Primary retroperitoneal teratomas involving adrenal glands are exceedingly uncommon accounting for only 4% of all primary teratomas. They are more common in childhood and rarely occur in adults. Only a very few case reports have been documented in literature so far. Herein, we report a mature (benign) cystic retroperitoneal teratoma in the region of left adrenal gland in a 22-year-old otherwise healthy male patient who presented with a 1-month history of left flank pain. In addition, a literature review on teratomas is included. Primary mature teratomas are uncommon nonseminomatous germ cell tumors. They are made up of well-differentiated parenchymal tissues that are derived from more than one of the three germ cell layers  . They usA 22-year-old otherwise healthy male patient presented to King Faisal Specialist Hospital and Research Centre with a 1-month history of left flank pain. Physical examination was remarkable for a palpable, nontender, and limitedly mobile mass on the left flank. All laboratory investigations were unremarkable. An abdominal contrast-enhanced computed tomography (CT) scan showed a large lesion in the region of left adrenal gland measuring 9.0 × 9.2 × 10.8 cm and demonstrating multiple cystic spaces with thin septations as well as multiple areas of fatty collections and coarse calcifications . The hugThe patient underwent complete surgical resection of the left adrenal mass. Grossly, the left adrenal mass weighted 484 g and measured 9.0 × 9.0 × 10 cm. The mass had well-circumscribed smooth borders and rubbery consistency. Cut-section of the mass revealed multilocular cystic spaces, whitish-gray walls, scattered yellowish adipose tissue collections, mucus secretions, and areas of calcifications . MicroscSince there was no evidence of immature or malignant components, no radio- or chemotherapy was offered. The patient was discharged uneventfully in a stable condition. A postoperative 6-month followup failed to show any evidence of tumor recurrence.Germ cell tumors (GCTs) can be broadly classified into two main categories: seminomatous and nonseminomatous GCTs. Teratomas belong to nonseminomatous GCTs and represent the most common form of all GCTs . TeratomGenerally, teratomas arise from uncontrolled proliferation of pluripotent cells: germ cells and embryonal cells. The type of pluripotent cell greatly influences the presentation time and involved location of teratoma. Teratomas of germ cell sources can be congenital or acquired and are usually found in gonads (testes and ovaries). In contrast, teratomas of embryonic cell sources are always congenital and are usually found in extragonadal locations, such as intracranial, cervical, retroperitoneal, mediastinal, and sacrococcygeal sites , 4.According to the location of tumor, teratomas can be classified into gonadal and extragonadal teratomas. Gonadal teratomas are more common, mostly primary neoplasms, mainly in adults, and usually take place in gonads (testes and ovaries) . ConversFurthermore, according to the content of tumor, teratomas can be classified into solid, cystic, or mixed teratomas. Solid teratomas lack organization and contain only parenchymal tissues. Cystic teratomas contain only sacs of fluid, semifluid, or fat, whereas mixed teratomas contain both solid and cystic components .Besides, according to the epithelial lining and dermal contents of tumor, teratomas can be classified into epidermoid, dermoid, and teratoid teratomas (cysts). Epidermoid teratomas are lined by stratified squamous epithelium and lack dermal contents. Dermoid teratomas are mostly lined by stratified squamous epithelium and contain various dermal contents such as hair, sweat, and sebaceous glands. Teratoid teratomas are mostly lined by respiratory columnar epithelium and contain sebum .In addition, according to the degree of tumor maturation, teratomas can be classified into mature and immature teratomas. Mature teratomas are generally benign, asymptomatic and more common, among females. They are highly variable on histology and can be solid, cystic, or mixed. They contain different types of parenchymal tissues that are well differentiated. Mature cystic teratomas (AKA dermoid cysts) may have partially to completely well-developed organ systems. On the contrary, immature teratomas are histologically solid teratomas and contain immature (undifferentiated/undeveloped) parenchymal tissues and can be possibly benign, possibly malignant, or frankly malignant. They are more common among males , 14.Some mature (benign) and immature  teratomas have an increased tendency to become frankly malignant teratomas, and frankly malignant teratomas have an increased propensity to metastasize. This group of exceptionally rare teratomas is known as teratomas with malignant transformation . The strOccasionally, a teratoma may contain various components of other germ cell tumor, and hence it is not a pure teratoma per se, but rather it is a mixed germ cell tumor and has malignant nature. In infants and young children, these components are frequently endodermal sinus tumor and choriocarcinoma. A pure teratoma can be benign, however, highly aggressive in its clinical course as in a growing teratoma syndrome (GTS). GTS refers to a rapidly growing pure mature (benign) teratoma that appears during or following chemotherapeutic eradication of malignant components of a nonseminomatous germ cell tumor, and it has normal serum tumor marker levels of alpha-fetoprotein and human chorionic gonadotropin .The vast majority of retroperitoneal teratomas are secondary neoplasms and mostly occur in males . PrimaryPrimary retroperitoneal teratomas involving adrenal glands are exceedingly uncommon accounting for only 4% of all primary teratomas –4 and caOnly a few case reports have been documented in literature so far . They arTeratomas can be diagnosed based on high index of clinical suspicion, routine laboratory, and radiographic investigations . With reWith respect to laboratory investigations, retroperitoneal teratomas can express a diversity of serum tumor markers such as elevated alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), and CA 19-9 –13. ThesWith respect to radiographic investigations, they play valuable roles in diagnosis of teratomas. Plain radiographs (X-ray) can identify calcified elements in 62% of cases –13 whereSurgical excision of benign (mature) teratoma is required for a definitive diagnosis   and remaPrimary retroperitoneal teratoma involving the region of adrenal gland is exceedingly rare , and its occurrence in an adult is exceptionally uncommon. However, it should be regarded in the differential diagnosis in any patient presenting with a flank pain. Histopathological examination of the resected tumor warrants a definitive diagnosis. Surgical excision of mature (benign) teratoma remains the mainstay of treatment with an excellent five-year survival rate of nearly 100%.


The present study analyzed a case of immature teratoma in the posterior cranial fossa of an infant and compared the clinical data with the associated literature. Ventricular drainage was initially performed upon the patient’s admission to the hospital. Following adequate pre-operative preparations, the tumor in the posterior cranial fossa was resected on the third day. No significant neurological function deficiency was observed following the surgery and no recurrence was noted within an 18-month follow-up period. In such cases, treatment should be conducted in a stepwise manner, with the hydrocephalus relieved first, followed by complete tumor resection subsequent to full preparation. Post-operative chemotherapy was not performed by conventional means as the infant was too weak, therefore, periodic reviews and long-term follow-up were required. Teratomas are ectopic tumors containing multiple tissues from more than one mesoderm. Encephalic teratomas are rare and account for 2 to 5% of infant teratomas and 0.3 to 0.6% of all ventricular tumors. Teratomas may occur at any age, but with a higher incidence rate in younger patients . In addiA male infant aged 4 months and 9 days was admitted to Fuzhou General Hospital of Nanjing Military Command with disease symptoms, including binocular convergence, a downward gaze for >10 days and sudden seizures for one day. The infant was vaccinated according to the recommended immunization schedule and no clear abnormalities were observed. The size of the infant’s head was normal for his age. The infant was born mature to a 34-year-old G3P3 mother, without any history of exposure to X-rays and toxic substances. No abnormalities were observed during the routine antenatal care. The infant weighed 3.5 kg at birth and did not have a history of asphyxia rescue. The patient’s Apgar score was 9. Breast-feeding was conducted after birth. A special examination of the infant revealed the following: a 5×5 cm bregma that was plump and bulging to a height of ∼3 mm, and a head size of 49 cm. Macewen’s sign was observed upon skull percussion. Additionally, the infant was in a moderate coma. The setting-sun sign was observed in the patient’s eyeball, while the bilateral pupil of the infant faced downwards, had a diameter of 1.5 mm and lagged in response to direct and indirect light. Muscular tension in the four limbs of the infant patient was reduced and limbs bent upon pain stimulation. Head magnetic resonance imaging (MRI) scans of the infant showed a quasi-circular long signal in the cerebellar vermis, which exhibited a marginally high signal in the water-suppression image. The lesion was ∼5.8×5.7×6.0 cm in size and had a clear boundary. The signal of the lesional substance was uneven. Small, circular long T1 and T2 signals and short, irregular T1 and T2 signals were also observed. The brain stem was compressed and deviated forward. The fourth cerebral ventricle had become narrowed due to compression. The third cerebral and two lateral ventricles were expanded. A small patch of long T1 and T2 signals was observed around the lateral ventricles, which showed a high signal in the water-suppression image. The fissures and sulci on each side were not broadened and the middle structures of the brain remained at the midline . The cliVentricular drainage was performed through a puncture in the anterior fontanelle as an emergency treatment once the patient had been admitted to the hospital. The patient’s consciousness gradually returned following the surgery. Tension in the anterior fontanelle was reduced. Thus, a fluid infusion was performed to stabilize the patient’s condition. After full preparations had been made, including fluid infusion, blood preparation and the use of intraoperative pathology, the cerebellar vermis tumor was resected from the posterior midline of the patient under general anesthesia on the third day. The tumor was located in front of the cerebellar vermis during the surgery and was lobulated with an irregular shape and uneven texture. Certain sections of the tumor were soft, whereas others were tough. Cystic changes were observed in several places. A large amount of the cyst fluid was yellow, whereas the remainder was white and dirty. In addition, several light-red small circular nodes, with a diameter of ∼3 mm and rough surfaces, were observed . CalcifiIntracranial teratomas exhibit different clinical features according to the growth zones. In the majority of infant patients, the main clinical feature of these tumors is intracranial hypertension. Intracranial teratomas are commonly accompanied by symptoms that include obstructive hydrocephalus, which results in headaches, vomiting, papilledema, outreach paralysis and progressive increases in head size. A small number of infant patients also experience epileptic attacks, even when they are in a coma, which may be associated with the intracranial hypertension and brainstem compression caused by the tumor. Infant patients with posterior cranial fossa teratomas do not exhibit dystaxia since their motor function is not yet fully developed. The reason that a medical consultation is required for these infant patients is typically the presentation of hydrocephalus or epilepsy . MRI hasIn the present study, the patient was admitted to the Fuzhou General Hospital of Nanjing Military Command due to binocular cohesion and a downward gaze for >10 days, and sudden hyperspasmia for one day. These symptoms are features of hydrocephalus and epilepsy. The patient did not undergo immediate tumor resection since he was weak and the hydrocephalus was serious. Therefore, the hydrocephalus was resolved first. Tumor resection was then performed from the posterior midline under general anesthesia on the third day after full pre-operative preparations had been made. A good therapeutic effect was subsequently achieved. A lateral ventricle puncture through the bregma was a suitable choice since the infant bregma was not yet closed. This surgery is simple and easy and is able to rapidly relieve severe intracranial hypertension caused by hydrocephalus, thereby stabilizing the vital signs of infant patients. This allows doctors more time to perfect the pre-operative preparations and improve the safety of the tumor resection. However, a ventricle puncture may cause transtentorial herniation when posterior cranial fossa occupation is accompanied by obstructive hydrocephalus. Such a case would require more attention during treatment. Resection is the main method for treating posterior cranial fossa teratomas. In solving the hydrocephalus, doctors should attempt to remove as much of the tumor as possible. The key aspect of treating a teratoma is opening the circulation passage of the cerebrospinal fluid. This type of tumor has a complete membranous envelope with a clear boundary and a low blood supply. Therefore, as much of the tumor as possible should be removed. Since tumors are often located within important brain structures, the surgery should be performed carefully to avoid damaging other organs. Teratoma surgeries should also be conducted carefully for sections of teratomas that exhibit cystic changes, in order to prevent cyst fluid from flowing into the resorption cavity of the arachnoid space, thereby preventing post-operative pyrexia.Platinum-based chemotherapy is the main post-operative chemotherapy for immature teratomas . The moset al (The prognosis for an immature teratoma is associated with tumor differentiation. Highly-differentiated tumors have a better prognosis, while those that are mainly composed of undifferentiated embryonal tissue have a worse prognosis. Ogawa et al  reportedet al . Teratomet al . Long-teet al . In the In conclusion, infant patients with immature teratomas in the posterior cranial fossa are brought to see doctors mainly due to the symptoms of hydrocephalus and epilepsy. Ventricular drainage through an anterior fontanelle puncture is an effective measure for treating patients with severe hydrocephalus. Immature teratomas have the potential to change into other malignant tumors, thus, a long-term follow-up should be conducted for patients even if a full resection has been performed.


