Odontomas are the most common type of odontogenic tumors. They are included under the benign calcified odontogenic tumors. Odontomas are basically classified into two types, complex and compound odontomes. Various theories or etiological factors are been quoted for the occurrence of odontomes. Generally, they are asymptomatic. Occasionally, signs and symptoms relating to their presence do occur. The sole management depends upon the early diagnosis, histopathological examination and excision of these tissues. This article briefs regarding its classification, etiological factors, occurrence, differences between complex and compound odontomes, diagnosis and management. This is because odontomas result from the growth of completely differentiated epithelial and mesenchymal cells that give rise to ameloblasts and odontoblasts.5 In a broad sense, it means a growth with both the epithelial and mesenchymal components exhibiting complete differentiation resulting in functional ameloblasts and odontoblasts.5 These cells in turn form variable amounts of enamel and dentin and pulpal tissue of the odontoma.7 This enamel and dentin were usually laid down in an abnormal pattern because the organization of odontogenic cells failed to reach the normal state of mor-phodifferentiation.5 So they are considered as developmental anomalies rather than true neoplasm.The term odontome was coined by Paul Broca in 1867. Broca defined the term as tumors formed by the overgrowth or transitory of complete dental tissue.2 Approximately, 10% of all odontogenic tumors of the jaws are compound odontomas.89 The incidence of compound odontome ranges between 9 and 37% and the complex odontome is between 5 and 30%.10 Odontomas are discovered during the second and third decades of life.378 The compound odontoma is slightly more common than the complex odontoma which in turn is more common than the ameloblastic odontoma. The majority of odontomas in the anterior segment of the jaws are compound composite in type (61%), whereas the majority in the posterior segment is complex composite in type (34%).5 Interestingly both type of odontomas occurred more frequently on the right side of the jaw than on the left, .5 The compound composite odontome most frequently occurred in incisor cuspid region of the upper jaw in contrast to the complex odontome which were commonly found in molar and premolar region of the mandible.11112 Some reports have reported presence of both the types of odontomes in different locations, such as maxillary sinus, according to Bland Sutton (1988)113 in which 300 denticles were seen bilaterally, mandibular ramus,14 subcondylar region15 or mental foramen,13 mid palate16 and the middle ear.17 Hermann (1957) reported a case of compound composite odontome which consisted 2,000 denticles.1Odontomas constitute about 22% of all odontogenic tumors of the jaws.1819 Association of odontomes with the deciduous dentition is rare. Tratman (1949)20 thought that the deciduous dentition was not prone to the formation of odontomes. Saeed and Khalid noted presence of multiple odontomas in both maxilla and mandible in a female aged 7 years.20 In the review done by Katz,21 only 5 (2%) of 396 odontomas were associated with failure of a primary tooth to erupt.Odontomes commonly occur in permanent dentition  and are 5 It has been related to various pathological conditions, like local trauma, inflammatory and/or infectious processes, mature ameloblasts, cell rests of serres  or due to hereditary anomalies , odontoblastic hyperac-tivity, alterations in the genetic component responsible for controlling dental development.22 Hitchin suggested that odontomes are inherited or are due to a mutagene or interference, possibly postnatal, with the genetic control of tooth development.23The etiology behind odontomes remains unknown.23 and Atkinson (1949)24 have both advanced opinion that these growth pressures may be of importance in some composite odontomes. This pressure theory was advanced by Hitchin and Ferguson(1958)25 that this might have arisen from a developing lower premolar germ of an inherited large crown form embraced by the roots of its deciduous predecessor, producing a pressure effect.The appearance of odontomes is liable to occur due to growth pressures because of inadequate space which have varying effects on the tooth development. Euler (1939)23 So in case of any infection, the occurrence of odontome may be due to the divison of a tooth germ or may interfere with tooth development. This may be pathologically related or affecting the genetic control of tooth development.Infection from the deciduous predecessor may also be a factor, though it is not likely to be early enough when derived from the deciduous predecessor; but a more generalized infection may be of some significance. The effects on the dentition of prenatal infection with treponema palladium, in children whose mothers have been infected with rubella during pregnancy, acute maxillitis of infancy, that acute pyogenic infection of the whole maxilla occurring shortly after birth in which, when examined at 61/2 years, was found to have a compound composite odontome. This was due to pyogenic infection causing division of a tooth germ.26 suggested that these specialized cells have the potential of developing tumors with a wide variation in appearance and content.The etiology of odontomas is believed to have its origin from mature ameloblasts. Torreti et al27 stated that cell rests of serres  of the retained tooth with some epithelial island undergoing proliferation to develop into odontomes, while others underwent degeneration to form a cystic cavity enclosing the tooth for which the stimulus may be a genetic defect in the tooth forming process.Fijerskov830 However, the transition from one type to another is commonly associated with varying degree of morphodifferentiation or histodifferentiation or both, and it is often difficult to differentiate between both the types.31They suggested that when these buds are divided into several particles they may develop individually to become numerous closely positional malformed teeth or tooth-like structures. When the buds develop without such uncommon division and consists of haphazard conglomerates of dental tissues, they may develop into complex odontome.Previous history of trauma has been implicated in production of the lesion, as has interference with the genetic control of tooth development, either inherited or due to mutation or due to extensive damage of the tooth germ.32 describes an odontoma, like malformation of the permanent tooth germ, due to intrusive luxation or avulsion of the primary tooth. This malformation is rare sequela to injuries in primary dentition. The mechanism described by Andreasen is based on a history of a permanent tooth bud preemptive trauma. A vertically directed force through the long axis of the deciduous incisor was transmitted to the permanent tooth germ causing extensive damage. According to this theory, the malformation occurs during the early phase of odontogenesis and affects the morphogenetic stages of the ameloblastic development of the permanent tooth germ.32 Glasstone (1952)23 has shown that, if tooth germ of a rabbit is cut in half, each part develops in tissue culture into a complete rudimentary tooth, and Rushton (1957)23 has described a large nodule of enamel developing following trauma to a tooth germ before the completion of the enamel cap. Moreover, there are cases of odontome which appear to be due to the detachment of a portion of a tooth germ which may be from the epithelial sheath of Hertwig or from the enamel organ.23 In contradiction to the study by Hitchin, Levy33 showed that the pathological process is initiated only after amelogenesis has occurred. Levy produced a complex odontome by inducing trauma to developing first molars in rats. He further mentioned that whether trauma would produce hypoplasia, odontome or a supernumerary depended on the stage of development of the cells traumatized. In 1979, Shteyer, Taicher and Marmary15 reported a case of odontome occurring at the subcondylar region associated with a sinus tract linking to the third molar region. They deduced that the missing third molar had migrated to the subcondylar region followed by the occurrence of trauma or infection which led to the development of the odontome.Trauma to a developing tooth germ can also produce a hard tissue odontome. Andreasen (1994)The hypothesis regarding the etiology of the hard tissue odontome is that they are either inherited or due to a mutant or interference, possibly postnatal, with the genetic control of tooth development. Odontome can occur in one or more of three ways: By interference with the mechanism whereby genes control tooth formation and form By a mutation in the genes concerned By inheritance of those abnormal genes.24Hitchin suggested that a mutation in the epithelial cells of the tooth germ may change the inherent capacity of odontogenic epithelium to go through the cap and bell stages necessary for tooth formation, and yet retain its ability to stimulate mesenchymal differentiation necessary to form functional ameloblasts and odontoblasts, leading to the formation of an odontoma.34 suggested that the differentiation of normal and tumor odontogenic cells is accompanied by the expression of some molecules. The gene products present in some mesenchymal cells were also seen in the odontogenic tumor epithelium. The data may be related to a tumor-specific overexpression of the corresponding genes transcribed at an undetectable level during normal development and/ or to an epithelial-mesenchymal transition proposed to occur during normal root formation. A plausible explanation for the result is that odontogenic tumor epithelial cells are recapitulating genetic programs expressed during normal odontogeneis, but the tumor cells demonstrated abnormal expression patterns for these genes.Papagerakis et al10 put forth the hypothesis that formation of a compound odontome is pathogenically related to the process producing hyperdon-tia, multiple schizodontia or locally conditioned activity of dental lamina.There are many unanswered questions about the origin of the odontomes. The occurrence is not necessary with one factor but it may be associated with multiple factors. It has been suggested that trauma and infection at the place of the lesion can offer ideal conditions for its appearance. This lesion is one of the odontogenic origin and is considered a self-limiting anomaly. Recently, Philipsen et alSo growth pressures, trauma, infection, mature am-eloblasts, cell rests of serres , extraneous odontogenic epithelial cells may be regarded as sources of disturbances in the mechanism of development. They are also guided by mechanism where genes control tooth development.1After Brocas (1866) first attempted to classify odontomas according to the stages of tooth development, many classifications were proposed according to structural tissues from which tumor arouse. 35 They are as follows:One of the most common classification is given by World Health Organization (WHO). Four lesions containing enamel and dentine of normal appearance are defined in the WHO classification.Ameloblastic fibro-odontome: Consists of varying amounts of calcified dental tissue and dental papillalike tissue, the latter component resembling fibroma. The ameloblastic fibro-odontome is considered as an immature precursor of complex odontomeOdonto-ameloblastoma: Its a very rare neoplasm which resembles an ameloblastoma both structurally and clinically but contains enamel and dentineComplex odontome: When the calcified dental tissues are simply arranged in an irregular mass bearing no morphologic similarity to rudimentary teethCompound odontome: Composed of all odontogenic tissues in an orderly pattern that results in many teeth-like structures but without morphologic resemblance to normal teeth.892836, two types of odontoma are recognized: (a) compound and (b) complex.On the basis of gross, radiographic and microscopic features337, in 1914, Gabell, James and Payne grouped odontome into three types:On the basis of their developmental origin Epithelial Composite  and Connective tissue.3839According to their position within the jaws:Intraosseous (erupted odontoma): They occur inside the bone and may erupt into the oral cavity. To date, 12 cases of the erupted variety have been described in the literatureExtraosseous or peripheral odontomas: These are odontomas occurring in the soft tissue covering the tooth bearing portions of the jaws, having a tendency to exfoliate.4041According to Thoma and Goldman (1946): Germinated composite odontomes―two or more, more or less well-developed teeth fused together Compound composite odontomes―made up of more or less rudimentary teeth Complex composite odontomes―calcified structure, which bears no great resemblance to the normal anatomical arrangement of dental tissues Dilated odontomes―the crown or root part of tooth shows marked enlargement Cystic odontomes―an odontome that is normally encapsulated by fibrous connective.3637Z Gorlin et al eliminated the term composite as redundant and classified odontomas as either complex or compound. There are essentially two types of odontome: Complex composite odontome Compound composite odontome.A new type known as hybrid odontome is also reported by some authors.42 in 1952, in his classification restricted the term odontome for those tumors which aroused from both epithelial and mesenchymal dental forming tissues. But presently, this term is used in a very restricted sense to designate only those tumors which consist of dental hard tissues.According to Robinson,Compound Odontome These are the malformations in which all dental tissues are represented in a more orderly pattern, so that the lesion consists of many tooth like structures or denticles composed of enamel, dentin , cementum and pulp. It is a tumor of enamel and dentin arranged in the form of anomalous miniature teeth. Several small abnormal teeth surrounded by a fibrous sac.Complex Odontome These are the malformation in which all dental tissues are represented but not in an organized form or disorderly pattern. It is an odontogenic tumor characterized by the formation of calcified enamel and dentin in an abnormal arrangement because of lack of morphodifferentiation.5 Compound odontomas seldom cause bony expansion but complex odontomes often cause slight or even marked bony expansion.2829 The presence of odontomas may lead to malpositioning or displacement of adjacent teeth, aplasia, malformation and devitalization of adjacent teeth.43.Most of the odontomes are asymptomatic, although occasionally signs and symptoms relating to their presence do occur. These generally consist of unerupted or impacted teeth, retained deciduous teeth, swelling and evidence of infection.5 A differential diagonosis is usually made through comparison of the degree of morphodifferentiation and histodifferentiation of the dental hard tissue.3Majority of odontomes are diagnosed most commonly during routine radiographic examination. A developing odontoma can be detected by routine radiography but may cause difficulty in identification due to lack of calcification.31 In comparison to visual examination and manual palpation, radiographic examination seems to be the most effective clinical method of discrimination between two types.44 In case of compound odontoma, radiographic image shows comparatively well-organized malformed teeth or tooth-like structures, usually is a radiolucent cyst like lesion. A complex odontoma shows an irregularly shaped oval radiopacity usually surrounded by a well-defined thin radiolucent zone. In case of compound odontoma in which extremely small, conglomerated malformed teeth or tooth-like structures are numerous, the radiographic image is similar to those obtained with complex odontomas and a differentiation between the two types may be difficult.2930A visual examination of the lesions cannot by itself define the differences between the complex and the compound types, because the odontomas are usually in the bone structures and do not show outward signs, such as expansion of the bone. Even in rare instances in which odontomas erupt into the oral cavity and can be examined visually and manually, the surface appearances of both types of odontoma are similar and differentiation between them is difficult.82845 However, because of the presence of transitions between types, even a histo-logic examination might not make a definite diagnosis possible.31 Microradiography is another useful procedure in the effort to establish a definite diagnosis. It enables histologic structures to be recognized from their various radiopacities and or radiolucencies. In addition, the resolution of microra-diography is equal to that obtained in histological examina-tion.46 Masayuki Kaneko (1998)46 et al showed in his case report that the low magnification radiograph showed the structural differences as clearly as did the histologic examination. High magnification microradiographs demonstrated even the finest dentinal tubules in detail, in correspondence with the histological images. So microradiographic findings showed that microradiographic images are not inferior to images obtained through hsitologic procedures.So conventional radiography cannot always demonstrate details of difference. Because of poor image resolution, neither visual nor radiographic examinations showed the morphologic features necessary for diagnosis. To establish a definite diagnosis, some other procedure must be used; most commonly, histologic examination.The diagnosis of odontomas cannot be made by visual or manual techniques. It has to be done in coordination with radiographic as well as histological examination. The advance technique, such as microradiography, should be used to confirm the diagnosis.40 Because of the very low recurrence, the treatment of choice is surgical removal of the lesion. As it is a capsulated tumor, its removal is a simple surgical procedure but special care should be taken to remove it totally in order to avoid a relapse which is specially critical in immature complex odontomas. Odontomas are easily enucleated and adjacent teeth that may have been displaced are seldom harmed by surgical excision because they are usually separated by a septum of bone. But sometimes due to extension of the odon-tomes, the adjacent tooth may be disturbed while removal of the odontomes.47Odontoma has a limited growth potential, but it should be removed because it contains various tooth formulations that can predispose to cystic change, interfere with eruption of permanent teeth and cause considerable destruction of bone.A thorough visual, manual as well as radiographic examination should be performed for all the pediatric patients who present with clinical evidence of delayed eruption, missing tooth or temporary tooth displacement, with or without history of trauma. Early diagnosis of odontomas helps us to: Adopt a less complex and less expensive treatment Ensures better prognosis Avoid relapse of the lesion Avoid displacement or devitalization of adjacent tooth.


