Paratesticular embryonal rhabdomyosarcoma (RMS) is a rare tumor arising from the mesenchymal tissues of the spermatic cord, epididymis, testis and testicular tunics. We report three cases of adult paratesticular RMS, two embryonic and one pleomorphic rhabdomyosarcoma. All the patients underwent diagnostic orchidectomy. The work up investigations revealed lung metastases. Chemotherapy with Ifosfamide and Doxorubicin was used in two cases, whereas Vincristin- Actinomycin D and Cyclophosphamide was received in one case. An objective partial response was reported in 2 cases, with complete response in one case. Paratesticular RMS is a rare and aggressive tumor. Because of the absence of protocols designed specifically for adult patients, it is necessary to follow therapeutic guidelines in pediatric protocols. Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin thought to arise from cells committed to a skeletal muscle lineage. Twenty percent of all cases arise from the genitourinary system . Parates2 day 1 and Ifosfamide 1800mg/m2 per day from day 1 to day 5 every 21 days) in combination with haematopoietic growth factors for six days. Evaluation after 6 months revealed a full disappearance of the pulmonary metastasis; the patient is monitored quarterly by a CT scan of the chest/abdomen/pelvis.A 18-year-old patient who presented to the department of surgical urology with a painless scrotal mass, he underwent a left inguinal orchidectomy. Histologic examination confirmed an embryonic rhabdomyosarcoma grade III of FNCLCC . The pat2 cyclophosphamide 25 mg / m2 orally) is ongoing.A 19-year-old patient, presented to our oncology department, with paratesticular embryonic rhabdomyosarcoma diagnosed because a painless scrotal mass. He had undergone initially an orchidectomy. Post operative work up investigations revealed lung and mediastinal lymph nodes metastases. Palliative chemotherapy was received with combined chemotherapy by Ifosfamide and Doxorubicin for six courses. Evaluation showed a full disappearance of pulmonary metastases and stability of mediastinal lymph nodes. Thereafter the patient received radiotherapy for mediastinal lymph nodes and a maintenance chemotherapy with navelbine intravenously associated with oral cyclophosphamide. After 6 months, the patient relapsed in the lung. Actually, a second-line chemotherapy  is the most common soft-tissue sarcoma of childhood. Its incidence is similar in Africo-American and Caucasian and appears to be lower in Asian populations. This histologic subtype develops with two peaks, the first at the age of 4 years and the second at the age of 18 years . Among aSpread of the tumor is mostly by lymphatics to the iliac and para-aortic nodes, but hematogenous spread does occur most commonly to the lungs and liver , 5. WorkParatesticular sarcomas are rare. There is no standard treatment. In the localized disease, treatment strategies include radical high inguinal orchidectomy, retroperitoneal lymph node dissection, chemotherapy and radiotherapy –9. Some Paratesticular RMS is a rare and aggressive tumor. In the absence of protocols designed specifically for adult patients, it is necessary to follow therapeutic guidelines in pediatric protocols. Systemic chemotherapy is essential in both early and advanced disease and has resulted in improved survival outcomes.


The incidence of segmental testicular infarction is extremely low. The condition usually presents with acute scrotal pain and may be confused clinically and radiologically with a testicular tumor or torsion. To the best of our knowledge, only a few cases have been reported in the English literature. In this study, we present a case of segmental testis infarction in a 23-year-old male with an acute onset of testicular pain. The diagnosis of testicular infarction was considered following sonography examination. Hemorrhagic infarction of the testis was confirmed by surgical exploration and pathological examination. Partial orchiectomy was performed. Although it is uncommon, segmental testicular infarction should be taken into consideration when acute scrotal pain is encountered, since the therapeutic strategy could be conservative. Global infarction of the testes is a common diagnosis in urologic emergencies. Possible causes include torsion of the spermatic cord, incarcerated hernia, severe epididymitis and iatrogenic injury . In contA 23-year-old male with unremarkable past medical history was admitted due to worsening right testicular pain that had been present for three days. Physical examination revealed a mildly swollen right testis with tenderness in the upper pole. The testis was otherwise normal with no mass or hernia. Results of a complete blood count, urinalysis and tumor marker profile were all within the normal range. Color Doppler sonography demonstrated a flowless, well-demarcated, hypoechoic mass located in the upper pole of the right testis . The remThe patient’s scrotum was immediately explored via a scrotal approach. During the surgery, the upper pole of the testis showed a faint blue discoloration; however, no torsion of the spermatic cord or the epididymis was detected. The tunica albuginea of the testicle was incised, revealing necrosis of the upper pole with grossly normal residual testis tissue . Partialet al (Segmental infarct of testicle is a rare clinical entity that is usually diagnosed following orchiectomy. Since the first reported case in 1909 , <70 casThe most common symptom of segmental testicular infarction is testis pain, which is unspecific and indistinguishable from that of other scrotal diseases. Differential diagnosis should thus depend on laboratory tumor marker screening and imaging. Although a previous study proposed the diagnostic value of magnetic resonance imaging in segmental testicular infarction , scrotalet al (The management strategy for segmental testicular infarction remains controversial. The primary intention of surgical intervention is the salvage of testicular tissue along with the pathological exclusion of malignancy. Testis-sparing surgery is therefore an optimal surgical choice, particularly for younger patients . Despiteet al , 16 out In conclusion, we propose that segmental testicular infarction should be considered in patients with acute testicular pain. Either surgical exploration or careful observation is suitable when a preoperative laboratory and imaging evaluation supports a firm diagnosis.


Deep scrotal hemangiomas are rare. Less than 50 case reports have been published. After systematic research of the literature, we found less than 5 cases of ulcerated scrotal hemangioma. The aim of this case report is to illustrate the challenges of scrotal hemangiomas pose and their potential therapies based on the successful surgical treatment of an ulcerated scrotal hemangioma in an 18-month-old male patient. Hemangiomas are the most common vascular tumors of childhood. Their incidence is historically reported as 1–3% of full term neonates and 10%–12% of children up to the age of 12 months –6. UlcerAfter analysis of the literature, we found less than 5 case reports of ulcerated scrotal hemangiomas. This observation was the motive for writing down the present study.An 18-month-old male patient was referred to our Outpatient Department seeking further consultation for a corrosive lesion over an already known hemangioma on the anterior surface of the scrotum. The patient was born at term without any abnormalities diagnosed during antenatal ultrasound scan and was free of any other medical history. The hemangioma was initially diagnosed 15 days after birth and its natural history of uncomplicated rapid growth was documented and followed by the family doctor. However, 40 days prior to consultation, a lesion at the hemangioma site was noted creating increased discomfort during urination and defecation of the patient.Clinical examination confirmed the presence of a cavernous hemangioma measuring 2.5 × 2.3 cm, occupying almost the entire anterior surface of the scrotum. In addition, an extended ulceration covering almost the entire hemangioma surface (2.1 × 1.9 cm) was also observed.Intrascrotal extension of the hemangioma was smooth at palpation, reaching deep in the scrotum, respectively, to the mesoscrotal diaphragm. No pathology was observed during the examination of intrascrotal structures of each hemiscrotum.A color Doppler ultrasonographic evaluation of the scrotum, perineum, inguinal area, and pelvis confirmed the presence of increased vascular flow inside the mass and excluded its expansion to the lesser pelvis. Finally, the normal echo graphic morphology of the intrascrotal anatomic structures was demonstrated.After discussion with the parents and following their preference, a decision for surgical excision was reached. Under general anesthesia, 4 stay sutures setting the skin limits of the lesion were placed. Following a tapered incision at the limits of the healthy scrotal skin and progressive dissection of the scrotal layers, the feeding vessels were identified and ligated Figures , and 3.The significant scrotal defect that remained after excision of the hemangioma was restored by repositioning the ventricular part of the scrotum dorsally .The patient had an uncomplicated postoperative period and was discharged the second postoperative day. Histological examination confirmed the diagnosis of the cavernous hemangioma . At one-Hemangiomas are the most common vascular lesions in childhood. Although scrotal hemangiomas are uncommon, they can create significant discomfort for both the patient and the family due to their location and potential complications. Complications that can be caused due to a deep scrotal hemangioma include hemorrhage, consequences of the extension of the lesion to the anatomical structures of the lesser pelvis  such as rectal bleeding and hematuria  ulceratiThe commonest complication of the lesion is ulceration which is believed to be triggered by exposure to stimulating factor such as the irritant action of secretions like urine and stools and the decreased oxygenation of the surface of the hemangioma . AlthougAlthough in our case the profound extension of the hemangioma was obvious, the development of a scrotal ulcer should be differentially diagnosed from trauma, herpes viral infection, intrauterine infection from varicella, infection from pyocyanin pseudomonas, aspergillus, pyogenic granuloma, pyoderma gangrenosum, and so forth . If therImaging studies must determine the vascularization of the lesion, its relation with intrascrotal structures, and its potential expansion to the penis, perineum, and structures of the lesser pelvis , 13, 14.Oral propranolol is considered as the first-line treatment for infantile hemangiomas , 18. The2, Nd: YAG, yellow-light), infusion of NaCl 15%, and cryotherapy can be considered [However, in cases of scrotal hemangiomas, many authors recommend the scheduled surgical removal of the lesion, with preservation of anatomic structures of the scrotum , 14, 16.nsidered –24. Flasnsidered . Sarig ensidered . In our In conclusion, both superficial and deep scrotal hemangiomas are rare lesions, which can induce increased discomfort to patients and their families due to their localization and their possible complications. It is crucial that conservative treatment with administration of propranolol starts within the stage of angiogenesis. However, in older babies or children, if no conservative treatment was initiated or has failed, laser treatment or surgical management and excision of the lesion should be considered.


