We present a 27‐year‐old woman with an adrenal oncocytoma. This is a very rare entity. We provide a review of the clinical, biochemical and pathological features of cases reported in the literature. Oncocytomas or oncocytic neoplasms are composed of tumor cells that show abundant granular eosinophilic cytoplasm by microscopy, and which may adopt an alveolar, tubular, or solid pattern The organs most commonly involved are the kidney, salivary glands, and thyroid gland. However, cases have been reported in parathyroid glands, pituitary gland, larynx, liver, breast, ovary, stomach, small intestine, thymus, prostate, and lung When located in the retroperitoneum, oncocytic neoplasms may be either renal or adrenal in origin. Limited cases of retroperitoneal heterotopic adrenal oncocytomas have been reported Oncocytic neoplasms arising from the adrenal gland are rare: 1.8% of adrenal masses, predominantly affecting the adult population Since the first description of oncocytomas in 1986, confirmed by electron microscopy According to their biologic behavior, oncocytomas are classified as malignant, borderline , and benign, based on the criteria proposed in 2004 by Bisceglia et al. As oncocytoma is a very rare entity, the aim of this article was to report a case of plurihormonal adrenal oncocytoma and to provide a review of the clinical, biochemical, and pathological features of cases reported in the literature.We present a case report and a systematic literature review of articles published over the 29‐year period between 1986 and 2015. OVID MEDLINE and PubMed were used to identify relevant articles. The key words “oncocytoma,” “adrenal gland,” “adrenal oncocytoma,” “adrenal oncocytic neoplasm,” and “adrenal oncocytic carcinoma” were used. No language restrictions were imposed. Original articles, review articles, and editorials were included and reviewed in order to select relevant articles. A total of 115 articles were identified. Only full‐text publications or abstracts containing a description of relevant clinical, biochemical, and pathological features were included. Six articles were excluded because the full text could not be obtained and the abstracts contained insufficient data. Data of 109 articles were included in the review, comprising 183 cases of oncocytoma. Data were collected on age, gender, form of presentation, symptoms, hormonal hyperfunction, size, weight, biologic behavior, and progress.A twenty‐seven‐year‐old woman with no relevant medical history presented with lower back pain of one‐year duration.She denied any history of medications, smoking, hypertension, headaches, palpitations, excessive perspiration, muscle weakness, bruising easily, fatigue, hematuria, menstrual disorders, acne, or hirsutism.Physical examination was unremarkable; specifically, no masses, lymphadenopathy, peripheral edema, hirsutism, acne, alopecia, cushingoid features, striae, or petechiae were noted. Blood pressure: 110/70 mmHg, with no orthostatic hypotension.An ultrasound scan was performed because of the lower back pain and showed a 46 × 40‐mm hypoechoic solid lesion in the left adrenal gland. A CT scan of the abdomen and pelvis was ordered, which demonstrated a 50 × 42‐mm rounded, solid, homogeneous expansive lesion of distinct borders in the left adrenal gland. The lesion had a spontaneous density of 46 Hounsfield units, showing enhancement after contrast administration Figs  and 2.The patient′s complete blood count, urine analysis, blood urea nitrogen, and creatinine levels were all within normal limits.am fasting, the results were as follows: total testosterone: 0.9 ng/mL , DHEA‐S: 2735.3 ng/mL , androstenedione: 3.3 , 17 hydroxyprogesterone: 3.1 ng/mL , aldosterone: 11 ng/dL : 0.2 ng/mL/h , aldosterone/ARP ratio: 55, sodium: 141 mmol/L , potassium: 3.9 mmol/L , plasma cortisol: 16.2 μg/dL , and ACTH: <10.0 pg/mL . Other measurements were ordered, including salivary cortisol at 23:00 h: 9.3 nmol/L , urinary free cortisol: 73.2 μg/24 h , adrenaline: 4.7 μg/24 h , noradrenaline: 14.2 μg/24 h , and vanillynmandelic acid: 3.8 mg/24 h ; 24–h urinary metanephrines measurement was not available in our country at the time of the patient evaluation.Laboratory tests were ordered during the early follicular phase, and at 8 am were 16.3 μg/dL . Endogenous hypercortisolism of adrenal origin was confirmed, associated with biochemical features consistent with elevated aldosterone/PRA ratio  and hyperandrogenism.Measurement of nocturnal salivary cortisol was repeated: 5.1 nmol/L . Overnight 1 mg dexamethasone suppression test was ordered: Plasma cortisol levels at 8 Because of tumor size and plurihormonal secretion, a decision was made to proceed with surgery, which was performed 10 months after diagnosis. A laparoscopic approach was used, but because of the large tumor size, conversion to open surgery was necessary. Left adrenalectomy was performed. No preoperative therapy was performed because there were no clinical or biochemical data suggesting catecholamine hypersecretion; however, careful intraoperative blood pressure monitoring was performed, which remained stable throughout the procedure. The patient made good progress, receiving postoperative hydrocortisone replacement 20 mg/day for 2 months.Left adrenal gland that weighed 134 g and measured 9.9 × 7.5 × 4.9 cm, with a brownish surface. On cut section, the parenchyma was replaced by a brownish‐yellowish tumor proliferation, with areas of hemorrhage and hard‐elastic consistency. Histological sections revealed tumor proliferation composed of polygonal cells of large hyperchromatic bizarre nuclei with isolated intranuclear vacuoles and prominent nucleoli; abundant eosinophilic cytoplasm. Cells were arranged in nests separated by thin fibrous septae; the tumor mass was surrounded by a thick capsule. The mitotic index was low: 2–3 mitoses per 50 high‐power fields. There was no evidence of vascular embolism or capsular invasion. Little residual adrenal parenchyma was observed at the periphery of the lesion. Ki‐67: 3%.Two months after surgery, hydrocortisone therapy was discontinued and hormonal laboratory measurements were performed, with normal results Table .The patient made good progress, with no evidence of tumor recurrence on the follow‐up computed tomography scans and laboratory tests performed annually during the last 9 years.Among adrenal tumors, oncocytomas are a rare subtype. Most cases reported in the literature are isolated cases, with the largest series being that reported by Mearini et al., with a total of 147 cases We have found 183 case reports of adrenal oncocytomas, from which we could collect clinical, biochemical, and histological data (data are presented as the mean ± SD). These tumors are more frequent in females (2/3) and may occur in all age groups, with a predominant occurrence approximately in the fourth and fifth decades of life (45.1 ± 15.9 years). In agreement with literature reports, about 20% were malignant (22.4%) and about 30% were functional (27.9%). As regards location, previous reviews report a higher frequency on the left side; in this review, 56.2% of tumors occurred on the left side, 38.9% on the right side, 0.6% were bilateral, and 4.3% were heterotopic: retroperitoneum There is consensus that the most important problem regarding adrenal oncocytomas concerns their biologic behavior Table Benign oncocytomas constituted 38.3% of all reported cases and were characterized by an average size of 5 cm. In most cases, these tumors were detected incidentally, while in 18.5% of cases patients presented with abdominal pain Oncocytomas of uncertain malignant potential (borderline) accounted for 39.3% of cases, with a larger size on average (11.6 cm). Most cases were incidental findings, while the rest presented with abdominal or lumbar pain (29.2%), virilization (6%) As regards malignant oncocytomas (22.4%), the average size was 13 cm. In 21.9% of cases, diagnosis was performed by abdominal pain or palpable mass and in 21.9% of cases by excessive hormone secretion symptoms (36.8%). In 10 cases, there was evidence of metastasis As regards imaging methods, in addition to tumor size, the appearance and behavior after contrast administration are important data for the differential diagnosis of adrenal masses. Lipid content, evaluated by CT or magnetic resonance imaging, is the most important parameter for differentiating adrenal adenomas from lesions with malignant behavior, such as adrenal carcinoma or metastases. The majority of adrenal adenomas present attenuation values of less than 10 HU on unenhanced CT, reflecting the abundant intracellular lipid content, while 30% present higher densities and are considered lipid‐poor adenomas. The behavior of lesions after contrast administration allows differentiation of adenomatous lesions from malignant tumors, as the former show a rapid washout of contrast (greater than 50%) on delayed scans, while carcinomas or metastases enhance intensely and retain contrast for longer periods. Khan et al. described a series of benign and malignant adrenal oncocytomas, comparing them with various adrenal carcinomas as to size, values of attenuation on CT scan and behavior of contrast material. Benign oncocytomas were more frequently homogeneous, with no evidence of calcification or hemorrhage. The value of attenuation was greater than 10 HU, which is consistent with the histological feature of poor intracellular lipid content. However, these tumors demonstrated high‐contrast washout percentages, behaving similarly to lipid‐poor adenomas. Oncocytic carcinomas showed similar features to those found in adrenocortical carcinomas, including larger size, heterogeneity, and presence of necrosis. They showed high values of attenuation on unenhanced CT, 39 HU on average, together with a washout percentage <50% after contrast administration Independently of the size and features of adrenal masses, in all cases it is necessary to perform a thorough hormonal assessment to determine whether there are signs of adrenal hyperfunction. Recommended tests include measurement of plasma or urinary fractionated metanephrines, low‐dose dexamethasone suppression test, measurement of ACTH, estradiol, androgens  aldosterone and plasma renin activity. In our review of the literature, approximately one‐third of benign oncocytomas were functional, secreting androgen in six cases As adrenal oncocytomas usually present as masses larger than 5 cm, adrenalectomy is the treatment of choice, generally by the open surgical approach, although recently the use of the laparoscopic technique has been increasingly reported in cases of encapsulated tumors. The laparoscopic approach reduces pain and the perioperative hospital stay; however, the indication of this technique in cases of larger or potentially malignant tumors remains controversial Our patient presented with lumbar pain as the only clinical manifestation; therefore, it was not an incidentaloma. Although the laboratory findings demonstrated a functional tumor, there were no clinical signs of adrenal hyperfunction. The hormonal evaluation showed ACTH‐independent cortisol secretion and androgen secretion, which returned to normal levels in postoperative laboratory tests. Our patient received short‐term hydrocortisone replacement therapy, with no clinical manifestations of adrenal suppression secondary to resection of the cortisol‐secreting tumor. In most studies that evaluated patients with hyperaldosteronism, an aldosterone/PRA ratio >20 is considered highly suspicious for primary aldosteronism. An aldosterone/PRA ratio >30, especially in the setting of plasma aldosterone concentration ≥15 ng/dL, has been shown to be 90% sensitive and 91% specific for the diagnosis In conclusion, adrenal oncocytic tumors are rare entities with a variable biologic behavior, which require a thorough hormonal evaluation for ruling out hyperfunction before surgical resection and for an adequate management in cases of cortisol‐secreting tumors that may cause postoperative contralateral adrenal suppression. The pathologic examination must be performed by experienced pathologists in order to be able to classify tumors according to their biologic potential and to establish the patient's prognosis.PRC: contributed to patient evaluation, design and conduction of research, data acquisition and analysis, and drafting of the manuscript. ALP: helped in design and conduction of research, data acquisition and analysis, and drafting of the manuscript. PK: contributed to analysis of the data, drafting, and critical review of the manuscript.None declared.


