followed by radiation and chemotherapy, but, less than 7 months later, he was readmitted for a surgical-site infection, and, after multiple surgeries, and his family terminated care. Recognizing this unusual tumor in the differential diagnosis of a heterogeneously enhancing intracerebral mass can help surgeons diagnose and treat it. This report also exhaustively reviews the literature and presents diagnostic and treatment strategies. Malignant peripheral nerve sheath tumors (MPNST) typically originate from nerves of the extremities and trunk or from preexisting neurofibromas. While being very uncommon in the general population, the lifetime incidence of this neoplasm in patients with neurofibromatosis type 1 (NF1) is estimated at 8%–13% [MPNST that arise from brain parenchyma are termed malignant intracerebral nerve sheath tumors (MINST) and are exceptionally rare with only 15 documented cases in the literature . MINST hIn addition to NF1 patients, others predisposed to MINST include those with a history of ionizing radiation exposure , 8. The We present the unique case of a frontal lobe MINST which mimicked a Glioblastoma Multiforme (GBM) both radiographically and histologically. Only 15 cases of MINST have been reported in the literature. This case is the eighth in a child, the fifth associated with NF1, and the first masquerading as a GBM. We also present a review of the literature with a summary of factors including tumor size, location, and histological grade.Approximately 4.4 years ago, an 18-year-old right-handed male presented to the emergency department complaining of awakening in the middle of the night with left side numbness  and severe headache. The pain was refractory to over-the-counter medication and worsened throughout the morning with associated vomiting. The left-sided numbness also did not diminish and was accompanied by pain within the left upper extremity. He had been diagnosed at age 9 with an optic glioma and treated with one year of temozolomide chemotherapy and radiation. His past medical history was also significant for NF1, ADHD, Tourette's syndrome, shingles, juvenile rheumatoid arthritis, and depression.The neurological exam was significant for a light left upper extremity pronator drift, 4/5 strength in the left upper and lower extremities, and decreased sensation to light touch and pinprick in his left face, arm, and leg. CT scan without contrast revealed an acute parenchymal hematoma in the right fronto-parietal region with a predominantly central high density and areas of low density, which may have represented active bleeding. The area measured 4.0 cm maximum dimension with a mild amount of surrounding vasogenic edema. MRI with contrast revealed a hematoma measuring 4.1 cm AP × 3.5 cm transverse × 4.1 cm craniocaudal with an associated mass effect resulting in approximately 5 mm leftward midline shift . CentralThe patient underwent a right fronto-parietal craniotomy for resection of what was suspected to be a hemorrhagic right posterior frontal glioma. A generous right fronto-parietal craniotomy was done, and the dura was dissected free and opened in a cruciate fashion. Active arterial bleeding was encountered on the cortical surface from abnormal mass. The exact location and dimensions of the hemorrhagic mass were delineated with intraoperative ultrasound. With the help of the surgical microscope, the hematoma cavity was entered and then evacuated using gentle suction and irrigation. Multiple biopsies and frozen sections were reviewed by a pathologist and were suggestive of GBM. Using standard microsurgical techniques, an anatomical plane between the hemorrhage/hemorrhagic mass and the gliotic brain was developed. The hemorrhagic mass was removed in a piecemeal fashion. After complete resection was attained, immaculate hemostasis was performed and ultrasonography confirmed an excellent resection.Grossly, the tumor was a 2.2 × 2.0 × 0.4 cm aggregate of soft, hemorrhagic, brown-tan pieces of tissue. Microscopic sections disclosed highly pleomorphic cells with hyperchromatic nuclei and numerous mitotic figures . Some ceThe patient awakened after surgery without any motor or sensory deficits. He was given radiation and 5 cycles of temozolomide chemotherapy. MRI demonstrated a persistent 1 cm enhancing lesion in the right frontal lobe within the surgical cavity . This lePropionibacterium acnes, and he was treated with appropriate antibiotics. Pathological analysis of the lesion confirmed the same neoplasm. The patient was started on a rehabilitation program and received radiation with concurrent temozolomide chemotherapy. Seven months later , the patient was readmitted for an infection in the surgical site, and, after multiple operations, the family decided to withdraw care.At 3.7 years after the initial surgery, there was evidence of a new 7 mm nodular area of enhancement at the superior aspect of the surgical cavity associated with the dura in the right frontal lobe with flare changes . The patPrimary MINST are extremely unusual. Similar to its MPNST counterpart, the diagnosis of MINST can be formidable and necessitates a thorough investigation of clinical findings, imaging features, and histopathological characteristics. The fact that the cellular source of these tumors is uncertain further complicates matters. Some suggest an origin from Schwann cells of perivascular nerves while others favor pluripotent mesenchymal cells, but their source remains debatable , 3, 16. In terms of cytopathological findings, our patient shared similar characteristics with previously reported MINST cases. This included hypercellular spindle cells with marked variation in cellular and nuclear size, interlacing fascicular arrangement, signs of nerve sheath differentiation , presence of syncytial epithelioid tumor cells and multinucleated giant cells, and foci of necrosis . Though Like MPNST, patients with MINST have varying outcomes with many having a poor prognosis , 17, 18 Our patient did well for more than 4 years with surgery along with radiation and chemotherapy. With a survival of approximately 52 months, it is the second longest survival reported in the literature. Additionally, as this is the fifth case of MINST in a patient with NF1, it adds further evidence to the possible association of NF1 with this neoplasm.Awareness of this tumor, extensive surgical extirpation, and thorough histological examination serve as essential components in management. It is recommended that MINST be part of a differential diagnosis for a patient with clinical and radiographic signs of an intracranial tumor as MINST have the potential to mimic glioblastomas. Reports of more cases with lengthier follow-up are needed to gain a better understanding of the effect of these modalities and help guide management plans for this infrequent tumor. MINST are rare, frequently recurring, aggressive malignant tumors with infiltrative capability which can exhibit clinical and radiographic signs of an intracranial tumor and mimic a glioblastoma. Recognizing the similarity of MINST to their peripheral counterparts, MPNST, and their possible relationship with NF1 can help surgeons correctly diagnose and treat this tumor. Although arduous, the diagnosis is confirmed through comprehensive histopathological analysis. The standard of care is surgical GTR, sometimes followed by radiation therapy and/or chemotherapy, and close, frequent follow-up due to its invasive nature and high risk of recurrence. Considering this unusual tumor in a differential diagnosis of a heterogeneously enhancing intracerebral mass can help surgeons recognize cases of MINST. More case reports and studies are needed for conclusive evidence for the best approach to managing MINST and in determining factors that influence survival.


