Cavernous hemangiomas are endothelial tumors that rarely affect the adrenal glands. Most of these tumors remain silent and are incidentally found on abdominal imaging. Hardly ever, these tumors are endocrinologically functional. They may present as vague abdominal pain. Surgical resection remains the mainstay for large masses. In this paper, we are presenting a case of adrenal cavernous hemangioma in a 83-year-old male patient who initially presented for workup of vague abdominal and bilateral flank pain. A computed tomography scan of the abdomen showed an 8 cm right adrenal adenoma which was metabolically nonfunctional. The mass was completely resected through an open subcostal incision, with no encountered postoperative complications. A highlight of all published cases of adrenal hemangiomas since 1955 is also presented and reviewed. Incidental adrenal masses are a growing concern, especially with the significant increase in their detection upon the many abdominal imaging modalities utilized for the workup of various patient's complaints. The prevalence of incidental adrenal masses approaches 7% in the general population . AdrenalA 83-year-old previously healthy male presented with vague abdominal and bilateral flank pain of several months duration. The pain was dull in nature, with no recent change in weight and appetite, no reported hematuria, no gastrointestinal symptoms, and no reported episodes of headache. All basic blood work up, including complete blood count, creatinine, electrolytes, and liver function tests, were within normal range. An initial imaging with an enhanced computed tomography (CT) scan of the abdomen and pelvis was performed, revealing a right supra renal mass measuring around 8 cm in greatest dimension, possessing a significant enhancement, with a Hounsfield Unit (HU) of 15 on noncontrast phase and a 55HU on the contrast phase. No other abdominal or pelvic findings were noted.For better characterization of such adrenal lesion, a Magnetic Resonance Imaging (MRI) of the abdomen with gadolinium was requested. Again showing was a mass in the right suprarenal space, measuring 7.3 × 6.5 × 6 cm, showing heterogeneous signal intensity on all sequences, predominately mildly increased on T2-weighted and predominately decreased signal on T1-weighted images, and no appreciable signal drop on the out-of-phase images. The lesion appeared inseparable from the lateral limb of the right adrenal gland and remained separate from the upper edge cortex of the right kidney .Imaging was also accompanied by a full metabolic work-up, as usually performed for any incidentally discovered large adrenal mass, including: urine and plasma metanephrines, dexamethasone suppression test, DHEA-S, and aldosterone/renin ratio, and all yielded results were within normal range.Due to the size of the tumor, a shared decision was made to surgically remove the adrenal gland due to a potential risk of being an adrenocortical carcinoma. The patient therefore underwent a right radical adrenalectomy through an open subcostal incision. Intraoperatively, the adrenal was noticeably friable and tended to bleed on minimal manipulation, which was expected from the preoperative imaging done . No signOn gross pathological examination, the specimen measured 8 × 7 × 3 cm and the tumor was shiny tan-yellow, in nature with distinct demarcation from the normal adrenal parenchyma. Histologically, the tumor was proven to be a hemorrhagic cavernous hemangioma .Patient recovered well after his operation with no complications encountered thereafter. He was discharged home on his fourth postoperative day, to follow-up in clinic one month from discharge.Cavernous hemangiomas are unusual tumors of the endothelial linings with a propensity for skin, liver, and brain involvement . CavernoAdrenal hemangiomas are one of the rarest nonfunctioning benign adrenal tumors that are commonly diagnosed postoperatively . AlthougSixty-six cases of adrenal cavernous hemangiomas were published between the years 1955 and 2018 , and ideOn imaging, 32 adrenal masses were associated with speckled calcification, a historically described finding in any adrenal hemangioma; and 29 cases failed to show calcifications. Sixty-five of the reported cases were managed surgically; out of them, 47 were excised through an open approach, and the remaining 16 cases were excised laparoscopically.Most of the cavernous hemangiomas reported in the literature are incidental findings on imaging performed for unrelated or unspecific complaints . These tHistopathologically, adrenal hemangiomas are stratified into two subtypes: cavernous and capillary. The cavernous subtype is composed of an enlarged mass of blood filled endothelially-lined sinusoids, displacing potentially normal tissues. Whereas in the rarer capillary subtype, it is composed of small tufts of submucosal capillaries arranged in radiating loops or lobules .Historically, adrenal hemangiomas were usually identified on plain abdominal radiographies for unrelated complaints. On radiographs, these neoplasms appear as calcified masses. Calcifications, if present, are universally speckled through the entire mass as opposed to the curvilinear calcifications usually associated with adrenal pseudocysts . ComputeAlthough not required for routine diagnostic workup of any adrenal masses, angiography on adrenal hemangiomas can reveal marked neovascularity with small vascular channels, usually arranged in a rim-like manner which retain contrast in delayed films . These aAfter identification of adrenal masses on imaging, the common practice necessitated a full hormonal and metabolic workup to rule out primary functioning adrenal neoplasms mainly pheochromocytomas. Most cases of adrenal cavernous hemangiomas are nonsecretory and hormonally silent neoplasms . Due to Tumors originating from vessels could be associated with syndromes, but these are rather neonatal tumors, and not acquired tumors such as our present case. Nevertheless, there has been a previous single report of an adrenal cavernous hemangioma associated with familial adenomatous polyposis .Most adrenal hemangiomas reported in the literature were managed surgically . AsymptoAdrenal cavernous hemangioma is a rare entity that might be encountered when dealing with an adrenal pathology. Surgical resection is sometimes necessary to rule out any malignant potential and alleviate symptoms secondary to mass effect. Retroperitoneal bleeding is a concern in such pathology, especially when large lesions are detected. Observation is an alternative in cases where lesions are small, asymptomatic, and metabolically inactive, especially when confirmed by biopsy.


Renal ectopia is a relatively rare situation which may complicate the diagnosis. In cases with renal ectopia, the normal route of the urinary tract alters and may be associated with increased incidence of infections or coexist with other malformations. We report a female patient with unobstructed pelvic renal ectopia complicated by urinary tract infection. After presenting our diagnostic and therapeutic dilemmas and strategy, we discuss the existing diagnostic and treating options including conservative and surgical approaches. The actual incidence varies among several studies from 1:500 to 1:1200 with an average occurrence of 1:900 with no significant difference between sexes.2A 22‐year‐old female, sexually active, patient referred to our department with fever and abdominal pain. The patient referred an obscure history of recurrent urinary tract infections. Palpation during clinical examination revealed acute pain of the left flank reflecting to the unilateral abdominal region. The patient presented high fever up to 39°C. Patient's history included left renal pelvic ectopia, and autoimmune hepatitis, which both diagnosed 8 years ago. During all this period, prezolon 10 mg x2 and azathioprine 50 x3 were administrated systematically. Leucocytosis with left turn of the type with predominance of neutrophils was the main finding in blood examinations. The urine analysis revealed increased white blood cells in the sample (100‐120 ps). Further examination with abdominal ultrasound and CT scan revealed an hypoplastic left pelvic kidney without hydronephrosis but with multiple abscesses in its upper pole Figures .3Ectopic kidney can be found in various sites including pelvis, iliac fossa, abdomen, thoracic cavity, and in some others more rarely. In our case, the ectopic kidney had an ipsilateral retroperitoneal location in pelvis. The pelvic kidney was located opposite to the sacrum below to aortic bifurcation. Autopsy studies reveal no significant difference between sexes. Several clinical studies suggest that renal ectopia is more often diagnosed in females. This is mainly due to the fact that women are submitted to detail uroradiologic examinations for urinary tract infections or associated genital anomalies more frequently.Pelvic ectopia's incidence has been estimated to occur in 1:2100 to 3000 autopsies while a small predominance in the left side.Ectopic kidneys are usually smaller than normal. The renal pelvis is usually anterior to the parenchyma because it has incompletely rotated. As a result, 56% of ectopic kidneys have a distended collecting system (hydronephrosis). Half of these cases result from obstructive at either at the ureteropelvic or the ureterovesical junction (70% and 30% respectively), 25% from reflux and 26% from malrotation alone.The majority of ectopic kidneys are clinically asymptomatic. Intermittent vague abdominal pain, ureteral colic due to obstruction, and urinary tract infections are the most frequent symptoms that may contribute to the correct diagnosis. Diagnostic algorithm includes ultrasonography, excretory urography, CT scan, radionuclide scanning, and retrograde pyelography. Arteriography may be helpful in defining the vascular supply in cases that demand surgical intervention. The ectopic kidney is not more susceptible to disease compared to a normal one except for development of hydronephrosis, calculus formation, and urinary tract infection.4Although the present case does not add something new in the existing knowledge and practice posses significant educational interest and aims to point out some general practical principles. The clinical key message is included in the following sentences. Ectopic kidneys are usually asymptomatic. Intermittent vague abdominal pain, ureteral colic, and urinary tract infections may present nonspecific symptoms and set the suspicion. Imaging examinations are essential to the correct and accurate diagnosis. Treatment is usually conservative, and only in complicated cases, surgerical intervention is indicated.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.The authors declare no conflict of interest.SM, SA, and DF: contributed in patient's treatment, collected the material and the references, wrote and approved the manuscript. DT, IK, and PS: wrote and approved the manuscript.