Brunner's Gland Adenoma is a very rarely seen benign tumor of duodenum. While it generally leads to obstruction and bleeding complaints, it may very rarely occur by mimicking a pancreatic tumor. A 48 years old male patient admitted to the gastroenterology clinic due to the epigastric pain spreading dorsally. No significant feature is present in his clinical history. A lesion containing cystic solid components in the size of 30x40 mm was detected in the head of pancreas as a result of the abdominal tomography. In the light of these findings, pancreaticoduodenectomy is applied to the patient. It is observed that tumor is in submucosal location and widely invaded the pancreatic head. In the histopathological examination, Brunner's Gland Adenoma is reported in pancreatic head localization. In this manuscript a case of Brunner's gland adenoma diagnosed by performing pancreaticoduodenectomy due to the mass in the head of the pancreas is presented. The Brunner glands were first described by Johan Conrad Brunner, a Swedish anatomist, in 1688. The Brunner gland adenoma (BGA), on the other hand, was first described by Curveilheir in 1835 . The BGAThe 48-year-old male patient applied to the gastroenterology clinic with the epigastric pain complaint. There were no characteristics in his background story. During the physical examination, sensitivity in the epigastric zone was detected. The routine blood test and tumor marker test results were normal. Any pathologies but antral gastritis were not detected as a result of the patient's upper gastrointestinal system endoscopy. A solid lesion which was in the size of 30 x 40 mm was detected in the head of pancreas and loss of fat plane between the pancreas and lesion, which raise doubt of a mass in the pancreas as a result of the abdominal tomography . There wth and the 6th decades and at equal rates with both genders. The findings from the clinical examination of patients may include upper gastrointestinal hemorrhage, obstruction, vomiting, stomachache and diarrhea [The BGA develops from the Brunner glands located in the deep mucosa and the submucosa. Since the Brunner glands are widespread on the proximal duodenum, the BGA usually (in 57% to 70% of the cases) develops from the duodenum medulla . In mostdiarrhea . Since tdiarrhea . In our diarrhea . Franzindiarrhea . Howeverdiarrhea . Anotherdiarrhea . It was diarrhea . HoweverBGA is a benign lesion with a good long term prognosis. It was reported within the literature that it could turn malign even to a rare extent . The BGAWe have presented a case of the BGA that mimicked a tumor located on the head of pancreas. The suspicion of malignancy is very high according to radiological findings. Even though it is very rare condition, its diagnosis and treatment should be planned carefully.The authors declare no competing interests.


A supernumerary ovary is an exceedingly rare disorder, in which the structure containing ovarian tissue is located at some distance from the normally placed ovary. 16 cases of endometriosis or tumors originating in a supernumerary ovary have been published in the English literature, but no case of coexisting endometriosis and a tumor has been published. We present the case of a 40-year-old female with cystic endometrioma with coexisting fibroma originating in a supernumerary ovary in the rectovaginal pouch. The present case is the first to be reported with coexisting endometriosis and a tumor originating in a supernumerary ovary. Our experience with this case and the results of our previous studies of rectovaginal endometriosis indicated that the possibility of originating in a supernumerary ovary shall be examined in cases of cystic endometrioma in the rectovaginal pouch. A supernumerary ovary is one of the rarest conditions of gynecological conditions, in which the structure containing ovarian tissue, such as the primordial follicle, is located at some distance from the normally placed ovary . It occuA 40-year-old 2-multipara female was urgently admitted to Juntendo University Nerima Hospital with a diagnosis of acute abdomen. She had no previous pelvic surgery. Her initial laboratory data revealed a normal range of WBC and CRP and an elevation of CA125 (114 U/mL) and CA19-9 (402 U/mL). We had a diagnosis of the rupture or torsion of the ovarian malignant tumor because of the findings of magnetic resonance imaging revealing the suspicion of hemorrhage within the rectovaginal mass , and an After mass fixation by formalin solution, the size of the cystic portion was 35 × 30 mm and that of the solid portion was 50 × 45 × 30 mm. The solid area showed a gray-colored fibrous appearance . HistoloHistologically, in the cystic area, variously sized cysts with the lining of endometrial tissue in the cyst wall were present Figures  and 5, wBased on the clinical  and histological (coexisting cystic endometrioma and fibroma) findings, the mass was diagnosed as cystic endometrioma with coexisting fibroma originating in a supernumerary ovary in the rectovaginal pouch.This case was approved by the Research Ethics Committee of Juntendo University Nerima Hospital.Cases of lobulated, accessory, and supernumerary ovary are among the rarest conditions of abnormalities. A lobulated ovary is a normally situated ovary divided by one or several fissures into two or more lobes. An accessory ovary attaches to normal ovarian tissue or a ligament of eutopic ovary . A superThe site and disease in the 17 cases (16 previously reported cases and the present case) with a tumor and/or endometriosis originating in a supernumerary ovary are presented in In rectovaginal endometrioma, fibrosis occurred around the endometrial tissues and it extended into fat and connective tissue as well as within the endometrial tissues and finally produced a nodular fibrous mass, while causing clinical symptoms, including obstinate, severe dysmenorrhea, dyspareunia, and chronic pelvic pain , 19. In Concerning the diagnosis of this patient, we initially considered rectovaginal endometriosis with coexisting fibroma. However, cystic endometriosis in the rectovaginal pouch was not found in the 195 specimens of the 63 cases with rectovaginal endometriosis in our previous study . So, we In rectovaginal endometriosis, fibrosis and smooth muscle metaplasia frequently occurred. So, concerning the pathogenesis of the coexisting fibroma in this case, metaplastic theory may be considered. However, fibroma is the most common ovarian stromal tumor , so the In conclusion, we reported the first case of cystic endometriosis with coexisting fibroma originating in a supernumerary ovary in the rectovaginal pouch. When cystic endometriosis is present in the rectovaginal pouch, our experiences with the present case suggest that the possibility of cystic endometriosis originating in a supernumerary ovary shall be examined.


Differentiating demyelinating conditions from neoplastic conditions can pose a significant challenge. There are a number of reports in the literature of large ring-enhancing, space-occupying lesions that were initially considered neoplastic or infectious but, on further review, were deemed demyelinating. Creutzfeldt-Peters cells (CPC) are reactive astrocytes with fragmented nuclear inclusions that are routinely seen in multiple sclerosis (MS) and generally exclude the diagnosis of glioblastoma (GB). The patient is a 78-year-old man with a history of prostate cancer, which was treated with radiation therapy, who presented with altered mental status. A magnetic resonance imaging (MRI) scan of the brain revealed a 4.6 x 3.1 cm mass lesion in the right posterior temporal lobe with minimal mass effect and heterogeneous contrast enhancement. The patient underwent an open biopsy of the mass, which on histology was rich with Creutzfeldt-Peters cells. Frozen histology was unclear and full resection of the mass was delayed. A molecular and immunohistochemical analysis confirmed glioblastoma. The patient returned four weeks later for a subtotal resection of the tumor. The case presented demonstrates an example of a challenging diagnosis. Differentiating between demyelinating and neoplastic conditions is critical since the management and prognosis differ greatly. More importantly, we present a case of glioblastoma rich with Creutzfeldt-Peters cells, which has previously not been reported in the literature. Creutzfeldt-Peters cells (CPC) are reactive astrocytes with fragmented nuclear inclusions. These cells are associated with demyelination and multiple sclerosis. Differentiating between multiple sclerosis and glioblastoma is usually straightforward but can be challenging in certain situations. In clinical practice, it is critical to differentiate between these two pathologies due to the vastly different management of multiple sclerosis (MS) and glioblastoma (GB). Here, we present a case of a GB that was rich with CPC and posed a diagnostic challenge. We present a 78-year-old male with a previous medical history of prostate cancer, which was treated with radiation therapy, who secondarily developed urethral strictures for which he had recently undergone a urethral stricture dilatation. He presented the following day with altered mental status, nausea, and lethargy. He had no previous symptoms of headaches or other neurologic deficits. He was physically active and had otherwise been in excellent health. His family history was positive for pancreatic cancer in his father. He did not smoke or consume alcohol. On admission to the hospital, he was hyponatremic with a sodium level of 120. After fluid resuscitation, his sodium normalized to 136. A computed tomography (CT) scan revealed a mass lesion in his right posterior temporal lobe for which neurosurgery was consulted. His encephalopathy improved and his neurologic exam at the time of consultation only demonstrated a superior quadrantanopsia in his left visual field that was consistent with confrontation testing. He was placed on intravenous steroids, anticonvulsants, and an MRI scan of the brain was performed, which revealed a 4.6 x 3.1 cm mass lesion in the right posterior temporal lobe with minimal mass effect and heterogeneous contrast enhancement , which are identifiable by light microscopy. These cells were first described and illustrated by Creutzfeldt in 1923. He postulated that multiple micronuclei and chromatin clumps were a byproduct of deficient karyokinesis. Peters subsequently described these cells in 1958 in the presence of acute demyelinating encephalomyelitis. The formation of micronuclei can be traced to chromosome breakage and dysfunction of the mitotic apparatus. Micronuclei consist of acentric chromosomes or chromatids or whole chromosomes/chromatids that lag behind in anaphase. The failure of micronuclei to migrate to the poles leads to their exclusion from the daughter cell during telophase [The presence of micronuclei has been reported in a variety of neoplastic and non-neoplastic conditions. They are commonly found in cancerous cells and can serve as a marker of clastogen- or aneugen-induced chromosomal instability . CPC areDifferentiating demyelinating from neoplastic conditions can pose a significant challenge. There are a number of reports in the literature of large ring-enhancing, space-occupying lesions that are initially considered neoplastic or infectious but on further review were deemed demyelinating. Zagzag et al. report 17 cases that appeared to be tumors but were later correctly diagnosed as demyelinating . Masdeu On histology, there are several factors that can lead to confusion between demyelinating and neoplastic conditions. Five such patterns were identified by Zagzag et al., which include hypercellularity, the presence of pleomorphic reactive astrocytes with bizarre nuclei, the presence of mitotic figures, necrosis, and areas of cystic degeneration or cavitation in edematous tissues . Erana-RThe case presented above is an example of a challenging clinical, radiographic, and histologic diagnosis. A circular contrast enhancement pattern on the MRI would support a neoplastic or infectious etiology. Histologically though, CPC and granular mitoses would suggest a demyelinating disease. In the literature, there is one reported case of a GB with CPC present. The patient underwent a stereotactic biopsy, which demonstrated reactive astrocytes, including CPC and lipid-laden macrophages, consistent with MS. Deeper sections from the same initial biopsy block demonstrated a lesion that was a brain-invasive astrocytoma with hypercellularity and frequent mitoses. The patient underwent resection and the specimen was consistent with GB without any sign of demyelination. The authors of this case report postulated that demyelination of the surrounding tissue was a result of the breakdown of the adjacent blood-brain barrier and subsequent inflammation due to an antigen-rich environment . Our casDifferentiating demyelinating and neoplastic conditions can pose a challenge in clinical practice. Clinical, radiographic, and histologic features help guide clinical practitioners to the appropriate diagnosis. Our case illustrates how tried and true guidelines may not always lead to the correct answer.


