Irreversible electroporation (IRE) is a non-thermal injury tissue ablation technique that uses electrical pulses to cause cell death. IRE damages the endothelial cells of blood vessels; however these cells re-grow, and thus IRE does not result in permanent damage to blood vessels. We report the novel use of IRE for ablation of microscopically positive margins after resection of colorectal liver metastases (CRLM) impinging on hepatic veins.A 68-year-old female was found to have colon cancer and synchronous bilateral unresectable liver metastases. Chemotherapy with FOLFOX and cetuximab was initiated, with subsequent conversion to resectability of the CRLM. The patient underwent colectomy followed by right liver posterior sectionectomy with wedge resection of segment 5. Resection of tumor impinging on the left and middle hepatic veins would have required left hepatectomy, with insufficient remnant liver volume. The CRLM were meticulously dissected off the hepatic veins leaving a microscopically positive margin, and IRE was then used for margin ablation, leaving intact hepatic veins and venous blood flow. The patient is alive and without recurrent disease now 30 months after resection. Herein we review the IRE technology and its use in ablation of liver metastases.Use of IRE margin ablation for microscopically-positive CRLM resection may lead to long-term patient survival; further prospective randomized trials are needed to confirm this finding. Resection of colorectal liver metastases (CRLM) has been well described, with reports of five-year survival up to 58% after complete resection of disease . The ovee.g., hepatic vein, portal vein) has been a relative contraindication to surgery if tumor-free margins are unable to be achieved while maintaining a sufficient liver remnant; however, with the use of irreversible electroporation (IRE), a relatively new ablation technique, in combination with hepatic resection, tumors may be safely treated while preserving the hepatic vasculature. Irreversible electroporation is a non-thermal injury tissue ablation technique that uses electrical pulses of short duration to permanently create defects in cell membranes leading to irreversible permeabilization and ultimately cell death [In the past, tumor adjacent to major hepatic blood vessels  with cetuximab. The patient underwent seven cycles of chemotherapy over three months and was subsequently referred to our institution for evaluation.Upon review of restaging CT and PET/CT scans obtained during and after chemotherapy, the patient was noted to have decrease in size of both the primary colonic lesion as well as the liver metastases, without evidence of new foci of metastatic disease Figure . Both thThe patient was taken to the operating room and underwent a right colectomy with ileocolic anastomosis without incident and attention was then turned to the liver. Intra-operative hepatic ultrasound revealed three multi-lobulated lesions within segments 6 and 7, a small segment 5 lesion, as well as a segment 2 and 4A lesion between the middle and left hepatic veins. The metastases were resectable, and the liver, while appearing to have some damage as a result of chemotherapy, was felt to have adequate volume for a functioning liver remnant.The segment 5 lesion was wedged out in a standard fashion using electrocautery and hemostatic clips. This was a margin-negative resection despite its close proximity to the portal venous system. A right posterior sectionectomy was performed removing segments 6 and 7, along with a cholecystectomy. The right hepatic vein as well as the right posterior portal pedicles, and a few tertiary branches of the right anterior portal vein, were seen entering the tumor and were taken. Intraoperative pathologic assessment of this specimen reported negative margins (3-4 mm).Attention was then turned to the lesions in segments 2 and 4A. A formal left hepatectomy would have allowed for a margin-negative resection; however the end result would have resulted in less than 30% liver remnant in an already chemotherapy-treated liver. Based on pre-operative volumetric data, the right anterior section constituted approximately 30% future liver remnant . The resection bed of segments 2 and 4A was ablated using electroporation needle pairs placed 1.5 cm apart and 1.5 cm deep into the hepatic parenchyma that remained just adjacent to the hepatic veins. The needle pairs were placed parallel to the veins and the covered with a free flap of omental fat that had been harvested to add extra tissue through which the electrical current would travel such that the positive surfaces of the veins would be treated. Treatments were performed with settings of 3000 V using 100 microsecond pulses for a total of 90 pulses between each needle pair. A total of seven overlapping ablations were performed across the entire raw surface area. The omental fat pad was discarded, and following the completion of IRE, adequate flow was again confirmed through the left and middle hepatic veins without evidence of thrombosis. The patient tolerated the procedure well and her post-operative course was uneventful. The patient was discharged home on post-operative day 9 in good condition with normal liver function.Pathology of the resected right colon demonstrated well-to-moderately differentiated adenocarcinoma invading through the muscularis propria to the subserosal tissue. All margins were negative and 0/43 regional lymph nodes were positive for carcinoma. The final pathology of the resected right posterior section demonstrated three masses, the largest of which was 3 cm, and negative margins. Pathologic result of the segment 2/4A resection specimen demonstrated a 3.6 cm mass with viable carcinoma present at the inked margin of resection, as had been anticipated, and which had been ablated by IRE. The patient now continues to be under active surveillance and has shown no demonstrable evidence of recurrence along the ablated margin 30 months after the operation Figure .Figure 5Resection of CRLM has been widely described, and survival has been reported to range anywhere from 25-70% following surgical resection . PreviouIrreversible electroporation is a non-thermal ablation technique whereby electrical pulses delivered to tissue between two or more probes cause the creation of permanent cell membrane nanopores, with subsequent breakdown of cell membranes and death by apoptosis . Notablyet al. reported their series of IRE in patients with perivascular hepatic tumors not amenable to thermal ablation due to tumor location [While response rates and long-term survival data from large prospective clinical trials using IRE are still awaited, multiple previous case report series have detailed the use of IRE for the treatment of both metastatic and primary carcinomas of the liver Table , and thelocation . Twenty-location . Of the The role of IRE in the spectrum of treatment for tumors metastatic to the liver currently is in evolution and will continue to be refined with time. Currently it appears that the role of IRE will be for local ablation of unresectable masses adjacent to major vascular or biliary structures or for margin ablation to facilitate extended resections, and at the present time IRE will not replace the need for systemic neoadjuvant or adjuvant chemotherapy. It is also currently unclear if disease requiring the use of both liver resection and ablation with IRE indicates more aggressive disease biology, although it is certain that patients with positive surgical margins after resection of CRLM will have worse biology of disease with decreased outcomes and survival .Herein we describe the use of IRE to treat resection margins when the likelihood of positive margins is significant, and the use of IRE in this fashion allows completion of major liver resections in one stage as opposed to the use of a two-stage operation with portal vein embolization. In our case the multiple areas of metastatic disease and disease abutting major vascular structures were impediments to performing a traditional metastasectomy with negative margins. Our resection left microscopic disease behind at the margin abutting the vasculature; however the addition of IRE allowed for ablation of this tissue and destruction of the remaining microscopic disease, thus allowing for complete eradication of the patient’s metastatic disease with preserved hepatic venous flow, an adequately functioning liver remnant, and long-term survival benefit.Large prospective trials studying the use of IRE for ablation of unresectable hepatic disease are ongoing; however long-term outcomes data on the use of IRE are currently lacking. We present a novel technique that combines liver resection with ablation of surgical margins using IRE with the intent to eradicate all disease in an area where use of standard microwave or radiofrequency ablation would have resulted in damage to the hepatic veins and a potentially disastrous patient outcome. We believe this technique is especially useful in those patients with poor hepatic reserve, tumors abutting major vasculature or bile ducts, and in those patients with large tumor burdens, and may offer patients the possibility of long-term disease-free survival.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.


