Esthesioneuroblastoma (ENB) is a neuroendocrine tumor that typically manifests as advanced stage malignancy in the superior nasal cavity. The hallmark symptoms include nasal obstruction and epistaxis, which result from local tissue invasion. Atypical clinical features can also arise and must be considered when diagnosing and treating ENB. These can include origin in an ectopic location, unusual presenting symptoms, and associated paraneoplastic syndromes. The case described here reports a nasal cavity ENB with atypical clinical features that occurred in a young female. Her tumor was low grade, appeared to arise primarily from the middle nasal cavity, and presented as syndrome of inappropriate antidiuretic hormone (SIADH). She also became pregnant shortly after diagnosis, which had implications on her surgical management. We review the atypical features that uncommonly occur with ENB and the clinical considerations that arise from these unusual characteristics. Esthesioneuroblastoma is a rare nasal malignancy that accounts for 3% of all intranasal tumors . A revieENBs arise from cells of neuroectodermal origin. In the nasal cavity, this type of tissue occurs in olfactory epithelium which can be found in the superior nasal cavity at the area of the cribriform plate, along the superior septum, and at the superior turbinate . The patWe describe a patient with ENB that presented in several atypical ways—low-grade tumor which appeared to originate in the middle nasal cavity, presentation as syndrome of inappropriate antidiuretic hormone (SIADH), and diagnosis during pregnancy. We review the unusual characteristics that may uncommonly occur in ENBs and which must be considered when evaluating patients with this malignancy.A 28-year-old female presented with recurrent episodes of emesis, malaise, and diarrhea over a 3-year time period, from 2004 to 2007. She was admitted twice for hyponatremia, with a nadir serum sodium of 114 mEq/L. Her sodium levels normalized each time with saline administration, and so her hyponatremia was believed to be secondary to gastroenteritis and dehydration. In July 2007, she was seen again for malaise and recurrent emesis and was admitted with a sodium level at 112 mEq/L. Endocrinology was consulted for further workup. Their evaluation was significant for low calcium level (7.8 mg/dL), low serum osmolality (247 mOsm/kg), low urine osmolality (419 mOsm/kg), low phosphorus level (2.9 mg/dL), and low parathyroid hormone level (2.9 pg/mL). A repeat urine osmolality was elevated (1026 mOsm/kg). The remainder of her chemistry panel, urine sodium, cortisol, and thyroid function studies were normal. The endocrinology service diagnosed her with chronic SIADH and placed her on an oral course of demeclocycline to maintain sodium levels. An abdominal CT scan revealed bilateral nephrolithiasis without any evidence of neoplastic processes. A brain MRI with gadolinium demonstrated a heterogeneous mass in the left nasal cavity, with high T2 and low T1 signal, with no intracranial involvement . CT scanThe patient was referred to otolaryngology for evaluation of the nasal mass. Endoscopic evaluation revealed a 2 cm smooth-bordered, polypoid mass at the left ostiomeatal unit which extended superiorly and toward the nasal septum. Outpatient tissue biopsy of this mass was consistent with glial tissue, most likely glioma. This tumor was believed to be the underlying cause of the patient's SIADH, and surgical excision was recommended. During this time, however, the patient also discovered that she was 10-week pregnant. In consultation with the Obstetrics department, the decision was made to perform surgical excision because of the potential risk of recurrent hyponatremia throughout the pregnancy. During second trimester, the patient underwent endoscopic total resection of the neoplasm. The tumor was noted to emanate from the lateral nasal wall and dissected easily from the underlying bone. Although it extended superiorly to the cribriform area, there was no apparent attachment to the superior nasal cavity.Histologic examination of hematoxylin and eosin-stained slides demonstrated a proliferation of small, hyperchromatic cells exhibiting a predominantly nested pattern, in a background of fibrillar material with prominent dystrophic calcifications and a few pseudorosettes . The tumFollowing receipt of the pathology report, a wider surgical resection was recommended to the patient to ensure negative surgical margins given in the malignant diagnosis. The patient elected to wait until after delivery before proceeding with additional surgical management. In the interim, her sodium levels and osmolality normalized. Of note, her hypoparathyroidism and bilateral nephrolithiasis, which were felt to be due to repeated gastroenteritis, resolved as well. Approximately 8 months after her initial surgery, and following the uncomplicated delivery of her pregnancy, the patient underwent wider excision of the left lateral nasal cavity wall. All specimens from this second procedure were negative for residual malignancy. Her case was presented at a multidisciplinary tumor board, and the consensus opinion was that she would not require any adjuvant radiation or chemotherapy. The patient has been followed for 2 years since surgical treatment and has not demonstrated any recurrence of disease. Her sodium levels continue to be normal. This case highlights several atypical manifestations that can occur with esthesioneuroblastomas. First, it is rare for an ENB to originate outside the region of the superior nasal cavity, where olfactory tissue is normally located. This disease is therefore a diagnosis of exclusion in ectopic locations. Several published reports confirm ectopic cases of ENB throughout the nasal cavity and the central nervous system. These include cases with primary origin in the posterior nasal septum , the nasENBs have also been reported to present with unusual signs and symptoms. For instance, some cases have occurred as bilateral noncontiguous tumors . Some tuVery rarely, ENBs present with symptoms that are associated with paraneoplastic syndromes, such as our patient. These syndromes are known to affect up to 8% of all cancer cases, most often in small cell carcinoma of the lung, breast cancer, and gynecologic and hematologic malignancies . Head anAlthough still infrequent, ACTH and ADH are the most common paraneoplastic processes to be associated with ENB. Ectopic ACTH production leads to elevated cortisol levels which usually results in Cushing syndrome. This syndrome consists of characteristic moon facies appearance, buffalo hump, and truncal obesity and can be associated with dysfunction in the cardiovascular, endocrine, neural, gastrointestinal, integumentary, and musculoskeletal systems. It is estimated that between 8% and 18% of total Cushing syndrome cases are due to ectopic ACTH production . Most ofExcess of ADH production leads to a different clinical presentation due to the resultant hyponatremia and hyposmolality. These patients have an associated increase in urine osmolality due to elevated urinary sodium excretion and are usually euvolemic. Inappropriately high levels of ADH are also seen and are typically out of proportion to the hypoosmolality . Other cDevelopment of SIADH does not appear to correlate with a higher cancer grade or stage in ENB cases. Two of the reported cases of SIADH occurred in low grade tumors, and one was mainly located around the middle turbinate, similar to our patient . There hWith most paraneoplastic processes, a diagnosis of cancer is usually made before discovery of ectopic hormone production . In our While most ENBs originate in the superior nasal vault and present with typical symptoms of nasal obstruction and epistaxis in late stages of disease, uncommon manifestations should be considered as well. ENB should be kept in the differential of masses throughout the nasal cavity, paranasal sinuses, and surrounding anatomical locations. A patient with a history of prior ENB can have recurrence in distant locations as well. Paraneoplastic processes, particularly excess ACTH and ADH, should warrant radiographic workup for ENB. Unusual manifestations should be remembered in all cases of  ENB, as the stage and grade of tumor do not seem to demonstrate association with atypical disease.


