Multifocal cerebral gliomas (MCGs) represent approximately 10% of gliomas and are frequently mistaken as metastases of an unknown primary cancer site. Most MCGs are glioblastomas with <4 lesions supratentorially, and are lack of typical symptoms and special detections.Through a rare MCG case, we aim to present this rarity and emphasize the need to correctly diagnose multiple intracranial lesions using a variety of diagnostic modalities to ensure that the patient receives proper treatment.We present a case of multifocal cerebral anaplastic astrocytomas with a total of 8 lesions located in the left frontal lobe and invading the lateral ventricle, presenting with dysphasia and phantosmia. The disease course, including diagnosis and treatment, is presented and analyzed in detail. The pertinent literature is reviewed regarding this uncommon entity.After an initial impression of brain metastasis from lung cancer because of the magnetic resonance imaging (MRI) resemblance and history of chronic bronchitis, we were able to use positron emission tomography (PET) and excisional biopsy to get the final diagnosis. After 10 months, the patient's overall condition deteriorated and succumbed to his disease.MCGs are easy to be misdiagnosed as metastatic diseases. In addition to MRI, PET adds more biochemical and molecular information and is helpful in the differentiation. Although uncommon, if multiple lesions are present in various locations in the hemispheres, MCG should be kept in mind. When the MCGs are resectable, surgery is recommended followed by radiotherapy and chemotherapy.5To be precise, MCGs could be classified into multicentric gliomas when there is no macroscopic or microscopic connection between the multiple brain tumors, and multifocal when there is evidence of microscopic connection or spread from a primary site. MCG can present with a clinical and radiological picture similar to that of metastatic disease, especially if there is a history of other cancers or clinical manifestation of other systems. Correct diagnosis can hardly be made without the help of advanced imaging technology and biopsy pathological examination. MCGs may either exhibit the same or different pathological patterns, among which, multiform glioblastomas are the most common.Herein, we present a case of a 59-year-old woman suffering from multifocal CAA with a total of 8 lesions in the left frontal lobe and invading the lateral ventricle, presenting with dysphasia and phantosmia. The location and pathotype of the lesions and the patient's clinical features in this case are all unique. To the best of our knowledge, this is the first reported case of MCG presenting in this manner. Because the rarity of MCG often leads to misdiagnosis of intracranial metastatic tumors, abscesses, lymphoma, and demyelination, we also reviewed the latest literature on the imaging, diagnosis, and treatment of MCG.A 59 year-old Chinese female complained of olfactory hallucination for 1 month. She said she could sometimes perceive a smell of “baked sweet potato” or something like “a just died diplopod” in the complete absence of any physical odors. Moreover, her husband found she usually could not talk with him as fluently as before. She herself admitted having difficulty remembering words and memory deterioration. About 10 days after the onset, she complained of headache and vertigo (for 3 weeks before admission). She had no symptoms of unconsciousness, convulsion, epilepsy, or cognitive disorder and was not taking any medications. She noticed a slight weight loss despite a normal appetite. Except for a past medical history of chronic bronchitis, the patient was previously healthy. We did not identify any special circumstances regarding her family history and personal history related to her presentation. Upon neurological examination, the patient was in spirit. She could not remain focused on a simple task such as counting from 1 to 15. When asked about historical or verifiable personal events, her answers were incoherent and inconsistent and with lots of paraphasic errors. When asked to do manual or written alternating sequence tasks, her movements were uncoordinated. Her bilateral pupils were round, equal in size, and constricted visibly to light. Her muscle strength was 3/5 to 4/5 in the right extremities. Sensory function was normal. No apparent changes in superficial and deep tendon reflexes were observed. Babinski sign and Oppenheim sign at the right side were positive. Bilateral Hoffmann signs were positive. Pathological signs were absent.6/L, WBC was 0, protein content was 560 mg/L. All the blood investigations were within normal limits. T/B cell subsets test: T 81.3%, T4 40.3%, T8 32.1%, T4/T8 1.26, B 10.2%.Magnetic resonance imaging (MRI) of her head: bilateral cerebral hemispheres were symmetrical; multifocal spotted and patchy long T1 and long T2 signals were present in the left frontal lobe involving the falx, the centrum semiovale and corona radiata with decreased gray–white matter differentiation; fissures, sulci, and gyri of the corresponding brain area were not clear; the left lateral ventricle was also invaded by a much larger lesion with irregular tumor margins; T1-weighted image (T1WI) plus Gd-DTPA (gadolinium-diethylenetriamine pentaacetic acid) enhancement showed spotty circular-like or irregular rings of inhomogeneous enhancement and some had no intensification; there was light edema round the lesions, mild space occupying effect : multifocal irregular lesions with inhomogeneous high 18F-fluorodeoxyglucose (18F-FDG) uptake were detected in left frontal lobe near centerline. Some adjacent areas were also highly 18F-FDG avid Figure   . In otThen, we performed a craniotomy and tumor resection. The lesions were in white matter, with a soft, lobulated, light gray-pink appearance. Its texture was not homogeneous with some cystic changes. Fast frozen pathology of resected specimen during operation reported astrocytoma . The paraffin histological examination confirmed the diagnosis as CAA. Immunohistochemical staining was positive for epidermal growth factor receptor, glial fibrillary acidic protein, Nestin, P53, S-100, CD34 , the patient's overall condition deteriorated about 10 months later secondary to the progression of her disease. She refused further treatment options and succumbed to her disease shortly thereafter. Autopsy was not performed.6 MCGs are a well-recognized but relatively uncommon entity, with a reported incidence of approximately 2 to 5% of total high-grade gliomas. There is a wide range in age at presentation of multiple gliomas, but the majority of patients are of middle–old age. No significant differences between sexes have been found. MCGs are often designated as multicentric or multifocal lesions. Multicentric gliomas are tumors arising independently in >1 site of the brain with absence of seeding along easily accessible routes. Multifocal gliomas are the result of dissemination of glioma cells from a primary focus to other parenchymal areas via the CSF, meninges or white matter tracts. According to the classifiation of Budka, multifocal gliomas are further grouped into 4 categories: diffuse, multiple, multicentric, and multiple-organ. MCGs can also be separated by time of occurrence. Present case is an example of multifocal CAA present at the same time.Described by Gower in 1896 for the first time,TP53 mutation, BRCA-1 mutation, and deletion of chromosome 1p36, whereas TP53 mutation and PDGFR (platelet-derived growth factor receptor) overexpression represent early changes during low-grade glioma development, anaplastic progression is associated with pRB alteration and loss of heterozygosity (LOH) of 19q, further malignant progression to glioblastoma multiform including LOH 10q and mutations of PTEN gene. In a patient with multiple CAA, hereditary colorectal cancer, transcobalamin II deficiency, agenesis of the corpus callosum and mental retardation, a germline mutation of the PMS2 gene was found. Furthermore, families with MCGs but without obvious connection to known tumor syndromes were described in several case reports and epidemiological studies.The pathogenesis of multiple gliomas remains unknown. Many pathogenetic theories have been suggested to explain multiplicity. Zulch stated that multiple lesions are metastases from a primary focus via CSF or the white matter tracts. In the second theory, MCGs may arise from cells that, although not neoplastic in themselves, are nevertheless “primed” by an acquired or inherited genetic defect and scattered throughout the nervous system during development. The most recognized was Willis’ “ 2-step process” theory. In the initiation step, a large area of brain undergoes neoplastic transformation, thus more susceptible to neoplastic growth. In the promotion step, multiple areas of malignant transformation occur following various kinds of stimulation , giving rise to multifocal glioma. A few genetic changes have been reported in MCGs, including MCGs may either exhibit the same or different histologies. Although glioblastomas are the most frequent pathotype, more benign glial neoplasms including astrocytoma and ependymoma have also been reported as multicentric. In the 51 cases reported by Kyritsis, there were 31 cases of glioblastomas followed by anaplastic gliomas (19 cases) and low-grade gliomas (1 case). In a case report, a 64-year-old female presented with a left frontal glioblastoma with astrocytic and neuronal differentiation and a temporal low-grade astrocytoma. In another case, a 43-year-old man presented with an oligodendroglioma and a JPA synchronously. A molecular analysis detected a deletion of chromosome 1p36 in the oligodendroglioma, but not in the JPA. A glioblastoma and a CAA plus a third unbiopsied glioma were reported in another patient. Other very rare cases of multifocality in low-grade gliomas have been reported in pleomorphic xanthoastrocytoma which has a favorable outcome with complete surgical resection. The histopathological features of multiple CAA include increased cellularity, distinct nuclear atypia and marked mitotic activity. Additional signs of anaplasia are nuclear inclusions, multinucleated cells, and abnormal mitoses. In our case also the histopathology was suggestive of CAA (WHO III). As a note of caution, however, histologic diagnosis and grading of astrocytomas is a somewhat subjective process.The tumor locations are not fixed. In a retrospective study, supratentorial localization prevailed, but no hemispheric predilection was identified. According to Kyritsis, the parietal lobe was the most common sites (37%) followed by frontal lobe (28%) and temporal lobe (22%). Only a few cases were reported in thalamus, brain stem, occipital lobe or even spinal cord. Mostly, the number of tumors in an individual patient is not >4. According to a retrospective survey, among all MCG patients, 92% had 2 to 3 lesions and only <8% had 4 lesions. In our case, a total of 8 lesions presented as synchronous primary brain tumors in the same patient, which is very uncommon.The clinical manifestations of are variable and non-characteristic, in relation to the extent of the lesions, including neurological focal signs (72%), epilepsy (40%), and symptoms of intracranial hypertension (68%) according to the above retrospective study. In our case, phantosmia and hypomnesis was 1 of the main complaints. Considering the lesions’ location, we speculate that the rhinencephalon (olfactory system) and part of the limbic system structures responsible for memory, were compressed and injured. This patient also presented with language impairment, which may be associated with lesions at dominant hemisphere.In general, iso-to hypodense lesions are seen on CT with a more or less diffuse mass effect. On MRI, as in present case, MCGs are generally hypo- to isointense on T1WIs, hyperintense on T2-weighted and FLAIR images, and enhances strongly after contrast administration in a heterogeneous or ring-like manner. Meningeal or ventricular enhancement, suggestive of a possible way of dissemination, can be observed sometimes. Presence of mass effect, edema, and large tumor size will differentiation between the low and high grade, whereas the presence of necrosis or cyst with ring-enhancement are characteristics for glioblastomas. In general, MCGs usually involve white matter with indistinct tumor margins, inhomogeneous tumor enhancement, and mild edema, whereas brain metastases are often located near white–gray matter border with regular or variable borders, ring-shaped homogeneous enhancement, and obvious edema. However, considering the radiographic features are nonspecific, it is still difficult to make an accurate differentiation neuroradiologically. In contrast to MRI, clinical and experimental use of PET is expanding and allows quantitative assessment of brain tumors’ pathophysiology and biochemistry including glucose uptake, amino acid transport, protein/DNA synthesis, cell proliferation, angiogenesis, and oxygen tension. PET therefore provides different biochemical and molecular information about brain tumors when compared to histological methods or neuroradiological studies and is very helpful in the detection of MCGs detection, grading, differentiation, prognosis prediction, and