Prenatally Diagnosed Infantile Myofibroma of Sartorius Muscle—A Differential for Soft Tissue Masses in Early Infancy

Background: Infantile myofibromatosis (IM) is a soft tissue disease with solitary or multiple benign tumors, and an etiology still unknown. IM is a mesenchymal disorder of early infancy and is more frequent in males. IM may present as a solitary lesion of the skin, bone, muscle, subcutaneous tissue, located at the head, neck, and trunk, with good prognosis; or, as a multicentric form, with or without visceral involvement (heart, lung, gastrointestinal tract, kidney), with a poor prognosis. The definitive diagnosis of IM is confirmed by pathology. Treatment may be conservative, surgical, or chemotherapeutical. Case presentation: A two months old female patient, prenatally diagnosed at 30 weeks, presenting with a tumor on the antero-internal aspect of the left thigh. She was admitted due to rapid postnatal evolution, and the patient required surgery for tumor resection. Previously, clinically, biological and imaging investigations were performed, but the final diagnosis was histological and by immunostaining. The patient had a favorable postoperative outcome. Conclusions: Despite its low frequency, IM should be considered in the differential diagnosis of soft tissue masses at an early age. The clinical form (solitary or multicentric), location, and visceral involvement will dictate the treatment and prognosis.

A Case of Tumor of Follicular Infundibulum in Parietal Scalp

Tumor of follicular infundibulum (TFI) is a rare benign cutaneous appendage tumor that does not have characteristic clinical features. It is mainly present in the head, neck, and trunk as a solitary lesion. In particular, TFI typically manifests as a plate-like proliferation with multiple thin epidermal connections comprise of monomorphic cells. TFI do not represent cutaneous characteristics, but have clinical significance because TFI is associated with basal cell carcinoma and Cowden's syndrome. We report a case of TFI in parietal scalp with a review of literatures.

Clinical Presentation and Treatment outcomes of Benign Renal Tumor: Angiomyolipoma

Background: Angiomyolipoma (AML) is a benign renal neoplasm composed of abnormal blood vessels, smooth muscle cells, and adipose tissue. About 40% of AMLs present with acute severe hemorrhage and is a potentially life-threatening condition. The risk of bleeding is proportional to tumor size and increasing significantly with size above 4 cm. Therefore, the patients with AMLs of 4 cm or more or spontaneous rupture of AMLs need interventional or surgical treatment. Objective: To evaluate the clinical presentation and treatment outcomes of the AMLs. Materials and Methods: The present report was a retrospective analytic study of the medical records and imaging studies in Phramongkutklao Hospital between January 2002 and December 2019. Results: Sixty-three patients, with a female:male ratio of 47:6, and a median age of 54.76 years with a range of 8 to 84 years, were included in the present analysis. Seven (11.1%) had tuberous sclerosis complex (TSC) and 56 (88.9%) had sporadic. Fifty-four had a solitary lesion, whereas nine had bilateral lesions. Asymptomatic AMLs were 50 (79.4%) and symptomatic AMLs were 13 (20.7%). There were 46 (73.0%) AMLs smaller than 4 cm and 17 (27.0%) AMLs of 4 cm or larger. AMLs smaller than 4 cm were incidentally discovered (n=40, 87%) and revealed due to symptoms such as flank pain (n=3, 6.5%) and hematuria (n=3, 6.5%). AMLs of 4 cm or larger were incidentally discovered (n=10, 58.8%) and revealed due to symptoms such as flank pain (n=7, 41.2%) and not presented with hematuria. AMLs smaller than 4 cm were mostly treated by follow up (n=39, 84.78%) or surgery (n=7, 15.22). AMLs of 4 cm or larger were treated by follow-up (n=11, 64.7%) then converted to arterial embolization (n=4, 23.5%) and converted to surgery (n=8, 47.1%). Two AMLs of 4 cm or larger died (11.8%). Conclusion: Significant differences in clinical manifestations and treatment outcomes were noted in respect to tumor characteristics, association with TSC, and treatment modality. Considering the benign nature of AML, size, and association with TSC ought to be considered when deciding upon active surveillance or prophylactic intervention. Keywords: Angiomyolipoma; Tuberous sclerosis complex; Embolization; Nephrectomy

Synchronous Thyrolipoma and Papillary Thyroid Carcinoma: A Rare but Significant Event

The presence of adipocytes within thyroid glands is a rare finding seen in thyrolipoma, diffuse lipomatosis, or thyroid teratoma. Although some cases present with multinodular goiter or autoimmune thyroiditis, the exact cause has not yet been elucidated. Among reported cases, thyrolipomas mainly occur in females and usually present as a solitary lesion. However, a few reported cases had coexisting papillary thyroid carcinomas. Herein, we present a 51-year-old female with synchronous thyrolipoma (2.0 × 1.5 × 1.3 cm) and papillary thyroid carcinoma (0.7 × 0.6 × 0.6 cm) within the same thyroid lobe. She had diabetes mellitus and hypertension and complained of anterior neck enlargement and discomfort for three months. Thyroid sonography showed multiple hypoechoic nodules, one of which was heterogeneous and ill-defined. Fine needle aspirate cytology for the ill-defined nodule was suspicious for papillary thyroid carcinoma. She subsequently received radical thyroidectomy and neck lymph node dissection. Histopathologically, one thyrolipoma and one papillary thyroid carcinoma were identified in the right lobe of the thyroid gland without metastases of lymph nodes, while other nodules were multinodular goiter. Notably, thyrolipoma may not be simply an incidental finding but might coexist with thyroid carcinomas. A brief review of the pertinent literature of prior reports is also provided.

