Acute liver failure histopathology: A 5-year retrospective study.

BACKGROUND: Acute liver failure (ALF) is a rare, life-threatening disease of diverse etiology. It is defined as severe acute liver injury for fewer than 26 weeks' duration with encephalopathy and impaired synthetic function (international normalized ratio [INR] of 1.5 or higher) in a patient without cirrhosis or pre-existing liver disease. The diagnosis rests mainly on the clinical ground with wide range of pathological features. The present study seeks to explore the diverse histological patterns observed in cases for ALF and assess their usefulness in determining the underlying causes for the condition. METHODOLOGY: A retrospective cross-sectional study was conducted among patients of ALF who underwent liver transplant and transjugular liver biopsy over a five-year period. From 1082 explant liver and 2446 liver biopsies, 22 cases of ALF (10 explants and 12 liver biopsies) were included in the study. Clinical and laboratory details were retrieved and histological findings were reviewed. RESULT: Age ranged from 10 to 72 years (mean age, 40 years). There was a female predominance with a male:female ratio of 1:1.7. The commonest cause for ALF was virus-induced hepatocellular damage in 36.3% (eight patients), followed by autoimmune hepatitis in 22.7% (five patients), drug-induced liver injury (DILI) in 18.1% (four patients), cryptogenic in 13.6% (three patients) and ischemic injury secondary to large vein thrombosis in 9.0% (two) patients. The histological patterns identified were categorized into six categories. A more comprehensive morphological evaluation was conducted specifically for cases of ALF associated with autoimmune hepatitis (AIH) and compared with other cases of ALF. CONCLUSION: In summary, our present study illustrates a morphological overlap in various patterns for the purpose of etiological assessment. In cases of AIH ALF, the presence of portal plasma cell infiltrate and central perivenulitis were identified as significant histological features to guide diagnosis.

Axillary Cystic Lymphangioma in an Adult: A Case Report.

Lymphangioma is a congenital malformation of the lymphatic system most often reported in children. Its occurrence in adults is rare. It usually develops in the head, neck, and axillary region. It mimics other conditions, such as cold abscess, simple cyst, hydatid cyst, and hemangioma, on clinical examination. Here, we report a case of cystic lymphangioma in the axillary region of a 32-year-old male. The patient underwent surgical excision and histopathology confirmed cystic lymphangioma. Although it is very rare in adults, cystic lymphangioma should be considered in the differential diagnosis of an axillary mass.

Gastric Neuroendocrine Tumor With Cystic Hepatic Metastases Mimicking Hepatic Echinococcosis: A Case Report.

Metastatic gastrointestinal neuroendocrine tumors classically appear as contrast-enhancing lesions on computed tomography. However, in a small percentage of patients, these lesions can be cystic in nature, leading to false diagnoses of benign or infectious lesions such as echinococcosis. Hence, every cystic lesion of the liver must be carefully investigated before making the treatment plan. We report a patient with hematemesis caused by a large gastric ulcer with multiple cystic lesions in the left lobe of the liver abutting the stomach. The liver lesions were misdiagnosed as hepatic echinococcosis, and the patient was started on medical therapy. However, when medical therapy failed, the patient underwent surgical excision and the histopathology showed cystic metastases of a gastric neuroendocrine tumor.

Liver histopathology in scope of hematological disorders.

Liver involvement is commonly seen in various haematological disorders. They present clinically with elevation of liver enzymes and organomegaly, with or without mass lesions. However, liver involvement may be silent in many hematological disorders or there may be specific findings in liver biopsy that can lead to the diagnosis of clinically inapparent hematological disorders. Present review highlights features of hepatic manifestations in various hematological diseases with special emphasis on histopathological findings. Among RBC disorders, secondary hemochromatosis is the commonest among patients with hemolytic anemia; whereas Sickle Cell Hepatopathy is a well known complication in Sickle Cell Disease, characterised by sequestration of sickled RBCs in sinusoids. Vascular complications such as Budd Chiari syndrome and portal venopathy with portal vein thrombosis are seen in patients with myeloproliferative neoplasms. However, sometimes primary hematological disease may remain occult. Various lymphomas show characteristic pattern of hepatic involvement, most common being sinusoidal and portal infiltration. Pattern of infiltration may give clues to different types of lymphomas. Amongst all lymphomas, Diffuse large B cell lymphoma is the most common lymphoma involving liver. Disseminated intravascular coagulation is a fatal systemic condition and liver involvement by widespread fibrin thrombi, is not an exception. Assessing liver histopathology in context of hematological conditions makes better understanding of pathophysiology and progress of these diseases. It is important for hematologists and hepatologist to be aware of possible liver involvement in various hematological diseases presenting with elevated LFTs and have a logical approach to abnormal LFTs.

