scholarly journals Metastatic Adrenocortical Carcinoma Co-Secreting Multiple Steroid Hormones

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A142-A142
Author(s):  
Tien-Hao Lee ◽  
Robert Galagan
Keyword(s):  
Ct Scan ◽  
Steroid Hormones ◽  
Adrenocortical Carcinoma ◽  
Exploratory Laparotomy ◽  
Surgical Pathology ◽  
Renin Activity ◽  
Low Grade ◽  
Ki 67 ◽  
Left Diaphragm ◽  
Adrenal Nodule

Abstract Background: Adrenocortical carcinoma is a rare disease which may be complicated by co-secretion of multiple steroid hormones. Clinical Case: A 53- year-old female was discovered to have 1 cm left and 1.7 cm right adrenal nodules by Chest CT scan in 2004. She had a follow-up abdominal CT scan in 2011 revealing enlargement of the left adrenal mass to 5.7 cm and a stable 1.8 cm right adrenal nodule. A laparoscopic left adrenalectomy was performed in 2012 and the surgical pathology diagnosis was benign adrenal hyperplasia. In 5/2016 the patient developed left abdominal pain and a CT scan revealed a 1.7 cm mass in the left adrenalectomy surgical bed, a 1.4 cm nodular density adjacent to the left diaphragm and the stable 1.8 cm right adrenal nodule. 6/2016 lab tests: 24 hr urinary cortisol 15 ug/24 hr (<50 μg/24hr), aldosterone 8.7 ng/dL (<31 ng/dL), renin activity 0.7 ng/ml/hr (0.5–4 ng/mL/hr) and DHEA-S 94 ug/dL (32–240 μg/dL). A re-examination of the 2012 surgical pathology resulted in an addendum diagnosis of an adrenal cortical neoplasm of indeterminate malignant potential. In 1/2018 she underwent an exploratory laparotomy with surgical resection of the 1.7 cm mass in left paracolic gutter and biopsy of numerous small retro-peritoneal and multiple liver lesions. Pathology revealed metastatic adrenocortical carcinoma with low grade mitotic activity (3 mitoses per 10 HPF) and intermediate grade Ki-67 (15–25%). 5/2018 lab results: 1. aldosterone 20 ng/dL, 2. renin activity 0.2 ng/mL/hr and 3. testosterone 34 ng/dL (<75 ng/dL). Mitotane was started in 06/2018 but was discontinued in 9/2018 due to side effects. In 3/2020 she was hospitalized for generalized weakness and was discovered to be severely hypokalemic K+ 1.5 mmol/L (3.6–5.2 mmol/L) with an aldosterone of 300 ng/dL and renin activity of 0.1 ng/mL/hr. She was treated with IV KCl to correct her hypokalemia and was discharged on oral KCl 20 meq bid and spironolactone 50 mg bid. She was readmitted to the hospital on 10/12/2020 after a near-syncopal event and lab tests revealed a K+ of 1.4, aldosterone 508 ng/dL, renin activity 0.7 ng/mL/hr, AM cortisol 13.6 μg/dL (5–20 ug/dL), testosterone 161 ng/dL, and DHEA-S 377 ug/dL, indicating co-secretion of multiple steroid hormones. Her hypokalemia was treated with IV KCl and her spironolactone dose was increased to 100 mg bid. Conclusion: This case report details the rare occurrence of an adrenocortical carcinoma which was hormonally silent but eventually metastasized and became hormonally active, co-secreting multiple steroid hormones with a predominance of aldosterone. Serial adrenal hormone lab profiles are important for optimal management of patients with this disease.

Littoral Cell Hemangioma of the Spleen.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3953-3953
Author(s):  
Seema Naik ◽  
Farida Chaudhri ◽  
Stephen Yang
Keyword(s):  
Platelet Count ◽  
Ct Scan ◽  
White Male ◽  
Metastatic Tumor ◽  
Low Grade ◽  
Significant Past Medical History ◽  
Ki 67 ◽  
Primary Tumors ◽  
Littoral Cell ◽  
Red Pulp

