Primary amyloid tumor of the breast. Case report and literature review

An example of localized amyloid deposition in the breast of an elderly woman is reported. Fine needle aspiration biopsy yielded clumps of amorphous material, which stained violet with the May-Grünwald-Giemsa technique. The amyloid appeared to be of the AA type on histochemical analysis. The lesion was clinically associated, probably coincidentally, with a stage II carcinoma of the cervix. The nature and classification of amyloid is briefly reviewed, and the role of fine needle aspiration cytology in its diagnosis is discussed.     

Sertoli-Leydig cell tumor - a rare androgen secreting ovarian tumor in postmenopausal women. Case report and review of literature

Sertoli-Leydig cell tumors (SLCT) constitute only 1-0.5% of all primary ovarian neoplasms. We report a SLCT in a postmenopausal woman aged 69 years. The physical examination revealed severe hirsutism. Basal hormonal evaluation showed high plasma testosterone and estradiol values, with suppressed plasma gonadotropins. Computer tomograph scan revealed a right ovarian tumor mass of 4,3/3 cm, confirming an androgen secreting ovarian tumor. The histopathological and immunocytochemical examination established the diagnosis of well differentiated Sertoli-Leydig cell tumor. The tumor was positive for cytokeratin KL 1 and S-100 protein and, in isolated tumor cells, positive for alpha-fetoprotein. Postsurgical evolution was favorable; controls after 6 months and 3,5 years showed marked reduction of hirsutism, normal plasma testosterone values and gonadotropins in normal postmenopausal range. We discuss the complex aspects of etiology and pathogenesis, the clinical and hormonal settings, the role of immunocytochemical markers in diagnosis, as well as the therapy and the prognostic features of this ovarian tumor.     

Gastric Burkitt's lymphoma. Case report and review of the literature

A case of gastric Burkitt's lymphoma with pancreatic infiltration in a 15-year-old boy is reported. Local lymph nodes were not involved. Review of the literature disclosed that the reported cases of Burkitt's lymphoma were localized in the intestine. The origin, evolution and prognosis of gastrointestinal lymphoma are briefly discussed.     

Ring chromosome 17. Case report and review of the literature.

A 46,XX,r(17) karyotype was observed in a 9-year-old infant with short stature, moderate mental retardation but without other physical abnormality. Eight cases with an r(17) have since been reported: 4 can be compared with our patient, one was detected by amniocentesis, and 3 have Miller-Dieker syndrome. Submicroscopic deletions in the subband p13.3 are probably the cause of Miller-Dieker syndrome. They are present in some cases of r(17) but, in others, this short arm region is entirely preserved.     

Ring chromosome 9. Case report and review of the literature

We report on a girl with ring chromosome 9, and review the 9 other cases of the literature. The main signs of this de novo chromosomal anomaly are: severe microcephaly, growth and psychomotor retardations, and heart malformations. Infectious complications occurs often. We found a decreased level of leucocyte interferon.     

Pheochromocytoma associated with adrenocortical adenoma: case report and literature review

The case of a 60-year-old woman with pheochromocytoma and concomitant adrenocortical adenoma in the same gland is presented. She complained of episodic headache, palpitation, nausea, vomiting and sweating. Physical examination revealed that the patient has generalized obesity, wet skin and paroxysmal hypertension, but no signs of Cushing's syndrome. Elevated levels of urinary noradrenaline, adrenaline and total metanephrine were sequentially observed. In addition, urinary 17-OHCS was also slightly elevated, but plasma cortisol was normal and suppressed after oral administration of 0.5 mg of dexamethasone. Abdominal echography and CT scanning demonstrated a left adrenal tumor, which took up both 131I-meta-iodobenzylguanidine and 75Se-scintadoren in the same region. A left adrenalectomy was performed and the tumor was found to consist of two parts, pheochromocytoma (2.5 X 2.5 X 2.5 cm) and cortical adenoma (2.5 X 3 X 5 cm). A total of 23 reported cases showing evidence of hyperfunction of the adrenal cortex and the medulla were noted. So far as we know, this patient was the second case of pheochromocytoma with adrenocortical adenoma in Japan.     

Carcinoid tumor metastatic to breast diagnosed by fine needle aspiration. Case report and literature review

Fine needle aspiration (FNA) biopsy was used to study a mass in the left breast in a patient with a previous history of an ileal carcinoid tumor and later lymph node metastases who presented with bilateral palpable breast masses. The FNA specimens showed the lesion to be a carcinoid tumor. The metastatic nature of the lesion was proven by positive restaining of FNA smears by both the Sevier-Munger technique (demonstrating abundant argyrophilic cytoplasmic granules) and the Fontana-Masson method (showing argentaffin cytoplasmic granules). The distinction between primary and metastatic carcinoid tumors of the breast is discussed, as is their origin and their differentiation from other malignancies of the breast.     

Necrosis of a Hürthle cell tumor of the thyroid following fine needle aspiration. Case report and literature review

A Hürthle cell tumor of the thyroid gland resected ten days after fine needle aspiration (FNA) showed extensive necrosis accompanied by a small area of hemorrhage. Although no vascular thrombosis was noted in the histologic specimen, it is possible that the necrosis was the result of compromised vascular supply due to the FNA.     

