Most of the intracranial epidermoid cysts are benign, but malignant lesions are occasionally reported. These lesions appear as squamous cell carcinoma and carry a dismal prognosis. Here, we report a case of a primary intracranial squamous cell carcinoma arising in a cerebellopontine epidermoid cyst. The relevant literatures were also reviewed.A 53-year-old woman presented with dizziness and diplopia 9 months in duration. Magnetic resonance imaging revealed an epidermoid cyst in the left cerebellopontine angle and prepontine region with a focal enhancing lesion on T1-weighted gadolinium-enhanced images.Histopathologic diagnosis revealed squamous cell carcinoma on a background of epidermoid cyst. Imaging studies excluded metastases.The tumor was removed subtotally through a lateral suboccipital approach. The patient received intensity modulated radiation therapy  postoperatively.The patient was free from recurrence of the tumor until 3 years after surgery, at which point she was lost to follow-up. The patient died 4 years after the surgery.The epidermoid cyst may occasionally become malignant. Finding an area of enhancement through preoperative magnetic resonance imaging can help to make a correct diagnosis. Based on the review of previous reports, surgical removal followed by radiotherapy shows the best result to treat malignant epidermoid cysts. They occasionally degenerate into squamous cell carcinoma (SCC), although this is extremely rare. We report the case of a woman with primary intracranial squamous cell carcinoma arising from an epidermoid cyst. The relevant literature for this rare disease is also reviewed.Intracranial epidermoid cysts are rare congenital lesions that account for 0.2% to 1.8% of brain tumors.22.1A 53-year-old woman presented with a 9-month history of dizziness and diplopic vision. She had no relevant past medical history. Ophthalmologic examination revealed lateral gaze palsy of the left eye, suggesting cranial nerve VI palsy. Cranial nerve examination revealed no other cranial nerve abnormalities other than left cranial nerve VI palsy. The patient was awake, alert, and oriented; motor, sensory and cerebellar functions were normal.2.2A non-contrast computed tomography (CT) scan of the head revealed an extra-axial low-density mass in the left cerebellopontine angle cistern extending to the prepontine region. The density of the mass was similar to that of cerebrospinal fluid. The pons and the basilar artery were slightly deviated toward the right due to compression by the mass.Magnetic resonance imaging (MRI) also revealed an extra-axial mass in the same region Fig. . T1-weig2.3A left suboccipital craniotomy was performed and the tumor was gross totally resected. The tumor was pearly white, and appeared to have the typical gross appearance of an epidermoid cyst. Cranial nerves were identified and preserved.Postoperatively, the patient had mild facial weakness, which improved gradually. Sixth cranial nerve palsy and hearing impairment remained the same.One month after surgery, the patient began to receive intensity-modulated radiotherapy for a total dose of 67.2 Gy in 28 fractions. The patient tolerated the radiotherapy well.2.4Histologic examination of the resected tumor specimen revealed the histological features of a poorly differentiated squamous cell carcinoma. However, in some portions, the tumor contained multiple layers of squamous epithelium lining and lamellar keratin, which are consistent with an epidermoid cyst Fig. .In order to rule out an extra-cranial primary tumor, abdominopelvic CT, chest CT, positron emission tomography (PET), and whole-body bone scan (WBBS) were performed, and all were normal. Given the histopathological features and the absence of extra-cranial malignancy, the SCC was thought to have arisen from a pre-existing epidermoid cyst.2.5An MRI taken 3 months after the surgery showed a small enhancement in the left tentorium, which was thought to be a radiotherapy-induced change or a postoperative change. Nine months later, the enhanced volume had decreased. The 20-month follow-up MRI revealed that the enhanced portion had nearly disappeared, and showed no evidence of recurrence.The patient's sixth nerve palsy had not improved after the radiotherapy, and the hearing impairment was aggravated, resulting in left ear deafness; facial weakness had resolved.Forty-six months after the surgery, the patient was stable without any new symptoms, and MRI at last follow-up evidenced no recurrence. However, mild ventricular enlargement was suspected. The patient was lost to further follow-up, but was found to have died 4 years after surgery.3A systematic literature review was conducted using PubMed, Medline, and Google Scholar databases to search for relevant English language articles published up to March 2016. The following search terms were used: “malignant epidermoid cyst” OR “intracranial squamous cell carcinoma” OR “epidermoid cyst degeneration.” All eligible studies were reviewed, and the references were checked for additional relevant publications. Information and data were extracted from all included literature. Data include patients’ age, sex, treatment modality, overall survival, and recurrence-free survival. The ethical approval was not necessary, because this is a retrospective case report. The informed consent of the patient was given.4,5–46 There were 23 men and 21 women in these reports, with a mean age of 54 years , including our patient. Their features do not differ from those of benign epidermoid cysts. Primary intracranial squamous cell carcinomas were present initially in 32 cases, while 12 cases were developed following an interval after the resection of a previous epidermoid cyst.,25,27,30We found 43 previously reported literature cases describing a malignant transformation from an epidermoid cyst Table .5,48 Malignant transformation of an epidermoid cyst is extremely rare, but it has been well documented since Ernst first reported a case in 1912.Most squamous cell carcinomas involve the brain as a manifestation of metastases primarily originating from elsewhere in the body or as a result of direct invasion from head and neck cancer. defined the criteria for such a malignant transformation as follows: the tumor had to be restricted to the intracranial, intradural compartment without invasion of or extension beyond the dura or cranial bones, and there must be no extension or invasion through intracranial orifices, no communication or connection with the middle ear, air sinuses, or sella turcica, and no evidence of nasopharyngeal tumor.Garcia et al reviewed 52 cases fulfilling Garcia's criteria and their additional criteria, as follows: presence of a benign squamous cell epithelium within the malignant tumor, and metastasis of carcinoma were excluded. Their review included SCC arising from other benign cysts rather than an epidermoid cyst, such as dermoid cysts, epithelial cysts, endodermal cysts, and craniopharyngiomas.Hamlat et alThe present case fulfills Garcia and Hamlat criteria. No other malignancy found by systemic evaluation. Given that the epidermoid cyst was the only squamous epithelial tissue in the brain, we concluded that the squamous cell carcinoma had developed from a pre-existing epidermoid cyst.–53 although the margin of the cyst may exhibit minimal enhancement.,54 An enhancement within an epidermoid cyst on CT or MRI suggests malignant transformation. Almost all of the cases we reviewed showed enhancement within the tumor, and, in some cases, malignant transformation of the enhanced portion was proven.,13,16,46 In Kodama's case, the enhanced nodule was not resected during surgery and the pathological findings revealed an epidermoid cyst without malignant change; however, a follow-up MRI 2 months after the surgery revealed growth of the enhanced region. The patient underwent stereotactic radiosurgery, but died 13 months after the first MRI evaluation. An autopsy revealed a poorly differentiated SCC originating from the epidermoid cyst. This case emphasizes that enhancement within an epidermoid cyst should be considered a sign of malignancy, and should be removed as possible.The typical CT appearance of a benign intracranial epidermoid cyst is a low-density lesion in the subarachnoid space without contrast enhancement, Epidermoid cysts grow linearly, thus, if they show exponential growth, malignant change should be considered.Sudden development of symptoms or a growing epidermoid cyst seen on imaging study also suggests a malignant change.,31Diffusion-weighted MRI could be useful for the differential diagnosis of epidermoid cysts and SCC. A normal epidermoid cyst shows diffusion restriction on diffusion-weighted MRI, whereas transformed squamous cell carcinoma may not.; however, this may result in unacceptable morbidity and mortality. The remnant tumor could lead to cyst recurrence or malignant transformation. If a patient has a remnant epidermoid cyst, follow-up to check for possible recurrence or a malignant transformation is necessary. The enhanced portion of the tumor should be removed to as great an extent as possible for accurate diagnosis and proper treatment. However, whether total resection of the tumor raises the survival rate compared with subtotal resection has not yet been investigated. Aggressive resection sometimes results in postoperative mortality.,10,11The ideal treatment of epidermoid cysts is complete removal of the tumor and capsule reviewed 36 cases of malignant epidermoid tumors and compared survival outcomes between the surgery-alone group and the surgery plus radiotherapy group. Patients treated with surgery alone had an overall survival of 6.6 months, whereas those treated with postoperative radiotherapy demonstrated a statistically significant increase in survival of 12.7 months . The mean dosage used during radiotherapy for the malignant epidermoids was 52.2 Gy. There was no significant correlation detected between the radiation dose and the survival outcome.Postoperative radiotherapy has been used for the treatment of intracranial SCC and has been proven to improve short-term survival. Nagasawa et al performed a meta-analysis for the survival of 24 intracranial malignant epidermoid cysts. The median survival time for patients treated with surgery alone (n = 9), surgery plus external-beam radiation (n = 11), and surgery plus stereotactic radiosurgery (n = 4) were 1, 18, and 44 months, respectively . However, this study was insufficient to advocate radiosurgery as a standard treatment. Because it was a retrospective study, there may have been selection bias, and the number of cases was small.Radiosurgery can be used as an alternative or adjuvant to radiotherapy. There have been 4 reported cases in which stereotactic radiosurgery was applied to the treatment of malignant epidermoid cysts. Tamura et al All 11 patients with leptomeningeal carcinomatosis died.The prognosis of malignant epidermoid cyst is poor. Of the 43 cases of malignant epidermoid cysts we reviewed, 13 patients were reportedly alive, only 1 patient survived to 5-year follow-up, while 26 patients eventually died.The unique point of our patient is that the preoperative MRI showed the appearance of a typical epidermoid cyst, with only a small enhancement. Unless the possibility of a malignant epidermoid cyst has been considered in advance, it may not have been found in squamous cell carcinoma in pathologic results. An accurate diagnosis enabled adjuvant radiotherapy of this patient and resulted in relatively long survival period compared with the cases reported so far.6Malignant transformation of an epidermoid cyst is rare, and exhibits poor prognosis. The standard treatment for malignant epidermoid cyst is surgery and radiotherapy. Radiosurgery may replace or reinforce radiotherapy. Malignant transformation should be considered when the cyst exhibits contrast enhancement.