The oncological safety of a laparoscopic approach for solid pseudopapillary tumors (SPTs) of the pancreas remains a matter of debate. We present the long-term follow-up of an adolescent girl with an SPT in the pancreatic tail. A multimodality workup including magnetic resonance imaging (MRI) revealed a complex, spherical mass of 4.4 cm × 3.6 cm × 4 cm most likely located in the pancreatic tail. All routine laboratory investigations and tumor markers were within normal limits . Diagnostic laparoscopy was performed to verify the origin of the tumor in the pancreatic tail. In a three-port technique the tumor was mobilized of the splenic vessels until a distal pancreatectomy could be completed. Histopathological examination confirmed the complete resection of a low-grade malignant SPT. The postoperative course was unremarkable. Regular pediatric oncological follow-up examinations for 3 years, including MRI every 6 months, ruled out recurrence and confirmed preservation of splenic and pancreatic functions. While data about the technical feasibility of a laparoscopic approach to pancreatic SPT are already available, this pediatric case report adds a long-term oncological and functional success to the available literature. Solid pseudopapillary tumors (SPTs) of the pancreas account for 8 to 17% of all pediatric pancreatic neoplasms.The ideal surgical approach mainly depends on the localization of the tumor. Lesions in the pancreatic body or the tail are usually treated by distal pancreatectomy. The technical feasibility of laparoscopic spleen-preserving distal pancreatectomy for Frantz tumors was first described in 1996.Herein, we present the case of a female adolescent patient with a SPT which was treated laparoscopically with spleen-preserving distal pancreatectomy. Three years of follow-up showed neither recurrences nor signs of metastasis.A 17-year-old girl was presented to a regional hospital with abdominal pain for several months without emesis or weight loss. Abdominal ultrasonography (US) was performed and revealed a tumorous lesion in the upper abdomen giving the indication for referral to our department.Upon clinical examination of the patient , there was slight tenderness in the left upper quadrant without a palpable mass. US was repeated and showed a complex, spherical mass of 4.4 cm × 3.6 cm × 4 cm in diameter with partly complex cystic and solid components. The mass was located between the abdominal wall, the medial margin of the spleen, and the anterior margin of the kidney. Magnetic resonance imaging (MRI) showed a well encapsulated mass at the tail of the pancreas which presented with partially cystic and partially solid areas with contrast enhancement at the periphery of the mass but not in the central cystic part. The lesion was in close relation to the splenic vein superiorly, the medial surface of the spleen laterally, the transverse colon and mesocolon anteriorly, and in front of the left kidney and suprarenal gland. Neither vascular invasions nor lymphadenopathy were detected . RoutinDue to the radiological findings and the negative tumor markers, a solid pseudopapillary tumor of the pancreas was suspected. The decision was taken to proceed with laparoscopy. With the patient in supine position, one 10 mm umbilical trocar and two additional 5 mm trocars in the left- and right-upper abdomen were placed. Laparoscopic distal pancreatectomy with preservation of the spleen was done using a 30-degree camera . DissecHistopathological examination revealed a completely resected pseudopapillary tumor of the pancreas with 5.5 cm in diameter. The proliferation fraction (Ki67 staining) within the tumor tissue was below 5%. Immunohistochemical examination was negative for keratin, chromogranin, CEA, and S-100 and positive for (neuron specific enolase) NSE, β-catenin, and α-1 antitrypsin. Additionally, single cells were positive for vimentin und synaptophysin.The postoperative course was uneventful except for a temporary peripancreatic fluid collection managed conservatively. Repeated ultrasound examinations were performed until complete resolution without pseudopancreatic cyst formation was seen 7 months postoperatively.Sonographic and clinical follow-up examinations, every 3 months during the first year and every 6 months thereafter, were unremarkable. In addition, MRI was performed twice a year in the first 2 postoperative years and at the end of the third year without evidence of a residual tumor or a recurrence. Moreover, body weight, as well as endocrine and exocrine function of the pancreas, was normal 3 years following removal of the tumor.SPTs, also termed as Frantz tumors, are rare low-grade malignant pancreatic tumors that typically occur in females aged between 20 and 40 years.Due to the rarity of these lesions, reports describing SPT of the pancreas only including pediatric patients are confined to case reports or case series.The clinical presentation is variable and ranges between asymptomatic incidental discovery, abdominal discomfort and pain, palpable mass, nausea, vomiting, and weight loss.The tumor can arise from any part of the pancreas . Moreover, rare cases of extrapancreatic manifestations, such as the adrenal gland, have been reported.US examinations of SPTs usually show a homogeneous, hypoechogenic mass with a hyerpechogenic rim.The use of preoperative biopsies is discussed controversially in the literature. While it may be useful in verifying the diagnosisTo achieve excellent long-term outcome, surgery is the mainstay of SPT treatment. Since local recurrences and metastases are reported following incomplete resection,The use of minimally invasive surgery (MIS) for oncological procedures is debated in the literature due to the risk of tumor rupture and subsequent spillage. For SPT, Coelho and coworkers have reported 20 patients with SPT of whom 7 underwent laparoscopic resection with one intraoperative tumor rupture.Nevertheless, laparoscopic procedures, like distal pancreatectomy, have been considered safe and feasible for SPT.In conclusion, laparoscopy in an adolescent patient with an SPT allowed complete resection following basic oncological principles, preservation of splenic and pancreatic function, and avoidance a major laparotomy. While data about the technical feasibility of a laparoscopic approach to pancreatic SPT are already available, this pediatric case report adds a long-term oncological and functional success to the available literature.


Mucinous cystadenomas are a common benign neoplasm of the ovaries that can grow much larger than other adnexal masses; they are recognized as precursors of ovarian cancer and may slowly transform to borderline tumors and invasive ovarian cancer. Prompt and accurate treatment is essential as these tumors can grow to massive sizes and be potentially lethal if left untreated. Health care providers must understand the patient, their expectations and their problems; without proper communication and follow-up, any treatment is destined to disappoint. We present a case of a 76-year-old female with limited access to health care. She presented with a giant cystadenoma that grew over 1 year. Complete resection was decided and the patient underwent complete recovery. On follow-up control patient is doing well. Giant abdominal tumors are now rarely seen because of the improved imaging modalities and minimally invasive approaches, allowing the diagnosis to be made at an earlier stage. Adnexal masses can appear at any age of life and may be caused by both gynecologic and nongynecologic diseases. Imaging, lab markers and surgery are necessary to properly diagnose and treat these masses , 2, 3. CThe patient is a 76-year-old female with a past medical history of diabetes and hypertension. She presented to our department with a 1-year history of a lower abdominal mass. She lived in a remote area so she did not have much access to healthcare. In the last 6 months, she noted that her abdomen was gradually distending until reaching enormous proportions. She remained completely asymptomatic and had no associated symptoms of constipation, vomiting or early satiety. She had delivered two children by vaginal delivery and they were all alive. Also, she did not smoke and did not drink alcohol or experience weight loss.Upon examination, her abdomen was markedly distended; however, bowel sounds were normal and she did not have any abdominal discomfort. An abdominal echography revealed a giant heterogeneous mass with a thick wall and areas of internal septation. Due to this, an abdominal contrast-enhanced computed tomography (CT) was requested, revealing a giant 18.4 × 31.2 × 25.9 cm cystic lesion ; the mass extended from the pelvis all the way through her upper abdomen, was dependent from the right adnexa and was in intimate contact with the bowel and omentum; however, the mass did not invade them. No lymph nodes or other masses were detected.  ComplemePathology revealed a 6 kg giant ovarian mucinous cystadenoma; its capsule measured 22 mm on average and was whitish with pink patches. Upon opening the cyst, 1000 cc of clear liquid was unveiled. The tumor was lined with nonciliated, mucin-secreting, columnar epithelium with goblet cells without atypia .Her postoperative course was uneventful, sips of liquids were initiated on her third postoperative day and full diet was started soon after. She was discharged without complications. On follow–up, the control patient is doing well.Adnexal tumors are a common problem in postmenopausal woman, and due to its wide spectrum of clinical manifestations, they may present as an asymptomatic mass detected only as an incidental finding or as a giant abdominal tumor. Either one of these possibilities holds the likelihood of malignancy; nonetheless, due to the advancement at imaging technologies and health care services, ovarian tumors that reach these massive sizes are now rarely seen , 2. In oOvarian tumors can be classified as stromal tumors ; germ cell tumors (undifferentiated and extraembryonic) and epithelial tumors  , 3. CystDiagnosing a giant abdominal-pelvic mass represents a challenge for the medical team since the clinical examination is usually nonspecific. Imaging studies of the abdomen including CT, ultrasonography or magnetic resonance imaging can aid to distinguish the tumor elements and their features , 6, 7. APathology determines if a completion surgery is needed. Nonetheless, there are many issues to contend including the time elapsed from the initial surgery to the final pathology, the waiting time for surgery and the technical and logistical difficulties faced during the completion surgery . BecauseAdnexal masses constitute a challenge especially in resource-limited countries like our own where poor access to health care and noncompliance are usually anticipated. Health care providers should have a broader view of what disease causes in a patient, and all its cultural, economic and social implications. The relationship between the doctors and the patients must be clear at all times to avoid possible misunderstandings especially in cases where patient compliance is limited. Also, in a unique way, this case proves that giant cystadenomas still occur and that prompt and accurate surgical treatment is vital.


