Case Report. A 52-year-old female presented to us with pain and lump in the right lower abdomen. The patient was earlier subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) for low-grade endometrial stromal sarcoma. Postoperatively patient received radiotherapy but no hormone therapy. After 10 years of follow up patient presented with a polypoidal lesion in the caecum. Patient was evaluated fully and subjected to resection of this polypoidal lesion, which proved out to be high-grade endometrial stromal sarcoma. Conclusion. Recurrence of endometrial stromal sarcoma in the caecum is very rare. However, this entity needs to be kept in mind for differential diagnosis of a caecal mass. Recurrence in such cases may present quite late.Endometrial stromal sarcomas of uterus are quite rare. Most of the recurrences in these tumors are seen in the pelvis. However, extrapelvic recurrences and metastases to other parts are quite unusual. Here, we are reporting a rare case of caecal recurrence of endometrial stromal sarcoma.  Endometrial stromal sarcomas (ESS) are a rare variant of malignant mesenchymal tumors. This disease entity belongs to the rarest uterine malignancies  . RecurreA 52-year-old female was subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) for LG-ESS. Patient received radiotherapy and was doing well for 10 years. After 10 years, she presented with the right lower abdominal discomfort and a lump. USG showed an ill-defined mass with heterogenous echogenicity in the right lower quadrant of the abdomen. MRI revealed a well-defined regular contour lesion, measuring 5.3 × 4.8 cm with isointense signals on T2-weighted images, anteromedial to right external iliac vessels . Lesion PET/CT scan  showed aTumor markers (Ca125 and CEA) and other baseline investigations were within normal limits. Operative findings revealed 5 × 4 cm solid thin pedicled, well encapsulated, and mobile mass arising from the caecum, without being adherent to surrounding bowel loops or other structures . PreviouHistopathological examination revealed features of high-grade endometrial stromal sarcoma . ImmunohLow-grade endometrial stromal sarcomas (LG-ESS) are usually slow-growing neoplasms with an indolent clinical course, whereas high-grade ESS often show more aggressive nature. For LG-ESS, total abdominal hysterectomy is recommended as primary treatment, whereas the need for bilateral salpingo-oophorectomy, adjuvant chemotherapy, radiotherapy, or hormonal therapy is still controversial . Our patRecurrence develops in one-third to one-half of patients with LG-ESS. It has been reported even 30 years after the initial treatment  and usuaLi and Chang  reportedESS may arise as a primary extrauterine endometrial sarcoma (EESS), mainly on the background of endometriosis, with predominant gonadal involvement. Very rarely does EESS arise primarily in the gastrointestinal or extragastrointestinal tract organs outside the pelvis or evolve without preceding endometriosis . Our patThe main differential diagnosis for EESS when arising from bowel is some other mesenchymal tumor, especially gastrointestinal stromal tumor (GIST). Spindle cells in EESS are characterized by monotonous arrangement, compared with sheets or fascicle arrangement in GIST. Proliferation of small arterioles, resembling spiral arterioles of the endometrium, is also typical of EESS. Mucocele of appendix can also present in this manner. However, the final diagnosis, especially in cases of unusual tumor location and absence of endometriosis, depends on immunohistochemistry. Positive labelling for CD10, PR, and ER with no reactivity for CD117 and CD34 confirms the diagnosis of ESS . DiagnosRecurrence of endometrial stromal sarcoma arising in the caecum is rare. Postoperative hormone therapy is important in such cases. Besides, this entity even though rare should be kept in mind for differential diagnosis of a caecal mass.


Its occurrence in the retroperitoneal region is extremely rare. The aim of this study is to report a case of retroperitoneal lipoma with a literature review.A 34-year-old female presented with abdominal distension and severe back pain for one year duration. She also reported weight loss and constipation. The examination revealed asymmetrical abdominal distension and everted umbilicus. There was a big irregular mass occupying the whole abdomen reaching into the xiphisternum, firm in consistency, smooth surface, and well defined borders. Abdominal ultrasound demonstrated a large retroperitoneal heterogeneous mass. Computed tomography (CT) scan showed a large well defined hyperechoic mass with fibrous septa extending from the left ovary up to the diaphragm. Exploratory laparotomy revealed a giant clearly demarcated fatty tumor adherent to the retroperitoneal fatty tissues. The histopathological examination of the specimen confirmed the diagnosis of the retroperitoneal lipoma.Retroperitoneal lipomas have been reported in various age groups; namely children, middle and old age patients. Based on the characteristic radiological features of the tumor, enormous diagnostic work-up is not justified.Retroperitoneal lipoma is a very rare variant of lipoma, presents with various signs and symptoms that may be misleading. Radiologic imaging especially CT scan is the diagnostic tool of choice. Surgical resection is the main modality of management. It is tThe aim of this study is to report a case of retroperitoneal lipoma in line with SCARE criteria with a literature review .1.1A 34-year-old female presented with abdominal distension and severe back pain for one year duration, during which she had been diagnosed and treated as a case of irritable bowel syndrome. She also reported weight loss and constipation. Her past medical history was negative.1.2The examination revealed an asymmetrical abdominal distension and everted umbilicus. There was a big irregular mass occupying the whole abdomen reaching into the xiphisternum, firm in consistency, smooth surface and well defined borders. It not attached to the skin and the examiner failed to get above it.1.3Complete blood count was normal. Abdominal ultrasound demonstrated a large retroperitoneal heterogeneous mass. The origin of the mass was not clear. It occupied the whole abdominal cavity beyond measurement, displacing the whole abdominal viscera. CT scan showed a large well defined hyperechoic mass with fibrous septa extending from the left ovary up to the diaphragm displacing the bowel to the right and the stomach upward with normal uterus and right ovary, normal size and density of liver and spleen, no evidence of pelvic lymph node enlargement or bony lesions in spine or pelvic bones , Fig. 2.1.4After interdisciplinary discussion regarding the management of the case, exploratory laparotomy was done. Intraoperatively, a giant clearly demarcated fatty tumor was found which was adherent to the retroperitoneal fatty tissues extended to the left ovary and measured about 45*48*13 cm . After r1.5rd postoperative day. The patient was discharged on the 6th postoperative day uneventfully. The patient was well six months after the operation and she was free from recurrence.Post operatively, the patient was given a unit of whole blood and kept on intravenous fluid with early mobilization. Bowel motion was observed on the 32Retroperitoneal lipomas have been reported in various age groups; namely children, middle and old age patients . WenigerClinical presentation of retroperitoneal lipoma varies in different reports ranging from abdominal distention to signs and symptoms of sciatica ,8. DuranBased on the characteristic radiological features of the tumor, enormous diagnostic work-up is not justified. However due to rarity of the disease and unawareness of the health care professionals regarding the condition, in most of the time, the provisional diagnosis is misleading . CT scanAlthough the weight of the retroperitoneal lipoma was not documented by most of the authors, the reported weight of the resected specimen is variable ranging from 145 g to 19.5 kg, Postoperative follow up may not be smooth in all cases of giant retroperitoneal lipoma due to prolonged compression of the abdominal organ  by the tumor ,11. WeniIn conclusion, retroperitoneal lipoma is a very rare variant of lipoma. It presents with various signs and symptoms that may be misleading. Radiologic imaging especially CT scan is the diagnostic tool of choice. Preoperative biopsy (fine needle and tru-cut biopsy) is not mandatory for the diagnosis. Surgical resection is the main modality of management.There is no conflict to be declared.No source to be stated.Approval has been taken from Kscien organization for scientific research, no. 51.A written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Mohammad Hasan M. Al-Ali, Okba F. Ahmed: preparing the draft with final approval of the manuscript.Shvan H. Mohammed, Marwan N. Hassan, Shadi H. Sidiq, Mohammed Q. Mustafa, Kayhan A. Najar, Ismael Y. Abdullah: revising the draft, reviewing the literature and follow up with final approval of the manuscript.Abdulwahid M. Salih: Revising the manuscript. Final approval of the manuscript.Fahmi Hussein Kakamad: writing the manuscript, reviewing the literature and final approval of the manuscript.It is a case report, not applicable.Fahmi Hussein kakamad.Not commissioned, externally peer reviewed.


