Borderline ovarian tumors are benign but relatively large tumors that are often initially mistaken as ovarian cancers. We report three cases of stage I borderline ovarian tumors having massive ascites that we (preoperatively) suspected of being advanced ovarian cancer. The three patients  reported feeling fullness of the abdomen before consulting their gynecologist. By CT scan, they were diagnosed with a pelvic tumor accompanied by massive ascites, the diameters of which were 11, 20, and 11 cm, respectively. Postsurgical pathology showed all were stage I borderline ovarian tumors without dissemination; two were mucinous and one was serous. The amount of ascites was 6,300, 2,600, and 3,600 mL, respectively, and was serous in all. Cytodiagnosis of the ascites found that one was positive for tumor cells and two were negative. After resection of the mass, the ascites disappeared in all three cases. No pleural effusion was present at any time. The literature is reviewed concerning ascites and pleural effusions linked to ovarian tumors, and a supposition is forwarded of why pleural effusion presents sporadically in these cases. Borderline ovarian tumors represent 10–15% of all ovarian tumors . HoweverWe report here on three cases of benign stage I borderline ovarian tumors with massive ascites that, before their operation, were suspected of being advanced ovarian cancers. Their ascites disappeared rapidly after resection of the mass and, strikingly, they lacked pleural effusion. We include a review of the literature concerning conjectures as to why pleural effusions do or do not occur in cases of pelvic tumors.A 35-year-old woman felt a fullness (distension) of her abdomen before consulting a gynecologist. She was diagnosed with a pelvic tumor by CT scan and was sent to our hospital for medical treatment. The maximum diameter of her pelvic tumors was 11 cm and was accompanied by massive ascites. A total abdominal hysterectomy (TAH), bilateral salpingo-oophorectomy (BSO), and a partial omentectomy (POM) were performed.Postoperatively, a pathological examination determined that the case was stage I serous borderline ovarian tumor. The amount of the serous ascites present was 6,300 mL, and the cytodiagnosis of the ascites was positive for floating tumor cells. After resection of the mass, and without adjuvant chemotherapy, the ascites disappeared rapidly. No recurrence has been found to date; the disease-free survival intervals were 1,264 days at last checkup (A 47-year-old woman felt a fullness (distension) of her abdomen before consulting a gynecologist. She was diagnosed with a pelvic tumor by CT scan and was sent to our hospital for medical treatment. The maximum diameter of her pelvic tumors was 20 cm and was accompanied by massive ascites. A total abdominal hysterectomy (TAH), bilateral salpingo-oophorectomy (BSO), and a partial omentectomy (POM) were performed.Postoperatively, a pathological examination determined that the case was stage I mucinous borderline ovarian tumor. The ascites was serous. The amount of the ascites present was 2,600 mL, and the cytodiagnosis of the ascites was negative. After resection of the mass, and without adjuvant chemotherapy, the ascites disappeared rapidly. No recurrence has been found to date; the disease-free survival intervals were 1,785 days at last checkup .A 73-year-old woman felt a fullness (distension) of her abdomen before consulting a gynecologist. She was diagnosed with a pelvic tumor by CT scan and was sent to our hospital for medical treatment. The maximum diameter of her pelvic tumors was 11 cm and was accompanied by massive ascites. A total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) were performed.Postoperatively, a pathological examination determined that the case was stage I mucinous borderline ovarian tumor. The ascites was serous. The amount of the ascites present was 3,600 mL, and the cytodiagnosis of the ascites was negative. After resection of the mass, and without adjuvant chemotherapy, the ascites disappeared rapidly. No recurrence has been found to date; the disease-free survival intervals were 1,750 days at last checkup .True Meigs' syndrome is characterized by a triad of symptoms: abdominal ascites and right-sided pleural effusion associated with a benign ovarian tumor, most commonly a fibroma, but occasionally a fibroma-like Brenner or granulosa cell tumor , 7. A coThe pathophysiology of ascites in ovarian tumors in general, and Meigs syndrome in particular, remains speculative. Several theories concerning the source of ascites in both Meigs' and pseudo-Meigs' syndromes have been proposed. Some involve proposed production of ascitic fluid by the tumor; others suggest that lymphatic obstruction, hormonal stimulation, release of inflammatory mediators, or tumor torsion is the cause. One early suggestion was that a solid ovarian tumor could physically irritate the peritoneum and stimulate the overproduction of peritoneal fluid . Anotherγ, IL-1β, IL-6, IL-8, IL-10, TNFα, PlGF, and HSP90B1, is frequently associated with epithelial ovarian cancers (EOC) [Release of multiple inflammatory cytokines into the plasma, including IFNrs (EOC) , 12. Thers (EOC) .The etiologies of ascites and pleural effusions of the Meigs spectrum of syndromes are equally poorly understood. The example of “atypical” Meigs' syndrome with “pleural effusion without associated ascites” suggests that the two body cavity fluid pools can be, in rare cases, independent pathological conditions.It is known that pleural effusions can result from inflammation, such as in infection or autoimmune disease, or from several other fairly common conditions. However, the pleural effusion associated with the hydrothorax in Meigs' and pseudo-Meigs' syndrome is thought to be caused by migration of excessive ascites fluid into the pleural cavity via a unique class of lymphatic channels in the diaphragm , 15. TheIn Meigs' and pseudo-Meigs' syndrome, the amount of pleural effusion that accumulates usually has no direct relationship with the amount of ascites present , nor is Presumably, whether hydrothorax occurs as a consequence of ascites depends on the presence and nature of a pathway for ascites flow from the abdomen to the pleural cavity. It is thus illustrative to note that a known complication of peritoneal dialysis for renal disease is the movement of the dialysate into the pleural space, causing a serous hydrothorax . PeritonIn addition to diaphragm porosity, the inherent intestinal circulation, lower hydrostatic pressure in the right upper quadrant, and liver capsule may contribute to this right-sided predominance. In addition to Meigs syndrome, similar right-sided presentations have been described in bilious effusions with gastric or duodenal perforations, hepatic hydrothorax, and nephrotic syndrome-related chylothorax . BecauseLeVeen et al. reported that hydrothorax occurred in only 5.3 percent of their ascitic patients. Eighteen of the 21 spontaneous hydrothorax cases in their study occurred on the right side . They prWe now know much more about the microscopic process of transdiaphragmatic fluid flow. Lymphatics in the diaphragm form a specialized system for draining fluid from the peritoneal cavity and returning it to the vascular system . Fluid fWhile the distribution of mesothelial stomata across abdominal organ surfaces, and subjacent lymphatic lacunae, varies in different species, these stomata appear to be predominantly on the peritoneal surface of the diaphragm. From the lacunae, fluid traverses the diaphragm via intrinsic lymphatic channels to reach collecting lymphatics beneath the diaphragmatic pleura. Both intrinsic and collecting lymphatics contain valves . These oClinically, these channels provide escape for tumour cells, pathogens, and toxins from the peritoneal cavity. They can also provide access for blood transfusions, for intraperitoneal chemotherapy to treat malignancies, and for peritoneal dialysis in treating chronic renal failure.Based on the most likely of these conjectures, and on scientific findings, we suggest that Meigs-associated hydrothorax is the result of the existence of either more numerous or more permissive lymphatic channels in the diaphragm of predisposed individuals. It follows that there can be bilateral differences in the distribution of these channels, and differences among individuals for pleural effusion. The corollary is that there may be individuals who have minimal numbers of lymphatic channels in the diaphragm and are thus more resistant to hydrothorax and more predisposed to ascites accumulation. In such cases, pleural effusion would be far less likely to occur, despite the existence of massive ascites.In all our cases, no one wished to preserve their reproductive capacity. But one-third of borderline ovarian tumors occur in women younger than 40 years, so they often wish that . LudovisIn conclusion, in our normal practice we have encountered three cases of borderline ovarian tumor accompanied with massive ascites. They were atypical in that they were not advanced ovarian cancers, nor did they fit within the criteria of Meigs' or pseudo-Meigs' syndrome, because they lacked pleural effusion. This is not unusual in that various types of benign to highly malignant ovarian tumors may present as massive ascites of unknown origin.Physicians should be aware of the differential diagnostics needed to determine what type of mass they are dealing with. Pathological confirmation of the tumor and its surgical removal are primary goals in such cases, in order to avoid unnecessary neoadjuvant therapy and radiation/chemotherapy.