This case report aims to describe the management of a complex odontoma with endodontic involvement of surrounding teeth utilizing a new bioceramic cement consisting of five mineral oxides (5MO) as a retro-filling material. The patient presented for routine consultation with slight dental mobility in the antero-superior region. Upon clinical and computed tomography examination, bone rarefaction was observed in the apical region of teeth 11 and 12, external root resorption in the involved teeth and necrotic pulp of tooth 12. Root canal treatment was performed in teeth 11 and 12. Later, local surgical excision of the lesion was carried out, finding a mass with clinical features of complex odontoma, with histopathological examination of the mass confirming this diagnosis. Retro-filling of tooth 12 with 5MO was carried out. No signs and symptoms were observed over twelve-months of follow-up, with bone neoformation observed in the region. Therefore, 5MO appears to be an effective bioceramic cement that has reparative features. Odontogenic tumors originate from tooth-forming tissues .Odontomas are benign odontogenic tumors of mixed origin , composed mainly of mineralized and conjunctive structure including enamel, dentin, cementum and pulp . OdontomComplex odontomas are mostly asymptomatic, are usually diagnosed during the first and second decade of life , and areFive mineral oxides (5MO) is a new bioceramic cement that’s use has been described in the literature as an apical plug of open apex teeth  and a caA Brazilian white 27-year-old female presented for routine examination with a slight dental mobility in teeth 11 and 12 with no history of trauma or previous treatments. Patient clinical history did not present relevant findings.The patient had no symptoms and no history of trauma or previous treatments in this region. Teeth 13 to 23 was tested by pulp vitality test (cold test) carried out by refrigerant gas  and relative isolation using cotton rolls and a dental saliva ejector. The apical region was also tested by digital palpation and perpendicular percussion and was asymptomatic, with no fistula. The depth of its gingival pocket varied between 1–3 mm with various exploring locations and grade I mobility. Panoramic radiographic examination revealed a radiopaque bone-like structure but with a greater density than bone, circumscribed by a thin radiolucent margin in the apical region of teeth 11 and 12 presenting features of complex odontoma . Cone beFirstly, teeth 11 and 12 underwent endodontic treatment due to the negative response to the pulp vitality test, utilizing a Reciproc system R50/0.05 file  and chlorohexidine gluconate gel 2%  as a lubricator and antimicrobial agent during the preparation of the root canals, and then obturating with gutta-percha and Ah Plus sealer . No medication was prescribed before or during the treatment. The patient was advised to take acetaminophen  in case of pain. A week later, after tomographic planning, surgical excision of the lesion was carried out under local anesthesia using one anesthetic tube , with intra-oral access to the mass achieved via intra-sulcular incision of the palatal region from tooth 14 to 24. After detachment of the flap, osteotomy was performed using a surgical carbide drill nº 06  under intense irrigation with saline solution.The enucleated lesion presented as a hyperdense mass located palatal to the roots of teeth 11 and 12, with clinical features of complex odontoma that involved the apical third of the tooth 11. A bone regularization procedure was carried out, followed by cleaning of the surgical cavity by irrigation with saline solution .Afterwards, the hard-dental surfaces were polished with McCall periodontal curette . Tooth 12 demonstrated external apical resorption; thus, an apicectomy was performed, which was then retro-filled with 5MO  . No boneImmediately after sample excision, the mass was immersed in 10% formalin for 48 hours in order to obtain tissue fixation and was sent for histopathological examination under a microscope for differential diagnosis. Routine histological techniques were performed for soft tissues embedding in paraffin. Hard tissues fragments were demineralized in Ethylenediaminetetraacetic acid (EDTA)  for two months, before embedding. The paraffin blocks were cut into sections and slides were subjected to hematoxylin and eosin (HE) staining and observed using an optical microscope. All slides images were obtained using a Pannoramic Scan .After seven days, no postoperative intercurrences were reported by the patient. Through intra oral evaluation, no hematoma or edema was noticed except for the first two days as the patient mentioned. No exudate was noticed.Soft tissue sections (correspondent to tumor surrounding tissue) demonstrated fragments of connective tissue composed of dense collagen fibers randomly arranged, with fibroblasts and hyalinization areas. Immersed in bundles of collagen fibers, many basophilic round calcification areas were observed. Inflammatory cells were not present. Odontogenic islands were observed.Histologically, the tumor mass was composed of tooth-like structures exhibiting an irregular arrangement. Hard tissue sections presented fragments of a mixture of material composed mainly of immature dentin, enamel matrix, cementum, and pulp tissue. Dentin immature matrix presented irregular tubules; enamel matrix demonstrated a basophilic pattern and barely prismatic spaces from hydroxyapatite loss during the demineralization process; cementum areas were superimposed to dentin, differing from dentin because of its basophilic irregular lines and the presence of cells inside the matrix (cellular cementum); finally, pulp tissue was observed surrounded by dentin matrix, composed of delicate collagen fibers and fibroblasts. In some places, the dentin matrix surface presented columnar cells similar to odontoblasts. Histopathological examination confirmed the diagnosis of complex odontoma .Three follow-up sessions were performed after 30 days, four months and 12 months. In these sessions, clinical intra oral examination was carried out as well as radiographic examination. Bone neoformation was observed by periapical radiograph in the lesion region at the second follow-up session (after four months) compared to the initial one. The periapical radiograph was taken using a radiographic positioner .Odontoma is an anomaly of benign asymptomatic development and slow growth,  , in addition to a brief phrase the new bioceramic cement of five minerals oxide 5MO. However, more details should be provided about this new cement, such as its composition and its clinical applications. The case report part was well explained, however, more control period is indicated for 2, and 5 years until the full healing is obtained, and make sure about the effectivity of this bioceramic cement. The discussion section was good as well. As a suggestion for the authors, the 5MO cement should be compared in the discussion with the similar bioceramic cements like MTA and its new versions like MTA HP and others, this should be more discussed.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Although rare, masses and mass-like lesions of developmental and genetic origin may affect the paediatric craniofacial skeleton. They represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation and disfigurement. The most common lesions include fibrous dysplasia, dermoid cysts, vascular malformations and plexiform neurofibromas. Less common lesions include torus mandibularis and torus palatinus, cherubism, nevoid basal cell carcinoma syndrome, meningoencephalocele and nasal sinus tract. This article provides a comprehensive approach for the evaluation of children with masses or mass-like lesions of developmental and genetic origin affecting the craniofacial skeleton. Typical findings are illustrated and the respective roles of computed tomography (CT), cone beam CT (CBCT), magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI) sequences and ultrasonography (US) are discussed for the pre-therapeutic assessment, complex treatment planning and post-treatment surveillance. Key imaging findings and characteristic clinical manifestations are reviewed. Pitfalls of image interpretation are addressed and how to avoid them.• Masses of developmental and genetic origin may severely impair the craniofacial skeleton.• Although rare, these lesions have characteristic imaging features.• CT, MRI and ultrasonography play a key role in their work-up.• Recognition of pivotal imaging pearls and diagnostic pitfalls avoids interpretation errors. Masses and mass-like lesions related to various developmental and genetic conditions can affect the developing craniofacial skeleton. The majority of masses and mass-like conditions of developmental/genetic origin are benign. Some of these conditions, such as torus palatinus and torus mandibularis, require no treatment other than alleviation of parental anxiety. Other conditions affecting the developing craniofacial skeleton, such as fibrous dysplasia, ossifying fibroma, familial gigantiform cementoma, cemento-osseous dysplasia, hereditary multiple osteochondroma or plexiform neurofibroma, may cause functional impairments due to the proximity to important neurovascular structures, organs of special senses and developing dentition. Cosmetic deformities, due to the lesion itself or due to treatment-related asymmetric facial growth, may cause a significant psychosocial impact on the patient’s life. Management of these patients, therefore, often requires close interdisciplinary work-up, precise and often complex treatment planning strategies and post-treatment surveillance into adulthood. Radiologists, as part of an interdisciplinary team, play an important role in the management of these young patients. In addition, as these rare lesions may mimic neoplasms of the craniofacial skeleton and vice versa, it is imperative to be aware of their characteristic imaging features in order to avoid unnecessary biopsy and expensive follow-up examinations.To the best of our knowledge, a review of the imaging features of these rare masses and their impact on treatment has not been published in the English literature during the past 20 years. Most published articles on the subject are isolated case reports or small case series dealing with the clinical presentation and with patient management. This article attempts to provide a comprehensive radiological review of the most common developmental masses involving the craniofacial skeleton, along with their multimodality imaging features, clinical manifestations and the role of imaging in their pluridisciplinary management –5.The majority of masses and mass-like conditions of developmental/genetic origin are benign. Imaging narrows down the differential diagnosis and helps in planning patient management. Traditionally, ultrasonography (US) and conventional x-ray radiography have been the mainstays of imaging in paediatric lesions. US allows differentiation between solid and