Primitive myxoid mesenchymal tumor of infancy: Case report and review of the literature.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an extremely rare soft tissue tumor with only a few cases. Herein, we report a case of 5-months-old girl presenting with a soft tissue mass on the sole of the left foot that recurred 2months after a first resection. The Doppler ultrasound imaging showed abundant blood flow in the tumor. Magnetic resonance imaging (MRI) showed a well demarcated tumor on the sole of the left foot. The surgically resected tumor consisted of primitive mesenchymal tumor cells dispersed in a myxoid background with delicate small blood vessels. Immunohistochemically, tumor cells expressed vimentin but were negative for AE1/AE3, Desmin, Muscle-Specific Actin, MyoD1, Myogenin, CD34, S-100 protein and CD99. Fluorescence in situ hybridization (FISH) showed absence of chromosomal translocation involving SYT and ETV6. Thus, the patient was diagnosed with a primitive myxoid mesenchymal tumor of infancy. To our knowledge, this is the first case of PMMTI affecting the sole of the foot.

Cellular angiofibroma of the vulva: a poorly known entity, a case report and literature review.

BACKGROUND: Cellular angiofibroma represents a newly described, site specific tumor. Histologically, CAF is a benign mesenchymal neoplasm characterized by two principal components: bland spindle cells and prominent small to medium-sized vessels with mural hyalinization. The indolent nature of the lesion is underscored by the uniformity of its constituent stromal cells, and their lack of nuclear atypia. Characterization by immunohistochemistry is helpful distinguishing Cellular angiofibroma from other mesenchymal lesions. CASE PRESENTATION: We report the case of a 37-year-old woman, presenting with a painless nodule involving the vulva. This lesion had gradually increased in size; a simple excision was performed, and follow up was unremarkable. Gross examination showed a well circumscribed, firm tumor measuring 3× 3 × 2,5 cm. Histologically, the tumor was composed of uniform, short spindle-shaped cells, proliferating in an edematous to fibrous stroma and numerous small to medium-sized thick-walled vessels. A panel of immunohistochemical stains was performed, and confirmed the diagnosis of Cellular angiofibroma. CONCLUSION: In this report we aim to describe the clinical, pathological and immunohistochemical features of this rare entity through a literature review, and to discuss other vulvar mesenchymal lesions.

Synovial hemangioma: a rare case of knee joint tumefaction in children.

Synovial hemangioma is a rare benign vascular malformation. We report a case of chronic swelling in the left knee of a 7-year-old boy. An intra-articular tumor of synovial origin could was detected after a magnetic resonance imaging scan. Surgical excision of the mass associated with partial synovectomy was carried out. A histologic examination confirmed the diagnosis of synovial hemangioma. After 36 months of follow-up, there was no sign of recurrence. We review the literature and discuss the current case, along with noting the diagnostic methods and the therapeutic aspects of this rare and benign lesion.

A Sertoli-Leydig ovarian tumor presenting as ovarian torsion: A case report.

Torsion occurs as a complication in 10% of cases of ovarian tumors. It predominantly occurs in benign ones, while malignant tumors are less prone to torsion. Sertoli-Leydig cell tumors are highly unusual sex cord-stromal tumors of the ovary, accounting for less than 0.2% of all ovarian cancers. A 39-year-old patient presented to the emergency department with a Sertoli-Leydig cell tumor diagnosed due to ovarian torsion. The clinical presentation was characterized by abdominal pain. Ultrasound indicated signs of torsion, and torsion of the right ovary was subsequently confirmed during laparotomy. A salpingo-oophorectomy was performed, and histological examination revealed a moderately differentiated Sertoli-Leydig cell tumor. Sertoli-Leydig cell tumors often present with hormone-related or non-hormonal symptoms. Surgery plays a crucial role in both diagnosis and treatment. Postoperative treatment is not necessary for well-differentiated Sertoli-Leydig cell tumors in stage IA-IB. However, patients with grade 2-3 disease, advanced stage, or heterologous elements may consider adjuvant treatment. As these tumors are rare, this case contributes to the documentation of Sertoli-Leydig cell tumors, with a case diagnosed due to ovarian torsion. The case highlights the importance of establishing international registries of Sertoli-Leydig cell tumor cases for standardized management.

Angiomyofibroblastoma of the vulva: A case report and review of the literature.

Angiomyofibroblastoma (AMFB) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region. It is usually diagnosed in middle-aged women. Histopathology and immunohistochemical study remain the key to diagnosis. Like other benign mesenchymal vulval tumors, AMFB shows indolent behavior and rarely recurs after complete surgical excision. Herein, we present a case of vulvar AMFB in a 51-year-old woman to highlight the diagnostic difficulties when considering this rare entity.

A Dental Extraction Revealing a Multisystem Burkitt's Lymphoma: A Case Report.

