Hypothalamic hamartoma: the role of surgery

A hypothalamic hamartoma associated with true precocious puberty in a 7-month-old girl is hereby reported. Hormonal studies disclosed elevated serum levels of luteinizing hormone (LH) and follicle stimulating hormone, both of which responded well to LH-releasing hormone stimulation. Following a subtotal removal of the tumor, the clinical manifestations of precocious puberty as well as associated endocrinological abnormalities returned to normal. The role of surgery for this lesion, which appears to be safe when a planned microsurgical course is employed, is discussed.     

Breast Hamartoma: a Case Report

Breast hamartoma is a benign and rare tumour consisting of glandular fat and fibrous tissue. A 36-year old woman was admitted to our clinic, complaining of a mass in her right breast since two months. Ultrasonographic and mammographic examinations revealed a 10 cm mass diagnosed as fibroadenolipoma. Total excision of the mass was performed without any complication. Histopathological examination revealed fibroadenomatous hamartoma.     

A Giant Mammary Hamartoma in a Young Breast Cancer Patient

BackgroundHamartomas of the breast are rare benign tumors. Pre- and also postoperative differentiation from other benign or even malignant tumors is challenging.Case PresentationA 36-year-old female presented with a giant tumor of the left breast. The patient had suffered from an early breast cancer of the contralateral right breast the year before, which was treated with breast-conserving therapy, radiation, and endocrine therapy ever since. The hamartoma was classified as BI-RADS 2 in mammography and BI-RADS 4 in ultrasound. On clinical examination, a tumor of nearly 15 cm in size led to an abstruse deformity of the breast and the nipple-areola complex. We found an indolent, grand bulging tumor with an elastic texture directly beneath the skin. A biopsy that had been performed before was compatible with the suspected hamartoma. Because of the remaining diagnostic uncertainties after contralateral breast cancer and the progressive malformation of the left breast, a tumor extirpation utilizing a reduction mammaplasty was performed without complications. Subsequent genetic analyses excluded a loss of PTEN in this patient.ConclusionWe presented the rare case of a 36-year-old woman with a history of breast cancer and a 700-g breast hamartoma. The preoperative and even the postoperative specification of a hamartoma remains challenging, and associations with genetic alterations should be considered.     

A rare cause of vaginal bleeding in a 7-month-old female infant

Ovarian sex cord stromal tumor (OSCST), sclerosing type, is an extremely rare ovarian tumor. Sex hormone production by OSCST can result in isosexual or heterosexual precocious puberty in younger patients. We present a case of a 7-month-old female infant found to have a sclerosing-type OSCST that presented with vaginal bleeding and very prominent vellus hair at the mons pubis. This represents the youngest patient reported in the literature with this subset of OSCST.     

A rare cause of an abdominal mass

A 57-year-old male presented with right upper abdominal lump since 3 months. He was diagnosed to have a hepatic artery aneurysm. He was investigated thoroughly but no cause was found. He was subjected to endovascular embolisation of the aneurysm using endovascular coils. Subsequently the aneurysm was completely occluded. Hepatic artery aneurysms are very rare among all visceral aneurysms. We report this rare case of hepatic artery aneurysm presenting as an abdominal lump. This case highlights the importance of early diagnosis and management of this rare entity as a rupture may be catastrophic.     

Primary malignant melanoma: a rare cause of mediastinal mass

A case of primary malignant melanoma in the mediastinum presenting as recurrent laryngeal nerve palsy is reported. Tissue biopsy at mediastinotomy yielded a diagnosis of malignant melanoma. The mass was fixed to the left aspect of the trachea and to the upper border of the left main bronchus and could not be removed surgically. Further extensive clinical and radiological investigations revealed no evidence of tumor elsewhere in the body.     

肾错构瘤的诊断与治疗(附31例报告)

目的：探讨肾错构瘤的诊断与治疗方法。方法：回顾性分析31例肾错构瘤的临床资料，其中30例行B超检查；25例行CT扫描；7例行MRI扫描；15例行IVP检查；2例行DSA检查。27例行手术治疗，其中7例术前诊断明确且肿瘤直径远大于4cm，行肾切除术；7例因肾破裂出血而争诊手术切除病肾；2例肾破裂出血者病情稳定，于次日明确诊断后进行手术切除；1例术前不能完全明确诊断，术中快速切片确定为肾错构瘤后行肿瘤剜除术；另4例作随访观察。结果：所有患者均经病理检查诊断为肾错构瘤，未发现有肿瘤恶变者。27例手术患者术后病情稳定。结论：CT和MRI是肾错构瘤最好的影像学诊断方法。对肿瘤直径＞4cm且逐渐增大伴明显症状者，应积极进行手术治疗，手术治疗应尽量保留有功能的肾组织。     

Haemangioblastoma: a rare cause of a cerebellar mass in the elderly

In the elderly, cerebellar lesions are commonly metastatic tumours with poor prognosis. We describe two octogenarians who presented with obstructive hydrocephalus, secondary to posterior fossa tumours that, on computed tomography, were thought to be cerebellar metastases. Both lesions were excised and the histology proved them to be cerebellar haemangioblastomas, primary benign tumours of the posterior fossa, which are rare in the elderly.     

Idiopathic azygos vein aneurysm: a rare cause of mediastinal mass

Venous aneurysm of the azygos arch is a very rare cause of mediastinal mass and is usually an incidental finding on chest radiography. Nowadays the diagnosis is made by non-invasive tests such as thoracic CT scanning and/or magnetic resonance imaging. The case is described of an asymptomatic woman in whom a mediastinal mass due to an azygos vein aneurysm was diagnosed by non-invasive procedures, the aetiology of which, in all probability, was idiopathic.     

