A Rare Case of Extragonadal Mixed Germ Cell Tumor of the Sacrococcygeal Region with Mature Thymic Tissue in the Teratoma Component.

A rare extragonadal mixed germ cell tumor of the sacrococcygeal area presenting with mature thymic tissue in the teratomatous component, a rare finding and the first reported case of such an association.

Cytological diagnosis of cutaneous granular cell tumor: Rare tumor with rare presentation.

ABSTRACT: Granular cell tumors (GCTs) are uncommon soft tissue tumors, which are difficult to diagnose merely by clinical examination. Fine-needle aspiration cytology (FNAC), being an effective first-line investigation, plays a significant role in the preoperative diagnosis of GCT. However, the tumor can mimic certain other lesions; hence, a cytopathologist needs to be aware of its characteristic morphology. We report here a case of GCT, presented as a subcutaneous nodule in the first finger web. A differential diagnosis of lipoma/neurofibroma was made clinically. FNAC was done and showed characteristic features of granular cell tumor along with intranuclear inclusions and subsequently, it was confirmed on histopathology.

The Great Mimicker of Gastric Cancer: A Case Report of Ménétrier's Disease.

This is the case of a 52-year-old Indian lady who presented with hematemesis, severe anemia, and an abdominal lump in cardiac failure. On radiographic evaluation, the lesion appeared to be gross circumferential asymmetric proximal gastric wall thickening, with suspicion of gastric lymphoma or tubercular hypertrophic gastritis. After stabilization with multiple transfusions, she underwent proximal D2 gastrectomy with esophago-gastric anastomosis and a total splenectomy. Grossly, the gastric rugae appeared to be hypertrophied and firm. No growth was identified grossly; however, necrotic areas were identified at the distal end. Microscopic examination of multiple sections studied showed significant foveolar hyperplasia, tortuous glands, and a few cystically dilated foveolar glands, which were limited up to the muscle layer. Mild serosal congestion was seen. No atypia or invasion was seen. An impression to consider is the possibility of Ménétrier's disease (MD). MD is an acquired protein-losing enteropathy with giant gastric rugal folds, decreased acid secretion, and increased gastric mucous production. Radiographically, endoscopically, and grossly, the condition can be confused with malignant lymphoma or carcinoma. It is difficult to diagnose, and histopathological confirmation of the resected specimen is needed for a definitive diagnosis. Our intention in presenting this case is to emphasize that MD can present as massive hematemesis and should be considered in a differential diagnosis. Surgical treatment by total or partial gastrectomy is recommended for cases with persistent, debilitating symptoms or a risk of cancer.

Endometrial stromal tumors: A clinico-histomorphological spectrum of endometrial stromal tumors with review of literature.

BACKGROUND: Endometrial stromal tumors (ESTs) are rare subset of mesenchymal uterine neoplasms. There are heterogeneous morphological, immunohistochemical, and genetic features. Approximately 50% of ESTs occur in perimenopausal women. In 2020, WHO sub-categorized ESTs into four groups: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS), and undifferentiated uterine sarcoma (UUS). OBJECTIVE: To review the morphological spectrum of endometrial stromal tumors. METHOD: This retrospective study reviewed the histomorphological features of 15 endometrial stromal tumors with respect to atypia, necrosis, mitosis, collagen bands, whorling around vessels, myometrial invasion, and inflammatory cells. Immunohistochemistry markers (CD10, SMA, and ER) along with special stains (Masson's trichrome, toluidine blue) were also studied. RESULTS: The age of the patients ranged from 32 to 60 years. Three patients were postmenopausal. The most common presenting symptom was vaginal bleeding. Five patients were operated with a clinical diagnosis of uterine fibroid. One patient presented with prolapse with no other complaint. All the 15 patients had total abdominal hysterectomy and salpingo-oophorectomy. One case showed necrosis, eight cases showed collagen bands, all the 15 cases showed whorling around vessels, one case showed vascular emboli, and seven cases showed inflammatory cells. In low-grade cases, one case showed focal atypia and one case showed focal coagulative necrosis indicating infarction. Thirteen cases were LGESS, and one case of ESN and HGESS. All cases were positive for ER and CD10. CONCLUSION: Endometrial stromal tumors demonstrate extensive permeation of the myometrium as irregular islands with frequent vascular invasion, whorling around vessels, collagen bands, and inflammatory cells. All these features should be observed thoroughly on microscopy by pathologists to clearly differentiate the low-grade and high-grade endometrial stromal tumors, and to understand the overlapping gray areas morphologically as it affects the prognosis of the patient.

