Cholangiocarcinoma is an uncommon tumor, often diagnosed in the context of obstructive jaundice. Brain metastasis rarely occurs with the cerebellum being a rare site of spread of this type of tumor. Few cases of cholangiocarcinoma have been reported in the literature and this type of tumor is associated with a very poor outcome. We present a very rare form of clinical presentation of cholangiocarcinoma with neurologic symptoms due to cerebellar metastases. Cholangiocarcinoma is a rare tumor of the bile duct, often diagnosed in the context of obstructive jaundice –3. UsualVery few cases of brain metastasis due to primary cholangiocarcinoma –8 have bAn autonomous 66-year-old Caucasian man with past medical history of arterial hypertension and social alcohol consumption (15 g/day) was admitted with two weeks of nausea, vomiting, and jaundice. About 4 months earlier, the patient started daily occipital headache with frequent recurrence to the emergency department, misdiagnosed as uncontrolled arterial hypertension. There was no history of blood transfusions, recent trips, over-the-counter drugs, prior infectious diseases, or jaundice episodes. Physical examination was unremarkable except for mucocutaneous jaundice and mild discomfort in the upper right quadrant of the abdomen. Neurologic examination revealed no significant abnormalities, including cerebellar signs such as vertigo, ataxia, nystagmus, dysmetria, dysarthria, dysdiadochokinesia, or other signs. Laboratory analysis showed lactate dehydrogenase 268 U/L (125–220 U/L), aspartate aminotransferase 150 U/L (<31 U/L), alanine aminotransferase 316 U/L (<34 U/L), alkaline phosphatase 955 U/L (40–150 U/L), gamma-glutamyl transferase 1465 U/L (<38 U/L), total bilirubin 4.5 mg/dL (0.3–1.2 mg/dL), and direct bilirubin 3.2 mg/dL (0.1–0.5 mg/dL). Serum albumin, serum amylase, and prothrombin time were normal. Tumor marker CA 19-9 was elevated  and alpha-fetoprotein and carcinoembryonic antigen (CEA) were normal. An extensive etiological workup was performed excluding liver diseases, infections, autoimmune conditions, and risk factors for cholangiocarcinoma. Abdominal ultrasound showed dilatation of the common bile duct (12 mm) and intrahepatic ducts and gallstones. Esophagogastroduodenoscopy was normal. Computed tomography scan (CT) of the brain revealed one mound-shaped lesion with biconvex dural contact in the right cerebellum with heterogeneous contrast enhancement . Other cThe patient started systemic steroids with initial improvement of neurologic symptoms. However, subsequently neurological worsening was verified with gait ataxia and vertigo and therefore brain radiotherapy was started after a multidisciplinary therapeutic decision meeting. The patient died within six weeks of diagnosis, having performed only one session of whole-brain radiotherapy (WBRT).Cholangiocarcinoma is a rare tumor and the most common malignancy of the biliary tract. Its incidence has been continuously increasing and usually develops in the setting of progressive cholestasis and chronic inflammation , 2. HoweIn early stage of cholangiocarcinoma, Whipple surgery represents the main treatment. However, most patients present with advanced disease and therefore curative treatment is almost never feasible. Given the intrinsic resistance to radiation and chemotherapy, the prognosis remains dismal with a 5-year survival rate of 27–37% . Thus, mCholangiocarcinoma often spreads to the regional lymph nodes and adjacent organs. Distant metastasis is rare with bones, muscles, and thyroid gland being the most frequently involved sites. Brain metastasis is extremely rare with very few cases –7 and onChindaprasirt et al. described the only existent case series of brain metastasis by cholangiocarcinoma. They showed that all eight cases were intrahepatic or perihilar tumors with the median age of 60 years. Diagnosis was based on neurologic symptoms in all patients, three of whom received WBRT and one underwent surgery. The median survival after diagnosis of brain metastasis was 9.5 weeks , being wAs far as we know, brain metastasis has never been reported in extrahepatic cholangiocarcinoma. In addition, this is the second reported case of initial neurologic symptoms and brain metastasis exclusively to the posterior fossa .In conclusion, we report the case of an atypical presentation of extrahepatic cholangiocarcinoma with neurologic symptoms related to cerebellar metastasis, a rare site of spread. The prognosis was very poor, similar to other locations of cholangiocarcinoma. Therefore, clinicians should consider the possibility of brain spread in any type of cholangiocarcinoma, including extrahepatic location. Neurologic symptoms may precede hepatobiliary dysfunction.


Two cases of inflammatory myofibroblastic tumor (IMT) of the bladder are reported here. Both patients were male and presented with macroscopic hematuria; in the first case terminal hematuria was associated with irritative voiding symptoms. The second case was a smoker with hematuria unresponsive to medical treatment and anemia. Clinical presentation, pathological features, treatment, and prognosis are discussed. Due to rarity of this pathological condition, there are no guidelines concerning treatment and follow-up. We present our follow-up scheme and highlight the use of laparoscopic partial cystectomy as a successful treatment approach. Inflammatory myofibroblastic tumor of the bladder was first described in 1980 by Roth .Most of the patients are young, with a mean age of 38.9 years and a slightly female predominance of 51.7% in some studies . The mosHerein, we report two cases with different presentations of this rare clinical entity.(1) A 38-year-old nonsmoking male presented with burning micturition and terminal macroscopic hematuria for one month period, with no other symptoms or previous relevant medical history. Physical exam and routine blood profile were normal. Pelvic ultrasound revealed an asymmetrical thickening of the right anterolateral bladder wall (maximum thickness 23.5 mm). Cystoscopy showed a well-defined solid lobulated lesion with a fibrinous exudate . It was  muscularis mucosa infiltration. CT showed a 6 cm right anterolateral bladder wall thickness, with involvement of the perivesical fat; no lymph nodes or other lesions were identified ((2) A 56-year-old male, with a 50-pack year history of smoking, was admitted from the urgency department for painless gross hematuria with clots. In blood tests, anemia (Hemoglobin 8.6 g/dL) was detected, no other abnormalities. Urgent transurethral bladder resection/fulguration (TUBR) was proposed to control the hematuria and remove bladder clots, where cystoscopy showed a solid and wide base/sessile lesion of the anterior wall. According to the histomorphological features and the immunohistochemical profile , similarentified . LaparosClinically and radiologically, IMT of the urinary bladder is indistinguishable from other entities, it has a broad differential diagnosis ranging from reactive to neoplastic malignant lesions, comprising postoperative spindle cell nodule, embryonal rhabdomyosarcoma, leiomyosarcoma or sarcomatoid urothelial carcinoma , 6. WhenThe final and definitive diagnosis can only be made by histopathological examination and immunohistochemical/molecular study, with IMT expressing mainly smooth muscle actin, desmin, and anaplastic lymphoma kinase (ALK-1) . PositivDespite being a benign lesion it can recidivate locally, possibly due to muscularis mucosa invasion, described in 41% of the patients in a review by Montgomery et al. . At leasThere are few case reports concerning conservative therapy in bladder IMT; in one case a successful reduction in the size of a bladder IMT with anti-inflammatory regime (prednisone and Cox-2 inhibitor) was achieved , while oComplete local resection with negative surgical margins at pathology is the more advisable treatment and the most important prognostic factor, which can be achieved by transurethral resection  or partial cystectomy. Concerning the possibility of recurrence it is our opinion that partial cystectomy is more effective in total removal of the lesion, as presented in this paper.The first description of the laparoscopic approach in partial cystectomy for IMT was done by Pradhan et al. in 2013 ; in our Laparoscopic partial cystectomy can play an important role in the management of this rare bladder tumor, allowing complete resection with few comorbidities and rapid recovery in a pathology that mainly affects young patients.Regarding follow-up, there are no standardized schemes but it is advisable since this tumor has 25% of recurrence . We adop


Laryngeal chondrosarcoma (LCS) is a rare laryngeal tumor that most commonly originates from the cricoid cartilage. The current trend for treatment of low-grade LCS is function-sparing surgical option with negative margins.We reported here a case of a 63-year-old male patient with a 3-month history of progressive hoarseness and throat pain. The patient had undergone surgical resection of a laryngeal mass 2 years prior. A supracricoid partial laryngectomy was performed this time. Histological examination supported the diagnosis of low-grade chondrosarcoma. Three years later, the radiological and clinical findings showed no evidence of recurrence.Currently, total laryngectomy is preferred for patients with recurrent low-grade LCS. However, the literature review and our case suggest that a second function-preserving procedure may be a reasonable choice for recurrent LCS. However, for patients with recurrent LCS, salvage laryngectomy has been recommend. Few studies have reported the prognosis and outcome of function-preserving surgical approach for patients with recurrent LCS.The clinical manifestations of laryngeal chondrosarcoma (LCS) are nonspecific. Occasionally, patients with low-grade chondrosarcoma may be misdiagnosed with chondroma because they are closely related.In this report, we present the case of a 63-year-old man with recurrent low-grade LCS arising from the thyroid cartilage. This case was misdiagnosed as chondroma 2 years earlier at a local hospital. This report describes successful salvage of the neoplastic recurrence by supracricoid partial laryngectomy rather than by total laryngectomy. Three years later, the radiological and clinical findings show no evidence of recurrence.This study was approved by the institutional review board of the First Hospital of Jilin University.2A 63-year-old man with a 3-month history of progressive hoarseness and throat pain presented to the outpatient department in May 2013. The patient had a history of surgical resection for laryngeal mass 2 years prior, which was diagnosed as laryngeal chondroma. Physical examination revealed a mass on the left side of his neck measuring approximately 3 cm in the anterior–posterior dimension. Additional lymphadenopathy was not observed in his neck. Electronic laryngoscopy revealed a spherical neoplasm with a rough surface, measuring approximately 3 cm × 3 cm. The vocal cord and the plica ventricularis were involved, while the hypolarynx was not. Computed tomography (CT) scans of the laryngeal mass showed a large, destructive tumor centered at the level of ventriculus laryngis with destruction of the thyroid cartilage and partial left arytenoid cartilage . Other case reports also support the use of second function-preserving procedures for patients with recurrent LCS.,24 Our case developed recurrence 2 years after the initial conservative surgery. Although total laryngectomy was recommended by the surgeons, a second conservative surgery was performed because the patient insisted that quality of life was more important. Three years after the second surgery, there was no evidence of recurrence.Literature reviews suggest an LCS recurrence rate of 16% to 18%.4LCS is a rare laryngeal tumor that most commonly originates from the cricoid cartilage. Function-sparing surgery with negative margins is the current treatment of choice for low-grade LCS. It allows the radical removal of the tumor through a larynx-preserving procedure, thus representing a valid alternative to total laryngectomy. Currently, for patients with recurrence of low-grade LCS, total laryngectomy is preferred. However, a second function-preserving procedure may be also a reasonable choice, especially for older patients or those with serious comorbidities. However, total laryngectomy remains the preferred treatment for high-grade LCS.