A surgical approach is the choice in young infants with MTH, who are furthest from the time of physiological involution of the thymus, and when the thymus achieves the largest relative size, a surgical approach is the choice. Steroid therapy has been shown to be ineffective . No surgical complications have been reported, and the outcome is excellent. Recurrence has been seen in only one case. He was admitted to our pediatric clinic and treated with ceftriaxone (80 mg/kg/d) and clarithromycin (15 mg/kg/d). A chest X‐ray after 5 days of treatment revealed an improvement in the thickening of the right lung, but persistence of mediastinal enlargement. A chest computerized tomography scan (CT scan) was done and showed enlargement of the anterior mediastinum, occupied by solid inhomogeneous predominantly hypodense hypovascularized tissue, with a total dimension of 9.6 × 6 × 10 cm, with left paramedian development with minimal imprint on the jugulo‐subclavian confluence and on the homolateral anonymous vein. This was associated with pleural effusion of a maximum thickness of about 7 mm. The thymus was not well recognized .Abdominal and neck ultrasounds were normal. Echocardiography showed minimum pericardial effusion but no heart chamber compression by the mediastinal mass. Oncologic markers  were negative. Subsequent complete blood count revealed an increase in lymphocytosis .Because of clinical improvement and radiological stability of the size of the mass, the patient was discharged. A steroidal therapy (prednisone 2.5 mg/kg/d) was prescribed for 40 days. Radiological investigations showed initially minimal change in tumor size, but subsequently a rapid increase in dimension that reached 10.8 × 11 × 9 cm, with compression of the heart chamber and right main bronchus with atelectasis. The child underwent surgical excision of the mass via right thoracotomy. The mass weighed 492 g and measured 15 × 11.5 × 5 cm. Microscopic finding showed preservation of the normal thymic architecture. Immunohistochemical analysis revealed TTH associated with extramedullary myelopoiesis. Postoperative recovery was uneventful, and the patient was discharged from hospital five days after surgery. Laboratory results (complete blood count and inflammatory indices) at 1 month were normal. A chest X‐ray 3 months after surgery was normal. At 10 months of follow‐up, the child was asymptomatic and showed regular growth; no recurrence was detected.2The thymus is a gland situated in the anterior mediastinum, embryologically derived from the third and fourth pairs of pharyngeal pouches.True thymic hyperplasia is a rare but significant pathology in pediatric age because of its potentially serious consequences. It is characterized by an increase in size and weight of the thymus gland with preservation of normal thymic architecture and immunohistochemical appearance, but without an apparent cause.Massive true thymic hyperplasia (MTH) is a variant of TTH. As there are no generally accepted criteria for defining “massive,” in literature, the following guidelines are suggested: (a) the thymus should be greater than the heart shadow on posterior‐anterior chest radiograph, (b) it should weigh several times the expected weight for the age of the patient, and (c) it should represent more than 2% of the body mass.Clinically, MTH could present with effects of mediastinal compression, for instance, respiratory distress, dysphagia or airway obstruction, acute or recurrent pulmonary infections, or less commonly as an incidental finding.Being a rare condition, there are no guidelines on its management.A review of the literature revealed that most patients (80%) were managed surgically with complete excision of the mass, with no postoperative complications.In our case, the decision as to the best management was not easy to take, because of the few cases described and the absence of clear guidelines on therapy. Following confirmation of TTH by biopsy, a period with steroid therapy was tried. It proved ineffective, with subsequent enlargement of the mass. Subsequently, we performed total surgical excision of the mass easily and with no complications.With the aim of analyzing clinical presentation and management of giant thymic hyperplasia in young infants, we reviewed the cases of MTH reported in the literature between 1976 and March 2018 in children aged <2 years of life, the age when the thymus is relatively largest.A total of 14 cases met the inclusion criteria, and they are all reported in Table Ten out of fourteen (71.4%) were male and four out of fourteen (28.6%) female. The median age at onset of symptoms was 10.3 months old. Twelve out of fourteen (85.7%) patients presented with respiratory symptoms. MTH was discovered incidentally in only one patient. Six out of fourteen (42.9%) patients were initially given steroid treatment, with no benefits: in one case, the thymus regrew after therapy was suspended; in the others five cases, it was ineffective also due to significant preoperative shrinkage.Finally, all patients, including our case, were managed surgically. In children aged <2 years of age with MTH, no conservative treatment has been reported. No peri‐ or post‐operative complications have been observed. In our case, the giant mass was well‐distinguished from the surrounding tissues and was not infiltrating; it was easily removed.Thirteen of the fourteen (92.9%) of patients were asymptomatic during follow‐up. Only in one case, a recurrence was observed during follow‐up and it was treated successfully with a second surgical resection.Neither this paper nor any part of its essential substance has been or will be published or submitted to another scientific journal or is being considered for publication elsewhere. This submission represents original work. All the authors contributed to the writing and/or revision of the manuscript, and we have all read and approved the submission of this manuscript. We declare no conflict of interest in relation to this paper. Parents give their consent for publication of textual material (case history) and radiographic images.ET, MC, and LP: revised the literature and drafted the manuscript. DD: contributed to the enrollment of the patient, diagnosis, and management. DD, GP, and AP: revised the article and approved the manuscript in the final form.


Rectus sheath hematoma (RSH) is an uncommon entity associated with predisposing factors such as anticoagulation. It may mimic more frequent abdominal conditions and its accurate diagnosis is important to focus on the correct treatments and improve morbidity and mortality.An elderly patient with shock, abdominal pain, palpable abdominal mass, and anemia was suspected of having a large RSH by point-of-care ultrasound (POCUS), which was then confirmed by computed tomography. Surgery was performed, markedly improving his clinical status.POCUS has a good sensitivity for the diagnosis of RSH and it is also an excellent tool for patient follow-up.The online version of this article (10.1186/s13089-019-0129-4) contains supplementary material, which is available to authorized users. Rectus sheath hematoma (RSH) is an uncommon entity, accounting for less than 2% of patients complaining of acute abdominal pain . It may Known risk factors predisposing to RSH are anticoagulation (nearly invariably present); older age; female sex; pregnancy; trauma; iatrogenic/surgery; chronic medical conditions, such as hypertension, atherosclerosis or hematologic diseases; coughing and forceful rectus muscle contractions –4.The overall mortality rate of RSH is around 4% and rises up to 25% in anticoagulated patients due to increased hemorrhage volume [We present the case of a patient with a large RSH suspected by the findings of point-of-care ultrasound (POCUS) and confirmed by computed tomography (CT) and discuss the importance of POCUS in the diagnosis and management of patients with this condition.An 85 year-old-male patient with a history of congestive heart failure and atrial fibrillation under anticoagulation with enoxaparin was admitted to the intensive care unit (ICU) presenting with shock and abdominal pain. Extremely pale skin and mucous membranes were noted, along with an extensive ecchymosis occupying the left hemiabdomen . The abdominal mass bulged on the left flank and was soft, painful and non-pulsating on palpation. Blood chemistry highlighted anemia (hemoglobin 5.7 g/dl), metabolic acidosis and elevation of creatinine and BUN; coagulation tests were normal. A POCUS abdominal ultrasound of the left flank Fig. : Video 1Rectus sheath hematoma results from bleeding into the rectus sheath, after damage to the epigastric arteries or by direct muscle tear . RSH is The most common presentation of RSH is abdominal pain and a palpable abdominal mass , 4, follTo reach a diagnosis, the clinical clues orienting toward an RSH are Carnett’s sign , Fothergill´s sign  and Cullen’s  and Grey Turner’s  signs. However, clinical data are fairly unreliable, with a failure rate higher than 50% for the diagnosis of an RSH . ConversConservative treatment is effective in the majority of patients with RSH. This group of patients is followed up by clinical and laboratory data, as well as serial ultrasound (US) examinations, measuring the size of the hematoma. US-guided drainage does not offer clear advantages compared to conservative treatment and may even prolong the length of hospital stay and predispose to a hematoma infection. Thus, this practice should not be routinely recommended . HoweverRSH, although uncommon, should be considered in any patient presenting with abdominal pain. Abdominal POCUS, as the first-line imaging modality for the patient in the ICU or the emergency department, may allow practitioners to reach this diagnosis in most of the patients, avoiding the performance of unnecessary tests and the administration of wrong treatments, and allowing to selectively use more costly, sophisticated or potentially harmful methods, including the use of iodinated contrast media, in certain cases requiring so. POCUS should also be considered as a suitable method for the follow-up period.Additional file 1: Video 1. Abdominal ultrasound from the left coronal view showing a large complex cystic mass with multiple internal septations, corresponding to a large rectus sheath hematoma.


KMT2D and KDM6A genes. Several tumors have been reported with KS; however, there have been no reports of hepatocellular carcinoma (HCC) or hepatic adenomatosis. We present an adolescent girl with KS and a novel KMT2D mutation who developed diffuse adenomatosis, HCC, and subsequently underwent liver transplantation.Kabuki syndrome (KS) is a rare disorder primarily associated with mutations in the  Kabuki syndrome (KS) is a rare disorder (1 : 32000 births) characterized by distinctive facial features including eversion of the lower lateral eyelids, arched eyebrows, depressed nasal tip, and prominent ears; skeletal abnormalities, such as brachydactyly and spinal and digital anomalies; mild to moderate cognitive delay; and postnatal growth impairment [KMT2D (MIM #602113) at 12q13.12 account for 55–80% of patients, while 9–14% of KMT2D-negative patients have deletions or mutations in the KDM6A (MIM #300128) gene at Xp11.3 [Mutations in Increasing numbers of tumors have been reported concurrently in KS including hepatoblastoma, neuroblastoma, pilomatricoma, pre-B-cell acute lymphoblastic leukemia, spinal ependymoma, and synovial sarcoma –7; howevHCC is the second most common pediatric liver tumor, accounting for 87% of liver tumors among adolescents . While cKMT2D gene, c.8594dupC, who presented with HA and HCC.We describe a novel case of KS due to a heterozygous mutation in exon 31 of the A 15-year-old female with KS diagnosed at 5 years of age presented with an incidental hepatic mass found during a follow-up echocardiogram of her repaired atrial septal defect. She was referred to Liver Clinic with complaints of intermittent abdominal pain, occasional dark urine in the preceding two months, and unexplained pruritus for 10 years. There was no history of jaundice, change in stool color, hematochezia, melena, fevers, anorexia, or recent weight loss. Her past medical history included medullary nephrocalcinosis, intermittent microhematuria, IgG deficiency, asthma, bilateral conductive hearing loss, and developmental delay. She had also been started on a low-dose estrogen containing oral contraceptive pill (OCP) 1 year prior to presentation which had been increased to a high-dose estrogen containing OCP six months previously for menorrhagia. The only other medications she had been on were Risperdal and a multivitamin. Physical examination revealed facial features consistent with KS as well as esotropia. She was noted to have palmar erythema but no other stigmata of chronic liver disease. She had hepatosplenomegaly with a firm palpable epigastric mass that extended 8 cm below the xiphoid process.Biochemical analysis demonstrated the following: AST 65 U/L, ALT 50 U/L, GGT 85 U/L, total bilirubin 1.2 mg/dL, direct bilirubin 0.6 mg/dL with normal complete blood count, electrolytes, urea, and creatinine. Alpha-fetoprotein and CA-19-9 were not elevated. Nonfasting bile acid profiles were obtained at her initial visit and revealed elevated cholic acid, chenodeoxycholic acid, and total bile acids. Tests for Wilson disease, alpha-1-antitrypsin, viral hepatitis, and autoimmune hepatitis were all negative.Imaging included an abdominal CT scan that demonstrated a 15 cm diameter left hepatic lobe mass with heterogeneous enhancement and coarse calcifications. Similar findings were present on abdominal MRI performed with Gadavist and Eovist. A 15 × 11 × 14.5 cm heterogeneous mass within the left hepatic lobe with retention of hepatobiliary contrast was demonstrated on abnormal background liver parenchyma with multiple other similar appearing smaller masses in both hepatic lobes Figures . These lAn ultrasound-guided liver biopsy taken of the large left hepatic lobe lesion histologically demonstrated an atypical hepatic adenoma with possible early malignant transformation. Reticulin staining showed focal disruption of the reticulin meshwork with thickened liver plates and a slightly increased Ki-67 proliferation index. Additionally, biopsy of the background hepatic parenchyma in the right hepatic lobe demonstrated benign appearing hepatocytes with changes suggestive of blood flow abnormalities. There was no evidence to suggest the presence of glycogen storage disease.She was subsequently listed and underwent deceased donor liver transplantation. The explanted liver demonstrated numerous hepatic adenomas. Within the left hepatic lobe adenoma (19.5 × 9.8 × 9.5 cm) , hepatocLiver tumors occur rarely in children. HCC usually develops in the context of underlying liver disease that gives rise to cirrhosis such as hepatitis B, hepatitis C, nonalcoholic steatohepatitis, or metabolic/genetic disease such as glycogen storage disease or tyrosinemia. Genetic diseases may also lead to the development of hepatocarcinogenesis, and in particular, those that alter tumor suppression pathways . Our patKMT2D is part of the lysine methyltransferase superfamily that is a group of evolutionarily conserved transcriptional regulators that play an important role in metabolic processes as well as the regulation of development, differentiation, and tumor suppression [KMT2D has been found to be mutated in HCC and has also been reported to be disrupted as a recurrent site of hepatitis B virus integration [KMT2D family mutations may be associated with a trend toward earlier recurrence of disease, greater microvascular invasion, and a more aggressive phenotype [KMT2D may also play an important role in other tumors including medulloblastoma [KMT2D gene may result in abnormal enhancer regulation and altered cell-type specific gene expression or may lead to changes in transcription, DNA breaks, and tumor formation [pression . KMT2D hegration . Furtherhenotype . Somaticblastoma , follicublastoma , and problastoma . It is pormation .KMT2D has also been found to be important in the regulation of the hepatic circadian clock and acts as a transcriptional coactivator of PPARƴ2 [KMT2D mutant mice [f PPARƴ2 , and theant mice . Elevateant mice . Of noteKMT2D plays in tumorigenesis would further elucidate whether patients with KS should undergo routine cancer screening.This is the first report of HA and HCC occurring in a patient with Kabuki syndrome. There is growing concern that KS may predispose to the development of malignancy. Research into the possible role that


