A Bochdalek hernia is a congenital diaphragmatic hernia that results from a failure of closure of the pleuroperitoneal folds during embryologic development. While it is most often diagnosed in neonates and infants, Bochdalek hernias can rarely present in adulthood for the first time. We describe the case of a 42-year-old lady who presented with sudden onset of severe abdominal pain following a Zumba dance session. Her chest radiograph showed an elevated left hemi-diaphragm with visualization of a gastric bubble in the thorax. A computed tomography (CT) scan of the abdomen showed a defect in the left hemi-diaphragm with herniation of the stomach and abdominal viscera through the defect. The patient was taken for diagnostic laparoscopy, and the diaphragmatic defect was repaired with a synthetic mesh. Perioperatively, perforation of the anterior wall of the stomach was noted, and a diagnosis of Bochdalek hernia with gastric strangulation was made. This case demonstrates a rare presentation of Bochdalek hernia in an adult with strangulation and perforation of the stomach. Clinicians need to be aware of this rare but life-threatening clinical entity in order to secure a timely diagnosis and institute appropriate management. Congenital diaphragmatic herniae (CDH) result from a failure of closure of the pleuroperitoneal folds during embryologic development. Vicente Bochdalek first described the posterolateral CDH in 1848 and since then, this variety of CDH is referred to as Bochdalek hernia . Here, wA 42-year-old lady of South African origin without any significant past medical history presented to the emergency department with a complaint of sudden onset epigastric and left upper quadrant abdominal pain for the past few hours. She described the pain as sharp in character, severe in intensity, and continuous and radiating to the left shoulder and back. This pain started while she was working out in her regular Zumba dance session and had remained constant since then. The abdominal pain was associated with a sensation of dizziness and shortness of breath. She felt nauseated but did not have any vomiting. She denied any previous history of similar abdominal pain, altered bowel habits, abdominal distension, urinary complaints, or menstrual complaints. She did not report any history of palpitations, chest pain, cough, or fever. She was not taking any regular medications and did not have a history of any previous surgery. She had no significant past medical history. She denied any history of trauma.Upon arrival to the emergency department, her vital signs were: pulse rate 96 beats per minute (regular), blood pressure 129/79 mm Hg (left arm), temperature 36.8°C , and respiratory rate 19 breaths/min. On physical examination, abdominal examination revealed epigastric and left upper quadrant tenderness with guarding. No rebound tenderness or other peritoneal signs were noted. Bowel sounds were audible and normo-active. Laboratory investigations were within normal limits, except for a mild neutrophilic leukocytosis (white cell count of 13.6 × 109/L with 85% neutrophils). Serum beta-human chorionic gonadotropin (β-hCG) was within normal limits. Her plain chest radiograph . The general surgery team was consulted and the patient was taken to the operating room for diagnostic laparoscopy. Perioperatively, a large posterolateral Bochdalek hernia was identified with strangulation of the stomach. Abdominal viscera were reduced to the abdominal cavity and the diaphragmatic defect was closed (from below) using a synthetic biodegradable mesh  secured with non-absorbable sutures , and the growth of the pleuroperitoneal membranes leads to the complete separation of the thoracic and abdominal cavities by the ninth week of gestation. Pleuroperitoneal hiatus are present in both sides of the septum transversum early in development, but both defects close around the eighth week of gestation. The left-sided defect is generally the last to close, and a failure of closure of this defect leads to a Bochdalek CDH . This alMost cases of Bochdalek CDH are diagnosed in neonates or infancy and the incidence of this hernia has been reported to be approximately one in 4000 . The mosFirst presentation of a Bochdalek CDH in adulthood is exceedingly rare, and most such cases are diagnosed when the hernia becomes obstructed and complications develop -4. PossiA wide variety of clinical features have been reported in adult patients with Bochdalek CDH. The contents of the hernia can dictate the type of clinical symptoms that a patient may experience . In leftThe presence of a CDH can be readily detected on plain chest radiographs. However, a Bochdalek CDH can be confused for pulmonary sequestration, an epicardial fat pad, pneumonic consolidation, or left lower lobe collapse -4. CT scTraumatic diaphragmatic rupture is an important differential diagnosis in the evaluation of adult patients with a suspected Bochdalek CDH -7. AlthoDiagnosis of a Bochdalek CDH in an adult generally warrants surgical treatment as it is prone to obstruction and strangulation . In the The use of mesh for repairing the diaphragm remains controversial and, in contrast with inguinal hernia repair, the superiority of tension-free diaphragmatic repair has not been demonstrated. Generally, elderly patients tend to have a thinner and weakened diaphragm, which makes the use of a mesh theoretically appealing. A variety of different surgical meshes are available, including non-absorbable prosthetic, acellular biological, and synthetic biodegradable meshes. To date, no clinical data have demonstrated the superiority of one type of surgical mesh over another. As biological meshes are costly and do not have any major advantages over synthetic biodegradable meshes, we chose to use the latter in the present case.Bochdalek CDH rarely presents in adulthood with signs of abdominal visceral obstruction and strangulation. Given that many such patients have vague clinical symptoms, a high index of clinical suspicion is necessary to secure a timely diagnosis and institute appropriate treatment. Clinicians in general, and emergency physicians in particular, need to be aware of this rare but life-threatening clinical entity.


Invasive cervical root resorption (ICR) is the reversible/irreversible loss of tooth structure in the connective tissue attachment zone with unclear etiology. In the present case a history of periodontal surgery was presumed to be the predisposing factor. Early diagnosis and proper treatment may lead to long-term retention of the tooth. The tooth is usually asymptomatic and diagnosis is commonly made as a result of a routine radiographic finding. Cone-beam computed tomography (CBCT) is extremely useful in diagnosis and treatment planning of ICR. The treatment should aim toward complete removal of the resorptive defect and reconstruction by placement of a suitable filling material. The present report demonstrates the management of a mandibular canine with invasive cervical root resorption using Biodentine for the defect. Resorptsorption .The etiology of invasive cervical resorption is poorly understood, several potential predisposing factors have been identified, in one study, intra-coronal bleaching has been suggested as the most widely documented factor  whereas The condition is usually painless and diagnosis is usually the result of a routine radiologic examination . HeitherRadiographic features of lesions range from well-delineated to irregularly bordered mottled radiolucencies, which can be confused with dental caries. A characteristic radiopaque line generally separates the image of the lesion from that of the root canal, because the pulp remains protected by a thin layer of predentin until late stages of the process . Cone-beThe basic treatment modalities are complete removal of resorptive tissue, and then dentinal walls are conditioned by 90% aqueous solution of trichloracetic acid for coagulation necrosis of the resorptive tissue without damaging the periodontal tissue. Any undermined dentin or enamel at the peripheries of the cavity are removed with a bur in a high-speed handpiece, and the cavity is restored by appropriate restorative material like glass-ionomer cement, composite resin , 10 or cBiodentine may prove to be a particularly suitable material for restoring these defects because it may combine acceptable aesthetics with the ability to support PDL attachment , 15. TheA 51-year-old female with a chief complaint of pain in the lower-anterior portion of her gums during past two weeks presented to Endodontic department, Dental School, Shahid Beheshti University of Medical Sciences. Medical history of the patient was noncontributory. The patient mentioned a history of recurrent localized swelling during past three months in buccal mucosa of mandibular left canine. Past dental history revealed that she had undergone periodontal surgery in anterior left segment of mandible two years earlier.On clinical examinations, the buccal gingiva of tooth was sensitive to palpation .The sensitivity tests including electric pulp test and thermal tests were negative while the adjacent tooth responded normally. On periodontal examination probing depth was approximately 6 mm on buccal sulcus of tooth and bleeding on probing was obvious. Tooth was not mobile and no caries or previous filling was present .On radiographic examination, radiolucency in cervical area and coronal third of the root was present and normal periradicular and periodontal ligament status was seen. CBCT images confirmed the presence of external cervical root resorption on buccal surface of the root and perforation of root canal wall .A diagnosis of pulp necrosis with external cervical root resorption (Heithersay’s type III) was made, therefore root canal therapy and surgery for sealing the resorptive defect was planned.Written informed consent was obtained from the patient. Under local anesthesia, buccal and lingual infiltration of 2% lidocaine containing 1:80000 epinephrine  was administered and rubber dam was placed. Access cavity was prepared on lingual surface. After working length determination with #15 K-file , cleaning and shaping of root canal was performed using ProTaper rotary system . S1, S2, F1 and F2 rotary files were employed up to the working length. At first visit, irrigation was performed with normal saline solely, due to the presence of the root canal perforation. During the cleaning and shaping process, profuse bleeding started, possibly from perforated area. Since proper cleaning and debridement of root canal system before complete removal of resorptive tissue and sealing the perforation area was not possible, the tooth was not obturated in first visit. Mild bleeding was still present at the end of the cleaning and shaping process. Root canal was partially dried with paper points and a tapered gutta-percha (#30/0.04) was placed in canal to maintain its patency during the restoration process in forthcoming surgery. The access cavity was then sealed with Coltosol .At second visit after administration of buccal and lingual infiltration, an envelope full mucoperiosteal flap was reflected . GranuloClinical and radiographic examinations were performed for treated teeth after 6, 12 and 24 months. Patient was completely asymptomatic and probing depth was within normal limits at each follow-up time point indicating that repair of resorption defect was successfully performed with Biodentine .The presented case describes an invasive cervical root resorption; the tooth required root canal treatment due to perforation of root canal wall and pulp necrosis, followed by sealing the resorptive area with Biodentine. The etiological factor for the resorption in this case is periodontal treatment which resulted into resorptive defects. Root resorption in permanent dentition has pathological basis and is classified into internal and external resorption. Cervical external root resorption also called as invasive cervical resorption (ICR) is a localized resorptive process that commences on the surface of the root below the epithelial attachment and the coronal aspect of the supporting alveolar process and is symptomless until the destruction reaches the pulp . ICR hasThere are different modalities for treatment of external cervical root resorption. Treatment alternatives are case-dependent and depends on the etiology of resorption. The basic aim of treating is the complete removal of resorptive tissue and the restoration of the defected area. The basic treatment modalities are flap reflection, complete removal of resorptive tissue, restoration of the cavity by appropriate restorative material like glass-ionomer cement, composite resin , calcium3SiO5) based inorganic restorative cement. Hydraulic calcium silicate cements are bioactive materials showing a dynamic interaction with dentin and pulp tissue interface and they can stimulate pulpal cell recruitment and differentiation. They also, up-regulate transformation factors and promote dentinogenesis [Biodentine is a new tricalcium silicate  and pulp capping and can be used as a dentine replacement material in restorative dentistry . The matIn the case presented the diagnosis was Heithersay’s class III ICR. The treatment plan was combination of surgical approach and non-surgical root canal treatment. The selected material was Biodentine because of better consistency after mixing, better handling properties, faster setting and also less tooth discoloration, compared to MTA. It is bioactive, biocompatible, and non-resorbable and having sufficient amount of push out bond strength with dentinal walls which makes it an excellent material for perforation repair. Also it produces tighter seal in the area of the defect and prevents microleakage . After tEarly diagnosis, correct case selection, an appropriate restorative/regenerative material and proper treatment are essential for long-term retention of the tooth with invasive cervical root resorption. Although this case report presents a favorable outcome, further studies are encouraged to support the use of Biodentine to fill external ICR defect.


