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Pathology - Plasma cell myeloma
Definition: A diffuse neoplasm of plasma cells originating from bone marrow, characterized by the presence of paraproteins in serum and/or urine.
Epidemiology • Incidence ranges from 3 to 5 per 100,000 individuals. •
Manifestations are observed in older persons, with a mean diagnostic age of 70 years. • There is a masculine predominance of 1.5:1.
Aetiology • Unidentified.
Pathogenesis The neoplastic plasma cells produce cytokines that activate osteoclasts, resulting in lytic bone lesions. Circulating paraprotein inhibits normal immunoglobulin synthesis, hence elevating the susceptibility to infections. Free light chains traversing the kidneys contribute to renal failure.
Presentation: • Ostealgia and recurring infections. • Anemia, elevated ESR, hypercalcemia, and renal dysfunction are prevalent.
Histopathology A definitive diagnosis necessitates a bone marrow biopsy. • The bone marrow exhibits an abundance of monoclonal plasma cells organized in clusters, nodules, or sheets. • Clonality can be confirmed immunohistochemically by demonstrating kappa or lambda light chain restriction.
Prognosis • Myeloma is an incurable condition. • Average survival is 3–4 years post-diagnosis.
Definition: A diffuse neoplasm of plasma cells originating from bone marrow, characterized by the presence of paraproteins in serum and/or urine.
Epidemiology • Incidence ranges from 3 to 5 per 100,000 individuals. •
Manifestations are observed in older persons, with a mean diagnostic age of 70 years. • There is a masculine predominance of 1.5:1.
Aetiology • Unidentified.
Pathogenesis The neoplastic plasma cells produce cytokines that activate osteoclasts, resulting in lytic bone lesions. Circulating paraprotein inhibits normal immunoglobulin synthesis, hence elevating the susceptibility to infections. Free light chains traversing the kidneys contribute to renal failure.
Presentation: • Ostealgia and recurring infections. • Anemia, elevated ESR, hypercalcemia, and renal dysfunction are prevalent.
Histopathology A definitive diagnosis necessitates a bone marrow biopsy. • The bone marrow exhibits an abundance of monoclonal plasma cells organized in clusters, nodules, or sheets. • Clonality can be confirmed immunohistochemically by demonstrating kappa or lambda light chain restriction.
Prognosis • Myeloma is an incurable condition. • Average survival is 3–4 years post-diagnosis.
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Pathology - Primary myelofibrosis
A myeloproliferative neoplasm distinguished by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, accompanied by reactive deposition of fibrous connective tissue and extramedullary hematopoiesis.
Epidemiology • Estimated yearly incidence ranges from 0.5 to 1.5 per 100,000 individuals. • Predominantly affects people aged 60 to 70, with no discernible gender preference. Aetiology • Predominantly unknown in the majority of instances. Genetics • No specific genetic anomaly has been identified.
Presentation: Abdominal discomfort resulting from significant splenomegaly. • Nocturnal hyperhidrosis, pyrexia, and weight reduction.
Peripheral blood • Increased platelets and/or white cell count. • Decreased hemoglobin. The blood film exhibits leukoerythroblastosis characterized by teardrop-shaped red blood cells. Bone marrow • Megakaryocytes exhibit significant abnormalities characterized by extensive clustering and pronounced cytological atypia. • Progression is accompanied by an increase in marrow fibrosis.
Prognosis • Survival is contingent upon the degree of marrow fibrosis at the time of diagnosis. Patients exhibiting significant fibrosis have median survival durations of 3 to 7 years. The primary causes of mortality are bone marrow failure, thromboemboli, and the onset of acute myeloid leukemia (AML).
A myeloproliferative neoplasm distinguished by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, accompanied by reactive deposition of fibrous connective tissue and extramedullary hematopoiesis.
Epidemiology • Estimated yearly incidence ranges from 0.5 to 1.5 per 100,000 individuals. • Predominantly affects people aged 60 to 70, with no discernible gender preference. Aetiology • Predominantly unknown in the majority of instances. Genetics • No specific genetic anomaly has been identified.
