Extremely high alpha-fetoprotein-producing adrenal hepatoid adenocarcinoma

2021 ◽  
Vol 14 (7) ◽  
pp. e239830
Author(s):  
Tawasapon Thambamroong ◽  
Naiyarat Prasongsook ◽  
Kantang Satayasoontorn ◽  
Siriwimon Saichaemchan

Hepatoid adenocarcinoma (HAC) is a rare tumour that produces an alpha-fetoprotein (AFP) mimicking hepatocellular carcinoma (HCC). Adrenal HAC is exceedingly rare. Here we report extremely high AFP-producing adrenal HAC, the first case in Thailand. A 47-year-old man presented with left flank pain and weight loss for 2 months. A palpably huge left flank mass was observed on physical examination. CT revealed a 7 cm enhanced mass involving the left adrenal gland and multiple contrast-enhanced hypodense masses in both liver lobes. The largest was a 3.7 cm at liver segment-VII without cirrhotic background, with an AFP level of 321 495 ng/mL. Both adrenal and liver biopsies were performed. This patient received a diagnosis of advanced adrenal HAC. Unfortunately, the tumour progressed, causing massive upper gastrointestinal bleeding and death. Adrenal HAC is challenging to diagnose, which multifocal HCC, pheochromocytoma and adrenocortical carcinoma should be excluded. Surgical resection is preferred among resectable patients. However, no systemic therapy has been standardised.

2021 ◽  
pp. 212-216
Author(s):  
Hisashi Komoto ◽  
Kazuhiro Kitajima ◽  
Yusuke Kawanaka ◽  
Nahomi Yoshimura ◽  
Ryo Kunimoto ◽  
...  

Primary angiosarcomas of the kidney are very rare but highly aggressive tumors showing poor prognosis. We present a case of primary renal angiosarcoma occurring in a 60-year-old man with left flank pain. CT images depicted a huge exophytic mass (14 cm in diameter) in the left kidney, exhibiting central extensive hemorrhage or necrosis without contrast enhancement. The mass showed centripetal peripheral nodular enhancement on dynamic contrast-enhanced CT images. We suggest its inclusion in the differential diagnosis of cases of hemorrhagic renal tumors with prominent vasculature.


2018 ◽  
Vol 8 (2) ◽  
pp. 1416-1418
Author(s):  
Shankar Bastakoti ◽  
Binay Thakur ◽  
Mukti Devkota ◽  
Amrita Paudel

Adrenal angiomyolipoma is a rare benign entity and only to sixteen cases have been reported in English literature till date. Other site of occurrence is liver, spleen, lungs, bone and ovary. We report a female aged 54 years, who presented with left flank pain, on CT scan showed left adrenal mass. Patient underwent laparoscopic adrenalectomy and final histopathological examination revealed angiomyolipoma of   left adrenal gland.


2020 ◽  
Vol 13 (3) ◽  
pp. 1552-1559
Author(s):  
Yuki Muroyama ◽  
Hiroyuki Tamiya ◽  
Goh Tanaka ◽  
Wakae Tanaka ◽  
Alexander C. Huang ◽  
...  

Lung hepatoid adenocarcinoma (HAC) is a rare primary lung carcinoma pathologically characterized by hepatocellular carcinoma-like tumor cells, the majority of which produce alpha-fetoprotein (AFP). The clinical prognosis of lung HAC is generally poor, and effective therapeutic regimens for inoperable or recurrent cases have not been established. Here, we report a case of AFP-producing lung HAC with brain metastasis with long-term disease control, treated with the 5-fluorouracil-derived regimen S-1. The patient was a 66-year-old male admitted to the hospital with alexia. Chest X-ray revealed a massive tumor in the left upper lobe, and a head CT scan revealed a metastasis in the left parietal lobe. The laboratory data showed a remarkably elevated AFP level (97,561 ng/mL). Pathological assessment of the resected brain tumor revealed HAC, which was compatible with the lung biopsies. Together with the absence of other metastatic lesions, a final diagnosis of primary lung HAC, stage IV T4N3M1b, was given. The patient first underwent non-small cell lung cancer chemotherapy regimens (carboplatin and paclitaxel as the first line, and pemetrexed as the second line), but had clinical progression. After third-line oral S-1 (tegafur/gimeracil/oteracil) administration, the serum AFP level significantly dropped and the patient achieved long-term disease control without relapse, surviving more than 19 months after disease presentation. The autopsy result was consistent with the diagnosis of primary lung HAC, and immunohistochemical staining was AFP+, glypican 3+, and spalt-like transcription factor 4+. Here, we report the case of a rare primary lung HAC with apparent disease control on S-1 therapy, together with a literature review.