Burkitt's lymphoma is rare but highly aggressive and very fast-growing B-cell non-Hodgkin's lymphoma (NHL). It can affect any organ such as the central nervous system, jaw, intestines, kidneys, ovaries, and other organs. It results from the malignant evolution and proliferation of B-type lymphoid cells. The diagnosis is based on a biopsy of a tumor mass or bone marrow aspiration revealing the presence of tumor cells. We report the case of a 7 year old child who was referred for a gingival swelling evolving since 1 month following a dental extraction. Imaging and anatomopathological examination after biopsy concludes to a multi systemic Burkitt's lymphoma. A chemotherapy was immediately started with spectacular complete remission.

Massive cellular angiofibroma of the vulva: a case report.

Cellular angiofibroma is a rare benign mesenchymal tumor, typically occurring in the vulvar region of middle-aged women. This report highlights the importance of histological analysis in diagnosing this uncommon condition and emphasizes its benign nature and straightforward management. We present a case of a 58-year-old North African woman who had a large, well-defined mass in the left labia majora, which had been evolving over 2 years. MRI confirmed the resectability of the tumor by delineating its boundaries. The tumor, despite its slow growth leading to delayed diagnosis, was effectively treated with wide surgical excision. Diagnosis was confirmed through histological and immunohistochemical evaluations, revealing spindle cell proliferation with thick-walled vessels. Cellular angiofibroma, although initially alarming due to its size, is generally managed successfully with surgery and prognosis is favorable with a low risk of recurrence.

Canine transmissible venereal tumor in Morocco: Clinical and pathological findings in 64 dogs-insights from a descriptive epidemiological study (2020-2023).

Background: Canine transmissible venereal tumor (CTVT) is a widely spread, contagious neoplasm commonly found in dogs. Mostly affects the external genitalia, however, it may also exhibit unusual clinical presentations. Aim: To describe the epidemiology, clinical appearance, cytologic and histopathologic features of dogs with TVT in Morocco. Methods: Within the realm of a nation-wide study on canine and feline tumors in Morocco between September 2020 and March 2023, dogs with histologically diagnosed TVT were identified and data on epidemiologic, clinical as well as cytologic, and histologic features were compiled and analyzed. Results: A total of 64 cases of canine TVT were diagnosed. 52 dogs were cross-breed (81.2%) while 4 Siberian Huskies (6.2%) and 3 German shepherds (4.7%) were the most affected pure-breed dogs. The median age of dogs at diagnosis was 3 years (range, 1-10years) and male gender was more common (male:female ratio; 1.3:1). Tumor was located exclusively in the genital area in 58 cases (90.6%), whereas 6 dogs (9.4%) had an atypical occurrence of TVT with locations including skin and nasal cavity. Cytology allowed for an early diagnosis in 2 cases. Histology revealed no differences between the genital and extragenital forms. Immunohistochemistry was necessary in 4 cases and revealed positive staining for vimentin and Alpha-1-antitrypsin, negative marking for CD3, CD20, and AE1/AE3, and low cytoplasmic labeling for lysozyme. Conclusion: CTVT is a widely distributed neoplasm in Morocco, mostly showing presence in young, cross-breed, and oftentimes stray dogs. An adequate understanding of this tumor's epidemiological features is necessary for its management and eradication.

Bilateral Nephroblastomatosis With a Unilateral Wilms Tumor: A Case Report Highlighting Imaging Characteristics.

Nephrogenic rests (NRs) are foci of embryonic nephrogenic cells that persist beyond the 36th week of gestation. They are precursor lesions of Wilms tumor and are found incidentally in approximately 1% of infants. The term nephroblastomatosis (NBS) is utilized when nephrogenic rests extensively or multifocally affect the kidneys. We report a case of a left nephroblastoma with bilateral nephroblastomatosis, treated with neoadjuvant chemotherapy followed by a left nephrectomy, highlighting the imaging features of nephrogenic rests on ultrasound, CT, and MRI, and the potential possibilities of distinguishing nephroblastomas from nephrogenic rests in one hand, and the possibilities of distinguishing active nephrogenic rests from inactive ones in the other hand. We furthermore discuss the role of histology in making those distinctions, and the role of chemotherapy in reducing nephrogenic rests' size and cellularity.

Primary Ewing sarcoma of the kidney: a rare entity with diagnostic challenges.