Ameloblastoma is locally aggressive benign odontogenic tumour with increased risk of recurrence rate. The choice of treatment depends on the histologic subtype. Radical therapy is the recommended modality for solid ameloblastomas. The possibilities of recurrence even after enbloc resection are still high. The author presents two case reports of recurrent ameloblastomas postradical resection. First case describes the recurrence of ameloblastoma in the bone graft which was used for reconstruction, and the second case depicts recurrence in the infratemporal fossa. Intraoperative radiography of the frozen section of the soft tissue margin plays an important role in the holistic management of these lesions. Ameloblastoma is the common locally aggressive benign epithelial odontogenic tumour of the oral cavity. It was first recognized by Cusack in 1827 and named in 1930 by Ivy and Churchill [This paper describes two cases of recurrence of ameloblastoma in patients who underwent segmental resection of the jaw. These case reports can be added to the list of reported cases of recurrent ameloblastomas.A 46-year-old male patient referred by a private practitioner complained of swelling in the previously operated area of the right lower jaw since one month. He had a history of surgery in the same region. While going through the records of the patient, he had undergone segmental resection and reconstruction of the defect with rib graft 15 years ago. Histopathology reports of the previous pathology were not available in the records. Panoramic radiograph and CT scan revealed multilocular radiolucent lesion in the previously operated site . The cliA 45-year-old male patient visited the Department of Oral and Maxillofacial Surgery with swelling over the right temple area of the face. He reported an asymptomatic swelling since 1 month which progressively increased in size. It was soft dumbbell shaped swelling in the right temporal region with no signs of infection.Back in 2012, he had been referred to the same unit by general practitioner for gross swelling in the right jaw. Panoramic radiograph and CT scan showed multilocular radiolucency. The patient was subjected for biopsy and reported as ameloblastoma of the right mandible. He underwent right hemimandibulectomy. On table, the resected specimen was subjected for intraoperative radiography to understand the clearance of 1.5 cm safe radiologic margin. The histological report also revealed ameloblastoma with atypical features showing hypercellularity. Hence, he was kept on regular follow-up.In 2016, he reported with a swelling on the right temple region. MR imaging studies showed heterogeneously enhancing altered signal intensity lesion (measures 4.4 cm × 3.7 cm × 4.6 cm) with tiny cystic areas noted in the right infratemporal fossa . The patAmeloblastoma is a locally aggressive, anatomically benign tumour of the oral cavity which rarely undergoes malignant transformation. It is the second most common odontogenic tumour, the first being odontoma. Ameloblastoma is a common tumour in developed countries (70%) compared to developing countries, probability of unreported case in the latter .WHO in 2005 classified ameloblastoma into four subtypes: multicystic/solid, unicystic, desmoplastic, and extraosseous type. Solid/multicystic variant is the most common type, and it is highly aggressive and has a 90% recurrence after conservative management such as curettage and enucleation  and in fPathogenesis of the ameloblastoma is unclear. The genetic theory explains the involvement of the BRAF protein in the mitogen-activated protein kinase pathway (MAPK) that has been commonly found to be mutated, rendering the pathway constitutively active . Ahlem eMore commonly, it affects the mandibular posterior region. The infiltration of the tumour cells occurs more predominantly in the cancellous portion of the cortical bone. Hence, CT scan is most promising in identifying the cortical destruction and soft tissue involvement .The management involves either conservative or radical approach. The conservative method involves enucleation with adjunctive procedure either chemical cauterization or peripheral ostectomy of 1–1.5 cm normal margin. Conservative approach can be utilized for unicystic type. According to Pogrel and Montes, a unicystic variant can be best managed by enucleation with an application of Carnoy's solution or cryotherapy .Radical approach is indicated for large ameloblastoma involving the inferior alveolar canal or below or for more aggressive variants like intramural ameloblastoma or multicystic type , 11. It The resection with 2-3 cm clear bone margin is indicated in cases of ameloblastic carcinoma .Possible factors for recurrence after radical resection may be the histologic type and location of the tumour and solid type particularly follicular variety is the most aggressive type. The mandible posterior region is the common site of occurrence, and as it invades the cancellous portion beyond radiologic margin, over a time it can cause the cortical perforation. Invasion of the periosteum can lead to spread of the tumour cells to the soft tissue. Inadequate resection of the hard and soft tissues beyond a tumour would cause recurrence .Several reports are available in literature related to the recurrence of ameloblastoma as shown in Recurrences are described in the study of radical treatment of ameloblastoma by Sehdev et al. , ShatkinIt is the known fact that the rate of recurrence with the conservative management is high (around 60%) compared to radical treatment (13%). The pattern of recurrences postresection has to be evaluated in a larger extent. Reports have been suggested that there are higher possibilities of retained soft tissue tumour islands during the surgical procedure in the complex regions like infratemporal fossa , 35.In the first case report, the grafted tissue underwent tumorigenesis. This could be due to the remaining cells at the osteotomy site. It would be wise to wait for the histological report with the free margin before reconstruction in any case of the solid type to avoid donor site morbidities. The complication of primary reconstruction should be explained to the patient before surgery.Lesions of infratemporal fossa remain asymptomatic, and the chief complaint of the patient is usually facial swelling or deformity.Infratemporal fossa is pyramidal in shape that consists of complex structures, located on the lateral aspect of the cranial base, deep to the zygomatic arch, masseter, and mandibular ramus. The base formed by the medial aspect of the ramus and floor of the skull forms the upper surface of the pyramid. The anteromedial aspect corresponds to the posterior aspect of the maxilla and the posteroinferior aspect to the pterygomaxillary fascia . It is aNumerous surgical approaches have been employed to access the infratemporal region, some of them being the transoral, transanal, transpalatine, transzygomatic, transcervical, and extended maxillectomy approach . We founA possible explanation for recurrence in the second case would be retained periosteum over the coronoid process. Composite resection would eliminate the retention of a tumour infiltrated tissues.Carlson and Marx described the technique of excising the next uninvolved anatomical structure to prevent recurrence of a tumour. We do agree with his opinion of scientific approach towards curative management with histopathologic free soft and hard tissue margin . SimilarLaborde et al. and Becelli et al. reported no recurrence in his study of 7 patients' postsegmental resection , 8. SimiVaishampayan et al. suggested the possible cause for recurrence in the infratemporal region after segmental resection would be due to the retraction of pathologically weakened coronoid process fragment during temporal dissection .Peacock et al. concluded that there no additional benefit in confirming the margin by performing frozen section in addition to intraoperative specimen radiograph in his study of 35 patients. However, this study did not include the soft tissue margin .125 brachytherapy is tried to irradiate the recurrent lesion by delivering high prescribed doses of radiation (110 Gy) with satisfactory outcome [Though several reports suggest the radio resistant nature of ameloblastoma, I outcome .MRI study should be done in large ameloblastomas to evaluate the infiltration of the tumour into the adjacent soft tissue planes.Intraoperative radiography should be done to rule out positive hard tissue margin.Compartmental resection of the tumour should be performed to involve all positive margins and on table frozen section of the soft tissue margins .Reconstruction should be performed as staged surgery in giant ameloblastomas after the complete histopathology report is available.Long-term follow-up with MRI and CT imaging should be conducted after the primary surgery to evaluate any form of recurrence.We propose few guidelines in the management when dealing with ameloblastoma eroding the cortical borders:Our experience with two case reports suggests high local aggressiveness of the solid type of ameloblastoma. Tumour-free soft tissue margin in three dimensions should be considered when treating large lesions with an erosion of cortical outline. Clinical and histological study in larger extent will provide added information in the management.