Although schwannomas are common spinal tumors with insidious presentations, acute neurological deterioration is an extremely rare manifestation that can occur in the setting of tumor torsion and infarction. The present case reports an unusual presentation of a spinal schwannoma that underwent torsion and infarction. A 65-year-old male presented initially with acute radicular pain progressing to cauda equina syndrome and confusion. MRI of the lumbar spine revealed an intradural extramedullary lesion at the level of L1/L2 measuring 1.1x0.9 cm. Intraoperatively, a reddish mass was seen caudally twisted around itself. Gross total resection was achieved with a final diagnosis of schwannoma with areas of infarction. At his six week follow up clinical visit, the patient was asymptomatic and his neurological exam was normal. The neurosurgeon should be aware of such atypical radiographic and clinical presentation amongst the spectrum of clinical manifestation of these nerve sheath tumors.  Spinal schwannomas are slow-growing tumors that account for one-fifth of all spinal tumors. Their presentation is usually insidious, and most patients complain of extremity pain . An acutA 65-year-old male presented to the emergency room with the acute onset of low back and right lower extremity pain, urinary retention, and confusion. Six days prior, the patient complained of low back pain with radiation to the right buttock, groin, and lower extremity. He was diagnosed as having sciatica at an outside emergency room and discharged with oral pain medications. At presentation, the pain was poorly controlled and complicated with progressive confusion and urinary retention. On exam, the patient was afebrile with normal vitals. He was awake but only oriented to person. Cranial nerves, deep tendon reflexes, and strength and sensation in the upper and lower extremities were all normal. Informed patient consent was obtained for treatment.An enhanced magnetic resonance imaging of the brain was normal. Complete blood count revealed a white blood cell count of 7,800 /uL. The erythrocyte sedimentation rate was 22 mm/hr and C-reactive protein was < 0.5 mg/L. Cerebrospinal fluid (CSF) from a lumbar puncture revealed a glucose of 85 mg/dL, protein of 195 mg/dL, RBC count of 185 /uL, and WBC of 2,530 /uL. The cell differential was 2% lymphocytes and 90% neutrophils. Microscopic examination revealed no organisms. CSF was also sent for herpes simplex virus PCR and enterovirus PCR, and serum was sent for West Nile IgM, RPR, and HIV, all of which were negative. An enhanced MRI of the lumbar spine revealed an intradural extramedullary lesion at the level of L1/L2, measuring 1.1 x 0.9 cm. The T2-weighted sequence showed low signal attenuation and the T1-weighted sequence showed high signal attenuation with minimal contrast enhancement  pathways. These tumors are most commonly intramedullary and are located in the upper cervical spine extending into the brainstem, and often times are associated with syringomyelia and syringobulbia .Our case demonstrates an unusual clinical presentation of spinal schwannoma with acute radiculopathy. This rapidly progressed over days to severe mental status changes as well as cauda equina syndrome manifesting as urinary retention. A lumbar puncture was done on presentation to evaluate for meningitis as a possible cause of the acute mental status changes. The findings were congruent with aseptic meningitis with an elevated white blood cell count, along with normal glucose. Moreover, the persistent confusion was likely attributed to aseptic meningitis. MR imaging demonstrated a minimally enhancing tumor that was congruent with intraoperative and, later, histopathological findings of an infarcted schwannoma. A standard surgical approach of laminectomy, dural opening, and microsurgical resection of the tumor was performed. The nerve sheath tumor was twisting the associated nerve roots. The tumor was easily unwound in the opposite direction and then resected, sparing all functional nerve roots of the cauda equina. Aseptic meningitis has never been reported in the context of intraspinal schwannoma. In this case, it might explain the acute mental status changes observed in our patient.A review of the literature found four other reports of spinal schwannoma undergoing torsion Table  2-5]. I-5. I2-5]Intraoperative and histopathological findings were similar to the present case. Reports often describe the identification of torsion of a well-circumscribed reddish brown lesion -5, with While the exact mechanism remains unclear, we suggest that at some point in time the tumor twisted mechanically and obstructed the blood supply Figure . This, iSchwannomas are common spinal tumors with insidious presentations. However, the present case highlights an atypical manifestation characterized by an acute neurological deterioration in the setting of tumor torsion and infarction. Despite the acute presentation, resolution of the pain and neurological symptoms followed resection of the torsed schwannoma. The neurosurgeon should be aware of such an atypical radiographic and acute clinical presentation amongst the spectrum of clinical manifestations of these nerve sheath tumors.


Embryonal rhabdomyosarcoma of the prostate in an adult is a very rare event with only a few cases published. Diagnosis usually occurs with advanced disease frequently already with metastatic spread. In adults prognosis is very poor, therefore early diagnosis is crucial. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published.We report an additional case of prostatic spindle cell embryonal rhabdomyosarcoma subtype in an adult.We discuss relevant clinicopathological features of spindle cell embryonal rhabdomyosarcoma of the prostate in adult patients in the context of the literature. Rhabdomyosarcomas can be classified as embryonal, alveolar and pleomorphic subtypes –3. Embry3. The clinical diagnosis was prostate adenoma. Prostate-specific antigen (PSA) level was 0,91 ng/ml and thus within normal range. After 4 weeks of therapy with the alpha1-receptorantagonist Tamsulosin the patient reported normal urinary function. Repeat ultrasound showed a postvoid residual volume of 150–200 ml and compared to the initial measurement further enlargement of the prostate (150 cm3). Magnetic resonance imaging (MRI) showed a prostatic tumor 9 × 8.5 × 7.5 cm in size with inhomogeneous tissue structure and polypoid infiltration of the bladder  were negative. The diagnosis of spindle cell rhabdomyosarcoma was confirmed by reference pathology .Histologically, all prostate biopsies showed atypical relatively uniform tumor cells with epithelioid to spindle cell morphology arranged in an irregular fascicular proliferation pattern. The nuclei were elongated and hyperchromatic and occasionally showed prominent nucleoli. Mitotic count was up to 30 mitoses/10 high power fields. In addition, necrotic areas were present. Very few cells were identified having enlarged nuclei and voluminous eosinophilic cytoplasma resembling rhabdomyoblasts Fig. , b. Immuwww.cws.olgahospital-stuttgart.de). Because of the presence of lung metastases the patient was classified Stage 4 – metastatic disease. Therapy consisted of nine cycles chemotherapy including Ifosfamide, Etoposide, Vincristine, Actinomycin D, Carboplatin and Epirubicin followed by oral chemotherapy with Trofosfamide, Etoposid and Idarubicin for 6 months. After 9 weeks of therapy disease reevaluation showed a significant reduction of the primary tumor size and of size and number of the pulmonary metastases. Since tumor resection would be accompanied by significant morbidity, radiation therapy was chosen as definitive local therapy. To better protect the surrounding structures  proton beam therapy to the primary tumor in parallel to chemotherapy was planned. Conventional radiotherapy of the bilateral pulmonary metastases was performed at the end of the intensive treatment. Unfortunately, the patient died from metastatic disease in June 2015, 17 months after initial diagnosis.After initial staging the patient was treated according to the therapy guidelines of the CWS Cooperative Soft Tissue Sarcoma (“Weichteilsarkom”) Group . Thus, correct subtyping of soft tissue sarcomas is of utmost importance even in adults where the development of new therapy protocols may in the future be dependent on the histological subtype.The histologic diagnosis of the spindle cell variant of ERMS is usually based on the presence of small, round to spindle-shaped tumor cells with moderate nuclear pleomorphism. Frequent findings are scattered large rhabdomyoblasts with an eccentric nucleus and striated, eosinophilic cytoplasm , 10.Immunohistochemically, ERMS typically express skeletal muscle markers: Tumor cells usually stain positive for vimentin, desmin, myogenin and myoglobin . HoweverBesides PPNET, differential diagnosis of spindle cell ERMS includes other sarcomatous tumors such as pleomorphic and alveolar rhabdomyosarcoma, leiomyosarcoma and fibrosarcoma. In addition, malignant peripheral nerve sheath tumors, spindle cell malignant melanoma, lymphomas and phyllodes tumors with focal rhabdomyoblastic differentiation have to be considered , 20. ImmERMS lack the characteristic translocation t and t of alveolar rhabdomyosarcoma. In contrast to ERMS, leiomyosarcomas stain negatively for myoglobin and myogenin. Fibrosarcomas do not show scattered rhabdomyoblasts in hematoxylin and eosin (H&E) staining and express neither desmin nor myogenin. In PPNET, immunohistochemical staining with myogenin is mostly negative . PPNET wMolecular pathological diagnostics to date have not detected any specific mutations in ERMS. In some cases, a loss of heterozygosity has been described in the chromosomal region 11p15.5 .Clinical symptoms usually include micturition problems like urinary retention, dysuria and seldom hematuria. Typical clinical findings include an enlarged prostate gland and normal serum value of PSA. The disease usually progresses rapidly so that diagnosis is often made at an advanced stage with presence of distant metastases . MacroscIn our case, a 25-year-old adolescent presented with micturition problems and an enlarged prostate gland. PSA levels were within normal range. At this age, ERMS of the prostate is rather seldom as ERMS occur more often in the lower urogenital tract in children. The most common prostate tumor in male adults is adenocarcinoma, however, this diagnosis is very rare in an age below 40 years . In the Treatment of ERMS depends on stage of disease and includes a combination of chemotherapy, radical surgery and radiotherapy . Our patIf resection of the primary tumor would be mutilating as in our case radiotherapy is a local therapy option. 50 Gy  is considered as sufficient for rhabdomyosarcoma with residual disease following induction chemotherapy without an option for secondary resection .Prognosis of ERMS in adults is generally poor. Most patients with prostatic ERMS die under therapy. Children and adolescents usually have a much better response to multimodal therapy than adults and primary surgical treatment is not standard of therapy , 11. In Prostatic rhabdomyosarcoma is a very rare tumor entity in adults. Prognosis is poor in contrast to children. In adults, optimal therapy may be radical surgery for early tumor stage in the absence of distant metastases. Therefore, especially in adults younger than 40 years, it is of utmost importance to consider this rare differential diagnosis in order not to delay appropriate treatment. Correct histologic subtyping of tumor should be performed to provide a basis for improvements of therapy.CT, computed tomography; ERMS, embryonal rhabdomyosarcoma; H&E, hematoxylin and eosin; MRI, Magnetic resonance imaging; PPNET, peripheral primitive neuroectodermal tumors; PSA, prostate-specific antigen