Ewing sarcoma is a very rare tumour with aggressive behaviour and a poor prognosis. It tends to metastasize rapidly. Renal Ewing sarcoma is extremely rare, and only 48 cases have been reported in the literature. Herein, we report the case of a 14-year-old female presenting with a painful left flank swelling. Ultrasound and magnetic resonance imaging showed a large tumour invading the left kidney, heterogeneously enhanced after injection, associated with lymph nodes and peritoneal carcinomatosis. A thoraco-abdomino-pelvic computed tomography scan revealed pulmonary nodules and osteolytic lesions. A biopsy was performed, and histology, immunohistochemistry, and molecular studies confirmed the diagnosis of retroperitoneal Ewing sarcoma. Multi-agent chemotherapy followed by radical nephrectomy was performed, confirming the renal origin, and histology showed a post-therapeutical response. After a 1-year follow-up, there was no evidence of recurrence. We report this case to highlight the rarity of this entity and its challenging clinico-pathological diagnosis when presenting as a renal tumour.

Pathophysiological, immunogenetic, anatomopathological profile of thrombophilia in pregnancy.

Thrombophilic states have been associated with early and/or late pregnancy loss and possibly other severe obstetrical complications. Pregnancy-induced hypercoagulability, increased stasis, and the consequences of inherited and acquired thrombophilia are just a few of the factors that contribute to the development of thrombosis in pregnancy. In this review, we illustrate the impact that these factors have on the development of thrombophilia during pregnancy. We also explore how thrombophilia impact pregnancy outcomes. Next, we discuss how human leukocyte antigen G plays a part in thrombophilia during pregnancy by regulating cytokine release to prevent trophoblastic cell invasion and maintain local immunotolerance constant. Human leukocyte antigen class E is briefly explored with thrombophilia in pregnancy. Regarding the anatomopathologic aspect, we describe the different histopathological lesions of the placenta found in women with thrombophilia.

A rare case of renal tumor in children: Clear cell sarcoma with an unusual presentation.

Clear cell sarcoma of the kidney is the most frequently misdiagnosed renal tumor in children. We report the case of a 6-year-old boy with clear cell sarcoma of the kidney with an unusual presentation, including a primary tumor of the left kidney with metastasis in the homolateral psoas muscle. The renal tumor was revealed by abdominal mass without hematuria. In a review of the literature. Clear cell sarcoma of the kidney is most commonly associated with bone and lung metastases. Muscular metastasis at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of clear cell sarcoma of the kidney. This also illustrates clear cell sarcoma of the kidney's ability to metastasize to other sites including the muscular. These tumors present a diagnostic challenge for the radiologist who should be aware of this entity to differentiate it from other renal tumors which are more frequent at this age. We aim to report the clinical, radiological features, and pathological presentation of this entity.

Mesenchymal Hamartoma With Elevated Alpha-Fetoprotein: A Diagnostic Pitfall.

Mesenchymal hamartoma (MH) is a benign liver tumor accounting for 3% to 8% of all liver tumors in children, commonly manifesting before 3 years of life. Distinguishing MH from hepatoblastoma and other liver tumors relies on imaging and alpha-fetoprotein (which is usually within normal range in MH), before histologic examination. We report a case of a hepatic MH associated with elevated alpha-fetoprotein, leading to a misdiagnosis of hepatoblastoma and the administration of chemotherapy. We draw the attention to the diagnostic difficulty and pitfalls related to alpha-fetoprotein elevation in the setting of a liver tumor, and we highlight the importance of imaging and histology in establishing the diagnosis.

Non Hodgkin Lymphoma Among Children: Pathological Aspects and Diagnostic Challenges.

Non-Hodgkin lymphoma (NHL) are common malignancies in children. Available data on clinico-pathological aspects of pediatric NHL in developping countries are limited and diagnostic approach appears more delicate with absence of molecular studies. The objectives of our study are: analyzing the pathological spectrum of NHL among children and highlighting challenges in the diagnosis including: limited biopsic material; unususal subtyptes, age group, or localization. We retrospectively analyzed clinico pathological characteristics of 101 NHL's cases among children diagnosed in the Pediatric's pathology unit over a period of 4 years There were 78 (77.2%) male and 23 (22.8%) female. The median age was 7.2 years. The most common histologic subtypes of NHL were Burkitt lymphoma in 65 patients (64.4% ); followed by lymphoblastic lymphoma in 22 patients, large B-cell lymphoma in 9 patients ( 8.9%); anaplastic T cell lymphoma in 3 patients; NOS mature T cell lymphoma and pediatric type follicular lympoma in 1 patient each. In conclusion, this study Morocco illustrates the pattern of distribution of NHL and emphasizes challenges in the diagnosis of these neoplasms.

Hyperphosphatemic familial tumoral calcinosis mimicking a cystic hemo-lymphangioma on MRI.

A tumoral calcinosis is a rare benign pathology characterized by calcium deposits (calcium phosphate crystals) in the periarticular soft tissues, giving a truly pseudotumor appearance. The same patients with tumoral calcinosis may have manifestation of hyperostosis hyperphosphatemia syndrome. The association is called Hyperphosphatemic familial tumoral calcinosis which is the case with our patient. We present a unique case of a 10-year-old female child without any notable history. No notion of consanguinity, a non-painful swelling of the right elbow for the last 3 years. She was presented with tumoral calcinosis in the context of familial hyperphosphatemic calcinosis tumor in which the diagnosis of lymphangioma was evoked and then redressed.

