Breast Fibromatosis: Radiologic–Pathologic Correlation

Fibromatosis of the breast is a rare, benign locally infiltrative tumor without metastatic potential. Patients typically present with a painless, palpable, firm breast mass, which may be mobile or fixed to the pectoralis muscle. While some cases are related to familial mutations in the adenomatous polyposis coli (APC) gene, the majority are sporadic due to somatic mutations or prior injury to the breast tissue. On mammography, fibromatosis is typically seen as an irregular, dense, spiculated mass. US demonstrates a hypoechoic, irregular mass with indistinct margins. Fibromatosis is indistinguishable from breast cancer on imaging, and core biopsy is required for definitive diagnosis. Wide local excision is the historical standard for treatment; however, recurrence rates are high, and other emerging therapies are being explored. This article reviews the clinical features, imaging and histopathologic findings, along with brief overview of management.

Gigantic vulvar masses: a case series of benign large vulvar tumors

Benign vulvar masses are uncommon condition of the lower genital tract. To date, there is no recognized classification for benign tumors. Most of the vulvar tumors show no symptoms, unless large enough to be noted on self-examination. Presented are 3 cases of large vulvar masses. The first case is a Bartholin’s gland cyst in 56 years old, G5P4 (4014) with a 5-year history of gradually enlarging mass characterized as fleshy, approximately 16x14x12 cm, soft, non-tender located in the medial portion of the left labia majora. Grossly, the specimen consists of a unilocular cyst filled with serous fluid and had a smooth glistening inner capsule wall. The second case is a cellular angiofibroma in 61 years old, G0, who developed a fleshy mass, approximately 13x18x15 cm, soft, non-tender, attached to the left labia majora via a pedicle, which developed for 10 years. The specimen is described as an irregular mass with well circumscribed nodules with soft to rubbery, flesh cut surface. The third case is lipoblastoma-like tumor of the vulva in 31 years old, G2P2 (2002), who had a fleshy hypopigmented mass, approximately 15x12x10 cm, pendulous, soft, smooth, non-tender in the right inferolateral labia majora, which grew for 8 years. The specimen is characterized as an irregular mass partially overlain by skin with cut sections showing cream fatty lobulated surfaces. Benign vulvar masses may present similarly to one another hence careful and detailed assessment should be done. Histologic evaluation is critical in establishing an accurate diagnosis due to the fact that multiple diagnoses may have similar gross characteristics. Management is based on type and size of mass and symptomatology of patient.

Neonatal oral teratoma: A case report

Background: Teratomas of the oral cavity are rare and can pose danger to a patient's life in terms of obstruction to the aerodigestive tract. These are the germ cell tumors that arise from all three layers of primordial germ cells. Case Presentation: A 2-day-old female neonate presented with a mass protruding from the oral cavity since birth with no significant antenatal history. Examination revealed a large (8 cm), firm, and irregular mass protruding from the oral cavity extending up to the inner surface of the upper lip and onto the gums, associated with cleft palate. Complete excision of the mass was done and histopathology confirmed the diagnosis. There were no postoperative complications. Conclusion: Oral cavity teratoma is a rare tumor with a good prognosis especially in its mature form. Surgical excision with multidisciplinary collaboration is needed in order to avoid any complications.

Granulomatous mastitis − treatment options and our experience

Granulomatous mastitis (GM) is a rare benign inflammatory disease of the breast, first described by Kessler and Wolloch in 1972. Clinically, it can present as unilateral, sometimes painful, increasing breast resistance, or as a hard, irregular mass. Sonography is the most useful diagnostic method for GM evaluation. The only method for definitive diagnosis is the use of biopsy. In histological findings, GM is characterized by non-caseifying granulomas, often associated with microabscess and fistula formation. There is considerable heterogeneity in treatment options; this may explain the high recurrence rate which is close to 50%. Such a high recurrence rate is alarming and proves that current treatments are suboptimal. Two treatment options are discussed worldwide: conservative strategies involving drug therapy with corticosteroids versus a surgical approach involving partial or total mastectomy. All conservative treatment options are associated with a high risk of recurrence, and most patients require surgery in the end. Thorough excision of inflammatory tissue is crucial for successful treatment while negative surgical margins are associated with a low recurrence rate. The surgical approach to GM requires sufficient radicality and presumes knowledge in the field of reconstructive breast surgery, similarly to oncosurgical breast conservation operations. However, alternatives to GM treatment with oral steroids may be acceptable for patients concerned about surgery. This article presents six case reports of patients treated at our department.

