Aggressive angiomyxoma: a second case of metastasis with patient's death

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient. We document this case and report a similar one, found in the literature, of a postmenopausal woman with pulmonary and mediastinic metastases. These cases may expand the current concepts of potential behavior of aggressive angiomyxoma.     

Multifocal aggressive angiomyxoma: a case report

A case of aggressive angiomyxoma in a 25 year old woman is presented. The patient was admitted to hospital with a history of hesitancy of micturation and pain in the right iliac fossa. She was found to have a left labial mass, which was clinically diagnosed to be a Bartholin gland cyst. A pelvic ultrasound revealed an additional mass in the right paravesical region. At surgery, two distinct masses were removed, one from the right perivesical space and the other from the left labium. Both masses were rubbery, white, and gelatinous and showed similar histopathology findings of thick and thin walled vascular channels set in a loose myxoid stroma. A diagnosis of multifocal aggressive angiomyxoma was made. This is the first reported case of aggressive angiomyxoma occurring as two distinct masses in one patient.     

Endobronchial metastasis of thyroid follicular carcinoma

Well-differentiated forms of thyroid cancer, including follicular carcinoma, usually have good prognoses. But they are also known to metastasize to the bones, lungs and central nervous system. Endobronchial metastasis is exceptionally rare. In this paper, we report on a patient with endobronchial metastasis of follicular thyroid carcinoma. A 77-year-old male patient presented to our hospital with hemoptysis and a growing mass over the right clavicula. Computerized tomography (CT) of the chest revealed multiple lung metastases. Flexible bronchoscopy revealed a fragile polypoid mass of 1.5 cm, 6 cm distal to the vocal cords. Biopsy from the supraclavicular mass was consistent with follicular thyroid carcinoma. Taking into account the advanced nature of the disease as well as the general condition and age of the patient, aggressive treatment modalities were not considered in the management. Palliation for hemoptysis was attained by external radiotherapy. After radiotherapy, hemoptysis did not recur, and the patient was discharged. Although endobronchial metastasis of thyroid follicular carcinoma is very rare, the presence of endobronchial metastasis may be life threatening due to massive hemoptysis, and such a lesion must be suspected in any cancer patients presenting with hemoptysis.     

Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling

Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that has high propensity for local recurrence. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in adult women in the reproductive age. Considering its locally aggressive nature, appropriate management and long-term follow-up is necessary. We describe a case of a 22-year-old young pregnant patient presenting with a large pedunculated swelling on the left labia majora.     

Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity

AIMS: Aggressive angiomyxoma of pelvic parts is a distinctive soft tissue tumour that chiefly involves the vulvar and perineal region of female patients. Several previous reports have demonstrated oestrogen receptor (ER) and/or progesterone receptor (PR) positivity in this neoplasm. The aim of this study was to confirm whether ER and/or PR positivity is present in aggressive angiomyxoma. We also wished to ascertain whether positivity may be found in the stromal cells of normal vulval skin and in other lesions at this site that can cause diagnostic confusion with aggressive angiomyxoma. METHODS: Five aggressive angiomyxomas in female patients and one involving male pelvic soft parts were stained immunohistochemically with antibodies against ER and PR. Other samples studied were normal vulval skin (n = 7), fibroepithelial polyps of vulva (n = 7), vulval smooth muscle neoplasms (n = 5), vulval nerve sheath tumours (n = 2), vaginal angiomyofibroblastoma (n = 1), and pelvic myxoma (n = 1). Nuclear staining was classified as negative, weak, moderate, or strong and the proportion of positively staining cells was categorised as 0, < 10%, 10-50%, or > 50%. RESULTS: All five cases of aggressive angiomyxoma in female patients were positive for ER (two with weak intensity involving < 10% of cells and three with moderate intensity involving 10-50% of cells) and four of five cases were strongly positive for PR in > 50% of cells. The other case was negative for PR. There was no staining with antibodies to ER or PR in the single male patient with aggressive angiomyxoma. Other samples exhibiting positivity of the stromal cells for either ER or PR were normal vulval skin (five of seven, ER; two of seven, PR), fibroepithelial polyps (four of seven, ER; five of seven, PR), smooth muscle neoplasms (three of five, ER; four of five, PR), nerve sheath tumours (one of two, ER; one of two, PR), angiomyofibroblastoma (one of one, ER; one of one, PR), and pelvic myxoma (one of one, PR). CONCLUSIONS: All cases of aggressive angiomyxoma of pelvic soft parts in female patients exhibited positivity for ER and/or PR. Because of its propensity to occur in female patients during the reproductive years, it is possible that aggressive angiomyxoma is a hormonally responsive neoplasm. However, dermal fibroblasts in normal vulval skin and stromal cells in a variety of vulval lesions can also be positive. ER or PR immunoreactivity cannot be used to distinguish aggressive angiomyxoma and its histological mimics.     