Hepatic basidiobolomycosis masquerading as cholangiocarcinoma: a case report and literature review.

Basidiobolus ranarum is known to cause subcutaneous mycoses; however, rare cases of hepatic and gastrointestinal involvement by basidiobolomycosis have been reported. Hepatic basidiobolomycosis may be confused with a carcinoma on imaging, and histological examination and fungal culture can help distinguish between these two. We report a rare case of basidiobolomycosis in a 16-year-old male with liver and gastrointestinal involvement.

A Giant Pediatric Chylolymphatic Cyst: An Extremely Rare Entity.

Chylolymphatic cyst, variant of mesenteric cyst, is a rare entity. Clinical presentation and radiological features are not characteristic, so diagnosis can be made finally on histopathology. We report an extremely rare case of giant chylolymphatic cyst measuring >15 cm. A 2-year-old female presented with abdominal pain and vomiting. On examination, a ill-defined and firm mass was palpable just below umbilicus. Positron emission tomography-computed tomography scan revealed a large ill-defined lesion, which measured 16 × 13.2 × 6.7 cm in size and was seen in relation to the abdominal mesentery. Provisional diagnosis of mesenteric cyst was made. Laparotomy revealed multiple lymphatic cysts of variable size arising from the mesentery of proximal ileum. Histopathology examination confirmed the presence of a giant chylolymphatic cyst. Chylolymphatic cysts are rare entity and should be kept in mind while diagnosing a pediatric case of abdominal cysts.

Small Cell Type Undifferentiated Carcinoma of Gall Bladder with Pas Positive Hyaline Globule Masquerading as Liver Mass: A Case Report and Literature Review.

An undifferentiated carcinoma (UC) of the gall bladder behaves aggressively and has a grave prognosis. Small cell type undifferentiated carcinoma of the gall bladder is a rare variant. This paper reports a case of UC of gall bladder with PAS-positive diastase- resistant eosinophilic hyaline globules present as liver mass (on imaging) in a male patient. The microscopic findings of the liver and gall bladder after a right tri-segmentectomy showed an un-differentiated malignant neoplasm composed of cells with round to oval nuclei, prominent nucleoli, and scanty neoplasm. No definite cell pattern was identified with these neoplastic cells. A section from the gall bladder revealed a tumor arising from the lining epithelium and infiltrating through the muscularis. This tumor was infiltrating the adherent liver tissue directly and forming a mass of undifferentiated malignant cells. The focal area within the tumor mass showed the presence of PAS-positive, diastase-resistant, eosinophilic hyaline globules within the neoplastic cells. The immunohistochemistry test was diffusely positive for perinuclear anti-neutrophil cytoplasmic antibodies and negative for chromogranin, vimentin, Desmin, alpha-fetoprotein, leukocyte common antigen, CD34, and bcl2. When the clinical and radiological data are inconclusive, careful analysis of the histological and immunophenotypic features is needed to make the final diagnosis of UC of the gall bladder. The biological behavior and prognosis of this tumor remain unclear because of its rarity. Further studies will be needed to understand the characteristics of this deadly tumor and to establish an effective therapy for it.

Hypertrophic Perianal Herpes Mimicking Squamous Cell Cancer: A Case Report and a Review of the Literature.

Clinical practice frequently involves the discovery of perineal lesions. The human papillomavirus, molluscum contagiosum, and herpes simplex virus are to blame for the majority of these anogenital lesions. In the majority of cases, these lesions may be identified by their distinctive appearance. It is challenging to make a clinical diagnosis in immunocompromised people since these lesions might be large and have uncommon appearances. Verrucous perianal herpes is a rare type of herpes that resembles squamous cell carcinoma in gross appearance. We present a case of a 71-year-old man on azathioprine, an immunosuppressive drug for autoimmune pancreatitis, who developed a perianal lesion resembling squamous cell carcinoma. Excisional biopsy revealed a benign ulcerative lesion with herpetic inclusions. The patient received antiviral treatment, and the perianal wound completely healed. He developed a similar lesion in the perineum at one year follow up, which was successfully treated with oral and topical antivirals.