Primary Orbital Chondromyxoid Fibroma: A Cause of Monosymptomatic Periocular Pain

Chondromyxoid fibroma (CMF) is a very rare entity, accounting for <1% of all bone tumours. So far, only 4 cases have been reported with the orbit as primary location. Here, we present a case of orbital CMF with periocular pain as the presenting symptom and as the only symptom when the tumour recurred after surgery. A 41-year-old man underwent a CT scan and later an MRI as part of evaluation for left periocular pain that had persisted for a year. Clinical examination was normal. CT and MRI revealed an irregular mass in the lateral part of the left orbital roof. The tumour was removed by curettage, and histopathological examination showed a CMF. The pain was absent for 3 months. A second MRI 5 months postoperatively revealed a recurrence, which was removed surgically, resulting in pain alleviation. In conclusion, CMF is a potential cause of periocular pain, and persistent periocular pain with normal clinical findings should warrant a CT or MRI scan.

Cardiac angiosarcoma in the right ventricle treated by surgical resection

Cardiac angiosarcoma is a rare malignant neoplasm, the gold standard treatment is surgical resection. Our patient, an 81-year old Japanese woman, was admitted to hospital after chest pain over a month-long period. Transthoracic echocardiography (TTE) showed a heterogeneous and irregular mass-like lesion measuring approximately 45 mm and arising from the right ventricular free wall. Transesophageal echocardiography showed the lesion had a mobile portion. Considering the possibility of malignancy and a high risk of embolism and obstruction, we performed surgical resection of the tumour. Histological and immunohistochemical findings led to diagnosis of cardiac angiosarcoma. One year after surgery, TTE and CT showed no evidence of recurrence of angiosarcoma.

Training Radiologists to Interpret Contrast-enhanced Mammography: Toward a Standardized Lexicon

Objective Using terms adapted from the BI-RADS Mammography and MRI lexicons, we trained radiologists to interpret contrast-enhanced mammography (CEM) and assessed reliability of their description and assessment. Methods A 60-minute presentation on CEM and terminology was reviewed independently by 21 breast imaging radiologist observers. For 21 CEM exams with 31 marked findings, observers recorded background parenchymal enhancement (BPE) (minimal, mild, moderate, marked), lesion type (oval/round or irregular mass, or non-mass enhancement), intensity of enhancement (none, weak, medium, strong), enhancement quality (none, homogeneous, heterogeneous, rim), and BI-RADS assessment category (2, 3, 4A, 4B, 4C, 5). “Expert” consensus of 3 other radiologists experienced in CEM was developed. Kappa statistic was used to assess agreement between radiologists and expert consensus, and between radiologists themselves, on imaging feature categories and final assessments. Reproducibility of specific feature descriptors was assessed as fraction of consensus-concordant responses. Results Radiologists demonstrated moderate agreement for BPE, (mean kappa, 0.43; range, 0.05–0.69), and lowest reproducibility for “minimal.” Agreement was substantial for lesion type (mean kappa, 0.70; range, 0.47–0.93), moderate for intensity of enhancement (mean kappa, 0.57; range, 0.44–0.76), and moderate for enhancement quality (mean kappa, 0.59; range, 0.20–0.78). Agreement on final assessment was fair (mean kappa, 0.26; range, 0.09–0.44), with BI-RADS category 3 the least reproducible. Decision to biopsy (BI-RADS 2–3 vs 4–5) showed moderate agreement with consensus (mean kappa, 0.54; range, −0.06–0.87). Conclusion With minimal training, agreement for description of CEM findings by breast imaging radiologists was comparable to other BI-RADS lexicons.

UNCOMMON ANORECTAL PATHOLOGY: ANORECTAL MELANOMA

Anorectal melanoma (AM) is a rare and aggressive tumor, characterized by a poor prognosis. It accounts for 0.4–1.6% of all melanomas, and only 1% of anorectal malignant tumors [1]. It is difficult to diagnose due to the hidden site and nonspecific symptoms, occurring usually at a late stage. The most common symptom is rectal bleeding. There are various histological variants of AM. There is currently no consensus of treatment the typical therapeutic approach remains surgical resection however, this is not associated with improved overall survival [2,3]. We present a case of a 52-year old male complaining of rectal bleeding and pain for about 4 months, which were attributed to hemorrhoids. Rectal examination revealed an irregular mass near the anal verge. Biopsies were taken for analysis, they were fixed in 10% formalin, paraffin embedded and routinely stained with Hematoxylin– Eosin. Immunohistochemical investigations were done by using antibodies against cytokeratin (CK), P40, HMB45 and Melan A.