Glioblastoma multiforme (GBM) is the most common and malignant primary intracranial tumor, and has a median survival of only 10 to 14 months with only 3 to 5% of patients surviving more than three years. Recurrence (RGBM) is nearly universal, and further decreases the median survival to only five to seven months with optimal therapy. Tumor-treating fields (TTField) therapy is a novel treatment technique that has recently received CE and FDA approval for the treatment of RGBM, and is based on the principle that low intensity, intermediate frequency electric fields (100 to 300 kHz) may induce apoptosis in specific cell types. Our center was the first to apply TTField treatment to histologically proven GBM in a small pilot study of 20 individuals in 2004 and 2005, and four of those original 20 patients are still alive today. We report two cases of GBM and two cases of RGBM treated by TTField therapy, all in good health and no longer receiving any treatment more than seven years after initiating TTField therapy, with no clinical or radiological evidence of recurrence. Glioblastoma multiforme (GBM) is the most common and malignant primary intracranial tumor, representing as much as 30% of primary brain tumors with increasing incidence in some geographic regions. Its incTumor-treating fields (TTField) therapy is a novel treatment technique with the potential to treat various forms of cancer. TTField therapy is based on the principle that low intensity, intermediate frequency electric fields (100 to 300 kHz) have an anti-mitotic effect which acts during late metaphase and anaphase, with specific frequencies affecting specific cell types. The appOur center was the first in the world to apply TTField treatment to histologically proven GBM patients in a small pilot study of 20 individuals in 2004 and 2005 Table. The incBaseline characteristics of all 20 participants in the original pilot study. Dates are presented as month/year for simplicity, age calculations were performed on exact dates.GBM, glioblastoma multiforme (newly diagnosed); KPS, Karnofsky performance scale; L., left; No., number; R., right; RGBM, recurrent glioblastoma multiforme.A 52-year-old woman with a history of epileptic seizures and left-sided hemiparesis was diagnosed with an intra-axial brain tumor, suspected to be a high-grade glioma based on the magnetic resonance imaging (MRI) findings Figurea. The tuA 41-year-old male presented with pronunciation difficulties in December 2003. Neurological examination revealed slight right-sided hemiparesis, and MRI revealed an intra-axial tumor suspected to be high-grade glioma  have survived until the time of this report, roughly seven years Figure. These iYounger age and a higher KPS have been proposed as prognostically favorable parameters for longer survival. The meain vitro and in vivo[TTField therapy has been shown to effectively inhibit glioma cell replication d in vivo,9. The pd in vivo. The phad in vivo. The reaIn the present paper we report two cases of GBM and two cases of RGBM treated by TTField therapy, all in good health and no longer receiving any treatment more than seven years after initiating TTField therapy, with no clinical or radiological evidence of recurrence. Our results indicate that TTField treatment may be remarkably successful in a subgroup of GBM/RGBM patients, and further investigation is needed to identify any unique characteristics of this patient group.Written informed consent was obtained from all patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.BOLD-fMRI: Blood oxygen level dependent functional magnetic resonance imaging; FLAIR: Fluid Attenuated Inversion Recovery; GBM: Glioblastoma multiforme; KPS: Karnofsky performance scale; LTS: Long-term survival; MRI: Magnetic resonance imaging; PET: Positron emission tomography; RGBM: Recurrent glioblastoma multiforme; TTField: Tumor-treating fields; WHO: World Health Organization.JV, JS, VD and FT are consultants for NovoCure, Ltd, Haifa, Israel. AMR, JiK, JaK and MS have no competing interests to declare.JV participated in conception and design of the manuscript; acquisition, analysis and interpretation of the data; initial drafting and revision of the manuscript; and approval of the final version. AMR participated in conception and design of the manuscript, drafting and revision of the manuscript, preparation of images and approval of the final version. JiK participated in conception and design of the manuscript, drafting and revision of the manuscript and approval of final version. JaK, JS, VD, MS and FT participated in the acquisition, analysis and interpretation of the data, revision of the manuscript and approval of the final version. All authors read and approved the final manuscript.


Solid pseudopapillary tumor (SPN) of the pancreas is a rare tumor, but has favorable prognosis. It is typically observed in young women. Only few cases have been reported in young men. We report the observation of a 73-year-old man presented with a palpable mass in the left upper abdomen. CT scan showed 10 cm mass at the tail of the pancreas. This mass had mixed cystic and solid components. The patient underwent a distal pancreatectomy and splenectomy. SPN of the pancreas was diagnosed based on histopathological features. The patient recovered uneventfully and didn't receive adjuvant therapy. A CT scan performed 16 months postoperatively showed no evidence of disease recurrence. Although SPN of the pancreas is typically observed in young women, the diagnosis should not be discounted in old male patients. Male patients and those with old age, atypical histopathology and incomplete resection may have a higher risk of recurrence and death, deserving particular attention. Solid pseudopapillary tumor (SPN) of the pancreas is a rare tumor that constitutes of 0.2 to 2.7% of the primary non-endocrine tumors of the pancreas. It is usually observed in young women, but it occurs in men in less than 10% of cases, that's why the diagnosis is frequently delayed and tumor size at presentation is frequently large. Due to its rareness and unusual behavior, SPN of the pancreas is often associated with diagnostic and therapeutic challenges. We report the case of a 73-year-old man having a solid pseudopapillary tumor of the tail of the pancreas.A 73-year-old male who was in his usual status of good health, presented with intractable vomiting and nausea for two days. He had epigastric pain, increasing abdominal girth of about 4 months duration and 7 kg weight loss over two months. There was no jaundice, change in stool color, diarrhea, or flushing. On examination there was mild diffuse abdominal tenderness in all quadrants, as well as a palpable mass on left upper quadrant. All blood tests were normal, including tumor markers. Ultrasonography was carried out and revealed a 10cm X 8 cm solid-cystic mass in the left upper quadrant of the abdomen. CT scan showed a well-marginated, encapsulated, heterogeneous, hypovascular mass, at the tail of the pancreas, with a thickened, peripherally enhancing capsule. This mass had mixed cystic and solid components .At laparotomy, there were no metastasis, and no severe adhesion or invasion of the pancreatic mass to the adjacent organs and near-by vessels was grossly noted. A distal pancreatectomy with splenectomy were performed. On macroscopic examination, the tumor was round, fluctuant and measured 10,5x 8x 6,5 cm. It was encapsulated and well-demarcated from the surrounding pancreas. The cut section revealed light brown solid areas with zones of hemorrhage and necrosis at the periphery of the tumor and cystic degeneration centrally.Microscopically, the growth pattern was heterogeneous with a combination of solid, pseudo-papillary and haemorrhagic-necrotic and pseudocystic structures. Solid areas were composed of poorly monomorphic, eosinophilic and clear cells admixed with hyalinized to myxoid stromal bands. Neoplasic cells delicately infiltrated the fibrous capsule, separating the tumor from the normal pancreas . VasculaSPN has been first described by Franz in 1959 . It is aTypically, SPNs are observed in young women , which supports the ovarian cell theory described above; indeed, over 90% of patients have been reported in women under the age of 35 , with onMost patients present with vague symptoms which include abdominal pain, increased abdominal distension, gastric outlet obstruction which are related to tumor compression on the adjacent organs, although almost 30% of the patients with SPN are asymptomatic. Generally, there is no specific clinical syndrome for SPN of the pancreas, and the disease tends to be misdiagnosed, especially in male patients.Radiologic imaging is an essential part of the diagnosis of pancreatic SPN. In CT scan SPN tumors demonstrate no enhancement of the cystic portions but slight enhancement of the solid portions in the arterial phase and marked enhancement in the portal venous phase . OperatiDespite the locally aggressive features, solid-cystic pseudopapillary tumor of the pancreas has a low-grade malignant potential and tends to have a favorable prognosis, even in the presence of metastatic disease . OverallSolid pseudopapillary neoplasm of the pancreas is a rare low-grade malignant tumor. Although this tumor is typically observed in young women, the diagnosis should not be discounted in old male patients. Surgical treatment alone is the best treatment of solid cystic pseudopapillary tumor of the pancreas; it should be pursued regardless of distant metastasis and size. Total resection guarantees best prognosis.