Purpose: To increase the awareness of longus colli tendinitis (LCT) among spine specialists and to present a practical overview of diagnostic and treatment options, so that unnecessary interventions are avoided. Five sample cases from a German spine center will also be presented.Methods: Literature review and case series. A PubMed search was performed in May 2015, and the articles found were reviewed for clinical presentation, investigations, and treatment. The frequency of publication of LCT cases and the specialty of journals were also noted. Recent cases treated in our institution were also reviewed. The clinical findings, investigations, and therapeutic interventions were summarized.Results: The PubMed search from May 2015 found 104 articles, published over 51 years, on the topic of LCT. Only four were published in spine journals. A review of this literature yielded a total of 242 cases. The classic clinical triad included neck pain, limitation of movements, and swallowing complaints. C-reactive Protein (CRP) values were available in 21 cases (mean 23.66 mg/dL). A contrast-enhanced computed tomography (CT) scan was the best diagnostic modality. LCT is usually a self-limiting condition, but non-steroidal anti-inflammatory drugs (NSAIDs) may help alleviate discomfort. Five cases of LCT were diagnosed and treated in our center over the past three years.Conclusions: LCT, which is uncommon and has non-specific symptoms, is often referred to spine centers. Spine specialists should be aware of its clinical presentation and radiographic findings in order to avoid unnecessary interventions. The condition is self-limiting and can be treated conservatively. Calcific aseptic tendinitis of the longus colli, also known as calcific retropharyngeal tendinitis or calcific prevertebral tendinitis, was first described in Swedish by Fahlgren and Loefstedt in 1963 . The patLongus colli tendinitis (LCT) is not listed among the differential diagnoses for neck pain in many textbooks, which generally only include more common possibilities such as inflammatory, infectious, and rheumatic conditions . FurtherThe aim of this review is to increase the awareness of LCT among spine surgeons. We present here five cases of LCT from our institution, and we also review the literature and summarize information regarding clinical findings and useful diagnostic tools. To the best of our knowledge, this is the first comprehensive review of LCT papers and cases published between its first recognition as a condition in 1964 up until 2015 and is the first summary that puts the information contained in these papers into a practical context.A PubMed search was performed in May 2015 using “longus colli tendinitis”, “prevertebral tendinitis”, and “retropharyngeal tendinitis” as keywords and covered the years from 1964 until 2015. Only English-language, full-text articles were reviewed. The following data were extracted and recorded: number of cases, epidemiological characteristics, symptoms, examination findings, diagnostic investigations (laboratory and imaging), therapeutic measures, and duration until resolution of symptoms. The collected data were summarized in order to help develop possible diagnostic and therapeutic guidelines for LCT. Specialty and subspecialty journals were also reviewed to determine the number of LCT cases per publication, per year from 1964 to 2015.Five cases of LCT have been diagnosed in our institution over the last three years. Here we describe the presentation, therapeutic interventions, and clinical course. Examples of key imaging studies are also provided. None of the below-mentioned cases reported any history of recent trauma or whiplash injury.Consent was obtained from the patients for the reporting of their cases.3/mm3  and C-reactive Protein (CRP) value of 7.8 mg/dL . Computed tomography (CT) and magnetic resonance imaging (MRI) were done and showed prevertebral soft tissue swelling and calcifications anterior to C1 at the insertion of the longus colli especially affecting the left side (A 45-year-old lady presented to the emergency room (ER) with severe neck pain radiating to the left shoulder and arm, torticollis, and dysphagia for about two days. A clinical examination revealed limited cervical movement in all directions with exacerbation of pain on extension of the head. The primary clinical diagnosis in ER was cervicobrachialgia. The laboratory investigations revealed a leukocyte count of 11.5 × 10eft side . After rA 50-year-old man was referred to our hospital with severe occipital headache (VAS 9), odynophagia. The CRP was 38 mg/dL. The main differential diagnosis was a retropharyngeal abscess, so the patient was first admitted to the ENT department where a clinical examination revealed posterior pharyngeal edema. Antibiotics and anti-inflammatory drugs were prescribed. A contrast-enhanced CT was done and revealed the prevertebral calcifications. The neuroradiologist suggested the diagnosis of longus colli tendinitis that was confirmed with contrast-MRI. A soft cervical collar and analgesics were prescribed. Symptoms rapidly improved after three days that the patient could be discharged. The patient reported complete remission of symptoms after seven days.A 63-year-old lady was presented to the ER with acute neck pain and dysphagia. She was afebrile. Laboratory investigations showed elevated CRP (39.7 mg/dL). MRI with contrast was done and showed inflammatory changes and contrast enhancement at the insertion of longus colli without prevertebral effusion. CT revealed the calcifications and confirmed the diagnosis of LCT.3/mm3 and 51.3 mg/dL consecutively). Contrast-MRI revealed prevertebral edema, prevertebral effusion, and bone marrow edema of the anterior arch of atlas at the insertion of the upper oblique fibers of the left longus colli muscle mimicking spondylitis. CT scan was done and revealed the prevertebral calcifications. Under NSAIDs the inflammatory parameters were decreasing. Soft cervical collar helped to alleviate discomfort. Remission of symptoms was complete after 10 days.A 48-year-old man referred to our center with severe left-sided neck pain and dysphagia. Severe limitation of movements in all directions was evident on examination. The leukocyte count and CRP were elevated  were published between 2008 and 2015 . Twenty-3/mm3 (range 5.3–21 × 103/mm3), the term “normal” was used to describe the leukocyte count in 10 cases, while in four cases the leukocyte counts were described as “increased” but no exact values were provided. Erythrocyte sedimentation rate (ESR) values were available in 45 cases with a mean of 31.57 mm/h (range 5–98 mm/h). CRP values were available in 21 cases with a mean of 23.66 mg/dL (range 0.3–91.3 mg/dL). In 15 cases the CRP values were normal.Some laboratory information was generally provided in the cases reported. The leukocyte count was available in 48 cases with a mean of 11.58 × 10Computer tomography (CT) and Magnetic resonance imaging (MRI) were the diagnostic imaging modalities described in the later literature. CT was used for diagnosis in 88 cases, and MRI was used in 51 cases. Before 1986, the diagnosis was mainly based on history, clinical data, plain X-rays and, in few cases, polytomography. In those cases which used CT or MRI, calcifications were evident in 185 cases (about 76.44%), and in 190 (78.51%) cases, prevertebral soft tissue swelling or effusion was present. In 14 (5.7%) cases, there was no comment on the presence of effusion or soft tissue edema, while in six cases soft tissue swelling or effusion was noted to be absent. In cases where CT or MRI was not done, an increased prevertebral soft tissue shadow on the lateral plain X-ray of the cervical spine was taken to indicate soft tissue swelling or effusion.There were reports of unnecessary interventions or treatments. In five cases, fine needle aspiration from the retropharyngeal space was done. In another five cases, unnecessary surgical intervention was performed due to misdiagnosis , 7. AntiThe oral administration of NSAIDs was sufficient treatment in 131 cases. In 13 cases opioid-containing analgesics were added, in 22 cases steroids were administered, and muscle relaxants were added in five cases. Thirty-two patients received antibiotics either before confirmation of the diagnosis as LCT or as adjuvant to NSAIDs.Soft cervical collars were also used in 10 cases. The length of time required for resolution of symptoms was available in 76 cases with an average of 12 days (range ½–42 days). Many authors used non-numerical descriptions like “a few days” or “a few weeks”.LCT is an uncommon condition; however, accurate data regarding its incidence is lacking. Horowitz et al. estimated the mean annual crude incidence of LCT to be 0.50 cases per 100,000 persons-years. Although not common, LCT is probably severely underdiagnosed because LCT symptoms are non-specific, treating physicians are often unfamiliar with it, and it has a self-limiting pathology . The mosThe longus colli muscle lies in the prevertebral space, posterior to the retropharyngeal space. It is the deepest anterior cervical muscle and consists of three parts: upper oblique, vertical, and lower oblique . Upper oLCT commonly affects the C1/2 level or the upper oblique part of the muscle. Few studies reported a more caudal focus of calcification with the incidence of calcific tendinitis at the origin of the longitudinal part of the muscle mainly at the C4/5 and C5/6 levels –16.The term “retropharyngeal calcific tendinitis” is somewhat misleading as the disease actually begins within the prevertebral space, sometimes but not always extending into the retropharyngeal space. Hence, the term LCT or acute calcific prevertebral tendinitis (ACPT) is preferred .Although the calcifications appear to induce the inflammatory response, the precise mechanism is not yet fully understood. This is similar to the idiopathic nature of calcium hydroxyapatite deposition disease (CHADD), and by definition, LCT falls into the spectrum of CHADD. It has been proposed that calcium deposition may be due to local or systemic metabolic derangements, such as those following ischemia, necrosis, or trauma . That isClinical symptoms of LCT are non-specific and variable including pain and stiffness in the neck , shoulder and arm pain, odynophagia, dysphagia, a choking sensation, upper back pain, occipital headache, dizziness, nausea, and torticollis. The duration of symptoms before presentation varies between one day and three months . In mostThe presenting pattern is that of gradually increasing pain in the neck and throat, sometimes with radiation to the occiput. This pain is aggravated by swallowing and motion of the head or neck. There is usually marked limitation of the extension of the neck. There may be mild fever and elevation of the sedimentation rate. The pain usually reaches a maximum at 2–4 days, at which point the patient typically seeks medical help. After reaching its peak, the pain gradually subsides and is usually resolved within 1–2 weeks .The headache associated with LCT generally