Ultrasound can rapidly identify abnormal signs, which in high prevalence settings, are highly suggestive of extra-pulmonary tuberculosis (EPTB). Unfortunately experienced sonographers are often scarce in these settings.A protocol for focused assessment with sonography for HIV-associated tuberculosis (FASH) which can be used by physicians who are relatively inexperienced in ultrasound was developed.The technique as well as normal and pathological findings are described and the diagnostic and possible therapeutic reasoning explained. The protocol is intended for settings where the prevalence of HIV/TB co-infected patients is high.FASH is suitable for more rapid identification of EPTB even at the peripheral hospital level where other imaging modalities are scarce and most of the HIV and TB care will be delivered in the future. In sub-Saharan Africa, the convergence of human immunodeficiency virus (HIV) and tuberculosis (TB) epidemics has led to a resurgence of extrapulmonary TB (EPTB). EPTB accounts for 15% to 20% of all TB cases reported by TB control programs in the African region [Common manifestations of EPTB include pericardial effusion, pleural effusion, and abdominal TB. Tuberculous pericarditis is consistently reported as the predominant cause of pericardial effusion in Southern Africa and is in most of the cases associated with HIV co-infection . CardiacUltrasound (US) can rapidly identify abnormal signs, which in high prevalence settings, will be highly suggestive of EPTB ,8, but tA list of key ultrasound findings was included in the protocol. The selection criteria were based on the relative ease to recognize the findings and the relevance with respect to diagnostic and therapeutic decisions. The FASH basic assessment Table  is meantPatient is placed in a comfortable supine position. The procedure is briefly explained to the patient reassuring him/her that it is completely painless. A curved 3.5 to 5-MHz transducer is used for the examination of the abdomen. Gain, depth, and focus might need to be adjusted for optimal imaging. Sufficient amount of ultrasound gel is placed on the transducer and the patient's abdomen to ensure good acoustic coupling between the probe and skin. For the beginning of the examination (probe position 1a and 1b), the patient is asked to place his/her arms beside the body to relax the muscles of the abdominal wall.The patient is scanned by six different probe positions Figure . In eachProbe position 1a: pericardial effusionIndication: Pericardial effusion should be considered in every HIV-positive individual with signs of breathlessness, retrosternal pain, and signs of progressive heart failure, tachycardia or hypotension. A chest X-ray showing cardiomegaly is frequently caused by pericardial effusion and should be further assessed by ultrasound.a. Probe position: The probe is placed transverse in the epigastric angle; the transducer is then tilted cranially to get a view of the intra-thoracic organs and the heart. The patient should try to relax his abdominal muscles (arms placed beside the body) as an attempt is made to ‘scoop’ the transducer under the ribs. Commands asking the patient to inspire might help to displace the heart caudally and improve visualization.b. Normal findings: The left lobe of the liver will be visible in the upper parts of the image serving as an acoustic window. Below this, the right atrium as well as the right ventricle will be seen and can be easily recognized by the rhythmic contractions. The right ventricle increases in size during diastole and contracts during the systolic phase of the heart action. The pericardium is seen as an echogenic structure between the liver and the heart; the parietal and visceral pericardium are inseparable , as fluid collects in the dependant parts due to gravity.b. Normal finding: The diaphragm and apical parts of the liver should be visible. If no effusion is present, the air in the basal parts of the lung will cause artifacts resembling a curtain that moves up and down with the respiratory cycle.c. Pathological findings: Anechoic, black fluid may be visible in the costophrenic angle  [Ultrasound can be used to guide thoracocentesis, and the fluid should be examined for TB. As in pericardial fluid, the AFB smear is often negative, but higher rates of positivity were seen in patients with low CD4 counts (<200 × 106/L) . TB cult6/L) . Until fProbe position 3a: ascites in the hepato-renal pouch (Morrison's pouch)Indication: see probe position 1b.a. Probe position: The transducer is moved a few centimeters caudally and is slightly rotated so that the longitudinal axis is parallel to the long axis of the body. Again, it is important to bring the scanner dorsally to see fluid in the dependent parts of the abdominal cavity.b. Normal finding: The caudal edge of the liver and the right kidney should be visible. Between the two organs, there is usually an echogenic, white line .e. Probe position 5b: focal splenic lesionsIndication: see probe position 1b.a. Probe position: Mirroring the probe position 3b, the transducer is moved slightly upwards and turned to be parallel to the ribs using the intercostal space as a window.b. Normal finding: The homogenous tissue of the spleen should be visible.c. Pathological findings: Hypoechoic, dark lesions in the spleen (size approximately 0.5 to 2 cm)  Figure : Video 7Interpretation: Multiple hypoechoic lesions may represent small abscesses, which are frequently secondary to disseminated TB [e. nated TB . The imaProbe position 6: ascites in the pouch of DouglasIndication: see probe position 1b.a. Probe position: The probe is placed on the lower abdomen touching the upper rim of the symphysis pubis. The pelvic region can be scanned in the longitudinal axis  or in the transverse axis . For visualization of parts deeper in the pelvis, the transducer has to be tilted upwards so that the caudal structures of the small pelvis become visible.b. Normal finding: The bladder should be visible and its size will vary depending on how full it is. As urine is a fluid, it will be visible as an echo-free, black area, and care has to be taken not to confuse this with free fluid. Behind the bladder, the uterus might be visible as a pear-shaped organ in the female patient. Behind these structures and in front of the rectum, which cannot be seen due to air artifacts, the pouch of Douglas is located  or behind the uterus  in the Douglas' pouch. These represent free fluid  need to be considered. In comparison to free fluid, these differ in shape, are localized, and are usually limited by a wall.e. Focused assessment with sonography for HIV/TB is a technique that can be taught rapidly to physicians with little or no prior ultrasound experience. In the high prevalence setting, the learning process is facilitated by the fact that pathologic findings are present in a large proportion of HIV/TB co-infected hospital inpatients. The fact that many patients under investigation are underweight makes scanning easier because fat interfering with the scanning becomes less relevant. The examination takes only a few minutes and may provide important findings that alter patient management .If available, a more comprehensive US scan of the abdomen of the HIV-infected patients may reveal other pathological findings  such as It is important to remember that the FASH examination results have to be interpreted by a clinician within the clinical and epidemiological framework applying to the individual patient. In patients with findings suggestive of EPTB, a sputum examination  and a chFASH does not provide the health care worker with a definite diagnosis but may reveal findings that point in the direction of EPTB. The potential public health benefit of the protocol may be far reaching, in terms of more rapid identification of EPTB cases even at a peripheral hospital level, where imaging modalities are scarce and most HIV and TB care will be delivered in the future.The authors declare that they have no competing interests.TH developed the FASH protocol and used it extensively during ultrasound teaching, and assisted in drafting the manuscript. CW drafted the manuscript. SG revised the manuscript and added clinical discussion. EB used the FASH protocol during the ultrasound teaching and revised the clinical discussion. All authors read and approved the final manuscript.http://www.tropicalultrasound.org) held annually in Pavia, Italy, and the co-director of the course ‘Clinical Ultrasound in Tropical Infectious Diseases’ held annually in Lima, Peru. Both courses are co-sponsored by the World Health Organization, the American Society of Tropical Medicine and Hygiene, and WINFOCUS.TH is an internal medicine and infectious disease physician with long experience in teaching ultrasound. He developed the FASH protocol in Hlabisa Hospital, a district hospital in rural KwaZulu-Natal, South Africa. He taught and teaches FASH ultrasound in Pavia, Italy as well as in many African countries. CW is a microbiologist, infection control specialist, and infectious disease clinician with a strong interest in ultrasound, diagnostic methods, and epidemiology of HIV and TB. SG is an internal medicine-pediatric trained physician with an interest in research and clinical teaching of ultrasound in resource limited settings. EB is an infectious diseases clinician with long term experience in ultrasound and an assistant professor of infectious disease at the University of Pavia. He is the director of the ‘Short Course on Abdominal Ultrasound in Infectious and Tropical Diseases’ . A large (approximately 8 cm) homogenous lesion is seen in the parenchyma of the liver. By aspiration, an amebic liver abscess was diagnosed.Click here for fileMultiple hypoechoic focal lesions. The lesions are visible in the spleen representing micro-abscesses due to disseminated TB.Click here for fileAscites in the pouch of Douglas. Anechoic fluid can be seen behind the bladder and uterus.Click here for file


Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies. Vascular anomalies are congenital lesions of abnormal vascular development. Previously referred to as vascular birthmarks, vascular anomalies are now classified based on a system developed in 1982 by Mulliken and Glowacki that considers histology, biological behavior, and clinical presentation of these entities [Both vascular tumors and malformations may occur anywhere on the body. In brief, hemangiomas are vascular tumors that are rarely apparent at birth, grow rapidly during the first 6 months of life, involute with time and do not necessarily infiltrate but can sometimes be destructive. Vascular malformations are irregular vascular networks defined by their particular blood vessel type. In contrast to hemangiomas, they are present at birth, slow growing, infiltrative, and destructive. Almost all vascular malformations and nearly 40% of hemangiomas eventually require intervention. Thus, this paper offers pediatricians an update on recent developments in the diagnosis, management, and pathogenesis of vascular anomalies. Due to their complexity, a multidisciplinary approach is frequently necessary in managing these lesions and includes a team of specialists in pediatric otolaryngology, dermatology, hematology, interventional radiology, surgery, orthopedics, and sometimes psychology.Infantile hemangiomas are the most common tumor in infancy and occur in approximately 10% of the population. Identifiable risk factors include female sex, prematurity, low birth weight, and fair skin . They coHemangiomas are further categorized into two types: “infantile” or “congenital.” The rare “congenital” hemangioma is less understood and present at birth. Congenital hemangiomas either rapidly involute ) over a very brief period in infancy or never involute ). The remaining sections will focus on the more common “infantile” hemangiomas.The pathogenesis of infantile hemangiomas remains unclear, although two theories dominate current thought. The first theory suggests that hemangioma endothelial cells arise from disrupted placental tissue imbedded in fetal soft tissues during gestation or birth. Markers of hemangiomas have been shown to coincide with those found in placental tissue . This isInfantile hemangiomas present shortly after birth most often as well-demarcated, flat, and erythematous red patches. At this stage, hemangiomas may be confused with other red lesions of birth, but rapid proliferation and vertical growth will trigger the diagnosis . GeneralHemangiomas can be superficial, deep, or compound. The superficial hemangioma is red and nodular with no subcutaneous component. A deep hemangioma presents as a protrusion with an overlying bluish tint or telangectasia. Compound hemangiomas have both deep and superficial components . This neFollowing proliferation, hemangiomas enter a slower or no growth phase, known as quiescence. This phase typically lasts from 9 to 12 months of age. The final and unique phase of the hemangioma lifecycle is involution. This phase is marked by graying of the overlying skin and shrinking of the deeper components . HistoriAnother subclassification for hemangiomas is focal versus segmental disease. Focal hemangiomas are localized, unilocular lesions which adhere to the phases of growth and involution. Multifocal hemangiomatosis also exists, and infants with greater than 5 lesions should undergo workup to rule out visceral involvement. Segmental hemangiomas are more diffuse plaquelike and can lead to untoward functional and aesthetic outcomes. The limb and face are common locations for disease . Head anPatients with segmental hemangiomas should also undergo investigation to rule out PHACES syndrome  . The diagnosis of a hemangioma is best made by clinical history and physical exam. In cases of unclear diagnosis, the best radiographic modalities to use are either a Doppler ultrasound or MRI.Historically, hemangiomas have been managed with close observation over their lifecycle . HoweverMedical and surgical options are available for the treatment of “problematic” hemangiomas. Medical management includes one or more systemic therapies. Corticosteroids, interferon, and vincristine have been successful for massive and life-threatening disease –15. ThesSurgical management involves excision, laser treatment or both. Intralesional steroid treatment is also an option for focal hemangiomas of the parotid, nasal tip, subglottis, and eyelid. Repeat therapy is often required, but systemic side effects are limited . Excision is the appropriate for localized lesions the fibrofatty remnants (residuum) of involuted hemangiomas. Elective subtotal excision of massive protuberant proliferating hemangiomas can be employed in order to maintain aesthetic facial boundaries. Small remnants of disease are then left for involution. Residual erythema and telangiectasias frequently remain in involuted hemangiomas and are best treated by selective photothermolysis using the flash pulse dye laser (FPDL). Similarly, ulcerative lesions during proliferation can be treated with FPDL to induce healing and new epidermal growth.