Aim: Presumed congenital simple retinal pigment epithelium hamartoma is a rare benign lesion of the macula that mimics congenital hypertrophy of the retinal pigment epithelium (RPE) and combined hamartoma of the retina and the RPE; newer imaging modalities can help in diagnosis. We report three patients with presumed congenital simple RPE hamartoma, and describe the enhanced-depth imaging optical coherence tomography (EDI-OCT) and fundus autofluorescence (FAF) findings. Methods: Two patients were asymptomatic; one had an intraocular foreign body in addition to the hamartoma. All had a similar jet black, elevated lesion in the macula, sparing the fovea. EDI-OCT showed a characteristic hyperreflective layer with complete optical shadowing of the deeper layers; FAF showed pronounced hypoautofluorescence of the lesion. Conclusion: Multimodal imaging with FAF and EDI-OCT can help to differentiate simple RPE hamartoma from similar RPE lesions, and may serve as a useful adjunct to clinical diagnosis of this rare tumor. We present the second largest series of presumed congenital simple RPE hamartoma, and – to the best of our knowledge – the first report of FAF findings of this tumor. Tumors of the retinal pigment epithelium (RPE) are rare. They are classified into four groups, congenital hamartoma of the RPE, congenital hypertrophy of the RPE (CHRPE), combined hamartoma of retina and RPE (CHRRPE) and adenoma or adenocarcinoma of the RPE . The difA 25-year-old man presented with pain and redness OD one week following chisel-and-hammer injury. His best-corrected visual acuity (BCVA) was 20/30 OD and 20/20 OS. Intraocular pressures (IOP) were within normal limits OU. Anterior segment examination OD showed a full-thickness self-sealed corneal tear with an underlying iris hole and focal cataract. Fundus examination revealed a single, round, elevated, jet black lesion nasal to the fovea (mimicking a foreign body) on the surface of the retina (Figure 1a Case 2 was a 60-year-old woman who reported with poor vision OU despite undergoing cataract surgery OS. BCVA was 20/200 OD and 20/40 OS. Case 3 was a 63-year-old woman complaining of defective vision OD for six months: BCVA was 20/30 OD and 20/20 OS. Both patients had remarkably similar findings on biomicroscopy OU: A visually significant cataract OD and pseudophakia OS. Fundus examination OD was normal in both patients. Fundus examination OS revealed a jet black hamartoma similar to case 1 in both patients: close to the fovea nasally in case 2 and temporal to it in case 3 (Figure 1d,g The original description of congenital hamartoma of the RPE was given by Laqua in 1981, based on his clinical observations in two cases . In 1989The authors declare that they have no competing interests.


CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional.The A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression.CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.The  CIC rearrangements have been reported in soft tissue tumors [Round cell sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. They represent 1.1% of all malignant soft tissue tumors . CIC reae tumors . They shCIC-rearranged sarcoma arising from the colon and describe the presenting symptoms, imaging findings, pathological features and molecular genetics of the tumors.We report herein the first case of A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. On examination there was sensitivity on the left abdominal quadrant. An ultrasound scan revealed a complex mass evoking an hematoma.19-9 (CA 19-9), were normal. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon  Fig.  and magnlon Fig. .Fig. 1CoA wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The patient was discharged from the hospital in good health on the 7th postoperative day.Pathology examination showed on macroscopy a heterogeneous semi-solid cystic mass of the mesocolon without any involvement of the colic wall. The mass contained extensive hemorrhagic necrosis.CIC gene.The microscopic examination Figs. , 5, 6 reCIC-rearranged sarcoma. A whole-body bone scan revealed no abnormal increased uptakes and a thoracic CT scan did not detect additional metastases. After an uncomplicated post-operative course, the patient was referred for chemotherapy by vincristine, ifosfamide, doxorubicin and etoposide associated to growth-clony stimulating factors (G-CSF). Nevertheless, the patient developed a neutropenic fever at the first cycle; hence the dose reduction in the next courses. The CT scan at the 4th course revealed a peritoneal progression with nodules in paracolic gutters and the right upper abdomen. The greatest nodule was in the Douglas cul-de-sac and measured 8.5 cm. The patient underwent two courses of temozolamide–irinotecan. He developed peritoneal progression with pleural metastasis (without lung metastasis). He died 2 months later.The mesenteric lymph nodes were free. The surgical margins of the resection were clean. The final diagnosis was a CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It is currently being recognized as emerging entities. It is a huge family of undifferentiated round cells [CIC rearrangement sarcomas have recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. It shows an aggressive course with early metastasis and poor prognosis [The nd cells . Recent rognosis , 6.Round cell tumors can be considered as a differential diagnosis especially Ewing sarcoma family, alveolar rhabdomyosarcoma or poorly differentiated synovial sarcoma. Diagnosis requires radiology, histopathology, IHC and cytogenetic studies –10.CIC), located on the chromosome 19q13.2, is a gene involved in the way of the EGFR and is over-expressed in the cerebellar tissue.The gene capicua transcriptional repressor (In a recent review of Specht et al. , the digDespite aggressive treatment, they exhibit high rates of metastasis to lung and brain, often with a poor prognosis, and median survival is < 2 years.There is no standard therapy for this rare disease. It’s often treated “like Ewing sarcoma”, by surgery and chemotherapy. Chemotherapy combinations such as doxorubicin and ifosfamide, or doxorubicin, vincristine and cyclophosphamide are usually used.Undifferentiated small round cell sarcomas are aggressive tumors. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Lung and brain are common sites of metastasis, with poor prognosis. Generally, median survival is less than 2 years. Newer techniques have been developed recently which helped identify a subset of previously unclassifiable sarcomas, with promising prognostic value.


We report a case of an 85-year-old man with a known asymptomatic left femoral hernia who was admitted to the emergency ward a few hours after falling from a bicycle and suffering from blunt trauma of the handlebar to the left inguinal region. The clinical findings and a computed tomography (CT) scan detecting free air in the femoral hernia sac suggested bowel perforation. Emergency laparotomy 6 hours after the incident confirmed a tear of the sigmoid colon accompanied by free blood and faeces in the left inguinal region of the abdomen. A segmental sigmoid resection and a primary end-to-end colorectal anastomosis were performed. The postoperative course was complicated by delayed oral feeding, a local infection, and a partial left testicle necrosis that led to secondary resection. The patient was discharged after 32 days of in-hospital care. Three months post trauma, we recorded a restitutio ad integrum. The case exemplifies that blunt trauma to preexisting femoral hernias may cause potentially lethal bowel perforation and that the time interval between time of injury and surgical treatment may be a prognostic factor. CT scans seem most suitable for ruling out bowel perforation. The scarce literature for blunt trauma to hernias is reviewed. Groin hernias are common, with an estimated prevalence of between 5 and 10 percent . Femoral9/l). A computed tomography (CT) scan detected free air in the femoral hernia sac and abdomen, suggesting bowel perforation  < 1 mg/l, white blood cell count 9.4 × 10foration .The clinical presentation and the CT findings were indicative for laparotomy. Six hours post trauma, emergency laparotomy revealed a circumferential tear of the sigmoid colon accompanied with intra-abdominal perforation. The left inguinal fossa was contaminated with blood and faeces. The subphrenic site and the space between the bowel loops were free of neither faeces nor purulent fluid. A segmental sigmoid resection of 22 cm and a primary end-to-end colorectal anastomosis were performed. Optimal conditions with vital and uncontaminated bowel segments with excellent arterial perfusion justified omitting a diverting stoma. Following extensive lavage, the abdomen was closed leaving a drain. The postoperative course was complicated by delayed oral feeding and a local infection. Ten days after emergency laparotomy, the local conditions needed a surgical revision. The findings included a well-healed anastomosis as well as a partially necrotic left testicle, which had to be removed in an additional inguinal approach. The skin wound healed by secondary intention. With subsequent parenteral nutrition and simultaneous oral feeding, the patient was discharged 32 days after the accident and recovered fully until follow-up three months later.This case demonstrates that a simple hernia can be complicated by an uncommon and potentially dangerous trauma. In the English literature, we found only few reports on bowel perforation due to blunt trauma in the presence of inguinal hernias . High prThe outcome after surgery for colonic perforation is associated with high morbidity and mortality . The mosPreexisting inguinal or femoral hernias render the bowel more vulnerable to direct physical trauma potentially resulting in a life-threatening bowel perforation. This is one reason more to consider surgical repair in fit patients. In case of groin trauma, even if unspectacular, and subsequent signs of local peritonitis, the possibility of bowel perforation due to a preexisting hernia should be thought of and adequate diagnosis by CT scan should follow.


A 52-year-old female was referred because of melaena. After initital work-up, including gastroduodenoscopy, endosonography and CT scan, a duodenotomy was performed. Definite diagnosis was a duodenal lipoma based on histological findings. Lipomas of the gastrointestinal tract are rare. Only 4% occur in the duodenum. The peak incidence is around the 5th and 7th decade of life, with a slight female preponderance. Gastrointestinal lipomas are usually asymptomatic, but can present with mild to severe gastrointestinal bleeding, intussusceptions, abdominal pain, constipation and diarrhea. Clinical, endoscopical, surgical, and radiological features are described in this case of duodenal lipoma. Lipoma arises from adipocytes. Lipomas are benign, slow growing tumors, and are generally found in subcutaneous tissue of the proximal extremities and trunk. The gastrointestinal (GI) tract is an uncommon localization for lipoma, but if found, their most common localization is the colon. Generally they occur as a single entity, but they may be multiple. Ninety-percent of the gastrointestinal lipomas are located in the submucosa, 10% arise from the subserosa [A 52-year-old woman was admitted to our hospital because of melaena existing for one day, abdominal distension and vomitus. Anamnestic, there were no other complaints. There were no signs of hemodynamic shock. Physical examination showed abdominal distension but no other abnormalities. Her hemoglobin level was 5.7 mmol/l (9.18 g/dL). Gastroscopy revealed a submucosal tumor of the bulbus duodeni of 1.0 x 2.0 cm in diameter, with a central bleeding stigma . EndoscoThe patient underwent a duodenotomie 28 days after initial presentation. The lipoma was resected . The posLipomas are benign, slow growing tumors. In the gastrointestinal (GI) tract they are uncommonly found. Gastrointestinal lipomas account for 4% of all benign gastrointestinal tumors. The most common localization is the colon (64%). Lipomas of the small intestine comprise 17.5% of all benign neoplasm of the small intestine . Of all If a duodenal lipoma is symptomatic, the most common findings are ulceration, gastrointestinal bleeding, intussusceptions, or bowel obstruction. The differentiation between a duodenal lipoma and other gastrointestinal tumors, like GIST or liposarcomas, can be made by CT or MRI . On CT, Endoscopic ultrasonography usually shows a homogenous, hyperechoic mass within the submucosal layer which is highly characteristic .With the evolution of gastrointestinal endoscopy, this tool can be used for diagnosis and treatment, including hemostasis. Duodenal lipoma can be pedunculated or sessile. The pedunculated lipoma can be easily and safely removed by electro surgical endoscopic snare polypectomy -10. HoweIn conclusion, duodenal lipoma is an uncommon type of gastro-intestinal neoplasm. It is usually asymptomatic, but can present with (severe) gastrointestinal bleeding, intussusceptions or bowel obstruction. CT and MRI are highly accurate diagnostic tools, and should play an important role in treatment planning. Since lipomas are benign tumors, and no malignant transformation has ever been reported, it might be more desirable to employ the less invasive endoscopic snare polypectomy instead of extended surgical resection whenever this is possible. Moreover intervention should only be performed when the lesion becomes symptomatic (risk-benefit-weight). The definite diagnosis should always be confirmed histologically.