begins around the same time as the onset of the retropharyngeal tendinitis, significantly worsens with progression of the condition, is aggravated by extension of the neck, rotation of the head and/or swallowing, and is usually associated with tenderness over the spinous processes of the upper three cervical vertebrae , 21.Plain radiographs can reveal an increased soft tissue shadow in front of the upper cervical spine and sometimes amorphous calcifications anterior to the C1/2 vertebrae. CT more clearly demonstrates the calcification and soft tissue swelling. A contrast-enhanced CT scan with tissue phase is the examination of choice for diagnosis confirmation as it reveals best the retropharyngeal edema centered on the calcification and rules out infectious, traumatic, or neoplastic etiologies .A CT-guided biopsy of the prevertebral changes may be recommended in cases where the differentiation from a retropharyngeal abscess is difficult.MRI can also visualize the edematous longus colli muscle. The diffuse swelling of the muscle is associated with prominent high signal intensities in T2-weighted images . STIR (SClinically and radiographically, LCT sometimes mimics spondylodiscitis especially when associated with bone marrow edema at the insertion of the muscle . In our Other investigations and interventions include CT brain to exclude subarachnoidal hemorrhage or other causes of headache and lumbar puncture to exclude meningitis , 25.There is a long list of differential diagnoses to consider when a patient presents with the symptoms of LCT. Other conditions to be considered include: retropharyngeal abscess, adenitis, retropharyngeal cellulitis, pharyngitis, acute thyroiditis, traumatic injuries, spondylitis, meningitis, subarachnoid hemorrhage, and rheumatoid conditions.LCT is also counted among the painful, non-infectious craniofacial and cervical musculoskeletal pathologies, such as temporal tendinitis, Eagle’s syndrome, glossopharyngeal neuralgia, and carotidynia.Finally, it is also necessary to differentiate radiographically LCT from accessory ossicle, which is a normal variant found in the same location. This ossicle has well-defined borders and no soft tissue swelling. Styloid processes can also be misinterpreted as calcifications .A lack of awareness of this entity and its radiological findings can result in a misdiagnosis and unnecessary interventions, such as the incision and drainage of the retropharyngeal space, especially if retropharyngeal abscess is suspected. This could cause undue discomfort and delay in discharge from the hospital .LCT is a self-limiting condition. Symptoms usually resolve spontaneously within 2–3 weeks. Administration of NSAIDs usually alleviates the acute symptoms. Sometimes a soft cervical collar is prescribed.Although LCT is an uncommon condition with non-specific and heterogeneous presentations, spine surgeons with either an orthopedic or a neurosurgical background should be aware of the condition in order to avoid unnecessary investigations or interventions. Of course, priority must first be given to ruling out more serious and possibly life-threatening conditions. Likewise, other spine-related conditions including spondylitis, spondylodiscitis, and traumatic upper cervical injuries must be excluded.The authors have no conflict of interest.


Lymphangioma is a benign congenital malformation. The extremely rare and atypical localization of a lymphangioma in the chest wall was the real motive for the present case study. A 5-year-old boy was admitted to the Emergency Department of the 1st Department of Pediatric Surgery, Aristotle University of Thessaloniki, due to the presence of a mildly painful swelling in the left lateral chest wall, which was first noticed three months ago, after a blunt injury during sport. Physical examination revealed the presence of a palpable, spherical, painful, nut-sized subcutaneous lesion in the left lateral chest wall, respectively, with the anterior axillary line, at the height of the 6th to 7th intercostal space. Presence of ecchymosis on the overlying skin was also noticed. During palpation, we did not notice fluctuation, while transillumination was not feasible. Performance of ultrasonography, including Doppler color flow imaging, followed, depicting a subcutaneous cystic lesion, 2.1⁎3.2 cm in dimensions, without extension to the thoracic cavity. Scheduled surgical excision of the lesion was decided. Histopathological examination documented the diagnosis of cystic lymphangioma. Patient is still followed up on a 6-month basis. He remains asymptomatic, after 2 years, without indication of relapse. Lymphangioma is a benign congenital malformation characterized by proliferation of lymphatic vessels, resulting from the failure of communication between the primitive lymphatic sacs and the venous system, leading to the formation of a cystic structure . Rarely,Based upon the histopathologic features, lymphangiomas are divided into three subtypes: the cystic (macrocystic) lymphangioma, which is the most common, the capillary (supermacrocystic) lymphangioma, and the corpus  lymphangioma, which is the most rare . MacrocyTheir incidence in pediatric population rises up to 1 new case per 12000 births, while lymphangiomas represent almost 5-6% of all tumors in childhood . HancockA 5-year-old male patient was admitted as an outpatient to the Emergency Department of the 1st Department of Pediatric Surgery, Aristotle University of Thessaloniki, due to the presence of a mildly painful swelling in the left lateral chest wall, which was first noticed about three months ago, after a blunt injury in the context of participation in sport. Physical examination revealed the presence of a palpable, spherical, painful, nut-sized subcutaneous lesion in the left lateral chest wall, respectively, with the anterior axillary line, at the height of the 6th to 7th intercostal space. Presence of ecchymosis on the overlying skin was also noticed .∗3.2 cm in dimensions, without extension to the thoracic cavity. Solid insertion to the underlying epimysium was probable, according to the imaging findings. Lesion's periphery was lobulated, while no vascular structures, but thin septula (depicted as hyperechoic reflections), were found within it.Transillumination of the lesion was not feasible. No pathological signs were detected in face and left profile chest radiograph. Performance of ultrasonographic evaluation, including Doppler color flow imaging, followed, depicting a subcutaneous cystic lesion, 2.1Scheduled surgical excision of the lesion was decided, after conduction of the typical preoperative control, which was normal. With the patient under general endotracheal anesthesia, we conducted a cross section above the lesion, following the direction of Langer's lines. We then performed separation of the adhesions between the lesion and the adjacent subcutaneous fat tissue, but also of the adhesions between the posterior surface of the lesion and the underlying fascia of the anterolateral chest muscles Figures .The lesion was finally excised, without incision. We noticed the hemorrhagic perfusion of its capsule .Hemostasis and repair of the surgical wound according to the anatomic order followed.Patient was easily awakened after the completion of the surgical procedure. Postoperative course was uneventful and he was discharged home on the 1st postoperative day in excellent general condition. Histopathological examination of the excised lesion documented the diagnosis of cystic lymphangioma. It highlighted the presence of a thickened fibrous capsule invested by a flat endothelial layer, on the outer surface of which irregular striated muscle and elastic fibers were found. Thin septula were found within the lesion, while the subcavities were mainly occupied by hemorrhagic material. Adipose tissue in the vicinity of the lesion was also found, corresponding to the subcutaneous fat tissue surrounding the lymphangioma Figures  and 5.Patient is still followed up on a 6-month basis. He remains asymptomatic, after 2 years, without indication of relapse.Goldstein et al. focus on the early prenatal diagnosis of lymphangiomas, between the 15th and 22nd week of gestation, in their study . They coDespite the presence of a cystic lymphangioma at birth in 50% of all cases and by the age of 2 years in 90% of all cases, diagnosis may be delayed significantly, as in our case , 19–22. Physical examination and ultrasonography substantially contribute to the diagnostic approach of a chest wall cystic lymphangioma . TransilFindings of ultrasonography and Doppler color flow are crucial for the preoperative evaluation, highlighting the cystic structure, the hypoechoic content, the lobulated periphery, the absence of vessels, and presence of thin septula . In theiIn cases of diagnostic doubt, conduction of CT or MRI may be required, in order to determine the possibility of intrathoracic extension of the lymphangioma and the affinity with the adjacent anatomic structures , 24, 25.Surgical excision of a chest wall cystic lymphangioma is the treatment of choice . Other sIntralesional sclerotherapy (IS) constitutes an alternative treatment option in cystic lymphangiomas. Cahill et al. treated 17 patients with head and neck cystic lymphangiomas (10 macrocystic and 7 microcystic) by intralesional injection of doxycycline. The authors observed significant improvement in 11 out of 17 patients, while 8 out 11 featured a macrocystic type . ImpelliWe believe that complete excision is feasible and safe in cases of wall-localized cystic lymphangiomas, when performed by an experienced and skillful surgeon. In those cases, possibility of iatrogenic injury in the adjacent structures is minor. Besides, IS does not lack complications. According to previously mentioned study by Cahill et al., main observed complications were hemolytic anemia and delayed neurologic involvement .However, when surgical intervention is not feasible, due to either the large dimensions or the increased likelihood of iatrogenic injury to adjacent anatomic structures, Turner et al. and Reinhardt et al. suggested either the conduction of systemic chemotherapy or the administration of interferon-a, but with poor results , 37.Ozeki et al. administered propranolol at a single daily dose 2 mg/kg in 6 children with cystic lymphangioma, aged from 10 months to 19 years, over a period of 6 months . As prev2-40 and Lymphatic Vessel Endothelial Receptor-1 (LYVE-1) may be needed when lymphangioma should be differentially diagnosed from hemangioma or hemangioma-lymphangioma, by highlighting the presence of lymphatic endothelial cells on its inner surface, in cases of lymphangioma [Histopathologic examination documents the diagnosis of a cystic lymphangioma, as in our case. The presence of striated muscle tissue adjacent to the posterior surface of the lymphangioma was indicative for its solid adhesion to the anterolateral chest muscles. Immunohistochemistry by using markers such as DSince a cystic lymphangioma is fully excised, then there is practically no possibility of relapse , 41, 42.


