Primary abdominal “egg-shelled” lesions with positive anti-echinococcus IgG antibody were misdiagnosed as echinococcosis.Case 1: A 44-year-old woman presented to our department with a history of slight abdominal pain for 4 months. After admission, the laboratory tests indicated a positive anti-echinococcus IgG antibody status. The contrast-enhanced CT scan showed a 12 × 12 cm “me contrast-mass located in the hepatorenal area. The patient had the entire mass and the right adrenal gland resected. This patient recovered smoothly and was discharged uneventfully 20 days after the operation. The pathologic diagnoses was adrenal lymphangioma. Case 2: A 55-year-old woman was admitted with a history of an abdominal mass for over 10 years. After admission, her anti-echinococcus IgG antibody was positive. The contrast-enhanced CT scan revealed a heterogeneous, solid mass measuring 10 × 9 × 8 cm in the right hepatic lobe. A laparoscopic exploration was performed, and the surgery revealed that the mass arose from the retroperitoneal tissue rather than the liver. Finally, the pathologic diagnoses were paraganglioma with necrosis and cystic changes.Herein we report two cases with primary abdominal “egg-shelled” lesions were misdiagnosed as echinococcosis. Enhanced CT scan and contrast-enhanced ultrasound scans are important for distinguishing echinococcosis disease from the other “egg-shelled” lesions. Surgical resection is the main treatment method for this disease. Minimally invasive surgery is recommended but should be performed by experienced surgical teams. Immunohistochemical examination is important for the pathologic diagnosis. The “egg-shelled” lesion is a mass with an eggshell-like calcified wall, characterized by homogeneous intracapsular necrotic material, that will not or will only be slightly enhanced on contrast-enhanced computed tomography (CT) scans. Many primary lesions could present as egg-shelled lesions with calcification including retroperitoneal pseudocyst, adrenal tuberculosis, pelvic schwannoma, and acute calcific retropharyngeal tendinitis –5. As a Echinococcus granulosus) and alveolar echinococcosis   . Based ocularis) . Howevercularis) .Herein, we present two cases that had “egg-shelled” lesions that were misdiagnosed as hepatic echinococcosis. As abdominal “egg-shelled” masses are rare and can be present in different diseases, including echinococcosis, the purpose of this paper is to present our experience with treatment and to reduce the misdiagnosis rate. This work was supported by the Ethics Committee of Sichuan Provincial People's Hospital.A 44-year-old woman presented to our department with a history of slight abdominal pain for 4 months. She had a long history of living in an area with echinococcosis, and there was no significant familial medical history for this patient. The medical examination found a mass located in the middle right abdominal region with tenderness and unclear boundaries.9/L, (references 3.50–9.5 × 109/L)] and absolute eosinophil counts , the serum tumor markers were normal and a positive anti-echinococcus IgG antibody status. The contrast-enhanced CT scan showed a 12 × 12 cm “egg-shelled” mass located in the hepatorenal area  [4.74 × 10nal area . This manal area . The priThe resected specimen had a calculated shell , which w9/L) and absolute eosinophil counts (0.32 × 109/L), and the other significant index were negative, except her serum anti-echinococcus IgG antibody was positive. The contrast-enhanced CT scan revealed a heterogeneous, solid mass measuring 10 × 9 × 8 cm in the right hepatic lobe  are also important for distinguishing echinococcosis disease from others. In advanced AE, the cystic components are comprised of metacestodal vesicles and have liquefactive necrosis; the solid components have coagulation necrosis and calcifications with no or little microcirculation. After the injection of a contrast agent, the CEUS shows a nonenhancement, similar to the black hole effect . At the Radical surgical resection is needed for both echinococcosis and other “egg-shelled” lesions. Laparoscopic surgery is a safe technique for radical resection and has been utilized for years. However, if the adjacent tissue  is easily to be touched during laparoscopic surgery causing serious consequences, laparoscopic mass resection should be recommended. At the same time, the opening of “egg-shelled” cysts for pressure reduction is not recommended by previously reported cases. For some CE cases presented as In conclusion, “egg-shelled” lesions are rare diseases, and most of them are benign diseases mimicking hepatic echinococcosis. Accurately differentiating nonparasitic lesions from echinococcosis disease is important for surgical planning. Surgical resection is the main method for the treatment of “egg-shelled” lesions. Minimally invasive surgery is recommended and should be performed by experienced surgical teams. Immunohistochemical examination is important for the pathologic diagnosis.


Injuries to sporting dogs are becoming frequent, and precise knowledge of all of the anatomical structures involved in sport-related traumas is mandatory to achieve the optimum treatment for each case. In this case report, injury to the common digital extensor tendon of digits III and IV of the forelimb is reported for the first time, highlighting analogies with a similar pathology described in human athletes, called boxer’s knuckle.Injuries to canine athletes are becoming more and more frequent, and perfect knowledge of all injury-prone anatomic structures is mandatory to achieve correct diagnoses and treatments. In this small case series discussion, trauma-based injury to the common digital extensor tendon of digits III and IV of the forelimb is described for the first time. The anatomy as well as the clinical and ultrasonographic findings show similarities to the human spectrum of pathologies called boxer’s knuckle. The treatment options with a buddy taping technique led to a satisfactory outcome at three months from trauma, without a history of re-injury. Injuries to the extensor compartment of the forepaw should be considered in cases of forelimb lameness with dorsal metacarpophalangeal swelling in sporting patients, especially with a history of blunt force traumas. Injuries of the common digital extensor tendon (CDET) in sporting dogs are not described in veterinary literature, but the detection of swelling of the soft tissues at the dorsal aspect of the metacarpo-phalangeal (MCP) joint, in association with different degrees of lameness as a result of trauma occurred during sporting activities, suggests the involvement of this anatomical structure and some analogies with a pathology, described in human athletes, called “boxer’s knuckle”.The initial preliminary reports of boxer’s knuckle in human medicine date back to 1957, when Gladden described this pathology for the first time, reporting it in four cases .The common digital extensor muscle arises on the lateral epicondyle and runs distally, passing through the lateral distal sulcus of the radius. Its distal CDET divides into four distinct tendons, progressing distally after crossing the dorsal surface of the carpal joint. Each tendon, from digit II to digit V, glides on the roll-like dorsal part of the corresponding metacarpal bones and is deeply embedded in the fibrous tissue of the digits. A sesamoid bone, not present in all dogs, is embedded in the tendon at the level of the MCP joint. At the distal end of the proximal phalanx, each tendon bilaterally receives a thin extension of the tendon of insertions of the interosseous muscles that crosses obliquely from the palmar surface .In people, the dorsal hood is a retinacular structure that stabilizes the digital extensor tendon at the dorsal aspect of the MCP joint, keeping the tendon in place during flexion and extension. The sagittal band is the most important part of the dorsal hood, and its function is to maintain the stability of the extensor tendon . RuptureTo date, no descriptions of injuries of the CDET are available in the veterinary literature and, to the best of the authors’ knowledge, this is the first case report about this topic.In the present small case series discussion, injury of the common digital extensor tendon and its peritendinous soft tissues of three sporting dogs is described, and similarities to the human condition called boxer’s knuckle are described.All of the following cases were presented to the Sporty Dog Veterinary Clinic . All of the orthopedic evaluations and the radiographic assessment were double checked by two expert veterinary orthopedics (A.F. and M.G.E.). The lameness assessment for each case was based on the previously published evaluation system and is reported in Radiographic examinations of both manus on the dorsopalmar and mediolateral standard views were performed. All of the radiographic examinations were performed under general anesthesia conducted according to a previously published protocol for orthopedic examinations. Dexmedetomidine (5 µg/kg) and butorphanol  intravenously (IV), followed by propofol (1–4 mg/kg IV to effect) were used. The owners agreed to the procedure by signing a written informed consent form . All of A 17-month-old female Whippet competing in lure-coursing activity presented with an episode of lameness to the right forelimb. Symptoms had been observed for two days. The lameness occurred during a lure-coursing performance and blunt trauma on uneven terrain was reported by the owner.Upon clinical examination, the dog was found to be in good general health. Orthopedic examination showed no lameness at a walk, but grade two lameness was detected at a trot and gallop. Swelling of the dorsal aspect of the IV MCP joint was detected and pain at palpation of the swollen region was evident. Pain at passive forced complete flexion and extension of digit IV at the level of the MCP joint was also detected. Flexion and extension of the MCP joint were reduced. No signs of instability were detected at the palpation on the lateral nor on the medial aspect of the MCP joint and all of the interphalangeal joints of the affected digit.No changes were detected at the radiographic examination.The US exam showed swelling of the peritendinous soft tissues on the dorsal aspect of the MCP joint of digit IV, with normal echogenicity of the digital extensor tendon. Disruption of the peritendinous fibrous tissue was evident at the level of the joint with irregular echogenicity of the surrounding tissue. A clear anechoic layer was present around the tendon that appeared grossly intact. At the dynamic evaluation, with passive flexion and extension movement, the tendon was always in a physiological position. Thus, no luxation or subluxation of the tendon were suspected. The CDET and the peritendinous soft tissues of digit III were within normal limits from the ultrasonographic point of view. Based on the US findings, a suspected diagnosis of lesion of the peritendinous soft tissues on the dorsal aspect of the MCP joint was considered.A male Rhodesian Ridgeback aged six years and four months old competing in canicross activity, presented with recurrent lameness to the left forelimb. Symptoms had been observed for two months. An improvement with rest was reported by the owner and worsening with sporting activity was also reported. The first episode of lameness occurred the day after a canicross performance and the owner referred to a blunt trauma against a root found on the racetrack.Upon clinical examination, the dog was found to be in good general health. Orthopedic examination showed no lameness at a walk, but grade two lameness was identified during a trot and gallop. Swelling of the dorsal aspect of the IV MCP joint of the left manus was detected and mild pain at palpation of the swollen region was present. A slight swelling of the dorsal region of the MCP joint was also observed at the level of digit III and no pain was detected on palpation of this region. Pain at passive forced complete flexion and extension of digit IV at the level of the MCP joint was present, whereas no pain at the same manipulation of digit III at the level of MCP joint was observed. The range of motion of the MCP joint was reduced in the flexion and extension of digit IV, whereas it was apparently within normal limits for the MCP joint of digit III. No signs of instability were detected on the lateral nor the medial aspect of the MCP joint and of the interphalangeal joints of the involved digits.No significant changes were detected at the radiographic examination.The US exam showed digit IV swelling of the peritendinous soft tissues on the dorsal aspect of the MCP joint, with normal echogenicity of the digital extensor tendon. Disruption of the peritendinous fibrous tissue was evident at the level of the joint, with irregular echogenicity of the surrounding tissue. At the dorsal aspect of the MCP joint of digit III, an irregularly echogenic area was present around the CDET that was intact, with normal echogenicity and no evidence of