Unusual Adrenal Cortical Tumor of Unknown Biologic Potential: A Nodule in a Nodule in a Nodule

2005 ◽  
Vol 8 (4) ◽  
pp. 483-488 ◽  
Author(s):  
Sarangarajan Ranganathan ◽  
Kecha Lynshue ◽  
Jennifer L. Hunt ◽  
Timothy Kane ◽  
Ronald Jaffe

Adrenocortical tumors are uncommon neoplasms in childhood. Most pediatric adrenal tumors are virilizing and carcinomas are more common than adenomas. Recent molecular data suggest an adenoma-to-carcinoma progression sequence in adrenal cortical neoplasms. We report a case of a 5-year-old boy who presented with virilizing symptoms secondary to an adrenal tumor that was resected laparoscopically. The bulk of the tumor was a large, yellow mass with typical features of an adrenal cortical adenoma. In the center was a well-circumscribed tan-brown nodule that was distinct from the adenoma and had oncocytic features. A third minute focus (3.0 mm) was noted that was not circumscribed or encapsulated, but showed marked pleomorphism and abundant mitoses, including atypical forms and increased Ki67 compared with the outer 2 nodules. Molecular analysis to assess the clonality and mutation rates of the 3 distinct areas showed only 2 genetic loci with allelic imbalances.

2002 ◽  
Vol 126 (12) ◽  
pp. 1530-1533 ◽  
Author(s):  
Tomislav Ivsic ◽  
Richard A. Komorowski ◽  
Gary S. Sudakoff ◽  
Stuart D. Wilson ◽  
Milton W. Datta

Abstract Adrenal tumors often present with clinical features that are specific and unique to their endocrine metabolism. When these features are in conflict with the pathologic appearance of the tumor, there can be great consternation for both the pathologist and the surgeon. In the case reported herein, an adrenalectomy was performed for clinical features of pheochromocytoma that on gross and histologic examination had the pathologic features of an adrenal cortical adenoma. Electron microscopy subsequently revealed that the tumor cells contained adrenalin-type granules, explaining the clinical outcome. It is crucial for both the surgeon and the surgical pathologist to be aware of this possibility when the clinical and pathologic features of an adrenal tumor are not congruent.


F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 675
Author(s):  
Dwiki Haryo Indrawan ◽  
Fauriski Febrian Prapiska ◽  
Syah Mirsya Warli ◽  
Bungaran Sihombing ◽  
Ginanda Putra Siregar

Adrenal gland masses could be classified into functional, malignant, or benign. An adrenal cortical adenoma is one of the most common incidentalomas found with either functional or non-functional type. Pheochromocytoma is a neural crest cell origin tumor associated with catecholamine production. A classic triad of headache, sudden episodic perspiration, and tachycardia marked a pheochromocytoma. We report three patients with adrenal tumors. First, a 52-year-old woman with complaints of pain in the left flank suggests a left kidney tumor. The patient has an increased blood pressure intraoperatively. Adrenal cortical adenoma was found postoperatively. The second case is an Indonesian male 27-year-old with pain in the upper right abdomen. Intraoperative, the patient also has an escalation in blood pressure. Antihypertensive drugs are also used in this patient. Postoperatively, a pathology result of pheochromocytoma was revealed from this patient. The third case, adrenal myelolipoma, was suspected in a 48-year-old male and underwent surgery because of tumor growth. Later, a histopathological examination revealed myelolipoma of the adrenal. Management of adrenal tumor should be done individually based on each patient. In the first and second cases, blood pressure was unstable intraoperatively and was managed using several drugs, and was stable at follow-up. In the third case was no hemodynamic problem. In the case of an adrenal tumor, management tailoring should be based on the individual patient.


2020 ◽  
Vol 63 (6) ◽  
pp. 527-535
Author(s):  
Donatella Serio ◽  
Giovanni Furnari ◽  
Yola Metti

AbstractIt was noted that Mediterranean specimens collected at different stations from around Sicily, Italy and referred to as Laurencia dendroidea (as Laurencia majuscula) were similar to the recently described species Laurenciella marilzae. Presented in this study are the results of an integrative approach using both morphology and molecular data (COI-5P + rbcL) to establish which taxon these specimens should be referred to. Molecular analyses show these specimens belong to Laurenciella, and strongly suggest they are within the species L. marilzae. Morphological examinations of these Mediterranean specimens were also detailed and found to support the conclusion that they belong to L. marilzae.


