Periosteal Osteosarcoma of the Distal Shaft of Fibula: Case Report on Rare Entity.

Introduction: Periosteal osteosarcoma is a rare, intermediate-grade, malignant tumor arising on the surface of the bone. There are only a few numbers of periosteal osteosarcoma of fibula reported. However, there has never been a case concerning distal fibula recorded so far. Wide surgical removal is the commonly advised treatment. In the present report, we describe a case of periosteal osteosarcoma localized to the distal fibula, which was treated by wide resection and reconstruction of ankle mortise with the ipsilateral proximal fibula. Case Report: The patient was a 48-year-old female who presented with ankle pain and swelling. A surface lesion, with "hair on end" periosteal reaction but without obvious medullary involvement, was seen on the distal fibular shaft by imaging studies. The diagnosis of periosteal sarcoma was confirmed with tru-cut biopsy. Wide resection and ankle mortise reconstruction with ipsilateral proximal fibula were done and 1 year of follow-up shows a good outcome. Conclusion: Periosteal osteosarcoma is a well-defined pathological entity with characteristic radiological and histological features. It is essential to distinguish it from other surface osteosarcomas as treatment modalities are different. Controversy remains about the appropriate treatment of periosteal osteosarcoma. Reconstruction of ankle mortise with reversed proximal fibular autograft is a good option in low-to-intermediate-grade periosteal osteosarcoma of the distal fibula rather than going for extensive radical procedures or adding chemotherapy to the treatment regimen.

Clinical, Hematological, and Cytogenetic Profile of Myelodysplastic Syndromes in Adults: A Three-year Cross-sectional Study.

Objectives: Myelodysplastic syndromes (MDS) are a group of clonal hematological disorders with a diverse clinico-hematological profile. Studies in India have shown a different biology from the West. This study aimed to assess the clinicopathologic profile of MDS patients, classify them according to the World Health Organization classification system, categorize them into International Prognostic Scoring System (IPSS) and the revised IPSS prognostic subgroups, and evaluate the treatment outcome. Methods: A cross-sectional study was conducted on 48 patients diagnosed with MDS from January 2017 to December 2019 from Rajagiri Hospital, India. Clinical, hematological, and cytogenetic features were analyzed. The patients were stratified according to the IPSS and revised IPSS and were followed-up for a minimum of six months. Results: The patients most affected were those in the seventh decade of life. We found a slight female preponderance and a mean age of 57.5 years in females and 67.7 years in males. Anemia was the most common manifestation of MDS. On the other hand, thrombocytopenia was found to be the least common cytopenia. MDS with multilineage dysplasia was the most common subtype. Cytogenetic abnormalities were seen in 29.5% of cases. Most of the patients were in the low-risk prognostic categories. Conclusions: Our patients were older when compared to those of other Indian studies, with most in the low-risk categories which were like Western data.

A Rare Case of Metachronous Peripheral T-cell Non-Hodgkin Lymphoma Following Epstein-barr Virus-positive Diffuse Large B-cell Lymphoma, Not Otherwise Specified.

We report a case of an elderly male who presented with enlarged abdominal lymph nodes and was diagnosed as having Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified (EBV+ DLBCL, NOS). He was started on chemotherapy which had to be discontinued after three cycles due to the development of life-threatening pneumocystis carinii pneumonia and poor performance status. Within two years, the patient presented with features of relapse. A repeat histopathological examination of the lymph node showed features of peripheral T-cell lymphoma, NOS and the clonality was confirmed by T-cell receptor gamma chain rearrangement assay. More studies are needed to understand the association of EBV+ DLBCL, NOS with other lymphomas.

A Rare Case of a Solitary Fibrous Tumor in a Child, Presenting as a Congenital Mass, Displaying NAB2ex4::STAT6ex2 Gene Fusion with an Incidental Ganglioneuroblastoma.

A solitary fibrous tumor (SFT) is a ubiquitous tumor that occurs across all ages. It is rarely reported in pediatric patients, especially as a congenital mass. A 1 year 7-month old male child presented with a slow-growing gluteal mass since birth along with a recent episode of bleeding. Magnetic resonance imaging (MRI) showed a well-defined, T1-hypointense and T2-hyperintense mass in the skin and the subcutaneous plane. Subsequently, he underwent a surgical resection that revealed a 5.7 cm-sized fleshy mass. On histopathologic examination, there was a fairly well-circumscribed, cellular spindle cell tumor, with cells arranged in intersecting fascicles and hemangiopericytomatous pattern with areas of hemorrhage and fibrinoid necrosis. Cells revealed mild nuclear atypia. Mitotic figures were up to 8/10 hpf. Immunohistochemically, the tumor cells were diffusely positive for CD34 and STAT6. Furthermore, the tumor revealed NAB2 exon 4:: STAT6 exon 2 fusion by RT-PCR and Sanger sequencing. Post-excision, during follow-up, the patient developed an adrenal mass that was histopathologically proven as a neuroblastic tumor, not further specified on biopsy and finally as ganglioneuroblastoma, intermixed type on excision. This constitutes one of the rare cases of SFT in a pediatric patient, presenting as a congenital mass, confirmed by STAT6 immunostaining and further, molecular testing. A review of literature of similar cases, including treatment-related implications, is presented.

Disseminated tuberculosis associated with autoimmune haemolytic anaemia and adrenal deficiency: a rare association.

