Leiomyomas of esophagus, although rare, are the most frequent benign tumors of esophagus. Aim of this study is the presentation of 7 patients with esophageal leiomyomas who underwent surgical treatment during a 9-year period.Epidemiological data , the presenting symptoms, diagnostic examinations, tumor location, histopathological findings and the safety and efficacy of surgical resection are analyzed and assessed.5 men and 2 women with mean age of 56.9 years were operated. In 3 cases the tumor was located at the lower esophagus, while in the other 4 cases, the leiomyoma was found at the median third of esophagus. 4 patients had severe symptoms related to the leiomyoma, such as dysphagia and epigastric pain. All patients underwent a right postolateral thoracotomy with enucleation of the lesion. None of them received resection of part of the esophagus. The mean diameter of the resected tumors was 4.3 cm. The dimensions of leiomyomas were immediately associated with the symptoms. In no case was detected malignancy or recurrence. All patients were relieved from their symptoms, while postoperative morbidity and mortality did not occur.Esophageal leiomyoma is a benign tumor, which causes symptoms only if its size becomes large. Surgical enucleation is considered to be safe and effective, without complications. The esophageal leiomyoma is a benign tumor of the esophagus. Other non-malignant lesions of esophagus are hemangioma, lymphangioma, squamous papilloma, fibrovascular polyp and granular cell myoblastoma. The incidence of this kind of lesions is referred to be almost 1% of the esophageal neoplasms in the international literature . It is iThis study is a retrospective analysis of the medical records of patients, whose diagnosis was a possible esophageal leiomyoma. The study took place at the Theagenio Cancer Hospital of Thessaloniki from September 2000 to September 2009. Seven patients were detected. The epidemiological data , presenting symptoms, diagnostic examinations, tumor location, histopathological findings and the safety and efficacy of the surgical resection were analyzed. The standard examinations included preoperative esophagogastroscopy, endoscopic ultrasonography and computed tomography of the chest. Fine needle aspiration was not performed in any case.Patients' group was consisted of five males and two females. Their age ranged from 48 to 67 years (mean age: 56.9 years). The most common symptoms were dysphagia and epigastric pain, which were present in four cases. In addition, one patient was complaining for retrosternal burnings. The rest of the cases had limited symptomatology, like unspecified discomfort located at the chest or the upper abdomen. All patients were subjected to the standard examinations, which demonstrated an esophageal tumor with features compatible with leiomyoma. In three cases the tumor was located at the lower third of the esophagus, while four lesions were detected at the median third. In all cases a right postolateral thoracotomy carried out and myotomy of esophagus with enucleation of the neoplasm took place. Frozen sections showed typical leiomyoma of esophagus. None of the patients underwent resection of part of the organ. The mean diameter of the resected tumors was 4.3 cm. Malignancy or recurrence was not detected. The mean in-hospital staying was 7 days. Complications did not occur. All patients were relieved from their symptoms, after surgical removal of the tumor. Postoperative follow-up did not reveal any morbidity or mortality related to the primary diagnosis. Clinical presentation, diagnostic findings and management of the patients are summarized in table Leiomyomas belong to benign mesenchymal tumors of esophagus. They are the most common non-malignant lesions of esophagus, with an incidence approaching 60% of all benign tumors of the organ . The symLeiomyomas can mimic cancer of esophagus. Lack of specific symptoms as well as the similarity in initial clinical expression may cause diagnostic confusion. It is, therefore, obligatory the full preoperative investigation of each patient complaining for symptoms possibly relating with an esophageal lesion. Esophagogastroscopy combined with endoscopic ultrasonographic evaluation of the tumor is mandatory in order to exclude cancer of esophagus from the differential diagnosis ,5. LeiomEvery symptomatic leiomyoma should be excised. In case the tumor is discovered accidentally, some authors recommend regular follow-up with barium swallow and endoscopy . Our polDifferent kinds of approaches have been described depended from the location of the tumor. In the majority of the cases the tumor is discovered at the mean or the distal third of the esophagus. Right thoracotomy is suggested in first case; while a left thoracoabdominal approach fits better the second case . Our expHistopathologicaly, the tumor is composed of bland spindle cells and demonstrates low to moderate cellularity. The cells have eosinophilic and fibrillary cytoplasm Fig . MitoticIn conclusion, leiomyoma is a rare benign tumor of esophagus. Correct preoperative evaluation is of great importance in planning of the surgical excision. Enucleation of the lesion using a right postolateral thoracotomy is the most common approach. Postoperative complications are rare, while morbidity and mortality rates tend to be zero universally. Patients' relief from the symptoms is the rule and the prognosis is expected great.The authors declare that they have no competing interests.CA, DK, ML, AK, KS, and GZ took part in the care of the patients and contributed equally in carrying out the medical literature search and preparation of the manuscript. NB participated in the care of the patients and had the supervision of this report. All authors approved the final manuscript.Written informed consent was obtained from all patients for publication of this article and accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


Acquired gastric outlet obstruction (GOO) during infancy beyond the neonatal period is a very rare condition when other congenital causes like infantile hypertrophic pyloric stenosis, antral diaphragm, pyloric atresia etc are excluded. We report an unusual case of 6 month old male child who presented with recurrent episode of vomiting not relieved by medication. On gastrograffin study there was pre pyloric stricture of unknown etiology and was managed by stricturoplasty. We are reporting this case because of its rarity and with excellent outcome if diagnosed and managed properly. Even on extensive search of English literature we are not able to find a single report of this lesion in infants. Gastric outlet obstruction (GOO) in infancy and childhood may result from congenital causes , or acquired causes  ,4. AmongA 6 month old male child weighing 4.2 kg presented to our institute with continuous projectile nonbilious vomiting for the last two months. The frequency of vomiting was gradually increased in last one month. The baby was on medical treatment (ante-emetics) but the frequency of vomiting was not controlled. There was no history of caustic ingestion & analgesic intake. The child lost significant weight during this period. On physical examination child was lethargic and severely dehydrated. On examination there was fullness in epigastrium with visible peristalsis. Palpation does not reveal any added finding. On laboratory investigation the child was anemic with hypokalemic hyponatraemic alkalosis. Blood urea and serum creatinine was within normal limit. Child was managed with nasogastric aspiration and intravenous fluid and antibiotics till the serum electrolyte correction.Plain x-ray abdomen showed air fluid level figure . The uppThe diagnosis of prepyloric stricture was made and baby was planned for elective laparotomy. On exploration the stomach was distended with stricture in prepyloric region which was covered by greater omentum figure . LongituGastric outlet obstruction (GOO) is a classic indication for surgery in complicated peptic ulcer disease.  The gastAlthough there are few reports of GOO due to prolapsing gastric polyp in adults, but no report of GOO secondary to antral mass combined with gastric ulcer was found in pediatric age group . SometimThere are several rare causes of GOO in children that may present with a variety of symptoms. Evaluation of such patients may require a wide range of diagnostic studies. Different therapeutic modalities based on the specific cause and degree of obstruction is usually needed to manage this heterogeneous group childrenThe authors declare that they have no competing interests.SPS and PS operated the patient and reviewed the literature. VDU and RJ were the main writers of the manuscript, where as ANG and VK moderated the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the parents of 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.


Germ cell tumor of the pituitary gland is a very rare occurrence.We describe the case of a 28-year-old Malaysian Malay woman with lupus nephritis who complained of a three month headache and blurring of vision. She was found to have a pituitary mass, which was later proven to be a germ cell tumor. As of writing this case report, her disease is in remission.The disruption of the pituitary gonad axis could affect the disease activity by reducing immunoregulatory control. Germ cell tumor of the pituitary gland is a very rare occurrence. This case report describes a 28-year-old Malaysian Malay woman with lupus nephritis who complained of headache and blurring of vision. She was later found to have a pituitary germ cell tumor.Pituitary germ cell tumor is considered as a type of extragonadal germ cell tumor. They represent 5% of germ cell tumors and typically arise in midline locations. The specific location of the tumor varies with the patient's age . The mosThe age of onset, site of origin, and histologic type of the tumor are important distinguishing characteristics for its prognosis and treatment.A 28-year-old Malaysian Malay woman presented in 1994 with musculoskeletal manifestation of systemic lupus erythematosus (SLE). In 1999, she developed nephrotic syndrome secondary to lupus nephritis . She was immediately started on low-dose cyclophosphamide and mycophenolate mofetil treatment.A year later, however, she complained of amenorrhoea. Levels of follicle-stimulating hormone and luteinizing hormone were both lower than would have been expected. Nine months later, she complained of persistent headaches and blurring of vision. She was screened for chronic meningitis but had a normal brain computed tomography (CT) scan. However, we were not able to do a lumbar puncture as she refused this procedure. Her SLE was already in remission at that time.On examination, both her peri-orbital areas were swollen and red. Her conjunctivae were also injected. She had bilateral temporal hemianopia with left optic atrophy. Magnetic resonance imaging revealed a lobulated mass which extended into the suprasellar cistern and floor of her third ventricle, splaying the optic chiasm.She underwent pituitary resection and her histopathological examination later revealed a germ cell tumor Figure . She wasThis case study is unusual because our patient had both SLE and pituitary germ cell tumor. It is also unusual because when our patient's pituitary function was disrupted, her lupus nephritis went into remission. Initially, it was thought that the two mechanisms responsible for her amenorrhoea were gonadal injury secondary to SLE insult and cyclophosphamide usage.However, our patient's amenorrhoea was due to the pituitary involvement and its effect on her ovaries. At the same time, her SLE was also in remission. This can be explained by the fact that the reduction in immunoregulatory functions of estradiol, testosterone, progesterone, dehydroepiandrosterone (DHEA) and prolactin had resulted in the remission of our patient's SLE .The functional disruption of the pituitary-gonadal complex could affect SLE activity by reducing our patient's hormonal immunoregulation.Written informed consent was obtained from our 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.All the authors contributed to the management of the patient. Additionally, MS was the primary author of the manuscript. All authors read and approved the final manuscript.


Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years. We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver. The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy. Primary undifferentiated embryonal sarcoma (UES) of the liver is a rare and highly malignant neoplasm of mesenchymal origin. The majority of primary hepatic malignancies are carcinomas with primary hepatic sarcomas representing between 0.1% and 2% of primary hepatic cancers [Our institution is a leading tertiary referral centre for the treatment of hepatic malignancies in the region. From 1998 to 2009, 215 adult patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary UES of the liver mistaken for hydatid disease in a 21-year-old male patient. Hydatid disease is endemic in the Middle East and is top of the differential diagnosis for a cystic liver lesion presenting in this age group.A 21 year old male patient was referred to our institution following unsuccessful surgery for presumed hydatid disease of the right lobe of the liver. The initial operation was performed in a district hospital; an open approach was performed and an attempt to drain/resect the lesion failed. The operation was aborted and a percutaneous drain was inserted at the site of surgery. The patient was in hospital for 2 weeks with no improvement. The family decided to transfer him to our institution which is a tertiary care center specialized in hepatobiliary surgery.9/L (range 150-400) and haemoglobin 11 g/dl (range 13-18). ELISA test was negative for hydatid disease Serology was negative for hepatitis B, C and HIV.On admission, the patient was in respiratory distress with severe abdominal pain, distension and lower limb edema. The alpha-fetoprotein (AFP) at the time of diagnosis was 3.6 U/mL (range 1-9), carcinoembryonic Ag (CEA) 2.2 ng/mL (range 0-4) and human chorionic gonadotropin (Beta- HCG) 3.6 mlU/mL (< = 4). The laboratory tests included INR median 1.4 (range 0.9-1.1), serum bilirubin  1.4 mg/dL (range 0-1.2), serum aspartate aminotransferase (AST) 113 IU/L (range 0-50), gamma-glutamyl transferase 95 IU/L (range 0-50), creatinine 0.6 mg/dL (range 0.5-1.2), platelet count 680 × 10A computed tomography (CT) scan of the abdomen revealed a large, well defined lesion with intracystic septations occupying the right hepatic lobe measuring 22 × 19 × 23.6 cm containing the percutaneous drain (Figure A technically challenging extended right hepatectomy was performed. Histopathology revealed an unusual neoplasm composed of irregular spindle cells showing moderate pleomorphism and brisk mitotic activity Figure . ImmunohDiagnosis of primary hepatic sarcoma is challenging due to the lack of specific presenting symptoms, lack of serological markers, non-specific findings on radiological imaging and the rarity of the disease. Pachera et al recently reviewed the literature on primary hepatic UES and found that in the past 50 years only 51 cases have been reported in patients older than 15 years . UES oftHydatid disease is a common pathology in our region and the surgical options are numerous. Surgeons may opt to remove the cysts through an open approach or laparoscopic approach. In either approach, it should be decided whether a radical path or a more conservative one should be taken. With regards to open surgery, several recent studies have shown that the radical approach is associated with lower rates of recurrence, fewer complications such as bile leak and decreased mortality. Percutaneous drainage of hydatid disease is another option, but we try to avoid this due to the risk of intra-abdominal spillage.In our institution, both open and laparascopic procedures are performed and the choice of approach depends on the size and location of the hydatid lesion -11.Complete resection followed by adjuvant chemotherapy is the current standard of care for hepatic UES, however due to the rarity of the disease, limited data exists and treatment remains largely empirical. Positive resection margins and spontaneous or iatrogenic rupture of the tumour are associated with early recurrence and death . Lenze eThere have been three reports of liver transplantation for UES in children -15 howevThe case we have presented demonstrates the propensity of this tumour to rapidly recur along a percutaneous track however tumour control was achieved with a second radical resection followed by adjuvant chemotherapy.In summary, this case illustrates some important aspects of this rare disease:1. The discrepancy between CT and ultrasound appearances of this lesion is a key factor that should raise the index of suspicion when noted.2. Due to the propensity of this lesion to seed along a percutaneous track, percutaneous biopsy or percutaneous drainage should not be attempted.3. Complete resection must be attempted including resection of any potential percutaneous track followed by adjuvant chemotherapy.4. If a percutaneous track cannot be resected, radiotherapy should be considered to reduce the risk of recurrence.The authors declare that they have no competing interests.WF drafted the manuscript, NEM and DM participated in the design of the study, AcS assisted with the collection of data and conceived of the study, MK and AlS 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 case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Oncocytic neoplasms occur in several organs and are most commonly found in the thyroid, kidneys and salivary glands. Oncocytic neoplasms of the adrenal cortex are extremely rare and are usually non-functioning.We report the case of an adrenocortical oncocytic neoplasm with uncertain malignant potential in a 31-year-old man with Cushing's syndrome. The patient had been operated on following diagnosis of a 7 cm adrenal mass. Following surgery, the Cushing's syndrome resolved. The patient is still alive with no metastases one year after the surgery.Adrenocortical oncocytic neoplasms must be considered in the differential diagnosis of both functioning and non-functioning adrenal masses. Adrenocortical neoplasms are the most frequent abnormalities of the adrenal cortex, but only a small fraction of patients also present with endocrine disorders . Most ofOncocytic neoplasms are composed of oncocytic tumor cells, which are characterized by having large, eosinophilic, granular cytoplasm owing to the aberrant accumulation of mitochondria . The mosA 31-year-old man with progressive weight gain and fatigue for 10 years was admitted to hospital. He had developed edema of his hands and face and back pain during the previous two years. Physical examination revealed a typical cushingoid appearance including a plethoric moon face, truncal and centripetal obesity and abdominal cutaneous striae , follicle-stimulating hormone (FSH), luteinizing hormone (LH), and urinary catecholamine levels were all within normal limits. There were no increases in the plasma concentrations of parathormone, calcitonin, testosterone, dehydroepiandrosterone sulphate, androstenedione or 24-hour urinary calcium excretion  level was markedly suppressed (less than 10 pg/ml). Plasma free TChest X-ray was normal. Dual-energy X-ray absorptiometry (DEXA) scanning demonstrated osteopenia at the femur head . T scores of the trochanter, Ward's triangle, L3, and L4 were -2.54, -4.07, -4.42 and -3.36, respectively, compatible with osteoporosis. Computed tomography of the abdomen revealed a 7 cm × 5 cm mass in the right adrenal gland. The patient underwent surgery for a right adrenalectomy. The pathologic diagnosis was adrenal oncocytic neoplasm with uncertain malignant potential according to the criteria proposed by Bisceglia et al. [The tumor measured 7 cm at its greatest dimension, weighed 105 g and was surrounded by an intact capsule. The tumor was composed of polygonal oncocytes with granular, eosinophilic cytoplasm Figure . The tumFrom these findings, the final diagnosis for this patient with Cushing's syndrome was of a functioning adrenocortical oncocytic neoplasm.The Cushing's syndrome improved gradually after the surgery. The patient is still alive with no metastases and is receiving glucocorticoid replacement therapy 1 year after the surgery. His blood pressure and electrolyte levels remain normal.Oncocytic tumors originating from the adrenal cortex are extremely rare. To date, only 46 cases have been reported, and include 24 oncocytomas, 6 oncocytic neoplasms of uncertain malignant potential and 16 oncocytic carcinomas. The patients had a wide age range (27 to 74 years) with a significant female to male predominance. The neoplasms varied in size from 2.2 cm to 15 cm and in weight from 22 g to 865 g. All arose in the adrenal gland with the exception of two cases, which occurred in heterotopic retroperitoneal adrenal tissue [Oncocytic neoplasms of the adrenal cortex were non-functioning with the exception of two cases. Xiao et al.  identifiOur patient had a functioning adrenocortical oncocytic neoplasm. He was symptomatic and admitted to the hospital because of suspected Cushing's syndrome. The laboratory findings were compatible with the syndrome, and further investigation revealed a mass in the right adrenal gland. After the surgery, the Cushing's syndrome resolved.The biologic behavior of adrenocortical neoplasms is the most important practical problem. A combination of clinical, biochemical and histological features can differentiate benign and malignant adrenocortical tumors. Aside from the obviously malignant cases in which the diagnosis is based on common clinicopathologic features universally valid for tumors of any site, such as distant metastases, surgical unresectability and/or invasion of adjacent organs, the prediction of biologic behavior and clinical outcome can be difficult. There is no single histological parameter that is predictive of the clinical outcome and biologic behavior of adrenocortical oncocytic neoplasms. The Weiss system is the most widely used and accepted histological scheme to distinguish benign from malignant adrenal tumors . AccordiBisceglia et al.  proposedAdrenocortical oncocytomas are generally considered as benign neoplasms. In 22 of the 25 reported patients for whom follow-up information was available, no recurrence or metastases were observed within a follow-up period ranging from 1 to 99 months.Borderline adrenal oncocytomas also seem to have a benign clinical behavior. Bisceglia et al.  reportedRecurrence and metastases have been described in patients with an adrenal oncocytic carcinoma. Kurek et al. describeThere was no evidence of metastases in our patient. The tumor was compatible with the diagnosis of an oncocytic neoplasm with uncertain malignant potential. The mass was surgically removed and no other therapy was given. The patient will be followed-up to check for any recurrence or metastases every 6 months.Although rare, adrenocortical oncocytic neoplasms must be considered among the differential diagnosis of both functional and non-functional adrenal masses. Clinical, biochemical and histological features must be evaluated together to assess the biologic behavior of these tumours.ACTH: Adrenocorticotropic hormone; DEXA: Dual-energy X-ray absorptiometry; FSH: Follicle-stimulating hormone; LH: Luteinizing hormone; TSH: Thyroid-stimulating hormone.The authors declare that they have no competing interests.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.OYK conceived of the study, made substantial contributions to the acquisition of data, drafted the initial manuscript and revised the draft over the course of submission. DS coordinated the design and drafting of the initial version and revised the draft over the course of submission. GO carried out endocrinological evaluation of the patient, made contributions to the acquisition of data and revised the draft wherever necessary. BU made contributions to the acquisition of data, participated in study design and coordination, and helped to draft the manuscript. UK made contributions to the acquisition of data, participated in study design and coordination, and helped to draft the manuscript. SS carried out suppression tests and helped to draft the manuscript. AA carried out pathologic assessment and revised the draft where necessary. All authors read and approved the final manuscript.


Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature.A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes.Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause. Tumors of the minor papilla of the duodenum are very rare; the majority of tumors of the minor papilla of the duodenum are neuroendocrine tumors (NETs), such as somatostatinomas and carcinoid tumors -7. CarciPancreas divisum is the most common congenital anomaly of the pancreas, resulting from failure of fusion between the dorsal and ventral pancreatic ducts. Most exocrine secretions of the pancreas drain through the dorsal pancreatic duct and the minor papilla in the pancreas divisum. Patients with pancreas divisum usually have no clinical symptoms, but sometimes have recurrent abdominal pain or pancreatitis when disturbances in drainage of pancreatic secretion through the minor papilla occur.We report a very unusual case of a carcinoid tumor of the minor papilla arising in a patient with complete pancreas divisum who presented with recurrent abdominal pain.A 56-year-old female was referred to our department for evaluation of dilatation of the main pancreatic duct noted on abdominal ultrasonography and CT scan. She complained of intermittent epigastric pain 6 months prior to the referral. She did not drink alcohol or take any medications, and had no significant medical history. She denied sweating, diarrhea, or facial flushing.Laboratory data on admission were as follows: total bilirubin, 0.47 mg/dL; AST, 41 U/L; ALT, 33 U/L; ALP, 161 U/L; GGT, 32 U/L; and amylase, 147 U/L. The complete blood count, urine analysis, and serum electrolytes were within normal limits. The CA 19-9 was 7.99 U/ml and the 5-HIAA was 21.44 mg/day.On the CT scan, the pancreatic duct was diffusely dilated without a definite mass and the common bile duct was slightly dilated Figure . MagnetiUnder the diagnosis of a carcinoid tumor of the minor papilla in complete pancreas divisum, the patient underwent a pylorus-preserving pancreaticoduodenectomy. The resected specimen consisted of duodenum, pancreas, and CBD. There was a 1.2 × 1.0 cm submucosal lesion of the minor papilla Figure .On the microscopic examination, uniform and round-shaped cells were visualized in or around the heterotopic pancreas in the minor papilla, arranged in a solid nest or tubular pattern, suggesting a carcinoid tumor. There was vascular invasion, but no perineural invasion was noted Figures . Among tThe term carcinoid has been replaced with NET based on the development of diagnostic tools and immunochemistry. Recently, NETs have been reclassified within the spectrum of gastroenteropancreatic-neuroendocrine tumours (GEP-NETs) . HoweverAlthough more than 70 cases involving carcinoid tumors of the major papilla have been reported , only 8 Carcinoid tumors of the minor papilla of the duodenum are very difficult to diagnose because they are usually small in size and located at the submucosal area, and they are frequently asymptomatic  and rare13 because the tumors are usually submucosal in location [The endoscopic features of carcinoid tumors are small, round, sessile, or polypoid lesions with a smooth surface. Carcinoid tumors usually have normal overlying mucosa and seldom ulcerate . An EUS Though endoscopic resection may be an alternative to surgical resection ,17, radiWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors read and approved this manuscript. YGK analyzed and interpreted the patient's data. KOK collected the patient's data. TNK supervised this case report and followed up the patient.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/10/17/prepub


Cutaneous eccrine porocarcinomas are uncommon malignant tumors of the sweat gland.A 76-year-old Caucasian man presented to our hospital with a left temporal mass. We describe a case of eccrine porocarcinoma with metastasis to the parotid gland with special emphasis on the role of surgical resection and adjuvant radiation therapy.Besides surgical resection, little is known about the role of adjuvant therapy in managing eccrine porocarcinomas. Radiation therapy should be considered within a multidisciplinary approach in patients with primary or recurrent eccrine porocarcinomas. Eccrine porocarcinoma (EPC) is a rare malignant tumor of the sweat gland that accounts for 0.005% of all skin cancers . Due to We report the case of a patient with cutaneous EPC with secondary metastasis to the parotid gland who was treated with intensity-modulated radiation therapy after surgical resection.A 76-year-old Caucasian man presented with a long history of a left temporal lesion that had progressively enlarged only recently. Initial surgical excision yielded a mass measuring 2.5 × 2.2 × 1 cm. Histological examination revealed tumor islands infiltrating the dermis and connecting to the epidermis with a lobulated morphology Figure . A surroGiven the aggressive behavior of EPC described in the literature and evidence for the effective use of radiation therapy , our patThe six-beam heterogeneous plan entailed delivering a total of 60 Gy in 30 treatment fractions over six weeks. A CT scan with isodose distributions and a dose-volume histogram for the final treatment plan are shown in Figure Weekly on-site treatment visits were documented during the treatment phase of our patient, followed by scheduled three-month follow-up visits. Our patient tolerated the radiation treatment well, and developed only mild xerostomia and mild paresthesias. Further follow-up revealed that our patient remains disease-free 10 months after completion of the treatment course.Eccrine porocarcinoma is challenging to diagnose based on clinical presentation alone, and histopathological examination is almost always required. Typically, a patient presents with an erythematous papule with a recent change in size, bleeding or itching. The differential diagnosis includes squamous cell carcinoma (SCC), basal cell carcinoma, Paget's disease and metastatic cancer. Positive staining for periodic acid-Schiff (PAS), carcinoembryonic antigen (CEA) or angiotensin type 1 receptor can aid in making the diagnosis ,11. AlthWide local excision  and MohsBased on the literature, the role of chemotherapy in the treatment of EPC remains unclear. Orphan cases with good responses to 5-fluorouracil , thiotepOn the other hand, the role of radiation therapy in EPC seems to have changed over the years from radioresistance in earlier reports  to good et al. demonstrated that patients treated with post-operative radiation therapy had 89% local disease control, compared to 63% for those treated with surgery alone and 46% for those treated with radiation alone [Until further studies on EPC are conducted, one can extrapolate important lessons from clinical experience with the more common SCC of the head and neck. The parotid gland is a common site for metastasis of cutaneous tumors of the scalp, frontotemporal and periauricular regions. Poor outcomes have been associated with parotid disease, with a two-year survival of only 74%. Survival of patients drops further to 67% when parotid disease is concurrent with neck disease . Patienton alone .More than 45 years after its original description by Pinkus and Mehregan , guideliAJCC: American Joint Commission on Cancer; CEA: carcinoembryonic antigen; CT: computed tomography; EPC: eccrine porocarcinoma; HIV: human immunodeficiency virus; IMRT: intensity-modulated radiation therapy; PAS: periodic acid-Schiff; PET: positron emission tomography; SCC: squamous cell carcinoma.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.YHZ collected the data regarding our patient, prepared the manuscript, and also researched the related literature. AJZ assisted in interpreting the data from our patient. AKS and EJL supervised YHZ and AJZ and were major contributors in writing the manuscript. MB provided the pathology figures and their legends. All the authors read and approved the final manuscript.


