Acute appendicitis is the most common acute surgical condition of the abdomen. Diagnosis is made based on full clinical history and examination as well as supported by a routine blood investigation and urine test. Prompt diagnosis and surgical referral may reduce the risk of perforation and prevent complications. The mortality rate of non-perforated appendicitis is less than 1 percent. Perforated appendicitis is associated with a higher mortality rate - as high as five percent and may be particularly more in extreme of age group attributed to delay in clinical presentation or diagnosis in the younger group and multiple co-morbidities in the elderly group. The aetiology is unknown. It may be linked with lack of fibre, familial tendency, or viral infection. It may be precipitated by faecaliths. The commonest site of the appendix is retrocaecal.We report a case of a 46 year old male who was admitted under the surgical service in Mid-Western Regional Hospital, Limerick with suspected appendicitis which turned out to be a perforated caecum, a rare complication of an acute appendicitis. We performed a literature review comparing two main approaches - right hemicolectomy and primary closure with omental patch - discuss and highlight their differences as well as a guide to its management.There are limited studies to compare these two surgical options in the literature. A larger prospective study is needed to compare both approaches and long term outcome. Acute appendicitis remains the most common reason for intervention in acute abdominal pain. Diagnosis is made based on full clinical history and examination as well as supported by a routine blood investigation and urine test. It is a common condition can be difficult in making a diagnosis when the clinical picture is borderline suggestive of acute appendicitis. Especially in children, acute Meckel's diverticulitis must be kept in mind, as the clinical picture is indistinguishable from acute appendicitis. Perforation of a large bowel is associated with severe acute appendicitis but further surgical management of this condition uncommonly described in the literature. We highlighted this question and performed a literature review to compare two possible surgical approaches faced by surgeons.A 46 year old man presented with a day history of sudden onset of right iliac fossa pain associated with nausea, fever, and anorexia. No urinary and bowel symptoms. There was no significant past surgical or medical history. No history of recent travel and family history of colitis or inflammatory bowel disease. On physical examination, his temperature was 39.4 degree Celsius, pulse rate 91 beats per minute, blood pressure 159/80 mmHg, respiratory rate 20. His abdomen was not distended but tender in the right iliac fossa with some voluntary guarding. No rebound tenderness was elicited on examination. Rovsing's sign was positive.9/L, Hb 13.7 g/dl, Platelet 242 109/L. Na 137 mmol/L, K 3.8 mmol/L, urea 4.8 mmol/L, creatinine 92 mmol/L, amylase 24 IU/L. Urine Microscopy - negative for urinary tract infection, leucocytes < 10/ul and red cell < 10/ul.Full blood count shows elevated WBC 19.91 × 10Plain film of Abdomen and Chest X-Ray were not remarkable Figure  and 2. DOperation: Intravenous antibiotics were commenced pre-operatively. An extended McBurney's or grid iron incision was made. Dissection of the appendix was carried out with some difficulties and approximately 50 mls of pus found in the peritoneal cavity around the appendix. There was a large 3 × 3 cm caecum perforation seen at the base of the appendix Figure . A thoroPost operative progress. Inflammatory markers were responding with intravenous antibiotic. No further spiking temperature. The drain was removed postoperative day 5 and patient was discharged the next day. The histolopathology of the appendix showed acutely inflamed appendix with periappendiceal abscess formation. The epithelium shows reactive/reparative changes. No malignancy is seen.Appendicitis perforations, commonly occur at the tip of the appendix, are associated with the presence of a faecolith on CT scan and not the anatomical location of the appendix  as previously thought . PerforaWhile omental patch repair is a common surgical approach to management of stomach and duodenum perforation, there are only few reports in the literature that compare two very different surgical approaches - omental patch with primary repair vs right hemicolectomy. In the presence of an uncomplicated perforation, absence of severe infection, and well controlled localized haemostasis - a less invasive surgical approach with post operative intravenous antibiotics would be the management of choice.Right hemicolectomy carries a higher morbidity and mortality but it is generally recommended only in selected cases - severe inflammation, torsion, haemorrhage, and inflammatory mass or caecal neoplasm found intraoperatively . The preA caecum perforation is a very rare identity and so far only nine case reports have been published Table . The mosAlthough right hemicolectomy may be the conventional approach in some cases of caecal perforation, however, in a highly contaminated case as such in this scenario would have a significantly higher postoperative complication likely secondary to infection or systemic septicaemia. Therefore, the decision for a primary repair of the perforation was carried out.A primary hemicolectomy in perforated lesion of the caecum is recommended but there have been no recent studies comparing this approach with primary caecum repair with omental patch. A larger prospective study is needed to compare both approaches and long term outcome.The authors declare that they have no competing interests.MW drafted the manuscript, searched the literature and the findings, manuscript writing & editing and submission of the manuscript. SAN critically reviewed the manuscript. Both authors read and approved the final manuscript submission.


A 2.5-kg girl was born with a growth inside the mouth associated with feeding difficulty. There was no respiratory difficulty. Antenatal ultrasound did not detect any abnormality. On examination a round, 3x2 cm wide base, uneven, lobulated, pink-colored soft tissue mass was protruding into the mouth. Precise tumor attachment could not be appreciated during the initial examination . Mass was mixed in consistency with firm and cystic areas on palpation. Adjacent tissues appeared normal on examination. She was booked for excision which was done with an elliptical incision at the base of the lesion. Mucosal defect was left open. There was minimal blood loss. Postoperative recovery was uneventful. The neonate was discharged home on the following day. Excised tissue was sent for histopathological examination. The microscopic examination revealed mature teratoma with mature stratified squamous epithelium, mucinous gland and mesenchymal tissue scattered throughout . A baseline alpha fetoprotein level was normal. Patient was advised for regular follow-up to detect any recurrence.Oropharyngeal teratomas represent 2% of all teratomas. Epignathus is a term used to describe teratomas protruding from the mouth. These tumors arise from the soft or hard palates in the region of Rathke’s pouch. They generally fill the oral cavity and protrude out through the mouth. Pharyngeal teratomas arise from the posterior aspect of the nasopharynx. Cheek is the least mentioned site of teratoma in literature. Even after extensive search we could retrieve only three cases of teratoma arising from cheek or buccal mucosa [1-3]. Although most of these tumors are benign, they frequently cause significant airway and esophageal obstruction in the perinatal period and thus potentially fatal. Large tumors can interfere with fetal swallowing and produce polyhydramnios, cause severe respiratory distress at birth, and may lead to stillbirth [4]. Prenatal ultrasound is a reliable diagnostic tool for detecting these lesions as early as 21 week gestation, thereby allowing for careful arrangement of the time, mode, and place of delivery. Approximately one third of prenatally diagnosed cases are complicated by maternal polyhydramnios resulting in high incidence of preterm labor and delivery. Serial amnio-reduction may be required to prevent this complication . At birth, death may result from tracheal compression or deviation and inability to intubate. Prenatal diagnosis permits cesarean section and establishment of an airway before the cord is clamped. This is done by employing an EXIT (ex utero intrapartum treatment) procedure. Because precipitous airway obstruction may occur due to hemorrhage inside the tumor, tracheal intubation is indicated in all patients, regardless of the presence or absence of symptoms [5-7].Source of Support: NilConflict of Interest: None declared


Ewing's sarcoma of the calcaneus is rare. About thirty cases with calcaneus involvement have been reported in the literature. Talus skip metastases have rarely been described in the available literatureWe report a case of a 14-year-old Moroccan boy, who presented with Ewing's sarcoma of his right calcaneus, diagnosed by swelling of the calcaneus evolving over a year. Radiography, computed tomography and magnetic resonance imaging showed an important tumoral process of the calcaneus and talus skip metastases. The diagnosis was confirmed with histology after a biopsy. In spite of amputation and postoperative chemotherapy, our patient died six months later due to secondary respiratory distress after lung metastasis.Imaging, especially magnetic resonance, is important in the diagnosis of Ewing sarcoma and skeletal skip metastases. Treatment of Ewing's sarcoma consists of chemotherapy, radiation therapy and surgical resection depending on the stage and extent of the disease. With the exception of lesions in the calcaneus, the prognosis for disease-free survival of Ewing's sarcoma of the foot is excellent. Ewing's sarcoma is a rare malignant bone tumor that may affect any bone, usually occurring in long bones, pelvis and ribs, with only 3-5% of cases in the bones of the hands and feet . It is aA 14-year-old Moroccan boy presented with painful swelling of his right foot of 12 months duration. A general examination was unremarkable, while local examination revealed a diffuse swelling involving his right ankle joint and foot. The overlying skin was normal. The swelling was tender and mobility at the joint was restricted. Hematological and biochemical investigations revealed a normal hemogram and normal liver and renal function tests.Anteroposterior Figure  and lateA biopsy was performed and histopathology showed a malignant small round-cell tumor, identified as Ewing's sarcoma at immunohistochemistry study. Chest radiography and liver ultrasonography excluded the presence of any distant metastases. Our patient started neoadjuvant chemotherapy and underwent a below-knee amputation. Postoperative histology confirmed the diagnosis. Our patient remained disease-free for six months after diagnosis. Based on these findings, a diagnosis of Ewing's sarcoma of the calcaneus was made.Ewing's sarcoma is a rare malignant neoplasm, predominantly affecting young patients of the ages five to 20 years. It involves the diaphyses of long bones and occurs less commonly in flat bones . ClinicaCook listed 29 cases of Ewing's sarcoma of the calcaneus in the literature since 1921 . These rAccording to a retrospective study concerning 235 patients with non-metastatic Ewing's sarcoma of the bone, 15 patients were identified with a skip lesion at diagnosis. However, the skip lesions were located in adjacent juxta-articular bone in only two cases .The radiographic features of Ewing's sarcoma in our case were those of classic Ewing's sarcoma: a permeative, lytic and condensed lesion with cortical destruction, aggressive periosteal reaction, large extraosseous soft-tissue component and often sclerotic reaction ,6. In spCT can reveal a soft-tissue mass of the foot, such as permeative lytic lesions of the bone with aggressive periosteal reaction and cortical destruction, but the distinction between osseous remnants, reactive changes and tumor matrix can sometimes be challenging . Bone scT2-weighted MRI cannot adequately distinguish tumor from necrosis, and lesion boundaries are frequently overestimated because of the presence of edema and hemorrhage . The enhThe imaging features of local spread of Ewing's sarcoma, involving small bones to adjacent bones, have not been described in the recent literature. It wasn't possible to determine the exact local extent of the tumor by means of conventional radiography and CT ,9.Due to its superior contrast resolution and multiplanar capabilities, MRI is more sensitive than other imaging techniques, especially for the investigation of tumor spread to bony structures and bone marrow. MRI should always be performed in the analysis of Ewing's sarcoma since it allows accurate evaluation of the tumor extent, which is decisive for treatment .Skip lesions in patients with otherwise non-metastatic skeletal Ewing's sarcoma may be of the same importance as the molecular detection of marrow metastases, and possibly confer a worse prognosis. Newer imaging modalities like positron emission tomography-computed tomography and careful staging work-up may indicate that skip metastases in Ewing's sarcoma are more common than previously suspected ,10.This case report confirms that the routine radiological management of Ewing's sarcoma should include radiography and MRI of the affected region, together with whole skeleton bone scintigraphy and CT of the chest. MRI is essential in the determination of the true extent of the tumor. It is important to bear in mind that early recognition of an unusual appearance and location of Ewing's sarcoma is necessary for its adequate treatment.CT: computed tomography; MRI: magnetic resonance imagingWritten informed consent was obtained from the father of our patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.HJ, ZB and HE made, analyzed and interpreted our patient's imaging examinations. MM and TF are the traumatologists whom operated on our patient and made major contributions to the manuscript. The manuscript was prepared by HJ under the supervision of OE and AO. All authors read and approved the final manuscript.


