Cutaneous Metastasis of Oral Carcinoma Tongue: A Rare Case.

Cutaneous metastasis from head and neck cancers is rare, typically presenting as single or multiple nodules. This report presents a truly unique and intriguing case of squamous carcinoma of the tongue, in which the patient developed numerous metastatic nodules in the face and neck, a phenomenon rarely seen in clinical practice. The patient, a known case of carcinoma tongue, was treated radically with concurrent chemoradiation. He presented with small cutaneous lesions in his neck and upper chest, which were confirmed as cutaneous malignancies. Despite receiving one cycle of palliative chemotherapy, the management of this case posed significant and complex challenges, requiring a deep understanding of the nature of the spread and metastatic pathway for choosing the appropriate management.

Comparison of conventional unstained lymph nodal harvesting vs methylene blue-stained lymph nodal harvesting in colorectal specimen in staging left-sided colorectal carcinoma: a randomized controlled trial.

BACKGROUND: The management and prognosis of colorectal carcinomas (CRCs) are related to the stage of the disease, which, in turn, relies on the lymph node harvest from the surgical specimen. The guidelines recommend that at least 12 lymph nodes are required, which is not achieved in most resections. In this study, we propose a method to improve the lymph node yield in such cases. This study aimed to determine whether ex vivo injection of methylene blue into the inferior mesenteric artery or its branches improves lymph node retrieval in left-sided CRCs. METHODS: This study was conducted as a single-center, double-blinded, superiority randomized controlled trial. Patients who underwent elective surgery for left-sided CRCs with curative intent were randomized into 2 groups: stained and unstained. The sample size was calculated as 66. In all patients, details of disease stage, history of neoadjuvant therapy, and number of isolated lymph nodes were recorded. RESULTS: The mean number of lymph nodes extracted from the stained group was significantly higher than that from the unstained group (15.9 ± 5.2 vs 9.1 ± 5.7, respectively; P < .001). Among the patients who had received neoadjuvant therapy, the yield was higher in the stained group (P < .001). The yield was found to be greater in patients who had undergone upfront surgery than in those who had undergone neoadjuvant therapy, even in the stained group (100% vs 66.7%, respectively). CONCLUSION: The use of methylene blue injection into resected specimens of left-sided CRCs significantly improved the lymph node yield.

Skull Base Metastasis and Krukenberg Tumor in a Pregnant Woman: An Unusual Presentation of Metastatic Gastric Cancer.

Gastric cancer is an infrequent cause of vomiting during pregnancy. It is often diagnosed at an advanced stage due to late presentation by patients, mistaking it for gestational symptoms. We report a 24-year-old pregnant woman with gastric cancer with skull base metastasis and Krukenberg tumor on initial diagnosis. She underwent medical termination of pregnancy and best supportive care before dying of her illness.

Kaposiform Haemangioendothelioma of the Sublingual Gland in an Adult.

Kaposiform haemangioendotheliomas (KHE) are extremely rare, borderline malignant, locally aggressive vascular tumours. KHE is usually found in the retroperitoneum, over the extremities, the soft tissues of the trunk, mediastinum and the head and neck regions. We present a very rare case of KHE of the sublingual gland in an adult, which was not associated with Kasabach-Merritt phenomenon and was resected completely without any recurrence. To the best of our knowledge, this is the first reported case in the literature of a KHE arising from the salivary glands in an adult.

Clinicopathological Profile of Primary Hyperparathyroidism with Special Reference to Ki-67 Labelling Index.

Context: Primary hyperparathyroidism (PHPT) can occur due to a neoplastic process or hyperplasia. While the disease presentation is predominantly asymptomatic in developed countries, this is not the case yet in India. Differentiation of the type of lesion can only be done based on histomorphology but has its own challenges. Immunohistochemical markers like Ki-67 have been studied to aid in diagnosis but data on this is sparse from India. Aims: The aim of this study is to assess the clinical, biochemical and pathological profile of PHPT and to analyse the differences in immunohistochemical marker Ki-67 among the various lesions. Setting and Design: A descriptive study was carried out on 38 PHPT patients who were treated at our institute from January 2011 to March 2021. Materials and Methods: Post-surgery, the causative lesions were categorised as adenoma (31), hyperplasia (5) and carcinoma (2). Clinical, biochemical, radiological and histopathological features of all lesions were collected and analysed. Ki-67 proliferation index was calculated. The various parameters were compared across the three groups of lesions and correlated with Ki-67 index. Results: Out of 38 patients, 37 were symptomatic with skeletal symptoms being the most common followed by renal symptoms. There was no difference in clinical or biochemical parameters among the three types of lesions. Significant negative correlation was seen between serum iPTH and serum 25-OH Vitamin D levels (P0.006) The median Ki-67 index was found to be 0.40% in hyperplasia, 0.49% in adenoma and 5.84% in carcinoma. Conclusion: PHPT still presents as an overtly symptomatic disease in India. Diagnosis of the nature of lesion depends on the accurate application of morphological criteria. A high Ki-67 index was not found to be an absolute marker of carcinoma, as it was also seen in a small proportion of atypical adenomas.