Reconstruction of open abdominal defects is a clinical problem which general and plastic surgeons have to address in cooperation. We report the case of a 66-year-old man who presented an abdominal dehiscence after multiple laparotomies for a sigmoid-rectal adenocarcinoma that infiltrated into the abdominal wall, subsequently complicated by peritonitis and enteric fistula. A cutaneous dehiscence and an incontinent abdominal wall resulted after the last surgery. The abdominal wall was reconstructed using a biological porcine cross-linked mesh Permacol . Negative Pressure Wound Therapy (NPWT), instead, was used on the mesh in order to reduce wound dimensions, promote granulation tissue formation, and obtain secondary closure of cutaneous dehiscence which was finally achieved with a split-thickness skin graft. Biological mesh behaved like a scaffold for the granulation tissue that was stimulated by the negative pressure. The biological mesh was rapidly integrated in the abdominal wall restoring abdominal wall continence, while the small dehiscence, still present in the central area, was subsequently covered with a split-thickness skin graft. The combination of these different procedures led us to solve this complicated case obtaining complete wound closure after less than 2 months. Abdominal wall defects caused by trauma or surgery are a common surgical problem to solve. Reconstruction of complicated abdominal dehiscence is a challenging condition for both general and plastic surgeons and several techniques have been described.Predisposing factors for lack of skin closure include inadequate local fascial and muscular layers due to prior tissue loss, muscle denervation or vascular insufficiency due to prior irradiation or infection, wound infection, obesity, chronic pulmonary disease, malnutrition, sepsis, anemia, corticosteroid dependency, and/or concurrent malignant process. Biological abdominal mesh is a new possible therapeutic approach in patients with open abdomen that increased mesh abdominal repair indications in patients with abdominal wall defects .In some cases, when abdominal continence has been obtained but a wound dehiscence persists, Negative Pressure Wound Therapy (NPWT) could be a therapeutic option to obtain wound closure . This trWe report a case where we applied NPWT on a biological abdominal mesh in order to reduce wound dimensions over the implant and to prepare wound bed for a split-thickness skin graft which was subsequently used to definitely close wound dehiscence.This study widens the possible application of NPWT confirming its therapeutic role also if applied on biological mesh in complicated patients.We report a case of a 66-year-old man who was diagnosed in 2010 of sigmoid-rectal adenocarcinoma.At the moment of the diagnosis CT scan revealed a bulky mass (8 × 7 cm) in the sigmoid colon with no cleavage plane with the bladder and the abdominal wall and perilesional fat micronodulations. First operation was performed in another hospital. In that occasion the cancer was entirely removed and colostomy in the transverse colon was created. Local resection was followed by chemoimmunotherapy since there were no more signs of progression. exenteratio removing the sigmoid colon en bloc, part of the ileum, the bladder, and the area of the abdominal wall infiltrated by cancer. Abdominal wall was reconstructed with a monofilament abdominal mesh in the lower abdominal quadrants, together with a ureterocutaneous stomy.On December 2011 the patient came to our hospital. The cancer remained focal with no signs of metastasis. CT scan, indeed, showed a voluminous solid mass localised in the sigmoid colon that infiltrated the bladder and the abdominal wall including fascia and muscles up to the subcutaneous tissue. General surgeons proceeded with an anterior pelvicAfterwards, the presence of an enteric fistula caused a retroperitoneal pool and another surgery was needed. A relaparotomy was performed with abdominal mesh removal, enterorrhaphy, and an extended viscerolysis. Abdominal wall was finally closed with a macroporous polypropylene mesh. fossa. Primary closure of the abdomen was not possible to achieve and an open abdomen negative pressure therapy system was applied over the peritoneal organs with a pressure of −125 mmHg in order to reduce edema. Dimensions of cutaneous dehiscence were, at the moment, 16 × 10 cm.Four days later signs and symptoms of peritonitis were diagnosed and the patient underwent a third surgery: the mesh was removed for the second time and another explorative laparotomy was made, followed by drainage of peritoneal cavity, viscerolysis, and creation of a lateral ileostomy in the left iliacThree days later, a further valuation showed an improved clinical presentation of intestinal loops. The abdominal wall was then reconstructed using a biological porcine cross-linked mesh, Permacol , of 20 × 15 cm overlapped and fixed to the fascial edges of the incision using nonabsorbable stitches. Biological mesh was used for its lower recurrence rates if compared with allograft and other biological meshes . An advaPostoperative period was characterized by intestinal motility resumption through the ileostomy and conservation of renal function thanks to the ureterocutaneous stomy; oral alimentation was reintroduced. Patient was then transferred to our department.He presented a 12 × 7 cm abdominal dehiscence which could not be closed neither directly nor with local advancement flaps because of excessive tension on wound edges. Moreover, patient clinical conditions made adoption of myocutaneous regional or distant flaps contraindicated. We subsequently considered NPWT as the best therapeutic option in order to reduce wound dimensions and to obtain secondary closure. For the first period negative pressure was set at −70 mmHg and was renewed twice a week . We deci Pseudomonas Aeruginosa and Corynebacterium species, two of the most common nosocomial pathogens in our experience. The patient was then treated with Ciprofloxacin (500 mg/bid) and Meropenem (1 g/tid). Locally the wound was disinfected with chlorhexidine and treated with a silver antimicrobial barrier dressing combined with NPWT. Infection was controlled and after 10 days of systemic and local therapies we obtained a negative swab for both pathogens.After four days of treatment the wound showed signs of local infection. Swab revealed the presence ofTwo weeks after NPWT application and once local infection has been controlled patient was discharged. At that point he still presented a 6 × 4 cm dehiscence of the abdominal wall. Moreover granulation tissue have been started to cover biological mesh and we decided to increase the negative pressure to −90 mmHg in order to further improve granulation tissue growth and to obtain a total coverage of the biological mesh.Patient was therefore followed on outpatient basis twice a week. Wound dimension progressively decreased and granulation tissue flourished .NPWT was removed after a total therapeutic period of 43 days. During this period negative pressure was set at −70 mmHg for a total of 14 days and −90 mmHg for the remaining period. Our plan was to further increase negative pressure but the good clinical response allowed us to reduce time of NPWT. During all therapeutic period we adopted a polyurethane foam as dressing. Wound dehiscence starting dimensions were of 12 × 7 cm while, at the end of treatment, the final dimension was 3 × 5 cm .After NPWT removal wound bed was medicated with alginate and silver sulfadiazine for the following 14 days twice a week in order to prepare it for the final reconstructive step.When biological mesh was completely overlaid with granulation tissue, the remaining loss of tissue in the lower abdominal area was closed with a split-thickness skin graft taken from the left thigh. The graft takes roots perfectly allowing total closure of abdominal dehiscence after 55 days from patient transferral to our department. No recurrence and local complications occurred with a postoperative follow-up of 6 months.Colon cancer is the second most frequent neoplasm in the Italian population. However colon carcinoma very rarely presents with subcutaneous, retroperitoneal, or abdominal wall abscess. Abscess of the anterior abdominal wall developing as a result of direct invasion and perforation of the colon by cancer has been rarely described –7. Our pMany techniques have been described to reconstruct the abdomen wall but, if complications and reoperation occurs, options for deep abdominal layers reconstruction became progressively fewer. They include direct tissue closure, prosthetic mesh, local advancement or regional flaps, distant flaps, or combined flap and mesh. In particular regional flaps are the rectus abdominis flap and the external oblique flap while distant flaps options include the latissimus dorsi, tensor fascia lata (TFL), and rectus femoris muscles ., intra-abdominal adhesion, and fistula formation [Porcine acellular dermal collagen implants are made of a biologic material derived from processing porcine dermis cross-linked with diisocyanate . Some auormation . As theyBiological mesh is a new frontier in treating complicated open abdomen wounds and clinical evidences are progressively increasing in clinical practice.On the other hand, NPWT has already been shown to heal complicated abdominal dehiscence.It works in open abdomen providing mechanical containment of abdominal viscera, third space fluid loss estimation, and prevention of intestinal fistula and infection while in abdominal dehiscence it stimulates granulation tissue growth, removal of exudates, and promotion of neoangiogenesis.To improve infection control, if clinical condition requires, silver dressing could be added to both polyurethane foam and dressing gauze as in our clinical report.In our case, we decided to adopt NPWT in a patient who presented an abdominal dehiscence in order to reduce time of hospitalization and to obtain total abdominal wall closure.NPWT could be managed on outpatient basis; in this way time of hospitalization was reduced together with the nosocomial infection possibility, decreasing costs and improving the tolerability of the procedure.The association of NPWT and biological mesh implant prevented us to adopt other reconstructive procedures as regional or distant flap which could be much more invasive in our complicated patient.In our experience the biological mesh behaved like a scaffold for granulation tissue which growth was stimulated by negative pressure therapy allowing a final closure with a split-thickness graft. The two procedures seem to combine perfectly together.The mesh was rapidly integrated within the abdominal wall, restoring abdominal wall continence. Our clinical case shows that NPWT use is not contraindicated if a biological mesh is present and exposed; on the contrary NPWT could reduce hospitalization time with a decreased rate of complications.


Mesenteric venous thrombophlebitis represents a very rare complication of acute appendicitis. Based on the findings of a 45-year-old patient with mesenteric venous thrombophlebitis due to acute appendicitis, we herein describe the diagnostic difficulties and therapeutic options in this uncommon disease. The treatment in our case consisted of simple appendectomy and perioperative anticoagulation therapy. Mesenteric vein thrombophlebitis may be induced by hypercoagulable, malignant, or septic conditions. Although its incidence due to septic conditions is nowadays pretty low, the related mortality, especially by late diagnosis, is higher as 30% . Abdomin3). Abdominal computed tomography (CT) revealed the presence of an acute appendicitis with acute mesenteric vein thrombosis  were within normal laboratory range, with the exception of elevated white blood cells count WBC = 14.000/mm4.000/mm3 Figures . Particu Figures  showed i Figures , withoutWith these findings and prior to operation, the volume flow rate of blood in the portal/superior mesenteric vein was assessed using a duplex ultrasound system.The patient was treated with emergent open appendectomy and received broad spectrum antibiotics (cefuroxime plus metronidazole). A low-molecular-weight heparin was perioperatively administered, while the postoperative course was uneventful.The followup included a monthly performed duplex ultrasound, which never revealed thrombus expansion into portal or splenic vein. Furthermore, no splenomegaly or cavernosus transformation of the portal vein was observed. A remarkable thrombus shrinkage was detected during his last examination. The patient remained asymptomatic during the six-month follow-up period.Every septic condition that drains into the portal system can lead to a mesenteric thrombophlebitis or pylephlebitis, which is defined as thrombophlebitis of the portal vein or its tributary veins. Beside appendicitis, colonic diverticulitis, inflammatory bowel diseases, bowel perforation, pancreatitis or cholangitis are potential triggers . FurtherHigh index of clinical suspicion and imaging play a crucial role in the diagnosis of mesenteric venous thrombophlebitis, as the condition is usually misdiagnosed intraoperatively. CT scan is the most reliable diagnostic tool , being aThe current treatment approach is based on aggressive broad-spectrum antibiotic administration (blood cultures are strongly indicated), with immediate surgical removal or drainage of the septic focus (in the present case appendectomy) and anticoagulation therapy. Anticoagulants are administered in an effort to recanalise the thrombotic vein and reduce the possibility of septic embolisation and abscess formation in the liver . In caseConcluding, mesenteric vein thrombophlebitis is a rare clinical entity, which may have high morbidity and mortality, especially by misdiagnosis. In most of the cases, as in our report, the conservative approach is actually adequate, given the successful drainage or surgical removal of the septic focus.


This report describes the case of a 6-month-old girl with a large cardiac fibroma in the right ventricle. Ventricular tachycardia associated with the fibroma was successfully treated with amiodarone. At the age of 3 years, surgical resection was indicated because of right ventricular outflow tract obstruction caused by progression of the tumor. The fibroma was successfully resected, and further follow-up evaluation was uneventful. Primary cardiac tumors in children are rare. The incidence is reported to be 0.03–0.32%, with fibroma accounting for approximately 25 % [In a 6-month-old girl, a large cardiac tumor in the RV was discovered during evaluation for a soft systolic murmur. Except for the soft systolic heart murmur, the overall pediatric examination was normal. Echocardiography showed a large (4.0 × 4.5 × 4.0 cm) homogeneous mass appended to the RV free wall Fig. . There wAt the age of 3 years, surgery was clinically indicated because tumor progression had resulted in obstruction of the RV outflow tract. The tumor was completely resected, and the resection margins were plicated (Fig. The girl’s postoperative course was uneventful. Because arrhythmia did not occur during a 6-month follow-up period, the amiodarone treatment was discontinued. Long-term follow-up evaluation was without complications and showed normal cardiac function without recurrence of the tumor.Although fibromas are histologically benign, they can induce serious cardiovascular complications, including intracavitary obstruction and rhythm disturbances, as illustrated by the reported case and described in the literature . AlthougFibromas are not likely to decrease , and bec


