A rare clinical case of systemic AA amyloidosis with cardiac involvement complicating ankylosing spondylitis: a case report.

BACKGROUND: Ankylosing spondylitis (AS) is a type of chronic inflammation that is most prevalent in young adults and is characterized by an inflammatory enthesiopathy that gradually develops toward ossification and ankylosis. If inflammation is left unchecked, it can potentially lead to complications such as secondary amyloidosis, also known as AA amyloidosis, involving the deposition of amyloid serum A protein. Our case presents with a thyroid localization of AA amyloidosis which is secondary to this AS. Such a case has been described in only four cases in the literature. Cardiac localization of AA amyloidosis has been exceptionally described in the literature. CASE PRESENTATION: We report the case of a young patient with severe AS complicated by secondary amyloidosis with thyroid, cardiac, and probably renal localization. He was treated with anti-TNF therapy, and his condition improved significantly. CONCLUSIONS: Our case presents several localizations of AA amyloidosis secondary to this AS. Although cardiac involvement is rare in secondary AA amyloidosis, it should always be screened for, even in a cardiacly asymptomatic patient.

Ki-67 proliferation index to further stratify invasive breast cancer molecular subtypes: Northern African comparative cohort-study with external TCGA-BRCA and METABRIC validation.

Introduction: breast cancer (BC) is a malignancy with very high incidence and mortality in Africa, especially in Western Africa, where more than 25 thousand deaths are registered every year. Not all BC have the same prognosis, and being able to personalize treatment and predict aggressiveness is of crucial importance. The purpose of our study is to explore further subdivisions associated with prognosis, beyond breast cancer molecular classification that is routinely established in pathology departments. Methods: we conducted a 5-year retrospective cohort study on 1266 invasive BC of Moroccan patients, collected at the Pathology Department of Ibn-Rochd University Hospital in Casablanca, and followed at King Mohammed VI National Centre for the Treatment of Cancers. We elaborated an Estimation-Maximization Clustering, based on the main BC biomarkers: Ki-67, HER2, estrogen and progesterone receptors, evaluated by immunohistochemistry. Two independent datasets (TCGA-BRCA and Metabric) were also analyzed to assess the external reproducibility of the results. Results: each molecular subgroup could be partitioned into two further subdivisions: Cluster1, with average Ki-67 of 16.26% (±11.9) across all molecular subgroups and higher frequency within luminal BC, and Cluster2, with average Ki-67 of 68.8%(±18) across all molecular subgroups and higher frequency in HER2 as well as in triple-negative BC. Overall survival of the two Clusters was significantly different, with 5-year rates of 52 and 37 months for Custer1 and Cluster2, respectively (p=0.000001). Moreover, mortality rates within the same molecular subgroup, especially in luminal B HER2-, varied remarkably depending on Cluster membership (6% for C1 and 18% for C2 after 1 year of follow-up). Two different algorithms to evaluate the prognostic importance, variable selection using random forests (VSURF) and Minimal depth, ranked the subdivision proposed as one of the 4 most influential features being able to predict patient survival better than several histoprognostic features, both in the Moroccan and in the external datasets. Conclusion: our results highlight a new refinement of the BC molecular classification and provide a simple and improved way to classify tumors that could be applied in low to middle-income countries. This is the first study of its kind addressed in an African context.

Bone choristoma of the gingiva: A case report.

INTRODUCTION: Bone choristoma is a benign tumor with normal histology and ectopic location. This paper aims to report a rare case of bone choristoma in the maxillary gingival location. OBSERVATION: The authors report a case of a 39-year-old woman, with a history of maxillary full edentulousness, who consulted for a slight pain evolved for about six months, triggered by movements on the lesion. Clinical examination found growth at the level of the right maxillary gingival alveolar ridge. Surgical biopsy was indicated and performed under local anesthesia. The histological examination of the excisional specimen concluded at a bone choristoma. The patient had a good evolution after the surgical removal. CONCLUSION: Choristoma is a rare and benign condition. The management is surgical.

The solitary plasmacytoma of mandible: A case report.

BACKGROUND: the solitary plasmacytomas entities characterized by the neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal immunoglobulin. It represents less than 5% of plasma cell dyscrasias. The most common sites of solitary plasmacytomas are long bones. The jaws location remains extremely rare, only 4.4% of solitary plasmacytomas of bone occur in the mandible, the diagnosis is based on the biopsy evidence of plasma cell proliferation and absence of evidence of involvement of other bones. CASE PRESENTATION: The authors report the case of a healthy 49-year-old man with no general history, presented with a painless slow-growing lesion of the left jaw that had persisted and increased in size for one month. Clinical examination revealed a large lesion 4 × 4 cm with irregular borders of the retro-molar area on the left jaw, infiltrated into underlying tissue, with sensory disturbances and facial asymmetry. CONCLUSION: Treatment methods of plasmacytomas of the jaw include local surgery (curettage of the lesion), local irradiation, systemic chemotherapy, or a combining therapy. Early diagnosis and treatment are crucial for better resolution of the disease.