2020 ◽  
Vol 11 (4) ◽  
pp. 705-710
Author(s):  
Martin K. Walz ◽  
Klaus A. Metz ◽  
Sarah Theurer ◽  
Cathrin Myland ◽  
Pier F. Alesina ◽  
...  

AbstractThe morphological differentiation between benign and malignant adrenocortical tumors is an ongoing problem in diagnostic pathology. In recent decades the complex scoring systems have been widely used to calculate the probability of malignancy in adrenocortical tumors on the basis of a variety of histomorphological parameters. We herewith present a substantially simplified method to diagnose adrenocortical carcinoma by a single histomorphological parameter on a consecutive series of more than 800 adrenocortical tumors. Between January 2000 and May 2019, altogether 2305 adrenalectomies for of all types of diseases were removed, approximately 98% by minimally invasive approaches. After exclusion of pheochromocytomas, adrenal ganglioneuromas, adrenal metastases, Cushing’s disease related specimens, and Conn’s adenomas, the present series finally consisted of 837 adrenocortical tumors. All tumors were analyzed by experienced pathologists of a single institution using standard histopathological methods (Hematoxylin-Eosin and Ki67 stained sections). Clinical and histopathologic data were prospectively collected and retrospectively analyzed. Clinically, 385 patients had 420 functioning tumors (FT), and 417 had non-functioning adrenal tumors (NFT). The mean size of FT was 3.8 ± 1.4 cm (range 0.5–16 cm) and for NFT 4.5 ± 1.6 cm (range 1.5–18 cm). Histomorphologically, 32 adrenal tumors were classified as adrenocortical carcinoma (ACC; 3.8%). In all 32 cases (tumor size 9.1 ± 4.0 cm, range 3–18 cm), confluenting tumor necrosis could be demonstrated. The remaining 805 tumors (control group) completely lacked this highly reproducible single morphological feature. Ki67 levels above 10% were found in 31 of 32 ACCs and never in adrenocortical adenomas (ACA). With a mean follow-up of 8.2 years, 24 out of 32 patients primarily diagnosed as ACC developed distant metastases (75.0%), whereas all patients in the control group remained free of local or distant recurrence. We conclude that a single morphological parameter (confluenting tumor necrosis) is sufficient to predict a poor clinical course in adrenocortical tumors. The histomorphological diagnosis of this parameter is straightforward and highly reproducible.


2018 ◽  
Vol 104 (5) ◽  
pp. 1712-1724 ◽  
Author(s):  
Giada Poli ◽  
Carmen Ruggiero ◽  
Giulia Cantini ◽  
Letizia Canu ◽  
Gianna Baroni ◽  
...  

Abstract Context Novel tumor markers are urgently needed to better stratify adrenocortical cancer (ACC) patients and improve therapies for this aggressive neoplasm. Objective To assess the diagnostic and prognostic value of the actin-bundling protein fascin-1 (FSCN1) in adrenocortical tumors. Design, Setting and Participants A local series of 37 malignant/37 benign adrenocortical tumors at Careggi University Hospital and two independent validation ACC cohorts (Cochin, TCGA) from the European Network for the Study of Adrenal Tumors were studied. Main Outcome Measures FSCN1 expression was quantified by immunohistochemistry, Western blot and quantitative RT-PCR in ACC specimens; overall and disease-free survival associated with FSCN1 expression were assessed by Kaplan-Meier analysis and compared with that of Ki67 labeling index and tumor stage. Results Despite the low diagnostic power, in the Florence ACC series, FSCN1 immunohistochemical detection appeared as an independent prognostic factor, also refining results obtained with staging and Ki67 labeling index. The robust prognostic power of FSCN1 levels was further confirmed in two independent ACC cohorts. A positive correlation was found between FSCN1 and steroidogenic factor-1 (SF-1), with a substantially higher expression of both factors in ACCs at advanced stages and with at least one of the three Weiss score parameters associated with invasiveness. Moreover, we demonstrated FSCN1 role in promoting cell invasion in a human ACC cell line only in the case of increased SF-1 dosage. Conclusions These findings show that FSCN1 is a novel independent prognostic marker in ACC and may serve as a potential therapeutic target to block tumor spread.