Presentation: Abdominal discomfort resulting from significant splenomegaly. • Nocturnal hyperhidrosis, pyrexia, and weight reduction.
Peripheral blood • Increased platelets and/or white cell count. • Decreased hemoglobin. The blood film exhibits leukoerythroblastosis characterized by teardrop-shaped red blood cells. Bone marrow • Megakaryocytes exhibit significant abnormalities characterized by extensive clustering and pronounced cytological atypia. • Progression is accompanied by an increase in marrow fibrosis.
Prognosis • Survival is contingent upon the degree of marrow fibrosis at the time of diagnosis. Patients exhibiting significant fibrosis have median survival durations of 3 to 7 years. The primary causes of mortality are bone marrow failure, thromboemboli, and the onset of acute myeloid leukemia (AML).
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Pathology - Classical Hodgkin lymphoma
A lymphoid tumor characterized by dysfunctional neoplastic B-cells, referred to as Hodgkin/Reed Sternberg (HRS) cells, situated amid a dense non-neoplastic inflammatory milieu.
Epidemiology • Bimodal age distribution, characterized by a high incidence between 15 and 35 years, and a secondary peak in later life. • Males are predominantly affected, except in the case of the nodular sclerosis variation, which exhibits equal incidence across genders.
Aetiology • Unknown; although, Epstein-Barr virus (EBV) infection has been associated with certain kinds.
Presentation • The majority of patients have localized lymphadenopathy. • Fever, nocturnal diaphoresis, and weight loss are prevalent (referred to as 'B symptoms').
Histopathology • Lymph nodes are infiltrated by varying quantities of neoplastic HRS cells amid a robust inflammatory milieu
The standard diagnosis The Reed-Sternberg cell is a substantial cell characterized by two prominent nuclei, each containing distinct eosinophilic nucleoli. Four histological subtypes are identified based on the quantity and characteristics of the HRS cells and the reactive background: nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted. The immunophenotype of HRS cells is characterized by positivity for CD15 and negativity for CD30, exhibiting a distinctive membranous and Golgi staining pattern. PAX5 and MUM-1 are invariably positive in HRS cells, but CD20 and CD79a are typically negative or expressed at low levels.
Prognosis: Contemporary treatment protocols provide a cure rate over 85% for classical Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is acknowledged as a unique subtype of Hodgkin lymphoma. NLPHL constitutes 75% of all Hodgkin lymphomas. It generally occurs in young to middle-aged adults between the ages of 30 and 50. The atypical B-cells, referred to as lymphocyte-predominant cells, are immunophenotypically differentiated from classical HRS cells; they generally do not express CD30 and CD15, while exhibiting significant expression of CD20 and EMA. The disease progresses slowly and is seldom lethal.
A lymphoid tumor characterized by dysfunctional neoplastic B-cells, referred to as Hodgkin/Reed Sternberg (HRS) cells, situated amid a dense non-neoplastic inflammatory milieu.
Epidemiology • Bimodal age distribution, characterized by a high incidence between 15 and 35 years, and a secondary peak in later life. • Males are predominantly affected, except in the case of the nodular sclerosis variation, which exhibits equal incidence across genders.
Aetiology • Unknown; although, Epstein-Barr virus (EBV) infection has been associated with certain kinds.
Presentation • The majority of patients have localized lymphadenopathy. • Fever, nocturnal diaphoresis, and weight loss are prevalent (referred to as 'B symptoms').
Histopathology • Lymph nodes are infiltrated by varying quantities of neoplastic HRS cells amid a robust inflammatory milieu
The standard diagnosis The Reed-Sternberg cell is a substantial cell characterized by two prominent nuclei, each containing distinct eosinophilic nucleoli. Four histological subtypes are identified based on the quantity and characteristics of the HRS cells and the reactive background: nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted. The immunophenotype of HRS cells is characterized by positivity for CD15 and negativity for CD30, exhibiting a distinctive membranous and Golgi staining pattern. PAX5 and MUM-1 are invariably positive in HRS cells, but CD20 and CD79a are typically negative or expressed at low levels.