A 65-year-old man presented by a progressive scalp mass from 9 months before. Physical examination showed a huge mass on his skull  associatNeurological examination was normal. The laboratory data and preoperative evaluation was normal, too. The brain computed tomography (CT-scan) and magnetic resonance imaging (MRI) demonstrated a huge extra-axial mass in right parietal bone resulted to bone destruction and scalp invasion  and 3. In surgery, there was a huge oval-shape hemorrhagic and firm mass associated with scalp invasion and bone destruction . After skin incision, the mass was dissected from scalp and debulked; parietal craniectomy around the lesion was done; dura was opened and tumor was dissected from neural tissue and was resected totally. Helping synthetic patch and titanium mesh, the dura and skull were repaired. Histopathology revealed renal cell carcinoma (RCC).1Sepulveda, et al. reported a 62-year-old man presented with a large expansive and destructive mass within cranial base associated with bilateral internal carotid artery involvement. Incisional biopsy revealed clear cell adenocarcinoma in favor of renal tumor metastasis.2Rekhi, et al. reported a 15-year-old girl presented with a painless swelling in the occipital region. She had a history of excisional biopsy with a diagnosis of an alveolar soft part sarcoma. The lesion recurred within one month. CT-scan revealed a vascular destructive skull mass compressed the cerebellum. Metastatic RCC was ascertained after excisional biopsy and systemic work up.3Yeh, et al. presented an 80-year-old man admitted for an incidental mass on the left parietal. The CT-scan revealed a destructive soft tissue lesion in the left parietal with intracranial and scalp invasion. Needle biopsy demonstrated metastatic RCC.4Koutnouyan, et al. reported a 33-year-old woman who was referred for a mass in forehead region as a benign lesion. Because of intraoperative severe bleeding and presence of bone erosion, only biopsy rather than resection was done. Histopathology and metastatic work-up demonstrated metastatic RCC.A metastatic lesion should be in differential diagnosis of a destructive skull lesion with brain and scalp invasion, although it occurs rarely.


This is a rare case of giant lumbar pseudomeningocele with intra-abdominal extension in patient with neurofibromatosis type 1 (NF1). The patient's clinical course is retrospectively reviewed. A 34-year-old female affected by NF1 was referred to our institution for persistent low back pain and MRI diagnosis of pseudomeningocele located at L3 level with paravertebral extension. From the first surgical procedure by a posterior approach until the relapse of the pseudomeningocele documented by MRI, the patient underwent two subsequent posterior surgical procedures to repair the dural sac defect with fat graft and fibrin glue. One month after the third operation, the abdominal MRI showed a giant intra-abdominal pseudomeningocele causing compression of visceral structures. The patient was asymptomatic. The pseudomeningocele was treated with an anterior abdominal approach and the use of the acellular dermal matrix (ADM) sutured directly on the dural defect on the anterolateral wall of the spinal canal. After six months of follow-up the MRI showed no relapse of the pseudomeningocele. Our case highlights the possible use of ADM as an effective and safe alternative to the traditional fat graft to repair challenging and large dural defects. Pseudomeningoceles are extradural collections of cerebrospinal fluid (CSF) with no dural covering resulting from a dura-arachnoid defect or improper closure during spinal surgery . There aThis is the case of 34-year-old female with neurofibromatosis type 1 and scoliosis who underwent surgery for low back pain and MRI diagnosis of pseudomeningocele at L3 level with paravertebral extension Figures . The neuNeurofibromatosis type 1 (NF1) is a genetic multisystemic disorder involving skin, central and peripheral nervous systems, bones, and cardiovascular and endocrine systems. This condition is caused by inherited or de novo mutations of the NF1 gene at the 17q11.2 chromosomal region, a gene that codes for the protein neurofibromin , 9. Know


