Wilms’ tumor (WT) and neuroblastoma (NB), the two most common extra-cranial solid malignant tumors, are seldom seen together in the same patient. A 10-month girl presented with a right retroperitoneal mass. A preoperative diagnosis of Wilms’ tumor (WT) was made. She was given preoperative chemotherapy followed by surgery. At surgery a renal mass (WT) and a suprarenal mass (neuroblastoma – NB) were removed. She finally succumbed to metastatic NB in the postoperative period. Simultaneous occurrence of WT and NB often leads to confusion in diagnosis, staging, and management. No clear guidelines are available about which malignancy to target first with the appropriate adjuvant therapy. Here we present a rare case of synchronous WT and NB in a girl.A 10-month old girl presented with history of abdominal distension since 45 days associated with fever, cough and weight loss. There was a history of malignancy in multiple members of the maternal side of the family; however the exact details were not available. On examination a right-sided abdominal mass was found. On ultrasonography a 9 cm x 7 cm heterogeneous predominantly echogenic mass with multiple internal anechoic areas along anterolateral aspect of right kidney, causing its posterior displacement was reported. There was another well-defined 4.8 cm x 4.3 cm heterogeneous, predominantly echogenic mass in right suprarenal region causing inferior displacement of the kidney. Contrast enhanced computed tomography (CECT) showed a similar picture of the two masses . Chest radiograph showed no lesion. Trucut biopsy from the mass showed tubular and papillary structures lined by tall columnar cells, showing nuclear hyperchromasia with atypia and mitosis, suggestive of epithelial component of WT.As per SIOP protocol, a diagnosis of WT  was made and the patient was given vincristine and actinomycin-D for four weeks. Repeat CECT did not show significant reduction in tumor size. Surgery was then planned. On exploration, there was a 15 cm x 10 cm x 8 cm mass involving the right kidney, and another 5 cm x 5 cm x 3 cm mass located separately above the right kidney in the region of suprarenal gland . Enlarged supra-hilar, hilar, infra-hilar and mesocolic lymph nodes were also found. Patient underwent right nephroureterectomy with excision of suprarenal mass and lymph node sampling. There was no tumor spill. In postoperative period patient developed adhesive intestinal obstruction requiring repeat exploration with adhesiolysis on day 5.The final histopathology report showed WT of the right kidney with predominantly epithelial component (resected margins were free of the tumor) . The right suprarenal mass showed undifferentiated NB involving the capsule . The hilar and infra-hilar nodes showed tumor deposits of WT. A thorough work-up for risk factors and metastasis of neuroblastoma was done. It included skeletal survey, meta-iodobenzyl guanidine (MIBG) scan, 24-hour urinary vanillyl mandelic acid levels and bone marrow aspiration biopsy. They were within normal limits. However, N-myc amplification in tumor tissue was positive. Thus, a final diagnosis of WT stage III (intermediate risk) with NB stage I (high risk) was made. The WT was of higher stage and was thus targeted first with radiotherapy (21 Gy radiation over 5 weeks) given to tumor bed with opposite kidney shielding. The patient was then put on vincristine (oncovin), cisplatin, etoposide and cyclophosphamide (OPEC chemotherapy) for NB. During the second cycle of chemotherapy she developed metastatic NB in the left supraclavicular lymph nodes. Repeat CECT scan showed marked retroperitoneal lymphadenopathy and MIBG scan showed suspicious increased uptake in left superior mediastinum. Thereafter, the child had a stormy clinical course. She developed left sided hemorrhagic pleural effusion  with respiratory distress that was not responding to intercostal drain insertion. In view of unresponsiveness to OPEC, she was begun on rapid COJEC  chemotherapy. However, her clinical condition continued to deteriorate. She developed decreased oral intake as well as axillary lymphadenopathy followed by abdominal distension and finally succumbed to metastasis.There are few case reports of the simultaneous occurrence of WT and peripheral neuroectodermal tumors (PNET).[1-6] All reported cases of WT associated with NB died. There is also a strong association with vertebral, anorectal, cardiac, tracheoesophageal, renal and limb anomalies (VACTERL association) as well as with Fanconi’s anemia (FA). FA is a genetic  disorder, which manifests in childhood with various developmental defects.[7] It is associated with an increased risk of solid malignancies.[8]There was difficulty in the preoperative diagnosis as two growths were mistaken as one in spite of imaging. Thus biopsy of only the renal mass was taken. This led to a significant delay in the diagnosis of NB and initiation of appropriate chemotherapy. Although the WT was stage III and NB was only stage I, the patient finally succumbed because of NB. This highlights the negative effect of N-myc amplification on the prognosis.Source of Support: NilConflict of Interest: None declared


Introduction: Solitary fibrous tumor is associated with serosal surfaces. Location in the salivary glands is extremely unusual. Extrathoracic tumors have an excellent prognosis associated with their benign clinical behavior. We report an aggressive and recurrent case of this tumor. We review the clinical presentation, inmunohistochemical profiles and therapeutic approaches.Case Report: A 73-years-old woman presented a mass in her right parotid gland. She had a past history of right superficial parotidectomy due to a neurilemoma. FNAB and magnetic resonance were non-specific. After a tumor resection, microscopic findings were spindled tumor cells with reactivity to CD34, bcl-2 and CD99 and the tumor was diagnosed as Solitary Fibrous Tumor. The patient suffered two recurrences and the tumor had a histological aggressive behavior and a destruction of the cortical bone of the mandible adjacent to the mass. A marginal mandibulectomy with an alveolar inferior nerve lateralization was performed. Conclusions: Solitary fibrous tumor is a very rare tumor. Usually, they are benign, but occasionally they can be aggressive. Complete resection is the most important prognostic factor and no evidence supports the efficacy of any therapy different to surgery. Due to the unknown prognosis and to the small number of cases reported, a long-term follow-up is guaranteed. Key words:Solitary fibrous tumor, parotid mass, parotid gland, salivary gland, rare tumors. Solitary fibrous tumor was first described in the pleura by Lietaud in 1767, followed by Wagner in 1870. Klemperer and Rabin, in 1931, classified pleural tumors into two types: diffuse mesotheliomas and localized mesotheliomas or solitary fibrous tumor [SFT].SFT is one generally associated with serosal surfaces, but in recent years it has been reported in extrapleural sites such as the liver, adrenal gland, skin, and less commonly in the head and neck, accounting for around 6% of the cases . In 1991These tumors tend to present as a well defined, palpable and slowly growing masses. Clinical presentation and radiographic findings are similar to that of other tumors which are more common in salivary glands.Extrathoracic SFT has an excellent prognosis associated with their benign clinical behavior. We report an atypical and recurrent case of SFT with aggressive behavior. This article reviews the clinical presentation, histologic features, inmunohistochemical profiles and therapeutic approaches of SFT of the parotid gland in the context of a case report.A 73-year-old woman presented with a several months history of a mass in the right parotid gland region. She had a past medical history of right superficial parotidectomy due to the presence of a neurilemoma [cellular schwannoma] which had been treated 17 years previously in another medical center. She had hypertension and hypertriglyceridemia, but no toxic habits.She presented with a 2 cm palpable, firm, mobile, painless and slowly growing mass in her right parotid gland. There was no facial paralysis or constitutional symptoms and no palpable nodes on neck examination. FNAB was unspecific. Magnetic resonance imaging revealed a well-defined tumor measuring 2 cm in its greatest dimension, in the supposed location of the superficial lobe of the right parotid gland. It showed high signal intensity with homogeneous enhancement in T2-weighted images Fig. . The priThe patient underwent a tumor resection. The specimen was well-defined and not encapsulated, measuring 2 x 2 cm with a white surface. Microscopic findings were that of tumor cells arranged in a storiform pattern with numerous ramifying vessels and hyalinized walls Fig. . The tumTwo years later, the patient suffered a recurrence. She developed a mass measuring 6 cm localized under the masseter muscle. During the excision of the tumor, destruction of the adjacent mandibular cortical bone was observed. The margin of the tumoral resection in this location was microscopically positive.A new magnetic resonance imaging and a computed tomography were carried out Fig. . An heteIn this context, a marginal mandibulectomy with an alveolar inferior nerve lateralization was performed Fig. . We confThe clinical aggressiveness of bone destruction correlated histologically with an increased in Ki67 index [15%] and mitotic rate. Microscopically, bone remodelling without infiltration was observed. The patient has remained free of disease for 9 months postoperatively.et al. (SFT is an uncommon benign lesion of mesenchymal origin. The finding of SFT in the head and neck is rare. Moreover, very few cases affecting the partotid gland have been described in the literature. There is no predilection based on age or gender. Bauer et al.  presenteThese tumors usually present as a well defined, palpable, painless and slowly growing mass. Occasionally, they can produce compression symptoms which should prompt suspicion of extension to the parapharyngeal space . ClinicaTherefore, a diagnosis of SFT can only be performed based on histological and immunohistochemical findings. The macroscopic examination of the tumor shows a well-defined and encapsulated mass which may be accompanied by bone destruction, though it is normally free of infiltration. The presence of bone destruction should prompt a suspicion of malignancy, though this finding is not an indispensable requisite as a malignant tumor can have no bone erosion. Bone destruction can be attributed to a long-standing pressure effect . In addiMicroscopic findings of SFT include tumor cells arranged in a storiform pattern with numerous ramifying vessels and hyalinized walls. There are alternating zones of hypercellularity and hypocellularity in fibrous areas. Tumor cells are round to spindled with tapering cytoplasm. Histologically, a diagnosis of SFT is also difficult because there are many tumors that display similar findings.For this reason, immunohistochemical examinations are required in order to make a differential diagnosis. SFT shows reactivity to CD34, bcl-2, vimentin and CD99, but is negative to keratin, EMA, S-100, desmin, smooth muscle actin and muscle-specific actin. The presence of EMA, any keratin or S-100 protein will rule out many common salivary gland tumors such as pleomorphic adenoma. CD34 is a hematopoietic progenitor cell antigen that is strongly positive in most cases of SFT, though it may not be positive in all cases . CD34 teet al. (et al. (et al. (Complete local surgical excision with negative microscopic margins is the single most important prognostic factor in SFT. Some authors have documented disease recurrence at other extrapleural sites decades after the primary tumor was first identified  and for et al.  report a (et al.  also add (et al.  present et al. (Ganly et al.  suggest In the case we presented, there were both clinical and histological indications of aggressiveness. However, in the end we were able to successfully achieve negative microscopic surgical margins. We presented the case to the Head and Neck Oncology Committee and decided that no adjuvant therapy was necessary.The prognosis of this tumor is generally unknown. Most extrathoracic SFT have an excellent prognosis, but some authors have reported cases with aggressive behaviour and multiple recurrences, invasion of local tissues, as well the presence of distant metastases ,14.