An autopsy report with a diagnostic clinical profile on fatal overdose of life-saving iron supplement.

Ferrous sulfate is a commonly prescribed drug for prophylaxis and treatment purposes, particularly in women and adolescent girls. However, its easy availability, potential toxicity at higher doses, and vague clinical presentation make it a drug of concern when evaluating a case of poisoning. We present the case of a 28-year-old female who allegedly consumed 60 ferrous sulfate (60 mg of elemental iron in 200 mg of ferrous sulfate) tablets in a suicidal attempt. She presented with gastrointestinal disturbances on the same day to a tertiary care health facility. Investigations revealed deranged liver function tests, serum iron levels ten times the normal range, and high levels of saturated transferrin. Despite treatment, she succumbed to the poisoning 4 days after the incident. Autopsy showed features of liver failure, which was confirmed by histopathology. Chemical examination detected free ferrous and chloride ions. This fatal case of adult iron toxicity highlights the different causes of death in various stages of iron toxicity, providing a wider perspective on clinical management and aiding in the determination of the cause of death during an autopsy. This article adds a rare fatal iron poisoning case in adults to the literature, emphasizing the necessity for regulating iron tablet supplementation and raising public awareness of the toxicity of iron, which could save millions of lives.

Coexistence of malignancy and tuberculosis: is it double disease or double hit related to COVID-19? - Experience from a tertiary care center.

BACKGROUND: Coexistent malignancy and tuberculosis (TB) are rarely encountered. Cancer patients are a highly vulnerable subgroup during this Covid crisis. Delayed treatment for malignancy because of COVID-19 pandemic leads to higher chances to get infections. PURPOSE: The present study aimed to present the clinicopathologic profile of the patients with coexistent carcinoma and TB during the COVID-19 pandemic in a tertiary care center. MATERIALS AND METHODS: This was a retrospective study conducted during the COVID-19 pandemic between April 2020 to May 2021 in the Department of Pathology of our Institute. 11 patients with coexistent malignancy and caseous necrotizing granulomatous inflammation with Langhans giant cells and or acid-fast bacilli (AFB) positivity were included in the study. Cases of ill-defined granulomas coexistent malignancy were excluded. We studied varied clinical and histopathologic features of these cases. RESULTS: Eleven cases were reported with coexistent malignancy and tuberculosis, of which 8 were reported in 2021 and 3 cases were reported in 2020. Adenocarcinoma comprised 9 cases (81.8%) and the remaining 2 were squamous cell carcinoma (18.1%). Out of 11, 10 (90.9%) were new TB cases. Of these, 10 were extrapulmonary TB and one pulmonary TB case with cancer. Regarding chemotherapy, four patients accepted that chemotherapy was delayed because of the COVID-19 crisis. CONCLUSION: In this covid pandemic, India being the 2nd most populous country and endemic for TB, there is a higher chance of latent and active TB. The coexistence of two different pathologies is rare, even in a region with a high incidence of TB. Delayed chemotherapy in a pandemic situation leads to an increased incidence of infectious diseases such as TB.

Diagnostic utility of immunohistochemical markers alpha methyl acyl coA racemase (AMACR) and Ets related gene (ERG) in prostate cancer.