cystic lesions of the facial soft tissues and enables rapid assessment of the vascularisation and localisation of the extraosseous components. The US transducer types to be used should be adapted to the small parts investigated. High-frequency linear array transducers  yield excellent spatial resolution, but at the expense of a shallower depth of penetration. US should be ideally coupled with a Doppler evaluation. Based on the Doppler flow waveform, differentiation between infantile haemangioma, a vascular tumour gradually involuting over the years, from low-flow (venous), lymphatic or high-flow (arteriovenous) vascular malformations is facilitated. However, US has a very limited role in the evaluation of the craniofacial skeleton itself. Imaging techniques using ionising radiation, including orthopantomography (OPT), computed tomography (CT) and cone beam CT (CBCT) warrant careful use in accordance with the ALARA (As Low As Reasonably Achievable) principle . As OPT After the initial evaluation, the first decision is whether to use CT or magnetic resonance imaging (MRI) for further work-up or whether the acquired US and OPT images enable reasonable patient management without further imaging. If the lesion is suspected to be of vascular nature, to have a large extraosseous component or of the type that may have a small intracranial connection (e.g. dermoid cyst), MRI is the modality of choice. Many times, however, the exact nature of the lesion is not known and the clinical presentation suggests a primary intraosseous lesion, in which case, CT becomes the imaging modality of choice. Nevertheless, it is important to note that, in cases with involvement of the skull base or prior to planning complex surgery, both MRI and CT/CBCT are required. High-resolution MRI, low-dose CT and CBCT are the standard of care for the assessment of facial masses with skeletal involvement or of primary craniofacial skeletal lesions. When performing CT or CBCT, the radiation dose is usually reduced as much as possible by limiting the scanned area to the absolute minimum, by avoiding the lens if possible and by applying a low-dose protocol . In geneMRI, in addition to having excellent soft tissue contrast, offers the advantages of multiplanar imaging and early detection of marrow oedema, as well as accurate depiction of invasion by tumours and tumour-like lesions . The useF18-Fluoro-deoxy-D-glucose (FDG) positron emission tomography CT (PET-CT) has a limited role, reserved for suspected multifocal disease manifestations or suspected sarcomatous progression of a plexiform neurofibroma into a malignant peripheral nerve sheath tumour (MPNST) , 11. As Common developmental lesions of the facial skeleton include fibrous dysplasia, torus palatinus and torus mandibularis. They present as slowly progressing facial masses and palatal or mandibular palpable indurations. They may occasionally mimic neoplasms on clinical examination.Fibrous dysplasia (FD) is typically seen in children/adolescents, with a slight predilection for girls. It is a sporadic non-neoplastic disease of the bone-forming mesenchyme in which normal lamellar bone is replaced by immature woven bone with irregular trabeculae . The con−3 mm2/s); however, lower ADC values are not exceptional and they do not help in the differential diagnosis. On FDG PET-CT, FD may display high metabolism, mimicking malignancy [In most patients with FD, CT and CBCT reveal characteristic bony expansion with a “ground-glass” appearance Figs.  and 2 orlignancy . Howeverlignancy , 19. Altlignancy . VariousThe management of craniofacial FD includes a “wait and see” policy till the completion of the facial skeletal growth and surgery is performed in cases of persistent or increasing facial deformity Fig. . BecauseCherubism is a rare autosomal dominant disorder with unknown prevalence. It results from a genetic mutation affecting bone metabolism and remodelling, and at least 15 mutation types have been identified so far , 23. MulOPT shows well-defined bilateral multilocular radiolucent lesions causing symmetrical bilateral enlargement of the mandible and maxilla Fig. . CT/CBCTThe differential diagnosis of cherubism includes Noonan-like/multiple giant cell lesion syndrome, FD and central giant cell granuloma of the mandible and maxilla , 28. PatPTCH1 gene on chromosome 9p22.3. This multisystemic disorder is characterised by predisposition to multiple basal cell carcinomas (BCCs), odontogenic keratocysts, desmoplastic variant of medulloblastoma (DVM) and skeletal, dental, ophthalmological and neurological abnormalities [The triad of nevoid basal cell carcinomas, jaw cysts and bifid ribs is known as nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin–Goltz syndrome , 31. NBCmalities –34. NBCCOdontogenic keratocysts (OKCs), formerly called keratocystic odontogenic tumours , are thoTreatment options in OKCs include surgical enucleation and cryotherapy. The reported recurrence rate after enucleation is as high as 60% . NBCCS p−3 mm2/s), therefore, allowing differentiation from glial tumours, which tend to have high ADCs [CT and MRI of the central nervous system (CNS) in patients with NBCCS typically reveal ectopic calcifications of the falx cerebri and tentorium cerebelli, bony bridging of the sella turcica, spotted meningeal calcification, corpus callosum agenesis/dysgenesis with/without lipoma and vermian dysgenesis Fig. . Other cigh ADCs .Conventional x-rays may detect other sites of musculoskeletal involvement, such as polyostotic bone cysts and flame-like finger lesions (hamartomas), bifid, fused, splayed or missing ribs, scoliosis, spina bifida occulta, pectus deformities, polydactyly, syndactyly and Sprengel type scapula involvement . As suggTorus palatinus (TP) is an exostosis occurring along either side of the midline suture of the hard palate. Torus mandibularis (TM) is an exostosis occurring along the lingual surface of the mandible, whereas torus maxillaris (TMax) occurs on the palatal or vestibular side of the alveolar process of the maxilla. According to anthropological studies, tori appear to be more common among populations living in the northern hemisphere as compared to those residing in the south . AlthougAlthough rare, together with Langerhans cell histiocytosis (LCH), dermoid cysts (DCs) are the most common lesions of the paediatric skull. They result from epithelial sequestration during midline union of the embryonic first and second branchial arches. The generic term “dermoid cysts” comprises three entities: (1) epidermoid cyst , (2) true dermoid cyst  and (3) teratoma (showing tissues derived from the primitive germ layers) . Only abDCs are slow-growing lesions causing bone scalloping when arising in immediate bone vicinity Fig. . When arNasal dermoid  sinus cyst (NDSC) includes all nasal lesions containing stratified squamous epithelium (ectoderm derivatives) and adnexal structures (mesoderm derivatives) . It is tHigh-resolution CT and thin-section MRI Fig.  are esseSeveral developmental lesions of the face or genetic conditions with subsequent development of tumours may lead to bone remodelling and scalloping of the craniofacial skeleton, thereby leading to severe functional problems. These lesions include DCs (see above), vascular malformations (VMs) and plexiform neurofibroma (PNF) .Neurofibromas are benign peripheral nerve sheath tumours with the nerve of origin usually incorporated within the lesion . NeurofiPNF of the orbit, face and the temporal region is a rare but devastating complication of NF1. The disease is unilateral and leads to progressive bowing and scalloping of the maxillofacial skeleton Fig. . BuphthaAs PNF can mimic a venous malformation on MRI, it is important to recognise the classic “target sign” of PNF . This characteristic aspect should not to be confused with phleboliths. Occasionally, PNF may be misdiagnosed as lymphatic malformation (LM) if no iv contrast material is administered. Unlike LM, PNF shows mild to moderate enhancement of the infiltrative and solid mass on contrast-enhanced CT and CEMRI.Vascular malformations (VMs) of the face are rare congenital anomalies that do not regress over time and which may rapidly enlarge following trauma or endocrine changes. They are subdivided into capillary, venous, arterial and lymphatic malformations. Arterial vascular malformations (AVMs) are high-flow malformations with tortuous arteries and enlarged veins, resulting in serpiginous flow voids on T1 and T2. Capillary and venous vascular malformations (VVMs) are low-flow lesions with patchy to intense contrast enhancement and, occasionally, phleboliths. If the tissues under the skin are affected, the superficial, subcutaneous lesions may appear as slightly blue-coloured skin stains. However, most often, VVMs present as non-pulsatile compressible soft tissue swellings. As VVMs are present at birth, they usually grow with the child and may have long-term cosmetically and functionally disabling consequences, including pain, respiratory compromise and disfigurement. If the lesions are deeply located, there is only slight facial asymmetry, but no colour change. During a Valsalva manoeuvre, VVMs typically enlarge. The slow venous flow predisposes to repeated episodes of intralesional thrombosis, and sudden enlargement may occur . IncreasThe diagnosis of VVMs is made with colour Doppler US and with MRI Fig. . On US, Lymphatic malformations  are benign vascular lesions, which tend to vary in size, location and extension , 63. TheClinical presentations of LMs are related to infection, haemorrhage and mass effect. Mass effect can cause displacement of vital structures, thus impairing vision, breathing or swallowing. Juxtaposed bones show scalloping, sutural widening and remodelling. Trauma and infection may cause spontaneous bleeding or purulent discharge.Optimal evaluation of superficially located LMs is possible with Doppler US. Uncomplicated LMs appear as anechoic cystic lesions with internal septations and no vascularity on colour Doppler US. US allows differentiation between LM-LMVs and venous malformations, as well as other mixed vascular lesions . CT and A cephalocele is a generic term applied to several congenital or acquired conditions. Encephalocele is herniation of brain parenchyma through an osseous-dural defect of the skull base or cranial vault, whereas meningocele is herniation of meninges alone. Meningoencephalocele comprises herniation of brain parenchyma along with meninges, whereas a meningoencephalocystocele includes herniation of ventricle besides the other two . GliocelImaging plays a major role for the delineation of parenchymal and bone abnormalities, and helps in determining the surgical approach Fig. . High-reThis article provides a comprehensive approach to the understanding of the clinical, radiologic and histologic features of masses and mass-like lesions of developmental and genetic origin involving the craniofacial skeleton, with emphasis on the imaging findings that are essential for diagnosis, treatment and surveillance.


