Metastatic presentation of leiomyosarcoma in the heart is very rare. We present transthoracic echocardiography and combined PET/CT images of a case with a large right ventricular metastasis of leiomyosarcoma. The patient was placed on cytostatic drugs for palliative purposes, but passed away one month later because of an untreatable ventricular tackycardia. The word sarcoma is derived from the Greek word fleshy substance, and this group of neoplasm constitutes a very heterogeneous group . Leiomyo18F-fluorodeoxyglucose (FDG) [The patient was a 69-year-old woman who 3-years earlier had undergone surgical resection of a leiomyosarcoma located in the corpus uteri. A recent chest X-ray had showed a tumor-suspected structure in the left lung. This was shown by fine needle biopsy to be a metastatic tumor of leiomyosarcoma. For primary tumor staging the patient underwent a combined computed positron emission tomography (PET) and computer tomography (CT) examination by an integrated PET/CT system , after injection of se (FDG) . Figure The present case report includes transthoracic echocardiography and combined PET/CT images of a rare case with a large right ventricular metastasis of leiomyosarcoma. Metastatic manifestations of a leiomyosarcoma to the heart are associated with very poor prognosis and our patient passed away only one month later because of an untreatable ventricular tackycardia. The use of a radiopharmaceutical agent such as FDG provides the capability for imaging tumor glucose metabolism, whereas CT images provide anatomical information . The CT The echocardiography diagnosis of a tumor within the heart is not always straightforward . DiffereThere is naturally no consensus or any guidelines for the optimal management of patients with ventricular metastasis of leiomyosarcoma. Surgical resection has been attempted in some cases ,5, and tThe authors declare that they have no competing interests.MD performed the echocardiography and wrote the manuscript. SV were responsible for the PET/CT. MS was the clinician. All authors approved the final version of the manuscript.Movie 1. Movie of echocardiography examination from an apical four-chamber view. Note the significant reduction of contractility in the interventricularseptum, presumably due to tumor growth.Click here for fileMovie 2. Movie from a parasternal long-axis view that show tumor engaging the interventricularseptum.Click here for fileMovie 3. Movie from a parasternal short-axis view that show the obliteration of the right ventricular cavity because of tumor growth.Click here for file


Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas. They are considered to carry a poor prognosis with current therapeutic approaches. Successful treatment depends on a multimodal approach.The authors report two cases with malignant peripheral nerve sheath tumors arising from pre-existing neurofibromas in the grounds of neurofibromatis-type I. Complete surgical removal of all lesions is considered before and after induction chemotherapy. Correlation of the response to chemotherapy in the context of the immuno-histopathological features of the tumors is also discussed with reference to the existing literature.A need for a multidisciplinary approach with chemotherapy, surgery and radiotherapy is anticipated in the management of malignant peripheral nerve sheath tumors as described in these two reported cases. It is felt that further research on the molecular aspects of malignant peripheral nerve sheath tumors and neurofibromatis-type I will optimize treatment strategies in the future. Neurofibromatosis (NF) is an autosomal dominant disorder of neural crest origin affecting all three germinal layers. It can therefore involve in any organ system. Clinically two distinct types are recognized; NF-type 1 (NF1), or von Recklinghausen disease affecting 85% of patients, and NF-type 2 (NF2), or bilateral acoustic neuromas/schwannomas affecting 10% of patients [Neurofibromas in NF1 may undergo malignant degeneration in 3% of patients. Schwannomas are composed entirely of Schwann cells, and malignant transformation is extremely rare; however, when it occurs, it is associated with von Recklinghausen disease in 75% of patients. NF1 is associated with an increased incidence of malignant neoplasms at any age, with a predominance of intracranial neoplasms such as, optic tract gliomas, cerebral gliomas, cranial nerve schwannomas, hamartomas, and craniofacial plexiform neurofibromas [Malignant peripheral nerve sheath tumors (MPNST) constitute a heterogeneous group of malignant tumors that probably arise from cells of the peripheral nerve sheath and are categorized as soft tissue sarcomas with an associated poor prognosis and in general, limited treatment options. Factors contributing to tumor progression remain largely unknown and undefined. They represent one of the most frequent non-rhabdomyosarcomatous soft tissue tumors in pediatric age and usually occur in young adults from a previously anticipated plexiform neurofibroma in the context of NF1, with a noted change in size and pain . At presth right rib extending to the adjoining bone and muscles, representing a second primary tumor. She immediately started neo-adjuvant chemotherapy with ifosfamide 2 gm/m² and doxorubicin 60 mg/m²/day × 3 days followed by cisplatin 100 mg/m² and doxorubicin 60 mg/m² every three weeks. Dose reduction in all regimens was necessary because of grade IV neutropenia. The patient completed four cycles and a new MRI revealed minimal reduction in size of both tumors, which did however, made surgical operation possible. In April 2005, she was submitted to a surgical removal of the tumor in her leg. The adjoining bone, nerve, and vascular structures remained intact. The biopsy confirmed the presence of an 18 cm MPNST with pathological features of intense mitotic activity and nuclear atypia, vascular infiltration with surgical margins being free of tumor. The patient recovered uneventfully after surgical removal of the leg lesion and was planned to be submitted to a second operation for the excision of the lesion in the chest. However, two months later she presented in the emergency room with acute respiratory failure with chest X-rays revealing rapid enlargement of the thoracic mass compressing mediastinal structures. She developed a cardiac arrest and died despite intensive resuscitation efforts.A 23-year-old white female with a previous known history of NF1 was admitted in August 2004 after having noticed a painful, enlarging mass in her left lower leg, which rapidly increased in size within the last two months. An MRI was performed in December 2004 and revealed an extensive plexiform neurofibroma in the lower leg extending from the superior margin of the distal thigh to the ankle with areas of cystic necrosis and hemorrhage . Howevercafé-au-lait spots . No grade 3 or 4 myelosuppression was noticed. The patient continued chemotherapy with ifosfamide 2 gr/m2/day over days 1-5, alternating every three weeks with cisplatin 100 mg/m2 and doxorubicin 60 mg/m2 both administered on day one and recycled every 3 weeks × 4 cycles. Grade 4 neutropenia was observed after cisplatin-doxorubicin administration, however no dose reduction was carried-out. A new radiographic evaluation performed in May 2005 showed complete eradication of the tumors in the dorsum and spine and a significant size reduction of the axillary nodal block. Thereafter, the patient received chemotherapy with carboplatin AUC2 and paclitaxel 40 mg/m2 administered once weekly concurrently with radiotherapy on all sites of residual disease for a total of four weeks. After the end of chemoradiation, complete remission (CR) was achieved. Eight months later, the patient developed multiple lung metastases and dyspnea. He was not subjected to further chemotherapy and died from progressive disease in the lung.A 70-year-old white male with a previous history of NF1 was referred to our hospital in February 2003, where physical examination revealed the typical neurofibromas over the skin, the anterior and posterior chest and abdominal walls and it spots  and in ait spots . A thoraMalignant peripheral nerve sheath tumor (MPNST) represents a dramatic complication of NF1 and approximately 2-5% of patients with NF1 develop MPNSTs usually arising from pre-existing plexiform neurofibromas see . The minIn case 1, we analyzed our patient's original tumor after surgical excision with immunohistochemistry see . The resIn case 2, complete remission was achieved in our patient after a multimodal approach. A successful surgical excision of all lesions with tumor-free margins was feasible in the first place and this approach remains the cornerstone of management of MPNST. Chemotherapy with carboplatin and etoposide provided no benefit in disease control leading to SD. This is in contrast to a previous report, showing promising results with this combination as first-line therapy. A possible explanation for this lays in the fact that immunohistochemistry of our patient's biopsy demonstrated diffuse lack of expression of TopoIIa marker with only focal, positive expression in 10-20% of neoplastic cells. However, no data exist to date regarding any correlation between TopoI or TopoIIa level of expression and drug efficacy. The administration of ifosfamide plus doxorubicin appeared favorable and should be considered as the mainstay chemotherapeutic regimen of this disease as pertains to other common soft tissue sarcomas. Moreover, radiotherapy had an important role in improving local control in the areas with minimal residual disease.In conclusion, the currently described cases emphasize that MPNSTs represent rare tumors that often occur in patients with NF1. Surgical resection represents the mainstay of treatment with consideration of neoadjuvant chemotherapy pre-operatively in order to enhance resectability . Radiati


