Papillary fibroelastoma revealed by an acute coronary syndrome with transient ST segment elevation: a case report.

Cardiac papillary fibroelastoma is a rare, benign tumour, arising from the valvular endocardium, which could lead to life-threatening complications as myocardial ischemia. We report a case of a 54-year-old male patient who presented in the emergency room with an acute coronary syndrome with transient ST segment elevation. After ruling out coronary artery disease by coronarography, we established the diagnosis of papillary fibroelastoma by performing echocardiogram completed by computed tomography angiography. The reversible acute coronary syndrome has been caused by the prolapse of pedunculated coronary cusp tumour into the main left coronary ostium. The patient was scheduled for emergent surgery. The surgical management included a complete resection of the tumour sparing the aortic valve. The patient recovered well. A papillary fibroelastoma of the aortic valve can be revealed by an acute coronary syndrome with transient ST segment elevation. More investigations must be done to eliminate such diagnosis in the case of a normal coronarography.

Endoscopic Resection of a Large Colonic Lipoma Simulating a Tumor Revealed by Hemorrhage: A Case Report and Literature Review.

Lipomas of the colon are benign tumors of the digestive tract. They are usually asymptomatic, and often discovered incidentally during a colonoscopy. However, lipomas larger than 2 cm may present with abdominal pain, bowel changes, and rectal bleeding. They may mimic cancer, depending on multiple factors including tumor size, location, and complications, which often makes preoperative diagnosis difficult. In this report, we discuss the case of a 34-year-old woman who presented with paroxysmal abdominal pain in the left iliac fossa withmoderate hematochezia that had been evolving for six months. The patient denied melena or hematemesis, and she had no significant medical history. Colonoscopy revealed a large polyp of over 5 cm located 40 cm from the anal margin. She underwent endoscopic resection without complications. The histological examination confirmed the lipomatous nature. An accurate preoperative diagnosis of lipomas is necessary.It can often be difficult to choose between endoscopic and surgical treatment. The choice of treatment depends on the size and location of the tumor and complications. Endoscopic resection may obviate the need for surgery and can potentially reduce surgical morbidity. We aimed to report and discuss the management of this patient who underwent endoscopic resection for a large mass with a definitive pathology of colonic lipoma.

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Idiopathic portal hypertension (IPH) has been reported rarely in POEMS syndrome. The precise etiopathogenesis of IPH is not fully elucidated. We report a 46-year-old male patient presenting with POEMS syndrome. He presented postprandial vomiting and left tinnitus. Loss of appetite, leanness, and excessive sweat were concomitant symptoms. Abdominal examination revealed splenomegaly and an absence of hepatomegaly. Abdominal ultrasonography was therefore performed, revealing hepatosplenomegaly with dilatation of the splenic vein. An abdominal computed tomography confirmed the presence of an 18.5 cm splenomegaly with dilatation of the splenic and portal vein. Upper endoscopy with biopsy showed minimal, non-atrophic, mildly active chronic, follicular antrofundic gastritis without esophageal varices. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with IPH. The patient initially received six courses of the CTD (cyclophosphamide, thalidomide, and dexamethasone) protocol. He subsequently proceeded to an autologous stem cell transplant (ASCT), and the patient achieved a considerable improvement. POEMS syndrome could be complicated with IPH. There are only a few cases of IPH associated with POEMS syndrome in the literature. This case highlights the manifestation of portal hypertension in POEMS syndrome.

Kikuchi-Fujimoto's Disease or Histiocytic Necrotizing Lymphadenitis Following mRNA COVID-19 Vaccination: A Rare Case.

Kikuchi-Fujimoto disease (KFD), also known as necrotizing histiocytic lymphadenopathy, is a rare and benign lymph node disorder that mainly occurs in young women. It is clinically characterized by fever with tender and painful cervical lymphadenopathy mostly; however, all areas of lymph nodes can be involved. This disorder is often mistaken for malignant lymphoma or infection. The precise pathophysiology of KFD remains unknown, but it is theorized that it may be post-viral or associated with an autoimmune disease. The diagnosis is based on the histological analysis of the excised involved lymph node. The treatment is mainly supportive with favorable outcomes within a few weeks or months. In this case, we present a 24-year-old woman without a past medical history, who consulted for painful bilateral cervical lymphadenopathy associated with fever that has been evolving for one month following the coronavirus disease 2019 (COVID-19) vaccination. The initial diagnostic workup was performed and the diagnosis of KFD was confirmed based on the histopathological findings of the excised lymphadenopathy. Therapeutic management was based on oral corticosteroid treatment with clinical and radiological improvement after a few days without recurrence during follow-up. This article aims to report a rare case of KFD in a patient after receiving the messenger ribonucleic acid (mRNA)-based COVID-19 vaccine. Therefore, this case highlights the possible association between COVID-19 vaccination and KFD and this should be considered in the differential diagnosis.

Associated Pyoderma Gangrenosum, Erythema Elevatum Diutinum, and Chronic Recurrent Annular Dermatosis: The Neutrophilic Disease Spectrum.

Neutrophilic dermatoses (ND) refer to a group of skin diseases characterized histologically by a cutaneous infiltrate of mature polymorphonuclear cells without an identifiable cause. Previously described as autonomous, these clinically distinct entities are included in the spectrum of neutrophilic disease due to the existence of overlapping forms, as described in our observation. Erythema elevatum diutinum (EED) is a rare dermatosis characterized by reddish-violaceous to browning papulonodular and plaques and belongs to the spectrum of cutaneous leukocytoclastic vasculitis. Chronic recurrent annular neutrophilic dermatosis (CRAND) is an exceptional neutrophilic dermatosis characterized by chronic annular lesions and the absence of generalized signs or hematological abnormalities. Histological features are similar to those seen in Sweet's syndrome. A 55-year-old woman with a history of pyoderma gangrenosum (PG) presented successively with two rare forms of ND, namely, EED and CRAND. There were no clinical or paraclinical arguments for any underlying systemic disease. Treatment with azathioprine 100 mg/day and topical steroids led to a total regression of lesions after a nine-month follow-up. Our observation is important because it reports two rare entities, CRAND and EED. Their occurrence in a single patient with a history of PG illustrates the concept of "neutrophilic disease" reported in the 1990s.