Schwannoma and angiosarcoma are infrequent pathologies that have been rarely reported in the kidney. Angiosarcoma is an uncommon malignant tumor presenting a recognizable vascular differentiation. It can develop in any site but the most common locations include the skin, soft tissues, breast, bone, liver, and spleen while renal localization has been very rarely reported in the literature. Schwannoma is a benign peripheral nerve sheath tumor composed of cells with the immunophenotype and ultrastructural features of differentiated Schwann cells. It has a wide anatomical distribution but the most frequent locations include subcutaneous tissues of the extremities and the head and neck region and the retroperitoneal and mediastinal soft tissues. The occurrence of an angiosarcoma in a pre-existing schwannoma is an extremely rare event with <20 cases reported in worldwide literature. In the present study, a renal case of angiosarcoma arising in schwannoma is presented with a detailed review of the pertinent literature.A 56-year-old man was admitted with a few days history of lower back pain and hematuria. Abdominal ultrasound showed a mass inside the left renal medulla. Subsequent imaging investigations with computed tomography and magnetic resonance confirmed the presence of the lesion and showed a pulmonary metastasis.The final histopathological examination led to the diagnosis of epithelioid angiosarcoma arising in a schwannoma. The patient came to death a few months later due to a massive hemothorax. To the best of our knowledge, the present is the first case of an angiosarcoma arising in a schwannoma of the kidney. Schwannoma  is a benign peripheral nerve sheath tumor composed of cells with the immunophenotype and ultrastructural features of differentiated Schwann cells. It occurs in patients of any age with a slight predilection for adults . The anaAngiosarcoma is an uncommon malignant tumor presenting a recognizable vascular differentiation. It accounts for only 2 to 4 % of soft tissue sarcomas  and occuWe describe a case of a complex renal lesion that consists of two components changing abruptly within the tumor: a larger, malignant neoplasm diagnosed as an epithelioid angiosarcoma and a minor benign neural tumor diagnosed as schwannoma. It is well known that angiosarcomas can develop in neurofibromas and malignant peripheral nerve sheath tumors, especially in patients with von Recklinghausen’s disease. The occurrence of an angiosarcoma in a schwannoma is a very rare event as only 14 cases have been reported in the literature. To our knowledge, the present is the first case of an angiosarcoma arising in a schwannoma of the kidney.A 56-year-old man with a history of non-insulin dependent diabetes mellitus was referred to the surgical department because of the onset of lower back pain and hematuria that occurred the previous day. The patient was not a smoker and denied alcohol consumption; his family history was unremarkable and there was no other complaint. The ultrasound of the left kidney revealed the presence of stones in the renal pelvis causing obstruction of the ureteral meatus and consequent dilatation of the upper calix. During the examination, the presence of a solid neoplasm was noticed that involved the renal medulla reaching the hilum area without signs of hilar infiltration. For further investigations, the patient underwent CT (computed tomography) and MR (magnetic resonance) that showed the slight enhancement and extensive necrotic areas of the lesion and the existence of a solid nodule with same features in the left lung, likely due to metastases Figs. a-1b. A lGrossly, the kidney presented irregular profile due to a hilar lesion measuring 4 cm × 2.5 cm. On cut sections, it was highly hemorrhagic, soft, and reddish with infiltrating borders Figs. c-1d. TheAngiosarcoma is an infrequent neoplasm with a very poor prognosis which is why it has been very rarely described in the kidney. The diagnosis of this lesion is extremely difficult if not impossible when considering just the clinical and radiological features. So, it is often referred to post-operative time, and pathological examination remains the gold standard. The occurrence of an angiosarcoma in a pre-existent schwannoma is an exceptionally rare event with an unknown pathogenesis. In view of the rarity of the lesion, an extensive review of the literature was undertaken through a MEDLINE search using the search terms “primary renal schwannoma/primary schwannoma of the kidney,” “primary renal angiosarcoma/primary angiosarcoma of the kidney,” and “angiosarcoma arising in schwannoma.” Only reports in English have been taken into account. In Tables Twenty-one cases of renal schwannoma have been reported in literature Table  12–27].–27.12–27Twenty-nine cases of primary renal angiosarcoma have been reported in literature –55. The Angiosarcomas arise very rarely in the context of a pre-existing schwannoma. To the best of our knowledge, only 14 cases have been reported in literature to date –62. In aWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Odontomas are hamartomas composed of various dental tissues, i.e. enamel, dentin, cementum and sometimes pulp. They are slow-growing, benign tumors showing nonaggressive behavior. Most of the odontomes are asymptomatic with unknown etiology, although occasional signs and symptoms related to their presence do occur. Presented here is the case report of 10-year-old girl with impacted left central incisor. They are slow-growing, benign tumors showing nonaggressive behavior.4 Most of the odontomes are asymptomatic, although occasional signs and symptoms related to their presence do occur. They generally are unerupted or impacted teeth, retained deciduous teeth, swelling and evidence of infection.The etiology of odontomas is unknown, although local trauma, infection, and genetic factors have been suggested. One aspect of the etiology of odontomas is most result from extraneous buds of odontogenic epithelial cells.A 10-year-girl was referred to the Department of Pedodon-tics and Preventive Dentistry due to the failure of the left maxillary central incisor to erupt. Past family and medical histories were unremarkable. There was no history of trauma, deformations, or swelling of the maxillofacial region. Intraoral examination revealed normal colored mucosa, increased in volume of the ridge and absence of maxillary left central incisor. The space was sufficient for eruption of the tooth .An intraoral periapical radiograph  and 3 shSurgical removal of the mass was accomplished under local anesthesia. A full thickness mucoperiosteal flap was reflected. A thin layer of the bone overlying the labial surface was removed and the calcified mass was exposed . There wHistopathologic examination  after deThe postoperative period was uneventful . The pat7 Odontomas often cause disturbances in the eruption of teeth such as, impaction or delayed eruption, retention of primary teeth, or abnormalities in the position of the teeth such as tipping or displacement of adjacent teeth.8 In this case, the reason of uneruption of the permanent maxillary left central incisor was the presence of a complex odontoma. Complex odontomas are usually located in910 the first and second molar area of the mandible. A slight majority of odontomas are localized on the right side of the mandible compared to the left. Compound odontomas are approximately twice as common as complex odontomas, and more of the former occurs in the incisor and canine areas of the maxilla.910 However, the present complex odontoma was associated with an impacted maxillary left central incisor. This localization was regarded as rare.Although, the majority of unerupted teeth are seen in the permanent dentition, it is relatively common in the early-mixed dentition. It has been suggested the possible reasons for failure of eruption may be a lack of space, malformation from early trauma, and mechanical obstruction due to such conditions as a supernumerary tooth, an odontoma, or scar tissue due to early loss of primary teeth.1112 Although, the compound type variety is approximately equally distributed between the genders, 60% of complex odontomas occur in women.29 Compound odontomas seldom cause bony expansion, but complex odontomas often cause slight or even marked bony expansion.212 The age, gender, and slight bony expansion in this case are in accordance with previous reports. The lesion should be surgically removed and the specimen should be carefully examined microscopically to rule out ameloblastic odontoma or myxofibrous hyperplasia.10 Kramer et al mentioned when a tooth fails to erupt, the follicle may become thickened and it may have an appearance similar to that of an odontogenic fibroma or myxoma.13Most odontomas are detected during the first two decades of life, and the mean age at the time of diagnosis is 14 years.25 Osteoid osteomas are characterized by a small ovoid or round radiolucent area surrounded by a rim of sclerotic bone; the central radiolucency exhibits some calcification. Cemento-ossifying fibroma presents as a well-defined radiolucency with increasing flecks of calcification as it matures; it is not surrounded by a radiolucent rim and it is diffuse with normal bone.14 Also, none of these is associated with an impacted tooth.In our case study, we present a mature complex odontoma, which should be differentiated from cementoblastoma, osteoid osteoma and fibro-osseous lesions, such as cemento-ossifying fibroma. A cementoblastoma presents as a well-defined radiopaque mass attached to the tooth root and surrounded by a radiolucent rim.15 The first stage is characterized by radiolucency due to the absence of dental tissue calcification, the second or intermediate stage shows partial calcification and the third or classically radiopaque stage exhibits predominant tissue calcification with the surrounding radiolucent halo described above.The odontoma presents as a well-defined radiopacity situated in bone, but with a density that is greater than bone and equal to or greater than that of a tooth. It contains foci of variable density. A radiolucent halo, typically surrounded by a thin sclerotic line, surrounds the radiopacity. The radio-lucent zone is the connective tissue capsule of a normal tooth follicle. The thin sclerotic line resembles the corticated border seen in a normal tooth crypt. The developmental stages can be identified based on radiologic features and the degree of calcification of the lesion at the time of diagnosis.16 Since the occurrence of a complex odontoma in this particular location is rare, removal of the mass overlying the tooth led to the eruption of the permanent incisor in its position.Surgical exposure and elimination of mechanical obstruction is frequently the treatment of choice and spontaneous eruption can then be expected.Early diagnosis of odontomas is important for preventing craniofacial and tooth developmental problems. The early diagnosis accompanied by a proper treatment at the right time will result in a favorable prognosis. In order to diagnose developmental abnormalities as soon as possible, a professional team of pediatric dentists should be aware of the importance of clinical and radiographic examinations.


