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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 - 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 - Mantle cell lymphoma
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
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Pathology - Primary amyloidosis
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
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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 - 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 - Parathyroid carcinoma
Definition: A malignant epithelial neoplasm originating from the parathyroid gland.
Epidemiology: Rare, constituting around 1% of primary hyperparathyroidism cases. • The majority manifest in individuals in their 40s and 50s, exhibiting no gender preference.
Aetiology • The cause remains unidentified, however anecdotal evidence suggests a connection with secondary hyperparathyroidism and previous neck irradiation. • Parathyroid cancer has not been associated with MEN 1.
Carcinogenesis • The most commonly observed aberration is the loss of genetic material at chromosome 13q.
Presentation • In contrast to individuals with parathyroid hyperplasia or adenoma, patients typically exhibit symptomatic primary hyperparathyroidism accompanied by a discernible neck mass. • Calcium concentrations are typically elevated (3.5–4mmol/L), accompanied by symptoms such as polyuria, polydipsia, weakness, renal colic, and ostealgia. Macroscopy: Parathyroid carcinomas are typically significantly larger than adenomas, averaging a weight of 12g. • They may exhibit well-defined margins or possess distinctly infiltrative borders.
Histopathology: Parathyroid carcinomas consist of sheets of epithelial cells that frequently appear deceptively benign. The development of follicles is atypical. • Tumors frequently possess a robust capsule and are interspersed with dense fibrous tissue bands that partition the tumor into several expansive nodules. • Capsular invasion, vascular invasion, tumor necrosis, and an elevated mitotic index are all strongly indicative of malignancy.
Prognosis • The 10-year survival rate is around 50%. • The majority of patients succumb to the unmanageable metabolic consequences of severe hyperparathyroidism caused by recurrent tumors.
Definition: A malignant epithelial neoplasm originating from the parathyroid gland.
Epidemiology: Rare, constituting around 1% of primary hyperparathyroidism cases. • The majority manifest in individuals in their 40s and 50s, exhibiting no gender preference.
Aetiology • The cause remains unidentified, however anecdotal evidence suggests a connection with secondary hyperparathyroidism and previous neck irradiation. • Parathyroid cancer has not been associated with MEN 1.
Carcinogenesis • The most commonly observed aberration is the loss of genetic material at chromosome 13q.
Presentation • In contrast to individuals with parathyroid hyperplasia or adenoma, patients typically exhibit symptomatic primary hyperparathyroidism accompanied by a discernible neck mass. • Calcium concentrations are typically elevated (3.5–4mmol/L), accompanied by symptoms such as polyuria, polydipsia, weakness, renal colic, and ostealgia. Macroscopy: Parathyroid carcinomas are typically significantly larger than adenomas, averaging a weight of 12g. • They may exhibit well-defined margins or possess distinctly infiltrative borders.
Histopathology: Parathyroid carcinomas consist of sheets of epithelial cells that frequently appear deceptively benign. The development of follicles is atypical. • Tumors frequently possess a robust capsule and are interspersed with dense fibrous tissue bands that partition the tumor into several expansive nodules. • Capsular invasion, vascular invasion, tumor necrosis, and an elevated mitotic index are all strongly indicative of malignancy.
Prognosis • The 10-year survival rate is around 50%. • The majority of patients succumb to the unmanageable metabolic consequences of severe hyperparathyroidism caused by recurrent tumors.
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Pathology - Parathyroid adenoma
A nonmalignant epithelial tumor of the parathyroid gland.
Epidemiology: Common, constituting approximately 80% of primary hyperparathyroidism. • Peak incidence occurs between the ages of 50 and 60 years. • Women are impacted more significantly than men, at a ratio of 3:1
Aetiology • The underlying causes are inadequately comprehended, however previous irradiation of the neck seems to elevate the risk. The pathogenesis involves the autonomous secretion of parathyroid hormone (PTH) from the adenoma, resulting in hypercalcemia due to uncontrolled calcium mobilization from the bone and increased calcium absorption in the kidneys and gastrointestinal tract.
Presentation • Patients exhibit primary hyperparathyroidism, characterized by hypercalcemia accompanied by an abnormally normal or elevated PTH level. • A multitude of patients are asymptomatic when this is identified inadvertently. • Some individuals may exhibit nonspecific symptoms such as tiredness, nausea, constipation, polyuria, and arthralgia.
Macroscopy: A solitary parathyroid gland exhibits enlargement, measuring over 6mm in size and over 60mg in weight. The adenoma is often smooth, firm, soft, and light brown in hue. Histopathology: The parathyroid gland exhibits a well-defined, often encapsulated mass comprised of parathyroid epithelial cells devoid of adipose tissue. A compressed rim of normal parathyroid tissue frequently exists at one margin. Chief cells typically dominate, however an interspersing of oncocytic cells is also occasionally observed. The cells might be organized into solid sheets, trabeculae, or follicles. Stromal edema, fibrosis, and hemorrhage are frequently observed.