OBJECTIVES: To study the sensitivity and specificity of IHC markers AMACR and ERG in prostatic adenocarcinoma. METHODS: The study was a prospective one and samples were collected from August 2014 to June 2016. A total of 186 samples were obtained from the Department of Urology, in which 112 of these were benign prostatic hyperplasia (BPH), and 71 were prostatic adenocarcinoma. The adenocarcinoma cases were evaluated by two histopathologists, and appropriate Gleason score was given according to the modified ISUP Gleason grading system (2016). IHC markers AMACR & ERG were performed on the adenocarcinoma cases and their sensitivity and specificity were calculated. RESULTS: AMACR was a highly sensitive and specific marker for detecting prostatic carcinoma with a sensitivity and specificity of 95.8% and 96.5% respectively. ERG was a very specific marker with poor sensitivity in detecting prostate cancer. The sensitivity and specificity of ERG were 35.2% and 100% respectively. ERG expression decreased with increasing Gleason grade, PSA level, and tumour volume, which was statistically significant while the association of AMACR with Gleason grade or with tumor volume was not significant. CONCLUSION: ERG is a marker of early prostatic carcinogenesis and tumors may be positive or negative subtypes. Special histomorphologic features like perineural invasion, glomerulations, and intraluminal blue mucin were also studied. AMACR was a highly sensitive marker for detecting prostatic adenocarcinoma, while ERG was highly specific.

Clinicopathologic profile of gastroenteropancreatic neuroendocrine neoplasms in a referral center of South India.

BACKGROUND: The neuroendocrine system of the gastroenteropancreatic (GEP) region gives rise to unique, heterogeneous malignancies that need a high index of suspicion to make a diagnosis owing to their indolent course. AIMS: The present study aimed to find the incidence and the differences in the morphologic and immunohistochemical profile of gastroenteropancreatic neuroendocrine tumors (GEPNET) in a referral center of South India, JIPMER, Puducherry, India. METHODS: There were 55 gastroenteropancreatic region neuroendocrine neoplasms (NEN) assessed for demographic, clinical and radiological features. Gross morphological features, histopathological features, mitotic index, Ki67 proliferation index, and immunohistochemical positivity for synaptophysin, chromogranin-A, CD-56, NSE (Neuron Specific Enolase) and pan-cytokeratin (Pan-CK) were also assessed. RESULTS: The majority were nonfunctional tumors presenting with abdominal pain, gastrointestinal bleed, vomiting, jaundice, and loss of weight and appetite. The sites of involvement according to the order of frequency were duodenum, stomach, rectum, pancreas, ileum, appendix and jejunum. The endoscopic appearance of duodenal and jejunal tumors showed polypoidal, nodular and ulceroproliferative growth. These tumors were diagnosed by preoperative biopsy; 54% of them were grade-1 neuroendocrine tumors exhibiting nesting, trabecular, cord, and solid sheet patterns. All 55 cases were synaptophysin-positive with variable positivity for chromogranin, neuron-specific enolase, CD56, and Pan-CK. Mixed adenoneuroendocrine carcinomas (MANECs) involving the duodenum and stomach comprised 7.3% of all GEPNETs. Pancreatic neuroendocrine tumors constituted 9% of all tumors; one was multifocal. Lymph node metastasis was seen in 12/55 tumors; 6/12 showed liver metastasis also. All metastasizing tumors measured less than 4 cm in size. Statistical correlation of the tumor grade, mitotic count and Ki67 index as analysed by Spearman's correlation between the paired data denoted by rs in 55 tumors showed a strong correlation between mitotic count and Ki67 index; a moderate correlation was noted between the tumor grade and Ki67 index. CONCLUSION: The clinicopathologic profile of 55 GEPNET revealed a majority to be sporadic Grade 1 tumor. Tumors that showed lymph node and liver metastasis were less than 4 cm in size. MANECs were found in the duodenum and stomach.

Vermiform Appendix and the Potential for Missed Pathologies.

Introduction The appendix is considered an appendage of little value and is often treated disdainfully, be it as part of evolutionary process, on a grossing table, under a microscope or while archiving specimens and slides. It is only recently, with data indicating its importance in gut immunity and as the origin of pseudomyxoma, that its space in a human body appears vindicated. Aim Our aim was to screen the histopathologic spectrum of appendix lesions observed in our hospital for rare, incidental or clinico-radiologically uncertain lesions that would help emphasize a necessary seriousness in its sampling. Method All appendectomy specimens over ten years were screened for diagnosis other than acute/chronic/resolving appendicitis and pseudomyxoma peritonei. Among the recorded rare diagnoses, one representative case each, based on interesting history or pathology, was selected for discussion. Observation Forty-three lesions were found to meet inclusion criteria comprising 12 varied etiologies. Among these, 25 had a normal-appearing appendix and 27 were not suspected on radiology or on clinical/surgical assessment. Histopathology comprised, among others, neoplastic entities such as (Diffuse large B-cell) lymphoma, metastasis, carcinoid as well as interesting non-neoplastic diagnoses such as pinworm infestation (in the elderly) and (post-menopausal) endometriosis. Conclusion Sampling and histopathologic assessment of the appendix should be compulsory, careful and representative. Each specimen must be treated as harboring a potential pathology, until microscopically proven otherwise because missed "rare" diagnoses could delay therapy or alter key management decisions as cancer staging.

