American Journal of
Case Reports
| Received: | 2023.01.02 |
| Accepted: | 2023.04.10 |
| Available online: | 2023.04.19 |
| Published: | 2023.05.29 |
A Rare Case of Primary Adrenal Epithelioid Angiosarcoma
Authors’ Contribution:
Study Design A
Data Collection B
Statistical Analysis C Data Interpretation D Manuscript Preparation E Literature Search F Funds Collection G
ABCDEFG 1 Komson Wannasai İD
BDF 2 Puwitch Charoenchue B 3 Sunhawit Junrungsee BEF 4 Chanakrit Boonplod İD
D
1 Kornkanok Sukpan İD
1 Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
2 Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
3 Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
4 Medical Student, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
Corresponding Author:
Komson Wannasai, e-mail: komson.wanna@gmail.com
Financial support:
This work was supported by the Faculty of Medicine, Chiang Mai University [grant number 029-2566]
Conflict of interest:
None declared
Patient:
Male, 59-year-old
Final Diagnosis:
Primary adrenal epithelioid angiosarcoma with a background of adrenal cortical adenoma
Symptoms: Abdominal bloating
Clinical Procedure:
Adrenalectomy
Specialty: Pathology . Radiology . Surgery
Rare disease
Objective: Background: Primary adrenal epithelioid angiosarcoma (PAEA) is a very uncommon primary adrenal gland tumor that usu- ally occurs around the age of 60 years and is more common among males. Owing to its rarity and histopatho- logical features, PAEA could be misdiagnosed as adrenal cortical adenoma, adrenal cortical carcinoma, or oth- er metastatic cancers, such as metastatic malignant melanoma and epithelioid hemangioendothelioma.
Case Report: A 59-year-old male patient presented to our hospital with a complaint of abdominal bloating that started 2 months prior. His vital signs and the results of his physical and neurological examinations were unremarkable. A computed tomography scan showed a lobulated mass arising from the hepatic limb of the right adrenal gland but no evidence of metastasis to the chest or abdomen. The patient underwent right adrenalectomy, and the macroscopic pathological findings from a right adrenalectomy specimen revealed atypical tumor cells with an epithelioid appearance in the background of an adrenal cortical adenoma. Immunohistochemical staining was performed to confirm the diagnosis. The final diagnosis was epithelioid angiosarcoma involving the right adre- nal gland with a background adrenal cortical adenoma. The patient had no postoperative complications, pain in the surgical wound, or fever. Therefore, he was discharged with a schedule for followup appointments. Conclusions: PAEA may be misinterpreted as adrenal cortical carcinoma, metastatic carcinoma, or malignant melanoma ra- diologically and histologically. Immunohistochemical stains are essential for diagnosing PAEA. Surgery and strict monitoring are the main treatments. In addition, early diagnosis is essential for patient recovery.
Keywords: Adrenal Gland Diseases . Sarcoma . Case Reports . Abdominal Neoplasms . Abdominal Pain
Full-text PDF:
https://www.amjcaserep.com/abstract/index/idArt/939397
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Background
Angiosarcoma is a rare tumor that accounts for less than 2% of all sarcomas [1]. It is typically found in the skin, head and neck, extremities, peritoneum, body wall, retroperitoneal area, breast, liver, spleen, heart, lung, bone, or adrenal glands [2,3], and rarely in the gastrointestinal tract, urinary bladder, penis, or testes [4]. Angiosarcomas can be subdivided into a number of subtypes based on differentiation. These subtypes range from well-differentiated tumors with numerous vascular channels to poorly differentiated tumors with solid sheets and no vas- cular formations [5]. Additionally, angiosarcomas can be sub- divided based on the histopathology of the tumor cells, such as spindle, polygonal, epithelioid, round, or solid sheets [1].