Ganglioneuroblastoma, nodular is defined as a composite tumor of biologically distinct clones. The peripheral neuroblastic tumors in this category are characterized by the presence of grossly visible neuroblastoma nodules coexisting with ganglioneuroblastoma, intermixed, or with ganglioneuroma. Making a correct diagnosis of ganglioneuroblastoma, nodular is often difficult by biopsy or partial tumor resection, because the neuroblastic nodule could be hidden and not sampled for pathological examination.MYCN, the boy was treated according to the intermediate-risk protocol and is now alive and well 4 years after the diagnosis.We report a case of a Japanese boy aged 3 years, 8 months, with an unresectable abdominal tumor and elevated vanillylmandelic acid and homovanillic acid levels. The initial biopsy was ganglioneuroma. However, after the second biopsy from a hidden neuroblastoma nodule that was clearly highlighted by fluorodeoxyglucose positron emission tomography/computed tomography, we reached the diagnosis of ganglioneuroblastoma, nodular. Because the nodule demonstrated neuroblastoma, differentiating subtype, with a low mitosis-karyorrhexis index (favorable histology) and nonamplified This case illustrates the critical role of fluorodeoxyglucose positron emission tomography/computed tomography for detecting a neuroblastoma nodule in a ganglioneuroblastoma. Peripheral neuroblastic tumor (pNT), derived from sympathoadrenal lineage of the neural crest, is one of the most common extracranial solid tumors in children. The International Neuroblastoma Pathology Classification (INPC) distinguishes four categories of pNTs as follows: neuroblastoma ; ganglioneuroblastoma, intermixed ; ganglioneuroma ; and ganglioneuroblastoma, nodular  , 2. INPC123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy. However, detecting/sampling the NB nodule for molecular testing and histopathological classification is essential for appropriate clinical management of patients with GNBn.Among these tumor categories, GNBn is defined as a composite tumor of biologically distinct clones. pNTs in this category are characterized by the presence of a grossly visible NB nodule coexisting with GNBi or GN. Making a correct diagnosis of GNBn is often difficult by biopsy or partial tumor resection, because the NB nodule could be hidden and not sampled for pathological examination. Although the nodule is frequently hemorrhagic and/or necrotic, it may not be easily detectable by conventional imaging analyses and We report a case of a pediatric patient with a huge and unresectable stage 3 pNT. After the initial biopsy diagnosis of GN, the final diagnosis of GNBn was made on the basis of the result of the second biopsy from the NB nodule, which was clearly identified/located by fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT). Successful clinical management of the patient was accomplished on the basis of the molecular and histopathological characteristics of the NB nodule of this case.123I-MIBG scintigraphy revealed no metastatic spread. The patient’s urinary catecholamine metabolites were markedly elevated; his vanillylmandelic acid (VMA) level was 48.7 μg/mg creatinine ; and his homovanillic acid (HVA) level was 221 μg/mg creatinine .A Japanese boy aged 3 years, 8 months was presented to our institution with marked abdominal distention. CT revealed a huge mass with calcification, measuring 13 cm in diameter, in the median abdominal region. The tumor, encasing bilateral common iliac arteries, was stage 3 according to the International Neuroblastoma Staging System [123I-MIBG, but the neuroblastic nodule was difficult to locate  of 4.20 in the primary tumor mass  (< 100/5000 cells) and favorable histology according to the INPC Fig. . Fluores123I-MIBG avid foci and normalized VMA/HVA levels, whereas the tumor size had slightly increased.According to the COG criteria, the patient was in the intermediate-risk group, and he was treated with one cycle of cyclophosphamide/vincristine/pirarubicin, two cycles of cyclophosphamide/vincristine/carboplatin, and six cycles of cyclophosphamide/vincristine/pirarubicin/cisplatin, in accordance with the protocol of the Japan Neuroblastoma Study Group. At the time of chemotherapy completion, the tumor had not shrunk, and the patient’s urinary VMA/HVA levels remained elevated. Because the GNBn in this case was biologically favorable, we opted for clinical observation without further therapy. Four years after the diagnosis, the patient was doing well with no detectable et al. first described a composite tumor that contained a malignant NB nodule coexisting with ganglioneuromatous tissue [et al. clearly defined the morphological characteristics of this category as pNT and named it “stroma-rich (ganglioneuroblastoma), nodular” [et al. identified two prognostic groups in this category, and the results were confirmed by the committee in 2003 [et al. reported the results of the study of the International Neuroblastoma Risk Group, and the event-free and overall survival rates of GNBn tumors in the favorable histology group were 87 ± 8% and 91 ± 6%, respectively, whereas those in the unfavorable histology group were 45 ± 6% and 61 ± 5%, respectively [In 1947, Stout nodular” . In 1999nodular” . In 2000 in 2003 . BrieflyFor clinical management of patients with pNT, detection/sampling of the NB nodule is critical when present and coexisting with GNBi or GN. However, in our routine practice, we could miss the nodule by biopsy or partial resection of the tumors that show only GN or GNBi histology. Currently, the COG Neuroblastoma Pathology Reference Laboratory recommends a disclaimer of “based on review of limited material” in the diagnosis line after “GN or GNBi, favorable histology” in the surgical pathology report. In this clinical situation, the COG Neuroblastoma Pathology Reference Laboratory also recommends measuring VMA/HVA levels, because hidden NB nodules can often be the source of high elevation of these markers. By contrast, GN and GNBi tumors, predominantly or mainly composed of Schwannian stroma, usually do not produce higher levels of VMA/HVA.123I-MIBG scintigraphy has been widely used for the diagnosis of pNTs, it was also not helpful for identifying the NB nodule in the otherwise GN background of our patient. As reported by Decarolis et al., 24.5% of GN tumors and 61.1% of GNBi tumors showed MIBG uptake, so that any NB nodule, when present, may not be highlighted by this scintigraphy method [max 4.10) in the NB nodule [max 2.02) in GN tissue [In our patient’s case, we conducted imaging analyses for detection of the NB nodule. Conventional CT and magnetic resonance imaging were not useful for this purpose. Although y method . HoweverB nodule  in contret al. [et al. [123I-MIBG avidity and/or elevated catecholamines. Considering the characteristics of the NB nodule (favorable histology and nonamplified MYCN) in our patient, we adopted an observational approach to wait for age-linked tumor maturation, regardless of persistently high levels of catecholamines. As clarified by Decrarolis et al., the large GN portion of this tumor, which is composed of abundant Schwannian stromal cells, would not shrink after chemotherapy [et al., incremental increase in tumor volume may result from the proliferation of Schwannian stroma cells in the course of the maturational step of a biologically favorable NB nodule to GN [Management of residual tumors after completion of chemotherapy has been controversial. Marachelian et al.  and Ieha [et al.  demonstrotherapy . As suggle to GN . After 4123I-MIBG scintigraphy for detection of NB nodules in GNBn.In summary, we report a case of a pediatric patient with unresectable stage 3 GNBn. Biopsy of the NB nodule, which was clearly highlighted by FDG PET/CT, led us to successful clinical management of the patient. Our report suggests the potential usefulness of FDG PET/CT rather than


Pediatric germ cell tumors account for approximately 3.5 % of all childhood cancers for children under the age of 15 years. Up to one-third are extragonadal neoplasms. Germ cell tumors are a heterogeneous group of malignant tumors with a wide variety of histopathological features. Yolk sac tumor is the predominant variant in newborns and younger children. We report for the first time, the presentation of a primary yolk sac tumor in the abdominal wall of a small child.An 18-month-old white girl underwent resection of a small, round subcutaneous lump (1.5×1.3×0.8 cm) of the abdominal wall in her right hypochondriac region. The histopathology was compatible with yolk sac tumor. Her alpha-fetoprotein was initially elevated but normalized after the resection. Magnetic resonance imaging of her abdomen was normal. The surgeon decided to observe and follow her alpha-fetoprotein level closely. One year after resection a local recurrence appeared and her alpha-fetoprotein rose to 58 ng/mL. The surgeon performed a wide resection of the lesion with normalization of her alpha-fetoprotein. Follow-up consisted of measuring alpha-fetoprotein, clinical evaluation, and abdominal ultrasound.Clinicians should be aware that a yolk sac tumor can present in an unusual extragonadal place, for example in this case it was subcutaneous. In some cases, conservative treatment can be carried out with careful monitoring of the patient and their alpha-fetoprotein. Pediatric germ cell tumors (GCTs) account for approximately 3.5 % of all childhood cancers for children under the age of 15 years. Between the ages 15 and 19 the frequency goes up to 16 %. Up to one-third are extragonadal neoplasms and the most common sites are the sacrococcygeal or retroperitoneal region, and the pineal gland. The incidence of extragonadal tumors varies widely by age (higher in younger age) and gender  [The only known risk factor for extragonadal GCTs is the presence of Klinefelter syndrome . In that case, there is a 50-fold increased risk of developing mediastinal GCTs in early adolescence , 3.GCTs are a heterogeneous group of malignant tumors with a wide variety of histopathological features. Yolk sac tumor is the predominant variant in newborns and younger children, while later in life a wide range of histologic subtypes are seen. Yolk sac tumors have a microcystic reticular pattern and are cytokeratin-positive. Alpha-fetoprotein (αFP) expression is characteristic and can be used for diagnosis and monitoring of therapy. To the best of our knowledge, this is the first report of the presentation of a primary yolk sac tumor in the abdominal wall of a small child. After resection of the tumor, close monitoring was conducted, without any adjuvant chemotherapy.An 18-month-old white girl underwent resection of a small, round subcutaneous lump 1.5×1.3×0.8 cm) of the abdominal wall in her right hypochondriac region. The tumor was connected to her skin and had the macroscopic appearance of a pilomatrixoma . Surprisingly the histopathology was compatible with yolk sac tumor, showing a microcystic reticular pattern Fig. . The res×0.8 cm oGCTs consist of neoplastic cells arising from germ line cells (egg or sperm). They occur either in the testis or ovary, or outside the gonads. The extragonadal location is caused by either malignant transformation of aberrantly migrated primordial germ cell misplaced during the embryogenesis, or by a metastatic lesion of an undetected primary gonadal GCT not yet macroscopically visible or already spontaneously regressed. Extragonadal yolk sac tumors are rare and typically occur in midline locations, other sites have rarely been described . Yolk saet al. studied 25 children with malignant GCTs of the abdomen and retroperitoneum as the primary site and examined survival and event-free survival rates using high-dose or standard-dose cisplatin-based combination chemotherapy and surgical resection for these patients [et al. reported an overview of primary skin GCTs, 16 of the 19 patients were children, and a mature teratoma was the most frequent diagnosis [et al. described a 3-year-old girl with a subcutaneous paraspinal yolk sac tumor with metastatic disease located in the scar tissue at the surgical site and lumbar vertebrae. She received chemotherapy  and has been in remission for several years [Billmire patients . Most tupatients . Maubec iagnosis . Tedgϋndal years . No otheIn yolk sac tumors, the tumor marker αFP is extremely sensitive for diagnosis and in follow-up after the appropriate treatment has been given . αFP is αFP is regarded as a characteristic tumor marker of malignant GCTs and epithelial liver tumors. It is not tumor-specific. Elevated αFP in the serum of a child is also associated with benign conditions, for example hepatic disorders, hereditary disorders , systemic lupus erythematosus, and other malignant tumors  .In regards of the patient presented, after the first resection, it was decided to observe her closely and not perform a wide resection. After local recurrence with an elevated αFP, a wide resection was done. No distant primary disease was found. Despite the availability of highly effective chemotherapy, we decided to observe. If the tumor reoccurs, then effective therapy can still be given. Due to the localization and the elevation of αFP we could easily perform intensive surveillance by monthly measurement of her αFP and yearly ultrasound of her abdomen (watch-and-wait strategy).We describe the first report of an 18-month-old girl diagnosed with a yolk sac tumor subcutaneously. A year after resection, she presented with local recurrence, which was treated with a wide resection only. Five years of follow-up did not reveal signs of local recurrence or distant disease. It is important that clinicians are aware that yolk sac tumor can present in an unusual extragonadal place. In some cases, conservative treatment can be carried out with careful monitoring of the patient and their αFP.


