Low-grade appendiceal mucinous neoplasms: Histomorphological spectrum in a tertiary care hospital.

BACKGROUND: Low-grade appendiceal mucinous neoplasms (LAMNs) are benign non-invasive epithelial proliferations of the appendix. These usually present clinically as mucoceles and these rarely exceed 2 cm in diameter. Lesions confined to the lumen are labelled as LAMN; however those in which mucin spreads outside the peritoneum are labeled as pseudomyxoma peritonei (PMP). AIMS AND OBJECTIVE: A retrospective study was conducted over a period of three years and all cases of appendectomies were studied. Twelve cases of LAMN were identified, which is a diagnostic dilemma for the pathologists and clinicians. RESULTS: LAMN was identified based on the histopathological features. Out of the 12 cases, 9 were classified as LAMN and 3 as appendiceal neoplasm with PMP. There was villous or flat proliferation of epithelial lining, loss lymphoid aggregates, and dissecting mucin within muscularis. CONCLUSION: LAMNs are rare neoplasms of the appendix, with clinical presentation similar to acute appendicitis. Mucinous collections within the appendiceal wall should be extensively searched for mucosal changes and, if found, should prompt a careful search for pushing invasion of LAMNs. A thorough and vigilant gross examination can be of great help. Appendicectomy is the treatment of benign and grossly intact mucinous neoplasm.

Primary mucosal malignant melanoma (PMMM) of nasal cavity: A report of two cases.

ABSTRACT: Primary mucosal malignant melanoma of the nasal cavity is a rare tumor with aggressive behavior and a dismal prognosis. An extremely rare tumor that accounts for 0.7% to 1% of all melanomas in Caucasian populations and between 4% and 8% of malignant tumors of the nasal cavity and paranasal sinuses. Taking into account the rarity, it is important to note that malignant melanoma should be considered when making a differential diagnosis of tumors of the nose and paranasal sinuses. Two cases of primary malignant melanoma of the nasal cavity both arising in females, one in a 60-year-old and the other in a 64-year-old, both of whom presented with nasal obstruction and brief symptomatic epistaxis are being presented here. The diagnosis being confirmed by a histopathological examination along with an immunohistochemical analysis by using S100 and HMB45.

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.

Direct immunofluorescence (DIF) versus immunohistochemical (IHC) staining of complements and immunoglobulins (Ig) in pemphigus group.

INTRODUCTION: Pemphigus is a group of bullous disorders of the skin characterized by the formation of autoantibodies present in the intercellular junction of the epidermis. Diagnosis is made by clinical, histopathological examination, and DIF. As DIF needs frozen sections, fluorescent tagged antibodies, UV light microscope for examination, and trained personnel, its non-availability makes a definitive diagnosis challenging. AIMS AND OBJECTIVES: To evaluate the utility of IHC staining of complements and Ig in cases of Pemphigus. MATERIALS AND METHODS: Twenty-six diagnosed cases of Pemphigus were stained by Peroxidase immunohistochemical method using monoclonal antibody to IgG, IgA, IgM, IgG4, C3, C4 d with DAB as chromogen. Pemphigus cases include twenty of pemphigus vulgaris (PV), four cases of pemphigus foliaceous (PF), and two of pemphigus vegetans (Pveg). Positivity was defined as the deposition of Ig and complements as distinct, continuous brown staining of keratinocytes at intercellular junctions. RESULT: On IHC total of 20 PV 17 showed positivity (85%) for IgG, 11 (55%) C4d, 19 (95%) C3d, and 16 (80%) IgG4 deposits at the intercellular junction of the epidermis. All cases of PF showed a deposit of IgG, with three (75%) cases for IgG4, C3d, and C4d. Both cases of Pveg showed positivity for IgG and C4d while one case was negative for IgG4 and C3d. The overall IgG, C3, IgG4, and C4d expression for pemphigus was seen in 88%, 88%, 76.9%, and 61.5% of cases. The relation between these markers, combination of IgG and C3, was best related to each other ( P value = 0.80). The sensitivities for IgG, IgG4, and C3 were 77.8%%, 73%, and 73% resp. CONCLUSION: We conclude that IHC is a useful tool in the diagnosis of PV with the highest sensitivity of IgG and C3d. The combination of IgG and C3d could replace the DIF in almost all of our cases, so IHC on FFPE sections be used as an alternative method to DIF.

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.

Sebaceous carcinoma with apocrine differentiation arising in a known case of basal cell carcinoma: A rare entity.

Sebaceous carcinoma is a ra malignant tumor of adnexal origin arising from sebaceous glands. It is most commonly seen arising from the eyelids and head and neck. It is predominantly seen in females with an average age of around 65 years. Apocrine differentiation in sebaceous carcinomas is rare but has been reported in the literature. Here, we present a case of sebaceous carcinoma with apocrine differentiation in a 62-year- old female who was a diagnosed case of basal cell carinoma.

