The pituitary gland is an uncommon site for metastases, in particular from rhabdomyosarcoma. Some authors have reported a recent increase in the incidence of metastases at infrequent sites, such as brain or bone, probably due to the expanded treatment options and the resulting improved survival. Treatment options are limited, but must be discussed and adapted to the patient profile.We report the case of a 17-year-old Arabic man, diagnosed with alveolar rhabdomyosarcoma of the left shoulder, who, after several cycles of chemotherapy, presented symptoms and signs of pituitary dysfunction. To the best of our knowledge, it is the first case described.Pituitary metastasis of rhabdomyosarcoma is a rare situation, which must be actively researched to have access to an optimal therapeutic approach. The occurrence of pituitary metastasis is a very rare complication in oncology . Data frA 17-year-old Arabic man was admitted to our medical oncology department for management of alveolar rhabdomyosarcoma (RMS) of the left shoulder. The initial workup included a computed tomography (CT) scan of the body and a bone scan, which revealed lung and bone metastases. Chemotherapy with an IVA regimen was initiated . His radiological and clinical evaluation after three courses of treatment showed a stable disease, with significant improvement in quality of life. After his fifth cycle of chemotherapy, he presented to our emergency department with polydipsia and polyuria, headache and vomiting associated to diplopia, and a decrease in visual acuity in his left eye. A CT scan of his brain revealed a 13 × 15mm diameter pituitary lesion (Figures RMS is the most common soft-tissue tumor of childhood, and responsible for approximately one-half of all soft-tissue sarcomas in this age group ,6. HowevTreatment, mostly palliative, depends on symptoms. Surgical exploration and decompression, alone or combined with radiation, is often necessary when suprasellar extension causes progressive deterioration in vision and/or pain . In our The prognosis is generally poor, because the vast majority of cases of pituitary metastasis occur in association with multiple systemic metastases and are typically associated with end-stage disease. . Mean suHere we report the case of a patient presenting with metastatic alveolar RMS, with a large sellar mass having appeared under treatment. This metastatic event is very uncommon, making it almost impossible to perform prospective clinical trials specifically designed to compare different treatment approaches. Thus, only a greater awareness of the problem along with a more accurate and timely diagnosis, will lead to choosing the best therapies suitable to the specific patient and, in turn, improve overall prognosis.Written informed consent was obtained from the patient’s next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors read and approved the final manuscript.


The eccrine poroma or Hidracanthoma Simplex is a rare benign adnexal tumor of ephitelial cells, with an incidence of 0.001 to 0.008%1. In two-thirds of the patients it appears on the soles and lateral borders of the feet. We report the case of a patient with pigmented eccrine poroma in abdominal skin, of a rare entity presentation with a single report in the literature in that location. Herein, we present a case of a 56-year-old female with a pigmented and painful nodule with progressive growth in the abdominal region with 15-year evolution, which presented easy bleeding in the last months prior to consultation. Upon physical exam, a hyperpigmented and angiomatous pedunculated nodule was found with hyperkeratotic surface The patient attended clinical controls post intervention, without lesion recurrence found until now.,-Eccrine poroma is a benign adnexal tumor, of rare occurrence, with an incidence of 0.001 to 0.008%, first reported by Pinkus in 1956Melanocytes and melanin are rare in eccrine poroma and two hypotheses are posed regarding its presence originating the pigmentation: the first is that it comes from melanocytes present in cell primordia of eccrine ducts during the 14th week of gestation that were not eliminated during the maturity process; the other hypothesis is that it comes from epidermal melanocytesThis benign tumor has a favorable prognosis, without recurrence after complete resection and low risk of malignancyA case is presented of a patient diagnosed with pigmented eccrine poroma on abdominal skin, of unusual presentation and location, with only one case reported in the literature


Background. Abdominal masses in female adolescents are uncommon. A rare cause of this condition is hematocolpos due to imperforate hymen. Case. We present a case of an unusually massive asymptomatic abdominal bulk in a 14-year-old female patient, who sought for medical advice after unusual abdominal pain lasting for few weeks. The patient was otherwise asymptomatic, apart from an unusual dramatic expansion of her abdominal wall during the last month. We describe the surgical management and the follow-up of the patient. Summary and Conclusion. Clinicians should keep in mind that an imperforate hymen can cause abdominal growth due to hematocolpos and include it in the differential diagnosis of such a clinical entity in female adolescents. 2D ultrasound is usually efficient for the confirmation of the diagnosis of hematocolpos, but 3D ultrasound is more accurate. Wide excision should be undertaken, as an initial approach, to avoid recurrence. An emerging abdominal tumor in young females is a rare situation and requires a specific clinical and ultrasonographic approach. Common causes of a newly diagnosed abdominal mass in young females include cysts and solid tumors of various origins. In this context, hematocolpos is a rare entity that can cause such symptoms: it comprises the blood collection in the distal closed vagina and is usually diagnosed in young adolescents with no menstruation and cyclic abdominal pain. Its incidence is approximately one every 2000 young adolescents  and in 9Usual clinical signs include cyclical low abdominal pain, urinary retention, back pain, primary amenorrhea, and/or a quickly enlarging pelvic tumor. It may also affect neonatal age and can be manifested as fetal ascites or renal failure , 3, someA 14-year-old girl was admitted to the Pediatric Emergency Department of the Department of Obstetrics and Gynecology, Johannes Gutenberg University of Mainz, Mainz, Germany, with primary amenorrhea, an expanding abdominal mass, and mild abdominal pain. There was no history of severe abdominal pain during the last year and the patient complained of polyuria during the last month; there were no signs of defecation. Her parents sought for medical assistance because of a growing tumor in her abdomen. At clinical examination, secondary sexual characteristics were present and within normal ranges. The clinical presentation was quite impressive: a thin girl with a BMI of 22 with a painless, nontender, soft, and homogenous mass, distorting her abdominal wall and expanding up to 5 cm over the umbilicus .The patient's vital signs were normal; laboratory tests revealed a hemoglobin concentration of 13 g/dL and white blood cell count of 11/nL, while CRP and tumor markers' concentrations were within normal ranges. In addition, her endocrine hormonal profile was indicating a girl with a mature hypothalamic-pituitary axis. Urinalysis was normal. Clinical examination of the abdomen did not reveal any pain or signs of peritonism. Clinical gynecological examination after retracting the labia minor revealed an imperforate hymen, which was bulging forwards. Rectal digital examination revealed a large bulky mass positioned anteriorly. A structure of 34 cm length, 11 cm width, and 11 cm height was evidenced at 2D transabdominal ultrasound .On the cranial, frontal end of the structure, cranial from the umbilicus and adapted to the front abdominal wall, we observed a uterus of normal size (no hematometra) , while b3D ultrasound displayed the clarity of the wall of this structure: it appeared straight, with no adherence to the neighboring organs, homogenous, with a fluid-like content in it.As the diagnosis was clear, surgical management was decided after providing the written informed consent of both parents and scheduled for the following day. A hymenotomy was performed under general anesthesia: at first, laser was used, followed by electrocoagulation, and an oval shaped piece of hymen was excised. A total sum of 2400 mL dark red, tarry blood was drained from the vagina. Of note, the maximum quantity reported in the literature is 3000 mL , 9; spon3D imaging during the first postoperative day revealed a waveform vagina, with a length of approximately 21 cm, whereas the size of uterus regressed for 10 cm under the umbilicus but did not enter the minor pelvis. The patient was discharged from the hospital after two days and a weekly follow-up with 3D ultrasound was scheduled. Menstruation occurred 20 days postoperatively and vaginal length was normalized 3 days after. During a scheduled follow-up appointment, 2 months postoperatively, a small amount of blood was detected in the vagina through 3D imaging; recurrence of the hematocolpos was confirmed after genital inspection. Reoperation was booked immediately, leading to a wider triangular tissue excision.The patient is not sexually active yet and during the last 12 months she has normal menstrual cycle and vaginal length, measured at ultrasound.The approach of a young patient presenting with a newly diagnosed abdominal tumor is always a demanding process. It causes fear to the child, emotions of guilt to the parents, and additional responsibility to the clinician. Apart from the pediatrician, other medical specialties can assist towards diagnosis and management, such as general surgeon, gynecologist, endocrinologist, and radiologist.Although hematocolpos consists of a rare clinical feature, it should be always considered a possible diagnosis in young females with primary amenorrhea and abdominal mass. Both diagnosis and treatment of hematocolpos are relative easy, but due to the sensitive nature of the disease, the approach of the patients presenting with that disease is demanding. Since the most serious complication is recurrence, in our experience, we recommend wide tissue excision as an initial approach, through a triangular or oval shape, instead of a cross or “X” shape incision. 2D ultrasound is the diagnostic tool of choice, but 3D ultrasound can reveal more details, such as the exact relationship of the feature with the neighboring organs and structures, since it provides better tissue differentiation and can assist in the vaginal length surveillance.