Dear SirA 2-day-old, full term (38 weeks), appropriate for gestational age, female baby was delivered vaginally to 22-year-old primigravida at peripheral health facility. The baby did not cry at birth, required positive pressure ventilation for 3 minutes at birth and Apgar score at 1 and 5 minutes after birth were 3/10 and 6/10 respectively. No features of hypoxic ischemic encephalopathy were noticed. The newborn was referred for further investigation and treatment to tertiary health center. There was no history of any drug intake or radiation exposure in mother during antenatal period. On examination, a mass of 4.3 cm × 4 cm × 2.5 cm with two skin appendages was found to be attached to the lower part of the chest wall of the baby . An exomphalos major and hamartoma of size 3.2 X 3.5 cm in left lower leg was present in the neonate. The liver was protruding into the exomphalos sac that was confirmed on abdominal sonography. Ultrasonography of chest, skull, spine and abdomen, apart from exomphalos were normal. Echocardiography revealed small ventricular septal defect (VSD). The thoracic mass was excised by elliptical incision after ligating the feeding vessels without any complications. The primary repair of exomphalos major was also done simultaneously. The contents of sac were part of liver and small bowel. Histopathological examination labeled the separated mass as teratoma. The postoperative period was uneventful and baby was discharged after 7 days. The baby is growing well in the follow up. The small VSD closed spontaneously and hamartoma did not increase in size during the follow up period. Associated malformations are not rare with omphalocele, but teratoma associated with congenital omphalocele is extremely rare. Only 11 cases of teratoma associated with omphalocele are reported in the literature hitherto. The teratoma in the reported cases was present as a part of omphalocele sac in six cases and five cases had umbilical cord teratoma [1-5]. Again, hamartoma in association with omphalocele major other than mesenchymal hamartoma of liver is extremely rare . So, we are reporting a rare case of exomphalos major associated with thoracic teratoma and hamartoma of left lower leg.Source of Support: NilConflict of Interest: None


A 24-year-old male presented with eight months of increasingly severe frontal headaches, decreased right facial sensation, and periodic vertigo. Magnetic resonance imaging demonstrated a heterogeneously contrast-enhancing mass involving and expanding the right foramen ovale.  A biopsy of the lesion was performed, and the final pathologic diagnosis revealed a neoplastic rhabdomyoma. To date, only five cases of intracranial rhabdomyoma have been reported, and a rhabdomyoma involving the trigeminal nerve has never been described in an adult. This manuscript reviews the available literature and highlights the clinical, imaging, pathologic characteristics, and surgical management of these exceedingly rare lesions. Rhabdomyomas are rare neoplasms of skeletal muscle that typically arise in oral, cervical, vulvovaginal, and cardiac tissue . In contWe report the case of a 24-year-old male with a heterogeneously enhancing expansile mass within the right foramen ovale on magnetic resonance imaging. On pathologic examination, the lesion demonstrated histologic features of a neuromuscular choristoma, but with proliferating Ki-67-positive cells, which therefore supports the diagnosis of neoplastic rhabdomyoma.A 24-year-old male presented with an eight-month history of bilateral frontal headaches that were progressively increasing in frequency and severity. The patient endorsed intermittent vertigo. Neurologic examination revealed decreased sensation to light touch on the right forehead and cheek. MRI with and without contrast revealed a heterogeneously enhancing mass within the foramen ovale with surrounding edema and enhancement of the pterygoid muscles; computerized tomography of the head revealed a smooth, nondestructive expansion of the right foramen ovale Figure . InformeA right subtemporal craniotomy was performed and exposure of the foramen ovale was achieved extradurally. This revealed the entire tumor, which involved the third segment of the trigeminal nerve and extended through the foramen ovale into the extracranial space. The lesion had the gross appearance of fibrous scar tissue.Hematoxylin and eosin (H&E) stained specimens revealed disordered skeletal muscle fibers, peripheral nerve bundles, dense fibrous connective tissue, and scattered mature adipose tissue. Intersecting and interwoven fascicles of myocytes, ranging from elongate strap cells to rotund globular forms, were observed. Figures Electron microscopy examination confirmed the presence of both well-differentiated skeletal muscle cells and highly differentiated but thinly myelinated peripheral nerve Figure . The musFinally, immunohistochemistry for Ki-67  was performed to determine if the lesion was, in fact, proliferating. As shown in Figure The patient did well after surgery with no new postoperative neurologic deficits. Relief of facial pain was achieved. He has remained free of tumor progression on serial MRI imaging for over 18 months of follow-up.The finding of benign skeletal muscle fibers admixed with myelinated axons and adipose tissue suggests a diagnosis of neuromuscular choristoma/hamartoma, rhabdomyoma, or myolipoma. In this patient, the predominate histopathology was intermingling benign muscle and nerve. Thus, a differential of neuromuscular choristoma versus rhabdomyoma is favored. Neuromuscular choristomas are hamartomatous lesions that have been reported to occur in cranial nerves, including the trigeminal nerve, but this is rare -2. In faWhile the literature suggests that lesions without a significant neural component are favored to be rhabdomyomas over choristomas , we prefLittle is known about the prognosis of intracranial rhabdomyomas. Vandewalle, et al. reported the case of a 41-year-old Caucasian male who suffered from progressive paresis of the right facial nerve since childhood . The patThere is currently no consensus on the optimal treatment of intracranial rhabdomyomas. Van Leeuwen, et al. reported the case of a six-year-old male who presented with several years of progressive hearing loss . An MRI Since patients almost exclusively present with neurologic deficits, a strong case can be made for mechanical decompression of involved neurologic structures. Given the slow natural progression of the disease as evidenced by the available six cases, restoration and preservation of neurologic function, rather than maximal resection at the risk of neurologic compromise, should remain the neurosurgeon's primary goal Table .Neurosurgeons should understand the natural progression and clinical behavior of intracranial rhabdomyomas. Nuclear proliferation indices, such as the Ki-67 immunostain, are important in differentiating intracranial rhabdomyomas from the more benign neuromuscular choristoma. Surgical management aimed at decompressing, restoring, and preserving neurologic function should remain the goal because these neoplasms demonstrate an indolent clinical progression.


