A Rare Case of Familial Methemoglobinemia with Congenital Heart Disease.

Methemoglobinemia is a rare dyshemoglobin disorder which can either be congenital or acquired. Dyshemoglobin disorders can be asymptomatic or symptomatic. We narrate the case of a 12-year-old girl who presented with a fever, cough, and oxygen saturation of 85%. She was diagnosed with COVID-19, along with a large atrial septal defect and pulmonary arterial hypertension. Arterial blood gas analysis revealed normal partial pressure of oxygen and on 100% exposure to oxygen, blood color turned chocolate brown. After the resolution of COVID-19 in 10 days, the patient was treated with oral ascorbic acid and successful atrial septal defect repair. It is important to suspect dyshemoglobin disorder in a patient who presents with hypoxia/hypoxemia.

Unusual histomorphological spectrum of urinary bladder cancers and their treatment modalities revisited: Our experience with series of five cases.

Background: Urinary bladder cancer is the eighth-most frequent carcinoma in men, commonly occurs in elderly male. Major risk factors are smoking, chronic cystitis, urinary stones. The most common histologic variant of bladder cancer is urothelial carcinoma (UC), but certain variants are unusual yet aggressive for which there is no consensus guideline of therapy. Those entities include neuroendocrine tumors both primary and metastatic, squamous cell carcinoma, and sarcomatoid carcinoma. Neuroendocrine tumors comprise carcinoid, small-cell carcinoma, and large-cell carcinoma. Aim: The present study is undertaken to highlight certain biological features of these unusual aggressive histological forms of bladder carcinoma and their recent treatment modality to prevent recurrence, metastasis, upgrading of tumor stage, and enable surprisingly complete remission. Methods: This piece of hospital-based perspective study was done from June 2018 to May 2020. Both transurethral resection of bladder tumor and cystectomy surgical samples of the symptomatic patients were collected from the urology department of our institute along with demographic data. Then processed, stained in both routine H and E stain and immunohistochemical stains (Immunohistochemistry [IHC]) like PanCK, NSE, synaptophysin, chromogranin, etc. Results: Total number of bladder cancer encountered was 42 cases; of these five rare variants were observed, i.e., one case each of primary and secondary small cell neuroendocrine carcinoma, one large cell carcinoma, one squamous cell carcinoma (SCC), and one case of sarcomatoid carcinoma. Histomorphology with IHC conferred the diagnosis then multimodality therapy (neoadjuvant/radiotherapy/surgery) was installed and followed up. Conclusion: The unconventional forms of UC can be easily diagnosed by histomorphology and can have better survival with the help of the recent multimodal treatment approach.

Hepatic Plasmacytoma With DEL13q14 Positive on Fluorescent In Situ Hybridization (FISH) on Tissue Biopsy.

A 40-year-old male presented with abdominal distension and dyspnea. On evaluation found to have hepatic plasmacytoma without marrow clonal plasma cells. Fluorescent in situ hybridization (FISH) on tissue biopsy revealed myeloma-defining cytogenetics. After treating with novel agents, the patient had a complete response to therapy.

Histopathological and Immunohistochemical Evaluation of CDX2 and Ki67 in Colorectal Lesions with their Expression Pattern in Different Histologic Variants, Grade, and Stage of Colorectal Carcinomas.

BACKGROUND: A variety of colorectal lesions are surgically treated encompassing both benign and malignant polyps and colorectal cancer (CRC). CRC is the third most common cause of death in developed countries. Over the last decade, CDX2 has been linked to CRC progression, with reduced expression of the protein associated with more advanced tumor stage, vessel invasion, and metastasis. AIMS AND OBJECTIVES: To analyze the histopathology and immunohistochemistry (IHC) of CDX2 and Ki67 with their expression pattern; in different lesions of colon and rectum with special reference to various grade/stage/histological variants of CRC and to find out whether they can be used as possible predictive marker. MATERIALS AND METHODS: The study conducted was hospital based, both retrospective and perspective type comprising colorectal samples of total 367 cases (N) within a period of 2½ years. Surgical samples were collected, then grossed, processed, stained with routine hematoxylin and eosin stain in our department followed by IHC of CDX2 and Ki67 in only 60 randomly selected cases (n = 60). RESULTS: Out of total 367 cases, 265 cases were prospective study and 102 cases were retrospective study (240 cases were colonic lesions, and 127 are rectal lesions). The samples included were both from colonoscopy biopsy (small) 319 cases and 48 colectomy specimen (large). Mean age of the study participants was 49.62 years with a standard deviation of 17.34 years and predominantly male, but the difference was not statistically significant (P > 0.05). Colon (238 cases, 64.9%) as a whole affected more than rectum and left sided tumors more than the right side. All 60 cases were found to be positive for CDX2 expression (i.e., 100%); majority (n = 38) being carcinoma cases possessing high score and was statistically significant (P = 0.008, using Chi-square test) indicating strong association, whereas Ki-67 showed an increased index from noneoplastic to neoplastic cases. CONCLUSION: These markers can be used as future predictive biomarkers which will precisely evaluate risk group, prognosis, and response to therapy hence can be used as target therapy reducing irrational treatment.