1994 ◽  
Vol 346 (1317) ◽  
pp. 333-343 ◽  

High mutation rates are generally considered to be detrimental to the fitness of multicellular organisms because mutations untune finely tuned biological machinery. However, high mutation rates may be favoured by a need to evade an immune system that has been strongly stimulated to recognize those variants that reproduced earlier during the infection, hiv infections conform to this situation because they are characterized by large numbers of viruses that are continually breaking latency and large numbers that are actively replicating throughout a long period of infection. To be transmitted, HIVS are thus generally exposed to an immune system that has been activated to destroy them in response to prior viral replication in the individual. Increases in sexual contact should contribute to this predicament by favouring evolution toward relatively high rates of replication early during infection. Because rapid replication and high mutation rate probably contribute to rapid progression of infections to aids, the interplay of sexual activity, replication rate, and mutation rate helps explain why HIV-1 has only recently caused a lethal pandemic, even though molecular data suggest that it may have been present in humans for more than a century. This interplay also offers an explanation for geographic differences in progression to cancer found among infections due to the other major group of human retroviruses, human T-cell lymphotropic viruses (HTLV). Finally, it suggests ways in which we can use natural selection as a tool to control the aids pandemic and prevent similar pandemics from arising in the future.


Author(s):  
Yuri I. Kantor ◽  
Magalie Castelin ◽  
Alexander Fedosov ◽  
Philippe Bouchet

In the ancillariid genus Amalda, the shell is character rich and 96 described species are currently treated as valid. Based on shell morphology, several subspecies have been recognized within Amalda hilgendorfi, with a combined range extending at depths of 150–750 m from Japan to the South-West Pacific. A molecular analysis of 78 specimens from throughout this range shows both a weak geographical structuring and evidence of gene flow at the regional scale. We conclude that recognition of subspecies (richeri Kilburn & Bouchet, 1988, herlaari van Pel, 1989, and vezzaroi Cossignani, 2015) within A. hilgendorfi is not justified. By contrast, hilgendorfi-like specimens from the Mozambique Channel and New Caledonia are molecularly segregated, and so are here described as new, as Amalda miriky sp. nov. and A. cacao sp. nov., respectively. The New Caledonia Amalda montrouzieri complex is shown to include at least three molecularly separable species, including A. allaryi and A. alabaster sp. nov. Molecular data also confirm the validity of the New Caledonia endemics Amalda aureomarginata, A. fuscolingua, A. bellonarum, and A. coriolis. The existence of narrow range endemics suggests that the species limits of Amalda with broad distributions, extending, e.g., from Japan to Taiwan (A. hinomotoensis) or even Indonesia, the Strait of Malacca, Vietnam and the China Sea (A. mamillata) should be taken with caution.


2019 ◽  
Vol 7 ◽  
pp. 232470961987031 ◽  
Author(s):  
Sreedhar Adapa ◽  
Srikanth Naramala ◽  
Vijay Gayam ◽  
Frank Gavini ◽  
Hemant Dhingra ◽  
...  

Adrenal myelolipomas (AMLs) are rare benign adrenal tumors, containing adipose and hematopoietic tissue, a result of reticuloendothelial cell metaplasia. Incidence on autopsy has been reported from 0.08% to 0.4%. AMLs are generally considered nonsecretory. The functional aspect of adrenal incidentaloma should be evaluated. In this article, we report a case of a 40-year-old male, who presented with uncontrolled hypertension and renal failure, with imaging revealing an adrenal incidentaloma. He was started on dialysis for acute fluid overload, and workup for pheochromocytoma revealed an elevated serum norepinephrine level of 1181 pg/mL. Free metanephrine and normetanephrine levels were low when checked pre- and post-dialysis. Complete resection of the encapsulated right adrenal mass was performed. Pathology of the adrenal tumor demonstrates an 11.5 × 9.5 × 7.5 cm well-circumscribed, partially encapsulated proliferation of mature adipose tissue with admixed hemopoietic elements consistent with myelolipoma weighing 29.3 g. This case highlights the inclusion of a full metabolic workup for all adrenal incidentalomas, including AML.