Until this 24 -year -old male was sexually active, the small dimple on his penile glans had never required any consideration. However, persistent focal irritation during sexual intercourse compelled him to pursue medical attention. Following urological consultation he was referred for imaging evaluation that demonstrated urethral duplication. After determining the subtype classification, urologic repair was successfully completed without complication. Urethral duplication is an exceedingly rare genitourinary anatomic anomaly with a variety of presentations and symptomatology. The vast majority of urethral duplications are diagnosed within the paediatric and adolescent populations. Although urethral duplication is managed by urologists, imaging identification of the particular subtype is critical for proper management planning. A thorough review of existing literature on the classification strategies and diagnostic criteria is discussed. Owing to the scarcity of clinical experience, a wide variety of diagnostic approaches are described. These often include unnecessary radiation, invasiveness and cost. A strategic and efficient diagnostic algorithm is included to guide future imaging evaluation of suspected urethral duplication. This provides the essential clinical information, while minimis ing radiation, invasiveness and cost. A 24-year-old previously asymptomatic male presented to his primary care physician with a complaint of focal penile irritation related to sexual intercourse. The irritation originates from a lesion along the midline on the dorsum of his penile glans. He was referred to a urologist for further evaluation.The patient reported noticing a small dimple along the midline on the dorsum of his glans for as long as he could remember. The lesion had not previously caused him any discomfort, nor did he report a history of discharge, including urine, semen, blood or pus from the orifice. In recent months, the patient has become more sexually active and he has become aware that during and after intercourse the site is painful and inflamed for a short period of time. The patient wants to make sure there is not something wrong with his anatomy and seeks a solution.The patient had no significant past medical history. There is no family medical history of relevance, specifically no one has reported any genital malformation. Physical examination by a urologist demonstrated a well-developed adult male. A small midline opening was present on the dorsum of the penile glans. There was no focal erythema or discharge present at the time of examination. Manipulation of the opening demonstrated a thin lumen that appeared to continue proximally along the dorsum of the penile shaft. The most likely aetiology was determined to be urethral duplication. Imaging was necessary for confirmation and to delineate the particular anatomic presentation in order to plan treatment. Demonstration of communication with the urethra or bladder would require more extensive surgical intervention to eliminate the sequelae of discharge accumulation in the lumen causing infection, cyst formation or recanalisation of the accessory orifice. The patient was referred to radiology for urethrography.Since no urine, semen or other discharge had ever been noted to exude from the accessory urethra, a retrograde urethrogram (RUG) was performed . A 5F paAfter confirming a Type 1-A urethral duplication the patient was offered and consented to operative reconstruction. A short rigid ureteroscope was used to inspect the main urethra and bladder demonstrating the appearance of normal verumontanum and sphincteric mechanisms confirming the ventral urethra as the functional urethra. The ureteroscope was then used to evaluate the accessory urethra and passed through to the blind-end. The accessory urethra was cauterized as the ureteroscope was withdrawn.The patient tolerated the procedure well without any reported postoperative complications. At follow-up the patient did not report recurrent irritation during sexual intercourse and was pleased with the cosmetic result.2 The most common classification systems are the Effmann3 or Woodhouse and Williams4 systems. The former is considered more therapeutically practical and is more commonly used to describe anomalies. The Woodhouse and William system is not as functional but allows for subtype differentiation of the less common coronally oriented anomalies. These are not incorporated within the Effmann system and tend not to alter surgical planning.Although rare, urethral duplication is known to occur along a spectrum of severity distinguished by discrete anatomic features. The precise incidence is difficult to gauge as there is no consensus from the literature. There are approximately 300 known cases.The Effmann system classifies a blind incomplete accessory urethra as Type 1. The Type 1-A subtype is an accessory urethra that lacks communication with the functional urethra or bladder. The Type 1-B subtype is a proximal accessory urethra that derives from the functional urethra and terminates blindly within the periurethral soft tissues, similar to a urethral diverticulum. Type 2 duplications represent a complete functional duplication of the urethra with subtypes differentiating the origin of the duplication at either the bladder or along the orthotopic urethra. Further subtype differentiation of Type 2 duplications are determined by their site of termination; either terminating at the penile dorsum, perineum, rectum or reconnecting with the orthotopic urethra more distally in its course. Type 3 duplications are complete duplications of the bladder and functional urethra .5 Urethral duplications occur most commonly in males; however, rare instances of female urethral duplication are described in literature.6 The diagnosis is most often made within the first few years of life but is usually not delayed beyond adolescence. It is uncommon for a case to be diagnosed in adulthood as in this case report. Because around 93% of urethral anomalies occur within the sagittal plane,4 the functional urethra, verumontanum and sphincteric mechanisms are most often located ventrally. It is a clinical imperative to ensure proper identification of the functional urethra prior to surgical intervention as the functional urethra must be preserved for proper repair and future function. 7The aetiology of the anomalous development is uncertain and likely results from several embryologic errors.9 No formal diagnostic strategy has been proposed to guide clinicians, surgeons and radiologists on the most efficacious diagnostic methodology to minimize radiation dosage, exposure to invasive examinations and cost. The following algorithm outlines an efficient strategy for determining the Effmann type of duplication and the functional urethra utilizing the most diagnostically informative and parsimonious methodology , however, compared with RUG, IVP is more invasive requiring intravenous contrast as well as involve more radiation and time to evaluate the kidneys and bladder which may not be necessary for the diagnosis. Furthermore, some duplications will have a non-dominant urethra that may be obstructed with debris or present too much resistance for downstream flow to adequately opacify, ultimately requiring a follow up RUG to evaluate the urethra anatomy further increasing the radiation dose, invasiveness and time.Initially, a thorough physical examination and history should be performed to determine the functional status of both urethras. If a second urethral orifice is identified along the perineum or rectum then Type 2-A2 Y duplication should be suspected. A RUG should be performed on both urethras to demonstrate proximal union of the urethras and to ensure the perineal/rectal orifice is not a fistulous communication of another etiology.10 If a single bladder distends from retrograde flow from both urethras then the patient should undergo ultrasound evaluation of the kidneys. Unilateral hydronephrosis will suggest Type 3 with partial duplication; however, in this case the ureters insert on separate bladders but the urethras derive from the same bladder resulting in outlet obstruction for the contralateral bladder and severe unilateral hydronephrosis and ultimately dysplasia of the affected kidney.11 If both kidneys demonstrate normal sonographic architecture then a Type 2-A1 duplication is present.If only one of the urethras produces urine then a RUG of each urethra should be performed. Identification of a blind ending accessory urethra anywhere along the longitudinal length of the penis will diagnose Type 1-A. If two urethras are demonstrated with terminations approximating the location of the bladder sphincteric mechanisms, additional cystogram images should be obtained to provide additional anatomic information. If there is demonstration of two bladders then Type 3 partial duplication is present. In this case the ureters insert on the same bladder while the urethras derive from different bladders; this anomaly is usually owing to a duplicated non-functional bladder in the sagittal plane.If both urethras excrete urine then Type 2 or 3 duplication is possible and a RUG should be performed on both urethras. If the proximal aspects of both urethras unify immediately distal to the bladder then the duplication is Type 2-A2. If the urethras terminate at the level of the bladder then further contrast should be administered through both urethras to opacify the bladder. If each urethra derives from a separate bladder then the duplication is Type 3 complete duplication. However, if a single bladder distends from retrograde flow from both urethras, similar to the same findings with a single voiding urethra, then the patient should undergo ultrasound evaluation of the kidneys to determine the presence of unilateral hydronephrosis to suggest a Type 3 partial duplication. If both kidneys demonstrate normal sonographic architecture then a Type 2-A1 duplication is present.Ambiguous cases should be referred for direct cystoscopic evaluation as it is unlikely further radiographic investigations will result in more information. Direct visualization of the verumontanum via cystoscopy may be necessary in some cases where determination of the functional urethra is ambiguous.14 Concurrent anatomic abnormalities are found more frequently in cases of bladder duplication.15 Further evaluation with cross sectional imaging of additional anomalies in the genitourinary, gastrointestinal or central nervous system may be pursued as indicated for surgical planning or clinical management. The decision to proceed with surgical intervention depends on symptom presentation and cosmetic considerations. Surgical approach depends on the anomaly type and can vary in complexity from simple meatoplasty to island flap urethroplasty, accessory urethral excision and, in the most severe cases, ureteral reimplantation.At least 60% of urethral duplications have a second associated abnormality such as: vesicoureteral reflux, bladder exstrophy, epispadias, hypospadias, cryptorchidism, anal stenosis and renal dysplasia.16 Extant literature espouses the use of MR urethrography for evaluation of urethral duplication, however, in the opinion of the authors, there is insufficient evidence to support exclusive use as the primary imaging modality. A complete discussion of the benefits and limitations of MR for the evaluation of genitourinary anatomy and pathology is beyond the scope of this article, however, an appreciation of the specific features supporting or excluding its inclusion in the diagnostic algorithm is appropriate.MR urethrography is an alternative modality available for the evaluation of the lower genitourinary system. This modality, although not new, is only recently developing into a common tool for the evaluation of genitourinary pathology.17 For patients requiring sophisticated surgical planning, or if a high degree of clinical suspicion exists for associated genitourinary or gastrointestinal anomalies, MR urethrography’s additional soft tissue information would provide clinically beneficial information and an efficient means to diagnose and provide broader management information.18 In fact, MR is the best available modality for detailing the presence of anomalies, fistulas or other comorbidities that will affect surgical planning.16 Additionally, MR provides all of these benefits without the use of ionizing radiation.The diagnostic reliability of MR urethrography for evaluation of urethral anatomy is likely adequate for employing its use delineating the Effmann subtype of urethral duplication.19 Avoiding the use of anesthesia in the pediatric population receiving MR evaluation often requires the use of distraction tools such as child life consultants and sophisticated interventions that further increase the cost and duration of the study.19 Furthermore, the practicality of MR urethrography itself is challenging requiring the injection of gel or saline into the urethra to ensure adequate urethral distention to optimize signal strength and anatomic delineation;16 this increases the invasiveness of the diagnostic process as well as compromising the reliability of the results owing to under-distension or inadequate distension.Nonetheless, according to the opinion of the authors, the limitations of modern MR urethrography preclude its use as a practical tool in the urethral duplication diagnostic algorithm. Since most patients with urethral duplication are young children, sedation with some type of anesthesia will be necessary, increasing the invasiveness and cost of making the diagnosis. Of all potential risks associated with pediatric MR evaluation the greatest source of complication derives from anesthetic use resulting, at worst, in as many as 1:4000 deaths.16 less invasive and significantly less expensive and time consuming.Ultimately, although exposing the patient to ionizing radiation, fluoroscopic evaluation with voiding cystourethrogramor IVP are equally if not more reliable than MR for diagnosing duplication subtype,Urethral duplication is a rare congenital anomaly with a variety of presentations each necessitating reliable determination of the anatomic subtype and identification of the functional urethra to ensure proper surgical management.Following the simple radiologic algorithm provided, efficacious diagnosis of urethral duplication subtype can be determined utilizing the least possible radiation, cost and invasive risk.Informed consent to publish this case was obtained and is held on record.