β-adrenergic antagonist, was serendipitously discovered to cause regression of proliferating hemangiomas in newborns receiving treatment for cardiovascular disease [A paradigm shift has occurred regarding the treatment of hemangiomas over the past few years. In 2008, propranolol, a nonselective  disease . Numerou disease –19. In f disease , 22. To  disease . Monitoring the administration of propranolol varies among institutions and practitioners. A unified approach has not yet been determined. However, elective admission with cardiovascular monitoring may be necessary. Outpatient administration with close monitoring has also been successfully performed . NonethePropranolol is currently employed for “problematic” hemangiomas, those that would have received either surgical or some other systemic therapy to prevent untoward side effects. Subglottic, periorbital, and massive hemangiomas seem to respond well . DespiteVascular malformations are rare vascular anomalies composed of inappropriately connected vasculature. Any blood vessel type, or a combination thereof, can be affected in a vascular malformation. These lesions infiltrate normal tissue which makes them very difficult to manage. The most common vascular malformations include lymphatic malformations (LMs), capillary-venular malformations (CM), venous malformations (VMs) and arteriovenous malformations (AVMs) which have been selected to be covered in this paper . While d3), microcystic (<2 cm3), or mixed [Lymphatic malformations (LMs) are composed of dilated lymphatic vessels with inappropriate communication, lined by endothelial cells and filled with lymphatic fluid. Their incidence is approximated to be 1 in 2000 to 4000 live births . LesionsThe etiology of LM is unclear. Although most are congenital, there have been reports of LM occurring after trauma or infection. Receptors involved in the formation of lymphatic vascular channels, such as VEGFR3 and Prox-1, may play a role in the development of this disease .Lymphatic malformations may be macrocystic, microcystic, or mixed. Gradual growth and expansion is typical. Approximately half of the lesions are present at birth and 80–90% by 2 years of age. Local infections approximating the course of lymphatic drainage will cause LM to swell, protrude, and sometimes become painful. This is a hallmark of a LM versus other vascular anomalies that do not present in this fashion.Clinically, the appearance of macrocystic disease differs from that of microcystic. Macrocystic LMs present as a soft, fluid-filled swelling beneath normal or slightly discolored skin . IntracyLM can occur anywhere on the body, and symptoms are determined by the extent of disease. Most LMs are found in the cervicofacial region and extend to involve the oral cavity or airway, especially when mixed or microcystic . SymptomAlthough these malformations can usually be diagnosed by physical examination, MRI is used to confirm diagnosis, identify cystic architecture, and determine extent of disease.An ideal option for treatment of LM does not exist. Several interventions may be required. There have been rare cases of sporadic resolution of a lesion although the majority of these malformations continue to enlarge with age . MacrocyLM may be detected on prenatal ultrasound and may require special interventions during delivery. The EXIT (ex utero intrapartum treatment) procedure provides good airway control of the infant if compromise is suspected to occur at birth.Sclerotherapy is frequently employed for lymphatic malformations, especially if deep seated and difficult to access surgically. It involves injection of a sclerosing agent directly into the lesion leading to fibrosis and ultimately regression of the cysts. Several treatments are usually required, and swelling is expected following therapy. Macrocystic lesions are more easily treated in this fashion, but there have been reports of success in microcystic lesions . SeveralCarbon dioxide laser therapy may also be employed in limited disease of the airway and oral mucosa . MacrocyOverall, treatment for LM should be aimed at complete elimination of disease. When this is not feasible, multiple treatment modalities are combined to control disease and provide satisfactory functional outcomes.Capillary malformations (CMs) are sporadic lesions consisting of dilated capillary-like channels. They occur in approximately 0.3% of children. CMs can present on any part of the body, but are mostly found in the cervicofacial region. They are categorized as medial or lateral lesions depending on their locations. Medial CM gradually lighten with time and eventually disappear. Colloquially they are referred to as stork bites on the nape of the neck and angel kisses on the forehead. Lateral lesions, commonly referred to as port-wine stains, have a more protracted course .Pathogenesis of isolated capillary malformations is unknown. A genomewide linkage analysis has identified a locus on chromosome 5q associated with familial disease . A rare CMs present at birth as flat, red or purple, cutaneous patches with irregular borders. They are painless and do not spontaneously bleed. Lateral CMs, or port-wine stains, usually involve the face and present along the distribution of the trigeminal nerve. CMs tend to progress with time as the vessel ectasia extends to involve deeper vessels to the level of the subcutaneous tissues. This causes the lesion to become darker in color, as well as more raised and nodular .Although they are mostly solitary lesions, CM may exist as a part of a syndrome. The most common of these is the Sturge-Weber syndrome (SWS) and is characterized by a CM in the region of the ophthalmic branch of the trigeminal nerve, leptomeningeal angiomatosis, and choroid angioma. Symptoms of SWS are variable among cases and include intractable seizures, mental retardation, and glaucoma. CM may also be present in Klippel-Trenaunay Syndrome (KTS). This syndrome consists of a combination of multiple lymphatic, venous, and capillary abnormalities.Diagnosis is usually made by physical examination alone. If here are findings inconsistent with CM exist, for example, pain or spontaneous bleeding, an MRI may be performed. An MRI of the brain as well as an annual ophthalmological exam is warranted when suspicion for SWS is present.The mainstay of treatment for CM is laser therapy. The FPDL is efficacious in treating these lesions. The laser slowly causes the redness of the lesion to fade; therefore, many treatments are often necessary . Early tVenous malformations (VMs) are slow-flow vascular anomalies composed of ectatic venous channels. These aberrant venous connections lead to venous congestion, thrombosis, and gradual expansion of these lesions. As a result, VMs persist and progress until therapeutic intervention. The incidence of VMs is approximately 1 in 10,000 . VMs morVenous malformations are often visible at birth but may present as a deep mass. Protrusion may be the only presenting symptom. They are known to grow proportionately with the child with sudden expansion in adulthood. Rapid growth may occur during puberty, pregnancy, or traumatic injury. VM can be either well localized or extensive. The overlying skin may appear normal or possess a bluish discoloration. With more cutaneous involvement, the lesions appear darker blue or purple . Upper aVMs are compressible and swell when the region is dependent or there is an increase in hydrostatic pressure such as during a valsalva maneuver. With time, pain and swelling will occur with the formation of phleboliths , or small clots, secondary to trauma or venous stasis. For very large lesions with significant thrombosis the risk of distal emboli remains low but real. D-dimers may be elevated and a marker of disease . When isNo single treatment modality is favored in the treatment of VMs and often more than one modality is utilized . SurgeryConservative observation of small VM in children may be an option with the knowledge that growth is imminent. Elevating the involved area can decrease hydrostatic pressure and vascular expansion and may impede growth. In large lesions, elevation also decreases swelling and improves pain and airway obstruction. Similarly, compression garments are the initial treatment of choice for advanced limb lesions allowing risks from other treatment options to be avoided. Low-molecular-weight heparin can improve pain from thrombosis .Treatment of larger airway and multifocal disease is often warranted. Symptom-directed therapy is the goal for these lesions. Management techniques typically aim to relieve airway symptoms, pain, and/or disfigurement. Surgical resection and sclerotherapy alone can, at times, be curative for smaller lesions. Local recurrence may occur years after treatment.Laser therapy provides good control of VM . Use of Sclerotherapy, as described above, has been used extensively for treatment of VM . The sclSurgery remains one of the most superior treatment options and may offer a cure for localized VM. Excision of complex lesions remains difficult secondary to intraoperative bleeding. Preoperative sclerosant can be used prior to excision (24–48 hours) to decrease surgical risk. Patients with extensive disease will often require combined modality therapy. Cure is not common, but disease control for many years is often achieved.Arteriovenous malformations (AVMs) are congenital high-flow vascular malformations composed of anomalous capillary beds shunting blood from the arterial system to the venous system. They are often misdiagnosed at birth as other vascular lesions because of the delay in presentation of characteristic signs of the malformation. Puberty and trauma trigger the growth of the lesion and manifestation of its troublesome symptoms . They arLittle is known about the origin and pathogenesis of AVM. A defect in vascular stabilization is thought to cause AVM, but it remains unclear whether these lesions are primarily congenital in origin. Most AVM, are present at birth, but there are several case reports of these lesions presenting after trauma in adults. Defects in TGF-beta signaling and a genetic two-hit hypothesis are the prevailing theories to the pathogenesis , 54. ProDiagnosis of AVM is based upon clinical examination and imaging. A growing hypervascular lesion may have been present as a slight blush at birth. AVMs are often quiescent for many years and grow commensurate with the child. Intermittent expansion will suggest the diagnosis . HormonaThe natural course of AVM is early quiescence, late expansion, and ultimately infiltration and destruction of local soft tissue and bone. Common sites for occurence are the midface, oral cavity, and limbs . Oral leImaging is essential in identifying the extent of AVM. MRI may be useful, but MRA and CTA can give a superior outline of these lesions . NumerouTreatment of AVM consists of embolization, surgical extirpation, or a combination of these modalities. Treatment and timing are often individualized to the patient and the extent of disease. For example, small-vessel AVMs are known to be localized and can be resected with good long-term outcomes . HistoriIntravascular embolization of AVM can be used alone or in combination with surgical excision. Absolute ethanol, polyvinyl alcohol, and ONYX have been employed as AVM embolization materials . These aIn general, surgical management of AVMs requires preoperative supraselective embolization, judicious removal of tissue, and complex reconstructive techniques. In focal lesions, surgical excision has been shown to cure AVM , 63. HowVascular anomalies embody a myriad of blood vessels abnormalities that are thought to occur perinatally. Correct diagnosis is imperative for appropriate treatment. The most common vascular anomalies in order of presentation include hemangiomas, lymphatic malformations, capillary malformations (port-wine stains), venous malformations, and arteriovenous malformations. Treatment of vascular anomalies is complex and often involves multiple disciplines and therapeutic options. Referral to a vascular anomalies team is recommended when considering therapy for “problematic” hemangiomas and vascular malformations.