2020 ◽  
Vol 34 (2) ◽  
pp. 113 ◽  
Author(s):  
Rafael Robles ◽  
Peter C. Dworschak ◽  
Darryl L. Felder ◽  
Gary C. B. Poore ◽  
Fernando L. Mantelatto

The axiidean families Callianassidae and Ctenochelidae, sometimes treated together as Callianassoidea, are shown to represent a monophyletic taxon. It comprises 265 accepted species in 74 genera, twice this number of species if fossil taxa are included. The higher taxonomy of the group has proved difficult and fluid. In a molecular phylogenetic approach, we inferred evolutionary relationships from a maximum-likelihood (ML) and Bayesian analysis of four genes, mitochondrial 16S rRNA and 12S rRNA along with nuclear histone H3 and 18S rRNA. Our sample consisted of 298 specimens representing 123 species plus two species each of Axiidae and Callianideidae serving as outgroups. This number represented about half of all known species, but included 26 species undescribed or not confidently identified, 9% of all known. In a parallel morphological approach, the published descriptions of all species were examined and detailed observations made on about two-thirds of the known fauna in museum collections. A DELTA (Description Language for Taxonomy), database of 135 characters was made for 195 putative species, 18 of which were undescribed. A PAUP analysis found small clades coincident with the terminal clades found in the molecular treatment. Bayesian analysis of a total-evidence dataset combined elements of both molecular and morphological analyses. Clades were interpreted as seven families and 53 genera. Seventeen new genera are required to reflect the molecular and morphological phylograms. Relationships between the families and genera inferred from the two analyses differed between the two strategies in spite of retrospective searches for morphological features supporting intermediate clades. The family Ctenochelidae was recovered in both analyses but the monophyly of Paragourretia was not supported by molecular data. The hitherto well recognised family Eucalliacidae was found to be polyphyletic in the molecular analysis, but the family and its genera were well defined by morphological synapomorphies. The phylogram for Callianassidae suggested the isolation of several species from the genera to which they had traditionally been assigned and necessitated 12 new generic names. The same was true for Callichiridae, with stronger ML than Bayesian support, and five new genera are proposed. Morphological data did not reliably reflect generic relationships inferred from the molecular analysis though they did diagnose terminal taxa treated as genera. We conclude that discrepancies between molecular and morphological analyses are due at least in part to missing sequences for key species, but no less to our inability to recognise unambiguously informative morphological synapomorphies. The ML analysis revealed the presence of at least 10 complexes wherein 2–4 cryptic species masquerade under single species names.


Zootaxa ◽  
2019 ◽  
Vol 4717 (1) ◽  
pp. 137-184 ◽  
Author(s):  
SAMUEL GEREMIAS DOS SANTOS COSTA ◽  
HANS KLOMPEN ◽  
LEOPOLDO FERREIRA DE OLIVEIRA BERNARDI ◽  
LUCIANA CARDOSO GONÇALVES ◽  
DANTE BATISTA RIBEIRO ◽  
...  

The life cycle of Parasitengona includes major morphological changes precluding an instar association based only on the morphology. This makes rearing and/or molecular data necessary to associate the heteromorphic instars. Most of the described species are known from either post larval instars or larva. Following a previous study on Palearctic Erythraeidae, in the present study the instar association was made through an integrative approach including rearing trials and molecular analysis of the cytochrome oxidase I (COI) gene with the Bayesian Generalized Mixed Yule Coalescent (bGMYC) algorithm for species delimitation. Two new cave dwelling Erythraeidae (Trombidiformes: Parasitengona) species are described Lasioerythraeus jessicae sp. nov. and Leptus sidorchukae sp. nov. including all active instars. Additionally, a complete description of the previously unknown adults of Charletonia rocciai Treat & Flechtmann, 1979 is provided with notes on the larva and deutonymph. We also demonstrate experimentally that Ch. rocciai larvae are not attached to the same individual host during the entire feeding stage. We discuss the presence of troglomorphisms in Le. sidorchukae sp. nov.; and the distribution of the species. 


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