Primary Malignant Deciduoid Mesothelioma: A Challenging Diagnosis.

Primary malignant deciduoid mesothelioma is a rare subtype of epithelioid mesothelioma that was first described in the peritoneum in young women without a history of asbestos exposure. It was thought to be a distinct clinicopathologic entity with ominous prognosis; recent studies have better characterized this entity. On morphology, primary malignant deciduoid mesothelioma is characterized by cytomorphologic features resembling decidualized tissue. Pleomorphism is variable. The immunoprofile is similar to other epithelioid mesotheliomas. The prognosis is the same as other epithelioid mesotheliomas and seems to depend on histological grade.

[Astroblastoma: A rare glial tumor]. / L'astroblastome : une tumeur gliale rare.

Astroblastoma is a rare neuroepithelial tumor most commonly seen in children and young adults. Due to its rarity, this tumor can be easily misdiagnosed as its classification, histogenesis and therapeutic management are still being discussed. We report the case of a 21 year old man, who presented at the Emergency Room for loss of consciousness. He reported a history of headaches, vomiting and decreased visual acuity. The CT and MRI showed a left temporoparietal solid-cystic mass with heterogeneous enhancement and perilesional edema. The patient underwent a total mass resection. On histopathological examination, tumor cells were organized in perivascular pseudorosettes which are typically encountered in astroblastoma, without neither necrosis nor endothelial hyperplasia. They had broad processes and rounded nuclei without any mitotic activity. Immunochemistry stains confirmed the diagnosis by showing a positive reactivity for GFAP, EMA, vimentin and S100. Astroblastoma is a rare glial tumor of uncertain origin. Clinical presentation and imaging are nonspecific. Therefore, its diagnosis is based on histopathologic findings: typical perivascular pseudorosettes. However, similar histological pattern may be seen in other glial neoplasms. In the 2016 WHO Classification, astroblastoma is among the "other glial neoplasms" without a grading system. So far, there are no reliable prognosis factors for this tumor; however, two entities have been described: well differenciated astroblastoma (considered as low grade) and anaplastic/malignant astroblastomas (considered as high grade). Gross total resection is the treatment of choice for astroblastomas. Adjuvant therapy is still controversial. This case illustrates a cerebral tumor which is rarely encountered in practice and that can cause diagnostic problems and subsequently, inadequate treatment.

[Primary colonic extranasal NK/T-cell lymphoma: about a case]. / Lymphome T/NK extra-nasal à localisation colique primitif: à propos d'un cas.

To better understand this cancer, we here report the case of a 43-year old patient diagnosed with localized and isolated primary colonic NK/T-cell lymphoma without associated enteropathy, treated wih 3 cycles of AspaMetDex with a poor response who died during treatment with a clinical picture of acute abdomen. Primary intestinal NK/T-cell lymphoma most commonly affects the young subject with poor prognosis. It is difficult to distinguish between intestinal NK/T-cell lymphoma and inflammatory or infectious intestinal disorders because of its non-specific clinical and endoscopic features. The histopathological and immunohistochemical data as well as the study of DNA allow to adjust the diagnosis and to classify this lymphoma according the European Enteropathy type T-cell lymphoma (ETL).

[Primary hodgkin lymphoma of thyroid: case report]. / Lymphome de hodgkin primitif de la thyroïde: à propos d'un cas.

Primary thyroid lymphoma is a rare clinical entity, which does not exceed 5% of the diagnosed lymphomas, occur more frequently in women than in men, with a peak incidence in the sixth decade of life. The relationship with chronic thyroiditis is well known. The Hodgkin subtype even rarer; little described in the literature; Posing a diagnostic problem. Diagnostic confirmation is usually carried out on the surgical specimen. To better understand this entity, we report the case of a 64-year-old patient, with no notion of chronic thyroiditis, admitted for Hodgkin's lymphoma of the thyroid, diagnosed on an anterior cervical mass. Thyroidectomy with histopathological and immunohistochemical studies confirmed the diagnosis. The patient had received chemotherapy type ABVD (Adriblastin-Bleomycin-Vinblastine-Dacarbazine) and programmed for radiotherapy.