A 46-year-Oldman with Gynaecomastia and Cirrhosis Hepatis Child Plug A. A case Report

Background: Gynecomastia is defined as the presence of excessive breast tissue in males, which can appear unilateral or bilateral. Bilateral prepubertal gynecomastia in the absence of endocrine abnormalities is extremely rare, with only a few cases in literature. Case Presentation: A 46-year-old man presented with a 2 years history of bilateral breast enlargement and pain. There is heartburn, shrinking testicles, and decreased sexual drive. Vital examination showed normal. BMI : 24. 0 kg/m². Head dan neck: normal. On the chest both breasts are enlarged as avocados, palpable time around areola, chewy contingency, press pain, galactorrhea negative. Lung and cast are normal. In the abdominal: hepar, lien and renal are not palpable, and ascites are not found. There is an atrophy of testicles. Previous history of hepatic cirrhosis with Esophageal Varicose Veins caused by Hepatitis B. Laboratory examination Estradiol 98.71 pg/mL (range: <62), Testosterone: 8.66 ng/mL (range: 3.0-10.6), LH5.53 mlU/mL, FSH 6.49 mlU/mL, Prolactin 5.08 ng/mL; Total T4 5.57 ng/dL , TSH 1.84 μIU/mL. SGOT U/L 53, SGPT 71 U/L, Hepatitis B (+), HBsAg Reactive S/CO 414.46; HBeAg: Non Reactive, HBV DNA Undetectable and Albumin 3.6 g/dL. Fibroscan: Cirrhosis Hepatis. Abdominal ultrasound: Cirrhosis Hepatis. Gastroscopy: Esophageal Varicose Veins (F1, LI, CB) + Pan Gastropathy. Conclusion: Reportedly A 46-year-old man presented with gynaecomastia bilateral with Cirrhosis Hepatis Child Plug A, given conservative treatment with tamoxiphen for 1 month

Estrogen-secreting adrenocortical tumor in a postmenopausal woman: a challenging diagnosis

Summary Adrenocortical carcinoma (ACC) is a malignant disorder with rapid evolution and severe prognosis in adults and most produce cortisol and androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, especially in women, tend to be larger and have worse prognosis compared with other types of ACCs. We report the case of a 58-year-old woman who presented with bilateral breast enlargement and postmenopausal genital bleeding. She presented high estradiol (818 pg/mL – 25 times above upper normal limit for postmenopausal women) and testosterone (158 ng/dL – 2 times above upper normal limit) levels and no suppression of cortisol after overnight 1 mg dexamethasone test (12.5 µg/dL; normal reference value: < 1.8 µg/dL). The patient had no clinical features of cortisol excess. MRI showed a 12 cm tumor in the right adrenal. Clinical findings of bilateral breast enlargement and postmenopausal genital bleeding with no signs of hypercortisolism associated with hormonal findings of elevated estradiol and testosterone levels would indicate either an ovarian etiology or an adrenal etiology; however, in the context of plasma cortisol levels non-suppressive after dexamethasone test and the confirmation of an adrenal tumor by MRI, the diagnosis of an adrenal tumor with mixed hormonal secretion was made. The patient underwent an open right adrenalectomy and pathological examination revealed an ACC with a Weiss’ score of 6. Estradiol and testosterone levels decreased to normal range soon after surgery. She was put on mitotane treatment as adjuvant therapy, but due to side effects, we were unable to up-titrate the dose and she never achieved serum mitotane dosage above the desired 14 µg/mL. The patient remained in good health without any local recurrence or metastasis until 5 years after surgery, when increased levels of estradiol (81 pg/mL – 2.5 times above upper normal limit) and testosterone (170 ng/dL – 2.1 times above upper normal limit) were detected. MRI revealed a retroperitoneal nodule measuring 1.8 × 1.2 cm. The pathological finding confirmed the recurrence of the estrogen-secreting ACC with a Weiss’ score of 6. After the second procedure, patient achieved normal estrogen and androgen serum levels and since then she has been followed for 3 years. The overall survival was 8 years after the diagnosis. In conclusion, although extremely rare, a diagnosis of an estrogen-secreting ACC should be considered as an etiology in postmenopausal women presenting with bilateral breast enlargement, genital bleeding and increased pure or prevailing estrogen secretion. Learning points Estrogen-secreting adrenocortical carcinomas are exceedingly rare in adults and account for 1−2% of adrenocortical carcinomas. Estrogen-secreting adrenal tumors can be present in females, but are even more rare, we found few cases described in the literature. In women, they present with precocious puberty or postmenopausal bleeding. Feminization in the context of an adrenal tumor is considered almost pathognomonic of malignancy. Feminizing ACCs tend to be larger and with worse prognosis compared with nonfeminizing ACCs.