Background. Spontaneous intracerebral hemorrhage (ICH) is common and causes significant mortality and morbidity. To date, optimal medical and surgical intervention remains uncertain. A lack of definitive benefit for operative management may be attributable to adverse surgical effect, collateral tissue injury. This is particularly relevant for ICH in dominant, eloquent cortex. Minimally invasive surgery (MIS) offers the potential advantage of reduced collateral damage. MIS utilizing a parafascicular approach has demonstrated such benefit for intracranial tumor resection. Methods. We present a case of dominant hemisphere spontaneous ICH evacuated via the minimally invasive subcortical parafascicular transsulcal access clot evacuation (Mi SPACE) model. We use this report to introduce Mi SPACE and to examine the application of this novel MIS paradigm. Case Presentation. The featured patient presented with a left temporal ICH and severe global aphasia. The hematoma was evacuated via the Mi SPACE approach. Postoperative reassessments showed significant improvement. At two months, bedside language testing was normal. MRI tractography confirmed limited collateral injury. Conclusions. This case illustrates successful application of the Mi SPACE model to ICH in dominant, eloquent cortex and subcortical regions. MRI tractography illustrates collateral tissue preservation. Safety and feasibility studies are required to further assess this promising new therapeutic paradigm. Spontaneous intracerebral hemorrhage (ICH) affects approximately 2 million people worldwide each year . OverallA lack of definitive benefit for operative management may be attributable to adverse surgical effect, collateral tissue injury . This isWe present the initial case of ICH evacuated via the Mi SPACE model. We use this report to introduce Mi SPACE and to examine its application to ICH in dominant, eloquent cortical and subcortical regions.A 64-year-old right-hand dominant male presented to the emergency department with a one-day history of progressive left-sided headache and aphasia. He was normotensive, awake, and alert but unable to express orientation. He was nonfluent and unable to follow 1-step verbal commands, repeat, name, read, or write a sentence. CT head imaging revealed a large left temporal hematoma. CT angiography denoted the absence of an identifiable vascular etiology. The patient was admitted for monitoring and blood pressure regulation.Past medical history was significant for an occult bleeding diathesis, having presented over the past decade with two other ICH episodes. These events were conservatively managed and resulted in mild residual left-sided motor and sensory deficits.http://dx.doi.org/10.1155/2014/102307). Given the risk of further deterioration, prompt surgical intervention was deemed necessary. As the hematoma centred upon highly eloquent cortex with subcortical extension, the Mi SPACE approach was favoured. Starting with neuronavigation, Mi SPACE  was as follows: repetition 4/6, naming 1/6, and reading intact. CT imaging showed drainage of the hematoma, with improved mass effect .In the following evening there was an episode of hypertension, peak SBP 173 mm Hg, requiring administration of intravenous (IV) antihypertensive agents. The patient subsequently developed recurrent emesis and worsening aphasia, approaching admission status. Repeat CT imaging revealed reaccumulation of the hematoma with added mass effect . As such, he returned for a second evacuation using Mi SPACE. Given this patient's occult bleeding diathesis and history of recurrent ICH, this course of perioperative management included antifibrinolytic agent tranexamic acid (Cyklokapron), 1 g IV loading dose followed by 300 mg IV every eight hours for forty-eight hours, in conjunction with tight blood pressure control .Again, postsurgical examination demonstrated significant improvement and on same-day CT imaging the hemorrhage was no longer visualized . On postoperative day one he was awake and alert and expressed full orientation. He followed one- and two-step commands and communicated in full sentences. Naming and repetition were intact on NIHSS. At a two-month follow-up, NIHSS language testing was normal, and he had returned to his baseline level of function. At nine months, MRI with tractography demonstrated a small surgical tract extending from the left temporal cortex to the atrium of the lateral ventricle . Fractional anisotropy (FA) maps revealed minimal areas of decrease in FA in the left inferior longitudinal fasciculus and left temporal subcortical white matter .Spontaneous ICH is relatively common and it causes significant mortality and morbidity , 2. DespThough inconsistent, there is data supporting potential benefits of surgical intervention in ICH . As suchIn an attempt to limit tissue damage and reduce surgical morbidity, MIS techniques have been analyzed , 4. SeveMi SPACE represents an integration of 5 core technologies ((1) Mapping, (2) Navigation, (3) Access, (4) Optics, and (5) Resection) into a single platform to address such challenges. Preoperative mapping via MRI tractography allows the calculation of a surgical trajectory, along the long axis of the most eloquent fibres, to minimize shear forces and fascicle injury. Intraoperative neuronavigation facilitates a transsulcal insertion of a 13.5 mm port , also specifically designed to minimize strain forces, along the preplanned trajectory. This creates a parafascicular corridor that enables (i) use of a novel telescopic optics system, Video Telescopic Assisted Microscopy (VTOM) , to optimize visualization, (ii) bimanual dissection technique, and (iii) use of specifically designed nonthermal automated MIS instrumentation  without the need for thrombolytics.This case illustrates the successful initial application of the Mi SPACE approach to ICH in dominant, eloquent cortical and subcortical regions, in a patient with an occult bleeding diathesis. There was clear survival benefit as well as a dramatic early and lasting functional response. MRI tractography demonstrated limited impact on the eloquent fibre tracts, including the arcuate fasciculus, despite the initial hemorrhage, ICH recurrence, and two Mi SPACE evacuations.Intraoperatively, clot over the arcuate fasciculus was removed and we postulate that removal of this irritant effect was largely responsible for the improved outcome. Although this case's hematoma had a lobar focus, with superficial elements, there was also considerable subcortical extension. These deeper components were also targeted in the evacuation. Earlier iterations of the Mi SPACE model have shown safety and efficacy in managing various subcortical lesions . Thus, iIt has been suggested that a greater volume of hematoma removal is associated with better outcome . The potMi SPACE allowed for adequate intraoperative hemostasis in both interventions, despite the patient's occult bleeding diathesis, and there was excellent postoperative functional recovery. Furthermore, after clot reaccumulation, presumed to be from a combination of postoperative hypertension and the diathesis, repeat surgery within twenty-four hours of initial intervention yielded even better clinical and radiological outcomes. Typically, this is the period in which postoperative edema from conventional surgery becomes of greatest concern. It should also be noted that, in both instances of intervention, the patient underwent early surgery, within hours of clinical deterioration, which may offer benefit in some ICH subgroups .Perioperative management following the second surgery included antifibrinolytic treatment of the bleeding diathesis and more aggressive blood pressure control, after which there was no recurrent bleeding. Additional experience with Mi SPACE could further assess these aspects of ICH medical management.This case illustrates the successful initial application of the Mi SPACE model to ICH, including those in dominant, eloquent cortex and subcortical regions. There was clear survival benefit and dramatic functional response. MRI tractography demonstrates collateral fascicle preservation. Safety and feasibility studies are required to further assess this promising new surgical paradigm in ICH care.Mindful of manuscript length, the authors have included several additional figures as Supplementary Materials. These images contribute to further case description.


IDH1 mutations that confer increase survival and improved response to standard-of-care therapies.  By mapping the genetic landscape of brain tumors in routine clinical practice, we enable rapid identification of targetable genetic alterations. Molecular profiling has uncovered genetic subtypes of glioblastoma (GBM), including tumors with IDH1 (p.R132H), TP53 (p.Y126_splice), ATRX (p.R1302fs*), HNF1A (p.R263H) and NF1 (p.H2592del) variants and a NAB2-STAT6 gene fusion event involving NAB2 exon 3 and STAT6 exon 18. Array comparative genomic hybridization (aCGH) further revealed a focal amplification of NAB2 and STAT6.  IHC analysis demonstrated strong heterogenous STAT6 nuclear localization (in 20 % of tumor cells).A 29-year-old male presented with new onset seizures prompting neuroimaging studies, which revealed an enhancing 5 cm intra-axial lesion involving the right parietal lobe. He underwent a subtotal resection and pathologic examination revealed glioblastoma with mitoses, microvascular proliferation and necrosis. Immunohistochemical (IHC) analysis showed diffuse expression of GFAP, OLIG2 and SOX2 consistent with a tumor of glial lineage. Tumor cells were positive for IDH1(R132H) and negative for ATRX. Clinical targeted-exome sequencing (DFBWCC Oncopanel) identified multiple functional variants including NAB2:STAT6 fusions are common in solitary fibrous tumors (SFT), we report this event for the first time in a newly diagnosed, secondary-type GBM or any other non-SFT. Our study further highlights the value of comprehensive genomic analyses in identifying patient-specific targetable mutations and rearrangements.While  Furthermore, other rare genetic changes that are potentially therapeutically actionable, such as BRAF (p.V600E) mutations, are not routinely profiled in high-grade gliomas [Glioblastoma (GBM) is the most common, primary malignant brain tumor of adults with a median survival of 15 months despite multimodal surgical, chemo and radiotherapy [ase IDH1/ mutation therapy . Despite gliomas . ComprehMGMT methylation testing revealed that the tumor was positive for promoter methylation.A previously healthy 29-year-old male presented to an outside hospital with a symptomatic intra-axial enhancing right parietal brain lesion necessitating surgical management and adjuvant temozolomide Fig. . HistoloIDH1 (p.R132H), TP53 (p.Y126_splice), ATRX (p.R1302fs*), HNF1A (p.R263H) and NF1 (p.H2592del) variants, several variants of unknown significance, and a NAB2-STAT6 fusion involving exon 3 of NAB2 and exon 18 of STAT6  of 300 cancer-associated genes and 113 introns from 35 genes for rearrangement was performed on DNA extracted from formalin-fixed paraffin embedded tumor tissue , 7. OncoAT6 Fig. , Table 1NF1 p.H252del variIDH1, TP53 and ATRX) [http://cancergenome.nih.gov/). This analysis included 50 tumors with whole genome sequencing (WGS) and 311 tumors with whole exome sequences (WES). Among this 361 patient cohort, we did not detect any evidence of NAB2-STAT6 fusions, nor fusion events involving NAB2 or STAT6 with other fusion partners.To determine whether this event might be recurrent in gliomas, and given multiple genomic alterations detected in this tumor closely resemble those found in adult low-grade gliomas (ALGGs) (mutations in nd ATRX) , we inveNAB2-STAT6 fusions but did identify two unique STAT6 fusion events STAT6-CPM and HIPK2-STAT6 and each co-amplified with oncogenes CDK4 and MDM2 in the amplicon on chromosome 12q13-15. These observations suggest that STAT6 fusions occur at a low frequency but may be recurrent with other 12q13-15 amplification events.We next analyzed TCGA adult GBM datasets including 42 and 164 tumors tested by WGS and RNA sequencing, respectively. Similar to our findings among ALGGs, we did not detect NAB2, STAT6 and CDK4 involving a 12q3-14 amplicon of 0.6 Mb , or copy number analysis, of our case study GBM and also identified co-amplification of NAB2-STAT6 fusion, which is a known oncogenic driver in SFTs but has not been reported in other cancers. In SFTs, the novel fusion product results in nuclear translocation of the STAT6 transcriptional activating domain and activation of the early growth response (EGR1) pathway leading to tumorigensis [NAB2-STAT6 fusion has raised interest as both a diagnostic and potentially druggable therapeutic target [NAB2-STAT6 fusion events are rare in gliomas but that STAT6 fusions are recurrent events with several partners in adult GBM. Despite the low recurrence frequency, this fusion supports repurposing drugs developed against the NAB2-STAT6 or the EGR pathway in SFTs as a potential alternative or adjuvant therapy for patients with genetically similar gliomas.Incorporating molecular analyses into current diagnostic pipelines for GBM patients may provide an avenue for identifying novel aberrations or events that have been previously reported in other tumor types . One sturigensis , 11. Thec target . AlthougSTAT6 rearrangement events in GBMs, we noted CDK4 amplification in this patient. Chromosome 12 is frequently subject to a storm of amplification/rearrangement events in GBMs, including CDK4, MDM2, and HMGA2 amplifications as well as other regions (including KRAS). These events suggest a more complex rearrangement involving much of the chromosome and are reminiscent of ring chromosomes found in dedifferentiated liposarcomas [CDK4 and MDM2 to STAT6 and NAB2 in the chromosomal region 12q13-15, the fusion product may represent a consequence of this more complex rearrangement. Understanding the precise functional consequences of this molecular alteration in glioma biology will be important to guiding future therapies of similar cases [Interestingly, similar to other sarcomas –15. GiveWritten informed consent was obtained for 10–417 and 11–104 (OncoPanel) from the patient for sequencing analysis, publication of this report and accompanying images.