Bilateral testicular neoplasia is rare, with an incidence ranging from 1 to 5 %. Long-term survival has improved in recent years due to advanced diagnostic approaches and new therapeutic methods that are highly effective against germ cell tumors.We present the case of a patient with a primary seminomatous testicular tumor, who developed a contralateral metastasis and a subsequent metachronous tumor following chemotherapy and consolidation radiotherapy treatment.Strict follow-up, including physical examination and ultrasound examination of the contralateral testis, enabled early diagnosis of the second tumor, giving the patient a high likelihood of a definitive cure. Testicular germ cell tumors (TGCTs) represent 1–2 % of all cancers in men and are the most common solid tumors found in male adolescents and young adults between 15 and 35 years of age .Bilateral testicular germ cell tumors (BTGCTs) occur with a global incidence ranging from 1 to 7.8 % , 6. BilaA 35-year-old man with no known risk factors for testicular malignancy presented at the urology department with a right testicular mass causing painful swelling. He had been experiencing discomfort and heaviness for 10 days. His general practitioner had started antibiotic and anti-inflammatory treatment a week prior to his arrival at our department. The patient had no past medical history of testicular issues.Physical examination revealed a lump, which testicular ultrasound confirmed as an 18 mm × 12 mm × 25 mm heterogeneous hypoechogenic mass localized to the upper pole of the right testis  classification was pT1pNxpMx according to the Union for International Cancer Control (UICC) staging system, seventh edition. Two weeks after the surgery, this case was discussed during a multidisciplinary uro-oncology meeting. From October 21 to November 10 of 2009, the patient underwent adjuvant radiotherapy with doses of 25.2 Gy delivered to the paraaortic lymph nodes in 14 fractions.The patient was considered to be disease-free and received follow-up in accordance with our standard protocol, which includes chest and abdominal CT, physical examination, and tumor marker assessment every 4 months for the first 2 years, and testicular ultrasound of the contralateral side once each year. A total-body CT scan at 1 year after radical surgical treatment showed a 16-mm lymph node under the patient’s left collarbone Fig. . The lesFour years later, a follow-up ultrasound of the left testis revealed a 15 mm × 6 mm node with microcalcifications Fig. . Blood sThe patient remained under surveillance and received androgen replacement therapy with long-acting testosterone undecanoate every 12 weeks (Nebido®). A bilateral testicular prosthesis was proposed but was refused. Sperm cryopreservation was not performed because the patient had children and did not desire any additional offspring. Follow-up was performed following the standard protocol. At 2 years after left radical orchiectomy, the patient remained disease free. At the most recent visit, the patient reported maintenance of libido, no adverse effects from the androgen replacement therapy, and comfortable sexual activity and quality of life.Reported TGCT incidence rates from multiple countries between 1991 and 2011 show geographical variations, with the highest rates observed in Denmark. Over recent decades, TGCT prevalence has gradually increased in most populations of European origin and in the USA . Some stA systematic literature review—including 50,376 men with TGCT between 1991 and 2011 from many countries—reported a BTGCT prevalence of 1.82 % , 15. BilAmong patients with metachronous tumors, the mean age at diagnosis of the first tumor is 28 years old and the mean age at diagnosis of the second tumor is 35 years old . Our preThe incidence of metachronous germ cell tumors in patients diagnosed with a seminoma is influenced by the patient’s age at the time of the initial diagnosis. Evidence suggests that men who develop a seminoma when they are 30 years of age or younger may be at greater risk of developing a second tumor , 15. PatAlthough the etiology of BTGCT remains unknown, both genetic and environmental causes are implicated. Presently known epidemiological risk factors for TGCT development include a history of cryptorchidism, Klinefelter syndrome, the presence of a contralateral tumor, infertility, and a history of testis cancer in first-degree relatives . The eleIn our present case, serum markers were negative both at the diagnosis of the first tumor and at tumor recurrence. This is in accordance with the typical presentation of a seminoma. Most second tumors are discovered by the physician via scrotal ultrasonography or by the patient via testicular self-examination. Ultrasonography is a safe and simple screening procedure. One major difficulty regarding the diagnosis of second tumors is that patients may be reluctant to seek help due to fear of castration.Ultrasound detection of microlithiasis in the contralateral testis is associated with a 30-fold increase in the risk of presenting with a second TGCT, and diagnosis of the first tumor is associated with a 5–8 % risk of testicular intraepithelial neoplasm (TIN) in the contralateral testis. These data highlight the need for long-term surveillance to support early detection of the second TGCT. Within 7 years, 70 % of all TINs will progress to invasive neoplasia , 24, altThe optimal management of patients with intratubular germ cell neoplasms remains controversial. The choices include surveillance and irradiation of the contralateral testis. Since radiotherapy can result in infertility and may affect Leydig cell function , surveilTreatment for advanced germ cell tumors includes combination chemotherapy with bleomycin, cisplatin, and etoposide, followed by surgical salvage for residual disease. Depending of the patient’s risk profile, 3–4 cycles of chemotherapy are needed . The patIn the present case, the detection of a contralateral supradiaphragmatic lymph node 3 years prior to the contralateral testis diagnosis indicated metastatic relapse. A review by Cooper et al. reported that approximately 75 % of seminomas present as stage 1, with disease limited to the testis . All tumPatients with a primary testicular tumor carry an increased risk of supraclavicular lymph node metastasis despite radiotherapy, and a metachronous second primary tumor can arise even with chemotherapy. A strict follow-up protocol involving physical and ultrasound evaluation of the contralateral testis supports early diagnosis of a second tumor, increasing the chance of a definitive cure. Notably, metachronous tumors may occur long after the initial tumor diagnosis. Treatment of the second tumor is dictated by its histology and its pathological and clinical stage at the time of diagnosis. Hormonal testosterone replacement can help promote a good quality of life.The patient gave written informed consent for the publication of this case report and any accompanying images. A copy of this written consent is available for review by the Editor-in-Chief of this journal.


Torsion of nongravid uterus is rare. Cases reported have occured during pregnancy. We report a case of a patient with acute abdominal pain. The patient showed a voluminous mass situated in the abdomen and noncontiguous to the uterus by ultrasound scan. Laparotomy confirmed the diagnosis of cystic ovarian mass with torsion of uterus. Uterine torsion is a rare condition in the nongravid uterus . UterineA 55 year old multiparous postmenopausal lady presented to casualty with severe pain abdomen for the past 2 days which was sudden in onset,continuous,associated with 2 episodes of vomiting,not passed stools and flatus in the last 2 days. There was no history of fever, bleeding per vagina or per rectum, previous surgeries, discharge per vagina or bladder disturbances. Patient was conscious, oriented and in severe pain. On abdominal examination there was distension with a tense, tender, cystic swelling arising from pelvis more on the right side, about 20x20 cm size. Cervix not seen on per speculum examination. On per vaginum uterus not separately made out,cervix high up behind symphysis pubis. On per rectal examination pouch of douglas was free,rectal mucosa free. On investigating ultrasonography showed a large cystic mass of 21x19cms with septae and internal echoes suggestive of ovarian cyst with hemorrhage. Examination under anesthesia revealed two separate masses, one in the right iliac fossa & the other in the suprapubic region with a groove in between them. A laparotomy was performed by vertical midline incision, and we found a 20x20cms right cystic ovarian mass with torsion of three turns involving infundibulopelvic ligament and ovarian ligament. Uterus had undergone 1800 rotation at the level of isthmus . The left tube and ovary were normal. A total abdominal hysterectomy with bilateral salpingo oopherectomy was performed . The uppUterine torsion is defined as rotation of the uterus on its long axis by more than 45 degrees . The posTorsion of the nongravid uterus is an uncommon condition. Delay in diagnosing this condition may prove fatal, hence there is a need for a high index of suspicion. Uterine torsion should be considered as a differential diagnosis in women presenting with acute abdominal pain.


Perivascular epithelioid cells neoplasms (PEComas) constitute a family of rare tumours which have been reported virtually in all anatomic sites. The histological clarification of the malignant potential of these tumours is still problematic despite the proposed risk stratification systems. Li-Fraumeni syndrome (LFS) is caused by a germline mutation in the TP53 tumour suppressor gene. It is a rare but well-characterized cancer predisposition syndrome leading to the development of a variety of different tumour types. To the best of our knowledge, an association between this syndrome and PEComas has not been previously documented. A 24-year-old lady with known LFS presented with two uncertain-in-nature lesions, one within the right part of the liver and one within the upper pole of the right kidney. The patient underwent an uncomplicated open simultaneous right partial nephrectomy and resection of segment 7 of the liver. The morphological and immunohistochemical features of both lesions were of epithelioid angiomyolipoma (PEComa). Although the obvious scenario was that the liver lesion was a metastasis from the renal lesion, the assessment of their malignant potential according to the existing risk stratification systems was rather in favour of two synchronous primary PEComas, pointing out that the histological assessment of malignant potential of PEComas is still problematic. Perivascular epithelioid cells neoplasms (PEComas) were first described by Bonetti et al. in 1992. In 2002 PEComas were recognized as an independent entity in accordance with the World Health Organization .The diagnosis of these rare tumours predominately depends on immunohistochemistry and the characteristic coexpression of melanocytic (HMB45 and/or melan A) and smooth muscle markers (actin and/or desmin) . The hisLi-Fraumeni syndrome (LFS) is rare but very well-characterized cancer predisposition syndrome . The undWe report a case of a young patient with known LFS, who developed PEComa. To the best of our knowledge, an association between PEComa and Li-Fraumeni syndrome has not been reported before.A 24-year-old lady presented with mild pain in the right lumbar region. The physical examination was unremarkable. Standard laboratory test results, including urinalysis and urine culture, were within normal range. Her past medical history included a rhabdomyosarcoma of the right buttock surgically treated at the age of six months and a germiline pathogenic p53 mutation (Li-Fraumeni syndrome). She did not take any regular medication and she was in good general health. An abdominal ultrasound scan revealed two uncertain-in-nature lesions, one within the right part of liver and one within the upper pole of the right kidney.A subsequent MRI scan of liver and kidneys confirmed the presence of the two lesions that were shown at ultrasound scan. The lesion within segment 7 of the liver was 1,8 × 1,3 cm in diameter. It was solid and showed restricted diffusion. It was enhanced early and fairly intensely following contrast administration and also exhibited early washout . The lesThe MRI features of the two lesions were not pathognomonic for a specific pathologic entity. Following discussion within the liver and urology multidisciplinary team meetings, a recommendation for simultaneous right partial nephrectomy and partial hepatectomy was made .The patient underwent an uncomplicated open simultaneous right partial nephrectomy and resection of segment 7 of the liver and she was discharged 5 days later.The morphological and immunohistochemical features of both lesions were of epithelioid angiomyolipoma (PEComa).Histopathological examination revealed that both lesions were confined to the liver and kidney, respectively, with no evidence of invasion into surrounding tissues. The border between the tumours and the surrounding liver and renal parenchyma, respectively, was well defined. The size of the kidney and liver lesions according to histopathological examination was 30 mm and 10 mm, respectively. There was no lymph node or lymphovascular invasion and both lesions were completely excised.Both lesions were composed of an admixture of spindle and epithelioid cells, with a predominance of spindle shaped tumour cells (>70% of the tumour cells). Within both lesions there were scattered mature adipocytes and also scattered pleomorphic cells with anaplastic and multinucleated nuclei. Definite tumour necrosis could not be identified within either of the lesions.Mitotic figures were easily seen only within kidney lesion, amounting to 10 per 10 hpfs with scattered atypical forms being present, while within liver lesion mitotic figures were not identified.Immunohistochemistry of both lesions shows the tumour cells to strongly express melan A, HMB45, smooth muscle actin (SMA), and caldesmon, with focal S100 expression. There was no expression of cytokeratin-AE1/AE3, cytokeratin-CAM5.2, CD10, CD117, or DOG1. Additionally there was no expression of EMA, PAX8, RCCAg, myogenin, and MyoD1 within the kidney lesion, and there was no expression of CK5/6, Hepar1, AFP, and CD34 within the liver lesion. The proliferation marker Ki67 was expressed in approximately 10 to 20% of the tumour cells within both lesions. The only difference between the two lesions regarding immunohistochemistry was the absence of desmin expression within the liver lesion while there was focal desmin expression within the kidney lesion.The patient did not receive any adjuvant systemic treatment and, at the last follow-up, 1 year from surgery, she remains disease-free.Li-Fraumeni syndrome is a cancer predisposition syndrome caused by a germline mutation in the TP53 tumour suppressor gene , 8. WhilPEComa is a family of rare tumours consisting of perivascular epithelioid cells (PECs) that coexpress melanocytic (HMB45 and/or melan A) and smooth muscle markers (actin and/or desmin) . The celPEComas have been reported in almost every anatomic site and at virtually all ages, with a median age of presentation of 43 years. They present a strong predominance in females, with a female : male ratio of 4 : 1 . PEComasThe only potentially curative treatment for primary PEComas and for local recurrences and metastasis is surgical resection , 13. DesThe establishment of malignant potential of PEComas is challenging. Although the vast majority of PEComas show a benign course, some are aggressive with locally destructive recurrences and distant metastasis –6.Until today, none of the three proposed risk stratification systems is worldwide accepted as all of them developed by the retrospective analysis of histopathologic features of a relative small number of cases –6. In 20In our case the presence of the liver epithelioid AML proposed that this lesion was a metastasis from the renal lesion, which definitely makes the renal lesion malignant. However, it was difficult to confirm or refute this possibility based on the histopathological findings, with the alternative hypothesis being that they were synchronous tumours.Assessment of the renal lesion malignant potential was tried despite the difficulties arising from the fact that there are competing grading systems. According to the grading system specifically developed for renal epithelioid AMLs , this tuLiver primary AMLs are so rare that specific malignant criteria are not established. Within the Brimo et al. grading system, liver lesion had no features indicating aggressive/malignant potential, but the lesion would be termed of uncertain malignant potential (due to nuclear pleomorphism/multinucleated giant cells) in the more general PEComa grading system –6.All the above clearly demonstrate the weakness of the existing risk stratification systems for PEComas. Even in a case such as ours, where the presence of a second metastatic tumour definitely makes the renal lesion malignant, this cannot be deduced safely based only on the histological features of the renal lesion.PEComas are rare tumours which develop in any anatomic site of the human body. The assessment of malignant potential of these tumours is still debated, as none of the proposed risk stratification systems is widely accepted. PEComas may be one of the many manifestations of Li-Fraumeni syndrome.


