Indian Journal of Pathology and Microbiology

: 2009  |  Volume : 52  |  Issue : 3  |  Page : 374--376

Congenital neuroblastoma with liver metastasis presenting with Hashimoto Pritzker disease

E Sajini Jacob1, Renu G Varghese1, Pampa C Toi1, R Bhaskaran2, Ramji Rai1,  
1 Department of Pathology, Pondicherry Institute of Medical Sciences, Kalapet, Puducherry - 605 014, India
2 Department of Pediatric Surgery, Pondicherry Institute of Medical Sciences, Kalapet, Puducherry - 605 014, India

Correspondence Address:
E Sajini Jacob
Department of Pathology, Pondicherry Institute of Medical Sciences, Kalapet, Puducherry - 605 014


A two-month-old female child presented with discrete skin-colored papules on the forehead and scalp. She was earlier diagnosed to have neuroblastoma of the adrenal gland which had metastasized to the liver. Histological sections of the skin lesion showed a nodule composed of sheets of cells with abundant eosinophilic cytoplasm and an occasional grooving of the nucleus. These cells were CD 68 positive. Eosinophilic abscesses were seen in between the neoplastic cells.

How to cite this article:
Jacob E S, Varghese RG, Toi PC, Bhaskaran R, Rai R. Congenital neuroblastoma with liver metastasis presenting with Hashimoto Pritzker disease.Indian J Pathol Microbiol 2009;52:374-376

How to cite this URL:
Jacob E S, Varghese RG, Toi PC, Bhaskaran R, Rai R. Congenital neuroblastoma with liver metastasis presenting with Hashimoto Pritzker disease. Indian J Pathol Microbiol [serial online] 2009 [cited 2021 Jun 20 ];52:374-376
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Full Text


Congenital self-healing reticulohistiocytosis (CSHRH) or Hashimoto Pritzker disease is a variant of Langerhan cell disease. [1] It presents on the skin as scattered papules and nodules. Usually, the lesion regresses within 12 months. Though there are occasional reports of concurrent neuroblastoma and Langerhan cell histiocytosis, [2],[3] the presence of CSHRH with neuroblastoma has not been documented. We report a case of congenital neuroblastoma presenting with skin lesions at two months of age.

 Case Report

A 20-year-old prima gravida, when examined in the 36 th week of pregnancy by ultrasonogram was diagnosed to have a fetus with neuroblastoma of the left adrenal gland. A term 2.76 kg, appropriate for gestational age baby was delivered. Fifteen days after birth, the baby was taken up for surgery.

On the operation table, the abdomen was opened and splenic flexure and descending colon were mobilized. An 8 x 6 cm-sized tumor was found in the left lumbar region. [Figure 1] The tumor was hemorrhagic. The kidney was not infiltrated by the tumor. It displaced the kidney downwards and laterally. The tumor was mobilized and excised after all the blood supply to the tumor was ligated. A biopsy was taken from the liver. Histopathological examination of the adrenal mass revealed a neuroblastoma [Figure 2] with vascular invasion and a metastatic deposit in the liver.

The postoperative period was uneventful. She was advised close follow-up with no chemotherapy. The baby was well and gained weight. Two months later discrete skin-colored nodules were seen on the forehead and scalp. These nodules were about 0.3 to 0.5 cm in size. A biopsy of one of the nodules was done.

Gross examination of the skin biopsy showed a single elliptical skin-covered tissue measuring 1 0.5 0.5 cm. Surface of the skin showed a nodule measuring 0.5 cm in diameter. Cut surface was gray-white in color.

Microscopy of multiple sections from the nodule showed a thinned out epidermis. The dermis showed a well-circumscribed nodule composed of sheets of cells with abundant eosinophilic cytoplasm [Figure 3]. The nucleus was vesicular, round to oval with an occasional grooving [[Figure 3] inset]. Numerous foreign body and Touton giant cells were seen. Eosinophilic micro-abscesses were seen [Figure 3]. Immunohistochemistry of the lesion showed that the tumor cells were positive for CD68.


Congenital self-healing reticulohistiocytosis (CSHRH) was first described in 1973 by Hashimoto and Pritzker. [1] Though these lesions are usually present at birth they may not appear for weeks after delivery. The lesion usually regresses within 12 months. The actual incidence is under-reported because it has a high rate of spontaneous regression. It is also less clinically recognized. [4]

Since these skin lesions occurred in a child with congenital neuroblastoma, metastatic neuroblastoma was considered. But metastatic neuroblastoma of infancy usually presents a picture of blueberry muffin baby or as blanching subcutaneous nodules. [5],[6] Clinically, the other variants of Langerhan cell disease were not considered here, as apart from the skin lesions no other organs were involved. Though acute disseminated variant may present at birth, the skin lesions are not nodular. By histological criteria alone it is not possible to differentiate CSHRH from other types of Langerhan cell disease. But it is said that the Langerhan cells in CSHRH have fewer Birbeck granules. They have laminated dense bodies that can be visualized by electron microscopy. [7]

Other histiocytic skin lesions which can present in the neonate include juvenile xanthogranuloma and benign cephalic histiocytosis. Juvenile xanthogranuloma, like CSHRH, can present at birth with spontaneous regression. In this lesion, though you get collections of macrophages, the giant cells seen are mainly of the Touton type. [8] In our biopsy the giant cells were mainly of foreign body type. Eosinophilic micro-abscesses and nuclear grooving have not been described in juvenile xanthogranuloma. A similar self-healing lesion limited to the skin is benign cephalic histiocytosis. The present lesion did not clinically fit into benign cephalic histiocytosis, since these lesions are red or yellow in appearance, whereas our lesion was skin-colored. Benign cephalic histiocytosis is common during the first three years of life. [9]

Cases of CSHRH should be carefully followed up as relapses can occur. Rarely, it can progress to acute disseminated state.[10] Recently, our patient came for review after 12 months. Most of her skin lesions had regressed. Another important point of significance of this case is that this is a rare case of concurrent CSHRH and neuroblastoma.


CSHRH is one of the spontaneously regressing skin disorders that occur in the neonate. It should be one of the differential diagnoses for histiocytic disorders occurring in the neonate. Here we have reported this case as it was seen in association with congenital neuroblastoma which is very rare.


We thank Col Dr. Anurag Mehta Prof & H.O.D, Department of Pathology, A.F.M.C, Pune for his help with the immunohistochemistry.


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