core lesions. A clear anechoic layer was present around the tendons of digits III and IV. At the dynamic evaluation, with passive flexion and extension movement, clear snapping of both tendons was not detectable. Thus, no luxation or subluxation of the tendons were suspected. The CDET and the peritendinous soft tissues of digit II were within normal limits from the ultrasonographic point of view.Based on the US findings, a suspected diagnosis of lesion of the peritendinous structures on the dorsal aspect of digit III and IV at the level of MCP joint was considered.A male Shetland Sheepdog aged eight years and two months old involved in high-level agility competitions presented with persistent lameness to the right manus. The lameness had occurred two weeks prior, and subsequent to an agility competition. Trauma involving a tunnel approach during the performance was reported by the owner. No improvement with rest was achieved and worsening at the return-to-work was observed.Upon clinical examination, the dog was found to be in good general health. Orthopedic examination showed no lameness at a walk, but a grade two lameness was identified at a trot, worsening to grade three at a gallop. Clear swelling of the dorsal aspect of the MCP joint of digit IV of the right forelimb was observed, with pain at palpation of the swollen region. Pain at passive forced complete flexion and extension of digit IV was detected and a clear snapping sensation at the gliding of the tendon in complete flexion was noticed . The ranRadiographic examination was within normal limits.The US exam showed digit IV swelling of the peritendinous soft tissues on the dorsal aspect of the MCP joint, with normal echogenicity of the CDET. Disruption of the peritendinous fibrous tissue was also detected at the level of the joint, with irregular echogenicity of the surrounding tissue. The CDET of digit IV was intact, without signs of core lesions. A clear anechoic layer was present around the tendons of digit IV. At the dynamic evaluation, with passive flexion and extension movement, clear snapping of the CDET of digit IV was detectable and the tendon shifted medially slightly during complete flexion of the joint. The CDET and peritendinous soft tissues of digit III were within normal limits from the ultrasonographic point of view. Based on the US findings, a subluxation of the CDET of digit IV with lesion of the peritendinous tissues was suspected.A conservative approach was chosen as the treatment option for all three cases. The involved digit was immobilized in extension for four weeks using a “buddy taping” technique, according to previously published literature . In the In the present case report, three cases of lesions to the extensor compartment of the paw, at the level of MCP joint, are described. The lesions affected three sporting dogs and occurred during the sport activity. The clinical presentation and the diagnostic findings of the injury seem to share many characteristics with the spectrum of lesions of the digital extensor tendon described in human medicine and called boxer’s knuckle.The veterinary literature about the damages of the surrounding tissues of the CDET in dogs is scarce, with only a few references regarding horses in which injuries to several structures of the dorsal aspect of the distal forelimb are described, possibly because of their superficial location. The vulnerable structures include the CDET and lesions reported comprise infections and inflammations mainly caused by direct blunt trauma . In sporTo date, no reports of damages to the surrounding structures of the extensor tendons of the forepaw in canine athletes have been published and this is, to the best of the authors’ knowledge, the first report of sport-related damages to the CDET in dogs.The CDET of dogs crosses the extensor surface of the carpal joints dividing the few tendons that pass over the dorsal surface of the corresponding metacarpal bones, reaching the distal phalanxes of digits II to V, inclusively. Dorsally to the MCP joint, a sesamoid bone, not always present, can be embedded in the tendinous structure. Each tendon, at the end of the proximal phalanx, is reached by a thin extension of the tendon of insertion of the interosseous muscles that cross obliquely from the palmar surface . This smBoxer’s knuckle was first described in human patients in 1957 by Gladden, indicating a spectrum of soft tissue injuries to the dorsal aspect of the hand, secondary to single or repetitive trauma to the metacarpophalangeal joint and the extensor mechanism of the hand and mainly involving the sagittal band . Three dIn human studies, the mechanism of injury is reported to be mainly related to direct blunt traumas, but the concurrence of forced flexion and resisted extension seems to have a predisposing role in the occurrence of the lesion. Thus, sports requiring gripping or grasping, such as tennis, also seem to be more problematic among human athletes . The supThe clinical signs observed in the study population of the present case report also seem to share similarities with those described in humans. Dogs showed swelling at the level of the affected MCP joint. Pain at palpation and at full passive forced extension and flexion of the affected digit was also detected. Clear instability of the tendon was present in case 3, whereas case 1 and case 2 exhibited no clear luxation. In people, the clinical presentation is reported to include pain, swelling, and tenderness of the involved anatomical site. In some cases, tendon instability is also present. Active movements of the MCP joints show limitations. Pain increases with full active and passive flexion of the joint. Based on the observed clinical findings, the clinical presentation in dogs would likely seem to be correspondent to that observed in humans.Diagnoses in human patients are performed with clinical examination with the recognition of all the clinical signs reported above. Radiographic assessments usually show no relevant radiographic signs. The help of other imaging techniques is considered useful, with dynamic high-resolution US of the clenched fist considered to be a reliable tool for the diagnosis of injuries of the extensor hood mechanism . Given tTreatment options in dogs are not reported in the literature. In human athletes, nonsurgical management has been reported from Kleinhenz et al. for the treatment of most type I and type II injuries, which are those without clear tendon luxation, as well as most of the type III injuries. In those cases, a buddy taping technique is applied to the involved finger for four to six weeks. In humans, surgical management is reserved to cases that do not respond to conservative treatment and to cases with chronic clear tendon luxation . Based oInjuries to the extensor compartment of the MCP joint in sporting dogs are not reported in the literature; this paper presents the first description. The anatomy of the involved structures shows similarities between dogs and humans, although considering specific obvious differences. The clinical signs and the US findings observed in the patients described in this case report also suggest an analogy with the pathology reported in human patients and called boxer’s knuckle. As in humans, in dogs too, the pathogenesis of the lesion seems to be mainly related to the occurrence of blunt force traumas during the sporting activity. Biomechanical factors related to the load on the central digit of the manus are supposed to have a role in the occurrence of the lesion. The treatment options are not described in literature and, for the three described cases, a conservative approach already described for digital tendons injuries in dogs with a “buddy taping” technique was chosen, with a satisfactory outcome at three-month follow-up .In conclusion, this small case series suggests to consider lesions to the CDET at the level of MCP joints among the differential diagnosis of thoracic limb lameness in canine sporting patients involved in sports that are prone to blunt trauma. Although no conclusions about treatment can be withdrawn from this small case series and considering that further studies are necessary to better understand the involvement of the extensor compartment of the forepaw in sport-related injuries in canine athletes, this first description of CDET lesion and its treatment in sporting patients gives a new insight to the growing body of sporting dog literature.


Case Report. A 50-year-old woman became aware of a painful, deep scar mass in her lower abdomen during her menstrual period 10 years after her second cesarean section. This was diagnosed as CSE after the biopsy. Under general anesthesia, the mass, a portion of the rectus abdominis, and a 1 cm tumor-free margin were resected as a whole, and the abdominal wall was reconstructed with a soft artificial mesh.  Currently, there are few reports describing the use of reconstructive techniques in the treatment of cesarean scar endometriosis (CSE). Here, we report a case of CSE, a rare form of endometriosis caused by scars from obstetric and gynecological surgeries. Discussion. Endometriosis appearing in a cesarean scar is rare; it is chiefly triggered by intraoperative mechanical implantation. In cases of surgical scar masses with a history of gynecological surgery and associated menstrual symptoms, this syndrome should be considered during diagnosis and treatment. No obvious recurrence or subjective symptoms were observed postoperatively or reported in the 1-year follow-up period.  Endometriosis is a benign gynecological disease characterized by the ectopic presence of active endometrial tissue outside the uterine cavity. Endometriosis occurs most commonly in the abdominal cavity, mainly in the ovaries, peritoneum, uterine ligament, and rectovaginal septum. Among cases of endometriosis occurring in the abdominal wall, those occurring in postcesarean scars are relatively rare, estimated to account for approximately 0.08% of all cases of endometriosis . HoweverA 50-year-old woman presented with a painful lower abdominal mass that appeared during her menstrual cycle 10 years after her second cesarean section. The patient had a history of two cesarean sections, and no history of drinking, smoking, or taking medication. Magnetic resonance imaging (MRI) performed at the first hospital suggested a desmoid tumor; therefore, she was placed under observation. However, the size of the mass increased, and her subjective symptoms worsened; consequently, she visited the gynecology department at our hospital. The high levels of CA19-9 (152.21 U/mL) and CA125 (48.0 U/mL) in a blood test, in addition to her symptoms, led to the suspicion of endometriosis. We performed an excisional biopsy, which confirmed the diagnosis of endometriosis. Hormone therapy using oral luteinizing hormone was commenced but discontinued because of nausea. Surgery was planned for a radical cure. At the time of the visit, a mature scar with a transverse incision was found in the lower abdomen, and a golf ball-sized hard mass was palpable under the scar. The mass was partially adherent to the overlying skin and firmly adherent to the underlying rectus abdominis, and there was no skin redness or heat . MRI shoHistopathological examination of the excised specimen revealed endometrial tissue, hemosiderin, phagocytic histiocytes, and granulation tissue; hence, the patient was diagnosed with endometriosis . The patScar endometriosis is rarely reported in the gynecological literature and occurs in patients who have previously undergone obstetric and gynecological surgeries such as cesarean section, hysterectomy, perineal incision, and tubal ligation , 8. PrevAlthough many theories have been postulated as to the cause of CSE, the most accepted theory is direct iatrogenic implantation of the endometrium into the wound margin during abdominal or pelvic surgery , 12.Only approximately 20% of all patients with CSE show classic endometriosis symptoms of menstrual pain and accompanying changes in tumor size . More ofAppropriate surgical treatment offers the best opportunity to make a definitive diagnosis and treat CSE. Resection should include a tumor-free margin of at least 1 cm around the solid tissue . In addiHormonal treatments such as gonadotropin-releasing hormone agonists, danazol, and progesterone have been used and provide only temporary relief, and symptoms often recur with discontinuation . HoweverOur case demonstrates that accurate diagnosis of CSE, complete resection, and reconstruction with artificial mesh, can lead to good treatment outcomes and patient satisfaction. However, the long-term postoperative results cannot be determined at this time and require careful follow-up in the future.Given the rarity of CSE, its diagnosis may be delayed, leading to frustration for both patients and physicians. CSE should be a differential considered by all surgeons. Proper mass resection and reconstruction of the abdominal wall where necessary are important for complete diagnosis and treatment.