Cloacal exstrophy is a very rare and complex malformation. We report a neonate of cloacal exstrophy with mature teratoma presenting as a component of exstrophy. To our knowledge this has not been reported in the literature.  A female full term baby (2.6 kg) was born by caesarean section to a 25-year-old primigravida with no antenatal history of any infection, drug intake or radiation exposure to mother. The marriage was non consanguineous. The baby cried immediately after birth. APGAR score was 8/10. Examination showed stable vital parameters, presence of a protruding and exposed bowel and bladder plates in the lower part of abdomen and perineum. Urine was draining from left ureteric orifice in the bladder plate and meconium seen coming from the bowel plate. There was a hair bearing structure on right side of bowel plate and another cystic structure in the perineum continuous with it. There were no palpable gonads or phallus like structure seen . Systemic examination of baby was normal. Spine x-ray revealed normal spine with radio-opaque mass in the pelvis . Ultrasound of abdomen showed prominence of right pelvicalyceal system and dilated left ureter. A mass was seen in presacral region in midline in pelvis. USG spine was normal for posterior vertebral elements, para-spinal muscles, and spinal cord. Echocardiogram was normal. Immediate management was directed to stabilization of the infant and the bowel and exposed tissues were covered with warm saline soaked gauze and protective plastic dressing. After counseling of parents regarding guarded prognosis and staged repair, baby was taken for surgery on day of life 3. Operative steps: Bilateral ureteric orifices were cannulated with 5fr infant feeding tube. Teratoma component had parts of head like structure with hairs, cyst like structures and a bony component which was attached to anterior part of sacrum. Soft tissue component and easily accessible part of bony component were removed. Bony component could not be removed completely due to close proximity to sacrum. Bowel plate freed from the bladder plate and fashioned into end colostomy leaving the patient with whole of small intestine, ileocecal junction, cecum, appendix and part of ascending colon. The two halves of bladder plate separated and then joined in the midline creating the posterior wall of bladder. Right and left hemivaginal openings were kept in the bladder plate below openings of bilateral ureters. Histopathological examination of presacral mass revealed glandular structure, mesenchyme with vessels, smooth muscles, skeletal muscles, adipose tissue. No immature element seen. Separately sent bony tissue showed bony trabeculae with hematopoietic marrow. These features were suggestive of mature teratoma.Patient is on regular follow up for 18 months. Patient has been admitted several times for failure to thrive. At age of 2 years baby weighs 6.5 kg, can stand and walk without support. MRI pelvic floor muscles showed atrophic coccygeus muscle while other pelvic diaphragm muscles were not appreciated. Considering poor general condition and minimal chance of urinary and fecal continence after any definitive procedure patient is being managed conservatively. During the fourth and seventh weeks of gestation, the cloaca is subdivided by the urorectal septum to form the anorectal canal and the primitive urogenital sinus.[1] A simultaneously occurring sacrococcygeal teratoma could encroach between the layers of the cloacal membrane and prevent descent and fusion of the urorectal septum to the cloacal membrane. An anterior location of the tumor could thus result in the absence of rectum and anus. The physical presence of a teratoma could also prevent fusion of the genital folds, resulting in a bifid scrotum and hypospadias [2] in male neonate. In our case this same mechanism could be responsible for the external appearance of bifid bladder plates, anorectal malformation, bifid vagina with part of tumor protruding externally. The massive growth of teratoma in presacral space could have hindered the development of Mullerian structures in our patient. Cloacal exstrophy is associated with many other anomalies including cardiovascular, central nervous system, omphalocele (70-90%), vertebral anomalies (46%), upper urinary tract (42%), malrotation (30%), lower extremity anomalies (30%), double appendix (30%), absent appendix (21%), short small bowel (19%), small bowel atresia (5%), and abdominal musculature deficiency (1%) [3]. Upper urinary tract anomalies include pelvic kidney, horseshoe kidney, hypoplastic kidney and solitary kidney [4]. Vertebral malformations include sacralization of L5, congenital scoliosis, sacral agenesis, and interpedicular widening. To the best of our knowledge association of cloacal exstrophy with sacrococcygeal teratoma has not been described in literature.Immediate management of exstrophy patients is directed to the medical stabilization of the neonate. For infants who have minor associated malformations and are medically stable, staged closure can be considered within 48-72 hrs after birth. Evaluation of the genitalia and gender assignment should be made by a gender assignment team, including a pediatric urologist, pediatric surgeon, pediatrician, and pediatric endocrinologist. The initial operation consists of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and re-approximating, closing, or leaving the exstrophied bladder undisturbed. In our case, management of teratoma was additional challenge as it was large and extending deep in to the pelvis. We excised the teratomatous component as much as possible thereby helping the bladder plates to get approximated. Bilateral hemivaginal openings kept in bladder plate below ureteric openings. Hindgut segment was brought out as end colostomy thus avoiding ileostomy and its complications.Source of Support: NilConflict of Interest: Nil


Synovial sarcoma (SS) primarily occurs in the para-articular soft tissue of the lower extremities in young adults and it is extremely rare in the prostatic region. We report a case of a 46-year-old man who presented with urinary retention. Pelvic ultrasound (US) examination, computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated an 8.5 cm mass that appeared to originate in the prostatic fascia of the right lobe. Preoperative prostatic ultrasound transrectal needle biopsy revealed mesenchymal neoplastic tissue. Patient underwent surgery. The final pathologic findings were consistent with the diagnosis of monophasic synovial sarcoma. Synovial sarcoma (SS) is a soft tissue sarcoma of uncertain histogenesis, chiefly occurring in young adults, primarily in the para-articular region of the extremities. Other unusual sites include the middle ear, orofacial or oropharyngeal region, esophagus, larynx, lung, pleura, heart, blood vessels, abdominal wall, gastrointestinal tract, and retroperitoneum; involvement of the lower genitourinary tract is exceedingly rare. Although a variety of sarcomas, including rhabdomyosarcoma, fibrosarcoma, leiomyosarcoma, and stromal sarcoma, have been described in the prostate, SS is a rare occurrence, with only seven previously reported cases –6. The pA 46-year-old man presented with urinary retention. On digital rectal examination a large prostatic mass was palpable. His serum prostate-specific antigen (PSA) level was 1.03 ng/mL . Pelvic ultrasound (US) showed an 8 × 7.5 × 8.5 cm lesion localized in the prostatic region and behind the bladder with heterogeneous mixed echogenicity .Computed tomography (CT) demonstrated a well-marginated soft tissue tumor that appeared to originate in the prostate and extend to the retrovesical soft tissue, with fluid cystic and solid structure .No metastasis is evident. Magnetic resonance imaging (MRI) confirmed the origin of the mass in the prostatic fascia of the right prostatic lobe, its prevalent cystic myxoid component with septa and irregular eccentric solid tissue homogeneously enhanced after gadolinium. Right seminal vesicle was compressed.Posteriorly the lesion was in close contact with the rectum, laterally with the right obturator internus muscle and inferiorly with the levator ani muscle .Preoperative prostatic ultrasound transrectal needle biopsy revealed mesenchymal neoplastic tissue. Patient refused to receive radical prostatectomy.The patient underwent debulking surgical removal. The extension of the mass has prevented its complete removal.Routine hematoxylin eosin stained microscopical sections showed a spindle cell neoplasm made up of small, uniform spindle cells with a high nuclear-cytoplasmic ratio, and finely stippled chromatin. The neoplastic cells were closely packed, growing in short interlacing and intersecting fascicles around a branching, “hemangiopericytoma-like vasculature” . The mitThe fluorescence in situ hybridization (FISH) test for t(p11q11) was positive showing splitting of the fluorescent signal consistent with a rearrangement involving SYT . The pat2) and ifosfamide (2500 mg/m2) was offered at days 1, 2, and 3 of fortnightly cycles. At 3 months of follow-up, after 6 cycles of chemotherapy, pelvic RMI showed persistence of the neoplasm, with cystic and solid component, confined in the prostatic loggia and with invasion of the right prostatic lobe  of the tumors of his study were markedly cystic .The signal heterogeneity was described by Jones et al.  in theirIn a study of 18 SS Liang et al. reported that all cases demonstrated areas of hyperintensity, isointensity, and hypointensity relative to the muscle. Hyperintensive signals on T1-weighted and T2-weighted were observed in necrotic and cystic areas. Nine cases demonstrated a hypointensive T1 signal, suggesting hemorrhage. Three cases with hypointensive T1 signals were considered to possess fibrous tissue. In total, 12 lesions exhibited a hypointensive T2 signal with internal septations. Contrast-enhanced images revealed heterogeneous lesions, with no enhancement in the areas containing cysts, necrosis, or septation .The survival of patients with primary SS of prostatic fascia is poor, within a few months after diagnosis and aggressive resection surgery that remains the current treatment of choice for locally confined lesion. It is uncertain whether chemotherapy has a substantial effect on long-term survival. Documentation of further cases is needed to establish appropriate therapy.