In clinical practice, broad ligament (BL) tumors are seldom encountered. Paraganglioma of the BL is exceedingly rare entity. Here we present an unusual case of broad ligament paraganglioma, presenting as a cystic mass, in a 50-year-old postmenopausal female. A high degree of suspicion along with detailed immunohistopathological work-up is needed for arriving at an accurate diagnosis. In clinical practice, broad ligament (BL) tumors are seldom encountered . ParaganHere we present an unusual case of broad ligament paraganglioma, presenting as a cystic mass, in a 50-year-old postmenopausal female. A high degree of suspicion along with detailed immunohistopathological work-up is needed for arriving at an accurate diagnosis.A 50-year-old multiparous, postmenopausal female presented to gynecology outpatient department with burning micturition since one month. Physical examination revealed a blood pressure of 140/96 mmHg and a pulse rate of 76/minute. Vaginal examination revealed 8 cm × 8 cm cystic mass in the anterior fornix. Urine examination showed presence of pus cells. Other systemic examination and routine laboratory findings were noncontributory.Pelvic ultrasonography (USG) showed well-defined large thick walled cystic mass measuring 7.6 cm × 6.8 cm in the left adnexa. Few incomplete septa were seen within it. Left ovary was not seen separately. Right ovary appeared to be normal. No free fluid was seen in the abdomen and pelvis. USG findings suggested the possibility of ovarian neoplastic mass. She was taken for exploratory laparotomy. Uterus and both ovaries were atrophic. Cystic mass measuring 8 cm × 6 cm was seen in the left broad ligament. Cyst was excised and sent for frozen section which showed cyst wall lined by sheets of tumor cells having eccentric vesicular nucleus and moderate amount of eosinophilic granular cytoplasm. Frozen section report of sex cord-stromal tumor was given. Complete excision of the lesion was advised for histopathological confirmation. Omental biopsy and peritoneal fluid were also sampled.Gross examination showed presence of multiple flattened bits, all aggregating to 10 cc . The extOn microscopic examination, sheets of tumor cells having vesicular nuclei and moderate amount of eosinophilic granular cytoplasm Figures  and at pIHC was performed with the following panel of antibodies: Pancytokeratin (PanCK) , vimentin , EMA , CD99 , calretinin , inhibin , S-100 (Leica), neuron-specific enolase (NSE) (Dako), chromogranin-A , synaptophysin , desmin , smooth muscle actin (SMA) , and S-100 (Leica). Hormonal status markers like estrogen receptor (ER)  and progesterone receptor (PR)  were also studied. Cell proliferation markers such as Ki-67  and p53  were also included. The tumor cells showed strong cytoplasmic immunoreactivity for vimentin , chromogPheochromocytoma of Adrenal Gland Scales Score (PASS)  was usedBased on histopathological and IHC studies, final diagnosis of paraganglioma of the BL was reached. The sections from omentum and peritoneal fluid cytology were unremarkable. Postoperatively, the patient is disease-free and on follow-up there is no evidence of recurrence for a period of six months.Although secondary involvement of the broad ligament by malignant tumors arising elsewhere in the abdomen is common, primary tumors in this location are rare. Paraganglioma of the BL is exceedingly rare. A Medline search of the English literature revealed only 4 cases of BL paraganglioma documented so far .Paragangliomas are derived from the extra-adrenal paraganglionic system, which is composed of cells from the neural crest which are associated with the autonomous nervous system. They are formed by chromaffin or nonchromaffin neural epithelioid cells, which are related to sympathetic or parasympathetic nerves. They are commonly found in abdomen, pelvis, head, and neck regions. Paragangliomas have rarely been reported in the female genital tract . PossiblParagangliomas have the potential to present as a mass with paroxysmal symptoms such as palpitations, pallor, tremor, headache, and diaphoresis as well as hypertension due to their catecholamine secreting properties, observed in only 25% of the paragangliomas . The curUSG, computerized tomography (CT), and magnetic resonance imaging (MRI) are noninvasive and sensitive techniques in assessing BL tumors. Extra-adrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing soft tissue mass on CT and multiple areas of signal void interspersed with hyperintense foci within tumor mass on magnetic resonance imaging (MRI). An additional advantage of CT and MRI is their ability to evaluate sites of extra-adrenal pheochromocytomas . It mustAlthough radiological modalities help in evaluating broad ligament tumors, immunohistopathology is mandatory in finally clinching the diagnosis and predicting its malignant potential. Careful assessment of routine histology may raise the suspicion of paraganglioma and prompt immunohistochemical evaluation leads to confirmation. Paragangliomas darken after exposure to potassium dichromate due to chromaffin reaction as seen in our case when done retrospectively . AlthougThe following clinicopathological parameters may be taken into account for risk assessment of behavior of paragangliomas: location, size, PASS score , S-100 iSurgery for removal of the lesion remains the primary modality of treatment of paragangliomas. Due to the possibility of neoplasm recurrence and metastasis especially after incomplete surgical excision, patients need periodic checks and long-term follow-up .


We present a rare case of histologically proven neurofibromatosis of the liver, hepatic hilum, retroperitoneum, and mesentery. An adult male who had been diagnosed with neurofibromatosis (NF) type 1 underwent a computed tomography (CT) scan for abdominal pain and vomiting. The CT scan showed a large low-attenuating lesion in the region of porta hepatis which was infiltrating along portal tracts into the liver, encasing the major vessels, and extending into the retroperitoneum and mesentery. Based on the radiological findings, a differential diagnosis of plexiform neurofibroma was given, although sarcomatous transformation could not have been entirely excluded from imaging alone. The tumor was subsequently biopsied, and the histopathological analysis confirmed the diagnosis of neurofibroma. This case highlights the importance and diagnostic dilemmas in the presence of this tumor at atypical locations in this disease spectrum. Neurofibromatosis Type 1 (NF-1), also known as von Recklinghausen’s disease, is a complex multisystemic, autosomal dominant phakomatosis. It is classically distinguished by cutaneous and non-cutaneous manifestations . AbdominA 52-year-old male, who had been diagnosed with NF-1 in his childhood, was referred to our hospital with an acute history of abdominal pain and vomiting for two days. He had a known medical history of hepatitis C; however, all other laboratory test results obtained at admission, including platelets, clotting profile, liver function tests and alpha-fetoprotein levels, were generally within normal limits  was performed to rule out hepatocellular carcinoma based on the history of hepatitis C.The CT scan revealed a large multilobulated, low-attenuating, minimally enhancing mass involving the liver and porta hepatis. The lesion measured approximately 13.5 x 10.8 cm in size at the porta hepatis , also known as von Recklinghausen’s disease, is a complex multisystemic, autosomal dominant phakomatosis caused by mutations of the NF-1 gene located on chromosome 17q11.2. Its estimated prevalence is reported to be one in 3,500 [Plexiform neurofibroma, which is a variant of neurofibroma, is believed to be virtually diagnostic of NF-1. These are benign nerve sheath tumors which tend to grow along the length of a nerve involving the fascicles and branches . The lesRadiological features of plexiform neurofibromas on magnetic resonance imaging (MRI) include nodular T2-hyperintense lesions with central hypointense areas giving rise to a target sign. The lesions are usually low in signal on T1-weighted images. On CT, they appear as multilobulated, fusiform, low-attenuation masses, usually along the path of a major nerve and its branches . A normaAccording to Malagari et al., adjunctive imaging techniques, like CT portography, show both the perivascular involvement and the preservation of normal vessel distribution throughout the lesions. Angiography may also be used to exclude true splanchnic vessel involvement, stenoses, aneurysmal formation, and non-aneurysmal ectasias that are associated with spindle cell infiltration. Although not typically hypervascular, these tumors may illustrate faint vascularity, seen only on arteriography. Asymmetry in the size or attenuation of these lesions, inhomogenous enhancement, or increased vascularity on angiography may suggest malignant transformation . HoweverInternal plexiform neurofibromas are slow growing, usually asymptomatic, and are mostly incidentally discovered on imaging. The current mainstay of treatment is surgery; however, due to the significant infiltration of surrounding tissues and nerves, complete excision always poses a surgical challenge. Differential diagnosis of these lesions includes other infiltrative hypoattenuating tumors, such as lymphomas, sarcomas, atypical cystadenocarcinomas, and mixed epithelial and mesenchymal tumors. Other primary liver neoplasms that are associated with NF-1, such as schwannomas, angiosarcomas, hepatomas, neurofibrosarcomas, and malignant schwannomas, should also be kept in mind -10.In conclusion, we have reported an unusual case of neurofibromatosis of the liver, porta hepatis, retroperitoneum, and mesentery in an adult patient with von Recklinghausen’s disease. These tumors, although rarely encountered at such locations, have distinctive imaging features with normal vessel distribution patterns. Due to their diffuse and infiltrative nature, complete surgical resection is generally not possible in most cases and, therefore, histopathological correlation and regular surveillance are often needed to exclude malignant transformation.