Osseous metaplasia in the gastrointestinal tract is a rare phenomenon.We present the case of a 62-year-old Hispanic man with two colonic polypoid lesions, one of which, upon resection and histopathological examination, was found to be a traditional serrated adenoma with a focus of stromal osseous metaplasia.Our patient’s case is the third report of stromal osseous metaplasia in a traditional serrated adenoma of the sigmoid colon. Epithelial metaplasia is a common occurrence in the gastrointestinal tract (GIT), especially in the upper GIT (excluding the liver and pancreas). However, mesenchymal metaplasia, particularly osseous metaplasia (OM), is a rare and incidental finding in the GIT and is composed of histologically benign bone tissue -3. FewerIn 1939, Dukes described four cases of OM in colonic adenocarcinomas; since then, it has been described in both benign and malignant GIT lesions. The rectal region is particularly affected (adenocarcinoma), although the spectrum of presentation includes Barrett’s esophagus; tubular, tubulovillous and serrated adenomas; juvenile and hyperplastic polyps; and appendicular tumors  of colonic mucosa (sigmoid) with exuberant, villiform growth pattern and complex serration. High power examination disclosed eosinophilic pencillate cells, numerous ectopic crypts and low-grade epithelial dysplasia. Figures  and 2 ThMesenchymal metaplasia, particularly osseous metaplasia, is an unusual finding in GIT, composed of histologically benign bone tissue. This is an unexpected variation of the usual histological features encountered in colorectal TSA. In 1939, Dukes described four cases of OM in colonic adenocarcinoma; since then, it has been described in both benign and malignant GIT lesions, but the rectal region is particularly affected .Despite the absence of a conclusive explanation of the induction mechanism of ossification, the hypothesis proposed by Rhone and Horowitz in 1976 postulated the metaplasia of a pluripotent cell into an osteoblast under the influence of factors generated by epithelial cells. One possible mechanism includes the production of bone morphogenetic proteins (BMP)-5 and BMP-6 by epithelial cells and BMP-2 and BMP-4 by adjacent fibroblasts ,13,15,16An extensive review of the literature reveals that OM is mostly described in association with colorectal carcinomas and adenomas (both tubular and villous); however, no risk association has been determined for sex, age or polyp size ,8,9,11. According to our bibliographic review, our patient’s case is only the third report of osseous metaplasia in the stroma of a traditional serrated adenoma located in the sigmoid colon. No characteristic has been identified that would suggest a traumatic or ischemic process, nor any foci of calcification, either proximal or distal.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NM performed the histological examination and diagnosis of the colonic polyp. JB and LR conducted a thorough literature review on osseous metaplasia in the gastrointestinal tract and were the major contributors in writing the manuscript. All authors read and approved the final manuscript.


Pulmonary carcinosarcoma is a biphasic tumour with an unfavourable prognosis. The differential diagnosis includes pulmonary blastoma and is often challenging.KRAS, EGFR, and beta-catenin revealed no mutations. Comparative genomic hybridization detected +1q, +6p, +6q24qter, +8q, +11q12q14, +11q23qter, +12q12q21, +12q24qter, +17q, +20q, -5q14q23, -9p13pter, -13q21q21, and amplifications at 12q14q21, 15q24qter, 20q11q12.We here describe a case of blastomatoid pulmonary carcinosarcoma in a 58-year-old patient, who underwent surgical resection. Histopathological examination revealed immature glandular epithelium resembling high-grade fetal adenocarcinoma expressing epithelial markers and membranous beta-catenin, and blastomatoid spindle cells with partial rhabdomyosarcoma-like differentiation. Both elements expressed p53, MDM2, and cyclin-dependent kinase 4 (CDK4), but not thyroid-transcription factor 1 (TTF-1). Mutation analysis of TP53, MDM2, CDK4, and PTPN1 may be involved in tumourigenesis.The observed molecular and cytogenetic findings may provide additional tools for the differential diagnosis of biphasic pulmonary neoplasms. Furthermore,  The differential diagnosis of biphasic pulmonary tumours consisting of a mesenchymal component combined with adenocarcinoma includes pulmonary blastoma and carcinosarcoma -3. The dA 58-year-old male patient was admitted to hospital after suffering for several weeks from shoulder pain, dyspnoea, and cough. His medical history included chronic obstructive pulmonary disease and smoking (80 pack years). Computed tomography (CT) detected a 12.9-cm sharply marginated mass lesion in the upper right hemithorax with central necroses and marked FDG-glucose uptake in positron emission tomography with integrated computed tomography , pan-cytokeratin (Dako), carcinoembryonic antigen , CK7 (Dako), and focal p63 . Thyroid transcription factor 1  was not expressed. Staining for beta-catenin  demonstrated a membranous expression in the epithelial structures. The spindle cell component expressed vimentin (Dako), desmin  in 10% of tumour cells, and myogenin (Dako) in 10% of tumour cells indicating partial rhabdomyosarcoma-like differentiation, EGFR (Invitrogen), and CD56 (Invitrogen). Both components expressed p53 (Dako), MDM2 (Zytomed Systems), and cyclin-dependent kinase 4 . Proliferative activity was assessed by Ki67 (Zytomed Systems) immunostaining and was estimated at 60%.SYT-SSX fusion gene suggestive of synovial sarcoma was not detected by RT-PCR. Sequencing analysis of KRAS exon 1 and 2, EGFR exon 19, 20, and 21, and beta-catenin exon 3 showed no mutations. CGH analysis was performed as described previously [MDM2.A eviously  and reveThe diagnosis of the blastomatoid variant of pulmonary carcinosarcoma was established and the tumour was finally staged at pT3, pN0 (0/15), pMX, G3, R0, UICC stage 2b. The patient recovered well and was discharged 21 days after surgery. An optional adjuvant therapy was discussed with the patient, but he refused. Currently the patient is doing well and there is no evidence of tumour relapse 30 months after the resection.SYT-SSX fusion gene was not detected [The differential diagnoses of biphasic pleuropulmonary tumours in adults include glandular malignant peripheral nerve sheath tumour (MPNST), synovial sarcoma, and malignant mesothelioma ,3,5. Whedetected . MalignaFurthermore, pulmonary blastoma should be considered in the differential diagnosis if the epithelial component consists of adenocarcinoma . As repoIn the case presented here, the gross appearance and localization of the tumour were not helpful in the differential diagnosis, since both pulmonary blastoma and carcinosarcoma are known to arise either central/peribronchial or in the periphery of the lung, forming a large bulk ,8. PulmoThe epithelial component in the present case showed focal cellular atypia, mitotic figures, and branching glands composed of columnar cells with palisading elliptic nuclei with subnuclear vacuoles resembling high-grade fetal adenocarcinoma as described for carcinosarcoma . In contTP53, but not of KRAS or beta-catenin[EGFR, MDM2 or CDK4 have not yet been investigated in this entity. Cytogenetic aberrations reported for this entity include allelic gains at 1q, 3q, 5p, 8q, 12p, and losses at 3q, 5q, 17p [TP53, MDM2, and beta-catenin mutations, whereas EGFR and KRAS mutations are usually not detected [Previous immunohistochemical and molecular analyses in pulmonary carcinosarcoma revealed mutations of a-catenin,2. To oudetected ,12,13. Fdetected ,12.EGFR, KRAS or beta-catenin mutation were found. Additionally, we observed a high number of chromosomal aberrations by CGH including gains at 1q, 6p, 6q24qter, 8q, 11q12q14, 11q23qter, 12q12q21, 12q24qter, 17q, 20q, losses at 5q14q23, 9p13pter, 13q21q21, and amplifications at 12q14q21, 15q24qter, 20q11q12. Interestingly, the observed gains at 1q, 8q, and losses at 5q are among the aberrations described for pulmonary carcinosarcoma [Immunohistochemically, an expression of p53 was detected in the present case consistent with an underlying TP53 mutation, but no MDM2 and CDK4 were confirmed immunohistochemically and by CISH analysis in the present case and may play a role in tumourigenesis [PTPN1 gene located at 20q12 which serves as a non-receptor tyrosine phosphatase involved in growth regulation [The observed chromosomal changes may give insight into tumourigenesis and help to identify possible candidate genes. The amplicon 12q13q21 (including the observed 12q14q21) is also typically detected in different types of sarcoma, particularly in liposarcoma and osteosarcoma . It harbigenesis ,16. Overigenesis . FurtherAs reported, complete surgical resection is the treatment of choice in patients with resectable tumours . ChemothMDM2 and lack EGFR mutations. Therefore, presence of TP53, MDM2, and lack of KRAS and EGFR mutations may not be helpful in the differential diagnosis of both entities. Only the presence or absence of beta-catenin mutations will serve as a useful diagnostic tool in certain cases. In the present case, the diagnosis was discussed with reference pathologists who favoured the diagnoses of either pulmonary blastoma or blastomatoid carcinosarcoma. Although high-grade fetal adenocarcinoma was not present in every tumour sample, the diagnosis of pulmonary carcinosarcoma was finally made based on the mutation analyses, particularly of beta-catenin.The present case illustrates the diagnostic difficulties in differentiating pulmonary carcinosarcoma from biphasic pulmonary blastoma, in particular if the carcinosarcoma contains fetal adenocarcinoma and a blastomatoid mesenchymal component. It also highlights the usefulness of additional molecular and genomic analyses. From our findings we conclude that similar to pulmonary blastoma pulmonary carcinosarcoma may harbour beta-catenin mutation, and detection of 1q, +8q, and -5q by CGH in a biphasic lung tumour in adult patients favours the diagnosis of pulmonary carcinosarcoma. Furthermore, mutations of TP53, MDM2, CDK4, and PTPN1 may play a role in development and progression of this tumour entity.In conclusion, the presence of high-grade fetal adenocarcinoma without TTF-1 expression, lack of The authors declare that they have no competing interests.IMS and SS performed the histopathological, immunohistochemical and genetic examinations and established the diagnosis. COS, TO, and JM examined, treated and observed the patient, including follow-up. IMS, COS, TO, SS, and JM participated in writing the manuscript. COS and JM provided the radiographic, and IMS the histological and CGH images. All authors read and approved of the final manuscript.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 Series Editor of this journal.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/12/424/prepub