Primary splenic angiosarcoma is an extremely rare and aggressive neoplasm of the vasculature. Uniformly, primary splenic angiosarcoma is a fatal disease despite early diagnosis and treatment. Only patients with localized disease amenable to surgical resection achieve long-term, disease-free survival. We present a review of the literature and report a case of a 3-year-old girl with metastatic primary splenic angiosarcoma who was offered aggressive surgical and medical treatment with curative intent despite her advanced presentation. Primary splenic angiosarcoma (PSA) is an extremely rare and aggressive neoplasm of the vasculature that occurs in adults and children. Uniformly, PSA is a fatal disease despite early diagnosis and treatment. To date, only patients with localized disease amenable to surgical resection are likely to achieve long-term, disease-free survival. In this review, we summarize the literature and report a case of a 3-year-old girl with metastatic PSA who was offered aggressive surgical and medical treatment with curative intent despite her advanced presentation. Following splenectomy, our patient was given a gemcitabine-docetaxel chemotherapeutic regimen, which has never been described before in children with PSA. This treatment regimen achieved a partial response and was followed by liver metastasectomy. This enabled us to render her free of any gross disease and allowed her to undergo the living-related liver transplantation evaluation as a viable curative option.We report on a previously healthy 3-year-old girl who presented with complaints of abdominal pain and distention, and decreased oral intake in the setting of high fevers. On physical examination, she was found to have massive splenomegaly. Her laboratory work-up was significant for anemia and mild thrombocytopenia. An abdominal X-ray showed a markedly enlarged spleen with displacement of bowel and an abdominal ultrasound revealed several hyperechoic nodules in an enlarged spleen. This prompted magnetic resonance imaging (MRI) of the abdomen which showed massive splenomegaly with central hemorrhage and multiple nodular lesions in the liver Figure .Fearing Her post-operative work-up included positron emission tomography-computed tomography (PET/CT), which revealed fluorodeoxyglucose-avid disease exclusive to the liver Figure . Given tAfter extensive discussion in our multidisciplinary tumor board, and given her favorable response to chemotherapy, the option of living-related liver transplantation was made available to the family as a means to render the patient disease-free. However, during the transplant evaluation time period, the patient had clinical deterioration and was admitted to the hospital with worsening abdominal pain and distention. She was found to have bilateral pleural effusions and extensive ascites. Liver failure rapidly ensued and, within days of her admission, her family opted for comfort care measures. She died shortly thereafter.First described by Theodor Langhans in 1879 , PSA is To date, only two case series have been published that describe the pathologic behavior of PSA on a combined 68 adult patients , 4. HoweHistologically, angiosarcomas are rapidly proliferating, highly infiltrating anaplastic tumors that recur locally, spread widely, and have a propensity for rapid lymph node and systemic dissemination , 4, 13. Radiologic features of PSA can range from ambiguous lesions to highly aggressive-appearing tumors in the spleen, often with concomitant metastases at the time of diagnosis . UltrasoMultiple studies on angiosarcomas have reported the lack of association between histological appearance or grade with survival . It appeThe prognosis for patients with PSA is grim. In the two major case series that examined the behavior of PSA, the overwhelming majority of patients presented with advanced disease , 4. The Owing to the rarity of PSA in the pediatric population, the choice of treatment regimen on this particular case was based on the adult angiosarcoma literature. Case reports in adults, while lacking large cohorts of patients, seem to indicate that patients with angiosarcoma respond to gemcitabine and docetaxel, or to other taxanes , 22, 23.A retrospective review conducted by Leu and colleagues examined 35 patients with bone and soft tissue sarcomas, including four patients with angiosarcoma . Some paAdditional clinical trials have demonstrated the efficacy of other taxanes at treating angiosarcomas. Penel and colleagues enrolled 30 patients with metastatic or unresectable angiosarcomas in a Phase II clinical trial examining the efficacy and toxicity of weekly paclitaxel . Eight pThe seven pediatric patients previously described in the literature with the addition of the patient in this case are listed in Table Two of the eight pediatric patients achieved durable remission. The patient with disease limited to the spleen was treated by splenectomy without adjuvant therapy. The patient described by den Hoed and colleagues presented with splenic rupture and liver metastases . She wasPSA is an extremely rare and aggressive neoplasm of the vasculature that has a very poor prognosis. We present an aggressive multidisciplinary approach in a 3-year-old girl with metastatic PSA who was treated with splenectomy, liver metastasectomy, chemotherapy, and an attempt to liver transplantation. This case report and review of the literature reveals that angiosarcoma in children may be chemo-responsive in some cases. However, this case also illustrates that, in patients with gross disease, surgical extirpation of the primary neoplasm and all metastases is necessary to achieve durable remission. Furthermore, this case underscores the importance of an aggressive multi-disciplinary team approach in these complicated cases aiming for a cure.Written informed consent was obtained from the patient’s family for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The patient was diagnosed with glucagonoma syndrome, and a distal pancreatectomy and splenectomy were subsequently performed. Microscopy revealed that the tumour cells exhibited nest- and belt-like arrangements. The immunohistochemical staining identified positive reactions for glucagon, synaptophysin and chromogranin A, which are consistent with a diagnosis of glucagonoma. Following surgery, the symptoms disappeared and the glucagon level returned to normal. In conclusion, imaging examinations are useful for determining the location and size of a glucagonoma. In particular, MRI is able to identify the distinctive morphological features of the lesion. Immunohistochemical staining provides diagnostic evidence based upon the neuroendocrine features.Glucagonoma syndrome appears as an extremely rare neuroendocrine tumour, with few studies ever having detailed its imaging manifestations. In particular, the magnetic resonance imaging (MRI) features of the lesion have not yet been reported. The present study describes a 54-year-old male who presented with uncontrollable skin erythema and weight loss that had been apparent for two years, and diabetes mellitus that had been apparent for five years. The glucagon level was 180 pg/ml. The plain abdominal computed tomography (CT) scan revealed a solid tumour in the neck of the pancreas, which was slightly reinforced during the arterial phase of the enhanced CT scan. Upon MRI, the lesion exhibited a low signal on T1-weighted imaging, and a slightly high signal on T2-weighted and half-Fourier acquisition single-shot turbo spin echo sequence imaging, which measured ~4.5×3.0×3.0 cm in size. Upon diffusion-weighted imaging, the lesion demonstrated heterogeneous hyperintensity, which was mildly enhanced during the arterial phase and washed out during the portal venous phase of gadopentetate dimeglumine-enhanced MRI.  The present study describes and discusses these imaging features.Glucagonoma is an extremely rare neuroendocrine tumour that accounts for 1% of neuroendocrine tumours and <5% of all primary pancreatic malignancies . AlthougA 54-year-old male was admitted to the First Affiliated Hospital of Shandong University  due to persistent and progressive skin eruptions and weight loss of ~20 kg over the past two years. The medical history revealed that the patient had previously been diagnosed with Behçet’s disease and treated with corticosteroids during multiple hospital admissions. However, there had been no significant improvement of the symptoms. In addition, the patient had suffered with diabetes mellitus for five years. The patient stated that the skin erythema had recently become progressively worse. The family history was negative for multiple endocrine neoplasia and diabetes mellitus. Upon physical examination, the patient exhibited cyclical itchy skin lesions on the face, back, groin and lower limbs. The centres of the lesions were hypopigmented or slightly scaly. There was no palpable mass evident in the abdomen.12/l ; haemoglobin level, 81 g/l ; mean corpuscular volume, 86.1 fl ; mean corpuscular haemoglobin level, 29.5 pg ; and mean corpuscular haemoglobin concentration, 343 g/l ], hyperglucagonaemia  and hyperglycaemia . In addition, the expression of the tumour marker, carbohydrate antigen 19–9, was markedly increased , whereas the α-fetoprotein and carcinoembryonic antigen levels were within the normal ranges. The exocrine function was also normal.Laboratory analysis revealed normocytic anaemia [red blood cell count, 2.59×1018F-FDG PET-CT revealed mild 18F-FDG uptake by the lesion  in the neck of the pancreas, which corresponded to the location of the tumour identified by the CT and MRI scans (The plain abdominal CT scan identified an obscure mass in the neck of the pancreas with a vague margin. Upon enhanced CT, the lesion was slightly enhanced during the arterial phase and washed out during the portal venous phase. The body and tail of the pancreas were atrophied. There was no evidence of enlarged lymph nodes or liver metastases . Upon MRRI scans .Based upon the clinical presentation and imaging findings, the patient was diagnosed with glucagonoma syndrome. A distal pancreatectomy and splenectomy were subsequently performed. The regional lymph nodes were also dissected. The histopathological examination revealed that the tumour was composed of uniform round and polygonal cells, with pale cytoplasm and round nuclei. The tumour cells exhibited nest- and belt-like arrangements. The immunohistochemical staining identified positive reactions for glucagon, synaptophysin and chromogranin A, a weakly positive reaction for insulin , and negDuring the eight-month post-surgery follow-up period, the skin lesions disappeared and the plasma glucagon levels returned to normal.The present study was approved by the Institute Ethics Committee of the First Affiliated Hospital of Shandong University, and written informed consent was obtained from the patient for the publication of the study and any accompanying images.et al (Glucagonoma may appear as a benign or slow-growing metastasising malignant tumour ,6. The fet al  in 1942,In the present study, the CT scans identified an obscure mass in the head and neck of the pancreas, but no distinctive features. However, the MRI scans using T1WI or T2WI identified morphological characteristics, including the contour and internal structures of the lesion. DWI is extremely sensitive to the motion of water protons at the microscopic level in response to thermal energy . In contet al (Teixeira et al  reported18F-FDG is considered to be a tracer of glucose metabolism, as its molecular structure is similar to that of glucose. Subsequent to injection, 18F-FDG can be transported into the cell through the glucose transporter proteins on the cell membrane. Consequently, PET-CT identifies high 18F-FDG uptake in rapidly growing tumours, in which the glycolysis rate is increased (18F-FDG uptake, and no metastases were detected in any other organs.PET-CT remains as a promising imaging technique for detecting the presence of tumours. ncreased . In the Stacpoole  stated tSurgical resection is the optimal strategy for the treatment of glucagonoma . DependiIn conclusion, glucagonoma syndrome exhibits certain typical clinical manifestations. Imaging examinations are useful for determining the location and size of a glucagonoma, and in particular, MRI can identify distinctive morphological features. Immunohistochemical analysis provides diagnostic evidence based upon the neuroendocrine features. The present study summarized the multimodality imaging features of glucagonoma, which are of great importance for the differential diagnosis of pancreatic tumours.


Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury of the brain are the most common causes of secondary CPP. In this report, we present the case of a 2 years and 9 months old male patient who had a 30x40 mm contrast-enhancing suprasellar mass and was histopathologically diagnosed with giant HH. However, since HHs are designated as non-enhancing masses, considering the possibility of an incomplete diagnosis of a glial tumor, the patient was followed up. Clinical and radiological follow-up revealed stable findings with no evidence of tumor growth until the third year after surgery when he presented with neurological deficit due to the rapid growth of the suprasellar mass. After the second surgery, histopathological examination of the biopsy specimen revealed the lesion to be a juvenile pilocytic astrocytoma (PA). The concomitance of HH and juvenile PA is very rare. To our knowledge, this is the first report of a patient with concomitant juvenile PA and HH who developed CPP and did not have gelastic epilepsy despite the rapidly growing giant mass. Central precocious puberty (CPP) or gonadotropin-releasing hormone-dependent precocious puberty, which is caused by premature activation of the hypothalamo-pituitary-gonadal axis is more common in girls than in boys . ApproxiIn this report, we present the case of a patient with concomitant HH and juvenile PA who developed CPP within the second year of clinical follow-up and who showed no epileptic symptoms despite the giant mass.A 29/12 years old male patient had been admitted to the emergency department with complaints of headache, vomiting and gait disorder which had developed within the past month. In brain magnetic resonance imaging (MRI), a lobulated contoured and contrast-enhancing suprasellar mass was detected. The mass was 30x40 mm in size, consisted of solid and cystic components and had led to hydrocephalus . After iThe patient was referred to the pediatric endocrinology clinic for evaluation of probable anterior and posterior pituitary hormone deficiencies. On physical examination, his weight was 16 kg (75-90p) [+1.28 standard deviation score (SDS)] and height was 98 cm (90-95p) (+1.36 SDS). Clinical findings were normal and puberty was compatible with Tanner stage 1. Determination of hormone levels in a morning plasma specimen revealed an adrenocorticotropic hormone level of 46 pg/mL , a serum cortisol level of 19.7 µg/dL , a prolactin level of 3.19 ng/mL . Thyroid-stimulating hormone level was 3.98 mIU/mL  and free thyroxine level was 1.05 ng/dL . In view of the location of the mass, it was decided that the patient needed to be followed up for the possible development of hypothalamo-pituitary disorders such as CPP and growth failure. He was also being followed up clinically and radiologically for his giant intracranial mass. At the age of five years and six months, the patients’ weight was 23 kg (75-90p) (+1.23 SDS), his height was 116 cm (75-90p) (+0.98 SDS) and his testicular volumes were 5 mL and 6 mL. He had no pubic hair. During the physical examination, aggressive behavior and mental retardation were noted. However, he had no history of any type of epilepsy. Biochemical measurements revealed a basal follicle-stimulating hormone level of 2.48 mIU/mL , luteinizing hormone (LH) level of 1.45 mIU/mL  and a total testosterone level of 50 ng/dL . Scrotal USG revealed age-inappropriate enlargement of the testicles with normal echotexture - the right testicle was 22x15x35 mm and the left testicle was 21x16x34 mm in diameter. The bone age was compatible with the chronological age. In LH-releasing hormone test, a peak LH value of 15 mlU/mL (>5) was detected by direct chemiluminometric technique. The patient was diagnosed with CPP and started on gonadotropin-releasing hormone (GnRH) agonist treatment (triptorelin acetate 3.75 mg every 4 weeks). A normal electroencephalogram ruled out subclinical epileptic activity. Until the third year after the surgery, the patient was clinically and radiologically stable with no evidence of tumor growth. However, at the age of six years and three months, the patient was referred with neurological deficit due to the rapid growth of the suprasellar mass. After the second surgery, he died because of brain herniation. Histopathological examination of the biopsy specimen revealed the lesion to be a juvenile PA .HHs are rare 1:200 000) heterotopic congenital intracranial malformations with a mean diameter of 17.9-18 mm. They consist of benign neural and glial cells and usually originate from tuber cinereum, mamillary bodies and posterior hypothalamus ,7,8. HHs00 000 heThe treatment of choice in HH is surgical; however, due to adhesion and invasion to the adjacent structures, it cannot be usually removed completely. In HH cases with intractable epilepsy and mass-related clinical findings, while surgical resection is recommended, wide resections must be avoided to prevent the development of neurologic sequelae ,10. In cPA is the most common benign brain tumor in childhood. It usually arises within the cerebellum and rarely in the hypothalamic/chiasmatic region, but it may occur in any area where astrocytes are present ,12,13,14CPP due to astrocytoma has been reported previously . The curPreviously, Park et al  have repIn conclusion, although HH is the most frequent organic cause of CPP, in a patient with a hypothalamic mass, the presence of pathological contrast enhancement in MRI should alert the physician to consider a diagnosis other than HH, particularly a glial tumor. With this case report, we would like to emphasize that although rare, HH and juvenile PA may occur concurrently in a patient.