Leiomyoma of the Nipple: A Rare Entity at Rare Site.

Cutaneous leiomyomas are benign and rare smooth muscle tumors. Genital leiomyoma includes leiomyomas located in the nipple, scrotum, areola or vulva. Nipple leiomyomas are the least common genital leiomyomas and are commonly seen to occur in middle-aged women. Here, we present a case of a 40-year-old female complaining of a growth over the right nipple for six months. On local examination, it was a 1cm x 1cm growth on the lateral aspect of the nipple. Finally, a treatment plan of complete excision was done. Grossly, it was a well-circumscribed skin-covered soft tissue bit with a skin flap measuring 0.6cm x 0.6cm and soft tissue measuring 0.6cm x 0.5cm x 0.2cm. Histopathological examination revealed a skin-covered section with dermis showing a well-circumscribed unencapsulated lesion that showed intersecting fascicles of spindle cells with no atypia or mitoses noted. Microscopically, the growth had tumor-free resection margins. Immunohistochemical confirmation with S100, smooth muscle actin (SMA) and caldesmon was done. Diagnosis of nipple leiomyoma was given with strong SMA positivity. Nipple leiomyoma is a rare, benign lesion and needs to be correctly diagnosed microscopically. Biopsy and immunohistochemistry is a confirmatory investigation that can lead to timely management of the patient.

Schwannoma-like pleomorphic adenoma: A pathological quandary.

ABSTRACT: Pleomorphic adenoma usually presents as a painless growing mass in locations of the salivary glands and is notorious for local recurrence if previous surgical resection is incomplete. Schwannoma is a benign peripheral nerve tumor with microscopic features of sheets of spindle-shaped cells with nuclear palisading. However, when a pathologist encounters a salivary gland tumor with microscopic morphological features of biphasic components with areas of schwannoma-like morphology, it is crucial to make a prudent diagnosis by differentiating Schwannoma-like pleomorphic adenoma and its differential diagnosis as they have remarkably different outcome in patients. We herein present a case of Schwannoma-like pleomorphic adenoma and discuss its approach to diagnosis and literature search.

Histopathological response to denosumab in giant cell tumours of bone - A review of 11 cases.

Background: Giant cell tumor (GCT) of the bone is a locally aggressive primary bone tumor, that can rarely metastasize. Arising mostly in epiphysis of the long bones in young adults, the tumor is composed of mononuclear cells that are admixed with osteoclastic giant cells(OLGCs), which express RANK ligand and RANK respectively. Denosumab a monoclonal antibody against RANK ligand has been shown to reduce the tumor by causing bone lysis by inhibiting RANKL. Histological changes in 11 patients of GCT who were treated with denosumab are presented here. Materials and Methods: Clinical records and slides of 11 patients of GCT who had been administered neoadjuvant denosumab were included in the study. Evaluation of pre and post therapy GCT specimens was performed by two pathologists (RK and VM). There were 4 males and 7 females. Their mean age was 30 years. All the patients received 120 mg denosumab subcutaneously every week with additional 120 mg on days 8 and 15 of therapy. The histological slides were reviewed and following points noted: 1) degree of ossification,2) fibrosis,3) loss of osteoclastic giant cells,4) proliferation of mononuclear cells,5) atypia,6) Permeation of osteoid by malignant cells. Results: Out of 11 cases, 2 cases did not show any significant histological improvement. 7 cases showed reduction in giant cells, increased fibrosis, enhanced mononuclear cell proliferation and ossification consistent with a pathological response. Atypia and osteoid permeation were noted in 2 cases which showed transformation to osteosarcoma. Conclusion: Denosumab treated giant cell tumor show dramatic histological changes. The post therapy lesions may bear no resemblance to pretherapy lesion. There may be complete resolution or may be confused with benign or malignant lesions Rarely they may show sarcomatous transformation. It is imperative that the pathologist is aware of these changes to prevent diagnostic pitfalls as it poses therapeutic and prognostic implications.

Role of CD44 as cancer stem cell marker in triple-negative breast cancer and its association with histological grade and angiogenesis.