For a long time, chordoma has been known as an osseous tumor mainly found at the clivus and sacrococcygeal region. However, spine extra-osseous chordoma (SEC) with a better prognosis than the classic type has been neglected. According to our literature review, only several case reports have been published in English literatures. Here in this article, three cases of SEC, plus a literature review, are presented.Three cases of SEC were presented from our center. Surprisingly, neurologic tumors were considered as the first diagnosis. Thereafter, en bloc resection was performed in all the three cases. Especially, the dumbbell-shaped one in the cervical spine was removed by en bloc through the combined anterior and posterior approach for the first time. Follow-up within 12–58 months after surgeries proved no recurrence or metastasis.Spine extra-osseous chordoma, commonly located in the cervical and epidural region, is extremely rarely met. SEC is characterized with less aggressiveness, the lower rate of recurrence and metastasis, and better prognosis than those of the osseous origin. Though complete excision can be achieved generally, differential diagnosis of spine neurogenic tumors and the following en bloc resection should be made as carefully as possible. Chordoma is a type of relatively rare tumor with an incidence of about 0.8 per million , charactRarely, some chordomas of the spine present no bone involvement, a condition that we call spine extra-osseous chordoma (SEC). SEC, for a long time, has been neglected because of its rather low incidence. Referring to the literatures published in English, 17 cases –21 of SEFrom 1991 to 2014, 251 spine chordoma surgeries were conducted in our spine tumor center, only three cases being SEC  demonstrated that a giant dumbbell-shaped mass was located in the right intervertebral foramen of C4 and C5, expanding from C4 to C5 vertebra, with rare involvement of the bone. Inevitably, the spinal cord was compressed. Moreover, hypointense signals on T1 and hyperintense signals on T2 were witnessed Fig. , b, as wTo make the preliminary diagnosis of this special mass and a better treatment plan, we first carried out the needle biopsy. The pathological result surprised us that a chordoma existed. In the light of this condition, it was extraordinary for a dumbbell-shaped mass to be diagnosed as chordoma. We believe that it may represent an uncommon morphology of chordoma: spine extra-osseous chordoma (SEC).Justified with these preoperative experiences, a posterior-anterior approach was projected for the total en bloc resection of this tumor. Eight lateral mass screws were placed in the lateral masses of C2–3 and C6–7, followed by the left rod fixation and the osteotomy of spinous processes at C4–5. Subsequently, the intraspinal  capsuled part was dissected from the compressed dura matter but without removal. The involved right fifth nerve root was ligated. The posterior approach was done after fixation and suture.Then, the patient was placed in the supine position for the anterior approach. When exposing the surface of C3–6 vertebras, a big capsulated mass came into sight. The right vertebral artery was embraced. Taking the aggressiveness and high recurrence rates of chordoma into consideration, ligation of the right VA was performed. After that, with a sagittal excision of C4–5 vertebras, total resection of the tumor was finished successfully, with an appropriate titanium mesh filled with allogeneic bone and a suitable titanium plate placed at C3 and C6 vertebra. The dumbbell-shaped tumor was totally resected without intraoperative complications.Normally, the resected mass was well-capsulated. Microscopically, the mass was composed of lobules full of vacuolated physaliphorous cells rich in intra-cytoplasmic mucin. There was no evidence of mitotic activity. The immunohistochemical result was positive for the existence of S-100, epithelial membrane antigen (EMA), CK (pan), and vimentin. The Ki-67 index was about 5 % Fig. –e. AccorThe patient was able to walk around with the help of a collar 3 weeks after the operation. His discomfort, numbness, pain, and weakness of the lower extremities dwindled soon after the operation, without any perioperative complication. To examine the surgical efficiency, we advised him to take radiotherapy. The cervical CT and MRI at 12 months after the surgery demonstrated no recurrence of the tumor and the perfect fusion of the bone graft Fig. . FurtherWe carried out the literature research by retrieving the keywords like intradural, intraspinal-extraspinal, intraspinal, extra-osseous, and dumbbell-shaped chordoma. What is more, the abstracts were scanned by two authors to choose the suitable articles, and then they read the contents and the references carefully for other possible reports. Sixteen articles containing 17 SEC cases , vimentin, and S-100. With antibodies against carcinoembryonic antigen (CEA), aktin, and desmin, negative reactions were observed. Ki67, a proliferation index, was recorded only in two cases (about 1 %).Chordoma, comprising 1 to 4 % of all primary bone malignancy, is a type of locally aggressive tumor predominantly involving the sacrococcygeal and the clival region. Only 10–15 % of the chordomas are located in the mobile spine, in particular, the cervical region. Histologically, chordomas are classified into conventional, chondroid, and dedifferentiated variants. The chordoma is well-known for its slow-growing but strong local aggressiveness and high recurrence rate. At present, therapeutic options mainly include surgery, radiotherapy, and targeted therapy. Chemotherapy was seldom used. Surgery, especially the total en bloc spondylectomy, is thought to be the most preferred treatment for chordoma even at the cost of neurological function , 22–24.Chordoma is characterized with bone involvement. Yet, the 20 SEC cases showed intradural or epidural  or minimal secondary bone involvement. As was illustrated by MR, 8 of the 20 cases presented hypointense to isointense signals on T1-weighted image, and 6 showed hyperintense signals on T2-weighted image. Furthermore, 9 of them showed enhancement after the injection of gadolinium. These features were similar to those of neurinoma. Before the operation, 6 cases were first misdiagnosed with neurinoma. Others misdiagnosis included arachnoid cyst, meningioma, and myxopapillary ependymoma. Therefore, for their special locations , shapes and similar MR manifestations, the radiographic differential diagnosis between SECs and neurogenic tumors becomes quite difficult. Despite this, differential diagnosis between them must be taken into consideration in the face of possible neurogenic tumors.Histologically, the main body of classic chordomas contains normal physaliphorous cells and lobulated structures. In the cases collected in our study, most of the histological and immunohistochemical features were consistent with those of the classic chordoma. Positive immunoreaction was strong for the appearance of cytokeratin, epithelial membrane antigen (EMA), vimentin, and S-100. Other positive immunohistochemical findings included the platelet-derived growth factor receptor (PDGFR) α and β, Ki67. Yet, antibodies against CEA, aktin, and desmin did not show up. Few studies –27 reporThe postoperative recurrence, approximately 17 to 35 %, is thought to be a very important predictive prognosis indicator for classic chordomas. Additionally, classic chordoma has the potentiality to metastasize to the lung, bone, and liver in about 3 to 48 % patients , 30–32. In summary, SEC is an extremely rare and special subtype of chordoma with the following characteristics: (1) the absence of bone involvement; (2) the similar morphology with neurogenic tumors; (3) the low aggressive biologic behavior; (4) a better prognosis than the classic chordoma. Because of the limited quantities, further studies are required to better understand SEC’s origination, immunochemistry, differential diagnosis, biologic behavior, and prognosis.Spine extra-osseous chordoma, usually located in the cervical and epidural region, is extremely rare. SEC is characterized with the less aggressiveness, the lower rate of recurrence and metastasis, and the better prognosis than those of the osseous origin. Though complete excision can be achieved generally, differential diagnosis of spine neurogenic tumors and the following en bloc resection should be made as carefully as possible.


Bronchogenic cysts are uncommon, predominantly benign, congenital malformations arising from the primitive foregut. The occurrence of such cysts in the retroperitoneum is extremely rare. The present study presents the case of a 30-year-old female who presented with a left adrenal mass. Imaging investigations revealed a cystic mass located medially to the left adrenal gland. Retroperitoneal laparoscopic excision and complete resection were performed, and the subsequent pathological examination confirmed the diagnosis of a bronchogenic cyst in the retroperitoneum. The patient was discharged on the fourth post-operative day and received no further treatment, however, regular follow-up was performed due to the lesion being benign. A rare case of bronchogenic cyst and literature review is presented, which may aid in improving the understanding of the etiology and pathogenesis of retroperitoneum bronchogenic cysts. Bronchogenic cysts are foregut-derived congenital abnormalities that occur following the third week of embryonic life . When atA 30-year-old female was referred to the First Hospital of Jilin University  for the evaluation of a left adrenal mass, which was identified incidentally during an examination for a persistent fever, associated with coughing and expectoration lasting for three days. A computed tomography (CT) scan of the abdomen revealed a cystic mass measuring 32 Hounsfield units, with a diameter of ~1.9 cm, located medially to the left adrenal gland. However, the cystic mass was not clearly demarcated from the upper pole of the left kidney . An enhaThe patient had no palpitations, diaphoresis, weakness or hypertension. In addition, no other significant medical history was noted. The patient’s endocrine evaluation results for adrenal hypersecretion were negative, and the results of testing for the renin ratio, aldosterone, plasma and urinary catecholamines and metanephrines were all within the normal ranges. The patient was admitted for surgical removal of the mass, and the laparoscopic surgery revealed a 1.5×2.0×2.0-cm cystic structure with a complete capsule, which was adherent to the upper pole of the left kidney. The cyst was located deep within the retroperitoneum in the immediate vicinity of the medial margin of the adrenal gland and adjacent tissue. The pathological evaluation revealed a cystic mass filled with a white seromucinous fluid. In addition, the histopathological examination revealed that the cyst was partially lined with ciliated pseudostratified epithelium, with the cyst wall containing a small number of seromucous glands .The patient had an uneventful post-operative recovery and was discharged on the fourth post-operative day. The patient received no further treatment, however, regular follow-up was performed due to the lesion being benign.When the tracheobronchial tree undergoes abnormal budding and pinching off at approximately week five of gestation, bronchogenic cysts develop . If the Although bronchogenic cysts are rare, such cysts must be considered in the diagnosis of a retroperitoneal mass. However, the pre-operative diagnosis remains a clinical issue. Retroperitoneal bronchogenic cysts may easily be misdiagnosed as adrenal cortical or medullary tumors, or enteric, urothelial or pancreatic cysts by their clinical and radiological presentation. However, a histological diagnosis may differentially determine a bronchogenic cyst from such lesions. Microscopically, bronchogenic cysts are predominantly unilocular or oligolocular, lined by pseudostratified ciliated columnar epithelium with bronchial glands, mucoid material, cartilage and smooth muscle ,8. In thThe mainstay of treatment for retroperitoneal bronchogenic cysts is surgical removal. Although the majority of cysts are asymptomatic and exhibit benign behavior, excision is recommended to establish a diagnosis, to alleviate any symptoms and to prevent infection and the documented risk of malignant transformation . AccordiIn conclusion, the current study presents a rare case of a retroperitoneally localized bronchogenic cyst as an unusual differential diagnosis of a retroperitoneal tumor. The combination of clinical, biochemical and radiological features may aid in the characterization of lesions, however, only a histological analysis can currently provide a definite diagnosis.


Buschke-Lowenstein tumor is a relatively rare sexually transmitted disease. It is a neoplasm of the anogenital region which has benign appearance on histopathology but is locally destructive. It carries a high recurrence rate and a significant potential for malignant transformation. Human papilloma virus has been implicated as an etiologic agent for this tumor. Since this disease is rare and no controlled studies exist, radical excision of this anogenital lesion is generally recommended as the first line therapy and close vigilance and followup are essential. We have discussed an overview of etiopathogenesis, clinical presentation, diagnosis, and management of this uncommonly encountered disease. A 65-year-old man presented to the hospital with 1-week history of bloody and foul smelling discharge from his groin. The patient had initially noticed a small lesion of approximate dimension of a pea in his right inguinal area about 20 years ago. Since the lesion did not bother him at that time, he did not seek medical care until when he started to notice blood oozing from it. The lesion had increased in size multifold over the years and had extended into the left groin and the perineal region. It was not painful, but caused some discomfort mainly during sitting. He reported no fevers, loss of weight, urinary symptoms, or difficulty in defecation and denied any other skin lesions elsewhere. All other reviews of systems were essentially unremarkable. He was in a monogamous relationship with his wife who had passed away 10 years prior to presentation. He denied previous promiscuous behavior.3), hemoglobin 14.3 gm/dL, and platelet count 284,000 per mm3. Renal and liver function tests were normal.On examination, patient was afebrile and hemodynamically stable. An extensive, foul-smelling cauliflower-shaped lesion was apparent in the inguinal regions bilaterally. It measured around 15 cm in width in each groin along the maximum dimension, and about 40 cm in length, extending from the waist line through the groin into the intergluteal cleft towards the rectum . It had Human immunodeficiency virus and rapid plasma reagin serological tests were nonreactive. Urine analysis and blood cultures were unremarkable. Contrast enhanced computed tomography of chest, abdomen, and pelvis was done that did not reveal any regional lymph node involvement or evidence of distant metastasis.Based on the clinical presentation, a diagnosis of giant condyloma acuminatum of Buschke-Lowenstein was suspected. The lesion was radically excised and the entire specimen was sent for gross pathological examination  and histA final diagnosis of condyloma acuminata of BLT  was made. The patient has not had a recurrence of the tumor for 3 years from the time of radical excision.Giant condyloma acuminata was first described by Buschke and Loewenstein in 1925 in the penis and they named it “condyloma acuminate carcinoma-like” . While tIt is a sexually transmitted disease with an estimated incidence of about 0.1% in the general population . Human pTo confirm histopathologically, deeper tissue must be biopsied to ensure that no malignant cytological characteristics are missed in superficially biopsied specimens . RadicalWhile a variety of treatment modalities , carbon dioxide laser therapy, and photodynamic therapy) have been used for the treatment of BLT, wide surgical excision by Mohs technique is recommended as the most important therapeutic intervention . Since tSince radical excision was achieved with peripheral and deep margins free of tumor and no regional lymphadenopathy was noted, the oncologist did not recommend additional modalities such as chemotherapy in our patient. Vigilant and prolonged surveillance was suggested. This is important because this tumor has been reported to have a significant rate of recurrence (66%) and malignant transformation (56%) with an overall mortality of 20% . The tumSince all lesions of BLT initially start as condyloma acuminata and progress over many years, this entity is likely preventable to an extent by vaccination against certain strains of HPV and seeking timely medical attention. Our case adds to this relatively rare disease and highlights the importance of timely detection, aggressive management, and close surveillance to improve patient outcomes.


