Non-hematopoietic cutaneous metastases in children and adolescents: thirty years experience at St. Jude Children's Research Hospital.

BACKGROUND: The spectrum of cutaneous metastasis of non-hematopoietic neoplasms in the pediatric population is not well documented. We report the histologic diversity of this unusual process over a 30-year period at a tertiary care center for pediatric malignancy (St. Jude Children's Research Hospital, Memphis, TN, USA). METHODS: Of 1,971 pathology accessions which included histologic material on skin (1,604 surgical cases and 367 autopsy cases) we found 40 cases (2% of total skin accessions) coded for metastatic non-hematopoietic malignancy. RESULTS: The patients (n=34) ranged in age from 1 month to 20 years (mean=9.8 years) and had a male:female ratio of 1:1. The histologic diagnoses were as follows: rhabdomyosarcoma NOS (6 cases), embryonal rhabdomyosarcoma (4 cases), alveolar rhabdomyosarcoma (4 cases), neuroblastoma (8 cases), osteosarcoma (2 cases), choriocarcinoma (2 cases), peripheral neuroepithelioma or Ewing's sarcoma (2 cases), malignant rhabdoid tumor (1 case), paraganglioma (1 case), nasopharyngeal carcinoma (1 case), sarcoma NOS (1 case), colon adenocarcinoma (1 case), and malignant melanoma (1 case). CONCLUSIONS: Cutaneous or subcutaneous metastasis of non-hematopoietic malignancies in children and adolescents is a rare occurrence but in a high percentage of cases may be the first manifestation of disease. The tumors most likely to metastasize to the skin in children are rhabdomyosarcoma and neuroblastoma and they are more likely than adult malignancies to disseminate to multiple distant sites.

A fatal case of pancreatic panniculitis presenting in a young patient with systemic lupus.

BACKGROUND: Subcutaneous fat necrosis associated with pancreatic disease is a rare event. The clinical cutaneous findings are non-specific erythematous nodules with central softening located predominantly on the lower extremities. The histopathologic features of these lesions are very characteristic and diagnostic. METHODS: We present an unusual case of pancreatic panniculitis associated with lupus pancreatitis in a 21-year-old African American female. The patient presented with lower extremity skin nodules, arthralgia, and serositis prior to the diagnosis of systemic lupus and pancreatitis. The skin lesions progressed despite normalization of serum pancreatic enzymes. Following femoral vein catheterization for renal dialysis, she developed a large indurated area over the left lower quadrant, flank, groin, and upper thigh measuring 25 cm. She was treated with repeated debridement, tissue grafts, and hyperbaric oxygen because of a clinical suspicion of necrotizing fasciitis. RESULTS: Examination of skin biopsies and debrided tissue revealed the pathognomonic features of pancreatic panniculitis without any evidence of necrotizing fasciitis. Organisms were not detected by tissue examination or microbiologic cultures. CONCLUSIONS: This case illustrates the potential role of vascular trauma in the pathogenesis of pancreatic panniculitis.

Metastatic adenocarcinoma of the breast located within a benign intradermal nevus.

We report an exceptional case of metastatic adenocarcinoma of the breast occurring within a benign intradermal nevus. An extensive review of the literature on metastatic breast carcinoma is included.

Necrosis of the hand after extravasation of intravenously administered phenytoin.

Extravascular escape of intravenously administered phenytoin can result in serious local soft tissue complications. Injury can range from simple phlebitis to chemical cellulitis or, in extreme cases, frank tissue necrosis that necessitates amputation. The histopathologic findings include extensive necrosis and sloughing of epidermis, widespread necrosis of dermis, subcutaneous tissue, muscles and nerves, and extensive thrombotic occlusion of vessel lumens. Results of elastic tissue stains reveal that the thrombosed vessels are exclusively veins and venules. Vascular fibrinoid necrosis, leukocytoclasis, and true inflammatory vasculitis are not features. This is the first detailed report of the histopathologic alterations associated with this therapeutic misadventure.

Efficacy of bimolane in the Malassezia ovalis model of psoriasis.

Bimolane, an analog of razoxane has been used in China with comparable efficacy but less toxicity than razoxane in the treatment of psoriasis. In an attempt to characterize further its mode of action it was administered both systemically and topically in the Malassezia ovalis animal model of psoriasis. Intravenous methotrexate and topical 0.1% betamethasone valerate were also used as positive control treatments. The animal model of psoriasis was effectively treated by bimolane, both systemically and topically, and also by parenteral methotrexate and topical betamethasone valerate. The time course of bimolane's effect with this model was different from methotrexate's suggesting the possibility of a different mode of action. Because bimolane, like razoxane, is an ethylene diamino tetraacetate acid (EDTA) derivative, it is possible that its effects on this reaction relate to its chelating properties and that inhibition of complement activation is important to its mode of action.

Squamous cell carcinoma of the breast: a clinicopathologic analysis of eight cases and review of the literature.

Malignant neoplasms of the female breast composed of squamous cells are rare and constitute a particularly poorly understood facet of breast disease. Two pure squamous cell carcinomas ( SCCs ) of the breast and six tumors that displayed various combinations of malignant squamous elements and ductal breast adenocarcinoma were identified. Electron microscopy confirmed the diagnosis of SCC in two cases by the demonstration of regularly spaced desmosomes, tonofilament bundles, and keratohyaline granules. A review of the literature indicated that breast cancers with components of SCC are histologically variable. Approximately 30 cases of pure SCC and 80 cases of mixed adenocarcinoma and SCC (adeno-SCC) have been reported. Some of these squamous epithelium-containing tumors have displayed areas of cyst formation, spindle cell metaplasia, and pseudosarcomatous desmoplastic response, or origins in preexisting breast lesions, such as cystosarcoma phyllodes or dermoid cysts. Although this variability indicates a heterogeneous group of neoplasms, the overall prognosis for carcinomas of the breast with malignant squamous elements appears to be quite similar to that for ordinary breast adenocarcinomas of similar size and stage.

