Leiomyosarcoma of the rectum is a very rare mesenchymal tumor. Because of its rarity, its diagnosis, treatment, and pathology often present challenges to the clinician. The characteristics of this tumor, such as its anatomical location, heterogeneous solid features on imaging, and nonspecific lower gastrointestinal tract symptoms, can be confused with those of primary ovarian carcinoma. Here, we report the case of a 52-year-old-woman presenting with a low abdominal mass that was later pathologically confirmed to be a rectal leiomyosarcoma. The findings of preoperative ultrasonography, pelvic magnetic resonance imaging, and abdominopelvic computed tomography were suggestive of a malignant pelvic mass, most likely a primary ovarian carcinoma. The patient underwent explorative laparotomy, and intraoperative frozen examination revealed a sarcoma originating from the gastrointestinal tract. Low anterior resection and supracervical hysterectomy with bilateral salpingo-oophorectomy were performed. The patient’s postoperative course was uneventful, and adjuvant chemotherapy is currently being administered. Rectal leiomyosarcoma is an uncommon malignancy accounting for less than 0.1% of all malignancies of the colon and rectum . The preA 52-year-old woman  presented to the gynecology clinic at Guro Hospital, College of Medicine, Korea University, with a complaint of low abdominal pain. In addition to low abdominal pain, the patient had experienced low back pain, urinary frequency, and tenesmus for approximately 1 year. She had undergone myomectomy 2 years previously in China.18F-FDG PET/CT, a large pelvic mass with heterogeneous metabolism was identified, suggesting the presence of a malignant mass (Figure The patient’s menses had ceased at the age of 50. She had no complaints of vaginal bleeding or low abdominal distension. She has never undergone a cervical screening test. On pelvic examination, the cervix was found to be small, and no specific abnormalities were observed. On physical examination, a hard mass with an irregular surface was palpable. A Pap smear and a HC2 test were performed. The Pap smear result was a low-grade squamous intraepithelial lesion, and The HC2 test result was negative for high-risk human papillomavirus. Transvaginal ultrasonography examination revealed an approximately 11-cm mixed echogenic solid mass of the left adnexa, which strongly suggested the presence of a malignant tumor (Figure s Figure . No specExplorative laparotomy with a supraumbilical incision was performed. A large mass with a longest diameter of approximately 20 cm was identified. This mass was located in the cul-de-sac and was densely adhered to the rectum, retroperitoneal surface, and posterior uterine body. The uterus and both ovaries and tubes were normal on gross appearance. A portion of the mass was subjected to frozen examination, and the result was a mesenchymal tumor originating from the gastrointestinal tract, such as a gastrointestinal stromal tumor. We then performed supracervical hysterectomy and bilateral salpingo-oophorectomy. After the completion of gynecologic surgery, a surgical team from the department of colorectal surgery subsequently performed anterior resection of the colon and appendectomy. The operation was uneventful, and the patient was transferred to the department of colorectal surgery after the operation.3 in size, with free mucosal resection margins. The mitotic count was ≥20 per 10 high-power fields, and an area of necrosis was identified. The tumor cells had characteristically elongated, pleomorphic, and blunt-ended nuclei and eosinophilic to pale cytoplasm (Figure Histopathological analysis of the full specimen revealed a grade 3 leiomyosarcoma, 22 × 17 × 6 cmm Figure . On immuLeiomyosarcomas account for 10–20% of all soft tissue sarcomas. They arise most frequently from the uterus, gastrointestinal tract, and retroperitoneal region . Those oRadiological differentiation between leiomyosarcomas and leiomyomas is difficult, and the final diagnosis needs to be confirmed by postoperative pathological examination. Histological analysis of superficial biopsy samples might not reflect the entire tumor mass and leiomyosarcoma can be misdiagnosed as benign leiomyomas. In addition, malignant potential can be missed on CT of MRI. Given that leiomyosarcoma has a very poor prognosis, misdiagnosis may give a detrimental effect on patient outcome. In this case, all preoperative evaluations, excluding those of serum tumor markers, identified primary ovarian cancer as the most probable diagnosis. To this point, no specific tumor markers have exhibited clinical utility for the diagnosis of leiomyosarcomas. In one series of 10 cases of colorectal leiomyosarcomas, 9 of 10 patients had lobulated tumor margins on CT or MRI . Except A distinguishing feature of our case is that the rectal tumor mass was not identified on total colonoscopy. Generally, colonoscopic examination including sigmoidoscopy shows a polypoid, submucosal mass occupying the lumen, leading to the suspicion of a tumor originating in the rectum. In such cases, rectal bleeding or obstruction can occur, prompting an evaluation of the possibility of colorectal problems. However, some rectal leiomyosarcomas growing away from the lumen, so-called exocolic growth, may not be detected on colonoscopic examination. The growth pattern of the tumor and the lack of specific symptoms might be responsible for the failure of preoperative diagnosis.Pathologically, leiomyosarcomas can be distinguished from leiomyomas on the basis of the following features: larger tumor cells, fewer stromal fibers, increased mitotic activity, and nuclear pleomorphism . Among tThe spread of rectal leiomyosarcoma is mainly local or hematogenous, although lymphatic metastasis has been reported in some poorly differentiated tumors . In our In this study, we presented a case of rectal leiomyosarcoma mimicking primary ovarian carcinoma. We can draw a lesson from this case report. When a woman presents with a pelvic mass that is suggestive of ovarian carcinoma on radiological evaluation but is suggestive of ovarian cancer on the basis of normal serum tumor marker levels, the possibility of rectal leiomyosarcoma should be suspected even in the absence of rectal bleeding or pain. We also caution that appropriate surgical treatment should not be delayed solely on the basis of normal tumor marker levels. Because of its poor prognosis, early diagnosis and prompt surgical removal are important for patients with rectal leiomyosarcoma.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.Pap: Papanicolaou; HC2: Hybrid Capture 2; CA 125: Carbohydrate antigen 125; CEA: Carcinoembryonic antigen; AFP: Alpha fetoprotein; beta-hCG: beta-human chorionic gonadotropin; MRI: Magnetic resonance imaging; CT: Computed tomography; FDG: fluorodeoxyglucose; PET: Positron emission tomography; CD: Cluster of differentiation.The authors declare that they have no competing interests.YTO drafted the manuscript. JHH supervised the study. KJM and KAS were involved in design and acquisition of data. JKL revised the manuscript and gave final approval of the version to be published. All authors read and approved the final manuscript.


The authors describe the combination of awake craniotomy and minimally invasive endoscopic port surgery to resect a high-grade glioma located near eloquent structures of the temporal lobe. Combined minimally invasive techniques such as these may facilitate deep tumor resection within eloquent regions of the brain, allowing minimum white matter dissection. Technical aspects of this procedure, a case outcome involving this technique, and the direction of further investigations for the utility of these techniques are discussed. Awake craniotomy with cortical mapping has served as the gold standard for functional preservation in brain tumor surgery since its introduction over half a century ago . For theFor deep-seated brain tumors, resection using a minimally invasive cylindrical retractor has the potential to minimize damage to the surrounding white matter incurred as a result of the surgical channel into the tumor. This approach, initially championed by Kelly –7, has bIn this paper, the authors describe the combination of awake craniotomy with electrophysiological brain mapping and EPS to resect a glioblastoma of the dominant temporal lobe. The case is an example of a deep-seated, eloquent tumor for which the combination of the two techniques seemed intuitive in order to facilitate a favorable outcome. A 63-year-old, right-handed man presented with a 6-month history of gait deterioration, requiring a walker. For the 3-4 weeks prior to presentation, he had also experienced a new onset of memory difficulties and subtle trouble with word finding, as well as mild headaches and declining vision. Of note, the patient had a history of spinal cerebellar atrophy (SCA). The patient's neurologic exam was remarkable for mild dysmetria, dysdiadochokinesia, and a positive Romberg sign, in addition to subtle fluent dysphasia and memory dysfunction. An MRI scan revealed a left temporal lobe mass measuring 3.5 cm × 2 cm abutting the temporal horn of the lateral ventricle . NotableA craniotomy for tumor resection was recommended to the patient in order to achieve a tissue diagnosis, alleviate neurologic symptoms, and extend survival given the presumptive diagnosis of high grade glioma.The surgical technique of the authors is generally to use a twilight-awake-twilight model of anesthesia for these cases. The patient is fully off sedation for the entirety of the intradural portion of the case during which time the patient undergoes cortical mapping followed by continuous interaction with the neurophysiology team including reading flashcards, performing recall exercises, and answering simple questions. In this particular case, a temporal craniotomy was performed in the standard fashion centered over the middle temporal gyrus. Frameless image guidance was used to guide the placement of the bone flap, which was approximately 3 cm in diameter. Cortical mapping of the exposed temporal lobe did not reveal any changes in speech functions. The tumor was cannulated through the middle temporal gyrus using a previously described method  Figure . ResectiPostoperative MRI scan demonstrated a 93.3% volumetric resection of the tumor, with minimal residual tumor noted in the anteroinferior margin of the tumor bed . PostopeAt a one week postoperative followup, the patient was observed to have slight horizontal nystagmus that was noted postoperatively. He reported that his vision seemed to be improving. The patient did slur his speech on occasion; however, this was consistent with his baseline speech preoperatively. No new impairments of language or cognition were noted secondary to the surgery. The patient was undergoing speech and physical therapy and had been referred for radiation therapy and chemotherapy due to his GBM diagnosis. After 7 months, the patient had an MRI showing multifocal enhancement within the dominant hemisphere. MR spectroscopy was suggestive of multifocal tumor progression. The patient was subsequently treated with two cycles of avastin. At the 9-month followup, the patient has been noted to have improved T2 and FLAIR images consistent with the avastin effect. On the neurological exam, the patient was mildly dysarthric as had been noted prior to surgical intervention. The patient continued to demonstrate mild abnormalities with word finding but could recall 3/3 words in 5 minutes when given categorical clues. The patient had no other new motor, sensory, or cognitive defects noted on exam.Removal of brain tumors within eloquent brain regions can result in iatrogenic motor deficits, speech disorders, and complex cognitive deficits , 3. HoweNumerous publications have described successful awake craniotomy protocols , 14 and Of the factors portending good functional outcome in the removal of high grade gliomas in the temporal lobe using awake craniotomy, the most significant has been reported to be the distance of the surgical margin from the nearest language site . This hiThe authors describe a case of awake craniotomy with cortical mapping followed by resection of an intraparenchymal tumor using an endoscopic port. In rare cases, the combination of these two techniques may be an option to facilitate deep tumor resection within eloquent regions of the brain.