treatment follow-up. In our case, the brain tumors were highly FDG avid, whereas no obvious hypermetabolism areas were detected elsewhere. Especially, as PET represents a novel technology for molecular imaging assays of metabolism and signal transduction to gene expression, reporter gene assays are used to trace the location and temporal level of expression of therapeutic and endogenous genes. Some differential points among MCGs, CAA, and brain metastasis are listed in Table 5 Although whole-brain radiotherapy is preferable, 3D conformal radiotherapy may be applied in order to avoid toxicity.13 The chemotherapy schedule of MCG is similar to common single glioma. Although the prognosis is associated with pathological types and molecular markers such as MGMT promoter methylation, IDH1 mutation, 1p/19q co-deletion,11 generally speaking, it remains unfavorable and most patients die within the first year after diagnosis as it is in our case.A definite diagnosis of MCG should be confirmed by histopathological examination. So surgical intervention and tumor decompression not only have a significant impact on longer and better survival but also facilitate histopathological analysis. Moreover, adjuvant treatment may be more useful when the tumor bulk is already reduced. This statement stems from the fact that an aggressive surgical procedure for malignant gliomas with the resection of at least 90% of the tumor burden can result in increased survival duration for selected groups of patients. However, some other authors recommended biopsy as the fist step, believing that extensive resection increases the risk of hemorrhage and neurological deficits without influencing survival. Stereotactic biopsy has become a powerful and safe tool for providing tissue samples for diagnosis with minimal disruption of normal brain function, and plays a vital role in the management especially in cases where the lesions are located in sites inaccessible to surgery. In our case, we performed open surgical decompression of the larger lesions to improve the neurological deficits and also to get tissue for histological diagnosis. Apart from surgery, chemotherapy or radiation therapy may be considered.We report an uncommon case of multifocal CAA with a total of 8 lesions in the left frontal lobe and invading lateral ventricle, suffering from uncommon symptoms of dysphasia and phantosmia. MCGs are rare entities with the pathogenesis still unknown. Glioblastomas are the most frequent pathotype, but more benign ones such as the present case can also occur. MCGs are easy to be misdiagnosed as metastatic diseases according to radiological findings as it is in our case. In line with MRI, PET adds more biochemical and molecular information and is helpful in the differentiation. If multiple lesions are present in various locations in the hemispheres, MCG should be kept in mind. In such cases, advanced imageological examination or a biopsy is recommended for its differential diagnosis.12.


PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction.Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring.Large perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment. Infantile hemangiomas are sometimes complicated and associated with systemic malformations. PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag . Eleven We report the 2 years follow-up results of cloacal reconstruction in a girl with PELVIS syndrome. The manuscript was performed with the approval of the Bioethics Committee of the Childrens Hospital, affiliate Vilnius University Hospital Santariskiu clinics and is in compliance with the Helsinki Declaration.Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left tight Fig. . ColostoDue to the extreme rarity there is no treatment algorithm of this syndrome. Every individual case dictates timing and the choice of therapy for the best long term outcome. Congenital anomalies of a urogenital tract, like persistent cloaca, are by itself associated with a great chance of poor functional outcome even after successful surgical procedure. The association of a disfiguring haemangioma in the area of reconstruction gives first impression of poorer prognosis.The recent discovery of propranolol effectiveness and the improved employment of Laser technologies may improve the perspective for these children.et al. reported the antiproliferative effect of propranolol on infantile hemangiomas on June 2008 [p < 0.001) [Léauté-Labrèze < 0.001) . PropranLaser therapy for proliferating hemangiomas is controversial. One study have demonstrated good results with either the 585 nm or 595 mm pulsed-dye laser on superficial but not deep infantile hemangiomas . Other sWith our choice of Nd:YAG laser 1064 nm ice cube method and pulsed dye laser therapy 595 nm we didn’t observe complications and the growth of hemangiomas has stopped and regression induced. Combined treatment-systemic therapy and multiple laser applications seems to be the most effective treatment.Reconstructive operation mobilizing rectum and urogenital sinus and performing genitoplasty by posterior sagital approach in this case requires extensive dissection and mobilization of delicate structures what could be harassed by bleeding . That iswe support the current evidence of common association between large perineal hemangiomas and extracutaneous anomalies. Delaying reconstructive surgery until the safe reduction of hemangiomas by complex treatment does not seem to worsen long term functional and cosmetic results.Written informed consent was obtained from the parent of the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Introduction. Uterine leiomyoma is the most common benign pathology in women and lipoleiomyoma is an extremely rare and specific type of leiomyoma. Here, we report an unusual case of giant pedunculated subserous lipoleiomyoma misdiagnosed preoperatively as leiomyosarcoma. Case. A 45-year-old woman admitted to our gynecology outpatient clinic for complaints of abdominal distention, tiredness, and pelvic pain for the last 6 months. Sonography and abdominal magnetic resonance imaging (MRI) showed a giant semisolid mass that filled whole abdominal cavity from pelvis to subdiaphragmatic area. A primary diagnosis of uterine sarcoma or ovarian malignancy was made. On operation, total abdominal hysterectomy with a pedunculated mass of size 30 × 23 × 12 cm and weighing 5.4 kg and bilateral salpingo-oophorectomy were performed. The histopathology revealed a lipoleiomyoma with extensive cystic and fatty degeneration without any malignancy. Discussion. The diagnosis of leiomyoma is done usually with pelvic ultrasound but sometimes it is difficult to reach a correct diagnosis especially in cases of giant and pedunculated lipoleiomyoma that included fatty tissue which may mimick malignancy. Conclusion. Subserous pedunculated giant lipoleiomyoma should be kept in mind in the differential diagnosis of leiomyosarcoma or ovarian malignancy. Uterine leiomyomas are the most commonly seen gynecologic tumors and their prevalence is stated as 25–40% in reproductive age . The fibA 45-year-old premenopausal multiparous woman was admitted to our hospital's outpatient gynecology clinic with complaints of lower abdominal pain and abdominal distension for the last 6 months. On detailed anamnesis, the patient had noticed a mass in her abdomen for 3 months and a gradually increasing pain with easy tiredness. She had four previous vaginal deliveries with no abdominal surgical operation. Her medical history was remarkable for 10 years of type II diabetes and hypercholesterolemia and had no history for family member with genital malignancy. She had no complaints related with menstrual bleeding. On physical examination, her vital signs were normal and abdominal palpation revealed a distended abdomen with palpable hard, solid mass filling whole abdominal cavity which cannot be lateralized. No abdominal rebound or tenderness was observed. The speculum examination showed a normal uterine cervix and vagina but fornices were full on pelvic examination. Initially, a transvaginal ultrasound was applied and showed a large, solid, and complex mass in pelvic cavity which extended to subdiaphragmatic area and its origin could not be found. An MRI scan of abdomen showed that a large solid mass with somewhere in cystic and fatty content, approximately 33 × 17 × 25 cm in size, which could not be separated from uterus was noticed (Figures Laboratory examinations for whole blood count, liver function tests, coagulation parameters, urea, creatinine, and serum electrolytes were in normal limits. Blood glucose, HgA1c level, and cholesterol levels were higher than normal limits. The serum level of cancer antigen-125 was detected to be high as 210 mIU/ml. So in the light of these clinical findings from ultrasound, MRI examinations and laboratory findings, we thought that leiomyosarcoma is the most likely diagnosis. We planned surgery for the patient and a midline xiphopubic vertical incision was made. At laparotomy, on inspection, a giant multilobulated solid mass with white-yellowish colour was noted and we thought a huge mass of ovarian malignancy, however when we put up the mass from abdomen out, then we saw that it was a pedunculated giant subserosal myoma with a thin stalk . FirstlyMacroscopically, pathologic examination revealed a multilobulated solid mass measuring 32 × 23 × 12 cm showing the appearance of leiomyoma. The microscopic examination showed lipoleiomyoma of uterus with extensive cystic and fatty content as admixed with mature adipocytes without histologic signs of malignancy . Both ovLeiomyomas are benign tumors of uterine wall and accounting in approximately one-third of women of reproductive age . These bThere are numerous diagnostic modalities for leiomyoma, leiomyosarcoma, or ovarian malignancy including ultrasonography, MRI, computed tomography, hysteroscopy, and saline infusion sonography. But none of these diagnostic imaging modalities can differentiate the benign and malignant growths exactly without confirmation by the pathological examination. Like this, as a blood test, the cancer antigen-125 is a useful marker in diagnosis of malignant ovarian mass but it can be seen in high levels also in uterine fibroids as well as in other benign gynecologic diseases. Ultrasonography is the preferred imaging tool for diagnosis of the initial evaluation after bimanual examination because of its wide availability, being inexpensive, and least invasive character. On ultrasonography, only those parts of the tumor that are close to the probe can be demonstrated clearly. Although uterine mass containing fat can be diagnostic of lipoleiomyoma in ultrasonography, MRI is an important imaging tool for precision for identification, number, and location of these tumors and can exclusively show the fat content within the tumor as well as differentiation from an adnexal mass especially in cases of giant mass which could not be differentiated from an adnexal malignancy . In our Pelvic mass during perimenopausal state should be clarified and majority of these are benign gynecologic conditions including primarily uterine fibroids. In case of a giant pelvic mass, the diagnosis is difficult and frequently leads the physicians to suspect favouring the malignant growths of ovarian tissue or leiomyosarcoma. A color Doppler flow can be used to differentiate the malignant and benign ovarian tumors but it cannot be applied to differentiate the lipoleiomyoma and uterine leiomyoma.As far as we know from literature, there are some reports about giant myomas including lipoleiomyoma which have different clinical scenarios such as in a case report by Akbulut et al. reporting a case of symptomatic giant lipoleiomyoma of the uterine corpus that may be associated with diabetes mellitus and hypothyroidism . In a reIt was reported that uterine lipoleiomyoma may be associated with metabolic diseases including hyperlipidemia, hypothyroidism, and diabetes mellitus . Lin andThere are numerous established managements of uterine fibroids and also of lipoleiomyoma including one or a combination of the following as expectant therapy, surgery, medical or hormonal treatment, myolysis, and uterine artery embolization. The treatment should be individualized according to many factors including the patient age, fertility status, the severity and type of symptoms, suspicion of malignancy, the location and size of myomas, and desire of patient. The surgery is most frequently preferred for treatment of giant leiomyomas. The surgical approach of these giant tumors concerns some intraoperative technical difficulty such as the increase of blood loss, any injury to adjacent organs due to dense intestinal adhesions, or anatomical change of ureters because of huge mass within the pelvic cavity.In conclusion, lipoleiomyoma is a rare and specific type of uterine leiomyoma and it can be diagnosed easily with examination and diagnostic modalities. However, in case of giant myomas the diagnosis can be difficult and masquerading as a malignant adnexal mass especially if there is fatty degeneration and pedunculated myoma. So the physicians must be aware of those giant pedunculated lipoleiomyomas in the differential diagnosis of semisolid and multilocular pelvic masses.