A super long-acting LH-RH analogue induces regression of hypothalamic hamartoma associated with precocious puberty

Summary We treated a 1-year-old female with a hypothalamic hamartoma and precocious puberty with leuprolide acetate depot, a super long-acting hormone-releasing hormone analogue (Tap-144-SR; [D-Leu⁶-[des-Gly¹⁰-NH₂]-LH-RH ethylamide acetate). The infant's major symptoms were genital bleeding and gynaecomastia. The LH-RH analogue (30 g/kg) was injected subcutaneously once every 4 weeks. Clinical and laboratory manifestations of precocious puberty showed marked improvement. A follow-up after 16 months of treatment, the size of the tumour decreased significantly and remained unchanged for 2 years of further follow-up. To the best of our knowledge, this is the first hypothalamic hamartoma case in whom a decrease of tumour size under treatment with LH-RH analogue has been documented. But, because diagnosis of hamartoma is only based on neuroradiological and not on histological examinations, the possibility of a gangliocytoma cannot be excluded with certainty.     

Abdominoscrotal hydrocele: a rare cause of a cystic abdominal mass in children

Abdominoscrotal hydrocele is a rare cause of cystic lower abdominal masses in children. A timely diagnosis is important because early surgical treatment is curative. Graded compression during the ultrasound evaluation might better demonstrate the communication between the abdominal and scrotal components of the fluid collection, thereby avoiding the use of ionizing radiation and/or the sedation required with alternative imaging modalities.     

罕见6岁女童卵巢Leydig细胞瘤:临床特点、免疫组织化学和电镜研究

目的对1例罕见6岁女童卵巢Leydig细胞瘤患者的临床特点、肿瘤组织的免疫组织化学和电镜结果、治疗及随访情况进行总结分析,提高对此疾病的认识。方法收集患者的临床资料、生化检查、免疫组织化学和电镜结果。结果 (1)雄激素增多引起男性化表现,外院误诊为"先天性肾上腺皮质增生症",予氢化可的松治疗6个月,睾酮水平未降低。(2)生化检查:血FSH 1.3 U/L,LH 1.1 U/L,雌二醇(E2)246pmol/L(N55pmol/L),孕酮(P)22.1nmol/L(N 1.3~5.7nmol/L),睾酮(T)16.1nmol/L(N 0.3~1.6nmol/L),硫酸脱氢表雄酮(DHEAS)2.34μmol/L(N2.46μmol/L),17-羟孕酮(17-OHP)169.5nmol/L(N9nmol/L)。(3)影像学:右附件区可见约4cm囊实性包块。骨龄9~10岁。(4)病理和免疫组织化学:a-抑制素(inhibin)(+),Calretinin(+),符合Leydig细胞瘤。(5)电镜结果:可见同心圆样异常形态线粒体。(6)随诊:术后T和P下降到正常水平,痤疮消失。结论 P和17-OHP水平升高并非先天性肾上腺皮质增生症的特异性改变,卵巢Leydig细胞瘤也可分泌性激素的中间产物。同心圆样异常形态线粒体可能是Leydig细胞瘤的特异性电镜下改变。本例国内年龄最小的卵巢Leydig细胞瘤,拓宽了我们对此疾病发病年龄的认识。     

Brucella orchitis: a rare cause of testicular mass: report of a case

Brucellosis is a multiorgan infectious disease. The genitourinary system is affected in 2–20% of the cases and the most common form is orchitis. Rarely, patients may present with a testicular mass and it must be distinguished from malignant processes. We report brucellar orchitis, a rare cause of testicular mass, in a 22-year-old man. We described the clinicopathological features of this rare entity and reviewed the literature.     

Multicystic biliary hamartoma: A report of a rare entity and a review of the literature

INTRODUCTION

Multicystic biliary hamartoma is a rare liver tumor that was first described in 2005. Only nine cases are reported in the literature and all of them originate from Eastern patient populations, specifically Japan and Korea.

PRESENTATION OF CASE

Herein we report the occurrence of the tenth multicystic biliary hamartoma reported to date, arising in a Caucasian American woman initially presenting with abdominal pain. At 4.7 cm this is the second largest tumor reported to date and the only one arising in a Western patient population.

DISCUSSION

The patient underwent multimodality imaging and the tumor was biopsied preoperatively, but the diagnosis remained unclear. An extended right hepatectomy was performed for resection of her tumor, and the tumor was definitively diagnosed based on the surgically resected specimen. As all nine of the previously reported cases also underwent resection, the natural history of this lesion remains unknown. The lack of both recurrence and tumor spread in the previously reported cases indicates that this may be a benign lesion not requiring surgical resection unless symptomatic.

CONCLUSION

Multicystic biliary hamartoma is an extremely rare tumor. Increased awareness of the radiologic and pathologic features will likely lead to the diagnoses of further cases in both Western and Eastern populations and could potentially assist with preoperative diagnosis. The natural history and optimal management of this tumor remain uncertain.     

Osteosarcoma invading adrenal gland: rare cause of adrenal mass

Adrenal gland metastasis from osteogenic sarcoma is extremely rare and is an atypical location for metastasis. Vascular hepatic exclusion techniques and the use of venovenous bypass with cardiac surgical techniques may be required to resect large adrenal masses that have invaded the inferior vena cava to achieve curative resection. The use of newer chemotherapeutic agents and aggressive surgical resection have prolonged the survival of patients with osteosarcoma.     

Polycystic ovaries, precocious puberty and acquired hypothyroidism: The Van Wyk and Grumbach syndrome

Long-standing acquired hypothyroidism can rarely be associated with precocious puberty and polycystic ovaries. The authors report such a case, which responded to the simple replacement of thyroid hormone. It is important to look for hypothyroidism in girls with ovarian masses and precocious puberty to avoid surgery on the ovaries.