Two cases of giant intra-articular osteochondromas (knee and ankle joint) are reported; pathologically they are rare representations of synovial chondromatosis. A 17-year-old man presented with a tumorous mass which had been localized in his left ankle for many years, increasing in volume during the last months. The lesion was removed by posteromedial ankle arthrotomy. The second case was observed in a 39-year-old woman with a slow-growing mass in her right knee joint. The lesion was removed from the Hoffa fat pad by open anteromedial arthrotomy. Synovial osteochondromatosis (SOC) is a benign lesion of nodular cartilaginous neoplastic development of the synovium that can lead to loose bodies within the articular space . This coThe term giant SOC was first used by Edeiken et al.  in 1994 The patients described in this report presented with large intra-articular osteochondromatous lesions in unusual locations.A 17-year-old male patient was referred to us because of a growing mass adjacent to the left posteromedial ankle . The lesPlain radiographs in anteroposterior and mediolateral projection of the ankle showed a polycyclic slightly condensed bone structure located dorsal to the tibiotalar joint . A CT scThe patient underwent complete excision through a posteromedial arthrotomy of the tibiotalar joint Figures . To obtaThe macroscopic aspects of the tumors were polylobular, plain delimited masses of bone and cartilage; the larger fragment was made of cancellous bone and cartilage of variable thickness (from 0.5 to 3 mm); the smaller one was completely composed of cartilage .Histological findings showed that the larger fragment was made of normal cancellous bone without increased osteoblastic or osteoclastic activity; there were normal adipocytes in the bone marrow; thin cartilaginous coverage was of normal appearance . The smaAt the last follow-up appointment, two years after the operation, the patient reported no pain and no limitations during daily and recreational activities on any type of surface. Gait was normal with plantar flexion and dorsal extension in sagittal motion of 30° and 15°, respectively. The hindfoot alignment was normal. Function of collateral ligaments and neurovascular status were intact. Plain radiography did not show any relapse of the lesion .The patient, a 39-year-old woman in good health, presented with a prominent mass beside the patellar ligament in right knee joint. It was slow-growing and restricted flexion of right knee joint. Plain radiographs showed a partially calcified intra-articular lesion in the Hoffa fat pad .Exact location and structural analysis were confirmed with MR imaging. The masses were mostly of low signal intensity on T1-weighted MR images, but with some sites of high signal intensity corresponding to areas of calcification. In the T2-weighted MR images, the masses were heterogeneously of high signal intensity . The imaThe mass was enclosed within Hoffa's fat pad in close contact to the synovialis.Macroscopic aspects of the tumors were multiple nodules of which the largest one measured 5 × 3.8 × 2.5 cm, superficially lined with cartilage, with yellow bone tissue at the cut surface.Histological findings were as follows: the largest lesion was described as cartilage with an uneven distribution of chondrocytes and with multiple foci of enchondral ossification. Peripherally, it was possible to sharply define a margin with the connective tissue of the synovial membrane. In the sample of joint capsule, no metaplastic cartilaginous foci were found.At the clinical follow-up, 20 years after the operation, the patient reported no pain and no limitations during daily and recreational activities. Gait was normal with flexion and extension in sagittal motion of the knee of 120° and 0°, respectively.Giant SOC is rarely reported in the literature and its characteristics are largely unknown. Currently, our knowledge of giant form is chiefly based on that of the usual form of SOC.The SOC diagnosis is based on concurrent clinical, radiological, and histological findings and exclusion of other conditions.Clinical presentation of SOC is often subtle, with slow progression. Originating from articular synovial tissue, local tenderness on palpation, reduced range of joint motion, and palpable masses are frequently found .The typical characteristics of “ring-and-arc” chondroid mineralization and bony erosions in the MR images are suggestive of synovial chondromatosis . ComputeThe primary pathological abnormalities are subsynovial cartilage neoplasia, synovial hyperplasia, and the production of round cartilaginous nodules, known as chondromas. These nodules may continue to grow, nourished by synovial fluid; most chondromas calcify and are then termed osteochondromas. These isolated osseous bodies will only continue to grow if they reattach to the synovium .Milgram  suggesteDifferential diagnoses include chronic articular infection, osteoarthritis, pigmented villonodular synovitis, monoarticular inflammatory arthritis, and periarticular neoplasms such as synovial sarcoma . SOC mayWith a reported relative risk of malignant transformation of 5%, synovial chondrosarcoma is a decisive differential diagnosis of high prognostic importance for the patient . ShearerSynovial chondrosarcoma typically presents irregular contours, clumping calcifications, and bony destructions. Permeative and destructive margins rather than an erosive margin with adjacent marrow invasion suggest a malignancy , 13.The treatment of choice for SOC is surgical excision with an open or arthroscopical approach . AccordiThe usual form of SOC in the ankle joint is a rarity. It was found in less than 5% of the cases . To our While the literature describes the knee as the most common location of the usual form of SOC, the confined location in Hoffa fat pad is a rarity. Referring to the giant form, the study by Osti et al.  is to ouBecause of the characteristic intra-articular localization of such lesions, the only chief complaint may be a reduced range of joint motion. In these terms, the two presented cases seem to be instructive. Surgery in the two presented cases of giant SOC showed lasting results, in the first case for a medium-term follow-up period (2 years) and in the second case for a long-term follow-up period (20 years). Given the pathological findings in presented cases, it remains unclear whether giant synovial chondromas are a distinct entity or a rare variant of the typical synovial chondromatosis with multiple small nodules.Further reports and analyses of the giant form of SOC are necessary to improve our understanding of this pathological entity and its differences from the usual form to optimize clinical management.


Gastrointestinal stromal tumor (GIST) is defined as a c-kit-positive gastrointestinal, mesenteric, or omental mesenchymal tumor that very rarely metastasizes to the brain. Metastasis to the cerebellum is particularly rare. An 80-year-old man presented with nausea and vomiting with disturbance of consciousness. Magnetic resonance imaging (MRI) revealed tumor in the cerebellar vermis causing obstructive hydrocephalus. The patient subsequently underwent midline suboccipital craniotomy, and the tumor was totally removed. Immunohistochemical analysis showed tumor cells positive for c-kit and CD34, and cerebellar metastasis of GIST was diagnosed. Postoperative radiotherapy was administered. Following surgery and radiotherapy, the patient developed ileus caused by tumor in the small intestine and underwent laparotomy for tumor removal. Following abdominal surgery, left hemiparesis and consciousness disturbance were noted. Computed tomography showed recurrent large tumor with perifocal edema in the right frontal lobe of the brain. The patient died 3 months after initial craniotomy. Intracranial metastasis of GIST is extremely rare. In cases such as the present, where the condition of the patient rapidly deteriorates and features such as rising intracranial pressure and ileus prevent the use of oral agents, molecular-targeted agents administered by intravenous infusion should be utilized. Most intracranial metastases from GIST have been located in cortical and subcortical areas with relatively easy surgical access and have usually been treated surgically followed by adjuvant therapy such as radiotherapy or chemotherapy.The development of molecular-targeted anticancer agents is giving new hope to these patients. Imatinib mesylate is the first effective systemic therapy for locally unresectable or metastatic GIST . Since bHowever, imatinib mesylate is believed to be ineffective against brain metastases, because it cannot pass through the blood-brain barrier (BBB) and does not achieve adequate levels in the brain when administered in lower concentrations . As a seThe effectiveness of radiotherapy for GISTs has not been confirmed. GISTs are considered “radioresistant” tumors due to their histological relationship to soft tissue sarcomas, which show relatively poor responsiveness to radiotherapy. Some case reports have indicated that radiotherapy can reduce the tumor burden and produce durable local control for locally advanced and metastatic tumors –11. RadiHere, we report the case of a patient with cerebellar metastatic GIST that was treated by surgical resection and radiation. In addition, we summarize and review the relevant literature regarding intracranial metastasis of GIST.The patient was an 80-year-old man who presented with nausea, anorexia, and gait disturbance. Neurological examination revealed dysmetria and dysdiadochokinesia on the left extremities. Magnetic resonance imaging (MRI) showed a round, 4-cm tumor appearing isointense on T1- and T2-weighted imaging with homogeneous enhancement Figures  causing α-smooth muscle actin  and peritoneum (41%), while concurrent GIST and metastases to the central nervous system are extremely rare.As shown in The majority of metastatic intracranial GISTs are present in older adults with a median age at presentation of approximately 60 years . Patients have sometimes been known to show metastatic disease elsewhere prior to the development of CNS metastases. The interval between treatment of the primary lesion and diagnosis of CNS metastasis is considerably long , and the prognosis is dismal once intracranial metastasis is diagnosed. Mean overall survival has been documented as 8.6 months . In the present case, cerebellar tumor was diagnosed based on immunohistochemical findings prior to diagnosis of the primary lesion. The primary lesion turned out to be located in the small intestine and was identified when the patient suffered from ileus following craniotomy. In the three reported cases in which intracranial metastases were discovered first (including the present case), the prognosis was worse than that in those with intracranial metastases diagnosed after treatment of the primary lesion.The current clinical practice guidelines recommend surgical resection for limited disease, particularly cortical or subcortical lesions, and adjuvant therapy as an option for patients with substantial risk of relapse .Since tumor size (>5 cm) is one of the prognostic factors for aggressive behavior of GIST , the aimRecently, however, imatinib mesylate has been introduced as a molecular-targeted chemotherapeutic agent. Imatinib mesylate is an orally administered selective inhibitor of certain tyrosine kinases, including c-kit. Activating mutation of KIT or PDGFRA is found in the vast majority of GISTs, and the mutational status of these oncoproteins can be predictive of the clinical response to imatinib therapy , 25, 26.Following resistance to imatinib mesylate, sunitinib, a multitargeted small-molecule tyrosine kinase inhibitor that selectively blocks vascular endothelial growth factor receptors (VEGFRs) with potent activity against KIT and PDGFA, has proven effective as a second-line therapy and is currently approved worldwide for metastatic GIST in patients with imatinib resistance or intolerance , 8. UnliAlthough radiotherapy is not a standard therapy for GIST, Cuaron et al. described the importance of this option for the management of locally advanced and metastatic GISTs . In a seDespite the limitation of therapeutic strategies available, our findings remain hypothesis generating and offer important insights into a treatment modality that appears safe and effective for the management of patients with metastatic or locally advanced GIST.


Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14–0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal. Primary splenic angiosarcoma (PSA) is among the most unusual types of malignancies . It is t9/L, with 55% neutrophils, 42% lymphocytes, 2% monocytes, and 1% eosinophils and elevated platelet count, 551 × 109/L. After admission, a thoracentesis was performed, for shortness of breath relief, and pleural fluid had findings consistent with a reactive effusion. A computed tomography (CT) scan was done, revealing splenic enlargement and two solid mass lesions measuring 10 cm and 12.5 cm in greatest diameter with ill-defined margins within the spleen. These two mass lesions were isodense to the splenic parenchyma, while they did not present contrast blush on the arterial phase and no other specific characteristic was revealed. The fundus and body of the stomach seemed compressed and dislocated anteriorly due to splenomegaly. A CT scan of the brain and thorax followed, but no other enlarged lymph nodes were found, nor indications of metastatic disease. Despite elevated white cell count and platelet count, splenomegaly was due to distinct masses identified on CT scan and no other signs indicated hematological disease.An 82-year-old woman presented to our hospital with shortness of breath and left chest pain. On primary investigation with chest X-ray a moderate left-sided pleural effusion was revealed. On history the patient complained of weight loss, fatigue, and mild afternoon fever for the last 3 months. From her past medical history, hypertension and breast cancer treated with surgery and adjuvant chemotherapy and radiation therapy 16 years ago, were significant. More specifically the patient had received six cycles of cyclophosphamide, methotrexate, and 5-fluorouracil. Physical examination revealed a palpable mass in her left upper abdominal quadrant. Laboratory findings at initial presentation revealed marked leukocytosis 44.41 × 10Surgical approach in an open manner was decided for accurate diagnosis and treatment. The patient was placed on a supine position and a midline incision was preferred. Open splenectomy was performed with care not to violate the splenic capsule and not to disrupt the pancreatic tail. During the procedure, the patient received 2 units of concentrated red blood cells and 2 units of fresh frozen plasma. Her postoperative hospital course was uneventful.The spleen was 1.200 g in weight and 19 × 15 × 11 cm in size, with nodular appearance and areas of necrosis . The finPSA is an aggressive malignancy originating from splenic vascular endothelium. Mesenchymal-derived elongated endothelial cells which line the spleen's spongy network of sinusoids are the primitive cells of this neoplasm. It is thought to be extremely rare, with an incidence of 0.14–0.23 cases per million, slightly predominating in men. It may arise in any age group, as cases reported are from 14 months to 89 years of age [Pathogenesis is not accurately defined. As for every kind of angiosarcoma, thorium dioxide, vinyl chloride, and arsenic have been accused as causative ingredients; however no clear relationship between these substances and splenic angiosarcoma has been established. A few PSA patients had a history of receiving chemotherapy for lymphoma or radiation therapy for other malignancies, as our patient had prior chemo- and radiotherapy for breast cancer. In particular, alkylating agents as cyclophosphamide, which our patient received 16 years ago, are thought to be predisposing factors for other malignancies. On the other hand previous reports of PSA reported no association with chemotherapeutic agents –6. Our cClinical presentation is nonspecific and may range from asymptomatic disease revealed by investigations for unrelated reasons to splenic rupture and lethal hemorrhage –9. AbdomImaging modalities are helpful for establishing a diagnosis of splenomegaly, although lacking diagnostic accuracy for the specific disease. Ultrasound scan, computed tomography (CT), and magnetic resonance imaging (MRI) scans all display well evidence of splenomegaly. The most common findings on ultrasound are solitary or multiple, solid, and cystic mass lesions with heterogeneous echotexture. Sometimes areas of necrosis and hemorrhage are noted as cystic areas within the mass . CT scanIn general, as with many splenic masses preoperative percutaneous biopsy is contraindicated in PSA, because of high risk of rupture and disease dissemination. Histopathologic studies can therefore only be made after operative approach and splenectomy. Specimens often weigh more than 1 kilogram. On gross examination, the spleen may be replaced entirely with malignant diffuse infiltrate. Areas of hemorrhage and necrosis are often detectable macroscopically. On the other hand, microscopically, PSA consists of disorganized anastomosing vascular channels lined by large, atypical endothelial cells with significant irregular hyperchromatic nuclei. Poorly differentiated areas have sarcomatous features, whereas well-differentiated regions appear very similar to sinuses of the spleen. Immunohistochemically, pathologists usually search for at least two vascular proliferation markers  plus at least one histiocytic differentiation marker (lysozyme and/or CD68).The only established prognostic factors are considered to be the mitotic counts and tumor size , for casPSA is usually treated surgically. Splenectomy is the mainstay of treatment, because the lesion is highly refractory to adjuvant treatments with radiation and chemotherapy. PSA is an aggressive malignancy and metastases tend to occur early in disease course . The repMontemayor and Caggiano indicated that splenectomy prior to rupture and dissemination of the disease is of paramount importance and median survival is 14.4 months versus 4.4 months after that incident . Open apIn conclusion, primary angiosarcoma of the spleen is an aggressive disease that needs to be included in the differential diagnosis of splenomegaly, in cases where a distinct tumor of the spleen is revealed on imaging. Surgery is the only potentially long-term therapeutic option.


In recent years with the increase in cesarean section rates, the frequency of placenta accreta cases rises. It causes 33–50% of all emergency peripartum hysterectomies. We present a 42-year-old case who was caught with early postpartum hemorrhage due to retained placental products. The ultrasonography showed a 65 × 84 mm mass in the uterine cavity after the delivery. Due to presence of early postpartum hemorrhage which needs transfusion, an intervention decision was made. The patient underwent curettage but the mass could not be removed so that placental retention was ruled out. Submucous leiomyoma was made as first-prediagnosis. Hysterectomy operation was performed as a curative treatment. Placenta increta diagnosis was made as a final diagnosis with pathological examination. As a result, placental attachment disorders may be overlooked if it is not a placenta previa case. Placenta accreta is defined when the whole placenta or a part of it invades myometrium in an abnormal way. As the pathogenesis, defective decidualization during implantation is responsible. When the chorionic villi invade only the myometrium, the term placenta increta is used, whereas placenta percreta describes the invasion through the myometrium and serosa and rarely adjacent organs. In clinical practice, the general term “placenta accreta” is used to describe all 3 grades of abnormal placental attachment. The frequency of this life-threatening obstetric complication is responsible for out of 533 to 2510 childbirths [42-year-old female patient's, Gravida 7, Parity 5, Abortion 1, previous births were spontaneous vaginal delivery and obstetric history was unremarkable. At the 40th week of pregnancy she underwent limited ultrasonographic evaluation: fundus located placenta and estimated fetus with a weight of 4000 g were identified; the additional examination was unremarkable. The patient is presenting with complaints of cramps, and as the result of the evaluation, she was admitted to the delivery room to be followed-up. Hematocrit was 31% during the hospitalization. The patient was living in the eastern region of Turkey, a rural region, that is why the antenatal follow-ups were inadequate. First examination of the patient was performed during the first trimester, as the detection of the pregnancy, and the second/last examination was held when the patient was admitted to the delivery room. In addition, we have not reached any record or report of ultrasonographic evaluation of pregnancy.At 12th hour of admission, the patient gave birth to a 3900 g weight and 52 cm tall girl with normal spontaneous vaginal delivery. The placental delivery was uneventful and placental evaluation after delivery revealed normal appearance. There was no suspicion of lacking in cotyledons on maternal surface of the placenta. There was no sign for placental abruption. The patient was put in the service for follow-ups to be carried out without bleeding. Postpartum 4th hour of hemorrhage was treated with using uterotonic agents (oxytocin and methyl ergonovine) and the bleeding was stopped. The ultrasonography showed a 65 × 84 mm mass with irregular borders in the uterine cavity. Primarily submucosal fibroids or stalked leiomyoma came to mind, and placental retention was considered as the second preliminary diagnosis due to succenturiate placenta, although removed placenta has normal appearance. During the follow-ups intermittent heavy vaginal bleeding occurred and the family was informed for curettage to be taken on. However, we were unsuccessful during the curettage; the mass was rigid and was fixed in contrast with placenta so that placental retention was ruled out.On the 2nd day of postpartum intermittent heavy vaginal bleeding continued. Despite of 4 units of packed red blood cells transfusion the hematocrit level was found to be 26-27%. Because of being unable to perform further radiological investigations and ongoing vaginal bleeding, surgery was offered to patient as a curative treatment. Families were informed and their consent was taken for surgery. The family, who does not request for fertility preservation, asked for hysterectomy.In the operation a 20 week-size uterus was observed. When the uterine cavity is opened postoperatively, approximately 8 cm of mass filling the cavity was witnessed . On the In the past 50 years with increasing cesarean rates, placental invasion abnormalities were observed 10 times more than usual . MoreoveClinically, patients with risk factors  are usually suspected for diagnosis antenatally. High maternal age, multiparity, myometrial damage , and uterine artery embolization constitute as the other risk factors for placenta accreta. Patients without antenatal diagnosis may apply to emergency room with heavy vaginal bleeding or the diagnosis may be done by massive bleeding during placental separation after caesarean section or vaginal delivery. Bleedings may be life threatening.The diagnosis usually starts with suspicion on antenatal follow-ups. In a patient with placenta previa the diagnosis is suspected with sonographic evaluation. Sonolucent areas in the placenta, loss of hypoechoic areas between placenta and myometrium, hypervascularization on the surface between serosa and bladder on Doppler ultrasound, and vascular stasis with turbulent flow are suggestive findings for placenta adhesion abnormalities sonographically. In the literature of the ultrasound are given sensitivity of 89.5% and specificity of 91% for the diagnosis of placenta accreta . MagnetiIn the case presented here, cesarean delivery and placenta previa as the most important risk factors are not available. Although grand multiparity and high maternal age are counted as risk factors, it is not suspected of diagnosis of accreta antenatally. At this point, inadequate and improper antenatal surveillance may be the cause of missing the diagnosis. In the literature it is reported that, through the cases with five or more pregnancies, risk of accreta increases 3,9 times .Uterine atony is the most common cause of early postpartum hemorrhage. Other common early postpartum hemorrhage reasons are upper and lower genital tract lacerations, lower urinary tract lacerations, and retaining placenta. Retaining placental products due to the placental adhesive disorders are rarely detected in cases with early postpartum hemorrhage. As it is presented, we can face patients with placental attachment disorder who are not diagnosed with placenta previa. In such rare cases, it is difficult to doubt and diagnose antenatally. The management of the cases encountered during operation or postpartum period is also rough and this situation increases the maternal morbidity and mortality. As in the case presented here, sometimes the discrimination cannot be made clearly in ultrasonographic examination.In patients with postpartum hemorrhage the diagnosis of placental retention and placenta accreta should be kept in mind if the mass cannot be removed with curettage. In conclusion, the antenatal diagnosis seems to be the key point for management of placenta accreta.