Second malignancies, mostly in the form of bone sarcomas, are known to occur in hereditary retinoblastomas, which usually present with bilateral disease. Only 2 cases of Ewing’s sarcoma have been reported in the literature following sporadic unilateral retinoblastoma. A 5-year-old boy presented to our hospital with Ewing’s sarcoma of the right humerus (proven by biopsy and immunohistochemistry) following successful treatment of retinoblastoma of the left eye with enucleation and chemotherapy 2 years previously. He was treated with 2 cycles of chemotherapy followed by radiation therapy. At 15 months follow-up, the tumor had reduced in size and the child had a good functional outcome. The cumulative risk of second malignancies in retinoblastoma survivors is 32%. Ninety-eight percent of second malignancies occur in patients with bilateral retinoblastoma. Germ line mutations have been considered in sporadic tumors occurring bilaterally and multifocal unilateral sporadic tumors. Bone and soft tissue sarcomas are the most common second malignancies. Radiation therapy increases the risk of developing a second malignancy in the irradiated field. Unilateral retinoblastomas, which comprise the majority of retinoblastomas, are not immune from the development of second malignancies. Close follow-up of all retinoblastomas—even in the early period—can improve the outcome by facilitating the early detection and aggressive treatment of second malignancies. Retinoblastoma (RB) is the most common malignant ocular tumor in the pediatric age group, occurring in 1/15,000 to 1/30,000 live births , althougPreviously, six cases of Ewing’s sarcoma in patients with a prior history of bilateral , 8, 16 aA 3-year-old male child was brought to the eye center of our hospital with a yellow reflex in the left eye (cat’s eye reflex), and was found to have retinoblastoma of an advanced stage. He was subsequently fully investigated and found to have nonmetastatic locally advanced retinoblastoma. Since there was no vision in the left eye, enucleation was performed as definitive treatment and a specimen was sent for histopathological analysis. It showed degeneration of all layers of the eyeball, with areas of calcification, bone formation and chronic inflammatory infiltrates. Focally, a tumor with a collection of small round blue cells with a high nucleo/cytoplasmic ratio and hyperchromatic nuclei consistent with retinoblastoma was evident Fig. . The optAt the age of 5 years, the child was brought to the orthopedic clinic with complaints of painful swelling of the right arm that had been present for 1 month, which was gradually increasing in size Fig. . The paiRoentgenograms revealed a lamellated pattern of periosteal reaction and a permeative pattern of osteolysis involving the distal shaft of the humerus with soft-tissue shadows Fig. . GadolinA whole-body bone scan showed no evidence of bony metastasis. However, contrast-enhanced computed tomography (CECT) of the chest showed two pulmonary nodules in the right lung, suggestive of metastasis. A guided biopsy of the nodules confirmed the diagnosis of metastasis.MIC2(CD99) was positive, and neuron-specific enolase was negative, strongly suggesting a diagnosis of Ewing’s sarcoma, excluding the possibility of metastasis following retinoblastoma, and ruling out other small round cell tumors (Fig. Open biopsy of the tumor was planned as part of the staging studies. Biopsy revealed small round tumor cells arranged in sheets, infiltrating into the skeletal muscle. Cells were small with hyperchromatic nuclei, inconspicuous nucleoli and a high nuclear-to-cytoplasmic ratio. No rosette formation or Azzopardi phenomenon was seen. This was an indication of a high-grade tumor with a poor immunologic/host response. Immunohistochemical staining for cell-surface glycoprotein p30/32ors Fig. a, b, c.FThe patient was classified as being at an advanced stage of the disease due to the presence of metastasis. Treatment was directed towards palliating symptoms at the local site by radiotherapy and aggressive chemotherapy. Since the patient was already being treated with a standard chemotherapy protocol, a modified chemotherapy regimen with ifosfamide and etoposide was undertaken. The patient underwent 2 cycles of chemotherapy with ifosfamide and etoposide followed by radiotherapy in 2 phases, initially with 44 Gy and subsequently with 20 Gy. The patient was followed up regularly for 18 months. By that time, the swelling decreased in size and was not tender. Complete elbow range of motion was regained, and the vision in the right eye was normal.Survivors of retinoblastoma invariably carry a high risk of developing a second malignant neoplasm, with the cumulative risk being 32% . Ninety-In children previously irradiated for retinoblastoma, 70% of second malignant neoplasms have occurred in the field and 30% outside the field of radiation . AbramsoHeritable retinoblastoma and osteosarcoma were found to be associated in some cases with the deletion of the 13q14 locus of the RB-1 gene . A varieSkeletal scintigrams are not economically feasible to use for screening purposes to detect second malignant neoplasms, despite their capacity for earlier detection . MRI is Children of affected parents have a 50% risk of having retinoblastoma . The risOur patient developed a second malignancy 2 years after the initial diagnosis of RB, but this period is much shorter than those described in previous reports Table : 4.3 yeaThe present case highlights the fact that patients with retinoblastoma are at an increased risk of developing a second malignant neoplasm, the latency of which is highly variable. It is also worth noting that unilateral retinoblastoma, which represents the majority of cases, is not immune from the development of second malignant neoplasms, considering that a germline mutation can never be ruled out. Ewing’s sarcoma, a tumor which responds excellently to chemoradiotherapy, should not be missed in the primary diagnosis of bone sarcomas presenting as second malignant neoplasms. Close follow-up, early detection and aggressive treatment of second malignant neoplasms can improve the outcome.Written consent was obtained from the parents of the child before submission of this case report and of any accompanying images.