Chordomas are rare malignancies of bone origin that occur in the axial skeleton, typically the skull base and lumbar/sacral regions. Although often classified as low-grade neoplasms, its locally infiltrative behavior may result in significant morbidity and mortality. Optimal surgical resection may be curative, but up to 50% of the cases relapse within 5 years, and currently there are no systemic treatments approved in this setting. A large proportion of these tumors express stem-cell factor receptor (c-KIT) and platelet-derived growth factor receptors (PDGFRs), providing a rationale for the use of tyrosine-kinase inhibitors (TKIs).  A 27-year-old male presented with recurrent chordoma of the lumbar spine 4 years after initial diagnosis. Salvage therapies in the interval included repeat resections and radiation therapy. He ultimately developed multifocal recurrence not amenable to complete excision or reirradiation. A comprehensive genomic profiling assay was performed and revealed nondrugable alterations. Decision was made to proceed with systemic treatment with pazopanib 800 mg/day, resulting in tumor reduction (−23.1% reduction in size) and prolonged disease control.  For this patient with a multiple recurrent chordoma and limited treatment options, pazopanib resulted in sustained clinical benefit following initial tumor reduction. Chordomas are uncommon neoplasms that originate from embryonic remnants of notochord and account for only 1–4% of all bone tumors. They predominate in men around the sixth decade of life, and have an approximate incidence of 0.1–0.8 per 1,000,000 individuals per year . ApproxiEn bloc resection with negative margins remains the treatment of choice [f choice , 6. Howef choice , 4, 5.α (PDGFR-α), platelet-derived growth factor receptor β (PDGFR-β), and vascular endothelial growth factor (VEGF) [For patients with advanced disease or refractory to conventional therapeutic modalities, there are no systemic treatments approved for clinical use to date. It is estimated that approximately 75–90% of chordomas exhibit positive immunostaining for tyrosine kinase receptors involved in signaling pathways related to cell proliferation and angiogenesis, such as the stem-cell factor receptor (C-KIT), platelet-derived growth factor receptor r (VEGF) . Based or (VEGF) . Howeverr (VEGF) .In this report, we describe the case of a 27-year-old male diagnosed with a lumbar spine conventional chordoma with multiple recurrences following surgery and radiotherapy, who presented an important clinical and radiological response to pazopanib.A 27-year-old white male presented in December 2015 for evaluation of a relapsed conventional chordoma arising from the lumber spine. His diagnosis dated back to August 2013, when he underwent additional evaluation due to a recurrent lower back pain. Magnetic resonance imaging (MRI) of the lumbar spine and sacrum revealed a solid expansive lesion on L2 topography, with an extensive epidural soft tissue component, infiltrating the bone marrow and determining compression of the dural sac. At that time, a core biopsy was performed, which revealed a lesion consistent with a grade 2 chordoma with positive immunostaining for pan-cytokeratin (AE1/AE3), S-100 protein, epithelial membrane antigen (EMA), and Ki-67 index of 20%. The immunohistochemical assessment of brachyury was not conclusive. Three months after the diagnosis, the patient underwent L2 vertebrectomy with negative microscopic margins that confirmed the diagnosis of chordoma of the lumbar spine.Surgical treatment was followed by adjuvant intensity-modulated radiotherapy (IMRT) to the tumor bed (45 Gy in 25 fractions), and he was then managed expectantly until September/2015, when he presented with disease relapse. MRI of the lumbosacral spine showed multiple lesions of cystic appearance and of small dimensions distributed in paravertebral soft parts at the level of L2, in the right psoas muscle and in the vertebral bodies of T11 and T12, measuring up to 2.2 cm, the largest diameter, without significant contrast enhancement. A new biopsy was then performed and reviewed by a reference pathologist, with histological confirmation of atypical chordoma. The neoplasia showed an expansive and multilobulated pattern of invasion and aspect of chordoma, with epithelioid and eosinophilic cells in the chondromyxoid matrix background. The mitosis figures were observed up to 04/10 high-power fields (HPFs) Figures . There wA repeat MRI performed after 1 month revealed an increase in previously existing lesions, compression of the dural sac and medullary cone by a lesion extending from T11 to T12, and additional noncontiguous lesions at the level of L2 and multiple nodules measuring up to 1 cm in the right posterior abdominal musculature. In view of the spinal cord compression, a new surgical approach was recommended, and the patient underwent a T11-T12 level partial resection/decompression.Eight months later, disease progression was once again documented in the thoracolumbar transition and the patient underwent another surgical procedure, with partial resection of the epidural lesion between T11 and T12, as well as the additional lesions between L1-L2 and L3. One month later, the control MRI showed an increase in the dimensions of the preexisting lesions in the lumbar spine and in the abdominal musculature, without new foci. The patient was then treated with image-guided radiotherapy (IGRT) at the dose of 54 Gy in the surgical bed and in regions of gross disease . One month after the end of the radiotherapy treatment, a new MRI was performed Figures , which iATM Q2593∗ mutation, ARID1A S2149fs∗47 mutation, CDKN2A/B loss, EP300 L415P mutation , and MLL3 Y306∗ mutation. INI-1/SMARCB1 was included in the panel and showed no mutations. Despite the rationale for the use of poly (ADP-ribose) polymerase (PARP) inhibitors in patients with loss-of-function ATM mutations, there were no ongoing clinical trials for those alterations at that time.Considering the lack of salvage therapies available in this scenario, a FoundationOne®Heme genomic profiling assay was requested, which revealed 5 nondrugable genomic alterations: Given the limited therapeutic options in the scenario, systemic treatment with pazopanib 800 mg/day was recommended. The patient developed grade 3 neutropenia, requiring dose reduction , with subsequent good tolerance.Restaging scans were performed following approximately 3 months of treatment with pazopanib, showing a reduction in size of previously described lesions in anterior paravertebral region, right psoas, and abdominal musculature, and stable dimensions of remaining foci of disease; no new lesions were characterized Figures . The sumWe report a case of a patient with a multiple recurrent lumbar spine chordoma initially treated with gross total resection with wide margins followed by adjuvant radiation therapy. Despite multimodal therapy, consisting of multiple surgical resections and reirradiation, the patient experienced locoregional disease progression and distant metastasis. Prolonged disease control following initial tumor reduction was achieved with the introduction of pazopanib, suggesting a role of this agent in the management of this challenging disease.en bloc surgical resection with wide margins remains the standard treatment; nevertheless, recurrences may develop in up to 50% of the cases. Radiotherapy is often used in patients presenting with locally advanced disease not amenable to complete excision or in scenarios in which surgery with negative margins is not feasible. For patients who fail standard approaches, treatment alternatives are limited to “salvage” re-resection with curative intent in a very small percentage of patients who present with isolated lesions, long disease-free interval, good performance status, and acceptable morbidity; for those with multifocal recurrences, a history of piecemeal resection, tumor rupture, or prior treatment with high-dose RT, curative-intent therapies are definitely not recommended based on most recent guidelines from the Chordoma Global Consensus Group [Although considered low-grade neoplasms that usually exhibit an indolent clinical course, conventional chordomas have a significant negative impact on patients' quality of life and may result in pronounced morbidity . Completus Group . The lacus Group , 3, 4.α/β and VEGF [β overexpression, with clinical benefit demonstrated in 64% of patients and median PFS of 9 months [β), stable disease was observed in 80% of the cases (4/5 cases) of the chordoma cohort [Based on the frequent expression of markers associated with aberrant activation of signaling pathways related to cell proliferation and angiogenesis, including C-KIT, PDGFR-and VEGF , 8, multand VEGF . A prosp9 months . In anota cohort . The lara cohort .In imatinib-pretreated patients, a study performed by Stacchiotti et al. comprising 10 cases of chordoma (9 arising in the sacrum and 1 in the skull-base) reported a PFS rate at 6 months of 87% with the combination of imatinib and sirolimus, supporting the role of the mammalian target of rapamycin (mTOR) pathway as a mechanism of resistance to imatinib in these tumors . The useSaccharomyces cerevisiae-based vaccine encoding brachyury (GI-6301) brought a new insight into the treatment of chordomas [β and mTOR pathways in advanced chordomas, we wait for the outcomes of the phase II study testing the efficacy of imatinib and everolimus combination therapy (EudraCT 2010-021755-34).More recently, the development of a recombinant hordomas . In the In conclusion, chordomas are challenging neoplasms with an unpredictable clinical course and a high chance of relapse. Patients presenting with multiple recurrent disease bring an unmet need for systemic therapies. Despite the lack of randomized trials, this case report adds to the available literature suggesting a role for the use of pazopanib.