Primary adrenal epithelioid angiosarcoma (PAEA) is an extremely rare primary adrenal gland tumor that typically occurs around the age of 60 years and is more common in men [6]. The tu- mor is composed of sheets of pleomorphic, atypical, endothe- lial cells that exhibit an epithelioid morphology [7]. Owing to its rarity and histopathological characteristics, PAEA may be misdiagnosed as adrenal cortical adenoma, adrenal cortical carcinoma, or other metastatic tumors, including metastat- ic malignant melanoma and epithelioid hemangioendotheli- oma [8], and can be diagnostically challenging with regard to both radiologic and pathologic findings. Herein, we report a case of PAEA diagnosed at our hospital and review previous- ly reported cases of PAEA with a discussion on radiologic and pathologic findings with possible differential diagnoses to fa- cilitate accurate diagnosis of the disease.
Case Report
A 59-year-old male patient presented to our hospital with a complaint of abdominal bloating that started 2 months prior. He reported that the severity of his symptoms increased after eating, particularly after eating fatty foods, and that he experi- enced intermittent pain on the right side of his abdomen. The patient reported that he had no diarrhea, anorexia, weight loss, fever, palpitations, or headaches. However, he had a 30-year history of alcohol consumption and smoking, both of which he had quit 1 year prior to his visit. On physical examination, the patient was fully conscious, cooperative, and oriented. His vital signs were unremarkable. The patient did not have pale conjunctivae, icteric sclerae, an irregular heartbeat, heart mur- murs, or abnormal respiration, and except for a right intra-ab- dominal mass, there was no evidence of hepatosplenomegaly. The results of his neurological examination were unremarkable.
A computed tomography (CT) scan of the patient’s abdomen (Figure 1) showed an 8.7×7.5×6.3 cm lobulated mass aris- ing from the right adrenal gland. The tumor compressed the
surrounding right lobe of the liver, right kidney, and inferior vena cava without any apparent invasion. No evidence of me- tastasis to the chest or abdomen was detected. Several punc- tate calcifications were visible. On contrast-enhanced images, there were a few peripheral arterial enhancing foci with pro- gressive enhancement in later-phase images. However, inho- mogeneous enhancement persisted, representing necrosis. As these CT findings were not indicative of a specific tumor, mag- netic resonance imaging (MRI) was performed.
MRI (Figure 2) revealed that the mass was heterogeneously en- hanced, with non-enhanced areas of necrosis, similar to those observed on CT. Additionally, MRI revealed T1-hyperintense re- gions in fat-suppressed images, indicating intra-tumoral hem- orrhages. A small area of intracytoplasmic fat was observed as T1-signal drop on the opposed-phase T1-weighted image. However, no gross fat component was detected on MRI or CT images. Despite the indeterminate nature of the mass as re- vealed by the MRI, imaging exhibited a notable absence of vas- cular invasion, lymphadenopathy, or distant metastases, there- by implying the likelihood of surgical resectability.
Preoperative laboratory analysis (Table 1) revealed eosinophil- ia with no anemia and normal blood electrolyte levels and re- nal function. The metanephrine and vanillylmandelic acid lev- els in the patient’s urine were unremarkable.
The patient underwent right adrenalectomy. The gross speci- men (Figure 3) revealed the presence of a well-circumscribed encapsulated adrenal tumor measuring 8.5×6×6.5 cm. Cut sur- faces of the tumor showed 3 yellow-tan nodules with hem- orrhagic areas. On microscopic examination (Figure 4), the tumor demonstrated anastomosing vascular channels with atypical neoplastic cells in the background of an adrenal cor- tical adenoma. The tumor cells were characterized by large, atypical epithelioid appearance with abundant eosinophilic cy- toplasm, vesicular nuclei, and prominent nucleoli. The mitot- ic count was 7/10 high-power fields. Immunohistochemistry showed that the tumor cells were strongly positive for CD31 (Figure 5), CD34, and Factor VIII but only partially positive for cytokeratin (CK) (Figure 5). These features suggested a vascu- lar origin of the tumor cells. To confirm the diagnosis of angio- sarcoma, immunohistochemical staining for FLI1 and INI1 was performed, which showed positive results (Figure 5). However, we needed to exclude other malignant tumors, such as adrenal cortical carcinoma, metastatic malignant melanoma, neuroen- docrine tumor, or germ cell tumor. Thus, additional immuno- histochemical stains for inhibin, calretinin, melan-A, synap- tophysin, S-100, and HMB45 were performed. All the stains returned negative results. The final diagnosis was epithelioid angiosarcoma involving the right adrenal gland with a back- ground adrenal cortical adenoma. The surgical margins of the specimen were free of malignancy.