Chondroid syringoma is a cutaneous adnexal tumor originating from sweat glands origin. It is rare in occurrence and usually benign, having an incidence of 0.01 to 0.098%. As these tumors are uncommon, their diagnosis is missed many times and are misdiagnosed. Hence in any case of facial skin swelling increasing slowly in size, this entity should be kept in mind as one of the possibilities and differential diagnosis. Histopathological examination of the excision biopsy gives the definitive confirmatory diagnosis. Surgically excising the swelling locally along with a surrounding normal tissue cuff is the standard treatment given which prevents recurrence. Hereby we present a 35-year-old case of facial chondroid syringoma having a focal component of eccrine hidrocystoma, keratinous cyst as well as syringocystadenoma papilliferum on the chin that was clinically suspected to be an epidermoid cyst or mucocele.  Chondroid syringoma (CS) is a skin adnexal tumor with a very low incidence ranging from 0.01% to 0.098% of all the primary skin neoplasms. It is a sweat gland tumor, usually benign, alias mixed tumor of the skin, comprised of epithelial as well as mesenchymal elements . They usClinically it presents as a firm, nodular lesion which is painless, increasing slowly in size, attached to the overlying skin but not the deeper tissue . CS is aA 35-year-old male presented with a history of swelling over the chin below the lower lip measuring 1.5x1.0 cm. It was non-tender and soft to firm in consistency. The overlying skin was greyish-brown in colour and adherent to the swelling. Intraoral examination showed no vestibular edema and no obvious intraoral pathology. The swelling had been growing steadily for the past two years, but remained asymptomatic otherwise. The patient sought treatment only for cosmetic reasons and had consulted at various hospitals previously where it was labelled as folliculitis and treated accordingly. However there was no improvement, so he came for consultation in the Outpatient Department (OPD) of Dermatology in our hospital. Herein the probable clinical diagnoses were either epidermoid cyst or mucocele. Fine needle aspiration cytology (FNAC) was performed. Blood mixed with sticky fluid was aspirated. Smears showed abundant macrophages, scattered mature squamous cells, anucleated squames, few neutrophils and lymphocytes in hemorrhagic and keratinous background Figure . This waAn excision biopsy was performed which was grossly soft to firm, and nodular measuring 1.5x1.3x1 cm with a greyish-brown outer surface. On cut section it was partly solid and partly cystic Figure . MultiplSections examined showed a well-circumscribed and unencapsulated benign adnexal tumor composed of cells arranged in solid cords, clusters as well as forming ductal structures showing focal branching and lined by two layers, inner cuboidal and outer cuboidal to flat myoepithelial cells Figure . There wHence it was diagnosed to be a benign adnexal tumor of the skin with features suggestive of chondroid syringoma (mixed tumor of skin) with apocrine as well as focal eccrine features. A component of eccrine hidrocystoma, keratinous cysts and syringocystadenoma papilliferum were also present.Thereafter patient was evaluated one week postoperatively. There was appropriate local healing without any complication. The patient was explained about the need for regular follow-up visits to monitor recurrence. For the past four months the patient is being observed regularly showing complete healing and no signs of recurrence or complications.CS or mixed tumor of the skin is an unusual, benign, cutaneous appendageal tumor having both epithelial as well as mesenchymal components . In 1859CS is an uncommon lesion having male predominance usually in middle or old age ,16. MaleIt is most frequently localised at the nose, cheek, upper lip, scalp, forehead, and chin in the head and neck region ,10. NasoThe tumor is commonly erythematous, violet or skin coloured, firm, intradermal or subcutaneous, nodule measuring 0.5 to 3 cm in size, usually asymptomatic and growing slowly -27. HoweAs a case of CS usually has a firm, round, movable nodule which is going slowly and frequently causes no symptoms, it can be readily interpreted as an epidermal cyst clinically . NeverthHowever, FNAC can be done pre-excision, especially in cases with unusual presentation, to diagnose CS. But it has limitations, as CS is a mixed tumor and may show multiple components, and so depending upon the area which is aspirated, only one component can be seen on aspirates. These sampling errors can lead to a misdiagnosis . Like inHistopathologically CS is well-circumscribed with epithelial, myoepithelial, and stromal components . The morHistologically, our case fulfilled all the five characteristics of CS as there was presence of nests of polygonal cells; intermixed tubulo-alveolar structures encircled by two and more layers of cuboidal cells; ductal structures lined by one or two layers of cuboidal cells; keratinous cysts; and a chondromyxoid matrix. CS may have few or all of these five characteristics . The tumWe studied this case as the clinical diagnosis as well as the FNAC findings were different from the definitive diagnosis on biopsy. The reasons for the same were evaluated and we concluded that this uncommon lesion should always be kept in mind during the clinical as well as cytopathological evaluation. The morphological findings in this case of chondroid syringoma included similar findings to the cases reported earlier in literature. But additional foci of eccrine hidrocystoma as well as syringocystadenoma papilliferum were also identified. In the literature it is mentioned that multiple components to other tumors can be seen in chondroid syringoma, but such a case with various components is rarely reported. And hence it should be remembered that the diagnosis may be missed on FNAC if a single component is aspirated.The unusual malignant variants increase to a size of more than 3cm and show local invasion. The signs of malignancy on histopathological examination are cytologic atypia, tumor necrosis, infiltrative margins, satellite tumor nodules, and invasion of the deep structures ,34. RegiImmunohistochemistry (IHC) can be used as an adjunct to support the diagnosis of CS. The tubuloalveolar elements show immunopositivity for cytokeratin (CK), epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), S-100, neuron-specific enolase (NSE), vimentin, and sometimes glial fibrillary acidic protein (GFAP) ,35. The There was a limitation in the number of cases of CS. If we had more cases, we could have studied various clinical presentations and morphological findings in our region. Also, we were unable to do adjunctive immunohistochemistry due to technical considerations.Treatment of choice for benign CS is surgical excision with a surrounding cuff of normal tissue without affecting functional and esthetic and structures . ElectroAs seen in our case, FNAC may mislead in the diagnosis of CS due to its variable components. Excision biopsy is a must for final diagnosis of CS as we can encounter multiple components comprising other lesions as well. Like in our case we encountered a focus of eccrine hidrocystoma, keratinous cysts as well as syringocystadenoma papilliferum. These case findings fit within the existing literature. Hence, in spite of being a rare entity, CS should be considered one of the differential diagnoses of cutaneous nodular swelling in the head and neck region. It is imperative to go for complete excision of the lesion along with a surrounding cuff of normal tissue. Missing the morphological diagnosis can lead to an incompletely excised tumor followed by recurrence. A frequent follow-up of the case is essential even after its complete removal, as it is susceptible to malignant transformation.


We present a case of a neonate who presented with multiple cutaneous and subcutaneous nodules, which was found to be metastatic embryonal rhabdomyosarcoma. Rhabdomyosarcoma is a soft tissue malignancy that usually occurs in children aged one to five but is rare in neonates. The histopathological analysis and molecular genetics are important in the classification of subtype and in guiding treatment options and informing prognosis. A three‐week‐old infant presented with rapidly developing pink/erythematous to yellow/brown cutaneous and subcutaneous papules and nodules varying in size from 0.4 to several centimetres. The first lesion was noticed by his parents at 1 week of age. At the presentation to our dermatology clinic review at 6 weeks of age, the patient had numerous cutaneous and subcutaneous lesions Figure . The nodInitial investigation involved taking multiple biopsies of the back, debulking of an ear lesion and laryngoscopy to exclude airway nodules under general anaesthetic. Histopathology Figure  showed aMRI brain, whole body and USS renal tract were performed, which showed extensive disseminated metastatic disease with innumerable muscle, cutaneous, subcutaneous tissue nodules and multiple osteolytic lesions most prominent in the right third metatarsal, but no central nervous system involvement Figure . The diaRhabdomyosarcoma is rare with an incidence of 5 in 1 million in children and very rare in neonates.There is variability in the classification of rhabdomyosarcoma with multiple credible classification systems and diagnostic methods. The minimum requirements for diagnosis include morphologic evaluation by H&E staining, and Myogenin, desmin and MYOD1 immunohistochemistry.The two main rhabdomyosarcoma subtypes are embryonal and alveolar, with embryonal being the most common in children, and especially in age groups <5 years.While around 85% of alveolar RMS contain the FOXO1 rearrangement, a small number of alveolar RMS cases lack this rearrangement and so it must be considered that this case is a fusion negative metastatic alveolar RMS, given the strong myogenin/MYOD1 staining. There have been reported cases in the literature of congenital cutaneous RMS with both positive FOXO1 fusion, supporting a diagnosis of alveolar subtype, as well as without FOXO1 fusion supporting a diagnosis of embryonal RMS.The patient received chemotherapy under oncology specialist supervision with vincristine, dactinomycin and cyclophosphamide (ARST1431 Regimen A ‐VAC only) and had treatment for multiple other complications including feeding support, otitis externa, urinary obstruction, hypertension, and a hypoxic brain injury as a complication of an infection. An MRI scan completed 6 weeks after the start of chemotherapy demonstrated dramatic reduction in the extent of metastatic disease, where the soft tissue lesions, including the subcutaneous tissue, intramuscular, pulmonary, pleural, penis, renal and nodal disease had resolved. Unfortunately, weeks after this last cycle of chemotherapy was completed the patient presented with signs of hydrocephalus and was found to have extensive leptomeningeal disease. Neurosurgery performed a VP shunt and referred to palliative care and sadly the patient died 1 month later.The authors declares that there is no conflict of interest.


