Immature Teratoma of Ethmoid Sinus Masquerading as Antrochoanal Polyp: A Rare Case Report with Literature Review.

Teratomas are rare neoplasms that arise from totipotent stem cells. Teratomas of the head and neck are extremely rare, constituting about 10% of all cases and usually present in the neonatal period. Extensive literature search has shown that there are only two cases reportedof teratoma of the ethmoid sinus; one as a mature teratoma in a neonate and another was histologically immature teratoma in an adult male (Mwang'ombe et al. in East Afr Med J 79(2):106-107, 2002; Aggarwal et al. in J Postgrad Med 59(2):138-141, 2013). We hereby report the second case of immature teratoma of ethmoid sinus origin in an adult male.

Multicystic renal dysplasia, a histomorphological spectrum: Seven years experience from a tertiary care hospital.

OBJECTIVES: Multicystic dysplastic kidney (MCDK) is defined as the presence of multiple noncommunicating cysts of various sizes, detected sonographically, without evidence of functioning renal parenchyma on dimercaptosuccinic acid renal scan. It has an incidence of 1:4000 live births. They are more commonly diagnosed in boys, usually on the left side, but may also be bilateral. There is the presence of primitive ducts surrounded by fibromuscular connective tissue. These are because of the disturbed connection of the ureteric bud with renal blastema and abnormal division at the stage of metanephros, resulting in an abnormal metanephros differentiation. MATERIALS AND METHODS: Thirty cases of MCDK were included to study their histomorphology along with their clinical features. Cases were retrieved from the last seven years (2015-2021) from the Department of Pathology, Maulana Azad Medical College. RESULT: Age ranged from 10 days to 18 years. The cases were between 1 years and 5 years of age. Six out of 30 cases (20%) were infants with three of them being neonates. Twenty-one cases were males. All the cases had unilateral kidney involvement with the left kidney being involved in 20 out of 30 cases. Twenty-eight cases underwent nephrectomy in view of small contracted nonfunctional kidneys with one of them being horseshoe shaped. Five cases had associated hydronephrosis (two ipsilateral and three bilateral). One case had Hirshprung's disease, four had anorectal malformation, two had posterior urethral valves with vesicourethral reflux, one had duplex moiety, and one had undescended testes. On histopathological examination, all of them showed the presence of immature disorganized tubules surrounded by a collarette of immature mesenchymal stroma. One of the cases showed osteoid formation and four had areas of immature cartilage. Normal kidney parenchyma was seen at the periphery in four cases. CONCLUSION: This series has been presented to highlight the various histomorphological features of MCDK. MCDK can be managed conservatively in most of cases due to autoinvolution and, hence, needs to be differentiated from other close differentials like polycystic kidney disease, cystic nephroma, and cystic partially differentiated nephroblastoma in order to avoid unnecessary surgical intervention.

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.

Leiomyosarcoma of sphenoid sinus- A rare tumor at an unusual site.

Leimyosarcoma (lms) is a malignant soft tissue tumor of smooth muscles. The tumor arises intramuscularly and in subcutaneous locations. It is unusual to encounter lms in head and neck region, even more infrequent to discover lms in nasal and paranasal sinuses. A case of 28 years old male with leiomyosarcoma originating from sphenoid sinus with intracranial extension is being presented with aim to highlight its rarity and to highlight the differential diagnosis and the need for prudent diagnosis in the work-up of the patient.

Pancreatic heterotropia in gallbladder with adenocarcinoma: A rare incidental finding.

ABSTRACT: Heterotopic pancreas is defined as the presence of aberrant pancreatic tissue present outside the pancreas without connection to its neural, vascular, and anatomic location. The commonly seen locations are stomach, duodenum, jejunum, Meckel commonly seen locatio, and ampulla of Vater. The gallbladder is an extremely rare site for pancreatic heterotropia. Its association with adenocarcinoma has not been described yet. A case of 50-year-old male with pancreatic heterotropia along with carcinoma gallbladder is being presented here.

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.

Coexisting ovarian serous cystadenoma with fibroma: A very unusual combination.

Surface epithelial neoplasms are the most common ovarian tumors, constituting around 60% of all ovarian malignancies. They are classified as benign, borderline, and malignant. Ovarian cystadenomas are common benign epithelial neoplasms which carry an excellent prognosis. Ovarian thecoma-fibroma groups are uncommon sex cord-stromal neoplasms, constituting 1.0%-4.0% of all ovarian tumors. Most of them are benign and often found in postmenopausal patients. Combination tumors in the ovary are known. The most common combination is mucinous cystadenoma which occurs in association with Brenner tumor, mature cystic teratoma, Sertoli-Leydig cell tumor, or even a serous cystadenoma. A combination of surface epithelial and thecoma-fibroma group is very rarely encountered. A case of one such combination of serous cystadenoma and fibroma of the ovary is being presented here in a postmenopausal woman.

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.

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.

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.

Osteosarcoma of the jaw: Primary versus secondary - A report of two cases.

ABSTRACT: Osteosarcoma (OS) is the most common primary malignancy of bone excluding hematological malignancies. Most common sites of tumor are long bones of extremities. OS of the jaw are extremely rare with mandible being more commonly after than maxilla. Hereby, we present two cases of OS of jaw with one patient being male and other female.