Infantile myofibroma is a rare and benign tumour of children presenting in the head and neck region. Rendering a final diagnosis of infantile myofibroma can be challenging in the light of nonspecific clinical, radiological findings and its histopathological similarities with a number of neoplasms especially spindle cell tumours. In this paper we discuss a case of infantile myofibroma in a 2-month-old infant, enumerating the various differential entities that have to be eliminated in reaching its specific diagnosis and highlighting the importance of immunopositivity to vimentin and smooth muscle actin (SMA) in establishing its myofibroblastic differentiation. Infantile myofibroma (IM) is a rare benign tumour of the myofibroblasts commonly found in the head and neck region of an infant. It usually manifests as a swelling in the dermis and subcutis with equal propensity of occurrence in male and female infants . The sweWe report a case of infantile myofibroma (IM) discussing its clinical, radiological, histopathological, and immunohistochemical features and the problems faced in its diagnosis.A 70-day-old baby was noticed to have a persistent swelling on its forehead since birth by her mother. The pregnancy was normal with no history of trauma during delivery reported. The family history was also irrelevant. On clinical examination, the infant appeared systemically healthy with a firm, subcutaneous nodule situated in the right frontal bone region of the skull measuring 2.5 × 3 cm in dimension . The sweUnder ET, general anesthesia, supine position, a left supraorbital curvilinear incision was given to expose the lesion. Pericranium was elevated. The lesion was whitish, fleshy, and infiltrating the pericranium. Piecemeal total excision was done. Bony edges were nibbled. Dural surface was scrapped. Involved pericranium was excised and the wound was closed in layers Figures . MacroscMicroscopically, there was a multinodular growth pattern that had a biphasic pattern owing to the alternation of light and dark staining areas . The ligMyofibromatosis was initially described in 1951 as congenital fibrosarcoma  and subsThe aetiopathogenesis of myofibroma is obscure. Some have reported its inheritance as an autosomal dominant pattern while others have suggested an autosomal recessive pattern , 10. IntThe solitary nodules are most commonly seen in the head and neck region which include scalp, forehead, orbit, parotid region, and oral cavity , 7. Alth1 weighted image revealed a well-delineated osteolytic lesion which appeared hyperintense as compared to the brain which prompted a provisional diagnosis of leiomyoma/eosinophilic granuloma. Radiographically, myofibromas appear as a well-defined unilocular radiolucency in most cases with a few exhibiting multilocularity. Intraosseous lesions may show lytic areas with marginal sclerosis . MRI is Microscopically, the lesion presented with a biphasic cellular pattern which is usually seen in myofibromas , 9, 10. Histopathologically, the definitive diagnosis of a myofibroma was challenging in light of the various differential diagnosis that had to be excluded. Nodular fascitis was excluded as it is rarely seen in infants and has a prominent myxoid matrix and the absence of hemangiopericytoma-like pattern of myofibroma. Leiomyoma and leiomyosarcoma were excluded based on the negativity of tumour cells to desmin. Another closely resembling tumor which was considered was fibrous histiocytoma, which is typically composed of polymorphous cells arranged in a storiform pattern and exhibits only focal staining with SMA. Neurofibroma and neurofibromatosis were excluded based on the immunonegativity of myofibroblasts to S-100. Fibromatosis usually has a monophasic growth pattern consisting of long fascicles of spindle cells among abundant wavy collagen fibres which was not seen in the present case. Fibrosarcoma could be ruled out by the presence of nuclear atypia, high mitotic counts, and abnormal mitosis, which was, however, absent in the present case.Conservative surgical treatment is the most effective treatment of IM . Some caInfantile myofibroma is a benign, self-limiting, and localized tumour consisting predominantly of myofibroblasts. The diagnosis of this apparently benign lesion is important as it may histologically mimic other forms of aggressive fibromatoses. Careful exclusion of IM from these with the help of specific markers may help in accurate diagnosis and appropriate surgical management. The case described here is a very rare type of solitary IM eroding into the frontal bone without intracranial involvement.


Giant lymph cysts are a relatively frequent complication after surgical procedures in the abdomen, often after kidney transplantation, but there are also cases after pelvic surgery such as lymphadenectomy and others. In the recent literature, there have been no reported cases of idiopathic giant lymphocyst.We present the case of a 76-year-old Caucasian man who had a lymph cyst he had known of for more than 15 years. Laparoscopic treatment was necessary because of hydronephrosis of the left kidney.This case shows that laparoscopic drainage and partial resection of the lymph cyst is a safe and effective treatment. Lymphoceles are bothersome postoperative complications, most frequently occurring after pelvic or retroperitoneal lymphadenectomy or renal transplant surgery . SymptomThe method of treatment is controversial, ranging from conservative observation to aggressive deperitonealizing marsupialization at surgery -5.This report concerns a very rare case of an idiopathic giant lymph cyst and its laparoscopic treatment.A 76-year-old Caucasian man was referred to our Department of Urology with a low-risk urothelial bladder cancer. The patient told us about an abdominal lymph cyst that he had known about for more than 15 years; a puncture revealed no malignant cells. His surgical history consisted of an open cholecystectomy twenty years ago. Twenty months after first presentation with bladder cancer, ultrasound examination and a computed tomography (CT) scan showed a giant lymph cyst. In the past years, the patient noticed a gradual increase in abdominal girth. Physical examination revealed a well-nourished man  with a grossly distended abdomen without ascitic fluid wave or tympany.2 body surface , left kidney with 27% of function and hydronephrosis. The serum creatinine level was 105 μmol/L. At that time, he had the first symptoms because of this giant lymph cyst; an insertion of a 70-cm-long ureteral catheter on the left side was possible  showed a less-than-normal tubular function of 139 mL/min/1.73 mTime delay of further treatment was caused by a new diagnosed atrial fibrillation. After cardiologic diagnostics and medical treatment two months later, laparoscopic partial resection of the giant lymph cyst was performed. Percutaneous drainage of a portion of the fluid was necessary to create adequate space for port placement. After drainage of one litre of fluid, a right-sided midabdomen 10-mm trocar was achieved, a second 5-mm trocar was inserted in the left side. In total, the mechanical aspirated volume during the operation was eight litres. The color of the fluid was more tan or brown than the straw-colored fluid typical of seromas or dark red of hematomas. Microscopic evaluation of the fluid revealed mostly lymphocytes and red blood cells; in the resected wall of the lymph cyst, collagen and muscle fiber were found, with no malignancy. Laboratory study was consisted with lymphatic fluid as well; the result was sterile lymphatic fluid. After surgery, a prolonged secretion of lymph fluid for more than 10 days was obvious. External pressure of the abdomen was necessary.2 body surface , and the left kidney was now better with 39% of function and better outlet. The serum creatinine level was 92 μmol/L.Two months later, the lymph secretion interrupted. The position of the elongated left ureter is shown in Figure Pelvic and retroperitoneal lymphoceles may cause venous obstruction with subsequent edema and thromboembolic complications. Large lesions cause abdominal distension, and pain may become unbearable as the lymphoceles fill much of the abdomen ,2,4,6.Although lymphoceles are rarely fatal, they create a number of problems, some of which may be serious. They may compromise kidney function by various means: ureters may become obstructed, secondary infection may occur and blood supply may be diminished ,2,4,6. APelvic and retroperitoneal lymphoceles may cause venous obstruction with subsequent edema and thromboembolic complications. Large lesions cause abdominal distension, and pain may become unbearable as the lymphoceles fill much of the abdomen.Lymphoceles may appear from days to years ,6; an inUltrasound examination is excellent in the detection of lymphoceles. Lymphoceles generally occur in areas amenable to ultrasonography such as the pelvis, abdomen or retroperitoneum ,6. UltraCT displays characteristic, albeit not pathognomonic, signs. When uncomplicated, lymphocele masses produce low attenuation values. Negative CT numbers usually indicate fat content and are highly suggestive of lymphocele. Low CT numbers could effectively give an indication for abscess and hematoma from consideration and are helpful diagnostically. Thoracic and idiopathic lymphoceles are relatively uncommon .Diagnosis by needle aspiration is fairly straightforward and includes gross inspection and chemical examination of the fluid (to exclude urinoma) and Gram stain and cytology to exclude infection and tumor. The appearance of the lymphocele fluid varies from tan to dark yellow or brown, depending on the amount of fat.Because most lymphoceles resolve spontaneously, drainage should be reserved for large or symptomatic collections. Treatment options for symptomatic lymphoceles include percutaneous, open and laparoscopic drainage. Although performed with minimal difficulty, percutaneous drainage has a 50% to 80% rate of recurrence and therefore is often reserved for diagnosis ,8. CatheThe open drainage procedure provides definitive therapy for symptomatic lymphoceles; however, patients experience discomfort because of the laparotomy incision and may remain hospitalized for five to seven days .et al. [et al. [In 1991, McCullough et al. ,11. In 1 [et al.  reported [et al. .The size of this giant lymph cyst created a technical challenge. Minimizing morbidity remained a priority; the laparoscopic approach was attempted for this reason. Three ports were needed for the identification, manipulation and stapling of the cyst wall. In this particular case, the cyst wall was easily seen. If difficulty occurs, either laparoscopic or external ultrasound examination could be used to ascertain the location of any other cyst. Furthermore, the placement of a pigtail catheter externally into the cyst or methylene blue injection into the lymphocele could facilitate laparoscopic viewing . In our Reports of giant lymphoceles in either the immediate or distant posttransplant period are sparse. Most patients present earlier in the development of a lymphocele with complaints that include decreased renal function or increased creatinine, perirenal discomfort, cutaneous fistula formation or weight gain. This patient did not have any complaints; increased abdominal girth, hydronephrosis in ultrasound examination and decreased renal function occurred very late. Because the patient was symptomatic on presentation, we considered surgical drainage the best therapeutic option. The laparoscopic approach was used initially in the attempt to minimize the patient's discomfort and hospital stay. To prevent reaccumulation of the lymph, a large peritoneal window was created. Despite the large size of this lymphocele and the extent of the laparoscopic dissection required, the patient was able to be discharged from the hospital 13 days after the procedure without any untoward events.This case provides evidence that laparoscopic drainage should not be restricted to small and uncomplicated lymphoceles. Complex and giant lymphoceles may be drained safely and effectively by laparoscopy as well.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.THE was involved in drafting the manuscript and in the review of the literature and in performing the clinical follow-up. HG was involved in drafting the manuscript and in the review of the literature. CJH was involved in drafting the manuscript and in the review of the literature. SH participated in the surgery and was involved in the clinical follow-up. CL was involved in the review of the literature and the clinical follow-up. JR was involved in the clinical follow-up and supervised this report. All authors read and approved the final draft.