Liposarcoma of the spermatic cord (LSC) is a rare condition characterized by a painless inguinal or scrotal mass. To our knowledge, only about 200 cases have been previously reported in the literature. These tumors are often mistaken for common scrotal swellings, such as hydroceles and hernias. We present a LSC case in which a definitive diagnosis was obtained upon histological examination. We also provide a literature review of other cases that have been reported. Liposarcoma is a rare soft tissue malignancy with aggressive behavior and poor prognosis. It is derived from mesenchymal tissue and can occur in fat cells anywhere in the body. Most malignant paratesticular tumors are sarcomas but 5 to 7% are liposarcomas . To the A 53-year-old male presented with a slow-growing, painless, left scrotum mass of two years duration and was admitted to the outpatient general surgery department for hernioplasty in December 2010. The provisional diagnosis made by his family practitioner was a left–sided inguinal hernia, and the patient was referred for surgery. Physical examination showed a large, non-tender, mobile left scrotal mass. The mass was larger with increased abdominal pressure when the patient was standing, but smaller when he was supine. Trans-illumination testing was negative. There were no constitutional symptoms, voiding complaints, history of local trauma, infection, weight loss or hereditary disease. All pre-operative laboratory tests, including complete blood count, biochemistry and chest X-ray, were normal. A pelvic computerized tomography (CT) scan was negative for retroperitoneal metastasis. Scrotal ultrasonography  revealed a 55 x 42 mm mass on the left side of the inguinal canal with internal echogenecity resembling fatty tissues and extending to the scrotum.The patient underwent exploratory surgery via a left inguinal canal approach, during which a well-defined 6 × 5 × 3 cm round mass located above the left testis and epididymis was discovered; the vas deferens was involved. Upon close inspection, there was no evidence of hernia or laxity in the inguinal floor. Intraoperative frozen-section biopsy showed malignancy of the spermatic cord. A complete radical left orchidectomy was performed with wide excision and high ligation of the spermatic cord. An ipsilateral inguinal lymph node was also removed for biopsy.The gross appearance was a solid mass of adipose tissue with a yellowish lipoma-like texture of the cut-surface. It was encapsulated, and attached to the spermatic cord. A hard tumor could be palpated in the center of the mass Figure .Histological examination confirmed a well-differentiated myxoid liposarcoma, composed of mature adipose tissue and a few scattered lipoblasts separated by fibrous septa into the lobules of varying sizes. Lipoblasts with hyperchromatic nuclei, irregularly-shaped spindle cells and abnormal cells were present in myxoid areas Figure . The suret al. described a patient who developed LSC after radical prostatectomy for prostate cancer [et al. reported a case of a renal transplant recipient in whom LSC presented on the same side as the graft at four years post-transplantation [Liposarcoma of paratesticular tissues , first reported in 1952 ,4. Most e cancer . Manzia antation . The tumThe tumor occurred more frequently in adults than children, with a range of 24 to 79 years of age and a mean age at presentation of 61 years. Overall, 22 of 38 cases 57.9%) were 60 years of age or older. The duration of disease ranged from one week to five years. The typical clinical manifestation of LSC was a slowly growing, non-tender, painless, nodular mass of varying size, located intra-scrotally above the testis or in the groin 7.9% were-12, preoHigh-resolution ultrasonography, computed tomography with contrast and magnetic resonance imaging (MRI) have become the imaging modalities of choice for the examination of the scrotum and its contents, and all can provide useful information about the lipomatous nature of these masses ,12,20. Uet al. found that the neoplasms were dominant on the right side [Vorstman ght side . Howeverght side  than on ght side . Furtherght side ,6,23, whght side ,24. Gianght side .et al. reported a case of a well-differentiated myxoid liposarcoma (WDML) combined with angiolipoma [Grossly, liposarcoma resembles lipoma, especially the lipoblastic types, but the surface may show foci with a mucinus appearance. In large tumors, multinodularity and multilocularity of fatty or cartilaginous tissue were often observed ,25,27. Hiolipoma ,21,26, aLiposarcomas tend to spread primarily by local extension. Once diagnosed or suspected preoperatively, radical orchiectomy with wide local excision and high ligation of the spermatic cord is recommended -30, as wIn spite of the likelihood of recurrence, the prognosis was satisfactory and the rate of mortality was reduced if radical orchiectomy resulted in complete clearance with a negative margin. Even if patients underwent incomplete resection, improved disease-free survival could be achieved by re-operative wide resection . Tumor sThe one reported death was of a patient with PDML who developed widespread metastases after six months follow-up and underwent chemotherapy . DetaileWe report a rare variety of spermatic cord mass having a misleading presentation. LSC is a very rare condition that can be encountered in urology or outpatient general surgery departments. It should be highly suspected in patients experiencing recurrent hernias of the inguinal region. Therefore, all surgeons should be aware of this malignancy. Careful clinical and radiological examination is helpful for appropriate preoperative diagnosis. The treatment of choice is radical orchiectomy and wide excision with high ligation of the spermatic cord. If the margin is in doubt, adjuvant radiotherapy is indicated. Given the unfavorable prognosis of sarcomatous tumors and the high frequency of recurrence, long-term periodic follow-up is necessary.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.A: Angiolipoma; DL: Dedifferentiated liposarcoma; LSC: Liposarcoma of spermatic cord; L.s: Left side; ML: Myxoid liposarcoma; NA: Not available; NRD: No recurrence of disease; PDL: Poorly-differentiated liposarcoma; PDML: Poorly-differentiated Myxoid liposarcoma; PL: Pleomorphic liposarcoma; rad: Radiotherapy; RO: Radical orchiectomy; RP: Retroperitoneum; R.s: Right side; St: Sclerosing type; TR: Tumor Resection; WDL: Well-differentiated liposarcoma; WDML: Well-differentiated myxoid liposarcoma.The authors declare that they have no competing interests.FL conceived of the study design. RT and CY collected relevant literature and modified the draft. NX and HW performed the operation. KG wrote the initial draft. XD checked the manuscript. All authors read and approved the final manuscript.