Limited evidence is available regarding the dissemination of tumor tissues due to compression during massage therapy, a routine procedure in patients with various symptoms in Asian countries.18Fluorine-labelled fluorodeoxyglucose-positron emission tomography and computed tomography revealed multiple metastases in the spine, liver, and lung. Consistent with osteosarcoma, histopathological examination revealed tumor cell proliferation with extensive pleomorphism and mitoses. Despite undergoing chemotherapy, radiation therapy, and hip disarticulation, the patient died due to multiple metastases 13 months after the initial diagnosis.A 12-year-old male presented at a massage clinic with pain and swelling of his left knee, which worsened the same night. Consistent with conventional osteosarcoma, radiography revealed cortical bone destruction, osteoblastic changes, and periosteal reactions. Magnetic resonance imaging revealed a tumor in the distal femur, an extraskeletal mass, and an infiltrative lesion in the intramuscular and neurovascular areas surrounding the distal femur; this was considered as hemorrhage and dissemination of the tumor tissue. The present case suggests association of massage therapy with the local dissemination of tumor tissues, although influence of massage therapy on metastatic lesions remains unclear. Massage therapists should be aware of the possibility for dissemination of hidden malignancies due to the procedure. Despite it being the most common primary malignancy of the bone in adolescents and young adults, the incidence of osteosarcoma is only 5–7 cases/million/year . StandarOsteosarcoma most commonly affects the distal femur , and pat18Fluorine-labeled fluorodeoxyglucose-positron emission tomography revealed tumor metastasis in the femur and multiple metastases in the thoracic and lumbar spine, liver, and pelvis  with osteosarcoma underwent alternative medical treatment including massage therapy before their initial visit to the hospital . A remar


Primary angiosarcomas of the right atrium are extremely rare, often resulted in missed diagnosis or misdiagnosis with routine examination tools. These malignant cardiac tumors are highly aggressive with generally poor prognosis. Surgical excision is the mainstay of treatment as it is essentially not responsive to current regimens of chemoradiotherapy.Herein, we describe a patient who initially presented with paroxysmal atrial fibrillation and was subsequently treated with radiofrequency catheter ablation (RFCA). Prior to RFCA, an initial transesophageal echocardiography revealed a local thickening of the intratrial septum. Three months later, she was hospitalized with progressive dyspnea and massive pericardial effusion. A large immobile, non-pedunculated mass, occupying almost half of the right atrium was detected by transthoracic and transesophageal echocardiogram. Multimodality cardiac imaging was useful in further characterizing this mass, which was ultimately diagnosed as an angiosarcoma based upon biopsy results. The growth rate was extremely rapid following RFCA, and patient underwent surgical excision. After discharge, the angiosarcoma recurred and patient survived for 7 months from the first episode of tamponade.Primary cardiac angiosarcoma of the right atrium can easily be mistaken for structural anomalies in its early stages, losing the opportunity for initiating earlier treatments to improve potential patient outcomes. The correct diagnosis of this rare case relied on the comprehensive utilization of multimodal imaging techniques including biopsy. Angiosarcomas are malignant tumors that originate from the vascular endothelium. Incidence of angiosarcoma is extremely low , but ang. Three months after RFCA, the patient was readmitted following the sudden onset of chest tightness, dyspnea, transient palpitation, and recurrent pericardial effusion. Chest x-ray examination revealed moderate cardiomegaly and T-wave abnormalities in precordial leads were found in electrocardiogram. Transthoracic echocardiography (TTE) examination revealed a large immobile, non-pedunculated mass (47 mm × 17 mm × 11 mm) occupying almost half of the right atrium (RA)  was performed which showed a small, localized thickening of the interatrial septum that was considered as a structural variant Fig. a. She suBased upon these findings, our attention focused on the identity of the mass: cardiac tumor or hematoma? If it was a tumor, was it benign or malignant? If it was malignant, had it already metastasized? A F18-fluorodeoxy glucose-positron emission tomography (18F-FDG PET-CT) scan was advised to further characterization of the mass and assessment of metabolic activity. The scan revealed an area of abnormal tracer activity in a hard tissue mass occupying the RA and encroaching on the adjacent left atrial roof Fig. a, b. WitPrevalence of primary angiosarcomas of the right atrium is extremely rare . These mThree months later at a follow-up visit a fully developed angiosarcoma complicated by right atrial wall invasion and pericardial tamponade were found. No malignant or atypical cells were found in the bloody pericardial effusion. Analysis of pericardial fluid does not always show malignant cells even in the presence of pericardial invasion which emphasizes the limited diagnostic value of this test . Reviewismall local lesions of the atrial septum are generally difficult to detect by TTE, because of low spatial resolution and poor penetration through the chest wall. TEE is a useful imaging modality with high resolution as the proximity between the transducer and heart, which could provide a superior evaluation of the tissue characteristics compared to TTE [There has been increased use of noninvasive imaging techniques for diagnosing the presence of tumors . While ed to TTE . MRI is Due to its more restricted availability and higher cost, 18F-FDG PET-CT has been reserved as a non-invasive imaging modality for suspected metastasis of unknown origin and for preoperative staging of various neoplasms. It offers a high sensitivity scan for metabolic activity with precise anatomical localization with a better analysis of soft tissues. It enables assessment of the tumor’s infiltration of the myocardial walls, the pericardium and adjacent organs. This is valuable since malignant neoplasms and their metastases are always characterized by enhanced glucose utilization and therefore are detectable by 18F-FDG PET-CT.Complete surgical excision provides the greatest survival benefit and outcome of the patient depends on if there is a recurrence of the tumor or not . It is tA large angiosacroma without metastasis, the diagnosis was suggested by 18F-FDG PET-CT, is highly unusual as these tumors are generally very aggressive and highly invasive  Sabzi etIn conclusion, primary cardiac angiosarcoma of the right atrium is an extremely rare but a highly aggressive tumor. In its early stages, these tumors can easily be mistaken for structural anomalies. The successful diagnosis of our case relied on the comprehensive utilization of multiple imaging techniques including biopsy examinations.


Epithelioid sarcoma is a malignant soft tissue tumor arising from mesenchymal tissue and usually occurs in the extremities. The tumor involving the head and neck region is extremely rare. We present radiologically well-documented case of an epithelioid sarcoma arising from the temporal space.18F-fluorodeoxyglucose positron emission tomography/computed tomography (18FDG-PET/CT) was used to stage the tumor and demonstrated intense FDG uptake in the mass without regional lymph node or distant metastasis. After the pathologic diagnosis of epithelioid sarcoma, the patient underwent total surgical resection of the tumor followed by postoperative irradiation. There was no evidence of recurrent disease during the follow-up period of 18 months.A 35-year-old woman presented with a slowly growing, painless palpable mass in the left temporal area. Ultrasound (US) revealed a lobulated hypoechoic mass with internal vascularity. On magnetic resonance (MR) imaging, the mass showed heterogeneous signal intensity with a central necrotic area and peritumoral infiltration. On the basis of the clinical and radiological characteristics, the lesion was considered to be a malignant tumor originating from soft tissue. An incisional biopsy was performed. The diagnosis of epithelioid sarcoma was based on microscopic examination and immunohistochemical analysis. An epithelioid sarcoma should be considered in the differential diagnosis of a locally aggressive lesion occurring in the temporal space of head and exhibiting a heterogeneous appearance on imaging studies, including a central necrotic area and signal intensity suggestive of infiltration of soft tissue adjacent to the tumor. It is, however, true that head-and-neck involvement is very rare, and the radiological findings are not pathognomonic. The tumor principally presents as a subcutaneous or dermal mass that spreads along the fasciae and tendon sheaths.,3 Although several reports on tumor features evident on computed tomography (CT) and/or magnetic resonance (MR) imaging have appeared, the radiological findings of epithelioid sarcoma are variable. No characteristic features allowing the tumor to be differentiated from other malignant soft tissue tumors have been defined to date. The present study reports a case of epithelioid sarcoma presenting as a subcutaneous mass in the temporal space. We also briefly review the literature. To our knowledge, no tumor in this location has been imaged to the extent that we report.Epithelioid sarcoma is a rare mesenchymal tumor accounting for 1% of all soft tissue sarcomas. Epithelioid sarcoma was first described and established as a distinct clinicopathological entity by Enzinger in 1970.2A 35-year-old woman presented with a 1-year history of a progressively growing painless mass in the left temporal area. Physical examination revealed a firm, nontender palpable mass in the left temporal area just superior to the zygomatic arch. The lesion exhibited no superficial ulceration or cutaneous erosion. All laboratory data were normal. Initial work-up with ultrasound (US)  revealed a somewhat ill-demarcated, lobulated hypoechoic mass 3.0 × 2.5 × 1.5 cm in dimensions located subcutaneously in the left temporal region   was used to stage the tumor and revealed focally intense FDG uptake  were multinodular in appearance, or exhibited multilobulated contours. Such patterns were suggestive of epithelioid sarcoma, but it was crucial to distinguish such sarcomas from other soft tissue sarcomas, although smaller lesions were round and well-circumscribed. Histologically, the tumors exhibited varying degrees of cellularity, necrosis, fibrosis, and hyalinization. In particular, central degeneration or necrosis was commonly apparent,7; this contributed significantly to the tumor enhancement pattern evident upon postcontrast imaging. In our case, the tumor was clearly multilobulated in nature and exhibited nonenhancing portions on postcontrast images, corresponding to the hyperintense areas apparent on T2-weighted images, and to pathologically proven regions of intratumoral necrosis.Several reports on the imaging characteristics of epithelioid sarcoma have appeared. Most radiological findings are MR images and are found in various case reports. Only a few studies on MR imaging findings in a series of patients with epithelioid sarcoma have appeared; no obvious characteristic MR features have been documented. In the context of treatment, it is critical to distinguish peritumoral infiltration or invasion from reactive inflammatory change when soft tissue adjacent to the tumor exhibits signaling abnormalities upon MR imaging. In the present case, a heterogeneous increase in the signal intensity of soft tissue adjacent to the tumor was evident on both T2-weighted and postcontrast images. Pathologically, this corresponded to regions of tumor infiltration. This finding is in contrast to what was noted by Hanna et al, who found that a peritumoral, edema-like hyperintense signal on T2-weighted images corresponded, histologically, to an inflammatory reaction.In gross section, epithelioid sarcoma usually exhibits indistinct infiltrating margins.,8 but calcification, hemorrhage, or bone involvement is infrequent.,8,14 Our present case did not exhibit nodal involvement, calcification, or bone invasion either radiologically or pathologically. However, hemorrhagic tumor components were evident in the specimen cross-section. T2-weighted imaging showed regions of low signal intensity within the tumor; these may correspond to the hemorrhagic foci that were pathologically documented.On the basis of previous imaging studies, regional lymph node metastasis is common (50–62.5% of cases),,6 However, most tumors are negative for S-100 protein. In the present study, the tumor was positive for CK, vimentin, EMA, and CD 34, but negative for S-100 protein, consistent with the data of previous studies.Although the radiological findings thus do not permit a specific diagnosis of epithelioid sarcoma, immunohistochemical analysis is useful in this context. Epithelioid sarcomas are commonly positive for CK, vimentin, and EMA, and for CD 34 in about half of all cases.In conclusion, we present here an epithelioid sarcoma arising from the temporal space. An epithelioid sarcoma should be considered in the differential diagnosis of a locally aggressive lesion that is heterogeneous in appearance on imaging, although the head-and-neck region is rarely involved and radiological findings are not pathognomonic. A multilobulated tumor contour and a central necrotic area may aid in distinguishing an epithelioid sarcoma from other soft tissue sarcomas. In addition, an abnormal signal from soft tissue adjacent to the tumor suggests peritumoral infiltration.Conceptualization: Seungbae Hwang.Data curation: Yong Seek Kim, Yo Na Kim.Formal analysis: Seungbae Hwang.Investigation: Yong Seek Kim.Resources: Yo Na Kim.Supervision: Hyo Sung Kwak, Gyung Ho Chung, Seungbae Hwang.Validation: Hyo Sung Kwak, Gyung Ho Chung.Writing – original draft: Yong Seek Kim.Writing – review & editing: Hyo Sung Kwak, Gyung Ho Chung, Seungbae Hwang.Seungbae Hwang orcid: 0000-0002-2420-3182