Myeloid sarcoma is a rare extramedullary malignant tumor, which is often accompanied by the development of systemic myeloid disease at various sites. The involvement of the central nervous system is uncommon and spinal cord compression is particularly rare. In November 2012, a 27-year-old male presented with a paroxysmal headache, accompanied by nausea and vomiting, which had persisted for one year, and eyesight deterioration that had been apparent for five months. Magnetic resonance imaging (MRI) indicated a space-occupying disorder, a craniotomy to resect the brain tumor was undertaken, the pathological diagnosis of which was myeloid sarcoma. Two months after receiving 40 Gy of radiotherapy, the patient experienced numbness of the right thigh. MRI of the lumbar spinal canal revealed a mass involved both inside and outside the lumbar spinal canal. Pathological examination of the mass following resection also indicated myeloid sarcoma. Immunohistochemical analysis was positive for the ETO fusion gene in the bone marrow. Following six cycles of chemotherapy treatment, the patient achieved complete remission. At present, the patient is stable and is attending follow-up examinations regularly. Myeloid sarcoma is a malignant tumor consisting of myeloblasts, with or without maturation, at anatomical sites other than the bone marrow ,2. MyeloIn November 2012, a 27-year-old male was admitted to the Beijing Tiantan Hospital  with a paroxysmal headache that had persisted for one year and numbness of the right thigh that had been apparent for two months. The patient had experienced occasional paroxysmal headaches accompanied by nausea and vomiting for one year. In the five months prior to admission, the patient’s eyesight had deteriorated. The patient also exhibited an unsteady gait due to the numbness of the right thigh. Magnetic resonance imaging (MRI) of the head revealed a space-occupying disorder beside the bilateral occipital sinus, which involved a posterior region of the superior sagittal sinus. The patient underwent a craniotomy for the resection of the brain tumor in April 2012. The tumor mass was grey/white in color, pliable and located in the bilateral occipital lobes. The mass exhibited clear borders and a rich blood supply, had adhered slightly to the surrounding brain tissues and had invaded the sagittal sinus, with evidence of tumor thrombosis. The surgery removed a 38×69×3-mm section of brain tissue. The post-surgical biopsy identified a myeloid sarcoma positive for myeloperoxidase (MPO), cluster of differentiation (CD)34 and Ki-67 (50%). Subsequently, the patient underwent routine post-operative radiation therapy  for one month. In September 2012, the patient had suffered hypoesthesia and pains primarily on the right side in the anogenital region and along the anterior and inner femoral surfaces. The patient had also experienced difficulty in defecating. A lumbar MRI scan revealed a space-occupying lesion inside and outside of the lumbar spinal tube. In October 2012, a repeat MRI identified multiple space-occupying disorders in the lumbar spinal canal of L2–3 and S2–4. After three days, surgery was performed to resect the majority of the tumor mass, which subsequently relieved the pain in the right thigh. The post-operative biopsy confirmed a myeloid sarcoma. Further immunohistochemical analysis revealed that the neoplastic cells were positive for MPO, CD34, lysozyme, vimentin, CD99, CD56, CD68 and Ki-67 (20%), but negative for CD3, CD20, glial fibrillary acidic protein, CD79α and cytokeratin. A physical examination revealed that the superficial lymph nodes, liver and spleen were impalpable, but there was no tenderness of the sternum. Bilateral visual acuity was reduced and the lower visual field was contracted. Furthermore, the superficial sensations in the front and inside of the right thigh and perineal region were reduced. The morphological analysis, immunophenotyping and chromosome genotyping of the bone marrow were normal. The ETO fusion gene was positive, with a quantity of 0.44%. Myeloid-derived tumor cells, expressing CD117, CD34, CD33, CD56 and human leukocyte antigen-DR were detected in the cerebrospinal fluid and accounted for 1.49% of the total cellular components.Subsequent to the admission to the Beijing Tiantan Hospital in November 2012, the patient was administered chemotherapy in order to treat the myeloid sarcoma. The daily therapeutic regimen consisted of 10 mg idarubicin and 1.5 g cytarabine for three days, with intrathecal injections of 10 mg methotrexate and 5 mg dexamethasone. Following two rounds of chemotherapy, the patient’s eyesight returned to normal and the numbness of the right thigh was markedly relieved. Polymerase chain reaction amplification of the ETO fusion gene and immunophenotyping of the cerebrospinal were negative. MRI revealed that the spinal tumor mass had significantly decreased in size  and 2. TMyeloid sarcoma, which was previously known as granulocytic sarcoma or chloroma, is an uncommon malignant tumor characterized by the extramedullary blast proliferation of myeloid lineages that subsequently destroy the normal architecture of adjacent tissues . Three cet al (Due to its low incidence, primary central nervous system myeloid sarcoma has not been widely reported. Myeloid sarcoma, as a group of heterogenetic diseases, presents with differential features depending upon the original affected site. Once a pathological diagnosis has been established, comprehensive evaluations, including bone marrow morphology, immunophenotyping, chromosomal banding and fusion gene analyses should be performed in order to determine an optimum treatment regimen . A studyet al  revealedet al .Due to the risk of leukemic cell metastasis to the spinal leptomeningeal tissues via the cerebrospinal fluid during the surgical excision of intraparenchymal myeloid sarcomas , a combiin situ hybridization, there was no evidence of leukemia based upon the morphological, immunophenotyping and chromosome band analyses of the bone marrow, which supports the hypothesis that the myeloid sarcoma occurred outside of the bone marrow. If the patient had received a bone marrow examination at the time of disease onset, an improved outcome may have been achieved following chemotherapy. As primary intracranial myeloid sarcoma is rare, further investigations are required in order to achieve an improved understanding of the mechanisms involved in the pathogenesis of myeloid sarcoma and to identify the neurophilic features that precede the onset of clinical leukemic disorders.The present study described a case of myeloid sarcoma with primary intracranial lesions. The myeloid sarcoma recurred beside the lumbar vertebrae subsequent to surgery and radiation therapy. Although an ETO fusion gene was detected using fluorescence