Retropharyngeal space schwannomas are rare entities. About 20-45% of schwannomas occur in the head and neck region; however, they represent less than 1% of all head and neck tumors. We present the case of a 36-year-old woman complaining of dysphagia caused by a large schwannoma arising in the posterior pharyngeal wall with remarkable reduction of the oropharyngeal space. The tumor was resected through a combined transoral and cervicotomic transmandibular approach due to its dimension. No recurrence was observed after a two-year follow up. This case represents the thirteenth case reported in international literature. Preoperative settings for rare tumors such as retropharyngeal schwannomas should include radiological investigations and preoperative biopsy. In order to obtain a successful result in terms of radicality, a combined surgical approach may be necessary to completely control the extension of the lesion. Schwannomas are uncommon benign neurogenic tumors, arising from the sheath of the Schwann cells. They may originate from any neural structure in the head and neck region . Most scA 36-year-old woman was admitted to our department with a 3-year history of a foreign body perception in the throat with gradually progressing dysphagia, associated with occasional odynophagia. A significant weight loss (6 kg) in the previous 2 months occurred. Physical examination revealed an anterior bulging of the left posterior pharyngeal wall due to the presence of a large, smooth mass, with central mucosal ulceration, causing a remarkable reduction of the oropharyngeal space .The endoscopic evaluation pointed out the extension of the mass from below the left Eustachian tube to the omolateral piriform sinus. Nasal cavities were free and morphology and functionality of the larynx were preserved. Neck examination was otherwise normal. Magnetic resonance (MR) showed a well-defined encapsulated tumor in the left retropharyngeal space (size 70 mm x 40 mm x 30 mm), with high signal intensity on T2-weighted sequences and isointensity on T1-weighted, which was strongly enhanced by gadolinium administration . The mass, with predominant submucosal development, was extending into the retropharyngeal space from the nasopharynx to the hypopharynx. An angio-CT scan was performed before the main surgical procedure, to evaluate the relationship between the tumor and the main vascular structures of the neck. A posterior displacement of the external carotid artery with an apparently preserved dissection plane was observe. The internal carotid artery was not in contact with the lesion. A biopsy, was performed under general anesthesia in order to define the lesion’s nature, which was diagnosed as a schwannoma. The patient underwent a combined transoral and cervicotomic transmandibular approach. The tumor possessed a plurilobulated aspect, bulging out in the oropharynx and developing in the retropharyngeal depth. Dissection was performed accurately and the lesion was removed in en-block throughout the oral cavity . Tracheotomy was performed temporarily in order to prevent eventual postoperative dyspnea and was closed in 8 days later. Tumor size was 60 x 55 x 32 mm and the cross-section showed a “fish flesh” soft tan appearance. Histologically, the tumor was highlighted by a fibrous capsule composed of spindle cells with focal palisading of the nuclei arranged in short bundles and fascicles, which raise suspicion for schwannoma. The patient was fed through a feeding tube for 7 days and was dismissed 12 days after surgery, with a normal diet. No recurrence was observed during the two years of follow-up.th, Xth, XIth, XIIth cranial nerves or the cervical sympathetic chain . . th, XthDiagnosis is challenging since in the retropharyngeal space, which contains essentially fat and lymph nodes, the most frequent diseases are metastasis from primary head and neck cancer and lipomas, neuroblastomas and pleomorphic adenomas (13). MR with gadolinium enhancement is particularly helpful in defining schwannomas. Usually these tumors have a low signal intensity on T1-weighted sequences, a high signal intensity on T2-weighted sequences, and a strong enhancement after gadolinium administration. No flow voids are seen in these tumors . HoweverThe determination of the nerve of origin with imaging studies, before the surgical exploration, still remains difficult in many cases. Various authors described the criteria for the determination of the nerve’s origin in the parapharyngeal space on CT or MR findings . HoweverThe treatment of schwannoma is based on a radical surgical resection with the preservation of nerve function, except when the size or the location of the tumor does not allow for a complete excision, forcing only a partial removal or sacrifice of the nerve.However, in view of the fact that schwannomas are separated from nerve fibers by a fibrous capsule, in contrast to neurofibromas in which the nerve is an integral part of the neoplasm and must be sacrificed in order to excise the tumor, in most cases it possible to enucleate the tumor with the preservation of the nerve . Many surgical procedures have been described, depending on the site and the size of the tumor, such as trans-oral, trans-cervical and trans-parotid approaches, variously combined .Retropharyngeal schwannomas are uncommon diseases with a challenging diagnosis. Radiological examinations are mandatory in order to define the non-invasive and avascular nature of the lesion. A preoperative biopsy has a diagnostic significance and should be taken into account. In order to obtain a successful result in terms of radicality, a combined surgical approach may be necessary to completely control the extension of the lesion.


Rectus sheath hematoma is a rare clinical diagnosis, particularly in pregnancy. Due to unspecific symptoms, misdiagnosis is likely and could potentially endanger a patient as well as her fetus.A 26-year-old white woman presented with mild right-sided abdominal pain, which increased during palpation and movement, at 26 + 3 weeks’ gestational age. Ultrasound imaging initially showed a round and well-demarcated structure, which appeared to be in contact with her uterine wall, leading to a suspected diagnosis of an infarcted leiomyoma. However, she reported increasing levels of pain and laboratory tests showed a significant drop in her initially normal hemoglobin level. A magnetic resonance imaging scan finally revealed a large type III rectus sheath hematoma on the right side. Because of progressive blood loss into her rectus sheath under conservative therapy, with a significant further decrease in her hemoglobin levels, surgical treatment via right-sided paramedian laparotomy was initiated. During the operation the arterial bleed could be ligated. She eventually achieved complete convalescence and delivered a healthy newborn spontaneously after 40 weeks of gestation.This case report highlights the clinical and diagnostic features of rectus sheath hematoma and shows the anatomical aspects of the rectus sheath, simplifying early and correct diagnosis. Rectus sheath hematoma (RSH) is a rare condition and only a few cases during pregnancy have been reported . RSH reset al. reviewed ten cases of spontaneous RSH in pregnancy. In 50 % of these cases, immediate caesarean section was performed, eventually leading to preterm deliveries .–5.3–5].The blood supply is provided by the epigastric arteries, which run longitudinally within the rectus sheath Fig.  6, 7]., 7.6, 7]RSHs are mostly caused by trauma of the inferior epigastric artery. Whetzel and Huang  showed aRisk factors include conditions that elevate intraabdominal pressure, such as coughing fits, severe vomiting, or straining during passing a stool, but also trauma, hereditary or iatrogenous coagulation abnormalities, vascular malformation, female sex, and previous surgery .Symptoms of profuse bleeding into the rectus sheath are often relatively unspecific and may include sudden or increasing – often unilateral – abdominal pain, palpable masses in the abdominal wall, and general conditions of a hypovolemic state, such as tachycardia, hypotension, tachypnea, and pallor. A clinical examination may show Fothergill’s sign , Carnett’s sign , Cullen’s sign , or Turner’s sign (flank ecchymosis). Furthermore, low-grade fever, nausea, and vomiting have been reported in patients with acute RSHs –13.The risk of misinterpreting the aforementioned symptoms is elevated during pregnancy and correct diagnosis may be difficult. Differential obstetric diagnoses include acute abruptio placentae, infarcted myoma, preterm labor, uterine rupture, or HELLP syndrome. There are also multiple non-pregnancy-related differential diseases, such as gastroenteritis, nephritis, nephrolithiasis, perforated ulcer, pancreatic disease, hepatitis, gallbladder disease, acute appendicitis, ovarian torsion, or an intraabdominal bleed , 14, 15.Ultrasound poses a viable diagnostic feature in RSH. It can be used as a first-line test and is also secure in pregnant patients. RSHs, located above the arcuate line, often occur as a unilateral spindle-shaped mass. Underneath the arcuate line hematomas commonly appear spherical , 16. Labet al. found a diagnostic classification of RSH in CT scans [In non-pregnant patients, a computed tomography (CT) scan is the diagnostic gold standard as it enables a sensitivity and specificity of 100 %. Berna CT scans . AccordiCT scans . To avoiCT scans , 19.et al. reported 7 out of 10 cases (70 %) of antepartum RSH in which surgical evacuation was performed [Conservative treatment, including analgesic therapy and fluid resuscitation, is usually possible in hemodynamically stable patients with smaller RSHs. These hematomas will normally resolve themselves within a few weeks , 21. PatRSH is a serious and potentially life-threatening condition, which presents with acute abdominal pain. As in the present report, unspecific symptoms of an acute RSH may mimic several other acute conditions and can easily lead to a wrong diagnosis. To prevent a hemorrhage, which potentially endangers mother and fetus or leads to preterm labor, early diagnosis is important .Diagnosis may be reached by transabdominal ultrasound; however, correct interpretation and location of the multiple compartments of the abdominal wall, especially in pregnant women, may be difficult , 19. AltAfter diagnosis, a conservative therapy should be conducted. If the patient presents with hemodynamic instability and a profound bleeding is detected, invasive therapy is indispensable and reduces mortality and morbidity for the mother and the fetus  Fig. 3)3).Fig. 3Several cases in the literature report uneventful, spontaneous vaginal delivery after RSH , 23. AltWe present a case of a woman with RSH during pregnancy, which initially appeared as a pedunculated myoma. Our cadaver study highlights the anatomical features of the anterior abdominal wall, explaining the pathophysiology of this disease. In conclusion, RSH is a rare diagnosis during pregnancy but should always be considered a potential differential diagnosis of abdominal pain. In case of unspecific symptoms early imaging  should be taken into consideration as an early and correct diagnosis significantly reduces maternal as well as fetal morbidity and mortality.


Intraspinal synovial cyst is a rare cause of myelopathy. These cysts present most often in the lumbar and cervical parts of the spine but are more infrequent in the thoracic spine. We present a case of a 73-year-old man with an intraspinal, extradural synovial cyst at the Th2-3 level causing paraesthesia and weakness in the legs. A laminectomy and excision of the cyst were performed and the patient recovered fully. In the thoracic spine, synovial cysts are almost exclusively found in the lower part. Laminectomy, with excision, is the treatment of choice, although steroid injections have been described. Intraspinal synovial and ganglion cysts are a rare cause of spinal cord compression . They ofA 73-year-old man, with a past history of autoimmune hepatitis, presented to the hospital with progressing neurological symptoms. He had, in over a period of two months, developed ascending paraesthesia, abnormal gait, weakness in the legs, and trouble controlling vesical and anal sphincters. Physical exam revealed paraesthesia at the level of the nipple (Th-4 level) and in the legs. In the lower extremity there was bilateral weakness at the hip and knee joint as well as dorsal flexion of the foot.Suspecting spinal stenosis an MRI of the spine was ordered. This showed a 4 × 8 mm intraspinal, extradural cystic process at the Th2-3 level on the right side Figures  and 2. TThe patient was started on oral prednisolone 75 mg per day and transferred to Iceland for surgery. A decompressive laminectomy was performed and the cyst was excised. The operating surgeon found the operative material congruent with a synovial cyst; it was however not sent for pathological study. The patient had immediate improvement of the symptoms. Within a few days he was able to walk unassisted. Over a period of six weeks he regained his full strength; only the paraesthesia remained.The patient was seen 2.5 years after surgery where he reported that the paraesthesia had remitted completely but it had taken a couple of years.We present a case of an elderly man, with no history of degenerative disease of the spine or trauma, with a cyst in the upper part of the thoracic column. It is unclear how long the patient had the cyst; probably it could have been there for years. He presented with symptoms progressing over a period of a few weeks, primarily paraesthesia with some weakness of the lower limbs.Intraspinal cysts occur mostly in the lumbar segments; they are very rarely seen in the thoracic part of the spine  and accoTo our best knowledge only 25 cases describing synovial or ganglion cysts in the thoracic region have been reported , 2, 4–8.The prevalence of intraspinal cysts is to our knowledge unknown. There are many case reports and case series, but no long-term observational studies. It is unlikely we will ever know the true prevalence, because of the rarity of the condition and the fact that an MRI is required to diagnose it.The treatment for synovial cysts consists mostly of a decompressive laminectomy and excision of the cyst. In a few cases conservative treatment has been tried. In one series four patients had significant improvement with steroid injections, although the relief was only short term. In one case the cyst regressed completely with injection; however pain persisted due to degenerative disease . With suIntraspinal synovial cysts of the thoracic spine are a very rare cause of medullary compression. They are mostly found in the lower part of the thoracic column. MRI of the spine is the gold-standard imaging modality for both diagnostics and planning the surgical procedure. Most often the treatment consists of laminectomy and excision, but conservative treatment with steroid injection has been described. Clinicians should be aware of this rare cause of paraesthesia and weakness of the lower limbs. Acute neurosurgical intervention can reduce the risk of irreversible neurological damage of the medulla spinalis.