Prognosis: Contemporary treatment protocols provide a cure rate over 85% for classical Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is acknowledged as a unique subtype of Hodgkin lymphoma. NLPHL constitutes 75% of all Hodgkin lymphomas. It generally occurs in young to middle-aged adults between the ages of 30 and 50. The atypical B-cells, referred to as lymphocyte-predominant cells, are immunophenotypically differentiated from classical HRS cells; they generally do not express CD30 and CD15, while exhibiting significant expression of CD20 and EMA. The disease progresses slowly and is seldom lethal.
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Pathology - Diffuse large B-cell lymphoma
A mature B-cell neoplasm characterized by big B-lymphoid cells exhibiting a diffuse growth pattern.
Epidemiology: Represents 25–30% of all non-Hodgkin lymphomas. • Primarily impacts senior individuals over the age of 60.
Aetiology • Often unidentified in numerous instances. • Significant correlation with immunodeficiency conditions, including HIV or post-transplant scenarios, where the lymphoma is induced by EBV, HHV-8, or both.
Genetics • Several genetic modifications have been identified in diffuse large B-cell lymphoma (DLBCL). The most prevalent translocation pertains to a segment of 3q that encodes the BCL6 gene.
Presentation: A rapidly enlarging mass that may be nodal (60%) or extranodal (40%). The gastrointestinal system is the most prevalent extranodal site, however almost any location may be involved.
Histopathology • Affected tissues are substituted by extensive sheets of giant atypical lymphoid cells, typically exceeding twice the size of a normal lymphocyte. • Apoptotic debris is commonly observed, and confluent regions of tumor necrosis may be present. Immunophenotype: Positive for B-cell markers PAX5, CD20, and CD79a. Cyclin D1 is absent. • Elevated proliferation index (typically 40–90% of cells).
Prognosis: Survival rates have significantly improved with the introduction of the anti-CD20 inhibitor, rituximab, achieving long-term survival rates of approximately 60–75%. Bone marrow involvement is typically linked to a bad prognosis.
A mature B-cell neoplasm characterized by big B-lymphoid cells exhibiting a diffuse growth pattern.
Epidemiology: Represents 25–30% of all non-Hodgkin lymphomas. • Primarily impacts senior individuals over the age of 60.
Aetiology • Often unidentified in numerous instances. • Significant correlation with immunodeficiency conditions, including HIV or post-transplant scenarios, where the lymphoma is induced by EBV, HHV-8, or both.
Genetics • Several genetic modifications have been identified in diffuse large B-cell lymphoma (DLBCL). The most prevalent translocation pertains to a segment of 3q that encodes the BCL6 gene.
Presentation: A rapidly enlarging mass that may be nodal (60%) or extranodal (40%). The gastrointestinal system is the most prevalent extranodal site, however almost any location may be involved.
Histopathology • Affected tissues are substituted by extensive sheets of giant atypical lymphoid cells, typically exceeding twice the size of a normal lymphocyte. • Apoptotic debris is commonly observed, and confluent regions of tumor necrosis may be present. Immunophenotype: Positive for B-cell markers PAX5, CD20, and CD79a. Cyclin D1 is absent. • Elevated proliferation index (typically 40–90% of cells).
Prognosis: Survival rates have significantly improved with the introduction of the anti-CD20 inhibitor, rituximab, achieving long-term survival rates of approximately 60–75%. Bone marrow involvement is typically linked to a bad prognosis.
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Pathology - Myelodysplastic syndromes
Definition: A collection of hematopoietic neoplasms distinguished by dysplasia in one or more myeloid cell lineages, accompanied by inefficient myelopoiesis, cytopenias, and an elevated risk of acute myeloid leukemia (AML) development.
Epidemiology • Estimated yearly incidence ranges from 3 to 5 per 100,000 individuals. • Predominantly affects older adults, with a median age of 70 years.
Aetiology • Predominantly unknown in the majority of instances. Genetics • Several recurrent chromosomal abnormalities have been identified in myelodysplastic syndromes (MDS). Cytogenetic and molecular analyses are essential for establishing clonality and assessing prognosis.
