Transjugular intrahepatic portosystemic shunt in an infant

BACKGROUND: Merkel cell (neuroendocrine) carcinoma (MCC) is a very aggressive primary cutaneous neoplasm most often occurring on the head and neck of the elderly. Spontaneous regression of MCC was first described in this journal in 1986. Since then, other such cases have been reported. This case represents the sixth case of spontaneous regression of MCC. OBJECTIVE: To describe to clinical course in a patient with MCC who underwent spontaneous regression of metastatic disease. METHODS: Clinical records including detailed history and frequent follow-up examination made this observation possible. RESULTS: Complete clinically evident regression of metastatic MCC was observed in this case. However, the patient received no treatment known to be effective for MCC. CONCLUSIONS: Spontaneous regression of MCC has been documented. The reason for regression is unknown. Further study of these rare cases may in the future provide more answers than questions.     

Microcystic adnexal carcinoma. First reported case in a black patient

BACKGROUND: Microcystic adnexal carcinoma is an uncommon malignancy of the skin with a propensity for local aggressive growth and high rates of recurrence. To date, this neoplasm has been described mainly in the Caucasian population. We describe here the first reported case in a black patient. OBJECTIVE: To discuss the successful identification and management of this aggressive neoplasm in the non-Caucasian population. METHODS: Mohs micrographic surgery was performed on this tumor. RESULTS: The tumor was successfully excised without evidence of recurrence at 6 months. CONCLUSIONS: We present the first reported case of microcystic adnexal carcinoma in a black patient. This tumor was identified in its early stages and removed by Mohs micrographic surgery without sequelae.     

Lymph node Merkel cell carcinoma with no evidence of cutaneous tumor--report of two cases

We report two cases of Merkel cell carcinoma within inguinal and axillary lymph nodes, respectively, showing no clinico-pathologic evidence of a primary (extranodal) tumor; one of our patients was alive with no evidence of disease five years and ten months after the surgical excision of the neoplasm with no postoperative chemotherapy. The diagnosis of nodal Merkel cell carcinoma needs to be supported by a careful immunohistochemical study: in fact, cytokeratin- and neurofilament-positive paranuclear "dots", as well as epithelial antigens and neuroendocrine markers may be variably expressed in tumor cells, thus requiring the application of a complete antibody panel. In the presence of a nodal Merkel cell tumor, an exhaustive clinico-radiologic search for a primary tumor must be carried out. After the exclusion of any reasonable starting point of the neoplasm, a provisional diagnosis of "primary" nodal Merkel cell carcinoma may be acceptable; since a primary extracutaneous tumor is expected to follow a less aggressive course than a metastatic one, follow-up data may provide indications as to the truly extracutaneous origin of Merkel cell carcinoma.     

Excision of selected skin tumours using Mohs' micrographic surgery with horizontal paraffin-embedded sections

Histological interpretation of frozen sections made during Mohs' micrographic surgery may be difficult, depending on the morphological and staining characteristics of the tumour and on the nature of the associated inflammatory infiltrate. We have employed an adaptation of micrographic surgery in which horizontal, formalin-fixed, paraffin-embedded sections were used to improve histological assessment in the excision of 18 non-melanoma skin tumours in which frozen sections had been or were likely to be unsatisfactory. We describe our experience of this method in the management of squamous cell carcinomas (11), extramammary Paget's disease (two), microcystic adnexal cell carcinomas (two), dermatofibrosarcoma protuberans (two), and primary cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) (one). The use of horizontal paraffin-embedded sections lengthens the duration of the procedure but facilitates accurate assessment of histological sections in selected tumours.     

Squamous cell carcinoma arising from a nonhealing wound and osteomyelitis treated with Mohs micrographic surgery: a case study

The treatment of choice for squamous cell carcinoma arising from chronic ulcer at the lower extremity has traditionally been amputation. Recently, Mohs micrographic surgery has been used as a limb-saving option in selected patients. One of these patients, a 69 year-old white male, presented with a two-year history of a progressive non-healing would and osteomyelitis. A four-month history of an enlarging mass within the ulcer suggested squamous cell carcinoma, which was confirmed by biopsies. The patient was treated with Mohs micrographic surgery for local eradication of the tumor, intravenous antibiotics, and skin grafting.     

Merkel cell tumor of the head and neck. Five new cases with literature review

Merkel cell carcinoma (MCC) of the skin is an uncommon, but highly aggressive neoplasm with a marked propensity for local and distant metastasis. Despite the fact that more than half of the 600 cases of MCC reported in the literature involved primary sites in the head and neck, MCC has rarely been discussed in otolaryngology publications. We present five new cases of MCC of the head and neck and summarize 89 additional cases from the literature in which detailed treatment and survival data were given. Our findings again emphasize the difficulty in making the initial histopathologic diagnosis of MCC and demonstrate the necessity of early diagnosis and multimodality treatment.     