Disc fragments are well known to migrate to superior, inferior, or lateral sites in the anterior epidural space, posterior epidural migrated lumbar disc fragments is an extremely rare disorder. Posterior epidural migrated lumbar disc fragments are often confused with other posterior epidural space-occupying lesions . We reported the case of a 52- year-old man presented with progressive not systematizes bilateral radiculopathy complicated one week before admission a difficulty dorsiflexion prevents the start, and the stared to use crutches. Clinical examination revealed steppage gait and a strength score of 3/5 on dorsiflexion of feet. MR imaging of lumbar spine showed right posterolateral epidural mass that compressed the dural sac at the L3-4 level. Patient underwent surgery using posterior approach, an L3 laminectomy was performed, the extruded disk fragment was gently removed and L3-L4 interspace was explored. Histopathology confirmed the (PEMLIF). Postoperative course was uneventful. The migration of the lumbar intervetebral disc fragment to the posterior epidural space (PEMLIF) is a rare event just few cases are reported in English literature . ClinicaA 52-year-old man presented with 2 years history of intermitent lumbago. Since 2 months ago, the experienced a progressive not systematizes bilateral radiculopathy complicated one week before admission a difficulty dorsiflexion prevents the start, and the stared to use crutches. Clinical examination revealed steppage gait and a strength score of 3/5 on dorsiflexion of feet, patellar and Achilles reflex were depressed, and there were any sphincter symptoms or saddle hypoanesthesia. Past history revealed no history of trauma neither fever, nor weight loss. Laboratory test was also normal. Plain radiographs of the lumbar sacral spine were essentially normal, MR imaging of lumbar spine was realized based emergency and objectified a reshuffle osteoarthritis associated with degenerative disc disease and right posterolateral epidural mass that compressed the dural sac at the L3-4 level . PatientVicenzo Lombarti the first author to publish two cases of PEMLIF in 1973 , 5. PostDespite their rarity PEMLIF should be raised before any CES, or isolated neurological deficit of lower limbs resent or old, to realize an MRI emergency is to propose if appropriate, a precocious and radical surgical treatment.


Inflammatory myofibroblastic tumour (IMT) is an uncommon mesenchymal tumour, which can occur anywhere in the body, rarely in esophagus. Mostly, the diagnosis is postoperative, after the hispathological evaluation of the specimen. There are no definite guidelines regarding the diagnosis and management. Here, we report a 60 year old lady presenting with dysphagia, diagnosed to have a submucosal esophageal tumor with Barium esophagogram and contrast enhanced computed tomography. She was managed successfully with surgical enucleation with the final histopathological diagnosis of IMT. Surgical excision is not only therapeutic but also diagnostic in such cases. Inflammatory myofibroblastic tumor  is a rare mesenchymal tumor. It is a distinctive lesion composed of myofibroblastic spindle cells with an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. It occurs most commonly in the lungs and uncommonly in the sites such as brain, trachea, breast, spleen, kidneys, liver, stomach and the ampulla of Vater. It is extremely rare in the esophagus and there are very few cases of esophageal IMT reported in English literature till date. It is seen mostly in children and young adults but can occur in any age, affecting females and males equally  [We report an elderly female who underwent complete excision of the esophageal IMT and is doing fine without evidences of recurrence at follow up visits.A 60 year old diabetic, non smoker, housewife presented to us with complaints of gradual onset, progressive dysphagia initially for solid and later for liquid food for one and a half year and foreign body sensation in throat for 6 months. She had lost 10 kg over last 6 months, despite having a good appetite. She did not have significant medical or surgical illness or intervention in the past. General and systemic clinical examination was normal. Her hematological and biochemical investigations were normal too. Barium esophagogram revealed smooth narrowing of the mid thoracic esophagus with proximal dilatation Fig. . ContrasThe etiology of IMTs is supposed to be an aberrant response to tissue injury with myofibroblast being the primary cell type . The comAlthough esophageal IMT is an extremely rare entity, it should always be considered a differential diagnosis in a patient presenting with dysphagia and submucosal esophageal tumour. The complete excision of the tumour is not only therapeutic but also helps to confirm the diagnosis with histopathological examination and immunohistochemistry. As it is an intermediate grade tumour with recurrence potential, patient should be followed up regularly.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity. A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness. PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas. MRI of the abdomen demonstrated two 6-7 cm masses in the liver. MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation. Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1). Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver. FISH analysis of these lesions demonstrated an ALK (2p23) gene rearrangement. The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission. We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation. This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy. Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm more commonly occurring during the first two decades of life. IMTs can arise in various anatomic locations –6 but ocA 45-year-old obese Hispanic female, status post cholecystectomy, presented initially with left leg pain, low back pain, generalized weakness, and right-sided blurred vision. Physical examination at that time revealed an abnormal left leg gait and a right lateral strabismus. Laboratory studies revealed mildly elevated liver enzymes . Imaging revealed multiple small liver masses and lesions in the lumbar spine. A liver biopsy was performed and was initially interpreted as nonspecific subacute and chronic inflammation with abscess formation. No granulomata, organisms, or evidence of malignancy were seen, and the patient was treated with intravenous antibiotics. Due to high suspicion of tumor in the lumbar region, an exploration of the L3-L4 region was also performed. Operatively, the nerve roots were found to be inflamed in the area, and a proteinaceous cyst was also seen. The patient was subsequently discharged after extensive workup and cultures proved negative.The patient was readmitted three months after initial discharge due to worsening of low back pain, pain on ambulation, and bilateral lower extremity weakness and numbness, as well as an increase in size of the hepatic and spinal lesions on imaging. Physical exam now showed decreased pinprick, dysesthesias, and weakness with dorsiflexion in the lower extremities bilaterally. Right third cranial nerve palsy was also noted. Repeat MRI of the lumbar spine with and without Gadolinium revealed diffuse enhancing bony lesions involving the L2 to L4 spinous processes, posterior paraspinal muscles, and posterior subcutaneous tissues, and an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. MRI of the abdomen with and without Gadolinium demonstrated a poorly defined 6-7 cm mass in the left lobe of the liver and a 6 cm mass in the right lobe of the liver . PET/CT Histologic examination of the liver and spine lesions revealed a spindle cell proliferation admixed with an inflammatory infiltrate composed of lymphocytes, plasma cells, histiocytes, and eosinophils . The spiInflammatory myofibroblastic tumor is defined by the World Health Organization as a distinctive lesion of myofibroblastic spindle cells with an inflammatory infiltrate composed of lymphocytes, eosinophils, and plasma cells . It is aPathologic features of IMTs including tumor size, mitotic rate, presence or absence of necrosis, and cellular atypia do not appear to correlate well with clinical outcome. A small subset of IMTs demonstrate histologic progression to malignancy with increased cellularity/pleomorphism of spindled, round, or polygonal cells with vesicular nuclei, high mitotic rate, and atypical mitoses . HoweverWhile the nature of IMT is debated and historically thought to be driven by an inflammatory process , 27, GriBased on a recent study in the New England Journal of Medicine of successful use of ALK-inhibitor in a treating a patient with ALK-positive IMT , our patIn summary, this case represents a distinct presentation of inflammatory myofibroblastic tumor with systemic involvement and ALK gene rearrangement. This case illustrates how personalized medicine through the use of molecular testing has made individual targeted therapy successful.