Complete androgen insensitivity syndrome  is specified by a 46 XY karyotype and negative sex chromatin, bilateral undescended testes, female genitalia appearance, and lack of mullerian derivatives.A 28-year-old woman with complete (severe) androgen resistance underwent prophylactic laparoscopic bilateral gonadectomy because of the eventually increased risk of gonadal malignancy. Although the gonads appeared grossly normal, microscopic examination revealed bilateral well differentiated sertoli–leydig cell tumor (SLCT).Our Medline search revealed that this is the first reported case of bilateral sertoli–leydig cell tumor (SLCT) in androgen insensitivity syndrome. Complete androgen insensitivity syndrome  is specified by a 46 XY karyotype and negative sex chromatin, bilateral undescended testes, female genitalia appearance, and lack of mullerian derivatives. The patient is phenotypically female without uterus and a blind shortened vagina . This syComplete AIS is accompanied with abnormal testicular development and increase risk of germ cell malignancy beginning after puberty. For these reason, prophylactic gonadectomy is advised in the postpubertal period to avoid potential malignant transformation . We presA 28-year-old nulligravida was referred for consultation and laparoscopic bilateral gonadectomy after diagnosis of complete androgen resistance. The patient complained of primary amenorrhea but she did not have inguinal hernia or labial mass. She had female external genitalia. In the physical examination, development of her breasts was normal (tanner stage IV), but axillary hair was scarce (tanner stage III). Abdominal examination was normal. No inguinal or labial mass was palpated. In gynaecological examination, the vulva and perineum appeared normal. She had a blind-ending vaginal pouch of approximately 6 cm depth and the cervix was invisible. No adnexal masses were palpated.In pelvic ultrasonography, the uterus was absent and an ovoid heterogenous mass (38 × 23 mm in size) with a 13 mm nodule in the right pelvic sidewall had been reported. Abdominal ultrasonography did not reveal any para-aortic lymphadenopathy. Pelvic ultrasonography failed to identify the left gonad. Blood tests for hormones showed Follicle-stimulating hormone and luteinizing hormone levels were 7 mIU/mL and 32 mIU/mL, respectively and serum testosterone was 3.1 ng/mL, all of which were within the normal ranges. Cytogenetic analysis showed a male karyotype .The patient underwent laparoscopic gonadectomy under general anesthesia in the supine position. Visualization of pelvic contents revealed absence of uterus and fallopian tubes. There was not any evidence of abdominal or pelvic lymphadenopathy. Laparoscopic study demonstrated a 4 × 2.5 cm gray-purple colored ovoid mass with 1 cm cystic mass near the right internal inguinal ring. The left gonad (4.5 × 2 cm) was hidden behind the bowels in the left pelvic sidewall. The gonad and the attached structure were retracted medially. The posterior peritoneum and gonadal vessels were incised after coagulation and ligation, and the gonads were removed from the pelvic sidewalls .Macroscopically, the specimen consist of two ovoid gray mass which one of them contained two well-delineated, homogenous yellow-tan nodules measuring 1.5 and 1 cm on cut surface, respectively. Another mass on cut surface contained one 2.3 cm nodule similar to above description. Histopathological examination of the gonads in non-neoplastic region showed prepubertal immature tubules with lined sertoli cells. Microscopic examination of delineated nodules revealed a luminal-tubular pattern populated by monomorphus cells with dark round to oval nuclei reminiscence of immature sertoli cells. Intervening stroma was scant and focally contained clusters of polygonal cells with abundant eosinophilic or vacuolated cytoplasm and round nucleus with prominent nucleoli. Mitotic activity was not prominent and necrosis was not seen . These fAIS is the most common type of male pseudohermafroditism characterized by absence of androgen receptor (AR) activity due to a mutation at Xq11–q12 localization on the androgen receptor gene -5. It isThe complete form is 10 times more common than the incomplete form and the initial presentation is primary amenorrhea in adolescents with female phenotype, lack of pubic hair, or inguinal hernia containing testis and characterized by a 46 XY genotype and normal male androgen and gonadotropin value. Pelvic ultrasonography usually shows absence of mullerian derivates and vaginal examination reveals a blind-ending vagina without a cervix. Notably, on physical examination, all of these characteristics were present in our patient . In chilTestis tumor developing risk is thought to be 3.6% by 25 years and 33% by 50 years , 13. In In AIS pateints, prophylactic gonadectomy is advised in the postpubertal period due to avoid potential malignant transformation of the gonads , 28. GonSertoli-leydig cell tumor is a rare sex cord stromal neoplasm that account for less than 1% of ovarian tumors, occurring often in young adults , 30. It


Intrathoracic lipomas are rare benign tumors; their behavior is not completely clear and their surgical removal may be challenging. We report a case of a giant right intrathoracic myxoid fusocellular lipoma compressing the lung, tracheobronchial tree, and esophagus which was removed through a posterolateral thoracotomy. Complete removal resulted in resolution of the chest pain and improvement of the dyspnea, with no recurrence at 4-year follow-up. Lipomas are common benign tumors of mesenchymal origin. They are usually located in the subcutaneous fat and are easy to treat; their surgical removal is usually simple, even for large tumors . IntrathWe report a 70-year-old male patient with a history of pleural effusion during childhood, stroke, nasopalpebral basocellular carcinoma excised 5 years before and severe heart disease resulting in NYHA III heart failure. His main actual complaints were pain and worsening dyspnea with no response to medical therapy. Chest X-ray  and CT sSurgery was performed using a large posterolateral thoracotomy. The approach was very difficult due to dense adhesions between the lung and the chest wall . A complete extrapulmonary tumor covered by the parietal pleura was found, with 3 vascular pedicles arising from the posterior intercostal vessels which required separate ligation. There was a cleavage plane that allowed the dissection of the tumor from the trachea, esophagus, and aorta and complete removal of the tumor .The operative specimen measured 17 × 10 × 8 cm and weighed 1850 g . PatholoThe postoperative course was complicated by a bronchopneumonia requiring prolonged antibiotic treatment. There was an obvious improvement of the dyspnea and resolution of the chest pain. At 4-year follow-up, there are no signs of recurrence.According to their origin, intrathoracic lipomas may be classified as endobronchial, pulmonary, mediastinal (including cardiac), diaphragmatic, and pleural; an hourglass development through the intercostal space is also possible , 4. In oThe modern diagnosis of intrathoracic lipomas is based mainly on CT, which shows a well-delineated tumor with fatty densities . HoweverDue to their rarity, the exact behavior of these tumors is not known. In the available literature, we were able to find only case reports and small series. Sakurai et al. emphasize that their clinical-pathological behavior is not always as straightforward as expected, with the possibility of liposarcoma or an infiltrative development . MalignaThe indication for surgery is a matter of debate. Although it is a benign tumor, most authors advocate surgical removal due to the risks associated with the increasing of the dimensions and possible complications , 10, 11.In selected cases, smaller tumors may be removed using a minimally invasive approach , but thiIntrathoracic lipomas may represent a challenge despite their benign nature. A careful dissection and an adequate approach allow complete removal even in large tumors, with clinical improvement secondary to the removal of the compression.