Although cavernous hemangioma is one of the most frequently encountered benign hepatic neoplasms, hepatic sclerosed hemangioma is very rare. We report a case of hepatic sclerosed hemangioma that was difficult to distinguish from an intrahepatic cholangiocarcinoma by imaging studies.A 76-year-old male patient with right hypochondralgia was referred to our hospital. Abdominal ultrasonography revealed a heterogeneously hyperechoic tumor that was 59 mm in diameter in segment 7 of the liver. Dynamic computed tomography showed a low-density tumor with delayed ring enhancement. Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI) demonstrated a low-signal intensity mass with ring enhancement on T1-weighted images. The mass had several high-signal intensity lesions on T2-weighted images. EOB-MRI revealed a hypointense nodule on the hepatobiliary phase. From these imaging studies, the tumor was diagnosed as intrahepatic cholangiocarcinoma, and we performed laparoscopy-assisted posterior sectionectomy of the liver with lymph node dissection in the hepatoduodenal ligament. Histopathological examination revealed a hepatic sclerosed hemangioma with hyalinized tissue and collagen fibers.Hepatic sclerosed hemangioma is difficult to diagnose preoperatively because of its various imaging findings. We report a case of hepatic sclerosed hemangioma and review the literatures, especially those concerning imaging findings. The preoperative diagnosis of hepatic sclerosed hemangioma is very difficult, even with recent developments in radiological modalities, because it is an extremely rare benign disorder and its radiological features resemble those of hepatic malignancies such as cholangiocarcinoma and metastatic liver cancer ,2. We reA 76-year-old male patient had consulted a doctor for upper abdominal pain 16 months before being referred to us and had been followed up. Because plain computed tomography (CT) revealed a space-occupying lesion in the liver, he was referred to our hospital. A laboratory workup on admission showed that total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, albumin, and creatinine were all within normal ranges. Tumor markers including alpha-fetoprotein, protein induced by vitamin K absence or antagonist-II, carcinoembryonic antigen, and carbohydrate antigens 19–9 were also within the normal limits  revealed a well-defined, heterogeneously hyperechoic mass that was 59 mm in diameter in segment 7 of the liver Figure . Plain CLaparoscopy-assisted posterior sectionectomy and cholecystectomy including lymph node dissection in the hepatoduodenal ligament were performed for a preoperative diagnosis of intrahepatic cholangiocarcinoma. The resected specimen revealed a white solid mass, sized 61 × 46 mm. The cut surface of the tumor was elastic, soft, and homogeneous with the smooth margin including some faint red spots up to 10 mm in size , performed in just 6 cases, showed no accumulation of [18F]-FDG (Table Concerning the imaging studies, Doyle et al. summarized the imaging findings of 10 hepatic sclerosed hemangioma lesions and found the characteristic features to include a geographic pattern, capsular retraction, decrease in size over time, loss of previously seen regions of enhancement . And addSurgical resection for hepatic sclerosed hemangioma is controversial. Most of the tumors reported were resected due to a preoperative misdiagnosis of malignancy Table . To makeMakhlouf and Ishak compared the findings of sclerosed hemangioma and sclerosing cavernous hemangioma. According to their theory, recent hemorrhages and hemosiderin deposits rich in mast cells are present in the sclerosing hemangioma. While, fibrosis, increased elastic fibers, and dystrophic or psammomatous calcifications with a decreased number of mast cells can be observed in the sclerosed hemangioma . Our casWe report a case with a hepatic sclerosed hemangioma. Although it is a rare disease, it is important to distinguish hepatic sclerosed hemangioma from hepatic malignancies. However, it is extremely difficult to diagnose precisely from imaging studies. If the possibility of a malignant tumor cannot be ruled out, hepatic resection might be selected for diagnostic therapy.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


A rare case of complete large bowel obstruction in a pregnant woman, without previous surgical history, due to previously undiagnosed reversed intestinal rotation is presented. The young woman was admitted with progressive nausea and vomiting which did not respond to conventional therapy. Her plain abdominal film revealed signs of small bowel obstruction. On laparotomy, her transverse colon was found to be located beneath the root of small bowel mesentery and completely obstructed by congenital fibrous bands. Postoperative recovery was unremarkable. Surgery for this unusual developmental anomaly is discussed. Nausea and vomiting are common in pregnancy. Most of these patients achieve symptomatic control with medical management. Since bowel obstruction is infrequent during pregnancy it may be masked by more commonly occurring emesis or hyperemesis gravidarum. We describe a case of a young woman who presented in second trimester of pregnancy with progressively worsening nausea, vomiting, and abdominal distention. She was diagnosed with a small bowel obstruction, and, upon laparotomy, the transverse colon was fond behind the root of small bowel mesentery, completely obstructed by fibrous bands. Recognizing this reverse rotation and surgical treatment led to successful outcome of the patient and her pregnancy. By reporting this case, we emphasize the role of early recognition of bowel obstruction in a pregnant patient and the role of uncommon developmental anomalies and surgical treatment of reverse intestinal rotation.A 27-year-old primigravida at 25th week of gestation was admitted to the OBGYN Department of Rabin Medical Center, Israel, with nausea, vomiting, and abdominal pain. Her past medical and surgical history were unremarkable. During the past three weeks, she complained of nausea and vomiting, which were attributed to hyperemesis gravidarum. During this period, she was seen several times in the emergency room and treated symptomatically with intravenous fluids and antiemetics. On physical examination, she was afebrile, with stable vital signs, frequently vomiting jejunal contents. Her abdomen was compatible with gestation age, but distended and tense. She did not pass flatus for three days. Plain abdominal X-ray film showed distended small bowel loops in the upper abdomen and gasless lower abdomen with fetal bony silhouette . After nAt laparotomy, the duodenum was situated on the right of midline without being curved behind the mesenteric vessels . There wAfter the surgery, the patient's postoperative course was complicated by a prolonged paralytic ileus. She was placed on Total Parenteral Nutrition for ten days. Her condition gradually improved, ileus resolved, and she was discharged on the 14th postoperative day. She completed her pregnancy uneventfully and gave birth to a healthy child. Now, she is two years after the surgery and remains completely asymptomatic.obstruction of the transverse colon due to compression by the superior mesenteric vessels;volvulus of the mobile right colon or the entire midgut;duodenal-jejunal obstruction.Our case represents the first type of reversed rotation. In 1927, Donald described a case of reversed rotation in a pregnant woman. In his paper, the woman had volvulus of small and large bowel [Midgut rotation occurs in two stages. During the first stage, the midgut begins its counterclockwise rotation and herniates through the umbilical cord. In the course of the second stage, the midgut normally completes its rotation and reduces back into the abdominal cavity. Developmental abnormalities of this process may occur at any stage of this process with midgut volvulus being the most common . Reversege bowel . To the Here, we present a rare case of reverse intestinal malrotation in a young pregnant woman. The clinical recognition of the bowel obstruction was delayed in her case due to natural disinclination to perform potentially teratogenic radiological investigations in pregnancy and nonspecific nature of her complaints, mimicking more common hyperemesis gravidarum. She was taken to the operating room based on clinical and radiological ground with suspected mechanical bowel obstruction. The surgical exploration was challenging because of distended bowel and gravid uterus, obscuring lower abdomen and pelvis. After the malrotation was identified and obstructed malpositioned colon was found behind the small bowel mesentery, we performed a simple release of the constricting bands by dissecting small bowel mesentery off its retroperitoneal attachment. It was not possible to perform antemesenteric reposition of the transverse colon because it was almost fused to the posterior abdominal wall with a short mesentery. We considered right colectomy in order to liberate the large bowel from its unfavorable retromesenteric location, but, given the patient general condition, emergency nature of the procedure during pregnancy, distended bowel, and poor nutritional status due to relatively long standing bowel obstruction, we opted for simple lysis of constricting bands and the colon was left in situ. This operative approach worked well for the patient with resolution of the acute obstruction and no further events during followup. Simple release of constricting bands without antemesenteric transposition seems to be the safest option in this situation.