Paragangliomas (PGL) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, which makes diagnosis often challenging. Malignant paraganglioma is defined by the presence of this tumor at sites where chromaffin cells are usually not found or by local invasion of the primary tumor. Recurrence, either regional or metastatic, usually occurs within 5 years of the initial complete resection but long-term recurrence is also described. Malignancy is often linked to a SDHB mutation. Preoperative embolization has been applied in the surgical management of PGLs with the objective to decrease intra-operative blood loss and surgery length without complications.We report two cases of patients with abdominal or pelvic malignant PGLs who have been treated surgically at our center after preoperative embolization. Surgery was a very challenging procedure with multiple surgical teams involved and embolization did not prevent major blood loss and intraoperative complications. Patients required adjuvant treatment with either chemotherapy or radiotherapy.Many studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposing. Surgical resection is the mainstay of treatment, but a multimodal approach is often required due to the complexity of cases.  The role of preoperative embolization is not established and in our experience it has provided little benefit and major complications. Paragangliomas (PGLs) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. They may extend from the skull base to the mediastinum following the vagus nerve, but may also occur in the abdomen. Most of intra-abdominal PGLs present at the organ of Zuckerkandl, located on the left of the aorta near the origin of the inferior mesenteric artery or between the aorta and left renal vein. When these tumors manifest in the adrenal glands, they are classified as pheochromocytomas .Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, as in most head and neck PGLs. Often, the diagnosis is made incidentally after imaging or family screening performed in certain syndromes .The incidence of PGL varies from 0.2 to 0.8 per 100,000 persons per year with a peak incidence between 30 and 40 years of age in the general population. The average age of diagnosis in the pediatric population is 11 years, but there are case reports as young as 5 years of age [Malignant paraganglioma is defined by the presence of tumor in sites where chromaffin cells are usually not found or by local invasion of the primary tumor. The rate of metastatic disease is very variable in the literature, with reports ranging from 2.5 to 40 %. Recurrence, either regional or metastatic, usually occurs within 5 years of initial complete resection, but there are reports of recurrence up to 40 years postoperatively . AlthougAn 11-year-old boy presented with a growing but painless pelvic mass for the past 2 years. He denied headaches or tachycardia and presented in good clinical conditions. He was submitted elsewhere to two surgical attempts of tumoral resection 12 and 6 months previously, both being unsuccessful due to diffuse bleeding. In the second procedure, a biopsy was performed, which revealed large cells with eosinophilic cytoplasm positive for S-100 and chromogranin, suggestive of PGL.Physical examination was normal except for an infra-umbilical midline scar and a large abdominal mass visible and palpable in the hypogastric region. Laboratory exams were unremarkable, except for increased urinary excretion of noradrenaline 571 μg/24 h) and 4-OHmetoximandelate (13.2 mg/24 h). Abdominal ultrasound disclosed a well-defined retroperitoneal mass in the left paraaortic region below the renal vessels. Abdominal MRI revealed a large mass with intense vascularization, anterior to the aorta and iliac bifurcation  and 700–1000 μ particles of Pathological evaluation revealed that the specimen measured 12.0 × 9.0 × 7.0 cm and weighed 463 g. Sectioning revealed a multilobular, heterogeneous tumor mass with foci of hemorrhage and necrosis. Microscopically, the tumor displayed intense cell pleomorphism and mild nuclear enlargement. Chromogranin A, synaptophysin, and S-100 stains were positive. Mitotic index was 3/10, there were no signs of extracapsular invasion but vascular invasion was confirmed.After 6 months, the patient is in good health, without abdominal or urinary symptoms, but presented with paresthesia of the left thigh in the early postoperative period with no clinical improvement after 3 months. There is no sign of local abdominal recurrence. The patient received radiotherapy for the left femoral metastasis and is currently being treated with systemic chemotherapy. The patient was referred for genetic testing and awaits final results.A 12-year-old male presented with left flank pain for the past 3 months. He noticed an abdominal mass growth during the last month along with a left scrotal swelling. He denied having headaches, palpitations, or high blood pressure. Physical examination revealed a left upper quadrant abdominal mass and left varicocele. The patient had no clinical signs of catecholamine release, and this tumor was considered biologically inactive despite a slightly elevated serum dopamine . Therefore, no preoperative alpha-adrenergic blockade was done, and the patient remained hemodynamically stable throughout the surgery. A computerized tomography (CT) scan of the abdomen showed a congenital solitary left kidney and a 12-cm heterogeneous, markedly hypervascular mass located on the left paraaortic region with apparent involvement of the renal artery  revealed no abnormal uptake. Arteriography was performed to identify blood supply to the tumor Fig. . Aiming At surgery, preoperative ureteral stenting was performed in order to help identification during tumor dissection and prevent ureteral injury. Nevertheless, the possibility of autotransplantation of the solitary kidney was considered. A subcostal Chevron incision was made with extension to the left. The first surgical finding was extensive left colon necrosis, which was dissected from the anterior face of the mass, and resected completely at the end of the procedure, a right-sided Hartmann colostomy being performed.The mass was exposed by mobilizing the splenic flexure and left colon medially. It was located medial to the left kidney and ureter and pushed the renal artery, as well as the aorta medially, being partially adherent to all those structures. Careful and tedious dissection liberated the tumor completely from the neighboring structures, requiring intermittent aortic and iliac clamping, with estimated blood loss of 1200 ml that was treated with three blood cell packs. Despite the bleeding, the patient remained stable during the whole procedure, without any hypertensive crisis. Pathological evaluation revealed that the specimen measured 13.0 × 10.0 × 4.0 cm and weighed 471 g. Sectioning revealed a multilobular, heterogeneous tumor mass with foci of hemorrhage without necrosis. Microscopically, the tumor consisted of nests of ovoid cells with nuclear enlargement. Chromogranin A and synaptophysin stains were both positive in the tumor cells, confirming the diagnosis of extra-adrenal retroperitoneal paraganglioma. Ki-67 stain was also positive. Mitotic index was extremely low and there were no signs of extracapsular invasion. Images of surgical specimens and surgical site after mass removal are demonstrated in Fig. The patient had a good postoperative recovery with functioning colostomy and no renal function impairment and was discharged on the seventh postoperative day after removal of the ureteral stent. After 6 weeks, the patient had a normally functioning colostomy, but became acutely oliguric. A mid-ureteral stenosis was identified, requiring an emergency ureteral stenting. Follow-up evaluation with MIBG scan and bone scintigraphy also depicted multiple areas of intense activity suggestive of metastatic disease in the left forearm, right costal arches, and left iliac bone who were absent in preoperative staging. Figure The diagnosis of PGL can be difficult in cases presenting as a non-secreting abdominal mass. It can be found during the monitoring of a patient with a predisposition syndrome or incidentally before clinical manifestations related to hypersecretion of catecholamines . HyperteWell-established hereditary disorders associated with PGL are MEN 2A and 2B, von Hippel-Lindau syndrome and neurofibromatosis type 1 , 10, 11.In the pediatric population, elevated plasma-free and fractionated metanephrines have a high sensitivity close to 100 % and a specificity of 95 %. A concentration of plasma-free metanephrine greater than four times the standard was uniformly associated with the presence of a tumor . VanillyAbdominal CT scan, magnetic resonance imaging (MRI), metaiodobenzylguanidine (MIBG) scintiscan, and octreotide scan (Octreoscan) have all been described as means of identifying PGL . As longSome features such as central necrosis, high mitotic index, large size (greater than 5 cm), capsular rupture, or vascular invasion suggest malignancy. The only formal criterion for malignancy is the presence of metastasis in a site that does not contain chromaffin tissues . An important feature in pediatrics is the possibility of developing such metastases years after the discovery of the primary tumor, which warrants long-term surveillance, in particular, through an annual determination of urinary catecholamines . In the Possible biochemical indicators of malignant disease include dopamine hypersecretion or markedly elevated plasma or urinary metanephrines. The most well-defined genetic risk factor for malignant disease is a mutation on the SDHB gene, which is clinically associated with an earlier onset of disease and more aggressive malignancy. Often, metastatic disease is related to retroperitoneal location (69 %) and is associated with SDHB mutation (72 versus 10 %). SDHD mutations are more frequently found in the cephalic or cervical tumors. These results should be considered in clinical practice especially during the etiologic diagnosis and monitoring. Given the possibility of very late metastases, genetic investigation is of major importance because it allows to identify patients with a high risk of developing recurrence and metastasis and thus requiring prolonged surveillance. Children with a cervical spine tumor should primarily benefit from analysis of the SDHD gene and those with metastatic retroperitoneal form from a study of SDHB .The management of malignant PGL is primarily surgical as resection is the only potentially curative treatment, but depends on tumor staging . The overall prognosis for tumors with complete surgical resection is excellent . PreoperInitial complete resection with intent to cure has been shown to improve survival, while surgical debulking is often used in an attempt to achieve biochemical control, improve response to systemic therapies, palliate symptoms, or simply to decrease tumor burden. Debulking procedures in patients with extra-abdominal metastases should be employed only for palliation and not for biochemical remission. Conversely, a more aggressive operative approach should be offered to patients with exclusively intra-abdominal disease with a goal of postoperative biochemical remission when a complete resection (RO/R1) is possible .The surgical technique depends on the tumor location. Laparoscopy can also be offered in cases of abdominal localization . HoweverPreoperative embolization is not routinely utilized because of the theoretical concern that blocking the arterial supply will induce ischemia and cell death, releasing catecholamines into an open venous outflow leading to hypertensive crisis. However, preoperative embolization has been described in the surgical management of carotid body tumors where it was shown to decrease intra-operative blood loss and time of surgery without complications . It can Surgical ablation of metastasis should be considered whenever possible. The use of percutaneous techniques  is a possible means to obtain local control in more than 50 % of cases, or at least temporarily reduce symptoms. The choice of the appropriate technique depends on the child’s age, clinical presentation, location and number of metastasis , 18.131 seems to be the most effective treatment in adults. This is a complex treatment that requires a number of prerequisites such as MIBG injury setting and hematopoietic stem cell availability. The procedure is particularly of limited value in pediatrics because of the high radiation dosage [Currently, the use of MIBG therapy with In dosage . Externan dosage , 21.In the presence of metastatic PGL, overall life expectancy at 5 and 10 years is estimated at 78 and 31 %, respectively, but is very poorly evaluated in the literature . The proMany studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposition. Better understanding of genetics allows a better assessment of risk metastatic and therefore prognosis, especially in familial forms. Surgical resection is the mainstay of treatment and should be performed despite the difficulties involved in the procedure. The role of preoperative embolization is not established, and in our experience, it has provided little benefit and major complications.The complexity of this pediatric pathology requires a multidisciplinary management involving pediatricians, endocrinologists, pediatric surgeons, urologists, anesthesiologists, geneticists, radiologists, nuclear medicine physicians, and oncologists.Written informed consent was obtained from the patients for publication of this case report and any accompanying images.


Pulmonary carcinosarcoma represents a category of extremely rare tumours accounting for 0.1% of all lung malignancies. It is defined as a poorly differentiated non-small-cell carcinoma that contains a component of sarcoma or sarcoma-like elements. These biphasic tumours typically have a poor prognosis due to late diagnosis and early metastases. Preoperative tissue diagnosis is usually difficult due to the heterogeneity of the tumour, with biopsies often just reflecting one element of the tumour. By means of a case illustration and review of the literature, we discuss the optimal management of patients with pulmonary carcinosarcoma. Pulmonary carcinosarcoma can be defined as a poorly differentiated non-small-cell carcinoma that contains a component of sarcoma or sarcoma-like elements. They are extremely rare tumours, accounting for only 0.1% of all lung malignancies [We present the case of an 83-year-old lady who had been followed up over a three-year period with a small peripheral right upper lobe lesion. She underwent repeated surveillance imaging with PET CT. The mass was of low avidity and the density of the mass appeared to wax and wane over the years. The patient also had repeated bronchoscopies with endobronchial washings and biopsies, which were negative for malignancy. At her most recent interval scan however the mass had grown rapidly in size to over 3 cm.Following discussion at the lung cancer MDT (Multidisciplinary Team) meeting, she was clinically staged as a T2b N0 M0 lung cancer. Her lung function tests were excellent with an FEV1 of 109% predicted and a TLCO of 83%. The patient was referred to our service and underwent successful surgical management with a right thoracotomy and upper lobectomy. She was treated for fast response atrial fibrillation in the postoperative period but otherwise the patient made an excellent recovery and was discharged on the eighth day following surgery.This case illustrates the diagnostic uncertainty of managing patients with what at first appeared to be a benign solitary pulmonary nodule.Subsequent histological examination of the specimen was performed. Sections from lung demonstrated the presence of a rather ill-defined 50 mm mass which microscopically had a heterogeneous appearance . In areaPulmonary carcinosarcoma was first described by Kika et al. in 1908 as a poorly differentiated non-small-cell carcinoma containing a component with sarcoma or sarcoma-like features .These tumours tend to present in middle age with the average age of diagnosis around 60 years although patients as young as 31 have been reported [Preoperative diagnosis of carcinosarcoma is difficult with only a few case reports on the imaging diagnosis of this rare tumour. The tumours are however usually bulky (>5 cm) and have a high tendency to invade adjacent structures such as the pleura, chest wall, diaphragm, or mediastinum. Large low attenuation areas are found on CT that correspond to regions of necrosis and myxoid degeneration. Calcification may be observed within the primary tumour as well as metastases on CT scan, which would likely represent an osteosarcomatous or chondrosarcomatous component to the tumour .Similarly, there is a paucity of data on FDG uptake on PET-CT imaging for these tumours with one report of significantly higher uptake than in other types of lung cancer and another report of an SUV max of 4.1 [Surgical resection therefore seems to be the most important part in order to obtain sufficient tissue to make an accurate histological assessment. Clinically, two subtypes have been described: a central endobronchial type and a peripheral invasive type also called parenchymal carcinosarcoma. The peripheral parenchymal type often presents as a large mass. These tumours are often asymptomatic in the early stage, during which time they may involve the adjacent organs or structures such as the mediastinum, pleura, and thoracic wall . The endIn addition to surgical resection, radiation is well established and effective as the standard therapy in combination with surgery. Even if the sarcoma is resected incompletely and the resection margins show residual tumour on microscopy the rate of local recurrence can be reduced to about 15% by local radiation therapy . Chemoth