The diagnosis of hydatid cyst in female genital tract is rare and difficult. A high degree of clinical suspicion is needed for pre-operative investigations to exclude hydatid cyst of female pelvis. The objective of this presentation is to highlight a pelvic hydatid cyst that presented as an ovarian tumour. A 22-year-old female, presented with constipation and haematuria with acute urinary retention. On examination, a mass measuring 15×13 cm was palpable in the left iliac region reaching up to the umbilicus. It was smooth, movable and non-tender and a provisional diagnosis of ovarian teratoma was made pre-operatively. At laparotomy, a cystic mass was found attached to the broad ligament, excised, and a frozen section was sent for histopathology. Gross features were consistent with hydatid cyst; the cystic wall was white and there were multiple small thin-wall daughter cysts. Microscopic diagnosis with paraffin sections showed cystic lesions with laminated wall and scolices in the daughter cyst. Indirect haemagglutination test for specific antibodies was positive (128 IU). The patient responded well to surgical excision followed by albendazole administration.This case highlights the fact that a pelvic hydatid disease may resemble neoplastic ovarian cyst, clinically and radiologically. The possibility of pelvic hydatid disease should be included, in endemic areas where differential diagnosis of cystic ovarian lesions is needed, so that the patient can be managed accordingly. Echinococcus granulosus and Echinococcus multilocularis. The disease is endemic in sheep and cattle grazing countries like India, Australia, Middle East, Africa and South America   America . It is tA 22-year-old Ethiopian lady was admitted to Mubarak Al-Kabeer Hospital complaining of urinary retention and haematuria. There was no associated fever or loss of appetite. The patient also had constipation with dull abdominal pain for the past 2 weeks. Her menstrual cycle was regular and her last menstrual period was 16 days back. The patient is single and has been working in Kuwait as a house maid for 2 years. There was no history of recent travel. There was no history of tuberculosis nor were there dogs or any pets where the patient lived. The patient denied having been on a farm.−1. The patient’s respiratory, cardiovascular and neurological systems were normal. The abdomen was soft and lax with mild diffuse tenderness but there was no rigidity. There was no hepatosplenomegaly and no ascites. A mass measuring 15×13 cm was palpable in the left iliac region reaching up to the umbilicus that had smooth surface, was movable and non-tender. There was no lymphadenopathy.On general physical examination, the patient was thin built, well nourished and pale. Temperature was 36.8 °C, blood pressure was 125/65 mm Hg and pulse was 72 beats min−1, eosinophil count 0×109 l−1, neutrophil count 28.9×109 l−1, Hb 99 g l−1 and platelet 394×109 l−1. Beta human chorionic gonadotrophin was negative. Biochemical parameters, including the liver and kidney function tests, were normal.Routine haematological parameters revealed the following: white blood cell 33.2×109 lUltrasonography of the abdomen was ordered and it revealed a large pelvic and lower abdominal multi-septated cystic mass measuring 17×12 cm that was thought to be most likely from the left ovary. There was also bilateral hydronephrosis and hydroureter most likely due to pressure changes of distal ureters from the mass lesion. The other abdominal organs were normal and there was no free fluid. In order to properly assess the mass, a magnetic resonance imaging (MRI) of pelvis was done. It revealed a large multi-loculated cystic mass, 11×13.3×14 cm, occupying the whole pelvis and extending into the left lower abdominal quadrant, which grossly compressed and deviated the uterus anteriorly and to the right of the pelvis. The mass had compressed the mid-portion of the rectum causing prominence of its proximal part in keeping with the patient’s history of constipation. The ovaries could not be seen properly, but there was no evidence of lymphadenopathy, free fluid or localized collection in the pelvis. The radiological report suggested a cystic mass that might be a neoplastic ovarian cyst, e.g. cystic teratoma.Thereafter, the patient was promptly transferred to the maternity hospital for further gynaecological consultation and laparoscopic exploration. The patient underwent exploratory laparotomy through an intra-umbilical midline incision till the symphysis pubis. A cystic mass was found occupying the left broad ligament and displacing the uterus to the right with the left fallopian tube stretched over the mass. Both ovaries were normal. Only partial removal of the cyst was possible as a small part of the cyst wall was adherent to the rectum and uterus. It was not possible to dissect the cyst wall completely and so drooling and marsupialisation was performed on the remaining cyst wall to avoid injury to the rectum and uterus. No other intra-abdominal pathology was found. The liver was explored and no lesions were found. Peritoneal toilet and irrigation with hypertonic saline of the cavity was done many times and a suction drain was left in the Douglas pouch. The mass was a cyst filled with clear fluid and multiple daughter cysts. It was sent for histopathology and serology and fluid aspirated from left broad ligament cyst was sent for culture.Histopathology report of the frozen section was as follows: macroscopic diagnosis  revealedE. granulosus antibodies as an adjunct for the diagnosis of hydatid cysts. It was positive with a reading of 128 IU (diagnostic range ≥128 IU). Bacteriological culture of the fluid revealed no growth after 48 h. Blood sent for malarial parasite was also negative.Serology test was performed on the patient’s serum using indirect haemagglutination assay (IHA) for the quantitative detection of Surgical removal of the cyst was carried out during the exploratory laparotomy. After the diagnosis of pelvic hydatid disease was confirmed, the patient was managed with albendazole 400 mg twice a day orally for 28 days, followed by a period of rest and a repeat cycle again thereafter.The infectious disease hospital was informed of the case. The post-operative period was uneventful. The patient was stable and well. The drain was removed after 24 h. The liver function and renal function test were monitored twice weekly together with the complete blood count and coagulation profile. The patient was discharged on the 7th post-operative day to be followed up in the outpatient clinic.E. granulosus that causes cystic echinococcosis (CE) and E. multilocularis that is the causative agent of alveolar echinococcosis (AE). CE and AE are the two forms most frequently encountered. Our patient presented with CE.Human echinococcosis (hydatidosis or hydatid disease) is caused by E. granulosus  (It is very important that a correct pre-operative diagnosis is made since all precautions must be taken to prevent dissemination and seeding of the surgical field. Unfortunately, the presentation in this case was atypical and, as such, the recommendation of image-based stage-specific approach for CE by Brunetti −1 day in 2 divided doses should be given, followed by 2 weeks of rest without therapy. The alternative agent, mebendazole, is poorly absorbed and must be taken at higher doses of 50–70 mg kg−1 day, for several months to achieve a therapeutic effect.According to WHO-IWGE report  when theIn conclusion, this case highlights the fact that a pelvic hydatid disease may resemble neoplastic ovarian cyst, clinically and radiologically. The possibility of pelvic hydatid disease should be included, in endemic areas where differential diagnosis of cystic ovarian lesions is needed, so that the patient can be managed accordingly.