Prostatic blue nevus was first described as a benign lesion of uncertain and controversial histogenesis by Nogogosyan in 1963. Currently, 30 cases have been reported in the world literature.A 63-year-old Hispanic man presented with prostatism of several months’ evolution. Histopathological examination revealed a blue nevus associated with nodular hyperplasia and acute inflammation.Prostatic blue nevus is a rare and unusual, histologically benign prostatic lesion with limited clinical significance and a favorable prognosis. Melanocytic lesions, comprising blue nevi (BN), melanosis and malignant melanomas, are neoplastic processes observed, though rarely, in the prostate gland . ExtracuProstatic blue nevus (PBN), first described in 1963 by Nogogosyan, is a benign pigmented lesion similar to its cutaneous counterpart, in which melanin pigment is deposited in the cytoplasm of normal, hyperplastic or malignant stromal cells. Its identification is usually incidental, after a histopathological study. Its pathogenesis remains unclear and controversial, but the accepted hypothesis is that of the centrifugal migration of melanoblasts from the neural crest to the prostate during the embryonic period, resulting in their ectopic location in this organ and in others. Currently, 30 cases have been reported in the world literature ,6.We present the case of a 63-year-old Hispanic man with a history of type 2 diabetes mellitus, dyslipidemias and right nephrectomy due to a tumor 8 years previously, who presented with prostatism with a 3-month progress. He was diagnosed with benign prostatic hyperplasia during a urological examination. A digital rectal examination revealed an enlarged gland with a soft nodule in the left lobe; prostate specific antigen was within normal limits (3.4ng/mL) and a suprapubic prostatectomy was performed.Macroscopic study showed a surgical specimen measuring 4.5×4×2cm, weighing 27g that, when cut open, was multinodular, elastic in consistency, with a well-defined blackish area 1.4cm in diameter in the left lobe  and malignant lesions (melanomas). The origin of ectopic melanocytes remains controversial. It has been postulated that melanoblasts migrate from the neural crest along the mesoderm, differentiating into melanocytes in the stroma of various organs and tissues.Blue nevus of the prostate is histologically similar to its cutaneous counterpart; it is characterized by a proliferation of spindle-like stromal cells with abundant intracytoplasmic melanin pigment ,2. The dPBN is a rare lesion exclusively diagnosed incidentally, especially after histopathological examination of simple suprapubic prostatectomy specimens, as occurred in our case. The literature shows that case reports of PBN mostly concern suprapubic prostatectomy specimens and transurethral resection is commonly involved, whereas only two fine-needle biopsy cases have been reported -3.It is vital to exclude a diagnosis of primary or metastatic malignant melanoma of the prostate, which constitutes less than 1% of all melanomas. Such a diagnosis is difficult and results in aggressive surgical treatment. Very few cases of melanoma have been published and most have been secondary, either metastatic or arising in the prostatic urethra. Given the negligible possibility of malignant transformation, additional periodic checks are not advisable after a PBN diagnosis ,6.We report the case of a PBN incidentally associated with nodular hyperplasia and superimposed acute inflammation. PBN is a rare and unusual, histologically benign prostatic lesion with limited clinical significance and a favorable prognosis.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declared that they have no competing interests.NM performed the histological examination and diagnosis; LR conducted a thorough literature review and manuscript preparation. The authors read and approved the final manuscript.


Brain metastasis is a rare and late manifestation of ovarian carcinoma. A 30-year-old woman initially presenting with neurologic symptoms was later found to have mucinous ovarian carcinoma. The patient had a 6 cm adnexal mass with multiple millimetric brain metastatic lesions. Following a surgical staging laparotomy procedure, the patient received brain irradiation and systemic chemotherapy. Brain metastasis from ovarian carcinoma is very uncommon; only less than 600 cases have been documented to date in the literature and it might be seen as a late manifestation of an ovarian tumor . It usuaA 30-year-old gravida 2, para 2 woman admitted to our hospital with complaints of headache, nausea, vomiting, and right-sided blurred vision. She did not report any previous medical history or malignancy. Her neurologic examination revealed a right optic disc edema suggesting a posterior orbital mass. Her cranial computerized tomography (CT) scan showed multiple lesions that are a 6 mm mass on the right parietal lobe, a 16 mm mass on the left occipital, and another 7 mm mass on the left temporal lobe  was administered postoperatively. While documenting the patient, she was still alive 1.5 years after the initial diagnosis.The patient underwent a diagnostic laparoscopy for further evaluation of the adnexal mass. Biopsy of the adnexal mass and the liver was obtained and the pathologic examination revealed an ovarian mucinous cystadenocarcinoma with metastatic lesions of liver. The patient then was subjected to a laparotomy for a surgical staging procedure including total abdominal hysterectomy, bilateral salpingooophorectomy, bilateral pelvic and para-aortic lymphadenectomy, and infracolic omentectomy and appendectomy. Her postoperative course was uneventful and she was discharged at the 6th postoperative day. The patient was determined to have a stage IV ovarian carcinoma with brain metastasis. An adjuvant therapy including whole brain irradiation  with simultaneous dexamethasone and systemic chemotherapy , stereotactic radiosurgery (SRC), gamma knife surgery (GKS), chemotherapy, and alternative combination of these methods could be applied for metastatic brain lesions which were described in the literature before.Anupol et al. used gamma knife surgery (GKS) for a patient and had a good outcome; GKS treats multiple lesions at the same time, is easy to perform for recurrent and new lesions, and has a short hospital stay with less risk of deficit . The surOur patient was 30 years old with simultaneously diagnosed multiple metastatic brain lesions of ovarian cancer, so a surgical approach to brain lesions would not be approved. She was treated with surgical staging with the removal of the primary tumor in the abdomen, systemic chemotherapy (taxol and carboplatin) with brain radiation therapy. However, the cause of death in the majority of the patients was related to both brain metastasis and abdominal spread of disease .And although brain metastasis is uncommon in ovarian carcinoma, it may develop even in the early phase of the disease.


Pulmonary blastoma, a rare primary lung malignancy, is considered to be distinct from other lung tumors based on pathological features, clinical course, and prognosis. More than one hundred cases have been reported in literature highlighting an interesting fact about their distinctive biologic manner from histopathological features. Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. Surgery is the mainstay of treatment. The prognosis of this rare malignancy is poor and the overall 5-year survival is around 15%. Our patient presented with respiratory symptoms and was found to have right-sided chest wall mass. The patient underwent complete surgical excision followed by 6 cycles of platinum-based chemotherapy. The patient showed good subjective and objective response with no evidence of disease recurrence. We report this rare malignancy with a review of literature, and the potential to use adjuvant chemotherapy in the management of this condition. Pulmonary blastoma (PB) is classified as one of the rare primary lung malignancies and is considered to be distinct from other lung tumors based on pathological features, clinical course and prognosis. PB is composed of a mixture of epithelial and mesenchymal tissues resembling embryonic lung tissue. Surgery is the standard treatment and the efficacy of adjuvant chemotherapy and radiotherapy has not yet been established. Platinum-based chemotherapy has been used with or without postoperative radiotherapy. The prognosis of this rare tumor is poor. We describe a case of this rare pulmonary malignancy and the role of adjuvant chemotherapy in the management and outcome.A 16-year-old young Egyptian female presented with progressive shortness of breath over a period of five years. The dyspnea was gradual in onset, at