Esophageal leiomyoma is a relatively rare tumor of esophagus but it is the most common benign neoplasm of the esophagus. Small esophageal leiomyoma can be observed but larger ones and those producing symptoms should be excised. As observed for other esophageal tumors, dysphagia is its main symptom. Traditionally, open thoracotomy and enucleation are its main treatment but in the last few years video assisted thoracoscopic surgical (VATS) enucleation is gaining recognition with proven advantages of minimally invasive surgery. Herein we present our experience with patient presenting with cough rather than dysphagia as a main symptom, who was diagnosed to be having giant esophageal leiomyoma. VATS guided enucleation was accomplished successfully. Size of lesion was 16 × 4 × 3 cm. Postoperative recovery was uneventful and patient is not having any signs of recurrence, after three years during follow-up period. Benign esophageal tumors are relatively rare; they constitute 1% to 10% of all esophageal neoplasms  which usA 25-year-old female first presented to general physician with nonproductive cough, dull aching chest pain for 10 months with no history of weight loss, and dysphagia. Chest X-ray  showed oPatient was given prone position with a pillow beneath the chest to avoid abdominal compression and to allow free diaphragmatic excursion; double lumen tube was used to collapse the right lung. Four trocars were introduced. The camera port (10 mm) was placed through a seventh intercostal space 2 cm lateral to posterior axillary line. Three 5 mm trocars were used first at the fifth intercostal space at posterior axillary line and second at ninth intercostal space one inch lateral to camera port, third port was placed approx 5 cm lateral to camera port through the same intercostal space posteriorly . After eBarium swallow at day 3 after surgery revealed no leakage and the patient was started on a liquid diet on day 4 . The patHistopathological report mentioned presence of interlacing fibres of smooth muscle cells arranged in somewhat whorled appearance with areas of hyaline degeneration without any evidence of malignancy suggestive of leiomyoma of esophagus. Immunoperoxidase stainings were positive for smooth muscle actin and desmin, and negative for C-kit.Esophageal leiomyoma is the commonest benign tumor arising from smooth muscle cells of the esophagus. It can involve any part of esophagus but reportedly it affects distal third in 60%, middle third in 30%, and upper third of esophagus in 10% of the cases . LeiomyoOften esophageal leiomyoma clinically manifests with no specific symptoms and diagnosis often is an incidental finding . Though Preoperative diagnosis of esophageal leiomyoma is often a challenge. As in our case, it can present as mediastinal mass or it may be an incidental radiologic finding. Esophagoscopy will show normal mucosa and submucosal lesion. Barium swallow is the most common imaging study advised for esophageal lesions; it will show smooth filling defect in esophageal lumen without mucosal abnormality . ComputeConsensus regarding threshold for surgical resection of these tumors has not been reached so far. As malignant change in leiomyoma is rare, some authors recommend regular follow-up for small asymptomatic tumors (<5 cm) provided malignancy is excluded , 12, whiThe conventional surgical approach especially for giant esophageal leiomyoma has been open thoracotomy or tumor resection through thoracoabdominal incision and sometimes along with gastroesophagostomy , 13–17. Chen et al. , Kent etVATS guided enucleation is a safe technique for giant esophageal leiomyoma; it reduces morbidity over open thoracotomy or segmental resection of esophagus.Occasionally, giant esophageal leiomyoma of esophagus may mimic as respiratory tract pathology, with cough as a main symptom, so one needs to consider this possibility even in treating such patients empirically.


Most prolactin-secreting pituitary adenomas demonstrate slow growth and are effectively managed with medical/surgical therapy. Rarely, these tumors can behave aggressively with rapid growth and invasion of local tissues, and are refractory to medical, surgical, or radio-surgical therapies. We report a case of a prolactin-secreting adenoma in a young woman, which became progressively aggressive and refractory to usual treatment modalities, but responded to treatment with the chemotherapeutic agent temozolomide. In addition, we review the literature for treatment of refractory adenomas with temozolomide. The clinical and pathologic characteristics of aggressive prolactin-secreting adenomas are reviewed, as well as their response to dopamine agonists, surgery, radiotherapy, and chemotherapy. Pituitary adenomas are one of the more common intracranial neoplasms . They teA 37-year-old gravida-1, para-0 female presented in 2002 with secondary amenorrhea and galactorrhea. She described the onset of menses at age 13 and had normal menstrual periods through her teens. She was on birth control pills until 1999 with normal menstrual periods. After stopping birth control pills, her periods did not resume, and she noted intermittent galactorrhea. Evaluation revealed an elevated prolactin level of 110 ng/ml , and MRI demonstrated a 10 mm pituitary lesion. She also had central hypothyroidism and was started on levothyroxine. She was placed on bromocriptine and had an excellent initial response with normalization of her prolactin and resumption of menstrual periods. Follow-up MRI showed regression of the tumor to 7 mm shown as in Figure After six months, however, amenorrhea recurred with an elevation of prolactin to 55 ng/ml despite maximal tolerable bromocriptine therapy (15 mg daily), and in Figure A trans-sphenoidal adenomectomy was performed in June 2005, with subsequent normalization of prolactin levels 11.3 ng/ml). Pathology was consistent with a pituitary adenoma showing sparse immunoreactive prolactin, with increased nuclear pleomorphism, elevated mitotic activity, and increased p53 labeling suggesting increased biologic aggressiveness and invasion as shown in Figure  ng/ml. PDue to these findings on pathology, further treatment with stereotactic radiosurgery was recommended, but the patient declined due to concerns regarding infertility. Over the ensuing year, the tumor continued to increase in size to 17 mm diameter with further invasion of the cavernous sinuses. She subsequently developed headaches as well as right third and sixth nerve palsies, which resolved following a second trans-sphenoidal debulking surgery in June 2006. In November 2006, she underwent single-dose Cyberknife radiosurgery . However, she became amenorrheic despite normalization of her prolactin level and lack of radiographic progression for four years. Repeat MRI in 2010 showed further progression of the tumor into the right cavernous sinus, for which she was treated with Gamma Knife radiosurgery in January 2011 (20 Gy single fraction). The clinical status and MRI studies remained stable for the next three years, but in 2014, she developed progressive right third and sixth nerve palsies and MRI showed further tumor growth up to 3.4 x 3.1 x 3.8 cm. Because she was not a candidate for further radiosurgery, she underwent another trans-sphenoidal debulking surgery, and at that time, the decision was made to commence TMZ chemotherapy in October 2014. Pathology was consistent with an atypical prolactinoma with increased nuclear pleomorphism, marked elevation of the Ki-67 labeling index to 25%, and p53 labeling index to 70%. These histologic findings are shown in Figures Next Generation sequencing of her tumor identified mutations in TP53, IDH2, CDH1, and KIT. O(6)-methylguanine methyltransferase (MGMT) proliferation markers were not done on this particular case. While awaiting insurance authorization for TMZ therapy, she developed visual field deficits in her left eye as well as radiographic progression of her tumor as shown in Figure She then underwent a right, frontotemporal craniotomy in January 2015 for decompression of the optic nerves and chiasm, with postoperative restoration of full visual fields. The postoperative MRI is shown in Figure 2 for the first cycle and increased to 200 mg/m2 for subsequent cycles. MRI of the brain two months following completion of radiation, in Figure The histology was similar to the 2014 pathology. Given the rapid recurrence and intracranial invasion, she was treated with fractionated Cyberknife radiosurgery over 5 fractions (25 Gy), followed by a course of chemotherapy with 12 cycles of TMZ given on a five-day schedule every 28 days at 150 mg/mFollowing eight cycles of TMZ to date, surveillance MRI scans--demonstrated in Figures Prolactinomas, which represent 40% of all pituitary tumors, are the most common variant, with an incidence of 27 cases per million persons per year and a higher incidence in females of child-bearing age -2.  LactRefractory prolactinoma is defined as a tumor in which dopamine agonist therapy is ineffective and where surgical resection is unsuccessful [Aggressive prolactinomas likely represent a further subset of refractory prolactinomas. Fortunately, aggressive pituitary adenomas are rare, comprising between 2.7%-15% of all pituitary adenomas [ccessful . In addiccessful . Aggressadenomas . There iadenomas . Though adenomas . The abnadenomas . Althougadenomas . MorpholIn a retrospective study of 410 patients where characteristics that were predictive of aggressive behavior were defined, Trouillas, et al., proposed a classification system based on combining histochemical proliferative markers  and radiographic evidence of cavernous or sphenoid sinus invasion [Although currently there is not a single, reliable immunohistochemical marker, imaging characteristic or universally accepted definition for aggressive adenomas; it is believed that benign adenomas may undergo genetic transformation, which may lead to invasive behavior and may eventually transform into malignant pituitary carcinomas , 7-8. InThe challenge of treating an aggressive adenoma is inherent in its diagnosis – these tumors are resistant to medical, surgical, and radiotherapy treatments. When approaching benign micro and macroprolactinomas, initial treatment is a dopamine agonist such as cabergoline or bromocriptine, both of which have been shown to reduce tumor size, normalize serum prolactin levels, and restore gonadal function in more than 70-95% of patients , 5, 8. IWhen clinical and radiographic evidence leads to the diagnosis of an aggressive adenoma, repeated surgical debulking can be helpful, though these treatments are palliative and often will lead to surgical complications including visual field deficits, infection, cerebrospinal fluid leaks, and panhypopituitarism . MedicalRadiotherapy is often used in conjunction with surgery to treat aggressive or recurrent pituitary adenomas. Radiotherapy aims to slow or stop tumor growth, restore normal prolactin levels, and minimize radiation dose toxicity . Radioth6-methylguanine-DNA methyltransferase (MGMT) by tumor cells can predict clinical response to TMZ therapy, with multiple studies demonstrating a strong inverse relationship between low MGMT expression and high responsiveness to TMZ and conversely, high MGMT expression with resistance to TMZ [2 for five days in 28-day cycles [TMZ, an oral alkylating chemotherapeutic drug used primarily for the treatment of brain tumors such as glioblastoma multiforme, has been used for the past 10 years for pituitary carcinomas and aggressive pituitary adenomas . TMZ prie to TMZ , 12-13. y cycles -12. Sevey cycles . AggressIn our case, the patient had an aggressive prolactinoma resistant to dopamine agonist pharmacotherapy, with recurrence after multiple surgical resections and radiotherapy. TMZ was started as a salvage therapy for recurrent mass effect and neurological deficits in conjunction with radiosurgery. After eight cycles of TMZ, the patient responded with excellent clinical results on her MRIs demonstrating continued radiographic regression of her tumor coincident with clinical improvement. The excellent response rate of progressive pituitary adenomas treated with TMZ therapy, combined with the relatively low morbidity of this drug, raises the question as to whether TMZ should be considered as an initial component of treatment at the time tumor recurrence is detected. TMZ for the treatment of aggressive pituitary adenomas, especially prolactinomas, has shown promise in controlling these invasive and highly aggressive tumors. Based upon reported anecdotal experience, TMZ may be more effective than radiotherapy as a second-line treatment after surgery. Although this patient’s tumor is an example of atypical adenoma histology and immunohistochemistry, more research is needed to better define and discover accurate biomarkers for aggressive pituitary adenomas for earlier diagnosis and prediction of treatment response. Future prospective clinical trials should be considered to evaluate the role of TMZ in the management of patients with aggressive pituitary adenomas. The relationship between MGMT and TMZ sensitivity also requires further understanding and may lead to better patient selection and tailored treatments for these invasive tumors.