Mature cystic teratoma of the ovary rarely undergoes malignant transformation. There is no consensus for a treatment modality because of the rarity of the disease. Herein we present a case of squamous cell carcinoma (SCC) arising in a mature cystic teratoma (MCT) in a 66-year-old patient. The patient underwent total hysterectomy and bilateral salpingo-oophorectomy, omentectomy, appendectomy, and bilateral pelvic + paraaortic lymph node dissection. The histopathological examination revealed malignant invasion of the appendix and uterus. The patient, who refused the continuation of treatment initiated with the administration of a single dose of cisplatin, died 5 months later because of the disease. It is imperative that gynecologists consider appendectomy in SCC arising from MCT cases. Mature cystic teratomas (MCTs) of the ovary, which are a type of germ cell tumors, account for 20%–25 % of all ovarian neoplasms. They are the most common benign germ cell tumors of the ovary in younger women (<45 years). They may be composed of mature or immature tissues deriving from the 3 germ cell layers .Most patients with MCTs are asymptomatic but mass compression effect can lead to pain and abdominal distension. The complications of MCT are rupture, torsion, and malignant transformation .Malignant transformation is very rare in MCT's components 2%). Squamous cell carcinoma (SCC) arising from the ectoderm is the most common type (approximately 80%), which is followed by adenocarcinoma and carcinoids %. Squamo.Herein we present a case of SCC arising in an MCT with appendix metastasis and uterus infiltration in a 66-year-old woman and review the published literature to determine possible risk/prognostic factors and treatments.A 66-year-old multipara patient was referred to our clinic with abdominopelvic mass and abdominal pain. Pelvic examination revealed a large immobile pelvic mass with regular contours extending up to the umbilicus. Ultrasound and computed tomography imaging showed a 20 × 18 cm cystic mass with solid components and calcifications in the left adnexal region extending up to umbilical area with severe ascites. The uterus and right ovarium were normal. The patient's tumor marker profile was as follows: CA-125: 33.1 U/mL , CA 19-9: 10.54 U/mL , and β-HcG was negative. The Pap smear being negative for malignancy, and endometrial biopsy revealed atrophy. The patient underwent exploratory laparotomy for ovarian malignancy. 1500 mL serous ascites was noted and used for cytology. The pelvic mass at this time was identified as arising from the left ovary. The posterior surface densely adhered to the pelvic sidewall. The tumor also had massive adhesions to the bladder from its anterior. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Analysis of a frozen section revealed SCC arising within an MCT. Based on this finding, omentectomy, appendectomy, and bilateral pelvic + paraaortic lymph node dissection were performed. There was no evidence of gross residual tumor.Gross examination of the resulting specimen revealed a solid-cystic mass measured 20 × 15 × 4 cm with a nonintact capsule . The cysThe histopathological examination revealed a moderately differentiated SCC arising in an MCT of the ovary. The tumor was composed of solid islands and cords of atypical squamous cells, and in a different side of the cystic wall, teratoma components were seen as a stratified squamous epithelium with adnexal structures . In situThe surgical stage of the case was classified as FIGO stage III. A taxol and carboplatin chemotherapy protocol was planned for the patient. However, the patient refused the planned treatment after the first administration of chemotherapy, and she died five months after the operation.SCC arising from MCT is a rare pathologic event. Although preoperative diagnosis of MCT is relatively easy, diagnosis of malign transformation is very difficult. Regardless of underlying etiology, SCC of the ovary does not seem to have any pathognomonic features. Malignant transformation is currently diagnosed only by postoperative histopathologic examination in most cases . In our Patient age, tumor size, serum tumor markers, imaging characteristics, and stage constitute risk factors for malignancy arising from MCT . As somePrognostic factors for SCC include capsular invasion/rupture, tumor dissemination, ascites, abdominalpelvic adhesions and tumor types . The groSurgical cytoreduction with proper staging, adjuvant therapy with platinum-based or paclitaxel-based chemotherapy, and concurrent whole pelvic radiation have been recommended as methods of treatment in advanced stage and recurrent disease of SCC arising from MCT . FertiliIf squamous cell lesions of the ovary are detected in a patient, the differential diagnosis should include SCC arising from MCT and metastatic carcinomas. The primary focus of metastasis of squamous cell carcinoma to the ovary is often from uterus and vagina.Metastasis of the appendix accounts for 4%-5% in early stage cases of epithelial ovarian tumors. Ayhan et al. reported appendix metastasis in 100 of 285 patients with ovarian cancers, and they recommended appendectomy in all ovarian carcinomas for best cytoreduction and surgical staging . AppendeThe prognosis is poor in SCC arising from MCT. However, early stage of the disease and performing an optimal cytoreductive surgery are good prognostic factors .In conclusion, although malign transformation arising from MCT is extremely rare, clinicians must be aware of this risk before surgery. Because surgical procedure is highly different between benign teratomas and malign transformation arising from MCTs, physicians should consider appendectomy for SCC arising from MCT cases.


Primary appendiceal mucinous adenocarcinoma is a rare entity, while appendiceal carcinoma invading the urinary bladder is even rarer. The current study presents the case of a 54-year-old male with primary appendiceal mucinous adenocarcinoma, without gastrointestinal symptoms, presenting only with signs of bladder irritation. Abdominal computed tomography scans demonstrated a mass at the right anterior level of the bladder. During intraoperative exploration, the mass was detected to connect and adhere to the ileocecal junction, and normal appendix tissues were not found. The frozen section revealed a mucinous adenocarcinoma of the appendix and the patient immediately underwent a subsequent hemicolectomy. The present case indicates that a diagnosis of a primary appendiceal mucinous carcinoma must considered by radiologists and clinicians for patients who do not exhibit gastrointestinal symptoms, but show involvement of the nearest organs and the bladder wall. Primary adenocarcinoma of the appendix is a rare malignancy with ~0.12 cases per 1,000,000 individuals diagnosed annually –5. The mA 54-year-old previously healthy male presented at the Shandong Provincial Qianfoshan Hospital Affiliated to Shandong University  with the clinical presentation of continuous frequent micturition, urgent urination and odynuria for approximately six months. The patient did not have a fever or any gastrointestinal symptoms. A physical examination was normal and laboratory tests showed microscopic, but no gross, hematuria. The level of tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, were normal. A previous ultrasound at the patient’s local hospital showed a bladder tumor . The enhanced abdomen computed tomography (CT) scan revealed a hypodense mass (5.4×4.6 cm at the maximum section) at the level of the right anterior bladder wall, with sporadic calcifications in the peripheral area. The mass was moderately and heterogeneously enhanced in postcontrast images . From thDuring the surgery, a mass with a maximum diameter of 5 cm was identified at the level of the right anterior bladder wall, markedly adhesive to the surrounding tissues. Following dissection of the peritoneum, the mass was detected to connect and adhere to the ileocecal junction, and normal appendix tissues were not found. Following consultation, gastrointestinal surgery experts considered that the tumor was of appendiceal origin, invading the urinary bladder. The frozen section revealed a mucinous adenocarcinoma of the appendix, and the patient immediately underwent a subsequent hemicolectomy. The final histological examination confirmed the presence of a primary appendiceal mucinous adenocarcinoma infiltrating the appendix and urinary bladder wall. The pathological staging was as follows: TNM classification, pT4N0M0; and Dukes’, Astler-Coller classification, stage B.et al (et al (Histologically, adenocarcinomas of the appendix are classified into three distinct types: Mucinous, intestinal and signet ring cell. Various controversies in the field of appendiceal mucinous neoplasms have arisen over the past several decades, focusing on the classification of appendiceal mucinous tumors and the nature, classification and clinical significance of pseudomyxoma peritonei . Invasivet al . The preet al ,14. The l (et al  describeIn the present case, the patient did not exhibit any gastrointestinal symptoms, with signs of bladder irritation, existing since morbidity, the only clinical presentation. Ultrasound and abdomen enhanced CT scans revealed a urinary bladder mass. Combined with the laboratory test results and symptoms, an initial diagnosis of urinary bladder carcinoma was made. However, from the CT MPR images, the mass was found to connect with the ileocecal junction, and the density of the liquid content was low. The region of the lesion was in line with the appendix. Therefore, the primary diagnosis was changed. It was considered that the lesion had originated from the intestinal tract, then invaded the bladder, and that the appendix was the most likely organ of this origin. This was confirmed by explorative surgery, followed by a subsequent hemicolectomy due to the frozen section analysis.In the present case, the patient’s clinical presentation was confusing and the rarity of an appendiceal carcinoma invading the urinary bladder led us to initially ignore the possibility of its presence. The signs and symptoms of an appendiceal adenocarcinoma were not specifically apparent, but the CT MPR technique was useful to identify a correlation with the appendix. Therefore, a diagnosis of a primary appendiceal mucinous carcinoma must be considered by radiologists and clinicians for patients who do not exhibit gastrointestinal symptoms, but show involvement of the nearest organs and the bladder wall. It is possible that CT MPR images may provide more information.


Salivary mucocele is an accumulation of saliva in a single or multiloculated cavity lined by connective tissue that is contiguous to a salivary gland-duct complex and is the most common condition affecting the salivary glands in dogs. Occasionally, different types of metaplastic lesions, such as squamous and osseous metaplasia - which are rare lesions in animals - can be observed in association with salivary mucocele.A right facial enlargement was suddenly observed in a 4-year-old non-spayed female Shih-Tzu dog. The lesion presented itself as a soft and fluctuant mass located in the right side of the face near to the neck. Histologically, the mass consisted of a cavitary formation without an epithelial lining. Additionally, microscopic examination revealed the presence of osteoid-producing cells which gave rise to areas of bone formation, probably induced by irritation due to the presence sialoliths. Such cells and bone formations were also present in the cavity wall, consequently leading us to classify the condition as a salivary mucocele with osseous metaplasia.In the present case, the pathogenesis was probably associated with the presence of sialoliths, which can behave as etiological agents for the metaplastic lesion. The occurrence of osteoid metaplasia is a rare peculiar condition in the canine salivar y gland, and due to the rarity and lack of information about this specific disease, no clinical data can yet be associated with the development of salivary mucocele with osseous metaplasia in dogs. Salivary gland disorders are uncommon diseases of dogs, with an overall reported incidence of less than 0.3 percent . SalivarA 4- year-old, non-spayed female Shih-Tzu dog was admitted to the School of Veterinar y Medicine and Animal Science from the São Paulo State University - UNESP, Botucatu, Brazil, for a soft and fluctuant non-ulcerated right facial swelling measuring 10 × 10 × 2 cm. The lesion was located in the neck near to the mandibular gland. Hematological and serum biochemistry findings revealed no alterations in blood urea nitrogen, creatinine and total protein concentrations. However, hyperalbuminemia and hypoglobulinemia were detected. An excisional biopsy with salivary gland resection was performed and the excised mass was submitted to the Veterinary Pathology Service of the same institution for histopathological examination. Grossly, the excised mass was cystic and thin-walled. Additionally, the presence of sialoliths was detected on gross examination. Fragments were formalin-fixed, routinely processed, and paraffin- embedded. Subsequently, 4 μm thick sections were obtained and stained with hematoxylin and eosin for histopathological examination. Histologically, the mass consisted of a cavitary formation without epithelial lining, leading us to diagnose the lesion as a salivary mucocele. Additionally, sialoliths located inside the cyst were observed on microscopic examination Figure , consequSalivary mucoceles are uncommon salivary gland disorders in dogs that do not have breed or sex predilection . HoweverIn the present case, the pathogenesis was probably associated with the presence of sialoliths, which can behave as etiological agents for the metaplastic lesion due to trauma and inflammation. Histological analysis allowed us to classify the process as occurrence of granulation tissue associated with an osteoid metaplastic lesion in a salivary mucocele, consequently characterizing an ectopic ossification in this case.Written informed consent was obtained from the patient's owner 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.TRF performed the analysis and interpretation of cytologic and histologic findings, photographed images and helped to write the manuscript. FG, LNM, BSS participated in clinical research, helped to write the manuscript and has contributed to the analysis and interpretation of cytology and histology and for the preparation of the manuscript. RMR conducted clinical research and was responsible for collecting samples. NSR is the supervisor responsible for the case report and the review of the manuscript. All authors read and approved the final manuscript.