Hospital do Servidor Público Estadual “Francisco Morato de Oliveira”, complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum . We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to  These tumors are classified into two main categories: seminomas and non-seminomas, and non-seminomas sub-classified in yolk sac, choriocarcinoma, embrionary carcinoma and mixed germ cell tumors.3 These tumors are aggressive and highly malignant, which can be explained by fast grown of germ cells, and normally unresectable at diagnosis. In this paper, we report a case of primary mediastinal yolk sac, rare subtype and highly malignant of GCT.Concerning subtype mentioned, we highlight primary mediastinal yolk sac, also called endodermal sinus tumor, first described by Teilmann et al.Hospital do Servidor Público Estadual “Francisco Morato de Oliveira”, complaining about dyspnea to efforts and dry cough for 1 year. Upon physical examination, a reduction of vesicle murmur in left hemithorax was observed. Systemic arterial hypertension and hyperuricemia were the preexisting conditions.A 38-year-old man admitted at The thorax radiography upon admission showed homogeneus densification of anterior mediastinum, suggesting round contouring mass and pleural reaction of septate aspect of left hemithorax . The folThe patient was followed-up in the thoracic surgery unit, and exams were requested to clarify the clinical picture. Exams showed elevated and rising alpha-fetoprotein (greater than 170mg/mL), without elevation of other serum tumor markers. Ultrasonography of testicle sac did not show changes.Neoadjuvant chemotherapy was initiated with bleomycin, etoposide and cisplatin, there was an increase of serum alpha-fetoprotein level. We decided to change scheme for combination of paclitaxel, ifosfamide, and cisplatin (TIP) in case of clinical response associated with reduction tumor marker.Patient underwent thoracic surgery for resection of mediastinal mass. After the surgery, he evolved with septic shock with pulmonary focus, and he was transferred to intensive care unit. After stabilize, the patient underwent new exams to disease restaging, and he was referred to clinical oncology to continuing the chemotherapy.The macroscopy observed the mediastinal mass with extensive necrotic-hemorrhagic areas infiltrating the pulmonary parenchyma by contiguity with focal vascular permeation. The resected fatty mediastinal mass as well as pericardium and surgical margins free of cancer.The histopathology showed reticular area with glandular-like structures and prominent nucleus in direction to the lumen. There was surrounded vessels by fibrous tissue located in cystic space coated by neoplastic cells radially lined forming a Schiller-Duval body, pathognomonic findings of yolk sac tumor, therefore, confirming the diagnosis of GCT of primary yolk sac tumor of mediastinum .4 These tumor probably derivate from fails in migration of primitive germ cells that migrate through urogenital crest during embryogenesis to develop regular function and hematological and immunological transport information.Extragonadal primary GCT account for 1% to 5% of all GCT, and mediastinum is the most common site, constituting 50 to 70% of all extragonadal GCT.We report a case of young man with yolk sac tumor, a type of non-seminomas GCT.6 In our report, the patient did not present lymph nodal affection nor secondary dissemination.This disease affect mainly young men and it occurs as anterior mediastinal masses , locally invasive, that trigger early metastasis by lymphatic and hematogenous metastasis, observed in approximately 25% of patients upon diagnosis.., reported in a study that most common symptom in patients with GCT are dyspnea (25%), thoracic pain (23%), fever (13%) night sweats or weight loss (11%). Fatigue, hemoptysis and superior vena cava syndrome were seen in less than 10% of patients with mediastinal GCT.7Symptoms depend on size of the injury. Bokemeyer et al9Some clinical conditions are associated with yolk sac tumor, according to literature, such as Klinefelter syndrome (around 20% of cases), precocity of pubertal development and hematological neoplasia, such as leukemia and myelodysplastic syndrome.8Alpha-fetoprotein serum levels are useful for diagnosis, and, mainly, in the follow-up of this tumor, therefore showing if there is or not a response to treatment.In yolk sac tumor, alpha-fetoprotein is elevated, and in some cases carcinoembrionary antigen can also be elevated.5However, our patient did not respond well to treatment, and we need to submit him to TIP. This fact corroborated with other studies that highlight prognosis reserved to these tumors with failure on rescue therapy, as well as high indexes of local recurrence compare with non- seminomas GCT of other local.Standard treatment for mediastinal primary non-seminomas GCT is combination of surgical resection with neoadjuvant systemic chemotherapy with bleomycin, etoposide and cisplatin.Primary mediastinal yolk sac tumor is an extremely rare tumor, diagnosed using image finding, increase of alpha-fetoprotein serum levels and histopathological evaluation of the injury. These local invasive tumors present a reserved prognosis and high recurrence rates, although there was an increase of patients’ survival after implement cisplatin-based chemotherapy. Therefore, patients with this disease need early diagnosis and specialized and interdisciplinary management.


Renal angiomyolipoma is a rare tumor that can be either sporadic or found together with tuberous sclerosis or pulmonary lymphangioleiomyomatosis. These tumors are hormone sensitive and therefore tend to grow during pregnancy and their main complication is the risk of rupture. Optimal management is still controversial because there are very few cases reported in the literature. We expect that the case of our patient, who delivered her baby vaginally at 36 weeks of gestation and underwent definitive treatment (nephrectomy) thereafter, to further enhance the knowledge about the management of these rare tumors during pregnancy. These tumors arise from epithelioid cells around blood vessels, can be sporadic, but may also be associated with tuberous sclerosis complex (TSC) or pulmonary lymphangioleiomyomatosis (LAM). Although symptoms like hematuria or flank pain may occur, they are more frequently detected incidentally during imaging for unrelated reasons.Renal angiomyolipoma (RA) are rare tumors with an incidence of 0.3% in the general population and are even rarer among women who are pregnantThese hormone-sensitive tumors  may rupture during pregnancy due to elevated levels of estrogen and progesterone; most of the cases in the literature ruptured during pregnancy. We present a patient who was pregnant whose renal mass was diagnosed during pregnancy. The patient was managed conservatively in the antenatal period without any complication, and then given definitive treatment after the birth. We reviewed the literature about cases of RA that have been managed during pregnancy.th week of gestation with left flank pain. Her medical history was not significant for any disease. The patient had no costovertebral angle tenderness on either side. Her urine analysis revealed pyuria but not hematuria. A urinary tract infection was treated with oral ampicillin. The patient’s vital signs were stable and there was no fever. Fetal biometric measures were compatible with 34 weeks and the amniotic fluid index was normal on ultrasound evaluation. A urinary system ultrasound revealed a 9.9x5.8-cm hypoechoic solid lesion on the left kidney that had distorted the renal calices. Magnetic resonance imaging (MRI) results showed a lobulated, heterogeneously contrasted 11x6.5x5.5-cm soft tissue mass lesion arising from the middle segment of the left kidney that contained necrotic areas  are the main options of treatment. For asymptomatic patients, a conservative approach may be chosen, especially during pregnancy. For these patients, definitive treatment may be postponed until the postpartum period. Similarly, we delayed the definitive treatment of the tumor to the postpartum period. However, there is as yet no consensus in the literature as to how long the conservative approach is suitable. Close follow-up of patients who are pregnant may be preferred because of the high risk of rupture.Although most angiomyolipomas are located in the kidneys, they can also be diagnosed in other organs like liver, spleen or uterus as they arise from epitheloid cells around blood vessels,8,9.Most of the cases during pregnancy reported in the literature ruptured during pregnancy . To the best of our knowledge, only four (15%) of the 26 cases reported (including ours) have not ruptured during pregnancy . These w. Therefore, estimation of the risk of rupture using only tumor size would not be accurate.The mean age and mean gestational age of the patients in the literature at the time of diagnosis was 31.4 years and 27.7 weeks, respectively. Twenty-one of these cases (81%) ruptured . The aveMost patients with RA in the literature delivered their babies via cesarean section , whereas only 5 (19%) were delivered vaginally . However. Fourteen (54%) of the patients reported in the literature needed nephrectomy, whereas 12 (46%) were treated conservatively with or without arterial embolization   and liquefactive necrosis. POS generally resolves spontaneously, whereas percutaneous drainage might be needed for liquefactive necrosis. In contrast to embolization or ablation, surgery has the important advantage of allowing for pathologic evaluation for a definitive diagnosis. Differential diagnosis of these lesions includes renal cell carcinoma, oncocytoma, and metastatic lesions from primary tumors elsewhere. Radiographic features can usually distinguish these from each other. For those patients in whom radiographic evaluation is not enough for definitive diagnosis as in our case, either biopsy or nephrectomy (as a definitive treatment) can be performed.Current treatment options for RA include partial/total nephrectomy (open or laparoscopic), cryoablation, radiofrequency ablation, or arterial embolizationlization . EmbolizIn conclusion, renal angiomyolipomas are rare tumors but physicians who encounter renal masses during pregnancy should keep these in mind, because they can grow and be symptomatic for the first time during pregnancy. MRI is usually enough for diagnosis, but for uncertain cases, biopsy or surgery can be performed. Treatment strategies should be individualized due to insufficient data in the literature to support any one in particular. More experience with these strategies is needed before an optimal treatment method can be recommended. It is our hope that the distinctive features of our case will further enhance the knowledge of the management of these rare tumors during pregnancy.


Dear Editor,A two-year-old male patient was referred to the pediatric emergency room with persistentgross hematuria. Abdominal ultrasound (data not shown) revealed an echogenic formationwithin the urinary bladder, the formation remaining fixed during changes in decubitus.Contrast-enhanced computed tomography of the abdomen showed a partially delimited,solid-to-cystic expansile urinary bladder lesion with a vegetative component, presentinglobulated contours, a small focal calcification, and enhancement of the solid component;the epicenter of the lesion was at the bladder dome . A subse,2. There have been fewer than 100reported cases of histologically proven hemangioma of the urinary bladder.Hemangiomas are benign tumor formations of capillaries and blood vessels, common invarious organs; they are extremely rare in the urinary bladder, accounting for only 0.6%of all urinary bladder tumors. A hemangioma usually presents as an incidental finding duringthe investigation of hematuria. The most common symptom is gross hematuria, which can beaccompanied by irritative urinary symptoms and abdominal pain. Urinary bladderhemangiomas occasionally coexist with cutaneous hemangioma or are associated with one oftwo conditions-5: Sturge-Weber syndrome and Klippel-Trenaunay-Webersyndrome.Most urinary bladder hemangiomas are solitary and smaller than 3 cm in diameter,affecting the dome, posterior wall, or trigone of the bladder. Although hemangiomas canoccur in individuals of any age, they are seen most often in individuals under 30 yearsof age and are slightly more common among males. Themain differential diagnoses for pigmented lesions seen on endoscopy includeendometriosis, melanoma, and sarcoma. Imaging tests, such as ultrasound, computed tomography, and magneticresonance imaging, are useful in defining the location and extent of ahemangioma.In young patients, endoscopic findings of a bluish, sessile mass and gross hematuria arehighly suggestive of hemangioma,6,7. Although hemangioma has a benign course, follow-up is mandatory inorder to detect recurrence or residual disease,7,8. The purpose of this case report was to underscore theimportance of early diagnosis of hemangioma of the urinary bladder and ofdifferentiating it from malignant neoplasms, which would affect the therapeutic strategyand patient survival.For individuals with hemangioma, the treatment is controversial. Although there are manyoptions available, partial cystectomy is currently the most widely used treatment forhemangioma of the urinary bladder


Dear Editor,A 58-year-old male was the victim of physical aggression with impalement by a broomstick.He presented mental confusion, poor response to verbal requests, tachypnea, sweating,and chest pain. Physical examination demonstrated anal bleeding. Thoracoabdominalcomputed tomography (CT) showed a well-delineated, cylindrical, hypodense (−501 HU)structure extending from the pelvis to the left hemithorax, with pulmonary lacerationaccompanied by hydropneumothorax . Thefor-6. Many transanal impalement injuriesresult from the insertion of foreign bodies (solid elongated objects) into the rectum.Transanal injuries include iatrogenic cases and those related to sexual activities andviolent actions. They often involve damage to multiple organs, depending mostly on thedirection and nature of the penetrating object-9. Impalement injuriesare often associated with vascular and visceral damage, entailing significant morbidityand mortality. However, some cases involve no major injuries, potentially because therounded ends of foreign objects act as blunt tunnelers, resulting in displacement,rather than penetration, of the major visceral and vascular structures-11. The clinical diagnosis of these injuries can be challenging. Inthe case reported here, the patient presented mental confusion and the impaling objectwas not visible on physical examination. The use of CT allowed us to establish thediagnosis and to predict the extent of the various injuries preoperatively.Acute abdominal diseases have been the subject of several recent publications in theradiology literature of BrazilIn conclusion, combined thoracic and abdominal trauma after rectal impalement is aserious medical situation that calls for the involvement of a multidisciplinary surgicalteam, with the participation of a thoracic surgeon and an abdominal surgeon. CT is auseful tool for the assessment of retained wooden foreign bodies and for the evaluationof the extent of the injuries.