SYT-SSX fusion gene. Due to the disease’s rarity and severity, diagnosis and management of PRSS rely upon a demanding and multidisciplinary approach.Primary renal synovial sarcoma (PRSS) is an extremely rare malignancy. The diagnosis of PRSS is unforeseen due to the absence of clinical and radiological typical aspects. Here, we present a case of a 69-year-old male with complaints of hematuria and left lumbar pain. Abdominal-pelvic computed tomography scan with contrast injection showed a solid mass of 8cm diameter in the left kidney and renal vein tumor thrombus. The patient was further subjected to robotic-assisted left radical nephrectomy and renal vein thrombectomy. We concomitantly performed left adrenalectomy and paraaortic lymphadenectomy. Immunohistochemical and genetic analysis revealed PRSS. This entity is characterized by abnormal chromosomal translocation t and consequently the characteristic  Synovial sarcoma (SS) is a soft tissue sarcoma that typically occurs in young adults . This co2. Slight abdominal tenderness more pronounced on the left upper quadrant was detected, but there was no evidence of palpable mass. Hematological investigations showed a hemoglobin level of 15.8 g/dL and urea and creatinine levels of 5.16 mmol/L and 102.55 μmol/L, respectively. The estimated glomerular filtration rate was 64 mL/min/1.73m2. Computed tomography (CT) scan imaging with contrast of the abdomen and pelvis showed a large hypodense mass of 8cm diameter in the left kidney and a thrombus in the left renal vein . The patient had undergone robotic-assisted left radical nephrectomy through the transperitoneal approach with distal milking of the thrombus to the renal vein and paraaortic lymphadenectomy. Adrenalectomy was also performed. The procedure had no evident complications. The entire operative time was 170 minutes. Estimated patient’s blood loss was 200ml. After surgery, the patient was discharged on the fourth postoperative day. There were no intercurrences in the postoperative evolution.Macroscopic examination revealed a solid brownish-white tumor with 8.7cm in greatest dimension. The tumor invaded the pelvicalyceal system and extended to the left renal vein. A tumor thrombus with 2.5cm diameter was detected. Surgical margins were negative. Microscopic examination showed a monomorphic population of spindle-shaped cells with scant cytoplasm, mild atypia, and conspicuous mitotic activity, with a fascicular growth pattern and areas of necrosis Figures , 4. Immu2, the patient presented progressive worsening of his condition and died. After surgery, an intensive follow-up protocol was initiated. Chest-abdominal-pelvic CT scan was performed every three months, with no evidence of metastasis until nine months after surgery, where widespread metastatic disease was detected in the liver, pancreas, peritoneum, and lungs. This case was discussed at multidisciplinary evaluation, and doxorubicin was proposed and accepted by the patient. However, after one cycle of doxorubicin 75mg/mOverall, sarcomas of the kidney are diagnosed in 0.8-2.7% of patients with malignant kidney tumors . LeiomyoThe absence of pathognomonic findings makes the diagnosis of PRSS demanding. However, CT scan and magnetic resonance imaging (MRI) may provide useful information. In a retrospective study with five patients carried out by Lv et al., either a well-defined solid-cystic mass with heterogeneous contrast enhancement or a cystic lesion with septal enhancement can be observed . AdditioHistologically, PRSS classification is similar to SS in other locations. Macroscopically, this tumor presents as a large solid mass with areas of cystic changes, hemorrhage, and necrosis . At a miPatients diagnosed with PRSS should be referred to a tertiary center with an experienced multidisciplinary team specialized in soft tissue sarcoma . Due to PRSS is an extremely rare entity. Considering clinical and radiologic features, PRSS is not easily distinguishable from other renal tumors. Typical aspects of immunohistochemical and cytogenetics evaluation demonstrating an abnormal chromosomal translocation t and SYT-SSX gene fusion proteins are key for diagnosis. Radical nephrectomy is the treatment of choice. Adjuvant chemotherapy should be only considered in selected patients since there are few reporters available in the literature. Therefore, more studies are needed to better understand the role of adjuvant treatments. Multidisciplinary evaluation is recommended in order to ensure the optimal health care plan for the patient. However, the prognosis remains fairly poor.


Chordoma is a rare tumor, often occurring in the cervical spine and sacrococcygeal spine with a lytic appearance, but rarely in the thoracolumbar spine. Chordomas can occasionally be sclerotic and are included in the differential diagnosis for an ivory vertebra. We present a case of a sclerotic chordoma in an upper lumbar vertebral body with corresponding multimodality imaging. This case demonstrates that chordoma should be a concern for an older adult with a sclerotic vertebral lesion, particularly if it is a solitary lesion. Knowledge of the variable location and appearance of chordomas is critical so it is not mistaken for a metastasis. Chordoma is a rare, slow-growing, malignant tumor originating from notochordal remnants. It most often occurs at the base of the skull, cervical spine, and sacrococcygeal spine, rarely occurring in the thoracolumbar spine. It typically has a destructive, lytic appearance on imaging. It occurs in adults in the fifth to seventh decades of life with an overall male predominance. Chordomas are often asymptomatic and only present clinically when the lesion is large and compressing local structures. The first line treatment is en bloc resection with wide surgical margins with or without adjuvant radiotherapy and chemotherapy. Radiation is typically used for patients that decline surgery, cannot achieve tumor-free surgical margins, or have metastatic disease Here, we present an unusual case of a chordoma of the L2 vertebral body with a sclerotic appearance, occurring in a 57-year-old female. Such a lesion may be mistaken for a metastasis, and awareness of the variable location and appearance of chordomas is important so that it can be included in the differential diagnosis.A 57-year-old female presented with intermittent lower back pain, exaggerated with increased activity. Occasionally, there was radiating pain to the left buttock and numbness and tingling in the bilateral thighs. She reported no weakness or bowel incontinence. She reported no significant weight loss, fevers, or chills. She had no significant past medical, surgical, or family history.The patient underwent physical therapy without significant improvement. Radiographs of the lumbar spine demonstrated sclerosis in the posterior aspect of the L2 vertebral body and degenerative changes . SubsequCT-guided biopsy of the sclerotic osseous lesion at L2 was consistent with a chordoma . The patChordoma is a rare neoplasm originating from the primitive notochord. Their incidence is low, about 0.08 per 100,000 people per year. Lumbar spine chordomas account for only 6% of all chordomas The typical imaging findings of vertebral chordoma are well-described in the literature. On CT, chordomas typically appear in the axial skeleton as a lytic lesion. Lesions are usually well-circumscribed due to slow growth and may include calcification and marginal sclerosis . On T1-weighted MRI, chordomas appear as a low to intermediate-intensity lesion that may extend into or encase the adjacent neurovascular structures ,8. On T2Chordomas can occasionally be sclerotic and are included in the differential diagnosis for an ivory vertebra. This case demonstrates that chordoma should be considered in the differential diagnosis for an older adult with a sclerotic vertebral lesion, particularly if it is a solitary lesion.On pathology, chordomas macroscopically appear lobulated and can grow up to 15 cm. They often contain calcification and hemorrhage and can erode through bony structures. Pathognomonic physaliphorous cells are seen in chordomas Despite increasing prevalence of spinal metastatic disease, primary neoplasms of the spine do occur and should be recognized. Chordomas are rare primary neoplasms of the spine that arise from embryological remnants of the notochord. Our case serves as an illustrative example of why chordoma should be included in the differential diagnosis of a vertebral lesion despite atypical location and imaging findings.


Pleomorphic adenoma (PA) of the parotid gland is considered one of the commonest benign salivary gland neoplasms in both adults and pediatrics. However, metastatic pleomorphic adenoma (MPA) is extremely rare. In the past three decades, multiple cases were reported in the literature of MPA, where the metastatic phase has been preceded by a local recurrence for the majority of the cases. Metastases to the lungs, liver, bone, head and neck were reported. This paper will discuss a rare case presentation of MPA that metastasised solely to the face and scalp subcutaneous tissue with no other sites of metastases in a male adult.  Salivary glands are divided into major and minor glands. The major glands are parotid, submandibular, and sublingual glands. Tumours of the salivary glands are considered rare entities as they only constitute 3-4% of all head and neck tumours . The latA 40-year-old male presented to the clinic at the age of 25 with a right-sided parotid swelling progressing over a few months. Fine needle aspiration was done and the results came back positive for pleomorphic adenoma, for which he underwent a right-sided parotidectomy in 2007. With the initial presentation, the patient had no lymph node involvement both clinically and radiologically. Ten years post-surgery, he developed local recurrence and underwent revision parotidectomy as shown in the CT scan in Figures In a recent case review study, over 5000 cases of salivary gland tumours were reviewed over a period of almost 40 years . The autThe literature has established that PA exhibits the capability of recurrence at the primary site or metastasising to a secondary site ,8,9. BotIn reference to our case, PA originating from the parotid glands that metastasise to the scalp is virtually not encountered as often. After a thorough literature review, we concluded that the first and only case report of such a clinical scenario was described by Tarsitano et al. back in 2014 . The papThe exact pathophysiology of MPA is still not completely understood. It was hypothesised that the rationale behind it was due to surgical manipulation leading to seeding and dislodgment of PA cells, the presence of a known risk factor such as a history of irradiation, positive margins post-surgical excision, or genomic alterations with selective advantage gaining the ability to metastasise ,8,15-17.For further elaboration, surgical excision is considered the cornerstone of PA treatment ,19. HoweAdjuvant radiotherapy proved effective in lengthening the remission period and providing local control -26. GeneTo the best of our knowledge, this is the first reported case of scalp MPA in Saudi Arabia. Despite the fact that MPA is extremely rare, the diagnosis should be suspected in patients with a known history of PA presenting with lesions after recurrence. Although salivary gland neoplasms account only for 3-4% of head and neck tumours, the morbidity and mortality associated with them are burdening our healthcare system. Especially in tertiary and specialized hospitals, where the majority of the cases being referred would be neoplasms such as PA. The latest research showed that the majority of cases could be treated by surgery alone, however, the risk of metastasis of such tumours is still high. Some patients were treated with neoadjuvant, adjuvant, and even concurrent chemoradiotherapy. Thus, the available treatment options are not curative. Indeed, extensive research has to be done to establish newer treatment modalities. Biological agents revolutionized the management of many medical conditions across all medical specialities. Specifically, in otolaryngology, biologics have been integrated into the management of chronic rhinosinusitis, allergic rhinitis with polyposis, and head and neck benign and malignant neoplasms. Since they showed exquisite outcomes; we suspect that biologics might play an important role in treating PA in the future.