Adenoma malignum (AM) of the cervix is a rare disease and it is difficult to diagnose due to the deceptively benign appearance of the tumor cells. These lesions have mucin-rich cystic lesions and are usually situated deep in the cervix. Since AM is very rare, standard screening tests, diagnostic tools and treatments have not yet been established. Radiologically, it mimics multiple nabothian cysts as a benign-looking tumor. Histologically, AM is a well-differentiated adenocarcinoma and could be misdiagnosed as a benign lesion. These findings make a preoperative diagnosis of AM difficult and can result in surgery being performed based on a misdiagnosis. We report here on four cases of pathologically confirmed AM. Adenoma malignum (AM), also called minimal-deviation adenocarcinoma, has been reported in the literature since Gusserow first described a very highly differentiated adenocarcinoma of the uterine cervix in 1870. The worA 54-year-old woman presented at our hospital with several months of leukorrhea and menorrhagia. Physical examination revealed normal external genitalia and a large amount of clear mucoid fluid in the posterior vaginal fornix. Her cervix was grossly normal in appearance except for the profuse mucoid discharge. Her uterus was normal in size, shape and consistency. No adnexal masses were palpable. Transvaginal sonography showed several small intramural myomas and nabothian cysts. The patient underwent a total abdominal hysterectomy and bilateral adnexectomy. Pathologic examination of the specimen revealed AM of the cervix  revealed multiple cystic lesions of the cervix that were of high T2 and a smooth marginated mass around the bladder that was of low T2 isosignal intensity .AM: adenoma malignum; BA: bilateral adnexectomy; CCRT: concurrent chemoradiation therapy; CTx: chemotherapy; DOD: died of the disease; ERT: external radiation; NED: no evidence of disease; PLND: pelvic lymph node dissection; RH: radical hysterectomy; TH: total hysterectomy.The authors declare that they have no competing interests.EYK wrote the initial draft. SYH, JSP and SJL performed the surgery and helped collect clinical information. SYH and SWB designed the study and helped write the manuscript. All authors read and approved the final manuscript.


Unicornuate uterus with noncommunicating rudimentary horn occurs due to incomplete fusion of mullerian ducts. Pregnancy in this horn is a rare phenomenon usually resulting in rupture during second trimester of pregnancy. Prerupture diagnosis of pregnancy in rudimentary horn with ultrasonography is technically difficult, with sensitivity of 30%. We report a case of ruptured non-communicating rudimentary horn at 19 weeks in a woman with previous Cesarean delivery. She had a routine malformation scan in which diagnosis was missed. Later she presented to emergency in shock, with massive hemoperitoneum and ruptured horn. So a high index of suspicion is required to save this catastrophic event and associated maternal morbidity and mortality. In our opinion, routine excision of rudimentary horn should be undertaken during nonpregnant state laparoscopically. However, those women who refuse should be adequately counseled regarding potential complications and if pregnancy occurs in rudimentary horn, first trimester laparoscopic excision should be done. Unicornuate uterus with a rudimentary horn is a rare mullerian anomaly that has a high incidence of obstetric complications that include ectopic pregnancy in the rudimentary horn . PregnanA 26-year-old woman gravida 1, para 1 came to emergency with severe lower pain abdomen and vomiting since last two hours. She was 18 weeks pregnant and her routine malformation scan done one week back was normal. Her previous delivery was by Cesarean section for transverse lie. She denied history of dysmenorrhoea or of pain earlier in previous pregnancy. The patient had no significant medical or surgical history. No records of previous cesarean section were available. On examination patient was in hypovolemic shock with severe pallor, hypotension, and tachycardia. The abdomen was tense and symphysiofundal height was 24 weeks. Her bowel sounds were normal. On pelvic examination cervix and vagina were healthy, there was no bleeding through os, and size of uterus could not be made out due to intense guarding. Per rectal examination was within normal limits. Immediately two large bore intravenous cannulas were inserted, one liter of fluid was rushed, patient was catheterized (she passed 50 mL of clear urine), and urgent investigations and cross match was sent for four units of blood. Her hemoglobin was 4.6 g%, one unit blood was rushed, and after stabilization urgent emergency ultrasound was done. Her uterus was found to be empty, with a hyperechoic shadow adjacent to it. There was marked free fluid in abdomen. Immediately consultant review was sought, which revealed unicornuate uterus with noncommunicating rudimentary horn . The rudMariceau and Vassal published the first description of a rudimentary horn pregnancy in 1669, and 600 cases have since been described . PregnanThe prerupture diagnosis of pregnancy in rudimentary horn has drastically reduced maternal mortality . But the Tsafrir et al. proposed the following criteria for ultrasonographic diagnosis: (1) a pseudo pattern of an asymmetrical bicornuate uterus, (2) absent visual continuity tissue surrounding the gestation sac and the uterine cervix, and (3) the presence of myometrial tissue surrounding the gestation sac . In any This case highlights the fact that despite having risk factor for suspected uterine anomaly, that is, previous cesarean section for fetal malpresentation, this patient was missed on routine malformation scan one week prior to the catastrophic rupture of the rudimentary horn. Her previous operative records were not reviewed, which when subsequently reviewed clearly stated the presence of unicornuate uterus with noncommunicating rudimentary horn. This woman was also not warned regarding potential complications. Hence this case is being reported to familiarize the radiologists regarding this rare but potentially lethal presentation which if diagnosed safely in prerupture state can be managed laparoscopically without the associated sequelae of rupture uterus. Three-dimensional ultrasound imaging and MRI are useful tools in the improvement of diagnostic accuracy, guiding both counseling and surgical planning . This case further raises the question of whether routine excision of rudimentary horn be undertaken in women with unicornuate uterus as a prophylaxis to prevent such catastrophes. A further evaluation of timing of such a surgery is required in a case series, which seems highly unlikely considering the rarity of the condition. In our opinion this decision should be extrapolated from isolated case reports only and routine laparoscopic excision of rudimentary horn with ipsilateral fallopian tube should be offered to these women and those refusing should be adequately counseled regarding the potential complications and if pregnancy occurs in rudimentary horn first trimester laparoscopic excision should be done.