A true incidence of postcoital fistula is not known as it is seldom suspected. It presents as acute pneumoperitoneum with signs and symptoms same as that of perforation of a hollow viscus. A 38-year-old parous woman presented with postcoital fistula 10 weeks after panhysterectomy for carcinoma ovary stage IIIc. Pneumoperitoneum was detected, with large amount of gas under the diaphragm on radiograph of the abdomen. A breach was found in the vaginal vault. All other causes of fistula were excluded. Fistula healed spontaneously on follow-up. The most common causes of pneumoperitoneum are iatrogenic: postoperatively after laparotomy or laparoscopy. These findings usually resolve by 1 week postoperatively. Nonsurgical pneumoperitoneum accounts for approximately 10% of all the cases.We report a rare case of vaginal vault fistula following coitus, following a hysterectomy done for malignancy.A 38-year-old parous woman, presented to outpatient gynecology department with complaints of abdominal distension and bloating for the last 2 months. On clinical examination, ascites was present. On per vaginum examination, bilateral adnexal masses were present; vagina was healthy. There was absence of nodularity or deposits in the pouch of Douglas. Ultrasonographic evaluation revealed 4 × 4 cm complex adnexal cyst on the left side, with the presence of ascites. Ascitic fluid examination revealed malignant cells following which a decision was taken for laparotomy. Laparotomy was performed with panhysterectomy, bilateral pelvic lymphadenectomy, and infracolic omentectomy. Peroperatively, 800 mL straw colored ascites was present. Right ovarian 5 × 5 cm and left ovarian 4 × 4 cm masses were present with the presence of variegated consistency and necrotic areas on cut sections. Omental caking was present. Histopathologic diagnosis of papillary serous cystadenocarcinoma of ovary, stage IIIc was confirmed. There was no evidence of metastasis to vagina. She was discharged in a stable condition with advised abstinence for 6 weeks to allow vaginal vault healing. Postoperatively, 6 cycles of chemotherapy with paclitaxel and carboplatin, at 3 weekly intervals was planned.She presented in gynecologic emergency 10 weeks after her hysterectomy (after her third cycle of chemotherapy) with acute abdominal pain. There was no associated fever or bowel or bladder symptom. She had been tolerating her meals well. There was a history of coital act, the night before. This was her first coitus after surgery. Her general physical examination was unremarkable. Her pulse rate was 86/min, respiratory rate was 18/min, blood pressure was 120/80 mmHg. She was afebrile; oral temperature was 99F. On local per speculum examination, 1 cm rent negotiating a finger tip was discovered at the vaginal vault. There was no active bleeding or signs of inflammation. Upright chest radiograph revealed gas under diaphragm indicating pneumoperitoneum . UltrasoSurgical opinion was taken to rule out acute intestinal perforation. In view of absence of clinical bowel symptoms, perforation seemed unlikely, so barium meal test and barium enema were not performed. There was no history of any foreign body insertion, vigorous douching, fall, or any other trauma. Iatrogenic persistence of air in the peritoneum is unlikely to persist 10 weeks postsurgery. On clinical examination, there was no evidence of fresh tumor deposits or nodularity in the pouch of Douglas, which might have caused fistula as a progression of malignancy. In 10 weeks, the vaginal vault is expected to heal. The patient was asymptomatic till she had coitus allowing the authors to assume there was no defect in healing. In the face of other negative results, onset of pain after coitus, and presence of rent in the vault, a diagnosis of vaginal vault fistula, postcoitus was made.She was administered antibiotics, analgesics, and asked to keep abstinence. She was told to avoid any vaginal douching or undergo any intervention. Pain gradually subsided over the next 3 days. Because the size of the fistula was small, it was decided not to actively intervene. Per speculum examination after 1 week revealed healed ulcer at the vaginal vault , and a fIn most cases, such acute abdomen and pneumoperitoneum present to the surgeon, and perforation of a hollow viscus is the underlying cause. The firsVesicovaginal fistula and pneumperitoneum due to rough coital activity and vigorous intercourse have usually been reported in young nullipara status females.56 Postpa5There has been no such case report of postcoital fistula after hysterectomy done for malignant female genital disease. With presentation of our case, we further hypothesize that in hysterectomy done for malignant indication, the vaginal vault might be relatively weaker than hysterectomy done for benign diseases. This may be due to low immunity status and poor healing, making these women more prone to such sexual fistulae.Surgical intervention might not be needed in all cases. If there is any suspicion of perforated viscus, diagnostic laparoscopy and laparotomy to explore and repair the bowel should be performed. If the patient’s examination is negative for the signs of surgical abdomen, “wait and watch” policy may be adopted. Cases with resolution of symptoms may be followed-up. The cases with worsening of symptoms or with persistent or new appearance of signs of peritonitis or inflammation may be considered for surgical intervention. In peritWhile assessing a woman with acute abdomen with pneumoperitoneum in emergency, it is necessary to have a high index of suspicion and consider all etiologies, including sexual trauma. In surgical specialties, a history of woman is not complete without gynecologic history, which should include detailed menstrual and sexual history. Coital activity should be considered in the differential diagnosis of pneumoperitoneum. This can help to avoid unnecessary negative laparotomy and late diagnosis of coital fistula.10 Decisi


Background. In today's fast-paced and high-acuity emergency departments, clinicians are often compelled to triage cases so rapidly that a differential diagnosis consistent with the history and physical examination is not comprehensive. Case Report. This case report describes the unexpected finding of a cystic ovarian neoplasm in a young female with an abdominal mass and a ventriculoperitoneal shunt, initially diagnosed as a cerebrospinal fluid pseudocyst. We use this case to illustrate that the astute clinician must always synthesize a diagnosis from all data sources and not to rely on initial radiographic evaluations. Conclusions. This remarkable case demonstrates that all differential diagnoses must be entertained in order to rapidly and accurately diagnose a patient with a cystic abdominal mass. The modern Emergency Department is frequently a venue for fast-paced, high-acuity cases, and in many instances clinicians are compelled to limit their differential diagnosis due to time constraints. As an example, ventriculoperitoneal cerebrospinal fluid shunt failure is commonly the top differential diagnosis when a shunted patient presents with a wide variety of clinical problems—this is certainly a reasonable approach as long as it is not fixated on to the exclusion of all other appropriate differential diagnoses. The following case illustrates the pitfalls of such an oversimplification of differential diagnosis.A 21-year-old female with history significant for ventriculoperitoneal shunt-dependent hydrocephalus since infancy for myelomeningocele presented to our Emergency Department with an 8-day history of mild nausea and fevers up to 39°C. The patient had no history of shunt failure or revisions. She noted no neurological signs or symptoms, nor did she complain of a headache. The physical examination revealed a firm, nontender mass in the lower right abdominal quadrant. CT of head showed unremarkable ventricle size, good proximal shunt catheter placement, and no transependymal flow, although no previous imaging was available for comparison. An abdominal ultrasound was interpreted as a multiseptated fluid collection within the right lower quadrant extending into the lower abdomen, distinct from bowel, ovary, and uterus. A small portion of the shunt catheter was reported to be within this fluid collection; however; the tip could not be identified . As the Because the tubing could not be localized within the mass, and the patient's history was not entirely consistent with a CSF pseudocyst  , 2, the  Ventriculoperitoneal shunt placement has proven to be an effective treatment of certain types of hydrocephalus; however, complications can occur. Unlike more common complications associated with ventriculoperitoneal shunts such as ventricular catheter obstruction, tubing disconnection, valve malfunction, and infection, peritoneal pseudocysts are a relatively rare complication. Originally described by Parry in 1975, ventriculoperitoneal CSF pseudocysts occur with an incidence ranging from 1% to 5% and are often associated with previous infections or revisions . In the acute and fast-paced setting of the emergency ward, there is sometimes a tendency to suspect the CSF shunt system when a hydrocephalic patient presents with a wide variety of clinical problems. This is certainly a reasonable approach as long as it is not fixated on to the exclusion of all other appropriate differential diagnoses. In the setting of an abdominal mass, and with an initial ultrasound interpretation of a cyst in close proximity to the peritoneal tubing, the presumptive diagnosis of a CSF pseudocyst was quickly established. However, the history and physical examination of this patient were not consistent with this diagnosis. Accurate CT visualization of the patient's shunt system placed the abdominal tubing outside the cyst, and in conjunction with accurate anatomical correlation of the cyst and ovary, leads to the correct diagnosis and ultimately appropriate treatment.This case illustrates the unexpected finding of a cystic ovarian neoplasm in a young female with a cystic abdominal mass in the setting of a ventriculoperitoneal shunt. Although accurate radiographic imaging and interpretations serve an invaluable role within medicine and surgery, clinicians must always rely on the foundation of an accurate and detailed history and physical examination to help guide them through all potential differential diagnoses in order to accurately diagnose and treat the patient.


Calcific myonecrosis is a rare posttraumatic complication characterized by the replacement of muscles of one or more compartments with central liquefaction and peripheral calcification. We report magnetic resonance imaging (MRI) and CT imaging features of calcific myonecrosis arising 43 years after trauma. A 62-year-old man presented with a slowly enlarging mass in the left lower leg. Plain radiographs revealed a soft tissue mass with extensive amorphous calcification. The middle third of the tibia and fibula were eroded. MRI demonstrated peripheral ring enhancement on postcontrast fat-suppressed T1-weighted images. Volume-rendered images extracting only the images of bone and vessels also showed many branches from the tibialis posterior and peroneal arteries around the bone defect. During the operation, bleeding continued heavily from the vessels penetrating the cortical bone of the tibia, from the posterior compartment, and from the branches of tibialis posterior artery. The total blood loss was approximately 2,400 milliliters. There may be a causal relationship between massive bleeding and the hypervascularity of this tumor as evidenced by MRI and volume-rendering CT studies. Calcific myonecrosis is a condition characterized by the latent formation of a dystrophic calcified mass occurring exclusively in the lower extremity . This raA 62-year-old man presented a 5-month slowly enlarging mass in the anterior part of the left lower leg. Forty-three years earlier, he had his left lower leg squeezed between two rollers during his work, and the popliteal vessels were injured. At the initial examination, there was no clinical evidence of a compartment syndrome. Since this accident, he noticed that the left foot showed some weakness at dorsiflexion. Physical examination showed a bulging, non-tender and elastic hard mass covered with thin and atrophic skin on the mid-portion of the anterior aspect of the left calf . The masSix months after the biopsy, for the purpose of relieving pain, we performed marginal excision using a tourniquet. Surgical exploration through an anterolateral incision revealed a cystic cavity filled with brown-colored fluid with necrotic muscle debris. The cyst was surrounded partially by thin bone shells. The middle portion of the tibia was eroded, and there was a small area of cortical erosion involving the medial aspect of the fibula. Although the cystic cavity was attached to the bone erosion, it could easily be peeled from the bone. The cyst, the necrotic muscles, and calcification were removed. The surgical site of the anterior aspect of the left lower leg demonstrated a large deficit by resection of calcified tissue. Blood continued heavily from the vessels penetrating the cortical bone of the tibia, from the posterior compartment, and from the branches of the tibialis posterior artery. The total blood loss was approximately 2,400 milliliters. The wound was closed after placing a suction drain tube in the deep portion of the wound, and a bulky compressive dressing was applied to the lower leg. Postoperatively, the wound healed without infection. At 7-month follow-up, the patient was able to walk without pain. The muscle strength and sensory of his lower leg were normal.Calcific myonecrosis was first reported by Gallie and Thompson in 1960 . This enThe differential diagnosis of calcific necrosis includes malignant tumors with the propensity to mineralize, such as synovial sarcoma and soft tissue osteosarcoma ,10,16,25Calcific myonecrosis has distinct radiographic features. The first one is the smooth pressure erosion affecting the outer cortex of the adjacent bone. It represents a chronic nature of this disease. The second is calcification of soft tissue, which is thin with a plaque-like or linear configuration organized around the margin of the lesion . ContrasTo the best of our knowledge, this report is the first to describe the anatomical relationship of the vessels with calcific myonecrosis.. In general, after contusion injury, skeletal muscle is remodeled often with fibrosis, not with necrosis (The exact pathophysiology of calcific myonecrosis has not been elucidated, but neurovascular compromise has been suggested to be the primary cause. It mostly occurs after tibial or fibular fracture resulting in a compartment syndrome. O'Keefe et al. hypothesized that an initial compartment syndrome decreases the circulation within a limited space resulting in necrosis and fibrosis . They spnecrosis –31. We hTreatment for calcific myonecrosis is controversial. With regard to the reported cases, excision has commonly been performed ,16,17,23The diagnosis of calcific myonecrosis must be considered in cases with a history of remote trauma and the presence of calcific mass. Excision is thought to be the treatment of choice if the patient has pain and especially if imaging studies show no evidence of hypervascularity.


