Premature Puberty Revealing an Ovarian Tumor in a Five-Year-Old Girl

Background: Ovarian tumors in the girl child are sometimes revealed by the development of secondary sexual characteristics. The authors report the case of a five-year-old girl in whom the disease was revealed by early puberty. Case presentation: A five-year-old girl with an enlarged abdomen for about four months. The onset of pain and the sensation of a mass prompted the consultation. The development of secondary sexual characteristics (SSC) noted by the family had not been mentioned. The patient was classified as pubertal stage 2 according to the Tanner classification. An abdominal ultrasound and a CT scan showed a large left ovarian mass, an enlarged uterus for the patient’s age and a normal right ovary. The hormonal workup was not contributive. The treatment consisted only of a left salpingo-ovarectomy, without complementary chemotherapy. Anatomic pathological examination of the surgical specimen concluded to a juvenile tumor of the granulosa. The evolution was good with a beginning of regression of the HSC one month after the ovarectomy. Discussion: Granulosa tumors are sometimes secretory cancers, generally with a low potential for malignancy and therefore a very good prognosis. Surgery based on total adnexectomy is the first-line treatment. The large size of the tumor, the presence of ascites and capsular rupture are factors of poor prognosis, hence the importance of early diagnosis. Conclusion: Routine comprehensive physical examination should be de rigueur for abdominal masses in girls, especially in the context of various beliefs that may impede early referral to care.

Crohn's disease-related'gastrocnemius myalgia syndrome'successfully treated with infliximab:A case report

BACKGROUND Extra-intestinal manifestations in inflammatory bowel diseases(IBD)are frequent and involve virtually all organs.Conversely,the clinical characteristics and course of inflammatory myopathies in IBD remain poorly described and mostly related to orbital myositis.Moreover,alternative therapeutic strategies in non-responder patients to corticosteroid therapy must still be clarified.CASE SUMMARY A 33-year-old woman with a history of unclassified colitis presented with acute bilateral calf pain.On admission,her clinical and biological examinations were non-specific.However,magnetic resonance imaging showed bilateral inflammatory changes in gastrocnemius muscles suggestive of myositis.Muscle biopsy confirmed the diagnosis of myositis and demonstrated an inflammatory infiltrate mainly located in the perimysial compartment including lympho-plasmocytic cells with the formation of several granulomatous structures while the endomysium was relatively spared.The combined clinical,biological and histomyopathological findings were concordant with the diagnosis of‘gastrocnemius myalgia syndrome’(GMS),a rare disorder associated with Crohn’s disease(CD).Ileocolonoscopy confirmed CD diagnosis and systemic corticosteroids(CS)therapy was started,resulting in a rapid clinical improvement.During CS tapering,however,she experienced a relapse of GMS together with a severe active ileocolitis.Infliximab was started and allowed a sustained remission of both conditions at the latest follow-up(20 mo).CONCLUSION The GMS represent a rare CD-associated inflammatory myopathy for which anti-tumour necrosis factor-αtherapy might be considered as an effective therapeutic option.

Cutaneous Melanoma:A Single Center Experience in Brazil

The aim is to evaluate the profile of cutaneous melanoma in rural and urban areas of the State of Espírito Santo and compare the results between them.The information collected included gender and age at diagnosis,city of residence,tumor morphology,anatomic site,histological subtype,level of invasion,tumor thickness and staging.A retrospective observational study was conducted,using the Pathology Department’s database of Hospital Universitário Cassiano Antonio de Moraes(HUCAM).Statistical analysis was performed using SPSS Statistics.A total of 258 histopathological reports of Cutaneous Melanomas were identified between 2003 and 2016.This skin cancer was more common among women,in the age group from 50 to 79 years.The majority of lesions was localized in the malar region of the face,usually classified as in situ or with superficial invasion and presented Breslow’s thickness less than 1 mm.Regarding the diagnosis,the male population was older and had more aggressive melanomas with higher rates of Clark’s levels IV and V and Breslow’s thickness when compared to the female group.There was a great concentration of patients in the central mesoregion(173 cases—67.06%),probably because this is the most populous regional area of Espírito Santo.The prevalence of melanoma between 2003-2016 was higher in the rural area(21.52 cases/100,000 habitants)when compared to the urban area(4.15 cases/100,000 habitants).

Relevance of Protein Content within the Renal Scaffold for Kidney Bioengineering and Regeneration

Chronic kidney disease is currently a major public health problem around the world. Although hemodialysis increases survival of patients with end-stage renal disease, kidney transplantation remains the only potentially curative treatment. However, transplantation as a therapeutic option is limited by availability of suitable donor organs. This situation highlights the urgent need to find new and potentially inexhaustible sources of transplantable organs. Perfusion decellularizarion of whole organs is a novel approach to organ engineering and regeneration. In the present research, we used a continuous perfusion decellularization protocol to eliminate cellular componet of kidney and evaluated residual scaffold components after decellularizarion process by proteomics analysis. Our proteomic data show that this protocol results in incomplete removal of cellular proteins. However, unlike other authors, we assume that proteins retained within decellularized kidney scaffold could be the basis for specific homing and celular differentation in the recellularization process.

DLST mutations in pheochromocytoma and paraganglioma cause proteome hyposuccinylation andmetabolic remodeling

Dear Editor,Of all human tumors,pheochromocytomas and paragangliomas(PPGLs)have the highest heritability rate.Over 15%of PPGLs harbor mutations in genes encoding tricarboxylic acid(TCA)cycle-related enzymes that cause oncometabolite accumulation and drive tumorigenesis via metabolic adaptation to hypoxia and global hypermethylation[1].The dihydrolipoamide S-succinyltransferase(DLST)gene was recently described as a new PPGL susceptibility gene[2].