Angiomyolipoma is apparently a part of a family of neoplasms that derive from perivascular epitheloid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon and the most common extrarenal site is the liver. Only two cases of adrenal angiomyolipoma are reported in English literature. Authors wish to add one more case to world literature. Because of its large size and symptomatic presentation of extremely rare tumor merits documentation. Angiomyolipomas are rare mesenchymal tumors derive from perivascular epitheloid cells. These are commonly found in Kidney but extrarenal sites are also mentioned. Angiomyolipoma arising in adrenal is very rare entity, usually asymptomatic, diagnosed incidentally on radiological investigation of abdomen for other conditions. Angiomyolipoma are known diagnostic challenges to pathologists and a deligent search for adipocytes and abnormal blood vessels may help in confirming diagnosis.A 45-year-old female presented with epigastric discomfort off and on. Upper GI endosocopy was normal. Sonography for hepatobiliary system was normal but revealed a well-defined 15×12 cm mass in the retroperitoneum . The CECT abdomen further defined the mass as of left adrenal origin and a possibility of adrenocortical tumor . LaboratAn increasing number of incidental adrenal lesions have been reported recently, which may be attributable to the increasing use of better imaging techniques. Fatty tumors of the adrenal gland are uncommon and their features have received little attention in the literature. These include myelolipoma, lipomas, teratoma, liposarcoma and angiomyolipoma. AngiomyoAngiomyolipomas are rare lesions, often arising in the kidney and are a part of a group of tumors with a diverse appearance known as PEComas (tumors of perivascular epitheloid cell origin). Angiomyolipoma most commonly occurs in the kidney. The next common site is the liver. Extrarenal angiomyolipomas are extremely rare and have been reported in the liver, colon, suprasellar region, small intestine, skin, intranodal, omentum, breast and adrenal gland.–5 AdrenaLarge angiomyolipomas even if asymptomatic should be removed to avoid complications like spontaneous rupture owing to the presence of abnormal elastin and poor vascularity in the tumor. Neverthe


The oral cavity may exhibit a vast number of pathologic conditions, often dealt with by different medical disciplines. Combined with a substantial variation in clinical appearance, an accurate diagnosis may provide difficult to establish in selected cases. Histopathological investigations are therefore mandatory for correct diagnosis and adequate treatment. We describe a common, truly benign condition in the oral cavity, which due to histopathological misinterpretation was planned for major surgery and subsequent chemotherapy. This was avoided by spontaneous regression of the lesion. The case illustrates that uncritical trust in laboratory diagnostic tests may lead to severe mistreatment. The oral cavity may exhibit a myriad of expansive lesions, including cysts, reactive lesions, and benign and malignant neoplasms. Additional laboratory examinations are therefore often used to specify the diagnosis. However, the clinical impact of errors committed in the laboratory is poorly described ,2. RecenA woman aged 31 years experienced in her fifth, and only successful, in vitro fertilization attempt, after approx. 20 weeks of pregnancy a growing tumour palatinally to tooth 22 , and after about a month, a new tumour, similar to the previous and at the very same position, occurred. This also disappeared spontaneously after abortion.e.g. insufficient clinical data [Pyogenic granuloma is considered a reactive lesion, quite common in the oral cavity. It may clinically mimic several other lesions, including malignancies. The so-called pregnancy tumour is a pyogenic granuloma that seems to appear more easily in the gingiva during pregnancy due to hormonal influence. It can be fast-growing and reach a considerable size. After delivery or termination of pregnancy, they most often disappear spontaneously. The histopathology shows proliferating endothelial cells, the stroma is fibrillar, often arranged in lobular aggregates . The actcal data . To detecal data . In thiscal data  were perThe clinical course in this case is highly consistent with a pregnancy tumour. It is, to our best knowledge, not been reported that neither angiosarcomas nor hemangiopericytomas may undergo spontaneous regression. However, the possibility for hormonal and endothelial growth factor influence in vascular tumours during pregnancy is discussed ,11.The patient has to this day not experienced any later recurrences of the lesion. Her two twin boys delivered as preparation for tumour treatment have also grown up perfectly healthy.As medicine in general becomes increasingly dependent of laboratory investigations, sample analyses, and advanced imaging techniques, it is of great importance to emphasise the value of the clinical consultation as well. The case also reminds us that simple conditions may be misinterpreted, possibly causing serious mistreatment.Written informed consent was obtained from the patient for publication of this case report. 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.KT was responsible for diagnosis and treatment of the patient and has contributed to the manuscript preparation. SL has re-examined the case, contributed to patient follow-up consultations and drafted the manuscript.


At the time of primary diagnosis the tumors are most often in advanced stage and the patients complain of abdominal pain due to large tumor size. Endosonographically, the tumors impress as submucous mass with suspicion to malignancy. Sarcomas following hereditary retinoblastoma in childhood are in generally located in the soft tissue. Structural alterations of the A 37-year-old german male suffered from reflux disorder. In endoscopic examination a small polypous tumor was detected in the stomach. The resection specimen revealed an intramucosal leiomyosarcoma. At the age of one year, the patient had a retinoblastoma.This is the unique report of an intramucosal gastric leiomyosarcoma and the first account of a gastric leiomyosarcoma after retinoblastoma in childhood. A careful clinical follow-up is advised because of increased risk of developing further metachronous malignancies. RB1 seem to be involved in the pathogenesis of the secondary malignancy after treatment of retinoblastoma.Leiomyosarcomas are of smooth muscle origin and develop in the soft tissue of the vessel wall and in the smooth muscle layer of visceral organs. In the gastrointestinal tract, they normally arise in the submucosa and bulge out the mucosa and serosa. They present most often in advanced tumor stage. In this case we report of an intramucosal gastric leiomyosarcoma. Medical history referred treatment of a retinoblastoma in childhood. Sarcomas following hereditary retinoblastoma are in generally located in the soft tissue. Structural alterations of RB1 was detected in 1988. In family history there is no further case of retinoblastoma. Due to tumor prevention the patient underwent endoscopic examination. In gastroscopy, a 1 cm in diameter polypous lesion was found in the antrum of the stomach. It was suspicious to be a hyperplastic polyp or an adenoma of gastric mucosa. The polypous lesion was resected endoscopically and was sent to histopathological investigation. Representative 4 μm sections of formalin-fixed, paraffin-embedded tissue from the tumor specimens were stained with hematoxylin and eosin (H&E) and periodic acid-Schiff. Immunohistochemical staining was performed using the standard avidin-biotin method with antibodies against smooth muscle actin , S100 , CD34  and KIT . The proliferative activity was assessed by staining the tissue with the antibody MiB-1 .A 37-year-old man with reflux symptomatic was sent to endoscopic examination. During the examination strabismus was striking. The patient reported about eye operation with 1 year of age, due to a retinoblastoma. Both eyes were affected. The right eye was enucleated. The left eye was treated with laser. There was no tumor relapse. A germline mutation in The polypous lesion, 1 cm in diameter, showed regular foveolar gastric glands and a diffuse spindle cell infiltrate in the mucosa. The spindle cells were arranged in parallel and whorl like bundles. The nuclei were elongated with plump ends and focally mild atypia Fig.  and negaRB1 [RB1 are thoroughly investigated in soft tissue tumors [RB1 copy seems to be involved in tumor development. Radiation combined with chemotherapy was associated with a heightened risk for leiomyosarcoma in this study.Leiomyosarcomas of the stomach are rare. They usually present in older age and are typically of high grade malignancy WHO 2000). They arise from the smooth muscle of gastric wall and were mostly located in the submucosa. The histological diagnosis of a leiomyosarcoma is unequivocal on the basis of the immunohistochemical expression of SMA. At the time of primary diagnosis the tumor size is normally large. Complete tumor resection is the standard treatment. The reported case was exceptionally, clinical and histological. The leiomyosarcoma resembled a polyp of the gastric mucosa without criteria of a stromal tumor or signs of malignancy. The tumor was limited to the mucosa, showed mild nuclear atypia but a high proliferative activity. It is the unique intramucosal leiomyosarcoma of the stomach and the first gastric leiomyosarcoma described in a survivor of a retinoblastoma in childhood. Because of the young age of the patient, the high proliferative activity of the tumor and the visceral tumor site, a resection with large tumor free margins was striving. There is an increased risk for the development of a metachronous malignancy following hereditary retinoblastoma due to the prior treatment and/or genetic susceptibility of 000. Theye tumors . Most ofe tumors . A leiome tumors . Viscerae tumors ,7. The pRB1 may have a protooncogenetic effect on the development of secondary malignancies. As described earlier there is an increased risk to develop a third tumor.Since neither radiation nor chemotherapy treatment is reported in our case, primary genetic alterations, i.e. in This is the unique report of an intramucosal gastric leiomyosarcoma and the first account of a gastric leiomyosarcoma after retinoblastoma in childhood. A careful clinical follow-up is advised because of increased risk of developing further metachronous malignancies.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.UP drafted the manuscript with review of the literature and took the microscopic imaging.HG participated in the care of the patient, contributed the clinical data and revised the manuscript for intellectual content and given final approval of the version to be published.Both of the authors read and approved the final manuscript.