Presentation • Refractory anaemia is the predominant manifestation. • Neutropenia and thrombocytopenia occur with lesser frequency. 2 Hepatosplenomegaly is rare in myelodysplastic syndromes. Peripheral blood • Cytopenias affecting one or more myeloid lineages. • Blood films may reveal macrocytes and atypical neutrophils with poorly developed nuclear segmentation and hypogranular cytoplasm. Bone Marrow • Morphological indicators of myelodysplasia may be observed in one or more myeloid lineages within the bone marrow. • Dyserythropoiesis is defined by nuclear budding, internuclear bridging, karyorrhexis, multinuclearity, nuclear hypolobulation, megaloblastic alterations, ring sideroblasts, and cytoplasmic vacuolization. • Dysgranulopoiesis is characterized by reduced size, nuclear hypolobation, irregular hypersegmentation, and cytoplasmic hypogranularity. • Dysmegakaryocytopoiesis is marked by reduced size, nuclear hypolobation, or multinucleation.
Prognosis: Survival is contingent upon various criteria, including morphological subtype, karyotype, degree of cytopenia, and age. • Low-risk variants of MDS exhibit a protracted natural history and demonstrate a minimal incidence of progression to AML. • High-risk variants are more aggressive, with numerous individuals rapidly succumbing to bone marrow loss or AML.
Definition: A collection of hematopoietic neoplasms distinguished by dysplasia in one or more myeloid cell lineages, accompanied by inefficient myelopoiesis, cytopenias, and an elevated risk of acute myeloid leukemia (AML) development.
Epidemiology • Estimated yearly incidence ranges from 3 to 5 per 100,000 individuals. • Predominantly affects older adults, with a median age of 70 years.
Aetiology • Predominantly unknown in the majority of instances. Genetics • Several recurrent chromosomal abnormalities have been identified in myelodysplastic syndromes (MDS). Cytogenetic and molecular analyses are essential for establishing clonality and assessing prognosis.
Presentation • Refractory anaemia is the predominant manifestation. • Neutropenia and thrombocytopenia occur with lesser frequency. 2 Hepatosplenomegaly is rare in myelodysplastic syndromes. Peripheral blood • Cytopenias affecting one or more myeloid lineages. • Blood films may reveal macrocytes and atypical neutrophils with poorly developed nuclear segmentation and hypogranular cytoplasm. Bone Marrow • Morphological indicators of myelodysplasia may be observed in one or more myeloid lineages within the bone marrow. • Dyserythropoiesis is defined by nuclear budding, internuclear bridging, karyorrhexis, multinuclearity, nuclear hypolobulation, megaloblastic alterations, ring sideroblasts, and cytoplasmic vacuolization. • Dysgranulopoiesis is characterized by reduced size, nuclear hypolobation, irregular hypersegmentation, and cytoplasmic hypogranularity. • Dysmegakaryocytopoiesis is marked by reduced size, nuclear hypolobation, or multinucleation.
Prognosis: Survival is contingent upon various criteria, including morphological subtype, karyotype, degree of cytopenia, and age. • Low-risk variants of MDS exhibit a protracted natural history and demonstrate a minimal incidence of progression to AML. • High-risk variants are more aggressive, with numerous individuals rapidly succumbing to bone marrow loss or AML.
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Pathology - Follicular lymphoma
Definition: A mature B-cell neoplasm consisting of germinal center cells (centrocytes and centroblasts).
Epidemiology • Represents approximately 20% of all non-Hodgkin lymphomas. • Primarily impacts individuals aged 50 to 60.
Aetiology: Unknown. Genetics: 90% of cases exhibit a distinctive t(14;18) translocation, leading to the fusion of the BCL2 gene with the IGH locus. Deregulated synthesis of the anti-apoptotic Bcl-2 protein leads to clonal proliferation.
Presentation: Extensive lymphadenopathy and splenomegaly. • Patients are often asymptomatic.