Acinar cell pancreatic carcinoma is a rare solid malignant neoplasm. Recent review of the literature showed occasional cases with papillary or papillocystic growth patterns, ranging from 2 to 5 cm in diameter. We report a large 10 cm pancreatic tumor with papillocystic pathology features involving the pancreatic head. The growth pattern of these tumors could be mistaken for intraductal papillary mucinous tumors or other pancreatic cystic neoplasms. Acinar cell carcinoma of the pancreas is a rare tumor and account for less than 2% of all pancreatic carcinoma. These tumors are commonly large well-circumscribed solid and highly cellular lesions. Recent literature review showed occasional rare cases with papillary or papillocystic growth pattern that can be mistaken for mass forming cystic neoplasms of the pancreas . Cystic μg/L, reference rage: ≤5 μg/L). A combination of imaging studies, including transabdominal ultrasound and computed tomography (CT) of the abdomen and pelvis (A 48-year-old Caucasian male presented in September 2007 with a 10-year history of intermittent epigastric pain. He reported a recent increase in frequency of the pain, but not in severity. There were no other gastrointestinal symptoms such as weight loss, melena, hematochezia, nausea, vomiting, dysphagia, change in bowel habits, or jaundice. Serum amylase, lipase, and tumor markers were normal (CA 19-9: 7 KU/L, reference range: <35 KU/L; CEA: 1.0 d pelvis , showed d pelvis . The tumd pelvis . By immud pelvis  and focaAcinar cell carcinomas of the pancreas are rare tumors and account for less than 2% of all pancreatic carcinomas. The disease most often presents in the seventh decade of life, but can occur at any age. There is a 2 : 1 male predominance. The presenting symptoms are generally nonspecific including abdominal pain and weight loss. In contrast to ductal adenocarcinoma, jaundice is rare . The lonWe have presented a rare case of papillocystic variant of acinar cell carcinoma. Acinar cell carcinoma rarely exhibit papillary or papillocystic pattern, and, therefore, fall into the challenging differential diagnosis of papillary and cystic pancreatic tumors. The correlation of clinical, radiographic, histological, and immunohistochemical findings would be helpful to distinguish such a tumor from other pancreatic papillary lesions as they carry different prognostic outcome.


Chordomas are rare intracranial tumors. There are several reported cases of these tumors arising in patients with tuberous sclerosis (TSC), a neurocutaneous disorder inherited in autosomal dominant fashion that predisposes patients to hamartomatous and neoplastic lesions.A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope. Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma. The patient underwent a left far lateral approach for removal of the tumor. Upon opening of the dura, the tumor could be seen under the arachnoid. The tumor was carefully debulked within the limits of safety. The patient did well postoperatively and was referred to the radiation oncology department at our institution for follow-up radiotherapy of the tumor bed.This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC. Chordomas are identified as rare tumors. They represent <1% of all intracranial neoplastic processes. They are29142226A 38-year-old man presented to the emergency room at our institution with the complaints of dizziness and a near-syncopal episode. This episode was preceded by a 1-week history of nausea, vomiting, and dysarthria. He also reported a 2-month history of new headache. The patient denied any visual disturbances, hearing changes, recent seizures, loss of consciousness, weakness, or sensory changes. Medical history was significant for a diagnosis of TSC with mild mental retardation and seizures in the distant past as well as hypertension. The patient had had a cataract removed remotely. Physical examination revealed adenoma sebaceum on the patient's malar surfaces. Complete neurological examination revealed no focal deficits. The results of laboratory tests were within normal ranges. A computed tomography (CT) of the head from an outside hospital demonstrated a brain mass with high attenuation. The mass appeared adjacent to the left cerebellar hemisphere near the Foramen of Lushka and extending into the foramen magnum. The patient had mild hydrocephalus evidenced by the prominence of his ventricular system. Periventricular calcifications were consistent with his diagnosis of TSC . The patThe patient was taken to the operating room for a left far lateral approach for removal of the tumor. Upon opening of the dura and release of cerebrospinal fluid, the tumor could be seen under the arachnoid and appeared to originate intradurally. Several cranial nerves were adherent to the mass, eliminating the possibility of a total resection. During the operation, no connection between the intradural mass and the clivus could be appreciated. The tumor was carefully debulked within the limits of safety. A significant amount of cerebellar swelling was encountered during the case and ultimately led to early termination of the operation before subtotal resection could be completed. The patient was then transferred to the ICU. He remained intubated but was following commands with all four extremities. Postoperative MRI showed significant reduction of tumor burden from surgical debulking . Grossly, the tumor was gray-tan in appearance. Microscopic examination of the tumor revealed plump, vacuolated cells within a mucinous matrix . Tumor cPostoperatively, the patient developed dysphagia and required placement of a percutaneous endoscopic gastrostomy (PEG) tube as well as a tracheostomy. The patient also developed a pneumonia, which was treated with moxifloxacin on the recommendation of the infectious disease team. The patient's pneumonia resolved and his dysphagia improved, and he was discharged with plans to follow up with radiation oncology for radiotherapy of his residual tumor.Chordomas are found to be rare tumors that are believed to arise from remnants of the notochord. About 45-50% of chordomas are reported to arise in the sacrococcygeal region; 35-40% in the sphenooccipital region; the remaining 15% of these tumors arise along the rest of the spinal column.111222252830 222528Radiographically, chordomas are well-defined extra-axial masses that enhance with contrast on CT.30 The tuPathologically, chordomas appear grossly gray to tan in color with gelatinous texture and are often lobulated and encapsulated.30 They cTuberous sclerosis is a neurocutaneous disorder inherited in an autosomal dominant fashion. It's prevalence is estimated to be 1:6800. ClinicalTuberous sclerosis is believed to involve two separate genes; TSC1, which is found on the long arm of chromosome nine (9q32–q34) and which encodes the protein product hamartin, and TSC2 found on the long arm of chromosome 16 (16q13.3) that encodes for the protein tuberin. Both of these proteins are believed to play a role in tumor and growth suppression. Because of the dysfunction of these gene products, TSC patients are at risk of developing a number of tumors. The classic central nervous system tumor associated with TSC is the subependymal giant cell astrocytoma (SEGA), which arises in 10% of patients with TSC. These tumors usually arise within the ventricles. Althoughet al., who presented a newborn male with sacrococcygeal chordoma, thick sclerotic ribs, and thoracic and abdominal aortic aneurysms.[et al. reported on a chordoma involving the clivus in a 15-week-old male baby with family history of TSC and imaging features consistent with this diagnosis.[et al. reported on a chordoma arising in the cervical region in a 16-year-old patient with a history of TSC.[et al. presented another clivus chordoma in a 4-year-old female baby with TSC,[et al. reported on a clivus chordoma in 4-year-old boy with family history of TSC.[et al reported on sacral chordomas in two patients, one a 33-week-old female fetus electively aborted due to cardiac abnormalities diagnosed by prenatal ultrasound and the other a newborn female.[Although SEGA is the tumor classically associated with TSC, there are a number of case reports of chordomas arising in patients with this genetic disorder29–14222628142226iagnosis. The prevn female.et al reported on a patient with a chordoma arising in Meckel's cave.[et al. presented a chordoma in a 9-year-old male child arising in the region of the jugular foramen.[It is worth noting that in many of these cases, TSC patients presented with chordomas at a very early age, much younger than is typical for this tumor in the general population. Furthermore, while several of the patients had chordomas arise in locations typical of this tumor, others have had tumors present in more eccentric locations, such as with cutaneous metastasis, or, in the case of our patient, in a more posterolateral position within the skull base. Certainly, chordomas can arise in atypical locations. Lu l's cave. Kaufman  foramen. et al., and shared by our case, is that the tumor was found and appeared to originate intradurally. Although the MRI of our patient demonstrated the enhancement of the clivus, the primary tumor that was symptomatic was found lateral to the medulla. Typically, chordomas arising from the clivus are midline and extradural in location. However, Kaufman et al. point out that there are cases of intradural chordomas, and proffer an explanation as to why, embryologically, this could be possible. They suggest that these chordomas represent neoplastic transformation of an ecchordosis physaliphora, rests of notochord cells found intradurally and reported in about 2% of all autopsies.[16An unusual characteristic of the case reported by Kaufman utopsies.16 They autopsies. Nevertheopsies.1631 as welopsies.1629et al. analyzed pathological specimens of four chordomas arising in one pedigree that suggested an autosomal dominant pattern of inheritance.[et al. performed cytogenetic analysis on a chordoma in a patient who had a family history of chordoma.[1381013182124et al. in their study of 7 primary tumors and 11 recurrences, highlighted four genetic loci  affected in primary chordomas as well as recurrences.[et al.'s study also identified cytogenetic changes in chromosome 1p in their analysis of two recurrences of a clival chordoma.[324et al. identified a cytogenetic abnormality in chromosome 21q22 in their analysis of two primary sacral chordomas. Nearly every chromosome has been highlighted as having additions, deletions, hypo- or hyper-diploidy in the above referenced studies.There is evidence to suggest a genetic influence in the development of chordomas. Stepanek eritance. Dalpra echordoma. There haa.138101318212425 hordoma.32425 Gibaet al.[While all of these studies have highlighted various cytogenetic abnormalities in the tumors they have analyzed, to date no consistent cytogenetic abnormality has been identified in any of the chordomas. Furthermore, there is no indication that any of the chordomas analyzed in these studies reported from patients with TSC. The only study to specifically look at the cytogenetics of chordomas arising from patients with TSC was Lee-Jones et al. They subet al. These fiChordomas are rare intracranial neoplasms. They are typically located midline, extradurally, in the sphenooccipital region. Rarely, these tumors may be found intradurally or laterally in the skull base. This study represents an unusual presentation of a chordoma arising in the lateral medullary cistern. Furthermore, this case joins several case reports suggesting a link between chordomas and TSC, a neurocutaneous disorder predisposing patients to neoplastic processes. Further studies need to be done to elucidate the relationship between tuberous sclerosis and chordomas.


To describe a rare presentation of vertebral artery dissection (VAD) as a small but congruous incomplete homonymous hemianopia demonstrating use of visual field testing in the diagnosis.A 30 year old woman had been unwell for 4 months with difficulty focusing, vertigo, dizziness and a feeling of falling to the right. A small but congruous right inferior homonymous quadrantanopia was found on examination leading to further investigation that uncovered a vertebral artery dissection and multiple posterior circulation infarctions including a left occipital stroke matching the field defect.We describe an atypical case of VAD presenting with a small congruous quadrantanopia. This is a rare but significant condition that predisposes to multiple thromboembolic infarction that may be easily misdiagnosed and a high index of suspicion is required to make the diagnosis. Vertebral arterial dissection (VAD) may occur after neck trauma ,2, and aA 30 year old woman became unwell after swimming with crocodiles while suspended under water in a protective cage in the Australian Northern Territory. She was vertiginous after coming out of the water and required admission to a tertiary hospital emergency department and was discharged with a diagnosis of vestibular neuronitis.She continued to experience symptoms of vertigo, difficulty focusing, dizziness and a feeling of falling to the right for over 4 months and saw various healthcare providers with no cause found. She had a plain computerized tomography of the head without contrast that was reported as normal. Eventually she was referred to a neuro-ophthalmologist for the complaint of "difficulty focusing" and automated perimetry showed a small incomplete congruous homonymous quadrantanopia Figure . MagnetiVertebral artery dissection (VAD) is a potentially fatal condition where disruption of the vessel wall results in thromboembolism and subsequent ischemic stroke. It may occur following neck injury resulting in neck extension, flexion or rotation -3. SymptThis case demonstrates the importance of ancillary imaging in making the diagnosis. CT angiography provides accurate imaging of the vessel lumen and dissection length. It can identify VAD in both large and medium vessel dissections in the neck . T1-weigManagement of VAD to prevent further embolic stroke is done on a case by case basis. There is no data to support the therapeutic superiority of anticoagulants over antiplatelet agents. In thisIn conclusion, we describe an atypical case of VAD presenting with a small congruous quadrantanopia. This is a rare but significant condition that may be easily misdiagnosed and a high index of suspicion is required to make the diagnosis.The authors declare that they have no competing interests.AM carried out preparation and drafted the manuscript. CSC and AWL were the treating doctors involved in the investigation and management of the case. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/10/14/prepub


Cholecystocolic fistula is a rare biliary-enteric fistula with a variable clinical presentation. Despite modern diagnostic tools, a high degree of suspicion is required to diagnose it preoperatively. Biliary-enteric fistulae have been found in 0.9% of patients undergoing biliary tract surgery. The most common site of communication of the fistula is a cholecystoduodenal (70%), followed by cholecystocolic (10–20%), and the least common is the cholecystogastric fistula accounting for the remainder of cases. These fistulae are treated by open as well as laparoscopic surgery, with no difference in intraoperative and postoperative complications.We report here a case of obstructive jaundice, which was investigated with a plain film of the abdomen, abdominal ultrasonography, and endoscopic retrograde cholangiopancreatography, but none of these gave us any clue to the presence of the fistula was discovered incidentally during an open surgery and was appropriately treated. Spontaneous biliary-enteric fistulae have been rarely reported. In fact, most such fistulae result as complications of gall stone disease. A sequence of events occurs in acute calculous cholecystitis. During these attacks, the adjacent serosal surface becomes inflamed and adherent to the gall bladder. The ischemic area in the wall of the gall bladder becomes gangrenous, and because of the increased pressure within, its contents penetrate its own necrotic wall first, and then, the wall of the adjacent colon, forming a cholecystocolic fistula.Another cause is from pressure necrosis of an impacted gall stone usually at the neck of the gall bladder, which gradually erodes into the colon.A 59-year-old woman presented to the emergency department of our hospital with a history of fever, increasing jaundice, and right upper quadrant abdominal pain for two weeks. On examination, she was found to be febrile, deeply icteric, and tender in the right hypochondrium. Blood examination revealed a serum bilirubin level of 11.7 mg%  and a serum alkaline phosphatase level of 320 IU . An abdominal ultrasonography revealed an enlarged liver, dilated intrahepatic biliary radicals, and a dilated common bile duct with a 12× 10-mm calculus in the infraduodenal common bile duct (CBD) . The galAs the patient showed features of cholangitis, she was given parenteral cefazolin and vitamin K. Forty-eight hours later, after the pyrexia had settled, an endoscopic retrograde cholangiopancreatography (ERCP) was done; the cholangiogram revealed a dilated CBD with a filling defect in the lower end of the CBD, which was suggestive of a calculus. The cholangiogram did not show any fistulous communication between the gall bladder and the colon. A sphincterotomy was done along with an extraction of the stone and clearance of the CBD with a temporary stenting of the common bile duct.Two weeks later, the patient was posted for laparoscopic cholecystectomy. During laparoscopy, it was evident that there were dense adhesions between the gall bladder, transverse colon, and the omentum. The Calot's triangle was virtually inaccessible. It was therefore decided to convert to an open cholecystectomy, and a right upper paramedian incision was used. Dissection in the Calot's triangle was extremely difficult although the stent in the CBD was palpable. Hence, it was decided to proceed by the “fundus first” method. Dense adhesions between the gall bladder and omentum were separated, and an attempt was made to create a plane between the neck of the gall bladder and the right colon at the site of the stone impaction which could be clearly felt. As this was impossible, the gall bladder neck was incised over the stone on the gall bladder to prevent damaging the right transverse colon, thereby keeping a cuff of the wall of the gall bladder on the colon. The gall stone measuring 15 × 15 mm was removed, and it became evident that there was a fistula between the gall bladder and right transverse colon .A cholecystectomy was performed with excision of the fistula and primary repair of the colon enterotomy. This was done in two layers after excising the cuff of the gall bladder that was initially kept on the wall of the colon to prevent creating a fistula. The patient made an uneventful recovery and was discharged on the 15th postoperative day. A histopathological examination of the specimen revealed no evidence of malignancy. Twelve weeks later, the CBD stent was removed after confirming a normal abdominal ultrasonography and ensuring that the liver function tests were within normal limits.Spontaneous cholecystocolic fistulae comprise 10–20% of all biliary-enteric fistulas. In the majority of cases, they are a sequel to cholecystitis but are reported to complicate only 0.13% cases.[Cholecystocolic fistulae can present with abdominal pain, nausea, weight loss, diarrhea, and dyspeptic symptoms. This fistula can alter the normal enterohepatic circulation of bile acids, leading to their malabsorption. The bile acids also stimulate the colonic mucosa directly to secrete water and electrolytes excessively, leading to steatorrhea and diarrhea. Rare casIf found incidentally during laparotomy, these fistulae should be taken down because of the increased risk of cholecystitis, cholangitis, and a 15% chance of malignancy in the gall bladder.The most useful techniques for diagnosis are a plain film of the abdomen, abdominal ultrasonography, barium studies, biliary scintigraphy, and ERCP. Although a diagnosis of cholecystocolic fistula is rarely suspected clinically, it should be considered in elderly patients with unexplained pneumobilia or unexplained persistent diarrhea. ERCP can be helpful in establishing the diagnosis, especially if barium studies give negative results.The standard treatment of a cholecystocolic fistula is open cholecystectomy and closure of the fistula. However, recent developments in laparoscopic surgery have shown its potential use in treating these rare fistulas. Similar techniques of open surgery have been used in laparoscopic surgery. The results have shown no significant differences in intraoperative and postoperative complications. Although


The adrenal cyst is a rare disease that represents approximately 5% of discovered adrenal lesions, which are usually discovered incidentally. True adrenal cysts originate to cells from mesothelium. The potential of cyst adrenal to become malignant has been reported to be 7% and a radical excision of a potentially malignant mass are indicate. We report a case of a 48 year old woman that presented with pain in left hypochondrium and epigastrium, nausea, vomiting, weight loss and microscopic hematuria. After the diagnosis suspicion surgery was performed with a monoblock resection of left kidney and left adrenal gland because of kidney cancer diagnosis as considered. The microscopically analysis of surgical specimen, diagnosed a true epithelial cyst of adrenal gland. The adrenal cyst is a rare disease. Its incidence in autopsy series varies between 0.064% and 0.18% . Most caAdrenal epithelial cysts originate to cells from mesothelium that was included in adrenal gland during embryogenesis . When thWe report a case of true adrenal cyst, emphasizing its symptoms, differential diagnosis and treatment.A 48-year-old Caucasian woman, related pain in left hypochondrium and epigastrium 1 year ago that exacerbated 3 months after the diagnosis, fisgada type that radiates to the back and periumbilical region, associated with symptoms of nausea, vomiting and weight loss (4 kg in 2 months). Myalgia and fatigue were also reported. Personal and Family history had not relevant information’s. On physical examination, abdominal mass was palpable in the left flank.A urine analysis revealed microscopic hematuria. Abdominal ultrasonography demonstrated a large cystic mass in abdominal upper left quadrant. Computed tomography scans of the abdomen with and without contrast confirmed presence a large cystic lesion, homogenous, well-defined contours, measuring 10.9 × 9.5 cm in largest diameter, in the upper pole of the left kidney and left adrenal without plan between the kidney and adrenal gland.Based on clinical tests and supplementary examination was considered the diagnosis of kidney cancer and surgery was performed with a monoblock resection of left kidney and left adrenal gland.A microscopically analysis of surgical specimen, diagnosed a true epithelial cyst of adrenal gland. Immunohistochemistry demonstrated that the cells lining the cyst were positive for Citoceratina 8, Calretinin, Alpha-inhibin and Sinaptofisina and negative reaction to S-100 protein. A positive reaction for citoceratina 8, alpha-inhibited, melan-A, and calretinin sinaptofisina, confirming presence of adrenal cortex cells -3.The adrenal glands are very small organs located in the deep retroperitoneal space and are common site of cancer of parasitic disease and inflammatory disease originated in other organs .With the development of advanced image techniques in recent years and growing indications this examination for other purposes, there were increased incidence of adrenal masses detected incidentally. Moreover, it is still not clear if radiological images can distinguish malignant or benign adrenal cysts .The cysts adrenal represent approximately 5% of incidentally discovered adrenal lesions . OccasioRozenblit and colleagues suggested that it is reasonable to evaluate the malignant potential of adrenal cystic lesions by using the Bosniak classification of renal cystic masses. Khoda and associates also used the Bosniak criteria to stratify their patients, and they safely and conservatively managed their patients with uncomplicated adrenal cysts. Even with the Bosniak classification criteria, however, the sensitivity and specificity of definitive differentiating benign and malignant complex cystic adrenal tumors have not been well reported. MRI is thought to be a good diagnostic modality to reveal a variety of tissue characteristics and thus to differentiate the benign or malignant adrenal tumors .A case reported this article, the patient had flank pain, hematuria, and a palpable abdominal mass, triad that are the classic presentation of renal-cell carcinoma ,7. CompuFan et al. observed that fibrous proliferation can eliminate the plane between the kidney and adrenal gland and make radiologic distinction impossible. Indeed, the fibrous capsule surrounding the adrenal gland is known to fuse on occasion with the capsules of the kidneys or liver ,10.True adrenal cysts are unusual lesions and usually asymptomatic . Foster (1966) related, in histopathology, four types of cysts adrenal: endothelial cysts (45%), pseudocysts (39%), epithelial cysts (9%) and parasitic cysts (7%).Immunohistochemistry analysis of surgical specimen is useful to identify cellular products and markers of cell surface, in case reported, the positive Citoqueratina 8 shows epithelial origin of cyst. The positive immunostains for calretinin and WT-1 lend support to the postulate of Medeiros et al nearly 20 years ago of a mesothelial origin for these cysts .Because of the low incidence of this disease, there were only limited reports of adrenal cyst management in the literature. There is wide acceptance that small, asymptomatic or nonfunctioning adrenal cysts should be managed conservatively. Percutaneous aspiration can be done under guidance of US or CT .en bloc removal of the whole lesion without breaking its integrity before leaving the body - cannot be overemphasized. Potentially malignant or complex adrenal cysts are usually fairly large. Although pure laparoscopic resection of the tumor without violating the cystic wall is not technically impossible, it poses a real challenge to determine how to remove the large tumor intact from the body without making a large skin incision. Laparoscopic adrenalectomy brings many benefits, including smaller incisions, shorter hospital stays, and faster recovery, but open adrenalectomy for adrenal cystic tumors for which there was suspicion of malignancy still accounted for most cases in a review article of adrenal tumors [Reported indications for operative intervention of adrenal cysts include size (cysts with 6 cm in diameter or more), symptomatic cysts, cysts that secrete excessive amount of hormones, or cysts that are suspicious for malignancy (as determined from imaging studies). For radical excision of a malignant or potentially malignant mass, the principle of surgical oncology - namely, l tumors .The treatment is surgical resection of cystic mass, indicated in presence of symptoms, when there are hyperdense components or irregular masses in imaging examinations. It must be accompanied by adrenalectomy when have doubt about the biological behavior of cyst.Most adrenal cysts are benign and silent clinically, but larger ones up to 30 cm in diameter may require surgical attention .


A 61-year-old woman presented with low grade fever and an epigastric mass eight yearsfollowing resection of a stage Clark IV infraclavicular cutaneous melanoma followed by axillarynode dissection. Investigations revealed a tumor in segment II, III, IV and V of the liver anda thrombus involving the main portal vein. Liver resection with extended left hepatectomy (lefttrisegmentectomy) and portal vein thrombectomy is reported.


Hemangiomas are the most common benign neoplasms affecting the liver. They occur at all ages. Most cases are asymptomatic and do not require any treatment. Rarely, hemangiomas can be pedunculated. İf they undergo torsion and infarction, they become symptomatic. Herein; we report the case of a 31 year old male presenting with features of acute appendicitis: continuous right iliac fossa pain, rebound, guarding tenderness at McBurney' s point, nausea, anorexia, shifted white blood cell count and a Mantrels score of 6. At laparotomy a normal appendix was observed and a torsioned pedinculated liver hemangioma turned out to be the cause. Hemangiomas are the most common benign neoplasms affecting the liver with an incidence of 0.4-20% in autopsy series . Women aHerein; we report the case of a torsioned giant pedunculated liver haemangioma that mimicked acute appendicitis.A 31 year old man admitted to our emergency department with a 2 day history of right iliac fossa pain which he described as continuous. He also had anorexia, nausea. On physical examination, his pulse rate was 96 beats/min, his body temperature was 37.1°C. His abdomen was markedly tender at the right iliac fossa with guarding and rebound tenderness at McBurney's point. The rest of the systemic examination was normal and the Mantrels score of the patient was 6. Laboratory data was as follows; hemoglobin 15.8 g/dl, total leukocyte count 9700/mm3, with 75% polymorphonuclear leukocytes, 37% lymphocytes, 3,2% monocytes, and 1% eosinophils; erythrocyte sedimentation rate was 2 mm for 1 h. Liver function tests, serum electrolytes, and creatinine were all within normal ranges. His bowel movements were regular on oscultation. Per rectum examination was normal. The abdominal X-ray was normal and because of the manifest clinical picture, abdominal ultrasound was not performed. Under the light of medical history and signs on abdominal examination, the patient was diagnosed as having acute appendicitis with a Mantrels score of 6 and was taken to theatre for appendectomy. At operation a normal appendix was found. At further exploration, a large soft reddish mass was palpated near the caecum. Macroscopically, the mass measured 10 × 12 × 15 cm. It was connected to the right inferior margin of the liver with a thin pedincule. It had undergone a 360° clockwise torsion on its pedincule. The mass was easily detorsioned and resected (Fig Cavernous hemangioma is the most common benign tumor of the liver. They are probably of congenital origin and have no potential for malignant transformation. Most are diagnosed incidentally and are asymptomatic. Hemangiomas are usually found at the right lobe of the liver in a subcapsular or marginal location. Most hemangiomas are diagnosed incidentally and are small and asymptomatic. Their size usually remains stable and can vary from a few milimetres to more than 20 cm. Lesions larger than 4 cm have been defined as giant hemangiomas .Giant hemangiomas may cause abdominal discomfort, swelling, abdominal pain, icterus and thrombocytopenia . Very raRarely, hemangiomas can be pedunculated . At ultrTo the best of our knowledge, this present case is the first example of a torsioned pedunculated hemangioma in the literature, mimicking acute appendicitis with a Mantrels score of 6 [In conclusion, this case report impresses that; even incidentally detected pedunculated hemangiomas must be managed by surgery for their tendency to get torsioned. In addition; the surgeon must look for different ethiologies when a normal appendix is found during operation.The authors declare that they have no competing interests.All the authors participated in the admission and the care of this patient, the conception, the design, data collection and interpretation, manuscript preparation and literature search.All authors have read and approved the final manuscriptWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We report a case of an angioleiomyoma occurring in a 40-year-old Kenyan female. The patient presented with recurrent pain and a soft tissue swelling at the posteromedial aspect of her right knee. Angioleiomyomas are benign soft tissue tumours, which occur most commonly in the skin of the lower extremities in middle-aged females. They very rarely occur in the knee and are treated curatively with resection. A brief literature review is included. Angioleiomyomas, or vascular leiomyomas, are infrequent benign tumours originating from smooth muscle cells of arterial or venous walls. They were first described in 1937 by Stout AP . Since tA healthy 40-year-old Kenyan lady was referred to our hospital with an 8-year history of recurrent pain at the posteromedial aspect of her right knee. The onset of pain was sporadic and it had become progressively worse over the last few months thus reducing her mobility. Cold exposure and light touch often exacerbated the pain and conservative therapy with analgesia and local bandaging was of no avail. She underwent steroid injections in Germany at the onset of her symptoms 8 years ago, however this provided little relief.On physical examination the tumour presented as a subcutaneous swelling at the posteromedial aspect of her right knee. This was tender to palpation and owing to its location was initially thought to be a medial cutaneous neuroma. An MRI scan was also suggestive of this diagnosis .Definitive treatment involved excision of the tumour via a medial incision to the right knee. The lesion was easily visualized and was attached to a very small cutaneous nerve found running in a transverse fashion across it. This small cutaneous nerve was sacrificed under traction in order to free the lesion which was then completely excised.Macroscopically, the lesion was a firm white tumour measuring 10 mm × 8 mm × 6 mm. The histological appearance was consistent with that of an angioleiomyoma  and 2B. Angioleiomyomas are benign soft tissue tumours composed of smooth muscle cells derived from vascular walls. They are more common in the lower extremities than in the upper extremities ,9. In adPain, tenderness and temperature sensitivity are common findings . It is uth, 5th and 6th decades of life and more than 80% of angioleiomyomas are less than 2 cm in diameter [Most angioleiomyomas have a duration of between 4 to 7 years depending on tumour type . Our patdiameter ,9. TheseThree case reports in the literature -6 brieflMRI is useful in the workup as it delineates the extent of the tumour making excision easier . OkahashAngioleiomyomas should be considered in the differential diagnosis of a soft tissue mass as this will expedite treatment. Excision to date is curative in all patients in the literature and recurrence is very rare however if a tumour does recur one must consider leiomyosarcoma as a diagnosis ,11.


Cystic lesions of the gallbladder are very rare and they are generally lined by single columnar or mucinous epithelium. A ciliated cyst of foregut origin is extremely rare in gallbladder. To our knowledge, only five cases have been reported so far. Here, we present the sixth case found incidentally in ultrasonographic examination in a 41-year-old woman suffering from chronic right upper quadrant pain. Laparoscopic cholecystectomy was performed for the gallstones and a benign appearing cyst in ultrasonography. Macroscopically, a submucosal unilocular cyst was located in the neck of the gallbladder. There was no communication with the lumen. Histologically, the cyst was lined by pseudostratified ciliated epithelium containing goblet cells and had a muscular wall. The postoperative course was uneventful. Patient was discharged on the second day of the operation and was well after 2 months. Ciliated foregut cyst is an uncommon developmental anomaly that usually develops above the diaphragm in the form of bronchial and esophageal cysts. Foregut cysts below the diaphragm are very rare and generally found in the liver . ExtraheCholecystectomy material was fixed in 10% formaldehyde. Representative slices were obtained and embedded in paraffin. The paraffin blocks were cut into 5 m thick sections and stained with hematoxylin and eosin. Periodic acid-Schiff stain was used for histochemistry. Smooth muscle actin (SMA), estrogen receptor (ER), and progesterone receptor (PR) antibodies were used for immunohistochemistry.A 41-year-old woman was admitted to our hospital with right upper quadrant pain. Laboratory data of the liver function tests, biliary enzymes, and serum tumor markers were normal. Abdominal ultrasonography revealed gallstones and a benign appearing cystic lesion located in the neck of the gallbladder. Laparoscopic cholecystectomy was performed. Macroscopic examination of the specimen revealed a submucosal unilocular cyst, located in the neck of the gallbladder, measuring 3.5 × 2 × 1.5 cm, without communication to the lumen . The cysIn the microscopic examination, the cyst wall was made up of muscular tissue and it was lined by pseudostratified ciliated epithelium . The epiThe postoperative course was uneventful. The patient was discharged on the second date of the operation and was well after 2 months.Cystic lesions of the gallbladder are rare. They include acquired, neoplastic, or congenital cysts. Ciliated foregut cysts are congenital cysts arising from the remnants of the embryonic foregut and are extremely rare in the gallbladder. To our knowledge, only five cases were reported so far –6. KakitAlthough no serous cysts or endosalpingios had been reported so far in the gallbladder, these are considered in the differential diagnosis for their epithelial resemblance to our case, as most of the reported ciliated foregut cysts are from female patients. But unlike these lesions, our case has its own smooth muscle wall and the epithelium lack ER or PR positivity immunohistochemically.Among the subdiaphragmatic organs, ciliated foregut cysts are mostly located in the liver (1). They are generally asymptomatic benign lesions and they generally do not communicate with the gallbladder although there is only one case communicating with the gallbladder . SquamouThe presented case is a rare case of ciliated foregut cyst located in the gallbladder. Such a cyst must be considered in the differential diagnosis of a gallbladder cyst. Although malignant transformation was not reported so far, squamous cell carcinoma must be considered during the microscopic examination as it was reported in a ciliated hepatic foregut cyst .


Rapture of a renal angiomyolipoma and massive retroperitoneal hemorrhage, during pregnancy is rare and occasionally fatal. The association of this complication with pregnancy has been reported sporadically in the literature.We report a case of a 28 years old woman, in 33 week of her first pregnancy who came to our hospital complaining of abdominal pain in the right hemiabdomen, associated with nausea and vomiting. The ultrasound and MRI (Magnetic resonance imaging) scan showed a 7 × 7 × 5 cm mass suggestive of angiomyolipoma in the right kidney, with evidence of retroperitoneal bleeding right perirenal and intrarenal haematoma. Given the size of the tumor, presence of symptoms and hemodynamic instability of the patient right nephrectomy was performed, following emergency caesarean delivery. The histological study of the resected mass revealed the presence of angiomyolipoma.In conclusion, it seems that these tumours show a greater growth index in pregnant women. Angiomyolipoma with spontaneous bleeding during pregnancy is a dangerous condition that may cause mortality in the mother and fetus, but elective, simultaneous cesarean section and radical nephrectomy can be performed. We have also done a review of the literature focusing on its management and its relationship with pregnancy. Renal angiomyolipoma (AML) is a relatively infrequent clinical entity observed in 0.3% of the general population and accounting for 3% of all solid renal masses. AMLs are benign mesothelial tumors, with three histologic characteristics: mature adipose tissue, blood vessels, and smooth muscle cells. Most of AMLs are asymptomatic and found incidentally on imaging examinations. It has been described 2 types:isolated angiomyolipoma and angiomyolipoma associated with sclerosis. When AML is associated with complex clinical situations, such as developed in a solitary kidney, bilateral presence, large or multicentric tumors of those associated with tuberous sclerosis (Bourneville's disease), or pregnancy, have medical interest and the management of these are quite challenging. To the best of our knowledge, during the past 10 years only three cases of massive retroperitoneal hemorrhage, resulting from rupture of a renal angiomyolipoma during pregnancy have occurred. In addition of report of this rare medical case we include brief comments of pathophysiology, diagnosis and treatment of this rare tumor.We report a case of a 28 years old woman, in 33 week of her first pregnancy, who was admitted to our department for severe right flank pain, detected in right hypochondrium, associated with nausea, vomiting, and irritative bladder symptoms. Personal and familial histories were unremarkable. The patient was hemodynamically stable without hematuria, lumbar pain or other urological symptoms. Physical examination revealed no specific findings, a good general condition, an axillary temperature of 38°C, blood pressure of 120/75 mmHg and a heart rate of 78 bpm. Abdominal palpation revealed no masses. The only pathological laboratory test parameter was the hemoglobin 8,7 g/dl and hematocrit of 25,5%, that required the transfusion of two red cell concentrate units.Abdominal ultrasound examination revealed a mass, with mixed echogenity, without acoustic shadowing well circumscribed, expanding at the upper pole of right kidney Fig . The masAfter a couple of hours she was developed an episode of fetal bradycardia, hypotension, and a hematocrit continued to decline, despite repeated blood transfusion, which combined with symptoms of intense lumbar pain and hematuria.Considering the hemodynamic instability of the patient, emergency cesarean delivery, under general anaesthesia, was undertaken, because of foetal distress. Exploration of the retroperitoneal space after foetal extraction, confirmed the presence of a large haematoma and the renal mass., which occupied the intrarenal space Fig. . Right nThe histological study of the resected mass revealed the presence of with admixture of mature adipose tissue, smooth muscle, and thick-walled blood vessels.Fig. A healthy male infant was delivered and the patient had an uneventful recovery.Renal AML are not very frequent in urologic activities, especially when it is associated with pregnancy. The majority of AMLs are asymptomatic , but theThe majority of this kind of tumor, are often solitary, the mean age of presentation is 43 years, 4 times more common in men and, interestingly, involve the right kidney .The true nature of the tumor has not been fully elucidated. Immunological mechanisms seems to be related, since increased numbers of estrogen and progesterone receptors at muscle cell level and the tumour cells contain melanosomes and synthesize melanin because they contain dopa oxidase, are helpful microscopical findings to determine the best management approach ,7. This The management of AML is widely discussed in the literature. Asymptomatic tumors smaller than 4 cm in size should be subjected to periodic ultrasound and CT controls every 6 months ,9. SymptIn conclusion, it seems that these tumors show a greater growth index in pregnant women and the question that may be raised is when is the appropriate time for surgical interference. The second trimester of pregnancy seems to be ideal , since tAML: angiomyolipoma; MRI: Magnetic resonance imaging; CT: computer tomography.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in Chief of this journal.The authors declare that they have no competing interests.All authors have made substantial contribution to concept this case reports