Ganglioneuromas (GNs) are hamartomatous tumors derived from the autonomic nervous system. It is rare to encounter GN in the gastrointestinal tract. Patients with these tumors usually present with abdominal pain, constipation, ileus, weight loss, or even bleeding. GNs are categorized into three different morphological subtypes, namely, polypoid GN, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. We present a case of hematochezia from GN in a colon polyp discovered on diagnostic colonoscopy. Due to a lack of guidelines, we reviewed the literature to discuss treatment and other associated conditions for GN. GNs are uncommon, especially in the gastrointestinal tract. Patients with GN can present with constipation, obstruction, abdominal pain, weight loss, and bleeding depending on the lesions size and location. We present a case of hematochezia from GN in a colon polyp discovered on diagnostic colonoscopy.A 43-year-old male with medical history of asthma presents with self-limited episode of hematochezia for 2 days. The patient denies any melena, hematemesis, abdominal pain, constipation, diarrhea, weight loss, or NSAID use. The vital signs and physical examination were within normal range. Routine labs including complete blood count, comprehensive metabolic panel, and coagulation profile were normal. A diagnostic colonoscopy was then performed which revealed a 0.6 cm sessile polyp in the cecum see . A hot fThe incidence of lower gastrointestinal bleeding in the United States is estimated to be approximately 36/100,000 cases [Ganglioneuromas (GNs) are hamartomatous tumors derived from the autonomic nervous system. These tumors rarely present in the gastrointestinal tract and most frequently occur in head, neck, or adrenal glands. They typically originate from the undifferentiated neural crest, including ganglion cells, nerve fibers, and supporting cells of the enteric nervous system  . They arPatients with solitary GN are often asymptomatic but can present with constipation, abdominal pain, weight loss, obstruction, ileus, and bleeding depending on the lesions size and location. Treatment of solitary GN involves endoscopic resection via hot biopsy forceps. Histology of the lesion will show multiple spindle cells on hematoxylin and eosin stain and will be immunoreactive to S100 protein stain and neuron-specific enolase (see Figures In conclusion, GN is a rare cause of hematochezia in adults and confirmatory diagnosis can only be revealed through histologic examination. Further tests for systemic and familial diseases should be considered in patients with GN.


Aim. To report the case of congenital bilateral upper eyelid eversion with severe chemosis that was successfully managed conservatively. Report. The patient was a six-hour-old male neonate with bilateral congenital upper eyelid eversion and severe chemosis, following uneventful delivery. Conservative management consisted of the application of antibiotic ointment and padding the exposed conjunctiva with 5% hypertonic saline-soaked gauze. The eyelids reverted spontaneously on day 3 and the condition was completely resolved by the third week. Conclusion. Congenital upper lid eversion is usually a benign condition which responds well to conservative treatment. Creating awareness amongst healthcare professionals is essential. Congenital eyelid eversion is a rare condition in which the eyelid is completely turned out, with prolapsed conjunctiva and chemosis. Usually, cases present at birth, although late presentations have been reported , 2. It iThe exact cause is unknown. Most cases reported have no associated ocular or general abnormalities. The condition is, however, more frequently seen in black infants, in Down syndrome, and in collodion skin disease , 3.Most cases are treated medically. Surgical management is reserved for cases with late presentations or with complications or cases not responding to medical management.Two cases have so far been reported in Cameroon. Management in one case consisted of manual repositioning of the tarsal conjunctivae and bandaging of the eyelids following antibiotic ointment application . In the We report a case of bilateral upper eyelid eversion with severe chemosis that was successfully managed conservatively leading to the spontaneous inversion of the eyelids.A six-hour-old male newborn was referred to our eye clinic from a private hospital in which he was born for the management of the eversion of both upper eyelids. He was the sixth child to a 29-year-old woman. A history of malaria in the third trimester was reported. This was managed accordingly. Pregnancy was regularly followed up. Delivery was at term. Labour and delivery were both uneventful.Examination revealed bilaterally everted upper eyelids with severe chemosis . ExaminaThe baby was admitted and managed as follows: application of neomycin and polymyxin eye ointment and padding of the prolapsed conjunctiva with gauze dressings soaked in 5% hypertonic saline once daily. Daily eye cleansing was performed to keep the external eye clean of any discharge.On the second day, there was reduction in the size of the chemosis . TreatmePaediatrician examination revealed no morphological abnormality. C reactive protein was positive at 12 mg/L. Full blood count showed white cell count in the normal range but the differential count revealed granulocytosis. Urine and cerebrospinal fluid cultures were negative. The baby was admitted and treated for neonatal sepsis with intravenous Ampicillin, Netilmicin, and Cefotaxime for 8 days. While being admitted, the eyes were no longer padded and 5% hypertonic saline eye drops were administered four times daily; antibiotic ointment was used twice daily. Fever subsided on the 5th day.At 3-week followup, the child could open the eyes spontaneously . Ocular Complete eversion of the upper eyelids with chemosis is a rare condition. Sellar et al.  in 1992 Congenital eyelid eversion can be treated conservatively. The goal of management is to prevent desiccation of the exposed conjunctiva and allow spontaneous inversion of the lid. Conservative management includes applying moist dressings, eyelid taping, and pressure patching along with topical antibiotic and lubricants. Additionally, topical 5% hypertonic saline has been employed as a conservative treatment by some authors , 8. The Surgical treatment options reported in the literature include scarification of the exposed conjunctivae , temporaOur case was managed using topical antibiotic ointment, to avoid infection and drying of the exposed conjunctiva, padding with 5% hypertonic saline soaked gauze to reduce chemosis by osmosis. No manual manipulation of the lids was done. Improvement was observed within 3 days and complete resolution occurred in 3 weeks with no sequelae. Our case had neonatal sepsis. In a report on three cases, Adeoti et al.  reportedLid manipulations can lead to autonomic effects such as respiratory arrest in neonates. This has been reported by Watts and Dapling . This fuIf not treated early, it could lead to complications like secondary infections and epidermization of the conjunctiva. The Chemotic conjunctiva protects the cornea from exposure and hence, corneal complications are rare. However, a case of corneal perforation has been reported in a baby with Down syndrome .The presentation of congenital eyelid eversion is usually alarming to both the parents and the healthcare professional, especially if he/she is seeing it for the first time. Its benign course, however, as shown in this case, justifies a conservative approach in anticipation of an excellent result. It is important to create awareness among healthcare professionals in obstetric and neonatal care of the existence of this congenital anomaly, as the condition responds well to early treatment.


Appendicovesical fistula (AVF) is an uncommon type of enterovesical fistula and a very rare complication of acute appendicitis. Herein, we report a case of 39-year-old male patient who presented with persistent urinary tract infection, recurrent abdominal pain, and pneumaturia. Imaging techniques including ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI) were performed to identify the abnormality. However, definitive diagnosis of AVF was made by cystoscopy. AVF is a rare condition, which usually occurs following appendicitis , 2. In a3 and CRP: 35). Urine analysis showed fecal content and increased number of bacteria accompanied with elevated WBC count in the urine sample, which suggested a possible enterovesical fistula. USG examination revealed mildly increased bladder wall thickness and accumulating sediments at the base of bladder. Appendix could not be visualized, but intraperitoneal fat planes around caecum showed increased echogenicity which suggested an inflammatory process. Ultrasound also revealed heterogeneous fluid collection which located lateral to the anterior aspect of the urinary bladder. Prostate and distal portions of the ureters were normal. We obtained an oral contrast CT scan to elucidate the underlying reason. CT examination demonstrated collection of fluid extending from the right anterolateral border of the bladder to the caecum. Outer diameter of the appendix exceeded normal limits (measured approximately 9 mm) and appendix was attached to the right anterolateral part of the bladder. Additionally, hyperdense fecalith image was seen in the distal portion of appendix; also there were 2 cm calculi in bladder (A 39-year-old man was admitted to urology department of our hospital, who had been suffering from recurrent periumbilical pain and persistent urinary tract infection. He also complained about foul smell of his urine. He had a history of acute right abdominal pain for a couple of days nearly 10 years ago but he did not receive any medical or surgical treatment. On physical examination, mild abdominal tenderness at lower quadrants was noted. Routine blood counts and biochemical analyses indicated the presence of an inflammatory condition (WBC: 13200/mm bladder . But our bladder . To confAppendectomy, surgical repair of bladder and catheter drainage of the area were the preferred definitive treatment for the patient .AVF is a very rare type of enterovesical fistula. Although it may accompany various conditions like Crohn's disease and malignancies, it is usually seen as a complication of acute appendicitis , 4, 5. IIn conclusion, if a patient presents with persistent urinary infections, recurrent abdominal pain, and pneumaturia, AVF should be kept in mind for diagnosis.Although CT urogram is generally the best diagnostic modality, cystoscopy remains an alternative method for the diagnosis of AVF in cases where the CT findings are equivocal.


