Microcystic Adnexal Carcinoma (MAC) of the Scalp with Extensive Pilar Differentiation

BACKGROUND: Microcystic adnexal carcinoma (MAC), or sclerosing sweat duct tumor, is an uncommon, locally aggressive tumor. It typically involves the upper lip or face of middle-aged adults and rarely involves the scalp. It is characterized histologically by both pilar and eccrine differentiation and is associated with frequent perineural invasion. MAC is frequently misdiagnosed because of its bland and asymptomatic clinical presentation. In addition, its defining histologic features may be missed with a superficial biopsy. OBJECTIVE: To describe a case of MAC in the scalp treated with Mohs surgery. We describe a case with extensive pilar differentiation and clear cell changes. In addition, the clinical and histologic characteristics of this rare neoplasm are reviewed. METHODS: Mohs micrographic surgery (MMS) was performed on this neoplasm. RESULTS: The tumor was successfully excised in one surgery (five stages) and is without evidence of recurrence at 18 months. CONCLUSION: We present a case of MAC of the scalp in a 64-year-old white man treated successfully with MMS.     

A Case of Primary Mucinous Carcinoma of the Scalp Treated With Mohs Surgery

BACKGROUND: Primary mucinous carcinoma of the skin is a rare sweat gland malignancy that is associated with locally aggressive behavior and a high rate of local recurrence following simple excision. OBJECTIVE: A patient with primary mucinous carcinoma of the scalp, which was treated with Mohs micrographic surgery (MMS), is described. METHODS: Case report and literature review. RESULTS: The patient underwent MMS to remove the tumor. Thirty months after the procedure, the patient remains tumor free. CONCLUSION: Simple excision of primary mucinous carcinoma of the skin is associated with a high recurrence rate. Given the low rate of metastasis and characteristic histologic tumor continuity associated with primary mucinous carcinoma of the skin, as well as the tendency for the tumor to involve cosmetically sensitive areas, such as the face and eyelids, MMS appears to represent a preferable treatment alternative for this particular sweat gland tumor. MMS appears to be associated with a very low risk of tumor recurrence.     

Two distinctive subungual pathologies: subungual exostosis and subungual osteochondroma

BACKGROUND: The purpose of this study was to present features that differentiate subungual exostosis from subungual osteochondroma. METHODS: We treated 11 patients for subungual masses. All were confirmed by radiographic and histologic evaluations to be subungual exostosis or subungual osteochondroma. The study patients comprised eight female and three male patients with a mean age at presentation of 18.7 years (range 1.5 to 70.9). In the five patients with subungual exostosis, three (60%) had a toe lesion, and two (40%) had a finger lesion. In the six patients with subungual osteochondroma, four (67%) had a toe lesion, and two (33%) had finger lesions. We analyzed the clinical features, including trauma history, the existence of infection before surgery, tumor recurrence, and postoperative nail deformity. RESULTS: In all patients, the lesions presented as an exophytic tumor of the nail apparatus, beneath the nail plate, which varied in size from 0.6 x 0.4 cm to 1.2 x 0.9 cm. Excision of these masses produced useful toes or fingers without pain, a tender scar, or nail deformity. Although nails were deformed preoperatively, they grew back without ridges or cracks within 3 to 5 months postoperatively. There were no recurrences based on clinical and radiographic evaluations, and both tumor types showed characteristic radiographic and histologic differences. CONCLUSIONS: Subungual exostosis and subungual osteochondroma are benign but have distinct osseous pathologies. We concluded that subungual exostosis is clinically, developmentally, radiographically, and histologically distinct from subungual osteochondroma.     

Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up

The clinicopathologic and immunohistochemical features of 69 pediatric examples of infantile digital fibroma/fibromatosis (IDF) were analyzed. Thirty males, 26 females, and 1 child (sex unstated) ranging from newborn to 120 months of age (median, 12 mo) manifested 74 lesions (5 identified in follow-up) involving the toe or finger (n=71) and the hand or foot (n=3). Tumors ranged in size from 3 to 35 (median, 10) mm. All but 4 study members presented with a solitary lesion. Metachronous IDFs developed in 7 patients within 17 to 82 months. Microscopically, a cytologically bland, fibroproliferative lesion was observed forming a dome-shaped/polypoid nodule directly beneath the epidermis and invading dermal adnexa. Mitotic figures per 20 high-powered fields ranged from 0 to 7 (median, 1). Paranuclear cytoplasmic inclusions were identified in 57 tumors. Tumor cells immunohistochemically expressed calponin (11 of 11 tumors), desmin (9/9), alpha-smooth muscle actin (11/11), CD99 (11/11), CD117 (6/8), heavy caldesmon (2/11 and scattered cytoplasmic inclusions in 4 tumors), CD10 (1/9), nuclear beta-catenin (2/11), and CD34 (1/11), but not muscle actin (HUC1-1), keratins, estrogen/progesterone receptor proteins, or activated caspase-3. Twenty-eight of 38 patients (74%) experienced recurrent/persistent disease (single in 22; multiple in 6) (median, 4 mo after surgery). One recurrent tumor spontaneously regressed and the size of another remained unchanged for almost 17 years before reexcision. All 23 patients with >5 years follow-up are currently disease free (median disease-free interval, 23 y). Minor postoperative functional/cosmetic complaints were reported in 47%. No patient with adequate clinical data developed the digitocutaneous dysplasia syndrome or a conventional fibromatosis, or relayed a family history of IDF/conventional fibromatosis. Our results indicate that IDF is a unique myofibroblastic process separable from conventional fibromatoses and from histologic mimics. Conservative excision or observation after biopsy (with additional surgery employed as necessary) are recommended treatment options.     

Granular Cell Tumor Treated with Mohs Micrographic Surgery: Report of a Case and Review of the Literature

BACKGROUND: Granular cell tumors are uncommon soft tissue neoplasms of neural origin that most often arise in the oral cavity. Penile lesions are distinctly uncommon. Mohs micrographic surgery (MMS) is a highly effective treatment for several cutaneous neoplasms when tissue conservation is crucial. OBJECTIVE: To examine the use of MMS for this soft tissue malignancy. METHODS: We report only the eighth granular cell tumor of the penis. The literature was reviewed regarding the use of MMS for these neoplasms. RESULTS: MMS was performed to minimize the destruction of normal tissue in treating this patient's granular cell tumor. Only one previous article documented the use of MMS for this soft tissue tumor. CONCLUSION: Although utilized infrequently to treat granular cell tumors, MMS may prove beneficial when lesion location or size render tissue conservation or assuredness of cure paramount.     

Scalp Tumors Treated with Mohs Micrographic Surgery: Clinical Features and Surgical Outcome

BACKGROUND: There are only a small number of reports on the outcome of scalp tumors treated with Mohs micrographic surgery (MMS). OBJECTIVE: The objective was to present a large series of patients with scalp tumors treated with MMS in Australia between 1993 and 2002. METHODS: This prospective, multicenter study included all patients with scalp tumors who were monitored by the Skin and Cancer Foundation Australia. The variables analyzed were patient demographics, reason for referral, preoperative tumor size and postoperative defect size, recurrences before MMS, histologic subtypes, perineural invasion, and 5-year recurrence. RESULTS: The study included 316 patients (68% men) with a mean age of 65+/-15 years. The most common tumors were basal cell carcinoma (BCC), 57.9%; squamous cell carcinoma (SCC), 35.8%; Bowen's disease (BD), 4.1%; and atypical fibroxanthoma (AFX), 1.6%. Recurrent tumors comprised 37% of cases. The recurrence rate for 70 BCC patients who completed the 5-year follow-up was 5.7%, and for 31 SCC cases it was 3.2%. No cases of recurrence were noted in the patients with BD and AFX. CONCLUSION: BCC was the most common scalp tumor managed by MMS. The low 5-year recurrence rate emphasizes the importance of margin controlled excision of scalp tumors.     