2020 ◽  
Vol 26 (6) ◽  
pp. 1505-1510
Author(s):  
Onder Tonyali ◽  
Onur Gonullu ◽  
Mehmet Akif Ozturk ◽  
Aysun Kosif ◽  
Ozlenen Gonca Civi

Introduction Hepatoid adenocarcinoma of the lung is an extremely rare type of the non-small cell lung cancer. Treatment principles and prognosis are similar to that of lung adenocarcinoma. Case report A 62-year-old female smoker presented with a huge mass in the left upper lobe. After diagnostic biopsy, she underwent left pneumonectomy and mediastinal lymph node dissection. A diagnosis of stage T4N1M0 hepatoid adenocarcinoma of the lung with positive surgical margins was made. Management and outcome After the operation, the level of serum alpha fetoprotein was 9010 ng/ml (N: <10). The level of serum alpha fetoprotein was decreased with concurrent chemoradiotherapy and chemotherapy. Disease progression was detected at 11 months after diagnosis. No response was obtained to other therapies. The patient died at 14 months from the time of diagnosis. Discussion Usually, patients with hepatoid adenocarcinoma of the lung are male smokers. Hepatoid adenocarcinoma tends to settle in the upper lobes of the lung. The most important prognostic factor of the hepatoid adenocarcinoma of the lung is the disease stage at the diagnosis and patients with metastatic disease have poor survival.


2003 ◽  
Vol 127 (3) ◽  
pp. 367-369 ◽  
Author(s):  
Michael O. Idowu ◽  
Melissa Contos ◽  
Satinder Gill ◽  
Celeste Powers

Abstract Merkel cell carcinoma (MCC) is an uncommon, highly aggressive cutaneous neoplasm of neuroendocrine differentiation with a poor prognosis. MCC most often presents as a painless, firm, raised lesion in sun-exposed sites of the head and neck region of the elderly. We report a case of a metastatic MCC to the stomach presenting as upper gastrointestinal bleeding. To our knowledge, this is the second reported case of MCC presenting as upper gastrointestinal bleeding and the first case confirmed by the newer immunohistochemical techniques. The literature is reviewed.


Author(s):  
Manjeet Kumar ◽  
◽  
Sanjeev Chauhan ◽  

A 38 years female presented with left flank pain, nausea, and vomiting. Ultrasound and Noncontrast CT scan were suggestive of left hydronephrosis with left Vesicoureteral stone (Figure 1). Conservative medical treatment previously was not successful. Blood investigations were Hb 11.5 gm%, TLC 11500/mm3 , urea 22, creatinine 0.6, urine was full of RBCs. She was taken to the operation theatre for emergency double j stenting. Cystoscopy showed impacted left vesicoureteral stone (Figure 2,3).


2020 ◽  
Author(s):  
Zhihong Chen ◽  
Lutong Fang ◽  
Ying Zhang ◽  
Yuanzi Ye ◽  
Wenshu Ji ◽  
...  

Abstract Background: Hepatic mesenchymal hamartoma (HMH) is an uncommon benign tumor in children. While mesenchymal hamartomas may be angiomatous and blood vessels may be identified, HMH with a malignant tumor symptom on the contrast-enhanced hepatic computed tomography angiography (CTA) has not been described. Here, we present the first case of HMH mimicking hepatoblastoma on the hepatic CTA from pathological point of view and review the imaging and histological features of this unique lesion. Case presentation: A 2-year-old female child was found a distention in the right abdomen and was admitted to our hospital. The Hepatic CTA showed that the blood vessels were thickened, the tumor blood vessels were clustered in the tumor. According to the hepatic CT findings, the tumor was considered to be malignant, possibly a hepatoblastoma. Microscopic examination showed a tumor arranged in lobules, composed of loose myxoid mesenchyme surrounding ductal structures, with intervening vascular channels. The Immunohistochemical staining revealed positive CK7 and CD34 for the bile duct elements and the lining endothelial cells of the vascular channels. There were abundant blood vessels around the nodules and the margins of remaining hepatocytes, but few in the central region. The blood vessels are small, thin-walled vessels and presented like capillaries and venules. Conclusions: A histological diagnosis of Hepatic Mesenchymal Hamartoma was confirmed by the microscopic examinations. This case adds learning points to radiologists when heterogeneous reinforcement on enhanced CT scan was presented in such a large childhood liver tumor.


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