Teratomas are germ cell tumors, manifested with a great variety of clinical features; the most common extragonadal site is the anterior mediastinum. In this case, we report the patient with a large mature mediastinal teratoma with several components of ectodermal and endothermal epithelium. A 24-year-old female patient presented with history of persistent chest pain and progressively aggravating dyspnea for the previous 3 months. A chest X-ray showed a large opacity of the entire left hemithorax. Transcutaneous needle aspiration revealed a purulent fluid. The tube thoracostomy was introduced and the effusion was evacuated. Some weeks later, patient was seen in emergency for persistent cough and lateral chest pain. CT scan revealed a mass of the left hemithorax. The mass showed heterogeneous density, without compressing mediastinum great vessels and left hilar structures. Lipase value was elevated in needle aspiration. The patient underwent a total resection of the mediastinum mass via a left posterolateral thoracotomy. Microscopy revealed a mature teratoma with cystic structures. The patient subsequently made a full recovery. This case provide benign mediastinal teratoma with total atelectasis of left lung and elevated lipase value in needle transcutaneous aspiration; this event is explained by pancreatic component in the cystic tumor. Total removal of the tumor is adequate treatment for this type of teratoma and the prognosis is excellent. Germ cell tumors are pronominally found in gonads. Teratomas are classified to be composed of ectopic tissues from 2 or 3 germs layers, including mature, immature, or malignant components .Most mediastinal teratomas produce no symptoms; they are more commonly associated with compression of mediastinal structures .Teratomas mostly occur in young adults, with an approximately equal incidence in males and females .We present the case of a patient with mediastinal mature teratoma taken originally for empyema.A 24-year-old female student patient was seen in the university hospital because of chest pain and dyspnea. The patient had been in her usual health until several months before presentation when dyspnea, dry cough, fatigue, and lateral chest pain developed.The patient had no history of medical or surgical problems; she could independently perform activities of daily living, and she had no history of smoking or drinking alcohol.On examination, the patient was normal, in no distress; she spoke with a normal voice.The blood pressure was 120/70 mm Hg, the pulse was 64 beats per minute, oxygen saturation was 95% while she was breathing ambient air, the weight was 57 kg, and the body mass index was 20.2.On chest auscultation, breath sounds on the left side were absent, with no clinically detectable lymphadenopathy; the remainder of the examination was normal.The chest X-ray showed a large opacity of the entire left hemithorax .Thoracic ultrasound showed a large fluid collection of the left hemithorax.In transcutaneous needle aspiration, the fluid removed was purulent. Therefore, tube thoracostomy was introduced, and the effusion was evacuated. The control of chest radiograph was good .Cytological analysis shows the presence of many polymorphonuclear structures, some altered without suspect cell. Mycobacterium tuberculosis in fluid aspiration.The Xpert MTB/RIF detects no DNA sequences specific forBlood levels of glucose, total protein, creatinine, aspartate aminotransferase, and alanine aminotransferase were normal. Mantoux test, serology of HIV, and amebiasis were negative. Combination therapy with amoxicillin-clavulanic acid and ciprofloxacin was begun.Bronchoscopy showed thickening of the interlobular spur and stenosis with extrinsic compression of the lingula's apertures. Bronchial biopsies revealed no specific inflammatory reshuffle.Transthoracic echocardiography showed no pericardial effusion but heart was repressed by fluid chest training. ENT examination was normal. The patient had good recovery and was discharged home.Few weeks later, the symptoms worsened, and new dyspnea on exertion and cough developed. Chest X-ray revealed opacity at the lower part of the left hemithorax .Biochemical analysis of fluid aspiration showed protein 51 g/L, glucose 2.5 mmol/L, amylase 14 U/L, lipase 4920 IU/L, hyaluronic acid 12.9 mg/L, and LDH 1820 U/L.CT scan was obtained and showed a mass of the left hemithorax, which probably originated in the mediastinum and extended to the whole left pleural space .The mediastinum tumor markers  were both normal.The patient underwent a total resection of the mediastinum mass via left posterolateral thoracostomy and entry into the pleural space was performed; the two pleural layers were intact.Many adhesions existed within the left pulmonary artery, the pericardium, and the diaphragm.Combination of blunt and sharp dissection for the division was applied uneventfully.The tumor excised en bloc was pink-colored and well circumscribed .A tube thoracostomy was introduced.The collapsed left lung was easily reexpanded, the patient was extubated and recovered well from the operation, and she was discharged on the 6th postoperative day.Pathological examination of the mass revealed mature teratoma with cystic structures lined by stratified squamous epithelium and at places cartilage, respiratory epithelium, pancreatic epithelium, urothelial epithelium, mucous secreting glands, muscle fibers, and epidermal cyst Figures  and 7.Teratomas are germ cell tumors and they are uncommon neoplasms that arise in the gonads; the mediastinum is the second most common extragonadal site of these tumors , 4.They usually occur in the middle of the body, which is the route of germ cell migration during embryogenesis; their migration can be misplaced en route to their appropriate organs, leading to the development of tumors later in life .In fact, several theories exist; one of them suggests that benign teratoma is derived from the region of the third bronchial cleft or pouch. A second theory states that these tumors arise from germinal nests of cells located along the urogenital ridge that failed to migrate to the gonads in embryological development .Primary mediastinal nonseminomatous germ cell tumors (NSGCT) are uncommon neoplasms and are clinically and biologically distinct from other germ cell tumors, and they are classified as a poor prognosis group in the IGCCC .αFP or βHCG) after surgical resection [Rivera et al. demonstrate two prognosis factors of those tumors: the presence of residual viable cancer in tumor residue and the positivity of serum tumor markers (esection . The mulesection .Benign teratomas are often asymptomatic and are discovered on chest radiograph for unrelated reasons, but sometimes they give symptoms leading the patient to consult. In the case reported, the patient had chest pain, dyspnea, and dry cough.If symptoms are present, this will be because of the mass effect caused by mediastinal teratoma.Rarely, a patient may have expectoration of hair, and this is a pathognomonic symptom. However, this distinctive symptom is extremely rare and occurs late in the natural history of this condition following tumor rupture into the tracheobronchial tree. Laboratory exams are often normal, and serum levels of human chorionic gonadotropin and alpha-fetoprotein are always normal in patients with benign teratoma.Radiology exams reveal usually a well-circumscribed mediastinal mass that often protrudes into one of the lung fields.Pleural effusion also can be the most common ancillary CT finding in ruptured mediastinal teratomas. Pericardial effusion, especially in patients with a teratoma adhering to the pericardium, also appeared to suggest rupture of the tumor into the pericardium.In the case under discussion, CT scan showed a mass of the left hemithorax extended to the whole left pleural space.High level of amylase or lipase can be noted in pleural fluid that originated from a mediastinal teratoma and suggest pancreatic component in mediastinal mass.Surgical excision is the treatment of choice for mediastinal teratomas.The aim of surgery is to achieve complete clearance of the tumor without damage to associated or adjacent structures. This requires a careful and meticulous surgical dissection.The prognosis of mature teratomas of the mediastinum is excellent after complete resection. Recurrences are rare, especially related to incomplete resection –12.


We describe the first reported case of a tumour deposit within the rotator cuff presenting as a bizarre, progressive, and fixed external rotation deformity of the shoulder. It is also the first reported case to our knowledge of an oesophageal primary metastasising to the rotator cuff. Malignant tumour metastasis to skeletal muscle is extremely rare, but in these rare cases, spinal and shoulder musculature tend to be affected . They arA 63-year-old right hand dominant gentleman presented to our orthopaedic clinic with a six-month history of an unusual, progressive, painful, and fixed external rotation deformity of his left shoulder Figures .One year previously, he was diagnosed with oesophageal adenocarcinoma, which was treated with a course of neoadjuvant chemotherapy followed by oesphagectomy. Preoperative staging CT had shown no evidence of distant metastases. Histology revealed a poorly differentiated adenocarcinoma with focal perineural invasion but clear resection margins. He had declined adjuvant chemoradiotherapy.The patient initially experienced reduced range of movement in his left shoulder six months following his oesphagectomy and began to notice that self-hygiene was becoming troublesome. He was referred to the physiotherapists who noted a progressive permanent external rotation deformity of his left shoulder with a fixed abduction of 30 degrees and a maximum abduction of 90 degrees. He complained of a continuous pain from the shoulder radiating to his elbow, and was slightly tender over the infraspinatus muscle bulk, although no mass was palpable. The awkward position of his arm increasingly interfered with washing and dressing. The external rotation deformity worsened and became fixed at 40 degrees, at which point he was referred to the orthopaedic clinic where he presented a great diagnostic challenge.As the shoulder pain and deformity progressed, CT and MRI were performed to aid the diagnosis. These showed a discrete mass measuring 4 × 4 × 4 cm in the infraspinatus muscle Figures .Biopsy subsequently confirmed metastatic oesophageal adenocarcinoma with the histology showing infiltration of the infraspinatus muscle by poorly differentiated adenocarcinomatous cells with calcific deposits. Further imaging revealed local recurrence of the primary. The decision was made in liaison with the patient and oncologist to have palliative radiotherapy to his shoulder to help with pain control. However, his shoulder pain deteriorated despite oral morphine and amitriptyline and necessitated a suprascapular nerve block to aid symptom control. The shoulder deformity did not improve, and the patient passed away after 10 months.Haematogenous spread of malignant tumours to skeletal muscle is extremely rare. Those patients who do have muscular deposits tend to have advanced disease with poorly differentiated primaries , as was There have been reports of renal cell, lung, and thyroid carcinomas metastasising to the infraspinatus –4. HowevThis case draws attention to the fact that metastases in the rotator cuff can give rise to unusual upper limb positional deformities; recognising this is the first step in achieving a diagnosis in such cases which are rare and previously unreported. This case also highlights the importance of the orthopaedic surgeon and oncologist liaising closely in such rare cases, keeping an open mind on the possible reasons for such a progressive shoulder deformity. The diagnostic puzzle can rapidly be solved by combining a detailed history and examination with imaging in the form of CT or MRI, as long as one recognises that tumour deposits can arise in the rotator cuff in the first instance.It is also important to recognise the appearance of such lesions on CT as such lesions tend to be incidentally found on initial staging CTs for the primary tumour. The classic appearance is that of a rim-enhancing intramuscular mass with central hypoattenuation or calcification. MRI classically provides superior soft tissue contrast to CT and such muscular tumour deposits appear as a ring of peripheral oedema around the mass , 6. InteThe infraspinatus muscle is the main external rotator of the shoulder and we hypothesise that the increased bulk created by the metastasis caused the external rotational deformity seen with our patient; the infraspinatus muscle is encased in a thick fibrous fascia which would be resistant to stretch, thereby allowing any tumour to exert direct pressure effects on the muscle itself which is incarcerated within the infraspinatus fossa. Another hypothesis is that it is usual for surrounding stromal tissue to undergo myofibroblastic conversion in the presence of neighbouring tumour and this gives rise to stiff fibrotic tissue as seen in wound healing . This coThe management of such lesions is difficult in view of their location, but as the primary histology is likely to be poorly differentiated with a poor prognosis, as in our patient, the treatment is likely to be palliative. However, further reporting of similar cases would be useful to ascertain other management strategies which obviously are customised towards the specific patient's level of disease.