Hepatic sclerosing hemangioma, a very rare benign tumor, is characterized by fibrosis and hyalinization occurring in association with degeneration of a hepatic cavernous hemangioma. Such atypical hemangiomas can be diagnosed incorrectly as primary or metastatic malignancies based on imaging characteristics. We present herein a rare case of giant and multiple hepatic sclerosing hemangiomas that are difficult to differentiate from hepatic malignancies and review the relevant literature.18F]-FDG. A provisional diagnosis of multiple scirrhous hepatocellular carcinomas was made on the basis of these imaging studies, and caudate lobectomy of the liver and partial hepatectomy of S2 and S6 were performed. Histopathological examination showed that the tumors were composed of various sized irregularly dilated vessels with some blood thrombi, inflammatory cell infiltration, fibrous and hyalinized sclerotic or myxomatous stroma, resulting in a diagnosis of multiple hepatic sclerosing hemangiomas.The patient was a 48-year-old male who was found to have multiple hepatic tumors and a giant tumor (67 × 53 mm) superior to the inferior vena cave by an abdominal ultrasonography during a routine medical examination. The patient was referred to our hospital for further evaluations and diagnosis of the multiple hepatic tumors. Dynamic CT showed low-density tumors in the delayed phase suggestive of membrane-covered lesions. EOB-MRI demonstrated a mass with low-signal intensity mass on T1-weighted images and areas of high-signal intensity on T2-weighted images and a hypointense mass in the hepatobiliary phase, which showed high intensity on DWI-based ADC map. FDG-PET showed no accumulation of [Differentiation of multiple sclerosing hemangiomas from other hepatic malignant tumors, such as intrahepatic cholangiocarcinoma, metastatic liver cancer, and scirrhous hepatocellular carcinoma characterized by abundant fibrous stroma, is difficult because the radiological findings are very similar. Inclusion of hepatic sclerosing hemangioma in the differential diagnosis of multiple liver tumors could enable optimal management; this possibility is important to consider before planning invasive therapies. Hepatic sclerosing hemangioma, a very rare benign tumor, is characterized by fibrosis and hyalinization occurring in association with degeneration of a hepatic cavernous hemangioma . Even wiA previously healthy 48-year-old male was found to have multiple hepatic tumors and a giant tumor (67 × 53 mm) superior to the inferior vena cave by abdominal ultrasonography during a routine medical examination. He consulted a general physician for further evaluations and had been referred to us because plain CT confirmed multiple tumors, including a giant tumor, in the liver. A laboratory workup on admission showed that total bilirubin (0.6 mg/dL) and albumin (4.2 g/dL) concentrations were within their normal ranges, whereas aspartate aminotransferase (37 IU/L), alanine aminotransferase (70 IU/L), alkaline phosphatase (176 U/L), and gamma-glutamyl transpeptidase (170 IU/L) concentrations were mildly increased. Tumor markers, including alpha-fetoprotein (2.9 ng/ml), protein induced by vitamin K absence or antagonist-II (11 mAU/ml), and carcinoembryonic antigens 19-9 (4.0 IU/L), were within normal limits.b value of 1000  and 2.45 × 10−3 mm2/s , and the ADC mean was 2.33 × 10−3 mm2/s. It was described as a high-intensity mass on the ADC map  revealed well-defined, hypo echoic masses in segment 1 (S1) (67 × 53 mm in diameter), S6 (13 mm), and S2 (9 mm) in the liver Fig. –c. Abdom000 Fig. . Its ADCmap Fig. . There wPET Fig. . HepaticPET Fig. , h. In aOn the basis of these imaging findings, a preoperative diagnosis of multiple scirrhous hepatocellular carcinomas characterized by abundant fibrous stroma was made, and caudate lobectomy of the liver and partial hepatectomy of S2 and S6 were performed. The resected specimen contained a 55 × 56 mm yellowish white solid mass. The cut surfaces of the tumor were elastic and soft with smooth margins Fig. .Fig. 3ReHistopathological examination showed that the tumors were composed of various sized irregularly dilated vessels with some blood thrombi, inflammatory cell infiltration, fibrous and hyalinized sclerotic or myxomatous stroma Fig. , c. On iHemangiomas are the commonest benign hepatic tumors, being found in up to 7% of autopsies in one series . HemangiThese pathological changes can result in the radiological features on CT and MRI being atypical of hemangioma; thus, these lesions can be diagnosed as malignant tumors such as intrahepatic cholangiocarcinoma, metastatic liver cancer, and hepatocellular carcinoma. We searched for “hepatic sclerosing hemangioma” in PubMed and ICHUSHI, a bibliographic database established in 1903 and being updated by the Japan Medical Abstracts Society, and identified 41 patients, including our patient, the characteristics of which we here summarize.18F]-FDG in six patients who underwent this procedure -FDG, it suggested the possible benign character of this sclerosing hemangioma. However, it should be noticed that negative predictive value of FDG-PET for primary hepatocellular carcinoma is less than 50% with sensitivity over 80% [Concerning imaging studies, Miyamoto et al.  summarizrocedure . The radrocedure , 11. In over 80% . TherefoHistopathologically, the cut surfaces of our patient’s resected tumors corresponded with cross-sectional CT images; the dynamic enhancement pattern is related to the vascular spaces component and the central areas of low density on the arterial and delayed phase to the sclerotic component, which on examination were yellowish-white with many hyalinized areas with poor vessels and fibrous changes , 14. AddConfirmation of our provisional diagnosis would have required obtaining a liver biopsy. Although a fine needle biopsy can differentiate a sclerosing hemangioma from hepatocellular carcinoma, this procedure can potentially lead to rupture or seeding of hepatocellular carcinoma. Fine needle biopsies should only be performed to confirm inoperable hepatocellular carcinoma because seeding of tumor in the needle tract has been reported in 1–3% of cases . We suspThis case report illustrates the atypical imaging appearance of sclerosing hemangioma, and these imaging may cause confusion and a diagnosis of scirrhous hepatocellular carcinoma. Although sclerosing hemangiomas are not frequently encountered in clinical practice, they are included in the differential diagnosis of hepatic malignant tumors, such as intrahepatic cholangiocarcinoma, metastatic liver cancer, and scirrhous hepatocellular carcinoma.


