Fibroma of tendon sheath (FTS) is an uncommonly encountered soft tissue mass, which is morphologically distinct from the more commonly seen giant cell tumor of tendon sheath (GCTTS). Initially described in 1936, FTS is typically a slow growing, painless, firm mass with a predilection for the upper extremity, frequently involving the hand. Cases of associated triggering or compression neuropathies have been described when underlying tendons or nerves are affected. Currently, the literature on FTS is sparse and largely limited to case reports. More recently, few reports of cytogenetic analysis on FTS have been reported in the literature. Cellular and chromosomal analysis of FTS tissue revealed chromosomal translocations with yet unknown clinical significance. Here, we present a case report of FTS in a 14-year-old male with a painless enlarging mass on the palmar side of the left hand treated by excision. Subsequent karyotypic analysis revealed a chromosomal translocation t , add (10)(q22)[24]. To our knowledge, this is the first description of this chromosomal aberration in the literature. Fibroma of tendon sheath (FTS) is an uncommonly encountered soft tissue mass, which is morphologically distinct from the more commonly seen giant cell tumor of tendon sheath (GCTTS). Initially described by Geschickter and Copeland in 1936, FTS is typically a slow growing, painless, firm mass with a predilection for the upper extremity .The literature on FTS is sparse and largely limited to case reports or small series –7. CommoMore recently, few reports of cytogenetic analysis on FTS have been reported in the literature. One case of FTS identified a clonal chromosomal abnormality t9;11) , while another identified a cellular abnormality t in half the cells sampled , 9. The ;11  , add (10)(q22)[24].A 14-year-old right-hand-dominant Caucasian male presented with a history for several years of a slowly enlarging mass of the left palm centered about the third metacarpal head . The masPlain radiographs did not show any abnormalities. Magnetic resonance imaging (MRI) with intravenous gadolinium demonstrated a 3.6 × 1.6 × 2.4 cm well-defined soft tissue mass centered about the third metacarpophalangeal joint abutting the flexor tendon sheath of the third digit . The masThe patient underwent excisional biopsy under general anesthesia with tourniquet control after gravity exsanguination. An incision was made in the distal palmar crease overlying the mass, and soft tissue dissection was carried down through the level of the palmar fascia. Intraoperatively, a solitary, solid tan-colored mass was encountered. It was well-encapsulated without communication to any neurovascular or tendinous structure aside from the A1 pulley of the third flexor sheath Figures  and 4. TFinal pathologic examination revealed a 3.8 × 2.5 × 1.3 cm spindle-cell lesion with mild lobulation. The spindle cells resembled fibroblasts, and no nuclear atypia was seen. The lesion was relatively hypocellular without necrosis or hemorrhage. Multinucleate giant cells were not seen . The tumThe patient's postoperative course was uneventful, and he returned to baseball activities 6 weeks postsurgery. He is currently at 21-month status postsurgical excision, reports pain-free activity, and exhibits no evidence of local recurrence .FTS is a relatively uncommon tumor that typically presents as a painless, slow growing, firm nodule in the palm or digits. Typically, FTS is seen in the young to middle-aged population, with some series reporting an up to 3 : 1 male to female ratio . UncommoA combination of history, clinical examination, and magnetic resonance imaging may raise suspicion for this tumor; however, FTS is most effectively differentiated from other soft tissue masses by its histopathologic features. FTS is comprised of fibroblast-like spindle cells with elongated, basophilic nuclei located within a dense collagenous matrix. Generally, there is no cytologic atypia or abundance of mitotic figures .The typical clinical course for FTS is usually uneventful and based primarily on location. Palmar or digital FTS may interfere with gripping activities or sports, as seen in our case. Proximity to neurovascular structures may present as compressive neuropathies with impairments in sensation or strength , 7, 14. st web space of a 38-year-old male. A unique t was identified , in a FTS excised from the palm of a 14-year-old male. As of yet, the clinical relevance of this translocation is unknown. This report provides a starting point for further research into the underlying significance of this abnormality. As seen with anomalies in the chromosome 2q and nodular lesions, specific genetic loci may have implications in the development of benign proliferations . Our stu


Testicular germ cell tumor is the most common cancer in 20-to 35-years-old men. There are known risk factors such as undescended testicle(s) and history of testicular cancer. Most lesions are germ cell tumors with two main subtypes: seminomas and non-seminomatous germ cell tumors. Testicular germ cell tumor is the most common cancer in 20-to 35-years-old men. There are known risk factors such as undescended testicle(s) and history of testicular cancer. Most lesions are germ cell tumors with two main subtypes: seminomas and non-seminomatous germ cell tumors. Burned out testicular tumor (BOTT) refers to a histological fibrous regression of the primary testicular lesion that generally presents at the stage of metastases . This caCase 1 - The first patient, a 57-years-old Caucasian man, came to the hospital with afebrile abdominal pain. Physical examination was normal. The biology laboratory results showed slight leukocytosis. Urine culture was sterile. Considering the abdominal pain with leukocytosis, we performed a contrast-enhanced CT-scan. The CT-scan showed a retroperitoneal hypodense mass containing gas . There wGiven a medical history of bilateral undescended testicles treated by orchidopexy in childhood, we carried out a testicular ultrasound (US) showing bilateral testicular atrophy, microlithiasis, and hypoechoic areas involving the left testis . NeverthThe patient underwent radical orchiectomy of the left testis and a seminoma with burned out main component was diagnosed. Finally, the patient underwent a fine needle biopsy of the mass revealing ischemic necrosis without any tumor cell.Case 2 - The second patient, a 41-years-old Caucasian man, came to the hospital for the same reason, afebrile abdominal pain. Physical examination was also normal. The laboratory results showed slight leukocytosis and urine culture was sterile. In the same way, a contrast-enhanced CT-scan was performed and showed the same results: necrotic retroperitoneal mass containing gas . The scrRadical orchiectomy was performed and pathological examination found hyaline fibrosis without any tumor cell consistent with BOTT . The CT Case 3 - The third patient, a 34-years-old Caucasian man came to the hospital with afebrile lumbar pains and presented similar physical examination  and laboratory results.The contrast-enhanced CT-scan also revealed a gas-containing retroperitoneal mass . The scrTo summarize, each patient presented with abdominal pain and leukocytosis revealing gas-containing retroperitoneal mass and hypoechoic hypovascular area with microlithiasis in the testis. In all three cases, tumor markers including lactate dehydrogenase, α-fetoprotein and human chorionic gonadotropin levels were normal.Pathological examination has been a challenge to diagnose BOTT with metastatic nodes  .After orchiectomy, each patient received chemotherapy by Bleomycin, Etoposide and Cisplatin. They are still free of disease after 14 years (Case-1), 8 years (Case-2) and 1 year (Case-3) of follow-up.Most reported BOTT are discovered because of symptomatic metastatic nodes, such as presenting with back or flank pain -5. GenerThe fibrous replacement and the residual seminomatous part in BOTT may be explained by an intensive immunological response. This mechanism has not been demonstrated in the case of BOTT but an autoimmune response was described in a testicular ‘in situ’ carcinoma . Indeed,The spontaneous regression of the testicular germ cell tumor results in hypoechoic avascular areas corresponding to hyaline fibrosis, and sometimes in atrophy of the testis .Due to the abundance of necrosis, it is sometimes impossible to identify any tumor cell in the retroperitoneal biopsy sample. In these cases, repeat biopsy may be needed. In the second case, the core biopsy was performed with a 16G needle.Orchiectomy is generally followed by cisplatin-based combination chemotherapy protocols. This therapy is very effective in the treatment of seminomas and non-seminomatous germ cell tumors.Presence of gas in a retroperitoneal mass is an uncommon presentation of metastatic retroperitoneal lymph nodes of testicular cancer.It is mandatory to perform a testicular ultrasound in the diagnostic process, despite, sometimes, a normal physical examination. Hypoechoic hypo/avascular areas at US may suggest the diagnosis of BOTT. Retroperitoneal biopsy with a large needle in the less necrotic part may be the key of the diagnosis.