Visceral artery pseudoaneurysms are rare and only few cases have been reported. They are considered to be life threatening in case of rupture. Rapid treatment is mandatory and endovascular procedure is recommended as the treatment of choice. Occasionally, endovascular approach is difficult to achieve, owing to unusual vascular anatomy. Whenever it is the case, an alternative method has to be considered. We report the case of a jejunal artery pseudoaneurysm that required an access via collateral vessels to accomplish complete occlusion in a 34-year-old woman who presented with a sudden epigastric pain 14 days after a cephalic duodenopancreatectomy. Jejunal artery pseudoaneurysms are known to be rare and only few cases have been reported , 2. As oA 34-year-old female patient presented a jejunal artery pseudoaneurysm after cephalic duodenopancreatectomy (CDP), which required an access via collateral vessels to accomplish complete occlusion. The patient was addressed to our institution for a long-lasting history of idiopathic chronic pancreatitis and was submitted to CPD with the intention to treat a severe recurrent abdominal pain syndrome associated to stenosis of the main pancreatic duct. During the recovery period, pulmonary embolism was diagnosed and the patient received full dose anticoagulant therapy. On day 14, she presented acute epigastric pain and abdominal CT imaging demonstrated a well-defined 1.7 cm pseudoaneurysm of the first jejunal branch close to the origin of the superior mesenteric artery (SMA) . After mThe most frequent sites of visceral pseudoaneurysms are splenic, hepatic, SMA, and celiac trunk. Other sites, such as gastroduodenal, pancreatic-duodenal, inferior mesenteric, and jejunal artery, are rare. Surgical resection, ligation, and bypass were historically the treatments of choice for visceral pseudoaneurysms, but nowadays current therapeutic modalities consist of percutaneous transcatheter techniques such as embolization  and prosThe technique of transcatheter superselective coil embolization may be sometimes complex to obtain because of vascular anatomy, tortuous nature of vessels, or problematic superselective catheterization. Sometimes, a second direct catheterization of the pseudoaneurysm is not possible to perform, even after several attempts, as experienced by the authors with this case. At this situation, some possibilities should be considered, such as placement of stent-graft, entire vessel embolization, percutaneous direct pseudoaneurysm puncture, or surgical approach. Placement of stent-graft may be problematic in pseudoaneurysms that originate from SMA or jejunal branches. Since it could result in occlusion of other normal vascular branches next to the pseudoaneurysm by extension of the stent-graft, the risk of intestinal ischemia is significantly increased. Furthermore, stent-graft can be complicated by occlusion and it usually requires prolonged postprocedure anticoagulation therapy. Alternatively, direct percutaneous puncture of the pseudoaneurysm followed by injection of cyanoacrylate or thrombin can be obtained under ultrasound or CT guidance. However, this particular technique may be difficult due to the localization of such pseudoaneurysms and interposition of intestinal loops. In this case, the pseudoaneurysm was located posterior to the main trunk of the SMA and, thus, difficult to reach by direct percutaneous approach.The well-known procedure usually performed to treat splenic pseudoaneurysms involves isolation and exclusion of the afferent and efferent arteries close to the pseudoaneurysm. This method is not suitable for a pseudoaneurysm of the SMA, because of the high risk of intestinal ischemia.In this case, superselective catheterization of the pseudoaneurysm by proximal approach was initially obtained, but complete embolization was not achieved . SubsequEmbolization of a splenic aneurysm via collateral vessels was already reported . These aThe present case report is exquisite due to the rarity of jejunal artery pseudoaneurysms and the unusual approach used to occlude this vascular anomaly. However, it shows that when the initial treatment strategy has to be reconsidered during the procedure, in order to avoid an emergency laparotomy with related high morbidity rate, transcatheter embolization via collaterals vessels should be explored.


Clavicular bone tumors occur in less than 0.5 percent of bone tumors. Primary chondrosarcoma is very rare even among clavicle tumors. The main symptom is a touchable mass in 69 % of patients. Dedicated centers using FNA and cytology can reach a correct diagnosis in 94% of cases. Treatment planning is done using simple X-ray, CT-scan, shoulder MRI, chest CT-scan and whole body technetium scan. Treatment of choice for primary chondrosarcoma of clavicle is surgical resection. They occur mainly in pelvis, femur, homerus and scapula.2 The patient was a 22 year old woman who was referred to the surgery ward early in the February 2014 from the orthopedic ward for multidisciplinary surgery. Patient had a mass in anterior thorax and clavicle, discovered 6 months earlier. It had grown steadily and was not painful at first but the patient complained of pain at the time of referral. She had no history of alcohol or drugs, nor did she have a history of smoking. She had no family history of known diseases.In examination, a large mass with the dimensions of 10*6 centimeters was discovered, taking up two thirds of the clavicle. Head, neck, heart, lungs, organs and the pulse were normal in physical examination.Needle biopsyThe patient underwent core needle biopsy and was diagnosed as grade II chondrosarcoma.CT scan and MRI In CT scan and MRI, a 91*57*59 millimeters mass was observed, proximal. There was no metastasis .Due to the tumor’s situation and its proximity to neurovascular network, the patient was sent to Shariati Hospital's surgery ward to be operated on by a multidisciplinary team consisting of thoracic, vascular, orthopedic surgeons. Decision on surgical resection of the mass was made after a review meeting between the mentioned surgical team and oncologists.Pathology reportPathologic stage is definitely important in prognosis, grade I chondrosarcoma has low malignant nature while grade II and grade III chondrosarcoma are high malignant. Five year survival in grade I is 90%, it falls to 60% for grades II and III. Chondrosarcoma is a malignant tumor result of neoplastic chondrogenesis.The other reason for delayed diagnosis is FNAC (needle biopsy) not giving definite answers; in non-specialized centers only 26% of diagnosis is accurate. In specialized centers this number rises to 94%.   8 4,5  In patients who undergo wide resection, less recurrence has been observed, recurrence mostly occurs in the first five years after surgery.   10 In most cases, chondrosarcoma shows up as a radiolucent lesion with indistinctive edges, calcification is observed as well.Total claviculectomy was performed on our patient, a quarter of sternum was resected alongside the tumor due to the tumor's position  and so that proximal margin and distal margin were free of tumor , 5 and 6   11  Total claviclectomy is the best treatment choice when the complete cure is intended.Unlike Osteosarcoma and Ewing sarcoma, chondrosarcoma does not answer to chemotherapy and radiotherapy.Follow-upOne year after surgery and because of close margin, the patient was referred to medical and radiation oncologist for adjuvant chemotherapy and radiotherapy. To summarize, in a patient with a clavicle tumor, needle biopsy's results should not be trusted even in the absence of pain, if the tumor comes up as benign, the test should be repeated. The main treatment for primary chondrosarcoma of clavicle is surgery.


Purpose. Tailgut cysts with malignant transformation are rare entities. We discuss the diagnostic strategy and treatment of a malignancy within a tailgut cyst. Methods. In this study we report on the case of a 61-year-old man with a malignant neuroendocrine tumour arising within a tailgut cyst and an overview of the literature emphasising the histopathological characteristics and differential diagnosis. Results. Our patient presented with lower back pain, rectal pain, and increased urgency of defecation. MRI scan and CT-guided biopsy on histological analysis revealed a diagnosis of carcinoid tumour of the presacral space. The patient subsequently underwent an abdominoperineal excision of the rectum. Conclusions. This case highlights the importance of tailgut cysts as a differential diagnosis of presacral masses. It is a rare congenital lesion developing from remnants of the embryonic postanal gut and is predominantly benign in nature. Approximately half of cases remain asymptomatic; therefore, diagnosis is often delayed. Magnetic resonance imaging is the investigation of choice and an awareness of the possibility of malignant potential is critical to avoiding missed diagnosis and subsequent morbidity. Complete surgical excision allows accurate diagnosis, confirmation of oncological clearance, and prevention of mortality. Tailgut cysts are rare developmental cysts, which occur in the presacral region. They have also been described as retrorectal cysts, cyst of postanal intestine, retrorectal cystic hamartomas, simple cysts, mucus secreting cysts, and myoepithelial hamartoma of the rectum –3. It isApproximately 23 cases of malignant transformation in tailgut cysts have been reported in the literature, of which eleven have been carcinoid tumours. They are mainly reported in middle-aged women, are usually asymptomatic, and are found during routine physical examination or at childbirth; therefore, diagnosis is often delayed. We describe a case in which a 61-year-old man presented with a three-week history of dull aching pain in his lower back, rectal pain, and increased frequency of defecation. Following abdominoperineal excision of the rectum, the presence of a tailgut cyst was established. Arising within this was a low grade epithelial neoplasm with morphological and immunohistochemical evidence of neuroendocrine differentiation.A 61-year-old man presented with a history of dull aching pain in his lower spine, rectal pain, and increased urgency of defecation lasting for three weeks. Abdominal examination and digital rectal examination were unremarkable. A barium enema showed extrinsic compression of the lower rectum from a presumed low pelvic mass and two persistent filling defects within the descending colon. Colonoscopy showed a pedunculated polyp at 50 cm. Biopsies of this polyp confirmed it to be a tubulovillous adenoma with mild dysplasia. The rectal mucosa was normal. CT-guided biopsy of the presacral mass initially suggested a prostate cancer. However, on review of the histology and scan, a carcinoid tumour of the presacral space was diagnosed. An MRI of the pelvis carried out showed a loculated mass 6 × 4.3 × 4.8 cm, proximal to the lower rectum. The rectal mucosa was found to be intact whilst the serosal surface was inferiorly breached and in direct contact with the mass. The mass displaced the inferior aspect of levator ani laterally and there was evidence of direct extension into muscle on the left side. Staging CT scan did not show any evidence of distant metastases. The patient underwent abdominoperineal excision of the rectum.Microscopy of the CT-guided biopsies of the presacral mass showed fibrous tissue extensively infiltrated by an epithelial neoplasm. The tumour was moderately to poorly differentiated and had a focally cribriform growth pattern. Despite being described as moderately to poorly differentiated, the tumour had a low Ki-67 index with no necrosis or other features of poor prognosis. Immunohistochemical staining of the tumour cells strongly expressed prostatic acid phosphatase and showed weak patchy CD56 positivity. Furthermore, it revealed strong and diffuse positivity for neuroendocrine markers: synaptophysin and chromogranin. The tumour cells also coexpressed the cytokeratin MNF116 and showed patchy AE1/3 positivity.Macroscopically, the surgical resection specimen showed a tumour mass measuring 59 × 42 × 27 mm which was attached posteriorly to the rectum in the midline, close to the distal margin. The tumour surface had a grey-white appearance, and yellow discoloured areas were present . DistallTailgut cysts are rare congenital retrorectal lesions. They are thought to develop from the remnant of the tailgut , a primitive gut which is temporarily present at the caudal portion of the embryo . During Tailgut cysts are found in adults and children but are predominantly reported in middle aged women with a female to male ratio of 3 : 1 , 7. TailOn macroscopic examination of excised specimens tailgut cysts are typically well circumscribed . These cDiagnosis based on biopsy can be difficult as biopsy specimens commonly contain inflamed fibrous tissue with no epithelia or only one type of epithelium, making it difficult to determine whether the mass is a tailgut cyst or another type of developmental cyst . MalignaThis case highlights the importance of tailgut cysts as a differential diagnosis of presacral masses as malignant transformation can occur and result in mortality if surgery is not carried out , 6. Diag