Epithelioid hemangioendothelioma (EHE) is a rare tumor of intermediate malignancy. We report a case of intracranial and intraorbitar EHE. A 3-year-old girl presented with a 3-month history of progressive left exophthalmia. Neuroradiologic imaging (CT scan and MRI) showed an intraorbitar process with an intense enhancement extending to temporal fossa, ethmoidal bone, nasal fossa, maxillary sinus, and cavernous sinus. The angiogram was normal. The tumor was operated through subfrontal approach but only a partial resection was performed. The histological diagnosis was epithelioid hemangioendothelioma. The patient was neurologically intact 2 months after surgery without exophtalmia. However 4 months after surgery he displayed a fall of the right eye vision with intense headache. Control CT scan showed persistence of important tumoral residue. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total removal must be possible. Otherwise, we recommend a complementary chemoradiotherapy and close followup. We propose this interesting case history of a tragical evolution of EHE in contradiction with what has already been reported. Epithelioid heamangioendothelioma (EHE) is a rare vascular tumor that represents a transitional histology between a well-differentiated hemangioma and anaplastic hemangiosarcoma . The terEHE occurs usually in the lung, liver, and long bone. Cranial EHE is very rare. The tumor can occur at any age and does not show sexual predominance . We repoA 3-year-old girl without pathological antecedents presents with a one-month history of exophthalmia  with sigAxial and coronal CT scan through the left orbit with contrast  shows anA surgical resection with cranioorbital approach was performed; it consisted of left orbital exenterating and partial resection of the nasal and cranial extension. The peroperative finding was a reddish solid tumor. Postoperative course was uneventful.Histopathologic examination  revealedA therapeutic complement with radiotherapy base and chemotherapy is proposed but the patient relatives refused. Two months later, the patient presented blindness of the right eye and an appearance of the tumor through the left orbit and the oral cavity . The patEpithelioid hemangioendothelioma is a rare vascular tumor of intermediate malignant features within the progression of hemangioma to angiosarcoma  and relaWeiss and Enzinger described the first cases and proposed the term epithelioid hemangioendothelioma in 1982 . Of thesThe tumor can occur at any age and does not show sexual predominance . ConcernThe hypercellularity and the poor vascular differentiation of the tumor eliminate a diagnosis of hemangioma, whereas the low mitotic index and the absence of necrotic foci role out a diagnosis of angiosarcoma.The exact prognosis of EHE was not established clearly. Weiss et al. have reviewed the biological behaviour of 46 cases in soft tissue, lung, liver, and bone. They reported a recurrence rate of 13%, a metastasis rate of 31%, and a mortality rate of 13%, all within a 4-year period . HoweverTreatment of EHE depends on its location and degree of malignant potential. It consists of surgical resection. Wide local excision and possible regional lymph node resection are advocated because the lymph node is a common metastasis site , 10. Rad


The underlying causes of labor dystocia can be various. Lack of expulsive forces or fetal malpresentation are amongst the most common ones. However, pelvic masses are described as well. Here we describe two cases of labor dystocia as first presentation of pelvic malignancy. Labor dystocia is responsible for more than 50 and 20 percent of secondary cesarean sections in nulliparous women and repeat cesareans, respectively . DystociAfter an uncomplicated pregnancy a 32-year-old G2P1 underwent a secondary cesarean section at GA 41 weeks for labor dystocia. Despite active support of labor maximal cervical dilation measured 7 cm, her peripartum medical record reported no abnormal findings. A healthy daughter 3, 170 g was born. During surgery a large elastic to solid abnormality was palpable in the anterior vaginal wall; for no obvious reasons histology samples were not taken.Her first pregnancy ended in a primary cesarean section for breech presentation and preeclampsia at 37 wks—giving birth to a healthy son 2, 130 g. Further medical history showed an appendectomy and a correction for inguinal herniation.Four weeks after delivery patient was evaluated at an outpatient clinic of a general nonteaching hospital for vaginal discharge and fever. Again the mass in the anterior vaginal wall was palpable. Vaginal cytological and histological samples were taken but showed no evidence of abnormality. The patient was treated with oral antibiotics (amoxicillin with clavulanic acid) and reassessed 15 days later. At this last visit the mass was still present but its consistency felt rigid. Seven weeks postoperative a MRI-scan of the pelvis showed a tumor 3.5 × 10 cm in the anterior vagina wall with benign impression without any pathological lymph nodes detectable.Thereafter the patient was referred to our Gynecologic Oncology Department of the Radboud University Nijmegen Medical Centre (RUNMC), Nijmegen, The Netherlands. Here she underwent an examination under anesthesia, combined with a cystoscopy and obtainment of histology samples. A rigid solid tumor was palpated in the anterior vaginal wall reaching from the cervix to midurethral extending laterally to the bilateral parametria. The trigone of the urinary bladder was elevated. Since we suspected ureter obstruction, ultrasonic evaluation of the kidneys followed, showing a dilated left kidney and ureter. A true cut biopsy of the anterior vaginal wall was taken.Histology of this biopsy revealed a diffuse large B-cell non-Hodgkin lymphoma.The left kidney function was restored by placing a nephrostomy catheter. Patient was referred to the hematology department for further evaluation and chemotherapy treatment , Oncovin (vincristine), Prednisone). She showed a complete remission after eight R-CHOP courses; also true cut biopsy revealed no evidence of disease. However, since a reduced mass was still visible on CT-scan adjuvant brachytherapy was initiated.A second patient was a 38-year-old G6P2 who underwent a secondary cesarean section which was performed for labor dystocia at GA 41 weeks. Her medical history revealed a secondary cesarean for fetal distress and a vaginal delivery. Both children were full-term and healthy.The pregnancy was complicated by recurrent vaginal blood loss in the second half, without any macroscopic cervical abnormalities seen at gynecologic examination. Cervical cytology at 27-week gestational age showed atypical squamous cells of undetermined significance, and the smear was scheduled to be repeated 6 weeks after delivery. The current delivery was induced for oligohydramnios. The cervix dilated to a maximum of 4 cm, despite good contractions. The cervix felt firm to hard in consistency. After the cesarean section histology samples of the cervix were taken in the same theatre session, showing grade 2 squamous cell carcinoma (SCC). She delivered a healthy son, 4,084 g.After the diagnosis of SCC of the cervix, she was referred to our Gynecologic Oncology Department (RUNMC) and underwent an examination under anesthesia, revealing a severely altered cervix with a tumor measuring 5 cm involving the right parametrium. She was clinically staged as a cervical cancer FIGO stage IIb. CT-scan showed no pathological lymph nodes, but due to the short postpartum interval the uterine cervix could not be assessed reliably.2. Examination under anesthesia after chemotherapy showed a substantial decrease in tumor size to 1.5 cm. Patient was scheduled for radical hysterectomy, bilateral salpingo-ooferectomy, and bilateral pelvic lymphadenectomy. Histopathological analysis of the specimens showed no further malignancy; all 18 removed pelvic lymph nodes were negative for malignancy.Since delivery by cesarean section might have led to spill of tumor cells intra-abdominally or might have provoked hematological metastasis, the patient received 6 courses neoadjuvant chemotherapy consisting of cisplatinum and placitaxel, both 70 mg/mCervicovaginal, intraabdominal, or retroperitoneal abnormalities obstructing labor have been described since one of the first publications by Freeth in 1950 . These aIn the Netherlands cancer is among the leading causes of nonaccidental death in young women, with mortality rates of 26% in women aged 20 to 30 years and 42.5% aged 30 to 40 years . TherefoGynecologic malignancies associated with pregnancy can be detected and diagnosed throughout pregnancy. However, most gynecological cancers are found in the first and second trimester of gravidity. Recurrent vaginal blood loss or abnormal findings on prenatal ultrasound screening may lead to early diagnosis. Adequate and repeated pelvic examination throughout routine antenatal care may detect pelvic masses at an early stage. This might reduce obstructed and complicated deliveries, but more importantly it may prevent late complications due to metastatic disease . Early dAs a result of frequent antenatal examination, obstructive labor is seldom the first presentation of a pelvic malignancy. However, it still occurs, and pelvic malignancy obstructing labor should be considered peripartally in case of dystocia.


Intravascular fasciitis is an uncommon variant of nodular fasciitis, which is a reactive proliferative lesion of myofibroblasts. Since its identification in 1981, only 32 cases of intravascular fasciitis have been reported in the English literature. The lesion is commonly located in the head, neck, and extremities, with only three cases arising from the trunk. Here we report the fourth case involving the trunk (the flank area).A 21-year-old African-American female presented with a subcutaneous mass on her flank. Grossly, the mass was red-tan, oval, and well-demarcated, measuring approximately 0.5 cm in diameter. Microscopically, the mass was composed of spindle cells arranged in a swirling and intersecting pattern inside the lumens of two blood vessels. It extended through the vascular walls into the surrounding fibroadipose tissue; in some sections, the spindle cells were intermixed with the perivascular fibrous tissue. Elastin stain revealed remnants of elastic lamina partially surrounding the lesion. The nuclei of the spindle cells were relatively uniform with tapered ends and prominent nucleoli. No significant mitotic activity was observed. Multinucleated giant cells were scattered among the spindle cells, along with infiltrating lymphocytes and extravasated red blood cells. Immunohistochemical stains showed the spindle cells were positive for smooth muscle actin, focally positive for muscle specific actin, and negative for S-100, confirming their myofibroblastic differentiation. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis.By reporting this rare case, we would like to raise the awareness of this non-neoplastic lesion to avoid misdiagnosing it as a sarcoma with vascular invasion. Previously reported similar cases were also reviewed and compared with this case. Intravascular fasciitis is a rare benign lesion characterized by reactive proliferation of myofibroblasts in the superficial or deep fascia with involvement of arteries and/or veins. Intravascular fasciitis is a variant of the more common condition of nodular fasciitis, which does not show vascular invasion. Intravascular fasciitis was originally described by Patchefsky and Enzinger in 1981 . Here weThe patient was a 21-year-old African-American female who presented with a single nodule on the flank area. Grossly, the subcutaneous nodule was red-tan, oval, and well-demarcated, measuring approximately 0.5 cm in diameter. Microscopically, the mass was composed of spindle cells arranged in a swirling and intersecting pattern inside the lumens of two blood vessels (Figure Thirty-two previously reported cases and the current case of intravascular fasciitis are summarized in Table Microscopically, intravascular fasciitis was characterized by a spindle cell proliferation inside the lumens or associated with the walls of arteries or veins of all sizes. Depending on the numbers of blood vessels involved and the longitudinal extension of the lesion, the mass exhibited a single or multi-nodular appearance. In two cases, organizing thrombi were found within the lesion ,10. One Immunohistochemistry studies showed that the spindle cells were positive for vimentin and SMA, negative for keratin, S100 protein, desmin, CD31, CD34, and c-kit, confirming their myofibroblastic differentiation. The multinuclear giant cells were CD68 positive, suggesting that they are cells of histiocytic origin.The pathogenesis of intravascular fasciitis has yet to be understood. The immunohistochemical characteristics of the spindle cells confirm their myofibroblastic origin. However, the factors initiating myofibroblast proliferation have not been clearly identified. Possible risk factors that have been proposed include preceding trauma, thrombosis, and high levels of estrogen. Specifically, two patients developed the lesion following trauma ,5. OrganIntravascular fasciitis can be misdiagnosed as a sarcoma with vascular invasion because of its intravascular proliferation. However, the cytologic features of intravascular fasciitis, including the absence of large atypical hyperchromatic nuclei and abnormal mitotic figures, are helpful in distinguishing these two conditions .Intravascular fasciitis is a rare variant of nodular fasciitis associated with blood vessels. By reporting this rare case, we would like to raise the awareness of this non-neoplastic lesion to avoid misdiagnosis.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.SMA: Smooth muscle actin; LMWK: Low molecular weight keratin; EMA: Epithelial membrane antigen.The authors declare that they have no competing interests.MG collected and interpreted data, and made the diagnosis. YZ and FC were the major contributors in writing and revising the manuscript. All authors read and approved the final manuscript.