This procedure is technically challenging and time consuming. In the present report we describe a novel technique, the extended subtotal mandibulectomy, as a possible alternative in cases of mandibular body tumors that have invaded the mandibular canal. This technique allows the complete excision of the mandibular canal whilst retaining the coronoid and condylar processes.Mandibular tumors in the oral cavity of dogs can be locally aggressive and infiltrative, involving adjacent soft and hard tissues. Tumors that invade the mandibular canal are considered likely to extend rostrally and caudally within that structure due to minimal tissue resistance. When this occurs, a total mandibulectomy is thought to be the treatment of choice as it allows  On oral examination the mass was noted to extend from the level of the right mandibular third premolar tooth to the first molar tooth. Tumor staging was performed via mandibular lymph node fine needle aspirates and thoracic and abdominal computed tomographic (CT) images. No evidence of regional or distant metastasis was noted. A CT scan of the head was performed at the same time and this found a mass measuring 16 × 40 × 26 mm and extending form the distal aspect of the right mandibular third premolar tooth to the mesial root of the first molar tooth. The right mandibular fourth premolar tooth was missing. The mass was largely on the dorsal and buccal aspect of the mandible and resulted in lysis of the underlying alveolar bone. The lytic process extended into the mandibular canal.Based on the clinical findings and the confirmed histological diagnosis of osteosarcoma, a right-sided extended subtotal mandibulectomy was performed. A surgical soft tissue margin of 15–30 mm was obtained around the gross tumor, including a skin wedge on the buccal aspect of the neoplasm. No surgical complications were encountered. Histological evaluation of the excised tissues later confirmed an osteosarcoma with tumor-free margins.The patient recovered uneventfully from general anesthesia and was monitored overnight for evidence of hemorrhage and assessed for signs of pain. A constant rate infusion (CRI) of fentanyl (3 μg/kg/h); as well as meloxicam , and ampicillin/sulbactam  were also administered. The patient ate well overnight and appeared comfortable. The patient was discharged to the care of its owners the following day with a fentanyl patch (50 μg/h), meloxicam , gabapentin , and amoxicillin/clavulanic acid . The owners were instructed to feed the patient a soft food diet and prevent any access to chews or toys for 3 weeks.Due to geographic distance, the patient was examined by the referring veterinarian 2 weeks postoperatively. According to medical records available, the surgical site had healed and the patient was reported to be considerably more comfortable, playful, and had regained a historical voracious appetite. A 1-month postoperative examination with the referring veterinarian revealed that the patient was comfortable with no gross evidence of reoccurrence of the oral tumor. Based on a follow-up phone call with the owner, the patient continued to be in good health with no evidence of recurrence of the tumor for another 19 months before being euthanized due to unrelated disease.A 9-year-old male castrated Labrador retriever dog was presented to CUHA's Dentistry and Oral Surgery Service for treatment of a left mandibular osteosarcoma located in the premolar and molar region. Tumor staging was performed via left and right mandibular lymph node fine needle aspirates and a thoracic and abdominal CT scan. No evidence of regional or distant metastasis was noted. A head CT scan performed at the same time found a mass measuring 23 × 55 × 10 mm and extended from the level of the left mandibular third premolar tooth to the mesial aspect of the second molar tooth. The left mandibular fourth premolar and first molar teeth were missing. Bone lysis was evident along the dorsal surface of the mandible and invasion of the mandibular canal was noted at the level of the tumor.Given the location and extent of the tumor, a left-sided extended subtotal mandibulectomy was performed. A surgical soft tissue margin of 15–20 mm was obtained around the gross tumor. No surgical complications were encountered. Histopathological assessment of the surgical specimen showed complete excision of the osteosarcoma.The patient recovered uneventfully from general anesthesia and was monitored overnight for evidence of hemorrhage, was assessed for signs of pain, and was maintained on intravenous fluids and administered a CRI of fentanyl (3 μg/kg/h) and dexmedetomidine (0.5 μg/kg/h), as well as ampicillin/sulbactam . The patient ate well overnight and appeared comfortable. The patient was discharged to the care of the owners with gabapentin (5 mg/kg q8–12 h), carprofen (2 mg/kg q12 h), and amoxicillin/clavulanic acid . The wonders were instructed to feed the patient a soft food diet and prevent any access to chews or toys for 3 weeks.The patient was assessed by the primary care veterinarian 2 weeks postoperatively and then again at our facility ~1 month postoperatively. The surgical site had healed well, and the patient was active and eating without difficulty. Right-sided mandibular drift and occlusal trauma caused by the right mandibular canine tooth to the palate was noted. A crown-height reduction and endodontic therapy of the canine tooth were offered. However, the owner declined due to financial constraints. Follow-up with an oncologist was recommended for ongoing monitoring. The owner was contacted again 8 months following the procedure and reported that the patient continued to do well with no evidence of tumor recurrence.A 9-year-old male castrated Pug dog was referred to CUHA's Oncology Service for evaluation of an incompletely excised oral melanoma at the level of the right mandibular third premolar tooth. On oral examination, no gross disease was evident. However, a scar and some suture material from the biopsy site were noted at the gingival margin at the level of the right mandibular second and third premolar teeth. The patient underwent tumor staging including an abdominal ultrasound, thoracic radiographs, and fine needle aspirates of the mandibular lymph nodes. No evidence of metastasis was found. A CT of the head was obtained and showed alveolar bone lysis and invasion of the tumor into the mandibular canal between the right mandibular first and third premolar teeth. The left and right mandibular lymph nodes were subjectively enlarged. Surgical excision and immunotherapy were elected as the treatment of choice and the patient was referred to the Dentistry and Oral Surgery Service.A right-sided extended subtotal mandibulectomy was performed. A surgical soft tissue margin of 10–20 mm was obtained around the visible scar. The left and right mandibular lymph nodes were also surgically excised and submitted for histopathological analysis. Histopathological assessment of the submitted mandibular tissues showed a narrow (2 mm) margin at the ventral aspect of the soft tissue margin rostrally. All other aspects of the resected mandible were tumor-free. Both the left and right mandibular lymph nodes were found to be reactive and contain draining melanocytes and melanophages. However, the neoplastic nature of the melanocytes was not clear to the pathologists.The patient recovered uneventfully from general anesthesia and was monitored overnight for evidence of hemorrhage and assessed for signs of pain. Intravenous fluids and a CRI of fentanyl (3 μg/kg/h) and dexmedetomadine (0.5 μg/kg/h) were administered, as well as meloxicam , and ampicillin/sulbactam . The patient was comfortable overnight and ate well when hand-fed. The patient was discharged to the care of the owners the following day with meloxicam , pregabalin , and amoxicillin/clavulanic acid . The owners were instructed to feed the patient a soft food diet and prevent any access to chews or toys for 3 weeks.The patient was examined 2-weeks postoperatively and was found to have healed well. A mild left-sided mandibular drift and tongue protrusion were noted. However, due to the patient's brachycephalic confirmation, there was no occlusal trauma. The Oncology Service commenced immunotherapy and monitoring. Eight-months postoperatively, recurrence of a pigmented mass in the rostral oral soft tissues, at the level of the previously reported narrow margin, was noted. The patient underwent a fine needle aspirate of the mass which was found to be a recurrence of the oral melanoma. Thoracic radiographs were obtained and no evidence of metastases noted. A full-thickness soft tissue excision with a 10 mm gross margin around the visible tumor was performed. The retropharyngeal lymph nodes were removed and submitted for histopathological analysis. Histopathological evaluation found tumor-free margins around the excised oral soft tissues. However, metastasis to the retropharyngeal lymph nodes was confirmed. The patient was examined 2-weeks postoperatively. The oral cavity had healed and the patient was reported to be comfortable and eating well. The patient returned to the Oncology Service for further monitoring and immunotherapy.In this report we describe the technical aspects of an extended subtotal mandibulectomy that can be applied for the treatment of invasive mandibular body tumors that have extended into the mandibular canal in dogs, and illustrate its application using three clinical cases.Excision of the mandibular body, from the level of the mental foramen to the mandibular foramen, is the accepted treatment modality for oral squamous cell carcinoma (OSCC) that has invaded the mandibular canal in human patients –13. The The surgical approach used to perform a total mandibulectomy in dogs is invasive, technically demanding and time consuming. It consists of the removal of the mandibular body and the ramus, and requires disarticulation of the temporomandibular joint. Additionally, extensive dissection of the insertions of the muscles of mastication  is required. With appropriate case selection, the described extended subtotal mandibulectomy technique achieves the same goal of complete mandibular canal resection. In addition, the procedure is considerably less invasive and time consuming, potentially reducing patient morbidity. Exact surgical time to complete this procedure was not included in this report due to a number of variables that were not controlled including; tumor size, complexity of the dissection, experience of the surgeon, small sample group, and the lack of a control group for comparison. Finally, the risk of hemorrhage from inadvertent trauma to the maxillary artery during temporomandibular joint disarticulation is eliminated . Based oAppropriate patient selection is critical when considering an extended subtotal mandibulectomy. Diagnostic imaging to assess the size, location, and extent of a tumor, in particular with reference to the mandibular canal and the ramus, is an essential step when determining the most appropriate surgical procedure. Computed tomography is considered the gold standard of diagnostic imaging of neoplasms affecting the maxillofacial region in cats and dogs . The exten bloc the entire mandibular canal while reducing surgical trauma and operating time. Although case number 3 had local reoccurrence of the oral melanoma, likely due to a narrow soft tissue margin, none of the cases had evidence of recurrence of the tumor more caudally, at the level of the mandibular foramen. Postoperative histological assessment of the excised tissues was only performed at the surgical margins. Future studies assessing the extent of various tumors along the mandibular canal, including perivascular and perineural involvement, would be of clinical importance in the future.The three cases described here had tumors of the mandibular body with clear invasion of the mandibular canal. Two of the cases were presenting with osteosarcomas and one case with oral melanoma. Both malignancies are locally invasive and destructive. The extended subtotal mandibulectomy was found to achieve the goal of excising en bloc excision of mandibular body tumors invading the mandibular canal. The technique, when applied to the appropriate case, provides the surgeon with the ability to adhere to sound oncological principles while reducing the necessity of the more invasive total mandibulectomy technique.We propose that the extended subtotal mandibulectomy is a viable surgical technique that allows for adequate All datasets generated for this study are included in the manuscript/supplementary files.The use of archived diagnostic material or review and data collection from medical records of client-owned animals for the purposes of this study was approved by Cornell University's Veterinary Clinical Studies Committee and was considered exempt from review by Cornell University's Institutional Animal Care and Use Committee.NF and SP contributed equally to the concept of the manuscript. The writing was primarily by NF.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