Cesarean section scar pregnancy is a rare but serious complication. It occurs in women with previous uterine scar when implantation takes place at the site of this scar. If early diagnosis is missed the woman's future fertility and eve her life are at risk. Earlier reports on the condition suggesting different management approaches have been described.A 37 year-old woman gravida 4, para 3, was referred to our emergency department, as a case of missed miscarriage following 14 weeks amenorrhea. Ultrasound examination revealed a picture suggestive of intramural pregnancy near the Cesarean section scar. The case was managed by laparotomy evacuation of products of conception and repair of the scar.The diagnosis of ectopic intramural pregnancy in a cesarean section scar is possible with ultrasound and high level of suspicion. This serious complication must be suspected in a pregnant woman with previous uterine scar when early ultrasound show a gestational sac that is implanted anteriorly in the lower uterine segment, near the uterine scar. Ultrasound criteria for diagnosis include empty uterus, empty cervical canal and a discontinuity on the anterior wall of the uterus demonstrated on a sagittal plane of the uterus running through the amniotic sac. Early intervention is recommended to avoid serious consequences in such cases. Pregnancy in a Cesarean Section scar (CSS pregnancy) is a rare complication that may have serious consequences affecting the woman's future fertility and may even affect her own life. It is believed to develop as a result of the presence of a microscopic tract in the scar allowing the blastocyst to be implanted deep in the myometrium .Early diagnosis of CSS pregnancy is essential to avoid serious complication such as sever hemorrhage which may require hysterectomy and endanger the woman's' life. Diagnosis is based on high level of suspicion when a gestational sac is seen at sonography on the anterior lower part of the uterus in a woman with a previous Cesarean section .The differential diagnosis includes spontaneous abortion and cervico-isthmical pregnancy. It is essential to get a proper history and a high resolution ultrasound scanning in order to avoid wrong diagnosis .Treatment modalities are diverse with no one option being superior to others. Most reported cases are sporadic and subject of trials.Surgical treatment by evacuation of the sac, excision and repair of the gaped scar through laparotomy  or laparA 37 year-old United Arab Emaerite woman gravida 4, para 3, was referred to our emergency department, as a case of missed miscarriage following 14 weeks amenorrhea. The patient's obstetric history included three Cesarean sections last one 9 years back. The first Cesarean section was done at 7 month gestation due to placental abruption, the second was done for placenta previa and the third was done electively. The patient presented with history of lower abdominal pain and bleeding per vagina for the last 12 days. Vaginal examination revealed an enlarged uterus of 8 weeks size that is slightly tender on movement. The cervical os was closed with no vaginal bleeding. Trans-abdominal ultrasound in transverse scan  the ultrasound picture was already clear.The challenge in diagnosis is further complicated by a challenge in treatment. Due to the relative rarity of the condition there are no optimal lines for therapy. Treatment modalities are either medical or surgical. The medical treatment consists of administration of MTX locally  or systeThe surgical approach involves the traditional laparotomy and evacuation of the sac and repair of the uterine defect . The samIt is important however to emphasize that due to the lack of enough available data no treatment modality can guarantee uterine integrity or can be promoted as optimal . TreatmeIn our case although her condition was stable yet laparotomy was selected as a line of management as the mother did not want to have a prolonged follow up period. Although the pregnancy was nonviable and expectant management by follow up with BHCG was possible, the size of the gestational sac and the woman's refusal of a long follow up period justified the active approach.Counseling patients with CSS pregnancy is difficult. There is no enough data regarding the optimum treatment or a guideline for management. With the accumulation of reported cases it may be soon possible to have a guideline for optimum management as it is now clear regarding diagnosis.The incidence of Cesarean Section is increasing year after year. The complications of this mode of delivery are not limited to the time of surgery. Remote complications especially in subsequent pregnancies are also of significance. Cesarean Section scar pregnancy is also is one of these possible complications that may lead to rupture of the uterus, bleeding and may endanger the woman's life. Early diagnosis is possible with high level of suspicion and with the aid of high quality ultrasound scanning. The appropriate treatment of such cases is not yet clear. The accumulation of data may help to understand the disease better and to develop the appropriate plan of management.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AA analyzed and interpreted the patient data and contributed in preparation of the manuscript. LO performed the ultrasound. MW assisted in the ultrasound and in the preparation of the manuscript. TA was involved in the imaging preparation and reviewed the manuscript. MA reviewed the literature and participated in reviewing the manuscript. All authors read and approved the final manuscript.


Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis), there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF. Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon fibrosarcoma of intermediate-grade malignancy and has lately been recognized as a distinct clinical entity . SEF is Together with low-grade fibromyxoid sarcoma (FMS) and hyalinizing spindle cell tumor with giant rosettes , SEF belThe only consistent immunohistochemical finding is a strong and diffuse reactivity for vimentin, while almost all other markers are negative , 4. Pecuossifying fibroma or desmoplastic fibroma was reported. Intermittently, the pain relieved and the patient was discharged. Four weeks later, local pain was recurring and an X-ray control showed progression of the osteolytic lesion. Hence, curettage and reconstruction with autologous bone graft of the iliac bone was performed. Again, tissue specimens, now described as non-ossifying fibroma, presented as a benign process in the histological workup. Decreasing pain and present stability of the leg allowed the patient's discharge. Three months later the patient was reassessed by open biopsy due to recurrent and increasing pain as well as swelling of the proximal right tibia. This time, tissue specimens were analyzed by independent local and reference pathologists who finally established the diagnosis of SEF of the bone. As seen in other cases of SEF the resected tumor only stained positive for vimentin in immunohistochemistry. Routine staging was negative for metastases (CT-scan of the lung and 18-FDG-PET/CT scan). Clinically, only one single enlarged lymph node at the right outer thoracic wall was detected, which, however, showed no tumor cell infiltration on resection biopsy.We report on a 16-year-old female who presented with a four-week history of persistent and progressive load-dependent pain focused on the right proximal tibia. On physical examination, no signs of inflammation or other malfunctions could be seen. Conventional X-rays of the right tibia revealed a 4 × 4 cm osteolytic cavity, which was presumptively diagnosed as a benign lesion. Thus, open biopsy was carried out, and pathological differential diagnosis of an Taking into consideration the bone association of the tumor and the lack of a standardized treatment regimen, the local interdisciplinary tumor board decided to treat the patient following the EURAMOS-1 (European-American-Osteosarcoma-1) protocol designed for the treatment of osteosarcoma. The EURAMOS-1 treatment plan consists of chemotherapy elements with Doxorubicin, Cisplatin, and high-dose Methotrexate , and sevCooperative Weichteilsarkom Studie) designed for the treatment of soft tissue sarcoma [After two courses of Cisplatin/Doxorubicin and five courses of high-dose Methotrexate our patient underwent tumor resection and endoprosthetic reconstruction . The res sarcoma . The adj sarcoma , 34 and ossifying fibroma in our patient. Indeed, osseous differentiation of SEF has been described in the literature and was seen in the first biopsy, but was absent in specimens of the second biopsy [In general, SEF appears to be a slowly growing tumor often present for several months or years before diagnosis. In most cases of SEF, it took 33 months from the first onset of symptoms to correct diagnosis . The deld biopsy –4. Althod biopsy , 28. SEFDue to the rareness of this tumor, there are no established treatment regimens. So far, patients have been treated with amputation, wide excision, radio- and chemotherapy, or various combinations thereof . Our expWritten 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.


Pleomorphic adenoma, also called benign mixed tumor, is the most common tumor of the salivary glands. About 90% of these tumors occur in the parotid gland and 10% in the minor salivary glands. The most common sites of pleomorphic adenoma of the minor salivary glands are the palates followed by lips and cheeks. Other rare sites include the throat, floor of the mouth, tongue, tonsil, pharynx, retromolar area and nasal cavity. In children, intraoral pleomorphic adenomas of the cheek are extremely rare with only three cases reported to date. Here we report a case of pleomorphic adenoma of minor salivary glands of the cheek in a 17-year-old girl. The mass was removed by wide local excision with adequate margins, and after a follow-up period of three years there were no recurrences. To conclude, pleomorphic adenoma should be considered in the differential diagnosis of cheek masses in youngsters. Wide local excision is to be recommended as the treatment of choice. A close follow-up is necessary postoperatively. Pleomorphic adenomas are benign salivary gland tumors that represent about 3- 10% of the neoplasm of the head and neck region . The palPleomorphic adenoma consists of cells with epithelial and mesenchymal differentiation (mixed tumor). The highly variable morphology of this neoplasm is the result of interplay between these elements. Now it is widely accepted that both epithelial and mesenchymal  elements often arise from same cell clone, which may be a myoepithelial or ductal reserve cell. There is no difference in behavior of this neoplasm based on proportion of various elements . Lee et The mucosa of the cheek is a uncommon site of occurrence for intraoral pleomorphic adenoma  and mostA 17-year-old girl presented with a slowly growing painless swelling in the right cheek of four years duration. Clinical examination revealed a 2.0-cm, firm, mobile mass in the right cheek . There was no history of trauma, fevers, disturbance of salivation, or oral surgeries. The laboratory tests were unremarkable. Radiological examination showed no abnormality in the panoramic radiograph Figure . The masPleomorphic adenoma occurs more frequently in women than in men and is most common from the fourth to sixth decades with a mean age of 43-46 years . SalivarThe clinicopathologic features of the case reported here concurs with previous studies. Yamamoto et el reported a 9-year-old Japanese girl with pleomorphic adenoma of the cheek mucosa. Cohen and Kronenberg reported two more cases of juvenile pleomorphic adenomas of the cheek  -15. No rThe differential diagnosis of the juvenile pleomorphic adenoma of the cheek Table  includesTo conclude, juvenile pleomorphic adenoma of the cheek is a rare neoplasm and therefore its diagnosis requires a high index of suspicion. Complete wide surgical excision is the treatment of choice. Recurrence after many years of surgical excision as well as malignant transformation should be a concern and therefore long- term follow- up is necessary.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TD outlined the general concept, was involved in the literature search, preparing the materials, supplying the relevant clinical details, providing the radiological details and clinical figures, drafting and revising the manuscript. MRH was involved in histopathology evaluation, interpreted the histopathology, providing the histopathology details, drafting and revising the manuscript. Both authors have read and approved the present manuscript.