Pancreatoduodenal Tuberculosis: A Rare Site of a Common Disease.

The most common site of abdominal tuberculosis (TB) is the ileocecal region. The duodenum and pancreas are rare sites of abdominal TB. It is usually observed in immunocompromised patients with miliary or disseminated TB. Pancreatoduodenal TB is often misdiagnosed as malignancy due to a lack of specific symptoms. Here, we present a case of a 55-year-old immunocompetent man having abdominal pain, bilious vomiting, weight loss, and high-grade fever for three months. Contrast-enhanced computed tomography of the abdomen showed a localized abscess with air foci in the pancreatic head and duodenopancreatic groove. Additionally, there was retroperitoneal lymphadenopathy and mild ascites. Upper gastrointestinal endoscopy revealed an ulceroproliferative lesion in the second part of the duodenum suspicious of a malignant tumor. However, the endoscopic biopsy showed epitheloid-like histiocytes, Langerhans type of giant cells, and granuloma formation suggestive of TB. The patient responded to antitubercular treatment and became symptom-free.

Multifocal Dedifferentiated Liposarcoma of the Jejunal Mesentery.

Abdominal liposarcoma is most often located in the retroperitoneum. Small bowel mesentery is a rare site of liposarcoma. Dedifferentiated liposarcoma (DDLS) is a rare variant of mesenteric liposarcoma with only 12 cases reported in the literature. DDLS has a worse prognosis compared to a well-differentiated liposarcoma. Here, we present a case of giant liposarcoma located in the jejunal mesentery diagnosed on the contrast-enhanced computed tomography of the abdomen. The patient underwent complete surgical excision with uneventful postoperative recovery. DDLS was confirmed on histopathological examination of the resected specimen. The patient is currently receiving adjuvant therapy with doxorubicin and ifosfamide in view of close resection margin, mitotic rate and high rate of recurrence.

Lambda-Restricted Crystal-Storing Histiocytosis of Stomach: A Case Report and Review of Literature.

Crystal-storing histiocytosis (CSH) is a rare tissue phenomenon that is usually associated with lympho-proliferative diseases. The disease is characterized by prominent collections of macrophages with abundant eosinophilic cytoplasm and fibrillary cytoplasmic inclusions. The inclusions appear as linear crystals within the macrophages which are usually kappa restricted. The disease usually involves lungs, lymph nodes, bone marrow, thymus and spleen with rare involvement of the gastrointestinal tract. We report a rare case of lambda-restricted CSH of the stomach. The diagnosis of CSH triggered further hematological evaluation. The patient was later diagnosed to have diffuse large B-cell lymphoma involving lymph nodes and bone marrow. He received chemotherapy for the same and is on regular follow up. The index case highlights the need to identify CSH of stomach prompting evaluation for hematological malignancies and to increase its awareness among clinicians and pathologists.

Primary multifocal cystic signet ring neuroendocrine tumor of liver: a case report.

Primary signet ring neuroendocrine tumors of the liver are extremely rare tumors. Morphologically, they mimic signet ring cell adenocarcinomas; however, the absence of mucin by special stains and the expression of neuroendocrine markers help to diagnose these tumors. We herein report a case of a 47-year-old female who presented with multiple solid and cystic lesions in both liver lobes, which were initially suggested to be biliary cystadenocarcinoma on imaging. Liver biopsy of the lesion revealed the presence of a signet ring neoplasm with diffuse expression of synaptophysin and pan-cytokeratin. The case was subsequently diagnosed as a primary hepatic signet ring neuroendocrine tumor. The patient was offered 3 cycles of chemotherapy and is well preserved after 14 months of diagnosis. Although this is an extremely rare entity, its possibility should be considered in the differential diagnosis of neoplasms characterized by signet ring cell morphology.

A Case of Ruptured Perineal Epidermal Cyst.

Epidermal cysts are common skin lesions. They are usually seen in the chronic sun-exposed areas of the skin. They can get complicated by inflammation, infection, rupture, or malignancy. Epidermal cyst in the perineal region is rare. We report a case of an infected ruptured perineal epidermal cyst mimicking gluteal abscess. The patient was successfully treated by complete surgical excision. Histology revealed epidermal cyst with foreign body giant cell reaction and no evidence of malignancy.