FGFR1 duplication with additional variants of unknown significance in several genes . This is one of only a very few reported cases of anaplastic PA with characterization of molecular alterations.We report the case of a 27-yr-old male with visual field loss who had a 4.9-cm complex cystic mass in the right occipital lobe. Histologic examination showed pilocytic astrocytoma (PA) with anaplasia, and molecular characterization revealed  A 27-yr-old male presented to the emergency department with a complaint of left visual field loss after 2 mo of intermittent blurry vision. On examination, he had left homonymous hemianopsia. Magnetic resonance (MR) imaging demonstrated a 4.9 × 2.7 × 2.5-cm complex multiloculated ring-enhancing lesion in the right occipital lobe with hemorrhagic foci A. Right Microscopic examination of the tissue showed a glial neoplasm with atypical cells displaying thin bipolar cytoplasmic processes B, focallFGFR1 gene was identified. In addition, five variants of unknown clinical significance were also identified . The test performs at a median depth of coverage of 500×.The diagnostic challenges associated with this case relate to the presence of histologic features that overlap between PXA and PA and the increased mitotic activity with vascular proliferation, focally elevated Ki67 index, and p53 staining in a young patient. PA occurs in adults, with lobar localization being a frequent finding (∼44% in some series) . HoweverBRAF-KIAA1549 fusion, respectively) were not present. However, an FGFR1 duplication, involving exons 10–18, which contains the protein tyrosine kinase domain (TKD), was identified. Duplication of the FGFR1 TKD has been reported in low-grade astrocytomas (including PA), usually in an extracerebellar location, and in dysembryoplastic neuroepithelial tumor (DNET)  pathway alterations are common in PA; in fact, PA is considered to be predominantly a single pathway disease resulting from MAPK dysregulation . PXA andr (DNET) . In one igenesis . The oveFGFR1 TKD duplication, this case of PA with anaplasia showed additional genetic alterations of unknown biological and clinical significance (ARID1A p21_S222INSPP variant in ClinVar (reported as likely benign), but we consider the evidence insufficient to make a final determination. There is one COSMIC entry for the CHEK2 T367fs*15 variant, and ClinVar shows 14 submissions of frameshift mutations in CHEK2, starting at codon 367. The variant is considered likely pathogenic in ClinVar. However, the significance of this variant in the context of our case is uncertain.In addition to the ificance . The COSARID1A, ARID1B, and CHEK2 have been reported in brain tumors . However, the molecular defects associated with anaplasia in PA are not well known, although an association with neurofibromatosis type 1, the PI3K pathway, and p16 loss have been reported (FGFR1 TKD in anaplastic PA.Genetic defects in n tumors . It remareported . This careported  and highhttps://www.ncbi.nlm.nih.gov/clinvar/) and can be found under accession numbers SCV000692539, SCV000692540, SCV000692560, and SCV000692561.The variants were submitted to ClinVar  at the University of Texas MD Anderson Cancer Center (# PA17-0216) with waiver of informed consent.The authors have declared no competing interest.Mariarita SantiKar-Ming FungAnonymous


An 80-year-old woman with a medical history of diabetes and duodenal cancer presented to our emergency department (ED) complaining of sudden severe dyspnea after vomiting. She was alert and oriented on arrival, but showed tachypnea and poor oxygenation. Inspiratory stridor was evident. A computed tomography (CT) revealed a dilated esophagus with food bolus and intraluminal air compressing the trachea at the level of the sternoclavicular joint . Her symThis case presented reversible, severe tracheal compression by dilated esophagus with no functional abnormality. Cases of tracheal compression by dilated esophagus with structural diseases such as hiatal herniationWhat do we already know about this clinical entity?Cases of tracheal compression by dilated esophagus with hiatal herniation or esophageal achalasia have been reported.What is the major impact of the image(s)?Reversible tracheal compression by dilated functionally normal esophagus was evident on the first CT.How might this improve emergency medicine practice?Spontaneous esophageal dilation is rare, but worth considering when we see the ED patients complaining of sudden severe dyspnea.Documented patient informed consent and/or Institutional Review Board approval has been obtained and filed for publication of this case report.


We report a case of hyperphosphatemic tumoral calcinosis (TC) that occurred after total knee arthroplasty. A 64-year-old Japanese man presented with painful swellings in both shoulders, the left elbow, and the right hip that developed after he underwent total knee arthroplasty (TKA). The pathology of the patient's bone at the time of TKA included a thick osteoid seam with calcareous deposition at the margin of the trabecular bone, which is not generally seen in osteoarthritis. Computed tomography scans of the swollen joints demonstrated leaflet and amorphous calcification masses around the joints. We diagnosed the patient with TC. The present case highlights that TC lesions are rare but should be considered in the differential diagnosis of subcutaneous soft and hard masses around the joint. GALNT 3), fibroblast growth factor 23 (FGF-23), and αKlotho [Tumoral calcinosis (TC) is a distinct disease of uncertain etiology that is defined by massive extra-articular soft tissue deposition of calcium phosphate around large joints, such as the hips, knees, shoulders, elbows, and wrists , 2. Seco αKlotho , 5.Idiopathic TC has two subtypes. The normophosphatemic TC subtype is characterized by normal serum phosphate and calcium levels, and the hyperphosphatemic TC subtype is characterized by elevated serum phosphate levels with normal serum calcium levels .The total knee arthroplasty is common surgery in orthopaedics. In the past, there was no report that TC has occurred after total knee arthroplasty. Here, we report a case of TC that occurred after total knee arthroplasty.µL; creatinine, 0.73 mg/dL; calcium, 9.7 mg/dL; phosphate, 7.5 mg/dL ; total type I procollagen N-terminal propeptide, 605 µg/L ; and tartrate-resistant acid phosphatase-5b, 6710 mU/dL . Rheumatoid factor and anti-cyclic citrullinated peptide antibody were absent. Plain radiographs of the left shoulder disclosed a large, lobulated, and periarticular soft tissue calcareous mass. Computed tomography (CT) scans demonstrated leaflet and amorphous calcification masses around both shoulders and into the latissimus dorsi muscle and axilla, the right hip, and the left elbow with same calcification masses (Figures A 64-year-old Japanese man presented with pain, swelling, and limited motion in both knees with gait disturbances. We diagnosed the patient with osteoarthritis and performed total knee arthroplasty in both knees. Intraoperative findings revealed that the patient's bone was very hard. The pathology of the patient's bone included a thick osteoid seam with calcareous deposition at the margin of the trabecular bone, such as is not usually seen in osteoarthritis . About 1 Figures . The calTC is a heterogeneous disorder of obscure etiology characterized by extensive nonosseous calcification, especially in the periarticular soft tissue regions of major joints . TC commGALNT 3 and FGF-23 in familial TC. The novel homozygous missense mutation in exon 3 of the GALNT 3 has been reported as one of the factors in the gene [GALNT 3 mutation-related calcification disorders have yet to be clarified.The etiology of TC, including the mechanism and genetic basis of the entity, remains obscure. Recent molecular research has suggested a role of the gene . HoweverIn our case, the patient underwent total knee arthroplasty before the onset of TC. The pathological findings of the bone at surgery indicated calcium deposition. This finding may suggest the bone of TC. We had better doubt abnormal bone metabolism including TC if there is a finding of such a bone.The treatment of TC is considered to be the reduction of serum phosphate through a restricted diet and oral phosphate-binding drugs, such as calcium carbonate. Clinical and radiographical improvement has been reported after phosphorus deprivation therapy . SurgicaThe case has occurred TC after total knee arthroplasty. We suggested that there was no casual connection between the surgery and the onset of TC. The present case highlights that TC lesions are rare but should be considered in the differential diagnosis of subcutaneous soft and hard masses around the joint. Moreover, when a surgeon observes a hard bone during surgery, TC should be considered. Therefore, we think that the most important thing is to actively examine as CT for early diagnosis after the surgery.


Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms. In 1811, MS was described for the first time by Burns. Afterwards, in 1853, King called the disease chloroma due to its greenish appearance . The incMS is an uncommon neoplastic condition , 5 and aIn this paper, the authors describe the MRI findings and clinical manifestations of an uncommon case of spinal MS at the sacral level in a 5-year-old girl who presented with radiculopathy symptoms and had a previous history of AML.2 per dose ×6, in each cycle. Intrathecal therapy was also carried out. The patient was in remission by morphology and flow cytometry assessment after the first cycle and in molecular remission after the second cycle by flow cytometry assessment. Four months after the end of therapy, she presented progressive radicular pain of the lower right extremity and gait claudication. Neurological assessment revealed symmetrical depressed reflexes of the lower limbs and reduction of right knee extension. The posterolateral regions of the right buttock and thigh were painful to the touch. Laboratory evaluation showed normal blood counts. The imaging study evaluation with an MRI of the spine revealed a soft tissue intracanalar epidural mass located from L5 to S4, which extended through multiple sacral neural foramina to the presacral space. The tissue involved sacral nervous roots (A 5-year-old girl with acute myeloid leukemia (AML) with t underwent relatively standard AML chemotherapy, which consisted of 2 cycles of induction chemotherapy with daunorubicin, cytarabine, and etoposide, followed by 2 cycles of consolidation chemotherapy with high-dose cytarabine 3 gr/mus roots  and the us roots . The infus roots . Based oFollowing the institutional guidelines for relapsed AML, the patient started FLAG-IDA as a relapse protocol. Intrathecal chemotherapy was not performed in the first cycle due to the mass's large volume.The patient was in remission by morphology and flow cytometry assessment after the first cycle of FLAG-IDA. Neurological assessment revealed normal and symmetric reflexes of the lower limbs, as well as symmetric and normal knee extension. At that time, the patient did not have pain in the right buttock or thigh. The MRI evaluation revealed a significant tumor mass volume reduction at that time .Triple intrathecal therapy was performed at the beginning of the second FLAG-IDA cycle and there were no blasts detected in the cerebrospinal fluid. Following the second cycle, the patient experienced a four-month period of hematologic aplasia, which postponed the allogeneic stem-cell transplant . Meanwhile, she remained in remission but did not fully recover from the hematologic aplasia, while the platelets levels remained low. She started maintenance chemotherapy, in addition to intrathecal therapy (no blasts in the CNS fluid) as a bridge to allo-HSCT. At this point, the patient is waiting for allo-HSCT. The decision to undergo radiotherapy has been postponed until further evaluation after the transplant, as there was no growth of the sacral mass during the prolonged bone marrow aplasia.In literature, MS is classified according to the predominant cell type  . MS can  cauda equina syndrome. Those complications are rare [Haversian channels serve as a bridge for tumor cell migration from the bone marrow to extramedullary sites. Spinal cord involvement is infrequent  and the are rare .AML reservoirs are usually bone marrow, the central nervous system, and the testes. The sacral spinal canal is a specialized AML reservoir  and the Langerhans cell histiocytosis X, lymphoma, metastatic tumors, and Ewing sarcoma/PNET are the differential diagnoses of MS . Bone maMRI is the best imaging study to distinguish MS from other pathologies . During Systemic chemotherapy using AML-like regimens should be initiated early, even in nonleukemic disease , with inIn this paper, the authors describe a rare case of leukemia relapse, presented as sacral MS in a girl with a progressive right-sided lumbosacral plexus neuropathy. The reader should bear in mind that sacral MS presents symptoms similar to other spinal lesions. MS should not be neglected and should be maintained under the differential diagnosis of spinal mass lesions. MRI, bone marrow aspiration, and pathological and histological staining can result in its correct diagnosis.