Spinal angiolipomas (SAL) are rare benign tumors, commonly presenting with slow progressive spinal cord compression. There are only about 170 cases identified so far. Magnetic resonance imaging (MRI) has become the modality of choice for SAL. The purpose of this article is to report three cases of SAL and their characteristic MRI features.Three cases of epidual spinal angiolipoma from ethnic Han Chinese patients are presented here, including one lumbar and two thoracic tumors. MRI imaging findings were reviewed.Multiple MRI technology for characterization of SAL provides useful information for differential diagnosis and therapeutic management. Spinal angiolipomas (SAL) are rare benign tumors, accounting for about 0.14–1.2% of all spinal axis tumors and 2–3% of spinal epidural tumors [Here we report 3 cases of epidual spinal angiolipoma, one lumbar and two thoracic SALs, and we evaluated them by employing multiple MRI techniques including diffusion weight imaging (DWI) and dynamic contrast-enhanced (DCE) MRI.A 25-year-old Chinese woman with no significant medical history presented with lower back pain and left lower limb weakness and numbness for 1 year. No remarkable neurological signs were found except left ankle showed oversupination.MRI imaging showed an epidural mass at L3–L4 level. The mass was T2 hyperintense, hypointense in fat suppressed T2 and showed no enhancement in dynamic contrast-enhanced images Fig. .Fig. 1CaThe patient underwent lumbar laminectomy and this revealed a grey yellowish tumor mass with a final pathologic measurement of 2.5 cm × 1.5 cm × 0.2 cm.Pathological examination confirmed an angiolipoma composed of mature adult fat cells and numerous small vascular channels containing red blood cells and fibrin thrombi. Immunohistochemistry showed CK(−), CD31(+), CD34(+), S-100(+), SMA(+), Ki-67(2%+).The patient had an uneventful recovery after the surgery, and remained asymptomatic at 2 month follow-up checkup.A 77-year-old Chinese woman complained numbness of both lower extremities for 2 years, worsened for half year. The patient reported the numbness first started below umbilical level, then progressively ascended to the breast level in the recent half year. She also reported weakness and coldness of both legs, and had difficulty with balance.Physical examination revealed reduced muscle power and tendon reflex in the lower limbs, reduced superficial sensation below breast level,bilateral lower limb Babinski(+), left Pussep(+), deep sensation impaired bilaterally, Romberg(+). The patient was unable to walk in a straight line.MRI scan demonstrated a tumor mass in the epidural space, dorsally extending from T2 to T4. The mass was hypointense in T1 weighted image, hyperintense in both T2 and fat suppressed T2 weighted images. Fat signals can be seen around the edges, showing hyperintense in both T1 and T2 weight images, but reduced signal in fat suppressed T2. DWI showed low signal of tumor but high signal in apparent diffusion coefficient (ADC) maps. Dynamic contrast-enhanced curve showed a rapid rising phase followed by a wave-like phase without obvious decay , S-100(+), HMB45(+), Ki-67(5%+).The patient had an uneventful recovery after the surgery, and regained normal neurological functions after the follow-up period of 10 months.First reported in 1890 by Berenbruch  spinal eThe clinical symptoms of SALs are consistent with other benign, space-occupying spinal lesions that are related to spinal cord/nerve root compression, or infiltration of surrounding tissue. The most common initial symptoms include lower limb numbness or paraesthesia, leg weakness and back pain. It can evolve over a span of months to years, but occasionally sudden onset or worsening of neurological symptoms occurred when there is a rapid increase in tumor size due to intratumoral thrombosis, haemorrhage , 8.Etiology and pathogenesis of spinal angiolipoma remain largely unknown. It was proposed that an insufficiency of blood supply in thoracic spine contributes to the thoracic predominance of SAL . In addiThe first pathological report describing anatomopathological composition of SAL was done by Howard et al. . Under gHistological, SAL is a lesion with two distinct components: mature adipose tissue and proliferating abnormal blood vessels including capillary, sinusoidal, venous or arterial vascular elements . The ratCurrently, MRI is the best imaging modality of choice in the diagnosis of SAL. The common features of SALs in MRI are presented as a spindle shaped lesion with both ends pen-like, usually located in the posterior epidural space extending longitudinally along the spine. The normal dura displayed as a slit-like low signal on T2-weighted imaging between the tumor and the spinal cord, and the subarachnoid space was narrowed and the spinal cord displaced. Occasionally the lesion may extend into the adjacent intervertebral forame with foraminal widening. The MR signal of SAL is comprised of two components: adipose tissue and vascular architecture. The fatty content was hyperintense on both T1- and T2-weighted images, hypointense on fat-suppressed images. The vascular component was hypointense on T1-weighted and hyperintense on T2-weighted, and showed intense enhancement in post-contrast images. Depending on its composite ratio of fat tissue versus vascular component, the lesion displayed as inhomogenous in pre-contrast images. Vascular flow voids sign is rarely seen on the MR images, as there is usually lack of well-developed arterioles inside the tumor.In this case report, the MRI images of case 2 and case 3 demonstrated features of vascular component dominating SAL, as hypointense in T1, hypertintense in T2 and remarkable enhancement post-contrast. This type of SAL can be differentiated from other vascular originated tumors like hemangioma in that the fat signals from SAL are part of the tumor itself while in hemangioma the surrounding fat tissue clearly delineated from the tumor. Vascular void sign can also used to distinguish from arteriovenous malformation which is rarely seen in SALs. However, in this report one strip of vascular void signal was shown in case 2.Case 1 can be easily confused with lipoma as it showed hyperintense in T2, reduced signal in fat suppressed T2, and no contrast enhancement. In this particular case, contrary to previous report by Hu et al.  fat suppDynamic contrast-enhanced MRI (DCE-MRI) was performed to assess the tumor vascular characteristics. The three SAL cases showed distinct shape in the time-intensity curve. No enhancement was observed in case one, while there were initial onsets of wash-in phase observed in both case two and three, followed by a wave-like washout phase in case two, and slow decaying washout phase in case three.Diffusion-weighted imaging (DWI) is now widely appreciated as an indispensable tool in the examination of the central nervous system (CNS), especially for detection of acute ischaemic stroke and for characterization and differentiation of CNS tumors. In this study, all three cases of SAL showed low signal on DWI map, but high signal on ADC diagram, with no obvious diffusion restriction. A large scale of study would be helpful in order to assess the utility of DWI in the diagnosis of SAL.SALs are treated exclusively by surgical removal of the lesion, gross total resection for noninfiltrating SALs and sub-total excision for infiltrating ones. The outcomes of surgery are good and recurrence is unusual in SALs.In summary, we reported three cases of SAL with the emphasis on their MRI features. MRI findings indicate that one case is fat tissue predominant and the other two cases are vascular component predominant. By employing multiple MRI technology, better characterization of SAL can be obtained in regard to the composition and infiltration of the tumor. In spite of the ever-growing acuity of MRI imaging, diagnosis of SAL should be made in combination with clinical, radiological and pathological examination findings.


Pituitary dysfunction in adults are often associated with tumors of the gland and manifests with mass effects and hypopituitarism. MRI of pituitary region often provides confirmation of the diagnosis and assists in planning neurosurgery.A 69 years old female evaluated for chronic headache was found to have a supra-sellar mass lesion that mimicked a pituitary tumor, with biochemical evidence of hypopituitarism. Cerebral angiogram confirmed the diagnosis of an aneurysm of the intracavernous internal carotid artery. She was successfully treated with coil embolization of the aneurysm and achieved resolution of symptoms and return of biochemistries to normal.Carotid aneurysm can mimc pituitary tumours clinically and radiologically on MRI scan. This rare possibility should be considered in evaluating supra-sellar masses to avoid catastrophic consequences. Pituitary tumours are the commonest type of intracranial tumours . Their pIn contrast, intracranial aneurysms are rare and their incidence range from 0.4 to 3.6% according to autopsy studies and from 3.7 to 6.0% according to studies involving patients undergoing cerebral angiography , 6, a raA 69 years old female presented with gradual onset slowly progressive intermittent diffuse headache of moderate to severe intensity for 3 years. No diurnal pattern of symptoms or associated vomiting or visual disturbance was noted. She was clinically euthyroid and denied galactorrhea, postural dizziness, limb weakness or numbness, convulsions or sphincter dysfunction. Her past history was unremarkable.General physical examination was unremarkable with normal vital parameters , normal visual fields on confrontation method and had no papilledema or other focal neurological deficits.Investigations revealed an abnormal thyroid profile suggestive of secondary hypothyroidism . Further investigation showed low cortisol levels (4.03 µg/dL) and markedly raised prolactin level of 1634 mU/L (< 400 mU/L). Thyroxine (75 mcg daily) and hydrocortisone  replacement therapy was commenced and titrated with regular monitoring of free T4 and cortisol levels.Visual fields assessment by perimetry showed a right homonymous inferior quadrantanopia. Non contrast CT scan of the brain showed an old cerebral infarct in the left parietal lobe Fig. . An MRI However on re-evaluation by the neurosurgeon, doubt was cast on the diagnosis of a ‘pituitary tumor’ due to the multilayered and ‘halo’ like appearance of the lesion on MRI Fig. . A CT anThus a potentially catastrophic event was averted which could have occurred had trans-sphenoidal surgery been attempted for a pituitary tumor. It is also noteworthy that old infarct could also have been an embolic consequence of the aneurysm.The patient eventually underwent endosaccular coiling Fig.  and stenCommon presentations of intracranial aneurysms are subarachnoid haemorrhage, cerebral ischaemia or pseudotumoral syndromes .However, intracavernous aneurysms are well known to present with ophthalmoplegia due to compression of third, fourth and sixth cranial nerves as they traverse the cavernous sinus, as well as visual field defects due to chiasmal compression , 10. AccRarely, an intracavernous aneurysm can present as hypothalamo-pituitary axis dysfunction when it extends into the sellar region , 12. SucIntracavernous sinus aneurysms are thought to have a benign course . HoweverDifferentiation between pituitary adenoma and an aneurysm is vital as these two have different management options and mistakenly attempting trans-sphenoidal resection of an aneurysm could have disastrous consequences. Halo appearance produced by the two dural layers encasing the cavernous portion of carotid artery should raise the suspicion of this diagnosis. Filling defects within the mass represent thrombosis. In contrast, a pituitary tumour would appear isointense to grey matter on T1 and T2 weighted MR imaging and would enhance with gadolinium contrast. Large tumours may show heterogeneity and erosion of clinoid processes. Probable higher risk of having an intracranial aneurysm along with the pituitary tumour should also be borne in mind. Infact several authors have reported on subarachnoid haemorrhage during transphenoidal surgery for pituitary adenomas, due to accidental damage to previously undetected intracranial aneurysms –17. SomeIn summary, this case report illustrates the rare possibility of misidentifying an intracavernous carotid aneurysm as a pituitary tumour. Therefore we emphasize the need of very careful evaluation of the MRI scan before proceeding to pituitary surgery, particularly observing for indirect evidence of an aneurysm such as multi layered halo like appearance, presence of intralesional filling defects and whenever a doubt exists, to perform angiogram to confirm the presence of an aneurysm.