A case of follicular ameloblastoma of the left maxilla in a 74-year-old man is reported. The tumor was presented as a radiographically solid mass filling the left sinonasal cavity and invaded maxillary alveola. After radical surgery, the patient has pursued a non-aggressive clinical course after 4 years of follow-up. The radiopathological features of this tumor were reviewed and the possibility of its sinonasal epithelium origin was discussed. Ameloblastoma is a benign epithelial odontogenic tumor, locally invasive and of slow growth. This lesThe estimated incidence of ameloblastomas is approximately 0.5 per million populations per year. There is no distinct gender predilection. Most cases are diagnosed between 30 and 60 years of age. Solid amThe literature showed that solid ameloblastoma occurred as the least frequent in maxillary bone.5]6] Am[6] Am5][ Am[6] AmThe overwhelming majority of cases affecting the sinonasal cavity are tumors that grow in the maxilla and secondarily extend through the nasal and paranasal cavities. There are only few genuine primary sinonasal ameloblastomas (SNAs) reported without connection with gnathic areas.The aim of this article was to report a case of follicular ameloblastoma in the maxillary sinus in a 74-year-old man presenting as sinusitis. The clinical features misled a primary care physician into incorrect diagnosis and treatment. Pitfalls of diagnosis and management of ameloblastoma in the maxillary sinus were briefly reviewed.A 74-year-old man was referred to our department with recurrent sinusitis and a non-healed extraction wound was left after removal of the regional tooth about two years ago. The patient was diagnosed with acute sinusitis by a family doctor. The patient was managed by antibiotic therapy ‘without’ radiographic and histopathological examination.The patient’s medical and familial histories were unremarkable. The clinical appearance of the lesion showed a central depressed part with superficial yellowish-white necrotized tissue surrounded by a narrow erythematic rim and mucosal hyperplasia. The lesion was not tender nor bleeding. There was a bony hard alveolar swelling in buccal aspect of premolar and molar region. No significant pus discharge was detected. Mucosal surface was normal in color and texture. Other parts of oral cavity showed normal color and texture. Oroantral fistula had been considered as an early diagnosis .On panoramic view, complete loss of alveolar process in left premolar and molar region was detected. Borders of left antrum were not clearly visible. There was no remnant of bone trabequla or sequestra . Water`sCT demonstrated a massive expansile lesion invading the entire left maxillary sinus. The lateral nasal wall and labial cortex of the maxilla as well as ethmoidal sinuses were involved. Osteomeatal complex and inferior and lateral wall of antrum in the left side were not visible .Regarding the size of lesion in antrum, an origin of sinus was suspected.The incisional biopsy revealed fragments of a benign neoplasia of odontogenic origin, characterized by the proliferation of small odontogenic epithelium islands and cords interlarded by a dense fibrous stroma, exhibiting intense collagenized areas. Follicular islands of odontogenic epithelium were bordered by columnar and palisaded ameloblastic cells with polarized hyperchromatic nuclei. Some of the stellate reticulum components of this follicular ameloblastoma showed squamous differentiation .A radical left maxillectomy was performed. Multiple fragments from left middle and inferior turbinate, left maxillary sinus, left maxilla, left ethmoid tissue and left masseteric region were fixed in formalin submitted to laboratory. Sinonasal follicular ameloblastoma was reported along with mild chronic inflammation in the left ethmoid, maxilla and left middle turbinate tissue. The patient had no sign of recurrence after 4 years follow up.Ameloblastomas are epithelium-derived odontogenic tumors that typically are originated in jaw bones, primarily involving the mandible and less often the maxilla. The presence of ameloblastomas in the sinonasal region is usually secondary to an extension of a tumor of gnathic origin into this area. However,In this case with alveolar process involvement, radiographic views were not enough to make the diagnosis of sinonasal ameloblastoma. In some CT scan slices, primary involvement of maxillary sinus can be suspected. So, it is not easy to exclude extension into maxillary bone from a primary sinonasal tract ameloblastoma. This may support the theory of creating a pathway to maxillary bone through extraction socket. However, the pathologist impression was a sinonasal ameloblastoma.Some studies claim that, the ameloblastomatous epithelium could have originated in the submucosa and secondarily extended to involve the surface epithelium rather than originating from this epithelium. However,As we previously noted, most gnathic ameloblastomas appear in patients 35 to 45 years old. By contrThe differential diagnosis may remain a challenge in small biopsies if tissue fragments obtained for diagnosis are superficial and the typical histology of the tumor is not well represented in them. CT may aid diagnosis, but MRI seems to be more useful imaging technique, which demonstrates the heterogeneity of the signal, due to its multicystic composition and its irregular contrast uptake, which was not available in this case. AccordinSurgery is usually the treatment of choice. Recently endoscopic management of benign sinonasal tumors has resulted in less radical surgical approach, decreased morbidity and better tumor control. Hertog eGenerally, ameloblastomas are originated in jaw bones. Sinonasal ameloblastomas are exceedingly rare tumors without extension from the gnathic region. The prognosis of the treatment is basically dependent to the extension of the lesion and adjacent structures involvement rather than origin of lesion.


Appendiceal tumours are uncommon but may be present in 0.9–1.4% of all appendicectomy specimens. While carcinoid tumours and adenocarcinomas comprise the majority of appendiceal tumours, rarely, lymphomas or sarcomas may also present in the appendix. Appendiceal leiomyosarcomas are rare, and to date, only a handful of cases have been reported. The current paper presents a case of giant appendiceal leiomyosarcoma followed by a review of the literature. A 52-year-old man presented with iron-deficiency anaemia (haemoglobin of 58 g/L) and 40 kg weight loss for investigation. He had previously undergone colonoscopy for family history of colorectal cancer 2 years prior to presentation with no significant findings reported. Physical examination revealed a large mildly tender mass in the right lower quadrant of his abdomen. According to the patient, this mass had been present and slowly enlarging over two years. There was no generalised lymphadenopathy, and rectal examination was normal. Tumour markers  were normal. CT scan revealed a large necrotic mass measuring 19 × 17 cm  with no Pathologic examination showed a large tumour arising from the wall of the appendix . MicroscAn outpatient PET scan did not demonstrate any residual intra-abdominal or distant disease. Radiation and medical oncology reviews were sought but in view of complete surgical excision and nature of leiomyosarcomas, and the patient initially was not offered any adjuvant therapy. Unfortunately, the patient represented with symptomatic anaemia nine months later and was found to have local and systemic recurrence on CT and PET scan. He is currently receiving radiotherapy for symptomatic control.Appendiceal neoplasms are rare and occur in approximately 0.9–1.4% of all appendicectomies . CarcinoDue to its rarity, little is known specifically about appendiceal leiomyosarcomas. Instead, most data is extrapolated from the behavior of sarcomas and experience from treatment of leiomyosarcomas of the colon. Based on the review by Hatch et al., most leiomyosarcoma of the appendix and colon occur between the age of 20 and 70 and that most are symptomatic, presenting with pain, acute or chronic gastrointestinal bleeding, weight loss, or constipation . PreoperAs with the management of any soft-tissue sarcoma, surgical excision with en bloc excision of contiguously affected viscera with clear resection margins is the mainstay of treatment and the only option that offers a chance at cure , 4. Unli