Inflammatory myofibroblastic pseudotumors are initially described in the lung and various extrapulmonary sites such as the orbits, palatine tonsils, ears, gingiva, pterygomaxillary space, and periodontal tissues. These tumors rarely involve the larynx and predilection to the glottis occurs in an indolent manner. This case describes a laryngeal myofibroblastic tumor in a 46-year-old woman who presented with an aggressive tumor that extended to the floor of the mouth and the base of the tongue. Extended supraglottic laryngectomy was undertaken for the patient. The diagnosis was spindle cell proliferation with dense lymphoplasma cell infiltration compatible with inflammatory myofibroblastic tumor (Inflammatory pseudotumor or plasma cell granuloma). Definitive diagnosis was achieved with immunohistochemical (IHC) staining. We believe that further IHC studies are required to define the true nature of these tumors especially for those that behave in an aggressive pattern. Inflammatory myofibroblastic tumors (IMFT) represent a heterogeneous group of unusual pseudo neoplastic lesions that appear to encompass a wide spectrum of histopathological features ranging from plasma cell rich to a high presence of myofibroblasts ,2. AlthoA 46-year-old woman presented with symptoms of potato voice and globus sensation for 4 years with no history of trauma or previous operation. She underwent a direct laryngoscopy, which revealed a thickness of the aryepiglottic fold and vallecula on the right side with a submucosal mass at the laryngeal surface of the epiglottis. The patient underwent a conservative laser resection of the involved area. Pathology showed fibrosis only, without any presence of atypias. One and half year later, symptoms recurred. Another laser procedure was undergone but the report remained the same. In the last 2 years, tumor growth occurred so rapidly that a CO2 laser transoral resection was preformed twice.Unfortunately, endoscopic photos of the preoperative intra laryngeal surgeries do not exist. Before the open surgery, pathology showed squamous and respiratory type mucosa with superficial edema and fibrosis of the deep stroma with no mucosal epithelium atypia. CT showed a large heterogeneous enhanced mass, 30×24 mm in size, located on the right side of the base of the tongue with involvement of the genioglossus, hyoglossus, and mylohyoid muscles. There was a posterior-inferior extension of the mass to the vallecula and pre-epiglottic space; in addition to a thickened aryepiglottic fold in the presence of intact fat planes . Upon admission, the patient had a stridor at rest due to the involvement of the base of the tongue and supraglottic region. The patient underwent extended supraglottic laryngectomy due to repeated recurrence and surgeries. The surgical area was primarily reconstructed because of the unilateral mass. This mass presented as an irregular-shaped piece of rubbery, which was a firm and grayish brown tissue, measuring 3×2.5×2 cm and was sent for evaluation. Immunohistochemical staining showed spindle cell positive reaction for SMA and ALK. No reaction for CD34, AE1/3 or desmin was observed; but BcL2 showed a positive reaction in inflammatory cells .The diagnosis was spindle cell proliferation with dense lympho- plasma cell infiltration compatible with inflammatory myofibro- blastic tumor (Inflammatory pseudotumor or plasma cell granuloma). IMFT, termed by Weing et al., indicates a proliferative myofibroblastic growth. IMFT has been reported throughout the body under a variety of names and the majority of the lesions follow a benign clinical course and rarely reoccur locally. IMFT should be considered when histological findings from an inflammatory mass are inconclusive. Development of this pseudotumor in the head and neck should always be kept in mind otherwise the diagnosis could be difficult. The etiology and pathogenesis of IMFT is still a mystery. Some investigators consider it to be an immunological host response to many different stimuli; but cytogenetic and molecular studies have pointed to the possibility that at least some subsets of IMFT are, in fact, true neoplasms. However, one subset of IMFT is mostly probably associated with infection. Histologically, IMFTs are composed of myofibroblastic spindle cells that are mixed with a prominent infiltrate of lymphocytes, plasma cells, and acute inflammatory cells. The three basic histological patterns were described by Coffin et al and are as follows: myxoid, vascular, and inflammatory areas resembling nodular fascitis; compact spindle cells with intermingled inflammatory cells resembling fibrous histiocytoma; and dense plate-like collagen resembling a desmoid or fibrous scar ,8. The mIMFTs of the larynx are uncommon and carry a good prognosis but further IHC studies are required to define the true nature of these tumors and to categorize the aggressive subtypes of this tumor.


The peripheral giant cell granuloma (PGCG) is a nonneoplastic lesion commonly caused by local irritation. This report describes a 46-year-old Caucasian male who presented with a PGCG associated with a dental implant. The dental implant was originally placed in August 2012. Ten months later, the patient presented with a well-circumscribed lesion associated with and covering the implant, at which time the lesion was excised. Four months later, due to recurrence of the lesion, a deeper and wider excisional biopsy with curettage of the adjacent bone was performed. No evidence of recurrence has been reported after 12 months of follow-up. Immunohistochemistry, using the antibody CD68, was performed to investigate the origin of the multinucleated giant cells, with their immunophenotype being similar to those of other giant cell lesions, including central giant cell granuloma, foreign-body reactions, and granulomatous reactions to infectious agents. The peripheral giant cell granuloma (PGCG) is a nonneoplastic lesion, characterized by reactive hyperplasia in the presence of local irritation , 2, whicThere is little data in the literature regarding the prevalence of reactive lesions associated with dental implants and whether they develop due to mechanical or biological irritation, with the former being associated with inappropriate implant placement and the latter with poor oral hygiene. The complications that may arise in and around implant sites are numerous and may include dehiscence, mucositis, gingival hyperplasia, and the formation of a biofilm –6.This case report describes a peripheral giant cell granuloma associated with a dental implant and reviews similar cases published in the English literature.A 46-year-old Caucasian male presented to the Department of Implant Dentistry, Faculty of Dentistry, University of São Leopoldo Mandic, Campinas, São Paulo, Brazil, in August 2012, requesting implants for aesthetic and functional purposes. Titamax Ex external hexagon implants  were placed in the regions of the upper left premolar and the lower left first molar, with their respective healing abutments. In June 2013, the patient presented with a lesion associated with and covering the lower left first molar implant site. Intraoral examination showed a well-circumscribed, pedunculated, painless, purple mass measuring approximately 1 cm, rubbery in consistency. Radiographically, in the lower left molar region, the presence of an implant was observed without evidence of radiographic features that would be compatible with bone involvement . The cliIn October 2013, the patient was referred to the Oral Medicine Clinic, Faculty of Dentistry, University of São Leopoldo Mandic, Campinas, São Paulo, Brazil, with a recurrence of the lesion. A deeper and wider excisional biopsy, curettage of the adjacent bone, and application of surgical cement were performed. The biopsy was once again forwarded to the Department of Oral Pathology, Faculty of Dentistry, University of São Leopoldo Mandic, Campinas, São Paulo, Brazil. The histopathological diagnosis was peripheral giant cell granuloma. The patient remains lesion-free following one year of follow-up.μm sections were deparaffinized, hydrated, and subsequently immersed in 3% hydrogen peroxide for 30 minutes . For antigen retrieval, the slides were put into a steamer immersed in a citrate buffer (pH 6.0) for one hour at 95°C . The sections were then incubated with the primary antibody overnight at 4°C at a dilution of 1 : 1200 . The sections were then incubated with Labeled Streptavidin Biotin  for 30 minutes, stained for 5 minutes at 37°C with 3.3′-diaminobenzidine tetrachloride , and counterstained with haematoxylin . Macrophages were used as the positive control.The paraffin-embedded blocks from each biopsy were selected for immunohistochemical staining using the antibody CD68. Five Immunohistochemical staining with CD68 was strongly positive for multinucleated giant cells, as shown in When compared with peri-implantitis, reactive lesions associated with dental implants, such as PGCG, are considered rare, with only 12 cases reported in the English literature  5, 7–13–137–13.Dental implants aside, pyogenic granuloma is considered more common than PGCG; it is, therefore, extremely interesting that PGCG is in fact more common than pyogenic granuloma in the dental implant population. A search of the English literature (available for download) revealed 12 cases of PGCG and only five cases of pyogenic granuloma associated with dental implants , 14–16. Although the case presented in this study refers to a male patient, our review of the literature revealed that, as PGCG is not associated with dental implants, there is a female preference –3, 17, wIn the present review, the age range was noted to be 31–74 years, with an average age of 50.9 years, as observed by Katsikeris et al.  who descPGCG has been reported as being more common in the mandible , which cIn the cases reviewed, a recurrence rate of 46.2% was observed, with some lesions reported as recurring five times , which iSix of 13 cases recurred, three of which had initially been treated solely with curettage. Only one case was excised from the start, which also recurred. Of the seven cases that were initially excised and curetted, only two recurred. Five of the thirteen cases required surgical removal of the implant (38%).Bischof et al.  describeThe presence of peri-implant metal particles has been reported as being caused by the insertion mechanics, an inadequate abutment placement, and early removal of failing implants , all of In terms of treatment, it is important that any exacerbating factors, such as fractured prostheses and restorations, large or poorly adapted restorations, and dental calculus, should be removed. Other factors should also be considered, such as diastema, which can promote retention of food particles, orthodontic appliances, and dental implants. Following removal of the causal factor, the surgical excision of the lesion should be performed, associated with debridement of the adjacent bone, which is paramount for the prevention of recurrence .α-1-antichymotrypsin, and CD68, suggesting histiocyte/macrophage or osteoclast origin [Immunohistochemistry for CD68 was performed in order to confirm the origin of the multinucleated giant cells in the lesion. The literature is controversial in terms of the origin of these multinucleated giant cells, with speculation over a macrophage or osteoclast origin. Various studies have shown that multinucleated giant cells in PGCG demonstrate positivity for the immunohistochemical markers MB-1, vimentin, t origin , 26. Galt origin .In conclusion, reactive peri-implant lesions should be removed in their entirety in order to prevent recurrence and implant failure. In addition, clinical experience reveals that differences in opinions exist regarding whether the implant should also be removed during excision of the lesion; therefore, it is critical that these lesions are reported so as to arrive at a treatment consensus. Furthermore, it is paramount that histological examination is performed on all peri-implant soft tissue reactions in order to arrive at an appropriate diagnosis, as other neoplastic and nonneoplastic lesions such as pyogenic granuloma, central giant cell granuloma, the brown tumor of hyperparathyroidism, and malignancy must be excluded .