Neuroectodermal tumours of the sino-nasal tract: a clinicopathological study of 18 cases with a newly proposed revised classification and a brief review of literature.

AIM: Sino-nasal tract tumours constitute 3% of the head and neck malignancies. Among these tumours, neuroectodermal tumours are rare with histo-morphological and immunohistochemical overlap making them a challenge for the pathologist. We included Ewing's/PNET, olfactory neuroblastoma (OFN), mucosal malignant melanomas (MMM), Melanotic neuroectodermal tumour of infancy (MNTI), small-cell neuroendocrine carcinoma (SNEC), and the newest entity Adamantinoma like Ewing's sarcoma (ALES) as part of the neuroectodermally derived tumours of the sino-nasal tract. The last three entities were added to the existing ones, which also has been emphasized in this paper. METHODS AND RESULTS: A comprehensive analysis was done on all neuroectodermally derived tumours from 2016 to 2020. A total of 18 cases were collected, which included OFN (10 cases), SNEC (2 cases), MMM (2 cases), Ewing's/PNET (2 cases), MNTI (1 case), and ALES (1 case). The most common presentation in NE tumours was nasal obstruction (80-100%). Except for OFN, all other tumours were confined to the nasal and paranasal sinuses. 4/10 cases of OFN showed orbital extension. Cervical lymph-node metastasis was seen in 50% of cases of SNEC and MMM groups. An array of relevant immune-histochemical markers were performed. The marker expression was very subtle among the groups. On follow-up, recurrence was seen in the OFN and MMM groups in 30 and 50%, respectively. Metastasis was seen in SNEC group (100%) and OFN group (10%). CONCLUSION: As sino-nasal neuroectodermal tumours pose a diagnostic challenge and have different therapies and are prognostically different, the pathologist must be aware of the subtle morphological, immunohistochemical clues which have been dealt with in-depth in this study.

Co-infection of intestinal tuberculosis and mucormycosis in a patient with Down syndrome: a unique case report with literature review.

Mucormycosis represents several unusual opportunistic infection caused by saprophytic aseptate fungi. There is a recent rise in cases of mucormycosis due to an increase in diabetic and immunodeficient patients like patients on long-term steroids, immunomodulators due to organ transplantation, malignancies, mainly haematological malignancies, and autoimmunity. Anatomically, mucormycosis can be localised most commonly as rhino-orbito-cerebral followed by pulmonary, disseminated, cutaneous and gastrointestinal, rarest being small intestinal. Patients with Down syndrome are immunodeficient due to their impaired immune response. Disseminated tuberculosis is also common in immunodeficient patients. We report a rare case of small intestinal mucormycosis in a patient with Down syndrome with coexisting intestinal tuberculosis. Due to the invasiveness of mucormycosis, the patient succumbed to death despite providing aggressive surgical debridement and medical management.

Malakoplakia of Endometrium with Osseous Metaplasia on Evaluation of Postmenopausal Leukorrhea: A Rare Case Report.

Malakoplakia is a chronic xanthogranulomatous condition that affects the genitourinary tract reported earlier as urinary granulomas and pelvic masses. We report a different clinical manifestation of malakoplakia presenting as postmenopausal pyometra. A 64-year-old postmenopausal female presented with foul-smelling vaginal discharge with a past history of induced abortion, followed by dilatation and evacuation. On examination, abdomen was soft, vaginal examination revealed pus discharge, parous size uterus with free fornices, and pap smear ruled out malignancy. Ultrasonography revealed linear, echogenic structures in the endometrial cavity suspicious of bony spicules with fluid around. Hysteroscopy revealed congested endometrium with multiple pieces of shredded bone-like structures that were removed followed by curettage. Histopathological examination was suggestive of malakoplakia with osseous metaplasia. Retained bony spicules can cause chronic granulomatous inflammation that may become symptomatic postmenopause due to absent cyclical shedding. This is the first reported case of malakoplakia of uterus following retained bony spicules.

Cholemic Nephrosis: An Autopsy Study of a Forgotten Entity.