Inferior vena cava (IVC) leiomyosarcoma is a rare tumor of smooth muscle origin. It is often large by the time of diagnosis and may involve adjacent organs. A margin-free resection may be curative, but the resection must involve the tumor en bloc with the affected segment of vena cava and locally involved organs. IVC resection often requires vascular reconstruction, which can be done with prosthetic graft.We describe a 39-year-old man with an IVC leiomyosarcoma that involved the adrenal gland, distal pancreas, and blood supply to the spleen and left kidney. Tumor excision involved en bloc resection of all involved organs with reimplantation of the right renal vein and reconstruction of the IVC with a polytetrafluoroethylene graft. The patient recovered without renal insufficiency, graft infection, or other complications. Follow-up abdominal imaging at 1 year showed a patent IVC graft and no locally recurrent tumor. Prosthetic graft provides a sufficient diameter and length for replacement conduit in extensive resection of IVC leiomyosarcoma.To our knowledge, this is the first case of resection of an IVC sarcoma with prosthetic graft reconstruction in combination with pancreatic resection. Aggressive surgical resection including vascular reconstruction is warranted for select IVC tumors to achieve a potentially curative outcome. Inferior vena cava (IVC) leiomyosarcomas are rare malignancies; fewer than 300 have been reported in literature. This mesenchymal tumor is derived from medial smooth muscle cells and most often originates from the IVC segment between the hepatic veins and the renal veins . It is mLocally involved organs are commonly the kidney, adrenal gland, and liver ,3,5-8. RA previously healthy 39-year-old man presented to the emergency department with a 2-month history of intermittent dull abdominal ache with weight gain as well as intermittent right upper extremity numbness. Physical examination revealed a mildly obese abdomen with a subtle mass in the left upper quadrant. No lower extremity edema was noted. Abdominal magnetic resonance imaging revealed a 15 × 6 × 5-cm, well-circumscribed, preaortic retroperitoneal mass, which involved the IVC, causing mass effect on the surrounding organs Figure . The masSurgical resection of the mass was performed through a bilateral subcostal incision. Careful abdominal exploration confirmed the preoperative findings, and no other metastases were present. The tumor appeared to arise from the IVC at the level of the left renal vein and extend intraluminally in the IVC up to the caudate lobe, involving the left kidney, left adrenal gland, distal pancreas, splenic artery, and left renal artery. The tumor was mobilized en bloc with the left kidney, left adrenal gland, distal pancreas, and spleen. Proximal and distal control of the IVC was obtained, and the tumor was resected. The pancreas was transected with a linear stapling device, the main pancreatic duct was identified and oversewn, and a closed suction drain was placed at the transection site. Adequate margins were ensured by frozen section. IVC reconstruction was performed from the level of the caudate lobe to the distal IVC in an end-to-end fashion with a 14-mm external ring-reinforced PTFE interposition graft Figure . The graPostoperatively, the patient underwent adjuvant chemotherapy with 4 cycles of docetaxel and gemcitabine, resection of a metastatic left deltoid tumor mass, and 40.0 Gy of radiation therapy to his left upper extremity. Follow-up abdominal imaging at 1 year revealed no recurrent abdominal disease and a patent IVC graft (Figure Primary leiomyosarcoma of the IVC is a rare malignant tumor first described in 1891 by Perl at autopsy. The most common presenting symptoms are abdominal pain, palpable abdominal mass, and lower limb edema . The segIVC leiomyosarcomas are relatively resistant to chemotherapy and radiotherapy, and complete resection of the tumor is the only known method for a chance of cure. The prognosis for leiomyosarcoma of the IVC treated medically is poor, with an average survival of less than 3 months . HoweverEarly diagnosis is rare, and the tumors often invade surrounding organs. The amount of vascular involvement by the retroperitoneal tumor accounts for the high surgical risk and technical difficulties seen during attempts at complete resection. Accurate preoperative imaging to determine the extent of the tumor is essential for adequate planning, and magnetic resonance imaging is the preferred modality.Caval management after IVC resection is controversial. Options include primary repair, autologous patching, ligation, or reconstruction with prosthetic graft. Extensive venous involvement and large tumor size often preclude short segment resection with simple repair or patching. Ligation of the IVC is favored by some and has been shown to be well tolerated and generally safe, especially in those with preoperative IVC thrombosis ,3. HowevBecause of the considerable size of these tumors at diagnosis, wide retroperitoneal dissection is often necessary for complete tumor resection, disrupting the preexisting venous channels. This dissection negates any collateral flow that achieved venous decompression preoperatively. Long segments of tumor involvement of the IVC necessitate ligation of a larger amount of lumbar veins that serve as collaterals. Kieffer et al  used a pAlthough increasing the complexity of the operation, partial or total resection of locally involved organs is necessary for complete tumor removal because prognosis is highly dependent on a tumor-free margin. Patients with inadequate resections are at high risk for local recurrence, causing death from a retroperitoneal sarcoma . MultiviAlthough often not curative, aggressive surgical resection combined with chemoradiotherapy has been definitively shown to prolong survival in patients with IVC leiomyosarcomas. Vascular reconstruction is often required, and prosthetic replacement of the IVC with PTFE has been shown to be a safe option for retroperitoneal sarcomas. Graft-related complications are low but may be increased by tumor involvement of the pancreas. However, pancreatic involvement did not preclude resection in this case, giving the patient the survival benefit of a margin-free radical en bloc resection.IVC: inferior vena cava; PTFE: polytetrafluoroethyleneWritten 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.JAS participated in care of the patient and data collection, participated in study design, participated in literature review and manuscript drafting, participated in manuscript writing and revision, and read and approved the final manuscript. GPF participated in care of the patient and data collection, participated in study design, and read and approved the final manuscript. MKD participated in care of the patient and data collection, participated in study design, and read and approved the final manuscript. REN participated in data collection and study design and read and approved the final manuscript. WJM participated in care of the patient and data collection, participated in study design, and read and approved the final manuscript. JHN participated in care of the patient and data collection, participated in study design, participated in manuscript writing and revision, and read and approved the final manuscript. All authors read and approved the final manuscript.


Diffuse involvement of the gastrointestinal tract by graft versus host disease (GVHD) is a common complication of allogeneic hematopoietic stem cell transplant (HSCT). Gastrointestinal GVHD usually presents 3 or more weeks after HSCT and is characterized by profuse diarrhea, anorexia, nausea, vomiting, abdominal pain and gastrointestinal bleeding. We report a case of a 23-year-old male who had undergone allogeneic HSCT and presented with bloody diarrhea on the 90th day post-HSCT. On the fourth day of admission, the patient passed per rectum a 27-cm long pinkish colored fleshy material recognized as a “colon cast”. Sigmoidoscopy showed a congested and erythematous rectum with the remaining portion of the “colon cast” attached to the proximal part of the sigmoid colon. A biopsy from the rectal wall was suggestive of grade IV GVHD. The patient was treated with methylprednisolone, cyclosporin and mycophenolate mofetil, with a partial response , but then he developed multiple other medical complications and died after 3 months. Acute graft-versus-host disease (GVHD) occurs after allogeneic hematopoietic stem cell IA transplantation (HSCT) as a consequence of an immune reaction by the donor immune cells against host tissues. About 35% to 50% of HSCT recipients develop acute GVHD.14The spontaneous passage of a full-thickness colon cast has been described eight times previously,coli and Streptococcus viridans septicemia, febrile neutropenia, cytomegalovirus (CMV) antigenemia, GVHD involving the skin, and aspergillosis of the lung. On admission the patient was febrile and appeared pale. The skin was hyperpigmented from GVHD. The oral cavity showed features of mucositis. The lower abdomen was tender on palpation. The cardiovascular, respiratory and nervous system examination was unremarkable. Broad-spectrum antibiotics and other supportive measures were begun and the patient was moved to the intensive care unit. On the fourth day of admission the patient passed a 27-cm long, tubular, pinkish-colored fleshy material rectally , hemoglobin 104 g/L , platelet count 10×109/L , urea 5.3 mmol/L , creatinine 88 micromols/L , potassium 3.1 mmol/L , sodium 138 mmol/L , carbon dioxide 19 mmol/L , albumin 26 g/L , bilirubin 21 μmol/L , lactic acid 0.8 mmol/L , ALT 30 U/L , and alkaline phosphatase 223 U/L . The histology of the sigmoid colon showed granulation tissue and base ulcers suggestive of grade IV acute GVHD -alpha and interleukin 1, which lead to activation of host antigen-presenting cells. The donor T-cells recognize alloantigens on host antigen-presenting cells and activate the donor T-cells. The activated T-cells then proliferate and differentiate into effector cells and secrete cytokines such as interferon (IFN)-gamma. TNF-alpha and IFN-gamma cause further injury to the gastrointestinal epithelium leading to the tissue damage characteristic of acute GVHD.12The gastrointestinal system is the third most common organ involved in acute GVHD. Gut GVHD may involve any part of the gastrointestinal tract and is frequently the most severely involved organ. It is often difficult to treat gut GVHD. Diarrhea and abdominal cramping are generally the hallmarks of gut involvement. Clinical manifestations of gut GVHD include diarrhea, nausea, vomiting and crampy abdominal pain. There may be a large volume of diarrhea, which is often bloody. Abdominal distention and paralytic ileus may develop.Upper and lower gastrointestinal endoscopy is a useful modality to diagnose acute GVHD and to differentiate it from other causes of diarrhea. Endoscopic findings of gut GVHD may vary from normal to extensive edema, mucosal sloughing or diffuse bleeding from the gut mucosa. The endoscopy may show prominent lesions in the cecum, ileum, and ascending colon. The mucosa involvement can be seen in the stomach, duodenum, and rectum as well.13Endoscopy is a useful tool to grade the severity of acute GVHD, helping the physician to choose appropriate treatment and to predict prognosis. Endoscopic grading is based on the appearance of colonic mucosa during colonoscopy or sigmoidoscopy. Endoscopically, acute GVHD can be classified as stage 1 to 4. Stage 1 is normal appearing mucosa, stage 2 will show a loss of vascular marking of normal colonic vascular pattern or there will be focal mild erythema. Stage 3 acute GVHD will have colonic mucosal edema, erythema, erosions, and/or bleeding. Stage 4 is characterized by colonic ulceration and bleeding.To our knowledge, passage of the colonic cast as a manifestation of acute GVHD has never been reported. Previously, one case was reported in the literature, with passage of esophageal mucosal cast as a complication of chronic GVHD following bone marrow transplantation.


