2024: Vol 11 No 7

2024: Vol 11 No 7
  • Salman Khan, Ekrem Yetiskul, Georges Khattar, Araji Ghada, Sarah Afif, Malik Waleed Zeb Khan, Umesh Manchandani
    Views: 212
    PDF: 105 HTML: 15

    Introduction: Renal squamous cell carcinoma (SCC) is a neoplasm with an extremely rare occurrence compared to other renal malignancies. The classic presentation includes a palpable mass and flank pain; however, the presentation is seldom non-specific. Our study describes the significance of programmed death ligand-1 (PD-L1) expression in renal cancer and its association with clinical outcomes, alongside available treatment options.
    Case description: An 80-year-old female with a history of hypertension and cerebral aneurysm presented with right flank pain and blood in urine and was diagnosed with pyelonephritis and left renal mass/phlegmon. A biopsy revealed SCC of the kidney with metastasis to the lung and aortocaval lymph node. Positron emission tomography (PET) scan confirmed malignancy in the kidney and lung. Treatment with pemrolizumab and carboplatin plus paclitaxel was initiated but poorly tolerated as the haemoglobin dropped rapidly.
    Conclusion: SCC poses a diagnostic challenge due to its rarity and non-specific symptoms, often leading to advanced stage diagnosis. PD-L1 expression is pivotal in assessing tumour aggressiveness and prognosis. PD-L1 inhibitors offer promise, but their efficacy in renal SCC warrants further investigation. Radical nephrectomy and systemic chemotherapy show potential in advanced cases, necessitating vigilant management of treatment-related side effects. This case emphasises the need for ongoing research to refine therapeutic approaches and enhance outcomes in renal SCC patients.

  • Noel Lorenzo-Villalba, Charlene Habib, Léa Docquier, Edward Nasco, Alessio Imperiale, Emmanuel Andrès, Jean Edouard Terrade
    Views: 111
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    A 50-year-old patient with a history of limited cutaneous scleroderma began with polyarthralgia (left shoulder, elbows and hips) without stiffness or associated inflammatory syndrome. Treatment with oral anti-inflammatory drugs was started on suspicion of peripheral spondyloarthritis with partial response. This progressed with the appearance of stiffness and functional limitation of the hips as well as an increase in the inflammatory syndrome two weeks after onset. It was decided to perform an 18F-FDG-PET scan compatible with polymyalgia rheumatica. The patient was treated with oral corticosteroids with an excellent response after one week of treatment.

  • Fatih Kaya, Tarek Alsafdi
    Views: 138
    PDF: 85 HTML: 10

    Medullary thyroid carcinoma (MTC) is a rare and challenging type of thyroid cancer originating from parafollicular cells (C cells) that produce calcitonin. Diagnosing and monitoring this carcinoma can be complex due to its unique biomarkers. Procalcitonin (PCT), a precursor of calcitonin, and carcinoembryonic antigen (CEA) are important markers for MTC. Elevated PCT levels, particularly when they remain high post-infection treatment, and elevated CEA levels are significant indicators for suspecting MTC. This report emphasises the diagnostic and prognostic importance of these biomarkers in MTC, highlighting their roles in detecting and monitoring disease progression. Integrating PCT and CEA measurements into routine clinical practice can enhance detection, provide understanding of therapeutic responses and aid in the effective management of MTC.

  • Murad Aldarayseh, Sohail Singh Sodhi, Gamal Musleh, Dyuti Kumar, Michelle Cholankeril
    Views: 163
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    A 53-year-old post-menopausal Indian female presented with invasive ductal carcinoma, treated with neoadjuvant chemotherapy and pembrolizumab due to a PD-L1 combined positive score of 5. Following a right mastectomy and axillary dissection, she received a breast expander and AlloDerm™ graft. After resuming pembrolizumab and paclitaxel postoperatively, she developed severe breast redness and high-grade fever, necessitating expander removal due to suspected pembrolizumab-induced complications. This case underscores the unique and severe adverse effects of pembrolizumab on breast reconstruction, highlighting the need for careful monitoring and management in patients undergoing similar treatments.