Spontaneous hepatic hemorrhage is a rare condition that may be caused by an underlying hepatic tumor, most commonly hepatocellular carcinoma or hepatic adenoma. A spontaneous rupture of a hepatic metastasis from a gastric gastrointestinal stromal tumor is also extremely rare, and the majority of affected patients present with hypovolemic shock or an acute abdomen. In this article, we report the case of a 65-year-old man with a spontaneous rupture of a hepatic metastasis from a gastric gastrointestinal stromal tumor that presented as hypovolemic shock. Cross-sectional imaging studies (computed tomography or magnetic resonance imaging) play a significant role in the diagnosis of this condition and guides its management. Gastrointestinal stromal tumors (GISTs) are relatively common subepithelial tumors that occur most frequently in the stomach, small bowel, esophagus, and omentum. The liver is the most common metastatic site of a GIST. The spontaneous rupture of a hepatic metastasis from a gastric GIST is extremely rare. To the best of our knowledge, only 2 cases have been reported in the English literature to date. The present report describes the case of a patient who presented with hemoperitoneum and hypovolemic shock due to the spontaneous rupture of a liver metastasis from a malignant gastric GIST.3, red blood cells 12.6 mg/dL, platelet count 113 000/mm3, and C-reactive protein 0.06 mg/dL. The liver function test results were the following: aspartate aminotransferase 178 IU/L , alanine aminotransferase 201 IU/L , alkaline phosphatase 254 IU/L , and α-fetoprotein 1.8 ng/mL .A 65-year-old man presented at the emergency department of our hospital with sudden right upper quadrant and chest pain, respiratory difficulty, and a 3-day history of fever, chills, nausea, and vomiting. During physical examination, his blood pressure was 100/70 mm Hg, and his pulse rate was 78 beats/min. The results of a peripheral blood examination were the following: white blood cells 10 370/mmAbdominal multidetector computed tomography (CT) showed a large hematoma in the intrahepatic and subcapsular regions of the liver. A spherical hepatic mass identified in the posterior segment of the liver was connected to the hematoma . The mas3 Lipiodol and 20 mg Doxorubicin  was slowly infused. Because most of the Lipiodol mixture leaked, complete occlusion of the feeder vessel at the leakage site was performed using a 50% mixture of n-butyl cyanoacrylate .An emergency angiography showed hepatic tumor vessels emerging from the anterior segmental branches of the right hepatic artery with abnormal pear-shaped active contrast leakage . DiffusePostembolization celiac angiography showed no further visible contrast leakage, exhibiting only residual tumor staining. The vital signs of the patient were stabilized, and an abnormal dumbbell-shaped gastric submucosal mass was identified during gastrofiberscopy at the level of the gastric body . Three dThe cellularity was relatively moderate, and the frequency of mitotic figures was 15 mitoses per 50 high-power fields. In immunohistochemical studies, the tumors were diffusely immunoreactive for CD 34 and C-kit (CD 117) and negative for SMA, Desmin, and S-100 protein. Based on these pathologic and immunohistochemical findings, a diagnosis of ruptured hepatic metastasis from a malignant gastric GIST was made. The patient had an uneventful postoperative course over the subsequent 6-month period.1Hemoperitoneum secondary to spontaneous ruptured hepatic metastases from primary tumors of the lung, pancreas, stomach, kidney, breast, prostate, testicle, gallbladder, skin (melanoma), and nasopharynx as well as choriocarcinoma and hepatic lymphoma has been reported.2 Most individuals are older than 50 years at the time of presentation. GISTs rarely occur in patients younger than 40 years of age.3 GISTs are most common in the stomach (70% of cases), followed by the small bowel (20% to 30%), anorectum (7%), colon, esophagus, omentum, and mesentery.2 Based on several immunohistochemical studies, it has become widely accepted that GISTs differentiate from the interstitial cells of Cajal.4GISTs are common mesenchymal tumors arising from the gastrointestinal tract with an incidence of 10 to 20 cases per million.5 The clinical features of GISTs over long follow-up periods have been investigated. Most GISTs (62.6%) were identified incidentally through endoscopic screening. The most common symptom was pain, followed by gastrointestinal bleeding, signs of obstruction, and masses.Immunohistochemical analysis of the tumor in our case revealed diffusely positive immunoreactivity for C-kit (CD 117), tyrosine kinase growth factor receptor, and CD 34 but negative immunoreactivity for smooth muscle actin, Desmin, and S-100 protein. These results indicate that the tumor had differential features of the interstitial cells of Cajal, leiomyomas, leiomyosarcomas, schwannomas, and neurofibromas.6 However, the rupture of a hematoma and the development of hemoperitoneum from a metastatic GIST are very rare. These symptoms are frequently associated with primary hepatic tumors, such as hepatocellular carcinoma or hepatic adenoma, which have greater vascularity than metastatic lesions. Since the first reported case of a ruptured hepatic metastasis from GIST by Suzuki et al in 2003, only 2 additional cases (one in the greater omentum and one in the jejunum)8 have been documented in the English literature. At imaging, the diagnosis of a hemorrhagic metastasis is suggested if blood is identified in one or more liver lesions in a patient with known hepatic metastases or a known primary hepatic tumor. If the hemorrhage is severe, a subcapsular hematoma or hemoperitoneum may also be noted. The presence and extent of intrahepatic or subcapsular hematoma and hemoperitoneum can be easily identified using CT and may appear as a hyperattenuating mass or a mass with high-density material or hyperdense ascites. During the first 24 to 72 hours, acute hematomas are hyperattenuating on precontrast CT scans. Afterward, the attenuation decreases, and a pseudocapsule may develop after the subacute stage.Malignant GISTs commonly metastasize to the liver or peritoneum. In contrast, lymph node and extra-abdominal metastases are rare. The liver is the most common metastatic site at both initial presentation and relapse of the disease.9The treatment of hemoperitoneum depends on the size of the hepatic tumors, their location, and the rate of bleeding. Controlling the hemorrhage is the main objective. Regardless of the treatment, the prognosis is very poor. One patient died 3 months after the recurrence of bleeding, and another patient survived for less than 6 weeks.The precise cause of the spontaneous rupture of a GIST or metastatic GIST is unknown. The rupture may originate at a wall of a mass that is weakened by cystic or hemorrhagic degeneration. It is generally believed that the malignant potential of a GIST, such as that of other mesenchymal tumors, is related to hypercellularity, nuclear atypia, and mitotic activity. Local and/or distant metastases can develop from a potentially malignant GIST. However, it is often difficult to establish a preoperative diagnosis. In most cases, the clinical behavior of a GIST can be predicted with relative accuracy using a combination of tumor size and mitotic activity. Gastric GISTs measuring at least 10 cm or with more than 5 mitoses per 50 high-power fields have high risk for progression, metastatic spread, or relapse after resection. All GISTs are considered potentially malignant. Once GISTs recur, metastasize, or invade adjacent organs, they are considered malignant.10Complete surgical excision with a wide margin of normal tissue is the treatment of choice for GISTs. Regional node resection is not always required because nodal metastases are not usually associated with GISTs. If a patient with a GIST has metastatic lesions in the peritoneal cavity and/or the liver, complete resection of the lesions may improve survival. Imatinib  is a new molecularly targeted tyrosine kinase receptor blocker that targets tumor cells specifically, limits their growth, induces apoptosis, and leads to a dramatic treatment response and markedly improved long-term survival of patients with GISTs.In conclusion, we reported a rare case of a spontaneously ruptured hepatic metastasis from a malignant gastric GIST that presented as hemoperitoneum. We also described the proper treatment of the condition.