Lipomas can be found anywhere in the body with the majority located in the head and neck region as well as in the shoulder and back. They are not very common in the hand and those involving the fingers are very rare. Although, it is not the only case reported, lipoma of the index finger is very uncommon.A 52-year-old Caucasian man presented with a lipoma of the right index finger. He complained of no pain but he had difficulty in manual movements. Treatment was surgical excision of the lipoma. There has been no recurrence for two years.Although lipomas of the fingers are rare entities, their awareness is imperative since the differential diagnosis from other soft tissue tumors and from the special lipomatous subtype involved is quite extensive. Lipomas are benign, mesenchymal neoplasms occurring in areas of abundant adipose tissue . They canddigit with no disturbance of sensibility. He had no ulceration or pigmentation on the overlying skin, neither any other inflammatory changes such as redness, heat or pain on palpation. Plain anteroposterior radiograph of the digit showed no invasion of the bone, thus excluding parosteal lipoma from the differential diagnosis. Unenhanced MRI shows the exact position and morphology of the lesion in sagittal view, with the characteristic capsule of the mass suggesting a working diagnosis of lipoma (Figure A 52-year-old Caucasian man was presented with a massive soft tumour of the volar aspect of the proximal and middle phalanx of the right index digit. The mass appeared five years prior to the medical consultation, and was asymptomatic until 12 months ago, when the patient first complained of limitation in the range of motion, especially at flexion and pain during manual maneuvers. On clinical examination he had a soft mobile, elastic mass at the volar aspect of the 2Under general anesthesia and the use of tourniquet, we performed a 'Z' shape incision in the volar aspect of the finger. The fatty, lobulated mass was found to be localized in the subcutaneous tissue and was attached close to the proximal and middle phalanx of the digit without interfering with the fascicles of the tendon Figure . The neoLipomas are tumors of non-neural origin such as lipofibromatous hamartoma and haemangioma and account for approximately 16% of soft tissue mesenchymal tumors . AccordiThey are seldom encountered in the hand and are extremely rare in the digits. Lipomas appear mostly in the fifth and sixth decade and probably are the most common solid cellular hand tumors. These lightly encapsulated tumors are composed of mature fatty tissue where the central lipid droplet and peripherally located nucleus forms the characteristic signet ring cell . They arClinically, superficial lipomas commonly appear as asymptomatic, slow growing, soft fluctuant, and bulging, lobulated and mobile mass. Unless they lie in canals commonly associated with nerve compression, they cause pain and distal sensory changes and motor weakness . BecauseRadiological evaluation is diagnostic in up to 71% of cases . ComputeLipomas arising from the deep palmar space tend to present in the periphery of the hand because of the unyielding nature of the overlying palmar aponeurosis. Diagnosis in the hand and digits can be difficult, because of their rarity and deeper location. The differential diagnosis as mentioned, includes other soft tissue tumors such as ganglion cysts, giant-cell tumors, myxomas, angiolipomas and intraneural lipofibromas . HoweverCareful dissection is necessary during the surgical procedure in order to avoid recurrence. Recurrence after marginal excision is less than 5%. In our case the tumor located subcutaneously, with a part extended into the tendon sheath. Clinical diagnosis was confirmed with histological examination. Histological examination showed a yellowish, lobulated, ulcerative lipoma confirming the initial diagnosis but unfortunately the picture is not available, although it was thoroughly searched in all involved departments. No complications occurred during the postoperative period while the patient achieved full range of motion. There were no sings of recurrence after a follow-up of 24 months. Surgical dissection and removal of the neoplasm was performed with no post-operative complications and is the treatment of choice.Although lipomas of the fingers are rare entities, their awareness is imperative since the differential diagnosis from other soft tissue tumors and from the special lipomatous subtype involved is quite extensive.All reasonable attempts to gain consent have been made. The patient is anonymous and there is no reason to think that the patient or their family would object to publication.The authors declare that they have no competing interests.CE, KA and PA are the orthopedic surgeons that contributed to the excision of the lipoma in the OR. NP and KC were the authors that contributed mostly to the medical writing and formatting of the article and finally NI, LF, TI and CA were the members of the radiology department that made the diagnosis and the thorough literature research.


Carcinoids are the most common tumours of the appendix. These tumours show prevalence in white children. The clinical presentation of the appendiceal carcinoids is similar to that of acute appendicitis, although in many cases the tumour is diagnosed incidentally during an operation. The diagnosis should be confirmed histologically. The prognosis in patients with local disease is excellent. In small lesions isolated appendicectomy is considered as the most appropriate treatment, while in larger lesions right colectomy should be performed. We report a case of a carcinoid tumour in the tip of the appendix of a thirteen year old girl which was diagnosed intraoperatively. The patient received isolated appendicectomy due to the small size of the lesion. Ten years after the operation there is no evidence of recurrence or metastases, and the patient is considered free of disease. Carcinoid tumours (CTs) are the most common neoplasm of the appendix ,2. The oIn our case a carcinoid tumour in the tip of the appendix presented as acute appendicitis. It was the only appendiceal tumour throughout a total of 1540 appendicectomies, performed during the last eighteen years.A 13-years-old female patient presented complaining for abdominal pain in right lower quadrant (RLQ), nausea and decreased appetite the last two days. Physical examination revealed a healthy looking female with a mildly elevated temperature 38°C, blood pressure 110/80 mmHg and pulse rate 95/min. The patient showed no sings of acute abdomen suggestive of acute appendicitis, as direct and rebound tenderness in the RLQ. Rectal examination revealed a mild tenderness in the RLQ, but no blood or palpable masses were observed. Hematocrite was 40%, white blood cells 13700/mm with neutrophil prevalence 87%, platelets 175000/mm, erythrocytes sedimentation rate 55 mm/h, while the rest blood analysis was normal. Abdominal ultrasound revealed appendiceal inflammation with transverse appendix diameter to be 9 cm. The patient was operated for acute appendicitis. The appendix found inflamed with distension of the tip, which was palpated as a solid, moderately hard, elastic and yellowish mass with diameter 1 cm.Figure th day. Ten years later the patient is free of local or metastatic disease.5-hydroxyindoleacetic acid (5-HIAA) which was measured postoperatively was normal at 3,2 mg/24 h. The patient's postoperative course was uncomplicated and she was discharged the 4Carcinoid tumours of the appendix are relatively uncommon neoplasms. Although are considered rare pathology in children, these are the most frequent tumours of the gastrointestinal tract in childhood and adolescence . They arThe reported incidence of appendiceal carcinoids in several studies ranges from 0.08 to 0.7% in surgical specimens -8. WilloThe clinical presentation of the appendiceal carcinoids is similar to that of acute appendicitis, but in some cases the disease is incidentally found during surgery performed for another diagnosis or problem. Recurrent episodes of abdominal pain were reported in many cases may indicate partial obstruction of the appendiceal lumen by a tumour . In two At present, the site and the size rather than the depth, are used for the assessment of the tumour . In the Metastasis of an appendiceal carcinoid is very rare in children probably because most reported tumours in this age group are small and less aggressive. In this case the tumour extended thought the entire diameter of the appendix and involved all layers of the wall to the overlying serosa surface without distant metastasis.The treatment of the carcinoid tumours in the appendix depends to the size and the site of the tumour. Tumours smaller than 2 cm can be adequately treated by appendectomy, while right hemicolectomy is recommended for pediatric patients with appendiceal carcinoid tumours larger than 2 cm, especially when the mesoapendix is involved or in cases with residual tumours at the margin of resection . Our patIn conclusion, CTs are the most common tumours of the appendix. In children, they occur more commonly in white females with a mean age of 13 years. The clinical presentation of the CT is similar to acute appendicitis, but the CT can be an incidental finding during surgical procedures other than appendicectomy. CT was diagnosed on histological examination of the removed appendix. The site and the size of the tumours rather than the depth, are used for the assessment of the CT. Localized disease has an excellent prognosis. Patients with metastatic CT fare poorly. Single appendicectomy is considered the appropriate treatment, while right colectomy is indicated in tumour larger than 2 cm. We report a case of an appendiceal CT in a 13 year old girl with diameter 1 cm which was treated with isolated appendectomy. The patient is disease free of ten years follow-up. Clinical awareness and early diagnosis of CT of the appendix may significantly decrease morbidity and mortality.CTs: Carcinoid tumours; CT: Carcinoid tumour; cm: centimeters; RLQ: right lower quadrant; mm: millimeter; mm/h: millimeters per hour; °C: Celcious degrees; min: minutes; 5-HIAA: 5-hydroxyindoleacetic acid; mg: milligrams.The authors declare that they have no competing interests.EC participated in the patients treatment, had the concept of the case report and contributed in the first draft, NP participated in the first draft and in the revisions, MC participated in the diagnosis of the case and in the presentation of its pathology, GF contributed in writing the paper, SR contributed in writing the paper, and DF participated in patient;s treatment, co-wrote the first draft and performed all the revisions.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.