Sinonasal hemangiopericytoma-like tumor with true pericytic myoid differentiation: a clinicopathologic and immunohistochemical study of five cases

BACKGROUND: Sinonasal hemangiopericytoma-like tumor (SHPCL) is an uncommon vascular tumor that is sometimes difficult to diagnose. METHOD: We report five cases arising from the nasal cavity. The tumors were subdivided into two types, prototype and soft tissue type, by the difference in histologic features. RESULTS: The prototype, made up of three tumors, is characterized by uniform, spindle-shaped myoid cells that have eosinophilic cytoplasm and uniform nuclei and lay down in an orderly fashion. The soft tissue type, made up of two tumors, is similar to the hemangiopericytoma of soft tissue. Immunohistochemically, the tumors were positive for vimentin (100%), smooth muscle actin (80%), muscle-specific actin (100%), fibroblast growth factor-2 (100%), and vascular endothelial growth factor (100%). All were negative for cytokeratin, desmin, CD34, and factor VIII-related antigen. These histopathologic features suggest myoid differentiation and angiogenic activity of the tumors. All the tumors were treated with wide surgical excision. Only one case of soft tissue-type tumor with active mitoses received additional postoperative radiotherapy. No recurrence was found during 13 to 120 months of follow-up. CONCLUSIONS: Our data suggest that SHPCL with convincing myoid differentiation may have a more favorable clinical course than its soft tissue counterpart.     

Utility of Rush Paraffin-Embedded Tangential Sections in the Management of Cutaneous Neoplasms

BACKGROUND: Mohs micrographic surgery (MMS) modified by the use of tangential, formalin-fixed, paraffin-embedded histologic specimens is advantageous in treating selected skin neoplasms. OBJECTIVE: To review the use of our experience with a modification of MMS to treat lentigo maligna melanoma (LMM), lentigo maligna (LM) and other melanoma in situ (MIS) lesions, dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and angiosarcoma. METHODS: Our experience utilizing a modification of MMS in the treatment of 77 patients with LM or other MIS, 23 patients with LMM, 11 patients with DFSP, 1 patient with AFX, and 1 patient with angiosarcoma was reviewed. Length of follow-up and rate of recurrence were examined. A literature review of this pertinent modification of the Mohs technique was performed. RESULTS: One hundred fourteen patients underwent MMS for melanocytic (LM, MIS, LMM), spindle cell (DFSP, AFX), and vascular malignant neoplasms. One patient developed locally recurrent LM and one patient with LMM developed satellite metastasis. Regional lymph node metastasis occurred in one patient with LMM and in a patient with angiosarcoma. CONCLUSION: The use of Mohs micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential histologic sections provides the accuracy and tissue conservation of the Mohs procedure while ensuring more confident interpretation of histology in cases of lentigo maligna, lentigo maligna melanoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, and angiosarcoma.     

Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular identity with gastrointestinal stromal tumor

Gastrointestinal autonomic nerve tumor (GANT) is a gastrointestinal neoplasm that ultrastructurally recapitulates the enteric neural plexus. This study identifies and defines the features of 10 cases of this rare mesenchymal tumor and compares its clinicopathologic and molecular genetic features with the data on gastrointestinal stromal tumor (GIST). The majority of patients in this series presented at an older age (mean 64 years). Tumors arose from the stomach (6), small intestine (2), and retroperitoneum (2). Mean tumor size was 14 cm; however, four neoplasms were <6 cm. Histologically, tumors were spindled or epithelioid; one epithelioid tumor demonstrated a previously undescribed rhabdoid histologic phenotype. All tumors were positive for CD117 (KIT), while eight of 10 were positive for CD34. In contrast, only two were positive for S-100, and all were negative for actin and desmin. Five GANTs demonstrated GIST-specific gain-of-function mutations in the juxtamembrane domain of the c-kit gene (50%). Three of 10 patients died of disease in 22-30 months, one patient died in the postoperative period, and one patient died of complications of CML. The clinicopathologic, histologic, immunohistologic, and molecular features of GANT are similar to GIST, indicating that GANT merely represents a phenotypic variant of GIST.     