Giant cell tumor of the sacrum, especially involving the sacroiliac joint, is rare, but is particularly challenging to treat. The long term outcome of a patient was studied with giant cell tumor involving the sacroiliac joint treated with selective arterial embolization and curretage.One patient with giant cell tumor involving the sacroiliac joint was treated with selective arterial embolization and curettage in our hospital in October 2002. The curettage and bone grafting was done after two times of selective arterial embolization;1600 ml of blood were transfused and no complications developed during the operation.At the final follow-up of 9 years after the operation, no local recurrence and metastasis developed and she retained normal activity in daily life.We think it is an optimal treatment for giant cell tumor involving the sacroiliac joint, with repeated selective arterial embolization and curettage, which has the advantage of less injury, less blood loss and fewer complications. Giant cell tumor (GCT) is a locally aggressive, benign bone tumor with a high risk of local recurrence and a low risk of metastasis after treatment. Giant cell tumor of the sacrum, especially involving the sacroiliac joint, is rare, but is particularly challenging to treat since the tumor is frequently diagnosed late, and is often quite extensive within the bone and surrounds the sacral nerve roots. The sacral canal can accommodate large, slowly growing GCTs that become symptomatic only when they become large enough to compress adjacent nerves or pelvic organs. Patients often present with nonspecific low back pain. Treatment of GCT involving the sacroiliac joint is not straightforward. Excision of the affected sacral and iliac bone almost always results in loss of function of the sacral nerve roots with incontinence and lumbopelvic discontinuity. Resection of a considerable portion of the sacrum has a high incidence of neurological complications, which may affect bowel and bladder control and may lead to impotence in men. CurettaIn this manuscript, we report the long-term clinical outcome of a case with GCT involving the sacroiliac joint that was successfully managed by twice performing RSAE and curettage, and bone grafting. We stress the effectiveness of the procedure as being a less invasive and less complicated primary treatment for GCT of the sacrum and ilium. Informed consent was given before the operation and the patient was informed that data concerning the case would be submitted for publication.A 31-year-old woman presented with severe pain in her left lower back and buttock, which severely restricted her gait. Radiography and computerized tomography (CT) revealed an eccentric geographic destructive osteolytic lesion involving the sacrum and the posterior superior iliac spine Figure . IntraleAfter completion of the second embolization, the patient’s buttock pain improved significantly. Her gait became normal. Radiography demonstrated many iodized oil shadows in the osteolytic lesion involving the sacrum and ilium Figure . Then shGCT involving the sacrum and ilium presents a significant challenge and treatment methods, including surgical resection and radiation, and is associated with morbid complications and high recurrence rates,6. Partiet al.[et al.[Postoperative adjuvant therapy has a limited place in the treatment of GCT. Radiotherapy has been abandoned, since limited therapeutic benefit is combined with a risk of malignant change. Embolizet al. reported[et al. reportedet al.[Previous reports demonstrated that a large population of patients responded favorably to intra-arterial embolization, with improvement in pain,3,4. Rapet al. concludeThese results prompted us to employ this therapeutic modality for our patient to decrease bleeding during the operation. After completion of preoperative selective arterial embolization (SAE), she underwent tumor curettage and bone grafting with a combined anterior-posterior approach. We had enough time to completely remove the tumor tissue and manage the tumor cavity to minimize blood loss. At the final 9-year postoperative follow-up, there was no local recurrence or metastasis, and the patient has returned to normal life. The good long-term outcome suggests that for this challenging disease, repeated preoperative SAE, curettage and bone grafting could be an effective and relatively less complicated technique, resulting in less blood loss.We think it is an optimal treatment for giant cell tumor involving the sacroiliac joint, with repeated selective arterial embolization and curettage, which has the advantage of less injury, less blood loss and fewer complications.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 of this journal.CT: Computerized tomography; GCT: Giant cell tumor; RSAE: Repeated selective arterial embolization; SAE: Selective arterial emolizationThe authors declare that they have no competing interests.YXC led the coordination of this case report. FZH has assisted with the embolization operation. XM and XSF have made great effort in the follow-up of this patient. YXC wrote this manuscript. All authors participated in the modification of drafts and read and approved the final manuscript.


The present study reports the diagnostic utility of endoscopic ultrasound-guided fine needle aspiration (EUS-FNAC) in two patients affected by gastrointestinal stromal tumours (GISTs) of the stomach. Clinically, the patients demonstrated skin pallor, melena, gastric discomfort and pain that had lasted three days or weeks. The cytological findings are discussed; these were strongly supported by immunocytochemical procedures that were performed on cell blocks and further confirmed following post-surgical histopathological examination. The crucial aim of GIST management is to determine a correct diagnosis in early-phase disease in order to realize an adequate curative surgical resection before the tumour becomes unresectable or metastatic. Moreover, a correct pre-surgical differential diagnosis of GISTs from other mesenchymal neoplasms may be easily made by EUS-FNAC, supported by cytological and immunocytochemical features. Gastrointestinal stromal tumours (GISTs) are uncommon mesenchymal neoplasias of the gastrointestinal tract that may occur between the oesophagus and anus, and even in the omentum and mesentery –7. GISTsThe clinical presentation of a GIST is largely dependent on the site of occurrence, as well as the size of the tumours, although the clinical signs and symptoms, including nausea, abdominal pain, weight loss, anaemia or melena are non-specific and therefore not useful for the diagnosis –7. HowevA large series of GIST cases revealed that these tumours have a broad spectrum of clinical behavior at all sites of occurrence. However, they are considered to be potentially malignant ,12–14 anThe present study reports two cases of gastric GISTs that occurred as submucosal or intramural nodules. The diagnosis of a GIST was achieved by endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) and immunocytochemistry, which was further confirmed by post-surgical examination. Written informed consent was obtained from both patients; the original corrresponding declarations were available at the Department of Human Pathology, University of Messina, Italy.μm thick sections were cut and routinely stained by haematoxylin and eosin. Parallel serial sections of the same thickness were mounted onto silane-coated glasses and submitted for immunohistochemical procedures, as previously described  revealed a 40-mm oval lesion with defined margins measuring 56.4×33.5 mm. The lesion was localised in the submucosal layer of the gastric wall between the corpus and the antrum, along the small gastric curvature . No lesiescribed –20.A 76-year-old male presented to the Surgery Department, University-Hospital Health Network ‘Polyclinic G. Martino’, with gastric discomfort and pain in the mesogastric region that had lasted three weeks. During a general examination, the pain increased with palpation and a pale skin tone was noted. Ultrasonography of the abdomen revealed a 22.4×17.4-mm hypoechoic round lesion with a well-defined margin. The lesion was localized in the superficial muscular layer of the gastric corpus, between the posterior wall and the large gastric curvature . No lesiFollowing the FNAC procedures, the two patients were observed for a period of 48 h for any procedure-related complications.The smears from the two cases exhibited haemorrhagic backgrounds with a well-represented cellularity. They were organized in cohesive groups, arranged in three-dimensional clusters or as single cells  and 2B. μm serial sections, utilizing the following commercially obtained antisera from DakoCytomation : CD117 , CD34 , smooth muscle actin , vimentin , S-100 , desmin , glial fibrillary acidic protein  neurofilaments  and Ki67 . In cases 1 and 2, a strong and diffuse cytoplasmic immunostaining was encountered for vimentin  have been proven to be of significant value in the diagnostic evaluation of benign and malignant diseases, as well as for the staging of malignant tumours of the gastrointestinal tract and adjacent organs –23. The Another notable point from the present study is the pre-surgical opportunity to perform a correct differential diagnosis of gastric GISTs from other mesenchymal neoplasias, including leiomyoma, schwannoma and solitary fibrous tumours, or alternatively, metastatic diseases, such as spindle cell amelanotic melanoma or carcinoma. The additional value that immunohistochemistry may provide as a diagnostic confirmatory procedure, together with the specific cytological findings, should be considered either in smears or in cell blocks. Leiomyomas are strongly positive for desmin and SMA, but negative for CD117 and CD34, while schwannomas show positivity for S100 protein, with a lack of CD117 and CD34 expression ,3,6,7. APredicting the clinical behavior of GISTs remains a complex and noteworthy task, as numerous indicators have been proposed and extensively evaluated without a widespread consensus being achieved ,13,25–29