Meningiomas are common benign tumors with accepted treatment approaches and usually don't challenge healthcare specialists. We present a case of a huge unresectable hemicranial meningioma, which was successfully treated with hypofractionated irradiation.A male patient, sixty-two years of age, suffered for over 12 years from headaches, facial deformity, right eye displacement, right eye movement restriction, right-sided ptosis, and facial hypoesthesia. MRI and CT studies revealed an extended hemicranial meningioma. Prior to irradiation, the patient underwent four operations. Eventually, the tumor was irradiated with the CyberKnife in August 2009. Tumor volume composed 1085 cc. The mean dose of 35.3 Gy was delivered in 7 fractions (31.5 Gy at 72% isodose line comprising 95% of tumor volume). The patient was followed during six years and experienced only mild (Grade 1-2 CTCAE) acute skin and mucosa reactions. During the follow-up period, we observed target volume shrinkage for 17% (for 26% after excluding hyperostosis) and regression of intracranial hypertension signs.Due to the extreme volume and complex shape of the tumor, spreading along the surface of the hemisphere as well as an optic nerve involvement, the case presented would not be generally considered suitable for irradiation, especially for hypofractionation. We regard this clinical situation not as a treatment recommendation, but as a demonstration of the underestimated possibilities of hypofractionation regimen and CyberKnife system, both of which are limited with our habit of conventional treatments. Meningiomas are common benign tumors, which usually can be successfully managed with standard treatment options (surgery or/and irradiation). Contemporary surgical and irradiation methods provide high tumor growth control rates with low morbidity and mortality -3.Nevertheless, despite extensive clinical experience, there are situations when standard approaches are inapplicable. In such cases, healthcare specialists rely on their previous experience, on their assessment of the clinical situation, and some radiobiological calculations to provide optimal treatment.In this paper, we present a case of successful CyberKnife hypofractionated irradiation of a large complex-shaped meningioma.In 2009, a 62-year-old male patient was referred to the Radiation Oncology Department of the Burdenko Neurosurgical Institute. He had been previously treated with consecutive surgical resections of a right-sided cranio-orbital meningioma in 1998, 2000, 2001, and 2009. All histological investigations showed a typical WHO Grade I meningioma with an infiltrative growth pattern. At the time of admission, he had headaches, facial deformity, right eye displacement, right eye movement restriction, right-sided ptosis, and right-sided facial hypoesthesia. Signs of intracranial hypertension were identified with an eye fundus study. Informed patient consent was obtained for treatment.MRI with contrast enhancement revealed right-sided extended calvarial, skull base, and facial meningioma. The tumor caused calvarial, skull base, and facial hyperostosis, infiltrated chewing muscles, and thickened meninges and periosteum, up to 15 mm. Due to massive unilateral meninges thickening, septa pellucida displacement was found.The patient was treated with the CyberKnife. Tumor volume composed 1085 cc. Due to hyperostotic changes of the skull, the target included both soft tissue (702 cc) and bone components (383 cc). The following treatment regimen was chosen: 35 Gy total median dose in 7 fractions with 48-72 hours interfraction interval. Two conic collimators with 50 and 25 mm aperture diameter were chosen for treatment delivery. The acceptable treatment plan was generated after several cycles of alternation of iterative and simplex algorithms , skin hyperemia , and oral mucositis (Grade 2 CTCAE 4.02). All reactions regressed spontaneously or with a short course of medications.The patient was followed for six years after his treatment with consecutive MRI studies and clinical evaluations. No late reactions were detected during this period. After treatment, the patient noticed headaches ceasing and partial regression of his facial deformation. Follow-up neuro-ophthalmological evaluation six months after treatment detected regression of the intracranial hypertension signs.Follow-up images revealed tumor shrinkage six years after the treatment Figure . Tumor sWe presented a rare case of a huge meningioma invading half of the skull. Despite extreme volume and extent, the tumor caused only mild symptoms and slightly affected life quality. We supposed this was due to the slow growth rate. Tumor extent, benign behavior, and patient status were regarded as contraindications for surgery. After interdisciplinary consultation, we proposed irradiation. To our knowledge, it is the only published case of hypofractionated irradiation of such a large intra-extracranial tumor.Because of the uniqueness of the clinical situation, the most difficult part was to select an optimal treatment facility and treatment regimen.Due to department equipment with Gamma Knife, CyberKnife, Novalis with ExacTrac, and Primus, we had different opportunities for treatment delivery. CyberKnife was chosen due to several reasons. The first was irradiation with multiple non-isocentric, non-coplanar beams. The beams could be directed tangentially for more favorable dose distribution in this roughly hemispherical tumor. The second reason was the inverse treatment planning algorithm, which was favorable in this complex tumor. Hypofractionated irradiation with the CyberKnife was demonstrated to be effective for meningiomas -5. We suOur department practice is to prescribe the mean dose. The mean dose of 35 Gy in 7 fractions was assigned. After treatment planning, it was 31 Gy for 72% isodose line covering 95% of the target volume. Since that time, we have reduced hypofractionation doses for meningiomas. Now, we would rather treat this tumor with 5 fractions of 5.5 Gy (mean dose) .When treating, we expected several possible complications: severe skin reaction, brain tissue edema, and optic neuropathy. Fortunately, only mild reversible reactions were registered: alopecia (Grade 2 CTCAE 4.02), skin hyperemia , and oral mucositis (Grade 2 CTCAE 4.02). The key features which allowed us to diminish adverse reaction probability are: irradiation conformity and selectivity ; treatment support with medications provided ; increased interfraction interval; original tumor location, and growth along the brain surface without forming nodes invading the brain. We suggest that increased interfraction interval (for 48 or 72 (for weekend) hours) could also provide additional normal tissue sparing. Meningiomas are characterized by low a/b ratio (around 3.3) . Larger The case presented appeared to be unique. However, each year we observe several cases which present similarities to the presented one. These also seem to be treatment challenge. We believe that such cases are not local problems associated with low MRI availability. Rather, the fact of detection of such meningiomas is determined by tumor biology. These subtypes of meningiomas are characterized by slow, long-term asymptomatic tumor growth along the meninges.  We reviewed published papers and did not find similar cases. Nevertheless, there were successful radiation treatments (radiotherapy and radiosurgery) reported, which still may be considered risky.Deibert and Kondziolka reported a case of radiosurgical irradiation of a meningioma filling the posterior two-thirds of the superior sagittal sinus . Target Ganz, et al. reported a series of 97 patients treated with Gamma Knife radiosurgery for large meningiomas of 10-43 cc (mean - 15.9 cc) with 12 Gy marginal dose . Three cWe present a case of successful hypofractionated irradiation of a huge hemicranial meningioma with the CyberKnife system. While the outcome in this patient was positive, more experience is essential before we recommend it as an accepted clinical alternative. The suggestion is not to simply copy our experience but to take it into consideration when discussing similar clinical situations.For decades, the territory of radiosurgery and standard fractionation has been thoroughly investigated. As a result, radiation oncologists and radiosurgeons have stated limitations of practical safe treatment and comfort level when performing it. Overcoming these constraints could increase therapeutic capabilities and allow treating of unusual tumors. For the moment, hypofractionation appears to be “terra incognita”, which still should be discovered and conquered.