Epithelial myoepithelial carcinoma: A case report.

ABSTRACT: Epithelial myoepithelial carcinoma is a rare tumor with the parotid gland being the most common primary site of origin. The tumor may also very rarely originate in minor salivary glands of the soft palate. Due to the rarity of its occurrence and histogenesis, clear-cut therapeutic guidelines are not defined. The present report describes the case of a 56-year-old female patient who was diagnosed to have epithelial myoepithelial carcinoma (EMC) of the minor salivary gland of the soft palate, Stage T2 N0 M0 (Stage group II). The patient was treated with radical radiotherapy followed by adjuvant chemotherapy and is alive with no evidence of disease after 12 months following the end of treatment.

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.

Infantile Fibrosarcoma of the Tongue Base.

Infantile fibrosarcoma is an uncommon soft tissue tumour in infants and children mostly located in the extremities. Its occurrence in the head and neck, particularly in the oral cavity is extremely rare. Here, we report a case of infantile fibrosarcoma of the tongue base in a 9-year-old boy and discuss the complexities in arriving at diagnosis and its management. The patient underwent wide local excision of the tumour. Histopathology was essential in confirming the diagnosis. As per search in English literature, this is the first case of infantile fibrosarcoma of the tongue base to be reported till date. Treatment of choice is wide local excision with or without chemotherapy.

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.

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.

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.

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.

Cytological Diagnosis of Malignant Mesothelioma: A Case Series.

Background: Mesotheliomas are neoplasms of the serosal lining of the body cavities. Diagnosis requires a multimodal approach of clinical findings, cytology, and histopathology with immunohistochemistry (IHC). The published sensitivity of cytology for diagnosing mesothelioma ranges from 30% to 75%. Aim and Objectives: This study aimed to calculate the incidence of malignant mesothelioma (MM) at our institute and to study the cytological features of MM. Materials and Methods: A retrospective study of pleural, peritoneal, and pericardial fluids submitted at our institute was done. The duration of the study was 8 years (2011-2019). Apart from examining Giemsa smears, a panel of immunocytochemical (ICC) and cell block immunohistochemical (IHC) markers was applied to achieve the diagnosis. These included calretinin, mesothelin, CK5/6, Hector Battifora mesothelial cell antibody (HBME), WT1, MOC31, CK7 and CK20. Histopathological correlation was done wherever possible. Result: In the present study, we compiled four cases of MM over 8 years diagnosed on serous effusion cytology and confirmed by immunocytochemistry (ICC)/cell block immunohistochemistry (IHC)/biopsy. This indicates a rare incidence of MM. The Cytological features of MM were studied. Conclusion: The diagnosis of MM is difficult, especially cytologically. It was found to be a rare entity in the malignant cases diagnosed on effusion cytology.

Primary leiomyosarcoma kidney - A rare entity with a diagnostic challenge.

Primary leiomyosarcoma kidney is a rare tumor with an aggressive nature. Leiomyosarcoma is one of the common histologic types of the sarcomas, comprising 60% of all sarcomas. Sarcomatoid renal cell carcinoma is a close differential of renal leiomyosarcoma as both tumors have spindle-shaped cells. The former has a more pleomorphic character with nuclear grade four, which can be differentiated on the basis of immunohistochemistry. Hence, the diagnosis of primary renal leiomyosarcoma poses a diagnostic challenge. One such case of renal leiomyosarcoma in a 45-year-old male is being discussed here.

USG Guided Fine Needle Aspiration Cytology along with Immunocytochemistry to Diagnose Primary Malignant Mixed Mullerian Tumors: A Three-Year Study from a Tertiary Care Center.

Aims and Objectives: To study the diagnostic utility of fine-needle aspiration cytology (FNAC) and immunocytochemistry in diagnosing primary malignant mixed Mullerian tumors (MMMT). Materials and Methods: A 3-year retrospective study carried out in a tertiary care hospital, which included all the gynecological patients who underwent USG-guided FNAC of their abdominopelvic masses. Observations and Results: Out of the 324 total cases, 05 (1.5%) were reported as primary malignant mixed Mullerian tumors. Out of these 05 cases, 03 were ovarian, 01 was uterine, and 01 involved both uterus and one-sided adnexa. The FNA smears from the masses revealed cytomorphological features of a biphasic neoplasm with elongated pleomorphic spindle cells and dispersed, focal attempted acinar pattern, thus indicating the possibility of MMMT. Immunocytochemistry was further carried out which showed both vimentin and cytokeratin positivity. The diagnosis was confirmed on subsequent biopsy and immunohistochemistry (without any histopathological-cytological discrepancy). Conclusion: Though the literature is replete in establishing a histo-pathological diagnosis of MMMT, the diagnosis on USG-guided FNAC has been rarely described. Emphasis should be made on the careful examination of small sarcomatous elements in smears. Utilization of cell block and immunocytochemistry with histopathological correlation should be done to avoid misdiagnosis.