Dear Sir,Here we report a case of mature sacrococcygeal teratoma (SCT) in a small girl which was not diagnosed early and was treated as collection of fluid in gluteal region with recurrent needle aspirations since the age of three months.One year and nine month old baby girl brought to the hospital with swelling in the left gluteal region noticed by the parents since three months of age. They consulted a local practitioner who suggested aspiration by needle, which was done multiple times. The size of the swelling used to decrease immediately after aspiration but returned to its usual size within 1-2 months. On examination, the child was alert, active with normal growth and development. Left gluteal region was more prominent with skin over the region showing multiple puncture marks (fig. 1), palpation revealed an ill-defined, immobile, and non-tender mass with variable consistency. Per rectal examination showed mass extending up to the presacral region. Bimanual palpation revealed extension of the mass up to the level of the pelvic brim. Ultrasound revealed a well-defined anechoic cystic lesion near coccyx. MRI of the left gluteal region showed two peripherally enhancing lesions near the anterior-inferior part of the coccyx confirming the diagnosis of SCT (fig. 1). Serum alpha fetoprotein level was normal. At operation, a large dumb-bell shaped mass with both solid and cystic (mainly) components, arising from the coccyx, was completely excised along with coccygectomy. Postoperative recovery was uneventful. Biopsy report showed it to be mature teratoma (fig. 1) with margins free of tumor. The parents were counseled for regular follow-up.SCT is one of the tumors in the neonatal period which carries an excellent prognosis provided timely diagnosis, prompt surgical treatment and complete excision along with coccygectomy are done. The chances of malignancy in SCT relates to factors like the tumor size of >10 cm, Altman Type III and IV tumors, presence of more solid component, and late presentation.[2] Our patient presented late but fortunately the tumor was mainly cystic. Recurrence has been reported in SCT as a benign or malignant tumor especially during the first 3 years of life. Factors like tiny foci of malignant endodermal sinus cells, incomplete resection of tumor, tumor spillage, and omission of coccygectomy increases the possibility of recurrence. In our case despite late presentation, the mass was removed completely along with the coccygectomy, and histopathology confirmed it as mature teratoma. Thus a favorable outcome is noted. Awareness programs are needed for general practitioners about common pediatric surgical diseases and their management.Source of Support: NilConflict of Interest: None declared


The larynx is an extremely rare site of involvement by lymphomatous disease. We present two cases of isolated laryngeal high-grade and another low-grade lymphoma, together with a literature review of laryngeal lymphoma management. Staging with computed tomography only confirmed disease in the laryngeal area .Biopsy of the laryngeal mass showed that the mucosa and submucosa were infiltrated by large atypical lymphoid cells with irregular nuclear outlines and multiple prominent nucleoli. The cells were positive for CD45 and CD20 and negative for CD3 and bcl2 on immunohistochemistry . These fThe patient was administered three cycles of R-CEOP  followed by involved field radiotherapy. Anthracyclines were avoided in view of a decreased left ventricular ejection fraction of 40% on echocardiography performed at diagnosis.The first cycle of chemotherapy was complicated by an acute coronary syndrome, from which the patient recovered with supportive care. The tracheostomy was removed following the second cycle of cytotoxics and the patient remains alive and well 5 years later.9/L, haemoglobin of 148 g/L, platelets of 213 × 109/L, and a normal serum lactate dehydrogenase level of 192 U/L.A 65-year-old Caucasian gentleman, with a history of ischaemic heart disease, presented to ENT with a two-month history of hoarseness. Physical examination was unremarkable, with no lymphadenopathy or organomegaly. Investigations showed a white blood count of 5.7 × 10Indirect laryngoscopy showed vocal polyps, which were excised. Histology showed a monotonous population of lymphoid cells within the lamina propria of mucosal polyps, with slightly irregular nuclear contours and inconspicuous to clear cytoplasm. Immunohistochemistry was positive for CD45, CD20, and bcl2 and negative for CD5, CD23, bcl6, CD138, CD43, CD10, and cyclin D1 , consistStaging with computed tomography and bone marrow aspiration and biopsy showed no evidence of disease involvement outside of the larynx.The patient was managed with involved field radiotherapy to the larynx with clinical resolution of symptoms and no radiological evidence of residual disease. He remains alive and well 5 years later.Extranodal lymphomas confined to the larynx are rare, accounting for <1% of all laryngeal neoplasms, with only about 100 cases having been described in the literature to date . This raLaryngeal lymphoma presents clinically in a similar fashion to squamous cell carcinoma, with symptoms such as hoarseness, dyspnoea, a foreign body sensation in the throat, or stridor. Uncommonly, it may present catastrophically with acute airway obstruction requiring immediate surgical intervention, as in Case 1 presented above. Systemic symptomatology is unusual, since laryngeal lymphomas tend to remain localized for prolonged periods, though more aggressive forms tend to spread earlier . InteresThe commonest anatomical site involved is the supraglottic region (47%), with glottic involvement accounting for 25% of cases. The transglottic and subglottic regions are much less commonly affected .Macroscopically, these tumors present as smooth or polypoid masses as depicted in our two cases, respectively, rather than ulcerated masses . In fact H. pylori and other urease-splitting organisms, which may colonize the larynx, and the development of Primary Marginal Zone Laryngeal Lymphoma. When investigating Primary Marginal Zone Laryngeal Lymphoma one should also exclude autoimmune conditions such as Sjögren's syndrome [There is some emerging evidence in the literature of the association ofsyndrome , 8.It is imperative to stage the lymphomatous process correctly, since treatment varies depending on both the grade and the stage of the disease. PET-CT, in particular, is finding an important place for radiological staging in laryngeal lymphoma, both low-grade and high-grade , 10. In A review of the literature on isolated laryngeal lymphomas published over the last 2 years (from 1994–2014) mainly revealed case reports and reviews. No specific studies have been carried out specifically looking at the management of this rare type of lymphoma. Most reports described cases of MALT lymphomas as in our Case 2 and others related to T/NK lymphomas . TherapyOne should have a high index of suspicion for lymphoma of the larynx, since the management is very different from that of other more commonly occurring tumors at this site.Due to the small number of cases reported, there is no definite consensus regarding best management of laryngeal lymphoma; it would be advisable that for the time being one follows international lymphoma guidelines on limited stage disease for the management of laryngeal lymphoma .The discussions ensuing from case reports regarding lymphoma affecting unusual sites highlight the lack of evidence regarding the actual biology of these tumors and best treatment options for patients. This may spur the formation of regional/international databases for the description of lymphomas affecting specific unusual sites. With this approach these cases may be studied in greater depth and may prompt the development of comparative treatment trials, providing patients with more evidence-based therapeutic modalities.From this review, the most important points to emerge are as follows.


Annular pancreas (AP) concurrent with pancreaticobiliary maljunction (PBMJ), an unusual coexisted congenital anomaly, often presented symptoms and subjected surgical treatment at the early age of life. We reported the first adult case of concurrent AP with PBMJ presented with symptoms until his twenties, and performed a literature review to analyze the clinicopathological features of such cases comparing with its pediatric counterpart.The main clinical features of this case were abdominal pain and increased levels of plasma amylase as well as liver function test. A complete type of annular pancreas with duodenal stenosis was found, and dilated common bile duct with high confluence of pancreaticobiliary ducts was also observed. Meanwhile, extremely high levels of bile amylase were detected both in common bile duct and gallbladder. The patient received duodenojejunostomy (side-to-side anastomosis) as well as choledochojejunostomy (Roux-en-Y anastomosis), adnd was discharged in a good condition.AP concurrent with PBMJ usually presents as duodenal obstruction in infancy, while manifests as pancreatitis in adulthood. Careful long-term follow-up is required for children with AP considering its association with PBMJ which would induce various intractable pathologic conditions in the biliary tract and pancreas. Annular pancreas (AP) is a rare congenital anomaly that is frequently associated with duodenal atresia (DA) or duodenal stenosis (DS) . PancreaThe patient, a 26-year-old man, presented to our department with abdominal pain. He was born at 40 weeks’ gestational age with a birth weight of 3,010 g. Since his 3 years of age, the patient presented with relatively greater appetite compared with corresponding age cohorts and wiggly epigastric mass which would disappear two hours after meal. He remained free from vomiting and acute abdomen until the first episode of abdominal pain at the age of 23 years. Since then, he underwent totally 3 episodes of acute abdomen, and was diagnosed with acute pancreatitis., <190 U/L). Abdominal sonography revealed a dilated common bile duct. Computerized tomography additionally showed the dilation of proximal duodenum with stenosis at the distal end of descending duodenum. Magnetic resonance imaging and cholangiopancreatography revealed fusiform dilatation of the common bile duct with high confluence of pancreaticobiliary ducts with a common channel measured 24 mm in length , alanine aminotransferase , total bilirubin , amylase  and lipase (279 U/L; normal rangeDuring laparotomy, a complete type of annular pancreas and a fusiform-type dilatation of common bile duct were confirmed. Air charging demonstrated an enlarged pylorus and excessively ectatic duodenal bulb with duodenal stenosis at the distal end of descending part Figure . PancreaThe postoperative course was uneventful. The patient was discharged on the 21th postoperative day on full oral feeding and without abdominal pain. The patient has been free from abdominal pain with normal serum amylase levels in the follow-up period of 12 months postoperatively.There are three types of pancreatic fusion anomalies: annular pancreas, pancreas divisum and portal annular pancreas . While pUntil now, there were totally 12 cases of concurrent AP with PBMJ, of which the 11 pediatric cases were described previously, Table PBMJ can be divided into PBMJ with biliary dilatation and PBMJ without biliary dilatation . Out of The diagnosis of PBMJ in patients with AP is often delayed and even missed, not only because of the gradual and variable development of bile duct dilatation, but also because of lacking accurate diagnostic maneuver for PBMJ . Thus, aAP concurrent with PBMJ usually presents as duodenal obstruction in infancy, while manifests as pancreatitis in adulthood. Although prophylactic flow-diversion surgery remains controversial, careful long-term follow-up is required for patients with AP associated DA or DS considering the association of concurrent PBMJ with various intractable pathologic conditions in the biliary tract and pancreas.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.AP: Annular pancreas; PBMJ: Concurrent with pancreaticobiliary maljunction; DA: Duodenal atresia; DS: Duodenal stenosis.The authors declare that they have no competing interests.LC contributed to the design of the study and direction of its implementation. FZT conceived and designed the experiments and supervision of the field activities. LC and TJZ carried out the prepared the Materials of patient and prepared the literature review as well as the Discussion sections of the text. JDR and ZLL conducted the data analysis. All authors read and approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/13/153/prepub