肱骨髁上骨折并发肘内翻畸形病理形态学分析

目的 :探讨肘内翻发生的病理形态学的改变特点。方法 :对 5 0例肘内翻进行X线片及术中所见病理形态学分析。结果 :大多数病例肱骨外髁膨大 ,内髁变小 ,滑车萎缩 ,尺骨鹰嘴窝变浅变形 ,肱骨远端尺偏、尺嵌及旋转 ,关节发育不均衡 ,尚有部分尺骨近端向肱骨尺神经沟方向滑脱。结论 :肘内翻是肱骨髁上骨折后由于肱骨远折端尺偏、尺嵌及内旋所引起 ,与骨骺损伤无关。内翻严重且日久者可造成肘关节的不稳定 ,关节面的不平衡从而出现肘外侧压痛等症状。宜早期发现 ,及早手术矫正。     

Basal Cell Carcinoma of the Nipple: A Case Report and Review of the Literature

BACKGROUND: The nipple is an extremely unusual location for basal cell carcinoma (BCC). OBJECTIVE: To report a case of BCC originating on the areola and nipple region in a 47-year-old Dominican woman treated with Mohs micrographic surgery (MMS). METHODS: We discuss a case of BCC originating on the areola and nipple region treated with MMS and review the literature regarding treatment of BCC of the nipple. RESULTS: BCCs of the nipple occur rarely, with a total of 19 cases reported in the literature, 6 of which occurred in females. While many of the reported cases were treated with simple excision, several of these required postoperative radiation therapy and/or mastectomy due to their large size and aggressive nature. CONCLUSION: MMS should be considered for treating BCCs at this site as a tissue-sparing measure to minimize deformity of this important anatomic area and to minimize the risk of recurrence.     

Kinematic changes after bicondylar resurfacing knee arthroplasty

AIM: Knee joint motion appears as a hinge movement around the flexion - extension axis. But corresponding to the rolling-sliding-mechanism and alternative models of knee joint kinematics an instant center pathway must be expected. Objective of the study was the comparison of this pathway when assessed in the sagittal plane in patients with preoperative varus or valgus deformity. METHOD: A total of 40 patients either with varus or valgus deformity (20 in each group) were examined before and 6 months after implantation of PFC(R)-SIGMA prostheses. Apart from the influence of knee joint deformity (35 degrees varus to 24 degrees valgus), a so called soft tissue ratio was investigated. Therefore the relation of thigh and femur as well as lower limb and tibia diameters was calculated and combined in one value. The examination by means of photogrammetry consisted of a standardized chair with passive knee joint movement (90 degrees to 0 degrees flexion) supported by an electric motor. The position of defined skin markers was assessed by digital camara with online transmission to PC. The instant center pathway was then evaluated applying the Reuleaux-technique based on the changes of skin marker positions. RESULTS: Results showed a dependence of the course and dimension of the instant center pathway on the extent of soft tissue ratio as well as knee joint deformity. Small values of the ratio (W = 2.9) were represented by round or oval courses of the pathway whereas increasing ratios (W = 4.0) led to triangular and slightly increased dimensions of the courses. Varus deformitywas primarily combined with an increase of the pathways dimension and in contrast, valgus deformity showed decreased pathways. In each case, dimension normalized after prostheses implantation. CONCLUSION: The instant center pathway of knee joint motion showed for both patient groups characteristic phenomen. They were clearly combined with constitutional and clinical features of each patient.     

Kinematische Analyse vor und nach bikondylärem Oberflächenersatz des Kniegelenks

Knee joint motion appears as a hinge movement around the flexion-extension axis. Corresponding to the rolling-sliding mechanism and alternative models of knee joint kinematics, an instant center pathway must be expected. The current photogrammetric investigation aimed to analyze these pathways when assessed in the sagittal plane in patients with preoperative varus or valgus deformity. A total of 40 patients either with varus or valgus deformity (20 in each group) were examined before and 6 months after implantation of PFC-SIGMA prostheses. Apart from the influence of knee joint deformity, a so-called soft tissue ratio was investigated. Therefore, the relation of thigh and femur as well as lower limb and tibia diameters was calculated and combined in one value. The investigation unit consisted of a standardized chair with passive knee joint movement (90-0 degrees flexion) supported by an electric motor. The position of predefined skin markers was assessed by digital camera with online transmission to a PC. The instant center pathway was then evaluated applying the Reuleaux technique based on the changes of skin marker positions. Results showed the following characteristics: small values of soft tissue ratio were represented by round or oval courses of the pathway whereas increasing ratios led to triangular and slightly increased dimensions of the courses. Varus deformity was primarily combined with an increase of the pathway's dimension and in contrast, valgus deformity showed decreased pathways. In each case, dimensions normalized after prosthesis implantation. The instant center pathway of knee joint motion showed for both patient groups characteristic phenomena. They were clearly associated with constitutional and clinical features of each patient.     