Small bowel adenocarcinomas (SBAs) are rare carcinomas. They are asymptomatic and usually neither endoscopy nor contrast studies are performed for screeningA 72-year-old Japanese male had a positive fecal occult blood test at a regular check-up in 2006. He suffered appendicitis and received an ileosigmoidostomy in 1966. A colonoscopy revealed an irregular mucosal lesion with an unclear margin at the ileum side of the anastomosis. A mucosal biopsy specimen showed adenocarcinoma histopathologically. Excision of the anastomosis was performed for this patient. The resected specimen showed a flat mucosal lesion with a slight depression at the ileum adjacent to the anastomosis. Histological examination revealed a well differentiated intramucosal adenocarcinoma (adenocarcinoma in situ). Immunohistological staining demonstrated the overexpression of p53 protein in the adenocarcinoma.Adenocarcinoma of the ileum at such an early stage is a very rare event. In this case, there is a possibility that the ileosigmoidostomy resulted in a back flow of colonic stool to the ileum that caused the carcinogenesis of the small intestine. Small bowel adenocarcinomas (SBAs) are rare carcinomas. They are asymptomatic and usually neither endoscopy nor contrast studies are performed for screening. Most of SBAs are detected at the advanced stage. Early stage SBAs are extremely rare cases.We report a case of an intramucosal adenocarcinoma (adenocarcinoma in situ) of the ileum mucosa an ileosigmoidostomy. A few cases with adenocarcinoma in situ of small bowel have been reported . There iA Japanese male suffered severe appendicitis and received an ileosigmoidostomy without appendectomy in 1966. A prostatectomy was performed for benign prostate hypertrophy at the age of 67. He also received medical treatment for hypertension. A regular check-up in August 2006, when the patient was 72 years of age, revealed a positive fecal occult blood test. A colonoscopy was conducted by his family practitioner and an irregular mucosal lesion with an unclear margin was detected at the ileum mucosa adjacent to the anastomosis. Histological examination of a mucosal biopsy revealed a well differentiated adenocarcinoma. He was then referred to our hospital in October 2006.He showed no abdominal complaints upon admission. Physical examination showed no abnormal findings other than the operation scar from the bypass operation. Carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were within the normal range. We conducted another colonoscopy and identified an ileo-colonic anastomosis 28 cm from the anal verge. It showed an irregular mucosal surface with a diameter of 4 cm at the ileum  adenocarcinoma of the ileum which originated at the site of the ileosigmoidostomy. This case may provide important information regarding the pathways involved in carcinogenesis of the small intestine.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SS, ST, SK and TO participated in the surgical resection. HM, MI, MK, and TK carried out the histological examination. All authors read and approved the final manuscript.


Angiosarcoma is a relatively rare soft tissue tumour. It usually occurs in the head and neck, and especially in the scalp, in elderly people. Its presentation varies from a small plaque to multifocal nodules. The treatment depends on the extent of the disease. Most cases are treated with wide excision with reconstruction. Radiotherapy and chemotherapy are advocated in the recurrent or extensive lesions with regional or distant metastasis. Other modalities such as immunomodulation have been tried. A case of a 55-year-old female patient with a bleeding scalp lesion is presented. Initially thought to be a pyogenic granuloma, on excisional biopsy it was diagnosed as angiosarcoma with microscopic involvement of the margins. Wide excision with reconstruction using a local rotation flap was done at a second stage. The patient was not given postoperative radiotherapy or chemotherapy. There has been no recurrence for two years. Angiosarcoma is a rare soft tissue sarcoma usually seen in the head, face and neck. Five per cent of the soft tissue sarcomas occur in the head, face and neck of which 10% comprise angiosarcomas.1711 Angio711511A 55–year-old female patient presented to the outpatient department with profuse bleeding from a scalp swelling discovered during combing of her hair. The swelling had bled similarly about four to five weeks ago. However, it had now rapidly increased in size over the last week and was bleeding more frequently, even with minor friction.On examination, a 2 cm × 1.5 cm × 1 cm lesion was seen over the right parietal region of the scalp. It was brownish black in colour, firm in feel with irregular margins and started bleeding even as it was being palpated. The rest of the scalp was normal. There were no palpable lymph nodes in the neck. Excision of the lesion with primary closure was performed under local anaesthesia on the same day with a provisional diagnosis of infected scalp granuloma, and the specimen sent for biopsy. Histology, however, revealed dissecting and anastomosing vascular channels with focal mild cytological atypia in the dermis present beneath ulcerated and inflamed squamous epithelium. Secondary haemorrhagic infarction and necrosis were also noted. Histologically, features were consistent with a vasoformative neoplasm involving the dermis, a cutaneous angiosarcoma of the scalp, with involvement of the excised margin on one side .Since histopathological examination of the excised margin on one side showed involvement, revisional surgery in the form of a wide excision of the scar  was thenSutures were removed on the ninth postoperative day and the wound healed well . Histopaet al., have reported a diffuse widespread angiosarcoma of the scalp presenting with Kasabach-Merritt phenomenon with consumption coagulopathy and thrombocytopenia which resolved only with the regression of the tumour[et al., have reported a rare case where the angiosarcoma of the scalp presented as extensive scarring alopecia.[et al., have emphasized on the need for early diagnosis and aggressive management which includes wide tumour excision.[11Angiosarcoma is a rare soft tissue sarcoma arising from the endothelial cells varying from well-differentiated tumours to poorly differentiated ones. Microscopically, they involve the dermis while the poorly differentiated ones may invade into deeper structures. Low-grade angiosarcomas are well differentiated while the high-grade lesions are poorly differentiated and consist of sheets of pleomorphic cells with areas of haemorrhage, disordered architecture, cells with hyperchromatic and pleomorphic nuclei with prominent mitotic activity. Both types are associated with extensive local growth. The grade of the tumour, however, does not have any correlation with survival.11 Cutane351157111711he tumour while Knalopecia. It has talopecia. Delayed excision. Multifoccision.11 while prCutaneous angiosarcoma is difficult to treat due to its multicentric occurrence and the presence of extensive microscopic spread that is very common in these tumours. The treatment described is wide excision of the lesion to achieve histologically tumour-free margin as this has a direct impact on the prognosis.81114 As 811Angiosarcoma of the scalp is a very aggressive tumour with poor prognosis. Outcome can be improved with early diagnosis and aggressive early treatment. In all elderly patients with lesions on the scalp and head and neck region, the possibility of angiosarcoma should be kept in mind to avoid unnecessary delay in treatment. Wide surgical excision to achieve tumour-free margin is associated with improved survival. Widespread lesions where tumour-free margins cannot be achieved, or patients with local recurrences and metastasis, need other modalities. Lifelong surveillance is advised to detect any delayed distant metastasis.


In Crohn's disease, the extension of active terminal ileitis into a Meckel's diverticulum is possible, but usually has no impact on clinical decision-making. We describe an original surgical approach in a young woman presenting with a combination of perforated Meckel's diverticulitis and active Crohn's ileitis.We report the case of a 22-year-old woman with Crohn's disease, who was admitted for abdominal pain, fever and diarrhoea. CT scan demonstrated active inflammation of the terminal ileum, as well as a fluid collection in the right iliac fossa, suggesting intestinal perforation. Laparoscopy was performed and revealed, in addition to extensive ileitis, a 3 × 3 cm abscess in connection with perforated Meckel's diverticulitis. It was therefore possible to avoid ileocaecal resection by only performing Meckel's diverticulectomy; pathological examination of the surgical specimen revealed the presence of transmural inflammation with granulomas and perforation of the diverticulum at its extremity.Crohn's disease of the ileum may be responsible for Meckel's diverticulitis and cause perforation which, in this case, proved to be a blessing in disguise and spared the patient an extensive small bowel resection. The prevalence of Meckel's diverticulum in patients with Crohn's disease is probably similar to the general population, although some authors have reported an increased 5.8%) frequency . Extensi.8% frequA 22-year-old Caucasian woman was admitted because of severe abdominal pain, fever and diarrhoea. She had been diagnosed with Crohn's disease 2 months before, and a computed tomography (CT) scan demonstrated active inflammation of the terminal ileum, as well as a 3 × 3 cm abscess in the right iliac fossa Figure . The iniLaparoscopy was performed, and revealed features typical of extensive small bowel Crohn's disease, involving the last 80 cm of the ileum, as well as a fistulising 3 × 3 cm abscess adherent to the anterior abdominal wall. The origin of the abscess proved to be a perforated Meckel's diverticulum Figure . A conseThe postoperative course was uneventful and medical treatment of the underlying Crohn's disease proved subsequently successful, with clinical and biological parameters of inflammation returning to normal within 10 days. Seven months after surgery, the patient reports one episode of diarrhoea per week; her blood tests are normal and azathioprine was reduced to 100 mg OD.We report the case of a young woman who presented with extension of ileal Crohn's disease lesions into adjacent Meckel's diverticulum, resulting in perforation and abscess formation. A similar case was previously described in an elderly patient who did not have active Crohn's disease of the ileum, either distal or proximal to the diverticulum . In our sine qua non conditions for performing a Meckel's diverticulectomy were met in this specific case: 1) the absence of stricturing disease distal to the perforation; and 2) the fact that the diverticulum was quite long with a relatively healthy base, which appeared suitable for linear stapling. Obviously, great care was taken to fire the stapler alongside the axis of the small bowel in order to avoid any reduction of the bowel endoluminal diameter.Two surgical strategies were conceivable in this peculiar situation: either an ileocaecectomy or a Meckel's diverticulectomy. The first option offered the opportunity to perform a relatively safe ileo-caecal anastomosis, but required an extensive (80 cm) small bowel resection in a young patient. We chose to preserve as much as possible of her small bowel and limited the resection to the Meckel's diverticulum. This alternative, in a septic environment, on top of active Crohn's disease, carried the risk of staple line disruption, and would not have been our first choice in an older patient. Two This case illustrates how Crohn's disease may extend into adjacent Meckel's diverticulum and cause perforated diverticulitis. In this young patient, however, this unusual combination was a blessing in disguise, the septic complication being taken care of without any small bowel resection.BD: twice daily; CT: computed tomography; OD: once daily; TID: three times dailyThe authors declare that they have no competing interests.FS and PG analyzed and interpreted the data, operated on the patient and wrote the manuscript. PS was involved in the endoscopic and gastrointestinal follow-up of the patient before and after surgery. TMK performed the histological examination. PM was a major contributor in writing the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this Journal.