Primary appendiceal adenocarcinomas are extremely rare entities. Preoperative diagnosis is very difficult and is mainly based on computed tomography (CT) scan findings. Furthermore, in many cases, difficulties in establishing an accurate intraoperative diagnosis have resulted in a two-stage surgical intervention. We herein report a case of a primary appendiceal mucinous adenocarcinoma in a 67-year-old Caucasian man who presented with atypical symptoms of persistent coughing and weight loss. The chest CT showed lesions with features favorable of malignancy. Further investigation with abdominal CT and colonoscopy revealed a large tumor of the cecum expanding to the ascending colon. Typical right hemicolectomy was performed and the histopathological examination confirmed mucinous adenocarcinoma of the appendix. As some cases are accidentally discovered, the presented case describes an extremely rare first presentation of this tumor and emphasizes that the preoperative diagnosis of appendiceal cancer is challenging due to the lack of specific symptoms and signs. Primary tumors of the appendix are unusual. Carcinoids are revealed in almost 85% of the cases whereas appendiceal adenocarcinomas are very rare malignant neoplasms with about 0.12 cases per 1,000,000 individuals diagnosed annually . Apart fWe herein report an extremely rare case of appendiceal adenocarcinoma in a 67-year-old male with first presentation of persistent coughing.A 67-year-old Caucasian man presented with a persistent coughing for approximately 1 month. His symptom was not accompanied with loss of appetite, weight loss, or changes in bowel habits. The patient was smoking for about 25 years (approximately 40 pack-years) despite a 27-year abstinence from smoking. On laboratory investigation, peripheral blood counts and biochemical markers were within normal limits, except for a high rate of LDH. Serum concentrations of carcinoembryonic antigen (CEA), carbohydrate antigen (CA19-9), and alpha fetoprotein were within normal range as well.Chest computed tomography (CT) showed multiple diffuse smooth thickening of the interstitial pulmonary parenchyma as well as an enlargement of the right paratracheal lymph nodes with a maximum diameter of 2.5 cm . ContrasA colonoscopy was performed revealing a large mass in the cecum expanding in the lower portion of the ascending colon . BiopsieHistopathological examination of the resected specimen revealed a poor differentiated appendiceal mucinous adenocarcinoma  whereas There are four major histological subtypes of appendiceal adenocarcinoma: cystic, colonic, carcinoid, and adenocarcinoid. Carcinoids are the most common, comprising nearly 90% of all primary appendiceal tumors while mucinous cystadenocarcinoma is the second most common type. Primary appendiceal cancer is diagnosed in 0.9-1.4% of appendectomy specimens .Clinical presentation of appendiceal tumors usually is non-specific and misleading, thus accurate preoperative diagnosis is difficult and errors in the selected treatment can occur. Some are entirely asymptomatic. They are never suspected preoperatively and seldom intraoperatively and the diagnosis is usually made at histopathological examination of the surgical specimen or as an incidental finding during exploration for another disease . AdenocaIntestinal endoscopy, barium enema, and selective ileocolic arteriography have been used for the preoperative diagnosis but no established method currently exists. In the same pattern, the role of colonoscopy is controversial. According to a previous review, appendiceal lesions were recognized during colonoscopy in only 11% of the patients with appendiceal adenocarcinoma . DiffereTreatment of appendiceal adenocarcinoma depends on the stage of the disease. When limited in the appendix, right hemicolectomy is the treatment of choice, whereas the presence of distant metastases or peritoneal spread requires aggressive debulking followed with neoadjuvant and adjuvant chemoradiotherapy along with it . HoweverThe purpose of this report was to highlight that a suspicion of appendiceal carcinoma is fundamental, when pathology is focalized near the ileocolic valve so as to eliminate errors concerning the therapeutic strategy. In the present case, the patient’s clinical presentation was confusing and led us to initially ignore the possibility of appendiceal carcinoma. The signs and symptoms of an appendiceal adenocarcinoma were not specifically apparent, but the CT and the colonoscopy were useful to identify a correlation with the colon. However, the final diagnosis was established by the histopathological examination of the resected specimen.


Endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) has proven to be of significant value as a diagnostic method for the evaluation of esophageal mesenchymal tumors, such as true leiomyomas. Utilizing the cell block procedure, the present study reports the diagnostic approach of EUS-FNAC in two patients affected by this lesion, describing the cytological and immunocytochemical findings. Spindle-shaped elements with elongated nuclei were appreciable; moreover, the cytoplasmatic immunohistochemical positivity for smooth muscle actin and desmin strongly supported the diagnosis of leiomyoma when also taking into account the constant negativity for CD34, CD117 and S100. The differential diagnosis between spindle cell mesenchymal tumors and leiomyomas, and the clinico-therapeutic management of the latter are also discussed in the study. Mesenchymal tumors of the gastrointestinal tract are uncommon, representing only a small percentage of gastrointestinal neoplasms –7. The tEndoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) has proven itself to be a reliable method for the diagnosis of GISTs  and otheA 43-year-old male presented with dysphagia that had been apparent for 2 months. A physical examination revealed no abnormalities, and the standard serum laboratory tests were in the normal range. Computed tomography (CT) scans of the chest revealed a hypodense mass developing in the distal esophagus and causing substenosis of the lumen, which was extended to 65 mm in length, with non-homogeneous contrast enhancement . EUS exaA 39-year-old female presented with dyspepsia and esophageal reflux that had been apparent for 4 weeks. There was no weight loss, but nausea and mild vomiting were occasionally present. Upon physical examination, local peri-gastric discomfort and pain were noted. EUS scanning showed a 27.8×16.4-mm ovoid, homogeneous and hypoechoic well-delimited mass originating from the esophageal sub-mucosa . 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 a hemorrhagic background, with loose clusters or small aggregates of spindle-shaped cells  and 2B tImmunohistochemical procedures were carried out on the 3-μm serial sections, utilizing the following commercially obtained antisera : Vimentin , smooth muscle actin , desmin , CD117 , CD34 (w.d. 1:200), S-100  and Ki67 . In each of the two cases, strong and diffuse cytoplasmic immunostaining was encountered for vimentin, desmin and SMA  and 2C. In light of the microscopic examination and immunohistochemical findings, the two esophageal lesions were diagnosed as intraparietal true leyomiomas, without atypia. The patients refused surgical procedures, and were lost to follow-up subsequent to a period of 12 months.It is well known that the diagnostic yield of EUS-FNAC greatly depends on the site, size and characteristics of the target tissues, as well as certain procedural aspects ,15,18. BEsophageal leiomyomas are rare benign tumors, with a frequent asymptomatic occurrence, that do not metastasize . In factIn summary, the present study provided further indications that EUS-FNAC has great clinico-diagnostic pre-surgical value, also allowing a correct differential diagnosis of other esophageal mesenchymal/stromal neoplasias with unpredictable biological behavior to be generated by immunohistochemistry.


The present study describes the case of a 62 year-old female patient with a metastatic tumor in the right hemi-liver of >25 cm in diameter, who presented to The Affiliated Hospital of Guilin Medical University  with acute abdominal pain and severe malnutrition. Radical surgery was performed to remove the tumor by open surgery. A biopsy was not performed prior to the surgery, so the tumor was diagnosed as end-stage primary liver cancer (PLC) based solely on the character and appearance of the tumor on computed tomography prior to surgery. However, subsequent to the surgery, upon analysis by the Department of Pathology, the mass was identified as an ovarian granulosa cell tumor (GCT). These tumors occur rarely, representing only 2–3% of all ovarian tumors, and are well known for late recurrences, with an incidence of 25–30%. As metastasis of the liver with GCT is extremely rare and the data available on the subject is limited by the small number of studies, and due to the absence of a biopsy report prior to surgery, the patient was initially misdiagnosed with PLC. However, despite this misdiagnosis, a good result was obtained, as the patient was later diagnosed with GCT following a detailed pathological examination and was treated with rational therapy. The performance status and quality of life were significantly improved, and the patient remains disease-free at one year post-surgery. Granulosa cell tumors (GCTs) are rare sex cord-stromal tumors, and are classified into either adult or juvenile forms and the median age at presentation, for the adult form is 50 years. GCTs are low-grade neoplasms, whose early symptoms are uterine bleeding and pain, in addition to pressure symptoms with a palpable mass . GCTs haA 62-year-old female was admitted to The Affiliated Hospital of Guilin Medical University  in 2013 with acute abdominal pain and severe malnutrition. Previously, in 1986, at 35 years of age, the patient had undergone a total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH+BSO) for a stage 1 grade 1 adult GCT of the ovary in the Second Hospital of Guangxi Province . The patient did not receive any adjuvant chemotherapy and remained disease-free until 2013. Upon admittance to hospital in 2013, the blood test for the α-fetoprotein (AFP) tumor marker was negative. A computed tomography (CT) scan of the patient was performed and reviewed. A 2.5-mm slightly enhancing mass was observed in the tumor of metastasis; the tumor was ~10×15×25 cm in size . A biopsOvarian cancer has the fifth highest mortality rate of all cancers in females, after breast, bowel, lung and uterine cancer, representing 5–6% of cancer-related mortalities . In totaIt may be difficult to differentiate GCT from PLC prior to surgery. In the present study, the patient underwent a TAH+BSO for stage 1 grade 1 GCT in 1986, and no adjuvant chemotherapy was administered. The patient remained disease-free for >20 years until recurrence, which presented as abdominal pain and a large mass in the liver. Consequently, it is important that patients with GCT should be followed up regularly, even if the disease-free interval is long, and that adjuvant treatments may be reserved for patients with large residual or inoperable tumors. The literature on GCTs commonly advocates the use of radiofrequency ablation for hepatic metastases from GCT . HistoriIn the present study, the patient initially presented in 2013 with acute abdominal pain and severe malnutrition. A biopsy was not performed prior to surgery. A metastatic tumor with a maximum diameter of >25 cm was detected, and even though the mass was misdiagnosed as a PLC, surgery was performed to remove the tumor. The patient made a good recovery and remains disease-free at present.Although the present reported case is rare, it indicates the role of surgical resection for hepatic metastases of GCT, particularly in patients with a record of a long disease-free period. Doctors, and specifically hepatobiliary surgeons, should be aware that patients with GCT should be regularly followed up, even if the disease-free interval is long. Hepatic resection for GCT may significantly improve a patient’s survival time and quality of life.