Primary carcinoid tumors originating in a normal kidney are exceptionally rare. We describe a case of a 52-year-old woman who presented with vague back pain and was found to have a 4-cm mass in the medial aspect of the left kidney. Radical nephrectomy and retroperitoneal lymphadenectomy were performed. Histopathology revealed a typical carcinoid tumor with 1 out of 17 lymph nodes involved. She has no evidence of recurrence or metastasis on 7-month follow-up.


Fibromas are classified in a spectrum from fibromas to fibrosarcomas according to the number of mitosis they include. Malignant fibrosarcomas which have aggressive pattern show higher mitotic activity and nuclear atypia. Cellular fibromas with less than 4 mitotic figures under 10 high power fields (HPF) are benign. “Mitotically active cellular fibromas” that are classified between the cellular fibromas and fibrosarcomas, have ≥4 mitotic figures in 10 HPF but do not have nuclear atypia. A very few cases of mitotically active cellular fibromas have been reported in the literature. In this report, we present the case of mitotically active cellular fibroma in a patient who applied to our clinic with the complaint of pelvic mass. There are few cases with benign features but high mitosis count, named as “mitotically active cellular fibroma”,3,4,5,6. These cases have more than four mitotic figures but behave as benign. Clinical outcome is different from fibrosarcoma which is an aggressive disorder. In this report, a case of mitotically active cellular fibroma (MACF) will be discussed.Cellular fibroblastic tumors are classified as fibroma or fibrosarcoma. Cellular fibromas include less than 4 mitoses (<4MF/10HPF), have normal nucleus and benign feature. Fibrosarcomas have more than 4 mitoses, display nuclear atypia and they are malignantA twenty-four-year old single woman presented with abdominal distention and a palpable abdominal mass. On physical examination, a pelvic mass measuring 15 cm in diameter was palpated up to the level of the umbilicus. In abdominal ultrasonography, a 14 cm left adnexal mass which had solid-cystic pattern was observed. Magnetic resonance imaging (MRI) revealed increased signal intensity in the fat-suppressed T1A sequences of the solid components of the lesion and probable hemorrhagic lesions were considered. In the diffusion-weighted series, low signal intensity was observed in the solid components . Serum CThere were no intra- or post-operative complications and she was discharged on the second day following the surgery. She is still followed up by our clinic and there is no recurrence.. However, some case reports in the literature and the widest study on this subject which was reported by Irving et al. claimed that tumors with four and more mitoses, but without nuclear atypia should not be regarded as fibrosarcoma. These cases should be named as “mitotically active cellular fibroma” (MACF).Malignant sex cord stromal tumors are the second most common ovarian tumors after epithelial ovarian tumors. Categorized within this group, fibrosarcomas which have excess in number of cellular mitosis and with nuclear atypia are distinguished from fibroma. Prat and Scully made the first analysis on this issue and reported that tumors with four or more mitoses are named as fibrosarcoma. The median age of the patients in MACF group was 41 years. Our patient, who was 24 years old, is one of the youngest patients in the literature. The most important symptom is pelvic mass, as in our case. Abdominal pain, acute abdomen due to torsion, dysfunctional uterine bleeding, and ascites can be other symptoms in a decreasing frequency. Incidentally detected cases on physical examination, ultrasound, or intraoperatively  have been reported. Meigs syndrome with pleural effusion was found in a patient. In the literature, 2 cases mimicking epithelial ovarian cancer with CA125 elevation have been reported. In our case, CA125 level was above the normal limits; 297 U/mL.Irving et al. reviewed 40 MACF and 35 cellular fibroma cases. In our case, the tumor size (15 cm) is larger than the tumors reported in the literature. Extraovarian disease has also been reported in some patients. This involvement is usually in the form of adhesions to other genital organs  or on the peritoneal surface of pelvic sidewall, it can also be on the omentum or bowel mesentery. In our case, the tumor was found in the mesentery of the sigmoid colon and in adhesions to the bladder peritoneum.The average size of MACF has been reported to be 9.4 cm,7. Prat and Scully reported no recurrences in mild nuclear atypia group and in 66% (4/6) of moderate nuclear atypia patients(7). One of the remaining 2 patients died due to recurrence, 2.75 years after the diagnosis. Other patient with recurrence died from non-tumoral causes. Once more, it is necessary to re-emphasize that patients with recurrence have moderate nuclear atypia. Irving et al. reported 4.75-year follow-up of 18 MACF cases with 3 extraovarian involvement and evidence of recurrence was observed in none of the patients. Kaku et al. reported a case of MACF with 10 mitoses under 10 HPF without any recurrence in one-year follow-up.In the microscopic evaluation, MACF includes more cellularity compared to a cellular fibroma but does not contain nuclear atypia (bland nuclei). The median mitosis count was found to be 6.7 although 19 mitoses were reported under 10 HPF. This case was a 65-year-old woman and recurrence was seen in the Douglas pouch 5 years after the operation. Adjuvant tamoxifen (20 mg/day) was started after surgical resection and no recurrence was reported at six months follow-up.The only study that reported recurrence in patients with MACF in the literature was published by Bucella et al.In these cases, fertility-sparing surgery is of great importance due to average age of occurrence of the disease. Therefore, the correct diagnosis of the disease has the most important role in protecting the patient’s fertility. Due to subjective evaluation of the cellular atypia, diagnosis should be correlated with clinicopathologic and immunohistochemical data in order to perform fertility-sparing surgery and to prevent initiation of adjuvant treatment.As a result, the clinical behavior cannot be predicted accurately and local recurrence can be seen in these tumors. Thus, it is indicated that they should be regarded as a low malignant potential tumor. Therefore, morphological findings should be correlated with intraoperative and other clinical/imaging findings and close monitoring of patients is recommended.


Primary hepatic lymphomas (PHLs) are rare liver tumors, frequently misdiagnosed preoperatively. As these tumors could be successfully treated with chemotherapy, their early recognition is essential, potentially, to avoid useless surgery. We report on the case of a cirrhotic patient with hemochromatosis who presented a PHL, initially diagnosed as a hepatocellular carcinoma (HCC), and we analyze recent data from the literature on this subject.  A 45 mm liver tumor was found is a 68-year-old man with alcohol cirrhosis and hemochromatosis. At imaging, the diagnosis of HCC was suspected according to vascular characteristics and the presence of cirrhosis. FDG PET scan showed a solitary hypermetabolic liver tumor. Tumor markers were negative. Surgery consisted in left lateral hepatectomy. At pathology, the diagnosis of the primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) type was demonstrated. Twenty-two articles reporting 33 cases of true PHL of MALT type were found. Presentation lacked specific symptoms (70% asymptomatic). Half of patients were suspected to have other etiologies of liver mass , and thus diagnosis was established postoperatively. In the patient, diagnosis was made by preoperative biopsy, and chemotherapy was first-line treatment.  Preoperative diagnosis of PHL, and particularly of primary hepatic MALT lymphoma, is challenging. This case illustrates that PHL remains to be considered among the differential diagnosis of isolated solid liver tumors. Further, it indicates that biopsy could be still indicated in case of suspected HCC in cirrhotic patients, particularly in the presence of unusual findings such as the combination of a FDG PET scan positive tumor in the absence of elevated alpha-fetoprotein. Primary hepatic lymphoma (PHL) represents less than 10% of space occupying focal hepatic lesion . PrimaryWe performed a systematic review of the literature using two databases (PubMed and Scopus). In the PubMed search, we used the following search terms (“Liver Neoplasms” [Mesh]) AND “Lymphoma, B-Cell, Marginal Zone” [Mesh]. Free terms included . In the Scopus database search, we used the terms TITLE-ABS-KEY .The search strategy had no publication date or publication type restriction. In addition, the reference lists of relevant reviews or included articles were also searched to find other eligible studies. We included only studies published in English.Study characteristics such as patient age, sex, and presenting symptoms, associated liver disease and lesions, the presence of cirrhosis, associated liver lesions, timing of diagnosis (preoperative biopsy or postoperatively), type of treatment, and outcome were evaluated.λ and IG κ (kappa). The pathology of the nontumor liver confirmed the presence of cirrhosis.A 68-year-old male patient with hemochromatosis and Child A cirrhosis due to alcohol consumption was referred to our department for a liver tumor. The patient is known to have a Child A cirrhosis without any previous episode of liver decompensation. Physical examination was normal, and laboratory tests showed a minimal elevation of transaminases levels. Serologies for hepatitis B and C and tumor markers, including alpha-fetoprotein (AFP) and CA19.9, were negative. Contrast-enhanced liver MRI showed a 46 mm tumor in segment III with arterial wash in and portal phase washout Figures . Liver mThe patient's disease was classified as Ann Arbor stage IE , and no adjuvant therapy was proposed.A total of 158 articles were identified from the PubMed and Scopus. After removing duplicated articles, 124 articles were assessed further assessment. A total of 92 articles were excluded on the basis of the titles and the abstracts. Only true cases of primary hepatic MALT were included (Ann Arbor stage IE). All cases of extrahepatic biliary tract MALT were excluded. Of the remaining 32 articles, only 22 articles had full text published in English. After full-text review of these remaining articles, they were all eligible and included in the systematic review .A total of 22 articles  reportinThere were 17 male (51.5%) and 16 female (48.5%) patients with a mean age of 61.7 years (range 36 to 85 years). Twenty-one patients (63.6%) were asymptomatic at presentation, 3 had abdominal pain (9.1%), and 2 had elevated liver enzymes (6.1%). Fifteen patients had associated liver disease. Cirrhosis was documented in 7 cases (21.2%) including hepatitis C and B and primary biliary origins. Two patients (6.1%) had associated HCC lesion at the time of diagnosis. MRI and CT scan reports were available in only 6 and 7 cases, respectively. PHL of MALT type was described in most cases to be hypointense T1 and iso- or hyperintense T2 on MRI and hypodense with variable enhancement on contrast-enhanced CT. The number of hepatic MALT lesions ranged between 1 and 6 with a mean of 1.34 and median of 1. The mean size of the lesions was 35.61 mm (15–90 mm). Detailed FDG PET report was available in only 1 case series of 5 patients, suggesting that these tumors only show minimal increase in FDG uptake, as indicated by mean SUV of 5.5 (3.5–8.2) . In anotPrimary hepatic MALT was diagnosed based on biopsy in 15 cases (45.5%) and postoperatively in 16 (48.5%) cases. Treatment options varied depending on the timing of diagnosis. Exclusive chemotherapy (most had R-CHOP), and exclusive radiotherapy were administered in 6 patients (18.2%) and 2 patients (6.1%), respectively. Tumors were resected in 23 patients (70%); of these, 4 were diagnosed posthepatic transplantation (12.1%), and the remaining had surgical resection of the tumor.Helicobacter pylori eradication treatment, radiotherapy in 5 (15.2%), 1 (3%), 1 (3%), and 1 (3%), respectively. The follow-up period ranged between 9 months and 5 years with a median of 24 months. At the end of follow-up, only 3 relapses (9.1%) were noted and they were treated by chemotherapy (R-CHOP) in 2 patients [Adjuvant therapy was administered in 8 patients and consisted of chemotherapy, antiviral treatment, patients . In operIn this case, the initial presentation was highly suggestive for the diagnosis of HCC, according to vascular imaging features, the presence of cirrhosis, and for the diagnosis of hemochromatosis. On this basis, in the presence of Child A cirrhosis without significant portal hypertension, surgical resection was decided, without preoperative biopsy , 31. On In conclusion, primary hepatic MALT lymphoma is an extremely rare tumor with poorly contributive clinical, laboratory, and imaging findings. Early recognition is infrequent, and the definite diagnosis is mostly based on histopathologic examination of operative specimen. The present case illustrates that this diagnosis should be kept in mind and that preoperative biopsy could be still indicated, particularly when the diagnosis of HCC is suspected but in presence of unusual findings such as the combination of positive FDG PET scan with normal AFP level.According to literature review, the preoperative diagnosis of this tumor remains a challenge, due to the scarcity of specific clinical and imaging signs , 14, 24.