Histopathology Nodal architecture is supplanted by contiguous neoplastic follicles. Neoplastic follicles are devoid of mantle zones and consist of haphazardly arranged neoplastic centroblasts and centrocytes. Tangible body macrophages are typically lacking. • Neoplastic cells typically exhibit interfollicular dissemination. Bone marrow involvement is defined by paratrabecular clusters of malignant centrocytes and centroblasts. Immunophenotype PAX5, CD20, and CD79a, which are B-cell markers, exhibit positivity. Bcl-2, Bcl-6, and CD10 are furthermore positive. • CD5, CD23, and cyclin D1 are absent.
Prognosis is contingent upon the severity of the disease and the grade of the tumor. Approximately 25% progression into a high-grade lymphoma, typically diffuse large B-cell lymphoma, correlating with a swift clinical deterioration and mortality.
Definition: A mature B-cell neoplasm consisting of germinal center cells (centrocytes and centroblasts).
Epidemiology • Represents approximately 20% of all non-Hodgkin lymphomas. • Primarily impacts individuals aged 50 to 60.
Aetiology: Unknown. Genetics: 90% of cases exhibit a distinctive t(14;18) translocation, leading to the fusion of the BCL2 gene with the IGH locus. Deregulated synthesis of the anti-apoptotic Bcl-2 protein leads to clonal proliferation.
Presentation: Extensive lymphadenopathy and splenomegaly. • Patients are often asymptomatic.
Histopathology Nodal architecture is supplanted by contiguous neoplastic follicles. Neoplastic follicles are devoid of mantle zones and consist of haphazardly arranged neoplastic centroblasts and centrocytes. Tangible body macrophages are typically lacking. • Neoplastic cells typically exhibit interfollicular dissemination. Bone marrow involvement is defined by paratrabecular clusters of malignant centrocytes and centroblasts. Immunophenotype PAX5, CD20, and CD79a, which are B-cell markers, exhibit positivity. Bcl-2, Bcl-6, and CD10 are furthermore positive. • CD5, CD23, and cyclin D1 are absent.
Prognosis is contingent upon the severity of the disease and the grade of the tumor. Approximately 25% progression into a high-grade lymphoma, typically diffuse large B-cell lymphoma, correlating with a swift clinical deterioration and mortality.
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Pathology - Adrenal cortical adenoma
A nonmalignant epithelial tumor of the adrenal cortex. Epidemiology: The majority of instances manifest in adults, exhibiting no gender preference. The actual incidence figures remain unknown, mostly due to the difficulty in differentiating nodular adrenal hyperplasia from genuine neoplastic adenomas.
Aetiology • Predominantly unidentified in the majority of instances. Genetics • No consistent genetic anomalies have been identified. Presentation • The majority of non-functional tumors are identified accidentally during abdominal imaging conducted for unrelated purposes. Aldosterone-producing adenomas manifest as primary hyperaldosteronism (Conn’s syndrome), which is characterized by hypertension and, in certain patients, hypokalaemia. Cortisol-secreting adenomas manifest as Cushing's syndrome.
Macroscopy • The adrenal gland exhibits a well-defined tumor that may be encapsulated. The median tumor weight is 40 grams. Aldosterone-producing adenomas may exhibit a brilliant yellow coloration, whereas those linked to Cushing’s syndrome may range from yellow to tan. A limited quantity of adenomas exhibit a black coloration, referred to as 'black adenoma.'
Histopathology: The tumors consist of massive polygonal cells organized in nests and trabeculae, interspersed with a delicate vascular network. The cells have cytoplasm that is either transparent and microvesicular or dense and eosinophilic. The nuclei are typically spherical to oval in shape and generally have a bland appearance. • At times, pronounced nuclear pleomorphism may be observed; nevertheless, this does not imply malignant behavior. A compressed ring of normal adrenal cortex may be observed at the periphery.
Prognosis: Adrenal cortical adenomas are benign tumors that lack the potential for malignant behavior. The prognosis is mostly influenced by the severity of the endocrine effects of functional tumors.