Nephroblastoma of the Big Child, a Rare Entity: About a Case.

Nephroblastoma is a renal blast tumor, the most common malignant renal tumor in children between 1 and 5 years of age. The average age of onset is 3.5 years, rarely occurring in children over 10 years of age. Its treatment is a model of medical-surgical collaboration. The prognostic factors are mainly the stage but recent studies have also shown that the advanced age of the child is a negative prognostic factor. We report a case of a 14-year-old child who presented with pain with swelling of the right hypochondrium, ultrasound showed a heterogeneous right retroperitoneal mass, MRI showed a large retroperitoneal tumor process with a right renal origin, in heterogeneous T2 signal, in T1 hyposignal, diffusion restricted, containing necrotic areas and heterogeneously enhancing after injection, responsible for pyelocalic dilatation and right renal venous thrombosis, extended to the IVC and the right atrium, with adenopathies, suggesting first a sarcoma. The extension workup showed pulmonary nodules of secondary appearance. An echo-guided biopsy was performed and the anatomopathological study confirmed the diagnosis of nephroblastoma.

Congenital hepatic fibrosis: case report and review of literature.

Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease derived from biliary dysgenesis secondary to ductal plate malformation; it often coexists with Caroli's disease, von Meyenburg complexes, autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). Although CHF was first named and described in detail by Kerr et al. in 1961. Its pathogenesis still remains unclear. The exact incidence and prevalence are not known, and only a few hundred patients with CHF have been reported in the literature to date. However, with the development of noninvasive diagnostic techniques such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), CHF may now be more frequently detected. Anatomopathological examination of liver biopsy is the gold standard in diagnosis of CHF. Patients with CHF exhibit variable clinical presentations, ranging from no symptoms to severe symptoms such as acute hepatic decompensation and even cirrhosis. The most common presentations in these patients are splenomegaly, esophageal varices, and gastrointestinal bleeding due to portal hypertension. In addition, in younger children, CHF often is accompanied by renal cysts or increased renal echogenicity. Great variability exists among the signs and symptoms of the disease from early childhood to the 5th or 6th decade of life, and in most patients the disorder is diagnosed during adolescence or young adulthood. Here, we present two cases of congenital hepatic fibrosis in 2-years-old girl and 12-year-old male who had been referred for evaluation of an abdominal distension with persistent hyper-transaminasemia and cholestasis, the diagnostic was made according to the results of medical imaging (CT or MRI), a liver biopsy, and genetic testing.

Primary mature adrenal teratoma in infant.

Teratomas are neoplasms of the embryonic tissues that typically arise in the gonadal and sacrococcygeal regions. Primary adrenal teratoma are extremely rare and only few cases were published in literature. Teratomas contain more than one embryonic germ cell layer, mostly elements derived from ectoderm and least frequently from endoderm. Though these tumors are mostly benign, malignant transformation may occur. Treatment includes surgical removal. We report a rare case of a primary mature retroperitoneal teratoma in an infant with liver metastasis. Imaging modality CT and MRI were useful in diagnosis. The diagnostic and therapeutic challenges of dealing with such a case have been discussed and the literature reviewed.

[Histopathological lesions of the placenta associated with severe intrauterine growth retardation: about a clinical case]. / Les lésions histopathologiques du placenta au cours de retard de croissance intra utérine sévère: à propos d'un cas clinique.

The examination of the placenta is part of the assessment of intrauterine growth retardation (IUGR). Its interest lies in retardation etiology research and in maternal-fetal consequences which can arise from it as well as in the implementation of preventive strategies for subsequent pregnancies in patients with recurrent diseases. We conducted a study of patients with severe IUGR monitored in the Department of Neonatology of the Ibn Sina University Hospital in Rabat in order to identify possible anatomopathological lesions in the placenta.

Intracardiac Teratoma in an Infant: Report of a New Case and Literature Review.

Primitive intracardiac tumours are rare, especially in childhood, and are often discovered on autopsy. The intracardiac teratoma is the rarest intracardiac tumours of childhood. Herein, we report the case of an 11-month-old infant, which featured recurrent bronchoalveolitis since the age of 3 months, with a thoracic deformation. Physical examination did found discrete respiratory distress signs. Chest radiography showed large mediastinal enlargement. The computed tomography showed a solid cystic-cloisonned mass with fat and central calcification highly suggestive of an intracardiac teratoma. A radical surgical excision was made and the histological examination found a well circumscribed tumour containing elements of the three germ layers confirming the diagnosis of mature well-differentiated teratoma, with no need of immunohistochemical support.