Plasmacytoma of the breast. A report of two cases diagnosed by aspiration biopsy

BACKGROUND: Extramedullary plasmacytoma of the breast is an uncommon neoplasm, occurring either as a solitary tumor or as evidence of disseminated multiple myeloma. CASE: Two cases of plasmacytoma of the breast were diagnosed by fine needle aspiration cytology. Aspiration smears showed a dispersed population of plasmacytoid cells with eccentric nuclei, abundant cytoplasm and the characteristic paranuclear hof. CONCLUSION: The clinical, cytologic and immunophenotypic features of plasmacytoma are characteristic, and the importance of distinguishing these neoplasms from primary mammary tumors is important to avoid unnecessary surgery.     

Biopercular syndrome: report of two cases and literature review

The anterior opercular or biopercular syndrome is a cortical pseudobulbar palsy due to bilateral lesions of the anterior brain operculum. It is characterized by preservation of reflex function and automatic activity, without mental impairment. Two cases are reported herein and the relevant literature reviewed. The first case was a 73-year-old female with a history of a stroke occurring seven years previously, without sequelae in the interim. She presented with sudden loss of consciousness. The neurological examination showed a right facial central palsy and anarthria, with reflex acts such as smiling, blinking and yawning, not elicited by commands; she also had a right hemiparesis and walking impairment. A brain CT scan showed an old ischemic infarction in the region of the right medial cerebral artery. Because the right motor involvement did not correlate with the findings of the initial CT scan, another CT scan two days later showed an acute brain infarction in the vicinity of the left medial cerebral artery. The second case was an 8-year-old girl with mental retardation and impairment of verbal development, caused by of biopercular pachygyria. Facio-pharyngo-glosso-masticatory diplegia and volitional selective palsy of the oro-facial muscles was seen in both patients. The neuropsychological assessment showed cognitive, emotional and social interaction impairment in both cases -as part of the frontal convexity syndrome in the first case and of mental retardation in the second. The two patients had difficulty in mastication and swallowing. The prognosis for recovery of verbal capacity is poor, although generally most patients recover the ability to swallow.     

Behavioral disorders as unusual presentation of pediatric extraventricular neurocytoma: report on two cases and review of the literature

Background

Extraventricular neurocytomas (EVNs) are rare parenchymal brain tumors, distinct from central neurocytomas that are typically located within the supratentorial ventricular system. Seizures and headache represent the most common symptoms of extraventricular neurocytomas in the cerebral hemisphere both in adult and pediatric population.

Case presentation

We describe two cases of pediatric EVN with clinical onset characterized by behavioral and attention deficit/ hyperactivity disorders. The association between behavioral/attention disorders in childhood and the presence of a frontal neurocytoma has never been described before. Furthermore, inappropriate levels of inattention, hyperactivity and impulsivity are common among the neurobehavioral and developmental disorders in childhood. We reviewed 43 pediatric cases of extraventricular neurocytoma included in the PubMed database and their clinical presentation, and we never found this unusual relationship.

Conclusion

In childhood, the attention/hyperactivity disorders seem to be often over-diagnosed. When these deficits are more subtle and do not well-fit in a specific neurocognitive disorder, the clinicians should have a suspicion that they might mask the clinical features of a frontal lesion. This paper is focused on the clinical presentation of the extraventricular neurocytoma and the possible organic etiology of an attention and hyperactivity deficit.

     

Endobronchial lipoma: two cases and review of the literature.

Endobronchial lipoma is a benign tumour of the large bronchi occurring in middle-aged men. To the 38 successfully treated cases in the English literature a further 2 are added. The symptoms are those of obstructive pneumonitis mimicking bronchogenic carcinoma, and the result of delayed therapy may be bronchiectasis. Treatment includes local resection through a bronchoscope or a bronchotomy incision, or removal, if necessary, of the obstructed lobe or lung at thoracotomy. Smoking may be important in the pathogenesis of this tumour.     

Epididymal sarcoidosis: a report of two cases and a review of the literature

Two cases of epididymal sarcoidosis, presenting as scrotal masses, are described. Biopsies of the epididymis and scalene nodes established the diagnosis. The literature of epididymal sarcoid and its differential diagnosis is discussed.     