Human pulmonary dirofilarial granuloma.

A case of pulmonary dirofilariasis in a human being is presented in which the lesion was visible roentgenographically for eighteen months prior to resection and in which the correct etiological diagnosis was made on frozen-section examination at the time of thoracotomy. The documented geographical spread of dirofilariasis in the canine population in the United States and southern Canada suggests that pulmonary dirofilariasis in human beings may be seen with increasing frequency by thoracic surgeons. The epidemiological and pathogenetic features of this unusual disease are discussed. A serological test for dirofilariasis is now available that may allow some patients to escape thoracotomy for this self-limited disorder.

Atheromatous embolization to the skin.

Atheromatous emboli were found in arterioles in the dermis of the ear of a 74-year-old man. This distinctive form of arteriosclerosis disease is well recognized as a cause of renal failure in nephropathology, but its significance is not sufficiently appreciated in dermatopathology. Atheromatous emboli may cause infarcts in the skin and biopsy of the skin may be a reliable method for diagnosis of systemic atheromatous emboli.

Cytomegalovirus infection of the skin.

A case of fatal generalized cytomegalovirus infection with particular attention to lesions involving the skin is reported. Characteristic cytomegalovirus inclusion bodies were present within nuclei of endothelial cells in the dermis beneath ulcerations. Skin biopsy may be useful in the diagnosis of disseminated infections by cytomegalovirus.

Carcinoma of sebaceous glands on the head and neck. A report of four cases.

Carcinoma of sebaceous glands most commonly occurs on the eyelids and caruncles of the elderly. Local recurrences after would-be adequate treatment and distant metastases are common in this malignancy in this site. Carcinomas of sebaceous glands arising elsewhere in the skin also have a strong tendency to recur locally, but are said to be much less likely to metastasize. Our experience with four such carcinomas on the head and neck have led us to believe that the malignancy is biologically aggressive anywhere if not adequately treated at the onset.

The cytological diagnosis of pulmonary blastomycosis.

We reviewed 30 episodes of pulmonary blastomycosis among 29 patients to compare the methods of diagnosis and to assess the usefulness of cytological sputum examination. Complement fixation and skin tests were not helpful in diagnosis. Confirmation by culture of sputum or tissue required, on average, five weeks. We compared the cytological examination of bronchial secretions with that of microbiologic wet smears. The cytological technique yielded a positive diagnosis in 93% of patients and in 70% on the first specimen. Sixty-one percent of microbiologic wet smears proved positive, with 32% positive on the first specimen. The cytological findings were reported significantly sooner. In addition, cytological diagnosis of Blastomyces dermatitidis in sputum and bronchial washings of patients suspected of having a pulmonary cancer or infection reduced the time of diagnostic workup and the incidence of major surgery.

Porphyria cutanea tarda complicating Wilson's disease.

A young woman is described in whom symptomatic porphyria cutanea tarda (PCT) developed during copper chelation therapy for Wilson's disease. Termination of ethanol ingestion and oral contraceptive use resulted in cessation of blistering skin lesions and reduction in urinary porphyrin excretion. This is the first recorded coincidence of these two rare hepatic diseases. Therapeutic implications are discussed.

PIP: A case report is presented of a young woman in whom symptomatic porphyria cutanea tarda (PCT) developed during copper chelation therapy for Wilson's disease. The 22 year old white woman was seen in the summer of 1978 because of development of blisters on the dorsa of the hands associated with focal atrophic hypopigmentation, generalized hyperpigmentation of the skin, and hpertrichosis of the lateral forehead and face. A sibling had died in childhood with Wilson's disease. When the patient developed hepatomegaly, ascites, and an acute hepatitis syndrome at the age of 11, penicillamine therapy was empirically started, with gradual symptomatic improvement. When evaluated at the age of 22, abnormal laboratory values included a total bilirubin of 1.2 mg%; alkaline phosphatase, 96 U; serum glutamic oxaloacetic transaminase (SGOT), 175 U; serum glutamic pyruvic transaminase (SGPT), 122 U; gamma glutamyl trans peptidase (GGTP), 64 U; and Bromsulphalein (BSP) retention, 21% at 45 minutes. Skin biopsy from the hand revealed a noninflammatory subepidermal bulla with prominently PAS positive vessel walls in the festooned dermal papillae at the base of the blister. A fragmented liver biopsy failed to reveal evidence of active hepatitis or cirrhosis, but considerable stainable iron was present in both hepatocytes and Kupffer cells. A rubeanic acid stain for copper was negative. The patient was diagnosed as having Wilson's disease, hepatic hemosiderosis, and PCT. Cessation of all ethanol consumption and discontinuation of the oral contraceptives which she had been taking for 6 years, was recommended. On examination 9 and 22 months after these modifications were instituted, the patient felt asymptomatic and was without evidence of any new blisters or scars of her skin. The hyperpigmentation and hypertrichosis persisted, but she rigidly adhered to a program of penicillamine, topical sunscreen application, and abnegation of alcohol. Liver function studies were normal, and urinary porphyrin levels returned toward normal values. The clinical onset of this patient's blistering disease was temporally associated with ethanol and exogenous estrogen medication.