Introduction: Cancer stem cells (CSC) within the tumors play a central role in tumorigenesis. It is, thus, of utmost importance to identify these cells to develop effective cancer therapy. Triple-Negative Breast Cancer (TNBC) is an aggressive molecular subtype of breast cancer associated with poor patient outcomes. The role of CD44 immunohistochemistry (IHC) as a putative CSC in breast carcinomas, particularly of the TNBC-subtype is ambiguous, with equivocal results. Aims and Objectives: The present study aims to assess the role of CSC in breast carcinoma by immunohistochemical analysis of CD44 expression in TNBC. The association of TNBC expressing CSC with histological grade as well as with angiogenesis (using CD34 IHC) has been studied. Materials and Methods: Biopsy samples from 58 patients with infiltrating ductal carcinoma, NST were studied. The histology of the tumor was sub-classified into grades 1-3. Based on immunohistochemical analysis (ER, PR, HER2/Neu), the cases were divided into TNBC and NTNBC groups. The tissue sections were also subjected to analysis for CD44 to identify the CSC-phenotype and CD34 to evaluate angiogenesis, to determine the microvascular density (MVD). Results: Out of the 58 cases in the study, 28 were TNBC and 30 were NTNBC. CSC phenotype (CD44 positive) was expressed significantly higher in the TNBC (78%) versus the NTNBC (53%) (p-value 0.043). The MVD estimated using CD34 IHC was lower in the TNBC group in our study, though the difference was not statistically significant. A larger proportion of cases in TNBC showed a higher histological grade (35%) in comparison to NTNBC (27%). However, statistically, it was not significant. Conclusion: Our study demonstrated that CD44 as a CSC marker is seen significantly more in the TNBC category of invasive ductal carcinomas. Further large-scale studies, to confirm these findings, will be of potential therapeutic and prognostic value.

Bizarre parosteal osteochondromatous proliferation Nora's lesion: Case report of two cases with review of the literature.

ABSTRACT: Bizarre parosteal osteochondromatous proliferation (BPOP) is also known as Nora's disease. It is a benign lesion. Even though recent studies showed probable neoplastic etiology, the exact cause is unknown. BPOP commonly involves small bones of hands and feet. This condition is rare and very few cases are reported. In this report, two cases are presented with clinical, radiological, and histopathological findings. The first case was a 38-year-old female presented with 3-year history of mild painful swelling in the left middle finger and the second case was a 28-year-old male with the left leg swelling for 8 years. On radiology, both cases showed surface lesion with uninvolved medullary cavity. Excision specimen of both the lesions subjected for histopathological examination. Microscopically, there was irregular maturation of the bone and cartilage. Cartilage showed purplish-blue color (blue bone) with bizarre chondrocytes. BPOP is a rare benign condition. Awareness of clinical radiological and microscopic findings is needed for correct diagnosis and to differentiate it from other mimicking benign and malignant conditions.

Utility of Glasgow Microenvironment Score as a prognostic tool in colorectal carcinoma.

Background: Colorectal carcinoma (CRC) is third most common malignancy in the world. The presence of Lymphocytes particularly at the invasive margin of the tumor have been associated with good immune response indicating better prognosis. The relative tumor stroma is also important in deciding the course of the disease. The Glasgow Microenvironment Score (GMS) comprises of assessment of tumor cell infiltrate using Klintrup-Makinen (KM) grade and tumor stroma percentage. Aims: The aim of the present study is to evaluate the utility of GMS score in relation to parameters of adverse histopathological outcome in carcinoma colon that is grading, staging, LVI, PNI and nodal metastasis. Setting and Design: Colectomy specimens received over 3 year period were subjected to microscopic evaluation for LVI, PNI, grade, stage & lymph node metastasis. Materials and Methods: Lymphocytes at the deepest invasive margin of tumor were counted as per KM score in 5 HPF by two independent pathologists . Patients were classified as Low grade (0/1) or high grade (2/3) response. Tumor stroma percentage was calculated as stroma poor (<50%) and stroma rich (>/= 50 %). The GMS was scored by combining the two and scored 0, 1 and 2. Statistics and Results: Total 37 patients with no prior therapy were included out of which 23 were males and 14 females. 15 patients (40.54%) had GMS of 0, 6 (16.21%) had GMS 1 and 16 (43.24%) had GMS 2. A high GMS was associated with LVI (P = 0.02), PNI (P = 0.01) lymph node metastasis (P = 0.003). However, no significant association was found between GMS with Grade (P = 0.98) and Stage (P = 0.36). Conclusion: Low GMS was associated with good outcome and high GMS with poor outcome. This score can be used for risk stratification, can be of clinical utility and may be applied to pathological descriptions of CRC.