Transurethral resection of bladder tumor (TURBT) may be applicable for the treatment of deeply invasive tumors in high-risk patients who are not suitable candidates for radical cystectomy. Intraperitoneal perforation is much less common, however, bladder wall perforation is still a cause for concerns because after perforation several studies have been reported to have peritoneal or abdominal metastases.An 84-year-old female patient with multiple large enhancing masses in the urinary bladder underwent radical TURBT to remove the deeply invasive tumors because she was not a suitable candidate for a major operation. Microscopic examination of the resected specimen confirmed the muscle invasive urothelial carcinoma with partial invasion into the perivesical fat tissue. She visited again 6 months later after the operation complaining abdominal distension. Computed tomography showed perforation of urinary bladder with intraperitoneal seeding and adnexa metastasis.Radical TURBT could be associated with delayed perforation of urinary bladder and intraperitoneal seeding. Transurethral resection of bladder tumor (TURBT) is the primary treatment modality for bladder cancer . BladderAn 84-year-old female patient had been visiting our urologic clinic due to her painless intermittent gross hematuria for a year. Evaluations revealed multiple enhancing masses in the urinary bladder, measuring 3.5 × 3.0 cm and 3.3 × 3.4 cm in the left lateral wall, 2.0 × 1.3 cm in the anterior wall, and 1.2 × 1.7 cm in the right lateral wall (Figure The patient visited our clinic again 6 months later after the operation complaining abdominal distension and change in her mental status. Computed tomography revealed a large amount of ascites with enhancing peritoneal thickening and peripheral nodular enhancement of the right adnexa, indicating peritoneal seeding and metastasis Figure a. The uret al. noted that intraperitoneal perforation was followed by extensive peritoneal and liver metastasis within 4 months of resection [et al. reported the first case of tumor seeding to both adnexa in a patient with low grade urotherlial cancer conservatively treated with TURBT that had intraperitoneal perforation [et al. insisted surgical repair of a bladder perforation during transurethral resection of bladder tumor increases the risk of extravesical tumor cell recurrence and negatively affects patient prognosis, however, we assume their study had a lower level of evidence due to their retrospective approach [The objective of TURBT is to eradicate all visible disease while attaining an accurate histological diagnosis. To achieve this goal, deep and extensive resection beyond the basement membrane is generally warranted. This, however, puts the patient at risk of bladder perforation . The reaesection . Bus et foration . Skolariapproach . For exaapproach .TURBT may be applicable not only in the management of superficially invasive urothelial cancer but for the treatment of deeply invasive tumors in high-risk patients to whom the radical cystectomy is not suitable . PerforaRadical TURBT could be associated with delayed perforation of urinary bladder and intraperitoneal seeding. We take into consideration whether this procedure is appropriate to take the risk, even though the risk of tumor implantation outside the bladder is small.Written informed consent was obtained from the patient’s guardians 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.The authors declare that they have no competing interests.JHK and WJY contributed with the conception and design of the study and drafted the manuscript. Both authors read and approved the final manuscript.


Introduction. To report the robotic-assisted abdominal cerclage performed in two nonpregnant women and the success of live birth outcomes. Presentation of Cases. A 36-year-old woman with a complaint of recurrent second trimester pregnancy losses and a 35-year-old patient with a complaint of preterm deliveries and cervical insufficiency underwent robotic assisted abdominal cervicoisthmic cerclage placement in nonpregnant period. The two patients had spontaneous pregnancy after the robotic-assisted abdominal cerclage and delivered healthy infants. Discussion. The limitations of traditional laparoscopic abdominal cerclage have been accomplished with robotic surgery advantages especially intuitive movements and increased range of motion. There are only a few studies in the literature including robotic assisted abdominal cerclage in nonpregnant women, and only five successful live birth outcomes were reported. In this paper, we reported the sixth and seventh cases of achieved live pregnancy after robotic assisted abdominal cerclage in the literature. Conclusion. Robotic assisted abdominal cerclage is a good alternative surgical method with successful pregnancy outcomes. Cervical insufficiency is a condition which is characterized with painless cervical dilatation and is an important factor causing second trimester pregnancy loss. Cervical insufficiency occurs in 0.5–1% of all pregnancies . CervicaA 36-year-old woman with a history of gravida 4, para 0, was admitted to our clinic with a complaint of recurrent second trimester pregnancy losses . The patient had two unsuccessful vaginal cerclages during the last two pregnancies. The rest of her medical history was unremarkable. The physical examination revealed insufficient cervical tissue. We decided to perform robotic-assisted abdominal cerclage prepregnancy. The patient was appropriately counseled and a written informed consent was obtained. The robotic procedure was approved by the institutional review board at our hospital. The patient underwent a successful robotic assisted abdominal cerclage. Da Vinci S surgical system with four-trocar transperitoneal approach was used for the procedure. The surgical technique was as follows. The vesicouterine peritoneal flap was taken down, and it visualized the uterine vessels bilaterally. An avascular area between the uterus and the bifurcation of the uterine artery was prepared. A 5 mm mersilene tape with a flattened needle was sent to the abdominal cavity and was passed medially to the uterine artery from the anterior to the posterior direction. The same procedure was performed at the other side. The passed tape was tied firmly posteriorly. The surgical technique of robotic assisted abdominal cerclage is shown in A 35-year-old patient with, gravida 2 presented to our clinic with a complaint of preterm deliveries. In her past medical history, she had two deliveries which were at the 20th and 26th weeks of gestation. On physical examination, the cervix was short in appearance, and the transvaginal 21 mm cervical length measurement supported the finding. The patient was offered to be operated with robotic surgery, for abdominal cerclage. After her acceptance, she underwent robotic surgery and the procedure was successfully completed in 43 minutes. The same surgical technique was used as in case  1. No surgical complications occurred during the intervention and the postoperative stay and no conversion to laparotomy. The patient was discharged from hospital one day after operation. The patient have a spontaneous pregnancy. The followup of the patient was uneventful, and she had a cesarean section at 38th week of gestation and delivered a healthy infant.Increased neonatal survival rates are associated with transabdominal cerclage . HoweverThere are only a few studies including robotic assisted abdominal cerclage in the literature. Barmat et al. reported a case which included the first robotic assisted abdominal cerclage in a nonpregnant woman . The patThe first robotic-assisted laparoscopic cerclage in a pregnant patient was performed by Fechner et al. in 2009 . The patThe most comprehensive study including robotic assisted abdominal cerclage was reported by Moore et al. in which a total of 24 nonpregnant patients underwent robotic procedure . Only inAlthough the number of cases in our study was limited, we reported the sixth and seventh cases of achieved live pregnancy after robotic assisted abdominal cerclage in the English literature. It is now clearly established that robots have an important place in the gynecologist's armamentarium for minimal invasive surgeries; however, the long-term outcomes of several gynecologic procedures which are performed with the da Vinci surgical system have yet to be evaluated. Further randomized and controlled trial studies are warranted to determine if robotic surgery truly offers a benefit over laparoscopy in terms of surgical outcomes.


Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia.We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss.An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention. Extramedullary hematopoiesis (EMH) occurs commonly in patients with severe thalassemia who receive inadequate treatment. In this condition, beside the usual regions of hematopoiesis, blood cells can be formed in unusual sites like the liver, spleen and lymph nodes to meet the demands of hematopoiesis ,2. ThereA 34-year-old Caucasian man, with known beta thalassemia intermedia, presented with the complaints of chronic headache during the last four months, with recent onset nausea, vomiting, disequilibrium and rapid progressive visual loss.He had pallor of the skin with a cachectic appearance and a prominent skull deformity with hepatomegaly. Neurological examination showed bilateral severe optic disc atrophy and his visual acuity revealed no light perception (NLP) in both eyes. His extraocular movements and examination of his other cranial nerves were normal. His speech and sensorium were intact but there was gait disturbance due to disequilibrium. His Glasgow Coma Score was 15 and there were no paresis in his extremities. His deep tendon reflexes and cerebellar functions showed no abnormality and Babinski's sign was absent. Our patient had irregular blood transfusions, a splenectomy and cholecystectomy in his past medical history. He did not consume any kind of medicine. A family history revealed that his parents were both carriers .9/L, platelet count 265 × 109/L and mean corpuscular volume 68.9fl. A peripheral smear showed anisopoikilocytosis, tear drop cells, polychromasia, nucleated red blood cells and target cells. A hemoglobin electrophoresis showed 96.7% fetal hemoglobin and 3.3% hemoglobin alpha 2. Adult hemoglobin was absent.On admission, a sample of his blood sent for laboratory investigations. The results showed hemoglobin 8 g/dL, white blood cell count 13.2 × 10In a skull X-ray, expansion of the diploic space with a so-called hair-on-end appearance inline with EMH was seen. Computed tomography (CT) study of his brain disclosed a large hyperdense lobulated lesion in the vicinity of the left occipital horn with significant surrounding edema which enhanced after intravenous contrast injection. Magnetic resonance imaging (MRI) of his brain revealed a hyperintense lesion in T1-weighted images which was signal void in T2-weighted imaging, with significant peripheral edema Figures . There wAfter correcting the hemoglobin level before the operation, with the impression of a meningioma of a lateral ventricle or high grade glioma, a craniotomy was performed, using a left posterior parietal transcortical approach.Intraoperatively, the lesion was found to be lobulated, very well differentiated from the peripheral white matter without adhesion to it and well capsulated with prominent vessels over the capsule of the tumor. The lesion was excised entirely up to its final attachment to the choroid plexus, which came in to view, and the occipital horn was seen.A pathologic examination of the surgical specimen was compatible with hematopoietic tissue. Macroscopically, the specimen measured 2.5 cm×1.5 cm×1 cm and was soft and grayish white. Microscopically, the specimen was composed of brain tissue, hematopoietic cells and megakaryocytes Figure . On immuSpinal cord compression due to EMH is a well-known condition, however intracranial involvement is extremely rare and there are few reported cases about this disorder ,15. EMH In our case, our patient had symptoms of increased intracranial pressure and visual loss. The latter symptom may have been a secondary effect of the mass lesion upon adjacent structures of his visual pathways and/or increased intracranial pressure. Moreover, an intracranial EMH arising from the base of the cranium near the sphenoid and ethmoid bones may extend into the intracranial cavity and compress the visual pathways, with resultant progressive visual failure .In one previous case report, Aarabi and colleagues presented a similar case of an intracranial EMH in a thalassemic patient with symptoms of decreased visual acuity. They also surgically excised the intrasellar mass, but their patient had a previously determined EMH arising from the base of his cranium near the sphenoid and ethmoid bones, extending into the intracranial cavity. Their management included partial resection of the mass .Musolino and his colleagues also reported a similar case of an intracranial EMH with symptoms of decreased visual acuity and endocranial hypertension, but their patient had acquired immune deficiency syndrome with chronic bone marrow dysfunction. It was mentioned that the decreased visual acuity was due to cytomegalovirus chorioretinitis, contrary to that seen in our case which was due to a compressive effect of the intracranial EMH .An interesting finding in the current case report was that we were presented with a rare case of an intracranial EMH originating from the choroid plexus, and our patient underwent successive total surgical resection and the signs and symptoms of increased intracranial pressure subsided. Although total resection of this lesion is a hemorrhagic procedure, it seems that, in operable cases, total resection may be safe and help with the early remission of the clinical signs and symptoms.This case report showed that although rare, an intracranial EMH should always be considered in a differential diagnosis of any mass lesion and increased intracranial pressure symptoms in patients with thalassemia. Therefore, appropriate investigations should be performed to diagnosis the probable intracranial ectopic marrow lesion before any surgical intervention.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SAF performed the chart review and manuscript preparation. HT, ASH, SA and SAF carried out the operation. PM and MM were the pathologists who examined the specimen. All authors read and approved the final manuscript.


Odontomas are malformations of the dental tissues and may interfere with the eruption of the associated tooth. Complex composite odontoma (CO) was described as a distinct entity for the first time by Broca in 1866. This lesion takes place due to the developmental disturbances where the dental components are laid down in a disorganized manner, due to failure of normal morphodifferentiation. Very few cases of erupted complex composite odontomas have been reported in the literature. The case reported here is of an odontoma found in the left mandibular body, associated with an impacted second molar of a 17-year-old Saudi male. Under local anesthesia the odontoma was surgically removed. Histopathological examination confirmed the diagnosis of CO. The impacted second molar which was left in the mandibular body erupted clinically after 6 months. Erupted CO is rarely seen in the mandibular left body. The early diagnosis, followed by a proper treatment at the right time, will result in a favorable prognosis. According to the latest classification of the World Health Organization WHO, 2005), two types of odontomas can be found: complex odontomas and compound odontomas, the latter being twice as common as the former. Odontomas erupting into the oral cavity are rare. The first case was published in 1980, and since then only 17 cases have been reported in the literature. Eight of the 17 cases were complex composite odontomas (COs); the rest were compound odontomas. Pain, swelling, and infection were the most common symptoms, and 13 cases presented an impacted tooth associated with it [005, two A 17-year-old male visited the dental OPD of King Khalid University, Abha, KSA, with a chief complaint of pain in upper left first molar tooth. Routine clinical examination revealed a partially erupted hard mass on the alveolar ridge distal to left mandibular first molar and missing mandibular second molar . OrthopaThe WHO classification defines CO as follows: “a malformation in which all the dental tissues are represented, individual tissues being mainly well formed but occurring in a more or less disorderly pattern” . The etiRadiologically, the compound odontoma appears as a collection of tooth-like structures while composite type appears as a calcified mass with a radiodensity similar to tooth structure; both are further surrounded by a narrow radiolucent zone. However there was absence of any corticated border in our case as normally published in previous literature. Unerupted teeth are more commonly associated with compound composite odontoma. However, in our case, presence of unerupted tooth with CO was seen . The lesRarely odontomas may form peripheral or soft tissue lesions in which they arise outside alveolar bone and may exfoliate or erupt. Such a rare erupted odontoma was present in our case .Microscopically, this lesion consists primarily of a well-delineated, roughly spherical mass of a haphazard conglomerate of mature hard dental tissues. Some examples may include better-ordered, tooth-like structures . Clear sConservative surgical enucleation is considered to be the treatment of choice in most cases of CO. During the removal of pathological structures in the mandibular retromolar region in an early age, care should be taken to preserve bone structures on the anterior border of the mandibular ramus, because it might impair jaw development. If needed, a control X-ray should be taken during the surgery. The prognosis is always good since these tumors do not tend to recur . As odonOdontomas rarely erupt into the mouth and tend to be associated with impacted teeth. Despite their benign nature, however, their eruption into the oral cavity can give rise to pain, inflammation and infection and different clinical appearance. The treatment of choice is surgical removal of the odontoma, followed by histopathological analysis. In the case of odontomas associated with impacted teeth, the latter should be preserved in situ when a favorable path of eruption exists for facilitating passive eruption of the impacted teeth into the oral cavity.


Meningeal melanocytoma is a primary melanocytic neoplasm with certain MR and immunohistochemical characteristics worthy to note. In a 38-year-old man with a complaint of headache for a couple of years and recently added nausea, vomiting, diplopia, progressive visual blurring and hearing loss, magnetic resonance imaging (MRI) was remarkable for T1 shortening of leptomeninges and certain nodules in precontrast study. Subsequent contrast-enhanced MR imaging of the brain and spine revealed enhancement in the basal cisterns extending throughout the spinal canal. Contrast-enhanced MRI revealed diffuse enhancement in the basal cisterns extending throughout the spinal canal. Immunohistochemical analysis on one of the intraspinal nodules proposed leptomeningeal melanocytoma. The characteristic shortening of T1 and T2 relaxation times in MRI as a result of the paramagnetic stable free radicals that exist within melanin, often suggests a diagnosis of a melanocytic leptomeningeal process. Moreover, there are unique immunohistochemical characteristics for these varied lesions. In appropriate clinical settings, certain radiologic findings, especially both T1 and T2 shortening in nodular CNS lesions should propose meningeal melanocytoma. Meningeal melanocytoma, as a primary melanocytic neoplasm, can exhibit distinguishing radiologic characteristics. This is useful, since a good postoperative survival rate is expected for patients with meningeal melanocytoma if complete resection is undertaken. Hence, the surgeon should be advised of this possible diagnosis in suspected cases of meningioma. Notions that can make the surgeon accomplish maximal effort at tumor resection and eliminate the risk of recurrence , 2.A 38-year-old man with a complaint of headache for a couple of years and recently added nausea, vomiting, diplopia, progressive visual blurring and hearing loss came to the hospital four years ago. Neurological examinations disclosed the following findings; visual acuity diminution, bilateral papillary edema, sensory-neural hearing loss, mild gait ataxia, bilateral upward plantar reflexes and decreased pin-prick sensation over the territory of the maxillary nerves.Noncontrast head CT scan showed hyperattenuated foci in both temporal and occipital lobes, basal meninges and the cerebellum . MeanwhiBecause of progression of symptoms and development of hydrocephalus, the patient underwent a programmable VP shunt one year later, but thereafter, the patient’s complaints were still present. Further imaging examinations revealed aggravated brain and spinal lesions and a new onset tonsillar herniation . Thus, aPrimary melanocytic neoplasms are rare lesions which originate from normally existing leptomeningeal melanocytes . MelanocBiological behavior is variable; incomplete resection may yield in recurrence, an event that may occur because of extensive leptomeningeal involvement or persistence of neglected small foci of tumor. Some believe transition into malignant melanoma does not occur, however, we encountered to four cases of malignant transformation of meningeal melanocytoma, in the literature -13. We dIn appropriate clinical settings, certain radiological findings, especially both T1 and T2 shortening in nodular CNS lesions should propose meningeal melanocytoma in the mind; a primary melanocytic neoplasm in which complete resection heralds a favorable prognosis.


Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (LCTs) are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11 Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens.β-hydroxylase deficiency is the second most common cause of CAH, accounting for less than 5% of cases. It is characterised by the overproduction of adrenal androgens and deoxycorticosterone, leading to virilization of female fetuses, pseudoprecocious puberty in male infants, and hypertension with or without hypokalemia in both genders [Ninety percent of CAH cases have a defect in 21-alpha hydroxylase enzyme [The rising of adrenocorticotropic hormone (ACTH) synthesis results in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs) . It is eβ-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.Hereby, we present an unsual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency since the age of 2-year old when he was evaluated for growth acceleration, advanced bone age, virilization, vomiting, diarrhea, generalized pigmentation, hypertension, and hypokalemia. Hormonal analyses were suggestive for the diagnosis of 11β-hydroxylase deficiency: 17-hydroxyprogesterone (17-OHP), 22 ng/mL ; androstenedione, 11.9 ng/mL ; 11-deoxycortisol: 69.6 ng/mL  8H Cortisol, 277 ng/mL . His symptoms were controlled with 20 mg of hydrocortisone. He was irregular in followup and was not compliant with treatment. The patient was born of a fourth degree consanguineous marriage, and his family history was remarkable for early neonatal deaths.The patient was a known case of CAH due to 11At the age of 19 years, he was hospitalized for severe hypokalemia. On physical examination, he had a blood pressure of 180/110 mmHg, pulse rate of 70/min, temperature of 37°C, height of 163 cm (−2 SD), and weight of 58 kg (10th–25th percentile). A systemic melanoderma was noted. Genital examination demonstrated an enlarged right testis measuring 6.5 by 5.5 by 4.5 cm, and a 1.5-cm firm mass was noted in its upper pole. The left testis was not palpable in the scrotum or inguinal canal. The patient did not have any testicular discomfort or pain. He had no gynecomastia.μg/mL ; plasma deoxycorticosterone (DOC) 9648 ng/L ; testosterone 6.9 ng/mL . Tumor markers  were negative for malignancy. Semen analysis showed severe oligo-astheno-teratospermia.He had normal biochemistry values and a poorly compensated hormone profile: ACTH 2500 pg/mL ; 17-OHP 17.5 ng/mL; androstenedione 19.9 ng/mL; dehydroepiandrosterone sulfate (DHEA-S) 90 In testicular sonography, the right testis was present in the normal position and was enlarged (6.8 × 5.7 × 4.5 cm). However, the left testis was atrophic (3.5 × 2.8 × 1.5 cm) and ectopic, near the common iliac vessels. In addition, ultrasound scan of right testis revealed an hypoechoic heterogeneous mass within the lower pole measuring 4.8 × 3.8 cm in diameter. The left testis contained three round-to-oval hypoechoic heterogeneous lesions, 0.9–2.3 cm in diameter, located in the mediastinum testis . There wThe patient underwent a magnetic resonance imaging (MRI) examination. Coronal images of the pelvis confirmed the ectopic position of the left testis, in front of the left common iliac vessels . BilaterFour months after the administration of a stronger suppressive medical treatment with exogenous steroids (hydrocortisone 30 mg daily), a decrease in the size of right testis was noted (4 cm × 3 cm). Scrotal ultrasound showed reduction in the size of the right testicular mass (3.8 × 2.3 cm) but did not show any reduction in the size of the epididymal nodule. Hormone profile still remained poorly responsive to the compensation .The patient was diagnosed as having bilateral testicular adrenal rest tumor associated with a suspicious-appearing mass in the right epididymis.The patient underwent orchidopexy for the left ectopic testis, as well as an excisional biopsy of the right epididymal nodule. Bilateral biopsies of testicular parenchyma were also taken during the operation. Pathologic examination of the right testis revealed that the epididymal nodule was well-circumscribed, encapsulated, firm, yellow, and measuring 15 mm in diameter. Microscopy showed that this nodule was composed of large polygonal cells with abundant eosinophilic, granular cytoplasm, and round, regular, vesicular nuclei with prominent nucleoli. The tumor cells were separated by an endocrinoid stroma. There was no evidence of infiltrating margins, necrosis, calcification, nuclear atypia, or vascular invasion. Both the lipochrome pigment and crystalloids of Reinke's were lacking . ImmunohTesticular biopsy parenchyma showed a decrease in tubular diameter with peritubular fibrosis and tubular hyalinization. The lamina propria of the tubules was thickened. Extensive Leydig cell hyperplasia was seen in the interstitium, but no germ cells were present in seminiferous tubules.After 24 months of followup, the patient is in good general health and continuing treatment with hydrocortisone. Scrotal ultrasound at the last visit showed bilateral multiple nodules stable in size. The patient is now preparing for partial orchiectomy.β-hydroxylase deficiency, presenting bilateral TARTs and epididymal Leydig cell tumor. To the best of our knowledge, such association has not been previously reported.CAH patients with testicular enlargement present a difficult diagnostic dilemma. Tumors of adrenal rest tissue, LCTs, and Leydig cell hyperplasia are the primary etiologic considerations . In thisTARTs are one of the known complications of CAH. Their reported prevalence is up to to 94% of CAH adults, and they may already appear during childhood . They prLCTs are rare testicular tumors of the male gonadal interstitium, accounting for 1–3% of all testicular neoplasms. These tumors are most common in prepubertal boys aged 5–10 years and in adults aged 30–60 years , 11.The etiology of LCTs remains unknown. Unlike germ cell testicular tumors, Leydig cell neoplasms are not associated with cryptorchidism. It is thought that an endocrine role may contribute to the development of these tumors. The disruption of the hypothalamic-pituitary testicular axis leading to excessive stimulation of Leydig cells by excess luteinizing hormone may induce Leydig cells oncogenesis . High esLCTs are frequently hormonally active, leading to feminizing or virilizing syndromes. These tumors were once managed primarily with radical orchiectomy. However, the experience with conservative procedures has been growing .The occurrence of leydigioma in CAH patients is exceptional, and only six cases of Leydig-cell tumour associated with CAH have been reported so far , 15–18. In our patient, unilaterality, the lack of response to glucocorticoid therapy and immunohistological features were suggestive of the diagnosis of a Leydig cell tumor. In addition, imaging characteristics argue also for this diagnosis. Indeed, as seen in our patient, LCTs are typically isointense on T1-weighted, -showed enhancement and are hypointense on T2 weighted with central areas of increased signal .β-hydroxylase deficiency. The distinction between these two tumors is often difficult, but interesting as treatment modalities are different. CAH patients with testicular tumor need cautious approach during investigations to avoid inappropriate orchiectomies.Our report illustrates an unusual association of epididymal Leydig cell tumor with TARTs in a patient with CAH due to 11