Angiokeratoma Corporis Diffusum: An Uncommon Case with Suspected Anderson Fabry Disease.

Angiokeratomas are variable sized hyperkeratotic vascular papules that are characterized histologically by superficial dilated capillaries in papillary dermis with epidermal proliferation. They can occur as a single lesion to a generalized form (angiokeratoma corporis diffusum). Angiokeratoma corporis diffusum though initially synonymous with Anderson Fabry disease, is now known to occur in a variety of lysosomal enzyme deficiencies. We report a case of 22 year old male with angiokeratoma corporis diffusum associated with acroparesthesias, febrile episodes, sensorineural hearing loss and renal involvement. Histopathological evaluation showed characteristic ectatic blood vessels with vacuolated endothelial cells in papillary dermis. Based on the clinical evaluation and available investigations, we suspected him to be having to Anderson fabry disease. Resource constraints limited our ability to confirm our diagnosis with enzyme assay and electron microscopy. We report this unusual case in desire of re emphasizing the importance of clinical evaluation for reaching a diagnosis in a resource poor setting.

A report on three patients with Echinococcus multilocularis: Lessons learned.

Echinococcus multilocularis (EM) is the most virulent species of the genus Echinococcus. It causes a highly lethal helminthic disease in humans. The disease may present as hepatic mass mimicking a malignant neoplasm. Due to the vascular and neural invasion, protean clinical manifestations including Budd-Chiari syndrome (BCS) may be the clinical presentation of this condition. We herein report three cases of Echinococcus multilocularis; the first case presenting as multiple hepatic space-occupying lesions, second as liver mass infiltrating the nerve bundles, and the third as a hepatic mass infiltrating the large vessels including inferior vena cava and right hepatic vein presenting as BCS. EM is a parasite with capabilities of mass-forming effect, neural and vascular invasion. Though cases of BCS have been described, most of these are due to secondary compression and rarely by direct parasitic invasion.

Early cirrhosis in a young female with protein C deficiency: An extremely unusual case report with review.

Protein C deficiency is a well recognized risk factor for development of venous thromboembolism but has never been reported to be associated with development of liver cirrhosis .We report a case of a 26 years old female who presented with multiple thrombosis involving superior mesenteric vein ,main portal vein and multiple cerebral veins. Liver biopsy done was reported as cirrhosis possibly due to Wilson's disease. However no improvement was seen with D penicillamine and patient's condition detiorated. Further, work up of patient revealed absence of Protein C levels in the plasma. So finally the case was diagnosed as Cirrhosis liver with Protein C deficiency as the likely etiology. We conclude that Protein C deficiency should be investigated in patients with cirrhosis with thrombotic lesions of unknown etiology.

Different Nodules Identified during Liver Explant Gross Examination: Relevance and Need for Sectioning-Experience from India.

Objective. The goal of this study was to determine the etiopathological association of various hepatic nodules identified during gross examination of liver explants specimen and the grossing aspects of these abnormal nodules especially those smaller than 1 cm in diameter. Our aim was to analyze whether there is any association of macroregenerative and dysplastic nodule with hepatocellular carcinoma. Materials and Methods. Fifty consecutive liver explants specimens were analyzed for the presence of any abnormal nodule (abnormal nodule defined as any nodule different in color, texture, and appearance from adjacent liver tissue). Results. Of the total 40 abnormal nodules identified in 50 liver explant specimens, there were 12 (30%) HCC [including 5 small HCC (41% of total HCC) and 1 steatohepatitic HCC (8% of total HCC)], 11 (27%) MRNs, 8 (20%) dysplastic nodules, and 9 (22%) necrotic nodules. Most cases (72%) of MRN are seen in hepatitis C virus related cirrhosis with only 2 cases having associated HCC. Most cases of HCC were seen in cases of HBV associated cirrhosis (60%). The association of MRN was not found to be significantly associated with HCC with a p value of 1.0. Dysplastic nodules were found to be significantly associated with HCC with a p value of 0.02. Conclusion. In hepatic carcinogenesis, the role of MRN does not appear to be significant. However, the presence of dysplastic nodules is significantly associated with HCC. The study identified another variant of cirrhotic nodules herein called necrotic nodules that are mostly tan greenish in color and <0.5 cm in diameter. No dysplastic changes were identified in any of these nodules disqualifying the need of sectioning in such nodules.