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The patient had no postoperative complications, pain in the surgical wound, or fever. In a multidisciplinary team confer- ence, the oncologist decided against chemotherapy or radio- therapy owing to complete (R0) resection of the tumor. Thus, the patient was discharged by the surgeon with a followup ap- pointment for imaging 4 months from discharge. Unfortunately, the patient did not visit for a postoperative examination and was eventually lost to followup.
Discussion
We report a 59-year-old male patient who presented with ab- dominal bloating. Radiologic examinations revealed a right
adrenal mass. A diagnosis of PAEA was made according to pathologic findings.
PAEA is a very rare malignant vascular tumor of the adrenal gland [6]. In 1988, Kareti et al [9] first described PAEA in a 54-year-old male patient who presented with abdominal pain and a left adre- nal mass. Although the tumor - showing characteristics of an epi- thelioid angiosarcoma - was surgically removed, the patient expe- rienced recurrence and died 7 months after surgery. In 2018, Yang et al [6] reviewed 37 cases of PAEA, including the case reported by Kareti et al, while Wei et al [10] reported 1 case of PAEA in 2021.
The cause of PAEA remains unknown [11]. However, it has been reported that PAEA is linked to exposure to insecticides
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containing arsenic [12]. PAEA may also be found in patients with a history of vinyl chloride exposure [13]. Livaditou et al [14] described a 59-year-old male patient who presented with ab- dominal pain in the right upper quadrant and had a history of exposure to an insecticide containing arsenic, for over 20 years. A mass was detected in the upper pole of the kidney, and pathological examination confirmed that it was an epi- thelioid angiosarcoma.
Patients with PAEA typically present with an adrenal mass and non-specific symptoms such as abdominal pain, weight loss, and fatigue. However, some patients may be asymptomatic and the tumor may be discovered incidentally through imaging [8].
As PAEA is uncommon and has histopathological characteris- tics similar to those of many other types of tumors, including adrenal carcinoma, metastatic carcinoma, malignant melano- ma, and other types of tumors with epithelioid morphology characteristics, its diagnosis is challenging. However, imaging and pathological studies with immunohistochemical staining can aid in the accurate diagnosis of the disease [6].
Given that CT and MRI of PAEA reveal peripheral hypervascu- larity with intratumoral hemorrhages, necroses, scant punctate calcifications, and minute intracytoplasmic fat, its differential diagnoses include benign or malignant pheochromocytoma, cortical carcinoma, bleeding adenoma, bleeding hemangioma,
| Parameter | Value | Reference range |
|---|---|---|
| Complete blood count | ||
| Hemoglobin | 15.6 g/dL | 13.0-18.0 g/dL |
| Hematocrit | 48.6% | 36.0-48.0% |
| White blood cells | 9550 cells/uL | 5000-10 000 cells/uL |
| Neutrophils | 49.0% | 40.0-74.0% |
| Eosinophils | 9.4% | 0.0-7.0% |
| Basophils | 0.7% | 0.0-2.0% |
| Lymphocytes | 35.0% | 19.0-48.0% |
| Monocytes | 5.0% | 3.0-9.0% |