Splenic rupture following colonoscopy is rare, first reported in 1974, with incidence of 1–21/100,000. It is critical to anticipate splenic trauma during colonoscopy as one of the causes of abdominal pain after colonoscopy especially when located in the left upper quadrant or left shoulder. Postoperative adhesions is a predisposing factor for splenic injury, and management is either operative or nonoperative, based on hemodynamic stability and/or extravasation which can be seen on contrast-enhanced CT scan of the abdomen. We present a case of a splenic rupture after colonoscopy in a patient with splenocolic adhesions, requiring splenectomy as definite treatment. Colonoscopy is a reliable and widely used procedure for the diagnosis and management of colorectal disorders, but it is not without its risks, with the commonest being bleeding and perforation, with estimated incidences of 1.8–2.5% and 0.34–2.14%, respectively [51-year-old female presented to our emergency room with abdominal pain, one day after a routine screening colonoscopy done in an outside facility. Patient had colonoscopy done in the morning of the previous day and by evening she noticed a sharp left upper quadrant abdominal pain with radiation to the left shoulder, which is aggravated by respiration. Her medical history is remarkable for hypertension, diabetes, and obstructive sleep apnea. She had colonoscopy in 2009 for iron deficiency anemia and hysterectomy done in 2010. In the emergency room, she had a blood pressure of 124/67 mmhg and pulse rate of 75 bpm; respiratory rate was within normal limits. Her laboratory results revealed hemoglobin of 11.9 g/dl, white cell count of 11.1 k/ul ml, platelets of 221 k/ul ml, INR of 1.0, BUN of 14 mg/dl, and creatinine of 0.7 mg/dl. Physical examination revealed no abdominal distension, but tenderness of the left upper and lower quadrants, guarding but no rebound. Chest radiograph did not reveal any pneumothorax or air under diaphragm. Computerized tomography (CT) of the abdomen revealed a 4 × 7 cm perisplenic/subcapsular hematoma with an area of active bleeding along the subcapsular region and possible splenocolic adhesions . Management of splenic injury may be one of 3 therapeutic options: conservative, surgery, or embolization of the splenic artery, based on patient's hemodynamics and ongoing extravasation.


A 41-year-old woman presented with an asymptomatic left infraorbital mass that had continuously grown over a 4-year period. A magnetic resonance image showed a retroseptal mass without involvement of the neurovasculature or intrinsic musculature of the eye. Excisional biopsy showed a well-circumscribed, benign vascular proliferation, consistent with a cavernous hemangioma or cavernous venous malformation.What is a cavernous-hemangioma or cavernous venous malformation of the orbit?How do orbital cavernous venous malformations present?What are some differential diagnoses of orbital cavernous malformation?What are the current diagnostic tools and treatment options for orbital cavernous malformations?1 Typically, these malformations are classified as noninfiltrative and slow growing at a rate reported as 10% to 15% increase per year. Although the nomenclature is often confusing, the preferred term of these lesions is “cavernous venous malformations.”2 Histologically, dysplasia or hypercellularity is not seen and only show features of slow flow venous lesions that are typically lined by a single layer of endothelium and composed of large, thin vessels. They are differentiated from infantile hemangiomas histologically due to the adult form lacking in GLUT-1.2A cavernous venous malformation or cavernous hemangioma of the orbit represents approximately 6% of intraorbital/retrobulbar masses of the orbit. Comparatively, vasculogenic masses represent 17% of orbital masses.4 In our patient, these venous malformations presented asymptomatically and caused only a deformity of the extraocular tissues, bringing it to the patient's attention.Orbital cavernous venous malformations typically present in middle-aged individuals and will cause mass-effect symptoms such as proptosis, pain, diplopia, and visual disturbance by compression of the optic nerve. They may also be completely asymptomatic/found incidentally on imaging.1Imaging helps establish a differential diagnosis of numerous benign and malignant masses. The differential diagnosis for orbital cavernous malformations is broad and includes various types of cysts, other vasculogenic lesions, peripheral nerve lesions, optic nerve and meningeal tumors, osseous versus lipocytic lesions, etc. Vasculogenic lesions with flow such as a carotid or dural cavernous fistulas, capillary hemangioma, or lymphangioma must be evaluated by imaging to prevent complication and to plan the surgical approach.5 , especially with the use of contrast dye that allows for the enhancement of the hemangioma. However, it is common for patients to sustain both magnetic resonance imaging (MRI) and CT as our patient did5 . Color D7 (5 Gross examination of the patient's mass showed a tan-red, well-circumscribed mass in a fibrous membrane and measured 1.5 × 1.4 × 0.4 cm (Treatment of orbital cavernous venous malformations can be either a nonsurgical method or various surgical excision methods. Location of the lesion also determines the recommended surgical approach. Nonsurgical methods may be indicated for small asymptomatic nonenlarging masses; however, surgical excision is required for definitive diagnosis. Lateral orbitotomy, supraorbital, transconjunctival, transantral, pterional, endoscopic, and extradural approaches have all been described as surgical approaches7 . The rat× 0.4 cm .


Schwannomas origin from Schwann cells sheath and generally are benign, slow-growing, and asymptomatic neoplasms which frequently appear in the head and neck. Although gastrointestinal schwannoma is really rare, the most affected organ in GI system is the stomach. Gastric schwannoma forms 0.2% of all gastric tumors. This neoplasm is always detected as a submucosal mass, the same as other gastrointestinal stromal tumors. Although these tumors have almost the same presentations, they are completely different at therapeutic options and prognoses. Hence, it is important to distinguish them apart and make an accurate diagnosis to optimize treatment outcomes. Herein, we report a case of 28-year-old woman with frequent vomiting and abdominal pain caused by 5 × 6 cm schwannoma in the antrum of the stomach. This is a rare case of gastric outlet obstruction due to a massive schwannoma. In addition, all other probable submucosal masses will be discussed at different aspects. Submucosal tumors (SMTs) include a wide spectrum of benign-to-malignant lesions. SMT employs to define the intramural neoplasms underneath the mucosa. The incidence of SMTs in the whole gastrointestinal (GI) tract is unknown. However, the stomach is frequently the most affected organ . GastricIn this case report, a 28-year-old woman with a 5 × 6 cm mass in the antrum of the stomach will be presented. She was complaining of frequent vomiting (sometimes bloody) and abdominal pain. After the surgery, the complaints were relieved and histopathological and immunohistochemistry assessments revealed a gastric schwannoma. In addition, other SMTs, as differential diagnoses, will be discussed at different aspects.A 28-years-old woman was admitted to our emergency department due to abdominal pain, recurrent vomiting, and hematemesis. The patient had been suffering from abdominal pain for several weeks, and the symptoms gradually have worsened until the time of admission when she had severe anorexia and recurrent vomiting. She did not have any systemic and local complication, and the physical examinations did not reveal anything special. After the admission, the patient underwent diagnostic endoscopic study which showed a 5 cm × 6 cm antral submucosal mass with central umbilication. The mass was causing gastric outlet obstruction. Double contrast-enhanced abdominopelvic CT scan was performed and revealed a hypodense mass along the anterior wall of the gastric antrum with subsequent pyloric stenosis and luminal obstruction with gastric dilatation in the body and fundus . PossiblPathological examinations were done, and characteristic histological findings for gastric schwannoma were detected . The tumThe SMTs are divided into three major categories including myogenic tumors (leiomyomas or leiomyosarcomas), neurogenic tumors , and GISTs .Gastrointestinal neurogenics derive from different components of nerve fibers, mostly autonomic components of Auerbach's plexus . They coMyogenic tumors include leiomyomas and leiomyosarcomas which are rare and can be found anywhere along the GI tract. However, they are more common in the esophagus, stomach, and colon and arise in muscularis mucosae or muscularis propria .GIST is the most common kind of submucosal mesenchymal neoplasms of the GI tract. The incidence of GISTs is 10–20 per million and the stomach is the most common location. Approximately 60% to 70% of GISTs arise in this organ and form 0.1%–3.0% of all GI malignant tumors , 14. GISGastric SMTs frequently are so small and noninvasive to become symptomatic. According to a report by Perea and Gregory, abdominal pain is the most common symptom. GI bleeding, anemia, epigastric mass, nausea, and vomiting can also occur. Dyspeptic symptoms, anorexia, and epigastric tenderness are among rare symptoms , 17. AboIt is obvious that clinical symptoms are nonspecific, and distinction of gastric submucosal masses with clinical presentation is approximately impossible. The standard procedures for establishing diagnoses in patients with GI tumors are endoscopic mucosal forceps biopsy. However, its false negative rate can be as high as 50%. Therefore, the endoscopic ultrasound (EUS) and EUS-guided biopsy (EUS-B) would be helpful as the most accurate procedures for definite diagnosis of GI tumors with negative endoscopic biopsies. EUS shows typical findings for some lesions, but in hypoechoic lesions, such as leiomyomas, GISTs, and schwannomas, EUS findings are not enough and EUS-Bs are needed. Due to deeper location than epithelium, biopsies cannot be taken from the subepithelial lesions by conventional endoscopic methods, and EUS-B should be employed , 23.Also, imaging techniques are needed and computed tomography (CT) is a more common diagnostic tool for these cases. The CT scan presentation of GISTs depends on its size, aggressiveness, and the course of the disease. Primary GISTs are mostly large hypervascular lesions which exhibit as enhancing masses at contrast-enhanced CT scans with heterogeneous presentations due to necrosis, hemorrhage, or cystic degeneration , 25. In Although imaging techniques are helpful, histopathological analyses should be established to confirm the diagnosis . The neuImmunohistochemical analysis should be done to approve the probable diagnoses in all SMTs. GISTs are mostly considered CD117-positive, generally CD34-positive, actin-positive, and S100-negative , 31, 37.In our case, the tumor cells were negative for CD117, CD34, and actin while were positive for S100. Therefore, GIST and myogenic tumors were excluded, and histological pattern of lymphoid aggregation plus capsule presence highly suggested schwannoma rather than neurofibroma.This case was operated due to relief of obstruction symptoms with a probable diagnosis of GIST. In the course of surgery due to the presence of the 5 cm × 6 cm mass in the lesser curvature of gastric antrum and to obtain a 2 cm safety margin for the mass resection, subtotal gastrectomy was evitable. The lesser curvature masses resection causes vague nerve eradication and subsequent pylorus dysfunction. Therefore, for the lesser curvature masses, subtotal gastrectomy is the choice plan. In addition, subtotal gastrectomy is the treatment of choice for middle- and distal-third gastric neoplasm . Therefo


Congenital bilateral ectropion of the upper eyelids is a rare, benign condition reported in ophthalmic literature. It is more frequently associated with Down’s syndrome, ichthyosis, and sporadic cases in newborns from black population. We report three cases of congenital bilateral upper eyelid ectropion associated with Down’s syndrome. Management of these patients usually requires medial and lateral canthoplasties, full-thickness pentagonal resection of the upper eyelids and placement of skin grafts. We present herein the evolution of one of these patients and we will discuss the mechanism of the eyelid ectropion and its treatment. Congenital bilateral ectropion of the upper eyelids is a rare, benign condition , 2], , , [3], 44. Its etAn eight-month-old boy with clinical signs and diagnosis of Down’s syndrome was brought to consultation because of bilateral ectropion of the upper eyelids that was noted at birth Figure 1 . He was Due to the previous findings, the patient was taken to surgery for bilateral correction of the upper eyelid ectropion. A horizontal skin incision was performed 2 mm above the upper eyelid margin and the edges of the wound were undermined and separated. Once the skin was loose, the eyelid returned to its normal position. A severe laxity of both upper eyelids was noted due to canthal laxity that was corrected at this point with a medial and lateral canthopexy using a 5-0 Vicryl suture for bony attachment, and a full-thickness pentagonal resection of 3 mm at the junction of the lateral one-third and the medial two-thirds of the lid. The margins are closed with three 6-0 black silk suture and the tarsus are closed with interrupted 5-0 Vicryl suture. This helped to improve the horizontal laxity. Finally, two elliptical retroauricular skin grafts measuring 12 mm long and 5 mm wide were grafted into upper eyelids and sutured with interrupted 6/0 black silk suture Figure 2 . PostopeA 10-month-old boy was brought to consultation because of bilateral ectropion of the upper eyelids since the neonatal period. He was born to a 35-year-old mother following an uncomplicated pregnancy and delivery. Initial management was conservative, consisting of frequent application of topical lubricants and ointments and patching of eyelids. Ocular examination revealed: upward slant of the eyelid fissures, epicanthal folds, and ectropion of both upper eyelids, which were able to be repositioned easily, but returned to the everted position spontaneously with crying and forced closure Figure 4 . ChromosA boy with Down’s syndrome was first brought to medical attention to us at the age of 11 months because of ectropion of the upper eyelids since birth that accentuated while crying. His 33-year-old mother reported that the pregnancy was uneventful, the labour was not prolonged and delivery was normal vaginal. As with the previous cases, the eyelids could be returned easily to their normal positions but immediately turned out again with force closure Figure 5 . His karCongenital ectropion of the upper eyelids was first described by Adams in 1896 , 3], , , [6]. ThAlthough the physiopathology of congenital upper eyelid ectropion is unknown, multiple factors have been implied, including absence of an effective lateral canthal ligaments, lateral elongation of the eyelid, hypotonia of the orbicularis, vertical shortening of the anterior lamella, and failure of the orbital septum to fuse with the levator aponeurosis , 2], , , 11], , , 13], , .Treatment of congenital upper eyelid ectropion is controversial. Different options have been suggested. Some believe that a simple and conservative management with lubricants ointments and moist chambers may be enough to prevent desiccation of the exposed conjunctiva, reduction of conjunctival edema and to allow spontaneous inversion of the eyelid within 2 to 3 weeks. , 11], , .Surgical treatment for more severe cases that did not respond to conservative treatment include sub-conjunctival injection of hyaluronic acid , 8], , , tarsorrWe believe that correct management to the congenital ectropion upper eyelid in these cases should include the correction of the underlying anterior lamella shortening with full-thickness skin grafts that should be extended beyond the horizontal limb of the canthal tendon to compensate for subsequent contraction of the graft. In addition, horizontal lid laxity needs to be addressed with a lateral and medial tarsal strip procedure and a full-thickness pentagonal lid resection. In our three cases, the patients did not fulfill the conservative treatment parameters due the congenital abnormalities of the eyelids that may occur in Down’s syndrome.In conclusion, congenital ectropion of the upper eyelid is a rare abnormality that can threaten the cornea and visual acuity if not treated early. In cases where the eyelids can be repositioned mechanically but continue to evert with forced closure or crying, we recommend surgical intervention to prevent further complications. Management’s goal is to protect the cornea, improve cosmesis, and prevent amblyopia. The authors declare that they have no competing interests.Written informed consent was obtained from the patients’ parents for the publication of these case reports and accompanying images.


Hepatic paraganglioma (HPGL) originates from the sympathetic nervous tissue in the liver, and is an extremely rare type of the sympathetic paragangliomas. Till now, only 11 HPGL cases have been reported.131I-MIBG scan showed that there was no evidence of recurrence and metastasis in >2 years.A 49-year-old woman presented to our hospital with a lesion in the right lobe of the liver, which grew from 2 to 6 cm in 2 years. In addition, she had a 6-year history of diabetes. The patient was initially diagnosed as hepatocellular carcinoma and hepatectomy was performed. Surgical resection of the liver lesion was successful, but the blood pressure rose and fell sharply when the lesion was being removed. The pathological examination of the liver lesion showed that it was HPGL. After the operation, the patient recovered uneventfully. Follow-up examination showed the blood glucose level went back to the normal range in 20 days after the operation, and MRI and By means of reporting this case and reviewing 11 reported cases, we conclude that the incidence of HPGLs is extremely low and the clinical and radiological characteristics of HPGLs are nonspecific; thus, it is hard to diagnose HPGLs correctly. Surgical resection is curative therapy for HPGLs, whereas the removing of HPGLs may cause the releasing of catecholamine, and then lead to hypertension crisis and arrhythmia. Thus, antihypertensive therapy is necessary during the operation. Follow-ups after the operation are important for HPGL patients, for pathological examinations are not sufficient to differ malignant HPGLs from benign ones, and follow-ups are helpful for HPGL patients to find the recurrent foci or metastases timely. Among them, the first group is defined as head and neck paragangliomas (HNPGLs) that have a close relationship with the parasympathetic nervous system. However, HNPGLs cannot produce catecholamine. They function as chemical sensors. The other group is related to the sympathetic nervous system and their location is close to sympathetic nerves that can produce catecholamine. According to the World Health Organization (WHO), tumors which are related to sympathetic tissues in the adrenal gland should be named as pheochromocytomas (PCCs), whereas tumors related to extraadrenal sympathetic tissues should be named as sympathetic paragangliomas (sPGLs). sPGL often occurs along the axis of the body, from skull-base to the pelvic floor and corresponds to the distribution of the sympathetic nerve. In summary, PGLs include sPGLs and HNPGLs. Research has demonstrated that in PGLs, 55.2% occurred in the retroperitoneal space, 25.6% in the head and neck, 5.6% in the bladder, and 3.2% in the mediastinum. However, hepatic PGLs (HPGLs) are extremely rare. To date, there has only been 11 reported HPGL cases. Among them, 7 cases were reported in China and 4 in other countries.–15 Some HPGL patients were admitted to hospital for symptoms relating to catecholamine excess, including chronic headache, palpitation, hypertension, and so on, but others for lesions in the liver during check-up. However, it remains difficult to diagnose HPGLs correctly because their manifestation and the results of radiological examinations show no specificity as to their cause.Paraganglioma (PGL) is defined as a rare neuroendocrine neoplasms, and forms the main branch of pheochromocytomas and paragangliomas (PPGLs).131I-MIBG scan of her entire body after surgery revealed no positive findings to support a diagnosis of HPGL.Herein, we report the case of a woman with HPGL who was first diagnosed with hepatocellular carcinoma (HCC). After this first diagnosis, the patient received a hepatectomy. During the operation, her blood pressure underwent severe fluctuation, especially when her tumor was removed. A final pathological examination confirmed that this tumor was PGL, and a 2Colored 3D reconstruction of CT images was carried out by using an IQQA-Liver reconstruction system . This study was approved by the Ethics Committee of Tongji Hospital, Huazhong University of Science and Technology. Because our case report does not violate the patient's privacy, informed consent is not necessary.3A 49-year-old female patient presented to our hospital with a solitary lesion in her liver, which was found accidentally by an abdominal ultrasonic scan during a physical examination. This patient was asymptomatic, and physical examination of this patient showed no positive signs. The patient had no history of viral hepatitis and alcohol abuse. She had a 6-year history of diabetes and her blood glucose level was not controlled well. The blood pressure of the patient was normal. The patient found this lesion with its diameter of 2 cm in her checkup 2 years before, and she refused further examination and chose follow-up at that time. The patient had undergone breast operation 20 years before and the pathological result of the resected breast lesion was benign. The results of laboratory tests were almost in the normal range except blood glucose and α-fetoprotein (AFP). Blood glucose was significantly high (13.18 mmol/L) and AFP was a slight elevation . The Child-Pugh score was <5. An ultrasonic scan of her liver showed the mass of the lesion to be located in the right lobe of her liver, with a size of 5.7 × 4.9 cm. The shape of the lesion was anomalistic, but its edges could be easily discerned  showed no significant symptom or uncharacteristic symptoms, including abdominal pain and distension. Additionally, several dormant symptoms of catecholamine excess may reveal themselves when HPGL is irritated. For example, when HPGLs are surgically removed, patients always endure a severe fluctuation of blood pressure caused by the hypersecretion of catecholamine. This phenomenon occurs even in HPGL patients presenting with no symptoms of hypertension before surgery. In this case, although the patient had no history of hypertension, we observed the drastic increase and decrease of blood pressure during surgical resection of liver mass. Similar phenomenon was also observed and reported by You et al. Therefore, it is necessary for doctors to prepare antihypertensive agents (α-blocker and β-blockers) to prevent or mitigate complications, including hypertensive crisis and arrhythmia during surgery. Finally, it is important for surgeons to remove the lesion carefully to reduce irritation to HPGLs.Symptoms of catecholamine excess are a characteristic manifestation of sPGLs, although approximately 10% to 15% of sPGLs are asymptomatic. These characteristics have a close connection to the abundant vascular networks in tumors. The characteristics of enhanced MRI scanning for HPGLs is similar to that of CT scans; thus, MRI scans have difficulty in distinguishing HPGLs from HCCs. The most valuable examination for PGL is 131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy. It has been previously demonstrated to show a high specificity and sensitivity for detecting PCCs, and its sensitivity is 77% to 95% and specificity is 95% to 100% to PGLs. In addition, somatostatin receptor imaging is often used for diagnosing PGL. PGLs belong to NETs, and somatostatin receptors are expressed on the surface of tumor cells. Although octreotide signed by radionuclides can find tumors, several neuroendocrine tumors have somatostatin receptors; thus, the sensitivity and specificity of this method are lower than 131I-MIBG scintigraphy. Although PET-CT has obvious advantages in malignant tumors, it shows no specificity in PGLs. Previous studies reported that the sensitivity of 18F-levodopa to malignant PGL reaches 100%, but this result requires confirmation by further studies.The characteristic of CT imaging of HPGL is similar to that of HCC, which showed hypoattenuating lesions in the liver, and these lesions were enhanced in the hepatic arterial phase of the CT scan, and de-enhanced in the portal venous and delayed phase. In huge HPGLs, CT scanning of the liver showed that the area of contrasted enhancement lies mainly in the periphery of the liver lesion, which was consistent with hemangioma of liver. Furthermore, we can exclude the possibility of renal cell carcinoma if CgA, CD56, and Syn are positive, and CK and EMA are negative. HCC will be eliminated if AFP, hepatocyte, and EMA are negative. Perivascular epithelioid cell tumor (PEComa) is not supported if HMB45, CD34, and SMA are negative.The diagnosis of HPGL depends on pathological examination. According to the literature, most PGLs showed a round but not lobulated appearance, and were often solid or hydatidiform in shape. The pathological characteristic of HPGL involves chief cells forming nests and sustentacular cells surrounding them. In addition, there can be abundant sinus-like vascular networks among these nests. Immunochemical staining revealed that CgA and NSE exert positive effects for chief cells, with an accuracy of 100% if they are both used. The expression of S-100 is positive in supporting cells. Disappointingly, even pathological examination is not sufficient to distinguish benign from malignant PCC/PGLs, which makes the diagnosis of malignant PCC/PGLs difficult because they are routinely based on the presence of distant metastases during follow-up. We summarized the follow-up data of 11 reported HPGL cases 3 years after operation and discovered that only 1 case involved metastases in the spleen and the area below the right posterior lobe of the liver. Interestingly, this patient was diagnosed as having a malignant HPGL  for her technical assistance in analyzing CT and MR images of this case. We also thank Dr. Dong Kuang  for his help in analyzing immunohistochemical results of this case. In addition, we thank CureEdit Bioscience Ltd.  for the assistance in revising this manuscript.Conceptualization: Peng Zhu.Data curation: Ze-yang Ding, Wei Liao, Lin Chen, Bixiang Zhang, Peng Zhu.Formal analysis: Ze-yang Ding, Wei Liao, Lin Chen, Xiao-ping Chen, Peng Zhu.Funding acquisition: Ze-yang Ding.Investigation: Ze-yang Ding, Wei Liao, Binhao Zhang, Lin Chen, Jing-jing Wu, Gan-xun Li.Methodology: Binhao Zhang.Supervision: Bixiang Zhang, Xiao-ping Chen, Peng Zhu.Writing – original draft: Ze-yang Ding, Wei Liao.Writing – review & editing: Bixiang Zhang, Xiao-ping Chen, Peng Zhu.


Introduction. We discuss a rare gastrointestinal stromal tumor (GIST) case detected at the 10th postpartum week and we want to pay attention to the challenges and improvements in the diagnosis, surgery, chemotherapy, and follow-up of this rare tumor accompanied with the review of the current literature. Case Presentation. A 32-year-old multiparous woman presented with abdominal swelling 10 weeks after her second vaginal birth. Abdominal examination revealed a mass starting from the pelvic level and extending to the right upper quadrant. Radiological examinations showed a solid, multiloculated, and hypervascular mass starting from the pelvis and extending to the transverse colon. En bloc mass with a 20 cm jejunal segment resection and a left pelvic side wall peritonectomy with omentectomy was performed. The pathologic examination revealed a high-risk GIST which originated from the jejunum and disseminated to the peritoneum. The patient has been given imatinib 400 mg/day since then. She did not reveal any progression during the 15-month follow-up postoperatively. Conclusion. GIST tumors are rare and there is not sufficient information in the literature regarding its management. In this patient having high risk GIST and GIST sarcomatosis we successfully treated the patient by surgery and adjuvant imatinib chemotherapy. Cancers seen in the reproductive period are the second cause of death after cardiovascular diseases . AlthougA 32-year-old multiparous woman (G3P2) presented with abdominal swelling 10 weeks after her second vaginal birth. She had a normal pregnancy follow-up and birth process, until now, and her medical and family history did not show any feature. In the gynecological examination, external and internal genital organs were normal in size and structure. Postpartum uterine involution was regular. In the abdominal examination, a mass starting from the pelvic level, but with indistinct relation to genital organs and extending to the right upper quadrant, was palpated. In the abdominal ultrasound screening, a multiloculated mass divided with septal structures, including cystic and solid areas starting from the suprauterine level reaching to the subxiphoid region, was detected. In the further evaluation using computerized tomography  and magn En bloc mass with a 20 cm jejunal segment resection and a left pelvic side wall peritonectomy with omentectomy was performed  were evaluated on frozen section. A spindle cell tumor was seen on microscopy but the surgeon was informed that the subtype of the tumor could be given on paraffin sections. Small intestinal resection together with resection of a large lobulated mass, originating from the serosal surface and mesentery of the intestine, measuring 27 × 22 × 15 cm and weighing 2991 gr, and omentectomy specimen were also submitted. On cross section, the tumor was lobulated, hemorrhagic, soft, and friable with necrotic areas. On microscopy, spindle cell tumor with infiltrative pattern of growth, identical with the tumors seen on peritoneal surfaces, was seen. On immunohistochemistry, tumor cells with moderate atypia were strongly and diffusely positive with C-kit, CD34, and vimentin, focally positive with smooth muscle actin and caldesmon, and negative with S-100, inhibin, desmin, and keratin. Ki-67 index was <5% when counted on 2000 cells. Six mitoses were counted per 50 high power fields or 5 mm2. Omentum did not contain any tumor.2). Molecular mutational analysis was not performed.The tumor was reported as GIST with peritoneal dissemination and as a high-risk group tumor according to Miettinen and Lasota , becauseThe patient was given imatinib 400 mg/day and is still continuing. She did not reveal any progression during the 15-month follow-up postoperatively and during the writing of this manuscript.Lack of literature-based evidence and being relatively less common are such difficulties associated with the management of PAC . A maligThe process of the management of a diagnosed malignancy after the birth may lead to many negative consequences related to the breastfeeding, infant, and maternity care, although the risk related to the fetus is reduced. In our patient, breastfeeding is stopped because of delicate maternity process which is interrupted due to surgical intervention, healing process, and adjuvant chemotherapy treatment. The survival rates after the treatment of cancers related or not related to PAC are reported to be not significantly different , 8, 9. HDue to the limited number of cases, in which direction the healthy mother effect would be in GIST is unpredictable. These nonepithelial tumors, which may be localized all over the gastrointestinal system, emerge from the interstitial cells of Cajal in the myenteric plexus or its precursors. Therefore, GIST is also called Cajal tumors . AccordiGIST's main treatment is surgery . After aDepending on the conditions in the developing world, postponing the age of pregnancy is increasing the incidence of cancer seen in pregnancy. PAC diagnosis requires awareness in terms of treatment and follow-up. Basic difficulties in PAC result from the delays in treatment of the mother and worsening of the survival, besides the concerns due to the protection of the vulnerable fetus. Compared with other PACs, GISTs are fairly rare. In numerous terms, our case is followed up without any progression after optimal surgery and adjuvant chemotherapy. In the literature search, we have not come up with a GIST related to PAC; therefore, clinical follow-up will show in which direction the healthy mother effect takes place.


Although malignant melanomas (MM) are common in the head and neck region; primary malignant melanoma of the external auditory canal (EAC) is rare. We present the case of a 50-year-old symptomatic man with a malignant melanoma of the external auditory canal, which clinically masqueraded as a haemangioma. The patient subsequently developed extensive loco-regional metastasis, requiring extensive surgery. We describe the clinical presentation, differential diagnosis, both clinical and pathological in terms of other pigmented lesions in the external auditory canal, detailed histopathology, and literature review. We want to emphasize the importance of immediate and adequate biopsy of any pigmented lesion in the head and neck region to rule out MM. Also, we emphasize the importance of deep biopsy for proper histopathological assessment in addition to distinguishing it from benign melanocytic nevi, in order to initiate treatment. Malignant melanoma (MM) is a malignancy of pigment-producing cells or the melanocytes present predominantly in the skin. They are also found in the eyes, ears, GI tract, leptomeninges, and mucous membranes of the oral cavity and genital tract. The incidence of MM has continued to increase dramatically in the last few decades. This is due to increased exposure to sun in the general population. Head and neck melanomas are complex due to the anatomical complexity of this region and thus the treatment is also challenging. It is a lethal disease, as it accounts for 65% of all deaths from skin cancers . The majAn apparently healthy 50-year-old man presented to the ENT clinic with a 2-year history of blood stained discharge from his left ear. He also had pain and mild hearing loss. Examination revealed a bluish-black mass arising in the EAC. Examination of the right ear and the head and neck showed no other significant findings. There was no lymphadenopathy and no enlargement of the parotid gland. Appearance of the mass (bluish), presence of occasional bleeding and no other significant clinical findings led to the clinical diagnosis of haemangioma. Due to the clinical impression of a haemangioma, the mass was locally excised under local anaesthesia. Histopathological evaluation of this surgical specimen showed fragmented strips of stratified squamous epithelium with heavily pigmented melanocytic cells proliferating intraepidermally . A smallThe surgical specimen was sent for histopatho- logical examination. Microscopic examination of the mass in the left EAC showed an ulceroproliferative growth consisting of medium to large pigmented cells with prominent nucleoli. The tumour cells were seen to infiltrate the deeper subcutaneous tissue, parotid gland, and intra parotid lymph nodes . The thickness of the tumour was 15mm (Clark level V). There was evidence of vascular and perineural invasion. Immunohistochemistry revealed that the tumour cells were positive for HMB-45 . The deeMM is a lethal disease with a poor outcome. As MM was not a differential diagnosis in our case and the initial biopsy was superficial; a definitive diagnosis and therefore treatment, were delayed for about 5 months. Within that short time, our patient presented with extensive metastasis, which contributed to a poor outcome. This emphasises the importance of keeping MM in the differential diagnosis of pigmented cutaneous lesions at unusual sites.Rare lesions in the EAC can range from benign to malignant tumours. Haemangiomas, benign vascular neoplasms, are commonly seen in the head and neck region. However, a literature search showed that haemangiomas are extremely rare in the EAC. They are commonly of two types: capillary and cavernous. In the EAC, cavernous haemangioma is common. Histopathological examination will show proliferation of large, tortuous vascular structures of varying sizes within abundant fibrous stroma. Surgical excision is the treatment of choice and long term follow up of reported cases did not show recurrence -10. Our Nevus is a benign melanocytic lesion, which is the most common melanocytic lesion in the skin. However, it is extremely rare in the EAC. So far, only a few cases are reported in the literature. Nevus can also present with external ear symptomatology with a pigmented mass , which wMalignant tumours are also rare in the EAC. Adenoid cystic carcinoma, squamous cell carcinoma, adenocarcinoma, basal cell carcinoma, mucoepidermoid carcinoma, etc… are seen in the EAC, but they are rare. Occurrence of MM in the EAC is even more rare than these tumours. Many published studies about malignant tumours of the EAC do not have a case of MM at this site ,13. JahnThe presenting feature of MM is non-specific, which does not contribute to raising suspicion unless a proper visual examination is performed. Patient may present with deafness, pain, bleeding, periauricular swelling, etc…. However, a mass is seen on visual inspection. In many reported cases, the initial diagnosis was of more common pathologies, such as aural polyp, ceruminous accumulation or infectious aetiology -7. In ouAny cutaneous melanoma thicker than 4mm is considered to have a poor prognosis. Thickness of the lesion is the most sensitive indicator for development of metastasis and disease free survival. Ear MM is a high risk lesion as it has higher thickness at presentation . Poor prThe ABCDE criteria developed by The American Cancer Society are not useful as EAC is inaccessible for visual inspection by the patient. Irrespective of the site, the gold standard for melanoma diagnosis is histopathological examination of a full thickness biopsy. An excisional biopsy (or deep saucerisation technique) with narrow margins is preferred when possible. In an unsuspected case, if histopathological findings suggest a melanocytic nature of the tumour, the patient should be promptly taken for further work up and completion of resection; because surgery currently offers the best probability for cure of malignant melanomas of the head and neck region . Total sBoth benign and malignant tumours are rare in the EAC. MM is also rare and it has a poor prognosis with a high chance of recurrence and metastasis, so early diagnosis and prompt treatment is necessary. Hence, external ear symptoms with a pigmented mass should raise suspicion and a full thickness biopsy should be obtained for histopathological examination.


Central giant cell lesion is a non-neoplastic proliferation, usually asymptomatic, of unknown etiology. The purpose of this case report is to report the diagnosis and the treatment of a recurrent central giant cell lesion in the maxilla.A 31-year-old Brazilian woman presented to our Surgery Service for evaluation of a cystic lesion in her teeth 13 and 15, although she had previously received endodontic treatment for her teeth 13 and 15 without regression of the lesion. On clinical examination, an increase and painless swelling was observed in her right jaw. An excisional biopsy of the lesion was performed under general anesthesia; the material was sent for pathological examination and a diagnosis compatible with central giant cell lesion was made. She presented again, 10 months after the removal of the lesion, with a recurrent lesion that surrounded her incisors, canine, and right premolar. We suggested that she underwent treatment with intralesional corticosteroids injection. The lesion was significantly reduced and the remainder of the lesion was enucleated. She is monitored at 3-month intervals; at 6 months postoperatively there has been no recurrence.Central giant cell lesion can have a high degree of invasiveness, which increases the importance of early diagnosis. Combination therapies can provide a favorable prognosis. Periodic monitoring is recommended, thus avoiding the chance of a relapse. Central giant cell lesion (CGCL) is a benign proliferative and asymptomatic intraosseous lesion. It is more common in females, in the third decade, without predilection for race –3. It acA 31-year-old Brazilian woman presented to our Surgery Department of Oral and Maxillofacial Surgery for evaluation of cystic lesions in her teeth 13 and 15 and a swelling on her right maxilla Fig. .Fig. 1PaShe underwent preoperative laboratory tests and an excisional biopsy of the lesion was undertaken under general anesthesia. The choice of the use general anesthesia was due to the size of the lesion and possibility of enucleation without removal of the teeth involved. The collected material was sent for pathological examination. The result was compatible with CGCL Figs.  and 4, wShe self-monitored for a period of 10 months; she then presented again with a complaint of swelling on the site, but without the presence of pain. A new CT scan was requested and indicated recurrence of the lesion, which now involved the region’s incisor, canine, and premolar Fig. . New labWe proposed new treatment with intralesional corticosteroid injections to our patient in an attempt to reduce the lesion for a future enucleation. She was given a drug application schedule: nine applications in the affected regions with intervals of 15 days between applications. Infiltrations were composed of: Triancil® (triamcinolone hexacetonide) + Xylestesin® , paresthesia, root reabsorption, cortical perforation, a high rate of post-treatment enucleation recurrence and often produces edema .On radiographic examination, CGCL varies from small apical lesions to large lesions involving multilocular radiolucent areas of maxilla . The preOn histological examination, CGCL are represented by multinucleated giant cells in a prominent fibrous stroma. Osteoclasts have irregular distribution and are associated with areas of hemorrhage. Structurally the proliferative cells include spindle-shaped fibroblasts, myofibroblasts, and mononuclear inflammatory cells . Foci ofen bloc resection. This choice depends on factors such as aggressive and non-aggressive form, location, size, and radiographic appearance. Other treatments include radiation, systemic injections of calcitonin, interferon, and intralesional injections with corticosteroids [The traditional treatment of CGCL is surgical excision, enucleation, or steroids . The appsteroids . Howeversteroids . Interfesteroids .Intralesional injections with corticosteroids are increasingly used clinically, and some studies show excellent results. They can be considered a first treatment option. With a less invasive approach, these injections can be used individually or in combination with other therapies, such as surgery and calcitonin , 10. IntThe most aggressive types of lesions require a more radical approach. The management of these lesions depends on clinical and radiographic findings. In general, the enucleation of well-defined and localized lesions is associated with a low recurrence rate. In extensive lesions, based on imaging tests, where there has been cortical drilling, a more radical excision is mandatory . EnucleaGiven the above, the correct diagnosis of CGCL and its degree of aggressiveness is achieved through an analysis of clinical, radiographic, and pathological examinations. The treatment plan may vary from noninvasive therapies, such as medication, to surgical approach, wherein enucleation is proposed. There is also the possibility of a combination of techniques. The combination of two different therapies can be a good solution, since in some cases a single surgical approach leads to facial mutilation.


Richter’s hernia is a high-risk ischaemic gastrointestinal disorder that is typically diagnosed in a delayed manner due to a lack of obvious symptoms. Spontaneous umbilical enterocutaneous fistula (ECF) resulting from an incarcerated Richter’s hernia is extremely rare.A 62-year-old female presented with a chief complaint of recurrent umbilical region infection for the preceding 20 months with no symptoms of ileus. Preoperative CT and fistulography revealed an incarcerated Richter’s hernia complicated by an ECF. Exploratory laparotomy revealed a loop of the distal ileum adherent to the umbilical region that was retrieved back into the abdominal cavity. Side-to-side ileo-ileal anastomosis was performed using a 75 mm linear stapler to remove the affected ileum segment. The internal hernia ring was closed using plication sutures instead of via mesh repair due to the patient’s small defect and infection risk.Richter’s hernia can be observed at any age but is particularly common in frail, elderly patients. This nonspecific clinical and laboratory findings of this condition are associated with a high misdiagnosis rate, resulting inrelatively high mortality. Abdominal CT and gastrointestinal imaging are recommended if Richter’s hernia is suspected. Timely surgical intervention is crucial for reducing mortality and improving prognosis. Richter’s hernia has been well established as a specific type of enterocele since the firstconcrete definition of this condition was provided by a German surgeon,August Gottlied Richter, in 1785 . Only whA 62-year-old female presented to the outpatient department of Wuhan Central Hospital of Tongji Medical College in September 2015with a complaint of recurrent infections in the umbilical region. She reported abdominal pain similar to a burning sensation that accompanied the discharge of faecal matter. These symptoms and signs waxed and waned but lasted for 5years. Our attention was piqued by the fact that the patient’s family described the patient as an individual who cried easily. The patient had no history of diarrhoea, constipation or other abdominal disturbances. No surgical treatment was mentioned in her prior medical history.Escherichia coli and Enterococcus faecalis were detected in the fistula secretion culture. Other findings from laboratory examinations were normal. A CT scan of the abdomen revealed that part of the intestinal wall was adhered to the abdominal wall in the navel region, although no bowel obstruction was detected  was found entrapped in the internal hernia ring; this finding was consistent with the preoperative contrast image. The defect in the abdominal wall was less than 1.0 cm, and an extremely small portion of the bowel wall was stuck and could not be retrieved back into the cavity Fig. . NonetheRichter’s hernia is named after August Gottlieb Richter; however, Fabricius Hildanus provided the earliest known record of this type of hernia in1598 . Singh eRichter’s hernia refers to a condition in which a portion of the bowel wall is entrapped in the hernia sac without symptoms of ileus; this condition tends to occur most frequently in aged patients, particularly elderly females . The mosThe pathophysiology of Richter’s hernia is not well understood. To date, a small abdominal wall defect with hard peripheral tissue was regarded as a key feature of the evolution and progression of this type of hernia. Increased intra-abdominal pressure due to any cause forcesa portion of the circumference of the bowel wall into the hernia sac through the internal ring. The patient in our case exhibited a susceptibility to weeping, which disturbs abdominal pressure. Spontaneous ECF is typically attributable to a blood circulation disorder with chronic bowel wall ischaemia, complicated by an abscess, that spreads through subcutaneous tissues. The external fistulous tract that is formed is ultimately caused by sustained inflammatory stimulation . In 2003Richter’s hernia is a type of incarcerated hernia; similarly to other incarcerated hernias, Richter’s hernia features characteristics such as intestinal haemo dynamic disorder, ectopic bacteria, and surgical area scarring or infection. Given these characteristics, one-stage repair with mesh via exploratory laparotomy is not appropriate for the treatment of incarcerated hernia without perforation due to the risk of adhesion and ECF formation . CurrentRichter’s hernia can be observed at any age but is particularly common among frail, elderly patients. This nonspecific clinical and laboratory findings of this condition are associated with a high misdiagnosis rate, resulting in relatively high mortality. If strangulation in Richter’s hernia is suspected, then auxiliary examinations, such as ultrasound examination, abdominal CT and gastrointestinal imaging are recommended. Timely surgical intervention is crucial for reducing mortality and improving prognosis.