Hyperplasia of the endometrial stroma is a poorly recognized lesion, lacking widespread recognition with most, if not all, such cases sequestrated in the literature as endometrial stromal nodules or low-grade endometrial stromal sarcomas. In this paper, we describe three examples of “endometrial stromal hyperplasia” which have a remarkable morphological similarity with the normally proliferating endometrial stroma and the endometrial stromal neoplasms, but which also possess subtle, but sufficient, differences to justify their taxonomic separation. The hyperplastic process in the endometrium is, for the most part, diffuse involves both glands and stroma (simple hyperplasia), less frequently, is focal or multifocal, and affects exclusively endometrial glands  . Endomet2 fragment of tissue together with a small endometrial polyp 0.7 cm at maximum diameter.A 71-year-old woman was referred for evaluation of a thick endometrium detected by routine ultrasound as part of her annual check-up. The patient had a past medical history of left colectomy for adenocarcinoma of the caecum 3 years ago, and polypectomy for a tubulovillous adenoma of the large intestine 2 years ago. She suffered from arterial hypertension for several years. There was no history of hormonal therapy. The patient had two normal deliveries and two induced abortions. Hysteroscopic biopsy produced a 0.5 cmOn histological examination, the larger fragment was formed, almost entirely, of sheets of small uniform cells, with ovoid to spindle-shaped nuclei, having scanty cytoplasm and ill-defined cell borders . There wA 47-year-old woman was referred for gynaecological consultation by her family doctor because of a 30-month history of abnormal vaginal bleeding. She had two children, both delivered by Caesarean section. Physical examination revealed multiple uterine leiomyomas. Her prior medical history was left ovariectomy for mucinous cystadenoma 5 years ago. She was not receiving hormonal therapy. Total abdominal hysterectomy and right salpingo-oophorectomy were performed. The hysterectomy specimen contained multiple typical leiomyomas. There was widespread adenomyosis. The cervix was normal. The endometrium exposed a dense cellular polyp, 0.6 cm in greatest diameter, composed entirely of small spindle-shaped cells with little cytoplasm and ill-defined cell borders . Thick-wA 75-year-old woman presented with a one-week history of heavy abnormal vaginal bleeding. She had two normal deliveries. The patient was obese, hypertensive and had a 10 mm thick endometrium with two small polyps protruding into the endometrial cavity. She was currently taking thyroxin for hypothyroidism; she was not receiving hormonal therapy. Diagnostic and therapeutic curettage yielded four endometrial fragments composed entirely of small, regular stromal cells with ovoid to spindle-shaped nuclei and an ill-defined cytoplasm, reminiscent of normal late proliferative stroma. There were no cellular pleomorphism, mitotic activity, or invasion of lymph-vascular channels. The cells were arranged in sheets in which many vessels of the arteriolar cell type were present . Of the α-smooth muscle actin, calretinin. Immunohistochemistry was performed in all cases and revealed a diffuse positivity for ER , PR, vimThere is now additional evidence that cellular proliferations in the endometrium may take the form of pure stromal hyperplasia—a rare, but specific, pattern of growth which is distinct from other forms of proliferative activity at this site. This is hardly surprising for a dynamic tissue, as the endometrium, having two main structural components, the endometrial glands and the specialized endometrial stroma, either of which, alone or in any combination, possesses the potentiality for intense proliferation and growth in response to appropriate hormonal stimuli. It has been recognized, in this respect, that prolonged unopposed oestrogenic stimulation of the endometrium may afford a pattern of hyperplastic growth which involves both endometrial components, that is, the glands and the stroma (simple hyperplasia), or it may, less frequently, induce a form of hyperplasia with proliferations restricted to endometrial glands  ; analogoThe belief that stromal cell proliferations in the endometrium are by definition neoplastic was first challenged by Stewart et al.  when theThe few examples of endometrial stromal hyperplasia recorded so far are only an approximation of its true incidence and for many cases have not been reported, while others remained unrecognized and recorded as endometrial stromal nodules or low-grade endometrial stromal sarcomas this is largely because the diagnostic label of stromal hyperplasia was not established. Nevertheless, these examples illustrate the point that “pure” stromal cell proliferations of the endometrium are, in itself, not necessarily neoplastic, but some may take the form of stromal hyperplasia. Yet, the recognition of this form of endometrial hyperplasia and its separation from the endometrial stromal tumours may present difficulties, particularly in curettage material, the hyperplastic stromal lesions are histologically very similar, almost identical, to those which are truly neoplastic, and there are only subtle morphological differences and molecular translocations that make the distinction possible. In fact, analysis of our cases showed several features to be of diagnostic value in this respect.t translocation [Thus, in accordance with Stewart et al. views , the endlocation . t(p15q21) that results in a JAZF1-JJAZ1 gene fusion [By contrast, neoplasms of the stromal nodule type are usually large, up to 20 cm in diameter, with an average size 5-6 cm diameter, although they can be as small as 0.8 cm . These lJAZF1/JJAZ1 caused by the t translocation [The tumour cells of low-grade endometrial stromal sarcomas still resemble normal endometrial stromal cells, but may show mitotic activity (<3 mitoses to >10 mitoses per 10 HPF) and perhaps areas of nuclear pleomorphism. The distinctive feature of this tumour is, however, deep myometrial invasion, usually accompanied by invasion of vascular channels and rarely metastases. The tumours, in common with stromal nodules, show the fusion gene p1; q21 tralocation . The undp1; q21 tralocation . The clinical features of patients with endometrial stromal hyperplasia are very much the same as those of women with stromal cell neoplasms, the commonest complaint being abnormal uterine bleeding. Similarly, the hyperplastic condition occurs over a wide age range, from before the 1940s to over 75 years of age, with a mean of 58 years. Following surgical removal of the uterus and ovaries, adenomyosis/endometriosis and uterine leiomyomas were commonly encountered, and, with the exception of Stewart's et al. case, there was a high incidence of coexistent endometrial polyps. These pathological features are in no way specific or characteristic for this particular form of hyperplasia, but it seems to imply that an oestrogenic milieu prevails. t. Despite the short followed up time, it is believed that any hyperplastic lesion lacking cytological atypia and, for that matter, endometrial stromal hyperplasias have probably a favourable outlook and, therefore, merit taxonomic separation. It is apparent from this discussion that pure stromal cell proliferations in the endometrium are not necessarily neoplastic, as the few examples described appear to be genuine hyperplastic in nature. It is suggested that if a stromal lesion is of small size, intraendometrial in localization, and multifocal in distribution, and if it has a tendency to form polyps or arise in a preexisting polyp and lacks vascular invasion, then it is justifiable to be considered as endometrial stromal hyperplasia. A specific molecular genetic test that might support the diagnosis in problematic cases is


To the best of our knowledge this is the first case where a Silastic drain is used in ureteral surgery instead of a common urological stent. Patients coming from other institutions, especially in peripheral areas, can be treated with non conventional devices and if traditional imaging is inconclusive, computed tomography (CT) can provide valuable information to make the right diagnosis.We present the unusual case of a 32F Silastic drain found inside the urinary tract in a female patient who had previously undergone ileal loop replacement of the left ureter for post-hysterectomy stricture at another Institution, and had subsequently repeated surgery due to persistent hydronephrosis. Radiological findings on plain abdominal X-ray were quite misleading, while CT allowed a correct assessment of the drain features.While double J stents of different lengths, sizes and materials are used in ureteral surgery, the use of Silastic drains has not been previously reported. In light of the present experience we don’t suggest its routinely use. While double J stents of different lengths, sizes and materials are used in ureteral surgery, to the best of our knowledge the use of Silastic drains has not been reported. This uncommon finding is due to the fact that the procedure had been performed by General Surgeons, and is also explained by the size of the intact ileal loop. The poor outcome of bowel segments incorporated into the closed urinary system was reported first more than 30 years ago , and at Plain radiography of the abdomen is the diagnostic procedure most commonly performed to assess the position of ureteral stents. Its main advantages are its low cost, technical ease, ubiquitous availability even in small hospitals, very fast imaging time, and relatively low radiation dose. However, while interpretation of X-ray images is usually straightforward in patients with correctly positioned or (at the opposite extreme) grossly displaced ureteral stents, findings can be inconclusive or misleading when nonconventional devices are used or in case of subtle rupture or displacement of ureteral stents -6. In suIn this article we report an unusual case of a 32F Silastic drain found inside the urinary tract in a female patient who had previously undergone ileal loop replacement of the left ureter for post-hysterectomy stricture at another Institution. Radiological findings on plain abdominal X-ray were quite misleading, while CT allowed a correct assessment of the drain features.A 59-year-old woman was admitted for recurrent urinary tract infection (UTI). Past history revealed right hip prosthesis and hysterectomy for cervical cancer at age 56, followed by radiotherapy. An extensive stricture of the left ureter had been treated with ileal loop substitution in a General Surgery environment at age 58. The procedure was performed in a general surgeon setting instead of a urologist because the patient lives in a peripheral area with first level hospitals. Three months later, due to worsening of left hydronephrosis, the patient underwent pyeloplasty with placement of a 'stent' of unknown characteristics using a flank approach. Ten months after the second procedure, the patient was evaluated at our institution for recurrent UTI and fever, with irritative bladder symptoms (frequency and urge incontinence). Serum creatinine was 1 mg/dL; urine culture revealed >1 million E. coli CFU (Colony Forming Units) per mL.As a second step a plain X-ray of the abdomen was performed Figure , that shIn order to get more detailed information about the morphology of the left urinary tract, CT urography was subsequently carried out using a 64-row CT scanner. Axial images, Maximum Intensity Projection and Volume Rendering (VR, Figure The patient underwent left nephrectomy and removal of the ileal loop, containing a 32F partially encrusted Silastic drain with a radiopaque marker (Figure In our case, CT proved to be superior to plain X-ray due to its higher contrast resolution and its ability to display a body volume as a stack of cross-sectional images, rather than as a projective representation of the attenuation of all tissues crossed by a relatively wide X-ray beam ,8. MoreoWhile double J stents of different lengths, sizes and materials are used in ureteral surgery, the use of Silastic drains has not been previously reported. In light of this experience we don’t suggest its routinely use.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 Series Editor of this journal.The authors declare that they have no competing interests.GF-GP contributed to conception, design, acquisition and drafting of the manuscript. FM and AM contributed in drafting the manuscript. SF contributed to acquisition of radiological information. CS contributed in drafting the manuscript and revising it critically for important intellectual content. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/12/34/prepub