Anaplastic Thyroid Carcinoma with Rhabdomyoblastic Differentiation: A Case Report with a Good Clinical Outcome

Anaplastic thyroid carcinoma is a rare and highly malignant disease. Usually, this type of tumor is irresectable, and almost all patients die within 1 year after diagnosis. We present a case of anaplastic thyroid carcinoma with rhabdomyoblastic differentiation and good therapeutic outcome. A 76-year-old female was referred to our hospital with a growing, painless neck mass which existed for 3 months. Biopsy of the mass showed microscopically and immunohistochemically a follicular (Hürthle cell) carcinoma of the thyroid, dedifferentiated to an anaplastic carcinoma with divergent differentiation along rhabdomyoblastic cell lines. The patient underwent a total thyroidectomy with additional lymph node dissection, followed by two episodes of 150 mC I¹³¹ therapy and external radiotherapy. After a follow-up of 3 years, the patient still remains in a good condition without any signs of recurrent disease. Other cases of rhabdomyoblastic and rhabdoid anaplastic thyroid carcinomas have been described. Both types of carcinoma are associated with an aggressive clinical course. In our case, the patient is still alive without evidence of disease 3 years after primary therapy. The good clinical outcome of our patient suggests that surgical resection, radiotherapy, and I-131 ablation therapy with curative intent seems to be an adequate treatment option in patients with anaplastic thyroid carcinoma with rhabdoid and rhabdomyoblastic differentiation.     

Palliative Radiation in Primary Squamous Cell Carcinoma of Thyroid: A Rare Case Report

Primary squamous cell carcinoma of the thyroid is an extremely rare neoplasm with aggressive behavior. Until date, only around 60 cases have been reported in the literature. Primary treatment of the patient is radical surgery. With optimum treatment survival is not more than 6 months in this aggressive malignancy. However in our patient surgery it was not possible because of unresectability of the mass due to encroachment of major vessels. Hence, we have delivered radiotherapy alone, with which effective palliation could be achieved and patient is leading a good quality-of-life for last 1 year.     

Malignant hidradenoma: a rare sweat gland tumour

Malignant hidradenoma is a rare sweat gland carcinoma, which can have an aggressive course with recurrence and/or metastasis. A case is reported, in an elderly male. The tumour had a histologic similarity to its benign counterpart, but exhibited additional features of infiltrative growth pattern and invasion of adjacent tissue.     

Desmoplastic fibroma of bone with extensive cartilaginous metaplasia

Desmoplastic fibroma of bone is a rare tumor demonstrating the same histologic and biologic features of its soft tissue counterpart, aggressive fibromatosis. We report the second case of desmoplastic fibroma of bone with extensive chondroid metaplasia. The tumor arose in the left ischium of a 51-year-old male, with extension into adjacent musculature as a pseudoencapsulated mass. The infiltrating growth and quality of the fibrous component are characteristic of desmoplastic fibroma, and in addition, abrupt transitions into bland hyalin cartilage were frequent. Discriminating features of this lesion from other bone tumors capable of biphasic expression of fibrous and chondroid elements are discussed.     