EWSR1-CREB1 gene fusions. Its pathogenesis is unknown, and histologically it can be easily confused with a variety of intra-abdominal neoplasms. We describe a case of CCSLGT with molecular characterization, presenting as an acutely obstructing small bowel mass in a 33-year-old male, which occurred as a second malignant neoplasm 20 years after treatment with surgery, radiotherapy, and cisplatin and doxorubicin chemotherapy for childhood hepatoblastoma. This gives further insight into the clinical setting of this highly aggressive neoplasm and highlights the use of radiation therapy as a possible etiologic factor.Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant neoplasm arising within the wall of the small bowel, stomach, or large bowel, predominantly in children and young adults. It is an aggressive tumor with a high rate of local recurrence, metastases, and early death from disease. Histologically, it is composed of relatively monomorphic ovoid or round cells with clear to eosinophilic cytoplasm, arranged in sheets and sometimes papillary or alveolar architectures, often with CD68-positive osteoclast-like giant cells in variable numbers, and is associated with  EWSR1-CREB1 gene fusion by reverse transcription polymerase chain reaction, which occurred in a 33-year-old male who had previously had surgery, irradiation, and chemotherapy for childhood hepatoblastoma. This expands the clinical spectrum of this highly aggressive neoplasm and highlights the possibility of radiation therapy as a predisposing cause.Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant neoplasm arising within the wall of the small bowel, stomach, or large bowel, predominantly in children and young adults. It is associated with high rates of local recurrence, metastasis, and early deaths from disease. Histologically it consists of relatively monomorphic ovoid or epithelioid cells with clear to eosinophilic cytoplasm arranged in sheets and papillary or alveolar architectures, with variable numbers of CD68-positive osteoclast-like giant cells. Because of the nonspecific immunoprofile of focal S100 protein expression and general lack of immunoreactivity to other antibodies, it may be misdiagnosed as a variety of neoplasms, including melanoma, malignant peripheral nerve sheath tumor, or gastrointestinal stromal tumor (GIST). We describe a case arising in the small bowel wall and harboring A 33-year-old Caucasian male presented to the Emergency Department of his local hospital with worsening abdominal pain, exacerbated by eating, which he had had intermittently for the preceding five months and which had led to weight loss of two stone over the last three months. On clinical examination, the patient had abdominal distension and pain in the lower right quadrant, along with high pitched bowel sounds. 20 years previously, at the age of 13, he had been treated for hepatoblastoma with six cycles of PLADO regimen (cisplatin and doxorubicin) chemotherapy followed by partial hepatectomy and radiotherapy in the context of a Society of Pediatric Oncology Liver Cancer (SIOPEL) Group clinical trial. He had been fit and well since, without detectable late effects from this regimen. The patient's father and grandmother had both been previously successfully treated for colon cancer in their 60s, but there was no other family history of cancer. Abdominal X-ray showed prominent small bowel loops and no air in the rectum. Abdominal computed tomography (CT) scan revealed distal small bowel obstruction due to an intussusception in the right lower quadrant of the abdomen. A 3 cm mass was present at the apex of the intussusception, along with two enlarged mesenteric lymph nodes. The imaging also showed features relating to the patient's previous treatment, with extensive metallic artefact deep into the liver, abutting a patent portal vein with an unusual course of marked anterior looping, a very small pancreatic head and neck, and absent gallbladder, with clips around the portal vein at the head of the pancreas. No focal abnormalities were seen within the liver or spleen. The small bowel mass was resected with clear margins and a primary anastomosis formed. The patient made a good recovery and was discharged shortly afterwards, and owing to the local hospital diagnosis of gastrointestinal stromal tumor (GIST) he was referred to our tertiary center. The diagnosis was revised to clear cell sarcoma-like tumor of gastrointestinal tract, as below. Given the absence of residual disease and the largely resistant nature of this neoplasm to standard chemotherapeutic regimens, there was no role for adjuvant chemotherapy, and hence the treatment plan was for close clinical and radiological followup. Two months later, the patient was admitted to his local hospital with a two-week history of fevers with night sweats and rigors. No specific source of infection was noted clinically, but ultrasound scan of the abdomen showed abnormal liver lesions which were thought to represent possible abscesses. Treatment with intravenous metronidazole and tazocin was commenced for pyrexia of unknown origin and he was again referred to our institution. CT scan showed rapid progression of locally recurrent disease with the development of peritoneal nodules, widespread liver metastases, and prominent mesenteric adenopathy. The liver was biopsied and showed metastatic tumor with similar morphology and immunoprofile to that in the small bowel. Owing to the rapid disease progression, the patient did not wish to be considered for systemic therapy and died from advanced malignancy seven months after initial presentation with this neoplasm.EWSR1 rearrangements by fluorescence in situ hybridization (FISH), and for specific fusion transcripts by reverse transcription polymerase chain reaction (RT-PCR). For FISH, 1 μm thick FFPE sections were dewaxed overnight at 60°C, treated with hot buffer wash at 80°C (2-3 hr) and then proteolytic enzyme treatment at 37C°, and washed in distilled water and then an alcohol series before addition of EWSR1 DNA probes . Hybridization was performed overnight according to manufacturer's protocols. RT-PCR was performed to assess EWSR1-CREB1 and EWSR1-ATF fusion transcripts, according to standard or previously described methods [The histopathological features were noted, and a comprehensive immunohistochemical panel was applied , and early death from disease [EWSR1-CREB1 activates the melanocyte transcription factor MITF, which in turn activates transcription of c-Met, an oncogenic receptor tyrosine kinase recently shown to be activated in clear cell sarcoma [We describe a case of clear cell sarcoma-like tumor of the gastrointestinal tract, arising in the small bowel of an adult male, which harbored  disease –6, in co disease –6, growi sarcoma . Inhibit sarcoma , 9.EWSR1-CREB1 fusions, although some harbor EWSR1-ATF1 fusions that are associated with conventional CCS. While the latter can also arise in the gastrointestinal tract, most contain EWSR1-ATF1 fusions [CCSLGT was first described by Zambrano et al. in 2003 , 10 and  fusions –22. The  fusions .EWSR1-CREB1 fusion transcript that occurred as a rapid and fatal second malignancy in a patient who had had chemotherapy and radiation treatment for childhood hepatoblastoma two decades previously. The treatment of hepatoblastoma has been one of the success stories of pediatric oncology over the last few decades, with a dramatic increase in overall survival rates that are now as high as 70%. This has been in no small part due to the aggressive treatment regimens administered to young patients. However survivors of childhood cancer are at known risk for developing second malignant neoplasms, and CCSLGT has been rarely documented as a subsequent malignancy in this group of patients. One case of CCSLGT has been reported as a second malignancy following neuroblastoma in infancy [EWSR1-ATF1 rearrangement [This study describes a molecularly confirmed CCSLGT, with  infancy . Another infancy . While t infancy , 13, oneangement . While wGiven the rarity of this neoplasm and the fact that it can easily mimic more common lesions occurring at intra-abdominal sites including conventional CCS, metastatic melanoma, GIST, malignant perivascular epithelioid cell neoplasm (PEComa), and clear cell carcinomas such as those from the kidney, it seems likely that the true incidence of CCSLGT is underrepresented because it has been misdiagnosed due to unfamiliarity of surgical pathologists with the entity and the largely nonspecific immunophenotype.EWSR1-CREB1 fusion oncogene or its relevant downstream pathways may be effective at treating disease in the future.In summary, we report a case of CCSLGT, which occurred as a second malignant neoplasm in a 33-year-old male who had had previous chemotherapy and irradiation for childhood hepatoblastoma. The etiology of these rare and largely rapidly fatal neoplasms remains unknown, and this report draws further attention to childhood irradiation as a possible precipitating factor. It remains to be seen whether CCSLGT will emerge as a potential late complication of the presumed irradiation component of the treatment for hepatoblastoma or other pediatric neoplasms or whether this case simply represents an unrelated second malignancy in a patient who had increased susceptibility to neoplasia compared to the general population. Documentation of any CCSLGT is nevertheless important due to its extreme rarity, particularly when occurring in patients with relevant past medical histories, so that we can come closer to establishing its cause. While effective treatments are not yet available for this highly aggressive neoplasm, future targeted therapies that inhibit the function of the


Infantile Fibrosarcoma is a rare soft tissue tumor in infants and children mostly located in extremities. An infantile and adult form has similar histopathological patterns but survival prognosis is much better in infantile form. Recurrence of infantile fibrosarcoma is common but the rates of metastasis are less than 10 percent in children younger than five Years and 50 percent in children more than 10 years old.In this case report, we presented a nine years girl with a relapsing mass in her left hand. The pathologic findings showed sheets of spindle-shaped cells with suggested diagnose of infantile fibrosarcoma. She was successfully treated with combination of surgery and chemotherapy with a good outcome.Infantile fibrosarcoma is a differential diagnose of soft tissue mass in infants and children. It has a good prognosis and distant metastasis is uncommon. Choice of treatment is surgery but chemotherapy and radiotherapy were useful in decrease metastasis. Infantile fibrosarcoma is a rare early childhood malignancy. It includes aproximately 10 % of all sarcomas in children . InfantiA nine years old girl was presented with a mass in her left hand in polmar side. Initially, her mass was noticed four years ago with 3*4cm size which was growing gradually. After excision surgery, it was diagnosed as soft tissue mass (lypoma). She was referred to Shahid Sadoughi clinic of pediatric oncology due to the relapse of the tumor four years later. On physical examination, a firm, immobile mass with mild tenderness and discrete borders was palpable. Her left finger movement was limited in all ranges. No other abnormalities were found in the examinations. Laboratory tests were normal.Magnetic Resonance Imaging (MRI) findings showed 4*5cm neoplast lesion in left hand. Deformity of left hand was presented. Fifth metacarpal bone was not seen. Invasive soft tissue mass (recurrence or remained sarcoma) was suggested in MRI results.IV (intravenous) and oral contrast and thorax scan with IV contrast were normal. Abdomen and chest ray were normal.The histology of the mass was reanalyzed because of the relapse. The findings showed sheets of spindle-shaped cells with suggested diagnose of infantile fibrosarcoma. Afterwards, excisional surgery was do and pathological report revealed cellular spindle neoplastic tissue with fasicular pattern of slightly pleomorphic tumoral cells with vesicular nuclei and occasionally prominent nucleoli admixed by foci of hemangiopericytoma like pattern. Tumoral cells infiltrated into fatty tissue, bone and skeletal muscle. In some focus more than five mitotic figures in per HPF were also seen moderate infiltrations of chronic inflammatory cell with follicular aggregation. . Base onAfter the surgery, the treatment was completed with systemic chemotherapy using oncovin, actinomycin and endoxan.High response to chemotherapy was observed. No relapse was observed up to now.The main cause of infantile fibrosarcoma still remains unknown, but some gene fusions due to translocation and some trisomies have been reported. InfantiInfantile fibroracoma usually presents in the first five years of life, most of them under three years and about 40 percent under three months,8. It isClinically the most common sign is a local, progressive mass with no discrete borders in distal part of extremities. In some cases, the surface is necrotic or ulcertic which makes the appearance similar to vascular malformations like hemangioma. Also itTwo types of infantile fibroracomas are: Desmoplastic type and medullary type. Desmoplastic type in children resemble to adults . The his Local recurrence is reported in 7-34 percents of patients. The chance of recurrence is much more in older than younger ones. In this case, recurrence occurs after 4 years.Distant metastasis is uncommon and was reported only in four percent of cases at the beginning of diagnoses. Children who are less than 5 years old are at the risk of relapse of tumor, whereas the incidence of metastases is under 10% in these patients. The rate of metastatic spread in children who are 10 years old or older is 50% at 5-year follow-up. In our Infantile fibroracoma has a good prognosis. A five-year survival is reported 80 to 100 percent.Currently, the main treatment in most cases is surgery with wide local excision which sometimes leads to amputation. Radiotherapy and chemotherapy were shown to be useful in reduction of tumors bulk especially in metastatic, relapsing and un-respectable tumors. In older children chemotherapy is offered to decrease the possibility of metastasis . In thisThe effective regimen for chemotherapy is vincristine, actinomycin D and cyclophsphamide. Orbach et al reported that 71% of patients with infantile fibrosarcoma responded to vincristin-actinomycin-D chemotherapy. The 5-year survival in these patients was 89% .Although infantile fibrosarcoma is rare and usually presented after birth, but it is necessary to consider it as a differential diagnose of soft tissue mass in infants and even in children. Patients should be followed up for detecting further relapse or metastasis especially in older ages.