Malignant perivascular epitheliod cell tumor (PEComa) is a very rare entity composed of distinctive perivascular epitheliod cells with variable immunoreactivity for melanocytic and muscle markers. At present this neoplasm does not have a known normal cellular counterpart and the natural history is often unpredictable. Up to now, few cases of PEComa have been described and treatment modalities are still controversial, particularly in advanced conditions.We handled the case of a 42-year-old man with unresectable PEComa of the abdomen. A 7 cm hepatic hypodense lesion between segment V and VIII of the liver and diffuse intraperitoneal nodules of 0,3-3,5 cm along the right subcapsular hepatic region, were documented by a CT scan. Radiological images showed abnormal lymph nodes of the right internal mammary chain and anterior mediastinum. The patient underwent an explorative laparotomy for uncontrolled intrabdominal hemorrhage without a well-defined preoperative tumor diagnosis. At surgery, multiple lobulated nodules containing hemorrhagic fluid on the liver surface, peritoneum and omentum were confirmed. The procedure had a palliative intent and consisted of hemostasis, hematomas evacuation and omentectomy. The diagnosis of PEComa was made after surgery on the basis of morphological and immunohystochemical criteria. Radiological and intra operative findings suggest that the mass has an hepatic origin with diffuse involvement of hepatic capsule and suspensory ligaments. The patient received medical support care with blood and plasma transfusions. In our experience, PEComa was clinically malignant, leading to a fatal outcome 25 days after hospital admission of patient.Here we report and discuss the peculiar clinical, radiological and morphological presentation of unresectable PEComa. Although in the majority of the reported series, PEComas show a more better prognosis, our case presents with a particular aggressive biological behaviour. The importance of a correct preoperative diagnosis, the need for more effective targeted therapies based on tumor molecular knowledge and evidence-based clinical studies are emphasized together with a revision of the concerning scientific literature. PEComa is a mesenchimal neoplasm, predominantly affecting young adults and female individuals . It inclA 42-year-old male was admitted to our observation with dyspnea, temperature, abdominal discomfort and weight loss. He had previously experienced tuberculosis infection, and his past surgical history was uneventful. The physical examination confirmed epigastric and mesogastric pain. The radiological images demonstrated right fluid pleurical collection with passive collapse of the lung inferior lobe, abnormal lymph nodes of the right internal mammary chain and anterior mediastinum Figure . At the The histological diagnosis of malignant PEComa is based on the published criteria by Folpe and co-workers. In our case, the tumor is characterized by the proliferation of epitheliod and spindle cells. The neoplastic cells are arranged in small-nests or sheet-like patterns, traversed by a delicate vasculature consisting of a rich network of sinusoid-type blood vessels. The tumor cells showed a round to oval nucleus, often with prominent nucleolus and exhibited high nuclear pleomorphism. The tumor is comprised of a population of large polyclonal cells with abundant cytoplasm. The mitotic index is elevated  and the coagulative necrosis, a prominent feature, appeared as multiple foci of variable sizes. Microscopically the tumor border was infiltrative.2O2/3% for 5 minutes to inhibit endogenous peroxidase and then washed in H2O. Antigen was unmasked by treatment with EDTA at pH 9, or with citrate buffer at pH 6 in a microwave oven (two 5-minutes courses). The slices were then held for 20 minutes at room temperature. After washing in PBS/Tween-20, sections were incubated for 30 minutes with the primary antibodies. Then, they were washed and stained with Bond™ Polymer Refine/HRP Detection Kit according to the manufacturer's protocol  or Bond™ Polymer Refine Red Detection Kit (Leica) for HMB-45 an Melan-A. For negative controls, we substituted non-immune sera for the primary antibodies. The immunohistochemistry analysis demonstrated positive staining for Vimentin (Novocastra), Melan-A (Dako), HMB-45 (Dako), smooth muscle actin (SMA) (Dako), MIB-1(Novocastra), and CD31 (Novocastra). It was negative for S-100 protein (Dako), Cytokeratin-AE1/AE3 (CKAE1/AE3) (Novocastra), Cytokeratin-5 (CK5) (Novocastra), CD30 (Dako). Based on this specific immunophenotype profile, diagnosis of PEComa was made  and the term PEComa was first introduced by Zamboni four years later . Only inThe authors declare that they have no competing interests.FS and DR analyzed the data and wrote the manuscript. DA and RC carried out the histological and bio-molecular studies. RC participated in the acquisition and interpretation of radiological data. DP, AD, RC and ML have been involved in the acquisition of clinical data and in the reviewing the scientific literature. FS and PI contributed to the final version and carried out the clinical case report. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2482/11/3/prepub


Cases of primary colorectal adenocarcinoma metastasized to the palatine tonsil are extremely rare. To the best of our knowledge, only 10 cases have thus far been previously documented in the English literature. A 37-year-old Chinese woman presented with a right palatine tonsil swelling and odynophagia 5 months after a surgical resection of rectal adenocarcinoma was performed. The patient underwent a tonsillectomy, and a metastatic poorly differentiated adenocarcinoma from a colorectal origin was revealed by immunohistochemical analysis. The manner in which tonsillar metastases are involved remains unknown and should be further studied. Here, we report a new case, briefly summarize these 10 cases and review the literature. The most common sites of distant metastases from primary colorectal carcinoma are in the liver, lung, and brain, and less commonly in the bone, ovary, and adrenal gland. Metastasis to palatine tonsil from a primary colorectal carcinoma is an extremely rare event. Only 10 cases have thus far been previously documented in the English literature. Hematogenous dissemination is a probable explanation for the mechanism of metastasis to the palatine tonsils .A metastatic tumor in an unusual site may sometimes be troublesome to distinguish between a synchronous or metachronous primary cancer and a metastatic disease, especially when it is asymptomatic. In this paper, we report the case of a 37-year-old Chinese woman with a metastasis to the right palatine tonsil from a rectal adenocarcinoma and review the literature.A 37-year-old Chinese woman was evaluated for right tonsil swelling and a sore throat in our hospital. She was diagnosed in September 2011 with rectal cancer revealed by generalized peritonitis evoked by tumor perforation. At diagnosis, the preoperative evaluation did not show distant metastasis (M0). The patient underwent an urgent exploratory laparotomy. The tumor measuring 10.0 cm×4.0 cm×3.5 cm was identified at the anterior rectal wall under the peritoneal reflection intraoperatively. A low anterior resection using a total mesorectal excision technique was performed, with a colostomy using noninflamed descending colon, and the divided end of the rectum was closed. The excised specimen contained a poorly differentiated adenocarcinoma invading into nonperitonealized perirectal fat with negative surgical margins (T3). Nine regional lymph nodes were positive for tumor extension (N2b). The tumor was classified as stage IIIc disease based on the American Joint Committee on Cancer TNM staging system.The patient did not receive any adjuvant radiotherapy and chemotherapy postoperatively. In March 2012 the patient was admitted to our department on account of abdominal pain and distension triggered by small bowel obstruction. A nonenhanced abdominal computed tomography scan revealed enlarged para-aortic lymph nodes measuring approximately 4 cm in diameter  were all within normal limits. A punch biopsy was taken for histological examination, which showed a poorly differentiated adenocarcinoma Figure . The patMetastases to tonsils from nonhematological malignant neoplasms are rare events , accountMetastasis from a primary colorectal adenocarcinoma to the palatine tonsil is an extremely rare event. We searched PubMed, MEDLINE, and Google Scholar, from inception to December 2012, using the terms ‘colorectal/colon/colonic/rectum/rectal’; ‘cancer/ carcinoma/adenocarcinoma’; ‘palatine tonsil/tonsil’; and ‘metastasis’. The literature was limited to English-language case reports. References of included articles were also searched. Only 10 cases have been documented previously. We present a summary of all these 10 cases, as well as the present case, to highlight their clinicopathological profiles (Table In the metastatic process, tonsillar involvement could either be the first station or a part of widespread systematic distant metastases. Although the pathway by which malignancies metastasized to the tonsil remains controversial and difficult to determine, some hypotheses have been built. Brownson and colleagues suggested that retrograde cervical lymphatic spread through the thoracic duct may be a potential mechanism, since the palatine tonsil does not have afferent lymphatic vessels . On the Malignancies of the palatine tonsil are unusual. Squamous epithelial carcinomas and lymphomas are generally observed in this area. In the present case, the palatine tonsillar tumor cells shown a glandular epithelial phenotype histologically Figure , and werMetastatic palatine tonsil cancer from a primary colorectal adenocarcinoma is an extremely rare malignancy with a poor prognosis, and may lay a pitfall for clinicians. Immunohistochemical examination should therefore be performed. Immunomarkers including CK20, CK7, CDX-2, and villin are facilities in immunohistochemistry examination. The route of metastasis to the tonsil remains unclear and should been studied further.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.CK: Cytokeratin; H & E: Hematoxylin and eosin.The authors declare that they have no competing interests.HW performed the majority of this study and drafted the manuscript. PC provided the collection of material from the database. All authors read and approved the final manuscript.