Adrenal tumors are detected incidentally in 4 to 8% of patients in imaging studies. Adenomas, pheochromocytomas and adrenocortical carcinomas represent the most common tumors of the adrenal glands. Rarely are final histopathological reports are surprising.The aim of our study is a retrospective analysis of selected clinical characteristics and hormonal studies in five cases of rare adrenal tumors.We present five interesting cases of adrenal tumors: two medullary hyperplasia, one adenomatoid tumor, one hydatid cyst and a primary angiosarcoma of the adrenal gland. The final diagnosis was established by means of microscopic examination of the specimens.The number of adrenal tumors was increased due to widespread use of imaging procedures. In patients without any known extra-adrenal malignancy most lesions are benign, non-hyper functioning adenomas. Adrenal tumors should be evaluated biochemically and radiologically. Adrenal tumors are detected incidentally in approximately 4 to 8% of patients in imaging studies. AdenomaTwo patients were admitted to our hospital in order to examine the cause of muscle weakness headaches, perspiration, palpitation and facial redness. A 46-year-old male presented with hypertension of 15-months duration (case one). The patient’s family had no history of hypertension, cardiac arrhythmias or thyroid disorders.A 34-year-old female was referred for the evaluation of palpitation and facial redness of one-year’s duration (case two). The patient’s parents and sister had no history of hypertension or other endocrine disorders.On admission, there were no cushingoid signs. Paroxysmal hypertension had occurred, and was poorly controlled by antihypertensive drugs in both cases. Levels of urinary catecholamines were increased during the paroxysmal hypertension . A 47-year-old woman with two year history of arterial hypertension was referred to our department in 2003. A general examination revealed high blood pressure (180/100 mmHg). No other physical signs suggestive of hypercortisolism were noted. A blood analyses showed hypereosinophilia  of the adrenal gland, clinically suspected to be a malignant tumor. The right adrenal tumor was found incidentally on a routine medical examination in 1997. There was no family history of any endocrine pathology and she did not present with any symptoms of palpitations, diaphoresis or flushing. Laboratory examinations revealed that the urinary vanilinmandelic acid, catecholamine and cortisol levels all were within normal limits . AMH, when present in patients with familial MEN type 2 (MEN2), usually occurs bilaterally. The occurrence of unilateral AMH not associated with MEN is extremely rare and has been documented only in several cases. This hyThe patients described in this study had AMH without evidence of MEN2 or a family history of pheochromocytoma. A pathologic study showed diffuse adrenal medullary hyperplasia that decreased the cortico-medullary ratio. Because of the well-recognized metachronous nature of the adrenomedullary lesions, patients will maintain prolonged follow-up after successful unilateral adrenalectomy, 4, 6. NEchinococcus granulosus; much less common is Echinococcus multilocularis. Hydatid cysts occur throughout the world but are endemic in the pastoral and farming regions of the Mediterranean, Eastern Europe, the Middle East, South America, Australia and South Africa[Hydatid disease (Echinococcosis) is a parasitic infection caused by several species of the cestode Echinococcus. The most common form is h Africa, 9.Hydatid cysts may be found in almost any part of the body, but most often they are found in the liver and lungs. A hydatid cyst of adrenal gland is very rare; this entity is found in only 7% of all adrenal cysts. Parasitic cysts involving the adrenals are usually secondary and are part of generalized Echinococcosis. Rarely is Echinococcus infection limited to the adrenal gland. Only a few cases of primary hydatid cyst of the adrenal gland have been reported. Our patSince most adrenal cysts are asymptomatic, they are usually incidental findings on imaging studies or discovered incidentally during surgery performed for other abdominal pathologies. The sensitivity of a CT scan in an abdominal hydatid cyst is 97%. To identify a hydatid cyst, CT and/or magnetic resonance imaging can reveal a cystic lesion and the presence of daughter cysts, 9. On CThe definitive treatment method for hydatid cysts of the adrenal gland is surgical excision. We qualPAA is an extremely rare aggressive neoplasm. The first example of PAA was described by Kareti in 1988–14. PatiAngiosarcoma is a local invasive neoplasm. An open adrenalectomy and block resection approach would have been chosen for patients suspected of having PAA. This tumor is derived from vascular endothelial cells, therefore the traditional chemotherapy agents ifosfamide and epirubicin were used in combination with anti- angiogenic drugs bevacizumab or sorafenib. The chemotherapy plan for patient four was doxorubicin and ifosfamide but she refused the treatment. Bevacizumab is effective for angiosarcomas in 57% patients. Sorafenib provides a better progression-free survival, but the response rate is lower than standard cytotoxic agents. Radiation therapy may be not effective. In the AT is a rare benign neoplasm of mesothelial origin which usually occurs in the genital tract of both sexes. Occasionally these tumors are found in other locations such as the heart, pancreas, skin, pleura, omentum, lymph nodes, retroperitoneum, intestinal mesentery and adrenal glands. So far in the English literature only 27 cases of adrenal AT have been described. Most paATs are usually asymptomatic. In the available literature only one patient had symptoms which could be connected to the adrenal mass. In our patient (case five) the adrenal tumor was found incidentally.The adrenal AT tumor can mimic all other entities occurring in this area such as adenomas, carcinomas, pheochromocytomas, myelolipomas, benign cystic lesions and metastases from distant locations. It may also be easily misdiagnosed as a vascular neoplasm (lymphangioma or angiosarcoma). The immunohistochemical profile was consistent with the one described in AT in usual locations  and was typical for a tumor of mesothelial origin: calretinin (+), CK(cytokeratin) 5/6 (+), CK 7(+), vimentin (+), D2-40 (+), CD31(-) and CD34 (-).Numerous irregular cystic spaces lined with flattened cells may suggest a vascular neoplasm, especially lymphangioma. However, the cells are positive for CK and they lack reactivity to the usual vascular markers CD31, CD34, factor VIII, which exclude vascular tumors. It is important that AT may show reactivity to D2-40, a marker which is known to be positive in the endothelial cells of lymphatic vessels. In at least two cases reported in the literature, AT were primary misdiagnosed as lymphangioma cavernosum–18 similRadiology has the important role in the detection of adrenal masses. CT is usually the primary imaging modality for the differentiation of adrenal masses. A large tumor diameter, unclear boundaries, infiltration of the neighboring structures, non-homogenous density and the presence of a thick, irregular peripheral zone becoming enhanced after contrast administration may suggest a malignant character of the lesion, however the specificity of those criteria does not exceed 75%, 2. MostA medical history and physical examination may suggest possible cause. For example, paroxysmal hypertension may suggest a hormonally active mass, like in our AMH cases. Evaluation of hormonal excess should be done when imaging characteristics do not univocally suggest a necessity for surgery.The clinician and surgeon must make the decision to operate jointly, trying to achieve the best calculus of what is known and predicted. Finally, histopathological examinations could be surprising.In the differentiation of adrenal tumors, we should first take into consideration benign lesions, followed by malignancies, metastases and pheochromocytomas.The suggestion of malignancy in CT scans was the reason for total adrenalectomy in three our cases.Hormonal activity inclined us to laparoscopic adrenalectomy in two cases.Finally, in all cases the final histopathological reports were surprising and unexpected.Written informed consent was obtained from the patients for publication of this report and any accompanying images.


Intravascular papillary endothelial hyperplasia or Masson's tumor is a rare reactive disease of vascular origin characterized by exuberant proliferation of endothelial cells notably occurring within blood vessels of head, neck, and extremities. The importance of this entity is its ability to mimic a variety of diseases both benign and malignant in the orofacial region. Here, we present a case of Masson's tumor within the masseter muscle in a 40-year-old female with emphasis on the sequential investigative procedures performed to diagnose this entity. Vascular tumors in the oral region have been traditionally described as hamartomas or malformations rather than as true neoplasms. Stout stated that a vascular tumor, in contrast to a hamartoma, contained more endothelial cells than was necessary to line the lumina. Clinically, vascular lesions have a tumor-like appearance, due to endothelial proliferation in vessels and enlargement of vessels with secondary reactive change . IntravaA 40-year-old female patient reported with a complaint of pain and increased swelling on the right side of the face of 1-week duration. The swelling was first noticed by the patient on the right cheek region 4 years back and was not associated with any trauma or chronic irritation. The swelling had gradually increased in size and was asymptomatic until a week before reporting to us during which the patient claimed that the swelling exhibited rapid increase in size and was associated with continuous moderate pain which aggravated on manipulation. No meal time variation in size of swelling was noted and patient did not give history of dryness of mouth. The patient's medical history was otherwise noncontributory.Clinical examination revealed a solitary diffuse extraoral swelling on the right cheek, measuring about 2 × 2 cm. The skin overlying the swelling was normal with no secondary changes. On palpation, the swelling was tender, with no local rise in temperature, soft to firm in consistency, and lobulated with a smaller hard nodule palpable at the inferior portion of the swelling . The sweSoft tissue radiograph of the lesion using puffed cheek technique revealed 3 well-defined radiopaque concentric calcifications which were also visible on posteroanterior skull view and on the panoramic radiograph they were superimposed over the right mandibular third molar region Figures , and 5.Ultrasonographic examination of the lesion revealed a fairly well-defined hypoechoic mass in the right cheek devoid of vascularity. Three foci of calcifications were noted within the right masseter muscle. The right parotid gland was normal in size and architecture Figures .CT revealed a well-marginated round to oval iso to hyperdense lesion seen anterior to the right masseter muscle measuring 1.5 × 1.3 cm with well-defined planes and attenuation values of around 100 HU. The lesion was seen to have continuation with anterior aspect of the masseter muscle which also exhibited irregular hyperdense calcific foci Figures .MRI revealed a well-defined isointense lesion adjacent to the anterior aspect of the right master muscle, appearing isointense on T2 and T1 sequences; also, hypointense foci with fatty infiltrations involving right masseter muscle were noted Figures .FNAC yielded a thick brown material containing sheets of hemosiderin laden macrophages, many spindle cells, and degenerated erythrocytes.Excisional biopsy was performed under general anesthesia employing stringent aseptic conditions and endotracheal intubation. Considering the location of the lesion, that is, the muscular plane in relation to masseter muscle, an extra oral submandibular approach was chosen to perform the biopsy.Right submandibular incision measuring about 6 cm in length was marked to gain access to the masseteric area. Local anesthesia with adrenaline was infiltrated at the incision site. After the initial skin incision, parotidomasseteric fascia was then incised to gain access to the lesion within masseter muscle; care was taken not to involve any branches of facial nerve and blunt dissection was used. Once the masseter muscle was accessed, it was explored using blunt dissection and the masses of the lesion were localized. The lesion was found to have feeding blood vessels which were ligated before excision. The surgical site was checked for any residual bleeding keeping in mind the suspected nature of lesion. Upon achieving hemostasis, the surgical site was irrigated with povidone iodine solution and saline; suturing was done in layers using 3-0 vicryl and 4-0 prolene sutures .The excised lesion grossly 3 × 3 cm in size was reddish blue in colour and firm in consistency, with small feeding blood vessels .Histopathological examination revealed a dilated vein with lumen showing papillary processes and anastomosing channels lined by benign endothelial cells. Fibrin deposits and areas of hemorrhage were also seen. No evidence of increased mitotic activity was observed and no atypia of the endothelial lining was evident Figures .A final diagnosis of intravascular papillary endothelial hyperplasia/Masson's tumor was made.In 1922, Ewing described a rare and obscure intravascular endothelioma in the corpus cavernosum. He also described some cases of encapsulated, slow growing, subcutaneous tumors with abundant endothelial cells occurring in dilated varicose veins with previously obstructed circulation. These tumors grew in broad papillary masses formed by enlarged cells with hyperchromatic nuclei. In 1923, Pierre Masson first described an intravascular