Primary neuroendocrine tumors of ovary: A clinicopathological spectrum in a tertiary care hospital with review of literature.

AIM: Primary neuroendocrine tumors in the ovary are rare. These tumors arise from the neuroendocrine cell system of ovarian stroma and surface epithelium, and may also arise from teratoma. We present four primary ovarian neuroendocrine tumors and discuss clinical, histomorphological, and immunohistochemical findings. METHODS: Four primary ovarian neuroendocrine tumors were identified from our 4-year departmental archives. H and E slides and immunostains were reviewed. Clinical history, imaging studies, and follow-up data were obtained from medical records. RESULTS: Patients' age ranged from 37 to 45 years. All the patients presented with abdominal discomfort, abnormal uterine bleeding, and unilateral or bilateral ovarian masses. Two of the cases were primary ovarian carcinoid tumors, one being the pure primary ovarian carcinoid tumor (PPOCT), and the other being associated with mature cystic teratoma, while the other two cases were neuroendocrine carcinoma. All the cases showed expression of neuroendocrine markers, namely, synaptophysin, chromogranin, and NSE. CONCLUSIONS: Primary neuroendocrine tumors in the ovary are rare and consist of a group of heterogeneous tumors that express similar immunohistochemical markers.

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.

Transformation of diffuse large B-cell lymphoma to lymphoblastic lymphoma.

Transformations in diffuse large B-cell lymphoma (DLBCL) are extremely rare. Here, we are presenting a very rare case of DLBCL transforming into lymphoblastic lymphoma (LBL) diagnosed by fine-needle aspiration cytology (FNAC) and flow cytometry. A 31-year old male on antiretroviral therapy and a known case of diffuse large B-cell lymphoma diagnosed 1 year back on cervical lymphadenopathy, presented with left axillary swelling for 3 months. FNAC and Flow cytometry were performed from the left axillary swelling which confirmed the diagnosis of LBL.

Sarcomatous Nodules Associated with Mucinous Neoplasms of Ovary: A Report of Two Cases.

Ovarian mucinous neoplasms (benign/borderline/malignant), one of the common surface epithelial tumours of ovary, at times can be associated with mural nodules. These mural nodules can either be sarcomatous, sarcoma like or anaplastic carcinomatous. The nodules can be single or multiple and appear solid, white to brown in color. Sarcomatous nodules tend to occur in older patients and microscopically are poorly circumscribed, comprising of a population of pleomorphic spindled cells. Two cases of sarcomatous nodules associated with ovarian mucinous neoplasm are being presented here.

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.

Nasopharyngeal Carcinoma With Bilateral Breast Metastasis: A Report of an Elusive Case.

Nasopharyngeal carcinoma is a prevalent head and neck cancer, especially in Southeast Asia. Although its potential for distant metastasis is well established, metastasis to the breast has seldom been reported. To the best of our knowledge, this is the fourth report of a case of nasopharyngeal carcinoma metastasizing to bilateral breasts. A 35-year-old patient presented with left nasal obstruction, epistaxis, and a palpable mass in her left breast, without any cervical or axillary lymph nodal enlargement. Radiological examination with contrast-enhanced computed tomography scan and magnetic resonance and imaging of breast revealed the presence of enhancing mass lesions in bilateral breasts. Histopathology of the nasal mass was suggestive of undifferentiated nasopharyngeal carcinoma. Breast fine needle aspiration revealed an abundance of metastatic squamous cells. Immunohistochemistry examination was positive for chromogranin A, synaptophysin, and cluster of differentiation-56, confirming the diagnosis of a primary nasopharyngeal malignancy metastasizing to bilateral breasts. Differentiation between metastatic disease and a coexisting second primary is imperative for planning appropriate treatment and defining the further outcomes.

Diagnostic utility of flow cytometry in identifying histiocytic sarcoma-A case report.

Histiocytic sarcoma (HS) is a malignant neoplasm of hematopoietic origin. It is an exceedingly rare and aggressive malignancy commonly seen in adults. Diagnosis is difficult owing to lack of specific clinical manifestations with the absence of precursor lesions or causative agents. Hence, it primarily relies on histopathological morphology combined with immunohistochemistry, which is time-consuming, hence resulting in delayed treatment. However, diagnostic utility of flow cytometry is not well established in this. We report a case of a 45-year-old man who presented with right axillary lymphadenopathy for 1 month. FNAC was performed on the axillary lymph node, which showed large, atypical lymphoid/histiocyte-like cells. On flow cytometry, these cells were CD64+, CD11c+, and CD45+ suggesting histiocytic sarcoma. Similar morphology was seen on incisional biopsy. On immunohistochemistry, the cells were negative for B and T cell markers, PAX5, EMA, CK, ALK, and CD1a and expressed CD68, S100, and CD11c. A diagnosis of histiocytic sarcoma was made. Hence, flow cytometry can be a highly effective and powerful tool for the early detection of HS and can help in prompt treatment, given its aggressive clinical course and low survival interval.

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.