Considered to be rare, mucosal melanomas are rare type of melanoma that are found on mucosal surfaces and are primary or metastatic in origin. We report a case of a 66-year-old Hispanic female who presented with vague abdominal pain and upon further endoscopic work-up revealed 2 gastric lesions. Endoscopic biopsy results revealed gastric melanoma in the distal lesion. A PET/CT scan indicated it to be suspicious for the primary site of metastasis but was ultimately diagnosed as a benign nevus on biopsy. An extensive clinical exam showed no other probable sites of origin. The patient underwent a subtotal Billroth II gastrectomy and enterostomy tube placement for temporary feeding. Primary melanoma of the stomach is an exceptionally rare occurrence with limited cases that can be accounted for in literature; thus we report this case for review. Melanoma is the 6th most common cancer in North America . The incAs stated, mucosal melanomas are found to be primary or secondary in nature. Primary mucosal melanomas are usually seen in older populations with an average age of 70 years and accounts for one percent of all melanomas –7. PrimaA 66-year-old Hispanic female presented with vague abdominal pain and exertional chest pain. She had a ten-year history of worsening epigastric pain attributed to gastritis that was treated with Dexilant. A general physical exam was done and did not reveal any significant abnormalities; she also denied any fevers, chills, weight loss/gain, or change in bowel habits. Environmental history was noncontributory as she denied extensive sun-exposure or use of tanning beds and reported that she regularly would use sunscreen with a 30 SPF. Still, there was an extensive past medical history for anemia, diabetes mellitus type II, coronary artery disease, asthma, hypertension, hyperlipidemia, hemorrhoids, and osteoarthritis. She also had a broad surgical history, involving a coronary artery stent in the left anterior descending artery [2016], percutaneous transluminal coronary angioplasty [2016], tubal ligation [1985], cholecystectomy [2007], bladder prolapse repair [2012], rectal prolapse repair [2012], hysterectomy [2012], and total right knee replacement [2013].There is no report of primary tobacco use; however, she does report an extensive second-hand smoke exposure as a result of her biological father. Her family history is significant for multiple cancers, with her father dying of cancer related complications from a head and neck malignancy at the age of 65. The patient's sister died of breast cancer at the age of 44 and half paternal sister also died of breast cancer at the age of 38. Lastly, her brother died of colon cancer at the age of 70 and her mother's death at the age of 67 was due to a myocardial infarction unrelated to cancer. Interestingly, her eldest daughter was diagnosed with melanoma found in a cutaneous lesion, in 2016. Her physical exam did not reveal any concerning skin lesions or palpable abdominal lesions and her vitals were stable.A near total gastrectomy was performed and multiple adhesions from prior surgeries were divided at the beginning of the procedure. A thorough surgical exploration of the abdomen demonstrated no lesions of the liver. The gastric lesions, having been preoperatively marked on endoscopy, were inspected. The proximal lesion was near the GE junction and another was located more distally closer to the antrum. Reconstruction was performed with a Billroth II anastomosis. Due to the almost complete removal of the stomach, a pouch was created via a jejunojejunostomy. A temporary feeding tube was placed distal to the anastomosis. No blood was transfused through this procedure and the estimated blood loss was 200 ml. The pathology associated with the gross specimens is seen in Figures Her immediate postoperative course was unremarkable except for a fever of 101 F during the first 24 hours after surgery, which returned to 98.7°F about 9 hours after the initial fever spike in temperature. She remained afebrile for the remainder of her hospital stay. Overall, the patient progressed as expected postoperatively and tolerated her temporary tube feedings well. An upper GI series on postoperative day 5 showed no anastomosis leakage; thus a postgastrectomy diet was started soon thereafter. After a continued unremarkable postoperative course she was discharged on postoperative day 7 to a subacute rehab.Mucosal melanomas are a very rare type of melanoma and their etiology and pathogenesis continue to remain elusive. Current literature does not have specific staging for primary mucosal melanomas, though the American Joint Committee on Cancer consider mucosal melanomas of any origin are applicable to the current guidelines used for the following mucosal melanomas: head, neck, and vulva (11). This classification system is depicted in Lagoudianakis et al. established specific diagnostic criteria for primary malignant melanoma of the gastrointestinal tract . The criAdditionally, our patient's lack of physical exam findings with a positive histopathology for S100, Melanin A, and HMB-45 suggests that this patient has a true primary gastric melanoma. Overall, even without clear indication of metastasis, the treatment of choice for mucosal melanomas is surgical resection with clear margins , 14. CurMoreover, the patient's family history of head and neck, breast, and colon cancer as well as cutaneous melanoma implores us to explore possible genetic elements that may have played a role in the development of her disease. For example, CDKN2A is a cyclin dependent kinase inhibitor and encodes for specific tumor suppressor proteins –17. EarlGiven the fact that there are very few cases regarding primary mucosal melanomas of gastric origin reported in literature, primary gastric melanomas can be considered to be rare malignancies that can present in a number of different ways . Most ofThe limitations to this study are mostly concerned with the proper physical examination to confirm the lack of primary lesion. The patient was thoroughly evaluated to confirm the lack of any skin lesions that could have been a primary site of metastasis and none were found. However, the patient had not been evaluated by an ophthalmologist. Therefore, an ocular melanoma at this time has not been ruled out.


Primary cardiac angiosarcoma (PCAS) is a malignancy seldom seen in pregnancy. A 23-year-old G1P0 Chinese female was found to have PCAS during her first trimester when she presented with tamponade physiology. The transthoracic echocardiography (TTE) results did not indicate the presence of an intracardiac lesion, and pericardial fluid cytology analysis showed no evidence of malignancy. Cardiac magnetic resonance imaging (CMRI) exhibited a right atrial mass, and tissue biopsy indicated a high-grade angiosarcoma. MRI of the abdomen was suggestive of liver metastasis. She underwent an abortion and was started on combination chemotherapy, with a reduction in both the cardiac and liver masses. In cardiac angiosarcomas, advanced imaging modalities such as MRI should be utilised when there is high clinical suspicion or in the case of pregnancy when trying to minimise foetal harm. Prognosis is poor, and a standardised treatment protocol regardless of pregnancy continues to elude the medical community. Angiosarcoma is a rapidly proliferating malignant neoplasm derived from anaplastic endothelial cells. It most commonly arises from the soft tissues of the head, neck, breast, liver, skin and deep tissue, and is clinically unpredictable , 2. It cPrimary cardiac tumours are exceedingly rare, with an estimated incidence of 0.001%–0.03% . ApproxiA 23-year-old previously healthy Chinese female who was 4 weeks gravid at admission presented with 2 weeks of nausea, vomiting, abdominal pain and chest pain. Her chest pain was sharp and retrosternal, unrelated to activity, and did not vary with position or respiration.On admission, she was tachycardic but otherwise haemodynamically stable. Physical exam was notable for decreased heart sounds, abdominal distention and ascites. Electrocardiogram (EKG) findings demonstrated sinus tachycardia and low voltage QRS. Labs were significant for hyponatremia (131 mEq/L), anion gap metabolic acidosis, elevated International Normalized Ratio (INR) (1.8), Aspartate Aminotransferase (AST) (64 U/L) and Alanine Aminotransferase (ALT) (58 U/L). Chest x-ray revealed bilateral pleural effusions, and abdominal ultrasound showed hyperechoic masses on the liver.Paracentesis of the ascitic fluid suggested a transudative process. Abdominal magnetic resonance imaging (MRI) demonstrated a 3.7 cm mass in the right hepatic lobe with decreased signal intensity on T1 and numerous hyperintense nodules on T2-weighted imaging concerning for metastatic disease. Marked cardiomegaly was incidentally noted. Liver biopsy showed anastomosing hemangiomas without evidence of malignancy. During workup, she developed hypotension, tachycardia and dyspnoea. An emergent transthoracic echocardiography (TTE) showed a large pericardial effusion with early tamponade . She hadThe patient had an open biopsy of the cardiac mass via a right anterior thoracotomy approach that indicated a high-grade PCAS . StagingPCAS is a malignancy that is seldom documented. There is a male-to-female predominance (2:1 ratio) and it occurs between the third and fifth decades of life , 15. ThePCAS commonly arises from the right atrial free wall . SymptomEarly diagnosis of PCAS is clinically difficult. Pregnancy further complicates workup due to the teratogenic, carcinogenic and mutagenic risk associated with certain imaging modalities on the developing foetus. TTE often shows a broad-based mass  though nImaging modalities should be utilised with caution in any pregnant female wishing to carry their pregnancy to term, and a multidisciplinary discussion regarding a cancer staging workup should be considered. When determining imaging modalities for staging, safety of the foetus, likelihood of metastatic disease and the minimal radiation dose to achieve optimal staging accuracy should be considered . With reAs the patient in this case opted for elective abortion, radiation exposure to the foetus was not a limiting factor. Regardless, CMRI was indicated to assess the patient’s cardiac structure and function, given her unprovoked cardiac tamponade and progressive heart failure seen on serial TTEs .If imaging is suggestive of a cardiac mass that is concerning for malignancy, further workup with cytology and tissue biopsy is necessary to confirm the diagnosis. Pericardial fluid cytology has a sensitivity of 87%–100%, specificity of 93.3%–100% and diagnostic accuracy of 94%–95.4% , 32. HowThe prognosis for PCAS is poor, with a median survival of 6–30 months , 33, 35.The anatomic location of PCAS often dictates management. Right-sided PCAS are bulky, infiltrative and rapidly metastasise. Alternatively, left-sided PCAS are well circumscribed and are more often associated with heart failure . In the PCAS is a rare malignancy and this is the first known case of PCAS diagnosed in a first-trimester pregnant patient. PCAS should be on the differential diagnosis in pregnant females presenting with tamponade or heart failure symptoms. If foetal viability is the goal, imaging modalities should be chosen carefully to limit harm. Routine imaging modalities such as TTE may not always detect the presence of intracardiac tumours, as this case suggests. Pericardial fluid cytology has high sensitivity and specificity but may miss the diagnosis as demonstrated in this case. Advanced imaging modalities such as CMRI have excellent diagnostic yield and can demonstrate findings specific to PCAS. Although there is a theoretical risk impacting cellular development, this has not been substantiated in humans. Treatment in the pregnant patient is often a palliative surgical approach if detected at an early stage to let the foetus mature to a viable age. Chemotherapy puts the foetus at high risk of harm if used in the first trimester, but certain agents can be used in the second/third trimester with minimal risk to the developing foetus. If chemotherapy is used, combination chemotherapy is favoured. Overall prognosis remains poor, and it is unknown if pregnancy impacts mortality or leads to accelerated pathogenesis compared to the nonpregnant population.The authors declare that they have no conflicts of interest.The authors received no specific funding for this work.


Shoulder pain is a very common symptom especially in young and active population. Rotator cuff tendinopathies are believed to be the most common cause of shoulder pain up to 86%. Tumors around the shoulder area can cause pain or joint stiffness when expanding in the subacromial space. We present a rare case of a distal clavicle osteochondroma. It is a benign tumor which in this area causes supraspinatus tendinopathy and every physician should suspect this kind of diseases during the diagnostic approach of a shoulder pain. In this case, early diagnosis and appropriate treatment with excision of the lesion gave us a very good outcome with fully relief of the symptoms. The pain of the shoulder is a very common condition especially in young and active population. Except from the common diagnoses such as the impingement syndrome or traumatic lesions of the tendons and the labrum, less common causes can also exist . The tumA 17-year-old male presented with persistent pain of the right shoulder for two months. The pain was exacerbated during abduction and external rotation of the shoulder. The clinical examination was consisted with supraspinatus tendinopathy. The plain radiographs of the right shoulder revealed a solitary lesion of the distal clavicle suggestive for a benign bone lesion  was consisted with a distal clavicle tumor with benign characteristics arising from the distal posterior-inferior surface of the bone Figure .The mass seemed to limit the available space for the supraspinatus muscle near to the musculotendinous junction. An open resection of the tumor was scheduled. With the patient in beach chair position, a longitudinal incision between the distal clavicle and the coracoid process was performed followed by superficial and deep dissection of the soft tissues. The distal clavicle was palpated and a subperiostical exposure of the anterior surface was performed. Because of the posterior-inferior location of the tumor, a distal clavicle resection about one centimeter (cm) from the acromioclavicular (AC) joint was necessary for better approach. With the use of a saw and a curved osteotome the tumor was completely resected including the adjacent clavicle cortex Figure .The coracoclavicular ligaments were checked intraoperatively and found intact. However, an anterior deltoid reattachment to the distal clavicle, through transosseous nonabsorbable sutures, was performed to secure the stability of the clavicle Figure .The postoperative radiograph showed complete resection of the tumor Figure .The arm was protected in sling immobilization for three weeks. The pathology report confirmed the diagnosis of osteochondroma. Gentle exercises were permitted after three weeks with full range of motion (ROM). In six weeks postoperatively, the patient returned to his normal activities and 10 weeks postoperatively he got back in sport activities.Osteochondroma is a true benign bone tumor arising on the external surface of a bone, containing a cavity of marrow covered by cartilage. It is the second most common benign bone tumor following nonossifying fibroma formed by three layers-bone, cartilage and perichondrium . The carBenign and malignant tumors of the clavicle are not common although currently, clavicle bone tumors are more commonly recognized than in the past -10. OsteThe reports about distal clavicle osteochondromas are limited in the literature. Ogawa et al. reported two cases with painful shoulder because of impingement syndrome caused by a distal clavicle osteochondroma . Vander A painful shoulder with rotator cuff tendinitis symptoms should be suspicious for distal clavicle osteochondroma. Although this lesion is not common, delayed diagnosis could be harmful for the patient. A delayed diagnosis of such a case seven years after the beginning of the symptoms is reported in the literature .In our case we preferred an open excision in order the tumor to be excised in clear margins although the imaging characteristics of the tumor were suggested for a benign lesion. However, an arthroscopic excision has been described in 2011 . No recuIn diagnostic approach, MRI imaging seems to be very useful and highlights the cartilage tissue of the lesion . PreoperDistal clavicle osteochondroma is not common. However, it can present and cause shoulder pain and stiffness because of subacromial space narrowing and supraspinatus tendinopathy. Early diagnosis and treatment is necessary for a good outcome.