Metaplastic breast carcinoma: Analysis of clinical and pathologic features, a five-year study.

Background: Metaplastic carcinoma breast (MCB) is a rare tumor comprising of both glandular and non-glandular patterns with epithelial and or mesenchymal components. Due to their varied clinicomorphological features, diagnosis has been challenging. Aim: To study the clinicopathological and histomorphology of cases of metaplastic carcinoma breast diagnosed in a tertiary care hospital along with literature review. Materials and Methods: This is a retrospective study including data of 11 patients who were diagnosed with MCB either on trucut or mastectomy specimens conducted between January 2014 and December 2018. Results: The study includes 11 patients, out of which 10 were diagnosed on mastectomy while one on trucut specimen. All the patients were women with the mean age of presentation being 43.8 years. The most common presentation was palpable breast lump with mean tumor size of 7.3 cm in greatest dimension. Skin involvement was seen in 36.3% of cases. While eight cases (72.7%) were classified as epithelial, three (27.2%) were classified as mixed. Amongst epithelial variety, in eight cases, squamous component was seen along with infiltrating ductal carcinoma (IDC) while one was pure squamous type. In mixed variety, one case showed spindled areas along with squamous areas and areas of IDC. Other two showed focal sarcomatous and cartilaginous areas in one and angiosarcomatous, bone and cartilage formation admixed with areas of IDC in other case. Conclusion: MCB are rare breast tumors with aggressive course and are characterized by their large size and rapid growth rate. Recently, there has been an upsurge in the cases of MCB due to increase in recognition of this entity. It has to be distinguished from other tumors by the pathologists so as to guide proper treatment.

Mucinous carcinoma of the gallbladder- two rare case reports.

Gallbladder carcinomas are uncommon with female predominance in the sixth or seventh decades. Mucinous carcinomas of the gallbladder are rare with few cases documented in the literature. We present two cases of mucinous carcinoma of the gallbladder one of which was incidentally detected. The first case is a 65-year-old female who was diagnosed clinically with a suspected case of carcinoma of the gallbladder with the help of contrast-enhanced computed tomography. Microscopy showed extensive areas of mucin pools (>90% of the tumor), atypical cells in three-dimensional clusters, and scattered singly in the mucin pools. Immunohistochemistry revealed a strong expression of MUC2 by the tumor cells. The second case is a 60-year-old female who was diagnosed clinically with a case of chronic cholecystitis; however, the cholecystectomy specimen showed a mucinous growth in the fundus and body of the gallbladder, microscopy of which showed extensive mucin pools (>90% of the tumor) along with scattered signet-ring cells. Both the cases were reported as mucinous carcinoma of the gallbladder. Mucinous carcinoma of the gallbladder exhibit more aggressive behavior than conventional gallbladder carcinomas. Documentation of these rare cases will encourage further research to find out their true incidence and adequate management of the patient.

Dermatofibrosarcoma protuberans: Clinicopathological spectrum with emphasis on lymph node metastasis and fibrosarcomatous transformation.

Introduction: Dermatofibrosarcoma protuberans (DFSP) is an uncommon skin tumor with a low-to-intermediate grade of malignancy, characterized by progressive growth and a propensity for local recurrence. In this series, we are analyzing the clinicopathological spectrum of DFSP cases. Materials and Methods: A retrospective study of 12 patients with DFSP who were diagnosed at our institute over the last 2 years (2018-2020) was performed. Results: The clinicopathological spectrum and immunohistochemistry of DFSP cases were studied with one case with lymph node metastasis, which is a rare entity, and two cases of the fibrosarcomatous DFSP were also diagnosed and warrant a special mention. Conclusion: Pathologists should be aware of metastasis and unusual variants while reporting dermatofibrosarcoma.

Anaplasia in childhood rhabdomyosarcoma: An under reported entity.