A 71-year-old man with known history of atrial fibrillation (treated with routine rivaroxaban therapy) was found to have incidental biochemical elevated calcium and parathyroid hormone (PTH) levels. His physical examination demonstrated the presence of a palpable right neck mass. Subsequent imaging studies revealed a large parathyroid mass as well as multiple bone lesions, raising the suspicion of parathyroid carcinoma. The anticoagulant therapy was stopped 5 days prior to his elective surgery. The night before his elective surgery, he presented to the emergency room with profound hypocalcemia. The surgery was postponed and rescheduled after calcium correction. Intraoperative findings and detailed histopathological examination revealed an infarcted 4.0 cm parathyroid adenoma with cystic change. His bony changes were related to brown tumors associated with long-standing hyperparathyroidism. Autoinfarction of a large parathyroid adenoma causing severe hypocalcemia is a rare phenomenon and may be considered in patients with large parathyroid adenomas after withdrawal of anticoagulants. Primary hyperparathyroidism (pHPT) is the most common cause of hypercalcemia in the outpatient, with an incidence between 27 and 30 per 100,000 person-years [In addition to illustrating this diagnostic uncertainty, this case report describes a scenario where severe hypercalcemia due to primary hyperparathyroidism was reversed following tumor autoinfarction after cessation of oral anticoagulation.A 71-year-old man, originating from Vietnam, was referred to a tertiary care endocrine surgery center with incidentally found severe hypercalcemia of 3.65  mmol/L during routine follow-up for atrial fibrillation. His only symptoms of hypercalcemia on further questioning were fatigue and polyuria. The patient's history included the left leg dystrophy due to childhood polio, hypertension, atrial fibrillation, chronic kidney failure (stage IIIB), and dyslipidemia. He had no family history of parathyroid or other endocrine disease, and he had no prior neck irradiation.On physical examination, a palpable mass was noted on the right lower neck without associated palpable lymphadenopathy. Flexible nasolaryngoscopy showed normal vocal cord movement, suggesting no invasion into the recurrent laryngeal nerve. Laboratory results showed markedly elevated levels of calcium at 3.3 mmol/L, PTH at 271 (1.4–7.6) pmol/L, alkaline phosphatase (ALP) at 446 (40–150) U/L, and phosphate of 1.06 (0.8–1.4) mmol/L. Ultrasound of the neck showed a complex, predominantly cystic nodule with solid components inferior of the right thyroid lobe measuring 4.1 × 3.6 × 3.1 cm .th rib. Differential diagnosis of the bone lesions included both metastasis as well as brown tumors secondary to long-standing hypercalcemia related to hyperparathyroidism. We did not evaluate the lesion with FNA preoperatively.Sestamibi scan ((99m)Tc) displayed a dominant right-sided neck lesion with minimal uptake of technetium. Additionally, two well-defined lucent bone lesions, located in the right clavicle and scapula, showed increased tracer uptake . SubsequDue to the clinical suspicion of parathyroid cancer, en bloc parathyroidectomy with the right thyroid lobectomy and ipsilateral level VI lymphadenectomy was planned. Rivaroxaban, a direct Xa inhibiting anticoagulant indicated for atrial fibrillation, was stopped 5 days prior to surgery. The evening before the scheduled surgery, the patient presented to the emergency department with symptomatic hypocalcemia including perioral, finger, and toe numbness. Laboratory results showed profound hypocalcemia with a corrected calcium level of 1.85 (2.32–2.62) mmol/L, phosphate of 0.61 (0.8–1.4) mmol/L, and a decrease in PTH level to 87.5 (1.4–7.6) pmol/L .Repeated ultrasound showed further avascular solid components of this parathyroid mass but no changes in size of the lesion. Parathyroid tumor autoinfarction was suspected with concomitant hungry bone syndrome. The surgery was postponed, and the patient was treated with high-dose calcium supplementation and activated vitamin D. After eucalcemia was achieved, the originally planned surgical intervention was performed.Rapid intraoperative PTH assays showed a significant drop of PTH after the removal of this mass . During the surgery, we used intraoperative nerve monitoring (IONM) for assessment of the right recurrent laryngeal nerve. The nerve signal was not lost throughout the operative dissection, and there were no adhesions to the recurrent laryngeal nerve from the parathyroid lesion. Postoperative movement of vocal cords was normal, calcium levels remained stable, and the patient was discharged 1 day after surgery.The right hemithyroidectomy specimen identified a 4.0 cm mass located at the right perithyroidal region. The mass was an infarcted enlarged cellular parathyroid gland with cystic degeneration . There wAt the 6-month follow-up, calcium and PTH levels remained improved and no persistence or recurrence of disease was suspected.Hypocalcemia after autoinfarction of a parathyroid adenoma is an extremely rare entity . This isPrevious literature proposed that fragile vascularization of parathyroid adenomas can lead to subsequent autoinfarction. The mechanism of this occurring in large parathyroid adenomas is focused on the solid aspects of parathyroid tissue, which are most sensitive to changes in vascular supply. This occurs around the rim of the tumor, while the center usually undergoes cystic degeneration . The ultAlthough calcium levels can normalize after autoinfarction of a parathyroid adenoma, surgical intervention is still warranted. This is based on previous studies where calcium normalization after autoparathyroidectomy with subsequent nonsurgical surveillance showed recurrence of hyperparathyroidism , 11. A sThe physiologic process of hypocalcemia after autoparathyroidectomy can be explained by the shift in balance of bone metabolism from osteoclastic bone resorption to enhanced osteoblastic bone formation due to the sudden relative decrease in PTH levels. Enhanced bone formation requires increased calcium uptake and consequent lower levels of circulating calcium. Patients with previous manifestations of high bone turnover or bone disease are prone to develop hungry bone syndrome after parathyroid resection, specifically those with high baseline ALP levels preoperatively as in this patient .One issue of diagnostic uncertainty was the presence of bone lesions signifying possible metastatic disease which was later determined to be brown tumors. These benign lesions, seen in up to 3% of pHPT patients, represent a reparative cellular process in regions with high osteoclastic bone turnover . Rapid hFinally, the lack of reliable tools to determine preoperative malignancy risk makes management of large parathyroid tumors difficult. Although parathyroid malignancy is the cause of pHPT in only 1% of the patients, it must be considered when levels of calcium and PTH are highly elevated in combination with a large palpable neck mass, especially if there is clear evidence of locoregional invasion or distant metastasis. Unfortunately, fine needle aspiration (FNA) cytology is unsuitable for the evaluation when parathyroid carcinoma is suspected. FNA can lead to tumor seeding and potentially cause parathyromatosis. In addition, previous interventions including FNA can also result in worrisome histological changes simulating malignancy . TherefoPathological analysis integrating the use of ancillary biomarkers remains the best diagnostic tool for malignancy –4.reviews the clinicopathological work-up and operative approach to primary hyperparathyroidism when there is suspicion of carcinomashows that autoinfarction of parathyroid tumors can mimic operative ablation in terms of physiologic effects on calcium homeostasis and that this should be considered in patients with significant decrease in calcium levels preoperatively, especially after withdrawal of anticoagulantsemphasizes that bone can be profoundly involved in benign parathyroid disease in the form of brown tumors, signifying long-standing hyperparathyroidismhighlights the clinical challenge in distinguishing benign and malignant parathyroid tumors due to the lack of definitive preoperative diagnostic toolsThis case report


Congenital extrahepatic portosystemic shunt is also known as Abernethy malformation. It is a rare anomaly of the portal venous system where partial or complete portal blood flow drains directly into the systemic vein via this abnormal shunt, bypassing the liver. Occasionally, this condition is incidentally detected on imaging studies performed for other indications. An early diagnosis is challenging due to the lack of specific symptoms—often leading to delayed diagnosis and development of subsequent complications.A 4-year-old boy presented with multiple cutaneous hemangiomas with concomitant splenomegaly. While the diagnostic work-up for the definite cause of splenomegaly was in progress, an Abernethy malformation was incidentally noted by abdominal ultrasonography. After computerized tomography (CT) imaging confirmed the diagnosis, the patient underwent a successful surgical shunt ligation without any complications through a one-year follow-up period.Various imaging studies, including Doppler ultrasonography, CT imaging, and Magnetic Resonance Imaging (MRI), are useful tools to diagnose an Abernethy malformation. Preoperative angiography with a balloon occlusion of the shunt is highly recommended in order to opt for the most appropriate intervention—including one-or-two-step surgical closure, endovascular occlusion, and liver transplantation.An Abernethy malformation should be considered in the differential diagnosis of unexplained portal hypertension or any of its associated complications in pediatric patients. Early diagnosis and proper management of this condition can lead to a favorable prognosis, as in this case. Accordingly, a pre-operative evaluation is important to determine the proper therapeutic method. The oveTo our knowledge, this is the first report of a congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, in Thailand. Our objective was to report and clearly delineate the approaches and management of this rare case. This case report has been reported in accordance with the surgical case report (SCARE) guidelines .2A 4-year-old boy presented with multiple discrete subcutaneous reddish papules and nodules over the entirety of his body. Physical examination revealed splenomegaly and multiple cutaneous hemangiomas. Neither of jaundice, hepatomegaly nor abnormal ascites was noted. The cutaneous hemangiomas resolved nine months after treatment with oral propranolol. Initial blood tests revealed only an elevated serum ammonia level of 85 μg/dL . Complete blood count (CBC) and liver function tests proved normal. In order to investigate the cause of his splenomegaly, Doppler ultrasonography initially detected an anomalous vessel in the liver. An Abdominal CT scan subsequently confirmed a portosystemic shunt connecting the left portal vein to the inferior vena cava (IVC). The Enlarged main portal, splenic and superior mesenteric veins were examined . Also noA cavoportogram via a trans-jugular access was performed, revealing a large portal-IVC shunt measuring 15 mm in diameter and 8 mm in length. However, the embolization with a vascular plug could not be done in this case due to an inadequate length of the affected vessels to place the device. Therefore, we decided to proceed with a surgical shunt ligation two weeks later. The intraoperative findings demonstrated a left portal vein-IVC shunt, measuring 15 mm in diameter—the same size as was evaluated with the cavoportogram. The hypoplastic right portal vein was 3 mm in diameter. The shunt was encircled with a vascular sling and temporarily clamped. The portal vein pressures were measured before and after procedures: 15 and 23 mmHg, respectively. The shunt was then completely ligated with a non-absorbable 1-0 silk suture.The patient had attended follow-up visits over a 12-month postoperative period. Doppler ultrasonography and CT imaging of the liver at 1 month, 2 months and 6 months showed a favorable resolution . Additio3CPSS is divided into two main categories as follows: (1) intrahepatic type including portohepatic shunt and patent ductus venosus (PDV), and (2) extrahepatic type or “Abernethy malformation”. The latter is named such because it was first described by John Abernethy in 1793. He reported a postmortem examination in a 10-month-old female infant, who showed multiple congenital anomalies including dextrocardia, transposition of great vessels, polysplenia, and termination of the portal vein in IVC at the level of the insertion of the renal vein with the enlarged hepatic artery . Since tBecause of the non-specific clinical presentation, the imaging studies play an important role for diagnosis. Doppler ultrasonography is usually the initial investigation, and it is also useful in the follow-up period after treatment. CT or MR imaging is helpful in confirming the diagnosis, providing a vascular map, and searching for complications and its associated anomalies such as liver nodules. Angiography and portal pressure measurement while temporary occluding the shunt (shunt occlusion test) is a crucial procedure to evaluate the degree of hypoplasia presenting in the intrahepatic portal veins and to measure the portal pressure during shunt occlusion . This diClosure of the extrahepatic CPSS should be considered early, especially in patients who present with associated complications. Because of its rarity, there is no standard guideline for the management of CPSS. We hereby propose an algorithm for the management of extrahepatic portosystemic shunts . The theIn general, an endovascular closure is considered as the first-line treatment because of its less invasiveness compared with surgery ,9. A perThe principle steps of the surgical closure include (1) insertion of a catheter into the portal system; (2) isolation and temporary occlusion of the shunt, the change in venous pressure before and after shunt occlusion is recorded; (3) evaluation of the intrahepatic portal venous blood flow via Doppler ultrasonography or intraoperative portal venography before and after occlusion; and (4) during the 15-minute shunt occlusion, the small bowel is examined whether it is edematous or cyanotic. If a rise in portal pressure does not exceed 25 mmHg, the appearance of the bowel is not worrisome and there is an accompanying increase in portal venous flow, then the shunt could be permanently ligated. If the previous criteria are not met, an initial procedure is performed to partially occlude the shunt where there is a portal pressure of less than 20 mmHg. Subsequently, when imaging studies show adequate development of intrahepatic portal vein branches, a second procedure will be performed to completely ligate the shunt. This generally occurs several months after the initial procedure ,10. The Postoperative imaging by Doppler ultrasonography, CT or MRI angiogram should be performed to confirm the degeneration of the shunt and the development of intrahepatic portal branches. Careful follow-up is necessary for several years to monitor the resolution of complications, and to detect the presence of any additional shunts which may be required for the closure to be completed successfully .4This case report has delineated the successful detection and management of a young male patient with extrahepatic CPSS. Physicians should consider CPSS in their differential diagnosis of children who present with unexplained portal hypertension and its associated symptoms. Early diagnosis and appropriate management of CPSS will lead to a favorable prognosis and prevent serious complications that may arise.No source of funding.This is a case report study; no ethical approval was needed.The informed consent was obtained from the patient’s mother for publication of this case report.Dr. Suthida conceptualized and wrote this paper.Dr. Chollasak, a main surgeon involved in care of the patient, edited the manuscript.Dr. Suporn edited the manuscript.Dr. Nichanan reviewed the radiological findings in this patient.Not applicable.The Guarantor is Dr. Chollasak Thirapattaraphan.Not commissioned, externally peer-reviewed.No conflict of interests in this article.


Cardiac disease is a well-known complication of antiphospholipid syndrome (APS), with many patients presenting with valvular thickening or vegetations, referred to as Libman–Sacks endocarditis (LSE). Because cases of APS with cardiac involvement are relatively rare, paucity of large clinical trials studying this complication has made management challenging. In the absence of acute heart failure and embolic events, a medical approach is usually selected, consisting of anticoagulation and possibly corticosteroids when another underlying autoimmune disease is present. However, the role of various anticoagulant classes and the duration of steroid therapy continue to be debated. Here, we present a 45-year-old woman who developed two vegetations in the setting of secondary APS while taking rivaroxaban before experiencing marked improvement with the use of enoxaparin and steroids. This ultimately manifests clinically with clotting in the venous and/or arterial systems. APS is often secondary to another autoimmune disease such as systemic lupus erythematosus (SLE), or may be a primary phenomenon in the absence of other immunological conditions. In order to make a diagnosis, clinical features must be accompanied by the presence of at least one of three antibodies: anticardiolipin, anti-In some cases, clotting can result in the formation of vegetations on cardiac valves, a form of nonbacterial thrombotic endocarditis (NBTE) called Libman–Sacks endocarditis (LSE). The disease is characterized pathologically by the presence of small, warty lesions of platelets and fibrin usually measuring about 1–5 mm, occurring mainly on the mitral valve, followed by the aortic valve in frequency .Treatment of LSE has been controversial, largely due to the lack of adequate randomized clinical trials. A surgical approach is typically used when criteria similar to those for infectious endocarditis (IE) are met –5. When Here, we describe a case of LSE occurring in the setting of secondary APS with SLE in which an abnormally large vegetation formed on the aortic valve concurrently with a smaller vegetation on the mitral valve.The patient is a 43-year-old female who presented with sharp, substernal, nonexertional chest pain, and shortness of breath. Her past medical history included secondary APS (lupus anticoagulant positive) in the setting of SLE, with multiple, recurrent deep venous thromboses of the lower extremities. She was diagnosed with myopericarditis during a hospitalization one year prior when she presented with similar symptoms and an elevated troponin, after which a coronary catheterization demonstrated no significant coronary artery disease. Examination revealed a 2/6 systolic ejection murmur, without any radiation, gallops, rubs, or jugular venous distension. Auscultation of the lungs revealed clear breath sounds.An echocardiogram was ordered when she was found to have an elevated troponin level of 0.209 ng/ml without any evidence of acute ischemic pathology on her electrocardiogram. Subsequently, a 2 cm mass was seen on the aortic valve with evidence of obstructive pathology. A follow-up transesophageal echocardiogram redemonstrated this mass, along with a smaller mass on the mitral valve associated with mild mitral regurgitation . The aorThe patient's chest pain improved with colchicine, but her shortness of breath remained and was presumed to be from partial obstruction of the aortic valve by the large mass. After consultation with cardiothoracic surgery, medical therapy was initiated with a goal to avoid surgery if there were signs of improvement. She was already taking rivaroxaban when she originally presented, given that she had failed warfarin therapy in the past with persistently subtherapeutic INRs and recurrent DVTs, so she was started on therapeutic enoxaparin and aspirin. After a rheumatology consultation, hydroxychloroquine and prednisone were initiated as well. The patient remained stable with no new symptoms or signs of embolic events during her follow-up visits, and repeat transesophageal echocardiograms at 12 and 24 weeks demonstrated improvement in the size of both vegetations . Her preThis case highlights many of the challenging aspects of managing patients with cardiac involvement in APS. The optimal treatment approach continues to be poorly defined, largely due to the fact that many recommendations regarding management originate from expert opinion and small observational case studies rather than randomized trials.A treatment strategy begins with assessing the necessity of a surgical approach. Symptoms of acute heart failure, acute valvular regurgitation/stenosis, or recurrent embolization when recognized are considered to be relatively straightforward indications to consider surgical evaluation, in addition to patients who have failed medical management. Surgery is often offered to patients with mobile, left-sided vegetations greater than 1 cm for infective endocarditis, but this approach is not generalizable for cases of NBTE , 5. GiveAfter a commitment is made to a medical approach, a decision about the best form of anticoagulation is imperative. Warfarin with an INR goal of 2-3 has been considered the gold standard in patients with newly diagnosed APS presenting with their first thrombosis –8. GivenAdditionally, the proper anticoagulant to use in the setting of warfarin failure continues to be perplexing. Various approaches include using high intensity anticoagulation with an INR goal of 3-4, the addition of aspirin or hydroxychloroquine to a moderate intensity anticoagulation regimen, or switching to a new anticoagulant class despite the lack of evidence to support any benefit of one class over vitamin K antagonists –14. GiveWhen another underlying autoimmune process is present, steroids are frequently administered despite controversy over their benefits. Several case reports have demonstrated the ability of steroids to acutely decrease the amount of inflammation and disease activity, leading to rapid improvement in symptoms and imaging of the valves –17. DespThis case demonstrates the challenges in managing a complicated presentation of Libman–Sacks endocarditis in the setting of warfarin and rivaroxaban failure. Anticoagulation with enoxaparin and aspirin in combination with prednisone ultimately lead to dramatic improvement, although questions still remain about whether or not this approach could be extrapolated to similar cases with reliable outcomes.


A 58-year-old female presented to the hospital with respiratory distress several days after a right hallux amputation. A new lytic lesion within the fourth thoracic (T4) vertebral body and mediastinal lymphadenopathy was noted on chest computed tomography scan. A bone biopsy was performed, revealing bone and collagenous fragments only. Two months later, new imaging revealed approximately 60% lytic destruction of the T4 vertebral body with new right pedicle involvement. Surgical treatment was offered. Intraoperative frozen pathology indicated a hemangioma. An intralesional debulking and stabilization was performed. The right T4 nerve was sacrificed to gain access to the entire vertebral body. Curettage was then used to push the tumor away from the spinal canal into the vertebral body. The spine was reconstructed with 5-10mm beads of Simplex P bone cement  which contained 40 grams of poly-methyl methacrylate and 1 gram of tobramycin. Five months after resection, the patient presented with computed tomography and magnetic resonance imaging findings of recurrent disease at T4 and spread to the adjacent T5 vertebral body with lytic changes. At 18 months following her second debulking surgery and radiation treatment, the patient was doing well with no pain or numbness. Long-term imaging compared to the patient’s preoperative imaging displayed improvement in spinal debulking with minimal residual enhancement of tumor despite significant artifact. Epithelioid hemangioendothelioma was first named by Weiss and Enzinger in 1982 [This is a rare tumor with a prevalence of less than one in one million . OptimalA 58-year-old female presented to the hospital with respiratory distress several days after a right hallux amputation. A new lytic lesion was noted within the fourth thoracic (T4) vertebral body and mediastinal lymphadenopathy on chest CT, and previous baseline imaging depicted no evidence of disease Figures , 2. A boThe T4 lesion appeared hypointense on T1-weighted magnetic resonance imaging (MRI), hyperintense on T2-weighted MRI, and displayed avid homogenous contrast enhancement in T1-weighted MRI with contrast Figures -3C. TherThe patient was placed prone on a Jackson table. Uniplanar fluoroscopy was used to confirm the operative level. Standard midline open approach was used to dissect the T3-5 laminae and transverse processes. Pedicle screws (5.5 x 40 mm) were placed at T3 and T5 bilaterally. A complete laminectomy at T4 was performed using a Midas Rex® high-speed drill . The right facet at T4-5 was removed, and the pedicle at T4 was then biopsied. Purple highly vascular mass was encountered within the right pedicle. Intraoperative frozen pathology was hemangioma.Due to the frozen diagnosis of hemangioma, we performed an intralesional debulking and stabilization rather than an en-bloc resection. The right T4 nerve was sacrificed to gain access to the entire vertebral body. Curettage was then used to push the tumor away from the spinal canal into the vertebral body. Approximately 50% of the vertebral body infiltrated with tumor was removed using curettage. The spine was reconstructed with Simplex P bone cement  that contained 40 grams of poly-methyl methacrylate and 1 gram of tobramycin. We introduced 5-10 mm beads of the bone cement into the vertebral column deformity; then before hardening, it was contoured to fit the defect  for the reconstruction of large and irregular spinal defects after large tumor resections. PMMA has a Young’s modulus ten times lower than that of cortical bone and thus acts as an elastic interlayer . The addFurther studies with long term follow-up are needed to continue to characterize the optimal surgical and post-operative strategies for epithelioid hemangioendothelioma lesions.Epithelioid hemangioendothelioma is a rare and malignant vascular tumor with potential for metastasis. The optimal treatment has not yet been elucidated but may include endovascular intervention, radiation, and surgical resection. The ideal surgical technique and approach are mostly surgeon-specific; however, the focus should be on complete resection and potential cure. In our case, the patient was not a candidate for extensive en-block resection due to comorbidities. However, we were able to show good long-term tumor control after debulking, decompression, and spinal reconstruction with AI-PMMA followed by radiation.


Surgeons sometimes must plan pancreatoduodenectomy (PD) for patients with a variant common hepatic artery (CHA) branching from the superior mesenteric artery (SMA) penetrating the pancreatic parenchyma, known as a transpancreatic CHA (tp-CHA).A 67-year-old man was admitted to our hospital because of liver dysfunction. A duodenal tumor was identified by gastrointestinal endoscopy, and a biopsy revealed a neuroendocrine tumor. Computed tomography showed multiple metastases in the left three sections of the liver. As an anatomical variant, the CHA branched from the SMA and passed through the parenchyma of the pancreatic head, and all hepatic arteries branched from the CHA. Furthermore, the arcade between the left and right gastric artery (RGA) was detected, and the RGA branched from the root of the left hepatic artery. PD and left trisectionectomy of the liver were performed. The tp-CHA was resected with the pancreatic head, and the gastric arterial arcade was preserved to maintain the right posterior hepatic arterial flow. Postoperatively, there were no signs of hepatic ischemia.When planning PD, including hepatopancreatoduodenectomy, for patients with a tp-CHA, surgeons should simulate various situations for maintaining the hepatic arterial flow. The preservation of the gastric arterial arcade is an option for maintaining the hepatic arterial flow to avoid arterial reconstruction.The online version of this article (10.1186/s40792-018-0474-8) contains supplementary material, which is available to authorized users. The common trunk formed by the common hepatic artery (CHA) and the superior mesenteric artery (SMA) is referred to as the hepatomesenteric trunk; this is only found in 1.5–2.3% of the population , reconstWe herein report a case of duodenal neuroendocrine tumor (NET) with multiple liver metastases for a patient with a tp-CHA. The patient was successfully treated with PD and left trisectionectomy with caudate lobectomy combined resection of the tp-CHA and preservation of the gastric arterial arcade in order to maintain the hepatic arterial flow.A 67-year-old man was admitted to our hospital because of liver dysfunction during a screening examination. Enhanced abdominal computed tomography (CT) revealed a hypervascular mass of 35 mm in diameter in the descending portion of the duodenum Fig. , and theAfter laparotomy, the gastric arterial arcade was exposed and encircled, and the LHA, RHA, and proper hepatic artery (PHA) were encircled Fig. . The LHAPostoperatively, the patient developed pancreatic fistula (Clavien-Dindo IIIa) and biliary leak (Clavien-Dindo IIIa), and these complications were treated conservatively. There were no signs of hepatic ischemia. The patient was discharged on postoperative day 39. The pathological diagnosis was duodenal neuroendocrine tumor G2 with multiple liver metastases. The Ki-67 labeling index was < 20%, and staining for chromogranin A and synaptophysin were positive. There was no evidence of invasion of the pancreatic parenchyma; however, the duodenal tumor was confined to the MP layer, and one of the 25 examined lymph nodes was positive, and moderate lymphovascular invasion was observed. The final diagnosis was pMP, med, INFa, ly1, v2, pPM0, pDM0, and pEM0. The patient has shown no recurrence in the 22 months since the operation. Enhanced abdominal CT at 4 months after surgery revealed the blood flow of the RPHA via the gastric arcade


The age range varies from 4 to 57 with a mean age of 28.06 years. Ectopic eruptions of a tooth in a non-dentate region is a rare entity and such cases have been reported to occur in the nasal cavity, orbital floor, maxillary sinus, mandibular condyle, chin, palate and coronoid process.We report a case of bilateral ectopic eruption of maxillary third molars associated dentigerous cyst. A 27 years old female patient reported to us with chief complaint of purulent discharge form nose and recurrent facial swelling for last 2 years. Complete removal of cystic lining and extraction of ectopic maxillary third molars along with mandibular third molars was carried out.Post-operative healing was uneventful.Bilateral ectopic eruptions of maxillary third molars in Antrum of Highmore (Maxillary sinus) are rarely seen and scantly documented in the literature. Dental ectopia may occur in antrum and present with local sino-nasal symptoms attributed to chronic sinusitis. Due to its rarity and lack of consensus over its management, the incidence deserves to be added to the literature & discussed. In most2A 27 Years old female patient reported to us with chief complaint of purulent discharge form nose and recurrent facial swelling for last 2 years. She experienced pain over right cheek region which was mild, intermittent and dull- aching in nature, she also noticed salty taste occasionally. She had a history of three-four courses of antibiotics prescribed by physician, but she had no permanent relief. Therefore, she was referred to our centre for management.On clinical examination, mild facial swelling over right middle third of the face extending supero-inferiorly from infraorbital rim to the imaginary ala-tragus line and antero-posteriorly from right ala to body of the zygoma. Mild tenderness was present on palpation. Intraorally, other than missing maxillary third molars, no other significant findings were noted. Orthopantomograph (OPG) revealed ectopic maxillary third molars in the maxillary sinus along with impacted mandibular third molars . In ordeOn the basis of clinical and radiological examination, surgical removal of all impacted third molars along with cyst enucleation was planned through intraoral approach under general anaesthesia.Standard preparation was done. Under nasotracheal intubation, a vestibular incision was given from first premolar to second molar, a bony window was created in anterolateral wall of maxillary sinus. Cystic lining identified, pus was evacuated, swab was taken and sent for culture and sensitivity. Complete removal of cystic lining and extraction of ectopic maxillary third molars  along wi3According to Archer, Impacted tooth is, “The tooth, which fails to erupt in oral cavity in its functional position and which has lost its further potential of eruption. Maxillary third molars are the second most common tooth to be impacted, mandibular molars are the most common one. The third molar is the last tooth to erupt in the maxilla hence more likely to be affected by displacement while competing for space and this could be responsible for the high incidence of ectopic third molar in the maxilla. Odontogenesis begins in the sixth week in utero at the time of maxillary and mandibular dental lamina formation . A serieHamama et al. in the year 2018 , reporteRadiological examination is essential for diagnosing the presence of an ectopic tooth. Advanced imaging modalities are very useful for the determination of exact location of tooth and its surrounding anatomical structures. Cone beam computerized tomography (CBCT) and CT scans are certainly superior to panoramic radiographs. Both gives better precision in localization of pathology. CBCT is also useful to delineate the three dimensional morphology of the ectopic tooth, its inclination and proximity to the sinus which aids in surgical planning.The treatment of choice of ectopic teeth associated with cystic lesion in maxillary sinus is surgical removal of the tooth along with enucleation of the cyst. Various techniques have been discussed in the literature including intraoral approach, extraoral approach, and endoscopic procedures. Di Pasquale and Shermetaro used a nasal endoscope to create a middle meatal antrostomy and deliver the ectopic tooth and its cystic contents . Hamama Written informed consent was obtained from the patient for publication of this case report and accompanying images.None.Yes, Ethical approval has been taken.Yes, written informed consent has been taken for procedure, publication as well as for accompanying images.Dr. Sarwpriya Sharma & Dr. Jaideep Chauhan, both the authors contributed equally to the paper which includes Surgery, design of case report, writing, interpretation etc.Dr. Vilas Newaskar, Consultant and Head, Department of Oral & Maxillofacial Surgery, GDC Indore (MP).Not commissioned, externally peer-reviewed.No Conflict of interest.


Neuroblastoma is an embryonal malignancy of the autonomic nervous system and is the most common extracranial tumor of early childhood. However, neuroblastoma in adults is rare with an overall incidence of 1 in 10 million adults/year. Adults with neuroblastoma have a significantly worse prognosis than children with neuroblastoma.In this case report we describe a 75-year-old Han Chinese woman with bilateral lower extremities weakness, numbness, and fatigue for 1 week. She initially presented as primary hyperaldosteronism with hypertension, persistent hypokalemia, and an elevated aldosterone/plasma renin activity ratio. An abdominal computed tomography scan with intravenously administered contrast showed a solid mass arising from her left adrenal gland. She underwent retroperitoneal laparoscopic surgery that allowed the resection of the mass. Microscopic and immunohistochemical staining, which were positive for synaptophysin, CD56, and vimentin, confirmed the diagnosis of adrenal neuroblastoma. Surgical resection of the tumor was done and no chemotherapy or radiation therapy was done postoperatively. She died from lung and brain metastases 22 months after surgical resection.Adrenal neuroblastoma in elderly adults is a very rare disease with sparse data available in the literature. Early stage disease could be managed by surgical resection alone. However, the prognosis is significantly worse than that observed in pediatric patients. Further research focusing on tumor biology and therapy for this rare malignancy in adults may help to improve disease outcome. A neuroblastoma (NB) is an embryonal malignancy of the autonomic nervous system and is the most common extracranial cancer of early childhood and the most frequently diagnosed malignancy in infancy. Common locations where NB can occur include the adrenal medulla at least 50%), neck, chest, and pelvis , 2. The 0%, neck,2. A physical examination was normal. A laboratory workup disclosed decreased serum potassium level of 2.7 mEq/L and a normal serum sodium level of 135.9 mEq/L. Her kidney function was within normal limits with blood urea nitrogen (BUN) of 8.9 mg/dL and a creatinine level of 0.79 mg/dL. Further testing was notable for an elevated serum aldosterone level of 174 ng/dL , normal plasma renin activity (PRA) level of 7.59 ng/mL per hour, and elevated level of aldosterone/PRA of 22.9. Her 24-hour urine cortisol, vanillylmandelic acid (VMA), and metanephrine levels were unremarkable. She was initially diagnosed as having primary hyperaldosteronism and was given orally administered potassium and spironolactone. Muscle weakness and numbness were partially relieved. However, her serum potassium level was not corrected. Abdominal computed tomography (CT) scans with intravenously administered contrast showed a 4.5 cm × 3.1 cm solid mass arising from her left adrenal gland  was 28.8 kg/mNB is an embryonal tumor originating from the autonomic nervous system. The cell of origin is thought to be a developing and incompletely committed precursor cell derived from neural crest tissue. NB is the third most common childhood cancer . HoweverNB is rarely reported in the adult population, with < 10% of the cases diagnosed after age 10. The incidence rate for patients aged over 30 is 0.2 cases per million inhabitants per year and its incidence becomes increasingly scarce in the elderly population . The cliMYCN oncogene locus and at chromosome 11q [A new International Neuroblastoma Risk Group (INRG) tumor staging system has been developed for the stratification of statistical and clinical risks of different subgroups of patients with NB. In the INRG Staging System, the extent of locoregional disease is determined by the absence or presence of image-defined risk factors . Stage M is used for widely disseminated disease . Four brsome 11q .et al. conducted a series with 27 patients with NB aged 12–69 years in Italy [In our case, regarding treatment, we only performed surgical resection of the tumor but no chemotherapy or radiation therapy was done after the operation. The treatment strategy in pediatric patients with NB has been well studied; it includes surgical resection, and optimal combination chemotherapy and radiotherapy. However, there are no standard treatment guidelines or chemotherapy protocols for adult or elderly patients with NB due to the rarity of NB in this population . A cohorin Italy . The trein Italy .NB in elderly adults is a very rare disease with sparse data available in the literature regarding natural history, genetic causes, treatment, and outcomes. Early stage adrenal NB in elderly patients can be managed with surgical resection alone. However, elderly adult patients have a worse prognosis than is observed in pediatric patients. Here we reported a rare case of an elderly patient with adrenal NB treated with surgical resection and with a survival of 22 months. According to the medical literature, surgical resection combined with local radiation therapy offers better outcomes in cases of adult local NB. Chemotherapy should be considered in disseminated disease. Further research focusing on tumor biology and therapy for this rare malignancy in adults may help to improve disease outcome.


The formation of gastrointestinal-type epithelium is found in 7–13% of mature cystic teratomas, which are the most common germ cell tumors of the ovary. Few cases harboring organized gastrointestinal tract formation have been reported, and a mucinous neoplasm arising in them is further rare. Here, we report a case of an ovarian mature cystic teratoma with intestinal structures harboring intestinal-type mucinous neoplasm, mimicking low-grade appendiceal mucinous cystadenoma. A 66-year-old female, with remarkably increased serum carcinoembryonic antigen (CEA) level, underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy due to the ovarian tumor. The immunoprofile of the tumor showed CK7−/CK20+. We review the past literatures, and then consider that the existence of mucinous neoplasm should be kept in mind if we find elevated level of serum CEA and the organized gastrointestinal development in an ovary. The immunoprofile of CK7/CK20 is useful to determine the origin of mucinous tumors associated with mature cystic teratomas. Mature cystic teratomas are the most common germ cell tumors of the ovary –3. HistoTherefore, we report a very rare case of mature cystic teratoma with intestinal structures harboring intestinal-type mucinous neoplasm, with literature review.A 66-year-old female, gravida 1, para 1, presented to our gynecologic unit with complaints of right lower abdominal pain. Magnetic resonance imaging (MRI) showed a bilocular 8 cm tumor with thick wall posterior to the uterus and demonstrated bleeding or mucinous component  was found at other hospital. Gastroscopy and colonoscopy, which were performed at the previous hospital, revealed no abnormal findings. Her past medical history was unremarkable. Bimanual pelvic examination demonstrated a normal-sized uterus and cervix as well as a fist-sized cystic mass at Douglas cavum. The cervical cytology was negative. Transvaginal ultrasound identifies 8.5 × 6.0 × 5.0 cm cystic tumor with partial thick wall posterior to the uterus. MRI showed a 9 × 6 cm pelvic tumor, including partial papillary nodules with contrast enhancement and little amount of ascites  after the surgery. The patient received no further treatment and remains free of disease for 1.5 years.We described the macroscopic findings below. The surgical specimen consisted of a 9.5 × 6.5 × 4.0 cm—sized left ovarian tumor, normal uterus, and right adnexae. The external surface of the tumor was smooth, and not disrupted. The cut section revealed partial thick wall, yellow gelatinous component and a few hairs within the tumor, but a solid part did not exist Fig. .On microscopic examination, sections from the cyst wall disclosed a mature cystic teratoma and contained mature tissue of stratified squamous epithelium, mucinous gland, and adipose tissue. The wall (in the circular marks of Fig. Mature cystic teratomas are the most common benign germ cell tumors of the ovary and usually consist of well-differentiated derivatives of three germ cell layers –3. They We reviewed the MRI findings and discovered that partial papillary nodules with contrast enhancement had clarified after 6 years in spite of the tumor size was almost the same and the component was not changed. The pathological findings revealed that the papillary nodules were actually equal to folds of organized gastrointestinal tract. We guessed that the change had occurred and the mucinous cystadenoma had been developed from the gastrointestinal-type epithelium during 6 years slowly, which may correlated with the serum level of CEA. Elevated levels of CEA have also been reported in patients with neoplastic appendiceal mucoceles , which aFujiwara also reported the case of mature cystic teratomas with complete intestinal wall differentiation which contained an intestinal-type adenocarcinoma . MakiharMost primary ovarian mucinous tumors are of surface epithelial–stromal origin and exhibit diffuse expression of CK7 combined with variable expression of CK20. This immunoprofile distinguishes them from gastrointestinal tract tumors secondarily involving the ovaries because the latter most often exhibit diffuse expression of CK20 coupled with lack of or limited expression of CK7 . A literThe detailed histopathological diagnosis is needed if we find elevated level of serum CEA and the organized gastrointestinal development in an ovary, and the immunoprofile of CK7/CK20 is useful to distinguish intestinal-type secondary neoplasms associated with mature cystic teratomas from primary ovarian mucinous tumors.


Ganglioneuroblastomas, particularly those that produce catecholamine, are extremely rare in adults. Here, we report an interesting surgical case of an adult patient with a catecholamine-producing ganglioneuroblastomas in her adrenal gland, suspected to be a pheochromocytoma, and with a cerebral aneurysm.The patient was a 73-year-old woman under treatment for hypertension. During a health check-up, a cystic retroperitoneal tumor was incidentally found in the superior pole of her right kidney. Her blood adrenaline level was slightly elevated, and her urinary adrenaline, noradrenaline, and dopamine levels were above the upper reference limits. In addition, 24-h urinary excretion of metanephrine, normetanephrine, and vanillylmandelic acid were all increased. 123I-Meta-iodobenzylguanidine scintigraphy showed an abnormal accumulation of the marker in the cyst wall. She was, therefore, diagnosed with a pheochromocytoma and scheduled for tumor resection. However, preoperatively, 8-mm-diameter cerebral aneurysm was incidentally found in her basilar artery. This required careful preoperative discussion. The aneurysm was difficult to approach and treat, and based on its position, shape, and size, the risk of rupture was low. Because hypertension is a major risk factor for aneurysmal rupture, we decided to proceed with the tumor resection. A lumbar catheter was placed to monitor the cerebral aneurysm for intraoperative rupture, and her transcranial motor-evoked potential and somatosensory-evoked potentials were monitored to track her intraoperative neurological function. During surgery, we carefully monitored fluctuations in blood pressure and resected the tumor with minimal mobilization. Postoperatively, head computed tomography confirmed that there was no sign of rupture. Histopathologically, the tumor was diagnosed as a catecholamine-producing ganglioneuroblastoma. The postoperative course was good, and the patient’s blood pressure improved.Careful perioperative management is needed for a patient with both a catecholamine-producing tumor and cerebral aneurysm. Ganglioneuroblastomas (GNBs) are derived from neural crest cells and comprised a mixture of neuroblasts and ganglion cells . Some GNDuring a routine health check-up of a 73-year-old woman, abdominal ultrasonography incidentally revealed a retroperitoneal tumor with a maximum diameter of approximately 80 mm at the upper pole of her right kidney. She was admitted to our institution for examination of the tumor. The patient had been taking medication for hypertension, and her blood pressure had been maintained at 120–130 mmHg with 20-mg/day nifedipine and 2.5-mg/day carvedilol. She had no notable abnormal findings in her general biochemistry, complete blood count, or the coagulation test. Various tumor markers, including carcinoembryonic antigen, carbohydrate antigen 19-9, squamous cell carcinoma-related antigen, and carbohydrate antigen 125, levels were also within normal ranges. Her blood noradrenaline and dopamine levels were within normal ranges, but her adrenaline level was elevated at 0.12 ng/ml and the 24-h urinary excretion of catecholamines and their metabolites were all increased Table . The tumHowever, preoperative MRI incidentally revealed a cerebral aneurysm, 8 mm in diameter, at the junction of the basilar and superior cerebellar arteries Fig. . We, theWe decided to conduct more rigorous blood pressure management for the patient’s surgery. Her preoperative blood pressure was managed under 120 mmHg using 32-mg/day doxazosin and 2.5-mg/day carvedilol. We used epidural anesthesia for thorough analgesic management and general anesthesia centered on propofol and remifentanil, and we carefully monitored the blood pressure. After inducing general anesthesia, a lumbar catheter was placed to monitor for intraoperative rupture of the cerebral aneurysm. In addition, we used transcranial motor-evoked potential and somatosensory-evoked potential monitoring to track her intraoperative neurological function. Subsequently, the surgical procedure was performed via a right subcostal incision with upper midline extension. During the surgery, we focused on fluctuations in blood pressure. Prior to full-scale tumor mobilization to prevent excessive secretion of catecholamine, we first ligated and separated the feeder arteries, consisting of three right adrenal arteries, followed by the drainage vein, consisting of a right adrenal vein. Next, we removed the tumor with minimal mobilization. The tumor had not noticeably invaded the surroundings. There was no blood outflow from the lumber catheter during the procedure. We withdrew the catheter immediately post and used head CT to confirm there had been no intracranial hemorrhage. No intraoperative blood pressure fluctuation was observed, and the cerebral aneurysm monitoring devices showed no abnormality. Complete resection (R0) was achieved by pathologically determining the negative surgical margin during surgery.Macroscopically, the cystic tumor was approximately 100 mm in diameter with an interior that was almost necrotic Fig. . MicroscThe postoperative course was good, and the patient’s blood pressure after surgery was maintained under 110 mmHg with no antihypertensive agents. She was discharged on the 7th day postoperatively. An examination after discharge confirmed that her blood and urinary catecholamine levels and metabolite excretion had returned to normal (Table GNBs frequently occur in children, but their onset in adults is extremely rare . Adult GIt was also noteworthy that our case had a cerebral aneurysm. A catecholamine-producing tumor with a cerebral aneurysm requires much more cautious management, but there is no definite consensus over management because of limited reports. A search of PubMed found no previous reports of GNB with a cerebral aneurysm, although there have been five reported cases of pheochromocytoma with cerebral aneurysms –21. In fFor catecholamine-producing tumors with a cerebral aneurysm, whether the tumor is pheochromocytoma or GNB, careful consideration should be given to the order in which the treatments should take place, taking into consideration the risk of rupture and the possibility of malignancy. Our strategy for this catecholamine-producing tumor with cerebral aneurysm may be helpful in the treatment of similar cases in the future.We reported here the case of an adult patient who developed an extremely rare catecholamine-producing GNB with a cerebral aneurysm. Such a case required careful consideration of the order in which the tumor and cerebral aneurysm should be treated. During tumor resection, careful perioperative management was important. Further similar cases should be accumulated to establish a consensus on the treatment strategy.