Bioassay of TNF-alpha in synovial fluid and ultrastructural observation of synovium from patients with osteoarthritis]

OBJECTIVE: To better define the role of synovium in the pathogenesis of osteoarthritis (OA). METHODS: We measured the bioactivity of tumor necrosis factor-alpha(TNF-alpha) in synovial fluid (SF) and examined the histologic and ultrastructural features of the synovium from patients with OA and from patients with acute and chronic traumatologic joint. RESULTS: The results showed that the levels of TNF-alpha in SF from OA were significantly elevated. Marked fibrosis and thickened intima were found in OA synovium, with much dense collagen, which, in some cases, was banded, but no specific inflammatory changes were detected. Type B cells in OA synovium had the characteristics of organelles of cells active in synthesizing and secreting protein. Type A cells in OA synovium had the features of the macrophage. CONCLUSION: Evident fibrosis in synovium may be either mediated by the cartilage degradation product or by the enhanced joint damage process in OA.     

Histologic Characteristics of Renal Cell Carcinomas with Lymph Node Metastasis

Background :
This study was conducted to determine if there are any specific histologic features that are associated with lymph node metastasis in renal cell carcinoma (RCC).
Methods :
TNM classification, histologic grade, mean nuclear volume, cell type, and histologic architecture of the tumors were evaluated in 66 patients who had undergone nephrectomy and lym-phadenectomy for RCC. In the 18 patients with positive lymph node metastasis, both primary lesions and metastatic lymph nodes were evaluated.
Results :
Lymph node status was correlated with primary tumor stage, venous involvement, and distant metastasis. The tumor grade was higher, and the mean nuclear volume was larger, in both primary and metastatic lesions of RCCs with lymph node metastasis than in tumors with no metastasis. In primary lesions of RCCs with lymph node metastasis, clear cell, alveolar, or cystic patterns were observed less frequently, and granular or spindle/pleomorphic cells and papillary or solid patterns, were observed more frequently, as compared to those lesions without metastasis. Comparison between primary and metastatic lesions in individual patients revealed no significant difference in grade or mean nuclear volume. The development of new cell types or histologic architectures, which was not noted in the primary lesions, was also a rare event in the metastatic lesions.
Conclusion :
Several characteristic histologic features, which may reflect the increased metastatic potential of the tumor, were observed in both primary and metastatic lesions in cases of RCC with lymph node metastasis. No substantial difference in histologic features was observed between the primary or metastatic lesions of individual patients.     

Tubular carcinoma of the breast. Predicting axillary nodal metastases and recurrence.

BACKGROUND: Tubular carcinoma of the breast, a well-differentiated variant of infiltrating ductal carcinoma, has been regarded as a very favorable histologic subtype. The infrequency of nodal metastases and low recurrence rates have led to questions about the value of axillary dissection. OBJECTIVE: The authors evaluated the frequency of axillary nodal metastases, the prognostic value of histologic features of the primary tumor, and treatment outcome in patients with tubular carcinoma of the breast. METHODS: This retrospective analysis evaluated 50 patients who received diagnoses of tubular carcinoma from The University of Texas M. D. Anderson Cancer Center between 1944 and 1992. Medical records were reviewed for information about the patient, the tumor, treatment, and outcome. Hematoxylin-eosin stained sections were reviewed to confirm the diagnosis and assess histologic features. RESULTS: The median tumor diameter was 1.0 cm. Nine (20%) of 44 axilla had histologically confirmed lymph node involvement. Lymph node involvement was neither infrequent nor predicted by features of the primary tumor. Recurrences developed in 4 patients, and 1 patient died of tubular carcinoma. The 5-year disease-free survival rate was 88%. The local therapy used, mastectomy or breast preservation, did not affect disease-free survival. Contralateral cancer was noted in 26% of the patients. CONCLUSIONS: Axillary metastases occur in 20% of patients and are not predicted by features of the primary tumor. Breast preservation is a safe treatment option for tubular carcinoma of the breast.     