Vulvar basal cell carcinoma (BCC) is rare, accounting for less than 5% of all vulvar neoplasms and less than 1% of all BCCs. Vulvar BCCs are usually diagnosed late because they are often asymptomatic and tend to grow at slow rates. They may be invasive and destructive if neglected or improperly treated. Nevertheless, they have a very low propensity for metastatic spread, but frequently recur after simple excision. We report a 78 year-old woman presenting with the complaint of painful vulvar ulceration and vaginal bleeding. The physical examination revealed a 3 × 2 cm indurated nodulo-ulcerative lesion involving the clitoris, both labia minora and left labium majus. The histopathology was consistent with the “solid type BCC” that invaded the subcutaneous tissue without lymph node metastasis. The patient underwent wide local excision with clitoral amputation and remained disease free at post-surgical follow-up after 18 months. Basal cell carcinoma (BCC) is the most common malignancy of the skin, accounting for approximately 70–80% of all cutaneous cancers.556In this paper, we report an unusual case of locally invasive BCC located at the clitoris extending to the labia minora and left labium majus of a 78-year-old woman treated with wide local excision.A 78-year-old female presented with complaints of painful vulvar ulceration and bleeding for at least 4 months. She had been evaluated first by the gynecologist and then she was referred to us. She did not have a history of an antecedent skin disease or local irritation. She was hypertensive and underwent a total abdominal hysterectomy because of the intramural leiomyomas 6 months ago. She denied any personal or family history of skin cancers or internal malignancies. There was no history of sexually transmitted disease, irradiation or tobacco use. Physical examination revealed multiple indurated nodular lesions on the clitoris and a highly indurated ulcerous lesion (3 × 2 cm) with an elevated telangiectatic border on the labium majus . The lefVulvar BCC is a rare malignancy accounting for approximately 2–3% of all vulvar neoplasms.23323365


Primary Non Hodgkin's Lymphoma (NHL) of the lacrimal sac is rare.The clinical features of a 78 year old female who presented with epiphora and swelling of the left lacrimal sac are described.Computerised tomography showed a mass involving the left lacrimal sac. Histopathological examination revealed a diffuse large B cell NHL. Immunohistological examination demonstrated B cell origin. Chemotherapy could not be administered due to co morbid conditions. The patient was treated with radiotherapy to a dose of 45 Gy in 25 fractions. Patient is disease free and on follow up after 36 months.Primary radiotherapy is a treatment option with curative potential for localized NHL of the lacrimal sac and may be considered in patients who cannot tolerate appropriate chemotherapy. Tumors involving the nasolacrimal drainage system are rare and more than 90% of these tumours are of epithelial origin . MajoritIn this report, we present the clinical features of a patient with primary NHL of the lacrimal sac treated successfully with radiotherapy.A 78 year old female patient presented with epiphora of the left eye of 6 months duration and a painless swelling of the left lacrimal sac area of 2 months duration. Physical examination showed a firm, non-tender mass measuring 3 × 4 cm in the right lacrimal fossa. Irrigation test revealed complete obstruction of the lacrimal drainage system. Rest of the eye and adnexae were normal. Computerised tomography (CT) scan of the orbit showed a heterogeneous soft tissue mass arising from the lacrimal sac, infiltrating and pushing the globe laterally and forward, causing exophthalmos with no bony erosion figure . There wAn incision biopsy was performed from the mass and pathological examination of revealed diffuse large cell lymphoma. Immunohistochemistry demonstrated malignant clonal cells of B cell origin (CD20 positive), lacking T cell antigens. Physical examination, biochemical tests, CT scan of the chest and abdomen, and bone marrow biopsy did not show evidence of lymphomatous involvement elsewhere. The patient had diffuse large B cell Non Hodgkin's lymphoma of the right lacrimal sac, stage IE by the Ann Arbor staging system and IPI score of 2 (low intermediate).The patient was treated with radiotherapy to a dose of 45 Gy in 25 fractions over 5 weeks by three dimensional conformal lens sparing technique. Systemic chemotherapy was not proposed in view of the advanced age of the patient, poor performance status and concurrent co morbidities. The patient continues to be on follow up after 36 months with no evidence of local or systemic recurrence and preservation of visual acuity.Primary lymphoma of the lacrimal sac is rare, with most reported cases representing secondary involvement from systemic lymphoproliferative malignancy . Only 16The typical presentation of a lacrimal sac tumour is as a medial canthal swelling with nasolacrimal duct obstruction. The differentials for a lacrimal sac mass include nongranulomatous inflammation and granulomatous infections, dermoid cyst, mucocele, lipoma, lymphangioma and tumors of the lacrimal duct like adenocarcinoma, squamous cell carcinoma, adenoid cystic carcinoma, hemangiomas, sarcomas, melanoma, lymphoma and secondaries.et al [et al [Nasolacrimal sac tumors may arise within the sac or from structures surrounding the sac or duct. In previous reports of primary NHL of the lacrimal sac, Jordan and Nerad  observedet al  demonstrl [et al  treated The vast majority of NHLs of the orbit and ocular adnexa are of B cell origin. T cell lesions are rare and usually are associated with disseminated cutaneous lymphomas, such as mycosis fungoides, Sézary syndrome, or adult T cell leukemia. Primary lacrimal sac lymphoma also appears to be commonly of B cell origin and the usual subtypes are either indolent types like MALT lymphoma or extranodal marginal B cell lymphoma or aggressive subtypes like diffuse large B cell lymphoma .The treatment of lacrimal sac tumors of epithelial origin is mainly surgical. However, in cases of orbital lymphoma extensive surgery is to be avoided for adequate cosmesis and functional preservation of the eye -14.Chemotherapy is considered the standard treatment for localized aggressive NHL with either CHOP based chemotherapy alone or limited cycles of chemotherapy followed by a lower dose of involved field radiotherapy after a good response . ChemothRadiotherapy which is considered the standard treatment for indolent orbital lymphomas, has also been found to be successful in aggressive NHL in some reports ,13,14. TThe present result suggests that radial radiotherapy alone may be a treatment option with curative potential for primary localized NHL of the lacrimal sac and may be considered in patients who cannot tolerate appropriate chemotherapy.The author(s) declare that they have no competing interests.RV, MKG conception and design, acquisition of data, analysis and interpretation of data have been involved in drafting the manuscript, revising it critically for important intellectual content and have given final approval of the version to be published.


Capillary hemangiomas of spinal nerve root, mostly affecting the cauda equina, are extremely rare.A 20-year old man was presented with back pain, radiculopathy, and urogenital symptoms. Magnetic resonance images revealed an intradural extramedullary mass, isointense in T1-weighted and hyperintense in T2-weighted images, with noticeable post injection enhancement. The clinical and radiological findings simulated neurinoma.However, a pinkish lesion was removed surgically and histopathological examination revealed lobules of capillary vessels separated by fibrous tissue, suggesting capillary hemangioma.Although rare and sometimes indistinguishable from more common lesion, capillary hemangioma should be in differential diagnosis of any enhancing intradural extramedullary mass at the level of cauda equina or conus medullaris. Tumors of cauda equina account for only 6% of all spinal tumors. The diffIn this report, a case of capillary hemangioma of cauda equina is described and different aspects regarding diagnosis, treatment and pathophysiology of the lesion, and differentiation from more common intradural extramedullary conditions are discussed.A 20-year old man presented with three months history of low back pain radiating to the legs. He also complained of urinary retention, impotence, and retrograde ejaculation of recent onset. He had bilateral weakness in knee flexion and extension of 3/5. There was also parasthesia of both feet and asymmetrical diminished deep tendon reflexes in both lower extremities, which was worse in right side.On plain radiography, evidence of vertebral scalloping and widened foramen at the level of L3 was considered probable Figure . MagnetiRegarding its location and MR appearance, the lesion was considered a cauda equina scwhanoma. Surgical resection was performed through a midline posterior approach. Following a one-level laminectomy at L3, midline duratomy was performed and a pinkish intradural mass was identified. The tumor was attached tightly to one of the rootlets, albeit there was no dural attachment. The adjacent radicular vessels seemed to be enlarged. The mass had squishy consistency with high bleeding tendency. Enbloc resection was performed to reduce blood loss.Histopathological assessment revealed encapsulated mass with lobular components containing small size capillary vessels lined by a single layer of flattened endothelial cells figure . There wFollowing surgery, the patient's motor symptoms were entirely alleviated, and urogenital problems improved marginally. During 1 year follow-up, there was no clinical or radiological evidence of recurrent disease.Vascular tumors account for about 2% to 7% of all intraspinal tumors.  Amongst Haemangiomas of the cauda equina may present with intermittent low back pain, intermittent radicular symptoms, sensory deficits, saddle anaesthesia, paraplegia, sphincter dysfunction, symptoms of hydrocephalus, and subarachnoidal hemorrhage.  Indeed, The diagnosis can be established by MRI. The mass usually appears isointense relative to the spinal cord on T1-weighted, and iso- or hyperintense on T2-weighted MR images. Gadolinium administration results in uniform intense enhancement of the lesion.  After a The signal characteristics may help differentiate hemangioma from more common intradural extramedullary tumors.,7 MeningIn the spinal intradural extramedullary space, hemangiomas may arise from the blood vessels of the nerve roots in the cauda equina, the inner surface of the dura, or the pial surface of the spinal cord.  Two hypoSurgery is the treatment of choice for spinal intradural capillary hemangioma, and most of the symptoms would be reversed postoperatively. ,5On histopathological specimens, capillary hemangiomas usually are of lobulated structures of capillary-sized vessels lined with flattened endothelium, with the lobules being separated by a collagenous stroma.  The lesiAlthough intradural capillary hemangioma is a rare entity and may be clinically or radiologically indistinguishable from other lesions, it should be considered as the differential diagnosis of any intradural extramedullary mass at the level of conus medullaris or cauda equina.Written informed consent was obtained from the patient and his parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SMM made contribution to conception and analyzed the patient data. ZH made contribution in collecting data and drafting the manuscript. MH was contributor in drafting the manuscript. ATM was contributor in revising the manuscript critically.SAFT has given the final approval for the version to be submitted.