Introduction. Ovarian dysgerminoma is a rare malignant ovarian germ cell tumor with its peak incidence in young women. Abdominal pain, abdominal distention, and the presence of a palpable mass are common symptoms at presentation. Depending on the FIGO stage at presentation the prognosis of dysgerminomas after surgical treatment, adjuvant chemotherapy, and radiotherapy is promising. Case Presentation. A 7-year-old girl was presented at our clinic with abdominal pain in all abdominal quadrants. Later the pain localized in the region of her right ovary. CT scan revealed a massive formation which was connected to her right ovary. Conclusion. Although malignant ovarian germ cell tumours are rare in children, physicians must always consider the possibility of MOGT-occurrences. The clinical symptoms might not be specific: abdominal pain, abdominal distention, nausea, and vomiting. In order to make a correct diagnosis the patients should undergo a complete clinical examination including radiological scans. Initial management is frequently surgery, followed by adjuvant chemotherapy and radiotherapy. Although disgerminoma is malignant tumor, the prognosis is promising. Dysgerminoma is a rare malignant ovarian germ cell tumor (MOGT) which is highly malignant and has its peak incidence in young women. Approximately one-third of all dysgerminomas show KIT mutations and these are associated with advanced stage at presentation . ClinicaWe report the case of a 7-year-old Albanian girl who was presented with abdominal pain and a palpable mass in the region of her right ovary. Initially the girl complained about abdominal pain in all quadrants of the abdomen. Later the pain was localized on the right side. According to her mother the pregnancy with her daughter was uncomplicated; the girl had never been ill before, had no allergies, and was physically healthy looking except for light skin paleness. The laboratory results were normal except for haemoglobin  and haematocrit . The radiological CT scan of the abdomen and pelvis revealed a massive, solid, and clearly bordered formation with the dimensions 12 × 8 × 15 cm. The suspicious formation showed a connection to the right ovary that was hyperdense and had an inhomogeneous appearance. Some hypodense tissue within the tumour mass was identified as probably “fatty tissue” Figures . After aThe radiologist assumed an embryological origin of the tumour mass and suspected an “Immature teratoma of the right ovary with probable adhesion to the surrounding area.”The transversal infraumbilical laparotomy revealed a huge formation which involved both ovaries and the uterus. After intrasurgically consulting the gynaecologist the surgeons made the indication for a total hysterectomy with bilateral salpingo-oophorectomy. After extirpating the tumour mass suspicious lymph nodes were also removed and the surgical preparation was sent to the pathologist. The histopathological examination revealed a dysgerminoma with FIGO stage IIIc. The tumor mass was 692 g and measured 17 × 11 × 8.5 cm. On cut surface the tumor had multinodular appearance with confluent areas of necrosis and hemorrhage. Histological examination revealed infiltrating islands of uniform tumor cells with ample clear cytoplasm with central or slightly excentric nuclei with a vesicular chromatin pattern and prominent nucleoli Figures  and 5. TA review of Andrés et al. revealed the percentage of dysgerminomas (15%) in solid tumours in childhood . The meaβ-HCG levels were increased in older patients (40% versus 9%). Compared to these findings our case is rare regarding the age and the occurrence of a FIGO stage IIIc.Drozyńska et al. published differences of dysgerminomas in young children (younger than 10 years) and older children (between 11 and 18 years) regarding histology, primary localization, and biochemical markers. The marker AFP was higher in younger patients (76% versus 44%), whereas Lymph node metastasis is present in 28% of dysgerminomas and is significantly associated with poor survival . In ordeDespite these tumours being rare an overall survival of 97% can be achieved with conservative surgery and platinum-based chemotherapy . We usedOur case shows that inconspicuous symptoms like abdominal pain, abdominal distention, nausea, and vomiting might occur due to a malignant ovarian germ cell tumour. Although dysgerminomas rarely occur in childhood, the physician should not exclude the possibility of dysgerminomas appearing in this age. Therefore a complete clinical examination with radiological scans is necessary in order not to miss growing malignant formations. In cooperation with gynaecologists and paediatricians the best individual therapy option should be found. Adjuvant chemotherapy and radiotherapy show favourable outcome and future genetic examinations will hopefully reveal new biological and genetic targets to improve overall survival and fertility.


Male pseudohermaphroditism is a sex differentiation disorder in which the gonads are testes and the genital ducts are incompletely masculinized. An 8 years old dog with normal male karyotype was referred for examination of external genitalia abnormalities. Adjacent to the vulva subcutaneous undescended testes were observed. The histology of the gonads revealed a Leydig and Sertoli cell neoplasia. The contemporaneous presence of testicular tissue, vulva, male karyotype were compatible with a male pseudohermaphrodite (MPH) condition. XY embryos will develop testes whereas XX ones will develop ovaries . The process regulating gonadal development is under genetic control and is referred as sex determination. Once gonads are well developed and fully functional, hormones play a key role in phenotypic sex establishment. In male, embryonic testes produce two key hormones: anti-Muellerian hormone  and testosterone. These hormones, together with testosterone derivative dehydrotestosterone, are responsible for the development of internal and external male phenotype. In the XX embryos the absence of these hormones lead to female phenotypic sex development  cell tumor with solid-diffuse and cystic-vascular patterns (right testicle) and a Sertoli [sustentacular] cell tumor (left testicle) .SRY gene, together with a 78, XY chromosome complement, were present, even if in this gene a new SNP (Single Nucleotide Polymorphism) was identified. The SNP discovered is not reported in any public database or in any publication involving dog SRY gene studies  the change occurs outside the HMG box and it is clear that this domain represents the unique important functional domain of this gene ; [SRY sequence analysis on a large number of normal male dog in order to establish the real nature of this SNP (rare polymorphism or mutation?) but in this case we can consider that it is possible a not fully expression of the mutation (as this condition is yet reported in human cases of SRY missense mutation) so we can observe the SNP in normal dog. Nevertheless our hypothesis is that this SRY SNP could not be responsible for the observed phenotype.Male pseudohermaphroditism is a condition of sex differentiation disorder in which the gonads are testes and the genital ducts or external genitalia are incompletely masculinised . In the s Figure . To estatation); ; b) The tation); ,13; c) Btation); , of thistation); -17. One XX) patients are described in human medicine [In addition to the pseudohermaphroditism in the described case, neoplastic growth was found in both testicles, an interstitial cells tumour and a Sertoli cells tumor. Already at ultrasonograpic examination, the increase of blood flow was suggestive of a neoplastic growth . Only femedicine , probablmedicine -22. To tRetained testes lack spermatogenesis and have an increased risk of tumor development , particuDue to the good differentiation of both testicular tumours, immunohistochemistry was only performed for vimentin, CK and Melan A. Histological appearance of most testicular tumours in dogs is highly specific and immunophenotyping of the neoplasia is not necessary. In cases of mixed tumours or poorly differentiated testicular neoplasias the use of 3-beta-hydroxysteroid dehydrogenase (3beta-HSD) and LH receptor markers may help in the identification of the tumour type . Sertolird phase. This may have occurred due to the lack of the scrotum, the particular conformation of the whole genital system and the hormonal metabolism of the dog.Descent of testes, epididymis and spermatic cord is a complex series of events that requires hormonal, constitutive, and nervous control. This process occurs in three main stages: the relative transabdominal migration phase, the intra-inguinal phase and extra-inguinal migration . In thisExamination of the testes revealed the presence of inactive seminiferous tubules and epididymes. In this case there were atrophic seminiferous tubules and epididymes, which is probably due to the compression of the tumour on the surrounding testicular parenchyma.Both testicles also presented a complete tunica albuginea, epididymes, vessels and cremaster muscle but no deferent ducts, probably because the surgical exeresis did not include it.This results confirm that when a congenital problem is suspected, karyotyping should be performed before any other test and if the reproductive problem of intersex is confirmed, surgical resection of genital tract is the treatment of choice to avoid clinical problems and the development of neoplastic diseases .The authors declare that they have no competing interests.BE performed the clinical and ultrasonographic examination, conceived, designed and wrote the paper. PaP and DL carried out the genetic analysis and helped to draft the manuscript. PeP performed the clinical examination and surgery procedures. WP, JS and PB performed the histological and immunohistochemical examination, intellectually contributed and helped to write the paper. CAM was responsible for the histological procedures and reviewed the paper. All authors read and approved the final manuscript.