The primitive neuroectodermal tumor (PNET) belongs to a group of highly malignant tumors and is composed of small round cells of a neuroectodermal origin. Categorized in the same tumor family as Ewing sarcoma, the PNET is most likely to occur in bones and soft tissues. However, a small number of PNET cases arising in the pelvis have been reported as well. We present three cases of pelvic PNET: two cases in the ovary and one case in the broad ligament. The PNET often exhibits aggressive clinical behavior with worse outcomes than other small round cell tumors. The significant prognostic factors of the PNET include site of tumor, volume of neoplasm, and presence of metastasis. The treatment protocol is multimodal and includes local surgical treatment followed by chemotherapy. We herein describe three PNET cases as a rare entity in the pelvis. Pelvic PNETs should be included in the differential diagnosis of pelvic masses. A group which often involves the sympathetic nervous system or soft tissues and bones is described as the peripheral PNET; this group arises from the neural crest and primitive neuroendocrine cells.2 Risk factors for the PNET have yet to be clearly identified.3 Pelvic PNETs are usually observed in the uterine corpus, ovaries, cervix, and vulva.4,5 Also, the literature contains rare cases of the PNET in the broad ligament.6The primitive neuroectodermal tumor (PNET) of the female genital tract is a rare entity. The term “PNET” was first used by Hart and Earle in 1973 to introduce a group of tumors derived from fetal neuroectodermal cells.7 Radiological studies such as Computed Tomography (CT) scan and Magnetic Resonance Imaging (MRI) are essential in the diagnosis of tumor involvement and ruling out of metastatic disease. A review of CT and MRI findings in PNET cases suggests that no characteristic finding aids in the preoperative diagnosis of this tumor. Clinical manifestations depend on the surrounding structures on account of mass effect. Treatment option for the PNET includes surgical resection of the tumor, followed by additional chemotherapy. After successful chemo or/and radiotherapy, the 5-year survival rate is only 7.6-8%.8 A review of literature reported that the 2-year disease-free survival rate in patients with only localized disease is 25%.9 Fortunately, advances in diagnostic modalities and neoadjuvant and adjuvant chemotherapeutic regimens may be improving long-term disease-free survival.Conventionally, the diagnosis of the PNET can be made based on histological examinations. However, immunohistochemical profiles and genetic studies commonly help the pathologist to differentiate between the PNET and other small round cell tumors. Immunohistochemically, the PNET is frequently reactive for vimentin, HMWCK, and CD-99.We herein present three unusual cases of the PNET arising in the pelvis.2A1) who referred to the Tumor Clinic of Ghaem Hospital. The patient’s history included two month’s pregnancy with abortion.Pelvic ultrasonography revealed , mixed, echogenic mass in the left adnex (mean diameter=102×68 cm) with multiple internal septation and adhesion to the surrounding organs. The concentrations of all tumor markers were normal . Pelvic examination detected a palpable, firm, irregular mass in the posterior vaginal fornix. An exploratory laparotomy was performed, and a predominant mass (about 20×15 cm in diameter) situated in the pelvis in the broad ligament was observed. The tumor was soft and friable with hemorrhagic, necrotic, and cystic lesions. The uterine serosa was coated with the tumor. The tumor infiltrated most of the posterior peritoneal wall. After the resection of the tumor, frozen-section analysis identified a malignant tumor, probably lymphoma of the pelvis. The medical oncology consultant required bone marrow aspiration, which showed normocellular bone marrow without evidence of metastatic involvement. Additional metastatic work-up yielded a negative bone scan. Permanent sections and immunohistochemical profile with positive immunoreactivity for HMWCK, vimentin and CD-99 as well as negative immunostaining for LCA, desmin, NSE, and chromogranin allowed the pathologist to distinguish the PNET from other small round cell tumors , involving the left lower abdomen with extension to the xiphoid. Pelvic ultrasonography revealed a multi-loculated, echogenic mass in the pelvis and abdomen, and CT-scan identified a large retroperitoneal mass with liver metastasis . The CA-The third patient was a 43-year-old woman (G3 P3) with a history of pelvic pain and distention in the hypogastric area. In pelvic examination, a large, solid, round mass (about 25×30 cm in diameter) with extension to the umbilical area was detected. Pelvic ultrasonography revealed a well-defined, mixed, echogenic mass (mean diameter=22×35 cm) in the right adnex with internal echo with adhesion to the surrounding organs and little ascetics. All tumor markers were normal (CA-125=25 and CEA=4). Exploratory laparotomy showed an irregular, solid mass (about 20×30 cm in diameter) in the right ovary that had infiltrated the other organs of the pelvis. Frozen section reported a malignant tumor, most probably adenocarcinoma of the ovary. Accordingly, surgical staging surgery was performed. Permanent sections and immunohistochemical profile were compatible with the PNET . Four we10 Most cases of the PNET occur in the soft tissue; however, rare cases have been reported in the ovary. Therefore, the PNET must be considered in the differential diagnosis of an unusual tumor in the pelvis, particularly in young patients.11 Clinical symptoms, including pain and swelling of the surrounding organs, are related to the location of the tumor. All of our patients had no specific sign and symptom recommended for the diagnosis of a pelvic PNET.The PNET accounts for 1% of all soft tissue sarcomas and occurs even less frequently in the female reproductive tract. Nearly 80% of the patients are younger than 20 years.In 99% of PNET cases, the reciprocal translocation t is observed in cytogenetic examination. We, however, were not able to ascertain this in our patients because our center lacks the required means for this technique. 12 The most common sites of PNET metastases are the lung, bone, and bone marrow.13 One of our patients had lung metastasis and in the others brain metastasis was encountered in the early postoperative period.In general, the PNET is a very highly aggressive tumor with tendency to extension out of the pelvic cavity. Patients may have metastatic disease at the first visit, so a full metastatic work-up is indicated in a suspected case of the PNET.14 In one of our patients, we did not succeed in performing complete surgical resection. Also, in the other two patients, we found complete pelvic and paraaortic lymphadenectomy impossible.Surgical management of the PNET includes complete resection of the tumor with an effort for debulking if possible. By reducing the tumor size, we can reduce the potential reservoir of drug-resistant tumors. Nevertheless, complete surgical resection of these tumors is not possible due to the aggressive behavior and dissemination of the neoplasm.15All of the three PNET patients in this report had very poor prognoses and died before complete postoperative chemotherapy.Chemotherapy regimens have significantly improved outcomes in patients with the PNET. A postoperative chemotherapy program as an adjuvant therapy is recommended immediately following diagnosis. Many centers utilize the same chemotherapy regimens that are used for gem cell tumors. In the current treatment protocols, chemotherapy regimens encompass a combination of Vincristine, Actinomycin D, Cyclophosphamide, and Doxorubicin. Moreover, a combination of Ifosfamide and Etoposide appears to offer the greatest survival advantage. The prognostic factors of the PNET are dependent on the site, volume, and extension of the tumor.The PNET is an aggressive, malignant tumor and should be considered in the differential diagnosis of pelvic masses.


Peritoneal dissemination with no further metastases of prostate cancer is very rare, with only three cases reported in the available literature. We report the first case of iatrogenic peritoneal dissemination due to laparoscopic radical prostatectomy.A 59-year-old Japanese man underwent laparoscopic radical prostatectomy for clinical T2bN0M0 prostate cancer, and the pathological diagnosis was pT3aN0 Gleason 3+4 adenocarcinoma with a negative surgical margin. Salvage radiation therapy was performed since his serum prostate-specific antigen remained at a measurable value. After the radiation, he underwent castration, followed by combined androgen blockade with estramustine phosphate and dexamethasone as each treatment was effective for only a few months to a year. Nine years after the laparoscopic radical prostatectomy, computed tomography revealed a peritoneal tumor, although no other organ metastasis had been identified until then. He died six months after the appearance of peritoneal metastasis. An autopsy demonstrated peritoneal dissemination of the prostate cancer without any other metastasis.Physicians should take into account metastasis to unexpected sites. Furthermore, we suggest that meticulous care be taken not to disseminate cancer cells to the peritoneum during laparoscopic radical prostatectomy. Peritoneal dissemination with no further metastases of prostate cancer is very rare with, to the best of our knowledge, only three cases reported in the available literature. There has not yet been a report of a patient undergoing surgical treatment that might have resulted in iatrogenic dissemination. We report the first case of iatrogenic peritoneal dissemination due to laparoscopic radical prostatectomy (LRP).A 59-year-old Japanese man presented to our urology clinic with lower urinary tract symptoms. His serum prostate-specific antigen (PSA) level was 9.5 ng/mL. A digital rectal examination revealed a hard induration of his prostate. He had no personal or familial history of malignant disease. A prostate biopsy was performed and showed Gleason score 3+4 adenocarcinoma of the prostate. Computerized tomography (CT) and bone scintigraphy showed no metastasis. He was referred to our Department of Urology for treatment of cT2bN0M0 prostate cancer, and underwent LRP. The operation was performed with a transperitoneal approach. The pathological diagnosis was pT3aN0 Gleason score 4+4 adenocarcinoma with a positive surgical margin.After the operation, his PSA level dropped to 0.7 ng/mL at its lowest, and so salvage radiation therapy with 50 Gy was carried out. His serum PSA level initially dropped to 0.5 ng/mL but began to increase, to 3.5 ng/mL, shortly after. Medical castration was then started. The therapy was effective for 24 months, after which he needed additional anti-androgen agents  and estramustine phosphate because of an increase in his PSA level. Sixty-six months after the prostatectomy (PSA 76.3 ng/mL) dexamethasone was administered, and provided the minimal PSA level, 0.58 ng/mL, 18 months after the initial administration. However, his PSA level increased again, so the endothelin receptor antagonist was replaced by dexamethasone for 12 months with no effect on his PSA level. Thereafter dexamethasone was administered again, and his PSA decreased from 340 ng/mL to 118 ng/mL.After that, his PSA level continued to increase without any metastasis visible on CT or bone scans. Our patient could not undergo chemotherapy with docetaxel because of complications with heart failure and interstitial pulmonary disease. At age 69, 114 months after the LRP, CT showed a peritoneal tumor that was considered to be a peritoneal metastasis , lung 46%), liver 25%), pleural 21%) and adrenal (13%) metastases are reported in some large autopsy series [% and adr5%, pleur6%, liver0%, lung is risks . HoweverThe pathological diagnoses of the previous three cases were Gleason 4 and/or 5 adenocarcinoma with or without mucinous adenocarcinoma Table . Two of Peritoneal dissemination of prostatic carcinoma is a very rare occurrence. Meticulous procedures during LRP should be performed to avoid a dissemination of cancer cells to the peritoneum. The treatment should be performed in accordance with the standard strategy for prostate cancer, including hormone therapy and chemotherapy.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.CT: computerized tomography; LRP: laparoscopic radical prostatectomy; PSA: prostate-specific antigen.The authors declare that they have no competing interests.HY, HK, ST, NM, TS and TT were involved in conception, design and interpretation. HY and HK wrote the manuscript. MT, TM and TT performed the histological examination and provided the histopathological images. All authors read and approved the final version submitted.