Pathological staging of adrenal cortical carcinoma Primary tumour (T)
pT1: tumour 5cm or less in size, localized.
pT2: tumour > 5cm, localized.
pT3: tumour of any size, locally invasive, but not involving adjacent organs.
pT4: tumour of any size, involving adjacent organs.
Regional lymph nodes (N)
N0: no regional lymph node metastasis.
N1: regional lymph node metastasis
A nonmalignant epithelial tumor of the adrenal cortex. Epidemiology: The majority of instances manifest in adults, exhibiting no gender preference. The actual incidence figures remain unknown, mostly due to the difficulty in differentiating nodular adrenal hyperplasia from genuine neoplastic adenomas.
Aetiology • Predominantly unidentified in the majority of instances. Genetics • No consistent genetic anomalies have been identified. Presentation • The majority of non-functional tumors are identified accidentally during abdominal imaging conducted for unrelated purposes. Aldosterone-producing adenomas manifest as primary hyperaldosteronism (Conn’s syndrome), which is characterized by hypertension and, in certain patients, hypokalaemia. Cortisol-secreting adenomas manifest as Cushing's syndrome.
Macroscopy • The adrenal gland exhibits a well-defined tumor that may be encapsulated. The median tumor weight is 40 grams. Aldosterone-producing adenomas may exhibit a brilliant yellow coloration, whereas those linked to Cushing’s syndrome may range from yellow to tan. A limited quantity of adenomas exhibit a black coloration, referred to as 'black adenoma.'
Histopathology: The tumors consist of massive polygonal cells organized in nests and trabeculae, interspersed with a delicate vascular network. The cells have cytoplasm that is either transparent and microvesicular or dense and eosinophilic. The nuclei are typically spherical to oval in shape and generally have a bland appearance. • At times, pronounced nuclear pleomorphism may be observed; nevertheless, this does not imply malignant behavior. A compressed ring of normal adrenal cortex may be observed at the periphery.
Prognosis: Adrenal cortical adenomas are benign tumors that lack the potential for malignant behavior. The prognosis is mostly influenced by the severity of the endocrine effects of functional tumors.
Pathological staging of adrenal cortical carcinoma Primary tumour (T)
pT1: tumour 5cm or less in size, localized.
pT2: tumour > 5cm, localized.
pT3: tumour of any size, locally invasive, but not involving adjacent organs.
pT4: tumour of any size, involving adjacent organs.
Regional lymph nodes (N)
N0: no regional lymph node metastasis.
N1: regional lymph node metastasis
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Pathology - Phaeochromocytoma
A neoplasm originating from the chromaffin cells of the adrenal medulla.
Epidemiology: Rare, having a yearly incidence of 8 per million. Aetiology • The majority are irregular. Approximately 10% are linked to hereditary illnesses, including MEN 2, neurofibromatosis type 1, and von Hippel–Lindau disease. Hereditary tumors are more prone to manifest at a younger age and exhibit bilateral occurrence. Genetics • Sporadic tumors exhibit a significant prevalence of 1p loss of heterozygosity.
Presentation • Patients experience sudden episodes of pulsating headaches, diaphoresis, palpitations, thoracic pain, and abdominal discomfort resulting from elevated circulating catecholamines produced by the tumor. • Hypertension is frequently observed
Elevated urine catecholamines and their metabolites serve as a significant diagnostic tool.
Macroscopy • A well-defined tumor exhibiting a firm grey cut surface. • Average dimensions of 6 cm and a mass of 200 g.
Histopathology • Tumor cells exhibit distinctive clusters ('zellballen') interspersed with a fine vascular network. • The cells are polygonal, possessing granular basophilic cytoplasm. • Nuclei display a peculiar stippled chromatin pattern. • Scattered pleomorphic nuclei may be observed.
Prognosis • 90% exhibit a benign behavior. • Regrettably, histological analysis is not dependable in forecasting the 10% that may be malignant; however, concerning indicators include invasive development, necrosis, and elevated mitotic activity.
A neoplasm originating from the chromaffin cells of the adrenal medulla.