Demyelinating lesions larger than 2 cm in diameter, with or without edema, are known as tumefactive demyelinating lesions (TDLs). They constitute a rare inflammatory demyelinating disorder of the central nervous system. TDLs are typically characterized by headaches, cortical symptoms such as aphasia, hemiparesis, hemisensory deficits, seizures, and changes in consciousness. TDLs may occur in patients with or without an established diagnosis of MS or may occur as the initial demyelinating event. They may also be observed during follow-up in patients with MS, neuromyelitis optica, acute disseminated encephalomyelitis, or other autoimmune diseases. Differential diagnosis includes brain tumors, abscess, granulomatous diseases, and vasculitis. In some cases, it may be very difficult to distinguish TDLs from a tumor, such that biopsy might be needed. However, no cases of asymptomatic TDLs have yet been reported in the literature. Hence, in this report, we present a case involving an asymptomatic TDL detected incidentally during magnetic resonance imaging in an 18-year-old man. The patient did not develop any symptoms during the 1-year follow-up period. During follow-up, the patient was diagnosed with a radiologically isolated syndrome. TDLs have not previously been identified as radiologically isolated syndrome. Thus, reporting similar cases in the future will help in further understanding this phenomenon. A tumefactive demyelinating lesion (TDL) is an inflammatory demyelinating disorder of the central nervous system (CNS), which may occur in patients with or without an established diagnosis of multiple sclerosis (MS). TDLs are defined as tumor-like demyelinating lesions larger than 2 cm, with or without surrounding edema, mass effect, and ring enhancement. These lesions are often found in the supratentorial region, most commonly in the frontal and parietal lobes. Furthermore, basal ganglia, infratentorial and spinal cord lesions, as well as butterfly lesions involving the corpus callosum, can also occur. Tumefactive lesions typically affect white matter, but gray matter may also be affected . TDLs caWhen clinical and imaging features are typical, biopsy may be unnecessary . FurtherClinical presentation of TDLs often differs from MS, depending on the lesion location and size. Headache, cognitive abnormalities, confusion, changes in consciousness, aphasia/dysphasia, apraxia, hemiparesis, hemisensory deficits, visual field deficits, and seizures can occur due to TDLs . HoweverThe term “radiologically isolated syndrome (RIS)” was first used by Okuda et al. , 8 to deOkuda described CNS demyelination as a “silent thief” that disturbs neuronal and cellular functions slowly and only becomes apparent when a threshold is exceeded . SeveralHerein, we have presented a case with RIS and asymptomatic TDLs and the associated findings during radiological follow-up of the affected patient.An 18-year-old man was referred to our outpatient clinic because of unique MRI findings. MRI was performed by another physician because the patient exhibited tremor in both hands, which began 1 year earlier. However, this tremor did not affect his quality of life. His father had also exhibited a similar tremor in both hands for many years. There was no history of previous health problems. The patient did not complain of headache, fever, arthritis, or skin rash. He had no behavioral, psychiatric, or cognitive complaints. There was no history of vaccination or infection history before the MRI was performed. Detailed neurological examination of the patient revealed only bilateral postural tremor in his hands. No aphasia, apraxia, cortical sensory disturbance, or visual field defect was detected upon examination.MRI revealed a tumefactive edematous lesion in the left frontal area, which was hypointense on T1-weighted images, and hyperintense on T2-weighted and fluid attenuation inversion recovery (FLAIR) images. After gadolinium administration, T1-weighted images demonstrated ring enhancement. Hyperintense lesions were observed in the subcortical and deep white matter in the right hemisphere on T2-weighted and FLAIR images . Spinal Complete blood count, erythrocyte sedimentation rate, and levels of biochemical parameters, including glucose, urea, creatinine, lactate dehydrogenase, activities of alanine aminotransferase, and aspartate aminotransferase, were normal. Vasculitis screening tests for ANA, ANCA, anti-DNA, anti-Ro, and anti-La were negative. The anti-HIV 1/2 test was also negative. Cerebrospinal fluid (CSF) analysis revealed a normal cell count, as well as normal levels of protein and glucose; oligoclonal bands were observed in the CSF that were absent from in the serum. Anti-NMO and anti-MOG antibodies were negative. Thoracic computed tomography and abdominal ultrasonography were normal. Studies of visual evoked potential revealed prolonged P100 latency in the right eye. The optical coherence tomography was normal and the contrast sensitivity was also normal.Neuropsychological examination was performed to determine the cognitive dysfunction. The Stroop test, PASAT-3 (Turkish), and BICAMS battery  were used to assess cognitive performance. His PASAT-3 score was 60/60. Stroop test, SDMT, CVLT-II, and BVMTR scores were within normal values.A 10-Hz symmetric tremor was recorded with surface electromyography (EMG) from the forearm flexor and extensor muscles. Clinical and electromyographic features and positive family history supported an essential tremor diagnosis. The tremor was not associated with demyelinating lesions in MRI images and was therefore regarded as an essential tremor.The patient was not treated and underwent radiological follow-up.Follow-up MRI performed one month later revealed an improvement of the tumefactive lesion in the left frontal area. However, a new tumefactive lesion with perifocal edema, which demonstrated ring-like gadolinium enhancement on T1-weighted images and peripheral restriction on DWI, was observed in the right frontal area . One yeaThe patient was monitored without treatment. One year later, a follow-up was conducted, which revealed that he had not developed any obvious neurological symptoms. Written informed consent was obtained from the participant for the publication of this case report.TDLs in clinically isolated syndrome (CIS) and MS cases typically occur during the first demyelinating attack in most patients. However, these lesions can also occur during follow-ups in patients with known MS. TDLs associated with treatment with fingolimod and natalizumab treatment have been reported in patients with MS. TDLs can also occur during other inflammatory diseases, such as acute disseminated encephalomyelitis (ADEM), acute hemorrhagic leukoencephalopathy, NMO, and MS variants . TDLs have also been reported during viral infection (HIV), malignancy , autoimmune diseases  , 11, 12.In the present case, the essential tremor was not associated with TDLs, suggesting that the TDLs were asymptomatic and were solely detected incidentally on MRI images. Thus far, asymptomatic TDLs have not been reported in the literature, except in patients with known MS. The initial MRI findings of the patient in this report do not meet the criteria for RIS. Follow-up of this patient revealed that the asymptomatic TDLs progressed to a classical form of RIS. A new concept, "RIS-like TDLs" or “silent tumefactive demyelinating lesions” may be appropriate in this case. In the literature, it has been reported that 66-70% of patients with TDL as the initial demyelinating event had progressed to clinically definite MS , 13. OurConclusions from this case report include the following: (1) MRI activity is not always equivalent to clinical appearance. (2) We do not know the mechanisms that prevent symptoms despite the higher MR activity in this patient and other patients with RIS. (3) How we can classify silent demyelinating lesions that do not fulfill the Barkhof criteria for DIS. To our knowledge, this is the first report to describe asymptomatic TDLs. In terms of RIS and its diagnostic criteria, the specific characteristics of tumefactive lesions are not clear. Reporting similar cases in the future may clarify whether such demyelinating lesions can be included as RIS.Tumefactive lesions may also be presented as RIS-like appearance. The contribution of silent tumefactive lesions to risk for development of MS in RIS-like appearance will be determined by long-term follow-up.