The objective of this study is to discuss the presentation, diagnosis, and surgical management of a young, healthy patient with a symptomatic mesenteric cyst. He had a 5-month history of abdominal pain from this disorder, and the case is presented to illustrate the clinical picture and operative management of this rare disorder. Mesenteric cysts are benign lesions that are found within the abdomen. They have an incidence that is less than 1 in 100,000 patients  subsequeThese lesions can present with symptoms such as abdominal pain, nausea, vomiting, anorexia, and a change in bowel habits, however, most commonly they are asymptomatic, and detected incidentally via physical exam, or imaging. Although most mesenteric cysts are benign, these lesions do occasionally cause complications, including intestinal obstruction, volvulus, torsion, or even hemorrhagic shock secondary to bleeding or rupture .This case was presented at the Annual Clinic Day of the Combined Meeting of the Brooklyn & Long Island Chapters of the American College of Surgeons and the Nassau Surgical Society, December 7, 2011, General Surgery & Vascular Section.A 23-year old male presented to his primary care physician's office with a five month history of vague abdominal pain. The pain was described as intermittent and was located in the left lower quadrant. The pain was not brought on by any particular events, nor was it relieved by anything specific. The patient had six episodes of pain in the previous five months, each lasting approximately one hour.The patient had a past medical and surgical history that was only significant for a congenital megaureter on the left side, for which he underwent ureteral reconstruction as an infant, with no further issues. Prior to our operation for the mesenteric cyst, he was found to have normal laboratory values, including markers for malignancy. A preoperative CT of the abdomen  showed aBased on the preoperative CT, a minimally invasive operation was chosen. A total of three trocars were used for the surgery. Initially, a 10 mm trocar was placed at the umbilicus via the open Hasson technique, with two additional bladeless 5 mm trocars placed under direct vision in the bilateral lower quadrants .During the laparoscopic exploration, it was noted that the mesenteric cyst was in the left lower quadrant. It was a thin walled structure, with yellow fluid, and was fixed to the mesentery posteriorly, with no other points of attachment . The cysPathological examination of the cyst revealed the fluid to be benign. The wall of the cyst was found to be fibrous with a histology consistent with a lymphangioma .Mesenteric cysts are rare lesions that commonly are asymptomatic, and found incidentally. However, they can also present with abdominal pain that can be due to rupture, torsion, acute hemorrhage, or compression of nearby structures. In addition, patients can present with nonspecific symptoms, such as anorexia, nausea, vomiting, fatigue, and weight loss.The presence of these symptoms makes it important to assure that these lesions are benign. There have been similar lesions that have been found to be cystic lymphangiomas, cystic stromal tumors, and mesotheliomas on pathologic analysis. In addition, rarely, carcinomas have been found to arise in mesenteric cysts . The etiology of these cysts still remains unclear. It is often discussed that they are a result of degeneration of mesenteric lymphatics, or a congenital anomaly. However, they may also result from a number of etiologies, including previous pelvic surgery (as in our patient), trauma, pelvic inflammatory disease, endometriosis, or neoplasia.The most common physical finding for a mesenteric cyst is Tillaux's sign. This is described as a mass lesion of the abdomen only mobile in the horizontal and not the vertical direction . Even with the presence of Tillaux's sign, it is important to confirm the diagnosis via CT, ultrasound, or MRI. In addition, preoperative imaging can help guide the decision to plan the operation laparoscopically or via an open approach .The first report of a mesenteric cyst was in 1507 and was actually found during an autopsy by Benevenni in Italy, and in 1880 the first successful surgical resection of a mesenteric cyst was performed by Tillaux. Due to the rarity of mesenteric cysts, it was not until 1993 that the first successful laparoscopic surgery was reported by Mackenzie et al.  and subsTherapy for these cysts should only be sought if they are symptomatic or cause complications. Treatment modalities that have been attempted include simple drainage, enucleation, marsupialization, and excision. Although many modalities exist, surgical excision is considered the mainstay for therapy, as recurrence of the cyst can occur with lesser procedures. It should be noted that with cysts that are adherent, it may be necessary to remove part of the mesentery with the mass.


Primary leiomyosarcoma of the seminal vesicle is exceedingly rare.We report a case of a 59-year-old man with tumour detected by rectal symptoms and ultrasonography. Computed tomography and magnetic resonance imaging suggested an origin in the right seminal vesicle. Transperineal biopsy of the tumour revealed leiomyosarcoma. A radical vesiculo-prostactectomy with bilateral pelvic lymphadenectomy was performed. Pathological examination showed a grade 2 leiomyosarcoma of the seminal vesicle. The patient received adjuvant radiotherapy. He developed distant metastases 29 months after diagnosis, and received chemotherapy. Metastatic disease was controlled by second-line gemcitabine-docetaxel combination. Fifty-one months after diagnosis of the primary tumour, and 22 months after the first metastases, the patient is alive with excellent performance status, and multiple asymptomatic stable lung and liver lesions.We report the eighth case of primary leiomyosarcoma of the seminal vesicle and the first one with a so long follow-up. Less than 5% of soft tissue sarcomas (STS) arise from the genitourinary tract. Leiomyosarcomas of the seminal vesicle are exceedingly rare. To our knowledge, only seven cases have been reported to date in the English literature and all with a short follow-up -6. DiagnThe patient was a 59-year old man, Caucasian type, without any specific medical personal or familial history. In October 2006, he underwent a pelvic ultrasonography (US) because he presented a "pressure sensation" in the rectum and rectal imperiosity for four weeks. Physical examination revealed no additional symptom, except the rectal examination that detected a 4-cm hard pre-rectal mass filling the right side of the pelvis. The WHO performance status was equal to 0. Ultrasonography (US) discovered a tumour located at the right side of the pelvis. Serum prostate-specific antigen levels were normal. The patient was thus referred to hospital.Computed tomography (CT) of abdomen and pelvis revealed an 8-cm heterogeneous tumour predominantly located on the right side of the pelvis likely arising from the right seminal vesicle, reaching the median line and adherenting to the posterior bladder wall and the anterior rectal wall. Due to the rectal clinical symptoms and the suspicion of rectal adherence on CT scan, the core needle biopsy was CT-guided transperineal rather than transrectal. Pathological analysis revealed a well-differentiated leiomyosarcoma. Pelvic magnetic resonance imaging (MRI) confirmed the presence of a mass centred on the right seminal vesicle, causing mass effect on the prostate and left seminal vesicle, without a cleavage plane with the right prostate and the right obturator muscle, suggesting invasion Figure . OtherwiBefore surgery, the examination under general anesthesia revealed that the mass was difficult to mobilize. The treatment consisted of a radical vesiculo-prostatectomy with limited (ilio-obturator and hypogastric) bilateral pelvic lymphadenectomy in January 2007, associated with resection of the internal obturator muscle. No anterior rectal resection was necessary. Excision was macroscopically complete. The post-operative course was uneventful. The pathological macroscopic examination of the surgical resection specimen showed a small prostate (4.5 × 4 × 3 cm) and an 8 × 8 × 6.5 cm well-limited hard white-tan mass, centred on the right seminal vesicle, tangent to the prostate base, pushing the vas deferens, the prostate and the left seminal vesicle. On cut section the mass showed a grey-white whorled appearance with foci of necrosis. Microscopic analysis Figure  confirmeIn April 2009, 29 months after diagnosis, he developed two subcutaneous nodules on the scalp. Surgical removal and pathological analysis confirmed the diagnosis of distant recurrences of leiomyosarcoma. At the same time, CT and TEP scans detected the presence of multiple asymptomatic liver and lung metastases. Performance status was excellent (WHO 0). First-line chemotherapy consisted of six cycles combining doxorubicin and ifosfamide, but metastases progressed (October 2009), and the patient was referred to our institution for second-line chemotherapy. We delivered a gemcitabine-docetaxel combination. After three cycles, CT scan showed stable lung and liver lesions, whose size decreased after the sixth cycle, then after the ninth cycle, with a 30% response when compared with October 2009 , or after surgical resection (1 case). In all cases, definitive diagnosis was done by histological examination of the resected tumour. In our case, the finding of a small adenocarcinoma raised the possibility of a sarcomatoid carcinoma but the macroscopical presentation of a clearly extraprostatic tumour, the absence of any connection between the two proliferations, and negativity of epithelial markers by immunochemistry did not favour this diagnosis. Differential diagnosis also concerns leimoyosarcoma arising from adjacent organs  and secondarily invading the seminal vesicle. But today, thanks to the advent of modern imaging tools, the organ of origin can be documented in most of cases by looking at the epicentre of the mass and the pattern of displacement of adjacent organs .The prognosis of leiomyosarcoma of the seminal vesicle is poor, and more unfavourable than other urological sarcoma arising from the bladder or paratesticular site . This isData on optimal treatment are obviously limited. The main treatment is surgery, which consisted of a cystoprostatectomy with a pelvic lymphadenectomy in all reported cases, except ours where vesiculo-prostatectomy was done without cystectomy and without any local relapse during the follow-up. As usually in STS, only complete resection of the tumour offers a chance for a cure. In the literature, the margins were positive in only 2 cases. In our case the margins were very close, less than 1 mm, justifying the use of adjuvant radiation therapy. The role of adjuvant radiation therapy in visceral STS is not established , and onlWe report the eighth case of primary leiomyosarcoma of the seminal vesicle with the longest follow-up. As these tumours are exceptional and information about standard treatment is lacking, we think that case reports such as this one may serve as the only reference for clinicians taking care of these patients. Radical surgical excision is the best chance for cure, but the need for cystectomy must be discussed. Furthermore, like leiomyosarcomas from other sites, a multimodality treatment deserves to be discussed and systemic therapy to be improved given the metastatic risk and overall poor prognosis. In this context, the gemcitabine-docetaxel combination is likely so efficient than in other locations.Written informed consent was obtained from the patient (February 2011) 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.STS: soft tissue sarcoma; US: ultrasonography; WHO: World Health Organisation; CT: computed tomography; MRI: magnetic resonance imaging; FNCLCC: Fédération Nationale des Centres de Lutte Contre le Cancer.The authors declare that they have no competing interests.Conception and design: CC, LMZ and FB. Manuscript writing: CC, LMZ and FB. Final approval: FB, CC, LMZ, BC, WH, YD, JJ, JG, AS. Pathological explorations: BC, YD, JJ. Patient's management: FB, LMZ, WH, AS, JG.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/11/323/prepub