Introduction Rapunzel syndrome is characterized by a large trichobezoar in the stomach with a tail extending beyond the pylorus into the small bowel, causing mechanical obstruction of the small bowel. A 7-year-old girl presented to the emergency room with severe epigastric pain. Computed tomography suggested trichobezoar causing jejuno-jejunal intussusceptions, bowel wall thickening, and dilated small bowel loops proximal to the obstruction. On laparotomy, two concealed perforations were noted at the duodenojejunal (DJ) junction and 40 cm distal to the DJ junction. An enterotomy incision was given at the antimesenteric border of the distal jejunal perforation site, and the mass was successfully extracted. Primary repair was done at the DJ perforation site, and resection was followed by an end-to-end anastomosis at the distal jejunal perforation site. Surgery confirmed a complex mass of tangled hair within the gastric cavity with a tail extending into the pylorus of the stomach and small intestine, consistent with trichobezoar.Conclusion Computed tomography is superior to other radiological imaging modalities for diagnosing trichobezoars as it helps diagnose and demonstrate mechanical bowel complications. Bezoars are formed by indigestible material within the gastrointestinal tract that eventually transforms into an agglomerate nondigestible mass. They are classified according to their contents as phytobezoars (composed of fruit fibers or plants), lactobezoars (composed of milk), trichobezoars (concretions of hair), and pharmacobezoars (composed of medications).2firm, nontender, well-delineated lump occupying the epigastrium with a smooth surface was noted. There was no rigidity or guarding. Routine blood and urine investigations performed demonstrated mild anemia. The rest of the parameters were within normal limits . Urine examination, liver function tests, and serum electrolytes were within normal limits. Blood urea 22 mg% and serum creatinine 0.8% mg%. Total serum proteins 3.5 g%, albumin 1.2 g%, globulin 2.3 g%, blood glucose 105 mg%, iron 23 microgm%, ferritin 11 microgm%, and total iron binding capacity raised. She appeared well nourished and weighed 24.1 kg. Her height is 121 cmA 7-year-old female child presented to the emergency room with severe colicky epigastric pain and nonbilious vomiting for 5 days. The patient had a past medical history of trichotillomania and trichophagia. No signs of mental retardation. On general examination, the patient appeared pale. She was afebrile, and all other vital signs were normal for her age . On abdominal examination, a firm nontender mass was palpated, extending from epigastrium to left hypochondrium. Approximately 7.2 × 5.5 cmThe plain radiograph demonstrated a distended stomach gas shadow. Routine ultrasound abdomen showed nonspecific echogenicity with intense acoustic shadow within the stomach and pylorus region. Upper gastrointestinal endoscopy revealed a large trichobezoar  fillingA midline laparotomy incision was given, and a duodenojejunal (DJ) junction was noted to the left of the L1 vertebra. Masses were palpated at the stomach and small bowel. Two concealed perforations were pointed out at the DJ junction and 40 cm distal to the DJ junction. Trichobezoar was successfully removed through a separate enterotomy incision at the antimesenteric border of the distal jejunal perforation segment as the perforation site is close to mesentery . DJ junRapunzel was a fairy tale character with long hair. Because of the resemblance of the tail of a trichobezoar extending into the small intestine to the hair of Rapunzel, this condition was named Rapunzel syndrome.Trichobezoar is a complex mass made of swallowed hair and makes up less than 6% of all bezoars.Nour et al reported a rare case of Rapunzel syndrome with generalized edema. Protein-losing enteropathy, poor intake, malabsorption, and bacterial overgrowth may contribute to hypoalbuminemia that occurs insidiously over a long period.The abdominal radiograph is nonspecific and may demonstrate a distended stomach shadow with an intragastric mottled gas pattern outlined by fundal gas, which may resemble a food-filled stomach. Barium studies may show an intraluminal filling defect with a mottled gas pattern without attachment to the bowel wall. Transabdominal ultrasound may demonstrate a hyperechoic curvilinear mass associated with posterior acoustic shadowing within the stomach and pylorus region.With its limitations, magnetic resonance imaging is less beneficial than computed tomography for diagnosing trichobezoar. The upper gastrointestinal endoscopy may demonstrate a complex mass of hair within the stomach and detect other complications such as gastric inflammation and ulcers.Enzymatic degradation and medical treatment of trichobezoars are futile as they resist them. Endoscopic removal of trichobezoars is mostly ineffective. The large size and dense composition of the bezoar limit its endoscopic fragmentation. On endoscopic removal, we should consider the possibility of migration of lysed bezoar fragments into the small bowel, causing a secondary obstruction. Surgical removal through laparotomy is the treatment of choice for trichobezoars given its high success rate, low complication rate, low complexity, and potential to examine the small bowel and management of intussusception. Long-term psychiatric counselling is essential to reduce the risk of recurrence of trichotillomania and trichophagia.We present a case of Rapunzel syndrome with small bowel complications. In young females with underlying psychiatric illness presenting with the features of small bowel obstruction, one should exclude the possibility of trichobezoar. Computed tomography is superior to other radiological imaging modalities for diagnosing trichobezoars as it helps diagnose and demonstrate mechanical bowel complications.


Becker’s nevus, also known as Becker’s melanosis or Becker’s pigmentary hamartoma, is a concurrent melanosis first described by S. William Becker. It is a type of acquired hyperpigmentation characterized by well-defined, unilateral lesions with regular borders. It is associated with hypertrichosis and hyperpigmented brownish patches with a mean diameter of 15 cm. The shoulder, scapular area, and upper arms are the most commonly affected areas, but it can occur on any area of the body, including the forehead, face, neck, lower trunk, extremities, and buttocks. The lesion usually appears around puberty, and males are more likely to be affected than females.A 27-year-old male of Arabic descent who was medically free presented to the dermatology clinic complaining of bilateral, symmetrical, hyperpigmented patches on the upper back. The lesions started almost at birth, gradually growing in size and darkening in color over time. On local skin examination, the lesions were bilateral, symmetrical, hyperpigmented patches on the upper back. They were both homogeneous and brown in color with irregular borders and blotchy hyperpigmented macules on both sides of the upper back associated with sparse hair development. Histopathological examination revealed epidermal hyperkeratosis, acanthosis, and focal regular elongation of rete ridges with clubbing. Increased basal layer pigmentation was noticed. The dermis showed focal areas with pigment incontinence. Based on the above clinicopathological findings, the patient was diagnosed with Becker’s melanosis. He was referred to the laser clinic for further treatment. Becker’s nevus (BN), also known as Becker’s melanosis or Becker’s pigmentary hamartoma, is a concurrent melanosis first described by S. William Becker. It is a type of acquired hyperpigmentation characterized by well-defined, unilateral lesions with irregular borders. It is associated with hypertrichosis and hyperpigmented brownish patches with a mean size of 15 cm. Although it occurs mostly on the shoulders, scapular area, and upper arms, it can also be found on any area of the body, including the forehead, face, neck, lower trunk, extremities, and buttocks. It is rare when it appears as a bilateral lesion or in unusual locations. Males are more prone to be affected than females, with a ratio of 5:1 and a prevalence of 0.5%. The lesion generally appears around puberty; however, there have been a few reports of BN appearing at birth or in early infancy. The majority of the cases are sporadic, whereas some are familial . It is oA 27-year-old healthy male of Arabic descent presented to the dermatology clinic complaining of bilateral, symmetrical, hyperpigmented patches on the upper back. The lesions started almost at birth with a gradual increase in size and darkening in color over years and were associated with the development of hypertrichosis on the lesions. There were no relieving or aggravating factors and no associated pain, itching, bleeding, or ulceration. A review of the systems and past medical, surgical, drug, and allergy history were all unremarkable. There was no family history of any skin diseases or medical or congenital conditions. The patient’s four siblings were normal, with no active or previous complaints or skin manifestations. The patient’s antenatal history included normal vaginal delivery with no complications, as well as full-term and up-to-date immunization records. The mother was healthy, and she did not receive any medications during pregnancy. Body, hair, nails, and mucous membrane examinations were normal, and the patient had not previously received any treatment for this lesion and had not sought medical care. On local skin examination, the lesions were bilaterally and symmetrically hyperpigmented patches on the upper back, with the right one measuring 15 × 7 cm Figure . There wcafe au lait macules, congenital melanocytic nevus, congenital smooth muscle hamartoma, and plexiform neurofibroma. All were excluded because of the presence of clinical and histopathological features of BN. The therapeutic modalities were limited and mainly indicated for cosmetic reasons. Various types of lasers have been shown to be effective in BN. Two types of lasers are mostly used, namely, Q-switched ruby lasers and Q-switched Nd-YAG lasers. However, both are associated with a high recurrence rate [BN lesions may develop for a year or two after they first appear before becoming fixed in size. Pigmentation fades with time, but associated hypertrichosis persists. The specific genetic basis of BN is unknown. Given the mosaic/regional distribution of BN and the presence of sporadic and familial instances, a dominant mechanism of inheritance is proposed . Accordince rate . Our patBN is a type of acquired hyperpigmentation characterized by a well-defined, unilateral lesion with irregular borders. It is associated with hypertrichosis and hyperpigmented brownish patches. It can be found in any area of the body, but it is rare when it appears as a bilateral lesion. Our case represents a congenital, bilateral, symmetrical, atypical BN not associated with syndromic features, which is a rare presentation. The purpose of reporting this case is to increase the understanding of the various manifestations of this disease.