Extrarenal rhabdoid tumors (ERRTs) are extremely rare neoplasms; of these, colorectal ERRTs are the most rare, and only nine cases have been previously described in the English language literature. The current study reports the pathological features of a case of poorly differentiated cecal adenocarcinoma with prominent rhabdoid feature, which was combined with mucinous cystadenoma of the appendix in a 73-year-old male, and additionally reviews the previously reported cases. Microscopically, the majority of tumor cells were non-cohesive or loosely cohesive, with a polygonal morphology and prominent rhabdoid feature, showing eccentric vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) and vimentin, but negative for CK20, CK7, desmin and smooth muscle actin. This indicated a diagnosis of poorly differentiated adenocarcinoma with prominent rhabdoid features, combined with appendiceal mucinous cystadenoma. At two months following surgery the patient succumbed to peritoneal seeding and metastasis of liver and bone The emergence of the rhabdoid phenotype is invariably associated with an aggressive and almost always fatal clinical course. The present case is the 10th example of such a tumor in the colon, and to the best of our knowledge, this is the first case of colonic rhabdoid tumor coinciding with appendiceal benign mucinous neoplasm. Rhabdoid tumors (RTs) are aggressive neoplasms, initially described by Beckwith and Palmer as a sarcomatoid rhabdoid variant of Wilms’ tumor . Tumors A 73-year-old male was admitted to the Department of Surgery, Chosun University Hospital  with a 2-week history of pain in the right lower quadrant. Abdominal computed tomography (CT) with enhancement by contrast media revealed acute appendicitis and a cecal edema; based upon this finding and inflammation, cancer was suspected. Upon a clinical diagnosis of acute appendicitis, appendectomy was performed. During surgery, a cecal mass was identified, and an examination of the frozen section of the cecal lesion revealed malignancy. Therefore, in addition to appendectomy, a right hemicolectomy with regional lymph node dissection was conducted.A protruding mass of 4.0×3.0×1.5 cm in size, with central ulceration and necrosis was identified in the cecum . Microscet al  or adenocarcinoma with rhabdoid features. Although MERTs have been well-defined and characterized as a clinicopathological entity ICD-O 8963/3) , the exi3/3 , theAll rhabdoid colorectal tumors (RCTs), including those previously reported and the present case, are listed in et al  with a review of the previously reported RCTs. In the present case, separate appendiceal mucinous cystadenoma was concomitant with RCT. All RCT cases exhibit similar clinicopathological features, as well as the characteristic histological feature of sarcomatous dedifferentiation of rhabdoid cells, which appears to indicate aggressive biological behavior. Further investigations into this highly aggressive colonic carcinoma showing rhabdoid feature are required in order to determine specific and effective treatment for this tumor type.


Pneumomediastinum, and subcutaneous emphysema usually result from spontaneous alveolar wall rupture and, far less commonly, from disruption of the upper airways or gastrointestinal tract. Subcutaneous neck emphysema, pneumomediastinum, and retropneumoperitoneum caused by nontraumatic perforations of the colon have been infrequently reported. The main symptoms of spontaneous subcutaneous emphysema are swelling and crepitus over the involved site; further clinical findings in case of subcutaneous cervical and mediastinal emphysema can be neck and chest pain and dyspnea. Radiological imaging plays an important role to achieve the correct diagnosis and extension of the disease. We present a quite rare case of spontaneous subcutaneous cervical emphysema, pneumomediastinum, and pneumoretroperitoneum due to perforation of an occult sigmoid diverticulum. Abdomen ultrasound, chest X-rays, and computer tomography (CT) were performed to evaluate the free gas extension and to identify potential sources of extravasating gas. Radiological diagnosis was confirmed by the subsequent surgical exploration. Subcutaneous cervical and mediastinal emphysema usually can occur as a result of surgery or trauma. Their spontaneous onset in absence of previous disorders or provocating factors is very rare.Potential sources of extravasating gas are the respiratory tract , the gastrointestinal tract (perforation), and infective causes (necrotising fasciitis) .Pneumomediastinum and subcutaneous emphysema are very rare; reported signs of colonic perforation most often are associated with diverticulitis, toxic megacolon, and colonoscopy .The continuum of fascial planes connecting cervical soft tissues with the mediastinum and retroperitoneum allows this dissection .Clinical appearance depends on the degree and the extension of emphysema, and typical findings of spontaneous subcutaneous emphysema are swelling and crepitus over the involved site.Imaging studies are helpful to confirm the diagnosis on doubtful cases, exclude local associated complications, determine the extension, and monitor the evolution.Most commonly the diagnosis of subcutaneous emphysema and pneumomediastinum is made by chest X-ray, except in case of small gas collections that can be identified only by chest CT scan.We describe a rare case of spontaneous subcutaneous emphysema, extending from the soft tissues of the abdominal wall to the neck, pneumomediastinum, and pneumoretroperitoneum resulting from an unknown sigmoid diverticulum perforation.A 57-year-old man was referred to our institution with a few days' history of severe left iliac fossa pain and pyrexia. The patient medical history included chronic diverticular disease.At clinical examination he was pasty, sweaty, tachycardic and tachypneic, and in pain and he had a temperature of 38°C. The abdomen was slightly distended, vaguely tender on palpation over the lower region, without signs of peritoneal irritation, but he became febrile and confused. Peristalsis remained active and bowel function was normal.His initial blood tests showed polycythemia and rise up transaminases.The patient was transferred to our radiology department for ultrasonography exams, basic X-ray studies, and CT scanning.We performed an abdomen ultrasonography that excluded subdiaphragmatic parenchymal tissue alterations.Chest X-ray showed no sign of pneumothorax or pneumomediastinum.A CT scan of the abdomen  showed iGas was finally visualized within the scrotal sac, indicative of pneumoscrotum.CT scan revealed also mesenteric and mural thickening of the sigmoid colon, multiple sigmoid diverticula, peritoneal fat stranding, and evidence of inflammatory changes.Considering patient's history and clinical and radiological features, the main diagnostic hypothesis was a diverticular perforation.A few hours later the clinical evaluation showed subcutaneous emphysema and crepitus developed in correspondence to neck's soft tissue, to sternoclavicular joints, and to maxillary muscles bilaterally so a second chest radiograph was performed.The radiograms  showed sDue to the clinical and radiological worsening, the patient underwent a new computed tomographic evaluation.The whole body CT scan  revealedFree gas was also noted in correspondence of the dorsal and pectoral soft tissues.This condition was suggestive of some communication between the chest and the retroperitoneum. Based on the CT scan, no other possible cause of the extraperitoneal gas except a diverticular perforation was found.After the tomography, an urgent exploratory laparotomy was undertaken, under the suspicion of sigmoid diverticulitis rupture. During the operation, a perforation over the posterior wall of the sigmoid colon was found. Segmental resection of the sigmoid colon and end-colostomy (Hartmann's procedure) were performed.Histologically, severe inflammatory changes with a mixed population of leucocytes, edema, and fresh haemorrhage at the edges of the rupture site were observed, with no sign of malignancy. The patient's subcutaneous emphysema and pneumomediastinum disappeared few days after the operation. One month later, the patient was still alive.Pneumomediastinum and subcutaneous emphysema are uncommon clinical entities that occur when gas leaks from the lungs or any of the luminal organs, such as the bronchial tube, larynx, trachea, esophagus, and very rarely, the colon, with subsequent dissection into the mediastinum .They can occur in trauma: subcutaneous emphysema has been observed in hanged persons and reported in the literature.In these cases, the mechanism of closing of the upper airways by compression of the larynx or pharynx and pressure of the tongue against the palate with an increase of intrathoracal pressure was assumed to be the main pathogenesis .Another rare cause has been described in a case of sudden infant death syndrome with intravascular gas due to intraosseous medication application .In this case gas embolism may passively occur when a puncture is made in a vessel with a pressure lower than the atmospheric one. The gas can enter the vessel when the needle is disconnected from the syringe to change medication or by accident .Emesis may also cause esophageal rupture, which may lead to pneumomediastinum and subcutaneous emphysema .Instead spontaneous pneumomediastinum commonly occurs when an increased intra-alveolar pressure  leads to the rupture of the marginal pulmonary alveoli. The air ascends along the bronchi to the mediastinum and the subcutaneous space of the neck, causing cervicofacial subcutaneous emphysema in 70–90% of cases .In the literature there are only a few cases of subcutaneous emphysema of gastrointestinal origin reported –9, that Other causes include Borehaave's syndrome, perforated peptic ulcers, traumatic perforation, appendicitis, and diverticulitis .The anatomical site of perforation largely determines the route of escape of the gas to the subcutaneous position. In addition, the direction of gas diffusion usually follows the least resistance, loose areolar fascial structures .In our case the perforation was located in the posterior wall of the sigmoid colon, so the escaped gas penetrated first into the retroperitoneal space and then may continue diffusing superiorly through the paravertebral retroperitoneal tissues and diaphragmatic hiatus into the mediastinum and finally into the neck and facial areas.Diagnosis is made most commonly by chest X-ray, which reveals subcutaneous emphysema and pneumomediastinum. Small pneumomediastinum not seen on chest X-ray will be seen on chest CT.Although, as mentioned above, subcutaneous neck emphysema, pneumomediastinum, and retropneumoperitoneum are rarely caused by nontraumatic perforations of the colon, this possibility should be considered when no obvious case can be found for the origin of free gas at these sites.In this case, abdomen CT scan plays an important role in identifying an occult perforation and whole body CT allows us to evaluate the extension of escaped gas to different body districts.In fact, in cases of suspected soft tissues emphysema, for a fast management of the patient, we recommend performing a CT, that is, the most preferable diagnostic tool for the detection of air in soft tissues .Finally radiological imaging plays also an important role in allowing a proper therapeutic planning and followup.We conclude that performing CT is very useful in making a secure diagnosis of pneumomediastinum and soft tissue emphysema because it allows us to define a precise extension of gas to different body districts.Patients can be scanned very quickly and easily and other information supplied by CT is also necessary to achieve the final diagnosis and ruling out other causes of pneumomediastinum and soft tissues emphysema.Radiological evidence of pneumomediastinum and soft tissues emphysema may lead to early diagnosis and surgical management with a better outcome.