滤泡树突细胞肉瘤二例报道并文献复习

目的探讨滤泡树突细胞肉瘤（FDCS）的临床表现、病理形态、免疫组化、生物学行为和预后,提高对该病的认识。方法对2例滤泡树突细胞肉瘤进行报道,并结合临床资料、肿瘤大体及镜下特征、免疫组化标记结果及预后和国内外文献进行分析。结果2例FDCS,1例发生于颈部淋巴结,另1例发生于肠系膜,镜检肿瘤由梭形及卵圆形细胞组成,呈束状、漩涡状、编织状排列;免疫组化显示肿瘤细胞CD21、CD23和CD35阳性;患者预后不良。结论滤泡树突细胞肉瘤是一种罕见且容易误诊的低度恶性肿瘤,组织化学和免疫组化染色有助于该肿瘤的诊断及鉴别诊断,减少误诊。     

Primary malignant melanoma of the nasal cavity: report of two cases and review of the literature

Primary malignant melanomas of the nasal cavity are rare, as only 400 cases have been reported to date. The present paper describes two cases recently seen in Caucasian women. The authors point out the difficult clinical diagnosis, as the symptoms are rather aspecific. From the histopathological point of view, diagnosis is easy in the melanotic cases while can show interpretating problems in the amelanotic ones, when melanoma is almost indistinguishable from other malignant neoplasms. A correlation between histological grading and prognosis was not detected, as both cases showed local recurrences within one year after surgery although they were, respectively, of epithelioid and undifferentiated type. While surgery appears to be the choice treatment of the primary lesion, the treatment of cervical metastasis is still disputable. On the whole, most authors think that the role played by radio- and mainly chemo-therapy is still limited and that cervical adenopathies should be treated by a simple lympho-adenectomy rather than by a neck dissection.     

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.     

Left atrial myxoma: report of six cases and review of the literature.

Six left atrial myxomas were detected at one hospital in a 15-month period. These tumours are not as rare as was once thought and they frequently cause systemic problems. The diagnosis may easily be missed, but echocardiography is a simple way of establishing the diagnosis. Excision of the tumour usually results in marked symptomatic improvement.     

Coronary artery-bronchial artery fistulas: report of two Dutch cases with a review of the literature

Background

Coronary bronchial artery fistulas (CBFs) are rare anomalies, which may be isolated or associated with other disorders.

Materials and methods

Two adult patients with CBFs are described and a PubMed search was performed using the keywords “coronary bronchial artery fistulas” in the period from 2008 to 2013.

Results

Twenty-seven reviewed subjects resulting in a total of 31 fistulas were collected. Asymptomatic presentation was reported in 5 subjects (19 %), chest pain (n = 17) was frequently present followed by haemoptysis (n = 7) and dyspnoea (n = 5). Concomitant disorders were bronchiectasis (44 %), diabetes (33 %) and hypertension (28 %). Multimodality and single-modality diagnostic strategies were applied in 56 % and 44 %, respectively. The origin of the CBFs was the left circumflex artery in 61 %, the right coronary artery in 36 % and the left anterior descending artery in 3 %. Management was conservative (22 %), surgical ligation (11 %), percutaneous transcatheter embolisation (30 %), awaiting lung transplantation (7 %) or not reported (30 %).

Conclusions

CBFs may remain clinically silent, or present with chest pain or haemoptysis. CBFs are commonly associated with bronchiectasis and usually require a multimodality approach to be diagnosed. Several treatment strategies are available. This report presents two adult cases with CBFs and a review of the literature.     

Paraneoplastic cerebellar degeneration as a presentation of breast cancer – a case report and review of the literature

Paraneoplastic cerebellar degeneration is part of a rare spectrum of neurological syndromes whereby gynaecological, lung or breast cancers present primarily with neurological manifestations. The presence of onconeural antibodies and PET scanning help in the challenging diagnosis of these conditions but despite the treatment of the primary cancer, the prognosis for the neurological symptoms is poor.     