papillary proliferation formed within the lumen of inflamed hemorrhoidal plexus in a man which he believed was a neoplastic lesion and which he called Hémangioendothéliome végétant intravasculaire. Henschen described similar changes in nasal and laryngeal vessels which he considered to be a reactive phenomenon , 5.Various terminologies have been put forth by different authors for this lesion including papillary fibroendothelioma and intravascular endothelioma, papillary proliferation of the endothelium, papillary endothelioma, Hémangioendothéliome végétant intravasculaire, L'endovasculite proliférante trombopoiétique, intravenous atypical vascular proliferation, intravascular angiomatosis, intravascular papillary endothelial hyperplasia, Masson's vegetant intravascular hemangioendothelioma, Masson's pseudoangiosarcoma, intravascular endothelial hyperplasia, Masson's lesion, and papillary endothelial hyperplasia . The mosThe pathogenesis of IPEH is poorly understood. One possible mechanism is a benign neoplastic process involving endothelial cell proliferation and papillary formation in the vascular lumen that undergoes degeneration and necrosis in the manner of a red infract. Alternative mechanisms include a benign endothelial proliferation arising from a thrombus as a variant of angiolymphoid hyperplasia with eosinophilia, a reactive process of endothelial cells induced by blood stasis and perivascular inflammation, and a pseudotumoral lesion caused by endothelial proliferation with papillary formation proceeded by an accumulation of thrombotic material, which serves to facilitate development of the lesion .Various putative factors have been suggested preceding the appearance of the lesion including minor trauma ; Pins etInoue et al. cited classification of IPEH by Hashimoto et al. as .Type I is also called the primary or pure form which occurs within dilated vascular spaces. It is the most common subtype .Type II is a secondary or mixed form that occurs in preexisting varices, cavernous or capillary hemangiomas, pyogenic granuloma, lymphangiomas, arteriovenous malformation, and blue rubber bleb nevus.Type III or the undermined type is found in an extravascular location, the lesion develops in the bed of a hematoma and trauma is usually a prerequisite. It is the least common variant.The IPEH in our case was a pure or Type I variant, as it was found to arise from a dilated vein.Clinically, IPEH presents as a firm, tender, two-to-four cm nodule or mass with slight elevation and slow growth. It is usually characterized by red or blue discoloration of the overlying mucosa. This tumor most often occurs in the head and neck region 23%), lower extremities (17%), and fingers (16%) ; the rar%, lower The lesion has been found to have a female preponderance, as in our case, with a male/female ratio of 1 : 1.3 and based on this finding a possible hormonal role has been suggested. The lesion commonly occurs in the middle age usually in the 5th decade with a mean age at onset being 42.6 years which was coinciding with the age of our patient .Clinically, the lesion has been mistaken for and should be differentiated from mucocele, hemangioma, lymphangioma, angiosarcoma, hematoma, Kaposi sarcoma, hemangioendothelioma, thrombosed vein, phlebectasia, traumatic fibroma, melanoma, fibroepithelial polyp, nonodontogenic soft tissue infection, intramassetric abscess, cysticercosis, benign neoplasms of smooth muscle origin, and reactive and neoplastic neural lesions like traumatic neuroma, neurofibroma, and neurilemmoma , 10, 11.IPEH can have varied appearance on imaging studies depending on the degree of thrombosis. In our patient, concentric calicifications were noted initially on conventional and digital radiographs. Performing an ultrasonographic study elucidated a well-defined cyst-like lesion located within the masseter. Color Doppler sonography revealed that the lesion lacked any obvious vascularity. Advanced imaging like CT and MRI enabled us to further identify the exact plane in which the lesion and associated calcific foci were located. The Hounsfield value of 100 HU and the isointense appearance on T2-weighted MRI indicated that the lesion was solid and not cystic. The lesion was also shown to have continuation with the right masseter muscle on CT and fatty infiltration of masseter on MRI which explains the difficulty in resecting the tumor mass alone.Studies have shown that some IPEH masses are homogenous on CT and MRI, whereas others are nonhomogenous. MRI characteristics, such as hypointensity on T1-weighted images, hyperintensity on T2-weighted images, and some degree of contrast enhancement, are nonspecific to IPEH .Such signals are probably related to the fact that lesions are likely to have varying amounts of both solid “parenchymal” tissue areas (papillary structures and thrombi) and anastomotic, stagnant, or low-flow vascular channels.Areas of microcalcification have been noted in IPEH lesions. The presence of calcification is said to be typical in IPEH and the involved vessels may either be occluded or patent . PhlebolWhen IPEH causes bone erosion as a result of pressure effects, its radiologic features can be even more suggestive of malignancy. Therefore, while radiologic investigations can assess the nature of the lesion and contribute to management, they are not sufficiently characteristic by themselves to make a diagnosis of IPEH .Thus, imaging plays considerable role in the diagnosis of IPEH to determine the extent of the lesion or its vascularity and guide the surgical management.Histopathologically, in cases of IPEH associated with thrombi, an organizing thrombus is observed in an expanded blood vessel. The endothelial cells proliferated in a papillary pattern towards the lumen of the enlarged blood vessel from the area of the organizing thrombus. The structure of papillary proliferation was covered with no more than 2 layers of endothelial cells, and no atypia or mitotic activity was seen around the cores of fibrous connective tissue, which were frequently hyalinized and hypocellular . HistoloSalyer and Salyer as referred by Robertson and Hernández, described the features which distinguish this lesion from angiosarcoma. (1) The lesions are well circumscribed and have a predominately intra- vascular location. (2) Extravascular lesions are associated with hematomas. (3) Rare but normal mitotic figures occur. 4) There is a lack of cellular atypia. (5) There are no necrotic areas. (6) Cells do not invade the perivascular space. (7) Solid areas exist with or without vascular differentiation. (8) Papillary fronds are one or two cells thick. (9) Papillary structures are mainly supported by a core of thrombotic material [ There isConstantino et al. as alluded by Robertson and Hernández hypothesized the stages through which the lesion progresses into 4 stages: the process probably starts with a recently formed thrombus and early endothelial proliferation occurs. Later, papillary projections develop when endothelial cells cover the irregular surface of the thrombus. In the more advanced stage, thrombotic material is slowly resorbed and papillary projections become smaller. At an end stage, the thrombotic material is completely resorbed and only a nodule composed of endothelial cells remains [Ki-67 (MIB-1) is a large nuclear protein preferentially expressed during all active phases of the cell cycle but absent in resting cells. Avellino et al. have demonstrated the presence of a small number of Ki-67 (MIB-1) positive cells in IPEH tumors by immunohistochemistry, suggesting that these are slow-growing benign neoplasms and not reactive growths .α-SMA, factor VIII, XIIIa, CD 31, CD 34, and ULEX europaeus agglutinin (UEA-1) corresponding to the endothelial cells being the proliferative component and the lesion having mesenchymal origin [Immunohistochemically, IPEH reacts with vimentin, l origin . CD 105 Sarode et al. cited various treatment modalities, most cases are cured by simple total excision with healthy margins. Cohen et al. used sclerotherapy  followed by surgery with good esthetic results and minimal intra-operative bleeding. Endoscopic surgery has been used to treat an extensive IPEH of the sinonasal cavity. Noninvasively IPEH has been successfully treated by the beta-adrenergic antagonist nebivolol .The prognosis of IPEH is excellent. Follow-up of some large series showed no evidence of local invasion or metastasis. Nevertheless, IPEH may recur if it arises in a primary vascular lesion which may itself recur or if the lesion is incompletely excised. Therapy in these cases should be planned according to the nature of underlying lesions. It has also been suggested that, in recurrent cases exhibiting strong immunolabeling of proliferative markers, the possibility of angiosarcoma should be investigated .In our case, surgical excision with clear margins was performed and there was no evidence of recurrence at 6-month follow-up.Intravascular papillary endothelial hyperplasia is a rare reactive lesion occurring notably in the head and neck region and can mimic many pathological lesions, both benign and malignant. Utilizing appropriate imaging modalities helps ascertain the location, proximity to vital structures, and the vascularity of the lesion aiding in diagnosis and surgical treatment planning, the gold standard for diagnosis being histopathological examination. It is vital to precisely diagnose this lesion so that it is neither inadequately nor aggressively managed both of which are crucial to prevent recurrence and morbidity.


Colocolic intussusceptions are rare clinical entities in adults and almost always caused by a leading lesion which often warrants resection. Mostly being malignant, the leading lesions are rarely benign lesions where intraluminal lipomas are the most frequent among them. Most adult intussusceptions require surgical resection owing to two major reasons: common presence of a leading lesion and significantly high risk of malignancy—reaching as high as 65% regardless of the anatomic site—of the leading lesion. Resection of the affected segment is usually the treatment of choice, since preoperative diagnosis of the lesion is usually ineffective and most leading lesions are malignant. This paper represents two cases of adult colocolic intussusception caused by intraluminal lipomas with a brief review of the literature. An intussusception is by definition the insertion of a proximal segment of gastrointestinal (GI) tract into the lumen of subsequent segment in a telescopic fashion . AlthougMost adult intussusceptions require surgical resection owing to two major reasons: common presence of a leading lesion and significantly high risk of malignancy—reaching as high as 65% regardless of the anatomic site—of the leading lesion , 4, 6. AHereby, we present two cases of adult colocolic intussusceptions owing to intraluminal lipomas of the colon of different anatomic sites with a brief review of the literature.A 53-year-old male patient without any known history of chronic comorbidities or surgery presented with one-month history of upper abdominal discomfort and meteorism accompanied by seldom vomiting. The complaints were reported to appear roughly two hours after meals and lasted for hours. With an initial diagnosis of biliary colic and dyspepsia he was examined with abdominal ultrasonography, which revealed a pseudo kidney image in the transverse colon and showed no other abnormalities.Further examination with abdominal tomography revealed diffuse thickening of the transverse colon wall along a 9 cm segment and colocolic intussusception . EndoscoUnder these findings the patient underwent left hemicolectomy with end-to-end anastomosis. The postoperative course was uneventful and the patient was discharged on the 6th postoperative day.Histopathologic examination of the specimen revealed a lipomatous lesion with a diameter of 5 cm of pure benign nature and 11 lymph nodes without any signs of malignancy.A 51-year-old female patient without any known history of chronic comorbidities or surgery presented with progressive distention for over 20 days accompanied by vomiting and vague pain in the left lower quadrant of the abdomen. She was examined with a barium enema study revealing an intraluminal mass in the sigmoid colon, before admission .She was further examined with colonoscopy again revealing an intraluminal mass accompanied by signs of intussusception.She underwent sigmoid resection with an end-to-end colorectal anastomosis and did well on the postoperative course. She was discharged on the 4th postoperative day.Histopathologic examination of the specimen revealed a polipoid lesion of 4 cm in diameter with slight ulceration consisting of pure fatty cells without any signs of malignancy. Examination of 9 lymph nodes again showed no signs of malignancy.Intussusception is a very rare cause of intestinal obstruction in adults constituting only 5% of all bowel obstructions . Almost Since adult intussusceptions are rare clinical entities, a high index of clinical suspicion is essential in diagnosis. Contrary to pediatric intussusceptions, ultrasound imaging is rarely useful in adult population. With colonoscopy still being the golden standard of diagnosis, computerized tomography proved to be the most useful diagnostic method in adult intussusceptions, usually revealing a smooth, well demarcated sausage shaped mass .Lipomas of the colon are rare tumors of mesenchymal origin, composed of well-differentiated adipose tissue, that rarely become symptomatic and are usually detected incidentally , 9. TheyIn conclusion, colocolic intussusceptions in adults are rare clinical entities with vague and nonspecific symptoms. Almost always they are caused by a leading lesion, which is commonly malignant. Lipomas of the colon are rarely encountered as the leading point but they should be resected nonetheless since malignancy cannot be totally excluded in large lesions.