Postoperative neonatal intestinal obstruction has a myriad of etiology. An operated case of imperforate anus developed intestinal obstruction early postoperatively. At re-operation, missed complete congenital pouch colon with a small perforation and displacement of small bowel into the lesser sac were found. The patient was managed by adhesionolysis, excision of pouch colon, and end ileostomy. The patient did well postoperatively.  An 8-day-old male neonate, known case of imperforate anus with a colostomy at the left lower quadrant, was referred to our hospital with abdominal distension, bilious vomiting, and not passing stool for 3 days. History revealed that the colostomy was formed on 2nd day of life at another hospital which started working on the following day. The patient however developed abdominal distension and bilious vomiting on 3rd postoperative day followed by failure to pass stool from the stoma. After failure of conservative measures, the patient was referred to us. Abdominal radiograph showed large gas shadows and air fluid levels. Ultrasound of the abdomen depicted dilated bowel loops with absent peristalsis. Laboratory tests showed Hemoglobin of 10g/dl and platelets count of 65000/mm3. Other laboratory parameters were normal. After resuscitation and optimization, the patient was operated, which showed fecal peritonitis and two cystic structures in the abdominal cavity. On dissection of upper cystic structure, few loops of small bowel were found trapped beneath the stomach (lesser sac) and appeared herniated through a well appreciable opening . Further dissection showed that opening was formed by free margin of greater omentum which was adhered with bowel loops and posterior abdominal wall. There was no actual defect in the greater omentum. In the lesser sac, the small bowel loops were adherent with each other and the posterior wall of stomach; feculent colored exudate was visible over bowel loops . The displaced and adherent bowel loops were viable; adhesionolysis was performed. The second cystic structure was a complete pouch colon with a small perforation . A wall of pouch colon was exteriorized as window colostomy during first surgery in peripheral centre. The pouch colon was mobilized and excised with repair of colo-vesical fistula; end ileostomy was done. The postoperative course remained uneventful. The patient was discharged on 7th postoperative day. The patient is in our follow-up for further procedures.Earlier we have studied etiology of first week postoperative intestinal obstruction in neonates and children and found a host of etiologies including postoperative intussusception, internal herniation through a mesenteric defect, soft adhesions causing intestinal kinking, gossypiboma, and missed incomplete congenital pouch colon (CPC).[1] In the index case, missed complete CPC with a small perforation and displacement of small bowel into the lesser sac were the etiologies of first week postoperative intestinal obstruction.Internal herniation may be paraduodenal, transmesenteric or transmesocolic, paracolic, and transomental etc. Herniation to lesser sac may occur through natural opening i.e. foramen of Winslow, transmesocolic, transgastrocolic ligament, through greater omentum, and after Roux-en-Y gastric bypass.[2-5] In our case, translocation of small bowel into the lesser sac was not through the above cited routes; in fact, free margin of greater omentum was adherent to the gut loops and posterior abdominal wall thus simulated an opening. While probing the etiology of entrapment of small bowel loops into the lesser sac in the index case, following points should be considered; the absent or malformed gastrocolic ligament owing to complete CPC would have allowed free movement of small bowel behind stomach; and perforation peritonitis might have resulted in adhesions between greater omentum small bowel loops, and stomach thus entrapping the small bowel there. The presentation of internal herniation may range from pain abdomen to as sinister as intestinal gangrene and perforation peritonitis.[2-5] The index case presented with signs of intestinal obstruction within the first week of operation. Moreover, the entrapment did not lead to perforation; the missed complete CPC might had not emptied properly through window stoma and resulted in its small perforation or a delay in forming stoma in the index case (2nd day of life) might be the reason. Nevertheless, X-ray did not show pneumoperitoneum. Retention of CPC during initial surgery is associated with a number of complications which often proved fatal.[6] In our case, the operative notes of first surgery were silent as to CPC which may indicate that it was missed and not retained intentionally. The ideal staged treatment of CPC is excision of the pouch with end ileostomy, [6] which we later did during exploration at our department.Source of Support: NilConflict of Interest: None


Congenital sternal defects are rare deformities frequently associated with other anomalies of the chest wall and other organ systems. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity, in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy. In contrast, the etiology of an isolated defect is not well understood. We observed a short sternum  in a completely asymptomatic 13-year-old woman. A comprehensive instrumental exams panel was performed to exclude associated anomalies of the heart and of the other organ systems. The patient was completely asymptomatic and she did not need any medical or surgical treatment. To our knowledge, this is the first case of isolated short sternum reported in literature. Congenital sternal defects are rare deformities frequently associated with other anomalies of the chest wall and other inner organs. The etiology is not well understood .We observed a type of isolated sternal defect to our knowledge not previously reported in literature, an abnormal development of the sternum in which a dysmorphic manubrium, a hypoplastic body, and the complete absence of the xiphoid process were identified, in an asymptomatic adolescent female without inner organs anomalies.A healthy 13-year-old female was referred to our department for a postural kyphosis. The physical examination showed a healthy adolescent girl, 170 cm tall, with a normal and flexible kyphosis and no signs of spinal deformities. Only a hypermobility of the shoulder toward the anterior midline was observed . BecauseThe patient was completely asymptomatic and she did not need any medical or surgical treatment.Congenital abnormalities of the anterior thoracic wall comprise a spectrum of deformities such as thoracic ectopia cordis, cervical ectopia cordis, thoracoabdominal ectopia cordis, pectus excavatum, pectus carinatum, cleft sternum, and short sternum .Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity (less than 30 cases of isolated cleft sternum are reported in literature) –6, in moIn contrast, the etiology of an isolated defect is not well understood. Many factors have been associated with murine models, like alcohol, riboflavin and methylcobalamin deficiency, and HOX4 gene disruption, but no significant associations have been reported in humans –7.A case that is worth mentioning is that of Santa Rosa da Viterbo, in which a careful study of the well-preserved body showed that she was suffering from total agenesis of the sternum without other apparent malformations , 9.We reported a case of an isolated short sternum in a healthy 13-year-old female patient. The clinical and instrumental examination ruled out other anomalies of inner organs related to known syndromes.Although there is a large literature about the sternal defects and some reported cases of syndromic short sternum  –17, aftePatients affected by chest wall deformity should be interdisciplinarily evaluated for other symptoms related to thoracic and abdomen organs to exclude eventual known syndromic causes.


The greater omentum is an uncommon location for primary tumors. Metastatic tumors of the omentum are common. In contrast, primary tumors of the omentum are very rare. Sporadic cases of primary rhabdomyosarcoma (RMS) arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We report a well-documented case of primary intra-abdominal RMS in a 21-year-old man who presented with complaints of abdominal pain and lump in left hypochondrium region. Imaging revealed it to be a large mass in the left hypochondrium region displacing the bowel loops. Further investigations revealed omental RMS. The mass had originated from the greater omentum and was excised. Our case is doing well and is presently receiving chemotherapy. Sporadic cases of primary RMS arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. Intraperitoneal RMS, and in particular with omental involvement, in any age has been rarely reported in literature. RMS usually manifests as an expanding mass. Tumors in superficial locations may be palpable and detected relatively early, but those in deep locations  may grow large before causing symptoms. Cells are usually positive for intermediate filaments and other proteins typical of differentiated muscle cells, such as desmin, vimentine, myoglobin, actin, and transcription factor myoD. Treatment responses and prognoses widely vary depending on location and histology. While the optimal management of this rare tumor is unknown, early recognition and diagnosis, and a prompt multimodality treatment approach of surgery, chemotherapy, and radiotherapy offers the best chance of cure.RMS is an uncommon neoplasm in the adult population. The name is derived from the Greek words A 21-year-old-man was admitted to our hospital with a 1-month history of abdominal pain. Physical examination revealed a palpable mobile lump in the left hypochondrium region and extending up to the epigastrium region. There is no past history of any surgical interventions and any chronic illness. No positive family history of any hereditary disease or any carcinoma. No abnormalities in routine blood workup. Ultrasound abdomen was suggestive of a well defined but irregular hypoechoic mass lesion in the left hypogastric region and extending into the lumbar region. Colonoscopy was done which was negative for any intramural growth and no signs of malignancy. Ultrasound guided FNAC from the left hypochondrium region showed deposits of adenocarcinoma. The abdominal CT revealed a large well defined mass lesion in the left hypochondrium, measuring 9.8 × 7.4 cms, causing displacement of the bowel loops  compared with vincristine, Actinomycin, and Cyclophosphamide with Vincristine, topotecan, and cyclophosphamide (VAC/VTC) for intermediate rhabdomyosarcoma was done at weeks 3, 9, 21, 27, 33, 39 did not show an improvement in outcome for patients with intermediate-risk RMS. The estimated 4-year FFS rates were 73 % for VAC and 68 % for VAC/VTC, which did not differ significantly [Radiotherapy plays an important role in the treatment of RMS and is a major tool in the treatment of children. A cumulative dose of between 36 and 41.4 Gy is generally sufficient to control microscopic residual disease, and doses of between 50.4 and 54 Gy are utilized to control gross residual disease. Patient is to be followed every 3 months for the 1st year, then every 6 months for the 2nd and 3rd year and then yearly after .The diagnosis of tumors in the omentum is very difficult and requires confirmation by excision and histologic evaluation. A primary tumor originating in the omentum is rare but possible even as a malignant tumor. The prognosis depends mainly on tumor biology yet the malignancy of the omentum might cause severe outcomes because these masses can easily metastasize to mesentery and peritoneum tissues.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