To evaluate the use of magnetic resonance imaging (MRI) in preoperative delineation and surgical planning for the management of female urethral and periurethral cystic vaginal swellings, with emphasis on postoperative surgical outcomes.This is a retrospective analysis of females complaining of periurethral and urethral cystic swellings referred to our tertiary center, who underwent MRI for preoperative planning in the period from January 2014 till January 2021, with a total number of 57 patients. Data retrieved from the medical records included: patients’ demographics, presenting symptoms and signs, preoperative radiological investigations, duration of symptoms, previous surgical intervention, detailed intraoperative data, postoperative complications, and postoperative follow-up.Urethral diverticulum was the commonest cystic lesion representing (64.9%) followed by Skene gland cysts in 14%, Mullerian cysts in 7%, Gartner cysts in 3.5%, and dermoid inclusion cysts in 10.5%. MRI precisely diagnosed the various pathological entities and anatomical complex lesions prior to surgery. This was confirmed after surgery and pathology analysis. All patients were followed up with a mean duration of 35 months, without any evidence of recurrence.MRI as a standalone imaging technique is mandatory for diagnosis of all urethral and periurethral cystic lesions, as it offers the most accurate diagnostic modality for delineation of these lesions and hence aids in the preoperative surgical planning, aiming to avoid recurrence and improving surgical outcomes. Female urethral and periurethral cystic swellings are not a common pathology. Presenting symptoms are multiple, including vaginal fullness and discomfort, urinary symptoms including recurrent urinary tract infections (UTIs), urinary incontinence (UI), obstructive urinary symptoms, and discharging pus or urine from urethral meatus. Differentiating these lesions clinically is challenging due to symptoms overlap and difficulty in identifying the origin of these cystic swellings .There is a wide range of differential diagnoses of these cystic lesions, with urethral diverticulum , Skene duct cyst with classical juxtameatal location, and less commonly anterior vaginal wall cysts such as Mullerian remnant cyst, Gartner cyst, and inclusion dermoid cysts .The incidence of female UD is 1–6% , often aDifferent urethral imaging modalities had been utilized to identify the nature of these swellings, including voiding cystourethrogram (VCUG) and double-balloon urethrography (DBU). They allow both delineation of the UDs by contrast and identification of their ostial communication to the urethra. However, they are invasive tools due to need for catheterization, radiation exposure, necessity to void, and technically difficult to perform. All these shortcomings make these modalities equivocal, and thus, additional imaging studies are needed .Various ultrasound (US) techniques  were compared to VCUG, showing higher sensitivity in detecting the neck of UD compared to contrast modalities. It is also helpful in detecting nearby lesions such as infected periurethral cysts and leiomyomas, which are all not visualized with VCUG. However, US is operator-dependent, and depends on diverticular size and its connection to the urethra .More recently, magnetic resonance imaging (MRI) has become the gold standard modality in the diagnosis of these vaginal swellings. It is non-invasive, with no need for catheter placement. There is no radiation exposure nor contrast injection. MRI gives global spatial configuration of the soft-tissue swellings present with accurate anatomical demarcation of the urethra and the vaginal tissues , 7.The objective of this study was to evaluate the use of MRI in preoperative delineation and surgical planning for the management of different female urethral and periurethral cystic swellings, paying particular attention to the postoperative surgical outcomes.This was a retrospective analysis of the medical records of females with anterior vaginal cystic swellings, being referred to our tertiary center, in the period from January 2014 to January 2021. Local institutional review board and ethics committee  approval was obtained. Fifty-seven patients were eligible for the study. Data collected were patients’ demographics, presenting symptoms and signs, urogynecological examination data, preoperative radiological investigations performed, duration of symptoms, previous surgical intervention, detailed intraoperative surgical data, post-operative complications, and post-operative follow-up. Preoperative MRI studies were available for all the 57 females included. Out of the 57 females, 13 patients were referred to our center with recurrent vaginal swellings after being operated upon in other hospitals. All these thirteen patients did not perform MRI pelvis prior to their first surgical intervention.Patients underwent non-contrast MRI pelvis prior to the proposed surgical procedures. MRI was performed on a 1.5 Tesla closed-configuration system Siemens ESPREE  using a pelvic phased-array coil. Patients were instructed not to urinate for at least 1 h prior to the examination to ensure a semi-full bladder. The patients underwent imaging before and after urination, with adoption of postvoiding sequence to monitor any increase in size of the suspected mass, which was likewise detected live during straining sequence performed before and after voiding. No urethral catheterization was needed. Multiplanar axial, coronal, and sagittal T2-weighted (T2W) turbo spin-echo sequences (TSE) were performed.For every swelling, these parameters were looked for: shape, number and size, location in relation to the urethra, location of the ostium, and signal intensity across all sequences to detect the nature of the fluid inside the swelling. On T2W sequences, ostial communication of UD was defined as a beaklike linear extension throughout the mucosa and submucosa of the urethra.Based on MRI findings, the surgical plan is determined according to ostial number and location, complexity of the UD (septae and number of compartments), location of the UD compartments in relation to the whole circumference of the urethra. Prior to the surgery, we perform a urethroscopy to identify the ostium if possible. Once the ostium is identified, it is calibrated with a 4-Fr ureteric stent. If it fails to pass, a guide wire is introduced instead. Inverted U-shaped anterior vaginal incision is done to provide maximal exposure of the swelling. Care is taken not to perforate the swelling during dissection to help in its complete dissection. In cases of UD, the periurethral layer must be identified and incised transversely to ensure adequate exposure of the UD and its extensions around the circumference of the urethra to facilitate its complete excision.Dissection is continued in all directions around the urethra till the UD is only connected to the urethra at the site of the communication. The connection is then cut, and the urethral defect is closed with 4–0 interrupted polyglactan sutures. In cases where extensive dissection is done with fear of possible sphincteric derangement, marsupialization is only done to prevent postoperative SUI or urethrovaginal fistula. A urethral catheter is left in place for 3–10 days according to the extent of the urethral defect.Post-operative visits were planned as follows: 1 week, 3 months, and 6 months post-operatively, to evaluate the presence of complications and any recurrences of the masses or the symptoms.t test and the Mann–Whitney U test were performed to compare continuous parametric and nonparametric variables, as appropriate. Continuous variables were reported as %. All values in the text and tables are expressed as %.Statistical analysis was performed with IBM-SPSS v.17 for Windows . Student’s A total of 57 patients with urethral/periurethral vaginal swellings were considered for the analysis. Patients’ demographics and characteristics are shown in Table Thirteen patients (22.8%) presented with recurrent vaginal swelling after experiencing surgical intervention to remove it in other hospitals. Of these, two patients did not perform any kind of preoperative imaging, seven underwent transvaginal US only, two underwent VCUG only, and two underwent both VCUG and transvaginal US, while none underwent MRI. However, these 13 patients underwent MRI in our institute before their definitive surgery.For the remaining 44/57 (77.2%), 34 patients underwent MRI only, seven underwent MRI and transvaginal US, and three underwent MRI and VCUG.MRI identified eight 14.04%) skene gland cysts, four 7.02%) Mullerian cysts, two (3.51%) Gartner cysts, and six (10.53%) inclusion dermoid cysts . Complete excision of the UD was amenable in 32 cases (86.4%). The remaining 5 cases had marsupialization of the cyst only due to difficult dissection and fear of extensive urethral defect. Perioperative and postoperative complications were minor. Nine patients (9/57) had minimal intraoperative bleeding from the vaginal dissection, which was controlled intraoperatively, and a vaginal pack was left for 24 h. Five patients (5/57) had UTI postoperatively. We did not experience a single case of recurrent vaginal swelling after our surgical intervention in our institute. Mean duration of follow-up of our patients was 35 months (with a range of 6–62 months).Female urethral and periurethral cystic swellings represent a great challenge for the urogynecologists. Diagnosis of these lesions is difficult due to short female urethra, intermingling urogynecological symptoms, rarity of these lesions, and the anatomical location of these lesions in relation to the complex pelvic structures , 8.All these obstacles reflect on the duration to reach accurate final diagnosis and type and number of preoperative radiological imaging. The mean duration to reach accurate diagnosis is variable in the literature, ranging from 2 to 6 years with a mean of 5.2 years , 9. In oThese lesions usually present with a wide range of overlapping symptoms. Symptoms include vaginal bulge, classical 3Ds of dyspareunia 12–14%) , urinary–14% , urRadiological modalities available for accurate detection of the nature of these cystic lesions are variable. VCUG was frequently used in the past to delineate UD. It is considered hazardous due to radiation exposure and need for catheterization with risk of UTI. It is also solely dependent on voiding of the patient. In acutely inflamed UD, VCUG will not opacify them and thus will miss their diagnosis . SensitiDifferent ultrasound techniques are also available. It is non-invasive without risk of radiation exposure or contrast injection. It also has higher sensitivity than VCUG and DBU, but it is operator-dependent and cannot be interpreted by the surgeons .MRI is considered the gold standard imaging modality. It does not require catheterization, no contrast injection, and no radiation exposure, and is not dependent on voiding of the patient . It alloThese cystic swellings include UD, which is the most common lesion representing 84% of all periurethral lesions , 6, SkenUD arise from the posterolateral aspect of mid-urethra. Classically, non-complicated UD exhibit signal of clear urine which is hypointense on T1W images and hyperintense on T2W image. If they are complicated by blood or protein content, they display variable iso- to hyperintensity on T1W sequences, and variable hypointensity on T2W images. Inflamed/infected UD show heterogeneous signal intensity on T1W images, and marked hyperintensity on T2W fluid–fluid level , 25. OstGartner duct cysts are embryological retention cysts arising from Wolffian duct remnants, anywhere from the mesosalpinx through the broad ligament to the cervix and anterolateral upper third of vagina, above the level of symphysis pubis . A classOn MRI, Skene gland cysts are teardrop like cysts, typically located juxtameatal, being lateral to the external urethral meatus and inferior to the pubic symphysis. They secrete mucus-containing fluids which is protein-based, hence giving the protein signal of iso- to hyperintensity on T1W sequences. This becomes highest when obstructed, turning the retained secretions into cheesy like material .Epidermal vaginal inclusion cysts are situated at sites of prior surgery or vaginal trauma. They are located mainly at posterior vaginal walls. They demonstrate intermediate signal intensity on T1W images . In our Recurrent UD is reported in up to 40% after transvaginal excision of UD. Risk factors for UD recurrence are multiplicity, proximal location, and complex configuration of UD. When MRI is not utilized, the risk of incomplete removal of UD is high due to underdiagnosis of the complexity and the extent of the UD . Repeat The 13 patients presenting with recurrent vaginal swellings were previously evaluated with imaging modalities other than MRI. They underwent MRI in our institute which revealed complex UD  in all cases. We believe if these patients had performed MRI prior to their initial surgery, the complexity of the UD would have been evident, and appropriate preoperative surgical planning would have been beneficial in achieving complete excision and avoidance of recurrence. In our institute, we perform MRI as a standalone diagnostic imaging modality in all vaginal swellings. MRI is a mandatory tool for proper preoperative surgical planning to understand the complexity of the lesions and precise anatomy of the urethra in relation to the vaginal swelling.In our series, UD was identified in 37 cases. Five were simple diverticula, while 32 cases were of complex anatomical configuration. MRI was helpful in precise identification of all complex lesions as confirmed during the surgery. This allowed complete excision of the entire UD. All patients were followed-up with a mean duration of 35 months, without evidence of recurrence. This signifies the importance of preoperative MRI to ensure surgical success, especially in complex UD.One of the limitations of the utilization of MRI in every suspected case is the cost. However, if we consider its high sensitivity in detection of these lesions, we will avoid multiple preoperative imaging modalities which will collectively add up to a probably equivalent cost and needless unplanned surgical interventions, with a high chance of failure and recurrence. This will make MRI a cost-effective tool in diagnosis of these lesions with proper surgical planning.To our knowledge, this is the largest series of cystic urethral and periurethral swellings in the literature, with longest follow-up period. All the cases were operated by the same surgical team, and the preoperative MRI was interpreted by the same radiologist, who was blinded to the clinical data. Limitations of our study include the retrospective nature with subsequent lack of statistical data and the fact that we did not perform postoperative MRI to ensure complete excision of the lesions; instead, we depended on the clinical symptoms and examination.Periurethral and urethral cystic swellings are uncommon lesions, with overlapping urogynecological symptoms. Identification of the nature and anatomical complexity of these lesions, especially complex UD, is of crucial importance. MRI as a standalone imaging technique is mandatory for diagnosis of all these cystic lesions, as it offers the most accurate modality for delineation of these lesions. This will aid in the preoperative surgical planning, aiming to avoid recurrences and improve surgical outcomes.


