Sibel Perçinel

Dendritic cell sarcomas/tumours of the breast: report of two cases

Extranodal follicular dendritic cell sarcoma/tumours (FDCS/Ts) and interdigitating dendritic cell sarcoma/tumours (IDCS/Ts) are rare neoplasms. We present two cases of FDCS/T and IDCS/T of the breast. The FDCS/T case (case 1) presented in a 31-year-old woman and the IDCS/T case (case 2) in a 67-year-old woman who both showed a firm lump in the left breast. The FDCS/T lesion superficially appeared as an anaplastic carcinoma and the IDCS/T was reminiscent of a spindle cell sarcomatoid carcinoma. Nevertheless both lesions were negative for keratins while case 1 displayed neoplastic cells strongly positive for CD21, vimentin and focally for CD68 and S-100 protein. The tumour cells of case 2 were positive for S-100, CD68 and CD45. In breast, an unusual keratin negative tumour composed predominantly of spindle cells arranged in fascicles, storiform pattern or whorls with a lymphoid rich stroma should raise suspicion for FDCS/Ts or IDCS/Ts. The distinction from malignant tumours with similar features is discussed.

Adenohibernoma of the breast

Dear Sir, Brown fat is mainly found in infants and children and gradually disappears from most sites of the body with increasing age. During adulthood, deposits of brown fat persist around kidneys, adrenal glands, and aorta and within the mediastinum and neck. Tumors composed of brown fat (hibernomas) are rare in breast [1, 2, 4, 5]. A rare case of hamartoma containing brown fat has been reported by Garijo et al. [5]. Adenohibernoma, hibernoma-containing glands, is also a very rare lesion as one case only has been described until now [2]. Here, we are reporting a second case. The patient, a 57-year-old lady, underwent excisional biopsy 2 cm across due to right breast lesion detected mammographically. The family history was unremarkable. Histologically, in addition to tubular carcinoma, an incidental lesion was detected nearby which had not been detected on mammograms. This latter lesion was well circumscribed and measuring 3 mm. It was formed partly by a fibroepithelial proliferation reminiscent of fibroadenoma with myxoid stroma (Fig. 1). Nuclear atypia and epithelial proliferation were not present. The remaining part of the nodular lesion showed dilated glands immersed within fat tissue, which showed uniform, normochromatic, and centrally located nuclei with vacuolated granular eosinophilic cytoplasms consistent with brown fat (Fig. 2). Accordingly, the vacuolated granular brown fat cells were positive with avidin–biotin complex peroxidase for S-100 protein (Dako, polyclonal, 1:1500; Fig. 3) and negative for wide spectrum keratin (Dako, MNF116, 1:200) and cluster of differentiation (CD)68 (Neomarkers, monoclonal, MAC387, 1:200). There was no residual lesion within the re-excision specimen which consisted of fibrofatty tissue measuring 4 cm. Seven lymph nodes from lower axillary level were reactive. There is no evidence of disease after a 6-year follow-up. Adenolipomas are lesions composed of glands immersed in adult classical adipous tissue and are closely associated with hamartomas. Both lesions are fibroglandular and are well circumscribed [3] and the amount of fat is by definition conspicuous in adenolipomas compared with hamartomas. Hamartomas are lesions that result from faulty development in an organ, composed of an abnormal mixture of normally occurring tissue elements or an abnormal proportion of a single element normally present at that site. Brown fat is not a normal constituent of adult breast tissue and in this respect adenohibernomas cannot be regarded as hamartomas. Damiani and Panarelli [2] suggested a hamartomatous origin from remnants of fetal brown adipose tissue or, alternatively, the possibility of de novo mixed proliferation showing brown fat differentiation in its mesenchymal component for adenohibernomas. In addition, the focus that the granular cells are associated to a fibroadenoma is a further proof that the adenohibernoma is not a hamartoma. This situation was favored by Fisher et al. [3] who believed that fibroadenomas and hamartomas were Virchows Arch (2008) 452:351–352 DOI 10.1007/s00428-007-0553-x

Benign Localized Fibrous Tumor of the Pleura: Report of 25 New Cases

OBJECTIVE Benign localized fibrous tumors (BLFT) of the pleura are very rare slow-growing neoplasms that generally have a favorable prognosis. The aim of this manuscript is to evaluate the predictors of outcome with the review of the literature in a series of 25 patients with BLFT. METHODS Between January 1985 and November 2009, 25 patients underwent an operation due to BLFT. Of these patients, 14 (56%) were male; mean age was 41.1 (25 to 64) years. All patients underwent thoracotomy. Left thoracotomy approach was used in 16 patients. The mass lesions were totally excised. The histopathological examinations were performed with hematoxylin-eosin and immunohistochemical staining methods. RESULTS Of the patients, 18 (72%) were symptomatic. Symptoms were cough in 36%, shortness of breath in 32%, and chest pain in 20% of the patients. One patient (4%) appeared to have some symptoms (pain and swelling of the joints) associated with pulmonary osteoarthropathy. Seven patients (28%) underwent an operation due to mass lesion detected at routine control visits. None of the patients had a history of exposure to asbestos. Radiological investigations revealed 16 (64%) mass lesions in the left. Of the lesions found on exploration, 5 (20%) were intrapulmonary localized lesion without pedicle and 20 were pedicled. Of the pedicled masses, 5 were connected to parietal pleura and 15 to visceral pleura and all were intrathoracic extrapulmonary localized lesions. Eight (32%) lesions connected to left lower lobe. Additionally, three pedicled lesions were located in the lung fissure. Pedicled lesions were totally excised together with their pedicles. Intraparenchymal mass lesions were resected using wedge resection. The diameter of the resected masses was ranging between 3 and 22 cm (mean: 8.7). Macroscopically, all were encapsulated with a homogeneous cut surface. Intraoperative mortality and morbidity was not observed. The average hospitalization duration for all patients was 8.6 days (5 to 12). The mean follow-up was 33.6 (9 to 142) months with no recurrence. CONCLUSIONS Benign localized fibrous tumors are uncommon and treated by surgical means. According to our data obtained from literature review, clinicians should be aware of recurrence possibility even after complete resection of benign localized fibrous tumor and the risk of malign transformation.