This report describes two newborn girls with single perineal opening (cloaca), and pseudoexstrophy in the form of divergent pubic bones and rectus muscles, and a low-set umbilicus. Both patients had a type II congenital pouch colon (CPC) with one hemiuterus and vagina on each side in the pelvis. In one patient, a Meckel’s diverticulum was present 5 cm from the ileocecal junction. In both girls, a diverting proximal ileostomy was the initial surgery. Case 1A 20-day old full-term girl, weighing 3.1 kg, was brought with complaints of passing urine and stools from a single perineal opening. The baby was feeding normally and thriving. There was no significant antenatal history. Examination revealed the stigmata of pseudoexstrophy with a low-set umbilicus, scarring around the umbilicus, and wide pubic diastasis . Perineal examination showed a wide cloacal opening along with folds radiating inwards and upwards from the margins of the opening. The perineum was well-developed. The rest of the clinical examination was normal. Hematological and biochemical parameters, including renal function tests were within normal limits. Plain X-ray abdomen showed a large air-fluid level on the left side of the abdomen and a normal sacrum. Abdominal ultrasound (US) showed normal kidneys. Laparotomy revealed a type II CPC with 5 cm of normal colon, a single appendix, and one hemiuterus and vagina flanking the lower terminal portion of the colonic pouch on each side. A divided ileostomy was constructed proximal to the colonic pouch. Postoperative recovery was uneventful.Case 2A preterm (32 weeks gestation with 1.814 kg weight) girl was brought soon after birth with a single perineal opening. Clinical and radiological examination  showed similar findings as reported in the first case except for mild hydronephrosis of the left kidney and a distended urinary bladder on ultrasound abdomen. Laparotomy revealed type II CPC with a distended thin-walled colonic pouch, a single appendix, a distended flaccid bladder, and one hemiuterus and vagina on each side in the pelvis, flanking the lower terminal portion of the colonic pouch . A Meckel’s diverticulum was present just 4-5 cm proximal to the cecum. The grossly distended pouch was decompressed by a small incision on its anterior surface which was closed subsequently. A divided ileostomy was constructed proximal to the pouch and a suprapubic cystostomy performed with a No. 10 Fr Foley’s catheter. Postoperative recovery was uneventful and the child is at present awaiting further corrective surgery.Pseudoexstrophy or covered exstrophy is a variant of classical vesical exstrophy in which all the usual musculoskeletal findings of classical exstrophy are present but the bladder is closed with varying degrees of skin and subcutaneous cover [1-3]. Abnormalities of the external genitalia are frequent and these include a bifid clitoris in 50 % girls, a small laterally displaced penis, partial diphallus with incomplete urethral duplication, and two widely separated scrotal halves [4-6]. Unlike classical exstrophy, abnormalities of the upper urinary tract like vesicoureteric reflux or renal dysplasia have been reported , and an associated ARM may be present .There are 2 reports in the literature of patients of CPC with associated pseudoexstrophy . Chadha et al [1] described a male newborn with similar pseudoexstrophy and type IV CPC. Herman et al [2] reported a newborn girl with type II CPC and pseudoexstrophy associated with various spine, spinal cord, GIT, and genitourinary anomalies.Exstrophy variants are explained by incomplete rupture or persistence of an abnormal infraumbilical cloacal membrane which acts as a wedge and prevents the lateral mesoderm from organizing and progressing medially between the ectodermal and endodermal layers [4]. CPC is believed to result from a very early arrest in partitioning of the cloaca by the urorectal septum (URS) [8] which may be due to early errors in organization, growth, and proliferation of the mesenchyme in the region of the URS. If this mesodermal defect also affects the mesenchyme in the adjacent developing hindgut, ‘defective organogenesis’ of the hindgut at an early stage before it lengthens could account for later development of the colonic pouch with its unique anatomic and physiological characteristics . Significantly, especially in reports from India, CPC is infrequently associated with major malformations in other organ systems and the sacrum is usually normal . There is a high incidence of a Meckel’s diverticulum  with sometimes, as in one of our cases, the diverticulum being close to the ileocecal junction [8]. This suggests that the abnormal development and shortening may affect the entire post-arterial limb of the midgut and hindgut [8]. It is tempting to suggest, therefore, that if the defective differentiation and growth of the mesenchyme in the URS and adjacent hindgut is associated with a similar mesodermal defect in the infraumbilical region of the ventral body wall, a combination of CPC and pseudoexstrophy may result. The multiple anomalies in the case reported by Herman et al [2] and in some cases of pseudoexstrophy reported in the literature  may be because in these cases, the abnormality was part of a more generalized error in mesodermal development and differentiation in the caudal developmental field.Source of Support: NilConflict of Interest: None declared


Paravertebral ganglioneuroma and scoliosis is a rare clinical benign disease. The case we reported is about a 12-year-old girl who was hospitalized due to neoplasm with spinal deformity in the right abdomen for 1 month. Based on a careful preoperative evaluation and found no obvious surgery contraindications, the patient was treated with surgical resection of the tumor and correction of the deformity by surgery. Postoperative pathologic examination confirmed it was a ganglioneuroma. After the operation, the patient recovered well. Her spinal deformity was corrected, and she was 5 cm taller. Complete resection of ganglioneuroma following with a low recurrence rate and a good prognosis, patient does not need further chemotherapy, radiation therapy, or other treatments. All follow-up radiographic studies demonstrated no relapse of the tumor in the following 18 months. Combining this case with similar cases at home and aboard and reviewing related literature, we formed conclusions based on the manifestations, diagnosis, treatment, and prognosis of this disease and provided treatments for similar cases. Ganglioneuromas are rare benign tumors that originate from a neural crest or sympathetic ganglion . They mo12 to L2 vertebrae (Fig. A 12-year-old girl visited our hospital with “a right abdominal mass and spinal deformity 1 month” on July 22, 2014. When she was 3, the girl was received a mass resection operation in the local hospital, but without postoperative pathologic examination. She recovered well after the surgery. At the age of 5, her parents found that their daughter had mild claudication, but they did not take it seriously until 1 month ago, when she complained of a right abdominal mass with mild pain. In the meantime, the parents noticed the girl had scoliosis. She was then hospitalized in our orthopedics department. The child did not have a family history of tumors. Upon physical examination, we found an old vertical operation scar sized 5 cm in the right abdominal region. In the right lumbar region, we identified a lump measuring 10 cm × 7 cm that was solid, without clear boundaries, immobilized, and having no tenderness. A lumbar right curvature could be observed, and there was no direct or indirect percussion pain or neurological deficits. From a radiographic examination, the lower segment of the thoracic and lumbar spine showed a right tumefied thoracolumbar curve and left thoracolumbar curve around the 1st lumbar body at a Cobb angle of 33.7° and Ferguson angle of 69.4° Fig. , respectrae Fig. –e. The p1 nerve root and extended into the intervertebral space between L1 and L2. The posterior and partial paravertebral elements of the lump were removed following the reclamation of a vertebral column using a screw-rod system as an internal fixation by a posterior approach. The mass was 11 cm × 7 cm × 4 cm and well encapsulated, luidity section, and was solid with a tough texture (Fig. 1 to L2, extending T10 to L4, measuring 13 cm × 8 cm × 6 cm, identical in character to the first mass, the giant tumor (Fig. The large tumor was adjacent to important organs such as the right kidney and inferior vena cava. Moreover, the patient presented with scoliosis. Therefore, a staging operation was performed. The first stage operation by posterior approach aimed to resect the giant tumor, which was derived from the nerve, and to correct the scoliosis. In surgery, we saw that the mass originated from the Lure Fig. . Postopeure Fig. –f. The smor Fig. . The pos1 vertebral body maintained a right direction thoracolumbar scoliosis. We measured a Cobb angle of 15.3° and a Ferguson angle of 44.3° (Fig. In postoperative plain X-radiographs, the instrument, used for internal fixation, formed an image in the vertebral body and paravertebral region. The lower thoracic and lumbar vertebra surrounding the L.3° Fig. , b. A fu.3° Fig. , d. AfteTo review the cases of paravertebral ganglioneuroma and scoliosis, we used “scoliosis” and “ganglioneuroma” as keywords to search Medline for publications from the preceding 40 years. Using the strategy, we found 16 reported cases in 13 papers. For details regarding age, position, clinical characteristics, and follow-up, see Table Neurogenic tumors may be broadly classified as arising from nerve cells or nerve sheaths. The former group includes ganglioneuroma, ganglioglioma, ganglioneuroblastoma, and neuroblastoma, and the latter includes neurilemmoma, neurofibroma, and malignant schwannoma . In 1941Osteoidosteoma is one of the most common types of scoliosis deformities caused by tumors . The scoThe typical manifestation of ganglioneuroma is low density and punctate calcification on plain CT, showing a high T2 signal on MRI and a gradual increase in enhancement on dynamic images; it presents as non-enhancement or mild enhancement in the arterial phase of CTs or MRIs and progressive mild enhancement in the delayed phase. If ganglioneuroma shows an atypical manifestation on a CT and an MRI, we consider the tumor to contain a malignant component . In receThe ganglioneuroma shows complete capsular and basal growth by expansive patterns. The most effective therapy is a surgical removal operation as soon as possible, which can reduce the risk of malignant transformation, paraplegia, and other abnormalities. Moreover, surgery has a good prognosis. If the tumor does not affect the vital organs, it should be removed completely; however, if complete resection may cause serious complications, then a partial resection should be performed . In thisGanglioneuroma has low recurrence rate, a good prognosis, and does not require chemotherapy, radiation therapy, or other treatments following complete resection , 28. TheThoracolumbar paravertebral giant ganglioneuroma and scoliosis is quite uncommon. Through clinical manifestations and auxiliary examination, we can make a preliminary diagnosis, but we nevertheless require pathological diagnosis results for confirmation. The treatment of a ganglioneuroma is divided into a two-stage surgical operation: first, the correction of the deformity and then the resection of the tumor. Long-term, postoperative follow-up series indicate a low incidence of recurrence and a good survival rate if the tumor is completely resected.The patient and the families were informed that related data and attached images in the case would be submitted for publication. Since they had signed the Informed Consent Form, its copy can be provided to the journal.