  • Shahad Alchalabi, Abdulrahman S. Museedi
    Views: 117
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    Introduction: In-situ right atrial (RA) thrombus is a rare occurrence typically associated with heightened inflammatory or hypercoagulable states. Here, we present a case of in-situ RA thrombus mimicking atrial myxoma in a patient with sepsis and bacteraemia.
    Case description: A 41-year-old man presented with septic arthritis and bacteraemia caused by methicillin-resistant Staphylococcus aureus (MRSA). A transoesophageal echocardiogram revealed a large pediculated mass resembling atrial myxoma, which was not visible on transthoracic echocardiography performed four days earlier. Cardiac magnetic resonance (CMR) imaging strongly suggested a thrombus, leading to the patient undergoing transcatheter aspiration. Subsequent pathology confirmed an organised fibrin thrombus without evidence of infection.
    Discussion: The patient’s in-situ RA thrombus likely developed in response to a heightened inflammatory state associated with sepsis. Limited data exist on in-situ RA thrombi in the absence of atrial fibrillation, though some reports suggest a correlation between heightened inflammation and a hypercoagulable state.
    Conclusion: CMR imaging is invaluable for characterising such masses and can aid in distinguishing a thrombus from a myxoma.

  • Yasaman Navari, Justine Chinnappan, Thair Dawood, Huda Marcus
    Views: 114
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    The maze procedure for atrial fibrillation carries risks, including pleural effusion. We report a case of a 54-year-old woman with right-sided pleural effusion post maze surgery, presenting with dyspnoea. Despite treatment, complications arose, including atrial flutter. Prompt recognition and multidisciplinary management led to a favourable outcome. This case underscores the importance of vigilance for rare post-operative complications and highlights the need for collaborative care in optimising patient outcomes following cardiac surgeries. Further research is warranted to refine management strategies for such occurrences.

  • Yasaman Navari, Efosa Bazuaye, Philip J. McDonald
    Views: 100
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    Calcium plays a crucial role in the heart’s electrical conduction system and facilitating the contraction of cardiac muscles. Hypocalcemia can result in electrocardiogram findings such as a prolonged QTC interval and eventually torsade de pointes, which in severe cases can progress to cardiac arrest. In cases of B-cell lymphoma, hypocalcemia may arise from various factors. Tumor infiltration can disrupt calcium homeostasis by affecting the parathyroid glands or bone tissue. Acidosis in the context of B-cell lymphoma can cause significant cardiovascular adverse effects. It will reduce peripheral vascular resistance and cardiac muscle contractility, promote dysrhythmias, and disturb oxygen uptake in the lungs. These combined effects markedly compromise cardiac function, increasing the likelihood of cardiac arrest. These mechanisms necessitate comprehensive management strategies in B-cell lymphoma patients. In this case report we present a case of cardiac arrest in a 59-year-old female woman with hypocalcemia and lactic acidosis secondary to B-cell lymphoma.

  • Mohamad El Labban, Stevan Oluic, Hussein Guleid, Mohamed Hassan, Rawan Diab, Mohamad Ali Rida
    Views: 226
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    Introduction: Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder that causes stiffness and pain in the proximal joints, including the shoulders, hips and neck. The exact cause of polymyalgia rheumatica is yet to be fully understood, but research suggests that both genetic and environmental factors may contribute to it. Studies have previously linked the onset and relapse of polymyalgia rheumatica symptoms to the influenza and COVID-19 vaccines. The Food and Drug Administration approved the respiratory syncytial virus (RSV) vaccine, which is a recombinant protein vaccine for adults over 60, in May 2023. No previous reports of polymyalgia rheumatica onset or relapse have been linked to the RSV vaccine. The human proteome shares some peptides with the RSV F antigen, suggesting a high risk of cross-reactivity when using that antigen in vaccination formulations.
    Case description: A 72-year-old man experienced a new onset of bilateral shoulder pain and stiffness three days after receiving the Abrysvo® RSV vaccine. The symptoms lasted more than an hour (up until noon) and interfered with his activities of daily living. Inflammatory markers such as C-reactive protein were elevated. The patient’s symptoms and inflammatory marker levels significantly improved with prednisone therapy.
    Conclusion: In patients with typical PMR symptoms, it is important for clinicians to carefully review immunisation history to rule out any potentially related adverse effects.