Extra-adrenal pheochromocytomas are rare tumors that originate from the chromaffin tissue of the sympathetic nervous system. Ovarian extra-adrenal pheochromocytoma is even more rare. The present study reports a rare case of an extra-adrenal pheochromocytoma that was localized to the right ovary, but was gynecologically asymptomatic. Computed tomography angiography (CTA) detected the tumor and indicated that it was well defined, highly vascularized and obtained its blood supply from the right ovarian artery. This is the second case of ovarian extra-adrenal pheochromocytoma reported in the literature, and the first description of the CTA manifestations in the ovary. Gynecologists and radiologists should consider the possibility that an ovarian mass could be an extra-adrenal pheochromocytoma, which would allow time to prepare appropriately for the surgical removal of the mass. Pheochromocytomas  are functional or non-functional tumors of the sympathetic nervous tissue . CatechoA 43-year-old female with abdominal pain was referred to Xiangya Hospital  and underwent abdominal CTA with a suspected diagnosis of an abdominal aortic dissecting aneurysm. A right ovarian tumor was found, and no dissecting abdominal aortic aneurysm was detected.The patient’s history included three viable pregnancies, regular menstrual cycles and no history of vaginal bleeding. For the past seven years she had experienced sustained hypertension, with the highest blood pressure recorded being 200/100 mmHg. The patient had not taken any medication to treat the hypertension until one month prior to the present hospital visit for abdominal pain. No headache, syncope or palpitations were indicated.Upon physical examination, the patient has a body temperature of 36.4°C , with a heart rate of 106 beats/min , a respiratory rate of 20 breaths/min  and a blood pressure of 167/114 mmHg . The gynecological examination indicated a normal uterus and cervix, and no significant mass was palpable. Abdominal CTA showed a well-defined 5.2×5.7×4.0-cm tumor in close proximity to the right ovary . The tumSurgical resection of a 5×5×6-cm mass located superior to the right ovary was performed. Immunohistochemically, the tumor cells were positive for chromogranin A, neuron-specific enolase and synaptophysin , and theet al (The majority of extra-adrenal pheochromocytomas are located in the uterus, retroperitoneum, lumbar spine, bladder, ocular orbit, heart and mediastinum –8. To thet al  reportedExtra-adrenal pheochromocytomas are divided into two categories, namely, functional and non-functional tumors. The clinical manifestations of the former are similar to those of adrenomedullary pheochromocytomas. In functional tumors, increased catecholamine secretion is responsible for symptoms such as hypertension, sweating, headache, diaphoresis, anxiety, tachycardia and/or palpitations . In addiAlthough rare, the possibility of pheochromocytoma should be included in the differential diagnosis of ovarian tumors, particularly in females with hypertension. Further examinations, such as tests for hematuria VMA, are necessary. Surgical resection is the only treatment option for extra-adrenal pheochromocytomas ,12. GyneIt can be difficult to observe any histological distinction between benign and malignant cases, and therefore predict malignant potential, due to the variable and non-specific clinical presentation and imaging data of these patients. Local recurrence or distant metastases are common. The establishment of an effective diagnosis, management strategy and follow-up regime is vital and requires a multidisciplinary approach involving collaboration between geneticists, endocrinologists, anesthesiologists, surgeons, laboratory specialists, oncologists, radiologists and pathologists.In conclusion, although ovarian paragangliomas occur infrequently, it is important that they are recognized as a potential cause of an abdominal mass. Clinical and imaging data of patients with extra-adrenal, intra-abdominal paragangliomas are inconsistent. These tumors can even be asymptomatic, as shown in the present case. Therefore, it is essential that a comprehensive histological and immunohistochemical evaluation is performed as the only safe modality for diagnosis. Since these tumors can localize to the ovary and are difficult to definitively diagnose pre-operatively, gynecologists and radiologists should consider the possibility of an extra-adrenal pheochromocytoma prior to preparing to surgically remove a pelvic mass.


Background: Hydrometrocolpos (HMC) develops as a result of vaginal outflow obstruction and the accumulation of secretions. It might be secondary to persistent cloaca, urogenital sinus, some syndromes, presence of the vaginal septum, vaginal atresia, and imperforate hymen. Each of them has different treatment options and follow-up protocols. This study was performed to identify the etiology and the related management of patients with HMC. Materials and Methods: A descriptive series of patients with HMC managed in our hospital between 2004 and 2011 is being presented. The medical record of these patients was analyzed for etiology, management, and outcome.Results: Eight patients with HMC were managed during 7 years at our department. Underlying etiologies included urogenital sinus (n=3), and 1 each of imperforate hymen, transverse vaginal septum, Herlyn-Werner-Wunderlich syndrome, persistent cloaca, and a variant of the cloaca. Four patients were prenatally diagnosed. The patient with imperforate hymen was managed successfully with incision and drainage. Abdominal vaginostomy was done in three patients with urogenital sinus as initial procedure. In patient with persistent cloaca, a colostomy and abdominal vaginostomy were performed. Patient with cloaca variant died due to persistent acidosis and salt wasting. Conclusion: HMC may have different etiological factors which may dictate different surgical management. Etiology of HMC can be as simple as imperforate hymen to the most severe cloacal malformations. Hydrometrocolpos (HMC) develops in the female as a result of a vaginal outflow obstruction and the accumulation of secretions. It might be secondary to a transverse vaginal septum, vaginal atresia or imperforate hymen, and cloacal anomaly [1-3]. Many of these females may present with associated malformations and syndromes, such as cloacal dysgenesis sequence, McKusick–Kauf-man, Ellis–van Creveld or Bardet–Biedl syndromes [4-6]. The underlying etiology of HMC dictates the surgical management. We describe our experience of 8 cases of HMC with different pathologies leading to variable surgical therapy.The medical record of 8 patients with HMC, managed in our hospital between 2004 and 2011, was analyzed for etiology, management, and outcome.Eight patients with a diagnosis of HMC presented to us at different ages (Table 1). Four of them presented for the management of HMC . Two patients presented with a pelvic mass, whereas, two other patients presented for ambiguous genitalia/anorectal malformation; and HMC was diagnosed while evaluation.In the subset that was prenatally diagnosed to have HMC, one patient had imperforate hymen leading to the pathology. Two patients had urogenital sinus and one patient had persistent cloaca as etiology of HMC. One of the patients with urogenital sinus also had type IV sacrococcygeal teratoma (SCT). Of those who presented with pelvis mass, one patient was operated for acute abdomen else-where and then referred to us for the management of HMC. On examination, transverse vaginal septum was found. The other patient presented with a mass behind urinary bladder and left renal agenesis. On evaluation, she was found to have bifid uterus and vaginal septum. However, spontaneous drainage of the mass occurred following menstruation and Herlyn-Werner-Wunderlich (HWW) syndrome was assumed as her diagnosis. One patient of ambiguous genitalia with HMC had persistent cloaca. The patient however succumbed to persistent metabolic acidosis and electrolyte imbalance, the cause of which could not be identified. In all cases, hydronephrosis secondary to lower urinary tract obstruction was noted. In fetal HMC cases, one had severe oligohydramnios. In one case of urogenital sinus, urinary ascites was noted in prenatal period. The management in these patients is tabulated in table 1.HMC is a manifestation of many different non-syndromic/syndromic anomalies. Imperforate hymen, vaginal atresia, persistent urogenital sinus, and cloacal malformation, are the main etiological factors [1-3]. It may account for 15% of lower abdominal masses in females [1-3]. The differential diagnoses of HMC include meconium pseudocyst, enteric duplication cyst, fetal ovarian cyst, dilated bowel, mesenteric cyst, rectal duplication, cystic neuroblastoma, or bladder duplication [4-6].HMC may be associated with urinary and intestinal tract malformations through a wide spectrum of more complex malformations, from persistent urogenital sinus to cloacal dysgenesis [2-9]. Although these malformations share common embryological origins, they might lead to different anatomical defects [10]. In persistent urogenital sinus, failure of urethrovaginal septation at 6 weeks' gestation leads to drainage of the bladder and the vagina into a common channel. In this case, the perineum exhibits two distinctive orifices, a ventral urogenital sinus and a dorsal anus. In cloacal malformation, complete developmental failure of the urorectal septum, which normally divides the cloaca into a ventral urogenital sinus and a posterior anorectal canal at 5 weeks' gestation, leads to a single exit chamber and severe anorectal atresia. This chamber is connected to a single perineal orifice . An increase in cholinergic innervation in both the cloaca and colorectum during development had been determined in another study [12-15]. Presentation of HMC depends on the degree of compression of the surrounding structures by the uterovaginal swelling , Commonly, the urinary tract is compressed leading to varying degrees of hydronephrosis [16-19]. Pressure on the bowel to cause obstruction is not common . The diagnosis of HMC is often delayed in older girls which often have prolonged investigations to exclude more common causes of intestinal and urinary obstruction [16-18]. HMC should be considered in the differential diagnosis of a female infant with abdominal mass with or without constipation and urinary obstruction. When hydrocolpos is suspected prenatally, subsequent ultrasound examination should rule out associated malformations and provide precise information of the pelvic anatomy. The contribution of MRI has been excellent for diagnosis when ultrasonography was unable to specify fetal pelvic anatomy . Ultrasound and MRI are currently recognized as a valuable tool for imaging. The characteristic features of HMC are as follows: (1) an abdominal mass locate in the midline posterior to the bladder, (2) the mass is fluid-filled with a mid-plane septum, (3) the mass is connected to the dilated uterus, and(4) if a four-chamber appearance is observed, then it is consisting of the septate vagina and two uterine cavities . Surgical intervention