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype. Design: A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia. Results: Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia. Conclusion: Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.

Primary breast sarcoma: A case series.

BACKGROUND: Primary Breast Sarcomas (PBS) are rare malignancies and seen in less than <1 % of all breast malignancies. PBS are non epithelial, composed of mesenchymal mammary tissue and are difficult to diagnose from other sarcomas arising in breast. MATERIALS AND METHODS: A retrospective study was conducted in the Department of Pathology and slides of breast malignancies over a period of 5 years were reviewed. Out of total 1570 breast malignancies, 5 cases were reported as PBS. Diagnosis was made on the basis of Histopathology and IHC findings. RESULTS: Out of total 1570 cases, 5 cases were diagnosed as PBS (i.e. 0.32% of all cases). 3 out of 5 cases were males comprising of 60% of cases and 2 cases were females accounting for 40% of cases. The age group of presentation was 32-65 years with mean age being 48.5 years. A diagnosis of MPNST was rendered in two cases ( 1=M, 1=F), one each was diagnosed as DFSP ( with fibrosarcoma), Leiomyosarcoma and Fibrosarcoma. CONCLUSION: PBS is an extremely rare entity and locally aggressive. It requires diagnosis as its treatment protocol is different.

Acinic cell carcinoma of the parotid gland with neuroendocrine differentiation.

Acinic cell carcinoma (ACC) is a malignant salivary gland tumor characterized by tumor cells displaying acinar features. Usually presenting as a slow-growing tumor, ACC, however, may show dedifferentiation to a higher grade including neuroendocrine carcinoma. In addition, ACC may rarely show focal neuroendocrine differentiation without any frank evidence of neuroendocrine carcinoma. We describe such a case of ACC of the parotid gland in a 65-year-old female, which showed neuroendocrine differentiation. The diagnostic clues, immunohistochemistry panel, and prognostic and treatment aspects are also presented.

Histomorphological patterns of hepatoblastoma in a tertiary care hospital.

BACKGROUND: Hepatoblastoma (HB), rare malignancy in itself, is the most common primary liver tumor in children. Most common presenting features are abdominal distension or abdominal mass. Several patterns are associated with HB with different prognosis. Furthermore, some patterns have overlapping features with other childhood tumors. AIMS AND OBJECTIVES: The aim of this study is to discuss various patterns of HB which we came across in a tertiary care hospital during our study. H and E slides were reviewed with respect to different patterns, postchemotherapy changes including extramedullary hematopoiesis, necrosis, osteoid metaplasia, necrosis, and fibrosis. CONCLUSION: Different patterns of HB should be kept in mind by the pathologists to avoid any misdiagnosis.

Neuroendocrine carcinomas of the breast: Case series with review of literature.

BACKGROUND: The breast tumors with neuroendocrine differentiation show features similar to their counterparts in other organs. Neuroendorine carcinomas account for less than 0.1% of all breast carcinomas. AIMS: To study the demographics and clinicopathological prameters ten cases showing neuroendocrine carcinoma breast. Material and Methods: Ten cases showing neuroendocrine carcinoma were studied. The data was analysed for demographics and clinicopathological prameters. The Immunohistochemistry for ER, PR, Her2neu, Synaptophysin, Chromogranin, NSE, Ki67 index and EMA were done in these cases. RESULTS: Nine Trucut biopsies were reported as infiltrating duct carcinoma and one case as IDC with neuroendocrine differentiation with focal mucinous areas.The histopathological slides of breast excision specimens revealed clusters of cells arranged in sheets and small nests separated by thin fibrous septae in eight of the cases. Trabeculae were noted in two case and in another rosettes were noted. DCIS component was noted in two cases. Infiltration into fat in five of the cases. One case showed pools of mucin. The tumour cells were positive for synaptophysin in 5/10 cases, chromogranin in 8/10 cases and NSE in 9/10 cases. Estrogen receptor positivity was noted 6 cases (6/10), progesterone receptor positivity in 8 cases (8/10) and Her2neu positivity in 5 cases (5/10). CONCLUSION: NECB cases are more likely to ER/PR positive with variability of expression of neuroendocrine markers. These tumors are more aggressive with propensity for distant metastasis. Endocrine therapy may be more beneficial than standard chemotherapy. Anti-angiogenic markers are an exciting new approach for these case, which is yet to be explored.