Mohs Micrographic Surgery for Melanoma: A Case Series, a Comparative Study of Immunostains, an Informative Case Report, and a Unique Mapping Technique

BACKGROUND: Mohs micrographic surgery (MMS) has been established as an alternative to standard surgical excision for local cutaneous malignant melanoma (MM) and melanoma in situ (MIS). The use of melanocyte-specific immunohistochemical stains may improve the diagnostic accuracy of MMS frozen sections. OBJECTIVE: We used MMS with immunostains to determine the maximum and minimum margins required to clear 52 melanomas, mostly MIS of the head and neck. We sought to identify the most sensitive melanocyte immunostain for use in MMS frozen sections and to improve the clinicopathologic correlation of perilesional pigmented lesions. METHODS: We studied 52 consecutive cases of invasive melanoma (n = 10) and MIS (n = 42), tabulating the maximum and minimum margins required for complete tumor resection in any one direction during MMS. In 10 of these cases we studied the relative sensitivity of the immunostains MART-1, S-100, and HMB-45 in Mohs frozen sections. We describe a case highlighting the importance of the increased sensitivity of MART-1. In 12 patients we used a unique mapping technique to help determine the clinical relevance of a total of 35 perilesional pigmented foci. RESULTS: The maximum margin averaged 10.1 mm (range 3-26 mm) for MM and 9.4 mm (range 3-18 mm) for MIS. The minimum margin averaged 7.0 mm (range 3-13 mm) for MM and 5.5 mm (range 3-11 mm) for MIS. For individual tumors, the difference between the minimum and maximum margin averaged 3.7 mm (range 0-13 mm). This difference was >or=5 mm in 38% (20/52) and >or=10 mm in 10% (5/52), highlighting that subclinical tumor extension is often broad and asymmetric. While five of six MM less than 1.0 mm in depth would have been cleared with a routine 1 cm excision, a standard 5 mm margin would have cleared less than one-quarter of the cases of MIS (10/42). In Mohs frozen sections, MART-1 proved superior in sensitivity to both HMB-45 and S-100. Our mapping technique provided clinically relevant histologic correlation for perilesional pigmented lesions, improving the Mohs surgeon's ability to evaluate equivocal foci in frozen sections. CONCLUSION: MM, especially MIS on the head and neck, often exhibits an asymmetric growth pattern, making it quite suitable for treatment with MMS. The use of MART-1 immunostain may improve the diagnostic accuracy of Mohs surgeons. We believe that HMB-45 should not be used to rule out the diagnosis of MIS in equivocal sections because of its inferior sensitivity. We introduce a new mapping technique as an adjunctive measure to aid in the clinicopathologic evaluation of perilesional skin.     

应用Ilizarov技术治疗下肢复杂畸形

目的：总结应用Ilizarov技术治疗下肢短缩，大段骨缺损合并其他病变的复杂畸形的体会。方法：应用骨段皮质截骨延长输送或楔形骨压缩迟延延长等方法治疗开放性骨缺损伴骨髓炎4例；股骨或胫骨肿瘤瘤段切除3例；肢体短缩伴成角畸形3例。结果：10例均恢复了支体长度和持重功能。2例股骨延长者发生膝关节屈曲受限，1年后恢复。1例儿童出现胫骨过生长。结论：Ilizarov技术是治疗短肢，修复创伤，炎症，肿瘤等所致骨缺损的简单而有效的新方法。     

Myofibroblastoma of the Breast: Cytologic, Histologic, Immunohistochemical, and Ultrastructural Findings in Two Cases with Differing Cellularity

Abstract: We present the cytologic, histologic, immunohistochemical, and ultrastructural features of two cases of myofibroblastoma with different cellularity, one paucicellular the other hypercellular. Fine-needle aspiration suggested the diagnosis in the case of the hypercellular tumor of the breast and pointed out the benign nature of the paucicellular case, arising in the breast of a young woman. Immunohistochemistry, showed smooth muscle-specific actin, vimentin, and desmin positivity consistent with myofibroblastic differentiation, in both cases. The positivity of both tumors to CD34 suggests that they may be related to the so-called solitary fibrous tumor. In addition, there was strong progesterone- and weak estrogen-receptor positivity ion the hypercellular case, a novel finding in these tumors, with possible histogenetic implications. Ultrastructural findings in the first case were consistent with the myofibroblastic origin of the tumor.