Gastrointestinal stromal tumors (GISTs) compose the largest category of well-recognized nonepithelial neoplasms of the gastrointestinal tract (GI). GISTs of the gallbladder are extremely rare tumors. Only four malignant, two benign and one GIST-like tumor of the gall bladder have ever been described. The four malignant GISTs were all positive for CD 117 antigen (c-kit). We present for the first time a malignant gastrointestinal stromal tumor of the gallbladder, immunoreactive for platelet-derived growth factor receptor alpha (PDGFRA) and negative for CD 117 antigen (c-KIT). Gastrointestinal stromal tumors (GISTs) are well recognized nonepithelial neoplasms of the gastrointestinal tract (GI). After decades of ultra structural, immunohistochemical, and genetic studies, it is now evident that most GISTs are believed to differentiate from Cajal (ICCs) pacemaker cells. The Cajal pacemaker cells are involved in gastrointestinal tract mobility and regulation of autonomous neuronal transmission . The vas3, Total Bilirubin/Direct Bilirubin (TBil/DBil) of 4.80/3.60 mg/dL, alkaline phosphatase (ALP) 440 IU/L, and γ-glutamyl transpeptidase (γ-GTP) 210 IU/L. Tumor markers were within the normal range. Ultrasound demonstrated a thickened gallbladder wall, bile stones, and moderate common bile duct (CBD) dilatation. Bile duct lithiasis was suspected and an endoscopic retrograde cholangiopancreatography (ERCP) was performed. This demonstrated an almost complete obstruction at the level of the cystic duct and common hepatic duct confluence. However, the underlying pathology could not be identified, and an exploratory laparotomy was decided. This revealed a large irregular hard mass at the site of the gallbladder. The mass was adherent to the duodenum involving the confluence of the cystic duct with the common hepatic duct and the retroduodenal part of the common bile duct. Cholecystectomy was performed along with wedge resection of the gallbladder bed. The common bile duct and common hepatic were excised en bloc with the specimen ensuring free surgical margins along the extrahepatic billiary tree. Lymph node dissection was performed of the hepatoduodenal ligament, the hepatic artery lymph nodes, and the retropancreatic lymph nodes. The enteric-billiary continuity was established through a retrocolic Roux-en-Y hepatico-jejunostomy. The postoperative course was uneventful, and the patient was discharged ten days after operation. A 72-year-old woman was admitted to the First Department of Surgery, University of Athens with symptoms suggestive of obstructive jaundice: fever and chills, dark urine, intermittent right upper quadrant (RUQ) abdominal pain, and jaundice. Clinical examination revealed mild tenderness in the RUQ only with no palpable masses. Initial investigations showed a white cell count (WCC) of 11,556 cells/mmIn the pathological review, the gallbladder was dilated and measured 7.5 cm × 3.5 cm in size. Within the gallbladder, there was a wide mass covering the entire wall, neck, and body, while the remainder of the mucosal layer was ulcerated. Microscopically, tumor cells infiltrated the muscle layer were spindle shaped in nature, arranged in a short fascicle pattern and immunopositive for PDGFRA Figures . The cells demonstrated hypercromatic nuclei, severe pleomorphism and an increased mitotic rate of up to 50 mitosis/50 high power field (HPF). The tumor exhibited focal necrosis and was infiltrating into the pericystic adipose tissue.μm sections were cut from the paraffin-embedded tissue block and treated with xylene/ethanol. The samples were digested overnight at 37°C using proteinase K. DNA was extracted with phenol-chloroform, precipitated in ice-cold ethanol, redissolved in distilled water, and quantitated using a Picodrop Microliter spectrophotometer.Ten μL of LightCycler LC480 High Resolution Melting Master (Roche), 3.5 mM MgCl2 and PCR-grade water adjusted to a total volume of 20 μL.For the detection of c-Kit exon 9, 11, 13, and 17 mutations as well as PDGFRA mutations in exons 12 and 18, a real-time polymerase chain reaction approach followed by high-resolution melting curve analysis has proved to be a rapid, highly sensitive, and efficient screening method. Subsequently, sequencing of the PCR products is applied for mutation identification. PCR and HRM were consecutively performed on a LightCycler 480  in one single run, and all reactions were repeated twice. Each reaction consisted of 20 ng of DNA, 200 nmol/L of each primer, 10 PCR products with an abnormal HRM pattern were sequenced using the Big Dye terminator cycle sequencing kit . The sequencing products were analysed on an ABI Prism 310 Genetic Analyzer (Applied Biosystems).The specimen was analysed for the presence of activating mutations in exons 9, 11, 13, and 17 of c-Kit gene as well as exons 12 and 18 of PDGFRA gene by performing High Resolution Melting Analysis and Sequencing.A mutation at codon 824 coding for valine in exon 18 of PDGFRA gene was detected by high-resolution melting analysis (HRM) and was further confirmed by sequencing as c. 2472 C>T, p. V824V . This siNo c-Kit mutations were found. Results were verified by a second independent PCR reaction and HRM analysis followed by sequencing.An estimated five-to-six thousand new cases of GIST are diagnosed annually with 10% to 30% of these being malignant . Most tuGISTS are diagnosed based on the morphological and immunohistological features. Histology can often be varied, but GISTs are broadly divided into spindle, epithelioid, or mixed cell types. In general, the risk of malignancy is greater in epithelioid tumors than in spindle-celled neoplasms , 12. WitWe present the case of a malignant GIST of the gallbladder which demonstrated spindle cell morphology and was PDGFRA positive and Kit negative. There have been a small number of gallbladder GISTs described in the literature, of which only four were malignant and all were Kit positive. To our knowledge this presents the first ever recorded presentation of such a GIST. The significance of the Kit negative genotype has implications on the response to further management. The identification of specific cellular markers has led to the development of effective targeted agents, namely, tyrosine kinase inhibitor (TKI) therapy . These have had dramatic effects in prolonging progression free survival in advanced unresectable disease . Most KiPrimary GISTs, as in the case here, have the potential for curative treatment with surgical resection. Overall 5-year survival rates for resectable GISTs have been shown to range from 46% to 78.5%; however, predicting the recurrence rate of primary resectable GISTs has been very challenging , 18. TheThis paper, presents a very unique case of GIST, located in the gallbladder, negative for Kit, and positive for PDGFRA. Apart from this being a previously undocumented case, it highlights the challenges in establishing the diagnosis, prognosis, and most effective management for this unpredictable tumour.


MIDD is characterized by the deposition of light and heavy chain proteins. Depending on the spatial arrangement of the secreted proteins, light chain-derived amyloidosis (AL) can be distinguished from non-amyloid light chain deposition disease (LCDD). We present a case of an extremely rare A 61-year-old woman presented with a 3½-year history of neurologic symptoms due to a progressive white matter lesion of the left subcortical parieto-insular lobe and basal ganglia. 2 former stereotactic biopsies conducted at different hospitals revealed no evidence of malignancy or inflammation; thus, no therapy had been initiated. After performing physiological and functional magnetic resonance imaging (MRI), the tumor was removed under intraoperative monitoring at our department. Histological analysis revealed large amorphous deposits and small islands of lymphoid cells.wait-and-see” strategy can delay possible therapy for this disease. The impact of surgical removal, radiotherapy and chemotherapy in LCDD obviously remains controversial because of the low number of relevant cases.LCCD is a very rare and obscure manifestation of primary central nervous system low-grade lymphomas that can be easily misdiagnosed by stereotactic biopsy sampling. If stereotactic biopsy does not reveal a definite result, a “ Primary central nervous system lymphomas (PCNSL) are defined as non-Hodgkin’s (NHL) lymphomas that primarily arise in the central nervous system . PCNSL aMonoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of monotypic light and/or heavy chain proteins in various tissues and organs. MIDD mainly affects the kidneys, but the involvement of other organs  is not uncommon . All forWe provide an update regarding the diagnosis and classification of primary cerebral low-grade B-cell lymphomas and cerebral light chain deposit diseases. We present the case of a patient with a 3½-year progressive hemiparesis and hemi-hypoesthesia of the right side due to a delayed diagnosis and therapy of the extremely rare, tumor-presenting cerebral restricted LCDD, which can be called “aggregoma” . We furtA 61-year-old woman was admitted to our department with progressive brachiofacially accentuated hemiparesis, dysdiadochokinesia and hemi-hypoesthesia of the right side of the body, which began 3½ years previous. She initially presented with dyspraxia and fluctuating hypoesthesia of the right hand at the end of 2006; her cranial nerves were not affected. She complained of increased fatigue but did not present with weight loss, night sweats, fever or headache. The woman had a history of hypothyroidism related to Hashimoto’s thyroiditis and suffered endocarditis and streptococcal sepsis in 1982. She was under a long-term medication treatment of 100 μg of thyroxin daily. An MRI scan performed in May 2007 revealed a 2.8 × 2.0 × 2.4 cm lesion of the white matter at the level of the left subcortical parieto-insular lobe and basal ganglia  under continuous intraoperative monitoring with sensory evoked and motor evoked potentials . The lesion appeared intraoperatively as an amorphous, gelatinous mass with some regions appearing dull and lucid Figure . The tumHistological examination of the resected tumor revealed large, amorphous, proteinaceous eosinophilic deposits and small islands of lymphoid cells between them Figure a. The amamyloidoma; regarding LCDD, the corresponding term aggregoma was suggested by Rostagno et al. in 2002. He first reported 3 patients who had tumoral masses of non-amyloid κ-light chain aggregates: 2 with cervical masses and 1 with a solitary lung nodule [Insoluble amyloid- or light chain-related non-amyloid aggregates can be formed by systemically secreted proteins, mainly involving the kidneys or occurring in an organ-specific manner . Differeg nodule . To dateg nodule -16. In ag nodule ,16. The g nodule . By contg nodule ,18 and 1g nodule . For detaggregoma. To create a better understanding of the relationships of the involved diseases they were classified in a pedigree , which protects the brain sufficiently from circulating harmful metabolites  . Neverthlusively . Hence, As in other neurological diseases, the clinical presentation of MIDD depends mainly on the location of protein deposition and not on the histological finding. The major clinical signs of LCDD in the brain are epileptic seizures, cognitive impairments, headaches, and in the case presented here, hemiparesis, all of which were the main neurological symptoms observed in intracerebral amyloidoma Table . Cerebra, Case I). Although Pantazis and colleagues also demonstrated stability of the disease by 20 months after chemotherapy with Rituximab , Trofosfamide (Ixoten™) and steroids, the control MRI new lesions after 16 months . The data suggest that chemotherapy can provide at least a temporary benefit for disease progression but is most likely not sufficient to cure the disease possibly because of the drugs that cannot enter the brain across the intact BBB in sufficient levels to exert their full effect. One would assume an even weaker effect on aggregomas because penetration of the drugs across the BBB into the space-occupying lesion is strongly impaired, as it was also demonstrated for ICA [Because both, the clinical presentation and imaging features of various cerebral disorders are not specific, histological analysis functions as a pivot point for further therapeutic strategy. Depending on the location of the disease, the distinction of adjacent healthy brain tissue and the involvement of eloquent areas, a straightforward surgical removal, or an open alternative stereotactical biopsy would be the methods of choice. For ICA, it is known that medical treatment is not successful, but surgical removal has a good prognosis if the tumor can be removed completely ,23. For 1 for ICA .MIDD is a very rare and obscure manifestation of primary central nervous system low-grade lymphoma. The disease is characterized by monoclonal production of immunoglobulins that can occur in an isolated manner in the brain due to the BBB. The case on tumoral aggregation of λ-light chain deposits presented here, confounds the former assumption that tumoral aggregation of light chains is restricted to κ-light chain amino acids. To date, only 53 cases of MIDD have been published, including 50 cases of cerebral-restricted light chain-derived amyloidosis and 3 cases of the even less frequent non-amyloid light chain deposition disease. In the case presented here, the lesion was located in an eloquent area of the brain, which necessitated 2 stereotactic biopsies, both of which were performed at other hospitals (one in June 2007 and the other in July 2008) and demonstrated no evidence of malignancy or inflammation. However, the patient showed continuous tumor progression and clinical deterioration. After resection, histopathological examination determined that most of the tumor consisted of large, amorphous, proteinaceous, eosinophilic deposits with small islands of lymphoid cells between the deposits. The lack of awareness of LCDD and its unique histological presentation contribute to the risk of misdiagnosis and underscore the need for a careful description of this disease and the limitations of biopsy. To date, the best therapeutic strategy for this disease remains controversial because the impact of surgical removal, radiotherapy and chemotherapy cannot be reliably evaluated due to the low number of cases thus far.