Primary giant cell tumor of soft tissue

BACKGROUND: Primary giant cell tumor of soft tissue, also known as soft tissue giant cell tumor of low malignant potential, is a rare soft tissue tumor located in both superficial and deep soft tissue. Histologically, these lesions bear a close resemblance to their bony counterparts, giant cell tumor of bone, with round to spindle-shaped cells intimately admixed with uniformly scattered osteoclast-like multinucleated giant cells. In 1989 in the dermatology literature, two malignant giant cell tumors of soft parts were described that filled the dermis and extended into the subcutaneous tissue. METHODS: The authors report the rare occurrence of a giant cell tumor of soft tissue occurring primarily in the dermis that lacks overtly malignant features and clinically was thought to be an epidermal inclusion cyst. RESULTS: Light microscopy revealed a non-encapsulated cellular dermal tumor containing numerous osteoclast-like giant cells. Cytologic atypia was minimal and the mitotic count averaged 2-3/10 HPF. The histologic differential diagnosis is also discussed. CONCLUSION: Giant cell tumor of soft tissue is a rare neoplasm of the skin, however, recognition of this tumor is important due to its behavior as a low-grade malignancy.     

Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update

The fibrohistiocytic tumors of intermediate malignancy are uncommon mesenchymal tumors, which typically occur in the skin and subcutis and which may pose significant problems for the dermatopathologist. This article reviews the clinical, histopathologic, and genetic features of dermatofibrosarcoma protuberans, giant cell fibroblastoma, angiomatoid (malignant) fibrous histiocytoma, plexiform fibrous histiocytoma, and soft tissue giant cell tumor (of low malignant potential). The differential diagnosis of these tumors with a variety of benign and fully malignant cutaneous soft tissue neoplasms is discussed.     

Soft-tissue masses as presentation of non-Hodgkin's lymphoma in AIDS patients

Primary soft tissue Non-Hodgkin lymphomas are very rare and account only for 0.1 % of the cases. Generally, Non-Hodgkin lymphomas of the soft tissue present as large subcutaneous masses without evidence of nodal or skin involvement. We describe four cases of primary Non-Hodgkin lymphomas of the soft tissue in patients infected with the human immunodeficiency virus. The most common site of involvement was the chest wall in all the patients; histopathological and immunophenotypic examination of the biopsy smears revealed two cases of plasmablastic lymphomas, one Burkitt and one diffuse large B-cell lymphoma. Non-Hodgkin lymphomas should be included in the differential diagnosis of soft tissue masses in human immunodeficiency virus - seropositive patients.     

肉芽肿性松弛皮肤特殊类型的蕈样肉芽肿一例

目的 报告一例蕈样肉芽肿之极其罕见的肉芽肿性皮肤松弛.方法 对其临床、组织病理、免疫组化、超微结构和分子生物学进行研究.结果 在皮肤肿块内致密淋巴细胞、组织细胞和多核巨细胞浸润,少数淋巴细胞向表皮性.弹性纤维几乎完全消失.免疫组化示淋巴细胞表达CD3,CD45RO.分子生物学检查示T细胞受体β链基因重排.超微结构示巨噬细胞有许多绒毛样结构和溶酶体.结论 此例为一种罕见变异的蕈样肉芽肿.     

Primary giant cell tumor of soft tissue of the groin – a case of 46 years duration

Background: Soft tissue giant cell tumor (GCT-ST) of low malignant potential is an uncommon neoplasm, considered the soft tissue counterpart of giant cell tumor of bone. GCT-ST mainly affects young to middle-age adults and presents as a painless growing mass mainly located in the lower extremities and trunk. Histologically, this tumor is characterized by a mixture of uniformly scattered osteoclast-like multinucleated giant cells intimately admixed with short fascicles of spindled cells. Complete excision with negative surgical margins is associated with a benign clinical course in most cases.
Methods: The authors report the clinicopathological and immunohistochemical features of an unusual GCT-ST of 46 years duration previously histologically misdiagnosed as Kaposi's sarcoma.
Results: Histologically, the tumor was characterized by a multinodular growth pattern with osteoclast-like multinucleated giant cells admixed with spindle cells partially arranged in a storiform pattern, fibrosis and foci of haemorrhage and mature bone. Immunohistochemistry revealed CD68 reactivity of the multinucleated giant cells.
Conclusion: GCT-ST is a rare neoplasm characterized by benign clinical course if excised adequately, as shown by our case of exceptionally long duration. Emphasis is placed on the importance of differential diagnosis with other giant cell-rich soft tissue neoplasms because clinical behaviour, prognosis and treatment significantly differ.     

Malignant giant cell tumor of soft parts presenting as a skin tumor

Malignant giant cell tumor of soft parts is a rare neoplasm that histologically resembles a giant cell tumor of bone. It has a distinctive multinodular growth pattern and is composed of numerous osteoclast-like giant cells, histiocytes, and fibroblasts. Although this tumor is usually found in deep soft tissues, a superficial form has been described in the subcutaneous tissue and fascia. The authors report two patients, aged 75 and 78, with malignant giant cell tumors presenting as ulcerating skin nodules of the arm and foot. The tumors were relatively small, measuring less than 3.5 cm in greatest dimension, and involved the entire dermis and subcutaneous tissue. The clinical differential diagnoses included Kaposi's sarcoma, melanoma, and hematoma. Dermatopathologists and dermatologists should be aware of this entity to avoid confusion with other benign and malignant neoplasms that may contain multinucleated giant cells. The distinguishing histologic and immunohistochemical features of this tumor are discussed.     

