Adrenal incidentaloma (AI) poses a diagnostic challenge in part due to its rarity, whereas radiological studies are the cornerstone for diagnosis [10]. Most of AIs are benign tumors, but a careful evaluation is required to rule out malignancy and functioning adenomas. Primary adrenal angiosarcoma and non-Hodgkin’s lymphoma are uncommon adrenal lesions; therefore, diagnosis and management are still a matter of debate till now [10,11,12].
Primary adrenal angiosarcoma is challenging to the clinician because of its scarcity [11]. Besides, the lesion is usually masked by concomitant necrosis and hemorrhage adding more difficulty in diagnosis. In a recent case reports, the incidence of adrenal angiosarcoma is more common among males, especially in the fifth and sixth decades of life [12, 13]. Likewise, our patient’s age and gender were matched with previously documented data. The clinical presentations are variable; some cases are asymptomatic, and others can show non-specific complaints as abdominal pain, weight loss, and fever [12, 13]. According to our findings, the patient was asymptomatic with an incidentally discovered adrenal mass during periodic follow-up. Grossly, the tumor may be predominantly cystic. Microscopically, adrenal angiosarcomas are frequently characterized by epithelioid appearance lacking the vasoformative patterns. These tumors typically stain positive for cytokeratin, an epithelial tumor marker, which can be seen in metastatic epithelial tumors or other mesenchymal neoplasms [3]. Cells are large and round with prominent nucleoli, while nuclei may appear vesicular [14]. Pathological examination for the excised adrenal mass in our case showed typically the previous reported pathological findings. Alternatively, accurate diagnosis by imaging is quite challenging, as there are no pathognomonic findings [3]. CECT images demonstrate heterogeneous low attenuation suggesting tumor necrosis. Whereas hyper attenuation suggests hemorrhage or calcification, postcontrast images may reveal heterogeneous enhancement and areas of necrosis [13]. CECT of the abdomen in our case showed large ill-defined soft tissue mass arising from the body and lateral limb of left suprarenal gland, and the mass exhibited heterogeneous post-contrast enhancement with areas of cystic degeneration and necrosis.
There is no doubt that adrenalectomy serves both diagnostic and therapeutic purposes for angiosarcoma. Almost all cases could be managed by adrenalectomy alone. On the other hand, postadrenalectomy treatment is still controversial. A multi-modal treatment approach was previously adopted and included postoperative doxorubicin-based chemotherapeutic regimens and adjuvant radiation therapy (XRT) [12]. Fleutra et al. discussed forty reported cases of primary adrenal angiosarcoma managed by different approaches of treatment. Twenty-two patients (55%) were managed by adrenalectomy only without concomitant adjuvant or neoadjuvant therapies. Of them, five patients (22.7%) were manifested by either local recurrence or distant metastasis at median (range) 21 (6–24) months, and eight patients (36.4%) were disease-free at follow-up at median (range) 59 (6–144) months. The remaining patients (40.9%) either died postoperatively or had not follow-up data [11]. In our report, the patient was managed by wide surgical excision only without adjuvant treatment. After 36 months, the patient was alive and disease-free.
Primary adrenal lymphoma is presented commonly in the elderly with a mean age of 62 years at presentation except in some rare reports [15]. The adrenal glands are involved in 24% of patients with multi-organ lymphoma [16]. Isolated unilateral primary adrenal lymphoma is very rare and constitutes 1% of extra-nodal lymphoma [16, 17]. The most common subtype found in the adrenal glands is diffuse large B-cell lymphoma (DLBCL) [16]. Flank or abdominal pain and fatigue are considered the most presenting symptoms, and only 1% of tumors were detected incidentally. Associated skin hyperpigmentation, organomegaly, and lymphadenopathy were presented in 27%, 15%, and 7%, respectively [7]. By MRI, primary adrenal lymphoma is characterized by isointense or hypo intense lesions in T1-weighted images and hyperintense lesions in T2-weighted images [18]. On diffusion-weighted imaging (DWI), lymphomas usually generate restricted diffusion and high signal intensity on DWI due to high cellularity of the tumor [19]. Prognostic factors including age, adrenal insufficiency, and tumor size have a significant impact on treatment outcomes and survival. Our case diagnosed as of non-germinal center origin with negative staining for CD-10 and BCL-6. Adjuvant chemotherapy is given to prevent disease recurrence (CHOP/CHOP-like) and regimens are the most commonly administered chemotherapy protocols to treat the primary adrenal lymphoma [20]. Also, the prognosis has been slightly improved with the recent use of rituximab as a new chemotherapeutic agent [16, 21]. Although a median survival of nearly 3 months was previously reported [21], the most recent data suggest disease-free survival of 12 months [16]. Our case was a 63-year-old female presented with an incidentally diagnosed isolated unilateral adrenal lesion with no signs of immune deficiency (excluding HIV infection with also negative staining for HHV8). Heterogeneous hyperintense signal intensity at T2WI was a characteristic sign in MRI. Postadrenalectomy, adjuvant six cycles of chemotherapy (CHOP protocol) were given to the patient. After six months of follow-up, the patient was disease-free.