“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 Series Editor of this journal”.The authors declare that they have no competing interests.MS composed the abstract, introduction and discussion, as well as the conclusion of the manuscript and created the illustration of the involved diseases. GP did the histological analysis, created the histological figures and wrote the histological section. SB created the MRI figures, did the MRI image analysis and wrote the MRI section. GF treated the patient on the ward, helped with the draft of the clinical case and performed the literature search. MS performed the surgical intervention, prepared the intraoperative picture and wrote the surgical section. MT did the surgical intervention, initiated the draft of the case report and helped with the design of the manuscript. RR participated in the design of the case report and coordinated and substantially helped with the draft of the key manuscript sections. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/13/107/prepubLCDD - case summary.Click here for file


Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life. CPMs are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.With improved resolution of foetal sonography and Doppler studies, many of these lesions are detected “in utero.” Their natural history can be quite variable. Lesions may resolve prior to birth. Some CPMs may present in the early neonatal period with respiratory distress due to a mass effect, while others may be asymptomatic only to be detected in later life on CXRs performed for other reasons. Certain forms of CPM have the potential to undergo malignant change. Conversely, pleuropulmonary blastoma may masquerade as some forms of CPM. When lesions are symptomatic in early life there can be no doubt that surgical resection is the only option. However, the decision-making process becomes more complex when clinicians encounter asymptomatic lesions, in healthy infants, which have been detected either by antenatal USS or incidental chest radiograph.The presentation, natural history, diagnosis and management of congenital pulmonary airway malformation, congenital lobar emphysema, pulmonary sequestration, bronchogenic cyst, and pleuropulmonary blastoma will be discussed.Congenital pulmonary airway malformation (CPAM) was previously known as congenital cystic adenomatoid malformation (CCAM). Histologically, the lesion of CPAM is characterised by solid adenomatous areas, which consist of closely packed tubular structures resembling terminal bronchioles without mature alveoli . These aThe incidence of CPAM is 1 per 8300 to 35,000 . Types 1 The natural history of CPAM can be quite variable. Spontaneous regression during the course of gestation is not uncommon. In 29 patients with CPAM, during fetal life, the size of the lesion decreased in 8 patients, of whom 7 required postnatal surgical resection, and disappeared in 4 patients, of whom 3 required postnatal surgical resection . In chilWith improved resolution of foetal sonography, many of the CPAM lesions are detected “in utero.” However appropriate postnatal investigations, rather than antenatal diagnosis, are essential for surgical decision . No corrn = 13), pulmonary sequestrations (n = 6), bronchogenic cysts (n = 3), congenital lobar emphysemas (n = 9), Shanmugam et al. reported that respiratory distress, respiratory infections/pneumonias, and dyspnoea occurred in 9, 22, and 9 out of these 31 patients, respectively [ CPAM may present in the early neonatal period with respiratory distress due to a mass effect, while others may be asymptomatic only to be detected in later life on CXRs performed for other reasons. Sauvat et al., reported that 3 out of 29 patients with CPAM experience symptoms during the first weeks of life . As manyectively . In 16 pectively . The diagnosis is suggested by CXR, however, a thoracic CT scan is required to confirm the diagnosis , 26. Win An association between type 1 CPAM and bronchoalveolar carcinoma has been reported . In patiIn 13 patients with predominantly microcystic CPAM, Curran et al. showed that fetuses, who had CPAM volume-to-head ratio >1.6 or nonimmune hydrops, experience an improvement following prenatal betamethasone . In thesAir travel in children suffering from cystic lung lesions is controversial because of the risk of pneumothorax. Most clinicians caution against air travel in children with enlarging cystic lesions. All infants with antenatally diagnosed CPAM should be evaluated. Symptomatic children require surgical resection . When CPBronchogenic cyst is a rare CPM, with a prevalence rate of 1 per 68,000 . BC repr BC may be detected as incidental findings on chest radiographs and account for 10% of mediastinal masses in children. The postnatal diagnosis of BC may be suspected on chest radiograph but a thoracic CT scan is needed to confirm the diagnosis . In a caA rare association between BC and neuroblastoma had been reported in 2 children . A recenSurgical excision should be considered in all cases because of the likelihood of eventual development of symptoms and malignant change .CLE is relatively rare affecting 1 in 20,000 live births . It occu Lesions may be asymptomatic or present with respiratory distress in the newborn period . Later, Type I pleurapulmonary blastoma may mimic CLE. In one report of a patient with asymptomatic CLE, the lesion was resected due to increasing size and pleuro-pulmonary blastoma type I was confirmed on histology . This ov Early surgical excision is required for newborns with respiratory distress , 51. HowBPS is a rare congenital malformation of the lower respiratory tract and comprises 0.15 to 6.4% of all CPM. The incident rate of pulmonary sequestration is 0.29% [ILS lesions account for 80% of sequestrations . The seqOther congenital anomalies occur in 3/18 and 3/4 of ILS and ELS, respectively . In 28 pIn a previous study of pregnant women with antenatally diagnosed ELS, the lesions regressed before delivery in 28/41 (68%) cases . PostnatThe age of presentation is variable in children with ELS. In a case series of 50 patients with ELS, 24% were diagnosed prenatally and 61% were diagnosed by three months of age . The adjThese include haemoptysis, haemothorax, and malignant transformation. Levine et al., documented severe congestive heart failure in 2 infants with BPS and large arterio-venous shunts . A localIn fetuses with BPS and hydrops, open fetal surgery, coagulation of the blood supply, or thoracoamniotic shunting has been recommended . BPS wit Immediate surgical intervention is required in patients with respiratory distress. In view of the high rate of complications, elective resection is often recommended even in asymptomatic patients with BPS . Ayed anIt is a rare tumor of pleura and lung in young children. The incidence of PPB is 1 in 250,000 live births . There aCongenital Lung malformations are becoming more frequently recognised with the advent of improved antenatal imaging. There is considerable debate among clinicians as to the best approach to these lesions in asymptomatic infants as many of the lesions have the potential to involute over time. Further longitudinal studies are needed to address the appropriate approach towards management for each of the CPM discussed.


Primary chondroid tumors of the larynx represent less than 1% of all laryngeal tumors. Most of them are chondromas and they often involve to the cricoid cartilage. A 31-year-old male patient applied to the oto-laryngology service with a history of dysphonia and dyspnea. Microlaryngoscopy revealed 2 cm sized, ill-defined, covered with regular mucosa, porous, and hard mass on posterior surface of crycoid cartilage in subglottic area. Following the excision of the lesion, histopathologic examination revealed as chondroma. Two years later, local recurrence was detected and the diagnosis was again chondroma. There was no complaint of the patient in last 3 and half years of follow-up. Chondroma should carefully be differentiated from chondrosarcoma and the patients should be followed for possible recurrences.  Cartilaginous tumors of larynx are very rare. Primary chondroid tumors of larynx are less than 1% of all laryngeal tumors . But theThe incidence of malignancy for chondroid tumors is higher in old patients. However chondromas were seen in 10-year younger patients than chondrosarcomas. Male : female ratio is 1 : 4 –11. AlthCartilaginous tumors of larynx are classified in 4 groups : (1) metA 31-years-old male patient was referred to the Chest Medicine Department with a history of dysphonia and dyspnea. Laryngeal subglottic mass was detected with bronchoscopic evaluation. The patient was consulted to the Department of Otolaryngology. 2 cm sized, ill-defined, porous, and hard mass on posterior surface of cricoid cartilage in subglottic area was detected in microlaryngoscopic evaluation. This mass was covered by regular mucosa. This lesion was scraped from posterior wall of cricoid cartilage with sickle scalpel. Mitomycine was applied to the lesion locally to prevent the development of postoperative scar. Clinically tracheostomy was not required and no complication was seen. He was discharged next day.Macroscopically total volume of 2 cc, 0.2–0.5 cm sized, fragmented, gray-pink colored, medium-hardness, homogenous materials were seen. Microscopically well-defined, lobulated-contoured mass was seen near of the focally calcified cartilaginous tissue . The tumThis patient was referred to the Department of Otolaryngology with stuck feeling in the throat after 2 years from first operation. 1 × 0.8 cm sized, hard, gray-coloured mass, which is covered by regular mucosa, was detected in same localization (right subglottic area). Second microlaryngoscopy was applied and the lesion was excised in november 2007. This lesion was diagnosed as “laryngeal chondroma” too. In routine follows-up no complaints and lesion were detected (42 months).Chondroid tumors of the larynx are uncommon neoplasms and they are comprised up to 1% of all laryngeal neoplasms. However they are most common mesenchymal tumors of larynx . Some stChondroid tumors of larynx can be easily recognized histologically, because of their characteristic features. But morphologic features must be well known for differential diagnosis between subtypes.Chondrometaplastic nodules are smaller than 1 cm sized and multiple. This lesion is characterized by loose of lobular pattern, mucochondroid changes in soft tissue matriks, and well-defined nodules –12, 15. Differential diagnosis between chondromas and chondrosarcomas can be difficult. Macroscopically chondromas are smaller than 2 cm (average 1.5 cm) but chondrosarcomas are generally larger than 3 cm sized (average 4.3 cm) . MicroscIncreased cellularity (more than 40 nuclei/HPF), nuclear atypia, binucleation, mitosis, and pleomorphism are variable in low- and high-grade chondrosarcomas. Our lesion was characterized by hypocellularity, no nuclear atypia, mitosis, binucleation, and pleomorphism. We diagnosed this lesion as “laryngeal chondroma” with these morphologic findings. Our patient referred to the Department of Otolaryngology with recurrent mass in same localization after 2 years. Microlaryngoscopic resection of lesion was diagnosed as “chondroma” too. Routine follows-up (42 months) of patient have been normal.Chondromas are rare mesenchymal tumors of larynx. They are benign lesions but incidence of locally recurrence is very high. Histopathologicly, differential diagnosis of laryngeal chondromas should be planned very carefully. Especially differential diagnosis between chondromas and low-grade chondrosarcomas is important for planning of treatment.Laryngeal chondroma was described in a few numbers of reports.We suggested that the differential diagnosis of chondromas must be done carefully and the follows-up of patients must be planned more frequently. Our case had typical clinical, histopathological, and prognostic features of laryngeal chondromas.