| Platelets | 264 000 cells/uL | 14 0000-450 000 cells/uL |
| MCV | 82.8 fL | 80.0-99.0 fL |
| MCH | 26.6 pg | 27.0-31.0 pg |
| MCHC | 32.1 g/dL | 31.0-35.0 g/dL |
| RDW | 11.7% | 11.5-14.4% |
| Blood coagulogram | ||
| PT | 10.70 s | 9.50-13.00 s |
| INR | 0.93 | <5.00 |
| aPTT | 33.10 s | 28.10-38.90 s |
| Biochemistry analysis | ||
| Glucose | 150 mg/dL | 0.00-1.20 mg/dL |
| Serum cortisol | 7.27 mg/dL | ≤0.30 mg/dL |
| BUN | 26 mg/dL | 6-20 mg/dL |
| Creatinine | 1.99 mg/dL | 0.97-1.77 mg/dL |
| Na | 142 mmol/L | 136-145 mmol/L |
| K | 4.5 mmol/L | 3.4-4.5 mmol/L |
| Cl | 109 mmol/L | 98-107 mmol/L |
| Total CO2 | 22 mmol/L | 22-29 mmol/L |
| Ca | 9.5 mg/dL | 8.6-10.2 mg/dL |
| P | 4.4 mg/dL | 2.5-4.5 mg/dL |
| Mg | 1.70 mEq/L | 1.32-2.14 mg/dL |
| Metanephrine in urine | 74.91 mg/day | <350 mg/day |
MCV - mean corpuscular volume; MCH - mean corpuscular hemoglobin; MCHC - mean corpuscular hemoglobin concentration; RDW - red cell distribution width; PT - prothrombin time; INR - international normalized ratio; aPTT - activated partial thromboplastin time; BUN - blood urea nitrogen; Na - sodium; K - potassium; CI - chloride; Ca - calcium; P - phosphate; Mg - magnesium.
metastasis, and mesenchymal tumors such as sarcomas or neu- rogenic tumors, as well as collision, which have been reported to show heterogeneous enhancement with possible calcifica- tions [15-17]. The clinical manifestations and laboratory find- ings of PAEA are usually benign. Imaging cannot be used to precisely diagnose the mass; therefore, surgical resection and histopathological analysis are required. However, in surgical planning, the size of the adrenal mass obtained from imaging can be used as another essential factor [16,17].
Some previous case reports indicate that the imaging findings of adrenal angiosarcoma include a circumscribed mass with T1-hyperintensity, which is likely an intratumoral hemorrhage, punctate calcifications, heterogeneous enhancement, and de- layed enhancement [18-21]. However, a greater proportion of
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cystic components in adrenal angiosarcoma has been report- ed in other case reports [22,23]. Considering the present case and previous reports, adrenal lesions with these characteris- tics have numerous differential diagnoses; thus, their diag- nosis is inconclusive. The purpose of imaging is to determine the resectability of the tumor and search for other indicators, such as intratumoral fat or a primary tumor that has spread to the adrenal gland. Surgical resection and histopathological examination are required in the absence of other indicators.
Bosco et al [24] described a case of PAEA in the right adrenal gland of a 54-year-old patient and reported that the tumor cells tested positive for factor VIII and cytokeratin. In another case of PAEA in the right adrenal gland of a 63-year-old patient re- ported by Croitoru et al [25], the tumor cells tested positive for CD34, vimentin, and factor VIII but negative for S-100, chro- mogranin, HMB 45, and CAM 5.2. Ben-Izhak et al [26] also re- ported positive results for FVIII-RA, UEA-I, vimentin, CAM 5.2, and broad-spectrum CK in the cells of a PAEA in a male pa- tient aged 41 years.