  • Abdelhadi Farouji, Mohammad N. Kloub, Ahmad W. Haddad, Amaar S. Ahmad, Priscilla Chow, Richard Miller
    Views: 95
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    Background: Chylothorax can be classified into traumatic and nontraumatic based on the etiology. Nephrotic syndrome is a very rare cause of nontraumatic chylothorax in adults.
    Case presentation: A 66-year-old woman with membranous nephropathy who was non-compliant with her management, presented with dyspnea, and was found to have a large right sided chylothorax. Her chylothorax was secondary to membranous nephropathy after excluding other causes, which has been rarely reported in literature.
    Conclusion: This case highlights the possibility of nephrotic syndrome causing chylothorax, especially in patients with undiagnosed nephrotic syndrome or patients non-compliant with their management. When evaluating a patient with chylothorax, providers should consider nephrotic syndrome in the differential diagnosis.

  • Daniyal Raza, Natalie Bzowej, James Rini, Rachel Rhinehart, Hrishikesh Samant
    Views: 119
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    This case report details the complex diagnostic odyssey of a 60-year-old female grappling with chronic liver disease, initially diagnosed with hepatic encephalopathy (HE). Despite initial treatment with lactulose and rifaximin, her neurological symptoms worsened, leading to the identification of concurrent acquired hepatocerebral degeneration (AHD). This condition is characterised by cognitive decline, movement disorders and distinctive imaging abnormalities. The discussion highlights the challenges in distinguishing AHD from HE, underscoring the sophisticated diagnostic and management strategies required for such intricate cases in the realm of chronic liver disease.

  • Ahlame Benhamdane, Tarik Adioui, Sanaa Berrag, Fouad Nejjari, Mohamed Allaoui, Mouna Tamzaourte
    Views: 101
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    Introduction: Primary squamous cell carcinoma of the liver (SCC) is a rare and challenging pathology. As an aggressive cancer, the prognosis is extremely poor with less than 12 months overall survival. In view of its low prevalence, we report the case of an elderly patient with primary squamous cell carcinoma of the liver.
    Case description: A 74-year-old female, with no pathological history, presented with acute right hypochondrium pain associated with shivering, asthenia and weight loss. We diagnosed primary hepatic squamous cell carcinoma by pathological analysis.
    Conclusion: Primary hepatic squamous cell carcinoma represents a rare malignant tumour with extremely poor prognosis. There is no established treatment protocol for this disease and a multidisciplinary approach is needed to choose the best therapeutic option.

  • Sierra Sullivan, Jesus Davalos, Jacob Nichols, Flint Smith, Tanis Welch, Alanna Woodward, Alyssa McLean
    Views: 127
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    Brevibacillus laterosporus, an endospore-forming, aerobic, Gram-positive bacillus, is not only a potential biocontrol agent against plant pathogens but also a rare cause of human infection. Its pathogenicity in humans, especially in immunocompetent individuals, is still not fully understood. Skin infections caused by B. laterosporus are typically opportunistic, entering the skin through cuts, wounds, or other breaches in the skin’s protective barrier, as in the case presented here.

  • Amal El-Ouakhoumi, Hajar Joulal, Jaouad Yousfi, Laila Benjilali, Mouna Zahlane, Lamiaa Essaadouni
    Views: 142
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    Background: The psychiatric manifestations of Sjögren’s syndrome are often overlooked despite their prevalence. They can be revelatory of the disease and include anxiety, depression, dementia and, rarely, psychosis.
    Case description: We report a case of 18-year-old female in whom a major depressive syndrome revealed primary Sjögren’s disease, with a favourable outcome after treatment with rituximab.
    Conclusion: The diagnostic of Sjögren’s syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms.

