ABSTRACT
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of uncertain differentiation with low metastatic potential, most commonly occurring in children, adolescents, and young adults, involving extremities. Due to its rare nature and diverse presentation, both clinically and morphologically, it is often misdiagnosed. It becomes important to correctly diagnose this lesion, given its distinct therapeutic implications. Here, we present the clinical, radiologic, and pathologic findings of two rare cases of AFH. Since AFH is a rare soft tissue tumor with low malignant potential, both pathologists and clinicians should be aware of this entity, when encountered with a soft tissue mass in extremities of a child or adolescent, so as to accord appropriate treatment in such cases.
ABSTRACT
Background: Angiomatoid fibrous histiocytoma (AFH) is an unusual soft tissue tumor (STT), characterized by recurrences, but rarely metastasis. Later, certain molecular signatures have been identified underlying this tumor, which at times, is either underdiagnosed as a benign vascular tumor, or over diagnosed as a high‑grade pleomorphic sarcoma, including a malignant fibrous histiocytoma. Materials and Methods: Over a 14‑year‑period, five diagnosed cases of AFH were analyzed. Results: Five tumors occurred in three males and two females, over a wide age‑range (median = 21, mean = 30 years); mostly in the extremities (4) (80%). Microscopically, most tumors were circumscribed, comprising large, blood‑filed spaces with surrounding histiocytic cells and a “cuff” of lymphoplasmacytic cells. Three tumors revealed solid growth pattern with polygonal to spindle cells, including myxoid matrix in one of these tumors. On molecular analysis, this tumor exhibited EWS‑CREB transcript. Immunohistochemically, various tumors were positive for CD68 (n = 2/2), epithelial membrane antigen (n = 3/4), CD99/MIC2 (n = 2/3), and desmin (n = 1/4). All tumors were surgically excised. On follow‑up (n = 2), a single patient, who underwent wide‑excision was free‑of‑disease (24 months), while another patient had a recurrence 4 months post tumor excision. Conclusions: This forms as the first documented series on clinicopathological features of AFH, a rare STT, from our country. Significant clinicopathological features include younger age, extremities as commonest site and histopathological appearance of blood‑filled spaces with surrounding “cuff” of histiocytic cells and lymphocytes. Tumors with unusual histopathological tumor patterns require molecular confirmation. Surgical resection remains the treatment mainstay.
ABSTRACT
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor, with a low-grade malignant potential, occurring predominantly in children and young adults. Association between AFH and other malignancies has been rarely reported. A 27-year-old man who presented with a palpable abdominal mass was diagnosed as having testicular cancer with multiple liver and lung metastases. At 16 months after chemotherapy, a follow-up computed tomographic scan revealed a supraclavicular mass measuring 3 cm in size, which was suspected to be a recurrence. The patient underwent surgical excision, and the mass was pathologically diagnosed as a AFH. The patient has had no local recurrence and no distant metastasis for 12 months after resection. To the best of our knowledge, this is the first case report of AFH as a second tumor in a patient with testicular cancer.
Subject(s)
Adult , Child , Humans , Young Adult , Follow-Up Studies , Histiocytoma , Histiocytoma, Benign Fibrous , Liver , Lung , Neoplasm Metastasis , Neoplasms, Second Primary , Recurrence , Testicular NeoplasmsABSTRACT
AFH is a singular osculant tumor and difficult to be diagnosed. A case of AFH was treated in the author's department sucessfully. The author retrospected the course of the treatment and regarded that expanded excision is necessary and radiolagical therapy after operation is helpful.
ABSTRACT
Angiomatoid fibrous histiocytoma is a rare soft tissue tumor that generally affects children and young adults. We report a case of angiomatoid fibrous histiocytoma in an 11-year-old boy who complained of a back mass for 3 years. Surgical excision was performed. The excised specimen showed a 4.0 x 3.6 x 3.0 cm, well circumscribed, grayish white tumor, with multicystic changes. Histological examination showed proliferation of spindle or round shaped tumor cells. There was a dense fibrous pseudocapsule with prominent chronic inflammatory cell infiltrates.
Subject(s)
Child , Humans , Male , Young Adult , Histiocytoma, Benign Fibrous , ImmunohistochemistryABSTRACT
Angiomatoid fibrous histiocytoma is a rare soft tissue tumor, occurring predominately in children and young adults, with a low grade malignant potency. This tumor should not be confused with, and is not identical to, the aneurysmal fibrous histiocytoma of skin. We reported a case of a 6-year-old female who presented with an asymptomatic 1.5 cm x 1.5 cm sized erythematous nodule on the left knee of 3 years duration. The lesion showed a cystic tumor surrounded by fibrous pseudocapule in the dermis and subcutis and lymphoplasmacytic infiltrations in the pericapsular area. The tumor consisted of fibroblast-like and histiocyte-like cells and pseudovascular cysts. Clinical and histological findings led to the final diagnosis of angiomatoid fibrous histiocytoma.
Subject(s)
Child , Female , Humans , Young Adult , Aneurysm , Dermis , Diagnosis , Histiocytoma, Benign Fibrous , Knee , SkinABSTRACT
We report on a case of angiomatoid fibrous histiocytoma located deep in subcutaneous tissue of the left shoulder in an 11-year-old boy. An ovoid firm mass was slowly increasing in size for 5 months without any pain or tenderness. The tumor was grossly multicystic, with dense fibrous capsule and it measured 7.5 x 7.5 x 4.0 cm. The locules contained hemorrhagic fluid, and the inner surface was purplish brown and trabecular. The septal solid areas were cellular, and they consisted of histiocytoid spindle cells that were arranged in a storiform or fascicular pattern. The tumor cells showed immuno-positivity for CD99, CD68 and desmin. These findings were compatible with angiomatoid fibrous histiocytoma of an uncommon location.
Subject(s)
Child , Humans , Male , Desmin , Histiocytoma, Benign Fibrous , Shoulder , Subcutaneous TissueABSTRACT
Fibrous histiocytoma is a common benign tumor which occurs usually in the dermis. It can be classified as fibrous type, cellular type, mixed type and angiomatoid type, depending on its major cellular components. Angiomatoid fibrous histiocytoma is a rare subtype, showing characteristic hemorrhagic cyst. Fibrous histiocytoma arises rarely in deep soft tissues such as fascia, muscle or periosteum, and shows clinical and histopathological characteristics somewhat different from the cutaneous histiocytoma. We report a case of angiomatoid fibrous histiocytoma of deep location which arose in the galea of scalp in a 7-year-old boy, who had a tender slightly fixed deep seated nodule, measuring 0.5 cm, for 5 months.