The aim of this report is to highlight the challenges in diagnosis and management of this condition.Peripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1–C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor.We present a case of 5-year-old primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine.After this illustrative case, we review the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral  Primitive neuroectodermal tumors (PNET) are heterogeneous group of malignant neoplasms that occur mostly in childhood and early adulthood . These tA 5-year-old Moroccan boy presented with a 2 months history of torticollis with radiation of the pain to the left upper limb. The patient had no medical history. Physical examination revealed hypoesthesia of the upper limbs, evidence of bilateral lower extremity weakness with motor power 4/5 in distal muscular group, and hypoactive reflexes in four extremities. There was no bowel or bladder dysfunction.Cervical CT-scan and MR imaging demonstrated the presence of a 75 × 36 × 24 mm extradural mass, with extra-spinal extension at the left C1–C6 level   chromosomal translocation is supporting the diagnosis of pPNET. Due to limited evidence regarding the therapeutic aspects of these tumors, no definite protocol can be formulated for their treatment, but resection, radiotherapy, and multipotent chemotherapy are the preferred treatment options for these patients. Clinical outcomes still need to be evaluated in prospective trials.


Hepatic Angiosarcoma is an extremely rare malignant neoplasm of the vascular or lymphatic endothelium accounting for about 2% of all sarcomas. It is considered idiopathic in up to 70% of cases. We describe the case of a 32-year female transferred to our center for evaluation of intractable gastrointestinal bleeding. Definitive diagnosis remained elusive despite multiple endoscopic assessments with repeated cauterization of arteriovenous malformations (AVMs). Autopsy results confirmed metastatic hepatic Angiosarcoma. Hepatic Angiosarcoma is an extremely rare malignant neoplasm of the vascular or lymphatic endothelium accounting for about 2% of all sarcomas . AngiosaWe describe the case of a 32-year-old female with a past medical history of anemia who was transferred to our center for further assessment and management of multiple medical problems including anemia, GIB, hemoptysis, and reportedly lung and liver lesions. She reported to the outside center with a 7-month history of progressive fatigue, hemoptysis, splinter hemorrhages and diffuse joint pain that involved both knees, hips, and left wrist. At the outside hospital her severe anemia persisted despite 9 units of PRBC transfusions. She underwent extensive assessment including esophagogastroduodenoscopy (EGD) and colonoscopy. EGD revealed gastric lesions consistent with AVMs while colonoscopy was negative. A bronchoscopy was negative but CT imaging showed liver and lung lesions of unknown etiology. On presentation to our center, vitals and physical examination were unremarkable. Initial blood work was remarkable for anemia, thrombocytopenia and hypokalemia. Repeat EGD and capsule endoscopy revealed active bleeding in the duodenum secondary to diffuse mucosal vascular lesions. Due to persistent bleeding she was transferred to the intermediate care unit (IMC) for closer monitoring. She continued to receive transfusions of PRBCs, FFP, cryoprecipitate, and platelets on a daily basis. During her hospitalization, she received a total of 4 EGD, 2 capsules and a push enteroscopy with similar findings of blood in the stomach, duodenum and small bowel with scattered AVMs, which were repeatedly cauterized. A bone marrow biopsy with pathology was inconclusive but concerning for possible angiosarcoma. A PET CT skull base to mid thigh did not show any evidence of malignancy but revealed diffuse low-level activity through out the axial skeleton that was deemed secondary to increased hematopoiesis. About 2 weeks following admission to our center, the patient became hypotensive from worsening hematemesis. She was transferred to the medical ICU. In the ICU, she continued to require daily transfusions of blood products with persisting GI bleed. She was started on estrogen/progesterone therapy, Avastin and aminocaproic acid for suspected hereditary hemorrhagic telangiectasia (HHT). Subsequently, she developed fulminant DIC and was rapidly transfused blood, factors, and platelets with improvement. A few days later, she developed respiratory failure requiring intubation and mechanical ventilation in addition to hemorrhagic shock requiring pressors and continued transfusions. Given her extremely poor prognosis, and through multiple discussions, the family decided against further escalation of care. Autopsy revealed angiosarcoma most likely arising from the liver and spreading to the lungs, spleen, mediastinal lymph node, gastrointestinal tract, skin and bone marrow. The liver appeared to be the primary site due to the size of the lesion involving approximately 75% of liver compared to the diffuse patchy involvement of lungs and spleen. Immunohistochemistry with CD31 and CD34 which are markers specific for endothelial cells and vascular tumors such as Angiosarcoma positively stained the tumor , 2. The Hepatic Angiosarcoma is an extremely rare malignant neoplasm of the vascular or lymphatic endothelium accounting for about 2% of all sarcomas . It is cWe believe that the case outlined above shows that metastatic angiosarcoma of any organ system should be suspected and actively investigated in any case of intractable gastrointestinal bleeding. This could lead to early diagnosis and definitive treatment which in many cases is surgical resection.


Endodontic-periodontal diseases often present great challenges to the clinician in their diagnosis, management, and prognosis. Understanding the disease process through cause-and-effect relationships between the pulp and supporting periodontal tissues with the aid of rational classifications leads to successful treatment outcomes. In this report, we present several treatment modalities in patients with different endodontic-periodontal lesions. A modification to the new endodontic-periodontic classification, Al-Fouzan's classification, was also added. The first case was classified as retrograde periodontal disease . The second case was diagnosed as an iatrogenic periodontal lesion caused by root perforation. The third case was diagnosed as an iatrogenic periodontal lesion caused by tooth trauma due to orthodontic treatment. The first two cases were managed with a nonsurgical approach, whereas the third case was managed with nonsurgical and surgical approaches. All patients showed complete healing of soft and hard tissue lesions. A thorough understanding of the disease history and the patient's signs and symptoms, complete examination with full investigation, and the use of a systematic step-by-step approach in the management of such challenging endodontic-periodontal lesions with regular recall visits were very useful and successful. An endodontic-periodontal lesion consists of concurrent pulpal and periodontal disease in the same tooth. The spread of infection between the dental pulp and the periodontal ligament can occur through the apical foramen, lateral canals, dentinal tubules, and palatogingival grooves. Moreover, nonanatomic factors can have a role in this communication such as iatrogenic root canal perforations or a vertical root fracture. These pathways cause the spread of infection and bone destruction in a coronal-to-apical direction in the case of periodontal infection or in an apical-to-coronal direction in the case of endodontic infection .Primary endodontic lesion with drainage through the periodontal ligamentPrimary endodontic lesion with secondary periodontal involvementRetrograde periodontal diseasePrimary periodontal lesionPrimary periodontal lesion with secondary endodontic involvementCombined endodontic-periodontal lesionIatrogenic periodontal lesionIn 1964, Simring and Goldberg  were theThis rational classification increases the understanding of the disease process and its origin. This understanding is essential in determining the correct diagnosis and providing treatment with predictable success. The success rate of joined endodontic-periodontal lesions without a regenerative procedure is between 27% and 37% . These rA 55-year-old woman with a noncontributory medical history was referred to the endodontic specialist clinic at Prince Sultan Military Medical City  complaining of intraoral sinus with pus drainage in the right mandibular molar area. Clinical and radiographic examinations revealed a large amalgam restoration with recurrent caries and periapical and furcal radiolucency related to tooth #46. There was localized swelling in the gingival sulcus and the sinus in the gingival area. Tooth mobility was grade II. A midbuccal area with a narrow periodontal pocket > 10 mm was noted. The tooth had a negative response to the thermal vitality test . The diagnosis was a necrotic pulp and chronic apical abscess.Endodontic treatment was accomplished in two visits with calcium hydroxide [Ca(OH)2] medication between appointments. Local anesthesia  was administered, and the tooth was isolated with a rubber dam. An access opening was created and four canals were located. During the second visit, the localized swelling and sinus opening were thoroughly resolved, and the root canal treatment was completed with RaCe NiTi rotary files  and 5.25% sodium hypochlorite irrigation. The tooth was obturated with lateral condensation of gutta-percha and AH Plus sealer . No periodontal treatment was administered. Follow-up X-ray images were obtained from 1 year to 6 years, which showed complete healing of the bone in the periapical and furcation areas .A 30-year-old woman with a noncontributory medical history was referred to the endodontic specialist clinic at PSMMC complaining of pain and localized intraoral swelling in the left first mandibular molar. One year earlier, the tooth had undergone root canal treatment with cementation of the post and core and placement of a permanent crown. The clinical examination revealed localized swelling in the gingival sulcus. Periodontal probing through the furcation showed increased probing values with a grade II defect  Figure . The radAfter the administration of local anesthesia , the crown was removed with a crown removal instrument, and the post was removed with an ultrasonic instrument using a light brush and cutting motion to break up the cement around the post. A paper point was used to check for the presence of blood spots to determine the location and size of the perforation. The perforation site was irrigated with 2.5% sodium hypochlorite followed by drying of the canal and sealing of the perforation with mineral trioxide aggregate  mixed with saline and placed with a microapical placement system  and condensed with a paper point . A wet cThe patient was referred to the prosthodontics department for permanent crown placement. Follow-up appointments at 3 months, 6 months, 9 months, and up to 4 years showed complete healing of the soft tissue and bone lesions and a normal pocket depth of 3 mm.A 27-year-old woman with a noncontributory medical history was referred from the orthodontic department at PSMMC complaining of pus discharge from the gingival sulcus and slight gingival swelling on the palatal side opposite tooth #22  after orthodontic treatment. Clinical and radiographic examinations revealed a sound tooth #22 with a mobility of grade II and a deep periodontal pocket > 10 mm mesial to tooth #22 with pus discharge from the pocket . The theThe diagnosis was necrotic pulp due to trauma during orthodontic treatment with symptomatic apical periodontitis. The first stage of the treatment plan was endodontic treatment, which was performed during two visits with calcium hydroxide medication between appointments . ChemicoLocal anesthesia  was then administered labially and palatally. A mucoperiosteal flap was raised mesially to the upper left canine tooth, and two vertical releasing incisions were formed in the anterior palatal area opposite teeth #21 and #23. A horizontal incision was formed from the left maxillary central incisor to the left maxillary canine. After flap reflection, a sling