Primary cardiac sarcomas are rare clinical entities with an incidence rate of 0.0001% in collected autopsy series and are regarded as very aggressive tumors. We herein describe a 21-year-old woman presenting with syncope, dyspnea, and abdominal distention. She suffered from massive ascites, plural effusion, and liver congestion demonstrated by abdominal sonography and chest X-ray. Transthoracic echocardiography revealed a heterogeneous solid mass located in the right atrium; therefore, the patient underwent radical surgical excision of the tumor and 3 cycles of adjuvant chemotherapy. Fifteen months after surgery, she was having a favorable life quality without any evidence of recurrence. Standard surgery, adjuvant chemotherapy, and radiotherapy have been consistently unsuccessful.3 These sarcomas constitute the main malignant primary heart tumors with higher prevalence in men between the third and fourth decades of life and are predominantly located in the right atrium. As benign lesions, the clinical presentation of malignant cardiac tumors also depends on the location and not their histological type. 4Primary cardiac sarcomas are rare clinical entities with an incidence rate of 0.0001% in collected autopsy series and are considered as very aggressive tumors and rare malignancies, comprising approximately 5% of cardiac sarcomas and 1% of all primary cardiac tumors.A 21-year-old woman presented with dyspnea and weakness. The symptoms were initially managed as the common cold. However, with the progression of the symptoms and addition of abdominal distention and syncope to the condition, she underwent abdominal sonography, which showed massive ascites, pleural effusion, and liver congestion. Chest X-ray confirmed bilateral pleural effusion, but there no evidence of abnormality in limb X-ray. Transthoracic echocardiography (TTE) revealed a large heterogeneous solid mass, measuring 7.5 × 5.5 × 3.2 cm, located in the right atrium and protruding into the pulmonary artery causing pulmonary artery obstruction. A metastatic workup, including computed tomography (CT) scan of the chest, abdomen, and pelvis, as well as a laboratory workup disclosed no abnormalities. ECG illustrated nonspecific repolarization changes. Therefore, the patient underwent surgical excision of the tumor. A huge mass was detected in the right atrium, on the posterior wall, involving the atrioventricular node and the posterior leaflet of the tricuspid valve and protruding into the right ventricular outflow tract. After the radical excision of the tumor, tricuspid valve replacement was done using a biological St. Jude valve (No. 21), and a permanent epicardial pacemaker was established because complete heart block had emerged after radical surgery . The patIn the pathological examination, a malignant spindle cell tumor was seen. Desmin and myogenin were negative in the tumoral cells, whereas epithelial membrane antigen, creatine kinase, and vimentin were strongly positive in them . MoreoveThe patient underwent 3 cycles of adjuvant chemotherapy following radical surgery. At 15 months’ follow-up, she was fortunately having a favorable life quality, and there was no evidence of local or metastatic recurrence in serial TTE and thoracoabdominal CT scan.3 Their occurrence in the myocardium is deemed a rare entity. There is a male preponderance of 2.5:1. The right side of the heart is the most frequent location for cardiac synovial sarcomas, but these tumors can occur in the left side of the heart and in the pericardium as well.5Synovial sarcomas usually occur in the soft tissues of the extremities.6 In 33% of the patients, complete surgical resection is possible; however, in those with apparent complete excision, high recurrence rates have been reported.7 Treatment is usually palliative resection, followed by chemotherapy with or without radiation.8 Bakeen FG et al.9 reported the results of the surgical treatment of a series of 27 patients with a median survival of 24 months, and even better results with multi-modality treatment options. Hannachi Sassiet et al.8 reported a possible exception to this poor prognosis in the case of a 45-year-old man with a pedunculated right atrial mass, who had complete surgical resection and was reported to be alive for 5 years. In our patient, radical surgery and adjuvant chemotherapy was done, and she was still alive at 15 months' postoperative follow-up without evidence of recurrence. Hopefully, radical surgery combined with chemotherapy and radiotherapy would improve survival in patients with cardiac sarcomas.Patients with primary synovial sarcomas usually present with dyspnea, chest pain, and evidence of congestive heart failure. These tumors often cause death through widespread infiltration into the myocardium or obstruction of the flow within the heart.Ethical standard: There is no conflict of interests regarding the publication of the present article by any of the writers.Due to the rarity of cardiac synovial sarcomas, there are no large studies comparing different therapeutic approaches. Our current case provides further support for the role of radical surgery combined with chemotherapy and radiotherapy.


Extracranial metastasis of malignant meningioma to soft tissues is extremely rare and its clinical, radiological and pathological features are not well-characterised.We report a case of a 58 year old man who presented with a mobile mass within the left trapezius muscle. The patient had previously undergone surgery for a right frontal lobe high grade anaplastic meningioma. Histology of the soft tissue lesion showed metastatic anaplastic meningioma with clumps of pleomorphic tumour cells which expressed epithelial membrane antigen, cytokeratin and P63 but were negative for other epithelial and mesenchymal markers. A PET-CT scan revealed additional metastatic lesions in the left pleura, liver and iliac bone.Metastatic malignant meningioma can very rarely present as a high grade pleomorphic malignant soft tissue tumour and needs to be distinguished from soft tissue sarcomas and metastatic carcinomas that express epithelial antigens. Meningiomas are relatively common primary intracranial tumours that arise within the leptomeninges or dura mater; these tumours exhibit morphological and immunophenotypic evidence of origin from meningothelial cells which are present in the arachnoid membrane and arachnoid villi associated with the intradural venous sinuses and their tributaries . The majMeningiomas show considerable morphologic variation and are most commonly benign tumours (Grade I) , 3. AtypThe most frequently reported sites of extracranial metastasis of malignant meningioma are lung, spine and liver –13. TherA 55 year old man was admitted to hospital with a head injury that resulted in hearing loss on the left side with pulsatile tinnitus and a vague feeling of disorientation. A CT scan showed an irregular extra-axial solitary lesion lying adjacent to the falx on the right of the midline measuring 3 × 4 × 6 cm with significant mass-effect and peritumoral oedema involving most of the right frontal lobe. There was evidence of underlying bone remodelling with vascular dural supply and heterogeneous enhancement after contrast administration with a deep cystic component. There were fringes of tumour interdigitating with the brain substance indicating probable brain invasion Fig. . The finA year later, the patient experienced an episode of seizures and a CT scan revealed three new lesions at the site of the previously excised meningioma with extensive perilesional oedema. An MRI confirmed the CT findings indicative of tumour and also detected invasion and occlusion of the anterior part of the superior sagittal sinus Fig. . PartialThe patient completed a course of radiotherapy and, nine months later, a mobile soft tissue lump on his back was noted. A thoracic CT scan revealed a soft tissue lesion superficially located in the inferior portion of the left trapezius muscle. The lesion was lobulated, had a low attenuation centre, showed peripheral enhancement and did not exhibit evidence of matrix calcification Fig. . The lesThe lesion was biopsied and showed a largely lobulated tumour containing collections of tumour cells, many of which showed similar features to those seen in the frontal lobe tumour. There were a few whorled collections of tumour cells with round or oval vesicular nuclei Fig. . There wA wide local excision of the lesion, which measured 5 × 3 × 2 cm, was performed; this included a cuff of normal muscle around the lesion and a 1 cm margin of skin around the previous biopsy scar. Histological findings in the resection specimen were similar to those of the biopsy. Further follow up of the patient by CT scan showed no recurrence of the soft tissue lesion but progression of disease in the brain.Extracranial metastasis of meningioma occurs rarely with only a few case reports documenting deep soft tissue as a site of metastasis , 14–17; A recent review of the literature on all reports of distant metastasis of intracranial meningiomas found that in 115 patients with 164 metastatic lesions, 83.9% were Grade I (benign), 20.0% were Grade II  and 40% Grade III (anaplastic) meningiomas [Radiological features of the chest wall tumour in our case were not specific; the diagnosis of a metastatic tumour was supported radiologically by the interval increase in the size of the lesion, as well as high FDG uptake and the finding of lesions at other sites on the PET-CT scan. Multiple deposits were also observed in other extracranial sites in previous reports of soft tissue metastasis from malignant meningioma , 4. The Histological features of the chest wall tumour showed that it was focally necrotic and contained collections of tumour cells with elongated cytoplasm and round or oval vesicular nuclei; there was prominent nuclear pleomorphism and increased mitotic activity. Morphologically, the differential diagnosis was wide and included sarcoma, lymphoma, metastatic melanoma and carcinoma. Immunohistochemistry showed expression of epithelial markers on tumour cells with diffuse, strong staining for EMA and focal staining for cytokeratin. In the absence of any previous history, and taking into account other immunohistochemical findings, these features were thought to favour metastatic carcinoma or a sarcoma expressing epithelial markers. Several sarcomas are known to express epithelial markers, including synovial sarcoma, epithelioid sarcoma, epithelioid malignant peripheral nerve sheath tumour, myoepithelioma and leiomyosarcoma  MorpholoKnowledge of the clinical and radiological information was essential to establish the diagnosis of metastatic malignant meningioma in our case, particularly the previous history of anaplastic meningioma and the radiological identification of FDG avid lesions at several sites. Although very rare, the possibility of metastatic meningioma should be considered in the differential diagnosis of a malignant soft tissue tumour, where tumour cells strongly express EMA and other epithelial markers.