Failure of eruption of primary teeth can be considered rare, especially in maxillary anterior teeth. The problem can be either mechanical obstruction of eruption or a failure of the eruption mechanism. This case report presents failure of eruption of the maxillary right deciduous central incisor in a 4-year-old girl. The unerupted primary tooth was removed surgically. The histological finding revealed fibroma with reactive giant cells. Periodic follow-up visits were advised to monitor the developing dentition and to ensure enough space for the permanent incisor.How to cite this article: Shakra KA. Management of Unerupted Maxillary Deciduous Central Incisor: A Case Report. Int J Clin Pediatr Dent 2014;7(1):58-60. Prevalence of impacted primary teeth has been reported to occur in 1:10,000 times, most commonly involving the primary second molars and usually affecting females.5Unerupted maxillary incisors are of concern to both the dentist and the parents since this can have a big Influence on dental and facial esthetics. In addition for its psychological effects, this can cause speech and mastication problems. Therefore, it is important to detect and manage the problem as early as possible.A 4-year-old girl attended the Pediatric Dentistry Clinic at Queen Alia Military Hospital  complaining of unerupted upper right central incisor tooth. As reported by the parents, the tooth had never erupted, and they could not recall any trauma to the oral cavity or the head and neck region. The girl had no medical problem and had a normal past family history. The patient was not taking any medications. There was no history of caries or intraoral infection.Extraoral examination revealed no abnormal signs, no asymmetry and no lymphadenopathy.The intraoral examination showed primary dentition stage, where all the primary teeth were present except the maxillary right central incisor. There was a labial swelling (bulge) in the region of the unerupted tooth, which was asymptomatic. The contralateral maxillary left central incisor had erupted normally.Anterior occlusal and periapical radiographs were taken. A single radiopaque structure representing the crown of the impacted tooth was present. This was surrounded by dense radiopaque irregular bone. Root formation was incomplete at that time. The successor tooth was present with wide radio-lucent pulp chamber. No odontomes and no supernumerary teeth were present .Considering the age of the child, the position of primary central incisor, and developmental stage of the permanent central incisors, it was decided to surgically remove the impacted primary central incisor. There was a little chance for normal spontaneous eruption of the tooth and also because it appeared that it may interfere with the development and eruption of the permanent tooth. Informed consent was taken from the child's parents before the surgery.The patient was very cooperative, so the surgical procedure was carried out under local anesthesia. A minimal approach was used in which a small semilunar full thick-ness flap was made by the periodontist, in order to preserve the interdental papillae . The decThe patient was given regular postoperative follow-up visits. The healing was good and the labial bulge had reduced . The pat6Failure of eruption can be considered rare in primary teeth; it is usually more common in permanent teeth and occurs because of many etiologic factors, such as mechanical obstruction in the path of eruption, including odontomas, odon-togenic tumors, ankylosis, trauma and dentigerous cysts. Malpositioning of the tooth germ is another factor, either due to trauma or unknown reasons, which may cause impactions due to lack of space or primary failure of eruption.7In this case report, a rare case of primary failure of eruption of primary central incisor localized to the maxilla was presented. Failure of eruption was associated with giant-cell fibroma, which is a benign non-neoplastic lesion, not associated with trauma or irritation. It is a localized reactive proliferation of fibrous connective tissue. It occurs in the first three decades of life and predominates in females. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells.4There are few case reports in the literature regarding unerupted primary anterior teeth. Kapur A et al in 2008 reported an unusual case of inverted impacted primary incisors.8 Surgical exposure was done and the mechanical obstruction was eliminated to allow spontaneous eruption of the permanent tooth. Lambert M and Rothman DL also reported an unusual impaction of primary lateral incisors.9The inverted primary maxillary central incisors were surgically extracted and prosthetic rehabilitation was done to improve the esthetics, speech and psychosocial behavior of the child. While Das UM et al in 2002 published a case of unerupted maxillary canine associated with compound composite odontoma.10Bodner L and Horowitz I studied impaction of primary incisor.It is important to carefully monitor the eruption of primary and permanent teeth for early identification and management of any abnormality in tooth eruption. Unerupted deciduous teeth with defects should be extracted at the proper time taking into consideration the development of the successor tooth and the space relationships in the permanent dentition.


A 54-year-old woman presented with bi-temporal hemianopia, palpitations, and diaphoresis. An invasive pituitary macroadenoma was discovered. The patient had biochemical evidence of secondary hyperthyroidism and GH excess; however, she did not appear to be acromegalic. Surgical removal of the pituitary mass revealed a plurihormonal TSH/GH co-secreting pituitary adenoma. TSH-secreting adenomas can co-secrete other hormones including GH, prolactin, and gonadotropins; conversely, co-secretion of TSH from a pituitary adenoma in acromegaly is infrequent.This case highlights an unusual patient with a rare TSH/GH co-secreting pituitary adenoma with absence of the clinical features of acromegaly.Plurihormonality does not always translate into the clinical features of hormonal excess.There appears to be a clinical and immunohistochemical spectrum present in plurihormonal tumors. Thyrotropin (TSH)-secreting pituitary adenomas are rare and account for 1% or fewer of all pituitary adenomas. Approximately one-third of TSH-secreting pituitary adenomas co-secrete other hormones, the most common of which is growth hormone (GH) (16%) followed by prolactin (11%) and gonadotropins (1%). Conversely, in GH-producing pituitary adenomas, 13% have been shown to demonstrate immunopositivity to TSH A 54-year-old woman was noted on a routine visit to her ophthalmologist to have bi-temporal hemianopia. She had reported difficulty with reading, but had not noted peripheral vision loss. Her medical history included atrial fibrillation, hypertension, cardiomyopathy, and sleep apnea. On review of symptoms, she reported hirsutism, feeling ‘hot’, palpitations, and diaphoresis. Physical examination revealed an obese woman with a BMI of 44, hirsutism, a moon-shaped face, and supraclavicular fullness. She had no goiter or dysthyroid eye disease. She had no overt features of acromegaly such as coarse facial features or broad fingers.4) 11.4 (5.0–11.0 μg/dl), free thyroxine (fT4) 2.1 (0.7–1.8 ng/dl), TSH 2.8 (0.4–5.5 μU/ml), and tri-iodothyronine (T3) 185 (94–170 ng/dl), in addition to hypersomatotropism: insulin-like growth factor 1 (IGF1) 747 (87–267 ng/ml) and GH 1.58 ng/ml  of the sella revealed a large pituitary macroadenoma measuring 2.3 cm and left cavernous sinus invasion, with suprasellar extension compressing the optic chiasm, principally to the left of midline. Initial laboratory testing revealed central hyperthyroidism: thyroxine , GH, follicle-stimulating hormone, luteinizing hormone, prolactin (PRL), and TSH was performed. Immunoreactivity with antibody directed against GH was strong and diffuse, while that of TSH was exceptionally rare and scattered throughout the adenoma (4 10.2 (5.0–11.0 μg/dl) and TSH 4.9 (0.4–5.5 μU/ml). Residual adenomatous tissue was present on sellar imaging , with recurrent evidence of biochemical hyperthyroidism and GH excess; methimazole and octreotide were re-introduced and 2 weeks later thyroid function normalized, T imaging A and B;  imaging B. Methim imaging . Post-op imaging C and D. Plurihormonality does not always translate into the clinical features of hormonal excess. In addition to being distinct from one another with varying degrees of prominence, the clinical signs of hyperthyroidism and acromegaly can overlap. Patients with thyrotropic and somatotropic adenomas tend to present when the adenomas are large and frequently can be invasive and less amenable to surgical cure. Cure rates after surgery have been reported in around a third of patients with invasive tumors De novo transformation of a thyrotropinoma to a thyro-somatotrope adenoma appears to be exceedingly rare. The only case reported arose in a patient with hyperthyroidism from a thyrotropinoma, who developed evidence of GH co-secretion only after treatment with octreotide for 1 year. The authors speculated that a series of genetic mutations resulted in transformation of a monoclonal adenoma to a plurihormonal tumor in utero for normal development of the mammosomatropes that produce GH and PRL, is also required for the maintenance of normal expression of GH, prolactin, and TSH after the separate embryonic origin of thryotropes and has been postulated to play a role in the development of co-secretory pituitary adenomas In our case, the predominant symptoms were those of hyperthyroidism, although the clinical signs of hypertension, cardiomyopathy, and sleep apnea could be a clinical manifestation of acromegaly or morbid obesity as well. Interestingly, immunohistochemistry with antibodies directed against TSH showed rare and sparse positive cells. In contrast, although IGF1 levels elevated to approximately three-times normal and showed diffuse robust staining against GH in the adenoma, the patient did not exhibit signs of acromegaly. One explanation is that the associated ‘classic’ signs often lag behind the elevation of IGF1/GH. Another plausible explanation would be that even though the pituitary macroadenoma has been present for some time as indicated by its size In summary, this case highlights an unusual patient with a rare TSH/GH co-secreting pituitary adenoma in whom symptomatic hyperthyroidism was the dominant and only clinical feature, without acromegaly. Immunohistochemistry was positive for GH but only rarely and sparsely positive for TSH. Secondly, while the patient had symptomatic relief of the signs and symptoms of hyperthyroidism for several weeks after what was thought to be gross total resection of a macroadenoma delimited by an elevated but anatomically intact diaphragm sella, the rapid recrudescence of hyperthyroidism led to early reappraisal and identification of residual component of tumor located wholly within the supra-diaphragmatic, intracranial space. Resection of the invasive, intracranial component of the adenoma led to resolution of biochemical evidence of acromegaly and the patient's clinical hyperthyroidism.All authors were involved in the preparation and writing of the manuscript.