Gastric Duplication Cyst Revealed After an Endoscopic Ultrasound-Guided Fine-Needle Aspiration of a Suspected Mucinous Cystadenoma of the Pancreas.

Gastrointestinal duplication is a rare congenital anomaly of the gastrointestinal tract. Gastric duplication cysts (GDCs) are uncommon in adults, and most cases are discovered incidentally. Here, we report a fortuitous discovery of a rare case of an asymptomatic noncommunicating GDC in an adult revealed after an endoscopic ultrasound-guided fine-needle aspiration of a suspected mucinous cystadenoma of the pancreas. A 34-year-old female presented with renal colic. Her abdominal examination was normal. She presented a cystic image at the left lumbar discovered fortuitously during ultrasonography. On uro-computed tomography, there was a suspicion of a pancreatic cystadenoma. Magnetic resonance imaging of the pancreas suggested a mucinous cystadenoma of the pancreatic tail. The endoscopic ultrasound showed a cystic thick-walled formation in the tail of the pancreas. After guided fine-needle aspiration, a split aspect of the gastric wall appeared evoking a GDC. The cytology showed epithelial cells without mucin. Three years later, the patient does not have any gastrointestinal symptoms. GDCs are a rare anomaly, and accurate diagnosis of these cysts is difficult. Surgical resection can offer a definitive diagnosis. The mainstay of treatment is surgery to avoid the risk of malignancy.

Encephalomyeloradiculoneuropathy Revealing a Rare Case of Intravascular Large B-Cell Lymphoma.

Intravascular large B cell lymphoma (IVLBCL) is a rare form of extranodal non-Hodgkin's lymphoma, usually of B-cell lineage. Several organs are affected, most commonly the skin and the nervous system. We report a case of a 52-year-old man, with no medical history admitted with a five-month history of back pain with lower extremity numbness and tingling evolved to weakness associated with urinary retention, constipation and abdominal pain. Spinal magnetic resonance imaging (MRI) showed a gadolinium-enhancing lesion in the conus medullaris (CM). Electromyography (EMG) and nerve conduction velocity (NCV) test was consistent with demyelinating polyradiculoneuropathy in lower limbs. Slight clinical improvement with corticosteroids was observed. Three months after discharge, he presented a generalized tonic-clonic seizure. Cerebral MRI showed patchy lesions in the subcortical white matter with infiltration of the internal table of the skull with elevated serum lactate dehydrogenase (LDH). Calvarial biopsy revealed an intravascular large B-cell lymphoma. Treatment with cyclophosphamide and high-dose corticosteroids was initiated but the patient developed impaired consciousness and died of respiratory and circulatory failure six weeks after his readmission. Intravascular large B cell lymphoma should be considered in patients with a rapidly progressive severe encephalomyeloradiculoneuropathy. A biopsy of involved organs including the brain should not be delayed when IVLBCL is suspected, to initiate prompt systemic therapy.

Primary Plasma Cell Leukemia Revealed by a Mandibular Lesion: A Case Report.

Primary plasma cell leukemia (PCL) is a rare and aggressive hematological malignancy exhibiting a circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count >2000/mm3. We report a case of a 37-year-old woman presented to the Department of Hematology with a two-month history of growth inside the oral cavity in the upper jaw and weakness. The physical examination revealed a voluminous mass involving the left side of the maxillary gingiva. The maxillofacial computerized tomography (CT) scan confirmed the presence of a solid tissue mass at the left upper maxilla. A biopsy sample obtained from the lesion showed a plasma cell infiltration. The laboratory findings revealed anemia, renal impairment with high levels of creatinine and calcium. Serum protein electrophoresis found a monoclonal peak at IgG lambda, a high level of lambda free light. The diagnosis was subsequently confirmed by a peripheral-blood smear revealed 25% of plasma cells and bone marrow aspiration with 50% of plasma cell infiltration. Primary plasma-cell leukemia (pPCL) was confirmed. The patient received VTD chemotherapy (bortezomib, thalidomide, and dexamethasone) followed by autologous stem cell transplant (ASCT), which resulted in complete remission. At the six-month follow-up, the patient relapsed with extramedullary multiple lesions under ineffective rescue therapy. Response to frontline treatments may be significant initially but short-lived with a dismal median overall survival below one year. This case report aims to highlight the need for awareness among clinicians of the relevance of examining other associated clinical features of pPCL, given its aggressive course and rapid progress without the therapy.

Sacral hemangiopericytoma involving the retrorectal space: report of a case.

A primary hemangiopericytoma (HP) of the bone is rare, because the vast majority of these tumors arise in soft tissue. This report presents a case of a hemangiopericytoma in the sacrum (S1-S2) with extension to the retrorectal space. Only a few cases of osseous hemangiopericytomas in the sacrum and involving the retrorectal space have so far been reported. The difficult diagnosis of HP and the surgical strategy was chosen according to the location of the lesion in the sacrum and retrorectal space. A local excision was indicated. A sacral resection should be considered for tumors below S4. This report demonstrated the safety of this strategy. Adjuvant radiotherapy is useful in HP. The value of chemotherapy is still doubtful, although patients with high-grade tumors or metastatic spread seem to gain substantial benefit. Due to the often unpredictable behavior of this neoplasm, extended follow-up is strongly recommended.