Individuals with neurofibromatosis type 1 are much more likely to develop gastrointestinal stromal tumors than those without this condition. The median age for patients with neurofibromatosis type 1-associated gastrointestinal stromal tumors is approximately 65 years. We present a case of a young male with a history of neurofibromatosis type 1 who presented with symptomatic anemia and melena and was ultimately found to have a gastrointestinal stromal tumor. Neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease, is the most common form of the three major clinically and genetically distinct classifications of neurofibromatosis  .NF1 has a predilection for cancer development with a five-fold increase in overall cancer risk, which is the highest cancer incidence yet reported. It is closely associated with malignant peripheral nerve sheath tumors, breast cancers, and pheochromocytomas . It has Patients with NF1 are 34 times more likely to develop GISTs than those without NF1 . AlthougA 21-year-old male with a past medical history of NF1 presented with shortness of breath, lethargy, and melena. He had been admitted to an outside hospital (OSH) two weeks prior with similar symptoms and was found to be anemic due to a gastrointestinal bleed. Colonoscopy and esophagogastroduodenoscopy (EGD) did not reveal any pathology, so the patient was discharged and scheduled for an outpatient capsule endoscopy. This was not performed due to his abrupt re-hospitalization to the OSH due to tachycardia and anemia with hemoglobin of 6 g/dL. Laboratory workup was non-contributory, so the patient was transfused with three units of packed red blood cells and was transferred to our institution .The patient reported continued lethargy and denied abnormal bowel movements between his two earlier hospitalizations. His abdominal and rectal examinations were unremarkable, and skin examination revealed café-au-lait spots on the right arm. Hemoglobin was 8.5 mg/dL, total bilirubin was 2.3 mg/dL, and direct bilirubin was 0.3 mg/dL. Vital signs were within normal limits. An abdominal computed tomography (CT) scan revealed a 2.45 x 2.04 x 3.51 cm well-circumscribed ovoid mass at the jejunum adjacent to a loop of small bowel in the left upper quadrant Figures , 1B. ThiThe differential diagnoses of patients who present with anemia are broad. In the setting of anemia with melena, CT scans, EGDs, and colonoscopies are often ordered. If these examinations are non-revealing, patients are subjected to repeat studies or more specialized studies, such as capsule endoscopies and balloon enteroscopies. This adds to the physical and financial burdens of patients and may still fail to identify the underlying etiology of their symptoms. After admitting the patient in this case to our institution, we had initially planned for a push enteroscopy and a capsule study but were able to avoid this course by taking a thorough history of his NF1, researching its link to GISTs, and correlating this with the suspicious findings on imaging .Without understanding this connection between NF1 and GISTs, achieving a prompt diagnosis would have been difficult given the patient’s limited number of symptoms. Although our patient endorsed fatigue and had melena, he lacked abdominal pain, nausea, vomiting, bowel obstruction, a palpable abdominal mass, early satiety, dysphagia, and odynophagia, all of which have been reported as the most common signs and symptoms of GISTs . His lacIdentifying this tumor early allows for prompt intervention and potential cure or halt of disease progression because GISTs are treatable tumors. Approximately 60% of GISTs are cured with surgery alone, especially in cases that involve low-to-moderate risk GISTs. With higher risk GISTs, a randomized phase III multi-center clinical trial demonstrated that patients who underwent surgery had significantly better recurrence-free survival and overall survival rates with three years of adjuvant imatinib therapy when compared to those who had only one year of adjuvant imatinib therapy . DespiteThis clinical suspicion should not be limited to older patients, despite the estimated median age for NF1-associated GISTs being 65 years, as the patient in this report was significantly younger . A populProviders should have a high index of suspicion for GISTs when taking care of patients with NF1 who present with gastrointestinal symptoms, regardless of age and/or a limited number of symptoms. Early detection of GISTs can minimize the financial and physical burdens associated with unnecessary diagnostic tests. Furthermore, since GISTs are treatable tumors, a prompt diagnosis can lead to effective treatment and prevention of disease progression.


Aspiration of teeth is a rare, potentially fatal complication of tracheal intubation. Early diagnosis and treatment are key to preventing further complications. Interpretation of the physical examination and radiographic evidence together with a high degree of suspicion are necessary to achieve early diagnosis of foreign body aspiration. We examine one such case of a misdiagnosed tooth in lung event. Tracheal intubation carries many risks; one rare and potentially fatal complication is the aspiration of dislodged teeth. Early diagnosis and intervention are required to prevent the complete obstruction of the affected airway. A high degree of suspicion and interpretation of the physical examination and radiographic evidence are required to achieve early diagnosis of foreign body aspiration. It is noted that foreign body aspiration can be tolerated in semi-conscious or unconscious patients for a long time and that it is sometimes difficult to interpret the radiology screening performed. We examine one such case of a misdiagnosed tooth in lung event secondary to tracheal intubation in the intensive care setting.A 70-year-old man was admitted for an elective right hemicolectomy for a large bowel tumour. He was previously fit and well and lived independently. He smoked 12 cigarettes a day and drank 30 units of alcohol per week on average.His operation was uneventful. He underwent a laparoscopic right hemicolectomy for stenosing cancer of the hepatic flexure. The anaesthetic record states a grade one laryngoscopy with dentures and few native teeth. He was extubated post-surgery and was admitted to the high dependency unit for 24 hours. He was then discharged to the surgical level 1 facility.One day after discharge to the level 1 facility, the patient underwent further emergency laparotomy for an anastomotic leak. On the anaesthetic chart, it was noted that a left upper tooth was very loose. This was removed on induction and kept. Intubation proceeded uneventfully.Postoperatively, he was kept sedated and intubated due to the high risk of further deterioration because of bowel contents soiling during the operation. The plain film chest X-ray taken showed no abnormality. As he was stable, he was extubated that afternoon.The following morning, he desaturated and was reintubated. Flexible bronchoscopy was performed due to capillary oxygen saturation (SpO2) 70% with the fraction of inspired oxygen being (FiO2) 100%. There was an unexpected finding of a large tissue mass located 5 cm into the left main bronchus; it was difficult to pass the bronchoscope past this lesion. This lesion is shown in Figure A chest X-ray, shown in Figure Advice was sought from a respiratory consultant who performed flexible bronchoscopy that afternoon. His findings were documented as ‘left main bronchus partially occluded by poorly vascular polypoid necrotic lesions fixed to bronchus’. Washings were sent and biopsies were performed.A repeat chest X-ray that evening at 23:09 showed the tooth had moved from the left main bronchus to the right, as seen in Figure The patient recovered well, with no further complications, and was later discharged home.Dental injury is one of the well-recognised complications of tracheal intubation. This is due to several causative factors, including the anterior teeth being used unintentionally for support and biting on the endotracheal tube. The frequency of dental injury has been shown to be between 0.17% and 12.1%, with an increase in frequency where there is limited mouth opening, limited mobility of the mandibular area and neck, poor visibility of the hypopharynx, and small thyromental distance. Additionally, poor dental health can further increase the risk of tooth in lung events. These include the presence of dental caries, marginal periodontitis, insufficient restorations, and existing ceramic restorations [Tooth aspiration can cause airway obstruction and asphyxiation, atelectasis, inability to clear secretions, and secondary infection . FactorsChest radiographs are useful in identifying tooth aspiration, however, teeth overlying the mediastinum or cardiac muscle may be interpreted as an artefact or not seen at all. On occasion, chest computed tomography (CT) is required to make the diagnosis .In this instance, endoscopic retrieval was not necessary. In cases where it is, flexible bronchoscopy is becoming increasingly successful. However, rigid bronchoscopy may still be required. Both approaches use an oral as opposed to a nasal approach. Foreign bodies that lie in the distal airways are more suited to a flexible bronchoscope. Generally, grasping forceps or rubber tip forceps are used to retrieve hard foreign bodies. However, given the wide, round shape of a tooth, a basket or snare retrieval device may provide better purchase .The location, surface and shape of the foreign body influence how successful these procedures can be. In some cases, it may be necessary to undertake an open thoracotomy, however, it is noted that this should only be used in extremis .In this case, the tooth was able to be removed using deep suction and removal of the endotracheal tube. It highlights how a detailed interpretation of chest radiographs by experienced practitioners should be used to identify foreign bodies early in the clinical course and prevent misdiagnosis.An elective surgical patient with poor dentition was intubated three times. The morning after the second extubation, he desaturated likely due to the aspiration of a tooth and build-up of secretions behind it. Initially thought to be a neoplasm on bronchoscopy, repeat chest X-rays clearly show an aspirated tooth; however, this was not recognised at the time. The tooth was extracted unintentionally with suction during his third and final extubation. The patient made a full recovery.


Neuroendocrine tumors (NETs) of the gallbladder are rare and generally considered low-grade malignancies. We herein describe a case of a patient with a 0.8-cm clear cell NET G1 of the gallbladder with nodal involvement.A 65-year-old man with no medical history indicative of von Hippel-Lindau (VHL) disease underwent laparoscopic cholecystectomy for cholecystolithiasis. There was a 0.8-cm tumor in the neck of the gallbladder. Histologic examination revealed nests or trabecular growth of clear cells with small round-to-oval nuclei. Immunohistochemically, tumor cells showed positivity for chromogranin A and synaptophysin; Ki-67 index was < 1.0%. Based on the World Health Organization 2010 classification, we made a diagnosis of clear cell variant of NET G1 without VHL disease. The tumor invaded the muscular layer and had no extension to the perimuscular connective tissue but had metastasized to a cystic duct node. A radical second resection with regional lymphadenectomy of the gallbladder was performed, and there was no metastasis on histology. After the definitive surgery, he was followed up for 10 months without adjuvant therapy and is alive and well with no evidence of recurrence.Our experience suggests that, even when smaller than 1 cm, NET G1 of the gallbladder can metastasize. When NET G1 is incidentally identified in the gallbladder of a surgical specimen, detailed pathologic examination of the cystic duct node, when found, should be performed to guide whether a radical second resection with regional lymphadenectomy is appropriate. Neuroendocrine tumor (NET) is traditionally an umbrella term that encompasses a broad family of neoplasms originating from neural and endocrine structures located all over the body, especially in the gastrointestinal tract and lung , 2. PrimThe World Health Organization (WHO) 2010 classification divides these tumors into NET G1, G2, and G3 (or neuroendocrine carcinoma: NEC), based on the histologic evaluation of the proliferation rate and mitotic index of NET cells . This cl3/μL). Abdominal ultrasonography showed the gallbladder and common bile duct stones and a low echoic nodule in the neck of the gallbladder preoperatively suspected as Rokitansky-Aschoff sinuses  and the white blood cell count  in 1907 for these “presumptively” benign neoplasms , followeIn gastroenteropancreatic NETs, it is known that the risk of metastasis largely depends on tumor size –14. As fThe WHO 2010 classification divides NETs into three categories according to mitotic count and Ki67 labeling index: well-differentiated, low-grade (NET G1); well-differentiated, intermediate-grade (NET G2); and poorly differentiated, high grade (NET G3 or NEC). This classification enables prognostically relevant patient stratification, and NET G1 is thought to be a relatively “benign” category. However, in our case, a tiny gallbladder NET G1 was associated with nodal involvement. To the best of our knowledge, this is the first report that describes a case of gallbladder NET G1, smaller than 1 cm, with nodal involvement. Our experience suggests that, even when smaller than 1 cm in diameter, NET G1 of the gallbladder can metastasize.Preoperative diagnosis of NETs of the gallbladder is rare because, in general, the patients present with non-specific symptoms such as upper abdominal pain, discomfort, jaundice, and weight loss . PreviouNET cells have eosinophilic to amphophilic cytoplasm. Rare cases, however, have rich clear cytoplasm and are described as clear cell variants. The clear cell NETs of the pancreas have been reported to be a manifestation of VHL disease . Only thGallbladder NET G1, even when smaller than 1 cm, can metastasize. When NET G1 is incidentally identified in a gallbladder surgical specimen, detailed pathologic examination of the cystic duct node, when found, should be performed to guide whether a radical second resection with regional lymphadenectomy is appropriate.