Aneurysmal Bone cysts (ABC) are extremely rare in the head and neck region and even rarer in sinuses. ABC is a benign multicystic mass that is locally-destructive and rapidly expandable. Hemorrhagic fluid content (like in this case) and septated appearance are the characteristic feature of ABC. Established treatment options for ABCs include sclerotherapy, embolization, radiotherapy, simple curettage, surgical excision, or a combination of methods. In this article, a 5 year-old boy with a recurrent nasal mass is presented. The patient was finally diagnosed with this rare entity: ABC of the paranasal sinuses. The patient was treated through complete surgical removal.ABC can be considered as a rare differential diagnosis of recurrent nasal hemorrhagic mass in a pediatric population. Aneurysmal Bone cysts (ABC) are vascular tumors that show bony expansion. ABC rarely occurs in the head and neck region and even less so in the maxilla and sinuses ,2. ABC iIn this article, a 5 year-old boy with a recurrent nasal mass is presented. The patient was finally diagnosed with this rare entity: ABC of the paranasal sinuses. The patient was a 5-year-old boy who was presented to our clinic with symptoms of nasal stiffness, snoring, and right nostril purulent rhinorrhea since 3 months. His parents also explained that he had progressive proptosis of the right eye during this time. He had no pain, visual disturbances, or rhinorrhagia. Three days before admission, he had a discharge from his eye. Adenoidectomy had been done one month ago, but his symptoms had partially recovered; and purulent rhinorrhea remained after his previous surgery.He complained about repeating common colds in the last eight months, which were treated by antibiotics and symptomatic medication; however, their effect did not completely erase the symptoms. He had no previous medical history.Nasal examination revealed a right and left firm nasal mass, extending to the inferior conchae with a non-bloody nasal discharge. Ophthalmological examination revealed limited lateral gaze on the right eye. His laboratory data contained: WBC: 8000 , Hemoglobine: 10.8, Hematocrite: 34.4, MCV: 75.6, MCH: 23.7, MCHC: 31.4, Platelet: 371000, PT: 13, PTT: 31, INR:1.A spiral CT scan, without contrast, of the orbit was performed. A large well-defined heterogeneous mass lesion (5-6cm) in the right nasal cavity, extending to the nasopharynx and involving the entire sphenoid sinus, was observed. The lesion showed hypodense foci and remodeling of bones was seen. The mass compressed the maxillary sinuses  resulting in a compressive effect on the right inferior muscle, medial rectus muscle, and optic nerve. Obstruction of the drainage of the antral, ethmoid, and sphenoid sinuses; in addition to the right nasolacrimal duct was seen. No destruction of bone was seen. There was a dense shell around the mass, which seemed to be calcium. The lesion had a sinonasal origin. Erosion of the sella turcica was seen; but the mass did not extend to the brain. These findings suggested a slow growing mass. Radiological findings suggested ossifying fibroma or angiofibroma .MRI of the nasal cavity with Gadolinium was done. It revealed that the nasal cavity had been replaced by an expansile non-homogenous mass lesion. Cystic lesions (of multiple and variable size) between solid parts were evident. No intracranial extension was seen. The lesions extended into the right orbit. Following administration of contrast media enhances in solid part. MRI suggested an expansile multiple cystic lesion with a bony origin such as Aneurysmal Bone cyst, Esthesioneuroblastoma, Hystyocytosis or parasitic cyst . Through endoscopic approach, a midline mass extending from the posterior septum to the lateral nasal wall was observed. After opening of the nasal septal mucosa, through shaver necrosis, the tumor was drained. In the right side, the right and left ethmoidal sinus was opened and cleared until the medial wall of the orbit. In the nasopharynx, the tumor was located anterosuperiorly to the skull base, and was cleaned. Except for the mid-part of the fovea ethmoidalis, other parts of the skull base were free of tumor. Fovea ethmoidalis had bony erosion and was cleaned. After surgery, the patient had a decrease in Hemoglobin level from 108 to 5.9, so 350 cc packed cell were infused. The patient was in ICU for two nights and was then discharged on the third day.Pathological study showed macros- copically soft pink to yellow gray tissues. During microscopic examination, there was an extensive vascular proliferation in a fibrosis context, with macrophages contained in hemocydrine and hemorrhage. The diagnosis of Angiofibroma was suggested. After five months, the patient was again referred with symptoms of proptosis, nasal stiffness, bloody purulent rhinorrhea, and vertigo. Visual acuity decreased and there was optic disc swelling in the left eye, and pallid optic disc after swelling in the right eye. New facial CT scan was done and there was an expansion observed in the posterior aspect, from the ethmoidal until the sphenoidal sinus accompanied with soft tissue density. It caused anterior displacement of the posterior wall of the right maxillary sinus. Displacement of the medial wall of both the frontal bone and muscles to the lateral side was also seen. Aneurysmal Bone cyst, Burkitt lymphoma, Hystiocytosis were the suggested differential diagnosis. CT scan of abdomen, thorax, and neck were normal .An endoscopic surgery was done for the second time. There was a cystic mass, which was aspirated. The aspirated fluid was an old brown bloody liquid. The lateral wall of the mass was then excised, and the mass of the sphenoid and skull base was removed. Dura, skull base, and perineural spaces were cleaned from the tumor.A new pathological study revealed diffuse mesenchymal proliferation, with long nuclei and moderate focal pleomorphism. There was bony and vascular extension .Immunohistochemical study revealed Vimentin: positive, B Catenin: positive, CD117: positive, SMA: positive, Desmin: negative, CD34: negative. Once again, these finding suggested cellular angiofibroma. Due to the mismatch between clinical, radiological, and pathologic findings, the pathological slides were consulted with different pathologists. They reported proliferating stromal cells with intervening scattered multinucleated giant cells of osteoclastic type in some areas. The lesion is vascular and contains blood field spaces surrounded directly by stromal and giant cells. Deposition of osteoid is also present near the cavernous spaces. They reported Aneurysmal Bone cyst, nasal, and paranasal sinus involvement.The patient was referred to a pediatric oncologist for further follow up. They evaluated the whole body to rule out metastases of sarcoma, and also evaluated the bone marrow. Bone marrow aspiration was normal. In the abdominal sonography, there were multiple para-aortic lymph nodes (maximum size was 4 mm). Other images revealed no metastases or any evidence of other tumors.The patient was followed clinically for any signs or symptoms of recurrence. The patient has not had any problems so far (six months after surgery).This article presents a rare case of ABC in the paranasal sinuses, which was diagnosed as angiofibroma during the first evaluation. The radiological and pathological findings suggested that this tumor, and surgical findings were compatible with ABC. ABCs can be primary or secondary to another skeletal lesion. Secondary ABCs may be related to fibrous dysplasia, giant cell tumors, chondroblastoma, chondromyxoid fibroma, non-ossifying fibroma, fibrous histiocytoma, osteoblastoma, and osteosarcoma . It can Few cases of ABC in the sinuses were reported up to now -9. SkullHemorrhagic fluid content (like in this case) and septated appearance is the characteristic feature of ABC . ABC is ABCs have some characteristic features in CT and MRI. In CT images, erosion, thinning of cortex, and ridges in the bony walls, with 30% Fluid levels on bone, are seen. In MRI images, well-defined heterogeneous signal intensity lesion on T1, hypointense capsule, and a homogenous increase in signal intensity on T2 is observed. During angiograpy, the blood supply to these vascular lesions can occasionally reveal arteriovenous shunts . This caAneurysmal Bone cyst (ABC) is extremely rare in sinuses. It can be diagnosed by specific radiographical findings containing an eccentric lytic lesion with an expanded and remodeled "blown-out" or "ballooned" bony contour of the affected bone, in addition to a delicate trabeculated appearance, and fluid-filled spaces on CT scans and MRI. ABC is a benign multicystic mass that is locally-destructive and rapidly expandable. Even in childhood this should be considered as a differential diagnosis.