Ovotesticular Disorder of Sex Development: An Unusual Presentation

Disorder of sex development is an inclusive term that refers to any problem where the genital organ is atypical in relation to chromosomes or gonads. Ovotesticular disorder of sex development, which is formerly known as “true hermaphroditism,” is the most rare form among all disorders of sex development in humans. It is characterized by the simultaneous presence of both ovarian and testicular tissues in the same individual and characteristically presents with ambiguous genitalia in neonates or infants. Herein, we present an unusual case of a 19-year-old individual with phenotypically nearly normal male genitalia who presented with the complaint of bilateral breast enlargement.

The Place of Reductive Surgery in the Management of Gestational Ulcerative Gigantomastia: A Case Report at Sourô Sanou Teaching Hospital

Introduction. Gestational gigantomastia is a rare benign disease of unknown cause. We report a case of bilateral gigantomastia in pregnancy in order to locate the place of reductive surgery in its care which is quite controversial. Case. A 25-year-old woman gravida 2 para 1 was referred for an exaggerated bilateral breast enlargement at 32-week gestation. The examination showed bilateral giant breasts with collateral venous circulation and trophic changes marked by the necrosis of the distal third of the mammary skin involving the nipple-areolar complex. She underwent a biopsy of the ulcerative breast tissue, and the histology report did not show a malignant cell. After active foetal lung maturation and induced delivery, a breast reductive surgery with nipple plasty was performed 21 days postpartum. The postoperative course was marked by a period of lymphangitis. The cosmetic and psychological result was satisfactory at 6 months and at 18 months. Conclusion. Gestational gigantomastia is a benign disease that can simulate carcinomatous mastitis. The breast reductive plasty keeps its place in our context.

Prepubertal gynaecomastia in a boy with Peutz-Jeghers syndrome: managing the aromatase overexpression

Background Gynaecomastia, although rarely related to testicular tumours, in boys with Peutz-Jeghers syndrome (PJS) usually occurs due to large-cell calcifying Sertoli cell tumour (LCCSCT). Case presentation A 4-year-old boy, with a genetic diagnosis of PJS, presented gynaecomastia since the age of 2, associated with increased height velocity (HV). He exhibited bilateral breast enlargement (Tanner-B4) and a testicular volume of 4 mL. Testicular ultrasound revealed multifocal microcalcifications in both testicles. A laboratory evaluation showed undetectable gonadotrophins, testosterone and oestrogen and inhibin A of 4.6 pg/mL (0.9–1.7). The boy was subjected to therapy with anastrozole. In the last follow-up, 2 years after the start of therapy, he experienced a less tense Tanner-B2 and a decrease in HV; serum inhibin A had become negative. Conclusions This is one of the most precocious PJS-related gynaecomastia treated with aromatase inhibitors (AIs) reported in the literature. Oestrogen levels, although under the detection limit, may be sufficient to stimulate breast tissue/growth plates. Inhibin A is a good marker of LCCSCT and an indicator of response to AIs.

An Uncommon Case of Bilateral Breast Enlargement Diagnosed as Tumoral Pseudoangiomatous Stromal Hyperplasia: Imaging and Pathological Findings

The incidence of reported pseudoangiomatous stromal hyperplasia (PASH), as well as the variability and severity of clinical presentations, is increasing in the literature. In parallel, several authors posit the need for an improved classification of PASH to avoid possible variables associated with this diagnosis. Here, we present a 25-year-old woman with PASH accompanied by severe bilateral and symmetrical breasts enlargement, highlighting an uncommon clinical presentation of PASH as much as the careful interdisciplinary review and correlation of histology and all available imaging studies to confirm the definitive diagnosis.