Septicum is causative in over 70% of cases with associated colonic malignancy.•Clinicians should be aware of this clinical entity and consider it in any deteriorating patient with associated colonic malignancy.•Early antibiotics are essential however surgery remains the mainstay of treatment. Clostridium Septicum, mycotic aneurysm and colon neoplasm.There is a known association between We report the case of a 90 year old female admitted with abdominal pain to the general surgery unit. Admission CT scan demonstrated a thick walled caecum and pericaecal inflammation suspicious for a perforated carcinoma. This was subsequently confirmed at colonoscopy with biopsy demonstrating a poorly differentiated adenocarcinoma. A laparoscopic right hemicolectomy was completed the following week after perioperative workup and intravenous antibiotic therapy. On the 9th postoperative day, fever and rising inflammatory markers prompted repeat abdominal CT scan which demonstrated a mycotic aneurysm of the upper abdominal aorta. After discussion with the vascular surgery and infectious diseases team, along the patient and family, the decision was made to palliate. The patient died at home from presumed spontaneous rupture two weeks after discharge.C. Septicum is causative in over 70% of cases with concomitant colonic malignancy.Mycotic aneurysm in colonic malignancy is a rare and often lethal complication. Mycotic aneurysm should be considered in any deteriorating patient with concomitant colonic malignancy. Septicum, mycotic aneurysm and colonic malignancy. We present the case of a 90 year old woman who developed fever and general malaise post right laparoscopic hemicolectomy for caecal adenocarcinoma in a private metropolitan hospital. A mycotic abdominal aortic aneurysm was later discovered on CT scan, with the view that this infection was likely seeded from the caecal rupture. A literature review was also conducted, reviewing the association between C. Septicum and colonic malignancy. This case has been reported in line with the SCARE criteria [Mycotic Aneurysm (MA) is a rare, severe variant of aortic aneurysm accounting for 0.7%–3.3% of all aortic aneurysms, and without treatment all cases are fatal . There icriteria .2A 90 year old woman was admitted to our General Surgery Unit with widespread abdominal pain after recent self discharge from another hospital with a diagnosis of caecal colitis. Past medical history was significant for normal colonoscopy two years prior to presentation, as well as appendicectomy, cholecystectomy and hysterectomy. On admission the patient underwent an abdominal/pelvic CT scan as well as basic pathology testing. Pathology results were unremarkable aside from a CRP of 65 mg/L (<5). The CT scan demonstrated a thick walled caecum and pericaecal inflammation suspicious for a perforated carcinoma .Fig. 1AdA colonoscopy was performed following intravenous antibiotic therapy and echocardiography. Endoscopy demonstrated an obvious neoplasm in the caecum. Histology confirmed an infiltrating poorly differentiated adenocarcinoma. Laparoscopic right hemicolectomy was performed by the consultant colo-rectal surgeon a week later. Post op recovery was uneventful. On the seventh postoperative day the patient developed low grade fever of 38 ° on the context of increasing malaise, lethargy and non-specific abdominal pain. A septic screen was performed which demonstrated a white cell count rise to 15.5 (10^9/L) and a CRP of 90 mg/L, however chest x-ray, urine culture and blood cultures all remained negative. Subsequent CT scan demonstrated a mycotic abdominal aortic aneurysm in the upper abdominal aorta involving coeliac axis and superior mesenteric artery. Tazocin was initiated and the vascular surgery team was consulted. The aneurysm was not suitable for endovascular stent due to anatomic location across major visceral arteries, and major surgery for open repair deemed inappropriate , Fig. 3.Available treatment options were discussed with the patient and family. The patient was later discharged and palliated at home, dying from presumed spontaneous aortic rupture 2 weeks later.3C. Septicum being a rare cause, responsible for only 1.3% of all clostridial infections [Streptococcus Bovis endocarditis being associated with concommitant colonic malignancy in 16–62% of cases, however C. Septicum aortitis forms a distinct clinical entity [C. Septicum aortitis, with colonic malignancy or premalignant lesions identified in 71% of cases [C. Septicum vasculitis, with a 100% mortality in those who did not undergo operative treatment. In the reported case, no organism was isolated, blood cultures remained negative and an autopsy was not performed, precluding organism isolation from the tissue. Despite this, given the strong association between C. Septicum and mycotic aneurysm in patients with colonic malignancy, C. Septicum was the most probable organism.William Osler first used the term mycotic aneurysm in 1885 to describe the complications of syphilitic endarteritis in a 30 year old man leading to multiple saccular aneurysms . Presentfections ,3. Other4C. Septicum being causative in over 70% of cases with concomitant colonic malignancy. Clinicians should be aware of this clinical entity and consider it in any deteriorating patient with concomitant colonic malignancy.Mycotic aneurysm in colonic malignancy is a rare and lethal complication with The authors have nothing to disclose.Epworth Research Institute Major Research Grant No. 11.952.000.80982. This paper is not based on a communication to a society or meeting.This study was supported by This case report has been exempted from Ethics approval by Epworth Health.Singed consent was not able to be obtained, despite exhaustive attempts by the hospital team. The case report has been completely de-identified, so not to cause harm to the patient or their family. A signed statement to this effect provided by the Coloretal Surgeon, Department of Surgery.Sam Cresser: Data collection, writing the paper.Lachlan Maddock: Study concept and design, writing and review of paper.Phillip Smart: Study concept and design, writing and review paper.N/A.The Guarantor for this case report is Sam Cresser.Not commissioned externally peer reviewed.


Angioma serpiginosum is a rare vascular anomaly whose pathogenesis is still unknown. It is characterized by the onset of vascular reddish macules and papules during childhood, lesions are usually monolateral with a linear serpiginous pattern. It is rarely associated with extracutaneous findings. This entity has not yet been included in the classification of the International Society for the Study of Vascular Anomalies.We describe the first Italian report of angioma serpiginosum with a congenital symmetrical presentation. The patient had a further extension of macules during puberty involving both of the soles. No extracutaneous manifestations were present. Diagnosis was confirmed with dermoscopy and light microscopy that revealed the typical clusters of dilated, thickened and PAS+ capillaries in the upper dermis. Moreover, Immunohistochemistry showed positive WT-1 staining. Genetic analysis with next generation sequencing did not detected any mutation.Our patient presented a peculiar symmetrical and planar extension with a serpiginous linear pattern. The proliferative nature of this condition has been widely discussed in literature. In our case immunohistochemistry was positive for Wilms tumor-1, a new endothelial marker expressed during angiogenesis in reparative processes and endothelial tumors.Clinical evolution, histological and immunohistochemical findings suggest that angioma serpiginosum should be considered as a vascular proliferation. For these reasons we think it should be included in the international classification as a tumor. Angioma serpiginosum (AS) was first described by Hutchinson in 1889 . This enAS occurs at all ages, more frequently in childhood, with a female/male ratio of 9:1 . This enAS is characterized by the onset of vascular reddish macules and papules, grouped in a linear, serpiginous or gyrate pattern, sometimes on an erythematous skin , 7. The We report the case of a male child born at term from spontaneous delivery after an uncomplicated pregnancy. At birth, the newborn presented a vascular red macule on the right leg. At the age of 8, the lesions extended symmetrically to both lower limbs. The patient has been regularly followed by the paediatrician and never presented extracutaneous signs or symptoms. He has been referred to our vascular anomalies reference center at 14 years of age due to the increasing in color intensity of the lesions and the recent onset of new macules on the left forearm. Physical examination showed asymptomatic purple maculopapular lesions, with a symmetrical serpiginous linear pattern along both the lower limbs including the soles Fig. a. Based AS is a rare vascular disorder, which over the years, has been alternatively considered as a nevoid vascular malformation or a vascular neoplasm .The condition is asymptomatic and usually affects female patients, starting in childhood or early adolescence. Lesions are typically unilateral and located on the extremities with a predilection for lower limbs and buttocks . HoweverOur patient is male; he manifested macules since birth with a progressive serpiginous bilateral and symmetrical evolution, involving the lower limbs and the soles. The lesions were stable until the age of 14 years, when they spread to the upper arm. Dermoscopic features were similar to those reported in literature and they are correlated to the capillary dilation in the upper dermis –20. MultPathogenesis of AS remains still unknown. Although most cases are sporadic, both autosomal dominant and X-linked dominant inheritance have been suggested. Accounting a female preponderance and progression of lesions in pregnancy, raised levels of estrogens have been postulated in the etiology, however, further studies refuted this hypothesis by the absence of estrogen-progesterone receptors . NeumannThe proliferative or malformative nature of this entity has been widely discussed in the literature . HistoloGenetic analysis, using the panel for vascular malformations containing all genes published in the last ISSVA classification , did notTreatment is indicated at any age when AS causes psychological discomfort to the patient. 532 nm potassium titanyl phosphate (KTP) laser ad pulsed dye laser (PDL) have been successfully used in AS with excellent response , 35 Tab. In our In our opinion, on the basis of the clinical evolution, histological and immunohistochemical findings, AS should be considered as a vascular tumor, and, anyway, it should be inserted in the ISSVA classification.