times precipitated by stress, but most of the time there was no precipitating factor. It was associated with severe nonproductive cough and bilateral headache. There was no significant past medical history. There was no history of fever, night sweats, and wheeze or chest pain. Also, there was no hoarseness of voice or hemoptysis. The patient sought medical advice at a private health center when the patient's daily activities gradually started getting affected. The patient was prescribed Salbutamol inhaler which gave her temporary relief. At the same time, the patient was diagnosed to have hyperthyroidism and was started on carbimazole. The patient took these medications for about 4 weeks and discontinued them against medical advice as she experienced lethargy and easy fatigability due to the sideeffects of the prescribed drugs. After a brief period of relief of symptoms, the patient again started to have dyspnea and presented to the Cardiothoracic Unit in our hospital for evaluation. Baseline investigations were done, which included complete blood count, renal function tests, and liver function tests; all were within the normal range. Chest X-ray showed a well-demarcated right upper lobe mass close to hilum . Chest cPulmonary blastoma is a rare primary lung malignancy, comprising only 0.25–0.5% of all malignant lung neoplasm . In 1952More than one hundred cases have been reported in literature highlighting an interesting fact about their distinctive biologic manner from histopathological features . PB is cAbout 80% of CBPB occur in adults, and can occur at any age between birth and seventy years . Unlike Immunohistochemistry plays a vital role in the diagnosis of CBPB. Each component can be clearly distinguished with each other when a combination of both epithelial and mesenchymal markers is used . In our Surgical resection is the mainstay of treatment for the patients with PB. A number of cases of surgical approach with curative intent have been reported including an interesting case with a 24-year complete remission postsurgery . Literature regarding the efficacy of adjuvant chemotherapy and radiotherapy is scarce. The vague role of chemotherapy in the adjuvant setting is probably due to rarity of this malignancy and seems to be individualized in reported cases. Various combinations of chemotherapeutic drugs have been tried as neoadjuvant and adjuvant treatment. However, several case reports document the use of platinum-based adjuvant treatment , 4. Our patient received 6 cycles of the ICE protocol comprising of ifosfamide, carboplatin, and etoposide. Reevaluation was done by CT scan of chest after 3rd and 6th cycles of chemotherapy which showed no evidence of recurrence. The patient experienced acute emesis postchemotherapy during the first 3 cycles. Prophylactic granulocyte colony-stimulating factor (G-CSF) was given for 1 week from day 5 after each cycle. The patient had not been considered as a candidate for radiotherapy due to the presence of poor prognostic features, in addition to lack of sufficient data supporting the definite role of radiation in the adjuvant setting. The patient is on ongoing 3 monthly follow up with complete remission. Reevaluation CT scans will be done, and the patient is advised to seek immediate medical care in case of appearance of previous or novel symptoms. Although, several individual case reports of long-term survival have been documented, the overall prognosis of this rare malignancy is poor, and two-thirds of patients die within 2 years of diagnosis . In one The factors contributing to the unfavorable prognosis are the biphasic type, tumor recurrence, metastatic disease on presentation, tumor size over 5 cm, and frequent lymph node involvement , 2.In our case, the decision to start adjuvant treatment was based on the presence of two important prognostic factors, namely, the large size of the tumor on presentation (around 11 cm) and biphasic pattern of the disease. Despite not receiving any postoperative radiotherapy, the patient continues to be in remission until last followup. In conclusion, whether adjuvant treatment plays a role in the remission or not is a debatable issue, this case suggests a wait- and- watch approach when platinum-based chemotherapy is given as an adjuvant in the management of PB. Although a large series of case studies is required to further analyze and establish a framework for the definite role of adjuvant chemotherapy, and owing to the rarity of this malignancy, adjuvant management following surgery may be based on individual cases depending on the histopathology and stage of the tumor.


Purpose. Meningioangiomatosis (MA) is a rare, benign lesion that commonly mimics other intracranial malformations in clinical presentation and appearance on imaging. The case presented and the literature review performed highlight the importance of combining MRI and CT results to better characterize intracranial lesions and including MA on the list of differential diagnoses of patients presenting with seizures. Methods. The case described is of a 19-year-old male with a 10-year history of worsening seizures refractory to multiple drug regimens. MRI revealed an atypical vascular malformation. The patient underwent surgical resection of the epileptogenic cortex. Results. Although the radiologic impression of the lesion was a vascular malformation, pathological examination revealed MA. A literature search performed highlights the variability of the appearance of MA on CT and MRI and suggests the utility of the T2 GRE sequence in illustrating the presence of calcification and, in a lesion with other characteristic features, the diagnosis of MA. Conclusion. MA can be a difficult diagnosis to make based on imaging findings alone. However, in a patient with a characteristic history and presentation, the presence of a calcified mass on CT and MRI brain susceptibility artifact on a T2 GRE sequence may suggest MA. Headaches and seizures are the most common clinical presentation in sporadic meningioangiomatosis (MA) , and seiHere, we present a case of MA, emphasizing the difficulty in establishing the diagnosis, the surgical technique used to resect the epileptogenic foci, and the postoperative outcome in terms of minimized frequency of seizures. The case reported demonstrates that MA can mimic several other pathologies on imaging and clinical presentation. It is therefore, imperative to educate clinicians on the possibility of this diagnosis especially in the setting of seizures and nonspecific neuroimaging findings. A review of the published literature on MA was done in May 2010 using PubMed to find all articles on meningioangiomatosis, meningeal angiomatosis, meningio-angiomatosis, meningoangiomatosis, or meningo-angiomatosis. In order to be included, case reports and series of patients published in the literature had to be in English and had to include data about the imaging appearance of the lesions in the patients reported either within text and/or figures. The search resulted in 91 papers, 61 which were used in data collection [The patient was a 19-year-old male with a ten-year history of epilepsy, as well as transient episodes of severe morning headaches with nausea, vomiting, and dizziness. At age nine during a workup for severe headaches, he was found to have a presumed right insular vascular abnormality on imaging. A month later, the patient began having seizures. He was managed medically for ten years; however, during this time, his seizures progressed in frequency from occurring approximately once every six to nine months to almost daily simple or complex partial seizures and rare generalized tonic-clonic seizures despite maintenance on high-dose antiepileptic therapy. Given his refractory and progressive disease, he eventually presented to clinic for consultation about possible surgical management. The only finding on physical examination was distal left upper extremity weakness and hand muscle atrophy resulting from an injury in a motor vehicle accident sustained at the time of a seizure. A magnetic resonance imaging (MRI) scan performed one year prior to surgery showed a suspected single right posterior frontal flow void with adjacent cortical foci of susceptibility artifact compatible with calcification or hemosiderin from remote, small hemorrhages suggesting an atypical vascular lesion Figures . The floThe patient was elected to undergo intracranial seizure monitoring with subdural grid and depth electrode placement in order to determine the location of all epileptogenic foci.A craniotomy was performed, and a 64-contact grid was placed over the lateral surface of his right frontal, temporal, and parietal lobes. Three depth electrodes consisting of 12-contact