Type IV sacrococcygeal teratoma is a rare pediatric tumor that is confined to the presacral area with no external component. The signs and symptoms often arise due to mass effect and compression of adjacent organs. Urinary retention is an uncommon presenting symptom in these patients. A wide spectrum of imaging findings may be encountered in cases with sacrococcygeal teratoma because of variability of tumor size and components. We hereby present a unique magnetic resonance urography finding in a type IV sacrococcygeal teratoma which caused bladder displacement. A meticulous and complete resection of tumor with special attention to the pelvic plexus led to preservation of normal voiding function and normal bowel function in this patient.  Sacrococcygeal teratoma (SCT), though the most common germ cell tumor in children, is a rare entity occurring in 1 in every 35000 to 40000 births .Different imaging techniques play an important role in diagnosis and evaluation of SCTs, especially in type IV Altman SCTs that are not visualized externally. However, a wide spectrum of imaging findings may be encountered, given the wide variety in these tumors and different solid or cystic components. We hereby present unique magnetic resonance imaging (MRI) and magnetic resonance urography (MRU) findings in a boy with type IV SCT and we also briefly discuss the management of this patient.A 32-month-old boy presented to our pediatric urology center with multiple episodes of urinary retention in the previous month. He was otherwise healthy and did not have any history of previous surgical or medical conditions. Physical examination revealed a large nontender mass in the lower abdomen. Routine laboratory data were all within normal limits. Plain radiography of pelvis showed a mass composed of soft tissue and calcifications in its lower part, as well as a short sacrum. Ultrasonography (US) revealed a pelvic mass with both cystic and solid components and also bilateral hydronephrosis.2 large presacral mass with septated cystic and solid components arising from coccygeal area without any connections to spinal canal. The lesion caused complete displacement of the bladder anteriorly and to the left side. The right ureter had an S-shaped course around the inferior portion of the mass to enter the shifted bladder (Computed tomography (CT) was taken in the district hospital before patient's admission to our center. It confirmed previous findings but it did not delineate the exact relationship of the mass with spinal column to narrow down the differential diagnoses. MRI and MRU were performed to investigate the characteristics of the mass and its possible connection to the spinal cord. MRI showed a 144 × 63 mm bladder . VoidingSurgical resection of the tumor was recommended. Prior to incision, a urinary catheter and a rectal tube were fixed to guide the surgeon. First, in prone (jack-knife) position a posterior sagittal incision was made. With guidance of rectal tube the mass was carefully separated from rectum, but due to its large volume complete excision through posterior sagittal incision was not possible. Subsequently, the patient's position was changed to supine and an anterior midline incision was made to access the tumor from abdomen and to complete the excision . ComponeHistopathological investigations of the mass showed a cystic neoplasm composed of various mature tissue components including skin, pilosebaceous glands, well-developed respiratory and gastric epithelium, and glial tissue with numerous psammoma bodies. Based on pathological studies the final diagnosis was confirmed as mature type IV SCT.Type IV SCTs are the least common type in children and may cause diagnostic difficulties since they are not visualized externally and the diagnosis is largely dependent on imaging studies. These tumors are usually asymptomatic but if symptoms arise, they are mainly caused by tumor's mass effect on adjacent organs. This can lead to bowel and bladder dysfunction , venous or lymphatic obstruction, or even lower extremity paralysis , 4. In sRisk of recurrence might be high especially in immature and malignant SCTs and in case of incomplete resection of tumor, nonresection of coccyx, and spillage of tumor components during surgery . The surWe assume that magnetic resonance imaging is an excellent modality for precise anatomical diagnosis and surgical planning in SCTs. The imaging findings in SCTs are widely variable based on tumor size, location, and constitution. Careful and complete tumor resection with special attention to neural structures is needed to reduce risk of recurrence as well as functional urologic sequels.


Congenital cutis laxa is a genetically heterogeneous condition presenting in the newborn with loose, redundant skin folds, decreased elasticity of the skin, and general connective tissue involvement. A 2-day-old full term neonate with congenital cutis laxa presented with respiratory distress. Investigations revealed huge hiatal hernia. Patient underwent loose Nissen’s fundoplication. In postoperative period patient developed intussusception which was manually reduced at re-surgery. Cutis laxa is an uncommon disorder characterized by degenerative changes in the elastic fibers; it can be inherited  or acquired.[1] Postoperative intussusception is reported in various kinds of operations especially involving retroperitoneal dissection.[2] We herein report a neonate of cutis laxa with hiatus hernia who developed postoperative intussusception.A-2-day-old full term and normally delivered male neonate with features of congenital cutis laxa was referred with severe respiratory distress . His facial features included; a broad flat nose with everted nostrils, a short columella, a long upper lip, and everted lower eyelids with large ears. His skin was lax and had a hoarse cry. Hip joint was lax. Physical examination revealed slight tachypnea. Arterial blood gas and electrolytes were normal. Thoraco-abdominal x-ray demonstrated coiled nasogastric tube (NGT) above diaphragm . Barium swallow showed stomach in the thoracic cavity, and echo was normal. He underwent surgery at 6th day of life. Operative findings revealed an intact diaphragm with a huge combined sliding-rolling (mixed) hernia, containing an upward dislocation of both the cardia and the gastric fundus into thoracic cavity. The gap of the esophageal hiatus was 3 cm. Hiatal hernia was repaired along with loose Nissen’s fundoplication. Immediate postoperative period was normal however he developed abdominal distension on 3rd postoperative day. Abdominal ultrasound revealed only bowel distention. Contrast study with oral gastrographin showed target singe. He was re-explored through pervious incision. An ileoileal intussusception found which was manually reduced . Patient is doing fine on follow-up.Cutis laxa is an acquired or inherited skin disease characterized by wrinkled, inelastic skin. Congenital problems of elastin synthesis and structural defects of extracellular matrix proteins have been described leading to the decreased elasticity and redundant sagging skin.[3] The autosomal recessive cutis laxa is highly heterogeneous with organ involvement as, lung atelectasis and emphysema, diverticulae of the gastrointestinal and genitourinary organs and vascular abnormalities . Diminution of elastic fibers throughout the dermis and abnormal elastin components by electron microscopy are pathognomonic.  Autosomal dominant cutis laxa are often recognized in beginning of childhood with a mild form of cutis laxa with systemic involvement.[1] Vascular abnormalities include arterial aneurysms leading to severe heart failure.[6]. Our case was a congenital cutis laxa that demonstrated vomiting, tachypnea and tachycardia at the birth. He had no family history of this disease.Kothari et al reported the first infant with cutis laxa coupled with multiple hernias and cardiac disorder.[7] Patient had para-esophageal hernia, right gluteal hernia and ventricular septal defect. He was operated in infancy period .Our case presented on first day of life with respiratory distress and underwent surgery in neonatal period.Postoperative intussusception may develop in a number of abdominal and extra-abdominal surgeries. It has special predilection for surgeries with retroperitoneal dissection.[2] It is mostly ileo-ileal as in the index case.Source of Support: NilConflict of Interest: None declared


A one-month-old male terrier dog was referred in shock status with a history of anorexia, tachypnea, abdominal distention and progressive weight loss. Auscultation of right side of the lungs found enhanced respiratory noises. The thorough auscultation of the opposite side of the chest revealed the presence of typical intestinal sounds. Cardiac auscultation revealed muffled heart sounds and a diminished palpable precordial cardiac impulse was evident. The radiograph showed the presence of gas within the bowel in abrupt contrast to the adjacent structures of soft tissue opacity. Conservative treatment was failed and the animal died. At necropsy, cranial displacement of abdominal viscera into the pericardial sac was seen. A definitive diagnosis of peritoneopericardial diaphragmatic hernia was made. Although congenital pericardial diseases are rare in dogs, awareness of the clinical manifestation of these kinds of defects combined with early use of available imaging modalities can yield a preoperative diagnosis. An embryonic development defect of the dorsolateral septum transversum in the so-called sternocostal triangle structure can cause this developmental abnormality.2,3 Other congenital anomalies such as hydrocephalus, umbilical hernias, sternal defects, cranial midline abdominal hernias, abnormal swirling of hair on the ventral abdomen, intra-cardiac defects, and pulmonary vascular can be associated with this anomaly,4-6 whereas, umbilical hernias being the most frequent finding.3 Peritoneopericardial diaphragmatic hernia has been reported in dogs, cats, rabbits, a donkey, and a calf.4 Generally, Weimaraners are mentioned among the dog breeds that show a predilection for this disorder, as PPDH accounts for 0.5% of their congenital cardiac diseases.3 Possible potential causes for PPDH occurrence include malformations and teratogen-induced factors.7 Peritoneo-pericardial diaphragmatic hernias are rare but should be included as a differential diagnosis in dogs with an enlarged cardiac silhouette. The main tool for diagnosis of congenital diaphragmatic hernia is radiography, whereas treatment is surgical.1,8,9 These hernias are also incidental findings at necropsy.10-12 This report describes the post mortem findings, clinical and radiological features of a rare case of peritoneopericardial diaphragmatic hernia in a terrier dog.Congenital pericardial diseases are rare in dogs and cats and most of them have been reported as incidental findings on post-mortem examinations. Peritoneopericardial diaphragmatic hernia (PPDH) is the most common congenital defect involving the pericardium of dogs and cats.A 1-month-old male terrier dog was referred with a history of anorexia, tachypnea, abdominal distention and lower body-weight gain comparing its littermates. Body temperature, pulse rate, and heart rate were considerably increased and the animal was in shock status. The mucous membranes were slightly cyanotic and auscultation of right side of the lungs found enhanced respiratory noises. The thorough auscultation of the opposite side of the chest revealed the presence of typical intestinal sounds. The evaluation of abdomen was negative for fluid accumulation or palpable mass. Cardiac auscultation revealed muffled heart sounds and a diminished palpable precordial cardiac impulse was evident. Conservative treatment was initiated with intravenous Ringer’s solution and hydrocortisone. Laboratory examination revealed leukocytosis and tendency to left shift to metamyelocytes. Radiological evaluation was performed in lateral projection. The radiograph showed the presence of gas within the bowels in abrupt contrast to the adjacent structures of soft tissue opacity. An indistinguishable outline to the ventral diaphragmatic surface and the caudal silhouette was produced by the communication between two structures . Despite13 whereas, this case was characterized by significant clinical signs. The most part of patients suffering from diaphragmatic peritoneopericardial hernia are identified within the first years of life (48.0%), but a relatively high percentage is identified later, after 8 years of age (6.0%),3,13-15 In summary, the low incidence of peritoneopericardial diaphragmatic hernia should not be the cause of omission of the condition in differential diagnosis when consistent clinical signs are present. Thus, reporting similar cases could yield useful information about the diagnosis and treatment of this anomaly.Clinical signs of peritoneopericardial diaphragmatic hernia are not specific and depend on the degree and nature of herniation. The most frequent clinical signs involve the digestive tract  and the respiratory system . Abdominal discomfort or swelling, shock and collapse occur less frequently,of life 4.0%, but