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Diagnosis and Treatment > Medication

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Natasha Shrikrishnapalasuriyar Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Mirena Noyvirt Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Philip Evans Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Bethan Gibson Department of Intensive Care, Royal Glamorgan Hospital, Llantrisant, UK

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Elin Foden Department of Intensive Care, Royal Glamorgan Hospital, Llantrisant, UK

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Atul Kalhan Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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A 54-year-old woman was admitted to hospital with a presumed allergic reaction to a single dose of amoxicillin given for a suspected upper respiratory tract infection. She complained of chest tightness although there was no wheeze or stridor. On examination, she was pyrexial, tachycardic, hypertensive and had a diffuse mottled rash on her lower limbs. Her initial investigations showed raised inflammatory markers. She was treated in the intensive care for a presumed anaphylactic reaction with an underlying sepsis. Further investigations including CT head and CSF examination were unremarkable; however, a CT abdomen showed a 10 cm heterogeneous right adrenal mass. Based on review by the endocrine team, a diagnosis of pheochromocytoma crisis was made, which was subsequently confirmed on 24-h urinary metanephrine measurement. An emergency adrenalectomy was considered although she was deemed unfit for surgery. Despite intensive medical management, her conditioned deteriorated and she died secondary to multi-organ failure induced by pheochromocytoma crisis.

Learning points:

  • Pheochromocytoma have relatively higher prevalence in autopsy series (0.05–1%) suggestive of a diagnosis, which is often missed.

  • Pheochromocytoma crisis is an endocrine emergency characterized by hemodynamic instability induced by surge of catecholamines often precipitated by trauma and medications (β blockers, general anesthetic agents, ephedrine and steroids).

  • Pheochromocytoma crisis can mimic acute coronary syndrome, cardiogenic or septic shock.

  • Livedo reticularis can be a rare although significant cutaneous marker of underlying pheochromocytoma crisis.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Hormone, Adrenaline, Dopamine, Metanephrines, Noradrenaline, Normetanephrine, Condition/ Syndrome, Phaeochromocytoma, Phaeochromocytoma crisis, Patient Demographics, Age, Adolescent/young adult, Gender, Female, Ethnicity, White, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Agitation, Altered level of consciousness, Atrial fibrillation, Breathing difficulties, Hypertension, Hypotension, Poikiloderma, Pyrexia, Rash, Sepsis, Tachycardia, Investigation, Adrenaline, Alanine aminotransferase, Bilirubin, Blood pressure, Catecholamines (24-hour urine), C-reactive protein, Creatinine, CT scan, Dopamine, Echocardiogram, Kidney function, Liver function, Metanephrines (urinary), Noradrenaline, Normetanephrine, Urea and electrolytes, White blood cell count, Intervention, Fluid repletion, Medication, Alpha-blockers, Amoxicillin, Antibiotics, Epinephrine, Glucocorticoids, Hydrocortisone, Norepinephrine, Phenoxybenzamine, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-17-0170
Volume/Issue:
Volume 2018: Issue 1
Open access
A León-Suárez Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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P Roldán-Sarmiento Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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M A Gómez-Sámano Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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A Nava-De la Vega Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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V M Enríquez-Estrada Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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F J Gómez-Pérez Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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D Cuevas-Ramos Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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Summary

Non-Hodgkin lymphoma (NHL) is a hematological tumor caused by abnormal lymphoid proliferation. NHL can arise in any part of the body, including central nervous system (CNS). However, pituitary involvement is a quite rare presentation. The diffuse large B-cell lymphoma (DLBCL) is the most common subtype when pituitary is infiltrated. Here, we report a case of pituitary infiltration of NHL DLBCL type in a woman with hypopituitarism and an infundibulum-hypophysitis-like image on magnetic resonance imaging (MRI). A female aged 64 years, complained of dyspepsia, fatigue, weight loss and urine volume increment with thirst. Endoscopy and gastric biopsy confirmed diffuse large B-cell lymphoma. Treatment with chemotherapy using R-CHOP was initiated. During her hospitalization, hypotension and polyuria were confirmed. Hormonal evaluation was compatible with central diabetes insipidus and hypopituitarism. Simple T1 sequence of MRI showed thickening of the infundibular stalk with homogeneous enhancement. After lumbar puncture analysis, CNS infiltration was confirmed showing positive atypical lymphocytes. Pituitary and infundibular stalk size normalized after R-CHOP chemotherapy treatment. In conclusion, pituitary infiltration of NHL with infundibular-hypophysitis-like image on MRI is a rare finding. Clinical picture included hypopituitarism and central diabetes insipidus. Diagnosis should be suspected after biochemical analysis and MRI results. Treatment consists of chemotherapy against NHL and hormonal replacement for pituitary dysfunction.

Learning points:

  • Pituitary infiltration by lymphoma can present with signs and symptoms of panhypopituitarism and diabetes insipidus.

  • MRI findings can resemble an autoimmune hypophysitis.

  • Patients can recover pituitary function as well as normalization of MRI after chemotherapy treatment.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, Cortisol, FSH, LH, Oestradiol (E2), Prolactin, Thyroxine (T4), Condition/ Syndrome, Adrenal insufficiency, Diabetes insipidus - neurogenic/central, Hypopituitarism, Hypothyroidism, Non-Hodgkin lymphoma, Panhypopituitarism, Pituitary tumour (malignant), Patient Demographics, Age, Adult, Gender, Female, Ethnicity, Hispanic or Latino - Mexican, Mexican American, Chicano, Country of Treatment, Mexico, Diagnosis and Treatment, Signs and Symptoms, Dyspepsia, Fatigue, Hypoadrenalism, Hypogonadism, Hyponatraemia, Hypopituitarism, Hypotension, Hypothyroidism, Nausea, Polydipsia, Polyuria, Vomiting, Investigation, ACTH stimulation, Biopsy, Cortisol (serum), CT scan, Endoscopy, FSH, FT4, Gastric biopsy, Histopathology, LH, MRI, Oestradiol (E2), Prolactin, Sodium, Urine 24-hour volume, Urine osmolality, Intervention, Chemotherapy, Medication, Anthracyclines, Antibiotics, Desmopressin, Dexamethasone, Doxorubicin, Glucocorticoids, Hydrocortisone, Levothyroxine, Norepinephrine, Prednisolone, Rituximab, Related Disciplines, Oncology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-16-0103
Volume/Issue:
Volume 2016: Issue 1
Open access