Primary testicular lymphoblastic lymphoma is a rare entity. We report a case of a 13-year-old boy referred with unilateral testicular swelling. After preliminary work-up orchiectomy was performed Histopathology detected primary testicular lymphoblastic lymphoma. Lymphoblastic lymphoma should be considered in the differential diagnosis of testicular masses in children. Lymphoblastic lymphoma is usually reported in children and adolescents. In approximately half of the cases mediastinal mass is present. Malignant lymphoma constitutes 5% of all testicular malignancies.[1] In adults primary diffuse large B cell lymphoma is the most frequent lymphoma, whilst the majority of testicular lymphomas in children express secondary involvement by Burkitt lymphoma, diffuse large B cell lymphoma, or lymphoblastic lymphoma.[2] Testicular infiltration occurs more commonly during disease dissemination or relapse. Primary testicular lymphoblastic B-cell lymphoma is a rare entity. Here we report a case of primary testicular precursor B-lymphoblastic lymphoma in a teen ager boy.An otherwise healthy 13-year-old boy presented to a local private clinic for a sudden unilateral enlargement of the left testis. His past medical history was unremarkable. There was no history of trauma or fever. Examination of the scrotum showed moderate swelling and tenderness of the left testicle; right testis was normal. There was no lymphadenopathy. Ultrasound revealed a highly vascularized, hypoechoic lesion, completely infiltrating the testis. The clinical impression was testicular tumor and a total orchiectomy was done through inguinal approach. On gross examination, a complete replacement of the testis by a whitish, relatively homogenous tumor was observed. Microscopically, there was a diffuse pattern of proliferation of relatively small lymphoid cells, between and displacing seminiferous tubules . The neoplastic cells had scanty cytoplasm and nucleus with delicate nuclear membrane convolutions. The chromatin was finely stippled, and nucleoli were inconspicuous. Mitotic activity was extremely high. The neoplastic cells expressed CD 45, TdT, CD10, CD99, HLA DR, and CD34; and were negative for CD117, CD2, CD 20 and CD3. Ki67 was positive in 90% of tumor cells . He had no superficial lymphadenopathy or hepatosplenomegaly. Chest CT scans and abdominal ultrasound did not indicate mediastinal and retroperitoneal enlarged lymph node. Laboratory test results were within normal range. The result from cerebrospinal fluid and bone marrow aspiration showed that there was no infiltration. He was diagnosed with B lymphoblastic lymphoma, Ann Arbor stage I.A multidisciplinary consultation with urologists, oncologists, radiation oncologists and hematologists was done. Despite the absence of bone marrow infiltration and other mass lesions, the oncologist chose to treat the patient with a high dose combined systemic and intra-thecal chemotherapy, followed by intensive consolidation therapy . His parents refused radiation therapy. At the completion of the consolidation phase of treatment a PET scan was done which showed no abnormality. After 8-month of follow-up, the patient is without disease.Primary testicular lymphoblastic lymphoma is labelled if the disease is restricted to the testis and no signs of bone marrow involvement are present at diagnosis or develop during follow-up. In our case no lymph node, central nervous system, or bone marrow involvement was detected at diagnosis. There are few reports in literature about primary testicular B-lymphoblastic lymphoma.[3-5] Literature review disclosed five cases of primary testicular pre B lymphoblastic lymphoma.[3-6] Primary testicular lymphoma usually presents as a unilateral testicular mass with occasional bilateral involvement. In 10-18% of cases there is synchronous or asynchronous involvement of contra lateral testis. [7] Scrotal ultrasound can be used for initial evaluation and follow-up after therapy to determine recurrence. Sonography usually reveals a focal hypoechoic mass without a distinctive capsule or diffuse enlargement and decreased echogenicity of the whole of the testis which is different from the normal testis.[8] However, primary testicular lymphoma is not associated with any specific findings at ultrasound and is not different from other testicular tumors.A round blue cell tumor of testis has following major differential diagnoses as lymphoblastic lymphoma, Burkitt lymphoma, blastic variant of mantle cell lymphoma, small lymphocytic lymphoma, myeloid sarcoma, Ewing sarcoma/peripheral neuro-ectodermal tumor, neuroblastoma, and embryonal rhabdomyosarcoma.[9] The age of the patient, clinical presentation, morphologic evaluation and, finally immunohistochemistry allow discrimination among these entities. Similarly in our case, immunohistochemistry was helpful in achieving the correct diagnosis. Once diagnosis of testicular lymphoblastic lymphoma has been made, a complete staging is essential. Contrary to the localized presentation of disease, primary testicular lymphoblastic lymphoma is a systemic process and treatment should be provided to minimize recurrence. Result of one study showed that chemotherapy without radiation therapy for localized lymphoblastic lymphoma in children led to a relapse rate of 30%.[10] In the current case, the oncologist chose to treat the patient with a high dose combined systemic and intra-thecal chemotherapy, followed by intensive consolidation therapy. However, his parents refused radiation therapy. Our therapeutic approach was similar to Garcia et al.[6] In conclusion, though very rare, primary testicular lymphoblastic lymphoma should be considered in the differential diagnoses of testicular masses, especially in young patients.Source of Support: NilConflict of Interest: None declared


Dental lamina cysts are cysts that occur as white or pink small nodules, often multiple, approximately 1 to 3mm in diameter. They are typically located on the midpalatine raphe and less frequently on the maxillary and mandibular alveolar mucosa; in the latter case these can be appear to be neonatal teeth.On microscopic examination, these lesions show a stratified squamous epithelium (two to three cell layers); it is possible to find protein, keratin and/or exfoliated epithelial cells in the lumen of the lesions.Neonatal cysts usually show no particular symptoms. They are associated with an excellent prognosis because they regress spontaneously within a few weeks and are not associated to any complications. However, if pain, bleeding or other symptoms occur, a surgical excision is required.In this paper, we present an anomalous case of symptomatic dental lamina cyst which affected a 60-day-old male Caucasian newborn. The surgical treatment was elective in this case and 6-month follow-ups were mandatory.We can underline the successful predictability of the surgical approach; however, we consider that the treatment choice should take place in the light of medical history and clinical considerations, and always be evaluated on a case-by-case basis. Further studies and reviews in this field should be performed in order to suggest guidelines for clinicians, although these cases are rare. Many features of a baby’s mouth are unique and peculiar to this development period, such as gingival cysts, inclusion cysts and natal teeth-4.In 1967, according to histogenesis and location in the oral cavity, Fromm classifiThis case report describes the unusual clinical presentation and management of multiple oral cysts in a newborn baby.A Caucasian 60-day-old male newborn baby came to our observation. His parents complained that he had bleeding gums in his lower and upper jaws, pain in sucking and consequent difficulties in feeding. He was full-term born and his weight at birth was 2900g. His past medical history was not significant.No noticeable findings were recorded during an extraoral examination. The intraoral examination revealed multiple whitish small masses localized on the alveolar ridge of his maxilla and mandible. His mandibular alveolar ridge showed small nodular lesions in the region of the right deciduous canine and left deciduous canine and first molar Figure . The sizA surgical removal of the lesions was planned in order to solve feeding problems and bleeding.The excisions were performed in different sessions in order to limit the postoperative discomfort to the baby. All oral surgeries were performed under local anesthesia  with a 15C scalpel blade. After excision of the cysts a sponge of fibrin was used and the wounds were sutured with resorbable polyglactin suture  that was removed after 10 days.The first surgery involved the excision of two lesions on his left mandibular gum Figure .The secoThe obtained specimens were fixed in 10% formalin solution and submitted for histological examination.Periodic recall visits were advised to verify the absence of new lesions and to monitor the developing dentition Figure .All threThe dental lamina cyst, also known as gingival cyst of newborn, is considered a true cyst because it is lined by thin epithelium with a lumen usually filled with desquamated keratin and occasionally inflammatory cells,7. TheseIt is more difficult to elucidate the method of their disappearance. In a study of 32 human fetal heads from 8 to 22 weeks of fetal age, not more than 20 midpalatal raphe cysts were found in any fetus by week 14, and they did not increase in frequency with time. The autet al.[et al.[et al.[As regards the prevalence of oral cysts in newborns, George et al. in a stuet al. reportedet al., in a st[et al. found pa[et al., throughWhereas some authors reported a female predilection,15, otheDonley and Nelson demonstrThe particularity of this case is due to two unusual features that occur in this patient: the presence of symptoms and the different onset time of the lesions. In fact, all the cysts showed spontaneous bleeding and tenderness to palpation. In addition, during the first observation only the mandibular cysts were present; after 30 days the maxillary lesion also appeared.The treatment options have been: no treatment and follow up, marsupialization or surgical extraction. In this case the surgical treatment was mandatory because of the inability of the baby to feed properly.The role of histopathology in establishing the final diagnosis is not essential because the treatment was required by the symptomatology of the newborn; however, it is important to prevent any misdiagnosis, such as hemangioma, melanoma, unicystic ameloblastoma, keratinizing cystic odontogenic tumor and mucocele-18.Inclusion cysts may be associated to natal or neonatal teeth like in this case. When natal or neonatal teeth interfere with feeding, have high mobility, and/or are poorly developed, there is the indication to remove them.The present case of inclusion cysts was successfully managed by the surgical excision of the lesions and the extraction of the neonatal tooth, in order to promote the baby’s natural breastfeeding, without hurting his mother. The parents were well informed about the pathology and its implications. After surgery, both the child and his mother had no complications during feeding; normal eruption of primary teeth occurred by the 6th month. Although for this patient surgical excision of the lesions was the ideal approach, in our opinion the treatment choice must be carefully evaluated on a case-by-case basis after an adequate analysis of clinical findings. Moreover, further studies  on this topic should be performed in order to draw up guidelines for clinicians and oral surgeons, although these cases are quite rare.Written informed consent was obtained from the patient’s parents for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.RM conceived the study and drafted the manuscript, NC and AM participated in its design and coordination, DV performed the histopathological analysis and GLS performed the surgery. All authors read and approved the final manuscript.


Introduction. Arterioportal shunts (APS) are sometimes encountered in patients with hepatocellular carcinoma (HCC) and associated with poor prognosis. The management of HCC with APS is a challenge so far. Case Presentations. We report here in detail a 37-year-old man who was diagnosed as an advanced HCC accompanied with severe APS and treated by two sessions of transcatheter arterial chemoembolization (TACE) and three sessions of transcatheter arterial chemotherapy (TAC) plus sorafenib therapy. The tumor shrinks were revealed continuously during 152 days after the diagnosis. Although tumor progress emerged at 209 days after the diagnosis, the patient remarkably achieved 366-day survival. Discussion. TACE plus sorafenib may be a promising treatment for advanced HCC accompanied with APS. Prospective case-control studies should be advocated to evaluate the combination of TACE, TAC, and sorafenib in the management of HCC with APS. Arterioportal shunts (APS) are sometimes encountered in patients with hepatocellular carcinoma (HCC) and associated with poor prognosis. The management of HCC with APS is a challenge so far. Sorafenib and transcatheter arterial chemoembolization (TACE) combination shows promise as an effective and tolerable treatment strategy for intermediate stage and advanced HCC . To our Written informed consent was obtained from the patient's wife for publication of this report and any accompanying images. A 37-year-old man presented with 2-month duration of intermittent discomfort in the right upper quadrant of the abdomen. His past medical history was inconsequential. His physical examination revealed no abnormality. This patient was referred for an ultrasound (US) examination for further evaluation which revealed a hypoechoic mass in the left lobe of the liver.μmol/L , conjugated bilirubin 5.6 μmol/L , albumin 41 g/L , α-fetoprotein (AFP) 6173 ng/mL , and cancer antigen 19-9 139.9 U/mL . Hepatitis B surface antigen, hepatitis B e-antibody, and hepatitis B core antibody were positive.Initial laboratory results upon presentation included serum alanine aminotransferase 58 U/L , aspartate aminotransferase 41 U/L , total bilirubin 13.7 Transverse unenhanced CT scan demonstrated a low-attenuation mass about 12.5 cm in diameter in the left hepatic lobe. Contrast-enhanced CT images confirmed a hypoattenuating mass with areas of heterogeneous enhancement . Visible3N0M0) HCC using the TNM staging system [According to clinical diagnosis and staging criteria in China , the masTumor shrinks were revealed by hepatic arteriography at 27 days, 84 days, and 151 days after the diagnosis of the tumor . The diaAlthough TACE is a safe and effective treatment for HCC with major portal vein invasion, the reported median survival period was only 6.2 months . SorafenAPS may impact on the management of TACE by the redistribution of arterial flow into portal venous flow in the patient with HCC; however, transcatheter arterial occlusion of APS is technically feasible . AlthougAFP is probably useful in predicting prognosis and treatment response in HCC patients . A studyAPS is considered as a contributor to the induction of portal vein hypertension and upper gastrointestinal bleeding. Although upper gastrointestinal bleeding did not occur in the present case, CT scanning at 335 days after the diagnosis did identify gastric varices that implied portal vein hypertension. Portal vein hypertension as well as potential peritoneal metastasis may cause the ascites of the patient revealed by CT images 213 days after the diagnosis.In summary, the present case reported a large HCC with severe APS treated by TACE, TAC, and sorafenib. The patient achieved 366-day survival and died from liver function failure probably due to portal vein hypertension and tumor invasion of the whole liver. Prospective case-control studies should be advocated to evaluate the combination of TACE, TAC, and sorafenib in the management of HCC with APS.