Delayed solitary metastasis from primary follicular thyroid carcinoma to the scapular bone, a case report, and review of the literature

Most of the bone metastasis origination from Follicular Thyroid Carcinoma (FTC) will present as non-solitary and non-isolated. We present an extremely unique case of an isolated and solitary lesion in an unusual site, scapula, originated from FTC presenting incredibly about ten years after the initial successful treatment.

AB0777 A RARE CASE OF A SOLITARY PLASMOCYTOMA OF A LUMBAR VERTEBRA

Background:Solitary plasmocytoma is a rare tumour that represents around 2 to 5% of all plasma cell dyscrasias. It normally affects soft tissu but rarely the bone. Diagnosis is based on histology, the absence of bone marrow involvement.Objectives:To drag attention to think of solitary plasmocytoma of bone when dealing with a vertebral fracture in the absence of the CRAB criteria of multiple myeloma.Methods:We report a rare case of a vertebral fracture of the 4th lumbar vertebra (L4) revealing a solitary plasmocytoma of bone.Results:A 67-year-old female patient presented to our rheumatology department with back and left radicular pain of brutal onset, 15 days prior to her visit. Pain was severe and awakened her at nights. On examination, mobilities of the spine were unchanged but on palpation she had exquisite pain of L4. Laboratory tests showed a normal sedimentation rate of 15mm the first hour, a negative c-reactive protein, normal calcemia and kidney tests. X-rays of lumbar spine showed a vertebral fracture with a destruction over 50% of the vertebral size and cortical rupture. MRI of the spine showed the absence of other lytic lesions or other fractures or spinal cord compression and showed the total destruction oft he anterior vertebral body of L4 (Figure 1). Protein electrophoresis was in normal range and 24h urinary proteinuria was negative. Other tests rules out gynecological, thyroid, and renal neoplasms. Sternal puncture showed a rich bone marrow of normal cells without further infiltration. Bone biopsy of the detected lesion showed tumour cells made of mature plasmocytes confirming the diagnosis of solitary plasmocytoma of the bone. The patient was treated with radiation therapy. The evolution after 24 months showed a stabalised lesion and the absence of progression to multiple myeloma.Figure 1.T2 weighted MRI showing the vertebral fracture of L4Conclusion:It is important to keep in mind the diagnosis of solitary plasmocytoma of bone when facing a solitary lesion or vertebral fracture despite relatively non agressive radiological signs. It is also important to note the possible evolution to multiple myeloma and keep a hawk-eyed guard.References:[1]Masmoudi K, Elleuch E, Akrout R, et al. Le plasmocytome solitaire osseux: à propos de 3 cas et revue de la littérature. Pan Afr Med J; 25. Epub ahead of print 6 December 2016. DOI: 10.11604/pamj.2016.25.219.10933.Disclosure of Interests:None declared

Cysticercal Brain Abscess: A Distinct Entity Mimicking Pyogenic Brain Abscess.

Cysticercal brain abscess is a rare entity with the clinical presentation and radiological findings closely mimicking a pyogenic abscess. We report three cases of cysticercal abscess presenting as solitary lesion in the brain with radiological appearance like an abscess. All the patients underwent excision of the lesion with histopathological diagnosis of cysticercal brain abscess. The clinical outcome was satisfactory after surgical excision. Cysticercal abscess, though rare, should be considered as a differential diagnosis of a solitary rim-enhancing lesion, mainly if the patients belong to the endemic zone of neurocysticercosis. Surgical excision with or without a short course of the anti-parasitic agent is the treatment of choice.

Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study

Background and Objectives: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment of LCH at our institution and to add to the evidence for LCH. Materials and Methods: We reviewed six cases of LCH treated in our hospital between November 2005 and February 2016. Patient age at the first visit, sex, site of origin, symptoms, image tools used for diagnosis, biopsy site, complications, treatment, and final clinical outcome were evaluated. The median follow-up period was 41 months. Results: The median patient age at the first visit was 13.5 years. Three male and three female individuals were enrolled. Multiple lesions were observed in five cases, and a solitary lesion was observed in one case. Pain was the chief complaint in five cases. Radiography was the most commonly used imaging tool. Bone scintigraphy or magnetic resonance imaging and positron emission tomography-computed tomography were also used to diagnose systematic LCH. Biopsy of the femur was performed in two cases, and biopsy of the tibia, lumbar vertebrae, rib, and radius was performed in one case each. Regarding comorbidities, one case of hepatitis B and one case of autism were observed. Chemotherapy was initiated in two patients. The other four patients were observed naturally. Continuous disease-free survival was observed in five patients. One patient remained alive but not without disease during the final follow-up examination. Conclusion: LCH should be diagnosed as early as possible to treat it appropriately.

Clear Cell Acanthoma of the Mammary Region: A Case Report

Clear cell acanthoma, also known as Degos acanthoma, is a rare benign epithelial skin tumor. Generally, it appears as a solitary lesion on the lower legs, but in this case, it appeared on the right mammary area near the areola, which is an exceedingly rare and exciting finding. This lesion's clinical features revealed elevated dome-shaped plaque with surface lobulations and shiny pink color with a size of 9 mm. Dermoscopy was characteristic with blood vessels lined up in a strings pattern. Excision biopsy was done as a therapeutic measure. Histopathology showed a collection of glycogen-containing cells in the epidermis consistent with the diagnosis of clear cell acanthoma. Classical dermoscopy features and diagnostic histology were exemplary for a case report.