长期误诊的肉芽肿性皮肤松弛症一例

报告1例伴皮肤慢性溃疡而长期误诊的肉芽肿性皮肤松弛症.患者男,40岁.左臀部皮下肿块及溃疡5年伴疼痛,皱褶部位皮肤松弛1年.皮肤科检查:左臀部弥漫性暗红色斑块,其上多个大小不一的深在性溃疡,皱褶部位皮肤松弛明显.组织病理检查:单个核细胞移入表皮,真皮全层可见淋巴细胞、组织细胞弥漫性浸润,可见多核巨细胞.免疫组化示淋巴细胞CD3、CD43、CD45RO均阳性,CD20和CD790α阴性;多核巨细胞CD68阳性.     

A light and electron microscopic study of a case of radiation induced malignant giant cell tumor of the soft tissue.

A case of malignant giant cell tumor of the soft tissue in a 59-year-old female is presented. Total hysterectomy was performed for uterine carcinoma (histologically squamous cell carcinoma) in 1963, followed by radiation treatment for 6 months. Chronic radiodermatitis developed thereafter on the skin of the hip joint region, buttocks and lower abdominal region. A tumor developed in the lesion of chronic radiodermatitis of the left hip joint region in August 1976 and this was excised on February 23, 1977. Two recurrent tumors and a metastatic lesion in the inguinal lymph node developed, which were surgically removed. The main tumor, measuring 7.5 cm in maximum diameter, was found chiefly in the subcutaneous adipose tissue. Histologically, it was composed of a mixture of fibroblastic spindle-shaped cells, histiocytic mononuclear cells, bizarre giant cells and osteoclast-like multinucleated giant cells. Occasionally osteoid tissues were seen. Electron microscopically, undifferentiated cells, fibroblastic cells, monohistiocytic cells, osteoclast like multinucleated giant cells and macrophages were observed, and these cells were considered to be divided into three cell lines, that is an undifferentiated cell line, fibroblastic cell line and histiocytic cell line. The histogenesis of malignant giant cell tumor in this case, is considered to have been from undifferentiated cells to both fibroblastic and histiocytic differentiation through the neoplastic stimulus of radiation.     

Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin

We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.     

Bilateral inguinal hernia with dislocation of great saphenous vein as complication of long-standing granulomatous slack skin: a case report

Granulomatous slack skin (GSS) represents a rare variant of mycosis fungoides, histologically characterized by a variably deep T helper lymphocytes infiltrate with alteration of the dermal elastic tissue and consequent elastolysis, elastophagocytosis and numerous giant cells. Clinically, a development of unelastic, slack skin, especially on flexural areas, is observed. Hereby, we describe a man with a 12-year history of GSS. In 2002, for practical (limitation of movement, deambulation) and cosmetic reasons, he underwent the surgical excision of loose and sagging skinfold over inguinal area, and, afterwards, of the opposite affected inguinal skin. The surgical treatment of bilateral inguinal hernia with reposition of inguinal dislocated vasculature is also reported. In both cases the excised material confirmed the former diagnosis of GSS and revealed a very deep, muscular infiltrate of neoplastic lymphocytes. One year later, a new excision of GSS on the axillae was made. Now, after 2 years, deambulation keeps improving, although an initial relapse of the inguinal slack skin has been observed.     

Granulomatous slack skin‐like clinical findings in Sézary syndrome

Granulomatous slack skin (GSS) is a very rare condition that has been described as a variant of mycosis fungoides. It is characterized by the development of bulky and pendulous skin folds in flexural areas that are histologically formed by atypical T lymphocytes, histiocytes and giant cells. We report the case of a 37‐year‐old African‐American female with history of Sézary syndrome (SS) that while on treatment for the disease and in a space of 1 month developed exorbitant slack folds in the axillae and cervical area mimicking GSS. The absence of giant cells and epithelioid granulomas in the biopsy ruled out this diagnosis. We report this peculiar SS presentation that clinically resembles GSS, but with histopathology that does not show the typical features of this condition. We also review the literature in regard to SS, GSS and granulomatous mycosis fungoides (GMF), particularly the existing criteria to differentiate these various entities.     

淋巴瘤伴发皮肤肉芽肿的现象及其意义

淋巴瘤可伴发皮肤肉芽肿,其模式主要有两种:一种是淋巴瘤的特异性皮损中出现肉芽肿反应,例如肉芽肿性蕈样肉芽肿和肉芽肿性皮肤松弛症;另一种是淋巴瘤出现非特异的肉芽肿性皮损,主要见于一些系统性淋巴瘤.其组织病理学类型可为结节病样、环状肉芽肿样、结核样等.其机制及其与预后的关系尚不明确.肉芽肿形成可能是造成某些淋巴瘤特殊临床表现的原因,也容易掩盖淋巴瘤的真实面目,在临床和组织学诊断上需谨慎.     