leads were also placed stereotactically into the amygdala, the anterior/middle hippocampus, and the middle/posterior hippocampus.Over the next ten days, the patient remained on the inpatient ward for intracranial seizure monitoring. The patient had 4 seizures that all localized to the area of the abnormality on his MRI scan, providing good evidence for a possible surgical cure with resection.The patient was taken back to the operating room for resection. Intraoperatively, the points on the grid with the highest activity were identified. Utilizing navigation, the area was confirmed to localize to the lesion on the MRI scan. The pia was then opened to demarcate the borders of the proposed resection. Subpial dissection was then carried out, and, after initial removal of the superficial cortex, there was a significant amount of fibrous tissue identified. A portion of the tissue was sent for frozen section which the pathologists felt was consistent with MA. A subtotal resection was completed without any apparent complications.Postoperatively, the patient had some left-sided weakness, dysarthria, and left facial weakness. At one month postoperatively, he was almost completely back to his baseline with only minimal residual left facial weakness. The patient did not experience further complex partial seizures. In the immediate postoperative period, he had occasional auras every two to three days, but at one month postoperatively, this had decreased to once every four to six days. At his 20 month visit, he had no auras and his previously almost daily seizures had been eliminated. He was continued on his antiepileptic medications; however, a few days prior to his one year postoperative visit, he stopped taking his seizure medications for two days and suffered a generalized tonic-clonic seizure. A surveillance MRI performed at this visit showed encephalomalacia and no change in the size of the residual lesion Figures . The patOn pathological examination, there were numerous thickened blood vessels surrounded by sheaths of well-differentiated meningothelial tumor cells, generally ranging from one to five cell layers thick, noted throughout the distorted cerebral cortex Figures . There wMeningioangiomatosis (MA) is a rare, benign lesion usually affecting the leptomeninges and underlying cerebral cortex but has also been described in the brainstem and thalamus , 3, 5. TThe patient in this case report presented in a way that is typical for MA without NF. The majority of those affected are children and young adults , 21. MA Because of its rarity, information about the characteristics of MA and its appearance on imaging comes mostly from case reports with few numbers of patients. Moreover, to our knowledge, there has not been an attempt to combine data from the different case reports available to determine with better accuracy how these lesions appear on MRI or CT. The literature search performed found 61 reports describing 101 cases of MA that included either a discussion or illustration of the characteristics of the lesion on imaging. The sex, age, side of involvement, lobe involved, and presenting complaint of patients found in literature review are shown in The first imaging modality analyzed was computed tomography (CT). Out of the 101 patients included in the literature search, 68 patients' workups included CT scanning, with 35 patients obtaining contrasted studies. Nine patients had completely normal CT scans. The 59 remaining patients were evaluated for density of the lesions on CT relative to the surrounding brain parenchyma, enhancement, and presence of calcification. The results are shown in The second imaging modality examined was magnetic resonance imaging (MRI). From the 101 patients included in the literature search, 79 patients' workups included MR imaging. Two patients had completely normal MRI scans. The 77 remaining patients were evaluated for intensity of lesions on T1- and T2-weighted sequences, enhancement on T1-weighted sequences, presence of susceptibility artifact, and surrounding edema. The results are summarized in The third imaging modality analyzed was cerebral angiography. The results are summarized in MA can be even more diverse in its presentation on imaging because of the different morphologies that lesion can assume; for example, MA can be gyriform , 23–30, Based on the findings of the literature search conducted herein, the most common findings in MA are enhancement on T1-weighted MR imaging, and calcification on CT, with a prevalence of 79.6%, and 89.6%, respectively. With the exception of these characteristics, no other generalizations can be made. The reported findings are observation based and as such are subject to bias and intra-observer variability given that the studies were interpreted by different radiologists. In addition, some reports identified had inadequate figures or descriptions and were limited as only select or representative images are shown as opposed to the entire study. Moreover, missing or “unspecified” data could skew the results particularly if the omitted images are not randomly distributed, and the majority of them were to demonstrate a particular phenomenon. For example, if the 27 “unspecified” study findings in reality demonstrated edema, this would change the results drastically. However, with the acknowledgement of these limitations, the literature review conducted herein does identify certain trends that could be important in helping diagnose MA in the future. Several of the cases where a T2 GRE MRI sequence was obtained identified susceptibility artifact which correlated well with CT findings of calcification. That is, the MRI finding of susceptibility artifact appears to correlate with the presence of calcification on the CT. While chronic hemosiderin from cavernous malformations will show susceptibility artifact on T2-GRE, it will not typically show calcification on CT. Cavernous malformations will also usually be a single focus rather than a gyriform morphology and will typically have a “popcorn” appearance of T2 hyperintensity and hypointensity on standard T2-weighed imaging. The T2 GRE sequence has become a fairly routine part of brain MRI protocols and is particularly important for epilepsy protocols. This case report and the review of the literature suggest yet another use for the T2 GRE sequence, suggesting the presence of calcification, and in a lesion with other characteristic features, the diagnosis of MA.Another imaging characteristic of MA identified in the literature review is that a significant number of the lesions exhibit at least some edema and mass effect. This is in contrary to some reports that describe that MA is typically a mass with relatively little mass effect and edema for its size due to its origin as a hamartomatous or vascular process rather than a malignant neoplasm. In our search, almost 46% of cases showed at least some edema in the area surrounding lesion; therefore, clinicians should not rule out MA simply by the presence of edema or mass effect.CT and MRI are both important modalities for establishing MA as a potential diagnosis. In our case, both calcification on the head CT and T2 GRE susceptibility artifact on the MRI of the brain were present and in retrospect perhaps should have moved up MA in our differential. Moreover, the present case illustrates well that what may appear to be flow voids on MRI preoperatively may simply be signal void from coarse calcification, a consideration that should be kept in mind. MA can be a difficult diagnosis to make based on imaging findings alone and can be mistaken on MRI of the brain for other pathologies such as meningiomas, cavernous malformations, and other vascular abnormalities. A diagnosis of MA should be considered when a young patient presents with a headache, seizure, or with increasing difficulty in controlling a known seizure disorder in the setting of a calcified mass on CT and difficult to characterize mass on MRI. If calcification is present on a CT scan of the head, the possibility of a diagnosis of MA should be considered, especially if the mass is noted to be consistent with an atypical vascular malformation on MRI of the brain, as was the situation in this case report. The case reported herein and the review of the literature suggest that the presence of MRI brain susceptibility artifact on a T2 GRE sequence in a patient with a characteristic history and presentation may suggest the presence of a calcified lesion and possibly MA if seen in conjunction with the other typical findings on imaging; thus, in this patient population, when T2 GRE susceptibility artifact is present, this should prompt evaluation with a CT scan of the head if one has not already been obtained.