The role of radiation therapy and chemotherapy in the treatment of Merkel cell carcinoma

BACKGROUND: Merkel cell carcinoma is a rare and highly aggressive skin tumor. The purpose of this study was to determine the role of radiation therapy and chemotherapy in the treatment of patients with Merkel cell carcinoma. METHODS: A retrospective analysis of 27 patients treated at Rabin Medical Center in Israel is presented, focusing on the treatment details. Data for 40 patients (the authors' 27 patients and an additional 13 patients from the Israeli Cancer Registry), were analyzed for prognostic factors using univariate and multivariate analyses. RESULTS: Univariate analyses revealed regional lymph node involvement and the coexistence of a second primary tumor as unfavorable prognostic factors. On multivariate analysis, only lymph node involvement showed borderline statistical significance. Radiation therapy was highly effective when given as consolidation after surgery or chemotherapy. In 11 patients irradiated effectively, only 1 (9%) in-field recurrence occurred. Radiation therapy yielded responses in 15 of 15 measurable sites (5 complete responses and 10 partial responses). Chemotherapy produced responses in 18 of 26 patients (69%), mostly complete (41%). However, in the absence of radiation therapy, the responses were short lived. CONCLUSIONS: These data support the use of combined treatment with chemotherapy followed by radiation therapy for patients with advanced locoregional Merkel cell carcinoma. In patients with metastatic disease, chemotherapy as well as radiotherapy can provide effective palliation. Further large scale investigations are warranted to confirm this approach.     

Childhood sarcoidosis presenting with hypercalcaemic crisis

Squamous cell carcinoma is a rare, but well documented complication of chronic osteomyelitis. Many authors have recommended amputation as the treatment of choice for locally invasive disease. Presented is a patient with squamous cell carcinoma arising in the draining sinus tract of chronic osteomyelitis of the lower extremity that was treated successfully with Mohs micrographic surgery (MMS). After ten year follow-up he remains tumor free and continues to enjoy use of his leg. We propose MMS as a therapeutic option to amputation for control of locally invasive disease.     

Mohs' method of micrographic surgery as treatment for recurrent basal cell carcinoma

OBJECTIVE: Evaluation of Mohs' micrographic surgery as treatment for recurrent basal cell carcinoma of the skin. DESIGN: Retrospective. SETTING: University Hospital Maastricht, the Netherlands. METHOD: In the period April 1992 to December 1995, 91 recurrent basal cell carcinomas (88 patients) were treated by Mohs' micrographic surgery. Medical records were analysed retrospectively with respect to different aspects. RESULTS: The mean age of the patients was 69 years. The recurrent basal cell carcinomas, with an mean diameter of 19.7 mm, were mainly localized on the nose and forehead. There were equal numbers of solid and morphea-like types of basal cell carcinomas. Most of these tumours had been treated by means of surgical excision in the past. The last treatment had taken place 3 years previously on average. Reconstruction was performed by means of primary closure, a graft or a flap. The mean follow-up period after Mohs' micrographic surgery was 12 months, in which one tumour recurred. CONCLUSION: Mohs' micrographic surgery is a surgical technique which provides the best prospect of total tumour removal together with maximal functional and cosmetic preservation. Mohs' micrographic surgery is of particular value for the treatment of recurrent basal cell carcinomas.     

Merkel cell tumor. Report of case and treatment with octreotide

Merkel cell carcinoma is a rare neuroendocrine tumor of the skin. Prognosis is very poor particularly when systemic disease is present. Surgery, chemo and/or radiotherapy treatment are not able to guarantee long survival and quality of life is also very poor. We know that neuroendocrine tumor can be in possession of receptors for somatostatin; during the past years, these receptors have been demonstrated in vivo by octreoscan. We report a case of a patient suffering from metastatic Merkel cell carcinoma; because he was elderly, neither chemotherapy nor radiotherapy were possible as a consequence of the explosion of the disease after surgery. The presence of receptors for somatostatin analogues (octreoscan) allowed treatment with octreotide causing the immediate disappearance of metastasis. After ten months of treatment the patient presents a complete remission of disease. Octreotide, the most important somatostatin analogue, represents a primary role in the neuroendocrine tumor management; the drug also lacks of toxicity.     

Merkel cell carcinoma in the nasal cavity: a case report

Merkel cell carcinoma is an aggressive dermal neoplasm of neuroendocrine origin with a predilection for the head-and-neck region in elderly patients. We present a case of Merkel cell carcinoma of the nasal fossa. Local wide excision was performed.     