Rubinstein-Taybi Syndrome (RSTS) is an autosomal dominant disorder that is classically characterized by prenatal and postnatal growth restriction, microcephaly, dysmorphic craniofacial features, broad thumbs and toes, and intellectual disability. We describe the first reported case of a pituitary macroadenoma associated with RSTS.A 39-year-old Caucasian female with a past medical history of RSTS diagnosed at age two was found to have a gadolinium-enhancing pituitary mass on magnetic resonance imaging (MRI) of the brain three years ago during workup for migraine-like headaches. Subsequent serial imaging showed radiographic evidence of growth up to 11.5 x 14.0 x 10.0 mm in size. The pituitary sellar lesion was resected through an endoscopic transnasal transsphenoidal approach and was found to be a thyrotroph adenoma.RSTS is a rare, neurodevelopmental genetic disease where most patients with disabilities survive into adulthood. The disorder is associated with an increased predisposition for development of nervous system tumors, including pituitary adenomas. Rubinstein-Taybi syndrome (RSTS) is an autosomal dominant disorder characterized by mutations in the cyclic-AMP-regulated enhancer binding protein (CREBBP) gene in 1p13.3 and its homolog, E1A binding protein p300 (EP300) on chromosome 22. RSTS is clinically characterized by prenatal and postnatal growth restriction, microcephaly, dysmorphic craniofacial features, broad thumbs and toes, and intellectual disability. Other common associated findings include syndactyly, musculoskeletal anomalies including patellar dislocations, scoliosis and cervical spondylolisthesis, and congenital heart abnormalities such as patent ductus arteriosus, arterial septal defects and ventricular septal defects. Over 90% of RSTS individuals with disabilities survive to adulthood, and healthcare for these patients is complex, involving an interdisciplinary approach across multiple subspecialties [Patients with RSTS have an increased predisposition for the development of tumors of the nervous system . We descA 39-year-old Caucasian female, with a past medical history of RSTS diagnosed at age two, presented with new persistent migraine-like headaches of several weeks duration. A magnetic resonance imaging (MRI) of the brain performed in 2012 showed a 10 mm intrasellar, gadolinium-enhancing mass enlarging non-functional pituitary macroadenoma, which was followed for three years. Her past medical history is significant for anxiety, chronic nasal obstruction, congenital macroglossia, and chronic sinusitis. Her past surgical history is significant for repair of polydactyly at birth, mandibular surgery for significant prognathism and correction of nasal septum at 13 years of age, and surgical repair of large atrial septal defect at age 18. Medications include aspirin, escitalopram, lorazepam, and oxymetazoline. She has not had genetic testing for gene mutation analysis, and she is the middle child of a family with an elder female sister and younger male brother, neither of whom exhibit any characteristics of RSTS. On physical examination, she exhibited maxillofacial dysmorphism and some mild cognitive delay, but otherwise was neurologically intact with normal visual fields.Follow-up neuroimaging in 2015 showed evidence of tumor growth and prompted surgical intervention. MRI brain with and without gadolinium revealed a 11.5 x 14.0 x 10.0 mm hypoenhancing mass in the inferior pituitary gland, causing upward bowing of the diaphragma sella and mild sellar expansion  surgeon was required for the endoscopic approach to the sella turcica. Bilateral endoscopic sphenoidotomies and septoplasty were performed to expose the rostrum of the sphenoid sinus, which was removed to identify the sella and site of the tumor. A white-colored, fibrotic tumor was encountered and dissected from the adjacent pituitary tissue. Image guidance was then used for a volumetric excision. Multiple Valsalva maneuvers were performed without evidence of cerebrospinal fluid leak. The floor of the sella turcica was reconstructed using bone and septal cartilage from the exposure and layered with fibrin glue.Excisional biopsy of the pituitary lesion showed expanded acini and trabeculae of minimally pleomorphic oval nuclei with conspicuous nucleoli associated with eosinophilic granular cytoplasm Figure . ImmunohThe patient was extubated following surgery but developed acute airway obstruction due her pre-existing macroglossia. She was reintubated to secure the airway and was monitored overnight in the intensive care unit, where she was successfully extubated the following day. A consideration to facilitate reintubation in this patient with a known difficult airway would be utilizing an airway exchange catheter as a bridge to a difficult extubation. She was placed on hydrocortisone taper and the remainder of her postoperative course was uneventful. She was discharged on postoperative day 2 without evidence of diabetes insipidus or cerebrospinal fluid leak.This case describes an adult female with RSTS and clinically non-functional, TSH-positive pituitary macroadenoma. RSTS is a rare, neurodevelopmental and plurimalformative autosomal dominant genetic disease with a low population prevalence . Although there are no precise and standard diagnostic criteria for RSTS, individuals have distinctive features including typical facial features, microcephaly, broad thumbs and first toes, intellectual disability and postnatal growth retardation. Other organs and systems may be affected as well; however, there is no particular sign or symptom that is pathognomonic for RSTS.Facial features in RSTS are characterized by low frontal hairline, downslanting of palpebral fissures, dysplastic and low-set ears, an arched palate, mild micrognathia, and dental abnormalities . Other kThere is a known pattern of increased predisposition for tumors of the brain and of tissue derived from neural crest origins in patients with RSTS. The estimated incidence of such types of tumors, malignant or benign, in patients with RSTS is five percent . An epidA genetic cause of RSTS was first discovered in 1991, when a de novo reciprocal translocation with breakpoints in chromosomal region 16p13.3 was observed in some afflicted patients. Molecular analysis of 16p13.3 identified mutations in the CREBBP gene in approximately 50% of patients with RSTS -22. MutaRSTS is a rare, neurodevelopmental autosomal dominant genetic disease where most patients with disabilities survive into adulthood. The disorder is associated with an increased predisposition for development of nervous system tumors, including pituitary adenomas. We now add a pituitary macroadenoma to the literature regarding tumors associated with RSTS.


Dear SirAbdominal wall defect in Gastroschisis is usually located to the right of the umbilical cord. We discuss a rare case of left-sided gastroschisis. A 3-day-old female, borne of caesarean delivery at term to a 20year-old mother, came to the emergency with bowel loops lying outside the abdominal cavity. No significant antenatal history was present. Antenatal sonogram done outside the hospital at 28 weeks gestation had detected the lesion. On examination, this 2 KG child was lethargic and febrile. Matted and oedematous small bowel loops without any covering membrane were lying outside the abdominal cavity through a defect of approximate size 2.5x2.5cm, to the left of the umbilical cord . There was a perforation in the exposed bowel through which meconium came out. Routine blood investigations revealed leukocyte count of 13800. At surgery, the defect was extended for 2cm on either side vertically. The eviscerated bowel loops were meticulously separated to reach the site of perforation in the proximal ileum. There were multiple atretic segments in the distal bowel. Abdominal wall was manually stretched before returning the bowel loops inside the abdomen and fashioning a stoma at the site of perforation. Patient had stormy postoperative course and died on the 5th postoperative day due to sepsis-related complications.Left sided gastroschisis (LSG) is rare with only 23 reported cases in literature.  Most of these cases have been females. Compared to the commonly observed right sided lesions, LSG have increased incidence of associated extra-intestinal and intestinal anomalies which make their prognosis worse. [3] Situs inversus, pubic diastasis, bifid clitoris, double vagina, anteriorly placed anus, atrial/ventricular septal defect, patent ductus arteriosus, Choledochal cyst, cerebral arterio-venous malformation, macrocephaly, scoliosis and stenosis of superior vena cava have all been variously reported.  Intestinal atresia in gastroschisis is a known association. [3] Hombalker et al. described a case of LSG with caecal agenesis. [4] Our patient had multiple atresias and a perforation in proximal ileum. This worsened sepsis and prognosis in our patient. Source of Support: NilConflict of Interest: None