suture was placed in the tissue flap to secure it with the premolar tooth on the opposite side of the maxillary arch to aid the surgeon in improving visual and operative access by eliminating the need to manually retract the flap in the palatal area  [8]. CorThe management of endodontic-periodontal lesions is a true challenge for the dentist because of the deleterious effects on the tooth structure and the supporting periapical structures . The key to success in treating these cases depends on taking a correct history to determine the cause and reach an exact diagnosis of the case development. In addition, a clinician's ability to classify a lesion makes the treatment strategy or protocol very clear and precise. In this report, several successful endodontic-periodontal lesion cases were presented with different treatment modalities and classified according to the new classification system reported by Al-Fouzan in 2014 [Root perforation is an unnatural communication between the root canal system and the supporting tissues of the teeth or the oral cavity . One stuMineral trioxide aggregate is a bioceramic material, composed of tricalcium silicate, tricalcium aluminate, tricalcium oxide, and silicate oxide, that forms a colloidal gel on hydration and solidifies in approximately 3 hours. The calcium oxide in MTA reacts with tissue fluids to form calcium hydroxide, which may then encourage hard tissue deposition because of its high pH. In addition, several studies –15 have Dental pulp is a soft connective tissue encased in a rigid, noncompliant chamber; therefore, changes in pulpal blood flow or vascular tissue pressure can have serious effects on the health of the pulp. Many studies , 17 haveIn endodontic-periodontal lesions, the cause of the lesion is endodontic or periodontal in origin, and the patient may benefit from undergoing root canal therapy first , 21 becaRegenerative techniques involve cell differentiation, cell proliferation and induction, and/or tissue formation conduction. All of these factors work together to complete the healing of damaged periapical tissues. This healing can be obtained by using bone grafts , periodontal membranes , growth factors, or a combination of these , 23.In the first two cases, soft and hard tissue healing commenced with administering root canal therapy alone or perforation repair. However, in the third case, follow-up for 3 months after completing the root canal revealed a persistent bone defect and periodontal pocket, which indicated the need for a further treatment modality to restore the health of the periodontal tissues.Von Arx and Cochran , DietricControversial findings challenge the current understanding of the ideal interval between endodontic treatment and periodontal surgery. It has been reported that root canal treatment performed 10 weeks, 3 months, or 6 months before periodontal surgery did not impair periodontal healing –34. In t


Background: The present case report describes an extremely rare case of a norepinephrine secreting extraadrenal paraganglioma (PGL) located in the seminal vesicle.Case Presentation: A 36-year-old patient had signs of intermittent hypertensive derailments, bradycardia, increased norepinephrine excretion in 24-hour urine, an increased metanephrine plasma concentration, and a positive clonidine suppression test. A suspicious mass was detected in an (18)F-DOPA-PET/CT-scan in the left seminal vesicle. Following adrenergic blockade, a robotically assisted laparoscopic left vesiculectomy with negative soft tissue surgical margins was performed. The patient sustained a couple of few months of voiding difficulties of the lower urinary tract and obstruction of the left upper urinary tract after the surgery, which resolved spontaneously with home medical treatment. Two years after the initial treatment, the patient relapsed, which was confirmed by endocrinologic follow-up tests with increased urine catecholamine, a positive clonidine suppression test, as well as an elevated blood pressure. Staging with (18)F-DOPA-PET/CT-scan confirmed the diagnosis of a recurrent PGL. This was followed by subsequent open surgical removal of the suspicious lesion in the seminal fossa. The patient is still recurrence free 15 months after the second surgery. Complications after the second surgery included an intermittent paresthesia of the left leg lasting 3 to 4 months. No other urologic symptoms such as voiding or erectile dysfunction occurred.Conclusion: DaVinci-assisted laparoscopic vesiculectomy is a viable procedure to treat such cases providing satisfactory results. Relevant for clinical practice are the regular and lifelong follow-up examinations to detect recurrences. They originate from chromaffin cells in the paraganglia in localizations such as the retroperitoneum and thorax in the sympathetic system or they can be of parasympathetic origin and are localized, for example, in the aortic arch, neck, and skull base.1P2 Most PGL are benign tumors, but depending on the location and presence of genetic mutations, the probability of malignant PGL reaches up to 25% metastatic potential. The highest rate of metastases occurs in hereditary PGL syndromes and is associated with mutations of genes of the succinate dehydrogenase complex (SDH gene). The majority of PGL-related metastases occur in local and distant lymphatic nodes, bones, liver, and lungs.PGL may produce and release surplus catecholamines (mostly normetanephrine and norepinephrine) into the blood. This leads to intermittent or permanent hypertension, episodic sweating, headaches, and palpitations.2–4Optimal treatment of regional PGL that have spread to neighboring organs or lymph nodes consists of complete surgical removal of the tumor.5–7A localization of a PGL in small pelvis in male is extremely rare. Moreover, so far, only three cases have been described involving the seminal vesicle.A 36-year-old patient presented initially with intermittent hypertensive derailments (RR 220/180 mm Hg) of a known arterial hypertension, accompanied by low heart rates of 40/minute at the Heidelberg University Hospital's Endocrinology Department in January 2013 for further diagnostic procedures.Arterial hypertension was initially diagnosed in 2009. Since then, recurrent hypertensive crises had occurred. The hypertensive episodes were accompanied by weakness, paleness of the face, serious holocephalic headache, nausea, and hyperhidrosis.The thyroid metabolism in terms of TSH and fT3 values was normal. Also, all the tumor markers such as the carcinoembryonic antigen, the prostate-specific antigen (PSA), and the calcitonin levels were within the normal range. Urologic symptoms such as voiding difficulties, urinary incontinence, erectile dysfunction, or other symptoms involving the lower or upper urinary tract were not present. Physical examination showed no abnormalities. Renal retention parameters were unremarkable. However, an increased noradrenaline excretion in 24-hour urine and an increased metanephrine level in plasma were determined. A clonidine suppression test was carried out in January 2013 and showed an insufficient suppression suspicious of PGL with DD pheochromocytoma (PHEO) of norepinephrine phenotype. However, an external CT scan of the abdomen did not show pathologic changes and in particular no suspicious adrenal masses.For further localization of the assumed lesion, a 131IMIBG scintigraphy was performed in February 2013, without clear evidence of a neoplastic lesion. The case was presented in the interdisciplinary tumor conference. For further diagnostics, a CT and (18)F-DOPA-PET/CT examination were performed, which detected a suspicious lesion measuring 3.6 × 2.8 cm in the left seminal vesicle  and a maximal width of 3.5 cm. The tumor was composed of confluent nests of moderately polymorphous, polygonal tumor cells , the patient wrote a voiding diary in June 2013. After exclusion of mechanical obstructions of a lower urinary tract, a urodynamic examination was performed showing a normal and stable bladder function, normal capacity of the bladder, and a normal detrusor pressure build-up during voiding without any evidence of neurogenic disorder. To stimulate the bladder function, postoperative medical therapy with pyridostigmine (cholinesterase inhibitor) and tamsulosin  was initiated, which resulted in a normalization of the micturition.Moreover, the suprapubic catheter was removed 4 months postoperatively after improvement of the residual urinary amounts and urine flow. After the surgery, the potency was preserved.The initial follow-up was undertaken ex domo in March 2015.The endocrinologic tests identified an increased metanephrine level in 24-hour urine, slightly elevated blood pressure, and showed a positive result during the clonidine suppression test. A repeat (18)F-DOPA-PET/CT imaging  was present in the current case, no signs of hematuria, hematospermia, metastatic disease, or genetic mutation were presented. LUTS were at the present case and for the other three cases were not present.5–7 PGL can be divided into functional and nonfunctional types due to their ability for synthesis, storage, and secretion of catecholamine.2 In the current case, the patient presented with hypertension due to catecholamine excess.The clinical presentation and symptoms: one case presented with signs of acute appendicitis,The primary treatment of extraadrenal PGL consists of surgical resection of the tumor after appropriate pretreatment with alpha- and beta-blockers to block the catecholamine receptors and prevent sympathetic overload and perioperative cardiovascular complications. This is the first published case of a DaVinci-assisted minimally invasive surgery to remove a tumor mass from the area of the seminal vesicle. As for tumor recurrence, after initially negative soft tissue margins, it cannot be clearly established whether it is residual tissue after primary surgery or a second separate tumor.8Advantages of a robotically assisted laparoscopic retroperitoneal approach are minimal surgical trauma, less blood loss, fewer hospital days, less pain, better presentation of the surgical area do to optical magnification, faster healing, and early recovery.9–11So far, only three case reports with benign cystadenoma of the seminal vesicles have been described in the literature, which were effectively treated using DaVinci robot-assisted surgical resection.vs minimally invasive laparoscopic surgery can be considered. After surgical removal of the tumor, a significant reduction or normalization of catecholamine release can be observed, reducing the need for an adrenergic blockade, daily medication intake, and a better response to the subsequent therapy.12In the case of tumor recurrence, an open surgery with laparotomy 13If left untreated, PGL may lead to elevated cardiovascular morbidity and mortality rate.14In the primary tumor resection, a complete tumor removal should be performed. If possible, a careful and precise dissection should be carried out to prevent capsule rupture, as this may contribute to/predispose to late tumor recurrences in the surgical bed and seeding.Work-up of the histology specimen and diagnosing are difficult. There are no reliable criteria that indicate a malignant behavior. The definitive criterion of a malignant PGL, according to the WHO, is the development of metastases. In this case, a small size constant lung lesion was present for years.In summary, most cases of PGL in the small pelvis are nonfunctional, surgical tumor excision is in most cases curative, and late recurrence is not common. Genetic testing for mutation was not routinely performed.In the event of paroxysmal severe headache with hypertensive derailments and vegetative symptoms, a catecholamine excess syndrome should be considered. The determination of metanephrine and normetanephrine levels in plasma and urine is a suitable screening test for catecholamine release in PGL. PGL are unusual in the small pelvis. Nevertheless, they should be included in the differential diagnosis of an incidentally found mass with features suggesting a vascular tumor. Possible symptoms may include LUTS, hematuria, headaches with an unknown cause, hematuria, hemospermia, hypertension or upper urinary tract obstruction. Including PGL in the differential diagnosis may facilitate biopsy diagnosis and guide further therapy in case of functionally dormant tumor before or during a surgery or a biopsy. An interdisciplinary approach with precise imaging to differentiate PHEO and PGL followed by sympathetic blockade and minimally invasive surgical tumor removal are recommended for therapy at centers with appropriate expertise to ensure a favorable outcome. Relevant for the clinical practice is a lifelong follow-up and regular examinations of patients to detect and prevent late recurrences or delayed appearance of multiple tumors.Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We present the case of a young female patient who presented to the outpatient department with a history of bleeding per vagina, diagnosed with leiomyosarcoma of the cervix; the patient underwent total abdominal hysterectomy with pelvic lymph node dissection. In this article, we mainly discuss multimodality therapy in the management of an unusual variety of tumour in the uterine cervix. Leiomyosarcoma is one of the rare histopathological variants of tumours of the cervix comprising nearly 1% of overall tumours of the cervix. Diagnosis is made on the basis of histopathological findings and immunohistochemical analysis. The treatment of choice in such patients is a triple therapy comprising surgery, chemotherapy and radiation therapy [A twenty-year-old female patient presented with a history of increased blood flow during her menstrual cycles for the past 3 years. She was symptomatically treated. However, as her symptoms did not subside, she sought further treatment elsewhere, where magnetic resonance imaging (MRI) of the pelvis in August 2017 showed a T1W hypointense, T2W and SPAIR hyper-intense polypoidal mass arising from the cervix measuring 6.2 × 6.9 × 7.2 cm with mild diffusion restriction and the mass was filling the vaginal fornices .A provisional diagnosis of a cervical polyp was made and she underwent a hysteroscopic polypectomy on 8 September 2017. The post-operative histopathology report was in favour of a high grade or poorly differentiated malignancy with the possibility of pleomorphic spindle cell sarcoma/carcinosarcoma  and 4.3 arising from the uterine cervix, located predominantly on the proximal vagina with relatively well-defined margins and no evidence of significant regional lymphadenopathy (Fluoro-deoxy-glucose positron emission tomography - computed tomography (FDG-PET CT) done on 9 October 2017 showed an exophytic mass measuring 7.0 × 5.5 × 6.3 cmenopathy .The hysteroscopic polypectomy slide was reviewed, suggestive of poorly differentiated malignant tumour with high grade sarcomatous content.2 involving predominantly the anterior lip of the cervix, OS (opening into uterus) was not visualised and bilateral fornices free. She underwent radical hysterectomy type III and bilateral salphingo-oophorectomy with regional pelvic lymph node dissection and omentectomy on 28 October 2017. The post-operative histopathological examination report showed undifferentiated endocervical sarcoma of cervix with all resected lymph nodes, margins and omentum free of tumour  and docetaxel (75 mg/m2) given every 21st day until the end of March 2018. The patient tolerated the adjuvant treatment without any issue.The patient and her family members were counselled about the condition and treatment options, regarding the role of adjuvant radiotherapy in view of the high degree of proliferative activity and were planned for 50 Gy in 25 fractions followed by adjuvant chemotherapy. The patient has completed adjuvant external beam radiation therapy (EBRT)  and intrThe incidence of leiomyosacroma is less than 1% of all uterine malignancies .et al [et al [Regarding the epidemiology of the tumour, it commonly presents during the 4th–6th decades of life. It is rarely seen in a young age group similar to the patient in our case report . Whitcomet al  reportedet al . Bansal l [et al  analysedl [et al .Leiomyosarcoma presented with nonpuerperal inversion of the uterus was treated in a 49-year-old nulliparous woman which is an extremely rare event and rare mode of presentation .Ovarian involvement in sarcomas of the cervix, in particular to leiomyosarcoma of the cervix, is a very rare mode of presentation. In view of the rarity of ovarian involvement, the role of oopherectomy in sarcomas of the uterine cervix is a debatable issue. As similar to the other histological variants of sarcoma with some exceptions, the mode of spread to distant sites is through the blood. Lung is the most common site of metastasis .et al [Casanova et al  reportedet al .Diagnosis of leiomyosarcoma of the cervix is based on histopathological findings. Microscopic study shows spindle-shaped cells can be seen which may be both hypercellular and hypocellular in nature. The presence of tumour cell necrosis, mitotic index of 10/10 high power field and atypia of cells ranging from moderate to diffuse are histopathological diagnostic criteria . VariousThere are no well-established risk factors in the aetiology of leiomyosarcoma of the cervix, various risk factors postulated in the aetiology are radiation exposure to the pelvis in childhood; tamoxifen which is an hormonal drug used in breast carcinoma is postulated in the aetiology without any proper explanation for the associated risk; Epstein–Barr virus infection in immunocomprised status is also recognised as an etiological factor without any proper explanation. Patients with abnormal hereditary retinoblastoma gene are also at an increased risk .Signs and symptoms are bleeding per vagina, discharge per vagina, pain and fullness in the pelvic and abdominal region. Most of the tumours are bulky at the time of presentation ranging from 10 to 12 cm in size. Various pressure symptoms such as increased frequency of micturition and sometimes urinary retention can also be seen. The patient is presented with h/o weight loss due to decreased appetite.Since it is a rare disease, there is no definitive guideline for management of leiomyosarcoma of the cervix. Most recommended treatment is multimodality comprising surgery, total abdominal hysterectomy with bilateral salpingo-oophorectomy in patients having disease confined to the cervix, followed by post-operative radiation therapy to reduce the incidence of local recurrence. In view of the low incidence of lymph node involvement in leiomyosarcoma of the cervix, the role of prophylactic pelvic lymph node dissection along with primary surgery is debatable, unless there is gross pelvic lymph node involvement .The role of chemotherapy has been extrapolated from sarcomas of the uterine corpus; most commonly used drugs including doxorubicin and ifosfamide. This regimen has an overall response rate of 30% in advanced and metastatic forms of disease . Other cRadiation therapy is usually given in the adjuvant setting to prevent loco regional recurrence. The pattern of radiation therapy delivered is external beam radiation therapy dose consisting of 50 Gy in 25 fractions over 5 weeks with 2 Gy per fraction followed by brachytherapy consisting of 2–3 sessions of brachytherapy which can also be delivered during EBRT also to reduce the total duration of treatment which has an impact on locoregional control rates , 11. TheThe main treatment modality of this rare histopathological variant involving the cervix is multimodality management consisting of surgery, chemotherapy and radiation therapy. Multimodality management increases the survival rates in such patients .Leiomyosarcoma of the cervix must be treated with a multimodality management protocol in view of the rarity of the disease, due to the lack of guidelines for its management. Surgery is the main modality of treatment. Radiation therapy in the form of external beam irradiation and intravaginal brachytherapy improves the loco-regional control rate via decreasing the loco-regional recurrence. The role of chemotherapy and drugs to be used are extrapolated from sarcomas of other anatomical areas and sarcoma of the uterine corpus.None


Malignant phyllodes tumors (MPT) are rare breast neoplasms. Preoperative diagnosis is often challenging due to the unspecific clinical, radiological, and histological characteristics of the tumor. Dissemination pathways are local with chest wall invasion, regional with lymph nodes metastasis, and distant, hematogenous, mostly to the lungs, bones, and brain. Distant metastasis (DM) can be synchronous or appear months to years after the diagnosis and initial management. The current report describes the case of a 57-year-old woman presenting with a giant/neglected MPT of the breast, with no DM at initial staging, treated by radical modified mastectomy. Motor disorders due to medullar compression by a paravertebral mass appeared at short follow-up, also treated surgically. The patient died from several DM of rapid evolution. To our knowledge, this is the only case described of MPT with metastases to soft tissue causing medullar compression. We present a literature review on unusual metastatic localizations of MPT. Phyllodes tumors (PT) are fibroepithelial tumors characterized by a double-layered epithelial component arranged in clefts surrounded by an overgrowing mesenchymal component organized in leaf-like structures. Grading between benign, borderline, or malignant depends on histological criteria: stromal cellularity, cellular pleomorphism, mitotic activity, margin appearance, and stromal distribution . The aveSurgery allowing tumor-free margins ≥ 1 cm is the first line of treatment . AxillarConsidering all PT, local recurrence (LR) and distant metastases (DM) rates are estimated around 20% and 3.5%, respectively –9. The eWe report the case of a woman with an atypical metastasis from a MPT with a rapidly fatal outcome. We present a literature review about unusual localizations of MPT metastasis.A 57-year-old menopausal woman presented herself to our institution with a giant necrotic breast tumor and ipsilateral axillary lymphadenopathy . RadicalFew weeks later, the patient came back with cervical and back pain. She also showed a delirious melancholic episode, successfully treated with neuroleptics.The spinal MRI revealed several paravertebral lesions: a right paravertebral soft tissue tumor extending from C3 to C5 causing spine displacement and a mass in T11-T12 causing mass effect on the conus medullaris . The occShe then presented multiple and rapidly growing metastasis in soft tissues. Chemotherapy with Adriamycin was initiated but she died rapidly, 4 months after primary diagnosis.We presented an unusual case of rapidly fatal metastatic evolution of a breast MPT with atypical distant metastasis to paravertebral tissues.In their retrospective analysis of 295 patients, Mitus et al. found that five-year DFS was 96.9% in patients with benign PT, 83.3% in patients with borderline PT, and 71.7% in patients with malignant PT .Several grading systems have been proposed, but the three-tiered system including benign, borderline, and malignant PT is preferred . The graThe patient waited for more than 18 months before consulting. She demonstrated a strong denial of the disease and of the treatments that were planned. In addition, this patient was in a situation of socioeconomic deprivation. Observing low-income women, Nonzee et al. studied the reasons of delayed breast cancer screening, follow-up, and treatment . They shSelection of review of literature for unusual metastasis is summarized in In case of initially nonmetastatic MPT with high risk of recurrence, adjuvant therapy including radiotherapy and/or different chemotherapeutic agents  can be used, although their role is uncertain . Here thRecently, the potential key role of genomic markers in the characterization of PT has been highlighted. MED12 somatic mutations have been identified as a highly recurrent event in fibroadenomas (FAs) and phyllodes tumors (PTs), with an inverse correlation between the frequency of this mutation and histologic grade –36. Laé MPT are uncommon breast neoplasm, whose prognosis can be very poor in case of DM. Medullar compression due to soft tissue metastasis is extremely rare. This strengthens the value of an accurate initial diagnosis, so as to enable the identification of high-risk patients. Their management, including monitoring and treatment, is yet to be determined, as the efficiency of treatments used for DM is still low.