Diverticular disease (DD) can have different presentations, including chronic colitis. However, diverticular disease-associated colitis (DAC) is a separate entity that can be associated with a granulomatous inflammation. DAC usually affects the left colon with no involvement of the cecum and the ascending colon. In this setting, Crohn's disease is high in the differential diagnosis. Although granulomatous colitis associated with diverticulosis has been previously described, this is the first case affecting the right colon to be reported in the English literature according to our search of the PubMed database. The patient presented with a tumor-like mass abutting the right colon that was further diagnosed as a granulomatous reaction secondary to DAC. Diverticular disease (DD) encompasses a broad scope of manifestations, including chronic colitis. Diverticular disease-associated colitis (DAC) is a distinct entity that can occasionally exhibit granulomatous inflammation. It usually affects the left colon, excluding the cecum and the ascending colon. In this setting, caution is advised to avoid an inappropriate diagnosis of Crohn's disease. Although granulomatous colitis associated with diverticulosis has been previously described, our patient is the first case affecting the right colon to be reported in the English literature based on our search of the PubMed database. He presented with a hypermetabolic tumor-like mass abutting the right colon that was further identified as a granulomatous reaction secondary to DAC.A 77-year-old man with diabetes mellitus and hypertension presented with right lower quadrant (RLQ) abdominal pain that started a few weeks prior to his presentation. Physical examination revealed a palpable RLQ mass. An abdominal computed tomography (CT) scan revealed a 5.5 cm irregular soft tissue mass abutting the ascending colon medially Figures , 2.Colonoscopy revealed diverticulosis in the sigmoid and ascending colon. On positron emission tomography (PET) scan, the mass exhibited increased metabolic activity suspicious for biological tumor activity Figure .A CT-guided biopsy revealed inflammatory cells. The patient underwent a diagnostic laparoscopy, and a fresh frozen section was inconclusive for malignancy. This was followed by a robotic-assisted right hemicolectomy en bloc with the mass. Pathology showed diverticulitis with localized suppurative granulomatous inflammation in pericolic fat Figure .The non-caseating granulomas consisted of epithelioid histiocytes with abundant eosinophilic cytoplasm and eccentric nuclei Figure . ImmunosThe patient improved clinically and was successfully discharged after few days of his surgery. http://www.nature.com/articles/ajg2017312.pdf). Recurrence rates and long-term outcomes of DAC are not well-defined and could range from a benign course to an overt IBD. More studies are needed in order to further characterize this entity [DD is a leading cause of hospitalizations in developed countries affecting 30 - 50% of individuals older than 60 years -2. DAC ds entity .Diverticular disease-associated colitis is a known but uncommon presentation of diverticular diseases. A clinical and pathologic overlap between this entity and inflammatory bowel diseases, namely, Crohn’s disease, poses a diagnostic and therapeutic dilemma in the management of affected individuals. In addition, rates of recurrence and long-term outcomes are not well-defined, ranging from a benign course to manifestations of overt IBD. More studies are needed in order to further characterize this entity.


Pyoderma gangrenosum is a rare, idiopathic, inflammatory, neutrophilic dermatitis characterized by sterile skin ulceration. It can be associated with an underlying pathology, especially inflammatory bowel disease and hematological malignancies. Its association with a malignant pathology in the context of a paraneoplastic syndrome is more commonly described in hematological malignancies, with solid tumors being rare.We report a case of a 39-year-old West African man with pyoderma gangrenosum that developed 6 months before the clinical expression of rectosigmoid junction cancer. The removal of the cancer resulted in the patient’s recovery.Recurrent pyoderma gangrenosum lesions may be the expression of colonic adenocarcinoma in paraneoplastic syndrome and require colonoscopy, especially in at-risk patients. Pyoderma gangrenosum (PG) is a rare, idiopathic, inflammatory, neutrophilic dermatosis that is generally characterized by recurrent sterile skin ulceration, with rare extracutaneous involvement –3. BecauThis case is peculiar because of the occurrence of PG lesions 6 months before the onset of rectosigmoid junction cancer symptoms and their disappearance after tumor removal. This shows that PG may be the first manifestation of colorectal adenocarcinoma in paraneoplastic syndrome.A 39-year-old West African man who was a teacher was referred for weight loss and recent abdominal pain. He presented the following symptoms: generalized episodic abdominal pain of progressive onset that was relieved by diarrheal stools occasionally associated with rectal bleeding over the past 2 months. The patient had recurrent skin lesions of different ages on the lower limbs that had started to appear 6 months before the onset of abdominal pain. These lesions began with an inflammatory nodule that resolved within a few days with a pustule that ruptured quickly and spontaneously gave way to an ulceration whose diameter quickly reached several centimeters. The healing was spontaneous and left hypertrophic stigmata.The patient was not a drinker or smoker and was of middle socioeconomic status. He had no personal or family medical history, especially cancer or blood or inflammatory disease. Upon admission, he had a body temperature of 37.4 °C, a pulse rate of 81 beats per minute, and a blood pressure of 110/85 mmHg. His physical examination revealed a moderate general condition, moderately colored conjunctiva, pain in the left iliac fossa, and hypogastrium without a palpated mass. The result of his rectal examination was normal. His dermatological examination demostrated objectified large, circumferential scarring lesions on both lower limbs and progressive ulceration on the posterior side of the left thigh (Fig. We concluded that these skin lesions were PG. Colonoscopy for the exploration of abdominal symptomatology revealed a necrotic and hemorrhagic budding lesion 20 cm from the anal margin, confirmed by contrast enema, which showed a narrowing of the lumen of the rectosigmoid junction. Biopsies taken during colonoscopy revealed an adenocarcinoma. An abdominopelvic computed tomographic scan revealed thickening of the rectosigmoid without associated metastases Fig. . The tumOur patient presented with the association of PG and adenocarcinoma of the rectosigmoid junction, which is rarely described. However, this case report is distinguished by the appearance of PG lesions before the clinical expression of cancer and their disappearance after tumor excision.et al. [et al. [et al. [PG is a dermatosis that can occur in isolation. However, in more than 75% of cases, it is associated with an underlying pathology, including inflammatory bowel disease; endocrine, hematological, and rheumatological disorders; and nonhematological neoplasia . Few caset al.  describe [et al. . However [et al. . The pat [et al. , 14. For [et al. , this isThe prognosis of PG is marked by a long-term evolution that remains unpredictable . The relFor technical reasons, immunofluorescence, although it has no diagnostic or pathogenic value, was not performed. Immunofluorescence should show immunoglobulin M and C3 deposits around the vessels at the dermal-epidermal junction.PG may be associated with rectal adenocarcinoma in paraneoplastic syndrome. In subjects at risk, early consideration is necessary, and a colonoscopy should be part of the etiological assessment in order not to miss colorectal cancer, especially when it is the recurrent ulcerative form.


Retroperitoneal desmoid-type fibromatosis (DF) is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. Cystic changes of DF have been described in the literature in association with prolonged medical treatment or abscess formation. However, spontaneous cystic change is rarely reported.Here we report the case of a 46-year-old patient with a DF mimicked a large cystic tumor in the retroperitoneum. Ultrasonography and computed tomography were performed in order to search for localizations and characteristics of the cystic tumor. Radiological findings showed an oval cystic mass with a relatively thick wall, measuring 18.3 × 12.3 × 21.5 cm in the left upper abdomen. Laparoscopic spleen-preserving distal pancreatectomy was performed and histopathological examination by immunohistochemical study enabled us to diagnose a DF invading the pancreatic parenchyma. The patient remained asymptomatic during an 8-month follow up period.We report an extremely rare case of retroperitoneal DF with spontaneous cystic change. DF can manifest as a mainly cystic mass with a thick wall, as in our case, which makes the correct diagnosis difficult. DF should be included in the preoperative differential diagnosis of a cystic retroperitoneal mass, regardless of its rarity. Desmoid-type fibromatosis (DF), also called desmoid tumor or deep fibromatosis, is an uncommon mesenchymal neoplasm composed of fibrous soft-tissue proliferation. The tumor is characterized by locally aggressive growth and frequent recurrence, although it never metastasizes . DF usuaDF usually presents as a well-circumscribed solid mass on imaging studies . Cystic We report here an unusual case of sporadic retroperitoneal DF with spontaneous cystic change mimicking a cystic tumor, including histopathologic correlations.A 46-year-old man visited the emergency department of our institution due to left lower quadrant pain and a palpable mass in the left upper abdomen. He had no specific relevant past medical history or family history. Physical examination disclosed a large, tender mass in the left abdomen. All laboratory findings were within normal ranges except a slightly increased CRP level 5.82 mg/L). Abdominal plain radiographs showed a large mass-like opacity in the left abdomen Fig. . Ultraso mg/L. AbIntra-operatively, the mass was confirmed to have arisen from the retroperitoneum, closely related to the pancreas tail. Surgeons found hemorrhagic fluid within the cystic mass. The surgical specimen was a large round lump of soft tissue measuring 13 × 10.5 × 4.3 cm in size. On gross section, the cut surface revealed a rubbery texture with a whitish to light yellowish color. Almost half of the mass was composed of a cystic space that was filled with clear, light brownish fluid. The mass was diffusely infiltrating the pancreatic parenchyma. Microscopically, the tumor was composed of uniform sheets of elongated, spindle-shaped cells in a collagenous stroma Fig. . The tumThe term “fibromatosis” describes a group of conditions that consist of fibroblastic proliferation in a collagenous extracellular matrix. Fibromatosis can be either superficial  or deep (musculoaponeurotic). In 2002, the World Health Organization (WHO) used the term ‘desmoid-type fibromatosis’ for deep fibromatosis , which iDF is more common in young women, from puberty to 40 years of age . HoweverHistologically, DF is composed of elongated, uniform spindle cells within a collagenous stroma. Although DF appears well-delineated at gross analysis and cross-sectional imaging, at the microscopic level its margins appear to infiltrate the adjacent structures. Immunohistochemically, the tumor cells are negative for CD34, CD117, and S-100 protein .DF usually appears as a well-defined solid mass on imaging studies. The ultrasonographic appearance of DF is a solid, usually well-circumscribed and hypoechoic mass of variable vascularity , 11. On Cystic changes of DF have been rarely reported and an association with prolonged medical treatment or abscess formation has been suggested , 6. HoweIn the present case, cystic DF invasion to the pancreas was confirmed by pathology. Only 13 cases have been reported of intra-abdominal DF involving the pancreas , 14. NinA multidisciplinary approach can help patients with DF receive optimal management. Stable, asymptomatic DF can be observed. However, treatment is necessary for symptomatic subjects like our patient. If feasible, surgical excision is regarded as the conventional treatment of choice. However, recurrence is common. The recurrence rate is 15–30% for intra-abdominal DF .We report an extremely rare case of retroperitoneal DF with spontaneous cystic change. It can manifest as a mainly cystic mass with a thick wall, as in our case, that makes it difficult to reach a correct diagnosis. DF should be included in the preoperative differential diagnosis of a cystic retroperitoneal mass, regardless of its rarity.