Background. Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade soft tissue neoplasm originating from the dermal layer of the skin. It usually occurs in adults; however, it can rarely be seen in infancy and childhood. Diagnosis of DFSP in children is quite difficult-given-rarity of this lesion, its variegated appearance, and its presentation sometimes at unusual sites. Case. We present the case of five-year-old boy who came with painless lesion on a forehead. Fine needle aspiration cytology (FNAC) suggested possibility of mesenchymal neoplasm. Patient was advised excision biopsy. Final diagnosis of DFSP was made based on histopathological findings. The patient was then advised reexcision surgery with wide margins. The patient was lost to followup and later turned up after two months with recurrence of a similar swelling at the same site. Conclusion. DFSP in children is rare and difficult to diagnose. Treatment of childhood DFSP is often delayed leading to incomplete excision. Hence, there is need to recognize and appropriately manage this uncommon childhood neoplasm. Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, intermediate grade neoplasm originating from the dermal layer of the skin . It is mDFSP in children is a rare and difficult diagnosis particularly when presenting at uncommon site like head and neck. Treatment of childhood DFSP is often delayed because of misdiagnosis leading to local growth and incomplete excision. Hence, pediatricians should be aware of this uncommon entity and should ensure wide excision and reduce risk of recurrence.We present a case of a five-year-old boy who presented with a slightly raised, painless lesion on base of the nose near the medial side of a the left eyebrow  since 6 The patient was then advised reexcision surgery with wide margins. Patient was lost to followup and later turned up after two months with recurrence of similar swelling at same site.Pathological Finding: May-Grünwald Giemsa (MGG) stained cytological smears showed scanty cellularity comprising few cohesive clusters and singly dispersed oval to spindle cells in hemorrhagic background Figures  and 3. TSubsequently an excision biopsy was performed. We received tiny skin covered soft tissue, measuring 0.8 × 0.6 × 0.4 cm. Hematoxylin and eosin stained sections showed cellular and poorly circumscribed tumor in the dermis comprising interwoven bundles and fascicles of uniform spindle shaped cells arranged in “storiform” or “cartwheel” pattern . The tumDFSP is a slow-growing, locally aggressive tumor of intermediate malignancy with a marked tendency to local recurrence, but which rarely metastasizes . AlthougCytological smears of DFSP usually show scanty to moderate cellularity comprising scattered single cells and clusters with a storiform pattern and naked nuclei. The oval to spindle nuclei contain fine homogeneous chromatin with small nucleoli . SimilarThe DFSP in pediatric age group should be differentiated with other fibrohistiocytic tumors like dermatofibroma, leiomyoma, neurofibroma, dermatomyofibroma, infantile myofibromatosis, and fibrous hamartoma of infancy . Dermato DFSP in children is often misdiagnosed, leading to delay in treatment. Wide local excision (WLE) is necessary, since DFSP often exhibits extensive infiltration beyond gross margins. Particularly for neoplasm developing in the head and neck area it has been shown that they have a high rate of recurrence even after wide local excision with 2- to 3-cm margins. In order to achieve negative resection margins and simultaneously preserve the uninvolved tissue from resection, some authors suggest the use of Moh's micrographic surgery . AdjuvanDFSP in children is a rare and difficult to diagnose particularly when presenting at uncommon site like head and neck. Treatment of childhood DFSP is often delayed because of misdiagnosis leading to local growth and incomplete excision. Hence, pediatricians should be aware of this uncommon entity and should ensure wide excision and reduce risk of recurrence.


There is a well-documented relationship between urinary bladder diverticula and intradiverticular neoplasms. The great majorities of these tumors are urothelial carcinomas, but may also be of glandular or squamous type. Sarcomas occurring within bladder diverticula are exceptionally rare and highly malignant lesions, with only 20 well documented cases published in the literature to date (including carcinosarcomas). We report a case of osteosarcoma of the bladder diverticulum in a 68-year old man, which clinically mimicked intradiverticular calculus. To our knowledge, this is the second case described in the literature to date, and the first in English literature. Bladder diverticula mainly present as small, clinically insignificant anomalies. Rarely, probably due to urinary stasis and prolonged exposure to carcinogens, malignancies may arise within them. Analogous to overall distribution of bladder tumors, most intradiverticular tumors also originate from urothelial cells. Adenocarcinomas and squamous cell carcinomas are uncommon and carcinosarcomas and sarcomas are exceptionally rare within diverticula. There are only 20 well documented cases of intradiverticular sarcoma with or without carcinomatous component published in the literature to date . Eleven A 68-year old man was admitted to our clinic in November 2006 because of an isolated episode of gross hematuria. Intravenous urography, performed a few days later, identified a right-sided bladder diverticulum measuring 5 cm in the largest diameter, with no wall irregularities, or filling defects, which would point to the presence of neoplasm. In October 2007, after a few additional episodes of painless gross hematuria, the patient returned to the clinic and was hospitalized for further diagnostic procedures. Cystoscopy revealed a diverticulum over the right side of the urinary bladder. Intravenous urography showed a large bladder diverticulum measuring 7 × 7 cm with a 3 cm stone within Figure . The divGrossly, the bladder diverticulum exhibited tan to brown mucosa with a 3.2 × 3.5 × 1.8 cm greyish, solid tumor with gritty, firm center Figure . MicroscSix months postoperatively the patient was alive and well. Extensive clinical examination revealed no signs of other primary tumor.In April 2009, the patient was hospitalized again due to macrohematuria. A CT scan revealed extensive tumorous intrapelvical mass Figure  which waPatient died in July 2009, 2 years and 8 months after the initial symptoms had occurred. The autopsy revealed a large tumor of urinary bladder, measuring 29 × 26 × 18 cm, filling the pelvis, with extension to abdomen Figure . There wUrinary bladder diverticula are outpouchings of bladder mucosa through weakened muscular areas of the bladder wall. They are commonly located in the posterior wall above the trigone, near the ureteral orifices and in the dome at the site of an obliterated urachus. They are mostly acquired, developing secondary due to increased intravesical pressure in patients with urethral or bladder neck obstruction and in patients with neurogenic bladder .There is a well documented relationship between urinary bladder diverticula and intradiverticular neoplasms, with a reported prevalence of 1-10% ,5. It seIn the present case, the tumor mimicked diverticular lithiasis on radiological examination, but histologically it was composed of irregular, lacelike osteoid and closely packed anaplastic, round to spindle cells with prominent mitotic activity. More towards the center of the tumor, the osteoid was mineralized. These findings, which clearly describe the image of osteosarcoma, should be differentiated from other bone-forming tumors, like carcinosarcoma, urothelial carcinoma with osseous metaplasia and, theoretically, metastasis of osteosarcoma of other primary sites ,15. The In most cases, bladder diverticula are small and asymptomatic, but occasionally malignant transformation may occur within them. Overall incidence of sarcomas is the lowest one among urinary bladder tumors. Our case of primary intradiverticular osteosarcoma is the first such case reported in English literature. We find it very important to make a thorough investigation in patients with symptomatic diverticula because once a malignant transformation had occured, there is a predisposition for rapid spread of the tumor due to lack of muscular layer in diverticular wall.Written informed consent was obtained from the patient's family for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.IG supplied relevant clinical information about the patient, drafted the manuscript and acquired radiographs. TL acquired photomicrographs, was involved in the histopathological evaluation and drafted the manuscript. DT and BK participated in the histopathological evaluation and have been involved in revising the manuscript critically. TD was involved in literature search and drafting the manuscript. DT and IT supplied relevant clinical information about the patient and were involved in preparing the material. All authors have read and approved the final manuscript.


Introduction. Our objective is to report a case of an infrequent entity as the giant hydronephrosis. Case Report. We report the case of an 82-syear-old male referred for a poor general condition. A radiological study revealed a great left hydronephrosis secondary to an urothelial carcinoma. The patient died due to his poor general condition. A histological diagnosis revealed a transitional cell carcinoma of renal pelvis and ureter and atrophic renal parenchyma. Conclusion. Giant hydronephrosis represents a very often entity to be taken into account in cases with big cystic abdominal masses in absence of unilateral or bilateral kidney. Simple nephrectomy is the treatment of choice in most cases. Nevertheless, in cases of nonsubsidiary surgery, percutaneous drainage may be necessary. Since Stirling defined giant hydronephrosis as the amount of fluid exceeding 1,000 cc in the urinary tract of adults, very few cases have been reported in adults, because it is very difficult to diagnose due to the lack of a well-defined pattern. The aim of this paper is to present one case of hydronephrosis along with a review of the current literature.An 82-year-old male was brought to the emergency room for a serious deterioration of his general condition with abdominal pain and an abdominal mass effect. It was impossible to obtain information about the patient's clinical history due to his lack of cooperation and lack of information from his companion. Upon physical examination, the patient showed symptoms of a stupor state without focal neurologic signs, severe malnutrition, dehydration (positive skin pinch), and a tense abdomen with a large abdominal mass, which appeared to be located on the left hemiabdomen. No signs of peritonitis were observed.The blood laboratory tests highlighted a haematocrit of 14.3%, haemoglobin of 4.0 g/dL, creatinine of 2.1 mg/dL, and urea of 89 mg/dL. The abdominal radiograph  showed aA complementary radiological study via abdominal ultrasound and CT scan  was perfUpon admission, we punctured the cystic mass and removed 5,000 cc of serosanguineous fluid. The biochemical analysis of the fluid revealed the levels of 6 mg/dL for glucose, 1.5 mg/dL for creatinine, 127 mg/dL for urea, 137 mEq/L for sodium, and 4 mEq/L for potassium. The patient progressed unfavourably during admission and died two days later despite fluid resuscitation and blood transfusions.During necropsy, the abdominal mass was reported as a left renal mass weighing 4.500 g, with dimensions of 30 × 21 × 10 cm, resulting from ureterohydronephrosis. The histopathology sample showed a multifocal, extensively necrotic, and WHO Grade 2 papillary transitional cell carcinoma with invasion of the superficial muscular layer (pT2a), which affected the renal pelvis and the left ureter up to the bladder. Remnants of atrophic renal parenchyma were observed.Thromboembolism of the left main pulmonary artery was determined as the cause of death. We present this case to show the final evolution of the urothelial carcinoma using images. Giant hydronephrosis is a condition caused by the accumulation of more than 1,000 mL in the excretory system of either kidney, as defined by Stirling in 1939 . The firThe most common cause of giant hydronephrosis, as described in the literature, is congenital stenosis of the ureteropelvic junction associated with other urinary tract abnormalities; other causes include urolithiasis and tumour pathology , 4 of thEpidemiologically, giant hydronephrosis is more common in the left kidney of males with an average content of 1-2 L in the collecting system [The clinical symptoms of these patients are not specific but typically involve increased abdominal girth due to the presence of a mass in the flank. Other symptoms were described in the literature, including pain at the flank along with haematuria resulting from trauma in the area .Historically, only 50% of giant hydronephrosis cases are properly diagnosed because of its nonspecific clinical presentation , 5. HoweThe following differential diagnoses were considered: massive ascites , 6, intrThe ideal treatment for giant hydronephrosis is simple nephrectomy, as proposed by Hoffman, because of the frequent association of foci of dysplasia and tumour changes of the parenchyma and collecting system of kidneys as a result of chronic irritation . HoweverIn our patient, giant hydronephrosis was caused by terminal-stage, upper urinary tract transitional cell carcinoma, which is particularly uncommon.In conclusion, giant hydronephrosis is a rare condition that must be considered upon the occurrence of cystic abdominal masses and the absence of one or both kidneys. Although its aetiology is diverse, late-stage tumour pathologies such as urothelial carcinoma must be considered as potential causes of giant hydronephrosis.