MRI of the abdomen revealed a large (5 × 5 × 3 cm) peripherally enhancing, heterogeneous mass arising from the left adrenal gland. The patient was treated for a suspected pheochromocytoma. However, his blood pressure was not responsive to alpha‐blockade. Blood pressure was controlled with a calcium channel blocker and an angiotensin‐converting enzyme (ACE) inhibitor. A complete surgical resection of the mass was performed. Postoperatively, his blood pressure normalized and he did not require antihypertensives. On pathological examination, the tumor tissue stained negative for chromogranin and positive for renin. The final diagnosis was renin‐secreting adrenal corticoadenoma, an extremely rare adrenal tumor not previously reported in a pediatric patient. Malignant hypertension due to a renin‐secreting tumor may need to be distinguished from a pheochromocytoma if alpha‐adrenergic blockade is ineffective.A previously healthy 7‐year‐old male presented with hypertensive emergency, hypokalemia, and elevated plasma renin activity and aldosterone levels. There was no evidence of virilization or cushingoid features.  The following case report describes an exceedingly rare tumor in an even more rare age demographic: an ectopic renin‐secreting adrenal adenoma in a 7‐year‐old male child presenting with hypertensive emergency. The rarity of the tumor and age of presentation served as compounding factors that made this case particularly difficult from a diagnostic and treatment standpoint. The family consented to this case report. The differential diagnosis of hypertensive emergency in a pediatric patient with an adrenal mass includes pheochromocytoma, adrenal cortical adenoma, and neuroblastoma, among others.At present, there exists a fine line between ordering the appropriate test and shotgun testing. In this particular case, the correct tests were ordered. The interpretation of the results, however, was difficult because they revealed such a rare diagnosis: ectopic renin secretion.This case report aims to not only describe a physiologically, medically, and surgically interesting and rare tumor, but also to place ectopic renin‐secreting tumors on the differential diagnosis when presenting with elevated plasma renin activity (PRA), low aldosterone, and negative catecholamines. The consequence of this outcome will be to effect rapid treatment of the patient with appropriate angiotensin‐converting enzyme (ACE) inhibition and surgical removal.A previously healthy 7‐year‐old boy presented to an outside hospital with altered mental status and seizures. He had history of weight loss, intermittent blurry vision, polyuria, and polydipsia for 2 weeks. His initial blood pressure was 220/130 mmHg. Computed tomography scan of the brain was normal. His seizures were controlled with lorazepam. He was started on a calcium channel blocker, nicardipine drip, and transferred to Children's Hospital Los Angeles (CHLA) for further management and work‐up of the hypertension.At CHLA, physical examination was significant for tachycardia (136 beats/min) and hypertension (166/96 mmHg). The patient was noted to have a waxing and waning mental status and a hyperdynamic precordium. He had a normal respiratory and abdominal examination. There was no evidence of virilization. An abdominal ultrasound revealed a mass at the superior pole of the left kidney, concerning for an adrenal mass. Serum electrolytes revealed marked hypokalemia (2.1 mEq/L) and normal sodium (138 mEq/L). TSH, T4, and cortisol levels were normal. Further tests of serum and urinary catecholamines and of the renin–angiotensin aldosterone system (RAAS) were obtained to rule out pheochromocytoma as summarized in Table Renin (PRA) and aldosterone levels were significantly elevated. However, markers of sympathetic output were within normal limits with the exception of VMA (vanillylmandelic acid) Table . This unOnce the blood pressure was controlled, the patient was weaned off the nicardipine and started on the ACE inhibitor enalapril and calcium channel blocker amlodipine. Amlodipine was eventually transitioned to the alpha‐blocker phenoxybenzamine due to the elevated VMA level and the concern for pheochromocytoma. The elevated plasma renin activity was postulated to be due to ectopic renin secretion from the tumor, although pheochromocytoma remained highest on the differential. The patient was discharged to home on enalapril 2.5 mg PO BID ( by mouth two times daily )and phenoxybenzamine 2.5 mg PO TID (by mouth three times daily) for blood pressure control, as well as potassium supplementation for hypokalemia. Surgical resection was planned for after 2 weeks of alpha‐blockade.The patient's blood pressure was not well controlled as an outpatient despite good medication compliance. He was admitted twice with headache and hypertension. The dose of phenoxybezamine was increased gradually to 40 mg PO TID and dose of enalapril increased to 7.5 mg PO BID. After controlling his blood pressure as a presurgical inpatient, the patient underwent an open left adrenalectomy with retroperitoneal lymph node sampling. Despite aggressive manipulation of the tumor during the dissection, there was no hypertensive response intraoperatively, nor were additional blood pressure medications required intraoperatively, both suggesting that this lesion was not a pheochromocytoma. The adrenal vein was not sampled intraoperatively.Following surgery, his blood pressure medications were stopped. On postoperative day 2, plasma renin activity and aldosterone levels had normalized to 1.02 ng/mL per h and <1 ng/dL, respectively. Sodium (135 mEq/L) and potassium (3.9 mEq/L) levels also normalized. The patient was discharged home on postoperative day 4. There were no recurrent hypertensive events.Pathologic sections of the tumor demonstrated a well‐circumscribed, well‐vascularized mass with a fibrous capsule. Histologic examination of the tumor showed classic neoplastic characteristics, including mitotic figures and prominent nuclei. Immunohistochemical staining was positive for the calcium‐binding protein calretinin and the gonadal hormone inhibin, and negative for the neuroendocrine secretory protein chromogranin. Staining for the inflammation and tumor marker S100 did not demonstrate the classic sustentacular pattern expected in a pheochromocytoma Lloyd . This paThis patient's presentation and laboratory values did not fit into any of the classic, albeit rare, adrenal tumors. The patient presented with malignant hypertension, hypokalemia, and an aldosterone‐to‐renin ratio (ARR) of 0.53 indicative of secondary hyperaldosteronism, a condition of excessive renin secretion unresponsive to normal negative feedback mechanisms. This is most commonly observed in juxtaglomerular cell tumors and renal artery stenosis, neither of which our patient exhibited. Catecholamines and prostaglandins act through physiologic mechanisms to stimulate aldosterone synthesis (Willenberg et al. β1 activation. This is inconsistent with pheochromocytoma.Laboratory investigations can aid in narrowing the broad differential diagnosis of an adrenal mass with elevated blood pressures. In the absence of familial pheochromocytomas, 24‐h urine fractionated metanephrines is most specific for pheochromocytoma Young  and has While it is known that the juxtaglomerular apparatus of the kidney secretes renin that promotes the systemic effects of angiotensin II, several organs, including the adrenal gland, brain, heart, and blood vessels, contain a regional coincidence of mRNA and protein for renin, angiotensinogen, ACE, and angiotensin receptors (Bader and Ganten The increased plasma renin activity measured in this patient was associated with a high abundance of anti‐renin antibody immunoreactivity in the adrenal tissue Fig. . The antAn extrarenal renin‐secreting tumor in a child is rare. CHLA is a major pediatric referral hospital and has seen over 50 pediatric patients with pheochromocytoma since the late 1960s, all of which have arisen from the adrenal gland. During this time, there has been only one instance of a renin‐secreting tumor in a child, reported in 1979 (Warshaw et al. A case report of a previously healthy 19‐year‐old male describes a similar scenario as our patient (Kawai et al. From 1995 until present, only one other case report describing similar adrenal ectopic renin secretion was found (Yamanaka et al. If a similar scenario were to present itself, controlling the blood pressure with ACE inhibition may result in less hospital days and, more importantly, shield vital organs from dangerously high pressures that can result in irreversible end organ dysfunction. While renin‐secreting adrenal tumors are exceedingly rare, they should be included in initial broad differential diagnosis given applicable clinical situations and laboratory values and the appreciation that there are local RAS systems in adrenal as well as other extrarenal sites. In the setting of malignant hypertension, pheochromocytoma must be considered since adequate blood pressure control is necessary prior to surgical intervention. Intraoperative hypertensive crisis can be life threatening. However, failed blood pressure management with adequate alpha‐blockade should provoke the physician to question the more common diagnosis of pheochromocytoma and consider extrarenal renin‐secreting tumor.None declared.