The case is a 34-year-old woman with long-standing type 1 diabetes mellitus with existing follow-up in the outpatient clinic at the Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, UHCW. She had maintained good glycaemic control and glycaemic stability with basal bolus regimen for many years. She had not developed any diabetes-related complications and had no other co-morbidities. Six months ago, she presented to A&E with sudden-onset, well-localised and severe pain in the right iliac fossa, just lateral to the para-umbilical area. Her biochemistry was normal. Ultrasound scan, however, revealed a right-sided ovarian cyst, which was thought to have caused pain to her. She was discharged from A&E with simple analgesia. On subsequent gynaecological follow-up 4 weeks later, her pain remained severe and examination revealed an exquisitely tender subcutaneous nodule at the same location measuring 2 cm in diameter. Magnetic resonance imaging (MRI) scan at the time revealed a 1 cm mass in the subcutaneous adipose tissue, which co-localised to her pain. The mass demonstrated a central fat signal surrounded by a peripheral ring: observations consistent with fat necrosis. There were other smaller subcutaneous nodules also observed in the left para-umbilical area. Subsequent surgical resection of the main area of fat necrosis was performed. The patient made an excellent recovery and her pain resolved post-operatively. Histology confirmed the presence of fat necrosis. Fat necrosis is a rare complication of s.c. insulin injection. This case illustrates the importance of considering this diagnosis in patients who inject insulin and develop localised injection-site pain.Fat necrosis is a rare complication of insulin injections that can manifest with severe, persistent and well-localised pain.Fat necrosis can masquerade as other pathologies causing diagnostic confusion.The imaging modality of choice for accurate diagnosis of fat necrosis is MRI.Histological confirmation of fat necrosis is important.Appropriate management of localised fat necrosis is surgical excision, with avoidance of further insulin injections into the affected area. Diabetes mellitus (DM) is very common. The prevalence of DM overall is increasing, and the number of patients with DM who inject insulin therapies is also increasing. Problems associated with insulin injections, including erythema, pruritus and lipohypertrophy, are relatively common and often mild and transient in their severity and duration respectively. However, we report a case of an extremely rare complication relating to insulin injections, and one that was associated with severe, persistent and localised pain with substantial adverse impact on lifestyle: superficial fat necrosis. Seemingly innocuous when viewed on an magnetic resonance imaging (MRI) scan , the nodule caused the patient much distress over a prolonged period.The importance of our case is that it raises awareness among the relevant healthcare professionals of superficial fat necrosis as a rare complication of insulin injections in patients with DM, the difficulties associated with its diagnosis and the importance of MRI scanning and prompt surgical excision as a highly effective treatment modality.The case is that of a 34-year-old lean female nursery nurse who had been followed-up in the Diabetes Clinic at WISDEM centre, UHCW. She had longstanding type 1 DM (T1DM), for which she was being treated with a basal-bolus regimen of insulin injections . She had a good injection technique and had had no problems with her insulin therapy. She had good glycaemic control and stability. She had not developed any diabetes-related complications and her past medical history was otherwise nil of note. There were no other medications and no known allergies.Six months ago, and a few weeks following her diabetes outpatient review at WISDEM centre, she developed sudden-onset, well-localised, persistent and severe sharp pain in the right iliac fossa, just lateral to the para-umbilical area. The pain did not radiate but was worsened by activity  and relieved by simple analgesia. The pain was associated with nausea and occasional dizziness. There were no other symptoms relating to the gastrointestinal, urinary or reproductive systems.in situ. A pelvic ultrasound scan revealed a right-sided ovarian cyst. Following discharge from A&E with simple analgesia, she had subsequent gynaecological follow-up 4 weeks later. At this time, her pain remained severe and constant, often requiring her to take time off from her work. Menstrual history revealed regular periods and no other symptoms suggestive of gynaecological pathology. On examination, she was noted to have an exquisitely tender subcutaneous nodule measuring 2 cm in diameter, co-localising to the site of her pain. An MRI scan was arranged, following which she was seen in the Diabetes Clinic.On presentation to A&E, it was noted that her appendix was still Clinical assessment in the Diabetes Clinic did not reveal any history of abdominal wall trauma or pancreatitis. Her injection technique was observed to be good, and she confirmed that she had been rotating her insulin injection sites in her thighs and abdomen, but had been avoiding abdominal insulin injections as she developed pain. On examination, presence of a localised tender abdominal subcutaneous nodule was confirmed. There was no guarding and abdominal examination did not reveal any other abnormalities.During her A&E assessment, a pregnancy test was found to be negative, and her biochemistry was normal . She was noted to have excellent blood glucose control with a recent HbA1c level of 47 mmol/mol. There was no indication of any infective or inflammatory pathology. A transvaginal ultrasound scan showed a 27 mm right-sided ovarian cyst, with no free fluid in the abdominal cavity. The ovarian cyst was thought to be the cause of her symptoms. Repeat ultrasound scan during subsequent gynaecological review confirmed the presence of a simple 10 mm physiological cyst on the right ovary and no other abnormalities in the region of interest.A subsequent MRI scan  revealedMacroscopically, histopathology of the excised lesion revealed ragged fatty tissue fragments measuring 53 mm in aggregate. Microscopically, there were small areas of fat necrosis, fibro-hyaline scar tissue, focal foreign body-type giant cells and small amount of foreign materials. Features were in keeping with the clinical diagnosis of fat necrosis .Following her MRI scan and diagnosis of fat necrosis, she was referred urgently for surgical opinion. She proceeded to surgical excision of the fat necrosis nodule and was discharged the following day after her operation with no complications. She maintained stable glycaemia throughout.Post-operatively, she made an excellent recovery. On further follow-up, her surgical wound has healed well. She has been pain-free since her operation, and there has been no need for her to take any further analgesia. She has not developed any further painful nodules. She continues to rotate her insulin injection sites.Complications relating to insulin injections, including erythema, pruritus and indurations, are relatively common and can interfere with metabolic control . Lipohypertrophy, presenting as a soft non-tender dermal nodule with associated fat cell hypertrophy, is the commonest complication relating to insulin therapy Fat necrosis, first described in the breast in 1920 Most case reports of fat necrosis in the literature are linked to trauma Perhaps, owing to the apparent rarity of fat necrosis resulting from insulin injections, this possibility was not even entertained when our case first presented to A&E with abdominal pain. Instead, her pain was wrongly attributed to an ovarian cyst, illustrating the potential for superficial fat necrosis to masquerade as other pathologies, especially when affecting the abdominal subcutaneous adipose tissue. What was striking about the clinical presentation of our case though was the severity and persistence of her pain, lasting for months. Suggestive features of localised fat necrosis, illustrated by our case in her presentations following her initial A&E attendance, include extreme tenderness over the site of her pain, associated with a nodular swelling. Our case illustrates the utility of MRI used diagnostically for fat necrosis, and the importance of subsequent histopathology. Our case demonstrates the effectiveness of surgical excision in such cases, and the excellent clinical response to such management, our patient being pain-free following her operation.Although rare, and with very few published similar case reports, it remains possible that fat necrosis resulting from insulin injections is under-recognised and under-reported. It is possible that cases with milder and more transient painful symptoms are not reported by patients. It is important though for healthcare professionals to be mindful of fat necrosis as a possible complication of insulin injections and to institute prompt and appropriate imaging investigations and surgical management wherever possible in those patients with localised and persistent superficial nodular pain and tenderness at injection sites.Informed consent was obtained from the patient for publication of this (anonymised) submitted case report and accompanying images.I confirm that all co-authors listed contributed substantially to the preparation of this manuscript. The corresponding author (Dr T M Barber) is the named physician of the patient.