A case of appendiceal involvement by acute myeloid leukemia (AML) in an adult with recent history of AML transformed from myelodysplastic syndrome (MDS) was presented. Being aware of this rare presentation in particular in a patient with history of MDS and/or AML is important for prompt clinical diagnosis and management. An acute appendicitis was suspected and a laparoscopic appendectomy was performed. Visual inspection of the peritoneal cavity during the procedure was essentially unremarkable. The appendix appeared to be inflamed and grossly showed no adhesions. Histologic sections (A 57-year-old man with a seven-month history of refractory anemia with excess blasts (RAEB-2), recently transformed to acute myeloid leukemia (AML) presented with a 3-day history of lower abdominal pain, nausea, and constipation. There was no fever or vomiting. Physical examination revealed tenderness in his abdominal bilateral lower quadrants. CAT scan revealed a thickened appendix and periappendiceal stranding. The liver, spleen, pancreas, adrenal glands, and kidneys were normal. The gall bladder was previously removed and was not visible. His peripheral blood count showed white blood cell count of 2.3 × 10sections  revealedAcute appendicitis due to involvement by acute leukemia is exceedingly rare


Synchronous development of primary hepatocellular carcinoma and intrahepatic cholangiocarcinoma has been reported rarely. In literature review, there have been only 35 reported cases of synchronous hepatocellular carcinoma and intrahepatic cholangiocarcinoma, and most of these tumors developed in patients with hepatitis C-related liver cirrhosis. Here, we present synchronous development of hepatocellular carcinoma and intrahepatic cholangiocarcinoma in two patients with chronic B-viral hepatitis.Two patients with chronic hepatitis B were referred to our hospital due to a hepatic mass. Patient 1 had a 6.4 cm multinodular hepatic mass in the left lobe and a small nodule in the right lobe. Patient 2 had a 4.3 cm hypervascular mass in the right lobe and a 1.1 cm nodule in the left lobe. The pre-operative diagnosis of both cases was hepatocellular carcinoma with metastatic nodule, however, surgical resection pathology revealed that hepatocellular carcinoma and intrahepatic cholangiocarcinoma existed independently in the other side of the liver in both cases. Additionally, the background liver histology of both cases was hepatitis B-related chronic hepatitis without cirrhotic change.Our cases suggest that hepatitis B virus infection can also predispose to development of double liver cancers. Although hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC) are the two major forms of primary liver cancer, coincidence of both in a single patient is very rare ,2. MoreoA 66-year-old woman with chronic hepatitis B was referred to our clinic due to a hepatic mass. The patient had no other apparent conditions. Laboratory tests, including platelet counts and liver function, were normal. Hepatitis B surface antigen (HBsAg) was positive and hepatitis B e antigen (HBeAg) was negative. The serum hepatitis B virus deoxyribonucleic acid (HBV-DNA) level was 2,490 IU/ml. Anti-HCV antibody was negative. Of the tumor markers tested, alpha-fetoprotein (AFP) was 3.54 IU/ml, protein induced vitamin K antagonist (PIVKA-II) was 22 mAU/ml, carcinoembryonic antigen (CEA) was 4.28 ng/ml, and carbohydrate antigen 19–9 (CA19-9) was 0.1 U/ml. She was a social-drinker, and had no metabolic impairment.Computed tomography (CT) and dynamic magnetic resonance imaging (MRI) revealed a 6.4 cm multinodular hepatic mass with heterogeneous arterial rim enhancement in the left lobe (S2 and 3), and a small nodule in the right lobe (S7) Figure A-C. The et al., the tumor in the left lobe was pure mucin-producing adenocarcinoma [Unexpectedly, the pathological examination revealed that the main mass in the left lobe was moderately differentiated ICC Figure E. Accordarcinoma . AdditioFor one month following surgical resection, the patient was treated with adjuvant chemotherapy using gemcitabine hydrochloride, after which she underwent six cycles of chemotherapy. At seven months post resection, there was no evidence of recurrence.A 68-year-old woman with chronic B viral hepatitis was referred to our hospital due to a hepatic mass. She was asymptomatic and her laboratory tests were normal. HBsAg was positive and HBeAg was negative. The serum HBV-DNA level was 217,000 IU/ml. Anti-HCV antibody was negative. Of the tumor markers tested, AFP was 46.54 IU/ml and PIVKA-II was more than 2,000 mAU/ml. The CEA and CA19-9 level was 1.03 ng/ml and 12.8 U/ml, respectively. She was a social-drinker, and had no metabolic impairment. CT and MRI revealed a 4.3 cm hypervascular mass with internal hemorrhage in segment 5 (S5), and the radiological appearance of the tumor suggested an HCC. Additionally, a 1.1-cm solid lesion with capsular retraction was identified in the left lobe (S3) and suspected as a small HCC or a bile duct adenoma  can differentiate into either hepatocytes or cholangiocytes ,12. Accoogenesis .Along with advanced comprehension of carcinogenesis, there have been additional efforts to find clinicopathological factors related with these tumors ,3. PreviIn contrast to HCV infection, HBV infection has not been associated with the development of synchronous liver tumors, despite being the most common cause of chronic liver disease . Of 35 ret al. stated that most cases of multiple liver tumors were diagnosed initially as HCCs, with only about 20% being accurately diagnosed as separate primary liver tumors prior to treatment [Indeed, Inaba reatment . There areatment . HoweverInterestingly, in our cases, the double primary cancer developed from non-cirrhotic liver with HBV-related chronic hepatitis, whereas most of these tumors did from HCV-related cirrhotic livers ,4. TheseAs the incidence of synchronous primary liver tumors is extremely low, treatment of these patients is challenging. Although several other methods have been tried, surgical resection is regarded as the treatment of choice because it can provide an accurate diagnosis as well as a chance of cure. But there is no definite guideline after surgical resection, and the postoperative plan should be individualized.et al., which is based on histopathological features, might help to identify characteristics of the tumors, these limitations would not allow us to understand carcinogenesis of our cases thoroughly [We are aware of the limitations of our study. Most of all, several immunohistochemical markers, which represent human hepatic stem cell or mature cholangiocytes such as epithelial cell adhesion molecule (EpCAM) or secretin receptor, were not available in our cases. Although the novel classification of cholangiocarcinoma proposed by Komuta oroughly ,25. TherIn summary, we report here rare cases of synchronous development of HCC and ICC in patients infected B-viral chronic hepatitis. Accurate diagnosis and individualized treatment is important. Further research is needed because knowledge of the biology of these tumors might shed light on the behavior of HCC and ICC and elucidate a more appropriate treatment plan.Written informed consent was obtained from both patients for publication of this case report and accompanying images. A copy of the written consents are available for review by the Editor-in-Chief of this journal.HCC: Hepatocellular carcinoma; ICC: Intrahepatic cholangiocarcinoma; HCV: Hepatitis C virus; HBV: Hepatitis B virus; HBsAg: Hepatitis B surface antigen; HBeAg: Hepatitis B e antigen; HBV-DNA: Hepatitis B virus deoxyribonucleic acid; AFP: Alpha-fetoprotein; PIVKA-II: Protein induced vitamin K antagonist-II; CEA: Carcinoembryonic antigen; CA19-9: Carbohydrate antigen 19–9; CT: Computed tomography; MRI: Magnetic resonance imaging; HPC: Hepatic progenitor cell; EpCAM: Epithelial cell adhesion molecule.The authors declare that they have no competing interests.KSJ, KHC and HSS collected the clinical data of patients and wrote the manuscript. GHC contributed to the editing and design the article. HMJ and YNP contributed to the analysis and interpretation of pathological data. JYP planned to report the cases and supervised the whole work. All authors gave final approval for publication.


Pyometra can be caused by various etiologies. We present a rare case of diverticular disease of the colon presenting as pyometra. This type of presentation can be challenging even for an astute clinician.A 74-year-old Caucasian woman with a history of pyometra was referred to our gynecology clinic as an urgent case. She was obese, diabetic and hypertensive. Due to the patient profile and the clinical presentation, clinicians were misled toward a diagnosis of possible endometrial cancer. After further investigations, she was found to have colouterine fistula secondary to a diverticular abscess of the sigmoid colon.Persistent vaginal discharge due to pyometra can be caused by diverticular disease of the colon. Clinicians should be aware of this important differential diagnosis. Diverticular disease of the bowel commonly affects the sigmoid colon. Symptoms can range from mild to acute bouts of diverticulitis complicated by abscess or frank perforation. Colouterine fistula is an uncommon complication of diverticular abscess, which can present as pyometra and clinically mimic endometrial cancer.A 74-year-old Caucasian woman was referred to our gynecology clinic for suspected pyometra. She was obese, diabetic and hypertensive. Outpatient hysteroscopy revealed a thickened white endometrium and pyometra. A pipelle endometrial sample was taken and sent for histology.Three days later, she presented to the accident and emergency department feeling unwell, with profuse foul-smelling vaginal discharge and bleeding per rectum. She was admitted and started on intravenous antibiotics for suspected uterine sepsis.The pipelle biopsy showed pus cells only. Her carcinoembryonic antigen (CEA) level was normal and CA-125 slightly raised at 73. Vaginal swabs showed no pathogens. As the clinical picture was highly suggestive of endometrial cancer, a magnetic resonance imaging (MRI) scan was performed, which revealed pyometra but no obvious tumor.A repeat hysteroscopy was done under general anaesthetic and drainage of pyometra was performed. About 50ml of pus was drained and an endometrial biopsy was taken. Pus culture showed mixed anerobic growth and the repeat endometrial biopsy revealed acute on chronic endometritis. She improved clinically with intravenous antibiotics and was discharged home.She presented 10 days later to the accident and emergency department with a history of collapse, acute abdominal pain, diarrhoea, vomiting and bleeding per rectum. A contrast computed tomography (CT) scan of the abdomen requested by the general surgeons was unremarkable. A sigmoidoscopy and colonoscopy revealed significant diverticular disease with prominent thickened folds. She improved with conservative management.She presented to the surgical team three months later with acute abdominal pain. A CT scan was repeated, which revealed an inflamed thick-walled sigmoid colon that tracked down to the uterus (a colouterine fistula). Our patient underwent laparotomy where a large diverticular mass was seen perforating into the uterus (a colouterine fistula) and adherent to its posterior wall. Pus was draining through the vagina. A Hartmann’s operation with total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed.She made a slow but complete recovery. At follow-up six months later she was asymptomatic. Our patient is now deceased but her death occurred due to a cause unrelated to the condition mentioned.Pyometra can be caused by a number of gynecological conditions, both benign and malignant, leading to cervical stenosis -3. Causeet al. suggested that the most common cause of pyometra is genital tract malignancy and treatment by radiotherapy [Yildizhan otherapy . Howeverotherapy . Spontanotherapy ,4. Our pThe important differential diagnoses in these patients include gynecological malignancies  and diverticular disease -5. OtherOur patient had multiple admissions and investigations to find out the cause of her pyometra. A hysteroscopy and a pipelle biopsy were done on initial presentation. In view of her presentation with abdominal pain with rectal bleeding, she was evaluated by general surgeons and a contrast CT scan and an MRI scan were performed. Only when a repeat contrast CT was arranged three months later, was the correct diagnosis of colouterine fistula made.et al. reported a case of a colouterine fistula at the level of the isthmus diagnosed at hysteroscopy [Diverticular disease of the bowel commonly affects the sigmoid colon . Symptomeroscopy . The fisDiagnosis depends on a high index of suspicion. It should be suspected in any patient with a persistent vaginal discharge and diverticulitis of the sigmoid colon. Presence of air and fluid within the uterus on ultrasound or CT scan in patients with diverticulitis or diverticular abscess is highly suggestive of a fistulous communication -3. ImagiGynecological malignancy is a common cause of pyometra and can mislead clinicians. Persistence of pyometra with a negative endometrial biopsy should prompt investigations for presence of a fistulous tract especially in the presence of known diverticular disease of the colon. Colouterine fistula, though a rare complication of diverticular disease of the colon, can masquerade as pyometra.All reasonable and exhaustive attempts to contact our deceased patient's next of kin failed. This case report contains no patient-identifiable features, and no images were included.The authors declare that they have no competing interests.SP, AR and UB were involved in the management of our patient. SP collected the data/details and prepared the manuscript, revised it and submitted it. AR collected the data/details and prepared the manuscript. UB prepared and revised the manuscript. All authors read and approved the final manuscript.