We report an extremely rare case of inflammatory myofibroblastic tumor of the posterior edge of the nasal septum. An 11-year-old boy presented with frequent epistaxis and nasal obstruction persisting for one year. Based on the clinical presentation and imaging studies, juvenile angiofibroma was suspected, but angiography suggested the possibility of another type of tumor. Transnasal endoscopic surgery found that the tumor protruded into the nasopharynx from the posterior end of the nasal septum. Histological examination identified spindle cells with immunoreaction for vimentin, smooth muscle actin, and anaplastic lymphoma kinase (ALK), but not for desmin and cytokeratin. This is a report of inflammatory myofibroblastic tumor mimicking juvenile angiofibroma. This case suggests that angiography is helpful in the differential diagnosis of epipharyngeal tumor in adolescence. Inflammatory myofibroblastic tumor (IMT) was first observed in the lung in 1990 , 3. IMT An 11-year-old boy had visited a provincial hospital because of frequent epistaxis and nasal obstruction persisting for one year. A tumorous mass was found in the nasopharynx, so computed tomography (CT) and magnetic resonance (MR) imaging studies were performed. Juvenile angiofibroma was suspected, so he was referred to our hospital for further examination and treatment.Physical examination found a smooth reddish mass in the nasopharynx. The imaging studies performed at the previous hospital were reexamined. CT scans showed a homogeneously enhanced soft tissue mass in the nasopharynx without bone destruction . The T1-Transnasal endoscopic extirpation of the tumor was performed under general anesthesia one week after the biopsy. The tumor was a red mass with a smooth surface originating from the posterior end of the nasal septum. The tumor was totally removed en bloc by resection at the posterior edge of the nasal septum. The surgery was completed with little blood loss and no complications.Histological examination with routine hematoxylin-eosin staining demonstrated proliferation of spindle cells with enlarged nuclei and infiltration of lymphoplasmacytic cells Figures . MitoticThe patient showed no evidence of recurrence at 8 months after surgery.IMT manifests as various symptoms depending on the site of origin. IMTs in the head and neck region do not always cause systemic symptoms, such as fever, weight loss, and pain, unlike IMTs in visceral organs . IMTs maIMT has no characteristic findings on CT or MR imaging, but invasive extension to the surrounding tissues may lead to misdiagnosis of malignant neoplasms , 7. FindIn our case, angiography showed only faint staining of the tumor and no obvious feeding artery. Although the clinical presentation and imaging studies were confusingly similar to juvenile angiofibroma, the angiography findings suggested the possibility of another type of tumor, so we decided to perform biopsy preoperatively.Pathological examination is essential to diagnose IMT. Histologically, IMT consists of myofibroblastic spindle cells with prominent infiltration of lymphocytes and plasma cells. Three histological patterns have been proposed : (1) myxImmunohistochemically, vimentin has been expressed most frequently (99%) in extrapulmonary IMTs, as well as SMA (95.8%), desmin (69%), and cytokeratin (36%) . In our Recently, ALK gene rearrangements have been reported in 50% to 75% of extrapulmonary IMTs , which sCorticosteroid administration, radiotherapy, chemotherapy, and surgery have been used as treatment modalities for IMT. The definitive therapy of extrapulmonary IMTs, including head and neck IMTs, seems to be total surgical excision, which is curative in more than 90% of cases , 6. On tThe present case of IMT of the nasal septum shows that, to avoid unnecessary overtreatment, the correct diagnosis must be established based on histological and immunohistochemical examinations. Although the tumor was thought to be removed completely, close followup is essential because the tumor was ALK positive, and recurrence in months to years after the surgery has been reported , 2.


A 25 year old man was admitted to otorhinolaryngology department with a mass in the ear canal. The patient had a history of tympanoplasty with mastoidectomy in another centre (for chronic otorrhea)2 years earlier. Details of the surgery were not available. He had no discharge during this time and he came to our hospital for routine follow-up visits. On otomicroscopy a white mass was noticed in the superior part of the canal, the origin of which could not exactly be localized. On palpation with a blunt instrument it proved to be soft. No otorrhea was noted in the canal and the tympanic membrane seemed to be intact in the visible area.The most likely diagnosis was cholesteatoma recurrence but the chief differential diagnosis to be ruled out in such cases is meningocele and dural herniation.An HRCT was ordered and the mass origin from attic was obvious. The intact tegmen tympani rules out intracranial involvement. The patient was operated and a rather extensive cholesteatoma was found and cleaned. It seems that he has had an attic cholesteatoma which went unnoticed and consequently unmanaged in the previous surgery and tympanoplasty had been done on the epidermal remnants in the attic.


Endometriosis is a chronic gynaecological disorder characterized by the presence of endometrial tissue outside the uterus. The disease most often affects the ovaries, uterine ligaments, fallopian tubes, and cervical-vaginal region. Urinary tract involvement is rare, accounting for around 1%-2% of all cases, of which 84% are in the bladder. We report a case of isolated lumbar ureteral stenosis due to endometriosis in a 37-year-old patient. The patient came to our observation complaining from lumbar back pain and presented with severe fever. The urological examination found monolateral left positive sign of Giordano. Blood tests evidenced marked lymphocytosis and increased valued of C-reactive protein. Urologic ultrasound showed hydronephrosis of first degree in the left kidney and absence of images related to stones bilaterally. Uro-CT scan evidenced ureteral stenosis at the transition between the iliac and pelvic tracts. We addressed the patient to surgery, and performed laparoscopic excision of the paraureteral bulk, endoscopic mechanical ureteral dilation, and stenting. The histological examination evidenced glandular structures lined by simple epithelium and surrounded by stroma. Immunohistochemical test of the glandular epithelium showed positivity for estrogen and progesterone receptors and moreover stromal cells were positive for CD10. The finding suggested a very rare diagnosis of isolated lumbar ureteral endometriosis. Endometriosis is a disorder characterised by the ectopic presence and growth of functional endometrial tissue, glands, and stroma, outside the uterus , 2.It is classified depending on the number, size, and superficial and/or deep location of endometrial implants, plaques, endometriomas and/or adhesions, as follows: stage I , stage II , stage III , and stage IV (severe >40 points), following the revised American Society for Reproductive Medicine classification for Endometriosis  .Approximately 10% of women in reproductive age are estimated to be affected by this disease , 5. CommPelvic pain could be expressed in a wide range combination of type, such as dysmenorrhea, dyspareunia, dysuria, dyschezia, and nonmenstrual chronic pelvic-abdominal muscle pain .As is suggested by many authors , 9, the The disease most often affects the ovaries , uterine ligaments, fallopian tubes, rectum, cervical-vaginal region, and urinary tract. Urinary tract involvement is rare accounting 1%-2% of all cases , of whicHowever, endometriosis can be encountered in other abdominal organs such as the liver, pancreas, intestinal tract, spleen , gallblaWe report a case of isolated lumbar ureteral stenosis due to endometriosis in a 37-year-old female patient.A 37-year-old female patient presented with lumbar pack pain, and severe fever. She had a past medical history of recurrent renal-ureteric colics accompanied by fever, pelvic pain and meteoric bowels and multiple drugs allergy . She reported the first menstruation when she was 10 years old, and following regular menses. Moreover she underwent cesarean section for fetal indication  during her only pregnancy. The urological examination found monolateral left positive sign of Giordano, left kidney area, and costovertebral angle tender to palpation. The ureteral trigger points on the left side were positive to deep palpation and the abdomen was painful but tractable.E. coli. Blood test showed neutrophilia and urine analysis showed >1.000.000 of colony forming units of Antibiotic therapy with amoxicillin + clavulanic acid 1 gr × 2/die e.v. and corticosteroid 4 mg/die i.m. was administrated.Urologic ultrasound  showed lFurther investigation was done by Uro-MRI  which shβ estradiol value.Suspecting a rare form of endometriosis and according to gynaecology consultant, we performed dosage of tumour markers and hormonal levels, which showed the values reported in We addressed the patient to laparoscopic surgery, debulking the endometriotic-like tissue. A contemporary ureteroscopy and ureteral stenting was performed.Histopathological findings suggested a diagnosis of endometriosis.μm sections for the histological examination with haematoxylin and eosin stain. Microscopically, variably sized endometrial-type glands lined by a columnar epithelium embedded in an endometrial-like stroma were evident within muscular tissue , as a result of continuous pacing by yet unknown stimuli .In the case that we have previously described, we hypothesize that endometriotic focus on left lumbar ureteral may be derived from endometrial debris refluxed by retrograde menstruation, or via uterine vessel circulation. According to this way of developing, it is quite uncommon that we have not found any other endometriotic implants in the peritoneum or in other pelvic sites, nor fibrosis and adhesions between pelvic organs. For this reason, another possible hypothesis to explain the isolated left lumbar ureteral endometriosis (that we observed) is that it could be due to Müllerian-derived progenitor cells that, after certain stimuli, evolved to form the typical implant.Depending on location and extension of endometriotic implant, we could summarily divide among superficial peritoneal, ovarian, and deep infiltrating endometriosis (DIE); this last form, characterized by infiltration for more than 5 mm beyond the wall of the pelvic peritoneum, usually involving uterosacral ligaments, rectovaginal spaces, the upper third of the posterior wall of the vagina, the bowel, and urinary tract  is reporOur case is according to that described by Traşcă et al. , because