Fibromuscular dysplasia (FMD) is a nonatheromatous, noninflammatory arterial disorder of unknown etiology resulting in vessel stenosis and/or aneurysm formation. The renal and cephalocervical (mainly carotid arteries) arterial beds are classically involved; involvement of visceral arteries is rare. Mesenteric panniculitis (MP) is an inflammatory process of mesenteric fat considered to be of unknown etiology. The majority of cases involve the small bowel mesentery; colorectal MP is rare. To our knowledge, no example of MP due to FMD has been described.A 52 year old man presented with steadily worsening lower abdominal pain. Investigation revealed ischemic rectosigmoid mucosa associated with a large mesenteric mass of unknown nature. Angiography showed the disease was limited to the distribution of the inferior mesenteric artery. Subsequent symptoms of large bowel obstruction necessitated a left hemicolectomy. Pathologic examination showed bowel wall necrosis and massive panniculitis of the rectosigmoid due to FMD. Subsequent angiographic imaging of other vascular beds was negative.Several features of this case are noteworthy: FMD limited to the inferior mesenteric artery has not been previously reported, FMD has not previously been implicated as a cause of MP, and the massive extent of panniculitis. An accompanying literature review of cases of visceral FMD, traditionally believed to almost exclusively affect females, highlights a greater than anticipated number of males (33 %), and a gender difference regarding concomitant involvement of cephalocervical and/or renal vascular beds . The latter observation may have implications regarding the value of radiologic screening of other vascular beds, particularly in asymptomatic males, in patients presenting with visceral artery FMD.The online version of this article (doi:10.1186/s12876-015-0303-5) contains supplementary material, which is available to authorized users. Fibromuscular dysplasia (FMD) is a nonatheromatous, noninflammatory arterial disorder of unknown etiology resulting in vessel stenosis and/or aneurysm formation . The renA 52-year old man came to the emergency department after ten days of steadily worsening crampy pain in the left flank and iliac fossa. His medical history was unremarkable. He had stopped smoking ten years previously (15 pack-years). The temperature was 38.8 C. Physical examination of the lower abdomen elicited some guarding, but the abdomen was otherwise supple with no palpable mass. The leucocyte count was normal. He was admitted to hospital.An abdominal CT scan showed features compatible with colitis, probably ischemic, extending from the splenic angle to the rectum. The bowel wall was thickened with “infiltration” of the surrounding fat. A 3 cm left renal mass was also noted. Although colonoscopy showed colitis of irregular distribution, perhaps infectious, a biopsy specimen revealed normal colonic mucosa. The patient was discharged with a prescription for an antibiotic.Four days later he returned to the emergency department with pain similar to that at his first admission. He was admitted to hospital. A CT scan was performed Fig.  which shThree weeks later a diagnostic laparoscopy was performed which found ascites and multiple whitish epiploic appendices, one of which was biopsied with a subsequent microscopic diagnosis of fat necrosis. A loop colostomy was performed.Six weeks later symptoms of large bowel obstruction developed; a left hemicolectomy with transverse colostomy was therefore performed. During the same intervention a left partial nephrectomy was carried out. The rectal stump was left open with a drain.The renal mass showed microscopic features characteristic of an oncocytoma.Macroscopic examination of the recto-sigmoid resection specimen Fig.  showed dMicroscopic examination Fig.  confirmeThe postoperative course was uneventful and the patient was discharged home. Ten months later, he is asymptomatic and he is due to have his colostomy reversed.Fibromuscular dysplasia (FMD) involving visceral arteries in adults is rare (or rarely reported), with only 75 well-documented cases published since 1963 (Table Mesenteric panniculitis (MP) is a rare inflammatory disorder leading to “tumorlike” enlargement of the mesentery, the vast majority of cases involving the small bowel , 3. It iFMD is defined as a “nonatheroscleotic, noninflammatory vascular disease that may result in arterial stenosis, occlusion, aneurysm, or dissection ”. The reThe angiographic appearance of medial FMD is classically described as “string-of-beads”   and is sA 2014 consensus document  recommenTable Of note, regarding the reported cases of visceral FMD, we must mention an extraordinary publication from 2005  which taIn conclusion, we describe FMD as the etiology of a case of massive colorectal MP. FMD of visceral arteries may have “atypical” clinical and radiologic features, and, although a rare entity, should be considered when MP is diagnosed.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Myeloid sarcomas (MS) are rare tumours occurring at extramedullary sites. They are usually associated with other haematology disorders such as acute myeloid leukaemia, myelodysplastic syndrome, and chronic myeloproliferative neoplasms. They frequently occur with a diagnosis of acute myeloid leukaemia (AML) or with relapse of preexisting disease. Patients with myeloid sarcomas without history or evidence of myeloid leukaemia typically progress to form AML.  A case report of a patient diagnosed with an isolated myeloid sarcoma that rarely did not transform to AML but instead spread to form multiple myeloid sarcomas throughout the body.  This case identifies the risk of metastatic spread of these tumours rather than the development of AML which is poorly documented in the literature, due to the rarity of cases, and may be significant in the investigation and management of isolated myeloid sarcomas. This case highlights the need for clinicians to consider repeat cross-sectional imaging to investigate unexplained clinical decline or symptoms, when there is no sign of AML progression and to consider radiotherapy treatment early. Myeloid sarcoma (MS) is a rare tumour containing immature myeloid cells or blasts occurring in an extramedullary site, associated with acute myeloid leukaemia (AML), myelodysplastic syndrome (MDS), or chronic myeloproliferative neoplasms. Myeloid sarcoma usually presents with AML, following diagnosis, or as relapse of previous disease and is reported in 2–9% of AML cases. The term isolated myeloid sarcoma is considered where tumour is found without any current evidence of blood or bone marrow involvement or previous history of myeloid disease. Most cases of isolated myeloid sarcomas will progress to AML within the year. Myeloid sarcomas can be found in various sites, most commonly in soft tissue, bone, and lymph nodes –4.This case reports a patient with isolated MS which fails to progress with blood or bone marrow involvement but disseminates to form multiple myeloid sarcomas throughout the body within two years of diagnosis. We present this case to highlight the risk of metastatic spread of these tumours rather than development of acute leukaemia which is poorly documented in the medical literature and may be significant in the investigation and management of the patient.A 53-year-old male, with worsening chronic back pain but no focal neurology, was referred for a haematology opinion following a magnetic resonance imaging (MRI) scan which showed a large extradural soft tissue mass from T5 to T9 that was displacing the spinal cord, pressing on his descending aorta, and extending through his left intercostal muscles. Biopsy with morphology and immunohistochemical staining of this paraspinal mass confirmed a myeloid sarcoma. Many of the neoplastic cells found on morphology were myeloblasts with eosinophilic cytoplasm, fine chromatin, and prominent nucleoli  scan performed at a similar time demonstrated metabolic activity of this paravertebral mass shown in . Due to During the second intrathecal chemotherapy administration, cerebrospinal fluid (CSF) taken identified myeloid blasts. The patient was subsequently changed to FLAG-IDA chemotherapy, the gold standard therapy at the time, for high risk leukaemia.An MRI scan following the first course of FLAG-IDA chemotherapy showed a good response with 50% reduction in size of the mass, with subsequent CSF samples showing no signs of further blasts. The intended plan of weekly intrathecal Cytarabine became difficult for the patient to tolerate and was hindered by episodes of neutropenic sepsis. However, as FLAG-IDA chemotherapy contained high dose Cytarabine with more effective CNS penetration the need for intrathecal Cytarabine was reduced. Two further CSF samples following FLAG-IDA chemotherapy showed no blasts and therefore intrathecal therapy was discontinued.Following three courses of FLAG-IDA chemotherapy a repeat PET scan was performed shown in . AlthougFollowing radiotherapy treatment aimed at the site of the mass  but also with PET scan imaging .There is limited evidence on the prognosis of myeloid sarcomas, with most conclusions from retrospective studies or case comparisons. Some case reports identifying isolated myeloid sarcoma with no progression to AML were lost to follow-up during the study period with the progression of disease unknown , 10, 11.Yamauchi and Yasuda  found isAlthough the role of radiotherapy as additional treatment for the management of myeloid sarcoma has not been fully established, the data is promising. Bakst et al.  analysedThere are limited studies on prognosis of myeloid sarcoma due to the rarity of cases and lack of randomised controlled trials. Not only is the progression of disease by development of AML, but also, as highlighted in this case, further metastatic deposits can occur. This risk should prompt clinicians to consider repeat cross-sectional imaging to investigate unexplained symptoms or clinical decline when blood monitoring shows no progression to AML.Currently evidence supports the management of isolated MS with systemic chemotherapy regimens used for treatment of acute myeloid leukaemia; however, the role of radiotherapy should be explored further and considered earlier in treatment.