Extragenital malignant mixed Müllerian tumor is an extremely rare presentation of malignant mixed Müllerian tumor, especially when combined with a synchronous ovarian cancer.We report the clinical course and pathologic findings of a case of mesorectal malignant mixed Müllerian tumor with synchronous ovarian cancer, in a 50-year-old, gravida 0, para 0, Han Chinese woman with regular menstruation. This is the sixteenth case in the English literature of extragenital malignant mixed Müllerian tumor combined with synchronous or metachronous malignancy reported.Although extragenital malignant mixed Müllerian tumor is very rare and has a poor prognososis, a longer survival time might be achieved with treatment by cytoreductive surgery, radiotherapy and chemotherapy. Malignant mixed Müllerian tumor (MMMT) is an uncommon tumor in females and the occurrence of this disease outside the genital tract is extremely rare. In a review of the English literature since 1955, only 48 cases of extragenital MMMT have been reported other than the presented case. Sixteen out of these 49 (32.7%) extragenital MMMTs [The patient case was a 50-year-old, gravid 0, para 0 (G0P0), unmarried Han Chinese woman with regular menstruation. Six months ago, she visited another medical center in Southern Taiwan for abdominal bloating, where bilateral ovarian tumors were diagnosed. At laparotomy, a left ovarian cystic tumor (35 × 20 × 10 cm) and a right ovarian tumor (12 × 8.5 × 6 cm) with normal uterus and cervix were noted. An additional tumor of about 12 × 9 × 8 cm in size was also found in the mesorectum of the rectosigmoid colon. Resection of the mesorectum and bilateral oophorectomy was performed at the first operation at another medical center. The histopathology report revealed bilateral ovarian cancer (endometrioid adenocarcinoma) and malignant mixed Müllerian tumor from the mesorectum with biphasic differentiation (adenocarcinomatous and spindle cell sarcomatous elements). No heterologous element was identified. No further treatment was performed after the first time of operation. However, she felt progressive abdominal bloating and dysuria recently. She, therefore, visited the department of surgery of our hospital. On physical examination a lower abdominal mass was palpated. An abdominal computed tomography scan revealed a large low density mass in the pelvic cavity  and sarcoma  components, this disorder is also named carcinosarcoma. MMMT is classified into homologous or heterologous according to the sarcomatous component. Extragenital MMMT can occur at any site of peritoneum and is one type of primary peritoneal carcinomas (PPC) which was first described by Swerdlow in 1959 . It has et al. asserted that optimal cytoreduction could significantly improve the prognosis of patients [The majority of PPCs present in pathology as serous papillary carcinomas, as well as peritoneal mixed epithelial carcinomas, while the extragenital MMMTs are rarely reported. PPC is a rare cancer closely related to epithelial ovarian cancer and develops in cells from the lining of the pelvis and abdomen (peritoneum). These cells are similar to the cells on the surface of the ovaries. Like ovarian cancer, PPC tends to spread along the surface of the pelvis and abdomen. Symptoms of patients with PPC are similar to those with ovarian cancer, including abdominal pain or bloating, nausea, vomiting, indigestion and change in bowel habits. Women with PPC are usually treated similarly to those with widespread ovarian cancer. The therapeutic modalities include cytoreductive surgery as much as possible, followed by the same chemotherapy regimen administrated for ovarian cancer. Look patients . HoweverMost PPCs are serous papillary adenocarcinomas with a relatively good prognosis but the primary peritoneal MMMT, a rare type of PPC, usually has an unfavorable outcome according to the previous literature . MMMT ofLittle information about the management of extragenital MMMT is available. All suggestions for the treatment extragenital MMMT are based on individual cases. Treatments including cytoreductive surgery and chemotherapy have been reported. Surgical management is usually mandatory due to the clinical presentation caused by the mass effect. However, a radical surgical treatment is often obtained with difficulty. It seems that chemotherapy is more important than surgical treatment and the treatment choice of MMMT is similar to that of genital MMMT.et al. reported a patient with MMMT of the ovary who had a suboptimal response to single-agent cisplatin chemotherapy but who demonstrated a complete response with ifosfamide, mesna, Adriamycin (doxorubicin) and dacarbazine [There are several reports regarding platinum-based chemotherapy activity against MMMT of the ovary. Simon arbazine . Paclitaarbazine . The medet al. reported a patient who received postoperative radiotherapy and survived for eight months [et al. reported six cases of metastasized MMMTs receiving cytoreductive surgery plus intraperitoneal hyperthermic perfusion and adjuvant treatment of CDDP (cis-diamminedichloroplatinum), mitomycin and ifosfamide applied via intraaortic catheter [The role of radiotherapy remains controversial. When a patient presents with a grossly residual tumor, radiotherapy may be considered. Garamvoelgyi t months . Converscatheter . Four paet al.[A similar case of MMMT of mesenteric origin was reported by Ma et al.. The patet al. investigated the role of carcinomatous and sarcomatous components on the response to chemotherapy and disease outcome. It also observed that patients with a predominating carcinomatous component had a higher therapeutic response rate (87.5%) than those with a predominating sarcomatous component (66.6%) [Recently, it has been demonstrated that the presence of BRCA mutations may predispose to primary peritoneal cancers and this neoplasm could be a part of the hereditary breast and ovary cancer syndrome . Immunoh (66.6%) .Extragenital MMMT is extremely rare and has a poor prognosis due to its aggressive biological behavior. Synchronous or metachronous gynecologic tumors often exist and a detailed examination of the genital tract must be made before and during the operation. Moreover, improved survival times would probably be obtained if accurate diagnoses and aggressive treatment, including cytoreductive surgery and chemotherapy, are applied earlyMMMT: malignant mixed Müllerian tumor; PC: paclitaxe/carboplatin; PI: platinum/ifosfamide; PPC: primary peritoneal carcinomas.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CCH drafted the article. CJM analyzed and interpreted the patient data. WTH photographed and interpreted the pathologic findings. TFC took part in the critical revision. and JYW took part in the surgical approach and final approval of the manuscript. All authors have made substantive intellectual contributions to this study and to the manuscript and have read and approved the final manuscript.


Due to recent advances in imaging diagnostic techniques, there are an increasing number of case reports of segmental arterial mediolysis. However, there are only a limited number of reports on segmental arterial mediolysis-related abnormalities of abdominal organs other than the intestine. This report describes a case of segmental arterial mediolysis accompanied by abnormalities of abdominal organs without clinical symptoms.A 52-year-old Japanese man with hematuria and no prior medical history was referred to a urologist and was diagnosed as having urinary bladder cancer. He underwent trans-urethral resection of the bladder tumor and intra-vesical instillation therapy, which was followed by observation. During follow-up, although no abdominal symptoms were observed, an abdominal computed tomography scan revealed a dissection of the superior mesenteric artery. A false lumen partially occluded by a thrombus was located distal to this occlusion. The lumen was irregularly shaped with narrow and wide sections. Similar irregularities were also observed in the wall of the inferior mesenteric artery. Arterial dissection with thromboembolism in the left renal artery and renal infarction was also observed. Follow-up computed tomography after two months revealed an enlargement of the pancreatic tail adjacent to the splenic artery. Follow-up three-dimensional computed tomography showed gradual re-expansion of the true lumen of the superior mesenteric artery, improvement in arterial wall irregularities, and a reduction in the pancreas enlargement and renal infarction. Over the following 15 months, these changes gradually normalized. On the basis of the vascular changes in multiple arterial systems that resolved spontaneously, we considered that the lesions were associated with segmental arterial mediolysis.We present a rare case of segmental arterial mediolysis accompanied by abnormalities of abdominal organs without clinical symptoms. Three-dimensional computed tomography was useful for follow-up evaluation in our patient. Segmental arterial mediolysis (SAM) is a condition characterized by segmental lysis of the tunica media of muscular arteries and subsequent formation of aneurysms primarily in abdominal organs. It is an acute, non-inflammatory, non-arteriosclerotic degenerative disease, frequently associated with ruptured aneurysms and intra-abdominal hemorrhage requiring immediate surgery. An increasing number of cases with this condition have been reported in the literature . AlthougA 52-year-old Japanese man with no significant prior medical history and unknown smoking habits was referred to a urologist for investigation of hematuria, and was diagnosed as having urinary bladder cancer after further examination. He underwent three sessions of transurethral resection of the bladder tumor and intra-vesical instillation therapy  and was then placed under observation. During the follow-up period no abdominal symptoms were observed, and an abdominal computed tomography (CT) scan revealed the incidental finding of dissection of the superior mesenteric artery (SMA). Our patient was then referred to our hospital.His physical examination findings on admission were height of 176cm, weight of 64.5kg, blood pressure of 111/69mmHg, and pulse rate of 70bpm. Our patient had no fever and no other abnormal physical findings such as abdominal symptoms. His blood biochemistry test results were also normal. A contrast-enhanced abdominal CT scan revealed an arterial aneurysm and a dissection located approximately 1.5 and 5cm distal to the ostium of the SMA , pregnancy, and trauma. Of these, FMD has been the most extensively discussed. Lie iography . Howevercede SAM . FMD priM [et al.. In our While most reported cases of SAM involve ruptured aneurysms, lesions that do not rupture have hyperplasia of granulation tissue in the defect formed by mediolysis. At the same time, similar changes occur around the hematoma, which include a thrombus being formed and then organized, followed by absorption and shrinkage of the hematoma. While some reports describe cases in which an aneurysm resolved spontaneously after multiple angiography sessions , others Treatment selection in our patient’s case was based on previous case reports identified by a search using ‘SMA dissection’ as the keyword. This choice was based on the marked similarity of findings between SMA dissection and our patient’s case. These similarities included a lesion present between the root and proximal part of the SMA, formation of the aneurysm and uncommon rupture, and the presence of a false lumen that remained open, as occurs in about one-half of reported cases. The reported treatment options for SMA dissection include conservative treatment, anticoagulants, endovascular treatment, and surgical treatment, although there is no general agreement on these treatment options. In light of recent findings that suggested early introduction of anti-platelet therapy may reduce the need for surgery, we chose this course of treatment in our patient’s case. Although SMA dissection is associated closely with hypertension and smoking , it is cThe literature contains an interesting case in which all the veins running adjacent to the artery affected by SAM exhibited varying degrees of changes, such as edema, dissection, lysis and defects in the muscle layer, and hyperplasia of elastic fibers . The patIn our patient’s case, the middle colic artery, which is most closely associated with rupture and ischemia, was derived from the true lumen on the dorsal side of the dissected part. This may have contributed to the absence of clinical symptoms and the development of pancreatic enlargement as a secondary change around the affected artery. Three-dimensional CT was useful for follow-up evaluation  and showThe lesions observed in our patient’s case were considered to be associated with SAM as they included irregularities in arterial walls, and the development of an aneurysm and dissections in multiple arterial systems that resolved spontaneously. Our patient’s case constitutes a very rare example of SAM accompanied by organ involvement, including renal infarction and pancreatic enlargement. Three-dimensional CT was useful for follow-up evaluation in our patient.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NI was a major contributor in writing the manuscript. GK, YS and HT contributed to the conception and interpretation of the disease on the basis of several articles. All authors read and approved the final manuscript.