Mirizzi’s syndrome is a rarely observed disorder that presents with obstructive jaundice. The condition is caused by a stone impacted in the gall bladder neck or cystic duct that impinges on the common hepatic duct, with or without a cholecystocholedochal fistula. The condition is often confused with other serious conditions such as hilar cholangiocarcinoma, which present with similar clinical and imaging findings, and a pre-operative diagnosis may be a serious challenge.We present the case of a 44-year-old Asian man with Mirizzi’s syndrome who was initially diagnosed as having cholangiocarcinoma based on his clinical presentation, raised cancer antigen 19–9 levels and radiological findings. Our patient was diagnosed as having Mirizzi’s syndrome intra-operatively and subsequently a cholecystectomy was performed with restoration of biliary drainage. Careful clinical assessment during surgery with the help of intra-operative frozen section helped in establishing the definitive diagnosis and altered the surgical procedure for our patient.Pre-operative diagnosis of Mirizzi’s syndrome could be challenging as the clinical, biochemical and radiological presentation is similar to other conditions causing obstructive jaundice such as choledocholithiasis, bile duct stricture or cholangiocarcinoma. A high index of suspicion and careful surgical assessment may help in establishing a diagnosis and alter the clinical course for our patient. Mirizzi’s syndrome (MS) is a rarely observed condition characterized by impaction of stones in the neck of the gall bladder or cystic duct, causing mechanical obstruction of the common hepatic duct and presents clinically as intermittent or persistent jaundice. This syndrome was first described in 1948 by an Argentinean surgeon, Pablo Mirizzi ,3. We reA 44-year-old Asian man, an agriculturalist by occupation, presented to a local hospital with a four-day history of upper abdominal pain and vomiting. He also had history of progressively increasing jaundice for the last two months. He had significant comorbid conditions including hypertension and ischemic heart disease for which he underwent a coronary artery bypass grafting two years previously. A clinical diagnosis of cholangitis was made at presentation. He was resuscitated and an abdominal ultrasound (US) followed by endoscopic retrograde cholangiopacreaticography (ERCP) was performed. ERCP revealed a tight stricture involving the proximal bile duct extending to the confluence of right and left hepatic ducts Figure . A plastIn order to delineate the underlying pathology, further investigation was performed. His CA19-9 levels were found to be elevated at 71.10U/mL . A computed tomography (CT) scan of his abdomen showed moderately dilated intra-hepatic ducts up to the porta hepatis, soft tissue thickening involving the gall bladder neck and proximal bile duct and a few enlarged upper abdominal nodes. The findings were suggestive of neoplastic lesion involving the gall bladder neck and proximal common bile duct causing bile duct stricture Figures  and 3. TSix weeks after his initial presentation, our patient was scheduled for resection of the tumor. On surgical exploration, a thick-walled gall bladder was seen with a large stone impacted in the cystic duct causing external compression on the common hepatic duct (Mirizzi’s syndrome). After mobilization of the gall bladder, an intra-operative cholangiogram showed some decrease in distensibility of the bile duct, but no filling defect, tumor or stricture was identified Figure . In viewMirizzi’s syndrome is a rare condition characterized by presence of a common hepatic duct obstruction caused by an extrinsic compression of an impacted stone in Hartmann’s pouch or the cystic duct. It presents in approximately 0.35% of cholecystectomies performed . PredispPatients usually present with clinical and biochemical signs of biliary obstruction, sometimes in the setting of an acute cholecystitis, acute cholangitis or pancreatitis. There is usually a chronic history of biliary symptoms and CA19-9 levels may be moderately elevated, as seen in our patient. However there have been a few case reports where very high levels of CA19-9 along with other suggestive radiological findings have been thought of as cholangiocarcinoma ,8,9. In Whether CA19-9 should be used in the clinical diagnostic investigation of patients with biliary tract diseases still remains a difficult question to answer. CA19-9 is synthesized from normal human pancreatic and biliary ductal cells. Because the exact pathway between tissue and blood is unknown, the actual mechanism for elevated serum CA19-9 concentration is uncertain. It seems that extremely high and continuously increasing CA19-9 levels, together with well assessed clinical information, may point towards neoplasia . HoweverPre-operative diagnosis of Mirizzi’s syndrome is crucial in order to avoid complications of unrecognized cholecystobiliary or cholecystoenteric fistulas and damage to the common hepatic duct during surgery. For this reason, the pre-operative diagnosis requires the use of combined imaging modalities such as US, CT, ERCP, and percutaneous transhepatic cholangiography (PTC). US or CT scans are not often definitive, although both may demonstrate findings that strongly suggest a diagnosis, such as: (1) dilatation of the biliary tree above the level of the gall bladder neck, (2) impaction of a stone in the gall bladder neck, and (3) abnormal caliber common bile duct below the level of impaction. All these findings may also be present in obstruction caused by a neoplastic lesion; hence specificity remains an issue. The radiological appearance of this condition may be misinterpreted as a tumor of the gall bladder or cystic duct, a cholangiocarcinoma, metastatic disease of the hilum or acute cholecystitis . A similERCP is considered an effective pre-operative method for diagnosing the condition in these patients and can provide a relatively accurate localization and characterization of the cause of the biliary obstruction. Typical findings of Mirizzi’s syndrome at ERCP include (1) mid-bile duct obstruction with dilated proximal common hepatic duct and intra-hepatic ducts combined with normal duct caliber distal to the obstruction, (2) insertion of the cystic duct at the point of obstruction and/or complete obliteration of the cystic duct, and (3) a stone visualized at the point of obstruction either within the cystic duct or the common duct . If a stAlthough diagnostic imaging techniques have been perfected, pre-operative diagnosis of Mirizzi’s syndrome is not an easy task and continues to be a challenge for the surgeon. Therefore, even intra-operative cautious recognition of the condition and application of the appropriate surgical judgment according to the characteristics of each case will lead to successful treatment. Surgical intervention remains the definitive treatment for the majority of patients and should satisfy three goals: extraction of the obstructing stone, removal of the gall bladder, and restoration of normal biliary drainage. An intra-operative cholangiogram should be obtained for clarification and confirmation ,14. DepeDespite improvements in clinical, biochemical and radiological investigation techniques, pre-operative diagnosis of Mirizzi’s syndrome remains a challenging task. The condition can easily be confused with choledocholilithisis, bile duct stricture or cholangiocarcinoma because of the close resemblance of radiological findings and may be overlooked due to the rarity of the condition. A high index of suspicion and careful surgical assessment may help in establishing a diagnosis and alter the clinical course for the patient. In our patient, the diagnosis of Mirizzi’s syndrome was made intra-operatively and a cholecystectomy was performed with extraction of the stone and temporary drainage of the biliary system. Our patient recovered completely with no complications within a few days.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MRK conceived the study and interpreted the data. SR collected the data and prepared the initial manuscript. The manuscript was reviewed and approved by all the authors.


An abdominal pseudocyst is a rare complication of a ventriculo-peritoneal shunt. Etiological factors include infection, obstruction and dislodgement. This is the first report of a hepatic cerebrospinal fluid pseudocyst mimicking hydatid liver disease.We report the case of an 18-year-old Caucasian male patient who presented with a hepatic pseudocyst secondary to a ventriculo-peritoneal shunt, misdiagnosed as hydatid disease of the liver.in situ.Hepatic pseudocysts, a rare complication of a ventriculo-peritoneal shunt, have similar clinical and radiological characteristics to those of hydatid liver disease. The formation of a pseudocyst should always be considered in patients with ventriculo-peritoneal shunts  An abdominal pseudocyst is a rare complication of a ventriculo-peritoneal shunt. Such cysts may cause diagnostic problems in regions such as the Middle East, where echinococcosis disease of the liver is endemic, due to similarities in clinical presentation and radiological appearance.An 18-year-old Caucasian male patient presented with a 10-day history of generalized tonic-clonic seizures. His past medical history included right ventriculo-peritoneal (VP) shunt insertion at two weeks of age for bacterial meningitis complicated by hydrocephalus. Four years prior to his current admission he had presented with abdominal pain and a computed tomography (CT) scan of his abdomen at that time was interpreted as being consistent with a right hepatic hydatid cyst (8 × 6 cm). Serology workup was negative for hydatid disease at the time of the CT scan, however, due to the characteristic radiological findings, antihelminthic treatment  was commenced and he was subsequently lost to follow-up.During his current admission, he underwent a CT scan of his brain and abdomen, which revealed an increase in the size of the cerebral ventricles and an increase in the size of the liver cyst11 × 9 cm), revealing the presence of the VP shunt tip inside the cyst  test was negative does not rule out hydatid disease however, in retrospect, would make a peritoneal cyst or pseudocyst as likely. The sensitivity and specificity of the IHA are 86.7% and 95% respectively . Only a The suspicion of an abdominal pseudocyst is often made at the time of physical examination and on the basis of conventional radiology . VisualiThe standard treatment of a hepatic pseudocyst secondary to catheter tip migration, in cases with no infection or prominent inflammatory reaction in the peritoneal cavity, should be simple repositioning of the peritoneal catheter in the abdominal cavity. This procedure may be combined with percutaneous or open drainage in resistant cases . Howeverin situ and can be easily treated by simple repositioning.We report a case of a hepatic CSF pseudocyst secondary to the migration of a VP shunt, mistaken for hepatic hydatid disease with serious sequelae. The formation of a pseudocyst should always be considered in patients with VP shunts CNS: central nervous system; CSF: cerebrospinal fluid; CT: computed tomography; IHA: indirect hemagglutinin; VP: ventriculo-peritoneal.The authors declare that they have no competing interests.WF drafted the manuscript; HHA and DM participated in the design of the study; MK participated in the design and coordination of the study. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Ganglioneuroblastoma is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system, and is found mostly, but not exclusively, in babies and young children.To the best of our knowledge, there have been no previously reported cases of ganglioneuroblastoma of the mediastinum from Pakistan. We present a case of ganglioneuroblastoma in an eight-year-old Pakistani Sindhi boy incidentally found to have a large posterior mediastinal mass that on biopsy initially looked like ganglioneuroma. He underwent successful excision of the mediastinal mass and remained stable post-operatively. Final pathology findings showed a ganglioneuroblastoma. He has remained free of symptoms on long-term follow-up.The rarity of this tumor along with its almost exclusive occurrence in the pediatric population necessitates a thorough investigation of patients presenting with a symptomatic mass. Ganglioneuroblastoma is a rare variety of peripheral neuroblastic tumor (neuroblastoma) that can arise anywhere along the sympathetic nervous system. It occurs almost exclusively in the pediatric population, with some reported cases in the adult population. It is the third most common childhood malignancy after leukemia and brain tumors, and is the commonest solid extracranial tumor among children . Its truAn eight-year-old otherwise previously healthy Pakistani Sindhi boy presented to our clinic with complaints of right-sided lumbar pain for a week. At initial clinical assessment our patient's weight was 36.3 kg and his height was 142 cm. His overall nutritional status was good and had a normal height and build for his age with no history of weight loss. He had no other associated comorbidities. His family history was significant for diabetes mellitus, hypertension and untreated pancreatic cancer and renal cancer. The rest of his history and test results were unremarkable. A physical examination was grossly unremarkable. Ultrasound of the abdomen was performed, with normal results. A chest X-ray was performed that showed a homogenous soft tissue density mass in the posterior mediastinum with no evidence of rib erosion. To evaluate the mass further, an MRI was performed -guided biopsy of the mass was advised, which was carried out under general anesthesia. No complications were observed after the procedure and a post-procedural CT scan did not reveal any pneumothorax. Histopathology of the biopsy material showed multiple cones of tissue exhibiting spindle-shaped cells arranged haphazardly, having moderate cytoplasm and wavy spindle shaped nuclei. Scattered mature ganglion cells were also identified, characterized by abundant cytoplasm and round to oval nuclei; as such no immature element was identified nor was there any evidence of increased mitosis or areas of necrosis. The specimen was also treated with immunohistochemical stains and was found to be positive for S-100, synaptophysin, chromogranin, and leucocyte common antigen (LCA). These morphological and immunohistochemical findings were consistent with a diagnosis of ganglioneuroma, since no malignant features were identified.In addition, whole body skeletal scintigraphy was performed with Tc-99 m for metastatic investigation, which showed bilateral symmetrical tracer distribution in both the axial and appendicular skeleton with no areas of abnormal tracer accumulation and normal excretion of the tracer from both kidneys and urinary bladder.Bone marrow aspirate and trephine from the iliac crest performed for staging of the tumor showed all three cell lines with normal erythroid and myeloid precursors. The specimen was stained with hematoxylin and eosin and a few atypical mononuclear cells were noted; however, the sample was inadequate showing mainly cartilage, muscle and clot, and was hence non-diagnostic. There was nothing to suggest malignancy or granuloma in the sections examined. It was suggested to repeat an adequate length bone marrow trephine if there was a clinical indication to do so.Surgery was planned for resection of tumor. Prior to that, pre-operative evaluation included a normal complete blood count, coagulation profile and serum lactate dehydrogenase levels. Surgery was approached using left thoracotomy. After entering the chest cavity, tumor was excised from the aorta, subclavian vessels and vertebral column. After adequate hemostasis, chest tubes were placed and routine closure of thoracotomy was carried out. The resected mass measuring 9.5 × 7 × 4.5 cm was sent for histopathology that confirmed the pre-operative diagnosis and presence of tumor-free margins.Post-operatively, our patient remained hemodynamically stable. He received intravenous antibiotics and epidural marcaine infusion for pain relief, which was switched over to oral antibiotics and intravenous analgesics. Our patient was running a fever by the third post-operative day, which was followed by a transient episode of desaturation; however this improved without consequence. A post-operative chest X-ray was also unremarkable. On the fourth post-operative day our patient was discharged from the hospital on oral augmentin and non-steroidal anti-inflammatory drugs (NSAIDs) for pain. At discharge, the wound was clean, healing and healthy.At follow-up a week later our patient's condition was stable. The final post-resection histopathology report showed a circumscribed and partially capsulated neoplasm composed of spindle cells arranged irregularly in short fascicles, scattered ganglion cells and abundant cytoplasm. Some nodules within the mass showed small, round neoplastic cells, scant cytoplasm, increased nuclear to cytoplasmic ration and hyperchromatic nuclei. The mitotic rate was identified as two to three cells per high-power field and the mitosis karyorrhexis index (MKI) was < 2%. Focal areas of hemorrhage and necrosis were also identified. Sections of tissues were also stained with immunohistochemical stains and were found to be positive for neurofilament, synaptophysin and chromogranin. The features showed that the tumor was a ganglioneuroblastoma, nodular type, which are composite Schwannian stroma-rich/stroma-dominant and stroma-poor element.At a year later our patient had complaints of some bone pain for which a skeletal survey was performed and was found to be within normal limits. Our patient has thereafter remained stable.Ganglioneuroblastoma is an uncommon peripheral neuroblastic tumor. The International Neuroblastoma Pathology Classification, popularly known as the Shimada system, grades these into four distinct categories based upon the histopathological balance between neural-type cells  and Schwann-type cells (Schwannian-blasts and mature Schwann cells). These include neuroblastomas (Schwannian stroma-poor), ganglioneuroblastoma intermixed (Schwannian stroma-rich), ganglioneuroma (Schwannian stroma-dominant) and ganglioneuroblastoma nodular (composite Schwannian stroma-rich/stroma-dominant and stroma-poor) and neuroblastic tumors unclassifiable .Ganglioneuroblastoma have intermediate malignant potential, between that of neuroblastomas and ganglioneuromas. Histologically, they are considered malignant because they contain primitive neuroblasts along with mature ganglion cells. In contrast, their benign counterparts, ganglioneuromas are fully differentiated tumors that contain all mature cell types but lack the immature elements (such as neuroblasts), atypia, mitotic figures, intermediate cells, or necrosis .Ganglioneuroblastomas occur with equal frequency in both the genders and most commonly in babies and in young children, with occurrence after 10 years of age being extremely rare . They ocWhen dealing with pediatric masses, differentials such as rhabdomyosarcomas, Wilms tumor, germ cell tumors, and so on, should be taken into consideration. Rhabdomyosarcoma is the most common soft tissue sarcoma in children and usually originates in the head and neck (28%), extremities (24%), and genitourinary (GU) tract (18%). The cause is unclear; however, several genetic syndromes such as neurofibromatosis, Li-Fraumeni syndrome, and so on, are associated with it. Symptoms usually depend on the location and the tumours usually present as an expanding mass. Wilms tumor, by contrast, is the most common cancer of the kidneys in children. The majority are unilateral, encapsulated, vascularized, and usually do not cross the midline of the abdomen. They can go undetected early on because the tumor can grow large without causing pain. Children usually present with abdominal swelling or blood in the urine.Patients with ganglioneuroblastoma often present clinically with pain caused by either the primary tumor or by metastatic disease. Patients with mediastinal tumors can present with stridor and shortness of breath secondary to tracheal deviation or narrowing. Large thoracic tumors can cause mechanical obstruction resulting in superior vena cava syndrome. Nerve or nerve root compression by the mass can result in peripheral neurological signs. Patients with cervical masses can present with Horner's syndrome . HoweverHistological confirmation is required for definitive diagnosis. Tissue is obtained by incisional biopsy of the primary tumor or bone marrow trephine/aspirate in patients suspected of having metastatic disease in the bone marrow.The most common site for metastasis in ganglioneuroblastomas is bone, which may mean patients present with limping and unexplained irritability (Hutchinson's syndrome). Another site of metastasis is the liver. Pepper syndrome is the presence of large liver metastases in babies such that intra-abdominal pressure becomes so high that there might be possibility of respiratory compromise. In children younger than a year old, skin metastases are common, which are darkly pigmented masses resembling blueberries  . In our A CT scan is the imaging modality of choice to evaluate neuroblastic tumors. There is enough evidence to suggest that it is superior both in terms of determining tumor size and other characteristics including organ of origin, tissue invasion, vascular encasement, lymphadenopathy, and calcifications .The prognosis for patients with localized disease and younger age is better; several screening programs were developed for the detection of neuroblastoma in infancy by measuring urinary catecholamines  but wereAge is taken into account when defining neuroblastoma histology as favorable or unfavorable. The younger the age at diagnosis, the better the survival rate  with chiMediastinal neuroblastomas are better in terms of prognosis than abdominal neuroblastomas in that patients with the former tend to present earlier when the size is still small, and hence complete resection of the tumor is possible . CompletDifferential markers for distinguishing ganglioneuromas from ganglioneuroblastomas at present are not available; immunohistochemical stains with antibodies such as neurofilament, synaptophysin, chromogranin, s-100 and LCA are generally positive in both ganglioneuromas and ganglioneuroblastomas . HoweverThe rarity of ganglioneuroblastoma, along with its occurrence in the pediatric population, warrants a great deal of suspicion when a younger patient presents with a symptomatic mass. Given the wide variety of clinical symptoms the tumor can present with, it is essential that neuroblastoma remains as one of the differential diagnoses while working on such a case.Written informed consent was obtained from the patient's next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SHF analyzed our patient's details and was primarily responsible for obtaining the full investigation results, including the surgery performed on our patient. SAB was involved in assisting the primary faculty, obtaining relevant details about the case and confirming its rarity, and was a major contributor to writing the manuscript. AA contributed a significant effort to writing the manuscript and editing the final draft. All authors read and approved the final manuscript.


