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Notes: The histogenesis of atypical fibroxanthoma (AFX) and its relationship to malignant fibrous histiocytoma (MFH) arc a subject of controversy. Many investigators have proposed that AFX may represent a reactive process, while others contend that it is a true fibrohistiocytic neoplasm, closely related to MFH. In an attempt to determine whether biologic differences between AFX and MFH may be accounted for at the cellular DNA level, we performed ploidy analysis on 14 cases of AFX by flow cytometry and compared our results with previously reported DNA analyses of MFH. Thirteen of the 14 lesions demonstrated diploid distribution of nuclear DNA, and only 1 case had an aneuploid population. This contrasts with prior data on MFH, the vast majority of which are aneuploid. Our results suggest that, despite histologic similarities, AFX may be distinguished from MFH on the basis of DNA content. These findings may be significant in understanding the biologic behavior of AFX.

Notes: Fibrous papules (FPs) are common benign lesions usually of the face. Most are readily recognizable histologically, although several variants exist that may not be as easily diagnosed. These include hypercellular, clear cell, pigmented, pleomorphic, and inflammatory variants. A granular cell variant has also been described. We microscopically evaluated 212 FPs, and of those, 184 demonstrated features of one of the variants. We conclude that variants of FP may be encountered not uncommonly. Dermatopathologists should be aware of these to avoid misdiagnosis.

Notes: Abstract Although most examples of cutaneous malignant melanoma are easily recognized by their clinical appearances, in some cases this serious neoplasm may clinically simulate other less serious forms of skin cancer or benign processes. This study was undertaken to assess both the sensitivity of clinical diagnosis of cutaneous malignant melanoma and the efficacy of biopsies of clinically unsuspected melanomas in yielding specimens on which complete and accurate histologic assessments could be made. A retrospective analysis of 1784 cases of histologically proven melanomas diagnosed between 1985 and 1990 was performed in search of lesions not clinically suspected. Biopsy techniques used to sample these lesions were subjected to critique of their efficacy in yielding specimens that could be accurately diagnosed and completely assessed histologically. Of 1784 histologically proven primary cutaneous melanomas, 583 were not clinically suspected, yielding a sensitivity of 61%. Clinical diagnosis included nevi (33%), no diagnosis (17%), multiple diagnoses (13%.), basal cell carcinoma (12%), keratosis (9%), and lentigo (9%) among others. The biopsy methods used to sample these lesions were shave (56%), excisional (24%), punch (11%), curettage (2%), and undetermined (6%). Eighty-six percent of shave biopsies could be accurately assessed while only 32% of punches and no curettages provided sufficient material for both definitive and complete evaluation of melanomas. Eighteen percent of specimens histologically reviewed were considered inadequate for complete evaluation. In 34%, the actual diagnosis of melanoma was uncertain because of inability to assess diagnostic features as a consequence of the biopsy technique. Melanoma may be unsuspected clinically in a significant number of cases and may be mistaken for less serious cutaneous neoplasms. Biopsy material that is not representative of the entire process may lead to misdiagnosis. Biopsy specimens measuring 5–6 mm in breadth and extending to at least the mid-reticular dermis for 4–5 mm will provide representative sections in most cases and should allow for detection of clinically occult melanoma.

Notes: Background:  Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant neoplasm that has the potential for aggressive local growth and destruction if not treated appropriately. Although the storiform arrangement of spindle cells in DFSP is relatively characteristic, histologic patterns simulating other benign as well as malignant neoplasms such as dermatofibroma, neurofibroma, malignant fibrous histiocytoma, and atypical fibroxanthoma have been described.Methods:  We collected and analyzed six cases of probable DFSP in which a specific diagnosis could not be rendered due to the predominant neurofibromatous changes in the histologic sections. In an attempt to reach a definitive diagnosis, the clinical history and physical characteristics of the lesions were taken into account, and all cases were further evaluated using immunostaining for CD34 and S-100 protein.Results:  The average age of the patient was 56 years (range 21–80), and the male to female ratio was 1 : 1. The location of lesions included the scalp, neck, back, and abdomen. All cases displayed two distinct histological patterns: (i) a proliferation of spindle cells with wavy nuclei in a loose mucinous stroma suggesting neural differentiation and (ii) a proliferation of spindle cells which interweaved and filled the reticular dermis extending into the subcutis. The wide variety of clinical impressions and descriptions indicated that the diagnoses were not always straightforward, and clinical information did not always assist in the clinicopathologic correlation. All lesions stained positively for CD34; however, three of six cases also stained positively for S-100. The three cases which were CD34 positive and S-100 negative were likely DFSP, and this was the final diagnosis given. The three cases that were CD34 and S-100 positive did not allow for a straightforward diagnosis.Conclusions:  DFSP may demonstrate areas with features more characteristic of a benign neural lesion, such as a neurofibroma, which can lead to underdiagnosis and subsequent failure to treat. Clinicians and pathologists should recognize this potential diagnostic pitfall and understand that equivocal clinical information, combined with non-specific immunohistochemical staining patterns, can further complicate the dilemma. In these situations, where DFSP is the likely diagnosis but definitive evidence cannot be obtained, full excision of the lesion should be recommended to avoid mistreatment of a potentially malignant lesion.