Angiomyolipoma is a rare benign mesenchymal tumor, which is mostly renal in origin. A sixty-year-old male patient with the diagnosis of angiomyolipoma located in the larynx has been presented here, and the literature is reviewed. Angiomyolipoma is a rare benign mesenchymal tumor which is most frequently located in the kidney. Accompanying tuberous sclerosis syndrome is present in 50% of angiomyolipoma patients. Angiomyolipoma has been reported in 80% of tuberous sclerosis patients . The secA 60-year-old male patient presented to our clinic with the complaints of hoarseness persisting for one year. He had respiratory distress appearing in the last 3-4 months. His medical history was unremarkable except for history of smoking (1 package per day for 40 years). On his videolaryngoscopic examination, a 1.5 cm vascularized, pedunculated polypoid lesion, originating from the posterior half of the vocal cord and vocal process of the arytenoid, was observed. The mass was obstructing the endolarynx, subtotally . A trachThe surgical specimen was fixed in a 10% neutral buffered formaldehyde solution and embedded in paraffin. On routine hematoxylin and eosin stains, microscopic examination revealed a tumour composed of an intimate admixture of tortuous blood vessels, bundles of smooth muscle, and mature adipose tissue . Smooth Angiomyolipoma is a rare benign mesenchymal tumor composed of varying proportions of mature lipoid tissue, smooth muscle fibers, and vessels with thick walls. It is most commonly seen in women. The most common form of angiomyolipoma is renal angiomyolipoma. The lesion, which is asymptomatic and usually detected incidentally, is also seen in systemic diseases like tuberous sclerosis . While 5The most commonly known mesenchymal tumors of the larynx are lipomas, chondromas, vascular tumors, and paragangliomas. Schwannoma originates from the superior laryngeal nerve and may usually be encountered in a pedunculated form in the aryepiglottic plica or submucously. Chondromas may usually be located in the posterior lamina of the cricoid cartilage and cause a subglottic tumefaction , 11. In While laryngeal angiomyolipoma may be asymptomatic, symptoms may vary according to the size and location of the lesion . The mosEndoscopic preoperative histopathological diagnosis is difficult due to submucosal development of the tumor. Other tumors of adipose tissue  and angioleiomyoma should initially be taken into consideration in the microscopic differential diagnosis. Angiolipoma lacks myoid differentiation. Lipoid component does not exist in an angioleiomyoma. In order not to misdiagnose a liposarcoma, lipoid component must be searched carefully for lipoblasts. It has been reported that HMB-45 positivity in angiomyolipoma when located in the kidneys is related to tuberous sclerosis. In our case, the tumor did not take up the HMB-45 dye, as well as another melanocytic marker Melan-A –5. Melanocytic marker positivity is constitutional for angiomyolipomas. It was not observed in our case as well as the other reported cases. However, the presence of adipose and muscle tissue and tortoise vessels allows us to make the diagnosis of angiomyolipoma easily . HMB-45 In conclusion, we aimed to emphasize the importance of the histopathological diagnosis for differentiation from other laryngeal pathologies by presenting a laryngeal angiomyolipoma case rarely encountered in the literature.


Pulmonary metastases of benign meningiomas are extremely rare. The case of a 34-year-old man with bilateral parasagittal meningioma who developed pulmonary metastases is described. The meningioma was an enormous hypervascular tumor with invasion of the superior sagittal sinus. The tumor was resected completely and histologically diagnosed as transitional meningioma. The Ki-67 labeling index was 5%. Four months after operation, the patient subsequently developed bilateral multiple lung lesions later identified as metastases. The lung lesions were partially removed surgically and histologically diagnosed as meningothelial meningioma WHO grade I. The Ki-67 labeling index was 2%. The histological findings demonstrated that the tumor occupied the arterial lumen and the perivascular space, suggesting that pulmonary tumors might metastasize via the vascular route. The histopathological features and mechanisms of metastasizing meningiomas are reviewed and discussed. Meningioma is one of the most frequently encountered tumors of the central nervous system; it consists of meningoepithelial cells and generally originates from intracranial meninges. Meningiomas are considered benign lesions that are not locally invasive and are usually cured by surgical resection . AtypicaHowever, meningiomas have a potential to become more aggressive and invade the brain and/or calvaria or to metastasize outside the central nervous system. According to previous reports, most meningiomas with pulmonary metastases were histologically atypical (WHO grade II), anaplastic (WHO grade III) or initially benign meningiomas that changed to atypical or anaplastic tumors on recurrence , 3. MetaThere are no definitive criteria to predict local recurrence or metastases in meningiomas. Metastases outside the central nervous system are known to occur rarely even from meningiomas that at first diagnosis are considered grade I tumors.The patient case was a recurrent parasagittal meningioma that invaded the superior sagittal sinus and eventually metastasized to the lung. Histologically, the intracranial meningioma was transitional type, whereas the pulmonary meningiomas were meningothelial meningiomas. The proliferative potential of the intracranial and pulmonary meningiomas was evaluated immunohistochemically by Ki-67 labeling .In view of the slow-growing nature of these metastases and their good prognosis after resection, surgery is the treatment of choice .A 34-year-old man who had previously undergone craniotomy a total of three times in 2005 and 2006 presented with a progressive gait disturbance and was referred to our institution for further treatment. He presented with paraplegia involving bilateral lower extremities and a memory disturbance. Computed tomography (CT) revealed a huge isodense mass, approximately 8 cm in diameter, with strong perifocal edema involving the falx and superior sagittal sinus (SSS). The tumor invaded the calvarium and scalp, with compression of bilateral frontal lobes. Magnetic resonance imaging (MRI) showed the mass as isointensity on T1-weighted imaging and heterogeneously intense on T2-weighted imaging, with heterogeneous enhancement with gadolinium Figures . IntratuThe tumor was highly vascular, composed of elastic-hard, fibrous, grayish-white tumor and soft reddish components that invaded the calvarium. The tumor adhered tightly to the cerebral cortex with involvement of the falx and SSS, and there was arterial bleeding from the falx. Dense vascular networks were cauterized, the SSS was completely obliterated, and the tumor was dissected from the edematous cortex and then totally removed. Blood loss was 6500 mL. Postoperatively, the patient had paraparesis and was transferred for extensive rehabilitation. Postoperative MR images showed complete removal of the tumor without recurrence Figures .Two months after the last operation, X-rays and CT scans of the chest demonstrated well-circumscribed, multinodular tumors in bilateral lungs Figures . The rig The recurrent intracranial tumor showed spindle-shaped cells with atypical nuclei and high cellularity, together with marked collagen deposition and whorl formation . Lobular Histopathological examination showed spindle meningothelial cells arranged in fascicules with psammoma bodies infiltrating the lung . As showThese histological and immunohistochemical findings together with the clinical history of a previous intracranial meningioma were suggestive of metastatic meningioma WHO grade I.Meningiomas (WHO grade I) are usually noninvasive tumors and do not metastasize and, hence, are perceived as benign tumor . DistantIn the present case, a huge parasagittal meningioma invaded the superior sagittal sinus and metastasized to the lung following surgical removal. This recurrent tumor was accompanied with dense vasculature and occlusion of the superior sagittal sinus. As for histological malignancy, metastasizing meningiomas are usually histologically benign. Characteristically, meningiomas can be locally invasive of the dura, dural sinuses, adjacent bones, and soft tissues.There are no clear criteria to identify the subset of aggressive tumors that recur locally or metastasize. The literature suggests that previous craniotomy, venous sinus invasion, local recurrence, papillary morphology, and histological malignancy may be risk factors for systemic spread , 19. TheOn the other hand, others have described that such locally aggressive behavior is not necessarly indicative of malignancy, and it is usually associated with benign cellular features , 11, 20.The differential diagnosis of benign metastasizing meningioma with pulmonary meningioma or minute pulmonary meningothelial-like nodules is occasionally difficult. The histological features of primary pulmonary meningioma are round, sharply defined pulmonary nodules. The nuclei are round, oval, or fusiform and eccentrically located in an eosinophilic cytoplasm. Because the cell borders are often not recognizable, the tumor cells are arranged partially in sheets or whorls. Certain features are not recognized, such as the formation of psammoma bodies. There is no cellular pleomorphism or mitosis. These characteristics are the same as those of certain cranial meningiomas , 13. In Primary pulmonary meningioma usually presents as a solitary pulmonary nodule. Only one case of multiple pulmonary meningiomas has been reported. However, the largest tumor was 1.5 cm in diameter, and the tumors of this case did not involve any lung blood vessels . In the Metastatic meningiomas usually display histologic clues that help in addressing the diagnosis, such as their multicentricity and blood vessel involvement as a distance from the larger nodules , 19. MinThe other differential diagnoses of meningiomas include hemangiopericytoma and solitary fibrous tumor. Hemangiopericytomas have the classical staghorn pattern at low magnification and a distinctive turbulent pattern at higher magnifications , 30. SolBenign meningiomas commonly invade into the dural sinuses and thus have access to the vascular system. However, dural sinus invasion is not predictive of hematogenous dissemination. It is impossible to disregard the mechanical spread of cells during surgery or as a result of vascular wall invasion. On the other hand, different authors have drawn attention to the low incidence of metastasis associated with surgical management, as well as the frequent involvement of the venous sinuses in meningiomas, because some cases were not operated on before metastases were discovered , 11, 26.In the present case, there has been absolutely no recurrence and no metastases clinically and radiologically to date. In view of the slow-growing nature of metastatic meningiomas and their good prognosis after resection, surgery is the treatment of choice.


There are few case reports of thymoma with a thymic cyst. Such an association renders it difficult for any pathologist to differentiate from other neoplasms, such as a cystic thymoma.A 50-year-old Berber woman from Morocco was admitted with a chronic cough of more than 10 years duration. Her medical history and physical examination were normal. Anterior chest radiography demonstrated a calcified opacity in her right anterior mediastinum. A chest-computed tomogram revealed a round cystic tumor, with significant calcification in her right anterior mediastinum. A surgical exploration was performed. The tumor seemed to be a well-encapsulated and totally calcified lesion, arising from the right lobe of her thymus. It was removed by partial resection of her thymus. Through histology, the calcified tumor exhibited some areas of multilocular fibrous-wall cysts. These cysts were partially lined by small cuboidal cells with severe chronic inflammation and an AB thymoma that arose from the wall of the cyst.Greater attention should be given to multilocular thymic cysts, to exclude the possibility of neoplasm, especially when the cyst wall is thickened. Multilocular thymic cysts are uncommon lesions of the anterior mediastinum. On a regular histopathology examination, they show significant inflammation and fibrosis that can be associated with thymic neoplasm such as thymoma or thymic carcinoma. It is worth noting that an association between thymoma and multilocular thymic cysts has hardly ever been observed. However, the possibility of other cystic thymic lesions, essentially cystic degeneration of thymoma, must always be considered .We report an unusual AB thymoma case of a 50-year-old woman in the wall of a calcified multilocular thymic cyst. Our study suggests that difficulties related to the diagnosis have been noted, and we advise that special attention be given to every anterior mediastinal cystic lesion.A 50-year-old Moroccan Berber woman was admitted with a chronic cough that has been going on for more than 10 years, without history of smoking or neoplasm. On admission, her physical examination and routine biochemical tests were within normal limits. Chest radiography showed a round calcified opacity on her right anterior mediastinum. Computed tomography (CT) of her thorax revealed a round cystic tumor, with significant calcification, in her right anterior mediastinum  was well-encapsulated and focally showed multilocular thick-wall cysts Figure . HistoloBenign thymic cysts are uncommon lesions that account for approximately 3% of all anterior mediastinal masses. Frequently, they are asymptomatic and the actual identification of the tumor is usually made after surgery and histological examination .Such cysts can either be congenital or acquired in origin. Congenital cysts are typically unilocular. They contain clear fluid and have walls that are thin to the point of translucency; they show no evidence of inflammation on careful histopathological examination . By contMultilocular thymic cysts may be associated with thymic neoplasm such as thymoma or thymic carcinoma. They may adhere to adjacent structures and simulate an invasive neoplasm at thoracotomy ,6.Multilocular thymic cyst associated with thymoma or a malignant tumor is rare ,7. In suIn the case of thymic cyst with thymoma, the thymoma arises from the wall of a thymic cyst. This consists of a large round mass and small flattened spindle-shaped nodules ,9. BecauIn our case, the calcified pattern hinders the possibility to distinguish a multilocular cyst from cystic thymoma. However, good sampling of the specimen allowed us to see that the wall was lined with epithelial cells, and to therefore and make a diagnosis of multilocular thymic cyst associated with thymoma.For these lesions, total excision should be performed. This remains the curative treatment of choice, and histological examination is the only definitive means of diagnosis ,6. AlthoIn suspicious cases of multilocular thymic cysts, thorough sampling of every "thymic cyst" must be carried out. This is not only to establish an accurate diagnosis, but also to exclude the possibility of neoplasm, especially when the cyst wall is thickened.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.HE drafted the manuscript. FZ performed the case management, and drafted the manuscript. AJ participated in confirming the diagnosis. LL participated in the patient's management. ZB and NB corrected the manuscript. All authors read and approved the final manuscript.