Background. Granulosa cell tumors (GCTs), representing ~2% of ovarian tumours, are poorly understood neoplasms with unpredictable and undetermined biological behaviour. Design. 5 unusual presentations of GCT and a retrospective 14-year (1997–2011) surgical pathology review based on patient sex, age, tumour type and concurrent pathology findings are presented to discuss the “myths and realities” of GCTs in the context of relevant evidence-based literature. Results. The 5 index cases included (1) a 5 month-old boy with a left testicular mass, (2) a 7-day-old neonate with a large complex cystic mass in the abdomen, (3) a 76-year-old woman with an umbilical mass, (4) a 64-year-old woman with a complex solid-cystic pelvic mass, and (5) a 45 year-old woman with an acute abdomen. Pathological analysis confirmed the final diagnosis as (1) juvenile GCT, (2) macrofollicular GCT, (3) recurrent GCT 32 years later, (4) collision tumour: colonic adenocarcinoma and GCT, and (5) ruptured GCT. Conclusion. GCT is best considered as an unusual indolent neoplasm of low malignant potential with late recurrences that can arise in the ovaries and testicles in both the young and the old. Multifaceted clinical presentations coupled with the unpredictable biological behaviour with late relapses are diagnostic pitfalls necessitating a high degree of suspicion for accurate clinical and pathological diagnosis. Granulosa cell tumours (GCTs) though accounting for approximately 70% of malignant sex-cord stromal tumors are rare, comprising only 2–5% of all ovarian neoplasms –3. TheseThe aim of this study is to discuss the “myths and realities” of GCTs through a series of indexed cases in the context of relevant evidence-based literature. Five patients presented to our hospital with “unusual” GCTs, prompting a complete chart review for each patient. Further, a comprehensive fourteen-year (1997–2011) search using the Laboratory Information System (LIS) identified additional patients with GCTs. Original slides were obtained to verify their histomorphological diagnosis. Patient demographics including age, sex, and tumour site were collected for analysis. A literature search using the National Library of Medicine Interface PubMed was conducted using the search terms “granulosa cell” limited to the English language. The bibliographies of these manuscripts further identified relevant secondary sources. This study was conducted with ethics approval from the University of Saskatchewan Biomedical Research Ethics Review Committee.A left scrotal mass was detected in a 5-month-old baby boy. Ultrasonography confirmed the mass to be a 2 cm lesion, and an orchiectomy was carried out. On histological examination, the tumour was solid with regions of cystic spaces Figures . Amphophα-fetoprotein, and β-human chorionic gonadotropin (β-HCG). The young girl was taken to the operating room for a laparotomy, which revealed a large cystic mass of the left ovary with torsion  scan, which showed the mass to be surrounded by a large volume of fluid . Hematol torsion . An ooph On histological examination, a multicystic lesion lined by granulosa cells was identified , without A 76-year-old woman presented with a growth at the umbilicus and bowel obstruction. CT scan showed a complex mass arising from the anterior bladder wall/dome of the bladder . With th An incidental pelvic mass was detected in a 64-year woman with a past history of total abdominal hysterectomy and bilateral salpingooophrectomy for a GCT 16 years prior. A CT scan confirmed the presence of a large heterogenous solid-cystic pelvic mass . The pre A 45-year-old woman presented to the Emergency Room with a two-year history of menorrhagia and acute dyspnea. An enhanced CT scan revealed a giant mass  filling the pelvis and abdomen and causing displacement of the bowel loops. A pulmonary embolus was additionally detected, and she was started on anticoagulants. The patient developed imminent renal failure and her hemoglobin levels continued to drop despite receiving 9 units of blood. It was suspected that her mass was haemorrhaging, and she was taken to the Operating Room. A 35–40 cm, multilobulated, haemorrhagic mass was found in her right ovary, and a total abdominal hysterectomy with bilateral oophorectomy, with en-bloc mass resection and partial omentectomy were carried out.  Microscopic examination showed the presence of sheets of neoplastic cells interspersed with rupture and acute hemorrhage . The ova A 14-year (1997–2011) surgical pathology review yielded 37 cases of GCT, with an overall prevalence of 0.0041% (37/902 100). Of these cases, 36 were females and one male (Index case 4). Within the female population, patient's age ranged from 7 days to 85 years, with a mean of 52 years (median 48 years). Locations of GCTs included 17 cases in the right ovary (47.2%), 15 in the left ovary (41.7%), and one (2.8%) in each of the bowel, abdominal wall, and omentum. Only four (10.8%) GCTs were of the juvenile type. Concurrent pathologies included leiomyoma of the uterus (21%), benign cysts (14%), adenomyosis (7%), coexisting ovarian adenocarcinoma (3%), and endometrial carcinoma (3%).FOXL2 gene [ Granulosa cell tumours (GCTs) were first described in 1855 by Rokitansky as chronicled in Chew et al.'s manuscript . GCTs arXL2 gene . In men,The typical clinical scenario of a GCT is usually a middle-aged female presenting with a pelvic mass who is cured by en-bloc resection of the mass. Pathological diagnosis is often straightforward in these typical cases and the tumour rarely reoccurs. These characteristics however are not generalizable to all GCTs as these lesions can present in many different facets and have a tendency to behave unpredictably, thus complicating both diagnosis and therapeutic management. Accurate recognition of the true “realities” of multifaceted GCT is therefore vital for the precise diagnosis and management of these lesions. Some of the existing myths of GCTs will now be contrasted with their true realities in the context of evidence-based literature.Ovarian cancer is the second most common type of gynecological malignancy . This caAlthough predominantly occurring in the granulosa cells of the female ovary, GCTs are also reported to arise within the male testis, as seen in our index case 1. Testicular sex-cord stromal tumours are rare, comprising only 4% of all testicular tumours . JuvenilMost patients with GCTs are perimenopausal or early postmenopausal, with a median age of diagnosis between 50–54 years . NeverthDifferences between AGCT and JGCT are distinct; therefore, accurate identification is critical to guide patient management. Unlike AGCT, JGCT is considered by many to be a relatively benign tumour and in infantile males is the common type of sex-cord stromal tumour of the testis . Cells pTwo-thirds of GCT patients present with endocrine syndromes due to functional tumours . EstradiGCTs are the most common estrogen-producing neoplasms in females and are found to produce estradiol in approximately 40–60% of patients. This estradiol production is directly related to the release of testosterone secreted by the theca cells. However, not all GCTs are hormonally active or have theca cells that secrete testosterone, and therefore diagnostic testing for these hormones lacks sensitivity and specificity. Normal granulosa cells are responsible for production not only of estradiol but also peptide hormones including inhibin, activin, follistatin, and antimullerian hormones . GCT patMullerian inhibitory substance (MIS) which is produced in the developing follicles is often elevated in GCTs, though it is not specific for diagnosis , 3. ThisFollicle regulatory protein is secreted by the granulosa cells and is elevated in some patients with GCTs. However, the clinical significance of this marker is still undetermined , 27. Primary synchronous malignancies are rare, with an incidence of 1–6% among cancer patients. The most common pair of synchronous lesions involves the endometrium and the ovary . GCTs haUterine pathologies that have been reported to occur with GCT include glandular hyperplasia, atypical adenomatous hyperplasia, adenocarcinoma insitu, and invasive carcinoma . Endomet The rarity of these lesions prevents randomized control trials to determine the specific best consensus practice guidelines for the management of GCT. No standard management protocols exist for the management of recurrent GCT. The mainstay treatment for GCT is the same as for epithelial ovarian cancer, that is, surgical excision , 20, 27. Three forms of adjuvant therapy have been suggested to use in combination with surgery: hormonal therapy, radiotherapy, and chemotherapy. Hormonal therapy is believed to act directly by affecting the tumour and/or indirectly by suppressing gonadotropins or endogenous steroids . AromataGCTs are generally large, smooth, or lobulated tumours . On grosGCTs are sex-cord stromal neoplasms that on microscopic examination contain sex-cord-derived epithelial elements admixed with mesenchymal elements with a variety of combinations and degrees of differentiation . Fibrobl Immunohistochemical analysis can be used to confirm the diagnosis of GCTs if the lesion's morphology is non-predictive of histogenesis. A study by Nofech-Mozes et al. recently described the concordant immunohistochemical characteristics of primary and recurrent GCTs. Inhibin, calretinin, CD56, and CD99 are part of the immunoprofile for both types of GCT; however, the lack of a single specific marker necessitates a panel of antibodies for the detection of these lesions . GCT celGCTs are unpredictable neoplasms that have the ability to extend locally or spread by lymphatics, especially to the para-aortic lymph nodes. Alternatively, dissemination may occur through hematogenous spread, as evidenced by parenchymal involvement . DistantRecurrent disease tends to occur many years after the initial diagnosis. A quarter of GCT patients will have recurrences, and the mean time to their detection is 5–10 years , 27. 10–The overall ten-year survival rates in patients with GCT range between 60 to 90% . Approxi GCT is best considered an unusual indolent neoplasm of low malignant potential with late recurrences that can arise in the ovaries and testicles in both the young and the old. The multifaceted clinical presentations coupled with the unpredictable biological behaviour with late relapses are diagnostic pitfalls necessitating a high degree of suspicion for accurate clinical and pathological diagnosis. Surgery continues to be the primary cornerstone of initial treatment with chemotherapy and/or radiotherapy being reserved for advanced or recurrent disease states. Lack of evidence-based predictive and prognostic factors continues to be a deterrent in accurately predicting the biological behaviour of individual GCTs. However, long-term lifelong followup including physical/pelvic exam, abdominal/pelvic CT scan, and/or tumor markers as available is recommended in all patients with GCTs as delayed tumor recurrences beyond 5 years are characteristic of this disease.