Optic nerve astrocytomas (ONAs) are frequent types of optic nerve gliomas (ONGs), which can affect the visual pathway. An 18-year-old male patient was admitted to our department with right-sided intraorbital/retrobulbar swelling, which progressively grew over several months. Clinical examination showed right-sided diplopia, mydriasis, low visual acuity (0.4), exophthalmus (3 cm), epiphora, and severe retrobulbar pain. There was a family history of high-grade (IV) astrocytomas in which two of the family members died due to the disease. Preoperative MRI scan revealed a soft tissue mass around the retrobulbar area of the right eye with intact orbital bony walls. Surgery was performed whereby it was dissected freely from the muscles and was separated from the optic nerve and the globe. Histopathologic analysis confirmed a benign astrocytoma. The follow-up examination revealed no recurrent or residual tumor. A systemic review of the literature indicates that early diagnosis and experienced multidisciplinary management are required in case of unilateral, resectable forms of ONAs with no distant metastasis, in order to provide a long-time survival of patients. Surgical intervention of unilateral ONAs is a relatively safe procedure, allowing complete or partial tumor removal with minimal morbidity and low recurrence rate. Optic nerve astrocytomas (ONAs) usually affect the visual pathway, mainly in the pediatric age group. In the orbit, ONAs might arise from the optic nerve and tend to be a dura-bound fusiform swelling with distinct imaging and histologic characteristics. These tumors often extend into the optic chiasm and into the frontal brain lobe and may be associated with neurofibromatosis (type I), respectively .Pilocytic astrocytoma (PA) form of ONA is a rare, slow-growing glioma, classified as grade-I tumor by the World Health Organization (WHO)  Table 1Table 1. The macroscopic appearance of ONAs is typically well-circumscribed, cyst-like mass with a discrete mural nodule. Microscopically, the tumor often reveals a biphasic pattern in which more compact areas composed of bipolar areas and brightly eosinophilic Rosenthal-fibers that alternate with looser, spongier areas with prominent microcysts. Eosinophilic granular bodies are found in both compact and loose areas , 3.The present study reports a case of a young male patient with unilateral ONA in association with no distant metastasis. The neuroradiological and histopathologic findings and multidisciplinary management are discussed in detail.An eighteen-year-old male patient was referred to the outpatient ward of our department on November 2016. He was diagnosed with a right-sided retrobulbar tumor at the ophthalmology department of another medical institution ; however, the ophthalmological team declined to perform the surgical procedure  astrocytoma (glioblastoma multiforme), affecting the left side of temporal lobe.Ophthalmological and clinical examination revealed right-sided diplopia, mydriasis, low visual acuity (0.4), right-sided exophthalmus (3 cm), epiphora, significant eye movement disorders, and severe retrobulbar pain. Exophthalmus was not only evident, but also it clearly dislocates the ocular bulb caudally and laterally. The symptoms started slowly and progressed gradually until eye vision was severely impaired. On the other hand, exophthalmus progressed rapidly.Preoperative orbital MRI scan revealed the size and exact position of the soft tissue mass around the retrobulbar area of the right eye, its relation to the optic nerve, and the ocular bulb with intact orbital bony walls Figures  and 2.https://doi.org/10.1155/2017/4283570). When performing the surgery, we made infraciliary incision to expose the surgical field; thereafter, we bluntly dissected the orbital fat tissue and mobilized the superior rectus muscle, medial rectus muscle, and superior oblique muscle, respectively. Thereafter, we carefully and bluntly separated soft tissues from the muscular structures. Firstly, we applied pressure gauzes, hot saline-soaked gauzes, and sometimes careful and limited usage of electrocoagulation to control bleeding without damaging the surrounding structures. We could observe the tumor infiltrating the dorsal surface of the ocular bulb, closely related to the optic nerve .The tumor had a definite encapsulated and cystic character; however, it clearly showed inhomogeneous gelatinous fragments of solid tumor Figures –6. The tPrior to wound closure, we checked the eye globe mobility and tension, and we observed that no distension was detectable. The eye muscles were placed in their original anatomical position. The individual soft tissue sections were reconstructed with 4.0 PDS sutures. We reconstructed the orbicularis oculi muscle and the superior levator palpebral muscle with 4.0 Monosyn, using simple sutures. The skin edges were intracutaneously closed with 4.0 Dafilon stitches Figures 4 . The 8th day after the surgery, the patient was discharged. Histopathologic examination confirmed the diagnosis of benign glial cell tumor based on the expression of neuron-specific enolase (NSE) and synaptophysin (S-100), while cytokeratin AE1/3 reaction and Mib1 markers were negative . ClinicaOptic nerve astrocytomas (ONAs) are usually low-grade tumors although their presentation and clinical course are highly variable and unpredictable . GliomasONGs are extraordinarily rare and seldom seen in routine clinical practice. The pilocytic variant of glioma (astrocytoma) usually affects childhood or early adolescents and is benign and noninvasive in nature –8. TheseThere are two possible explanations for the origin of the tumor. One possibility is that brain heterotopia occurred during development, with subsequent sequestration and anatomic discontinuity. This would have been followed by tumor development with effacement of all heterotopic brain tissue. A second possibility is mesenchymal stem cells giving rise to the tumor. The presence of a complete layer of meninges and a cystic cavity would favor a developmental outpouching of embryonic brain rather than a mesenchymal stem cell derivation. It is interesting to note that the glioma was “invading” the surrounding orbital fat through a dural defect. It is worth speculating whether this defect may have represented the point at which the heterotopic tissue was tethered to the rest of the brain before losing its anatomic connection . In geneWhen imaged in cross section, two thirds of all ONAs demonstrate a classic appearance: a cystic mass with an enhancing mural nodule. However, less common appearances are quite nonspecific . A featuUnderstanding the natural history of these tumors is essential if patients are to receive optimal treatment. For WHO grade I gliomas, the surgical outcome is usually good . Poor prGood quality MRI has almost negated the need for surgical biopsy and is invaluable in the accurate planning of treatment . SurgicaAlthough gross total resection of the tumor is often curative, location in critical or deep areas  can render it unresectable and require additional management approaches . StrategPrompt clinical suspicion aided by imaging and histopathologic studies can aid in early diagnosis and subsequent management to improve outcome and reduce morbidity. In this case, surgical excision was done with clinical improvement seen. As mentioned earlier, follow-up MRI examination is planned few months later, and depending on the result, appropriate next management plan will be adequately applied.Surgical removal of orbital pilocytic astrocytoma via supraciliar approach (parts 1-3).


Local recurrence of colorectal cancer is a major cause of morbidity and mortality that usually implies a worse prognosis. Its etiopathogenesis is still a subject of debate. Recurrence on the perineal wound caused by anal retractor device is rarely reported.We present the case of a 75-year-old woman with perineal skin recurrence on the site of Lone Star Retractor™ from rectal adenocarcinoma. The patient underwent a curative proctectomy followed by a hand-sewn coloanal anastomosis using Lone Star Retractor™ 2 years ago for a tumor of the lower rectum. The recurrence was most likely caused by the seeding of exfoliated tumor cells into the perianal skin which was abraded by the retractor.This case is the fourth case reported in the literature and highlights the importance of the use of less traumatic endoanal retractors to prevent such perianal recurrence. Recurrence on the perineal wound caused by anal retractor device is rare but possible. Further studies are needed to define preventive measures able to reduce cutaneous implants. Local recurrence (LR) of colorectal cancer is a major cause of morbidity and mortality that usually implies a worse prognosis. The etiopathogenesis of LR is still a subject of debate, and this has led to major improvements in colorectal management –3. AfterLR on the perineal wound caused by anal retractor device is rarely reported, with only three cases reported so far, one case on the scar of a Gelpi Retractor  and two Herein, we report the fourth case of cutaneous perineal recurrence on the site of a Lone Star Retractor ™ system after a curative proctectomy and hand-sewn coloanal anastomosis for rectal cancer.A 75-year-old woman presented with a left hemicircumferential adenocarcinoma of the rectum located 4 cm above the anal verge. Her past medical history included type 2 diabetes and hypertension. Her medical history was negative for perineal or anal diseases such fistula or hemorrhoids. The tumor was classified preoperatively as T3 according to the pelvic magnetic resonance imaging (MRI) and endorectal ultrasound. On preoperative workup imaging, there was no evidence of distant metastases. Six weeks after a neoadjuvant chemotherapy, the patient underwent a laparoscopic proctectomy with total mesorectal excision (TME) and intersphincteric resection. The Lone Star Retractor™ was used for the exposure of anal verge, and the perineal dissection was performed transanally. The anal canal was washed with a povidone-iodine solution, and hand-sewn side-to-end coloanal anastomosis was created. A diverting lateral ileostomy was made. The immediate postoperative course was marked by the occurrence of a grade B anastomotic fistula, treated by a transanal drainage system. Pathologic examination of the specimen showed a well-differentiated adenocarcinoma ypT3N0 12N−/12N) with a poor therapeutic response Dworak 1. The mesorectum was complete, and the circumferential resection margin was 5 mm; the distal margin was 6 mm. The diverting stoma was closed 4 months postoperatively. No adjuvant chemotherapy was administered. On the fifth week postoperatively, the patient presented an acute dehydration with functional renal insufficiency complicated by hyperkalemia leading to cardiac arrest, which was resuscitated and recovered without visceral damage. At 14-month follow-up, the patient presented in poor general condition, with rectal bleeding and right femoropopliteal venous thrombosis. Clinical examination showed a 2-cm budding lesion on the right of the anal verge, developing from the perianal skin, 1 cm from the anal border without continuity with the coloanal anastomosis; the location of the lesion corresponded to the insertion site of one of the elastic hooks of the Lone Star Retractor (Figs. 2N−/12N wLocal recurrence is a major cause of morbidity and mortality, usually implies a worse prognosis. It may occur as a result from two potential etiopathogeneses. First, metastases through endothelial-lined channels occur to both lymphatic and hematogenous routes , which aSince the first case of cell implantation into the anal fistula in 1954 by Guiss , fistulaWe propose, as Tranchart  did, theThe management of colorectal cancer LR is still a matter of debate, it ranges from local excision –17 to a Local recurrence of colorectal cancer is a major cause of morbidity and mortality that usually implies a worse prognosis. Recurrence on the perineal wound caused by anal retractor device is rare but possible. The knowledge of this mechanism of occurrence may change surgical practices and push for further studies to define preventive measures that can reduce skin implants.