Retro-rectal cystic hamartoma (tailgut cyst) is a rare congenital developmental lesion arising from post-natal primitive gut remnants in the retro-rectal space. The rarity of the lesion and its anatomical position usually leads to difficulty in diagnosis and surgical management. Complete surgical resection remains the cornerstone of treatment. A dozen or so surgical approaches have been described in the literature to date to make the operation as simple as possible, but the trans-rectal access route is extremely rarely reported and not well described. Here, we present a case that demonstrates the trans-rectal approach to a retro-rectal tumor is a feasible option in terms of surgical radicality, minimal invasiveness and safety for carefully selected patients with this rare type of retro-rectal cystic lesion.A 29-year-old Caucasian woman was referred to our institution due to perineal pain extending to the right inguinal region. Her symptoms had been present for the last two months. She was first examined at her regional hospital for a suspected ruptured ovarian cyst; however, after consultation with a gynecologist and a computed tomography scan of her pelvis, a tumor in the retro-rectal space was discovered. Our patient was admitted to our hospital and when a pelvic magnetic resonance imaging study confirmed the diagnosis of the retro-rectal cystic formation, a complete extirpation of retro-rectal tumor fixed to the coccyx using trans-rectal approach was performed. The final pathological diagnosis was retro-rectal cystic hamartoma (tailgut cyst) with no evidence of malignancy. Her post-operative course was uneventful, and at four months after surgery our patient is symptom free with no evidence of recurrent or residual disease.Trans-rectal excision is feasible in terms of surgical radicality and is a simple to perform, minimally invasive and safe option, providing complete recovery for carefully selected patients with retro-rectal cystic hamartoma treated operatively. Retro-rectal cystic hamartoma, also known as tailgut cyst, is an uncommon congenital developmental lesion arising from post-natal primitive gut remnants, generally located in the retro-rectal space ,2. The aA 29-year-old Caucasian woman was referred to our institution due to perineal pain extending to the right inguinal region. Her symptoms had been present for the last two months. Previously, she had been examined in a regional hospital, where a ruptured ovarian cyst was suspected. After gynecologist consultation and a pelvic CT scan, a tumor in her retro-rectal space was discovered. Our patient was admitted to our hospital for further examination and treatment. On digital rectal examination a lesion at the projection from four o’clock to eight o’clock in the lower rectum behind the rectal wall was palpated, with the lower boarder extending to as low as 2cm above the dentate line. A pelvic MRI scan detected a large cystic formation (7×5×4cm) in the retro-rectal space (slightly more to the right) Figure . The comThe retro-rectal  space is the virtual anatomic region bounded by the rectum  anteriorly, the sacrum  posteriorly, the peritoneal reflection  superiorly and the levator ani and coccygeus muscles  inferiorly -10. The Tumors of the retro-rectal  space in the adult population are uncommon and even rare . These tThere is a lack of information on retro-rectal cystic hamartoma natural history and biological behavior ; only liHalf of developmental cysts are diagnosed incidentally during examination for other conditions . DiagnosIt is postulated that the best treatment for retro-rectal tumors remains complete surgical excision of the retro-rectal mass (cyst) as mentioned above, which eliminates the problems of potential recurrence, hemorrhage, infection, compression and the possibility of malignant change ,6. HistoThe prognosis of surgical management depends on achieving complete surgical excision of the tailgut cyst with clear margins. Trans-rectal excision is feasible in term of surgical radicality and can be an easy to perform, minimally invasive and safe option, providing complete recovery for carefully selected patients with retro-rectal cystic hamartoma treated operationally.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.EK and NES examined and treated our patient, and analyzed and interpreted the data from our patient. Both authors read and approved the manuscript.


High-intensity focused ultrasound (HIFU) is playing an increasingly important role in cancer therapy. Primary synovial sarcomas of the chest wall are extremely rare. We report the first case of noninvasive HIFU therapy for the control of synovial sarcoma. A 51-year-old man was diagnosed with spindle cell sarcoma on the left chest wall through lumpectomy. After four cycles of chemotherapy, local recurrence of the sarcoma was detected. Subsequent extended resection confirmed synovial sarcoma. After five cycles of a new chemotherapy option, the sarcoma relapsed again. Then the patient received five courses of HIFU; this completely ablated the sarcoma without complications. No chemotherapy, radiotherapy, or biological therapy has been applied since. Now the patient is stable and has a high quality of life. Synovial sarcoma is a type of rare sarcoma in the soft tissue near the large joints of the arm or leg. Primary synovial sarcoma of the chest wall is extremely rare. The cytological features of the monophasic spindle cell and biphasic subtypes of synovial sarcoma, samples of which were obtained through fine needle aspiration, have been reported in several case studies . Surgery1/T2 990 ms/10 ms; 40 transmissions per therapeutic point with a distance of 2 mm between adjacent therapeutic points; treatment of each unit (five therapeutic points) for 200 seconds with an interval of 2 minutes between each unit; a spacing of 5 mm between adjacent treatment slices. The ablation effect was measured by MRI after the HIFU procedure.A solid texture mass, 2 cm in diameter, was found on the left chest wall of a 51-year-old man accompanied with local swelling and tenderness 1 year ago. Lumpectomy was performed in a local hospital. Postoperative pathological analysis confirmed the mass to be a spindle cell sarcoma. The patient was treated with a chemotherapy regimen of MTX 14.5 d1 + MTX 14.5 d8 + DDP 180 mg d15 + ADM 110 mg d17. After four cycles of chemotherapy, a similar mass with a more solid texture and some tenderness was found in the surgical site. Therefore, the patient was admitted to our hospital for a second surgery, expanded resection. Postoperative immunohistochemistry results indicated ER−, AR−, CK(Pan)±, CD34−, EMA−, CerbB2−, S-100−, Vimentin+++, EGFR−, P63−, SMA+, Calponin−, D2-40+, MBP−, CD99+++, β-catenin++, confirming diagnosis of synovial sarcoma. The patient received a new chemotherapy regimen of IFO 2 d1-5 + VP16 0.1 d1-5. However, follow-up magnetic resonance imaging (MRI) revealed that the tumor relapsed again after five cycles of chemotherapy. A new therapy plan was designed: HIFU. The HIFU procedure was performed using an FEP-BY02 HIFU system . A vertical scanning mode was chosen with a slice thickness of 2 mm. The ultrasonic transmitter worked at a frequency of 1.1 MHz and 11.0 MHz. GE LOGIQ 400CL was used for real-time monitoring during the therapy. The ultrasonic power was 130 W. Detailed therapeutic parameters were as follows: TAfter the first HIFU procedure, MRI demonstrated that the treated region was indicative of coagulation necrosis and homogeneous enhancement at the edge of the tumor. In total, five HIFU procedures were conducted, on 15 February 2011, 26 April 2011, 30 May 2011, 28 June 2011, and 6 September 2011. MRI conducted on 11 January 2011 and 7 September 2011 found complete coagulation necrosis in the therapeutic area (Figure Synovial sarcoma is characterized by local recurrence and difficulty in treatment. Surgery is the mainstream option for synovial sarcoma, accompanied by chemotherapy and radiotherapy. Further studies on molecular biology have revealed specific oncogene mutations and corresponding protection expression could serve as therapeutic targets . UnfortuThis is the first report of effective noninvasive therapy for local control of synovial sarcoma. Without doubt, the development of HIFU will bring revolutionary technologies for cancer therapy.Written informed consent was obtained from the patient’s relatives for publication of this case report and accompanying images. A copy is available for review from the editor-in-chief of this journal.H & E: Hematoxylin and eosin; HIFU: High-intensity focused ultrasound; MRI: Magnetic resonance imaging.The authors declare that they have no competing interests.XH and HC collected the data and drafted the manuscript. MZ performed data analysis. HH, YH, and WT recruited the patient and collected study materials. YD designed the study and revised the manuscript. All authors read and approved the final manuscript.