Adenoid cystic carcinomas, formerly known cylindromas, were originally described by Foote and Frozell 1953. These are epithelial malignancies that develop at the expense of salivary glands, other rare localizations have been described in particular in the oral cavity, the sinonasal tract, lacrimal glands or nasopharynx. Clinical and radiological signs are not specific, and are common to all sinonasal tumors, including squamous cell carcinomas and adenocarcinomas. The adequate treatment is surgery, postoperative radiation therapy improves long-term prognosis. These tumors are characterized by aggressiveness and a high incidence of local recurrence and distance metastasis regardless of therapeutic modalities used. We report a case of a 70 year old patient, hospitalized for exploration of a large sinonasal tumors, CT Scann of the face and sinonasal cavities disclosed a large right ethmoïdomaxillonasal tumor, with tissue density with and contrast enhancement, lysing all the walls of the maxillary sinus, septum and the horizontal branch of the mandible. With extension to the infratemporal fossa, the orbital floor, the contralateral nasal cavity, the floor of the nasal cavity and nasopharynx. A nasal biopsy concluded to an adenoid cystic carcinoma, The staging revealed no distant metastases, the tumor was classified T4NoMo. The patient refused any surgery, he was referred for palliative radiotherapy, it was lost sight of.


We report a rare case of delayed abdominal wall abscess after abdominoperineal resection (APR) for rectal cancer.A 63-year-old woman was diagnosed with rectal cancer and received chemo-radiotherapy, followed by APR. One year after surgery, the patient complained of pain and skin redness in the lower abdomen. A low-density mass lesion with 5.9-cm diameter was found in the lower abdominal wall by computed tomography, which showed high uptake on positron-emission tomography. These findings suggested the possibilities of either delayed abscess formation or abdominal wall recurrence of rectal cancer with central necrosis. Percutaneous drainage was performed. The content was a purulent exudate, without neoplastic cells in the cytology. The lesion quickly disappeared after the drainage, and no recurrence of the tumor was observed for more than 2 years.In this case, the un-absorbable yarn, such as silk, has not been used during the operation, no foreign body was retained in the abdominal wall, and there was no associated inflammatory bowel disease. Use of neoadjuvant chemoradiotherapy was the only possible cause of delayed abscess formation in this case.In case local recurrence is suspected by imaging modalities in the postoperative of colorectal cancer, especially those with precedent chemoradiotherapy or radiotherapy, although rare, the possibility of a delayed abscess formation should also be considered. Surgical site infection (SSI) is a common complication of gastrointestinal surgery, especially colorectal surgery. Although rectal surgery is accompanied by a higher incidence of SSI compared to colon surgery (17–28%) . Preoperative chemoradiotherapy was performed with a total dose of 50.4 Gy of radiation and concomitant oral administration of tegafur-uracil, followed by APR with curative intent. The pathological finding of the resected specimen was ypT2N0M0 stage I. The postoperative course was uneventful without any SSI. No adjuvant chemotherapy was administered, and no recurrence or abscess formation was seen on the CT imaging 6 months after the APR Figure a.Figure Streptococcus. The cytological examination of the exudate was negative for neoplastic cells. After the drainage of the abscess, followed by antibiotic administration, the tumor quickly regressed, without recurrence of cancer or the abscess even 2 years after the treatment.One year after the APR, the patient complained of pain and redness of the skin in the lower abdomen. The CT imaging showed a 5.9-cm diameter low-density mass lesion in the lower abdominal wall Figure b. The maDelayed abscess formation in the abdominal wall is a rare postoperative complication, and as late as the abscess develops, the more difficult the differential diagnosis. Surgical treatment of infectious diseases, such as cholecystitis or appendicitis, is known to be associated with a higher risk of late abscess formation, due to retained stones in the abdominal wall at the time of surgery , were strongly suggestive of locally recurrent cancer. In this case, the un-absorbable yarn, such as silk, has not been used during the operation, no foreign body was retained in the abdominal wall, and there was no associated inflammatory bowel disease. Use of neoadjuvant chemoradiotherapy was the only possible cause of delayed abscess formation in this case.In conclusion, although rare, the possibility of delayed abscess formation should be considered as a differential diagnosis in case of suspected local recurrence by imaging modalities in the late postoperative of colorectal cancer cases with precedent chemoradiotherapy or radiotherapy.An approval by an ethics committee was not applicable.Informed consent was obtained from the patient for being included in the study.


Cavernous hemangioma is an encapsulated mass of dilated, endothelial lined vascular channels filled with slowly flowing blood. Cavernous hemangioma of the spleen is a rare condition with less than 100 reports so far. Hemangioma of the vertebral is a benign vascular legion around one or two vertebrae. These are usually asymptomatic and discovered incidentally. In this study we reported an extreme rare case of splenic hemangioma coexistence with vascular malformation of the vertebrae. To our knowledge this is the first report of coexistence of splenic hemangioma and hemangioma of the vertebra.A 20-year-old iranian male with splenomegaly, abdominal pain, diarrhea and pancytopenia who was first highly suspicious for malignancy referred to our center for evaluation of the diagnostic workup. After full examination we detected a very rare case with a giant, solitary cavernous hemangioma of the spleen and multiple hemangiomas in his vertebrae. Histopathology of the spleen showed a large cavernous hemangioma occupying almost the entire spleen with large areas of infarction necrosis with multiple hemangiomas of the vertebrae.It is extremely rare to have a splenic hemangioma concurrent with vertebra hemangioma and this is clinically very important to consider splenic hemangioma in differential diagnosis of splenomegaly for a better therapeutic management in related patients. Hemangioma is the most common benign primary neoplasm in spleen and the prevalence ranges from 0.3 to 14 % at autopsy . This vaThis is a report of a very rare case of a giant, cavernous hemangioma of the spleen with multiple hemangiomas in the vertebrae. A 20-year-old iranian male referred to the hematology department, Imam Reza hospital, Tehran in Feb 2010 with occasional pain in the left upper abdomen which lasted for 2 months. The abdominal pain radiated to lower back. He gave the history of 13 kg weight loss during last 7 months.There was no history of fever and urinary symptoms. Occasionally night sweating was in his past medical history. He had the same type of abdominal pain 10 years ago. There were not any important positive findings in his familial history. The general physical examination was unremarkable except for the spleen which was grossly enlarged, extending to 4 cm under umbilicus. Blood counts showed a mild pancytopenia. Serology of viral hepatitis and HIV, liver and renal function tests and hemoglobin electrophoresis did not show any abnormality. Bone marrow aspiration was normal. Ultrasonography of spleen showed multiple diffuse cystic lesions, suspicious to cavernous hemangioma or splenic abscess.Contrast-enhanced computed tomography (CECT) of the abdomen showed a large, lobulated mass with multiple hypodense round lesions in different sizes in its counter in anatomic site of spleen, suggestive of splenic hemangioma or metastasis. There was not any free fluid in his abdomen. Without contrast magnetic resonance (MR) imaging of lumbosacral spine showed multiple abnormal bone marrow signal alteration through vertebral body in favor of metastatic lesion referred as a vertebral hemangioma Fig. . Upper GThe resected specimen and imprint cytology was sent for pathology evaluation but it did not show any evidence of malignancy. Postoperative recovery of the patient was uneventful. Histopathology of the spleen showed a large cavernous hemangioma occupying almost the entire spleen with large areas of infarction necrosis Fig. . BecauseIn this study we reported a rare case of a giant cavernous hemangioma with multiple hemangiomas in vertebra. To our knowledge this is the first report of concurrent of cavernous hemangioma of the spleen and hemangioma of the vertebra. The most common benign primary neoplasm in spleen is hemangioma , 2. CaveIn this study we reported a rare case that was suspicious to malignancy. After evaluation we found that the patient has a giant, cavernous hemangioma of the spleen presenting as a massive splenomegaly and multiple hemangiomas in his vertebrae. At the end this is important to consider splenic hemangioma in differential diagnosis of splenomegaly and the concurrent of the splenic hemangioma with vertebral hemangioma indicates the need for urgent therapeutic procedures as the condition is so critical.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Dear SirCongenital transmesenteric hernia in neonates is a rare cause of intestinal obstruction with devastating outcomes. Patients are often managed with urgent surgical exploration and may require extensive bowel resection.[1-3] We present a case of a newborn presenting with intestinal obstruction found to have a strangulated transmesenteric hernia through a congenital defect in the ileal mesentery, which was managed successfully. A 5-hour-old newborn baby boy was admitted to neonatal intensive care unit for tachypnea and decreased activity. He was born at 39 weeks of gestation to a 33-year-old mother, with an unremarkable prenatal history. He was a product of an emergency cesarean section for antepartum bleeding. His Apgar score was 8 and 10 at 1 and 5 minutes respectively, with a weak cry. No active resuscitation was done. His birth weight was 3.2kg and growth performance was >50th percentile. On admission, baby had stable vital signs and mildly distended abdomen. His initial laboratory workup showed elevated white blood cell count and raised C-reactive protein. A plain abdominal X-ray at 8 hours of age showed a soft tissue shadow occupying right and mid abdomen with bowel pushed on left side with no free air in the peritoneal cavity . He was managed with nil per oral, orogastric tube, intravenous fluids and antibiotics. Within 12 hours of admission, he developed further abdominal distension associated with increased orogastric bilious output but remained clinically stable. At about 22 hours of age, the patient developed tachypnea and required elective intubation and mechanical ventilation. Further evaluation revealed distended and tense abdomen, increase in oro-gastric tube aspirate and change of aspirate color to dark greenish. The baby’s clinical findings prompted for early surgical intervention. Upon exploration, peritoneal cavity was full of meconium. A mesenteric defect was noticed at 30-cm from the terminal ileum  through which a 15-cm segment of mid-ileum was herniated, which was ischemic and had multiple perforations. The gangrenous small bowel segment was resected and an end-to-end anastomosis was performed. The mesenteric defect was also repaired and after thorough wash of the peritoneal cavity, abdomen was closed. Patient’s post-operative course was smooth and he was started on feeds on 7th post-operative day. The histopathology of resected bowel confirmed infarcted small bowel. In follow up, patient has been reviewed in clinic at regular intervals and has remained stable with normal growth and development. Congenital transmesenteric hernia (CTMH), a herniation of viscera through an anatomic defect of about 2-5 cm diameter in the mesentery, is rare in newborns. Less than 20 neonates with CTMH have been reported in English literature. Internal hernias are very rare and reported to occur in 0.2-0.9% of the populations in general, of which 5-10% are CTMHs  and at or near to the duodeno-jejunal junction [1].CTMH in neonates often present as bowel obstruction [4-9], which may progress rapidly to strangulation and septic shock. Bowel ischemia has been reported as early as six hours after the onset of symptoms [1]. Murphy et al reviewed 11 pediatric cases with mesenteric hernias presenting with intestinal obstruction/strangulation, which included 9 neonates. Five of these neonates had associated bowel anomalies, like jejunal and ileal atresia, intestinal duplication, Hirschsprung’s disease, and malrotation [4]. In our case, the baby also presented within few hours after delivery with early signs and symptoms of small bowel obstruction but didn’t have any associated congenital malformations of gastrointestinal and other systems as contrary to Murphy et al cases.In neonates, CTMH can be fatal and the outcome depends greatly on early diagnosis and intervention as the herniated bowel can rapidly progress to strangulation leading to sepsis and death. Janin and Murphy et al. have reported high morbidity and mortality in their series [1] [4]. In our case, even though we intervened early , the herniated bowel segment was infarcted with multiple perforations and peritonitis. After surgery, the patient recovered well. This highlights that an early diagnosis and surgical intervention is helpful to avoid the high morbidity and mortality in neonates with CTMH.Source of Support: NilConflict of Interest: None