Intradiploic arachnoid cysts have scarcely been reported in the literature, mostreported cases being secondary to trauma. Nontraumatic arachnoid cysts are quiterare and have been reported mostly in adults. Here, we report the case of a16-year-old male presenting with a slowly growing mass in the occipital regionand intermittent headaches. On the basis of the findings of X-rays, computedtomography scans, and magnetic resonance imaging scans of the head, the mass wasdiagnosed as a giant intradiploic arachnoid cyst. Intraosseous cerebrospinal fluid (CSF) cysts areuncommon lesions of the cranium. The terms arachnoid cyst, leptomeningeal cyst, andCSF fistula have been used interchangeably to describe these cysts, which constitutea rare type of growing skull fracture and occur following severe head injury inindividuals ≤ 3 years of age. Nontraumatic CSF-containing cystic lesionsof the skull are extremely rare and are presumed to be congenital.Arachnoid cysts are benign developmental abnormalities of the arachnoid membrane thatcan occur anywhere along the cerebrospinal axis. They are rare lesions, accountingfor only 1% of all intracranial space-occupying lesions, most inindividuals over 50 years of age. To our knowledge, this is the first case of agiant intradiploic arachnoid cyst in a young male.Here, we describe the X-ray, computed tomography (CT), and magnetic resonance imaging(MRI) findings of a giant nontraumatic intradiploic arachnoid cyst in a 16-year-oldmale. Nontraumatic intradiploic arachnoid cyst is rare, only 15 cases having beenreported to dateA 16-year-old male presented with an 8- to 9-year history of a painless, slow-growingswelling on the scalp and intermittent headache. He had no history of head trauma.Physical examination revealed that, in the occipital region, there was a large,ill-defined, swelling that was non-tender, bony, and hard. For further evaluation,an X-ray of the skull and a CT scan of the head were ordered. The X-ray revealed alarge, ill-defined, well-demarcated, expansile, multiloculated osteolytic lesion inthe occipital bone . The CT On the basis of the CT and MRI findings, a diagnosis of giant intradiploic arachnoidcyst was made. The patient was advised to undergo exploratory surgery but refusedand was therefore discharged with a prescription for analgesics. At one month offollow-up, he was asymptomatic..The terms arachnoid cyst, leptomeningeal cyst, and CSF fistula have been usedinterchangeably. They are described as benign intradiploic cystic lesions of thecranium. The characteristic findings in these lesions are an intact outer table andCSF-filled cyst which communicates with the subarachnoid space intracraniallythrough a defect in the inner table. These cysts are not entirely between the twotables of the skull, because they almost always feature a communication with theintracranial subarachnoid space,5. There isherniation of the arachnoid membrane through the bony fissure, caused by pulsatileCSF flow,7. Over the course of several years, continuous pulsationserode the edges of the fractured inner table and lead to the development ofintraosseous cysts. The intradiploic cyst thus formed elevates the outer table andflattens the inner table.Intraosseous CSF-containing cysts are typically seen in association withpost-traumatic growing skull fractureNontraumatic CSF cysts are comparatively much rarer than are those occurring afterhead trauma. Nontraumatic intradiploic arachnoid cysts develop as a diverticulum ofthe arachnoid membrane through small defects in the dura mater. The most commonlocation is the suboccipital midline region. Other sites include the frontotemporalregion and anterior cranial fossa. Although most patients are asymptomatic and arediagnosed incidentally, some present clinically with progressive swelling, with orwithout headache.. OnCT scans, it presents as an intradiploic cystic lesion, in a location associatedwith bone remodeling and communicating with the cistern magna. On T1- andT2-weighted MRI scans with complete suppression on FLAIR, as well as ondiffusion-weighted images, a fluid-intensity lesion can be seen. A definitecommunication with the subarachnoid space can be demonstrated in the majority ofcases.Intradiploic arachnoid cyst is a rare osteolytic lesion of the skull. It ischaracterized by multiple, symmetrical, well-demarcated occipital osteolytic lesionson routine skull radiography, who characterizedthem as multiple, well-demarcated, osteolytic lesions, commonly found in theparasagittal occipital region. Due to rarity of the condition, its natural historyremains unclear. The lesions are characteristically thin-walled and enlargegradually, rendering the bone wall papery thin. Normal CSF pressure would not beenough to cause an expansile lesion in intact bone, and this process is thereforepresumed to have started before the ossification of the cranium. In some cases, asmall amount of dysplastic brain tissue has also been seen herniating, together withCSF, through the bony defect.Nontraumatic intradiploic arachnoid cysts are rare, only 15 cases having beenreported to date. These cysts were first described in 1989 by Weinand etal..The various differential diagnoses of CSF-containing, intraosseous, expansile cysticbony lesion of the brain include intraosseous epidermoid/dermoid cyst, aneurysmalbone cyst, fibrous dysplasia, eosinophilic granuloma, hydatid cyst, and malignantconditions such as plasmacytoma, myeloma, osteogenic sarcoma, and intraosseousmeningioma. Eosinophilic granulomas and neoplasms are osteolytic rather thanexpansile lesions and their contents are not of CSF density. Fibrous dysplasiacystic lesions are usually quite small and multiple with attendant sclerosis ofbone. The use of MRI, especially with fat suppression sequences, can help excludeepidermoid and dermoid cysts,11.The surgical management of these cysts includes excision of the pedicle and repair ofsmall dural and bony defects. However, given the benign nature of the lesion, smallasymptomatic lesions can be monitored radiologically


Introduction: Primordial odontogenic tumors are a rare recently described mixed odontogenic tumor composed histopathologically of dental papilla like tissue and enamel organ like tissue. Only nine cases have been documented worldwide and we are reporting the tenth case which is from Egypt.Clinical finding: A 2-year-old Egyptian boy that presented with an asymptomatic swelling of the mandible which appeared with multilocular radiolucency associated with an impacted developing tooth on a computerized tomography (CT) scan.Diagnoses, interventions, and outcomes: The lesion was excised and diagnosed as a primordial odontogenic tumor. The patient was followed up for two years with no recurrence.Conclusion: Differentiation of primordial odontogenic tumors from other odontogenic tumors, which resemble it histopathologically is crucial to avoid unnecessary aggressive treatment. This tumor has been described as other entities in the past, because of its histological similarity to other odontogenic tumors as ameloblastic fibroma, odontogenic myxoma, and odontogenic fibroma and hyperplastic dental follicles2.Primordial odontogenic tumor (POT) is a recently described mixed odontogenic tumor described in the last WHO classification of head and neck tumorset al.3 described and denominated this novel lesion which did not fulfil the criteria of any of the previously classified odontogenic tumors by reporting the clinicopathological and immunohistochemical features of six cases diagnosed as primordial odontogenic tumor.Mosqueda-Taylor1.Primordial odontogenic tumors are characterized histologically by a variably cellular loose fibrous tissue with areas similar to the dental papilla, covered by cuboidal to columnar epithelium that resembles the internal epithelium of the enamel organ, surrounded at least partly by a delicate fibrous capsuleOnly nine cases have currently been reported, and we report an additional case of an primordial odontogenic tumor from Egypt.A 2-years-old Egyptian boy referred to Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Cairo University in November 2015 with a fleshy swelling arising from site of marsupialization performed two months previous. By taking patient’s history we established the lesion arose as a painful swelling covered with normal mucosa causing obliteration of the vestibule with two months duration. Manual examinations of the regional lymph nodes were negative on examination. By computerized tomography (CT) scan, a multilocular radiolucent lesion was seen associated with an impacted developing tooth in the mandibular posterior area measuring 3cm × 4cm .On aspiration, straw cystic fluid was noted. Complete surgical excision of the lesion with the impacted tooth was performed. And the excised lesion was sent to the Department of Oral and Maxillofacial Pathology, Faculty of Dentistry, Cairo University. The gross specimen showed a cystic lesion which showed areas of thickening. Hematoxylin and eosin stained sections revealed surface columnar and cuboidal epithelium covering a loose and myxoid fibrous tissue  and thisRaw histological imageA photomicrograph of Hematoxylin and eosin (H&E) stained sections showing primitive connective tissue stroma covered by columnar epithelium, (×200)Click here for additional data file.Copyright: © 2018 Amer H et al.2018Data associated with the article are available under the terms of the Creative Commons Zero "No rights reserved" data waiver (CC0 1.0 Public domain dedication).et al.3. Then another two cases were reported in 2015 and 2017 by Slateret al.4 and Andoet al.5 respectively then in 2018 Bajpai and Pardhe6 described another case. This novel lesion was added to the new WHO classification of odontogenic tumors1.POT is a new entity first reported in a case series of 6 cases in 2014 described as benign mixed odontogenic tumor by Mosqueda-TaylorAll reported lesions were expansile and asymptomatic which are opposite to our case as it was painful during presentation, which may be the result of the previous marsupialization.6 who reported a case that appeared mulitlocular. Radiographically, POT presents with a well-defined radiolucent lesion, either unilocular or bilocular, except in the case of Bajpai and Pardheet al.3, as did our present case, where loose and myxoid connective tissue stroma resembles the dental papilla covered by columnar epithelium of a single layer, with the epithelium resembling the inner enamel epithelium.All documented cases shared similar histopathological criteria proposed by Mosqueda-TaylorRegarding the treatment approach, all previously documented lesions were treated with enucleation with different periods of follow up and reported no recurrence, in line with our presented case.In conclusion, this is the first report case of POT from Egypt after it was defined in the latest WHO classification. Differentiation between POT and other closely resembling odontogenic tumors is crucial, especially in the case of odontogenic myxomas as it is a more aggressive tumor and requires more aggressive treatment.The clinical, radiographical and histopathologic data of the nine previously documented cases in addition to our case will be useful to differentiate this new tumor from other odontogenic tumors, which resemble it histopathologically, to avoid unnecessary aggressive treatment modalities.Written informed consent for publication of clinical details and images was obtained from the patient's parent.The data referenced by this article are under copyright with the following copyright statement: Copyright: © 2018 Amer H et al.Data associated with the article are available under the terms of the Creative Commons Zero "No rights reserved" data waiver (CC0 1.0 Public domain dedication).10.5256/f1000research.14735.d2022147Dataset 1: Raw histological image. A photomicrograph of Hematoxylin and eosin (H&E) stained sections showing primitive connective tissue stroma covered by columnar epithelium, (×200). The case report article provides a good idea about POT especially because they are rare tumors and only a few cases have been reported. Article also writes all diagnosis methods and treatment outcomes and compared all cases of POT. Follow up of cases, I think, need a longer period than two years to decide no recurrence.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This is an interesting case report describing in a full details a rare recently described mixed odontogenic tumor. I would prefer the authors to suggest further diagnostic test to differentiate peimordial odontogenic tumor from the aggressive odontogenic myxomas especially when cases unexpectedly appear radiographically multilocular radiolucent as in the present case.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The case report presented here is a well constructed case, the rarity and novelty of the lesion makes it useful for the clinicians and indeed, as stated, it may lead to a more conservative treatment and save the patient an aggressive treatment. The young age of the patient is interesting and gives a  probable reassurance for the parents as only a conservative treatment is needed. A clinical picture for the patient would have been useful if present and also a clinical picture after full recovery. A need to switch the magnification on the histopathologic pictures is to be done.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This is a well-written case report describing a rare entity which was recently described. I would encourage the authors to add more histopathological details and elaborate on the discussion section mainly focusing on important differentiating points with histopathological mimics.here for the annotated PDF of this article. Please clickI have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