Methotrexate, an immunosuppressant, is widely used as the standard therapeutic drug for rheumatoid arthritis. With the increasing frequency of use of methotrexate, adverse effects of methotrexate have been reported, one of which is known as methotrexate-associated lymphoproliferative disorders. The etiology of hepatic methotrexate-associated lymphoproliferative disorders remains largely unknown. To date, there have only been ten cases of hepatic methotrexate-associated lymphoproliferative disorders reported in the English literature and a case report is very rare.An 82-year-old Japanese man with rheumatoid arthritis treated with methotrexate presented with fever. Contrast-enhanced computed tomography showed multiple hypovascular nodules in his liver, spleen, and lung, and para-aortic lesions. Endoscopic ultrasound-guided fine-needle aspiration biopsy for liver tumors was performed, and pathological results identified cluster of differentiation 20-positive lymphocytes. Discontinuance of methotrexate led to regression of the nodules and a final definitive diagnosis of methotrexate-associated lymphoproliferative disorders was made.We review 11 reported cases of hepatic methotrexate-associated lymphoproliferative disorders including the present case. Physicians should discontinue methotrexate in patients with rheumatoid arthritis treated with methotrexate when elevated soluble interleukin-2 receptor and hypovascular lesions in contrast-enhanced computed tomography are confirmed considering the possibility of methotrexate-associated lymphoproliferative disorders. Methotrexate (MTX), an immunosuppressant, is widely used as the standard therapeutic drug for rheumatoid arthritis (RA) . With thAn 82-year-old Japanese man with a history of RA presented with fever and malaise. His medical history revealed RA for 10 years. He had been undergoing treatment with MTX, prednisolone, and bucillamine for 9 years and 6 months . The MTX dose was initially 4 mg/week; however, the RA symptoms were not well controlled. Therefore, the dose was gradually increased to MTX 12 mg/week, and the total dosage was 3454 mg. Other medical history included benign prostatic hyperplasia and gout. He smoked cigarettes at 1.5 packs per day for 20 years but did not drink alcohol. His family history was unremarkable.2 with no noticeable body weight changes. He had a fever, but his other vital signs were stable: blood pressure, 128/57 mmHg; pulse, 88/minute; body temperature, 39.2 °C; respiratory rate, 18/minute; and oxygen saturation, 98%. No particular abnormal physical findings were noted other than chronic swelling of his wrists and ulnar deviation of his digits, although he was adequately treated. Furthermore, no enlargement of superficial lymph nodes was observed. Laboratory studies revealed findings of elevation of C-reactive protein (CRP) and soluble interleukin-2 receptor (sIL-2R). Serum hepatobiliary enzymes, rheumatoid factor, and anti-cyclic citrullinated peptide antibody were also elevated -positive B cell lymphocytes with necrotic tissue and no malignant findings , spleen (n = 2), abdominal para-aortic lymph node (n = 2), adrenal grand (n = 2), cervical lymph node (n = 1), and retroperitoneal lymph node (n = 1). Six cases (54.5%) only had liver involvement. To date, most of the reported cases of MTX-LPD in the liver, including the present case, have had multiple lesions. Among the 11 reported cases, eight had multiple lesions compared to three cases of single lesions.Table The laboratory findings are limited because some reports do not mention them. Considering as many data as we could obtain, liver function enzymes were slightly elevated, while sIL-2R, LDH, and CRP were relatively increased. The median values at the presentation were 1.9 mg/dL of total bilirubin , 90.5 IU/L of aspartate aminotransferase , 71.5 IU/L of alanine aminotransferase , 4067/mL of sIL-2R , 557 IU/L of LDH , and 7.555 mg/dL of CRP . In particular, all cases were associated with a high value of more than 1000 U/mL for sIL-2R. As for the possible mechanisms of elevated liver enzymes, there are three major causes. The first one is tumor involvement of the small intrahepatic biliary tree, which can cause progressive cholangitis, duct necrosis, and liver function impairment. The second mechanism is that extensive infiltration of sinusoids and hepatic vasculature by the tumor causes diffuse hepatic necrosis. Finally, cytokines such as interleukin 2, which are particularly secreted from tumor cells, might damage interlobular bile ducts and cause portal fibrosis either directly or by an immunological mechanism .n = 11), followed by ultrasound (n = 8), FDG-PET (n = 4), magnetic resonance imaging (n = 3), and gallium scintigraphy (n = 1). For the patient in the present case report, contrast-enhanced CT scans featured multiple hypovascular lesions, but it is difficult to distinguish hepatic lymphoma from hepatocarcinoma or metastatic tumors because, to the best of our knowledge, there are no reported diagnostic features of hepatic lymphoma [As previously stated, about half of the cases involved only the liver, so the imaging modality is important for the diagnosis. Some previous reports suggested imaging studies, especially ultrasonography and CT, are helpful for diagnosis. However, other types of imaging modalities, such as magnetic resonance imaging, fluorodeoxyglucose-positron emission tomography (FDG-PET), and gallium scintigraphy have been reported. CT was used in almost all cases , postoperation with no recurrence (n = 2), and death (n = 1). Recurrence occurred in one case, but chemotherapy led to remission. Operations were performed in two cases; four cases were treated with only chemotherapy. One of the patients died, and the remaining ten cases survived. When it comes to the patient who died, there are no specific characteristics compared to the other cases, but only discontinuation of MTX was done and chemotherapy was not performed as the treatment. Therefore, these findings suggest that an early diagnosis and immediate treatment, especially the discontinuance of MTX, are essential to prevent deterioration; if the lesions fail to regress, even after the discontinuance of MTX, chemotherapy should be considered.Regarding treatments, to the best of our knowledge, there is no established standard method of treatment for MTX-LPD . DiscontWe reported a case of hepatic MTX-LPD presenting multiple hepatic lesions treated with discontinuation of MTX, and reviewed 11 previously reported cases including the present case. Physicians should discontinue MTX in patients with RA treated with MTX when elevated sIL-2R and hypovascular lesions in contrast-enhanced CT are confirmed considering the possibility of MTX-LPD.


Leiomyosarcomas (LMSs) of the colon are extremely rare and highly aggressive. Although treatment of gastrointestinal LMS is not standardized, surgical resection is generally performed. The fact that the tumors are usually large at the time of diagnosis may explain why no report on laparoscopic resection of a colonic LMS has appeared.A 46-year-old male presented with hematochezia 1 month in duration. Abdominal examination including palpation was normal. The levels of several blood tumor markers were normal. Colonoscopy revealed a polypoid lesion approximately 30 mm in diameter in the sigmoid colon 30 cm from the anal verge. Contrast-enhanced computed tomography revealed that the tumor was 28 mm in diameter, and that no lymph node or distant metastasis was apparent. Histopathological examination of a biopsy specimen revealed spindle-shaped cells exhibiting significant nuclear atypia and a trabecular proliferation pattern upon hematoxylin-eosin staining. Immunohistochemically, the sample was positive for SMA and desmin, and negative for c-kit, DOG-1, CD34, and S-100. Furthermore, the Ki-67 index was > 50%. We thus diagnosed a leiomyosarcoma of the sigmoid colon without any metastasis. We performed laparoscopic sigmoid colectomy and regional lymphadenectomy using five trocars. After complete curative resection, a colorectal end-to-end anastomosis was created employing the double-stapling technique. All surgical margins were negative, and no lymph node metastasis was observed. The postoperative course was uneventful, and the patient was discharged 9 days after operation. No recurrence was noted to 1 year after surgery.We report the first case of a colonic LMS treated via laparoscopic surgery. Although further work is necessary to assess prognosis and to develop the treatment further, laparoscopic surgery to treat small colonic LMSs may be feasible, being both minimally invasive and curative. Leiomyosarcoma (LMS) of the colon is an extremely rare and highly aggressive neoplasm , 2. AfteA 46-year-old male presented to our institution complaining of hematochezia 1 month in duration. His past medical history included coronary vasospastic angina and diabetes mellitus that was treated with insulin. He drank socially and had a 50-pack-year smoking history, but had quit smoking 4 months prior. Abdominal examination including palpation was normal. The levels of carcinoembryonic antigen 4.2 ng/mL) and carbohydrate antigen 19–9 (13.2 U/mL) (commonly measured blood tumor markers) were normal. Colonoscopy revealed a polypoid lesion approximately 30 mm in diameter in the sigmoid colon 30 cm from the anal verge  was placed in the umbilicus using an open method. Another 12-mm trocar was placed in the right lower abdomen, and three 5-mm trocars were placed in the left lower and either side of the abdomen Fig. a. We ideThe postoperative course was uneventful, and the patient was discharged 9 days after the operation. He was followed-up every 6 months by contrast-enhanced computed tomography and every 12 months by colonoscopy; he did not receive adjuvant chemotherapy. There was no evidence of recurrence to 1.5 years after surgery. The clinical course of this patient is shown in Fig. LMS of the colon is often diagnosed later than LMS of the rectum. Therefore, only a few such patients can undergo laparoscopic surgery. We here report the first case of curative laparoscopic surgery to treat LMS of the colon.LMSs originate from smooth muscle, which is widespread in the body; LMSs are most common in the retroperitoneum (including the pelvis), large blood vessels , and the lower extremities . GastroiWe searched the PubMed database using the MeSH terms “leiomyosarcoma” and “colon.” English-language reports published after 1998 investigating cases that were clearly immunohistochemically diagnosed as LMSs were selected. All cited references were also reviewed. The search yielded 197 publications, of which 26 were ultimately evaluated . Table 1Surgery is the standard treatment for localized soft tissue and visceral sarcomas . In all We report an LMS of the colon treated via laparoscopic surgery. Although further work is necessary to assess prognosis and to develop the treatment further, laparoscopic surgery to treat small colonic LMSs is feasible, minimally invasive, and curative.