A nontraumatic spontaneous extradural hematoma, in a fully conscious 10-year-old male child, caused by a solitary eosinophilic granuloma of calvarium presented as a case of localized painful swelling of the head, which rapidly expanded and decreased in size. A plain CT-scan of the head with bone window revealed eroded right parietal bone with subperiosteal debris and extradural hematoma of mixed density. Immediate evacuation of the extradural clot and complete excision of the lesion was performed to prevent the deterioration of the patient and to achieve the histological diagnosis for further management. Extradural hematomas of nontraumatic and pathological origin have been reported occasionally for the last six decades.2 There hA 10-year-old, fully conscious, boy presented to the accident and emergency room of Neurosurgical Centre, Sher -i- Kashmir Institute of Medical Sciences (SKIMS), Kashmir, with one month history of a gradually appearing almond-sized, swelling on the right side of the head with dull aching pain. The swelling suddenly increased to the size of a walnut with increase in the intensity of pain for last 15 days. However, for the last one week, the swelling rapidly decreased in size but became pulsatile and at the same time pain continued unabated. There was no history of trauma, episodes of unconsciousness, headache, vomiting, drug intake, fever, hemoptysis, hematuria or abdominal pain. Clinically, the patient was fully conscious and intelligent too. General physical examination was normal. Locally a single, 3 × 3 × 4 cm, diffuse, pulsatile, soft and compressible but tender swelling was found on the right parietal region . The temThe spontaneous, nontraumatic or pathological extradural hematomas are uncommon. Their presentation depends upon the nature of underlying lesion, pain and tenderness locally, swelling of the scalp or rise in the intracranial pressure. The eosinophilic granulomas (a type of histiocytosis X) are solitary, nonrecurring and moderately vascular lesions of the skull found in older children and young adults. Local tenderness and pain usually call attention to the lesion. It is rare for these lesions to bleed extradurally.–11 At SK512631419


Liposarcomas are the most common of sarcoma tumours, they are usually located in the lower limbs, retroperitoneum, or abdominal cavity; up to date, only a few cases of omental liposarcoma with different histotype have been described. We present a case of omental well-differentiated liposarcoma and discuss imaging findings on ultrasound, computed tomography, and magnetic resonance to differentiate omental liposarcomas from other abdominal tumour entities. Primary tumours of the omentum are very rare, we don't know their incidence and most of the information available is from case reports. Liposarcomas usually involve the limbs, retroperitoneum, or abdominal cavity in adults . Up to dWe present a case of omental well-differentiated liposarcoma and discuss imaging findings on ultrasound (US), computed tomography (CT), and magnetic resonance (MR) scans.A 34-year old man suffering from autism presented a 2-year history of worsening abdominal distension. Physical examination revealed a large tender abdominal mass across the midline in the middle and lower quadrants. Laboratory tests were normal. Abdominal US (Philips ATL HDI-5000 system) revealed a hypomesogastric mass, measuring 18 × 13 cm in diameters, characterized by two components: a main hyper-echoic solid portion; and, superiorly, a second portion formed by multiple cystoid cavities  and intensity (MR) similar to those found in muscles and may show enhancement by gadolinium-contrast MR imaging. Some authors found focal areas with a signal different from fat tissue both in well-differentiated liposarcomas and benign lipomas . These fMyxoid liposarcomas show a dishomogeneous aspect with CT attenuation values lower than muscular tissue. Fat and soft tissues distribution pattern may sometimes produce fluid attenuation values, therefore the lesion appears cystic on scans without contrast medium misleading diagnosis. MR images reveals a signal intensity similar to water; however it is sometimes possible to observe indented, linear, or amorphous areas with increased signal intensity on T1- and mean signal intensity on T2-weighted images. These features indicates the presence of intratumoral fat and allow the correct diagnosis. Furthermore, these lesions are better defined after intravenous contrast medium injection showing a slow progressive and reticular enhancement, which indicates the solid nature of the tumour.The diagnosis of a dedifferentiated liposarcoma should be considered when an abdominal mass shows on CT and MR imaging intense enhancement, adjacent organs invasion, vascular infiltration, calcification or ossification, and areas of necrosis or haemorrhage , 9.In our case, we observed a liposarcoma with two components: a solid area with fat signal intensity and a second multicystoid portion. Fat suppression sequences confirmed the presence of adipose tissue in the former area and the cystic appearance of the latter. No foci with signal intensity different from fat were detected; CT and MR scans didn't show any significant variation after intravenous contrast medium injection. There was no evidence of other organs or vascular invasion. These features were consistent with a well-differentiated lipasarcoma which was then confirmed by pathological examination.In conclusion, omental well-differentiated liposarcomas are extremely rare and differential diagnosis with other abdominal tumour entities can be challenging. We stress the importance of fat suppression sequences and gadolinium administration on MR scan to identify abdominal masses of difficult diagnosis.


We describe a case of ovarian carcinosarcoma occurring in a 60-year-old female. The neoplasm was excised after neoadjuvant chemotherapy and contained a predominant heterologous pleomorphic rhabdomyosarcomatous component in which there were numerous multivacuolated rhabdomyoblasts that strongly mimicked lipoblasts. The clear cell variant of rhabdomyosarcoma is rarely documented, but this case shows a highly unusual finding in which the rhabdomyoblasts show the prominent multivacuolation with nuclear indentation characteristic of and indistinguishable from pleomorphic lipoblasts. This appears to represent a posttreatment phenomenon. As this finding might conceivably occur in other rhabdomyosarcomas after chemotherapy, we highlight the potential for diagnostic confusion with pleomorphic liposarcoma, which is usually diagnosed by morphology so that immunohistochemistry for muscle markers might not be performed. Carcinosarcomas of the gynaecological tract  are neoplasms composed of malignant epithelial and mesenchymal components that most frequently arise in the uterus but also occur at a variety of other sites in the genital tract. Heterologous mesenchymal differentiation can occur toward a variety of lineages, including to rhabdomyosarcoma, chondrosarcoma and osteosarcoma, and more rarely liposarcoma. Here, we present a case of ovarian carcinosarcoma with heterologous pleomorphic rhabdomyosarcomatous differentiation, which was excised after neoadjuvant chemotherapy and showed a morphologically striking pattern of lipoblast-like rhabdomyoblasts. This is in keeping with an unusual posttreatment degenerative change, and we highlight its potential for diagnostic error as pleomorphic liposarcoma.A 60-year-old female presented acutely with fever, abdominal pain, and a palpable mass after several months' history of mild abdominal discomfort and bloating. She was previously fit and healthy, without significant past medical or family history. Computed tomography (CT) and magnetic resonance imaging (MRI) scans showed complex solid cystic bilateral adnexal masses measuring up to 11 cm and consistent with malignant ovarian tumor, with bulky pelvic and paraaortic lymphadenopathy. No other disease foci were noted. CA125 was raised at over 2000 IU/mL, but CA153, CA19-9, CEA, AFP, and BHCG were all within normal range. She was treated with intravenous antibiotics, and the pelvic lesion was biopsied. Needle core biopsy showed high grade serous adenocarcinoma, in keeping with either primary peritoneal, tubal, or ovarian origin. The patient was commenced on 3 cycles of neoadjuvant carboplatin and paclitaxel, after which CT scan showed partial response with reduction of tumor size (particularly of the solid component) from 11 cm to 8 cm and reduction in size of abdominopelvic nodes. CA125 fell from 2,191 to 194 IU/mL. Approximately 12 weeks after initial presentation, the patient proceeded to primary ovarian debulking surgery, involving total abdominal hysterectomy and bilateral salpingoophorectomy (TAH BSO), bilateral ureterolysis, appendicectomy, left sided pelvic lymphadenectomy, paraaortic lymphadenectomy, and omentectomy.Immunohistochemical staining  was performed on formalin-fixed, paraffin-embedded (FFPE) tumor tissue using a panel of commercial antibodies .Gross examination of the TAH BSO specimen showed a 110 mm × 50 mm × 30 mm uterus with normal serosal surface, with unremarkable right ovary and fallopian tube. The left ovary was replaced by a lobular 110 × 90 × 70 mm and 80 × 60 × 50 mm “dumb-bell” shaped solid and cystic mass, with firm white or extensive yellow necrotic cut surface, with the left fallopian tube stretched over its surface. The cervix and endometrial cavity were unremarkable. The appendix tip was attached to the outer surface of the tumor mass, but the appendix was normal.Histologically, the left ovarian mass showed almost complete ovarian effacement by a cellular malignant neoplasm with two components. The smaller component consisted of markedly atypical epithelial cells in glandular formations and trabeculae , with foThe predominant component (accounting for at least 75% of the tumor) Figures  compriseThe neoplasm was therefore diagnosed as carcinosarcoma with a predominant pleomorphic rhabdomyosarcomatous component and smaller high grade serous adenocarcinomatous component. Metastatic carcinoma was also present in the left pelvic and paraaortic lymph nodes. The appendix tip was adherent to the left ovarian surface by a fibrotic adhesion but was otherwise unremarkable, as were the left and right fallopian tubes. The right ovary showed hyalinization and small granulomas consistent with site of regressed tumor after therapy. The patient was discharged uneventfully and three months later completed six cycles of carboplatin and paclitaxel after which CT scan showed only small volume pelvic lymphadenopathy. CA125 was within normal range.Clear cell change is an uncommon finding in rhabdomyosarcoma and can be seen in all subtypes, including alveolar (and its solid variant) , embryonCarcinosarcomas of the genital tract (MMMT) are clinically aggressive neoplasms that predominantly occur in older, postmenopausal women aged >65 years. Most arise in the uterine corpus, where they account for <5% of uterine malignancies, although other gynaecological sites include the cervix, ovaries or fallopian