Epidemiology: Rare, having a yearly incidence of 8 per million. Aetiology • The majority are irregular. Approximately 10% are linked to hereditary illnesses, including MEN 2, neurofibromatosis type 1, and von Hippel–Lindau disease. Hereditary tumors are more prone to manifest at a younger age and exhibit bilateral occurrence. Genetics • Sporadic tumors exhibit a significant prevalence of 1p loss of heterozygosity.
Presentation • Patients experience sudden episodes of pulsating headaches, diaphoresis, palpitations, thoracic pain, and abdominal discomfort resulting from elevated circulating catecholamines produced by the tumor. • Hypertension is frequently observed
Elevated urine catecholamines and their metabolites serve as a significant diagnostic tool.
Macroscopy • A well-defined tumor exhibiting a firm grey cut surface. • Average dimensions of 6 cm and a mass of 200 g.
Histopathology • Tumor cells exhibit distinctive clusters ('zellballen') interspersed with a fine vascular network. • The cells are polygonal, possessing granular basophilic cytoplasm. • Nuclei display a peculiar stippled chromatin pattern. • Scattered pleomorphic nuclei may be observed.
Prognosis • 90% exhibit a benign behavior. • Regrettably, histological analysis is not dependable in forecasting the 10% that may be malignant; however, concerning indicators include invasive development, necrosis, and elevated mitotic activity.
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Pathology - Addison's disease
Definition: Primary adrenocortical insufficiency. Epidemiology • Uncommon, with an estimated annual incidence of 1 in 100,000 individuals. • The majority of instances occur in young to middle-aged adults. • Women are disproportionately impacted compared to men.
Etiology • Autoimmune degradation in industrialized nations. • Disseminated TB in underdeveloped nations. • Alternative etiologies, such as adrenal metastases, are infrequent.
Pathogenesis • Addison's disease results in a significant deficiency of glucocorticoid and mineralocorticoid synthesis by the adrenal cortex.
Clinical manifestations do not become apparent until approximately 90% of the gland has been obliterated.
Presentation: Fatigue, lethargy, and debilitation. • Anorexia, nausea, emesis, and diarrhea. • Weight loss may be significant.
The clinical appearance is frequently subtle and non-specific, complicating the diagnosis.
Biochemistry • Sodium and potassium levels. • Urea concentration resulting from dehydration. • As much as fifty percent of patients have hypoglycemia. • Circulating anti-adrenal autoantibodies are frequently observed. Diagnosis • Individuals suspected of having Addison’s disease should undergo dynamic adrenal cortex testing via a Synacthen test. This entails an intramuscular injection of synthetic adrenocorticotropic hormone (ACTH). The typical response is an elevation in plasma cortisol levels. In Addison's disease, there is either an absence of cortisol elevation or merely a negligible increase.
Prognosis: Favorable if diagnosis is established and lifetime replacement medication is initiated with synthetic glucocorticoid (hydrocortisone) and mineralocorticoid (fludrocortisone). It is essential for patients to comprehend the necessity of augmenting their hydrocortisone dosage throughout any concurrent illness. 3 Untreated or inadequately treated Addison's disease may lead to abrupt adrenal failure ('Addisonian crisis'), characterized by a perilous combination of hypovolemic shock, significant hypoglycemia, and hyponatremia
Definition: Primary adrenocortical insufficiency. Epidemiology • Uncommon, with an estimated annual incidence of 1 in 100,000 individuals. • The majority of instances occur in young to middle-aged adults. • Women are disproportionately impacted compared to men.
Etiology • Autoimmune degradation in industrialized nations. • Disseminated TB in underdeveloped nations. • Alternative etiologies, such as adrenal metastases, are infrequent.
Pathogenesis • Addison's disease results in a significant deficiency of glucocorticoid and mineralocorticoid synthesis by the adrenal cortex.
Clinical manifestations do not become apparent until approximately 90% of the gland has been obliterated.
Presentation: Fatigue, lethargy, and debilitation. • Anorexia, nausea, emesis, and diarrhea. • Weight loss may be significant.
The clinical appearance is frequently subtle and non-specific, complicating the diagnosis.