Abstract Introduction: Splenic hemangiomas are rare, range from 0.03–14% in autopsy studies. The primary tumors of the spleen are benign and originate from the vascular endothelium and rarely from the lining cells of red pulp sinuses, giving rise to Littoral cell angiomas (LCA), first described by Falk et al in 1991. It usually is an incidental benign tumor presenting as mild to moderate splenic enlargement seen at any age in both sexes. Grossly it usually has multiple distinct nodules are spongy with dark bloody spaces ranging from 0.2 to 9.0 cm in diameter. Case Report: 65 years old white male patient presented with thrombocytopenia, platelet count 84000/cmm, Hemoglobin 11.8 and WBC 5500. He had no significant past medical history. Review of systems was noncontributory. There were no other physical findings. The patient had normal upper gastrointestinal series. Colonoscopy revealed tubular adenomatous polyp. CT scan chest revealed thoracic vertebral hemangioma. CT scan of abdomen showed enlarged spleen measuring 15cm axially with multiple low attenuation areas throughout the spleen. FNA of spleen showed spindle cells positive for factor VIII and CD31. The patient had normal bone marrow biopsy as well as cytogenetics. The patient underwent splenectomy which showed littoral cell angioma with heterogeneous population of lymphocytes, histiocytes, eosinophila, neutrophils, platelets, and large polylobated cells. The patient had marked clinical improvement with normallization of platelet count post splenectomy. Discussion: Littoral cell angiomas are seen as hypo echoic areas seen on ultrasonography and CT scan of abdomen. The natural history is benign and do not recur post splenectomy. Spontaneous rupture can occur in 25% of patients. On MRI, siderosis is seen due to hemophagocytosis by littoral cells. The lesions are of variable size and solitary or multinodular located in the red-pulp of the spleen. They are composed of anastomosing vascular channels with irregular lumina featuring cyst-like spaces. The LCA cells have a dual-endothelial/histiocytic characteristics, that react with F VIII Ag and are positive for CD68 antigen. Angiostatin inhibits angiogenesis and metastatic tumor growth. The mildly atypical cells, but not frankly malignant with low Ki 67 staining and diploid DNA histogram are in accordance with a low-grade malignancy. LCA has also been associated with synchronous malignancies such as lymphoma, colonic cancer, renal cancer, ovarian cancer, pancreatic cancer, seminoma and gastric leiomyosarcoma, a long-term follow-up for these patients is recommended.

scholarly journals MON-LB042 A Rare Case of Adrenocortical Carcinoma Arising From Ectopic Adrenal Tissue in the Mesentery

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Gemy M George ◽  
Soumya P Thumma ◽  
Lakshmi P Menon
Keyword(s):  
Ct Scan ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Adrenal Glands ◽  
Abdominal Mass ◽  
Cushing's Syndrome ◽  
Low Grade ◽  
Adrenal Tissue ◽  
Free Cortisol ◽  
Ectopic Adrenal Tissue

Abstract Background: ACTH independent Cushing’s syndrome usually arises from benign or malignant tumors of the adrenal gland. Ectopic adrenal tissue can undergo malignant transformation resulting in the development of adrenocortical carcinoma (ACC) with normal adrenal glands. We present a unique case of ACTH independent Cushing’s syndrome from a cortisol and androgen producing ACC arising from the mesentery. Clinical Case: A 72-year-old woman presented with a 6-month history of progressive weakness, lower extremity edema, worsening hypertension and uncontrolled DM. She had moon facies, hirsutism, multiple bruises, and proximal muscle weakness. Labs revealed hypokalemia (2.6 mmol/L, N 3.5 -5.1 nmol/L), elevated random cortisol (51.3 mcg/dL, Nl- 5-23), suppressed ACTH (<1 pg/mL, Nl-7.2-63.3) abnormal 8 mg dexamethasone suppression test (58.1 mcg/dL, N <1.8 mcg/dL), elevated DHEAS (538mcg/dL, N 10-90 mcg/dL), elevated testosterone (590.2 ng/dL, N <75 ng/dL) and elevated 11-deoxycortisol (4650 ng/dL N-<32 ng/dL). 24 hour urinary free cortisol was 2810mcg/mL (<45mcg/dL). MRI of pituitary was normal. CT scan of abdomen/pelvis showed normal bilateral adrenal glands and innumerable enhancing masses throughout the abdomen with the largest mass near the distal ileum and cecum. Biopsy of right lower abdominal mass revealed adrenocortical morphology with immunohistochemical staining positive for inhibin, synaptophysin and calretinin. Ki-67 index was 10-15%, suggestive of low-grade adrenocortical carcinoma. A CT scan done one and half years prior noted a 4.4 cm soft tissue mass in the right lower mesentery supporting origin of the tumor from the mesentery. Hypercortisolism was controlled with Metyrapone 250 mg BID. Mitotane 1000 mg bid was initiated but patient developed peritoneal carcinomatosis within 1 month. Conclusions: Our case is remarkable for the development of a metastatic ACC from an ectopic adrenal tissue with normal bilateral adrenal glands. Ectopic ACC is very rare with only a handful of cases reported in the literature. This is the first reported case of ACC arising from the mesentery. Ectopic adrenal tissue can be found close to the adrenal glands, along the path from gonads to adrenal glands or in association with the gonads. In the setting of ACTH independent Cushing’s syndrome with normal adrenal glands, physicians should direct their search to a functioning ectopic adrenocortical tissue. Concomitant DHEAS secretion suggests ectopic ACC. If surgery is not an option due to metastatic disease, a multidisciplinary approach should be adopted to control tumor growth and associated symptoms. In such cases, control of the hypercortisolemia can be achieved with adrenolytic medications such as Metyrapone, Ketoconazole or Mitotane. Adjuvant chemotherapy (Mitotane and combination of cytotoxic drugs) might be considered for metastatic ACC treatment.