Papillary fibroelastoma (PFE) is a rare primary cardiac neoplasm that is usually discovered incidentally at autopsy or during cardiac surgery. PFE combined with rheumatic heart disease (RHD) is extremely rare, and only a few cases have been reported. Additionally, the growth rate of the tumor is unknown.Here, we present a very rare case of PFE of the aortic valve combined with RHD, which were identified in a female patient who survived for 5 years without surgical intervention, and who subsequently underwent successful surgical treatment.PFEs may be generally slow-growing tumors, however, the better treatment of choice may be surgery because it produces good curative effects with very low risk of complications, while preventing serious disease consequences. Although papillary fibroelastoma (PFE) is rare, it is the second most common primary cardiac neoplasm, accounting for 4.4 % to 8 % of all tumors of the heart . PFE is Here, we present the case of a 55-year-old female patient who had a PFE of the aortic valve in combination with rheumatic heart disease (RHD). She was diagnosed with RHD in 2010. Although transthoracic echocardiography revealed a mass on the non-coronary cusp at that time, the patient initially refused surgery for economic reasons. A surgical intervention was ultimately performed 5 years later. In the intervening time, she did not develop any symptoms related to the PFE, which did not grow significantly. We view this case as being instructive, both because PFE rarely appears in combination with RHD, and because few clinicians are able to observe the natural history of surgically untreated PFE over such a long interval of time.A 50-year-old woman was first admitted to our hospital in 2010, when transthoracic echocardiography (TTE) revealed a mass on the non-coronary cusp for the first time , moderate mitral regurgitation, mild tricuspid regurgitation, mild to moderate aortic valve regurgitation, and a round mass (6 × 5 mm) on the aortic side of the non-coronary cusp  functional class of III was admitted with the diagnosis of RHD presented as exertional shortness of breath  accompanied by occasional palpitations and dizziness. The patient denied any chest pain, orthopnea, or paroxysmal nocturnal dyspnea. She denied any history of cardiac tumors, coronary artery disease, pulmonary disease, or cancer, as well as smoking, alcohol consumption, or use of illicit drugs. Physical examination showed blood pressure of 130/65 mmHg, temperature of 36.7 °C, and heart rate of 76 beats/min. Findings of chest radiography were normal, while electrocardiography detected atrial fibrillation. The results of routine blood laboratory investigations were unremarkable. TTE revealed moderate mitral stenosis  was found on the aortic side of the non-coronary cusp Fig. . BecauseHistopathological examination of the resected tumor revealed avascular papillomas with a single layer of endocardial cells covering the papillary surface. The connective tissue of PFE contains mature collagen with irregular elastic fibers that are oriented longitudinally Figs.  and 4. TPFE is a rare, benign cardiac neoplasm with an estimated frequency of 0.021 % in autopsy series . PFE is The natural history of PFE has not been defined because longitudinal follow-up studies have not been performed. In this unusual case, the patient provides us with the opportunity to learn about the natural history of PFE indirectly. The tumor did not grow significantly during the 5-year period, indicated that PFEs may be generally slow-growing tumors.Although the cause of PFE remains unknown, Kurup and colleagues have noted that PFE could potentially be related to cardiovascular intimal trauma. A history of heart surgery or radiation to the chest may cause PFE . RegardiMost PEFs have been asymptomatic, and they have usually been discovered incidentally at the time of echocardiography, cardiac surgery, or autopsy . Patient For symptomatic patients, especially for those with mobile tumor, surgical resection with or without valve replacement is recommended. These patients are at high risk for thrombosis, which may be life-threatening, whereas surgical removal poses low risk and the outcome is excellent , 10. HowDuring the 5-year period passed between her initial presentation and the second admission, our patient did not take anticoagulants and did not suffer any thrombosis, which, however, does not mean that it is completely safe to leave PFE without treatment. In our opinion, the better treatment of choice may be surgery because it produces good curative effects with very low risk of complications, while preventing serious disease consequences. Still, further studies are required to determine the best treatment for PFEs.Written informed consent was obtained from the patient for the publication of this case report and any accompanying image. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Following the treatment, a marked reduction in the tumor size was observed with a tolerable level of toxicity. The sequencing analysis also revealed an in-frame deletion (delE746-A750) in exon 19 of the epidermal growth factor receptor gene. Based on this result, gefitinib was administered to the patient at a dose of 250 mg/day.Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma, but rarely originates in the chest wall. Surgical resection is considered to be the most reliable treatment, however, no consensus has been reached concerning the best treatment for unresectable MFH. The current study presents the case of a 77-year-old male with MFH of the chest wall. The patient developed a painless mass and intermittent fever over a four-month period. A computed tomography scan demonstrated a large inhomogeneous lesion in the right chest wall, which was subsequently diagnosed via biopsy as a MFH. Since the tumor was an unresectable mass, CyberKnife The patient’s medical history included prostatic hyperplasia, cholecystolithiasis and a cyst of the right kidney. The patient had no history of smoking or asbestos exposure. The tumor was elastic-hard, tender and characterized by a distinct border between the tumor and the normal tissue. The laboratory examinations showed mild anemia, hyponatremia, hypoalbuminemia and a high C-reactive protein concentration. Additionally, the levels of tumor markers, including carcinoembryonic antigen, carbohydrate antigen 19-9, neuron specific enolase and squamous cell carcinoma antigen, were within the normal limits. Computed tomography (CT) revealed a well-defined inhomogeneous mass in the right chest wall and the lesion showed a heterogeneous isointense signal on T1-weighted magnetic resonance imaging (MRI) and a high-intensity signal on T2-weighted MRI. The CT and MRI observations indicated the diagnosis of MFH or myxoid type liposarcoma. In addition, a percutaneous needle biopsy was performed and yielded three tissue fragments from the mass; grossly, the tissues appeared red and soft. The pathological slices was visualized using an Olympus BX31 microscope  at ×100 and ×400 magnification. Microscopically, a storiform pattern of spindle cells and multinucleated giant cancer cells with atypical mitotic figures were observed, as well as hemorrhaging and necrosis. Furthermore, the immunohistochemical studies were negative for cytokeratin 5/6 and calretinin, but positive for vimentin ; therefo®  technology, which is a robotic, image-guided, full-body RT system. The system was equipped with the Synchrony® Respiratory Tracking Device (Accuracy Corporate), which enables the CyberKnife® technology to compensate for any tumor motion; however, the device requires the insertion of gold fiducial markers close to the tumor. Briefly, patients must have three gold fiducial markers (3×0.8-mm gold seeds) implanted inside the tumor to determine the tumor position. The fiducial markers may be placed by percutaneous needle placement using an 18-/19-gauge coaxial needle under image guidance and local anesthesia. Approximately one week following the placement of the fiducial markers, CT simulation was performed for the purpose of treatment planning. This time interval is sufficient to allow resolution of the edema and to identify any injury that may have occurred during the fiducial placement, as well as to allow for fiducial stabilization. In the present study, the MFH of the right chest wall measured 14.8×8.1 cm, with a 1,097-cm3 volume. The patient was subsequently prescribed an RT dose of 75 Gy to the 75% isodose line, which was delivered in five fractions. In addition, the volume of the right lung receiving ≥20 Gy was 15%.SBRT was performed using CyberKnife® RT, the tumor had reduced from 14.8×8.1 to 10.9×4.5 cm in size and notably, the solid lesion of the mass had significantly reduced , trunk (16%) and retroperitoneum (16%), however, rarely presents in the chest wall . The maj%, trunk et al  for the palliation of a patient with an MFH arising in the chest wall. No grade 3 or higher toxicity was observed in the patient, which indicates that this technique may be a useful treatment for unresectable MFH of the chest wall. However, further study is required to determine the optimal target dose, long-term toxicity and efficacy of this approach.In conclusion, the present study reports the feasibility of SBRT (specifically, CyberKnife


Cavernous hemangiomas of the gastrointestinal tract are quite rare and, until now, have been difficult to diagnose preoperatively due their nonspecific presentations and imaging features, as well as a lack of histologic description pertaining to small superficial biopsies such as those obtained endoscopically. We report a unique case of a 4 cm transmural cavernous hemangioma in the terminal ileum with literature review and describe a new histologic finding—the “endothelialized muscularis mucosae,” which was discovered upon review of the endoscopic biopsy and could potentially facilitate preoperative diagnosis of these lesions from endoscopic biopsies in the future. These lesions have classically required surgical resection in order to make a definitive diagnosis and rule out malignancy, with which they share many historical and radiographic features. Due to their potential to cause bowel obstruction, intussusception, perforation, and hemorrhage, these lesions may ultimately require surgical resection to relieve symptoms or prevent or treat complications—however, surgical planning and patient counseling could be greatly improved by a preoperative diagnosis. Therefore, gastroenterologists, pathologists, and surgeons should be aware of the “endothelialized muscularis mucosae” which can be very helpful in diagnosing GI cavernous hemangiomas from endoscopic biopsies. Cavernous hemangiomas of the gastrointestinal tract are quite rare and, until now, difficult to diagnose preoperatively  due to aThe patient was a 20-year-old female who presented to the emergency department with a progressive 2-week history of diffuse intermittent abdominal pain, which was aching at rest and sharp upon movement. It was accompanied by nausea for the past two days, with one episode of vomiting. She denied recent changes in her stool but reported passing stool only once per week for the past few years.The patient also reported a 2-year history of vasovagal syncope, with an increase in presyncopal episodes during the 2-week course of her abdominal pain. These episodes were sometimes associated with heart palpitations, shortness of breath, and chest pain, which had previously been diagnosed as anxiety and gastroesophageal reflux disease. The history was otherwise noncontributory.The patient's vital signs were unremarkable and she was afebrile. There were no masses or tenderness upon abdominal palpation. Initial labs were within normal limits, and screening tests were negative for pregnancy and sexually transmitted infections.A computed tomography of the abdomen and pelvis without contrast was notable for a 4.7 cm mass involving the wall of the terminal ileum, with speckled calcifications, adjacent lymphadenopathy, and a small amount of dense fluid in the pelvis . No hepaThe patient subsequently underwent laparoscopic evaluation and resection of the mass. Upon entry into the abdomen, a small amount of blood was noted to be pooled in dependent locations within the abdomen and pelvis. At the terminal ileum, a soft, maroon, cystic, friable mass associated with scant bleeding and a tortuous, dilated blood supply was noted . A laparThe resected specimen was composed of an 11 cm segment of terminal ileum and 4 cm portion of cecum . Gross eReview of the original biopsy revealed a second layer of fibromuscular tissue apposed to the muscularis mucosae of the small bowel mucosa. On the antiluminal side of the intestinal lumen, this second layer of fibromuscular tissue was lined by a layer of flat endothelial cells . ImmunohIntestinal hemangiomas are very rare, with multiple histologic variations accounting for a combined incidence of only 0.05% of all gastrointestinal tract (GI) tumors . They arSmall bowel tumors pose several diagnostic challenges, beginning with their nonspecific symptoms of abdominal pain, nausea, and distension, which could be more easily explained by many prevalent physiologic GI issues. Additionally, many are asymptomatic until later stages. Therefore they are often discovered either incidentally, or after a wide-ranging work-up and a significant delay since the onset of symptoms . Late stCompared to other small bowel tumors, cavernous hemangiomas are more often symptomatic and more likely to present with anemia or bleeding, rarely even causing massive hemorrhaging , 6, 11. Nonspecific features on imaging and limitations in visualization pose additional challenges to preoperative diagnosis of small bowel tumors. Patients who present with abdominal pain or obstructive symptoms are often evaluated by computed tomography (CT), which has a poor detection rate for small bowel tumors and is unlikely to visualize most cavernous hemangiomas , 12. ForPatients who present with recurrent anemia or GI bleeding due to these lesions are often evaluated by traditional endoscopy, which is unable to reach most of the small bowel, or by capsule endoscopy . When thLittle has been written in the literature on the histologic findings in biopsies of cavernous hemangiomas of the gastrointestinal tract. Accordingly, even lesions accessible for biopsy have evaded diagnosis due to inconclusive findings , and cavWhile cavernous hemangiomas are benign, they can be locally destructive by way of invading, exerting pressure, or disrupting the physiology of surrounding structures. Due to these factors and the prevalence of complications such as bleeding or bowel obstruction, cavernous hemangiomas require surgical resection more often than other intestinal hemangiomas . SurgeryDue to the nonspecific presentation, many patients with GI cavernous hemangiomas will be evaluated with endoscopy at some point, if not early on in their work-up . When thSmall bowel cavernous hemangiomas are extremely rare vascular lesions which present with nonspecific GI symptoms such as abdominal pain and nausea, anemia, and GI bleeding. Complications include bowel obstruction, intussusception, perforation, and hemorrhage. Their size and propensity for bleeding may be affected by comorbid patient conditions which impair venous flow. Diagnostic challenges include nonspecific presentation and features on imaging, inconclusiveness of biopsy, or inaccessibility by endoscopy. Pathologists should be aware of the “endothelialized muscularis mucosae” which can be very helpful in diagnosing GI cavernous hemangiomas, particularly in small biopsies. The ability to acquire a histologic diagnosis preoperatively has important implications in patient counseling and surgical planning. A conservative surgical resection, which may be successfully performed laparoscopically, is recommended in order to relieve symptoms or prevent complications and provide definitive diagnosis when necessary.