N-acetyl aspartate peaks compared with the peaks on the initial MRS, indicating a high-grade glioma. Subtotal resection was performed for the CPA lesion. Histopathologic examination showed discrepant features of different parts of the CPA lesion. The patient received no further chemotherapy or radiotherapy and died 2 months after surgery. The multifocal and exophytic features of this case and the heterogeneous manifestations on neurological images were rare and confusing for both diagnosis and surgical decision-making. Our case report may contribute knowledge and helpful guidance for other medical doctors.Multifocal pontine glioblastoma exhibiting an exophytic growth pattern in the cerebello-pontine angle (CPA) is rare. We present a case of a 5-year-old girl with consecutive neurological imaging and other clinical findings indicating progressive multifocal exophytic pontine glioblastoma. Three lesions were reported, of which two were initially presented, and one was developed 2 months later. One lesion demonstrated a progressing exophytic extension in the cistern of the left side of the CPA. The other two lesions were located and confined within the pons. Initial magnetic resonance imaging and positron emission tomography–computed tomography indicated low-grade glioma or inflammatory disease. However, 2 and 3 months later, subsequent magnetic resonance spectroscopy (MRS) displayed elevated choline and depressed  Although brainstem tumors account for 10%–20% of cerebral neoplasms in children and 90% of them are gliomas –6. BecauN-acetyl aspartate (NAA).Here, we report a special case of multifocal glioblastoma derived from the pons, exhibiting an exophytic growth pattern, in a 5-year-old girl. In total, three lesions were revealed in the posterior fossa. Repeated magnetic resonance imaging (MRI) showed the progression of the lesions in the left side of the CPA and the upper left side of the pons as well as the appearance of a new lesion in the right side of the pons. Furthermore, repeated magnetic resonance spectroscopy (MRS) over 3 months revealed a gradually elevated ratio of choline (Cho) to The 5-year-old girl was admitted to the Neurosurgery Department of Guangzhou First People’s Hospital, Guangzhou, China on October 6, 2015 and presented with a 2 month history of a medial gaze of the left eye. She did not complain of headache, dizziness, dysphagia, or vomiting. The outpatient medical record revealed that the patient frequently had fevers and coughs since the age of three. The last fever and cough were reported 1 week before the admission. The patient showed age-appropriate physical development. Cardiopulmonary auscultation results were normal. A neurological examination revealed left cranial VI nerve palsy without involvement of other nerves such as the ocular motor , trigeminal, facial, and acoustic nerves. No cerebellar signs or hemiparesis were present. The patient showed no signs of ataxia or long tract dysfunction.N-acetyl aspartate (NAA) and choline (Cho)  was performed 2 days after the admission, demonstrating two well-defined, ring-enhanced lesions at the left side of the CPA  Fig. C. The Chho) Fig. D.Fig. 1IThe patient showed weak expression of serum antibodies for tubercle bacillus (TB). The results of sputum smear examination and blood interferon-gamma release assay for the detection of TB infection were negative. Serum levels of tumor markers  were within normal ranges, except for a slight increase in the level of neuron-specific enolase. The cerebrospinal fluid was extracted via lumbar puncture and tested for TB-DNA and TB-RNA, yielding negative results. After consultation with a tuberculosis specialist, the current evidence for intracranial tuberculoma was deemed insufficient. We proposed a biopsy of the left CPA lesion, but the patient’s parents refused and demanded discharge.Two months later, the patient underwent another MRI. No involvement of additional nerves was observed, except for the cranial VI palsy. However, progression of the disease was demonstrated: (1) the left CPA lesion was enlarged and extended along the cistern of CPA Fig. C. A peakOne month later, the patient was admitted again because of malnutrition and weakness with frequent vomiting. Re-examination with MRI demonstrated progression of all three lesions, especially the CPA lesion, which progressed and expanded to the anterior CPA cistern Fig. D, E. MRSHistopathologic diagnosis and safe resection of the lesion were the goals of the suggested surgery. Under these circumstances, the CPA lesion was the target of the operation, whereas the other two lesions were left untouched. The surgery was conducted using a suboccipital retrosigmoid approach. After occipital craniotomy and dura opening, the cerebellum was cautiously separated from the CPA, and the cerebrospinal fluid was slowly released and suctioned. The arachnoid of the CPA was opened, and a yellowish nodule-like mass was encountered with a clear boundary to the cerebellum Fig. a. The veThe final pathologic diagnosis was glioblastoma with diffuse and strong expression of glial fibrillary acid protein  lymphomas, and inflammatory disease were considered in the differential diagnosis. It is notable that the patient presented with frequent fever and cough and had weak positivity for blood TB antibodies. Infectious diseases, such as tuberculosis, were considered. Although previous articles mentioned that ring-like enhancement and multiple lesions are indications of posterior fossa glioblastoma , these nImmunohistochemical examination showed diffuse expression of GFAP, P53, and S-100 in our case. The expression of S-100 is not stable in glioma, whereas GFAP is a qualitative diagnostic marker of glioma . The comThe progressive growth pattern of the CPA lesion is a confusing characteristic of this case, especially given the coexistence of multiple lesions. We searched PubMed and Google Scholar and found only two English-language articles reporting the coexistence of multiple lesions and a CPA lesion , 14. AccAccording to the imaging and histopathologic evidence, this case of pontine glioblastoma can be categorized as a brainstem glioma. However, the classification of brainstem gliomas is not uniform. Choux et al.  classifiThe changes in the MRS results of our case merit attention. It was reported that the Cho:NAA ratio calculated according to MRS data can be used to monitor metabolism and glioma behavior . In pediGlioblastomas are divided into two types of genesis patterns: primary glioblastoma, also termed de novo glioblastoma, which is defined as a fully developed tumor with no clinical, radiological, or histopathologic evidence of relative benign precursor lesion –34, and Immunohistochemical examination of our case demonstrated a high positive rate of P53 (80%–90%), which is a frequently expressed marker of DIPG . It was Multiple gadolinium-enhanced lesions are rarely seen in brainstem gliomas –49. MultDiffuse pontine glioma is the most common type of brainstem tumor and carries the poorest prognosis in children . Early sIn summary, we presented a rare case of progressive, exophytic, multifocal pontine glioblastoma in a 5-year-old girl. A preoperative differential diagnosis was difficult because of the atypical neurological imaging features and frequent fever and cough symptoms. The clinical evidence supported a secondary glioblastoma. Regretfully, an early histopathologic diagnosis was not achieved.


Stereotactic body radiation therapy (SBRT) is an option for selected patients with metastatic disease. However, sometimes these lesions are located in such close proximity to critical normal structures that the use of safe tumoricidal SBRT doses is not achievable. Here we present a case in which real-time imaging and tracking with a magnetic resonance linear accelerator (MR-LINAC) provided a novel treatment approach and enabled safe treatment of the tumor using SBRT. Our case is a 69-year-old female who presented with localized recurrent small cell lung cancer with a retroperitoneal (FDG-avid) soft tissue lesion measuring 2.4 x 4.1 cm that was causing pain and right hydronephrosis. A Food and Drug Administration (FDA)-approved MR-LINAC system was utilized for planning and the delivery of 21 Gy in three fractions to the retroperitoneal lesion planning target volume (PTV), limited by the neighboring small bowel tolerance. The gross tumor volume (GTV) itself received 27 Gy (9 Gy per fraction). Simulation was performed using a volumetric MR imaging study in treatment position co-registered to a 4D-computed tomography (CT) image set for contouring of the target and organs at risk (OAR). Treatment planning was performed using the primary CT dataset. We developed a reasonable SBRT treatment plan to deliver the prescribed dose without exceeding tolerance doses to the right kidney, the small bowel and all other OAR’s. Real-time MR imaging and tracking during treatment delivery enabled assessment of respiratory-induced target movement in relation to the small bowel and kidney. Gating was performed to halt treatment when PTV movement exceeded the 2-mm range as specified by the treating physician. The treatment course was concluded successfully. The patient denied any acute gastrointestinal or genitourinary toxicity. The pain was significantly improved within a short time following treatment. Follow-up CT showed a near complete response of the mass with total restoration of renal functions, allowing the ureteric stent to be removed. This response has been maintained for five months till the last follow-up. In conclusion, MR-guided planning and delivery using real-time MR imaging and tracking facilitated the treatment of the retroperitoneal mass accurately and efficiently with excellent clinical and radiological response and minimal to no toxicity. We would not discern it safe to treat this mass utilizing SBRT without this ability to accurately visualize the tumor boundary using magnetic resonance imaging (MRI), and offer tracking of the target within the millimeter of surrounding critical OAR’s. Small cell lung cancer (SCLC) represents approximately 14% of newly diagnosed lung cancer patients with 29,540 new cases expected in 2017 . AlthougNovel immunotherapeutic agents have been incorporated in the options for relapsed SCLC after the results of the CheckMate 032 clinical trial that showed improved response rates and one-year overall survival of 30 and 42% for nivolumab with or without ipilimumab, respectively . HoweverSBRT provides a very useful tool to deliver higher doses in a short duration of time for this group of patients with limited survival and possible comorbidities . Deliver2; D1) and etoposide  which concluded 150 days after diagnosis with tolerable acute toxicity. Follow-up PET-CT and brain MRI showed CT partial response for the right paratracheal lesions that were totally PET-lucent with complete resolution of all other lesions including the brain metastases.A 69-year-old life-long non-smoker female with a non-significant past medical history presented with progressive hemoptysis and a painless lump in the neck. Core needle biopsy of this lump revealed the diagnosis of SCLC and a positron emission tomography (PET)-CT and brain MRI were performed for staging. Staging workup demonstrated two FDG-avid right neck masses (2.2 x 2.1 and 1.1 x 1 cm), a 3.8 x 3.3 cm right paratracheal lesion associated with enlarged bilateral hilar lymph nodes (LN) as well as pre-carinal and subcarinal enlarged LN. MRI brain showed two metastatic foci in both cerebellar hemispheres >1 cm and the bone scan revealed a questionable metastatic focus. Accordingly, the case was deemed metastatic ES-SCLC and received whole brain RT and six cycles of first-line chemotherapy: cisplatin  = 6.1), a left hilar mass (max SUV = 5.2) and an enlarged right cervical LN (max SUV = 4.1) consistent with recurrent metastatic disease. At that point, RT was prescribed to areas of increased PET-CT activity and the patient received 50 Gy over 20 fractions for lung and neck lesions using intensity-modulated RT (IMRT) with Radiation Therapy Oncology Group (RTOG) grade 1 mucositis and lung toxicity. A near complete response was maintained for 4.5 months after finishing RT as per follow-up imaging.Four months after completion of RT, PET-CT demonstrated resolution of all irradiated regions with the emergence of new metastatic lesions in the left supraclavicular region (max SUV = 5.4) and right femur (max SUV = 10.6). In addition, the PET-CT showed a new 2.8 X 4.1 cm right retroperitoneal mass with highly intense FDG uptake (max SUV = 10.2) as shown in Figure This mass was painful and was associated with right hydroureteronephrosis, which contributed to deteriorated renal functions with creatinine (1.29 mg/dl), nearly double the patient’s baseline (0.71 mg/dl). This condition mandated ureteric stent placement to relieve the obstruction. Brain MRI was negative for disease progression. The decision of the multidisciplinary tumor board was taken to proceed with palliative RT for the painful femoral lesion, and subsequent SBRT to the retroperitoneal focal lesion to be followed by systemic therapy as the patient at that time was still having a performance status of 1 based on Eastern Cooperative Oncology Group (ECOG) scale. The femoral bone lesion received 30 Gy in 10 fractions.Options for the management of the retroperitoneal mass lesion were complicated by its close proximity to the right kidney that was already having hydroureteronephrosis, as well as to the small bowel. Moreover, the respiratory-induced motion of this mass was significant. Given the patient’s co-morbidities, soft tissue lesion, and proximity of the lesion to several critical organs, we surmised that the MRI-linear accelerator (MR-LINAC) system, incorporating MRI-based imaging, and real-time tumor tracking offered the assurance of being able to deliver hypofractionated doses to this patient safely. The MR LINAC system used was the first MR-LINAC system to receive 510(k) clearance, K162393, from the US Food and Drug Administration (FDA) and has been in clinical use at our institution since July 2017. The multi-leaf collimator (MLC) constitutes a novel double-focused and double-stacked design, with banks offset by half-a-leaf width, to improve spatial resolution considerably. The 0.35 T on-board MRI scanner enables fast real-time monitoring (4 frames/second) of the tumor target, and the 6 MV, flattening filter free LINAC is equipped with a standard dose rate of 650 MU/min for efficient delivery of hypofractionated doses.During CT-simulation, a 10-phase 4D-CT and end-exhale CT scans were acquired. The patient then underwent MR-simulation in the immobilized treatment position on the MR-LINAC. Due to lesion excursion (~1 cm in the superior/inferior direction) and proximity to the kidney and small bowel, it was determined that end-exhale breath-hold phase would be used for treatment planning, as this yielded the greatest separation between the lesion and the small bowel. Contours for gross tumor volume (GTV), planning target volume (PTV) and organs at risk were delineated and a nine-field step and shoot IMRT plan were used to deliver 27 Gy in three fractions to a simultaneous integrated boost volume receiving 21 Gy as shown Figure To deliver ~2500 MU, the total beam-on time was 4.2 minutes per fraction (~2500 MU’s), and the overall delivery time of 17 minutes. A 2-mm isotropic tracking boundary around the target was used with a 5% region of interest overlap allowed during tracking. A summary of one treatment session depicting this process is shown in Video Audio coaching was implemented to guide the patient’s breath holding.After completion of RT, the patient received nivolumab combined with ipilimumab until the last follow-up. The patient denied any post-SBRT acute gastrointestinal (GI) or genitourinary (GU) toxicity in follow-up. Follow-up CT performed two and four months after the receipt of MR-guided SBRT demonstrated an interval complete resolution for the left retroperitoneal mass as well as the other lesions as shown in Figure Renal functions returned to normal, the patient had her ureteric stent removed and she is still in a good performance status (ECOG-1) with minimal use of analgesics as per her last clinical encounter.Despite poor prognosis that is usually associated with ES-SCLC, this case report showed promising outcomes with the integrated use of RT, chemotherapy and immunotherapy for this patient with ES-SCLC. SBRT to the retroperitoneal mass administered after the second relapse was concluded in a markedly short time using only three fractions of MR-guided RT compared to classical fractionation using conventional techniques. This treatment achieved a rapid near complete response and allowed the initiation of nivolumab and ipilimumab within less than one month taking in consideration the lack of evidence for concomitant administration of RT with immunotherapy . This reThis case highlights the importance of real-time tracking and imaging of the soft tissue during the delivery of RT beam by MRI on table. The MR-guided technology here represents an important advancement for the treatment of these lesions close to critical organs and those that move with respiration . PatientAt the same time, more validation of this novel approach is warranted to improve patient localization time and treatment time. Other potential challenges include those related to planning using both MRI and CT datasets with possible disparities and relatively longer planning time. Good patient selection and accurate simulation and preparation of the patient will help in utilizing MR-guided treatment for those who are expected to attain optimal benefit of this approach. Over time, development of a learning curve and the results of randomized trials will formulate guidelines for utilizing the MR-guided RT with real-time tracking and gating.MR-guided radiation therapy with real-time tracking and gating appeared to help our patient to achieve a rapid near complete response of her retroperitoneal mass lesion with minimal to no toxicity. This great response allowed her to initiate immunotherapy in a relatively short time after the second relapse and remain in a remission with excellent quality of life until the last follow-up.