Mammary and extramammary myofibroblastoma: multimodality imaging features with clinicopathologic correlation, management and outcomes in a series of 23 patients

Objective: The purpose of this study was to describe the imaging appearance, diagnosis, and management of mammary and extramammary myofibroblastoma (MFB) in a series of 23 patients. Methods and materials: Following institutional review board approval, cases were identified by searching for “myofibroblastoma” in radiology reports. Multimodality imaging and pathological features were assessed. Results: 23 cases of myofibroblastoma were identified in 15 males and 8 females. Most cases were in the breast (20/23, 87%), presenting as a palpable mass or discovered incidentally on mammography in females or chest CT in males. Extramammary MFB lesions (3/23, 13%) presented with symptoms related to mass effect. At imaging, MFB most often demonstrated an oval or irregular mass that was hypoechoic or heterogeneously echogenic with variable margins. MRI showed T2 hyperintensity, diffusion restriction, and plateau kinetics. Extramammary MFB appeared as an enhancing mass with variable fat content and T2 intensity. Conclusion: Here we describe imaging and clinicopathological features of mammary and extramammary myofibroblastoma. Advances in knowledge: Imaging description of this rare tumor is limited in the literature, and to date this is the largest case series describing the imaging findings.

The Diagnosis and Treatment of Orbital Solitary Fibrous Tumor

BackgroundSolitary fibrous tumor (SFT) is an uncommon spindle-cell tumor of mesenchymal origin, it commonly occurs within pleura, the occurrence of SFT in the orbit is very rare. The diagnosis of orbital SFT is challenging in clinical, and requires an integrated approach that includes specific clinical, histological, immunohistochemical, and even molecular findings.MethodsIn our retrospective study, we collected 13 patients with orbital SFT. All patients were diagnosed with orbital SFT by postoperative histopathological and immunohistochemical examination. Patient charts and medical records were reviewed for demographic information, relevant medical and family history, clinical presentation, radiological examination, histopathological and immunohistochemical examination, the treatment and prognosis.ResultsThis study included 7 males and 6 females. The age ranged from 11 to 78 years. 7 patients presented with predominant features of exophthalmos. 7 patients showed eyeball dislocation. 6 patients with motility disturbances. 9 patients had the palpable masses. 6 patients had the secondary lesions. On CT scan, there were 6 patients were located in the superomedial quadrant of the orbit, 3 patients were located in the inferomedial quadrant of the orbit. The morphology of lesions was solitary ovoid mass in 10 patients, and irregular mass in 3 patients. The CT value of the tumor was 22.8-64.4Hu, with the median of 45.9Hu. On MRI, 3 patients showed hypointense mixed signals on T1WI, 10 patients showed isointense mixed signal on T1WI. 3 patients showed hypointense mixed signals, 4 patients showed isointense mixed signals, 6 patients showed hyperintense signals on T2WI. There were 12 patients showed obviously enhancement, and we found patchy lesions which were no enhancement in the tumor. All patients were treated by surgery. Immunohistochemical analysis showed that the tumor cells exhibited diffuse immunoreactivity for CD34 and CD99 in all the patients. The lesions were positive for Bcl-2 in 11 patients. The lesions were positivity for Ki-67 <5% in one patient, 5%-10% in 10 patients, >10% in 2 patients. ConclusionsThe lesions are often outside the muscular cone, most commonly located at superomedial quadrant and inferomeddial quadrant of the orbit. The density of the lesions is uneven in most cases, the mean CT values of the tumors on CT scans are variable. The signal of lesions on orbital MRI is uncertainty, it is inheterogeneous enhancement, and the lamellar unenhanced regions can also be found in the lesion. The complete gross resection, and even more aggressive wide excision is preferred. Although the Ki-67 labelling index is very low, malignant forms with an increased propensity for local recurrence have been found. A longer follow-up is needed to determine the recurrence rate of the disease, and to identify the causes of recurrence.