Torsion of the omentum is a benign self-limiting disorder, which is difficult to diagnose because the main symptoms are similar to those of other abdominal diseases. Most of the published cases had been diagnosed during operation via direct eye view. According to several studies, it is important that the correct preoperative diagnosis is made as omental torsion can be treated conservatively in most cases without any complications avoiding surgical intervention. However, patients should be under clinical and laboratory observation in order to detect symptoms that would lead to surgical intervention in which case a laparoscopy is the appropriate surgical treatment.Torsion of the great omentum is a rare cause of acute abdominal pain which is usually misdiagnosed. In this study we report two cases, a 52-year-old Greek woman and a 68-year-old Greek man, who presented at our emergency room with symptoms such as right lower quadrant pain and tenderness similar to acute appendicitis. In both cases a surgical exploratory laparotomy of the abdomen revealed a twisted heavily congested segment of the right part of the greater omentum accompanied by intra-abdominal serosanguinous fluid.Greater omental torsion is difficult to diagnose preoperatively. It presents as acute abdominal pain located more often in the right iliac fossa. It is very important to make a correct preoperative diagnosis because omental torsion is a benign self-limiting disorder that can be treated conservatively, avoiding laparotomy. When a patient’s clinical, laboratory and radiological findings worsen or diagnosis is doubtful then laparoscopy is the appropriate method for diagnosis and treatment. Omental torsion is a rare cause of acute abdominal pain ; diagnosOmental torsion was first described by Eitel in 1899. Bason and Jones analyzed 223 cases of primary torsion and revealed that only one patient had been correctly diagnosed preoperatively . Since tThe primary form of omentum torsion is usually caused by rotation of the movable portion of the omentum and is influenced by several factors . Causes Torsion of the great omentum is difficult to diagnose preoperatively with pinpoint accuracy limited to rates of 0.6 % to 4.8 % of all cases. Clinical manifestations include sudden pain especially in the side where the torsion is located and the stimulation of the abdominal wall seems to cause peritoneal irritation , 8. OtheThe major complications of omental torsion include rupture and intraperitoneal bleeding, filtration purulent peritonitis and intraperitoneal abscess while fibrosis and inflammatory reaction may occur at occlusion. The diagnosis is difficult and requires confirmation process to display. Abdominal ultrasound as a diagnostic method may exclude from the differential diagnosis conditions such as cholecystitis . The diaIn this case report we analyze two cases of greater omental torsion in a 52-year-old Greek woman and a 68-year-old Greek man who presented at our emergency room (ER) with clinical symptoms and laboratory findings of acute appendicitis. They underwent laparotomy where the diagnosis of greater omental torsion was made. The appendix in both cases was normal and they had no other disorders. Due to the rarity and the difficulty of diagnosis we present the current aspects of diagnosis and treatment of greater omental torsion.Case 1 was a 52-year-old Greek woman who presented at our ER referring abdominal pain at the right iliac fossa. There was no history of any previous abdominal operations or signs of recent infections.A physical examination revealed slightly rigid pain at the lower part of her abdomen and especially at the right iliac fossa with positive McBurney point, rebound tenderness and Rovsing sign. Her abdominal sounds were diminished. A clinical examination revealed normal cervix and appendages without any palpable space; her blood pressure, pulses and body temperature were normal.Her laboratory findings were white blood cells 8.81μL , neutrophils 47.7 % (45 to 75 %), lymphocytes 35.7 % , red blood cells (RBC) 4.20M/mL , hemoglobin (Hb) 11.6g/dL (12.3 to 15.7g/dL), hematocrit (Hct) 36.3 % , platelets (PLT) 288K/uL , blood glucose 100mg/dL , blood urea nitrogen 49mg/dL , creatinine 0.7mg/dL , total bilirubin 0.4mg/dL , direct bilirubin 0.1mg/dL , aspartate aminotransferase 18U/L , alanine aminotransferase 10U/L , lactate dehydrogenase 126U/L , creatine kinase 55U/L (55 to 160), sodium 140mmol/L , potassium 4.3mmol/L , and amylase 73U/L . Urine tests revealed PH acidic, Hb traces; WBC 1 to 3, RBC 1 to 3 and urine amylase 216U/L. The chest and abdominal X-rays were normal.Due to intense clinical signs and worsening of the symptoms the patient underwent an operation with the probable diagnosis of acute appendicitis. In this case a laparotomy with a McBurney incision was performed. A small amount of intra-abdominal serosanguinous fluid was present and revealed a dark red congested area, with vessel thrombosis and necrotic omental mass. Her appendix was normal. The affected portion of her omentum was on the right distal free edge, twisted many times in a clockwise direction free of any adhesions Fig. .Fig. 1ThThe operation proceeded with resection of the affected part of her omentum. Exploration of any other possible abdominal diseases was also performed even if clinical signs and symptoms were negative. An appendectomy was performed. She recovered well with immediate resolution of the symptoms and without any postoperative complications.Case 2 was a 68-year-old Greek man who presented at our ER with abdominal pain and high sensitivity at the right iliac fossa. There was no history of any previous abdominal operations.A physical examination revealed rigid abdomen with positive McBurney point, rebound tenderness and Rovsing sign. His bowel sounds were diminished to absent. His blood pressure was 140/85mmHg, pulse 80 beats/minute and body temperature 37.5 °C. His laboratory findings were WBC 14.000 , neutrophils 79.4 % (45 to 75 %), lymphocytes 16.6 % , RBC 5.20M/mL , Hb 12.3g/dL (13.3 to 16.4g/dL), Hct 36.3 % (40 to 52 %), and PLT 320K/uL (150 to 400). The results of the rest of the biochemical tests, and chest and abdominal X-rays were normal.Diagnosis of acute appendicitis was so profound that abdominal ultrasound and CT scan were not performed. The patient underwent laparotomy with a McBurney incision. There was a large amount of bloody stained serous fluid present in his abdomen. A large segment of his greater omentum (18 × 10cm) was found twisted around its base and was loosely attached to his underlying right colon. The excised segment had a brisk red to gray color and it was heavily congested with multiple hemorrhagic infiltrations; these findings were also stated in a histological examination.The surgical operation proceeded with omentectomy of the affected segment. The exploration of the rest of his abdominal cavity showed no other abnormalities and revealed a normal appendix. He recovered well, with a postoperative period free of complications and symptoms.Torsion of the greater omentum is a rare clinical condition which is caused by twisting of the omentum around a pivotal point, more often in a clockwise direction; it is associated with ischemia and acute abdominal pain. Omental infarction is a benign self-limiting disorder , 10, 11 Omental torsion may be primary, in which there is no obvious cause, or secondary, where it can be caused by several factors . SecondaPrimary forms occur more often in the third and fifth decade of life and are more common in men than women . OmentalOmental torsion has no certain etiology and sometimes it is idiopathic. Possible causes of primary torsion include long omentum, long pedicle of omentum, and relative increase in the bulk of the distal portion of the omentum by fatty deposition or bifid omentum . MechaniOmental torsion is difficult to diagnose preoperatively . It presLess often, pain is located on the right hypochondrium revealed as acute cholecystitis , 19. WheOn examination guarding rebound tenderness is frequently presented in the right iliac fossa but away from the McBurney’s point , 21. A mLeukocytosis with increased levels of C-reactive protein of the plasma and elevated erythrocyte sedimentation rate are nearly always present , 24, whiet al. reported on the cases of 40 patients with epiploic appendicitis and omental torsion. All patients recovered well under conservative treatment without any complications [According to the literature it is very important to make a correct preoperative diagnosis because omental torsion is a benign self-limiting disorder and it is proposed that it can be treated conservatively, avoiding laparotomy . Van Breications . Patientications . When thications , 29.CT scan analysis was not performed in our cases because the clinical signs were very typical for acute appendicitis, especially in Patient 2 where the clinical signs were accompanied by low grade fever and mild leukocytosis. An ultrasound scan was performed for Patient 1 and free abdominal fluid was recognized. Even if omental infarction is not usually diagnosed preoperatively almost all the published cases had been diagnosed after laparotomy.Surgical exploration reveals serosanguinous fluid, a normal appendix and the hemorrhagic necrotic portion of the greater omentum. The affected omental portion should be excised; the clinical signs and symptoms subside immediately after omentectomy.Greater omental torsion is difficult to diagnose preoperatively. It presents as acute abdominal pain located more often in the right iliac fossa. Omental torsion is a benign self-limiting disorder and in most cases can be treated conservatively avoiding laparotomy. When the patient’s clinical, laboratory and radiological findings worsen or when diagnosis is doubtful surgical intervention such as laparoscopy is the proper method for diagnosis and treatment.Written informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of the written consents is available for review by the Editor-in-Chief of this journal.