scholarly journals Impact of sex in the prevalence and progression of glioblastomas: the role of gonadal steroid hormones

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Claudia Bello-Alvarez ◽  
Ignacio Camacho-Arroyo
Keyword(s):  
Cell Proliferation ◽  
Steroid Hormones ◽  
Physiological State ◽  
Migration And Invasion ◽  
Low Grade ◽  
Main Body ◽  
Protective Effects ◽  
Gonadal Steroid Hormones ◽  
Gonadal Steroid

Abstract Background As in other types of cancers, sex is an essential factor in the origin and progression of glioblastomas. Research in the field of endocrinology and cancer suggests that gonadal steroid hormones play an important role in the progression and prevalence of glioblastomas. In the present review, we aim to discuss the actions and mechanism triggered by gonadal steroid hormones in glioblastomas. Main body Glioblastoma is the most common malignant primary brain tumor. According to the epidemiological data, glioblastomas are more frequent in men than in women in a 1.6/1 proportion both in children and adults. This evidence, and the knowledge about sex influence over the prevalence of countless diseases, suggest that male gonadal steroid hormones, such as testosterone, promote glioblastomas growth. In contrast, a protective role of female gonadal steroid hormones (estradiol and progesterone) against glioblastomas has been questioned. Several pieces of evidence demonstrate a variety of effects induced by female and male gonadal steroid hormones in glioblastomas. Several studies indicate that pregnancy, a physiological state with the highest progesterone and estradiol levels, accelerates the progression of low-grade astrocytomas to glioblastomas and increases the symptoms associated with these tumors. In vitro studies have demonstrated that progesterone has a dual role in glioblastoma cells: physiological concentrations promote cell proliferation, migration, and invasion while very high doses (out physiological range) reduce cell proliferation and increases cell death. Conclusion Gonadal steroid hormones can stimulate the progression of glioblastomas through the increase in proliferation, migration, and invasion. However, the effects mentioned above depend on the concentrations of these hormones and the receptor involved in hormone actions. Estradiol and progesterone can exert promoter or protective effects while the role of testosterone has been always associated to glioblastomas progression.

scholarly journals Choledochal Cyst Coexisting with Gallbladder Carcinoma - An Uncommon Case Report

2017 ◽  
Vol 16 (4) ◽  
pp. 602-605
Author(s):  
Jahangir Hossain Bhuiyan ◽  
Mohibul Aziz ◽  
Omar Faruk ◽  
Mahbub Hasan
Keyword(s):  
Choledochal Cyst ◽  
Gallbladder Carcinoma ◽  
Medical Science ◽  
Rare Condition ◽  
Exploratory Laparotomy ◽  
Abdominal Ultrasound ◽  
Low Grade ◽  
College Hospital ◽  
Medical College ◽  
Operative Recovery

Choledochal Cyst is a relatively rare condition. Even rarer is a choledochal cyst in association with a gallbladder carcinoma. This study reports a rare case of choledochal cyst coexisting with gallbladder carcinoma in a Bangladeshi patient. A 35 year old lady presented at IBN Sina Medical College Hospital, Kallyanpur, Dhaka with the history of recurrent right upper quadrant abdominal pain from childhood, which became severe for last 4 days before admission. The pain was colicky in nature and radiated to the back. Episodes were associated with low-grade fever, anorexia as well as vomiting. The preoperative diagnosis was made by abdominal ultrasound and MRCP. Exploratory laparotomy, enbloc cholecystectomy with excision of the choledochal cyst and roux-en-Y hepaticojejunostomy was also done. Post operative recovery was uneventful. Patient was followed up for six months and no obvious complication was noticed. Early suspicion of this rare pancreato-billiary disease is important because surgical treatment is the only way to avoid the complications of the disease.Bangladesh Journal of Medical Science Vol.16(4) 2017 p.602-605

scholarly journals A Rare Cutaneous Adnexal Tumor: Malignant Proliferating Trichilemmal Tumor

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Omer Alici ◽  
Musa Kemal Keles ◽  
Alper Kurt
Keyword(s):  
Differential Diagnosis ◽  
Elderly Women ◽  
Low Grade ◽  
Outer Root Sheath ◽  
Ki 67 ◽  
Morphological Findings ◽  
Root Sheath ◽  
Adnexal Tumor ◽  
Proliferating Trichilemmal Tumor ◽  
Malignant Proliferating Trichilemmal Tumor