OBJECTIVE: The aim of the study is to do a clinicopathologic study of post mortem kidney biopsies with significant deposition of bilirubin pigment within tubular epithelial cells and in the lumen of distal tubules as a bile cast. MATERIAL AND METHOD: All post mortem specimens with acute tubular necrosis, with the presence of bile casts in tubules or bile pigment deposition in the tubular epithelium during the period 2015-2018 were examined for gross and histopathology along with biochemical parameters and viral markers. RESULTS: Bile casts with sloughed renal tubular epithelial cells and occasional macrophages were present in the distal convoluted tubule in 78.6% of biopsies (11/14). The plugging of distal convoluted tubule with casts was similar to that seen in myeloma and myoglobin cast nephropathies. Bilirubin pigment deposition was present in 35.7% (5/14) of cases. The frequency of bile casts in each biopsy was variable and it did not have any association with serum bilirubin levels or etiology of liver dysfunction. A striking difference from earlier studies is the high number of toxin-induced liver damage including six cases of paraquat and 2 cases of yellow phosphorus poisoning. CONCLUSION: This study proves importance of the bile cast nephropathy as a reason for kidney injury, especially with varied hepatotoxic etiologies, especially paraquat and yellow phosphorus.

Effect of preoperative finasteride on perioperative blood loss during transurethral resection of the prostate and on microvessel density in patients with benign prostatic hyperplasia: An open label randomized controlled trial.

OBJECTIVE: Transurethral resection of the prostate (TURP) is a common procedure for the treatment of benign prostatic hyperplasia (BPH). Previous studies on the effect of 5-alpha reductase inhibitors on perioperative blood loss in TURP and microvessel density (MVD) in the prostate are equivocal. We evaluated whether pretreatment with finasteride for 2 weeks before surgery can reduce perioperative blood loss in TURP and MVD in the prostate. MATERIALS AND METHODS: Sixty-eight patients of BPH planned for TURP were randomized into two groups. The study group comprising 34 patients was treated with finasteride (5 mg/day) for 2 weeks and the placebo group comprising 34 patients received placebo for 2 weeks, before TURP. Blood loss was measured in terms of a reduction in the blood hemoglobin (Hb) and hematocrit (HCT) levels between preoperative values and 24 h after surgery. MVD was measured in the resected prostate tissue stained with anti-CD31 monoclonal antibody. RESULTS: The reduction of Hb and HCT in the finasteride group was significantly lower than the reduction in the placebo group (P < 0.05). The artery (P = 0.005), vein (P = 0.05), and gland (P = 0.008) densities were significantly less in the finasteride group than in the placebo group. There was no significant correlation between blood loss and MVD. CONCLUSIONS: Our study suggests a clear advantage of the preoperative use of finasteride for 2 weeks by reducing the perioperative blood loss in TURP in patients with BPH. While there is a significant reduction in MVD in the prostate on treatment with finasteride, it is not clear that this is the mechanism of reduction in blood loss in TURP.

Malignant melanoma of urethra in a young female - Case report and Literature review.

Malignant mucosal melanoma (MM) is a rare and aggressive neoplasm, which behaves differently from its cutaneous counterpart. MM of head and neck account for 55.4%, genital tracts - 18%, ano-rectal - 23.8% and urinary tract - 2.8%. Less than 200 cases of urethral melanomas have been reported in literature, out of which MM of female urethra represent only 0.2% of primary melanomas. Owing to the delayed presentation, early onset of metastasis and aggressive tumor biology, prognosis has remained largely poor despite adequate local control in MM. Surgery is the mainstay of management, with adjuvant radiation contributing to local control but not overall survival benefit. Chemotherapy and immunotherapy options are being explored in both adjuvant and palliative setting. We hereby, report the clinical presentation, management, and course of a rare case of urethral melanoma in a young female patient. After ruling out metastatic disease at presentation, local control was achieved by wide local excision with distal urethrectomy and modified Singapore flap reconstruction along with bilateral modified superficial inguinal block dissection; followed by adjuvant radiotherapy in view of close margins. However, she developed distal recurrence three months post adjuvant therapy, thus reflecting the aggressive biology of this class of rare tumor.

Descriptive study of the histopathological subtypes and programmed death-ligand 1 in metaplastic breast carcinoma.