We report an unusual case of a giant lithobezoar that was extending from the caecum to the anal canal, and the patient had no features of absolute constipation or peritonitis. It is believed to be the first such giant colonic lithobezoar in the literature. Bezoars are rare foreign bodies in the gastrointestinal tract. The stomach is the most common site. Primary colonic bezoar is an exceptionally rare situation. Colonic lithobezoars are very rare findings in children.A 9-year-old male child was admitted with a 3-year history of pica, recurrent constipation, abdominal pain, failure to thrive and painful defecation. Abdominal examination revealed moderate distention with multiple palpable intraluminal masses along the rectosigmoid, descending colon and the ascending colon up to the illeocaecal region, with no features of peritonitis. Anal inspection revealed stone pellets protruding through the anus. The rectal examination revealed hard, prickly masses filling the dilated rectum. It was impossible to negotiate and pass around the masses occluding the rectum.th day, another radiograph of the abdomen revealed no residual stones. Mental health assessment by the pediatric psychiatrist did not reveal any gross abnormality. The patient was subsequently discharged on day 9 and was followed-up for a period of 6 months and was put under strict parental supervision. He had increased appetite and gained weight.A plain radiograph of the abdomen showed numerous opaque shadows of different sizes scattered throughout the colon . Under gPica is persistent eating of nonnutritive substances for a period of at least 1 month. It is considered normal for children <2-years-old to put anything in their mouth. After this age, eating nonfood items is thought to be abnormal. The cause of pica is unknown, but multifactorial etiology is suggested. Some causes include iron deficiency, psychological factors like poverty, maternal neglect and abuse, lack of parental supervision, disorganized family situation, mental retardation, autism and brain behavior disorders like Kleine–Levin syndrome. The variBezoars may be composed of hair (trichobezoars), vegetable matter (phytobezoars), milk curds (lactobezoars), sand bezoar and, ver6Clinically, these patients often present with signs and symptoms of bowel obstruction. A palpable abdominal mass is occasionally found. On rectal examination, the presence of the “colonic crunch sign” can increase the suspicion of bezoar obstruction. The colonic crunch sign is defined as the palpation of a prickly mass on digital rectal examination and can be found in sunflower seed bezoar and lithobezoar.Plain abdominal radiograph is especially important in the diagnosis of this kind of colonic intraluminal mass. The scattered radioopaque nature is typical of lithobezoar. This unique appearance on plain abdominal radiograph was called as “corn on the cob.” Anal dilWe conclude that pica is not as rare as once thought, and can lead to an array of surgical complications if left untreated. These patients can be managed conservatively in the initial stages, but frequently need surgical intervention if complications occur. Finally, these patients should be kept under strict follow-up and psychiatric assessment should not be forgotten. Strict parental supervision is the single most important factor to be considered if the disease incidence is to be decreased.


Leiomyomas are benign smooth muscle cell tumours. They are the most common uterine neoplasms, although they may also occur elsewhere, such as in the gastrointestinal and urinary tracts. Leiomyomas are uncommon in von Recklinghausen's neurofibromatosis (NF1). However, the literature suggests that the association of NF1 and leiomyomas or leiomyosarcoma is not entirely coincidental.We report here the unusual case of a 47-year-old woman with NF1 who presented menorrhagias and a hard, tender pelvic mass composed of uterine leiomyomas. Positron emission tomography/computed tomography disclosed an area of increased 18-fluorodeoxyglucose (FDG) uptake in the upper right part of the mass that raised the suspicion of malignancy. Magnetic resonance imaging revealed numerous intramural leiomyomas ranging from 1 to 6 cm, including a 6-cm submucosal leiomyoma that had abundant cellularity, matching FDG uptake. Abdominal hysterectomy was performed and microscopic examination confirmed the diagnosis of multiple benign smooth muscle tumours (uterine leiomyomatosis).Our case illustrates several diagnostic issues that arose while exploring this pelvic mass. Moreover, the association of uterine leiomyoma and NF1 may not be fortuitous. Leiomyomas are benign smooth muscle cell tumours. They are the most common gynaecologic and uterine neoplasms, although they may also occur in other organs . These tA 47-year-old woman was followed for sporadic NF1 diagnosed by the association of numerous café-au-lait spots, cutaneous neurofibromas (NFBs) of the limbs and trunk, nodular NFBs of the scalp, and a plexiform NFB of the ankle. Her medical history was notable for a thoracic paraspinal NFB extending into the neural foramina of T12-L1, which had been resected neurosurgically in 2005, and numerous cutaneous NFBs treated by CO2 laser sessions on a regular basis. During follow-up, a 10-cm, hard, tender pelvic mass was discovered that had been absent at the prior examination 12 months earlier. She acknowledged having experienced menorrhagias and pelvic pain and pressure for several weeks. Laboratory testing revealed decreased ferritin  without anaemia and a mild inflammatory syndrome with a slightly elevated erythrocyte sedimentation rate at 30 mm, elevated fibrinogen of 5.5 mg/l (N < 4 mg/l), and C-reactive protein within the normal range. Transabdominal ultrasound imaging displayed numerous leiomyomas with a normal endometrium. Positron emission tomography/computed tomography (PET/CT) disclosed an area of increased 18-fluorodeoxyglucose (FDG) uptake in the upper right part of the pelvic mass. Magnetic resonance imaging (MRI) was then performed to evaluate the diagnosis of uterine leiomyomatosis, to map the lesions, and to rule out the diagnosis of NFB or malignant lesion-like sarcoma. MRI confirmed numerous intramural leiomyomas ranging in size from 1 to 6 cm. A 6-cm submucosal leiomyoma displayed abundant cellularity, matching the FDG uptake on PET/CT. The endometrium and ovaries were normal. The patient subsequently underwent abdominal hysterectomy without salpingo-oophorectomy to rule out a malignant lesion. The specimen consisted of a uterus with attached cervix. The uterus measured 14 × 12 × 10 cm and the cervix measured 4 × 3 cm. The myometrium contained multiple nodules consistent with intramural, submucosal and subserosal leiomyomas. The endometrium was smooth without anomaly. Microscopic examination of the myometrium revealed long fascicles of spindle cells without nuclear atypia and eosinophilic cytoplasm, few mitotic figures < 1/10 (at × 400) and no tumour necrosis. The endometrium and the cervix were normal. The aspect was in favour of multiple benign smooth muscle tumours (uterine leiomyomatosis). No leiomyosarcoma or neurofibroma was diagnosed.Leiomyomas, which are benign smooth muscle cell tumours, are the most common uterine neoplasms. Approximately 20-30% of women over 35 years with uterine leiomyomas have clinical manifestations  . ExtrautWe report here a case of uterine leiomyomatosis in a 47-year-old patient with NF1 that raised some diagnostic issues. The lesions were asymptomatic until the patient presented within a year of her last examination with a highly suspect, tender abdominal and pelvic mass. Moreover, one of the lesions displayed focal FDG uptake, suggesting a potential neoplastic lesion. However, MRI confirmed that the lesion was a leiomyoma with abundant cellularity. This situation is uncommon but not rare in pre- and post-menopausal women. Abundant cellularity and hormonal dependency without malignant transformation are responsible for such uptake . AnotherNF1 gene is a tumour suppressor gene located on chromosome 17 that encodes neurofibromin, a widely expressed negative regulator of intracellular RAS signalling in various tissues. Patients with NF1 are prone to develop various tumours. Case reports of gastrointestinal leiomyomas in patients with NF1 have been described on a regular basis in the literature since the fifties [et al reported a case of a 40-year-old woman with NF1 and an abdominal mass that revealed both uterine and gastric leiomyomas [et al reported the case of a 39-year-old woman with a plexiform NFB of the uterus associated with adenomyosis and uterine leiomyoma [At first glance, the association of uterine leiomyoma and NF1 may seem coincidental, as the first condition is frequent in the general population. However, there are several reports suggesting a true link between leiomyoma and NF1. The  fifties ,8,9. Pat fifties ,10. In a fifties ,12. To tiomyomas . Moreoveeiomyoma . The femeiomyoma . NFBs areiomyoma . Differeeiomyoma .We here report a new case of uterine leiomyomatosis during NF1. This condition may be under-recognised because uterine leiomyomas are frequent in the general population. Physicians should keep in mind that female patients with NF1 presenting nonspecific symptoms such as menorrhagias, pelvic pain, discomfort and pressure should be examined for pelvic conditions including neurofibromas and leiomyomas ,15. In oWritten informed consent was obtained from the patient for publication of this case report.A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NK and BG made major contributions to conception and design, acquisition of data, analysis and interpretation of data and drafting the manuscript. HP performed the pathological examination of the leiomyomas and was involved in drafting the manuscript and critically revising it for important intellectual content. All authors read and approved the final manuscript.