for isolated HMC solely with lower tract obstruction is associated with excellent results, however, surgery for persistent urogenital sinus is uncertain and even more so for cloacal malformation. Some authors have suggested that in persistent urogenital sinus or cloacal malformation, fetal urine may escape from the fallopian tubes into the abdomen . In the 8th case that we have described, biochemical analysis of the ascitic fluid not only confirmed the presence of urine but also demonstrated the presence of digestive enzymes. This information, together with the absence of visualization of the anal canal and rectum, made the diagnosis of cloacal malformation even more likely. The prenatal differentiation of female urogenital anomalies can be difficult because of their rarity, variations in presentation and poor imaging by ultrasonography, especially in late gestation. Although a prenatal diagnosis of HMC has been reported as early as 25 weeks of gestation, most cases are described in the third trimester of pregnancy or after birth . Although the cystic mass could be distinguished from the bladder, which was identified by the surrounding umbilical arteries, the origin of this mass could not be determined by ultrasonography. MRI was useful for identifying the uterus and the rectum using T1-weighted imaging, particularly as it revealed the continuity of the mass with other organs .In conclusion, etiology of HMC is as simple as imperforate hymen to the most severe cloacal malformations. Treatment planning is tailored according to causes of HMC. Parents should be counseled about treatment and prognosis as to the underlying pathology causing HMC.Source of Support: NilConflict of Interest: None


Oncocytoma or oxyphilic adenoma is uncommon salivary gland tumour, occurs predominantly in the in patients older than 60 years of age. Clinically oncocytoma resemble other salivary tumours while histology is typically consisting of oncocytes with many hyperplastic mitochondria. It usually occurs in the parotid gland. Because the features of oncocytoma are similar to those of other benign and low-grade malignant salivary tumours, clinical diagnosis is often challenging.This report presents the pathologic and imaging findings of an oncocytoma arising in the deep lobe of the right parotid gland in a 74-year-old male. Oncocytoma was diagnosed on the basis of histological, magnetic resonance imaging (MRI), scintigraphic findings and fine needle aspiration cytology (FNAC).This case was unique because in the literature there are few articles about the rare presentation and deep lobe location of this type of parotid oncocytoma. Oncocytoma or oxyphilic adenoma is a rare benign salivary gland tumor, which mostly occurs in the parotid glands, few affect the submandibular and rarely affect the minor salivary glands -4. OncocThe clinical presentation of oncocytomas is usually identical to other benign salivary tumors. The image modalities of choice to identify the well-defined homogeneous mass are CT Scan MRI, ultrasonography, radionuclide salivary scintigraphy and positron emission tomography CT scans , 12.Complete surgical removal with superficial or total parotidectomy is the treatment of choice, whereas radiation therapy is not indicated because oncocytes are radioresistant . RecurreLong-term follow-up using imaging refinements is recommended to evaluate the patient’s progress. Here we are presenting the case of a 74-years-old male patient with right oncocytoma of the deep lobe of the parotid gland extending into para pharyngeal space.A 74-year-old man underwent a computed tomographic (CT) scan to determine the status of cervical lymph node involvement and discrete swelling of the right parotid gland. One year before, the patient had undergone partial resection of soft palate for mucoepidermoid carcinoma. Neck lymph node involvement was negative, but 32 x 24 mm well-circumscribed mass was incidentally detected in the deep lobe of the right parotid gland . MRI of The mass showed low-signal intensity to parotid gland parenchyma on T1- and T2-weighted images and homogeneous enhancement after intravenous gadolinium-based contrast medium administration. On the basis of these findings, the lesion was diagnosed as a deep lobe parotid tumour. FNAC with screening performance was applied immediately prior to operative treatment. A parotidectomy was performed with mandibular osteotomy, selectively excising a portion of a deep lobe of the right parotid gland . PostopePostoperative period was uneventful. The patient was treated with antibiotics and analgesics for five days. There was no clinical evidence of neurological deficit and was discharged with good health on the third day after surgery. The patient was comfortable at review after two months.Oncocytoma accounts for less than 1 - 2.2 % of all salivary gland tumours , 8-10. TThe parotid gland is a preferred site of oncocytoma. The tumours are generally noncystic, encapsulated, and rarely exceed 5.0 cm in diameter. Their location at onset is usually unilateral, but sometimes multiple or bilateral (7%) oncocytomas are seen , 11, 12.The growth grade rate is typically slow. Although malignant transformation and local recurrence of this tumour are unusual and uncommon, clinical follow-up is important because malignant oncocytoma may not be correctly diagnosed owing to histological similarities with benign oncocytoma . DiagnosChakrabarti  reportedWe have evaluated the present case with scintigraphy and supplementary MRI. Adenolymphoma -Warthin’s tumour and oncocytomas reveal intense uptake with marked retention of activity after stimulation on salivary radionuclide imaging while other primary or metastatic malignancies appear cold on radionuclide scintigraphy with technetium-99m-pertechneate. In general, MR images of Warthin’s tumours show decreased signal intensity on T1-weighted images and increased signal intensity on T2-weighted images.In our patient, oncocytoma showed decreased signal intensity on both T1- and T2-weighted images. This difference made it possible to distinguish oncocytoma from Warthin’s tumour. The decreased signal intensity on both T1- and T2-weighted images is attributed to the high cellularity and low free water content of oncocytoma.Fine-needle aspiration cytology (FNAC) is another fairly accurate preoperative procedure for the diagnosis of parotid tumours. Capone et al.,  reportedBecause the scintigraphic findings of the present case were not clearly compatible with Warthin’s tumours or oncocytoma, preoperative FNAC analysis under ultrasonography guidance was performed. The postoperative histological examination was performed for final evaluation of the tumour.In conclusion, oncocytoma of the deep lobe of the parotid gland is extremely rare but have distinctive imaging characteristics that have not been described for other benign or malignant parotid gland tumors. The other salivary gland tumors where oncocytes are found can usually be differentiated on the basis of their more typical cytologic details.Imaging findings are helpful in preoperative assessment to distinguish these benign lesions from other parotid gland tumors in different clinical circumstances. Through this case, we highlight the importance of histopathological examination in the positive diagnosis of parotid oncocytoma as well as in its differential diagnosis and also the importance of FNAC.


Acute bilateral paramedian thalamic and mesencephalic infarcts are uncommon. Occlusion of the artery of Percheron (AOP) is presumed to cause this specific stroke syndrome. However, occlusion of the AOP is rare and early diagnosis is challenging. Here we described a 70-year-old male patient who presented with acute disturbance of consciousness due to acute bilateral paramedian thalamo-mesencephalic infarction secondary to AOP occlusion. Anticoagulant therapy was administered, and his consciousness gradually improved. Acute bilateral paramedian thalamic and mesencephalic infarcts are uncommon . OcclusiA 70-year-old man presented at the emergency department (ED) after being found unresponsive in bed. He was last seen normal approximately 8 h ago. He had a 10-year history of hypertension, type 2 diabetes mellitus, hyperlipidemia and gout. There was no recent history of fever, headache, seizure, or trauma and no known toxic substance exposure. On arrival at the ED, his body temperature was 35.5 °C, blood pressure 131/61 mm Hg, heart rate 49 beats/min and respiratory rate 17 breaths/min. On neurological examination, he was comatose. His neck was supple and his pupils were anisocoric, with a 4 mm right pupil and a 6 mm left pupil. The pupillary light reflex was absent in both eyes. The vertical oculocephalic reflex was absent, and his left eye did not show adduction with turning of the head to the left. His limbs moved in response to painful stimuli. The rest of examinations were unremarkable.Laboratory findings including blood glucose, full blood count, electrolytes, liver and renal function tests, thyroid function tests, calcium, arterial blood gas and ammonia were unremarkable. Electrocardiogram (ECG) showed a normal sinus rhythm. Emergent head computed tomography (CT) revealed a faint hypodense lesion in the bilateral paramedian thalamus . Acute iHe was then admitted to the neurointensive care unit. Cerebrospinal fluid analysis was normal. Electroencephalography demonstrated diffuse background slowing without epileptiform activity. Diffusion-weighted magnetic resonance imaging (MRI) performed on the next day demonstrated hyperintensities in the bilateral thalami and rostral mesencephalon, consistent with restricted diffusion secondary to an acute ischemic stroke in the AOP territory . MR angiOur case illustrates the importance of considering ischemic stroke in the AOP territory in the differential diagnosis of acute disturbance of consciousness in the elderly. The clinical presentation can mimic non-convulsive status epilepticus, subarachnoid hemorrhage, metabolic or toxic encephalopathy, and encephalitis. The low sensitivity of CT makes AOP infarction diagnosis difficult. Diffusion-weighted MRI is the imaging modality of choice . HoweverAOP prevalence remains unknown. In a recent study, AOP infarction was found in 0.4% patients with a first-ever stroke in their stroke registry . Small-aThese patients must be differentiated from those with “top of the basilar artery” syndrome , 3, 4. HDeep cerebral venous thrombosis (DCVT) may also be confused with AOP infarction, because bilateral thalamic infarcts can also result from DCVT , 5. HoweOther differential diagnoses considered in our case were hypertensive encephalopathy, Wernicke’s encephalopathy, osmotic demyelination, Japanese encephalitis and Creutzfeldt-Jakob disease. These diagnoses were excluded according to clinical characteristics and imaging features .Successful tissue plasminogen activator therapy for AOP occlusion is reported in the literature , 7, but In conclusion, occlusion of the AOP is a rare cause of coma in elderly patients. Diffusion-weighted MRI is the imaging modality of choice for early diagnosis. Early recognition of AOP occlusion may lead to favorable outcomes.