Jackstone is a bladder stone that has a similar appearance to toy jacks. However review of the English language medical literature revealed only a few previous reports of jackstone calculus.We report a case in which a large jackstone calculus was incidentally detected during the evaluation of 67 year old male presenting with lower urinary tract symptoms. X-Ray kidney, ureter, bladder showed a large irregular shaped radio-opaque shadow in the pelvic region. He underwent cystolitholapaxy and transurethral resection of the prostate.It is important to recognize the characteristic shape of the urinary bladder calculi in the diagnosis of the jack stones and to treat the primary cause of calculi formation. This calculi type is referred as a “jack” stone because of its similar appearance to the children’s game toys, Jacks. The commonest site of Jackstones is urinary bladder and rarely in the upper urinary tract. They are almost always composed of calcium oxalate dihydrate. They grow forming radiating spicules due deposition of new minerals resulting in irregular shape. Here we describe a patient that presented with obstructive lower urinary tract symptoms and Jack stones in the urinary bladder.A 67-year-old male presented with worsening, predominantly voiding lower urinary tract symptoms of 4 years duration with worsening of symptoms for last 1 year duration. He had hesitancy, poor stream of urine, frequency of micturition.Also he had experienced only one episode of visible, painful haematuria without clots at the end of the urinary stream which settled with one week treatment with ayurvedic medications. There was no history of other symptoms such as dysuria, fever suggestive of urinary sepsis, acute or chronic retention of urine, urgency, nocturia, nocturnal enuresis or episodes of urinary incontinence or passage of calculi. There was no history of previous urinary tract surgeries or invasive procedures such as catheterization. His past medical history was unremarkable. The physical examination was unremarkable except clinically benign prostatomegaly (about 40 g).His uroflowmetry showed a significant limitation of maximum flow rate (Q max = 4.2 ml/s). On ultrasonography he was found to have 50 g prostate with slightly thickened bladder wall (5 mm) with a large stone 2.5x3cm) in the urinary bladder. He had a significant post voidal volume 109 ml) in the presence of 376 ml of prevoidal volume of urine. Upper parts of the urinary tracts were sonogrphically normal. X-Ray KUB showed a large irregular shaped radio-opaque shadow in the pelvic region [Fig. 09 ml in cm in theOnly few cases of jackstones had been reported previously , 2. As tBladder outlet obstruction remains the most common cause of bladder calculi in adults. Most common factors predisposing to bladder stone formation includes prostatic disease, previous lower urinary tract surgery, metabolic abnormalities, intravesical foreign bodies, spinal cord injuries, upper urinary tract calculi . Stones It is important to recognize the characteristic shape of the jackstones as they are susceptible to lithotripsy. We were able to manage his problems, vesicle stone and bladder out flow obstruction by minimally invasive surgery in single index admission.It is important to recognize the characteristic shape of the urinary bladder calculi on radiological investigation in the diagnosis of the jack stones. Identification and treatment of the primary cause of calculi formation is important in the overall management.


While the lung is the most common extrauterine organ afflicted, benign metastasizing leiomyomas (BML) of the heart are rarities. We report an incidental finding of a cardiac mass in a 36-year-old woman who presented to the Emergency Department after a motor vehicle accident. CT scan of the chest revealed 2 well-circumscribed pulmonary nodules and a filling defect in the right ventricle. Echocardiogram showed a 4 cm mass attached to the right ventricular (RV) septum. The cardiac tumor was resected and showed benign histologic features. Immunohistochemical staining was positive for smooth muscle  We present the case of a cardiac benign metastasizing leiomyoma incidentally found in a patient who presented to our hospital after a motor vehicle accident.A 36-year-old female patient presented to the emergency department of our hospital with right-sided blurry vision after being involved in a motor vehicle accident. The patient was a restrained back seat passenger and sustained a right eye trauma with no other injuries nor loss of consciousness. She was found to have traumatic uveitis. CT scan of the head and sinuses showed no evidence of fracture or acute pathology. A CXR was performed to rule out any rib fracture and found a 1.1 cm pulmonary nodule in the right midlung. A contrast CT scan confirmed the presence of 1.1 cm nodule within the anterior inferior aspect of the right upper lobe and found an additional 1 cm nodule within the posterior superior aspect of the right upper lobe. Both nodules were well circumscribed, compatible with benign nature. Unexpectedly, a large defect was noted within the contrast-enhanced right ventricle and was thought to be a large thrombus extending to the right ventricular outflow tract . The patThe patient denied any symptoms including chest pain, dyspnea, syncope, or weight loss. Physical exam was significant for a systolic murmur. On further history taking, she reported that she underwent a hysterectomy 12 years earlier for “myomas” but denied any personal or family history of malignancy.Abdominal/pelvic CT scan was performed and confirmed the absence of the uterus. No definite evidence of primary or metastatic disease was found with the exception of a 2.2 × 1.7 cm soft tissue nodule at the left aspect of the vaginal cuff, which was thought to be the left ovary. CA 125 level was within normal limits.In the absence of evidence of primary malignancy, surgical resection of the cardiac tumor was pursued.Intraoperatively, the mass was noted to be encircling the tip of a papillary muscle which was supporting the chordae to the anterior leaflet of the tricuspid valve. The mass was completely excised with sacrifice of the chordae tendinae traversing the lesion  and subsHistologically, the tumor was composed of smooth muscle proliferation and a very low index of proliferation (Ki-67 < 10%), supporting a benign process. No atypia or necrosis was noted.α-actin, desmin, estrogen, and progesterone receptors, supporting a leiomyoma lineage.Immunohistochemically, tumor cells were strongly positive for smooth muscle The diagnosis of fibroma was excluded in light of the desmin stain positivity and elastin stain negativity. Immunostain for myogenin was negative, excluding the diagnosis of hamartoma of mature cardiac myocytes.FDG PET-CT was performed postoperatively and revealed a faint FDG uptake within the 2 previously identified pulmonary nodules (SUV 1.4) and the soft tissue nodule on the left aspect of the vaginal cuff, later identified as parasitic leiomyoma by transvaginal ultrasound (US) and not the left ovary as initially thought.No additional sites of metastasizing leiomyoma were identified.The patient recovered from cardiac surgery without complications. She was subsequently started on aromatase inhibitor treatment (anastrozole). CT scan of the chest-abdomen-pelvis 3 months after surgery showed stable pelvic and lung masses and no evidence of recurrent disease in the right ventricle. No interval growth of other masses was found. The patient remains asymptomatic 6 months after surgery.Uterine leiomyomas are the most common gynecological tumors in pre-menopausal woman, found in up to 30% of women older than 35 years.Despite being histologically benign, leiomyoma has clinically malignant potential. Metastases have been reported to a number of different sites, including the spine –4, skullDifferent types of extrauterine growth of benign uterine leiomyomas are described as follows: disseminated peritoneal leiomyomatosis (DPLM), retroperitoneal leiomyomatosis (RPLM), parasitic leiomyoma, BML, and intravenous leiomyomatosis (IVL) .DPLM and RPLM are characterized by multiple leiomyomatous masses seen in the submesothelial tissues of the abdominopelvic peritoneum and the abdominopelvic retroperitoneum, respectively . ParasitIn contrast, BML and IVL can cause more distant metastases, particularly to the chest.Intravascular leiomyomatosis is characterized by the presence of vascular invasion and extension of benign smooth muscle lesions in a worm-like manner into the pelvic and systemic veins. No macroscopic vascular invasion is found in BML. Owing to its intraluminal growth, leading to symptoms of venous obstruction , IVL hasIn IVL, US shows vascularized thrombi within the pelvic veins and inferior vena cava. CT and MR imaging demonstrate continuity in intraluminal growth from the pelvic veins . None ofPathogenesis of BML is controversial . HoweverWhile several cases of cardiac metastases have been reported with IVL , 16, to The mass initially identified on the chest CT scan was erroneously thought to be a thrombus, leading to initiation of anticoagulation. Thrombus accounts for the most commonly encountered intracardiac mass and typically appears on CT scan as a hypodense, low attenuation filling defect, similar to intracardiac tumors . While CUterine leiomyomatosis is highly hormone sensitive, and treatments are based on hormonal manipulation –27 with However, medical treatment alone is highly insufficient in the case of intracardiac tumors. This is related to the risk of heart failure and possible sudden death caused by total outflow tract obstruction. Regardless of the pathogenesis of intracardiac leiomyomatosis, review of the literature suggests that surgical removal of the intracardiac tumor is curative , 31. ComThis case underlines the importance of considering BML as a potential differential diagnosis in any female patient with a history of hysterectomy who presents with an intracardiac tumor.