儿童肉芽肿性皮肤松弛症

肉芽肿性皮肤松弛症系一种罕见的T细胞性淋巴瘤,临床表现为皮肤松弛、斑块、结节。病理特点为皮肤的淋巴样细胞浸润及巨大的多核细胞性肉芽肿伴弹性纤维消失。本文报告1例男性患儿,10岁发病,15岁死亡。     

Axillary giant lipoma: A report of two cases and published work review

Lipomas are common, benign, small‐sized, soft‐tissue tumors. However, giant lipomas are uncommon and the tumor size can cause pain and nerve compression syndrome. The axilla is an extremely rare location for development of giant lipomas. We report two cases of axillary giant lipoma. A 47‐year‐old man (case 1) and a 42‐year‐old woman (case 2) presented with a large mass in the axillary region. Case 2 had tenderness in the shoulder and numbness in the upper arm. Magnetic resonance imaging of each tumor showed a homogenous soft‐tissue mass in the axillary region, suggestive of lipoma. In case 2, the tumor extended from the axilla to the supraclavicular region and split and compressed the neurovascular bundle. Each lesion was successfully excised surgically without serious complications and recurrence. In case 2, the tenderness and numbness disappeared. Histologically, each lesion was composed of multilobulated, mature adipose cells, which led to a diagnosis of benign lipoma. Axillary giant lipoma is preferably excised surgically to avoid damage caused by tumor compression to the major vessels or nerves, to offer better local control and to establish a correct final diagnosis.     

Primary cutaneous Ewing sarcoma - Case report

Ewing sarcoma is a primitive neuroectodermal tumor rarely occurs in the skin and sobcutaneous tissues. Generally Ewing''s sarcoma is a primary bone tumor, but when present in soft tissues it characterizes an extremely uncommon clinical picture. It usually involves the deep subcutaneous tissue or muscles, and more rarely occurs like a primary skin cancer. Most patients are white, women, and in the second decade of life. The clinical features are a superficial mass, in average measuring 2-3 cm, of soft consistency, freely mobile and sometimes painful. The more affected locations are upper and lower extremities, trunk, head, neck or multiple lesions. The presence of metastases is very rare.     

Giant cell fibroblastoma: an immunohistochemical study

Giant cell fibroblastoma is a rare, benign soft tissue tumor occurring in childhood. A 34-year-old woman presented with a giant cell fibroblastoma involving the chest wall. Histologic features include an infiltrating spindle-cell tumor involving the dermis and subcutaneous fat containing characteristic sinusoidal spaces rimmed by spindle cells and multinucleate giant cells. Immunohistochemical studies support a fibrohistiocytic differentiation.     

Primary giant cell tumor of soft tissue in the finger

Primary giant cell tumor of soft tissue (GCTST) arising in a finger is a rare event. We report a case of a 54-year-old man with a primary finger giant cell tumor that appeared histologically identical to giant cell tumor of bone. The patient presented with a cystic mass of the finger. The magnetic resonance imaging showed no relation between the nodule and bone, tendons or synovial tissues. The distinction of this entity from other more common primary finger tumors with giant cell morphology is emphasized.     

肉芽肿性皮肤松弛症

报告1例肉芽肿性皮肤松弛症.患者男,58岁.因全身皮肤松弛,出现红斑、斑块,腋窝及腹股沟有巨大垂吊的皮肤肿块而就诊.皮损组织病理学改变为真皮全层弥漫淋巴样细胞浸润,可见巨大的多核细胞性肉芽肿,弹性纤维缺失.该文对肉芽肿性皮肤松弛症临床、组织病理、免疫组化和分子生物学进行了研究.     

Granulomatous Slack Skin in Childhood

Abstract: Granulomatous slack skin is an uncommon cutaneous T- helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in children. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdomen, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid Infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43+ (MT1), CD45+, CD45RO+, CD20-. The phenotype of the giant cells was lysozyme positive, CD68+ and Mac387–. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (CβTCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cutaneous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease.     

Giant cell angiofibroma or localized periorbital lymphedema?

Giant cell angiofibroma represents a rare soft tissue neoplasm with a predilection for the orbit. We recently encountered a mass removed from the lower eyelid of a 56‐year‐old female that histopathologically resembled giant cell angiofibroma. The process consisted of haphazardly arranged CD34‐positive spindled and multinucleated cells within an edematous, densely vascular stroma. However, the patient had recently undergone laryngectomy and radiotherapy for a laryngeal squamous cell carcinoma. A similar mass had arisen on the contralateral eyelid, and both had developed several months post‐therapy. Lymphedema of the orbit can present as tumor‐like nodules and in some cases may share histopathologic features purported to be characteristic of giant cell angiofibroma. A relationship between giant cell angiofibroma and lymphedema has not been established, but our case suggests there may be one. The potential overlap of these two conditions should be recognized, as should other entities that may enter the differential diagnosis.