A full-term male neonate with anorectal anomaly and external perineal anomalies was referred to our service. Physical examination showed an epithelized perineal mass with cutaneous orifices, which had urine fistulization, hipotrofic perineal musculature, bilateral congenital clubfoot, hipospadic urethra, criptorquidy bilateral with nonpalpable testis and imperforate anus. A colostomy was constructed immediately after birth. The child underwent excision of perineal mass, bilateral orchidopexy, Duplay neourethroplasty and coloanal anastomosis at 3 months of age. The histopathological examination of the perineal mass revealed a hamartoma. We report here a case of an otherwise healthy newborn with anorectal anomaly and a perineal mass. Initially, the mass was believed to be a sacrococcygeal teratoma. The patient underwent mass excision and the histopathologic evaluation revealed a benign hamartoma. This case is presented because of its rarity.Neonates presenting perineal masses are uncommon. When encountered, most perineal masses are anorectal malformations, sacrococcygeal teratomas, rectal prolapse, or duplication cysts.A full-term male neonate with anorectal anomaly and external perineal anomalies was referred to our service. Antenatal ultrasound at a gestational age of 20 weeks detected a perineal mass, but other parameters were normal. He was born via spontaneous vaginal delivery after an uncomplicated pregnancy, at a gestational age of 38 weeks and his birth weight was 3,280g. The mother was healthy and did not use any medication during pregnancy nor in the year before pregnancy.The child's physical examination revealed an epithelized perineal mass with cutaneous orifices which had urine fistulization, hipotrofic perineal musculature, bilateral congenital clubfoot, ambiguity of genitalia with a hipospadic urethra, criptorquidy bilateral with nonpalpable testis and imperforate anus .After birth, he presented fecal and urine content trough an urethral orifice and a colostomy was constructed. Subsequent laboratorial investigation showed karyotype 46 XY and normal ultrasound of urinary upper tract. The investigation with magnetic resonance showed a volumous perineal sac extending superiorly to the right obturator, with very thick walls and heterogeneous content with neurovascular pedicle from the right sacral region. In addition, we observed a slightly dilated rectosigmoid communicating with hernia sac, bladder with small repletion and elongated morphology with proeminent urachus. The urethra was poorly characterized, apparently continuing to a micropenis externalized outside of the hernia sac. Ureters had normal caliber. No structure was characterized as follows with their usual morphologies: uterus, ovaries, prostate and testicles . When th–4Association of a perineal hamartoma with anorectal anomaly is uncommon, and the additional presence of criptorquidy and congenital clubfoot turns our case extremely rare. Masses of the perineum in infants primarily consist of accessory or ectopic scrotum or perineal lipomas.Surgical excision of these lesions is generally straightforward. However, when the lesion occurs in a patient with an anorectal malformation and it is at the site where the future anus will be, surgical management may be more complex. Accurate pathological interpretation of the excised specimen is also of great importance. In this review, eight cases of hamartomas were mentioned. All patients were girls and had a variety of different anorectal malformations. The typical lesion was a polypoid protuberance from the perineum at the site where the neoanus was reconstructed. A variety of unusual tissues such as nephrogenic rests, ectopic bone, and endocervical type mucosa were found in these lesions. One patient had initial treatment at a community hospital where the pathologist found perineal glandular tissue (endoderm).A review with 2,000 cases of anorectal malformations revealed 22 cases with association to unusual perineal masses divided into 3 categories of lesions characterized as lipomas, vascular anomalies, and hamartomas/choristomas.In the present case, sacrococcygeal teratoma was a differential diagnosis. In the radiological study, sacrococcygeal teratomas present with soft tissue density and calcifications in 50% of cases , all suggestive of benign tissue. On magnetic resonance imaging these tumors are characterized by hypointense signal on T1 and hyperintense in T2. Magnetic resonance image is considered the method of choice during the preoperative evaluation and it provides exact boundaries, topographic location, relationship to other pelvic organs and spine. Stephens attempted to explain the embryogenesis of these anomalies including perineal anorectal malformations through a deformation by an abnormal pressure on the developing fetus. This pressure occurs in complicated pregnancies by oligohydramnios. The results of this pressure are skin dimples or joint contractures.The association of hamartoma perineal with anorectal malformations is uncommon, and with genital abnormalities is extremely rare. Various hypotheses have been developed to explain the association between these pathologies.,7 It has been postulated that the normal development of the urogenital system and anorectum in human embryos may depend on the integrity of the mesenchymally-derived urorectal septum. Once the patient has an hipotrofic perineal musculature, both urinary and bowel continence may be affected. Because of the complexity of the case we report, long term results are difficult to predict.The presentation of perineal mass with anorectal malformations, scrotal abnormalities and genital malformations, suggests possible growth of embryonic tissue structures of the phallus, from the urethral fold and genital, anal and perineal region that develop these events.


Paratesticular liposarcoma is a rare tumour of the genitourinary track but the most common of all sarcomas in adults. The dedifferentiated variation occurs only in 10% of liposarcoma cases. The typical clinical presentation is similar to an inguinal hernia or a benign lipoma. We present the case of a dedifferentiated paratesticular liposarcoma with osseous metaplasia of the spermatic cord, in a male presented with acute scrotum. Liposarcoma is a malignant neoplasm of the adipose tissue representing the second most common type of sarcoma following malignant fibrous histiocytoma (20% of the cases) . It is tα-fetoprotein (AFP), human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH)) within normal limits. Radiological evaluation included scrotal ultrasonography (US) and computed tomography (CT) scan. US scan revealed a right-sided solid paratesticular mass with normal testes , HHF-35 (−), vimentin (+), C-kit (−), desmin (+), SMA (+), and CD 34 (+). Cytogenetic evaluation in substains showed strong and diffuse nuclear positivity for murine double minute 2 (MDM2) and cyclin-dependent kinase-4 (CDK4) consistent with translocation of these genes on the long arm of chromosome 12. These findings were suggestive of a low grade dedifferentiated liposarcoma (DDL) . A supplDue to high comorbidity , the patient was not submitted to adjuvant chemotherapy as decided in our multidisciplinary tumor board. Additionally, the option of local radiotherapy was not considered beneficial. Therefore an intensive follow-up schedule was applied, with chest/abdomen CT scans every 3 months for the first year and every 6 months for the second year. Two years after the initial diagnosis there is no local or systematic recurrence.Liposarcomas are classified according to WHO classification of soft-tissue tumors 2013) in four major subtypes: atypical lipomatous tumor/WDL, DDL, myxoid liposarcoma, and pleomorphic liposarcoma 013 in fo–7. DedifThe clinical presentation of DDL typically includes a large painless mass, which may be incidentally detected (particularly in the retroperitoneum). Genitourinary localization of DDL has been scarcely described in the literature while other subtypes represent the most common diagnosis of genitourinary sarcomas, in adults. Occasionally, scrotal pain or discomfort can occur due to rapid mass enlargement or infection . CT or MA pretreatment surgical or US-guided needle biopsy is also indicated to diagnose the type and the grade of the sarcoma. Paratesticular sarcomas should be treated by radical orchiectomy with high ligation of the spermatic cord . In the External adjuvant radiation therapy is a controversial option. There are several studies published regarding combination therapy, in large retroperitoneal liposarcomas and the results were beneficial for the patients. Other studies reported that the administration of adjuvant radiotherapy did not seem to confer survival benefit. As far as paratesticular liposarcoma is concerned the data are limited. The trend is that adjuvant radiotherapy should be used in patients with positive surgical margins or when the adequacy of local excision is in doubt. In these cases there is a decrease in local recurrence up to 44% but there is no impact on the overall survival rate at 5 years . DefinitSystemic chemotherapy should be given to patients with evidence of retroperitoneal or distant metastases. A variety of chemotherapeutic agents and combinations are available based mainly on doxorubicin, ifosfamide, mesna, and dacarbazine . LimitedThere are several survival rates reported, depending on the histologic subtype, grade, size, surgical margins status, and the primary site of liposarcomas. Five-year survival rates of paratesticular liposarcomas range from 20% to 80% while the long-term survival of men with all paratesticular sarcomas is approximately 50%. Dedifferentiation is mainly considered to be a poor prognostic factor especially for local recurrence whereas well-differentiated recurrences may be noted. Low grade is not a prognostic factor in these types and metastatic risk is up to 20% .An intensive follow-up schedule is worthwhile for these patients. Radiologic evaluation with CT or MRI is highly recommended at least every 3–6 months for the first 3 years and annually thereafter .In our case, the patient had a low grade paratesticular DDL that was surgically excised with negative surgical margins. The osseous metaplasia was not considered to confer any aggressiveness to the tumor. The radiologic evaluation was negative for lymph node disease or distant metastasis. The patient had significant comorbidities and medium performance status and thus no adjuvant therapy was applied.Paratesticular liposarcoma requires aggressive surgical approach for curative treatment with radical orchiectomy. The dedifferentiated subtype is less common with a trend to recur locally. Osseous metaplasia is rare and does not represent an unfavorable prognostic factor. However, these tumors, due to their potential metastatic risk, should be treated with radical orchiectomy followed by additional therapy in cases of local recurrence or distant metastases and the patients should undergo intensive radiologic follow-up.