Spontaneous rapture of a germ cell tumor (GCT) metastases causing massive hemoretroperitoneum in a patient without choriocarcinoma component who has not received previous systemic chemotherapy is an exceedingly rare event. In such a devastating case scenario, a high index of clinical suspicion for early diagnosis and appropriate management is crucial.We report on a 25-year-old male patient with a 4-month history of orchiectomy for testicular GCT (tGCT), who presented in the emergency department with acute abdomen and hemodynamic instability. Urgent computed tomography scan depicted a retroperitoneal mass measuring approximately 13 × 11.4 × 15 cm and massive intraperitoneal hemorrhage. Hemoperitoneum caused by spontaneous rapture of the metastatic retroperitoneal mass was suspected. COVID-19 pandemic-related deviation from the oncologic surveillance standards combined with COVID-19-related patient’s emotional distress and self-neglect had led to loss of opportunity for appropriate adjuvant chemotherapy, obviously leading to the development of this devastating complication. An emergency, surgical exploration was decided. The bleeding mass was adequately exposed following a Cattell–Braasch maneuver and active bleeding was controlled by a challenging resection of approximately 80% of the lymph node mass volume. Pathological evaluation of the specimen revealed teratoma with low volume of yolk sac tumor component and extensive necrosis, findings compatible with the patient’s history. Postoperative recovery was uneventful, followed by early start of adjuvant chemotherapy. Two years after the operation the patient is doing well with no evidence of recurrent disease.Massive hemoperitoneum is a devastating event that exceedingly rarely can complicate the clinical course of patients with advanced tGCT. Emergency surgical intervention is usually necessary however, sound judgement and careful surgical techniques are required for a positive and uneventful outcome. During COVID-19 pandemic, first-line medical personnel push their limits further not only to ensure health care services standards but also, to manage unpredictable, life-threatening cancer-related complications, associated with COVID-19-related deviation from appropriate oncologic surveillance and care. Testicular germ cell tumors (tGCT) are common malignant lesions among young men aged 20–45 years. They often spread via lymphatics or hematogenously, and they typically metastasize to the retroperitoneal lymph nodes, lungs, liver, bones or brain. . Germ ceThe present report describes the successful surgical management of an exceedingly rare case of hemoperitoneum caused by spontaneous rapture of tGCC retroperitoneal lymph node metastases in a patient without choriocarcinoma component who has not received previous systemic chemotherapy. Active bleeding was uneventfully controlled by a challenging surgical resection of approximately 80% of the metastatic lymph node mass volume.Regardless of the rarity and favorable outcome of the present case, it should be emphasized that this exceptional complication is actually a consequence of COVID-19 pandemic-related disruption in oncologic surveillance, which inevitably resulted in loss of opportunity for appropriate adjuvant therapy and the development of this devastating complication.9/L), relatively low hemoglobin levels (10.4 g/dL), moderate hyperglycemia (225 mg/dL), slightly elevated urea and creatinine levels  and normal lactate dehydrogenase levels (140 U/L). An urgent contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis depicted a large 13 × 11.4 × 15 cm retroperitoneal mass extending to the preaortic region below the renal veins in addition to a significant amount of free intraperitoneal fluid suggestive of hematic content  with however, normal blood pressure. Abdominal distention, diffuse abdominal tenderness and a palpable mass in the periumbilical region were also evident. Initial laboratory tests revealed high white blood cell count , embryonal cancer (40%), seminoma 10%) and yolk sac tumor (5%), and was classified as pT2 according to the 8th edition of AJCC cancer staging manual  predicte% and yolThe patient was urgently admitted to the intensive care unit (ICU) for hemodynamic resuscitation. Vital signs at arrival in the ICU were pulse of 130 beats per minute, blood pressure 95/60 mm Hg, and hemoglobin level of 8.6 g/dL, suggesting a deteriorating hypovolemic shock. The patient was resuscitated with 2 L of intravenous fluids, 2 units of packed red blood cells, 2 units of fresh frozen plasma and vasopressors. He did not respond satisfactory to fluid load, while vasopressor needs were increased, with post-transfusion hemoglobin of 8.1 g/dL, and ABGs lactate levels 3.1 mmol/L. However, apart from detailed description of resuscitation requirements, the most decisive clinical sign was the progressively worsening abdominal distension of the patient, indicating an ongoing, uncontrolled intraperitoneal hemorrhage. This, combined with ICU monitoring data, laboratory values and computed tomography findings (massive active hemoretroperitoneum with a significant amount hemoperitoneum) led to the decision of an emergency exploratory laparotomy.The peritoneal cavity was immediately accessed through a midline laparotomy. Massive hemoperitoneum was evident, necessitating evacuation of 2.5 L of blood, in order to identify the source of bleeding. A massive retroperitoneal hematoma surrounding a bleeding retroperitoneal lymph node metastatic necrotic mass was evident. Shearing and infiltrating forces exerted from the bleeding retroperitoneal metastatic mass, teared in several sites the posterior peritoneum, through which the blood entered the peritoneal cavity causing massive hemoperitoneum.Following a right-sided medial visceral rotation maneuver (the Cattell–Braasch maneuver) consisting of extensive lateromedial-craniomedial detachment and complete mobilization of the right hemi-colon and small bowel mesentery from the retroperitoneum along the fusion fascia of Toldt  the retrA huge lymph node metastatic mass was evident located in the centromedial retroperitoneum extending from the renal veins to the aortic bifurcation. The mass was very soft and fragile with necrotic surface areas, which were actively bleeding Fig. . AdditioPathological evaluation of the surgical specimen revealed morphological features of immature teratoma composed of undifferentiated cells with foci of primitive neuroectodermic differentiation forming immature neuroepithelial tissue and extensive necrosis, findings compatible with the patient’s history of mixed tGCT (metastases derived from the teratoma component of the primary mixed GCT) Fig. . PostopeIn response to the increase prevalence of the coronavirus disease at the end of October 2020 in Greece, several restrictive measures were accommodated, which unavoidably have had drastic impacts on healthcare system services. In particular, COVID-19 pandemic-related deviation from the standard oncologic continuum has profoundly impacted cancer care leading to delay or even loss of opportunity for appropriate treatment . In the Notwithstanding, severe and devastating complications related to testicular cancer metastases, such as massive retroperitoneal and/or intraperitoneal hemorrhage, are exceedingly rare. Most of the reported cases are related to rupture of liver metastases or retroperitoneal metastatic masses after the onset of chemotherapy. These cases are mainly associated with choriocarcinoma syndrome, –16 whichLymphatic spread of testicular germ cell cancer follows usually a predictable pattern, metastasizing most commonly in the retroperitoneal lymph nodes. Specifically, right testicle GCT often metastasize to the interaortocaval nodes while left-sided tumors metastasize to the preaortic and paraortic lymph nodes , 19. ThiMassive spontaneous hemoperitoneum secondary to metastatic masses usually necessitates an emergency surgical exploration however, it should be taken into account that it demands surgical skills and expertise and that it can be associated with dismal intra- and post-operative complications and considerable morbidity. Therefore, accurate diagnosis, sound judgement and careful surgical techniques are required for a positive and uneventful outcome. Indeed, caution is needed considering that retroperitoneal bleeding may also develop as a result of laceration of major retroperitoneal vessels during systemic therapy .Special attention is warranted to the usefulness of the Cattell–Braasch maneuver in these extreme emergencies. This master surgical technique succeeds safe, immediate and adequate exposure of the retroperitoneum in salvage and damage control operations . ParticuUrgent surgical exploration has been usually performed in a handful of similar cases reported in the literature. Komori et al.  reported. If this is not the case, surgical approach should be considered. In the present case, despite hemodynamic resuscitation, the patient remained hemodynamically unstable, which led to the decision of an emergency, surgical exploration. Nevertheless, it should always be kept in mind that this devastating clinical scenario represents a lethal cancer-related complication and appropriate management should be individualized, given that there are no evidence-based guidelines.Moore et al.  reportedIn conclusion, spontaneous rapture of a germ cell cancer retroperitoneal lymph node metastasis in a patient with tGCT without choriocarcinoma component who has not received previous systemic chemotherapy is exceedingly rare. Emergency surgical intervention is usually necessary however, dismal intra- and post-operative complications can frequently occur. Sound judgement, experience and careful surgical techniques are required for a positive and uneventful outcome. During COVID-19 pandemic, first-line medical personnel push their limits not only to ensure healthcare services standards, but also to manage unpredictable, life-threatening cancer-related complications associated with COVID-19-related deviation from appropriate oncologic surveillance and care.