We present the case of a 61-year-old patient who was evaluated for benign infravesical obstruction due to a pseudosarcomatous fibromyxoid tumor of the prostate. This entity is rare and difficult to distinguish from a malignant lesion. A discussion of the pathological features and a review of the literature are given.


Three dogs were presented with lumbosacral hyperesthesia. Computerized tomography scans were performed in all the cases, and magnetic resonance imaging was also performed in cases 1 and 3. There was intervertebral disc (IVD) protrusion causing nerve root compression and epidural gas accumulation in all the three cases. The gas-filled cystic structures in cases 1 and 3 were within the spinal canal; in case 2, the gas was within the disc protrusion. The IVD vacuum phenomenon is relatively common in dogs, but the formation of an epidural gas accumulation in cases of a lumbar disc protrusion is rare. The clinical significance of these epidural gas accumulations is unknown. Two of the dogs were treated surgically, improved after surgery, and showed no signs of pain in the follow-up examinations. Pneumorrhachis is a rare clinical diagnosis of gas within the spinal canal and was reported for the first time in human medicine in 1977 . EpiduraA 2-year-old intact male cane corso dog was presented for the evaluation and therapy of lumbosacral pain. The results of the general examination were unremarkable. During the orthopedic and neurologic examinations, a clear pain reaction was found during the palpation and extension of the lumbosacral junction. The computerized tomography (CT) and magnetic resonance imaging (MRI) scans showed reduced signal intensity and marked dorsal protrusion of the L7–S1 IVD together with a VP. A surgical dorsal decompression for removal of the protruded disc was performed, and the dog improved to a normal condition within 2 months of rehabilitation.Three years after that surgery, the dog was referred again because the clinical signs had recurred. Palpation of the lumbosacral junction caused pain, and the dog vocalized when standing up or climbing stairs. There were no neurological deficits. A new CT scan showed that a lumbar disc protrusion with an epidural gas accumulation had formed, compressing the cauda equina from the ventral side Figure . Surgicavia a dorsal laminectomy L7-S1 and a partial discectomy  lesion with a large fragment in the vertebral canal on the left side caudal of the L7-S1 IVD. Cranial of the OCD fragment, an IVD protrusion with gas inclusion could be seen in the vertebral canal on the right Figure . Becausey Figure . After 6A 4-year-old castrated male French bulldog was referred with a history of progressing ataxia of the pelvic limbs. The blood values and results of the general examination were normal, but marked ambulatory ataxia of both pelvic limbs with reduced proprioception was recorded. The reflexes of the pelvic limbs were increased; the thoracic limbs were normal. In addition, strong pain was detected in the lumbosacral area. With the clinical localization of the neurological deficit causing pathology within the spinal cord segments Th3–L3 and the pain in the lumbosacral area detected during the clinical examination, CT and MRI scans of the thoracolumbar and lumbosacral spine were performed. In the area of Th6–Th7, malformations of the vertebrae with ventral spinal cord compression were detected together with a T2-hyperintense signal change within the spinal cord. These findings could explain the ataxia and proprioceptive deficits of the pelvic limbs. Furthermore, a small VP and other degenerative changes of the L7–S1 IVD were visible. The L7–S1 IVD was protruded, and a gas-filled cystic structure within the spinal canal had developed, leading to moderate ventral compression of the cauda equina  is an important cause of chronic pain in dogs . In thisThe VP, which refers to the presence of gas in the IVD, is a relatively common radiological finding in humans and dogs, especially from CT, with an incidence of 6% in canine vertebral CT scans , 30, 31.Computerized tomography is the investigation of choice for diagnosis , 15, 34.The IVD VP is a sign of IVD degeneration and is relatively common in dogs, but the formation of a lumbar disc protrusion with an epidural gas accumulation is rare. This gas-filled cystic structure can be a part of tissues compressing the cauda equina; however, the clinical significance is unknown. In this study, the surgical treatment of the clinical DLSS by removing all the causes of the stenosis, including the epidural gas accumulation and disc protrusion, was successful in the two cases in which it was performed, although follow-up was limited.The study describes three cases of canine patients treated at our hospital following the best knowledge of the authors.DS contributed to writing the manuscript and literature review. HS contributed to writing the manuscript and was the primary surgeon for the cases.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Intestinal malrotation occurs if midgut does not complete or partially completes its 270° counter-clockwise rotation around the superior mesenteric artery during embryologic life. In general, it frequently manifests with vomiting due to duodenal obstruction and volvulus in the initial months of life, and it is very rare to manifest in the adulthood. A 20-year-old male patient who had severe abdominal pain, nausea, vomiting, and distention for one day was evaluated at the emergency department. On abdominal tomography “swirling appearance of structures around the superior mesenteric artery” was reported. CT appearance was considered compatible with a rotational anomaly. Emergency surgery was planned for the patient. In laparotomy, it was observed that an approximately 100 cm long small intestine segment was rotated around a band (Ladd) and ischemia was developed in this segment due to rotation of its mesentery. The rotation of the small intestinal mesentery was corrected by opening the bands. After the warm application to the intestinal mesenteric ischemia for a while, the color and the peristalsis of the intestines became normal. The patient was discharged on postoperative day 2 with suggestions. Intestinal malrotation occurs if midgut does not complete or partially completes its 270° counter-clockwise rotation around the superior mesenteric artery during embryologic life . Treitz In this case, we present a 19-year-old patient who had acute abdomen and had small intestinal ischemia due to volvulus and discuss the relevant literature.3) in laboratory tests. The erect abdominal direct graph was noncontributory. On abdominal tomography “swirling appearance of structures around the superior mesenteric artery” was reported  is the most specific finding of malrotation . On coloLadd's procedure is the elective surgical treatment of intestinal malrotation since its first description in 1936 . In thisIn conclusion, intestinal malrotation in adults, although rare, is an important anomaly since its complication may require emergency surgical intervention. Although its diagnosis in adults is difficult due to atypical presentation, intestinal malrotation should be considered in the differential diagnosis of patients with recurrent abdominal pain, intestinal obstruction, and dyspeptic complaints.


Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. The least common form of these malformations is Herlyn-Werner-Wunderlich syndrome characterized by obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). The most common presentation of this syndrome is a mass secondary to hematocolpos, pain, and dysmenorrhea. Clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan.We report two cases of this syndrome, featuring two very rare clinical presentations: hematosalpinx and pyocolpos. The clinical course of the pathology is not standard and each patient is treated accordingly. A didelphic uterus with an obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital anomaly constituting 0.16–10% of all Müllerian duct abnormalities described only in case reports in medical literature . It is mWe present two cases of OHVIRA syndrome with two different rare clinical presentations.The first case is a 16 year-old Iraqi patient referred to our clinic for chronic pelvic pain and progressive painful distention of the lower abdomen. Her medical history was uneventful and menarche was at the age of 12. The clinical exam showed normal external genitalia. The vagina was occupied by a large soft painful mass and a normal cervix was identified on the speculum exam. The pelvic ultrasound showed two endometrial cavities, the left side being dilated with a fluid collection. It also showed a para-ovarian left pelvic mass suggesting a hematosalpinx, and a vaginal collection of 6x4 cm2. Abdominal ultrasound showed the absence of a left kidney. Subsequently, MRI was performed to better characterize the pelvic anatomy and better identify the anatomic location of the pelvic fluid collections. A didelphic uterus was demonstrated. The right endometrial cavity appeared normal along with the right cervix. On the left side, it showed a hematosalpinx. The endometrial cavity was distended, and contiguous with the left obstructed hemivagina .The patient was scheduled for surgical treatment. The laparoscopy showed a bicornuate uterus with a dilated left horn, and an ipsilateral hematosalpinx. The posterior wall of the vagina was bulging into the peritoneal cavity. No endometriosis implants were identified .The second case is a 20 year-old Iraqi patient, who presented to our clinic for a 2 years history of primary infertility and recent dyspareunia. History was uneventful and menarche was at the age of 13.The clinical exam showed normal external genitalia, and a vaginal fluctuant painful mass obliterating the right part of the vagina. The speculum exam showed a normal cervix. Ultrasound showed a didelphic uterus with a 3x4 cm2 homogenous collection in the vagina. Abdominal ultrasound showed the absence of a right kidney. An OHVIRA syndrome was suspected and the patient was sent for an MRI for further evaluation. The MRI showed a bicornuate uterus, with two crevices, the right one communicating with a right vaginal collection due to a longitudinal hemi vaginal septum .The laparoscopy showed a bicornuate uterus and normal adnexa. Unlike the first case, the vaginal collection was not clearly identified .Whereas in the first case we proceeded with a left salpingectomy, we treated both vaginal collections with the same approach: vaginoplasty with excision of the vaginal septum. In the first case, the drainage of the collection showed a dark hematic thick fluid. Surprisingly, excision of the right vaginal septum in second case drained a purulent discharge. Cultures grew out sensitive Escherichia coli .In both cases, we performed a marsupialization of the vaginal edge after a large excision of the septum to prevent re-obstruction and the recurrence of collection .Müllerian duct abnormalities cover a wide range of developmental anomalies, resulting from non- development, defective fusion, or defects in regression of the septum during fetal development. Herlyn-Werner-Wunderlich syndrome (HWW) is a rare disease comprising a triad of didelphic uterus, obstructed hemivagina and renal agenesis  . It is cA mass secondary to hematocolpos due to retained menstrual flow is a characteristic clinical finding while long standing dysmenorrhea and vaginal pain are the most frequent symptoms . In incoIt has been postulated that reversed menstruation increases the risk of endometriosis, pelvic adhesions, infertility and pelvic inflammatory disease . NeverthThe first patient, although 4 years younger than the second, was expected to experience less number of menstruations, yet she had larger hematocolpos and hematosalpinx, demonstrating the individual response to vaginal expansibility to accommodating large volume of menses and to change the course of the pathology.Both patients had intercourse however only the older patient with a smaller collection without any history of fever or shivering, presented pyosalpinx, probably due to an occasional low immunity and consequent ascending E. Coli infection. A larger number of patients are necessary to determine factors that affect the course of the pathology in term of blood amount and infection risk. However, infection and pyocolpos risk is higher once intercourse is reported.Ultrasound and MRI are the modalities of choice for the diagnosis and surgical planning of OHVIRA. Although ultrasound can be used to diagnose this condition, MRI is superior to ultrasound by allowing better uterine shape characterization and anatomic relationships of adjacent organs, given its multiplanar capabilities and wider field of view.Despite its high sensitivity, MRI couldn’t identify preoperatively the purulent character of the collection in the second case. In fact we kept a low index of suspicion since the patient never reported episodes of high fever or shivering. We conclude that diagnosis of infected hematocolpos should be kept in mind even in the absence of the infection clinical manifestations such as fever and chills.Most surgeons completely excise the vaginal septum and then both normal vaginal mucosal edges are sutured together over the defect. Others prefer to marsupialize the edges of the septum . Large eWe performed laparoscopic salpingectomy in the case of associated hematosalpinx. As recommended by Grimbizis et al.  in the cWomen with uterus didelphys have a high pregnancy rate of 80%  but withOur patients were followed up for a year after surgery, there was no recurrence of the collections, but none of them got pregnant.OHVIRA should be suspected in menstruating women with cyclic pelvic pain, vaginal mass, and unilateral renal agenesis. The syndrome can present with associated hematosalpinx or pyocolpos that can happen independently of the pathology course. In the presence of MRI characterization, laparoscopy has a limited role as a diagnostic tool, but remains necessary for treatment of associated tubal pathology. Dr. Zaki Sleiman is a consultant for Karl Storz endoscope. He declares that his relation with the company mentioned above has no impact upon the scientific value and the content of the submitted.


Brachial cleft cysts are rare congenital malformations of the lateral neck and typically present in the second and third decades of life. Many theories have been proposed to explain the aetiology of these cysts including incomplete obliteration of branchial mucosa, persistence of vestiges of the precervical sinus and cystic lymph node origin. Preoperative imaging investigations show a fluid-filled cyst and evaluate anatomic relationships. Differential diagnosis includes metastatic squamous cell carcinoma, lymphadenitis, lymphoma, cervical dermoid cyst, cystic hygroma and parotid pathology. Surgical excision is the treatment of choice and results in a good prognosis. We present the case of a 37-year-old woman referred to our department for assessment of a neck swelling that had gradually increased in size during the previous nine months. The clinical examination found a 6 cm non-tender mass of the upper-left neck. The MRI revealed a cystic lesion measuring 4×5×6 cm, displacing the sternocleidomastoid muscle posterolaterally, and the carotid and internal jugular vein medially. The mass was completely resected through a lateral cervicotomy. The histological examination revealed a cystic wall lined with stratified squamous epithelium consistent with the diagnosis of a branchial cleft cyst. The postoperative course was uneventful. Long-term follow-up has shown no evidence of recurrence.


Ulcerative colitis has long been linked with an increased risk for colonic adenocarcinoma, whereas Crohn's disease (CD) has recently been reported to pose a similar increased risk. We report a 33-year-old healthy female with no family history who presented with abdominal pain and a colon mass. Histopathology revealed a moderately differentiated adenocarcinoma extending through the muscularis propria with metastatic lymph nodes and intact mismatch repair proteins by immunohistochemical expression and gene sequencing. The nonneoplastic grossly uninvolved background mucosa showed marked crypt distortion, crypt abscesses, CD-like lymphoid hyperplasia, transmural inflammation, and reactive epithelial atypia. Additional patient questioning revealed frequent loose stools since she was a teenager leading to diagnosis of a previously undiagnosed CD without primary sclerosing cholangitis (PSC). The adenocarcinoma is suspected to be related to the underlying CD. Newly diagnosed adenocarcinoma in a young female as the presenting sign for CD in the absence of PSC is extremely rare. Patients with ulcerative colitis (UC), particularly long-standing and extensive disease, have long been established to have an increased risk for developing colonic dysplasia and colonic adenocarcinoma –6. CrohnInflammatory bowel disease that is associated with primary sclerosing cholangitis (PSC) results in different clinicopathologic parameters as compared to inflammatory bowel disease that is not associated with PSC. Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease that is characterized by mucositis and fibrosis, which causes strictures of the intrahepatic and extrahepatic bile ducts . Sano etOur patient is a 33-year-old previous healthy female with no documented past medical history and insignificant family history who presented with severe abdominal pain, nausea, and emesis in July 2012 at an outside institution. A computed tomography (CT) scan of the abdomen revealed thickening of her cecum. She was taken to emergent surgery for what was thought to be a perforated appendix. Intraoperatively, the procedure was switched to a right hemicolectomy due to the presence of a right-sided colon mass.Gross pathologic examination of the 14.8 cm in length right hemicolectomy specimen revealed a 7.3 cm cecal mass extending to the ileocecal valve and terminal ileum with no macroscopic perforation. Histopathology revealed a moderately differentiated colonic adenocarcinoma with invasion through the muscularis propria into the subserosal adipose tissue and areas of CD-like lymphoid aggregates adjacent to and deep to the tumor with mild to moderate intratumoral lymphocytes Figures . While FMismatch repair protein (microsatellite instability [MSI]) immunohistochemical stains were performed at the originating outside regional hospital and revealed no loss of MLH1, MSH2, MSH6, and PMS2 protein expression within the invasive tumor cells Figures . This isThe patient then underwent eleven cycles of adjuvant FOLFOX chemotherapy, which was completed in January 2013. A follow-up CT scan of her abdomen in February 2013 showed diffuse colonic thickening, which was concerning for inflammatory bowel disease versus irritable bowel syndrome. Of note, the CT scan revealed no evidence of PSC. In June 2013, a colonoscopy demonstrated patchy erythema, change in vascular pattern, mild friability with no spontaneous bleeding, numerous superficial ulcerations, and granularity in the colon in a patchy distribution. Biopsies revealed cryptitis in the terminal ileum and chronic crypt destructive colitis in the right colon, transverse colon, left colon, and rectum. In July 2013, a positron emission tomography (PET) scan revealed a 1.3 cm paraduodenal lymph node anterior to the inferior vena cava and multiple mesenteric lymph nodes with diffuse uptake. Endoscopic ultrasound-guided biopsy revealed metastatic carcinoma. She was placed on FOLFIRI chemotherapy.Upon further questioning, the patient reported chronic diarrhea with loose, frequent bloody stools since she was 14 years old. She was referred to the gastroenterology service of our university health system for consultation to confirm her diagnosis of inflammatory bowel disease and plan treatment. The initial right hemicolectomy case was sent to us to evaluate for presence of inflammatory bowel disease, to confirm whether the colitis and chronic diarrhea were a result of inflammatory bowel disease or the chemotherapy regimens.Upon our pathologic review of the right hemicolectomy case, the background nonneoplastic colonic mucosa located a few centimeters away from the tumor was noted to show focal chronic-active colitis characterized by marked crypt distortion, crypt abscesses, and reactive epithelial atypia  highlighted presence of all four proteins with no loss of protein expression, confirmed with no pathogenic mutations detected on gene sequencing for the four MSI genes. Thus, HNPCC/Lynch Syndrome is very unlikely, given the combination of a normal mismatch repair protein expression panel, no pathogenic gene mutations, and no family history of malignancy. The etiology of this young woman's colonic adenocarcinoma is suspected to be related to the ongoing inflammation of the previously undiagnosed (and untreated) inflammatory bowel disease.This case highlights the necessity to scrutinize the “uninvolved” sections of colonic mucosa in colon cancer cases for evidence of inflammatory bowel disease. Not only may it provide supplemental information to account for symptoms, but it can influence treatment and surveillance regimens after the carcinoma has been treated with surgery and chemotherapy.