This case is quite rare, and the surgery resulted in a desirable outcome. The patient has been disease-free for 5 years since the surgery.An 82-year-old woman with common bile duct (CBD) dilatation observed during routine ultrasonography was referred to our hospital. Preliminary blood tests revealed elevated levels of hepatobiliary enzymes. Computed tomography (CT) scan showed lower bile duct wall thickening and enhancement. Esophagogastroduodenoscopy revealed mildly swollen papilla of Vater, without ulceration. Endoscopic retrograde cholangiography demonstrated that the CBD was grossly dilated with a constriction in the lower part. The final diagnosis indicated poorly differentiated adenocarcinoma of duodenal papilla with signet-ring cells; pT3N0M0,  Most duodenal papillary carcinomas (DPCs) are well differentiated . Poorly An 82-year-old woman with common bile duct (CBD) dilatation, as observed during routine ultrasonography, was referred to our hospital. She was asymptomatic. Laboratory test results were aspartate aminotransferase level, 278 IU/L; alanine aminotransferase level, 184 IU/L; alkaline phosphatase level, 1877 IU/L; total bilirubin level, 0.42 mg/dL; amylase level, 47 IU/L; and presence of routine inflammatory markers. Laboratory tumor marker levels, carcinoembryonic antigen, and carbohydrate antigen 19–9 levels were normal.Esophagogastroduodenoscopy (EGD) revealed mildly swollen papilla of Vater, without any mucosal erosion Fig. .Fig. 1EGDynamic computed tomography (CT) showed bile duct wall thickening that was enhanced in the lower part of the CBD Fig. . EndoscoDPC is a rare clinical entity, occurring in less than 6 per million people annually. It represents 0.2% of all gastrointestinal cancers and accounts for only 6% of all cancers developing in the periampullary region . The JapThe 5-year survival rate in patients with DPC after radical resection is 30–68% –19. In mIn our case, although the tumor consisted of poorly differentiated adenocarcinoma with signet-ring cells and had infiltrated into the pancreatic parenchyma pT3, both of which indicating poor prognosis, the outcome was desirable. She was diagnosed with DPC incidentally in the health checkup before having jaundice; therefore, the ratio of signet-ring cells was relatively low. Similar reports are very few for DPC .It is well known that surgical resection is the only curative treatment for DPC. No evidence-based chemotherapy regimens exist either for the treatment of unresectable cancers or for postoperative adjuvant therapy; therefore, we simply followed up the patient. Fortunately, the patient is doing well, without any signs of tumor recurrence since the last 5 years.Here, we have presented favorable results in a case of poorly differentiated DPC with signet-ring cells. It is however important to study some more cases with similar outcomes to establish its characteristics.


Missing of mandibular second premolar is one of the most common types of tooth agenesis. In such cases, maintenance of the primary second molar, if possible at all, can prevent many treatment procedures in future. The present case report represents the endodontic management of a necrotic left mandibular primary second molar that had developed an abscess. Considering the missing of the permanent successor, the tooth was disinfected during endodontic preparation and the root canal system was filled with calcium-enriched mixture (CEM) cement in the same session. After 12 months of regular follow-up, not only the tooth was functional and symptom-free, but also healing of the inter-radicular bone lesion and re-establishment of the lamina dura was indicative of treatment success. Further trials are suggested to confirm CEM biomaterial use for management of infected primary molars associated with endodontic lesion. Hypodontia, missing of one to six teeth, is one of the most common dental developmental anomalies in human . A systeFirst, extraction of the primary second molar (PSM) to enable the mesial drifting of the permanent first molar [second approach is to maintain the PSM as long as possible to avoid the potential complexity of inclination-free space closure and possibility of future periodontal issues [in situ is favorable [For management of infected primary molars, in cases with agenesis, selection of a beneficial treatment plan with the best results over the long term brings up a challenge ahead of the clinician. Two different approaches are possible. st molar  which isst molar . The secl issues . The decl issues ; most rel issues . It may l issues . The oveavorable . Studiesavorable .Candida albicans has been proved to have close proximity to MTA; they both induce complete death of fungal cells after 24 h [When extensive caries affects the dental pulp of primary molars, vital pulp therapy becomes necessary; however, if pulpal inflammation and infection extends to the root canal, then a pulpectomy procedure is indicated . The treter 24 h . The alkter 24 h , 15.This report represents the one-year successful treatment outcome of a retained necrotic PSM without permanent successor that had developed an endodontic abscess in the inter-radicular area. The tooth chamber and root canals were sealed/filled with CEM cement.A 12-year old girl came to a private dental clinic complaining of pain and discomfort in the left mandibular area. Her parents did not mention any contributory medical issue. Upon clinical examination, a localized swelling in the PSM area was detected that was tender on palpation. The PSM had a large amalgam filling. A parallel diagnostic radiography was representative of a previous pulpotomy treatment that according to the patient’s parents was done almost 2 years before. The noteworthy issue was the missing of the permanent second premolar. Inter-radicular lucency extending to the periradicular area was indicative of the treatment failure . Tooth extraction carried the risk of mesial drifting of the permanent first molar. The situation was discussed with patient’s parents and the final decision was made. Treatment plan included endodontic treatment of the necrotic tooth. Should the problem continue, the alternative approach would be tooth extraction. After administration of local anesthesia through infiltration of lidocaine with 1:80000 epinephrine, a small incision was made on the most prominent surface of the localized swelling to enable drainage of the puss. Then the amalgam filling was removed with a high speed handpiece. The underlying zinc-oxide eugenol was also removed that allowed drainage through the canals. Four root canals  were detected. The root canals were irrigated with 5.25% NaOCl complemented by slight agitation of the solution with #20 and 30 H-files . This process continued for almost 15 min and the irrigation solution was refreshed every 5 min. Then the canals were dried with paper points .CEM cement liquid and powder  were mixed according to the manufacturer’s instructions. The paste was carried into the canals, using a carrier gun and packed with hand pluggers  and paper points. Then the tooth was restored with amalgam in the very same session. After radiographic checking , chlorheThe patient was put on a regular scheduled follow-up. Her pain totally vanished the day after. After six months, the tooth was asymptomatic and radiographically checked . The forThis report discussed the successful endodontic treatment of a necrotic PSM associated with an apical abscess by sealing/filling the root canals and pulp chamber floor with CEM cement. After one year, the tooth was functional and the inter-radicular bone lesion had healed.In the presented case, the perfect sealing/filling of the chamber floor and root canal walls by CEM cement was a leading factor in healing of the endodontic lesion. CEM cement is a differently-formulated biomaterial that offers the combination of superior biocompatibility of MTA with appropriate setting time (<1 h), profound handling and reasonable price , 17-19. Pseudomonas aeruginosa, Enterococcus faecalis, Staphylococcus aureus and Escherichiacoli were almost similar and both superior to that of MTA [Candida albicans [In addition, it is noteworthy that treatment of the present case was finished during a single visit without prescription of systemic/local antibiotics. The antimicrobial activity of the root canal filling material played an undeniable role in healing of the apical abscess. The lethal effect of calcium hydroxide and CEM cement on isolated and mixed strains of t of MTA . Anotheralbicans . CEM cemalbicans . What maalbicans . in situ or extracted. In some cases, the contralateral premolar and the maxillary premolars are also extracted, with spontaneous space closure or closure with orthodontic appliances. Other options are implant-supported prosthetic replacement or a tooth-supported bridge and pontic [In cases of mandibular second premolar agenesis, the PSM may be left d pontic . Long-ted pontic . Howeverd pontic . In thesd pontic , 9.Although extraction of the PSM with conditions similar to this case seems to be more prescribed, it cannot be denied that by providing chances of abscess healing and tooth functioning, the clinician has prevented the future complexity of space maintaining. In other words, the PSM can now play a role as a space maintainer until is natural exfoliation, which is postponed due to agenesis of the permanent successor , or implIn many cases with missing of the permanent teeth, the necrotic primary tooth can be saved via endodontic management to play its role as a space maintainer. CEM cement has proven sealing, antibacterial/fungal and regenerative properties that make it a suitable biomaterial for root canal filling of the infected primary teeth.