Osteochondromas are common benign bone tumors, frequently found in adolescents or young adults. Most often asymptomatic and discovered by accidental findings, they may be diagnosed because of compression or dislocation. Vascular complications are an atypical presentation of osteochondromas, and include vessel perforation and thrombosis, arterial thromboembolic events and pseudoaneurysm formation. Popliteal artery thrombosis and acute lower limb ischemia caused by a tibial osteochondroma are rarely observed. Starting from a case of temporary lower extremity ischaemia caused by thrombosis of the subarticular popliteal artery due to an osteochondroma of the proximal tibial protruding in popliteal fossa, we focused a literature analysis on diagnostic and management aspects. A combined vascular-orthopedic approach was performed with intra-arterial locoregional thrombolytic therapy and then a surgical tangential resection of the tibial osteochondroma. The adequate approach for these patients includes clinical evaluation, plain radiographs, CT scan and MRI. The purpose of the present review article is to underline the importance of a combined vascular-orthopedic approach to correct diagnosis and prompt surgical management of vascular complications caused by tibial osteochondromas. Osteochondromas are common benign tumors of bone, most frequently found in adolescents or young adults. Osteochondromas can be sessile or pedunculated, presenting as a solitary lesion in 85% of the cases, while 15% occur in the context of hereditary multiple exostoses (HME), a genetic autosomal dominant disorder. Although often asymptomatic and discovered incidentally, symptoms may derive from compression or dislocation of near structures such as adjacent vessels or nerves, fractures, osseous deformities, bursa formation or malignant transformation . The invVascular complications are rare and include vessel perforation and thrombosis, arterial thromboembolic events and pseudoaneurysm formation . From anA systematic search of the literature was done to identify studies reporting on patients treated for vascular complications secondary to the lower limb osteochondroma. English and non-English language literature were searched in Pubmed using the terms ‘osteochondroma [MeSH Terms]’, ‘vascular’, ‘complications’, ‘pseudoaneurysm’, ‘thromboembolic events’, ‘artery’, ‘lower limb’ and ‘lower extremities’ in different combinations and in ISI Web of Knowledge database. The search was done on literature published between 1965 and 2022, resulting in about 210 articles  describing over 60 cases . VasculaA 34-year-old male was referred to the emergency department with acute left lower limb ischemia.His clinical history showed a similar ischemia at the same lower left limb four years before and other temporary symptoms. Oncologic history included acute lymphoblastic leukemia treated with chemotherapy, radiotherapy and bone marrow transplantation, and thyroidectomy for papillary thyroid carcinoma. He reported a gradual decrease of walking distance with increasing pain, cold and functional impairment of lower limb. On clinical examination he presented ecchymosis at heel level a.Physical examination revealed loss of popliteal and peripheral pulses whereas the ipsilateral femoral pulses were normal, with no neurological deficit of the affected limb. A CT scan revealed an osteochondroma on the posterior face of the tibial plateau, close to the vascular bundle b, and thHe was urgently treated by vascular surgeons with intra-arterial locoregional thrombolytic therapy in place for four days. At subsequent checks, occlusion remained in the upper third of the interosseous artery. Interestingly, at ultrasound doppler evaluation the blood flow disappears at complete extension of the knee and appears during flexion. The patient started oral anticoagulant therapy with enoxaparin 4000 UI twice a day at first, followed by warfarin for six months. Two months later, a surgical resection was performed in our orthopedic department: using a posteromedial approach, the bone surface was reached, the lesion was isolated and a tangential resection was performed a. Histo-Osteochondromas are the most common benign primary bone tumors in growing patients representing about 35–45% of all benign bone tumors. About 90% of these occur sporadically and are usually solitary ,29,30,59Pedunculated osteochondromas around the knee have a typical appearance, extending away from the adjacent joint, described as stalactites and stalagmites. In any bone that develops from enchondral ossification, osteochondromas have a marrow, cortical and periosteum continuity with the underlying native bone. This direct continuity clearly appreciable in CT scan is a pathognomonic finding . The carIt is important to include bone tumors in differential diagnosis in patients with vascular symptoms. The adequate approach for these patients includes clinical evaluation, plain radiographs, CT scan and MRI. In the presence of bone tumors, CT and MRI are the most sensitive examinations to exclude malignant appearance, soft tissue extension and signs of popliteal entrapment syndrome (PES). In young adult patients presenting with intermittent claudication or vascular insufficiency mimicking atherosclerotic peripheral vascular disease, alternative causes such as trauma, tumors, thromboangioitis obliterans, fibromuscular dysplasia, vasculitis and PES should be suspected . PES refWith the reported case, as underlined by other Authors, we strongly support the use of doppler ultrasound because it could be used as a functional examination showing the blood flow asset during complete extension of the knee ,61,62,65The main consideration about management is how to approach the vascular complication considering that treatment of osteochondroma is mainly simple surgical resection at the base of the tumor with meticulous dissection of the neurovascular structures. Most cases were managed by osteochondroma resection with or without open vascular reconstruction. Open surgical options include bypass and interposition grafting, direct repair, patch angioplasty, and aneurysm resection with direct end-to-end anastomosis. Arterial reconstruction should be considered by either closing the arterial defect in cases of pseudoaneurysm formation or using a saphenous vein graft in cases of arterial thrombosis and extensive arterial damage. From the literature review, pseudoaneurysm resection associated with venous bypass or end-to-end anastomosis represent the most frequent surgical approach, because of the high risk of recurrent pseudoaneurysm or stenosis after direct closure. Vasseur et al. reviewed 97 patients with osteochondroma associated with vascular complications, surgically repaired . Among tEndovascular management is infrequently described. In two hybrid procedures, one patient underwent thrombectomy for embolus in the tibioperoneal trunk with subsequent vein patching of the popliteal pseudoaneurysm, and another had coil embolization of the pseudoaneurysm followed by direct repair of the artery one week later ,63. SomeLocal recurrence of osteochondroma after resection is low at 1.8% but may be higher in skeletally immature patients ,30. Two Osteochondromas are the most common bone tumors. Most are asymptomatic and discovered incidentally. This frequent benign tumor may occasionally cause severe arterial complications requiring surgical urgent procedures. CT scan and MRI with contrast medium are adequate for diagnosis, even if simple functional doppler ultrasound is able to show clearly the correlation between vascular compression and ostheocondroma. A combined vascular-orthopedic approach is mandatory from diagnosis to surgical management, and it is crucial for limb salvage procedure in cases of vascular involvement.


Langerhans cells (LCs) are antigen presenting dendritic cells found in epidermis, respiratory and genital epithelia.LCH is a very rare disease seen in approximately one case per 2 million children, males are more frequently affected than females, and presenting age varies between a few months to 15 years with a peak incidence of 1–4 years.In this case presentation, we demonstrated a LCH patient with facial paralysis as initial symptom.Written consent was taken from the patient's parents for this case presentation.An otherwise healthy 2 years old male patient was consulted from pediatric emergency department with acute onset peripheral facial nerve palsy. According to the history achieved from his parents, he had no otalgia or otorrhea complaint. In the morning after he woke up, his parents realized that he could not close his left eye during crying. On his physical examination otoscopic findings revealed narrowing of the external ear canal from the posterior part by a mass originating from the mastoid bone. Tympanic membrane was hardly seen and a bluish color mass was present on the middle ear. He had left House–Brackmann grade IV peripheral facial nerve paralysis. Cranial tomography revealed a mass comprising left mastoid air cells, extending to the middle ear . MagnetiLangerhans cell histiocytosis is characterized by pathognomonic LC proliferation which are histiocytes normally found in dermis. Etiology of LCH is unknown, variable hypotheses such as neoplastic, immunologic or inflammatory processes are investigated but still there is no proof explaining the exact pathogenesis. LCH is not familial or hereditary, there is no race predominance. The disease is mostly seen in flat bones of children such as ribs, pelvis, scapula and skull. Temporal bone involvement is rare.When temporal bone involvement presents in LCH, a great suspicion is needed to correctly diagnose this rare disease, because sign and symptoms are non-specific and they mimic more common diseases of the ear such as suppurative otitis media, chronic otitis media, aural polyps, simple otitis externa and acute mastoiditis. Common presenting symptoms are postauricular swelling, chronic otorrhea, otalgia, hearing loss and bleeding. Involvement of the inner ear and sensorineural hearing loss are less common presenting symptoms due to compact nature of petrous apex and otic capsule.Differential diagnosis of pediatric peripheral Facial Nerve Paralysis (FNP) is broad including Bell's palsy, infections like acute otitis media, trauma, congenital anomalies and rarely malignancies. Most of the cases are acquired rather than congenital and number of cases is much less  when compared to adult patients. Tumors account for 2–12% of all pediatric facial nerve paralysis cases. The most common types are leukemias, teratomas, soft tissue sarcomas and neuroblastoma.For the diagnosis of LCH, pathological investigation of the biopsy specimen is mandatory. For biopsy achievement in cases of temporal bone involvement, a mastoidectomy is mostly required like our case.For treatment options of temporal bone LCH cases there is a great variability between authors. Surgery, radiation therapy, chemotherapy and combination of these treatment options exist. In the current treatment options most of the authors agree that surgery must be chosen for tumors which can be totally excised. Chemotherapy is helpful in multifocal disease and must be preferred when surgical total removal could not be achieved.The prognosis of LCH is dependent on the stage of disease and age of the patient. Age lower than 2 years, multisystem disease and organ dysfunction are bad prognostic factors for LCH cases.As a conclusion, for children with acute onset peripheral facial paralysis, langerhans cell histiocytosis must also be thought in differential diagnosis. Treatment plan must be made after imaging studies for determining widespread of the disease with a multidisciplinary approach including Otorhinolaryngologist, Pediatric Oncologist and Radiologist.The authors declare no conflicts of interest.