  • Marta Castelli, Mauro Betelli, Anna Valenti, Barbara Merelli, Alessandro Loglio, Mauro Viganò, Alberto Benetti
    Views: 172
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    We describe a rare case of polyserositis with chylous ascites following nivolumab therapy, highlighting the challenges in recognizing and managing immune-related adverse events (irAEs) associated with immune checkpoint inhibitors (ICPIs).

  • Nikhil Sood, Aniket Vadnerkar, Murali Kodali, Lyn K. Hamacher
    Views: 245
    HTML: 18 PDF: 109

    Pneumocystis jirovecii is an opportunistic fungus that infects the lungs but can involve other organs, including the skin and lymph nodes. Risk factors include human immunodeficiency virus (HIV), solid organ/haematological malignancies and a CD4 cell count of fewer than 200 cells/µl. Pneumocystis jirovecii pneumonia (PJP) infection is reported less frequently these days with the advent of prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX).
    We report a case of extrapulmonary PJP infection in a patient while receiving pentamidine prophylaxis in a T-cell prolymphocytic leukaemia, who underwent an allogeneic stem cell transplant. There are plenty of reported cases of PJP on pentamidine prophylaxis; however, none had cutaneous PJP infection. Cutaneous P. jirovecii infection (CPJ) is an extrapulmonary infection that is rarely reported. Our patient’s skin biopsy was inconclusive, but the skin nodules improved once he was initiated on TMP-SMX. Many transplant patients cannot tolerate TMP-SMX for various reasons and are placed on second-line prophylaxis for PJP, which does not prevent extrapulmonary PJP infections. Our case highlights the challenges of diagnosing such a rare infection in immunocompromised patients. Extrapulmonary PJP should be suspected in patients with a history of pulmonary PJP and persistent elevated Fungitell® levels in low CD4 counts.

  • Benedetta Marigliano, Luigi Scuro
    Views: 265
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    Hyponatremia is a common complication in patients undergoing neurosurgery. If undiagnosed, it has a negative prognostic impact. The two dominant causes of refractory hyponatremia include syndrome of inappropriate ADH secretion (SIADH) and cerebral salt wasting syndrome (CSWS). Discrimination between the two types of disease is not always obvious. We present a case of undiagnosed chronic hyponatremia caused by CSWS after neurosurgery, which not only resulted in a longer hospital stay but also slowed the patient’s postoperative recovery. Meticulous clinical evaluation and the performance of appropriate laboratory tests are therefore essential not only for decisive treatment, but also for the establishment of comprehensive diagnostic algorithms that allow timely diagnosis and decisive therapy.

  • Maria Elizabeth Karavassilis, Dmitriy Chernov, Sinda Dakhlia, Paramalingam Saravanan
    Views: 214
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    Background: We describe a case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in a 16-year-old patient who initially presented with clinical features of septic meningoencephalitis. This case outlines the importance of considering a diagnosis of MOGAD in patients who fail to improve with appropriate antimicrobial therapy or show a positive clinical response to glucocorticoids (often used in treatment of meningococcal meningitis). We emphasise the importance of recognising that an infectious prodrome can precede MOGAD.
    Case description: A 16-year-old male was admitted with vomiting, fever, headache, photophobia and altered mental state. He was treated for meningoencephalitis with initial clinical improvement. Lumbar puncture findings were suggestive of viral meningoencephalitis. During admission the patient went through several periods of transient clinical and biochemical improvement, alternating with periods of symptomatic relapse. On day 17 of admission, he was transferred to a tertiary centre for suspected autoimmune disseminated meningoencephalitis (ADEM) and two days later, he suffered a catastrophic neurological decline with new dysarthria, dysphagia, aphasia, horizontal nystagmus and facial paralysis. He made a remarkable neurological recovery after commencing treatment with IV immunoglobulin, IV methylprednisolone and plasma exchange, with complete resolution of symptoms.
    Conclusion: MOGAD can run a variable course and present soon after a central nervous system infection, making the diagnosis more challenging. Nonetheless, patients can achieve a full neurological recovery with early recognition, diagnosis and treatment of this rare entity.