STK11 mutations, presumed to be somatic. In conclusion, this is an unusual case of a sporadic Peutz-Jeghers type polyp occurring in the appendix.Hamartomatous polyps of Peutz-Jeghers type are strongly associated with Peutz-Jeghers polyposis syndrome and are predominantly encountered in the small intestine. Sporadic cases are uncommonly reported. We report a case of a polyp identified incidentally in the appendix of a patient undergoing diagnostic imaging due to a history of hepatitis C infection. Histopathologic evaluation after appendectomy showed a polyp with bands of muscularis mucosae bundles with arborizing architecture and variable amounts of inspissated mucin, morphologically indistinguishable from Peutz-Jeghers type hamartomatous polyp. A family or personal history of abdominal cancers was not reported by the patient, suggesting a sporadic occurrence. Next generation sequencing revealed only two pathogenic low-level Appendiceal dilatation of more than 6 mm detected on abdominal imaging can potentially trigger appendectomy due to concern for presence of infection or malignancy . The preThe most common tumors involving the appendix are mucinous tumors, such as low grade appendiceal mucinous neoplasm (LAMN) and mucinous carcinomas, adenomas, serrated polyps, goblet cell tumors, neuroendocrine tumors, and colonic type carcinomas . HamartoSporadic Peutz-Jeghers type polyps are considered very uncommon and are mainly documented in case reports and small case series , 5. AddiA 60-year-old male with a prior history of alcoholism, tachycardia, hypertension, schizophrenia, and hepatitis C infection underwent regular surveillance protocol for hepatocellular carcinoma. Imaging revealed an incidental dilatation of appendix, which slowly increased in size over a 10 year period. More recent images revealed a dilatation of the midportion of the appendix measuring 16 mm in widest dimension, concerning for a mucinous appendiceal tumor  and an aThe patient was referred for a surgical consult due to increase in size of this appendiceal lesion and progressive luminal dilatation. Ultimately, an appendectomy was pursued due to concern for the presence of a mucinous tumor of the appendix. During the procedure, the appendix appeared dilated without signs of perforation or carcinomatosis. The appendix was resected without complication and was submitted intact for histopathologic evaluation.Gross examination revealed a 1.4 cm well-circumscribed pale brown intraluminal mass in the mid portion of a 7.5 x 2.5 x 2.2 cm appendix, 4 cm from the resection margin. Microscopically, the mass appeared to be a pedunculated polyp supported by broad bands of arborizing fibromuscular bands at low power, lined by colonic type epithelium without dysplasia . Altoget STK11  and PTEN . Genomic DNA was extracted from FFPE tissue followed by hybridization capture to enrich for the regions of interest and next generation sequencing. Two low-level mutations (presumed somatic) were detected in STK11 gene: a frame-shift mutation in exon 1  at variant allele frequency (VAF) of 1.8% and a nonsense mutation in exons 5  at VAF of 2.7%. Even though both of these mutations are slightly below the established limit of detection (LOD) of 5% VAF of this assay, the high quality scores and bidirectional and staggered reads  assessing mutational hotspot regions in 44 genes includinged reads  support Peutz-Jeghers polyps are diagnosed histologically based on the presence of broad bands of arborizing muscularis mucosae smooth muscle bundles, described as resembling a “Christmas tree” at low magnification, with variable amounts of inspissated pink mucin. Typically, the polyp is lined by benign epithelium without adenomatous change. The differential diagnosis includes adenomas, juvenile polyps, serrated polyps, inflammatory polyps, and mucosal prolapse. In the current case there was no adenomatous change to suggest a villous adenoma. Juvenile polyps and inflammatory polyps typically have more pronounced inflammatory components and dilatation of crypts. Features of mucosal prolapse include fibromuscular hyperplasia of the lamina propria extending towards the luminal aspect of the mucosa as well as distorted diamond shaped glands in the deeper aspect and congestion of dilated capillaries, and these features were not seen in the current case. The observed morphologic findings were all in keeping with a Peutz-Jeghers type polyp.This case is uncommon because the Peutz-Jeghers type polyp presented in the appendix of a patient without clinical or familial history suggestive of a polyposis syndrome; therefore both the site and patient demographics were unusual. There are only rare documented reports of appendiceal Peutz-Jeghers polyps , 6–11. A STK11 mutations. These mutations are presumed to be somatic based on low variant allele frequencies; however, their germline origin cannot be completely excluded as the assay performed is not designed to distinguish between somatic and germline variants. The exact role of these mutations in the pathogenesis of the polyp is uncertain. Additionally, the performed panel is designed to detect only single nucleotide variants (SNVs) and insertion and/or deletions (Indels) and only in the exons captured. Gross  deletions (known to cause a subset of Peutz-Jeghers syndrome cases) would not be detected by this panel and their presence in this patient cannot be completely excluded. Although these results do not completely rule out the syndromic origin of the polyp, with the combination of the negative family and patient's history they make it much more unlikely.Classic Peutz-Jeghers polyps occur predominantly in the small intestine, followed by the stomach and colon. There is a strong association with Peutz-Jeghers polyposis syndrome, which is a rare autosomal dominant condition, characterized by hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation, and a predisposition to a variety of neoplasms including carcinomas of the pancreas, colon, stomach, small bowel, breast, ovaries, and testes . A thoroIn conclusion, we report a very unusual case of likely sporadic Peutz-Jeghers type polyp, which presented with a dilated appendix and was radiographically concerning mucinous tumor of appendix.


Arteriovenous malformations (AVMs) of the spine include a broad spectrum of lesions that vary from a simple arteriovenous fistulous connection to a more complex net of abnormal vessels involving multiple spinal levels. These entities are poorly studied and understood because of their rarity and are often either managed conservatively with observation if the lesion is complex, or treated surgically or interventionally in the presence of an accessible and distinct fistulous connection. Most surgeons avoid intervening on more intricate lesions until they become symptomatic with progressive neurological decline.We describe the case of a 38-year-old man who presented with severe sharp back pain after an appendectomy procedure. A magnetic resonance angiogram (MRA) revealed an arteriovenous malformation of the conus medullaris, with a compact glomus-type nidus and arterial feeders originating from an enlarged artery of Adamkiewicz. The malformation was resected through a posterior midline approach, and the patient was neurologically intact at his discharge on postoperative Day 2. Follow-up angiography showed complete obliteration of the lesion. Our operative video is meant to serve as a step-by-step and systematic guide to the approach and management of conus arteriovenous spinal lesions, which can be difficult to treat.We provide a pre- and postoperative radiological description of the anomaly as well as a technical guide to the resection of a spinal vascular lesion. This video could serve as an operative guide and reference to neurosurgeons—both established and in training—when confronting similar disease processes in the future. Arteriovenous malformations (AVMs) of the conus medullaris are rare entities whose natural history is defined by continued progression once they become symptomatic . They usA 38-year-old man experienced sharp lower back pain after an appendectomy procedure. The pain was followed by tingling in the bilateral lower extremities and resolved quickly. A magnetic resonance angiogram (MRA) of the lumbar spine was obtained Figure  and reveArteriovenous malformations of the conus can be challenging to treat especially for asymptomatic or mildly symptomatic lesions . If the Arteriovenous malformations of the spinal cord are complex entities that can cause debilitating hemorrhages, especially in the young adult population. Surgical management of these lesions can be definitive but could carry significant surgical morbidity, and a systematic technical approach is required with multidisciplinary teamwork in both the intraoperative and the perioperative period. Preoperative embolization is usually not a good option because of the associated high risk of spinal infarction. Neuromonitoring for somatosensory evoked potential (SSEP), electromyogram (EMG) and motor stimulation, including anal sphincter function is required to avoid excessive manipulation of the conus. Good intraoperative vascular imaging should be available. Indocyanine green angiogram is non-invasive and can be used repeatedly. Sectioning of the filum terminale can improve mobilization of the lesion. Following a pial resection plane into the cord or conus should be avoided. Appropriate patient counseling about the possible risks and benefits of surgery is important given the potential morbidity of the procedure and of the natural history if left untreated. Follow-up vascular imaging postoperatively is necessary to confirm the obliteration of the lesion or to monitor any residual.


Osteoid osteoma is a painful benign skeletal tumour of unknown aetiology. Most often it occurs in the long bones of extremities and responds well to nonsteroidal anti-inflammatory medications. However, unusual localization and atypical presentation of this tumour might present a diagnostic challenge, especially if symptoms mimic that indicative of juvenile spondyloarthritis.A misdiagnosed ten-and-a-half-year-old girl with osteoid osteoma involving the distal phalanx of a little finger is presented. Her initial symptoms were pain and swelling of the little finger resembling dactylitis, while various imaging modalities showed signs of tenosynovitis, indicating a possible development of juvenile spondyloarthritis. Several trials of different non-steroid anti-inflammatory drugs gave no satisfactory results and ultrasound guided triamcinolone-hexacetonide injection provided only a short relief. Finally, almost three years after initial presentation, persistent clinical symptoms warranted repeated imaging that raised suspicion of an osteoid osteoma. Directed treatment with surgical intervention led to almost immediate and complete resolution of her symptoms.Osteoid osteoma should be suspected in case of a tender swelling of a digit in children and adolescents, regardless of initial imaging findings and clinical presentation. Early diagnosis and treatment of this benign condition can have a substantial impact on quality of life of patients and their families and protect them from many unnecessary diagnostic procedures and treatment.The online version of this article (10.1186/s12891-018-2383-1) contains supplementary material, which is available to authorized users. Osteoid osteoma (OO) is the third most common benign bone tumour typically found in children, adolescents, and young adults between 10 and 35 years of age . It accoThe aim of our report is to address the diagnostic pitfalls observed in atypical OO and emphasize OO as a possible cause of long-lasting symptoms resembling those seen in juvenile spondyloarthritis.A ten-and-a-half-year-old girl presented to her primary care physician with a three-month history of swelling and pain in the distal part of the small finger of her left hand. The pain was most intense during movement and palpation, although occasionally it was present at rest as well. There was no history of preceding trauma, acute infection, or fever. Initial physical examination showed a thickened distal phalanx of the affected finger without motion restrictions. Initial radiograph showed normal bone structure and mineralization, without signs of fracture or other pathology Fig. a and b,The presented case of OO at an unusual localization with an atypical presentation took almost three years to be correctly diagnosed and treated  demonstrated characteristic signs indicative of juvenile spondyloarthritis, although no other joint and/or enthesis was involved, and despite no family history of HLA-B27-associated diseases. Therefore, after several years of follow up with persistent symptoms, and without adequate response to NSAIDs or intraarticular glucocorticoids, re-evaluation by a second paediatric orthopaedics was suspicious for another pathology. Finally, three years after the initial symptoms and various, mostly inadequate treatment trials, repeated imaging led to the diagnosis of OO of the distal phalanx as a possible cause of the disturbances.Although OO and rheumatic diseases have almost completely different underlying mechanisms, they do both share excessive production of prostaglandins at the affected site and therefore benefit from NSAIDs treatment. Nevertheless, several reports demonstrated that conservative management with NSAIDs may take years before the OO is resolved –32. TherThe other dilemma elicited in our case is the sensitivity and specificity of various imaging modalities in the assessment of OO. While in the vast majority of cases OO can be easily identified with plain radiographs, computed tomography (CT) scans, MRI and/or skeletal scintigraphy, there are some areas of the skeleton, including spine, femoral neck and small bones of hands and feet, that are difficult to assess , 34. AltIn conclusion, we would like to emphasize OO as a possible cause of a tender swelling of a digit, a symptom more commonly seen in juvenile spondyloarthritis. Although OO is a benign disease, it might represent a diagnostic and treatment challenge for paediatric rheumatologists, indicating the need for consultation with other specialists, including the paediatric orthopaedics.Additional file 1:Timeline for case report entitled “Unusual localization and presentation of osteoid osteoma mimicking juvenile spondyloarthritis: a case report.” (PDF 53 kb)


Hemangioblastomas are rare, slow-growing, highly vascularized tumors of the central nervous system which often occur in the spinal cord. When presenting as sporadic, isolated tumors without Von-Hippel Lindau disease, they are curable through surgery with a low rate of recurrence. Tumor recurrence in these cases is usually associated with prior subtotal resection. However, to the best of our knowledge, cases of recurrent, sporadic spinal hemangioblastoma have not been reported to spontaneously arrest without intervention or symptoms. We report a patient who underwent an initial complete resection of a cervical spinal hemangioblastoma, a subtotal resection of tumor recurrence four and a half years later, and nine years of neurologic and radiographic stability with no additional interventions. Hemangioblastomas are rare, slow-growing, highly vascularized tumors of the central nervous system most commonly found in the posterior cranial fossa and spinal cord . The onlRecurrence of sporadic spinal hemangioblastomas is primarily associated with subtotal resection. To the best of our knowledge, there have been 13 described cases to date of sporadic spinal hemangioblastomas recurring postoperatively and requiring repeat surgery for symptomatic deficits . HoweverA 72-year-old woman presented with nine months of neck pain and left upper extremity pain and numbness, three months of right upper extremity pain, and more recent weakness in the left arm. She also had a long history of urinary stress incontinence with no changes in bowel or bladder function during the nine months of symptoms preceding her presentation. Physical examination was notable for 4/5 triceps strength, effort-limited 4+/5 left grip strength and symmetric reflexes. Magnetic resonance imaging (MRI) revealed a cervical intramedullary syrinx from C5-C7 with a nodule suggestive of a hemangioblastoma Figure .She underwent uncomplicated elective C5-C7 laminectomies and resection of the nodule. Pathological testing confirmed the lesion to be a hemangioblastoma.Imaging performed seven months postoperatively revealed abnormal hyperintensity of the left posteromedial spinal cord at C6 with a small, nodular, enhancing lesion of the most posterior portion of this abnormality. Subsequent imaging showed a progressive increase in the size of this lesion, and imaging four years postoperatively revealed increased nodular enhancement and recurrence of the syrinx Figure . Four yeFour and a half years after her index surgery, increasing enhancement size prompted additional surgery, including extension laminotomies/laminectomy from C3-T1 and resection of the pathology-proven hemangioblastoma. Imaging two months after the second surgery showed a punctate focus of enhancement at the dorsal aspect of C6, suspicious for residual or recurrent tumor. Subsequent annual imaging, with the most recent scan nine years after the second operation, has shown no significant changes in the focus of enhancement at C6 , which is involved in the regulation of cell metabolism and vascular proliferation . The rolSporadic hemangioblastomas have no known causative factors. Only 20% of these lesions occur in the spinal cord, and they have a different presentation when compared to VHL-associated lesions . SporadiFor both sporadic and VHL-associated hemangioblastomas, recurrence is associated with subtotal resection of lesions; however, the recurrence is rare in sporadic disease while one-third of patients with VHL-associated disease will recur every two years . OverallRecurrence of sporadic hemangioblastomas occurs less frequently than VHL-associated hemangioblastomas. When sporadic hemangioblastomas do recur, they are usually associated with a subtotal resection. To the best of our knowledge, the sporadic recurrent hemangioblastoma presented here is the first reported case to spontaneously arrest without intervention. The mechanism of spontaneous arrest is unknown. Our patient was diagnosed with complex regional pain syndrome one year before her second operation, and it is unknown whether the CRPS contributed to the arrest of the tumor.


Pediatric nasopharyngeal carcinoma is a rare malignancy strongly associated with Epstein-Barr virus infection. Patients typically present with non-specific symptoms of epistaxis or serous otitis from eustachian tube obstruction and therefore diagnosis is often delayed. We present a case of a previously healthy 17 year old female who initially complained of migraines which was resistant to oral medication. Symptoms progressed and she saw a dental surgeon for concern of a dental infection and was prescribed antibiotics with no relief. Her symptoms continued to progress until an otolaryngologist visualized a large mass along the floor of the left nasal cavity. Subsequent imaging showed a large mass in the posterior left nasal cavity and biopsy was consistent with nasopharyngeal carcinoma. The scan demonstrated an intense FDG avid mass in the left nasopharynx and nasal cavity extending to the left maxillary sinus (SUV max 10.8) and a metastatic left cervical lymph node (SUV max 6.9) with no distant metastasis  with no relief. Her symptoms progressed over another 3 months to unexplained weight loss, headaches, loss of hearing, frequent bloody nose, tooth pain, and hoarseness. Approximately 9 months after her initial symptoms, she eventually was seen by ENT and a rigid nasal endoscopy was performed which demonstrated a polypoid mass along the floor of the left nasal cavity. MRI with and without gadolinium contrast performed on a 3-T Siemens Skyra showed a large mass in the posterior left nasal cavity and nasopharynx invading the left maxillary sinus, nasal septum, left pterygopalatine fossa, left masticator and pharyngeal spaces and left longus colli muscle causing narrowing of the nasal airway and nasopharynx as well as a unilateral mastoid effusion , top rowNasopharyngeal carcinoma (NPC) is a mucosal tumor of the lateral pharyngeal recess and is a relatively rare disease in the US with an annual incidence of 0.5–2.0 cases per 100,000. Peak incidence is 40–60 years with males being at a higher risk. NPC is a rare malignancy in children accounting for <1% of all pediatric neoplasms. They typically present at a later stage in children due to the non-specific symptoms of epistaxis, headaches, nasal congestion, and conductive hearing loss secondary to middle ear obstruction. There is a strong association of NPC with Epstein-Barr Virus (EBV) infection where it is found in 90–100% of cases . In our According to the World Health Organization, there are 3 different histologic subtypes of NPC. Keratinizing squamous cell carcinoma (WHO type 1) represents about 25% of all NPCs and has a weak association with EBV and an overall 5 year survival of 20–40%. Non-keratinizing differentiated carcinoma (WHO type 2) represents about 12% of all NPCs and has a strong association with EBV and an overall 5 year survival of 65%. Non-keratinizing undifferentiated carcinoma (WHO type 3) as seen in our patient is the most common subtype accounting for >60% of all NPCs and is the most frequent pediatric subtype. It has a strong association with EBV and a 5 year survival of 73–95% (The gold standard for initial diagnosis of NPC is with nasopharyngeal endoscopy with biopsy and radiologic imaging to aid in the staging and extent of disease. CT and MRI are often performed with MRI being the preferred imaging modality , 8. T1 iNPC is a rare pediatric malignancy that often goes undiagnosed until a later stage. This case highlights the importance of recognizing the signs and symptoms of NPC tumors and the importance of radiographic imaging. As in our case, many of the pediatric cases of NPC tend to be diagnosed at a later stage due to the non-specific symptoms.RaM wrote and edited the manuscript and captured images. FV and HG captured pathology slides and assisted with figures. RiM captured MRI images and edited the manuscript. MO assisted in writing and editing the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.