Osteochondroma arising from the tibial tuberosity is very rare. We report such a case which mimicked OsgoodSchlatter’s disease in an adolescent. A 12 years-old boy presented with swelling over his right proximal tibia of one year duration associated with pain in the last three months. Examination revealed a 4 x 2cm bony mass arising from the proximal tibia. Radiographs revealed an osteochondroma of the tibial tuberosity. Computer tomography and magnetic resonance imaging confirmed the continuity of the medulla of the bony mass to that of the parent bone. Excision biopsy was done. At the final follow up, he was asymptomatic and returned back to his daily activities. We present this case for its rarity, challenges involved in diagnosis and the difficulties encountered in planning the surgery because of involvement of the apophysis and extensor mechanism attachment in a skeletally immature boy. We report a rare case of osteochondroma arising from the tibial tubercle in an adolescent who presented with anterior knee pain. The challenges in diagnosis and treatment options are discussed.Osteochondroma is the most common benign bone tumour. It accounts for 10-15% of all bone tumours and around 30% of all benign bone tumoursA 12 years-old boy presented to our outpatient department with swelling and pain over his right knee. The swelling was present for one year, insidious in onset and gradually progressive. The onset of pain was also insidious beginning three months prior to presentation. Pain was aggravated on prolonged walking, playing, sitting cross legged and squatting. There was no preceding history of significant trauma or fever.On examination, there was a 4 x 2cm globular swelling arising from the anterior aspect of his proximal tibia. The swelling was hard in consistency and non tender. The range of movement of the knee was full but painful on extension and terminal flexion. There were no distal neurovascular deficits.Considering the age of the patient and the location of the mass, clinically we suspected apophysitis of the tibial tuberosity. Blood investigations were normal except alkaline phosphatase which was 713 IU/L . Radiograph of the knee revealed a bony outgrowth from the anterior aspect of the tibial tuberosity without any fragmentation suggesting probably an osteochondroma . Computein toto along with the periosteum. The limb was immobilised for two weeks after which knee mobilisation was started. The histopathological report of the excised globular mass measuring 4 x 3 x 3cm with cartilage cap thickness of 0.2cm was suggestive of osteochondroma . It is necessary to clinically differentiate between the two conditions as both are common in adolescents. OSD is due to traction apophysitis of the tibial tubercle, and usually presents with soft tissue swelling near the tibial tubercle and pain is mostly during extension of the kneeRadiographs in osteochondroma usually show a bony projection arising from the underlying bone. In OSD, there is usually fragmentation of the tibial tubercle whereas Trevor’s disease there are irregular multiple opacities in the epiphysis. CT and MR imaging help in identifying the normal and the abnormal bone, the soft tissue involvement and to determine the continuity of the abnormal mass with that of the medullary cavity of the parent bone.Decision making on whether to operate or to treat them nonoperatively is very important. The issues on surgical intervention in skeletally immature patients are damage to the physis as a result of extensive excision leading to limb length discrepancy. Ideally, excision of osteochondroma is done after skeletal maturity, so that the mass is usually away from the growth plate thereby reducing the risk of physeal damage.We decided to operate in this case as our patient had persistent pain affecting his daily routine. The dilemmas we faced before operating were whether to approach the lesion through transtendon or parapatellar approach. What do we do regarding the attachment of the extensor apparatus if the lesion is involving the entire tibial tubercle? How far should we resect the lesion as it is very close to the proximal physis? We decided to approach the mass through a medial parapatellar approach as the mass was more anteromedially based on the imaging. The patellar tendon was retracted laterally and the mass was resected in toto just distal to the level of the physis. We would have had difficulty in excising the medial aspect of the mass if we had used tendon splitting approach. Since the mass was just proximal to the distalmost attachment of the patellar tendon, we were able to preserve the extensor tendon attachment in this particular case. Had it involved the entire distal attachment of extensor apparatus, it would be better to excise the whole mass and attach the tendon to the bone using suture anchors or by attaching the tendon to the bone through a tunnel using non-absorbable sutures by Krakow stitch on the tendon. Though we have excised the entire mass just adjacent to the physis, long term follow up is necessary till skeletal maturity to watch for recurrence or growth disturbance.In conclusion, clinicians should be aware of this rare entity of osteochondroma of the tibial tubercle, its clinical presentation, differential diagnoses and management difficulties. Adequate imaging is necessary particularly when the patient is symptomatic, to arrive at an early diagnosis. Proper preoperative planning is required along with early intervention so that adolescents will be able to resume their regular day-to-day activities soon after surgery.Conflict of interest: The authors declare that there is no conflict of interest.


A 46-year-old man presented with shortness of breath and recurrent, left-sided pleural effusions. A computed tomography (CT) scan of the chest showed a large, left-sided pleural effusion with a mass in the posterior mediastinum. A pleural tap showed lymphocytic exudate. The biopsy of the mass was inconclusive. A left posterolateral thoracotomy was performed, which showed a large posterior mediastinal mass adherent to the left lung. The mass was completely excised and the histopathology proved it to be giant predominantly cystic schwannoma. The pleural effusion resolved after tumor resection. Schwannomas, also known as neurilemmomas, are rare, benign, neurogenic tumors of Schwann cells that form the outer insulating layer of peripheral nerves. In adults, neurogenic tumors account for around 20% of mediastinal tumors, with most of the schwannomas occurring in the posterior mediastinum . SchwannA 46-year-old male, with no known comorbidity, presented with an episode of urticaria along with shortness of breath. According to the patient, the urticaria had started from the extremities, progressed to the trunk, and was associated with generalized itching and swelling of lips. This was accompanied by shortness of breath and a cough that progressed from dry to productive with white sputum. He had a history of undocumented weight loss over the last few months. The rest of the symptom review was unremarkable. The patient was a long-term smoker who had recently quit smoking in the past six months and was currently an active betel nut chewer. He had no known allergies, no prior hospitalizations or surgical procedures, and was not on any routine medications. His family history was unremarkable.A chest radiograph revealed a left-sided pleural effusion. A pleural tap showed a predominantly lymphocytic exudate. The pleural effusion was recurrent and had not settled despite repeated pleural taps. A subsequent computed tomography (CT) scan of the chest revealed a large, left-sided pleural effusion, as well as a large soft tissue mass in the posterior mediastinum in a paravertebral location without extension into the spinal canal, as can be seen in Figure Biopsy attempts had proven inconclusive. Routine laboratory investigations were unremarkable. The patient was advised tumor excision. A left posterolateral thoracotomy was done, in which a large posterior mediastinal mass was seen adherent to the left lung, as seen in Figure The mass was completely excised, as shown in Figure The patient had an uneventful postoperative recovery. Histopathology of the mass, which measured 12.5 x 8.5 x 7.5 cm, showed it to be a giant, predominantly cystic Schwannoma, with medium- to large-sized, congested hyalinized vessels with surrounding hemorrhage, as well as dense acute and chronic inflammatory changes without evidence of malignancy. The S-100 immunohistochemical stain was positive. Subsequent follow-up visits of the patient showed a complete resolution of the pleural effusion.Posterior mediastinal masses may be classified as per their origin and morphology as neurogenic, esophageal, cystic, extramedullary hematopoiesis, or lymphomas. Of these, neurogenic tumors account for 80% of the posterior mediastinal masses. They may be peripheral, sympathetic, or paraganglionic in origin, and only a minority of these tumors are malignant (10% to 20%) . In adulMost schwannomas are asymptomatic and may be incidentally found on routine imaging. If symptoms do occur, they are usually because of their compressive effect on adjacent mediastinal structures, such as the airway, esophagus, heart, and great vessels. Rarely, when schwannomas are malignant, symptoms may also be due to the invasion of local structures and may present with respiratory symptoms, such as stridor, dyspnea, hemoptysis, and cough, or gastrointestinal (GI) symptoms such as dysphagia. Adjacent nerve involvement may present as hoarseness, pain, diaphragmatic paralysis, or Horner's syndrome. Hemothorax has also been reported with posterior mediastinal schwannomas .Rarely, schwannomas may cause a pleural effusion. Very few cases have reported pleural effusions associated with schwannomas, and, in these reports, the schwannomas primarily originated from structures within the hemithorax such as from the intercostal nerves or from the chest wall . In our Another unusual and noteworthy finding was the predominantly cystic nature of the schwannoma. Most schwannomas are solid and may have cystic components; however, totally cystic schwannomas are rare. Reported cases have usually been in close proximity to the spine and, consequently, have presented with neurological symptoms such as back ache and paresis , rather Biopsy attempts may be inconclusive, as in our patient, and, as a result, surgical resection is necessary both for diagnosis and for treatment. Benign schwannomas have an excellent prognosis while malignant schwannomas may need additional radiotherapy .Giant cystic schwannomas may arise from the posterior mediastinum and cause recurrent pleural effusions. Patients may present with respiratory distress, and CT scans of the chest help identify the mass. Treatment involves a resection of the mass, after which the pleural effusions resolve spontaneously.