Tubo-ovarian abscess as a serious complication of pelvic inflammatory disease is very uncommon in sexually inactive girls. We report a case of tubo-ovarian abscess in a 24-year-old sexually inactive girl with transverse vaginal septum who was presented with abdominal pain and a pelvic mass and without prior surgical history and no evidences of appendicitis, inflammatory bowel disease, or cancer. A huge unilateral tubo-ovarian abscess was recognized at laparotomy. Unilateral salpingoophorectomy, hysterectomy and postoperative antibiotic therapy cured the patient.Early diagnosis and treatment are essential to prevent further sequel including infertility, ectopic pregnancy, and chronic pelvic pain which cause morbidity. Pelvic inflammatory disease (PID) is a complex polymicrobial infection. It is thought to arise from ascending of microorganisms from the lower genital tract to the endometrium and fallopian tubes, pelvic peritoneum and adjacent structures, causing inflammation . It is gTubo-ovarian abscess (TOA) is an end-stage process and a serious manifestation of PID that usually reflect an agglutination of pelvic organs  forming a palpable complex . They maTreatment of TOA involves the use of an antibiotic regimen administered in a hospital. About 75% of women with TOA respond to antimicrobial therapy alone. Failure of medical therapy suggests the need for drainage of the abscess . LaparosA 24 year- old blind virgin, with history of mental retardation, primary amenorrhea and normal secondary sexual development, was presented to infectious ward of Imam Khomaini Hospital, Tehran, Iran; with severe right lower quadrant pain, diarrhea and fever since two days ago. Her mother mentioned a history of lower abdominal pain, low-grade fever, and medical therapy in her since a few months ago. In physical examination, external genitalia were grossly normal and hymen was intact, but rectal examination revealed blind vagina of about 1 cm length and an ill-defined right pelvic mass. Abdominal palpation revealed moderately tenderness in lower abdomen particularly in right lower quadrant.Laboratory analysis showed a white blood cell count of 188000/µl, hemoglobin level of 11.5g/dl, ESR=121 and negative blood and stool culture. The chest radiograph was negative. Preoperative transabdominal sonogram and computed tomographic scan of the abdomen and pelvis were done. The sonogram demonstrated a 7×7.5 cm complex mass in right pelvis with irregular borders and multiple septa. Dilation of the right urinary collecting system due to extrinsic compression was noted on sonograghy. Abdomino pelvic CT-Scan confirmed ultra sonographic findings .The patient was placed on intravenous ceftriaxone, but subsequently developed spiking fever after 5 days. On abdominal exam, she developed severe tenderness and rebound. We discussed with her family and emphasized the clinical suspicion of pelvic tumor necrosis and abscess formation or to lesser degree, apandicular abscess and the need for surgical resection.She underwent exploratory laparotomy. The abdomen was free of frank pus and adhesions, but there were severe adhesions in pelvis. After release of adhesions, about 300 cc greenish yellow, pus like foul smelling material was coming from right adnexal swelling into the pelvic cavity. Pus was drained and exploration revealed a10×12cm right-sided pelvic mass with extensive adhesions to the recto-sigmoid. The mass was identified as a right tubo-ovarian abscess, with no evidence of diverticular or appendiceal diseases at surgical inspection. All abdominal organs were found to be normal. Right salpingo-oophorectomy, was performed. During surgery pus was removed from TOA and transported to the laboratory, cultured for both anaerobic and aerobic organisms. Due to her mental retardation, after consent of her mother, the patient underwent total abdominal hysterectomy and then peritoneal lavage was done. The thin lower third vaginal septum also was resected. The culture of pus was positive and mixed. Postoperatively, the patient received ceftriaxone 1g i.v. every 8 hr plus clindamycin 900 mg i.v. every 8 hr. She defervesced gradually and became afebrile after three days. Parenteral antibiotics were replaced with oral antibiotics 72 hours later and recommended for 14 days. Histophatologic analysis showed a right tubo-ovarian abscess with severe inflammatory changes and necrosis with marked neutrophil infiltration. Postoperative recovery was uneventful and the patient was discharged on the fifth postoperative day.Tubo-ovarian abscess is an advanced form of pelvic inflammatory disease, most often caused by spread of bacteria from the lower genital tract . TOA is The infection in TOA is usually polymicrobial with mixed aerobic-anaerobic organisms. The clinical presentation of TOA tends to be non-specific. Vaginal discharge is present in less than 5% of patients. The typical ultra sonographic appearance of a tubo-ovarian abscess is a multilocular, cystic and complex adnexal mass often with debris and thick septations. Hartmann reported two cases with purulent fluid collections in the fallopian tubes that were not evident on imaging, but the laparoscopic evaluation revealed them . Therefore he recommends the use of laparoscopy for diagnosis in atypical cases. Ruptured TOA is associated with a high risk of development of septic shock, in case that urgent surgical intervention is not undertaken . Broad sComplications of tubo-ovarian abscesses include tubal occlusion, increasing the risk of infertility and ectopic pregnancy, on the other hand, pelvic adhesions leading to chronic pelvic pain. Early diagnosis and surgical treatment are essential to prevent further sequela and improving outcome.We must consider TOA in the differential diagnosis for abdominal pain and pelvic mass with fever in adolescent females regardless of sexual history.


The peripheral primitive neuroectodermal tumor (pPNET) is a rare malignant tumor originating from neuroectoderm. The accurate diagnosis is essential for the treatment of pPNET.we performed the largest cases of retrospective analysis thus far to review the unique computed tomography (CT), magnetic resonance imaging (MRI), and clinicopathological features of pPNET. The tumor location, morphological features, signal intensity, contrast enhancement characteristics, and involvement of local soft tissues of 36 pPNETs were assessed.Our results showed that there were more men (25/36) than women pPNETs patients. Unenhanced MRI (16 cases) showed that 14 cases were isointense and 2 cases were hypointense on T1WI. Nine cases were isointense and 7 were hyperintense on T2WI. Most pPNETs had heterogeneous signal intensity with small necrosis  as well as heterogeneous enhancement . The tumors usually had ill-defined borders and irregular shapes . Pathologic exam showed small areas of necrosis in all tumors.The diagnosis of pPNET should be suggested in young men when the imaging depicts a single large ill-defined solid mass with small area of necrosis, especially for those whose images show iso-intense on T1WI and T2WI and have heterogeneous enhancement. The term primitive neuroectodermal tumor (PNET) was coined by Hart and Earle in 1973 . The incIn this study, we performed the retrospective analysis of CT, MRI, and clinicopathological characteristics of 36 pPNETs diagnosed by pathology. Our results suggested that pPNET has unique imaging and clinical features which can be used for the diagnosis.p < 0.05). There were more male patients (25/36) than females (11/36), especially in the bone group . Twenty-nine patients presented with intermittent pain, 4 with cough, 1 as an incidental finding on physical examination and 2 with dizziness and fatigue. Out of 36 patients, 25 (69.4%) had 12 month postoperative follow-up, 12 patients died of which 9 had metastases and 3 had complications, 9 patients recurred of which 3 had metastases and 6 local recurrence, and 4 had no recurrence. pPNET patients arising from organs had a much poorer prognosis than those tumors arising in bone or soft tissues.The clinical findings from the 36 cases are summarized in Table n = 19: limbs 11, vertebrae 4, craniofacial skeleton 4), soft tissues , and organs .Dynamic CT and MRI findings from 36 patients were summarized in Table n = 20) or hypodense (n = 16). Unenhanced MRI (16 cases) showed that 14 cases were isointense and 2 cases were hypointense on T1WI. Nine cases were isointense and 7 were hyperintense on T2WI.  revealed that solid tumors were either isodense  Figure  and liveThe cut surface of pPNETs was gray white and granular. It had significant necrosis and vague boundaries between the tumor edge and normal tissue. Most (33/36) tumor margins contained local invasion with tumor cells. These gross pathological changes were consistent with the appearance on CT and MRI. Tumors were composed of atypical, small round-oval and spindle-shaped cells Figure –8. Many Primitive neuroectodermal tumor (PNET) is a rare malignant tumor originating from neuroectoderm. It consists of primitive undifferentiated small round cells and can be divided into central PNET (cPNET) and peripheral PNET (pPNET) tumors based on the tissue of origin . The radpPNET can be found in all age groups, but, is more common in young children. Khong et al.  and IbarPatients generally presented with rapidly enlarging masses and symptoms related to tumor mass effects . Out of n = 19: limbs 11, vertebrae 4, craniofacial skeleton 4), soft tissue , and organs . pPNETs occurred most frequently in the bones of our patients, which was not consistent with previous reports.pPNET often occur in the thoracopulmonary region (Askin tumor), retroperitonium, and extremities . UncommopPNET were generally large soft tissue masses (>5 cm on average) with ill-defined margins and exhibited aggressive local extension into normal tissue , 20], , , 22]. I. I22]. IpPNET is a locally invasive tumor, the pPNET grew aggressively, showing both an infiltrative and an expansive pattern , 21], , . Our CT pPNET belongs to the family of small round cell tumors, originating from primitive undifferentiated neuroepithelial cells. The pathological features were first reported by Hart  and defiSurgery, radiation therapy and chemotherapy are the primary treatments for pPNET. The patient responses for these treatments were poor and had low survival rate. Patients with pPNET often developed distant metastases and local recurrence within 2–3 years after surgery. Distant metastases most often occurred in the lung, bone, liver, adrenal gland, brain, and retroperitoneum . JurgensIn conclusion, the further diagnosis of pPNET should be suggested in young men when the following criteria was matched: images show a single large ill-defined solid mass with small area of necrosis; scarcely had classification or hemorrhage; locally invasive to adjacent structures, especially for those show iso-intense on T1WI and T2WI, which have significant enhancement.Fifty-three patients with pPNETs diagnosed by pathology were collected between 1 January 2006 and 1 December 2013 at two hospitals. Inclusion criteria: patients with pPNETs underwent a dynamic CT or MRI examination before surgery. Exclusion criteria: recurrent pPNETs and pPNETs treated with radiotherapy or chemotherapy before CT or MRI examination. According to the criteria, 36 patients underwent a dynamic CT examination and of which 16 underwent MRI before surgery. Thirty-three patients underwent tumor resection and 3 underwent tumor biopsy. The institutional review board (IRB) of our hospitals approved this retrospective study and informed consent was waived.2–38 × 38 cm2, and matrix 512 × 512. Iohexol was used as the contrast agent at a dose of 1.5 ml/kg. The injection flow rate was 2.5 ml/s. A 25–30 s after the injection, arterial phase scanning was performed. Venous phase scanning was performed approximately 50 s after injection, and delayed phase scanning was performed 5 min after injection. The raw data were reformatted in the coronal and sagittal planes with a 3 mm section thickness and a 3 mm interval.A Siemens Somatom Sensation multidetector 64-section CT system (Germany) was used for imaging. Settings were as follows: 3 mm section thickness, 5 mm intersection gap, field of view: 25 × 25 cmAll examinations were performed with a Signa HDX 3.0 T MRI scanner using an eightchannel array coil. Settings were as follows: 6 mm section thickness, 1.5 mm intersection gap, field of view 380 × 380, and matrix 288 × 288. A respiratory triggered fast spin-echo (FSE) T1-weighted sequence  and a FSE T2-weighted, fat-suppressed (TSET2) sequence  were used. FSET1-enhanced MRI was performed using Gd-DTPA as the contrast agent. A dose of 0.2 mg/kg was used with an injection flow rate of 1.5 ml/s. Arterial phase imaging was performed approximately 25–30 s after injection, venous phase imaging approximately 50 s after injection, and delayed phase imaging approximately 5 min after injection.The tumor location, morphology, size, margin (well-defined or ill-defined) were measured. The signal intensity, CT density, lesion texture , contrast enhancement characteristics , and involvement of surrounding soft tissue of the 36 tumors were also determined.Thirty-three patients underwent tumor resection and 3 patients underwent biopsy. The histological analysis consisted of hematoxylin and eosin (HE) staining and immunohistochemical (IHC) staining. Neuron specific enolase (NSE), cluster of differentiation 99 (CD99), S100- protein, and vimentin (VIM) expression were examined. All the tumor specimens were reviewed by pathologist. The diagnosis of pPNET was made when there was evidence showing that cells were small round-oval and spindle-shape, which arranged with or without rosettes (Homer-Wright rosettes), and were positive for two or more kinds of neuron-specific antibodies.P < 0.05 was considered to be statistically significant.SPSS18.0. statistical analysis software was used. The age of patients with pPNET in different locations were compared by using one-way ANOVA test.