Mesenteric cyst is a rare clinical entity especially in pregnancy; therefore, few cases have been reported in the literature. The standard method of their treatment is surgical excision either with laparotomy or laparoscopy. In addition, mesenteric vein thrombosis is a rare and life-threatening condition in pregnancy and needs immediate treatment because it can lead to intestinal necrotic ischemia. This is the first report of the coexistence of mesenteric cysts and mesenteric vein thrombosis during gestation.A 27-year-old Greek woman, gravida 2 para 1, presented at 10 weeks’ gestation to the Emergency Unit of our hospital complaining of diffuse abdominal pain which deteriorated the last 3 days, which was localized in her right iliac fossa, along with vomiting. She had undergone open laparotomy and right salpingo-oophorectomy at the age of 23 due to an ovarian cyst. Besides this, her personal and family medical history was unremarkable. She had never received oral contraceptives or any hormone therapy. On arrival, a clinical examination revealed tenderness on palpation of her right iliac fossa, without rebound tenderness or muscle guarding. Within 10 hours of hospitalization, her symptoms deteriorated further with rebound tenderness during the examination, tachycardia, and a drop of 12 units in her hematocrit value. An emergency laparotomy was performed. Two mesenteric cysts and a 60 cm necrotic part of her intestine were revealed intraoperatively. In the postoperative period, she complained of acute abdominal pain, tachycardia, and dyspnea. Computed tomography imaging revealed mesenteric vein thrombosis and pulmonary thromboembolism. She was treated with low molecular weight heparin and she was discharged on the 11th postoperative day.To the best of our knowledge, this is the first report in the literature of a simultaneous mesenteric cyst and mesenteric vein thrombosis in pregnancy. It is known that pregnancy is a state of hypercoagulation and clinicians should bear in mind this rare clinical condition in their diagnostic algorithm for acute abdominal pain. Mesenteric cysts and cystic mesenteric tumors constitute a very rare abdominal entity. The location of these cysts can be anywhere in the mesentery, from duodenum to rectum . Since tA 27-year-old Greek woman, gravida 2, para 1, presented at 10 weeks’ gestation to our hospital complaining of diffuse abdominal pain for a week with deterioration the last 3 days and localization in her right iliac fossa, with simultaneous vomiting . Her medical history was unremarkable, although she had undergone laparotomy and right salpingo-oophorectomy at the age of 23 due to an ovarian cyst.2. Her respiratory rate was normal. A cardiac examination showed S1, S2, mild tachycardia, and no murmur. A thorough neurological examination was performed; she was alert, attentive, and oriented. Her speech was clear and fluent with good repetition, comprehension, and naming. In addition, muscle bulk and tone were normal. Leukocytosis  and increased serum C-reactive protein (185 mg/dl) were the only pathologic findings. Obstetrical and gynecological causes were the first to consider; therefore, we tried to make an evaluation of the patient by transvaginal ultrasonography excluding causes of acute abdominal pain such as placenta disruption, abortion, ectopic pregnancy, or huge myomas which may have led to this deterioration. An intrauterine pregnancy with a live fetus and normal bilateral adnexa were revealed. A vaginal examination and surgical physical evaluation were normal.On arrival, a clinical examination revealed tenderness on palpation of her right iliac fossa, without rebound tenderness or muscle guarding. She had a temperature of 36.2 °C, her blood pressure was 154/97 mmHg, and her pulse rate was 97 beats per minute. Her body mass index (BMI) was 37.1 kg/mWithin 10 hours of hospitalization her symptoms deteriorated further with rebound tenderness, tachycardia (130 beats per minute), and a drop of 12 units in her hematocrit level. A transvaginal ultrasonography showed accumulation of free fluid in the pouch of Douglas, with normal left adnexa; a transabdominal ultrasound of her upper and lower abdomen by radiologists revealed a cyst approximately 9 cm diameter which was of unknown origin. Due to her increased BMI, her history of a previous laparotomy, and the lack of available qualified specialists for laparoscopy at that time, an immediate emergency laparotomy was decided  and was treated with piperacillin and tazobactam . She was mobilized very early and wore antithrombotic socks continuously; she was administered low molecular weight heparin (LMWH) with tinzaparin by injection (Innohep) 5000 IU subcutaneous injection once per day. Blood cultures revealed 2) 29 mmHg, partial pressure of oxygen (pO2) 63 mmHg, bicarbonate anion (HCO3-) 21.6 mmol/L, oxygen saturation (SO2) 94%, and her serum level of d-dimers was 28,132 mg/l. Based on the fact that she was pregnant, that she had recent surgery, the presentation of thrombosis during surgery, her obesity, and her clinical symptoms, an emergency thorax CT scan for the possibility of pulmonary embolism (CT pulmonary angiogram) was ordered, while the dosage of LMWH (tinzaparin by injection) was increased to 7000 IU subcutaneous injection once per day. The thorax CT scan showed embolism to branches of her pulmonary artery, small pleural effusion with atelectasis in the dorsal part of her lungs, and small pericardial effusion. A supplementary abdominopelvic CT scan with an injection of iodine-rich contrast material administered intravenously revealed a small filling defect – thrombus – in the celiac axis of her abdominal aorta, mesenteric vein thrombosis (MVT) was revealed as filling defect in her mesenteric vein, as well as filling defect in her common iliac vein and in the initial portion of her left external iliac vein , respiratory rate of 25 breaths per minute, dyspnea without chest pain, and a blood pressure of 121/76 mmHg. An electrocardiogram (ECG) revealed sinus tachycardia; her fibrinogen was 3.6 g/dl, activated partial thromboplastin time (APTT) 35 seconds, international normalized ratio (INR) 1.16, prothrombin time (PT) 13.4 seconds, pH 7.48, partial pressure of carbon dioxide  Leiden mutation, heterozygous for methylene tetrahydrofolate eductase (MTHFR) C677T mutation, and heterozygous for MTHFR C1298T mutation. A paroxysmal nocturnal hemoglobinuria (PNH) test was negative. The results for lupus anticoagulant, anti-cardiolipin antibodies (ACA), and anti-beta-2-glycoprotein I (Aβ2GpI) antibodies (IgG and IgM) were all in normal ranges. Protein S and protein C deficiency had normal values. She was discharged on the 11th postoperative day with normal blood and urine tests. A histological diagnosis confirmed two lymphatic cysts, ischemic intestinal necrosis, hemorrhagic mesenteric fat, and a hemorrhagic and swollen appendix; a thrombosed vascular venous branch was observed in the mesenteric adipose tissue proximate to the smaller cyst Fig. .Fig. 3HiShe was discharged with instructions to take one tablet of rivaroxaban 20 mg (Xarelto) administered orally once per day for 6 months. She continues to receive this anticoagulant therapy, although she does not complain of any suspicious symptoms. She is in continual communication with surgeons, gynecologists, and hematologists of our department and her next appointment will be in the next few days at the time of this writing.et al., in a recent paper (2014), described the seventh case of mesenteric cysts during pregnancy [Cysts of the mesentery were first described by Benevieni, a Florentine anatomist, in 1507 during an autopsy on an 8-year-old boy , 5. The regnancy ; it was nt paper 014, descnt paper 014, descregnancy . Almost Several causes have been described for these cysts: some believe that their origin is from continual growth of congenitally malformed or malpositioned lymphatic tissue, while others propose theories such as lymph node degeneration or trauma theory in which mesenteric cysts develop secondarily. Cysts of the mesentery can be located from the duodenum to the rectum anywhere in the mesentery .The cysts can be asymptomatic and on average 50% are detected incidentally  through et al. in 2000 divides the mesenteric cysts in six groups, namely lymphatic cysts and lymphangiomas, cysts of mesothelial origin, cysts of enteric origin, cysts of urogenital origin, mature cystic teratomas (dermoid cysts), and non-pancreatic pseudocysts [The classification proposed by de Perrot udocysts . Their fudocysts . Malignaudocysts .Complete surgical excision is the treatment of choice for mesenteric cysts, either by laparoscopy or laparotomy .Despite the fact that surgical operations should be avoided during pregnancy, non-obstetric laparotomy is performed in 1.6 to 2.2% of pregnancies. Pregnancy used to be a contraindication for laparoscopy but recent data show that laparoscopic procedures can be performed safely during pregnancy [Pregnancy as a hypercoagulable state “is a normal situation associated with increased risk of venous thromboembolism.” Pregnant women have a fourfold to fivefold increased risk compared with non-pregnant women . FactorsMesenteric venous thrombosis is very difficult to diagnose due to its nonspecific clinical symptoms, the most frequent complaint in cases of acute MVT is abdominal pain ; apart fMesenteric venous thrombosis is a rare cause of acute mesenteric ischemia, representing only 5 to 15% of patients . MVT haset al. in a recent case report, after reviewing the literature, stated that only 16 cases of small bowel ischemia during pregnancy have been reported and only nine cases (56.2%) were associated with MVT [Intestinal ischemia after MVT is an uncommon entity during pregnancy. Lin et al. described the tenth case report in which, despite the existence of MVT and bowel resection laparoscopically, fetal viability was preserved, in this case for a pregnant woman in her 7th week of pregnancy [Recently (2016), García-Botella regnancy .et al. (2004) [Plenty of risk factors have been described for MVT including portal hypertension, postoperative complications, neoplasia, trauma, inflammatory bowel disease, malignancy, pregnancy, hemoglobinopathies, protein S deficiency, protein C deficiency, oral contraceptive agents, and the use of estrogen-containing compounds . MVT due. (2004) . At leas. (2004) .The early diagnosis of MVT, which is a potentially life-threatening condition, is very important to prevent high mortality and morbidity . Non-chaOur case report is the 11th case in the literature of MVT during pregnancy which led to mesenteric ischemia, whereas it is the eighth case in the literature of a mesenteric cyst during pregnancy. According to the literature, this is the first case of a mesenteric cyst and simultaneous MVT in a pregnant woman.In our case report, we cannot be sure of the exact cause of thrombosis and intestinal necrosis, but we guess that thrombosis is due either to the strangulation of the vessels from the lymphatic mesenteric cyst or it is idiopathic, without forgetting that pregnancy plays a pivotal role leading to MVT as a “hypercoagulable” situation.This is the first case in the literature of a simultaneous mesenteric cyst and MVT in a pregnant woman who initially presented with acute onset abdominal pain. Despite the rarity of this condition in the gestational period, clinicians should take into account that pregnancy is a state of hypercoagulation and the existence of a mesenteric cyst and MVT, a potentially life-threatening condition, should be ruled out in the diagnostic process even in women with no known thrombophilia.