Primary extracranial meningiomas (PEMs) originating from the nasal septum are extremely rare, as are extracranial metastases of meningiomas.A 44-year-old male presented with a 2-month history of left-side nasal obstruction and frequent episodes of epistaxis. A friable mass originating from the nasal septum was resected completely via an endoscopic endonasal approach. According to WHO criteria, the tumor was diagnosed as an atypical meningioma radiologically and histopathologically. Two years later, a tumor recurred at the primary site with the same histopathological findings, and the patient was given local external radiotherapy (6840 cGy in 38 fractions). Two months after this local recurrence, a left anterior chest wall mass and a left parietal area scalp mass were observed. The subcutaneous mass was resected and showed histological evidence of malignant transformation. Several months after the last operation, the patient died.We describe the clinical, radiological, and bio-pathological features of this unique case and review the literature on atypical PEMs originating in the nasal septum. To our knowledge, this is the first reported case of an atypical PEM originating from the nasal septum that recurred with malignant transformation and extracranial metastasis. Meningiomas are relatively common entities, accounting for 13–18% of intracranial neoplasms in adults, but they are rarely seen in extracranial locations, such as the spine, skull convexities, neck, chest, shoulder, and peritoneum . MeningiAtypical meningioma is a rare variant that comprises 5-15% of all meningiomas. The reported rate of malignant change in meningiomas is less than 7% . AlthougA 44-year-old male was admitted to our clinic with a history of left-side nasal obstruction, episodes of epistaxis, hyposmia, and postnasal discharge over the previous 2 months. The patient had undergone endoscopic sinus surgery 2 years earlier for bilateral sinusitis at another clinic. Otherwise, his medical history was unremarkable. General and neurological examinations and routine laboratory test results were unremarkable. Non-contrast-enhanced computed tomography (CT) sections revealed an extensive hypodense non-calcified lesion occupying the left nasal cavity and pushing against the lateral nasal wall Figure A. The leMicroscopically, the tumor cells were arranged in sheet-like growths or were uninterrupted and patternless and showed nasal septum invasion Figure A. No celTwo years later, the patient was readmitted to the Hematology and Oncology Department of our hospital with recurrent severe epistaxis and general weakness, which had developed over the previous month. He also described breathing difficulties. Paranasal sinus CT showed recurrence of the left nasal cavity tumor, with palate and maxilla region involvement. Histological investigation of this second tumor showed morphological characteristics similar to the primary tumor Figure A. PositrTwo months after local recurrence, subcutaneous tumors were found located on the left anterior chest wall (1.5 × 1.2 cm) and left parietal region 0.5 × 0.4 cm). The tumors were removed under local anesthesia. Histological examination showed morphological characteristics similar to the primary tumor ; 5–15% of meningiomas are atypical (WHO Grade II); and 1–2% are anaplastic/malignant (WHO Grade III) [Atypical meningiomas are diagnosed when increased mitotic activity (defined as ≥4 mitoses/10 HPF) or three or more of the following features are encountered: increased cellularity, small cells with a high nucleus:cytoplasm ratio, prominent nucleoli, uninterrupted patternless or sheet-like growth, and necrosis .Malignant meningiomas (WHO Grade III) exhibit histological features of malignancy, including obviously malignant cytology  and/or a high mitotic index (≥20 mitoses/10 HPF) . MoreoveThe incidence of metastases in meningiomas is very low. Although surgical removal may increase the risk of iatrogenic metastases of histologically aggressive meningiomas , malignaWe presented the case of a middle-aged male with distant metastasis from a PEM of atypical histology originating from the nasal septum. Although classified histologically as atypical, the meningioma exhibited unusually aggressive behavior and transformed to a malignant meningioma after surgery. Additionally, it is known that meningiomas tend to become more histologically aggressive with each recurrence . In our Currently, complete surgical excision of PEMs is the treatment of choice and there is no need for adjuvant treatment . HoweverWritten informed consent was obtained from the patient’s relatives for publication of this case report and accompanying images. A copy is available for review from the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BJB conceptualized the case report and wrote the manuscript as a major contributor. JMS and KHL were involved in drafting the manuscript and revising it for intellectual content. JHL carried out the pathologic analysis. CKL was involved in collection of data. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/12/275/prepub


Abdominal wall metastasis either incisional, drain, or port is rather rare in patients treated for cervical carcinoma. We present a case of a patient who underwent an abdominal radical hysterectomy for a moderately differentiated cervical adenocarcinoma stage Ib1 and presented an incisional site metastasis 36 months after her operation. Moreover, we performed a literature search for abdominal wall metastases after radical hysterectomy for cervical cancer, and we present a table of the relative case reports. After our literature search, we clarified that the median time of recurrence was 14 months (range 1.5 month to 45 months). Thirty-three out of 42, 8/42, and 1/42 were squamous, adeno-, and adenosquamous carcinomas, respectively. Wide excision was performed in 30/37 cases of which we have information regarding the treatment option, while 11/37 and 13/37 underwent radiotherapy and chemotherapy, respectively. The possible mechanism of such a metastasis as well as the treatment options is discussed. Common extrapelvic metastases of cervical carcinoma are mainly lymphatic, lungs, liver, and bone. Rarely 0.1–1.3%), metastatic recurrence of squamous cervical carcinoma could be identified in the abdominal wall especially in sites of incision –1.3%, me. Based oWe present a case of an incisional site metastasis three years after radical hysterectomy for cervical cancer, as well as a review of the current literature in the field. For this reason, a relevant search with the terms cervical cancer, metastasis, abdominal wall, drain-site, port-site, adenocarcinoma, and/or squamous cell has been performed in Pubmed.A 46-year-old morbidly obese patient underwent an abdominal radical hysterectomy for a moderately differentiated cervical adenocarcinoma stage Ib1 on 2007. No adjuvant radiotherapy was necessary at that moment. Two years later, she presented with incisional hernia, for which she underwent a repair. During this operation, her bowel was injured, and the colonic perforation led to faecal peritonitis. For this reason, Hartman's procedure was performed as an emergency and prolonged ITU admission was essential. Three years after her first operation, the patient presented to our clinic with a recurrence of adenocarcinoma in the anterior abdominal wall at the site of the hernia and intimately related to the stoma measuring 7 cm. The C/T-guided biopsy revealed cores of fibrofatty connective tissue with stromal desmoplasia and admixed sheets of foam macrophages. Focally, the cores showed a lining of glandular epithelium with marked nuclear pleomorphism and multiple mitoses consistent with metastatic adenocarcinoma. As the patient was cisplatin naïve, she completed six cycles of chemotherapy (cisplatin and topotecan). For 6 months, she had a good response to palliative treatment as there has been marked reduction in size down to 4.3 cm. That was the time of a new relapse and for this reason, a wide excision of the recurrence in the anterior abdominal wall as well as reverse of the stoma was performed, followed by use of a synthetic mesh to cover the defect. An 8 cm tumor was found in the left lower part of the rectus abdominis muscles. It was infiltrating the sheath but not involving any intra-abdominal structures. Histology identified adenocarcinoma identical to her primary cervical cancer.Incisional site metastasis is an extremely rare complication of cervical carcinoma especially of the squamous type. To clarify the rarity of such metastases, Zivanovic et al. showed that two cases out of 1694 patients with a malignant gynaecological condition operated laparoscopically had a port-site metastasis compared with 15 patients of ovarian cancer in the same series . More spThe actual incidence of incisional site metastasis might be higher than identified in this paper 31 Cases) caused by the lack on reporting them. It should be notified that 19 cases were identified after laparotomies, 21 cases after laparoscopic procedures, while only two cases were found in a robot-assisted radical hysterectomy , 30, as  Cases caAfter our literature search, we clarified that the median time of recurrence was 14 months (range 1.5 month to 45 months). Thirty-three out of 42, 8/42, and 1/42 were squamous, adeno-, and adenosquamous carcinomas, respectively. Wide excision was performed in 30/37 cases of which we have information regarding the treatment option, while 11/37 and 13/37 underwent radiotherapy and chemotherapy, respectively.Our results could be compared to a case series where 12 patients were identified with port-site metastasis after laparoscopy for cervical cancer with a median age of 44 years (range 31–74 years). Eighty per cent of them were squamous carcinomas, and 67% were diagnosed in an early stage. The median time for diagnosis was 5 months (1.5–19 months). Regarding their management, 78% of the tumors were excised, chemotherapy alone was used in 3/12 patients, combination of chemoradiation in 3/12, while radiotherapy in 1/12 patients .The potential risk factors of such metastases could be divided into four groups : the firBiopsy is necessary to prove the origin of the metastasis. Moreover, immunohistochemical findings such as strong CD31 positivity could help to identify the relativity of the metastasis to the primary lesion . 2 along trocars) caused by pneumoperitoneum, meaning that cells dislodged at the time of cervical manipulation may pass through the fallopian tubes and implant in the port sites [It should be noticed that it is very difficult to compare laparotomy to laparoscopy or robotic technique regarding incisional metastases. There is strong heterogeneity of the findings. Regarding the possible mechanism of metastasis to the abdominal wall, several theories have been raised. Kadar believed that dissemination results from the enhancement of tumor growth characteristic of early healing tissues and so metastasis could be prevented by appropriate postoperative treatment . Cell dirt sites . In openIn our case, during the second operation for hernia repair, there was no intraperitoneal disease. Colonic perforation as well as the following peritonitis could be the first trigger of this sequence of events causing spillage of tumor cells.Careful and close followup including examination and imaging with special attention to incisional, port, or drain sites is proposed to early identify such a recurrence. Treatment of such recurrences remains palliative and includes chemotherapy, radiotherapy, or wide surgical excision. For example, reconstruction of the abdominal wall with a latissimus dorsi musculocutaneous flap as well as mesh use are proposed in the literature . Platin-It is difficult to clarify the prognosis of such a recurrence as patients could die because of their extensive disease. Prognosis in such cases is usually poor because of the systematic contamination of the disease,; however, survival could reach even 4.5 years . RamirezAlthough, some could declare that incisional site metastasis and abdominal wall metastasis should not be confused, the possible mechanism of metastasis might be relatively similar. For this reason, we added possible hypotheses—pathways of such rare metastases. Abdominal metastasis after radical surgery for squamous cervical carcinoma is a rare entity; however followup and treatment should be further clarified. A multicenter analysis is proposed in order to clarify the presentation and management of such rare entities.