During recent decades, the concept of surgical treatment of malignant bone and soft tissue sarcomas has evolved, with the aim of preserving limb function. In this paper we report a case of metatarsal reconstruction by means of serratus and rib free flap after excision of a synovial sarcoma located in the dorsal aspect of the midfoot. Five years after the operation, the patient was free from recurrence and recovered full foot function. Amputation has been widely used in the past and this procedure still remains a valuable option when limb salvage is not possible. Nevertheless, in selected cases, reconstruction by means of composite free flaps may be successfully used for limb preservation in the treatment of malignant foot tumors after surgical excision. During recent decades the concept of surgical treatment of malignant bone and soft tissue sarcomas has progressively evolved, and the preservation of the uninvolved parts of the extremities and the achievement of an acceptable limb function have become a major goal of oncological and reconstructive surgery .The foot is a very difficult site for limb salvage surgery because bone, tendons and neurovascular structures are present in close proximity. Anatomical compartments may be therefore difficult to isolate and preserve during oncological excision. Furthermore, this area does not offer opportunities for performing local flaps effective for covering and reconstructing complex tissue defects.Amputation has been widely used in the past and this procedure still remains a valuable option in order to eradicate the tumor and preserve an overall good prognosis for the patient in many cases. However, free flaps have now become a possible solution for limb salvage and functional preservation.Until now the use of free flaps for the treatment of complex defects of the foot, secondary to oncological excision of bone and soft tissue tumors, was limited to a small number of patients. It has been described in the literature by a few authors, who reported isolated clinical cases or small clinical series –4.In this paper we report a case of metatarsal reconstruction by means of free serratus and rib flap after excision of a synovial sarcoma located in the dorsal aspect of the midfoot. This flap was previously used in a few cases reported in the literature for traumatic defect of the lower extremities –8. To ouA 42-year-old woman came to our attention after two previous operations performed in another hospital for excision of a tumor mass located in the dorsal aspect of the midfoot. The patient presented 1 year after the second excisional biopsy which reported a diagnosis of neurinoma because of the recurrence of the tumor that was clinically located on the dorsal aspect of the foot in the 3rd metatarsal space (Fig. En bloc tumor resection with wide margins, including the proximal and medial thirds of the 3rd and 4th metatarsal bone, was performed Fig. . The plaThe postoperative course was regular. K-wires were removed 2 months after the procedure. The vascularized rib graft healed with metatarsal bones and a progressive remodeling of the bones in the site of conjunction of the distal aspect of the transferred rib and proximal aspect of the 3rd and 4th metatarsal bones was observed Fig. . No adjuSerratus anterior and rib free flap based on the serratus branches of the thoracodorsal artery was described by Richards et al. . AnatomiMetatarsal and soft tissue reconstruction with serratus anterior and rib flap has been rarely described in sequelae of traumatic injuries of the foot. Kurokawa repaired four metatarsal bones in the same foot, transferring a flap which included the 5th and 7th ribs into the flap and Kitsiou used the serratus anterior and rib flap in three further cases without complications, achieving positive clinical results –8.In our case we performed the reconstruction after excision of synovial sarcoma involving the dorsal aspect of the midfoot. To our knowledge, this is the first case in which the serratus anterior and rib free flap was used for the reconstruction of defect following excision of foot sarcoma.As reported by Toma et al. , limb sa


Pleomorphic adenoma is a rare benign salivary gland neoplasm in children, which can be treated by simple excision. This tumor is rarely included in the differential diagnosis of solid submandibular masses in children. In the neonates, congenital pleomorphic adenoma usually presents in the nasopharynx. Surgical excision is the treatment of choice and recurrence is not expected. We report what appears to be the first case of congenital pleomorphic adenoma in the submandibular region in a one-day-old newborn.The case of a one-day-old term baby is presented with a 5x2 cm left submandibualr mass with extension to the oral cavity. The mass was hard and non-mobile. During Ultrasonography and Contrast-enhanced Computed Tomography (CT) scan, the mass was solid with a heterogeneous internal structure. The tumor was completely excised and proved to be a pleomorphic adenoma during histopathological examination.Congenital pleomorphic adenoma rarely occurs in the nasopharynx and is treated by surgical excision. Our case is unique because the congenital pleomorphic adenoma is located in the submandibular gland of a newborn.  Pleomorphic adenoma (mixed tumor) of the salivary gland, although a well-recognized tumor in adults, is a rare tumor in the pediatric population. These tumors usually occur in the parotid gland followed by the submandibular gland and are rarely included in the differential diagnosis of solid submandibular masses in children . In the A one-day old term newborn with a birth weight of 3500 gr who was delivered by cesarean, was referred to our hospital because of a large left submandibualr mass. Upon physical examination a 5x2 cm non-mobile hard mass with no overlying skin erythma was present in the left submandibular region with extension to the oral cavity. The baby was not ill or toxic. The moro and grasp reflexes were normal; however, the sucking reflex was impaired. Imaging was performed for the patient. Ultrasonography revealed a large hetero- echoic solid mass containing scattered hyperechoic foci and internal vascular flow . Contrast-enhanced Computed Tomography (CT) scan showed a multicompartmental 5.7x5.7x6.5 cm hypodense lesion with moderate heterogeneous enhancement in the left submandibular area extending to the sublingual space and left half of the tongue through the posterior aspect of the left mandibular ramus .The oropharyngeal cavity was almost obliterated by the lesion. Teratoma was suspected and serum levels of BHCG and alpha fetoprotein were checked which were within normal limits. Open biopsy was performed which revealed a pleomorphic adenoma characterized by a mixture of glandular epithelial/ myoepithelial compo- nent and fibromyxoid stroma containing areas of cartilaginous differentiation.6.5x6x6 cm, and firm with tan-white nodular chondroid cut surfaces , is a rare tumor that occurs in the nasopharynx . It is cPleomorphic adenoma is a histologically diverse tumor, therefore noted to be a benign mixed tumor. They are derived from a mixture of ductal and myoepithelial cells and reveal both epithelial and mesenchymal differentiation. This tumor tends to be firm and mobile with no evidence of fixation to the mandible or the floor of oral cavity .During ultrasonography, the tumor is either hypoechoic or isoechoic to the rest of the normal gland parenchyma with occasional hyperechoic calcified foci. CT scan or MRI features are commensurate with the size of the tumor, which is homogeneous and well-defined in small lesions and more heterogeneous and less well defined in larger ones . ImagingIn our case, physical examination revealed a firm submandibualr mass, which was in favor for the solid nature of the lesion. Ultrasonogarphy and CT scan also confirmed the solid mass with internal vascular flow. Scattered hyperechoic foci were also present in the mass during ultrasonography. The extension of the mass to the oral cavity with no evidence of bony remodeling or discernible abnormality was shown in the CT scan. Consequently, the more common cystic congenital masses were ruled out and teratoma was our first diagnosis. However, open biopsy proved it to be a pleomorphic adenoma with no evidence of malignancy. This diagnosis was confirmed afterwards in the specimen from total surgical excision of the mass. Treatment of pleomorphic adenoma includes complete resection of the mass. which was performed for our patient with a satisfactory result. Rupture of the tumoral capsule or misunderstood tumoral margin can lead to local recurrence.Congenital pleomorphic adenoma rarely occurs in the nasopharynx, which is treated by surgical excision. Our case is unique because the congenital pleomorphic adenoma is located in the submandibular gland of a newborn.


Air travel mostly causes minor ear, nose and throat complaints. We describe a second report in literature of airway obstruction caused by a drop in atmospheric pressure during a routine commercial flight.A 54-year-old male was referred to a head and neck surgeon with a 2 cm left submandibular mass that would enlarge during commercial flights. As the plane gained elevation, the mass would grow and cause him to become stridorous and short of breath. The shortness of breath and stridor would only resolve upon landing of the plane. A CT scan showed a large air sac extending from the larynx at the level of the true vocal cords up to the angle of the mandible. Based on the history and the CT findings a diagnosis of a laryngocele was made. The laryngocele was excised using an external approach, resolving the patient’s difficulty with flying.This article reports a rare case of upper airway obstruction caused by atmospheric pressure changes during air travel. The reported case is of significance as only a few uncomplicated laryngoceles have been reported to cause airway distress in the literature. This report highlights the epidemiology, presentation, complication and management of laryngoceles. With the exception of otic barotrauma, air travel has only been reported to cause minor complaints in the ear, nose and throat . We descA 54-year-old male smoker was referred to a head and neck surgeon with a 2 cm left submandibular mass. On history, the patient described a chronic non-painful left neck mass that fluctuated in size over the years. The patient’s chief complaint, however, was the problem he experienced during commercial flights. During the plane’s ascent, the left neck mass would enlarge, and he would become short of breath and stridorous. These symptoms would only resolve upon the plane’s descent. During these episodes he never sought medical attention. However, these episodes were severe enough that he has been avoiding all air travel, and he only pursued surgical consultation to attend his daughter’s wedding abroad. On palpation of the neck, no neck mass, swelling, nor lymphadenopathy were appreciated. Flexible laryngoscopy showed an infantile type epiglottis. A CT scan of the neck was ordered and showed a large air-containing sac in the left neck, extending from the level of the vocal cords to the level of the angle of the mandible. The air sac, insinuated between the left strap muscles and left sternocleidomastoid, was causing mass effect on the left submandibular gland and the laryngeal structures Fig. . Based oA laryngocele is an air filled abnormal dilation of the laryngeal saccule communicating with the laryngeal lumen. The exact etiology of laryngoceles is unknown. Some authors attribute laryngoceles to congenitally present dilation of the saccule exacerbated by factors that increase intra-glottic pressure such as professional trumpet playing . It is iGiven the size of the external component of the laryngocele presented in this report, an external approach, to the laryngocele excision was taken. A detailed description of the external surgical approach is available elsewhere . In brieThe patient’s follow up consisted of one office visit, 2 weeks after the operation, and one and a half year phone follow up. He was able to return to work 9 days after the operation and had no complaints at any time. He resumed air travel 3 months following his surgery, and he has not experienced airway obstruction or neck swelling during flights again.The presented case highlights a typical patient who might present with a laryngocele: a male in his fifth or sixth decade referred with a non-tender neck mass that fluctuates in size . The unuWe searched Embase®, Pubmed, Google Scholar, and Web of Science™ databases (last search June 2015) and found one case reports from 50 years ago of airway obstruction during air travel caused by a laryngocele . Given tThe presented case is the second case report of upper airway obstruction during air travel. Given the ubiquity of air travel, it is likely that other patients with laryngoceles have experienced at least some worsening of their symptoms during airplane’s ascent. We encourage practitioners to question the rare patient that presents with a suspicion of a laryngocele about symptom changes with air travel. As illustrated in this case, a change in symptoms during the ascent and descent of air travel can potentially support the physician’s diagnostic suspicion of a laryngocele.Patient provided written informed consent for publication of the case report. Editor-in-chief was provided with a copy of the written consent.