Biochemistry • Sodium and potassium levels. • Urea concentration resulting from dehydration. • As much as fifty percent of patients have hypoglycemia. • Circulating anti-adrenal autoantibodies are frequently observed. Diagnosis • Individuals suspected of having Addison’s disease should undergo dynamic adrenal cortex testing via a Synacthen test. This entails an intramuscular injection of synthetic adrenocorticotropic hormone (ACTH). The typical response is an elevation in plasma cortisol levels. In Addison's disease, there is either an absence of cortisol elevation or merely a negligible increase.
Prognosis: Favorable if diagnosis is established and lifetime replacement medication is initiated with synthetic glucocorticoid (hydrocortisone) and mineralocorticoid (fludrocortisone). It is essential for patients to comprehend the necessity of augmenting their hydrocortisone dosage throughout any concurrent illness. 3 Untreated or inadequately treated Addison's disease may lead to abrupt adrenal failure ('Addisonian crisis'), characterized by a perilous combination of hypovolemic shock, significant hypoglycemia, and hyponatremia
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Pathology - Parathyroid hyperplasia
Definition: An augmentation of parathyroid cell mass without an identifiable trigger.
Epidemiology • Rare, constituting around 20% of primary hyperparathyroidism. • Women are impacted more significantly than men, at a ratio of 3:1.
Aetiology • The majority of patients exhibit sporadic hyperplasia without a discernible etiology. • Approximately 20% of cases are attributed to family illness, predominantly Multiple Endocrine Neoplasia (MEN). 1.
Pathogenesis • Parathyroid hyperplasia results in excessive secretion of parathyroid hormone (PTH). Elevated PTH levels induce hypercalcemia via enhancing calcium absorption from the gastrointestinal tract and kidneys, as well as by augmenting osteoclastic activity in bone.
Presentation • individuals exhibit primary hyperparathyroidism, a biochemical illness characterized by hypercalcemia and an abnormally normal or elevated PTH level. • Numerous individuals are asymptomatic when this condition is identified accidentally. • Some individuals may exhibit nonspecific symptoms such as tiredness, nausea, constipation, polyuria, and arthralgia.
Macroscopy • All parathyroid glands exhibit hypertrophy, with weights exceeding 60 mg and dimensions surpassing 6 mm, but with variability among the glands.
Histopathology • The principal characteristic is an augmentation of cellular mass within the gland, correlated with a reduction in adipose content. • Typically, there is an elevation in both primary and oncocytic cell types. Secondary fibrosis and hemorrhage are prevalent observations.
Prognosis: Excellent after subtotal parathyroidectomy.
Definition: An augmentation of parathyroid cell mass without an identifiable trigger.
Epidemiology • Rare, constituting around 20% of primary hyperparathyroidism. • Women are impacted more significantly than men, at a ratio of 3:1.
Aetiology • The majority of patients exhibit sporadic hyperplasia without a discernible etiology. • Approximately 20% of cases are attributed to family illness, predominantly Multiple Endocrine Neoplasia (MEN). 1.
Pathogenesis • Parathyroid hyperplasia results in excessive secretion of parathyroid hormone (PTH). Elevated PTH levels induce hypercalcemia via enhancing calcium absorption from the gastrointestinal tract and kidneys, as well as by augmenting osteoclastic activity in bone.
Presentation • individuals exhibit primary hyperparathyroidism, a biochemical illness characterized by hypercalcemia and an abnormally normal or elevated PTH level. • Numerous individuals are asymptomatic when this condition is identified accidentally. • Some individuals may exhibit nonspecific symptoms such as tiredness, nausea, constipation, polyuria, and arthralgia.
Macroscopy • All parathyroid glands exhibit hypertrophy, with weights exceeding 60 mg and dimensions surpassing 6 mm, but with variability among the glands.
Histopathology • The principal characteristic is an augmentation of cellular mass within the gland, correlated with a reduction in adipose content. • Typically, there is an elevation in both primary and oncocytic cell types. Secondary fibrosis and hemorrhage are prevalent observations.
Prognosis: Excellent after subtotal parathyroidectomy.