Notes: Background:  Heparan sulfate (HS), unlike other glycosaminoglycans, is mainly located on cell surfaces but can be shed into the interstitium by a regulated process. It has been found in interstitial fluid drained from cutaneous wounds, but otherwise the conditions under which the release of HS from the cell surface occurs are unknown. To better characterize this process, we have investigated the presence of interstitial HS in various skin diseases with glycosaminoglycan accumulation.Methods:  Histologic routine material was stained immunohistochemically using an antibody recognizing HS.Results:  Heparan sulfate immunoreactivity is present in the interstitium of young cutaneous scars and in the interstitium of the inflammatory infiltrate of granuloma annulare. No reactivity was found in a number of non-inflammatory skin diseases with mucin deposition.Conclusions:  The selective presence of interstitial HS in only two of the investigated skin conditions supports the existence of a regulated mechanism to release HS from the surface of cells into the interstitium. It is suggested that HS modulates the biologic actions of growth factors and cytokines not only during wound repair but possibly also in inflammatory skin diseases such as granuloma annulare.

Notes: A 45-year-old white woman presented with several years’ history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient.On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (〈link href="#f1"〉Fig. 1). There were no lesions on the feet.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1980:IJD_1980_f1"/〉Small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, seen on the right medial hand show the characteristic changes of acrokeratoelastoidosisA biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (〈link href="#f2"〉Fig. 2).〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1980:IJD_1980_f2"/〉Hematoxylin and eosin stain from a biopsy of the right medial hand shows marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (× 4)She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.

Notes: A 50-year-old Hispanic woman presented to the medical walk-in clinic of a local community hospital complaining of shoulder pain. The problem had begun 9 months previously, and first manifested as dull right shoulder pain that developed after she carried a bag of groceries up one flight of stairs. A diagnosis of “muscle strain” and “arthritis” was made for which nonnarcotic analgesics were prescribed. The pain persisted, and 2 months later she was re-evaluated and diagnosed with degenerative joint disease. An orthopedics consultation was sought for further evaluation. During this examination, she pointed out to the physician that she had a “lump in her shoulder,” but she was informed that this was of no consequence and that her complaints were due to arthritis. Roentgenograms of the right shoulder and thoracic spine were performed and determined to be within normal limits. Once again she attempted to control the pain with nonsteroidal anti-inflammatory agents to no avail. Because the pain had become unbearable, she sought relief at the medical walk-in clinic. Physical examination was remarkable for a solitary, skin-colored, firm, deeply-seated tumor measuring 3×4 cm located over the rightposterior of the deltoid. The mass was exquisitely tender to palpation and was fixed to the underlying muscle. There was a full range of motion as well as good muscle strength of the shoulder, but movement of the arm in any direction was painful. The patient subsequently underwent a deep, partial excisional biopsy of the lesion, and a firm white tumor mass of about 3 × 3 cm in size was dissected away from the deltoid muscle. Although the majority of the tumor was excised, visible portions of the lesion were not removed because they were present deep within the bulk of the muscle. Histopathologic examination revealed a large, deeply-seated poorly-circumscribed fibrous proliferation containing areas in which there were numerous spindle cells arranged in fascicles ( 〈link href="#f1 #f2 #f3"〉Figs 1, 2, and 3). Neither cellular atypia nor mitotic figures were seen. The diagnosis of extra-abdominal desmoid tumor was made. The patient subsequently underwent a wide re-excision of the area and tolerated the procedure well, developing normal function of the arm following surgery, although there was slight persistent tenderness of the deltoid. Because of the known association of desmoid tumors with familial polyposis coli 1, a barium enema was performed. No colonic polyps were demonstrated.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD538:image_n/IJD_538.f1"/〉Histopathology of desmoid tumor at scanning magnification. There is a large, poorly-circumscribed, diffuse spindle cell proliferation that extends into the subcutis, muscle, and fascia (hematoxylin and eosin; original magnification, ×10)〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD538:image_n/IJD_538.f2"/〉At still higher magnification, abundant spindle cells in fascicles were appreciated and could be readily distinguished from collagen bundles. Note the elongated, wavy nuclei. No cellular atypia was seen (hematoxylin and eosin; original magnification, ×480)〈figure xml:id="f3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD538:image_n/IJD_538.f3"/〉At still higher magnification, abundant spindle cells in fascicles were seen and could be readily distinguished from collagen bundles. Note the elongated, wavy nuclei. No cellular atypia was seen. (H & E, original magnification ×480)The patient was seen in follow-up 6 months post-operatively. The pain had increased significantly since surgery and, although no mass could be palpated, it was thought that the increasing pain was most likely a consequence of deep local recurrence of the fibromatosis. The patient was offered a course of local X-irradiation, but refused and elected to pursue pain relief with a local range of motion exercises and analgesics.