Trigger finger is a relatively common clinical entity. The etiology is most often attributable to stenosing tenosynovitis though traumatic cases have been described. When trigger finger is associated with an underlying flexor tendon rupture, previous cases have reported a clear association with overt laceration or previous trauma. We present the case of a 23-year-old male active duty military service member who presented with a characteristic history and clinical exam consistent with trigger finger. The symptomatic onset was gradual, with no history of inciting trauma. Given symptomatic persistent triggering following failure of conservative management to include cortisone injections, the patient was taken for open A1 pulley release. Intraoperatively, the triggering was found to be attributable to a partial attritional rupture of the small finger flexor digitorum profundus tendon. Tendon debridement, tubularization, and A1 and partial A2 pulley releases were performed with subsequent resolution of triggering. We present the rare case of subclinical atraumatic attritional rupture of the FDP tendon to the small finger as a cause of clinically apparent trigger finger. This is an important consideration as the hand surgeon must be prepared to address more atypical pathologies. Trigger finger is a common condition typically arising spontaneously, with a reported lifetime prevalence of approximately 2.5 percent , 2. TrauA 23-year-old right-hand dominant male active duty military service member was initially seen in our orthopaedic hand clinic upon referral by his primary care physician for further evaluation and management of a suspected trigger finger. The patient complained of six months of progressively symptomatic locking of the dominant small and ring fingers with pain over the palmar aspects of the bases of both digits. The patient was unable to recall any inciting trauma. His daily activities included repetitive manual activities involved with military service.Physical exam was significant for painful active triggering of the small finger when brought from a position of maximal flexion to extension as well as palpable nodularity and tenderness to palpation over the palmar aspects of the small and ring finger metacarpophalangeal (MCP) joints. There was otherwise no extensor lag, and the patient was able to make a full composite fist.Given the patient's history and clinical presentation, a cortisone injection was administered to the small finger A1 pulley and flexor tendon sheath. He reported complete pain relief following the injection and was sent to occupational therapy. The patient returned to orthopaedic hand clinic with continued pain relief but persistent triggering of the small finger six months later. With the exception of no tenderness, the exam was otherwise unchanged. Given that his symptoms were refractory to conservative management, he was taken to the operating room for small finger A1 pulley release.A standard longitudinal incision was made overlying palmar aspect of the small finger MCP joint from the distal palmar crease to the palmodigital crease distally. The pulley was exposed and upon release a frayed, degenerative-appearing flexor tendon stump emerged . InteresPostoperatively, the patient was immobilized in a dorsal blocking splint for soft tissue rest for 1 week and subsequently enrolled in early active range of motion with occupational therapy. Follow-up 3 months postoperatively revealed excellent strength and range of motion with no further triggering.We present a unique case of an atraumatic partial rupture of the small finger FDP with a classic clinical presentation of trigger finger necessitating flexor tendon repair and A1 pulley release. This has previously not been reported in literature. Literature is replete with several case reports of trigger finger attributable to flexor tendon or pulley rupture following steroid injection or from laceration to the flexor tendon –5. KimurBoth open and percutaneous techniques for A1 pulley release have been described . TraditiWe present the rare case of subclinical atraumatic attritional rupture of the FDP tendon to the small finger as a cause of clinically apparent trigger finger. This is an important consideration as the hand surgeon must be prepared to address more atypical pathologies.


Ocular histiocytic sarcomas (as a presenting part of disseminated histiocytic sarcoma) are not commonly diagnosed. A 10-year-old female intact Rottweiler presented to the School of Veterinary Medicine, Trinidad with buphthalmia and pain in the left eye. The cornea of the left eye appeared diffusely opaque with a conjunctival mucopurulent ocular discharge. A thorough ophthalmic assessment identified an intraocular proliferative tumor to which a unilateral enucleation was performed, however the animal died soon after. Post mortem examination and light microscopy revealed that the intraocular lesion with visceral macro-metastases was in fact a histiocytic sarcoma. Further to this, immune-phenotyping was performed to confirm the diagnosis of disseminated histiocytic sarcoma. This is the first time such a tumor has been diagnosed in Trinidad and Tobago. Canine histiocytic disease is the umbrella under which a wide spectrum of proliferative disorders have been characterized according to varying biologic characteristics. This group of diseases include distinct subdivisions; canine cutaneous histiocytomas, canine reactive histiocytosis (cutaneous and systemic forms), and the histiocytic sarcoma complex  . HistoriThe disseminated neoplastic process of histiocytic sarcomas is an aggressive multisystem disease with the formation of multiple tumors in various organ systems carrying an unfavorable prognosis. Predilection sites for primary lesions include the spleen, lymph node, lung, bone marrow, central nervous system, skin and subcutis, and periarticular and articular tissues of the limbs. Secondary sites are also varied, but typically include the liver and lungs . When hiHistiocytic sarcomas most characteristically express the surface molecule CD18 . HoweverThis report describes a case of canine disseminated histiocytic sarcoma based on its morphology and immune-phenotype which is the first report of its type in Trinidad.A 10-year-old female intact Rottweiler presented to the Veterinary Teaching Hospital (VTH), School of Veterinary Medicine (SVM), The University of the West Indies, St. Augustine (UWI). It was diagnosed with an intra-ocular neoplasm of the left eye, which was subsequently enucleated, fixed in 10% formalin for 48 h, and sent to the veterinary anatomical pathology unit of the SVM for histological examination. The animal subsequently died and the carcass was also submitted to the veterinary anatomical pathology unit of the SVM, where a routine post mortem evaluation was conducted. The grossly visible lesions were identified, and described as needed. The fresh tissues were sectioned and immediately placed in 10% neutral buffered formalin for fixation. All histological tissues were routinely processed, cut with a microtome at 4 µm, and stained with hematoxylin and eosin (H&E). Immunohistochemistry (IHC) was performed using anti-CD18 , anti-MHCII , and anti-CD3 antibodies , at the Pathology Diagnostic Laboratory, College of Veterinary Medicine, University of Georgia. Basic steps include deparaffinization and antigen retrieval, endogenous peroxidase blocking, and power block. Then the slides were incubated with primary antibody at the following dilutions: CD3—1:1000, CD18—1:50, and MHCII—1:5000. They were then incubated with appropriate secondary antibodies, which were biotinylated anti-rabbit  for CD3 and biotinylated anti-mouse . Then they were incubated with streptavidin and horseradish peroxidase, followed by chromogen development using 3,3′-diaminobenzidine (DAB) and hematoxylin counterstaining. All of these used canine lymph nodes for positive control and isotype control serum for negative control.A 10-year-old female intact Rottweiler, with a satisfactory body conditioning presented to the VTH-UWI for a clinical evaluation. The patient had buphthalmia and pain in the left eye one-week prior to consultation. The left eye was diffusely opaque, exuding a mucopurulent discharge. An ophthalmic and ultra-sonographic evaluation was conducted immediately. The results indicated that the right eye was clinically normal. Conversely, the left eye displayed diffuse corneal edema, conjunctival discharge, and an increased intraocular pressure (39 mmHg) with a lack of fundic detail. A diagnosis of a unilateral intraocular tumor and secondary glaucoma of the left eye was assigned.® eye drops. However, a unilateral enucleation was the recommended course of therapy given the extent of the lesion. Subsequently, the left eye was surgically removed with the concomitant excision of a cutaneous tumor on 6 November 2014. Bloodwork indicated only an increased level in serum creatinine, but the animal was eating and bright, alert, and responsive prior to surgery. She became lethargic and passed dark tarry stool (melena) on 7 November 2014, and died the day after (two days after surgery).Consequently, ultra-sonographic findings displayed evidence of retinal detachment. The focally extensive ocular mass was assessed within the posterior chamber, measuring 13 mm at the temporal aspect of the globe. Medical management involved the use of LantanoprostThis dog was given a body condition score of 5/5 due to excessive discernible fat stores. Throughout the pulmonary parenchyma, there were multifocal white to pale yellow, semi soft masses ranging from 100 × 120 mm to 10 × 20 mm, with the largest occupying over 50% of the right cranial lobe . AdditioIn the left eye, over 60% of the retina was effaced by a large mass that occupied approximately 70% of the posterior chamber . The masThe neoplastic cells expressed strong plasmalemmal staining for CD18 . The celThis case presented with the initial complaint of an intraocular lesion manifesting itself as clinical buphthalmia. Histiocytic sarcoma has reportedly been an uncommon primary intraocular neoplasm , althougSynonymous with the human counterpart, the accurate diagnosis of canine localized and disseminated histiocytic sarcomas in dogs requires immunophenotypic evaluation for confirmation . Further