In the present case, the differential diagnoses included adrenal cortical carcinoma, metastatic carcinoma, and metastatic mel- anoma because the tumor cells exhibited characteristics sim- ilar to those of these tumors, such as enlarged nuclei, distinct nucleoli, a high N/C ratio, and abundant cytoplasm. Therefore, immunohistochemical staining was necessary to aid in accurate diagnosis. The stains returned positive results for cytokeratin and negative results for inhibin, calretinin, melan-A, HMB45, synaptophysin, and S-100. The negative results for calretinin, inhibin, and melan-A indicated that the tumor cells did not orig- inate from the adrenal cortex or exhibit adrenal cortical differ- entiation [6,27]. Additionally, the negative results for HMB-45 and S-100 excluded metastatic melanoma. We performed ad- ditional immunohistochemical staining for endothelial mark- ers (CD31, CD34, and factor VIII), FLI-1, and INI1. The results revealed that the tumor cells were positive for all 3 endothe- lial markers, exhibited a focally positive effect on FLI-1, and expressed INI1. The fact that the tumor cells expressed CD31, CD34, and factor VIII indicated that endothelial cells were in- deed the source of the tumor cells [28] and that metastatic carcinoma could be ruled out since carcinomas are negative
| Author | Pub. Year | Age (y) | Sex | Side | Size (cm) | Presentation |
|---|---|---|---|---|---|---|
| Kareti et al [9] | 1988 | 54 | Male | Left | 6.0 | Left upper quadrant abdominal pain |
| Yang et al [6] (1) | 2018 | 60 | Male | Left | 6.5 | High blood pressure |
| Yang et al [6] (2) | 2018 | 60 | Male | Left | 8.0 | Severe pain in left lumbar region |
| Wei et al [10] | 2021 | 51 | Male | Left | 4.0 | Lower back pain |
| Livaditou et al [14] | 1991 | 59 | Male | Right | 7.0 | Right upper quadrant pain |
| Li et al [13] | 2017 | 59 | Male | Left | 5.4 | Left flank pain |
| Imran et al [19] | 2020 | 38 | Female | Right | 8.0 | Hemoptysis |
| Bosco et al [24] | 1991 | 54 | Female | Right | 6.0 | Fatigue |
| Croitoru et al [25] | 2001 | 63 | Male | Left | 3.0 | Incidental finding |
| Ben-Izhak et al [26] | 1992 | 41 | Male | Right | 10.0 | Epigastric discomfort |
| Lepoutre-Lussey et al [33] | 2012 | 35 | Male | Right | 6.0 | Polyuria and hypertension |
| Pasqual et al [21] | 2002 | 70 | Female | Right | 5.0 | Abdominal pain |
| Rodríguez-Pinilla et al [11] | 2002 | 61 | Male | Right | 12.0 | Abdominal pain |
| Criscuolo et al [12] | 2013 | 68 | Male | Left | 7.0 | Pain in left thorax |
| Antao et al [8] | 2019 | 58 | Male | Left | 17.5 | Left-sided abdominal pain |
| Sung et al [22] | 2013 | 42 | Male | Right | 14.0 | Right flank pain |
| Author | Management | Chemotherapy | Radiotherapy | Outcome |
|---|---|---|---|---|
| Kareti et al [9] | Left adrenalectomy with en bloc resection | None | None | Free of tumor |
| Yang et al [6] (1) | Laparoscopic left adrenalectomy | None | None | Alive |
| Yang et al [6] (2) | Left adrenalectomy, left nephrectomy, and splenectomy | None | None | Died of disease |
| Wei et al [10] | Retroperitoneal laparoscopic resection of the left adrenal tumor | Liposomal doxorubicin and paclitaxel | None | Complete response |
| Livaditou et al [14] | Adrenalectomy with nephrectomy | None | None | Died 8 days after operation |
| Li et al [13] | Left laparoscopic adrenalectomy | N/A | N/A | Followup at 6 months after surgery |
| Imran et al [19] | Right adrenalectomy | Paclitaxel | N/A | Ongoing chemotherapy |
| Bosco et al [24] | Right adrenalectomy | N/A | N/A | No recurrence |
| Croitoru et al [25] | Left adrenalectomy | N/A | N/A | N/A |
| Ben-Izhak et al [26] | Laparotomy | N/A | Yes | Free of disease |
| Author | Management | Chemotherapy | Radiotherapy | Outcome |
|---|---|---|---|---|
| Lepoutre-Lussey et al [33] | Right laparoscopic adrenalectomy | Adriamycin/ ifosfamide | N/A | No recurrence |
| Pasqual et al [21] | Right adrenalectomy | None | N/A | No recurrence at 18 months after treatment |
| Rodríguez-Pinilla et al [11] | En bloc resection of tumor | None | None | No recurrence at 3 years after treatment |
| Criscuolo et al [12] | Laparoscopic ablation | Anthracycline | Yes | Died due to cachexia |
| Antao et al [8] | Left adrenalectomy, left nephrectomy, splenectomy, partial gastrectomy, and distal pancreatectomy | Paclitaxel, doxorubicin, carboplatin, etoposide- doxorubicin mitotane | N/A | Died due to disease progression and complications |
| Sung et al [22] | Right adrenalectomy and right nephrectomy | None | Yes | N/A |
N/A - data not available or no information in the article.