Synovial sarcoma of the neck masquerading as a malignant second branchial cleft cyst

Synovial sarcoma is an uncommon, aggressive malignant tumor of the soft tissues primarily involving the extremities of young adults. Head and neck synovial sarcoma is rare, and its diagnosis and therapy are still challenging.We report a case of a young patient with synovial sarcoma, clinically masquerading as cystic mass of the neck and malignant second branchial cleft cyst. The pathological diagnosis of the sarcoma was confirmed by a multimodality diagnostic protocol, including histological, immunohistochemical and molecular genetic analysis. The patient underwent complete surgical excision followed by postoperative radiotherapy and recovered well.     

Carcinoid tumor arising in a tailgut cyst of the anorectal junction with distant metastasis: a case report and review of the literature

Tailgut cyst is a rare congenital presacral lesion and is believed to arise from the persistent remnants of the postanal gut. Malignancy occurring in a tailgut cyst is extremely rare, and to our knowledge only 5 cases of carcinoid tumor arising in a tailgut cyst have been reported in the literature to date. We report a sixth case of carcinoid tumor arising in a tailgut cyst. The patient was a 41-year-old woman who presented with perianal pain. Sigmoidoscopy showed a 2-cm submucosal mass located 4 cm above the anal verge. The mass was a multilocular cyst with gray-tan solid portions. The cyst was lined by ciliated columnar, squamous, and transitional epithelia with small foci of carcinoid tumor in the cystic wall. The carcinoid tumor showed a trabecular growth pattern with uniform oval or round cells containing fine chromatin and positive immunoreactivity for chromogranin, synaptophysin, and cytokeratin. This case was unique because the tumor occurred at the anorectal junction, not in the retrorectal space, and unlike previously reported cases showed aggressive behavior and distant metastases.     

血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错分布的细胞密集区和细胞稀疏区组成，其间血管丰富，多为毛细血管至中等大薄壁血管。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。     

Primary squamous cell carcinoma of the liver: a case report

Primary squamous cell carcinoma (SCC) of the liver is very rare, and few cases have been reported in Korea. Primary SCC of the liver is known to be associated with hepatic cysts and intrahepatic stones. A 71-year-old male was admitted to our hospital, and a abdominal computed tomography scan revealed a 10 × 6 cm mass in the liver. Analysis of a biopsy sample suggested SCC, and so our team performed a thorough workup to find the primary lesion, which was revealed hepatoma as a pure primary SCC of the liver with multiple distant metastases. The patient was treated with one cycle of radiotherapy, transferred to another hospital for hospice care, and then died 1 month after discharge.     

Vulvar hypertrophy with lymphedema. A mimicker of aggressive angiomyxoma

We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.     

Aggressive angiomyxoma of male genital region. Report of 4 cases with immunohistochemical evaluation including hormone receptor status

Aggressive angiomyxoma (AA), first described by Steeper and Rosai (Am J SurgPathol. 1983;7:463-475), is a rare locally infiltrative tumor that usually arises in the pelvic and perineal soft tissues of young women. Approximately 150 cases have been reported in women. Aggressive angiomyxoma has a high rate of local recurrence because of its infiltrative growth and anatomical location making complete excision with wide margins difficult. To our knowledge, 39 cases of AA occurring in men have been reported in the literature. Sites frequently involved include the scrotum, spermatic cord, inguinal region, and perineum. The gross and microscopic appearances and clinical course are similar to those described in female cases. Immunohistochemistry evaluating estrogen and progesterone receptors (ER and PR, respectively), although frequently positive in the female cases, has rarely been studied in the male cases. We report the clinicopathologic features of 4 additional cases of AA in men with particular emphasis on hormone (ER/PR) receptor status. Hormone reactivity is significant in that AA may arise from specialized hormonally responsive stromal cells of the perineum and may potentially play a therapeutic role in unresectable tumors. From our small series, hormone positivity (1 case of ER+, 3 cases of PR+) does occur in the male cases of AA, and a large number of cases should be examined to determine the frequency at which these tumors express hormone receptors.     

Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature

Malignant ectomesenchymomas, rare and potentially aggressive tumors, occur in children and exhibit mesenchymal and neuroectodermal components. This report describes the first patient diagnosed with a malignant ectomesenchymoma of the hand. The patient was a 17-month-old male who developed a hypothenar mass on his left hand that was surgically excised. Microscopic evaluation revealed ganglioneuroblastic, rhabdomyosarcomatous, and chondrosarcomatous elements. Following excisional biopsy he was treated with cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide. After 3 courses of chemotherapy the patient had a wide reexcision with no residual tumor. The patient is 4 years from diagnosis, without evidence of disease.     

A case of meningeal carcinomatosis with gastric cancer which manifested meningeal signs as the initial symptom; the palliative benefit of radiotherapy

A 53-year-old male presenting with anorexia, intermittent diplopia, general fatigue, headache and vertigo was admitted to our hospital. He was diagnosed as having gastric cancer by endoscopy of his upper gastrointestinal tract. Brain computed tomography (CT) showed no abnormalities, but magnetic resonance imaging (MRI) showed slight enhancement in the cerebellar sulcus. Cytological examination of cerebrospinal fluid revealed malignant cells. He became blind one week after hospitalization. We diagnosed his condition as meningeal carcinomatosis (MC) and started radiotherapy. His vision improved after four weeks of treatment, and then he became totally blind again. Since his general condition remained poor, we did not perform chemotherapy. He died on the 127th day of hospitalization. MC is a rare pathosis of gastric cancer in comparison with leukemia and malignant lymphoma. This disease does not often show characteristic pictorial images, and early diagnosis is difficult. Moreover, it usually manifests itself in its late stages after several months or more of treatment, and it is rare for MC to be present at the time of initial diagnosis. We present a case of gastric cancer with meningeal signs present when the primary tumors were diagnosed. Radiotherapy alleviated some of the symptoms, and the patient survived for as long as patients undergoing enforced chemotherapy.     

Sarcoma of follicular dendritic cells in the dorsal mediastinum

Follicular dendritic cell sarcomas (FDCSs) are very rare and usually originate in lymph nodes. We report an exceedingly rare case with localization in the dorsal mediastinum and, for the first time, provide positron emission tomography (PET) data for this tumor. This report describes the case of a 76-year-old man with a clinically aggressive tumor in the dorsal mediastinum. Computed tomography scan revealed displacement of soft tissue and lymph nodes. PET showed that the tumor had a high proliferation rate. Investigation of the successfully removed tumor mass revealed reactivity of the tumor cells for follicular dendritic cell markers and desmosomes linking adjacent tumor cells at the ultrastructural level. Marked atypia, a high mitotic rate, and areas of coagulative necrosis were found. The tumor in our case revealed the typical features and thus was classified as FDCS. In contrast to previous reports in the literature, preoperative imaging, histology, and immunohistochemistry studies indicated at least an intermediate degree of malignancy. Nevertheless, the patient made a good postoperative recovery and remained apparently disease-free 2 years later.     

Cutaneous metastasis from carcinoma of tonsil

Hematogenous spread from carcinoma of tonsil is an uncommon event and skin is an extremely rare site of metastasis. We encountered a 40-year-old male patient who initially presented with carcinoma of the tonsil with T3N2cMO disease and treated by curative radiotherapy. After about 2 years, he developed a skin lesion in the periorbital region which on cytological examination turned out to be metastasis from tonsillar carcinoma. The present paper describes this rare case report along with a brief review of the literature.