Schwannoma is a benign, solitary, slow-growing neoplasm of the peripheral nerve sheath. These tumors are rarely found in the external genital system, and only a few cases of vulvar schwannoma have been reported. Herein, we report a case of a vulvar schwannoma. A 37-year-old woman presented with a 3-cm-sized painless mass of the vulva which had been present for 3 years. Magnetic resonance imaging (MRI) of the pelvis showed an isolated finding of a 4.6-cm-sized round mass with a well-defined margin in the midline vulvar area. Simple excision of the tumor was undertaken, and histological examination with immunohistochemical testing demonstrated a vulvar schwannoma. Although benign schwannoma only rarely occurs in the vulva and other external areas of female genitalias, we suggest that it should be considered a differential diagnosis for patients that present a vulvar enlargement or mass. Simple surgical resection and follow-up is the most convenient treatment. Schwannoma  is generally a benign, solitary, nodular tumor of a peripheral nerve sheath. It can occur sporadically or in patients with neurofibromatosis. Its most common locations are in the head, neck, upper and lower extremities, posterior mediastinum, and retroperitoneum . HoweverA 37-year-old patient presented with a painless palpable vulvar mass, which was first noted 3 years prior to presentation, and its size had increased steadily ever since. There was no associated ulceration, bleeding, or pain, except for mild discomfort. There was no remarkable past medical, social, or familiar history of cancer or hereditary genetic disorders. On admission, vital signs  were within normal limits. The patient was in good general health and had no significant weight loss. On physical examination, there was a 3 × 2 cm sized solid subcutaneous mass located adjacent and superior to the clitoris, which was normal in size and shape, involving the upper midline portion of both vulva. It was an immobile and non-tender solitary mass with an irregular contour Figure . The patSchwannoma is a benign peripheral nerve sheath tumor, arising from Schwann cells in the myelin sheath of the peripheral nerves. Schwannoma is solitary, encapsulated, slow-growing tumor which is characteristically painless . It is mWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The urachus is an embryologic remnant which degenerates after the birth. Defective obliteration of the urachus leads to urachal abnormalities. An infected urachal cyst is one of the urachal abnormalities and this pathology is rare in adult women. We report a case of 33-year-old woman with pelvic pain and dysuria who was diagnosed with infected urachal cyst. Infected urachal cyst is a rare pathology in adult women and this pathology should be considered in the differential diagnosis of acute abdomen. The urachus is an embryologic remnant which is formed by the obliteration of the allantois. This fibrous tubular structure is located in the midline and extends from the apex of the bladder to the umbilicus. The urachus degenerates after the birth and it is named as median umbilical ligament. The urachal abnormalities are caused by the defective obliteration of the urachus. Urachal abscess is an uncommon disease of the urachal abnormalities and it occurs especially rarely in adults , 2. We rμL and a C-reactive protein level of 8.6 mg/dL. Bimanual examination revealed pelvic tenderness and a suprapubic firm mass. Ultrasound examination showed an abscess-like mass (4.5 × 4 × 4 cm in diameter) located at the posterosuperior part of the bladder in the midline. The uterus and adnexa were regular. Abdominopelvic computed tomography revealed a hypodense abscess-like mass in the midline which extended to the rectus muscle and an indentation at the posterior part of the bladder (Figures A 33-year-old woman, gravida 2 and para 2, was admitted to the emergency department of our hospital with pelvic pain and dysuria lasting for 3 days. On her physical examination, there was tenderness in the lower abdomen, the body temperature was 38.4°C, the blood pressure was 110/70 mmHg, and the heart rate was 100 beats/min. Laboratory data revealed a white blood cell count of 12.800/ Escherichia coli, Enterococcus faecium, and Klebsiella pneumonia also can be isolated [The urachus is an embryonic connection between the bladder dome and the umbilicus, which elongates as the bladder descends. The urachus is obliterated by the fifth to seventh month of gestation and it forms the median umbilical ligament as a fibrous cord which lies between the transversalis fascia and parietal peritoneum , 2. Uracisolated . The recisolated . Clinicaisolated . Althoug


In medical practice, plastic surgeons confront with patients with sarcomas of the extremities that require a radical surgical approach. Knowing when to attempt limb-sparing surgery and when to give in to limb amputation is one of the most difficult decisions a surgeon can take. The correct approach and management of such cases ensure surgical success and the patient survival.In this paper, the case of a 56-year-old man, admitted in our clinic with a crush injury of the right calf and subsequent haematoma is presented. During haematoma drainage, the surgeon noticed abnormal tissue and performed an incisional biopsy. The patient was diagnosed with myxoid liposarcoma of the external compartment of the right calf. Limb amputation was proposed, but the patient refused. After the clinical examination, blood tests and diagnostic imaging, which allowed the correct evaluation of the case-tumor sizes and neighboring tissue reports, and preoperatory radiotherapy, limb sparing surgery, respectively primary tumor excision was decided to be performed. Negative margins could not be obtained by 3 successive resections or by adjuvant chemotherapy.The presented case supports the idea that limb-sparing surgery is only applicable to carefully selected patients with soft tissue sarcoma. In some cases, radical excision involving even mutilating amputations may provide a better oncologic and functional result.Abbreviations: HE = haematoma, MRI = magnetic resonance imaging, NCCN = National Comprehensive Cancer Network, CT-SCAN = computer tomography scan, PET-CT = positron emission computer tomography, FDG = fluoro-deoxy-glucose, SUV = standardized uptake value, AJCC = American Joint Committee on Cancer It involves balanced translocations , but additional chromosomal aberrations also occur [2-4]. It has a peak incidence during the fifth decade. It develops preferentially in the lower extremity (75%), in the medial thigh or popliteal region. They are generally well circumscribed but in particular cases, can interdigitate with muscles, tendons or can compress neurovascular structures. Typically, they appear as nonhomogeneous masses on CT scans [3]. Complete surgical resection is the only curative means for the localized disease; however, both radiation and conventional cytotoxic chemotherapy remain controversial for metastatic or unresectable disease . Their most frequent metastases are found in lungs or bone [6].Soft tissue sarcomas are rare malignancies that arise from mesenchymal pluripotent stem cells, of which liposarcomas are the most common. Myxoid liposarcomas account for about 30-50% of all liposarcomas [The case of a 56-year-old male patient, aged 56, polyallergic, known with high blood pressure, admitted in our clinic for a crush injury of the anterolateral aspect of the right calf and subsequent haematoma, is presented. The patient had been complaining for a discomfort to the anterior right calf during effort for the past 10 years. Four years ago, the discomfort became pain, accompanied by muscle weakness, and the patient noticed a slowly growing 5-6 cm soft tissue mass on the inferior pole of the tibialis anterior muscle. He seeked for medical attention, underwent a plain MRI investigation of the right lower limb that revealed a deep haematoma of the external aspect of the right calf 16.5/3.8/6.8cm that pierced the fascia entering the anterior compartment, with associated muscle atrophy, but without muscle invasion. The attending general surgeon diagnosed him with chronic venous insufficiency and prescribed medical treatment. After an accidental crush injury to the affected region, the patient was referred to our clinic. No medical documents were presented at admission.Physical examination showed a global enlargement of the right calf, more significant on the anterolateral compartment, measuring 27/ 10 cm, tender, with associated muscle weakness (M3), fixed to adjacent structures. After a blood test, EKG and a cardiologic evaluation, the patient was operated on. The haematoma was evacuated through an S-shaped incision on the external aspect of the right calf. Because of the abnormal gross anatomy, the surgeon also performed an incisional biopsy. The wound was sutured, dressed and the patient discharged after 3 days.Fig. 1 A,B).The pathologic examination described a low-grade myxoid liposarcoma showing multiple transition areas to round cell liposarcoma G1 with positive margins and subsequent muscle fiber invasion .The patient was referred to an oncologist and radiation therapy specialist. The imagistic evaluation of the tumor was recommended. Gadolinum enhanced magnetic resonance imaging (MRI) of the lower right limb showed a soft tissue mass, deep in the anterior compartment of 28.6/9.4/6.1cm, with increased intake, increased vascularisation, of lipomatous signal and central necrosis, invading the tibialis posterior muscle, and compressing the fibula .Computer tomography (CT) scan of the thorax, abdomen and pelvis revealed 2 right pulmonary nodules of 5/ 4mm in the superior right lobe and 8/ 6 mm in the inferior right lobe, without distant metastases. A benign nodule of the latissimus dorsi of 14/ 7 mm was discovered .Whole body positron emission computer tomography (PET-CT) confirmed the presence of a benign nodule of the latissimus dorsi and increased FDG intake in the upper two-thirds of the right calf on the anterolateral compartment  and subsequent skin ulceration  .Whole body bone scan excluded distant metastasis, showing a soft tissue mass with increased intake and high vascularisation .After a complete evaluation, the tumor was staged according to the American Joint Committee on Cancer (AJCC) system stage T2bN0M0 G1, stage IIB disease. Due to posterior and anterolateral compartment involvement, limb-sparing surgery could not provide the necessary margins of safety. The radical surgical procedure was imposed by the tumor’s large dimension, muscle invasion and invasion of vascular and nervous bundle. Amputation was proposed, but refused by the patient. In order to perform the radical excision of the tumor, radiation therapy was necessary. Neoadjuvant external beam radiation therapy was administered using a regimen of 2Gy in 25 fractions in 30 days. The MRI examination showed a significant decrease in tumor size to 18/7,5/5.6 cm, with no posterior compartment invasion. Unfortunately, fibular invasion and involvement of the tibialis posterior artery and were noted. An area of skin invasion of 2.5 cm was visible . The resected piece, consisted in a 6/ 2 cm skin paddle, 12 cm of fibula, tibialis anterior, extensor hallucis, extensor digitorum, peronier, tibialis posterior and external half of the soleus muscles were sent for pathologic examination . All the margins were positive. The patient was admitted in our Clinic, and after a preoperatory workup, an S-shaped incision was placed on the external aspect of the right calf, en bloc excision of the primary tumor, drainage, suture and splinting were performed . The excision margins were positive. The patient was discharged after 3 days and developed a 5/ 4cm necrosis on the anterolateral aspect of the right calf, that was surgically removed, en-bloc with a 3 cm mass of soleus muscle  the final treatment plan was established. After the patient’s refusal of the amputation, the surgical plan was adapted to the patient’s wish. The oncologic treatment was mandatory. The prognosis was uncertain, most probably, the patient already having suffered from the metastatic spread, even if it was not observed on the CT scans. All the efforts having been undertaken, the patient’s overall survival and disease free interval remained to be observed.Case particularities included misdiagnosis of the tumor due to plain MRI, traumatic injury of the soft tissue sarcoma causing inadvertent biopsy, the NCCN 2014  guideline-oriented approach, the refusal of the proposed amputation, the administration of neoadjuvant radiation therapy and the postoperatory association of chemotherapy and radiation therapy.The following conclusions can be drawn: • Soft tissue sarcomas are frequently misdiagnosed, due to improper clinical or imagistic investigations.• Preoperatory imagistic evaluation by gadolinium enhanced MRI represents the current golden standard evaluating the tumor’s extension both at surface and in depth, allowing the surgeon to accurately plan the radical excision.• The surgical treatment is the radical excision of the primary tumor with negative margins. This supposes, quite often, the neighboring muscle resection or total amputation. Whenever possible, limb-sparing surgery should be performed. • Even low-grade tumors such as myxoid liposarcoma can be invasive and may require limb amputation.• Patient refusal of the correct therapy, forced the surgeon to perform the incomplete tumor resection.• Repeated reinterventions with persisting positive margins cause significant morbidity and decrease overall survival rates, proving that limb sparing should never prevail to radicality.AcknowledgmentsThis paper was co-financed from the European Social Fund, through the Sectorial Operational Programme Human Resources Development 2007-2013, project number POSDRU/159/1.5/S/138907 “Excellence in scientific interdisciplinary research, doctoral and postdoctoral, in the economic, social and medical fields –EXCELIS”, coordinator The Bucharest University of Economic Studies”. Disclosures: None