for endothelial markers [1]. Additionally, the fact that the tu- mor cells expressed FLI1 and INI1 confirmed that the tumor was an epithelioid angiosarcoma [29,30].
PAEA is a malignant and aggressive tumor with a poor prog- nosis. The 5-year survival rate for patients with PAEA ranges from 24% to 31% [31]. Wenig et al [32], who studied 9 patients with PAEA, revealed that 3 patients had disease-free stages for 6, 11, and 13 years following surgery. Two patients died of unrelated causes 2 and 4 months after surgery; they had not experienced any recurrence. Another patient was diagnosed with a diffuse spread of the disease in the abdomen 6 months after the surgery. Extensive surgery to remove the metastatic lesions was performed and chemotherapy was administered but the patient died of unidentified causes with no evidence of tumors. Three of the patients with metastatic adrenal epi- thelioid angiosarcoma died.
Recovery from PAEA has been reported in some cases. In the report by Bosco et al [24], the patient showed symptoms of paraneoplastic syndrome (anemia, fever, cough, and headache); however, no tumor recurrence was detected after surgery. In the report by Ben-Izhak et al [26], a patient with PAEA of the right adrenal gland reported anemia and abdominal pain 2 weeks after surgery due to hematoma and a residual tumor. However, the patient underwent a second operation and ra- diation therapy, and was subsequently cured.
There are currently no clear treatment guidelines for PAEA [6]. In addition, the positive effects of chemotherapy on PAEA are still debated [6]. The chemotherapy agents, adriamycin and ifos- famide, were administered to a patient with PAEA in a previous- ly reported case [33]. The patient responded well to treatment and exhibited no recurrence. In a study by Cornejo et al [34], 4 patients underwent surgery for tumor removal but did not receive chemotherapy. Two of them died from recurrence at 5 and 16 months after surgery, one showed recurrence of the disease but was still alive 19 months after surgery, and one was cured of the disease. Regarding the patient in the pres- ent case, the treatment consisted of surgical removal of the tumor, with recommended followup examinations 4 months later. Table 2 summarizes the cases of PAEA published in the literature with essential information including that of chemo- radiation therapy and outcomes of the patients.
Conclusions
We report a case of PAEA arising from an adrenal cortical ad- enoma with a favorable clinical course. PAEA may be misdi- agnosed radiologically and histologically as adrenal cortical carcinoma, metastatic carcinoma, or malignant melanoma. Immunohistochemical stains play a crucial role in establish- ing a definitive diagnosis of PAEA. Surgical removal of the tu- mor is the primary treatment, followed by close monitoring. However, early detection of the tumor is crucial for the pa- tient’s recovery.
Acknowledgments
The authors would like to acknowledge Ms. Naorn Sriwangdang for her assistance with the submission of the research pro- posal and thank Editage (www.editage.com) for English lan- guage editing.
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Ethics Statement
This case report was approved by the Faculty of Medicine, Chiang Mai University Human Research Ethics committee (Approval number: PAT-2565-09169). This research was car- ried out in accordance with Good Clinical Practice (ICH GCP).
Declaration of Figures’ Authenticity
All figures submitted have been created by the authors who confirm that the images are original with no duplication and have not been previously published in whole or in part.
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