A six-year-old Omani girl presented with complaints of decreased vision in both eyes (OU) of two years duration. Ophthalmic examination revealed best corrected visual acuity of 0.6 OU. Cover test and stereo acuity were normal. Anterior segment examination showed posterior subcapsular cataract OU . Fundus What is the differential diagnosis for the lesion in the anterior vitreous OD?What is the most likely diagnosis for the above?Congenital vitreous cyst, intraocular hydatid cyst, cysticercosis, snowball opacity, Intraocular tumor.Pigmented congenital vitreous cyst.Congenital or acquired cyst of the vitreous is rare. A vitreous cyst may be categorized as occurring in one of the three situations. It may occur in eyes with remnants of hyaloid vascular system, in normal eyes, and in eyes with ocular disease. Acquired cysts have been reported to be usually traumatic or parasitic in origin. There was no history of ocular trauma or surgery in our patient. Parasitic cysts (Echinococcosis and cysticercosis) are often associated with some signs of vitreo-retinal disease and intraocular inflammation. Both these features were absent in our patient. Routine blood tests did not reveal eosinophilia and our patient was otherwise healthy. These features led us to diagnose the lesion in our patient as a pigmented, congenital vitreous cyst.pars ciliaris epithelium, while the nonpigmented variety, from remnants of the hyaloidal vascular system. The cyst in our patient was pigmented; more-over we did not find any remnant of the hyaloid system . The cyst in our patient therefore could have originated from the pars ciliaris. Congenital cysts may be spherical, oval, or lobulated and its surface may appear smooth or crenellated. They cysts are translucent or semitransparent with an optically clear cavity, and are usually free floating in the midvitreous.Pigmented, congenital cysts are believed to originate from the They may also be found in the retrolental space, as in our patient, or more posteriorly near the optic disc. Acquired cysts on the other hand are opaque or barely translucent.A scolex (head of the echinococcus) might be detected inside the cyst during slit-lamp and/or ultrasound examination in some instances. This was not seen in our patient.Patients with vitreous cysts occasionally report symptoms of floaters, visual field defects, or intermittent blurring of vision. Argon laser photocoagulation and Nd:YAG lasers have been employed to rupture these cysts. Symptomatic cysts can be aspirated by pars plana approach or can be removed by pars plana vitrectomy.However as a rule congenital cysts are stable and harmless, rarely interfere with visual acuity, and usually do not require treatment. With respect to our patient, the parents have been informed about the diagnosis and periodic observation without any intervention has been advised


Intraperitoneal (IP) chemotherapy has a proven survival advantage in patients with optimally cytoreduced advanced epithelial ovarian cancer . Placeme2) presented to her primary care physician with complaints of two weeks of lower abdominal pain, bloating, early satiety, and nausea. She was treated with antibiotics for presumed diverticulitis for 2 weeks without resolution of symptoms. A CT scan of the abdomen and pelvis revealed inflammatory changes in the pelvis surrounding the bilateral adnexa and sigmoid colon with multiple colonic diverticula present suggestive of uncomplicated diverticulitis, omental nodularity, and soft tissue prominence in the left adnexa. Pelvic ultrasound showed a 5 cm calcified fibroid with submucosal extension, and the ovaries could not be visualized. A serum CA125 level was elevated to 907 U/mL. She was referred to the Gynecologic Oncology clinic for further evaluation and scheduled for surgery. The patient underwent a diagnostic laparoscopy, which revealed omental caking, moderate straw-colored ascites, and bowel adhesions to the anterior abdominal wall. Due to findings concerning for malignancy, the procedure was converted to laparotomy and optimal cytoreduction to < 1 cm residual disease was performed, including total abdominal hysterectomy, bilateral salpingo-oophorectomy, infragastric omentectomy, appendectomy, resection of proximal descending colon with side-to-side re-anastomosis. IP port was placed at the time of surgery. Surgical pathology showed a stage IIIC high-grade serous adenocarcinoma of primary peritoneal origin.A 63-year-old nulligravid female with a past medical history significant for moderately controlled type II diabetes (glycosylated hemoglobin 7.5%), hypertension, hyperlipidemia, and morbid obesity  she presented to clinic with fecal material draining from the vagina. CT scan showed an anastomotic leak with fistula tract to the vagina. Review of the CT performed on postoperative day 10 revealed a small pocket of extraluminal air in the presumed ascites fluid, raising suspicion of a small anastomotic leak, which had not been identified on the initial CT read. Due to the patient's poor nutrition , surgical management was deferred and a percutaneous drain was placed. After 15 weeks, the fistula had completely resolved, and the drain was removed. She has completed 6 cycles of primary chemotherapy with IV carboplatin and paclitaxel without difficulty. CA-125 prior to starting chemotherapy (12 weeks after cytoreductive surgery) had decreased to 123 U/mL, and abdomen and pelvic CT performed to evaluate the anastamotic leak one week after initiating chemotherapy showed no radiographic evidence of disease. CA-125 normalized after the first cycle of chemotherapy, and was 7 U/mL at the completion of chemotherapy.Necrotizing fasciitis is a progressive, polymicrobial, postoperative infection of the muscular fascia with associated necrosis that was first described by Brewer and Meleney in 1926 . Non-necPatients often present with pain, possibly associated with fever or cellulitis or crepitus. A thorough history and physical examination are critical. Risk factors include diabetes, peripheral vascular disease, immune compromise, and recent surgery. Approximately 5% of cases occur in the setting of a postoperative infection, in both contaminated and clean–contaminated operations . On examMultiple studies have shown that administration of IP chemotherapy significantly improves overall survival, and placement of IP catheters for IP chemotherapy following optimal cytoreduction of stage III ovarian cancer has become a standard procedure for many gynecologic oncologists. The most common catheter complications are port malfunction (5–9%) and infection (4–10%) . This isIP catheter placement at the time of bowel resection is controversial. Authors have suggested avoiding placement of IP catheters at the time of large bowel surgery , howeverThis case contributes to the body of literature describing complications of IP catheters. It remains unclear if necrotizing fasciitis in this case was associated with the large bowel resection or coincidence. Delayed placement of IP catheters may be considered if large bowel surgery is performed at the time of cytoreduction, although data is not definitive that complication rate will be reduced with delayed placement. Careful consideration of the risks and benefits of primary versus delayed placement IP catheters should be performed in cases of cytoreductive surgery, especially at the time of large bowel resection with re-anastomosis.The authors declare that there are no conflicts of interest.


Undifferentiated pleomorphic sarcoma is known as a soft tissue sarcoma. Very few cases of this tumor originating from the renal parenchyma or renal capsule have been reported. We report a case of a 70-year-old patient admitted for enormous ureterohydronephrosis and pyelonephritis due to a pelvic ureter lithiasis. After draining by ureteral double J catheter, a nephroureterectomy was performed for nonfunctional kidney confirmed by scintigraphy. The histopathological study shows a pleomorphic undifferentiated sarcoma. The patient was sent to oncologists. Chemotherapy was proposed but the family decided to stop the treatment. The patient passed away 10 months later. Clinicians and pathologists should be aware of the very low occurrence of this renal tumor, which is extremely rare. Currently there is no consensus about its management. Our case extends the literature concerning this tumor. Undifferentiated pleomorphic sarcoma represents up to 10% of all adult soft tissue sarcomas (STSs) . Here weA 70-year-old Moroccan patient was admitted in emergency unit for flank pain and fever. He complained of pain in the right upper abdomen in the last 2 years associated to loss of weight and voiding difficulty. However, there was no history of hematuria. The clinical examination revealed an enormous nonmobile mass in the right lumbar region. Routine investigations showed anemia (hemoglobin 5 g/dL). Biochemical studies were within normal limits. Ultrasonography performed on the patient revealed a big transsonic masse hiding the right kidney. Computer-assisted tomography of the abdomen demonstrated a mass of an enormous hydronephrosis due to a big stone measuring 37 mm diameter in the pelvic ureter Figures  and 2. TPleomorphic sarcoma or undifferentiated high-grade spindle sarcoma is now the preferred term to designate high-grade soft tissue sarcomas failing to show any specific line of differentiation using currently available ancillary techniques . This pathere should not be any history of sarcoma elsewhere to exclude metastasis;the gross appearance must be compatible with origin in the kidney;sarcomatoid renal cell carcinoma must be excluded;metastasis, if present, should be smaller than the renal tumor.There are no specific criteria allowing the diagnosis of primary renal pleomorphic sarcoma. However, it has been suggested that there shoPleomorphic sarcoma has complex karyotypes. No specific structural or numerical abnormalities have been proved to be useful for identification purposes .The histological differential diagnoses of this tumor include other malignant and benign renal lesions benign. The unique curative treatment is the radical surgical excision . An incoThe small number of cases assessed so far does not allow ultimate evaluation of the role of adjuvant chemotherapy and/or and radiotherapy . HoweverPrimary renal pleomorphic undifferentiated sarcoma is an extremely rare tumor without any distinctive clinical or imaging features. Wide excision followed by radiotherapy is the recommended treatment. Adjuvant chemotherapy may be considered according to the profile of the patient.