The finding of gas within the gastric wall is not a disease by itself, rather than a sign of an underlying condition which could be systemic or gastric. We present the case of a woman identified with gastric emphysema secondary to the administration of high doses of steroids, with the purpose of differentiating emphysematous gastritis versus gastric emphysema due to the divergent prognostic implications. Gastric emphysema entails a more benign course, opposed to emphysematous gastritis which often presents as an acute abdomen and carries a worse prognosis. Owing to the lack of established diagnostic criteria, computed tomography is the assessment method of choice. Currently no guidelines are available for the management of this entity, since the evidence is limited to a few case series and a considerable number of single case reports. Radiologic detection of gas within the wall of the gastric chamber is not an entity per se but a sign of an underlying disease . DescribWe present a 44-year-old woman with history of mixed connective tissue disease, who was hospitalized due to exacerbation of her underlying condition for which she was treated with high dose pulses of methylprednisolone. Two days after discharge she arrives back to the emergency room, complaining mainly of abdominal pain located on upper quadrants, not related to food ingestion, associated with vomiting of gastric content in four occasions and one liquid stool free from mucus or blood. At admission with stable vital signs, without acute abdomen or other relevant findings revealed on physical examination, stool specimens, blood analyses, and cultures analyses were requested in search for infectious origin, resulting negative. The plain abdominal X-ray showed a radiolucent image in the left upper quadrant , which wFasting was indicated as well as management with proton bomb inhibitor and broad spectrum antibiotic ampicillin/sulbactam. After twelve hours of close monitoring, the patient persisted nauseous and vomiting, therefore, underwent a liberating mucotomy via superior endoscopy, without complications and minimum bleeding. However, 24 hours later the patient continued presenting abdominal pain and under the suspicion of gastric perforation, due to the presence of right subdiaphragmatic air on a chest X-ray, she was taken to the operating room for an exploratory laparotomy revealing free hematic fluid in the abdominal cavity; methylene blue dye was instilled into the gastric lumen without evidence of dye extravasation, concluding the absence of perforation, considering the procedure complete. Afterwards the evolution was torpid showing signs of rheumatologic activity associated to acute pulmonary deterioration that led to the patient's death due to a possible hemorrhagic alveolitis.The pathogenesis of this disease has been debated for decades; however, it may be approached through the questions, where did the gas come from? And how did it get there? Based on these are how three mechanisms of origin are proposed: (1) intraluminal, (2) bacterial production, and 3) gas of pulmonary origin [ gas of pIntestinal pneumatosis is referred to the presence of gas within the wall of the gastrointestinal tract and it can appear in any site from stomach to the rectum . The stoIt has been postulated that emphysematous gastritis is produced by gas forming bacteria and gastric emphysema by air dissecting the wall , throughWhen the origin of the gas is intraluminal, pneumatosis can occur even alongside an intact mucosa, with intraluminal high pressure accounted as the responsible mechanism. In the context of an injured mucosa, because of trauma or inflammation, a normal intraluminal pressure may be present or a combination of both .In the case gas derives from bacteria, the theory of counterperfusion-supersaturation has been postulated, and it sustains that the production of nitrogen by intraluminal bacteria overflows the plasma concentration producing a plasma-intraluminal gradient and causing a diffusion of nitrogen into submucosal vessels which would explain the gas pattern found through the blood vessels in the border of the mesentery .A pulmonary source has also been debated. The proposed theory is that air travels from an alveolar rupture into the blood vessels up to the gastrointestinal tract. Nevertheless the absence of interstitial emphysema in the mesentery has called this theory into question, giving place to a hypothesis of an increase in intraabdominal pressure hence intraluminal pressure, frequently seen in chronic cough patients causing transmural migration of air .Resuming the etiopathogenesis of this entity, some causes can be mentioned in referral to gastric processes associated to injury of the mucous wall of the stomach. It is well known that acute gastric distension can result in gastric emphysema, emphysematous gastritis, or necrosis. Massive distension causes ischemia with extension of intraluminal gas in to the wall . The ingPneumatosis can also result from a variety of extragastric processes, which favor air migration thru the wall of the colon despite maintaining intact. That being said we can include chronic pulmonary obstructive disease, polymyositis, perforated appendicitis, small bowel volvulus, intestinal infarction, gangrenous cholecystitis, superior mesenteric artery syndrome, cholangiocarcinoma, parastomal hernia, and multiple episodes of vomiting. In relation to drug related background, it has been linked to chemotherapy agents such as cyclophosphamide, adriamycin, and vincristine, in addition to high doses of dexamethasone in one case report , 17–23.Clinical manifestations are usually nonspecific, presenting with nausea, vomit occasionally resistant to antiemetics, mild to severe abdominal pain, abdominal distension, haematemesis, or melena; presentation as an acute abdomen is rare . Most frAmong the image battery available, computed tomography (CT) of the abdomen is the method of choice since it can detect a minimum amount of air inside the wall of the gastrointestinal tract and evaluate the abdominal cavity. Gastric emphysema is presented as a hypodense lineal or curve fringe on the gastric wall along with distension, without evidence of thickening of the wall. Occasionally pneumoperitoneum may be detected, in contrast to emphysematous gastritis where a streaky and linear pattern distribution of air and gastric wall thickening are characteristic, or air in some other bowel or biliary tract can be found , 17, 24.There is no available standard treatment for this condition; most of the reported cases have been treated in a conservative manner. A retrospective study by Morris et al.  includedThe prognosis of gastric emphysema is usually benign with spontaneous resolution even without a specific treatment . More soGastric emphysema and emphysematous gastritis are spectrum of intestinal pneumatosis; both belong to the least common forms of presentation, with different etiopathogenic possibilities , systemic impact, intraabdominal findings, and prognosis and with a distinctive feature of gastric emphysema that in most cases yields more indolent behavior. It is maintained as a misunderstood pathology, in great part since most of the evidence derives from single case reports and series in a significant number of clinical scenarios.The main suspicion in the presented case was an inflammatory cause which was sustained by the presence of hematic ascites, not rare in patients with connective tissue diseases. The implemented measures of endoscopic mucotomy plus coverage by board spectrum antibiotics turned out in a positive impact regarding the gastrointestinal aspect, unlike the rest of the progression of her base rheumatologic disease.


We describe a patient with solitary lymph node (LN) metastasis after three endoscopic mucosal resections (EMRs) in which a gastrointestinal stromal tumor was difficult to differentiate from the carcinoid and lymphoma tumors. A 77-year-old man underwent three EMRs at 62, 72, and 75 years of age, and all resections were determined to be curative. However, 2 years after the last EMR, screening abdominal ultrasonography detected a 20-mm solitary tumor at the lesser curvature of the upper stomach. Laparoscopic tumor resection confirmed the pathological diagnosis. Intraoperative pathological diagnosis showed that the adenocarcinoma was compatible with recurrence of gastric cancer; thus, total gastrectomy with D1 lymphadenectomy was performed. Metastasis was not recognized by pathological examination but was detected by preoperative radiological examinations of the LN. We report a rare recurrence case after several EMRs of intramucosal gastric cancers. In Japan, the Gastric Cancer Treatment Guidelines (GCTGs) ver. 3) define absolute indications for endoscopic resection (ER), which include ≤20-mm intramucosal differentiated cancers without an ulcer. Curativ define aA 62-year-old man was admitted in 1998 because early gastric cancer (EGC) was detected by annual screening endoscopy. There was no specific finding in the physical examination or laboratory data. He had no medical history of malignant tumors. The lesion was a 10-mm type 0-IIc moderately differentiated adenocarcinoma without an ulcer located at the lesser curvature of the antrum Figure a-d. We pet al. reported that piecemeal EMR elevated the risk of local recurrence but not of LN metastasis[Currently, indications for endoscopic treatment of gastric cancer are based on retrospective analysis of the incidence of lymph node metastasis. The GCTtastasis. Becausetastasis–9. Becautastasis. In the tastasis, 11, 12.tastasis. Our casThis case presents a rare case of late LN metastasis after EMR based on the indications for endoscopic treatment of gastric cancer. This case might represent a limitation of ER in evaluating LN status. Because piecemeal resection has a risk of not only local recurrence but also of LN metastasis, en block resection is required, and careful follow-up is mandatory.Written informed consent for publication of this case report and any accompanying images was obtained from the patient. A copy of the written consent is available for review by the Editor-in-Chief of this journal.