Juvenile breast hypertrophy: report of a familial pattern and review of the literature.

Juvenile hypertrophy of the breast is a relatively rare condition rapidly leading to gigantomastia in peripubertal females. The pathology is limited to the breast, with otherwise normal growth and development. We have reported the first familial pattern seen in this unusual condition. Ablation of the breast is always required. We favor reduction mammaplasty to avoid breast implants, despite the need for additional procedures secondary to recurrent hypertrophy. Our second patient provides support for this approach, demonstrating amelioration in breast growth despite the initial period of rapid regrowth.     

Radiation-induced breast angiosarcoma: report of two patients after accelerated partial breast irradiation (APBI) and review of the literature

BackgroundAngiosarcoma may rarely complicate radiotherapy of breast cancer. This so-called radiation-induced angiosarcoma (RIAS) occurs in less than 0.3% of patients that underwent breast conservation surgeries, usually years after completion of radiotherapy.Case presentationwe introduce two cases of invasive ductal carcinoma who underwent lumpectomy and accelerated partial breast irradiation (APBI) as an alternative protocol to whole breast irradiation (WBI). They received adjuvant partial breast radiotherapy on tumor cavity for a total dose of 38.5 Gy in 10 fractions in 5 days using 3D-external-beam RT. In both cases, RIAS occurred eight years after radiotherapy, in the sub-cicatricial area in one patient and outside the irradiated area in the other one. They both underwent radical surgery and chemotherapy was performed in one patient.DiscussionThe underlying mechanism for development of RIAS is not well known, but its incidence seems to be increasing. RIAS after partial breast irradiation is very rare and has been reported in two cases so far. As it may be suggested in case 2, it is still a matter of debate if the risk of radiation-induced sarcoma is radiation-dose dependent. Although mastectomy is considered as a standard treatment, choice of treatment should be made according to the patient’s specifications.ConclusionThere are very few studies in the literature that report RIAS after APBI. Present study is the only one reporting two cases after the external 3D technique APBI. Prognosis of RIAS remains poor. Only a careful evaluation in a multidisciplinary context can offer to the patients the best result in terms of local control and survival.     

原发性乳腺弥漫性大B细胞淋巴瘤5例并文献复习

目的探讨原发性乳腺弥漫性大B细胞淋巴瘤(primary breast diffuse large B-cell lymphoma,PB-DLBCL)的临床病理学特点、诊断及鉴别诊断、治疗及预后。方法采用HE染色和免疫组织化学SP法分析5例PB-DLBCL患者的临床表现、病理学及免疫表型特征,并复习相关文献。结果 5例均为女性患者,发病年龄48~70岁,中位年龄59.2岁,均为单侧乳腺肿块,其中左乳3例,右乳2例。镜下见乳腺正常结构被破坏,成片的中等偏大的肿瘤细胞弥漫浸润乳腺小叶、导管周围、间质及周围脂肪组织。根据免疫组织化学表型,4例为非生发中心型,1例为生发中心型;Ki67增殖指数为60%~90%;根据 Ann Arbor 分期标准,5例均为II E期。病例随访时间截止至2018年6月,随访期内,有2例复发,分别于7和19个月后死亡;2例健在,分别已存活12和72个月;另外一例失访。结论 PB-DLBCL是一种少见的恶性淋巴瘤,临床表现为单侧乳腺无痛性包块进行性增大,以右侧多见。确诊主要依靠病理活检及免疫表型,免疫表型以非生发中心为主,以化疗联合放疗等综合治疗方案为宜。     

Hypertrichosis cubiti: two new cases and a review of the literature

Two cases of hypertrichosis cubiti in combination with short stature, facial dysmorphias and retarded development are reported with a review of the literature. Hypertrichosis cubiti, the hairy elbows syndrome, consists of a localized form of long vellus hair on the extensor surfaces of the distal third of the upper arm and the proximal third of the forearm bilaterally. It can be associated with short stature and other physical abnormalities. The mode of inheritance has not been established yet; an autosomal recessive as well as an autosomal dominant inheritance trait are postulated.