Prognosis: Parathyroid adenomas are benign tumors that can be effectively treated with surgical removal.
A nonmalignant epithelial tumor of the parathyroid gland.
Epidemiology: Common, constituting approximately 80% of primary hyperparathyroidism. • Peak incidence occurs between the ages of 50 and 60 years. • Women are impacted more significantly than men, at a ratio of 3:1
Aetiology • The underlying causes are inadequately comprehended, however previous irradiation of the neck seems to elevate the risk. The pathogenesis involves the autonomous secretion of parathyroid hormone (PTH) from the adenoma, resulting in hypercalcemia due to uncontrolled calcium mobilization from the bone and increased calcium absorption in the kidneys and gastrointestinal tract.
Presentation • Patients exhibit primary hyperparathyroidism, characterized by hypercalcemia accompanied by an abnormally normal or elevated PTH level. • A multitude of patients are asymptomatic when this is identified inadvertently. • Some individuals may exhibit nonspecific symptoms such as tiredness, nausea, constipation, polyuria, and arthralgia.
Macroscopy: A solitary parathyroid gland exhibits enlargement, measuring over 6mm in size and over 60mg in weight. The adenoma is often smooth, firm, soft, and light brown in hue. Histopathology: The parathyroid gland exhibits a well-defined, often encapsulated mass comprised of parathyroid epithelial cells devoid of adipose tissue. A compressed rim of normal parathyroid tissue frequently exists at one margin. Chief cells typically dominate, however an interspersing of oncocytic cells is also occasionally observed. The cells might be organized into solid sheets, trabeculae, or follicles. Stromal edema, fibrosis, and hemorrhage are frequently observed.
Prognosis: Parathyroid adenomas are benign tumors that can be effectively treated with surgical removal.
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Pathology - Neuroblastoma
Definition • A malignant pediatric tumor originating from neural crest-derived cells of the sympathetic nervous system. Most originate in the adrenal medulla or paraspinal sympathetic ganglia.
Epidemiology: The third most prevalent malignant neoplasm in pediatric populations. • Incidence of 1 in 10,000 live births annually. • Predominantly occur throughout the first four years of life.
Aetiology: Unknown. Genetics • Tumor genetics possess significant prognostic consequences. Amplification of MYCN, diploidy, and deletions on chromosome 1p are all correlated with a worse prognosis. Presentation • The majority of children exhibit symptoms of illness characterized by weight loss, fever, watery diarrhea, and a discernible abdominal mass. Biochemistry • Elevated urinary levels of catecholamines and their metabolites, vanillylmandelic acid (VMA) and homovanilic acid (HMA), serve as a significant diagnostic tool. Macroscopy • A lobulated, soft, gray tumor mass of approximately 6–8 cm, closely associated with the adrenal gland or sympathetic chain.
Histopathology • Neuroblastoma is classified into four categories based on the degree of development of primitive neuroblasts into ganglion cells. • Undifferentiated neuroblastoma consists of undifferentiated neuroblasts lacking any signs of ganglionic differentiation. They resemble several other 'small round blue cell tumors' of childhood, necessitating other procedures to confirm the diagnosis (e.g., antibody for brain markers such as CD56 and synaptophysin). Poorly differentiated neuroblastoma exhibits minimal ganglionic differentiation (<5% of cells) and comprises neurofibrillary stroma. • differentiated neuroblastoma numerous ganglionic cells (> 5%, but
Definition • A malignant pediatric tumor originating from neural crest-derived cells of the sympathetic nervous system. Most originate in the adrenal medulla or paraspinal sympathetic ganglia.
Epidemiology: The third most prevalent malignant neoplasm in pediatric populations. • Incidence of 1 in 10,000 live births annually. • Predominantly occur throughout the first four years of life.
Aetiology: Unknown. Genetics • Tumor genetics possess significant prognostic consequences. Amplification of MYCN, diploidy, and deletions on chromosome 1p are all correlated with a worse prognosis. Presentation • The majority of children exhibit symptoms of illness characterized by weight loss, fever, watery diarrhea, and a discernible abdominal mass. Biochemistry • Elevated urinary levels of catecholamines and their metabolites, vanillylmandelic acid (VMA) and homovanilic acid (HMA), serve as a significant diagnostic tool. Macroscopy • A lobulated, soft, gray tumor mass of approximately 6–8 cm, closely associated with the adrenal gland or sympathetic chain.
Histopathology • Neuroblastoma is classified into four categories based on the degree of development of primitive neuroblasts into ganglion cells. • Undifferentiated neuroblastoma consists of undifferentiated neuroblasts lacking any signs of ganglionic differentiation. They resemble several other 'small round blue cell tumors' of childhood, necessitating other procedures to confirm the diagnosis (e.g., antibody for brain markers such as CD56 and synaptophysin). Poorly differentiated neuroblastoma exhibits minimal ganglionic differentiation (<5% of cells) and comprises neurofibrillary stroma. • differentiated neuroblastoma numerous ganglionic cells (> 5%, but
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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