tubes, vagina, and peritoneum. They are associated with chronic estrogenic stimulation, although a proportion occurs secondary to previous irradiation. These tend to be large, bulky neoplasms that have often spread beyond the primary organ at the time of presentation. Stage  is the most consistent prognostic factor.Histologically, carcinosarcomas are biphasic neoplasms that contain both malignant epithelial and mesenchymal components. These occur in varying proportions and occasionally one or the other component is virtually exclusive. They are morphologically heterogeneous with a huge spectrum of histological appearances. The carcinomatous elements are usually poorly differentiated, and the most common types are serous, high grade (not otherwise specified), and endometrioid carcinomas, but more rarely squamous, mucinous, and clear cell types occur, and all these can be present singly or in combination. The carcinomatous components are positive for cytokeratins and EMA.The malignant mesenchymal component can be histologically homologous or, in up to half of cases, heterologous, although this distinction has not been shown to be of prognostic significance. When homologous, these are usually nonspecific spindle cell sarcomas or can resemble fibrosarcoma, undifferentiated pleomorphic sarcoma or leiomyosarcoma. The most common types of heterologous differentiation are rhabdomyosarcomatous, chondrosarcomatous, or osteosarcomatous. Heterologous liposarcomatous differentiation is more rare, both in genital carcinosarcomas and those at other sites such as lung  and bladThe diffuse desmin and strong nuclear myogenin seen within the multivacuolated lipoblast-like cells of this case are a clear indicator that they are rhabdomyoblasts. S100 protein can also be expressed in both normal skeletal muscle and rhabdomyosarcoma as well as in lipoblasts, so that with positive S100 protein immunohistochemistry the diagnosis of rhabdomyosarcoma can be missed. A crucial diagnostic pitfall is that these multivacuolated cells could have been assumed to be lipoblasts on morphology alone, such that the diagnosing pathologist might neglect to perform immunohistochemistry on this part of the neoplasm, particularly in view of the absence of specific immunohistochemical markers for lipoblastic differentiation. This highlights the importance of performing a full immunohistochemical panel, including myoid markers, when working up a case of carcinosarcoma.The presence of rhabdomyoblasts and of cells with morphology intermediate between rhabdomyoblasts and the multivacuolated lipoblast-like cells suggests that the neoadjuvant chemotherapeutic protocol has strongly induced differentiation in the rhabdomyosarcomatous component of the carcinosarcoma . This would fit with the low mitotic index, which would be incongruous with pretreatment, highly proliferating rhabdomyosarcoma. Therapy related changes described in rhabdomyosarcomas are cellular maturation , reducedA point of importance is that the initial biopsy had sampled only the epithelial component of the neoplasm, and the biopsy diagnosis had been of high grade serous adenocarcinoma of gynaecological origin, for which the patient was given neoadjuvant treatment. The rhabdomyosarcomatous component was not diagnosed until it was excised at debulking surgery, after initial chemotherapy. In most instances of rhabdomyosarcoma excised postchemotherapy the diagnosis is usually established, and hence if multivacuolation or other unusual posttreatment changes occur they might be more likely to be overlooked or remain undocumented, in contrast to this case where the type of mesenchymal differentiation was actively sought.The type of heterologous differentiation in MMMT has not been yet shown to be of prognostic significance and it might be argued that there is little practical importance in distinguishing rhabdomyoblasts from pleomorphic lipoblasts. However, the accurate classification of heterologous sarcomatous subtype may be of benefit once better knowledge of individual tumorigenic pathways is established, so that targeted therapies might be used to shrink specific sarcomatous elements, particularly when they predominate.In summary, we describe an unusual finding of multivacuolated rhabdomyoblasts morphologically mimicking pleomorphic lipoblasts, in a case of postchemotherapy ovarian carcinosarcoma. This is a distinctive pattern that appears to represent a degenerative, posttreatment phenomenon, but might cause diagnostic confusion due to its strong resemblance to pleomorphic liposarcoma.


A myoepithelial hamartoma is a very uncommon submucosal tumor of the stomach. In an atypical presentation in our case, it mimicked the clinical presentation of a gastrointestinal stromal tumor. To the best of our knowledge, it is the first case of a hamartoma of the stomach reported from Bangladesh and one of few cases described in the literature.We describe the case of a 35-year-old Bengali man with recurrent epigastric pain and occasional vomiting with radiographic findings of a gut mass. An upper gastrointestinal endoscopy revealed a healed duodenal ulcer, deformed ‘D’ bulb and a submucosal swelling in his antrum. Ultrasonography and a contrast-enhanced computed tomography scan confirmed the presence of a well-defined, oval gut mass in his upper abdomen, compressing his duodenum. The mass had a mixed density and was considered to probably be a gastrointestinal stromal tumor. Ultrasonography-guided fine needle aspiration cytology was inconclusive. After resection at laparotomy, a histopathological examination revealed a myoepithelial hamartoma. These tumors are characterized by hypertrophic smooth muscle bands surrounding varied epithelial elements, which may be arranged in diverse patterns such as simple glandular structure, Brunner’s gland, pancreatic ducts and sometimes pancreatic acini. This case report is complemented by a literature review relating to the atypical presentation.Gut masses need to be investigated thoroughly and the possibility of rare tumors should not be excluded. Although the recommended treatment for such lesions is limited resection, radical procedures such as a pancreaticoduodenectomy are often performed when the lesion occurs in the periampullary area because of preoperative misdiagnosis as a carcinoma. Therefore, it is essential for clinicians to maintain current knowledge of the lesion to avoid inaccurate diagnosis and prevent unnecessary surgery. Hamartomas are relatively common in the gastrointestinal tract; the preponderant tissues they contain are connective tissue derivatives including lamina propria, smooth muscle, vasoformative tissue, and nerve elements, though epithelial tissues also occur . A myoep33 cases . AccordiHere, we report a case of a myoepithelial hamartoma of the stomach where gastric endoscopy had revealed a healed duodenal ulcer and a submucosal swelling in the antrum, and a contrast-enhanced computed tomography (CT) scan had shown the presence of a gut mass that resembled a gastrointestinal stromal tumor (GIST).A 35-year-old Bengali man presented with complaints of intermittent pain in his epigastric region with occasional vomiting for two years. Over the last year, our patient had several episodes of heartburn followed by vomiting. The episodes lasted for a couple of days and occurred at an interval of two to three months. He denied any other symptoms. He was previously prescribed anti-ulcerant medication, which relieved him of his symptoms for a brief period of time. A physical examination was unremarkable.In view of his history and clinical presentation, our patient was referred for an upper gastrointestinal endoscopy with the clinical diagnosis of peptic ulcer disease. Upper gastrointestinal endoscopy revealed duodenal ulcer disease in remission and a deformed bulb of the duodenum. An endoscopy 14 months before admission had shown erosions and a submucosal swelling at the antrum and an ulcer at the bulb of the duodenum, and an endoscopy made eight months before admission had shown a healed duodenal ulcer and partial narrowing of the channel. In view of his repeated intermittent abdominal pain, a barium meal follow-through of the stomach and duodenum was carried out. This showed the bulb of the duodenum to be deformed, with a smooth filling defect with eccentric linear passage of contrast in part of the duodenum Figure  and 3. TA diagnosis of GIST in the first part of duodenum was made.A xifo-umbilical median laparotomy was made; intraoperative findings confirmed the radiographic findings. In light of these findings, a subtotal gastric resection was performed, removing more than half of the stomach, followed by gastrojejunal anastomosis. Macroscopic examination revealed a lesion in the antrum extending 8cm along its longitudinal axis Figure . The cutThe postoperative period was unremarkable and our patient’s general conditions were optimal at discharge two weeks after surgery. Our patient was followed-up at two months and six months after discharge with a complete physical examination and ultrasonography of his whole abdomen, and had no recurrence of symptoms.et al.[A myoepithelial hamartoma of the stomach is a rare tumor. It is also referred to as myoglandular hamartoma, adenomyomatous hamartoma, gastric adenomyoma and heterotopia ,6. Lesioet al. suggesteet al. reportedAccording to our research, 39 cases have been reported so far in the literature, not including ours . In thesLesions like these have been recorded in the gastric antrum 85%) and in the pylorus (15%) . They us% and in et al.[et al.[Our case simulated a GIST, presenting on CT as an oval-shaped mass in his upper abdomen, compressing his duodenum. Endoscopy does not produce any useful biopsy for histology because most of these lesions, including the one in our case, are localized in the submucosal layer. Vandelli et al. had attel.[et al. for surgl.[et al.. In casel.[et al.. WheneveGut masses warrant a thorough investigation and the presence of rare tumors should be excluded. Hamartomas can only be diagnosed by endoscopy and histological evaluation. The authoritative opinion is that a hamartoma should always be treated surgically, to avoid possible malignant degeneration or, as in rare instances, intestinal obstruction. The complete removal of the hamartoma can give complete remission of symptoms. Clinically, it is difficult to differentiate these lesions from a carcinoma, especially when they occur in the Vaterian system, often leading to a needless pancreaticoduodenectomy. To avoid unnecessary radical surgery, it is essential for clinicians to maintain current knowledge of the lesion and the most effective modality of treatment.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JN and FNA analyzed and interpreted the patient’s clinical and radiological data and prepared the manuscript. JN performed the histopathological examination, prepared the pathology slides, and reviewed the manuscript. QA reviewed the manuscript and gave final input prior to publication. JN wrote the manuscript and prepared the radiology figures. All authors read and approved the final manuscript.