A 33-year-old man suffered sudden abdominal distension without traumatic episodes. He had undergone total splenectomy for hereditary spherocytosis 13 years ago. He was in shock, and his hemoglobin level was 10.5 g/dl. Contrast enhanced computed tomography revealed a giant mass in the left upper abdomen and extravasation of the contrast material into the mass. Excision of the mass was performed, and microscopic examination showed a giant hematoma surrounded by normal splenic tissue. We speculated that an accessory spleen or splenosis had enlarged for the 13 years and ruptured. The patient remained asymptomatic 4 months after the surgery. Spontaneous hemorrhage from accessory spleens or splenosis is extremely rare, and relevant case reports suggest that surgical resection of bleeding sites yields favorable prognosis although preoperative qualitative diagnosis seems to be difficult. We often encounter accessory spleens in radiological studies, abdominal surgeries, or autopsies. Splenosis is also well known as autotransplantation of splenic tissue caused by splenic trauma or splenectomy. These kinds of ectopic splenic tissue are usually associated with no symptoms. We here present a very rare case which suffered life-threatening bleeding from splenic tissue without traumatic episodes although total splenectomy had been done 13 years ago, followed by a review of the literature.A 33-year-old Japanese man suffered sudden abdominal distension and visited our hospital by ambulance. He did not have any symptoms before the onset and denied histories of trauma. He had undergone cardiac surgery for arterial septal defect at 0 year old and total splenectomy for hereditary spherocytosis at 20 years old. The splenectomy was performed at another hospital, and the patient had not been clinically followed up since the surgery. He was 162-cm tall and weighted 75 kg . His blood pressure, pulse, oxygen saturation, and body temperature were 70/42 mmHg, 102 beats/min, 99 %, and 37.0 °C, respectively. His abdomen was considerably distended, and tenderness was observed at the left side of the abdomen. Laboratory examinations revealed intense acute inflammation , anemia , mild liver dysfunction , elevated levels of pancreatic enzymes , and mild renal dysfunction . Contrast-enhanced computed tomography revealed a heterogeneously enhanced abdominal mass with extension to 25 cm in the longest diameter; it ranged from an intramesenteric space of the transverse colon to the left upper quadrant of the abdomen in which the primary spleen would exist if splenectomy had not been performed. Ascites in Douglas’ pouch and extravasation of the contrast material into the mass in a portal phase were also observed (Fig. We performed urgent laparotomy to restrain the bleeding. Bloody ascites was observed. A great amount of hematoma was contained in the mesentery of the transverse colon and removed. Connected to the intramesenteric hematoma, a giant mass was observed at the left upper quadrant of the abdomen. It looked like just a hematoma or some kind of neoplasm and was firmly adhered to the pancreatic tail; it was excised with the pancreatic tail. The intraoperative hemorrhage volume was 5943 g, and 1820 ml of concentrated blood cells and 480 ml of fresh frozen plasma were transfused. On gross examination, the excised specimen showed a giant hematoma surrounded by gray hard parenchyma with a smooth capsule. Microscopic examination of the specimen revealed that the parenchyma was pathologically normal splenic tissue (Fig. We speculated that an accessory spleen which had survived the past splenectomy or splenosis which had spread to the abdomen at the splenectomy grew to a large size and ruptured for any reasons. After the surgery, the patient suffered pancreatic fistula which corresponded to grade B in the International Study Group of Pancreatic Fistula classification , recoverAccessory spleens occur due to the failure of the normal fusion of multiple nodules of splenic tissue originating in the dorsal mesogastrium during embryonic life . They haSplenosis means autotransplantation of splenic tissue; cells from the pulp of the damaged spleen spill out and grow as nodules of the splenic tissue , 4. TherStreptococcus pneumoniae and the immediate use of broad-spectrum intravenous antibiotics, ideally based on the result of blood cultures [Accessory spleens and splenosis are distinguished from those microscopic findings but some researchers insist that the distinction is actually difficult , 13. In cultures .99mTc sulfur colloid study, or fine-needle aspiration cytology in six cases [Spontaneous bleeding from accessory spleens and splenosis is extremely rare, and only 18 cases have been reported including this case , 18–33 (ix cases –25, 29 (ix cases , 26, 28 ix cases , 28. FouWe experience a case of spontaneous hemorrhage from splenic tissue 13 years after total splenectomy. The tissue is considered to be an accessory spleen or splenosis which had enlarged for the 13 years and ruptured. Spontaneous hemorrhage from accessory spleens or splenosis is extremely rare, and relevant case reports suggest that surgical resection of bleeding sites yields favorable prognosis although preoperative qualitative diagnosis seems to be difficult.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.


Tumor-induced osteomalacia (TIO) is a rare acquired form of hypophosphatemia commonly associated with phosphaturic mesenchymal tumors (PMTs) located in the bone or soft tissue. Resection of the tumor can cure osteomalacia. Fibroblast growth factor 23 has been identified as a major pathophysiological factor responsible for phosphaturia. The majority of PMTs are benign, and malignant PMTs are uncommon. Even in rare cases, the malignant transformation of PMTs is extremely uncommon. The current study presents two cases in which the patients succumbed to malignant PMTs that developed in the pelvis. The first patient was a 35-year-old female with a malignant PMT occurring as a synchronous double cancer associated with papillary thyroid carcinoma. Diagnosis was difficult, as the multiple uptake on positron emission tomography with 18F-fluorodeoxyglucose presented as pseudofractures mimicking the metastases of thyroid carcinoma. The patient succumbed to rapidly progressive lung metastases. The second patient presented with a pelvic tumor that had developed over 26 years. The patient was diagnosed with a benign PMT by open biopsy and a complete resection was performed. However, two years later, the tumor recurred and lung metastases were observed. The patient ultimately succumbed to respiratory failure due to relapsing lung metastases and disseminated intravascular coagulation. These two cases demonstrate the potential lethality of malignant PMTs and the malignant transformation of benign PMTs. Therefore, TIO patients must be followed up even if diagnosed with a benign tumor. Although TIO is an extremely rare disease, the possibility of malignant PMTs must be recognized. In 1947terature . TIO is trations –7. Surgis , abnormal uptake was observed in the right pelvis , left first rib, T2 vertebra and right lobe of the thyroid gland (2)D co- ncentrations. The tumor decreased in size after TAE had been performed twice. The serum phosphate and ALP levels were gradually normalized, and the multiple uptake on FDG-PET also disappeared, with the exception of the pelvic lesion, which indicated that this uptake was due to pseudofractures as opposed to malignancies. However, regrowth of the pelvic tumor and multiple metastases in the lung and bones were observed 32 months after the second TAE. In addition, leukocytosis , without C-reactive protein elevation, and a high level of granulocyte colony-stimulating factor  were observed. It is possible that the tumor had been transformed from an FGF-23-producing tumor to a G-CSF-producing tumor. Chemotherapy consisting of combined Adriamycin (55 mg/m2) and ifosfamide (8 g/m2), and combined gemcitabine (900 mg/m2) and docetaxel (75 mg/m2), was administered, however no effect was observed and the patient succumbed to rapidly progressive lung metastases.In March 2008, a 35-year-old female was referred to the Osaka University Hospital  with lower back pain. Laboratory analysis revealed high ALP levels , with severe hypophosphatemia (1.4 mg/ml), high thyroglobulin (Tg) levels (65.2 mg/ml), normal calcium levels (8.7 mg/dl) and normal parathyroid hormone levels (58.7 mg/ml). Upon physical examination, a mass was identified in the right neck, and a needle biopsy of the mass revealed a papillary thyroid carcinoma. On positron emission tomography with id gland . X-ray eid gland , and T2-id gland . The diaid gland . Immunolid gland . In addi2) and docetaxel (120 mg/m2), with no effect. The patient then developed metastases in the bilateral lungs, bones and liver. Upon pathological analysis, the diagnosis was of metastases from the PMT with malignant transformation. The patient underwent liver metastasis resection, followed by two courses of Adriamycin (59 mg/m2) and ifosfamide (7 g/m2), and two courses of Adriamycin (71 mg/m2) and cisplatin (76 mg/m2). A complete response of the lung lesion was achieved briefly, whereas the intrapelvic tumor continued to grow despite radiation therapy and TAE. In addition, the skin of the buttocks became thinner, ultimately forming a malignant ulcer. The patient succumbed to respiratory failure due to relapsing lung metastases and disseminated intravascular coagulation.In 1982, a 10-year-old male was originally diagnosed with hypophosphatemic osteomalacia of unknown cause. At 25 years old, an intrapelvic tumor was incidentally found, however, the patient did not undergo a detailed examination due to a lack of enlargement in the subsequent two years. In December 2003, at 31 years old, the patient visited the Osaka Koseinen-kin Hospital  due to worsening bilateral thigh pain and gait disturbance. A TIO was suspected and as a result, the patient was referred to the Osaka Medical Center for Cancer and Cardiovascular Diseases . The laboratory analysis revealed severe hypophosphatemia (1.3 mg/ml), accompanied by high ALP  and FGF-23  levels. X-ray examination revealed a bony destructive lesion, with calcification in the right ischium and ununited fractures in the shaft of bilateral femora, indicating Looser’s zones . In addiThe first case presented in the current study was difficult to diagnose, as the malignant pelvic PMT presented as a synchronous double cancer with thyroid carcinoma. TAE was selected, as the patient was unable to accept the functional impairment that would be a result of surgery. Although TAE safely achieved a temporary cytoreductive effect without massive release of FGF-23, 32 months after the second course of TAE, regrowth of the pelvic tumor and multiple metastases emerged. The patient succumbed to the rapid progression of the lung metastases.The second case remained undiagnosed for a long period, as TIO was not commonly recognized at the time. Therefore, when the patient was eventually diagnosed with TIO, the tumor was too large to perform a wide resection with a curative margin. Upon analysis of a biopsy, the initial diagnosis was of a benign PMT-MCT, however, two years after surgery, multiple metastases appeared and resection of the liver metastasis revealed malignant PMT-MCT.et al (Ogose et al  reportedet al –15. Howeet al –20. Howeet al (et al (With regard to chemotherapy, few studies have investigated the chemotherapeutic treatment of malignant PMT ,22. Seijet al  reported