Necrolytic migratory erythma (NME) is an obligatory paraneoplastic syndrome. Here we describe a woman admitted to the dermatology ward with NME which was later found to be associated with glucagonoma, a slow-growing, rare pancreatic neuroendocrine tumor. Even more rarely, the tumor was located in the pancreas head, while most of such lesions are located in the distal pancreas. The diagnosis of this rare tumor requires an elevated serum glucagon level and imaging confirming a pancreatic tumor. After surgical removal of the tumor, the patient’s cutaneous and systemic features resolved. It is therefore imperative that clinicians recognize NME early in order to make an accurate diagnosis and to provide treatment for this rare tumor. Necrolytic migratory erythema (NME) is a rare skin disorder which was first described by Becker et al. in 1942 and are A 44-year-old woman presented with a 3-month history of pruritic rash of the extremities and oral cavity. The rash progressed to involve the groin, abdomen, axillae, and gluteal region. She was found to have well demarcated, erythematous plaques in these regions. Tiny fragile vesicles were observed at the margins of several plaques Figure . At thisBased on these findings, NME was considered. A glucagon level was obtained and a contrast-enhanced CT scan was conducted. Her level of serum glucagon was elevated at 720 ng/L   and anemia . Serum levels of chromogranin A, a tumor marker for neuroendocrine tumors, were elevated , as were levels of glycosylated hemoglobin  and the patient was diagnosed with diabetes mellitus.On hospital day 7, she underwent pancreaticoduodenectomy. Pathology confirmed a pancreatic neuroendocrine carcinoma with evidence of vascular and perineural invasion, consistent with a diagnosis of a glucagonoma. In the days following her operation, the patient was admitted to the ICU, where she faced pancreatic fistula that resolved with non-operative care. Her glucagon and glucose blood levels dropped dramatically post-operatively Table . One weeGlucagonoma is a slow growing PNET, which is often heralded by paraneoplastic phenomena. The key features of the glucagonoma syndrome, a rare disorder with an estimated incidence of 1 in 20 million, are NMENME is the most specific feature of the syndrome, and is the presenting symptom in approximately 70% of patients. The exaDiabetes mellitus is another common finding. Glucagon has many important effects on glucose metabolism. It stimulates hepatic gluconeogenesis, inhibits glycolysis and glycogen synthesis. Case studies suggest that 80% of patients with glucagonomas will eventually develop the disease.Glucagonomas are typically distal pancreatic tumors, generally large in size due to late detection, often measuring between 4 and 10 cm. HoweverContrast-enhanced CT is recommended to identify the number and location of tumors. Somatostatin receptor scintigraphy may be obtained if there is concern for distant disease. The besWritten informed consent was obtained from the patient for the publication of this report and any accompanying images.CT: computed tomography; NME: necrolytic migratory erythma; PNET: pancreatic neuroendocrine tumor.The authors declare that they have no competing interests.SLW and ZW conceived and designed the study. JGB and JX collected the clinical data. SLW and QYM wrote the manuscript. All authors approved the final manuscript.


Central neurocytoma is a relatively rare tumor of the central nervous system. Young adults are most commonly affected, with a similar incidence in males and females. The tumor is predominantly occurs in the ventricular system of the brain. The tumor is benign and exhibits a good response to surgical resection and radiation therapy. The typical central neurocytoma occurs in the supratentorial ventricular system in young adults. Extraventricular neurocytomas are rare in the spinal cord. In the present study, two cases of craniocervical neurocytomas and the clinical presentation, magnetic resonance imaging observations, pathological features and two-year follow-up results are reported. The first case presents a 26 year old male with an intramedullary mass extending from the medualla oblongata to the T4 segement of the spine. The second case presents the case of a 48 year old female with an intramedullary mass extending from the oblongata to the T2 segement of the spine. The two patients underwent subtotal resection of the masses and post-operative radiotherapy was administered for three months. Post-operative magentic resoance imaging revealed no tumor recurrence in the two cases, two years after resection. The relevant literature is also discussed. The patient’s general health was good and no relevant family history was reported. Upon neurological examination, muscle power in the left upper limb was rated as grade 4/5, according to the Medical Research Council scale (A magnetic resonance imaging (MRI) scan of the craniocervical region revealed an expansile intramedullary mass extending from the medulla oblongata to the T4 segment of the spine. The mass was isointense on T1-weighted images, hyperintense with partially cystic mass on T2-weighted images and showed intense heterogeneous enhancement of solid tumor following the injection of gadolinium diethylenetriamine pentaacetic acid (Gd-DTPA) .A C3-7 laminectomy was performed and an intramedullary solid mass extending from C3 to C7 was exposed. The tumor was gray-purple and exhibited features of infiltrative growth. A partial tumor resection was performed using a micro-neurosurgery technique, resulting in 85% of the tumor being resected. Hematoxylin and eosin staining revealed a neoplasm composed of uniform, round cells . ImmunohA 48-year-old female presented to Yuaquan Hospital, Tsinghua University, with a seven-year history of pain in the right lower limb and a five-year history of progressive numbness in all four limbs and lumbar zone anesthesia. MRI revealed a mass extending from the medulla oblongata to the T2 level, with an isointense T1-weighted signal and a hyperintense T2-weighted signal with a partially cystic mass. Intense heterogeneous enhancement of a solid tumor was shown following injection of Gd-DTPA . The tumNeurocytomas are tumors of the central nervous system that are derived from the neuronal precursor cells surrounding the central canal region in the developing fetus . The spiAccording to the literature review, six cases of cervical spinal neurocytomas occurred in young adults while three cases were reported in adults. In the current study, one patient was 24 years old and the other was 48 years old. The clinical presentation of neurocytoma depends on the location and size of the tumor. Upon neurological examination of case one, the patient was found to have abnormal sensation and loss of proximal muscle power in the upper limbs. MRI revealed a mass with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images. These tumors involved multiple spinal segments and contrast-enhanced MRI revealed either homogenous or heterogeneous enhancement of the tumor mass. The tumors did not exhibit any characteristic manifestations and their pre-operative diagnosis was relatively difficult, which is typical of spinal neurocytoma. The differential diagnosis of spinal neurocytomas include ependymoma and oligodendroglioma .et al (et al (Neurocytomas are benign and slow growing, and the majority of patients with ventricular neurocytomas may be treated effectively by surgery. However, total resection of spinal cord neurocytomas is relatively difficult, particularly in tumors involving the upper cervical spinal cord and in those involving multiple segments. Giant cervical cord neurocytomas may be treated with subtotal resection followed by radiotherapy, however, in the present literature review, only the two cases reported by Tatter et al  receivedl (et al  reportedl (et al ,21–23,25In the present study, the cases of two patients with craniocervical neurocytomas were reported, including the clinical presentation, radiological observations and histopathological features. The successful treatment of neurocytomas may be dependent on an early diagnosis and the total surgical resection of the tumors, however, it is unclear whether post-operative radiotherapy is required to prevent tumor recurrence. Although craniocervical neurocytomas are rare and difficult to diagnose, they must be considered as a presurgical differential diagnosis for craniocervical tumors. In addition, a long-term follow-up period is required for craniocervical neurocytomas due to the possibility of local recurrence.