Teratoma is a tumor that usually arises from one or more germ layers. They are most commonly found in the sacrococcygeal region and have a female preponderance. We present a very rare case of a boy with a benign cystic teratoma in the lumbosacral region.A 16-year-old Indian boy presented to our hospital with a history of a lump in the lower back region since birth. Initially, it was small, but its size increased gradually over time to a size of 15 cm × 15 cm at presentation. There were no other associated abnormalities. Investigations revealed the lump to be a benign cystic teratoma. The patient underwent surgery, and the whole tumor, from its base to the vertebrae, was excised. Bisection of the tumor revealed that it contained hair and pultaceous material consistent with a teratoma, which was later confirmed by histopathologic examination.Benign cystic teratomas should be diagnosed and managed aggressively because they generally have a greater tendency to progress toward malignancy. After extensively searching the case report database, we arrived at the conclusion that this was a rare case of a benign cystic teratoma in the lumbosacral region in a boy. Teratomas are germ cell tumors primarily composed of multiple types of cells derived from one or more of the three germ layers . The terA 16-year-old Indian boy was brought to our hospital with swelling in the midline lower back that had been present since birth (Figure The initial examination revealed a cystic, non-mobile, non-tender mass approximately 15 cm × 15 cm in size attached to the back in the midline in the lumbosacral region. However, the patient's blood counts, urine analysis, and liver function test results were normal. Furthermore, the radiographs of the lumbosacral region showed a well-defined swelling 15 cm × 20 cm in size with a smooth margin from the L3 vertebra to the S3 vertebra (Figure The tumor was excised by creating an elliptical incision over the cyst. A whitish yellow, well-encapsulated, non-mobile mass was observed. The tumor was carefully dissected to allow us to reach the base, which was found to be attached to the L5 lumbar vertebra. The attachments, along with a small piece of lumbar vertebra, were also removed to minimize the chance of recurrence.A teratoma is an encapsulated tumor with components resembling normal derivatives of all three germ layers . TeratomThe other differential diagnoses considered in this case were lumbosacral lipomeningomyelocele, congenital lipoma, and sacrococcygeal teratoma. Lipomeningomyeloceles commonly occur in the lumbosacral area, but the MRI examination of our patient revealed no communication with the spinal cord, so this possibility was ruled out . Similarin utero [The diagnosis of a teratoma is based mainly on histopathologic examination, although MRI is also helpful in determining its connection with the vertebral column or its extension into the spinal cord. Prenatally, teratomas are usually diagnosed on the basis of obstetric ultrasonography in utero . They apin utero . In thisEvidence indicates that if the base is not excised along with its attachment to underlying bone, a teratoma may recur because it might contain totipotent cells. Therefore, complete excision is imperative ,13. HoweThe site of the teratoma in our patient was the L5 vertebra, which is extremely rare -17. A stTeratomas are usually benign but sometimes may occur as malignant tumors. To avoid any diagnostic dilemma, it is significant to understand the rare presentation with regard to the tumor site and the possibility of malignancy. The case history and the very rare site of the tumor described in this report will help clinicians in diagnosing such cases and will help in enhancing clinical knowledge and experience for better treatment and patient care.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.MF was a major contributor to the writing of the manuscript. HHS analyzed and interpreted the patient data. VM and RK performed the histologic examination. DK and AS helped in the writing of the manuscript. All authors read and approved the final manuscript.


Liver cavernous hemangioma is the most common noncystic hepatic lesion, and a hemangioma that undergoes degeneration and fibrous replacement is called a hepatic sclerosed hemangioma.A 63-year-old Japanese man was admitted for detailed investigation of a liver tumor. Tumor markers carcinoembryonic antigen, alpha-fetoprotein, and CA19-9 levels in the peripheral blood were not elevated at any time. Plain computed tomography showed an approximately 1.5cm low density mass in the periphery of segment 8, which was marginally enhanced on contrast-enhanced dynamic computed tomography. On magnetic resonance imaging, the tumor was hypointense on T1-weighted image and hyperintense on T2-weighted image. The tumor was suspected to be an atypical hemangioma, metastatic, hepatocellular carcinoma, or cholangiocellular carcinoma. Segmental hepatectomy was performed. Histological examination of the resected tumor specimen revealed a sclerosed hemangioma with marked hyalinization and sparse stromal fibrosis. Immunochemically, the tumor cells were positive for CD34 and alpha smooth muscle actin. Electron microscopically, the residual hemangioma consisted of numerous caveolae and vesicles in endothelial cells in irregular shapes and sizes. Immunostaining for caveolin-1 showed decreased or no caveolin-1 reactivity in the hyalinized lesions of the sclerosed hemangioma, but abundant caveolin-1 reactivity in the residual cavernous hemangioma. Of interest, computed tomography images of the tumor obtained 10 years earlier at our hospital depicted a 3cm typical cavernous hemangioma.Hepatic sclerosed hemangioma is a rare condition. Comparison of radiological findings of the lesion over a period of 10 years was valuable in providing insight for the evolutional process from liver cavernous hemangioma to hepatic sclerosed hemangioma. Liver cavernous hemangioma (LCH) is the most common noncystic hepatic lesion, with a reported incidence of 20% of benign hepatic tumors . Most heComputed tomography (CT) features suggestive of HSH include geographic outline, capsular retraction, decrease in size over time, and loss of previously observed regions of enhancement. Additional features include the presence of transient hepatic attenuation difference, rim enhancement, and nodular regions of intense enhancement as seen in typical hemangiomas . The comCaveolae are cell plasma membrane microdomains and are responsible for transmembrane trafficking, endocytosis and lipid homeostasis, and also serve in signaling processes as a compartment where receptors and signaling proteins are concentrated . CaveoliA 63-year-old Japanese man was transferred to our hospital for detailed investigation of a liver tumor. At admission to our hospital, the hematologic and blood chemistry data were as follows: white blood cell count 3,800/μL, hemoglobin 15.9g/dL, platelet count 146,000/μL, blood urea nitrogen 17.9mg/dL, creatinine 0.82mg/dL, albumin 4.3g/dL, aspartate aminotransferase 26IU/L, alanine aminotransferase 33IU/L, and total bilirubin 0.9mg/dL. Serum hepatitis B (HB) surface antigen, anti-HBe antibody, and anti-hepatitis C virus antibody were negative. Alpha-fetoprotein, carcinoembryonic antigen, and cancer antigen 19–9 levels were within the normal ranges. Dynamic CT depicted a mass measuring 1.5 × 1.4cm with a bulging contour in segment 8 of the liver, which was enhanced in the arterial phase and showed subtle low density with focal capsular enhancement in the delayed phase -enhanced MRI study showed an internal heterogeneous mass. T2-weighted MRI showed the mass as hypointense in relation to cerebrospinal fluid. Although the final diagnosis of HSH was made histopathologically, understanding of its radiologic appearance is important to avoid unnecessary surgery, and HSH should be included in the differential diagnoses of a hepatic lesion with delayed enhancement . In the et al.  reportedElectron microscopy showed a hyalinized lesion, with cells resembling histiocytes and fibroblasts. In a previous study, the number of mast cells correlated significantly with vascular proliferation and correlated inversely with the degree of fibrosis . Gross aImmunohistochemical, electron microscopic, and various molecular pathological techniques have been utilized to diagnose pulmonary sclerosing hemangioma . The cauHSH is a rare condition. Comparison of radiological findings of the lesion over a period of 10 years was valuable in providing insight for the evolutional process from cavernous hemangioma to sclerosed hemangioma.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.YT performed the surgery. YS and MW collected the references and contributed to the writing. HI and HT reviewed CT and MRI. HYa interpreted the liver histology and contributed to the writing. HYo and YS wrote the paper. All authors have read and approved the final manuscript.Supplemental data.Click here for file


In children, benign neoplasms of extrahepatic biliary ducts are extremely rare. We report a case of 3 year old girl who presented with abdominal pain and jaundice for 6 months. The final diagnosis on histopathology was papillomatosis in lower common bile duct. Benign neoplasms of extrahepatic biliary ducts are infrequent in children. Their importance lies in their ability to mimic malignant lesions in such locations. Commonest presenting symptoms are pain, jaundice and acute cholangitis. Diagnosis of these cases predominantly is intraoperative.[1-3] This case highlights the above observations during management of the index patient.A 3-year-old female child presented with the complaints of pain in upper abdomen along with jaundice and fever for last six months. Abdominal examination was unremarkable. With the clinical possibility of a choledochal cyst, ultrasonography (USG) along with liver functions and routine haematological investigations were advised. USG showed impacted worm like appearance in the lower common bile duct with proximal dilatation . In view of deepening jaundice child was advised endoscopic retrograde cholangiopancreatography (ERCP) to clear the worm load in lower end of common bile duct (CBD) and to place the stent for establishing the bile flow. Endoscopic sphincterotomy with placement of 10 Fr stent was done but worm could not be cleared. Child also received antihelminthic drugs. Following stent placement child recovered from jaundice within two weeks. As there was a doubt of persistent worm in the distal CBD, exploration was planned and after obtaining informed written consent, child was explored under general anaesthesia. Through right upper transverse incision abdomen was explored. CBD was traced using stent as a guide. On opening the CBD, multiple polyps were found with stent in situ . No stone or worm was found. In view of possible malignancy a frozen section was sent and when confirmed to be benign, polyps were removed completely and CBD repaired over a T-tube. Stent was also removed. Postoperatively T-tube was removed after 3 weeks. Postoperatively child recovered uneventfully and presently she is in follow up and doing well. Histopathology of the resected specimen was reported as papillomatosis of the CBD.Benign tumors and tumor like lesions from the gallbladder and bile duct have a wide spectrum and despite this diversity these lesions share common embryologic origins and histologic characteristics. Benign neoplasms of epithelial origin are adenomas, cystadenomas and the rarely multiple polyps may be found as in present case of biliary papillomatosis. Granular cell tumors, neurofibromas, ganglioneuromas, paragangliomas and leiomyomas are examples of benign tumors that may originate from nonepithelial structures.Biliary papillomatosis is rare with only 140 cases reported in literature and that too in adults.[1] Chappet in 1894 reported the first case of biliary papillomatosis.[2] The malignant potential of benign polypoid lesions of the extrahepatic biliary system is controversial and not fully understood. The current literature supports the notion of malignant transformation of these benign epithelial lesions.[3-5] Reported male: female ratio is 2:1 with peak incidence in 4th decade of life. Commonest presenting symptoms are pain, jaundice and acute cholangitis as in our case. Diagnosis of these cases predominantly is intraoperative with few cases being suspected on ultrasound.[7] In present case it was discovered after CBD exploration. To conclude, although rare, biliary papillomatosis should be kept in mind when examining a patient with obstructive jaundice, acute cholangitis and abdominal pain in children.Source of Support: NilConflict of Interest: None declared