Conjunctival hemangioma over the age of 60 is rare, with few cases reported in the literature. We present a unique case of a conjunctival capillary hemangioma, adding to the sparse literature of this uncommon vascular tumor. Here, we present an interesting case of spontaneous development of this tumor at age 68, without associated systemic disease process or cutaneous manifestations. Vascular tumors of the conjunctiva are uncommon; capillary hemangiomas of the conjunctiva are quite rare -6. Here,A 68-year-old male presented with a ‘blood filled cyst’ in his right eye of three months duration. The patient denied pain, change in vision, size, or color of the lesion over this time period. He denied eye trauma, allergy, or use of medications (including) blood thinners and non-steroidal anti-inflammatory drugs (NSAIDS). His past medical history was significant for trans-sphenoidal surgery for pituitary macroadenoma two years prior. His best-corrected visual acuity was 20/25 in both eyes. The ophthalmic evaluation including a dilated fundus examination was unremarkable except for the slit lamp exam of the right eye which revealed a 2.5 mm, partially mobile, solid conjunctival hemorrhagic lesion with well-defined borders, without feeder vessels or overlying lissamine green stain. The tumor is demonstrated in Figures The clinical diagnosis at that time was conjunctival varix or conjunctival hemangioma. We suggested observation as the lesion did not appear suspicious of malignancy (or growth per the patient), and followed up six weeks later.On return, the lesion appeared to have grown in size by approximately 0.5 mm. This is demonstrated in Figures The lesion was excised completely and sent for histopathologic analysis. The patient was treated with a course of topical antibiotic and steroids and healed well.The conjunctival biopsy showed subepithelial proliferation of variably thin-walled vascular channels, lined by a single layer of endothelial cells; some filled with blood. Extravasated red blood cells were also identified; trichrome stain revealed prominent stromal collagen and minimal fibrosis within the walls of only a few of the lesional vessels. The lesion was negative for desmin stain , and positive for CD31 and CD34 stains, confirming the presence of vascular endothelial elements. The final pathologic diagnosis was conjunctival capillary hemangioma. Photos of the histology slides are shown in Figures Conjunctival capillary hemangioma is a rapidly developing tumor derived from endothelial cells that has rarely been reported in patients over 60 years old , 6.  MosPathology of this lesion usually shows an intact surface epithelium with positive markers for vascular endothelium and pericytes —as did ours [Management of these lesions depends on the presumptive diagnosis, size, and extent of the lesion , 6. SeriAs with all tumors of the conjunctiva, it is important to manage each case individually and observe for unusual characteristics and growth pattern. Our case is unique from those previously published in the literature, in that our patient spontaneously developed this tumor at age 68 without any associated systemic disease process or cutaneous manifestations. Most importantly, relatively rapid growth was documented photographically.


Neoadjuvant radiation therapy, followed by definitive surgical resection, remains the standard of care for resectable high-grade and unresectable soft tissue sarcomas. Proton therapy offers the promise of highly conformal dose distributions with improved sparing of neighboring normal tissues as compared with conformal and intensity modulated photon techniques. It is unclear whether proton therapy may offer an improved tumoral response, especially with dose escalation, in this relatively radio-insensitive tumor type. We, herein, present a patient with an excellent pathologic response to preoperative pencil beam scanning proton therapy despite a complex treatment course. Accounting for approximately 1% of all adult tumors, soft tissue sarcoma (STS) remains a rare disease entity with over half arising in the extremity . PreoperMore conformal techniques, e.g. intensity modulated radiation therapy (IMRT) and reduced treatment volumes have each effected lower rates of complication without sacrifices in disease control in institutional series and a major cooperative group trial, respectively -5. Due tSmall series and dosimetric studies have suggested the utility of PT in this setting -7. HowevA 62-year-old female with no major medical history initially presented with a one-month history of left hip pain and left leg swelling. Her pain progressed, and she developed trouble ambulating. This prompted diagnostic imaging, including magnetic resonance imaging (MRI) with contrast of the left femur. This study revealed a heterogeneously enhancing mass measuring 6.4 cm anterior-posterior x 7.5 cm transverse x 7.1 cm craniocaudal centered in the left abductor brevis muscle containing some cystic areas and a small amount of surrounding edema -guided biopsy of the mass was performed, which confirmed a diagnosis of high grade undifferentiated pleomorphic sarcoma. Two lymph nodes in the groin were biopsied and were negative for metastatic involvement. Positron emission tomography-computed tomography (PET-CT) examination demonstrated a metabolically active left inner thigh mass without evidence of regional lymphadenopathy or distant metastasis. Therefore, the patient was clinically diagnosed as Stage III, T2bN0M0 (Grade 3).She was recommended for and elected to undergo neoadjuvant radiation therapy prior to surgical resection. Given the deep-seated location of the tumor just inferior to the femoral head and neck and abutting the femoral shaft, PBS-PT was recommended to limit the dose to the left hip as well as to the vaginal structures. Dose reductions to the left thigh, femur, and hip were expected to reduce her risk of post-treatment edema or fracture.For radiation planning, an MRI with contrast was obtained in the treatment position and fused with the planning CT. As per our institutional guidelines and in concordance with the Radiation Therapy Oncology Group (RTOG) Phase II Protocol 0630 for Neoadjuvant Radiation for Soft Tissue Sarcoma, the gross tumor volume (GTV) was contoured based on post-gadolinium T1 imaging and expanded by 3 cm superiorly and inferiorly and 1.5 cm radially, respecting normal tissue and compartmental boundaries (unless apparently involved), to generate the clinical target volume (CTV).  All T2 flair changes were included in the CTV, if not covered by the expansion. A planning target volume (pPTV) was generated based on lateral setup uncertainty of 5 mm and beam-specific range uncertainty. However, robust evaluation of CTV coverage was assessed for plan approval. For image guidance during treatment, kilovoltage (kV) images were performed daily and aligned to the bone. Quality assurance computed tomography (QA-CT) scans were performed at week one and week three of treatment to evaluate for soft tissue swelling and tumor changes. An additional QA-CT was performed in the latter half of her treatment due to increased swelling noted on exam.The dose prescribed was 50.4 Gy RBE in 1.8 Gy RBE per fraction. The proton plan employed a single field optimization (SFO) technique with two coplanar fields entering anteriorly at gantry angles of 30 degrees and 350 degrees, respectively Figure . The lefThe patient developed significant swelling and pain during the course of treatment. She also experienced intermittent fevers and sweats. Infectious workup, including complete blood count (CBC), urinalysis, and chest x-ray, was repeatedly negative. Her pain worsened significantly over a short period of time, which completely impaired her ability bear weight on the affected leg. She underwent a repeat MRI midway through treatment that revealed the mass to appear noticeably larger, measuring 8.2 x 8.5 x 9.5 cm, with a large cystic region within the mass and increased surrounding muscular edema Figure . MediallBy the end of treatment, the patient was generally wheelchair-bound; however, she could bear some weight on her left leg.Due to her symptomology and concern for progression, the patient was taken to the operating room only 12 days after completion of radiation therapy. A gross total resection with primary closure was achieved without complication. Final pathology revealed an undifferentiated pleomorphic sarcoma, measuring 9.5 cm with negative resection margins. The closest margin was 3 mm at the deep margin but represented the periosteal layer. A near complete response was noted with greater than 80% of the tumor necrotic, consistent in appearance with treatment effect  antibiotics and surgical debridement with the application of a vacuum-assisted closure ((VAC) device. One month following this procedure, her wound was noted to be healing nicely. MRI performed four months post-treatment demonstrated no evidence of recurrent disease Figure .PBS-PT offers substantial dosimetric benefits over 3D-CRT and IMRT in the treatment of extremity STS that could lead to meaningful improvements in clinical outcomes, especially with regard to acute wound/bone and long-term limb toxicity . Beyond To date, there is relatively limited data regarding outcomes following PT for STS, especially in the extremity subset. The University of Pennsylvania has recently presented results with PT for sarcomas arising in previously irradiated fields in 23 patients . High-grThe current case presented, however, offers a number of critical clinical points to consider. First, a substantial increase in patient discomfort and tumoral volume must always raise concern for tumoral progression under treatment. This necessitates consideration of early surgical intervention or cessation of the neoadjuvant approach. However, tumoral swelling is also a well-documented phenomenon that should give some pause before aborting the planned course of therapy. In this case, a reasonable balance was struck by completing the preoperative RT expediently but then proceeding to surgical resection earlier than intended (12 days instead of four to six weeks).The patient did require aggressive wound care and antibiotics, consistent with wound complications occurring in approximately one-third of patients receiving neoadjuvant RT for STS . The neaThe critical importance of image-guidance and clinical vigilance in the delivery of PT is also highlighted. A tumoral volume change such as this may not compromise a 3D-CRT plan with adequate field "flash" or even an IMRT plan with numerous beam angles, but PT is highly sensitive to such anatomical changes. This swelling, if unmonitored, could have led to dramatic dose changes in tumor coverage and organs-at-risk (OAR) exposure. Image guidance with intermittent 3D imaging is a must. Re-simulation with an assessment of dose distribution (with re-planning as necessary) should be standardly employed when anatomical changes present during the delivery of PT, as was done in this case. Beam angles and delivery techniques, e.g. single field optimization (SFO) that increase robustness to such changes, should be consciously sought in the PT planning process if acceptable tumor coverage and sparing of OARs can be achieved.The impressive pathologic tumoral response is very encouraging. Pathologic response after neoadjuvant photon radiation for STS has been previously reported in a retrospective setting to range from 5% - 100% with a median response rate of 35% . In thisAnother study found a median pathologic response of 67.5% for low-grade and 50% for high-grade tumors . In the The exact mechanism for the rapid necrosis and cystic degeneration of this patient’s tumor is unclear. However, PT treatment effect from direct cellular damage with or without resultant vascular compromise is suspected. Considering the excellent pathologic response with minimal residual disease at only 12 days post-PT, it is likely that, given a normal post-neoadjuvant interval of several weeks, a pathologic complete response may have been achieved at resection. It is unclear whether PT will offer improved tumoral responses over traditional photon techniques in this relatively radio-insensitive histology or similar tumor types. Improved modeling of RBE across the PT beam is eagerly sought and may clarify these effects. However, PT’s potential for dose-escalation without substantially added toxicity holds promise for an improved therapeutic ratio. Additional research is justified to clarify the role of PT in STS.PBS-PT is safe and effective for the delivery of neoadjuvant RT prior to definitive surgical resection for high-grade or unresectable STS. PT may offer substantial sparing of normal tissues, as well as increased RBE across the SOBP , over that achievable with conformal photon techniques. An excellent tumoral pathologic response was achieved in this case, though it was clinically confounded by tumor volume increase and worsened patient discomfort. Additional investigation is required to optimize proton therapy delivery and RBE characterization.