Proliferating trichilemmal tumors (PTTs) are neoplasms derived from the outer root sheath of the hair follicle. These tumors, which commonly affect the scalp of elderly women, rarely demonstrate malignant transformation. Although invasion of the tumors into neighboring tissues and being accompanied with anaplasia and necrosis are accepted as findings of malignancy, histological features may not always be sufficient to identify these tumors. The clinical behavior of the tumor may be incompatible with its histological characteristics. Squamous-cell carcinoma should certainly be considered in differential diagnosis because of its similarity in morphological appearance with PTT. Immunostaining for CD34, P53, and Ki-67 is a useful adjuvant diagnostic method that can be used in differential diagnosis aside from morphological findings. In this study, we aimed to present the case of a 52-year-old female patient with clinicopathological features. We reported a low-grade malignant proliferating trichilemmal tumor in this patient and detected no relapse or metastasis in a 24-month period of follow-up.

Adrenocortical Carcinoma With Concomitant Myelolipoma in a Patient With Hyperaldosteronism

2005 ◽  
Vol 129 (6) ◽  
pp. e144-e147 ◽  
Author(s):  
Xichun Sun ◽  
Alberto Ayala ◽  
Claudia Y. Castro
Keyword(s):  
Adrenocortical Carcinoma ◽  
Severe Hypertension ◽  
Serum Potassium Level ◽  
Venous Invasion ◽  
Ki 67 ◽  
Unilateral Adrenalectomy ◽  
Aldosterone Level ◽  
Serum Aldosterone ◽  
Mitotic Figures ◽  
Atypical Mitosis

Abstract We present a case of aldosterone-secreting adrenocortical carcinoma with concomitant myelolipoma. To the best of our knowledge, this is the first such reported case. The patient was a 43-year-old man with severe hypertension. Clinical workup revealed an increased serum aldosterone level, hypokalemia, and metabolic alkalosis, and a left adrenal mass was found on computed tomography. The patient underwent a unilateral adrenalectomy, which led to improvement in blood pressure, the serum potassium level, and aldosterone concentration. The tumor weighed 70 g and measured 5.0 cm. On microscopic examination, we found necrosis, focal cytologic atypia, diffuse eosinophilic cells comprising more than 75% of the tumor, 5 to 7 mitotic figures per 50 high-power fields, rare atypical mitosis, and venous invasion. At the periphery of the tumor but within the capsule, microscopic areas of myelolipoma were seen. Ki-67 staining was positive in 20% of the tumor cells. Although rare, aldosterone-secreting carcinoma associated with myelolipoma should be included in the differential diagnosis of adrenal gland masses.

scholarly journals Ancillary techniques on the evaluation of canine cutaneous mast cell tumors from Brazil

2016 ◽  
Vol 46 (10) ◽  
pp. 1804-1810 ◽  
Author(s):  
Mariana Martins Flores ◽  
Renata Dalcol Mazaro ◽  
Ingeborg Maria Langohr ◽  
Alma Roy ◽  
Keith Strother ◽  
...  
Keyword(s):  
Mast Cell ◽  
Pcr Amplification ◽  
The United States ◽  
Growth Fraction ◽  
Grading System ◽  
Low Grade ◽  
Ki 67 ◽  
Mast Cell Tumors ◽  
Cutaneous Mast Cell ◽  
Exon 11

ABSTRACT: The use of histologic classification by a 2-tier grading system only, immunohistochemistry (IHC) for KIT and Ki-67 and polymerase chain reaction (PCR) for internal tandem duplications (ITD) on exon 11 has improved the prognostication of canine cutaneous mast cell tumors (CCMTs) particularly in the United States. However, these techniques are not commonly used in most Brazilian laboratories. Likewise, no studies, to date, have investigated the occurrence of ITD in CCMTs from the country. Thus, this study tested the 2-tier grading system, the immunohistochemistry for KIT and Ki-67 and the PCR for exon 11 in a group of Brazilian CCMTs with the goal of investigating the applicability of these tests in a Brazilian laboratory. Of the 39 CCMTs, 69.2% (27/39) were identified as low-grade and 30.8% (12/39) as high-grade by a 2-tier grading system. All tumors had a KIT expression pattern II, and 30.6% (11/36) had a high growth fraction (Ki-67). PCR amplification was successful in four of the 11 tumors examined. Two of these (50%) were positive for ITD. This study highlights the importance of using auxiliary techniques in the CCMT evaluation, identifies limitations and confirms the applicability of these methods on a routine diagnostic basis in Brazil. Our results will help to improve the prognostication of CCMTs in Brazilian diagnostic laboratories, encouraging the use of supplementary methods.

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