Merkel cell carcinoma of the skin

Primary neuroendocrine carcinoma of the skin or Merkel cell carcinoma is an aggressive primary neoplasm. It is commonly seen in the elderly, on the head, neck and extremities, where it can mimic a benign or less malignant skin tumour. Pathological examination shows a generally dense growth of small dark cells, with immunohistochemical evidence of neuroendocrine differentiation. The microscopic appearance is very similar to metastatic oat cell carcinoma from the lung and this must be excluded by clinical means and appropriate imaging studies. In this study we present 13 new cases of Merkel cell carcinoma (the largest published series in the UK) and summarize 214 cases from the literature in which the survival data are given. In our series, 5 of 13 patients died from spread of the Merkel cell carcinoma. From this and other studies, it appears that early diagnosis and wide local excision may be the only way to prolong survival. No other adjuvant therapy has proved effective.     

Retinal thickness variation in the diabetic patient measured by the retinal thickness analyser

BACKGROUND: Acral melanomas are uncommon. Due to the thick overlying stratum corneum, accurate estimation of margins is difficult for minimally pigmented or amelanotic melanomas on the palm or sole. OBJECTIVE: To describe the use of Mohs micrographic surgery using frozen sections and HMB-45 immunostaining in the treatment of a multiply recurrent acral melanoma that had failed both standard surgery and Mohs surgery. METHODS: The melanoma was excised by Mohs technique, and the margins were checked by frozen section and HMB-45 immunostaining. RESULTS: The melanoma was completely excised in 11 stages, resulting in a defect that covered much of the plantar surfaces of the ball of the left foot, great, second, third, fourth, and fifth toes. No recurrence has been noted in 22 months of follow-up. CONCLUSIONS: HMB-45 immunostaining is a very valuable adjunct to examination of surgical margins for melanoma, particularly when combined with such histologic features as clustering of cells, melanocyte position within the epidermis, and cytologic atypia.     

Fibromatosis in infancy and childhood: the spectrum

Fibromatoses form an interesting group of tumors occupying a midposition in the spectrum of fibrous tissue neoplasms. Within the fibromatoses subgroups there exists a variety of tumors whose clinical behavior spans the range from completely benign to locally aggressive. Four case histories are presented to illustrate this point. All four patients were initially seen with asymptomatic head or neck masses. The first had complete excision with no recurrence to date. The second underwent spontaneous regression after confirmation of diagnosis with biopsy. The third case had a very locally aggressive disease requiring three surgical resections to date, while the last case had one recurrence after the initial resection. With certain exceptions, initial wide local excision offers the best possible chance for cure.     

Axillary basal cell carcinoma: a need for full cutaneous examination

Basal cell carcinoma is the most common skin malignancy. While this lesion most often occurs in sun-exposed areas of the skin, it can also develop in sites that are not usually exposed to sunlight or artificial ultraviolet radiation, such as the breast, palm or groin. A periodic complete examination of the skin should be performed to ensure that atypical presentations of basal cell carcinoma are not overlooked or misdiagnosed. Treatment options include curettage and desiccation, cryosurgery, surgical excision, radiotherapy and Mohs micrographic surgery.     

S-phase fraction as a predictor of prognosis in juvenile respiratory papillomatosis

OBJECTIVE: To determine whether DNA ploidy and the S-phase fraction are predictive of the clinical course in children with recurrent respiratory papillomatosis. DESIGN: Masked compression of DNA analysis findings to the clinical course of the disease. SETTING: Tertiary referral center. PATIENTS: All pediatric patients treated for recurrent respiratory papillomatosis at our institution between 1989 and 1995 who had adequate follow-up and whose primary biopsy specimen was available for examination. Fifty-five patients met these criteria. METHODS: Information was collected from the case notes on the patient's age at presentation, sex, sites of disease, duration of active disease, and frequency of operative interventions. Flow cytometric analysis was performed on the archival paraffin-embedded primary biopsy specimen obtained at the initial surgical excision, providing DNA content and percentages of S-phase cells. The investigators who performed the DNA analysis were masked to the clinical course. RESULTS: The age of the patients at presentation ranged from 3 months to 16 years. Thirty patients had involvement in more than 1 anatomical site. The disease in 10 patients had spread to the distal tracheobronchial tree. The patients underwent a total of 1124 procedures, with a frequency range of 7 to 27 per year. All cell populations studied were diploid. The percentage of S-phase cells was significantly higher in the primary biopsy specimen from patients with disease characterized by more frequent recurrences, multiple sites, and distal extension (P<.05). In multiple regression analysis, the S-phase fraction was found to be an independent and powerful prognostic factor for aggressive disease. CONCLUSIONS: The S-phase fraction may be predictive of the clinical course in patients with juvenile respiratory papillomatosis. Prospective studies are needed to assess the diagnostic and clinical value of our primary results and to determine whether DNA analysis can assist in identifying patients at increased risk for an aggressive clinical course.     

Correction of hypospadias

A case of Merkel cell carcinoma of the skin is described. It is a rare primary skin tumor originating within the dermis with an aggressive biologic behavior. Clinical presentation of these tumors are not sufficiently distinctive to allow the preoperative diagnosis. Wide surgical excision with postoperative irradiation to the local site and regional lymphatics is recommended as the therapy of choice. Indications for chemotherapy are discussed and the guidelines for an appropriated follow-up are reported.     

Surgical wound healing complicated by Aspergillus infection in a nonimmunocompromised host

BACKGROUND: An unusual complication of cutaneous surgery and its management is presented. Aspergillus flavus was identified from a nonhealing surgical wound of the ear undergoing cartilaginous necrosis in an immunocompetent host. OBJECTIVE: We wish to alert clinicians that Aspergillus may infect surgical wounds of the ear causing significant morbidity. METHOD: A healthy man underwent Mohs micrographic surgery for invasive Bowen's disease of the ear. Due to the size and location of the defect it was allowed to heal by secondary intent. The patient developed inflammation and subsequent destruction of the ear cartilage. Aspergillus was demonstrated by touch preps and cultured from swabs and tissue from the necrotic wound. RESULTS: In spite of aggressive topical and oral antifungal therapy severe distortion of the pinna occurred, resulting in surgical removal of the upper two-thirds of the ear. CONCLUSIONS: In the presence of cartilage necrosis following surgery on the ear, Aspergillus infection should be considered. Early aggressive management with surgical debridement, and topical and oral antifungal therapy may prevent destruction of the cartilage and a significant cosmetic defect.     

Primary pulmonary lymphoma--clinical review from a single institution in Singapore

Primary pulmonary lymphoma is a rare and vexing subset of extranodal non-Hodgkin's lymphoma. We report 11 cases and provide a brief literature review. We also highlight an unusual case of a relapsed peripheral T-cell primary lung lymphoma that underwent apparent spontaneous remission. Eleven cases of primary pulmonary lymphoma treated in our institution were studied for their clinical characteristics, behaviour, response to treatment and clinical outcome. The median duration of follow up was 26 months. The mean age was in the 50s and the presenting symptoms generally respiratory and non-specific. LDH levels did not correlate with either stage or grade of disease. Lower lobe involvement was most common and nodules and mass-like lesions the main radiologic feature. Small lymphocytic lymphoma accounted for the majority of cases and were indolent in behaviour. Good symptom control and radiologic response was achieved with chemotherapy in disseminated low grade lung lymphomas. Combination chemotherapy was effective in the aggressive lymphomas. In conclusion, Small lymphocytic lymphoma of the lung is an indolent disease with a long symptom-free survival even after recurrence. Our series confirms the clinical characteristics of primary pulmonary lymphoma. The role of Ling Zhi in effecting the spontaneous remission in the peripheral T-cell lymphoma is speculative.     

Small-intestinal digestion of partially resistant cornstarch in healthy subjects

Primary adenocarcinoma of sweat glands is a rare tumor; approximately 220 cases have been reported in the last 30 years. We reviewed the charts of patients with primary diagnosis of this tumor treated at the Mayo Clinic between 1935 and 1995. We included only cases with initial histology slides available for re-examination. Tumors were classified into five recognizable histologic patterns (solid, ductal, mucinous, microcystic adnexal, and adenocystic carcinoma) and graded by the Broder system. Statistical analysis consisted of Kaplan-Meier product limit method and Cox multiple regression test. In total, 55 patients were identified, and age ranged from 13 to 85 years (mean 59 years). Thirty-six patients (65 percent) presented to the Mayo Clinic for initial treatment; all except one had disease limited to the primary site. Microcystic adnexal carcinoma was the most frequent type, and more than 50 percent were grade 2 tumors. Among these 36 patients, 4 had some type of recurrence. Patients who developed metastasis had a high-grade tumor in the initial biopsy. Nineteen patients were referred with recurrence; 13 had local recurrence, 4 had regional diseases, and 2 had distant metastases. The histologic distribution showed 47 percent solid tumors, and 37 percent of them were grade 3. Multiple regression analysis did not show a difference in recurrence or survival when gender, age, tumor location, or histologic pattern was evaluated. In addition, there was no difference in the outcome between wide surgical resection and micrographic surgery. The only predictive factor for distant metastases and/or death (p < 0.003) was histologic grade. Overall 10-year survival rate was 86 and 60 percent for primary and referred patients, respectively. We conclude that histologic diagnosis of sweat gland carcinoma must be complemented by clinical examination to evaluate metastases. Clinical behavior depends on the histologic type of tumor, degree of differentiation, and clinical stage. On recurrence, the likelihood of further recurrences and mortality increases dramatically. Aggressive initial local ablation with tumor-free margins is recommended. In high-grade tumors, prophylactic regional lymph node dissection may further characterize tumor aggressiveness and may justify adjuvant radiotherapy as part of the primary treatment.