We present a rare case of metacarpal chondrosarcoma with cutaneous metastases in the ipsilateral upper arm. Chondrosarcomas of the small bones of the hand rarely metastasise unlike chondrosarcomas elsewhere in the body. Excision/ray amputation rather than curettage may be preferable in the treatment of high-grade chondrosarcomas in the hand. Chondrosarcoma of the hand is a rare malignant tumour of the bone, which usually is slow growing and has an extremely low metastatic rate. We report a rare case of subcutaneous and pulmonary metastases from a metacarpal chondrosarcoma 10 months after ray amputation but without local recurrence.1 The most common sites of involvement are the long tubular bones (50%). In contrast, while enchondromata are common, chondrosarcoma of the small bones of the hand is extremely rare. Chondrosarcoma can metastasize to the lung, followed by the skin, soft tissues and, rarely, to other bones.2 Here we illustrate a rare case of a grade II chondrosarcoma of the thumb metacarpal with subcutaneous and pulmonary metastases. There are only three previously reported cases of chondrosarcoma of the small bones of the hand with cutaneous metastases in the literature. Our case demonstrates certain differences from the previous reports.Chondrosarcoma is one of the most frequent primary bone malignancies in adults aged over 40 years .T1 weighted images, the lesion was of low signal; on fluid-sensitive sequences, the lesion showed high signal with a thin and irregular septae. Following contrast medium administration, inhomogeneous, multilobulated, peripheral enhancement of the lesion was noted but also areas of non-enhancement predominated centrally, consistent with a cartilaginous lesion.We describe a case of a 40-year-old female nurse who presented in May 2003 with an 18-month history of pain in the left thumb. Clinical examination showed soft-tissue swelling around the left thumb metacarpal. Radiographs showed an expansile lesion occupying most of the metacarpal with periosteal reaction and cortical destruction. MR scan  showed eThe CT scan  demonstrAn open biopsy was performed in June 2003, the histology of which revealed a grade II chondrosarcoma with evidence of spread beyond the cortex into the periosteum. Vascular invasion was not identified. 1 month later, under general anaesthesia, a first ray amputation was performed with disarticulation of the thumb at the trapezioscaphoid joint. A tourniquet was used at the time of the surgery to exsanguinate the limb. Macroscopic examination revealed an expansion of the metacarpal bone due to a chondroid tumour measuring 35 × 25 mm, which eroded through the cortex but did not cross the metacarpophalangeal joint. Microscopic examination of the sections  confirmeT2 weighted images, intermediate signal on the T1 weighted images and peripheral enhancement after intravenous gadolinium administration. Subsequently, an excisional biopsy of the left upper arm lesion was performed. Macroscopically, the soft-tissue mass showed encapsulated grey myxoid tissue, which was centrally cystic and partially haemorrhagic. This was microscopically confirmed to be a grade II chondrosarcoma, most likely representing a metastasis from the previous thumb chondrosarcoma. There was no lymphoid tissue seen that could be associated with the subcutaneous lesion to suggest lymph node metastases. The closest margin was less than 1 mm. At the same time, a biopsy of the hypertrophic scar at the site of ray amputation was performed, which did not reveal any evidence of neoplasia on histological examination. Owing to the close margins of this subcutaneous lump on excision, a wider excision was performed a month later under general anaesthesia.In April 2004, the patient noticed a superficial, firm swelling in the medial aspect of the left upper arm, which was not particularly painful. There was no clinical or radiographic evidence of local recurrence in the hand. An MRI scan demonstrated a subcutaneous lesion  on the mIn June 2004, a CT scan of the chest was performed and a solitary tiny nodule in the right middle lobe , which w3 As many studies have already demonstrated, the survival is related to tumour grade.4 reported a 10-year survival rate of 89% in low-grade, 71% in intermediate-grade and 38% in high-grade tumour, and similar data were described by Lee et al1 and Fiorenza et al.5 However, in the study by Fiorenza et al,5 no distinction was made between de-differentiated chondrosarcoma and grade III chondrosarcoma, which are distinct tumours, and a large proportion of their patients had wide resection of tumours, unlike other studies, which accounts for their lower recurrence rates. It has already been well demonstrated that the nature of surgical treatment is a very important prognostic indicator, with a wider resection demonstrating better survival and lower recurrence rate than curettage.Chondrosarcoma is the second most common primary malignant bone tumour. The long bones are the most common site of this tumour. Benign cartilaginous tumours are common in the hand but chondrosarcoma is rare and represents less than 2% of all cases, as demonstrated in a large Mayo Clinic study.7 Little is known about the biological behaviour of phalangeal chondrosarcoma and controversial data are reported in the literature.8 have previously reported that Evans’ grading could not be applied to chondrosarcoma of the hand in predicting disease-free survival, because it appears to display a different behaviour from chondrosarcomas located elsewhere in the body. In addition, the radiological and histological findings by themselves are not always reliable in distinguishing phalangeal chondrosarcoma from enchondroma. Thus, a combination of clinical, histological and imaging features are important to differentiate low-grade chondrosarcomas from enchondromas of the hand.Chondrosarcomas of the hand usually display benign biological behaviour even when they demonstrate cytological and imaging features suggestive of malignancy. However, some cases, as reported in the literature, showed local recurrence and could metastasize.2 Bovèe et al8 reported a recurrence rate of 36% and it was likely owing to the low proportion of radical surgical treatment performed in their study.8 There is a relative lack of vascularity in cartilage tumour matrix and the neoplastic cells appear to depend on diffusion for nourishment. This probably allows cartilaginous lesions to be easily transplantable lesions, giving rise to recurrences.15 Chondrosarcomas metastasize usually by the haematogeneous route and rarely by the lymphatic route. Metastases occur when the tumour erodes the vein wall and forms intravenous plugs; neoplastic thrombi then develop within the venous tree and migrate to the lung. Thus, metastases are nearly always pulmonary and rarely to other sites, including the skeleton or the skin, in the absence of pulmonary metastases.16 Metastases can, however, occur after a significant period of time ranging from a few months to many years after the onset of symptoms.Similar to chondrosarcomas located elsewhere in the body, the incidence of local recurrences in chondrosarcoma of the hand relates to surgical treatment.17 involved a middle phalangeal chondrosarcoma with cutaneous and pulmonary metastasis. This patient developed multiple nodules in the skin of the face, neck and trunk. Karabela-Bouropoulou et al11 have reported a case of thumb chondrosarcoma with pulmonary and subcutaneous metastasis that developed 6 years after the initial diagnosis. Although many studies11 and the present report demonstrated a very aggressive behaviour and both patients died soon after the appearance of cutaneous metastases.Most previously reported chondrosarcomas metastatic to the skin originated in the bones of the extremities. These metastases can be either single or multiple with a slight predilection for the head and neck region. A review of the published papers of skeletal chondrosarcomas metastatic to the skin yielded a total of nine cases.18 In this case, the author suggested strong clinical evidence in keeping with mechanical tumour transplantation at the site of Bier block access for anaesthesia, which later developed into cutaneous metastasis. The Bier’s block is an intravenous regional analgesia and is best used for brief minor surgery of the hand and the forearm. An intravenous cannula or butterfly needle is inserted in a distal vein in the limb scheduled for surgery. A tourniquet is then applied to the upper arm. In our case, we could not identify any reason for direct tumour transplantation and our patient had a general anaesthetic for biopsy and resection rather than regional anaesthesia. However, in our patient, a blood pressure cuff was placed in the ipsilateral limb above the elbow, but the limb was never exsanguinated. The subcutaneous metastasis developed just distal to the site of the cuff. In addition, the skin metastasis was related closely to the cephalic vein, which drains the radial part of the dorsal network of veins of the hand, in particular the thumb and the index finger. Although unproven, it is plausible that there may have been venous dispersal of the tumour at surgery or during the open biopsy through the cephalic vein that lodged itself in the subcutaneous tissue just distal to the blood pressure cuff owing to the occlusive pressure on the veins by the cuff. Of course, the metastatic seedling could also have occurred prior to surgery owing to venous invasion by the tumour itself.As described above, a satisfactory wide resection of hand chondrosarcoma is usually associated with a very good long-term prognosis.We present a rare case of cutaneous metastasis in the ipsilateral upper limb from a metacarpal chondrosarcoma and suggest a plausible cause for its occurrence. The potential for metastases of chondrosarcomas of the hand is much lower than for chondrosarcomas of other parts of the body. Additional and more specific studies are necessary to clarify the behaviour of chondrosarcomas of the hand related to different histological subtypes. However, the authors believe that for grade II and III chondrosarcomas, wide excision rather than conservative treatment should be preferred in order to avoid local recurrence or metastasis, and adequate precautions to prevent transplantation of the tumour cells to other sites during surgical procedures are necessary. Since metastases can develop long after the initial diagnosis, these tumours should be followed up very closely.This is a very rare and unusual case of metacarpal chondrosarcoma with subcutaneous and pulmo-nary metastases.Hand chondrosarcomas are seldom metastatic in nature. Death is virtually unknown. Pulmonary metastases from hand chondrosarcomas are rare but not unknown.For higher grade chondrosarcomas of the hand, excision/ray amputation is probably a better treatment than curettage.This article also shows a rare occurrence of subcutaneous metastases from small bone chondrosarcoma, which is relatively unknown.It is plausible that inflatable cuffs, such as blood pressure monitoring devices and tourniquets, may have a role in determining where metastatic deposits may occur.