Tuberculosis (TB) is one of the greatest masqueraders in medical practice and can have manifestations involving any organ or organ systems of the body. The presentation of disseminated TB can range from typical features like fever, weight loss and fatigue to protean manifestations. We share the case of an elderly man who presented to us with weight loss, anaemia, weakness and lymphadenopathy involving thoracic and intraabdominal locations. Work up of anaemia revealed features of Coombs-positive autoimmune haemolytic anaemia (AIHA) and evaluation of weakness showed laboratory results consistent with adrenal insufficiency. Biopsy of the abdominal lymphnode yielded caseating granulomas with CB-NAAT positivity for Mycobacterium tuberculosis Anti-TB chemotherapy with short-term replacement dose of systemic steroids corrected the haemolysis, anaemia and addisonian crisis. The case alerts clinicians regarding the uncommon association of TB with Coombs positive AIHA and adds one more aetiology to the pathogenesis of anaemia in TB. Furthermore, the occurrence of AIHA and hypoadrenalism in the same patient with TB is exceedingly rare and has not been reported.

Autoimmune Haemolytic Anaemia following ChAd Ox 1 nCoV-19 in Haemodialysis.

Autoimmune haemolytic anaemia (AIHA) is caused by autoantibodies that react with self-red blood cells (RBCs) and cause them to be destroyed with or without complement mediated mechanism. Its clinical presentation is heterogeneous, ranging from asymptomatic to severe forms with fatal outcomes, also it can be either idiopathic or secondary to a coexisting disorder. We report an elderly gentleman on haemodialysis, who presented with severe haemolytic anaemia after one month of first dose of ChAd Ox 1 nCoV-19 vaccine (viral vector vaccine) for SARS- CoV - 2, who also had asymptomatic COVID 19 infection around 6 months prior to vaccination. This is the first reported case of AIHA following COVISHIELD vaccine and till now there are no other reported cases from India.

An Unusual Case of Severe Persistent Neonatal Thrombocytopenia in an Extremely Low Birth Weight, Extreme Preterm Neonate.

Neonatal thrombocytopenia is a common hematological problem but refractory thrombocytopenia is very rare in neonates. A systematic and diligent workup will result in arriving at the proper diagnosis and providing accurate management in rare causes of neonatal thrombocytopenia. We report a case of severe refractory thrombocytopenia in an extremely low birth weight (ELBW)/extreme preterm baby who presented with early onset severe thrombocytopenia associated with anemia and required multiple platelet transfusions. After ruling out COVID-19 infection, sepsis and neonatal alloimmune thrombocytopenia (NAIT), the cause for severe refractory thrombocytopenia was diagnosed as Type II congenital amegakaryocytic thrombocytopenia (CAMT) by bone marrow examination and MPL gene mutation studies.

Expecting the unexpected - Primary mediastinal large B cell lymphoma presenting as huge lung parenchymal mass.

The first possibility considered in the etiology of large lung masses is neoplastic lesions. The differential diagnoses of these masses include bronchogenic carcinoma, pulmonary sarcoma, primitive neuroectodermal tumor etc. Primary or secondary pulmonary parenchymal lymphomas presenting as large mass is distinctly rare. We share the case of a young lady who presented with a large left lung mass almost entirely replacing the left lung parenchyma, with associated intrathoracic lymphadenopathy. On evaluation she was proved to have primary mediastinal large B-cell lymphoma. Treatment with an aggressive chemotherapy regimen led to complete remission of the parenchymal and nodal disease. The uncommon radiological presentation and the excellent therapeutic response despite huge tumor load merit clinical attention.

Xanthogranulomatous pleuritis - An unusual presentation of tuberculosis.

In pulmonary practice, pleural effusion is a commonly encountered entity and has various etiologies. Pleural effusions in postpartum women can be an incidental self-limiting finding. The presence of systemic or respiratory symptoms, however, calls for prompt etiological workup and targeted therapy. Tuberculous pleuritis and lupus-related pleural disease are well known to flare up in the postpartum period. We describe the case of a young healthcare worker with no previous comorbidities who presented with fever, breathlessness, and chest pain 2 weeks after an uneventful confinement. Chest radiograph revealed moderate left-sided pleural effusion. Pleural fluid analysis was biochemically consistent with tubercular effusion. Pleural biopsy histological examination showed features of xanthomatous pleuritis and Cartridge based nucleic acid amplification test (CB-NAAT) showed evidence of Mycobacterium tuberculosis(MTB). She was initiated on antitubercular medicines to which she responded well with the resolution of clinical symptoms and pleural collection. This is the first case report describing an association of xanthogranulomatous pleuritis with tuberculosis.

Uterine Angioleiomyoma with Atypia, Raised CA-125 Levels, and Pseudo-Meigs Syndrome: An Alarming Presentation.

Angioleiomyomas are benign mesenchymal tumours commonly occurring in the subcutis of extremities. They are typically composed of interlacing fascicles of smooth muscle cells with intersecting vascular channels. Angioleiomyomas of the uterus are rare with only very few case reports available in literature. Herein, we report a case of this rare entity in a 47-year-old woman owing to its highly unusual features of cellular atypia, raised CA-125 levels, and pseudo Meigs syndrome.

Subcutaneous human dirofilariasis.

Subcutaneous human dirofilariasis is caused by the zoonotic filariae Dirofilaria repens. The frequency of reports is increasing in literature during the last few years and is considered by some authors as an emerging zoonosis. Here we report 21 cases of subcutaneous dirofilariasis, which were encountered over a period of 8 years. None of these cases were associated with peripheral eosinophilia. Since these lesions can mimic benign and malignant tumors, it is important to consider this as a differential diagnosis of subcutaneous nodules especially in an endemic area. In this report we would like to emphasize the importance of studying multiple deeper levels in suspected cases of parasitic granuloma to demonstrate the parasite.