Stewart-Treves syndrome: Case report and literature review

Lymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prognosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, including axillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account for approximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema. Lymphatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developing STS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema. Chronic lymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillary nodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction in the incidence of chronic lymphedema from 40% to 4%. The use of sentinel lymph node biopsy technique is also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STS decreased significantly with improved surgical and radiation techniques. The overall prognosis of STS patients is very poor. Only early radical surgical removal, including amputation or disarticulation of the affected limb, or wide excision at an early stage offers the greatest chance of long-term survival. Only a few case reports and series with a small number of patients with lymphangiosarcoma can be found in the literature. We present a case report of the first diagnosed STS at our department in an effort to highlight the need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.     

Primary Hodgkin's disease of the lung. Case report and review of the literature

A patient with primary Hodgkin's disease of the lung is described. Special features of this case were alcohol-induced chest pain as the main presenting clinical symptom and the documentation of the evolution of the pulmonary mass by serial X-rays. Complete remission was achieved by lobectomy and subsequent MOPP-therapy. Since then the patient has been in unmaintained remission for 36 months.     

Cytopathologic features of adenoid cystic carcinoma. Case report and literature review

A case of primary bronchial adenoid cystic carcinoma in a 40-year-old white female is reported. Cytologic findings in a bronchial wash specimen obtained at the time of bronchoscopic examination are described, with an emphasis on correlation with the histologic features of the tumor. The literature is briefly reviewed with respect to the occurrence of primary adenoid cystic carcinoma in the bronchial tree.     

Phenotypic variability of the cat eye syndrome. Case report and review of the literature.

We present a male infant with preauricular skin tags and pits, downslanting palpebral fissures, hypertelorism, ectopic anus, hypospadias, and hypoplastic left heart syndrome. The clinical features in our patient show phenotypic overlap with the cat eye syndrome, as illustrated by the review of 105 reported cases. Cytogenetic analysis revealed a supernumerary marker chromosome, which was identified by microdissection and fluorescence in situ hybridization as an isodicentric chromosome 22(pter --> q11.2::q11.2 --> pter). It was proved with probes specific for the cat eye syndrome critical region that this region was present in quadruplicate in the propositus. We conclude that CES is characterized by large phenotypic variability, ranging from near normal to severe malformations, as reflected in the neurodevelopmental outcome. Preauricular skin tags and/or pits are the most consistent features, and suggest the presence of a supernumerary bisatellited marker chromosome 22 derived from duplication of the CES critical region.     

Saksenaea vasiformis infections: Case report and literature review

Since the first human infection by Saksenaea vasiformis in 1976 another 26 cases have been reported. Here is a report of a new case which involved an Ecuadorian adolescent who suffered serious burns after a car accident. It developed as a localized cutaneous infection which was successfully treated with surgical debridement and amphotericin B. This is the second report of this infection from South America and the third involving a burn patient. The previously reported 27 cases are reviewed.     

Stereotactic ablative radiotherapy for bone metastasis of gastrointestinal stromal tumor: Case report and review of the literature

BackgroundGastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. These tumors are rare and only make bone metastases at a rate of 5%.Case summaryA 31-year-old male with a GIST presented with solitary bone metastasis at the right iliac bone. We performed stereotactic ablative radiotherapy (SABR) and achieved excellent local control. Herein, our case is presented, and a short review of the literature is carried out.ConclusionSABR should be considered as a treatment option in GIST with bone metastasis.     

Solitary fibrous tumor of the kidney: a case report and review of the literature

A solitary fibrous tumor (SFT) is an unusual spindle cell neoplasm that usually occurs in the pleura but has recently been described in diverse extrapleural sites. Urogenital localization is rare, and only 19 cases of SFT of the kidney have been described. We report a case of a large SFT clinically thought to be renal cell carcinoma arising in the kidney of a 70-year-old man. The tumor was well circumscribed and composed of a mixture of spindle cells and dense collagenous bands, with areas of necrosis or cystic changes noted macroscopically and microscopically. Immunohistochemical studies revealed reactivity for CD34, CD99, and Bcl-2 protein, with no staining for keratin, S-100 protein, or muscle markers, confirming the diagnosis of SFT. This tumor is benign in up to 90% of cases. The immunohistochemical study is the key to diagnosis.     

Primary retroperitoneal mucinous cystadenoma of borderline malignancy in a male patient. Case report and review of the literature

The spleen is an infrequent site for metastatic lesions, and solitary splenic metastases from squamous cell carcinoma of the esophagus are very rare: only 4 cases have been reported thus far. These lesions are whitish nodules that are macroscopically and radiologically similar to primary splenic lymphomas. We report a case of metachronous splenic metastases from esophageal cancer and multiple splenic abscesses, which developed nine months after apparently curative esophagectomy without adjuvant chemotherapy. The patient underwent splenectomy dissection followed by adjuvant chemotherapy, but liver and skin metastases developed, and the patient died 9 months later.