  • Daniel Arturo Martínez-Piña, Ana Laura Calderón-Garcidueñas, Elizabeth Gama-Lizárraga, Kevin Giuseppe Enríquez-Peregrino, José María Curiel-Zamudio
    Views: 222
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    Background: Hypertrophic pachymeningitis (HP) is a disease with diverse aetiologies, including the autoimmune one, either associated with antineutrophil cytoplasmic antibodies or immunoglobulin G4.
    Case description: A 65-year-old woman with a history of systemic arterial hypertension, presented with intense progressive headaches. HP and hemispheric vasogenic oedema were observed by nuclear magnetic resonance (NMR) study. During the six months before the headache, she had developed progressive hearing loss which she attributed to age. A biopsy of dura mater showed necrotising vasculitis with peripheral inflammatory infiltrate, made up of accumulations of epithelioid cells and multinucleated giant cells, and abundant eosinophils. A final diagnosis of HP with eosinophilic granulomatosis with polyangiitis (EGPA) was made.
    Discussion: The patient had eosinophilic granulomatosis with polyangiitis (EGPA) histology, ANCA-negative serology and HP. This case is important because it shows that EGPA seems to have a spectrum of clinical diseases, including HP with negative serology, and bilateral sensorineural hearing loss.
    Conclusion: We are facing a wide spectrum of EGPA, breaking the paradigm of only systemic involvement.

  • Zay Yar Aung, Khaled Elmagraby, Alexandra Nica
    Views: 305
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    Neutropenia by non-chemotherapy drugs is an extremely rare idiosyncratic life-threatening drug reaction. Ceftriaxone and meropenem are widely used broad-spectrum antibiotics and are generally safe and well tolerated. The authors present a case of neutropenia induced by ceftriaxone and meropenem in an adult patient. The resolution of neutropenia occurred within 48 hours of ceftriaxone and meropenem being discontinued. Although antibiotic-induced neutropenia is uncommon, clinicians should be mindful of this adverse drug effect because of its potential development of severe neutropenia, septicaemia, septic shock, deep-seated infections and even death. Therefore, neutropenic sepsis treatment should be initiated without delay, particularly if the patient becomes septic and febrile. Granulocyte-colony stimulation factor (G-CSF) may be administered to facilitate the recovery process with daily monitoring of neutrophil count. Mortalities from antibiotic-induced neutropenia remain rare, with a range of 2.5–5%.

  • Toshiaki Takahashi, Kensuke Takaoka, Kelsey Kwong, Sharina Macapagal, Manasawee Tanariyakul, Chalothorn Wannaphut, Yoshito Nishimura
    Views: 204
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    Evans syndrome (ES) is a rare autoimmune disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia and autoimmune neutropenia. The precise pathogenesis of ES remains unclear, but it is believed to involve immune-mediated destruction of erythrocytes and platelets. Thrombotic complications, such as stroke, are critical yet largely unrecognised in ES. Here, we present a case of an 80-year-old male with ES who developed multiple strokes, emphasising the complex management challenges associated with this condition. The patient, known for stage IIB lung adenocarcinoma, presented with right-sided weakness and was diagnosed with a stroke of undetermined aetiology. He was started on warfarin for secondary prevention alongside intravenous immunoglobulin (IVIG) and corticosteroids for ES. Stroke in ES is rarely reported, and the optimal management remains inconclusive due to its rarity. The patient’s management was guided by existing guidelines for stroke prevention and anticoagulation in the setting of antiphospholipid syndrome. While anticoagulants are recommended for venous thromboembolism prophylaxis in AIHA, there are no clear guidelines for stroke prevention in ES. This case underscores the necessity of individualised treatment approaches and highlights the gaps in evidence regarding stroke management in ES. Future research is essential to determine the optimal management of stroke in this complex clinical scenario.