Oral leiomyoma is a benign smooth muscle tumor that occurs most frequently in the uterine myometrium, gastrointestinal tract, and skin. Incidence in the oral cavity is considered uncommon. Most cases are reported in adults, with very few cases described in children. A rare case of multiple leiomyomas localized on the tongue, cheek, and floor of the mouth of an 8-month-old baby is reported. The diagnosis of leiomyoma in the oral cavity is mainly determined by histological studies; however, immunohistochemical tests are recommended in order to differentiate from other tumors. Surgical excision of the lesion appears to be the best treatment option. A review of the literature did not reveal any previously reported case of multiple oral leiomyomas. Leiomyomas are benign mesenchymal tumors arising from smooth muscles. They frequently occur in the uterus and gastrointestinal tract, but they can initiate wherever smooth muscle cells subsist. Histologically, there are three types of leiomyomas: leiomyoma (solid), angiomyoma (vascular leiomyoma), and the rare form of epithelioid leiomyoma (leioblastoma). Oral leiomyoma is a rare tumor, with most of the cases noted in adults and few cases reported in children. The first case of a mandibular intraosseous leiomyoma in an 8-month-old baby was recorded by Bertolini et al. . The tumμm to be stained with Hematoxylin-Eosin technique. Microscopically, fusiform cell proliferation with elongated nuclei and eosinophilic cytoplasm was observed. Several blood vessels lined by a thin layer of endothelial cells were observed intercalated in the fascicules (An 8-month-old girl was referred to the Department of Oral and Maxillofacial Surgery of the University Hospital San Vicente in Medellín, Colombia, for evaluation of multiple erythematous lesions on the tongue, cheek and floor of the mouth . The lesscicules . Immunohscicules . The immscicules . The patTo the best of our knowledge, this is the first case of multiple leiomyomas in an infant. Oral leiomyomas are considered uncommon neoplasms, representing only 0.016% to 0.065% of all the leiomyomas . The vasMost of the vascular leiomyomas are nodular, painless, slow growth lesions, less than 2 cm in diameter, and of a color that can vary from white to blue . This caα-smooth muscle actin, and muscle specific actin. In this context, the immunohistochemical technique is an important aid, in which these tumors express immunoreactivity for SMA and negativity for the S-100 protein. In this case, fusiform and epithelial-like cell areas were observed and the immunohistochemical study revealed the expression of vimentin, desmin, MSA, and SMA within the tumour cells. Immunohistochemical findings similar to those noted in the case presented here have also been reported [The vascular leiomyoma is characterized by a well-defined proliferation of mesenchymal tapered cells with eosinophilic cytoplasm and elongated basophilic nuclei that show tapered endings . Immunohreported , 6.Wide surgical resection is the most reported treatment in reviewed literature with successful results. However, to remove the tumor, a carbon dioxide laser has been used . Recurre


Dedifferentiated chondrosarcoma (DDCS) is a rare and aggressive bone tumor with poor prognosis. Primary DDCS of the mobile spine is extremely rare, particularly in the cervical spine. We herein describe a first case of cervical DDCS in an 81-year-old male presenting with a slowly growing mass. Radiographs showed an expansion of the cortical contour of the C2 lamina and a soft tissue mass with punctate calcification. Magnetic resonance imaging demonstrated a lobulated lesion expanding over the entire lamina and pedicles of C2 with the tumor protuberant to the adjacent soft tissue. A complete tumor resection was performed. Histologically, the majority of the tumor was a low-grade chondrosarcoma component. However, atypical spindle cells that had proliferated in a fascicular pattern with a collagenous stroma, mimicking fibrosarcoma, were focally observed without a transitional zone, and these features confirmed that the tumor was DDCS. There aet al.  showed tAn 81-year-old male consulted our department with a six-month history of swelling and mild cervical spinal stiffness. He also noticed a slowly growing mass. He had a history of malignant lymphoma that was treated by multi-agent chemotherapy and had achieved a partial remission. His physical examination was unremarkable except for the presence of a hard upper cervical spine mass. The clinical blood examination did not show any abnormality. Radiographs of the cervical spine showed an expansion of the cortical contour of the C2 lamina and a soft tissue mass with punctate calcification. Scalloping of the spinous process of C3 was also observed (Figures Microscopically, the majority of the tumor was composed of the proliferation of chondrocytic cells with enlarged hyperchromatic nuclei, accompanied by chondromyxoid matrix and permeating bone trabeculae indicating low-grade CS Figures a and b. The term DDCS was introduced in 1971 . It is tIn the 57 cases of DDCS reported, calcification was found in around 50% of the lesions and an extraosseous mass was observed in roughly 55% of tumors . Notablyen bloc resection with negative margins has been shown to decrease the rates of local and metastatic recurrence [en bloc resection, if possible, is now recommended for many primary tumors of the thoracic, lumbar, and sacral spine. However, in cases affecting the cervical spine, the proximity of the vertebral arteries, the complex bony structure, and the importance of the cervical nerve roots prevent the use of an ideal en bloc resection [en bloc resection of the tumor was practically impossible because of the bilateral tumor invasion into the C2 pedicle, so tumor curettage was performed. Although the tumor was totally removed macroscopically, the recurrence of the tumor was thought to be inevitable and a careful postoperative observation would be needed in such case.CS, particularly DDCS, is known to be resistant to the chemotherapy and radiotherapy. Thus, surgery remains the only potentially curative treatment for DDCS. The local recurrence rate of DDCS rate was over 50% in previous studies, but this decreased after radical surgery with a clean surgical margin ,11. Regacurrence ,13. Theresection . In the et al. [As mentioned above, DDCS is not sensitive to radiotherapy. However, if the tumor cannot be completely resected, or in cases of local recurrence, radiotherapy should be considered. Conventional radiotherapy using spinal cord dose limits of 45 to 50 Gy, given to the full cross section of the cord, is well below the dose needed to control most sarcomas including CS . Therefoet al.  reportedet al. .et al. suggested that there was an improved survival in the DDCS patients receiving doxorubicin and cisplatin as first-line chemotherapy, although subsequent papers have failed to confirm this [Distant metastasis, particularly lung metastasis, is the main cause of death due to DDCS and the development of effective adjuvant chemotherapy regimens to control distant metastasis is critical for improving the prognosis of DDCS. However, as mentioned above, DDCS is known to be resistant to chemotherapy. The efficacy of adjuvant chemotherapy has been reported in 23 patients out of 42 cases of DDCS . The fivirm this ,19. LargUrokinase and Tissue plasminogen activator are expressed in the metastatic lung lesions [An abrupt interface between the low-grade cartilaginous and high-grade sarcomatous components is a critical histological feature of DDCS. In the literature, the dedifferentiated component of DDCS could be variable, including conventional osteosarcoma, malignant fibrous histiocytoma, fibrosarcoma, anaplastic spindle-cell sarcoma or telangiectatic osteosarcoma . In this lesions . The resWe herein reported the first detailed case of DDCS in the cervical spine. Histologically, the low-grade chondrosarcoma component and atypical spindle cells resembling fibrosarcoma were observed in the resected tumor without a transitional zone. These features confirmed that the tumor was DDCS.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.CS: chondrosarcoma; DDCS: Dedifferentiated chondrosarcoma; CT: computed tomography; MRI: Magnetic resonance imaging; LC: local control; GyE: gray equivalent.The authors report no conflicts of interest.YM, YT and KH conceived of the study, collected data and drafted the manuscript. TN and KK participated in the design of the study. SO participated in the collecting and editing of images. MH helped in drafting the manuscript. AF corrected and revised the manuscript. AS, TM, YO and YI participated in coordination and helped to draft and edit the manuscript. All authors read and approved the final manuscript.


The authors report a clinical case of alveolar adenoma presenting as a solitary pulmonary nodule which was positive to PET and deeply located in the lung. Few cases of alveolar adenomas have been reported in literature; these lesions are considered pulmonary neoplasms with benign behaviour, usually presenting as a peripheral or subpleural coin lesion; the PET activities of such neoplasms were unknown.The present clinical case was singular for the deep location of the nodule and its tight adhesion to left inferior pulmonary vein requiring a lobectomy. In addition, alveolar adenoma PET behaviour has been reported as light positivity. Alveolar adenoma is a rare lung neoplasm and few cases have been reported in literature. Such lesions are considered as benign neoplasm and no recurrences have been described. These neoplasms are usually located peripherally in the lung and their PET activities are unknown.We report the first case of alveolar adenoma positive on PET scan, deeply located in left lower lobe.A 54-year-old, non-smoker woman presented to her primary care physician for chronic, non-productive cough and mild dyspnoea. Her past medical history included partial gastrectomy for gastric ulcer, colecystectomy, dyslipidemia and gastric reflux disease. She also reported a previous occupational exposure to benzene.Clinical examination was unremarkable and pulmonary function was normal. The chest X-ray and the subsequent enhanced chest computed tomography (CT) scan revealed the presence of a well-circumscribed, ovular, smooth edged, dense pulmonary lesion in the left inferior hilum Figure . The diaIn order to exclude a malignancy, a fibreoptic bronchoscopy with bronchial washing and a percutaneous CT-guided fine needle aspiration biopsy were performed. Both the examinations resulted negative for malignant disease.Notwithstanding the negative results of the cytological investigations, the PET finding and the contiguity to the hilar structures convinced the authors to remove the pulmonary nodule. Therefore, a minimally invasive muscle-sparing lateral thoracotomy was performed in order to biopsy the pulmonary lesion. The intraoperative histological examination by frozen section suggested a cystic, well-delineated lesion without sign of malignant disease; however, the finding had to be confirmed by the definitive histology obtained by paraffin block.Considering the nodule localization (inseparable from the inferior pulmonary vein) and the intraoperative histological finding, we decided for a left lower lobectomy. The surgical procedure and the postoperative course were uneventful. The patient has been discharged 5 days after the operation in good clinical condition; after one year, she is in good health and the chest CT scan doesn't reveal any sign of recurrence.The resected lung segment contained a well-defined yellowish, round mass, with a diameter of approximately 2 cm, with a tight adhesion to the wall of the adjacent left inferior vein.Histologically, the mass was well demarcated Figure  and compDue to the morphological apparence and immunohistochemical findings of the lesion the final diagnosis was alveolar adenoma of the lung.3 and another one was a multiseptated huge cystic mass5. Moreover, a case of simultaneous multiple localizations was also reported [Alveolar adenoma is a very rare and unusual pulmonary neoplasm first described in 1986 by Yousem and Hochhololzer ,4,5 and The cytological preoperative diagnosis is surely difficult and has been never reported; intraoperative frozen section is troublesome too. Majors histological features of alveolar adenoma are a well circumscribed unencapsulated cystic mass composed by cuboidal epithelial cells immunoreactive to TTF-1 antibody without atypia surrounded by a myxoid and collagenous interstitium, in which elongated, plump, cells are usually detectable .The origin and histogenesis of the neoplasm are unknown. This rare neoplasm should entered in differential diagnosis of solitary pulmonary nodule, whether in benign  or in malignant tumors .The case we experienced is unique for its hilar presentation and its isotope uptake on PET scan. This is the first case reported with a positive PET scan considering that, to the authors' knowledge, the only PET scan reported in a clinical case with alveolar adenoma resulted negative . Taking In conclusion, the authors suggest considering alveolar adenoma as possible diagnosis in cases with light PET positive pulmonary nodule.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MN wrote the article and participated in the surgical operation. PM and AP collected data and contributed to the pre- and postoperative patient's management, LR participated in the design of the study, DT participated in the surgical operation and collected data. MB e SF carried out the histopathological diagnosis. LS performed the surgical operation and revised manuscript. SF helped in the draft of the study and revised the manuscript. All the authors approved the final version of the manuscript.