Metaplastic Breast Carcinoma (MBC) is a rare heterogeneous group of tumors, the incidence of which is less than 1% of breast tumors. These are a unique set of tumors with varying subtypes, poor prognosis, and an increased chance of distant metastasis. We aimed to study the clinical, histomorphological, and immunohistochemical (IHC) features of Metaplastic Breast Carcinoma (MBC). This was a descriptive study of cases diagnosed as MBC at a tertiary care center in Southern India from January 2015 to December 2019. A total of 20 cases were diagnosed whose clinical, histomorphological, and IHC features were studied. PD-L1and CD8 IHC were performed and analyzed in 12 cases. The median age of presentation was 50 years. Seventy percent (14/20) patients were postmenopausal women. On excision, 75% (15/20) showed mixed typed MBC, the remainder showing epithelial type MBC. Metastasis to axillary lymph node was seen only in 20% (4/20) of the cases. Thirty percent (6/20) of the cases belonged to stage 3 disease and 5% (1/20) of the cases belonged to stage 4 disease with liver metastasis. Estrogen receptor (ER), Progesterone receptor (PR) were negative in all the cases, Her2neu was positive in three cases. Ki67 labeling index was greater than 14% in all the cases. PD-L1was positive in 41.5% of the cases and intratumoral CD8 positive lymphocytes were increased in 83.3% of the cases. MBCs are tumors occurring in elderly postmenopausal women, presenting with large tumor size, have lesser chances of lymph node metastasis, and a higher chance of recurrence and hematogenous spread. They are negative for ER, PR, Her-2 neu, with a high Ki67 index and a strong PDL-1 expression.

Narciclasine improves outcome in sepsis among neonatal rats via inhibition of calprotectin and alleviating inflammatory responses.

Sepsis is associated with exacerbated inflammatory response which subsequently results in multiple organ dysfunction. Sepsis accounts for high mortality and morbidity among newborns worldwide. Narciclasine is a plant alkaloid which has shown to possess anti-inflammatory properties. In this study we investigated the effect and mechanism of action of narciclasine in neonatal sepsis rat models. The excessive release of S100A8/A9 or calprotectin in neonatal sepsis could be detrimental as it could exacerbate the inflammatory responses. We found that narciclasine significantly reduced the plasma levels of S100A8/A9 and also suppressed its expression in the liver and lung. The systemic and local bacterial load was also reduced in the narciclasine treated rats. The systemic and local production of pro-inflammatory cytokines in plasma and organs (liver and lungs) was significantly reduced in the narciclasine treated rats. The histopathological studies showed that narciclasine prevents the organ damage associated with sepsis and improved the survival of neonatal rats. Sepsis increased the phosphorylated NF-κß p65 protein expression in the liver. Narciclasine suppressed the phosphorylation of NF-κß p65 and the degradation of NF-κß inhibitory protein alpha. It could also suppress the expression of adaptor proteins of the toll like receptor signaling pathway viz., myeloid differentiation factor 88 (MyD88), Interleukin-1 receptor-associated kinase 1 (IRAK1) and TNF receptor associated factor 6 (TRAF6). These results suggest that narciclasine protects against sepsis in neonatal rats through the inhibition of calprotectin, pro-inflammatory cytokines and suppression of NF-κß signaling pathway.

Malignant Epithelioid Soft Tissue Tumours- A Pathologist's Perspective With Review of Literature.

Background Soft tissue tumours with epithelioid morphology have many differential diagnoses, which include epithelioid sarcoma, malignant extrarenal rhabdoid tumour, epithelioid malignant peripheral nerve sheath tumour, epithelioid leiomyosarcoma, epithelioid angiosarcoma and sclerosing epithelioid fibrosarcoma. There are other rare entities also. They often express characteristic patterns of immunohistochemical markers that can be used to identify these tumours. Materials and methods This retrospective study comprises 22 cases of malignant soft tissue tumours with epithelioid differentiation diagnosed over a period of four years. Findings regarding clinical presentation, cytological findings, histopathological findings and immunohistochemical profile of the tumours were noted and analyzed. Results A total of 22 cases were included in the study including five cases of epithelioid sarcoma (conventional and proximal), three cases of epithelioid angiosarcoma and epithelioid myxofibrosarcoma, two cases of epithelioid malignant peripheral nerve sheath tumour, epithelioid gastrointestinal stromal tumour and clear cell sarcoma each, one case of epithelioid leiomyosarcoma, sclerosing epithelioid fibrosarcoma, malignant extrarenal rhabdoid tumour, monophasic synovial sarcoma and malignant and malignant perivascular epithelioid cell tumour each. Conclusion Pathologists should be aware of the various differential diagnoses of soft tissue tumours with epithelioid morphology. Over and above the clinical findings and morphological features, ancillary methods like immunohistochemistry help to arrive at a definitive diagnosis in most cases.