Giant osteomas of the ethmoid and frontal sinuses ary very rare, with only a few dozen cases reported in the literature. Given their rarity, the clinical characteristics and treatment of this disease remain controversial. In this study, the clinical presentation and surgical methods used to treat three patients with giant osteomas of the ethmoid and frontal sinuses are described, combined with a review of the literature from 1975 to 2011. In total, 45 patients with giant osteomas arising from the ethmoid and frontal sinuses (including the present cases) have been reported in 41 articles. Headache and ocular signs are the most common symptoms. This disease often leads to intracranial or intraorbital complications. The main treatment for giant osteoma is surgery via an external approach. The outcome of surgery for giant osteoma is good, with rare recurrence, no malignant transformation and few persistent symptoms. Osteomas occur predominantly in the head and neck region, particularly in the mandible and paranasal sinuses . These cThe patient was a 20-year-old male with a 5-year history of hypopsia and ptosis of the left eye. The patient had no associated symptoms, such as epistaxis, rhinorrhea, diplopia or nasal obstruction. He had no history of surgery or trauma and was in good health. An endoscopic examination found no obvious lesion in the nasopharynx or nasal cavity. Computed tomography (CT) revealed a multilobulated 4.0×3.5-cm mass of osseous density occupying the left ethmoid sinus that extended into the left orbit and base of the left frontal sinus. The lesion had displaced the lamina papyracea and compressed the left optic nerve . The iniAn endoscopic intranasal ethmoidotomy was performed under general anesthesia. Intraoperatively, the lesion was seen to arise from the anterior ethmoid sinus and abutted the lacrimal sac; the margin in the coronal plane extended from the frontal recess to the maxillary ostium. A biopsy of frozen tissue was performed and the histopathological examination confirmed the diagnosis of osteoma. An electric drill was used to resect the tumor in the nasal cavity. However, the anterolateral margin of the osteoma extending into the orbit was not visible. A 3-cm incision was therefore made between the inner canthus and anterior aspect of the nasal bones to expose the anterolateral margin of the tumor. The osteoma was removed using a drill and curette via an external approach combined with intranasal endoscopy. The left periorbital region was intact but was obviously displaced by the osteoma. After removal, the giant osteoma was found to measure ∼4×3×2 cm. A CT performed 1 week postoperatively revealed no evidence of the osteoma . PostopeA 22-year-old male presented with a 3-month history of pain over the region of the nasal bone but no other symptoms. A systemic examination was unremarkable. Endoscopy showed no abnormal findings in the nasal cavity. CT revealed a giant bone tumor filling the right frontal sinus and pushing into the left frontal sinus .Surgery was performed under general endotracheal anesthesia. A bicoronal frontal flap was raised with a skin incision along the hairline. Using a drill and bone shears, a frontonasal bone flap was raised. An irregular 6-cm bone-like lesion arose from the roof of the right frontal sinus and pushed against the septum. An intraoperative biopsy of a frozen section showed an osteoma of the frontal sinus. The osteoma was resected completely using a drill and curette. The nasofrontal duct was unblocked. The dura was adherent to the tumor and breached the posterior roof with a cerebrospinal fluid (CSF) leak. Two layers of periosteum and xanthan gum were applied to repair the dura. A bone channel was made in the anterior wall of the frontal sinus, in which the frontal bone flap was inserted to reconstruct the calvarium. Postoperatively, antibiotics were given for 1 week. The patient’s clinical course was uneventful and the nasal root pain was relieved. Three months postoperatively, CT revealed total resection of the osteoma . The patA 62-year-old female presented with a 30-day history of a right-side headache. The patient was first examined in the Department of Neurology and was then referred to the Department of Otolaryngology for treatment. The patient had no associated rhinorrhea, epistaxis or nasal obstruction. A systemic examination revealed good health with no evidence of lymphadenopathy or hepatosplenomegaly. The patient had no history of surgery or trauma. An endoscopic examination showed no lesion in the nasal cavity. CT of the sinuses revealed a 3.5×3-cm extremely dense bony mass with inflammation in the right frontal sinus approaching the edge of the sinus .en bloc using a high-speed drill and curette via intranasal endoscopy. The histopathological inspection confirmed the diagnosis of osteoma. The patient was discharged on the fourth postoperative day and has been disease-free for 7 months.Considering the large size and location of the tumor in the CT images, the disease may have resulted in fatal complications. The patient was admitted for excision of the tumor. An endoscopic Draf type IIb frontal sinusotomy was performed. The tumor was removed Osteomas are benign tumors often involving the paranasal sinuses, especially the frontal and ethmoid sinuses . They usThe literature on giant osteomas of the ethmoid and frontal sinuses from 1975 to 2011 was reviewed using the keywords ‘osteoma and ethmoid sinus’ or ‘osteoma and frontal sinus’, and 45 patients with giant osteomas in 41 articles were identified (including the cases presented here)  5–44). . 44). Thet al had no history of sinusitis or trauma and no obvious etiology. Four patients (8.9%) had a history of surgery, three (6.7%) had a history of head trauma and one (2.2%) had chronic sinusitis. It was postulated that trauma, especially surgical, might be a cause of giant osteomas. In this series, one patient’s father also had an osteoma of the sinuses and underwent surgery as a youth. Given the large number of asymptomatic undiagnosed osteomas, hereditary factors need to be considered in the etiology of this disease. Some authors have reported that osteomas of the paranasal sinuses are usually accompanied by nasal polyps. They inferred that these two diseases might have a similar pathogenesis but only one patient had nasal polyps in the current review. This implies that giant osteomas of the ethmoid and frontal sinuses have no relationship with nasal polyps.Osteomas may also be a part of Gardner’s syndrome, an autosomal-dominant hereditary disorder characterized by the clinical triad of intestinal polyposis, osteomas and cutaneous and soft tissue tumors . In affeGrossly, osteomas are round or oval, hard, yellowish-white and well-circumscribed and are attached to the adjacent bone by a broad base or occasionally a small stalk . HistoloOsteomas of the paranasal sinuses should be differentiated from osteosarcoma, osteoblastoma and fibrous dysplasia using their radiological and pathological features. The most important entity in the differential diagnosis is well-differentiated parosteal osteosarcoma. The neoplastic trabeculae of woven bone in parosteal osteosarcoma are separated by a cellular fibrous stroma that contains occasional mitotic figures. These features are not seen in osteomas .The main treatment for symptomatic osteomas of the sinuses is surgery using an endoscopic or external approach. Considering the slow growth and rarity of recurrence, some authors suggest incomplete resection of the tumor. However, the treatment of asymptomatic cases remains controversial. A conservative approach with regular radiological examinations is usually recommended in asymptomatic cases . SurgeryGiven their size, the giant osteomas in this study were all symptomatic and most caused intracranial or intraorbital complications. The classical method for removing frontal and ethmoid sinus osteomas is still considered to be surgery via an external approach, including bicoronal scalp flap surgery, frontal craniotomy or a lateral rhinotomy . In 35 cen bloc resection of these giant osteomas is a new method and is possible depending on the location and size of the osteoma. The outcome of surgery for giant osteoma is good, with rare recurrence, no malignant transformation and few persistent symptoms.Giant osteomas of the ethmoid and frontal sinuses are rare, with a male predominance and may lead to symptoms and intracranial or intraorbital complications. Headache and ocular signs are the most common symptoms. The main treatment for giant osteoma is surgery via an external approach. Endoscopic


Intrathoracic schwannomas are most frequently located in the posterior mediastinum. A Chinese woman presented with a benign pericardial schwannoma in the pretracheal space and aortopulmonary window, a location which has not been described previously in the literature.2 lobulated mass with sharp margins that presented as a capsule with heterogeneous enhancement and punctate calcification. Complete surgical resection was performed using a thoracotomy approach. The resected intrapericardial tumor was a firm, large mass with lobulation. Capsulation prevented infiltration of the mass into adjacent organs. Pathological examination verified that the tumor was a benign pericardial schwannoma.A 50-year-old Chinese woman initially reported a cough associated with a small amount of sputum. Contrast-enhanced computed tomography (CT) subsequently revealed a 9 × 11 cmThis is the first reported case of a benign pericardial schwannoma located in the pretracheal space and aortopulmonary window. While a contrast-enhanced CT scan was able to differentiate this pericardial schwannoma from other middle mediastinal tumors, the exact diagnosis and plan for treatment depended on a pathological examination. For similar cases involving pericardial schwannomas, complete surgical resection is recommended, particularly for the prevention of life-threatening cardiopulmonary complications. Intraet al. . In thatA 50-year-old Chinese woman reported the presence of a cough associated with a small amount of sputum for the previous month, and a fever over the previous week. A physical examination and laboratorytests showed no abnormal findings.2 lobulated mass exhibiting heterogeneous enhancement and punctate calcification with sharp margins was observed. The capsule of the mass was enhanced in the CT images. The mass was located in the pretracheal space and aortopulmonary window (Figure When a posteroanterior chest radiograph was obtained, a widened mediastinum, a mass protruding from the pulmonary segment were detected Figure . Using cComplete surgical resection was performed via a thoracotomy approach. The resected intrapericardial tumor was large in size and firm. The tumor was situated under the ascending aorta, with its right edge adhered to SVC, and was compressing the SVC and right atrium. The upper edge of the tumor was adjacent to the aortic arch, the left edge was attached to the pericardium and left hilus of lung, the lower edge was adhered to left atrium and pulmonary artery, and the posterior edge was adjacent to the trachea, primary bronchi and the descending thoracic aorta, The tumor compressed the trachea and was pushing the primary bronchi toward the vertebral column. The tumor exhibited lobulation and capsulation Figure A. CorresHistological examination revealed that the tumor was made up of two tissue types: Antoni A and Antoni B. The Antoni A type tissue was composed of spindle cells that were closely packed together and arranged in bundles and rows with palisading nuclei without mitoses Figure B. The AnThe patient’s general health was good. She exhibited no evidence of recurrence at a follow-up CT scan performed 5 months after the surgical resection.Primary schwannomas arising from the pericardium are extremely rare. Therefore, the present case of a benign pericardial schwannoma located in the pretracheal space and aortopulmonary window is of great interest. Moreover, to our knowledge, this case represents the first of its type to be reported.Since chest radiography does not establish a specific diagnosis of pericardial schwannoma, CT scans are used for precise localization and determination of the tumor’s approximate size and internal characteristics, as well as its proximity to surrounding structures ,5. In thThe benign pericardial schwannoma should be differentially diagnosed from other tumors that occur in the pretracheal space and aortopulmonary window, such as bronchial cysts, lymphomas, and metastatic lymphadenectasis. Bronchogenic cysts appear as single, smooth, round or elliptic non-enhanced masses with homogeneous attention. Lymphomas can manifest as multiple, rounded, and conglomerate soft-tissue masses with the capacity to infiltrate or invade neighboring vascular structures. Metastatic lymph nodes have solitary or multiple round soft tissue shadows in CT scans, can be integrated into clusters, and may be oppressing or infiltrating adjacent organs. Necrosis sometimes occurs in the nodes and patients with metastatic lymph nodes often have a history of malignant tumors.In most tumor cases, the exact diagnosis and plan for treatment are dependent upon pathological findings. Microscopically, the tumor in our patient was consistent with the typical schwannoma composition, including both hypercellular Antoni A and hypocellular Antoni B areas. Likewise, immunohistochemically, the tumor in our patient was strongly positive for S-100 protein, a schwannoma marker -9, and tIn conclusion, this is the first reported case of a benign pericardial schwannoma located in the pretracheal space and aortopulmonary window. Although a contrast-enhanced CT scan enabled us to differentiate this pericardial schwannoma from other middle mediastinal tumors, the exact diagnosis and plan for treatment depended on the pathological findings. For similar cases involving pericardial schwannomas, complete surgical resection is recommended, particularly for the prevention of life-threatening cardiopulmonary complications.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent form is available for review by the Editor of this journal.CT: Computed tomography; SVC: Superior vena cava.The authors declare that they have no competing interests.ZXH performed data analyses and wrote the manuscript. ZXH, WY, QXY, and LB conducted the clinical diagnosis and data collection. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2261/13/45/prepub