Early prophylactic thyroidectomy for RET mutation-positive MEN 2B.

Polyarthritis of JIA is diagnosed by clinical inflammatory findings at five or more joints within the first 6 months from onset of disease. The present patient initially had clinically apparent arthritis only at three joints without systemic disease or enthesitis, and was suspected of having oligoarthritis-onset JIA. The laboratory findings demonstrated extremely high levels of serum MMP-3 and HA and elevated ESR. Extremely high levels of serum MMP-3 indicated multi-joint involvement. As well, the levels of serum HA in patients with systemic arthritis or polyarthritis was significantly higher than those in patients with oligoarthritis. An elevated ESR was a predictor of disease extension and a destructive course. These facts suggested that the present patient had polyarthritis of JIA although, initially, clinical arthritis was seen in only three joints. This was supported by Ga scintigraphy, which is an established method for the detection of arthritis. Significant accumulation of Ga was detected along with whole articular surface in all of the present patient’s ankle, knee and shoulder joint regions, which was clearly different from the linear accumulation of Ga into epiphysis during the growing period in healthy children. Conclusively, ultrasound (US) is noninvasive, but has lower sensitivity and specificity for synovitis. MRI has higher sensitivity and specificity for synovitis, but is expensive for systemic scanning. Ga scintigraphy is very suitable for systemic scanning of synovitis, regardless of exposure to radiation. Gd-MRI confirmed synovitis of the multiple joints, indicating polyarthritis. Based on these laboratory and radiological findings, the patient was considered to be in an early stage of polyarthritis of JIA and was treated with PSL in addition to MTX and nonsteroidal anti-inflammatory drug (NSAID). Finally, polyarthritis needs early intervention because of its poor prognosis for the joints. Nevertheless, the diagnostic criteria require persistence of arthritis for >6 weeks, which often makes early diagnosis difficult. Ga scintigraphy is useful for the detection of early stage arthritis and is recommended particularly for such subclinical patients with high levels of hematological markers for arthritis.

The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?

Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old girl with CN and PS. Although some rare associations of renal and pulmonary lesions have been reported, this is the first case report in the English literature that shows the association of CN with PS.

The development of papillary serous carcinoma of the peritoneum subsequent to endometrial carcinoma: a case report and review of the literature

Metachronous papillary serous carcinoma of the peritoneum (PSCP) after endometrial carcinoma (EC) is an extremely rare condition. Only three patients have been reported in the English literature. We present the fourth patient who had a more aggressive and fatal clinical course. A 79-year-old multiparous woman complained of progressive abdominal pain and distension after 5 years, subsequent to total abdominal hysterectomy and bilateral salpingo-oophorectomy for endometrioid type EC. Serum CA-125 level being followed routinely rose above 500 IU/mL. Abdominal and pelvic computed tomography demonstrated ascites, omental thickening, and nodularity. Paracentesis showed malignant cells resembling papillary adenocarcinoma. Omentectomy and bilateral pelvic lymphadenectomy were performed as cytoreductive surgery. The histologic slides of the totally sampled ovaries obtained from the first operation were reexamined and the corresponding paraffin blocks were re-sectioned but no tumor was detected. The microscopic appearance of the tumor in the omentum differed from that of the previous EC. Immunohistochemically, while the tumor showed reactivity for low and high molecular weight cytokeratin (CK) cocktail, epithelial membrane antigen, CK7, CA-125, and Ber-EP4, the immunostains for calretinin, monoclonal carcinoembryonic antigen, and CK20 were negative. On the basis of these results and the criteria proposed by the Gynecologic Oncology Group, the tumor was diagnosed as metachronous PSCP developed after EC, which corresponded to stage IIIC according to FIGO criteria for ovarian carcinoma. The patient received two cycles of carboplatin and paclitaxel and died 2 months after the cytoreductive surgery

COLORECTAL INVOLVEMENT OF KAPOSI'S SARCOMA IN A HIV‐NEGATIVE CASE

A case of Kaposis sarcoma (KS) in a 70‐year‐old man who was using corticosteroid for the treatment of asthma is presented. KS lesions occurred in the skin, colon, and rectum. Macroscopic appearances of the lesions varied from polypoid, hemorrhagic mucosal nodules and ulcers to red macules in the mucosal plane to plaque‐like indurations of the wall. As the case was HIV negative, it is believed that KS developed due to corticosteroid‐induced immunosuppression.