  • Lamprini Bounou, Aimilios Kaklamanos, Theodoros Androutsakos, Elissavet Kemanetzoglou, Ioanna Moustaka , Athanasios Protogerou, Athina Euthimiou
    Views: 199
    PDF: 89 HTML: 16

    Background: Anti-leucine-rich glioma inactivated 1 limbic encephalitis (anti-LGI1 LE) is one of the most frequent autoimmune encephalitis, commonly coexisting with other autoimmune diseases. Rheumatoid arthritis (RA) and monoclonal gammopathy of unknown significance (MGUS) are commonly associated with autoimmune phenomena. However, neither RA nor MGUS have been described in the literature to date as coexisting with anti-LGI1 LE.
    Case description: We present the case of anti-LGI1 LE in a male patient with rheumatoid arthritis, who was also found to have an MGUS. The patient was initially treated with corticosteroids and IV immunoglobulin. After a mild relapse, his treatment was complemented with rituximab, resulting in complete regression of the disease symptoms.
    Conclusions: Our report provides evidence for the coexistence of anti-LGI1 LE with RA and/or MGUS, thus extending the differential diagnosis of patients suffering with these disease entities that present with neuropsychiatric symptoms suggestive of encephalitis. Moreover, this case raises challenges on the management of the coexistence of these diseases, given the lack of therapeutic guidelines and their potential interaction on a pathophysiological and a clinical level.

  • Nikhil Sood, Margarita Kushnir, Bindu Jayavelu
    Views: 212
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    Romiplostim and eltrombopag are synthetic agonists of the thrombopoietin receptor (TPO-R), commonly used for immune thrombocytopenic purpura (ITP) and sometimes in myelodysplastic syndrome (MDS). They are rarely associated with kidney injury. We report a case of acute kidney injury caused by romiplostim and eltrombopag in an 80-year-old male patient with MDS and ITP. He did not have systemic haemolysis syndrome but isolated acute renal thrombotic microangiopathy confirmed by kidney biopsy. He was treated with steroids, plasmapheresis and anticoagulation, with improvement in renal function. Interestingly, the patient had high antiphospholipid (aPL) antibodies noted upon screening, indicating a possible new antiphospholipid syndrome (APS) diagnosis. In the presence of circulating aPL antibodies, eltrombopag may have served as a trigger, causing endothelial injury and subsequent renal microangiopathy; aPL antibodies were still significantly positive at four weeks of outpatient testing. This case and a few others reported in the literature highlight the importance of screening for aPL antibodies before initiating TPO-R agonists in patients with ITP. We suspect that using TPO-R agonists, rather than underlying aPL, caused renal failure.

  • Mostafa Elbanna, Fahad Eid, Mostafa Zaalouk, Ahmad Nawid Latifi, Gaurav Sharma
    Views: 147
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    This report presents a 57-year-old female with a history of dyslipidaemia, intolerant to statins and currently managed on evolocumab. Despite a healthy lifestyle, lipid panel abnormalities persisted, leading to an investigation that revealed heterozygous mutations in the ABCG8 gene, confirming a diagnosis of sitosterolaemia. The patient’s unique response to lipid-lowering medications typified this rare disorder, necessitating specialised genetic testing for diagnosis. Management involved dietary modifications and the introduction of ezetimibe, evolocumab and atorvastatin, demonstrating the personalised nature of treatment. The case underscores the importance of considering sitosterolaemia in unexplained lipid abnormalities and highlights the challenges in diagnosis and management. Ongoing research is crucial for refining diagnostic and therapeutic strategies for this clinically significant disorder, emphasising the need for a multidisciplinary approach to patient care.

  • Natalija Jevdokimova, Denis Jevdokimov, Jelena Ivanova , Sergejs Isajevs, Aiga Staka, Aldis Pukitis
    Views: 241
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    Background: Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide.
    Case description: A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team's findings, liver transplantation was indicated as the only curative treatment.
    Conclusion: Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis.